Ann Thorac Surg 2005;80:50-55
© 2005 The Society of Thoracic Surgeons
Original article: Cardiovascular
Late Assessment After Biventricular Repair for Isomerism Heart
Hajime Ichikawa, MD,
Yoshiki Sawa, MD,
Norihide Fukushima, MD,
Toru Ishizaka, MD,
Shigemitsu Iwai, MD,
Haruhiko Kondo, MD,
Hikaru Matsuda, MD*
Department of Surgery, Osaka University Medical School, Suita, Osaka, Japan
Accepted for publication January 17, 2005.
* Address reprint requests to Dr Matsuda, First Department of Surgery, Osaka University Medical School, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan (Email: matsuda{at}surg1.med.osaka-u.ac.jp).
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Abstract
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BACKGROUND: Although biventricular repair is theoretically ideal for the treatment of isomerism heart, the long-term outcome is unknown. We assessed the outcome of biventricular repair for atrial isomerism.
METHODS: From 1984 to 2002 in our surgical database, 10 of 67 patients with atrial isomerism received biventricular repair. The age at operation was 7.7 ± 9.1 years. Preoperative ventricular volume was normal in all patients. Left ventricular ejection fraction was 62% ± 8%. Intra-atrial rerouting was required in 8. The atrioventricular septal defect with double-outlet right ventricle was closed using comma-shaped intraventricular conduit in 8. Other procedures included pulmonary valvotomy and infundibulectomy in 2, transannular patch in 1 and right ventricle to pulmonary artery conduit in 1.
RESULTS: There was one early death due to hemolytic phagocytic syndrome. The other 9 patients are in New York Heart Association class I at 12.7 ± 5.3 years postoperatively. There was no atrial baffle stenosis except in 1 patient; it was successfully treated by stent placement 10 years after the operation. There was no incidence of left ventricular outflow obstruction. Mitral replacement (4 months postoperatively) or repair (15 years postoperatively) was done in 2 patients. The other 5 patients with atrioventricular septal defect showed trivial to mild regurgitation in the long-term period. Arrhythmia was observed in 5 (left 4, right 1). Catheter ablation was needed in a patient with atrial flutter. Three of 9 patients require diuretics or digitalization, or both.
CONCLUSIONS: The long-term outcome of the biventricular repair for atrial isomerism was excellent. Late development of mitral regurgitation and arrhythmia could be managed adequately.
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Introduction
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The surgical treatment of atrial isomerism heart associated with complex cardiac anomaly was improved owing to the development of operative procedures and postoperative care for Fontan-type operations [1]. However, in the long-term follow-up period, there are still many problems after Fontan-type univentricular repair [2, 3], such as arrhythmia [4], low cardiac output syndrome, protein-losing enteropathy, and pulmonary arteriovenous malformation. Therefore, biventricular repair would be, if possible, preferable for the definitive repair of atrial isomerism heart even with atrioventricular septal defect (AVSD). The technique for the ventricular septation in the AVSD with conus deviation was improved by employing a comma-shaped ventricular patch [5–7]. By using this method for the ventricular septation, the midterm prognosis of the biventricular repair of isomerism heart was improved. However, it is still unclear whether these patients would be in the long-term follow-up period after biventricular repair for isomerism heart. In the present paper, the long-term functional and hemodynamic status of the patients with atrial isomerism heart who had undergone biventricular repair is presented.
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Patients and Methods
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From 1984 to 2000, surgical procedures were performed 114 times in 67 patients with atrial isomerism heart in Osaka University Medical School Hospital. There were 17 left isomerism and 50 right isomerism patients. The surgical procedures include 10 biventricular repairs, 27 Fontan-type univentricular repairs, 14 bidirectional Glenn procedures, 3 classic Glenn procedures, 37 systemic-pulmonary shunts, and 23 other procedures. The mean age of patients at the initial surgical procedure was 7.5 ± 9.0 years. The atrioventricular valve was a common atrioventricular valve in 64 patients and two atrioventricular valves in 3 patients.
Anatomy
Among the 10 patients who underwent biventricular repair, the age at the definitive repair was 7.7 ± 9.1 years. There were 3 males and 7 females. The mean follow-up period was 12.7 ± 5.3 years. There were 3 right isomerism and 7 left isomerism patients. The atrioventricular valve was a common valve in 7 patients and two atrioventricular valves in 3 patients. The ventriculoarterial connection was double-outlet right ventricle in 7 and normally related great vessels in 2. The loop of the great vessels was d-loop in 9 and l-loop in 1. The summary of the underlying anatomy is shown in Table 1. Anomalous systemic venous return was observed in 8 patients and total anomalous pulmonary venous return in 1 patient. The schematic diagrams of venous drainage to the atrium and ventriculoarterial connection are shown in Fig 1.
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Fig 1. Various venous drainage patterns of the patients: left isomerism = 7, right isomerism = 3. (PV = pulmonary vein.)
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Ventricular Volumes in Patients With Biventricular Repair
Preoperatively, all the patients were catheterized. Ventriculography was perfomed using biplane 35-mm cine angiography (Philip Poly Diagnost C; Philips Andover, MA). After the pressure measurement, ventriculograms were filmed in the frontal and lateral projections. The border of the ventricular cavity in both projections was traced manually at end-diastole and end-systole on the recorded selective ventriculogram. The largest ventricular projection was defined as end-diastole, and the smallest was defined as end-systole. Ventricular volumes were calculated from both projections using the area-length method. The ventricular volume was expressed as the index value for the body surface area (end-diastolic volume index [EDVI] or end-systolic volume index [ESVI]). Preoperatively, the percentage of left ventricular end diastolic volume (LVEDVI) to the normal value was plotted against the percentage of normal value of right ventricular end diastolic volume (RVEDVI) to the normal value in Fig 2. The mean LVEDVI was 180% ± 50% of normal (120 to 250) with the mean ejection fraction of 61.7% ± 7.7%, while the mean RVEDVI was 150% ± 90% of normal value (110 to 200) with the ejection fraction of 55.6% ± 7.9%. Even with the patients with decreased pulmonary blood flow (Qp/Qs < 1.0), ventricular volume was more than normal value. There was satisfactory ventricular volume and performance in all the patients. We performed biventricular repair in patients with situs solitus two atrioventricular valves, when each ventricular volume exceeded 80% of normal value. However, in this series of patients with atrial isomerism, we gave up biventricular repair unless both ventricular volumes exceeded 100% of normal.
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Fig 2. Percent normal of right ventricular end-diastolic volume index(%RVEDVI) was plotted over percent of normal of left ventricular end-diastolic volume index (%LVEDVI). The arrows indicate the patient with high pulmonary vascular resistance (PVR > 3.0). Boxes = medium flow [1.0 < Qp/Qs < 1.5]; diamonds = low flow [Qp/Qs < 1.0]; triangles = high flow [1.5 < Qp/Qs]. (Qp/Qs = pulmonary to systemic blood flow ratio.)
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Methods for AVSD Repair
Only if the common atrioventricular valve was a morphologically balanced shape was biventricular repair indicated. This was determined both by echocardiography and intraoperative findings. Seven of 10 patients had complete AVSD and were repaired using an endocardial cushion prosthesis [8] in 5 (Fig 3) and a two-patch repair in 3.
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Fig 3. The method for atrioventricular septal defect repair with autopericardial "winged" patch (Kawashima).
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Methods for Atrial Septation and Intraventricular Repair
Detailed methods for atrial septation and intraventricular repair are illustrated in Figure 4. In some patients with DORV with aorta anterior to the pulmonary trunk, extensive resection of subaortic conus was needed (see Fig 4 legend for detail).
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Fig 4. (A) The two left (L) isomerism cases with simple atrial septation and complete atrioventricular septal defect (AVSD) are shown. The case on the left required interventricular tunnel repair for double-outlet right ventricle. (B) The two cases with left (L) isomerism with complete AVSD and complex atrial septation are shown. These two cases also required extensive subaortic conus resection and complex interventricular baffles. (C) The three left (L) isomerism cases with two atrioventricular valves are shown. The first two cases also required a complex interventricular tunnel. The third case had no VSD but all the cavae and pulmonary veins were returning to the left-sided atrium; only the coronary sinus was draining in the right-sided atrium. (D) The three right (R) isomerism cases are shown. All cases required a complex atrial baffle and interventricular baffle. One patient required a valved pericardial right ventricle to pulmonary arterial conduit.(Ao = aorta; PA = pulmonary artery; PV = pulmonary vein.)
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Results
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There was 1 early death due to hemolytic phagocytic syndrome in a 21-year-old female patient, although the postoperative hemodynamics was excellent. The cause of this hemolytic phagocytic syndrome was suspected to be triggered by a massive hemolysis that occurred postoperatively from an unknown cause. The long-term survival after biventricular repair and Fontan type operation in our institute is shown in Figure 5. Although the short-term survival was improved with the Fontan type operation, our clinical database showed a superior prognosis in the biventricular repair group in terms of survival.
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Fig 5. The actuarial survival of the patients with biventricular repair (n = 10; open circles) and univentricular repair (Fontan-type repair; n = 25; open boxes) is shown. Solid boxes = patient died.
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Functional Class
Nine patients who survive the operation are in New York Heart Association (NYHA) class I at 12.7 ± 5.3 years postoperatively.
Atrial Partition
There was no atrial baffle stenosis except in 1 patient whose atrial partition was extremely complicated. This was successfully treated by stent placement 10 years after the operation (Fig 6) [9].
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Fig 6. (A) The complicated intra-atrial baffle at the biventricular repair. (B) The venous angiogram (left) and its schematic presentation (right) 10 years after the biventricular repair. The intra-atrial baffle shows multiple stenosis (black arrows). (C) The angiogram (left) and its schematic presentation (right) after the placement of two stents in the intra-atrial baffle. The white arrows indicate the enlargement of the baffle by two stents. (HV = hepatic vein; MV = mitral valve; SVC = superior vena cava; TV = tricuspid valve.)
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Hemodynamics and Performance
Seven of 10 patients were catheterized for the long term after the operation (years since biventricular repair was 8.7 ± 3.5). There was no incidence of left ventricular outflow obstruction. All the patients showed a left ventricular ejection fraction of more than 0.5. The mean central venous pressure was 7.5 ± 2.5 mm Hg, whereas that of the atrial isomerism patients after univentricular repair, who are in NYHA class I, was 11.1 ± 3.8 mm Hg. The mean maximum oxygen uptake was 23.8 ± 5.4 mL · kg–1 · min–1 in 7 patients (mean age at measurement was 16 ± 2 years old), which is superior to the mean oxygen uptake capacity of isomerism patients who had undergone univentricular repair, who remain in NYHA class I (17.3 ± 5.6 mL · kg–1 · min–1 in 14 patients, p > 0.01).
Atrioventricular Valve
A left-sided atrioventricular valve replacement occurred in 1 patient 4 months after the operation owing to infectious endocarditis. A left-sided valve annular placation and recleft suture 15 years after the operation was done in 1 patient. In this patient, the cleft seemed to grow in spite of the intact previous cleft stitch. The other 5 patients with AVSD showed trivial to mild regurgitation in the long-term period.
Arrhythmia
Arrhythmia that required treatment occurred in 5 of 9 the survivors in the long-term follow-up period. One of the 5 patients had right atrial isomerism and the other 4 had left atrial isomerism. In these patients, all 4 left isomerism patients showed sinus node dysfunction, and 2 patients required pacemaker implantation. Catheter ablation for paroxysmal atrial flutter was successfully done in a patient with right isomerism 10 years after the radical operation. The patient is not on an antiarrhythmic agent. The incidence of significant arrhythmia is comparable with that of univentricular repair patients with atrial isomerism.
Medication
Three of 9 patients required diuretics or digitalization, or both, during the long-term follow-up period.
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Comment
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The surgical strategy for atrial isomerism has been changed since the outcome of the univentricular repair was improved dramatically by the improvement of cardiopulmonary bypass, palliative procedures, postoperative care, and the introduction of a staged strategy with or without fenestration. However, biventricular repair is still a preferable choice when it is feasible. In the present study, we demonstrated the satisfactory late outcome of the patient with atrial isomerism who underwent biventricular repair.
Patient Selection
To obtain satisfactory hemodynamics after biventricular repair, it is essential to have sufficient biventricular volume, good-sized competent atrioventricular valves, and well-developed pulmonary vasculature. In these 10 patients undergoing biventricular repair for atrial isomerism heart, most of the conditions mentioned above were satisfactory. Although the accuracy of the methodology for the ventricular volume analysis, particularly those with AVSD is still controversial, it is an essential measurement before the biventricular repair for isomerism hearts. The results were consistent with the satisfactory recovery of the patients after the operation. In all patients in this series, left and right ventricular end-diastolic volume indexes exceeded 100% of normal (Fig 2). It is ideal to have a super-normal right ventricular volume in patients who need complex intraventricular tunnel repair because the right ventricular volume can be smaller after the operation. Although in some patients, the right ventricular end-diastolic volume was close to 100% of normal, no patient in this series showed a sign of right-side heart failure in the long-term follow-up periods. The hemodynamic indices after the operation were satisfactory in all patients, including 1 patient lost to hemolytic phagocytic syndrome. Another important factor when choosing biventricular repair over univentricular repair is whether the patient has a balanced AVSD. Although we had to replace a left-sided atrioventricular valve in 1 patient 3 months after the biventricular repair, this was not because of the morphology of the valve but because of infectious endocarditis. Proper morphologic evaluation by echocardiography and during surgery is essential.
Atrial Partition
In all the cases, complex atrial partition either with autopericardium or with polytetrafluoroethylene graft was required. No late complications related to the material were observed. In the case that required a stent placement in the atrial baffle [9], the intra-atrial pathway was extremely long and complicated (Fig 6). The procedure was successful, and no late stenosis was observed 5 year after the stent placement. To avoid this complexity, intra-atrial tube grafting could be another method of choice. However, tube graft may not be optimal method in the patient who will grow significantly after the operation. Careful follow-up is required in the patient who has a complex atrial baffle with a long pathway.
Interventricular Rerouting
The interventricular rerouting for the AVSD with posterior deviation of infundibular conus was first reported in 1977 [6]. Basically, the procedure was performed using a comma-shaped ventricular patch. In the present series of patients, the aortic overriding was more than 50% when the conotruncal anomaly was present. There was no patient whose aortic overriding was within the criteria of tetralogy of Fallot. This may be a specific future of the atrial isomerism. Although the interventricular rerouting is a technically demanding procedure, an appropriate diameter of the left ventricular outflow tract can be obtained with careful design and suture line of ventricular septal defect patch. There was no case that showed a left ventricular outflow stenosis in the late follow-up period.
Ventricular Function and Atrioventricular Valves
The cardiac function of the atrial isomerism heart long term after the biventricular repair may be predicted by the atrioventricular valve function and preoperative ventricular volume. In the present series of patients, except for 2 patients who needed reoperation, left-sided atrioventricular valve function was preserved, and all the patients showed satisfactory left ventricular function after biventricular repair. The right ventricular function was also preserved in all patients. However, in 1 patient who showed preoperative severe cyanosis and the smallest right ventricular end-diastolic volume, there is a subclinical right-sided atrioventricular valve stenosis without any symptoms, which needs close follow-up. There was no patient who showed tricuspid regurgitation or right ventricular dilatation, suggesting that a competent pulmonary valve and proper septation of the common atrioventricular valve play an important role to preserve the right ventricular function. Careful follow-up is necessary, especially of the patient with pulmonary atresia repaired with a valved conduit.
Arrhythmias
All the arrhythmias that occurred in left atrial isomerism patients were due to the abnormal sinus node function, and that in 1 patient with right isomerism was due to the surgical procedure. The incidence of arrhythmia was not low compared with the isomerism patients who underwent univentricular repair. The intrinsic cardiac rhythm problem in left isomerism patients could not be avoided even with biventricular repair. The incidence of medically significant arrhythmia of more than 50% is high. However, these patients with biventricular repair tolerated the arrhythmias and were treated successfully.
Limitation of the Comparison With Univentricular Repair
In the present series of patients, the long-term outcome was superior to that of the univentricular repair patient group. However, the recent advances in the outcome and the performance status of the patients with univentricular repair have improved after the introduction of total cavopulmonary connection, extracardiac total cavopulmonary connection, and more. In the near future, the outcome of the univentricular repair possibly might be comparable to that of biventricular repair.
Conclusion
With preferable anatomical features, the long-term outcome of patients with atrial isomerism who underwent biventricular repair was excellent not only in terms of survival but also in quality of life. Late complications can be managed adequately. In spite of the improved outcome with univentricular repair, biventricular repair is still the ideal procedure. Further follow-up and comparisons with the univentricular repair are to be continued.
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Acknowledgments
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The authors are grateful to Drs Shigetoyo Kogaki and Shunji Kurotobi for useful discussions.
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References
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Abstract
Introduction
