Rajnish Juneja

Univentricular repair: is routine fenestration justified?

BACKGROUND A decade after the introduction of baffle fenestration, the outcome of Fontan-type repair for hearts with a functional single ventricle finally looks promising. Our study was designed to assess the impact of fenestration on the outcome of univentricular repairs. METHODS From January 1988 to December 1997, 348 patients (104 with tricuspid atresia and 244 with other morphological diagnoses) underwent univentricular repair at our institute. Since 1994, routine fenestration of the atrial baffle was performed in all patients (n = 126). RESULTS The overall Fontan failure rate was 14% (50 of 348) and included 45 early deaths and five Fontan take downs. Absence of fenestration was the only and highly significant predictor of Fontan failure (risk ratio [RR] 3.3, 95% confidence interval [CI] 1.49 to 7.31, p = 0.002). Significant pleural effusion was seen in 27% of patients. Absence of fenestration of the atrial baffle (RR 3.97, 95% CI 2.17 to 7.26, p < 0.001) and aortic cross-clamp time more than 60 minutes (RR 2.15, 95% CI 1.3 to 3.5, p = 0.002) were found to be significant risk factors. The follow-up ranged from 6 to 120 months (mean 46.0 +/- 18.0 months). There were 12 late deaths and 5 patients were lost to follow-up. Actuarial survival (Kaplan Meier) at 90 months was 81% +/- 4%. Two hundred and fifty-eight patients (90%) were in New York Heart Association class I at their last follow-up visit. Oxygen saturation in the fenestrated group ranged from 85% to 94% (mean 89%). Thirty patients (26%) had spontaneous closure of the fenestration over a mean period of 34 months, and there has been no incidence of late systemic thromboembolism. In no instance has there been a need to close the fenestration. CONCLUSIONS Elective fenestration of the intraatrial baffle is associated with decreased Fontan failure rate and decreased occurrence of significant postoperative pleural effusions. Routine elective fenestration of the atrial baffle may, therefore, be justified in all univentricular repairs.

Gender differences in the utilisation of surgery for congenital heart disease in India

Background Corrective surgery for congenital heart disease may be life-saving, but its utilisation depends upon several social and economic factors. Girls with cardiac defects may not receive equitable care in India, but this has not been systematically studied. Methods In this prospective study, parents or guardians of 405 consecutive children aged up to 12 years (mean±SD age 3.43±3.44 years; 271 boys) who had been advised to undergo elective paediatric cardiac surgery were interviewed using a validated questionnaire. The status of the patients was reviewed after a year and the factors associated with non-compliance with treatment were analysed. In a qualitative sub-study the parents of 20 children who had not undergone surgery were interviewed. Qualitative data were analysed using an inductive analytical approach. Results Of the 405 patients studied, 44% (59/134) of girls had undergone surgery at 1 year compared with 70% (189/271) of boys (χ2=24.97; p<0.001). Independent predictors for non-compliance with surgery included female gender (OR 3.46, 95% CI −2.06 to 5.80; p<0.0001), lower socioeconomic classes (lower-middle: OR 18.62, 95% CI −2.14 to 161.8, p=0.008; upper-lower: OR 34.27, 95% CI −3.72 to 316.0, p=0.002) and higher cost of surgery (OR 1.92, 95% CI −1.06 to 3.47, p=0.03). In the in-depth interviews, apprehensions about future matrimonial prospects of girls and lack of social support emerged as the major factors responsible for delays in undergoing surgery. Conclusions Female gender is an important determinant of non-compliance with paediatric cardiac surgery. Deep-seated social factors underlie this gender bias.

Anatomic repair for congenitally corrected transposition of the great arteries

OBJECTIVE Anatomic repair is being actively evaluated as the preferred option for congenitally corrected transposition of the great arteries. We present our 13-year experience with this approach. METHODS Between May 1994 and September 2007, 68 patients with congenitally corrected transposition of the great arteries underwent anatomic repair. Thirty-one patients (group 1, mean age of 94.8 +/- 42.3 months) underwent a combined Rastelli and atrial switch operation. Thirty-seven patients (group 2, mean age of 36.1 +/- 46.9 months) underwent an arterial switch operation and atrial rerouting. Eight patients in group 2 had an intact ventricular septum. RESULTS Group 1 had 5 early deaths (17%) but no late deaths. Three patients underwent conduit revision at a mean follow-up of 62 months. Group 2 had 5 early deaths (13.5%). There were 4 late reoperations (2 pulmonary baffle revisions, 1 mitral valve replacement, and 1 permanent pacemaker implantation) and 4 late deaths (1 secondary to progressive left ventricular dysfunction, 2 secondary to uncontrolled atrial tachyarrhythmia, and 1 secondary to pulmonary hypertension and right ventricular failure). In group 2, 4 patients have a left ventricular ejection fraction less than 40%, 5 patients have moderate aortic incompetence, 5 patients have symptomatic tricuspid incompetence, 1 patient has tricuspid stenosis, 1 patient has superior cava obstruction, and 3 patients are receiving antiarrhythmic therapy. CONCLUSION The occurrence of left ventricular dysfunction indicate that anatomic repair in the arterial switch group is still fraught with imperfections. The Rastelli group required conduit revisions but has otherwise performed well.

Intrapericardial streptokinase in purulent pericarditis

Six consecutive children with proven purulent pericarditis were treated with pericardial irrigation with streptokinase. Mean (SD) 861 (678) ml (range 240–2000) of thick purulent fluid was drained, and five children had complete clearance of the pus within 3–8 days. One child developed intrapericardial haemorrhage with a submitral pseudoaneurysm and underwent patch closure of the neck of the aneurysm as well as anterior pericardiectomy. Follow up of 13 to 30 months revealed no pericardial constriction.

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 Ficks 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.

A Comparison of Lead Placement Through the Subclavian Vein Technique With Fluoroscopy-Guided Axillary Vein Technique for Permanent Pacemaker Insertion

BACKGROUND The intrathoracic subclavian venous technique for pacemaker implantation may be associated with serious complications. We describe an alternative technique for obtaining venous access for pacemaker implantation through axillary vein under fluoroscopic guidance and compare it with the conventional, subclavian approach. METHODS We conducted a single-centre, prospective, nonrandomized study. All adult patients with indication for permanent pacing who consented were recruited during a 3-year period. To access the axillary vein, we used the alternative technique with a new fluoroscopic landmark. The subclavian access was obtained as per the usual approach. RESULTS We studied 478 lead placements during 3 years; 315 lead placements through axillary venous technique (group 1) were compared with 163 lead placements through subclavian venous technique (group 2). Both routes had a high and comparable success rate, 98.09% in group 1 and 96.93% in group 2. The axillary approach was successful at the first attempt in 194 punctures (61.6%), as vs 60 in group 2 (36.8%) P < 0.0001. The average number of attempts in group 1 was 2.06 per patient and 2.56 in group 2 (P < 0.001). There were 3 (2.94%) pneumothoraxes in group 2 and none in group 1. During a mean follow-up period of 3.2 months in group1 and 3.7 months in group 2, 1 patient in group 2 had a lead fracture. CONCLUSIONS The fluoroscopically guided axillary venous approach for implanting permanent pacemakers is equivalent to the traditional anatomic landmark-guided intrathoracic subclavian approach and has fewer complications and shorter procedural time to access the vein.

Factors influencing late course of residual valvular and infundibular gradients following pulmonary valve balloon dilatation

BACKGROUND Though acute and follow-up benefits of pulmonary valve balloon dilatation (PVBD) for pulmonic valve stenosis are well known, the late course of residual gradients at individual valvular and infundibular levels is not well described. Furthermore, the factors influencing this late course have not been studied. MATERIALS AND METHODS We assessed the transpulmonary gradients by echo-Doppler in 96 patients (61 male, mean age 10.7 years) at a mean follow-up interval of 58.8+/-32.1 months (minimum 2 years) following PVBD. The patients were divided into three groups based on the residual gradients at valvular and infundibular levels immediately following PVBD as assessed by pull-back of an end-hole catheter across the right ventricular outflow tract: Group A with minor pressure gradients at both valvular and infundibular levels of less than 30 mmHg (n=60, 62.5%), Group B with predominantly infundibular gradients of more than 30 mmHg with a valvular gradient of less than 30 mmHg (n=27, 28.1%), and Group C with a residual valvular gradient of greater than 30 mmHg irrespective of the infundibular gradient (n=9, 9.4%). Demographic characteristics, hemodynamic parameters and procedural variables were correlated with the change in gradient at follow-up (late fall) and with long-term results. RESULTS The mean follow-up trans-pulmonary gradient was 20+/-14 mmHg which was significantly lower than that immediately post-PVBD (43+/-32 mmHg), P<0.001. The late fall (mean 24+/-29, range -55 to 110 mmHg) varied widely depending upon the acute result group: patients in Groups A and B showed significant late fall of 9+/-12 mmHg (P<0.05 for follow-up gradient compared to that following PVBD) and 58+/-31 mmHg (P<0.0001), respectively, while patients in Group C showed an insignificant late fall of 14+/-37 mmHg (P=0.21). In particular, each one of the patients in Group B showed decrease in trans-pulmonary gradients. On multivariate analysis, the extent of infundibular gradient emerged as the most important predictor of late fall (coefficient of determination 75%, P<0.0001). Patients who underwent PVBD at less than 2 years of age had a significantly greater late fall (41+/-33 mmHg) as compared to older patients (22+/-25 mmHg), P<0.05. A sub-optimal long-term result (transpulmonary gradient >25 mmHg, n=24) was significantly related to older age (P<0.001), dysplastic valve morphology (P=0.002), greater baseline trans-pulmonary gradients (P<0.001) and higher post-PVBD gradients (P=0.04). CONCLUSIONS The long-term course of patients following PVBD depends upon the site and magnitude of the residual gradients. Even high residual infundibular gradients show marked reduction at follow-up, especially in infancy.

Hypocalcemic heart failure masquerading as dilated cardiomyopathy

Hypocalcemia is a rare, but reversible, cause of congestive heart failure. We report a 4-month-old boy diagnosed as dilated cardiomyopathy who had prolonged QOTC with low blood levels of calcium, normal phosphate, elevated alkaline phosphatase and findings suggestive of rickets. In view of non response to calcium and vitamin D3, a possible diagnosis of VDDR I (Vitamin Ddependent rickets) was made and he was treated with calcium and calcitriol. The serum calcium levels normalised within 10 days, along with resolution of the signs and symptoms of heart failure, near normal left ventricular function and normalisation of QOTC. Pediatricians should be aware of the association of hypocalcemia with cardiac dysfunction and should keep it as a possible reversible cause of heart failure in children.

Repair of total anomalous pulmonary venous connection in infancy: experience from a developing country

BACKGROUND Corrective surgery for total anomalous pulmonary venous connection in infancy still carries high morbidity and mortality rates in developing countries. The present study evaluates the factors responsible for it. METHODS Seventy-three infants were operated on for total anomalous pulmonary venous connection from January 1987 through October 1997. Age ranged from 5 days to 12 months (mean, 3.9+/-0.24 months), with 10 (13.7%) patients younger than 1 month old. Patient weight varied from 2.0 to 5.2 kg (mean, 3.7+/-0.27 kg). Most (90.5%) patients were small for their ages (< 50th percentile). Anomalous connection was supracardiac in 42 (57.5%), cardiac in 18 (24.7%), infracardiac in 4 (5.5%), and mixed in 9 (12.3%) patients. Thirty-five patients had obstructed drainage. Preoperatively, 30 patients received antibiotic therapy for respiratory tract infection, 3 patients had balloon atrial septostomy, and 4 patients required mechanical ventilation. Fifteen patients (20.5%) were operated on as an emergency procedure. For supracardiac and infracardiac connections, a posterior approach was used for anastomosis. In cardiac type, coronary sinus was unroofed and the resultant defect along with atrial septal defect was closed with a single patch. RESULTS The operative mortality rate was 23.3% (17 of 73). Pulmonary hypertensive crisis was the cause of death in 10 patients. Emergency operation and weight less than the 25th percentile were the important risk factors for operative mortality. Young age (< 1 month) and type of drainage did not affect the mortality. Follow-up ranged from 1 to 108 months (mean, 56.4+/-26.0 months). There were two late deaths. The actuarial survival (Kaplan Meier) at 9 years was 72.87%+/-5.39%. CONCLUSION Failure of early recognition, and thus delayed referral, accounted for onset of cardiac cachexia, respiratory tract infection, and severe pulmonary hypertension, which had a major effect on unfavorable outcome.

Detection of coronary artery anomalies in tetralogy of Fallot using a specific angiographic protocol.

Patients with tetralogy of Fallot were studied with a prespecified angiographic protocol aimed to delineate the types and incidence of coronary artery anomalies and to look for any correlation with other abnormalities detected by simultaneous detailed angiography. The incidence of coronary anomalies was found to be 7.0%; no association was found with these anomalies with any of the other abnormalities detected.