Heart–lung or lung transplantation for Eisenmenger syndrome

Waddell, Thomas K.; Bennett, Leah E.; Kennedy, Renee; Todd, Thomas R.J.; Keshavjee, Shaf

doi:10.1016/s1053-2498(01)00420-x

Cited by 143 publications

(85 citation statements)

References 9 publications

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“…Although patients with Eisenmenger's syndrome due to simple shunts have been treated by isolated lung transplantation and repair of the cardiac defect [174], patients with ventricular septal defects may have a better outcome with heart-lung transplantation [175].…”

Section: Transplantationmentioning

confidence: 99%

Guidelines for the diagnosis and treatment of pulmonary hypertension

Galiè¹,

Hoeper²,

Humbert³

et al. 2012

Revista Portuguesa de Cardiologia (English Edition)

419

738

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Section: Transplantationmentioning

confidence: 99%

Guidelines for the diagnosis and treatment of pulmonary hypertension

Galiè¹,

Hoeper²,

Humbert³

et al. 2012

Revista Portuguesa de Cardiologia (English Edition)

419

738

View full text Add to dashboard Cite

“…In a a worldwide registry of 605 transplanted ES patients over a 10-year period, Waddell et al found that, in this heterogeneous group, post-transplantation survival related to the underlying cause of the ES, and HLT appeared to be better than LT with shunt correction: 30 days and 1-year survival rates were 80.7% and 70.1% compared with 68% and 55.2%, respectively. 47 In another study of 51 patients with ES transplanted in the UK, the 1-, 5-, and …”

Section: Transplantationmentioning

confidence: 99%

Eisenmenger syndrome: current perspectives

Nashat¹,

Kempny²,

McCabe³

et al. 2017

RRCC

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Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). It results from a cardiac defect allowing significant systemic-to-pulmonary (left-to-right) shunting, which triggers the development of pulmonary vascular disease (PVD) if the defect is not repaired in a timely fashion. Once severe PVD has developed, the defect cannot be repaired. With advances in pediatric cardiology and surgery, the prevalence of ES is steadily falling in developed countries; nonetheless, there will always be patients who are unsuitable for repair at the time of diagnosis, or emigrating from countries with less advanced healthcare, who will develop ES. ES is a multisystem disorder causing chronic hypoxemia and reduced cardiac output resulting in significant morbidity and mortality. While lung (plus defect repair) or combined heart and lung transplantation is thought be the definitive treatment for ES, transplant organs are a limited resource and long-term results are still suboptimal. PAH pharmacotherapy was, until quite recently, largely directed at symptomatic relief and had no impact on morbidity and mortality. Targeted PAH therapies have recently been proven to be beneficial in various forms of PAH in terms of functional status, progression of disease, and prognosis. Data on the effect of PAH therapies in the ES cohort remain limited, but available studies demonstrate evidence of improvement in symptoms, exercise capacity, and some evidence of survival benefit. ES patients should be followed in specialized centers, by means of an interdisciplinary approach by clinicians experienced in PAH and CHD. However, local physicians working in cardiology, respiratory medicine, primary care, and emergency services are likely to encounter ES patients and need to be aware of the main issues and pitfalls in their care. The authors present an overview of the management of ES, focusing on the most common issues and complications.

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“…The success of transplantation varies depending on the underlying cause of ES, and appears to be most beneficial for patients with VSD or multiple congenital abnormalities. 66 Overall, transplantation in patients with ES is associated with high perioperative mortality. 67 However, studies suggest that, although the postoperative course tends to be complicated in these patients, short-and long-term survival rates following HLT are similar to those reported in non-ES recipients.…”

Section: Thoracic Organ Transplantationmentioning

confidence: 99%

“…Five-and 10-year survival rates are 51% and 28%, respectively, after HLT. 66,68 Given the paucity of suitable donor organs, the small number of suitable recipients and the poor prognosis following HLT, any means to delay the need for HLT in ES patents would be very welcome. One recent retrospective analysis suggests that ES patients who received novel, advanced therapies including prostacyclin analogues and endothelin receptor antagonists may benefit from significantly longer mean times to death or inscription on the active waiting list, by comparison with patients without.…”

Section: Thoracic Organ Transplantationmentioning

confidence: 99%