CONGRESS ABSTRACT

A 38-year-old woman comes to our attention for regular follow-up of a known tricuspid valve dysplasia, diagnosed since she was 19 years old. The patient has been persistently asymptomatic and hemodynamically compensated. In previous cardiac assessments, there was moderate and stable tricuspid regurgitation (TR) that never reached the diagnostic criteria for Ebstein ‘s anomaly (7 mm). The right atrium was dilated, and the right ventricle was at the upper limits of normal dimensions. However, our evaluation revealed a worsening of TR to a severe degree, associated with valvular annulus dilation and severe dilation of the right ventricle, which remained normokinetic, and evident diastolic overload of the interventricular septum. Therefore, a cardiac surgery intervention was indicated to correct the valvulopathy. During hospitalization, a transthoracic echocardiogram was repeated to investigate a possible cause of right ventricular overload, not fully explained by the relatively short duration of severe TR. During the exam, suspecting Partial Anomalous Pulmonary Venous Return (PAPVR) as one of the possible determinants, a vessel compatible with left superior pulmonary venous return was visualized, which converged into the anonymous vein and then into the right superior vena cava. Further diagnostic investigation with magnetic resonance angiography and computed tomography angiography confirmed the severe dilation of the right cardiac chambers and the presence of left upper pulmonary venous return and part of the left lower lobe into the left anonymous vein, with a Qp/Qs of 1.7 in relation to the pre-tricuspidal shunt. Therefore, a cardiac surgery intervention was indicated for PAPVR correction. However, previously unnoticed thrombocytosis was revealed in the hematological exams, leading to the initiation of a pertinent hematological workup and the cardiac surgery intervention was postponed to reduce the risk of perioperative complications. In conclusion, PAPVR is a rare congenital condition that can remain asymptomatic until adulthood due to a chronic adaptation of the right heart to volume overload and should, therefore, be considered in the differential diagnosis of right cardiac chambers dilation, especially when other causes do not justify its pathophysiology.