Williams?Beuren Syndrome with Pseudoaneurysm of Aortic Arch and Infective Vegetations for Modified Broms Procedure: Anesthetic Concerns & Echocardiographic Illustrations

Williams?Beuren syndrome is a rare genetic malformation with predilection for supravalvular aortic stenosis. Apart from cardiovascular malformation, hypocalcemia, developmental delay, and elfin facies, challenging airway make perioperative management more eventful. Association of infective endocarditis within the aortic arch and pseudoaneurysm formation is infrequent. We, hereby report a case of pseudoaneurysm formation and infective vegetation within the aortic arch in a patient with Williams syndrome and the role of transthoracic echocardiography in its perioperative management.

Left ventricular outflow tract obstruction following aortic valve replacement: A review of risk factors, mechanism, and management

The presence of dynamic left ventricular outflow tract obstruction (LVOTO) can complicate the postoperative course of patients undergoing surgical aortic valve replacement (AVR). The phenomenon of LVOTO is a consequence of an interplay of various pathoanatomic mechanisms. The prevailing cardiovascular milieu dictates the hemodynamic significance of the resultant LVOTO in addition to the anatomical risk factors. A thorough understanding of the predisposing factors, mechanism, and hemodynamic sequel of the obstruction is pivotal in managing these cases. A comprehensive echocardiographic examination aids in risk prediction, diagnosis, severity characterization, and follow-up of management efficacy in the setting of postoperative LVOTO. The armamentarium of management modalities includes conservative (medical) and surgical options. A stepwise approach should be formulated based on the physiological and anatomical substrates predisposing to LVOTO. The index phenomenon occurs more frequently than appreciated and should be considered when the post-AVR patients exhibit hemodynamic instability unresponsive to conventional supportive measures. The present article provides an overview of various peculiarities of this under-recognized phenomenon in the context of the perioperative management of patients undergoing AVR.

Bleeding in the lung complicates a routine intracardiac repair: What went wrong!!!

Cyanotic congenital heart disease presents an increased tendency to bleed in view of subtle coagulation defects. Airway bleeding can be particularly difficult to manage while maintaining an adequate ventilation. An isolated lung bleed with the exclusion of possible traumatic, medical and surgical causes of bleeding, should alert the attending anesthesiologist to the possibility of the collateral-related bleeding. Preoperative coil embolization remains an important initial management step in a case of tetralogy of Fallot (TOF) with major aortopulmonary collaterals. Nevertheless, the coiling of the collaterals in certain specific case scenarios is not feasible, rendering the management of a lung bleed, all the more challenging. We, hereby discuss a case of a 7-year-old girl with a massive endotracheal bleed at the time of weaning from cardiopulmonary bypass after corrective surgery for TOF. The subsequent approach and management are discussed. The optimal management of tetralogy with collaterals mandates an effective communication among the cardiologist, radiologist, anesthesiologist, and the surgeon.

Left atrial ball thrombus with acute mesenteric ischemia: Anesthetic management and role of transesophageal echocardiography.

A 62 year old female with severe mitral stenosis, large left atrial ball thrombus and acute mesenteric ischemia emergently underwent mitral valve replacement, left atrial clot removal and emergency laparotomy for mesenteric ischemia. Peri‑operative management issues, particularly, the anesthetic challenges and the role of transesophageal echocardiography are discussed.

Caudal epidural sufentanil and bupivacaine decreases stress response in paediatric cardiac surgery.

Surgery and anaesthesia are known to cause stress response. Attenuation of stress response can decrease morbidity, postoperative hospital length of stay and, thus, cost. Intrathecal and epidural techniques produce reliable analgesia in patients undergoing surgery along with stress response attenuation. The present study was undertaken to evaluate the efficacy of caudal sufentanil and bupivacaine combination on perioperative stress response in paediatric patients undergoing open heart surgery. Thirty patients (ASA grade II-III) undergoing elective corrective cardiac surgery for acyanotic congenital heart disease, were randomly allocated to two groups. In group GA (n = 15), patients received balanced general anaesthesia. In group GC (n = 15), in addition to general anaesthesia, caudal block with bupivacaine and sufentanil combination was given after endotracheal intubation. Monitoring included electrocardiography, invasive arterial pressure, end-tidal carbon dioxide, pulse oximetry, arterial blood gases including serum electrolytes, blood glucose, serum cortisol, urine output, central venous pressure and temperature. Haemodynamic responses in both groups were statistically similar. Serum cortisol levels were significantly lower in GC group than GA group (P < 0.05) after sternotomy (9.8+/-7.5 vs. 34.74+/-27.35), on cardiopulmonary bypass (CPB) (12.17 +/- 6.2 vs. 35.36 +/- 24.15), after sternal closure (14.03 +/- 5.1 vs. 37.62 +/- 20.69), 4 hours (26.64 +/- 14.61 vs. 37.62 +/- 9.13) and 24 hours (14.30 +/- 8.11 vs. 28.12 +/- 16.31) after intubation. Blood glucose levels were significantly higher in GA group as compared to GC group at sternal closure (277.46 +/- 77.25 vs.197.73 +/- 42.17) and 4 hours (255.26 +/- 73.73 vs. 185.26 +/- 57.41) after intubation (P < 0.05). To conclude, supplementation of caudal epidural bupivacaine and sufentanil could effectively attenuate the stress response in paediatric patients undergoing cardiac surgery under CPB in acyanotic congenital heart anomaly.