Heart Surgery Tables

Within a few years in the mid- to late 1950s, surgeons corrected thirteen types of congenital heart defects. Congenital heart surgery later evolved into its own subspecialty.


A treatment that improves a condition but does not cure it.

Congenital Heart Defect Year

Atrial septal defect 1953

Ventricular septal defect 1954

Complete atrioventricular canal 1954

Tetralogy of Fallot 1954

Tetralogy of Fallot 1955

Total anomalous pulmonary veins 1956

Congenital aneurysm, sinus of Valsalva 1956

Congenital aortic stenosis 1956

Aortopulmonary window 1957

Double outlet right ventricle 1957

Corrected transposition of great arteries 1957

Transposition of great arteries: atrial switch 1959

Coronary arterial-venous fistula 1959

Ebstein's anomaly 1964

Tetralogy with pulmonary atresia 1966

Truncus arteriosus 1967

Tricuspid atresia 1968

Single ventricle 1970

Subaortic tunnel stenosis 1975

Transposition of great arteries: atrial switch 1975

Hypoplastic left heart syndrome 1983

Pediatric heart transplantation 1985

feedings and lost weight. She weighed only 8.3 pounds at the time the operation was performed by Dr. Alfred Blalock at Johns Hopkins University Hospital on November 29, 1944. During the operation, Blalock sewed an artery that normally supplies blood to the arm to the left pulmonary artery so more blood could get to the lungs and be oxygenated (Fig. 7.1). The successful operation required slightly less than an hour and a half. Although this was not a cure for her heart condition, it improved the patient's symptoms and quality of life substantially.

Thus, within a seven year period, three congenital cardiovascular defects — patent ductus arteriosus, coarctation of the aorta, and vascular ring — were all attacked surgically and treated successfully. However, the introduction of the Blalock-Taussig shunt was probably a much more powerful stimulus to the development of open heart surgery because the operation palliated a complex intracardiac defect and focused attention on the abnormal physiology of cardiac disease.

























Heart-lung machine (HLM) Cross circulation Cross circulation Cross circulation HLM

First directly viewed correction First closure using HLM Extemporarily devised correction

Physiologic total correction

Repair of atrialized tricuspid valve Aortic allograft Aortic allograft Physiologic correction

Anatomic correction Two-stage operation

The next major step forward in heart surgery needed to wait for the development of the heart-lung machine, which occurred in the middle 1950s. With the advent of techniques to support the circulation and oxygenate the blood, using either the cross circulation technique of Dr. C. Walton Lillehei or the modified Gibbon-IBM heart-lung machine of Dr. John Kirklin, the cardiac teams of the University of Minnesota and the Mayo Clinic led the way and did many of the first intracardiac repairs for a number of commonly occurring congenital heart defects. Palliative operations, however, continued to be used and developed to improve circulatory physiology without directly addressing the anatomic pathology. The palliative operations somewhat improved the patients' conditions but did not cure them. As the safety of the heart-lung machine steadily improved, surgeons addressed more and more complex congenital abnormalities of the heart in younger and younger patients. Some of the milestones in the development of op erations to correct congenital defects using cardiopulmonary bypass appear in Table 7-1.

Diagnosing a Congenital Heart Defect

The human heart begins to develop at the end of the first month of fetal life and takes about another eight weeks before it resembles an adult heart. During this period, about eight out of every one thousand newborns develop some form of congenital heart defect ranging from very mild to quite severe. The exact cause of congenital heart defects is unknown, but recent information suggests there may be genetic influences. In some cases, they are associated with other medical conditions, such as the mother contracting German measles (rubella) while pregnant. At this point, most doctors don't think congenital heart defects are hereditary in the strict sense of being passed from parent to offspring, but children of parents who were born with such a defect will be somewhat more likely to have a congenital heart defect.

Some congenital heart defects are diagnosed shortly after birth or even while the baby is in the uterus by using ultrasound or echocardiography. They

Table 7-2: Relative frequency of occurrence of cardiac malformations at birth



Ventricular septal defect. . . .


Atrial septal defect


Patent ductus arteriosus. . . .


Pulmonary stenosis


Coarctation of the aorta . . . .


Aortic stenosis


Tetralogy of Fallot


Complete transposition

of great arteries


Truncus arteriosus


Tricuspid atresia


All others


Source: Heart Disease: A Textbook of Cardiovascular Medicine.

may be diagnosed later when the child is of school age, or in rare circumstances, the congenital cardiac defect remains hidden until adulthood. One indicator of some types of congenital heart defect in a newborn is a faint bluish color in the skin. Some children with heart defects may not thrive, and many suffer from congested lungs, which may be related to heart failure. Heart murmurs can also indicate congenital heart defects, although not necessarily.

If a defect in a newborn is suspected, your child's pediatrician will recommend an electrocardiogram and probably an echocardiogram, which do not require any needle sticks. Other tests used to diagnose congenital heart defects include cardiac catheterization and magnetic resonance imaging. After the heart defect is diagnosed and analyzed, your pediatrician and pediatric cardiologist will develop a treatment plan. This may require nothing more than yearly checkups or perhaps medications. Occasionally, a catheter can be used to dilate a heart valve or to insert a plug to close a hole. Heart surgery may be recommended and, in rare cases, heart transplantation is the best option.

Specific Defects

There are many types of congenital heart defects. Of the following eleven congenital heart defects, the first nine are relatively common. The last two are much more rare and included for a sense of perspective on the challenges facing a congenital heart surgeon. I also have purely personal reasons for mentioning them. In the 1970s and early 1980s, I was fortunate enough to work with Dr. C. Everett Koop at the Children's Hospital of Philadelphia. It was my privilege to have been involved with the care and surgery of some of these patients. Koop, later to become surgeon general of the United States, was then chief of pediatric surgery and

Table 7-2:

This table shows the most common congenital heart and major vessel defects. The ventricular septal defect, comprising almost 30 percent of congenital heart defects, is by far the most common.

Ductus Arteriosus:

A tube connecting the pulmonary artery to the aorta. After birth, when the lungs begin to function, this tube normally closes.

Fig. 7.2: Ventricular Septal Defect:

In this defect there is a hole in the wall of muscle, or septum, that separates the left and right ventricles. It is usually corrected with a patch.

had cared for patients with both types of these very rare and difficult defects.

Ventricular Septal Defect

In this most common congenital defect there is a hole in the septum that separates the right and left ventricles (Fig. 7.2). As a result, blood is short-circuited back into the lungs, putting a burden on both heart and lungs. About 30 percent to 50 percent of these holes, especially the smaller ones, close over time. Patients with large- or moderate-size defects that do not close spontaneously, however, eventually need an operation to close them. Larger defects may have to be closed in the first year of life because they can cause shortness of breath and other symptoms of heart failure. If the defect is not closed, the patient can also develop pulmonary vascular disease, which damages blood vessels in the lungs and can eventually be fatal.

Ventricular septal defects vary in size and location, so naturally some of them are more easily closed surgically than others. While in surgery, the patient's heart and lung function are provided by a heart-lung machine, and the actual hole is closed


Right Ventricle '""---_ ___ Septal Defect with a patch. The chances of surviving the surgery in childhood and subsequently living a normal life are superb.

Patent Ductus Arteriosus

While the fetal heart is developing, a tube develops between the aorta and the pulmonary artery. This tube, called the ductus arteriosus, is responsible for bypassing the lungs, moving blood from the pulmonary artery to the aorta. Because the fetus receives oxygenated blood from its mother through the placenta, it has no need for functioning lungs. After the child is born, however, the lungs begin to function, and the ductus arteriosus is no longer needed. It normally closes from a couple of hours to a couple of days after birth. However, if it remains open, or patent, it is considered a congenital defect and usually needs treatment (Fig. 7.3).

In some cases, the patent ductus ar-teriosus is so large that enough blood is shunted back from the aorta into the low-pressure pulmonary artery to actually flood the lungs. Heart failure can develop because the heart is working so hard to pump blood, and much of it is just being short-circuited back to the lungs. In other cases, infections develop in the tube, or over time high blood pressure in the pulmonary arteries can result in what's called pulmonary vascular disease. This disease damages the blood vessels in the lungs where resistance to blood flow increases and, at some point, the blood flow can actually reverse. If this happens, the right ventricle, which should be pumping un-oxygenated blood into the lungs, is actually pumping unoxygenated blood through the ductus directly into the aorta. This condition causes blueness, or cyanosis, and is very serious. Fortunately, the open ductus is usually diagnosed well before this condition develops, and the defect can be corrected.

There are three ways to close a patent ductus. In newborn babies, especially

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