Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Associated with Right Coronary Giant Aneurysm.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation, usually diagnosed in the infant period due to myocardial ischemia and heart failure, with the need for emergency surgery. Less commonly, it can be asymptomatic until adulthood. Coronary artery aneurysms are also rare anatomical anomalies with symptoms of acute or chronic angina or even remain completely asymptomatic. We present an unusual case of ALCAPA, associated with a giant aneurysm of the right coronary artery. Meeting presentation: American Association for Thoracic Surgery 102nd annual meeting, Boston MA, USA, May 16, 2022.

Giant Left Anterior Descending Coronary Artery Aneurysm in a Patient with Behçet's Disease.

Coronary artery involvement is quite rare in the course of Behçet's disease. Complications secondary to coronary artery aneurysms, including rupture, dissection, and myocardial ischemia, may be fatal. In young patients without cardiovascular risk factors, systemic inflammatory vasculitis syndromes should be investigated in case of acute coronary syndrome secondary to dilated coronary arteries. In this report, we present our management strategy in a 31-year-old male patient with Bechet's disease.

Anakinra Treatment in Patients with Acute Kawasaki Disease with Coronary Artery Aneurysms: A Phase I/IIa Trial.

OBJECTIVES: To determine the safety, pharmacokinetics, and immunomodulatory effects of 2-6 weeks of anakinra therapy in patients with acute Kawasaki disease with a coronary artery aneurysm (CAA). STUDY DESIGN: We performed a Phase I/IIa dose-escalation study of anakinra (2-11 mg/kg/day) in 22 patients with acute Kawasaki disease with CAA. We measured interleukin (IL)-1RA concentrations after the first dose and trough levels up to study week 6. Markers of inflammation and coronary artery z-scores were assessed pretreatment and at 48 hours, 2 weeks, and 6 weeks after initiation of therapy. RESULTS: Up to 6 weeks of anakinra (up to 11 mg/kg/day) was safe and well tolerated by the 22 participants (median age, 1.1 years), with no serious adverse events attributable to the study drug. All participants were treated with intravenous immunoglobulin (IVIG), and 20 also received infliximab (10 mg/kg) before initiation of anakinra. Serum levels of IL-6, IL-8, and tumor necrosis factor α decreased similarly in patients with Kawasaki disease treated with IVIG, infliximab, and anakinra compared with age- and sex-matched patients with Kawasaki disease treated only with IVIG and infliximab. Anakinra clearance increased with illness day at diagnosis. Simulations demonstrated that more frequent intravenous (IV) dosing may result in more sustained concentrations without significantly increasing the peak concentration compared with subcutaneous (SC) dosing. CONCLUSIONS: Both IV and SC anakinra are safe in infants and children with acute Kawasaki disease and CAA. IV dosing every 8-12 hours during the acute hospitalization of patients with Kawasaki disease may result in a sustained concentration while avoiding frequent SC injections. The efficacy of a short course of IV therapy during hospitalization should be studied. TRIAL REGISTRATION CLINICALTRIALS.GOV: NCT02179853.

Coronary Artery Changes in Patients with Multisystem Inflammatory Syndrome in Children: Los Angeles Experience.

We compared cardiac findings in patients with multisystem inflammatory syndrome in children and Kawasaki disease in the first 6 months of the 2020 coronavirus disease pandemic to patients with Kawasaki disease during 2016-2019. We saw a high rate of coronary aneurysms in 2020, with a similar rate of coronary involvement but greater volume and incidence of cardiac dysfunction compared with previous years.

Giant coronary artery aneurysm at autopsy.

This case presents a woman in her early 20s who died after the sudden onset of chest pain. Five years earlier, she was investigated for a cardiac murmur during pregnancy and an echocardiogram revealed a 6.0×3.0 cm blood-filled sac compressing the left atrium and anterolateral aspect of the left ventricle with communication to the aortic root. She later had a CT scan of the chest with contrast, which showed aneurysmal dilatation of the left main coronary artery. She was placed on aspirin but defaulted from clinic 11 months post partum. At autopsy, a left coronary aneurysmal sac measuring 10.0×9.0 cm. was identified with a rupture measuring 7.0 cm in length and the pericardial sac contained 900 mL of blood with clots. The cause of death was cardiac tamponade secondary to rupture of the coronary artery aneurysm.

Stenotic Lesions and the Maximum Diameter of Coronary Artery Aneurysms in Kawasaki Disease.

OBJECTIVES: To determine the prevalence of subsequent stenotic lesions based on the maximum diameter of the largest coronary artery aneurysm in patients with Kawasaki disease and the threshold value of coronary artery diameter associated with risk of developing stenotic lesion. STUDY DESIGN: There were 214 patients (160 males) who had at least 1 aneurysm in a selective coronary angiogram (CAG) done <100 days after the onset of Kawasaki disease were studied. We measured the maximal coronary artery aneurysm diameter in 3 major branches in the initial CAGs. Branches were classified into 3 groups according to their maximal coronary artery aneurysm diameter: large, ≥8.0 mm; medium, ≥6.0 mm but <8.0 mm; and small, <6.0 mm. Subsequent CAGs were performed in the late follow-up period. We investigated the stenotic lesion in the follow-up CAGs, and evaluated the prevalence of stenotic lesion in each group based on body surface area (BSA) by the Kaplan-Meier method. Localized stenosis of ≥25% and complete occlusion were included as stenotic lesion in this study. We also determined the cutoff point for stenotic lesion. RESULTS: The median interval from the initial CAGs to the latest CAG was 8 years, with a maximum of 32 years. For a BSA of <0.50 m2, the 20-year prevalence of large and medium stenotic lesions was 78% (n = 62; 95% CI, 63-89) and 81% (n = 40; 95% CI, 63-89), respectively. For a BSA of ≥0.50 m2, large and medium stenotic lesions were 82% (n = 75; 95% CI, 67-91) and 40% (n = 56; 95% CI, 20-64), respectively (P < .0001). CONCLUSION: The cutoff points of the coronary artery diameter within the first 100 days after the onset of Kawasaki disease leading to a stenotic lesion in the late period, were a diameter of ≥6.1 mm with a BSA of <0.50 m2 and a diameter of ≥8.0 mm with a BSA of ≥0.50 m2. Those cutoff points would have corresponded with a Z score of at least 10 on 2-dimensional echocardiography. Careful follow-up and antithrombotic therapy should be provided to patients who meet these criteria.

Cardiac Events and the Maximum Diameter of Coronary Artery Aneurysms in Kawasaki Disease.

OBJECTIVES: To clarify the occurrence of cardiac events based on the maximal diameter of the maximal coronary artery aneurysm (CAA) in Kawasaki disease (KD). STUDY DESIGN: Two hundred fourteen patients (160 male and 54 female) who had had at least 1 CAA in the selective coronary angiogram less than 100 days after the onset of KD were studied. We measured the maximal CAA diameters in the major branches of the initial coronary angiograms. Death, myocardial infarction and coronary artery revascularization were included as cardiac events in this study. We divided the patients into three groups based on the maximal CAA diameter (large ≥8.0 mm; medium ≥6.0 mm and <8.0 mm; small <6.0 mm). Further, we also analyzed the cardiac events based on laterality of maximal CAA (bilateral, unilateral) and body surface area (BSA). RESULTS: Cardiac events occurred in 44 patients (21%). For BSA < 0.50 m2, the 30-year cardiac event-free survival in the large and medium groups was 66% (n = 38, 95% CI, 49-80) and 62% (n = 27, 95% CI, 38-81), respectively. For BSA ≥ 0.50 m2, that in large group was 54% (n = 58, 95% CI, 40-67). There were no cardiac events in the medium group for BSA ≥0.50 m2 (n = 36) and the small group (n = 56). In the large analyzed group, the 30-year cardiac event-free survival in the bilateral and unilateral groups was 40% (n = 48, 95% CI, 27-55) and 78% (n = 48, 95% CI, 63-89), respectively (P < .0001). CONCLUSIONS: The group with the highest risk of cardiac events was the patient group with the maximal CAA diameter ≥6.0 mm with BSA < 0.50 m2 and the maximal CAA diameter ≥8.0 mm with BSA ≥ 0.50 m2. At 30 years after the onset of KD, cardiac event-free survival was about 60%. Given the high rate of cardiac events in this patient population, life-long cardiovascular surveillance is advised.

Eficacia y seguridad de la inmunoglobulina intravenosa en el tratamiento inicial de pacientes con enfermedad de kawasaki en la fase aguda / Efficacy and safety of intravenous immunoglobulin in the initial treatment of patients with acute phase kawasaki disease

INTRODUCCÇÃO: Antecedentes: El presente informe expone la evaluación de la inmunoglobulina intravenosa como primera línea de tratamiento de pacientes con diagnóstico de enfermedad de Kawasaki debut y activa con alteraciones de las arterias carótidas. Aspectos Generales: La enfermedad de Kawasaki (EK), previamente conocida como síndrome linfo-mucocutâneo, es una de las vasculitis más comunes de la infancia. Es una condición médica tipicamente auto-limitada, con fiebre y manifestaciones de inflamaciónaguda de una duración promedio de 12 dias sin tratamiento. Tecnología Sanitaria de Interés: La inmunoglobulina es un producto biológico que se deriva del plasma de donantes humanos y es usada en el tratamiento de múltiples condiciones médicas, incluyendo los estados de inmunodeficiencia primaria y secundaria, y una variedad de enfermedades inflamatorias. METODOLOGÍA: Estregia de Búsqueda: Se realizó una búsqueda de la literatura con respecto a la eficacia y seguridad de la inmunoglobulina intravenosa para el tratamiento de primera línea de los pacientes con enfermedad de Kawasaki activa y anormalidades de las arterias coronarias. Esta búsqueda se realizó utilkizando los meta-buscadores: Translating Research into Practice (TRIPDATABASE), National Lbrary of Medicine (Pubmed-Medline) y Health Systems Evidence. RESULTADOS: Sinopsis de la Evidencia: Se realizó la busqueda bibliográfica y de evidencia científica para el sustento del uso de IGIV como tratamiento de primera línea en pacientes con enfermedad de Kawasakicon anormalidades de las arterias carótidas. CONCLUSIONES: No se identificaron estudios que hayan comparado los efectos del tratamiento con IGIV con los de Aspirina en pacientes con EK en fase aguda. En su lugar, se ha evaluado los efectos de la adición de IGIV a la apirina, en varios ensayos clínicos aleatorizados y controlados; pero la evidencia es insuficientes y no concluyente respecto ao beneficio que ofrece la aspirina al tratamiento con IGIV. El Instituto de Evaluación de Tecnologías Sanitarias -IETSI, aprueba por el periodo de 2 años a partir de la fecha de publicación del presente dictamen preliminar, el uso de IGIV para el tratamiento de pacientes pediátricos con EK en fase aguda con o sin anormalidades de las arterias carótidas, ya que se ha demostrado su eficacia en la reducción de anormalidades de las arterias carótidas y consecuentemente disminución de la morbimortalidad en la fase aguda y a largo plazo.

Progressive Coronary Dilatation Predicts Worse Outcome in Kawasaki Disease.

OBJECTIVE: To explore the implication of serial coronary changes on the late coronary outcomes in patients with Kawasaki disease (KD) with coronary aneurysms ≧ 4 mm. STUDY DESIGN: We performed a retrospective review of 78 patients with KD with large coronary aneurysms (1980-2013, male: 76.9%; 792 patient-years). Progressive coronary dilatation was defined for those with progressive enlargement of coronary arteries in 3 consecutive echocardiograms. RESULTS: We studied 27 patients with KD with giant aneurysms (≧ 8 mm) and 51 patients with KD with medium aneurysms (4-8 mm). All the giant and 43.1% of medium aneurysms persisted during the study period. For the patients with giant aneurysms, their 10-year freedom from acute myocardial infarction/cardiovascular death and all ischemia was 66% and 52%, respectively. The median intervals for the aneurysm diameters reaching their peak were 3.3 months (giant) and 0.25 months (medium), respectively. In patients with giant aneurysms, the 10-year freedom from ischemia was much lower in those with progressive coronary dilatation (28% vs 59%, P = .021). In patients with medium aneurysms, the probability of 5-year persistence of aneurysm was much greater (67.2% vs 14.8%, P < 10(-3)) in those with progressive coronary dilatation. Male sex and intravenous immunoglobulin therapy were not associated with the late outcomes in the patients with KD who had aneurysms larger than 4 mm. CONCLUSIONS: In addition to coronary diameters 1 month after the onset of KD, progressive coronary dilatation at 2 or more months after diagnosis may be an indicator of duration, and the severity of vasculitis and adverse dilative remodeling were associated with worse late coronary outcomes.

[Left main coronary artery aneurysm thrombosis in a young patient with acute myocardial infarction]. / Trombosis de aneurisma del tronco coronario izquierdo en paciente joven con infarto agudo de miocardio.

INTRODUCTION: The left main coronary artery aneurysm is rare, with an incidence of 0.1%, being the atherosclerosis its main etiology. Angiography is the gold standard for diagnosis and treatment. Depending on the severity of coexisting coronary stenosis, patients with left main coronary artery aneurysms can be effectively managed either surgical or pharmacologically. CLINICAL CASE: We present a case of a 44 year-old male with a history of obesity, smoking and dyslipidemia, complaining of oppressive chest pain, dyspnea and diaphoresis. An electrocardiogram showed an ST-segment elevation on the anterior and lateral wall and positive enzymatic curve for infarction. He was initially treated with streptokinase with no reperfusion evidence after 3 hours of the onset of symptoms, so he underwent to rescue angioplasty. Angiography reported left main coronary artery aneurysm thrombosis. Afterwards, he presented cardiogenic shock and was revascularized with a coronary artery bypass graft of the mammary artery to the left anterior descending artery and the saphenous vein to the obtuse marginal, however he did not survive. Determination for 4G/5G PAI-1 polymorphism, glycoprotein IIIa PLA1/A2 gene and Glu298Asp polymorphism of the endothelial nitric oxide synthase gene was performed. CONCLUSIONS: Left main coronary artery aneurysms are rare, finding ONE in an acute myocardial infarction is a serious situation because of the challenging reperfusion techniques that are implied, such as in this case. The search for genetic factors related with hypofibrinolysis could guide stratification and therapy towards medical surgical or interventional management.

Múltiplos aneurismas coronarianos em pacientes com síndrome coronariana aguda / Multiple coronary artery aneurysms in a patient with acute coronary syndrome

Paciente do sexo masculino, 49 anos, foi admitido no Serviço de Emergência com quadro de taquicardia ventricular após síndrome coronariana aguda. A estratificação não invasiva foi realizada, inicialmente, com ecocardiograma transtorácico que evidenciou aneurisma ventricular esquerdo e disfunção sistólica moderada. Na análise segmentar observou-se acinesia anteroapical. Evidenciou-se ainda regurgitação mitral moderada e regurgitação aórtica leve. O paciente foi submetido, a seguir, à cineangiocoronariografia que evidenciou oclusão de artéria descendente anterior, aneurisma em coronária direita, circunflexa, diagonal e trombo organizado em ponta de ventrículo esquerdo. Decidiu-se pelo tratamento clínico.

Male patient, 49 years, was admitted to the ER with signs of ventricular tachycardia after acute coronary syndrome. Non-invasive stratification performed initially with a transthoracic echocardiogram showed a left ventricular aneurysm and moderate systolic dysfunction. The segment analysis showed anteroapical akinesis, as well as moderate mitral regurgitation and mild aortic regurgitation. The patient then underwent coronary angiography that indicated occlusion of the anterior descending artery, aneurysm in the right coronary artery,circumflex, diagonal, and organized thrombus in the left ventricle. Clinical treatment was selected.

[Left main coronary aneurysm and antiphospholipid syndrome: survival at 12 years. A case report and literature review]. / Aneurisma de tronco de coronaria izquierda y síndrome antifosfolípido: sobrevida a 12 años. Informe de un caso y revisión de la literatura.

Coronary artery aneurysms are a relatively infrequent finding with an incidence of 1% to 2% per year. Its cause can be atherosclerosis, congenital or due to other causes less common. Its initial manifestation can be myocardial infarction and sudden death as a result of rupture or distal embolization. The large coronary aneurysms, non-atherosclerotic, located in the common part of the left main coronary artery are exceptional. The diagnostic method of choice is the coronary angiography; however, non-invasive techniques such as transthoracic including tridimensional mode and transesophageal echocardiography, magnetic resonance imaging and computed tomography may have an important role in the detection and follow-up of these anomalies. The natural history of coronary aneurysm is not quite known. We present the case of a patient of 44 years, following an acute coronary event was diagnosed with an aneurysm in the left main and antiphospholipid syndrome. The patient received conservative treatment on the basis of antiplatelet and anticoagulant without presenting major cardiovascular events or other complications in 12 years of follow-up.

Cirurgia de revascularização miocárdica após infarto agudo do miocárdio causado por trombose de aneurisma coronariano / Coronary artery bypass graft surgery after acute myocardial infarction caused by thrombosis of coronary aneurysm

A morfologia do aneurisma coronariano configura um fator predisponente para formação de trombos. Porém, a estase sanguínea causada pela alteração do fluxo pode não ser o suficiente para causar fenômenos tromboembólicos.

The morphology of coronary aneurysm sets a predisposing factor to thrombus formation. However, the blood stasis caused by the change in flow may not be enough to cause thromboembolic events.

Coronary artery bypass graft surgery after acute myocardial infarction caused by thrombosis of coronary aneurysm.

The morphology of coronary aneurysm sets a predisposing factor to thrombus formation. However, the blood stasis caused by the change in flow may not be enough to cause thromboembolic events.