Pulmonary hypertension and congenital heart disease: An insight from the REHAP National Registry

Alonso‐Gonzalez, Rafael; López-Guarch, Carmen Jiménez; Subirana-Domènech, Maria Teresa; Oliver, José M.; González, Isabel Otero; Cubero, Javier Segovia; Cerro, María Jesús del; Salvador, María Lázaro; Subirà, Laura Dos; Gallego, Pastora; Escribano, Pilar

doi:10.1016/j.ijcard.2015.02.031

Cited by 91 publications

(78 citation statements)

References 30 publications

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“…This may explain why patients with Eisenmenger syndrome from Cardiac MRI in Eisenmenger Syndrome post-tricuspid shunts have a better prognosis than those with pretricuspid shunts or idiopathic PAH. 3 This current investigation is novel in its use of CMR variables to predict mortality in patients with Eisenmenger syndrome. In a previous large case-controlled study, 1 this group of investigators were unable to demonstrate a relationship between semiquantitative echocardiographic measures of ventricular function and outcomes in a more heterogeneous group of patients with Eisenmenger syndrome.…”

Section: See Article By Jensen Et Almentioning

confidence: 99%

“…Patients with pretricuspid shunts (atrial septal defects and partial anomalous pulmonary venous return) were excluded because these represent a rarer form of Eisenmenger syndrome with different physiology and a poorer prognosis. 3 Each subject underwent a series of clinical investigations, including echocardiography, a 6-minute walk test, laboratory studies, and a cardiac magnetic resonance (CMR) examination. All subjects were censored for mortality 8 to 10 years after enrollment, and 12 subjects (25%) died during the study period.…”

Section: See Article By Jensen Et Almentioning

confidence: 99%

“…1,2 Despite the grim prognosis, those with Eisenmenger syndrome live much longer with their disease than patients with other forms of severe pulmonary arterial hypertension (PAH). 3 Importantly, the natural history of Eisenmenger syndrome is better than that of combined heart and lung transplantation. 4,5 Therefore, conservative management for years, or even decades, is often appropriate in Eisenmenger syndrome patients who have degrees of pulmonary hypertension that would trigger transplantation consideration in other forms of PAH.…”

mentioning

confidence: 99%

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The Sophistication of Simplicity

Krieger

Valente

2015

Circ: Cardiovascular Imaging

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Section: See Article By Jensen Et Almentioning

confidence: 99%

Section: See Article By Jensen Et Almentioning

confidence: 99%

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confidence: 99%

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The Sophistication of Simplicity

Krieger

Valente

2015

Circ: Cardiovascular Imaging

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“…In an echocardiographic study of 191 patients with ES and noncomplex CHD, the location of the defect was associated with different physiological adaptation of the RV to the PAH and, possibly, a different prognosis: pre-tricuspid shunts were older, had larger RVs, and a trend toward worse prognosis when compared to post-tricuspid lesions. 10,11 Indeed, the mechanisms behind shunt reversal in ES ASD patients differs significantly to post-tricuspid shunts, depending significantly on the relative compliance of the left and right ventricles and, perhaps, less so on the ratio between systemic and pulmonary resistances.…”

Section: Anatomical Variations In Patients With Es and Outcomementioning

confidence: 99%

Eisenmenger syndrome: current perspectives

Nashat¹,

Kempny²,

McCabe³

et al. 2017

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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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“…These are patients in who PAH persists or develops after repair of the defect. PVD may be mild to severe, and the absence of a defect means that the right ventricle (RV) lacks a "relief valve" [1,23]. Hence, depending on the severity of the PVD, clinical presentation can be quite severe and rapidly progressive compared to adult ES and demand aggressive management [7].…”

Section: A Eisenmenger Syndromementioning

confidence: 99%

The spectrum of pulmonary arterial hypertension in adults with congenital heart disease: management from a physician and nurse specialist perspective

Dimopoulos

Harries

Parfitt

2017

J Congenit Heart Dis

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Congenital heart disease (CHD) is one of the most common causes of pulmonary arterial hypertension (PAH), with a prevalence nearing that of idiopathic PAH in registries. Despite similarities in pulmonary vascular pathology, PAH-CHD differs from idiopathic PAH and other types of PAH. In fact, PAH-CHD is a term that includes a wide spectrum of conditions and pathophysiologies, ranging from Eisenmenger syndrome to milder forms of PAH with systemic-topulmonary shunts, to those with previously repaired defects. Significant expertise in both CHD and PAH is required to manage these patients. We provide an overview of the modern management of the wide spectrum of PAH-CHD in adults, both from a physician and clinical nurse specialist perspective.

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Pulmonary hypertension and congenital heart disease: An insight from the REHAP National Registry

Cited by 91 publications

References 30 publications

The Sophistication of Simplicity

The Sophistication of Simplicity

Eisenmenger syndrome: current perspectives

The spectrum of pulmonary arterial hypertension in adults with congenital heart disease: management from a physician and nurse specialist perspective

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