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Ann Thorac Surg 1997;64:1544-1548
© 1997 The Society of Thoracic Surgeons
Departments of Surgery, Texas Heart Institute and The University of Texas Medical School, Houston, Texas
Abstract
Experience in the surgical management of congenital heart defects led to the advent of open heart surgery as it is known today. Only after 1938, when Robert Gross first ligated a patent ductus arteriosus, did congenital anomalies yield to correction. Success with these anomalies encouraged surgeons to attempt other extracardiac and intracardiac repairs. These attempts resulted in a steady flow of advances that culminated in the practical application of cardiopulmonary bypass and the procedures it made possible. Today, less than 60 years since Gross's landmark operation, surgical intervention can fully or partially correct 95% of congenital heart defects.
In 1913, Sir William Osler recommended in The Principles and Practice of Medicine [1] that children with congenital heart disease be treated only for their symptoms, even advising parents to keep such children "warmly clad." That perspective changed forever in 1938 when Robert Gross [2], a young surgeon in Boston, successfully ligated a patent ductus arteriosus.
For children born with cardiac malformations, Gross's landmark procedure opened a world of possibilities, albeit only those achievable with "closed" techniques. Soon surgeons had devised palliative procedures to treat tetralogy of Fallot (Fig 1
) and coarctation of the thoracic aorta. Once surgeons achieved some success with extracardiac procedures, they attempted intracardiac operations, including correction of pulmonary valve stenosis. By 1949, Alfred Blalock and his associates at Johns Hopkins University had operated on 878 patients with pulmonic stenosis. In 1950, I [3] reported the results of 48 of these cases, in which vigorous cardiac massage was required because of imminent or actual intraoperative cardiac arrest. In 33 patients, cardiac function was successfully restored.
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The 1950s: The Modern Era
In 1952, F. John Lewis and Mansur Taufic [4], of the University of Minnesota, instituted a new era in surgical therapy by performing the first intracardiac procedure under direct vision: repair of an atrial septal defect through a right atriotomy. The repair was accomplished by using moderate hypothermia and inflow occlusion for circulatory arrest. Others who contributed to the use of hypothermia for intracardiac surgery were Wilfred Bigelow (Toronto), Henry Swan (Denver), and W. H. Muller, Jr (Los Angeles).
Unfortunately, the time available for repair by these early intracardiac techniques was extremely restricted, with an upper limit of about 10 minutes. Although repairs of some of the simpler septal defects could be accomplished, more complex lesions, such as atrioventricular canal and large ventricular septal defects, remained untreatable. Surgeons needed a longer safe period of ischemia and a bloodless field in which to operate.
Other techniques for intracardiac repair were also being tried. In 1952, Robert Gross and colleagues [5] described their experiments with a method that became known as the "Gross atrial well technique." Gross accessed the septum through the right atrium by suturing a rubber funnel to an incision in the atrium. Because the well filled with blood, however, the procedure had to be done by means of palpation, not direct vision. Subsequently, Gross and Elton Watkins [6] revised the indications for this operation to patients whose defects could not be repaired with external sutures—defects that were unusually large or located in the anterior septum.
Many experts believed that extracorporeal circulation would allow more extensive intracardiac repairs, but experiments had not yielded a dependable device with which to support a patient's circulation. Since the 1930s, John H. Gibbon had been publishing reports of his experiments in this field. At a cardiovascular symposium in Minneapolis in 1953, he described 4 previously unreported cases of patients who had undergone operations with the aid of "a mechanical heart-lung apparatus" [7]. Three of the 4 patients died, which Gibbon attributed to human error. However, the fourth patient, an 18-year-old girl, survived and thrived after Gibbon closed a large interatrial septal defect while the heart-lung machine maintained the patient's entire cardiopulmonary function for 26 minutes. Two months after the operation, follow-up cardiac catheterization showed that the septal defect remained closed and that there was no residual left-to-right shunt.
Before Gibbon's successful intracardiac procedure, others had tried heart-lung bypass machines for open heart and extracardiac procedures. In April 1951, Clarence Dennis and associates [8] had used a horizontal screen disc oxygenator in two unsuccessful open heart attempts. In the first case, the diagnosis was incorrect: the atrial septal defect was actually an atrioventricular canal. In the second case, a technician "failed to activate the level control circuit" [9]. A few months later, Mario Dogliotti [10], in Turin, successfully used a bubble oxygenator as a precautionary measure during removal of a mediastinal tumor that was compressing the heart. The patient recovered.
Attempts at cardiopulmonary bypass resulted in discouraging results until C. Walton Lillehei and his Minneapolis team [11] began performing open repairs using still another method: controlled cross-circulation (Fig 2). Lillehei and his colleagues used this method for 17 months, until July 1955, when the bubble oxygenator became the standard oxygenator. Of the 45 patients who underwent controlled cross-circulation, 22 (49%) survived and were in New York Heart Association functional class I 30 (or more) years later [12]. All of these patients had anomalies that were considered uncorrectable, and many were in advanced heart failure. More than half were 6 months of age or younger. In Lillehei's series, the corrected abnormalities included ventricular septal defect, tetralogy of Fallot, atrioventricularis communis, patent ductus arteriosus (misdiagnosed as ventricular septal defect), isolated infundibular pulmonary stenosis, and combined pulmonary stenosis, atrial (secundum) defect, and anomalous pulmonary venous drainage. This series included the first successful repair of tetralogy of Fallot. Because of the success of controlled cross-circulation, Lillehei and others became convinced that open heart surgery with temporary cardiopulmonary bypass was feasible.
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). Thus, the early pioneers of cardiac surgery showed that surgical correction could at last replace palliation in some cases of intricate anomalies. As a result, the ranks of operative candidates grew.
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). The oxygenator devised by I. M. Rygg [16] of Copenhagen and a modified model designed by Vincent Gott [17] of Minneapolis soon replaced the permanent, reusable type. In addition, Zuhdi and associates [18] proposed that the extracorporeal circuit be primed with a 5% dextrose solution instead of freshly drawn, heparinized blood. This approach, which was made a standard of open heart surgery by my team [19], relieved the heavy burden on blood suppliers, facilitated open heart techniques, and dramatically reduced the risk of postoperative pulmonary insufficiency, low cardiac output failure, mental confusion, and hepatitis. With the use of a nonblood prime, surgeons were able to perform an increasing number of open heart operations. As a result, open heart surgery proliferated around the world.
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After congenital heart disease yielded to surgical correction, progress in cardiac surgery became unstoppable. The advances necessitated more accurate diagnoses, thereby stimulating the advent of improved diagnostic instruments and methods. In turn, these breakthroughs permitted surgical triumphs well beyond what had been envisioned during the early years. As progress was made, a new era was born. Surgeons were easily able to correct septal defects and pulmonary and aortic valve stenoses. They began to dismantle earlier palliative shunts to proceed with more definitive correction.
Within 30 years, they would devise procedures for conquering the more complex defects: correction of total anomalous pulmonary venous drainage (Cooley and Ochsner, 1957) [20], intraarterial rerouting of venous flow for transposition of the great arteries (Senning, 1959) [21], simplification of Senning's intracardiac repair for transposition (Mustard, 1964) [22], bypass of an atretic pulmonary valve with an aortic homograft (Ross and Somerville, 1966) [23], corrective operation for congenital tricuspid atresia (Fontan and Baudet, 1971) [24], repair of single ventricle (Sakakibara and associates, 1974) [25], arterial switch for transposition (Jatene and colleagues, 1976) [26], and two-staged correction of hypoplastic left heart syndrome (Norwood and coworkers, 1981) [27].
In the early days of open heart procedures for congenital defects, most surgeons believed that the patient's optimal age at operation was at least 2 but more often 10 years, when the child would be large enough to withstand the operative procedure. However, fewer than half of babies born with congenital heart disease reached their first birthday, and most died at age 3 to 6 months. For this reason, we began to operate on younger patients. By 1959, we [28] had operated on 120 patients less than 1 year old (including 13 less than 1 month old). Although many had been in severe cardiac failure at the time of their operation, more than 70% survived. We have performed intracardiac procedures on more than 10,000 pediatric patients (<18 years of age) with congenital heart disease. Today, specialists in pediatric cardiac surgery must be ready to treat these patients even earlier, within the first days or weeks of life.
Although the incidence of congenital heart defects appears to have increased in recent years, this increase is thought to reflect improved training of pediatric cardiologists and advances in diagnostic technology afforded by Doppler color-flow echocardiography. The National Center for Health Statistics states that, in 1989, heart malformations were recognized at birth in 1.29 per 1,000 cases of live births; because these records came from birth certificates, however, some abnormalities remained undetected [29]. Estimates of the postnatal incidence of congenital heart disease range from a low of 3 to 5 per 1,000 live births to a high of 12 per 1,000 [30].
Since the advent of open heart surgery, surgical treatments have been devised to palliate or cure most complex congenital defects. Today, more than 95% of significant congenital heart conditions can be corrected or alleviated by surgical intervention [31]. However, finding replacement parts for anomalous portions of the cardiovascular system remains a problem. Recently, homograft valved conduits and homograft valves have been used in repairing a wide variety of congenital defects, including tetralogy of Fallot, pulmonary atresia, hypoplastic left heart syndrome, and aortic and pulmonary stenosis.
In the past 15 years, heart transplantation, which had formerly been reserved mostly for adults, has also been used to treat pediatric patients with complex anomalies or end-stage congenital heart disease. Although the long-term results are not fully known, children who receive heart transplants appear to fare as well as if not better than adults. Leonard Bailey of Loma Linda, California, pioneered heart transplantation in infants and children [32]. He has achieved excellent survival rates even in neonates, who usually require only minimal immunosuppressive agents for controlling rejection. Our results corroborate those of Bailey. In our 83 pediatric heart-transplant patients, survival rates have been similar to that of adult transplant patients at 1 year and better than those of adults at 5 years (pediatric 5-year survival rate, about 78%). In addition, we have found that graft atherosclerosis develops much more slowly in pediatric patients [33]. In 1984, we performed heart transplantation in an 8-month-old female infant with subendocardial fibroelastosis [34] who, at that time, was the youngest patient to have undergone transplantation. This child (who is now 13 years old) continues to grow and develop normally.
In the future, analysis of the outcomes of current procedures will determine whether techniques must be further refined. Long-term results of homograft implantation and cardiac transplantation will allow investigators to determine whether these procedures are, indeed, the best treatments for children with severe congenital heart disease. Axial-flow cardiac assist devices such as the Transcoil ventricular assist system being studied at our institution [35] may have an important future role in these cases. Such devices can be inserted or implanted relatively quickly, and they can be used in patients of all sizes, including children. With further miniaturization, these devices may also become available for neonates. Artificial hearts, which have shown promise for adult patients as bridges and alternatives to transplantation [36], may eventually be miniaturized so that children can benefit from their life-saving potential.
Summary
During the past 60 years, advances in the treatment of congenital heart disease have dramatically changed not only the lives of the patients involved but also the lives of the pioneering investigators in this field. As we grapple to overcome today's obstacles to innovative therapeutic breakthroughs, we must not forget the struggles and the contributions of the pioneers whose improvisation, cooperation, perseverance, and vision made the treatment of congenital heart disease a reality.
Footnotes
Presented at Cardiovascular Surgery—Then and Now, University of Virginia Medical Center, Charlottesville, VA, April 26, 1997.
Address reprint requests to Dr Cooley, Texas Heart Institute, PO Box 20345, Houston, TX 77225-0345.
References
This article has been cited by other articles:
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  T.-Y. Hsia and P. J. Gruber Factors Influencing Neurologic Outcome After Neonatal Cardiopulmonary Bypass: What We Can and Cannot Control Ann. Thorac. Surg., June 1, 2006; 81(6): S2381 - S2388. [Abstract] [Full Text] [PDF]
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