Severe Subaortic Obstruction Research Articles

Hypertrophic Obstructive Cardiomyopathy in an Infant With an Adrenocortical Tumor

Nonfamilial cardiomyopathies in childhood have been only sporadically ascribed to endocrine disorders. We report on a 4-month-old male infant presenting with Cushing's syndrome associated with excessive body weight (8.9 kg; >97th percentile) and features of virilization (Tanner stage 2 for pubic hair development). Abdominal sonography showed a large adrenal tumor. Echocardiography revealed myocardial hypertrophy with severe subaortic obstruction. Blood tests showed excessive androgen and cortisol serum levels with absent circadian rhythm as well as suppressed corticotropin. Urine catecholamine levels were within the normal range. Tumor resection with general anesthesia was performed after preparation with antihypertensive and anticongestive drug therapy. Continuous intravenous hydrocortisone substitution was started intraoperatively and subsequently tapered and switched to oral administration after 12 days. A gradual reduction in glucocorticoid substitution and its discontinuation after a total duration of 9 months were well tolerated. Histopathologic workup revealed an adrenocortical tumor of intermediate dignity. Postoperative tumor staging excluded both residual primary tumor and metastases. Both a normalization of body weight and myocardial mass were observed. The present article is, to our knowledge, the first to describe severe hypertrophic obstructive cardiomyopathy caused by an adrenocortical tumor and provides novel detailed data on postoperative glucocorticoid management.

Pregnancy and the Rastelli operation

The aim of surgical correction of congenital heart disease is for the patient to live a relatively normal life, including potential pregnancy and childbirth. The Rastelli repair operation using a valved conduit for certain complex congenital heart lesions has resulted in survival into adult life. We reviewed reproductive outcome of 22 women over 18 years of age who were survivors of the Rastelli operation performed between 1977 and 1999. Six women had a total of 12 pregnancies with six live births. Three women, with complex transposition of the great arteries, developed more severe subaortic obstruction during pregnancy. All required further heart surgery. Delivery was by Caesarean section in three patients and vaginally in the other three. All infants were healthy and free from defects. Two women had tubal ligations because of their cardiac problems. Women who have had Rastelli repair operation for complex congenital heart disease can have successful pregnancies. Subaortic stenosis can become a problem and should ideally be eliminated before pregnancy. Because of multiple operations, complications and genetic concerns, some women choose not to reproduce. Careful cardiac evaluation before, during and after pregnancy is essential.

Characteristics and management of cleft mitral valve

ObjectivesWe sought to highlight the clinical, morphologic, and pathogenetic features in patients with a cleft mitral valve (MV). BackgroundFew studies have addressed the morphologic features of cleft MV and the outcome of these patients. The pathogenetic features, including the developmental relation to an atrioventricular (AV) septal defect, remain unclear. MethodsWe reviewed the patients with cleft MV that were diagnosed by echocardiography since 1980. Patients with an AV canal, ventriculo-arterial discordance, and hypoplastic ventricles were excluded. ResultsTwenty-two patients were identified at a median age of 0.5 years (range 0 to 10.6). In three patients, no chordal attachments of the cleft to the ventricular septum were seen. Ten patients had significant mitral regurgitation (MR), and three had subaortic obstruction by the cleft. Associated cardiac lesions and extracardiac features were present in 13 and 10 patients, respectively. During the median follow-up period of 1.5 years (range 0 to 11.8), two patients died of extracardiac causes, and one neonate died of severe subaortic obstruction. Surgical repair was performed in 10 patients at a median age of 5.2 years (range 1.3 to 10.6). Multivariate analysis showed no predictors for MV surgery. One patient was re-operated for mitral stenosis associated with aortic valve stenosis. Follow-up echocardiography demonstrated moderate MR in two unoperated patients and moderate MV stenosis in two operated patients. ConclusionsA cleft of the MV comprises a wide spectrum. Important morphologic differences exist with an AV septal defect, although the two lesions may be pathogenetically related. Surgical repair always seems possible. Long-term echocardiographic follow-up is warranted.

A rare form of atrioventricular septal defect with severe subaortic stenosis

An unusual form of atrioventricular septal defect associated with severe subaortic obstruction is reported in a neonate who presented with intractable cardiac failure. The baby had a large defect in the atrioventricular septum allowing communication from the left ventricle to the right atrium, without interatrial or interventricular communication, and a cleft anterior mitral leaflet. The baby expired despite palliative surgery performed to bypass the subaortic stenosis.

SHOULD CONGENITAL HEART DISEASE BE A CONTRAINDICATION TO LIVER TRANSPLANTATION IN CHILDREN?

107 Significant congenital heart disease (CHD) has traditionally been considered a contraindication to pediatric liver transplantation. Cyanotic lesions, in particular, are believed to preclude successful outcomes. However, there is little in the way of data or case reports to support these beliefs. Since 1994, 7 infants and children with CHD have had a liver transplant (n=6) or are currently listed for liver transplantation (n=1) at our institution. We reviewed our experience with these patients to determine whether CHD is a risk factor for poor outcomes in pediatric liver transplantation. Three out of the 6 children with CHD who received liver transplants were less than 1 year of age at time of transplant, with a total age range at transplantation of 4 months to 9 years. Primary diagnoses of these children were divided evenly between extrahepatic biliary atresia (EBA;n=3/6) and Alagille's syndrome. Types of CHD were: pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (n=1), pulmonary artery stenosis (2), aortic coarctation (1), atrial septal defect (1) and hypertrophic obstructive cardiomyopathy with severe subaortic obstruction (1). Four of these patients had surgical repair (n=2) or transcatheter intervention (2) for CHD 1 to 6 months prior to liver transplantation, and 2 had surgical repair 11 months after transplantation. Three children received living related donor transplants. Early major complications after liver transplant included immediate graft failure in a single patient, who was successfully transplanted 4 days later, and a subhepatic hematoma in a different patient, necessitating exploratory laparotomy and drainage. The postoperative course following cardiac intervention was uncomplicated in all patients. Liver function tests were elevated in all patients who underwent surgical repair with cardiopulmonary bypass, but resolved to preoperative levels within one week. Five out of the 6 children with CHD who have received a liver transplant are alive and well at follow-up ranging from 12-48 months. The patient with hypertrophic obstructive cardiomyopathy expired 35 days after transplant from dehydration and septic shock. The single child with CHD currently listed for transplantation has EBA, is now 2 months of age, and is doing well following early surgical repair of his tetralogy of Fallot. In conclusion, our institutional experience indicates that children with a variety of forms of CHD, including cyanotic lesions, can undergo successful liver transplantation.

Aortopulmonary Fenestration with pulmonary artery septation for single ventricle and subaortic obstruction

A neonate with functional single ventricle and severe subaortic obstruction received a palliative procedure that involved a side-to-side anastomosis of the aorta and pulmonary artery, placement of a patch in the main pulmonary artery to divide the systemic and pulmonary circulations, and creation of a small opening in the patch to provide pulmonary blood flow. The patient recently underwent a bidirectional Glenn procedure at 10 months of age. This procedure obviates the need for a modified Blalock-Taussig shunt and may provide a more reliable source of blood to promote growth of both pulmonary arteries.

Interrupted Aortic Arch

Interrupted aortic arch (IAA) is often related developmentally to subaortic obstruction (SAO). When severe, SAO must be addressed in surgical management of IAA. From 1990 to 1993, 25 neonates presented for initial surgical management of IAA complexes. Associated lesions were ventricular septal defect (VSD) with or without atrial septal defect (19 patients), truncus arteriosus (3 patients), tricuspid atresia with transposition of the great arteries (1 patient), aortic atresia with VSD (1 patient), and d-transposition of the great arteries with VSD (1 patient). Overall hospital mortality was 20% (five deaths). One death was related to sepsis and two to sudden hemodynamic decompensation (a 2-kg premature infant after arch repair and VSD closure and a neonate with IAA-truncus arteriosus after arch repair and truncus repair with aortic root replacement). Two deaths were related to low cardiac output in patients with severe subaortic narrowing (< 3 mm by two-dimensional echocardiography), which was not addressed surgically. Of 10 additional patients judged preoperatively to have severe SAO, 1 underwent resection of the infundibular septum together with VSD closure and arch reconstruction, and 9 underwent a modification of Norwood's operation with arch reconstruction and proximal pulmonary artery to aortic anastomosis (7 with systemic to pulmonary artery shunts and 2 with right ventricle to pulmonary artery outflow tract reconstruction). One patient died 2 months after surgery of staphylococcal sepsis. All 9 others were discharged well. Subaortic narrowing is a physiologically important element of IAA complexes. When SAO is severe, satisfactory initial palliation can be achieved by a modification of Norwood's operation.

Transthoracic and transesophageal echocardiographic diagnosis of severe subaortic obstruction produced by an abscess cavity

1. Hollander JE, Quick G. Aortoesophageal fistula: a comprehensive review of the literature. Am J Med 1991;91:279-8’7. 2. Bogey, WM Jr, Thomas JH, Hermreck AS. Aortoesophageal fistula: report of a successfully managed case and review of the literature. J Vast Surg 1992;16:90-5. 3. von Oppell UO, de Groot M, Thierfelder C, Zilla P, Ode11 JA. Successful management of aortoesophageal fist& due to thoracic aortic aneurysm. Ann Thorac Surg 1991;52:1168-70. 4. West AB, Nolan N, O’Briain DS. Benign peptic ulcers penetrating pericardium and heart: clinicopathological features and factors favoring survival. Gastroenterology 1988;94:1478-87. 5. Fmelli FC, Piasio M, Mahfood S, Keshishian JM, Golocovsky M. Esophageal rupture complicated by a gastrocardiac fistula: review of the literature and report of a successfully treated case. Ann Thorac Surg 1989;48:582-3. 6. Grande JP, Pelliika PA, Edwards WD, Puga FJ. Cardiogastric fistula occurring 9 years after resection of left ventricular aneurysm. Int J Cardial 1990;27:327-31. 7. Bell MD, Tate LG, Hensley GT. Esophageal-atrial fistula resulting in systemic “meat and vegetable” emboli. Am J Forensic Med Path01 1992;13:137-41. 8. Mott JM, Austin GE. Cerebral embolization resulting from esophageal-atrial fistula. Arch Intern Med 1976;136:718-20. Transthoracic and transesophageal echocardiographic diagnosis of severe subaortic obstruction produced by an abscess cavity

Surgical Management of Patients with Interrupted Aortic Arch and Severe Subaortic Stenosis

Ten patients underwent palliative surgery for interrupted aortic arch and severe subaortic obstruction due to posterior displacement of the conal septum. Their ages ranged between 4 and 28 days (mean, 11.0 ± 7.7 days) and their weights, between 2.1 and 4.2 kg (mean, 2.85 ± 0.6 kg). Preoperative echocardiography and cardiac catheterization were performed on all patients. The ratios of the left ventricular outflow tract diameters and the ascending aortic diameters to the descending aortic diameters were 0.56 ± 0.03 and 0.56 ± 0.06, respectively, compared with 0.81 ± 0.12 and 0.95 ± 0.17, respectively, in 20 patients with interrupted aortic arch but without obstruction ( p < 0.001). Four of the 10 patients underwent pulmonary artery banding and insertion of a bypass graft between the ascending and the descending aorta. All 4 died of low cardiac output soon after operation (100% operative mortality). The remaining 6 patients underwent banding and insertion of a graft between the main pulmonary artery proximal to the band, and the descending aorta. All of these patients survived, and all except 1 are doing well 3 months to 4 years postoperatively. The use of a pulmonary artery-descending aorta conduit and of distal pulmonary artery banding provides good palliation for patients with interrupted aortic arch and major subaortic stenosis.

Surgical Management of Univentricular Heart with Subaortic Obstruction

In children with a univentricular heart and a rudimentary subaortic chamber, surgical relief of subaortic obstruction caused by a restrictive bulboventricular foramen is associated with high morbidity and mortality. A 6-year-old child with a univentricular heart of the left ventricular type, a rudimentary subaortic chamber, and atresia of the left-sided atrioventricular valve had pulmonary artery banding in infancy. Severe subaortic obstruction subsequently developed. At operation, the pulmonary artery was transected and the stump was anastomosed directly to the posterior aspect of the ascending aorta, diverting left ventricular blood into the aorta through the pulmonary valve. The distal pulmonary artery was anastomosed side-to-side to the ascending aorta to provide pulmonary blood flow. Cardiac catheterization fifteen months after the operation demonstrated an excellent hemodynamic result. When the pulmonary artery is adequate in size, a bypass operation by way of an anastomosis between the ascending aorta and the pulmonary artery is a relatively safe and effective means of relieving the ventricular outflow obstruction caused by a restrictive bulboventricular foramen.

Ventricular septal defect with silent discrete subaortic stenosis

Subaortic stenosis is rarely mentioned as a lesion that may be associated with a ventricular septal defect. We have encountered 4 patients with discrete subaortic stenosis adjacent to a ventricular septal defect, all of whom posed significant problems in diagnosis. In all 4 patients the subaortic stenosis was silent clinically and in 3 cases the obstruction was also not detected at the initial cardiac catheterization and angiocardiography. In the latter 3 cases, after surgical closure of the ventricular septal defect, there was a loud systolic murmur initially thought to be due to a small residual ventricular septal defect. In time, the clinical findings became more typical of isolated subaortic stenosis. In each of these cases the obstruction was verified at cardiac catheterization with peak systolic pressure gradients of 145, 45, and 70 mmHg. During reoperation, a discrete subaortic shelf was found opposite the patch used to close the ventricular septal defect. In the 4th case, the subaortic stenosis was unsuspected by clinical evaluation but was diagnosed by echocardiography as well as at cardiac catheterization, and both the ventricular septal defect and subaortic stenosis were corrected at the initial operation. A discrete subaortic shelf situated adjacent to a ventricular septal defect may be "silent," producing minimal, if any, pressure gradient and may pose diagnostic difficulties. Failure to recognize such a shelf and to remove it at the time of surgical closure of the ventricular septal defect, may result in the creation of a severe subaortic obstruction.

Use of Hancock porcine xenografts in children and adolescents

Between January 1,1974 and December 31,1978,47 xenografts (8 aortic, 28 mitral, 4 tricuspid, 3 left atrioventricular valve in I-transposition of the great arteries and 3 aortic-mitral) were implanted in 44 patients, aged 10/12 to 20 5/12 years (median 12 2/12). All eight patients with aortic valve replacement survived and were followed up for 22.5 ± 10.4 months (mean ± standard error of the mean). At postoperative cardiac catheterization in three patients, the peak systolic ejection gradient ranged between 24 and 38 mm Hg and the valve area index between 0.71 and 1.15 cm 2/m 2. Among 26 patients who underwent mitral valve replacement, 5 died operatively and 2 died 2 months postoperatively, leaving 19 long-term survivors who were followed up for 26.2 ± 15.6 months. In the 11 patients who underwent postoperative cardiac catheterization, the mean diastolic gradient was 6.1 ± 2.7 mm Hg and the mean valve index 1.79 ± 0.76 cm 2/m 2. Severe subaortic obstruction, due to the prosthetic mitral valve, occurred in three of five patients less than 5 years old. Among four patients who underwent tricuspid valve replacement there were no operative deaths; complete heart block occurred in three. At postoperative catheterization in two patients, the mean diastolic gradient was 3 and 5 mm Hg, and the valve index 1.32 and 1.7 cm 2/m 2, respectively. Three patients with I-transposition of the great arteries who underwent replacement of the systemic atrioventricular valve were followed up for 12 to 37 months. At postoperative catheterization the mean diastolic gradient was 0 to 4 mm Hg and the valve index ranged from 1.68 to 1.88 cm 2/m 2. Both aortic and mitral valves were replaced in three children. One died at operation and one 6 months after operation. The survivor has complete heart block but is in New York Heart Association functional class I. Eight xenografts (6 mitral, 1 left atrioventricular valve in I-transposition of the great arteries and 1 aortic) failed, requiring replacement 22 to 68 months after implantation. All were calcified to varying degrees. Valve failure began presenting 22 months after implantation, and of 25 valves implanted for 20 months or longer, 8 have been replaced. These 8 patients were younger (10.3 ± 0.2 years) at initial valve replacement than the other 17 patients (15.0 ± 0.3 years) followed up for 20 months or longer (p <0.05). High levels of γ-carboxyglutamic acid (GLA), a calciumbinding amino acid, were found in all six calcified xenografts analyzed, whereas none was found in a fresh porcine aortic valve or in an unimplanted xenograft. GLA-containing protein, laid down after implantation, may play a role in xenograft calcification.