Profile of Cardiac Involvement in Children After Exposure to COVID-19.

OBJECTIVE: To evaluate the incidence and pattern of cardiac involvement in children post-COVID (coronavirus disease) infection in a tertiary care referral hospital in India. METHODS: A prospective observational study was conducted including all consecutive children with suspected MIS-C referred to the cardiology services. RESULTS: Of the 111 children with mean (SD) age was 3.5 (3.6) years, 95.4% had cardiac involvement. Abnormalities detected were coronary vasculopathy, pericardial effusion, valvular regurgitation, ventricular dysfunction, diastolic flow reversal in aorta, pulmonary hypertension, bradycardia and intra-cardiac thrombus. The survival rate post treatment was 99%. Early and short-term follow-up data was available in 95% and 70%, respectively. Cardiac parameters improved in majority. CONCLUSION: Cardiac involvement post COVID-19 is often a silent entity and may be missed unless specifically evaluated for. Early echocardiography aided prompt diagnosis, triaging, and treatment, and helps in favorable outcomes.

Successful percutaneous closure of a recurrent aorto-left ventricular tunnel with an Amplatzer Vascular Plug II.

Aorto-left ventricular tunnel is an extremely rare CHD clinically impressing as aortic regurgitation. This is usually corrected surgically, sometimes by percutaneous catheter intervention. Recurrent aorto-left ventricular tunnel has been reported occasionally. Reports of percutaneous closure of such recurrent aorto-left ventricular tunnels are rare. We hereby describe successful closure of a recurrent aorto-left ventricular tunnel with an Amplatzer Vascular Plug II.

Unveiling post-MIS-N cardiomyopathy by longitudinal multimodality global cardiac assessment from neonatal insult to 16-month follow-up.

A full-term male neonate presented on the 11th day of life with late-onset multisystem inflammatory syndrome-neonate (MIS-N) (cardioneurological compromise). Immediate anti-inflammatory modulation led to a gradual recovery of neurological and coronary lesions. However, temporal evaluation unmasked silent myocardial dysfunction in echocardiography validated further by elevated biomarkers, myocardial fibrosis in cardiac magnetic resonance imaging, and abnormal strain study persisting till 16 months of follow-up. This revealed a hitherto unknown and rare progression of MIS-N into dilated cardiomyopathy.

Unexpected late-onset aortic valvulitis and moderate regurgitation during longitudinal evaluation of atypical infantile Kawasaki disease: The heart beyond coronaries!

Kawasaki disease (KD) is the most common pediatric vasculitis with coronary involvement feared as the most serious complication. The reported case describes a child presenting initially with atypical KD and coronary artery aneurysms. He was treated with intravenous immunoglobulin and aspirin. In spite of adequate compliance and no clinical recurrence, serial echocardiography revealed nonregression of aneurysm and new-onset moderate aortic regurgitation (AR) in the subacute phase produced by prolapse of noncoronary cusp of the aortic valve. AR without aortic root dilatation from persistent inflammation of the valve leaflets in KD is a rare phenomenon. This case demonstrates unusual cardiac manifestations of KD and reoriented our protocol for long-term surveillance in infantile KD.

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.

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.

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.

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.

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.

Unconventional uses of septal occluder devices: Our experience reviewed.

UNLABELLED: Device closure is now an accepted modality of treatment for cardiac septal defects such as fossa ovalis Atrial Septal Defect (ASD), Ventricular Septal Defect (VSD) and Patent Ductus Arteriosus (PDA) and have well-accepted indication and long term results. Devices used for these defects have been specifically designed for use in closing these defects. In this manuscript, we are reporting the efficacy of closure of nonseptal defects with devices conventionally used for septal cardiac defects although they have not been prototyped for use in such conditions. AIM: To study use of occluder devices in nonseptal defects/malformation. MATERIAL & METHODS: 39 patients, in the age group 2-67 yrs, were treated percutaneously with occluder devices for various conditions. These included: coronary arteriovenous (CAV) fistula (n = 6), pulmonary AV fistula (n = 4), systemic AV fistula (vascular plug; n = 1), closure of AP window (duct occluder; n = 3), closure of ascending aorta perforation (septal occluder; n = 2), ruptured sinus of Valsalva (RSOV) (duct occluder; n = 13), Fontan fenestration closure (ASD septal occluder, patent foramen ovale device, vascular plug n = 3,1 each), splenic artery (duct occluder; n = 1), Balock Taussig shunt (duct occlude; n = 1)and closure of mitral paravalvular leak (n = 3; duct occlude devices = 2, VSD device: n = 1) and aortic paravalvular leak n = 2 (duct occluder; n = 2 additional vascular plug = 2). RESULTS: Procedural success: Successful closure as signified by no residual shunt was achieved in all coronary AV fistula (immediately n = 2, at 3 months in all), ruptured sinus of Valsalva (immediate in all), fenestrated Fontan (immediately in all), and ascending aorta perforations (immediate), mitral paravalvular leak (immediate in none, and late in 2/3). The aortic paravalvular leak closed at 3 months follow-up in one and small residual persisted after 1 month in another. COMPLICATIONS: Local site Hematoma was observed in 4 patients. 2 of them required post procedure transfusion for the same. Hematuria was observed in 2 of the 4 patients of mitral paravalvular leak and 2 patients of RSOV device closure. Hematuria subsided with conservative management before discharge from hospital in all the 4 cases. One patient with residual mitral regurgitation required surgical management for continuing hematuria, anemia and hyperbilirubenemia. There was one mortality observed on table during the attempted closure of a very large RSOV who presented to us in severe congestive heart failure and shock. On follow up ranging from 2 months to 6 years, all the patients are asymptomatic. There was no late complication related to device in any patient. CONCLUSION: It is feasible in selected nonseptal defects, which traditionally have been subjected to surgical interventions, to treat successfully, non surgically with the use of non prototype occluder devices without significant complications. Conventionally these devices have not been recommended for closure of nonseptal defects but show good early outcome. Adequate sample size with good follow up data is necessary before concluding that it can be safe alternative to surgery on long term.

Unusual cause of absent pulse in one arm: Isolated subclavian artery.

Isolated origin of the subclavian artery is a congenital anomaly of the aortic arch in which one subclavian artery originates from the homolateral pulmonary artery through the ductus arteriosus. Isolated right subclavian artery with left-sided aortic arch is extremely rare. We report a case of isolated right subclavian artery from the right pulmonary artery via a right-sided ductus arteriosus in a 4-year-old girl. The child presented with subclavian pulmonary steal with perfusion of the right arm and lung from the right vertebral and carotid arteries. She underwent successful surgical repair.

Scimitar syndrome: A novel management approach for palliation in a sick infant.

Scimitar syndrome is a rare congenital anomaly essentially requiring surgical management. Nonsurgical interventional treatments are rarely possible and are associated with poor outcome. We report a case of Scimitar syndrome with obstructed partial pulmonary venous confluence, large patent ductus arteriosus, and severe pulmonary artery hypertension, where a significant decrease in the pulmonary artery pressure was achieved by pulmonary venous stenting in combination with coiling of aberrant vascular supply to the sequestered lung segment. However, the child succumbed to lower respiratory tract infection. To the best of our knowledge, no such procedure in combination has been reported in the literature. .

Inferior-type caval vein defect--echocardiographic and surgical description of a large series of patients.

AIM: This study was carried out to define the anatomical criteria for the diagnosis of inferior-type caval vein defect and compare the echocardiographic findings with surgical findings. METHODS: The records of 19 patients - 13 male and six female patients in the age group of 18 months to 27 years, who were diagnosed as inferior-type caval vein defect with or without anomalous drainage of right pulmonary vein(s) on echocardiography - were retrospectively reviewed and compared with surgical findings. RESULTS: Surgical diagnosis of inferior-type caval vein defect was confirmed in 17 of the 19 patients. In two patients, the surgical diagnosis was that of a large fossa ovalis atrial septal defect - confluent defect and fossa ovalis atrial septal defect with deficient inferior rim in one patient each. Surgical diagnosis of anomalous drainage of pulmonary vein(s) was based on the course of the superior rim of the defect in relation to the pulmonary veins. Our echocardiographic impression of the pulmonary veins appearing in its normal position but showing abnormal drainage to right atrium was in agreement with the surgical notes. Discrepancy was found in the number of pulmonary veins draining anomalously. The discordance was related to overdiagnosis of anomalous drainage in all except one, that is, three out of four. In one, only the right lower pulmonary vein was diagnosed to be anomalous, whereas both right upper and lower pulmonary veins were found to be anomalous. CONCLUSIONS: Echocardiography provides definite diagnosis of inferior-type caval vein defect. Inferior caval vein straddling and an intact fossa ovalis are prerequisites for diagnosis. Anomalous pulmonary venous drainage of the right pulmonary veins is very common in our series, although accurate diagnosis of the number of pulmonary veins was not possible in all cases. Multiple views on transthoracic echocardiography starting from the subxiphoid views delineate the morphology accurately. Transoesophageal echocardiography is required only in patients in whom the windows, especially the subxiphoid, are not adequate.

Is it really PPHN? Think before starting sildenafil!

The use of sildenafil has become a common practice in neonatal intensive care unit on clinical ground, because opinion by Pediatric Cardiologist is usually not available especially in peripheral centers. We consider it essential to share our experience that severe pulmonary arterial hypertension can be due to some unusual hemodynamics or extremely rare structural causes which do not require pulmonary vasodilator therapy.

Hypocalcemic cardiomyopathy presenting as cardiogenic shock.

Hypocalcemia is a less known but treatable cause for dilated cardiomyopathy, leading to severe heart failure in children. Cardiogenic shock related to hypocalcemic cardiomyopathy is a rare event. We describe 5 infants presenting with cardiogenic shock over 3 years, who were found to have severe hypocalcemia as a sole cause of myocardial dysfunction. The patients responded to calcium and vitamin D supplementation promptly and left ventricular systolic function normalized within months of treatment. In any case of cardiogenic shock, hypocalcemia should be included in the differential diagnosis and must be investigated.

Intermediate and long-term followup of percutaneous device closure of fossa ovalis atrial septal defect by the Amplatzer septal occluder in a cohort of 529 patients.

OBJECTIVES: The aim of present study is to analyze the intermediate and long-term follow up results of percutaneous closure of fossa ovalis atrial septal defect (ASD) with Amplatzer septal occluder (ASO) in a large cohort of patients including children and adults. METHODS: Between May 1998 and July 2008, 529 patients (age group 2-77 years, median 28 years) underwent successful device closure with an ASO at single tertiary referral cardiac center in India.. This was out of an attempted 543 cases. The procedure was carried out in catheterization laboratory under transesophageal echocardiographic and fluoroscopy guidance. The mean size of ASD was 20 mm (7-40 mm) while size of septal occluder was 10-40 mm (mean 24 mm). Two devices were deployed in four patients. Three patients developed transitory pulmonary edema in immediate postprocedure period requiring ICU care for 48 hrs. All patients were advised for Aspirin (3-5 mg/kg, maximum 150 mg) once daily for 6 months. In patients with device 30 mm or larger, Clopidogril ( 75 mg once daily) was given for 3 months in addition to Aspirin. Clinical evaluation, echocardiogram were done on 3 months, 6 months and then at 1, 3, 5, 7 and 10 years of follow up. Transesophageal echocardiography (TEE) was performed in case of any doubt on clinical evaluation or on transthoracic echocardiography (n=10). RESULTS: Followup data is available for 496 patients (93.7%). Followup period is from 12 months to 120 months (median 56 months). On followup, device was in position in all patients, no residual shunt and no evidence of thrombosis. Interventricular septal motion normalized on day of procedure in 89% patients, in 6% over 3 months while flat septal motion persisted in 5% (n=25, all in age group > 40 years) of cases, though right ventricular dilatation persisted in 10% (n=50, age more than 40 years) of patients. Symptom-free survival was 96.7 % (480/496) in patients who came for followup. Only one 68 year old patient with preexistent tricuspid regurgitation developed congestive heart failure, and one patient (58 years old) had a history of hemiparesis after 1 year of device on telephonic interview. Ten patients were in atrial fibrillation (AF) before the procedure and remained in AF on followup. CONCLUSIONS: Our study showed that percutaneous closure of fossa ovalis ASD is a safe and effective procedure on intermediate and long-term followup in both the children as well as adults.both. Technical factors during the procedure and proper follow up are important. Our single centre intermediate and long term experience in a large number of patients support the use of device closure as an alternative to surgery.

Nonsurgical management of a congenital aortocaval fistula from right subclavian artery to superior vena cava along with SVC obstruction.

Congenital arteriovenous malformations between the subclavian artery and the superior vena cava (SVC) is a very rare anomaly scantily described in the literature. We describe this anomaly in a 4-month-old infant who presented with congestive heart failure. He was diagnosed to have a right subclavian artery to SVC fistula associated with SVC stenosis. The child successfully underwent device (vascular plug) closure of the fistula and SVC stenting. Six months postprocedure, child is doing well, with complete resolution of symptoms.

Myocardial dysfunction due to hypocalcemia.

Hypocalcemia is a curable cause of myocardial dysfunction and clinical congestive cardiac failure, with only stray reports available in literature. We describe 15 infants presenting with severe left ventricular dysfunction, who were found to have hypocalcemia with or without hypomagnesemia. Vitamin D deficiency was identified as the main cause of hypocalcemia. These children improved on supplementation of vitamin D and calcium.

Ductal aneurysm masquerading as nonresolving pneumonia: A challenging differential!

We report here, the case of a six-and-a-half-month-old boy investigated for persistent respiratory distress and homogeneous opacity in the left upper lobe. Echocardiography revealed a giant ductal aneurysm compressing the left pulmonary artery and upper lobe division of the left bronchus. Computerized tomography angiogram delineated the exact anatomy and prompt surgical resection provided a successful cure to this lesser known entity.

Constriction of juxta-ductal aorta and rapid progression of obstruction in a newborn.

A 13-day-old baby girl presenting with features of congestive cardiac failure was found to have coarctation of the aorta (CoA) and patent ductus arteriosus (PDA) by echocardiography. Doppler spectral display revealed moderate CoA. Echocardiogram, 12 hours later, showed severe juxtaductal aortic coarctation with spontaneous closure of PDA. This case emphasises the need to keep a close watch on the progress of CoA in the neonatal period, even if the duct has narrowed to a small size thus demonstrating the role of constriction of juxtaductal aorta in pathogenesis of coaractation. Closure of even asmall PDA can cause acute progression CoA in the presence of posterior shelf.