Criss-cross heart or upstairs downstairs heart.

Criss-cross heart or ‘upstairs–downstairs’ heart.
Review by S.Gerboni

Lev and Rowlatt in 1961 described unusual arrangement of the cardiac inlets :
ventricular chambers arranged in a superoinferior fashion,
with the RV superiorly and the LV inferiorly located.
The term Criss-cross heart,was introduced by Anderson et al. in
1974, defined as crossing of the long axes of the AV valves.
Criss-cross heart is a rare abnormality,its frequency
is no more than eight per 1 000 000 and it accounts
for <0.1% of congenital heart defects.
The morphological spectrum of hearts with crossed
AV relationships includes a wide variety of segmental
arrangements. Usually, the venoatrial connections are
normal, and the AV connections are either concordant or
discordant (biventricular). The ventricular chambers are
arranged characteristically in a superoinferior fashion,
with the RV superiorly and the LV inferiorly located.
Most commonly the VA connection is DORV and
less often discordant. Associated anomalies include
RV hypoplasia, straddling and/or overriding of the
AV valves, and subaortic or subpulmonary stenosis.
Rarely, criss-cross hearts have been described with
an intact ventricular septum. Postnatal presentation
varies and may include cyanosis, heart failure or a
murmur, depending on the full sequential diagnosis and
associated abnormalities. The diagnosis can be established
by echocardiography, angiocardiography and magnetic
resonance imaging.

Key points

• It appears to be an isolated cardiac finding with no associated extracardiac
  or chromosomal abnormalities.

• usually have biventricular AV connections.
• criss-cross relationship with doubleinlet ventricles is rarely recognized.
• the diagnosis should be suspected when the parallel
  arrangement of the AV valves and ventricular inlets cannot
  be achieved, and the two valves are not easily visualized
  simultaneously.

• the clue to diagnosis is the inability
  to image flow across the two AV valves in the same
  plane or in the straight transverse cut through the fetal
  chest owing to the unusual superoinferior relationship
  of the ventricles, also called the ‘upstairs–downstairs’
  arrangement.

• to image the two valves and ventricles,
  the transducer must be tilted and a careful ‘sweep’
  (posteroinferior to anterosuperior) allows the operator
  to appreciate this rare spatial relationship of the AV
  valves, with axes of openings that are not in parallel,
  but across one another. Their imaging requires different
  ultrasound planes in order to demonstrate the twisted AV
  inflow tracts.

• if this spatial arrangement of the inlets is not
  appreciated during live scanning, the echocardiographic
  views may be misinterpreted, leading to an incorrect
  sequential diagnosis.

• color flow mapping can help in assessing the AV connection as this allows
  visualization of the relative direction of intracardiac blood flows and
  facilitates recognition of the crossover of the inflow
  streams.

• Surgical options vary according to the exact sequential
  segmental analysis and associated abnormalities. In
  general, however, a univentricular repair is required,
  owing to significant AV valve straddling, even with
  two good size ventricles.

• This is very important in prenatal counceling.

References

1. Lev M, Rowlatt UF. The pathologic anatomy of mixed
   levocardia. A review of thirteen cases of atrial or ventricular
   inversion with or without corrected transposition. Am J Cardiol
   1961; 8: 216–263.

2. Anderson RH, Shinebourne EA, Gerlis LM. Criss-cross atrioventricular
   relationships producing paradoxical atrioventricular
   concordance or discordance. Their significance to nomenclature
   of congenital heart disease. Circulation 1974; 50:
   176–180.

3. Abdullah M, Yoo SJ, Hornberger L. Fetal echocardiographic
   features of twisted atrioventricular connections. Cardiol Young
   2000; 10: 409–412.

4. Duncan WJ, Wong KK, Freedom RM. A criss-cross heart with
   twisted atrioventricular connections, ‘perfect streaming’, and
   double discordance. Pediatr Cardiol 2006; 27: 604–607.

5. Carminati M, Valsecchi O, Borghi A, Balduzzi A, Bande A,
   Crupi G, Ferrazzi P, Invernizzi G. Cross-sectional echocardiographic
   study of criss-cross hearts and superoinferior ventricles.
   Am J Cardiol 1987; 59: 114–118.

6. Fontes VF, de Souza JA, Pontes Junior SC. Criss-cross heart
   with intact ventricular septum. Int J Cardiol 1990; 26:
   382–385.

7. Alday LE, Juaneda E. Superoinferior ventricles with criss-cross
   atrioventricular connections and intact ventricular septum.
   Pediatr Cardiol 1993; 14: 238–241.

8. Robinson PJ, Kumpeng V, Macartney FJ. Cross sectional
   echocardiographic and angiocardiographic correlation in criss
   cross hearts. Br Heart J 1985; 54: 61–67.

9. Igarashi H, Kuramatsu T, Shiraishi H, Yanagisawa M. Crisscross
   heart evaluated by colour Doppler echocardiography
   and magnetic resonance imaging. Eur J Pediatr 1990; 149:
   523–525.

10. Danielson GK, Tabry IF, Ritter DG, Fulton RE. Surgical repair
    of criss-cross heart with straddling atrioventricular valve.
    J Thorac Cardiovasc Surg 1979; 77: 847–851.

11. N. NGEH*†, O. API*†, A. IASCI*†, S. Y. HO* and J. S. CARVALHO*†.
    Criss-cross heart: report of three cases with double-inlet
    ventricles diagnosed in utero – Ultr.Obstet Gynecol 2008; 31: 461–465.