S.A.M. Said

Solitary coronary artery fistulas: a congenital anomaly in children and adults. A contemporary review.

Congenital solitary coronary artery fistulas (CAFs) in adults are uncommon anomalies, which by themselves may resemble the whole spectrum of cardiac presentations from asymptomatic behavior to life-threatening and catastrophic events with syncope or shock and even sudden death. It may take decades to collect a reasonable series of patients in adults and children. From the literature between 1993 and 2004, 236 patients with CAFs were considered for evaluation. The present review is intended to assist cardiologists who are unfamiliar with congenital CAFs in adults by suggesting clues for decision making regarding diagnosis and management. Dyspnea and chest pain represented a frequent 91/128 (71%) clinical symptom in CAFs in adults while in the pediatric age group the majority were silent 105/133 (79%) and dyspnea and chest pain accounted for only 8% of the symptoms. The diagnostic modalities were mainly cardiac catheterization and coronary angiography. On the other hand, in the pediatric patients, echocardiography and coronary angiography mainly guided the diagnosis. Regarding treatment strategy in the reviewed subjects, percutaneous transluminal embolization was performed in 18% of the pediatric and in only 5% of the adult subjects. Surgical ligation (46% vs. 38%) and conservative medical strategies (36% vs. 24%) were reported in both pediatric and adult groups. Presentations of CAFs vary considerably in both groups. These differences include the diagnostic modalities, spontaneous closure, spontaneous rupture, and management. From this review, it seemed that--but it may be biased--surgical ligation remains the major mainstay for closure of CAFs in adult and pediatric populations. Recommendations are necessary for antibiotic prophylaxis and antiplatelet and/or anticoagulant therapy for prevention of endocarditis and thrombotic events in patients with CAFs associated with coronary artery dilatation or aneurysmal formation of the fistulous tract.

Female Gender and the Risk of Rupture of Congenital Aneurysmal Fistula in Adults

AIMS To delineate the risk factors for rupture of congenital aneurysmal fistulas in adult patients. METHODS We conducted a literature search of the Medline database using Pubmed search interface to identify reports dealing with rupture of congenital aneurysmal fistulas in an adult population. The search included the English and non-English languages between 1963 and 2005. RESULTS Fourteen adult patients (12 females) with serious and life-threatening complications secondary to aneurysmal fistulas were reported. Mean age was 62.9 years. The ethnic origins of these 14 patients were 9 Asian and 5 Caucasian. Most patients have had no other cardiac malformations. Five patients had a history of hypertension. One patient was asymptomatic. In 13 symptomatic patients, the clinical presentation was cardiac tamponade, pericardial effusion, syncope, heart failure, chest pain, dyspnea, fatigue, distal thromboembolic events with infarction, shock, and/or sudden death. Aneurysmal fistulas were identified in 10 patients; of these 6 were of the saccular type. Rupture occurred in 9 patients (8 females and 1 male). Eleven patients were treated surgically with 1 late death. Two male subjects experienced sudden unexpected cardiac death. CONCLUSION Rupture of congenital aneurysmal fistulas occurred more often in females. Identified risk factors for rupture, hemopericardium, tamponade, and death were among others saccular aneurysm, Asian ethnic race, origin of the aneurysmal fistulas from the left coronary artery and a history of hypertension may play a role. In this article, we present a literature review of congenital aneurysmal fistulas associated with or without rupture and a case report of a woman with unruptured aneurysmal fistula.

Feasibility of Cardiovascular Magnetic Resonance of Angiographically Diagnosed Congenital Solitary Coronary Artery Fistulas in Adults

OBJECTIVE To evaluate the use of cardiovascular magnetic resonance (CMR) to visualize angiographically-detected congenital coronary artery fistulas in adults. METHODS CMR techniques were used to study 13 patients, recruited from the Dutch Registry, with previously angiographically diagnosed fistulas. RESULTS Coronary fistulas were detected in 10 of 13 (77%) patients by CMR and, retrospectively, in two (92%) more. In 93% of these, it was possible to determine the origin and the outflow site of the fistulas. Cardiovascular magnetic resonance allowed demonstration of dilatation of the fistula-related coronary artery in all cases. Tortuosity of fistulas was detected in all visualized patients. Uni-or bilaterality of fistulas as seen on CAG was proven on CMR in all patients. Flow measurement could be performed in 8 patients. A fairly good correlation (r = 0.72) was found between angiographic (mean 6.2 mm, range 1-16) and cardiovascular magnetic resonance (mean 6.3 mm, range 3-15) measured fistulous diameters. CONCLUSIONS Cardiovascular magnetic resonance of congenital fistulas with clinical significant shunting is feasible and can provide additional physiological data complementary to the findings of conventional coronary angiography.

Congenital Circumflex Artery-coronary Sinus Fistula in an Adult Female Associated with Severe Mitral Regurgitation and Myelodysplasy—Case Report and Review of the Literature

BACKGROUND Coronary artery fistulas are uncommon anomalies. They occur in 0.1-0.2% of patients undergoing coronary arteriography. The origin of the fistulas is the right coronary artery followed by the left anterior descending and lastly by the circumflex artery (17%). Termination into the right heart side occurs in 90% of cases. Termination into the coronary sinus is rare in 3% of cases. Circumflex artery-coronary sinus fistulas are even rarer. DESIGN A single case report and literature review between 1993 and 2007. RESULTS We describe a 76-year-old female, who was analyzed for dyspnea on exertion (DOE) and chronic fatigue, with known myelodysplastic syndrome and an aneurysmal circumflex coronary artery-coronary sinus fistulous connection associated with severe mitral regurgitation. Mitral valve replacement using a bioprosthesis was performed as well as ligation of the fistula. The postoperative course was complicated with cardiac tamponade, which was successfully drained. CONCLUSION Our patient presented with chronic fatigue and DOE and was found to have a coronary artery fistula and severe mitral regurgitation associated with known myelodysplasia. Conventional coronary angiography failed to demonstrate the entire fistula characteristics (origin, pathway, and outflow). Multidetector computed tomography was complementary to demonstrate the complex anatomy of the fistula. The fistula was surgically ligated in combination with mitral valve replacement. She remains well.

Congenital and Atherosclerotic (Acquired) Coronary Artery Aneurysms: Coronary Angiographic and Morphologic Observations in 10 Patients

Coronary angiographic observations in 10 patients with coronary artery aneurysms (CAAs) are reported. Four patients had atherosclerotic (acquired) and six had congenital CAAs. The mean age of patients with acquired CAAs was higher (64.7 years) compared with the congenital group (53.2 years). Ipsilateral myocardial infarction (MI) occurred in three of four patients with acquired CAAs but MI was not located on the same side as the aneurysm-bearing coronary artery (contralateral) in two patients with the congenital variety. In the patients with congenital CAAs, an aneurysm predilection site was observed in the proximal portion of the aneurysm-bearing vessel. Dual and multiple aneurysms were more common in the acquired CAAs. Although congenital CAAs were sizeable, the small-sized atherosclerotic CAAs developed complications more frequently. During an average follow-up of 7.9 years, only one patient died of a noncardiac cause and another developed recurrent uncomplicated non-Q wave lateral MI. Both subjects had atherosclerotic CAAs. In our series, no rupture or sudden death occurred. Coronary artery bypass grafting (CABG) was performed in three patients with acquired and in one patient with congenital CAAs. In the latter patient, simultaneous ligation of the congenital aneurysm associated with a coronary arteriovenous fistula was performed. Regarding antiplatelet and anticoagulant policy for the whole group, three patients were on aspirin, four were on acenocoumarol, and in another three subjects with congenital CAAs, a medical regimen was followed. Larger series, however, are required in order to elucidate further angiographic characteristics of acquired vs congenital CAAs.

Incidental congenital coronary artery vascular fistulas in adults: Evaluation with adenosine-13N-ammonia PET-CT

AIM To assess the functionality of congenital coronary artery fistulas (CAFs) using adenosine stress 13N-ammonia positron emission tomography computed tomography (PET-CT). METHODS Congenital CAFs were incidentally detected during coronary angiography (CAG) procedures in 11 adult patients (six males and five females) with a mean age of 64.3 years (range 41-81). Patients were collected from three institutes in the Netherlands. The characteristics of the fistulas (origin, pathway and termination), multiplicity of the origins and pathways of the fistulous vessels were assessed by CAG. Five patients underwent adenosine pharmacologic stress 13N-ammonia PET-CT to assess myocardial perfusion and the functional behavior of the fistula. RESULTS Eleven patients with 12 CAFs, 10 unilateral and one bilateral, originating from the left anterior descending coronary artery (n = 8), right coronary artery (n = 2) and circumflex (n = 2). All fistulas were of the vascular type, terminating into either the pulmonary artery (n = 11) or coronary sinus (n = 1). The CAG delineated the characteristics of the fistula (origin, pathway and termination). Multiplicity of the origins and pathways of the fistulous vessels were common in most fistulas (8/12, 67% and 9/12, 75%, respectively). Multiplicity was common among the different fistula components (23/36, 64%). Adenosine pharmacologic stress 13N-ammonia PET-CT revealed normal myocardial perfusion and ejection fraction in all but one patient, who showed a reduced ejection fraction. CONCLUSION PET-CT may be helpful for assessing the functional status of congenital CAFs in selected patients regarding clinical decision-making. Studies with a larger patient series are warranted.