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Updated: Jan 30, 2026

Evaluation of Left Ventricular Structure and Function using 3D Echocardiography
Published on: October 28, 2020
Transient left bundle branch block and left ventricular dysfunction in a patient with NLRP1-associated
Mark R Garrelfs1, Esther Hoppenreijs2, Ronald B Tanke1
11Department of Pediatric Cardiology,Radboud University Medical Centre,Nijmegen,The Netherlands.
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.
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