Holt Oram Syndrome: Complete AV Block Treated with LBBAP

Mujdat Aktas*

Department of Cardiology, Prof. Dr. Cemil Tascioglu City Hospital, Istanbul, Turkey

^*Correspondance to: Mujdat Aktas 

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Abstract:

Holt Oram Syndrome is a hereditary disease with autosomal dominant inheritance. The most common mutation occurs in the T-box complex TBX5 gene located on chromosome 12. It is characterized by disorders of the upper limbs and disorders of the heart. This syndrome has a broad clinical spectrum and can range from subclinical radiologic findings to life-threatening disease. Although the heart can be affected in many ways, atrial and ventricular septal defects are common. In some rare cases, the conduction system is affected. Our case had no history of chronic disease or drug usage. ECG at presentation showed sinus bradycardia. We report a patient who was diagnosed with Holt Oram syndrome in childhood and presented with a high degree of atrioventricular block and was treated with conduction system pacing.

Keywords:

Holt Oram syndrome

Cite the Article:

Aktas M. Holt Oram Syndrome: Complete AV Block Treated with LBBAP. Ann Clin Case Rep. 2025; 10: 2715..