Marcely Gimenes Bonatto et al. Ana Karyn Ehrenfried de Freitas Letícia dos Santos de Oliveira Rocha Claudinei Collatusso … Rafael de Almeida Torres Gustavo Gavazzoni Blume Lídia Zytynski Moura
Introduction
Anomalous pulmonary vein dreinage (APVD) is a rare disease, accounting for nearly 1 to 3% of congenital heart diseases. It may be total, when all PVs are involved, or partial, when only some PVs drain anomalously into one or more systemic veins or directly into the right atrium (RA)., This condition occurs alone or in association with an interatrial septal defect, more often sinus venosus interatrial communication (IAC) (which accounts for nearly 5% of all IAC cases).
Patients with this condition may remain asymptomatic until adulthood. However, overload of right chambers may trigger symptoms, especially reduced functional capacity and progressive dyspnea, in addition to the development of pulmonary hypertension (PH) and ventricular dysfunction, which is the most severe disease scenario. Recognizing these symptoms and establishing early diagnosis and treatment is crucial to avoid disease progression and its prognostic implications.
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