Clinical Presentation & Protocol
Patient Usually Complains Of
Patient presents with progressive exertional dyspnea, non-productive cough, and fatigue. History notable for worsening functional capacity (WHO FC [I/II/III/IV]). Denies orthopnea or paroxysmal nocturnal dyspnea. No history of connective tissue disease or toxin exposure. Symptoms refractory to standard heart failure management.
Clinical Examination Findings
General: Patient appears tachypneic at rest. HEENT: No jugular venous distension. CV: Loud P2, holosystolic murmur at left sternal border (tricuspid regurgitation). Lungs: Fine bibasilar inspiratory crackles. Extremities: Trace peripheral edema, no clubbing. O2 saturation [X]% on room air.
Treatment Protocol
Caution: Pulmonary vasodilators (e.g., epoprostenol) may precipitate pulmonary edema in PVOD; initiate with extreme caution under specialist supervision. Oxygen therapy for hypoxemia. Diuretics for volume management. Referral for lung transplantation evaluation is mandatory. Avoid systemic anticoagulation unless indicated for other comorbidities.