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Interact CardioVasc Thorac Surg 2008;7:933-934. doi:10.1510/icvts.2008.181388 © 2008 European Association of Cardio-Thoracic Surgery
Surgical repair of triple coronary-pulmonary artery fistulae with associated atrial septal defect and aortic valve regurgitation
a Cardiothoracic Unit, Block C5, University Hospital of Wales, Cardiff, CF14 4XW, UK Received 7 April 2008; received in revised form 19 May 2008; accepted 22 May 2008
*Corresponding author. Tel.: +44 2920 747747; fax: +44 2920 745439, URL: http://www.ctsnet.org/home/hluckraz.
Coronary arterio-venous fistulae are congenital or acquired cardiac anomalies whereby blood bypasses the myocardial capillary network. Involvement of all three coronary arteries is rare. There is a wide variation in the clinical presentation and the actual morphological malformations. The mainstay of treatment is either surgical or coil embolisation. Associated congenital malformations can be dealt with during the same surgical setting if required. We present a case of coronary arterio-venous fistula involving all three coronary arteries with associated atrial septal defect and acquired aortic regurgitation, all of which were dealt with surgically with good surgical outcome.
Key Words: Coronary artery fistula
Coronary arterio-venous fistula (CAVF) is a major coronary anomaly by Odgen's classification [1], being present in 0.002% of the general population and first described by Krause in 1865 [2]. The majority are congenital in origin, representing 0.4% of all cardiac malformations. CAVFs arise due to persistence of sinusoidal connections between the lumens of the primitive tubular heart that supply myocardial blood flow in the early embryologic period. Single fistulae are more common, ranging from 74 to 90%. Multiple fistulae are present in up to 16%, and fistulae originated from both coronaries in 5% [3, 4]. Most patients with CAVF are asymptomatic and found incidentally, although they may present with symptoms e.g. dyspnoea, fatigue, palpitations, angina or congestive cardiac failure in the sixth decade of life. We describe a case of multiple coronary-pulmonary fistulae with associated aortic valve regurgitation and atrial septal defect along with the surgical strategy including pericardial patch repair. Multiple cardiac pathologies in association with CAVF should not make surgical consideration prohibitive.
A 79-year-old lady with a history of syncope, dyspnoea and palpitations was referred to our surgical unit. Physical examination revealed continuous murmur in the pulmonary area and a pansystolic murmur at the apex. Trans-thoracic echocardiography confirmed atrial septal defect, bicuspid aortic valve (significant aortic regurgitation) and good left ventricular function. Coronary angiography demonstrated multiple coronary artery to pulmonary artery fistulae originating from the left and right coronary arteries and no flow limiting coronary artery disease. CT-scan confirmed the coronary artery fistulae with vascular malformation over the main pulmonary trunk (Figs. 1 and 2).
The patient underwent aortic valve replacement (Ultra Mosaic, 23 mm) and repair of the atrial septal defect with autologous pericardial patch. Intra-operatively, the coronary fistulae were confirmed to be originating from the right coronary (RCA), the left anterior descending (LAD) and the circumflex arteries and terminating into pulmonary artery with an intervening ‘fistula lake’. The ‘lake’ next to LAD was identified and the vessels entering were occluded with surgical clips as were the vessels coming from RCA. The aneurysmal sac was further obliterated using a larger surgical clip and Bioglue (Cryolife Inc., Georgia, USA). The pulmonary artery was opened and the entry points of all the fistulae to the pulmonary artery were closed with a patch of autologous pericardium sealed with Bioglue. The patient made an uneventful postoperative recovery and at one-year follow-up she was symptom free.
CAVF may be isolated (80%) or associated with congenital heart disease (20%) including Tetralogy of Fallot, atrial septal defect, patent ductus arteriosus, and ventricular septal defect. Nomenclature is based on a descriptive analysis of the vessel origin and the chamber of termination; Types A and B: Type A – proximal type, proximal coronary segment dilated to the origin of the fistula, distal end normal and Type B – distal type, coronary dilated over entire length, terminating as a fistula in the right side of the heart (end-artery type), proximal to fistula, regular branching of coronary [3]. Coronary ‘steal phenomenon’ is the primary patho-physiologic problem seen in CAVF without outflow obstruction. The mechanism is related to the diastolic pressure gradient and runoff from the high pressure coronary vasculature to a low resistance receiving cavity (e.g. pulmonary vasculature). Eventually, myocardium beyond the site of the fistula's origin is predisposed to increased risk of ischaemia, which is most frequently evident in association with increased myocardial oxygen demand during exercise or activity. Coronary artery dilatation, aneurysm formation, intimal ulceration, medial degeneration, intimal rupture atherosclerotic deposition, calcification, side-branch obstruction, mural thrombosis and rupture are the possible complications. There is a general agreement that symptomatic patients should be treated. The surgical obliteration of the fistula is the cornerstone of the surgical treatment. The mainstay of surgical therapy is ligation of the fistula, first described by Bjorck in 1947, and remains until now the most effective treatment. The ligation can be performed from within the cameral chamber or externally. Coronary artery grafting may be performed if coronary circulation has been compromised during the procedure. Alternative treatments include coil embolisation, amplatzer vascular plug or PTFE-coated stent deployment [4–8]. Anatomy of fistula and associated anomalies determine which technique should be chosen in each individual patient. The treatment of asymptomatic patients is controversial. Spontaneous closure has been described by Sherwood et al. in patients with small size CAVF [9]. There is no evidence to suggest that clinically silent CAVF diagnosed incidentally are associated with adverse clinical outcome. In our case, the presence of an aneurysm, three separate fistulous tracts and presence of aortic valve pathology, trans-catheter closure was contraindicated and hence the surgical technique was adopted. A pericardial patch was used within the pulmonary artery (PA) to ensure that all the multiple ‘openings’ within the PA were dealt with. Multiple cardiac pathologies in association with CAVF can be dealt with surgically successful outcome.
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Abstract
1. Introduction
-year experience at the Texas Heart Institute. Tex Heart Inst J 2002;29:299–307.