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Double inlet to single ventricle of indeterminate morphology

MedGen UID:
868890
•Concept ID:
C4023301
•
Anatomical Abnormality
HPO: HP:0011557

Definition

The condition in which both atria are joined to a single ventricle each by its own atrioventricular valve. The morphology of this ventricle does not allow one to determine if it corresponds to the left or right ventricle. [from HPO]

Professional guidelines

PubMed

Prenatal Diagnosis, Associated Findings, and Postnatal Outcome in Fetuses with Double Inlet Ventricle (DIV).
Böckenhoff P, Hellmund A, Gottschalk I, Berg C, Herberg U, Geipel A, Gembruch U
Ultraschall Med 2023 Oct;44(5):e226-e240. Epub 2022 Jul 1 doi: 10.1055/a-1866-4538. PMID: 35777369Free PMC Article

Recent clinical studies

Etiology

Prenatal Diagnosis, Associated Findings, and Postnatal Outcome in Fetuses with Double Inlet Ventricle (DIV).
Böckenhoff P, Hellmund A, Gottschalk I, Berg C, Herberg U, Geipel A, Gembruch U
Ultraschall Med 2023 Oct;44(5):e226-e240. Epub 2022 Jul 1 doi: 10.1055/a-1866-4538. PMID: 35777369Free PMC Article
Quantification of Initial Right Ventricular Dimensions by Computed Tomography in Infants with Congenital Heart Disease and a Hypoplastic Right Ventricle.
Goo HW
Korean J Radiol 2020 Feb;21(2):203-209. doi: 10.3348/kjr.2019.0662. PMID: 31997595Free PMC Article
Septation of the single ventricle: revisited.
Margossian RE, Solowiejczyk D, Bourlon F, Apfel H, Gersony WM, Hordof AJ, Quaegebeur J
J Thorac Cardiovasc Surg 2002 Sep;124(3):442-7. doi: 10.1067/mtc.2002.122299. PMID: 12202859
Orthotopic cardiac transplantation for the failing Fontan circulation.
Carey JA, Hamilton JR, Hilton CJ, Dark JH, Forty J, Parry G, Hasan A
Eur J Cardiothorac Surg 1998 Jul;14(1):7-13; discussion 13-4. doi: 10.1016/s1010-7940(98)00130-4. PMID: 9726608

Diagnosis

Prenatal Diagnosis, Associated Findings, and Postnatal Outcome in Fetuses with Double Inlet Ventricle (DIV).
Böckenhoff P, Hellmund A, Gottschalk I, Berg C, Herberg U, Geipel A, Gembruch U
Ultraschall Med 2023 Oct;44(5):e226-e240. Epub 2022 Jul 1 doi: 10.1055/a-1866-4538. PMID: 35777369Free PMC Article
Quantification of Initial Right Ventricular Dimensions by Computed Tomography in Infants with Congenital Heart Disease and a Hypoplastic Right Ventricle.
Goo HW
Korean J Radiol 2020 Feb;21(2):203-209. doi: 10.3348/kjr.2019.0662. PMID: 31997595Free PMC Article
Septation of the single ventricle: revisited.
Margossian RE, Solowiejczyk D, Bourlon F, Apfel H, Gersony WM, Hordof AJ, Quaegebeur J
J Thorac Cardiovasc Surg 2002 Sep;124(3):442-7. doi: 10.1067/mtc.2002.122299. PMID: 12202859
Echocardiographic spectrum of double inlet ventricle: evaluation of the interventricular communication.
Shiraishi H, Silverman NH
J Am Coll Cardiol 1990 May;15(6):1401-8. doi: 10.1016/s0735-1097(10)80031-2. PMID: 2329242
Cross-sectional echocardiographic diagnosis and subclassification of univentricular hearts: imaging studies of atrioventricular valves, septal structures and rudimentary outflow chambers.
Sahn DJ, Harder JR, Freedom RM, Duncan WJ, Rowe RD, Allen HD, Valdes-Cruz L, Goldberg SJ
Circulation 1982 Nov;66(5):1070-7. doi: 10.1161/01.cir.66.5.1070. PMID: 7127691

Prognosis

Prenatal Diagnosis, Associated Findings, and Postnatal Outcome in Fetuses with Double Inlet Ventricle (DIV).
Böckenhoff P, Hellmund A, Gottschalk I, Berg C, Herberg U, Geipel A, Gembruch U
Ultraschall Med 2023 Oct;44(5):e226-e240. Epub 2022 Jul 1 doi: 10.1055/a-1866-4538. PMID: 35777369Free PMC Article
Septation of the single ventricle: revisited.
Margossian RE, Solowiejczyk D, Bourlon F, Apfel H, Gersony WM, Hordof AJ, Quaegebeur J
J Thorac Cardiovasc Surg 2002 Sep;124(3):442-7. doi: 10.1067/mtc.2002.122299. PMID: 12202859
Orthotopic cardiac transplantation for the failing Fontan circulation.
Carey JA, Hamilton JR, Hilton CJ, Dark JH, Forty J, Parry G, Hasan A
Eur J Cardiothorac Surg 1998 Jul;14(1):7-13; discussion 13-4. doi: 10.1016/s1010-7940(98)00130-4. PMID: 9726608

Clinical prediction guides

Cross-sectional echocardiographic diagnosis and subclassification of univentricular hearts: imaging studies of atrioventricular valves, septal structures and rudimentary outflow chambers.
Sahn DJ, Harder JR, Freedom RM, Duncan WJ, Rowe RD, Allen HD, Valdes-Cruz L, Goldberg SJ
Circulation 1982 Nov;66(5):1070-7. doi: 10.1161/01.cir.66.5.1070. PMID: 7127691
Ventricular morphology in the univentricular heart.
Anderson RH, Tynan M, Freedom RM, Quero-Jiménez M, Macartney FJ, Shinebourne EA, Wilkinson JL, Becker AE
Herz 1979 Apr;4(2):184-97. PMID: 447181

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