Clinical Presentation & Protocol
Patient Usually Complains Of
Patient presents for follow-up of Heritable PAH (BMPR2 mutation). Reports progressive exertional dyspnea (NYHA Class [I-IV]), fatigue, and occasional pre-syncope. Denies chest pain or orthopnea. Current medication adherence is [good/poor]. Family history is significant for [specify relatives] with confirmed PAH.
Clinical Examination Findings
Vitals: Stable, SpO2 [X]% on room air. Cardiovascular: Regular rate and rhythm, loud P2, holosystolic murmur at the left sternal border (consistent with tricuspid regurgitation), JVD present. Respiratory: Clear to auscultation bilaterally. Extremities: Trace bilateral lower extremity edema, no cyanosis or clubbing.
Treatment Protocol
Continue current PAH-targeted therapy: [List medications, e.g., PDE5i, ERA, Prostacyclin analogs]. Monitor for side effects. Advise strict adherence to medication schedule. Schedule repeat 6-minute walk test (6MWT) and NT-proBNP levels. Maintain fluid restriction and low-sodium diet.