Tricuspid valve avulsion or excision for right ventricular decompression in pulmonary atresia with intact ventricular septum.

Retrograde right ventricular decompression through the tricuspid valve, by damaging or excising the valve, was attempted in five patients with pulmonary atresia, intact ventricular septum, and severely hypoplastic right ventricle. Two patients were neonates in critical condition, one of whom had received previous palliation with a shunt; the other neonate had received pulmonary valvotomy plus shunt followed by repeat valvotomy plus shunt ligation. Three patients were infants or children who had undergone placement of isolated neonatal shunts. One procedure was performed during cardiac catheterization. Three patients survived. In one, tricuspid avulsion and a Rashkind septostomy were done by percutaneous methods; in two patients, tricuspid excision plus bidirectional cavopulmonary anastomosis was done by open operation. At restudy, all showed subsystemic pressure in the right ventricle and diminution or disappearance of sinusoids. One also showed improvement of left ventricular function. Incorporation of a small right ventricle in the right atrium at the time of the two orthoterminal palliations seemed, at least, innocuous. Right ventricular decompression by tricuspid avulsion or excision could be suggested for all patients with pulmonary atresia, intact ventricular septum, hypersystemic hypoplastic right ventricle, and major sinusoids without right ventricular dependent coronary circulation in whom the outlet portion of the right ventricle is not functional.