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.

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.

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.

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.

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.

Coarctation of aorta--intervention from neonates to adult life.

Coarctation of aorta is typically a discrete narrowing of the thoracic aorta just distal to the left subclavian artery. Managmnet of Coarctation of aorta depends upon age, clinical presentation, type of coarctation, associated arch hypoplasia, and associated heart defects as ventricular septal defect. In this article we are discussing management of coarctation of aorta from neonatal period to adult.

"Role of echocardiography in catheter intervention in congenital heart defects".

Echocardiography has evolved over last 3 decades from M-mode echocardiography, two-dimensional echocardiography, Dopplerimaging, three-dimensional echocardiography to intravascular /intracardiac echocardiography with its immense role in evaluation of a patient with congenital heart disease. The refinement in echocardiography imaging technique has made echocardiography as the main stay in diagnosis and decision making in patient with congenital heart defects (CHD). It is used fr defining anatomy, hemodyanamic assessment, guiding and immediate assessment of procedural results as well as detection of complication. In this review article, we are discussing the role of echocardiography (transthoracic/transesophageal) in catheter interventions in congenital heart defects.

Rare variants of total anomalous pulmonary venous connection to coronary sinus-echocardiographic recognition and surgical correction.

Total anomalous pulmonary venous connection (TAPVC) to the coronary sinus is a well-known entity but variations in connection sites are known to occur, the commonest among them is mixed connection. Here we describe two rare variants of TAPVC to coronary sinus. Group I (3 cases) in which there were dual sites of connection and group II (2 cases), TAPVC to coronary sinus was associated with a persistent left superior vena cava (LSVC). This was seen in total number of 45 cases of isolated TAPVC and 8 cases of TAPVC to coronary sinus between 2000 and December 2005 in our institute. All patients underwent surgical correction. In both of these groups, surgical correction may pose a challenge, which is discussed.

Giant congenital diverticulum of the right atrium.

Congenital diverticulum of heart is a rare entity, which may arise from the atria, atrial appendages, coronary sinus or the ventricles. A 3-year-old child presented with history of early fatigability for 6 months and recent upper respiratory tract infection. Chest X-ray and echocardiogram revealed marked right atrial enlargement. At surgery, a right atrial diverticulum was excised under cardiopulmonary bypass. Pathology revealed thickened endocardium with edema and myocardial fiber hypertrophy. Our experience with this rare congenital disease is presented along with a review of the literature.

Comparison between transcatheter closure and minimally invasive surgery for fossa ovalis atrial septal defect: a single institutional experience.

BACKGROUND: Although, conventional surgical closure of atrial septal defect (ASD) provides excellent results with very low mortality and morbidity, it leaves the scar of incision and postoperative pain. Newer treatment modalities like minimal invasive surgery and percutaneous closure are being increasingly used nowadays where available. AIM: To compare the patient population, success, safety, and efficacy of transcatheter closure of ASD (Group A) with that of minimally invasive surgery (Port Access) (Group B). METHODS: In this retrospective non-randomized study, a record of a total of 640 patients with diagnosis of ASD secundum between May 1997 and October 2006 were reviewed. A total of 470 out of 640 patients were selected for transcatheter closure (Group A) while 170 patients were taken for surgical closure by minimally invasive port access surgery (Group B). The safety and efficacy of two groups was evaluated on the basis of morbidity and mortality, duration of intensive care unit (ICU) stay, total duration of hospital stay, post-procedural complications, residual sequel at time of discharge, and residual flow across the ASD. RESULTS: Success rate in two groups was 97.1% and 99.4%, respectively and had no statistically significant difference. Similarly major complication rate also had no difference in statistical significance (1.8% and 2.9% for Group A and B, respectively). Group B patients had longer hospital stay. A small but significant number of patients were not found suitable for device closure. This number is likely to decrease as experience with technique increases. Port access surgery is currently not possible in small children (femoral artery diameter 35 mm) due to difficulty in cannulation. CONCLUSION: Percutaneous device closure of ASD can be offered as a treatment option in suitable patients. Port access is minimally invasive and an equally safe and effective alternative choice in ASDs with deficient rim in patient with appropriate age and weight.

Transcatheter closure of fossa ovalis atrial septal defect: a single institutional experience.

Transcatheter closure of the fossa ovalis atrial septal defect with adequate rims has become the procedure of choice in recent times. We report our experience with 439 patients who were selected for percutaneous device closure and 430 of whom underwent transcatheter closure of fossa ovalis atrial septal defects from May 1997 to May 2006. Device closure was not attempted in the remaining nine patients after initial evaluation due to high right atrial pressure following balloon occlusion (n=1 not parallel, high pulmonary vascular resistance (n=1), significant coronary artery disease (n=1), or the fact that the atrial septal defect was unsuitable for device closure (n=6). The anatomical size of the atrial septal defects ranged from 7-40 mm. Six patients underwent a combined procedure at the time of the atrial septal defect device closure (balloon pulmonary valvotomy in 4 cases, balloon pulmonary valvotomy and patent ductus arteriosus device closure in 1, and pulmonary ductus arteriosus coil closure in 1). The patients were in the age group of 2-77 years, the mean age being 20 years. Major complications occurred in seven cases. In 4 patients, there was device embolization immediately after deployment; there was left atrial appendage perforation, in one patient leading to cardiac tamponade during deployment of the device, and development of pulmonary edema in two patients. The cases in which device embolization took place underwent conventional surgery for atrial septal defect patch closure, while the patient with left atrial appendage perforation underwent emergency surgery (suturing of the left atrial appendage). Both patients with pulmonary edema were managed conservatively. The immediate success rate of device closure was 96.9%. All but one patient with multiple defects had no residual shunt at follow-up. On three months' to nine years' follow-up, the device remained stable, with no significant residual shunt and no history of thrombus formation on transthoracic echocardiography.

Total anomalous pulmonary venous connection to azygos vein: echocardiographic recognition of a rare variant.

Total anomalous pulmonary venous connection to the azygos vein is a rare congenital malformation in which all the pulmonary veins drain anomalously to the azygous vein. Among 55 consecutive patients diagnosed as isolated total anomalous pulmonary venous connection by echocardiography at our institute between 1995 and January 2005, this anomaly was diagnosed in two patients. The malformation was diagnosed correctly in both the patients by echocardiography and the findings were confirmed on surgery. The importance of differentiating this condition from total anomalous pulmonary venous connection to superior vena cava by echocardiography and the differential diagnosis are discussed here.

Aortico-left ventricular tunnel: echocardiographic recognition.

Aortico-left ventricular tunnel (ALVT) is a rare congenital malformation characterized by an abnormal communication between aorta and left ventricular cavity and the child presents with features of congestive heart failure in early infancy. We report the case of a 7-day-old baby who was diagnosed as a case of ALVT by echocardiography. The child was operated successfully and echocardiographic findings were confirmed.

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.

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.