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J Thorac Cardiovasc Surg 1995;110:1152-1153
© 1995 Mosby, Inc.

LETTERS TO THE EDITOR

Biventricular repair in patients with left isomerism

Department of Cardiovascular and Thoracic Surgery
Tsuchiura Kyodo General Hospital
11-7 Manabe Shinmachi, Tsuchiura
Ibaraki 300, Japan

Toshikatsu Yagihara, MD, Hideki Uemura, MD

Department of Cardiovascular Surgery
National Cardiovascular Center
5-7-1 Fujishirodai, Suita
Osaka 565, Japan

Reply to the Editor:

We congratulate Carotti and colleagues on their successful surgical approach to patients with isomeric left appendages. In our experience, when considering options for reparative operations in patients having visceral heterotaxy, it is the abnormal systemic and pulmonary venous connections, as well as the extent of ventricular malformations associated with atrioventricular septal defect or double-inlet atrioventricular connections, that have been the major determinants of success. In this light, we agree with Dr. Carotti's group that it is essential to describe all morphologic features present by means of logical methods of analysis. We admit that our use of the term "corrected TGA" was morphologically inappropriate. Indeed, we subsequently excluded these cases from the material analyzed in our review of cases of congenitally corrected transposition in which the so-called double switch procedure was used. ¹ The malformed hearts with visceral heterotaxy should have been described as being physiologically similar but anatomically distinct from those with discordant atrioventricular and abnormal ventriculoarterial connections, the presence of isomeric appendages clearly ruling out the possibility of discordant connections across the atrioventricular junctions. We should remind our colleagues in Rome, nonetheless, that the series of patients on whom we did report simply reflected our own experience of hearts with isomeric left appendages in which we were able to achieve anatomic repair.

We should also remind them that, although sequential analysis is essential to provide a precise understanding of the cardiac morphology, they should be aware that the method proposed by Van Praagh is not the only approach, nor necessarily the most appropriate. Indeed, the exquisite brevity provided by Van Praaghian codifications do not always produce clarity. ² Thus, although Dr. Carotti andcolleagues recommend the use of the codifications {A,L,L} and {A,D,D}, these codes do not necessarily express the morphologic features with the precision that comparable codes such as {S,L,L} and {I,D,D} might. Neither exact atrial arrangement (situs) nor the patterns of venoatrial connections are precisely indicated by the letter A.In this regard, Dr. Carotti and colleagues must surely be aware that Dr. Van Praagh and his colleagues ^3,4 have recently suggested that all malformed hearts be classified, on the basis of venoatrial connections, into groups of either situs solitus or situs inversus. Dr. Van Praagh's group, therefore, might no longer recognize an ambiguous nature for atrial situs. The suggestion that all cases fall into usual or mirror-image groups, nonetheless, has not been borne out by our most recent experience. ⁵ On the basis of our findings, we consider it essential to take note of both the morphology of the atrial chambers, according to the pattern of the appendages, and the precise venoatrial connections. Only in this light can equally important features such as ventricular topology properly be interpreted.

When striving to achieve optimal surgical results, it can be important not only to recognize the morphology encapsulated by codes such as {A,D,S}, but also to take into account the more detailed features, such as the surgical anatomy of the coronary circulation and the precise structure of the atrioventricular valves. In this respect, our studies have shown these features to be markedly abnormal in the setting of visceral heterotaxy. ^6,7 The findings derived from these investigations, together with more precise diagnosis based on complete sequential segmental analysis, might promote still better surgical strategies and results in the future. We hesitate to attempt to state categorically at this time our precise indications for biventricular repair in patients with visceral heterotaxy.

References

1. Yagihara T, Kishimoto H, Kawashima T, et al. Double switch operation in cardiac anomalies with atrioventricular and ventriculoarterial discordance. J THORAC CARDIOVASC SURG 1994;107:351-8.[Abstract/Free Full Text]
   
2. Anderson RH. What is meant by tetralogy of Fallot {S,D,I}? Ann Thorac Surg 1995;59:562-4.[Free Full Text]
   
3. Van Praagh R, Van Praagh S. Atrial isomerism in the heterotaxy syndromes with asplenia, or polysplenia, or normally formed spleen: an erroneous concept. Am J Cardiol 1990;66:1504-6.[Medline]
   
4. Van Praagh S, Kreutzer J, Alday L, Van Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy, with emphasis on the diagnosis of the atrial situs: a study of 109 postmortem cases. In: Clark E, Takao A, eds. Developmental cardiology, morphogenesis and function. Mt. Kisco, New York: Futura, 1990:671-721.
   
5. Uemura H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH. Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. Ann Thorac Surg [In press].
   
6. Uemura H, Ho SY, Anderson RH, et al. The surgical anatomy of coronary venous return in hearts with isomeric atrial appendages. J THORAC CARDIOVASC SURG [In press].
   
7. Uemura H, Anderson RH, Ho SY, et al. Left ventricular structures in atrioventricular septal defect associated with isomerism of atrial appendages compared with similar features with usual atrial arrangement. . J THORAC CARDIOVASC SURG [In press].
   

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