Surgical Management of Congenital Heart Disease in the 1990s Daniel M. Cohen, MD
Objective.\p=m-\Toprovide a broad overview of recent trends surgical management of congenital heart disease. Design.\p=m-\Reviewof recent literature. Setting.\p=m-\Majorcenters for surgical management of congenital heart disease. Selection Procedures.\p=m-\Analysisof important recent clinical publications. \s=b\
in
Interventions.\p=m-\None.
Results and Conclusions.\p=m-\Definitive,corrective surgery previously reserved for children beyond infancy. Recognition of the complications associated with palliative operations as well as the realization that neonates and infants did not fare worse than older children during open heart surgery provided the impetus for primary corrective surgery at an earlier age. Advances in our understanding of perioperative care, improved diagnostic capability, and increasing experience with corrective surgery in association with several different congenital heart lesions has resulted in an established therapeutic approach to surgical management of congenital heart disease in neonates and infants.
was
(AJDC. 1992;146:1447-1452)
Before
1426, 1429, and 1440.
Nylin3 reported the first successful repair of congenital coarctation of the aorta. Consequently, a new chapter in the management of congenital heart disease was opened. Both ligation of a patent ductus arteriosus and repair of a
coarctation may be considered corrective, ie,
definitive,
surgical procedures. Children who have undergone these operations usually reach adulthood and have normal lives. On the other hand, both pulmonary artery banding, which was introduced by Muller and Dammann4 in the early 1950s, and the Blalock shunt are palliative operations pro¬ viding only symptomatic relief for the affected child. The Blalock shunt is
an
anastomosis between the subclavian
Accepted for publication July 28,
1992. From the School of Medicine, Division of Cardiothoracic Surgery, East Carolina University, Greenville, NC. Dr Cohen is now with the Division
Surgery, Columbus (Ohio) Children's Hospital. Reprint requests to Division of Cardiothoracic Surgery, Columbus
of Cardiothoracic Children's
Cohen).
infancy. The
the 1930s, surgical treatment of congenital heart disease was nonexistent, and few children with con¬ genital heart disease lived beyond adolescence. In 1939, Robert Gross1 of Boston, Mass, ligated the first patent ductus arteriosus. Six years later, Alfred Blalock2 performed the first shunt procedure for management of cyanotic con¬ genital heart disease. In the same year, Crafoord and See also pp
and pulmonary arteries that provides an additional source of pulmonary blood flow. This shunt has been useful as palliative treatment for children with cyanotic congenital heart disease. Pulmonary artery banding constricts the main pulmonary artery, thereby reducing pulmonary blood flow and controlling congestive heart failure. Numerous complications have been associated with both of these palliative procedures and both are limited in their ability to provide effective palliation over a pro¬ longed period.510 Children who have undergone palliative procedures alone have rarely reached adulthood or led normal lives. In the early 1950s, controlled cross-circulation tech¬ niques, artificial oxygenators, and the heart-lung machine allowed surgeons to undertake corrective surgery for chil¬ dren with relatively simple intracardiac lesions.1115 These lesions included atrial septal defects, ventricular septal defects, tetralogy of Fallot, and atrioventricular canal de¬ fects. Because these operations were thought to be unsafe in infants, they were initially confined to children beyond
Hospital, 700 Children's Dr, Columbus OH 43205-2696 (Dr
was
impact
of
primary
corrective surgery in children
enormous; it markedly improved short- and long-term
survival.1618 As surgical experience expanded, morbidity and mortality from open heart surgery continued to decline and interposition of a palliative procedure before corrective operation became less attractive. PALLIATIVE VS DEFINITIVE SURGERY The application of a palliative rather than a definitive ap¬ proach to neonates and infants with congenital heart disease
persisted for several years because the results of early sur¬ gery were suboptimal. For almost two decades, an intuitive assumption prevailed that neonates and infants were more likely to fare worse than older children during open heart surgery.19 This assumption was based on prejudice rather than fact. Consequently, many children were subjected to multiple palliative operations before undergoing definitive surgery. Frequently, the complications of systemic-topulmonary shunts (Table 1) or pulmonary-artery banding (Table 2) rendered a corrective operation hazardous or con¬ traindicated. Finally, when definitive operation was under¬ taken, the outcome was not always favorable. Pediatrie cardiologists and surgeons alike began to reexamine their basic therapeutic approach to congenital heart disease. It has become obvious that the complica¬ tions associated with palliative operations often denied children the opportunity to grow and develop normally. This radical "change of heart" was based on several important facts: (1) Data from the New England Re¬ gional Care Registry have shown that one third of chil¬ dren with congenital heart disease became critically ill before age 1 year.20 (2) Palliative surgery was nonphys-
Downloaded From: http://archpedi.jamanetwork.com/ by a Karolinska Institutet University Library User on 05/27/2015
Table 1.—Complications of Systemic-to-Pulmonary Shunts
Early complications Mortality (
Objective.\p=m-\Toprovide a broad overview of recent trends surgical management of congenital heart disease. Design.\p=m-\Reviewof recent literature. Setting.\p=m-\Majorcenters for surgical management of congenital heart disease. Selection Procedures.\p=m-\Analysisof important recent clinical publications. \s=b\
in
Interventions.\p=m-\None.
Results and Conclusions.\p=m-\Definitive,corrective surgery previously reserved for children beyond infancy. Recognition of the complications associated with palliative operations as well as the realization that neonates and infants did not fare worse than older children during open heart surgery provided the impetus for primary corrective surgery at an earlier age. Advances in our understanding of perioperative care, improved diagnostic capability, and increasing experience with corrective surgery in association with several different congenital heart lesions has resulted in an established therapeutic approach to surgical management of congenital heart disease in neonates and infants.
was
(AJDC. 1992;146:1447-1452)
Before
1426, 1429, and 1440.
Nylin3 reported the first successful repair of congenital coarctation of the aorta. Consequently, a new chapter in the management of congenital heart disease was opened. Both ligation of a patent ductus arteriosus and repair of a
coarctation may be considered corrective, ie,
definitive,
surgical procedures. Children who have undergone these operations usually reach adulthood and have normal lives. On the other hand, both pulmonary artery banding, which was introduced by Muller and Dammann4 in the early 1950s, and the Blalock shunt are palliative operations pro¬ viding only symptomatic relief for the affected child. The Blalock shunt is
an
anastomosis between the subclavian
Accepted for publication July 28,
1992. From the School of Medicine, Division of Cardiothoracic Surgery, East Carolina University, Greenville, NC. Dr Cohen is now with the Division
Surgery, Columbus (Ohio) Children's Hospital. Reprint requests to Division of Cardiothoracic Surgery, Columbus
of Cardiothoracic Children's
Cohen).
infancy. The
the 1930s, surgical treatment of congenital heart disease was nonexistent, and few children with con¬ genital heart disease lived beyond adolescence. In 1939, Robert Gross1 of Boston, Mass, ligated the first patent ductus arteriosus. Six years later, Alfred Blalock2 performed the first shunt procedure for management of cyanotic con¬ genital heart disease. In the same year, Crafoord and See also pp
and pulmonary arteries that provides an additional source of pulmonary blood flow. This shunt has been useful as palliative treatment for children with cyanotic congenital heart disease. Pulmonary artery banding constricts the main pulmonary artery, thereby reducing pulmonary blood flow and controlling congestive heart failure. Numerous complications have been associated with both of these palliative procedures and both are limited in their ability to provide effective palliation over a pro¬ longed period.510 Children who have undergone palliative procedures alone have rarely reached adulthood or led normal lives. In the early 1950s, controlled cross-circulation tech¬ niques, artificial oxygenators, and the heart-lung machine allowed surgeons to undertake corrective surgery for chil¬ dren with relatively simple intracardiac lesions.1115 These lesions included atrial septal defects, ventricular septal defects, tetralogy of Fallot, and atrioventricular canal de¬ fects. Because these operations were thought to be unsafe in infants, they were initially confined to children beyond
Hospital, 700 Children's Dr, Columbus OH 43205-2696 (Dr
was
impact
of
primary
corrective surgery in children
enormous; it markedly improved short- and long-term
survival.1618 As surgical experience expanded, morbidity and mortality from open heart surgery continued to decline and interposition of a palliative procedure before corrective operation became less attractive. PALLIATIVE VS DEFINITIVE SURGERY The application of a palliative rather than a definitive ap¬ proach to neonates and infants with congenital heart disease
persisted for several years because the results of early sur¬ gery were suboptimal. For almost two decades, an intuitive assumption prevailed that neonates and infants were more likely to fare worse than older children during open heart surgery.19 This assumption was based on prejudice rather than fact. Consequently, many children were subjected to multiple palliative operations before undergoing definitive surgery. Frequently, the complications of systemic-topulmonary shunts (Table 1) or pulmonary-artery banding (Table 2) rendered a corrective operation hazardous or con¬ traindicated. Finally, when definitive operation was under¬ taken, the outcome was not always favorable. Pediatrie cardiologists and surgeons alike began to reexamine their basic therapeutic approach to congenital heart disease. It has become obvious that the complica¬ tions associated with palliative operations often denied children the opportunity to grow and develop normally. This radical "change of heart" was based on several important facts: (1) Data from the New England Re¬ gional Care Registry have shown that one third of chil¬ dren with congenital heart disease became critically ill before age 1 year.20 (2) Palliative surgery was nonphys-
Downloaded From: http://archpedi.jamanetwork.com/ by a Karolinska Institutet University Library User on 05/27/2015
Table 1.—Complications of Systemic-to-Pulmonary Shunts
Early complications Mortality (
