, Volume 34, Issue 4, pp 1057-1058
Date: 18 Nov 2012

Pulmonary Atresia With Restrictive Ventricular Septal Defect: A Rare Association

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Clinical Case

A 14-day-old female baby (weight 3.1 kg) with an echocardiographic diagnosis of pulmonary atresia and restrictive ventricular septal defect (VSD) was referred to us for surgical treatment. The baby was intubated in discrete general conditions. On echocardiography, the right ventricle was severely hypertrophied and hypokinetic with infundibular hypoplasia. Because of she had moderate tricuspid insufficiency, a sovra-systemic right-ventricular (RV) systolic pressure was calculated. The foramen ovale was patent with right-to-left shunt. The aortic arch was right-sided with a large, tortuous, patent ductus arteriosus–to–right pulmonary artery communication under prostaglandin. No significant collateral vessel were noted.

A peculiarity of this case was the restrictive VSD. In fact, the typical malalignment VSD was almost completely obliterated by aneurismatic accessory tissue allowing only for a tiny right-to-left shunt (Figs. 1, 2, Video 1). The main pulmonary artery was mildly