Background: Pulmonary arterial hypertension (PAH) involves many different clinical conditions and has an important impact on the right ventricular (RV) function and patient’s prognosis. Some of these conditions can be potentially curable, for example, congenital heart disease that could be surgically repaired. Case Presentation: We present a case of 41-year-old woman with a progressive shortness of breath, without evident explication. First, she was suspected to have chronic thromboembolic PAH, which was not confirmed by clinical and imaging data. Even arrhythmogenic RV cardiomyopathy was suspected according to the data obtained from the first cardiac magnetic resonance (CMR) imaging. However, due to a step-up in oxygen saturation during the right heart catheterization (RHC), the presence of rare congenital heart disease, such as partial anomalous of pulmonary venous return (PAPVR), was suspected and then confirmed by the repeated CMR. Conclusions: PAPVR is a rare congenital heart disease that could be suspected during the RHC and represents a potentially curable cause of PAH.

Keywords: Partial anomalous pulmonary venous return, pulmonary arterial hypertension, right ventricle, cardiac magnetic resonance, right heart catheterization