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Holt-Oram syndrome
A Skwarek-Dziekanowska1, A Wójtowicz-Ściślak, G Sobieszek
1Department of Cardiology, 1st Military Clinical Hospital with the Outpatient Clinic, Lublin, Poland. anetask@gmail.com.
Insights
Holt-Oram syndrome patients requiring pacemakers can benefit from His-bundle pacing. This technique offers physiological pacing, potentially preventing heart failure and maintaining cardiac function.
Area of Science:
- Cardiology
- Genetics
- Medical Devices
Background:
- Holt-Oram syndrome (HOS) is a rare genetic disorder.
- HOS involves upper limb abnormalities, congenital heart defects, and cardiac conduction issues.
- Patients with HOS often require pacemakers due to conduction disturbances.
Observation:
- A case report details a patient with HOS needing pacemaker implantation for bradycardia.
- His-bundle pacing was employed to prevent future heart failure.
- The implantation was successful, with the patient in good condition after one year.
Findings:
- The patient maintained over 90% pacing.
- Left ventricular ejection fraction (LVEF) remained stable at 60%.
- This demonstrates the efficacy of His-bundle pacing in HOS.
Implications:
- His-bundle pacing is a novel physiological pacing method.
- This technique may prevent heart failure in HOS patients.
- Further research is needed on optimal pacing strategies for HOS.
Background:
Holt-Oram syndrome (HOS) is a rare genetic illness, which concerns disturbances in the appearance of the upper limbs, congenital heart malformations, and cardiac conduction diseases. HOS usually requires the implantation of a pacemaker, because of cardiac conduction disturbances.
Case Report:
We present the case of a patient with HOS qualified for pacemaker implantation due to overt bradycardia. To prevent the development of heart failure in the future, the His-bundle pacing technique was used. The implantation was successful. In the control, after one year, the man remains in good condition. The pacing was over 90%, and the left ventricular ejection fraction (LVEF) was stable (60%).
Conclusions:
So far, there are no reports on which methods of stimulation are required when it comes to patients with HOS. His-bundle pacing technique is a new type of physiological pacing, which can avoid heart failure.
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