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How should we diagnose and differentiate hearts with double-outlet right ventricle?

  • Tara Bharucha (a1), Anthony M. Hlavacek (a2), Diane E. Spicer (a3) (a4), Paraskevi Theocharis (a1) and Robert H. Anderson (a1) (a5)...

Abstract

Many, if not most, of the controversies regarding the description of the congenitally malformed heart have been resolved over the turn of the 20th century. A group of lesions that remains contentious is the situation in which both arterial trunks, in their greater part, are supported by the morphologically right ventricle. It was considered, for many years, that presence of bilateral infundibulums, or conuses, was a necessity for such a diagnosis. It has now been appreciated that this suggestion founders on many counts. In the first instance, such bilateral infundibulums are to be found in patients with other ventriculo-arterial connections, including the otherwise normal heart. In the second instance, it is clear that such an approach abrogates the important principle now known as the morphological method. This states that entities should be defined in terms of their intrinsic morphology and not on the basis of other variable features. It is now also clear that, when assessed simply on the basis of the ventricular origin of the arterial trunks, a significant number of patients fulfil the criteria for so-called “200%” origin of the trunks from the right ventricle when there is fibrous continuity between the leaflets of the atrioventricular and arterial valves. In this review, we show how attention to the morphology of the channel between the ventricles now provides the key to accurately diagnose the ventriculo-arterial connection in patients with suspected double-outlet right ventricle.

This is because, when both arterial trunks arise exclusively or predominantly from the morphologically right ventricle, the outlet septum, of necessity, is itself a right ventricular structure. The channel between the ventricles, therefore, is roofed by the inner heart curvature, whether that structure is fibrous or muscular. Our observations then confirm that it is the attachment of the outlet septum, which itself can be muscular or fibrous, which determines the commitment of the interventricular communication to the subarterial outlets. The interventricular communication itself, when directly committed to the ventricular outlets, opens between the limbs of the septomarginal trabeculation or septal band. The defect is subaortic when the outlet septum is attached to the cranial limb of the trabeculation, subpulmonary when attached to the caudal limb, and doubly committed when attached to the inner heart curvature in the roof of the defect. Non-committed defects are no longer positioned within the limbs of the septomarginal trabeculation. Although readily demonstrable by a skilled echocardiographer, we show how these anatomical features are more easily demonstrated with added accuracy when using CT data sets.

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Corresponding author

Correspondence to: Prof. R. H. Anderson, 60 Earlsfield Road, London SW18 3DN, United Kingdom, Tel: +00 44 20 8870 4368. E-mail: sejjran@ucl.ac.uk

References

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1. Becker, AE, Anderson, RH. How should we describe hearts in which the aorta is connected to the right ventricle and the pulmonary trunk to the left ventricle? A matter for reason and logic. Am J Cardiol 1983; 51: 911912.
2. Jacobs, ML, Anderson, RH. Nomenclature of the functionally univentricular heart. Cardiol Young 2006; 16 (Suppl 1): 38.
3. Witham, AC. Double outlet right ventricle; a partial transposition complex. Am Heart J 1957; 53: 928939.
4. Sridaromont, S, Ritter, DG, Feldt, RH, Davis, GD, Mcgoon, DC, Edwards, JE. Double outlet right ventricle. Anatomic and angiographic correlations. Mayo Clin Proc 1978; 53: 556563.
5. Baron, MG. Radiologic notes in cardiology. Angiographic differentiation between tetralogy of Fallot and double-outlet right ventricle. Relationship of the mitral and aortic valves. Circulation 1971; 43: 451455.
6. Fallot, A. Contribution a l’anatomie pathologique de la maladie bleue (cyanose cardiaque). Mars Med 1888; 25: 77403.
7. Van Praagh, R, David, I, Wright, GB, Van Praagh, S. Large RV plus small LV is not single LV. Circulation 1980; 61: 10571058.
8. Walters, HL, Mavroudis, C, Tchervenkov, CI, Jacobs, JP, Lacour-Gayet, F, Jacobs, ML. Congenital heart surgery nomenclature and database project: double outlet right ventricle. Ann Thorac Surg 2000; 69 (Suppl 4): S249S263.
9. Sidi, D, Vouhé, P, Ou, P. Transposition and malposition of the great arteries with ventricular septal defects. In: Moller JH, Hoffman JIE, eds. Pediatric Cardiovascular Medicine, 2nd edn. Wiley-Blackwell, Oxford, UK, 2012.
10. Bailliard, F, Spicer, DE, Mohun, TJ, Henry, GW, Anderson, RH. The problems that exist when considering the anatomic variability between the channels that permit interventricular shunting. Cardiol Young 2015; 25: 1528.
11. Anderson, RH, Brown, NA, Mohun, TJ. Insights regarding the normal and abnormal formation of the atrial and ventricular septal structures. Clin Anat 2016; 29: 290304.
12. Rosenquist, GC, Clark, EB, Sweeney, LJ, McAllister, HA. The normal spectrum of aortic and mitral valve discontinuity. Circulation 1976; 54: 298301.
13. Cavalle-Garrido, T, Bernasconi, A, Perrin, D, Anderson, RH. Hearts with concordant ventriculoarterial connections but parallel arterial trunks. Heart 2007; 93: 100106.
14. Hosseinpour, AR, Anderson, RH, Ho, SY. The anatomy of the septal perforating arteries in normal and congenitally malformed hearts. J Thorac Cardiovasc Surg 2001; 121: 10461052.
15. Hosseinpour, A-R, Jones, TJ, Barron, DJ, Brawn, WJ, Anderson, RH. An appreciation of the structural variability in the components of the ventricular outlets in congenitally malformed hearts. Eur J Cardio-thorac Surg 2007; 31: 888893.
16. Wilcox, BR, Ho, SY, Macartney, FJ, Becker, AE, Gerlis, LM, Anderson, RH. Surgical anatomy of double-outlet right ventricle with situs solitus and atrioventricular concordance. J Thorac Cardiovasc Surg 1981; 82: 405417.
17. Lacour-Gayet, F., Haun, C, Ntalakoura, K, et al. Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch. Eur J Cardiothroac Surg 2002; 21: 10421048.
18. Macartney, FJ, Rigby, ML, Anderson, RH, Stark, J, Silverman, NH. Double outlet right ventricle. Cross sectional echocardiographic findings, their anatomical explanation and surgical relevance. Br Heart J 1984; 52: 164177.
19. Stellin, G, Ho, SY, Anderson, RH, Zuberbuhler, JR, Siewers, RD. The surgical anatomy of double-outlet right ventricle with concordant atrioventricular connection and non-committed ventricular septal defect. J Thorac Cardiovasc Surg 1991; 102: 849855.
20. Devaney, EJ, Lee, T, Gelehrter, S, et al. Biventricular repair of atrioventricular septal defect with common atrioventricular valve and double-outlet right ventricle. Ann Thorac Surg 2010; 89: 537542.
21. Lev, M, Bharati, S, Meng, CC, Liberthson, RR, Paul, MH, Idriss, F. A concept of double-outlet right ventricle. J Thorac Cardiovasc Surg 1972; 64: 271281.
22. Stellin, G, Zuberbuhler, JR, Anderson, RH, Siewers, RD. The surgical anatomy of the Taussig-Bing malformation. J Thorac Cardiovasc Surg 1987; 93: 560569.
23. Lincoln, C, Anderson, RH, Shinebourne, EA, English, TAH, Wilkinson, JL. Double outlet right ventricle with L-malposition of the aorta. Br Heart J 1975; 37: 453463.
24. Lopez, L. Double outlet ventricle. In: Lai WML, Mertens LL, Geva T, Cohen MS, (eds). Echocardiography in Paediatric and Congenital Heart Disease: From Fetus to Adult. Wiley-Blackwell, Hoboken, New Jersey, 2009: 417438.
25. Mahle, WT, Martinez, R, Silverman, N, Cohen, MS, Anderson, RH. Anatomy, echocardiography, and surgical approach to double outlet right ventricle. Cardiol Young 2008; 18 (Suppl 3): 3951.
26. Smallhorn, JF. Double-outlet right ventricle: an echocardiographic approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2000; 3: 2033.
27. Pushparajah, K, Barlow, A, Tran, V-H, et al. A systematic three-dimensional echocardiographic approach to assist surgical planning in double outlet right ventricle. Echocardiography 2012; 30: 234238.
28. Garekar, S, Bharati, A, Chokhandre, M, et al. Clinical application and multidisciplinary assessment of three dimensional printing in double outlet right ventricle with remote ventricular septal defect. World J Ped Congen Heart Surg 2016; 7: 344350.
29. Riesenkampff, E, Rietdorf, U, Wolf, I, et al. The practical clinical value of three-dimensional models of complex congenitally malformed hearts. J Thorac Cardiovasc Surg 2009; 138: 571580.

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How should we diagnose and differentiate hearts with double-outlet right ventricle?

  • Tara Bharucha (a1), Anthony M. Hlavacek (a2), Diane E. Spicer (a3) (a4), Paraskevi Theocharis (a1) and Robert H. Anderson (a1) (a5)...

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