Pancycle Aortic Incompetence and Double Alternans in Pediatric Heart Failure

A 12?year?old boy presented with bicuspid aortic valve, severe aortic regurgitation, and dilated dysfunctional left ventricle in heart failure. He underwent aortic valve replacement with a 23 mm TTK Chitra heart valve prosthesis (tilting disk). He was gradually weaned off milrinone and noradrenaline in the intensive care. Echocardiography showed severe left ventricular dysfunction with an ejection fraction of 24%. The radial pulse was regular and of normal volume but exactly half that of the heart rate. Evaluation of the rhythm and echocardiography revealed an interesting hemodynamic phenomenon with double alternans.

Programmed ventricular stimulation in structural heart disease: Implications of patterns of ventricular arrhythmias induced to long-term outcomes

Introduction: Currently available data gives some credence to utility of VT induction studies in patients with stable ischemic cardiomyopathy, there are some unresolved questions as to define sensitive threshold for low-risk and the prognostic relevance of ill sustained or non-specific tachycardia on induction study. We evaluated potential ability of VT inducibility to predict likelihood of SHD (Structural heart disease) patients for subsequent arrhythmic or adverse cardiac events. Material and Methods: All consecutive patients with syncope/documented arrhythmia who had VT induction done were included and patients with VT storm, ACS,uncontrolled HF were excluded. We studied in 4 groups-monomorphic VT, sustained polymorphicVT, ill sustainedVT/VF and no VT/VF induced. The primary-endpoints were e Sudden death, all-cause mortality and secondary-endpoints were e MACE (AICD shock, death,HF, recurrence of VT). We screened 411 patients and included 169 within inducible (n ¼ 79) and non-inducible group (n ¼ 90). Results: There were a higher number of patients with coronary artery disease, LV dysfunction, patients on amiodarone in inducible group and no difference in usage of beta-blockers. Recurrence of VT, composite of MACE was significantly higher in inducible group (p < 0.05). Mortality was not different in 3 groups compared with no VT/VF group. We found that monomorphic VT group had significantly higher MACE as compared to others and also predicted recurrence of VT and AICD shock and showed a trend towards significance for prediction of mortality. Inducible patients on AICD had mortality similar to noninducible group. Conclusion: Induction of monomorphicVT/polymorphicVT with 3extrastimuli is associated with a higher number of MACE events on follow up. Induction of monomorphicVT predicts recurrence of VT/ICD shock.

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.

Venogram-guided extrathoracic subclavian vein puncture.

BACKGROUND: Subclavian vein puncture is commonly performed to insert the pacing lead for permanent pacemaker implantation. Our aim was to study the safety and feasibility of venogram-guided extrathoracic subclavian vein puncture for permanent pacemaker lead insertion. METHODS AND RESULTS: Sixty patients (32 males, and 28 females) underwent permanent pacemaker lead insertion by extrathoracic subclavian vein puncture at our institute between March 2002 and December 2002. Fifteen patients underwent dual-chamber and 45 single-chamber pacemaker implantation. All the patients underwent extrathoracic subclavian vein puncture guided by venogram, except 1 who underwent dual-chamber pacemaker implantation in whom the ventricular lead insertion was via the cephalic vein on an elective basis. The procedure was successful in all the patients. Inadvertent subclavian artery puncture occurred in 2 patients without any complication. There was no incidence of pneumothorax, hemothorax or pacemaker site infection. CONCLUSIONS: Venogram-guided extrathoracic subclavian vein puncture is safe and successful. It may be adopted as one of the preferred approaches for permanent pacemaker lead insertion.

Long-term outcome of patients operated for large ventricular septal defects with increased pulmonary vascular resistance.

BACKGROUND: There is a paucity of data regarding the long-term outcome of patients operated for ventricular septal defect with severe pulmonary arterial hypertension and elevated pulmonary vascular resistance. METHODS AND RESULTS: We evaluated the long-term follow-up results of a selected cohort of patients with nonrestrictive ventricular septal defect and elevated pulmonary vascular resistance (>6 Wood units). Thirty-eight patients, median age 7.5 years (range 6 months-27 years), with nonrestrictive ventricular septal defect with severe pulmonary hypertension were operated between 1985 and 1996 at our institute. Preoperative pulmonary vascular resistance, ratio of pulmonary blood flow to systemic blood flow, and ratio of pulmonary vascular resistance to systemic vascular resistance were 7.63+/-1.8 Wood units, 1.9+/-0.48, and 0.41+/-0.12, respectively. The majority (68.4%) had perimembranous ventricular septal defect. Thirty patients (79%) had a good outcome and were asymptomatic at a mean follow-up of 8.7 years, with significant reduction in pulmonary artery pressures. Eight patients (21%) had a poor outcome, which included 5 immediate postoperative deaths, 1 late death and 2 surviving patients with persistent severe pulmonary arterial hypertension. There was no significant difference regarding hemodynamic parameters at baseline between those who had a good outcome and those who did not. Eleven patients with a preoperative pulmonary blood flow to systemic blood flow ratio of <2:1. who had a good outcome following surgery, underwent repeat catheterization at follow-up. There was a significant reduction in their mean pulmonary vascular resistance (8.03+/-1.4 v. 4.16+/-1.6 Wood units, p=0.001) and pulmonary vascular resistance to systemic vascular resistance ratio (0.41+/-0.12 v. 0.19+/-0.06, p=0.05). CONCLUSIONS: The late results of surgery on this selected group of patients with nonrestrictive ventricular septal defect with high pulmonary vascular resistance are encouraging. Operative correction of the ventricular septal defect should be actively considered in all children presenting with nonrestrictive ventricular septal defect with a significant left-to-right shunt, despite moderately elevated pulmonary vascular resistance. Even among older patients with ventricular septal defect and moderately elevated pulmonary vascular resistance, there is a specific group that does well after operation.

Supravalvar aortic stenosis: clinical and hemodynamic profile, and surgical outcome.

BACKGROUND: Supravalvar aortic stenosis is the rarest of left ventricular outflow obstructions. Data on this rare entity from India are scarce. METHODS AND RESULTS: We retrospectively analyzed the data of 15 patients (13 males, mean age 15.5+/-10.18 years) with a diagnosis of supravalvar aortic stenosis confirmed by cardiac catheterization. Five patients had morphological features of Williams' syndrome. One patient had diffuse while the rest had discrete type of supravalvar aortic stenosis. Five patients did not have any associated lesions. A 9-year-old male had an ascending aortic aneurysm, and 3 patients had associated peripheral pulmonary artery stenosis. One child had a subaortic ventricular septal defect, and another had severe mitral regurgitation. Twelve patients had electrocardiographic evidence of left ventricular hypertrophy. Three patients had mild aortic valvar stenosis while 2 had aortic regurgitation. Six patients had dilated coronary arteries. Two patients with supravalvar aortic gradients of 20 and 40 mmHg were kept on close follow-up. One patient was not willing to undergo surgery while the other is awaiting surgery. Eleven patients underwent surgical correction. Dacron or pericardial patch aortoplasty was done in all the patients. In addition, one patient each underwent pulmonary artery plasty, ventricular septal defect closure, repair of ascending aortic aneurysm, and mitral valve replacement. The patient with diffuse type of supravalvar aortic stenosis underwent augmentation aortoplasty. Two patients died perioperatively. One was lost to follow-up. Two had moderate residual gradients. The rest of the patients were in New York Heart Association functional class I on follow-up of 6.3+/-4.7 years. CONCLUSIONS: Repair of supravalvar aortic stenosis by single sinus aortoplasty is safe and produces good results.

Coexistence of congenital subaortic and sinus of valsalva aneurysms.

We report a rare association of an aneurysm of the sinus of Valsalva and a subaortic aneurysm. Congenital defective union of the aortic wall and ventricular muscle with the fibrous ring is probably the explanation for this condition.

Congenital coronary anomalies of origin and distribution in adults: a coronary arteriographic study.

BACKGROUND: Coronary anomalies should be recognized to avoid problems during coronary intervention and cardiac surgery. METHODS AND RESULTS: We retrospectively reviewed 7400 coronary angiograms to find out the pattern and incidence of coronary anomalies of origin and distribution. We excluded patients with congenital heart diseases, coronary artery fistulae and patients with separate origin of the conus artery. and found 34 cases (0.46%) (22 males), mean age 50.7 +/- 12 years with coronary anomalies. Six cases underwent angiography prior to valve replacement and the rest were part of the evaluation for atherosclerotic coronary artery disease. The most common anomaly was separate origins of the left anterior descending coronary artery and left circumflex coronary artery [n=12 (35.3%)]. The next most common anomalies were origins of the right coronary artery from the left coronary sinus [n=7 (20.6%)] and left circumflex artery from the right sinus [n=6 (20%)]. A single coronary artery was seen in 3 cases (8.8%) which included one case of postmyocardial infarction ventricular septal rupture with triple-vessel disease, and another with two small coronary fistulae. One case each of the following coronary anomalies was found: (i) double right coronary artery, (ii) left anterior descending coronary artery from the right coronary sinus, (iii) all three coronary arteries originating separately from the right sinus, and (iv) left main coronary artery from the right sinus. Of these 34 patients, 11 (32.4%) had significant atherosclerotic disease in the anomalous vessel. CONCLUSIONS: The incidence of primary coronary anomaly seems to be less than that in earlier reports, but the pattern of anomalies appears to be similar.