Delayed appearance of left-to-right shunt following aortic valvular replacement. Report of two cases.

Two cases are presented in which a left-to-right shunt occurred late following aortic valvular replacement. In one patient a fistula was found between the aortic root and the right atrium and ventricle. In the second patient a defect developed in the membranous septum, allowing shunting from the left ventricle to both the right atrium and right ventricle.     

Unusual presentation of a huge right coronary artery fistula; Technical issues with device closure

Coronary artery fistula (CAF) is considered an embryologic persistence of primitive intra-trabecular spaces which allow the developing coronary artery to communicate with the other cardiac chambers or vascular structures. It is observed in 0.05–0.25% of coronary angiographic studies, most of which drain into a right heart chamber or into the pulmonary artery, while a congenital right coronary artery (RCA) into a left heart chamber is less frequent.⁵ In this study, we describe an unusual case treated by closure device in the right coronary artery fistula to the left ventricle, and associated literature is reviewed. A 40-year-old female presented with chronic cough, otherwise asymptomatic. Echocardiogram revealed unusual flow into the LV with mild LV dilatation. A 64 multi-slice CT scan confirmed the presence of a huge right coronary opening with a fistula into the left ventricle. The decision was to close this fistula with device through the RCA into the left ventricle. The management of this unusually large fistula is described with focus on technical issues with device closure.     

Spontaneous closure of congestional coronary fistula.

Coronary fistula is a rare congenital anomaly. In the pediatric population they are usually asymptomatic and this entity is suspected from the presence of a continuous murmur. They usually have a favorable course, although rarely they can complicate or occlude spontaneously. Early treatment is generally advised. The authors describe a case of spontaneous closure of congenital right coronary fistula draining into the right ventricle in a twelve-year-old boy and they comment on the therapeutic management of this anomaly.     

Diagnosis of a right coronary artery-left ventricular fistula by cross sectional and Doppler echocardiography.

A case of right coronary to left ventricular fistula was diagnosed by cross sectional and Doppler echocardiography. The origin and site of entry into the left ventricle of the enlarged right coronary artery were shown by cross sectional echocardiography. Diastolic flow was detected in the left ventricle by both pulsed and continuous Doppler echocardiography. The fistula was confirmed by cardiac catheterisation and was successfully closed at operation.     

Noninvasive diagnosis of coronary artery fistula by Doppler color flow mapping

Sixteen patients with coronary artery fistula proved by coronary angiography or surgery were studied using two-dimensional echocardiography and Doppler color flow mapping. The coronary artery fistula drained into the right atrium in 4 patients, the right ventricle in 2 and the pulmonary artery in 10. The dilated coronary artery was visualized in 7 of the 16 patients with a fistula, as compared with none of the 40 control subjects. These 7 patients included 5 of 6 patients with a fistula draining into the right atrium or right ventricle and only 2 of 10 patients with a fistula draining into the pulmonary artery. Abnormal flow signals in the dilated coronary artery were visualized with Doppler color flow mapping in five of these seven patients. Color flow imaging visualized abnormal flow signals with mosaic appearance in the pulmonary artery in eight patients, the right atrium in four and the right ventricle in two. The chamber in which abnormal signals were detected corresponded with the entry site of the fistula by angiography. Intraoperative imaging during surgical repair was needed in two cases to confirm ligation of all arteries feeding into the fistula network. In conclusion, Doppler color flow imaging is diagnostically useful to visualize shunt flows originating from the opening or exit of a coronary artery fistula. Furthermore, intraoperative use of this technique may provide confirmation of successful surgical ligation of the fistula.     

少见冠状动脉畸形18例

目的 :探讨 18例少见冠状动脉 (冠脉 )畸形特征及评价选择性冠脉造影在其诊断中的作用。方法 :对17例患者进行冠脉造影 ,13例患者进行手术纠正治疗。另 1例二维超声心动图误诊为动脉导管未闭 ,术中证实为右冠脉 -右室瘘。结果 :18例患者中共发现有 9种冠脉畸形 ,包括冠脉起源异常 2例 ;左冠脉回旋支缺如 1例 ;左、右冠脉 -肺动脉瘘 3例 ;左冠脉 -肺动脉瘘 3例 ;右冠脉 -右心房瘘 3例 ;右冠脉 -右室瘘 2例 ;右冠脉 -静脉瘘 1例 ;左冠脉 -右室瘘 1例 ;左冠脉 -左心室瘘 2例。其中 13例患者经手术治疗得到再证实。结论 :冠脉畸形是少见的先天性血管畸形 ,选择性冠脉造影是确诊的重要手段 ,需与先天性心脏病和冠心病心绞痛鉴别 ,术后效果良好。     

Fistula of the right coronary artery near the right ventricle. An echocardiographic study

A case of a 42-year-old woman with a right coronary artery fistula into the right ventricle is reported; she was asymptomatic and a continuous precordial murmur was found on physical examination. We discuss the usefulness and limitation of the Echo 2-D/Doppler findings, concerning this diagnosis, which was only definitively established by angiocardiography.     

Postoperative iatrogenic left coronaroventricular fistula. Demonstration by color two-dimensional Doppler

The authors report the case of an asymptomatic 67 year old patient, in whom, 6 years after aortic valve replacement, Doppler color flow mapping showed the presence of a coronary artery--left ventricular fistula. The normality of preoperative coronary angiography suggested that this fistula was created during peroperative left ventricular purging: the implantation of a needle through the right ventricle and interventricular septum. A iatrogenic lesion of a septal branch probably caused the communication between the left anterior descending artery and the left ventricle. Postoperative normalisation of the left ventricular end diastolic dimension, the absence of dilatation of the left main coronary on 2D echocardiography, the narrowness of the Doppler color jet and the absence of a significant end diastolic Doppler signal in the aortic isthmus indicated a fistula of small size and simple Doppler echocardiographic follow-up was decided upon.     

A large hemodynamically significant right coronary artery fistula to right ventricle: prenatal detection and progression

Prenatal diagnosis of a congenital coronary artery fistula between the right coronary artery and right ventricle was made by two-dimensional and color Doppler echocardiography in a pregnant woman at 37 weeks of gestation. The right ventricle and right coronary artery were dilated with a fistula between them. A boy was delivered at 39 weeks' gestation. Progression of the fistula was monitored by serial echocardiography postnatally. Because there were severe symptoms associated with the fistula, the infant had an operation 6 months after he was born. Four years of follow-up examination showed that the boy is in good clinical condition.     

Successful transcatheter coil embolization of coronary artery to left ventricular fistula associated with absent pulmonary valve with tricuspid atresia in early infancy.

Transcatheter coil embolization for coronary artery to left ventricular fistula was successfully performed in a neonate. At 30 weeks' gestation, fetal echocardiography showed a hypoplastic right ventricle with intact ventricular septum, absent pulmonary valve, tricuspid atresia, and marked distension of the right coronary artery. After birth, the neonate had congestive heart failure and the electrocardiogram showed myocardial ischemic changes in the left ventricular area. Aortography showed a dilated right coronary artery arising from the ascending aorta and draining into the left ventricle. Transcatheter coil embolization was carried out on the 9th day after birth. Since the procedure, no myocardial ischemic changes have been detected. Transcatheter coil embolization is a useful therapy for coronary artery fistula associated with myocardial ischemia.     

Improved visualisation of a coronary artery fistula by the "laid-back" aortogram.

The preoperative assessment of patients with a coronary artery fistula requires exact angiographic evaluation of the coronary artery anatomy. A technique of transvenous balloon occlusion angiography of the aorta is described. This can be applied easily in infants with a persisting foramen ovale. Combination of this technique with the so-called "laid-back" aortogram gave excellent visualisation of a fistula between the right coronary artery and the right ventricle. This technique is a major improvement in the angiographic assessment of infants with coronary artery abnormalities and normal cardiac connections.     

Echocardiographic findings in right coronary arterial-right ventricular fistula: Report of a neonate with fatal congestive heart failure

Coronary arteriovenous fistula is a rare cause of congestive heart failure in the newborn. We describe a 2 day old infant with a large fistula between the right coronary artery and right ventricle that caused fatal congestive heart failure. An echocardiographic study revealed a large right ventricle, paradoxical motion of the interventricular septum and an enlarged aorta, suggesting an unusual location of a left to right shunt. The diagnosis was established at cardiac catheterization when a large left to right shunt was found at the ventricular level; the fistula was visualized by angiography.     

An elephant trunk appearance—what is the diagnosis?

Coronary cameral fistula is a rare congenital cardiac abnormality. An 18‐year‐old boy presented with features of right heart volume overload. Clinical examination was suggestive of hyperdynamic circulation with continuous murmur in precordium. 2D echocardiography showed dilated right coronary artery, and 3D echocardiography added information in tracing the track of the fistula which was consistent with the diagnosis of right coronary cameral fistula draining into the right ventricle. Coronary angiograms revealed an unusually dilated right coronary artery giving the appearance of an “elephant trunk” and with a fistulous tract into the right ventricle. Considering the higher risks of surgery in such difficult cases, we performed a successful transcatheter closure of the fistula using an Amplatzer vascular plug.     

Prenatal diagnosis of fetal isolated right coronary artery to left ventricle fistula

Coronary artery fistula (CAF) is a rare malformation and is seldom reported during pregnancy. Right coronary artery fistula commonly drains into the right ventricle, right atrium, or pulmonary artery. We describe here a rare case of fetal CAF draining into the left ventricle using cross‐sectional and color Doppler echocardiography. We also summarized our experience in the diagnosis of this uncommon malformation, in which tracing the origin, course, and outlet of the abnormal intra‐cardiac flow played a key role.     

Acquired coronary artery fistula after open heart surgery for congenital heart disease

BACKGROUND: Most coronary artery fistulas were reported as congenital. Acquired coronary artery fistula occurring after cardiac surgery has rarely been reported. METHODS: From 1998 to 2003, 10 patients with coronary artery fistula detected by echocardiography after open heart surgery for congenital heart disease were included. Their ages ranged from 2 months to 41 years (median 4.2 years). The underlying heart disease was tetralogy of Fallot in five patients, ventricular septal defect in three, double chamber right ventricle in one, and transposition of the great arteries with ventricular septal defect in the remaining one. RESULTS: Of these 10 patients, the coronary artery fistula originated from the left coronary artery in four, right coronary artery in two, and unknown origin in the remaining four. The coronary artery fistula drained into the right ventricle in nine and into the left ventricle in the remaining one. The incidence of acquired coronary artery fistula after open heart surgery for congenital heart disease was 0.44% (8/1832). The identified risk factors for acquired coronary artery fistula were reoperation and right ventricular muscle resection in ventricular septal defect. After follow-up for 0.5-12 years (mean 4.1+/-3.3 years), the coronary artery fistula persisted, but neither symptoms nor significant left-to-right shunt was noted. CONCLUSIONS: Acquired coronary artery fistula is a rare complication after cardiac surgery. Reoperation and resection of right ventricular hypertrophic muscle increase the risk of this complication. Although shunt flow did not increase during follow-up, the significance of acquired coronary artery fistula needs further investigation.     

Imaging of multiple coronary artery fistulas to right ventricle by transthoracic and transesophageal echocardiography.

A 20-year-old woman presented with extremely rare multiple coronary artery fistulas with left circumflex and right coronary arteries as the feeding vessels and two distinct sites of drainage into the posterior wall of the right ventricle near the apex in close proximity. The large left fistula was well depicted by transthoracic echocardiography, whereas the transesophageal approach better delineated part of the smaller right fistula.     

Coronary ventricular fistulas in patients with heart transplants

In detailed angiographic follow-up examinations in patients after heart transplantation, coronary artery-ventricular fistulas were observed in two of ten patients. In one patient, in the first postoperative year, 19 right ventricular biopsy procedures were performed obtaining a total of 71 tissue specimens and one year after transplantation, additionally, three specimens were obtained from the left ventricle. Coronary angiography demonstrated a fistula from the first anterior ventricular branch of the right coronary artery into the right ventricle (Figure 1) as well as a second smaller fistula between from a septal perforator of the left anterior descending artery into the left ventricle. At follow-up angiography two months later, the fistulas were unchanged in site and extent (Figure 2). Angiographically there was diffuse hypokinesis of the right ventricle and moderate tricuspid regurgitation. In the second patient, in the first year after transplantation, 20 right ventricular biopsy procedures were performed obtaining a total of 80 tissue specimens. One year after transplantation coronary angiography showed a fistula between a septal perforator of the left anterior descending artery into the right ventricle (Figure 3) which, two months later, was unchanged in morphology and, as in the first case, the size of the shunt was small. Angiographically, there was moderate tricuspid regurgitation and apical hypokinesis of the left ventricle. If a relationship between the fistulas and biopsies is postulated, from a total 196 biopsy procedures obtaining 748 tissue specimens, the rate of this complication would be calculated at 1.5%. It cannot be ruled out, however, that the fistulas had been congenitally present in the donor hearts.(ABSTRACT TRUNCATED AT 250 WORDS)     

Prenatal detection and progression of right coronary artery to right ventricle fistula.

Prenatal diagnosis of a congenital coronary artery fistula between the right coronary artery and right ventricle was made by colour Doppler echocardiography at 20 weeks of gestation. Its progression was monitored by serial echocardiography throughout pregnancy and postnatally.     

Ventricular dysplasia. Nosology and sudden death

The arrhythmia generating dysplasia of the right ventricle is a congenital anomaly suggesting a hereditary substratum. It usually manifests itself as follows: a young patient, with recurrent episodes of ventricular tachycardiac. There is no "pathognomonic sign" of dysplasia. The latter may only be determined by anatomical examination of the heart, macroscopically as well as microscopically. The most difficult differential diagnosis concerns minor or localized forms of dilated idiopathic myocardiopathy or sequelae of myocarditis with ventricular tachycardia originating in the right ventricle. It seems, currently, more appropriate to talk about "arrhythmia generating syndrome of the right ventricle" rather than arrhythmia generating dysplasia of the right ventricle, in these particular cases. The arrhythmia generating syndrome of the right ventricle could cause a sudden death in these young patients, especially during a violent physical exercise or practise of sports. The current nosological classification is based on an experience acquired by comparing a few isolated cases. The search for late potentials by summation-mean measurements and nuclear magnetic resonance could provide a new diagnostic approach.     

Transcatheter occlusion of a large coronary arterial fistula with new detachable platinum microcoils.

We report a 17 year old boy with a large fistula from the right coronary artery to the right ventricle. The fistula consisted of two major branches. The smaller branch could be embolised with three detachable platinum microcoils without problems. The high flow of blood in the larger branch, however, prevented conventional occlusion using coils. Interventional occlusion of this branch was achieved in a second attempt. Following creation of an arterio-venous wire-loop, we advanced a balloon catheter into the distal end of the fistula. Under temporary occlusion of the fistula, complete closure was achieved by delivery of 7 detachable platinum microcoils. Thus, even large coronary arterial fistulas can be closed safely with these new platinum detachable microcoils. The procedure, however, requires temporary control of the flow of blood by balloon occlusion.