Dataset Information

Complete heart block in a boy with hyperostosis-hyperphosphataemia syndrome: a case report.

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.

SUBMITTER: Hosseini Z

PROVIDER: S-EPMC6439360 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature

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Publications

Complete heart block in a boy with hyperostosis-hyperphosphataemia syndrome: a case report.

Hosseini Zahra Z Zanjani Keyhan Sayadpour KS Eslami Masoud M Mollazadeh Reza R

European heart journal. Case reports 20190205 1

<h4>Background</h4>Hyperostosis-hyperphosphataemia syndrome (HHS) is a rare metabolic disorder characterized by recurrent painful swelling of long bones and periosteal new bone formation.<h4>Case summary</h4>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 val ...[more]

PMID: 31020248

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Project description:Background Hypothyroidism can cause a variety of manifestations, including cardiovascular disorders. The most frequent clinical signs are sinus bradycardia and pericardial effusion. The affected patient generally has significant symptoms. Hypothyroidism infrequently results in a complete atrioventricular block. Case presentation A 19-year-old girl presented to our cardiology clinic with generalized tiredness, edema in her lower limbs and face, constipation, and a menstruation abnormality in the previous six months. With a normal ejection fraction on echocardiography, an electrocardiogram revealed complete atrioventricular block. When she was admitted, her laboratory testing showed that she had severely raised Thyroid Stimulating Hormone (TSH) levels, severe dyslipidemia with normal electrolytes, and normal liver and kidney function tests. The patient was treated with 50mg Thyroxine to her. She had significant improvement within two weeks of treatment. Up on the next follow-up (at one-month), her electrocardiogram returned to normal sinus rhythm without any evidence of atrioventricular block and that the lipid profile had returned to normal. Clinical discussion In its first stages, hypothyroidism can not show any obvious symptoms. Untreated hypothyroidism over time can lead to a variety of health issues, including obesity, joint discomfort, infertility, and heart disease. This current case demonstrates how levothyroxine medication successfully managed a young female patient's severe hypothyroidism, difficult total heart block, severe hyperlipidemia, and long-standing menstrual irregularity. Conclusion We found that overt hypothyroidism caused a complete atrioventricular block and severe dyslipidemia, and that thyroxin therapy completely corrected both conditions. Highlights • We have seen a literature review the importance of finding out possible underlying cause of.complete heart block before intervention.• Thyroid dysfunction can cause severe complication like complete heart block,dyslipidaemia and menstrual dysregulation.• According to reports, levothyroxine alone can correct both av block and dyslipidaemia in the setting of hypothyroidism.• Here we present a case of severe hypothyroidism complicating complete heart block and hyperlipidemia which was successfully treated with Levothyroxine.

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Dataset Information

Complete heart block in a boy with hyperostosis-hyperphosphataemia syndrome: a case report.

Background

Case summary

Discussion

Publications

Complete heart block in a boy with hyperostosis-hyperphosphataemia syndrome: a case report.

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