Pearson marrow-pancreas syndrome with cardiac conduction abnormality necessitating prophylactic pacemaker implantation.
Ann Noninvasive Electrocardiol
; 25(1): e12681, 2020 01.
Article
en En
| MEDLINE
| ID: mdl-31475425
ABSTRACT
Pearson marrow-pancreas syndrome (PS), an exceedingly rare mitochondrial disorder, involves multiple systems including hematologic system and pancreas. Other mitochondrial disorders have been associated with progressive infrahisian block but this has not yet been described as a major feature of PS. We report a 7-year-old girl with classical features of PS and cardiac conduction defect. Her electrocardiogram revealed QRS prolongation with right bundle and left anterior fascicular blocks. Follow-up Holter revealed bifascicular block, alternating left and right bundle branch blocks, supraventricular tachycardia (with alternating bundles), and suspicion for nonsustained ventricular tachycardia. She underwent successful transvenous single-chamber ventricular pacemaker.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Marcapaso Artificial
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Enfermedades Mitocondriales
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Electrocardiografía
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Trastorno del Sistema de Conducción Cardíaco
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Síndromes Congénitos de Insuficiencia de la Médula Ósea
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Sistema de Conducción Cardíaco
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Errores Innatos del Metabolismo Lipídico
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Enfermedades Musculares
Límite:
Child
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Female
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Humans
Idioma:
En
Revista:
Ann Noninvasive Electrocardiol
Asunto de la revista:
CARDIOLOGIA
Año:
2020
Tipo del documento:
Article