CHAPTER 14 



Physiologic consequences of congenital 

 heart disease' 



HIRAM W . MARSHALL 

 H. FREDERIC HELMHOLZ, Jr. 

 EARL H. VVOOD^ 



Mayo Clinic and Mayo Foundation, Rochester, Minnesota 



CHAPTER CONTENTS 



Types and Anatomy of Congenital Cardiac Defects 



Anomalies of the Great Vessels 



Intracardiac Anomalies 



Conduction Defects 



Abnormalities of Position 

 Methods of Study 



Clinical Methods 



Intracardiac Catheterization 



Determination of Blood Gases 



Calculation of Blood Flows and Shunts 



Indicator-Dilution Curves 



Angiocardiography 

 Normal Circulation 



Fetal Circulation 



Postnatal Circulation 



Normal Adult Circulation 

 Hemodynamic Alterations .\ssociated With Congenital Cardio- 

 vascular Defects 



Obstruction of Great Vessels 



Valvular Deformities Without Septal Defects 



Communications Between Right and Left Ventricles and 

 Between Pulmonary and Systemic Arteries 



Atrial Septal Defects 



Partial or Total Anomalous Pulmonary Venous Connection 



Persistent Common Atrioventricular Canal 



Septal Defects With Valvular Stenosis 



Right-to-Left Shunts 

 Summary 



' This study was supported in part by Research Grant 

 H-3532 from the National Institutes of Health, United States 

 Public Health Service. 



2 Career Investigator, American Heart Association. 



DURi.NG THE L.AST I oo ye.'-lRS a multitude of papers 

 and case reports ha\-e appeared in the literature on 

 congenital heart disease. However, there was very 

 little correlation and integration of this material 

 until the time of Dr. Maude Abbott (i ), whose work 

 provided the basis of our modern understanding of 

 the various congenital anomalies of the heart. When, 

 in 1938, Gross & Hubbard (124) performed the 

 first successful operation for ligation of a patent 

 ductus arteriosus and, in 1945, Gross & Hufnagel 

 (125) and Crafoord & Nylin (72) first successfully 

 corrected coarctation of the aorta, it brought the 

 realization that accurate diagnosis of congenital 

 cardiac defects had become imperative because 

 certain properly selected patients could now be 

 helped by surgical treatment. Tremendous added 

 momentum to this field was given by the operation 

 for supplementing the blood going to the lungs in 

 cases of congenital pulmonary stenosis, first reported 

 by Blalock & Taussig (32) in 1945. 



Physiologic studies of congenital cardiac defects 

 ha\e depended in large measure on development 

 of the technique of intracardiac catheterization. 

 Forssmann (107) in 1929 introduced a catheter into 

 his right atrium from his left arm, but only subse- 

 quent to the studies of Cournand & Ranges (70) 

 was general interest in the method aroused. It soon 

 became evident that important contributions to 

 intracardiac hemodynamics in congenital malforma- 

 tions of the heart were possible. Great progress has 

 been made in elucidating the physiologic effects of 

 congenital cardiac defects in the past decade. 



417 



