Frequency of Clinical Syndromes in children with congenital heart defects admitted at Children Hospital and Institute of Child Health Sciences

Abstract

INTRODUCTION:

Congenital heart disease is an important cause of birth defects, and is an important cause of hospital admission during infancy and childhood. Congenital heart disease is a multifaceted disorder associated with both genetic and environmental factors. The frequency of clinical syndromes in children with congenital heart defects differs depending on the region.

OBJECTIVE:

The objective of the study was to establish the number of clinical syndromes in children with congenital heart defects admitted at Children Hospital and Institute of Child Health.

MATERIAL AND METHODS:

 This Cross-sectional study was conducted during 6 months (Oct 4, 2106 till April 4, 2017) in the Development ward of the Children Hospital and The Institute of Child Health Lahore. Sample was collected by Consecutive (non-probability) sampling method. According to operational definitions, Children of CHD confirmed by echocardiography and of both gender and aged from 1 month up to 5 years were included in the study. Premature infants (birth before 28 weeks) and Children vitally unstable were excluded.

DATA COLLECTION PROCEDURE:

 Cardiac assessment was done by complete history, general physical examination, ECG, chest X-ray and echocardiography. Congenital heart disease was graded according to operational definitions. 2-dimentional echocardiography was carried out after detailed history and examination. Other than that, supportive tests e.g. CBC, serum electrolytes, renal function test, blood sugar, chest X-ray, routine urine examination, ECG, and other supportive investigations were done.

Every patient was assessed for syndromes described in operational definition on the basis of their phenotypic findings. Details of clinical syndrome and its type was recorded (according to operational definition). Data was saved by using the SPSS Version 2020. Comparisons of variables done accordingly.

RESULTS:

 The mean age of cases was 2.19 ± 1.46 years with age range of 1 month to 5 years. There were 72 (55.4%) male and 58(44.6%) female cases. According to CHD diagnosis, ASD was seen in 15(11.5%) cases, 38(29.2%) had VSD and in 77(59.2%) cases TOF was seen. Clinical syndrome was diagnosed in 50(38.5%) of the cases while rests of 80(61.5%) did not have clinical syndrome. Down’s syndrome was observed in 21(42%) of the total cases, Digeorge syndrome was seen in 7(14%) of the cases, Noonan syndrome was seen 10(20%) of the cases while Turner syndrome was seen in 12(24%) of the cases.

CONCLUSION:

Through the findings of this study we conclude that clinical syndrome was diagnosed in 50(38.5%) of the 130 cases with most common types of clinical syndrome as Down’s syndrome (42%), Noonan syndrome (20%), Turner syndrome (24%) and Digeorge syndrome was seen in 7(14%) of the cases. It was also found that Down syndrome was more common in males and digeorge syndrome was more common in females and patients with Down syndrome and Noonan syndrome had low BMI .Therefore, in cases of CHD timely clinical diagnosis is essential in order to effectively plan management and follow-up for these patients. Also, multidisciplinary approaches and care for the combined extra-cardiac anomalies may help to decrease illness and death associated with congenital heart disease.