Letrozole in Patients With Hepatopulmonary Syndrome

Open to people ages 18-99

• Diagnosis of moderate to very severe hepatopulmonary syndrome which consists of having all 3 of the following conditions:
• Presence of liver disease or portal hypertension
• Intrapulmonary shunting on contrast-enhanced echocardiogram
• Hypoxemia [A-a gradient ≥15mmHg (or ≥20mmHg if age >64) and PaO2<80mmHg on arterial blood gas testing]
• Child-Pugh class A or B liver disease
• MELD score < 20
• ≥ 18 years old
• Female subjects must be post-menopausal (defined as 12 months of spontaneous amenorrhea or 6 weeks postsurgical bilateral oophorectomy without or without hysterectomy)
• Ability to provide informed consent

• Enrollment in a clinical trial or concurrent use of another investigational drug or device therapy (i.e., outside of study treatment) during, or within 28 days of baseline
• Current hepatic encephalopathy
• Expectation of liver transplant within six months of randomization
• MELD exception score > 28
• Concomitant lung disease defined as restriction (TLC < 70%) or obstruction (FEV1 < 80% & FEV1/FVC < 70%)
• Inability to comply with the study protocol
• Osteoporosis
• Premenopausal women (those who have not reached 1 year absence of menarche)
• Vulnerable study population, including imprisoned individuals, non-English speaking patients