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

Abstract

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.

Method: We conducted a cross-sectional, descriptive, observational study of patients with PA-IVS and RVDCC without atresia of the coronary ostium during the study period.

Results: Three patients were identified, the clinical and angiographic characteristics and their evolution are described, and a new technique or maneuver for transient percutaneous decompress of the right ventricle is described theoretically for those cases where there is doubt about RVDCC during the angiographic study.

Conclusions: An accurate diagnosis regarding the existence of RVDCC is vital. In some cases, the interpretation of angiography for the diagnosis of RVDCC can be difficult to pinpoint and in many cases, it can be operator dependent, however we consider that is necessary to have a technique or maneuver that can objectively and without doubts determine RVDCC in those cases where angiography is not totally conclusive and thus be able to offer the best therapeutic option.