[Right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular . About three patients without coronary ostium atresia. Is a transient percutaneous decompression maneuver necessary?].

Objective: Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart disease characterized by a wide morphological spectrum that can be associated with abnormalities in the coronary circulation such as sinusoids, fistulas, stenosis or atresia. Some patients do not present ventriculo-coronary fistulas or intramyocardial sinusoids, other patients do present ventriculo-coronary connections, but only some of the latter will have right ventricle-dependent coronary circulation (RVDCC); timely establishment of the diagnosis of RVDCC is essential, since the prognosis of these patients is generally fatal. There are reports of patients with this type of coronary circulation (RVDCC) who were undergone to univentricular physiology, but this treatment option remains controversial, so the purpose of this manuscript is to share three cases with PA-IVS and RVDCC, their outcome and the reflections they provide us.