Hypertrophic cardiomyopathy in Noonan syndrome

Acta Paediatrica Japonica; Overseas Edition
T NishikawaK Momma

Abstract

Noonan syndrome, a well-known multiple congenital anomalies syndrome, is frequently accompanied by cardiovascular diseases including hypertrophic cardiomyopathy (HCM). The incidence of HCM in Noonan syndrome is approximately 20-30% and one-third of cases reveal ventricular outflow obstruction. HCM in Noonan syndrome is occasionally associated with a congenital heart defect, whereas classic HCM seldom accompanies cardiac malformations. Asymmetric septal hypertrophy and symmetric septal hypertrophy (concentric hypertrophy) can be observed both in HCM with Noonan syndrome and in classic HCM, but apical hypertrophy has not been reported in Noonan syndrome yet, although it appears in classic HCM. Congestive heart failure is the major cause of death in patients with HCM in Noonan syndrome, but cases of sudden death have also been reported. The histopathologic findings of ventricular myocardial tissue in HCM with Noonan syndrome are similar to those in classic HCM.

References

Dec 1, 1992·British Heart Journal·M BurchW J McKenna
Jan 1, 1973·British Heart Journal·C PhornphutkulA S Nadas
Jan 1, 1974·American Journal of Diseases of Children·J J NoraH A Lubs
Dec 1, 1994·British Heart Journal·H Watkins
Jan 1, 1993·Journal of the American College of Cardiology·J MasuraI D Sullivan
Feb 15, 1993·American Journal of Medical Genetics·M SharlandM A Patton
Feb 1, 1996·Acta Paediatrica Japonica; Overseas Edition·J Noonan, W O'Connor

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Citations

Jun 1, 2011·Palliative & Supportive Care·Mary W ByrneLonnie K Zeltzer
May 17, 2002·The New England Journal of Medicine·David C GoodmanKenneth S Schoendorf
Dec 9, 2000·Gastroenterology Nursing : the Official Journal of the Society of Gastroenterology Nurses and Associates·J W Decker
Sep 30, 1999·Current Opinion in Rheumatology·P H White
Sep 10, 2003·Journal of Pediatric Gastroenterology and Nutrition·Cristina Targa FerreiraJorge Pereira-Lima
Sep 28, 2004·Journal of Pediatric Gastroenterology and Nutrition·Cristina Targa FerreiraThemis Reverbel da Silveira
Feb 15, 2001·The Pediatric Infectious Disease Journal·E L LópezM M Contrini
Aug 13, 2005·The Journal of Trauma·Deependra Vikram SinghRaj Vardhan Azad
Apr 17, 2009·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Alan R HinmanUNKNOWN NDBS Business Process Analysis Workgroup
Aug 22, 2009·Current Opinion in Pulmonary Medicine·Patrick A Flume
Nov 30, 2012·Sexually Transmitted Diseases·Erika WikströmHeljä-Marja Surcel
Feb 5, 2011·Pediatric Emergency Care·Savithiri Ratnapalan, Lopamudra Das
Jun 7, 2012·Pediatric Emergency Care·Jessica RattoJane F Knapp
Jul 23, 2009·The Journal of Trauma·Brendan T CampbellGarry Lapidus
Apr 25, 2000·Annual Review of Medicine·R E Pyeritz
Feb 23, 2011·The Journal of Clinical Investigation·Talita M MarinMaria I Kontaridis
May 5, 2011·Journal of Molecular and Cellular Cardiology·Dominic P Del Re, Junichi Sadoshima
Nov 14, 2006·Cardiology Clinics·Francois P Bernier, Renee Spaetgens
Jan 29, 2011·Congenital Heart Disease·Edward J HickeyLee Benson
Apr 25, 2012·American Journal of Medical Genetics. Part a·Rebecca HobanBarbara Shephard
Apr 24, 2010·Congenital Heart Disease·Ebru AyparFikri Demir
Sep 27, 2014·Seminars in Cell & Developmental Biology·Jessica LauriolMaria I Kontaridis
Jan 1, 2013·The Journal of Molecular Diagnostics : JMD·Polakit TeekakirikulBirgit H Funke
Feb 5, 2016·Circulation Journal : Official Journal of the Japanese Circulation Society·UNKNOWN JCS Joint Working Group
May 2, 2000·Revista do Hospital das Clínicas·D R BertolaC H Gonzalez
Jul 28, 2004·Circulation Journal : Official Journal of the Japanese Circulation Society·Sadamitsu YanagiYoshifumi Kawano
Dec 13, 2016·JCI Insight·Jae-Sung YiAnton M Bennett
Mar 16, 2021·Frontiers in Pediatrics·Emanuele MondaGiuseppe Limongelli

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