Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication

ustification: 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 whenrecommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and mayhave co-existing morbidities and malnutrition. Process: Guidelines emerged following expert deliberations at the National ConsensusMeeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of MedicalSciences, 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 heartdiseases; (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 congenitalheart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductusarteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heartdiseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebsteinanomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Guidelines for the management of common congenital heart diseases in India: A consensus statement on indications and timing of intervention

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.

Utility of world heart federation and modified World Health Organization echocardiographic diagnostic criteria of subclinical rheumatic heart disease in children of Manipur, India

Background: A comparative prevalence studies using modified WHO and more stringent criteria of WHF2012 have not been conducted in this part of the country and so authors felt necessary to conduct such study in this North-eastern state of India.Methods: In this cross-sectional study, 3600 children aged 5-15 years (11.07±2.86, 51.90% male) from randomly selected schools of Manipur were examined clinically and by 2D colour doppler echocardiography.Results: Only one case of Clinical RHD with a combination of MR and AS was found, (prevalence of 0.28/1000) [CI: 0.04-1.97). Echocardiography detected 3 cases of definite subclinical (prevalence rate of 0.83/1000 (CI: 0.27-2.58], 14 cases of borderline subclinical RHD (prevalence rate of 3.9)/1000 (CI: 2.30-6.56) befitting WHF 2012 criteria, and 61 cases of borderline subclinical RHD befitting modified WHO criteria (prevalence rate of 16.9/1000)[ CI1.3-2.1].On follow up to 5 years, among the 3 cases with definite subclinical RHD, 2 had worsened and 1 remained non- progressive, while among the 14 subclinical borderline cases, 2 normalized, 6 remained non-progressive and 6 children were lost to follow up.Conclusions: Prevalence of RHD using echocardiography is several folds higher compared to clinical examination alone. While the number is reduced by about half using WHF criteria. However, the important of WHO criteria cannot simply be neglected, as all the subclinical cases by WHF criteria remains non-progressive, while one case of subclinical defined by WHO worsen on follow up.

Congenital Heart Disease in India: A Status Report

Considering a birth prevalence of congenital heart disease as 9/1000, the estimated number of children born with congenital heartdisease in India is more than 200,000 per year. Of these, about one-fifth are likely to have serious defect, requiring an intervention in thefirst year of life. Currently advanced cardiac care is available to only a minority of such children. A number of cardiac centers have beendeveloped over the last 10 years. However, most are in the private sector, and are not geographically well-distributed. Challenges topediatric cardiac care include financial constraints, health-seeking behavior of community, and lack of awareness. Government of Indiais taking a number of steps for improving health of children through its various program and schemes that are likely to benefit children withcongenital heart disease, especially those who are vulnerable and marginalized.

Oscillometric Blood Pressure in Indian School Children: Simplified Percentile Tables and Charts.

Background: Data on blood pressure recorded by oscillometric method is limited. Objective: To develop simplified tables and charts of blood pressure recorded by oscillometric method in children. Design: Cross-sectional. Setting:Ballabhgarh, Haryana. Participants: Healthy school-children. Main outcome measures: Blood pressure measured by oscillometric method. Results: The study group included 7,761 children (58.4% males) with mean (SD) age of 10.5 (2.8) years. Age and gender were used to create simplified percentile tables and charts, as height was seen to explain very little variability of either systolic or diastolic blood pressure. Formulae for SBP and DBP thresholds for hypertension were derived as [110 + 1.6 x age] and [79 + 0.7 x age], respectively, with 1 mm Hg to be added for females. 95th percentile values suggest simple levels indicating hypertension to be 120/80, 125/85 and 135/90 at ages of 5, 10 and 15 years, respectively. Conclusions: Simplified reference tables and charts, formulae for SBP and DBP, and simple convenient thresholds may be useful for rapid screening of hypertension using oscillometric method.

The National Rheumatic Heart Consortium: A nationwide initiative for the control of rheumatic heart disease in India.

CHALLENGES OF RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE CONTROL IN INDIA Rheumatic fever (RF) and rheumatic heart disease (RHD) continue to be important public health issues in many low- and middleincome (LAMI) countries affecting children and young people living in conditions of poverty, poor sanitation and overcrowding.1,2 These conditions have been nearly eliminated in the high-income countries, but continue to be common in Africa, Asia and the Pacific.3–5 Over 15 million people around the world suffer from RHD, resulting in approximately a quarter million deaths every year. RHD is the most commonly acquired heart disease found among children and young people in LAMI countries including India.1 There is a perception that the disease burden has declined in parts of India where human development indices have improved.6,7 Though epidemiological data are limited, RHD may have declined in some of the major cities of India, especially in southern India.8 However, in many parts of India with poor human development indices, the high disease burden may not have declined.9,10 There is a paucity of epidemiological data from poorly served rural populations, urban slums and tribal pockets.6 The loss of productivity and costs of care of the large number of currently affected patients with established valvular RHD in India is likely to be formidable. There are vertical disease-specific national programmes for tuberculosis, malaria and HIV infection, but RF and RHD have not received the same attention from policy-makers.11 This has contributed to the ‘neglect’ of RF and RHD in India. Other important reasons include the widespread perception among cardiologists, cardiac surgeons and key opinion leaders in cardiovascular medicine that RF and RHD are no longer important public health issues. These perceptions are formed because the population most affected by RF and RHD is getting increasingly marginalized and may not be in the consciousness of cardiovascular professionals. Most cardiologists and cardiac surgeons practise in tertiary centres in urban areas. It is difficult for the population affected by RHD to seek treatment at these centres. Besides, there are competing priorities for overworked cardiologists and cardiac surgeons that include the coronary artery disease burden which has acquired the status of an epidemic. Paediatric cardiologists and heart surgeons are now looking after an increasing number of children with congenital heart defects that have come to notice as infant mortality has declined in many parts of India.12,13 The consequences of the neglect of RF and RHD are potentially devastating. A good example is the unfortunate situation with penicillin in India. The majority of patients with RHD do not receive adequate secondary penicillin prophylaxis. A number of

Drug therapy of cardiac diseases in children.

Justification: The indications and doses of most drugs used for heart ailments in children are extrapolated from data in adult patients. Separate guidelines are needed for neonates, infants and children because of the differences in underlying heart diseases and metabolic clearance of some of these drugs. Process: Consensus emerged following expert deliberations at the National Meeting on Management of Congenital Heart Diseases in India, held on 13th September 2008, at the All India Institute of Medical Sciences, New Delhi, India, supported by Pediatric Cardiac Society of India. Objectives: To review the literature and frame evidence based guidelines for (i) indications, doses, adverse effects and safety profile of commonly used drugs in pediatric cardiology practice; and (ii) to provide an algorithm for treatment in various clinical settings. Recommendations: Consensus review and recommendations are given for drugs used in children for heart failure, hypertension, thrombosis, supraventricular tachycardia and intensive care. Guidelines are also given for use of intravenous immunoglobulins and sildenafil in children.

Prevalence of rheumatic heart disease: Has it declined in India.

Rheumatic heart disease continues to be a major health problem in many parts of the world. The epidemiology of rheumatic heart disease in India is of special interest as it may help to understand the effects of economic transition on this enigmatic disease. Critical appraisal of the published literature suggests the possibility of a real decline in the occurrence of the disease in some parts of the country, but a continuing onslaught in several other regions. The rate of decline seems to correlate more with improved public health facilities than with economic development alone. However, the cumulative burden of the disease remains high, and sustained efforts for the prevention of rheumatic heart disease are warranted.

Heart failure in children: clinical aspect and management.

Heart failure (HF) is a complex syndrome, with several definitions, the commonest being "an abnormality of cardiac function whereby heart in unable to pump at a rate commensurate with the requirement of the metabolizing tissues, or does so only at elevated filling pressures". In case of children, this requirement includes growth and development. Unlike as seen in adults, HF in children is commonly due to structural heart disease and reversible conditions. Thus the treatment for HF is commonly required for short term only. The overall outcome with HF is better in children than in adults. While the general principles on management are similar to those in adults, the evidence for the use of drugs in children is less convincing. It requires a judicious balance of extrapolation from adult medicine, keeping in mind that children are not small adults.

Consensus guidelines on pediatric acute rheumatic fever and rheumatic heart disease.

JUSTIFICATION: Acute rheumatic fever and rheumatic chronic valvular heart disease is an important preventable cause of morbidity and mortality in suburban and rural India. Its diagnosis is based on clinical criteria. These criteria need verification and revision in the Indian context. Furthermore, there are glaring differences in management protocols available in literature. These facts prompted Indian Academy of Pediatrics to review the management of rheumatic fever. PROCESS: Management of Rheumatic fever was reviewed and recommendation was formulated at national consultative meeting on 20th May 2007 at New Delhi. OBJECTIVES: To formulate uniform guidelines on management of acute rheumatic fever and rheumatic heart disease in the Indian context. Guidelines were formulated for the management of streptococcal pharyngitis, acute rheumatic fever and its cardiac complication as well as secondary prophylaxis for recurrent episodes. RECOMMENDATIONS: (1) Streptococcal eradication with appropriate antibiotics (Benzathine penicillin single dose or penicillin V oral or azithromycin). (2) Diagnosis of rheumatic fever based on Jones criteria. (3) Control inflammatory process with aspirin with or without steroids (total duration of treatment of 12 weeks). (4) Treatment of chorea according to severity (therapy to continue for 2-3 weeks after clinical improvement). (5) Protocol for managing cardiac complication like valvular heart disease, congestive heart failure and atrial fibrillation. (6) Secondary prophylaxis with benzathine penicillin and management of anaphylaxis.

Detection of 22 q11.2 hemizygous deletion by interphase FISH in a patient with features of CATCH 22 syndrome.

We report an eight years female child with clinical and molecular cytogenetic findings consistent with CATCH 22 syndrome characterized by cardiac defect, typical facial dysmorphism, mental deficiency and chromosome 22 q11.2 deletion. Interphase FISH with 22q 11.2 probe demonstrated hemizygous deletion in 98.5% nuclei. Interphase FISH for diagnosis of CATCH 22 syndrome has not been reported previously from India to our knowledge.

Use of Amplatzer duct occluder to close severely hypertensive ducts: utility of transient balloon occlusion.

BACKGROUND: The dividing line between a patent ductus arteriosus with severe but reversible pulmonary artery hypertension and an Eisenmenger duct remains elusive. Increasing experience with the Amplatzer duct occluder has made non-surgical closure of even large patent ductus arteriosus simple and safe. However, data on the use of this device in severly hypertensive ducti is sparse. METHODS AND RESULTS: There were six patients (2 males, 4 females), wherein transient ductal occlusion was done with an embolectomy balloon catheter. The post-occlusion hemodynamics along with overall clinical and hemodynamic assessment was used to decide the likelihood of benefit of closure and the choice of the device. In four of six patients of patent ductus arteriosus with severe pulmonary artery hypertension (pulmonary vascular resistance index> 8.0 U/m2) the duct was successfully closed using the Amplatzer duct occluder, while in two an Amplatzer ventricular septal defect occluder was used. CONCLUSIONS: Transient balloon occlusion is quite helpful for assessing patients with patent ductus arteriosus with severe pulmonary hypertension, and short-term non-invasive follow-up has shown this strategy to be safe and useful.

Congenital heart disease in India: a status report.

Pediatric cardiac care in India is still in its infancy. We have no data on congenital heart disease (CHD) prevalence at birth or on proportional mortality from CHD. The resources are not only limited but also are at times improperly utilized. There are very few specialized pediatric cardiology training programs, those that are, are concentrated in certain regions of India and are often imparted through combined adult and pediatric programs. The existing number of trained personnel for pediatric cardiology and pediatric cardiac surgery is inadequate. Above all there is no national policy for pediatric heart care. Increasing awareness of the problem amongst the pediatricians through CMEs, seminars, symposia is likely to be most helpful in early diagnosis and timely referral of cases. Training programs exclusively dedicated to pediatric cardiology and pediatric cardiac surgery need to be established in centres with good standards of pediatric cardiac care.

Fetal echocardiography: where are we?

This article reviews the indications, technique and benefits of fetal echocardiography. The impact of fetal echocardiography has become evident with better surgical outcome of infants with prenatal diagnosis of congenital heart disease. A major use of this technique has also been shown in the field of fetal arrhythmias. Fetal echocardiography is not only used to diagnose a specific type of arrhythmia, it is also very helpful in assessing the effect of the arrhythmia on the fetus and in guiding transplacental therapy. However it is important to remember that even experienced echocardiographers can make both false positive and false negative diagnosis of congenital heart disease. As fetal cardiac interventions including fetal cardiac surgery loom on the horizon, fetal echocardiography assumes further importance.

Duchenne muscular dystrophy: prevalence and patterns of cardiac involvement.

In about 10% cases of Duchenne muscular dystrophy (DMD), death is due to cardiac dysfunction. The recognition of cardiomyopathy in DMD is thus important. OBJECTIVE: To assess cardiac involvement in DMD patients by clinical, radiographic, electrocardiographic (ECG) and echocardiographic monitoring and correlate clinical parameters, CPK levels, presence of gene deletion and steroid therapy with cardiac involvement. METHODS: Thirty patients beyond 6 years age, with DMD in advanced stage disease/non-ambulatory were recalled. A detailed clinical evaluation, CPK levels, gene deletion studies were carried out. Cardiac investigations included Chest X-ray, 12 lead ECG and echocardiography. RESULTS: Nineteen patients were non-ambulatory at the time of enrollment. Symptoms or signs suggestive of cardiac dysfunction were seen in only 10%. Gene deletion was identified in 70.3%. Around one-third patients had cardiomegaly. ECG abnormalities were present in 93.3% patients and commonest abnormality was R > 4 mm in V1. Ejection fraction (EF) < 55% was observed in 64.2% and EF < 50% in 17.8%. CONCLUSION: Cardiomyopathy of DMD is characterized by lack of symptoms and few physical signs. Presence of subtle changes like sinus tachycardia may suggest early cardiac involvement. Thus echocardiography is required for evaluation of cardiac dysfunction. Presence of gene deletion was associated with higher CT ratio. Older children have been found to have higher heart rates. No other significant correlation with clinical parameters, CPK levels, genotype and steroid therapy was observed. Early detection possibly leads to appropriate treatment thus reducing the morbidity.