Congenital heart disease and pulmonary hypertension among Down syndrome pediatric patients

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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Efficacy of citrulline supplementation to decrease the risk of pulmonary hypertension after congenital heart disease surgery. A local experience.

Revista de la Facultad de Ciencias Médicas de Córdoba ◽

10.31053/1853.0605.v77.n4.27936 ◽

2020 ◽

Vol 77 (4) ◽

pp. 249-253

Author(s):

Silene Silvera Ruiz ◽

Carola L Grosso ◽

Margot Tablada ◽

Marcelo Cabrera ◽

Raquel Dodelson de Kremer ◽

...

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Congenital Heart ◽

Pediatric Patients ◽

Reference Level ◽

Plasma Citrulline ◽

Post Surgery ◽

Group A ◽

Group B

Pulmonary hypertension (PH) is a major cause of morbi-mortality among patients with congenital heart disease (CHD) and also a potentially severe complication after surgical repair. Oral citrulline, a precursor to NO synthesis, is safe and efficacious for decreasing the risk of postoperative PH. Objective: The aim of the present study was to investigate in pediatric patients the changes of plasma citrulline, arginine, homocysteine and nitric oxide (NO) metabolites and pulmonary artery pressures (PAP) pre-post cardiac surgery in order to describe our population status with regard to the risk of pulmonary hypertension and look for potential biomarkers for early detection and treatment. Main results/Discussion: 16 Argentine pediatric patients with CHD undergoing cardiopulmonary bypass were randomized in two groups: (A) with and (B) without perioperative citrulline supplementation. We found that plasma citrulline median levels before surgery were lower in both groups respect to referential values, probably due to the poor nutritional status of our patients; only group A surpassed post-surgery the minimum recommended level to avoid PH. Furthermore, none of the patients in group A showed mean PAP higher than 20 mmHg, whereas in group B, 67% of the measurements were ≥ than the reference level. Conclusions: We reaffirm that citrulline supplementation it is effective in reducing postoperative pulmonary hypertension and biomarkers could evidence patient status as a translational medicine application.

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

Journal of Perinatal Medicine ◽

10.1515/jpm.2004.030 ◽

2004 ◽

Vol 32 (2) ◽

Cited By ~ 46

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

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

Journal of Advances in Medicine and Medical Research ◽

10.9734/jammr/2020/v32i2030676 ◽

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.

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

Nepal Journal of Medical Sciences ◽

10.3126/njms.v2i2.8944 ◽

2013 ◽

Vol 2 (2) ◽

pp. 96-101 ◽

Cited By ~ 1

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

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The diagnostic and prognostic role of amino-terminal pro-brain type natriuretic peptides in pediatric cardiac diseases and pulmonary hypertension

Heart Vessels and Transplantation ◽

10.24969/hvt.2021.244 ◽

2021 ◽

Vol 5 (Volume 1) ◽

pp. 21

Author(s):

Kayirnisa Tilemanbetova ◽

Taalaibek Kudaiberdiev ◽

Jalil Sheishenov ◽

Damirbek Abibillaev

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Natriuretic Peptide ◽

Congenital Heart ◽

Pediatric Patients ◽

Heart Diseases ◽

Congenital Heart Diseases ◽

Amino Terminal

Objective: Several studies showed that N-terminal pro-brain type natriuretic peptide (NT-pro-BNP) as the reliable marker both in diagnostic and prognostic workup of pediatric patients with congenital heart disease complicated by pulmonary hypertension. Furthermore, it has well assisted both in therapeutic and surgical management of children with congenital heart diseases. In this review, we aimed to analyze recent studies concerning the role of the NT-pro-BNP in pediatric cardiac patients with pulmonary hypertension. Methods: PubMed, Cochrane, Science Direct and Google Scholar databases were used with the searching of the following words: “amino terminal pro brain type natriuretic peptide”, “pediatric pulmonary hypertension”, “congenital heart diseases”, emphasizing on original research articles published within recent 15 years and in English language. We critically analyzed and summarized the retrieved studies. Conclusion: NT-pro-BNP remains as the reliable diagnostic and prognostic marker of pulmonary hypertension and congenital heart diseases in pediatric patients. NT-pro-BNP and BNP remain as the universal marker of cardiovascular pathology in pediatric population. Improvement of their effectiveness in diagnostic workup in patients with CHD and PH undergoing surgery needs further clarification. Key words: pediatric pulmonary hypertension, N-terminal pro brain type natriuretic peptide, congenital heart disease

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