Ambrisentan Improves Exercise Capacity and Symptoms in Patients with Portopulmonary Hypertension

Halank, Michael; Knudsen, Lars; Seyfarth, HJ; Ewert, Ralf; Wiedemann, Baerbel; Kolditz, Martin; Höffken, G.; Hoeper, Marius M.

doi:10.1055/s-0031-1273393

Cited by 38 publications

(27 citation statements)

References 24 publications

(23 reference statements)

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“…Anecdotal reports suggest that ERAs, PDE-5is, sGC stimulators and prostacyclin analogues may be used in this patient population [214,[350][351][352][353][354][355][356]. This includes potentially hepatotoxic drugs such as bosentan, but it should be noted that this compound tends to accumulate in patients with severely impaired liver function (i.e.…”

Section: Therapymentioning

confidence: 99%

“…The absence of viral particles in the complex plexiform lesions found from these patients suggests that an indirect action of viral infection on inflammation and growth factors may act as a trigger in a predisposed patient. [344] It is recommended that the treatment algorithm for patients with other forms of PAH should be applied to patients with PAH associated with portal hypertension, taking into account the severity of liver disease C I [214,[350][351][352][353][354][355][356] Liver transplantation may be considered in selected patients responding well to PAH therapy C IIb [361][362][363] It is recommended that patients affected by PAH associated with portal hypertension should be referred to centres with expertise in managing both conditions C I [344] Anticoagulation is not recommended in patients with PH associated with portal hypertension C III [365] Liver transplantation is contraindicated in patients with severe and uncontrolled PAH C III [361][362][363] PAH: pulmonary arterial hypertension; PH: pulmonary hypertension. with other risk factors for PAH, such as liver disease (chronic hepatitis B or C), exposure to drugs or toxins or PE due to i.v.…”

Section: Pulmonary Arterial Hypertension Associated With Human Immunomentioning

confidence: 99%

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2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

et al. 2015

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Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.

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Section: Therapymentioning

confidence: 99%

Section: Pulmonary Arterial Hypertension Associated With Human Immunomentioning

confidence: 99%

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

et al. 2015

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“…Pojedyncze doniesienia sugerują, że ERA, inhibitory PDE-5, stymulatory rozpuszczalnej cyklazy guanylowej i analogi prostacykliny mogą być stosowane w tej populacji chorych [214,[350][351][352][353][354][355][356]. Zalecenie to obejmuje potencjalnie hepatotoksyczne leki takie jak bosentan, ale należy zauważyć, że związek ten ma tendencję do gromadzenia się u pacjentów z ciężkimi zaburzeniami czynności wątroby (tj.…”

Section: Leczenieunclassified

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Galiè¹,

Humbert²,

Vachiéry³

et al. 2015

Kardiol Pol

356

528

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“…Наиболее характер-ные признаки -одышка, утомляемость, снижение толерантности к физической нагрузке, синкопальные состояния [18]. Синкопальные состояния могут быть единственным проявлением легочной гипертензии.…”

Section: российский вестник перинатологии и педиатрии 2016; 61:5 Rosunclassified

Portopulmonary hypertension in a child

Садыкова¹,

Сабирова²,

Шакирова³

et al. 2016

Ross. vestn. perinatol. pediatr.

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Статья посвящена проблеме портопульмональной гипертензии (легочная артериальная гипертензия, связанная с порталь-ной гипертензией). Легочная гипертензия является редким, прогностически неблагоприятным осложнением портальной гипертензии, независимо от этиологического фактора последней. Диагностическими критериями портопульмональной ги-пертензии являются: наличие портальной гипертензии с или без заболевания печени, среднее давление в легочной артерии более 25 мм рт. ст. в покое, нормальные показатели давления заклинивания в легочной артерии (<15 мм рт. ст), легочное сосудистое сопротивление более 3 ед. Вуда/м 2 . Цель работы: описание развития клинической картины, диагностики и лече-ния портопульмональной гипертензии у больного на фоне корригированной портальной гипертензии. Существуют трудности в постановке диагноза портопульмональной гипертензии, так как ее симптомы неспецифичны. Наиболее характерные при-знаки -одышка, утомляемость, снижение толерантности к физической нагрузке, синкопальные состояния. DOI: 10.21508/1027DOI: 10.21508/ -4065-2016 The paper deals with portopulmonary hypertension (pulmonary arterial hypertension associated with portal hypertension). Pulmonary hypertension is a rare, prognostically unfavorable complication of portal hypertension, regardless of the etiological factor of the latter. The diagnostic criteria for portopulmonary hypertension are portal hypertension with or without liver disease; a resting mean pulmonary artery pressure >25 mm Hg; a normal pulmonary artery wedge pressure <15 mm Hg; and a pulmonary vascular resistance of more than 3 Wood units/m 2 . Objective: to describe of the development of the clinical picture, diagnosis, and treatment of portopulmonary hypertension in a patient during corrected portal hypertension. There are challenges in the diagnosis of portopulmonary hypertension because its symptoms are nonspecific. The most characteristic symptoms of the disease are dyspnea, fatigue, exercise intolerance, and syncope. Keywords: children, pulmonary arterial hypertension, portopulmonary hypertension, endothelin receptor antagonists, phosphodiesterase 5 inhibitors.

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Ambrisentan Improves Exercise Capacity and Symptoms in Patients with Portopulmonary Hypertension

Abstract: The present study demonstrates significant improvement of exercise capacity and clinical symptoms without relevant safety concerns during ambrisentan treatment in patients with PoPH.

Cited by 38 publications

References 24 publications

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Portopulmonary hypertension in a child

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