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J Thorac Cardiovasc Surg 1995;110:1151-1152
© 1995 Mosby, Inc.

LETTERS TO THE EDITOR

Biventricular repair in patients with left isomerism

Adriano Carotti, MD, Bruno Marino, MD, Guido Oppido, MD, Carlo Marcelletti, MD

Department of Pediatric Cardiology and Cardiac Surgery
Ospedale Bambino Gesù
Piazza S. Onofrio, 4
Rome 00165, Italy

To the Editor:

We read with interest the article by Dr. Hirooka and associates, Go 1 "Biventricular Repair in Cardiac Isomerism," and we wish to congratulate the authors for their outstanding results.

In our experience with patients with right isomerism, none fulfilled the anatomic criteria to undergo biventricular repair. Go 2 Vice versa, 12 of 40 patients with left isomerism underwent biventricular repair. Go Go 3, 4 With reference to atrial isomerism with situs ambiguus, we think it more appropriate to categorize the cardiac morphology according to Van Praagh's sequential approach. Go 5 For example, in describing the cardiac anomalies of patients 5, 8, and 12, the authors cite "corrected TGA [transposition of the great arteries]" as the main diagnosis. We believe that such a definition is improper in patients with situs ambiguus, because it provides no information concerning the ventricular loop and the relationship between the great arteries. Along with Van Praagh's approach, if one combines the information gleaned from the author's GoTable I and Fig. 1 for patients with left isomerism, we conclude that {A,D,S} occurred in nine cases, {A,L,I} in two, {A,D,D} in two, and {A,L,L} in one.


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  		Table I. Anatomic features of 12 patients with left isomerism who underwent biventricular repair*
 
In our experience, {A,D,S} occurred in nine cases and {A,L,I} in three. At variance with the authors' experience, we have never found the {A,D,D} or {A,L,L} arrangements suitable for biventricular repair.

Our patients underwent biventricular repair at a mean age of 3.8 ± 8.1 years and a mean weight of 13 ± 16.8 kg. Anatomic features and surgical procedures are listed in GoTables I and GoII.


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  		Table II. Surgical procedures
 
One early death occurred as a result of pulmonary arterovenous fistulas. Go 6 On follow-up (4.6 ± 1.2 years), two patients died suddenly, probably of an arrhythmia. One patient required revision of an intraatrial baffle and three required a pacemaker implantation.

Furthermore, four additional patients with {A,D,S} (n = 2) and {A,L,I} (n = 2) anatomy, potentially suitable for biventricular repair, had various forms of systemic obstruction, namely coarctation of the aorta (n = 2), mitral hypoplasia (n = 1), and left ventricular hypoplasia associated with supravalvular mitral obstruction (n = 1). One of them underwent patch repair of isolated coarctation and survived. The three patients with associated cardiac anomalies died after palliative procedures.

We conclude that (1) anatomic features of patients with atrial isomerism are best described according to Van Praagh's sequential approach; (2) biventricular repair of cardiac abnormalities associated with left isomerism is possible in the presence of balanced ventricles and concordant ventriculoarterial connection, independently on the ventricular loop ({A,D,S}and {A,L,I}) Go Go 3, 4; and (3) the occurrence of systemic obstruction in left isomerism with either dextro or levo ventricular loop must be ruled out when a biventricular repair is being considered.

References

  1. Hirooka K, Yagihara T, Kishimoto H, et al. Biventricular repair in cardiac isomerism: report of seventeen cases. J THORAC CARDIOVASC SURG 1995;109:530-5.[Abstract/Free Full Text]
  2. Di Donato R, di Carlo D, Squitieri C, Rossi E, Ammirati A, Marino B, Marcelletti C. Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy. Ann Thorac Surg 1987;44:35-9.[Abstract]
  3. Di Donato R, Marino B, Carotti A, Squitieri C, Marcelletti C. Biventricular repair in left atrial isomerism (polysplenia syndrome). Circulation 1989;80(Suppl):II363.
  4. Vairo U, Marino B, Parretti Di Iulio D, et al. Morfologia ventricolo-infundibolare nell'eterotassia viscerale con isomerismo sinistro. Studio ECO-angiografico. G Ital Cardiol 1991;21:969-74.[Medline]
  5. Van Praagh R. Segmental approach to diagnosis. In: Fyler DC, ed. Nadas' pediatric cardiology. 1st ed. Philadelphia: Hanley & Belfus, 1992:27-35.
  6. Amodeo A, Di Donato R, Carotti A, Marino B, Marcelletti C. Pulmonary arteriovenous fistulas and polysplenia syndrome. J THORAC CARDIOVASC SURG 1994;107:1378-9.[Free Full Text]



This article has been cited by other articles:


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T.-J. Yun, O. O. Al-Radi, I. Adatia, C. A. Caldarone, J. G. Coles, W. G. Williams, J. Smallhorn, and G. S. Van Arsdell
Contemporary management of right atrial isomerism: Effect of evolving therapeutic strategies
J. Thorac. Cardiovasc. Surg., May 1, 2006; 131(5): 1108 - 1113.
[Abstract] [Full Text] [PDF]

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