Journal of Pediatric Cardiology and Cardiac Surgery

Circumferential myocardial calcification within the left ventricular wall is rarely reported during the perioperative period of pediatric open heart surgery. This case report highlights a unique instance of whole circumferential myocardial calcification after surgery leading to fatal biventricular diastolic dysfunction. A 9-month-old female patient, diagnosed with type B interrupted aortic arch and 22q.11.2 microdeletion, underwent the Yasui operation following stage I hybrid palliation. Postoperatively, despite initial improvements, she developed lactic acidosis and peripheral edema, with catecholamines being unable to be discontinued. Serial imaging and cardiac catheter examinations revealed progressive whole circumferential myocardial calcification and worsening ventricular diastolic pressures. Surgical attempts to remediate these complications were unsuccessful, and the patient unfortunately succumbed shortly after the fourth intervention. Pathological examination revealed diffuse granular calcification deposits in the pericardium. Calcification was also observed in the submitted left ventricular myocardial tissue, with only a small amount of viable myocardial cells present. This case underscores the severity of myocardial calcification that can complicate the postoperative course in pediatric cardiac surgery, potentially leading to severe diastolic dysfunction and death. Early recognition and management would be critical, though effective treatment modalities need further exploration.