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Familial Risk of Wolff-Parkinson-White Syndrome a Nationwide Family Study in Sweden

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Názov: Familial Risk of Wolff-Parkinson-White Syndrome a Nationwide Family Study in Sweden
Autori: Ricci, Fabrizio, Pirouzifard, Mirnabi, Galanti, Kristian, Sundquist, Jan, Sundquist, Kristina, Sutton, Richard, Fedorowski, Artur, Zöller, Bengt
Prispievatelia: Lund University, Faculty of Medicine, Department of Clinical Sciences, Malmö, Cardiovascular Research - Hypertension, Lunds universitet, Medicinska fakulteten, Institutionen för kliniska vetenskaper, Malmö, Kardiovaskulär forskning - hypertoni, Originator, Lund University, Faculty of Medicine, Department of Clinical Sciences, Malmö, Family Medicine and Clinical Epidemiology, Lunds universitet, Medicinska fakulteten, Institutionen för kliniska vetenskaper, Malmö, Allmänmedicin och klinisk epidemiologi, Originator, Lund University, Profile areas and other strong research environments, Other Strong Research Environments, LUCC: Lund University Cancer Centre, Lunds universitet, Profilområden och andra starka forskningsmiljöer, Övriga starka forskningsmiljöer, LUCC: Lunds universitets cancercentrum, Originator, Lund University, Profile areas and other strong research environments, Strategic research areas (SRA), EpiHealth: Epidemiology for Health, Lunds universitet, Profilområden och andra starka forskningsmiljöer, Strategiska forskningsområden (SFO), EpiHealth: Epidemiology for Health, Originator, Lund University, Profile areas and other strong research environments, Strategic research areas (SRA), EXODIAB: Excellence of Diabetes Research in Sweden, Lunds universitet, Profilområden och andra starka forskningsmiljöer, Strategiska forskningsområden (SFO), EXODIAB: Excellence of Diabetes Research in Sweden, Originator, Lund University, Faculty of Medicine, Department of Clinical Sciences, Malmö, Family medicine, cardiovascular medicine and genetics, Lunds universitet, Medicinska fakulteten, Institutionen för kliniska vetenskaper, Malmö, Allmänmedicin, kardiovaskulär medicin och genetik, Originator
Zdroj: Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology.
Predmety: Medical and Health Sciences, Clinical Medicine, Cardiology and Cardiovascular Disease, Medicin och hälsovetenskap, Klinisk medicin, Kardiologi och kardiovaskulära sjukdomar
Popis: BACKGROUND: Wolff-Parkinson-White (WPW) syndrome is a rare cardiac disorder that predispose to supraventricular arrhythmias. Prognosis is usually benign, yet there is an increased lifetime risk of sudden death. While typically sporadic, familial clustering has been reported. This study aimed to assess the risk of WPW, arrhythmias, and mortality among siblings of individuals with WPW. METHODS: This population-based sibling cohort included 5,338,434 individuals born in Sweden (1932-2018), with 3,172 WPW cases identified from the Swedish National Patient Registers. Familial risks among siblings were assessed using incidence rate ratios (IRRs) and adjusted subdistributional hazard ratios (SHRs). Sensitivity analyses excluded syndromic WPW and cases without electrophysiologic procedural confirmation. RESULTS: Although familial occurrence of WPW was exceedingly rare with only 14 of 3,172 cases (0.4%; ≈0.0003% of the total population), siblings of affected individuals showed a significantly higher rate of WPW diagnosis (0.121 vs. 0.032 per 1,000 person-years; IRR 3.83; 95%CI 2.27-6.46; p<0.001) translating to an almost fourfold higher adjusted risk (SHR 3.79; 95%CI 1.81-7.97; p<0.001). Risks of atrial fibrillation (SHR 1.19; 95%CI 1.05-1.35; p<0.01) and ventricular arrhythmias (SHR 1.84; 95%CI 1.45-2.35; p<0.001) were also higher, whereas all-cause mortality was comparable irrespective of sibling history (HR 1.01; 95% CI 0.92-1.11; p=0.88). CONCLUSIONS: WPW features familial aggregation and increased arrhythmic risk among siblings of affected individuals despite its extremely low absolute frequency in the general population. The evidence of a measurable hereditary component within an otherwise sporadic, non-syndromic condition points to a genetic contribution driven by complex inheritance patterns.
Prístupová URL adresa: https://doi.org/10.1093/europace/euaf285
Databáza: SwePub
Popis
Abstrakt:BACKGROUND: Wolff-Parkinson-White (WPW) syndrome is a rare cardiac disorder that predispose to supraventricular arrhythmias. Prognosis is usually benign, yet there is an increased lifetime risk of sudden death. While typically sporadic, familial clustering has been reported. This study aimed to assess the risk of WPW, arrhythmias, and mortality among siblings of individuals with WPW. METHODS: This population-based sibling cohort included 5,338,434 individuals born in Sweden (1932-2018), with 3,172 WPW cases identified from the Swedish National Patient Registers. Familial risks among siblings were assessed using incidence rate ratios (IRRs) and adjusted subdistributional hazard ratios (SHRs). Sensitivity analyses excluded syndromic WPW and cases without electrophysiologic procedural confirmation. RESULTS: Although familial occurrence of WPW was exceedingly rare with only 14 of 3,172 cases (0.4%; ≈0.0003% of the total population), siblings of affected individuals showed a significantly higher rate of WPW diagnosis (0.121 vs. 0.032 per 1,000 person-years; IRR 3.83; 95%CI 2.27-6.46; p<0.001) translating to an almost fourfold higher adjusted risk (SHR 3.79; 95%CI 1.81-7.97; p<0.001). Risks of atrial fibrillation (SHR 1.19; 95%CI 1.05-1.35; p<0.01) and ventricular arrhythmias (SHR 1.84; 95%CI 1.45-2.35; p<0.001) were also higher, whereas all-cause mortality was comparable irrespective of sibling history (HR 1.01; 95% CI 0.92-1.11; p=0.88). CONCLUSIONS: WPW features familial aggregation and increased arrhythmic risk among siblings of affected individuals despite its extremely low absolute frequency in the general population. The evidence of a measurable hereditary component within an otherwise sporadic, non-syndromic condition points to a genetic contribution driven by complex inheritance patterns.
ISSN:15322092
DOI:10.1093/europace/euaf285