Consensus Statement of the Neurodevelopmental Pediatrics Chapter of Indian Academy of Pediatrics (IAP) on the Management of Children With Down Syndrome

Justification: The diagnosis of Down syndrome (DS) is easily made clinically but the management is multi-disciplinary and life-long. There is no standard protocol available for its management in India. Process: A committee was formed under the Indian Academy of Pediatrics (IAP) chapter of Neuro developmental pediatrics consisting of 20 experts working in the related field. The various aspects of the condition were discussed and allotted to the concerned experts related for preparing the guidelines. The material received was collated to form a set of guidelines, which were reviewed by the committee, and a consensus statement made. The guidelines were then approved by the chapter, and by the IAP. Objectives: To define the condition and to look into the various aspects of antenatal and postnatal diagnosis. To explain briefly about the involvement of the various systems that are involved and formulate recommendations for management. To recommend early and sustained interventional therapies to enable children with DS lead an independent life. Recommendations: The stress on bio-psycho-social strategy for the management of children with DS is reiterated, and the need for a medical, social and rights model is recommended after each section. The age-wise recommendations are also highlighted in addition to the recommendations under each system.

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.

Early Neurodevelopmental Outcomes After Corrective Cardiac Surgery In Infants

Objective: To assess neurodevelopmental status in Indian infantsundergoing corrective surgery for congenital heart disease (CHD)and to analyze factors associated with neurodevelopmental delay.Design : Cross-sectional study.Setting: Tertiary-care pediatric cardiology facility.Participants: Consecutive infants undergoing corrective surgeryfor CHD (January 2013 –December 2014). Palliative procedures,and patients with known genetic syndromes were excluded.Main outcome measures: Neurodevelopmental evaluation 3months, and one year after surgery using DevelopmentalAssessment Scales for Indian Infants (DASII); scores werecategorized as delayed if ?70.Results: Of the 162 children enrolled, delayed PDI and MDIscores were observed in 33.5% and 19.6% of patients at 3months, respectively; this reduced to 14.5 % on 1-year follow-up.On multivariate analysis, delayed PDI outcome at one year waspredicted by early term birth and one-year postoperative headcircumference Z-score <–2. Delayed MDI was associated withhigher mean perfusion pressure on cardiopulmonary bypass.Cardiac diagnosis and peri-operative factors did not impactneurodevelopmental outcomes.Conclusions: Neurodevelopmental status is delayed in 14.5% ofinfants one year after corrective infant heart surgery

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

Impact of the International Quality Improvement Collaborative on outcomes after congenital heart surgery: A single center experience in a developing economy.

Background: The International Quality Improvement Collaborative (IQIC) for Congenital Heart Surgery in Developing Countries was initiated to decrease mortality and major complications after congenital heart surgery in the developing world. Objective: We sought to assess the impact of IQIC on postoperative outcomes after congenital heart surgery at our institution. Methods: The key components of the IQIC program included creation of a robust worldwide database on key outcome measures and nurse education on quality driven best practices using telemedicine platforms. We evaluated 1702 consecutive patients ≤18 years undergoing congenital heart surgery in our institute from January 2010-December 2012 using the IQIC database. Preoperative variables included age, gender, weight at surgery and surgical complexity as per the RACHS-1 model. The outcome variables included, in- hospital mortality, duration of ventilation, intensive care unit (ICU) stay, bacterial sepsis and surgical site infection. Results: The 1702 patients included 771(45.3%) females. The median age was 8 months (0.03-216) and the median weight was 6.1Kg (1-100). The overall in-‑hospital mortality was 3.1%, Over the three years there was a significant decline in bacterial sepsis (from 15.1%, to 9.6%, P < 0.001), surgical site infection (11.1% to 2.4%, P < 0.001) and duration of ICU stay from 114(8-999) hours to 72 (18-999) hours (P < 0.001) The decline in mortality from (4.3% to 2.2%) did not reach statistical significance. Conclusions: The inclusion of our institution in the IQIC program was associated with improvement in key outcome measures following congenital heart surgery over a three year period.

Clinical Screening for Congenital Heart Disease at Birth: A Prospective Study in a Community Hospital in Kerala.

Objective: To develop a clinical strategy for detection of Congenital heart disease (CHD) in the newborn through a combination of clinical signs and pulse oximetry. Design: Prospective longitudinal study. Setting: Community level hospital in the city of Kochi, Kerala. Participants and interventions: All consecutive newborns between June 2006 and February 2009 were prospectively screened for CHD, 48 hours after birth. The on-site pediatrician performed clinical screening. A study nurse recorded pulse oximetry in a lower extremity; value of <94% was defined as abnormal. Echocardiography was performed on site by a trained research officer. A 6- week clinical follow-up evaluation was done for all. Main outcome measure: Detection of CHD by echocardiography. Results: Of 5487 babies screened, 425 (7.75%) had CHD. 17 (0.31%) had major CHD, two of whom (one ALCAPA and one large VSD) were missed during the initial evaluation. The rest were minor CHD (408 patients, 7.44%), most of which normalized by 6 weeks. On multivariate analysis, murmur, central cyanosis, abnormal precordial pulsations and abnormal pulse oximetry emerged as significant predictors of CHD. The sensitivity of clinical evaluation and pulse oximetry combined was 19% for all CHDs and 20% for major CHD; specificity was 88%. Conclusions: In the community setting of a developing country, clinical evaluation and pulse oximetry after birth had a very low sensitivity for detection of CHD. Though an abnormal screening warrants prompt echocardiography, a 6-week clinical evaluation is recommended to ensure that major CHD is not missed.

Blood Pressure Distribution in Indian Children.

Objective: To determine blood pressure distribution in schoolchildren and to derive population specific reference values appropriate for age, gender and height status. Design: Cross sectional observational study. Setting: Schools in Ernakulam district, Kerala, India, during 2005-06. Methods: Stratified random cluster sampling method was used to select the children. Blood pressure and anthropometric data were collected from 20,263 students of 5-16 years age. Three readings of blood pressures of each child were taken by mercury sphygmomanometer and mean was taken for analysis. Blood pressure percentiles in relation to gender, age and height were estimated from a non-overweight population of 18,931 children using polynomial regression models. Results: Children from study population have higher diastolic pressures for both sexes than international standard across all age groups. For systolic blood pressure, girls showed higher values than the international standard while for boys, the difference appears to be minimal. Conclusions: Blood pressure distribution in children from our study population demonstrates a different pattern in comparison to existing international reference. Higher blood pressure values in the study population are of considerable public health significance.

Vein of galen arteriovenous malformation: diagnostic clues from echocardiography.

We report a rare association of vein of Galen malformation in a young infant with associated sinus venosus atrial septal defect and aortic arch hypoplasia. This unique association of lesions provides insights into the embryological basis as well as offers potential echocardiographic clues for the diagnosis of the cerebral arterio-venous malformation.

Stenting the patent arterial duct to increase pulmonary blood flow.

BACKGROUND: Use of surgically created aoropulmonary shunt is well-established for improving pulmonary blood flow in infants with critical reduction in pulmonary blood flow. Recently, stenting the patent ductus arteriosus has emerged as an alternative in selected infants with congenital heart disease and reduced pulmonary blood flow. METHODS AND RESULTS: We reviewed records of consecutive infants undergoing stenting of patent ductus arteriosus between August 2003 and October 2005 at our institution. Two of 12 patients underwent patent ductus arteriosus stenting to facilitate preparation of left ventricle for transposition with intact septum. We report the case selection, technique, immediate and short-term follow-up outcome in the remaining 10 patients [median age: 16 days (range 4-290 days): weight 2.7 kg (range 2-6 kg)] with reduced pulmonary blood flow who underwent stenting of patent ductus arteriosus as an alternative to conventional surgical aortopulmonary shunts. Five of the 6 newborns were prostaglandin-dependent and 4 had previously undergone guidewire perforation of the pulmonary valve (n=2) or balloon dilation (n=2). Successful stent implantation was accomplished in all with no major patient-related complication (median fluoroscopy time: 18.6 min; range: 7.7-72 min). The intensive care unit and hospital stays were prolonged in 3 patients because of sepsis (n=2) and pulmonary over-circulation with sepsis (n=1). On follow-up (median 5.5 months; range 1-19 months) all implanted stents were patent. One patient underwent re-dilation of the implanted stent for declining saturations. CONCLUSIONS: The immediate and short-term follow-up results of stenting of the patent arterial duct, as an alternative to the surgical aortopulmonary shunt in carefully selected newborns and infants is encouraging.

Transcatheter management of patent ductus arteriosus in sick ventilated small infants.

BACKGROUND: Large patent ductus arteriosus can present in infancy with congestive cardiac failure and superadded pulmonary infection can necessitate mechanical ventilation. Surgical intervention is traditionally indicated for this subset of patients. We present our experience of transcatheter coil closure of the patent ductus arteriosus in such infants. METHODS AND RESULTS: Five infants weighing between 960 gm and 4.1 kg, aged between 17 days and 3 1/2 months were mechanically ventilated because of congestive cardiac failure with pneumonia. Echocardiography showed patient ductus arteriosus with a size of 1.8 to 4.2 mm and adequate ampulla. Bioptome-assisted coil delivery was done and successful patient ductus arteriosus closure was achieved in all. There were two instances of embolization of coils with successful retrieval and redeployment. All infants could be weaned off mechanical ventilation over the next 24-72 hours. A pre-term infant developed a Doppler gradient of 25 mmHg in the descending aorta that decreased to 12 mmHg five months later. There was no significant obstruction to pulmonary artery flow in any child. At three months follow-up, all the five infants were asymptomatic with no residual flow across the patient ductus arteriosus. CONCLUSIONS: Transcatheter coil closure of moderate to large patent ductus arteriosus is possible in sick ventilated infants weighing below 5 kg. It may be a better alternative to surgery in selected cases in view of minimal morbidity.