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We observed 10 children with bronchopulmonary dysplasia, evaluated initially by cardiac catheterization (mean age 18 months), for an average of 4.4 years. Age at last evaluation averaged 5.8 years; subjects reside in and around Albuquerque, N.M. (altitude 5000 ft). At initial cardiac catheterization, mean pulmonary artery pressure was 40 mm Hg, pulmonary vascular resistance index 8.9 units, and intrapulmonary shunt fraction was high; pulmonary wedge angiograms were normal. Over the period of follow-up the group has done poorly. Four of the 10 continue to receive home oxygen therapy, but none requires inotropic or diuretic therapy; four children have marked developmental or motor delays. Nine of 10 patients have abnormalities of respiratory function on spirometric testing. Four patients underwent recatheterization because of clinical indications; two had large atrial level left-to-right shunts not found on initial study. Reductions in pulmonary artery pressure (55 to 37 mm Hg) and pulmonary vascular resistance (11.9 to 7.8 units) occurred between the two studies in these four patients (average study interval 4.0 years); the still elevated levels of pressure and resistance fell further in response to 40% O2 administration. Pulmonary wedge angiograms were abnormal in each restudied patient. Although not uniformly bleak, the long-term outlook for children with severe bronchopulmonary dysplasia is diverse and guarded.

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A 5 MHz range-gated portable pulsed Doppler velocity meter was used to measure mean ascending aortic blood flow velocity noninvasively. Studies were performed from a suprasternal approach in 18 preterm infants with patent ductus arteriosus. Measurements were made in each patient before and after medical or surgical closure of the PDA. The internal ascending aortic diameter was determined echocardiographically and aortic cross-sectional area calculated according to the equation AAo = pi d2/4. Ascending aortic blood flow was computed as QAo (ml/min) = VAo (cm/sec) x AAo (cm2) x 60 (sec/min). Prior to PDA closure, QAo averaged 343 ml/min/kg, well above predicted normal values. After PDA closure, QAo fell to 252 ml/min/kg, significantly lower than the preclosure level (P less than 0.001), but slightly higher than the mean cardiac output of healthy newborn infants. The mean QAo after surgical ligation of the PDA was closer to the predicted normal value than after treatment with indomethacin alone. This study reflects the effect of left-to-right ductus shunting on left ventricular output and emphasizes the demands placed on the neonatal left ventricle by PDA.

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Mean blood flow velocity (VAo) in the ascending aorta was measured noninvasively in 33 children, ages 3 days to 17 years, by pulsed Doppler technique at the time of cardiac catheterization. Measurements were made from a suprasternal approach with a portable, range-gated device. The ascending aortic diameter was determined echocardiographically and aortic cross sectional area calculated (A = pi d2/4 cm2). Aortic flow (QAo) was computed from Doppler recordings: QAo (ml/minute) = VAo (cm/second) X A (cm2) X 60 (second/minute). Values were compared with cardiac outputs determined according to the Fick principle, using measured oxygen consumption calculated oxygen capacity, and oxygen saturation. Subjects with aortic valve abnormalities or left ventricular outflow tract obstruction were excluded from study. Agreement between the two methods was excellent (linear regression r = 0.98, slope = 1.07, y-intercept = -4.5 ml, range 403 to 5.540 ml/minute). The Doppler technique is a quick, noninvasive, and accurate method of determining ascending aortic blood flow in neonates and children.

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Digoxin therapy was evaluated retrospectively in a group of 30, and prospectively in a group of 16 low-birth-weight, premature infants with cardiorespiratory symptoms due to persistent patency of the ductus arteriosus. The response to decongestive therapy was equivocal. Digoxin levels in serum varied between 1.5 and 13 ng/ml. Digoxin half-life in serum exceeded three days in four patients. Fourteen of the combined group of 46 infants had signs of toxicity of digoxin. High dosage, inadvertent overadministration, and variable clearance of digoxin, as well as special characteristics of the patients studied, are postulated as explanations for the high incidence of toxicity. This study suggests that digoxin therapy in low-birth-weight, premature infants with patent ductus arteriosus is not without risk.

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Recent experience with implanted cardiac pacemakers in nine young patients is reported. Advance in technology include improved pacemaker generator design and placement, and improved lead design and placement. No patients died; our only complication, failure to capture, required repositioning of the lead. All pacemaker replacements were elective. This series helps to define the more favorable current prognosis for the young patient needing pacemaker therapy.

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Intracranial pressure has been monitored in ten patients with Reye syndrome. All of the patients were comatose and all were treated with dexamethasone, mannitol, hyperventilation, and multiple exchange transfusions. Five of these children are alive and well; five died. In each case ICP monitoring allowed for immediate treatment and evaluation of the mode of therapy used to reduce elevated ICP. Exchange transfusions in all patients either reduced the increased ICP or made the pressure rises easier to manage. ICP is not always reflected by lumbar spinal fluid pressure or clinical state. In our hands, ICP monitoring has proved to be a safe technique for the investigation of the significance of raised ICP in the outcome of patients with Reye syndrome and of the effectiveness of a chosen therapy in reducing this pressure.

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Four infants with skin rash, hepatosplenomegaly, lymphocytosis, eosinophilis, and histiocytic infiltration of the lymph nodes and skin are described; in each of these infants an initial diagnosis of the Letterer-Siwe syndrome was made. Postmortem findings of thymic dysplasia and poorly differentiated, lymphopenic peripheral lympoid tissue in each of the four infants, as well as antimortem clinical findings in one, established a diagnosis of severe combined immunodeficiency. From these and similar cases in the literature, we postulate that the Letterer-Siwe syndrome may not be an unusual presentation of combined immunodeficiency. Appropriate immunologic studies will help to differentiate the two disorders.

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