Pulmonary arterial hypertension

Abstract

Clinical Case Download : Download full-size image A 27-year-old female presented with progressive shortness of breath over 1 year. She was initially treated with inhaled bronchodilators after she was diagnosed with asthma. However, she continued to have progressive dyspnea and eventually experienced chest pain and syncope with moderate exertion. She had no past medical or surgical history, and her family history was unremarkable. There was no history of substance abuse and she had no travels outside the United States. On examination her blood pressure was 105/65 mmHg and her body mass index (BMI) was normal. Her jugular venous pressure was elevated but she had no peripheral edema. Cardiac examination demonstrated a regular rhythm with a prominent P2 and a right ventricular heave. Her lungs were clear. Noninvasive testing included an electrocardiogram which showed sinus rhythm with a right axis deviation and ST depressions in V1–V3 suggestive of right ventricular strain. An echocardiogram revealed a small left ventricular cavity size with septal shift consistent with right ventricular pressure and volume overload. There was right atrial enlargement, severe right ventricular enlargement with moderate dysfunction, moderate tricuspid regurgitation, and an estimated pulmonary artery pressure of 90 mmHg. A 6-min walk test was performed, and it was reduced at 330 m. How would you manage her next? Abstract Pulmonary arterial hypertension (PAH), a form of pulmonary hypertension (PH), is a complex disorder with sexual dimorphism. The disease preferentially affects women, and female sex influences both the development of PAH and right ventricular remodeling. Large registries have demonstrated that while women are more likely to develop PAH, older men with PAH have increased mortality. Sex hormones may impact right ventricular function as well as response to PAH-specific medications. Despite advancements in therapeutics for PAH, mortality remains high for both men and women. Further investigation is necessary to fully elucidate the impact of sex on PAH pathogenesis and help guide novel therapeutics.