Portopulmonary hypertension

Halank, Michael; Ewert, Ralf; Seyfarth, HJ; Hoeffken, Gert

doi:10.1007/s00535-006-1879-x

Cited by 24 publications

(26 citation statements)

References 88 publications

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“…KEYWORDS: Bosentan, cirrhosis, iloprost, portopulmonary hypertension, pulmonary hypertension T he term portopulmonary hypertension (PPHT) refers to the development of pulmonary arterial hypertension (PAH) in patients with portal hypertension, a rare but serious complication occurring in 1-2% of patients with cirrhosis [1,2]. Affected patients typically complain of progressive dyspnoea on exertion and may show signs of right heart dysfunction.…”

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confidence: 99%

Experience with inhaled iloprost and bosentan in portopulmonary hypertension

Hoeper¹,

Seyfarth²,

Hoeffken³

et al. 2007

Eur Respir J

163

114

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Novel treatments, such as prostanoids or endothelin receptor antagonists, have been introduced for various forms of pulmonary arterial hypertension, but the long-term effects of these treatments on portopulmonary hypertension (PPHT) are unknown.In a retrospective analysis, the present authors assessed the safety and efficacy of inhaled iloprost, a prostacyclin analogue, and bosentan, an endothelin receptor antagonist, in patients with PPHT. In total, 31 consecutive patients with Child class A or B cirrhosis and severe PPHT were treated for up to 3 yrs with either inhaled iloprost (n513) or bosentan (n518), and the effects on exercise capacity, haemodynamics and survival were evaluated.In the iloprost group, the survival rates at 1, 2 and 3 yrs were 77, 62 and 46%, respectively. In the bosentan group, the respective survival rates were 94, 89 and 89%. Event-free survival rates, i.e. survival without transplantation, right heart failure or clinical worsening requiring the introduction of a new treatment for pulmonary hypertension, was also significantly better in the bosentan group. Bosentan had significantly better effects than inhaled iloprost on exercise capacity, as determined by the 6-min walk test, as well as on haemodynamics. Both treatments proved to be safe, especially in regards of liver function.In the present series of patients with well-preserved liver function and severe portopulmonary hypertension, treatment with both inhaled iloprost and bosentan appeared to be safe. Patients treated with bosentan had higher survival rates, but prospective controlled studies are required to confirm these findings.

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confidence: 99%

Experience with inhaled iloprost and bosentan in portopulmonary hypertension

Hoeper¹,

Seyfarth²,

Hoeffken³

et al. 2007

Eur Respir J

163

114

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“…Definitionsgemäß besteht eine portopulmonale Hypertonie, wenn eine portale Hypertonie und eine Druckerhöhung im kleinen Kreislauf vorliegen und andere Ursachen der pulmonalen Hypertonie (hyperdyname Kreislaufsituation, Hyperhydratation, Linksherzerkrankungen, Lungenerkrankungen und/oder Hypoxämie, Lungenembolien) ausgeschlossen sind [42].…”

Section: Die Portopulmonale Hypertonie (Poph)unclassified

“…Diskutiert wird als wesentliche Ursache die häufig bei Zirrhose mitbestehende hyperdyname Kreislaufsituation [46]. Reduktion sowohl des pulmonalarteriellen als auch des portalen Drucks zu führen [42].…”

Section: Pathogenese Der Pophunclassified

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Pulmonale Affektion bei fortgeschrittener Lebererkrankung – hepatopulmonales Syndrom und portopulmonale Hypertonie

Herzer¹,

Post

Canbay³

et al. 2010

Med Klin

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Patients suffering from severe chronic liver disease, in particular cirrhosis, are at risk for pulmonary complications. The leading clinical symptom is shortness of breath, which can accompany the actual disease as indirect effect because of anemia, faint muscles or ascites. On the other hand, dyspnea can have multiple additive causes in case of accompanying cardial or pulmonary disease. The hepatopulmonary syndrome (HPS) and the portopulmonary hypertension (PoPH) belong to the most relevant pulmonary complications in liver cirrhosis. HPS appears to be more common than PoPH and the presence of either entity increases morbidity and mortality in patients with liver disease. The two diseases have to be strictly distinguished, as they have opposed histological and pathophysiological origin. While the HPS is a dilatative pulmonary- vascular disease, the PoPH is a constrictive or obliterative pulmonary-vascular disease in the context of a liver disease or a portal hypertension. Therefore, these diseases are separate entities also when it comes to diagnostics and therapy.

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“…According to estimates, 2-6% of patients with portal hypertension and ,1-2% of those with cirrhosis have PoPH [2][3][4][5]. PoPH is a relatively common cause of PH, representing 15% of patients enrolled in the French PAH registry [6].…”

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confidence: 99%

Efficacy, safety and pharmacokinetics of bosentan in portopulmonary hypertension

Savale

Magnier²,

Pavec

et al. 2012

Eur Respir J

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Data on treatment of patients with portopulmonary hypertension (PoPH) are limited, as they are usually excluded from randomised controlled trials with pulmonary arterial hypertension (PAH)-specific therapies. This study investigated the short-and long-term efficacy/safety of bosentan in these patients, as well as its pharmacokinetics.All 34 consecutive patients with PoPH treated with first-line bosentan (December 2002 to July 2009) were retrospectively evaluated. Assessments included the New York Heart Association functional class (NYHA FC), blood tests, haemodynamics, 6-min walk distance (6MWD) and eventfree status. The pharmacokinetics of bosentan in five patients with Child-Pugh (C-P) class B cirrhosis were compared with idiopathic PAH patients.Significant improvements from baseline were observed in NYHA FC, 6MWD and haemodynamics, and were largely maintained during follow-up. Patients with C-P class B cirrhosis (n59) had significantly larger haemodynamic improvement after mean¡SD 5¡2 months. Mean followup time was 43¡19 months; four patients died and seven patients had significant elevation of liver enzymes (annual rate 5.5%). Plasma concentrations of bosentan were higher in patients with C-P class B cirrhosis than those observed in idiopathic PAH.These data confirm the benefit of bosentan treatment for patients with PoPH. Haemodynamic improvements were particularly pronounced in patients with more severe cirrhosis. The safety profile of bosentan was consistent with previous studies.

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Portopulmonary hypertension

Cited by 24 publications

References 88 publications

Experience with inhaled iloprost and bosentan in portopulmonary hypertension

Experience with inhaled iloprost and bosentan in portopulmonary hypertension

Pulmonale Affektion bei fortgeschrittener Lebererkrankung – hepatopulmonales Syndrom und portopulmonale Hypertonie

Efficacy, safety and pharmacokinetics of bosentan in portopulmonary hypertension

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