Anomalous systemic and pulmonary veins-An unusual coexistence.

A 12-year-old child presented with recurrent respiratory infections and was diagnosed with Scimitar syndrome. Drainage of IVC and course of aberrant arterial supply from aorta were not clear by echocardiogram, and hence, additional imaging was planned. CT unraveled the presence of a rare combination of anomalous pulmonary venous connection (APVC) to IVC, with near-atresia/severe stenosis of IVC, superior to the drainage of scimitar vein. There was a prominent azygos vein with preferential contrast opacification on lower limb injection.

Long-term outcome of permanent epicardial pacemaker implantation in neonates: Experience from an Indian center.

Introduction: Permanent pacemaker implantation (PPI) in neonates is challenging with respect to indications, device selection, implantation technique, and long-term outcomes. Complex anatomy, the need for long-term pacing with high rates, and a problematic postoperative period are the major problems. Methods: We prospectively followed up 22 newborns who underwent PPI below 28 days of life at our institute. Results: The median age at implantation was 2 days (interquartile range 1-9 days), and 9% were born preterm. The average heart rate before implantation was 46.4 ± 7.2 bpm. Maternal lupus antibodies were positive in 8 (36.4%) neonates, whereas 11 (50.0%) had associated congenital heart disease. Nineteen neonates underwent single chamber (VVI) and three underwent dual chamber (DDD) pacemaker implantation. Over a median follow-up of 46 months (range 2-123 months), the average ventricular pacing percentage was 87.5 ± 24.9%, with a stable pacing threshold. Seven children underwent pulse generator replacement due to battery depletion at a median age of 47 months. Pacing-induced ventricular dysfunction was seen in five children at a median age of 23.6 months, and two underwent upgradation to cardiac resynchronization therapy. Overall mortality was 13.6%, all due to tissue hypoperfusion and lactic acidosis in the postimplantation period. Conclusions: PPI in neonates has a favorable outcome with excellent lead survival. Overall mortality is 13.6%, which is predominantly in the postimplantation period and related to myocardial dysfunction.

Atrial septal defect occluder device embolization: Experience of a tertiary care cardiac center.

Background: Percutaneous device closure of atrial septal defect (ASD) has become an increasingly popular procedure as it offers several advantages. However, it is associated with infrequent, but life-threatening complications such as device embolization. Objective: To analyze the risk factors, common sites of embolization, associated complications, timing of embolization, and the treatment executed. Settings and Design: A retrospective study was performed at a tertiary referral center for cardiac services. Material and Methods: Pre-procedure, intra-procedure, and post-procedure data of patients whose ASD device embolized was collected retrospectively and analyzed for risk factors, common sites of embolization, associated complications, timing of embolization, and the treatment executed. Results: Thirty devices were embolized, out of which 13 were retrieved percutaneously in the Catheter laboratory, whereas 17 patients underwent surgery. Fourteen patients had an unfavorable septal morphology for device closure. Ten devices were embolized in the catheter laboratory, five in the intensive care unit, and two in the ward. The devices were embolized to almost all chambers of the heart and great vessels. One patient had an inferior vena cava rim tear while attempting percutaneous retrieval. One patient required a short period of total circulatory arrest (TCA) for retrieval of the device from ascending aorta, while another required a lateral position for retrieval from descending aorta. One patient required re-exploration for bleeding, while another had an air embolism and succumbed. Conclusions: Once embolization occurs, the risks associated increase manifold. Most of the surgical extractions are uneventful; however, there could be certain complications that may need repair of valvular apparatus, the institution of TCA, or the need for the lateral position. Air embolization though very rare can occur which could be fatal.

Usefulness of Doppler derived end diastolic flow gradient across the patent ductus arteriosus in selecting coils for ductal occlusion.

Transcatheter closure of patent ductus arteriosus (PDA) with coils is accepted as an alternative to surgical ligation. We evaluated whether flow gradient across PDA, obtained by Doppler echocardiography, can aid in selecting coils for percutaneous ductal occlusion. 79 consecutive patients with PDA, who underwent successful percutaneous coil occlusion were retrospectively reviewed. Patients with other structural heart disease and pulmonary hypertension with right-to-left shunt were excluded. Echocardiogram and cardiac catheterization were done in all patients. Gianturco (Occluding Spring Emboli; Cook, Bloomington, IN) non-detachable coils of 0.038 and 0.052-inch core sizes were used for ductal occlusion. Trough diastolic gradient was correlated with the size and the number of coils used. Mean age was 8.6 years (range 1.3 to 27 years); 24 males and 55 females; PDA diameter ranged from 1.3 to 4.5 mm. Number of coils used varied from 1 to 4. Echocardiography measured PDA size was 2.5 ± 0.6 mm and significantly differed from angiographically measured size 2.9 ± 0.6 mm (P = 0.05). End diastolic gradient below 38 mmHg predicted use of multiple coils or coils with larger surface area. End diastolic gradient correlated inversely with total surface area of the coils, which indirectly predicted size and number of coils. Thus, the prediction of the size and the number of coils for PDA occlusion can be assisted by the trough diastolic gradients of PDA.

Left ventricular noncompaction in primary systemic carnitine deficiency: A rare association.

Left ventricular noncompaction (LVNC) is a rare phenotype of dilated cardiomyopathy. We report a child with primary systemic carnitine deficiency having associated LVNC.

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.

Type A aortic dissection with partial ostial occlusion of left main coronary artery.

A 48-year-old hypertensive male presented with acute retrosternal pain and aortic regurgitation. The electrocardiogram showed ST-segment depression with T-wave inversion in anterolateral leads. Transesophageal echocardiography in long axis view of aorta revealed a spiral intimal flap in ascending aorta extending to the arch, diagnostic of Type A aortic dissection. The short axis view of the aorta showed partial obstruction of the left main coronary artery (LMCA) by the intimal flap with turbulent flow at its ostium. An emergency repair of aortic dissection with reconstruction of aortic wall was done. Postoperative period and ECG were normal. At 12-months of follow up, patient was doing well.

Efficacy and safety of combination of extended release niacin and atorvastatin in patients with low levels of high density lipoprotein cholesterol.

OBJECTIVE: We investigated the safety and efficacy of combination therapy of extended release (ER) niacin and atorvastatin in patients with low HDL-C and compared the results with atorvastatin monotherapy. METHODS: This open label study recruited consecutive men and women who had coronary artery disease with HDL-C levels <35 mg/dL. These patients were already on atorvastatin therapy targeted to lower low density lipoprotein cholesterol (LDL-C), for a minimum period of 6 months. Group 1, n = 104 (mean age 52.7 years) received ER niacin in addition to atorvastatin and group 2 (n = 106) continued on atorvastatin (mean age 52.3 years). ER niacin dose was built up to a maximum of 1.5 g and atorvastatin dose titrated according to LDL levels in both the groups. The lipoprotein levels at baseline were similar (p = NS). RESULTS: At 9 +/- 1.8 months of follow-up, the mean dose of ER niacin was 1.3 g and atorvastatin 13.2 mg in group 1. In comparison, group 2 patients had mean atorvastatin dose of 15.9 mg. Patients in group 1 had significant elevation in HDL-C cholesterol (39.5 +/- 5.5 vs 35.7 +/- 4.5 mg/dL), reduction in total cholesterol (156.4 +/- 31 vs 164.5 +/- 39.3 mg/dL) and also LDL-C (88.9 +/- 28.3 vs 99.8 +/- 35.4 mg/dL) compared to group 2 (all p < 0.05). The magnitude of reduction in triglyceride levels was not significant between the groups (140.1 +/- 40.4 vs 145.2 +/- 46.5 mg/dL) (p = NS). No major adverse events or clinical myopathy occurred in either groups. Four patients (4%) discontinued ER niacin (2 due to gastro-intestinal symptoms and 2 due to worsening of diabetes). Flushing occurred in 3% patients, but none felt it to be troublesome. CONCLUSION: Adding ER niacin to atorvastatin exhibited beneficial effects on lipid profile with significant elevation of HDL-C cholesterol and further lowering of LDL-C compared to monotherapy. This treatment offered better targeted therapy and was well tolerated with proper monitoring in Indian patients.

Acute phase reactants predict mitral regurgitation following mitral valvuloplasty.

This report evaluated whether acute phase reactants can predict the development of mitral regurgitation following percutaneous mitral valvotomy. 58 patients who developed significant mitral regurgitation following valvotomy were retrospectively compared with 58 age, sex and procedure technique matched control patients, who had valvotomy without mitral regurgitation. ESR and total leucocyte count were significantly higher in the group who developed mitral regurgitation, than in the control group. Higher ESR and total leucocyte count may be indicative of ongoing low grade sub-clinical inflammatory process, which makes the valve tissue friable which can give way during balloon stretch and lead onto mitral regurgitation.

Comparison of percutaneous transmitral commissurotomy with Inoue balloon technique and metallic commissurotomy: immediate and short-term follow-up results of a randomized study.

BACKGROUND: The Inoue balloon technique for mitral commissurotomy is well established and carried out worldwide. Metallic commissurotomy is reported to be a cheaper and effective alternative to balloon mitral commissurotomy. METHODS: One hundred patients were randomized into 2 groups to undergo percutaneous transmitral commissurotomy (PTMC) by means of the Inoue balloon technique (IBMC, n = 49) or metallic commissurotomy (PMMC, n = 51). Patients were crossed over to the other technique when the initial technique was a failure. Success of valvotomy, procedure-related complications, and follow-up events of the 2 techniques were compared. RESULTS: Basal echocardiographic and hemodynamic data were similar in both groups. Procedural success was similar in both groups: 45 of 49 procedures (91.8%) in the IBMC group, compared with 46 of 51 procedures (90.18%) in the PMMC group (P = 1.0). Crossover was also comparable, with 1 occurring in the IBMC group, compared with 3 in the PMMC group. Complications such as cardiac tamponade and mitral regurgitation (requiring or not requiring mitral valve replacement) were similar in both groups, with 3 complications in the IBMC group, compared with 4 complications in the PMMC group (P =.29). After a follow-up period of approximately 4 months, both groups had similar event rates and comparable hemodynamic parameters (P = not significant). CONCLUSIONS: Both IBMC and PMMC are successful means of providing relief from severe mitral stenosis with a gain in valve area and reduction in transmitral gradient. Both techniques have similar procedural success, complication rates, and follow-up events.

Mitral valvotomy with the Inoue balloon in juvenile rheumatic mitral stenosis.

We conclude that balloon valvotomy of the mitral valve with the Inoue technique is a safe and effective procedure for treating juvenile rheumatic MS. The almost complete absence of iatrogenic atrial septal defect and the low incidence of significant mitral regurgitation is noteworthy.

Effect of age on atrial contribution to ventricular filling after balloon mitral valvuloplasty in mitral stenosis.

OBJECTIVE: Ventricular filling takes place during the conduit and pump functions of the atrium. While studying whether relief of mitral valve obstruction improves atrial filling, the effect of age on atrial contribution to ventricular filling was studied before and after balloon mitral valvuloplasty (BMV) and on follow-up at 1 year. METHODS: Patients with mitral stenosis (MS) and sinus rhythm (n = 59) were divided into group I (< 18 years, n = 13), group II (< 30 years, n = 29) and group III (> 30 years, n = 17). Two-dimensional mitral valve area (MVA in cm2), transmitral mean gradient (MG in mm Hg), velocity time integral (VTI in cm) of mitral valve flow, VTI contributed by atrial systole (A-VTI), difference between total VTI and A-VTI (E-VTI), percentage contribution of A-VTI to the total VTI (A-%) and difference between A-% before and after BMV (delta-A-%) were noted. Follow-up data was obtained at 1 year. The change in A-% at follow-up (A-%-FU) was calculated as the difference between A-% before BMV and A-% at follow-up. RESULTS: There was a similar increase in MVA with a reduction in MG among the three groups. Among the three groups, total VTI and E-VTI before and after BMV were similar. Before BMV, in all the groups, A-VTI and A-% were similar. After BMV, there was increase in A-VTI and A-% in all the groups with a trend to be more in younger patients. A-VTI was significantly higher in group I only. But E-VTI had decreased significantly in all groups and tended to be less in younger patients. In younger patients, delta-A-% after BMV was significantly higher (13.2 +/- 7.6, 7.9 +/- 5.1 and 6.5 +/- 4.5, respectively, in groups I, II and III; p < 0.01). Correlation coefficient of age against delta-A-% was -0.55 (p < 0.01). Correlation coefficients of delta-A-% against post-BMV-MVA and MG were not good. At follow-up of 11.3+/-1.2 months, changes achieved in total VTI, A-VTI, E-VTI and A-% were maintained. Total VTI, A-VTI, E-VTI and A-% were similar at the time of follow-up on comparing the three groups. But younger patients had significantly higher A-%-FU (12.1 +/- 5.8, 9.4 +/- 4.6 and 7.3 +/- 3.1, respectively, in groups I, II and III; p < 0.01). CONCLUSIONS: Prior to BMV, there is an age related reduction in atrial contribution to ventricular filling that improves with relief of MS. Advancing age reduces the immediate and late recovery of atrial contribution after BMV. This may be due to increasing left atrial fibrosis with age that prevents an improvement in atrial pump function. The differential improvement in atrial function in younger patients warrants earlier intervention in MS to achieve better recovery of atrial function.

Congenital bilateral aplasia of external iliac arteries.

A child with bilateral aplasia of external iliac arteries with normal internal iliac arteries, demonstrated by vascular Doppler and digital subtraction angiography is presented. Popliteal artery is reformed by collaterals. This anomaly is extremely rare.

Natural history and predictors of moderate mitral regurgitation following balloon mitral valvuloplasty using Inoue balloon.

BACKGROUND: We studied the predictors and natural history of moderate mitral regurgitation following valvuloplasty using Inoue balloon since it has not been well documented in a large series. METHODS: Balloon mitral valvuloplasty was performed in 590 consecutive patients with severe mitral stenosis with mitral regurgitation of mild or lesser grade. Echocardiography and haemodynamics of patients who developed moderate mitral regurgitation were compared with those who did not. They were followed-up. Factors that predicted the development of moderate regurgitation were studied. RESULTS: 21 patients (3.5%) developed moderate regurgitation (identified by auscultation, haemodynamics, angiography and colour flow mapping). They were managed conservatively. At 3 months, regurgitation decreased in severity to mild grade in 12 patients. At 1 year, it was trivial in 5, mild in 11 and remained moderate in 5. There was progressive symptomatic improvement. No clinical, echocardiographic, hemodynamic or procedural variables could predict the development of moderate mitral regurgitation. CONCLUSIONS: Patients with moderate regurgitation after mitral valvuloplasty show gradual improvement in regurgitation and symptoms. There were no factors-clinical, echocardiographic, hemodynamic or procedural-that predicted the occurrence of moderate MR after BMV.

Absent pulmonary valve and origin of left pulmonary artery from ascending aorta.

A rare combination of tetralogy of Fallot, absent pulmonary valve and absent left pulmonary artery is described. The clinical and angiographic profile and the impact on management are discussed. The embryology is outlined.