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Pulmonology / Respiratory

Pulmonary Capillary Hemangiomatosis (PCH)

ICD-10 Code
I27.89_3

Clinical Criteria for Pulmonary Capillary Hemangiomatosis (PCH).

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents with progressive exertional dyspnea, non-productive cough, and occasional hemoptysis. Symptoms are consistent with pulmonary venous hypertension and capillary proliferation. Denies orthopnea or paroxysmal nocturnal dyspnea. No history of connective tissue disease or toxin exposure.

Clinical Examination Findings

Physical exam reveals resting tachypnea and peripheral cyanosis. Cardiac auscultation demonstrates a loud P2 and a right ventricular heave, suggestive of pulmonary hypertension. Pulmonary auscultation reveals fine bibasilar inspiratory crackles. No evidence of peripheral edema or jugular venous distension.

Treatment Protocol

Management plan includes supplemental oxygen to maintain SpO2 >92%. Avoidance of pulmonary vasodilators (e.g., prostacyclin analogues) due to risk of precipitating pulmonary edema. Referral for lung transplantation evaluation is mandatory. Consider trial of immunosuppressive therapy if indicated by multidisciplinary team.

Detailed clinical guide coming soon.