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·TOWARD THE
DIAGNOSIS OF
CONGENITAL HEART
DISEASE
by
W CARLETON WIITESIDE, MD
FRCS(C) FACS, FICS DARTS
Victoria BC Canada
MOM
CHARLES C TII OMAS PUBLISHER
Springfield • Ilmow USA

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·THE HEART IS THE TOUGHEST STRONGEST
MOST PERSISTENT AND TENACIOUS ORGAN
AND
CARRIES
ON AND ADAPTS
ITSELF
AGAINST ALMOST ANY ODDS OFTEN FOR A
TIME
WITHOUT
SUPPORT
OF
OTHER
ORGANS
DR GORDON MURRAY

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·- - ALPHA — INTRODUCTION Many congenital diseases are not fully or correctly diag nosed until they come face to face with the cardiac surgical team performing the surgery Since the cardiac surgical field has expanded over the past decade the incidence of incorrect clinical diagnoses has decreased amazingly The Cardiologist, the Paediatrician the General Practitioner and the Cardiac Surgeon through team work hase caused these favorable diagnostic results There is no reason why a 95% diagnostic accuracy pre-operatively cannot be attained in the future Doctors are unable to look up the diagnosis as they can the treatment of the disease once the diagnosis has been made We must make the diagnosis from the symptoms and the signs presented by the patient In the case of an infant it is much more difficult for obvious reasons The diagnosis of a disease being the most important aspect of the practice of medicine or surgery it is tantamount that we learn the signs and the symptoms which are char acteristic of each disease After much logical consideration we should arrive at the correct diagnosis It is important in the diagnostic field that we be aware of all the other diagnostic possibilities If we are not aware of a disease or defect how then are we able to consider it in the diagnosis? It is what we do not know that is often more important than what we do know The education of a doctor should never cease Once we cease leaming the new and much of the old we soon become lost and wade blindly through the mure of despondency and V21 ·vui Toward The Diagnosis of Congenital Heart Disease ignorance We must keep abreast of the advances in medı cine through travelling reading, practical experience and carrying out research whenever possible The doctor should always be a student of his art In the following pages the student will find much common knowledge and perhaps some which is new Due to recent advances and greater interest in cardiovascular lesions it behooves us to be able to help these little patients who are brought for help and information regarding their particular ailment The present day public is too well informed through the medium of the press to accept many cloaked answers from their doctors as Just one of those things "He or she will grow out of it or that Nothing can be done They want to know and rightly so and they will find out what the trouble is and that something can be done It is our ethical duty to tell them correctly at the beginning of possible Too many neurotics have been made through the wrong diagnosis or misinformation or no information at all More mistakes are made by not knowing or by not looking than by knowing and not telling or using this knowl edge There is nothing worse than ignorance in motion If our diagnosis is correct then our treatment should be of benefit It is then that the patients are happiest and a better public relationship exists There is very little magic" left in medicine-only cold facts We should examine and re examine until we are sure of our diagnosis or diagnoses In medicine we should never take anything for granted but study and screen each patient carefully and follow the Golden Rule in so doing There may be exceptions to many rules but with an open mind and logical thinking each statement as listed within these covers may be found to be steeped in greater information At times all we need in a diagnosis is a "lead" There are many leads" if we but know of them The following arrangements classifications and abbrevi ·Touard The Diagnosis of Congenital Heart Disease LX ated statements on congenital heart disease are not original but are gathered from many authoritative and long experi enced cardiologists and cardiac surgeons throughout the world The author has attempted to collect a bouquet of other men s flowers and only the ribbon with which they are bound belongs to him These are arranged for possible easy reference because voluminous works too frequently obscure good judgment In conclusion I wish to extend my sincere gratitude to the following for their instruction and early encouragement to me in the field of thoracic and cardiac surgery Professor Clarence Crafoord of Stockholm Sir Russell Brock Sur Price C Thomas and Mr Holmes Sellors of London Professor Erik Husfeldt of Copenhagen Mi George Mason of New castle and Professor PR Allison of Oxford To Dr Alfred Blalock Dr Richard Overholt Dr Robert Gross Dr Charles Bailey Dr Claude Beck Dr George C Griffith and Dr Henry Swan of the United States To Dr WG Bigelow of Toronto Dr Pere Rendondo-Ramerez of Mexico City and many others from whom I have learned much through my association with them I am especially grateful to the Cardiologists Dr Charles B Rich and Dr Gordon Bell of the University of Alberta for their patience and helpful advice in reviewing the manu script for me The author accepts any and all responsibilities for errors or misinterpretations found within the covers of Toward the Diagnosis of Congenital Heart Disease Any statement is not necessarly the opinion of the reviewers My thanks are extended to Miss K Carley of the Labora tory Department of the Royal Jubilee Hospital for her ability to decipher and to type the manuscript from its orginal written form To others who have helped in any way my thanks are extended and last but not least to the publisher I am most grateful WC WHITESIDE MD Victoria BC

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·CONTENTS Page 11 3 5 20 23 25 Alpha Introduction Means of Diagnosing Heart Disease Some General Diagnostic Principles Clinical Diagnosis of Heart Disease in Children (Patterson) Foetal Anoxia and Cyanosis in the Newborn The Congenital Heart Diseases A Classification (Paul Wood) A Classification of Congenital Malformations of the Heart (Maurice Campbell) Patent Ductus Arteriosus Coarctation of Aorta Aortic Arch Vascular Rings Subaortic Stenosis Atresia of Aortic Orifice Right Aortic Sinus Aneurysm--Valsalva Aortic Pulmonary Window Tetralogy of Fallot Pulmonary Stenosis with Normal Aortic Root Pure Pulmonary Valvular Stenosis Pulmonary Atresia Pulmonary Stenosis with Tricuspid Atresia and Underdeveloped Right Vertricle Ebstein s Anomaly of Tricuspid Valve Types of Pulmonary Venous Connection The Eisenmenger Complex Transposition of the Great Vessels Interauricular Septal Defect Interventricular Septal Defect 27 28 32 35 37 38 39 40 41 44 49 51 52 53 54 56 59 60 63 X2 ·Toward The Diagnosis of Congenital Heart Disease XII Page 66 67 68 69 Mitral Atresia Mitral Stenosis (Congenital) Lutembacher Syndrome Cor Triloculare Biatratum Taussig Bing Complex Dextrocardia Tricuspid Atresia Persistent Common Atrioventricular Canal Bland White Garland Syndrome Persistent Truncus Arteriosus Cor Biloculare Congenital Pulmonary Arteriovenous Fistulae The Principle Clinical Findings in Cyanotic Heart Lesions When Should an Operation Tale Place Cardiac Arrest The Diagnosis and Treatment Omega 70 71 72 73 74 75 76 77 79 80 81 86 ·TOWARD THE DIAGNOSIS OF CONGENITAL HEART DISEASE ·CODE FOR ABBREVIATIONS APW-Aortac-Pulmonary Window AV-Arteno Venous A V Communis-Atrio Ventricular A AVR-Aortic Arch Vascular Ring BBB Bundle Branch Block (Rt or Lt) CA O C of A -Coarctation of Aorta CA-When associated with topic means Cardiac Arrest DD-Differential Diagnosis ECG-Electrocardiogram FO or PFO -Patent Fossa Ovalıs IASD or ASD-Interauricular Septal Defect IVSD or VSD-Interventricular Septal Befect IVC Inferior Vena Cava ICS --Intercostal Space LV-Left Ventricle LA-Left Auricle MV Mitral Valve MS Mitral Stenosis MI Mitral Insuffeciency PA Pulmonary Artery PS-Pulmonary Stenosis PPS-Pure Pulmonary Stenosis PDA-Patient Ductus Arteriosus AOA -Right Oblique Anterior R A -Zught Aurcle RV-Right Ventricle SAS-Sub Aortic Stenosis SVC-Superior Vena Cava SABE-Sub Acute Bacterial Endocarditis TA-Tricuspid Atresia TV-Trcuspid Valve T of F-Tetralogy of Fallot TGV-Transposition of Great Vessels VA-Venous to Artery V Ventricle Rt. or Lt 2 ·MEANS OF DIAGNOSING HEART DISEASE The following is a guide which if followed should bring the diagnosis to those who seek it 1 know the embryology anatomy biochemistry and the physiopathology of heart disease 2 know of all closely similar lesions which may exist 3 Possess a good diagnostic acuity and be logical 4 A complete history of the patient and two or three examinations in a quiet well lighted room If the patient is a child or an infant then a mild sedative will bring nch rewards 5 Examine before and after exercise and babies when asleep if possible 6 Position the patient in order to bring out sounds and murmurs to the advantage of the examiner Eg left lateral recumbent position for mitral disease 7 Use fluoroscopy and x rays in the most advantageous positions One examination is often not sufficient А lateral film should always be taken and other positions where indicated 8 The ECG and phonocardiograms 9 The CBC haematocrit sedimentation rate and blood cultures 10 Angiocardiograms arteriograms oesophagograms venograms and venous pressures 11 Catheterization for pressures and o left and nght chambers and great vessels 12 Circulation time, dilution dye tests and otmetry (ear) 13 Use good clinical judgment in all aspects of differential diagnosis in 3 ·SOME GENERAL DIAGNOSTIC PRINCIPLES (Congenital and Acqwred Lesions) 1 Central cyanosis indicates a right to left shunt (VA) 2 Central cyanosis is best seen in the tongue and the mucous membranes Clubbing of the digits is frequently present 3 Cyanosed nails in the newborn is in keeping with central cyanosis which means an opening between both sides of the heart and something else wluch raised the pres sure in the right side above that of the left Eg Pul monary stenosis Pulmonary hypertension Atelectasis 4 In central cyanosis and diminished pulmonary flow pa tients may be helped through operation 5 A diminished or increased blood flow to the lungs can be recognized by x ray fluoroscopy and the circulation time 6 Peripheral cyanosis does not indicate heart disease per se though it is common with mitral and pulmonary stenosis and equally in many normal children on a cold day 7 Congestive heart failure rarely occurs in childhood in the natural course 8 If the liver is enlarged in the presence of a gallop rhythm and cyanosis it is not apt to be Tetralogy of Fallot (T 9 "A small heart is a normal heart" (unless compressed) 10 Convulsions may be due to anova due to a shunt causing a low pulmonary pressure as seen in pulmonary stenosis (PS) of F) 4 ·Toward The Diagnosis of Congenital Heart Disease 5 11 An interventricular septal defect (IVSD) is the com monest cause of heart failure in children However cases of PDA and CA should not be overlooked in the diagnosis 12 If the BP is elevated in the arms then check the lower limbs for coarctation of the aorta (C of A) A throm bosis in any artery of the body will cause hypertension in varying degrees Eg Pulseless disease 13 Congestive failure in infancy is initially charactenzed by rapid respirations liver enlargement and rales 14 The blood pressure should always be obtained in all limbs in congenital heart disease and in all cases with hypertension 15 Questions concerning x rays of the heart (a) Which chambers are enlarged? (b) Which great vessels are enlarged and are both pres ent normal and in normal position? Are their branches normal? An RO A film shows the pul monary artery enlargement best (c) Is the systemic venous return normal? (d) Is the pulmonary venous return normal? 16 The presence of an atypical continuous murmur should always raise the possibility of a complete absence of the pulmonary arteries (Blood may pass via the bronchial arteries ) 17 A narrow great vessels shadow may indicate a hypo plastic aorta Eg (a) A V communis (b) Complete transposition of the great vessels The shadow is due to the two vessels lying behind the other in a PA view 18 Anginal pain in infants may be due to anomalous origin of the left coronary artery 19 In cerebral anoxia with convulsions in a baby who has slow respirations where clinically rapid ones would be expected the aorta most certainly arises from the RV ·6 Toward The Diagnosis of Congenital Heart Disease or is receiving large amounts of venous blood therefrom 20 Low voltage in the ECG may mean that the coronaries receive venous blood and an anoxic state exists 21 Pulseless disease is due to local arteritis with secondary thrombosis of the vessels arising from the aortic arch characterized by syncope amnesia blach outs, paresis and absent pulse in these vessels It is found more frequently in the adolescent female but thrombosis oc curs in old age from arteriosclerosis at the same sites 22 A wide pulse pressure suggests PDA aortic insuli ciency A V aneurysm heart block hyperthyroidism, arteriosclerosis of the aorta or an aortic pulmonary win dow or fistula 23 A narrow pulse pressure suggests shock aortic stenosis myocardial weakness cardiac tamponade or constructive pencarditis 24 In persistent tachycardia check with the ECG It may not be from hyperthyroidism anxiety or infarction 25 A large heart is an abnormal heart DO NOT mistake the wide compressed heart of pectus excavatum or kyphoscoliosis for an enlarged heart (See a lateral film of the heart ) 26 Diastolic murmurs are usually organic In congenital heart disease an apical diastolic murmur does not imply organic mitral valve disease even though organic heart disease exists 27 The FCG for coronary disease should be done follow ing or during exercise only if the heart is normal at rest 28 Dyspnoea is the chief symptom of heart disease and psychosis but may anse from pulmonary disease anae mia acidosis disease of the nerves, brain and the muscles of respiration If the heart size is normal there is apt to be a pulmonary cause for the dyspnoea One should exclude the hyperventilation syndrome 29 Cough is not often due to heart disease unless the lungs ·Toward The Diagnosis of Congenital Heart Disease are involved secondarly Eg Pulmonary hypertension pulmonary oedema or emboli 30 Pulmonary hypertension may cause pain similar to that of coronary disease but more frequently the hypertension 15 associated with dyspnoea asthma, emphysema V SD PDA ASD and mitral stenosis should be ruled out as a cause of pulmonary hypertension 31 Splenic colon flexure pain slipping costal cartilages and Tietzies disease may sunulate coronary pain 32 Examine the apical area in the left lateral recumbent position for best results 33 After the age of 50 calçıfic aortic disease is common 34 When all the pulmonary veins drain into the right auricle, then the lungs are congested The right auricle is greatly enlarged 35 A V shunts are found in the following (a) Anomalous pulmonary veins (b) IASD (c) PF Ovale (large) (d) A V ventricularis communis (e) IVSD (f) Aortic septal defect (Aortic pulmonary fistula) (g) Overndung pulmonary artery (h) Coronary sinus and truncus arteriosus (1) PDA (1) Ruptured sinus of Valsalya (k) A V aneurysm of the lung (Some consider this a V A shunt) (1) Traumatic and inflammatory lesions and new growths rupturing into an artery and vein 36 Cerebral anoxia in the infant or child may show (a) Slow breathing (apnoea) (b) Unconscious spells (c) Later the child may squat to overcome the spells ·8 Toward The Diagnosis of Congenital Heart Disease as is seen in Tetralogy of Fallot and other right to left shunts 37 The absence of a murmur in a cyanosed child raises the question of complete pulmonary atresia (Isolated PS with or without I ASD always has a murmur and is always best heard in the back ) 38 An unexplained fever of a weeks duration is sufficient grounds for suspecting bacterial endocarditis in a patient with known valvular or septal disease 39 PDA (Patent Ductus Arteriosus) may have a systolic murmur only in early life 40 Lest we forget pericarditis look for it more often The right pericardio enic angle is frequently observed to be acute in x ray films 41 Pulmonary emboli are often overlooked They are com mon post operatively They may cause pleuritic pain only or be lethal due to the large size of the embolus 42 Use nitroglycerine just prior to exertion to test the pres ence of coronary disease Coronary dilatation may be come irreversible with prolonged use of nitroglycerine causing thrombosis due to slowing of the blood serum 43 Haemoptysis may arise from pulmonary hypertension One may note frequent chest colds” from A V shunts many times mistaken for "bronchitis” 44 Leg cramps coldness numbness and non healing ulcers may be due to coarctation of the aorta (C of A) or similar arterial blockage Do not mistake for Buergers or LeRiches disease 45 Look for a congenital heart defect in a "squatting" child Children may also entwine their legs or lean on their laps This is more often associated with Tetralogy of Fallot 46 Pulmonary stenosis causes dyspnoea in a child best seen on a well populated school ground Watch the child who lags behind or who is squatting It is ·Toward The Diagnosts Of Congenital Heart Disease 9 47 Dysphagia and stridor in an infant may be due to a vascular ring about the trachea and oesophagus to an abnormal subclavian artery (Vasa lusoria), to severe pectus excavatum or an enlarged thymus 48 Increased venous pressure is found in pericarditis tamponade mediastinal tumors right heart failure, vena cava syndrome Ebsteins disease and tricuspid stenosis 49 Look to the lungs and mediastinum in obstruction of the vena cava (superior) 50 Lower body cyanosis in a child with clubbed toes may be found in infantile type of coarctation of the aorta (C of A) In adults these findings suggest a PDA with a reverse flow 51 "Bırd like movements of patients are seen in thyrotoxic heart disease 52 Alcoholics suffer from vitamin deficiency and may have enlarged hearts (abnormal) 53 Splinter hemorrhages may be seen in the nails and one may find a Grade II murmur present with bacterial endo carditas 54 Bradycardia is frequently present in hypothyroidism 55 Test the carotid body reflex carefully in all cases of tachy cardia of questionable origin 56 Syncope may arise from a sensitive carotid body Turn ing the head might drop the patient (syncope) as does a tight collar or local pressure 57 Pulmonary stenosis and IVSD cause a harsh murmur along the left sternal border A thrill is very inconstant. 58 To bring out murmurs use exercise Take the blood pres sure and ECG following exercise for better evaluation 59 A large pulmonary artery is seen in (a) Mitral stenosis (d) Essenmengers syn (b) IASD IVSD drome (c) PDA and aortic (e) Multiple pulmon pulmonary fistula ary emboli ·10 Toward The Diagnosis of Congenital Heart Disease (f) Pulmonary fibrosis (h) Emphysema (g) Pulmonary valve (1) "Idiopathic” dilatation of stenosis (post steno the pulmonary artery tic dilation) 60 Rarely does one see rib notching in (C of A) below age of fifteen years 61 The pulmonary second sound is increased in pulmonary hypertension Eg Mitral stenosis Eisenmengers complex aortic pulmonary window or emboli 62 Masqueraders of a farling heart (myocardosis) are (a) Gastric complaints (e) Tiredness (gas) (f) Personality changes (b) Insomnia and (Morose and tyrannical) dreams (8) Anoreva (c) Fatigue (h) Many changes from (d) Ergophobia (fear the normal” of doing usual acts) 63 Increasing venous pressures are seen in the early failing heart Check the venous and arterial pressures Haem orrhoids and bowel haemorrhages may be associated with heart failure pericardıtıs or cirrhosis of the liver 64 Do not mistake cardiac asthma for bronchial asthma as heard in the presence of a bronchial adenoma All that wheezes is not asthma' 65 CARDIOMEGALY is seen also in (a) Anomalous origin of the left coronary artery from the PA (b) Familia! (c) Fibroelastosis (d) Friedreich disease (e) Gargolisin (f) Von Gierkes disease (g) Rhabdomyoma (h) Myocarditis ·Toward The Diagnosis of Congenital Heart Disease 11 66 A systolic murmur at the apex may be the first physical sign of anemia hyperthyroidism or a dysfunctioning sym pathetic nervous mechanism 67 It is often wise to leave well enough alone after three score years and ten 68 Remember that the heart may not be alone or be the primary cause of the patients complaints Examine every system carefully Post operative complications are in direct proportion to the pre operative investigation prep aration selection and diagnosis 69 Collaterals prolong life they are safety valves for a time but help in a diagnosis of observed during the ex amination Varicose veins are frequently due to cardiac obstruction and not from standing 70 About 80% of the troubles in medicine are due to blocked lumina If all the lumina are open there will be few signs and symptoms of disease Eg Bowels ducts ureters valves arteries lymphatics 71 The principle function of the heart is mechanical 72 The heart is made of two pumps lying side by side each serving its own portion of the body If their streams meet other than at the pulmonary or systemic capillanes then there should be clinical evidence of a lesion 73 Supernumary coronary arteries are not infrequent. The coronary arteries and veids may be joined by a fistulous tract 74 Anoxia is a concept of pathologic physiology 75 If the bronchioles are obstructed orygen cannot enter the blood via the alveolar sacs therefore cyanosis occurs keep the bronchi clear Many cases of pulmonary oede ma die because of anoxia from blocked bronchu only Oxygen must have a direct passage to the alveoli to be of use 76 The hyperventilation syndrome causes many vague symp toms and signs until we understand its mechanism and ·12 Touard The Diagnosts Of Congenital Heart Disease biochemistry Look for it in all anxious and nervous patents 77 Anoxia may arise from (a) High altitudes (1) Obstruction of Oz or (b) Emphysema blood flow (c) Heart failure (3) Cigarette smoking (The (d) Shock elastic tissue of the lung (e) Anaemia 15 destroyed ) (f) Haemorrhage (h) Alveolar fibrosis (Rich (g) Oedema syndrome) (h) Cyanide, CO, and (1) A V fistula drugs (m) Respiratory alkalosis 78 CO, narcosis is not present in hyperventilation syndrome but an excess of O, is present There is an excess of CO: in emphysematous lungs Use care with oxygen therapy in the latter 79 Cyanosis from nutrites from a chicken yard from well water nearby reduces haemoglobin in the blood Ask regarding infint water supply in cases of cyanosis in babies who live in the country and use well water 80 Bilateral atresia of the posterior nares may cause anova and sudden death in newboms 81 There must be five grams of reduced haemoglobin per 100 cc of blood in peripheral circulation to produce isible cyanosis 82 Factors controlling cyanosis in the Systemic Circulation are (a) Volume of A V shunt (b) Rate of utılızation of O. (c) Peripheral stasis 83 Factors associated with the Pulmonary Circulation in the presence of cyanosis are (a) Vital capacity (b) Rate of flow through the lungs (c) Partial pressure of O.

·13 Touard The Diagnosis of Congenital Heart Disease (d) Congenital anomalies in the pulmonary circulation (e) Difficulty in return of orygenated blood to systemic circulation (f) Failure of the blood to be orygenated as it passes through the lungs (Eg A V aneurysm ) (g) Concentration of blood (Eg Polycythernia ) (h) Pulmonary disease (Rich syndrome) (1) Inadequate circulation (1) Drugs as morphia 84 Determine the cause of cyanosis in the malformed heart by (a) Size of pulmonary artery (Conus) (b) Oxygen saturation of arterial blood will demonstrate presence of reduced haemoglobin insufficient to cause "visible” cyanosis (c) Effect of inhalation of Oz upon 02 saturation of arterial blood (d) Circulation time 85 Look for clubbing in cardio-pulmonary lesions It is not infrequent in pulmonary malignancy also 86 Look for central and peripheral cyanosis 87 Aetiology of dyspnoea 1 Mechanical Impairment of Ventilation (a) Muscular weakness Eg Poliomyelitis and my asthenia gravis (b) Skeletal fixation Eg Pectus excavatum (c) Hydrothorax pneumothorax pencardial effusion or constrictive pericarditis (d) Diaphragmatic hernia (e) Bronchial or tracheal obstruction 2 Impairment of Pulmonary Distensibility (a) Pulmonary congestion (b) Pulmonary fibrosis (c) Alveolar fibrosis (Rich syndrome ) ·14 Toward The Dragnons Of Congenital Heart Disease 3 Pulmonary Insufficiency (a) Emphysema (b) Inflammation of lung parenchyma (c) Multiple pulmonary emboli 4 Inadequate delivery of O, to tissues (a) Arterial hypoten (c) Cardiac failure sion (b) Anaemia 5 Hyperventilation of Central Type as from Acidosis (a) Nephritis (c) Hyperthyroidism (b) Diabetes (d) Hyperventilation syn drome 88 here are no symptoms in eart disease which are abso lutely typical and can be accepted as evidence of heart disease We must ponder and weigh eich finding in order to come to a logical conclusion 89 A patient may have a severe lesion of the cardiovascular apparatus without symptoms 90 Precordial pain upon exertion is frequently associated with heart disease A check of the coronary arteries is in order 91 Sleeplessness is a common sign in cardiovascular disease and is too often os erlooked Too much sedation is fre quently handed out by the attendant First, find the cause 92 Electrically amplifying stethoscopes are useful and bring out tones better for those whose hearing is defective 93 For a classification of cardiovascular disease, the reader is requested to refer to LUISADA S book on Heart pages 164 165 94 Hoarseness may be present in lesions causing dilatation of the pulmonary artery due to compression of the re current laryngeal nerve 93 Pericardual effusion may cause the patient to rest on his hands and knees or lean over a pillow on his lap ·Toward The Diagnosis Of Congenital Heart Disease 15 96 There are dysfunctions of the heart from metabolic vita min and endocenne disturbances (a) Von Gierkes disease (g) Hyper and hypothyro- (b) Thiamine deficiencies idism (c) Fatty heart (h) Addisons disease and (d) Pellagra Phenochromocytomas (e) Scurvy (1) Thymus gland (My (f) Rickets asthenia gravis ) (1) Hyperparathydroidism 97 Always test for postural hypotension in weak spells Take the blood pressure lying and standing Do not over look petit mal 98 Lest we forget periarteritis nodosa-a biopsy of the mus cle should verify its presence 99 The blood pressure in PDA gives a low systolic and a wide pulse pressure Eg 91/45 and 98/35 after ever cise The average normal pulse pressure is 67 70 mm Hg 100 Pulmonary hypertension from PDA may cause a louder murmur and a larger heart or it may become less marked and disappear in the later stages 101 We may have the following combination PDA IV SD and pulmonary hypertension 102 We may have PDA IVSD and pulmonary fibrosis (arteritis) 103 Always palpate the femorals while taking the blood pressure of the upper limbs One may detect coarcta tion of the aorta more frequently by doing so Always verify the pulse to the area of complaint Eg Caronds in cases of dizziness or syncope 104 Hyperventilation syndrome cases require more (10%) CO, during anaesthesia to prevent apnoea and death There is a severe CO2 lack in the brain 105 All physicians should be capable of doing cardiac mas sage and BEFORE the three minutes allowed are up be ·16 Tovard The Dragnons Of Congenital Heart Disease fore brain damage takes place OXYCIN must be passing into the trachea freely with controlled respirations or by other artificial means 106 Relseve coronary pain by (a) Increase blood supply to ischaemic muscle by sasa dilators or collaterally produced crculabon (opera ton) Eg Becks or Thompsons Hypothermia is of much benefit in the severe cases of thrombosis of the coronary arteries (b) Decrease the metabolites and cholesterol (c) Section pain fibres to the heart (d) Avad nicotine and stress 107 Many patients pror to and following carduc surgery should be seen and treated by a psychiatrist The human fear of heart lesions is tremendous 108 Angina pectoris is diagnostic of coronary disease in the absence of arteriosclerotic aortic disease (stenosis) se vere anaemia, luetic coronary osteal stenosis and pulmon ary hypertension 109 Some manifestations of coronary disease 1 Angina pectons 2 Myocardial infarction herniation and rupture 3 Cardiac asthma or pulmonary oedema 4 Congestive heart disease (fallure) 5 Adams Stokes Syndrome 6 ECG Changes (a) Q ST-T changes or previous infarction (b) A V block (c) RBBB (Right bundle branch block) (d) Low QRS Low and inverted T yave 7 Yrays (a) an unexplained cardiac enlargement (b) Absence or reversal of pulsation (c) Calcified coronanes may be seen ·Toward The Diagnosis of Congenital Heart Disease 17 (d) Angiograms may show blockage in the coronary vessels 8 Digestive symptoms 9 Symptoms made worse by nicotine 110 Extra-cardiac pains may have origin in the heart via the nerve supply through pain points” Eg Loose click ing costal cartilage or bronchial lesions 111 Pulmonary hypertension secondary to cardiovascular (primary) disease may be recognized by (a) Long standing (d) Presence of two-type cough pain (one increased by (b) Persistent or inter deep breaths) Inittent cyanosis (e) Right ventricular en- (c) Associated dyspnoea largement 112 The heart is an independent organ 113 The biochemistry of the bundle of His is related to nerv ous tissue It produces acetylcholine 114 "The heart muscle is one of the unfatiguable muscles the others are bırd wings in long flight" 115 A recumbent position allows for greatest diastolic filling Fowler s position is not always suitable in heart failure cases 116 A large heart frequently gives short contractions and therefore poor propulsive power However in A V block there is a normal cardiac output The propulsive power of each beat must be tremendous and the heart is very large in diastole 117 “The heart is a pump with an intricate mechanism with individual physiology of each left and nght unit." 118 It is dificult to be sure of the exact site of the catheter in catheterizing the heart Errors may be present The catheter report should agree with the clinical findings 119 Pulmonary stenosis may give convulsions from cerebral anonia 120 The importance in diagnosing disease still remains with ·18 Tovard The Diagnosis of Congenital Heart Disease 10ns The history and clinical examinabon of the patient, flu oroscopy x rays and angiocardiograms Catheterization is used in borderline cases where verification is essential 121 Dr Forsman is "Father of Catheterization In Novem ber 1929, he was the first to carry out the procedure, and on himself 122 Speed up the machine (angiocardiograms) in children as their hearts are more rapid We may use the Weber manoeuvre to slow the heart rate in adults Medications may be used also 123 It is best to estimate the blood postassium in mitral les If it is nising, it is more dangerous for operation 124 Differential diagnosis Mitral Stenosis Mitral Insufficiency (1) Early dvspnoea (1) Earl fatigue, late late fatigue dyspnoea. (2) RV is enlarged in (2) LV enlarged presence of pulmon (3) Systolic murmur ary hypertension (4) Normal PA (3) Diastolic murmur (5) Pulmonary congestion (6) Less calcium in the (4) Large P.A valve (5) Pulmonary conges (7) L A less enlarged tron (6) Calcium deposits in the valve (7) LA enlarged 125 Balstocardiograms might have a place in the diagnostic setup in the future of heart disease 126 Be on the lookout for cardiac neurosus but do not make a patient a neurotic through a wrong diagnosis or mus placed treatment 127 Aitroglycerne relieves the pain of coronary disease but does not cure the patient Certain operative procedures are found to gne relief of symptoms due to formation at apex ·19 Touard The Diagnosis of Congenital Heart Disease of new collaterals to the ischaemic sites 128 Diabetes nicotine and coronary disease join hands fre quently and pass out together 129 Pulmonary embolus think of it for it is common! Aet ology Phlebitis somewhere (post op ) Evidenced by (a) Acute pleuritic pain (b) Cough and rales at the site of lodgement (c) Low grade fever (d) Haemoptosis and dyspnoea (e) Vray shows triangular and dome lung shadow more often at the bases 130 Lest we forgetl Obstruction of the SVC causes a def inite syndrome Venous pressures and venograms are of value here If both SVC and IVC are obstructed, think of constrictive pericarditis 131 There are cardiac tumors cysts and aneurysms which simulate other cardiac lesions Is it actually the heart at fault? 132 A dissecting aneurysm is too frequently forgotten in an early diagnosis 133 Generally we die from cardiovascular disease Cancer is now fourth on the list of causes of death 134 Regional hypertension is not only seen in coarctation of the aorta but arterial thrombosis as well Eg Aortic arch syndrome compression of the aorta or arteries by tumors horseshoe kidney tumors of arteries themselves and fibrosing lesions of the arteries 135 Consider the heart as two separate units-one supplying the lungs the other supplying the body generally The two units unite only through the alveoli and peripheral arterio Venuoles If other connections are present then an abnormality exists 136 The cardiac silhouette is frequently altered by hyper ventilation partial or complete bronchial obstruction and by cyst of the lung or congenital heart disease ·CLINICAL DIAGNOSIS OF HEART DISEASE IN CHILDREN (Congemtal and Acquired) (Patterson) "EYES FIRST AND MUCH A CYANOSIS Causes (1) Methaemoglobinemia (2) Cerebral (3) Pulmonary Decreased on crying, responds to O. (4) Cardiac Increased on crying indicates right to left shunt Does not respond to Os (5) Arteriovenous mixing of blood Eg Pul AV fistula B DYSPNOEA (1) Is the breathing more rapid than normal? (2) If it always breathes faster" without stridor or cyanosis, then it is apt to be left heart fail vre with pulmonary congestion or pulmonary stenosis with decreased pulmonary blood flow Check the red blood count haematocrit and look for diabetes salicylates poisons or fever C CARDIAC ENLARGEMENT (1) If more than 1 cm beyond mid clavicular line it indicates enlargement Check the ECG, position of the diaphragm and expiration for atelectasis pectus excavatum pneumothorax pulmonary hypertension pericardial effusion or agenesis of a lobe or lung 20 ·Touard The Diagnosis of Congenital Heart Disease 21 (2) If the palpating finger is Lifted at the apex it is apt to be left ventricular hypertrophy If the finger is lifted” over the pulmonary artery area it is apt to be pulmonary hyper tension (Right ventricular hypertension ) (3) Anaemia malnutrition myocarditis hyper thyroid endocardiofibroelastosis glycogen storage disease coarctation or medial arteriosclerosis are other causes of enlargement D TACHYCARDIA (1) If the pulse is above 110 per minute when asleep it suggests myocardial disease or hyper thyroidisti E BLOOD PRESSURE Always check for (using both arms and legs in CA suspect) (1) Coarctation of the aorta (CA) (2) Phenochromocytoma (3) Wide pulse pressure is present in PDA and aortic window (4) A narrow pulse pressure is present in aortic stenosis F RIGHT HEART FAILURE Note (1) Oedema (3) Neck veins filling (2) Hepatomegaly G MURMURS Note (1) Character (4) Position (2) Timing (5) Transmission (3) Quality (6) Thrull In children give sedation (1/10 gr barbiturate/Ib body weight rectally) H CATHUTERIZATION ANGIOCARDIOGRAMS RAYS AND PHONOCARDIOGRAMS These are other means of diagnosing congenital heart lesions ISABE Note (1) Blood cultures (2) Emboli in skin

·22
Touard The Diagnosis of Congenital Heart Disease
I COLLACEN DISEASES
(1) Lupus erythematosus
(2) Polyarteritis nodosa
(3) Rheumatoid arthritis
(4) Dermatomyositis
(5) Disseminated scleroderma
K ANAEMLAS
Check the blood
L MYASTTIENIA GRAVIS
Chech by giving prostigmine
M AVITAMINOSIS
Check the diet

·FOETAL ANOXIA AND CYANOSIS IN THE NEWBORN Anoxia in the new born is either from poor orygenation or from a circulatory shunt which prevents oxygenated blood from reaching the tissues INTRAUTERINE ASPHYXIA The foetal organ of respiration is the placenta It depends upon (a) Adequate supply of oxygen in the maternal blood (b) Intact placental structure and circulation (c) Intact pathways for blood from the placenta to the foetus These interfere with foetal orygenation (a) Maternal disease (b) Placental disease (c) Compression of cord (d) Atelectasis (e) Functional depression of respiratory centre from mother s drugs (f) Endothelial fibroelastosis Respiratory distress (Cyanosis) in the newborn (other than congenital heart lesions) may be caused from (1) Atelectasis (6) Aortic rings Vasa (2) Hyalıne membrane lusoria disease (7) Tracheo-oesophageal (3) Alveolar dysplasia fistula (4) Tension pneumothorax (8) Diaphragmatic hernua or cysts (9) Bilateral atresia of the (5) Congenital cysts of the posterior nares (10) Microglossia lung 23 ·24 Toward The Dragnons Of Congenital Heart Disease (11) Tetany (12) Myasthenia gravis (13) Intracranial hemorrhage (14) Agenesis of lobe or lung (15) Severe pectus excava tum "Anorid not only stops the machine but wrecks the machinery HALDANE

·THE CONGENITAL HEART DISEASES
A CLASSIFICATION
(Paul Wood)
ACYANOTIC
A No SHUNT
1 Simple
(a) Dextrocardia
(b) Congenital hypertrophy
(c) Heart block
2 Left Hypertrophy
(a) Coarctation of the aorta (CA)
(b) Congenital aortic stenosis
(1) Subvalvular (1) Valvular
(c) Bicuspid or quadricuspid valves
(d) Bicuspid plus aortic incompetence
3 Right Hypertrophy
(a) Pure pulmonary stenosis
(1) Valvular (u) Infundibular
(b) Muld pulmonary sternosis with VSD or A.SD or
PF Ovale
(c) Pulmonary valve incompetence from congested
dilated PA (Pulmonary plethora)
B A SHUNT (Left to right)
1 IVSD
3 PDA
2 IASD 4 Aortic pulmonary Fostula (Window)
CYANOTIC
A RICIIT TO LEFT SHUNT (Pulmonary Oligema)
(a) Cyanosis from birth
25

·26
Touard The Diagnosis of Congenital Heart Disease
1 Right Hypertrophy
(a) Tetralog of Fallot
(1) Infundibular stenosis (u) Valvular stenosis
(b) Marked PV stenosis with IVSD or IASD or
PF Ovale (Triology of Fallot)
(c) Persistent truncus
2 Left Hypertrophy
(a) Pulmonary valve or artery atresia with septal de
fects
(b) Tricuspid atresia with septal defects ("a and 5"
have non functioning right ventricle)
3 Late Cyanosis
(a) Eisenmenger Syndrome
(b) Moderate PS with septal defects
B LEFT TO RICHT SHUNT
1 Transposition of Great Vessels
ANATOMICAL DIAGNOSIS IN HEART DISEASE
WE MUST NOTE
(1) The pulse
(7) Heart sounds
(2) Neck veins (deep jugulars) (8) Heart murmurs
(3) Cyanosis
(9) Fluoroscopy and X rays
(4) The apex beat
(10) Cardioangiograms
(5) Liver and oedema (11) Catheterization
(6) Palpation of precordium (12) Arterial and enous
pressures

·HEART (Maurice Campbell) CONGENITAL MALFORMATIONS NON CYANOTIC CYANOTIC PULMONARY BLOOD FLOW C INADEQUATE 1 Pure pulmon A EXCESSIVE 1 PDA 2 IASD 3 IVSD 4 Anomalous pul monary veins 5 Aortic pulmonary window 6 Triology of Fallot? B NORMAL 1 Coarctation 2 Vascular ring 3 Aortic stenosis 4 Aberrant coronary ary stenosis PULMONARY BLOOD FLOW EXCESSIVE 1 Transposition of great vessels 2 Eisenmenger complex (Late) INADEQUATE 1 Pulmonary stenosis and I ASD (Triology of Fallot) 2 Tetralogy of Fallot 3 Tricuspid Atresia 4 Ebsteins deformity TRUE TRUNCUS ARTERIOSUS- -PSEUDO PULMONARY ART FROM SINGLE-PULMONARY ART FROM MAIN CHAMBER VENTRICLE RUDIMENTARY CHAMBER 27

·PATENT DUCTUS ARTERIOSUS (PDA)
(14% of all lesions, 1 5500 births)
1 Ser incidence 5 1 females
2 Growth stunted ( 45%-75% blood loss to lungs)
3 Fatigue and dyspnoea are dependent on the size of the
passage
4 Pulmonary congestion so called "chest colds
5 Epistans
6 No cyanosis or clubbing (until failure)
7 Corrigan pulse at the wrist
8 A machinery murmur" is found at the 2nd left ICS
systolic and diastolic in time Early there may be a
systolic murmur
9 A murmur at the 2nd left intercostal space (ICS) trans
mitted to axilla and interscapular area A med diastolic
murmur may be heard at the apex
10 A "machinery murmur" occurs when the systemic diastolic
pressure becomes greater than the diastolic pressure in
the pulmonary circulation
11 The continuous murmur may be lacking and only a systolic
is heard over the pulmonic area (in infants and children)
12 A thall is found in the 2nd and 3rd intercostal space in
75% of cases
13 The 2nd heart sound is sput
14 A wide pulse pressure A low diastolic
15 Cardiac enlargement and failure of the myocardium may
occur later
16 Hilar dance (Pulmonary Plethora) is rarely noted in
fluoroscopy
28

·Touard The Diagnosis of Congenital Heart Disease 29 17 The pulmonary conus is enlarged in x ray 18 Prominent aortic arch near the PDA which pulsates markedly 19 A high P A pressure 20 Increased O, blood in the pulmonary artery 21 Aphonia or hoarseness is present of PA presses on the recurrent laryngeal nerve 22 Severe dyspnoea and palpitation when failure occurs 23 ECG Left axis deviation The ECG may be normal in 83% of cases DIFFERENTIAL, DIAGNOSIS (Continuous murmur) Seen in (1) Venous hum (diastolic accentuation) (2) Severe anaemia (usually systolic murmur) (3) Pulmonary artery pressure up (4) A V fistula of lung (5) Rheumatı heart disease (6) IASD (systolic murmur) (7) Rupture of sinus valsalva into right ventricle (8) Compression of arteries Eg Cervical (9) Aortic pulmonary window (10) Coronary artery fistula May have reverse ductus flow-cyanosis GENERALLY "Any loud systolic murmur (without diastolic element) in acyanotic cases arises from a septal defect or pure pulmonary stenosis IN ECG If PR interval is prolonged it is apt to be an ASD as con duction is longer One may have a partial night BBB” (95%) In the early months the pressures between the aorta and the pulmonary artery are frequently equal Therefore there is no murmur sometimes One may have a systolic murmur only ·30 Toward The Diagnosis of Congenital Heart Disease DIFFERENTIAL DIAGNOSIS (DD) OF PDA AS TO DECREE 1 Small PDA (a) The heart is normal in size (b) The cardiac umpulse is not forceful (c) The x ray findings are minimal (d) The diastolic pressure is down slightly 2 Average PDA (a) The heart is enlarged The left border in the conus area is the chief site of enlargement (b) An intense murmur and thrill are present (c) The diastolic pressure is down (d) Pulmonary plethora (chest cold) is frequent 3 Tremendous PDA (a) The heart is large Early failure is noted (b) The heaving heart beat may even shake the body of the child (c) Forceful neck pulsations are present (d) Marked lung changes are due to excess blood flow and pressure (e) A great depression of the diastolic measurement pres ent (f) Additional murmurs may be heard CLASSIFICATION OF PDA BASED ON AUSCULATORY FINDINGS 1 Cases with typical continuous murmurs (a) Without diastolic at the apex (906) (b) With diastolic at the aper 2 Cases with an atypical murmur at the pulmonary area (a) Without diastolic murmur at the aper (1) No cyanosis (u) Gyanosis with reverse flow (b) With diastolic murmurs at the aper 3 Cases without murmurs at the pulmonary area

·Touard The Diagnosis of Congenital Heart Disease
31
Disease is from old and nothing about us has been
changed It w ue who change as we learn to recog
nize what has formerly imperceptible for we seek
only what we look for ue recognize only what we
know
CHARCOT

·COARCTATION OF AORTA (CA.) (8%) 1 16 000, Sales 51 A ADULT (Majority) (Post 117 Hypotension in the lower ductal) limbs or no pressure Ductus open or closed 18 Decreased abdominal B INFANTILE (Preductal) pulse or it is absent Ductus open or closed 9 Palpable vessels in the 1 Palpitation scapular areas 2 Throbbing in head 20 Collaterals are increased 3 Breathlessness 21 The upper body is well 4 Early Fatigue developed 5 Nervousness (tension) Cyanosis is not present 6 Headache except in preductal type 7 Dizziness of CA and seen in the 8 Epistaxis legs 9 Blurring of the vision 23 Leg ulcers may be pres 10 Precordial pain ent 11 Leg cramps (Do not 24 Systolic murmur (inter mistake these for Leriches scupular) is heard along disease) the left posterior chest 1 Cold feet Warm hands 25 Hypertrophy of the left 13 Tinnitus ventricle 14 Gnawing shoulder pains 26 The ascending aorta is and throbbing caroud dilated arteries 27 Absent aortic knob (Fig 15 Chest and back pain is ure "3") is noted at the due to arterial pressure knob site 36 Hypertension in the upper BS Rib notching (Rart be Lumbs fore 15 yerrs ) 32 ·Toward The Diagnosis of Congenital Heart Disease 33 29 Left axis deviation 30 Cardiac failure may occur early Aper out and down to left 31 PDA is associated with CA (In 5% of cases) 32 Bicuspid aortic valves (42%) are present with infantile CA 33 A berry aneurysm may be present in the circle of Willis This may rupture and cause death 34 Rupture of cerebral arteries show 50% of deaths 35 Normally BP is higher in legs than in arms 36 There may be a double stenosis 37 Associated with C.A there may be (a) Bicuspid valve (9%) (d) Mitral stenosis (5%) (b) Aortic stenosis (10%) (e) Intermittent claudication (c) PDA (5%) (8%) bug The optimal age for operation is the second decade (8 12 years) Earlier if severe 39 In 22% of cases there is aortic rupture from coarctation 40 A left SVC with coarctation and superficial collaterals have been noted on occasion 41 There may be post stenotic dilatation in C of A 42 Coarctation may occur at any site Check the BP at vanous levels as well as intra arterial pressures and aortograms 43 Murmurs and thrills in unusual places may be caused by CA 44 Eighty per cent die before 35 years of age 45 The majority are males 46 High blood pressure in the right arm and low pressure in the left arm is caused by (a) Atresia of the left subclavian artery (b) Stenosis of the left subclavian artery (c) Coarctation provimal to the left subclavian causing pressure in the S artery

·34
Toward The Diagnosis of Congenital Heart Disease
47 High blood pressure of the left arm and low blood
pressure
of the right arm may be from
(a) Stenosis nght of the subclavian artery
(b) Ongin of the right subclavian as a 4th branch of the
aorta at a point beyond the coarctation
48 A hypoplastic aortic arch knob is noted in the furoscopy
or in the r ray
49 A figure “3 is noted at the CA site
50 An altered contour of the esophagus has been noted
51 A prominent left border of the superior mecastinum is
due to a dilated subclavan artery
52 Angiograms when in doubt, should be done
53 The cardiac apex is rounded
54 The internal mamary and subclavian arteries are dilated
and may be seen in the lateral film
"We should not count our uords but make our
thords count

·AORTIC ARCH VASCULAR RINGS
(AAVR 1 250 000)
There are many vascular malformations in the superior media
stinum Many do not cause clinical symptoms
Among those anomalies which cause symptoms and signs
are the following
1 Double aortic arch
2 Right aortic arch with a left ligamentum arteriosum
3 Anomalous innominate artery
4 Anomalous left carotid artery
5 Aberrant right subclavian artery
Double Aortic Arch Features
1 The trachea and oesophagus are constricted There is
stridor and dysphagia
2 Increased respiratory rate is noted The baby struggles for
breath and regurgitates its food The symptoms are aggra
vated by Auds
3 There may be intercostal and subcostal indrawing
4 A loud wheeze can be heard with the stethoscope
5 Cyanosis is frequent in more constricted cases
6 The infant lies with the head hyperextended
7 The x ray tracheogram bronchoscopy and/or angiogratis
will visualize the lesion present
8 A blowing systolic murmur is present in 20% A gallup
rhythm is usually present
Right Aortic Arch with a Left Ligamentum Arteriosum
Here the ligamentum crosses on the posterior aspect of the
oesophagus and trachea and therefore constricts these
35

·36
Toward The Diagnosis of Congenital Heart Disease
structures Signs and symptoms are similar to those of
a double aortic arch except for the position of the arch
Anomalous Innominate Artery
Here the artery leaves the aortic arch too far to the left
and in order to reach the right cervical region must pass
anterior to the trachea causing constriction of this struc
ture with consequent signs and symptoms
Anomalous Loft Common Carotid Artery
Here the artery arises more to the right on the aortic arch
and in order to reach the left cervical area must constrict
the trachea The signs and symptoms are those of the
innominate anomaly
Aberrant Right Subclavian Artery
The artery takes off independently from the aortic arch
instead of off the innominate artery
It ascends and
passes to the right to reach the apex of the chest It
passes posterior to the oesophagus and so to the rght
arm
The heart ll the tinfatigable muscle"

·SUBAORTIC STENOSIS (SAS)
1 24 000
CLINICAL FEATURES
1 History of "heart disease" from birth
2 No history of rheumatic fever
3 A loud mid systolic murmur (80% of cases)
It is an
ejection type of murmur It is more commonly lower and
to the left than at the aortic area
4 Moderate left hypertrophy
5 Normal blood pressure
6 Like those found from rheumatic fever causing aortic
stenosis
7 Pallor and dyspnoea
8 Apical impulse may be displaced down and outwards
9 Pulse pressure may be down
10 A thrill may be felt passing to the neck Chiefly in the
2nd right intercostal space (ICS)
11 Pulsation of a dilated sinus of Valsaya may be seen in a
RO A view on screening
37

·ATRESIA OF AORTIC ORIFICE
(Functional two-chambered heart)
1 39,000
(Julius Tandler 1869 1936)
FINDINGS
1 PDA and PF ovale are present
2 The night atria the night ventricle and the pulmonary
artery are enlarged
3 The left ventricle is thich walled and a small chamber is
noted
38

·RIGHT AORTIC SINUS ANEURYSM -VALSALVA- A CONCENITAL (44 cases reported) B ACQUIRED Associated with coarctation SABE syphilis and aortic re gurgitation 1 Tend to rupture into the right heart 2 Severe overloading of the nght cardiac chambers when the rupture occurs 3 Sudden dyspnoea 4 A humming murmur 39

·AORTIC-PULMONARY WINDOW
(FISTULA)
(Aortic septal defect)
1 It is an incomplete division of the truncas arteriosus
2 Ofted many features of PDA are noted
3 It is associated with Essenmengers, of the opening is less
than 75 cm (Acts like a PDA but the murmur is lower)
4 A loud systolic murmur is present over the precordium
(Max in 3rd and 4th ICS left of the sternum
5 Pulmonary hypertension and PA aneurysm are complica
tions
6 Pulmonary plethora (chest cold) is frequent
7 Progressive cardiac enlargement is noted
8 There is absence of cyanosis and polycythemia
9 Cardioangiograms may show dilution of dye in PA and
an indentation of dye from aortic cross flow An aorto
gram will show flow to PA
10 Treatment is by closure, if no irreversible damage to the
PA system
"In surgery ue should do as little as possible but
as much as is necessary and herein lies good
fudgement"
40

·TETRALOGY OF FALLOT (T of F)
1 8,500
THE TETRALOGY CONSISTS OF
1 Aorta drains ventricles
2 Right ventricle enlarged
3 Pulmonary valve stenosis and infundibular stenosis in 99%
4 IVSD
FEATURES
1 Marked cyanosis variable May require exercise to show at
2 Dyspnoea
3 Dyspnoea increased on feeding Paroxysmal in character
may be severe
4 Unconscious spells may cause convulsions from anova or
thrombosis of the cerebral vessels may occur causing paral
ysis
5 Limited activity + +
6 Retarded growth + or
7 Clubbing of fingers + ++
8 A precordial bulge Right ventricle is enlarged
9 Turner s syndrome (gonads) associated (20%)
10 "Squatting" when tired Physical capacity greatly impaired
11 Deciduous dentition
12 A small peripheral pulse
13 A systolic murmur Thril (50%) If high 1
apt to be valvular type At 2 - 3rd left space (10% have no
murmurs)
14 Red blood count 6.7 million + + +
15 Haematocrit 60% + or
18 Pale lung fields (Oligemia)
2 it is

·42 Touard The Diagnosis of Congenital Heart Disease Raised apex 17 Sabot shaped heart-or + 18 Concaved pulmonary conus area 19 Pulmonary window In LA oblique x ray A small left heart is noted 20 ECG deep s' Leadı High R" in lead su 21 Single 2nd sound at base 22 Dextroposition aorta (20%) 23 Low arterial O. content 24 A heavy heart action is noted following exertion 25 The pulmonary artery is small 26 Post stenotic dilatation may be seen in the pulmonary artery, in the r ray and especially at operation OLIGAEMIC" =Light Lung Field (V to A) PLEON LEVIC -Dense Lung Field (A to V) INFUNDMULAN STENOSIS IS frequently present with T of F CATFIETERIZATIOV (Catheter) Features in T of F 1 O. unsaturation arterial blood 2 High pressure in the right ventricle, low in the PA The pressures in the aorta, right ventricle and brachial are increased 4 Right to left shunt Shows site and presence of pulmonary stenosis 5 Decreased pulmonary flow RECORDS (pressures) with a catheter in the pulmonary trunk (withdraw ) Note sequence (a) A sharp rise in the systolic to the right 1 entricle The systolic pressure as catheter passes through the vale indicates valvular stenosis (b) No change in the systolic pressure with a fall in duastolic pressure when the catheter passes via the valve fol lowed by a rise in the systolic to the ventricular level

·Toward The Diagnosis of Congenital Heart Disease
43
at the infundibular region indicates infundibular
stenosis
(c) A combined type indicates both lesions
"We should do the greatest amount of good and
the least amount of harm we should avoid latrogenic
lesions at all cost

·PULMONARY STENOSIS WITH NORMAL AORTIC ROOT 15% Infundibular, 90% Valvular I The systolic pressure is the same in PA and RV If former lower than latter then stenosis exists A gradient of 10 mm is normal All degrees are found 2 In valvular stenosis, the cusps are fused with a small circular hole present 3 Infundibular stenosis is circumscribed and is situated on the borderline between the main part of the right ventricle and the infundibulum The latter forms a cavity or third ventricle” 4 The whole infundibulum may be stenosed and forms a nar row channel which extrudes as far as the pulmonary valve 5 PS is often associated with other anomalies-chiefly IASD and I VSD 6 A large left to right shunt dilates the pulmonary artery 7 IVSD is not uncommonly combined with stenosis of the infundibulum 8 The stenosis may be so slight that the left to right shunt is still present through the defect 9 In severe stenosis a right to left shunt occurs and is more difficult to diagnose from T of F (An overriding aorta is present here) 10 In I ASD the direction of the shunt is determined partly by the resistance of the ventricle during diastole In an uncomplicated case the resistance is lower in the right ventricle than in the left ventricle Therefore the shunt is left to right With hypertrophy of the night ventricle 44 ·Toward The Diagnosis of Congenital Heart Duease 45 there is increased resistance of the nght ventricle and the shunt is right to left 11 In I ASD, if the stenosis is severe there is a night to left shunt 12 In a PFO with complete functional closure the shunt can take place in one direction only ie Right to left and may cause cyanosis (T of F) 13 Types of PS with a normal aortic root (a) Intact V and A septa (b) With IVSD and a normal A septum (1) With left to right shunt (1) With night to left shunt (c) With PFO and intact V septum (1) Without shunt (u) With right to left shunt (d) With I ASD and intact ventricular septum (Triology of Fallot) (1) With left to right shunt (u) With nght to left shunt All the above may be confirmed 14 Pulmonary stenosis hemodynamically is characterized by the inability to mamtain an adequate cardiac output and thus the right heart takes the burden Pressure increases in the right ventricle and the right auricle The more the stenosis the less the output-even at rest This is com pensated by an increase in the arteriovenous oxygen differ ence and slight penpheral cyanosis may appear 15 In septal defects with a right to left shunt the pulmon ary blood flow is still decreased further and there is arterial Oxygen desaturation 16 Severe right ventricular hypertrophy may result in a sufficient nse in pressure in the right auncle to cause a nght to left shunt 17 Marked cyanosis in IASD and PS comes later in life 18 Clinical features of PS (primary) (a) Loud systolic murmur over P A area ·46 Toward The Diagnosis of Congenital Heart Disease (b) Increased fatiguability (c) Dyspnoea (d) Slight cyanosis on exertion (e) Syncope on occasion Physical examination reveals the following (a) The child may be normally developed (b) Slight cyanosis is present An atrial wave is seen in the neck veins (c) Very occasionally the child squats-not nearly as com mon as in T of F (d) Right ventricular hypertrophy (e) Apex beat is displaced BACKWARDS and is not palpable (f) The enlarged nght ventricle strikes the chest at each heart beat causing a parasternal or substernal Ift Sounds and Murmurs in Primary PS 1 A systolic murmur over the 2nd left ICS 2 It occurs 05 and 06 after the first sound in the phonocardio gram 3 Pul second shows a decreased amplitude There is an in creased split of the second heart sound heard in mild and moderate cases 4 In severe ps the 2nd heart sound over the pulmonary area cannot be detected (due to aortic sound) 5 In infundibular stenosis the degree of splitting of the second sound is the same as in valvular stenosis The murmur is between the 3rd and 4th ICS The pulmonary component is audible as a faint extra sound after the end of the murmur Many believe that infundibular stenosis is secondary hyper trophy of the area due to PS and not pnmary This hon ever does not include the isolated" cases of infundibular stenosis I ray Findings in PS 1 The main trunk of the pulmonary artery is dilated 2 The penpheral vessels of the lungs are narrow ·47 Toward The Diagnosis of Congenital Heart Disease 3 The right ventricle is hypertrophied and rotated to the left 4 The R A may be enlarged with a prominent appendage 5 Post stenotic dilatation is a classic finding 6 The central vessels of the hilus are dılated the peripheral narrow 7 The apex of the heart is NOT tumed up in PS as it is in T of F Angiocardiography Inject locally (Stockholm technique) Best inject into the RV direct via catheter A conical or dome shaped membrane may be noted SIMPLE PULMONARY STENOSIS WITH A RIGHT TO LEFT INTERATRIAL SHUNT 1 These at first are acyanotic until teens or “twentys when the right side pressure rises giving right to left shunt Here valvotomy is correct 2 If no nght to left shunt then right ventricle failure occurs earlier T or F DIFFERENTIAL, DIAGNOSIS AND X RAY AXIOMS 1 Those with a large aortic knob and marked pulmonary con cavity (10%) are generally pulmonary atresia 2 Normal heart small pulmonary 'bay” (30%) 3 Those with infundibular stenosis (30%) 4 Those with right aortic arch and no concavity (30%) 5 Do right ant oblique to show conus area INCREASED VASCULARITY OF THE LUNGS IN T OF F PRESENT IN 1 Bronchial artery hypertrophy 2 Unilateral pulmonary artery atresia (A unilateral plethora is noted ) 3 A large left to nght ventricular shunt without cyanosis (1 40)

·48
I oward The Diagnosis of Congenital Heart Disease
DIFFERENTIAL DIAGNOSIS OF TETRALOGY OF FALLOY
1 Aortic atresia (Note heart enlargement and vascularity)
2 Transposition of great vessels (enlargement and vascul
anty
3 Single ventricle with pulmonary stenosis (Use Diodrast)
4 Trcuspid atresia (Note ECG)
5 Pure pulmonary stenosis (See ante)
8 Severe PS with PF ovale (Large heart large PA)
7 Eisenmenger (Less cyanosis, better tolerance large P
A)
8 Persistent truncus
9 Pulmonary artery atresia
10 Marfan's syndrome
11 Pulmonary arteriovenous fistulae
SQUATTING MAY BE SEEN IN
1 Tetralogy of Fallot (Others have different cardiac con
tour)
2 Truncus and pseudotruncus arteriosus
3 Certain cases of tricuspid atresia
4 Single ventricle
INFUNDIDULAR STENOSIS TYPES 3RD VENTRICLE"
1 Band
3 Intermediate
2 Subvalvular
4 Tubular
"The correct diagnose the pearl in medical
practice Let us not be satisfied without #*

·PURE PULMONARY
VALVULAR STENOSIS
(PPS) (No other defects)
1 14000
1 Pulmonary arthropathy + of fossa ovale remains open
2 Rough systolic thrill in upper part of the chest left side
Long of severe
3 Enlarged nght ventricle
4 Murmur is systolic Harsh 2nd left, near sternum
5 Cyanosis late and penpheral and with patent F ovale
on occasion
6 Globular heart enlargement
7 Increased shadow of main pulmonary artery
8 Progressive breathlessness on exertion
9 Anginal pains and syncopal attacks in severe cases
10 Conspicuous "a" wave in the venous pulse
11 Pulmonary oligeria Small peripheral pulmonary vessels
seen in x ray
12 Post stenotic dilatation of P.A
13 Hypoplasia of aorta and left ventricle
14 ECG right ans deviation Sharp "P" wave
15 Pressure gradient of 10 15 mm Hg considered evidence
of stenosis
16 If systolic pressure is below 70 mm Hg (RV) apt to be
no symptoms If above 70 mm Hg then symptoms are
present
17 Right ventricle "strikes" wall of chest ("A tap")
18 A precordial bulge is present
19 A negative "T" wave in VV, indicates severe stenosis
49

·50
Toward The Diagnosis of Congenital Heart Disease
"We see only what ue look for We recognize only
that which we know *

·PULMONARY ATRESLA
FEATURES
1 All blood flow to lungs is via the collaterals and bronchiolar
arteres
2 Individual pulmonary shadows are small
3 "In a cyanotic like Fallot plus a continuous murmur (like
PDA) most likely atresia of the pulmonary artery"
4 A loud single second sound (aortic) is present to the left
of the pulmonary area
5 Unusual concavity of PA area
6 Small isolated shadows in the upper right mediastinal area
and about hila of lungs
7 May have T of F with unilateral pulmonary atresia
51

·PULMONARY STENOSIS WITH
TRICUSPID ATRESIA AND
UNDERDEVELOPED RIGHT VENTRICLE
FEATURES
1 Increasing cyanosis from birth
2 A systolic murmur is present over the pulmonc area
3 Anomal sized heart
4 Relative left axis deviation
5 Left ventricular enlargement
6 Right ventricle is small
52

·EBSTEIN'S ANOMALY OF
TRICUSPID VALVE
1 210 000 of live births
FEATURES
A great deformity of the valves (except antenor) is
present and these are placed downward into the ventricle
1 The atrium is greatly dılated causing an enormous cham
ber
2 No signs of regurgitation
3 The patient is distinctly disabled A poor cardiac out put
is noted
4 IIe is flushed or red faced with cyanosis of the lips
5 Dyspnoea or fatigue is noted
6 Cyanosis upon exertion is present
7 Syncope is noted in a few Headaches are present in a
number A few may have seizures of petit mal type
8 Paroxysms of tachycardia are present
9 There is a systolic murmur and thrill (diffuse) Diastolic
murmur is present in a few cases If tricuspid insufficiency
is present a scratchy sound in early diastole is frequent
(70%) A large quiet heart on palpation is characteristic
of TI
10 Cardiomegaly is characteristic with Ebstein s anomaly
11 A split first sound is noted
12 Some have clubbing
(Refer to Kjellbergs book)
53

·TYPES OF PULMONARY
VENOUS CONNECTION
I PARTIAL
A Anomalous connection only to the left atrium
(a) With normal lobular venous pattern
(b) With abnormal lobular venous pattern
B Connection both of the right atrium or tributary veins
and to the left atrium
(a) With stenotic left atrial connection
(b) With normal left atrial connection
(c) With normal left atrial connection, but with mitral
or aortic atresia
C Connections only to the right atrium or tributary veins
(a) Without major intracardiac defects
(b) With major intracardiac defects
D Connection between a pulmonary artery and the left
atrium
E TO SVC or IVC
F There is a very close association of ASD secundum
with pulmonary venous anomabes
II TOTAL
Note
(1) Tigure 8 shape in x rays
(2) Engorged pulmonary vessels
(3) In the lateral view the anterior mediastinum is re
muhably clear
(4) Marked activity of the heart and the pulmonary vessels
in fluoroscopy There may be central cyanosis
(5) Angiograms show fillmg defect in SVC
54

·Toward The Diagnosis of Congenital Heart Disease
55
MAY HAVE
(1) Perşısting left SVC to the coronary sinus night
atrum nght SVC or to the portal vein
(2) The pulmonary veins may pass into the nght atrium or
the coronary sinus
(3) Total right P V drainage into IVC
(4) Many combinations have been found and should be
looked for
CLINICAL FEATURES OF ANOMALOUS PULMONARY VEINS
Malformation may cause early death
Venous and arterial blood are mixed in the heart
If no communication between the atria death occurs early
(1) A precordial bulge is present
(2) A parasternal 'lift" on palpation of the area is readily
noted
(3) A systolic murmur is present over the pulmonic area
(4) A wide mediastinum
(5) The right ventricle is hypertrophied
(6) Incomplete B B block is present
(7) A left to right shunt
(8) Marked lung vascularity
*Adventure on for from the littlest clue
Have come whatever man ever knew

·THE EISENMENGER COMPLEX
(EC) (8%)
(Victor Eisenmenger 1861 1932)
(Kindly refer to Dr Paul Wood's paper on the Complex and
the Syndrome Brit MJ May 1958)
CONSISTS OF TEV DIFFERENT ANOVALIES
FEATUHES
I Haemoptosis is prone to occur
2 The heart may be enlarged
3 A systolic murmur and thuill (harsh) are present at the
base and are transmitted through the arterial tree
4 Aortic insufficiency may be a common finding The pul
monary artery flow may be diminished
5 Arrythemias +
6 The second pulmonary sound is accentuated
7 The pulmonary blood low is not dinished The pul
monary artery pressure is raised
8 Right to left shunt
9 Cyanosis later than T of F May be absent in infant,
fater persistent
10 A normal pulmonary valve The PA is dulated and a
palpable impulse is present
11 There is sught or no clubbing There is no squatting as
in T of F
12 Hoarseness and brassy cough +
13 Sabat shaped heart when the PA is large We must
DD from Lutembacher The pulmonary arc is conver
56

·Toward The Diagnosis Oj Congenital Heart Disease 57 and prominent in Eisenmengers complex Hilar pulsations are to be noted 14 ECG or + Right axis deviation and a large biphasic QRS complex is noted 15 The arterial blood O is important Using 70% diodrast shows dye in the aorta as soon as it enters the right vent ncle (because of the overriding) 16 The aorta is dextroposed 17 A high ventricular defect 18 Abnormal aortic valve (frequent) 19 A thick right ventricular wall 20 Coronary insufficiency or + DIFFERENTIAL DIAGNOSIS FROM EISENMENGERS (in absence of cyanosis) The following lesions should be considered 1 Malade de Roger and high VSD (The murmur is not transmitted ) 2 Aortic and subaortic stenosis (normal pulse pressure) 3 Lutenbacher (Get rheumatic fever history-mutral) 4 RH diseases (rheumatic heart) 5 T of F 6 Ayerza s disease and pulmonary hypertension 7 Pulmonary alveolar fibrosis (Rich syndrome) WE MAY SEE EVIDENCE OF THE EISENMENGERS SYNDROME IN 1 PDA 5 Single ventricle 2 AP window 6 Ostium primum 3 IVSD 7 Transposition and IVSD 4 IASD and total anoma 8 Sungle atrium lous pul V drainage 9 ASD The cause of the main pulmonary dilatation is more likely to be due to the high pressure rather than to the flow between the two circulations One gets endarteritis fibrosa in the lungs and thrombo obstruc tive lesions-right to left shunt and cyanosis later

·58
Touard The Diagnos Of Congenital Heart Disease
"I hold that man į in the night who is most
closely in league with the future
N

·TRANSPOSITION OF THE
GREAT VESSELS (TGV)
(karl Rohitansky 1804 1878)
FEATURES
1 Two separate circulations (like foetal life)
2 The Aorta is from the right ventricle The pulmonary artery
is from the left ventricle
3 Severe cyanosis Only 10% of the pulmonary flow may pass
into systemic circuit
Therefore Circulation 10 times greater than normal
Therefore Large heart progressively
4 Variable systolic murmurs
5 Right ventricular hypertrophy
6 The second sound over the pulmonary area is greatly ac
centuated
7 The aorta is anterior to the pulmonary trunk
8 IVSD and PDA are present in a number of these
"Death! The spear that strikes but once"
59

·INTERAURICULAR SEPTAL DEFECT
(IASD)
17% 1 13 500
TYPES
I UNCOMPLICATED
1 Patent foramen ovale No shunt
2 Complete absence of septum
3 A persistent ostium primum Left to nght
4. A persistent ostium secundum Left to nght
5 Localized single or multiple defects Left to right
IT COMPLICATED
1 Atrioventricular communs Left to nght
2 PFO with
(1) A pulmonary stenosis with right to left shunt
(b) Mitral stenosis with left to right shunt
(c) Ebsteins tricuspid defect with a nght to left shunt
(d) Total anomalous pulmonary veins with right to left
shunt.
(e) The 2nd component in the second heart sound may
be widely separated during inspiration
Note the
large SVC and anomalous PV with this finding
3 IASD (I-CD or E above) with
(a) A pulmonary stenosis with a right to left shunt
(b) A mitral stenosis with a left to nght shunt
(c) Ebsteins malformation and a right to left shunt
(d) Tricuspid atresia with a night to left shunt
(e) Transposition of great vessels with a night to left
shunt
60

·Touard The Diagnosis of Congenital Heart Disease 61 DIFFERENTIAL DIAGNOSES The following should be considered 1 Rheumatic fever 2 Left to right shunts (PDA IVSD with anom veins) 3 Pulmonary hypertension from other causes 4 IASD with mitral stenosis indicates Lutembacher 5 IVSD 6 Aortic pulmonary "window 7 In ostium primum one gets early symptoms and pulmonary hypertension The M V and the TV are involved Pan systolic murmur is noted There is a left axis deviation FEATURES 1 There is a predominent female sex incidence 2 There may not be any symptoms until late 3 A feeding problem is usually encountered in infancy 4 The childs body is of the slender type and is often under developed Rheumatic fever affects them more frequently than normal children 5 The skin is frequently translucent 6 Marfans syndrome may be associated with an IASD in 150 of cases 7 Respirogenic changes occur in youth as dyspnoea on er ertion sighing fatigue and rapid respirations 8 Chest pain may be due to coronary insufficiency vascular dilatation or bronchial causes 9 A tapping apex beat or a tumultuous one due to over filling of the right ventricle is present There is a pre cordial bulge of the costal cartilages 10 There is a palpable pulsation in the out flow track 11 Cardıomegaly due to right auricle enlargement 12 Cervical venous pulsations are present when the normal child is recumbent but a wave is noted more distinctly 13 An hepatomegaly may be noted in failure of the heart It is insidious so that the hepatomegaly may lead to the diagnosis of longstanding heart failure

·62
Toward The Diagnosis of Congenital Heart Disease
14 Cyanosis occurs in complicated cases only Here there
may be added congenital lesions
15 A pulmonary systolic murmur with or without a thrill
16 A wide split second heart sound A thnll in 50% of cases
17 A diastolic murmur at the apex in h cases A systolic
murmur over the pulmonary artery area
18 A right bundle branch block is present in 95% of cases
19 In the ECG there is night aus deviation A large peaked
P2 in the ECG indicates an enlarged right auricle
20 Hılar pulsations are present
21 The aorta is small
22 A gross dilatation of the pulmonary artery
23 Pulmonary engorgement with repeated pneumonias
These are not inflammatory but due to alveolar congestion
by blood
24 Pulmonary pressure may rise while the systemic blood
pressure is normal
25 Globular shaped heart as seen in x ray
26 An enlarged right auricle and right ventricle
27 Pulmonary plethora Look for mitral stenosis in all cases
28 A left to right shunt Cyanosis is present when nght to
left shunt
29 Congestive failure may be early or late
30 Unexpected sudden death may occur at any age in infants
with I ASD

·INTERVENTRICULAR SEPTAL DEFECT
(IVSD)
(12%) 1 4000
A LEFT TO RIGHT SHUNT
COMMON
FEATURES
1 Symptoms may be absent until late Acute bacterial
endocarditis is common
2 Growth may be restricted if a large IVSD is present
Polydactylism and syndactylısm are commonly noted
(15%)
3 Cyanosis is absent Clubbing may occur of pulmonary
hypertension is present
4 Dyspnoea is noted if a large IVSD vanes by degree of
defect
5 A loud rasping systolic murmur and thrill over the 3rd and
4th ICS and middle sternum "It spreads like a npple
on the water after a stone is thrown"
6 A normal sized heart of the defect is not large
7 The pulmonary artery is enlarged and it pulsates
8 A normal blood pressure in the absence of hypertensive
lesions as C of A
9 A bulge may be present over the precordial region
10 A thrusting apex beat is noted in IVSD
11 The second heart sound at the pulmonary area is split A
pulmonary diastolic murmur is present when the pulmon
ary pressure is hugh in a small proportion of cases
63

·64 Toward The Diagnosts Of Congenital Heart Disease 12 A functional mitral diastolic murmur is present in 50% of cases 13 A pulmonary plethora is noted in the pray film The chambers are often normal in size 14 The ECG shows a left and right ventricular hyper trophy but this may occur if pulmonary hypertension is present 15 A high oxyhaemoglobin level is found in the right ventricle in the presence of this left to right shunt This is noted by catheterization 16 The defect is high and between the aortic and tricuspid valves (90%) 17 Tricuspid incompetence may be present 18 A right interventricular systolic pressure equal to the pressure in the systemic arteries is presumptive evidence of a large I VSD 19 Aortic regurgitation may be associated with I VSD 20 Catheterization shows the location of defect Pressure and blood gas changes are noted Oxygenated blood is found in the right ventricle and indicates left to right shunt 21 Cardioangiograms may localize the defect Here one may see a second filling of the R V from the LV DIFFERENTIAL DIAGNOSIS OF I VSD 1 Mild PV stenosis with a normal aortic root (higher site of defect) 2 Muld infundibular stenosis at aortic root (delayed P valve closure is noted) 3 Acyanotic examples of T of F 4 Mild aortic or subaortic stenosis (short systolic murmur) 5 Muld I ASD but heut contour is different 6 PDA A continuous murmur and is not systolic and alone as an IVSD 7 Haemic murmur

·Touard The Diagnosts Of Congenital Heart Disease
65
8 A hugh ventricular septal defect is not a true mal de
Rogers
9 Pectus excavatum may cause a mechanical systolic mur
mur over the same area

·MITRAL ATRESIA (MA)
(Jean Baptiste De Senac 1693 1770)
1 Cyanosis from buth (decreased pulmonary flow)
Note (a) Ductus is closed
(b) ECG There is right axis deviation
2 Severe disability Early death unless attended to surgically
3 Precordial systolic murmur
4 The left ventricle is small The right ventricle shows
hypertrophy
5 The great vessels may be transposed
6 ASD left to right shunt
7 Decreased pulmonary fox Due to high pressure in LA
8 Patent foramen ovale
9 There is a cor triloculare biatriatum with A V mixing of
blood in the heart
10 Less common than tricuspid atresia
11 The aorta arises antenorly and is narrower than the PA
12 Aortic valve atresia may be present
"A number of lesions in adult heart are con
genital in origin but missed or not looked for at a
younger age
66

·MITRAL STENOSIS (Congenital)
I Like acquired in symptoms but many variations
2 It is associated with I ASD in a number of cases
Always search for it
For a full study of the subject kindly refer to the following
detailed articles
1 Congemtal Mitral Stenosis
By Charlotte Trenencz MD
Circulation Vol IX No 2 February 1954
2 Congenital Anomalies of the Mitral Valve
by John T Prior MD
American Heart Journal Vol 46 No 5 November
1953
3 Congenital Mitral Stenosis
By Henry Swan MD
American Heart Journal Vol 38 No 6 December
1949
4 Congenital Mstral Stenosis
By Robert W Cooley MD
Circulation Vol 76 No 4 October 1956
5 Refer also to more recent literature on this lesion
"Knou ledge knows no frontiers
67

·LUTEMBACHER SYNDROME (LS)
(Not common)
FEATURES
1 IASD with mitral stenosis
2 Signs are those of these lesions separately
3 A large pulmonary artery is present
4 Jugular pressure is raised
5 Pressure gradient across the valve in IASD generally
means the presence of mitral stenosis
6 Incidence of stenosis as 5% with I ASD
7 In treatment one should open the mitral valve before
closing the I ASD

·COR TRILOCULARE BIATRIATUM
(Three chambered heart) (2 A plus I Vent)
(Thomas B Peacock 1812 1882)
1 Cyanosis is early mild and transient
2 A systolic murmur is heard over the entire precordium
3 X ray shows an unusual globular heart
4 Transposition of the aorta and the pulmonary artery is
rarely seen
5 The O, saturation of the arterial blood is subnormal
6 The ECG shows high voltage A diphasic Q R S com
plex is present
7 The o saturation of the haemoglobin in the ventncle
exceeds the haemoglobin of mixed venous blood (cathet
er)
"The reu ard of success will attend our efforts in
so far as the heart is treated as one of the ordinary
tissues of the body and the method of operation con
forms to the fundamental principles of surgery"
SIR RUSSELL C BROCK
The 1958 Bradshaw Lecture
1883 Billroth s unwise remark
"The surgeon who should attempt the future of the
heart would lose the respect of his colleagues"
"Even though the heart is but l cm from the surface
of the body It has taken * 2000 years to reach #t"
69

·TAUSSIG-BING COMPLEX (TBC) (Rarest)
(INCOMPLETE TRANSPOSITION WITH
BIVENTRICULAR ORIGIN OF
PULMONARY ARTERY)
1 Cyanosis
2 A transposed aorta
3 A large pulmonary artery from the right ventricle which
overndes the ventricular septum
4 A high VSD
70

·DEXTROCARDIA
1 The primitive cardiac loop swings in the reverse direction
to the normal
2 The course of the circulation is the mirror image of the
normal Situs inversus of viscera is always present
3 Asymptomatic unless other lesions are associated with it
4 The ECG 15 pathognomonic
5 May have a left aortic arch with cyanosis
6 Bronchiectasis as frequently associated with dextrocardia
due to pressure of the abnormal position of the heart on
the bronchi
7 Auscultation and x rays pick it up
DIFFERENTIAL DIAGNOSIS
1 A displaced heart from atelectasis pneumothorax or con
genital absence of the right lung
2 Incomplete rotation of the heart
3 Right ventricular hernia or a pericardial cyst
71

·TRICUSPID ATRESIA (TA)
(William Harvey 1578 1659)
FEATURES
1 Cyanosis from birth
2 The IVC and SVC syndromes are present The venous
pressures are elevated
3 Severe spontaneous dvspnoeic attacks may occur
4 A basal systolic murmur is present
5 If a picture of T of F is present but hypertrophy of the
Jeft ventricle and ECG changes it is not likely 1 TA
6 Eiglity per cent have an associated pulmonary stenosis
7 There is no communciation between the nght auricle and
the right ventricle
8 The PA is smaller than the aorta
9 There is a configuration of a Dutch shoe in the contour
of the heart
10 The ECG shows left aus deviation
11 A patent F ovale is present and the ductus arteriosus
is not patent
12 The pulmonarı trunk may arise from a small RV and
can be reached only via the IVSD
13 Cardioangiograms are useful here
72

·PERSISTENT COMMON
ATRIOVENTRICULAR CANAL
(Franklin Paine Mall 1862 1917)
FEATURES
1 A V canal is undivided and guided by a single valve of
a large posterior cusp and small lateral cusp
2 An I ASD is present
3 Variable cyanosis-mustaken for I ASD (primary)
4 A systolic murmur is heard over the mid precordium and
apex
5 A left and right ventricular hypertrophy is present
6 The ECG shows hugh voltage Biphasic RS com
plexes are noted
73

·BLAND-WHITE-GARLAND SYNDROME
FEATURES
1 Attacks of dyspnoea, cyanosis and pallor
2 Coronary ischaemic pain drawing up of the legs and
extension of the neck during and after feeding May
have a soft blowing murmur
3 The left auricle is greatly enlarged Feeding problems
occur after tvo months
4 There is evidence of anterio-lateral infarction in ECG
in the infant
5 The left coronary artery anses from the pulmonary artery
6 Sudden death may occur at 2 13 months
7 Should differentiate from subendocardial fibroelastosus
congenital aortic stenosis and Fredler s myocarditis

·PERSISTENT TRUNCUS ARTERIOSUS
(Sir Arthur Leith 1866-
FEATURES
1 A single artenal vessel (aorta and pulmonary artery are
one)
2 Anses from both ventricles
3 A short septum in the truncus divides it into a short aorta
and pulmonary artery
4 Cyanosis moderately severe
5 X ray-night and left ventricle enlarged
6 Hılar shadows increased when cyanosed
7 ECG shows right axis deviation
8 O, saturation is below normal
9 No PDA
75

·BLAND-WHITE-GARLAND SYNDROME
FEATURES
1 Attacks of dyspnoea cyanosis and pallor
2 Coronary ischaemic pain drawing up of the legs and
extension of the neck during and after feeding May
have a soft blowing murmur
3 The left auricle is greatly enlarged Feeding problems
occur after two months
4 There is evidence of anterio-lateral infarction in ECG
in the infant
5 The left coronary artery arises from the pulmonary artery
6 Sudden death may occur at 2 - 13 months
7 Should differentiate from subendocardial fibroelastosis
congenital aortic stenosis and Fredler s myocarditis
74

·CONGENITAL PULMONARY
ARTERIOVENOUS FISTULAE
A manifestation of hereditary haemorrhagic telangiectasis
(redundant Osler Weber disease) in percentage of cases
THE FOLLOWING FEATURES MAY BE NOTED
1 Cyanosis and clubbing of fingers and toes There is dy
spnoea on exertion
2 Haemoptosis and epistaxis
3 Chest pains thoracoepigastric pain in adults due to pul
monary hypertension
4 A continuous hum or systolic murmur with extraventricular
systoles is frequently detected
5 No cardiac enlargement
6 Forty percent have telangiectasis elsewhere in the body
Eg Maffucci s syndrome
7 The red blood counts are increased (polycythaemia)
8 The blood viscosity and blood volume are increased
9 The haematocrit is greatly increased
10 The O2 saturation is decreased to 70%
11 X ray findings of opacities (discs) or grape like shadows
in the lung field
12 The incidence of SABE increases
13 If cerebral thrombosis Occurs due to increased anoxia and
Viscosity then headaches vertigo convulsions and paresis
may occur
14 Variation in size of the lung shadow with Valsavas ex
periment. Must differentiate from Artilaga Ayerza s dis
77

·COR BILOCULARE
(TIVO CHAMBER HEART)
(William Hunter 1718-1783)
FEATURES
1 Infant death is characteristic of this lesion
2 No murmurs
3 Early and progressive cyanosis
4 A single atrum
5 A single ventricle
6 The PA lies behind the aorta
7 The flow to the lungs is via the PDA
8 Most primitive heart
9 Dextrocardia often is associated
10 Globular enlargement of the heart
11 The atrium receives blood from the lungs via the pulmon
ary veins
12 Peripheral blood via the IVC and a nght and a left
SVC are present
76

·CONGENITAL PULMONARY
ARTERIOVENOUS FISTULAE
A manifestation of hereditary haemorrhagic telangiectasis
(redundant Osler Weber disease) in percentage of cases
THE FOLLOWING FEATURES MAY BE NOTED
1 Cyanosis and clubbing of fingers and toes There is dy
spnoea on exertion
2 Haemoptosis and epistaxis
3 Chest pains thoracoepigastric pain in adults, due to pul
monary hypertension
4 A continuous hum or systolic murmur with extraventricular
systoles is frequently detected
5 No cardiac enlargement.
6 Forty percent have telangiectasis elsewhere in the body
Eg Maffucci s syndrome
7 The red blood counts are increased (polycythaemia)
8 The blood viscosity and blood volume are increased
9 The haematocrt is greatly increased
10 The Oz saturation is decreased to 70%
II X ray findings of opacities (discs) or grape like shadows
in the lung field
12 The incidence of SABE increases
13 If cerebral thrombosis occurs due to increased anoxia and
viscosity then headaches vertigo convulsions and paresis
may occur
14 Variation in size of the lung shadow with Valsavas ex
periment Must differentiate from Arrulaga Ayerzas dis
77

·78 Toward The Diagnosis of Congenital Heart Disease ease, Tetralogy of Fallot and Eisenmenger malformation 15 May associate Maffuccis syndrome and haemangiomata with A V fistula and hemihypertrophy of the limbs (See Wm Beans work of Iowa City) Let us not mistake extracardiac lesions as coelomic cysts pulmonary A V fistulae or pericardial effusions for primary cardiac lesions * ·The Principle Clinical Fındıngs In Cyanotic Heart Lesions 1 W Brown Signs and Te af Ry Fuenmenges Pul norefy Se to Tran post on Persistent Trunsngle Tr cuspid af Fall 1 Syne ptoms 55 FO al rit ttte a Venttiele or Grear v selsicus Afers ses CYANOSIS Late and Early and Early Proges Marked Marked Masted Marked marked sucht 5 + y mai 1 CLUBBING Ma ke 1 Slight Si ght to ma ked Marked Marked Marked Marked POLYCYTHEMIA Serere Later stages Sever Severe Severe Severe Severe SYMPTOMS Ch ldhood Severe Present early Present and Present early Present catly Severe severe and carly_ PHYSICAL Gross)y Littc Se ere Grossly Grossly Grossly Gro sly CAPACITY umpared DYSPNOEA Se ere Squats Some Severe Sesere Squats Severe Severe Srvere SYSTOLIL May be absent Pulmonary Pulmonary area Absent Often absent Present or Present MURMUR En pul asta WthI VSD absent arca or absent DIASTOLIC Rarely Frequ nt from Occas onally Absent Occasionally Absent MURMUR present aortic or pui incompetence PULMONARY Unaltered Accentuated Dimini hed Loud and not unaltered Loud and purc Vnaltered SECOND SOUND often reduplicated X RAY Aormal Slight to Sight Often present Enlargement Gross Moderate ENLARCEMENT moderate enlargement PULMONARY Concave Convex Consex and up Concave Concaye Conceit in Early slight ARC late childhood convexily HILAR Small Nomal Norma! Sma!) Normal Small Small SHADOWS KILAR NAS Present Present Absent Present Alisce Abscot PULSATION PULMONARY May be None None Present None CONCESTION prestait ECG Marked Marked Marked RT axis Oc RI Axis Rt. La RAXIS casional normal Axil P WAVES Tall & peaked Enlarged Enlarged Enlarged Enlarged Enlarged Enlarged CIRCUL TIME Shortened Shortened Unaltered Unaltered Unsltered and up IN? None Lelt 2x11 ·WHEN SHOULD AN OPERATION TAKE PLACE (LAM) Diagnose Type of Operation May be Dons Should be Done Youngest Successful AORTIO RING Division Soon as diagnosed Soon as diagnosed 3 weeks PATENT DUCTUS ARTERIOSUS Ligation or division Soon as diagnosed Soon as dragnosed 6 weeks COARCTATION Resection As early as possible 2 years plus ox carler 21 necessary 16 months VALVULAR PULMONARY STENOSIS Valulotomy As early as in trouble As early as in trouble 7 months TETRALOGY OF FALLOT Potts or Elalocks As early as in trouble 2 years plus or earlier if necessary 9 months TRICUSPID ATRESIA Blalocks Ar Potts As early as in trouble After 15 18 months of possible 5 saontha TAS.D Closure With rymptoms and pulmonary hypertension Enlarging auticle 6 months IVS.D Closure After SABE Sympion and Lt Rt. stunt 6 months ·CARDIAC ARREST -THE DIAGNOSIS AND TREATMENT 1 The heart is the most efficient pump in the world If at stops we must restore the beat "within three minutes" Each year it beats 37 879 700 times!! 2 Signs of cardiac arrest After a period of slowing and a lowering blood pressure there are the following (a) No pulse (b) No blood pressure (c) Dilated pupils When did the heart stop beating? We can only know the answer from the anaesthetist in charge It is he who is the best "monitor" An alert anaesthetist is the best mon itor" 3 Cardiac arrest is like turning off the ignition in a good In CA something has been turned off 4 The heart never ceases to beat except for an adequate reason Its cause can generally be found on review 5 It will be malpractice by 2000 AD not to attempt restora tion of the heart beat when cardiac arrest occurs in an otherwise healthy body A number have been restored following coronary infarction and should be attempted in such cases in the absence of severe arteriosclerosis or car old age 6 Cardiac arrest may occur anywhere at anytime during examination of performing even a minor operation upon a patient 81 ·82 Toward The Diagnosis of Congenital Heart Disease All who practice medicine should be able to massage an arrested heart within three minutes of its cessation One should never be criticized for trying to save a life 8 Every room in the Emergency Ward and the OR should have a small tin case hanging on the wall containing the following (a) Intratracheal tube (adult and child) (b) A scalpel (c) A 20cc syringe with a long fine needle (d) Calcium chloride-5% ampule (e) Procaine-1% ampule (f) Adrenaline-1 10000 impule Oxygen should always be available 9 Every OR should have a ready defibrillator 10 The brain Lust have blood loaded with O, to survive beyond three minutes Therefore remember Pump + OXYCEN + BRAIN + HEART before three minutes 11 Aetrology of Cardiac Arrest (a) Poor preparation of the patientmphysically and men tally (b) O, lack in the brain and heart (c) CO, increase in lungs and circulation (d) Electrolytic imbalance and excess of drugs (e) Reflex stimulation of vagus neru (f) Lowered blood pressure with anoxia (g) Lack of careful obser ation or "watchful expectancy" on the part of the attendant 12 Rarely do signs come on suddenly" They appear "sud den" because the pulse and the B P have not been watched constantly 13 Potassium citrate and acetylcholine cause cardiac arrest ·83 Touard The Diagnosts Of Congemtal Heart Disease 14 Calcium chloride controls the rhythm of the heart beat PREVENTION OF CARDIAC ARREST 1 Make the patient safe for surgery 2 Think of its possibility during any examination Eg Carohd body Before and danng any operation no matter how minor 3 Watch for it. The best "monitor is an alert anacsthetist 4 Use of an amplifying stethoscope ECG Electric monitor or a stethoscope strapped over the hicart where possible during the operation 5 The use of digitalis pre operatively in bad risk cases 6 Adequate Oz and CO, control 7 Prevent hypotension "Treat the tissues as though they were cobwebs thus prevent shock 8 Increase pulmopary reserve by respiratory exercises 9 Keep bronchi and bronchioles clear of secretions Allow Oto reach the alveolar sacs 10 Select the safest" anaesthetic 11 Adequate level of sedation 12 Careful consideration of added drugs during prolonged operations An accumulative effect may occur 13 Use of atropine (prevents bradycardia) 14 keep the fingers on the pulse instead of "crossing the fingers! Avoid constricting arm bands or twisted neck 15 Avoid compressing a sensitive carotid body 16 Careful and gradual induction of anesthesia 17 Adequate airway (size and depth) 18 Continuous observation of pulse and BP Alkit always slows before stopping this is the time to awaken to the danger of arrest Stay by the patient When did the heart stop beating? The answer yførstly prognosis 19 Anaesthetize the trachea before intutsatlon ·84 Toward The Diagnosis of Congenital Heart Disease 20 Care with hyperventilation with O, at end of operation Too sudden release of built up CO, by 0, is dangerous 21 Care with positioning and posturing the patient If in shght Trendelengerg on the operating table then the stretcher and bed must be ready in this position before moving the patient IF THE HEART HAS STOPPED BEATING "It is TIME teamwork massage oxygen and tena city all together 1 Do not question it or stand around 2 Do not listen for a "faint beat, that faint" beat has al ready passed! It's later than you think! 3 Do not wait for an ECG or B P apparatus 4 Do not ask for gloves or gown but ONLY for a scalpel knife or blade "You can clean up infection but you cannot clean up death 5 Do not use adrenalın into an unseen heart 6 Do not use mechanical respiration It is a pump you need the pump is your hand Use it immediately 7 Do not force O, into the trachea, the blood must be moving before it can reach the brain--give O, and massage to gether 8 Do not dilate the anal sphincter 9 Do not transfuse or increase I V unul massage has started and 02 is Teaching the lungs and brain If CA is from haemorrhage, then force blood quickly 10 Do not go through or massage diaphragm 11 Do Not Warr A SECOND or waste time "hoping“ it is not С.А BUT AT ONCE-take courage and get to the heart IMMEDIATELY while the following are being done (at the same time) 1 Note time by clock (Have a time-caller ) ·Toward The Diagnosis of Congenital Heart Ducase 85 2 Stop anaesthetic at once 3 Tip table down 15° Give Os via the tube or mask. 4 Open 5th left interspace wide (in 6 seconds) 5 Spread nbs with hands later with spreaders 6 Open pericardium Note heart. 7 If not fibrillating (only 10% do) massage with one or both hands Compress and relax 40 - 50 times per minute Steady and evenly BP will now appear 8 If fibrillating, apply defibrillator If not one about use an electric current to heart or rapid needle pricks into heart muscle might benefit. But, MASSAGE always Keep the brain orygenated until fibrillation is controlled and the heart beat restored. 9 The heart will always respond to massage but the brain cannot go without O. longer than three minutes 10 If the heart is flabby and irregular inject one of the follow ing and massage (a) 10 cc of 5% calcium chloride into left ventricle (b) 3 cc of 1 10 000 Adrenalin (c) 9.5 cc 1% procaine hydrochloride and 5 cc adrenalin into LV These may be repeated with care 11 Massage and observe but massage 12 Massage until ngor mortis sets in if necessary This will not set in if we massage early and give O, before three minutes of cessation 13 Bleeding from the wound is a good sign that blood is reaching the brain also 14 When all is restored, then attend to closure and observe the brain and kidney post-operatively for any damage due to cardiac arrest May use hypothermia for several days ·OMEGA If this little book causes you to see what you look for or stimulates you to learn what you do not know in the diagnosis of congenital heart disease, if it causes you to see the Great Path of Duty” in Medicine-correct diagnosing and to fol low it more bravely, more faithfully and more successfully if it will stimulate the graduate medical student to enter the field of cardiac surgery or cardiology in which field so much more can be accomplished it then will hive accom plished the cluef object that prompted the author to write "Toward the gnosis of Congenital Heart Discase 86