Shyam Sunder Kothari

Comparison of Echo and MRI in the Imaging Evaluation of Intracardiac Masses

We compared the efficacy of echocardiography (ECHO) and magnetic resonance imaging (MRI) for evaluating intracardiac masses. Over an 8-yr period, 28 patients, 21 males, 7 females, 16 days–60 years of age (mean 25 years) with a suspected intracardiac mass on ECHO (transthoracic in all; transesophageal in 9) underwent an MRI examination. Five patients had a contrast-enhanced MRI. ECHO and MRI were compared with respect to their technical adequacy, ability to detect and suggest the likely etiology of the mass, and provide additional information (masses not seen with the other technique, inflow or outflow obstruction, and intramural component of an intracavitary mass). With MRI, the image morphology (including signal intensity changes on the various sequences) and extracardiac manifestations were also evaluated. The diagnosis was confirmed by histopathology in 18, surgical inspection in 4, by follow- up imaging on conservative management in 5, and by typical extracardiac manifestations of the disease in 1 patient.Fifteen (54%) patients had tumors (benign 12, malignant 3), 5 had a thrombus or hematoma, and 4 each had infective or vascular lesions. Thirty-four masses (13 in ventricle, 11 septal, 7 atrial, 2 on valve and 1 in pulmonary artery) were seen on MRI, 28 of which were detected by ECHO. Transthoracic ECHO (TTE) and MRI were technically optimal in 82% and 100% of cases, respectively. Nine patients needed an additional transesophageal ECHO (TEE). Overall, MRI showed a mass in all patients, whereas ECHO missed it in 2 cases. In cases with a mass on both modalities, MRI detected 4 additional masses not seen on ECHO. MRI suggested the etiology in 21 (75%) cases, while the same was possible with ECHO (TTE and TEE) in 8 (29%) cases. Intramural component, extension into the inflow or outflow, outflow tract obstruction, and associated pericardial or extracardiac masses were better depicted on MRI. We conclude that MRI is advantageous over a combination of TTE and TEE for the detection and complete morphological and functional evaluation (hemodynamic effects) of cardiac masses.

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.

Pulmonary Hypertension Associated With Congenital Heart Disease: Pulmonary Vascular Disease: The Global Perspective

The incidence of congenital heart disease is approximately 8/1,000 live births and appears to be constant around the world. The currently accepted paradigm for the development of pulmonary vascular disease associated with congenital heart disease maintains that increased pulmonary blood flow and pressure trigger unfavorable vascular remodeling. Endothelial cell dysfunction, abnormal shear stress, circumferential wall stretch, and an imbalance in vasoactive mediators conspire to promote vasoconstriction, inflammation, thrombosis, cell proliferation, impaired apoptosis, and fibrosis. We estimate that worldwide 3 million children are at risk for the development of pulmonary vascular disease due to congenital heart disease. The majority of children at risk globally will have a reparable heart defect, such as an isolated atrial septal or ventricular septal defect or patent ductus arteriosus. Cardiac repair in the first 2 years of life would prevent the development of Eisenmenger syndrome, the most advanced form of pulmonary vascular disease secondary to congenital heart disease. Worldwide, only a small fraction of those at risk are offered surgical repair. Thus, access to timely medical care would eliminate the vast majority of suffering, disability, and death from Eisenmenger syndrome. Globally, pulmonary vascular disease associated with congenital heart disease may be the most preventable cause of pulmonary artery hypertension and related mortality and morbidity.

Predictors of arterial thrombosis after diagnostic cardiac catheterization in infants and children randomized to two heparin dosages.

Arterial thrombosis is the most frequent major complication of percutaneous arterial catheterization in children. We prospectively studied the effect of randomized dosage of heparin, 50 IU/kg-group I and 100 IU/kg-group II, on the incidence of arterial thrombosis in 366 children and analysed the various factors which may influence the occurrence of this complication. The age of patients ranged from 17 d to 11 yr (mean age 39.5 +/- 40.9 mo) and mean weight was 11.2 +/- 7.8 kg (range 3 to 39 kg). The incidence of arterial thrombosis was 9.8% in group I and 9.3% in group II (P = NS). There was no statistical difference in precatheterization and procedure variables in the two groups and also in the group with absent pulse (n = 35) to the group with pulse present post cath (n = 331). There were 24.9% infants in our study and 14.3% of these had arterial thrombosis. The loss of pulse was more often seen with more number of attempts at arterial puncture (P < 0.001), absence of back bleed at the end of the procedure (P < 0.001), and increased duration of catheterization (P < 0.01). Use of larger sheath size in a given weight and body surface area of children increased incidence of arterial thrombosis. The administration of heparin 50 IU/kg was equally efficacious to heparin 100 IU/kg. Of the patients with arterial thrombosis, 23 responded with intravenous heparin and 12 needed streptokinase. There was no bleeding or haematoma. Thus our study shows that less attempt for arterial puncture, use of smaller sheath size, maintaining shortest procedure time and ensuring back bleed minimises incidence of arterial thrombosis post catheterization.

Double switch for congenitally corrected transposition of the great arteries.

OBJECTIVE To evaluate the early and medium term results of operations for congenitally corrected transposition of the great arteries (CCTGA) wherein the left ventricle becomes the systemic ventricle. METHODS Fourteen patients with CCTGA who underwent anatomic repair from 1994 to 1998 were placed in one of two groups: those without pulmonic stenosis (PS) (N = 7) and those with PS (N = 7). Main associations in the group without PS were: severe left atrioventricular (AV) valve regurgitation (N = 4), ventricular septal defect (VSD) (N = 4), criss-cross AV connection (N = 1), and hypoplasia of the morphologic RV (N = 1). Main associations in the group with PS were: VSD (N = 7), anomalous pulmonary venous drainage (N = 2) and major aorto-pulmonary collaterals (N = 1). All patients in the group without PS underwent an arterial switch operation and all except one had a modified senning repair for atrial rerouting for anatomic correction. All seven patients with PS underwent a Rastelli repair and all except one needed a modified senning repair. RESULTS Early survivorship in both groups was 6/7. Follow-up ranges from 1 month to 48 months. The mean left ventricular ejection fraction (LVEF) in the arterial switch and senning patients was 65% and in the Rastelli and atrial rerouting was 52%. None of the survivors except one have any significant mitral regurgitation (MR). The one exception resulted from iatrogenic damage to a chorda during VSD closure. All except two patients are class I symptomatic and all are in normal sinus rhythm. CONCLUSION Satisfactory early and mid term results of anatomic repair support the double switch option as the procedure of choice for patients of CCTGA amenable to biventricular repair.

One and a half ventricle repair with pulsatile bidirectional Glenn: results and guidelines for patient selection

BACKGROUND The guidelines for performing a one and a half ventricle repair with pulsatile bidirectional Glenn remains controversial. This retrospective report summarizes the experience of a single institution, with an attempt at providing an answer. METHODS Fifty consecutive patients, aged 4 months to 42 years, underwent intracardiac repair along with a superior cavopulmonary connection. Twenty-seven of the patients had had previous surgical palliation. Repair consisted of patch closure of the ventricular septal defect (n = 25), tricuspid valve repair (n = 26), reconstruction of the right ventricular outflow tract (n = 34), transpulmonary annular patch (n = 34), right ventricle to pulmonary artery homograft conduit (n = 4), and concomitant repair of atrioventricular canal (n = 9). Ten patients were left with a fenestration in the atrial septum. RESULTS There were six hospital deaths (12%) and two late deaths (4.5%). Forty-two survivors were followed from 8 months to 116 months. Eighty-eight percent are in functional class I. Actuarial survival at 97 months was 74%. CONCLUSIONS Moderate right heart hypoplasia constitutes a safe anatomic category for a pulsatile bidirectional Glenn. It is advisable not to proceed with a one and a half ventricle repair if postoperative residual pulmonary artery hypertension is anticipated. Patients requiring an intricate intracardiac repair and those with concomitant right heart hypoplasia may be better suited for a Fontan type of repair to reduce the complexity of the procedure.

AETIOPATHOGENESIS OF PERIPARTUM CARDIOMYOPATHY: PROLACTIN-SELENIUM INTERACTION?

The aetiology of peripartum cardiomyopathy is unknown. Fragmentary evidence from the published literature are synthesised to suggest a hypothesis that prolactin-selenium interactions resulting in selenium deficiency and/or autoimmunity are responsible for peripartum cardiomyopathy. This hypothesis best explains the various known facts about the disease. The possible link between prolactin and selenium should be explored.

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.