Pulmonary Hemodynamics and Perioperative Cardiopulmonary-Related Mortality in Patients With Portopulmonary Hypertension Undergoing Liver Transplantation

Abstract

In the setting of moderate to severe pulmonary artery hypertension, orthotopic liver transplantation (OLT) may be complicated by pulmonary hemodynamic instability and cardiopulmonary mortality. We retrospectively studied the relationship between cardiopulmonary-related mortality and initial (untreated) pre-OLT pulmonary hemodynamics in 43 patients with portopulmonary hypertension who underwent attempted OLT. Thirty-six patients were reported in 18 peer-reviewed studies, and 7 patients underwent OLT at our institution since 1996. Transplantation procedure outcome, mean pulmonary artery pressure (MPAP), pulmonary vascular resistance (PVR), cardiac output, pulmonary capillary wedge pressure, and transpulmonary gradient (TPG) are summarized. Overall mortality was reported in 15 of 43 patients (35%). Fourteen of the 15 deaths (93%) were primarily related to cardiopulmonary dysfunction. Two deaths were intraoperative, 8 deaths occurred during the transplantation hospitalization, and 4 patients died of cardiopulmonary deterioration posthospitalization. In 4 patients, the transplantation procedure could not be successfully completed. Cardiopulmonary mortality was associated with greater pre-OLT MPAP (49 ؎ 14 v 36 ؎ 7 mm Hg; P F .005), PVR (441 ؎ 173 v 261 ؎ 156 dynes·s·cm Ϫ5 ; P F .005), and TPG (37 ؎ 13 v 22 ؎ 10 mm Hg; P F .005). MPAP of 50 mm Hg or greater was associated with 100% cardiopulmonary mortality. In patients with an MPAP of 35 to less than 50 mm Hg and PVR of 250 dynes·s·cm Ϫ5 or greater, the mortality rate was 50%. No mortality was reported in patients with a pre-OLT MPAP less than 35 mm Hg or TPG less than 15 mm Hg. Cardiopulmonaryrelated mortality in OLT patients with portopulmonary hypertension was frequent and associated with significantly increased pre-OLT MPAP, PVR, and TPG compared with survivors. Treated or untreated, we recommend intraoperative cancellation or advise against proceeding to OLT for an MPAP of 50 mm Hg or greater. Patients with an MPAP of 35 to less than 50 mm Hg and PVR of 250 dynes·s·cm Ϫ5 or greater appear to be at high risk for cardiopulmonary-related mortality after OLT. A prospective study is needed to define optimal pretransplantation treatments and pulmonary hemodynamic criteria that minimize OLT mortality associated with portopulmonary hypertension. (Liver Transpl 2000;6:443-450.) P ortopulmonary hypertension is defined as pulmonary artery hypertension (mean pulmonary artery pressure [MPAP], Ͼ25 mm Hg and pulmonary capillary wedge pressure [PCWP] less than 15 mm Hg) in association with portal hypertension. 1-3 Several investigators adhere to the additional criteria of increased pulmonary vascular resistance ([PVR] Ͼ120 dynes·s·cm Ϫ5 ). [4][5][6] A unifying hypothesis explaining the cause of this liver-lung relationship has yet to be proven. 2 Patients with portopulmonary hypertension have a high mortality rate. Within 15 months from the time pulmonary hypertension was diagnosed, death was reported in 38% (10 of 26 patients) to 41% (32 of 78 patients) of the patients describ...

Portopulmonary hypertension: Results from a 10-year screening algorithm

“…Elevated MPAP has been the most important parameter in discussing the multicenter diagnostic implications of POPH. 5,8 However, various reasons for elevated MPAP exist in the setting of liver disease (hyperdynamic circulatory state, increased central volume). Our data clearly demonstrate that elevated MPAP alone is not specific for the diagnosis of POPH, supporting the current diagnostic criteria that must include PVR.…”

“…CTP class A (score 5-6) is less severe than CTP class C (score 10-15). 5 The MELD score is quantitative, being derived from a formula with the variables serum total bilirubin, international normalized ratio, and serum creatinine. 17 The CTP classification and MELD score determinations were obtained based on data available at the time of the right heart catheterization in a retrospective manner.…”

“…1,2,[5][6][7] This pathophysiology characterizes the concept of portopulmonary hypertension (POPH), an uncommon hemodynamic problem associated with intraoperative liver transplantation death and posttransplant hospitalization mortality. 2,8 POPH is now recognized as a unique entity within the modified World Health Organization Group I category of pulmonary arterial hypertension.…”

Guidelines for the diagnosis and treatment of pulmonary hypertension

“…PAH can substantially increase the risk associated with liver transplantation and usually PAH is a contraindication if P pa is .35 mmHg and/or PVR is o250 dynes.s.cm -5 [206,212]. It has been suggested that PAH-specific drug therapy should be used to improve haemodynamics prior to liver transplantation, but the effects on the outcome of liver transplantation have not been evaluated sufficiently.…”

Intrapulmonary vascular dilatations are common in portopulmonary hypertension and may be associated with decreased survival

“…The primary focus of therapy is on control of pulmonary pressures with vasodilator therapy and improved right ventricle (RV) function, with consideration of LT if MPAP of 35 mm Hg or lower can be maintained. 5,[11][12][13][14][15] Thus, HPS and POPH have divergent clinicopathologic features and management.…”