Table 7.
Summary of the main recommendations for the supportive treatment of cardiac manifestations in FD.
| Recommendations for the Supportive Treatment of Cardiac Manifestations in FD |
| Angiotensin Converting Enzyme Inhibitors or Angiotensin II Receptor Blockers 1,2 |
| Angiotensin converting enzyme inhibitors (or angiotensin II receptor blockers, if not tolerated) should be used in patients with LV systolic dysfunction and heart failure [25,51,145]. |
| Beta-blockers 3 |
| Beta-blockers should be considered in patients with heart failure and LV systolic dysfunction; or in patients with angina [25,51,145,207,208]. Beta-blockers are recommended to relieve LVOT obstruction symptoms and to control the rate of atrial fibrillation/flutter [25,51,145]. |
| Mineralocorticoid receptor antagonists 1 |
| Mineralocorticoid receptor antagonists should be considered in patients with heart failure and LV systolic dysfunction [51,145]. |
| Loop diuretics |
| Loop diuretics should be considered to treat symptoms of congestion in patients with heart failure [134,207]. |
| Calcium channel blockers |
| Dihydropyridines2 should be considered for the treatment of angina [134,208]. Verapamil3 is recommended in patients with LVOT obstruction symptoms and should be considered in patients with angina [51,145,208]. Diltiazem3 should be considered in patients with LVOT obstruction symptoms or angina [51,145,208]. |
| Ivabradine 3 |
| Ivabradine should be considered for the treatment of heart failure or angina, according to ESC guidelines [145,207,208]. |
| Antiplatelet therapy |
| Antiplatelet therapy should be started in patients who suffered a stroke or myocardial infarction [134]. |
| Anticoagulation |
| Anticoagulation should be immediately started once atrial fibrillation or flutter is detected [145]. Direct oral anticoagulants (DOACs) should be considered as the first-line choice in patients without contra-indications [145]. |
| Anti-arrhythmic drugs |
| Amiodarone should be avoided in FD [213,214]. Dronedarone is contra-indicated in patients with heart failure (NYHA class III–IV) and renal failure (eGFR < 30mL/min) [145]. Sotalol, flecainide and propafenone are contra-indicated in patients with heart failure [145]. |
| Management of cardiovascular risk factors |
| Control of cardiovascular risk factors, including arterial hypertension, diabetes and dyslipidaemia, is indicated [134]. |
| Pacemaker |
| Pacemaker may be required to treat symptomatic bradycardia or symptomatic/advanced cardiac blocks, according to ESC guidelines [134,145]. Dual chamber pacemakers should be implanted unless patients are in permanent atrial fibrillation [145]. |
| ICD |
| ICD implantation is recommended in patients who suffered sudden cardiac arrest due to VT/fibrillation or sustained VT causing syncope/haemodynamic compromise and have a life expectancy >1 year [209]. ICD implantation should be considered in patients with advanced hypertrophy and fibrosis, who require pacemaker implantation and have a life expectancy >1 year [145]. ICD implantation may be considered in patients with severe LVH and advanced fibrosis or non-sustained VT, who have a life expectancy >1 year [145]. ICD implantation is recommended in patients with heart failure (NYHA class II-III) and LV ejection fraction ≤35%, despite ≥3 months of optimal treatment, who have a life expectancy >1 year [207]. |
| CRT |
| CRT should be considered in patients with LV ejection fraction ≤35%, according to ESC guidelines [207]. CRT-P should be considered in symptomatic patients with a pacing indication, LV ejection fraction <50% and QRS duration >120ms [145]. |
| Septal reduction therapy (myectomy/alcohol ablation therapy) |
| Septal reduction therapy is recommended in patients with a resting or provoked LVOT gradient ≥50 mm Hg, who are in NYHA class III–IV, despite maximum tolerated medical therapy [51,210,211]. Septal reduction therapy should be considered in patients with a resting or provoked LVOT gradient ≥50 mm Hg, who suffer recurrent exertional syncope, despite maximum tolerated medical therapy [51,210,211]. |
| Heart transplantation |
| Heart transplantation should be considered in patients with advanced heart failure with severe LV dysfunction and NYHA class III–IV despite optimal medical therapy, or intractable ventricular arrhythmia, depending on the extension of the extracardiac involvement by the disease [134,217]. |
CRT, Cardiac resynchronization therapy; eGFR, estimated glomerular filtration rate; ESC, European Society of Cardiology; FD, Fabry disease; ICD, Implantable cardioverter-defibrillator; LV, left ventricular; LVH, left ventricular hypertrophy; LVOT, left ventricular outflow tract; NYHA, New York Heart Association; VT, ventricular tachycardia 1 Caution should be taken in Fabry patients with nephropathy due to the risk of hyperkalaemia or worsening of renal function; 2 Should be avoided, if possible, in patients with resting/latent LVOT obstruction; 3 Caution should be taken due to the increased risk of bradycardia in Fabry patients.