Long-term results of percutaneous balloon valvuloplasty of congenital aortic stenosis in adolescents and young adults.

Balloon aortic valvuloplasty (BAV) is a well accepted modality of treatment in congenital aortic stenosis in all age groups. Although in infants and children it is the modality of choice, in adolescents and young adults, it is of debatable efficacy. AIM: To evaluate long-term results of aortic valvuloplasty particularly in adolescent and adults (>12 years) and compare the outcome in other age groups that are <1 year and between 1 are 11 years. SETTING: Tertiary referral center. PATIENTS: 165 consecutive patients treated at the median age of 9 years (1 day to 64 years). The follow-up was up to 14 years (median 3 years). The whole cohort was divided into 3 age-based subgroups: Group A (<1 year) n=45, Group B (1 year-11 years) n=52, and Group C (>12 years) n=68. The characteristics of each subgroup were mutually compared. INTERVENTION: Percutaneous balloon valvuloplasty with mean (SD) balloon to annulus ratio of 0.93. Main outcome measures were repeat BAV, significant aortic regurgitation (AR), and aortic valve replacement/repair. RESULTS: The incidence of significant AR from the whole cohort was 9.9% (8% moderate, 1.9% severe); n=16. Group A=significant AR 9.5% (7.1% moderate, 2.4% severe), Group B=significant AR 11.3% (9.4% moderate, 1.9% severe), and Group C=significant AR 9% (7.5% moderate, 1.5% severe); p value=0.99 (Group C vs Group A) and 0.92 (Group C vs Group B). Repeat BAV rate was 13.3% (n=22 out of 165 patients). Group A - n=5 (11.9%), Group B - n=10 (18.2%), and Group C - n=7 (10.3%). p Value=0.78 (C vs A) and 0.19 (C vs B). Surgery in follow-up was needed in n=4 (2.4%), none in Group A, 2 patients in Group B (3.6%), and 2 patients in group C (2.9%). Patients were followed up for a period of 14 years; Group A=up to 8 years, Group B=up to 13 years, and Group C=up to 14 years. Mean survival probability after the procedure was 8 years (Group A=6.5 years, Group B=8.1 years, and Group C=9.9 years), and p value=0.49 (A vs B), 0.23 (B vs C), and 0.4 (A vs C). CONCLUSION: There is no statistical difference in the long-term outcome in the adults and adolescents as compared to the children; thus BAV remains an obvious treatment of choice with good long-term outcome.

Off-pump midline repair of coarctation of aorta when associated with intracardiac defects: an alternate approach for single-stage correction.

OBJECTIVES: Strategies for the optimal surgical management of coarctation of aorta with associated intracardiac defects continue to be debated upon. We describe a previously unreported surgical technique for the management of this combination of defects and present our early results. METHODS: Thirty-one patients (median age: 73 days and median weight: 3.9 kg) underwent repair of coarctation of aorta with associated cardiac defects, in a single-stage, through a midline sternotomy. Twenty-eight patients had associated distal arch hypoplasia and 1 had a type I interruption. The coarctation and when necessary, the distal arch, was repaired prior to the institution of cardiopulmonary bypass. Cardiopulmonary bypass was subsequently instituted for the repair of the associated intracardiac defects. RESULTS: All patients had successful repair of coarctation and distal arch hypoplasia without the need for rescue cardiopulmonary bypass. In addition, 29 patients had closure of one or more ventricular septal defects, 3 patients had an arterial switch procedure and 1 had repair of double outlet right ventricle. Repair of supramitral ring and subaortic membrane was performed in another 3 patients. One patient underwent concomitant mitral valve repair. There was one in-hospital mortality (3.22%) due to fulminant sepsis. The median follow-up was 29.5 months (range: 1-58 months). There was no late mortality. Two patients required balloon dilatation for recurrent coarctation. Overall event-free survival rate was 88.5% at both 2 and 3 years of follow-up after surgery. However, intervention-free survival at 2 and 3 years of follow-up was 91.7% among the survivors. CONCLUSION: Repair of coarctation and distal arch hypoplasia or type I arch interruption is feasible prior to the institution of cardiopulmonary bypass without the use of hypothermic circulatory arrest or regional cerebral perfusion, with acceptable and reproducible results.

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.

Double orifice tricuspid valve in an infant with partial atrioventricular septal defect.

Double orifice Tricuspid Valve is an extremely rare malformation with few case reports. We report such a case in an infant associated with atrioventricular septal defect.

Submitral aneurysm: An antenatal diagnosis.

We describe the echocardiographic features of submitral aneurysm in a 26 week old fetus. The diagnosis was confirmed on neonatal echocardiography.

Unusual systemic venous Collateral channels to left atrium causing desaturation after Fontan operation closed percutaneously.

We present an unusual cause of progressive cyanosis in a child appearing 2 years after successful Fontan surgery for tetralogy of Fallot with hypoplastic right ventricle. The cause of cyanosis was identified as one large venous channel draining into the left atrium. The channel was closed by Amplatzer vascular plug resulting in improvement of oxygen saturation.

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.

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.

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.

Coronary artery fistulas in pulmonary atresia and ventricular septal defect.

Pulmonary atresia with ventricular septal defect is an anomaly with highly variable anatomy. Rarely, a coronary artery-to-pulmonary artery fistula may contribute to pulmonary blood flow. Since 1996, we have treated 4 patients with coronary-pulmonary fistula associated with pulmonary atresia and ventricular septal defect. Two fistulas originated from the left coronary, one from the right coronary, and one from a right-sided solitary coronary system. All terminated in the main pulmonary artery, which was adequate in all cases. The fistulas were managed by direct internal closure. Total intracardiac repair was then accomplished in all patients at the same sitting. There was one death. In children with favorable anatomy, direct closure of the fistula from the pulmonary artery is adequate and allows single-stage intracardiac repair.

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.

QSAR analysis of 1,3-diaryl-4,5,6,7-tetrahydro-2H-isoindole derivatives as selective COX-2 inhibitors.

Quantitative structure-activity relationship (QSAR) analysis was performed on a series of 1,3-diaryl-4,5,6,7-tetrahydro-2H-isoindole for their cyclooxygenase-2 (COX-2) inhibition. QSAR investigations were based on Hansch's extra thermodynamic multi-parameter approach and receptor surface analysis (RSA). QSAR investigations reveal that steric and electrostatic interactions are primarily responsible for COX-2 enzyme-ligand interaction. QSAR model derived from Hansch analysis demonstrated that COX-2 inhibitory activity is correlated with sum of atomic polarizability (Apol), number of hydrogen-bond donor groups (HBD), energy of the highest occupied molecular orbital (HOMO), desolvation free energy for water (F(H(2)O)) and fraction of areas of molecular shadow in the XY and ZX planes over area of enclosing rectangle (Sxyf and Sxzf) with r ranges 0.870-0.904. The best model was obtained from RSA model having r = 0.940 with good predictive ability (predicted compounds in training set and test set within +/- 1.0 unit of pIC(50)) and can be used in designing better selective COX-2 inhibitors among the congeners in future.