Results of surgery for congenital supravalvular aortic stenosis.

Of eight children aged 3 to 15 years with surgical correction of severe supravalvular aortic stenosis, 6 were evaluated 7 to 44 months later by repeat cardiac catheterization and aortography. Prosthetic patch angioplasty was performed in all cases. Preoperative systolic gradients ranged from 40 to 90 mm Hg (average 70); postoperative gradients ranged from 0 to 20 mm Hg (average 11). The postoperative anglographic appearance of the ascending aorta was near normal in all six patients, and none had new aortic valve insufficiency. These results of surgery for supravalvular aortic stenosis are judged to be excellent.

Considerations in the surgical management of infantile coarctation of aorta.

The results of a recent 5 year experience with resection of coarctation of the aorta in infants less than 1 year of age are compared with those of an earlier series from the same institution. The significant improvement in mortality and morbidity statistics is attributed to modifications in operative and postoperative care. Operative mortality has decreased from 38 to 17 percent and the incidence rate of significant restenosis has diminished from 60 to 33 percent. It is suggested that in patients with large associated intracardiac shunt banding of the main pulmonary artery should be performed before resection of the coarctation. Three of five patients have survived procedures performed in this sequence. Microsurgical techniques and careful approximation of the aortic lumen with interrupted sutures are the major factors responsible for the reduced incidence of recoarctation. Prolonged ventilatory support postoperatively with the occasional addition of controlled positive airway pressure and continued aggressive medical therapy for heart failure are recommended.

Treatment of d-transposition of the great arteries: management of hypoxemia after balloon atrial septostomy.

Between 1975 and 1979, a group of 43 patients with d-transposition of the great arteries were diagnosed and underwent Rashkind balloon atrial septostomy at the time of initial catheterization. Thirty-six (88 percent) survived to the time of intraatrial baffle repair, and 31 (72 percent) are long-term survivors, 2 of them now awaiting repair. Palliative operations were performed in nine patients before definitive surgery; four of these patients are long-term survivors. Prostaglandin E1 infusion improved oxygenation and relieved acidosis in four patients. It is concluded that most patients with d-transposition of the great arteries will survive to elective intraatrial baffle repair between 6 and 12 months without surgical palliation in spite of significant hypoxemia. Prostaglandin E1 infusion may be lifesaving and provide sufficient palliation in patients with persistent hypoxemia and acidosis after balloon atrial septostomy.

Spontaneous closure of secundum atrial septal defect in infants and young children.

The records of 264 pediatric patients with uncomplicated ostium secundum atrial septal defect (ASD) were reviewed. Eighty-seven patients were younger than age 4 years at the time of cardiac catheterization. Subnormal weight gain, frequent pneumonia, cyanosis or tachypnea were present in 26 patients (30%). Of the 36 infants at catheterization, 17 (48%) had the previously described symptoms, including 12 (33%) who had congestive heart failure. Eight of the 36 infants were found to have closed their defect at a subsequent catheterization. Six of 18 patients who underwent cardiac catheterization between 1 and 2 years of age also had spontaneously closed their ASD at subsequent study. Statistical analysis of hemodynamic data revealed no difference (except a smaller shunt size) between ASDs that closed and those that did not in patients who were less than 4 years at initial catheterization. Analysis of hemodynamic data revealed no statistical differences between groups of patients with an ASD who were younger than and those older than 4 years at time of diagnostic study. Patients with ASDs that closed were significantly different from patients with atrial level shunting thought to be secondary to a valve-incompetent foramen ovale with respect to age at initial study (11 versus 2 months, p less than 0.001), mean left atrial pressure (7.7 versus 12.3 mm Hg, p less than 0.02) and difference between mean right and left atrial pressures (1.0 versus 4.2 mm Hg, p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Congenital absence of left pericardium in a family.

Characteristic radiographic findings of absence of the left pericardium were observed in two relatives who were related to two other individuals with different types of congenital heart defects. Familial occurrence of congenital absence of pericardium has not been reported previously. The strong male sex predilection in the previously reported patients, the segregation pattern of affected individuals in the pedigree presented here, and the presence of two other individuals with multifactorially determined congenital heart defects in the same pedigree suggest multifactorial determination of congenital absence of the left pericardium.

Congenital arteriovenous malformations between brachiocephalic arteries and systemic veins.

Three patients under two years of age are described with unusual fistulas involving the brachiocephalic arteries and the innominate vein or the superior vena cava. Two patients were asymptomatic, and one newborn was cyanotic and in congestive failure. This unusual presentation has only rarely been reported in thoracic arteriovenous malformations. Two of the three patients underwent successful surgical repair. Two-dimensional echocardiography aids in the evaluation of these patients, but cardiac catheterization and angiography are indispensable diagnostic techniques for documenting the extent and location of these lesions and thus guiding proper therapy.

Noninvasive diagnosis of right-sided extracardiac conduit obstruction by combined magnetic resonance imaging and continuous-wave Doppler echocardiography.

Right-sided extracardiac conduits are frequently complicated by obstruction over time. We compared the utility of two-dimensional and Doppler echocardiography and magnetic resonance imaging in the diagnosis of postoperative right-sided obstruction with cardiac catheterization and angiography in 10 patients with xenograft or homograft conduits. Correlation (r = 0.95) between continuous-wave Doppler estimates and catheter pullback pressure gradients across the conduits was excellent. Echocardiography could only visualize five of 10 conduits in their entirety. Magnetic resonance imaging visualized all conduits and showed statistically significant (kappa = 0.58) agreement with angiography in the localization and estimation of severity of a variety of right-sided obstructions in these patients. However, flow voids created by the metallic ring around xenograft valves led to a false negative diagnosis of valvular stenosis in four patients when magnetic resonance imaging was used alone. Doppler studies correctly indicated obstruction in these patients. The combination of magnetic resonance imaging studies and continuous-wave Doppler echocardiography can be useful to noninvasively evaluate right-sided obstruction in postoperative patients with right-sided extracardiac conduits.

Surgical management of hypoplastic right ventricle with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum.

Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum includes 26 patients with pulmonary atresia and 4 with critical pulmonary stenosis. Group 1 consisted of 8 neonates managed initially by transventricular valvotomy; 6 later required a secondary procedure, with 100% survival. Group 2 had 11 neonates managed by aorta-pulmonary artery shunting without operative death. However, only 3 have survived over the long term and 1 has required an additional shunt procedure. Group 3 had 9 infants who underwent concomitant valvotomy and shunting. There were 4 operative deaths and 1 late death. Finally, Group 4 included 2 infants managed by primary repair at 3 days and 6 days old with prosthetic enlargement of the right ventricle; 1 required the addition of a shunt. Both are alive. Seven of the 15 patients in Groups 1, 2, and 3 who survived neonatal palliative procedures have undergone reparative operations. Two had no growth of the right ventricle and underwent repair after conversion to tricuspid atresia, by a Fontan procedure. Five had prosthetic enlargement of the right ventricle in childhood with 1 late death. Findings of this review were as follows: (1) effective palliation of pulmonary atresia and intact ventricular septum or critical pulmonary stenosis with cavitary hypoplasia of the right ventricle is rare unless transventricular flow can be established; (2) establishment of transventricular flow produces a high incidence of cavitary "growth," which permits later repair; (3) the Fontan operation is available for repair in patients who have no cavitary growth; and (4) when all three portions of the right ventricular cavity can be identified by angiography, a primary repair can be performed in the neonatal period with a good long-term prognosis.

Current management of transposition of the great arteries: immediate septostomy, occasional prostaglandin infusion, and early Senning operations.

Between January, 1979, and September, 1982, 30 infants with dextro(D)-transposition of the great arteries were managed with the Senning procedure for transposition of ventricular inflow. In 11 infants under 6 months of age, there were no associated cardiac malformations and no hospital deaths. Among 17 infants operated on between the ages of 6 and 12 months, 6 had associated cardiac malformations, and there were 2 hospital deaths. Two infants in the series were over 12 months of age; 1 had an associated malformation, and there were no hospital deaths. Analysis of cardiac rhythms in the postoperative period demonstrates that the first 2 patients operated on continue to have persistent junctional escape rhythm, while the remaining 26 survivors are in sinus rhythm. Twenty-four-hour Holter monitoring performed in 24 patients showed only 9 patients to be in sinus rhythm throughout the entire recording period. Seven patients had occasional atrial and ventricular premature contractions; the remainder had episodes of sinus arrest with junctional escape rhythm. Evidence of pulmonary caval or pulmonary venous obstruction has not appeared in any patient. Recently introduced technical modifications to the Mustard procedure have improved the results of that operation in regard to rhythm disturbances and baffle obstruction to venous return. This series, therefore, does not demonstrate superiority of the Senning procedure over the Mustard procedure. However, since results comparable to those of the Mustard procedure can be obtained in very young infants using the Senning operation along with deep hypothermia and circulatory arrest, the Senning procedure is deemed preferable to the Mustard procedure for this age group because of the ease with which it can be performed and because the procedure eliminates surgical judgment, and thereby surgical error, in the location of suture lines.