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. 2004 Apr;89(4):368–373. doi: 10.1136/adc.2003.029645

Cardiac complications of enterovirus rhombencephalitis

Y Fu 1, C Chi 1, Y Chiu 1, S Hsu 1, B Hwang 1, S Jan 1, P Chen 1, F Huang 1, Y Chang 1
PMCID: PMC1719879  PMID: 15033850

Abstract

Background: Epidemics of enterovirus 71 infection have caused the death of many children throughout the world. Rhombencephalitis, brain stem encephalitis, and heart failure were present in all of the fatal cases. However, no evidence of myocarditis was noted in the heart specimens, and the mechanism of heart failure remains unknown.

Aims: To characterise the presentation of cardiac complications in children with enterovirus rhombencephalitis and discuss its pathogenesis.

Methods: Ninety one consecutive patients with enterovirus rhombencephalitis underwent echocardiography. Of these, 17 patients (nine male, eight female; median age 14 months, range 4–57 months) with left ventricular dysfunction were studied.

Results: Tachycardia was noted in all patients and systemic hypertension in 12. Muscle-brain fraction of creatine kinase was >5% in 14 patients. Plasma norepinephrine and epinephrine levels were significantly raised in the three patients in whom these were analysed. Electrocardiographic abnormalities were noted in eight patients. Pulmonary oedema was complicated in 15 patients. The initial ejection fraction of the left ventricle was 22–58% (mean 37%, SD 11%). All patients deteriorated to hypotensive shock within 12 hours and 13 died. Heart specimens from seven patients showed no evidence of myocarditis, but significant coagulative myocytolysis, myofibrillar degeneration, and cardiomyocyte apoptosis were observed.

Conclusions: Acute heart failure was noted in 19% of patients with enterovirus rhombencephalitis, which had a fatality rate of 77%. It was not caused by myocarditis but possibly by neurogenic cardiac damage.

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Figure 1 .

Figure 1

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Anteroposterior projection of chest radiography in case 17 shows normal heart size and bilateral pulmonary oedema. At the same time, the ejection fraction of the left ventricle was 22% measured by echocardiography.

Figure 2 .

Figure 2

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Two dimensional echocardiography in 4-chamber view in case 11 shows generalised hypokinesia of the left ventricle with the ejection fraction of 39% (panel A in diastole and panel B in systole). Akinesia of apical septum was also noted (arrowheads). M-mode echocardiography shows poor wall motion of the left ventricle (panel C). Colour Doppler echocardiography shows grade 2+ mitral regurgitation (arrow), flowing into the left atrium (panel D). LA, left atrium; LV, left ventricle.

Figure 3 .

Figure 3

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Clinical course in case 12 shows that tachycardia, systemic hypertension, and high concentration of plasma catecholamines preceded the occurrence of heart failure. The arterial oxygen saturation was around 100% throughout the course. The patient eventually deteriorated to hypotensive shock.

Figure 4 .

Figure 4

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Histopathological examinations of the heart specimens from study patients (left panels) were compared to those from a control, a 4 year old boy who died of sepsis (right panels). Panel A in Masson's trichrome stain and panel C in haematoxylin and eosin stain show significant coagulative myocytolysis and myofibrillar degeneration and waving in the left ventricle, indicated by sarcoplasmic coagulation (C), granulation (G), vacuolisation (V), and myocytolysis (M). Some nuclei became condensed, pyknosis (P), and irregular in shape. Panel E shows significant cardiomyocyte apoptosis, indicated by green fluorescent nuclei and was more remarkable in the subendocardial region (arrow) (in situ TUNEL assay). The bar scales are 30 µm in length.

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