Diagnostic evaluation and management of pulmonary hypertension with concomitant incidental partial anomalous pulmonary venous return

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital heart condition which is often asymptomatic and hence remains undiagnosed, but could occasionally be detected on thoracic imaging as an incidental finding. For rare cases of newly diagnosed pulmonary hypertension with concurrent PAPVRs, the diagnostic workup and subsequent management are more complicated, requiring a thorough evaluation of secondary causes of pulmonary hypertension, and assessing relative PAPVR shunt contribution to the pulmonary hypertension. We herein report a case of a 74-year-old Chinese male patient, a chronic smoker of 50 pack-years, and past medical history of hypertension and diabetes mellitus, who was admitted to our intensive care unit with acute decompensated type 2 respiratory failure, and subsequently found to have newly diagnosed pulmonary hypertension with right heart failure and an incidental PAPVR identified on inadvertent central venous cannulation (CVC) of the anomalous pulmonary vein draining into the left internal jugular vein. There are a few key learning points from this case study: firstly, we profile the characteristics and clinical outcomes of cases of incidental CVC cannulation of undiagnosed PAPVR from a literature review; and secondly, we discuss the diagnostic and management approach to newly diagnosed pulmonary hypertension with concomitant, incidental PAPVR that may be useful for internists and critical care physicians.