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A Rare Shunt

Published:August 27, 2022DOI:https://doi.org/10.1053/j.jvca.2022.08.019
      A 12-DAY-OLD girl (weight: 3 kg; height: 50 cm) presented to the authors’ institution in respiratory distress. The child was tachypneic (70 breaths/min), with an arterial oxygen saturation of about 65%-to- 70% on 3 L/min oxygen flow through a nasal cannula, and had signs of severe heart failure (Fig 1). Transthoracic echocardiography revealed situs solitus, levocardia, atrioventricular and ventriculoarterial concordant connections, normal systemic and pulmonary venous drainage, membranous pulmonary atresia, and an intact ventricular septum. There was an atrial septal defect measuring 6 mm in diameter. The tricuspid valve was dysplastic with severe regurgitation (right ventricular systolic pressure 76 mmHg); the right atrium was grossly dilated; and the right ventricle was bipartite and enlarged. The patent ductus arteriosus was tortuous and measured 5 mm in diameter at the aortic end and 2.5 mm toward the pulmonary end. The main pulmonary artery was well-formed and measured 6 mm in diameter, the right pulmonary artery was 5 mm, and the left pulmonary artery was 4 mm. The child was taken to the cardiac catheterization laboratory for an urgent perforation of the doming pulmonary valve and transcatheter pulmonary valvuloplasty. After the pulmonary valvuloplasty, the arterial oxygen saturation did not improve (<75%) despite an increase in the inspired oxygen concentrations. Color Doppler blood flow demonstrated a torrential flow across the patent ductus arteriosus into the pulmonary artery (Fig 2 A, B, and C). The patent ductus arteriosus was occluded with a balloon for 10 minutes and the child tolerated this procedure without significant desaturation or hemodynamic compromise (Fig 3, Video 1). Transthoracic echocardiography before and after balloon occlusion of the patent ductus arteriosus demonstrated a decrease in the severity of the tricuspid regurgitation (Videos 2 and 3). A caudal displacement of the septal tricuspid leaflet also could be seen. The vena contracta of the tricuspid regurgitant jet prior to balloon occlusion was 0.768 cm, which was reduced to 0.438 cm after balloon occlusion of the patent ductus arteriosus (Fig 4). A continuous-wave Doppler signal interrogation of the main pulmonary artery blood flow showed a continuous flow pattern from the patent ductus arteriosus to the main pulmonary artery and right ventricle when the ductus was open, demonstrating a systolic and diastolic pulmonic regurgitation (Fig 5A, Video 4). When the ductus was occluded, antegrade flow across the main pulmonary artery because of right ventricular ejection, and retrograde flow because of the pulmonic regurgitation, were observed (Fig 5B, Video 5). What was the diagnosis?
      Fig 1
      Fig 1Chest radiograph displaying the gross enlargement of the cardiac silhouette.
      Fig 2
      Fig 2(A) Color Doppler flow map by transthoracic echocardiography displaying the torrential blood flow through the patent ductus arteriosus into the main pulmonary artery and the severe pulmonary regurgitation; (B) the magnitude of the tricuspid regurgitation; (C) and the right-to-left shunt across the atrial septal defect.
      Fig 3
      Fig 3Increase in systemic arterial pressures after balloon occlusion of the ductus arteriosus (white arrow).
      Fig 4
      Fig 4The vena contracta of the tricuspid regurgitant jet (A) prior to and (B) during balloon occlusion of the ductus arteriosus.
      Fig 5
      Fig 5Continuous wave signal interrogation of the pulmonary artery (A) prior to and (B) during balloon occlusion of the ductus arteriosus.

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