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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 Acardius amorphus: magnetic resonance imaging (MRI) can be helpful in the diagnosis when ultrasound (US) is inconclusive

Azian AA, Roslani AL

Med J Malaysia, 2011 Dec;66(5):510-2.
PMID: 22390115 MyJurnal

An acardiac twin is rare and the diagnosis is commonly achieved from antenatal ultrasound (US) scans. However there have been cases where the appearances of the acardiac twin can be confusing and mimics a mass or tumour, for example, a teratoma. We experienced a case where the findings were unclear from the antenatal ultrasound scans and we had to resort to Magnetic Resonance Imaging (MRI), where we finally made the correct diagnosis based on the identification of two umbilical cords, supplying the normal fetus and the 'mass' (acardiac twin) respectively.

Matched MeSH terms: Heart Defects, Congenital/diagnosis*
Fulltext Beyond Critical Congenital Heart Disease: Newborn Screening Using Pulse Oximetry for Neonatal Sepsis and Respiratory Diseases in a Middle-Income Country

Jawin V, Ang HL, Omar A, Thong MK

PLoS One, 2015;10(9):e0137580.
PMID: 26360420 DOI: 10.1371/journal.pone.0137580

Studies on pulse oximetry screening for neonatal sepsis and respiratory disease in a middle-income country are lacking. Newborn screening for critical congenital heart disease (CCHD) using pulse oximetry is an effective and life-saving strategy in developed countries. While most studies have reported false-positive results during CCHD screening, they have not elaborated on the detected disease types. We studied the effectiveness and outcomes of pulse oximetry newborn screening for non-cardiac hypoxemic diseases such as neonatal sepsis, respiratory diseases, and CCHD in a middle-income country.

Matched MeSH terms: Heart Defects, Congenital/diagnosis*
Survival and Associated Risk Factors for Mortality Among Infants with Critical Congenital Heart Disease in a Developing Country

Mat Bah MN, Sapian MH, Jamil MT, Alias A, Zahari N

Pediatr Cardiol, 2018 Oct;39(7):1389-1396.
PMID: 29756159 DOI: 10.1007/s00246-018-1908-6

Critical congenital heart disease (CCHD) is associated with significant morbidity and mortality. However, data on survival of CCHD and the risk factors associated with its mortality are limited. This study examined CCHD survival and the risk factors for CCHD mortality. Using a retrospective cohort study of infants born with CCHD from 2006 to 2015, survival over 10 years was estimated using Kaplan-Meier analysis, and the risk factors for mortality were analyzed using multivariate Cox proportional hazards regression. A total of 491 CCHD cases were included in the study, with an overall mortality rate of 34.8% (95% confidence interval [CI] 30.6-39.2). The intervention/surgical mortality rate was 9.8% ≤ 30 days and 11.5% > 30 days after surgery, and 17% died before surgery or intervention. The median age at death was 2.7 months [first quartile: 1 month, third quartile: 7.3 months]. The CCHD survival rate was 90.4% (95% CI 89-91.8%) at 1 month, 69.3% (95% CI 67.2-71.4%) at 1 year, 63.4% (95% CI 61.1-65.7%) at 5 years, and 61.4% (95% CI 58.9-63.9%) at 10 years. Weight of

Matched MeSH terms: Heart Defects, Congenital/diagnosis
Asymptomatic Balanced Double Aortic Arch Associated With Tetralogy of Fallot

Haranal M, Abdul Latiff HB, Sivalingam S

World J Pediatr Congenit Heart Surg, 2020 01;11(1):130-132.
PMID: 31835984 DOI: 10.1177/2150135119885889

Coexistence of asymptomatic balanced double aortic arch with tetralogy of Fallot (TOF) is extremely rare and represents a surgical dilemma in decision-making due to the lack of consensus on the management of this subset of patients. We report a case of asymptomatic balanced double aortic arch coexistent with TOF in a two-year-old girl.

Matched MeSH terms: Heart Defects, Congenital/diagnosis
Large anomalous systemic arterial supply to the left lung without pulmonary sequestration: a rare cause of heart failure in a child

Wong MN, Joshi P, Sim KH

Pediatr Cardiol, 2009 Jan;30(1):85-6.
PMID: 18663510 DOI: 10.1007/s00246-008-9287-z

A 10-month-old boy was referred for tachypnea and heart murmur. An echocardiogram showed unexplained left heart dilation without evidence of an intracardiac shunt. A 64-slice computed tomographic contrast-enhanced angiography showed a large tortuous anomalous artery arising from the descending thoracic aorta and supplying the lower lobe of the left lung. The venous return into the left atrium was normal. The affected lobe had normal lung parenchyma, and its bronchial tree was connected normally with the left main bronchus. Hence, it was not a sequestrated lobe. The boy underwent surgical lobectomy of the left lower lobe and improved. Anomalous arterial supply of a lobe without sequestration of its bronchial tree is a rare pathologic entity. It also is a very rare cause of congestive heart failure in children. Computed tomographic angiography was a useful tool for evaluation of the intrathoracic anomalous vessel in this case.

Matched MeSH terms: Heart Defects, Congenital/diagnosis*
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*
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*
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
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
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
Fulltext 22q11.2 deletion syndrome in diverse populations

Kruszka P, Addissie YA, McGinn DE, Porras AR, Biggs E, Share M, et al.

Am J Med Genet A, 2017 Apr;173(4):879-888.
PMID: 28328118 DOI: 10.1002/ajmg.a.38199

22q11.2 deletion syndrome (22q11.2 DS) is the most common microdeletion syndrome and is underdiagnosed in diverse populations. This syndrome has a variable phenotype and affects multiple systems, making early recognition imperative. In this study, individuals from diverse populations with 22q11.2 DS were evaluated clinically and by facial analysis technology. Clinical information from 106 individuals and images from 101 were collected from individuals with 22q11.2 DS from 11 countries; average age was 11.7 and 47% were male. Individuals were grouped into categories of African descent (African), Asian, and Latin American. We found that the phenotype of 22q11.2 DS varied across population groups. Only two findings, congenital heart disease and learning problems, were found in greater than 50% of participants. When comparing the clinical features of 22q11.2 DS in each population, the proportion of individuals within each clinical category was statistically different except for learning problems and ear anomalies (P

Matched MeSH terms: Heart Defects, Congenital/diagnosis*