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Hypercyanotic Spell

MedGen UID:
1713686
•Concept ID:
C5238741
•
Sign or Symptom

Definition

A clinical finding in which a patient has profound cyanosis associated with hyperpnea; classically associated with tetralogy of Fallot. (ACC-AHA) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHypercyanotic Spell

Professional guidelines

PubMed

Surgical repair of Tetralogy of Fallot following primary palliation: Right ventricular outflow track stenting versus modified Blalock-Taussig shunt.
Bigdelian H, Ghaderian M, Sedighi M
Indian Heart J 2018 Dec;70 Suppl 3(Suppl 3):S394-S398. Epub 2018 Jun 24 doi: 10.1016/j.ihj.2018.06.020. PMID: 30595296Free PMC Article
Management Issues in Intensive Care Units for Infants and Children with Heart Disease.
Iyer PU
Indian J Pediatr 2015 Dec;82(12):1164-71. Epub 2015 Nov 6 doi: 10.1007/s12098-015-1914-0. PMID: 26542311

Recent clinical studies

Etiology

Anaesthesia management for cleft lip in a child with unrepaired Tetralogy of Fallot in Malawi: a case report.
Pascal FNB, Anusa B, Chikumbanje S, Pollach G
Malawi Med J 2022 Sep;34(3):201-203. doi: 10.4314/mmj.v34i3.9. PMID: 36406090Free PMC Article
Surgical repair of Tetralogy of Fallot following primary palliation: Right ventricular outflow track stenting versus modified Blalock-Taussig shunt.
Bigdelian H, Ghaderian M, Sedighi M
Indian Heart J 2018 Dec;70 Suppl 3(Suppl 3):S394-S398. Epub 2018 Jun 24 doi: 10.1016/j.ihj.2018.06.020. PMID: 30595296Free PMC Article
Transcatheter stenting of the right ventricular outflow tract augments pulmonary arterial growth in symptomatic infants with right ventricular outflow tract obstruction and hypercyanotic spells.
McGovern E, Morgan CT, Oslizlok P, Kenny D, Walsh KP, McMahon CJ
Cardiol Young 2016 Oct;26(7):1260-5. Epub 2016 Mar 4 doi: 10.1017/S1047951115002231. PMID: 26939749
Complications of pediatric cardiac catheterization: 18-month study.
Tavli V, Kayhan B, Okur FF, Kirman M, Tekdoğan M
Turk J Pediatr 2000 Oct-Dec;42(4):294-7. PMID: 11196745

Diagnosis

Management Issues in Intensive Care Units for Infants and Children with Heart Disease.
Iyer PU
Indian J Pediatr 2015 Dec;82(12):1164-71. Epub 2015 Nov 6 doi: 10.1007/s12098-015-1914-0. PMID: 26542311
Intranasal midazolam for the emergency management of hypercyanotic spells in tetralogy of Fallot.
Montero JV, Nieto EM, Vallejo IR, Montero SV
Pediatr Emerg Care 2015 Apr;31(4):269-71. doi: 10.1097/PEC.0000000000000403. PMID: 25831027
Complications of pediatric cardiac catheterization: 18-month study.
Tavli V, Kayhan B, Okur FF, Kirman M, Tekdoğan M
Turk J Pediatr 2000 Oct-Dec;42(4):294-7. PMID: 11196745

Therapy

Intravenous prostaglandin E1 (alprostadil) bolus in ductus arteriosus-dependent CHD: valid or absolutely contraindicated?
Colín Ortiz JL, Cruz Hernández A, Silva Estrada JA, Maldonado Alonso R, Corona Villalobos CA, González Rebeles Guerrero C, Pérez Pérez LF
Cardiol Young 2024 Feb;34(2):314-318. Epub 2023 Jul 5 doi: 10.1017/S1047951123001403. PMID: 37403735
Management of a parturient with uncorrected tetralogy of Fallot in shock: an anesthetic challenge.
Bellapukonda S, Roncall BR, Mund M
BMJ Case Rep 2019 Dec 2;12(12) doi: 10.1136/bcr-2019-231517. PMID: 31796458Free PMC Article
Intranasal midazolam for the emergency management of hypercyanotic spells in tetralogy of Fallot.
Montero JV, Nieto EM, Vallejo IR, Montero SV
Pediatr Emerg Care 2015 Apr;31(4):269-71. doi: 10.1097/PEC.0000000000000403. PMID: 25831027
Treatment of tetralogy of Fallot hypoxic spell with intranasal fentanyl.
Tsze DS, Vitberg YM, Berezow J, Starc TJ, Dayan PS
Pediatrics 2014 Jul;134(1):e266-9. Epub 2014 Jun 16 doi: 10.1542/peds.2013-3183. PMID: 24936003
Disopyramide: a promising new approach to the medical treatment of the hypercyanotic spell complicating tetralogy of Fallot.
Tomita H, Fuse S, Hatakeyama K, Suzuki M, Chiba S
Jpn Circ J 1998 Nov;62(11):807-10. doi: 10.1253/jcj.62.807. PMID: 9856595

Prognosis

Surgical repair of Tetralogy of Fallot following primary palliation: Right ventricular outflow track stenting versus modified Blalock-Taussig shunt.
Bigdelian H, Ghaderian M, Sedighi M
Indian Heart J 2018 Dec;70 Suppl 3(Suppl 3):S394-S398. Epub 2018 Jun 24 doi: 10.1016/j.ihj.2018.06.020. PMID: 30595296Free PMC Article

Clinical prediction guides

Surgical repair of Tetralogy of Fallot following primary palliation: Right ventricular outflow track stenting versus modified Blalock-Taussig shunt.
Bigdelian H, Ghaderian M, Sedighi M
Indian Heart J 2018 Dec;70 Suppl 3(Suppl 3):S394-S398. Epub 2018 Jun 24 doi: 10.1016/j.ihj.2018.06.020. PMID: 30595296Free PMC Article
Transcatheter stenting of the right ventricular outflow tract augments pulmonary arterial growth in symptomatic infants with right ventricular outflow tract obstruction and hypercyanotic spells.
McGovern E, Morgan CT, Oslizlok P, Kenny D, Walsh KP, McMahon CJ
Cardiol Young 2016 Oct;26(7):1260-5. Epub 2016 Mar 4 doi: 10.1017/S1047951115002231. PMID: 26939749

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