U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Hypertrophic cardiomyopathy 25(CMH25)

MedGen UID:
895360
Concept ID:
C4225408
Disease or Syndrome
Synonyms: CARDIOMYOPATHY, FAMILIAL HYPERTROPHIC, 25; CMH25; Dilated cardiomyopathy 1N; TCAP-Related Dilated Cardiomyopathy
 
Gene (location): TCAP (17q12)
 
Monarch Initiative: MONDO:0011843
OMIM®: 607487

Definition

Any hypertrophic cardiomyopathy in which the cause of the disease is a mutation in the TCAP gene. [from MONDO]

Clinical features

From HPO
Hypertrophic cardiomyopathy
MedGen UID:
2881
Concept ID:
C0007194
Disease or Syndrome
Hypertrophic cardiomyopathy (HCM) is defined by the presence of increased ventricular wall thickness or mass in the absence of loading conditions (hypertension, valve disease) sufficient to cause the observed abnormality.
Wolff-Parkinson-White pattern
MedGen UID:
12162
Concept ID:
C0043202
Disease or Syndrome
Wolff-Parkinson-White syndrome is a condition characterized by abnormal electrical pathways in the heart that cause a disruption of the heart's normal rhythm (arrhythmia).\n\nThe heartbeat is controlled by electrical signals that move through the heart in a highly coordinated way. A specialized cluster of cells called the atrioventricular node conducts electrical impulses from the heart's upper chambers (the atria) to the lower chambers (the ventricles). Impulses move through the atrioventricular node during each heartbeat, stimulating the ventricles to contract slightly later than the atria.\n\nPeople with Wolff-Parkinson-White syndrome are born with an extra connection in the heart, called an accessory pathway, that allows electrical signals to bypass the atrioventricular node and move from the atria to the ventricles faster than usual. The accessory pathway may also transmit electrical impulses abnormally from the ventricles back to the atria. This extra connection can disrupt the coordinated movement of electrical signals through the heart, leading to an abnormally fast heartbeat (tachycardia) and other changes in heart rhythm. Resulting symptoms include dizziness, a sensation of fluttering or pounding in the chest (palpitations), shortness of breath, and fainting (syncope). In rare cases, arrhythmias associated with Wolff-Parkinson-White syndrome can lead to cardiac arrest and sudden death. The most common arrhythmia associated with Wolff-Parkinson-White syndrome is called paroxysmal supraventricular tachycardia.\n\nComplications of Wolff-Parkinson-White syndrome can occur at any age, although some individuals born with an accessory pathway in the heart never experience any health problems associated with the condition.\n\nWolff-Parkinson-White syndrome often occurs with other structural abnormalities of the heart or underlying heart disease. The most common heart defect associated with the condition is Ebstein anomaly, which affects the valve that allows blood to flow from the right atrium to the right ventricle (the tricuspid valve). Additionally, the heart rhythm problems associated with Wolff-Parkinson-White syndrome can be a component of several other genetic syndromes, including hypokalemic periodic paralysis (a condition that causes episodes of extreme muscle weakness), Pompe disease (a disorder characterized by the storage of excess glycogen), Danon disease (a condition that weakens the heart and skeletal muscles and causes intellectual disability), and tuberous sclerosis complex (a condition that results in the growth of noncancerous tumors in many parts of the body).
Left ventricular hypertrophy
MedGen UID:
57442
Concept ID:
C0149721
Disease or Syndrome
Enlargement or increased size of the heart left ventricle.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
J Am Coll Cardiol 2020 Dec 22;76(25):e159-e240. Epub 2020 Nov 20 doi: 10.1016/j.jacc.2020.08.045. PMID: 33229116
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
Circulation 2020 Dec 22;142(25):e533-e557. Epub 2020 Nov 20 doi: 10.1161/CIR.0000000000000938. PMID: 33215938
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
Circulation 2020 Dec 22;142(25):e558-e631. Epub 2020 Nov 20 doi: 10.1161/CIR.0000000000000937. PMID: 33215931

Recent clinical studies

Etiology

Desai MY, Owens A, Geske JB, Wolski K, Saberi S, Wang A, Sherrid M, Cremer PC, Naidu SS, Smedira NG, Schaff H, McErlean E, Sewell C, Balasubramanyam A, Lampl K, Sehnert AJ, Nissen SE
Circulation 2023 Mar 14;147(11):850-863. Epub 2022 Nov 6 doi: 10.1161/CIRCULATIONAHA.122.062534. PMID: 36335531
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
J Am Coll Cardiol 2020 Dec 22;76(25):e159-e240. Epub 2020 Nov 20 doi: 10.1016/j.jacc.2020.08.045. PMID: 33229116
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
Circulation 2020 Dec 22;142(25):e533-e557. Epub 2020 Nov 20 doi: 10.1161/CIR.0000000000000938. PMID: 33215938
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
Circulation 2020 Dec 22;142(25):e558-e631. Epub 2020 Nov 20 doi: 10.1161/CIR.0000000000000937. PMID: 33215931
Rowin EJ, Maron BJ, Olivotto I, Maron MS
JACC Cardiovasc Imaging 2017 Nov;10(11):1374-1386. doi: 10.1016/j.jcmg.2017.07.016. PMID: 29122139

Diagnosis

Lopes LR, Garcia-Hernández S, Lorenzini M, Futema M, Chumakova O, Zateyshchikov D, Isidoro-Garcia M, Villacorta E, Escobar-Lopez L, Garcia-Pavia P, Bilbao R, Dobarro D, Sandin-Fuentes M, Catalli C, Gener Querol B, Mezcua A, Garcia Pinilla J, Bloch Rasmussen T, Ferreira-Aguar A, Revilla-Martí P, Basurte Elorz MT, Bautista Paves A, Ramon Gimeno J, Figueroa AV, Franco-Gutierrez R, Fuentes-Cañamero ME, Martinez Moreno M, Ortiz-Genga M, Piqueras-Flores J, Analia Ramos K, Rudzitis A, Ruiz-Guerrero L, Stein R, Triguero-Bocharán M, de la Higuera L, Ochoa JP, Abu-Bonsrah D, Kwok CYT, Smith JB, Porrello ER, Akhtar MM, Jager J, Ashworth M, Syrris P, Elliott DA, Monserrat L, Elliott PM
Eur Heart J 2021 Aug 21;42(32):3063-3073. doi: 10.1093/eurheartj/ehab424. PMID: 34263907Free PMC Article
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
J Am Coll Cardiol 2020 Dec 22;76(25):e159-e240. Epub 2020 Nov 20 doi: 10.1016/j.jacc.2020.08.045. PMID: 33229116
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
Circulation 2020 Dec 22;142(25):e533-e557. Epub 2020 Nov 20 doi: 10.1161/CIR.0000000000000938. PMID: 33215938
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
Circulation 2020 Dec 22;142(25):e558-e631. Epub 2020 Nov 20 doi: 10.1161/CIR.0000000000000937. PMID: 33215931
Rowin EJ, Maron BJ, Olivotto I, Maron MS
JACC Cardiovasc Imaging 2017 Nov;10(11):1374-1386. doi: 10.1016/j.jcmg.2017.07.016. PMID: 29122139

Therapy

Lo ST, Walker AL, Georges CJ, Li RH, Stern JA
J Feline Med Surg 2022 Apr;24(4):277-283. Epub 2021 May 10 doi: 10.1177/1098612X211013736. PMID: 33966532Free PMC Article
Hegde SM, Lester SJ, Solomon SD, Michels M, Elliott PM, Nagueh SF, Choudhury L, Zemanek D, Zwas DR, Jacoby D, Wang A, Ho CY, Li W, Sehnert AJ, Olivotto I, Abraham TP
J Am Coll Cardiol 2021 Dec 21;78(25):2518-2532. doi: 10.1016/j.jacc.2021.09.1381. PMID: 34915982
Dybro AM, Rasmussen TB, Nielsen RR, Andersen MJ, Jensen MK, Poulsen SH
J Am Coll Cardiol 2021 Dec 21;78(25):2505-2517. doi: 10.1016/j.jacc.2021.07.065. PMID: 34915981
Ramaswamy VV, Bandyopadhyay T, Nanda D, Bandiya P, Ahmed J, Garg A, Roehr CC, Nangia S
JAMA Pediatr 2021 Jun 1;175(6):e206826. Epub 2021 Jun 7 doi: 10.1001/jamapediatrics.2020.6826. PMID: 33720274Free PMC Article
Heitner SB, Jacoby D, Lester SJ, Owens A, Wang A, Zhang D, Lambing J, Lee J, Semigran M, Sehnert AJ
Ann Intern Med 2019 Jun 4;170(11):741-748. Epub 2019 Apr 30 doi: 10.7326/M18-3016. PMID: 31035291

Prognosis

Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
Circulation 2020 Dec 22;142(25):e533-e557. Epub 2020 Nov 20 doi: 10.1161/CIR.0000000000000938. PMID: 33215938
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
Circulation 2020 Dec 22;142(25):e558-e631. Epub 2020 Nov 20 doi: 10.1161/CIR.0000000000000937. PMID: 33215931
Miron A, Lafreniere-Roula M, Steve Fan CP, Armstrong KR, Dragulescu A, Papaz T, Manlhiot C, Kaufman B, Butts RJ, Gardin L, Stephenson EA, Howard TS, Aziz PF, Balaji S, Ladouceur VB, Benson LN, Colan SD, Godown J, Henderson HT, Ingles J, Jeewa A, Jefferies JL, Lal AK, Mathew J, Jean-St-Michel E, Michels M, Nakano SJ, Olivotto I, Parent JJ, Pereira AC, Semsarian C, Whitehill RD, Wittekind SG, Russell MW, Conway J, Richmond ME, Villa C, Weintraub RG, Rossano JW, Kantor PF, Ho CY, Mital S
Circulation 2020 Jul 21;142(3):217-229. Epub 2020 May 18 doi: 10.1161/CIRCULATIONAHA.120.047235. PMID: 32418493Free PMC Article
Rowin EJ, Maron BJ, Olivotto I, Maron MS
JACC Cardiovasc Imaging 2017 Nov;10(11):1374-1386. doi: 10.1016/j.jcmg.2017.07.016. PMID: 29122139
Bille K, Figueiras D, Schamasch P, Kappenberger L, Brenner JI, Meijboom FJ, Meijboom EJ
Eur J Cardiovasc Prev Rehabil 2006 Dec;13(6):859-75. doi: 10.1097/01.hjr.0000238397.50341.4a. PMID: 17143117

Clinical prediction guides

Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
Circulation 2020 Dec 22;142(25):e533-e557. Epub 2020 Nov 20 doi: 10.1161/CIR.0000000000000938. PMID: 33215938
Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P
Circulation 2020 Dec 22;142(25):e558-e631. Epub 2020 Nov 20 doi: 10.1161/CIR.0000000000000937. PMID: 33215931
Heitner SB, Jacoby D, Lester SJ, Owens A, Wang A, Zhang D, Lambing J, Lee J, Semigran M, Sehnert AJ
Ann Intern Med 2019 Jun 4;170(11):741-748. Epub 2019 Apr 30 doi: 10.7326/M18-3016. PMID: 31035291
Rowin EJ, Maron BJ, Olivotto I, Maron MS
JACC Cardiovasc Imaging 2017 Nov;10(11):1374-1386. doi: 10.1016/j.jcmg.2017.07.016. PMID: 29122139
Herman DS, Lam L, Taylor MR, Wang L, Teekakirikul P, Christodoulou D, Conner L, DePalma SR, McDonough B, Sparks E, Teodorescu DL, Cirino AL, Banner NR, Pennell DJ, Graw S, Merlo M, Di Lenarda A, Sinagra G, Bos JM, Ackerman MJ, Mitchell RN, Murry CE, Lakdawala NK, Ho CY, Barton PJ, Cook SA, Mestroni L, Seidman JG, Seidman CE
N Engl J Med 2012 Feb 16;366(7):619-28. doi: 10.1056/NEJMoa1110186. PMID: 22335739Free PMC Article

Recent systematic reviews

Bayonas-Ruiz A, Muñoz-Franco FM, Sabater-Molina M, Oliva-Sandoval MJ, Gimeno JR, Bonacasa B
ESC Heart Fail 2023 Feb;10(1):8-23. Epub 2022 Oct 1 doi: 10.1002/ehf2.14142. PMID: 36181355Free PMC Article
Moolla M, Mathew A, John K, Yogasundaram H, Alhumaid W, Campbell S, Windram J
Int J Cardiol 2022 Jul 15;359:54-60. Epub 2022 Apr 12 doi: 10.1016/j.ijcard.2022.04.034. PMID: 35427704
Ramaswamy VV, Bandyopadhyay T, Nanda D, Bandiya P, Ahmed J, Garg A, Roehr CC, Nangia S
JAMA Pediatr 2021 Jun 1;175(6):e206826. Epub 2021 Jun 7 doi: 10.1001/jamapediatrics.2020.6826. PMID: 33720274Free PMC Article
Providencia R, Elliott P, Patel K, McCready J, Babu G, Srinivasan N, Bronis K, Papageorgiou N, Chow A, Rowland E, Lowe M, Segal OR, Lambiase PD
Heart 2016 Oct 1;102(19):1533-43. Epub 2016 May 27 doi: 10.1136/heartjnl-2016-309406. PMID: 27234160
Bille K, Figueiras D, Schamasch P, Kappenberger L, Brenner JI, Meijboom FJ, Meijboom EJ
Eur J Cardiovasc Prev Rehabil 2006 Dec;13(6):859-75. doi: 10.1097/01.hjr.0000238397.50341.4a. PMID: 17143117

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...