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Pulmonology / Respiratory

Idiopathic Pulmonary Arterial Hypertension (IPAH)

ICD-10 Code
I27.0_2

Clinical Criteria for Idiopathic Pulmonary Arterial Hypertension (IPAH).

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents with progressive exertional dyspnea (NYHA Functional Class [I-IV]), fatigue, and reduced exercise tolerance. Reports episodes of presyncope/syncope, exertional chest pain, and peripheral edema. No history of connective tissue disease, congenital heart disease, portal hypertension, or illicit drug use. Symptoms are chronic and insidious in onset.

Clinical Examination Findings

Vitals: Tachycardia, tachypnea, potential hypoxemia. Cardiovascular: Prominent P2 (pulmonology component of S2), right ventricular heave, holosystolic murmur of tricuspid regurgitation at the left sternal border. Jugular venous distension (JVD) present. Abdominal: Hepatomegaly, pulsatile liver. Extremities: Bilateral pitting edema. Lungs: Generally clear to auscultation, no wheezing or crackles.

Treatment Protocol

Initiate PAH-specific therapy: [PDE5 inhibitor / Endothelin receptor antagonist / Prostacyclin analogue / sGC stimulator]. Consider combination therapy for high-risk patients. Adjunctive therapy: Diuretics for volume overload, oxygen therapy for hypoxemia, anticoagulation if indicated. Scheduled follow-up for 6-minute walk test (6MWT) and echocardiographic monitoring of RV function.

Detailed clinical guide coming soon.