How should anesthesia be planned for a beta thalassemia patient with chronic anemia, iron‑overload cardiomyopathy, endocrine dysfunction, splenectomy‑related thrombocytosis or coagulopathy, and who is on regular transfusions and chelation therapy?

Anesthetic Management of Beta Thalassemia Patients

Beta thalassemia patients require meticulous hemodynamic monitoring during anesthesia with maintenance of their compensatory hyperdynamic circulation, avoidance of myocardial depressants, and careful fluid management due to their unique baseline physiology of chronic anemia, iron-overload cardiomyopathy, and altered vascular compliance.

Preoperative Assessment and Optimization

Cardiovascular Evaluation

• Obtain cardiac MRI with T2 measurement* to assess myocardial iron loading, as cardiac complications are the leading cause of death in thalassemia major, accounting for approximately 70% of mortality 1, 2
• Patients with T2* <10 ms have severe myocardial iron overload with a 47% risk of developing heart failure within one year 1
• Perform echocardiography to assess left ventricular ejection fraction and identify the hyperdynamic circulation pattern (increased cardiac index, elevated stroke volume, and heart rate) 3
• Recognize that mild tachycardia and cardiomegaly are physiological compensations for chronic anemia, not necessarily pathological findings 1

Hematologic Optimization

• Target pre-transfusion hemoglobin of 9-10 g/dL and post-transfusion hemoglobin of 13-14 g/dL to suppress ineffective erythropoiesis and reduce the hyperdynamic circulation 1, 4
• Transfuse preoperatively if hemoglobin is significantly below target, as severe anemia worsens the hyperdynamic state and increases cardiac oxygen consumption 3
• Assess for thrombocytosis in splenectomized patients or coagulopathy from liver dysfunction 1, 5

Endocrine and Metabolic Assessment

• Screen for and correct endocrinopathies including hypothyroidism, hypoparathyroidism, adrenal insufficiency, and diabetes mellitus, as these can mimic or exacerbate cardiac dysfunction 1
• Check thiamine, carnitine, and vitamin D levels (particularly if 25-hydroxyvitamin D <10 ng/dL), as deficiencies are common and replacement is benign 1
• Treat all patients as having potential adrenal insufficiency until proven otherwise, given the high prevalence of decreased adrenal reserve 1
• Optimize glucose control in diabetic patients, as insulin resistance is strongly associated with cardiac iron deposition and altered cardiac metabolism 1

Infection Risk Assessment

• Recognize that splenectomized patients are at high risk for severe infections from encapsulated organisms and sepsis is the second-leading cause of death in thalassemia major 1, 2
• Consider prophylactic antibiotics for splenectomized patients undergoing invasive procedures 6

Intraoperative Anesthetic Management

Hemodynamic Goals

• Maintain the compensatory hyperdynamic circulation rather than attempting to normalize it to standard values 1, 3
• Expect and accept baseline tachycardia, increased cardiac index, and lower systolic blood pressure as physiological adaptations 1
• Use invasive hemodynamic monitoring (arterial line, consider Swan-Ganz catheter) to track cardiac index and guide fluid management 3

Anesthetic Technique

• Use balanced anesthesia with low-dose volatile agents (e.g., isoflurane at low concentrations) combined with opioids (e.g., fentanyl) to avoid cardiovascular depression 3
• Avoid or minimize positive inotropes, as they increase intramyocyte calcium levels, worsen oxidative stress, and increase electrical automaticity, which may act synergistically with iron-mediated toxicity 1
• Reserve inotropes for desperate situations only and minimize doses whenever possible 1

Fluid Management

• Exercise extreme caution with fluid administration due to altered baseline hemodynamics 1
• Baseline preload is already high from chronic anemia; overdiuresis or excessive fluid restriction can precipitate acute renal failure 1
• Older patients may have restrictive physiology that tolerates neither overfilling nor underfilling 1
• Monitor urine output closely as a guide to adequate perfusion 1

Vasopressor and Afterload Management

• Titrate afterload reduction very carefully against urine output and clinical response rather than targeting standard blood pressure values 1
• Recognize that chronic anemia results in low systemic afterload, and poor vascular compliance (even in young patients) limits tolerated afterload reduction 1
• Standard blood pressure targets derived from non-thalassemia populations are not applicable 1

Ventilation Strategy

• Monitor for adequate oxygenation and ventilation, as anaerobic metabolism must be avoided 3
• Track lactate/pyruvate ratio if available to ensure aerobic metabolism is maintained 3

Postoperative Management

Monitoring

• Continue intensive hemodynamic monitoring in the immediate postoperative period 3
• Watch for arrhythmias, as atrial fibrillation and intra-atrial reentrant tachycardia are common, particularly with cardiac iron loading 1
• Monitor for signs of heart failure decompensation, especially in patients with known cardiac iron overload 1, 6

Transfusion Management

• Resume regular transfusion schedule promptly to maintain target hemoglobin levels 1, 4
• Continue iron chelation therapy as soon as oral intake is tolerated 1, 7

Infection Prevention

• Maintain high vigilance for sepsis, particularly in splenectomized patients, as infections can progress rapidly 1, 2, 6
• Implement early empiric antibiotic therapy if infection is suspected 6

Critical Pitfalls to Avoid

• Do not attempt to normalize heart rate or cardiac index to standard values, as the hyperdynamic circulation is compensatory 1, 3
• Do not aggressively reduce blood pressure to typical targets, as these patients have chronically low systemic vascular resistance 1
• Do not use standard heart failure protocols without modification, as the hemodynamic profile differs fundamentally from non-thalassemia patients 1
• Do not overlook endocrine dysfunction as a contributor to perioperative instability 1
• Do not underestimate infection risk in splenectomized patients 1, 2