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

2020 ◽  
Vol 9 (5) ◽  
pp. 1456
Author(s):  
Harald Kaemmerer ◽  
Matthias Gorenflo ◽  
Dörte Huscher ◽  
David Pittrow ◽  
Christian Apitz ◽  
...  
Keyword(s):  
Overall Survival ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Combination Therapy ◽  
Real World ◽  
Congenital Heart ◽  
Real World Data ◽  
Number Of Patients

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.

scholarly journals How to initiate eHealth in congenital heart disease patients?

2020 ◽  
Vol 1 (1) ◽  
pp. 83-86
Author(s):  
Mark J Schuuring ◽  
Dirkjan Kauw
Keyword(s):  
Health Care ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Mortality Rate ◽  
High Mortality ◽  
Congenital Heart ◽  
High Morbidity ◽  
Vulnerable Patients ◽  
Number Of Patients

Abstract Patients with congenital heart disease (CHD) are a vulnerable subgroup of cardiac patients. These patients have a high morbidity and high mortality rate. As the number of patients with CHD keeps growing, while also getting older, new tools for the care and follow-up of these vulnerable patients are warranted. eHealth has an enormous potential to revolutionize health care, and particularly for CHD patients, by expanding care beyond hospital walls and even moving some of the provided care to the comfort of home. As new eHealth tools continue to grow in number, such as invasive eHealth tools, health care delivered through eHealth continues to evolve. This teaching series summarizes current insights and discusses challenges yet to be overcome. Importantly, none of them are insurmountable. This all lays ground for a promising future for eHealth in the care of patients with CHD.

Asymmetric Dimethyl-l-Arginine is a Biomarker for Disease Stage and Follow-Up of Pulmonary Hypertension Associated with Congenital Heart Disease

2015 ◽  
Vol 36 (5) ◽  
pp. 1062-1069 ◽  
Author(s):  
Zhen-fei Fang ◽  
Yi-yuan Huang ◽  
Liang Tang ◽  
Xin-qun Hu ◽  
Xiang-qian Shen ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Disease Stage

P4695Pulmonary hypertension in grown-up congenital heart disease, 2018 Nice classification. Impact on mortality. GUCH Registry data

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
L N Nicolosi ◽  
C G Moros ◽  
M C Rubio ◽  
M Pacheco Otero ◽  
M Cazalas ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Rank Test ◽  
Survival Curves ◽  
Kaplan Meier ◽  
Eisenmenger’S Syndrome ◽  
Group 5

Abstract Background Pulmonary hypertension (PH) is a serious complication of grown-up congenital heart (GUCH) diseases. Its onset is associated with a marked increase in mortality in these patients. Purpose To classify GUCH patients with pulmonary hypertension (PH) according to PH subgroups, and explore association between PH and mortality during follow up. Methods An observational retrospective study was conducted on patients on the GUCH database. Overall data were analyzed. Patients diagnosed with PH were selected and classified according to the 2018 Nice HT guidelines on pulmonary arterial hypertension secondary to congenital heart disease: Group 1 (PAH1) and its 4 subgroups (SG1: Eisenmenger's syndrome; SG2: left to right shunts; SG3: PAH associated with congenital heart disease; SG4: post-operative PAH); Group 2 (PH2), Group 3 (PH3), Group 4 (PH4), Group 5 (PH5). Statistical analysis: Kaplan Meier Log Rank test using SPSS 20 software. Results The database included 2073 patients (P), mean age 31.8±12.9 years, 54.2% (1123 P) were women. Overall mortality: 4.2% (88 P). Follow up: 44.1±37 months. Results showed 5.1% of patients (105 P) had PH, 3.8% (79 P) of whom corresponded to group PAH1, and 1.3% (26 P) to the remaining groups. Analysis of P with PH showed that 67.6% (71 P) were women, mean age was 41.3±14.7 years, mortality during follow up was 25.7% (27 P). Classification according to group showed 75.2% (79) corresponded to PAHG1, 9.5% (10) to PH2, 0% to PH3, 1% (1) to PH4, and 14.3 (15) to PH5. Analysis of P in PAHG1 showed a 43% (34 P) prevalence of SG1, 27.8% (22 P) of SG2, 8.9% (7 P) of SG3, and 20.3% (16 P) of SG4. Mortality per group was 22.8% (18 P) in PAH1, 20% (2 P)in PH2, 0% in PH 3, 0% in PH 4, and 46.7% (7 P) in PH 5. Kaplan Meier survival curves showed significant association between PH and mortality, Log Rank=0.000, and worst survival in groups PH2, PAH1 and PH5. No differences in mortality were observed among PAH1 subgroups, Log Rank= 0.458. Survival curves Conclusions PH negatively affected survival of GUCHpatients. Analysis of mortality during follow up showed patients in group PH5 to have the worst outcomes, possible because patients with a single ventricle were included in this group. Group PAH1 comprised 75% of PH patients, with a greater predominance of patients with Eisenmenger's syndrome, but no significant differences in mortality during follow-up among the 4 subgroups.

scholarly journals Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

2014 ◽  
Vol 32 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Felipe Alves Mourato ◽  
Lúcia Roberta R. Villachan ◽  
Sandra da Silva Mattos
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Diseases ◽  
Heart Defects ◽  
Descriptive Analysis ◽  
Atrioventricular Septal Defect

OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

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