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Case Reports
. 2019 Aug 31;13(1):286.
doi: 10.1186/s13256-019-2190-9.

Berlin Heart EXCOR® pediatric ventricular assist device in a patient with Sotos syndrome: a case report

Rita Gravino  1 , Giuseppe Limongelli  2 , Andrea Petraio  3 , Daniele Masarone  4 , Maria Giovanna Russo  2 , Ciro Maiello  3 , Marina Verrengia  4 , Danilo De Paulis  5 , Giuseppe Pacileo  4
Affiliations
Case Reports

Berlin Heart EXCOR® pediatric ventricular assist device in a patient with Sotos syndrome: a case report

Rita Gravino et al. J Med Case Rep. .

Abstract

Introduction: Berlin Heart EXCOR® pediatric ventricular assist device is a mechanical circulatory support device currently used in pediatric patients. Sotos syndrome is a well-described multiple anomaly syndrome characterized by overgrowth, distinctive craniofacial appearance, cardiac abnormalities, and variable learning disabilities.

Case presentation: We describe a 7-year-old female Caucasian child with classic Sotos syndrome features subjected to implantation of Berlin Heart EXCOR® pediatric biventricular assist device mechanical support. A heart transplant was carried out after a support time of 459 days. After 5 years of follow-up, our patient is clinically stable and the performance of the transplanted heart is excellent.

Conclusion: This case confirms that Berlin Heart EXCOR® pediatric ventricular assist device can provide satisfactory and safe circulatory support for children with end-stage heart diseases, even in those with Sotos syndrome. The syndrome is not a contraindication to implantation, since the complications are the same as those observed in patients without the syndrome and the prognosis is not affected by the disease.

Keywords: Berlin Heart EXCOR® pediatric ventricular assist device; NSD1 gene; Sotos syndrome.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Pre-implantation echocardiographic images. Dilatation of the left ventricle and right ventricle, results of mitral valvuloplasty (arrow). LV left ventricle, RV right ventricle
Fig. 2
Fig. 2
Post-implantation echocardiographic image. Cannula in the left ventricular apex (arrow)
See this image and copyright information in PMC

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