Fatores associados ao aumento da espessura do septo atrial em lactentes com Síndrome da Hipoplasia do Coração Esquerdo (SHCE): implicações para septostomia percutânea

Introdução: Nos procedimentos usualmente realizados em recém-nascidos acometidos pela Síndrome da Hipoplasia do Coração Esquerdo (SHCE), variações fenotípicas influenciam o sucesso do tratamento, principalmente as características relacionadas ao septo atrial e ao forame oval (FO) no caso de septostomia por cateter. Objetivos: Analisar características macroscópicas do septo atrial de corações com SHCE, a fim de definir e orientar procedimentos terapêuticos nessa estrutura. Métodos: Foram avaliados 18 corações de pacientes falecidos e diagnosticados com SHCE quanto à perviedade e tamanho do FO, abaulamento da lâmina, atresia ou perviedade da válvula mitral e calibre da aorta ascendente e tronco pulmonar. Cortes histológicos do septo atrial foram feitos para medidas da espessura máxima e mínima da lâmina. Resultados: A idade média dos pacientes foi de 34,5 dias (57% do sexo masculino), com espessura do septo atrial médio de 1,90 mm (0,63 - 4,09 mm). O diâmetro médio do tronco e da aorta pulmonar foi de 1,16 cm e 0,22 cm, respectivamente. O FO era patente em 39% dos casos. A valva mitral era atrésica em 21% das amostras. Houve diferença significativa na espessura do septo atrial nos casos com FO patente ou fechada, sendo maior nos casos em que a FO era fechada (p = 0,047). A relação FO / idade apresentou correlação negativa estatisticamente significante com a espessura do septo atrial (r = -0,76 ep <0,05). Conclusões: Os resultados indicam que a perviedade e o tamanho do forame oval têm repercussões na espessura do septo atrial, sugerindo que esse fator pode limitar o sucesso de intervenções terapêuticas, principalmente na septostomia por cateter.Introduction: In the usual procedures performed on newborns affect by Hypoplastic Left Heart Syndrome (HLHS), phenotypic variations influence the success of the treatment, especially the characteristics related to the atrial septum and foramen ovale (FO) in case of catheter septostomy. Objectives: To analyze macroscopics features of the atrial septum of hearts with HLHS in order to define and guide therapeutic procedures on this structure. Methods: 18 hearts of deceased patients diagnosed with HLHS were evaluated for FO patency and size, bulging of its blade, atresia or patency of the mitral valve and caliber of the ascending aorta and pulmonary trunk. Histological sections of the atrial septum were made for measurements of maximum and minimum thickness of the lamina. Results: The mean age of the patients was 34.5 days (57% male), with a mean atrial septal thickness of 1.90 mm (0.63 - 4.09 mm). The mean diameter of the pulmonary trunk and aorta were 1.16 cm and 0.22 cm, respectively. FO was patent in 39% of cases. The mitral valve was atresic in 21% of the specimens. There was a significant difference in the thickness of the atrial septum in cases with patent or closed FO, being greater in cases where the FO was closed (p = 0.047). The ratio FO/age presented a statistically significant negative correlation with atrial septum thickness (r = -0.76 and p <0.05). Conclusions: The results indicate that the patency and size of the oval foramen have repercussions on the thickness of the atrial septum, suggesting that this factor may limit the success of therapeutic interventions, especially catheter septostomy

Aortic stenosis concomitant with microscopic polyangiitis: a challenge in medical reasoning and thinking

Microscopic polyangiitis (MPA) is part of the anti-neutrophil cytoplasmic antibodies (ANCA)-related vasculitis, which usually presents as renal pulmonary syndrome. It is defined as a pauci-immune necrotizing small vessel vasculitis, which usually affects the kidneys, followed by the lungs. It also presents systemic symptoms. The etiology of MPA is still unclear, but evidence reinforces the autoimmune mechanisms as the main etiopathogenic factor. Aortic valve stenosis (AS) is not an uncommon disease whose etiology varies according to geographical differences and the patient’s age. The natural history of AS begins with a prolonged asymptomatic period, but when symptomatic, respiratory failure is one of its main clinical presentations. The authors present the case of a 55-year-old woman who was admitted with the diagnosis of renal failure, anemia, and a cardiac murmur. The patient had been recently diagnosed with pneumonia. During hospitalization, diagnostic workup disclosed a normal kidney size as well as parenchymal thickness. A renal biopsy was undertaken but the specimen was exiguous, showing 4 sclerotic glomeruli and 1 glomerulus with crescentic glomerulonephritis. The search for ANCA was positive. The investigation of the cardiac murmur disclosed AS. The patient, on hemodialysis, presented episodes of respiratory failure, which was interpreted as acute pulmonary edema, but a suspicion of ANCA-related pulmonary renal syndrome was raised. However, the aortic valve replacement was prioritized. While awaiting cardiac surgery, the patient died because of respiratory insufficiency. Autopsy findings concluded that MPA with pulmonary hemorrhage due to vasculitis was the immediate cause of death. Although AS was present at autopsy and classified as moderate/severe, this lesion was a bystander in the process of this patient’s end of life, demonstrating the value of autopsy for medical learning and reasoning purposes

Chronic Chagas cardiomyopathy

First described by Carlos Justiniano Ribeiro Chagas(1879-1934) in 1909, Chagas disease (CD) or Americantrypanosomiasis is caused by the protozoan parasiteTrypanosoma cruzi. This zoonotic infectious diseasefollows a sylvatic cycle where blood-sucking triatomineinsect (known as kissing bug or in Portuguese as“barbeiro”), acquire the parasite by sucking smallmammals’ blood and transmit to humans throughtheir feces, left close to the bite site. The parasite,in turn, reaches the human blood stream when theparasite‑laden feces are dispersed at the itchingbite wound

Cardiac Myxoma

Primary cardiac tumors (PCT) are rare, accounting for 0.0017-0.03% in autopsy series,1,2 in contrast to metastatic tumors of the heart, which are 30 times more frequent.3,4 Nearly 75% of PCT are benign and most often represented by a myxoma in 50% of cases in the adult population.5 The World Health Organization defines a cardiac myxoma (CM) as a neoplasm composed of stellate to plump, cytologically bland, mesenchymal cells set in a myxoid stroma.6 Approximately 95% of CMs occurs isolated in a wide range of age. However, they occur more frequently among women in the fifth or sixth decade of life.7-9 For a time, myxomas were believed to arise from endocardial thrombi10 and some evidence suggests an association with Herpes simplex virus 1 infections11; however, their tumoral histogenesis remains unknown. Meanwhile, ultrastructure analysis—added to immunohistochemical investigation—suggests that CM is more likely derived from a pluripotent mesenchymal stem cell or sub-endothelial cell. Chromosomal clonal abnormalities, mostly on chromosome 2, 12, and 17, appear to be implicated in the myxoma formation, although defects on chromosome 1q32, the loss of the Y chromosome, and the telomeric association of chromosome 13 and 15, have also been involved.12-14 CMs are mostly pedunculated and solitary, and arise primarily adjacent to the lamina of the fossa ovalis (corresponding to the embryonic septum primum) and develop in the left atrium in 75% of cases, followed by the right atrium (18%),15 the right and left ventricles (3% in each), and the valves (1%).15-20 Multiple myxomas represent 5% of the cases, half of which are of bilateral origin.21 Although CM is a benign tumor, reports on its malignancy are well-known, which include: (i) local relapse; (ii) local invasiveness; and (iii) distant metastasis.12 The potential for malignant transformation is controversial, despite the publication of some reports of sarcomas arising from CM recurrences.22,23 Clinical manifestations of CMs are protean and may vary from asymptomatic cases (with a tumor < 4 cm) to unexpected sudden death (generally caused by blood flow obstruction or embolization). In most cases, the clinical presentation will depend on the tumor size, mobility, and location. One or more symptoms of the following triad will usually be present: (i) embolic phenomena (present in 30-40% of cases and usually associated with a villous surface of the tumour)24; (ii) intracardiac flow obstruction (present in almost 50% of cases); and (iii) constitutional symptoms (present in 20-60% of cases). In general, the most common signs and symptoms are non-specific and include dyspnea, palpitation (due to atrial fibrillation), lower limbs edema, hepatomegaly, angina, syncope, cough, and pulmonary edema. The constitutional symptoms, characterized by fatigue, fever, myalgia, arthralgia, and weight loss, are probably related to interleukin-6 cytokine production.25 The diagnosis of a CM is mostly done with echocardiography, both transthoracic and transesophageal, which represent the imaging modality of choice, although the latter permits precise information for the scheduling of surgery. Due to the rarity and consequent unfamiliarity of most general practitioners with this entity, CMs are sometimes misdiagnosed. Differential diagnosis should include intracardiac thrombus and other cardiac tumor

An inferior sinus venosus interatrial communication associated with a secundum atrial septal defect, clinically presenting in an adult patient: autopsy report

Atrial septal defects (ASD) are the most common congenital cardiac lesionin adults, representing up to 40% of acyanotic shunt lesions in patients olderthan 40 years. Secundum ASD comprises defects in the area of the ovalfossa, and may be associated with other types of interatrial communications.We present a case of a 25 year-old female patient who was asymptomaticuntil the age 22 when she started presenting exertion dyspnea and lowerlimbs edema, during her third pregnancy. The patient was admitted in theemergency department because of a febrile respiratory distress syndromedying in less than 36 hours after her arrival. The autopsy examination revealedpneumonia and a secundum ASD, associated with a rare form of interatrialcommunication, the inferior sinus venosus defect, which is characterized byan anomalous connection of the right pulmonary veins to the inferior caval veinat its opening in the right atrial cavity, while retaining partially their connectionto the left atrium. A dysplastic and thickened mitral valve was also present.Signs of pulmonary hypertension confirm the Eisenmenger syndrome in thisadult patient

Diretriz de arritmias cardíacas em crianças e cardiopatias congênitas SOBRAC e DCC – CP

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