Incidence and prognosis of critical congenital heart disease in neonates at Charlotte Maxeke Johannesburg Academic Hospital

Background: A significant number of neonates with congenital heart disease present with a life-threatening illness in the neonatal period where survival depends on timely diagnosis, management and referral. Objectives: To determine the incidence and prognosis of critical congenital heart disease in neonates admitted to Charlotte Maxeke Johannesburg Academic Hospital, and to compare it with international data. Method: This was a retrospective, descriptive study of neonates with critical and non-critical congenital heart disease admitted to Charlotte Maxeke Johannesburg Academic Hospital between 01 January 2006 and 31 December 2014. Results: There were 284 neonates diagnosed with congenital heart disease during the study period - 133 with critical congenital heart disease (8.2 per 1000 neonatal admissions) and 151 with non-critical congenital heart disease (9.3 per 1000 neonatal admissions). The mortality rate for neonates with critical congenital heart disease was 61/133 (45.9%). Conclusion: Critical congenital heart disease is not commonly diagnosed in neonates, however the mortality rate is high. Increased awareness and screening programs may improve early diagnosis and allow timeous intervention.LG201

Cardiopatias Congénitas Complexas: Influência do Diagnóstico Pré-Natal

INTRODUCTION: Complex congenital heart disease is a group of severe conditions. Prenatal diagnosis has implications on morbidity and mortality for most severe conditions. The purpose of this work was to evaluate the influence of prenatal diagnosis and distance of residence and birth place to a reference center, on immediate morbidity and early mortality of complex congenital heart disease. MATERIAL AND METHODS: Retrospective study of complex congenital heart disease patients of our Hospital, born between 2007 and 2012. RESULTS: There were 126 patients born with complex congenital heart disease. In 95%, pregnancy was followed since the first trimester, with prenatal diagnosis in 42%. There was a statistically significant relation between birth place and prenatal diagnosis. Transposition of great arteries was the most frequent complex congenital heart disease (45.2%), followed by pulmonary atresia with ventricular septal defect (17.5%) and hypoplastic left ventricle (9.5%). Eighty-two patients (65.1%) had prostaglandin infusion and 38 (30.2%)were ventilated before an intervention. Surgery took place in the neonatal period in 73%. Actuarial survival rate at 30 days, 12 and 24 months was 85%, 80% and 75%, respectively. There was no statistically significant relation between prenatal diagnosis and mortality. DISCUSSION: Most patients with complex congenital heart disease did not have prenatal diagnosis. All cases with prenatal diagnosis were born in a tertiary center. Prenatal diagnosis did not influence significantly neonatal mortality, as already described in other studies with heterogeneous complex heart disease. CONCLUSION: prenatal diagnosis of complex congenital heart disease allowed an adequate referral. Most patients with complex congenital heart disease were not diagnosed prenatally. This data should be considered when planning prenatal diagnosis of congenital heart disease

Sleep in Infants with Congenital Heart Disease

OBJECTIVES: To investigate hypoxia and sleep disordered breathing in infants with congenital heart disease. METHODS: Prospective study. In-hospital full polysomnography was performed on 14 infants with congenital heart disease, age 7 ±1 months, and in 7 normal infants, age 10 ±2 months. Congenital heart disease infants were classified as acyanotic (n=7) or cyanotic (n=7). RESULTS: Nutritional status, assessed by the Gomez classification and expressed as % weight for age, was 70 ±7, 59 ±11 and 94 ±16 in the acyanotic, cyanotic congenital heart disease and control infants, respectively (p<0.001). The respiratory disturbance index (AHI, events per hour) was [median (25-75%)]: 2.5 (1.0-3.4), 2.4 (1.5-3.1) and 0.7 (0.7-0.9) in acyanotic, cyanotic CHD infants and controls, respectively (p=0.013). Almost all congenital heart disease infants (11 out of 14) and only one control infant had an AHI >1 event/hour. The minimum oxygen saturation was 79% (74-82), 73% (57-74) and 90% (90-91) in the acyanotic, cyanotic congenital heart disease infants and controls, respectively (p <0.001). The arousal index (events/hour) was similar among the three groups at 8.4 ±2.4, 10.3 ±8.7 and 6.5 ±3, respectively (p=0.451). CONCLUSIONS: Infants with congenital heart disease frequently present with sleep-disordered breathing associated with oxygen desaturations but not arousals. Therefore, sleep may represent a significant burden to infants with congenital heart disease

Heart transplantation in children with congenital heart disease

ObjectivesThe aim of this study was to describe heart transplantation in children with congenital heart disease and to compare the results with those in children undergoing transplantation for other cardiac diseases.BackgroundReports describe decreased survival after heart transplantation in children with congenital heart disease compared with those with cardiomyopathy. However, transplantation is increasingly being considered in the surgical management of children with complex congenital heart disease. Present-day results from this group require reassessment.MethodsThe diagnoses, previous operations and indications for transplantation were characterized in children with congenital heart disease. Pretransplant course, graft ischemia time, posttransplant survival and outcome (rejection frequency, infection rate, length of hospital stay) were compared with those in children undergoing transplantation for other reasons (n = 47).ResultsThirty-seven children (mean [±SD] age 9 ± 6 years) with congenital heart disease underwent transplantation; 86% had undergone one or more previous operations. Repair of extracardiac defects at transplantation was necessary in 23 patients. Causes of death after transplantation were donor failure in two patients, surgical bleeding in two, pulmonary hemorrhage in one, infection in four, rejection in three and graft atherosclerosis in one. No difference in 1- and 5-year survival rates (70% vs. 77% and 64% vs. 65%, respectively), rejection frequency or length of hospital stay was seen between children with and without congenital heart disease. Cardiopulmonary bypass and donor ischemia time were significantly longer in patients with congenital heart disease. Serious infections were more common in children with than without congenital heart disease (13 of 37 vs. 6 of 47, respectively, p = 0.01).ConclusionsDespite the more complex cardiac surgery required at implantation and longer donor ischemic time, heart transplantation can be performed in children with complex congenital heart disease with success similar to that in patients with other cardiac diseases

Heart Murmur in Neonates: How Often Is It Caused by Congenital Heart Disease

Abstract Objective: Congenital heart disease (CHD) is the most common form of cardiovascular diseases in children. This study was performed from September 2006 to August 2007 in Ardebil, Westnorthern Iran. The aim was to determine the prevalence of heart murmur in newborns and its correlation with CHD. Methods: In a 1-year cross sectional descriptive-analytic study, 2928 newborns were screened for heart murmur during routine neonatal physical examination. All babies with murmur underwent echocardiography. Findings: Murmur was detected in 91 (3.1%) neonates, of whom 47 (51.6%) had a congenital heart disease. The most common (17.6%) abnormality was ventricular septal defect. Patent ductus arteriosus was found in 10 (11%) patients. Conclusion: Remarkable high (round 50 %) rate of CDH in newborns presenting with heart murmur, urges to observe these neonates closely to establish the diagnosis of congenital heart disease and early referral to pediatric cardiologist

The Levels of Ghrelin, TNF-α, and IL-6 in Children with Cyanotic and Acyanotic Congenital Heart Disease

Background/Aim. Ghrelin has effects on nutrient intake and growth. The cause of growth retardation in congenital heart disease is multifactorial. The aim of the present study is to investigate the ghrelin in congenital heart disease and the association of ghrelin with TNF-α and IL-6. Materials and methods. We measured serum ghrelin, TNF-α, and IL-6 levels using spesific immunoassay in 68 patients (47 acyanotic, 21 cyanotic with congenital heart disease) and in 25 control subjects. Results. In comparison to controls, serum ghrelin, TNF-α levels were significantly higher in acyanotic patients and cyanotic patients with congenital heart disease (P<.0001). In acyanotic and cyanotic patients with congenital heart disease, there was a positive correlation between ghrelin and TNF-α (r=.485, P<.05 and r=.573, P<.01, resp.). Conclusion. Serum ghrelin levels is elevated in acyanotic and cyanotic patients with congenital heart disease. Increased ghrelin levels represents malnutrition and growth retardation in these patients. The relation of ghrelin with cytokines may be explained by the possible effect of chronic congestive heart failure and chronic shunt hypoxemia

The contribution of pulse oximetry to the early detection of congenital heart disease in newborns

Approximately half of all newborns with congenital heart disease are asymptomatic in the first few days of life. Early detection of ductal-dependant cardiac malformations prior to ductal closure is, however, of significant clinical importance, as the treatment outcome is related to the time of diagnosis. Pulse oximetry has been proposed for early detection of congenital heart disease. The aims of the present study were: 1) to determine the effectiveness of a pulse-oximetric screening performed on the first day of life for the detection of congenital heart disease in otherwise healthy newborns and 2) to determine if a pulse-oximetric screening combined with clinical examination is superior in the diagnosis of congenital heart disease to clinical examination alone. This is a prospective, multi-centre study. Postductal pulse oximetry was performed between six and twelve hours of age in all newborns of greater than 35 weeks gestation. If pulse-oximetry-measured arterial oxygen saturation was less than 95%, echocardiography was performed. Pulse oximetry was performed in 3,262 newborns. Twenty-four infants (0.7%) had repeated saturations of less than 95%. Of these infants, 17 had congenital heart disease and five of the remaining seven had persistent pulmonary hypertension. No infant with a ductal-dependant or cyanotic congenital heart disease exhibited saturation values greater or equal to 95%. Conclusion: postductal pulse-oximetric screening in the first few days of life is an effective means for detecting cyanotic congenital heart disease in otherwise healthy newborn

The Relationship of Left Ventricular Trabeculation to Ventricular Function and Structure Over a 9.5-Year Follow-Up The MESA Study

Left ventricular (LV) trabeculation is highly variable among individuals and is increased in some diseases (e.g., congenital heart disease or cardiomyopathies), but its significance in population-representative individuals is unknown

Prevalence of Congenital Heart Disease in Infants of Diabetic Mothers in Children Welfare Teaching Hospital

Background: Women with diabetes in pregnancy (type 1, type 2 and gestational) are at increased risk for adverse pregnancy outcomes which also include infant development of congenital heart disease and even fetal death. Adequate glycemic control before and during pregnancy is crucial to improve outcome. Aim of the study: to observe the significance of the cardiac complication seen in new born of diabetic mother and its relation to glycemic control. Patients and methods: This prospective study was conducted in the nursery care unit in children welfare teaching hospital from the period 1st of July 2014 till 30th of June 2015 where one hundred newborns of diabetic mother were collected and screened by echocardiography for congenital heart disease. Those mothers were classified according to White classification, and the babies were classified according to their maturity to (full term, pre term and post term), in reference to their mode of delivery (Spontaneous vaginal delivery or caesarian section) and to their body weight. Results: One hundred newborns where collected, 61 male and 39 female. Male to female ratio was 1.5:1; 60% were normal with no cardiac complication, 40% had congenital heart disease, hypertrophic cardiomyopathy  23%, atrial septal defect 7 %, patent ductus arteriosus 3% and ventricular septal defect 2%, 1% Transposition of great arteries, 1% Trancus arteriosus, 1% Double outlet right ventricle, 1% Tricuspid atrasia and 1% coartication of aorta, it was found that there is a significant relationship between the type of diabetes of the mother and the development of congenital heart disease in infants (p value 0.02); the degree of glycemic control according to HBA1c of the mother  and development of congenital heart disease in infants (p value 0.005); birth weight of the infants and the congenital heart disease (p value 0.023), death was reported in only 2%. Conclusions: Hypertrophic cardiomyopathy was the most common type of congenital heart disease in infants of diabetic mothers. Most of infants of diabetic mothers were full term, delivered by caesarian section and their birth weights were more than 4 kg; Congenital heart disease in infants were more common in mothers with uncontrolled diabetes during pregnancy. Pregestational diabetes mellitus mothers are more liable to deliver babies with congenital heart disease than those with gestational diabetes mellitus. Keywords: Congenital heart disease, Hypertrophic cardiomyopathy, Type 1 diabetes, Type 2 diabetes, Gestational diabetes

Congenital Heart Disease

Cardiac MRI plays a pivotal role in both anatomical and functional assessment of shunts, admixture lesions, transpositions, and the surgical correction of these lesions. This unit presents basic techniques for the evaluation of congenital heart disease. While sequence parameters described are meant to be as generic as possible, parameters are most appropriate for the Siemens 1.5 T Vision or Symphony, and may need to be altered for magnets of different field strengths and manufacturers.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/145410/1/cpmia1001.pd