Clinical characteristics and follow up of Down syndrome infants without congenital heart disease who presented with persistent pulmonary hypertension of newborn

2004 ◽  
Vol 32 (2) ◽  
Author(s):  
P. S. Shah ◽  
J. Hellmann ◽  
I. Adatia
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Clinical Characteristics ◽  
Congenital Heart ◽  
Persistent Pulmonary Hypertension

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.

scholarly journals Nutritional Status and Pulmonary Hypertension in Children with Down Syndrome Presenting with Congenital Heart Disease: Retrospective Study

2020 ◽  
pp. 12-18
Author(s):  
J. M. Chinawa ◽  
O. C. Duru ◽  
B. F. Chukwu ◽  
A. T. Chinawa
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Retrospective Study ◽  
Nutritional Status ◽  
Congenital Heart ◽  
Children With Down Syndrome ◽  
Increased Risk ◽  
Hypertension In Children

Introduction: Children with Down syndrome are predisposed to having congenital heart defect. Objectives: This study is aimed to describe the clinical correlates, nutritional status and pulmonary hypertension in children with Down syndrome who presented with congenital heart disease. Patients and Methods: A retrospective study of children with Down syndrome who presented with congenital heart disease from 2016 to 2020 was carried out. Nutritional status was assessed with WHO Anthro software while pulmonary hypertension was assessed with standard procedures. Results: Out of 758 echocardiography done over the period of 5 years for children suspected of having cardiac disease, three hundred and eight one had confirmed congenital heart disease of which twenty-eight of them had Down syndrome 7.34% (28/381).  Ten 10/28 (35.7%) of them had pulmonary hypertension. This is commonly noted among infants than older ages. Among 28 children with Down syndrome, twenty-three had complete information for weight and height which was used to assess their nutritional status, 47.8% (11/28) presented with wasting and stunted, 8.7% (2/28) of those with Down syndrome were wasted and 8.7% (2/28) with stunting. Down syndrome is commoner in children with AV canal defect 50% (14/28) followed by PDA 21.4% (6/14). Fast breathing 86.7% (13/15) as the most common symptom followed by cough 64.3% (9/14) Conclusion: Children with Down syndrome who had congenital heart disease are at increased risk of malnutrition and pulmonary hypertension.

scholarly journals Pulmonary Interstitial Glycogenosis: An Unrecognized Etiology of Persistent Pulmonary Hypertension of the Newborn in Congenital Heart Disease?

2012 ◽  
Vol 34 (5) ◽  
pp. 1254-1257 ◽  
Author(s):  
Monique R. Radman ◽  
Patricia Goldhoff ◽  
Kirk D. Jones ◽  
Anthony Azakie ◽  
Sanjeev Datar ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Persistent Pulmonary Hypertension ◽  
Pulmonary Interstitial Glycogenosis

scholarly journals Down Syndrome and Congenital Heart Disease: Single centre, Prospective Study

2013 ◽  
Vol 2 (2) ◽  
pp. 96-101 ◽  
Author(s):  
Maniah Shrestha ◽  
U Shrestha
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Prospective Study ◽  
Congenital Heart ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Atrioventricular Septal Defect ◽  
Children With Down Syndrome

Background: The objective of this study was to evaluate the children with down syndrome regarding the frequency and types of congenital heart disease and associated pulmonary hypertension. Method: A prospective study was carried out to all the children with down syndrome visited in pediatric outpatient department over the period of one year. Necessary data were recorded in preformed format. Results: Fifty children with down syndrome were evaluated. Forty (80%) had an associated congenital heart disease. The median age at diagnosis was 2 years (range = 4 days to 12 years). In 26 patients (65%), the cardiac lesion was isolated, while 14 patients (35%) had multiple defects. The most common single defect was ventricular septal defect (VSD), found in 9 of the 40 patients (22.5%), followed by atrioventricular septal defect (AVSD) in 15%, atrial septal defect (ASD) and patent ductus arteriosus (PDA) each in 10%. The most frequent concomitant malformation found co-existing with other congenital cardiac lesions was PDA (15%). Pulmonary hypertension was found in 21 of 40 patients (52.5%) and AVSD was most frequently associated with pulmonary hypertension. Conclusion: Congenital heart disease is very common in patient with down syndrome. VSD is the most common cardiac defect and AVSD is second to VSD. Patient with down syndrome with CHD frequently develop pulmonary hypertension at younger age hence early cardiac screening by echocardiography in these patients is crucial. Early diagnosis and management is the key to avoid irreversible hemodynamic consequences of the defect. Nepal Journal of Medical Sciences | Volume 02 | Number 02 | July-December 2013 | Page 96-101 DOI: http://dx.doi.org/10.3126/njms.v2i2.8944

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.

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 SARS-CoV-2 Infection in Patients with Down Syndrome, Congenital Heart Disease, and Pulmonary Hypertension: Is Down Syndrome a Risk Factor?

2020 ◽  
Vol 225 ◽  
pp. 246-248 ◽  
Author(s):  
Usha S. Krishnan ◽  
Sankaran S. Krishnan ◽  
Shipra Jain ◽  
Mara B. Chavolla-Calderon ◽  
Matthew Lewis ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Risk Factor ◽  
Heart Disease ◽  
Congenital Heart
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