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Cardiac total anomalous pulmonary venous connection

MedGen UID:
866779
Concept ID:
C4021130
Anatomical Abnormality
Synonyms: Cardiac total anomalous pulmonary venous connexion; Total anomalous pulmonary venous connection, intracardiac; Type 2 total anomalous pulmonary venous connection
 
HPO: HP:0011720

Definition

Type 2 total anomalous pulmonary venous connection. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCardiac total anomalous pulmonary venous connection

Conditions with this feature

Meacham syndrome
MedGen UID:
373234
Concept ID:
C1837026
Disease or Syndrome
WT1 disorder is characterized by congenital/infantile or childhood onset of steroid-resistant nephrotic syndrome (SRNS), a progressive glomerulopathy that does not respond to standard steroid therapy. Additional common findings can include disorders of testicular development (with or without abnormalities of the external genitalia and/or müllerian structures) and Wilms tumor. Less common findings are congenital anomalies of the kidney and urinary tract (CAKUT) and gonadoblastoma. While various combinations of renal and other findings associated with a WT1 pathogenic variant were designated as certain syndromes in the past, those designations are now recognized to be part of a phenotypic continuum and are no longer clinically helpful.

Professional guidelines

PubMed

Fick TA, Scott DA, Lupo PJ, Weigand J, Morris SA
Cardiol Young 2022 Apr;32(4):550-557. Epub 2021 Jul 2 doi: 10.1017/S1047951121002535. PMID: 34210367Free PMC Article
Liufu R, Liu X, Liu T, Chen J, Wen S, Cen J, Zhuang J
Eur J Cardiothorac Surg 2021 May 8;59(5):959-966. doi: 10.1093/ejcts/ezaa470. PMID: 33491045
Bravo-Valenzuela NJM, Peixoto AB, Araujo Júnior E
J Clin Ultrasound 2021 Mar;49(3):240-247. Epub 2021 Jan 4 doi: 10.1002/jcu.22973. PMID: 33398887

Recent clinical studies

Etiology

Ebuoka N, Asai H, Kimura S, Tachibana T
Asian Cardiovasc Thorac Ann 2024 Jun;32(5):314-316. Epub 2024 Aug 16 doi: 10.1177/02184923241256408. PMID: 39149975
Shi G, Zhu F, Wen C, Yan Y, Zhang H, Zhu Z, Chen H
J Thorac Cardiovasc Surg 2023 Feb;165(2):449-459.e4. Epub 2022 Apr 19 doi: 10.1016/j.jtcvs.2022.03.036. PMID: 35570017
Imai Y, Baba K, Otsuki S, Kondo M, Eitoku T, Shigemitsu Y, Fukushima Y, Hirai K, Iwasaki T, Kanazawa T, Kotani Y, Kasahara S
Pediatr Cardiol 2022 Apr;43(4):756-763. Epub 2022 Jan 11 doi: 10.1007/s00246-021-02782-z. PMID: 35013751
Gumustas M, Donmez YN, Aykan HH, Demircin M, Karagoz T
Cardiol Young 2021 Nov;31(11):1853-1857. Epub 2021 May 12 doi: 10.1017/S1047951121001517. PMID: 33977891
Kilgore A, Lucas V, Collins T, Snyder CS
Catheter Cardiovasc Interv 2006 Oct;68(4):629-31. doi: 10.1002/ccd.20705. PMID: 16969875

Diagnosis

Gao J, Ou H, Xing H, Chen H, Huang Y, Cao F, Wang Y
Pediatr Radiol 2024 Jul;54(8):1399-1404. Epub 2024 May 16 doi: 10.1007/s00247-024-05938-0. PMID: 38750327
Yang L, Zhou L, Chen L
Medicine (Baltimore) 2021 Feb 26;100(8):e24912. doi: 10.1097/MD.0000000000024912. PMID: 33663125Free PMC Article
Perri G, Filippelli S, Kirk R, Hasan A, Griselli M
J Card Surg 2012 May;27(3):384-6. Epub 2012 Apr 12 doi: 10.1111/j.1540-8191.2012.01438.x. PMID: 22497294

Therapy

Ebuoka N, Asai H, Kimura S, Tachibana T
Asian Cardiovasc Thorac Ann 2024 Jun;32(5):314-316. Epub 2024 Aug 16 doi: 10.1177/02184923241256408. PMID: 39149975
Matsuhisa H, Oshima Y, Maruo A, Hasegawa T, Tanaka A, Noda R
Ann Thorac Surg 2013 Feb;95(2):729-30. doi: 10.1016/j.athoracsur.2012.09.080. PMID: 23336897

Prognosis

Gao J, Ou H, Xing H, Chen H, Huang Y, Cao F, Wang Y
Pediatr Radiol 2024 Jul;54(8):1399-1404. Epub 2024 May 16 doi: 10.1007/s00247-024-05938-0. PMID: 38750327
Shi G, Zhu F, Wen C, Yan Y, Zhang H, Zhu Z, Chen H
J Thorac Cardiovasc Surg 2023 Feb;165(2):449-459.e4. Epub 2022 Apr 19 doi: 10.1016/j.jtcvs.2022.03.036. PMID: 35570017
Imai Y, Baba K, Otsuki S, Kondo M, Eitoku T, Shigemitsu Y, Fukushima Y, Hirai K, Iwasaki T, Kanazawa T, Kotani Y, Kasahara S
Pediatr Cardiol 2022 Apr;43(4):756-763. Epub 2022 Jan 11 doi: 10.1007/s00246-021-02782-z. PMID: 35013751
Kilgore A, Lucas V, Collins T, Snyder CS
Catheter Cardiovasc Interv 2006 Oct;68(4):629-31. doi: 10.1002/ccd.20705. PMID: 16969875

Clinical prediction guides

Shi G, Zhu F, Wen C, Yan Y, Zhang H, Zhu Z, Chen H
J Thorac Cardiovasc Surg 2023 Feb;165(2):449-459.e4. Epub 2022 Apr 19 doi: 10.1016/j.jtcvs.2022.03.036. PMID: 35570017
Gumustas M, Donmez YN, Aykan HH, Demircin M, Karagoz T
Cardiol Young 2021 Nov;31(11):1853-1857. Epub 2021 May 12 doi: 10.1017/S1047951121001517. PMID: 33977891

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