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Dilated cardiomyopathy 1X(CMD1X)

MedGen UID:
370583
Concept ID:
C1969024
Disease or Syndrome
Synonyms: CARDIOMYOPATHY, DILATED, WITH MILD OR NO PROXIMAL MUSCLE WEAKNESS; CMD1X; FKTN-Related Dilated Cardiomyopathy
 
Gene (location): FKTN (9q31.2)
 
Monarch Initiative: MONDO:0012704
OMIM®: 611615

Definition

Any familial isolated dilated cardiomyopathy in which the cause of the disease is a mutation in the FKTN gene. [from MONDO]

Clinical features

From HPO
Primary dilated cardiomyopathy
MedGen UID:
2880
Concept ID:
C0007193
Disease or Syndrome
Dilated cardiomyopathy is a form of heart disease in which the heart (cardiac) muscle becomes thin and enlarged (dilated). The dilation, which typically starts in the lower left chamber of the heart (left ventricle), makes it harder for the heart to pump blood to the rest of the body. \n\nDilated cardiomyopathy is called nonsyndromic dilated cardiomyopathy when it cannot be explained by other causes, such as a heart attack or damage to the valves of the heart, and is not associated with signs and symptoms that affect other parts of the body.  \n\nThe signs and symptoms of nonsyndromic dilated cardiomyopathy vary among affected individuals, even among members of the same family. The signs and symptoms typically begin in mid-adulthood, but they can occur at any time from infancy to late adulthood. Affected individuals may have a sensation of fluttering or pounding in the chest (palpitations); shortness of breath, especially when lying down or during physical activity; fatigue; and swelling of the legs and feet. Affected individuals may also have episodes of dizziness or fainting (syncope). \n\nOver time, people with nonsyndromic dilated cardiomyopathy may develop life-threatening complications, which can include abnormal heart rhythms (arrhythmias) and heart failure. Although uncommon, sudden death can occur in people with nonsyndromic dilated cardiomyopathy, even if they have no other symptoms of the condition.\n\n
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.
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).
Proximal muscle weakness
MedGen UID:
113169
Concept ID:
C0221629
Finding
A lack of strength of the proximal muscles.
Gowers sign
MedGen UID:
65865
Concept ID:
C0234182
Finding
A phenomenon whereby patients are not able to stand up without the use of the hands owing to weakness of the proximal muscles of the lower limbs.
Calf muscle hypertrophy
MedGen UID:
335868
Concept ID:
C1843057
Finding
Muscle hypertrophy affecting the calf muscles.
Increased variability in muscle fiber diameter
MedGen UID:
336019
Concept ID:
C1843700
Finding
An abnormally high degree of muscle fiber size variation. This phenotypic feature can be observed upon muscle biopsy.
Elevated circulating creatine kinase activity
MedGen UID:
69128
Concept ID:
C0241005
Finding
The activity of creatine kinase in the blood circulation is above the upper limit of normal.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Diagnosis

Nanjo S, Yoshikawa K, Harada M, Inoue Y, Namiki A, Nakano H, Yamazaki J
Circ J 2009 Oct;73(10):1939-44. Epub 2009 Sep 4 doi: 10.1253/circj.cj-08-0965. PMID: 19729860

Prognosis

Nanjo S, Yoshikawa K, Harada M, Inoue Y, Namiki A, Nakano H, Yamazaki J
Circ J 2009 Oct;73(10):1939-44. Epub 2009 Sep 4 doi: 10.1253/circj.cj-08-0965. PMID: 19729860

Clinical prediction guides

Nanjo S, Yoshikawa K, Harada M, Inoue Y, Namiki A, Nakano H, Yamazaki J
Circ J 2009 Oct;73(10):1939-44. Epub 2009 Sep 4 doi: 10.1253/circj.cj-08-0965. PMID: 19729860
Chung N, Nishimura RA, Holmes DR Jr, Tajik AJ
J Am Soc Echocardiogr 1992 Mar-Apr;5(2):147-52. doi: 10.1016/s0894-7317(14)80544-0. PMID: 1571168

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