Acute petrified myocardium associated with meningococcal sepsis in childhood-onset systemic lupus erythematous: a fatal case

Acute petrified myocardium associated with septic shock, diagnosed by autopsy has rarely been described. A 15-year-old adolescent male was diagnosed with childhood-onset systemic lupus erythematosus. One year later, he was hospitalized with fever, myalgia, headache, arthritis, vomiting, dyspnea and was diagnosed with sepsis secondary to bronchopneumonia and meningitis. Blood culture identified Neisseria meningitidis serogroup Y. Despite antibiotics and intensive therapeutic measures, he died after 29 days of hospitalization. The autopsy revealed necrotic cardiomyocytes with dystrophic calcification and interstitial fibrosis.

Myocardial calcification; Sepsis; Neisseria meningitides; Lupus; Intensive care

Authorship

Ana Carolina Etrusco Zaroni Santos

Universidade de São Paulo, Hospital das Clínicas, Instituto da Criança, Unidade de Terapia Intensiva Pediátrica, São Paulo, São Paulo, BrazilUniversidade de São PauloBrazilSão Paulo, São Paulo, BrazilUniversidade de São Paulo, Hospital das Clínicas, Instituto da Criança, Unidade de Terapia Intensiva Pediátrica, São Paulo, São Paulo, Brazil

http://orcid.org/0000-0001-5185-168X

Michele Luglio

http://orcid.org/0000-0002-0584-905X

Artur Figueiredo Delgado

http://orcid.org/0000-0003-4362-9880

Constance Dell Santo Vieira Schuwartz

Universidade de São Paulo, Hospital das Clínicas, Instituto da Criança, Departamento de Infectologia Pediátrica, São Paulo, São Paulo, BrazilUniversidade de São PauloBrazilSão Paulo, São Paulo, BrazilUniversidade de São Paulo, Hospital das Clínicas, Instituto da Criança, Departamento de Infectologia Pediátrica, São Paulo, São Paulo, Brazil

http://orcid.org/0000-0002-9062-433X

Heloísa Helena Sousa Marques

http://orcid.org/0000-0002-1340-0463

Clarissa Carvalho de Miranda Valões

Universidade de São Paulo, Hospital das Clínicas, Instituto da Criança, Departamento de Reumatologia Pediátrica, São Paulo, São Paulo, BrazilUniversidade de São PauloBrazilSão Paulo, São Paulo, BrazilUniversidade de São Paulo, Hospital das Clínicas, Instituto da Criança, Departamento de Reumatologia Pediátrica, São Paulo, São Paulo, Brazil

http://orcid.org/0000-0002-6180-0736

Maria Andreina Cabrera Dominguez

http://orcid.org/0000-0002-3686-3778

Werther Brunow de Carvalho

http://orcid.org/0000-0002-9164-616X

Clovis Artur Silva

http://orcid.org/0000-0001-9250-6508

Amaro Nunes Duarte-Neto

Universidade de São Paulo, Hospital das Clínicas, Departamento de Patologia, São Paulo, São Paulo, BrazilUniversidade de São PauloBrazilSão Paulo, São Paulo, BrazilUniversidade de São Paulo, Hospital das Clínicas, Departamento de Patologia, São Paulo, São Paulo, Brazil

http://orcid.org/0000-0001-6659-7186

Correspondence to: Amaro Nunes Duarte-Neto Universidade de São Paulo, Hospital das Clínicas, Departamento de Patologia, Avenida Dr. Arnaldo, 455, Cerqueira Cesar, CEP 01246-903 São Paulo, SP, Brazil Tel: +55 11 98546-4225 E-mail: amaro.ndneto@hc.fm.usp.br

CONFLICT OF INTERESTS

The authors declare no conflict of interests.

SCIMAGO INSTITUTIONS RANKINGS

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Figure 1
Pathological findings in a heart with acute petrified myocardium: A) Gross exam of the heart (dorsal view) shows scattered yellowish subepicardial calcified plaques (arrow); B) scattered yellowish and calcified plaques (red arrows) in the myocardium on the left ventricle. An organized thrombus inside the cardiac left chamber (black arrow); C) postmortem, ex situ computerized tomography scan of the heart shows diffuse and coalescent hyperdense areas (calcification) in the entire myocardium on the ventricles, mainly in the left ventricle (arrow); D) necrotic cardiomyocytes with eosinophilic calcification and interstitial fibrosis (H&E); E) the calcification is within the cardiomyocyte cytoplasm and has lamellar (thick arrow) or granular (thin arrow) aspect (H&E); F) calcified cardiac fibers labeled in black with Von Kossa stain; G) interstitial collagen deposition (arrows) in the myocardium in the foci of necrotic and calcified cardiomyocytes (Masson trichrome stain); H) loss of desmin expression in necrotic and calcified cardiomyocytes (red arrows). Non-necrotic fibers maintain desmin expression and are labeled in brown (black arrow) (immunohistochemistry, anti-desmin, mouse monoclonal antibody, Cell Marque™); I) negative expression of Bcl2 in necrotic cardiomyocytes (immunohistochemistry, anti-Bcl2, mouse monoclonal antibody, Cell Marque™); J-M) electron microscopy analysis of thin sections of the myocardium shows diffuse and intense myocytolysis and mitochondrial crystolisis and edema (in J), calcification confined to cardiomyocyte cytoplasm (arrows in K), spicules of calcium with cottony aspect at the periphery of dense and lamellar calcification (in L), calcification in the entire mitochondrial matrix (arrows in M). Scale bars: L, M=0.5 µm; J=1 µm; K=5 µm; E=50 µm; I=100 µm; F, H=200 µm; D, G=500 µm.

Figure 1 Pathological findings in a heart with acute petrified myocardium: A) Gross exam of the heart (dorsal view) shows scattered yellowish subepicardial calcified plaques (arrow); B) scattered yellowish and calcified plaques (red arrows) in the myocardium on the left ventricle. An organized thrombus inside the cardiac left chamber (black arrow); C) postmortem, ex situ computerized tomography scan of the heart shows diffuse and coalescent hyperdense areas (calcification) in the entire myocardium on the ventricles, mainly in the left ventricle (arrow); D) necrotic cardiomyocytes with eosinophilic calcification and interstitial fibrosis (H&E); E) the calcification is within the cardiomyocyte cytoplasm and has lamellar (thick arrow) or granular (thin arrow) aspect (H&E); F) calcified cardiac fibers labeled in black with Von Kossa stain; G) interstitial collagen deposition (arrows) in the myocardium in the foci of necrotic and calcified cardiomyocytes (Masson trichrome stain); H) loss of desmin expression in necrotic and calcified cardiomyocytes (red arrows). Non-necrotic fibers maintain desmin expression and are labeled in brown (black arrow) (immunohistochemistry, anti-desmin, mouse monoclonal antibody, Cell Marque™); I) negative expression of Bcl2 in necrotic cardiomyocytes (immunohistochemistry, anti-Bcl2, mouse monoclonal antibody, Cell Marque™); J-M) electron microscopy analysis of thin sections of the myocardium shows diffuse and intense myocytolysis and mitochondrial crystolisis and edema (in J), calcification confined to cardiomyocyte cytoplasm (arrows in K), spicules of calcium with cottony aspect at the periphery of dense and lamellar calcification (in L), calcification in the entire mitochondrial matrix (arrows in M). Scale bars: L, M=0.5 µm; J=1 µm; K=5 µm; E=50 µm; I=100 µm; F, H=200 µm; D, G=500 µm.

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Acute petrified myocardium associated with meningococcal sepsis in childhood-onset systemic lupus erythematous: a fatal case

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[1] Correspondence to: Amaro Nunes Duarte-Neto Universidade de São Paulo, Hospital das Clínicas, Departamento de Patologia, Avenida Dr. Arnaldo, 455, Cerqueira Cesar, CEP 01246-903 São Paulo, SP, Brazil Tel: +55 11 98546-4225 E-mail: amaro.ndneto@hc.fm.usp.br