Blog
Hepatopulmonary Syndrome
Covenant Metabolic Specialists Health Library
Covenant Metabolic Specialists
Physician Reviewed
Dec 3, 2025
Hepatopulmonary syndrome (HPS) is a complication of chronic liver disease marked by intrapulmonary vascular dilatations that impair oxygen exchange, leading to hypoxemia. It is characterized by the triad of liver disease, arterial oxygenation defect, and evidence of intrapulmonary shunting.
Symptoms
Patients present with progressive shortness of breath, platypnea (worse when upright), orthodeoxia (O2 saturation drop on standing), digital clubbing, cyanosis, and spider nevi. Fatigue, syncope, and reduced exercise capacity limit quality of life.
Causes
The exact mechanism is unclear but involves liver failure–induced increased nitric oxide production causing pulmonary vasodilation and arteriovenous shunts. Cirrhosis from viral hepatitis, alcohol, or NASH is common, but HPS can accompany portal hypertension without cirrhosis.
Risk Factors
Any etiology of cirrhosis or portal hypertension can lead to HPS. Longstanding liver disease, male sex, and advanced MELD scores heighten prevalence.
Diagnosis
Pulse oximetry showing saturation <96 % prompts evaluation. Arterial blood gas reveals widened A–a gradient. Contrast echocardiography with microbubbles demonstrates delayed appearance in the left heart (>3 cardiac cycles), confirming intrapulmonary shunt. Lung perfusion scan with technetium albumin supports diagnosis.
Treatments
No medical therapy reliably reverses HPS. Supplemental oxygen relieves symptoms; liver transplantation is the only definitive cure, with most patients improving oxygenation within months post‑transplant. Pre‑transplant management focuses on optimizing lung function, vaccinating against respiratory pathogens, and avoiding hypoxia aggravators.
Prevention
Preventing cirrhosis through viral hepatitis treatment, alcohol moderation, and metabolic disease control indirectly reduces HPS incidence. Early cirrhosis referral for transplant evaluation catches HPS before severe hypoxemia develops.
Our Take
Shortness of breath in cirrhotics is often dismissed as ascites or deconditioning. Covenant routinely checks standing O2 sat—one cheap test that can unveil HPS and accelerate transplant listing.
Hepatopulmonary syndrome transforms liver disease into a pulmonary threat. Through vigilant screening and timely transplant referral, Covenant helps patients breathe easier—literally and figuratively.
