2026 FSA Podium and Poster Abstracts

Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect, accounting for 5–7% of cases. In the U.S., it occurs in 0.46 per 1,000 live births and remains a significant cause of congenital cardiovascular morbidity (1). Clinical severity depends on the degree of right ventricular outflow obstruction and typically presents as neonatal cyanosis. Survival of uncorrected TOF past the age of 40 years is only 3%, and outcomes are worse with pulmonary atresia than pulmonary stenosis (2). Here we present the perioperative management of a 60-year-old female with uncorrected TOF who presented to our community hospital with acute cholecystitis.

Case Presentation: A 60-year-old Cuban female with uncorrected TOF, reduced exercise capacity and chronic hypoxia (baseline saturation in the 80s) presented with right upper quadrant pain from acute cholecystitis requiring cholecystectomy. Prior echocardiography showed an ejection fraction of 58%, an unrestrictive anterior malaligned ventricular septal defect, a hypoplastic right ventricular outflow tract, and mild aortic root dilation. Due to elevated perioperative risk, she was transferred to our tertiary cardiac center.

Before surgery, she developed respiratory distress with worsening oxygenation concerning for sepsis. A multidisciplinary decision was made to perform ultrasound-guided percutaneous cholecystostomy under monitored anesthesia care to allow for patient optimization prior to definitive surgical treatment. Her course was complicated by ARDS and multiorgan failure, requiring intubation, mechanical ventilation, vasopressors, broad-spectrum antibiotics, and continuous renal replacement therapy. Blood cultures were negative; bile cultures showed few gram-negative bacteria. Oxygenation was blood pressure dependent, improving with higher systolic pressures, consistent with afterload sensitive right-to-left shunt physiology.

After stabilization, she was discharged to inpatient rehabilitation with outpatient surgical follow-up. Following resolution of inflammation with the biliary drain in place, she underwent successful laparoscopic cholecystectomy. A preoperative arterial line was placed. Induction proceeded with ketamine and midazolam, sevoflurane for maintenance, and support provided by phenylephrine and vasopressin to maintain afterload. The procedure had no complications, and she achieved full recovery.

Discussion: Adults with congenital heart disease have increased perioperative risk during noncardiac surgery and require individualized, multidisciplinary management (1). This case illustrates the rare survival of uncorrected TOF into adulthood and the significant physiologic complexity encountered during sepsis and noncardiac surgery. In preoperative evaluation, a 12 Lead EKG should be assessed for conduction abnormalities as these patients are at increased risk of sustained monomorphic ventricular tachycardia due to anatomical isthmuses (1). Acquired chronic hypoxemia may lead to secondary erythrocytosis, increasing blood viscosity and risk of microvascular impairment, thrombosis, embolism, infection, and gallstones (3). Anesthetic management parallels that of pulmonary hypertension: decreasing pulmonary vascular resistance enhances pulmonary blood flow, while maintaining systemic afterload to limit right-to-left shunting (4). Reduced physiologic reserve makes these patients particularly vulnerable to hemodynamic fluctuations, increasing morbidity and mortality (4). This case underscores the importance of multidisciplinary management in centers experienced in adult congenital heart disease and optimization prior to surgery to achieve favorable outcomes (1).

References:

1. AHA. 2025 Guideline for the Management of Adults With Congenital Heart Disease. 

2. DOI: 10.1016/0002-9149(78)90941-4

3. DOI: 10.19080/JOCCT.2018.10.555793

4. DOI: 10.1007/s11886-022-01639-y