AJA Asian Journal of Anesthesiology

Advancing, Capability, Improving lives

Correspondence
Volume 59, Issue 1, Pages 35-36
Yi-Chun Chou 1 , Jeng Wei 2 , Yuan-Shiou Huang 1
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Outline


To the Editor,

Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA), or Bland–White–Garland syndrome, is a rare type of congenital heart disease. It affects 1 in 300,000 live births.1 It is one of the most common causes of myocardial ischemia and infarction in children. Most of these patients will not survive for a year.2 Diagnosing this abnormality in adulthood is rare. We present a case of successful surgical repair of this coronary anomaly.

A 25-year-old female had no medical history before suddenly lost consciousness. Her family immediately called emergency medical services and initiated cardio-pulmonary-cerebral resuscitation (CPCR). After 10 minutes of CPCR, the emergency medical services arrived and activated an automated external defi brillator. She was sent to the hospital nearby after return of spontaneous circulation. Coronary angiography revealed left coronary artery (LCA) orifi ce in the pulmonary artery (PA) and congenital anomaly with right coronary artery (RCA) collateral circulation. She was transferred to our hospital for surgery.

In the operating room, standard monitoring showed initial heart rate = 116 bpm, blood pressure = 136/62 mmHg, room air SpO2 = 98%. After right radial arterial catheterization, general anesthesia was induced with IV midazolam 2.5 mg, fentanyl 50 mcg, propofol 200 mg, and rocuronium 50 mg. She was intubated with #7.0 endotracheal tube and controlled ventilation keeping EtCO2 at 38–40 mmHg. Sevofl urane and O2-air mixture were used to keep adequate entropy values of 40–60. Transesophageal echocardiography revealed slightly reduced left ventricular (LV) systolic function and hypokinesis of the LV anterior wall.

After midline sternotomy and pericardiotomy, the diagnosis of ALCAPA was confi rmed. The RCA was severely dilated and tortuous. The surgeon ligated the anomalous origin of LCA in combination with saphenous vein grafting between LAD and aorta. Under inotropic support with dopamine (3–5 mcg/kg/min), the cardiopulmonary bypass weaning successfully. The patient had an uncomplicated perioperative and postoperative course.

ALCAPA is the commonest congenital coronary anomaly. According to the collateral circulation development, it can divide into the infantile and the adult types. The adult type has better collaterals from the RCA, which provide adequate LV perfusion.3,4 It accounts for 10–15% of patients to survive in later childhood.2 With the pulmonary pressure decreasing, it may cause a reversal of flow in the left coronary circulation to pulmonary circulation. This situation was known as the “coronary steal phenomenon” and result in myocardial ischemia, myocardial infarction, LV dysfunction, and mitral regurgitation, leading to sudden cardiac death.

The anesthetic goals are to preserve adequate preload, contractility, and keep myocardial oxygen balance. Because of extensive RCA collateral circulation to LCA territory in adult ALCAPA, once decreased pulmonary vascular resistance (PVR) will amplify RCA-to-PA shunt, low FiO2, and higher EtCO2 were set to maintain normal to higher PVR.4-6 In this patient, kept entropy values of 40–60 to assure the depth of anesthesia. Perioperative fentanyl (25–50 mcg) and nicardipine (0.5–1 mg) bolus were used to manage hypertension and tachycardia. The systemic vascular resistance (SVR) is inversely proportional to cardiac output and also affects LV systolic wall stress. Maintain normal to low SVR can provide better RCA perfusion and myocardial oxygen balance. Being familiar with the pathophysiology of ALCAPA, the patient can have better pre-bypass management.


References

1
Cowles RA, Berdon WE.
Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review.
Pediatr Radiol. 2007;37(9):890-895.
2
Wesselhoeft H, Fawcett JS, Johnson AL.
Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases.
Circulation. 1968;38(2):403-425.
3
Nath MP, Kiran U, Talwar S.
Anomalous origin of left main coronary artery from pulmonary artery in an adult: peri-operative concerns.
Indian J Thorac Cardiovasc Surg. 2010;26(2):157-161.
4
Patil S, Shah M, Patel B, et al.
Incidental finding of the anomalous origin of left main coronary artery from pulmonary artery in an adult presenting with arrhythmia- induced myocardial ischemia.
Case Rep Cardiol. 2018;2018:6485831.
5
Gupta K, Gupta M, Mehrotra M, Prasad J.
Anaesthesia for repair of anomalous origin of left coronary artery from pulmonary artery.
Indian J Anaesth. 2015;59(2):136-137.
6
Kastellanos S, Aznaouridis K, Vlachopoulos C, Tsiamis E, Oikonomou E, Tousoulis D.
Overview of coronary artery variants, aberrations and anomalies.
World J Cardiol. 2018;10(10):127-140.

References

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