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Transient left bundle branch block and left ventricular dysfunction in a patient with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome

Published online by Cambridge University Press:  25 January 2019

Mark R. Garrelfs ,
Esther Hoppenreijs  and
Ronald B. Tanke
Mark R. Garrelfs*
Affiliation:
Department of Pediatric Cardiology, Radboud University Medical Centre, Nijmegen, The Netherlands
Esther Hoppenreijs
Affiliation:
Department of Pediatric Rheumatology, Radboud University Medical Centre, Nijmegen, The Netherlands
Ronald B. Tanke
Affiliation:
Department of Pediatric Cardiology, Radboud University Medical Centre, Nijmegen, The Netherlands
*
Author for correspondence: Garrelfs MR, Department of Pediatric Cardiology, Radboud University Medical Centre, Nijmegen, The Netherlands. E-mail: Mark.garrelfs@radboudumc.nl
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Abstract

The NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome is a rare novel autoinflammatory disorder. Cardiac involvement has not been previously reported. We present a 12-year-old girl with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome who was diagnosed with severely impaired left ventricular function and complete left bundle branch block during an exacerbation of the disease. Cardiac dysfunction proved to be rapidly reversible after initiation of high-dose methylprednisolone.

Keywords

NLRP1-associated autoinflammation with arthritis and dyskeratosisautoinflammationautoimmunecardiac involvementcardiac dysfunctionleft bundle branch block
Type
Brief Report
Information
Cardiology in the Young , Volume 29 , Issue 3 , March 2019 , pp. 435 - 438
Copyright
© Cambridge University Press 2019 

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Footnotes

Cite this article: Garrelfs MR, Hoppenreijs E, Tanke RB. (2019) Transient left bundle branch block and left ventricular dysfunction in a patient with NLRP1-associated autoinflammation with arthritis and dyskeratosis syndrome. Cardiology in the Young29: 435–438. doi: 10.1017/S1047951118002342

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