The Impact of Treatment of Portopulmonary Hypertension on Survival Following Liver Transplantation

Abstract

Pulmonary hypertension in the setting of cirrhosis and portal hypertension is known as portopulmonary hypertension (PPHTN). Moderate or severe PPHTN is uncommon, but has a poor prognosis and is considered to be a contraindication to liver transplantation. We assessed the impact of vasodilation therapy on pulmonary hemodynamics and outcome after liver transplant in these patients. Eighty-six patients evaluated for liver transplant between 1997 and 2005 had an estimated right ventricular systolic pressure >40 mm Hg or a clinical suspicion of PPHTN. Right heart catheterization confirmed PPHTN in 30 patients (ten mild, eight moderate, and 12 severe). Sixteen of the 20 with moderate-to-severe pulmonary hypertension (mPAP ≥ 35) were otherwise considered suitable liver transplant candidates and were treated with vasodilation therapy. mPAP fell to less than 35 mm Hg in 12 patients (75%) and 11 of them then underwent orthotopic liver transplantation. One-and five-year survivals in the transplanted patients were 91% and 67%, respectively. Nine of 11 were off vasodilator therapy after a median of 9.2 months following transplantation. None of the patients who failed vasodilator therapy survived (median survival, 8 months). Effective pharmacologic control of PPHTN before liver transplant is associated with excellent posttransplant survival that is similar to patients transplanted for other indications.

Clinical implications of the hyperdynamic syndrome in cirrhosis

“…The mortality rate after OLT is 50% if the MPAP is N35 mm Hg, and 100% if the MPAP is N 50 mm Hg [70]. In fact, efficient pharmacologic control of POPH before transplantation is associated with post-transplant survival that is similar to that of patients transplanted for other indications [70,71].…”

Systemic hemodynamics in advanced cirrhosis: Concerns during perioperative period of liver transplantation

“…However, the diagnosis and treatment of PPHTN have advanced during the past two decades [54] . Assessment of patients' preoperative reactivity and response to pharmacological therapies for moderate-to-severe PPHTN is important to ensure excellent survival rates after LT [102] . Prostaglandin I2 has drastically improved outcomes [103] and is currently considered a key drug in the control of PPHTN [103] .…”

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“…Typical criteria include (1) good RVF, considered to be the most important requirement for successful transplant, 23 and (2) responsiveness to pulmonary vasodilators. 24 Our patient had excellent RVF before the first transplant attempt and good response to epoprostenol and sildenafil.…”

“…6 The pharmacologic therapy of PH usually should be continued for several months after OLT. 6,24 This likely is related to the time required to reverse the pulmonary vascular remodeling that occurred. Ogawa and coworkers showed the viability of living-donor liver transplant in patients with POPH.…”

“…8,18,[24][25][26][27] Epoprostenol binds to endothelial receptors, resulting in increased cyclic adenosine monophosphate levels in the cytosol. This leads to smooth muscle relaxation with subsequent vasodilation.…”