Anomalous origin of the right coronary artery from the pulmonary artery and mitral regurgitation in a newborn.

Anomalous origin of the right coronary artery (RCA) from the pulmonary artery (PA) is a rare congenital anomaly, and only 28 cases have been reported in the pediatric age group. We describe the case of an infant who had progressive mitral regurgitation and papillary muscle dysfunction in association with anomalous origin of the RCA from the PA. The diagnosis was made by color flow Doppler, confirmed by angiography, and the case was successfully corrected by reimplantation of the anomalous RCA to the aorta. This is only the second case of anomalous origin of the RCA from the PA diagnosed in infancy without an associated congenital anomaly of the heart and great vessels.

Congenital coronary artery fistulae: a review of 18 cases with special emphasis on spontaneous closure.

Between 1972 and 1990, 18 patients (median age 3 years, range 0.1-14 years) with coronary artery fistulae (CAF) were seen at this institution. Of the 16 patients without associated heart defects, two patients presented with congestive heart failure and the remaining 14 were asymptomatic. Fifteen patients had normal origin of two coronary arteries. Two patients had atresia of the proximal right coronary artery and, in one patient, the right coronary artery originated from the left main coronary artery. The QP/QS ranged between 1.0 and 2.8, with a mean of 1.4. Fifteen patients underwent operative closure without any deaths. One patient is being followed medically at present. Two patients showed spontaneous clinical improvement of CAF (complete closure in one and near complete in the other) and remained asymptomatic during a decade of follow-up. Review of the literature suggests operative closure of symptomatic and asymptomatic small CAF during childhood. However, the natural history of minute CAF remains unclear. In addition, symptoms from CAF may spontaneously improve with time.

Augmented norepinephrine and renin output in response to maximal exercise in hypertensive coarctectomy patients.

To evaluate a possible neural or renal contribution to the hypertension that occurs in some patients following coarctation of aorta repair, 35 patients underwent graded bicycle exercise with serial measurements of plasma norepinephrine concentrations and plasma renin activity. Sixteen patients with coarctectomy who had systolic or diastolic hypertension at peak exercise were compared with 19 normotensive patients with coarctectomy. The average time interval between coarctation repair and study was significantly longer (p less than 0.05) in the hypertensive group than in the normotensive patients (12.8 +/- 4.8 versus 8.7 +/- 2.2 years). The heart rate response to exercise was similar for both patient groups. The systolic blood pressure in the hypertensive group was higher than in the normotensive group at rest in the supine and upright positions and at 5 minutes of recovery, in addition to peak exercise, and the diastolic blood pressure was increased at peak exercise. Plasma norepinephrine concentrations were significantly higher at peak exercise and during recovery in the hypertensive group than in the normotensive patients. Plasma renin activity was also significantly higher in the hypertensive group at peak exercise. These data suggest that patients with coarctectomy who have a hypertensive response to exercise have an augmented sympathetic nervous system output and increased plasma renin activity that may lead to peripheral vasoconstriction at peak exercise and that may contribute to the development of their hypertension.

Focal scleroderma and severe cardiomyopathy. Patient report and brief review.

A 21-month-old infant presented with simultaneous localized scleroderma and severe cardiomyopathy with heart failure. Cardiac abnormalities and serological changes (positive rheumatoid factor assay, elevated IgM and IgG levels, and elevated erythrocyte sedimentation rate) reverted to normal with prednisone therapy, and there was substantial, though incomplete, resolution of her skin changes during the same period. To our knowledge, this is the first patient with definite, clinically significant cardiac involvement associated with focal scleroderma. The possibility of internal organ involvement, including cardiac involvement, must be considered with focal scleroderma as well as with progressive systemic sclerosis.

Down's syndrome, complete atrioventricular canal, and pulmonary vascular obstructive disease.

We reviewed our experience over a 10-year period to determine whether children with Down's syndrome and complete atrioventricular canal develop pulmonary vascular obstructive disease earlier than children with normal chromosomes and this defect. Comparisons were made between Down's syndrome and normal chromosome children regarding (1) pulmonary blood flow and pulmonary vascular resistance at initial catheterization, (2) operability as related to elevation in pulmonary vascular resistance, and (3) age at diagnosis of fixed pulmonary vascular obstructive disease. The 45 patients with Down's syndrome catheterized under 1 year of age had a lower mean pulmonary blood flow (3.2 versus 5.7; p = 0.0001) and higher mean pulmonary vascular resistance (8.3 versus 4.6 Wood units.m2; p = 0.0003) than their 34 normal chromosome counterparts. When all ages were included, 38 of 81 (47%) of the children with Down's syndrome and 32 of 40 (80%) of the normal children were considered operable. Non-Down's syndrome patients who had operations had a higher pulmonary blood flow (5.8 versus 3.3; p = 0.004) and lower pulmonary vascular resistance (3.6 versus 6.0 Wood units.m2; p = 0.005) than Down's syndrome patients. Of the 34 patients who did not have operations because of pulmonary vascular obstructive disease, 31 had Down's syndrome. In 10 of 81 children with Down's syndrome, fixed pulmonary vascular obstructive disease was diagnosed before the age of 1 year, while this was found in none of 40 normal children. Our data demonstrate that Down's syndrome patients with complete atrioventricular canal have a greater degree of elevation of pulmonary vascular resistance in the first year of life and more rapid progression to fixed pulmonary vascular obstructive disease than children with normal chromosomes.

Exercise performance after repair of anomalous origin of the left coronary artery from the pulmonary artery.

Eleven patients underwent exercise testing after operative repair of anomalous origin of the left coronary artery from the pulmonary artery. Five patients repaired after 2 years of age comprised a childhood surgery group, and six patients repaired before 2 years of age comprised an infant surgery group. All patients were exercised using either a treadmill or electronically braked bicycle with simultaneous thallium 201 scintigraphy. Oxygen consumption, carbon dioxide production, pulmonary functions, and electrocardiogram were all monitored continuously. Pulmonary reserve was normal in all patients. Based on heart rate reserve, respiratory exchange ratio, and oxygen-consumption response to work load, two patients in the infant surgery group stopped exercise before achieving maximum aerobic capacity. All remaining patients achieved their maximum aerobic capacity. There was no difference in work rate or oxygen consumption during exercise between the infant and childhood surgical group. Four patients (two in each surgical group) had an impaired chronotropic response to exercise. Three of these four patients demonstrated perfusion defects by thallium scintigraphy. Thallium scintigraphy was normal in all remaining patients. Electrocardiographic abnormalities were noted in seven of 11 patients having ventricular arrhythmias or ST segment depression. It is concluded from this study that exercise performance after repair of anomalous origin of the left coronary artery from the pulmonary artery is not affected by the age at which surgery is performed. Exercise is frequently associated with electrocardiographic evidence of abnormal myocardial perfusion despite frequently negative simultaneous 201Tl scintigraphy.

Intracardiac teratoma in a newborn.

Primary tumors of the heart are infrequent at all ages. We present a newborn with hypoxia and a heart murmur, in whom an echocardiogram revealed a large tumor filling the right ventricle and the pulmonary annulus. To maintain pulmonary blood flow, the patency of the ductus arteriosus was achieved by infusion of prostaglandin E1. Successful surgical resection was accomplished. The pathological examination was characteristic of a benign teratoma. The patient remains asymptomatic and has shown no evidence of recurrence of the tumor during a follow-up period of 34 months. This represents the eleventh case of intracardiac teratoma and only the fourth case to undergo successful surgical resection.

Primary pulmonary artery sarcoma in two children.

The clinical and pathological features of primary pulmonary artery sarcoma in two children are reported. The first patient presented with right ventricular outflow obstruction and underwent successful surgical resection of his tumor. The second patient developed cardiac arrest following a relatively short period of symptoms of right heart failure and could not be revived. The pathological diagnosis in both patients was hemangiopericytoma. To the best of our knowledge, primary pulmonary artery sarcoma in children has not been previously reported.

Surgical and medical results of complete atrioventricular canal: a ten year review.

The files of 121 patients who presented to Children's Hospital of Michigan over the last 10 years with complete atrioventricular (AV) canal were reviewed to evaluate long-term management and overall outcome. Of 121 patients, 70 underwent corrective surgery, 21 (30%) of whom died perioperatively. The surgical mortality rate was 13% when patients with hypoplastic left or right ventricle (n = 6), double-orifice mitral valve or extreme deficiency of mitral tissue (n = 5), and pulmonary vascular obstructive disease (n = 5) were excluded. Of the 49 patients who survived operation, 36 are in New York Heart Association class I, 1 patient requires a pacemaker and 3 died late. In 34 of the 51 patients (28%) who did not undergo operation, pulmonary vascular obstructive disease developed; it occurred within 12 months in 10 patients (8%). Eight other patients who did not undergo operation died before planned surgery (age 1 to 9 months). Although surgical prognosis in good candidates is acceptable, the overall prognosis for children with complete AV canal is guarded because of the risk of early death or early pulmonary vascular obstructive disease and frequently unfavorable anatomy.

Pacing in children and young adults with nonsurgical atrioventricular block: comparison of single-rate ventricular and dual-chamber modes.

A prospective comparison of physiologic response to single-rate ventricular and dual-chamber atrioventricular pacing was conducted in 14 pediatric patients (age 1 to 24 years, median 14) with symptomatic nonsurgical second- or third-degree atrioventricular block. All patients were studied acutely during cardiac catheterization before and after 1 hour of both pacing modes. Following pacemaker implant, eight patients were reevaluated after 1 month of each mode with symptom questionnaire, resting ECG, resting echocardiogram, and Doppler cardiac output measurement at rest and at peak treadmill exercise. Cardiac outputs (mean +/- standard error) increased acutely (n = 14) with both ventricular (32 +/- 12%) and dual-chamber (39 +/- 10%) pacing over intrinsic rhythm values (p less than 0.01 in both). During chronic pacing (n = 8), symptoms were reported only with the ventricular mode. Dual-chamber synchronous pacing was associated with improved mean resting shortening fraction and cardiac output, slower mean resting sinus rate (89 +/- 5 compared to 73 +/- 4 bpm (p less than 0.02), and a 23% increase in mean excerise cardiac output (4.2 +/- 0.4 compared to 3.4 +/- 0.3 L/min/m2) compared to single-rate ventricular pacing. Exercise-induced dysrhythmias occurred only with ventricular pacing. This study demonstrates that pediatric patients with nonsurgical atrioventricular block can compensate for loss of atrioventricular synchrony at rest but exhibit improved cardiac function with chronic dual-chamber atrioventricular compared to single-rate ventricular pacing.

Serial ECG measurements at controlled plasma levels of nortriptyline in depressed children.

The authors investigated ECG measurements in 21 children with major depressive disorder treated with nortriptyline at plasma levels of 50-100 ng/ml. P-R and QRS intervals and heart rate measured at baseline and once a week during treatment remained within guidelines.

Dose and plasma levels of nortriptyline and chlorpromazine in delusionally depressed adolescents and of nortriptyline in nondelusionally depressed adolescents.

Eight adolescents with major depressive disorder were treated with nortriptyline and six adolescents with delusional depression were treated with combined nortriptyline and chlorpromazine. Dose and plasma levels of nortriptyline for the two groups were compared. The delusional group receiving combined drug treatment needed significantly less nortriptyline than did the nondelusional group receiving only nortriptyline to obtain similar mean steady state plasma levels of the drug. The mean plasma chlorpromazine levels were quite low.

Noninvasive evaluation of the inferior vena cava. The value of sonography.

Children who have abnormalities of the inferior vena cava (IVC) appear with diverse signs and symptoms. Those with congenital IVC malformations have symptoms related to associated cardiac disease. Those with IVC compression or obstruction may have ascites, hepatomegaly, edema of the lower extremities, and/or signs of portal hypertension. Sonography of the IVC has become a routine part of the evaluation of congenital heart disease and also part of the examination of patients with abdominal masses. Sonography should be the initial imaging modality in children at high risk for caval disorders. Previously used diagnostic procedures such as the inferior venacavogram should now be used only as a secondary investigative tool.

Asymptomatic intracardiac metastasis from osteosarcoma: a case report with literature review.

Osteosarcoma very rarely metastasizes to the heart. Thirteen cases have been reported in the literature so far. Diagnosis in some of these cases was made during investigation for severe cardiac failure and in most of them at autopsy. Our patient, a 13-year-old girl, showed right pulmonary metastases on chest x-ray 1 year after above knee amputation for osteosarcoma of the distal femur. Routine preoperative computerized axial tomography (CT Scan) revealed a calcified lesion in the heart in addition to the pulmonary metastases. She was very active and completely asymptomatic. Two-dimensional echocardiography, angiography, and right and left heart catheterizations were done. This revealed a large mobile metastatic lesion in the right ventricle. The intraventricular tumor was successfully removed, and 12 days later she had a second thoracotomy for removal of pulmonary metastases. Nine months after her intraventricular metastasis was removed she developed a solitary right pulmonary metastasis. This was successfully resected. Now, 10 months later, she is disease free and completely asymptomatic.