‘A twist in the heart’ – Echocardiographic diagnosis of criss-cross heart

Open ArchivePublished:May 01, 2015DOI:https://doi.org/10.1016/j.jccase.2015.04.004

      Abstract

      Criss-cross heart is an extremely rare congenital anomaly seen in less than 0.1% of all congenital heart diseases. We present a case of criss-cross heart in a 13-year-old boy. Suspicion of criss-cross heart occurred when all the four chambers of the heart could not be profiled in a single four-chamber view and a vertical septum was noticed in parasternal short-axis view. In this patient there was a ventricular septal defect along with this anomaly. Criss-cross heart usually presents with atrioventricular concordance and ventriculoarterial discordance. This case is unique and rare with atrioventricular and ventriculoarterial concordance.
      <Learning objective: Suspicion of criss-cross heart is made when it is not possible to visualize all the four cardiac chambers in the apical four-chamber view. Crossing of inlets is seen when posterior to anterior sweep is made by probe. Criss-cross heart throws light onto the concept of situs concordance and alignment. There is no exact mechanism described for this condition. One of the proposed mechanisms by Anderson et al. is the concept of post-septational rotational abnormality which suggests further rotation of heart post looping to decide the position of the apex.>

      Keywords

      Introduction

      Criss-cross is a rare congenital anomaly of the heart first described by Lev and Rowlatt in 1961 [
      • Lev M.
      • Rowlatt U.F.
      The pathologic anatomy of mixed levocardia. A review of thirteen cases of atrial or ventricular inversion with or without corrected transposition.
      ]. A total of 150 cases have been reported to date. There are no definite developmental mechanisms. One of the proposed mechanisms given by Anderson et al. is a result of clockwise or counter-clockwise post-septational rotation of the heart [
      • Anderson R.H.
      • Shinebourne E.A.
      • Gerlis L.M.
      Criss-cross atrioventricular relationships producing paradoxical atrioventricular concordance or discordance. Their significance to nomenclature of congenital heart disease.
      ]. Atrioventricular concordance and ventriculoarterial discordance are seen in 80% of cases. A ventricular septal defect is seen almost all the time. Other anomalies such as pulmonary stenosis, straddling of atrioventricular valves, tricuspid atresia, double outlet right ventricle, and congenitally corrected transposition are seen [
      • Anderson R.H.
      • Shinebourne E.A.
      • Gerlis L.M.
      Criss-cross atrioventricular relationships producing paradoxical atrioventricular concordance or discordance. Their significance to nomenclature of congenital heart disease.
      ].

      Case report

      A 13-year-old boy presented to us with dyspnea on exertion. During echocardiographic examination, it was not possible to image all the four cardiac chambers and the two atrioventricular valves in a single plane simultaneously (Fig. 1A ). Morphologic right ventricle was identified by its moderator band which was on the left side in relation to the left atrium. Left ventricle was identified by its smooth morphology and was situated on the right side. The two atrioventricular inlets were on different planes with one inlet crossing in front of the other (Fig. 1C and E). Color flow images confirmed crossing of atrioventricular inlets (Fig. 1D and F). Normal great arterial relationship was noted. There was a large perimembranous ventricular septal defect shunting from left to right. The interventricular septum was vertical in its orientation (Fig. 1B). A diagnosis of criss-cross heart with situs solitus and concordant atrioventricular and ventriculoarterial connections was made.
      Figure thumbnail gr1
      Fig. 1(A) Criss-cross heart illustration. (B) Vertical septum in criss-cross heart. (C) Echocardiogram image of left atrioventricular inflow. (D) Left atrioventricular inflow color Doppler image. (E) Echocardiogram image of right atrioventricular inflow. (F) Right atrioventricular inflow color Doppler image. LV, left ventricle; RA, right atrium; RV, right ventricle.

      Discussion

      Criss-cross heart is an extremely rare anomaly, constituting less than 0.1% of all the congenital heart diseases. Post-septational rotation of the ventricular mass along its long axis around the base of the heart during the early embryonic period was postulated as the possible mechanism for the genesis of criss-cross heart. Another rotation process post looping is seen to decide the location of the apex [
      • Anderson R.H.
      • Shinebourne E.A.
      • Gerlis L.M.
      Criss-cross atrioventricular relationships producing paradoxical atrioventricular concordance or discordance. Their significance to nomenclature of congenital heart disease.
      ,
      • Van Praagh R.
      • Weinberg P.M.
      • Van Praagh S.
      Malpositions of heart.
      ]. Concordant atrioventricular and discordant ventriculoarterial connections are found in around 80% of cases [
      • Muneer P.K.
      • Kalathingathodika S.
      • Chakanalil G.S.
      • Sony M.M.
      Crisscross heart with dextrocardia and intact interventricular septum.
      ]. Criss-cross heart with concordant atrioventricular connection occurs due to the clockwise twisting of ventricular mass around its long axis. In a criss-cross heart with discordant atrioventricular connection, counter-clockwise twisting of ventricular mass around its long axis occurs. Intact ventricular septum is rarely seen in criss-cross heart [
      • Sato K.
      • Ohara S.
      • Tsukaguchi I.
      • Yasui K.
      • Nakada T.
      • Tamai M.
      • Kobayashi Y.
      • Kozuka T.
      A criss-cross heart with concordant atrioventriculo-arterial connections.
      ].
      Few case reports with atrioventricular and ventriculoarterial concordance among criss-cross hearts have been reported to date. Earlier reports were based on autopsy studies and angiography [
      • Muneer P.K.
      • Kalathingathodika S.
      • Chakanalil G.S.
      • Sony M.M.
      Crisscross heart with dextrocardia and intact interventricular septum.
      ]. Magnetic resonance imaging is useful when echocardiogram cannot confirm the diagnosis [
      • Araoz P.A.
      • Reddy G.P.
      • Thomson P.D.
      • Higgins C.B.
      Images in cardiovascular medicine. Magnetic resonance angiography of criss-cross heart.
      ].
      Management depends upon the associated anomalies. A small right ventricle and pulmonary stenosis may require a staged Fontan repair. In other cases with simple ventricular septal defect or patent ductus arteriosus alone may just require closure of the defects. In case of isolated transposition of great vessels, arterial switch operation may be the only requirement [
      • Nakada I.
      • Nakamura T.
      • Matsumoto H.
      • Sezaki T.
      Successful repair of criss-cross heart by modified Fontan operation.
      ].

      Conflict of interest

      None declared.

      Funding

      None.

      Appendix A. Supplementary data

      The following are the supplementary data to this article:

      References

        • Lev M.
        • Rowlatt U.F.
        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: 216-223
        • Anderson R.H.
        • Shinebourne E.A.
        • Gerlis L.M.
        Criss-cross atrioventricular relationships producing paradoxical atrioventricular concordance or discordance. Their significance to nomenclature of congenital heart disease.
        Circulation. 1974; 50: 176-180
        • Van Praagh R.
        • Weinberg P.M.
        • Van Praagh S.
        Malpositions of heart.
        in: Moss A.J. Adams F.H. Emmanouilides E. Heart disease in infants, children and adolescents. 2nd ed. Williams and Wilkins, Baltimore1977: 395-399
        • Muneer P.K.
        • Kalathingathodika S.
        • Chakanalil G.S.
        • Sony M.M.
        Crisscross heart with dextrocardia and intact interventricular septum.
        Ann Pediatr Cardiol. 2014; 7: 70-71
        • Sato K.
        • Ohara S.
        • Tsukaguchi I.
        • Yasui K.
        • Nakada T.
        • Tamai M.
        • Kobayashi Y.
        • Kozuka T.
        A criss-cross heart with concordant atrioventriculo-arterial connections.
        Circulation. 1978; 57: 396-400
        • Araoz P.A.
        • Reddy G.P.
        • Thomson P.D.
        • Higgins C.B.
        Images in cardiovascular medicine. Magnetic resonance angiography of criss-cross heart.
        Circulation. 2002; 105: 537-538
        • Nakada I.
        • Nakamura T.
        • Matsumoto H.
        • Sezaki T.
        Successful repair of criss-cross heart by modified Fontan operation.
        Chest. 1983; 88: 569-570