A Population-Based Registry of Patients With Inherited Cardiac Conditions and Resuscitated Cardiac Arrest |
| |
| Affiliation: | 1. Greenlane Paediatric and Congenital Cardiac Services, Starship Children’s Hospital, Auckland, New Zealand;2. Instituto de Genética, Universidad Nacional de Colombia, Bogotá, Colombia;3. Cardiac Inherited Disease Group, Auckland, New Zealand;4. Department of Physiology, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand;5. Department of Cardiology, Waikato Hospital, Hamilton, New Zealand;6. Department of Cardiology, Wellington Hospital, Wellington, New Zealand;7. Genetic Health Service NZ, New Zealand;8. Greenlane Cardiovascular Services, Auckland City Hospital, Auckland, New Zealand;9. Department of Paediatrics Child and Youth Health, University of Auckland, Auckland, New Zealand |
| |
| Abstract: | BackgroundThe relative proportion of each cardiac inherited disease (CID) causing resuscitated sudden cardiac arrest (RSCA) on a population basis is unknown.ObjectivesThis study describes the profile of patients with CIDs presenting with RSCA; their data were collected by the national Cardiac Inherited Diseases Registry New Zealand (CIDRNZ).MethodsData were collated from CIDRNZ probands presenting with RSCA (2002 to 2018).ResultsCID was identified in 115 (51%) of 225 RSCA cases: long QT syndrome (LQTS) (n = 48 [42%]), hypertrophic cardiomyopathy (HCM) (n = 28 [24%]), Brugada syndrome (BrS) (n = 16 [14%]), catecholaminergic polymorphic ventricular tachycardia (CPVT) (n = 9 [8%]), arrhythmogenic right ventricular cardiomyopathy (ARVC) (n = 9 [8%]), and dilated cardiomyopathy (n = 5 [4%]). Seventy-one (62%) of 115 were male. Of 725 probands from the CIDRNZ with CID, the proportion presenting with RSCA was: CPVT, 9 (53%) of 17; BrS, 16 (33%) of 49; ARVC, 9 (25%) of 36; LQTS, 48 (20%) of 238; dilated cardiomyopathy, 5 (9%) of 58; and HCM, 28 (8%) of 354. Incident activity was: normal everyday activities, 44 (40%); exercising, 33 (30%); concurrent illness, 13 (12%); sleeping, 10 (9%); drugs/medication, 9 (8%); and emotion, 2 (2%). LQTS and CPVT predominated in those <24 years of age, 30 (77%) of 39; cardiomyopathies and BrS predominated in those >24 years of age, 49 (64%) of 76. For those >40 years of age, HCM was the most common (33%) CID. A genetic diagnosis in patients with CID was made in 48 (49%) of 98 tested. Diagnosis by age range was as follows: age 1 to 14 years, 78%; age 15 to 24 years, 53%; age 25 to 39 years, 54%; and age >40 years, 26%.ConclusionsThe commonest CID identified after RSCA was LQTS; the most common CID cause of RSCA for those >40 years of age was HCM. CPVT was the CID most likely to present with RSCA and HCM the least. Genetic yield decreases with age. Only one-third of RSCA cases due to CID occurred while exercising. |
| |
| Keywords: | cardiomyopathies channelopathies hypertrophic cardiomyopathy genetic testing long QT syndrome registry resuscitated sudden cardiac arrest ARVC" },{" #name" :" keyword" ," $" :{" id" :" kwrd0050" }," $$" :[{" #name" :" text" ," _" :" arrhythmogenic right ventricular cardiomyopathy BrS" },{" #name" :" keyword" ," $" :{" id" :" kwrd0060" }," $$" :[{" #name" :" text" ," _" :" Brugada syndrome CID" },{" #name" :" keyword" ," $" :{" id" :" kwrd0070" }," $$" :[{" #name" :" text" ," _" :" cardiac inherited disease CPR" },{" #name" :" keyword" ," $" :{" id" :" kwrd0080" }," $$" :[{" #name" :" text" ," _" :" cardiopulmonary resuscitation CPVT" },{" #name" :" keyword" ," $" :{" id" :" kwrd0090" }," $$" :[{" #name" :" text" ," _" :" catecholaminergic polymorphic ventricular tachycardia DCM" },{" #name" :" keyword" ," $" :{" id" :" kwrd0100" }," $$" :[{" #name" :" text" ," _" :" dilated cardiomyopathy HCM" },{" #name" :" keyword" ," $" :{" id" :" kwrd0110" }," $$" :[{" #name" :" text" ," _" :" hypertrophic cardiomyopathy LQTS" },{" #name" :" keyword" ," $" :{" id" :" kwrd0120" }," $$" :[{" #name" :" text" ," _" :" long QT syndrome RSCA" },{" #name" :" keyword" ," $" :{" id" :" kwrd0130" }," $$" :[{" #name" :" text" ," _" :" resuscitated sudden cardiac arrest SCD" },{" #name" :" keyword" ," $" :{" id" :" kwrd0140" }," $$" :[{" #name" :" text" ," _" :" sudden cardiac death |
| 本文献已被 ScienceDirect 等数据库收录！ |
|
