Trans-aortic DORV repair.

Double-outlet right ventricle (DORV) is a group of complex ventriculoarterial connections. In the literature, there are reports of trans-aortic correction of tetralogy of Fallot, as well as ventricular septal defects and complex DORV without right ventricular outflow tract obstruction. However, a pure trans-aortic approach for DORV ventricular septal defect (VSD) right ventricular outflow tract obstruction repair is not reported in the literature. We present a case of pure trans-aortic repair of DORV VSD pulmonary stenosis (PS) in an adult. A 20-year-old male with a known case of DORV, VSD, and PS presented with cyanosis. A pure trans-aortic repair of DORV was done. Complete trans-aortic DORV VSD PS repair is yet to be reported in the literature. The trans-aortic approach avoids a right atriotomy, right ventriculotomy, and injury to coronary arteries in cases of complicated tetralogy of Fallot and avoids injury to the tricuspid valve. This approach can be used in selected patients for intracardiac repair. The advantages are excellent visualization of the defects and avoidance of injury to the aortic cusps and bundle of His. In addition, placement of the VSD patch on the left ventricular outflow side may prevent residual shunts after repair. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-023-01564-x.

Comparison of Left Ventricular Global Longitudinal Strain with Ejection Fraction as a Predictor for Peri-operative IABP Insertion in Patients Undergoing Off-Pump Coronary Artery Bypass Grafting: A Pilot Study.

Background: Prophylactic use of intra-aortic balloon pump (IABP) mainly depends on left ventricular (LV) systolic function. Global longitudinal strain (GLS) is a robust prognostic parameter for LV strain. It has proved to be more sensitive than LV ejection fraction (EF) as a measure of LV systolic function and is a strong predictor of outcome. Aim: To determine whether GLS can be used as a reliable marker and its cut-off value for IABP insertion in patients undergoing elective off-pump coronary artery bypass grafting (OPCABG). Settings and Design: A prospective observational clinical study which included 100 adult patients scheduled for elective OPCABG. Materials and Methods: Two-dimensional (2D) speckle tracking echocardiography (STE)-estimated GLS was computed and compared with LV EF measured by three dimensional (3D) echocardiography for the insertion of IABP. The intensive care unit (ICU) parameters were correlated with echocardiographic parameters to predict early post-operative outcome. Results: IABP insertion correlates better with GLS (post-revascularization > pre-revascularization) than with 3D LV EF. Receiver operating characteristic (ROC) curve analysis revealed the highest area under the curve (AUC, 0.972) with a cut-off value of > -9.8% for GLS compared to 3D LV EF (AUC, 0.938) with a cut-off value of ≤ 44%. ICU parameters show better correlation with E/e'> GLS > WMSI than 3D LV EF. Conclusion: GLS is a better predictor of IABP insertion compared to 3D LV EF in patients undergoing OPCABG.

Complete transposition of great arteries with cor triatriatum: an unusual coexistence.

The combination of complete transposition of the great arteries and cor triatriatum is extremely rare. We report three infants with this rare combination and discuss the anatomic details apparently unique to the combined lesion.

Ventricular septal rupture in a 4-year-old child following blunt chest injury.

We report on a 4-year-old child who developed rupture of the ventricular septum after a blunt chest injury. The symptoms appeared more than 12 h later with progressive deterioration in his hemodynamic status. The diagnosis was made by echocardiography and the large defect in the muscular part of the ventricular septum was successfully closed at surgery.

Total anomalous pulmonary venous connection: surgical experience in Indians.

BACKGROUND: We report a retrospective analysis of the demographic. morphological and clinical profiles of patients along with results of operative repair for total anomalous pulmonary venous connection. METHODS AND RESULTS: In the last 15 years, 248 patients (168 boys, 80 girls) underwent repair for total anomalous pulmonary venous connection. Their ages ranged from I day to 24 years (median 8 months) and 145 of them were < or = 1 year of age. The patients' weight ranged from 2 to 52 kg (median 5 kg). About 70% of patients (n = 174) were less than the 50th percentile of predicted weight for age and sex. The anomalous connection was supracardiac in 134 (54%), cardiac in 80 (32.2%), infracardiac in 9 (3.6%) and mixed in 25 (10.1%) patients. Fifty (20.2%) patients had obstructed drainage and 76 patients (30.2%) had moderate or severe pulmonary arterial hypertension. Forty-five patients (18.1%) had to be operated upon on an emergency basis. All the patients were operated upon using moderately hypothermic cardiopulmonary bypass. In 114 patients, circulatory arrest was used. There were 45 (19.1%) in-hospital deaths. The major causes of early death were pulmonary arterial hypertensive crisis in 19 (7.7%) and low cardiac output syndrome in 17 (6.9%) patients. Age < or = 1 year (odds ratio 2.16; 95% confidence interval: 1.22-3.82, p=0.008), severe pulmonary arterial hypertension (odds ratio 5.86; 95% confidence interval: 2-17, p=0.001), and need for emergency surgery (odds ratio 3.65; 95% confidence interval: 1.59-8.38, p=0.002) were independent risk factors for early death. Follow-up ranged from 1 to 180 months (median 48 months). There were 4 lake deaths. Actuarial survival at 12 years was 92.6% +/- 2.8%. CONCLUSIONS: In Indian circumstances, mortality continues to be high in infants with total anomalous pulmonary venous connection. Severe pulmonary arterial hypertension appears to be the most important predictor of operative mortality. Severe malnutrition, delayed diagnosis and late referrals possibly contribute to the high mortality.

Light and electron microscopic features of surgically excised left atrial appendage in rheumatic heart disease patients with atrial fibrillation and sinus rhythm.

INTRODUCTION: There are few studies comparing the pathology of the remodeled substrate in patients of rheumatic heart disease with atrial fibrillation (AF) and normal sinus rhythm (NSR). METHODS: The study group comprised 30 patients with rheumatic heart disease undergoing mitral valve replacement. Excised left atrial appendages of these patients [17 with persistent AF and 13 NSR (control group)] were subjected to light and electron microscopic examination. RESULTS: The histopathological findings of the myocardium were characterized by cardiomyocyte hypertrophy (CH), nuclear enlargement (NE), perinuclear clearing (PC), sarcoplasmic vacuolation (SV), fibrosis, and inflammation in the patients with AF and NSR. NE (17/17 vs. 4/13; P=.004), PC (17/17 vs. 4/13; P=.004), SV (17/17 vs. 9/13; P=.06), and fibrosis (15/17 vs. 3/13; P=.001) were all significantly more common in patients with AF. Inflammatory cells were observed in 9/17 patients of AF as compared to 1 in NSR patients (9/17 vs. 1/13; P=.02). CH was common in the patients with AF as compared with those in NSR (17/17 vs. 10/13; P=.103). In AF patients, electron microscopy revealed cardiomyocytes with depletion of the contractile elements (Z-bands), glycogen particle accumulation, and an increase in mitochondria. Cells severely affected by AF showed loss of contractile elements with extensive areas of SV, presence of myelin figures, and mitochondrial aggregates. Majority of AF cases showed extensive fibrosis in the form of collagen bundles in the interstitium. CONCLUSION: The left atrial substrate in AF as compared with NSR, in rheumatic heart disease patients, is associated with significant degenerative remodeling and ongoing inflammation that is associated with extensive fibrosis.

Idiopathic left ventricular outflow tract pseudoaneurysm.

Left ventricular outflow tract (LVOT) pseudoaneurysm is a rare occurrence and may produce clinically unpredictable symptoms. A very few cases of LVOT pseudoaneurysm are reported and there has always been a predisposing factor in these reported cases such as history of infective endocarditis, myocardial infarction, prosthetic aortic valve replacement or chest trauma. Our patient did not have the above predisposing conditions. Intra operative transesophageal echocardiography helped patient management and guided the surgical team in securing and isolation of the aneurysmal sac from the LVOT.

An alternative technique for rechanneling of sinus venosus atrial septal defect with partial anomalous pulmonary venous connection using autogenous right atrial appendage.

We report a new technique for closure of sinus venosus atrial septal defect with high partial anomalous pulmonary venous connection. This technique consisted of preservation of the atriocaval junction, advancement of the posterior rim of the atrial septal defect anterosuperiorly and enlargement of the superior caval vein using right atrial appendage. We found this to be a convenient technique and recommend it for correction of this anomaly.

Right atrial myxoma with glandular differentiation: A rare entity in pediatric age group.

Cardiac myxomas (CMs) account for nearly half of the primary cardiac tumors in the elderly. They arise from sub-endocardial "reserve" or lepidic" cells, which may show divergent differentiation. We describe a CM with glandular differentiation in the right atrium of a 10-year-old child who presented with respiratory distress on exertion, of 2 months duration. On echocardiography, two large interconnected masses measuring 34×30 mm and 20×17 mm were seen to arise from the free wall of the right atrium. Cut surface of the excised mass was myxoid with areas of calcification. On microscopy, there were typical features of a myxoma with prominent glandular differentiation and characteristic immunophenotype. The case is being reported due to its rarity in pediatric age group as well as its glandular differentiation, which must be recognized as a spectrum of histomorphologic diversity and must not be mistaken for a metastatic adenocarcinoma.