Key result
Percutaneous intramyocardial septal radiofrequency ablation successfully reduced ventricular septal thickness from 22 mm to 16 mm and resting LVOT gradient from 41 mmHg to 3 mmHg at 7 months.
Why the study?
Does percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) reduce septal hypertrophy and LVOT obstruction in a patient with Fabry disease?
Case Report (n=1)
No
Does percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) reduce septal hypertrophy and LVOT obstruction in a patient with Fabry disease?
The innovative percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) procedure was safe and effective at dramatically reducing left ventricular obstruction and heart failure symptoms in a patient with Fabry disease.
Answers are grounded in this paper and cite their sources.
PIMSRA may relieve LVOT obstruction in Fabry disease; hypothesis-generating and requires prospective trials before wider use.
Background: This is a case report of a patient with Fabry disease (FD). We successfully treated a patient with ventricular septal hypertrophy and left ventricular outflow tract (LVOT) obstruction caused by FD. We report our exclusive new surgery for patients with LVOT obstruction, percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) procedure™ (percutaneous intramyocardial septal radiofrequency ablation). The patient recovered well 7 months after operation, and no LVOT obstruction and arrhythmia occurred. Case summary: A 51-year-old woman with chest tightness after satiety for ∼10 years, aggravated for 2 months. The patient had a history of multiple syncope, syncope after activity or emotional excitement. Echocardiogram demonstrated ventricular septal thickness of 22 mm by the report. Genetic testing revealed gene mutation, which was consistent with FD (GLA: c.643A > C). Biochemical evaluation reported reduced serum α-galactosidase A activity and abnormal serum Lyso-GL-3 enzyme activity. The patient and her families decided to perform PIMSRA operation. After the operation, the septal thickness steadily decreased to 16 mm over the course of 7 months. However, the patient's symptoms improved significantly. Discussion: This is the first case of cardiac involvement caused by FD mimicking hypertrophic cardiomyopathy treated with the innovative PIMSRA procedure. Percutaneous intramyocardial septal radiofrequency ablation procedure can be one of the effective treatments to alleviate cardiac hypertrophy and left ventricular obstruction in FD.
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Shi et al. (2024) conducted a case report in Fabry disease with ventricular septal hypertrophy and left ventricular outflow tract obstruction (n=1). Percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) was evaluated on Reduction in ventricular septal thickness and left ventricular outflow tract (LVOT) gradient. Percutaneous intramyocardial septal radiofrequency ablation successfully reduced ventricular septal thickness from 22 mm to 16 mm and resting LVOT gradient from 41 mmHg to 3 mmHg at 7 months.
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