Atrial flutter: yet another cause of arterial desaturation in atrial septal defect.

Pulmonary hypertension is not the only cause of arterial desaturation in patients with atrial septal defect. Arterial desaturation can also occur with normal pulmonary artery pressure making it mandatory to understand the mechanism to avoid erroneous diagnosis. In this report, for the first time, we demonstrate atrial flutter as the cause of arterial desaturation in a patient with large atrial septal defect despite normal pulmonary artery pressure, which was normalised following successful radiofrequency ablation.

Characteristics of Children with Acute Rheumatic Carditis from a High-Incidence Region: Importance of Unexplained Worsening of Functional Class.

BACKGROUND: Acute rheumatic fever (ARF) and acute rheumatic carditis (ARC) continue to be a major public health problem in developing countries. OBJECTIVE: To study the characteristics of children with ARC being treated at a tertiary centre. METHODS AND RESULTS: We studied 126 children (mean age 10.4 ± 2.3 years, range 5-15 years, 60% males) diagnosed with ARC by treating cardiologists. Most had lower socio-economic status. Fifty of 126 (40%) presented with a first episode of ARC. Joint symptoms were present in 29% and fever in 25%. Only 2.4% had subcutaneous nodules and none had erythema marginatum or chorea. Fifty-one percent presented in NYHA class II and 29% in NYHA class III or IV. Tachycardia and heart failure were present in 53% and 21%, respectively. Recent worsening of NYHA class (dyspnoea) was the commonest feature (48%). Laboratory investigations showed raised antistreptolysin O titres (>333 units) in only 36.7% of patients. Raised C-reactive protein (CRP) was present in 70%, while raised erythrocyte sedimentation rate was found in only 37% of patients. On the basis of above findings, the modified Jones criteria (2015) for the diagnosis of ARF were satisfied only in 46% of children. Echocardiography showed mitral valve thickening in 77% and small nodules on the tip of the leaflets in 43% (27 and 8%, respectively for aortic valve). Left ventricular ejection fraction was <50% in only 3 patients. The dominant valve lesion was mitral regurgitation (MR) (present in 95% of patients; severe in 78%, moderate in 15%), while aortic regurgitation was present in 44% (severe in 14%). CONCLUSIONS: The criteria are often not satisfied by patients being treated for ARC. Recent unexplained worsening of dyspnoea, young age, significant MR, echocardiographic nodules, and elevated CRP are important indicators.

Right bundle branch block pattern after uncomplicated right ventricular outflow tract pacing in a patient with a left sided superior vena cava and corrected tetralogy of Fallot.

Usually an electrocardiogram after right ventricular (RV) pacing should yield left bundle branch block (LBBB) pattern. However, the presence of right bundle branch block (RBBB) pattern after pacemaker implantation should alert the physician to a malposition of lead. We report a case of 18-year-old female who underwent dual chamber pacemaker implantation and had RBBB pattern post implantation. Detailed evaluation revealed an uncomplicated right ventricular outflow tract pacing. The possible causes of this abnormal pattern after an uncomplicated RV pacing are also reviewed.

Aortopulmonary window: Morphology, diagnosis, and long-term results.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital heart defect. We reviewed our experience with this condition over the last two decades. METHODS: Between September 1993 and December 2013, 62 patients underwent surgery for APW. Depending on the associated lesions, they were divided into two groups: Simple (Group 1) or complex (Group 2). In the complex group, six patients had a ventricular septal defect, five patients had interrupted aortic arch, three patients had tetralogy of Fallot, two patients had double outlet right ventricle, and one patient had the right pulmonary artery arising from the ascending aorta. RESULTS: Mean age at repair was 21.6 ± 32.02 months (median = 6, range 0.1-144 months). By preoperative echocardiographic assessment 27 out of 62 patients had severe pulmonary artery hypertension (52% of the cohort). Patch repair of APW was performed using the sandwich method (transwindow) (n = 27; 43.5%), transaortic (n = 18; 29%), and transpulmonary artery (n = 5; 8.1%) approaches; 10 patients (16.1%) underwent double ligation and two (3.2%) underwent division and suturing. Overall hospital mortality in group 1 was 6.97% (3/43) and in group 2 it was 21% (4/19), p = 0.085. Mean hospital stay in group 1 was 6.9 ± 2.4 days (median = 7 days) and in group 2 was 12 ± 6.1 days (median = 13 days), p = 0.0001. Follow-up in group 1 was 1.6-9.8 years (median = 6 years); in group 2, it was 1.8-8.9 years (median = 6.5 years). There were no late deaths. Two patients needed reintervention for distortion of the right pulmonary artery origin. All patients were in New York Heart Association Class I/II at last follow up. CONCLUSION: There are multiple acceptable surgical strategies for the treatment of aortopulmonary window. Despite a relatively advanced age and substantial number of patients with severe pulmonary hypertension the outcomes can still be good. Associated anomalies complicate the repair. Patients in the complex group had a protracted hospital course and a higher early mortality but similar late survival.

Resection of subaortic membrane for discrete subaortic stenosis.

BACKGROUND: We reviewed the long-term results of surgery for discrete subaortic membrane (SubAM) from a single institute. METHODS: A retrospective review of medical records of all patients (n = 146) who underwent resection of a SubAM for discrete subaortic stenosis between 1990 and 2015 at the All India Institute of Medical Sciences, New Delhi, India was undertaken. RESULTS: Median age at surgery was 9.0 years (9 months-47 years). There was one early death. Preoperative peak left ventricular outflow tract (LVOT) Doppler gradient was 83.4 ± 26.2 mmHg (range: 34-169 mmHg). On preoperative echocardiography, aortic regurgitation (AR) was absent in 69 (47.3%), mild in 35 (24%), moderate in 30 (20.5%), and severe in 12 (8.2%). After surgery, the LVOT gradient was reduced to 15.1 ± 6.2 mmHg (P < 0.001). Fourteen patients (9.6%) who had residual/recurrent significant gradients are currently being followed-up or awaiting surgery. There was improvement in AR for operated patients with freedom from AR of 92.6 ± 0.03% at 15 years. Kaplan-Meier survival at 25 years was 93.0 ± 3.9% (95% confidence interval: 79.6, 97.7). Freedom from re-operation at 25 years was 96.9 ± 1.8%. CONCLUSIONS: Long-term results of surgery for discrete SubAM are good. Resection of the membrane along with septal myectomy decreases the risk of recurrence.

Tilted ECG - An unusual tilt test response.

Tilt testing is a commonly used diagnostic tool to evaluate syncope of undetermined etiology. We hereby describe a twelve year old male child who developed presyncope during provocative head up tilt testing along with ST segment elevation in cardiac monitor during presyncope. Patient underwent repeat head up tilt testing along with holter monitoring which showed a left bundle branch block escape rhythm. Hence development of ST elevation was due to the improper filter application in single lead ECG monitor. This case highlights the importance of filters in electrocardiographic monitoring.

Perforating the atretic pulmonary valve with CTO hardware: Technical aspects.

OBJECTIVES: To review the success and technical aspects of pulmonary valve (PV) perforation using chronic total occlusion (CTO) hardware in patients with pulmonary atresia and intact ventricular septum (PA-IVS). BACKGROUND: Interventional therapy is possible in selected patients with PA-IVS. Among the various interventional options available, radiofrequency and laser assisted perforation may be more successful, but require expertise and may be substantially costly. METHODS: We describe the technique of mechanical catheter PV perforation using currently available coronary hardware meant for coronary CTO in nine cases with PA-IVS. After complete echocardiographic evaluation and informed parental consent was obtained, patients were electively intubated, mechanically ventilated, adequately heparinized and were placed on intravenous prostaglandin infusion. Basic steps involved were-localizing the atretic segment and accomplishing coaxial alignment of catheters using biplane fluoroscopy, crossing the atretic segment with the soft end of perforating guidewire, stabilizing the assembly and performing graded balloon dilatation with the balloon size never exceeding 130% of pulmonary annulus diameter. For crossing the atretic PV, a retrograde approach was used in one patient where the antegrade approach was not possible. RESULTS: The procedure was successful in 8/9 cases (89%). Valve opening was achieved in all eight patients with immediate fall in right ventricular (RV) systolic pressures. One neonate died following surgery after catheter induced RV perforation. All surviving cases were discharged from the hospital in good general condition with no evidence of heart failure and a room air oxygen saturation of >85%. No patient required an additional pulmonary irrigation procedure. CONCLUSION: With appropriate patient and hardware selection, PV perforation using readily available coronary hardware is feasible in PA-IVS. © 2014 Wiley Periodicals, Inc.

Paced QRS duration predicts left ventricular function in patients with permanent pacemakers - One-year follow-up study using equilibrium radionuclide angiography (ERNA).

Permanent pacing, being non physiological, often results in ventricular dysfunction over time. Narrower paced QRS duration from pacing the right ventricular outflow tract septum, might result in relatively preserved ventricular function over long term follow up.

Pericardiectomy in children <15 years of age.

Between January, 2002 and December, 2011, 27 patients (19 boys) underwent pericardiectomy. The mean age was 9.3 ± 4.96 years (range 0.4 to 15 years) and the mean duration of symptoms was 16.9 ± 22.15 months. In all, 25 patients had dyspnoea; eight were in New York Heart Association (NYHA) class IV; six had bacterial pericarditis; and 18 were on anti-tuberculosis treatment, although only nine had records suggesting tuberculosis. There were nine patients who underwent pre-operative pigtail catheter drainage of pericardial fluid. Surgical procedures were complete pericardiectomy (n = 20), partial pericardiectomy (n = 6), and pleuropericardial window (n = 1).The mean pre-operative right atrial pressure was 20.4 ± 4.93 mmHg. There were six hospital deaths due to low cardiac output (n = 5) and arrhythmia (n = 1). The mean intensive care unit stay was 2.7 ± 1.2 days and mean post-operative stay was 9.9 days. The mean right atrial pressure dropped to 8.7 ± 1.15 mmHg. Adverse outcomes defined as death/prolonged intensive care unit stay, prolonged post-operative stay were not associated with sex, diagnosis of tuberculosis or pyopericardium, or the duration of symptoms or pre-operative right atrial pressure. Younger patients had prolonged intensive care unit stay (p = 0.03) but not increased mortality. Advanced NYHA class predicted death (p = 0.02). The mean follow-up was 23.1 ± 23.8 months. All except one survivor are in NYHA class I and off all cardiac medications. Despite adequate surgery, pericardiectomy in children is associated with a high mortality, which is related to delayed surgery and poor pre-operative general condition. No specific pre-operative variable other than worse pre-operative NYHA class is a predictor of survival. Therefore, early pericardiectomy should be undertaken in such patients.

Retrospective study of results of Kawashima procedure.

BACKGROUND: Patients with single ventricle physiology and an interrupted inferior caval vein undergo the Kawashima procedure. With increasing follow-up, pulmonary arteriovenous malformations may develop requiring hepatocardiac venous redirection. METHODS: Between January 2005 and December 2012, 15 patients underwent the Kawashima procedure. Preoperative and postoperative characteristics along with functional class, intra-operative details, post-operative course, saturations and haematocrit were recorded. RESULTS: Median age at operation was five years (range 1- 20 years). Five patients had antegrade pulmonary blood flow interrupted and 10 patients had open antegrade flow. There were no deaths. Mean saturation in postoperative period was 90.8 ±1.36%. Median duration of mechanical ventilation and inotropic support was six and 16hours respectively. Median duration of pleural drainage was six days. Median duration of pleural drainage in antegrade flow open and interrupted group was six days and four days respectively. The mean hospital stay was 10.5 days ±7.1 days. Mean follow up was 40.2 ±31 months (median-30 months). Nine patients (60%) were in New York Heart Association (NYHA) functional class 1 and five patients (33.3%) were in NYHA functional class 2 at last follow-up. Only one patient was in class 3 and needs completion Fontan. CONCLUSIONS: The Kawashima procedure can be safely performed with acceptable early results. Although studies have shown the risk of pulmonary arteriovenous malformations after Kawashima procedure in the mid and long-term, our findings do not support this. Kawashima procedure with open antegrade pulmonary blood flow as a definite intervention in such patients is debatable.

Inflammatory markers are elevated in Eisenmenger syndrome.

Inflammation may be an important contributing factor to the progression of Eisenmenger syndrome (ES). Markers of systemic inflammation in ES have not been systematically studied. Inflammatory markers including high-sensitivity C-reactive protein (hs-CRP), interleukin-2 (IL-2), IL-6, and interferon-γ (IFN-γ) were measured in 42 consecutive ES patients (mean age, 24.3 ± 10.6 years) compared with their levels in 22 healthy control subjects. The patients were followed up for a mean duration of 16.3 ± 13.7 months. The levels of inflammatory markers were correlated with clinical and hemodynamic variables at baseline and the outcomes of death, hospitalization, and worsening World Health Organization (WHO) functional class at follow-up evaluation. Compared with the control subjects, ES patients showed a significant elevation in hs-CRP (2.99 ± 3.5 vs 1.1 ± 0.9 mg/dl; p = 0.002) and IFN-γ (41.3 ± 43.6 vs 10.4 ± 6.9 pg/ml; p < 0.001) levels. The levels of IL-2 and IL-6 also were elevated but did not differ significantly from those in the control subjects. The patients with hs-CRP levels higher than 3 mg/dl were significantly older (28.9 ± 10.6 vs 21.5 ± 9.8 years) and had a significantly shorter 6-min walk distance (421.5 ± 133.2 vs 493.3 ± 74.8 m). The levels of inflammatory markers did not correlate with baseline parameters or clinical outcomes. To conclude, the levels of hs-CRP and IFN-γ are significantly elevated in ES. Elevated hs-CRP in ES was associated with older age and shorter 6-min walk distance, but the levels of inflammatory markers were not predictive of clinical events.

Percutaneous transvenous angioplasty of left innominate vein stenosis following right side permanent pacemaker implantation- a left femoral vein to left axillary vein approach.

Central venous stenosis after the insertion of a permanent pacemaker is a well recognized complication. This late complication is encountered when there is a need to change the pacemaker lead or extract it. We describe a young male who had such a complication after many years after right side pacemaker implantation. The lesion was managed percutaneously leading to placement of a new lead from the left side.

Effect of Yoga on Clinical Outcomes and Quality of Life in Patients With Vasovagal Syncope (LIVE-Yoga).

OBJECTIVES: This study aims to determine the impact of yoga as an adjunct to standard therapy versus standard therapy alone on the symptomatic burden in patients with recurrent vasovagal syncope (VVS). BACKGROUND: There is a significant reduction in the quality of life (QoL) of patients with recurrent VVS. Existing management therapies have been largely ineffective. Recent trials have demonstrated the efficacy of yoga in diseases with autonomic imbalance, suggesting its possible utility in VVS. METHODS: Patients with recurrent VVS were randomized to receive either a specialized yoga training program in addition to current guideline-based therapy (intervention arm, group 1) or current guideline-based therapy alone (control arm, group 2). The primary outcome was a composite of the number of episodes of syncope and presyncope at 12 months. Secondary outcomes included QoL assessment by World Health Organization Quality of Life Brief Field questionnaire (WHOQoL-BREF) scores and Syncope Functional Status Questionnaire scores at 12 months, head up tilt test, and heart rate variability at 6 weeks. RESULTS: A total of 55 patients underwent randomization. The mean number of syncopal or presyncopal events at 12 months was 0.7 ± 0.7 in the intervention arm compared to 2.52 ± 1.93 in the control arm (P < 0.01). In the intervention arm, 13 (43.3%) patients remained free of events versus 4 (16.0%) patients in the control arm (P = 0.02). QoL at 12 months showed significant improvement of all Syncope Functional Status Questionnaire scores and 2 domains of WHOQoL-BREF scores (P < 0.05). CONCLUSIONS: Yoga as adjunctive therapy is superior to standard therapy alone in reducing the symptomatic burden and improving QoL in patients with recurrent VVS.

Acute and short-term hemodynamic effects of metoprolol in Eisenmenger syndrome: a preliminary observational study.

BACKGROUND: Progressive heart failure and sudden cardiac death are the common causes of death in Eisenmenger syndrome. ß-Bockers may be useful in Eisenmenger syndrome, but the safety and efficacy are not proven. The objective of the study was to evaluate the hemodynamic effects and safety of metoprolol in Eisenmenger syndrome. METHODS: Fifteen patients of Eisenmenger syndrome with a mean age of 22.6 (±8.9) years were studied. Hemodynamic parameters were measured at baseline, after 15 mg of intravenous metoprolol and 6 weeks after oral metoprolol (25 mg/d for 2 weeks and 50 mg/d for 4 weeks). RESULTS: Intravenous metoprolol was well tolerated, although there was a significant decrease in pulmonary and systemic blood flows. The calculated pulmonary vascular resistance index (23.3 ± 8.6 to 27.4 ± 10.6 Wood U, P = .005) and systemic vascular resistance index (34.9 ± 9.9 to 41.9 ± 13.5 Wood U, P = .005) increased significantly. After 6 weeks of oral metoprolol, the pulmonary artery mean pressure declined significantly (79.9 ± 12.9 to 73.4 ± 14.0 mm Hg, P = .04), which was associated with a slight decrease in mean aortic pressures as compared with baseline. The 6-minutes walk distance increased (401.2 ± 99.9 to 462.5 ± 81.7 m, P = .005). CONCLUSIONS: Preliminary observations suggest that metoprolol is safe and well tolerated in selected patients with Eisenmenger syndrome. Acute hemodynamic worsening recovers in the short term, and the exercise capacity improves in most patients. Larger studies are warranted.

Successful use of quinine in the treatment of electrical storm in a child with Brugada syndrome.

A 10-year-old girl developed life-threatening recurrent polymorphic ventricular tachycardia following surgical closure of a simple secundum atrial septal defect. Post hoc analysis of a Holter recording suggested Brugada syndrome. After managing the acute phase, a dual chamber defibrillator was implanted. One week later she experienced VF storm, needing 96 appropriate shocks within a few hours. Quinidine, by virtue of its I(to) blocking property, is the only drug reported to be useful in managing VF storm in Brugada syndrome. Nonavailability of quinidine led us to try its diastereomer, intravenous quinine, which succeeded in controlling the ventricular tachycardia. Arrhythmia storm in the setting of ion channelopathy can be difficult to manage, and sometimes requires innovative therapies.

Predictors of embolic events in pediatric infective endocarditis.

BACKGROUND: Infective endocarditis is an uncommon disease in children, but is often associated with significant morbidity and mortality. Embolization of vegetation to systemic or pulmonary circulation is a major contributor to morbidity and mortality of infective endocarditis, its incidence in adults varies from 22% to 40%. Very little data exists on the incidence and predictors of embolisation in children. We analysed the clinical and echocardiographic data of 50 consecutive children admitted to our centre with infective endocarditis over a five year period to determine the predictors of embolization. METHODS: This was a retrospective study of children below 12 years of age, who were admitted in the ward with a diagnosis of infective endocarditis. Their demographic, clinical, hematological and echocardiographic data were analysed to look for predictors of embolic events. The details of treatment and course in the hospital were also recorded. RESULTS: The mean age was 6 +/- 3.6 years with a range from 4 months to 12 years. Congenital heart disease was the underlying diagnosis in 44 cases. Twenty three cases had undergone cardiac surgery earlier. Blood cultures were positive in only 19 cases (38%). Seven children grew fungus in their blood culture. Echocardiography revealed vegetation in all; the size of vegetation was > 10 mm in 18 cases and < 3 mm in 11. Clinically obvious embolisation occurred in 23 cases (46%), the size of vegetation was > 10 mm in 13. Embolic events were more common when vegetation was present in the right sided valves or chambers (16/24, 66%). Embolic events were acutely fatal in 7 cases, 6 of these had vegetations which were > 10 mm. Repeat echocardiography failed to show regression of vegetation size in 19/23 cases with embolic events, as against 15/27 without embolic events. A total of 11 patients died. Thirteen patients underwent surgery, 9 within two weeks of treatment. CONCLUSIONS: In our study, large vegetations (> 10 mm) at presentation and failure to diminish in size on treatment are predictors of embolisation. Vegetations on right side of the heart are more likely to embolise.

Ivabradine Versus Amiodarone in the Management of Postoperative Junctional Ectopic Tachycardia: A Randomized, Open-Label, Noninferiority Study.

OBJECTIVES: This study sought to compare the efficacy of ivabradine and amiodarone in the management of postoperative junctional ectopic tachycardia (JET) after cardiac surgery in children. BACKGROUND: JET is a serious arrhythmia occurring in children after cardiac surgery and requires aggressive management. Amiodarone has been conventionally used in its treatment. Recent studies have reported the utility of ivabradine in this regard. METHODS: In this open-label randomized controlled trial, 94 children (age ≤18 years) who developed postoperative JET were allocated to receive either amiodarone or ivabradine. The primary endpoint was restoration of normal sinus rhythm. RESULTS: Sinus rhythm was achieved in 43 out of the 46 patients (93.5%) in the amiodarone group and 46 out of the 48 patients (95.8%) in the ivabradine group (mean difference of treatment effect: 2.3%; 95% confidence interval: -6.7% to 11.5%). The median (interquartile range) time taken to achieve sinus rhythm conversion was similar in both the groups: 21.5 (17-30.2) hours versus 22 (13.4-38.5) hours (p = 0.36)]. The time taken to rate control of JET was significantly less in the amiodarone group: median 7.0 (5.5-9.5) hours versus 8.0 (5.8-10.8) hours (p = 0.02)]. No drug-related adverse events were observed in the ivabradine group. CONCLUSIONS: Oral ivabradine is not inferior to intravenous amiodarone in converting postoperative JET to sinus rhythm. There was no difference in time taken to sinus rhythm conversion between the groups, although the rate control was earlier in patients who received amiodarone. Monotherapy with ivabradine may be considered as an alternative to amiodarone in the management of postoperative JET. (Comparison of Two Drugs, Ivabradine and Amiodarone, in the Management of Junctional Ectopic Tachycardia, an Abnormality in Cardiac Rhythm in Patients Under 18 years Who Undergo Cardiac Surgery: CTRI/2018/08/015182).

Inhaled nitroglycerin versus inhaled milrinone in children with congenital heart disease suffering from pulmonary artery hypertension.

OBJECTIVE: The aim of the present study was to compare the acute effects of inhaled milrinone and inhaled nitroglycerin on pulmonary and systemic hemodynamics in children with acyanotic congenital heart disease (left-to-right shunt) and pulmonary artery hypertension. DESIGN: Randomized clinical trial. SETTING: Catheterization laboratory of a tertiary care hospital. PARTICIPANTS: Thirty-five children below the age of 12 years who were suffering from acyanotic congenital heart disease with left-to-right intracardiac shunt and pulmonary artery hypertension (mean PA pressure > 30 mmHg). INTERVENTION: Right-heart catheterization was done using an end-hole balloon wedge pressure catheter. Baseline pulmonary and systemic hemodynamic parameters were recorded for all patients while breathing room air. All patients then underwent pulmonary vasodilator testing with 100% oxygen. Following this, patients were randomized into two groups and received either inhaled milrinone (group M, n = 18) or inhaled nitroglycerin (group N, n = 17) in a 50% air-oxygen mixture. Oximetry data were used to calculate systemic and pulmonary cardiac output based on Fick's principle. RESULTS: Systolic, diastolic, and mean pulmonary artery pressures decreased significantly in both the groups after drug nebulization, while there were no significant changes in systemic pressures. The percentage decrease from baseline in systolic (5.2% v 8.6%, p = 0.43), diastolic (19.5% v 16.8%, p = 0.19) and mean (14.9% v14.5%, p = 0.29) pulmonary artery pressures were comparable in both groups. The pulmonary vascular resistance index (PVRI) decreased from 9.0 ± 3.9 to 2.9 ± 1.7 Wood Units (WU)/m(2) in group M (p < 0.001) and from 8.6 ± 3.8 to 3.2 ± 3.3 WU/m(2) in group N (p < 0.001). The fall in pulmonary artery pressures after drug nebulization in both groups was comparable to the fall seen with 100% oxygen. CONCLUSION: Both milrinone and nitroglycerin when given via the inhaled route significantly decrease systolic, diastolic and mean pulmonary artery pressures as well as PVRI without significant effects on systemic hemodynamics. Both the drugs given via inhaled route therefore can offer a good therapeutic choice and can help decrease the high inspired oxygen concentrations needed to treat pulmonary artery hypertensive episodes in perioperative settings.