The Adult Patient with Eisenmenger Syndrome: A Medical Update after Dana Point Part II: Medical Treatment - Study Results

Mebus, Siegrun; Schulze‐Neick, Ingram; Oechslin, Erwin; Niwa, Koichiro; Trindade, Pedro T.; Hager, Alfred; Hess, John; Kaemmerer, Harald

doi:10.2174/157340310793566163

Cited by 16 publications

(11 citation statements)

References 57 publications

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“…The use of calcium channel blockers is, in fact, discouraged in ES, as they may cause significant peripheral vasodilatation and hypoxia. 50 So far, only three pathomechanistic pathways in PAH have been translated into clinical practice: prostacyclin, nitric oxide, and endothelin pathways. Oral and parenteral therapies have been designed to target these pathways, namely prostanoids, phosphodiesterase type-5 inhibitors (PDE-5i), and ERAs.…”

Section: Disease-specific Targeted Pah Therapymentioning

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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Section: Disease-specific Targeted Pah Therapymentioning

confidence: 99%

Eisenmenger syndrome: current perspectives

Nashat¹,

Kempny²,

McCabe³

et al. 2017

RRCC

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show abstract

“…The use of calcium channel blockers is, in fact, discouraged in ES, as they may cause significant peripheral vasodilatation and hypoxia. 65 So far, only three pathways in PAH have been translated into clinical practice: the prostacyclin pathway, the nitric oxide pathway, and the endothelin pathway. Oral and parenteral therapies have been designed to target these pathways, namely, prostanoids, phosphodiesterase type-5 inhibitors (PDE-5i), and endothelin receptor antagonists (ERAs).…”

Section: Advanced Therapiesmentioning

confidence: 99%

Pulmonary Arterial Hypertension Complicating Congenital Heart Disease: Advances in Therapy

Nashat

Brida

Price

et al. 2017

Semin Respir Crit Care Med

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Congenital heart disease (CHD) is the most common inborn defect, affecting approximately 1% of all newborns worldwide. Advances in its diagnosis and treatment have led to dramatic improvements in patients' quality of life and long-term survival. Nevertheless, despite these innovations many patients require life-long follow-up and are at risk of numerous complications, namely, residual or progressive hemodynamic lesions, arrhythmia, sudden cardiac death, and the development of heart failure and pulmonary arterial hypertension (PAH), despite timely surgical intervention. Ill advisedly, some patients are also lost to follow-up, assuming that their early life surgical or catheter-based intervention was curative, hence missing out on invaluable lifetime screening and timely interventions that may be necessary. Additionally, there are many patients with undiagnosed or unoperated CHD in the developing world presenting later in life with irreversible complications. Even in this modern era of diagnosis and screening, PAH-CHD remains a burden in patients with CHD, with approximations of up to 10% in this heterogeneous group and is associated with an adverse impact on quality of life and survival.

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“…Die schwerste Form und das Endstadium dieser Veränderungen ist das Eisenmenger-Syndrom, das häufig im zweiten Lebensjahrzehnt klinisch manifest wird [8,10].…”

Section: Pulmonalarterielle Hypertonie (Pah) Bei Angeborenen Herzfehlunclassified

Pulmonalarterielle Hypertonie (PAH) bei angeborenen Herzfehlern mit Shuntverbindung: eine heterogene und komplexe Konstellation

Zimmermann¹,

Schranz²,

Ewert

et al. 2013

Dtsch med Wochenschr

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The Adult Patient with Eisenmenger Syndrome: A Medical Update after Dana Point Part II: Medical Treatment - Study Results

Cited by 16 publications

References 57 publications

Eisenmenger syndrome: current perspectives

Eisenmenger syndrome: current perspectives

Pulmonary Arterial Hypertension Complicating Congenital Heart Disease: Advances in Therapy

Pulmonalarterielle Hypertonie (PAH) bei angeborenen Herzfehlern mit Shuntverbindung: eine heterogene und komplexe Konstellation

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