·No text on this page.

·The Salent Points and The l'alue of l'enous ingrocardiography
m the Dragnosis of the Cyanolic Types of
Cougemal Mallonations of the Heart

·No text on this page.

·The Salient Points and the
Value of Venous Angiocardiography
In the Diagnosis of the Cyanotic
Types of Congenital Malformations
of the Heart
A TEN YEAR STUDY OF 421 ANGIOCARDIOGRAMIS
DONE ON 283 PATIENTS
Ву
BENJAMIN M. GASUL, M.D.
GERSHON HAIT, M.D.
ROBERT F. DILLON, M.D.
EGBERT H. FELL, M.D.
I
SYMBOLSAS
CHARLES CTHOMAS PUBLISHER
Springfield · Illinois · U.S.A.

·CHARLIS C TIONIS. PUBLISHI.R
BAIAFRSION HOUSE
301-327 Cast LABRE\CO ATL\UE, SPRINGFIFI D, ILLINOIS, USA
Published simultaneously in the British Commonwealth of Nations by
BECKSEIL SCILATTIC PUBIICATIONS, LTD, OXFORD, ENGA\D
Published simultaneously in Canada by
TIF RYERSOY PRESS, TOROVIO
This book is protected by copyright No part
of it may be reproduced in any manner with
out written permission from the publisher
Copyright 1957, by CI SRLES C THOMAS
PUBLISHER
Library of Congress Catalog Card Number 56-12447
Printed in the United States of America

·CONTRIBUTORS
BHJAVIN W. GASUL, MD
Director, Pediatric Cardiophysiology Department
Cook County Children's Hospital and
Hektoen Institute for Medical Research
Clinical Professor of Pediatrics
larrrrsity of Hors College of Medicine
GERSION HAIT, SI.D
Fellow in Pediatric Cardiology
Cook County Children's Hospital
Chicago, Illinois
ROBERT F. Dilov, MD
(ardiophicologist, Cook County Ffosfatal
4 su strut Professor of educine, Strict School of Medicine
Chicago, Illinois
EGBERT H. FEIT, M.D
Cimical Professor of Surgery (Rush)
Interstly of nors School of Medicine
ftlending Surgeon, Cook County and The Presbyterian Hospitals
Chicago, Ilinois

·No text on this page.

·PREFACE Tlus lxxs is the result of a ten year study of 121 angiocardiograins, cach one consisting of approximately thenty films, taken in 283 patients with cyanotic types of congenital malformations of the heart. Tlıc salient points in the angiocardiographic diagnosis of ren enunjes are presented. In order to best establish the value of angiocardiography as a separate laboratory tool, the authors biased the angiocardiographic thiaguinis « cach entity only on the knowledge that the patients ucre cyanatu, and on the interpretation of the angiocardiographic incluang The accuracy of tlus angiocardiographic diagnosis was then clicched in cachi case against the final diagnosis arrived at ly a complete physical, fluoroscopic, roentgenologic and electrocardiographic clamination and, in addition, by cardiac catheterization and or surgery and 'or autopsy Twentyone entities of the cyanolic type of congenital mal. formations of the heart are classified into four groups. We wish to) implaswe that this manuscript represents only a suurimary of the faste findings of the most important types of congenital mal furt10ns of the heart. The authors ssisin ti e tend their thanks and appreciation in Ir (harles T Dexter, Professex and Chairman, Department of Kadiology of the tiniversity of Oregon Medical School, for read- 10% the posts and sering many helpful suggestions. Ile also wish to thank our clucl icchrician, Mr. Glen Volz and our artist, Vrs (loria Jones who have faithfully assisted in this work since its inception. the total number of angincardungsams that the authors hase studii on proven in a cys and monolic spes of congenual malformations of the heats Lien

·No text on this page.

·CONTENTS Y vit CONTRIBUTORS TAFTACE, INTRODUCTION 3 7 7 7 9 16 16 GROUP I TITRAI OXY OF FAI LOT Pathology Hemodynamics Salien angiographic features COMPITF TRAVSPOSITION OF THE GREAT VESSELS. Pathology (exy namics Salient angiographic features IXOMATOUS DRAIVACE OF ALL PULVINARI VFINS I TO TU SE PRACAKDIAC SISTEM VFINS Packakyy Il modynamics Salient angiograpluit features 16 18 20 20 99 27 27 27 GROUP 2 KIE PIP TKESIA Parolesy Semenynas Salicut angiographix (catures I'UTIONARY STE IS WITH NORMAL AORTIC ROOT AND ATRIAL (OU WICATION AV RIIT TO 1.FFT SHIUYT. l'achalogy Ilmasta.IS Salierit anigmaraph fratures L'EXWXXE TRE CJRTFRJOSUS Vathology Ilemelynamics Salient angiograpluc features 35 37 13 ·l'enous Angiocardiography and Congenital Malformations MITRAL ATRESIA WITH VENTRICULAR SEPTAL DEFECI 50 Pathology 50 Hemodynamics 50 Salient angiographic features 52 GROUP 3 TAUSSIG-BING ANOVALY 57 Pathology 57 Hemodynamics 57 Salient angiographic ſeatures. 59 EISENMENGER COMPLEX 60 Pathology 60 Hemodynamics. 60 Salient angiographic features 62 PONTALOGY 61 Pathology 61 Hemodynamics 61 Salient angiographic features. 66 DISCUSSION 68 Tetralogy of Fallot . 68 Transposition of the Great Vessels 68 Anomalous Drainage of All Pulmonary Veins into Supracar. diac Systemic Veins 70 Tricuspid Atresia 70 Pulmonary Stenosis with Normal Aortic Root and Atrial Communication and Right to Left Shunt 72 Persistent Truncus Arteriosius 72 Mitral Atresia with Ventricular Septal Defect 72 Taussig-Bing Anomaly 73 Eisenmenger's Complex 73 Pentalogy .. 73 COMMENTS 75 CO\CLUSIONS 77 REFERENCES 79

·The Salient Points and The l'alue of l'enous Angiocardiography
in the Diagnosis of the Cyanotic Types of
Congenital Malformations of the Heart

·The following key is for the drawings on pages 8, 17, 21, 28, 36, 14, 15, 51, 58, 61, 63
KEY
Venous blood
Arterial blood
Mixed blood predominantly arterial
Mixed blood predominantly venous
SVC-Superior Vena Cava
IVC Inferior Vena Citta
RI-Right Itrium
RV-Right Ventricic
P-l'ulmonary Artery
P! - Pulmonary Veins
L-Icft trim
LV- cft ventricle
10- torta
CT-Common Crunk
Br 1-Bronchial Artery
The ligures in the cardiac chambers and in the sessels denote the respectic per.
centage of their oxygen saturation

·INTRODUCTION Tllic angiocardiographic eltagnosis of the various cyanotic types of congenital malformacions of the lieart and to establish the value of angiocardiograplıy as a diagnostic tool for these entities. We, therefore, based our diagnosis on angiocardiographic findings alone and the knon ledge that the parents were cyanotic, In order to best establish the cagnostic value of angiocardiography as a separate tafxratory tool. all other available information regarding the history, pliysıcal, fluoroscopic, soentgenologic, electrocardiogapire careliac cathieteswation and autopsy findings was purposely <?pted Durming the past ten years 121 angiocardiograms, each consisting of an average of twenty filins, were done on 283 patients 11th canetic types of congenital malformation of the heart. men fue percent of our patients were under lifteen years of de complete physical, fluoroscopic, roentgenologic and electro- Carcuryrapluie camination was done routinely on all patients doc fore 211 2113 xarilogram was performed. In addition to these (ummatrons the diagnosis was confirmed in U19 patients by cardiac Beratum. in 101 patients by surgery and in 98 patients by pose littdmys. As a result of this study, we believe that the Guagunit salue of angixardiography can be best demonstrated di classifying the anodic types of congenital malformation of the heart into our groups and by the presentation of their salient anyn xarigraplus xsmts * from the lichten Insistente for tredical Research, the Pediatric Cardiophovickup tepariment of fun fronts Children's Hospital, the j'edaalric and 64kcal Depatiscant of the Lanersity of Nlinois College of Techcine and of the Irels (cuan lleputul Chisapn nous luis suis *as tuled by a grant from the cha katt sexuation 3 ·1 l'enous Angiocardiography and Congenital Malformations Group 1 Entities in which the diagnosis can ALMOST ALI'AY'S bc made by proper interpretation of a technically good angiocardiogram (Table 1). Group 2 Entities in which the diagnosis can USUALLY be made by proper interpretation of a technically good angiocardiogram (Table 2). Group 3 Entities in which the diagnosis can USUALLI'NOT BE ALADE by proper interpretation of a technically good angiocardiogram (Table 3). Group 4 Entities which ALIWAYS REQUIRE additional studies before a definite diagnosis can be established (Table 4). The salient points in the angiocardiographic diagnosis of the following ten cyanotic types of congenital malformations of the beart which belong to our Groups 1, 2 and 3 will now be presented briefly. Eleven other types of cyanotic congenital malformations of the heart belonging to Group 4, which represent entities where the value of angiocardiography alone is limited, will not be discussed.

·GROUP 1 ·Venous Angiocardiography and Congenital Malformations Group 1 Entities in which the diagnosis can ALMOST ALII'Y'S be made by proper interpretation of a technically good angiocardiogram (Table 1). Group 2 Entities in which the diagnosis can USUALLY be made by proper interpretation of a technically good angiocardiogram (Table 2). Group 3 Entities in which the diagnosis can USUALLY NOT BE MADE by proper interpretation of a technically good angiocardiogram (Table 3). Group 4 Entities which ALIVAYS REQUIRE additional studies before a definite diagnosis can be established (Table 1). The salient points in the angiocardiographuc diagnosis of the following ten cyanotic types of congenital malformations of the heart which belong to our Groups 1, 2 and 3 will not be presented briefly. Eleven other types of cyanotic congenital malformations of the heart belonging to Group 4, which represent entities where the value of angiocardiography alone is limited, will not be discussed.

·GROUP 1

·No text on this page.

·TETRALOGY OF FALLOT PATHOLOGY 1 Varying degrees of inlundibular stenosis or combined in. fundibular and valvular stenosis Rarely, only valvular stenosis urch is then usually associated with post-stenotic dilatation of the pulmonary artery Exceptionally, valvular or pulmonary atresta 2 large defect in the membranous and upper muscular portion of the ventricular septun larying degrees of functional de troposition (overriding of Borla 1 Because of the above anomalies, the right ventricle is lyper. crophed and, in some cases, dilated. HCJIODINAMICS Systemic lenous blood returning to the righe atrium and ven truke is dueried from the lung in proportion to the degree of ulastruc 21011 saused by the stenosis. The senous blood which can. not enter the stenone outflow tract will follow a path of lesser resistance and pass into the aorta Tlus produces cyanosis. The sys- 2016 pressure in the right ventricle is approximately the same as in the left sentru le In all cases the pulmonary blood fox is de +Teased and the systeinic fou is increased in cases where the pulmonan artery is atretc, the pulmonary circulation is maintained 1x brettchual arteries or other collateral vessels 7

·8
Venous Angiocardiography and Congenital Malformations
89.8%
69%
71.5%
96%
68%
71%
Gloria Jones
TETRALOGY OF FALLOT
(See key on page 2)

·9 Tetralogy of Fallot Salient Angiographic Features 1. Simultaneous visualization of the aorta and pulmonary arterjes (Ig. 1-a). ? In the majority of cases the main pulmonary artery and the branches are hypoplastic and the aorta is wide. In about 20% of thiese cases there is a right aortic arch (fig. 1-6) 3. Patients with valsalar or combined stenosis usually rescal a post-stenotic dilatation of tlic pulmonary artery. 4. In some patients with localized infundibular stenosis associated with valvular stenosis, there is opacification of a third renuncle. The site of this thurd ventricle varies depending upon the space between tlic localıed infundibular stenosis and the pulmonary valies (Fig. 1-c). Opacification of the peripheral pulmonary vessels is diminished. 6. Faint or no opacification of the left cardiac chambers. Usu. ally the ſainter ile opacification the more severe is the malforma- Tion. The opacification of the collateral vessels in these seiere malformations is oftentimes sell visualized (Fig. 1-d). ī The type of stenosis (valvular, infundibular vir combined) emik at (imes not be deterinined in our series (Fig l.e and l-I).

·8
l'enous Angiocardiography and Congenital Mfalformations
89.8%
69%
71.5%
96%
68%
71%
Glois Jones
TETRALOGY OF FALLOT
(See Key on page 2)

·9 Tetralogy of Fallot Salient Angiographic Features 1 Simultaneous visualization of the aorta and pulmonary arteries (Fig 1-a), 2 In the majority of cases the main pulmonary arter, and the branches are hypoplastic and the aorta is wide, In about 20% of these cases there is a right aortic arch (Fig. 1-b). % l'atients with valvular or combined stenosis usually reveal a post stenotic dilatation of the pulmonary artery, 4. In some patients with localized infundibular stenosis associated with valvular stenosis, there is opacification of a third sentrcle The site of this third ventricle varies depending upon the space between the localized infundibular stenosis and the pulmonary valves (Fig. 1-c). 5. Opacification of the peripheral pulmonary vessels is dimin. islied. 6 laint, or no opacification of the left cardiac chambers. L'1- ally iic fainter the opacification the more severe is the malforma- 1101. The opacification of the collateral sessels in these severe malformatívyns is oftentimes well visualized (Fig. 1-d). 7 The type of stenosis (valvular, infundibular or combined) would at cities not be determined in our series (Fig. l-e and l.).

·10 l'enous Angiocardiography and Congenital Malformations Ao L.P.A M.P.A. Figure 1-a. Tetralogy of Fallot A-P view of a typical severe tetralogy of Fallot at 11/2 seconds. Early and simultaneous visualization of hypo. plastic pulmonary arteries and of a marledly dextroposed wide aorta. Note the location of the pulmonary semilunar valves (arrow), the opacification of the peripheral pulmonary vessels is diminished MPAmain pulmonary artery; LPA-left pulmonary artery, AO-aorta

·11
Tetralogy of Tallot
РА.
Ao.
3V
R.V.
Figure 1.b .1.P sex at 2-seconds. Simultaneous visualization of pul.
monary arteries and of a wide aorta with a right aortic arch. RV-right
entrele, 3V thurd ventricle, l'A-pulmonary artery, AO aorta.

·12 l'enous Angiocardiography and Congenital Aſalformations MP.A. RV. RA Figure 1-c. A-P view at 1-second. Simultaneous visualization of a wide aorta with a left aortic arch, and of the pulmonary arteries The in. fundibulum is very narrok (lower arrow), and the pulmonary semilunar valves are visualized (upper arrow). The third ventricle is seen between the two arrows. RA-right atrium, Rl-right ventricle, MPAmain pulmonary artery.

·19
Tetralogy of Fallot
Figure 1-4. AP vien at 572 seconds Severe tetralogy of Fallot with
laint apaciliation of the lelt cardíac chambers. The reticulated ap.
perance of the peripheral lung fields is due to marked collateral cir.
cularton,

·l'enous Angiocardiography and Congenital Afalformations Ao. Figure l-e LAO view at 1/2 seconds. Vote the overriding of the aorta partially from the opacified right rentricle and partially from the non-opacified left ventricle There is simultaneous visualization of markedly hypoplastic pulmonary arteries A0-aorta, P.1.pulmonary arter), ·15 Tetralogy of Fallot Ао Figure 1.1 RAO view at 2 seconds Note the stenotic infundibulum of the right ventricle which persisted throughout several cardiac cycles and is, therefore, a sign of generalized infundibular stenosis, and is not dute to a momentary infundibular contraction in systole. There is an absence of the inferior vena cava and the hepatic veins enter the right atrium l-infundibulum, .10-aorta, Azl'azygos sein; H.V' hepatic vein.

·COMPLETE TRANSPOSITION OF THE GREAT VESSELS PATHOLOGY 1. Aorta originates from the right ventricle. Pulmonary artery from the left ventricle. Rarely, pulmonary stenosis may be present. 2. Associated defects in the form of atrial or ventricular septal defects or patent ductus arteriosus or anomalous entrance of pulmonary veins into the systemic seins or right atrium may be present. HEMODYNAMICS Venous blood enters the aorta from the right ventricle and ovygenated blood is pumped to the pulmonary circuit from the left ventricle. Life can only be maintained if associated defects that permit the mixing of venous and oxygenated blood are present. The more defects there are, the longer life is prolonged. 16

·17
Transposition of the Great l'essels
48%
33%
91%
96%
A410
499
Gliej.
37%
COMPLETE TRANSPOSITION OF ARTERIAL TRUNKS
(See hry on page 2)

·18 l'enous Angiocardiography and Congenital Malformations Salient Angiographic Features 1. Left anterior oblique is the best view for the demonstration of an anteriorly displaced, densely opacified ascending aorta and of a very wide aortic arch (Fig. 2-a). 2. Absence of, or faint opacification of the pulmonary arteries at the time of the opacification of the aorta is characteristic. 3. Associated lesions such as septal defects or patent ductus arteriosus may at times be demonstrated (Fig. 2-b). Ao Ao.I. RAA Figure 2-a. Transposition of the Great l'essels. LAO view at 42 second Note the size of the ascending aorta arising from the right ventricle and the anteriorly displaced wide aortic arch. The pulmonary artery is not opacified The infundibulum of the aorta is visualized in the region of the patent ductus arteriosus. R A-riglie atrial appendiige, 10 aorta; Ao.l.-aortic infundibulum.

·Transposition of the Grent l'essels 19 1 The area of the cardiac silliouette that is not opacified during right lieart filling (that is the area of the left atrium and of the leſt ventricle) is surprisingly large in view of the cyanosis. Ao. P.A. Figure 2b 110 view at 1-second Opacification of the transposed aorta with a wide aortic arch. Visualization of the patent ductus arteriosus anel of the pulmonary artery 10-aorta, PD 1-patent ductus arteriosis, P. pulmonary artery ·ANOMALOUS DRAINAGE OF ALL PUL- MONARY VEINS INTO THE SUPRA. CARDIAC SYSTEMIC VEINS PATHOLOGY 1. All pulinonary veins enter into either the persistent left superior vena cava, leſt innominate vein, coronary sinus, right atrium, right superior vena cava, portal vein or ductus venosus 2. Associated cominunications between the cardiac chambers may be present. HEMODYNAMICS The right atrium receives not only the venous blood, but also all the oxygenated blood returning from the lungs. Unless there are associated lesions present, death takes place upon closure of the foramen ovale. 20

·..
.
89%
98%
93%
Lyhen
***
.
ese
***
.
west
d
www
www.
tom
78%
Glene Jon
ANOMALOUS ENTRANCE OF ALL PULMONARY VEINS
(Sec Keg on page
2)

·22 l'enous Angiocardiography and Congenital Malformations Salient Angiographic Features 1. The pathognomonic sign is the early visualization of a markedly widened superior vena cava (Fig. 3.a) and its tributaries, followed first by the disappearance of the contrast substance (Fig. 3-b), and later by the reopacification of the above men. tioned wide vessels (Fig. 3-c). Thuis, of course, is only pathogno. monic if there is no delayed reinjection of the contrast substance. L.IV. S.V.C. Figme 3-a. Anomalous entrance of all Pulmonary l'ems. 1-P view at second. Marked dilatation of the right superior vena cava and of the left innominate vein. L. Subc. V.left subclavian vein; LIV-left innominate vein; SVC-superior vena casa.

·23
Drainage of Pulmonary l'eins into Systeinic l'eins
AO.
P.A.
---
Jugure 38 S-Pyrex of same angiocardiogram at to seconds Disap
xarance of the contrast substance froin tlie superior cna cara and
orcification of the right atrium, figlit sentricle and of the pulmonary
arteries 10 aorta. P.l-pulmonary artery.

·21 l'enous Angiocardiography and Congenital Afalformations LIV. An P. v. S.V.C. Figure 3.6. A-P view of same angiocardiogram at three seconds. Rcap. pearance of the radio opaque substance into the left innominate vein and into the superior vena cava without reopacification of the left subclavian rein, thus excluding a delayed re-injection of the contrast substance. The presence of such reopacification is a pathognomonic sign of anomalous entrance of pulmonary veins into the great systemic Teins. An. PV-anomalous pulmonary icins, Lil'left innominate sein, SVC-superior vena cava ·GROUP 2

·No text on this page.

·TRICUSPID ATRESIA PATHOLOGY Absence of the tricuspid valve 2. Patent foramen ovale or atrial septal deleti 3 typyplastic or absent righe ventricle 4 l'ulmonary stenosis or atresia 5. Ventricular septal defect and/or patent ductus may be prescnt o Uncommonly, tricuspid atresia may be associated with complete transposition of the great vessels. In these cases the pulmon. ary artery is usually wide, however, pulmonary stenosis may be present HICHODYNAMICS Venous blood from the right atrium must enter the left atrium and thence the left ventricle There is, therefore, always arterial ovigen unsaturation If there is no ventricular septal defect blood can enter the lung only through a patent ductus arteriosus or enlargect bronchual collateral sessels Unless there is an associated transposition of the great sessels with a large pulmonary artery originating from the left ventricle, pulmonary blood flow is always dininished

·No text on this page.

·TRICUSPID ATRESIA PATHOLOGY 1 Ibsence of the tricuspid valve ? Patent foramen ovale or atrial septal defect 3 Hypoplastic or absent right ventricle | Pulmonary stenosis or atresta ī Ventricular septal defect and/or patent ductus Inay be present o Uncommonly, tricuspid atresia may be associated with complete transposition of the great vessels. In these cases the pulmon. ary artery is usually wde, lionerer, pulmonary stenosis may be present TIEMODYNAMICS Venous blood from the right atrium must enter the left atriumi and thence the left sentricle There is, therelore, always arterial oxygen imsaruration. If there 15 no ventricular septal defect blood can enter the lung only through a patent ductus arteriosus or enlarged brunclual collateral sessels. Unless there is an associated transposition of the great vessels with a large pulmonary artery originating from the left sentricle, pulmonary blood flow is always sliminished

·28
Venous Angiocardiography and Congenital Aſalformations
78%
62.3%
77%
96%
Tu
76%
63.126
$75%
Clone Jone
654%
TRICUSPID ATRESIA
(See Key on page 2)

·29 Tricuspid Atresia Salient Angiographic Features I The best siew is the left anterior oblique because there is no superimposition of the atria in this view (Fig. 4-a). ? Immediate visualization of the shunt from right to lett airuum (Fig. 4-5). 3. The opacification of the left atrium is usually intense. A part of the diaplıragmatic surface of the heart in the A-P view xrsually occupied by the right ventricle may not be opacified in the carly dextro angiocardiograms This filling defect becomes opacified in the later films if a ventricular septal defect is present as the contrast substance enters the hypoplastic right sentricle from the left ventricle. The pulmonary arteries are usually small and the appendage of the left atrium is seen to occupy the left upper medial border of the heart (Fig. 10). ! There is an early dense opacification of a large left ventricle (Fig1 (1) 5. The large right atrium and the counter-cloch wise rotation of the scart due to the large left ventricle may simulate a destrocardia (Fig, l-e). 6 In cases of ricesped acresta that are associated wul complete transposition of the great sessels, the pulmonary artery originating from the large left tentricle is usually large and wide, and the gotta is fizpoplastic Honevet, pulmonary stenosis may be present euher in the usual 1)pe of tricuspid atresia or in those with trans- 101 of the great cssels

·l'enous angiocardiography and Congenital Malformations
L.A.
RA
T
Figure 4-1. Tricuspid Atresia. LAO vicw at one second. Note shunt
from right atrum into the left atrium and left ventricle. The location
of tle arial septal defect is kell visualized (arrow) R.l-right atram,
L-left atrium.

·31
Tricuspid Atresia
L.A
RA.
LV.
LVC
Figure 1-6 LAO new at one second. Another LAO view of a tricuspid
atresia with the contrast substance injected into the saphenous vein
There is simultaneous visualization right atrium, left atrium
and left ventricle. IVC-inferior vena cava, RA-right atrium; LA-left
triumi, LV leltcutricle

·32
l'enous angiocardiography and Congental Malformations
Ao.
LAA
R.A.
L.V.
figure 4-c. A-P view at one second Simultaneous visualization of the
right atrium, leſt atrium, large left ventricle, aorta and hypoplastic
pulmonary arteries. The area usually occupied by the right sentude
(arrow) is not opacified. Note visualızation of the appendage of the
left atrium. RA-riglit atrium, L\'-left ventricle, LM-leſt atrial ap.
pendage; AO-aorta.

·33
Tricuspid Attesta
Ao.
РА.
R.A.
LV
Figure 1. IPven at one second. Opachcation of the large right
atrium. left atrium, large left sentricle, hypoplastic pulmonary arteries
and aorta R.A right atrium, LV-left sentricle; P4 pulmonary artery,
10-sorta

·32
l'enous dugiocardiography and Congenital Malformations
Ao.
LAA
R.A.
L.V.
Figure 4-6 A-P view at one second Simultaneous visualization of the
right atrium, left atrium, large left ventricle, aorta and hypoplastic
pulmonary arteries. The area usually occupied by the right ventricle
(arrow) is not opacified. Note visualization of the appendage of the
left atrium. R 4-right atrium, L\' left ventricle, LAA-left atrial ap
pendage, AO-aorta

·93
Tricuspid Atresin
AO.
PA.
R.A.
LV
igure #d. All view at one second Opacification of the large right
alriui, leſt atrium, large leſt sentricle, hypoplastic pulmonary arteries
and aorta R I right at
m, Li-lelt ventricle, l-pulmonary artery,
O porta

·34
Venous Angiocardiography and Congenital Malformations
A.
PA.
R.P.A.
LV.
Figure 4-e. A-P view at 112 seconds. Visualizanon of the large riglie
atrium, left atrium and its appendage. The large aortic knob is seen
on the upper left cardiac border. The area usually occupied by the
right ventricle 15 not opacified and the pulmonary arteries are hypo.
plastic PA-pulmonary artery, RPA right pulmonary artery, Lilleft
atrial appendage; LV-left ventricle, A0-aorta,

·PULMONARY STENOSIS WITH NORMAL AORTIC ROOT AND ATRIAL COMMUNI. CATION AND RIGHT TO LEFT SHUNT PATHOLOGY 1. Varying degrees of valvular stenosis in rare cases amounting to atresia. Usually post-stenotic dilatation of the pulmonary artery. 2. Parent foramen ovale or atrial communication. HIEMODYNAMICS There is always right ventricular hypertension and low pulmonary arterial pressure Riglit ventricular hypertension results in an elevated right atrial pressure and a nght to left shunt at the atrial lesel

·36
l'enous Angiocardiography and Congenital Nfalformations
55.8%
87.4%
59%
96%
59%
59%
Gloria Jones
595%
PULMONARY VALVULAR STENOSIS
(See Rey on page ?)

·$7 Pulmonary Stenosis Salient Angiographic Features 1. The right anterior oblique is the best view for the demonistration of the pulmonary arteries and valies (Fig 5-a). 2 . In important diagnostic point is the very early opaciſication of the teſt atrium before the opacification of the aorta in the left anterior view (Fig 5-b). Since a portion of the contrast substance enters the right ventricle and the rest is shunted into the left atrium, the early opacification of the left atmun and of the left ventricle is not as intense as in patients with tricuspid atresia When tlic aorta is visualized it is not dextroposed (Fig 5-c). 3 In cases where there is a simultaneous visualization of the aorta and of the pulmonary artery, the presence of a post-stenotic filatation of the pulmonary artery and of a delinite enlargement of the transverse diameter of the heart usually exclude tecralogy of Fallot or pentalogy. } l'ost-stenotic dilatation of the main pulmonary artery is al. 1nost always present 3 The characteristic sign is the visualization of the jet-like formation of the contrast substance ihrough the dome-shaped sieratic salie Walt our techinue thus finding was not often demonstrated (1 13 5-d) 6 l'rolonged opacification of the pulmonary artery with a well opuſiedl aorta (Fig. 5.e).

·38 Venous Angiocardiography and Congenital Malformations PA. I. RA RV Figure 5-a Pulmonary Stenosis with Normal dortic Root you Atrial Communication. RAO view at two seconds. Opacification of the right atrium, right ventricular inflow tract, the infundibulum and of the post-stenotic dilatation of the pulmonary artery. The right border of the left atrium can be visualized. RA-right atrium; RV-right ventricle, I - Infundibulum; PA-pulmonary artery; LA-left atrium.

·39
Pulmonary Stenosis
LA
RA
legure 4 0. Ll0 view at one second Case of severe salvular pulmonary
stenosis with normal aortic root and atrial communication. Simul.
taneous opacification of large right suum, right venucle and left
atrium lote shunt from tight into leſt attium (arrow) RA right
num, L Heft atrium

·40
l'enous Angiocardiography and Congenital Malformations
Аа
L.A
Figure 5-c. LAO view of same case at two seconds Visualızation of
right atrium, right ventricle, leſt atruum, left ventricle, hypoplastic
pulmonary arteries and aorta Ilie aorta is not les troposed. I \-leſt
atrium; LV-left ventricle, AO-aorta

·41 Pulmonary Stenosis RA RV Figure 5 d RAO vien at 142 seconds Val ular stenosis. Opacification of the superior vena cava, right atrium, right ventricle and infundibulum Note the location of the tricuspid salve (arrow), the dome shaped area of the pulmonary sales and the jet-like propulsion of the contrast subscance into the post-stenotically dilated pulmonary artery Ringlit atrium, Rl-right sentricle, l-infundibulum, J.-jer ·42 l'enous Angiocardiography and Congenital Malformations Ao. PA. 1. R.V. Figure 5.e. RAO vien at 212 seconds Same case as Figure 5-a. Opacification of right atrium, right ventricle, post-stenotic dilatation of the pulmonary artery and aorta RV-right sentricle, I -infundibulum, P.l. pulinonary artery; 10-aorta.

·PERSISTENT TRUNCUS ARTERIOSUS L'ATTIOLOGY 1. Two tys are seen. Type 1. Common vessel arises from right and Icít ventricles. One or two large pulmonary arteries originate from the ascending por. Lion of the common vessci. Type 11. One or to lypoplastie pulmonary arteries or no pul. monary aricrics arise from the common trunk, 2. A higli ventricular septal dclect is present in both types. ICMODYNAMICS 7) I. Mivel venous anıl arterial byloxael frelski ventricles emers thic (10n Iersel The pressure 11 ft) venircles is alsout qual. 'l he pulmonary circun receives it blood supply chircilly from the common sessel under high pressure through the pulmo- Hary arteries Periplucral arteriat ovygen 1117saturation is always fileseni, bu because of the large volume blood entering the lungs, this cyanosis may not be crident clonally Typer il Tric licizelystammes are the sainc as un Type 1. cxcepri Hot muce it less bloul enters the lung under dpricase pressure, either from the small pulmonary arteries from bronchial collateral sessels (.) anosis is inteise Type 1 With cither one or two large pulmonary arteries coming «f? lle common trunk Typ ? With citlier one or two small pulmonary aricies coming off llie OnMON prunk. Rarely, tlıcre may loc no pulmonary arteries HA11. Tie angiocardiographic findings are entirely different in these inotypes 13 ·42 l'enous Angiocardiography and Congenital Malformations AO. PA 1. R.V. Figure 5-e RAO view at 21/2 seconds Same case as rigme 5-a. Opacification of siglittruum, right ventricle, post-stenotic dilatation of the pulmonary artery and aorta RV-right sentrule, I.infundibulum, P.1. pulmonary artery, 10-aorta ·PERSISTENT TRUYCUS ARTERIOSUS PITHOLOCY 1. Tortypes are ucen. Tot 1. Common sensel arises from right and left sencrirler. One er rularze pulmonary arteries originate from the asrending presince the common sensei. Type II. One or two hopsplayt.. pulmonary arteries or rto prilcrary arteries arise from the common trunk. 2. hinh sentricular epal defert is present in Ench coper. TIENODEVAMICS T; - I. Mivel senous and arterial blood from oth ventricle enriets the common reser! The presente in boch sentricles is a fret al The pulminaro circuit rerentes icy blood supplo srecilo fimicormen vessel under high pressure throach the fulmin Filmy arteriet. Peripheral arteriat neuten ursaiutaron e ala36 Fresne, tue kerause if the latte solume of blood enici, che 1.**29.15.1 carrers may not be eden clinirallo 756-11 The hersenamus are the same at m Toupe I enrept auh less for enters the lunz ander verteder! pressure. cm from the small pulmonary arterier or from. Lrrinchmal anal- Lepensels. COCAITL incense x 1 1:% ether one (no larze pulmonary arteries stint of pecommon runk TYPY Vi ber are le can stall pulmonary arteria cominc of lice common 19.n. Rarela there may be no pulmonary arteries F***. Ite armatimraphic findings are enurels different in +3 ·42 Venous Angiocardiography and Congenital Malformations Ao. PA. R.V. Figure 5.e. RAO view at 242 seconds Same case as Figure 5-a Opacification of right atrium, right ventricle, post-stenotic dilatation of the pulmonary artery and aorta. RV-right ventricle, I infundibulum, PApulmonary artery, AO-aorta.

·PERSISTENT TRUNCUS ARTERIOSUS PATHOLOGY 1. Two types are seen. Type I. Common vessel arises from right and left ventricles, One or two large pulsuonary arteries originate from the ascending portion of the common sessel, Type II. One or to liypoplastic pulmonary arteries or no pulmonary arteries arise from the common trunk. ? A high ventricular septal defect is present in both types. HEMODYNAMICS Typr I. Misc:1 venous and arterial blood from both ventricles enters the common sessel The pressure in both ventricles is about cual. The pulmonary circuit receives its blood supply directly from the common vessel under fugh pressure through the pulmomary arteries Peripheral arterial oxygen unsaturation 35 always present, but because of the large volume of blood entering the lungs, this cyanosis may not be evident clinically Type II The liemoxynamics are the same as in Type 1, except time much less blood enters the lung under decreased pressure, cifer from the small pulmonary arteries or from bronchual collateralicssels Clanosis is intense T PE 1 il'ith either one or tno large pulmonary arteries coming off the cottimon trunk Typ ? With either me or iso small pulmonary arteries coming oft the comun trunk Rarely, there may be no pulmonary arteries present. The angixardiographic findings are entirely different in these tho types 13

·S5620
44
l'enous Angiocardiography and Congenital Malformations
87%
53.4%
ber
96%
594%
Gloria Jones
TRUNCUS COMMUNIS-Type !
(See Key on page 2)

·45
Persistent Truncus Arteriosus
48%
96%
ARA
56%
Glami. Jang
TRUNCUS COMMUNIS—TYPE 2

·16 l'enous Angiocardiography and Congenital Malformations Salient Angiographic Features Type 1 1. In the A-P view there is usually a concavity in the pulmonary arca because the pulmonary arteries originate from the trunk and, therefore, are in a higher position than norinal. In addition, one of thic pulmonary arterics may be absent (Fig. 6-a). 2. The origin of the pulmonary arteries, as well as the oser: riding of the truncus arteriosus, can be best demonstrated in the left anterior oblique view (Fig. 6-b). 3 The pulmonary vessels are large and well opacified when prosent.

·Persistent Truncus Arteriosus Tr PA RA RV Figure 0-0 Persistent Truncus lrteriosus .1- view at two seconds l'usualuation of right atrum, large right ventricle, 1ery wide common irish and large left pulmonary artery The concavity in the region of the pulmonar unters-a rather characteristic sign in the malforma- 11011-15 seen to be due to the higlier origin of the pulmonary 3rch from the coinmon trunk and not from the outbou tract of the right senerule R 1-right atrium, RV right ventricle, Tr.truncus com. munis, Pi pulmonary artery.

·46 l'enous Angiocardiography and Congenital Malformations Salient Angiographic Features TYPE 1 1. In the A-P view there is usually a concavity in the pulmonary area because tlie pulmonary arteries originate from the trunk and, therefore, are in a higher position than normal. In addition, one of thie pulmonary arteries may be absent (Fig. 6-a). 2. The origin of the pulmonary arteries, as well as the overriding of the truncus arteriosus, can be best demonstrated in the left anterior oblique view (Fig. 6-b). 3. The pulmonary vessels are large and well opacified when prescnt.

·Persistent Trunctis Arteriosus 49 Tipe ? 1. The difference between this type and type one is the finding of small pulmonary arteries and diminished opacification of the peripheral pulmonary vessels (Fig 6-c). 2. This diagnosis can only be made if the small pulmonary arteries are seen to emerge from the truncus arteriosus. Usually this type cannot be distinguished from an extreme tetralogy of Fallot. PA RA AV I gue6e XP +1es •• common trunk and a energing from the 11. slight atrium, RV-right ventricle, PApulmonin arter, it-truncus communis.

·48 l'enous Angiocardiography and Congenital Malformations Tr P.A. RV Figure 6-0 LAO view at two seconds. Note the opacification of the right atrium, right ventricle and overriding wide common trunk. The pulmonary arteries are seen to originate from the common rrunh. RV-right ventricle; PA-pulmonary artery, Tr.-truncus communs.

·Witral Atresia
51
90%
67%
91%
N
96%
71%
Ch. Jan.
MITRAL ATRESIA
(See kry on page 2)

·MITRAL ATRESIA WITH VENTRICULAR SEPTAL DEFECT PATHOLOGY 1. Absence of mitral valve 2. Patent ductus arteriosus 3. This entity is always associated with the presence of one or more of some of the following additional defects* Atrial septal communication, ventricular septal defect, hypoplasia or atresia of the ascending aorta and of the aortic arclı, single ventricle or lıypoplasia of the left ventricle and anomalous return of the pulmonary veins. HEMODYNAMICS There is always arterial oxygen unsaturation. The oxygenated blood from the left atrium is shunted through an atrial communication into an enlarged right atrium, right ventricle, pul. monary artery and patent ductus arteriosus into the aortic arch and descending aorta Unless there are associated lesions in thic form of a ventricular septal defect or anomalous entrance of pul monary veins into the systemic ieins or right atrum, death cales place in early infancy. 50

·51
Mitral Atresia
90%
67%
91%
96%
90%
71%
MITRAL ATRESIA
(Sce Koy on page 2)

·MITRAL ATRESIA WITH VENTRICULAR SEPTAL DEFECT PATHOLOGY 1. Abschce of mitral valve 2. Patent ductus arteriosiis 3. This entity is always associated with the presence of one or more of some of the following additional defects. Atrial septal communication, ventricular septal defect, hypoplasia or atresia of the ascending aorta and of the aortic arch, single ventricle or hypoplasia of the left ventricle and anomalous return of tlie pulmonary veins. JIEMODYNAMICS There is always arterial oxygen unsaturation. The oxygenated blood from the leſt atrium is slunted through an atrial com. munication into an enlarged right atrium, righit ventricle, pul monary artery and patent ductus arteriosus into the aortic arch and descending aorta. Unless there are associated lesions in the form of a ventricular septal defect or anomalous cntrance of pul. monary veins into the systemic veins or right atrium, death tahes place in early infancy 50

·51
Nitral Atresia
90%
67%
91%
96%
C. Jose
71%
MITRAL ATRESIA
fee Key
on
page ?)

·52 Venous Angiocardiography and Congenital Malformations Salient Angiographic Features 1. The left anterior oblique view is the best view and, so far as we know, angiocardiography is the only laboratory test to help establish the diagnosis (Fig. 7-a). 2. The most important angiocardiographic finding is the opaci. fication of a hypoplastic left sidled cliamber in the presence of a large right atrium, right ventricle and pulmonary arteries (Fig 7.b). 3. Patent ductus may be visualized and the descending aorta may be wider and opacified to a greater degree than the provinal aorta.

·Mitral Atresia Ao , PA RL Higure 7 Mitial stresa LAO vies at two seconds Simultaneous walivation of the right atrium, right sentricle, large pulmonary arters and porta. Note the rudimentary left sided, posteriorly located churner huch communicates with the right ventricle through a Sentricular septal delect. 10 aorta, PA-pulmonary artery, RLV-rudimentar lelt ventricle ·54 l'enous Angiocardiography and Congenital Malformations Ао PA RA RV Figure 7.b. P- I view at one second Simultaneous visualization of the right atruum, right ventricle, pulmonary artery, rudimentary left sentricle and aorta. RA-right atrium, Rl-right sentricle, P.1.pulmonary artery; RLV-rudimentary left sentricle, AO-aorta ·GROUP 3

·No text on this page.

·TAUSSIG-BING ANOMALY PATHOLOGY 1. Transposition of the aorta. 2 levoposition of the pulmonary artery, i c., the pulmonary artery overrides both ventricles. $ Ventricular septal defect of the membranous and upper nuus. cular portion of the ventricular septum. HEMODYNAMICS Venous blood enters the aorta from the right ventricle. Venous blood from the right ventricle and arterialized blood from the luft ventricle enter the pulmonary circulation through the overruling pulmonary artery, Pressures in both ventricles and both great Wessels are approximately equal. Patency of the ductus arteriosus and the size of the ventricular defect, as well as the pulmonary resistance, determine the amount of blood that is stunteel from the systemic into the pulmonary circuit and vice CTS

·58
l'enons Angiocardiography and Congenital Malformations
433%
40.1%
84%
96%
40%
52
Cloria Jong
TAUSSIG-BING HEART
(Soe kry on pinge ?)

·Tausig-Bing Anomaly 59 Salient Angiographic Features 1. Simultaneous visualization of an anteriorly transposed aorta and levoposed large pulmonary artery, best seen in the oblique siens (Fig. 8 3). 2. The branches of the pulmonary arteries are usually well opacified. Aa P.A. RA. RY. Irgue & Taussig-Bing lieart R 10 view at one second Simultaneous visualization of the transposed aorta and of the les opposed large pul. Monors uiters RI right atrium, RV-right Ventricle, 10 aorta, PApulmon, arter ·EISENMENGER COMPLEX PATHOLOGY Type 1. The "anatomic" Eisenmenger-the rare type. 1 Functional dextroposition of the aorta is present from birth on. 2 Ventricular septal defect of the membranous and upper muscular portion. 3. Large pulmonary artery. Type II. The common type. 1. Large ventricular septal defect in the membranous portion of the septum. Riglit ventricular hypertrophy. Actual dextroposition of the aorta may be difficult to demonstrate even at autopsy. 2. Large pulmonary arteries. HEMODYNAMICS Type 1. There is arterial oxygen xinsaturation even though clinical cyanosis may not be evident, particularly early in life. The amount of venous blood shunted into the aorta depends upon the degrec of functional clestroposition of the aorta and the height of the pulmonary resistance Type II There is usually no arterial oxygen unsaturation for a number of years At first the shunt is from the left ventricle into the right one. For a number of years there is a gradual and progressive rise in the pulmonary resistance resulting in some of the blood from the right ventricle shunting into the aorta. Persistent cyanosis at rest is only present when the pulmonary resistance becomes higher than the systemic resistance. The degree of cyanosis depends upon the amount of blood shunted into the aorta 60

·.
.
61
Ersenmenger Compler
80%
659
739%
NU
N.
RN
*
.
79
wewe
667%
from Jones
EISENMENGER COMPLEX
See kry on prage ?)

·EISENMENGER COMPLEX PATHOLOGY Type 1. The "anatomic” Eisenmenger-the rare type. 1. Functional dextroposition of the aorta is present from birth on. 2. Ventricular septal defect of the inembranous and upper muscular portion. 3. Large pulmonary artery. Type II. Tlie common type. 1. Large ventricular septal defect in the membranous portion of thic scptum. Right ventricular liypertrophy Ictual dextroposi. tion of the aorta may be difficult to clemonstrate cien at autopsy 2 Large pulmonary arteries. HENODYNAMICS Type I. There is arterial oxygen unsaturation cien though clinical cyanusis may not be evident, particularly carly in lifc. Tlie amount of venous blood shunted into the aorta depends upon the degree of functional dextroposition of the aorta and the height of the pulmonary resistance. Type II. There is usually no arterial osyge unsaturation for a number of years At first the shunt is from the left ventricle into the right one. For a number of years there is a gradual and prog. ressive rise in the pulmonary resistance resulting in some of the blood from the right ventricle shunting into the aorta l'ersistent cyanosis at rest is only present then the pulmonary resistance be comes higher than the systemic resistance The degree of cyanosas depends upon the amolmt of blood shunted into the aorta

·61
Eisenmenger Complex
80%
6595
73.9%
.
677
---
667%
Storie JOACI
EISENMENGER COMPLEX
See Argon page 2)

·62 l'enons Angiocardiography and Congenital Malformations
Salient Angiographic Features
1. Simultaneous visualization of a large pulmonary artery and
aorta (Fig. 9-a).
2. The opacification of the pulmonary artery is usually gicater
than that of the aorta,
3. We divide the Eisenmenger's complex into tlo types
Ao.
PA.
Figure 9-a Eisenmenger's Compler 1.p view at 312 seconds Simul.
taneous visualization of large pulmonary arteries and of the aorta PA.
pulinonary artery, AO-aorta

·TIPE I
Inatomic type with definite descroposition of the aorta from
buth on (Fig 9-b)
Ture - The Common type
Desclopment of the Eisenmenger syndrome in later life from a
farge sentricular septal defect This latter type cannot be diagnosed
angiocardiographically before appearance of cyanosis, and the
demonstration of a sunultaneous visualization of thic dextroposed
aosta and of the large pulmonary arteries
RPA
LA
Ir vien alla seconds Simultaneous usualization of
91 theel enlarge pulmonary arteries and of a small aorta ote the
1914 Lital dilstation of the right pulmonary artery RPt-right pul
manat artens Ll leit atrium, AD-aorta

·PENTALOGY PATHOLOGY 1. Tetralogy of Fallot and an atrial communication in the form of a patent foramen ovale or an atrial septal defect. HEMODYNAMICS If there is no evidence of right heart failure and the diastolic pressure in the right ventricle is within normal limits, the liemo. dynamics arc the same as in a tetralogy of Fallot. In the presence. however, of an atrial septal defect and normal diastolic pressure in the ventricle, the shunt will be from the left atriun into the right atrium If the diastolic pressure in the right ventricle is elevated, there will be a shunt from right atrium to the left atrium, irrespective of whether there is a patent foramen ovalc or an atrial septal defect.

·上 一曲 一一 一一 。 : 重量 第一步 : 是 的 。 ” 鲁 中 的 。 本书 是 , 一 是一种 重 。 是 一种 了 。 台南 一 康 是 , 一 中学 者 。 一 量 是目前 看 上 一一 重生一世 一 者。 Pentalogy 15% 67% “是。” 由中 上一人 年,是一 1 三 中新建 , 是,事 。 :::: “ 。 | ET - 非 1。 一。 其中一种,一种非非 一中学 上学的重特性。 是的,,, 是一种非 一生。 1 - 青青 第一 一一 是,在中国 "", -- --- 一。 6% 神 “是。” 件事, 「, 在一 全 学学生 # 一事, 青年学情 中島 “ ·· 。 种特 号 费者销售清单事 物特輯 。 青春。 得 单鲁鲁 社會的 “是。” 。 有 一 的排量情。 村中学时时 。 一種 制作时的 請教 一一一 重量 “是。” 有的。 指导者。 - 特 「是 635% Ella Jone4 PENTALOGY (TETRALOGY AND ATRIAL COMMUNICATION) (See kes on page 2) ·66 Venous Angiocardiography and Congenital Nlalformations Salient Angiographic Features The presence of a right-to-left shunt at the atrial level makes the angiocardiographic diagnosis very difficult. 1. The left anterior oblique view is the best. 2. Early and, at times, simultaneous risualization of the right atriun, left atrium, right ventricle, pulmonary arteries and aorta (Fig. 10-a). 3. The left atrium is usually not as deusely opacified as in tricuspid atresia. The aorta overrides both ventricles-a point of greatest importance in the differential diagnosis from pulmonary stenosis with interatrial communication and a nornal aortic root (Fig. 10-a).

·67
Pentalogy
LP.A
L.A.
Tue 10 Pestalo. I loves all one second Simultaneous visual.
Wall of the well op.shed rglit atrium, right ventricle, pulmonary
ATEGIK and of the famils pacifies left atrium and dextroposed aorta
Il tuum IP ikit pulmonary artery, 10 aorta

·DISCUSSION Table 1 consists of the entities in which the diagnosis can almost always be made by proper interpretation of a technically good angiocardiogram; tetralogy of Fallot, transposition of the great vessels and anomalous drainage of the pulmonary veins into the systemic veins. TETRALOGY OF TALLOT Ninety-tlıree patients were studied. Out of 127 angiocardiograms reviewed the diagnosis was correctly made in 96 (75,5%) The diagnosis was highly suggestive, but not made with certainty, and the possibility of another diagnosis was considered in 7 (5.5%). Only in 6 (4.7%) of our angiocardiograms was the diagnosis incorrectly made. Of the total number of angiocardiograms, 13 or 10.1% were technically poor and visualization of the various chambers of the heart and the great vessels was impossible, and 5 angiocardiograms (3.9%) were missing from our files The technically poor angiocardiograms were encountered in the carlier years of this study. It is thus clear that the percentage of the correct angrocardiographic diagnoses could have been about 90% ir we add the 10.4", of the technically poor and 3 90, of the missing angiocardiograms to the total correctly diagnosed Of the 13 patients studied the diagnosis of retralogy of Fallot was confirmed by catheterization in fifty-six patients, by surgery in fifty patients and by autopsy in tienty-thice cases TRANSPOSITION OF THE GREAT VESSELS Thirty-five patients were studied Out of 18 angiocardiograms, «10 or 83.3 were correctly diagnosed, and only in the cases (4 %) was the diagnosis not definite. This group represents, thcretorc, cien a higher percentage ot correct angiocardiograpine diagnoses than those of tetralogy of Fallot in the 2 cases where the diagnosis was not definite the angiocardiograin was taken in the AP view Had they been taken in the left anterior oblique vieis -1 dich ne 69 ·I ABI C 1 TXTITIS IN WITH TIL DIAGNIKI (WILWO17 ILI 1) S BF MADE # PROPER INTERPRETATION OF A 1 CHINK AT GOOD INGIOCARDIOGRAM Diagnosis Confirmed Pricentage of Ingrecardiographic Dragnosts Post- Total Wumber .Ingioimproper cardio- Technique grams not Irarlable Cathe intindion Surgery forlem Exam Thighly Sugers- Fitablished Vamr of Ints Vet i stablished ingrocardiogramy 23 50 hients 56 23 3 9%- 2 4 7% 7 12 13 10 4% 2 4 2% 127 93 5 5% 2 4 20 8 3% 4 35 48 96 75 5% 40 83 3% 5 83 33% ciralogy of I allot Transposition of the Great Vresels Inomalous Drainage of All l'ulnonary Veins into Systemic Veins 67% 5 6 ·DISCUSSION Tabic I consists of the entities in which the diagnosis can almost always be made by proper interpretation of a technically good angiocardiogram; tetralogy of Fallot, transposition of the great vessels and anomalous drainage of the pulmonary veins into the systemic veins. TETRALOGY OF TALLOT Ninety-three patients were studied. Out of 127 angiocarliograms reviewed she diagnosis was correctly made in 96 (75.5%). The diagnosis was highly suggestive, but not made with certainty, and the possibility of another diagnosis was considered in 7 (5.5%). Only in ((4.7%) of our angiocardiogramis was the diagnosis incorrectly made. Of the total number of angiocardiograms, 13 or 10.4% were technically poor and visualization of the various chambers of the heart and the great vessels was impossible, and 5 angiocardiograms (3.990) were missing from our files. The technically poor angiocardiograms were encountered in the earlier years of this study It is tirus clear that the percentage of thic correct angiocardiographic diagnoses could have been abom 90% if we add the 1046 of the technically poor and 390, of the missing angiocardio ms to the total correctly dia osc Of the 93 pacients studied tlie diagnosis of cctralogy of Fallot was confirmed by catheterization in fifty-si\ patients, loy sulgery in fiſty patients and by autopsy 111 tsenty-three cases. TRANSPOSITION OF THE GREAT VESSCLS Thirty-five patients were studied. Out of 18 angiocardiograms, 10 or 83.3% were correcily ciagnosed, and only in two cases (4.2") was the diagnosis not definite This group represents, therefore, even a lugher percentage ol correct angrocardiographic diagnoses than those of tetralogy of Fallot In the 2 cases there the diagnosis was not definic the anglocardiogram was taken in the .1- ven. Had they been taken in the lelt anterior oblique view –which uc 68 ·TVBI.I. 2 Is 1 H THE DUAS (4) CHILLI BE VADE BY PROPER INTERPRETATION OF 4 1 VIL ALEX GOOD IGEKARDIOCRUM Dragnosu Corfumed Pourray of lagloresta grafiu Dragnosis Info los IVT Prch cardio garu nol Italable Caske- Iratron Swgry ros! Voreom Erta Vot Estab gu High!) Sueges و ر ء ج ۱ Estab Issford Whed Ps tarafs 12 cordion 3109 5 1 48% 21 16 61% 1916 20 8 * 5196 7 1. Trouped Strea 2 Puomonary Stenosis with Normal lortke Koot and Itrial Communication and Right to Left Shunt 3 l'existent Trincus Irteriosus 39 36% 3 2 69 350 1 956 20 6 146 21 46 4 66 7676 1 3676 2 33 36% 29 1 16 3 6 4 Vatral tresla with Ventikular Septal Defect ·70 l'enous Angiocardiography and Congenital Malformations consider the best one for the demonstration of the transposition of the great vessels-the correct diagnosis could have been most probably made. The number of the technically poor and missing angiocardiograms was 2 (42%) and 4 (8.3%) respectively. The diagnosis in the case of these 35 was confirmed in twclie patients by catheterization, in seven patients by surgery and in twenty. three patients by post mortem cxamination. ANOMALOUS DRAINAGE OF ALL PULMONARY VEINS INTO SUPRÁCARDIAC SYSTEMIC VEINS Six angiocardiograms were taken on five patients. The angio. carliographic diagnosis could have been most probably as high as 100% were it not for one technically poor angiocardiogram. We siish to emphasize that we are considering here only the anomalous drainage of all pulmonary veins into the systemic veins, and not those cases where the anomalous veins drain directly into the right atrium or into the coronary sinus. In the latter, the perceiitage of correct angiocardiographic diagnoses is considerably lower. The diagnosis in this group of five patients was confirmed in four patients by catheterization, in one patient by surgery and in three patients by post-mortem examination. Table 2 contains four entities in which the diagnosis can usually be made by proper interpretation of a technically good angio caudiogram; tricuspid atiesia, pulmonary stenosis with normal aortic root and atrial communication, persistent truncus arteriosus and mitral atresia. TRICUSPID ATRESLA Twenty-one angrocardiograms taken on 11 patients have been studied The diagnosis was made in 16 (76.2%) and was lughly suggestive in 1 (19.1%). One angiocardiogram (19%) was missing. In 5 instances the diagnosis was confirmed luy catheterization, in 12 by surgery and in 1 by post mortem examination, ·Discussion 73 Tal»lc 3 contains entities in which the diagnosis can usually not be made by proper interpretation of a technically good angio- Cardbogram: 'Taussig-Bing heart, Eisenmenger's complex and pentalogy TAUSSIC-BING ANOMALY Seventeen angiocardiograms done on eight patients were stud. sed The diagnosis was correct in 8 (47.2976), and in 5 (29.4%) thie diagnosis was lughly suggestie. T10 (117%) of the angiocardiograins are imissing The diagnosis was confirmed by catheteriration in six out of seven patients Because we believe that in the majority of cases it is impossible to differentiate with certainty angiocardiograplucally a Taussig.Ring heart from coniplete transposition of the great vessels with a large ventricular septal defect, and even all autopsy tlus differential diagnosis cannot always be made, we 114 luded in this group our patients with transposition of the great vessels wah large ventricular septal defects in which the pulmonary artery was visualized simultaneously with the transposed aorta CISEXMEXGER'S COMPLEX Out of the 21 anglocardiograms donc on sixteen patients 13 1719",) were currectly diagnosed The diagnosis was higlıly sug. gasten 7 (208"), and was not made in 3 (1250%) One (12%) Ingrexardregram was not used because of poor technique Diagnosta las been conlirmed in esglur patients by catheterization, m me patient by surgery and in esglut patients by post mortem 'Walton PENTALOGY 1 vele angrocardiograms were done on eight patients. The diagnosis was made in 7 (58 19:), nas lughly suggestise in 4 1934, 1 and was incorrectly made in 1 (839. The diagnosis was *onfumed in four of the patients by catheterizacion, ino by surers, and on four patients by post mortem examinations. Ihese three enthues belong to Group 3 because of the gencally lower fuentage of correctly diagnosed angiocardiograms, fughe peleculage of doutríul diagnosis and higher percentage of incorreta diagnosis ihan encountered in Group I and Group 2.

·72 l'enous Angiocardiography and Congenital Afalformations PULMONARY STENOSIS WITH NORMAL AORTIC ROOT AND ATRIAL COMMUNICATION AND RIGHT TO LEIT SHUNT Out of 39 angiocardiograms taken on thirty patients 27 (69.3%) were diagnosed. In 7 (17.9%) the diagnosis was highly suggestive and in 3 (7.6%) diagnosis could not be established. Only tho angiocardiograms (5.16) rere technically poor. The diagnosis was confirmed by catheterization in twenty patients, by surgery in tourteen patients and by post mortem examination in four pa tients. The doubtful diagnoses in this entity were due to the fact that the angrocardiograms were taken in the A-P view. Had tlıcse been tahen in the oblique view where the shunt from right-to-leht atrium can be best demonstrated, the diagnosis could have been made in a higher percentage. The need of a second angiocardiogram taken in the oblique vex is trus demonstrated PERSISTENT TRUNCUS ARTERIOSUS Sixteen patients were studied, including the two types of persistent truncus arteriosus Out of the 28 angiocardiograms examined, 20 (714) rere diagnosed connectis, and in 6 (211%) the diagnosis was highly suggestive. I correct diagnosis was not estabı. lished in only one patient (3 090) The diagnosis was conhrmerl by catheterization in seien patients, by surgery I four patients, and by post mortem examination in eight patients MITRAL ATRESIA WITH VENTRICULAR SEPTAL DETECT Three patients and 6 angurardiograms were studied. The lugnosis was made in four angrocardiograms (66.60.). The diag. nosis was confirmed in three pacients by surgery, one by callieterization and in four patients by post mortem examination. The abovc fom entities belong to Group 2 instcad of Group 1, because of the generally loner percentage of correc diagnosis and greater percentage of lughly suggestive diagnosis ·COMMENTS Our own interpretations of our angiocardiograms have been constantly changing in the light of a correlative clinical, roentgenologic, electrocardiograpluic, catheterization, surgical and post mortem findings The improvement in our techniques, such as the use of rectal barbiturate sedation for our infants and younger chuldren, the very rapid mjection of the contrast substance, the improved radiography. have all been very important factors in obtaining satisfactory visualization of the various cardiac chambers and of the great vessels and of their tributaries The results obtained in this study are based on our experiences in the use of venous angiocarlingraphy We used a Faircluld camera and our films were taken at 1,49 second intervals at 1/60 second exposure time. The tahnique of venous angiocardiography is rather simple and is close.ussed fully in the standard works on angiocardiography by Drs Dotter and Steinberg, Dr. Castellanos and associates, and others. This technique (loes not require the use of very expensive clafenate apparatus, and one plıysician and usually one technician are all that is neccssary for this procedure Dr. Kjellberg and assexuales macle extensive use of selective angrocardiography by injeeting the contrast substance through a catheter as closely as possible into a particular area of the heart under investigation. This some group as well as Drs Wegelius and Lind, have utilized a spre sulla consciuted mac lune that enables them to tale twentyfour timpur scrond-iele films in two different planes at right angles to each other and at an exposure time as fast as 0 003 sec- 07770 I heil contributions are most valuable and the many details but ther's were able to obtain, such as the exact location and size ut the stenosis, the degree of the dextroposition of the aorta and 75 ·TABLE 3 ENTITIES IN WHICH THIC DIAGNOSIS CAN USUALLY NOT BE MADF DY PROPER INTERPRETATION OF A TECHNICALLY GOOD ANGIOCARDIOGRAY Total.lumber Percentage of Angiocardiographic Diagnosis Diagnosis Confirmed Jame of Ently Inglo- Ingio- Improper cardio- Technique grams nol Available Pa lients cardiograms Estab- Iushed Vot Established Highly Suggestire Cathe terization Surgery Post Vortem Eram. 1 Tausig-Bing Inomaly 8 17 5 29 4% 2 11 7% 6 2 Eisenmenger's Complexos 8 47 2% 13 54 2% 16 24 1 2 11 7% 2 8 3% 3 12 5% 8 8 20 8% 3. Pentalogy 4 2% 8 12 4 4 2 4 58 4% 33 3% 8 3% ·CONCLUSIONS A study of 121 angiocardiograins done on 283 patients led to the following conclusions regarding the diagnostic value of venous angiocardiography. 1) The diagnosis of tetralogy of Fallot, complete transposition of the great vessels and anomalous entrance of the pulmonary veins into the great systemic veins can ALMOST ALIWAYS be made froin a properly performed and properly interpreted angiocardiograin 2) The diagnosis of incuspid atresia, pulinonary stenosis with, normal aortic root and atrial communication and right to left sunt persistent truncus arteriosus and mitral atresia with venrucular septal defect can USUALLY be made from a properly interpreted angiocardiogram, * The diagnosis of Taussig-Bing anomaly, Eisenmenger's comples and pentalogy in which the diagnosis can USUALLY NOT BL. WIL by proper interpretation of a technically good angiocardiogram 1) 11) other forms of olie (yanotic types of congenital mal formations of the heart A LII'AHS REQUIRE ADDITIONAL studies before a definite diagnosis can be established. is the salient angiocardiographic findings in ten entities are presented 27 ·76 Venous Angiocardiography and Congenital Malformations of the pulmonary artery, the location and the size of the septal defects, cannot be duplicated by venous angiocardiography, and by the use of a machine that takes only two films per second at 1/60 second exposure time. On the other hand, selective angiocardiography is a much more complicated and time consuming procedure. A general anesthetic, cardiac catheterization and a competent team of workers are necessary for this procedure. From our limited experience with selective angiocardiography, we believe that this angiocardiographic procedure is attended with greater danger to the patient. In this series of 421 angiocardiograms no death occurred directly attributable to the procedure. We wish to state that while we believe that in tlic vast majority of all cases, the experienced clinician can establish a clinical diagnosis without angiocardiography and/or catheterisation, it is the experience gained from correlative studies of angiocardiography, catheterization and autopsy material combined with the history, physical, fluoroscopic, roentgenologic and electrocardiographic studies that inahe it possible for the clincian to establish these clinical diagnoses. So far as we know 110 previous attempt to establish the value of angiocardiography as a separate laboratory tool has been published previously. We purposely omitted any references to the clinical, roentgenologic, clectrocardiograpluc and cardiac catheterization ſindings in any of these entities Although our division of the cyanotic types of congenital mallormations of the heart into groups may appear to be an artificial one and although we realize that some authorities in thus field wall differ with is regarding thus classification, the results recorded in this manuscript are those based on our personal experiences No attempt was made by lis to minimize the percentages of the technically poor angiocardie) grams as well as the percentage of those angiocardiograms which offcrcd difficulties in arriving at a correct diagnosis ·REFERENCES I CASTILLANOS, A, PEREIRAS, R., AND GARCIA, O: la Ingiocardiographia Radiopaca Arch. Soc. Estad. Cim. Habana, September October, 1937, pp 9-10. 2 Koze, G, P, AND STEIYBFRG, I. Visualization of the Chambers of the Heart, The Pulmonary Circulation and the Great Blood Vessels in Man: A Practical Method Am. J. Roentgenol, +1 1-17, 1949 3 COOLFY, RN, Batissos, H T., AND HAVLOV, C R.: Angiocardiography in Congenital Heart Disease of Cyanotic Type Hith Pulmontc Stenosis or Atresia I Observations on the Tetralogy of Fallot and “Pseudotruncus Arteriosus." Radı alogy, 52 329, 1919 1 Colcy. R N SLOAY, R. D, HALOS, C. R., AVD Balinson, HT Ingiocardiography in Congenital Heart Disease of Cyanuric Type 11 Observations on Tricuspid Stenosis or litesia with Hypoplasia of the Right Ventricle. Radiology, 1818, 1930 3 KFT. ), I), AND MU's, J. D. Angiocardiography in Infants and Children Ver Teclinic. l'ediatrics, 6. 20-32, 1950. POTTER CT, A\D STEINBERG, I Angiocardiography. New Tork laul B Hocber, Inc., 1931. 7 scurt N (s. The Development of Angiocardiography and tortography Radiolog, 56 185, 1951. * Con RX, ASD SLOAN, R. D. Angiocardiography in Congenital Ilean Disease of Cyanouc Type Radiology, 58.481, 1972 {f\ J. 41 W'FGFIILS, C.: Ingiocardiographic Studies in (huldren idrances in Pediatrics, l'ol II, pp. 151-170, 1932. 10 (xsu, B., Wriss, !!, FFIL, E, H., DILLOY, R. F, FISHER, Dus I., AND VARIFYFELD, C. ]. Angiocardiography in Son ·78 l'enous iugiocardiography and Congenital Malformations TABLE 4 ENTITIES WHICH ILIR.11S REQUIRE ADDITIOVAL STUDIES BEFORE A DEFTVITE DIAGNOSIS CAN BE ESTADLISHED* Vame of Entu!) Patients ingrocardingrams 3 Congenital Mitra) Stenosis Tricuspid Stenosis Tricuspid Atresia with Transposition of the Great Vessels Tricuspid Atresa sith Persistent Truncus Arteriosus Single Ventricle Ebstein's Anomaly Lcvocardia Isolated Dextrocardia Pulmonary Stenosis with Ventricular Septal Defect Pulmonary Stenosis with Patent Ductus Arteriosus Cor Pulmonale Patent Ductus arteriosus with Reversal of the Shunt Primary Pulmonary Hypertension Anomalous Entrance of All Pulmonary Verns into Right Atrium Cyanosis of Non-Cardiac origin 5 8 5 6 8 8 3 3 4 4 6 3 5 1 3 8 3 1 3 4 3 3 1 19 Total 55 93 • The authors realue that everal of these entities particularly Parent Ductus Arteriosus noth Raiersal of the Shant, could properly be placed in 7 ablc 11 or 117.

·This book TIE SILIEYT POINTS AND THE VALUE OF VEXOUS ANGIOCARDIOGRAPHY IN THE DI IGNOSIS OF THE CYANOTIC TYPES or CONGENITAL MALFORMATIONS OF THE HEART By DEVJAN 11. GASUL, MD, Grosio I{Art, M.D., Roborr F. DIILOY, MI). and EGEERT 11. FELI, M.D terus set, printer and bound by George Banta Company, Inc of Menasher, Il'isconsin. The engravings were made by the Northwestern Engraving Company of Nenasha, Il'usconsin the page trim size is 6 X 9 inches. The type page 14 26 X 43 picas The type face is Linotype Baskermille, set 11 point on 13 point. The text paper is 70 lb. II hale Lustro Gloss Cuaniel The cover is Rouile LS Tellum, 91268-II'M BOKS THROLS FEYMBOL LOERSHUS 11 «H INOV IS OOKS carrjul attention is given to all details of manufacturing and design. It is the Publisher's deure to pusent books that are satisfactory as to their phy we al qualities angl nislc possibilities and approfit. alr for their particular use. THOMAS BOOKS will be frut in these laws of quality that assure a good name and good will.

·80 l'enous Angiocardiography and Congenital Malformations genital Heart Disease Correlated with Clinical and Autopsy Findings. AMA Am. ). Dis. Chil., 35: 404-443, 1953. 11. LowE, JAMES B.: The Angiocardiograin in Tetralogy of Fallot. Brit. Heart )., 15: 319, 1953. 12. WrGELIU, C., AND LIND, J.: The Role of the Exposure Rate in Angiocardiography. Acta Radiol., 19: 177, 1953. 13. HILARIO, J., LIND, J., AND I'[GELIUS, C.: Rapid Biplane Angiocardiology in Tetralogy of Fallot. Brit. Heart I., XIL: 109, 1951. 11. LIND, J., SPLACER, R., AND WEGELIUS, C.: The Diagnosis of Cardiac Shunts by Intravenous Angiocardiography, Brt. Heart J., XVI: 407, 1954. 15. KJCLI BERG, S. R., MAMNUCINER, E., Rudur, U., AND Jovsson, B.: Diagnosis of Congemtal Heart Disease Chicago, The Year Book Publishers, Inc., 1955. 10. ABRAMS, HERBERT L., AND KAILAN, HENRY S.: Angiocardiographic Interpretation in Congenital Heart Discast'. Springfield, Thomas, 1956 17. KAPLAN, HENRY S, AND ABRAMS, HERBERT L. Angiocardio. graphic Interpretation in Congenital Heart Discuse. Springfield, Thomas, 1956.

·This book THE SALIENT POINTS AND THE VALUE OF YESOUS ANGIOCARDIOGRAPHY IN THE DIAGXOSIS OF THE CYANOTIC TYPES OF CONGENITAL MALFORMATIONS OF THE HEART Ву BEVJA VIIS MI. GASUL, M.D., GrRHO HAIT, M.D., ROBERT F. DILLON, M.D. and EGBERT H. FELL, M.D. u'ns ser, printed and bound by George Banta Company, Inc of Menasha, Ilusconsin The engravings were made by the Northwestern Engraving Company of Alenasha, Niescons. The page trim size is 6 X 9 inches The type frage is 26 X 43 picas. The type face is Linotype Baskereille, set 11 point on 13 point. The cere dager is 70 10. Il lle Lustro Gloss Chamel The roury is Route LS J'ellum, 51268-11M BOKS SEMBOLO Illath THOMAS BOOKS careful attention is given to all drlarls of manufacturing and design It is the Publisher's desire to present books that are satisfactory as to their physical qualities and artistic possibilities and approprare for their particular ise THOMAS BOOKS will be Irue to those lau's of quality that assure a good name and good will.

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