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Pacing temprano en un niño con síndrome de Lodder-Merla y disfunción progresiva del nodo sinusal: reporte de un caso
Ahmed J AlAraibi1, Fatema Naser Shakeeb1, Aditya Bhat2
1School of Medicine, The Royal College of Surgeons in Ireland, Medical University of Bahrain, Busaiteen P.O. Box 15503, Bahrain.
European heart journal. Case reports
|February 27, 2026
Resumen
El síndrome de Lodder-Merla, un trastorno genético raro, puede causar disfunción progresiva del nodo sinusal (DNS). El reconocimiento temprano y la implantación de un marcapasos estabilizaron a un niño pequeño.
Área de la Ciencia:
- Genética
- Cardiología
- Neurología
Sus antecedentes:
- El síndrome de Lodder-Merla es un trastorno autosómico recesivo raro.
- Pueden ocurrir manifestaciones cardíacas, como la disfunción progresiva del nodo sinusal (DNS).
Objetivo del estudio:
- Destacar la dimensión cardiovascular de los trastornos relacionados con GNB5.
- Enfatizar la naturaleza progresiva y potencialmente mortal de la DNS en este síndrome.
Principales métodos:
- Reporte de caso de un niño de 1 año y 10 meses con síndrome de Lodder-Merla.
- Las pruebas genéticas confirmaron la mutación GNB5.
- El ECG y el monitoreo Holter rastrearon el ritmo cardíaco.
- Implantación de marcapasos VVI epicárdico.
Principales resultados:
- El paciente presentó convulsiones, retraso global del desarrollo e hipotonía.
- El monitoreo Holter inicial mostró pausas sinusales intermitentes, con una marcada progresión en pruebas posteriores.
- La implantación del marcapasos estabilizó el ritmo cardíaco y suprimió las pausas prolongadas.
Conclusiones:
- La afectación cardíaca, específicamente la DNS, puede ser una característica crítica de los trastornos relacionados con GNB5.
- La DNS puede ser progresiva y requerir reconocimiento e intervención tempranos.
- La vigilancia proactiva del ritmo es crucial en niños pequeños con comorbilidades, incluso sin síntomas clásicos de bradiarritmia.
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