Down’s Syndrome with Congenital Heart Disease: Our Surgical Experience

Background: Our objective was to analyze the outcome of patients of Down’s syndrome with congenital heart diseases undergoing cardiac surgery. Methods: This was a retrospective study conducted between January 2013 and June 2019. 49 consecutive patients with Down’s syndrome with congenital heart disease admitted in pediatric cardiac surgery unit at National Institute of Cardiovascular Diseases (NICVD). Patients were followed up postoperatively for in-hospital outcome. Results: Among 49 patients the heart lesion ranked in incidence as follows- VSD 24(48.97%), AV canal defect 12(24.48%), TOF 6(12.24%), PDA 6(12.24%) and ASD 1(2.04%). Pulmonary hypertension was found in 63.25% patients. Moderate pulmonary hypertension was most common, found in 18(38.66%) patients. Severe and mild pulmonary hypertension was found in 10(32.38%) and 3(9.67%) patients respectively. All the patients had undergone surgical correction. The postoperative period was complicated in 44.89% of patients. The most frequent complication was pulmonary infection 20.40%, Wound infection 6.12% and low output syndrome 6.12% were the next. One patient had postoperative heart block, needed permanent pace maker implantation. In-hospital mortality was 12.24%. Conclusion: Patients with Down’s syndrome with congenital heart disease undergoing surgical correction had an acceptable postoperative morbidities and mortality. Cardiovasc. j. 2020; 13(1): 35-39

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Prevalence of Congenital Heart Disease and Pulmonary Hypertension in Down's Syndrome: An Echocardiographic Study

Journal of Cardiovascular Ultrasound ◽

10.4250/jcu.2015.23.2.72 ◽

2015 ◽

Vol 23 (2) ◽

pp. 72 ◽

Cited By ~ 22

Author(s):

Nilda Espinola-Zavaleta ◽

María Elena Soto ◽

Angel Romero-Gonzalez ◽

Lidia del Carmen Gómez-Puente ◽

Luis Muñoz-Castellanos ◽

...

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Congenital Heart ◽

Down's Syndrome ◽

Down’S Syndrome ◽

Echocardiographic Study

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The association of congenital heart disease with down's syndrome (mongolism)

The American Journal of Cardiology ◽

10.1016/0002-9149(69)90428-7 ◽

1969 ◽

Vol 24 (3) ◽

pp. 354-357 ◽

Cited By ~ 37

Author(s):

Lawrence Cullum ◽

Jerome Liebman

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Down's Syndrome ◽

Down’S Syndrome

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

Revista Paulista de Pediatria ◽

10.1590/0103-0582201432218913 ◽

2014 ◽

Vol 32 (2) ◽

pp. 159-163 ◽

Cited By ~ 17

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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Down's Syndrome with Congenital Heart Disease: Analysis of Cases Over Two Years in a Non-Invasive Laboratory of a Tertiary Hospital

Cardiovascular Journal ◽

10.3329/cardio.v2i2.6637 ◽

1970 ◽

Vol 2 (2) ◽

pp. 184-187

Author(s):

NN Fatema

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Doppler Echocardiography ◽

Congenital Heart ◽

Down's Syndrome ◽

Down’S Syndrome ◽

Tertiary Hospital ◽

Study Group ◽

Non Invasive ◽

Study Results

Background: Trisomy 21 or Down's Syndrome is the most frequent chromosomal aberration affecting live birth infants with an incidence of 1 in 660 live births. This syndrome is often associated with congenital cardiac lesions, Incidence of which is 40-60 percent. This study was conducted to see the frequency of Down’s syndrome cases and pattern of heart diseases they have in one of the busy non-invasive pediatric cardiac laboratory of the country. Methods: It was a retrospective study conducted in the non-invasive pediatric cardiac laboratory and pediatric cardiac outpatient clinic of a tertiary hospital over a period of two years (November 2007 to October 2009). The entire patients who had Down's Syndrome and had Doppler echocardiography were included in the study. Results: Out of total six thousand and fifty echocardiography, Down's Syndrome case was 205 (3.38%). Out of 205 cases, 185 cases were followed up in pediatric cardiac out patient clinic. Twenty cases had not reported in the out patient clinic. Seventeen of those patients had normal cardiac anatomy in Doppler echocardiography. Male were 43.90% and female were 56.09% amongst study group. Most of the patients are young infant (47.32%). Only 2.44% are in more than 10 years age group. Murmur was audible in 86.49% cases in study group and developmental delay was present in 100% of the cases. Doppler Echocardiography was found as most sensitive and specific investigation for detecting congenital heart disease. A-V canal defect was the commonest association (15.60%). Congenital heart disease was not detected in 8.29% cases. Surgical treatment was advised in 52.19% cases, Device closure was advised in 16.59% cases, medical management was advised in 21.46% cases. Conclusion: Down's syndrome is a very common chromosomal anomaly in our country. Incidence of this syndrome is increasing as number of working women, late marriage and elderly mother increasing. So, multidisciplinary approach for managing this disease should be adopted immediately. Keywords: Down's syndrome; Congenital heart disease. DOI: 10.3329/cardio.v2i2.6637Cardiovasc. j. 2010; 2(2) : 184-187

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Increased Perioperative Risk Following Repair of Congenital Heart Disease in Down's Syndrome

Anesthesiology ◽

10.1097/00000542-198608000-00020 ◽

1986 ◽

Vol 65 (2) ◽

pp. 221-223 ◽

Cited By ~ 18

Author(s):

JEFFREY P. MORRAY ◽

ROBIN MAC GILLIVRAY ◽

GARY DUKER

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Down's Syndrome ◽

Down’S Syndrome ◽

Perioperative Risk

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Pulmonary hypertension

ESC CardioMed ◽

10.1093/med/9780198784906.003.0181 ◽

2018 ◽

pp. 781-784

Author(s):

Shahin Moledina ◽

Bejal Pandya

Keyword(s):

Quality Of Life ◽

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Cardiac Surgery ◽

Heart Disease ◽

Congenital Heart ◽

Eisenmenger Syndrome ◽

Pulmonary Arterial ◽

Careful Assessment

Congenital heart disease is a major cause of pulmonary arterial hypertension (PAH) and this can largely be prevented by early repair. PAH in the presence of systemic-to-pulmonary communication, leads to shunt reversal and cyanosis, with multiple systemic consequences (Eisenmenger syndrome). Congenital heart disease patients with PAH are vulnerable and are at high risk from non-cardiac surgery, pregnancy, and inappropriate medical treatment (e.g. excessive venesection). Survival is reduced, but is better than in idiopathic PAH. Recommendations for surgery should be based on careful assessment by experts. Modern PAH pharmacotherapy is showing promise in improving quality of life.

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