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Mesocardia

MedGen UID:
506592
Concept ID:
CN167327
Finding
 
HPO: HP:0011599

Definition

Mesocardia is a abnormal location of the heart in which the heart is in a midline position and the longitudinal axis of the heart lies in the mid-sagittal plane. [from HPO]

Conditions with this feature

Floating-Harbor syndrome
MedGen UID:
152667
Concept ID:
C0729582
Disease or Syndrome
Floating-Harbor syndrome (FHS) is characterized by typical craniofacial features; low birth weight, normal head circumference, and short stature; bone age delay that normalizes between ages six and 12 years; skeletal anomalies (brachydactyly, clubbing, clinodactyly, short thumbs, prominent joints, clavicular abnormalities); severe receptive and expressive language impairment; hypernasality and high-pitched voice; and intellectual disability that is typically mild to moderate. Difficulties with temperament and behavior that are present in many children tend to improve in adulthood. Other features can include: hyperopia and/or strabismus; conductive hearing loss; seizures; gastroesophageal reflux; renal anomalies (e.g., hydronephrosis/renal pelviectasis, cysts, and/or agenesis) and genital anomalies (e.g., hypospadias and/or undescended testes).
Heterotaxy, visceral, 2, autosomal
MedGen UID:
381399
Concept ID:
C1854334
Disease or Syndrome
Heterotaxy ('heter' meaning 'other' and 'taxy' meaning 'arrangement'), or situs ambiguus, is a developmental condition characterized by randomization of the placement of visceral organs, including the heart, lungs, liver, spleen, and stomach. The organs are oriented randomly with respect to the left-right axis and with respect to one another (Srivastava, 1997). Heterotaxy is a clinically and genetically heterogeneous disorder. For a discussion of the genetic heterogeneity of visceral heterotaxy, see HTX1 (306955).

Recent clinical studies

Etiology

Falkensammer CB, Ayres NA, Altman CA, Ge S, Bezold LI, Eidem BW, Kovalchin JP
Am J Perinatol 2008 May;25(5):277-81. Epub 2008 Apr 9 doi: 10.1055/s-2008-1066874. [Epub ahead of print] PMID: 18401841
Rogers DP, Walker F, Chow AW, Lambiase PD
Pacing Clin Electrophysiol 2008 Apr;31(4):499-502. doi: 10.1111/j.1540-8159.2008.01021.x. PMID: 18373771
Paladini D, Volpe P, Marasini M, Russo MG, Vassallo M, Gentile M, Calabrò R
Ultrasound Obstet Gynecol 2006 Mar;27(3):281-5. doi: 10.1002/uog.2715. PMID: 16485324
Aziz S, Soomro GB, Luck NH, Hussain SM, Mirza R, Naqvi SA, Rizvi AH
J Pak Med Assoc 2005 Aug;55(8):350-2. PMID: 16164164
Gonzalez A, Krassikoff N, Gilbert-Barness EF
Am J Med Genet 1989 Apr;32(4):457-60. doi: 10.1002/ajmg.1320320404. PMID: 2773985

Diagnosis

Koca B, Oztunç F, Yalçın Y
Turk Kardiyol Dern Ars 2011 Sep;39(6):499-500. PMID: 21918322
Ozkan H, Cetinkaya M, Köksal N, Yapici S
J Matern Fetal Neonatal Med 2011 Mar;24(3):521-4. doi: 10.3109/14767058.2010.501120. PMID: 21291342
Clarke CJ, Jayakumar KA, Hoyer AW
Pediatr Cardiol 2010 May;31(4):562-3. Epub 2010 Jan 5 doi: 10.1007/s00246-009-9618-8. [Epub ahead of print] PMID: 20049427
Aziz S, Soomro GB, Luck NH, Hussain SM, Mirza R, Naqvi SA, Rizvi AH
J Pak Med Assoc 2005 Aug;55(8):350-2. PMID: 16164164
Gonzalez A, Krassikoff N, Gilbert-Barness EF
Am J Med Genet 1989 Apr;32(4):457-60. doi: 10.1002/ajmg.1320320404. PMID: 2773985

Therapy

Ozkan H, Cetinkaya M, Köksal N, Yapici S
J Matern Fetal Neonatal Med 2011 Mar;24(3):521-4. doi: 10.3109/14767058.2010.501120. PMID: 21291342
Clarke CJ, Jayakumar KA, Hoyer AW
Pediatr Cardiol 2010 May;31(4):562-3. Epub 2010 Jan 5 doi: 10.1007/s00246-009-9618-8. [Epub ahead of print] PMID: 20049427
Paladini D, Volpe P, Marasini M, Russo MG, Vassallo M, Gentile M, Calabrò R
Ultrasound Obstet Gynecol 2006 Mar;27(3):281-5. doi: 10.1002/uog.2715. PMID: 16485324

Prognosis

Ozkan H, Cetinkaya M, Köksal N, Yapici S
J Matern Fetal Neonatal Med 2011 Mar;24(3):521-4. doi: 10.3109/14767058.2010.501120. PMID: 21291342
Bader R, Perrin D, Yoo SJ
Fetal Pediatr Pathol 2004 Jul-Aug;23(4):257-63. PMID: 16095037
Russo P, Danielson GK, Driscoll DJ
Circulation 1987 Sep;76(3 Pt 2):III88-92. PMID: 3621545

Clinical prediction guides

Paladini D, Volpe P, Marasini M, Russo MG, Vassallo M, Gentile M, Calabrò R
Ultrasound Obstet Gynecol 2006 Mar;27(3):281-5. doi: 10.1002/uog.2715. PMID: 16485324
DaCosta H, Pathak A, Noronha O, Dalal S, Shah K, Merchant S
Eur J Nucl Med 1981;6(6):265-7. PMID: 7238544

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