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.

Congenital coronary artery fistula in pediatric patients: transcatheter versus surgical closure.

OBJECTIVES: Transcatheter closure (TCC) and surgical closure (SC) are the two main approaches for congenital coronary artery fistula (CCAF), but data on the comparisons of the efficacy and safety of these two approaches are limited. METHODS: We retrospectively reviewed pediatric patients with CCAF in Guangdong Cardiovascular Institute between January 2002 and December 2017. Patients who were qualified into our criteria were included into final analysis. The rate of successful closure and complications during hospitalization and at follow-up were compared between SC and TCC groups. RESULTS: In total, 121 pediatric patients (male, n = 69; female, n = 52) with CCAF were divided to TCC (n = 63) and SC groups (n = 58) according to the indications. The mean age was 5.3 ± 1.4 years. The baseline characteristics of these two groups were similar except for the fistula anatomic feature. After adjusted for the fistula anatomy, compared to SC, TCC was associated with higher risk of major complications (p = 0.013). Proportions of patients requiring blood transfusion and intra-operative blood loss were higher in SC versus TCC groups, as were longer duration of hospital and ICU stay during hospitalization. In contrast, myocardial ischemia (10.2% vs 0.0%, p = 0.028), residual shunts (16.9% vs 3.6%, p = 0.045) and new-onset moderate-to-severe valve regurgitation (11.9% vs 0.0%, p = 0.013) were higher in TCC group versus SC groups during follow-up. CONCLUSIONS: TCC has less invasive and faster recovery. However, SC had a higher successful rate and lower risk of major complications in pediatric patients.

Transcatheter Closure of Large Coronary-Cameral Fistulas Using the Patent Ductus Arteriosus Occluder or Amplatzer Vascular Plugs.

Transcatheter closure (TCC) has emerged as the first-line treatment for coronary artery fistulas. However, limited data exist regarding the long-term outcomes and technical aspects of this procedure. We aimed to report the long-term outcomes and technical aspects of TCC of large coronary-cameral fistulas (CCFs).All patients with large CCFs who underwent attempted TCC using the patent ductus arteriosus (PDA) occluder or Amplatzer vascular plug (AVP), from June 2002 to December 2017, were retrospectively reviewed. A total of 23 patients with large CCFs underwent attempted TCC using the PDA occluder or AVP. Most CCFs originated from the right coronary artery and drained predominantly into the right heart chamber. Procedural success was achieved in 21 (91.3%) patients. Devices were deployed using the arteriovenous loop in 15, transarterial approach in 4, and arterio-artery loop approach in 2 patients. Procedural complications included coronary spasm in one and side branch occlusion in one patient. Among these 21 patients with successful device implantation, follow-up angiograms or computed tomography angiograms were obtained in 14 (66.7%) patients at a median of 11.0 (range, 9.8-16.3) months. Late complications included thrombosis of residual fistula segment without myocardial infarction (MI) in one, coronary thrombosis resulting in MI in one, and recanalization necessitating re-intervention in one patient. No death and device embolization occurred.TCC of large CCFs using the PDA occluder or AVP is an effective therapy in anatomically suitable candidates, with favorable long-term outcomes. Given that potentially hazardous complications may occur late after the procedure, long-term periodic evaluation is mandatory.

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.

Transcatheter occlusion of giant congenital coronary cameral fistulae: a case series.

BACKGROUND: A coronary cameral fistula is a rare connection between a coronary artery and a cardiac chamber or vein bypassing the cardiac capillary bed system. Most of these fistulae are congenital and solitary, although they can be acquired and multiple. CASES PRESENTATION: Case 1: A 10-year-old black South African boy presented with a long-standing history of fatigue; he had a heart murmur, and a bounding pulse and wide pulse pressure. An echocardiogram demonstrated a large coronary cameral fistula involving his left coronary artery and his left ventricle. This was also confirmed on ascending aortogram. Surgical ligation was done and his symptoms improved afterward, but a small residual fistula remained. Case 2: A 7-year-old black South African boy had decreased effort tolerance and a heart murmur on the mid-sternal border. He had cardiomegaly on chest roentgenogram and a dilated left coronary artery origin on echocardiogram. An ascending aortogram confirmed a large left coronary cameral fistula draining to the left ventricle. Case 3: A 28-year-old black South African woman with decreased effort tolerance and chest pain on exertion had a continuous murmur over the lower sternal border. Echocardiography demonstrated a dilated right coronary artery with a fistulous connection to her right ventricle. An ascending aortogram demonstrated a tortuous coronary cameral fistula arising from her right coronary artery to her right ventricle. All three patients were successfully treated percutaneously using the Amplatzer vascular plug type II device. CONCLUSION: The availability of numerous vascular closure devices has made transcatheter occlusion the treatment of choice for the majority of coronary cameral fistulae, rather than the traditional surgical ligation.

Early and late presentation of coronary artery fistula: a possible natural progression?

We present two patients, one 10 years old and another 43 years old, who both had successful transcatheter closure of left main coronary artery to right atrium fistulas. The older patient had a larger fistula as well as more symptoms and a complicated post-procedure course. Closure of medium or large coronary artery fistulas should be considered at younger ages to minimise future complications.

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.

Coronary artery fistula detected with transesophageal echocardiography: An unexpected cause of pulmonary hypertension and chest pain.

Coronary artery fistulae are congenital cardiac abnormalities characterized by an abnormal communication between a coronary artery and a cardiac chamber, systemic or pulmonary vessel. Most of the cases are asymptomatic and are discovered incidentally during an angiography. We describe a rare case of a right coronary artery fistula draining to the right atrium, manifesting in chest pain and pulmonary arterial hypertension (PAH). The fistula was detected on transesophageal echocardiography during the workup for PAH.

Congenital coronary artery fistula: Presentation in the neonatal period and transcatheter closure.

BACKGROUND: Congenital coronary artery fistula is a rare coronary anomaly. Most commonly, such fistulae drain into the right side of the heart or the pulmonary artery. Children with coronary artery fistulae are generally asymptomatic, although they may have left ventricular enlargement in the setting of a moderate sized left to right shunt. Symptoms of congestive heart failure or ischemia are very rare in neonatal period, and suggest the presence of a very large shunt and/or coronary steal. METHODS: Single center retrospective review of transcatheter intervention on coronary artery fistulae presenting with symptoms in the neonatal period from January 2000 to March 2018. Patients with associated congenital heart diseases (except atrial septal defect) were excluded. Patient records, catheterization data, angiograms and noninvasive imaging were reviewed. RESULTS: Two patients underwent transcatheter intervention for symptomatic coronary artery fistula in the first few weeks of life. The first patient had multiple right and left coronary artery to right ventricle fistulae and presented with severe biventricular systolic dysfunction. Transcatheter closure of the fistulae was performed using multiple Gianturco coils. The second patient had a large left main coronary artery to left ventricle fistula (with left anterior descending and circumflex coronary artery atresia) presenting with symptoms of ischemia. This large fistula was closed using one Amplatzer Vascular Plug type-II and two Micro-Vascular Plugs. Both patients had improvement in symptoms post intervention and are doing well at the last follow up at 12 years and 7 months respectively. CONCLUSIONS: We hereby describe the rare presentation of symptomatic coronary artery fistulae in the neonatal period and their successful transcatheter management. This is also the first description of left anterior descending and circumflex coronary artery atresia in the setting of a large left main coronary artery to left ventricle fistula.

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.

Endocarditis of a congenital coronary fistula in a child.

Endocarditis of congenital coronary fistulas in the cardiac chambers is rare, especially in the paediatric age group. We describe the case of a 9-year-old boy with a fistula from the dilated right coronary artery to the junction of the superior caval vein to the right atrium, complicated by endocarditis. Treatment consisted of 6 weeks of antibiotics and interventional closure of the fistula 3 months later with an Amplatzer vascular plug.

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.

[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%).

Coronary Artery Fistulas: Case Series and Literature Review.

Congenital coronary artery fistulas are rare anomalies. As coronary angiography and multidetector computed tomography have become more accessible, they have been increasingly used in the investigation of chest pain and heart failure. Coronary artery fistulas are often an incidental finding, which raises the question of how patients with this condition should be managed. Intervention with either transcatheter closure or surgical closure is often technically possible. Many patients are asymptomatic early after closure. However, follow-up studies have shown post-closure sequelae, such as residual leakage, thrombosis with or without myocardial infarction, and coronary stenosis. Therefore, there has been a shift from intervention towards watchful waiting in asymptomatic patients. In this article, we review the published literature on the natural history and treatment outcomes in individuals with coronary artery fistulas. We present case reports from our clinic and discuss the management of incidental findings of coronary artery fistulas.

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.

Isolated Coronary Artery Fistula in Fetal Heart: Case Reports and Literature Review.

We report two cases of prenatally diagnosed isolated coronary artery fistula. The first fetus had a fistulous communication between the left circumflex coronary artery and the right atrium. The second fetus had right coronary artery to left ventricle fistula. In addition, we have also reviewed previously published reports on prenatally diagnosed coronary artery fistulae, focusing on their clinical profiles, outcome and postnatal treatment.

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.