1DHZC- Deutsches Herzzentrum der Charite Berlin Klinik für Kardiologie, Angiologie und Intensivmedizin, Campus Mitte Berlin, Deutschland; 2Deutsches Herzzentrum der Charite (DHZC) Berlin, Deutschland; 3Charité – Universitätsmedizin Berlin Klinik für Neurologie und Experimentelle Neurologie Berlin, Deutschland
Introduction
Amyloidosis is a rare disease in which malformed proteins are deposited in the extracellular tissue of several organs. Involvement of the myocardium is associated with a poor prognosis.
ATTR cardiomyopathy (ATTR-CM) is an underestimated cause of heart failure with preserved EF (HFpEF). As diseases of the conduction system and ventricular tachycardia occur frequently in this patient group, early diagnosis is crucial.
Using the registry of our Charité Berlin Amyloidosis Centre (ACCB), we analyze the number and reasons for the implantation of pacemakers in our amyloidosis patients.
Methods
We screened 125 patients diagnosed with wild-type transthyretin (wtATTR) amyloidosis from our registry for a pacemaker (PM), implanted cardiac defibrillator (ICD) or cardiac resynchronisation therapy (CRT) device and extracted the indication for implantation, device type and functional mode from the amyloidosis registry.
Results
Of 125 patients with wtATTR amyloidosis, 113 were male (90.4%) with an average age of 80 ± 6.5 years. 36 patients (28.8%) were diagnosed with a PM or other cardiac device, 33 of these patients were male (91.7%). 32 patients (88.8%) of these patients were on specific medication with tafamidis 61 mg once daily. 17 patients had heart failure with preserved ejection fraction HFpEF (47.2 %), 13 patients (36.1 %) had heart failure with slightly reduced ejection fraction (HFmrEF) and 6 patients had heart failure with reduced ejection fraction (HFrEF) with a mean left ventricular ejection fraction (LVEF) of 48.6 % ± 9.2 (range 31-64 %).
4 patients (10 %) had a 1-chamber PM, 16 patients (70 %) had a 2-chamber PM, one patient (2.7 %) had an ICD. 3 patients (8.1%) had a CRT-P implant and 5 patients (13.5%) had a CRT-D system.
14 patients (37%) were diagnosed with sick sinus syndrome, 18 patients were diagnosed with atrioventricular block (AVB), of which 5 were categorised as AVB I, 5 as AVB II and 8 as AVB III.
6 (16%) patients suffered from atrial fibrillation, of which 4 manifested as bradyarrhythmia absoluta and 2 as bradyarrhythmia-tachyarrhythmia syndrome. Left ventricular branch block (LBB) was detected in 6 patients and two patients had relevant pauses.
Conclusion
One third of patients with ATTR-CM suffered from either a conduction system disorder or cardiac arrhythmia requiring the implantation of a cardiac device such as a pacemaker, although clear recommendations for this specific patient group are still lacking. The treatment of diseases of the conduction system and cardiac arrhythmias in patients with ATTR-CM therefore remains a challenge. We know that patients have an increased risk of sudden cardiac death and that arrhythmias such as atrial fibrillation are often poorly controlled. Further research is needed to improve therapy and outcome in these patients.