|
|
||||||||
J Thorac Cardiovasc Surg 1995;109:530-535
© 1995 Mosby, Inc.
SURGERY FOR CONGENITAL HEART DISEASE |
Osaka, Japan
From the Division of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan.
Received for publication July 9, 1993. Accepted for publication August 26, 1994. Address for reprints: Kazunobu Hirooka, MD, Tsuchiura Kyodo General Hospital, Manabe-shinnmachi, 11 Tsuchiura, Ibaraki, 300, Japan.
Abstract
Ninety-three patients with cardiac isomerism were treated surgically from July 1985 to June 1991. Among them, three patients with right and 14 with left isomerism underwent biventricular repair. Ages ranged from 4 months to 41 years (mean 4.8 years). Anatomic repair was accomplished in 15 patients and functional repair with the right ventricle used as the systemic ventricle in two patients. Methods of atrial septation to separate pulmonary venous flow from systemic venous flow included atrial partition with a straight patch in seven patients, intraatrial rerouting with a tailored baffle in five, and a Mustard-type atrial switch in five. One hospital death (5.8%) and two late (12%) occurred. Two patients required reoperation (12%), one reconstruction of a stenotic systemic venous connection and one mitral valve replacement because of incompetence. Surgically induced complete atrioventricular block was not observed in any of the patients. Optimal atrial septation offers the possibility of biventricular repair for patients with acceptable intraventricular structure. (J THORACCARDIOVASCSURG1995; 109: 530-5)
Atrial isomerism is recognized as a cardiovascular anomaly associated with asplenia (right isomerism) and polysplenia (left isomerism)
1-6 It has been reported that 79% of patients with right isomerism and 61% with left isomerism die of cardiac failure or anoxia by the end of their first year.
4 Naturally, surgical treatment is complicated by the extent and variability of intracardiac anomalies. Here we report information on 17 patients with cardiac isomerism who underwent biventricular repair.
PATIENTS AND METHODS
Ninety-three patients with cardiac isomerism underwent surgical treatment between July 1985 and June 1991 at the National Cardiovascular Center. Three of 61 patients with right isomerism (5%) and 14 of 32 patients with left isomerism (44%) underwent biventricular repair. The mean age of these 17 patients at operation was 4.8 years.
Isomerism status was determined before the operation on the basis of identification of thoracic isomerism, and the structure of the atrial appendages was identified during the operation. The thoracic situs was determined on the basis of the relationship between the bronchi and pulmonary arteries. For example, short eparterial bronchi on plain chest radiograph or angiogram was diagnosed as right isomerism and long hyparterial bronchi as left isomerism.
7-9
Morphologic findings are summarized in
Table I. Among those with right isomerism, patients 2 and 3 had a double-outlet right ventricle; the ventricular septal defect (VSD) was noncommitted in one and subaortic in the other. Patient 2 had a common atrium and patients 1 and 3 had an atrioventricular septal defect (AVSD). Patients 2 and 3 had infundibular pulmonary stenosis and patient 1 had a stenotic bicuspid pulmonary valve. Total anomalous pulmonary venous connections were present in two patients.
|
Operations were performed with extracorporeal circulation, moderate hypothermia, and cold chemical cardioplegic arrest. A crystalloid cardioplegic solution was given at an initial dose of 10 ml/kg and then at a dose of 5 ml/kg every 30 minutes. All surgical shunts were ligated at the beginning of bypass. Extracorporeal circulation time ranged from 85 to 379 minutes (mean 238 minutes) and aortic crossclamping time from 38 to 171 minutes (mean 119 minutes).
Right isomerism
Patient 1 had TGA combined with pulmonary stenosis and an intact ventricular septum; therefore he needed the right ventricle to serve as the systemic ventricle. Atrial septation was performed according to the method of Mustard. Equine pericardium was used for a pantaloon-shaped intraatrial baffle and for an atriotomy patch to increase the volume of the newly constructed left atrium. Concomitantly, a prosthetic valve for an incompetent right-sided atrioventricular (AV) valve was replaced. In two patients with double-outlet right ventricle, an internal conduit of duplicated equine pericardium was patched to allow drainage of blood from the left ventricle through the VSD and into the aorta. In patient 2, the noncommitted type VSD was enlarged by resecting the muscle bar between the VSD and the aortic valve, where no tricuspid subvalvular component was attached. Because patient 2 had a single coronary artery whose branch ran across the right ventricular outflow, an external conduit repair from the right ventricle to the pulmonary artery was required. In patient 3, intraatrial rerouting was performed according to the two-patch method with a tailored baffle after total anomalous pulmonary venous connection was repaired by anastomosing the common pulmonary vein to the common atrium followed by ligation of the vertical vein (Fig. 1).
|
In eight patients with incomplete AVSD, atrial septal defect or common atrium was corrected with a single patch by moderate atrial septation. The two-patch method, which uses separate patches for septation of the atrium and closure of the VSD, was used in three patients with complete AVSD. Two patients with corrected TGA underwent a double switch operation, which was a combination of the Mustard operation applied as an atrial switch and of an internal conduit repair from the left ventricle through the VSD to the aorta as a ventriculoarterial switch. Another patient with corrected TGA underwent external conduit repair from the left ventricle to the pulmonary artery, with the right ventricle used as the systemic ventricle, because the atrial septum was shaped well and venous drainage was separated as in a situs inversus atrium. Obstruction of the right ventricular outflow tract in patients 6 and 9 was relieved by inserting a transannular patch with a monocusp.
In the presence of bilateral superior venae cavae, the vein without an azygos or hemiazygos connection was ligated except when the superior vena caval pressure rose more than 15 mm Hg after clamping of the ipsilateral vessel. When ligation was impossible and reconstruction of the systemic venous return was required, different types of atrial septation were used. Atrial septation was classified into three groups (see Fig. 1).
RESULTS
The mean duration of patient follow-up was 17.4 ± 15.7 months. All three patients with right isomerism survived the operation and all of them were in stable condition at the time of this report. One early death and two late deaths occurred among the 14 patients with left isomerism. Patient 6, a 13-year-old boy with tetralogy of Fallot who had severe left ventricular dysfunction before the operation, died early after the operation. Patient 15, who had AVSD and severe incompetence of the common AV valve, died of infection 3 months after the operation. Patient 5, who underwent a repair of corrected TGA, had an arrhythmia and died suddenly 18 months after the operation. Systemic venous obstruction developed in one patient with AVSD (patient 4), and the inflow opening for both cavae had to be enlarged with a patch 40 months after the initial operation. Severe mitral incompetence developed after the operation in patient 10. The mitral valve was replaced by a 17 mm Carpentier-Edwards prosthesis (Baxter Healthcare Corp., Edwards Div., Santa Ana, Calif.) on the seventeenth postoperative day.
Before the operation, 12 patients were in normal sinus rhythm. Junctional rhythm and atrial fibrillation were observed in two cases (patients 11 and 17, respectively). Three patients with congenital AV block had undergone permanent pacemaker implantation. Surgically induced complete AV block did not occur in any patient in this series. Postoperatively, normal sinus rhythm was maintained in three patients with right isomerism and seven patients with left isomerism. Of the other two patients who had sinus rhythm before the operation, one patient with left isomerism had a junctional rhythm immediately after the operation, and the other had sick sinus syndrome 18 months after the operation.
COMMENT
Biventricular repair is possible when the two ventricles are present, each has an adequate volume, and the anatomic relationship between the ventricles and AV valves is favorable.
10,11 In our series, anatomic repair with the morphologically left ventricle used as the systemic ventricle was possible in 15 patients; in the other two patients (Nos. 1 and 5) we used the morphologically right ventricle for systemic circulation. Some studies
12-15 have revealed disappointing late results with the correction of the morphologically right ventricle to support systemic circulation. Therefore patient 5 would currently be considered a candidate for anatomic repair with the double switch operation.
According to Di Donato and associates,
16 palliative procedures, such as ligation of a patent ductus arteriosus or systemic-pulmonary artery shunt, should be performed initially in critically ill patients who are too small to undergo a radical operation. Biventricular repair is the procedure of choice, instead of a functional repair such as the Fontan operation, because the long-term results attained with functional repair are still controversial.
17,18
The anatomic findings in the 93 patients with cardiac isomerism who had surgical treatment after 1985 are presented in
Table II. Thirty-six percent of hearts with right isomerism and 72% of those with left isomerism had two ventricles. Two AV valves were present in 3% of the patients with right isomerism and 31% of those with left isomerism. Because of the presence of anomalies of ventriculoarterial discordance or total anomalous pulmonary venous connection, there were few good candidates for biventricular repair, especially in the case of patients with right isomerism. A functional repair was performed in patients with two ventricles when one of the ventricles was hypoplastic or when two competent AV valves could not be created. In this series, the ventricular volume was measured angiographically and indexed to the normal volume on the basis of the body surface area.
19 The right ventricular volume ranged from 77% to 360% (mean 174%) and the left ventricular volume from 83% to 370% (mean 170%). The volume of both ventricles was more than 100% of normal in 12 patients.
|
Cardiac malformations associated with atrial isomerism include abnormalities of the conduction system. Congenital AV block has been reported to be more frequent in association with AVSD in left isomerism.
24-27 Paired AV nodes were reported to be linked or potentially linked by a sling conduction tissue in isomeric hearts.
26,27
We consider it advisable to locate the sinus node by intraoperative epicardial mapping. This reduces the risk of iatrogenic injury to the sinus node by caval cannulation or intraatrial manipulation. In patients with AVSD, the proper line of attachment of the baffle was difficult to ascertain, particularly when the coronary sinus ostium was absent. For such patients, endocardial mapping was attempted to identify the conduction pathway, but we did not have uniform success with this technique.
The clinical outcome in this series--early death in one patient and late death in two--was satisfactory when compared with the natural prognosis of these complex anomalies. The optimal surgical treatment for cardiac isomerism has not been established, but improved knowledge of the anatomy of these complex lesions will enhance the success of biventricular repair.
References
This article has been cited by other articles:
![]() |
H.G. Lim, E.A. Bacha, G.R. Marx, A. Marshall, F. Fynn-Thompson, J.E. Mayer, P. Del Nido, and F.A. Pigula Biventricular repair in patients with heterotaxy syndrome. J. Thorac. Cardiovasc. Surg., February 1, 2009; 137(2): 371 - 379.e3. [Abstract] [Full Text] [PDF] |
||||
![]() |
M. Koh, T. Yagihara, H. Uemura, K. Kagisaki, I. Hagino, T. Ishizaka, and S. Kitamura Biventricular repair for right atrial isomerism. Ann. Thorac. Surg., May 1, 2006; 81(5): 1808 - 1816. [Abstract] [Full Text] [PDF] |
||||
![]() |
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] |
||||
![]() |
J. S.L. Lim, B. W. McCrindle, J. F. Smallhorn, F. Golding, C. A. Caldarone, M. Taketazu, and E. T. Jaeggi Clinical Features, Management, and Outcome of Children With Fetal and Postnatal Diagnoses of Isomerism Syndromes Circulation, October 18, 2005; 112(16): 2454 - 2461. [Abstract] [Full Text] [PDF] |
||||
![]() |
Y. Oshima, M. Yamaguchi, N. Yoshimura, S. Oka, and Y. Ootaki Anatomically corrective repair of complete atrioventricular septal defects and major cardiac anomalies Ann. Thorac. Surg., August 1, 2001; 72(2): 424 - 429. [Abstract] [Full Text] [PDF] |
||||
![]() |
T. Gilljam, B. W. McCrindle, J. F. Smallhorn, W. G. Williams, and R. M. Freedom Outcomes of left atrial isomerism over a 28-year period at a single institution J. Am. Coll. Cardiol., September 1, 2000; 36(3): 908 - 916. [Abstract] [Full Text] [PDF] |
||||
![]() |
H. Uemura, S. Y. Ho, R. H. Anderson, and T. Yagihara Ventricular morphology and coronary arterial anatomy in hearts with isomeric atrial appendages Ann. Thorac. Surg., May 1, 1999; 67(5): 1403 - 1411. [Abstract] [Full Text] [PDF] |
||||
![]() |
J. A. van Son, J. Hambsch, and F. W Mohr Extracardiac Repair of Complex Unroofed Coronary Sinus Asian Cardiovasc Thorac Ann, March 1, 1998; 6(1): 52 - 53. [Abstract] [Full Text] [PDF] |
||||
![]() |
J. A. M. van Son, J. Hambsch, and F. W. Mohr Repair of Complex Unroofed Coronary Sinus by Anastomosis of Left to Right Superior Vena Cava Ann. Thorac. Surg., January 1, 1998; 65(1): 280 - 280. [Abstract] [Full Text] [PDF] |
||||
![]() |
A. Carotti, B. Marino, G. Oppido, and C. Marcelletti Biventricular repair in patients with left isomerism J. Thorac. Cardiovasc. Surg., October 1, 1995; 110(4): 1151 - 1152. [Full Text] |
||||
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| ANN THORAC SURG | ASIAN CARDIOVASC THORAC ANN | EUR J CARDIOTHORAC SURG |
| J THORAC CARDIOVASC SURG | ICVTS | ALL CTSNet JOURNALS |