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1.
Ann Pediatr Cardiol ; 16(2): 94-101, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37767174

RESUMO

Background: We present a large Indian series of Multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (COVID-19) infection. The aim of the study is to present the incidence and pattern of cardiac involvement in children with MIS-C and their short-term follow-up. Methods and Results: Consecutive 144 children younger than 18 years of age diagnosed with MIS-C referred for cardiac evaluation between June 1 and November 30, 2021, were included and were followed up till February 2022. In addition to the demographics, details of COVID-19 infection, and biomarkers, their cardiovascular assessment (echocardiogram and electrocardiogram) was documented at baseline and on follow-up. The median age of children with MIS-C was 60 (24-104) months. Abnormal cardiac imaging was noted in 59% of children. Ventricular dysfunction was noted in 13.9% and coronary abnormalities were noted in 25.7% of children. The median duration when the first cardiac abnormality was reported was 7 (5-10) days. The distribution of age categories between children with and without cardiac abnormality was comparable. Children with cardiac abnormalities were followed up for a median duration of 47 (30-58) days. Complete resolution was documented in 92% of children after a median duration of 20 (9-38) days. There were no readmissions or deaths during follow-up. Conclusion: Cardiac involvement in children with MIS-C is frequent with coronary abnormalities and ventricular dysfunction being the most common manifestations. Most children exhibit complete clinical and myocardial recovery with appropriate anti-inflammatory therapy. Studies on long-term outcome of these children are needed.

3.
Ann Pediatr Cardiol ; 15(5-6): 467-488, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-37152503

RESUMO

Justification: In recent years, there has been increasing recognition of children with heart disease in our country. These children belong to different age groups and have untreated, partially treated, or completely treated heart disease. The role of physical activity for optimal physical, emotional, and psychosocial well-being for children is well understood. There is a challenge for the parents and the medical professionals to take a decision regarding the type of physical activity safe for the child as heart disease may affect the hemodynamic demands. Most of the existing international guidelines focus on competitive sports in operated heart disease children. This may be of limited use when we have a mixed population of children with heart disease, different types of sports in our country and where a larger subset is looking for recommendations to leisure time activities. Process: The Pediatric Cardiac Society of India decided to formulate recommendations for physical activity in children with heart diseases. A committee of experts, who were well-versed with the subject of physical activity in children with heart disease, volunteered to take up the task of writing the guidelines. The recommendations emerged following deliberations of the committee members, on the virtual platform as well as mails. The final version of manuscript was approved by all committee members and all members are co-authors of this manuscript. The different types of physical activities were defined including leisure sports and competitive sports. The exercise was classified based on the mechanical action of muscles involved into dynamic and static components. Each type of exercise was then classified based on the intensity into low, medium, and high. Recommendations for the type of physical activity for individual heart lesions were decided based on the rationale available. Objectives: The recommendations here are made with an intention to provide general guidelines for physical activity in children with operated and unoperated heart diseases, not excluding a need for individualizing a plan, serial assessment, and comprehensive checkup in special situations. Recommendations: We hope the recommendations mentioned below would provide basic clarity in planning physical activity in children with heart disease. This is with the hope to encourage physically active life, at the same time ensuring a safety net.

4.
Ann Pediatr Cardiol ; 14(2): 139-145, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34103851

RESUMO

INTRODUCTION: Congenital unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly with the complete absence of intrapericardial segment of one of the branch pulmonary arteries. Sixty percent are associated with other congenital heart defects (CHD) that often need correction. AIM: To analyze the data of patients with UAPA and ipsilateral non-unifocalizable major aortopulmonary collateral arteries (MAPCAs) associated with other CHD to identify the commonly associated CHD, their management strategies and outcomes. MATERIALS AND METHODS: Retrospective data of patients admitted for congenital UAPA with other CHD was compiled from hospital records from 2002 to 2015. The associated CHD were categorized as group I with the decreased pulmonary flow and group II with increased pulmonary flow to the unaffected contralateral pulmonary artery. The determinants of their management were analyzed. RESULTS: Sixty-five patients of UAPA and ipsilateral non-unifocalizable MAPCAs associated with other CHD were identified. Group I had 41 patients and Group II had 24. The most common CHD associated with UAPA was tetralogy of Fallot (TOF) in 31 patients (47.7%). Fifty-three patients underwent surgery, 48 (73.8%) underwent single lung corrective surgery, 5 (7.6%) palliative surgery and 12 (18.4) received no surgery. Four operated patients died in the immediate postoperative period. The lowest Mc Goon ratio and Nakata index to undergo corrective surgery were 1.0 and 87.4 mm2/m2. A follow-up of 21 patients was done, among which 11 patients who underwent single-stage corrective surgery, all are in NYHA class II and saturating above 95%. CONCLUSIONS: Congenital UAPA is a rare anomaly and associated with a variety of CHDs, TOF being the most common. Single lung corrective surgery in patients with ipsilateral non-unifocalizable MAPCAs has good immediate and long term survival.

5.
Indian Pediatr ; 57(2): 143-157, 2020 02 15.
Artigo em Inglês | MEDLINE | ID: mdl-32060242

RESUMO

JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.


Assuntos
Cardiopatias Congênitas/terapia , Procedimentos Cirúrgicos Cardíacos , Fármacos Cardiovasculares/administração & dosagem , Fármacos Cardiovasculares/uso terapêutico , Criança , Pré-Escolar , Consenso , Humanos , Lactente , Tempo para o Tratamento
6.
Ann Pediatr Cardiol ; 12(3): 254-286, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31516283

RESUMO

A number of guidelines are available for the management of congenital heart diseases (CHD) from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, and others), obstructive lesions (pulmonary stenosis, aortic stenosis, and coarctation of aorta), and cyanotic CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children.

7.
Indian Heart J ; 71(3): 207-223, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31543193

RESUMO

INTRODUCTION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences. OBJECTIVES: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Países em Desenvolvimento , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/tratamento farmacológico , Humanos , Índia , Recém-Nascido , Complicações Pós-Operatórias/prevenção & controle , Fatores de Tempo
8.
Ethiop Med J ; 54(4): 237-40, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29115670

RESUMO

Myxomas occupying multiple chambers of the heart are extremely rare, being more common in familial cases. Recurrence of these tumors after surgical excision is also a rare condition, observed in about 3% of patients in sporadic cases. Systemic embolization especially to the brain is one of the common presentations of cardiac myxomas apart from intracardiac obstruction and systemic symptoms. We report an adolescent girl presenting with recurrent myxoma arising from multiple cardiac chambers with systemic embolization. As a diagnostic and preventive measure of recurrence and chronic systemic embolization, we recommend a regular follow up of such patients with echocardiographic evaluation following surgical resection.


Assuntos
Neoplasias Cardíacas/patologia , Mixoma/patologia , Recidiva Local de Neoplasia/patologia , Criança , Feminino , Neoplasias Cardíacas/cirurgia , Humanos , Mixoma/cirurgia
9.
Ann Pediatr Cardiol ; 8(1): 10-3, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25684881

RESUMO

BACKGROUND: Training of postgraduate students has traditionally been done in person in a hospital setting with hands-on training with each faculty member imparting knowledge to 2 to 4 students per year. Supplementing their practical education with online instruction could make a significant difference in standardizing pediatric cardiology education in India. OBJECTIVE: To present the rationale, methods and survey results of a live e-Teaching methodology implemented for Pediatric cardiology trainees in association with the National Board of Examinations, India. MATERIALS AND METHODS: Between March 2010 and March 2014, 310 e-classes were conducted in the Pediatric cardiac sciences by 24 e-teachers. Content of the e-Learning program was based on a 2-year pediatric cardiology curriculum and included twice-weekly live online video training sessions, a library of recorded sessions and online test quizzes for the students. A total of 231 students accessed the program at various times over the 4-year period. RESULTS: In our study, requests for access to the e-lectures increased from 10/year the first year to 100/year by the fourth year with feedback surveys conveying a high satisfaction level from the students and a high need for this knowledge. The advantages of virtual live e-Learning included the fact that one teacher can teach multiple students in multiple geographic locations at the same time, obviating the issue of quality teacher shortage and the same content can be disseminated to all students undergoing specialist training so there is a national consensus on diagnostic and management approach among all trainees. Additionally, the e-classes can be recorded and replayed so they can be viewed repeatedly by the same group or new trainees. CONCLUSION: This is the first sustained use of e-Teaching in a medical super-specialty in India. We believe that e-Teaching is an innovative solution that can be applied, not just to Pediatric Cardiology as we have done, but to all branches of specialist and super-specialist medical training in India and globally.

10.
Ann Pediatr Cardiol ; 8(1): 20-4, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25684883

RESUMO

AIM: The present study was undertaken to assess the accuracy of pediatric echocardiograms done at non-tertiary centers and to evaluate the relationship of inaccurate interpretations with age, echocardiogram performer and complexity of congenital heart disease (CHD). MATERIALS AND METHODS: The echocardiogram reports of 182 consecutive children with CHD (5 days-16 years) who were evaluated at a non-tertiary center and subsequently referred to our center were reviewed. Age of the child at echocardiogram, echocardiogram performer and complexity of CHD were noted. These reports were compared with echocardiogram done at our center. Discrepancies were noted and categorized. To assess our own error rate, we compared our echocardiogram reports with the findings obtained during surgery (n = 172), CT scan (n = 9) or cardiac catheterization reports (n = 1). RESULTS: Most of the children at the non-tertiary center (92%) underwent echocardiogram by personnel other than a pediatric cardiologist. Overall, diagnostic errors were found in 69/182 (38%) children. Moderate and major discrepancies affecting the final management were found in 42/182 (23%) children. Discrepancies were higher when the echocardiogram was done by personnel other than pediatric cardiologist (P < 0.01) and with moderate and high complexity lesions (P = 0.0001). There was no significant difference in proportion of these discrepancies in children ≤ 1 year vs. >1 year of age. CONCLUSIONS: A significant number of pediatric echocardiograms done at non-tertiary centers had discrepancies that affected the management of these children. More discrepancies were seen when the echocardiogram performer was not a pediatric cardiologist and with complex CHD.

11.
Pediatr Cardiol ; 35(1): 149-54, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23860614

RESUMO

Congenital heart disease contributes significantly to the health burden of children in Nigeria. Interventions for congenital heart disease have been available in the developed world since the first report on device closure of patent ductus arteriosus (PDA) in 1967 by Porstmann. However, this did not start in Nigeria until October 2010. This study aimed to document the profiles of the patients who had undergone interventions for congenital heart diseases since the availability of the procedure, the challenges encountered, and the prospects associated with the interventions at the study site. All the patients referred to undergo interventions for congenital heart disease at the study center between October 2010 and 2012 were studied. The profile of the patient, including diagnosis at referral, indication for interventions, and interventions performed, were documented. The patients ranged in age from 3 to 62 years (mean age, 13.54 ± 17.7 years), and the male-to-female ratio was 1:3. The diagnosis at referral included PDA in 10 (83 %) of the 12 patients and secundum atrial septal defect in 2 patients (17 %). They all had transcatheter closure of the defects. Interventional procedures for congenital heart diseases currently are available locally, but the high degree of manpower training required, the cost, and the local availability of consumables are major factors limiting their use. Regional and international collaboration could be mutually beneficial.


Assuntos
Cateterismo Cardíaco/estatística & dados numéricos , Procedimentos Cirúrgicos Cardiovasculares/estatística & dados numéricos , Permeabilidade do Canal Arterial , Comunicação Interatrial , Adolescente , Cateterismo Cardíaco/métodos , Procedimentos Cirúrgicos Cardiovasculares/métodos , Pré-Escolar , Estudos Transversais , Permeabilidade do Canal Arterial/diagnóstico , Permeabilidade do Canal Arterial/epidemiologia , Permeabilidade do Canal Arterial/cirurgia , Feminino , Acessibilidade aos Serviços de Saúde , Necessidades e Demandas de Serviços de Saúde , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/epidemiologia , Comunicação Interatrial/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Nigéria/epidemiologia , Avaliação de Resultados em Cuidados de Saúde , Estudos Prospectivos , Encaminhamento e Consulta/estatística & dados numéricos
12.
Ann Pediatr Cardiol ; 5(2): 156-9, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23129905

RESUMO

BACKGROUND: Fetal echocardiography is a well established sensitive tool to diagnose congenital heart disease (CHD) in utero. One of the determinants of effective utilization of fetal echocardiography is its awareness in the general population. The present hospital based study was undertaken to assess the awareness of the need for fetal echocardiography amongst Indian parents. METHODS: One thousand one hundred and thirty eight consecutive parents who visited the pediatric cardiology outpatient department of a tertiary care centre over a period of two months were asked to fill up a questionnaire that included their demographic data, educational status, history of CHD in children, awareness of fetal echocardiography and source of information and timing of fetal echocardiogram if performed. The data was categorized and awareness was noted in different groups. RESULTS: The awareness in the study population was 2.2%. Awareness was found to be similar across the study population irrespective of the demographics and high risk status of the parents. CONCLUSIONS: The awareness of fetal echocardiography, an important tool in reducing the incidence of complex CHD, thereby impacting public health, is alarmingly low in the population studied. Appropriate action to increase awareness of fetal echocardiography needs to be looked into.

13.
Indian Heart J ; 64(1): 50-3, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22572426

RESUMO

BACKGROUND: Factors that have led to the increasing popularity of medical travel include the high cost of healthcare, long wait times for certain procedures, the ease and affordability of international travel, and improvements in both technology and standards of care in many countries. AIM: The present study aims to elaborate the factors that attract international cardiac patients to India, to document the proportion of the admissions into the paediatric cardiac ward who are international patients, and to identify the sources of funding of the international patients. METHODS: This was a prospective, cross-sectional, and analytical study carried out between May 2009 and October 2009 in the paediatric cardiac care unit of a large tertiary care cardiac centre in India paediatric wards. Structured questionnaires were administered. RESULTS: A total of 1372 patients were admitted during the study period, of which 155 (11.3%) were patients from countries outside India. Majority of the patients were from Malaysia (45%), Nigeria (23%), and Tanzania (15%). The age ranged from 1 month to 39 years with an average of 61 months. The male to female ratio was 1:1.4 and the majority of subjects (72.5%) were in social classes 3 and 4. cheaper cost and better expertise was the prominent reason for choosing India. More than half of the respondents were either sponsored by the government or self-funded. For patients from Nigeria 53% (9) were sponsored by self (parent), 29% (5) by non-governmental organisations (NGO), 12% (2) by the parent employer, and 6% (1) by the government. CONCLUSION: There is a need for local development of facilities and training of personnel in specialised areas of healthcare to provide succour for a significant number of nationals who might otherwise have suffered and possibly have even died of their ailment. There is also the added advantage that such facilities would save foreign currency and help boost our economy.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Acessibilidade aos Serviços de Saúde , Cardiopatias Congênitas/cirurgia , Turismo Médico , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/economia , Criança , Pré-Escolar , Competência Clínica , Unidades de Cuidados Coronarianos , Redução de Custos , Estudos Transversais , Feminino , Financiamento Governamental , Financiamento Pessoal , Pesquisas sobre Atenção à Saúde , Acessibilidade aos Serviços de Saúde/economia , Cardiopatias Congênitas/economia , Custos Hospitalares , Humanos , Índia , Lactente , Masculino , Turismo Médico/economia , Estudos Prospectivos , Classe Social , Inquéritos e Questionários , Centros de Atenção Terciária , Fatores de Tempo , Meios de Transporte/economia , Listas de Espera , Adulto Jovem
14.
Ann Pediatr Cardiol ; 5(1): 69-71, 2012 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-22529606

RESUMO

A 4-year-old boy presented with repeated respiratory tract infections. Echocardiography showed dilation of the left main coronary artery with flow into the Right Ventricular Outflow Tract (RVOT). Diagnosis of Left Coronary Artery Aneurysm (LMCA) with RVOT fistula was made. A surgical repair of LMCA aneurysm by two-patch technique was performed. The patient had an excellent outcome postoperatively and is asymptomatic on follow-up.

15.
Ann Pediatr Cardiol ; 4(1): 41-4, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21677804

RESUMO

INTRODUCTION: Pulmonary vascular disease is a risk factor in the surgical management of patients with d-transposition of great arteries (d-TGA) and a ventricular septal defect (VSD). In older infants or children with this physiology, the question of operability often arises. Cardiac catheterization in this condition can be fallacious. It is well known that oxygen reduces pulmonary arterial pressure and pulmonary vascular resistance especially where irreversible pulmonary vascular obstructive disease has still not set in. We tried to implement this effect of oxygen in correlation with echocardiography in patients with TGA-VSD physiology where operability was in question. METHODS: Patients with d-TGA and a large post tricuspid shunt in whom operability was considered doubtful were selected for the study. We administered humidified oxygen at the rate of 10 litres/minute by mask for 48 hours in the ward or intensive care unit. After administration of oxygen we reassessed the child echocardiographically looking for signs of lowering of pulmonary vascular resistance which included increased pulmonary venous blood flow to the left atrium (LA) and right to left shunting across the VSD. OBSERVATION: We studied 1 patient with d-TGA and aortopulmonary window (APW), 4 patients with TGA / large VSD and 1 patient with Taussig-Bing anomaly. The age of the studied children ranged from 4 months to 3 years with a mean age of 1.1 years. After administering oxygen as described, echocardiogram showed an increase in pulmonary venous blood flow to the LA and right to left shunting across the VSD in 5 patients and increased flow reversal in aorta in presence of the APW. CONCLUSION: Patients with TGA/VSD physiology with doubtful operability can be subjected to this method of determining operability using echocardiography after administering oxygen. Although not 100% accurate in predicting long term postoperative pulmonary hypertension, this is a simple, noninvasive method that can aid in decision making in such a situation.

16.
Cardiol Young ; 21(1): 19-25, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-20883597

RESUMO

OBJECTIVES: To analyse the relative frequency and gender ratios in the procedures (both surgical and catheter-based interventions) performed for cardiac diseases in Indian children. BACKGROUND: Not many studies are available in the developing countries with regard to the prevalence, relative frequency, or gender distribution of cardiac diseases in children. As universal newborn screening is not carried out for congenital cardiac diseases, the statistics are difficult to ascertain. Do female and male children with cardiac disease get equal parental preference in terms of surgical correction and catheter interventions in India? This question is also unanswered. METHODS: Analysis of 15,066 consecutive Indian children aged <18 years who were operated upon or had catheter intervention at a single tertiary care centre. Relative frequency and male/female ratios of cardiac lesions in these children were determined and compared with the studies in literature. RESULTS: Overall male/female ratio in the study was 1.4:1. Ventricular septal defect was the most common lesion (24.2%) with male/female ratio of 1.5:1, followed by tetralogy of Fallot (18.7%, 1.6:1), atrial septal defect (14.4%, 0.9:1) and so on. Male children dominated the total number of procedures performed. CONCLUSION: In most of the individual cardiac lesions, the relative frequency was different from that of international studies. The gender ratio for the majority of the individual type of cardiac problems was different from that of international references. These findings may suggest a preference for the male child in the treatment of cardiac diseases in India, which could possibly be related to social causes.


Assuntos
Procedimentos Cirúrgicos Cardíacos/estatística & dados numéricos , Cardiopatias/cirurgia , Hospitais/estatística & dados numéricos , Criança , Feminino , Cardiopatias/epidemiologia , Humanos , Índia/epidemiologia , Masculino , Prevalência , Estudos Retrospectivos , Distribuição por Sexo , Fatores Sexuais
17.
Pediatr Cardiol ; 31(6): 785-91, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20405116

RESUMO

We present a large single-center series (>2200 cases) operated for Tetralogy of Fallot (TOF). We analyzed the incidence of associated unusual and uncommonly described cardiac lesions and their diagnostic and therapeutic implications in TOF patients. This retrospective study was conducted by reviewing records of patients operated for TOF at a large tertiary care pediatric cardiac centre. From 2002 to 2008, a total of 2235 cases of TOF were evaluated with echocardiography, cardiac catheterization, and/or cardiac computed tomography followed by cardiac surgery. Known and well-described associations were excluded from the study. Unusual associations were tabulated. Several unusual associations having an incidence >0.1% were detected. These included subaortic membrane (1%), pulmonary venous abnormalities (0.5%), small left ventricle (0.5%), interrupted inferior vena cava (0.5%), mitral valve abnormalities (0.4%), hemitruncus (0.4%), tricuspid valve abnormalities (0.4%), biventricular dysfunction (0.3%), retroaortic innominate vein (0.3%), bicuspid aortic valve (0.2%), and pericardial effusion (0.2%). This series describes unusual, not previously routinely reported cardiac lesions associated with TOF that may affect management and should be sought on preoperative evaluation. We demonstrate that more unusual associations do exist in not infrequent numbers, i.e., a TET is not just a TET.


Assuntos
Anormalidades Múltiplas/diagnóstico , Procedimentos Cirúrgicos Cardíacos , Valvas Cardíacas/anormalidades , Ventrículos do Coração/anormalidades , Veias Pulmonares/anormalidades , Tetralogia de Fallot/diagnóstico , Veia Cava Inferior/anormalidades , Anormalidades Múltiplas/epidemiologia , Adolescente , Adulto , Cateterismo Cardíaco , Criança , Pré-Escolar , Ecocardiografia , Feminino , Humanos , Incidência , Índia/epidemiologia , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Tetralogia de Fallot/cirurgia , Tomografia Computadorizada por Raios X , Adulto Jovem
18.
PLoS One ; 5(4): e10286, 2010 Apr 21.
Artigo em Inglês | MEDLINE | ID: mdl-20422016

RESUMO

BACKGROUND: Parental consanguinity is a risk factor for congenital heart disease (CHD) worldwide, suggesting that a recessive inheritance model may contribute substantially to CHD. In Bangalore, India, uncle-niece and first cousin marriages are common, presenting the opportunity for an international study involving consanguinity mapping of structural CHD. We sought to explore the recessive model of CHD by conducting a genome-wide linkage analysis utilizing high-density oligonucleotide microarrays and enrolling 83 CHD probands born to unaffected consanguineous parents. METHODOLOGY/PRINCIPAL FINDINGS: In this linkage scan involving single nucleotide polymorphism (SNP) markers, the threshold for genome-wide statistical significance was set at the standard log-of-odds (LOD) score threshold of 3.3, corresponding to 1995ratio1 odds in favor of linkage. We identified a maximal single-point LOD score of 3.76 (5754ratio1 odds) implicating linkage of CHD with the major allele (G) of rs1055061 on chromosome 14 in the HOMEZ gene, a ubiquitously expressed transcription factor containing leucine zipper as well as zinc finger motifs. Re-sequencing of HOMEZ exons did not reveal causative mutations in Indian probands. In addition, genotyping of the linked allele (G) in 325 U.S. CHD cases revealed neither genotypic nor allele frequency differences in varied CHD cases compared to 605 non-CHD controls. CONCLUSIONS/SIGNIFICANCE: Despite the statistical power of the consanguinity mapping approach, no single gene of major effect could be convincingly identified in a clinically heterogeneous sample of Indian CHD cases born to consanguineous parents. However, we are unable to exclude the possibility that noncoding regions of HOMEZ may harbor recessive mutations leading to CHD in the Indian population. Further research involving large multinational cohorts of patients with specific subtypes of CHD is needed to attempt replication of the observed linkage peak on chromosome 14. In addition, we anticipate that a targeted re-sequencing approach may complement linkage analysis in future studies of recessive mutation detection in CHD.


Assuntos
Consanguinidade , Ligação Genética , Estudo de Associação Genômica Ampla/métodos , Cardiopatias Congênitas/genética , Proteínas de Homeodomínio/genética , Fatores de Transcrição/genética , Cromossomos Humanos Par 14 , Genes Recessivos , Predisposição Genética para Doença , Genoma Humano , Humanos , Índia/epidemiologia , Escore Lod
19.
Indian Heart J ; 61(1): 55-7, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19729690

RESUMO

We prospectively analyzed the results of extracorporeal membrane oxygenation (ECMO) done in our Pediatric Cardiac Unit from January to December 2007. We present a series of nine patients who required ECMO after open heart surgery for congenital heart disease (CHD). The overall survival rate of these patients was 77.8%. The survival rate was 100% in patients who were electively put on ECMO after intracardiac repair for tetralogy of fallot, 50% in patients who were put on ECMO following the arterial switch operation and 50% in patients who were put on ECMO after VSD closure. Our results seem to suggest that patients placed on ECMO electively rather than after a cardiac arrest posts congenital cardiac surgeries have a better outcome.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Oxigenação por Membrana Extracorpórea , Cuidados Pós-Operatórios/métodos , Pré-Escolar , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Recém-Nascido , Estudos Prospectivos , Resultado do Tratamento
20.
Pediatr Cardiol ; 30(7): 1043-5, 2009 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-19705190

RESUMO

The commonly used Darling classification for total anomalous pulmonary venous connection (TAPVC) consists of supracardiac, cardiac, infracardiac, and mixed types (Craig et al., Lab Invest 6:44-64, 1967). In supracardiac TAPVC, the common pulmonary vein drains superiorly into the left innominate vein, the superior vena cava, or the azygos vein by way of an ascending vertical vein. We describe a case of supracardiac TAPVC draining into the azygos vein atypically by way of a descending vertical vein.


Assuntos
Cardiopatias Congênitas/diagnóstico , Veias Pulmonares/anormalidades , Diagnóstico Diferencial , Ecocardiografia Doppler em Cores , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Veias Pulmonares/cirurgia , Tomografia Computadorizada por Raios X
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