Improvement in pulmonary hemodynamics during intravenous epoprostenol (prostacyclin): A study of 15 patients with moderate to severe portopulmonary hypertension

Krowka, Michael J.; Frantz, Robert P.; McGoon, Michael D.; Severson, Cathy J.; Plevak, David J.; Wiesner, Russell H.

doi:10.1002/hep.510300307

Cited by 264 publications

(178 citation statements)

References 23 publications

(38 reference statements)

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“…In keeping with previously established criteria, the diagnosis of portopulmonary hypertension was based on the association of a MPAP above 25 mm Hg, a PCWP below 15 mm Hg, and PVR over 120 dynes.s.cm Ϫ5 . 4,7,8 Splanchnic Hemodynamics at Time of Pretransplantation Evaluation. Splanchnic hemodynamic measurements were performed immediately after right heart catheterization using the same vascular access.…”

Section: Methodsmentioning

confidence: 99%

Diagnosis of portopulmonary hypertension in candidates for liver transplantation: A prospective study

et al. 2003

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Portopulmonary hypertension represents a major risk factor for transplantation; therefore, preoperative detection is crucial. The aims of this study were to determine (1) whether Doppler echocardiography performed at evaluation is a reliable tool for detecting portopulmonary hypertension and (2) the incidence of acquired portopulmonary hypertension profile after evaluation. One hundred sixty-five patients had Doppler echocardiography and right heart catheterization at evaluation over a 9-year period. All patients had a prospective follow-up, and the results of catheterization at evaluation were compared with those obtained at the time of transplantation. Seventeen of 165 patients met the criteria for portopulmonary hypertension on Doppler echocardiography. Portopulmonary hypertension was confirmed by catheterization in 10 patients and ruled out in 7. There were no false negatives for echocardiography. Mean pulmonary artery pressure was significantly higher during the initial phase of transplantation than at evaluation (17.8 ؎ 4.3 vs. 20.3 ؎ 5.5 mm Hg, respectively, P < .0001), and there was no significant correlation between values obtained at these 2 time points. Three patients showed to have acquired portopulmonary hypertension profile while waiting for a graft within time intervals ranging from 2.5 to 5 months. In conclusion, Doppler echocardiography is a highly sensitive tool for detecting portopulmonary hypertension. However, because this technique has a poor positive predictive value, right heart catheterization is recommended for confirming portopulmonary hypertension. In addition, the absence of portopulmonary hypertension at evaluation does not exclude the occasional occurrence of acquired portopulmonary hypertension profile after listing. P ulmonary hypertension associated with portal hypertension, the so-called portopulmonary hypertension, is a rare complication of cirrhosis. When severe, this condition is a major risk factor for transplantation because, in most cases, patients are at best partially responsive to medical therapies. 1 If it is impossible to lower mean pulmonary artery pressure below 40 to 50 mm Hg during transplantation procedure, any significant hemodynamic changes, such as those observed at the time of caval clamping and reperfusion of the graft, may result in irreversible cardiac arrest, especially if right ventricular function is impaired. 2 As a consequence, many authors consider that severe portopulmonary hypertension (i.e., mean pulmonary artery pressure [MPAP] above 40 mm Hg) represents a contraindication for liver transplantation because it would carry an unacceptable mortality rate. 3 Because portopulmonary hypertension is frequently asymptomatic until mean pulmonary pressure exceeds 40 mm Hg, most authors recommend systematic screening at evaluation. Several studies have suggested that Doppler echocardiography, when performed during pretransplantation evaluation, is a useful noninvasive tool to document or exclude portopulmonary hypertension, 4-6 even though this technique ...

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Section: Methodsmentioning

confidence: 99%

Diagnosis of portopulmonary hypertension in candidates for liver transplantation: A prospective study

et al. 2003

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“…8 They showed that an acute challenge with intravenous epoprostenol significantly improved pulmonary hemodynamics (reductions in PVR and MPAP and an increase in CO), indicating some acute vasoreactivity was present. Acutely, 79% of patients had greater than a 20% decrease in PVR, and 43% of patients had a 20% decrease in MPAP.…”

Section: Discussionmentioning

confidence: 99%

“…8,11,14 It is unknown how long epoprostenol infusion should be continued after OLT. PPHTN does not reverse immediately.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with an MPAP of 50 mm Hg or greater have a cardiopulmonary mortality rate that approaches 100%. 2,8 It therefore has been recommended that moderate to severe PPHTN and significant RV dysfunction should be considered contraindications to OLT. 2 However, preoperative therapy to reduce pulmonary hypertension and RV dysfunction may improve clinical status and make OLT feasible.…”

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confidence: 99%

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Liver transplantation in patients with severe portopulmonary hypertension treated with preoperative chronic intravenous epoprostenol

Tan

Markowitz

Montgomery

et al. 2001

Liver Transplantation

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Portopulmonary hypertension (PPHTN) is no longer an absolute contraindication to orthotopic liver transplantation (OLT).P ortopulmonary hypertension (PPHTN) is pathophysiologically distinct from hepatopulmonary syndrome. Whereas PPHTN is characterized by pulmonary vasoconstriction, hepatopulmonary syndrome is characterized by pulmonary vasodilation, shunting, and hypoxemia. 1 The early clinical recognition of PPHTN may be frustrated by nonspecific cardiopulmonary symptoms. Late PPHTN can be complicated by hemodynamic compromise and right heart failure.PPHTN is defined by a mean pulmonary arterial pressure (MPAP) greater than 25 mm Hg in a patient with a pulmonary capillary wedge pressure (PCWP) less than 15 mm Hg, portal hypertension, and no intrinsic or underlying lung disease. 2 PPHTN may be categorized further into mild (MPAP, 25 to 35 mm Hg), moderate (MPAP, 36 to 45 mm Hg), and severe (MPAP Ͼ 45 mm Hg). 3 Up to 12.5% of patients presenting for orthotopic liver transplantation (OLT) evaluation have PPHTN, 3-5 but 60% of these patients are asymptomatic at the time of diagnosis. 6 The prognosis for patients with PPHTN is dismal, with a mean and median survival after diagnosis of 15 months and 6 months, respectively. 6 The 6-month mortality rate is estimated to be approximately 50%. 6 In patients with severe PPHTN who proceed to OLT, perioperative mortality is very high. In 5 patients with severe PPHTN who underwent OLT at the University of Pittsburgh, PA, the perioperative mortality rate was reported at 80% and resulted primarily from right ventricular (RV) dysfunction. 7 It has been estimated that in patients who undergo OLT with an MPAP between 35 and 49 mm Hg and pulmonary vascular resistance (PVR) of 250 dynes ⅐ s ⅐ cm -5 or greater, the mortality rate is 50%. Patients with an MPAP of 50 mm Hg or greater have a cardiopulmonary mortality rate that approaches 100%. 2,8 It therefore has been recommended that moderate to severe PPHTN and significant RV dysfunction should be considered contraindications to OLT. 2 However, preoperative therapy to reduce pulmonary hypertension and RV dysfunction may improve clinical status and make OLT feasible. Continuous intravenous epoprostenol (prostacyclin, or prostaglandin I 2 ) is a potent vasodilator shown to improve exercise tolerance, reduce PVR, and improve mortality in patients with primary pulmonary hypertension. 9 Similar hemodynamic improvements have been achieved before OLT in patients with PPHTN. 10,11 Kuo et al 10 treated 4 patients with severe PPHTN (MPAP, 53 to 63 mm Hg) with 10 to 28 ng/kg/min of epoprostenol over 6 to 14 months and reported a 29% to 46% decrease in MPAP, a 22% to 71% decrease in PVR, and a 25% to 75% increase in cardiac output (CO).

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“…Long-term treatment with epoprostenol, both preoperatively and postoperatively, seems to be efficacious in some patients. 17 The more extensive data being collected in the multicenter database may shed more light on the proper management of this small patient population. 18 …”

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confidence: 99%

Perioperative Mortality in Patients With Portopulmonary Hypertension Undergoing Liver Transplantation

Ramsay¹

2000

Liver Transplantation

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Improvement in pulmonary hemodynamics during intravenous epoprostenol (prostacyclin): A study of 15 patients with moderate to severe portopulmonary hypertension

Cited by 264 publications

References 23 publications

Diagnosis of portopulmonary hypertension in candidates for liver transplantation: A prospective study

Diagnosis of portopulmonary hypertension in candidates for liver transplantation: A prospective study

Liver transplantation in patients with severe portopulmonary hypertension treated with preoperative chronic intravenous epoprostenol

Perioperative Mortality in Patients With Portopulmonary Hypertension Undergoing Liver Transplantation

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