A63-28 Uncovering the Hidden Connection: Severe Pre-capillary Pulmonary Hypertension in an Adult With ASD, VSD, and PAPVR

Abstract Introduction WHO Group I pulmonary arterial hypertension (PAH) has numerous etiologies including congenital heart disease (CHD), and must be distinguished from Groups 3 and 4 pulmonary hypertension (PH). We present the case of a patient with severe pre-capillary PH who through thorough diagnostic work-up was found to have an atrial septal defect (ASD), a ventricular septal defect (VSD), and partial anomalous pulmonary venous return (PAPVR). Case Presentation The patient is a 72-year-old female with history of chronic obstructive pulmonary disease (COPD) and prior pulmonary embolism who presented with two months of worsening exertional dyspnea from right ventricular failure. Diagnostic work-up included contrast angiography negative for acute or chronic embolism as well as negative autoimmune serologies and no metabolic dysfunction. Echocardiography revealed a mid-septal defect that was equivocal for VSD. Right heart catheterization demonstrated severe pre-capillary PH with right atrial pressure of 5 mmHg, pulmonary artery systolic pressure of 74 mmHg, pulmonary artery diastolic pressure of 30 mmHg, mean pulmonary artery pressure of 50 mmHg, cardiac output of 4.2 L/min, cardiac index of 2.4 L/min/m2, and pulmonary vascular resistance of 9.5 Wood Units. The Qp:Qs was calculated to be 1.51, consistent with net left-to-right shunting. Cardiac magnetic resonance imaging (MRI) was performed which showed abnormal contrast transit from the left to right ventricles, indicating a VSD. Subsequent review of cardiac MRI and CT demonstrated both ASD and PAPVR. For her PH, she was treated with riociguat and ambrisentan and later reported improvement of her symptoms and functional status. Discussion The identification of congenital cardiac lesions during the workup of PH can complicate the diagnosis and it can be difficult to determine if the lesion is related to the development of PH. In our case, severe pre-capillary PH was identified in a patient with poor functional status along with a previously undiagnosed VSD, ASD, and PAPVR. The co-occurrence of these three incidental findings in an older adult is infrequent and has not been well-reported. At the time of diagnosis, the VSD was moderate in size and with evidence of left-to-right shunting, while the severity of the other two pre-tricuspid lesions could not be characterized. CHD is a strong contributor to the development of PAH due to pulmonary arteriolar remodeling. Early identification of pre and post-tricuspid lesions can prevent progression to Eisenmenger syndrome. Routine workup including echocardiography can identify these lesions while cardiac MRI has greater sensitivity and allows for quantitation of shunting. This abstract is funded by: None