Cardiomiopatia em ­dente de serra­ ­ cardiogenética em foco / Sawtooth cardiomyopathy ­ cardiogenetics in focus

APRESENTAÇÃO DO CASO: R.C, sexo masculino, 43 anos, hipertenso, encaminhado ao nosso serviço para investigação de insuficiência cardíaca (IC), oligossintomático. Realizada investigação etiológica, sendo evidenciado em ecocardiograma transtorácico disfunção ventricular esquerda de grau moderado (FEVE 38% pelo método de Simpson), com hipocontratilidade miocárdica de toda região apical e presença de endentações nas regiões apicais (do septo, da parede inferior e da parede lateral) e no segmento médio da parede inferolateral. Os mesmos achados foram identificados em ressonância nuclear magnética cardíaca e foi observado áreas de realce tardio subendocárdico nas regiões correspondentes as endentações. No holter foram registrados episódios de arritmias ventriculares isoladas (1129) e aos pares (19). Iniciado tratamento medicamentoso para IC com bom controle sintomático até o momento. Considerando o quadro clínico do paciente, podemos considerar uma forma benigna da doença. Realizada coleta de teste genético do paciente e convocado familiares de primeiro grau para rastreio. DISCUSSÃO: A cardiomiopatia em dente de serra é uma doença muito rara, com apenas três casos publicados desde sua primeira descrição na literatura. Tipo incomum de displasia ventricular esquerda que se caracteriza por múltiplas projeções do miocárdio compactado que faz aparência de ­dente de serra­ e pontes musculares entre as paredes inferior e lateral em imagens não invasivas. Todos os casos relatados são do sexo masculino o que levanta a possibilidade de distúrbio ligado ao cromossomo X. COMENTÁRIOS FINAIS: A história natural da doença ainda é incerta, assim como suas possíveis complicações, já sendo descrito distúrbios de condução em relatos prévios. Até o momento sendo indicado rastreio familiar genético. Casos adicionais são necessários para melhor entendimento e manejo da doença.

Fungal endocarditis: what is the best time for surgery? The role of a heart team

Fungal endocarditis is an uncommon affliction with high mortality rate; it represents 2% of infectious endocarditis cases with a mortality rate of about 50% and many times diagnosed post-mortem.¹² It most frequently affects an adult's aortic or mitral valves. Standardized treatment requires antifungal therapy associated with surgical treatment.³ If there is an uncontrolled fungal infection, an early valvular replacement is recommended (<7 days). Patients that have received only medical treatment have a 100% mortality rate.² 78-year-old male, diabetic, with a previous bioprosthetic aortic valve replacement due to severe aortic stenosis, attended the emergency department with fever for the past two months, initially once a week and recently twice daily, associated with nonspecific unwellness and chills. He brought transthoracic echocardiogram that suggested prosthetic valve endocarditis. The patient was hospitalized. Blood cultures were taken and a transesophageal echocardiogram done, which showed typical vegetation annexed to the prosthetic's aortic ring (7x7mm) with mobile component projecting out of the left ventricle and restricting the opening of the prosthetic valve in this region, causing an increase of its gradients. A multi-sensitive Candida parapsilosis was shown in the blood cultures and then Anidulafungin 200mg/day was initiated for an estimated 42-day treatment.The case was discussed with Heart Team because of the high surgical risk and the early need of valve replacement. Initial course of action had us schedule surgery for the seventh day of the antifungal treatment. Patient maintained daily fever and blood cultures were collected every 72h showing increase in Candida parapsilosis in all samples and surgery was sped up.The patient was submitted to a bioprosthetic aortic valve replacement with amplification of the ring due to the presence of abscess evidenced in mid-surgery. The patient was admitted in critical condition to the ICU, hemodynamically unstable in use of ascending vasoactive drugs, in vasoplegic shock with refractory septic component. The patient remained refractory to instituted measures evolving to death 24h after surgery. Fungal endocarditis is a rare but extremely serious condition, and a multidisciplinary approach is extremely important. Discussing a case of reoperation with high surgical risk with all those responsible for the care is the role of Heart Team and was essential to decide the best time for the surgery.

Case report: an unexpected case of minoca in a 56-year-old male with myocardial bridge

INTRODUCTION: A myocardial bridge (MB) is typically asymptomatic, but sometimes can lead to a myocardial infarction with several mechanisms such as: coronary spasm, thrombosis, coronary dissection, or the development of focal atherosclerosis immediately proximal to the MB. Sometimes more than one mechanism can be present. The use of intracoronary images modalities, as Optical Coherence Tomography (OCT) in patient with MB can accurately define the mechanism of the myocardial infarction and provide further guidance to management strategy. CASE REPORT: 56-yearold male with past medical history of systemic arterial hypertension and active tobacco use, attended the emergency department with severe oppressive chest pain after a long car trip in the heat. The initial electrocardiogram showed ST-elevation in anterior leads. Coronary angiography revealed a myocardial bridge with a systolic constriction of more than 90% in the proximal third of the left anterior descending artery (LAD), OCT demonstrate a lesion < 20% with no signs of rupture. The patient was diagnosed with MINOCA with multiple mechanisms such as supply and demand imbalance, cigarette-induced vasospasm and hypovolemia. The patient was treated with beta blockers and antiplatelet therapy and discharged 3 days later. There was no recurrence of chest pain at follow-up appointments. CONCLUSION: Myocardial bridge (MB) is a congenital anatomic anomaly whereby a length of the artery tunnels beneath a section of myocardium. It can potentially be associated with phasic arterial spasm and ischemia. About 67-98% of cases have their anatomical location in the LAD. The gold standard for diagnosis is autopsy, but angiography and OCT provides more information about MBs, such as assessment of vulnerable plaque and coronary morphology. Unlike classic atherosclerotic plaque that produces a fixed stenosis, MB produces a dynamic effect that varies with cardiac cycle, heart rate, and sympathetic tone. According to the classification by Schwarz 2009, this case is classified as type C, with altered intracoronary hemodynamics. The clinical management was maintained with pharmacological therapy, which is the mainstay treatment. Angioplasty would be suggested only for cases with refractory symptoms. The patient was conducted with medications for management of heart failure and general orientation, with no recurrence of chest pain.

Aneurysmal dilation of the coronaries: a case series

Aneurysmal dilation of the coronaries arteries has a prevalence rate between 1-3% (1). It can be classified in aneurism or ectasia. The first, defined as a focal dilation of about 1.5x the normal diameter and the second as a diffuse dilation The right coronary is the most targeted with 40% of cases, followed by 32% of the left anterior descending (2). Fifty percent are associated with atherosclerosis, the rest is caused by congenital anomalies and inflammatory diseases, such as Kawasaki`s (1). Most are clinically silent and are only detected incidentally during coronary angiography or computed tomography. Due to the lack of evidence, management is challenging. Usually, these patients are more susceptible to presenting complications like local thrombosis, distal embolization and myocardial infarction (5). The use of anticoagulants in the context of the acute coronary syndrome (ACS) is implicated in the reduction of major adverse cardiovascular events (MACE) in observational studies. Similar evidence sustains the use of dual antiplatelets (2,4). We present here a serie of 9 cases with aneurysm/coronary ectasia with median follow up of 3,5 years. All 9 patients had Myocardial infarction with non-obstructive coronary artery, 7 with ST-Elevation, due to aneurysm/coronary ectasia, of which 6 were men and 3 women, median age 54,5 years. Two patients had a family history of early coronary artery disease and previous infarction. Three patients had diabetes, history of smoking and obesity. Four patients were dyslipidemic and six had history of hypertension. One patient had atrial fibrillation/Flutter. The ejection fraction ranged from 40 to 60%, with an average of 52.7%. In the treatment, all patients were using statins and beta-blockers. Regarding antiplatelet and anticoagulation of these patients, 2 were using warfarin associated with P2Y12 inhibitors (iP2Y12), 1 was using warfarin alone, 1 was using direct-acting oral anticoagulant alone, 1 was using aspirin alone, 1 was using warfarin associated with aspirin and iP2Y12, and 2 used aspirin associated with iP2Y12. One of the patients evolved with dyspneia and 3 with angina. Three patients had another ACS. No deaths were recorded. Aneurysmal dilation of coronary vessels is a challenging and misdiagnosed entity, with lack of evidence, supporting an individualized management.

Infarto agudo do miocárdio como complicação de mixoma atrial / Acute myocardial infarction as a complication of atrial myxoma

INTRODUÇÃO: Os mixomas são as neoplasias cardíacas primárias mais comuns. Geralmente se apresentam com sintomas de doença valvar mitral ou de eventos tromboembólicos. Apesar de mais frequentes em mulheres, os homens apresentam maior probabilidade de apresentar evidências de embolização. A incidência de embolização está associada a tamanhos menores (≤4,5 cm). Embora a embolização sistêmica seja frequente, o envolvimento da artéria coronária, levando a sua oclusão e consequentemente infarto agudo do miocárdio (IAM), é extremamente raro. OBJETIVO: Descrição de uma série de casos de associação de mixomas com IAM por embolização coronariana. MÉTODOS: Análise retrospectiva de prontuários de hospital terciário de Cardiologia, dos últimos 38 anos. Foram avaliados 138 pacientes portadores de mixomas. RESULTADOS A associação de mixoma atrial com evento coronariano foi evidenciado em 3 pacientes, do sexo feminino, de 28, 47 e 48 anos. A apresentação clínica inicial foram sintomas de insuficiência cardíaca em 1 paciente; as outras duas eram assintomáticas. A ressonância magnética cardíaca (RMC) definiu realce tardio de padrão coronariano com acometimento transmural e redução significativa da fração de ejeção nas três pacientes. Os mixomas tinham grandes dimensões, o menor com 4,5 cm de diâmetro. As principais características dos eventos estão descritas na tabela1. CONCLUSÃO: A ocorrência de embolização coronária na evolução dos mixomas deve ser lembrada e pesquisada, principalmente nos pacientes que têm redução de fração de ejeção. A ausência de sinais e sintomas específicos dificulta o diagnóstico e retarda o tratamento. Estes achados sugerem a recomendação do tratamento cirúrgico precoce e anticoagulação assim que o diagnóstico de mixoma for feito para prevenção das complicações embólicas.