Membranous Pulmonary Atresia Treated by Radiofrequency-Assisted Balloon Pulmonary Valvotomy
A full-term neonate presented with systemic arterial hypoxia at 12 hours of age. Examination revealed a single second heart sound preceded by a holosystolic murmur. The ECG and chest X-ray demonstrated right atrial enlargement. Cross-sectional echocardiography demonstrated severe right ventricular hypertrophy and mild high-velocity tricuspid regurgitation. The pulmonary valve was normally formed and mobile, with a membrane connecting the leaflet tips, creating functional atresia (Figure 1 and Figure 2⇓). At cardiac catheterization, the diagnosis was confirmed and the presence of ventriculocoronary communications excluded. A 0.025-inch radio-frequency wire was introduced into a 5Fr right coronary catheter positioned in the right ventricular outflow tract directly beneath the atretic pulmonary valve, and 25W of energy delivered until the wire perforated the valve. Subsequent balloon pulmonary valvotomy was performed with 3-mm and 8-mm balloons. After the procedure, angiography and echocardiography demonstrated antegrade flow from right ventricle to pulmonary artery (Figure 3 and Figure 4⇓). Right ventricular hypertrophy and diastolic dysfunction necessitated a systemic to pulmonary artery shunt to augment pulmonary blood supply. The child was discharged 5 days later and remains well at follow-up. Serial fetal echocardiography has demonstrated a progression of pulmonary valve stenosis to functional atresia, and we believe this patient represents the point of transition.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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