Long-Term Outcome of Lung and Heart-Lung Transplantation for Idiopathic Pulmonary Arterial Hypertension

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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“…More recent data show that survival is increased to 52-75% at 5 years and to 45-66% at 10 years [267][268][269].…”

Section: Transplantationmentioning

confidence: 99%

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

et al. 2015

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“…A single-centre study found that 123 patients with IPAH undergoing lung transplantation had worse 10-year survival compared with patients with other categories of PAH including connective tissue disease-PAH (n5102), congenital heart disease-associated PAH (n577) and chronic thromboembolic pulmonary hypertension (n514) (42% versus 70%, p50.01) [83]. However, survival of patients with PAH undergoing lung transplantation is improving with 5-and 10-year survival rates of 50-75% and 43-66%, respectively, being reported [83][84][85]. A longterm US study of 89 IPAH patients undergoing lung transplantation between 1982 and 2006 reported 5-and 10-year survival rates of 40% and 33%, respectively, in patients who underwent combined heartlung transplants [84].…”

Section: Outcomes Following Lung Transplantationmentioning

confidence: 99%

“…However, survival of patients with PAH undergoing lung transplantation is improving with 5-and 10-year survival rates of 50-75% and 43-66%, respectively, being reported [83][84][85]. A longterm US study of 89 IPAH patients undergoing lung transplantation between 1982 and 2006 reported 5-and 10-year survival rates of 40% and 33%, respectively, in patients who underwent combined heartlung transplants [84]. No significant difference in survival was observed between patients receiving heartlung, single-lung or double-lung transplants [84].…”

Section: Outcomes Following Lung Transplantationmentioning

confidence: 99%

Severe pulmonary arterial hypertension: treatment options and the bridge to transplantation

Corris

Degano

2014

Eur Respir Rev

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Pulmonary arterial hypertension (PAH) is a rare disease leading to right heart failure and death. Prognosis remains poor, particularly for patients with severe disease, i.e. World Health Organization functional class IV. There have been significant improvements in treatment options. Several agents are available that target the three main established PAH disease pathways, and can be combined sequentially or upfront. Strong scientific evidence supports the use of intravenous epoprostenol in severe PAH; however, despite recommendations, many patients do not receive parenteral prostanoids and there is a lack of evidence from randomised clinical trials supporting the value of other PAH medications alone in severe PAH.Lung transplantation is an important option in patients with severe PAH who have not responded sufficiently to therapy, or who have worsened despite maximal treatment. Bridging techniques are available for patients who worsen while awaiting transplantation. The type of bridging technique used depends on various factors including patient illness severity, physician experience and the anticipated waiting time for transplantation. With the aim to facilitate the treatment decision-making process, herein we review the medical treatment options available for patients with severe PAH, and the bridging techniques that may be used to sustain patients awaiting transplantation. @ERSpublications Current options for treatment and bridging to lung transplantation in patients with severe PAH

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“…109 Generally, heart-lung transplantation is performed when there is either significant impairment of cardiac function (inotrope dependence) or PAH is secondary to a complex congenital heart disease. 110 …”

Section: Lung Transplantationmentioning

confidence: 99%

Pulmonary hypertension—“state of the art” management in 2012

Saxena

2012

Indian Heart Journal

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A B S T R A C TPulmonary artery hypertension (PAH) is a pathological condition of small pulmonary arteries, characterised by vascular proliferation and remodelling. The pulmonary artery pressure and pulmonary vascular resistance progressively rise, leading to right heart failure and death. Pulmonary artery hypertension may be secondary to various conditions, or it may be idiopathic where no underlying cause is identifiable. Earlier, only symptomatic treatment was available for such patients which did not change the natural history of the disease. However, over the years, improvement in understanding the pathogenesis has resulted in the development of targeted approaches to the treatment of PAH. Survival advantage has also been shown with some of the pharmacologic agents. This review article discusses the current management strategy for PAH with special emphasis on an idiopathic variety, in an Indian context.

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Long-Term Outcome of Lung and Heart-Lung Transplantation for Idiopathic Pulmonary Arterial Hypertension

Cited by 87 publications

References 23 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

Severe pulmonary arterial hypertension: treatment options and the bridge to transplantation

Pulmonary hypertension—“state of the art” management in 2012

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