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Complete Heart Block in a Boy With Hyperostosis–Hyperphosphataemia Syndrome: A Case Report Publisher



Hosseini Z1 ; Zanjani KS2 ; Eslami M1 ; Mollazadeh R1
Authors
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Authors Affiliations
  1. 1. Cardiology Department, Imam Khomeini Hospital, Tehran University of Medical Sciences, Keshavarz Blv, Tehran, 1419733141, Iran
  2. 2. Pediatrics Department, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran

Source: European Heart Journal - Case Reports Published:2019


Abstract

Background Hyperostosis–hyperphosphataemia syndrome (HHS) is a rare metabolic disorder characterized by recurrent painful swelling of long bones and periosteal new bone formation. Case summary A 6-year-old boy was referred to our centre due to bradycardia. He was diagnosed with HHS 3 years’ prior, after investigation for the cause of joint pain and genu valgum. During medical follow-up in 2013–16, the paediatric cardiologist discovered thickened and calcified mitral and aortic valves and progression of cardiac conduction disturbance from 1st degree to 3rd degree atrioventricular block (AVB). The patient died in 2017 due to multiorgan failure caused by hyperphosphataemia and ectopic calcification. Discussion Our case is unique in that ectopic calcification occurred in the aortic, mitral valve and cardiac conduction system, and AVB progressed from 1st degree to 3rd degree over time despite treatment with high-dose phosphate binders. © The Author(s) 2019. Published by Oxford University Press on behalf of the European Society of Cardiology.