Double-outlet right ventricle (DORV) is what Vierordt called partial transposition of the great arteries (TGA) in 1898, meaning that only the aorta was transposed from the left ventricle (LV) to the right ventricle (RV). Vierordt also renamed von Rokitansky’s anomalous transposition complete transposition, as opposed to his partial transposition. In 1923, Spitzer renamed Vierordt’s partial transposition simple transposition.
Around 1960, Dr. Jesse Edwards et al decided that more anatomically specific diagnostic terminology would be advantageous. So they introduced origin of both great arteries from the RV.
Eight years earlier, in 1952, Braun, de Vries, Feingold, Ehrenfeld, Feldman, and Schorr from Jerusalem, Israel wrote concerning a 19-year-old man with Down syndrome, “Thus the right ventricle serves as a ‘double outlet ventricle’. According to the classification of Lev and Volk, this malformation is a type of partial transposition of the Fallot type.” (Right. Lev and Volk were using Vierordt’s term and concept of partial transposition.) To the best of my knowledge, Braun et al in 1952 used double outlet referring to the RV for the first time.
In 1957, A. Calhoun Witham entitled his paper “Double Outlet Right Ventricle, A Partial Transposition Complex.” This is how DORV was introduced.
In the spring of 1984, Dr. Aldo Ricardo Castañeda asked me to make a presentation on DORV to the Society of Pediatric Cardiovascular Surgery that was going to meet at the Boston Children’s Hospital on May 5 and 6. My colleagues thought this was an excellent suggestion because almost nobody really understands DORV, not even the so-called experts! My colleagues were serious. They were saying, If you doubt that, just ask your favorite expert how many anatomic types of DORV exist? At this point, the expert is very likely to start mumbling, “Almost infinite . . . an impossible question . . . no one knows.”
My dear wife, Dr. Stella Van Praagh (who died in 2006), having studied DORV for years, used to say, “DORV is all of congenital heart disease. Each case must be diagnosed individually and completely, so that one can make the wisest decisions concerning management.” As the reader will see, what Dr. Stella said is literally true. Almost any form of congenital heart disease that one can imagine occurs with DORV. That is why DORV is so difficult to classify satisfactorily.
Definition and Differential Diagnosis
DORV is present when both great arteries are aligned entirely or predominantly with the morphologically RV. , Both great arteries do not arise from the right ventricular sinus. Instead, both great arteries arise from the infundibulum (or conus arteriosus), which may be a well-developed muscular structure or resorbed and merely an intervalvar fibrosa. In the normal heart, the subpulmonary infundibulum is a well-developed muscular structure, whereas the subaortic infundibular free wall has been resorbed by apoptosis (programmed cell death) and consequently is reduced to the aortic-mitral intervalvar fibrosa. Infundibulum means funnel (Latin). Conus means cone (Latin). Infundibulum is the inside look at the ventriculoarterial (VA) connecting segment, most often used in anatomic descriptions. Conus, or conus arteriosus, is the outside appearance of the VA connecting segment, often used in embryologic descriptions. I use infundibulum and conus interchangeably, but prefer infundibulum for anatomic description.
In DORV, the great arteries do not always arise above the RV. For example, in superoinferior ventricles with RV superior to the LV, DORV is the most common type of VA alignment, in our experience. The RV and the semilunar valves are almost in the same horizontal plane. The great arteries are often beside the RV, not above the RV. However, the great arteries are aligned predominantly or exclusively with the RV.
As far as differential diagnosis is concerned, we make the diagnosis of tetralogy of Fallot (TOF), for example, if there is aortic valve–mitral valve direct fibrous continuity. Even if there is a lot of aortic overriding, we do not call such cases DORV. Similarly, if there is pulmonary valve–mitral valve direct fibrous continuity, we call such cases transposition of the great arteries (TGA), not DORV, even if there is a lot of overriding of the pulmonary valve and artery.
Why? Because the degree of semilunar valvar overriding is a criterion that does not always work. In solitus normally related great arteries, for example, the aortic valve does not sit predominantly above the LV cavity. Instead, the normal aortic valve is mostly above the ventricular septum, to the right of the LV cavity. Similarly, the normally located pulmonary valve is not located predominantly above the RV cavity. Instead, the normal pulmonary valve is located predominantly to the left of the RV cavity, mostly above the infundibular septum. Consequently, we do not use the 50% rule, which does not apply well to normally related great arteries. This does not mean that we have given up on measuring the degree of overriding of the great arteries.
Instead, this means that we are interested in what aortic-mitral and pulmonary-mitral fibrous continuity really mean. Aortic-mitral fibrous continuity means that the normal embryonic aortic switch procedure has been performed more or less successfully. In turn, this also means that one should not make the diagnosis of DORV because DORV means that the embryonic aortic switch has not been done, hence DORV.
Pulmonary valve–mitral valve fibrous continuity means that the abnormal embryonic transposed pulmonary arterial switch has been performed. Hence, the diagnosis of DORV is not accurate. Thus, it is important to understand the biological meaning of these embryonic arterial switch procedures. If an embryonic arterial switch has been performed, DORV is no longer present.
Diagnoses should be specific and mutually exclusive. For example, the diagnosis should be TOF, or DORV, not TOF and DORV. Similarly, the diagnosis should be DORV or TGA, not DORV with (and) TGA. This is a basic principle of classification.
Findings
| DORV With Subpulmonary VSD (n = 11) | ||
| DORV {S,D,D} with bilateral infundibulum, subpulmonary infundibuloventricular VSD, and no outflow obstruction | 3 |
| DORV {S,D,D} with bilateral infundibulum, subpulmonary infundibuloventricular plus the AV canal–type VSD, and no outflow obstruction | 1 |
| DORV {S,D,D} with bilateral infundibulum, subpulmonary infundibuloventricular VSD, and AS | 3 |
| 4. | DORV {S,D,D} with bilateral infundibulum, subpulmonary infundibuloventricular plus AV canal type of VSD, and AS | 1 |
| DORV {S,D,D} with bilateral infundibulum, subpulmonary infundibuloventricular VSD, uncommitted muscular VSD, and AS | 2 |
| DORV {S,D,D} with subaortic infundibulum, subpulmonary infundibuloventricular VSD, and AS | 1 |
| DORV With Uncommitted VSD (n = 11) | ||
| DORV {S,D,D} with bilateral infundibulum, uncommitted infundibuloventricular VSD, and PS | 6 |
| DORV {S,D,D} with bilateral infundibulum, uncommitted infundibuloventricular VSD, AS, and PS | 1 |
| DORV {S,D,L} with bilateral infundibulum, uncommitted infundibuloventricular VSD, and PS | 1 |
| DORV {S,D,D} with subaortic infundibulum, uncommitted muscular VSD, and PS | 1 |
| DORV {S,L,L} with bilateral infundibulum, uncommitted infundibuloventricular VSD, and PS | 1 |
| DORV {S,L,D} with bilateral infundibulum, uncommitted infundibuloventricular VSD, and no outflow obstruction | 1 |
DORV with malformations mainly involving the infundibular and the great arterial segments ( Group 1 ) are summarized in Table 23.1 . There are 17 different anatomic types of DORV in Table 23.1 , involving about one-third of this series: 32 of 101 patients (32%). These are the least complex cases of DORV. As will soon be seen, there are two more complex groups of DORV:
Group II patients with DORV have malformations not only of the infundibulum and great arteries, but also of the atrioventricular (AV) valves and the ventricles (n = 42 cases).
Group III patients with DORV have anomalies of all five cardiac segments: viscera and atria, AV valves, ventricles, infundibulum, and great arteries (n = 27: asplenia 17 and polysplenia 10).
| Group I: DORV with anomalies of the infundibulum and great arteries only (n = 32/101 patients [32%]) | ||
| DORV With Subaortic VSD (n = 10) | ||
| DORV {S,D,D} with bilateral infundibulum, subaortic infundibuloventricular VSD, and no outflow tract stenosis | 1 |
| DORV {S,D,D} with bilateral infundibum, subaortic infundibuloventricular VSD, and PS | 5 |
| DORV {S,D,D} with bilateral infundibulum, subaortic infundibuloventricular VSD, and AS | 2 |
| DORV {S,D,L} with bilateral infundibulum, subaortic infundibuloventricular VSD and mild PS | 1 |
| DORV {S,D,L} with bilateral infundibulum, subaortic infundibuloventricular VSD, uncommitted muscular VSD, and AS | 1 |
| DORV With Subpulmonary VSD (5 of 42 Cases) | ||
| DORV {S,D,D} with subaortic infundibulum, subpulmonary infundibuloventricular VSD, no outflow obstruction, and straddling MV | 1 |
| DORV {S,D,D} with subaortic infundibulum, subpulmonary infundibuloventricular VSD, PS, and straddling MV | 2 |
| DORV {S,D,D} with subaortic infundibulum, subpulmonary infundibuloventricular VSD, uncommitted muscular VSD, and straddling MV | 1 |
| DORV {S,L,D} with subaortic infundibulum, subpulmonary infundibuloventricular VSD, PS, Ebstein anomaly of TV, and straddling MV | 1 |
| DORV With Uncommitted VSD (20 of 42 Cases) | ||
| DORV {S,D,D} with bilateral infundibulum, uncommitted muscular VSD, no outflow obstruction, MAt | 1 |
| DORV {S,D,D} with subaortic infundibulum, uncommitted VSD of the AV canal type, no outflow obstruction, MAt | 1 |
| DORV {S,D,D} with subaortic infundibulum, uncommitted muscular VSD, MAt, AS | 1 |
| DORV {S,D,D} with subpulmonary infundibulum, uncommitted muscular VSD, no outflow obstruction, MAt | 1 |
| DORV {I,D,D} with bilateral infundibulum, uncommitted VSD of the AV canal type, PS, and MAt | 1 |
| DORV {S,D,D} with bilateral infundibulum, uncommitted infundibuloventricular VSD, no outflow obstruction, straddling MV | 1 |
| DORV {S,D,L} with subaortic infundibulum, uncommitted infundibuloventricular VSD, PS, severe TS, and straddling MV | 1 |
| DORV {S,D,D) with subpulmonary infundibulum, uncommitted VSD of the AV canal type, AS, straddling TV | 1 |
| DORV {S,D,D} with bilateral infundibulum, uncommitted VSD of the AV canal type, PS, straddling TV, and MS | 1 |
| DORV {S,L,L} with subaortic infundibulum, uncommitted VSD of AV canal type, PS, double-inlet RV | 1 |
| DORV {S,D,D} with bilateral infundibulum, uncommitted VSD of the AV canal type, no outflow obstruction, MAt, and straddling TV | 1 |
| DORV {S,D,D} with bilateral infundibulum, uncommitted VSD of the AV canal type, common AV valve, no outflow obstruction | 1 |
| DORV {S,D,D} with subpulmonary infundibulum, uncommitted VSD of the AV canal type, common AV canal with MS, no outflow obstruction | 1 |
| DORV {S,D,D} with subpulmonary infundibulum, uncommitted VSD of the AV canal type, common AV canal, and PS | 1 |
| DORV {S,D,D} with subpulmonary infundibulum, uncommitted AV canal VSD, common AV canal with MS, and AS | 1 |
| DORV {S,L,L}, bilateral infundibulum, uncommitted AV canal VSD, common AV canal, PS | 1 |
| DORV {S,L,L} with subaortic infundibular, uncommitted AV canal VSD, common AV canal, AS | 1 |
| DORV {S,L,D} with subaortic infundibulum, uncommitted AV canal VSD, common AV canal, no outflow obstruction | 1 |
| DORV {I,D,D} with bilateral infundibulum, uncommitted AV canal VSD, common AV canal, and PS | 1 |
| DORV {I,D,L} with bilateral infundibulum, uncommitted AV canal VSD, common AV canal, PS | 1 |
| DORV With Intact Ventricular Septum (8 of 42 Cases) | ||
| DORV {S,D,D} with bilateral infundibulum, no VSD, PS, MAt | 3 |
| DORV {S,D,D} with subpulmonary infundibulum, no VSD, no outflow obstruction, MS | 1 |
| DORV {S,D,D} with subpulmonary infundibulum, no VSD, AS, MAt | 1 |
| DORV with subpulmonary infundibulum, no VSD, AS, absence of MV leaflets with small MV orifice, that is, MS | 1 |
| DORV {S,L,L} with bilateral infundibulum, no VSD, PS, MAt (R) | 1 |
| DORV {S,L,L} with bilateral infundibulum no VSD, PS, MS (R) | 1 |
Group I DORV contains malformations of one connecting segment and one main cardiac segment: infundibulum and great arteries, respectively.
Group II DORV contains anomalies of two connecting segments and two main segments: infundibulum and great arteries, and AV valves and ventricles.
Group III DORV contains malformations in all five cardiac segments: viscera and atria, AV valves, ventricles, infundibulum, and great arteries, because group III DORV involves the heterotaxy syndromes of congenital asplenia and polysplenia ( Table 23.2 ).
TABLE 23.2
Group III DORV With Asplenia and Uncommitted Ventral Septal Defect (13 of 17 Cases)
1.
DORV {A(I),L,A} with asplenia, bilateral infundibulum, uncommitted infundibuloventricular VSD, PS, AV valves divided
1
2.
DORV {A,D,D} with asplenia, bilateral infundibulum, uncommitted AV canal VSD, common AV canal (RV type), and PS
3
3.
DORV {A,D,D} with asplenia, bilateral infundibulum, uncommitted AV canal VSD, common AV canal (RV type), PAt
1
4.
DORV {A,D,D} with asplenia, bilateral infundibulum, uncommitted AV canal VSD, common AV canal (LV type)
2
5.
DORV {A,D,D} with asplenia, bilateral infundibulum, uncommitted AV canal VSD, common AV canal (balanced type), PS
1
6.
DORV {A,L,L} with asplenia, bilateral infundibulum, uncommitted AV canal VSD, common AV canal (LV type), PS
2
7.
DORV {A,L,L} with asplenia, bilateral infundibulum, uncommitted AV canal VSD, common AV canal (balanced), PS
1
8.
DORV {A,L,L} with asplenia, bilateral infundibulum, uncommitted AV canal VSD, common AV canal (balanced), PAt
1
9.
DORV {A,L,L} with asplenia, bilateral infundibulum, uncommitted AV canal VSD, common AV canal (RV type), PAt
1
AV, Atrioventricular; LV, left ventricle; PAt, pulmonary atresia; PS, pulmonary stenosis; RV, right ventricle; VSD, ventricular septal defect.
| DORV With Asplenia and No VSD (4 of 17 Cases) | ||
| DORV {A,D,D} with asplenia, bilateral infundibulum, no VSD, no outflow obstruction, common AV canal (RV type) | 1 |
| DORV {A,D,D} with asplenia, bilateral infundibulum, no VSD, PS, common AV canal (RV type) | 2 |
| DORV {A,L,L} with asplenia, bilateral infundibulum, no VSD, PS, common AV canal (RV type) | 1 |
| DORV With Polysplenia and Subaortic VSD (2 of 10 Cases) | ||
| DORV {A(S),D,D} with polysplenia, subpulmonary infundibulum, subaortic infundibuloventricular VSD, PS, and divided AV valves | 2 |
| DORV With Polysplenia and Uncommitted VSD (4 of 10 Cases) | ||
| DORV {A(S),D,D} with polysplenia, bilateral infundibulum, uncommitted infundibuloventricular VSD, PS, MV hypoplasia (MS) | 1 |
| DORV {A(S),D,D} with polysplenia, subpulmonary infundibulum, uncommitted VSD of the AV canal type, common AV canal (RV types), and AS | 2 |
| DORV {A(I),L,L} with polysplenia, subpulmonary infundibulum, uncommitted VSD of the AV canal type, common AV canal (balanced type), no outflow obstruction | 1 |
| DORV With Polysplenia and No VSD (4 of 10 Cases) | ||
| DORV {A(S),D,D} with polysplenia, subpulmonary infundibulum, no VSD, common AV canal (RV type), and AS | 1 |
| DORV {A(S),D,D} with polysplenia, subpulmonary infundibulum, no VSD, PS, common AV canal (RV type) | 1 |
| DORV {A(S),D,D} with polysplenia, subpulmonary infundibulum, no VSD, AS, absence of MV and LA | 1 |
| DORV {A(I),L,L} with polysplenia, subpulmonary infundibulum, no VSD, PS, common AV valve (RV type) | 1 |
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