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Rheumatic fever (RF) and rheumatic heart disease (RHD) are still highly prevalent, particularly in low- and middle-income countries. RHD is a neglected and underdiagnosed disease for which no specific laboratory diagnostic test is completely reliable. This is a retrospective observational study, which included 118 patients with RHD who underwent cardiac surgery from 1985 to 2018. The aim of this investigation was to evaluate the clinical, epidemiological, echocardiographic and pathological characteristics in two cohorts of RHD patients: one cohort with Aschoff bodies present in their pathological results and the other without such histopathological characteristics. No conventional clinical and laboratory tests for RHD myocarditis were able to identify active carditis during the preoperative phase of valve repair or replacement. Patients who had Aschoff bodies in their pathological results were younger (median age of 13 years (11-24 years) vs. 27 years (17-37 years), p = 0.001) and had higher rate of late mortality (22.9% vs. 5.4%, p = 0.043). In conclusion, the presence of Aschoff bodies in pathological findings may predict increased long-term mortality, emphasizing the importance of comprehensive pathology analysis for suspected myocarditis during heart surgery.

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We present an unusual case of a 67-year-old woman with an incidental finding of a cardiac mass on a chest computed tomography. Coronary angiotomography confirmed the diagnosis of right coronary artery aneurysm, with 5.7×5.7 cm. The patient underwent aneurysm resection and coronary bypass surgery, with subsequent histologic study suggestive of arteritis sequelae. Giant coronary artery aneurysms have a high risk of complications and aneurysm exclusion must be beneficial. This is a rare condition that can also be part of a systemic inflammatory disease.

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Abstract We present an unusual case of a 67-year-old woman with an incidental finding of a cardiac mass on a chest computed tomography. Coronary angiotomography confirmed the diagnosis of right coronary artery aneurysm, with 5.7×5.7 cm. The patient underwent aneurysm resection and coronary bypass surgery, with subsequent histologic study suggestive of arteritis sequelae. Giant coronary artery aneurysms have a high risk of complications and aneurysm exclusion must be beneficial. This is a rare condition that can also be part of a systemic inflammatory disease.

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BACKGROUND: Epidemiological data from the COVID-19 pandemic report that patients with pre-existing cardiovascular disease have worse outcomes and higher mortality, and that pregnant women should be considered at high risk. CASE SUMMARY: A 25-year-old pregnant woman on the waiting list for a heart transplant, with a history of complete atrioventricular canal surgery, mitral mechanical prosthetic implant (St Jude-27), and cardiac resynchronization therapy (Boston Scientific) was hospitalized at 30 weeks of gestation for treatment of heart failure. After 7 days of hospitalization, she had a positive RT-PCR test for severe acute respiratory syndrome coronavirus 2 with progressive worsening of her clinical condition and acute foetal distress. Hence emergency caesarean section was performed. After the birth, the patient required mechanical ventilation, progressing to multiple organ system failures. Conventional inotropic drugs, antibiotics, and mechanical ventilation for 30 days in the intensive care unit provided significant clinical, haemodynamic, and respiratory improvement. However, on the 37th day, she suddenly experienced respiratory failure, gastrointestinal and airway bleeding, culminating in death. DISCUSSION: Progressive physiological changes during pregnancy cause cardiovascular complications in women with severe heart disease and higher susceptibility to viral infection and severe pneumonia. COVID-19 is known to incite an intense inflammatory and prothrombotic response with clinical expression of severe acute respiratory syndrome, heart failure, and thromboembolic events. The overlap of these COVID-19 events with those of pregnancy in this woman with underlying heart disease contributed to an unfortunate outcome and maternal death.

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Severe tricuspid regurgitation is especially caused by pulmonary hypertension. Primary tricuspid regurgitation in the absence of pulmonary hypertension and of unknown etiology is a very rare condition with scarce data about its diagnosis, treatment, and follow-up. The particularities of surgery indication and outcomes are still not clearly known. A 72-year-old woman with a medical history of coronary artery bypass grafting three years ago presented with shortness of breath and low limb edema. Physical examination revealed a prominent bilateral jugular turgescence, hepatomegaly, peripheral edema, and a left midsternal border holosystolic murmur, suggestive of tricuspid regurgitation. The echocardiogram confirmed the diagnosis and showed preserved right and left ventricular dimensions and function. Coronary angiography showed no new obstructive lesions and patent surgical grafts. Right cardiac catheterization revealed mild pulmonary hypertension and increased right atrium pressure. Cardiac magnetic resonance showed mild right ventricular dilation with normal systolic function and normal left chambers. No late gadolinium enhancement was detected. Because of persistent symptoms, even after optimization of medical therapy, the patient was submitted to tricuspid valve replacement surgery. Immediately after the surgery, the patient developed significant right ventricular dysfunction, with the need of continuous hemodynamic support. She had progressive clinical recovery that was confirmed by serial echocardiograms that showed improvement in right ventricular volume and function. The patient was discharged with no signs or symptoms of right heart failure. The histopathological examination showed significant and diffuse myxomatous degeneration of the leaflets. No signs of infection or vegetation nor disruption of strands were observed. This report illustrates a very rare case of symptomatic primary isolated severe tricuspid regurgitation caused by myxomatous degeneration of the leaflets. The thoroughly diagnostic workup is presented, and only the histopathological analysis of the leaflets revealed the etiologic process. Surgical treatment indicated before the onset of right ventricular failure was essential to patient's full recovery.

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Rheumatic heart disease (RHD) remains to be a very important health issue worldwide, mainly in underdeveloped countries. It continues to be a leading cause of morbidity and mortality throughout developing countries. RHD is a delayed non-suppurative immunologically mediated inflammatory response to the throat infection caused by a hemolytic streptococcus from the A group (Streptococcus pyogenes). RHD keeps position 1 as the most common cardiovascular disease in young people aged <25 years considering all the continents. The disease can lead to valvular cardiac lesions as well as to carditis. Rheumatic fever valvular injuries lead most commonly to the fusion and thickening of the edges of the cusps and to the fusion, thickening, and shortening of the chordae and ultimately to calcification of the valves. Valvular commissures can also be deeply compromised, leading to severe stenosis. Atrial and ventricular remodeling is also common following rheumatic infection. Mixed valvular lesions are more common than isolated valvular disorders. Echocardiography is the most relevant imaging technique not only to provide diagnostic information but also to enable prognostic data. Further, it presents a very important role for the correction of complications after surgical repair of rheumatic heart valvulopathies. Three-dimensional (3D) echocardiography provides additional anatomical and morphofunctional information of utmost importance for patients presenting rheumatic valvopathies. Accordingly, three-dimensional echocardiography is ready for routine use in patients with RHD presenting with valvular abnormalities.

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Streptococcus pyogenes infections continue to be a worldwide public health problem, causing various diseases in humans, with rheumatic fever and rheumatic heart disease being the most harmful manifestations. Impetigo and post-streptococcal glomerulonephritis are also important sequelae of skin infections. We have developed a candidate vaccine epitope (StreptInCor) that presents promising results in diverse animal models. To assess whether the StreptInCor alum-adsorbed vaccine could induce undesirable effects, a certified independent company conducted a repeated intramuscular dose toxicity evaluation in Wistar rats, a choice model for toxicity studies. We did not observe significant alterations in clinical, hematological, biochemical, anatomical, or histopathological parameters due to vaccine administration, even when the animals received the highest dose. In conclusion, repeated intramuscular doses did not show signs of macroscopic or other significant changes in the clinical or histopathological parameters, indicating that StreptInCor can be considered a safe candidate vaccine.

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OBJECTIVE: To compare the arterial response of cases of acute and chronic embolism, seeking to associate ischemic pulmonary remodeling with progression to chronicity. METHODS: Retrospective analysis of 61 necropsies of patients who died in the Instituto do Coração (31 cases of pulmonary embolism and 30 cases of acute myocardial infarction). Slides of pulmonary tissue were obtained from all cases and analyzed qualitative and quantitatively (medial thickness measurement). RESULTS: Qualitative analysis enabled the differentiation between cases of embolism and the control group, thus characterizing the two groups and defining the adequate choice of the control group. The alterations predominated in patients with embolism (alveolar inflammation and edema, infarction, vasoconstriction, concentric intimal proliferation, presence of thrombus). Quantitative analysis demonstrated higher percent medial thickness in the cases of embolism than in the control group; among the cases of embolism, no differences in intra (acute--19.74 and chronic--20.04) and pre-acinar (acute--18.85 and chronic--18.68) arteries were observed. CONCLUSION: The lack of difference among the groups with embolism and the higher values of percent medial thickness in the peripheral arteries allow the conclusion that the vascular response is more intense and starts in these arteries.

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OBJETIVO: Comparar a resposta arterial dos casos de embolia aguda e crônica, procurando associar o remodelamento isquêmico pulmonar à evolução para cronificação. MÉTODOS: Análise retrospectiva de 61 casos de necropsia de pacientes que evoluíram a óbito no Instituto do Coração (31 casos de embolia pulmonar e 30 casos de infarto agudo do miocárdio). Foram obtidas lâminas de tecido pulmonar de todos os casos e análise qualitativa e quantitativa (mensuração da espessura da camada média das artérias). RESULTADOS: A análise qualitativa permitiu diferenciar os casos de embolia do grupo controle, caracterizando os dois grupos e definindo a escolha adequada do grupo controle. Houve predomínio das alterações nos pacientes com embolia (edema e inflamação alveolar, infarto, vasoconstrição, proliferação intimal concêntrica, presença de trombo). A análise quantitativa demonstrou porcentagem de espessura da camada média mais elevados nos casos de embolia do que no grupo controle; dentre os casos de embolia não houve diferença nas artérias intra (agudo - 19,74 e crônico - 20,04) e pré-acinares (agudo - 18,85 e crônico - 18,68). CONCLUSÃO: A ausência de diferença entre os grupos com embolia e os valores mais elevados de porcentual de espessura nas artérias da periferia permite concluir que a resposta vascular é mais intensa e se inicia nessas artérias.

OBJECTIVE: To compare the arterial response of cases of acute and chronic embolism, seeking to associate ischemic pulmonary remodeling with progression to chronicity. METHODS: Retrospective analysis of 61 necropsies of patients who died in the Instituto do Coração (31 cases of pulmonary embolism and 30 cases of acute myocardial infarction). Slides of pulmonary tissue were obtained from all cases and analyzed qualitative and quantitatively (medial thickness measurement). RESULTS: Qualitative analysis enabled the differentiation between cases of embolism and the control group, thus characterizing the two groups and defining the adequate choice of the control group. The alterations predominated in patients with embolism (alveolar inflammation and edema, infarction, vasoconstriction, concentric intimal proliferation, presence of thrombus). Quantitative analysis demonstrated higher percent medial thickness in the cases of embolism than in the control group; among the cases of embolism, no differences in intra (acute - 19.74 and chronic - 20.04) and pre-acinar (acute - 18.85 and chronic - 18.68) arteries were observed. CONCLUSION: The lack of difference among the groups with embolism and the higher values of percent medial thickness in the peripheral arteries allow the conclusion that the vascular response is more intense and starts in these arteries.

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Amiodarone is a class III antiarrhythmic medication used extensively to treat ventricular arrhythmias. It is pharmacologically classified as a cationic amphiphilic drug due to its polar and apolar components. During the past few years, amiodarone has proved to be an effective treatment therapy for patients with ventricular dysfunctions, regardless of the etiology, and in particular ventricular arrhythmia associated with Chagas disease. Nevertheless, despite its hemodynamic and electrophysiological benefits, amiodarone produces serious collateral effects such as a bluish skin discoloration, photosensitivity, thyroid dysfunction, corneal deposit, peripheral neuropathy, bone marrow suppression, hepatitis, heart blocks, pneumonitis, among others. The objective of this case report is to discuss one of the most serious complications related to amiodarone, pulmonary toxicity, which is described as a differential diagnosis for a chagasic patient that was on the waiting list for a heart transplant. Amiodarone pneumonitis is a relevant differential diagnosis for heart failure (HF) patients using amiodarone that are admitted to the emergency ward with dyspnea.

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A amiodarona é um antiarrítmico da classe III, amplamente utilizado em arritmias ventriculares¹. Farmacologicamente é classificado como uma drogra ampifílica catiônica, pelos seus constituintes polares e apolares. Nos últimos anos, a amiodarona obteve destaque pelo seu uso em portadores de disfunção ventricular por qualquer etiologia, em especial a chagásica, quando ocorrem arritmias ventriculares². Entretanto, a despeito de seus benefícios hemodinâmicos e eletrofisiológicos, a amiodarona produz efeitos colaterais relevantes, como coloração azulada da pele, fotossensibilidade, disfunção tireoidiana, depósito corneal, neuropatia periférica, supressão da medula óssea, hepatite, bloqueios cardíacos, pneumonites e outros³. Este relato de caso se propõe a abordar uma de suas mais sérias complicações, a toxicidade pulmonar, aqui especialmente descrita como diagnóstico diferencial em um paciente chagásico que aguardava em fila de transplante cardíaco. Pneumonite por amiodarona constitui-se em um importante diagnóstico diferencial entre os pacientes que se apresentam na sala de emergência com dispnéia, quando estes são portadores de insuficiência cardíaca (IC) e estão em uso dessa droga.

Amiodarone is a class III antiarrhythmic medication used extensively to treat ventricular arrhythmias¹. It is pharmacologically classified as a cationic amphiphilic drug due to its polar and apolar components. During the past few years, amiodarone has proved to be an effective treatment therapy for patients with ventricular dysfunctions, regardless of the etiology, and in particular ventricular arrhythmia associated with Chagas disease². Nevertheless, despite its hemodynamic and electrophysiological benefits, amiodarone produces serious collateral effects such as a bluish skin discoloration, photosensitivity, thyroid dysfunction, corneal deposit, peripheral neuropathy, bone marrow suppression, hepatitis, heart blocks, pneumonitis, among others³. The objective of this case report is to discuss one of the most serious complications related to amiodarone, pulmonary toxicity, which is described as a differential diagnosis for a chagasic patient that was on the waiting list for a heart transplant. Amiodarone pneumonitis is a relevant differential diagnosis for heart failure (HF) patients using amiodarone that are admitted to the emergency ward with dyspnea.

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