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Emergency surgical treatment of advanced endomyocardial fibrosis in Mozambique

Abstract

Background An 11-year-old girl presented to a specialist cardiac facility in Mozambique. She had severe heart failure and massive cardiac enlargement, herniation of the heart into the epigastrium, atrial fibrillation, signs of severe pulmonary hypertension and a low cardiac output.

Investigations Chest radiography, echocardiography, 24 h Holter monitoring, and cardiac catheterization.

Diagnosis Left and right endomyocardial fibrosis in conjunction with an aneurysmal left atrium, severe heart failure, and atrial fibrillation.

Management Left ventricular endocardiectomy with mobilization of the chordae tendineae, mitral valve repair, tricuspid annuloplasty, and left atrial resection.

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Figure 1: Chest radiographs demonstrating the extent of the patient's cardiomegaly.
Figure 2: Transthoracic echocardiograms in the four-chamber view, demonstrating a marked reduction in the size of the patient's left atrium, and normalization of the left ventricular shape and size.
Figure 3: Perioperative photographs of the patient's atrioventricular valves.
Figure 4: Photograph of the resected left atrial wall with attached atrial appendage.
Figure 5: Photomicrograph of tissue from the patient's left ventricle showing endocardial thickening due to the deposition of collagen in the subendocardium (hematoxylin and eosin staining).
Figure 6: Photomicrographs of tissue from the patient's left atrium showing irregular thickening of the endocardium with a downward spur into the myocardium (hematoxylin and eosin staining).
Figure 7: Photomicrographs of inflammatory infiltrates in tissue from the patient's left atrial wall showing an abundance of lymphocytes and the presence of a few eosinophils (arrows in B; hematoxylin and eosin staining).

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Acknowledgements

We would like to acknowledge the Magdi Yacoub Institute and the Chain of Hope for their sponsorship, and Professor M Beatriz Ferreira for her important role and continuous support in the implementation of this surgical collaborative project between the Institute and the Chain of Hope Charity.

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Correspondence to Magdi H Yacoub.

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The authors declare no competing financial interests.

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Mocumbi, A., Carrilho, C., Burke, M. et al. Emergency surgical treatment of advanced endomyocardial fibrosis in Mozambique. Nat Rev Cardiol 6, 210–214 (2009). https://doi.org/10.1038/ncpcardio1449

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