Giant left main coronary artery aneurysm with fistulous drainage to the right atrium: a rare case report and surgical management approach.

Christmas Lights on the Heart: A Rare Case of Multiple Giant Coronary Artery Aneurysms.

Giant right coronary artery aneurysm arising from a single coronary artery origin: A case report

Introduction: The existence of a coronary artery aneurysm (CAA) can pose significant risk for death. It can cause thrombosis, dissection, rupture or myocardial infarction. An exceedingly rare involvement of the left main coronary artery (LMCA), particularly giant-sized is even more catastrophic, a finding seen in only 0.1% of patients. Furthermore, co-existence with significant stenotic coronary artery disease (CAD) portends grim survival. Owing to the rarity of this combination, no data is available locally and only limited case reports are documented internationally. Hence, no consensus guidelines have been published yet. This paper aims to contribute to the sparse medical knowledge on the treatment approach and management of LMCA aneurysm with concomitant CAD. Case Presentation: A 62-year-old male, Filipino, hypertensive and hyperlipidemic sought consult due to one-year exertional chest pain. Coronary angiogram revealed the LMCA to be a diffusely aneurysmal, large-sized vessel measuring 9.7 mm x 7.9 mm with a significant two-vessel CAD affecting the proximal left anterior descending (LAD) and right coronary artery (RCA). As per multidisciplinary decision, the patient underwent surgical revascularization via cardiopulmonary bypass graft (CABG) addressing the CAD and LMCA aneurysm managed conservatively through guideline-directed medical therapy. The patient’s course of treatment was uneventful. He returned for follow-ups for three months post-surgery and remained symptom-free. Discussion: Giant coronary artery aneurysms (GCAA) are vessel dilatations that exceed 4x the diameter of a normal adjacent artery. The patient had a unique case of GCAA involving the LMCA combined with two-vessel CAD. Few studies have documented a medical or surgical approach and long-term outcomes are unknown. Without sufficient evidence-based guidelines, the multidisciplinary decision was to perform CABG and manage the LMCA aneurysm conservatively. Conclusion: Due to extremely limited information available on the giant LMCA aneurysm natural history, definitive management remains controversial. A multidisciplinary team approach is highly recommended for patient-specific needs to achieve favorable outcome and ensure survival. Keywords: Giant left main artery aneurysm, CABG, CAD

Successful surgical management of a giant coronary artery aneurysm complicated by a coronary-to-pulmonary artery fistula

Objective: To investigate the changes and significance of cardiopulmonary adaptations in children with Kawasaki disease sequelae of giant coronary artery aneurysm (KD-GCAA). Methods: A retrospective cohort study. Twenty-nine KD-GCAA children diagnosed at the Children's Hospital of Fudan University from January 2024 to March 2025 were enrolled as GCAA group, and 67 healthy children matched in age, gender and body mass index during the same period were selected as control group. The GCAA group underwent cardiopulmonary exercise test (CPET) after completing a questionnaire-based assessment, while the control group underwent CPET directly. Wilcoxon signed-rank test was used to compare the differences in daily activity and actual exercise capacity of children in the GCAA group, as well as differences in CPET-related indicators and treadmill exercise test (TET) related indicators. Independent sample t-test and Mann-Whitney U test were used to compare the differences in CPET indicators between the GCAA group and control group. Results: The GCAA group consisted of 20 males and 9 females, with the age of (10.9±2.2) years, while the control group included 45 males and 22 females, with the age of (11.1±2.1) years. Daily activity metabolic equivalent of task (MET) was 4.5 (4.5,5.0) in KD-GCAA group which was significantly lower than the maximum MET 10.8 (9.9,12.1) and MET at the anaerobic threshold 8.0 (7.1,8.9) measured by CPET (both P<0.001). All children completed the modified Bruce protocol up to stage 5-6 without adverse events. Decreased exercise tolerance was observed in 10% (3/29) of the GCAA group, all of which were missed by TET. Peak oxygen uptake per kilogram of body mass (peak V'O2/kg), peak V'O2/kg to predicted value, maximum MET, V'O2/kg at anaerobic threshold, V'O2/kg at anaerobic threshold to predicted value,MET at anaerobic threshold, heart rate at anaerobic threshold, maximum minute ventilation and ventilatory equivalent for carbon dioxide were significantly lower than those in the control group (all P<0.05). The maximum MET measured by TET was significantly higher than that by CPET in GCAA group (11.5 (10.2, 13.2) vs. 10.8 (9.9, 12.1), Z=2.24,P=0.025). Conclusions: KD-GCAA children have lower cardiopulmonary fitness and exercise tolerance than healthy children, along with insufficient daily physical activity. Compared with TET, CPET can more accurately evaluate the changes in cardiopulmonary fitness of these children, which holds important application value for clinical exercise guidance.

Giant Right coronary artery aneurysm fistulization to the coronary sinus: technique for surgical repair

Twelve-year natural progression of a giant right coronary artery aneurysm.

Giant coronary aneurysms are rarely described in current literature, with an incidence of only 0.02%. Treatment strategies vary and there is no definitive recommendation; however, surgical treatment should be considered to prevent future complications. We report the case of a 58-year-old patient with a history of hypertension, diabetes, and obesity who presented with non-ST-segment elevation myocardial infarction (NSTEMI), ischemic cardiomyopathy with reduced ejection fraction, coronary artery disease, and a giant right coronary artery (RCA) aneurysm with compressive effects and secondary hemodynamic repercussions, requiring surgical management. This case describes an acute myocardial infarction associated with a giant compressive RCA aneurysm initially mistaken for a mediastinal mass. It highlights its challenge as a differential diagnosis and the importance of early surgical intervention and literature production on this rare entity.

Vasculitis with Giant Coronary Artery Aneurysms: Role of Magnetic Resonance Imaging

Value of contrast echocardiography in the diagnosis of a giant right coronary artery aneurysm

BackgroundKawasaki disease (KD) is the leading cause of acquired childhood heart disease, with untreated cases progressing to giant coronary artery aneurysms (GCAAs) in 2-3% of patients. While outcomes are well-documented in high-income settings, data from low- and middle-income countries (LMICs) remain limited. This study examines clinical profiles, treatment responses, and outcomes of children with GCAAs managed at a tertiary care center in South India.MethodsWe conducted a retrospective review of 18 children with KD-associated GCAAs (z-score >10 or diameter >8mm) treated between 2019-2024 at a tertiary care center in Kerala, India. All patients were referred from external centers with documented IVIg resistance. Treatment intensification included infliximab, corticosteroids, cyclosporine, and anakinra. Primary outcome was GCAA dimension change at last follow-up; secondary outcomes included thrombotic events, mortality, and anticoagulation cessation.ResultsThe cohort comprised predominantly of males (n = 14, 77.8%) with median symptom onset at 1.1 (IQR 0.3 – 2.7) years; 50% were under one year old. While ten children (55%) experienced delayed diagnosis (>10 days after symptom onset) with median diagnosis at 11 (IQR 7 – 13.5) days; all faced delays in treatment intensification. Excluding two children lost-to-follow-up, during the median follow-up of 8.6 (5.2 – 19.4) months, complete GCAA resolution occurred in three (18.8%), reduction to small/medium aneurysms in six (37.6%), while five (31.3%) remained static. One mortality (6.3%) from myocardial infarction and a patient requiring coronary artery bypass grafting were observed. Anticoagulation was discontinued in 56.25% of children whose aneurysms resolved or reduced significantly.ConclusionDelayed diagnosis and treatment intensification may have contributed to mixed outcomes in this GCAA cohort. Early recognition, appropriate risk stratification, and timely immunosuppression intensification are essential to improve prognosis and reduce severe coronary complications in resource-limited settings.

We report a rare case of a giant right coronary artery (RCA) aneurysm (56 × 58 mm) with a fistulous communication to the coronary sinus, causing right atrial and ventricular compression in a 47-year-old female presenting with heart failure. Multimodality imaging confirmed the diagnosis. Surgical management included aneurysm resection, fistula closure, and saphenous vein grafting to the distal RCA. Despite initial stabilization, the patient developed low cardiac output and died postoperatively. This case highlights the diagnostic and surgical challenges of giant RCA–coronary sinus fistulas and underscores the need for early recognition and timely intervention to prevent fatal outcomes. Keywords: Coronary artery aneurysm, Coronary fistula, Right coronary artery, Coronary sinus, Aneurysmectomy, Case report

Background: The best treatment for children with KD who fail to respond to the first dose of IVIG (refractory KD) is currently unknown. The purpose of this study was to determine treatment practices of pediatric rheumatologists in North America who manage IVIG-refractory KD. Methods: A 34-item web-based survey was sent to 102 randomly selected members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA). The anonymous survey addressed the use of primary intensification as well as the treatment of IVIG-refractory KD. Results: The response rate was 82%; 56% (all pediatric rheumatologists) completed the survey. Primary intensification was used for macrophage activation syndrome (MAS), KD shock, and those at high risk for coronary artery aneurysms (CAAs) by 84%, 76% and 52% of responders, respectively, with corticosteroids (CSs) used most frequently. For IVIG-refractory KD without CAA, a second dose of IVIG was used most often (63% alone; 23% plus CS). With non-giant CAAs, only 15% used a second IVIG alone, 40% used IVIG plus CS, and 35% took infliximab, usually with CS/IVIG. With giant CAA, treatments used most frequently were CS, a second IVIG, and infliximab (91%, 69%, and 58%, respectively), usually as combinations of two or more medications. Conclusions: Treatment of IVIG-refractory KD varies significantly among North American pediatric rheumatologists, particularly in the presence of CAAs. Our findings emphasize the need for research to identify the most effective therapy for this KD subgroup. The current use of primary intensification and the presence and size of the CAA will need to be considered as consensus treatment plans are developed.

BACKGROUNDGiant coronary artery aneurysms (CAA), entailing thrombosis, myocardial infarction, and sudden death, are the most severe and life-threatening complications of Kawasaki disease (KD). Giant aneurysms rarely regress and can later transform into stenoses. Data on dynamic follow-up are scarce in the literature.AIMTo evaluate clinical features and long-term outcomes of giant CAA in children with KD.METHODSA single-center retrospective study included data from patients with KD and giant CAA in the Irkutsk region (2012-2023). CAA criteria according to the American Heart Association guidelines of 2017 were used: (1) Dilated coronary artery with diameter Z-score > 2 standard deviations (SD) but < 2.5 SD; (2) Small CAA with Z-score > 2.5 SD but < 5 SD; (3) Medium CAA with Z-score > 5 SD but < 10 SD; and (4) Giant CAA with Z-score > 10 SD or ≥ 8 mm.RESULTSThe mean age of children with coronary dilatation/aneurysms was 2.5 years, and the male-to-female ratio was 3:1. Patients with giant/medium CAA had symptoms of cerebral dysfunction more often compared with children with moderate (Z-score < 5 SD but > 2.0 SD) coronary dilatation (62.0% vs 21.0%, P = 0.019). Major cardiovascular events (myocardial infarction, coronary artery bypass grafting, acute coronary syndrome, ischemic cardiomyopathy, left ventricular aneurysm, and giant extracardiac aneurysm) occurred in 55.5% of patients who had giant CAA. At follow-up the complete regression of giant/medium CAA was observed in 58.0% and partial regression in 42.0% after a mean of 2.3 and 5.5 years, respectively. All thrombi detected by echocardiography, CT, and angiography in giant/medium CAA disappeared between 1 year and 5 years (mean: 15 months). All patients survived.CONCLUSIONRisk factors for giant CAA were male sex, early age, and cerebral dysfunction. Complete regression of giant coronary aneurysms occurred in 58.0% of patients after follow-up of 2.3 years.

According to the Centers for Disease Control (CDC) health advisory may 2020, Multisystem Inflammatory Syndrome in Children (MIS-C) is defined as an individual &lt;21yrs presenting with fever, laboratory evidence of inflammation and evidence of clinically severe illness requiring hospitalization with multisystem (≥= 2) organ involvement( cardiac, renal, respiratory, hematologic, gastrointestinal, dermatologic or neurological) and no alternative plausible diagnosis and Positive for current or recent SARS-CoV2 (COVID 19) infection by Reverse Transcription Polymerase chain Reaction (RT-PCR), serology or antigen test or COVID 19 exposure within the 4 weeks before the onset of symptoms and The most affected age group is 6–10 years. Fever meaning a temperature of &gt;38C for ≥= 24hours or a report of subjective fever lasting ≥= 24hours. Laboratory examination of changes in peripheral blood complete blood count (CBC) parameters during acute Kawasaki disease (KD) shows evidence of inflammation. CBC parameters were collected at the initial, pre-IVIG, and post-IVIG stages. The rank-sum test assessed parameter differences over time, including elevated C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), fibrinogen, ferritin, procalcitonin, D-dimer, lactic acid dehydrogenase (LDH), elevated neutrophils, reduced lymphocytes, and low albumin.

Giant coronary artery aneurysms with fistulas are rare and may present with heart failure and arrhythmias. We report a 62-year-old man with hypertension who developed acute decompensated heart failure and rapid atrial fibrillation. Imaging revealed a giant right coronary artery aneurysm with a large fistula draining into the right atrium. Cardiac MRI was not performed due to claustrophobia. Medical management stabilized the patient, and invasive treatment was deferred. This case illustrates the diagnostic and therapeutic challenges of complex coronary anomalies, emphasizing the role of multimodality imaging and a heart team approach for optimal care planning in such rare conditions.

Data on the use of infliximab (IFX) in infants with Kawasaki disease (KD) are scant. We retrospectively analyzed data of 35 infants with KD who received IFX. Effectiveness of IFX was assessed in the presence ofintravenous immunoglobulin (IVIG) resistance and coronary artery aneurysm (CAA) regression. Fifteen of thirty-five received IFX in the presence of IVIG resistance; nineteen of thirty-five for CAA; and one for persistently high c-reactive protein. Thirteen out of fifteen with IVIG resistance became afebrile by 24h. Twelve medium CAA regressed over 3months, four of ten giant CAA normalized, and three regressed to small CAA, median time being 7.5 (3.5, 18) months. IFX was well-tolerated, safe, and effective in infants with KD.

Multimodality vascular imaging in sequential treatment for giant coronary artery aneurysms with coronary-pulmonary artery fistulas.

Proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors offer clinical benefits by reducing low-density lipoprotein cholesterol levels and have emerged as valuable therapeutic agents for the management of cardiovascular diseases. Although their effectiveness in treating coronary artery plaques and abdominal aortic aneurysms has been reported, data on their effects on coronary artery aneurysms are limited. Herein, we report the case of a 43-year-old man with familial hyperlipidemia who presented with angina pectoris caused by a giant right coronary artery aneurysm accompanied by extensive plaques and severe stenosis. The patient underwent coronary artery bypass grafting at 6 anastomotic sites, and a PCSK9 inhibitor was initiated postoperatively. Five years after surgery, imaging demonstrated a gradual reduction in the size of the coronary artery aneurysm, and 8 years later, the size was further reduced. This case report illustrates the rare clinical course of a coronary artery aneurysms.