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Third degree atrioventricular block

MedGen UID:
56230
Concept ID:
C0151517
Disease or Syndrome
Synonyms: Complete heart block; Third-degree heart block
SNOMED CT: High grade atrioventricular block (27885002); Complete atrioventricular block (27885002); Third degree atrioventricular block (27885002); Third degree heart block (27885002); Complete heart block (27885002); CHB - Complete heart block (27885002)
 
HPO: HP:0001709

Definition

A disorder characterized by an electrocardiographic finding of complete failure of atrial electrical impulse conduction to the ventricles. This is manifested on the ECG by disassociation of atrial and ventricular rhythms. The atrial rate must be faster than the ventricular rate. (CDISC) [from NCI]

Conditions with this feature

Kearns Sayre syndrome
MedGen UID:
9618
Concept ID:
C0022541
Disease or Syndrome
A mitochondrial disorder featuring the triad of chronic progressive EXTERNAL OPHTHALMOPLEGIA, cardiomyopathy (CARDIOMYOPATHIES) with conduction block (HEART BLOCK), and RETINITIS PIGMENTOSA. Disease onset is in the first or second decade. Elevated CSF protein, sensorineural deafness, seizures, and pyramidal signs may also be present. Ragged-red fibers are found on muscle biopsy. (Adams et al., Principles of Neurology, 6th ed, p984)
Noonan syndrome with multiple lentigines
MedGen UID:
104494
Concept ID:
C0175704
Disease or Syndrome
Noonan syndrome with multiple lentigines (NSML) is a condition in which the cardinal features consist of lentigines, hypertrophic cardiomyopathy, short stature, pectus deformity, and dysmorphic facial features, including widely spaced eyes and ptosis. Multiple lentigines present as dispersed flat, black-brown macules, mostly on the face, neck and upper part of the trunk with sparing of the mucosa. In general, lentigines do not appear until age four to five years but then increase to the thousands by puberty. Some individuals with NSML do not exhibit lentigines. Approximately 85% of affected individuals have heart defects, including hypertrophic cardiomyopathy (HCM) (typically appearing during infancy and sometimes progressive) and pulmonary valve stenosis. Postnatal growth retardation resulting in short stature occurs in fewer than 50% of affected persons, although most affected individuals have a height that is less than the 25th percentile for age. Sensorineural hearing deficits, present in approximately 20%, are poorly characterized. Intellectual disability, typically mild, is observed in approximately 30% of persons with NSML.

Recent clinical studies

Etiology

Takaoka H, Funabashi N, Ozawa K, Uehara M, Ueda M, Horikoshi T, Uno T, Kobayashi Y
Int J Cardiol 2017 Feb 1;228:700-706. Epub 2016 Nov 11 doi: 10.1016/j.ijcard.2016.11.187. PMID: 27886613
Zhang S, Yang J, Jin X, Zhang S
BMC Cardiovasc Disord 2015 Dec 18;15:173. doi: 10.1186/s12872-015-0164-4. PMID: 26681197Free PMC Article
van Cleef AN, Schuurman MJ, Busari JO
BMJ Case Rep 2011 Oct 4;2011 doi: 10.1136/bcr.07.2011.4547. PMID: 22679160Free PMC Article
Rotstein A, Charrow J, Deal BJ
Pediatr Cardiol 2008 Jan;29(1):202-4. Epub 2007 Sep 13 doi: 10.1007/s00246-007-9057-3. PMID: 17851632
Kodama-Takahashi K, Kurata A, Ohshima K, Yamamoto K, Uemura S, Watanabe S, Iwata T
Chest 2003 Apr;123(4):1161-9. PMID: 12684307

Diagnosis

Takaoka H, Funabashi N, Ozawa K, Uehara M, Ueda M, Horikoshi T, Uno T, Kobayashi Y
Int J Cardiol 2017 Feb 1;228:700-706. Epub 2016 Nov 11 doi: 10.1016/j.ijcard.2016.11.187. PMID: 27886613
Zhang S, Yang J, Jin X, Zhang S
BMC Cardiovasc Disord 2015 Dec 18;15:173. doi: 10.1186/s12872-015-0164-4. PMID: 26681197Free PMC Article
Aaberg AM, Eriksson AE, Madsen PL, Dixen U
BMJ Case Rep 2015 Oct 27;2015 doi: 10.1136/bcr-2015-211441. PMID: 26508117Free PMC Article
Şahin Yıldız B, Astarcıoğlu MA, Başkurt Aladağ N, Aykan AÇ, Hasdemir H, Şahin A, Yıldız M
Ulus Travma Acil Cerrahi Derg 2015 May;21(3):193-6. PMID: 26033652
Theodoropoulos I, Cheeyandira A, Tortella BJ
J Emerg Med 2013 Aug;45(2):175-7. Epub 2013 Jun 15 doi: 10.1016/j.jemermed.2012.11.105. PMID: 23777772

Therapy

Behling J, Kaes J, Münzel T, Grabbe S, Loquai C
Melanoma Res 2017 Apr;27(2):155-158. doi: 10.1097/CMR.0000000000000314. PMID: 27977496
Zhang S, Yang J, Jin X, Zhang S
BMC Cardiovasc Disord 2015 Dec 18;15:173. doi: 10.1186/s12872-015-0164-4. PMID: 26681197Free PMC Article
Kodama-Takahashi K, Kurata A, Ohshima K, Yamamoto K, Uemura S, Watanabe S, Iwata T
Chest 2003 Apr;123(4):1161-9. PMID: 12684307
Guedira N, Hajjaj-Hassouni N, Srairi JE, el Hassani S, Fellat R, Benomar M
Rev Rhum Engl Ed 1998 Jan;65(1):58-62. PMID: 9523387
Kottkamp H, Chen X, Hindricks G, Willems S, Haverkamp W, Wichter T, Breithardt G, Borggrefe M
Eur Heart J 1995 May;16(5):647-50. PMID: 7588896

Prognosis

Miwa E, Tani T, Okada Y, Furukawa Y
Echocardiography 2017 Mar;34(3):474-475. Epub 2017 Jan 31 doi: 10.1111/echo.13445. PMID: 28139004
Behling J, Kaes J, Münzel T, Grabbe S, Loquai C
Melanoma Res 2017 Apr;27(2):155-158. doi: 10.1097/CMR.0000000000000314. PMID: 27977496
Portet N, Riu B, Bounes V, Minville V, Fourcade O
J Emerg Med 2012 Dec;43(6):e385-8. Epub 2011 May 28 doi: 10.1016/j.jemermed.2010.11.059. PMID: 21621364
Skog A, Wahren-Herlenius M, Sundström B, Bremme K, Sonesson SE
Pediatrics 2008 Apr;121(4):e803-9. doi: 10.1542/peds.2007-1659. PMID: 18381509
Lionakis N, Moyssakis I, Gialafos E, Dalianis N, Votteas V
J Clin Hypertens (Greenwich) 2008 Jan;10(1):69-72. PMID: 18174773

Clinical prediction guides

Tolbert D, Gordon J, Harris S, Walzer M, Bekersky I, Reid S
Clin Ther 2017 Oct;39(10):2073-2086. Epub 2017 Sep 27 doi: 10.1016/j.clinthera.2017.08.020. PMID: 28958437
Kosmidou I, Redfors B, Dordi R, Dizon JM, McAndrew T, Mehran R, Ben-Yehuda O, Mintz GS, Stone GW
Am J Cardiol 2017 May 1;119(9):1295-1301. Epub 2017 Feb 16 doi: 10.1016/j.amjcard.2017.01.019. PMID: 28267964
Hoxha A, Ruffatti A, Ambrosi A, Ottosson V, Hedlund M, Ottosson L, Anandapadamanaban M, Sunnerhagen M, Sonesson SE, Wahren-Herlenius M
Clin Exp Immunol 2016 Dec;186(3):284-291. Epub 2016 Sep 13 doi: 10.1111/cei.12854. PMID: 27548532Free PMC Article
Jordaens L
Heart 1996 Jun;75(6):542-3. PMID: 8697152Free PMC Article
Bergfeldt L, Rosenqvist M, Vallin H, Edhag O
Br Heart J 1985 Mar;53(3):328-34. PMID: 3970790Free PMC Article

Recent systematic reviews

Boriani G, Tukkie R, Manolis AS, Mont L, Pürerfellner H, Santini M, Inama G, Serra P, de Sousa J, Botto GL, Mangoni L, Grammatico A, Padeletti L; MINERVA Investigators.
Eur Heart J 2014 Sep 14;35(35):2352-62. Epub 2014 Apr 25 doi: 10.1093/eurheartj/ehu165. PMID: 24771721Free PMC Article
Everly MJ, Heaton PC, Cluxton RJ Jr
Ann Pharmacother 2004 Feb;38(2):286-93. Epub 2003 Dec 30 doi: 10.1345/aph.1C472. PMID: 14742768
White WB, Johnson MF, Anders RJ, Elliott WJ, Black HR
Am Heart J 2001 Dec;142(6):1010-5. doi: 10.1067/mhj.2001.119127. PMID: 11717605

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