Abdominal aortic aneurysm repair in an infant with tuberous sclerosis, cardiac rhabdomyoma, and hypercoagulability: The role of multidisciplinary management.

An infant diagnosed prenatally with tuberous sclerosis (TS) and cardiac rhabdomyoma presented at 6 months of age with an infrarenal abdominal aortic aneurysm. A multidisciplinary team comprising pediatric cardiologists, vascular surgeons, and pediatric cardiac anesthesiologists collectively managed the surgical repair. Given the possibility of diastolic dysfunction related to intracardiac rhabdomyomas, transesophageal echocardiography (TEE) was employed intraoperatively to guide fluid and inotrope management. Platelets were transfused based on clinical judgment due to ongoing blood loss; however, this led to postoperative graft thrombosis, likely related to TS-associated hypercoagulability - subsequently confirmed by thromboelastography (TEG). Prompt bedside vascular ultrasound enabled early detection of the thrombosis, facilitating emergency graft revision and initiation of anticoagulation. A planned conduit revision was advised to address future growth-related graft insufficiency. This case emphasizes the relevance of early TEG, intraoperative TEE guidance, and multidisciplinary decision-making in complex pediatric vascular surgeries. Case also highlights the need for structured pediatric cardiac anesthesia services in low- and middle-income countries, especially in peripheral centers, to address rare but life-threatening cases.