A longstanding, large atrial septal defect in a 52-year-old male led to severe precapillary pulmonary hypertension with a mean pulmonary arterial pressure of 41 and PVR of 6.2 Wood units.
Case Report (n=1)
This case highlights the critical importance of early detection and closure of atrial septal defects to prevent the development of severe precapillary pulmonary hypertension and potentially fatal Eisenmenger syndrome.
Abstract Pulmonary hypertension (PH) is a heterogenous group of conditions characterized by elevated mean pulmonary arterial pressure (mPAP) leading to right heart dysfunction. Atrial septal defects (ASD) can lead to pulmonary arterial hypertension (PAH) through vascular remodeling over time. We present a case of severe precapillary PH in the setting of a large ostium secundum ASD diagnosed in adulthood due to barriers to healthcare access. A 52-year-old healthy male presented to the ED with four months of progressive dyspnea on exertion. Physical exam was notable for faint systolic murmur, subtle S3/S4, tachypnea and oxygen saturation of 87% on ambient air. CTA chest revealed an enlarged pulmonary arterial trunk measuring 5.3 cm. Transthoracic echocardiogram showed a large ASD, elevated right ventricular systolic pressure (RVSP) of 84, decreased tricuspid annular plane systolic excursion (TAPSE) of 1.2, and left ventricular ejection fraction (LVEF) of 40-45%. Right heart catheterization (RHC) showed a mPAP of 41, normal pulmonary capillary wedge pressure (PCWP) of 12, and elevated pulmonary vascular resistance (PVR) of 6.2 Wood units. The pulmonary to systemic blood flow (Qp/Qs) ratio was increased at 1.62 reflecting a left-to-right shunt, with higher oxygen saturation in the right ventricle (RV) at 77% than the inferior vena cava (IVC) at 66%. The patient was diagnosed with precapillary PAH due to ASD and started on combination therapy of macitentan, tadalafil and selexipag, while remaining under cardiothoracic evaluation for possible ASD closure. ASDs account for 10% of congenital heart defects, with PH seen in up to 22% of cases. Large ASDs cause increased pulmonary perfusion and vascular changes in the pulmonary arteriole bed leading to PAH. This patient was diagnosed with PAH as a consequence of a longstanding, large ASD. He had never seen a doctor until immigrating to the United States; an ASD detected earlier, in absence of PAH, carries lower risks of heart failure, stroke, and death when surgically closed. The presence of a left-to-right shunt puts him at risk for developing a right-to-left shunt (Eisenmenger syndrome). A Qp/Qs 1.5 and PA systolic pressure/systemic systolic arterial pressure 2/3, placing him at the borderline of guideline recommendations for intervention. PAH therapy aims to relieve symptoms and lower PA pressures to make ASD closure safer. Recognition of PAH risk in a longstanding ASD is critical, as progression to a cyanotic right-to-left shunt can be fatal. Early evaluation and closure of ASD are key to avoiding these complications. This abstract is funded by: None
Bolourchi et al. (Fri,) conducted a case report in Precapillary pulmonary hypertension and atrial septal defect (n=1). Macitentan, tadalafil, and selexipag was evaluated. A longstanding, large atrial septal defect in a 52-year-old male led to severe precapillary pulmonary hypertension with a mean pulmonary arterial pressure of 41 and PVR of 6.2 Wood units.