Novel Peri-Operative Strategy for Sickle Cell Disease with Tetralogy of Fallot.

ABSTRACT: Peri-operative management of cyanotic congenital heart disease in a patient of sickle cell disease (SCD) can be challenging. We report a case of Tetralogy of Fallot and homozygous SCD with history of multiple blood transfusions and sickle cell crises who underwent intracardiac repair. Hemoglobin S level was reduced from 75% pre-operative to 21.8% postoperative with a combination of pre-operative blood transfusion, intraoperative exchange transfusion, and normothermic cardiopulmonary bypass (CPB). Pre-operative optimization and safe intraoperative conduct were essential to avoid sickling crises.

Aortic Uncrossing for Circumflex Aorta.

Circumflex aorta is described as a retroesophageal aortic arch, with opposite-sided descending aorta forming a true vascular ring with ligamentum arteriosum. We report two cases of right-sided circumflex aorta with varied clinical presentation. Computed tomography diagnosed this vascular ring anomaly. The patients were managed with the aortic uncrossing procedure as a primary surgical strategy. Preoperative diagnosis and thorough planning were essential for the successful outcome. The patients did not have any residual symptoms of tracheal and esophageal compression on follow up.

Rapid-Onset Obesity, Hypothalamic Dysregulation, Hypoventilation, Autonomic Dysregulation, and Neuroendocrine Tumour: Rare Syndrome with Myriad Anaesthesia Implications.

Anaesthesia management of a child with rapid-onset obesity, hyperphagia, and hypothalamic dysfunction syndrome is complex due to the multisystem involvement, the most important features being morbid obesity, autonomic dysfunction, and dyselectrolytemia due to hypothalamic dysfunction. The acronym of the disease was amended in 2008 to rapid-onset obesity, hyperphagia, hypothalamic dysfunction neural crest tumour to include the risk of ganglioneuroma or ganglioneuroblastoma. Patients usually require removal of tumour in the prone position. Obstructive sleep apnea, difficult airway and intravenous access, and haemodynamic lability all add to the trials faced by the paediatric anaesthesiologist. Invasive haemodynamic monitoring, ultrasonography, bispectral index monitoring, and meticulous calculation of drug dosages help in smoothening the course of anaesthesia in the presence of constant vigilance.

Pediatric cardiac surgery following severe acute respiratory syndrome coronavirus-2 infection: Early experience and lessons learnt.

Background: We evaluated our early experience of cardiac procedures in children with congenital heart defects (CHD) after asymptomatic severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, to understand whether recent reverse transcription-polymerase chain reaction (RT-PCR) positivity had a negative impact on their postprocedural recovery and outcomes. Materials and Methods: In this retrospective observational study, all patients with CHD who underwent cardiac surgery or transcatheter intervention at our institution between March 2020 and June 2021 who were detected to have asymptomatic SARS-CoV-2 infection on routine RT-PCR were included. Details of the cardiac procedure and postprocedural recovery were reviewed and compared with RT-PCR-negative patients who concurrently underwent similar cardiac surgeries or interventions at our center. Results: Thirteen patients underwent cardiac surgery after recent SARS-CoV-2 positivity after a mean interval of 25.4 ± 12.9 days. One patient expired with multiorgan dysfunction and systemic inflammatory response with elevated D-dimer, serum Ferritin, C-reactive protein, and significant ground-glass opacities on chest radiograph. Another patient developed spontaneous thrombosis of the infrarenal abdominal aorta, bilateral iliac arteries, and bilateral femoral veins, requiring low-molecular weight heparin postoperatively. This patient's postoperative recovery was also prolonged due to lung changes delaying extubation. All other patients had uneventful postprocedural recovery with intensive care unit and hospital stays comparable to non-SARS-CoV-2-infected patients. Conclusions: From our early experience, we can surmise that an interval of 2-3 weeks after asymptomatic SARS-CoV-2 infection is adequate to undertake elective or semi-elective pediatric cardiac surgeries. For patients requiring emergent cardiac surgery prior to this interval, there is potentially increased risk of inflammatory and/or thrombotic complications.

Anomalous right coronary artery from pulmonary artery in aortopulmonary window.

We report a case of a 1-year-old patient with aortopulmonary window, anomalous right coronary artery arising from the pulmonary artery, and a large perimembranous ventricular septal defect. Computed tomography was successful in diagnosing the coronary anomaly and the case was managed surgically with an intrapulmonary baffle, rerouting the right coronary ostium to the aortic root and a fenestrated patch closure of the ventricular septal defect. Pre-operative diagnosis and thorough planning were essential for the successful outcome of this case.