Pulmonary Hypertension in Congenital Heart Disease

Pascall, Emma; Tulloh, Robert

doi:10.2217/fca-2017-0065

Cited by 80 publications

(71 citation statements)

References 58 publications

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“…Other important predisposing factors for pulmonary vascular disease in CHD include previous Fontan operation and left heart obstructive disease causing post-capillary PH. A further distinction from other forms of PAH is that, in CHD, timing and extent of the development of PAH depends on various different factors, e.g., the pre-or post-tricuspid localization of the primary left-to right shunt [39]. For example, Eisenmenger syndrome will evolve even in large defects at the atrial level much later in life compared with defects at the ventricular or great artery levels.…”

Section: Demographics Hemodynamics and Treatmentmentioning

confidence: 99%

“…At that point, the therapeutic approach for adults with PAH and CHD differs from the approach for those with idiopathic PAH, as there are only few data to support routine up-front/early sequential oral combination therapy for Eisenmenger syndrome [1,6,26,[39][40][41][42]. In adults with PAH and CHD, sequential combination therapy is frequently initiated only upon symptomatic deterioration or if predefined, and often center-specific treatment goals are not achieved [43].…”

Section: Demographics Hemodynamics and Treatmentmentioning

confidence: 99%

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Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

Kaemmerer

Gorenflo

Huscher

et al. 2020

JCM

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Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. Methods and results: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients’ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan–Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. Conclusions: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.

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Section: Demographics Hemodynamics and Treatmentmentioning

confidence: 99%

Section: Demographics Hemodynamics and Treatmentmentioning

confidence: 99%

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

Kaemmerer

Gorenflo

Huscher

et al. 2020

JCM

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“…The imbalance of these vasoactive substances in the pulmonary vasculature results in net vasoconstriction and pulmonary vascular resistance elevation. Shear stress from persistently high flows leads to endothelial cell injury and the activation and repression of genes that participate in the vascular remodeling process, and is believed to play a role in various forms of PAH (33)(34)(35). Exposure of and damage to the underlying arterial smooth muscle results in smooth muscle proliferation and thickening of the tunica intima, media, and adventitia within the pulmonary vasculature (4).…”

Section: Pathogenesis Of Pophmentioning

confidence: 99%

Portopulmonary Hypertension: From Bench to Bedside

et al. 2020

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Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension and is a subset of Group 1 pulmonary hypertension (PH). PoPH is a cause of significant morbidity and mortality in patients with portal hypertension with or without liver disease. Significant strides in elucidating the pathogenesis, effective screening algorithms, accurate diagnoses, and treatment options have been made in past 20 years. Survival of PoPH has remained poor compared to IPAH and other forms of PAH. Recently, the first randomized controlled trial was done in this patient population and showed promising results with PAH specific therapy. Despite positive effects on hemodynamics and functional outcomes, it is unclear whether PAH specific therapy has a beneficial effect on long term survival or transplant outcomes. In this review, we will discuss the epidemiology, pathophysiology, clinical and hemodynamic characteristics of PoPH. Additionally, this review will highlight the lacunae in our current management strategy, challenges faced and will provide direction to potentially useful futuristic management strategies.

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“…For patients with ASDs, the incidence of PH is greater in those with sinus venosus defects (16%) than in those with ostium secundum defects (4%). In adults, the estimated prevalence of PH associated with unrepaired CHD ranges from 1.6 to 12.5 cases per million adults, with 25-50% of this population affected by the most extreme form, Eisenmenger syndrome [21][22][23].…”

Section: Anatomical Hemodynamic and Systemic Abnormalities Which Coumentioning

confidence: 99%

“…It is considered to be severe when < 85%. This is a consequence of unrepaired cyanotic CHDs, persistent venous-arterial shunts, low pulmonary flow CHDs, PH and Eisenmenger syndrome [22].…”

Section: Systemic Arterial Hypertensionmentioning

confidence: 99%

Impact of the SARS-Cov-2 Virus Pandemic on Children and Adults with Congenital Heart Disease: Its Burden and Risk Factors

Jairo¹

2020

Int J Clin Cardiol

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The novel coronavirus causes severe acute respiratory syndrome 2, and can infect any population. With the currently available information on the behavior of the pandemic, we conclude that the virus has come to stay. It is clear that the most aggressive behavior and worst outcome of the infection occurs in already established risk groups, such as those with cardiovascular disease. Those with congenital heart disease make up a special risk group. Due to this group's high heterogeneity and the paucity of published cases related to coronavirus infection, its ultimate behavior cannot yet be known. Based on the anatomical and hemodynamic abnormalities with systemic repercussions which accompany congenital heart disease, this review was carried out with the aim of describing and analyzing this population's risk factors and potentially increased vulnerability to coronavirus infection. Systemic hematological abnormalities, along with the prothrombotic state, proinflammatory state, and altered immune response in cyanotic congenital heart disease are highlighted; along with the arrhythmias, pulmonary hypertension, underlying heart failure and target organ damage, among others, present in unrepaired congenital heart disease. This review searched for articles in PubMed, Science Direct, SciELO, MD Consult and Google Scholar using the MeSH terms "coronavirus disease 2019", "COVID-19", "SARS-CoV-2 pandemic", "coronavirus and cardiovascular system", "congenital heart diseases", "hematologic disorders in congenital heart disease", and "pro-inflammatory mediators". Finally, a brief description of the collateral effects of the pandemic on the care of this population is provided.

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Pulmonary Hypertension in Congenital Heart Disease

Cited by 80 publications

References 58 publications

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

Portopulmonary Hypertension: From Bench to Bedside

Impact of the SARS-Cov-2 Virus Pandemic on Children and Adults with Congenital Heart Disease: Its Burden and Risk Factors

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