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J Thorac Cardiovasc Surg 2003;126:1225-1226
© 2003 The American Association for Thoracic Surgery

Letter to the editor

Spiral pattern: universe, normal heart, and complex congenital defects

Bruno Marino, MDa, Antonio F. Corno, MD, FRCS, FETCSb

a Department of Pediatric Cardiology, Institute of Pediatrics, University La Sapienza, Rome, Italy
b Department of Cardiovascular Surgery, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland

To the Editor:

In the fascinating article "Basic Science Review: The Helix and the Heart"1 in a recent issue of Journal, Gerald Buckberg, supported by the anatomic studies of Torrent-Guasp and colleagues,2 illustrated the spiral anatomy of the muscle bands and the helicoid shape of the myocardium. On the basis of recent observations on embryology and genetics, we have reached similar conclusions regarding the spiral pattern of the heart morphology in complex congenital defects.3

The normal heart has a clockwise spiral pattern of the outflow tracts and of the great arteries. The helicoid 3-dimensional movement driving to this condition, genetically determined in subjects with situs solitus, appears with the dextroventricular loop (right ventricle to the right, left ventricle to the left),4 progresses with the looping (anterior rotation of the right ventricle, posterior rotation of the left ventricle), and finishes with the spiral septations of the outflow tracts and the great arteries.5 Because the dextroventricular loop is the first recognized sign of asymmetry and lateralization of the body organs,4 the clockwise spiralization of the heart should be considered a specific pattern of chirality in vertebrates. In subjects with situs inversus, an incompletely understood genetic mechanism6 is responsible for a "mirror image" anatomy of the heart, with counterclockwise shape of the outflow tracts and great arteries. This condition appears with a levoventricular loop (right ventricle to the left, left ventricle to the right), progresses with an inverted looping, and finishes with an inverted spiral septation of the outflow tracts and the great arteries. Therefore the spiral shape of the heart is the consequence of an asymmetric formation, and it derives from coordinated developmental mechanisms involving, in a 3-dimensional fashion, the segments of ventricles and great arteries. In contrast to patients with situs ambiguus the genetically determined anomalies of lateralization6 cause, at the cardiac level, an interruption of the helicoid pattern consisting in isomerism of the atria, random ventricular loop, and transposition of the great arteries. This is particularly frequent in patients with right isomerism (asplenia syndrome).3 To summarize these observations, the helicoid shape of the heart is genetically determined and may be clockwise in situs solitus, counterclockwise in situs inversus, and absent or interrupted in situs ambiguus.

The helicoid shape of the heart is interrupted in other types of complex congenital heart defects. In congenitally corrected transposition of the great arteries [S,L,L], there is situs solitus of the atria, but with a levoventricular loop and a transposition of the great arteries. The resulting anatomic pattern is neither clockwise nor counterclockwise. In this complex malformation, the helicoid shape of the heart is inverted at ventricular level and interrupted at the level of the great arteries. In complete transposition of the great arteries [S,D,D], despite the presence of situs solitus of the atria and dextroventricular loop, the parallel position of the great arteries shows the interruption of the spiral pattern of the heart. Experimental research on animals,7 clinical studies on familial recurrence,8 and results of human genetic mutations9 suggest that both these complex cardiac defects, congenitally corrected transposition of the great arteries and complete transposition of great arteries, could be included in the pathogenetic group of the defects of lateralization.

In conclusion, the concept of a spiral pattern of the heart may have important implications not only for the fields of myocardial morphology, pathophysiology, and cardiac surgery1 but also for our understanding of the segmental morphogenetic mechanisms of some complex congenital heart defects. There is no doubt that the helicoid shape of the heart recalls some structural aspects of plants and animals, and also patterns observed in the microcosm and macrocosm alike.1,3 The physic and genetic causes of these similarities will advance our efforts toward comprehension, if we will be able to maintain the attitude of the student.1


   References
Top
 References
 

  1. Buckberg GD. Basic science review: The helix and the heart. J Thorac Cardiovasc Surg. 2002;124:863–883[Free Full Text]
  2. Torrent-Guasp F, Ballester M, Buckberg GD, Carreras F, Flotats A, Carrio I, et al. Spatial orientation of the ventricular muscle band: physiologic contribution and surgical implications. J Thorac Cardiovasc Surg. 2001;122:389–392[Free Full Text]
  3. Marino B. Transposition of the great arteries in asplenia and polysplenia phenotypes. Am J Med Genet. 2002;110:292–294[Medline]
  4. Levine M, Johnson RL, Stern CD, Kehun M, Tabin C. A molecular pathway determining left-right asymmetry in chick embryogenesis. Cell. 1995;82:803–814[Medline]
  5. de la Cruz MV, Sanchez-Gomez C, Arteaga MM, Arguello C. Experimental study of the development of the truncus and the conus in the chick embryo. J Anat. 1977;123:661–686[Medline]
  6. Casey B. Two rights make a wrong: human left-right malformations. Hum Mol Genet. 1998;7:1565–1571[Abstract/Free Full Text]
  7. Yasui H, Morishima M, Nakazawa M, Aikawa E. Anomalous looping, atrioventricular cushion dysplasia, and unilateral ventricular hypoplasia in the mouse embryos with right isomerism induced by retinoic acid. Anat Rec. 1998;250:210–219[Medline]
  8. Digilio MC, Casey W, Toscano A, Calabrò R, Pacileo G, Marasini M, et al. Complete transposition of the great arteries: patterns of congenital heart disease in familial recurrence. Circulation. 2001;104:2809–2814[Abstract/Free Full Text]
  9. Goldmuntz E, Bamford R, Karkera JD, de la Cruz J, Roessler E, Muenke M. CFC1 mutations in patients with transposition of the great arteries and double-outlet right ventricle. Am J Hum Genet. 2002;70:776–780[Medline]



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