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15 Clinical profile of transthyretin amyloid cardiomyopathy patients in Ireland

Heart (British Cardiac Society), 2021, Vol.107 (Suppl 2), p.A16-A17 [Peer Reviewed Journal]

2021 Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ. ;ISSN: 1355-6037 ;EISSN: 1468-201X ;DOI: 10.1136/heartjnl-2021-ICS.15

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  • Title:
    15 Clinical profile of transthyretin amyloid cardiomyopathy patients in Ireland
  • Author: Morris, J ; Starr, N ; Hewitt, K ; Murphy, L ; Mahon, N ; Coyne, M ; Joyce, E
  • Subjects: Cardiomyopathy ; Ejection fraction ; Mutation ; Pacemakers ; Peripheral neuropathy ; Spinal stenosis
  • Is Part Of: Heart (British Cardiac Society), 2021, Vol.107 (Suppl 2), p.A16-A17
  • Description: Background Transthyretin amyloid cardiomyopathy (ATTR-CM) is an under-recognised aetiology for heart failure with preserved ejection fraction. Early recognition and diagnosis of ATTR-CM should translate into better outcomes for these patients as targeted medications are licensed for use in ATTR-CM. European amyloid cardiomyopathy registries are establishing the clinical characteristics of this disease while creating diagnostic and prognostic tools with this data. Aim: To compare the clinical profile of patients with hereditary ATTR-CM (hATTR-CM) and wild-type ATTR-CM (wtATTR-CM) in Ireland. Methods A retrospective review was performed on the electronic clinical documents of all ATTR-CM patients referred to our centre. The review period included dates from 1st January 2019 to 1st February 2021. The two groups were compared in terms of clinical characteristics (mean age, female prevalence, New York Heart Association (NYHA), left ventricular ejection fraction (LVEF)), and the prevalence of co-morbidities such as arrhythmias, permanent pacemaker insertion, carpal tunnel syndrome, spinal stenosis, and peripheral neuropathy. Statistical analysis was made using software package SPSS. Results Twenty two patients in total were enrolled. 55% (n=12) had hATTR-CM compared to 45% (n=10) with wtATTR-CM. In the hATTR-CM group, 91.7% (n=11) of the patients had the T60A gene mutation and 8.3% (n=1) had the S50R gene mutation. There was a significant difference in the mean age between the two cohorts; hATTR-CM cohort mean age was 64.6±7.3 years compared to 76±8.4 years in the wtATTR-CM cohort (p-value = 0.003). (Table 1) There was a significant difference of the prevalence of atrial arrhythmia between the two groups; 58% (n=7) patients in the hATTR-CM group compared to all patients in the wtATTR-CM group (p-value = 0.02). In the hATTR-CM, 25% (n=3) were classified as NHYA 1, 50% (n=6) were NYHA 2, and 25% (n=3) were NYHA 3. In the wtATTR-CM group, 30% (n=3) were NHYA 1, 30% (n=3) were NYHA 2, and 40% (n=4) were NYHA 3. Pacemaker insertion rates were similar between groups 25% hATTR-CM and 20% wtATTR-CM respectively. The mean LVEF was 51±13.6% and 45.4±10.8% in the hATTR-CM and wtATTR-CM (p=0.3), respectively. The prevalence of carpel tunnel syndrome (33.3% vs 85.7%; p =0.03) and spinal stenosis (16.7% vs 60%; p=0.04) was significantly lower in the hATTR-CM group compared to the wtATTR-CM group. Peripheral neuropathy was significantly more prevalent in the hATTR-CM group compared to the wtATTR-CM group (91.7% vs 30%, p=0.003).Abstract 15 Table 1 Comparison of clinical characteristic Conclusion In this study, both sub-categories of ATTR-CM had similar prevalence. The T60A mutation is regarded as the most common hATTR gene mutation in Ireland which was reflected in this study. This study has limitations primarily due to the small size but demonstrates the emerging clinical profile of patients with ATTR-CM in Ireland. These findings will have implications on the development of assessment and management pathways for this under-recognised patient cohort.
  • Publisher: London: BMJ Publishing Group LTD
  • Language: English
  • Identifier: ISSN: 1355-6037
    EISSN: 1468-201X
    DOI: 10.1136/heartjnl-2021-ICS.15
  • Source: ProQuest Central
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