Electrocardiogram evolution in Anderson-Fabry disease patients during follow-up in relation to specific treatment and
Vanda Parisi1, Raffaello Ditaranto1, Nevio Taglieri2
1Cardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy; Department of Medical and Surgical Sciences (DIMEC), University of Bologna, Bologna, Italy; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.
Insights
Electrocardiogram (ECG) changes in Anderson-Fabry disease (AFD) patients evolve over time, with specific treatment influencing P-end-Q interval changes. ECG findings like P-end-Q interval, RBBB, and QTc are linked to cardiac hypertrophy progression in AFD.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Electrocardiogram (ECG) analysis is crucial for diagnosing and managing Anderson-Fabry disease (AFD).
- Understanding ECG evolution in relation to treatment and disease progression is vital for patient care.
Purpose of the Study:
- To assess changes in ECG parameters during follow-up in AFD patients.
- To investigate the relationship between ECG evolution, specific AFD treatments, and disease progression.
Main Methods:
- Retrospective analysis of a multicentric cohort of 181 AFD patients with at least two ECG and echocardiographic assessments.
- Patients were categorized by treatment status: chronic therapy (≥1 year), therapy initiated during follow-up, or no specific therapy.
Main Results:
- Significant ECG variations observed during a median 62-month follow-up included atrial fibrillation, P-end-Q interval changes, left atrial enlargement, new right bundle branch block (RBBB), QRS interval changes, QRS fragmentation, QTc interval alterations, and development of symmetric lateral negative T waves.
- The P-end-Q interval showed significant interaction with treatment, increasing in untreated or chronically treated patients, and decreasing in those starting therapy during follow-up.
- P-end-Q interval, new RBBB, and pathological QTc were associated with increased left ventricle wall thickness.
Conclusions:
- Several ECG parameters significantly change during follow-up in AFD patients.
- The P-end-Q interval's evolution is significantly influenced by treatment status.
- P-end-Q interval, new RBBB, and pathological QTc are indicators of cardiac hypertrophy progression in AFD.
Aims:
Electrocardiogram (ECG) analysis plays a central role in Anderson-Fabry disease (AFD) diagnosis and management. This study aimed to assess ECG evolution during follow-up in relation to specific treatment and disease progression.
Methods:
Retrospective study of a multicentric cohort of AFD patients with ≥2 ECG and echocardiographic data. Specific treatment status (enzyme replacement or chaperone) was defined as: chronic therapy (≥ 1 year before the first ECG); therapy started during the follow-up; no therapy.
Results:
One-hundred-eighty-one AFD patients (median age 46 years, IQR 35-58; 36 % male; 67 % classic phenotype composed the study population: 24 % were on chronic therapy; 39 % started the therapy during follow-up; 37 % had no specific therapy. During a median follow-up of 62 months (IQR 33-83), significant ECG variations were: atrial fibrillation detection (p = 0.005), PendQ interval (p = 0.04), left atrial enlargement (p < 0.001), new right bundle branch block (RBBB, p < 0.001), QRS interval (p < 0.001), QRS fragmentation (p = 0.024), QTc (p = 0.001), and symmetric lateral negative T waves development (p = 0.016). PendQ interval showed a significant interaction with treatment status, significantly increasing in patients without or on chronic therapy but slightly reducing in patients who started the specific therapy during follow-up (p for interaction = 0.035). PendQ interval, new RBBB and pathologic QTc were associated with left ventricle wall thickness increase (p = 0.003, p = 0.014, p = 0.019, respectively).
Conclusions:
In this multicentric cohort of AFD patients, several ECG parameters showed significant changes during follow-up. Only PendQ interval showed a significant interaction with treatment status. Moreover, PendQ interval, new RBBB and pathologic QTc development were associated with cardiac hypertrophy progression.
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