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PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease

Developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD)
      Nearly one third of all major congenital anomalies are heart defects, with an estimated 9 per 1000 live births afflicted by congenital heart disease (CHD) worldwide.
      • van der Linde D.
      • Konings E.E.
      • Slager M.A.
      • Witsenburg M.
      • Helbing W.A.
      • Takkenberg J.J.
      • Roos-Hesselink J.W.
      Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis.
      Remarkable advances in care have resulted in impressive gains in survival such that over 90% of children with CHD in developed countries today are expected to survive into adulthood.
      • Khairy P.
      • Ionescu-Ittu R.
      • Mackie A.S.
      • Abrahamowicz M.
      • Pilote L.
      • Marelli A.J.
      Changing mortality in congenital heart disease.
      Consequently, the past decades have witnessed historical shifts in population demographics, as adults now outnumber children with CHD. Population-based estimates indicate that there are currently over 1 million adults with CHD in the United States alone, over 100,000 in Canada, and 1.8 million in Europe.
      • Marelli A.J.
      • Mackie A.S.
      • Ionescu-Ittu R.
      • Rahme E.
      • Pilote L.
      Congenital heart disease in the general population: changing prevalence and age distribution.
      • Moons P.
      • Engelfriet P.
      • Kaemmerer H.
      • Meijboom F.J.
      • Oechslin E.
      • Mulder B.J.
      Delivery of care for adult patients with congenital heart disease in Europe: results from the Euro Heart Survey.
      • Go A.S.
      • Mozaffarian D.
      • Roger V.L.
      • Benjamin E.J.
      • Berry J.D.
      • Borden W.B.
      • Bravata D.M.
      • Dai S.
      • Ford E.S.
      • Fox C.S.
      • Franco S.
      • Fullerton H.J.
      • Gillespie C.
      • Hailpern S.M.
      • Heit J.A.
      • Howard V.J.
      • Huffman M.D.
      • Kissela B.M.
      • Kittner S.J.
      • Lackland D.T.
      • Lichtman J.H.
      • Lisabeth L.D.
      • Magid D.
      • Marcus G.M.
      • Marelli A.
      • Matchar D.B.
      • McGuire D.K.
      • Mohler E.R.
      • Moy C.S.
      • Mussolino M.E.
      • Nichol G.
      • Paynter N.P.
      • Schreiner P.J.
      • Sorlie P.D.
      • Stein J.
      • Turan T.N.
      • Virani S.S.
      • Wong N.D.
      • Woo D.
      • Turner M.B.
      Heart disease and stroke statistics—2013 update: a report from the American Heart Association.
      Rhythm disorders, which span the entire spectrum of brady- and tachyarrhythmias, are among the most prominent complications encountered by adults with CHD.
      • Walsh E.P.
      • Cecchin F.
      Arrhythmias in adult patients with congenital heart disease.
      Arrhythmias range in symptomatology and significance, from inconsequential and benign to poorly tolerated and potentially fatal. Taken together, arrhythmias are a leading cause of morbidity, impaired quality of life, and mortality in adults with CHD.

      Keywords

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