Clinical types of double outlet right ventricle (DORV)

Clinical types of double outlet right ventricle (DORV)

Double outlet right ventricle (DORV) had been divided into four clinical types earlier:

TOF like: Features resemble tetralogy of Fallot with subaortic ventricular septal defect (VSD) and pulmonary stenosis (PS): ECG shows right axis deviation.
TGA like: Subpulmonary VSD with or without PS: ECG shows left ventricular (LV) volume overload with right axis deviation.
VSD like: Subaortic VSD without PS: ECG shows LV volume overload and left axis deviation
Eisenmenger like: Subaortic VSD with pulmonary vascular obstructive disease

Society of Thoracic Surgeons (STS) and European Association of Cardiothoracic Surgery (EACTS) International Nomenclature defines four types of DORV based on the clinical presentation and treatment: VSD-type, Fallot-type, TGA-type (Taussig-Bing), and DORV non-committed VSD (remote VSD) [1,2].

VSD type present with clinical signs of unrestrictive ventricular septal defect. In VSD type, the VSD is subaortic.  Fallot type has either a subaortic or double committed VSD and pulmonary outflow stenosis. TGA-type (Taussig-Bing) present in new born period like transposition of great arteries. It has a subpulmonary VSD and the pulmonary artery does not arise 100% from the right ventricle. In DORV non-committed VSD or remote VSD type, the VSD is not related to either great vessel and can extend from perimembranous region to the inlet or trabecular region. The VSD is separated by muscle of size more than the aortic diameter from the great vessels [3]. Double conus is constantly present in this type and both great vessels arise fully from the right ventricle [4].

Eight echocardiographic types have been suggested by Pang KJ et al. The classification was based on 3 factors: the relative position of great arteries, whether it was normal or abnormal; the relationship between great arteries and the VSD, whether committed or non committed; and the presence or absence or right ventricular outflow tract obstruction [5]. They named types I, II, III and IV with subtypes A and B, making a total of 8 types. This classification was called Modified Fuwai Classification after the name of their hospital.

References

  1. Lacour-Gayet F, Maruszewski B, Mavroudis C, Jacobs JP, Elliott MJ. Presentation of the International Nomenclature for Congenital Heart Surgery. The long way from nomenclature to collection of validated data at the EACTS. Eur J Cardiothorac Surg. 2000 Aug;18(2):128-35.
  2. Artrip JH, Sauer H, Campbell DN, Mitchell MB, Haun C, Almodovar MC, Hraska V, Lacour-Gayet F. Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification. Eur J Cardiothorac Surg. 2006 Apr;29(4):545-50. 
  3. Belli E, Serraf A, Lacour-Gayet F, Hubler M, Zoghby J, Houyel L, Planche C. Double-outlet right ventricle with non-committed ventricular septal defect. Eur J Cardiothorac Surg. 1999 Jun;15(6):747-52. 
  4. Lacour-Gayet F, Haun C, Ntalakoura K, Belli E, Houyel L, Marcsek P, Wagner F, Weil J. Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch. Eur J Cardiothorac Surg. 2002 Jun;21(6):1042-8. 
  5. Pang KJ, Meng H, Hu SS, Wang H, Hsi D, Hua ZD, Pan XB, Li SJ. Echocardiographic Classification and Surgical Approaches to Double-Outlet Right Ventricle for Great Arteries Arising Almost Exclusively from the Right Ventricle. Tex Heart Inst J. 2017 Aug 1;44(4):245-251.