Few other medical disciplines have required for their development the degree of daring courage, tenacity, and drive that characterized the efforts of the early pioneers in the field of congenital cardiac surgery. Only a century ago, Theodore Billroth publicly condemned the dream of cardiac surgical intervention by stating that “Any surgeon who wishes to preserve the respect of his colleagues would never attempt to operate on the heart.”¹ Over the past six decades, the specialty of pediatric cardiac surgery has evolved from a heroic effort with occasional success into a consolidated, sophisticated specialty with excellent outcomes and, essentially, few limits imposed by pathology or age of the patient.

The history of pediatric heart surgery initially coincided with that of cardiac surgery itself. By far, the majority of early extra- and intracardiac procedures were in fact performed to address various forms of congenital (rather than acquired) heart disease. The adventure of intracardiac repair of congenital malformations, in turn, paved the way for technical advancements that pushed forward the field of adult cardiac surgery.

Each of the chapters in Part III of this book briefly touches on the historical highlights that pertain to specific malformations. In this chapter, the reader is offered a broad overview and key historical references that pertain to the initial evolution of this challenging field (Table 58-1), identifying four successive eras: (1) that of closed extracardiac operations; (2) the era of early closed or semiclosed intracardiac operations; (3) the initial phase of complete intracardiac repair; and (4) a subsequent period marked by the refinement of techniques and the expansion of the field to the correction or palliation of virtually any type of congenital heart disease.

At a time when transpleural surgery was at its dawn,^2–4 the repair of simple cardiovascular malformations was a natural initial surgical goal. Although these conditions were anatomically simple, the too often advanced disease state of the patients and the threat of infection in the preantibiotic era often made these initial daring attempts unsuccessful.

John Strider of Boston first performed surgical ligation of a patent ductus arteriosus on March 6, 1937, immediately noticing that the palpable thrill had disappeared after duct interruption.⁵ The 22-year-old patient was correctly diagnosed preoperatively with subacute bacterial endocarditis of the ductus arteriosus and succumbed to overwhelming sepsis on the fourth postoperative day. Necropsy disclosed a large endocarditic vegetation, extending from the arterial duct to the level of the pulmonary valve.

Robert Gross (Fig. 58-1) ushered in the field of congenital heart surgery by successfully interrupting a patent ductus arteriosus at Boston Children’s Hospital on August 26, 1938.⁶ Although he was the chief resident at the time, Gross performed the procedure in a very ill 7-year-old child, apparently against the advice of his chairman William Ladd.^7,8 The procedure took place while the latter was in Europe; the patient was uneventfully discharged in 10 days and survived for many years. Even though primacy of this milestone in cardiothoracic surgery has been debated,⁹ this landmark operation demonstrates that intervention on the great vessels within the depth of the chest was possible, with curative outcome.

Following this initial procedure, coarctation of the aorta was addressed. Alfred Blalock was the first to attempt correction of this problem in a canine model, by turning down the distally transected left subclavian artery and anastomosing the vessel to the proximal descending aorta (Blalock-Park procedure).¹⁰ A discouragingly high rate of paraplegia was noted, likely the consequence of relatively long clamp times, which limited flow through a normal-caliber subclavian artery and poor collateral circulation. Clarence Crafoord routinely clamped the aorta during ligation of the arterial duct, and first reported coarctation repair with end-to-end anastomosis in two patients operated at the Karolinska Institute in 1944.¹¹ Robert Gross described a case of successful coarctation repair shortly thereafter¹² and was first in addressing this malformation with an interposition homograft in 1949.¹³

These initial interventions were indeed curative, but the long-term outlook of children with complex cyanotic congenital heart disease remained dismal until the mid-1940s. Although somewhat approximative, preoperative diagnosis was possible and solely relied on clinical examination, chest radiographs, and electrocardiography; at Johns Hopkins, Helen Taussig was able, with such a rudimentary diagnostic armamentarium, to place children with congenital heart disease into broad diagnostic categories, underscoring the urgency of developing a method for stable palliation of cyanotic patients. The creation of an “artificial ductus arteriosus” was deemed unfeasible by Robert Gross; Taussig then turned to Alfred Blalock, the newly appointed chief of surgery at the Johns Hopkins Hospital (Fig. 58-2). While in Nashville, Dr. Blalock and Vivien Thomas (his laboratory technician) had established an experimental model of pulmonary hypertension that entailed ligation of the left pulmonary artery and anastomosis between the left subclavian and left branch pulmonary arteries.¹⁴ Although they were unable to augment pulmonary pressure because of the low impedance of the pulmonary vascular bed, the technique provided the increase in pulmonary blood flow that cyanotic children so desperately needed. This concept was introduced in the clinical arena on November 29, 1944, when Blalock successfully performed the first systemic-to-pulmonary artery shunt, thus opening the doors to long-term palliation for cyanotic patients (Fig. 58-3).^15,16 Eileen Saxon, then a 16-month-old infant with pulmonary stenosis (Fig. 58-4), had barely reached the operative weight of 4 kg from a nearly prohibitive birth weight of 1.1 kg.

Several modifications of this initial concept have followed and were all eventually rendered obsolete by the introduction of the “modified” Blalock-Taussig shunt.¹⁷ Among these, anastomosis of the descending aorta to the left pulmonary artery (Potts shunt)¹⁸ and of the ascending aorta to the right pulmonary artery (Waterston shunt)¹⁹ was reported in 1946 and 1962, respectively. These approaches often proved to be unreliable in regulating pulmonary blood flow, and were extraordinarily difficult to control at the time of late complete intracardiac repair.

Over the ensuing decades, systemic-to-pulmonary shunts—rather than the initial approach to staged repair of biventricular hearts—became the mainstay of palliation for univentricular heart disease (Fig. 58-5). Extracardiac palliative procedures are now more typically performed as the initial step toward achievement of complete right heart bypass, the so-called Fontan circulation (Fig. 58-6).^20–26

For children with malformations characterized by left-to-right shunting and pulmonary overcirculation, initial treatment of pulmonary hypertension by pulmonary arterial banding was first introduced by Muller and Dammann in 1952.²⁷ Although rarely utilized in biventricular hearts, pulmonary arterial banding still has a role in the initial staging of cardiac malformations characterized by univentricular morphology and pulmonary hyperperfusion (Chapter 77).

The first attempts at closed intracardiac intervention were made to address pulmonic stenosis in cyanotic infants. T. Holmes Sellors of London reported the first successful transventricular pulmonary valvotomy in December 1946.²⁸ He had originally intended to perform a systemic-to-pulmonary shunt, but the chronic sequelae of tuberculosis made this initial plan unfeasible in his patient with Fallot’s tetralogy. This approach was subsequently popularized by Lord Brock at Guy’s Hospital and became the standard of care for pulmonic stenosis for much of the following decade. This procedure was later to be largely supplanted by open valvotomy on cardiopulmonary bypass and, eventually, by the much more often utilized percutaneous balloon valvotomy, first introduced in 1982 by Jean Kan at the Johns Hopkins Hospital.²⁹