Zhuo Sun MD(1), Mark Banoub MD(1),Celeste Kessle RSDS(2),  Zahid Amin MD (2)
(1) Departments of Anesthesiology and Perioperative Medicine (1),  Pediatric Cardiology (2),  Medical College of Georgia, Augusta University, Augusta, GA, US

Coronary artery fistulas (CAF) are present in 0.002% of the general population representing 0.4% of all cardiac malformations. Fistulas can originate from the right coronary (50-60%), left anterior descending (25-42%), circumflex (18.3%), diagonal branch (1.9%), or left main coronary artery (0.7%) (1). While CAF are usually single in  74-90% of cases (2), multiple fistulas with separate origins from the coronary artery tree are rare. Here we present a case of multiple CAFs arising from different sites to pulmonary artery.

A 7 year old girl with a history of symptomatic heart murmur was found to have collateral flow entering the pulmonary artery by echo (Figure 1). CAF was suspected and a cardiac catheterization was performed. Inhalation induction was applied followed with endotracheal intubation and the whole procedure was uneventful. Multiple CAFs of various sizes were found arising from left main and anterior descending coronary (LAD) artery entering the proximal pulmonary artery (Figure 2). Because the child was asymptomatic and closure of the larger fistulas will likely result in rapid enlargement of the smaller others, it was decided to defer interventions for later in life when a long covered stent can be a viable option. This patient had an uneventful recovery at PACU and was discharged to home at the same day.

Most children with CAFs are asymptomatic and present with acontinuous cardiac murmur  on physical examination. Symptoms of myocardial ischemia arise due to diversion (steal) of oxygenated coronary blood flow to the lower pressured pulmonary artery circulation. Other symptoms may include volume overload due to left-to-right shunt, arrhythmia, aneurysm and rupture (3). Transthoracic echocardiography (TTE) has proved useful for  diagnosis but not for functional fistula evaluation. Cardiac catheterization  is the modality of choice for defining coronary artery anatomic connections and flow patterns. Therapeutic embolization can be performed with high efﬁciency and safety. Surgical treatment is generally reserved for single, large, symptomatic ﬁstulae that present with angina. For any procedure, a thorough preoperative evaluation, avoiding large increases in myocardial oxygen demand and maintaining coronary perfusion pressure are the major considerations for smooth anesthetic management. 

References:

1.	Dodge-Khatami A, Mavroudis C, Backer CL. Congenital heart surgery nomenclature and database project: anomalies of the coronary arteries. Ann Thorac Surg 2000;69 (3 Suppl 1):270 –7.

2.	Fernandes ED, Kadivar H, Hallman GL, Reul GJ, Ott DA, Cooley DA. Congenital malformations of the coronary arteries: the Texas Heart Institute experience. Ann Thorac Surg 1992;54:732– 40.

3.	Chirantan V. Mangukia. Coronary artery fitula.  Ann Thorac Sur 2012 Jun;93(6):2084-92 


06 / Multiple coronary artery fistulas to pulmonary artery