Methods: Between January 2008 and December 2012, 10 pediatric patients with CAVF were diagnosed in our institution. Demographic characteristics, symptoms and clinical findings, electrocardiographic, echocardiographic, angiographic findings, clinical courses after treatment were evaluated retrospectively.
Results: Congenital coronary arteriovenous fistulas were originated from the left coronary artery in six patients, right coronary artery in three patients, and the left circumflex artery in one patient. The drainage site of most CAVFs was the right ventricle, as expected and drainage to pulmonary artery (2) and right atrium (1) were other common anatomical locations, respectively. We followed the patients for the degree of shunt and for ongoing enlargement of coronary arteries for six months. Proper intervention for surgery or transcatheter occlusion was based on individual basis. Surgical ligation was the preferred management in three patients, as the anatomic features of fistulas were not appropriate for a catheter-based intervention. A catheterbased attempt for interventional closure was unsuccessful in a patient and underwent surgical ligation.
Conclusion: Our study results suggest that it is important to diagnose CAVF in childhood due to the high risk of complications seen in adulthood, particularly, such as heart failure, myocardial ischemia, infective endocarditis and arrhythmias.
There is a general consensus that symptomatic patients should be treated, although several reports have also demonstrated spontaneous closure.[6] Choosing between surgical intervention and transcatheter closure of the fistula is still tricky. However, trans-catheter closure seems reasonable in certain circumstances including fistulas with favorable anatomy (e.g. nontortuose vessel), the fistula in which distal portion is suitable for device closure, the fistula with narrowing distal end which avoid embolization, and the fistulas in which have no other indications for surgery.[6,7]
The ideal management strategy for patients with fistulas must be individualized.[8,9] Fistulas which originate from the proximal part of the coronary artery run the risk of a late giant aneurysm, dilation of the proximal coronary artery, and rupture. Therefore, in these cases, surgical or transcatheter closure seem reasonable at any age.[9,10] Transcatheter closure of fistulas has the advantage of less morbidity, a lower cost, and a shorter recovery time. In addition, there is no need for a thoracotomy and CPB.[9,11] In a report by Aydogan,[12] successful transcatheter embolization (coil/balloon) was performed on five children with CAVFs, and Karagöz et al.[8,9] reported that they closed a CVAF with a ductal occluder in a symptomatic infant and used a vascular plug in a newborn baby who had been prenatally diagnosed with a large CAVF.
Furthermore, a review of 174 patients with CAVF revealed that patients should undergo percutaneous or surgical closure in childhood, even if they are asymptomatic and have a small fistula size with a low Qp/Qs ratio, because of serious complications that can occur in adults.[13] However, based on recent reports, the optimal management for asymptomatic patients with low Qp/Qs ratio is still not clear.[1]
An examination of case reports reveals the diverse spectrum of CAVFs. For example, in one report, a 46-year-old man presented with chest pain and a fivemonth history of palpitation, but no significant shunt was detected on angiography.[14] Moreover, a fifteenmonth- old who presented with congestive heart failure and myocardial ischemia was diagnosed as having a CAVF. In our cohort, the only patient with any symptoms was a 14-year-old who presented with chest pain and fatigue during exertion. His past medical history was unremarkable. Electrocardiography revealed an ST segment depression in inferolateral derivations in this patient, and catheter angiography confirmed a fistula between the LCA and right ventricle. In addition, his Qp/Qs ratio of 1.47 was relatively low. Many reports have revealed that the majority of CAVF patients remain asymptomatic, especially during the first two decades of life,[5,13,15-17] whereas fewer have documented the occurrence of spontaneous closure.[6,18,19]
Consequently, when evaluating the relatively scarce number of patients in our study, we noted that a management strategy similar to the one used by Liang et al.[17] was employed. Their approach was to evaluate the symptomatic patients according to the degree of elevation of shunt magnitude and follow them up for six months to check for an enlarged persistency of dilatation of the coronary arteries. At that time, determined whether surgery or catheter-based occlusion is the best course of treatment is possible. With regard to asymptomatic patients, it might be prudent to closely follow them up via conservative methods until adulthood. Based on our findings, we suggest that multicenter, long-term, prospective studies be conducted to assess the optimal management strategy for patients with CAVFs.
Declaration of conflicting interests
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