Prevalence Of Congenital Heart Disease Research Articles

Extrasystoles in adults with congenital heart disease: treatment options.

The prevalence of congenital heart disease (CHD) is estimated to be almost one in 100 newborns, with > 90% of patients with CHD surviving into adulthood due to medical and surgical advances in recent decades. The rationale for treatment of ventricular premature beats (VPBs) in the general population without underlying structural heart disease is mainly based on the presence of symptoms and/or the risk for developing VPB-induced cardiomyopathy in patients with very frequent VPBs. In CHD, the same general principles apply, but the clinical picture is often more complicated due to the presence of symptoms and/or systolic dysfunction resulting from the underlying heart disease itself. Sudden cardiac death due to ventricular arrhythmias is amajor concern in the CHD population, although its incidence is relatively low (<0.1%/year). Beta-blockers are the first-line medical treatment for CHD patients with VPBs, although no dedicated studies are available on the use of beta-blockers or anti-arrhythmic drugs in patients with CHD for this indication. Catheter ablation has evolved in recent years as an important treatment modality for cardiac arrhythmias, generally showing superior efficacy over medical treatment for most types of arrhythmias. However, recent technological advances have led to improved methods for ablation even in complex underlying anatomical substrates, with possibilities for image fusion between three-dimensional imaging modalities and electroanatomical mapping systems during the procedure. In addition to a discussion of the above, the article also presents two examples of VPB ablation in CHD patients.

Incidence of congenital anomalies in newborns born to mothers with heart disease

Background: Heart disease is seen in 1% pregnancies. Prior studies have either looked at only the prevalence of congenital heart diseases (CHD) in newborns or the congenital anomalies (CA) in babies born to those with chronic medical disorders as a whole. There is only sparse literature looking specifically at their prevalence in mothers with heart disease.Methods: We aimed to study incidence of CAs in newborns born to mothers with heart disease and to study other relevant outcomes like prematurity, low birth weight and intrauterine growth retardation which have significant associations with CAs. The present study was a descriptive study consisting of retrospective and prospective data and consisted of pregnant women with heart disease from 2006 to 2010 and their newborns.Results: 55 pregnant women with their 55 newborns were selected. Among the 55 newborns, 15% babies had congenital anomalies including CHD, 13% had only CHDs. 21% babies with CHDs were born to CHD mothers and none of the mother baby diagnosis was completely concordant. No statistically significant outcome of CA in newborns born to mothers with heart disease was found. No statistically significant associations among low birth weight, prematurity and CAs was found.Conclusions: 15% had congenital anomalies including CHD. No statistically significant outcome of CA in newborns born to mothers with heart disease was found. Since the study population comprised of a small sample size, significant derivations could not be made. Further studies are required in this direction to see if associations are significant.

The Prevalence and Type of Congenital Heart Diseases in Cytogenetic and Monogenic Chromosomal Rearrangements

In this study, it was aimed to investigate the pattern of congenital heart diseases in cytogenetic and monogenic chromosomal rearrangements. The pediatric age group patients who had been followed between January 2009 and December 2014 by the Genetic Department because of their syndromic manner were enrolled in the study. A total of 301 patients with clinical features were evaluated for cardiac diseases. Echocardiography and standard karyotype studies were performed in all patients. Statistically significant difference of congenital heart disease prevalence between numerical and structural chromosomal abnormalities was found in terms of appearance (p < 0.001). Congenital heart diseases prevalence was 62.5% in trisomy 21, 91% in trisomy 18, and 100% in trisomy 13. Fourteen percent of Turner syndrome patients and 8% of patients with other sex chromosome abnormalities had congenital heart diseases. VACTERL association and DiGeorge syndrome were accompanied by congenital heart diseases with the same rate of 73%. Congenital heart diseases prevalence was 50% among the Williams syndrome patients and 42.4% among the Noonan syndrome patients. There was dilated cardiomyopathy in a patient who was diagnosed with Alstrom syndrome. The prevalence of congenital heart diseases in derivative chromosomal anomalies was statistically significantly higher than the other chromosomal abnormalities with the rate of 62.5% (p < 0.001). Complex cardiac malformations are seen in patients with autosomal chromosomal aneuploidies rather than in patients with sex chromosomal aneuploidies and single gene mutations. CHD has been found with a higher rate in patients with derivative chromosome abnormalities compared with the other structural chromosome abnormalities like deletion, inversion, and translocation. We believe that the patients who are diagnosed with derivative chromosomal abnormalities as a result of genetic testing should be considered for cardiac malformations.

Prevalence of congenital heart disease among patients with down syndrome in Southwestern Saudi Arabia.

Background:There is a paucity of data on the prevalence and pattern of congenital heart disease (CHD) among patients with Down syndrome (DS) in Bisha, Saudi Arabia. The aim of the study was to determine the prevalence and patterns of CHD in patients with DS and explore the association of participants’ sex with CHD in DS patients.Methodology:A total of 42 patients with confirmed DS were enrolled in the study. Relevant data were obtained from the patients’ records. The spectrum of CHD was analyzed and classified accordingly. Pearson's Chi-square was used to determine the association of gender with CHD.Results:Approximately 81% of the study participants had CHD. The most common CHD was atrial septal defect (28.5%), followed by ventricular septal defect (25%), patent ductus arteriosus (16%), and atrioventricular septal defect (14.3%). No significant association was found between CHDs and sex.Conclusion:The prevalence of CHD in the study group was consistent with reports from other regions of Saudi Arabia and globally. Participants’ sex was not associated with the occurrence of CHD in DS patients in this study.

Compendium of cardiac diseases among children presenting in tertiary institutions in southern Nigeria: a rising trend

ABSTRACT The burden of children with cardiovascular disease is on a rising trend. These rising trends are enhanced by the development of new technology in the diagnosis of cardiac lesions. This article is aimed at documenting the current trends in the occurrence of cardiac disease among children attending tertiary hospitals in Sothern Nigeria. This is a descriptive study which involved four hundred and thirty-nine (439) children who had cardiac disease confirmed with echocardiography from four health institutions, namely; Niger Delta University, Bayelsa, Nigeria; University of Nigeria Teaching Hospital, Enugu, Nigeria; Triple care hospital, Enugu, Nigeria and; Blessed children hospital, Enugu, Nigeria over a -five -year period from July 2016- July 2020. The prevalence of congenital heart disease was 83.6% and only 16.4% being acquired heart disease. Many of the children with congenital cardiac defects have associated features of Down syndrome (trisomy 21) and the frequency was related to the type of defect; 6.4% (28/439). These features of Down syndrome are seen mainly in children with AV canal defect 33.3% (14/42) and least in children with TOF 2.0% (1/51). Zero-point two nine percent (1/439) had dextro-cardia and 0.2% (1/340) had VACTERAL (ano-rectal malformation) association, 0.2 % (1/439) had cleft lip, 1.8% (8/439) had dysmorphism other than down syndrome. Five 62.5% (5/8) of such dysmorphic features were associated with VSD.The majority of the children with cardiac disease were from the middle class 63.6% followed by the higher class 21.9% and the least is from the lower class 14.5%. The prevalence values of heart disease were quite higher than that documented in previous studies.The is a rising prevalence of congenital heart disease (83.6%) and acquired heart disease (16.4%) among children with cardiac disease in southern Nigeria. Extracardiac anomalies were also associated with these trends.

Pattern of Cardiac Abnormalities in Children &amp;lt; 5 Years of Age - Experience in a Tertiary Care Hospital

<i>Background</i>: Congenital heart diseases (CHDs) are the most common birth defects, responsible for nearly one-third of all congenital birth defects. The birth prevalence of CHD is reported to be 8-12/1000 live births. Early diagnosis of Congenital as well acquired heart diseases are of paramount important for timely management plan and intervention and thus reduce the mortality and morbidity of childhood heart disease patients. <i>Method</i>: This is a prospective observational study carried out during January 2020 to December’ 2020 at Dhaka Shishu Hospital, Bangladesh. The study population were 100 enrolled cardiac Children who were clinically suggestive of having cardiac disease evaluated through chest X-ray, ECG, Echocardiography and other necessary Investigation. Proper informed consent was taken under the study and purpose of the study was explained. Ethical approval was obtained from the Institutional Ethics Committee. All data were processed, analyzed, and disseminated by MS Office and SPSS version 26 as per need. <i>Results</i>: Among the 100 enrolled cardiac patient 87 were diagnosed to have congenital heart disease and remaining 13% percent presented with acquired heart disease. Out of 87 patients suffering from congenital heart disease 59 (67.81%) had acyanotic heart disease where Ventricular septal defect (VSD) is in the top of the list 27 (31.34%). Cyanotic congenital heart disease was found in 28 (32.19%) where Tetralogy of fallot (TOF) was the most common lesion 9 (10.34%). <i>Conclusion</i>: Childhood Heart diseases are responsible for a significant number of mortality and morbidity. Earlier detection and timely initiative regarding medical or surgical management is of utmost importance to avoid complications, reduce mortality and improve quality of life.

Clinical Profiles, Congenital Heart Disease, and Other Comorbidities Among Egyptian Children with Down Syndrome: A Tertiary Center Study

Abstract Introduction Down syndrome (DS) is the most common chromosomal disorder. It is accompanied by several comorbidities, which could lead to severe morbidity and mortality. Congenital heart disease (CHD) is one of the most commonly described condition. Objective This study aimed to determine clinical profiles, dysmorphic features, CHD, and DS associated comorbidities in a tertiary center (Cairo, Egypt). Patients and Methods This descriptive study included 290 patients diagnosed with DS, who presented to the Clinical Genetics clinic, Cairo University Children Hospitals, from February 2018 to December 2019. The patients' ages ranged from 2 to 4 years old. All patients were evaluated by full history, clinical examination, anthropometric measurements, and assessment of developmental milestones. Patients' diagnostic investigations including karyotype, thyroid function, and echocardiography were checked. Results The study population consisted of 290 children with DS of which 196 (67.6%) were male, 115 (40%) had CHD, the most prevalent atrial septal defect (ASD), patent ductus arteriosus (PDA), and ventricular septal defect (VSD) accounting for 10.7, 7.1, and 4.2%, respectively. Common dysmorphic features were upward slanting palpebral fissures (98.6%), hypertelorism (97.9%), and sandal gap (60.7%). Thyroid dysfunction was the second prevalent comorbidity, found in 35 patients (12.1%). Global developmental delay was reported affecting language (99%), motor (94.8%), and social (92.8%) domains. Conclusion The prevalence of CHD among children with DS was 40% with ASD, PDA, and VSD being the commonest. Thyroid dysfunction was the second most common comorbidity. The most prevalent dysmorphic features were upward slanting palpebral fissures, hypertelorism, and sandal gap. Developmental delay was very common, language being the most affected domain.

Prevalence and Spectrum of Complex Congenital Heart Disease in the Neonatal Intensive Care Unit at High Altitude in China

Background: Previous studies from high altitudes have reported significantly higher prevalence of congenital heart disease (CHD), consisting almost solely of simple CHD. Little is known about the occurrence of complex CHD. Neonates with complex CHD are likely admitted to NICU. We examined the prevalence and spectrum of complex CHD in NICU in order to depict a truer picture of CHD at high altitude. Methods: We reviewed charts of 4,214 neonates admitted to NICU in Qinghai province (average altitude 3,000 m). Echocardiography was performed in 1,943 babies when CHD was suspected based on clinical examinations. Results: CHD was diagnosed in 1,093 (56.3% of echoed babies). Mild CHD in 96.8% (1058 babies). Moderate CHD in 0.8% (9) included 1 (0.1%) large secundum atrial septal defect, 3 (0.3%) moderate pulmonary stenosis, 2 (0.2%) aortic stenosis and 3 (0.3%) partial anomalous pulmonary venous connection. Severe CHD in 2.4% (26) included 6 (0.5%) complete atrioventricular septal defect, 5 (0.5%) complete transposition of the great arteries, 5 (0.5%) hypoplastic right heart, 3 (0.3%) hypoplastic left heart, 3 (0.3%) double outlet right ventricle, 3 (0.3%) tetralogy of Fallot, 2 (0.2%) truncus arteriosus, 2 (0.2%) total anomalous pulmonary venous connection, 2 (0.2%) severe aortic stenosis, 2 (0.2%) interrupted aortic arch and 2 (0.2%) severe pulmonary stenosis and 1 (0.1%) single-ventricle abnormality. At two-years follow-up in 737 (67.4%) patients, 18 (90%) with severe CHD and 38 (5.3%) with mild and moderate CHD died, and 15 underwent cardiac surgery with 1 early death. Conclusions: At high altitude, a wide spectrum of CHD exists, with many heretofore unreported complex CHD. There is urgent need for routine echocardiography and early interventions in newborns particularly in NICU.

There is a Strong Association between Early Preeclampsia and Congenital Heart Defects: A Large Population-Based, Retrospective Study

Objective: The aim of this study was to determine the prevalence of congenital heart defects and examine their association with preeclampsia (PE). Methods: A clinical-based, retrospective study was conducted in Shenzhen between 2004 and 2017. Data were collected from Shenzhen Maternal and Child Health Hospital Medical Record Database. This study included all infants who were born at the hospital with or without heart defects and their mothers (N = 177,434 newborns). Data processing and analysis were performed by SPSS23.0 (Chicago, IL, USA). Results: 6,852 women (3.9%) were diagnosed as PE and 1,289 newborns (7.30 per 1,000) have congenital heart disease (CHD). Prevalence of CHD in newborns of women with PE is 15.8 per 1,000 significantly higher than the overall prevalence (7.30 per 1,000). CHD in newborns has strong association with PE, especially early-onset PE (adjusted OR 3.29 and 95% CI 2.15–5.03) and severe PE (adjusted OR 2.75 and 95% CI 2.13–3.56). Among those with CHD, infants of preeclamptic women had higher prevalence of tetralogy of Fallot (43.78 vs. 28.14 per 100,000), atrial septal defect (335.67 vs. 53.93 per 100,000), ventricular dysplasia (102.16 vs. 89.69 per 100,000), and ventricular septal defect (525.39 vs. 212.22 per 100,000) than pregnant women with non-PE. Conclusion: PE, especially early-onset PE and severe PE, is strongly associated with offspring CHD. Our results help advance the current understanding of the association between PE and offspring CHD. So preventing PE and reducing PE may have a beneficial effect on the offspring CHD.

Congenital Heart Disease in Dogs: A Retrospective Study of 95 Cases

The aim of the study was to estimate the prevalence of congenital heart diseases in dogs attending 2 veterinary hospitals in Brazil and to identify possible associations between these conditions and epidemiological characteristics.A retrospective study was carried out in the cardiology sections of 2 veterinary hospitals during a period of 70 months from January 2012 and October 2017.Of a total of 6710 dogs that attended the cardiology sections of the hospitals, 109 congenital heart diseases were identified in 95 patients, representing a prevalence of 1.6%. Findings consistent with previous literature included subaortic stenosis and pulmonic stenosis as the most commonly diagnosed conditions, in addition to a higher predisposition of females to patent ductus arteriosus (PDA). In contrast, the novel findings included a higher prevalence of atrial septal defect and a lower prevalence of patent ductus arteriosus. The majority of the animals included were over 1 year of age at the time of diagnosis (67%) especially in the subaortic stenosis group. Also, a predisposition of the Maltese to ventricular septal defect was observed.The information obtained in the present study contributes to research that describes epidemiological characteristics of dogs with congenital heart disease in a previously unreported location.

The screening of congenital heart disease by cardiac auscultation and 12-lead electrocardiogram among Indonesian elementary school students.

Screening for congenital heart disease (CHD) in school students is well-established in high-income countries; however, data from low-to-middle-income countries including Indonesia are limited. This study aimed to evaluate CHD screening methods by cardiac auscultation and 12-lead electrocardiogram to obtain the prevalence of CHD, confirmed by transthoracic echocardiography, among Indonesian school students. We conducted a screening programme in elementary school students in the Province of Special Region of Yogyakarta, Indonesia. The CHD screening was integrated into the annual health screening. The trained general practitioners and nurses participated in the screening. The primary screening was by cardiac auscultation and 12-lead electrocardiogram. The secondary screening was by transthoracic echocardiography performed on school students with abnormal findings in the primary screening. A total of 6116 school students were screened within a 2-year period. As many as 329 (5.38%) school students were detected with abnormalities. Of those, 278 students (84.49%) had an abnormal electrocardiogram, 45 students (13.68%) had heart murmurs, and 6 students (1.82%) had both abnormalities. The primary screening programme was successfully implemented. The secondary screening was accomplished for 260 school students, and 18 students (6.9%) had heart abnormalities with 7 (2.7%) who were confirmed with septal defects and 11 (4.2%) had valve abnormalities. The overall prevalence was 0.29% (18 out of 6116). The primary screening by cardiac auscultation and 12-lead electrocardiogram was feasible and yielded 5.38% of elementary school students who were suspected with CHD. The secondary screening resulted in 6.9% confirmed cardiac abnormalities. The cardiac abnormality prevalence was 0.29%.

Risk factors and lifelong impact of community-acquired pneumonia in congenital heart disease.

The prevalence of congenital heart disease (CHD) in adults is rising necessitating a greater understanding of acquired diseases such as community-acquired pneumonia, which remains a leading cause of age-related mortality and morbidity in the general population. We hypothesise that the CHD population, given cardiopulmonary mechanics and altered immune function, bears a uniquely high risk for pneumonia-related hospitalisations and mortality. A countrywide cohort study was performed to calculate the relative risk and cumulative incidence of pneumonia hospitalisations and resultant 30-day mortality amongst the adult CHD population, matched 1:10 with non-CHD persons by gender, age, and adjusted for comorbidities. Cox proportional hazard regression quantified the impact of CHD severity and extracardiac defects. The CHD cohort includes 17,162 adults. The majority demonstrate mild/moderate CHD complexity. The cumulative incidence of pneumonia hospitalisation was higher for adults with CHD (hazard ratio 1.90; 95% confidence interval: 1.74-2.06) than the comparison cohort. This risk was increased for those with extracardiac defects or a syndrome (hazard ratio: 4.34; 95% confidence interval: 3.39-5.54). Additionally, CHD individuals with severe/univentricular subtypes demonstrate a heightened risk compared to the non-CHD cohort (hazard ratio: 2.35; 95% confidence interval: 1.94-2.84), as well as compared to those with mild/moderate CHD (hazard ratio: 1.28; 95% confidence interval: 1.07-1.53). In addition, pneumonia hospitalisation mortality was elevated above the comparison population with a 30-day mortality rate ratio of 1.31 (95% confidence interval: 1.00-1.73). Adults with CHD are at elevated risk of pneumonia hospitalisations and pneumonia-associated mortality. This risk is further elevated in those with severe CHD and extracardiac defects.

Global prevalence of congenital heart disease in school-age children: a meta-analysis and systematic review

BackgroundCongenital heart disease (CHD) is the commonest birth defect. Studies estimating the prevalence of CHD in school-age children could therefore contribute to quantifying unmet health needs for diagnosis and treatment, particularly in lower-income countries. Data at school age are considerably sparser, and individual studies have generally been of small size. We conducted a literature-based meta-analysis to investigate global trends over a 40-year period.Methods and resultsStudies reporting on CHD prevalence in school-age children (4–18 years old) from 1970 to 2017 were identified from PubMed, EMBASE, Web of Science and Google Scholar. According to the inclusion criteria, 42 studies including 2,638,475 children, reporting the prevalence of unrepaired CHDs (both pre-school diagnoses and first-time school-age diagnoses), and nine studies including 395,571 children, specifically reporting the prevalence of CHD first diagnosed at school ages, were included. Data were combined using random-effects models. The prevalence of unrepaired CHD in school children during the entire period of study was 3.809 (95% confidence intervals 3.075–4.621)/1000. A lower proportion of male than female school children had unrepaired CHD (OR = 0.84 [95% CI 0.74–0.95]; p = 0.001). Between 1970–1974 and 1995–1999, there was no significant change in the prevalence of unrepaired CHD at school age; subsequently there was an approximately 2.5-fold increase from 1.985 (95% CI 1.074–3.173)/1000 in 1995–1999 to 4.832 (95% CI 3.425–6.480)/1000 in 2010–2014, (p = 0.009). Among all CHD conditions, atrial septal defects and ventricular septal defects chiefly accounted for this increasing trend. The summarised prevalence (1970–2017) of CHD diagnoses first made in childhood was 1.384 (0.955, 1.891)/1000; during this time there was a fall from 2.050 [1.362, 2.877]/1000 pre-1995 to 0.848 [0.626, 1.104]/1000 in 1995–2014 (p = 0.04).ConclusionsGlobally, these data show an increased prevalence of CHD (mainly mild CHD conditions) recognised at birth/infancy or early childhood, but remaining unrepaired at school-age. In parallel there has been a decrease of first-time CHD diagnoses in school-age children. These together imply a favourable shift of CHD recognition time to earlier in the life course. Despite this, substantial inequalities between higher and lower income countries remain. Increased healthcare resources for people born with CHD, particularly in poorer countries, are required.

Congenital heart defects in patients with isolated microtia: evaluation using colour Doppler echocardiographic image.

The objective of this study was to delineate the characteristics and incidence of congenital heart disease (CHD) in patients with isolated microtia and to determine whether the prevalence of CHD among patients with isolated microtia increases with the severity of microtia. A total of 804 consecutive patients had a pre-operative colour Doppler echocardiographic examination. A retrospective study was performed with the clinical and imaging data from November, 2017 to January, 2019. The χ2 test was performed to analyse the interaction between isolated microtia and CHD. With the colour Doppler echocardiographic examination's data from 804 consecutive isolated microtia patients, we found CHD, including atrial septal defect, ventricular septal defect, tetralogy of Fallot, patent ductus arteriosus, and others, occurred in 52 of 804 patients (6.5%). Atrial septal defect prevalence in patients with isolated microtia was significantly higher than ventricular septal defect (24/804 versus 11/804, p < 0.05) and patent ductus arteriosus (24/804 versus 2/804, p < 0.001). Ventricular septal defect prevalence in patients with isolated microtia was significantly higher than patent ductus arteriosus (11/804 versus 2/804, p < 0.05). All four types of microtia (concha-type microtia, small concha-type microtia, lobule-type microtia, and anotia) had similar incidences of CHD with no difference in the incidences among these types (p > 0.05 respectively). Furthermore, there was no significant difference in the incidence of the atrial septal defect among the four subtypes (p > 0.05 respectively). Similarly, ventricular septal defect and patent ductus arteriosus also showed no differences (p > 0.05 respectively). The overall incidences of CHD and three most common CHD subtypes (atrial septal defect, ventricular septal defect, and patent ductus arteriosus) in patients with isolated microtia are higher than general population. The prevalence of CHD among patients with isolated microtia does not increase with the severity of microtia. According to our experience in this study, we suggest colour Doppler echocardiographic imaging should be performed for isolated microtia patients soon after birth if possible. Furthermore, for the plastic surgeon and anaesthesiologist, it is important to take pre-operative colour Doppler echocardiographic images which can help evaluate heart function to ensure the safety of the peri-operative period. Future studies when investigating CHDs associated with isolated microtia could focus on genetic and molecular mechanisms.

Abstract 13291: Impact of VAD Support Duration on Pediatric Heart Transplant Outcomes

Introduction: There is wide variability in the timing of heart transplant (HT) listing and consideration of organ offers after ventricular assist device (VAD) implant in children. While some centers wait weeks or months before proceeding with HT, others accept donor hearts within days of VAD surgery. We sought to determine if HT within 30 days of VAD implant impacts post-HT outcomes when compared to HT after ≥30 days of VAD support. Methods: Patients on VAD support prior to HT were extracted from the Pediatric Heart Transplant Study database. The primary endpoints were post-HT length of hospital stay (LOS) and one-year survival. Confounding was addressed by propensity score weighting using inverse probability of treatment. Propensity scores were calculated based on age at VAD implant, blood type, primary cardiac diagnosis, decade, VAD type, and allosensitization status. Group balance was verified with standardized differences. Results: A total of 1138 children underwent VAD implant prior to HT between 2000-2019 (Table 1). Most underwent HT ≥30 days after VAD (71%). The majority (70%) had an LVAD alone. Infants accounted for 22% of both groups, while those 1-11 years were 46% of the ≥30-day group and patients ≥12 years made up 42% of &lt;30-day group (p=0.001). There was no difference in the prevalence of congenital heart disease vs. cardiomyopathy (p=0.7) or high allosensitization status (p=0.8) between groups. Post-HT LOS was similar between groups (23 vs. 21 days in &lt;30 vs. ≥30 days, p=0.07). One-year survival was 90.5% in the &lt;30-day group vs. 94.5% in ≥30 days (adjusted mortality HR 1.81, 95% CI 1.15-2.86, p=0.01). Conclusions: In this large study of children undergoing HT after VAD, we found lower one-year survival in patients on VAD support for &lt;30 days prior to HT compared to those on VAD for ≥30 days before HT. These findings suggest that a strategy of delayed HT listing following VAD implant may favorably impact post-HT survival in the pediatric population.

Advanced heart failure and transplant in congenital heart disease

### Learning objectives Due to advances in medical and surgical palliations, >90% of children born with congenital heart disease (CHD) are expected to survive into adulthood.1 2 Recent studies estimate...

GENETIC PERSPECTIVE OF THE CONGENITAL HEART DISEASE

GENETIC PERSPECTIVE OF THE CONGENITAL HEART DISEASE

Clinical spectrum and prevalence of congenital heart disease in children in Botswana.

Reliable data on congenital heart disease (CHD) from diverse settings is important both for planning health systems in each country and to elucidate possible aetiologies of CHD in different settings. There is a lack of data on the clinical spectrum and prevalence of CHD in Botswana. The aim of this study was to describe the clinical spectrum and prevalence of CHD in Botswana. This was a retrospective, descriptive, cross-sectional study of all children from birth to 15 years who had had an echocardiogram performed as an in- or out-patient at Princess Marina Hospital (PMH) between 1 January 2010 and 31 December 2012. Of 377 enrollees, 140 (40%) had normal echocardiographs, 170 (45%) had CHD, and 57 (15%) had an acquired lesion. In the CHD patients, median age was 0.9 years (Q1: 0.2, Q3: 4.1) and 85 (50%) were male. Ventricular septal defect (VSD) (29%), patent ductus arteriosus (18%), atrio-ventricular septal defect (AVSD) (10%) and tetralogy of Fallot (TOF) (6%) were the predominant pathologies. VSD was the most common acyanotic lesion and TOF the most common cyanotic lesion. The estimated prevalence of CHD was between 2.8 and 4.95 per 1 000 live births. The clinical spectrum of CHD in Botswana is similar to that observed in other African countries and in the Western world, with VSD the most common acyanotic lesion and TOF the most common cyanotic lesion. The prevalence of CHD was 2.8-4.95 per 1 000 live births, in keeping with other settings. This is the first study to describe CHD in Botswana, and it aimed to stimulate subsequent studies in this field.

The association between congenital heart disease and small for gestational age with regard to the prevalence and outcomes.

To evaluate the association between small for gestational age (SGA) and the prevalence of congenital heart disease (CHD) and the association of the SGA status with the outcomes among infants with CHD. Echocardiography was performed within the first 5days of life in 5664 consecutive infants. Infants were classified into four groups according to the presence or absence of SGA and CHD. All CHD infants were followed up until either spontaneous resolution of all cardiac lesions, invasive intervention or death. All newborns without CHD were followed for mortality until the final follow-up date. A total of 303 infants were diagnosed with CHD, while 610 were diagnosed with SGA. Among the CHD infants, 56 were SGA, and 247 were not. A multivariable logistic regression analysis showed that the adjusted odds ratio of SGA (9.71, P<.001) was significantly higher than that of other parameters concerning predictors of invasive intervention or death. The mortality rate in the presence of both SGA and CHD (hazard ratio: 33.6, P<.001) was markedly higher than in the absence of both. SGA was a significant predictor of invasive intervention for CHD. The combination of CHD and SGA carried a high risk of death beyond that of either alone.

Prevalence and Outcomes of Congenital Heart Disease in Very Low Birth Weight Preterm Infants: An Observational Study From the Brazilian Neonatal Network Database.

To evaluate the prevalence of congenital heart disease and their outcomes in a Brazilian cohort of very low birth weight preterm infants. Post hoc analysis of data from the Brazilian Neonatal Network database, complemented by retrospective data from medical charts and a cross-sectional survey. Twenty public tertiary-care university hospitals. A total of 13,955 newborns weighing from 401 to 1,499 g and between 22 and 36 weeks of gestational age, born from 2010 to 2017. None. The prevalence of congenital heart disease was 2.45% (95% CI, 2.20-2.72%). In a multivariate regression analysis, risk factors associated with congenital heart disease were maternal diabetes (relative risk, 1.55; 95% CI, 1.11-2.20) and maternal age above 35 years (relative risk, 2.09; 95% CI, 1.73-2.51), whereas the protection factors were maternal hypertension (relative risk, 0.54; 95% CI, 0.43-0.69), congenital infection (relative risk, 0.45; 95% CI, 0.21-0.94), and multiple gestation (relative risk, 0.73; 95% CI, 0.55-0.97). The pooled standardized mortality ratio in patients with congenital heart disease was 2.48 (95% CI, 2.22-2.80), which was significantly higher than in patients without congenital heart disease (2.08; 95% CI, 2.03-2.13). However, in multiple log-binomial regression analyses, only the presence of major congenital anomaly, gestational age (< 29 wk; relative risk, 2.32; 95% CI, 2.13-2.52), and Score for Neonatal Acute Physiology and Perinatal Extension II (> 20; relative risk, 3.76; 95% CI, 3.41-4.14) were independently associated with death, whereas the effect of congenital heart disease was spotted only when a conditional inference tree approach was used. The overall prevalence of congenital heart disease in this cohort of very low birth weight infants was higher and with higher mortality than in the general population of live births. The occurrence of a major congenital anomaly, gestational age (< 29 wk), and Score for Neonatal Acute Physiology and Perinatal Extension II (> 20) were significantly and independently associated with death, whereas the association of congenital heart disease and death was only evident when a major congenital anomaly was present.