Hemodynamic effects of prostaglandin E1 in patients with congenital heart disease and pulmonary hypertension.

Investigations were performed to determine whether prostaglandin E1 (PGE1) would cause a favorable change in pulmonary hemodynamics in pediatric patients with congenital heart disease and elevated pulmonary vascular resistance. Twenty-seven patients (ages 0.5 months to 19 years) were studied. PGE1 caused a significant decrease in mean pulmonary artery pressure with 0.05 microgram/kg/min and 0.1 microgram/kg/min infusions (p = .0004). There were significant increases in pulmonary blood flow (p = .0007), and decreases in pulmonary vascular resistance (p = .0001); changes in systemic pressure, resistance, oxygen consumption, and heart rate were minimal. Twenty-three patients underwent heart surgery after catheterization, nineteen of these survived. The four nonsurvivors had higher initial pulmonary resistance (PVR) and less decline in PVR with PGE1 than survivors. This study shows that PGE1 is an effective pulmonary vasodilator in patients with congenital heart disease and elevated pulmonary vascular resistance.

Exercise echocardiography in the detection of anthracycline cardiotoxicity.

Twenty long-term survivors of childhood cancer underwent exercise echocardiography to evaluate possible late anthracycline-induced cardiac toxicity. Ten patients ages 10 to 20 years had received anthracyclines, and ten patients ages 8 to 27 years had not received anthracyclines as part of their medical regimen. Both groups had normal cardiac function at rest. Patients who had not received anthracyclines had a greater increase in M-mode shortening fraction (P less than 0.005), velocity of circumferential fiber shortening (P = 0.05), and Doppler aortic peak flow velocity (P = 0.01) than patients receiving anthracyclines. There were no significant differences in work performed, or increase in heart rate or blood pressure with exercise between the groups. These results suggest that subtle abnormalities in myocardial function exist which become apparent only after exercise in survivors of childhood cancer who have received anthracyclines and have normal resting cardiac function.

Hypoplastic left heart syndrome: palliation without cardiopulmonary bypass.

Our 100% mortality rate with first-stage palliation of hypoplastic left heart syndrome performed with cardiopulmonary bypass led us to a procedure not necessitating bypass. In nine neonates with this congenital heart defect, a woven Dacron graft was placed from the main pulmonary artery to the descending thoracic aorta. The patent ductus arteriosus was ligated and the main pulmonary artery banded distal to the graft and proximal to the bifurcation. Five patients were extubated within 4 days. Only low-dose inotropic support was required in eight of the nine. There were no bleeding problems. Four patients died in the hospital: one of Candida sepsis at 81 days, one of low cardiac output at 2 days, and two of restrictive atrial septal defect at 3 and 5 days. The five living patients were discharged 11 to 80 days postoperatively (mean 38 days). We now perform balloon septostomies preoperatively in all patients and believe that this will improve the survival rate. We believe this simpler approach to the treatment of hypoplastic left heart syndrome may allow survival for a cardiac transplant or a staged Fontan procedure at a later date for more definitive treatment.

Determination of the time of closure of the ductus arteriosus in severely ill premature infants.

In order to investigate the time of ductal closure in the premature infant, we performed multiple echocardiographic examination in each of 42 premature infants. Twenty eight of these infants had spontaneous closure of the ductus arteriosus (median date of closure--3 days). Twelve required medical or surgical closure of the ductus arteriosus and two died of severe lung disease. There was a significant relationship of decreasing birth weight to prolonged patency of the ductus arteriosus.

Prophylactic treatment of asymptomatic patent ductus arteriosus in premature infants with respiratory distress syndrome.

Early treatment of premature infants with indomethacin has been proposed as a means of reducing the morbidity associated with respiratory distress syndrome complicated by symptomatic patent ductus arteriosus. We identified 26 infants less than 48 hours old with severe respiratory distress syndrome who had an asymptomatic patent ductus arteriosus. These infants were treated with either indomethacin or placebo. There was a significant difference in the frequency of ductal closure after receiving indomethacin treatment. No significant difference was observed in the time required for mechanical ventilation, time receiving supplemental oxygen, or time in the hospital, and there were no significant differences in the incidence of bronchopulmonary dysplasia, intraventricular hemorrhage, retinopathy of prematurity, or death between the two groups.

Hemodynamic and echocardiographic evaluation of the stumptailed macaque: a potential nonhuman primate model for pulmonary vascular disease.

Stumptailed macaques (Macaca arctoides) were evaluated as to their suitability as an animal model for pulmonary hypertension. Animals used for this study were colony-bred. Necropsy material from 63 animals revealed 32% with severe medial thickening of pulmonary arteries. Twenty-nine stumptailed macaques underwent cardiac catheterization and M-mode echocardiography. Hemodynamic measurement including pulmonary artery pressure response to 12% oxygen exposure identified three groups of animals with elevated, normal, and intermediate pulmonary artery pressures. Stumptailed macaques with elevated pulmonary artery pressure could be distinguished from other animals by echocardiography.

A persistent fifth aortic arch in man: a double-lumen aortic arch (presentation of a new case and review of the literature).

Persistent fifth aortic arch manifested as a double-lumen aortic arch (DLAA) is a rare anomaly. We present such a case with transposition of the great arteries, ventricular septal defect (VSD), and pulmonary atresia. Review of the eight previously published cases indicates relatively frequent association of DLAA with preductal coarctation of the aorta. We attempt to explain the coexistence of this lesion with DLAA. We also discuss evidence supporting derivation of the inferior channel of the DLAA from the embryologic fifth aortic arch.

Gastroesophageal reflux in association with congenital heart disease.

The clinical course of 19 infants with severe respiratory symptoms associated with the presence of both congenital heart disease and gastroesophageal reflux is described. Down Syndrome or central nervous system disease was present in 12 of the 19 infants. The identification of reflux as a major or additional cause of the respiratory complications was often overlooked. Medical therapy alone was successful in only one of the 19 patients. Early repair or palliation of the cardiac malformation with or without subsequent antireflux surgical procedure was associated with relief of the symptoms in 13 patients.

Oxygen-induced alterations in lung vascular development in the newborn rat.

Newborn rats were exposed to air or hyperoxic conditions for the first 6 days of life. Resulting effects on the pulmonary vascular bed were determined by analysis of barium angiograms, scanning electron microscopy of methylmethacrylate corrosion casts and whole lung, morphometric estimations of pulmonary arteries/area and capillary number/area, and arterial blood gas measurements. Similar studies were also performed on the lungs of animals allowed to recover in air for 1 and 2 wk. Although the general pattern of the pulmonary arterial bed by barium angiograms appeared similar, diminished branching or underfilling of the distal arterial segments was more frequently encountered in hyperoxic-exposed animals. Morphometric examination and corrosion casts revealed differences in vascular pattern and density between hyperoxia and air-exposed animals. The number of capillaries/mm2 of lung tissue was less in hyperoxic-exposed pups than controls after 6 days of exposure to hyperoxia but markedly increased to slightly above control levels by 2 wk of air recovery. The number of 20--50 micrometers size vessels/mm2 followed a similar pattern of change. Corrosion casts of lung exposed to 6 days of hyperoxia revealed less microvascular density compared to air controls, but after 1 wk recovery in air, hyperoxic-exposed animal had a more extensive network of microvessels. Maximum PaO2 attained by animals in the various groups closely resembled the patterns of change in microvessel density. These findings support the thesis that a major alteration of lung vascular growth and development occurs subsequent to exposure of the newborn to hyperoxia.

Use of balloon-tipped catheters in the critically ill child.

A retrospective study of our experience in the placement of 19 consecutive balloon-tipped catheters in the pulmonary artery of 18 children disclosed that the procedure can be performed with relative ease in the intensive care unit without the aid of fluoroscopy. Insertion of the catheters was not associated with any serious complications. Catheter malfunction, however, occurred in 9 of 18 patients: balloon rupture in 6 and clot formation in 3. Comparison of pulmonary capillary pressure through a balloon-tipped catheter and venous pressure through a central venous line indicates that, in the absence of significant pulmonary disease requiring high positive end expiratory pressure, or significant left heart dysfunction, a central venous pressure line is frequently adequate for monitoring right heart pressures and as a guidance to fluid therapy.

The dreams of college men and women in 1950 and 1980: a comparison of dream contents and sex differences.

Two samples of dreams collected from college students in 1950 and 1980 under similar conditions were analyzed using some of the Hall-Van de Castle scales. It was found that there has been little change over a period of 30 years in what college students dream about. Moreover, the sex differences in the 1980 dreams are the same as those in the 1950 dreams.

Porcine xenograft valve failure in children: an immunologic response.

Thirty-six children underwent placement of either an intracardiac porcine xenograft valve (n = 17) or an extracardiac porcine xenograft-valve conduit (n = 20). Eleven of 17 patients with intracardiac porcine valves and only one of patients with 20 extracardiac porcine valved conduits had severe hemodynamic valvular changes (p less than 0.01). Twelve of 17 of the intracardiac xenograft patients had symptoms: eight had congestive heart failure, one had angina and three had exercise intolerance. The only extracardiac conduit patient with hemodynamic changes had congestive heart failure. Calcification was noted in 11 of 18 intracardiac porcine valves and in none of the extracardiac valved conduits. Pathologic valve material was available in nine of 18 intracardiac porcine valve patients and disclosed extensive ingrowth of host tissue in eight valves, histiocytic infiltrates in all nine valves, plasma cell infiltrates in three valves and positive immunofluorescence for immunoglobulin in all three valves. Three of 17 patients with intracardiac porcine xenograft valves had increased plasma immunoglobulins and one patient had bone marrow plasmocytosis. Our data suggest that children have a high incidence of early xenograft dysfunction only when intracardiac porcine valve replacement is performed. The infiltration of plasma cells and histiocytes and the immunoglobulin response in children with intracardiac glutaraldehyde-preserved porcine valves suggest the xenograft dysfunction may in part be due to an immunologic reaction.