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A Classification of congenital heart disease
Progress Notes in Cardiology Edited by EMAKUELGOLDBERGER, M.D., F.A.C.C. New York, New York
A Classification
of Congenital
Heart
Disease
A Physiologic Approach * KOBERT ROSE~TBLUM, bf.~. New York, New York
M
ALI’OI<~IATIONS of the Human Heart” tly Thomas R. Peacock, published in London in 1866, is credited with being the first comprehensive volume on congenital cardiac dcfects.’ Many since ha1.e contributed significantly to kmnvledge in this area, but it was Maude Al)bott‘s “Atlas of Congenital Cardiac Disease” that correlated the clinical and pathologic findings of congenital cardiac anomalies.2 Her influence was a powerful catalyst in accelerating \vork in this field. Most authors since Abbott have continued to classify congenital malformations of the heart This according to their anatomic defects. has many advantages, but the significant disadvantage is the difficulty of this approach in teaching. With clinical and physiologic modern diagnostic technics much information has been acquired about the physiologic effects on the heart of the various single or combined ‘I’he physical findings, abnormalities of lesions. the x-ray films or fluoroscopy and electrocardio,gram are the results of altered function of the heart due to the various malformations.RP5 The late Paul F\:ood clearly saw this change in clinical approach to cardiology, and his classification bcqan to deviate from the anatomic emphasis and stressed the physiologic importance of congenital anomalies of the heart.“,” In this paper a physiologic orientation has been employed to classify congenital malformaSuch grouping of congenital tions of the heart. heart disease has the disadvantage of not being useful in cataloguing the various diseases as in
an anatomic classification, because the same anatomic lesion may fall into two different categories depending on the influence of other factors, e.g., pulmonary resistance. Therefore, \zhat is presented is not a true classification in the usual definition, but rather a system which is an aid both in approaching the study of congenital heart disease and in understanding the clinical and physiologic features of the anomalies. This approach gives a clearer meaning to the clinical findings and laboratory studies in congenital malformations of the heart and No attempt is made to give the great vessels. differential diagnostic features or the laboratory data obtained in the different malformations nor are all congenital malformations listed; listed in this classification. These are well described in the textbooks and review articles on thesubject.“,i-‘2 In this classification congenital malformations of the heart ha1.e heen grouped into five physioeffects logic categories : four with abnormal on the heart and one without pathophysiologic Multiple complex defects are more alterations. clearly understood if approached physiologically, and although many would fall into two or more of the catcaories listed, their clinical and physiologic analyses are better evaluated under this system. Because of the many variables in a functional study, no attempt is made to list all described Some rare or theoretically possible lesions. lesions are listed to demonstrate better the application of this method. In any discussion
* From the Cardiology Service, Medical IXvision of Mont&ore 126
Hospital,
New York, N. Y.
THE: AMERICAN
JOURNAL
OF CARDIOLOGY
transposition. of corrected ventricle ih referred to and vl:ntriclc.
the not
physiologic the
anatomic
OR DECREASED PUUt0h.4~k BL,W,D FLO\~; KORMAL. RmsTmc~ IR'TFIE PULUONARY :++TERIoI..AR VAS~UL.~R RED
.UCIRMAL
SORZIAI. OR DECREASED PULMONARY BLOOD Fr.o\z-; HIGH RESISTANCE OF THE PULMONARY ARTERIOLAR \'ASCI:I.AR BED
ACYANOTI(: Increused Right Pulmonary Infundibular Stenosis of Congenital
Ventricular Work valvular stenosis* stenosis the peripheral pulmonary pulmonary insufficiency
Low Pulmonary Venous Presrure arteries
CY.L\NOTIC Increased I&ventricular Fl’ork Transposition of the great vessels with pulmanic stenosis Pulmonic stenosis and/or infundibular stenosis with IVSD Pulmonic stenosis and/or infundibular stenosis with persistent common atrioventricular canal Truncus arteriosus with hypoplastic pulmonary artery lncrvasrd
Right
Tetraloay Pulmonic
I~~mtrzcular
of Fallot stenosis
Work
with
Endocardial fibroelastosis \-on Gierke’q disease in\-olxing the myocardium (metabolic) Corrected transposition with malplaced tricuspid vaI\-e (mild) with insufficiency of the valves
(any lesion or Eisenmenger’s syndrome combination of lesions in which the pulmonary resistance is elevated to the degree that the net pulmonary flow is equal to or less than the systemic AL&) High Pulmonary I,7enousPressure Congenital mitral stenosis Car triatriatum (stenosis of common pulmonary vain) Congenital pulmonary venous stenosis INCREASED PULMON.~KY B~.om Fro\\ ACYANOTI:: Increased Right Ventricular Work
IXSD
(secundum
septal
tYPe Partial anomalous
type)
Common ventricle with rudimentary chamber and outflow area to pulmonary artery
LESKM INVOLVIK~ THE SYSTEMIC SIDE OF THE HE:\RT (LEFT VENTRICLE) WITHOUT INTRACARDIAC Smws Kf r2.5tanw !o Lrji I bnthilnr Ejectzon Aortic subvalvular stenosis Fit)1 01:s ring Muycular hypcrtrophy Aortic valvular stenosis Aortic supravalvular stenosis Coarctation of aorta with and without associated aortic valve involvement Otilrr LeAion. llffecting the Lejt Ventric.le ConSenital mitral insufficiency Aberrant origin of coronary vessels Idiopathic hypertrophy of the heart cardiomegaly )
Interatrial
defect
(L4SD)
pulmonary
secundum
venous
age Corrected transposition with IASD IASD-secundum type with plllmonic vular stenosis
drain-
val-
Increased Left Ventricular Work Patent ductus arteriosus (PDA) Ventricular septal defect (VSD) Corrected transposition with VSD Iru-reused Right and Left I ‘entricukw Niirk PDA or VSD with associated hyperkinetic pulmonary hypertension VSD with valbular or infundibular stenosis Persistent common atrio\.entricular canal, partial form’5 (IASD--ostium primum defect with cleft mitral valve) CYAKOTIC
(familial
Total anomalous puImonary venous drainage Transposition of the great vessels Taussig-Binq anomaly Truncus arteriosus Both vessels arising from right ventricle Common \xntricle
128
Progrrss
Notes in < hrdiolog~ 3.
Anomalies of the aortic arch and its /)ranches, e.g., v2tscular rings, aberrant origins of \ essels from the aorta IDextrocarclia Dcstrorotation of the heart Corrected transposition
A physiologic system has heen presented to facilitate the study of congenital malformation It is intended to of the heart and great vessels. aid in the clinical and physiologic understanding of the various anomalies of the heart. It is not meant to lx used as a catalogue of the diseases. Classifications in general are a method of current knowledge and serve, summarizing predominantly, as an orientation and a teaching Therefore, as new knowledge is obtained, aid. classifications should be changed in a more It is with this latter emphasis meanirqful way. that the material outlined is presented.
RUSHMEK, Ii. 1,‘. (Cardiovascular Dynamics. c,d. 2. Philadelphia anti I,onclon, 1961. W. R. Sanntlcrs. 4. RECK, W., SCIIRIK~, V., VOG~:LPO~I., :\., NI(I.LI:N, M. and SWANFOHI., A. Hemodynamic &ccts of amyl nitrite and phcnylr~phrinc on the normal human circulation and their relation to changes in cardiac murmurs. 4rn. J. Cardiol., 8: 341, 1961, 5. WOOD, I’. Disrasrs of the Heart and Circulation, cd. 2. Philadelphia, 1356. .J. 8. Lippincrltt Com6.
7.
8. 9.
10.
11. 12.
13.
I
should
son Young
like to thank
Dr. Louis
for their helpful
Leitcr
comments
and Dr. Denni-
and suggestions.
REFERENCES 1.
Introduction to facsimile edition, .4snow, M., Atlas of Congenital Heart Disrasr, 1954. American Heart Assxiation. Atlas of Congenital Heart Disease. 2. z\RROTT, M. facsimile rd.. 1954. American Heart Association. M.~RPI.E,
C.
14.
pany.
P. Eiscnmcnger’s syndrome or pulmonary hyprrtcnsion with revrrsed central shunt. &if. .ti. J., 2: 701,755,1958. ‘TAUSSIG, H. Congenital Malformations of the Heart, rd. 2. Cambridge, Mass., 1960. Amcrican Heart Association, Harvard Press. Pediatric Cardiology. NADAS, A. Philadelphia, 1957. W. B. Sallndcrs. KJELLBISKG. S. R., MANNIIEIM~~R, E., Rrlurre, ii. and JO~SSON, B. Diagnosis of Congenital Heart Diseaw, cd. 2. Chicago, 1959. Year Book Publishers. KEITH, J. D., Rowe, K. D., and VLAD, I’. Heart Disease in Infancy and Childhood. New York, 1958. Macmillan Company. S. E. Pathology of thr Heart, cd. 2. <;our.o, Springfield, III., 1960. Charles C Thomas. Congenital corrrctrd transSCHIEBLER, G. I>. vt al. position of the great vessels, a study of 33 cases. Pediatrics, 27 (Suppl.); Part II. 1961. KOSENBLC’M, K., MARK, H., ESCHER, I). I. W., STERN, W. %. and YOUNG, D. ‘The differentiation of pulmonic stenosis, ventricular srptal dcfcct with normal aortic root from tetralogy of Fallot. ilm. Heart .J., 46: 746, 1962. YOUNG, D. Critrria for surgery in persistent comAm. .J. Cardiol., 12: mon atrioventricular canal. WOOD,
80,1963. 15. WAKAT, C. S. and EDWARDS, .J. E. of pcrsistcnt
common
Pathologic study atrioventricular canal. Am.
Hmr/ .I.. 56: 779. 1958.
THE
AMERICAN
JOURNAI.
OF CARDIOLOGY
A Classification
of Congenital
Heart
Disease
A Physiologic Approach * KOBERT ROSE~TBLUM, bf.~. New York, New York
M
ALI’OI<~IATIONS of the Human Heart” tly Thomas R. Peacock, published in London in 1866, is credited with being the first comprehensive volume on congenital cardiac dcfects.’ Many since ha1.e contributed significantly to kmnvledge in this area, but it was Maude Al)bott‘s “Atlas of Congenital Cardiac Disease” that correlated the clinical and pathologic findings of congenital cardiac anomalies.2 Her influence was a powerful catalyst in accelerating \vork in this field. Most authors since Abbott have continued to classify congenital malformations of the heart This according to their anatomic defects. has many advantages, but the significant disadvantage is the difficulty of this approach in teaching. With clinical and physiologic modern diagnostic technics much information has been acquired about the physiologic effects on the heart of the various single or combined ‘I’he physical findings, abnormalities of lesions. the x-ray films or fluoroscopy and electrocardio,gram are the results of altered function of the heart due to the various malformations.RP5 The late Paul F\:ood clearly saw this change in clinical approach to cardiology, and his classification bcqan to deviate from the anatomic emphasis and stressed the physiologic importance of congenital anomalies of the heart.“,” In this paper a physiologic orientation has been employed to classify congenital malformaSuch grouping of congenital tions of the heart. heart disease has the disadvantage of not being useful in cataloguing the various diseases as in
an anatomic classification, because the same anatomic lesion may fall into two different categories depending on the influence of other factors, e.g., pulmonary resistance. Therefore, \zhat is presented is not a true classification in the usual definition, but rather a system which is an aid both in approaching the study of congenital heart disease and in understanding the clinical and physiologic features of the anomalies. This approach gives a clearer meaning to the clinical findings and laboratory studies in congenital malformations of the heart and No attempt is made to give the great vessels. differential diagnostic features or the laboratory data obtained in the different malformations nor are all congenital malformations listed; listed in this classification. These are well described in the textbooks and review articles on thesubject.“,i-‘2 In this classification congenital malformations of the heart ha1.e heen grouped into five physioeffects logic categories : four with abnormal on the heart and one without pathophysiologic Multiple complex defects are more alterations. clearly understood if approached physiologically, and although many would fall into two or more of the catcaories listed, their clinical and physiologic analyses are better evaluated under this system. Because of the many variables in a functional study, no attempt is made to list all described Some rare or theoretically possible lesions. lesions are listed to demonstrate better the application of this method. In any discussion
* From the Cardiology Service, Medical IXvision of Mont&ore 126
Hospital,
New York, N. Y.
THE: AMERICAN
JOURNAL
OF CARDIOLOGY
transposition. of corrected ventricle ih referred to and vl:ntriclc.
the not
physiologic the
anatomic
OR DECREASED PUUt0h.4~k BL,W,D FLO\~; KORMAL. RmsTmc~ IR'TFIE PULUONARY :++TERIoI..AR VAS~UL.~R RED
.UCIRMAL
SORZIAI. OR DECREASED PULMONARY BLOOD Fr.o\z-; HIGH RESISTANCE OF THE PULMONARY ARTERIOLAR \'ASCI:I.AR BED
ACYANOTI(: Increused Right Pulmonary Infundibular Stenosis of Congenital
Ventricular Work valvular stenosis* stenosis the peripheral pulmonary pulmonary insufficiency
Low Pulmonary Venous Presrure arteries
CY.L\NOTIC Increased I&ventricular Fl’ork Transposition of the great vessels with pulmanic stenosis Pulmonic stenosis and/or infundibular stenosis with IVSD Pulmonic stenosis and/or infundibular stenosis with persistent common atrioventricular canal Truncus arteriosus with hypoplastic pulmonary artery lncrvasrd
Right
Tetraloay Pulmonic
I~~mtrzcular
of Fallot stenosis
Work
with
Endocardial fibroelastosis \-on Gierke’q disease in\-olxing the myocardium (metabolic) Corrected transposition with malplaced tricuspid vaI\-e (mild) with insufficiency of the valves
(any lesion or Eisenmenger’s syndrome combination of lesions in which the pulmonary resistance is elevated to the degree that the net pulmonary flow is equal to or less than the systemic AL&) High Pulmonary I,7enousPressure Congenital mitral stenosis Car triatriatum (stenosis of common pulmonary vain) Congenital pulmonary venous stenosis INCREASED PULMON.~KY B~.om Fro\\ ACYANOTI:: Increased Right Ventricular Work
IXSD
(secundum
septal
tYPe Partial anomalous
type)
Common ventricle with rudimentary chamber and outflow area to pulmonary artery
LESKM INVOLVIK~ THE SYSTEMIC SIDE OF THE HE:\RT (LEFT VENTRICLE) WITHOUT INTRACARDIAC Smws Kf r2.5tanw !o Lrji I bnthilnr Ejectzon Aortic subvalvular stenosis Fit)1 01:s ring Muycular hypcrtrophy Aortic valvular stenosis Aortic supravalvular stenosis Coarctation of aorta with and without associated aortic valve involvement Otilrr LeAion. llffecting the Lejt Ventric.le ConSenital mitral insufficiency Aberrant origin of coronary vessels Idiopathic hypertrophy of the heart cardiomegaly )
Interatrial
defect
(L4SD)
pulmonary
secundum
venous
age Corrected transposition with IASD IASD-secundum type with plllmonic vular stenosis
drain-
val-
Increased Left Ventricular Work Patent ductus arteriosus (PDA) Ventricular septal defect (VSD) Corrected transposition with VSD Iru-reused Right and Left I ‘entricukw Niirk PDA or VSD with associated hyperkinetic pulmonary hypertension VSD with valbular or infundibular stenosis Persistent common atrio\.entricular canal, partial form’5 (IASD--ostium primum defect with cleft mitral valve) CYAKOTIC
(familial
Total anomalous puImonary venous drainage Transposition of the great vessels Taussig-Binq anomaly Truncus arteriosus Both vessels arising from right ventricle Common \xntricle
128
Progrrss
Notes in < hrdiolog~ 3.
Anomalies of the aortic arch and its /)ranches, e.g., v2tscular rings, aberrant origins of \ essels from the aorta IDextrocarclia Dcstrorotation of the heart Corrected transposition
A physiologic system has heen presented to facilitate the study of congenital malformation It is intended to of the heart and great vessels. aid in the clinical and physiologic understanding of the various anomalies of the heart. It is not meant to lx used as a catalogue of the diseases. Classifications in general are a method of current knowledge and serve, summarizing predominantly, as an orientation and a teaching Therefore, as new knowledge is obtained, aid. classifications should be changed in a more It is with this latter emphasis meanirqful way. that the material outlined is presented.
RUSHMEK, Ii. 1,‘. (Cardiovascular Dynamics. c,d. 2. Philadelphia anti I,onclon, 1961. W. R. Sanntlcrs. 4. RECK, W., SCIIRIK~, V., VOG~:LPO~I., :\., NI(I.LI:N, M. and SWANFOHI., A. Hemodynamic &ccts of amyl nitrite and phcnylr~phrinc on the normal human circulation and their relation to changes in cardiac murmurs. 4rn. J. Cardiol., 8: 341, 1961, 5. WOOD, I’. Disrasrs of the Heart and Circulation, cd. 2. Philadelphia, 1356. .J. 8. Lippincrltt Com6.
7.
8. 9.
10.
11. 12.
13.
I
should
son Young
like to thank
Dr. Louis
for their helpful
Leitcr
comments
and Dr. Denni-
and suggestions.
REFERENCES 1.
Introduction to facsimile edition, .4snow, M., Atlas of Congenital Heart Disrasr, 1954. American Heart Assxiation. Atlas of Congenital Heart Disease. 2. z\RROTT, M. facsimile rd.. 1954. American Heart Association. M.~RPI.E,
C.
14.
pany.
P. Eiscnmcnger’s syndrome or pulmonary hyprrtcnsion with revrrsed central shunt. &if. .ti. J., 2: 701,755,1958. ‘TAUSSIG, H. Congenital Malformations of the Heart, rd. 2. Cambridge, Mass., 1960. Amcrican Heart Association, Harvard Press. Pediatric Cardiology. NADAS, A. Philadelphia, 1957. W. B. Sallndcrs. KJELLBISKG. S. R., MANNIIEIM~~R, E., Rrlurre, ii. and JO~SSON, B. Diagnosis of Congenital Heart Diseaw, cd. 2. Chicago, 1959. Year Book Publishers. KEITH, J. D., Rowe, K. D., and VLAD, I’. Heart Disease in Infancy and Childhood. New York, 1958. Macmillan Company. S. E. Pathology of thr Heart, cd. 2. <;our.o, Springfield, III., 1960. Charles C Thomas. Congenital corrrctrd transSCHIEBLER, G. I>. vt al. position of the great vessels, a study of 33 cases. Pediatrics, 27 (Suppl.); Part II. 1961. KOSENBLC’M, K., MARK, H., ESCHER, I). I. W., STERN, W. %. and YOUNG, D. ‘The differentiation of pulmonic stenosis, ventricular srptal dcfcct with normal aortic root from tetralogy of Fallot. ilm. Heart .J., 46: 746, 1962. YOUNG, D. Critrria for surgery in persistent comAm. .J. Cardiol., 12: mon atrioventricular canal. WOOD,
80,1963. 15. WAKAT, C. S. and EDWARDS, .J. E. of pcrsistcnt
common
Pathologic study atrioventricular canal. Am.
Hmr/ .I.. 56: 779. 1958.
THE
AMERICAN
JOURNAI.
OF CARDIOLOGY