Risk factors and outcomes for surgical repair of obstructed total anomalous pulmonary venous connection.

Background: Surgical repair of obstructed total anomalous pulmonary venous connection (TAPVC) is a high-risk surgical cohort. This study aimed to assess surgical risk factors and outcomes in infants with TAPVC treated at a single centre. Methods: This was a prospective single-centre, observational study that included consecutive patients presenting with obstructed TAPVC. Patients with obstructed supra-cardiac, cardiac, and infra-cardiac TAPVC were included in the study. In-hospital mortality was the primary outcome. Multivariable logistic regression was carried out to identify risk factors for in-hospital mortality. Results: Of the 41 patients, 31 (75.6%) were males. Ages were distributed as follows: 20 (48.8%) between 1 and 3 months, 11 (26.88%) between >3 months, and 10 (24.4%) neonates. The in-hospital mortality was 19.5% (n=8). Of the remaining 33 patients, there were no deaths during the follow-up. Short-term follow-up was achieved for 100% of cases (up to 6 months post-operatively). The mean duration of follow-up was 43.6± 3.6 months. One (3%) early and 2 (6%) late pulmonary vein obstructions were observed during follow-up. Age less than 30 days and pre-operative ventilation were associated with significantly increased risk of in-hospital mortality. Conclusions: Despite logistical challenges, reasonable surgical outcomes are attainable in obstructed TAPVC cases. Age less than 30 days and need for pre-operative ventilation were independent predictors of in-hospital mortality, while pulmonary venous obstruction in the post-operative period contributed to re-interventions.

Congenital agenesis of anterior leaflet of tricuspid valve.

Complete absence of anterior tricuspid valve leaflet is a rare anomaly. We report the case of a 10-year-old girl who presented with severe tricuspid regurgitation with annular dilatation and non-coapting leaflets. Intra-operatively, an incidental finding of complete agenesis of the anterior leaflet was observed. A tricuspid valve replacement was carried out; the patient made an uneventful recovery and remains well at follow-up.

Urgent Excision of Primary Intracardiac Burkitt Lymphoma in a Child.

A 9-year-old girl presented with a recent history of shortness of breath, fatigue, visual disturbances, and gastrointestinal symptoms. Echocardiography demonstrated three large intracardiac masses in the right and left atria protruding into the mitral and tricuspid valve orifices causing bilateral inflow obstruction. She underwent urgent surgical excision of the masses. Histology revealed rare intracardiac Burkitt's Lymphoma.

d-Transposition of Great Arteries and Total Anomalous Pulmonary Venous Connection With Left Ventricular Regression-A Rarity.

Transposition of great arteries (TGA) in association with total anomalous pulmonary venous connection (TAPVC) has been rarely reported. With increasing age, left ventricular (LV) regression further complicates the clinical scenario. Management strategy is not clearly defined. We describe a rare combination of TGA, TAPVC, and LV regression and outline a successful management strategy.

Successful surgical management of combined presence of congenital diaphragmatic hernia, ventricular septal defect, and patent ductus arteriosus in a child with allergy to frusemide-a case report.

A 6-week-old male child presented with a right-sided congenital diaphragmatic hernia (CDH), large perimembranous (PM) ventricular septal defect (VSD) and multiple muscular VSDs, and a patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension (PAH). The child was found to be allergic to frusemide. The VSDs were closed, the PDA was ligated, and the CDH was repaired through a right subcostal incision. Postoperatively, there were recurrent episodes of right lung lobar collapse, but the child responded to intravenous antibiotics and nasal continuous positive airway pressure (CPAP). The child is medically stable and on outpatient follow-up. We present this rare combination of diseases in view of a successful simultaneous surgical repair.

Successful surgical management of periosteal chondroma of the left second rib: a case report.

We present a case of successfully resected periosteal chondroma of the left second rib in view of extremely rare occurrence of such cases and the success achieved in its management. A 12-year-old female child presented to our hospital with a swelling in the upper anterolateral aspect of the left chest wall. On chest X-ray and contrast-enhanced computed tomography (CECT), it was diagnosed to be a case of osteochondroma of the second rib. The tumor was approached through a left posterolateral thoracotomy and parts of the first and second ribs were excised along with the tumor. Patient is now on outpatient follow-up and hemodynamically stable with no recurrence of symptoms. The histopathology revealed periosteal chondroma of the second rib with all margins of the resected mass being free of tumor suggesting complete excision. Patient is now on outpatient follow-up and hemodynamically stable with no recurrence of symptoms. Postoperative results were very satisfactory not only in terms of oncological clearance but also from the functional and cosmetic point of view. We believe our case is going to add to the very limited number of such cases available in literature.

Objective assessment of delayed gastric emptying using gastric scintigraphy in post pancreaticoduodenectomy patients.

BACKGROUND: No objective assessment of delayed gastric emptying is carried out in patients undergoing Whipple's procedure. All definitions and criteria along with evaluation of incidence of delayed gastric emptying are based on subjective assessment of the patient with clinical overview, so this study was carried out for an objective and accurate assessment of delayed gastric emptying in post Whipple's patients using gastric scintigraphy. METHODS: Twenty-one patients undergoing Whipple's procedure performed by a single surgeon in a tertiary referral centre were included in the study. Gastric scintigraphy was performed preoperatively and on post-operative 10th and 21st days. Preoperative and post-operative gastric scintigraphy findings were used for the diagnosis of delayed gastric emptying and it was correlated with the incidence of clinical diagnosis of delayed gastric emptying. RESULTS: Thirteen out of 21 patients had clinical delayed gastric emptying. When compared with clinical delayed gastric emptying, the sensitivity, specificity, positive and negative predictive values of post-operative 10th day gastric scintigraphy were 61.53, 100, 100 and 61.53%, respectively (P = 0.004). The sensitivity, specificity, positive and negative predictive values of post-operative 21st day gastric scintigraphy were 38.46, 100, 100 and 50%, respectively (P = 0.04). Reflux across the gastrojejunal anastomosis was noted in 28.5% on dynamic scintigraphy with the severity of clinical symptoms related directly with the degree of reflux. No correlation was seen between delayed gastric emptying and variables such as diabetes mellitus, hypertension and pancreaticojejunal anastomotic leaks. CONCLUSIONS: Gastric scintigraphy may be used to diagnose delayed gastric emptying more objectively in post Whipple's patients.