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Superoinferior Ventricles


The malformation known as superoinferior (SI) ventricles is characterized typically by a superior morphologically right ventricle (RV), an inferior morphologically left ventricle (LV), and a relatively horizontal ventricular septum (VS). Rarely, the LV can be superior and the RV can be inferior.

Our Cardiac Pathology database indicates that we have seen and studied 22 cases of SI ventricles out of a total series of 3216 autopsied cases of congenital heart disease (0.68%). Of these 22 cases, 21 had a superior RV and an inferior LV (0.65% of the total series), whereas only 1 patient had a superior LV and an inferior RV (0.03%).

This chapter is based primarily on our 11 published cases. ,

  • Sex: Males 9, females 2; males 82%, females 18%; males-to-females = 9/2 (4.5/1). Thus, a strong male preponderance was found in this small series.

  • Age at Death: Mean, 982 days (2.69 years) ± 2572 days (7.05 years), minimum 7 days, maximum 23 10/12 years.

Segmental Anatomy

Double-outlet right ventricle (DORV) was the most common diagnosis, with 6 of 11 (54.55%). The variations in DORV segmental anatomy were: DORV {S,L,D} in 3; DORV {S,D,D} in 2; and DORV {S,L,L} in 1.

Transposition of the great arteries (TGA) was second in frequency, with 4 of 11 (36.36%). The variations in TGA segmental anatomy were: TGA {S,D,L} in 3 and TGA {S,L,L} in 1.

Solitus normally related great arteries with isolated ventricular inversion was the least frequent segmental anatomic set: {S,L,S} in 1 in 11 (9.09%).

These findings are summarized in Table 18.1 .

TABLE 18.1
Anatomic Types of Superoinferior Ventricles
Anatomic Types No. of Cases n = 11 % of Series ∗
  • 1.

    With DORV {S,L,D}

3 27
  • 2.

    With DORV {S,D,D}

2 18
  • 3.

    With DORV {S,L,L}

1 9
  • 4.

    With TGA {S,D,L}

3 27
  • 5.

    With TGA {S,L,L}

1 9
  • 6.

    With NRGA {S,L,S}

1 9

∗ Statistics rounded off to the nearest whole number.

Status of the Atrioventricular Valves

The atrioventricular (AV) valves were morphologically normal in only 3 of 11 cases (27%); all 3 patients had AV valves that were in situs solitus. The AV valves were malformed in 8 of these 11 cases of SI ventricles (73%). Specifically, the AV valvar anomalies were as follows:

  • The mitral valve (MV) overrode the VS in 2 patients. The MV was left-sided with a ventricular D-loop (solitus ventricular situs) in 1 case, and the MV was right-sided with a ventricular L-loop (inverted ventricular situs) in 1 case.

  • The MV (left-sided) was cleft in 1 patient.

  • The MV (right-sided) was atretic in 1 case.

  • The left-sided tricuspid valve (TV) obstructed the ventricular septal defect (VSD) in 1 patient with {S,L,S}.

  • A left-sided TV had Ebstein anomaly with tricuspid stenosis and tricuspid regurgitation in 1 case.

  • Both inverted AV valves overrode the VS in 1 patient.

Why is the frequency of AV valvar anomalies so high (73%) in these cases of SI ventricles? We think that the answer, at least in part, is because the ventricular malformations frequently result in significant ventriculoatrial malalignments.

Ventricular Situs.

In these 11 patients with SI ventricles, a ventricular D-loop (solitus organizational pattern) was present in 5 (45%) and a ventricular L-loop (inversus organizational pattern) was found in 6 (55%). So no predilection for either type of ventricular situs was apparent.

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