Spectrum Of Congenital Heart Disease Research Articles

Spectrum of congenital heart diseases in an African population: A necropsy study

Background: The spectrum and incidence of congenital heart diseases amongst African population are not well defined in literature. There is the need to further elucidate the spectrum and epidemiology of congenital heart diseases amongst African. Aim: This study was conducted to highlight the spectrum and occurrence of congenital heart diseases in an African population. Methodology: All paediatric patients who were autopsied over a 98-month period were checked for occurrence of congenital heart diseases. Attempts were made to ascertain the primary disease and causes of death. We noted their bio-data, ages, sexes and causes of death. The results were analysed with SPSS version 17. The results formed the basis for discussion and recommendations. Results: There were 135 cardiac related deaths and 36 of them were in paediatric age brackets. The mean age was 4.7 + 1.08 months with a standard deviation of 6.27. M:F ratio was 1:1.1. The commonest anomaly was ventricular septal defect. There was a weak association with other congenital anomalies. The commonest cause of death was bronchopneumonia. Discussion and Recommendation: The disease pattern showed reasonable similarity with reports from other parts of the world. However, there is need for development of capacity for prenatal diagnosis of congenital heart diseases in Africa. This will widen the net of diagnosis and improve accuracy of incidence studies in Africa.

Spectrum of congenital heart disease in children admitted for cardiac surgery at Rehman Medical Institute, Peshawar, Pakistan.

Objective: To assess age, gender distribution and relative frequency of congenital heart disease (CHD) in children who underwent palliative or corrective cardiac surgery at Rehman Medical Institute, Peshawar. Methodology: This retrospective study was conducted in Department of Cardiac Surgery at Rehman Medical Institute (RMI), Peshawar from May 2008 till May 2010. One hundred and twenty three patients up to age group of 16 years with confirmed diagnosis of congenital heart disease admitted for Cardiac Surgery at Rehman Medical Institute, Peshawar were included. Results: Out of 123 patients, there were 71 males (57.7%) and 52 females (42.2%), with male to female ratio of 1.3:1. Sixty five (52.8%) of the total cases had acyanotic heart defects. Cyanotic heart defects were seen in 58 patients (47.1%). Ventricular Septal Defect (VSD) followed by Patent Ductus Arteriosus (PDA) and Atrial Septal Defect (ASD), were the commonest acyanotic heart lesions, 33.8%, 23.0% and 16.9% respectively. Tetralogy of Fallot (TOF) was the commonest cyanotic lesion. Conclusion : Majority of patients with congenital heart disease had acyanotic CHD with the commonest lesion being VSD. Tetralogy Of Fallot (TOF) was the commonest cyanotic lesion. Most of the patients were less than five years with no significant difference in sex distribution. Availability of expertise locally will lead to more patients getting surgical treatment at an earlier age thereby reducing morbidity and mortality and improving quality of life for these children.

OP35.04: Effect of changes to UK fetal cardiac screening protocols across the spectrum of congenital heart disease requiring intervention in the first year of life

OP35.04: Effect of changes to UK fetal cardiac screening protocols across the spectrum of congenital heart disease requiring intervention in the first year of life

Oral Abstract: Left-right interaction and relationships across the spectrum of congenital heart disease * Friday 9 December 2011, 11:00-12:30 * Location: Kaposvar

Oral Abstract: Left-right interaction and relationships across the spectrum of congenital heart disease * Friday 9 December 2011, 11:00-12:30 * Location: Kaposvar

A Clinical Commentary on the Article “Biomaterials Advances in Patches for Congenital Heart Defect Repair”

Congenital heart disease remains the most common birth defect affecting almost 1% of the population and if one considers congenitally bicuspid aortic valves, the number of affected individuals rises to as much as 4% by many estimates. The spectrum of congenital heart disease is broad. Patients may have very complex structural anomalies including an absence of a ventricle (single ventricle), lack of connection to a great artery (aortic or pulmonary atresia), anomalies of venous connections (systemic or pulmonary venous), valvar abnormalities (semilunar and/or atrioventricular), abnormal connections (transposition or malalignment), and vascular hypoplasia (aortic coarctation, hypoplasia, or interruption). Individuals affected by variations of these problems may present with acute circulatory compromise immediately at birth, later in infancy or on more rare occasions, later in life. The physiology at presentation relates to the structural morphology and may include cyanosis, low cardiac output (shock), arterial or venous obstruction, excessive pulmonary blood flow, and congestive heart failure. It has long been recognized that infants presenting with symptomatic congenital heart disease have an overall very poor prognosis if left untreated. On the less structurally complex end of the spectrum, patients may present with defects between cardiac chambers in isolation or in combination with other problems. Defects of the atrial or ventricular septa are most commonly observed. Again, there is very broad range of symptomatology and physiology relating to the type, size, location, and number of septal defects. For larger defects, with significant intracardiac shunting of blood, the patient’s shortand long-term prognosis may be significantly affected if the lesion is left untreated. Surgical, and more recently, catheter-based treatments for serious congenital cardiac defects represent a true success story of modern medicine. The earliest palliative surgeries, performed in the 1930s (ligation of patent ductus arteriosus and coarctation repair) and 1940s (creation of a systemicto-pulmonary artery shunt) ushered in the era of physiologic treatment for congenital heart disease. In subsequent decades, enormous progress was made in addressing more and more complex conditions including aggressive reconstructions. In the 1950s, cardiopulmonary bypass to support the systemic circulation offered the prospect of intracardiac correction (tetralogy of Fallot, atrioventricular canal, and atrial/ventricular septal defects). In the 1960s and 1970s, durable palliations were developed for single ventricle (Glenn and Fontan procedures). The 1980s saw increasingly aggressive operations for serious defects in newborns and small babies (transposition of the great arteries, truncus arteriosus, aortic interruption, and ultimately, very complex lesions including hypoplastic left heart syndrome). In the 1990s and the first decade of the new millennium, treatments have been further refined to improve operative survival, reduce morbidity, and consider long-term outlook including neurodevelopmental opportunities. Recent striking advances have included successful surgical correction of complex lesions in very small, preterm neonates, the development and implementation of catheter-based treatments for many conditions, and in utero interventions for fetuses with critical cardiac conditions. This article is a commentary on the following article: 10.1007/s12265011-9289-8.

The Contegra conduit in the right ventricular outflow tract is an independent risk factor for graft replacement☆

A large spectrum of congenital heart diseases requires valved conduits to establish an anatomical continuity between the right ventricle outflow tract (RVOT) and the pulmonary artery. The aim of the present study was to compare the incidence of graft replacement in patients receiving the Contegra conduit (bovine jugular vein graft) with that in patients receiving a homograft implanted in the RVOT. We reviewed a total of 347 conduits (Contegra 54; homografts 293) implanted in the RVOT from 1989 to 2003 in 323 patients (median age 12.7 years, range 4 days-69 years). Indications were Tetralogy of Fallot (n = 148), Ross operation (n = 89), truncus arteriosus communis (n = 47), pulmonary valve atresia (n = 30), double-outlet right ventricle (n = 15), transposition of the great arteries (n = 12), and endocarditis (n = 6). Follow-up was 99.4% complete (mean time: 5.9 years; range: 0-14.2 years). Freedom from graft replacement at 1, 5, and 10 years of follow-up in the Contegra and homograft groups were 98.1 ± 1.9%, 78.3 ± 5.8%, and 63.5 ± 7.2% and 99.6 ± 0.4%, 94.0 ± 1.6%, and 81.4 ± 3.4%, respectively (log-rank test, p < 0.001). Independent predictors of graft replacement of the whole sample population were: graft size ≤ 20 mm (hazard ratio (HR) 3.6), age ≤ 10.4 years (HR 3.0), the non-anatomical position of the graft (HR 2.9), and the use of the Contegra conduit (HR 2.5). The multivariable analysis carried out on the propensity-score-matched population confirmed three independent predictors of graft replacement: graft size ≤ 20 mm (HR 8.0), the non-anatomical position of the graft (HR 2.3), and the use of the Contegra conduit (HR 3.7). Besides size of the graft, age of the patients, and the non-anatomical position of the graft, the use of the Contegra conduit was found to be an independent risk factor for graft replacement in the RVOT. Patients receiving this conduit were more than twice as likely to undergo re-operation for graft replacement as those receiving a homograft.

Prenatal ultrasound screening of congenital heart disease in an unselected national population: a 21-year experience

ObjectiveTo determine the prevalence and spectrum of congenital heart disease (CHD) and the impact of a national prenatal ultrasound screening programme on outcome in a well-characterised population.Design and settingA comprehensive...

1381: Four-Dimensional Echocardiography in Diagnostics of Heart Diseases

To estimate opportunities of a 4D echocardiography (4DEcho) in diagnostics of anatomical, morphological and functional abnormalities in patients with different heart diseases. The 4DEcho was used on GE Vivid 7 Dimension with 3V transducer. 150 patients with congenital heart diseases, 20 patients with valvular diseases were included in this study. Spectrum of congenital heart diseases was wide: septum defects, atrioventricular channel (AVC), coarctation of aorta, pulmonary stenosis, intracardiac mass and other. The acquire heart diseases were presented defeat of mitral, tricuspid, aortic valves, prosthetic valves. We made 35 fetal 4DEcho examinations with congenital heart disease. The 4D diagnostics of septal defects has led to the most exact estimation of their localization, the form, edges. At AVC three components have been investigated: atrial, ventricular, valvular portions of the channel. 4DEcho at coarctation of aorta have allowed to define a level and character of narrowing, hypoplasia of descending aorta, LV hypertrophy. At pulmonary artery stenosis and right ventricular outflow tract obstruction the type and character of pathology has been appreciated with 4DEcho. Fetal 4D echocardiography allows to improving of visualization of cardiac structures at congenital heart diseases from 22 to 40 weeks of pregnancy. At valvular diseases the details of anatomy and morphology have been investigated. Research of prosthetic valves allows to estimating their conditions and dysfunction. 4DEcho allows us in routine practice to diagnose details of anatomy and morphology of heart structures and prosthetic valves, to improve revealing of congenital heart diseases in fetuses.

SPECTRUM OF CONGENITAL HEART DISEASES IN BASRA: AN ECHOCARDIOGRPHY STUDY

This is a retrospective study that was set out to describe the spectrum of congenital heart disease using echocardiography in two main centers in Basra over a period of 24 months from June (2006-2008). Children with the diagnosis of congenital heart disease were selected; information obtained from their records included age, gender, clinical diagnosis and chocardiography finding. Five hundred seventy patients had congenital heart disease making 40% of 1414 examined children. There were 309(54%) males and 261(46%) females (ratio 1.2:1); their ages ranged from 3 days to 19 years. Two hundred forty four (42.8%) children referred for echocardiography before the age of one year and two hundred one (35.2%) were 1-4 years of age. Ventricular septal defect was the most common congenital heart disease present in 247 patients (43.3%), of these (81.8%) were membranous in type, 41(16.6%) were muscular, and 4(1.6%) were supracristal type. The second commonest congenital heart disease diagnosed by echocardiography was tetralogy of Fallot in 72(12.6%) of examined children. Sixty eight (11.9%) had atrial septal defects, out of which 55(80.9%) were of secundum type, 7(10.3%) were primum, 5(7.4%) had sinus venosus defect and only one case with coronary sinus defect. Distribution of specific lesions and sex distribution were similar to findings from other parts of the world; However, the overall detection rate at 1 year of age was lower, with increasing availability of echocardiography facilities more cases of congenital heart defects are likely to be identified early.

Left ventricular morphology influences mortality after the Norwood operation

Background:Within the spectrum of congenital heart disease referred to as hypoplastic left heart syndrome (HLHS), there is variation in the morphology and function of the left ventricle which could influence...

Damus–Kaye–Stansel anastomosis in a patient with tricuspid atresia, transposition of the great arteries, VSD and total cavo-pulmonary connection (TCPC)

A Damus–Kaye–Stansel (DKS) anastomosis, i.e. an end-to-side anastomosis between the pulmonary artery and the aorta, has been applied in a wide spectrum of congenital heart disease including the Fontan circulation. We hereby present a 19-year-old woman with tricuspid atresia, transposition of great arteries, hypoplastic right ventricle, and a ventricular septal defect who was operated with total cavo-pulmonary connection (TCPC) and a DKS anastomosis. The Cardiovascular Magnetic Resonance (CMR) study showed that the systemic ventricular outflow tract is not obstructed with a good overall result of the previous interventions. CMR therefore, is an ideal mean for studying detailed anatomy and physiology without any need for radiation or contrast media.

OC067: Detection rate of early fetal echocardiography and spectrum of congenital heart disease

To evaluate the detection rate of early fetal echocardiography and to describe the spectrum of congenital heart diease (CDH). Retrospective analysis of 3521 singleton pregnancies between 11 + 0–13 + 6 weeks of gestation of a mixed risk population from 1997–2006 in a tertiary referral centre with complete follow-up. A sequential two-dimensional and color coded Dopler echocardiography was performed in all cases using transvaginal and transabdominal high frequency probes. 104 CHDs were diagnosed in the study population. Seventy-eight (75%) within the first trimester, another fourteen (13.5%) in the 2nd trimester and further five (4.8%) in the 3rd trimester. Seven CHDs (6.7%) were diagnosed postnatally. The in utero detection rate of CHD was 93.2% (97/104). The spectrum of CHD changed with advancing gestation and differed from the cases diagnosed postnatally. Early fetal echocardiography is feasible with high accuracy in low- and high risk pregnancies in experienced hands. However, the developmental nature of many cardiac defects must be considered, and therefore the necessity of a mid-gestation fetal echocardiography is underlined.

Heterotaxia, Congenital Heart Disease, and Primary Ciliary Dyskinesia

The human heart is strikingly asymmetrical along the left-right body axis. If one begins with the position of the heart in the left chest, continues through asymmetrical venous drainage into the atria and asymmetrical orientation of the 2 anatomically and functionally distinct ventricles, and finally proceeds through the highly asymmetrical coil of semilunar valves and great vessels, the structure and function of the human heart are precisely aligned to the left-right axis. When cardiac asymmetries either fail to develop or align incorrectly relative to each other or relative to other organs, a plethora of congenital heart disease results. This group of heart diseases is called heterotaxy syndrome and represents both a difficult clinical challenge and a fascinating window into the biology underlying one of the most fundamental embryological processes, namely, the mechanism by which an organism establishes the 3 body axes. Positioning of organs along the left-right axis can be divided into 3 broad classes: situs solitus, in which all organs are positioned normally; situs inversus, in which there is mirror image reversal of all organs; and heterotaxy, in which there is any positioning of organs along the left-right axis differing from situs solitus and situs inversus (Figure). Pure situs inversus is found in 1 of 8500 in the general population and is usually not associated with intracardiac defects. In contrast, heterotaxy has a high degree of association with intracardiac defects. It has a reported incidence of 1 of 10 000 and is associated with at least 3% of cases of congenital heart disease.1 The anatomic spectrum of organ laterality. A, Situs solitus. B, Right atrial isomerism. The liver is midline, there are 2 eparterial bronchi, the position of the stomach and cardiac apex is indeterminate, and there is asplenia. C, Left atrial isomerism. The liver is midline, there …

Congenital heart disease reminiscent of partial trisomy 2p syndrome in mice transgenic for the transcription factor Lbh

Partial trisomy 2p syndrome includes a spectrum of congenital heart disease (CHD) that is characterized by complex malformations of the outflow and inflow tracts, defects in cardiac septation, heart position, as well as abnormal ventricular development. Lbh (limb-bud and heart) is a novel, highly conserved putative transcriptional regulatory protein, which displays a unique spatiotemporal gene expression pattern during early mouse heart development. Here we show that human LBH maps to chromosome 2p23, a genomic region related to CHD in partial trisomy 2p syndrome. Remarkably, transgenic overexpression of Lbh in mice throughout the embryonic myocardium from a cardiomyocyte-specific promoter of the cardiac ankyrin repeat protein gene (Carp/Ankrd1) models CHD reported in humans with partial trisomy 2p syndrome. The malformations in Carp-Lbh transgenic mice reflect impaired pulmonary outflow tract valvulogenesis, cardiac septation, inflow tract morphogenesis, as well as abnormalities in ventricular cardiomyocyte growth. Furthermore, we demonstrate that overexpression of Lbh in cultured mammalian cells represses the synergistic activity of key cardiac transcription factors, Nkx2.5 and Tbx5, leading to reduced activation of the common target gene, Anf (Nppa). Strikingly, reduced levels of Anf expression were also observed in embryonic day 9.5 Carp-Lbh transgenic mice. Thus, repression of Nkx2.5 and Tbx5-mediated gene expression by deregulated Lbh may account in part for the cardiac anomalies observed in these mice. Our findings implicate LBH as a candidate gene for CHD associated with partial trisomy 2p syndrome and suggest an important role of Lbh in transcriptional control during normal cardiogenesis.

Decision making for mechanical cardiac assist in pediatric cardiac surgery

The practice of pediatric cardiac surgery has evolved to the point where the majority of patients operated on represent the most complex end of the spectrum of congenital heart disease. Given this, the potential role for mechanical cardiac assist will continue to expand. Although extracorporeal membrane oxygenation remains the mainstay of mechanical circulatory assist, the increased use of centrifugal ventricular assist devices is changing the approach to the treatment of acute cardiac failure. A range of newly developed implantable and paracorporeal devices is beginning to make its way into the clinical practice of pediatric cardiac surgery. This article addresses the different types of support available for mechanical cardiac assist and the clinical considerations in selecting the appropriate device.

Congenital heart disease in infants of diabetic mothers: echocardiographic study.

Our objective was to study neonates born to insulin-dependent diabetes mellitus (IDDM) mothers to detect the spectrum of congenital heart disease (CHD). Between July 2000 and June 2001, a prospective study of 100 consecutive infants of diabetic mothers (IDMs) at King Khalid University Hospital in Riyadh was undertaken. Family and maternal history, physical examination with special attention to the cardiovascular system, and echocardiography were performed. A total of 100 consecutive IDMs examined. The most common echocardiographic findings were patent ductus arteriosus (PDA; 70%), patent foramen ovale (68%), atrial septal defect (5%), small muscular ventricular septal defect (4%), mitral valve prolapse (2%), and pulmonary stenosis (1%). Hypertrophic cardiomyopathy (HCMP) was observed in 38% of cases, mainly hypertrophy of the interventricular septum. Severe forms of CHD encountered were D-transposition of great arteries, tetralogy of Fallot, and hypoplastic left heart syndrome (1% each). Isolated aortic stenosis and coarctation of aorta were not encountered in this series. Overall incidence of congenital heart disease was 15% after excluding PDA and HCMP. Maternal IDDM is a significant risk factor for CHD. Careful evaluation and early diagnosis of CHD in this high-risk group are highly indicated. There is a need for development of prenatal screening programs for CHD in our population.

Incidence and spectrum of congenital heart disease in Oman.

We analysed the incidence and spectrum of congenital heart disease (CHD) in the Sultanate of Oman from 1994 to 1996. CHD was detected in 992 of 139,707 live births (incidence 7.1/1000 live births). The common CHDs were ventricular septal defect (24.9%), atrial septal defect (14.4%) and patent ductus arteriosus (10.3%). The frequency of atrioventricular septal defects (5.9%) was higher than reported from other countries. Age at diagnosis was under 1 month in 38% and 1-12 months in 40%. Cyanotic CHD was found in 21.7% of the whole group and 35% of neonates. Although this was a hospital-based study, we believe we included almost all the infants and children with CHD in the country. The incidence and pattern of CHD in Oman were similar to those reported from developed countries in Europe and America, except for a higher frequency of atrioventricular septal defects. The high prevalence of consanguinity in the country did not affect the overall incidence of CHD.

Ultra fast track in elective congenital cardiac surgery

Background. Changes in healthcare delivery have affected the practice of congenital cardiac surgery. We recently developed a strategy of limited sternotomy, early extubation, and very early discharge, and reviewed the perioperative course of 198 pediatric patients undergoing elective cardiovascular surgical procedures, to assess the efficacy and safety of this approach.Methods. One hundred ninety-eight patients aged 0 to 18 years (median 3.2 years) underwent 201 elective cardiovascular surgical procedures over a 1-year period. All patients were admitted on the day of surgery. Patients were divided into six diagnostic groups: group 1, complex left-to-right shunts (n = 14, 7.0%); group 2, simple left-to-right shunts (n = 83, 41.3%); group 3, right-to-left shunts with pulmonary obstruction (n = 33, 16.4%); group 4, isolated, nonvalvular obstructive lesions (n = 30, 14.9%); group 5, isolated valvular anomalies (n = 20, 10.0%); and group 6, miscellaneous (n = 21, 10.4%).Results. After 201 procedures, 175 patients (87.1%) were extubated in the operating room and 188 (93.6%) within 4 hours from operation. Four patients (2.0%) were extubated more than 24 hours from completion of the procedure, and 2 (1.0%) died while on respiratory support (never weaned). Five patients (2.6%) failed early extubation (<4 hours). Early discharge was achieved for the vast majority of patients. Overall median length of stay (LOS, including day of surgery as day 1) was 2.0 days, with a median LOS of 3.0 days for those patients requiring circulatory arrest duration exceeding 20 minutes. Of 195 patients, 43 (24.6%), 121 (74.0%), and 159 (81.5%) were discharged, respectively, at < 24, < 48, < 72 hours from admission. Longest and shortest mean postoperative LOS were in group 6 (9.9 ± 14.5 days) and group 2 (1.6 = 0.7 days), respectively. Six patients (2.9%) died, and 11 (5.5%) suffered in-hospital complications. Thirty patients (15.4%) were either treated as outpatients (n = 11, 5.7%) or readmitted (n = 19, 9.7%) within 30 days from the time of surgery. Only 8 of 195 patients (4.1%) were readmitted with true surgical complications requiring invasive therapeutic procedures.Conclusions. Selected patients with a broad spectrum of congenital heart disease may enjoy same-day admission, limited sternotomy, immediate extubation, and very early discharge with excellent outcomes and acceptable morbidity.

Spectrum of congenital heart disease in Malta. An excess of lesions causing right ventricular outflow tract obstruction in a population-base study.

To quantify birth prevalence and spectrum of congenital heart disease in the Island population of Malta, and compare these rates with previous studies. All patients diagnosed as having congenital heart disease by echocardiography, cardiac catheterization, surgery or post-mortem by 1 year of age between 1990-1994 were included. There were 231 cases of live born congenital heart disease with a birth prevalence of 8.8/1000 live births. The commonest lesions were ventricular septal defect, pulmonary stenosis and tetralogy of Fallot. The rates of individual lesions were compared with two recent epidemiological studies with similar methodologies. Although the overall birth prevalence of congenital heart disease was similar in three studies, significantly higher rates of pulmonary stenosis, tetralogy of Fallot and double outlet right ventricle were found in Malta, all of which predispose to right ventricular outflow tract obstruction. In contrast, there were lower rates of lesions causing left ventricular outflow tract obstruction. A higher rate of ventricular septal defect was also found. The Maltese gene pool may contain an inherent predisposition towards lesions causing right ventricular outflow tract obstruction.

Congenital heart disease and sudden death in the young

Sudden death is a frequent mode of fatal outcome in cardiac disease and does not exclude young people. The aim of this investigation was to establish whether and to what extent sudden death in the young may be ascribable to the substrate of underlying congenital heart disease. Among 182 young people (≤ 35 years) who died of cardiac sudden death and underwent postmortem examination, 58 (32%) had congenital heart disease. Seven showed an intrapericardial rupture of aortic dissection, in the setting of Marfan syndrome in two, isolated bicuspid aortic valve in two, and bicuspid aortic valve and isthmic coarctation in three; all exhibited equally severe degeneration of the aortic wall. Sixteen cases had conduction system anomalies, mostly bypass tracts; 15 coronary artery anomalies (three ostial valve-like stenosis, five origin from the wrong aortic sinus, and seven deep intramyocardial course); 12 hypertrophic cardiomyopathy; five postoperative congenital heart disease including scar following ventriculotomy, conduction system injury, and defects left unrepaired; and three congenital aortic valve stenosis. One third of sudden deaths in the young was ascribable to structural defects present since birth. A large spectrum of congenital heart disease involves the risk of sudden death, but most structural defects are usually not considered to be life threatening. Some of these concealed defects are potentially detectable in life by clinical imaging techniques.