Classic form of hypoplastic left heart syndrome diagnosed post-natally: an autopsy report.

Hypoplastic left heart syndrome (HLHS) is a congenital heart disease, which, despite the current improved knowledge about its management and surgical treatment, is still associated with high mortality, especially in the early neonatal period and before the second stage of reconstruction surgery. The low rate of prenatal diagnosis and delayed diagnostic suspicion results in unsuccessful therapeutic intervention, even though the real impact of early diagnosis and intervention on mortality and quality of life of patients is still uncertain. Fortunately, this syndrome of challenging treatment is not that frequent. It involves a spectrum of obstructions to the blood flow within the left heart and is characterized by an inappropriate size of the left ventricle associated with a wide variety of valvular dysfunctions. Treatment ranges from heart transplantation to palliative surgical procedures. The authors describe a case of a newborn with HLHS, whose diagnosis was made after birth because of early respiratory failure. Despite the use of prostaglandin the newborn died. An autopsy was performed and the anatomical findings were described.

Manifestações clínicas e análise histopatológica pulmonar relacionadas a diferentes doenças em pacientes com tromboembolismo pulmonar fatal – um estudo em autópsias / Clinical manifestations and pulmonary histopathological analysis related to different diseases in patients with fatalpulmonary thromboembolism – an autopsy study

Introdução: Tromboembolismo pulmonar (TEP) é uma das mais graves complicações dentre pacientes hospitalizados e permanece subdiagnosticado. Ainda hoje, sua fisiopatologia não está completamente elucidada. Objetivos: Correlacionar comorbidades, neoplasias, cirurgias e achados histológicos às manifestações clínicas associadas ao TEP. Métodos: Entre 2001 a 2008, foram revisadas 291 autópsias de pacientes cuja causa de morte foi TEP. Os seguintes dados foram obtidos: idade, sexo, manifestações clínicas, achados histológicos e principais doenças de base/comorbidades, neoplasias e cirurgias da última internação. Os achados histológicos foram categorizados em: dano alveolar difuso (DAD), edema agudo de pulmão (EAP), hemorragia intra alveolar (HIA) e pneumonia intersticial linfo-plasmocítica (PILP). Odds ratios foram obtidas por regressão logística e foram consideradas significativas quando p < 0,05. Resultados: A mediana de idade foi 64 anos. Cerca de 64% dos pacientes apresentava doenças cardiovasculares. O achado pulmonar mais prevalente foi EAP. Apenas 13% dos casos apresentaram suspeita clínica. Insuficiência respiratória esteve associada a EAP, HIA e DAD; assim como instabilidade hemodinâmica a HIA e DAD. Conclusões: Foram encontradas importantes associações entre achados clínicos e histológicos em pacientes com TEP. A compreensão dos mecanismos fisiopatológicos envolvidos com cada doença associada a TEP pode auxiliar no diagnóstico e no tratamento da doença.

Introduction: Pulmonary thromboembolism (PTE) is one of the most fatal complications among hospitalized patients and remains undiagnosed. Its physiopathology and its epidemiology aren’t widely known in literature. Objectives: To correlate underlying diseases, different cancers and surgeries to histological findings and in-vivo manifestations associated to fatal PTE from autopsy reports. Methods: From 2,001 to 2,008, were reviewed 291 autopsies of patients whose cause of death was PTE. The following data were obtained: age, sex, clinical invivo manifestations, post-mortem pathological patterns and mainassociated underlying diseases, cancers and surgeries performed in last hospitalization. The pulmonary histopathological changes were categorized in: diffuse alveolar damage (DAD), pulmonary edema (PE), alveolar hemorrhage (AH) and lympho/plasmacytic interstitial pneumonia (LPIP). Odds ratios of positive relations were obtained by logistic regression and were considered significative when p < 0.05. Results: The median age was 64 years. 64% ofpatients presented cardiovascular illness associated to PTE. The most prevalent pulmonary finding was PE. Only 13% of cases had clinical suspect. Acute respiratory failure was positively related to PE, AH and DAD; as well hemodynamic instability to AH and DAD. Conclusions: We found important relations between clinical data and histological findings of fatal PTE patients. The understanding of pulmonary physiopathological mechanism involved with eachPTE-associated disease can improve diagnosis in order to offer prompt treatment and reduce mortality.

Manifestação rara de edema agudo de pulmão associado à miocardite lúpica aguda - relato de caso / Unusual clinical manifestation of acute pulmonary edema associated to acute lupic myocarditis - case report

O lupus eritematoso sistêmico (LES) é a mais comum das doenças auto-imunes sistêmicas. Embora os rins classicamente sejam os órgãos mais acometidos no LES, o coração também pode ser afetado de forma significativa. Entretanto, a ocorrência de edema agudo de pulmão associado à miocardite lúpica é rara e de tratamento imunossupressor específico ainda incerto. O presente relato de caso revisa a literatura quanto a manifestações lúpicas do sistema cardiopulmonar, seu diagnóstico e tratamento, e descreve uma paciente lúpica jovem que evoluiu com edema agudo de pulmão decorrente de uma miopericardite lúpica aguda. O rápido diagnóstico pôde permitir o emprego da terapêutica imunossupressora adequada com reversão completa da disfunção miocárdica. Em pacientes jovens com quadro sugestivo de edema agudo de pulmão, o diagnóstico de LES deve ser considerado. O uso de pulsoterapia com corticóide endovenoso mostrou-se eficaz e seguro para o tratamento da manifestação cardíaca extrema.

Systemic lupus erythematosus is the most common systemic autoimmune disease. Although kidneys are the mainorgans affected, heart may suffer injury too. However, acute pulmonary edema associated to lupic myocarditis is rare and its specific immunosuppressive treatment is still undefined. The present case report reviews literature about lupic manifestations in heart and lungs, their diagnosis and treatment, and describes an young lupic patient that had pulmonary edema due toacute lupic myopericarditis. Prompt diagnosis enabled correct immunosuppressive therapy that resulted in a complete reversion of myocardial disfunction. Lupus is a possible diagnosis in young patients with pulmonary edema. The use of intravenous pulse therapy with corticosteroids was safe and efficient to healing of this severe myocardial manifestation.