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Successful Orthotopic Liver Transplantation in a Patient with Portopulmonary Hypertension Treated with Tadalafil and Perioperative Nitric Oxide

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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.21.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Vikas Pathak I

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Portopulmonary hypertension (POPH) is a subtype of WHO Group 1 pulmonary arterial hypertension (PAH). However, PAH therapies have not been extensively studied for this condition. Furthermore, there is a relative paucity of data on the use of PAH therapies in the context of liver transplantation (OLT). We report the use of tadalafil, with adjunct perioperative inhaled NO in a patient with POPH that underwent OLT.

Introduction: Portopulmonary hypertension (POPH) is a subtype of WHO Group 1 pulmonary arterial hypertension (PAH).  However, PAH therapies have not been extensively studied for this condition.  Furthermore, there is a relative paucity of data on the use of PAH therapies in the context of liver transplantation (OLT).  We report the use of tadalafil, with adjunct perioperative inhaled NO in a patient with POPH that underwent OLT.

Case description: The patient is a 30 year-old white male with primary sclerosing cholangitis and cirrhosis referred for pulmonary hypertension evaluation. The patient had no symptoms of dyspnea or right heart failure. Echocardiogram revealed a mildly dilated right heart and tricuspid regurgitant jet of 3.0 m/s. Right heart catheterization (RHC) revealed a mean pulmonary artery (mPAP) pressure of 40 mmHg and pulmonary capillary wedge pressure (PCWP) of 13 mmHg. Fick cardiac output was 5.55 L/min and pulmonary vascular resistance (PVR) was 4.8 woods units. The patient was started on sildenafil but did not tolerate it due to headache.  He was offered prostacyclin therapy and endothelin receptor antagonist therapy but declined both.  Repeat RHC two years later revealed mPAP 42 mmHg with PCWP of 15 mmHg and PVR of 3.7 woods units. He was started on subcutaneous treprostinil which he discontinued due to side effects at a dose of 8 ng/kg/min. Tadalafil was started at 40 mg daily. After 3 months RHC showed mPAP of 26 mmHg, PCWP of 4 mmHg, and PVR was 2.4 Woods units. He ultimately underwent OLT and required inhaled NO via ventilator circuit due to acute pulmonary hypertension intraoperatively.  The NO was easily weaned off postoperatively and he remains on tadalafil.

Discussion: For patients with moderate to severe POPH undergoing OLT (mPAP >35 mmHg and PVR >5.0 Wood units), the risk of operative mortality is high. Sildenafil has been used successfully in this cohort of patients, but there is scant data on the use of other PDE-5 inhibitors. Our patient was unable to tolerate sildenafil and parenteral prostacyclin therapy due to side effects, but tadalafil was tolerated and efficacious.

Conclusion: Tadalafil monotherapy was feasible and safe in this patient with portopulmonary hypertension.  OLT was successfully performed, though adjunct NO was needed that was easily weaned off post-transplant.  Further study of tadalafil in this population is needed to make broader conclusions, but it may play a role in the treatment of POPH, specifically in select patients undergoing OLT.

Type: Case study