Impact of COVID-19 pandemic on pediatric cardiac services in India.

BACKGROUND: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. AIMS: The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. METHODS: We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. RESULTS: The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). CONCLUSIONS: The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

Successful surgical repair of pulmonary dominant common arterial trunk without aortic arch obstruction in a neonate.

The pulmonary dominant variant of the common arterial trunk has always been reported to be associated with aortic coarctation, or interruption of the aortic arch, along with a duct-dependent systemic circulation. This mandates a complex surgical repair with attendant high surgical mortality. We report a 23-day-old baby with a pulmonary dominant trunk with mild aortic hypoplasia but with an arch free of coarctation or interruption, who underwent successful surgical repair. In the preoperative evaluation of a common arterial trunk, pulmonary dominance may not necessarily denote an adverse risk factor when the aorta is only mildly hypoplastic.

Pulmonary artery banding for univentricular heart beyond the neonatal period.

BACKGROUND: It is standard practice to band the pulmonary artery at 2 to 4 weeks of age in patients with univentricular hearts with increased pulmonary blood flow. The behavior of patients banded beyond the neonatal period has not been well elucidated. PATIENTS AND METHODS: This was a retrospective chart review of 32 consecutive patients (one neonate) who underwent pulmonary artery banding for functionally univentricular heart. The mean age at banding was 5.7 ± 6.0 months, and 34.4% were over 6-months old. RESULTS: Mortality was 15.6%. The mean systolic pulmonary artery pressure decreased from 43.6 ± 9.7 to 29.6 ± 7.0 mm Hg. The mean pre-discharge echocardiographic band gradient was 60.6 ± 13.6 mm Hg (mean systemic systolic pressure 73.7 ± 11.0 mm Hg) and systemic oxygen saturation was 81.7% ± 5.8%. At a mean follow-up period of 44.9 ± 30.0 months, 6 patients were lost to follow-up, 13 had undergone bidirectional Glenn shunt, and 7 had Fontan operations. Pulmonary artery mean pressure was 17.2 ± 4.6 mm Hg at pre-Glenn catheterization. Of the 5 patients who had not undergone further surgery, only one was inoperable. All were in functional class I or II. CONCLUSION: Pulmonary artery banding beyond the neonatal period in suitable patients with univentricular hearts provides reasonable palliation in the intermediate term, with a significant number successfully undergoing Fontan stages.

Prosthetic reconstruction of bicuspid pulmonary valve in tetralogy of Fallot.

BACKGROUND: Various techniques have been described for preserving pulmonary valve function in tetralogy of Fallot repair. In selected substrates, the pulmonary valve can be bicuspidized and preserved using polytetrafluoroethylene pericardial membrane. METHODS: This study was a retrospective review of 20 patients aged 11 months to 31 years with tetralogy of Fallot and a bicuspid pulmonary valve (with anteroposterior cusps) who underwent intracardiac repair from August 2010 to January 2013. The anterior cusp was augmented using polytetrafluoroethylene pericardial membrane to preserve the valve hinge. A transannular patch was used in all cases. Data relating to surgical outcome, intensive care unit course, pulmonary regurgitation, and right ventricular outflow tract gradient were collected. RESULTS: There was no mortality. Predischarge pulmonary regurgitation was ≤grade 2 in 18 (90%) patients. One patient had an outflow gradient >40 mm Hg. On follow-up of 3-24 months, there was no increase in outflow gradient; 18.7% had progression of pulmonary regurgitation. CONCLUSION: In selected substrates, this technique is associated with minimization of pulmonary regurgitation and an excellent functional outcome. The utility of this technique needs to be validated over a longer time scale in a larger series.

Surgical outcome of congenital heart disease in Down's syndrome.

BACKGROUND AND OBJECTIVE: only a few studies have compared surgical outcomes in children with and without Down's syndrome. The purpose of this study was to investigate the surgical outcome in children with Down's syndrome and congenital heart disease and to compare it with that in age- and sex-matched non-Down controls. PATIENTS AND METHODS: the charts of 32 consecutive Down's syndrome patients who underwent surgery for congenital heart disease in a single center from January 2006 to May 2010, were compared with the data of 32 age- and sex-matched non-Down controls, focusing on comparative echocardiographic details, surgical outcome, and intensive care unit data. The data were compared using the 2-group t test. The children were followed up for a median period of 48 months. RESULTS: the most common cardiac lesion in the study group was ventricular septal defect (41%) followed by atrioventricular septal defect (28%). Ventilation and intensive care unit stay were significantly longer in Down's syndrome children, with a trend towards more respiratory complications. At a median follow-up of 48 months, there were no cardiac events or mortality in the study group. Simple lesions were more common than atrioventricular septal defect in Down's syndrome. CONCLUSION: children with Down's syndrome can be operated on with negligible mortality and good functional outcome, but with a higher surgical morbidity.

Visceral heterotaxy in the developing world.

BACKGROUND: The challenge of visceral heterotaxy (VH) in the developing world has not been analysed in detail. METHOD: Retrospective chart review of 69 consecutive patients over ten years assessed the clinical profile and surgical outcome of VH. Median age: 3 years; median weight: 15kg. Diagnosis was made by echocardiography supplemented with blood smear (Howell Jolly bodies), Multi-Detector Computed Tomography (MDCT) angiogram and/or surgical inspection. RESULTS: In right isomerism (RI) group (n=32), 12 patients did not undergo surgery, five had Blalock Taussig shunt, 14 had bidirectional Glenn and one had Fontan completion, with surgical mortality of 5%. In left isomerism (LI) group (n=31), 11 patients underwent two ventricle repair (35%) and 15 (48%) had single ventricle repair, with surgical mortality of 3.8%; five did not have surgery. On follow up (median period 1.5 years), 33% of un-operated patients and 25% of operated patients died, mortality being higher for RI patients. Late mortality was due to sepsis, heart failure or arrhythmia. CONCLUSION: VH can be diagnosed by imaging based criteria. VH tends to present late in the developing world with a significant percentage inoperable. LI had better surgical outcome and higher long term survival.

Occult systemic lupus erythematosus with active lupus nephritis presenting as Libman-Sacks endocarditis.

The diagnosis of systemic lupus erythematosus (SLE) depends on clinical evidence of renal, rheumatologic, cutaneous, and neurologic involvement, supported by serological markers. A previously healthy 14-year-old girl presented with Libman-Sacks endocarditis involving the aortic valve as the first manifestation of SLE. Even though she did not satisfy the American College of Rheumatology criteria for diagnosing SLE, she had anemia, proteinuria, elevated erythrocyte sedimentation rate, low complement 4 (C4) levels, and strongly positive antinuclear antibody titer. A renal biopsy showed stage IV lupus nephritis. Treatment was initiated with immunosuppressants and steroids. This type of presentation may be misdiagnosed as infective endocarditis missing the underlying collagen vascular disease.

Factor VII for excessive bleeding following congenital heart disease surgery.

Recombinant activated factor VII may be effective in patients with severe bleeding following congenital heart disease surgery requiring cardiopulmonary bypass. From August 2009 through June 2011, 13 patients (median age, 5 months) were given recombinant activated factor VII for severe bleeding after open heart surgery, preventing sternal closure 2 h after completion of cardiopulmonary bypass, or chest tube drainage >10 mL · kg(-1) · h(-1) for 2 h. The median dose was 75 µg · kg(-1). The response was positive if postoperative bleeding decreased, permitting sternal closure in the operating room, or if there was >50% decrease in chest tube drainage. The 3 patients who received factor VIIa in the operating room had successful sternal closure within 1 h. Of the 10 patients who received factor VIIa in the intensive care unit, drainage decreased to <50% in 1 h in 6 cases, and a second dose was required in 4. None required surgical reexploration. There were 4 deaths, none was related to bleeding. Nine patients were discharged (median hospital stay, 29 days) and were doing well after 3 months. There were no thrombotic complications. Recombinant activated factor VII may be an effective rescue therapy for severe postoperative hemorrhage.

Scimitar syndrome with right hemianomalous pulmonary venous drainage into superior vena cava/right atrium junction.

Scimitar syndrome is characterized by anomalous pulmonary venous drainage of a part or the whole of the right lung into the inferior vena cava (IVC). It is extremely uncommon in scimitar syndrome for the pulmonary veins to drain anomalously to a site other than the IVC. The following case report describes an otherwise classical scimitar syndrome with anomalous drainage of the right lung cephalad into the right atrium near its junction with the superior vena cava.

64-slice multidetector-row computed tomographic angiography for evaluating congenital heart disease.

This study aimed to assess critically the role of 64-slice multidetector-row computed tomographic (MDCT) angiography for evaluating congenital heart disease. The study enrolled 60 consecutive patients (median age, 4.7 years; median weight, 16.5 kg) with congenital heart disease who underwent 64-slice MDCT angiography during the period June 2006 through September 2007. The results were classified as diagnostic categories, and the impact of the procedure on strategizing management was critically analyzed. In each of the groups, the current technique offered a clear advantage over conventional imaging and provided specific clues for surgical/interventional management. A management algorithm was evolved based on questions frequently asked about pulmonary artery anatomy. The correlation with surgical anatomy in all cases that involved surgery was excellent. Early results suggest that 64-slice MDCT angiography is a major breakthrough in cardiovascular imaging with an important diagnostic and decision-aiding role. Diagnostic cardiac catheterization, especially for evaluating great vessel anomalies, could be largely replaced by the described technique for congenital heart disease.