MyMedR

This prospective, descriptive study was carried out to determine the pattern and the type of congenital heart disease seen in the Paediatric Departments in 4 government hospitals. The accessibility of surgical or transcatheter interventional treatment was also assessed. Consecutive new patients seen for suspected congenital heart disease from 1/1/95 till 31/12/95 (Group I) were registered. Records of patients seen from 1/1/95 to 31/8/95 (Group Ia) were reviewed 6 months after presentation to determine the nature of treatment received. Group II were cardiac patients who were first seen prior to the year 1995 and had undergone cardiac surgery or transcatheter interventional procedures in 1995. Of the 375 patients registered in the 4 hospitals, 250 were new patients and 125 were patients first seen prior to 1995 and had surgery or transcatheter interventional procedures in 1995. Of the 250 new patients, 83.2% had acyanotic cardiac lesions and 16.8% had cyanotic lesions. Ventricular septal defect was the commonest lesion, constituting 37.2%, followed by patent arterial duct (18.8%) and atrial septal defects (9.6%). At 6 month review, access to close-heart surgery or transcatheter interventional treatment were readily available. However, for patients with ventricular septal defects, 42.1% who required closure were still waiting for surgery 6 months after presentation. Of the 4 patients with Fallot's Tetralogy who required surgery, 2 had modified Blalock-Taussig shunt and 2 were awaiting surgery. In 1995, a total of 204 cardiac procedures were performed, there were 114 (55.9%) open heart procedures, 50 (24.5%) surgical ligation of the arterial duct, 28 (13.7%) modified Blalock-Taussig shunts, 11 (5.4%) transcatheter duct occlusion and 1 (0.5%) balloon valvuloplasty. The mean age of Fallot's Tetralogy repair was 6.4 years. No corrective surgery was performed for patients with complex cardiac lesions from the 4 hospitals in 1995. In conclusion, the pattern of congenital heart disease was similar to that seen world wide. Except for patent arterial duct, access to surgical treatment was inadequate.

Matched MeSH terms: Heart Defects, Congenital/epidemiology*; Heart Defects, Congenital/therapy*

Biologic valved conduits in infants

Corno AF

Ann Thorac Surg, 2014 May;97(5):1853.

PMID: 24792290 DOI: 10.1016/j.athoracsur.2013.12.010

Matched MeSH terms: Heart Defects, Congenital/surgery*

Pediatric cardiology in Asia

Vongprateep C

Cor Vasa, 1981;23(1):4-7.

PMID: 7238054

The problems of pediatric cardiology in Asia can be summarized as follows. In the countries like India, Indonesia, Malaysia, Pakistan and some other countries especially with government support like Thailand, the priority is primary health care. Except in Japan, cardiac care for children is not well supported financially. Training in pediatric cardiology is not readily available. Consequently small numbers of pediatric cardiologists, pediatric cardiac surgeons and active centres are available even in Japan. It is importannt to reduce the incidence of rheumatic fever and rheumatic heart disease by prevention of streptococcal infections and to increase the standard of living and environment. Transportation and referral system diagnosis, and treatment and follow-up is a special problem in neonatal care in relation to heart disease. Concentration of physicians, medical schools and cardiac centers in urban areas, old customs and poor understanding of people about the treatability and preventability of heart diseases in children complicate the improvement in the management of children with heart diseases.

Matched MeSH terms: Heart Defects, Congenital/epidemiology*

Fulltext Cardiovascular malformations in Malaysian neonates with Down's syndrome

Hoe TS, Chan KC, Boo NY

Singapore Med J, 1990 Oct;31(5):474-6.

PMID: 2148028

A prospective study was done to determine the incidence of cardiovascular malformations in neonates with Down's syndrome. 17/34 (50%) of the babies with Down's syndrome born at the Maternity Hospital, Kuala Lumpur, Malaysia had congenital heart defects. These included 7 cases of ventricular septal defect (VSD), 3 cases of patent ductus arteriosus (PDA), 2 cases of atrio-ventricular canal defect, 2 cases of ventricular septal defect with patent ductus arteriosus, 1 case of hypertrophic cardiomyopathy, 1 case of hypertrophic obstructive cardiomyopathy and 1 case of complex cyanotic heart. Only 8/17 (47%) of these babies had any clinical signs suggesting underlying cardiac defects. In view of the common occurrence of cardiac anomalies, it is recommended that echocardiographic screening should be carried out on all neonates with Down's syndrome.

Matched MeSH terms: Heart Defects, Congenital/complications*

Fulltext The leopard (multiple lentigines) syndrome: a case report

Ting HC, Ng SC

Med J Malaysia, 1983 Jun;38(2):98-101.

PMID: 6621454

A case of the leopard (multiple lentigines) syndrome is described. To our knowledge this is the first documented case of this rare but interesting syndrome to be reported in this country.

Matched MeSH terms: Heart Defects, Congenital/complications*

Contrast echocardiogram in diagnosing rare type of persistent left superior vena cava

Koay HS, Anis M, Mokhtar SA

Med J Malaysia, 2018 12;73(6):413-414.

PMID: 30647217

We report a rare case of persistent left superior vena cava (PLSVC) with direct drainage into the left atrium in a 3-yearsold boy who had been electively admitted for hypospadias repair, when he was noticed to have finger clubbing and mild hypoxia but was otherwise asymptomatic. The diagnosis of PLSVC can be made without an invasive tool as direct drainage of PLSVC into the left atrium be visualised using transthoracic echocardiography (TTE) by injecting agitated saline into the left arm.

Matched MeSH terms: Heart Defects, Congenital/diagnosis

Fulltext Anomalous right pulmonary artery from aorta, surgical approach case report and literature review

Alhawri K, Alakhfash A, Alqwaee A, HassabElnabi M, Ahmed F, Alhawri M, et al.

J Card Surg, 2021 Aug;36(8):2890-2900.

PMID: 34047395 DOI: 10.1111/jocs.15618

BACKGROUND: Anomalous origin of one pulmonary artery from the aorta is a rare congenital anomaly affecting the right pulmonary artery more than the left. These patients are at risk for the early development of significant pulmonary hypertension. Early surgical treatment has been proven safe with excellent results. The surgical approach and technique is challenging and should be decided ahead before the patient to surgery. Different techniques were described including direct reimplantation, conduit interposition, aortic ring flap.

AIM: We present a neonate with anomalous origin of the right pulmonary artery from the aorta and discuss the surgical technique and complications in the literature.

Matched MeSH terms: Heart Defects, Congenital*

Protein loosing enteropathy after Fontan procedure

Vijayaraghavan R, Thay WY

Med J Malaysia, 2018 06;73(3):175-176.

PMID: 29962503 MyJurnal

Fontan and Baudet described the procedure in 1971 and was very useful to improve the quality of life in many complex cyanotic heart diseases. It has gone through various modifications since then to improve the outcome. The mortality was reported as 2.1% and survival rate of at 5 years, 10 years, 15 years and 20 years were 91%, 80%, 73%and 69% respectively. Though it was a useful palliative procedure to improve the quality of life, it has complications which may affect the morbidity and mortality like Protein loosing enteropathy ,reduced exercise capacity thromboembolism. The patients with protein losing enteropathy present with Pleural effusion, ascites, and edema and they need periodic replacement of Albumin and frequent reviews and close follow up in the management to reduce the mortality and to improve the quality of life .These complications we come across and they need our support in the management at district level hospitals.

Matched MeSH terms: Heart Defects, Congenital/surgery

Fulltext ECMO in children post cardiac surgery-opportunity for redress

Alwi M

Anatol J Cardiol, 2017 12;18(6):431-432.

PMID: 29256879 DOI: 10.14744/AnatolJCardiol.2017.26116

Matched MeSH terms: Heart Defects, Congenital*

Fulltext Ebstein's malformation of the tricuspid valve--a report of six cases

Danaraj TJ, LaBrooy EB

Singapore Med J, 1960 Mar;1(1):16-32.

PMID: 13719549

Six cases of Ebstein's malformation are reported. The notable clinical features seen in four patients were gross enlargement of the
heart, venous congestion and marked hepatosplenomegaly. There was complete heart block in one patient and auricular fibrillation in the remaining three. The unusual pathologic features in the four cases that came to necropsy were the large pericardial effusions associated with the cardiomegaly, the absence of an atrial septal defect or patent foramen ovale, the gross enlargement of liver with cirrhotic changes and the large spleen. Histologic examination of heart muscle revealed certain unusual features which are described.

Matched MeSH terms: Heart Defects, Congenital*

Noncompaction cardiomyopathy manifesting as retinal artery occlusion

Jin-Poi T, Shatriah I, Ng SL, Zurkurnai Y, Yunus R

JAMA Ophthalmol, 2013 Feb;131(2):263-5.

PMID: 23411903 DOI: 10.1001/jamaophthalmol.2013.587

Matched MeSH terms: Heart Defects, Congenital/diagnosis*; Heart Defects, Congenital/drug therapy; Heart Defects, Congenital/physiopathology

Fulltext Risk of all-cause and CHD mortality in women versus men with type 2 diabetes: a systematic review and meta-analysis

Xu G, You D, Wong L, Duan D, Kong F, Zhang X, et al.

Eur J Endocrinol, 2019 Apr;180(4):243-255.

PMID: 30668524 DOI: 10.1530/EJE-18-0792

Objective: Previous studies have shown sex-specific differences in all-cause and CHD mortality in type 2 diabetes. We performed a systematic review and meta-analysis to provide a global picture of the estimated influence of type 2 diabetes on the risk of all-cause and CHD mortality in women vs men.

Methods: We systematically searched PubMed, EMBASE and Web of Science for studies published from their starting dates to Aug 7, 2018. The sex-specific hazard ratios (HRs) and their pooled ratio (women vs men) of all-cause and CHD mortality associated with type 2 diabetes were obtained through an inverse variance-weighted random-effects meta-analysis. Subgroup analyses were used to explore the potential sources of heterogeneity.

Results: The 35 analyzed prospective cohort studies included 2 314 292 individuals, among whom 254 038 all-cause deaths occurred. The pooled women vs men ratio of the HRs for all-cause and CHD mortality were 1.17 (95% CI: 1.12-1.23, I2 = 81.6%) and 1.97 (95% CI: 1.49-2.61, I2 = 86.4%), respectively. The pooled estimate of the HR for all-cause mortality was approximately 1.30 in articles in which the duration of follow-up was longer than 10 years and 1.10 in articles in which the duration of follow-up was less than 10 years. The pooled HRs for all-cause mortality in patients with type 2 diabetes was 2.33 (95% CI: 2.02-2.69) in women and 1.91 (95% CI: 1.72-2.12) in men, compared with their healthy counterparts.

Conclusions: The effect of diabetes on all-cause and CHD mortality is approximately 17 and 97% greater, respectively, for women than for men.

Matched MeSH terms: Heart Defects, Congenital/diagnosis*; Heart Defects, Congenital/mortality*; Heart Defects, Congenital/physiopathology

Ten-year trend in prevalence and outcome of Down syndrome with congenital heart disease in a middle-income country

Zahari N, Mat Bah MN, A Razak H, Thong MK

Eur J Pediatr, 2019 Aug;178(8):1267-1274.

PMID: 31222391 DOI: 10.1007/s00431-019-03403-x

Limited data are available on the survival of patients with Down syndrome and congenital heart disease (CHD) from middle-income countries. This retrospective cohort study was performed to determine the trends in the prevalence and survival of such patients born from January 2006 to December 2015 in Malaysia. Among 754 patients with Down syndrome, 414 (55%) had CHD, and no significant trend was observed during the 10 years. Of these 414 patients, 30% had lesions that closed spontaneously, 35% underwent surgery/intervention, 9% died before surgery/intervention, and 10% were treated with comfort care. The overall mortality rate was 23%, the median age at death was 7.6 months, and no significant changes occurred over time. The early and late post-surgery/intervention mortality rates were 0.7% and 9.0%, respectively. Most deaths were of non-cardiac causes. The overall 1-, 5-, and 10-year survival rates were 85.5%, 74.6%, and 72.9%, respectively. Patients with severe lesions, persistent pulmonary hypertension of the newborn, atrioventricular septal defect, and pulmonary hypertension had low survival at 1 year of age.Conclusion: The prevalence of CHD in patients with Down syndrome is similar between Malaysia and high-income countries. The lower survival rate is attributed to limited expertise and resources which limit timely surgery. What is Known: • The survival of patients with Down syndrome with congenital heart disease (CHD) has improved in high-income countries. However, little is known about the survival of patients with Down syndrome with CHD from middle-income countries. • In the Caucasian population, atrioventricular septal defect is the most common type of CHD associated with Down syndrome. What is New: • In middle-income countries, the prevalence of CHD is the same as in high-income countries, but with a lower survival rate. • In the Asian population, ventricular septal defect is the most common type of CHD in patients with Down syndrome.

Matched MeSH terms: Heart Defects, Congenital/diagnosis; Heart Defects, Congenital/epidemiology*; Heart Defects, Congenital/therapy

The birth prevalence, severity, and temporal trends of congenital heart disease in the middle-income country: A population-based study

Mat Bah MN, Sapian MH, Jamil MT, Abdullah N, Alias EY, Zahari N

Congenit Heart Dis, 2018 Nov;13(6):1012-1027.

PMID: 30289622 DOI: 10.1111/chd.12672

OBJECTIVES: There is limited data on congenital heart disease (CHD) from the lower- and middle-income country. We aim to study the epidemiology of CHD with the specific objective to estimate the birth prevalence, severity, and its trend over time.

DESIGN: A population-based study with data retrieved from the Pediatric Cardiology Clinical Information System, a clinical registry of acquired and congenital heart disease for children.

SETTING: State of Johor, Malaysia.

PATIENTS: All children (0-12 years of age) born in the state of Johor between January 2006 and December 2015.

INTERVENTION: None.

OUTCOME MEASURE: The birth prevalence, severity, and temporal trend over time.

RESULTS: There were 531,904 live births during the study period with 3557 new cases of CHD detected. Therefore, the birth prevalence of CHD was 6.7 per 1000 live births (LB) (95% confidence interval [CI]: 6.5-6.9). Of these, 38% were severe, 15% moderate, and 47% mild lesions. Hence, the birth prevalence of mild, moderate, and severe CHD was 3.2 (95% CI: 3.0-3.3), 0.9 (95% CI: 0.9- 1.1), and 2.6 (95% CI: 2.4-2.7) per 1000 LB, respectively. There was a significant increase in the birth prevalence of CHD, from 5.1/1000 LB in 2006 to 7.8/1000 LB in 2015 (P heart surgery program is required.

Study site: Hospital Sultanah Aminah (HSAJB), Johor

Matched MeSH terms: Heart Defects, Congenital/diagnosis; Heart Defects, Congenital/economics; Heart Defects, Congenital/epidemiology*

Fulltext Stillbirth due to unrecognized cardiac anomaly: A forensic autopsy case report

Ganesh Raj S, Norliza I, Mansharan Kaur CS

JUMMEC, 2020;23(2):22-24.

MyJurnal

Congenital heart disease (CHD) is the result of a complicated interplay between genetic and non-genetic, or “environmental,” factors acting on the foetus and one of those environmental factors is maternal hyperglycemia. Maternal diabetes has teratogenic effects on the evolution of the foetal cardiovascular system; as a consequence, cardiovascular malformations are the most common anomalies in infants of diabetic mothers with transposition of the great arteries, tricuspid atresia and truncus arteriosus being some of the common cardiac malformations encountered. Thus, it is important to perform a detailed heart examination at autopsy of perinatal deaths in order to ascertain related anomalies. We present a case of stillbirth in a woman with Type 1 Diabetes mellitus on insulin therapy who claims she was unaware about her pregnancy.

Matched MeSH terms: Heart Defects, Congenital

Management algorithm in pulmonary atresia with intact ventricular septum

Alwi M

Catheter Cardiovasc Interv, 2006 May;67(5):679-86.

PMID: 16572430 DOI: 10.1002/ccd.20672

Pulmonary atresia with intact ventricular septum (PAIVS) is a disease with remarkable morphologic variability, affecting not only the pulmonary valve but also the tricuspid valve, the RV cavity and coronary arteries. With advances in interventional techniques and congenital heart surgery, the management of PAIVS continues to evolve. This review is an attempt at providing a practical approach to the management of this disease. The basis of our approach is morphologic classification as derived from echocardiography and angiography. Group A, patients with good sized RV and membranous atresia, the primary procedure at presentation is radiofrequency (RF) valvotomy. Often it is the only procedure required in this group with the most favourable outcome. Patients with severely hypoplastic RV (Group C) are managed along the lines of hearts with single ventricle physiology. The treatment at presentation is patent ductus arteriosus (PDA) stenting with balloon atrial septostomy or conventional modified Blalock Taussig (BT) shunt. Bidirectional Glenn shunt may be done 6-12 months later followed by Fontan completion after a suitable interval. Patients in Group B, the intermediate group, are those with borderline RV size, usually with attenuated trabecular component but well developed infundibulum. The treatment at presentation is RF valvotomy and PDA stenting +/- balloon atrial septostomy. Surgical re-interventions are not uncommonly required viz. bidirectional Glenn shunt when the RV fails to grow adequately (11/2 - ventricle repair) and right ventricular outflow tract (RVOT) reconstruction for subvalvar obstruction or small pulmonary annulus. Catheter based interventions viz. repeat balloon dilatation or device closure of patent foramen ovale (PFO) may also be required in some patients.

Matched MeSH terms: Heart Defects, Congenital/diagnosis; Heart Defects, Congenital/pathology; Heart Defects, Congenital/therapy*

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