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.

Evaluation of pulmonary arterial morphology in cyanotic congenital heart disease by magnetic resonance imaging.

Before and after surgical therapy, the anatomy of the pulmonary arteries in cyanotic congenital heart disease is often distorted. Pulmonary arterial anatomy was evaluated by magnetic resonance imaging (MRI) and angiography in 20 patients, ages 3 months to 20 years, with cyanotic heart disease associated with decreased pulmonary blood flow. Excellent correlation between MRI and angiographic estimates of pulmonary artery diameter was obtained (main pulmonary artery, r = 0.96; right pulmonary artery, r = 0.93; left pulmonary artery, r = 0.96). A similar excellent correlation (kappa = 0.83) was found in the assessment of the presence and severity of proximal pulmonary arterial stenoses. However, stenoses in the peripheral pulmonary arteries visualized with angiography were missed with MRI. MRI and angiography showed complete agreement in determining the patency of 11 surgical shunts. MRI did not demonstrate all of the systemic collateral vessels present with angiography, and the distal connections of collaterals were not detected with MRI. MRI is comparable to angiography in the evaluation of central pulmonary arterial anatomy over a wide range of ages. These findings suggest an important role for noninvasive MRI in the serial evaluation of pulmonary artery morphology in patients with cyanotic congenital heart disease before and after surgical repair.

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.

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.

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.

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)

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.

Improvement of left ventricular function after ligation of the anomalous left coronary artery during infancy.

Considerable controversy surrounds the optimal management of symptomatic infants with anomalous origin of the left coronary artery from the pulmonary artery; this includes the timing and type of surgical intervention. Long-term follow-up was obtained on three patients who had simple ligation of the anomalous left coronary artery at or before 8 months of age; long-term follow-up on an adolescent treated with a saphenous vein bypass graft from the aorta to the coronary artery was available for comparison. Remarkable increase in left ventricular contractility was observed in the three patients treated only by simple ligation of the anomalous left coronary artery during infancy. Thallous chloride T1 201 exercise studies disclosed no perfusion abnormalities in any of the patients despite angiographically demonstrable wall-motion abnormalities in two of the four patients. Our study suggests that simple ligation of the anomalous left coronary artery near its origin in patients with angiographically detectable left-to-right shunting can provide long-term survival with good left ventricular function and minimal clinical complaints.

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.

Familial Williams syndrome.

Characteristic features of the Williams syndrome were observed in a mother and her presumably dizygotic twin daughters, suggesting either an X-linked dominant, autosomal dominant or multifactorial inheritance in this pedigree. The Williams syndrome generally has been thought to have a sporadic occurrence. The rare familial occurrence, however, suggests a possible heterogeneity in its etiology and also points out the importance of careful examination of the other family members for proper genetic counseling.

Partial trisomy of the short arm of chromosome 8 resulting from balanced maternal translocation.

Extra chromosomal material on the long arm of chromosome 15 was found in an infant with growth retardation, dysmorphic features, skeletal abnormalities, and congenital heart defect. The phenotypically normal mother had a balanced translocation between the short arm of chromosome 8 and the long arm of chromosome 15: 46,XX,t(8:15)(p12:q25). Thus, the patient was partially trisomic for the short arm of chromosome 8 (p12 leads to pter). Comparison of the clinical data obtained from patients with partial trisomy of the short arm of chromosome 8 with those of full trisomy 8 (Warkany's syndrome) suggests that most of the clinical features of Warkany's syndrome require excess material from both the short and long arm of chromosome 8.

Partial trisomy 13 as a result of de novo (6p;13q) translocation.

A newborn infant with the clinical features of the Patau syndrome was found to have excess chromosome 13 material present as a tandem translocation involving the short arm of chromosome 6 and the long arm of an extra chromosome 13: 46,XY,t(6;13)(p24;q12). The major part of the long arm of the extra chromosome 13 was attached linearly (tandem translocation) to the short arm of chromosome 6. Both parents were phenotypically and karyotypically normal.

Blalock-taussig anastomosis: the preferred shunt in infants and newborns.

The Blalock-Taussig anastomosis (B-T shunt) has been regarded as an unsatisfactory procedure for infants and newborns with severe pulmonary oligemia. With use of microsurgical technique, B-T shunts were constructed in 11 infants under 6 months of age, six of whom were less than 1 month old (2 to 30 days). Weights ranged from 2.4 to 5.6 kg. Diagnoses included transposition of the great arteries (4), tetralogy of Fallot (4), pulmonary arterial atresia (2), and Taussig-Bing syndrome (1). There was no operative mortality; perioperative morbidity included paralysis of the diaphragm (2), Horner's syndrome (1), superficial wound dehiscence (1), congestive heart failure (1), and prolonged respiratory failure requiring ventilation assistance for more than 5 days (2). There has been one late death in the group at 2 months post-shunt. All surviving patients have had satisfactory palliation of cyanosis and hypoxemia. In view of the pulmonary artery scarring and kinking that follows the Waterston and Potts shunts, it is concluded that the Blalock-Taussig shunt is a reliable and preferable technique for palliation of pulmonary oligemia in infants and newborns.

Long-term function of aortic homografts as the right ventricular outflow tract.

Physical examination, chest roentgenography, phonocardiography, and right heart catheterization were used to evaluate the function of composite aortic homografts inserted as right ventricular outflow tracts in 13 patients 1.8 to 7 years previously. All homografts in these patients who were followed for 2 or more years demonstrated calcification of the aortic wall, but valve leaflets were spared. Moderate pulmonary insufficiency was present in eight patients with single cusp partial homografts, but was well tolerated in all. Homograft valve insufficiency was mild in five patients with complete homografts. No patient had an outflow tract gradient greater than 32 mm Hg. The average gradient was 18.5 +/- 2.7 (SEM) mm Hg. Abnormality of pulmonary blood flow was found in three patients and could be related to previous Waterston or Potts anastomoses. No late morbidity due to homograft degeneration has occurred and the long-term function of these conduits appears to be good.

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.