Coincidence of long QT syndrome and propionic acidemia.

Propionic acidemia and long QT syndrome (LQTS) are rare disorders. In addition, both conditions are potentially lethal. The patient presented in this article was initially diagnosed with propionic acidemia. Incidentally, she was found to have LQTS on electrocardiogram and verified by stress test and epinephrine challenge. Although the patient was asymptomatic and arrhythmia free, we started her on atenolol. This is the first report of the association between LQTS and propionic acidemia.

Cardiac function in fetuses and newborns exposed to low-dose aspirin during pregnancy.

OBJECTIVE: Our purpose was to evaluate cardiac function in fetuses exposed to low-dose aspirin. STUDY DESIGN: To assess its efficacy in preventing preeclampsia, 60 mg/day aspirin or a matching placebo was administered to women in a double-blind fashion during the second and third trimesters. As a part of this study, 146 echo Doppler studies were performed on 63 fetuses from 15 to 40 weeks' gestation. These studies allowed assessment of systolic and diastolic cardiac function. Cardiac function was also assessed in 87 neonates. RESULTS: Throughout gestation ductus arteriosus flow velocity, right ventricular output and diastolic area, and left ventricular output and diastolic area all increased and were similar in both aspirin- and placebo-exposed fetuses. Fetal diastolic flow velocities were also alike in both groups. The two groups had similar gestational ages at delivery (39.2 +/- 2.3 vs 38.7 +/- 2.7 weeks) and birth weights (3174 +/- 575 vs 3105 +/- 579 gm, respectively). Furthermore, there was no difference in fractional shortening, cardiac output, prevalence of tricuspid regurgitation, or patency of the ductus arteriosus at birth between the two groups. CONCLUSION: The circulation of fetuses and newborns is not affected adversely by exposure to low-dose aspirin during gestation.

Discrete subaortic stenosis after successful treatment of congenital aortic valve stenosis.

Two cases of discrete subaortic obstruction which developed in a previously normal left ventricular outflow tract of patients with congenital valvar aortic stenosis are described. These examples emphasize the need for careful scrutiny of the etiology of recurrent postoperative left ventricular outflow tract obstruction.

Interventional catheterization in congenital heart disease.

The field of interventional catheterization in congenital heart disease has seen an explosion in application and scope over the past 10 years. In some lesions, such as valvar pulmonic stenosis, the transcatheter approach has replaced surgery as the treatment of choice. Applications currently considered experimental will no doubt become standard therapy as experience is gained with their use. Being able to avoid or postpone surgery has emotional, aesthetic, and practical advantages to patients and their families. The role of the catheter interventionist will continue to grow as new technologies such as fiberoptics, lasers, and miniaturized electromechanical devices are incorporated.

Acquired left ventricular to coronary sinus fistula: an unusual complication of acute myocardial infarction.

Anatomical complications of myocardial infarction include ventricular septal defect and mitral regurgitation. Another unusual complication of myocardial infarction is described, and its diagnosis and surgical management are discussed.

Balloon and blade atrial septostomy facilitated by two-dimensional echocardiography.

Two-dimensional echocardiography (2-D echo) was used as an imaging device for the performance of balloon atrial septostomy (n = 25) and blade atrial septostomy (n = 3). Biplane fluoroscopy with 2-D echo or 2-D echo alone were used in the cardiac catheterization laboratory to place the balloon or blade septostomy catheter in the left atrium. Echocardiography confirmed the position of the septostomy catheter before pullback. Balloon inflation and balloon or blade pullback to the right atrium was performed with 2-D echocardiographic visualization. In 8 critically ill newborn infants, balloon atrial septostomy was done in the neonatal intensive care unit without flouroscopy. The safety and efficacy of 2-D echo to assist balloon or blade atrial septostomy in the catheterization laboratory or as the sole imaging device in the neonatal intensive care unit are confirmed. Use of the subcostal 4-chamber and sagittal views to image cardiac anatomy and catheter position during the procedure are emphasized. Two-dimensional echo is particularly helpful in cases of cardiac malposition and during transseptal puncture of the atrial septum before blade septostomy, and lowers radiation dose when used in conjunction with fluoroscopy.

An unusual complication of a conduit-mounted homograft: valve leaflet fixation in the open position.

Surgical management of left ventricular outflow tract obstruction in patients with complete transposition of the great arteries and an intact ventricular septum is difficult. Direct resection of subpulmonary stenosis has not produced satisfactory results. Thus, relief of stenosis has been accomplished with valved conduits from the left ventricle to the pulmonary artery. Initially, conduit tissue valves were porcine in nature. More recently, human-origin conduit tissue valves have been employed. In this report, we describe an unusual complication in a child with complete transposition in whom a homograft valve conduit was placed from the left ventricle to the pulmonary artery to relieve subpulmonary stenosis. The leaflets became fixed in an open position. Thus, the conduit was converted to a functionally nonvalved communication. The subpulmonary stenosis regressed, allowing egress from both the natural outflow tract and the conduit.

Experience with the Fontan procedure.

From 1975 to the present, 45 patients have undergone modifications of the Fontan procedure for complex congenital heart disease. There were 30 males and 15 females ranging in age from 2 to 38 years (mean 13 years). Primary diagnoses were tricuspid atresia in 19, univentricular heart in 24, and pulmonary atresia with intact ventricular septum in two. Right atrial-pulmonary arterial connections were performed in 32 patients, 11 with conduits (seven with valves and four without) and 21 by direct anastomosis with patch augmentation. Right atrial-right ventricular connections were made in 13 patients, six with valved conduits and seven without conduits. Follow-up ranged from 0.1 to 9 years, with a mean of 2.3 years. There were three early deaths (less than 30 days) (7%) and two late deaths (5%) in this series, all in patients with a univentricular heart. The late deaths were both related to venous hypertension. A venous assist device was used in eight patients in the immediate postoperative period and was effective in improving cardiac output and reducing fluid accumulation. Postoperative Doppler flow studies in 15 patients revealed biphasic pulmonary artery flow in all without distinction between the type of connection or the presence of a valve. Cardiac catheterization was performed in 16 patients a mean of 14 months postoperatively (range 1 to 42 months) and revealed a reduced cardiac index at rest. Exercise testing in eight patients demonstrated a marked rise in right atrial pressure with a reduced rise in the cardiac index, even in those without functional limitations. With a mean follow-up of 2.3 years, 78% of patients were in New York Heart Association Class I, 17% in Class II, and 5% in Class III. We conclude that the Fontan procedure is an excellent operation in carefully selected patients with tricuspid atresia and other forms of complex congenital heart disease.

Echocardiography in congenital heart disease.

The combination of M-mode and two-dimensional echocardiography provides precise anatomic and helpful functional information for most types of congenital heart defects. Great strides have been made in the past ten years in ultrasound technology and its applications. The examination of the congenitally deformed heart by echo methods is now an intrinsic component of patient evaluation; a remarkable array of important clinical decisions concerning such patients depends, at least in part, on the results of echocardiographic study.

Ultrasonography of mucocutaneous lymph node syndrome.

Clinical findings associate mucocutaneous lymph node syndrome (MCLS) with diffuse arteritis leading to the formation of large aneurysms. Ultrasound assists in the diagnosis of the associated aneurysms, and aids the clinical management of the patients.