Common Cyanotic Congenital Heart Disease Research Articles

Pattern of Congenital Heart Disease in Children Presenting at Paediatric Cardiology Unit in Chattagram Maa Shishu-O-General Hospital, Chittagong

Background: Congenital Heart Disease (CHD) is the most common congenital problem in children. Early detection and proper management of congenital heart disease is very important to reduce mortality and morbidity. The purpose of this study was to find out the disease pattern of CHD among children in present situation in a center outside Dhaka.Methods: This study was conducted over a period from March 2016 to June 2017 prospectively and all patients with CHD was diagnosed by Color Doppler Echocardiography aging from 1st day of life to 12 years were included in the study.Results: Acyanotic CHD was most common among CHD. Atrial Septal Defect (ASD) was the commonest acyanotic congenital heart disease 100 (35.7 %) followed by Ventricular Septal Defect (VSD) 77 (27.5 %). Commonest cyanotic CHD was Tetralogy of Fallot (TOF) 9 (3.2 %). Only 25.36 % CHD were diagnosed during neonatal period whereas 54.64 % were diagnosed during the period of 29 days to completion of 12 months. Most of the patient (63.21%) were male.Conclusion: ASD was the commonest acyanotic CHD whereas TOF was the commonest cyanotic CHD. About 80% cases were diagnosed before 1 year of age. With the advancement of diagnostic facility and neonatal care, early detection of CHD is possible and treatment can be started at an earlier age.Chatt Maa Shi Hosp Med Coll J; Vol.16 (2); July 2017; Page 40-43

The effect of tadalafil on functional capacity and echocardiographic parameters in patients with repaired Tetralogy of Fallot.

BACKGROUNDTetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart diseases (CHD) in children. Various surgical procedures including palliative shunts and TOF total correction (TFTC) were done with some complications, of which, the most common is pulmonary valve regurgitation (PR). Tadalafil is a phosphodiesterase 5 inhibitor which reduces pulmonary vascular resistance, and improves right ventricular function and vascular endothelium, and may have some beneficial effects after TFTC.METHODSWe studied 18 patients with TOF and PR, with some impaired right ventricular function after TFTC. Tadalafil tablets at a dose of 1 mg per kg (maximum 40 mg) per day as a single dose was administered orally for 8 weeks. In all patients, before and after taking tadalafil, functional class assessment, electrocardiography (ECG) changes, some echocardiographic and endothelial function parameters [flow-mediated dilation (FMD) and intima-media thickness (IMT) of carotid artery], and exercise test were determined.RESULTSThe patient's mean age was 10.11 ± 4.03 years, and the mean age of operation was 2.52 ± 1.12 years. The effect of tadalafil on different echocardiographic parameters and also on tricuspid valve regurgitation (TR) and PR severity and gradient was not significant. Moreover, it had no effects on QRS duration. Tadalafil had a significant effect on improving FMD and exercise test (P = 0.01). The effect of tadalafil on echocardiographic parameters, carotid artery IMT, and ECG parameters was not significant (P > 0.05). Tadalafil was tolerated well, and the most common side effects were headache and myalgia.CONCLUSIONThis study showed that tadalafil is a safe and well-tolerated drug. It might improve exercise performance, endothelial function, and functional class, and possibly could allow patients a longer period of well-being and could possibly delay the need for pulmonary valve replacement (PVR).

PP-528 - Outcomes of Pregnancy in Women With Repaired Tetralogy of Fallot

Introduction: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. With advances in medical techniques, most women with repaired TOF are surviving in to their child bearing age. The aim of this study was to investigate the pregnancy outcome in women with repiared TOF.

Does Pharmacological Therapy Still Play a Role in Preventing Sudden Death in Surgically Treated Tetralogy of Fallot?

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, with a familial recurrence risk of 3%. Despite performing an optimal surgical repair, TOF patients may feature a poor medium and long-term survival rate: atrial re-entrant tachycardia will develop in more than 30% of patients and high-grade ventricular arrhythmias will be seen in about 10% of patients. These life-threatening arrhythmias and consequent sudden death continue to represent serious complications following TOF repair. Radiofrequency ablation and implantable cardioverter defibrillator are today the most effective therapeutic tools in these subjects, while the administration of antiarrhythmic drugs (Ib agents, beta blockers, and amiodarone), widely prescribed in the past, is now limited to few conditions. However pharmacological therapy still plays a role in the management of those patients who are resistant to the above stated invasive electrophysiological treatments.

Prevalence, profile, and pattern of congenital heart disease in Central India: A prospective, observational study

Objective: The objective of this study was to assess the prevalence and pattern of congenital heart disease (CHD) using echocardiography. Materials and Methods: Patients of 0–12-year-old attending outpatient and inpatient of pediatric department clinically diagnosed with heart disease were further evaluated with chest radiography, twelve-lead electrocardiograms, and the confirmation of the diagnosis was done using two-dimensional echocardiography. Results: Among a total of 42,423 patients availing outpatient and inpatient facilities at the pediatric department of a tertiary referral center, 655 were clinically suspected as having heart disease among which 430 patients were identified as CHD, giving a prevalence of 10.13/1000 live births. About 56.28% patients were below 1 year and male:female ratio was 1.3:1. Consanguinity was observed in 42.09% and most of them were of third degree. Breathlessness was the most common symptom and tachycardia was the most common sign. About 66.74% of patients were diagnosed with acyanotic and 33.26% with cyanotic type. The most common CHD was ventricular septal defect (VSD) (30.01%) followed by atrial septal defect (20.70%), tetralogy of fallot (TOF) (16.05%), and patent ductus arteriosus (10.23%). The most common cyanotic CHD was TOF followed by transposition of great vessels, total anomalous pulmonary venous connection, and single ventricle. Case fatality rate was 19.23% and most of the deaths in neonatal age group due to complex CHD, refractory congestive cardiac failure, sepsis, and pneumonia. Conclusion: The prevalence of CHD in this study was 10.13/1000 live births. VSD and TOF were the most common acyanotic and cyanotic CHD, respectively. Case fatality rate was 19.23% and most of the deaths occurred in neonatal age.

Brain Abscess in Undiagnosed Tetralogy of Fallot

Cardiovascular disease is a leading cause of mortality in all ages worldwide. Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD) accounting for 10%. There have been several reports of neurological complications associated with TOF. Although it is known, brain abscess (BA) is a serious complication in patients with uncorrected CHD mostly in the age of 4-7 years-old. We report a case of a 7 year old male who presented with a 3 month history of left sided body weakness and a 3 week history of a headache and fever. Patient was chronically unwell since birth where he would experience occasional episodes of exertional dyspnoea which was never investigated. Chest xray showed a globular-shaped heart. CT scan brain showed a 1,8 x 1,3 x 1,5 cm ring-enhancing lesion in the right parietal region with minimal perilesional vasogenic edema communicating with the body of the lateral ventricle, with enhancement of the ependymal lining of the ventricle; echocardiography revealed ventricular septal defect (VSD), overriding of aorta, obstruction to right ventricular outflow tract and hypertrophy of right ventricle consistent with a diagnosis of TOF. We elected to manage the patient conservatively on intravenous antibiotics, Patient improved significantly neurologically and was transferred to the cardiothoracic surgeons for further management of TOF.

Burden of congenital heart diseases in a tertiary cardiac care institute in Western India: Need for a national registry

Objective: Congenital heart disease (CHD) is very common disease, and it is the major cause of childhood mortality and morbidity. Not much of Indian data are available particularly from the western part of the country. There are needs to further explicate the spectrum and epidemiology of the CHD. Materials and Methods: This retrospective study collected data of the paediatric patients received in our institute from January 2016 to June 2017. All simple and complex congenital anomalies diagnosed on echocardiography were included in our study. Results: Of total 22,275 cases, CHDs were diagnosed in 9728 cases with a male-to-female ratio of 1:0.6. Most CHDs (62.77%) were diagnosed between 1 month and 6 years of age. Incidence of cyanotic CHD was 27.14% and acyanotic CHDs was 72.86% with a ratio of cyanotic-to-acyanotic CHD being 1:2.7. Ventricular septal defect (26.24%) was the most common acyanotic CHD found followed by 2089 patients of atrial septal defect. Tetralogy of Fallot (13.57%) was the most common cyanotic CHD found followed by double-outlet right ventricle in 276 patients (2.84%). Conclusions: Ours is the largest Indian study till date in this spectrum which yielded new data on the epidemiology, incidence, and prevalence of CHD. Majority of the patients with CHD had acyanotic CHD with most common anomaly being ventricular septal defect. Tetralogy of Fallot was the most common cyanotic defect. This study will increase awareness in the people with a family history of CHD and health-care providers and will assist in early diagnosis.

Whole exome sequencing identifies novel mutation in eight Chinese children with isolated tetralogy of Fallot.

BackgroundTetralogy of Fallot is the most common cyanotic congenital heart disease. However, its pathogenesis remains to be clarified. The purpose of this study was to identify the genetic variants in Tetralogy of Fallot by whole exome sequencing.MethodsWhole exome sequencing was performed among eight small families with Tetralogy of Fallot. Differential single nucleotide polymorphisms and small InDels were found by alignment within families and between families and then were verified by Sanger sequencing. Tetralogy of Fallot-related genes were determined by analysis using Gene Ontology /pathway, Online Mendelian Inheritance in Man, PubMed and other databases.ResultsA total of sixteen differential single nucleotide polymorphisms loci and eight differential small InDels were discovered. The sixteen differential single nucleotide polymorphisms loci were located on Chr 1, 2, 4, 5, 11, 12, 15, 22 and X. Among the sixteen single nucleotide polymorphisms loci, six has not been reported. The eight differential small InDels were located on Chr 2, 4, 9, 12, 17, 19 and X, whereas of the eight differential small InDels, two has not been reported. Analysis using Gene Ontology /pathway, Online Mendelian Inheritance in Man, PubMed and other databases revealed that PEX5, NACA, ATXN2, CELA1, PCDHB4 and CTBP1 were associated with Tetralogy of Fallot.ConclusionsOur findings identify PEX5, NACA, ATXN2, CELA1, PCDHB4 and CTBP1 mutations as underlying genetic causes of isolated tetralogy of Fallot.

TETRALOGY OF FALLOT IN ADULT;

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease inchildren but occurs rarely in adults. The extent of cyanosis depends on the balance of systemicand pulmonary vascular resistance, which depends on the severity of right ventricular outletobstruction.3 The more severe the obstruction, the more blood flows into the left side causingdesaturation and cyanosis. The survival rate of patients who receive surgical full correction isabout 86% at 32 years follow-up and 85% at 36 years follow up.

English

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease inchildren but occurs rarely in adults. The extent of cyanosis depends on the balance of systemicand pulmonary vascular resistance, which depends on the severity of right ventricular outletobstruction.3 The more severe the obstruction, the more blood flows into the left side causingdesaturation and cyanosis. The survival rate of patients who receive surgical full correction isabout 86% at 32 years follow-up and 85% at 36 years follow up.

Intracardiac Repair in Adult Tetralogy of Fallots with Destroyed Lung (Post Tubercular)

Tetralogy of Fallots’ (TOF) is the commonest cyanotic congenital heart disease. In this modern era when we are doing minimally invasive cardiac surgeries (MICS) and Robotic surgeries it is quiet uncommon to see a tetralogypresenting at the age of 42 years for intra cardiac repair (ICR). We are reporting a case of adult TOF with destroyed lung (post tubercular) who presented to us with severe breathlessness. We did intracardiac repair and successfully managed the case. Tetralogy of Fallots presenting at 42 years is relatively uncommon. TOF with destroyed lung is again a rarity. By our case report we want to give message to surgeons and cardiologist that TOF can present with tuberculosis and destroyed lung at this stage also and can be operated and successfully managed and will be benefited by surgery.

Coexistence of abnormal systolic motion of mitral valve in a consecutive group of 324 adult Tetralogy of Fallot patients assessed with echocardiography

BackgroundThe presence of mitral valve prolapse (MVP) in congenital heart disease (CHD) patients is not well described. Tetralogy of Fallot (TOF) is the most common cyanotic CHD associated with overall...

Following up adult patients with tetralogy of fallot: The role of echocardiography

Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart diseases. With surgical repair and the advances in postoperative care, contemporary mortality has dramatically improved and an increasing number of patients survive into adulthood, leading to a growing number of adult TOF. However, residual anatomic and hemodynamic abnormalities are encountered in nearly all patients, making follow-up mandatory. Furthermore, mortality starts to increase 25 years after surgery, emphasizing that, in adult TOF, closer monitoring is necessary. We review here the role of echocardiography in the follow-up of the TOF patients, emphasizing the role of multiple echocardiographic techniques. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:79-95, 2017.

Spectrum of congenital heart diseases at tertiary-care hospital in north western Rajasthan in India

Background: Heart diseases constitute an important group of pediatric illness and major cause of childhood mortality and morbidity. According to a status report on congenital heart disease (CHD) in India, 10% of the present infant mortality may be accounted for by CHD. Objective: (i) To study the spectrum of CHD in children under 5 years of age and (ii) to study the correlates of different CHDs through demographic and socioeconomic variables. Materials and Methods: This study was conducted on 390 patients under 5 years of age in the Department of Pediatrics, SP Medical College attached to PBM Associated Group of Hospitals, Bikaner, Rajasthan, India, from September 2014 to August 2015. The criteria to suspect heart disease was followed as formulated by Alexander Nadas as NADA’S criteria. The diagnosis was confirmed by echocardiography. Result: Pattern of CHD in this study was 88.9% cases of acyanotic CHD and 11.1% cases of cyanotic CHD. Ventricular septal defect (VSD) was the most common CHD present in 38.5%, followed by atrial septal defect (ASD) in 12.8%, patent ductus arteriosus (PDA) in 8.5%, ASD + VSD in 7.4%, tetralogy of fallot (TOF) in 5.1% of total cases. A total of 131 (33.6%) patients had 85%–95%, and 55 cases (14.1%) with SpO2 > 95%. Conclusion: Acyanotic CHD was the most common. VSD was the most common CHD. TOF was the most common cyanotic CHD. According to the WHO Z-score, 52.6% patients showed moderate to severe malnutrition. About 72.1% of cases showed SpO2 between 85% and 95%, which shows that a pulse oximetry SpO2

Echocardiographic findings in children with Tetralogy of Fallot

Background and Aims: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in children. The aim of this study was to identify various cardiac defects associated with TOF by transthoracic echocardiography.Methods: All children presenting to Shahid Gangalal National Heart Centre, with the diagnosis of TOF from 2014 to 2015 were included in the study. Echocardiograpic reports of all eighty eight children were reviewed.Results: The mean age of presentation was 3.5 } 3.9 years. Fifty six (64%) children were male and thirty two (36%) were female. Pulmonary artery anomaly was found in nine (10.2%) children which included hypoplastic pulmonary arteries, absent left pulmonary artery, post stenotic and aneurysmal dilatation of pulmonary artery. Pulmonary valve anomalies were seen in 13(14.8%) children which include pulmonary atresia, pin-hole opening of valve and bicuspid and absent pulmonary valve. Double outlet right ventricle, patent ductus arteriosus/major aortico-pulmonary collaterals, atrial septal defect and persistent left superior venacava were present in 12 (13.6%), 21 (23.9%), 22 (22.7%) and nine (10.2%) respectively. Right sided aortic arch was seen in 20 (22.7%).Conclusion: TOF is associated with various anatomical variations which need to be identified to avoid surgical complications. These can be diagnosed by echocardiography which has greatly reduced the need for invasive cardiac catheterization for these patients.Nepalese Heart Journal 2015;12(2):65-68

Prospective study of congenital heart disease in children.

Descriptive analysis of patients with congenital heart disease. Prospective study. Professorial Paediatric Unit, Lady Ridgeway Hospital, Colombo 8. All patients with congenital heart disease (CHD) admitted to the Professorial Paediatric Unit at Lady Ridgeway Hospital from August 1998 to August 1999 were included in this study. Data were collected using a pretested questionnaire which was filled by one of the authors. Patients were followed up to date. 102 patients, 53 (52.0%) males with congenital heart disease were studied. All patients were evaluated with chest xray, ECG and 2 D echocardiography, except for two. Based on clinical and echocardiographic findings 69 (67.6%) were found to have acyanotic and 33 (32.4%) cyanotic heart disease; 22 (21.5%) had a ventricular septal defect. Tetralogy of Fallot was diagnosed in 10 (9.8%) patients and was the commonest cyanotic CHD. 52 (51.0%) cases were detected between the ages of 2 weeks to 12 months, and 39 (38.2%) were detected routinely at the postnatal examination of the newborn. Only 12 (11.7%) patients underwent corrective surgery, 10 in Sri Lanka, and 2 in India. 19 children died during the study period, a case fatality of 18.6%. Previous studies have shown that congenital heart disease accounted for between 16% to 30% of deaths in the Professorial Paediatric Unit at LRH. This is the second commonest cause of deaths in the unit. This study has revealed a high case fatality rate among the patients awaiting corrective surgery. Unless surgical care for patients with congenital heart disease in the public sector are improved, many such children will continue to die early due to lack of facilities.

Surgery of Tetralogy of Fallot with Abnormal Coronary Arteries

The Tetralogy of Fallot is the most common cyanotic congenital heart disease. The regular form combines from four anomalies: - A ventricular septal defect Pulmonary stenosis - Overriding aorta - A right ventricular hypertrophy. Irregular forms are all other forms where another heart defect is associated, among which are the anomalies of the coronary arteries found in 5-9% of cases that require special care because of their complexity and anatomic varieties. The purpose of this study was to evaluate the particular therapeutic specificity of complete cure of Tetralogy of Fallot associated with a birth defect of the coronary arteries through a retrospective study of 9 cases collected in the cardiac surgery department at the International University Hospital Cheikh Zayed of Rabat.

Anaesthetic management for drainage of frontoparietal abscess in a patient of uncorrected Tetralogy of Fallot.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, characterised by aortic overriding, right ventricular hypertrophy, pulmonary stenosis (PS) and ventricular septal defect (VSD). When left untreated, survival to adulthood is rare.[1] Brain abscesses can occur in them because of paradoxical embolism and absence of pulmonary phagocytic clearance of pathogens. There are few case reports of uncorrected TOF surviving to adulthood and then presenting for non-cardiac surgeries.[1,2,3] We present a case of drainage of a large left fronto-parietal abscess under general anaesthesia, in a patient of uncorrected TOF.

Tetralogy of Fallot

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease beyond 1 week of age. “Classic” TOF usually presents for surgical repair during the first 3–9 months of life. Surgical repair techniques vary among surgeons and institutions. The optimal timing and approach to complete (vs. staged) surgical repair remain a topic of continued debate in the modern surgical era. A pulmonary valve sparing or reconstruction surgical approach has become popularized over the past decade or so. The purpose of this chapter will be to review the morphologic and physiologic features of “classic” TOF with an emphasis on contemporary approaches to surgical treatment.

Nutritional profiles and selected parental factors among children with congenital heart diseases in Ibadan, Nigeria

Background: Among the many adverse sequelae of congenital heart disease (CHD), malnutrition stands out as one of the major concerns. Objective: This study was undertaken to compare the nutritional status, parental age, and mid-parental heights of children with CHD and their apparently healthy control group. Material and Methods: Electrocardiography and echocardiography were used to confirm the presence or otherwise of CHD in consecutive 100 children with CHDs and 100 age, sex, and social class matched apparently healthy control group. Nutritional parameters, parental ages, and mid-parental heights were also obtained. Associations were tested using student t test and Chi square test. Level of significance was P Results: There were 54 males with CHD and the age-range of the children was 1-96 months. The commonest acyanotic CHD was ventricular septal defect (49.0%) while the commonest cyanotic CHD was Fallot's tetralogy (10%). Children with CHD compared with the control group had significantly lower mean z scores for weight for height, height for age, and weight for age. The mid upper arm circumference, triceps, and subscapular skinfold thickness were also lower than that found in the control group. Older parental age and lower mid-parental heights were found among parents of children with CHD compared with those of controls. Conclusions: Malnutrition can be quite severe in children with CHD if unattended to. Early corrective intervention is necessary to prevent short-term and long-term sequelae of malnutrition in these children. Parental malnutrition and older parental age are likely predisposing factors to CHD.