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Pulmonology / Respiratory

Portopulmonary Hypertension (PoPH)

ICD-10 Code
I27.21_6

Clinical Criteria for Portopulmonary Hypertension (PoPH).

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents for evaluation of Portopulmonary Hypertension (PoPH) in the setting of known portal hypertension/cirrhosis. Symptoms include progressive exertional dyspnea, fatigue, and occasional syncope. No history of left-sided heart failure or significant parenchymal lung disease. Current MELD score is [Score]. NYHA functional class is [Class].

Clinical Examination Findings

General: Patient is alert and oriented, appearing chronically ill. Cardiovascular: Loud P2, right ventricular heave, and a holosystolic murmur consistent with tricuspid regurgitation. Jugular venous distention (JVD) present. Abdomen: Evidence of ascites and hepatosplenomegaly. Extremities: Bilateral 2+ pitting edema. Lungs: Clear to auscultation bilaterally.

Treatment Protocol

Management plan includes optimization of portal hypertension, initiation of pulmonary vasodilator therapy (e.g., PDE5 inhibitors, endothelin receptor antagonists, or prostacyclin analogs) as indicated by RHC hemodynamics. Close monitoring of liver function tests and fluid status. Referral for liver transplant evaluation if hemodynamics stabilize.

Detailed clinical guide coming soon.