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Dilated cardiomyopathy 1L(CMD1L)

MedGen UID:
335735
Concept ID:
C1847667
Disease or Syndrome
Synonyms: CMD1L; SGCD-Related Dilated Cardiomyopathy
 
Gene (location): SGCD (5q33.2-33.3)
 
Monarch Initiative: MONDO:0011702
OMIM®: 606685

Definition

Dilated cardiomyopathy, a disorder characterized by cardiac dilation and reduced systolic function, represents an outcome of a heterogeneous group of inherited and acquired disorders. For background and phenotypic information on dilated cardiomyopathy, see CMD1A (115200). [from OMIM]

Clinical features

From HPO
Sudden cardiac death
MedGen UID:
38841
Concept ID:
C0085298
Pathologic Function
The heart suddenly and unexpectedly stops beating resulting in death within a short time period (generally within 1 h of symptom onset).
Primary dilated cardiomyopathy
MedGen UID:
2880
Concept ID:
C0007193
Disease or Syndrome
Familial dilated cardiomyopathy is a genetic form of heart disease. It occurs when heart (cardiac) muscle becomes thin and weakened in at least one chamber of the heart, causing the open area of the chamber to become enlarged (dilated). As a result, the heart is unable to pump blood as efficiently as usual. To compensate, the heart attempts to increase the amount of blood being pumped through the heart, leading to further thinning and weakening of the cardiac muscle. Over time, this condition results in heart failure.\n\nIt usually takes many years for symptoms of familial dilated cardiomyopathy to cause health problems. They typically begin in mid-adulthood, but can occur at any time from infancy to late adulthood. Signs and symptoms of familial dilated cardiomyopathy can include an irregular heartbeat (arrhythmia), shortness of breath (dyspnea), extreme tiredness (fatigue), fainting episodes (syncope), and swelling of the legs and feet. In some cases, the first sign of the disorder is sudden cardiac death. The severity of the condition varies among affected individuals, even in members of the same family.
Congestive heart failure
MedGen UID:
9169
Concept ID:
C0018802
Disease or Syndrome
The presence of an abnormality of cardiac function that is responsible for the failure of the heart to pump blood at a rate that is commensurate with the needs of the tissues or a state in which abnormally elevated filling pressures are required for the heart to do so. Heart failure is frequently related to a defect in myocardial contraction.
Reduced left ventricular ejection fraction
MedGen UID:
868398
Concept ID:
C4022792
Finding
A diminution of the volumetric fraction of blood pumped out of the ventricle with each cardiac cycle.
Reduced systolic function
MedGen UID:
870560
Concept ID:
C4025008
Finding
Increased left ventricular end-diastolic volume
MedGen UID:
1660169
Concept ID:
C4748648
Finding
Abnormally high volume of blood in the left ventricle at the end of diastole (just before systole).
Elevated circulating creatine kinase concentration
MedGen UID:
69128
Concept ID:
C0241005
Finding
An elevation of the level of the enzyme creatine kinase (also known as creatine phosphokinase (CK; EC 2.7.3.2) in the blood. CK levels can be elevated in a number of clinical disorders such as myocardial infarction, rhabdomyolysis, and muscular dystrophy.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Etiology

ten Brinke EA, Klautz RJ, Tulner SA, Verwey HF, Bax JJ, Schalij MJ, van der Wall EE, Versteegh MI, Dion RA, Steendijk P
J Thorac Cardiovasc Surg 2010 Dec;140(6):1338-44. Epub 2010 Apr 9 doi: 10.1016/j.jtcvs.2010.01.029. PMID: 20381088
Staudt A, Dörr M, Staudt Y, Böhm M, Probst M, Empen K, Plötz S, Maschke HE, Hummel A, Baumann G, Felix SB
Am Heart J 2005 Oct;150(4):729-36. doi: 10.1016/j.ahj.2004.11.002. PMID: 16209975
Felix SB, Staudt A, Dörffel WV, Stangl V, Merkel K, Pohl M, Döcke WD, Morgera S, Neumayer HH, Wernecke KD, Wallukat G, Stangl K, Baumann G
J Am Coll Cardiol 2000 May;35(6):1590-8. doi: 10.1016/s0735-1097(00)00568-4. PMID: 10807465
Guazzi M, Pontone G, Agostoni P
Am Heart J 1999 Aug;138(2 Pt 1):254-60. doi: 10.1016/s0002-8703(99)70109-2. PMID: 10426836
von Scheidt W, Ziegler U, Kemkes BM, Erdmann E
J Heart Lung Transplant 1991 May-Jun;10(3):342-50. PMID: 1854762

Diagnosis

Moyssakis I, Moschos N, Triposkiadis F, Hallaq Y, Pantazopoulos N, Aessopos A, Kolettis M
Heart Vessels 2005 Sep;20(5):191-8. doi: 10.1007/s00380-005-0832-x. PMID: 16160899
Ogawa S, Matsumura S, Yoshikawa T, Satoh T, Kumagai H, Mitamura H, Iwanaga S, Umezawa A
Keio J Med 2002 Sep;51(3):165-77. PMID: 12371648
Backer CL, Zales VR, Idriss FS, Lynch P, Crawford S, Benson DW Jr, Mavroudis C
J Heart Lung Transplant 1992 Mar-Apr;11(2 Pt 1):311-9. PMID: 1576137
Kulick DL, Bhandari AK, Hong R, Petersen R, Leon C, Rahimtoola SH
Am Heart J 1990 Apr;119(4):878-83. doi: 10.1016/s0002-8703(05)80326-6. PMID: 2321507

Therapy

Felix SB, Staudt A, Dörffel WV, Stangl V, Merkel K, Pohl M, Döcke WD, Morgera S, Neumayer HH, Wernecke KD, Wallukat G, Stangl K, Baumann G
J Am Coll Cardiol 2000 May;35(6):1590-8. doi: 10.1016/s0735-1097(00)00568-4. PMID: 10807465
Guazzi M, Pontone G, Agostoni P
Am Heart J 1999 Aug;138(2 Pt 1):254-60. doi: 10.1016/s0002-8703(99)70109-2. PMID: 10426836
Backer CL, Zales VR, Idriss FS, Lynch P, Crawford S, Benson DW Jr, Mavroudis C
J Heart Lung Transplant 1992 Mar-Apr;11(2 Pt 1):311-9. PMID: 1576137
von Scheidt W, Ziegler U, Kemkes BM, Erdmann E
J Heart Lung Transplant 1991 May-Jun;10(3):342-50. PMID: 1854762
Sundram P, Reddy HK, McElroy PA, Janicki JS, Weber KT
Am Heart J 1990 Apr;119(4):891-8. doi: 10.1016/s0002-8703(05)80328-x. PMID: 2321508

Prognosis

ten Brinke EA, Klautz RJ, Tulner SA, Verwey HF, Bax JJ, Schalij MJ, van der Wall EE, Versteegh MI, Dion RA, Steendijk P
J Thorac Cardiovasc Surg 2010 Dec;140(6):1338-44. Epub 2010 Apr 9 doi: 10.1016/j.jtcvs.2010.01.029. PMID: 20381088
Ogawa S, Matsumura S, Yoshikawa T, Satoh T, Kumagai H, Mitamura H, Iwanaga S, Umezawa A
Keio J Med 2002 Sep;51(3):165-77. PMID: 12371648
Felix SB, Staudt A, Dörffel WV, Stangl V, Merkel K, Pohl M, Döcke WD, Morgera S, Neumayer HH, Wernecke KD, Wallukat G, Stangl K, Baumann G
J Am Coll Cardiol 2000 May;35(6):1590-8. doi: 10.1016/s0735-1097(00)00568-4. PMID: 10807465
Backer CL, Zales VR, Idriss FS, Lynch P, Crawford S, Benson DW Jr, Mavroudis C
J Heart Lung Transplant 1992 Mar-Apr;11(2 Pt 1):311-9. PMID: 1576137
von Scheidt W, Ziegler U, Kemkes BM, Erdmann E
J Heart Lung Transplant 1991 May-Jun;10(3):342-50. PMID: 1854762

Clinical prediction guides

ten Brinke EA, Klautz RJ, Tulner SA, Verwey HF, Bax JJ, Schalij MJ, van der Wall EE, Versteegh MI, Dion RA, Steendijk P
J Thorac Cardiovasc Surg 2010 Dec;140(6):1338-44. Epub 2010 Apr 9 doi: 10.1016/j.jtcvs.2010.01.029. PMID: 20381088
Lang CC, Karlin P, Haythe J, Tsao L, Mancini DM
Am J Cardiol 2007 Feb 1;99(3):404-5. Epub 2006 Dec 13 doi: 10.1016/j.amjcard.2006.08.047. PMID: 17261407
Staudt A, Staudt Y, Dörr M, Böhm M, Knebel F, Hummel A, Wunderle L, Tiburcy M, Wernecke KD, Baumann G, Felix SB
J Am Coll Cardiol 2004 Aug 18;44(4):829-36. doi: 10.1016/j.jacc.2004.04.055. PMID: 15312867
Ogawa S, Matsumura S, Yoshikawa T, Satoh T, Kumagai H, Mitamura H, Iwanaga S, Umezawa A
Keio J Med 2002 Sep;51(3):165-77. PMID: 12371648
Guazzi M, Pontone G, Agostoni P
Am Heart J 1999 Aug;138(2 Pt 1):254-60. doi: 10.1016/s0002-8703(99)70109-2. PMID: 10426836

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