The Italian Multicentric Study on Epidemiology of Congenital Heart Disease: first step of the analysis

1999 ◽  
Vol 9 (3) ◽  
pp. 291-299 ◽  
Author(s):  
Giuliano Bosi ◽  
Marco Scorrano ◽  
Giovanna Tosato ◽  
Elena Forini ◽  
Roxana Chakrokh ◽  
...  

AbstractWe present the aims, methodology and initial results from the Italian Multicentric Study for the registration and follow-up of congenital heart disease. The general aims are to measure the prevalence of congenital heart disease in different geographic areas of Italy, and to assess the survival and outcome of affected babies. During the years 1992 and 1993, eighteen centers for Pediatric Cardiology spread all over the Country enrolled 1445 new babies with congenital cardiac malformations from a population of 341, 647 surveyed livebirths. The new cases were registered using the same methodologic criterions of the EUROCAT study in order to evaluate differences and/or similarities between the studies. The prevalence varied between 1.8‰ and 8.1‰; the average being 4.6%. The large range in prevalence is presumed to be related to different customs and hierarchies in flow and referral of patients. We provide total prevalence of individual lesions, and distribution of sentinel cardiac anomalies, in the Italian study and compare them with EUROCAT. Isolated ventricular septal defect is the most common lesion (39%); followed by atrial septal defect (7.5%); pulmonary valvar stenosis (7.3%); atrioventricular septal defects (5.4%); patency of the arterial duct (3.8%); complete transposition (3.7%); tetralogy of Fallot (3.3%); aortic coarctation (2.4%); aortic valvar stenosis (2.2%); and left heart hypoplasia (1.8%). The echographic stratification of ventricular and atrial septal defects, by location and size, was in keeping with the findings of the EUROCAT study. Because of the recent widespread availability of color-Doppler tecniques, the stratification of aortic and pulmonary valvar stenosis was an innovative approach in our study. Among the complex cardiovascular anomalies, double inlet ventricle and pulmonary atresia had a proportion of about 2% each; with double outlet right ventricle, common arterial trunk, Ebstein's malformation, tricuspid atresia, interrupted aortic arch and totally anomalous pulmonary venous connection having a proportion ranging from 0.5 to 0.8%. We discuss clinical features, such as frequency of extracardiac anomalies and familial aggregation of congenital heart disease, in comparison with the EUROCAT data.

2013 ◽  
Vol 24 (5) ◽  
pp. 866-871 ◽  
Author(s):  
Dorra Abid ◽  
Anis Elloumi ◽  
Leila Abid ◽  
Souad Mallek ◽  
Hajer Aloulou ◽  
...  

AbstractAim: To investigate the previously unknown birth incidence, treatment, and mortality of children with congenital heart disease in Tunisia. Methods: We undertook a retrospective review of medical records of all patients who were born in 2010 and 2011, and were diagnosed in Sfax (Tunisia) with congenital heart defect. Results: Among 37,294 births, 255 children were detected to have congenital heart disease, yielding a birth incidence of 6.8 per 1000. The most frequently occurring conditions were ventricular septal defects (31%), ostium secundum atrial septal defects (12.9%), and pulmonary valve abnormalities (12%). Coarctation of the aorta, tetralogy of Fallot, univentricular physiology, pulmonary atresia with ventricular septal defect, and transposition of the great arteries were found in 4.3%, 6.2%, 3.4%, 2.7%, and 2.7%, respectively. During the follow-up of 1 year, 23% of the children died. About three-quarters of those deaths happened before surgery. Conclusion: The present study is in line with the general estimates in the world. It has revealed a high case of mortality among the patients awaiting corrective surgery. These children need more facilities.


2019 ◽  
Vol 3 (4) ◽  
pp. 1-4
Author(s):  
Julia Illner ◽  
Holger Reinecke ◽  
Helmut Baumgartner ◽  
Gerrit Kaleschke

Abstract Background Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical intervention is frequently high and catheter intervention may be complicated by complex anatomy and shunt calcification. Case summary We report the case of a 47-year-old man with uncorrected (palliated) pulmonary atresia and ventricular septal defect who presented with progressive cyanosis (oxygen saturation 69%) and decreasing exercise capacity. Computed tomography revealed a totally occluded modified left Blalock–Taussig (BT) shunt and a severely stenosed central shunt (Waterston–Cooley) in a patient with confluent but hypoplastic pulmonary arteries and multiple major aortic pulmonary collaterals. Due to a high operative risk, an interventional, percutaneous approach was preferred to re-do surgery. From a radial access the calcified BT shunt could be crossed with a hydrophilic guidewire. Then, a rotational thrombectomy, balloon dilatation, and bare-metal stenting at the proximal and distal anastomoses were performed. Post-interventionally, peripheral oxygen saturation increased from 69% to 82%. Clopidogrel was administered for 1 month after bare-metal stenting. At 1-year follow-up, the BT shunt was still patent on echocardiography and exercise tolerance markedly improved. Discussion This case highlights the benefit of percutaneous rotational thrombectomy followed by stenting of chronically occluded systemic-to-pulmonary artery shunts for further palliation in adult patients with complex congenital heart disease not suitable for surgical repair.


Introduction 94Ostium secundum ASD 96Ostium primum ASD 100Sinus venosus ASD 100Coronary sinus defect 102Patent foramen ovale 104Interatrial communications account for ~10% of congenital heart disease. Different types of atrial septal defect (ASD) are illustrated in Fig. 8.1.•...


1993 ◽  
Vol 3 (4) ◽  
pp. 441-444 ◽  
Author(s):  
Dianne N. Abuelo ◽  
Robert D. Corwin

SummaryA syndrome with characteristic facial and skeletal features resulting from a specific deletion of material on the short arm of chromosome 17 was first described by Smith et al in 1982.1 The common findings are brachycephaly, hypoplasia of the middle face, and short broad hands associated with mental retardation. More than 50 cases have been reported, and about one third have congenital heart disease. We report here a patient with atrial and ventricular septal defects together with pulmonary valvar stenosis.


2013 ◽  
Vol 20 (06) ◽  
pp. 898-903
Author(s):  
SHAKIL AHMAD ◽  
IMRAN SARWAR ◽  
NISAR KHAN SAJID

Objective: To evaluate the various types of congenital heart defects and to determine their frequency in children withDown’s syndrome. Study Design: Descriptive study. Place and duration of study: Department of Pediatrics, Independent UniversityHospital Faisal Abad Pakistan, from January 2010 to December 2012. Methodology: 93 children between the ages of day 1 to 12 years,diagnosed clinically as Down’s syndrome based on its characteristic phenotypic appearance, were included in the study. A detailedhistory, physical examination and evaluation of cardiovascular status (including Chest x-ray, Electrocardiogram and Echocardiography)were performed in each Down’s syndrome case. Variables of interest included age, sex, maternal age at birth and type and frequency ofcongenital heart disease. Results: Congenital heart disease was present in 48 (51.62%) children out of 93 children with Down’ssyndrome. Congenital cardiac defects in order of predominant type and their frequency included Ventricular septal defect (29, 60.4%),Atrioventricular septal defect (14, 29.1%), Atrial septal defect (2, 4.1%), Patent ductus arteriosis (2, 4.1%) and Tetralogy of Fallots (1,2%). 68 (73.2%) Down’s syndrome children (n=93) presented during their first year of life with mean age of 7±4 months. Malepredominance was observed in both with and without congenital heart disease Down’s syndrome children (male: female 1.7:1 and 2.5:1respectively). Mean maternal age at birth was 27±2 years. Conclusions: Congenital heart disease (CHD) is frequently associated withDown’s syndrome (DS). Ventricular septal defects and atrioventricular septal defects are the most common forms of CHDs in DS childrenof our region. Their earlier presentation (in infancy) and significant contribution to the morbidity and mortality of DS children warrantsearly diagnosis of DS and mandatory screening of all DS children for associated CHDs.


Author(s):  
Alireza Nezami ◽  
Ghobad Heidari ◽  
Fariba Tarhani ◽  
Masoumeh Kariminia

Aims:: Congenital heart disease is among the most common congenital anomalies in pediatrics. The aim of this study was to evaluate the prevalence of congenital heart disease in children in Khorramabad, Iran. Methods:: This is a descriptive-cross sectional study where all the children admitted to Shahid Madani Hospital who were diagnosed with congenital heart disease by echocardiography were enrolled. Patient information was collected by means of a questionnaire. Of 1600 children who underwent cardiac counseling, 9.75% presented congenital heart disease. These were most prevalent among the children of 0-28 days of the age (14.7%) and least in children aged 1 month-1year. According to this study, atrial (20.3%) and ventricular septal defect (10.5%) were the most common heart defects, respectively. Among signs and symptoms of cardiac disease, 49.1% of children had cyanosis, 89.7% with increased CT (cardiothoracic)-ratio, and 82.7% of had heart murmur. Congenital heart disease was more prevalent in male infants (58%) and 6.6% patients had heart failure and 1.4% had other congenital conditions, such as Down syndrome. Results:: According to our findings, atrial and ventricular septal defects are the most common congenital heart anomalies, respectively, in pediatric patients in Khorramabad.


Author(s):  
Rugea Mahmmed ◽  
Moftah Alhagamhmad ◽  
Naema Goobae ◽  
Abdelhamid Shaki ◽  
Mohamed Masood

Objective: To evaluate the pattern of Congenital heart disease (CHD) in Al-Mugref Teaching Hospital in the North Eastern part of Libya. Patients and Methods: This is a prospective descriptive study involved pediatric patients aged from day 1 until age of 15 years who were referred to the Cardiology department at Almugref Teaching Hospital for suspected CHD. Data were collected over a period of one year and all the recruited patients were subjected to a full cardiovascular system (CVS) examination and 2D Echocardiography (ECHO). Results and Conclusions: Out of the 719 total referred cases, 332 (46.1%) were confirmed having underlying CHD with predominant a cyanotic type 307 (92.5%). In terms of gender predominance, male to female ratio was 1.1: 1(176 vs. 156). Frequency of CHD in order frequency was as follow: Atrial septal defect (ASD) 134; 40.4%, Ventricular septal defect ( VSD) 102; 30.8%, Patent ductus arteriosus (PDA) 31; 9.3%, Pulmonary stenosis (PS)15; 4.5%, Atrioventricular (AV) canal defects 12; 3.6%, Aortic stenosis (AS)11; 3.3%, Tetraology of fallot (TOF) 9; 2.7%, Transposition of great arteries (TGA) 4; 1.2%, complex CHD (4; 1.2%), Rhabdomyoma (3;0.9%),  total anomalous pulmonary venous drainage ( 3; 0.9%), Truncus arteriosus (TA) 2; 0.6%, Pulmonary atresia (PA)1; 0.3%, Co-arctation of aorta (COA) 1; 0.3%. In terms of the outcome, majority of the patients in a cyanotic group were advised for scheduled follow-ups (240; 78.18%), and almost a quarter had their heart defects being closed spontaneously in the initial visits (53; 17.26%). Further, eleven patients have undergone a surgical closure (3.58%) and three patients died early (0.9%). In contrast, almost a third of the cyanotic group died shortly after the diagnosis was made (8; 32%), and seven patients were successfully operated (28%); whereas ten cases offered a close monitoring with frequent follow ups (40%).


2020 ◽  
Vol 27 (03) ◽  
pp. 660-666
Author(s):  
Abdul Razzaq Mughal ◽  
Zaigham Rasool Khalid ◽  
Bisma Safdar ◽  
Safia Mughal

Congenital heart disease (CHD) is the most common structural anomaly in Down syndrome children with a variable spectrum all over the world including Pakistan. Objectives: To determine the spectrum of congenital heart disease in Down syndrome at Faisalabad Institute of cardiology (FIC) Faisalabad. Study Design: Retrospective descriptive case series. Setting: Pediatric Cardiology Department of FIC, Faisalabad. Period: From January 2013 to June 2019. Material & Methods: All Consecutive patients of Down syndrome who underwent diagnostic Echocardiography at FIC were enrolled. Those having confirmed diagnosis of CHD were included in the study. Results: Out of 321Down syndrome children77.6% (n=249) had CHD and were enrolled for study. Male were 53.8% (n=134) while 46.2% were female (n=115). Majority of patients were below one year of age (57%, n=142). Acyanotic CHD was seen in 83.1 % of patients (n=207) while 16.9 % (n=42) had cyanotic CHD. Isolated cardiac defects was seen in 73.1% of patients (n=182) while 26.9 % had mixed cardiac lesions (n=67). Ventricular septal defect (VSD) was the most common (22.1%, n=55) solitary lesion followed by 14.5% cases of atrioventricular septal defect (AVSD), PDA (13.3%) and ASD (8.8%). Tetralogy of Fallot (TOF) was seen in 8.4%, AS in 1.2% while TGA, Tricuspid atresia, pulmonary valve stenosis, coarctation and Ebstein anomaly (0.8% each) were less common solitary defects. In mixed cardiac defects VSD with PDA was the most common (n=13, 5.22%) followed by VSD with ASD (n=12, 4.81%) and VSD with RVOTO (n=8, 3.21%). In AVSD cases, RVOTO was present in 2.81% (n=7), PDA with ASD was seen in 2% cases (n=5) while CcTGA, DORV, Pulmonary atresia were least common. Pulmonary Hypertension was present in 54.2% cases of left to right shunt lesions. Conclusion: Incidence of CHD in referred cases of Down syndrome is high (77.6%) at our setup. Acyanotic congenital heart defects are more common. VSD is the most common acyanotic CHD followed by AVSD while TOF is the most common cyanotic CHD.


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