Coronary Artery-Left Ventricular Fistula with Giant Right Coronary Aneurysm: A Case Report and Literature Review.

Right coronary artery-left ventricular (RCA-LV) fistula with associated giant right coronary artery aneurysm (CAA) is an extremely rare cardiac condition. This case study presents a patient with a large left ventricle (LV) and a giant right CAA with a maximal inner diameter of approximately 56.6 mm and an inner diameter of approximately 22 mm at its communication with the left ventricle. The patient underwent surgical management, involving suturing of the proximal end of the CAA and coronary artery bypass grafting (CABG). RCA-LV fistula with a giant right CAA may involve serious complications, such as thrombosis, rupture, and heart failure. Therefore, it is necessary to establish effective management strategies for this condition. Although this case is not unique, it serves as an illustrative example of the implementation of a classic surgical treatment method.

Long-Term Follow-Up of Pediatric Patients After Congenital Coronary Artery Fistula Closure.

The surgical closure of congenital coronary artery fistulas (CAF) is associated with excellent immediate outcomes. Few studies have investigated the long-term prognosis in patients who have undergone surgery for the closure of CAF or differentiated among types of CAF or types of surgical procedures. In this study, we performed clinical examinations and computed tomography angiography (CTA) to characterize outcomes after CAF closure in pediatric patients. The medical records of 79 pediatric patients who underwent surgical closure of CAF were retrospectively reviewed. The median age of the patients included in the study at the time of surgery was 3.4 years (range 0.2 to 15.3 years). The patients had been followed up for 11 years (range 1 to 17 years) with electrocardiography, echocardiography, and coronary CTA. There were 67 medium-to-large CAF and 12 small CAF. Twenty-six (32.9%) CAF arose from the branch coronary artery (proximal type); the others arose from the parent coronary artery (distal type). The surgical procedure included endocardial closure in 16 cases, epicardial distal ligation in 51 cases, epicardial proximal and distal ligation in 12 cases. There was no instance of perioperative death among the cases included in the study. Twenty-eight patients were treated with antiplatelet medication postoperatively. No patient required re-operation during the follow-up period. Coronary thrombi were detected in 27 patients (34.2%). There was no instance of myocardial ischemia related to thrombosis. Among the patients with thrombosis, 26 had medium-to-large CAF (96.3%), and 23 had distal-type CAF (85.2%). Average age at surgery was higher among the patients with thrombosis than among the patients without thrombosis (7.4 years vs. 3.3 years, t = 5.509, P = 0.000). Among the patients with distal-type CAF, thrombosis was more common among the patients treated with ligation than treated with endocardial closure (41.5% vs. 16.7%, χ2 = 3.742, P = 0.043). There was no difference in risk for thrombosis between the patients who did vs. did not receive antiplatelet therapy (P = 0.436). The most common complication after CAF closure was thrombosis. Increased risk for thrombosis was associated with large fistulae, distal-type CAF, and older age at presentation. Antiplatelet treatment did not appear to decrease the risk of thrombosis. Among patients with distal-type CAF, risk for thrombosis was lower among patients treated with endocardial closure, compared with patients treated with epicardial ligation.

Endovascular Transjugular Occlusion of Congenital Intrahepatic Portosystemic Venous Shunt Using Simultaneous Fluoroscopy and Transabdominal Ultrasound Guidance: Report of 2 Cases.

Congenital intrahepatic portosystemic venous shunts (CIPVS) are rare anomalies that can be detected before birth or in early infancy or later in life. Symptomatic shunts are treated as they carry high risk of complications like hepatic encephalopathy. Various treatment options include surgery, endovascular embolization, and percutaneous closure devices. We treated 2 infants with CIPVS successfully by endovascular embolization of the shunt using vascular plug through transjugular route. Transabdominal ultrasound guidance in addition to fluoroscopy was used at the time of vascular plug placement. We emphasize that the use of transabdominal ultrasound during endovascular occlusion enhances the safety and technical success rate.

Congenital aneurysmal right coronary artery with a fistula to the left atrium in an adult.

BACKGROUND: Congenital coronary artery fistula in association with aneurysm of the involved coronary artery in adults is rare. Moreover, the right coronary artery- left atrial fistula is also uncommon. Most of the cases are asymptomatic. However, symptomatic patients need therapeutic interventions. The potential complications associated with this anomaly are life-threatening, therefore, there is a need to explore more on differential diagnosis, investigations, management strategies and prevention of complications. CASE PRESENTATION: We present herewith a 26-year-old male patient with symptoms of chest pain and dyspnea. He was diagnosed with aneurysmal dilatation of the right coronary artery in its entire course which terminated as a fistulous communication into the left atrium. The closure of the fistula was done using autologous pericardial patch under cardiopulmonary bypass. Currently, the patient is being followed up after surgery and receiving anticoagulants. CONCLUSION: The advancement in the diagnostic imaging modalities have made it possible to find similar abnormalities more frequently. Due to rare nature of this anomaly, there is a need to explore and discuss management strategies that include medical management, surgical intervention or percutaneous interventions for a successful outcome.

Outcomes after Balloon Pulmonary Valvuloplasty for Critical Pulmonary Stenosis and Incidence of Coronary Artery Fistulas.

Outcome data for neonates with critical pulmonary stenosis (PS) is limited. We aimed to review the outcomes after balloon pulmonary valvuloplasty (BPV) for neonates with critical PS at our institution. All neonates with critical PS who underwent BPV from 1990 to 2017 were included. A total of 44 neonates underwent BPV for critical PS. Nonright ventricular dependent coronary artery fistulas was seen in 6/44 (13.6%) patients. Tricuspid valve z-scores were -1.9 (interquartile range [IQR] -3.04, -0.48) in those with coronary artery fistulas as compared with -0.27 (IQR -0.5, 0.8) in those without (p = 0.03). Fifteen of forty-four subjects (34.1%) patients underwent reintervention with 10 patients (22.7%) requiring an alternate source of pulmonary blood flow (3 patients subsequently underwent right ventricular overhaul, 2 underwent Glenn operations, and 1 underwent repeat BPV). Five patients underwent reintervention for right ventricular outflow tract obstruction. Pulmonary valve annulus z-score was significantly smaller in those who needed reintervention -2.4 (IQR -2.9 to -0.95) versus -0.59 (IQR -1.3, -0.15); p = 0.02. At a median follow-up of 8.2 (IQR 3.4 to 13.1) years, moderate or severe pulmonary regurgitation was seen in 22/42 (53.7 %) patients with biventricular circulation, 3 requiring pulmonary valve repair/replacement. In conclusion, coronary artery fistulas occur in a significant number of patients with critical pulmonary stenosis, occurring more frequently in patients with small tricuspid valves. Reintervention is required for 1/3 of patients. Patients with small pulmonary valve annuli are more likely to undergo reintervention for right ventricular outflow tract obstruction. Significant pulmonary regurgitation is common and may require eventual pulmonary valve replacement.

[Late discover of a coronaro-pulmonary fistula]. / Une fistule coronaro-pulmonaire de découverte tardive.

Anomalies of the coronary arteries mainly concern a pediatric population, bringing together a wide range of defects. In adults, the evolution is linked to the hemodynamic consequences of fistula. Several therapeutic options have been proposed such as surgery or embolization. We report the case of a 55 years old patient addressed because of dyspnea secondary to aortic insufficiency. The preoperative assessment shown the coexistence of coronary abnormality corresponding to a coronaro-pulmonary fistula. This type of coronary anomaly is rarely described in the adult population, because of its consequences secondary to the closure of the foramen ovale, resulting in angina symptoms in childhood. Without treatment, mortality from this type of malformation is important (90%).

A rare, giant coronary artery ectasia coexisting with a coronary artery fistula in an older infant.

Coronary artery fistula with giant coronary artery ectasia is a rare abnormal CHD. Multidetector CT is useful for the diagnosis. Early diagnosis and surgery are recommended.

Focal Nodulary Hyperplasia of the Liver Due to Congenital Portosystemic Shunt: A Rare Condition Mimicking Hepatocellulary Carcinoma.

Congenital portosystemic shunts are rare vascular malformations that lead to several complications including liver tumors, pulmonary hypertension, and metabolic encephalopathy. We describe a rare case of a 17-year-old girl with an extrahepatic portosystemic shunt presenting recurrent syncope episodes and a liver mass mimicking hepatocellulary carcinoma.

Outcomes of surgical repair of pediatric coronary artery fistulas.

OBJECTIVE: Isolated congenital coronary artery fistula is a rare condition, and the surgical experience for treating this condition is limited. METHODS: This was a retrospective review of 47 patients who underwent surgical repair of isolated congenital coronary artery fistula from January 2001 to March 2015. RESULTS: All but 1 patient presented with no symptoms. The median age at operation was 3.9 years (range, 0.4-15.2 years), and the median weight was 15.2 kg (range, 6.3-77.0 kg). Right coronary artery to right ventricle fistula was the most common pattern, which was present in 16 patients. Epicardial ligation without cardiopulmonary bypass was used to treat 4 patients, whereas cardiopulmonary bypass was used in the remaining 43 patients. Surgical techniques included transcoronary closure in 20 patients, transcameral or transpulmonary closure in 20 patients, a combination of both transcameral and transcoronary closure in 2 patients, and a conversion from transcameral closure to epicardial ligation in 1 patient. There were no deaths during our study period. Injury to the mitral valve occurred in 1 patient. Residual shunt was observed in 8 patients, 2 of which closed spontaneously during the follow-up. Extracardiac repair was associated with a higher risk of recanalization (P = .004). Freedom from recanalization was 89.4%, 85.1%, 83.0%, and 83.0% at discharge, 6 months, 1 year, and 5 years postsurgery, respectively. Two patients required transcatheter closure for residual shunts. CONCLUSIONS: Surgical repair of isolated congenital coronary artery fistula in pediatric patients can be performed with low mortality and morbidity. Careful evaluation after surgery is necessary to monitor the occurrence of residual shunt.

Peratrial Device Closure of a Congenital Coronary Artery Fistula through a Right Parasternal Approach: Innovative Use of Available Technology.

Current treatments for congenital coronary artery fistulas (CAFs) include surgical obliteration and transcatheter occlusion. However, surgical techniques involve significant trauma. Transcatheter occlusion is performed under fluoroscopy and angiography, in which radiation injury is inevitable. We present a patient, with a CAF from the left coronary artery to the right atrium, who underwent peratrial device closure of the CAF with a right parasternal approach under transesophageal echocardiography guidance. Complete occlusion was achieved by a symmetric ventricular septal occluder. We suggest that peratrial device closure of a congenital coronary artery fistula through a right parasternal approach may be a safe and effective option.

Congenital portosystemic shunts with and without gastrointestinal bleeding - case series.

BACKGROUND: The clinical presentation of congenital portosystemic shunt is variable and gastrointestinal bleeding is an uncommon presentation. OBJECTIVE: To describe the imaging features of congenital portosystemic shunt as it presented in 11 children with (n = 6) and without gastrointestinal bleeding (n = 5). MATERIALS AND METHODS: We performed a retrospective study on a clinical and imaging dataset of 11 children diagnosed with congenital portosystemic shunt. RESULTS: A total of 11 children with congenital portosystemic shunt were included in this study, 7 with extrahepatic portosystemic shunts and 4 with intrahepatic portosystemic shunts. Six patients with gastrointestinal bleeding had an extrahepatic portosystemic shunt, and the imaging results showed that the shunts originated from the splenomesenteric junction (n = 5) or splenic vein (n = 1) and connected to the internal iliac vein. Among the five cases of congenital portosystemic shunt without gastrointestinal bleeding, one case was an extrahepatic portosystemic shunt and the other four were intrahepatic portosystemic shunts. CONCLUSION: Most congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was uncommon, but the concomitant rate of gastrointestinal bleeding with this type of shunt was high.

Early Surgical Repair of the Coronary Artery Fistulae in Children: 30 Years of Experience.

BACKGROUND: Congenital coronary artery fistula (CAF) is a rare anomaly that may lead to the development of early heart failure or late complications. We reviewed our experience and outcomes of surgically corrected CAF. METHODS: From June 1982 to October 2012, 13 children aged between 3 days and 5.2 years (median 13.9 months) underwent repair of CAF. Four patients (30.8%) presented with congestive heart failure. Both coronary arteries were affected with equal incidence. All patients underwent preoperative cardiac catheterization. Repair was undertaken with cardiopulmonary bypass in 10 patients (76.9%), and 5 patients (38.5%) had concomitant repair of associated cardiac lesions. Seven patients (53.8%) underwent epicardial closure, and in 6 patients (46.2%), transcoronary/endocardial closure was used. RESULTS: There were no early or late deaths. Transient myocardial ischemia occurred in 2 patients (14.4%). One patient (7.7%) underwent reoperation (aortic valve repair) for a bicuspid aortic valve, 22 years after initial surgery. Follow-up data were complete for 12 patients (92.3%), with a median follow-up of 6 years (range, 1 month to 31 years). All patients were in New York Heart Association class I with no symptoms. There were no long-term cardiovascular sequelae, and no patient required coronary reintervention. CONCLUSIONS: Early surgical repair of CAF in children can be performed safely and carries an excellent long-term prognosis.

[Pediatric case of congenital coronary artery fistula; surgical result and late changes in coronary artery aneurysm].

Congenital coronary artery fistula is an uncommon heart anomaly involving the coronary arteries. We report here a case of a 4-year-old boy who had a coronary fistula from the right coronary artery to the right ventricle, with a coronary aneurysm. He was asymptomatic, but the calculated ratio of pulmonary blood flow to systemic blood flow was shown to be high [pulmonary flow (Qp)/systemic flow(Qs)=1.78]. The coronary angiography showed that the right coronary artery was dilated beginning at the ostium and had an aneurysm at the acute marginal portion. A large spherical aneurysm approximately 20 mm in diameter was found to have been connected with coronary fistula opening into the right ventricle. Surgical repair by closure of the fistula under direct vision, partial resection and suture closure of the aneurysm was performed. Plication of the proximal portion of the right coronary artery was not performed, and the diffusely dilated artery was left untouched. After this operation, he recovered well under anticoagulant treatment with warfarin and aspirin. Postoperative angiography was performed 17 months after the surgery to evaluate morphological changes in the coronary artery. The angiography confirmed the closure of the fistula and the regression of coronary artery dilatation.

Congenital coronary artery fistulae: a rare cause of heart failure in adults.

Coronary artery fistulae are uncommon, reported in 0.25% of patients undergoing coronary angiography. Two patients with congenital coronary artery fistula and coronary artery disease who presented with symptoms of exacerbated congestive heart failure out of proportion to their atherosclerotic burden were successfully treated by epicardial fistula ligation and coronary artery bypass grafting with marked improvement in functional status.

Interventional closure of RPA-to-LA communication in an oligosymptomatic neonate.

UNLABELLED: Direct communication between the right pulmonary artery (RPA) and the left atrium (LA) is a very rare cardiac malformation. Clinical presentation of RPA-to-LA communication depends on the size of the communication, the amount of right-to-left shunt, the patient's age, and pulmonary vascular resistance. Patients with small communications usually present oligosymptomatic and are diagnosed at an older age. A delay of diagnosis bears the risk of severe complications and needs to be prevented by proper work-up of oligosymptomatic neonates. Treatment of RPA-to-LA communications used to be performed by surgical closure, and the interventional approach has only been established as a less invasive alternative in recent years. CONCLUSION: Although patients with small RPA-to-LA communications usually present oligosymptomatic, early diagnosis and treatment is essential to prevent life-threatening complications.

[Congenital coronary artery fistulas: clinical and therapeutic consideration]. / Kongenitalne koronarne arterijske fistule: klinicko i terapijsko razmatranje.

A coronary artery fistula is a link between one or more coronary arteries with another heart cavity or a segment of systemic or pulmonary circulation. Arterial blood from a coronary vessel enters another segment via myocardial capillary bed. These are very rare anomalies which constitute approximately 0.2 - 0.4% of all congenital heart defects. Still, they are clinically significant if they are of medium or large size and are manifested with a series of clinical symptoms such as angina pectoris, arrhythmias, myocardial infarction, endocarditis, progressive dilatation, heart failure and cardiomyopathy, pulmonary hypertension, thrombosis of the fistula and formation of aneurysms with possible ruptures. We present six patients with a coronary arterial fistula, their history, diagnostic procedures and outcomes. Therapeutic closure of coronary artery fistulas is recommended in all symptomatic, but also in asymptomatic patients, if there are significant roentgenographic, electrocardiographic and other abnormalities. In recent times transcatheter closure of coronary fistulas has become a possible alternative to surgery and is becoming increasingly used thanks to improved diagnostic possibilities and technology. If possible, interventional closure of fistulas is precisely the method preferred in pediatric patients. The choice of method depends on the anatomy of the fistula, presence or absence of additional defects, and on the experience of an interventional cardiologist or a heart surgeon. If performed well, the effects of both methods are good. This paper presents two children with a fistula between the right coronary artery and the right ventricle (RV), one child with a fistula between LAD and RV, one child with a fistula between the main tree of the left coronary artery (LCA) and RV, one child with a fistula between LCA and the right ventricular outflow tract (RVOT), and one child with a fistula between LCA and the right atrium (RA). The last one (LCA-RA) is not described in the latest classification of anomalies of coronary blood vessels in children based on MSCT coronarography, so we consider our presentation to be a contribution to the new classification. Along with the descriptions of fistulas and presentations of interventional and cardiosurgical interventions, we are also presenting a rare case of spontaneous closing of the fistula within the first six months and of a reopening of the fistula between the right coronary artery and the right ventricle after six years.

Management of an unusual left coronary artery to right ventricular fistula in a neonate.

Congenital coronary arterial fistula is a rare cause of congestive heart failure in neonates. We describe a neonate who developed early symptoms because of an unusually large left coronary artery to right ventricular fistula. Computed tomography and coronary angiography delineated anatomy and facilitated successful surgical closure.

Successful transcatheter patent ductus arteriosus occluder embolization of a congenital left coronary artery aneurysm and fistulas draining into the right atrium.

Coronary artery aneurysms and fistulas are very rare congenital anomalies. They occur in 0.2%-0.4% of all congenital heart diseases. In this article, we report a case of a 39-year-old girl with a left coronary artery aneurysm and fistula draining into the right atrium. An intervention is successfully achieved by transcatheter occluder embolization using a patent ductus arteriosus (PDA) occluder.

Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula.

We report the case of a child with Abernethy malformation with an anomalous connection between the portal vein and the coronary sinus. After 30 months of close follow-up, the patient developed hepatoblastoma, a previously documented complication of the Abernethy malformation. This case reports a unique variant of Abernethy malformation and documents the first reported case of hepatoblastoma in a patient with type 2 Abernethy malformation.