Novel use of sildenafil in the treatment of portopulmonary hypertension

Chua, R.; Keogh, Anne; Miyashita, Masami

doi:10.1016/j.healun.2004.01.017

Cited by 59 publications

(34 citation statements)

References 9 publications

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“…As with other forms of secondary pulmonary hypertension, POPH is potentially reversible if the primary disease is cured, in this case by liver transplantation. While this approach has been discussed in the past, only individual cases have been reported to date (17)(18)(19)(20)(21)(22). Here we report on eight consecutive patients treated with sequential vasodilator therapy followed by OLT.…”

Section: Discussionmentioning

confidence: 99%

“…These facts present a potential therapeutic opportunity; POPH might initially be controlled by vasodilator therapy and subsequently cured by liver transplantation. This approach is still controversial, as pointed out in recent reviews (4,16) although several case reports have been published (17)(18)(19)(20)(21)(22). We have undertaken this study to examine the feasibility of sequential vasodilator therapy and orthotopic liver transplantation as a therapeutic option in a group of patients with POPH.…”

Section: Introductionmentioning

confidence: 99%

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Successful Liver Transplantation Following Medical Management of Portopulmonary Hypertension: A Single-Center Series

Sussman

Kaza²,

Barshes

et al. 2006

American Journal of Transplantation

127

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Severe portopulmonary hypertension (POPH) is an absolute contraindication to orthotopic liver transplantation (OLT). Vasodilators have been used, but the safety of subsequent transplantation and the reversibility of pulmonary hypertension after transplantation are uncertain. This study examined the feasibility and post-transplant effects of liver transplantation following medical control of POPH. Eight consecutive patients (three females and five males, ages 39-51) with POPH as their only contraindication to transplantation were treated with continuous intravenous epoprostenol. Liver transplantation was considered if the mean pulmonary artery pressure (PAM) was lowered to <35 mmHg. Epoprostenol 2-8 ng/kg/min successfully improved hemodynamics in seven of eight patients, usually within 6.5 months of initiating therapy. PAM declined from an average of 43-33 mmHg (p = 0.03); mean pulmonary vascular resistance declined from 410 to 192 dyn s cm −5 (p = 0.01) and cardiac output increased from 6.6 to 10 L/min (p = 0.02). Six of the seven responders were actively listed for liver transplantation. Two died on the waiting list; the remaining four were transplanted and remain alive and well 9-18 months post-OLTtwo without vasodilators, and two on oral medication. We conclude that pulmonary vasodilators permit safe liver transplantation in some cases, and that POPH may be reversible after transplantation.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Successful Liver Transplantation Following Medical Management of Portopulmonary Hypertension: A Single-Center Series

Sussman

Kaza²,

Barshes

et al. 2006

American Journal of Transplantation

127

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“…It has the advantage of being an oral compound with pulmonary vasoselective action but no hepatotoxicity [14]. The successful use of sildenafil in POPH has recently been reported in two case studies [15,16].…”

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confidence: 99%

Sildenafil treatment for portopulmonary hypertension

Reichenberger

Voswinckel²,

Steveling³

et al. 2006

European Respiratory Journal

194

121

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Portopulmonary hypertension (POPH) is regarded as a subtype of pulmonary arterial hypertension (PAH); however, established PAH therapies have not been evaluated for this condition.The current authors treated 14 patients (four male, 10 female; mean (range) age 55 (39-75) yrs) with moderate (n51) or severe (n513) POPH caused by alcoholic liver disease (n57), chronic viral hepatitis (n53), autoimmune hepatitis (n53), and hepatic manifestation of hereditary haemorrhagic teleangiectasia (n51) with oral sildenafil. Eight patients were newly started on pulmonary vasoactive treatment, while six patients were already on treatment with inhaled prostanoids (iloprost, n55; treprostinil, n51). During treatment with sildenafil, mean¡SD 6-min walk distance increased from 312¡111 m to 397¡99 m after 3 months, and 407¡97 m after 12 months. Mean¡SD pro-brain natriuretic peptide levels decreased from 582¡315 ng?mL -1 to 230¡278 ng?mL, and to 189¡274ng?mL -1 after 3 and 12 months, respectively. Two patients died after 1 and 2 months from liver failure and cardiac failure, respectively. There was a similar response to sildenafil treatment after 3 and 12 months in patients on monotherapy and those on combination therapy.In conclusion, sildenafil might be effective in monotherapy and in combination therapy with inhaled prostanoids in portopulmonary hypertension, leading to significant improvement by 3 months and sustained response over 12 months.

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“…Effective medical therapy is of great importance, as a markedly increased pulmonary pressure has a very poor prognosis in cirrhotic patients and is a contraindication for liver transplantation. The results of the studies by REICHENBERGER et al [1] and others [2,3] show that in these patients, inhibitors of PDE5 also lower PAP. However, REICHENBERGER et al [1] focused on the effect of sildenafil on PAP; the potential effect of this drug on portal pressure was not investigated.…”

Section: To the Editorsmentioning

confidence: 84%