Rare association between giant-cell aortitis and giant-cell aortic valvulitis.

Giant cell arteritis (GCA) is a type of chronic vasculitis that affects medium and large-caliber arteries, frequently related to aortic involvement and, consequently, to aneurysm formation. However, associated valvulitis with giant cells is uncommon. We describe the case of a 50-year-old female patient with aortic aneurysm and valvular insufficiency, whose anatomopathological examination revealed giant-cell aortic valvulitis associated with giant cell aortitis.

Understanding yellow fever-associated myocardial injury: an autopsy study.

BACKGROUND: Yellow fever (YF) is a viral hemorrhagic fever, endemic in parts of South America and Africa. There is scarce evidence about the pathogenesis of the myocardial injury. The objective of this study is to evaluate the cardiac pathology in fatal cases of YF. METHODS: This retrospective autopsy study included cases from the São Paulo (Brazil) epidemic of 2017-2019. We reviewed medical records and performed cardiac tissue histopathological evaluation, electron microscopy, immunohistochemical assays, RT-qPCR for YF virus (YFV)-RNA, and proteomics analysis on inflammatory and endothelial biomarkers. FINDINGS: Seventy-three confirmed YF cases with a median age of 48 (34-60) years were included. We observed myocardial fibrosis in 68 (93.2%) patients; cardiomyocyte hypertrophy in 68 (93.2%); endothelial alterations in 67 (91.8%); fiber necrosis in 50 (68.5%); viral myocarditis in 9 (12.3%); and secondary myocarditis in 5 (6.8%). Four out of five patients with 17DD vaccine-associated viscerotropic disease presented with myocarditis. The cardiac conduction system showed edema, hemorrhages and endothelial fibrinoid necrosis. Immunohistochemistry detected CD68-positive inflammatory interstitial cells and YFV antigens in endothelial and inflammatory cells. YFV-RNA was detected positive in 95.7% of the cardiac samples. The proteomics analysis demonstrated that YF patients had higher levels of multiple inflammatory and endothelial biomarkers in comparison to cardiovascular controls, and higher levels of interferon gamma-induced protein 10 (IP-10) in comparison to sepsis (p = 0.01) and cardiovascular controls (p < 0.001) in Dunn test. INTERPRETATION: Myocardial injury is frequent in severe YF, due to multifactorial mechanisms, including direct YFV-mediated damage, endothelial cell injury, and inflammatory response, with a possible prominent role for IP-10. FUNDING: This study was funded by Fundação de Amparo à Pesquisa do Estado de São Paulo, Bill and Melinda Gates Foundation, Conselho Nacional de Desenvolvimento Científico e Tecnológico, Coordenação de Aperfeiçoamento de Pessoal de Nível Superior.

Case Report: The Challenge for Diagnosis of Myocarditis and Transplant Rejection After COVID Infection in a Heart-Transplanted Adolescent.

Patients who have undergone organ transplantation are immunosuppressed hosts, leaving them at a higher risk of infections. SARS-COV-2 has been shown to affect heart-transplanted patients. In this case report, we present the case of a 14-year-old heart transplant recipient who developed signs and symptoms of heart failure, along with fatigue, after a COVID-19 infection. An endomyocardial biopsy was performed to diagnose rejection and to evaluate whether this was myocarditis due to SARS-COV-2. The biopsy showed intense acute cellular rejection (3R) and antibody rejection PAMR1 H+ but was negative for the SARS-CoV-2 virus. The patient received organ rejection therapy with high-dose methylprednisolone and human immunoglobulin. After treatment, her heart function recovered, with biopsy investigations showing a lower level of cellular rejection (1R).

Superior caval vein syndrome and cardiac inflammatory myofibroblastic tumour in an infant.

The inflammatory myofibroblastic tumour, although very rare, must be considered in the differential diagnosis of intracardiac masses in children as it has systemic implications. We present a case of an infant whose diagnosis was suspected on clinical basis and echocardiogram, but the anatomopathological analysis with immunohistochemical study was essential for the conclusion of the histological type and orientation of the clinical follow-up.

Partial pericardiectomy for refractory acute tuberculous pericarditis: A case report.

INTRODUCTION: Tuberculosis is an infectious disease that usually manifests in the lungs but can also affect other organs, including the cardiovascular system. In this article, we present a rare case of purulent pericarditis caused by Mycobacterium tuberculosis. PRESENTATION OF CASE: A 67-year-old man was admitted to the emergency department with a large pericardial effusion with evidence of cardiac tamponade caused by acute pericarditis. The patient underwent surgical pericardial drainage, and a total volume of 500 mL of purulent fluid was collected with a positive culture for Mycobacterium tuberculosis. Despite antituberculous drugs, the patient presented with clinical worsening and recurrence of large pericardial effusion. Therefore, he was submitted to a second intervention by full median sternotomy to drain the pericardial effusion and perform a surgical pericardial debridement associated with a partial pericardiectomy. After the procedure, he improved clinically and was discharged after 24 days of hospitalization. DISCUSSION: Pericardiectomy is recommended for patients with refractory tuberculous pericarditis after four to eight weeks of antituberculous treatment. We decided not to wait that long to perform an open surgical partial pericardiectomy and debridement with a median sternotomy approach. We believe that this more aggressive surgical approach would be more efficient to combat the infection, which was causing progressive deterioration of patient's clinical condition and early recurrence of significant pericardial effusion. CONCLUSION: Open partial pericardiectomy with surgical debridement could be an efficient approach for treatment of a refractory acute tuberculous pericarditis.

Rare association between giant-cell aortitis and giant-cell aortic valvulitis

ABSTRACT Giant cell arteritis (GCA) is a type of chronic vasculitis that affects medium and large-caliber arteries, frequently related to aortic involvement and, consequently, to aneurysm formation. However, associated valvulitis with giant cells is uncommon. We describe the case of a 50-year-old female patient with aortic aneurysm and valvular insufficiency, whose anatomopathological examination revealed giant-cell aortic valvulitis associated with giant cell aortitis.

Cardiovascular findings in Williams-Beuren Syndrome: Experience of a single center with 127 cases.

Williams-Beuren syndrome (WBS) is a rare, microdeletion syndrome characterized by facial dysmorphisms, intellectual disability, a friendly personality, cardiovascular and other abnormalities. Cardiovascular defects (CVD) are among the most prevalent characteristics in WBS, being supravalvular aortic stenosis (SVAS) the most frequent, followed by peripheral pulmonary stenosis (PPS). A comprehensive retrospective review of medical records of 127 patients with molecular diagnosis of WBS, in a period of 20 years, was done to evaluate the incidence, the natural history of cardiovascular disease, and the need for surgical intervention, including heart transplantation (HT). A total of 94/127 patients presented with CVD. Of these 94 patients, 50% presented with SVAS and 22.3% needed heart surgery and/or cardiac catheterization including one that required HT due to severe SVAS-related heart failure at 19 years of age. The patient died in the postoperative period due to infectious complications. Cardiovascular problems are the major cause of sudden death in patients with WBS, who have a significantly higher mortality risk associated with surgical interventions. There is a higher risk for anesthesia-related adverse events and for major adverse cardiac events following surgery. End-stage heart failure due to myocardial ischemia has been described in WBS patients and it is important to consider that HT can become their only viable option. To our knowledge, the case mentioned here is the first HT reported in an adolescent with WBS. HT can be a viable therapeutic option in WBS patients with adequate evaluation, planning, and a multidisciplinary team to provide the required perioperative care and follow-up.

Diagnostic challenges in systemic amyloidosis: a case report with clinical and laboratorial pitfalls.

Currently, there is growing evidence in the literature warning of misdiagnosis involving amyloidosis and chronic inflammatory demyelinating polyneuropathy (CIDP). Although inducing clinical manifestations outside the peripheral nervous system, light chain and transthyretin amyloidosis may initially present with peripheral neuropathy, which can be indistinguishable from CIDP, leading to a delay in the correct diagnosis. Besides, the precise identification of the amyloid subtype is often challenging. This case report exemplifies clinical and laboratory pitfalls in diagnosing amyloidosis and subtyping amyloid, exposing the patient to potentially harmful procedures.

3D Echocardiography for Rheumatic Heart Disease Analysis: Ready for Prime Time.

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.

Catheter Ablation of Focal Atrial Tachycardia with Early Activation Close to the His-Bundle from the Non Coronary Aortic Cusp. / Ablação por Cateter de Taquicardia Atrial Focal com Ativação Precoce Próxima ao Feixe de His, a Partir da Cúspide Aórtica não Coronária.

BACKGROUND: Atrial tachycardia (AT) ablation with earliest activation site close to the His-Bundle is a challenge due to the risk of complete AV block by its proximity to His-Purkinje system (HPS). An alternative to minimize this risk is to position the catheter on the non-coronary cusp (NCC), which is anatomically contiguous to the para-Hisian region. OBJECTIVES: The aim of this study was to perform a literature review and evaluate the electrophysiological characteristics, safety, and success rate of catheter-based radiofrequency (RF) delivery in the NCC for the treatment of para-Hisian AT in a case series. METHODS: This study performed a retrospective evaluation of ten patients (Age: 36±10 y-o) who had been referred for SVT ablation and presented a diagnosis of para-Hisian focal AT confirmed by classical electrophysiological maneuvers. For statistical analysis, a p-value of <0.05 was considered statistically significant. RESULTS: The earliest atrial activation at the His position was 28±12ms from the P wave and at the NCC was 3±2ms earlier than His position, without evidence of His potential in all patients. RF was applied on the NCC (4-mm-tip catheter; 30W, 55ºC), and the tachycardia was interrupted in 5±3s with no increase in the PR interval or evidence of junctional rhythm. Electrophysiological tests did not reinduce tachycardia in 9/10 of patients. There were no complications in all procedures. During the 30 ± 12 months follow-up, no patient presented tachycardia recurrence. CONCLUSION: The percutaneous treatment of para-Hisian AT through the NCC is an effective and safe strategy, which represents an interesting option for the treatment of this complex arrhythmia. (Arq Bras Cardiol. 2021; 116(1):119-126).

FUNDAMENTO: A ablação da taquicardia atrial (TA) com local de ativação mais precoce próxima ao feixe de His é um desafio, devido ao risco de bloqueio de AV completo por sua proximidade ao sistema de His-Purkinje (SHP). Uma alternativa para minimizar esse risco é posicionar o cateter na cúspide não coronária (CNC), que é anatomicamente contígua à região para-Hissiana. OBJETIVOS: O objetivo deste estudo foi fazer uma revisão de literatura e avaliar as características eletrofisiológicas, a segurança e o índice de sucesso de aplicação de radiofrequência (RF) por cateter na CNC para o tratamento de TA para-Hissiana em uma série de casos. MÉTODOS: Avaliamos retrospectivamente dez pacientes (Idade: 36±10 anos) que foram encaminhados para ablação de taquicardia paroxística supraventricular (TPSV) e haviam sido diagnosticados com TA focal para-Hissiana confirmada por manobras eletrofisiológicas clássicas. Para a análise estatística, um P valor d <0.05 foi considerado estatisticamente significativo. RESULTADOS: A ativação atrial mais precoce na posição His foi de 28±12ms da onda P, e a CNC foi 3±2ms antes da posição His, sem evidência de potencial His em todos os pacientes. Foi aplicada RF à CNC (cateter de ponta de 4-mm; 30W, 55°C) e a taquicardia foi interrompida em 5±3s sem aumento no intervalo PR ou evidência de um ritmo juncional. Os testes eletrofisiológicos não induziram novamente a taquicardia em 9/10 pacientes. Não houve complicações em nenhum procedimento. Durante o período de acompanhamento de 30 ± 12 meses, nenhum paciente apresentou recorrência de taquicardia. CONCLUSÃO: O tratamento percutâneo de TA para-Hissiana por meio de CNC é uma estratégia segura e eficiente, tornando-se uma opção interessante para o tratamento de arritmia complexa. (Arq Bras Cardiol. 2021; 116(1):119-126).

Diagnostic challenges in systemic amyloidosis: a case report with clinical and laboratorial pitfalls

Currently, there is growing evidence in the literature warning of misdiagnosis involving amyloidosis and chronic inflammatory demyelinating polyneuropathy (CIDP). Although inducing clinical manifestations outside the peripheral nervous system, light chain and transthyretin amyloidosis may initially present with peripheral neuropathy, which can be indistinguishable from CIDP, leading to a delay in the correct diagnosis. Besides, the precise identification of the amyloid subtype is often challenging. This case report exemplifies clinical and laboratory pitfalls in diagnosing amyloidosis and subtyping amyloid, exposing the patient to potentially harmful procedures.

Left Ventricular Noncompaction Is More Prevalent in Ventricular Septal Defect Than Other Congenital Heart Defects: A Morphological Study.

Left ventricular noncompaction (LVNC) is a condition characterized by prominent ventricular trabeculae and deep intertrabecular recesses and has been described as a possible substrate for arrhythmias, thromboembolism, and heart failure. Herein, we explored the prevalence of LVNC morphology among hearts with congenital heart defects (CHD). We examined 259 postnatal hearts with one of the following CHD: isolated ventricular septal defect (VSD); isolated atrial septal defect (ASD); atrioventricular septal defect (AVSD); transposition of the great arteries (TGA); isomerism of the atrial appendages (ISOM); Ebstein's malformation (EB); Tetralogy of Fallot (TF). Eleven hearts from children who died of non-cardiovascular causes were used as controls. The thickness of the compacted and non-compacted left ventricular myocardial wall was determined and the specimens classified as presenting or not LVNC morphology according to three criteria, as proposed by Chin, Jenni, and Petersen. Normal hearts did not present LVNC, but the CHD group presented different percentages of LVNC in at least one diagnostic criterium. The prevalence of LVNC was respectively, according to Chin's, Jenni´s and Petersen´s methods: for VSD-54.2%, 35.4%, and 12.5%; ASD-8.3%, 8.3%, and 8.3%; AVSD-2.9%, 2.9%, and 0.0%; TGA-22.6%, 17%, and 5.7%; ISOM-7.1%, 7.1%, and 7.1%; EB-28.6%, 9.5%, and 0.0%; TF-5.9%. 2.9%, and 2.9%. VSD hearts showed a significantly greater risk of presenting LVNC when compared to controls (Chin and Jenni criteria). No other CHD presented similar risk. Current results show some agreement with previous studies, such as LVNC morphology being more prevalent in VSDs. Nonetheless, this is a morphological study and cannot be correlated with symptoms or severity of the CHD.

Progressive dyspnea and a right atrial mass in an 80-year-old man.

Hepatocellular carcinoma (HCC) is among the five most frequent causes of cancer death worldwide, according to the WHO. The disease is related to alcohol abuse, viral infections, and other causes of cirrhosis, and unfortunately, in some developed countries, the incidence shows an increasing trend. Although the diagnosis of the HCC often relies upon the context of a chronic hepatopathy, some cases may present a silent course, and the initial symptoms ensue when the disease is in an advanced stage with no chance for any therapeutic attempt. The clinical picture of the HCC is varied, and unexpected forms may surprise the clinician. One of the unusual presentations of the HCC is shock by the blockage of the venous return to the right atrium by the inferior vena cava infiltration. Herein we present a case of an old patient who sought medical care complaining of dyspnea. The clinical workup disclosed a right thorax pleural effusion and imaging exams depicted a mass in the right hepatic lobe, invasion of the inferior vena cava (IVC) and the right atrium (RA). During the attempts of clinical investigation, the patient passed away. The autopsy disclosed an HCC involving the right hepatic lobe, with the invasion of the IVC and the RA. The authors highlight the importance of recognizing the bizarre presentation of not so rare diseases.