Palliative surgery for a borderline functional single ventricle patient with absent pulmonary valve syndrome.

Abstract

  Absent pulmonary valve syndrome (APVS) is a rare entity of congenital cardiac anomaly, and is often associated with tetralogy of Fallot. APVS associated with functional single ventricle (fSV) is even rarer. Respiratory tract compression due to pulmonary artery dilation is a severe complication for patients with APVS. fSV patients cannot tolerate worsening respiratory status due to respiratory tract compression, since maintaining good respiratory status is essential for Fontan circulation. We present a case with an APVSfSV patient with severe respiratory tract compression symptoms who improved after cardiac surgery, including bilateral pulmonary artery angioplasty and pulmonary blood flow reconstruction using Blalock-Taussig shunt.