Etiology and prognosis of non-Kawasaki disease induced coronary aneurysms in children: a retrospective case series study.

While Kawasaki disease (KD) induced coronary artery aneurysms (KD CAAs) in children are well studied, the features and prognosis of non-KD induced CAAs (non-KD CAAs) in the pediatric population are poorly documented. This case series study is to analyze the etiology and prognosis of non-KD CAAs in children and compare the characteristics of non-KD CAAs and KD CAAs. Non-KD CAA and KD CAA cases at our department from January 2022 to December 2023 were retrospectively collected. Etiologies and prognosis of non-KD CAAs were analyzed. Furthermore, demographic data, biochemical parameters and outcomes between children with Non-KD CAAs and children with KD CAAs were comparatively studied. Fifteen children with non-KD CAAs with a median age of 6 years and 117 children with KD CAAs with a median age of 2.0 years (p = 0.022) were included in this study. The causes of non-KD CAAs include: unknown etiologies (2 cases), coronary artery structural abnormalities (4), Takayasu arteritis (2), virus infection (2), cardiomyopathy (2), aplastic anemia with agranulocytosis (1), ANCA-associated vasculitis (1), and mucopolysaccharidosis (1). In the non-KD CAA group, there were a total of 19 CAAs with 3 being giant, 5 medium, and 11 small; 4 patients had complete CAA regression; an infant with a fistula between the right coronary artery and the coronary sinus complicated with cardiac enlargement died of heart failure. The KD group had significantly higher levels of CRP, white cells counts and ESR with zero mortality. Non-KD CAA cases had a significantly lower regression rate than KD-CAA cases (26.7% vs 66.7%, p = 0.004), and the probability of CAA regression in non-KD patients was 0.341 of that in KD patients (p = 0.006, OR = 0.341, 95% CI: 0.179-0.647). CONCLUSIONS: Various etiologies for Non-KD CAAs are identified. Patients with Non-KD CAAs were observed to have lower inflammatory indexes but poorer recovery than patients with KD CAAs. Therapeutic strategies different than those for KD may be needed for non-KD CAAs. WHAT IS KNOWN: • Coronary artery aneurysm (CAA) in children is most commonly induced by Kawasaki disease (KD CAA), with a 50 ~ 70% regression rate in 1 to 2 years. • CAA induced by diseases other than KD (non-KD CAA) in children is rare and its prognosis remains largely unknown. WHAT IS NEW: • Most non-KD CAA cases are caused by coronary artery structural malformations. • Non-KD CAA in children has poorer prognosis and lower regression rate compared with KD CAA. • In addition to guideline directed anti-platelet and anti-coagulant therapies, treatments targeting the causal factor are necessary for non-KD CAA.

1 Patient With Single Coronary Artery, Giant Coronary Artery Aneurysm, Contained Rupture, and Fistula.

Single coronary artery, giant coronary artery aneurysm, and coronary cameral fistula are rare congenital anomalies, and can cause a range of presentations. To our knowledge, this is the first reported case of all 3 entities occurring simultaneously in 1 patient, with largely unknown implications. Multimodal imaging was essential in prompt diagnosis and management.

Percutaneous Coil Closure of a Large Left Coronary Artery Fistula in an Asymptomatic Child.

Coronary artery fistulas (CAFs) are rare cardiac anomalies characterized by an abnormal connection between the coronary arteries and either a cardiac chamber or a large thoracic vessel. While the majority of CAF cases are asymptomatic, serious cardiac complications can occur, especially with moderate to large fistulas. We describe a case of a large-sized left coronary artery (LCA) fistula in an asymptomatic 11-year-old who was referred for cardiac evaluation due to a systolic murmur. An echocardiogram revealed a hemodynamically significant fistula arising from the LCA draining into the right ventricle. Diagnostic catheterization confirmed the origin and draining site of the fistula, along with aneurysmal dilation at the end of the fistula. The fistula was successfully closed percutaneously using a two-coil occlusive device, with no complication observed.

A simple, ingenious approach for intra-operative detection of coronary cameral fistula.

A 56-year-old female presented to us with rheumatic mitral stenosis with a left atrial clot. We found one coronary cameral fistula on the coronary angiography from the posterior left ventricular branch to the left ventricle. She was taken up for surgical mitral valve replacement and clot removal. A residual coronary fistula may increase the risk of infective endocarditis, especially in the presence of mechanical prosthesis. Henceforth, intra-operatively, we used the methylene blue dye injection as an easy technique to detect and safely close the coronary fistula from inside the left ventricular chamber. She recovered smoothly with a successful outcome.

Detection of Coronary Artery Fistula between the LAD and the Great Cardiac Vein on Coronary CT Angiography.

Coronary artery fistulas (CAFs) are abnormal communications of coronary arteries whereby venous circuits bypass the normal capillaries within the myocardium. Coronary artery-to-cardiac vein fistula is the third most common type of CAF, accounting for 7% of cases. Electrocardiographic-gated cardiac computed tomographic (CT) angiography has emerged as a noninvasive alternative method of choice for diagnosis due to its high spatial and temporal resolution and short acquisition time. Herein, we aimed to present a left anterior descending coronary artery opened into the greater cardiac vein at the distal level, consistent with a coronary artery-to-cardiac vein fistula in a 77-year-old woman. Teaching point: Coronary artery CT angiography provides a detailed evaluation of the complex anatomy of coronary artery fistula without the need for invasive methods.

Three-Decade Experience with Management of Coronary Artery Fistulas in Children.

BACKGROUND: Treatment approach of coronary artery fistulas (CAFs) is debatable and long-term outcomes are unknown. METHODS: Retrospective institutional data review of children in whom echocardiographically suspected CAFs were confirmed during cardiac catheterization from 1997-to-2023. Treatment approach and outcomes were assessed. RESULTS: We identified 94 CAFs in 78 patients (42.3% males), median age of 3.4 years (IQR, 0.9-6.6). 25 (32%) patients had other congenital anomalies. 41/52 (78.8%) patients with isolated CAFs were asymptomatic. Most common site of CAF origin and drainage was the left system (62.8%) and right cardiac cavities (80.8%). Overall median follow-up was 101 months (IQR, 41-185). 23 (29.5%) patients with 35 (37.2%) small or non-shunting CAFs had conservative management and 20/23 (87%) patients had an uneventful follow-up. 8 (10.2%) patients with 9 (9.6%) complex CAFs were directly sent for surgery. One had early surgical patch failure needing surgical reintervention. 47 (60.3%) patients had catheter closure of 50 (53.2%) medium or large-sized CAFs using coils (30%), vascular plugs (20%), Nitinol duct occluders (40%), or material combination (10%). Six serious complications occurred. 2/3 patients with unsuccessful catheter procedures had subsequent surgeries. 2/3 patients with mild shunts had successful redo closures. One asymptomatic patient had recanalization after 12 years and is under watchful observation. CONCLUSIONS: CAFs have various anatomies and clinical presentations. Transcatheter closure is effective in carefully selected patients but is not complication-free. Surgery is a valuable upfront option in complex CAFs or bailout of unsuccessful transcatheter closures even though not frequently used.

Aortico right atrial tunnel - Clinical presentation, transcatheter management, and follow-up from a large cohort of patients.

Background: Aortico right atrial tunnel (ARAT) is a rare extracardiac communication between the aorta and the right atrium with two anatomical types. A recent global review identified 59 patients. Methods: Patients with ARAT from two centers were analyzed for their demographics, symptoms, morphology, management, and follow-up thromboprophylaxis. Results: Among 21 patients including 8 males with a median age of 3 years (18 days-72 years) diagnosed as ARAT, 12 (57%) had posterior tunnels and 9 had anterior tunnels. Four patients had multiple exits. Eighteen tunnels were closed after arteriovenous circuit formation. Six patients (29%) weighing <10 kg presented early with heart failure. Transcatheter closure normalized the hemodynamics including in one infant after failed surgery. Two elderly patients (10%) above 60 years presented with angina and atrial fibrillation. The rest were asymptomatic. Occluders were positioned in the narrow proximal aortic end of the tunnel in all except two patients, where the distal atrial end was closed. All procedures were successful without complications. There was one late death after 1 year from subarachnoid hemorrhage. At a median follow-up of 96 months, all survivors were asymptomatic. Thromboprophylaxis with dual antiplatelets for 1-2 years followed earlier was recently changed to aspirin with Coumadin. Complete remodeling occurred when the proximal aortic end was closed, but partial persistence of the track was noted after distal closure. Conclusions: This largest cohort of ARAT showed the safety and efficacy of transcatheter closure even in neonates. The narrow proximal aortic end should be the target for closure rather than the distal atrial end to achieve complete remodeling.

Unlocking the Hidden Pathway: A Rare Encounter With Right Coronary Artery Fistula to Coronary Sinus and Right Ventricle.

Coronary artery fistulas (CAFs) are rare vascular anomalies characterized by abnormal connections between coronary arteries and cardiac chambers or adjacent structures. Advances in cardiac interventions have led to an increasing recognition of acquired CAFs, which are typically congenital. We present a case of a 62-year-old male with a complex medical history, including hypertension, atrial fibrillation, and heart failure, who presented with exertional chest pain and palpitations. Diagnostic evaluation revealed a significant CAF originating from the right coronary artery (RCA) and terminating into the coronary sinus and right ventricle. Despite the absence of significant coronary artery occlusions, the fistula was deemed clinically significant due to its potential to cause myocardial ischemia. Management involved guideline-directed medical therapy and lifestyle modifications. This case underscores the importance of early recognition and appropriate management of CAFs to optimize patient outcomes. Further research is needed to better understand the natural history and optimal management strategies of CAFs.

Outcomes of Transcatheter Closure of Congenital Left Circumflex Coronary Artery Fistula.

BACKGROUND: Congenital left circumflex coronary artery fistula (LCX-CAF) is a relatively rare type of coronary artery fistula (CAF); little is known about the outcomes of transcatheter closure (TCC) of LCX-CAF.Methods and Results: All consecutive patients admitted to Fuwai Hospital and scheduled for TCC of LCX-CAF between January 2012 and December 2022 were reviewed retrospectively. Of the 25 consecutive patients (mean [±SD] age 34±20 years; 48% male) admitted and scheduled for TCC of congenital LCX-CAF, the procedure was feasible in 22 (77.3%). The mean (±SD) diameter of the fistulas was 6.99±2.04 mm; 21 (84%) patients had a large fistula (i.e., diameter >2-fold greater than non-feeding coronary artery). Occluders were deployed via a transarterial approach and arteriovenous loop in 6 (27.3%) and 16 (72.7%) patients, respectively. No procedural complications were recorded. Although the procedural success rates are similar for single LCX-CAF and left anterior descending CAF (81.25% vs. 92.86%; P=0.602), the mean time from initial angiography to first occluder deployment is significantly longer for LCX-CAF (83.06±36.07 vs. 36.00±9.49 min; P<0.001). The mean (±SD) follow-up time was 62.2±45.5 months. The incidence of myocardial infarction and recanalization of the fistula was 4.5% (1/22) and 9.1% (2/22), respectively. CONCLUSIONS: TCC of LCX-CAF is a feasible and effective alternative to surgical repair, with comparable outcomes in selected patients. Optimal medical therapy to prevent post-closure myocardial infarction requires further investigation.

A successful surgical management for a thrombosed giant left coronary aneurysm with right ventricular fistula in a young patient.

Giant coronary aneurysm with ventricular fistula is rare. Due to the limited data from randomised clinical trials, therapeutic strategies for coronary aneurysms predominantly rely on on case series and anecdotal evidences. Reporting cases that provide practical experience in managing these aneurysms is therefore crucial. In this article, we report a rare case of a successful surgical management for a thrombosed giant left coronary aneurysm with right ventricular fistula, which is larger than any previously reported cases.

Acute myocardial infarction due to giant coronary artery aneurysm and arteriovenous fistula: a challenging case report and review of the literature.

BACKGROUND: A coronary artery aneurysm (CAA) is an abnormal dilation of a coronary artery segment often accompanied by coronary artery fistula (CAF), leading to communication between a coronary artery and a cardiac chamber or a part of the coronary venous system. Both CAAs and CAFs can present with symptoms and signs of myocardial ischemia and infarction. CASE PRESENTATION: We describe the case of a 46-year-old woman with non-ST-elevation myocardial infarction (NSTEMI) caused by a "giant" CAA. Various imaging modalities revealed a thrombus-containing aneurysm located at the right-posterior cardiac border, with established arteriovenous communication with the distal part of left circumflex artery (LCx). After initial treatment with dual antiplatelet therapy, a relapse of pain was reported along with a new increase in troponin levels, electrocardiographic abnormalities, reduced left ventricular ejection fraction (LVEF) and thrombus enlargement. Surgical excision of the aneurysm was favored, revealing its true size of 6 cm in diameter. Τhe aneurysm was excised without complications. The patient remained asymptomatic during follow-up. CONCLUSIONS: Management of rare entities such as "giant" CAAs and CAFs can be challenging. Cases such as this can serve as precedents to facilitate treatment plans and develop consistent recommendations, emphasizing the importance of personalized strategies for future patients.

A case report of a young woman with pulmonary emboli and a right coronary artery-to-coronary sinus fistula.

Background: Coronary artery fistulae are rare cardiovascular anomalies that can present with atypical symptomatology and therefore pose diagnostic challenges, especially in young patients. Case summary: A 34-year-old woman presented with left-sided pleuritic chest pain, haemoptysis, and flu-like symptoms. Initial evaluation revealed multiple left-sided pulmonary emboli, and her transthoracic echocardiography showed turbulent flow in a dilated coronary sinus. A right coronary artery (RCA) to coronary sinus fistula was confirmed by computed tomography coronary angiogram. The patient was treated with lifelong anticoagulation, and a subsequent stress cardiac magnetic resonance imaging did not show inducible myocardial ischaemia. As such, the patient was managed conservatively. Discussion: Utilization of multi-modality imaging is of utmost importance for diagnostic and therapeutic purposes in coronary artery fistulae. In this case report, our patient presented with unprovoked pulmonary emboli, which could be caused by the turbulent flow and stasis, due to the RCA-to-coronary sinus fistula.

Transcatheter closure of a coronary artery to pulmonary artery fistula with two sequential giant aneurysms.

A 73-year-old female presented with exertional dyspnea and was found to have a coronary artery to pulmonary artery fistula with 2 sequential giant aneurysms. Her chest radiograph showed a mass above the cardiac silhouette.

Surgical Management of Coronary Artery Fistulas in Children.

Background: This study investigated the surgical outcomes associated with coronary artery fistulas (CAFs) in children. Methods: We retrospectively reviewed the medical records of 23 pediatric patients who underwent surgical closure of CAFs between 1995 and 2021. At presentation, 7 patients (30.4%) exhibited symptoms. Associated cardiac anomalies were present in 8 patients. Fourteen fistulas originated from the right coronary artery and 9 from the left. The most common drainage site was the right ventricle, followed by the right atrium and the left ventricle. The median follow-up duration was 9.3 years (range, 0.1-25.6 years). Results: The median age and body weight at repair were 3.1 years (range, 0-13.4 years) and 14.4 kg (range, 3.1-42.2 kg), respectively. Cardiopulmonary bypass was used in 17 cases (73.9%), while cardioplegic arrest was employed in 14 (60.9%). Epicardial CAF ligation was utilized in 10 patients (43.5%), the transcoronary approach in 9 (39.1%), the endocardial approach in 2 (8.7%), and other methods in 2 patients (8.7%). The application of cardioplegic arrest during repair did not significantly impact the duration of postoperative intensive care unit stay or overall hospital stay. One in-hospital death and 1 late death were recorded. The overall survival rate was 95.7% at 10 years and 83.7% at 15 years. A residual fistula was detected in 1 patient. During the follow-up period, no surviving patient experienced cardiovascular symptoms or coronary events. Conclusion: Surgical repair of CAF can be performed safely with or without cardioplegic arrest, and it is associated with a favorable prognosis in children.

Anaesthetic management of a parturient with an unrepaired coronary arteriovenous fistula for caesarean section.

Coronary arteriovenous fistulas are an abnormal conduit between a coronary artery and another cardiovascular lumen, without an intervening capillary bed. The reported prevalence is 0.002-0.3%. Physiologic consequences such as congestive heart failure, coronary steal phenomenon and fistula aneurysm formation and rupture are possible. There are limited reports of symptomatic coronary arteriovenous fistulas in association with pregnancy. We describe a 19-year-old woman with symptomatic left circumflex artery to coronary sinus fistula, terminating into a large exophytic varix in the right atrium, presenting for an elective caesarean section at 37 weeks gestational age. Our anaesthetic management strategy aimed to optimise myocardial perfusion, maintain euvolemia, avoid right ventricular obstruction from exophytic varix and avoid sympathetic stimulation or sudden increases in pulmonary vascular resistance. A slowly titrated epidural was used as the primary anaesthetic. Our patient tolerated the procedure well and was discharged home on postoperative day two. Understanding of the potential physiologic consequence of coronary arteriovenous fistulas, and interaction with the physiologic changes of pregnancy and delivery, are essential for the management of these cases.

Adult-type anomalous origin of the left coronary artery from the pulmonary artery and right coronary-right atrial fistula: a case report.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac anomaly, mortality rates in infancy reach approximately 90%, with only a small number of patients surviving into adulthood, therefore, most of the literature reports mainly focus on infantile type. CASE PRESENTATION: A 55-year-old female was admitted due to persistent repeated chest pain experienced and had worsened for unknown reasons. Color doppler echocardiography, coronary computed tomographic angiography, and coronary angiography confirmed the diagnosis of ALCAPA and concurrent right coronary artery-right atrial fistula. The symptoms of chest pain exhibited notable improvement subsequent to corrective surgery for the anomalous origin of the coronary artery. CONCLUSIONS: This report shows an unique case of ALCAPA in an adult patient, characterizing the condition's combination with a right coronary-right atrial fistula, and it is prone to misdiagnosis and misdiagnosis. We aim to provide valuable insights for clinical diagnosis and treatment of ALCAPA.

Surgical Treatment of an Aneurysmal Coronary Artery Fistula between the Left Coronary Artery and Right Atrium: A Case Report.

A coronary artery fistula (CAF) is an abnormal vascular connection between the coronary arteries and the cardiac chambers or major vessels. Although rare, CAFs can lead to substantial coronary morbidity and mortality. This study outlines the surgical management of a CAF originating from the left coronary artery and connecting to the right atrium, in a patient experiencing angina with a marked left-to-right shunt. The surgical approach involved ligation of the coronary artery and reduction of the aneurysmal portion, resulting in the patient's uneventful recovery.

Coronary artery complications after left bundle branch area pacing: An increasingly reported issue in the era of physiologic pacing.

Coronary artery lesions related to pacemaker implantation are rare complications. With the increasing adoption of the technique of permanent transseptal pacing of the left bundle branch area pacing (LBBAP), an increase in the incidence of these complications may be expected. We report two cases of coronary lesions after permanent transeptal pacing of the LBBAP: the first with a small coronary artery fistula, and the second with an extrinsic coronary compression. Both complications occurred with stylet-driven pacing leads with extendable helix. In the first case, since the shunt volume was small and no major complications were reported, the patient was treated conservatively with good outcome. The second case required lead repositioning due to acute decompensated heart failure.

A Case of Abnormal Right Ventricular Color-Flow Doppler Jet.

Transthoracic echocardiography is used routinely during the follow-up after heart transplant surgery to screen possible complications and adverse events such as rejection. It often results in incidental findings that bring diagnostic challenges for sonographers. This E-challenge shows a Doppler flow abnormality associated with a rare cardiovascular diagnosis. Its physiopathology and its association with echocardiography findings are reviewed.