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Sudden cardiac failure, infantile(SCFI)

MedGen UID:
934631
Concept ID:
C4310664
Disease or Syndrome
Synonym: SCFI
 
Gene (location): PPA2 (4q24)
 
Monarch Initiative: MONDO:0014973
OMIM®: 617222

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).
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.
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.
Myocarditis
MedGen UID:
44553
Concept ID:
C0027059
Disease or Syndrome
Inflammation of the myocardium.
Myocardial fibrosis
MedGen UID:
56239
Concept ID:
C0151654
Pathologic Function
Myocardial fibrosis is characterized by dysregulated collagen turnover (increased synthesis predominates over unchanged or decreased degradation) and excessive diffuse collagen accumulation in the interstitial and perivascular spaces as well as by phenotypically transformed fibroblasts, termed myofibroblasts.
Bradycardia
MedGen UID:
140901
Concept ID:
C0428977
Finding
A slower than normal heart rate (in adults, slower than 60 beats per minute).
Seizure
MedGen UID:
20693
Concept ID:
C0036572
Sign or Symptom
A seizure is an intermittent abnormality of nervous system physiology characterized by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
Hypotonia
MedGen UID:
10133
Concept ID:
C0026827
Finding
Hypotonia is an abnormally low muscle tone (the amount of tension or resistance to movement in a muscle). Even when relaxed, muscles have a continuous and passive partial contraction which provides some resistance to passive stretching. Hypotonia thus manifests as diminished resistance to passive stretching. Hypotonia is not the same as muscle weakness, although the two conditions can co-exist.
Metabolic acidosis
MedGen UID:
65117
Concept ID:
C0220981
Pathologic Function
Metabolic acidosis (MA) is characterized by a fall in blood pH due to a reduction of serum bicarbonate concentration. This can occur as a result of either the accumulation of acids (high anion gap MA) or the loss of bicarbonate from the gastrointestinal tract or the kidney (hyperchloremic MA). By definition, MA is not due to a respirary cause.

Recent clinical studies

Etiology

Zahka K, Kalidas K, Simpson MA, Cross H, Keller BB, Galambos C, Gurtz K, Patton MA, Crosby AH
Heart 2008 Oct;94(10):1326-30. Epub 2008 May 8 doi: 10.1136/hrt.2007.127241. PMID: 18467358
Constantinou JE, Gillis J, Ouvrier RA, Rahilly PM
Arch Dis Child 1989 May;64(5):703-8. doi: 10.1136/adc.64.5.703. PMID: 2730124Free PMC Article

Diagnosis

Zhao A, Shen J, Ding Y, Sheng M, Zuo M, Lv H, Wang J, Shen Y, Wang H, Sun L
Clin Chim Acta 2021 Aug;519:163-171. Epub 2021 Apr 5 doi: 10.1016/j.cca.2021.03.029. PMID: 33826954
Miura A, Kondo H, Yamamoto T, Okumura Y, Nishio H
Int Heart J 2020 Sep 29;61(5):1079-1083. Epub 2020 Sep 2 doi: 10.1536/ihj.20-155. PMID: 32879264
Xie H, Chen X, Chen N, Zhou Q
Am J Forensic Med Pathol 2017 Mar;38(1):32-34. doi: 10.1097/PAF.0000000000000289. PMID: 28045696
Finsterer J, Stöllberger C
Pediatr Neurol 2015 Dec;53(6):473-7. Epub 2015 Aug 28 doi: 10.1016/j.pediatrneurol.2015.08.014. PMID: 26507755
Tein I
J Child Neurol 2002 Dec;17 Suppl 3:3S57-82; discussion 3S82-3. PMID: 12597056

Therapy

Sakurai Y, Toriumi N, Sarashina T, Ishioka T, Nagata M, Kobayashi H, Azuma H
J Med Case Rep 2022 Jun 30;16(1):268. doi: 10.1186/s13256-022-03448-x. PMID: 35773682Free PMC Article
Kim YM, Kim G, Ko H, Yoo HW, Lee HD
Medicine (Baltimore) 2018 May;97(20):e10813. doi: 10.1097/MD.0000000000010813. PMID: 29768383Free PMC Article
But WM, Lee SH, Chan AO, Lau GT
Hong Kong Med J 2009 Dec;15(6):474-7. PMID: 19966354
Tein I
J Child Neurol 2002 Dec;17 Suppl 3:3S57-82; discussion 3S82-3. PMID: 12597056

Prognosis

Miura A, Kondo H, Yamamoto T, Okumura Y, Nishio H
Int Heart J 2020 Sep 29;61(5):1079-1083. Epub 2020 Sep 2 doi: 10.1536/ihj.20-155. PMID: 32879264
Kasuda S, Kudo R, Yuui K, Kondo T, Hatake K
Forensic Sci Med Pathol 2019 Jun;15(2):300-303. Epub 2018 Dec 15 doi: 10.1007/s12024-018-0066-8. PMID: 30554350
Zahka K, Kalidas K, Simpson MA, Cross H, Keller BB, Galambos C, Gurtz K, Patton MA, Crosby AH
Heart 2008 Oct;94(10):1326-30. Epub 2008 May 8 doi: 10.1136/hrt.2007.127241. PMID: 18467358
Chong CR, Hutchins GM
Pediatr Dev Pathol 2008 Sep-Oct;11(5):405-15. Epub 2007 Aug 23 doi: 10.2350/07-06-0297.1. PMID: 17990935
Tanaka N, Sekimoto K, Naoe S
Arch Pathol Lab Med 1976 Feb;100(2):81-6. PMID: 3150

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