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Acardius

MedGen UID:
540110
•Concept ID:
C0266717
•
Congenital Abnormality
Synonyms: Acardiac Malformation; Acardiac Twin; Acardiac twins; Acardiacus; TRAP Sequence; Twin Reversed Arterial Perfusion Sequence Malformation
SNOMED CT: Acardius (84648007); Acardiacus (84648007); Acardiac twins (84648007)

Definition

A severe form of twin-twin transfusion syndrome that occurs in monochorionic pregnancies. The normal twin (pump twin) supplies the blood flow to its sibling that lacks heart or brain or both (acardiac/acephalic twin). Untreated, it may lead to the demise of the pump twin in some cases. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcardius

Professional guidelines

PubMed

ACR Appropriateness Criteria(®) Multiple Gestations.
Expert Panel on Women’s Imaging:, Glanc P, Nyberg DA, Khati NJ, Deshmukh SP, Dudiak KM, Henrichsen TL, Poder L, Shipp TD, Simpson L, Weber TM, Zelop CM
J Am Coll Radiol 2017 Nov;14(11S):S476-S489. doi: 10.1016/j.jacr.2017.08.051. PMID: 29101986
Prenatal diagnosis and outcome of multiple pregnancies with reversed arterial perfusion (TRAP-sequence).
Hartge DR, Weichert J
Arch Gynecol Obstet 2012 Jul;286(1):81-8. Epub 2012 Mar 6 doi: 10.1007/s00404-012-2283-9. PMID: 22392490
Acardiac twin: a systematic review of minimally invasive treatment modalities.
Tan TY, Sepulveda W
Ultrasound Obstet Gynecol 2003 Oct;22(4):409-19. doi: 10.1002/uog.224. PMID: 14528480

Recent clinical studies

Etiology

Hypothesized pathogenesis of acardius acephalus, acormus, amorphus, anceps, acardiac edema, single umbilical artery, and pump twin risk prediction.
van Gemert MJC, Ross MG, van den Wijngaard JPHM, Nikkels PGJ
Birth Defects Res 2022 Mar;114(5-6):149-164. Epub 2021 Dec 20 doi: 10.1002/bdr2.1976. PMID: 34931489Free PMC Article
ACR Appropriateness Criteria(®) Multiple Gestations.
Expert Panel on Women’s Imaging:, Glanc P, Nyberg DA, Khati NJ, Deshmukh SP, Dudiak KM, Henrichsen TL, Poder L, Shipp TD, Simpson L, Weber TM, Zelop CM
J Am Coll Radiol 2017 Nov;14(11S):S476-S489. doi: 10.1016/j.jacr.2017.08.051. PMID: 29101986
Prenatal diagnosis and outcome of multiple pregnancies with reversed arterial perfusion (TRAP-sequence).
Hartge DR, Weichert J
Arch Gynecol Obstet 2012 Jul;286(1):81-8. Epub 2012 Mar 6 doi: 10.1007/s00404-012-2283-9. PMID: 22392490
Twin-reversed arterial perfusion sequence: pre- and postoperative cardiovascular findings in the 'pump' twin.
Kinsel-Ziter ML, Cnota JF, Crombleholme TM, Michelfelder EC
Ultrasound Obstet Gynecol 2009 Nov;34(5):550-5. doi: 10.1002/uog.6431. PMID: 19780067
Epidemiology of acephalus/acardius monozygotic twins: new insights into an epigenetic causal hypothesis.
Martínez-Frías ML; ECEMC Working Group
Am J Med Genet A 2009 Feb 15;149A(4):640-9. doi: 10.1002/ajmg.a.32741. PMID: 19291778

Diagnosis

Prenatal diagnosis and outcome of multiple pregnancies with reversed arterial perfusion (TRAP-sequence).
Hartge DR, Weichert J
Arch Gynecol Obstet 2012 Jul;286(1):81-8. Epub 2012 Mar 6 doi: 10.1007/s00404-012-2283-9. PMID: 22392490
An acardiac twin with advanced brain development and a minor form of holoprosencephaly and intracerebral retina-like pigmented tissue: a case report and review of the literature.
Huss S, Fronhoffs F, Gembruch U, Loeffler K, Born M, Gessi M, Kuchelmeister K, Müller AM
Pediatr Dev Pathol 2011 Sep-Oct;14(5):411-7. Epub 2011 Apr 13 doi: 10.2350/10-09-0916-CR.1. PMID: 21488711
From monster to twin reversed arterial perfusion: a history of acardiac twins.
Obladen M
J Perinat Med 2010 May;38(3):247-53. doi: 10.1515/jpm.2010.043. PMID: 20121538
Sonographic diagnosis of placental teratoma.
Ahmed N, Kale V, Thakkar H, Hanchate V, Dhargalkar P
J Clin Ultrasound 2004 Feb;32(2):98-101. doi: 10.1002/jcu.10231. PMID: 14750143
Placental teratoma or acardius amorphus with amniotic band syndrome.
Calame JJ, van der Harten JJ
Eur J Obstet Gynecol Reprod Biol 1985 Oct;20(4):265-73. doi: 10.1016/0028-2243(85)90075-9. PMID: 4054420

Therapy

Maternal coagulopathy after umbilical cord occlusion for twin reversed arterial perfusion sequence.
Novak CM, Patel SV, Baschat AA, Hickey KW, Petersen SM
Obstet Gynecol 2013 Aug;122(2 Pt 2):498-500. doi: 10.1097/AOG.0b013e318295c8b9. PMID: 23884274
Are recommended phototherapy thresholds safe enough for extremely low birth weight (ELBW) infants? A report on 2 ELBW infants with kernicterus despite only moderate hyperbilirubinemia.
Moll M, Goelz R, Naegele T, Wilke M, Poets CF
Neonatology 2011;99(2):90-4. Epub 2010 Jul 17 doi: 10.1159/000302719. PMID: 20639681
Effects of ischaemia and hypoxia on the development of the nervous system in acardiac foetus.
Laure-Kamionowska M, Maślińska D, Deregowski K, Piekarski P, Raczkowska B
Folia Neuropathol 2004;42(4):227-33. PMID: 15679041
A monozygotic twin pregnancy discordant for acardia and X-inactivation pattern.
Masuzaki H, Miura K, Yoshimura S, Yoshiura K, Ishimaru T
Eur J Obstet Gynecol Reprod Biol 2004 Nov 10;117(1):102-4. doi: 10.1016/j.ejogrb.2003.12.028. PMID: 15474253
Acardius acephalus after induced ovulation: a case report.
Haring DA, Cornel MC, van der Linden JC, van Vugt JM, Kwee ML
Teratology 1993 Apr;47(4):257-62. doi: 10.1002/tera.1420470402. PMID: 8322219

Prognosis

Hypothesized pathogenesis of acardius acephalus, acormus, amorphus, anceps, acardiac edema, single umbilical artery, and pump twin risk prediction.
van Gemert MJC, Ross MG, van den Wijngaard JPHM, Nikkels PGJ
Birth Defects Res 2022 Mar;114(5-6):149-164. Epub 2021 Dec 20 doi: 10.1002/bdr2.1976. PMID: 34931489Free PMC Article
Prenatal diagnosis and outcome of multiple pregnancies with reversed arterial perfusion (TRAP-sequence).
Hartge DR, Weichert J
Arch Gynecol Obstet 2012 Jul;286(1):81-8. Epub 2012 Mar 6 doi: 10.1007/s00404-012-2283-9. PMID: 22392490
Twin-reversed arterial perfusion sequence: pre- and postoperative cardiovascular findings in the 'pump' twin.
Kinsel-Ziter ML, Cnota JF, Crombleholme TM, Michelfelder EC
Ultrasound Obstet Gynecol 2009 Nov;34(5):550-5. doi: 10.1002/uog.6431. PMID: 19780067
Mirror syndrome associated with hydropic acardius in triplet pregnancy.
Matsubara S, Ohmaru T, Ohkuchi A, Arai F, Kuwata T, Usui R, Takahashi K, Izumi A, Watanabe T, Suzuki M
Fetal Diagn Ther 2008;24(4):429-33. Epub 2008 Nov 13 doi: 10.1159/000171045. PMID: 19005259
Sonographic diagnosis of placental teratoma.
Ahmed N, Kale V, Thakkar H, Hanchate V, Dhargalkar P
J Clin Ultrasound 2004 Feb;32(2):98-101. doi: 10.1002/jcu.10231. PMID: 14750143

Clinical prediction guides

Hypothesized pathogenesis of acardius acephalus, acormus, amorphus, anceps, acardiac edema, single umbilical artery, and pump twin risk prediction.
van Gemert MJC, Ross MG, van den Wijngaard JPHM, Nikkels PGJ
Birth Defects Res 2022 Mar;114(5-6):149-164. Epub 2021 Dec 20 doi: 10.1002/bdr2.1976. PMID: 34931489Free PMC Article
Acardius anceps with neck cyst and cleft palate: Three dimensional skeletal computed tomography reconstruction with discussion of the literature.
van Gemert MJC, Streekstra GJ, Vandenbussche FPHA, Nikkels PGJ, van den Wijngaard JPHM
Birth Defects Res 2020 Jan 15;112(2):137-140. Epub 2019 Nov 27 doi: 10.1002/bdr2.1625. PMID: 31774253
Acardiac twinning: High resolution three-dimensional reconstruction of a low resistance case.
van Lier MG, Lopriore E, Vandenbussche FP, Streekstra GJ, Siebes M, Nikkels PG, Oepkes D, van Gemert MJ, van den Wijngaard JP
Birth Defects Res A Clin Mol Teratol 2016 Mar;106(3):213-7. Epub 2015 Dec 21 doi: 10.1002/bdra.23477. PMID: 26691208
Epidemiology of acephalus/acardius monozygotic twins: new insights into an epigenetic causal hypothesis.
Martínez-Frías ML; ECEMC Working Group
Am J Med Genet A 2009 Feb 15;149A(4):640-9. doi: 10.1002/ajmg.a.32741. PMID: 19291778
Mirror syndrome associated with hydropic acardius in triplet pregnancy.
Matsubara S, Ohmaru T, Ohkuchi A, Arai F, Kuwata T, Usui R, Takahashi K, Izumi A, Watanabe T, Suzuki M
Fetal Diagn Ther 2008;24(4):429-33. Epub 2008 Nov 13 doi: 10.1159/000171045. PMID: 19005259

Recent systematic reviews

Acardiac twin: a systematic review of minimally invasive treatment modalities.
Tan TY, Sepulveda W
Ultrasound Obstet Gynecol 2003 Oct;22(4):409-19. doi: 10.1002/uog.224. PMID: 14528480

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