Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study

Hascoët, Sébastien; Fournier, Éric; Jaïs, Xavier; Gloan, Lauriane Le; Dauphin, Claire; Houeijeh, Ali; Godart, François; Iriart, Xavier; Richard, A.; Radojevic, Jelena; Amedro, Pascal; Bosser, Gilles; Souletie, Nathalie; Bernard, Yvette; Moceri, Pamela; Bouvaist, Hélène; Mauran, Pierre; Barre, Elise; Basquin, Adeline; Karsenty, Clément; Bonnet, Damien; Iserin, Laurence; Sitbon, Olivier; Petit, Jérôme; Fadel, Élie; Humbert, Marc; Ladouceur, Magalie

doi:10.1016/j.acvd.2017.01.006

Cited by 40 publications

(30 citation statements)

References 24 publications

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“…The international COMPERA-CHD registry provides information on the characteristics, treatment patterns, and long-term outcomes in a large number of patients with PAH caused by CHD, in the modern treatment era. This is of paramount importance as there are relatively limited data on targeted PAH therapy and outcomes in adults with PAH resulting from CHD compared with idiopathic PAH [1,[23][24][25][26][27]. Furthermore, the existing data are mostly derived from small, uncontrolled studies with few patients, predominantly with simple congenital heart defects, and short monitoring periods [28].…”

Section: Discussionmentioning

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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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: Discussionmentioning

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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“…In advanced cases like ours, conservative treatment has been shown to be of some benefit [11]. Drugs like prostanoids, phosphodiesterase type-5 inhibitors, and endothelin receptor antagonists are targeted at modifying the course of endothelin dysfunction.…”

Section: Discussionmentioning

confidence: 91%

Secundum Atrial Septal Defect With Early Presentation of Eisenmenger Syndrome and Right-Heart Failure: A Rare Case Report and Literature Review

Malik¹,

Ikram²,

Kamal³

et al. 2020

Cureus

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Eisenmenger syndrome, the most advanced form of pulmonary arterial hypertension (PAH), poses a considerable risk to the survival and quality of life of patients. It is more commonly seen in large intra-cardiac defects like ventricular septal defects (VSD) or patent ductus arteriosus (PDA), and rarely in atrial septal defects (ASD). Early diagnosis is the single most important step in the definitive management of the condition; otherwise, only conservative treatment can be offered. In this report, we present the case of a 20-year-old female patient diagnosed with Eisenmenger syndrome secondary to a large secundum ASD. The patient responded well to medical treatment.

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“… 2 Furthermore, the prognosis in such patients with advanced disease (WHO class III or IV symptoms) is very adverse. 3 …”

Section: Introductionmentioning

confidence: 99%

Subcutaneous treprostinil in congenital heart disease-related pulmonary arterial hypertension

Skoro-Sajer

Gerges

Bálint

et al. 2018

Heart

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ObjectiveTo assess the efficacy and safety of subcutaneous treprostinil in adult patients with congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) after 12 months of treatment.MethodsConsecutive adult patients with CHD–PAH received subcutaneous treprostinil to maximum tolerated doses in an observational study.ResultsAdvanced CHD–PAH patients with WHO class III or IV disease (n=32, age 40±10 years, 20 females) received treprostinil for suboptimal response to bosentan (n=12), WHO functional class IV disease (FC, n=7) or prior to bosentan approval (n=13). In the multivariate mixed model, mean increase in 6 min walk distance (6-MWD) from baseline to 12 months was 114 m (76; 152) (P<0.001). WHO FC improved significantly (P=0.001) and B-type brain natriuretic peptide decreased from 1259 (375; 2368) pg/mL to 380 (144; 1468) pg/mL (P=0.02). In those 14 patients who had haemodynamic data before and after initiation of treprostinil, pulmonary vascular resistance decreased significantly (from 18.4±11.1 to 12.6±7.9 Wood units, P=0.003). The most common adverse events were infusion-site erythema and pain. One patient stopped treatment because of intolerable infusion-site pain after 8 months of treatment. No other major treatment-related complications were observed. Five patients died during early follow-up, having experienced a decrease in their 6-MWD prior.ConclusionsSubcutaneous treprostinil therapy is generally safe and effective for at least 12 months and may be used in CHD-related PAH class III and IV.

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Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study

Abstract: Outcomes are poor in ES, but seem better with PAH-SDT. ES with pretricuspid defects has worse outcomes despite the delayed disease onset.

Cited by 40 publications

References 24 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

Secundum Atrial Septal Defect With Early Presentation of Eisenmenger Syndrome and Right-Heart Failure: A Rare Case Report and Literature Review

Subcutaneous treprostinil in congenital heart disease-related pulmonary arterial hypertension

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