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J Thorac Cardiovasc Surg 2003;126:1746-1752
© 2003 The American Association for Thoracic Surgery
Surgery for congenital heart disease |
a Division of Pediatric Cardiology, UCLA Medical Center, Los Angeles, Calif, USA
b Division of Cardiothoracic Surgery, UCLA Medical Center, Los Angeles, Calif, USA
Received for publication January 2, 2003; revisions received May 8, 2003; accepted for publication June 18, 2003.
* Address for reprints: Anuja Gupta, MD, 10621 Ashton Ave, Los Angeles, CA 90024, USA
doctoranuja{at}yahoo.com
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Abstract |
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METHODS: We reviewed a retrospective case series of 104 patients with this complex lesion. Information was obtained from medical records and referring physicians.
RESULTS: Of the 104 patients treated with the staged repair, 58 achieved completion of anatomic repair. The 10-year mortality was 16.5%. In the patients with complete repair, the median right-to-left ventricle pressure ratio was 0.5. The overall surgical reoperation rate was 17%, and 15.5% of patients required postoperative interventional cardiac catheterization. In the multivariate analysis, the number of collateral vessels incorporated in the repair was found to be an independent risk factor for postoperative mortality and an elevated right-to-left ventricle pressure ratio after complete repair.
CONCLUSION: The staged repair can be successfully used to treat patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. This method yields a relatively low mortality with good functional results.
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Methods |
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Follow-up
Follow-up of all patients was performed through December 2000. The median length of follow-up was 10.2 years.
Procedure
Details of the staged approach surgical repair and the management strategy the staged approach used at our institution have been previously described.4,5 In brief, our staged repair consists of 3 sequential steps (see Figure 2): Stage 1 involves palliative interventions (surgical or cardiac catheterization) to promote growth of the true pulmonary arteries when present and/or to control excessive pulmonary blood flow in cases with congestive heart failure. Stage 2 involves unifocalization of MAPCAs in both lungs. Stage 3 involves completion of the anatomic repair with closure of the VSD and establishment of continuity between the right ventricle and the reconstructed pulmonary vasculature.
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Data analysis
After review of the data, we performed descriptive and adjusted analysis. The descriptive analysis includes patient characteristics, pulmonary vasculature characteristics, overall and stagewise mortality, pRV/pLV achieved after complete repair, surgical reoperation rate, and requirement for postoperative interventional cardiac catheterization. In the adjusted analysis risk factors for prediction of unfavorable outcomes (mortality and an elevated pRV/pLV > 0.55) were determined. The independent variables of interest included several patient demographic and surgical factors. We performed multivariate logistic regression to identify these risk factors. The acceptable level of significance used was P < .05, and all the statistical analysis was performed using the STATA statistical software package.
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Results |
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Stage 3
A total of 58 patients underwent stage 3 repair. The median age at stage 3 repair was 5.2 years (range 1-34 years). The median time interval between stage 2 and stage 3 repair was 1.95 years (range 1-8.6 years). The median cardiac bypass time during stage 3 surgery was 168 minutes (range 114-255 minutes). The median aortic crossclamp time was 114 minutes (range 47-190 minutes). The cardiac bypass time and aortic crossclamp time included repair of the associated cardiac malformations.
Of the 58 patients who achieved complete repair, 27 (46%) originally had hypoplastic pulmonary arteries with codominant MAPCA circulation, 22 (38%) had good-sized pulmonary artery with a dominant (>60%) pulmonary artery circulation, and 9 (15.5%) had absent true pulmonary arteries with a dominant MAPCA pulmonary circulation. Two (3%) patients required creation of an adjustable atrial septal defect or fenestration of the VSD for pRV/pLV > 0.75. Five (8.5%) deaths occurred in stage 3 repair.
Mortality
Figure 1 demonstrates the timing of deaths in relation to the surgical stage of repair. Excluding 1 patient who died prior to any intervention, there were a total of 17 deaths in the series and the overall 10-year mortality was 16.5%. The in-hospital (same surgical admission) mortality was 11.5% (12 deaths) and the late (after hospital discharge) mortality was 5% (5 deaths). The mortality rate in stage 1 repair was 6%, 9% in stage 2, and 8.5% in stage 3.
Of the 12 in-hospital deaths, 2 occurred during interventional cardiac catheterization after stage 1 repair, 2 resulted from hypoxia induced by bronchospasm, 3 from suprasystemic pRV/pLV, 2 from bacterial sepsis, 1 from hypoxia induced by clotted unifocalization shunt, and 2 from unknown causes. Of the 5 late deaths, 1 resulted from liver failure in a patient with associated Alagilles syndrome, 1 from suprasystemic pRV/pLV, 1 from hypoxia induced by clotted unifocalization shunt, 1 from hypoxia induced by pulmonary embolism, and 1 from unknown cause.
Morbidity
The median pRV/pLV obtained in the operating room immediately after complete repair was 0.5 (range 0.15-1.0). The median pRV/pLV estimated at follow-up visits by echocardiography as described in the Methods section was 0.46 (range 0.3-1.0). The median pRV/pLV measured by cardiac catheterization was 0.55 (range 0.35-1.0).
There were a total of 36 (17%) reoperations in the series. The reoperation rate for stage 1 repair was 16% (10 procedures). The indications for reoperation after stage 1 included excessive cyanosis or intractable congestive heart failure from excessive pulmonary blood flow. The reoperation rate for stage 2 repair was 19.5% (18 procedures). The most common indication for reoperation after stage 2 was excessive cyanosis secondary to thrombosis or stenosis of the unifocalization shunt. The reoperation rate for stage 3 repair was 14% (8 procedures). The indication for reoperation after stage 3 was pRV/pLV > 0.75 from stenosis of the right ventricle to pulmonary artery homograft conduit.
Postoperatively there were 16 interventional cardiac catheterization procedures in the entire group. This does not include the catheterization interventions performed for stage 1 repair. Of the interventions, 15% occurred after stage 1, 70% after stage 2, and 15% after stage 3 repair. The lesions treated by interventional cardiac catheterization included: branch pulmonary artery stenosis in 9 patients (5 responded favorably to balloon angioplasty and 4 required stent placement in stenosis sites); MAPCAs stenosis in 5 patients (3 responded to balloon angioplasty and 2 required stent placement and unifocalization tube); stenosis in 2 patients (1 responded to balloon angioplasty and 1 required stent placement) in the unifocalization tube.
The functional status of survivors was defined qualitatively by the New York Heart Association (NYHA) classification. Of the 53 survivors after complete repair, 47 (89%) were in NYHA class I or II and were either full-time students, were employed full-time, or were capable of full-time work. Six (11%) patients were judged to be in NYHA class III or IV with significant symptoms. The symptoms that these patients were experiencing included recurrent and refractory ventricular tachycardia or congestive heart failure due to deterioration of right ventricular function.
Results of multivariate analysis
In the univariate analysis the number of MAPCAs included in the unifocalization was found to significantly predict the risk of death and of an elevated pRV/pLV > 0.55 postoperatively. Patients with fewer than 3 MAPCAs included in the repair were at higher risk for both unfavorable outcomes when compared with those with more than 3 MAPCAs (P < .05). In the univariate analysis, independent factors such as age and weight at first surgery, age at last surgery, sex of patient, distribution of native pulmonary arteries, and surgical variables such as duration of cardiac bypass time and aortic crossclamp time were not found to be significant predictors of mortality or of an elevated pRV/pLV. Because of the small number of patients in each subgroup, meaning comparison using multivariate analysis became difficult.
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Discussion |
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The overall mortality rate observed in our series is comparable to that noted in previous studies.6-20 The pRV/pLV is widely regarded as a surrogate measure of functional status after complete anatomic repair of this lesion. The pRV/pLV observed in our study is also comparable to that reported in other large series.6-20 We believe that this favorable pRV/pLV, particularly in patients who had extremely hypoplastic true pulmonary arteries with relatively few MAPCAs, is the result of the aggressive approach used to encourage growth of the true pulmonary arteries and aggressive recruitment of as many MAPCAs as possible in the repair, resulting in a larger effective pulmonary vasculature. In the univariate analysis we found that unifocalization of fewer than 3 MAPCAs in the repair was significantly associated with an increased likelihood of mortality and pRV/pLV > 0.55 (P < .05).
The overall surgical reoperation rate noted in our study was 16%, which is slightly higher than that previously reported.6,7,15-18 There are several possible explanations for this. Because stages 1 and 2 of the repair are performed in early infancy, the physiologic requirements of the patient frequently change with growth. The most common indication for reoperation in after stages 1 and 2 was excessive cyanosis in patients who had previously acceptable oxygen saturations after surgery. These patient required reoperation with insertion of larger unifocalization shunts. Another indication for reoperation was pulmonary artery reconstruction in patients who did not respond favorably to interventional catheterization procedures.
Several patients with extremely hypoplastic pulmonary arteries required multiple surgeries to be successfully salvaged. Remarkably, no reoperations were required for stenosis in MAPCAs, all cases of which responded favorably to interventional cardiac catheterization techniques. Also, very few reoperations were required after complete repair. We believe this to be the result of the relatively late age of stage 3 repair, which enables the use of larger-sized conduits thus reducing the requirement for subsequent revision.
The requirement for postoperative interventional cardiac catheterization in our series was relatively low, only 16 procedures in the entire series. In our opinion, this is the result of staging the repair in patients with this lesion. The use of the lateral thoracotomy incision for performing the unifocalization procedure not only provides excellent exposure of the MAPCAs but also enable greater flexibility in recruiting them into the repair. This results in reduced torsion at the surgical anastomosis sites with a lower rate of subsequent stenosis.
The limitations of this study include a relatively limited follow-up period (median follow-up 10 years) and lack of generalizability of results to all patients with pulmonary atresia with VSD and MAPCAs. Follow-up of survivors from this series is ongoing and longer follow-up data will be available in the future. Because all patients in this study were treated with the staged approach, direct comparison with patients treated with the single-staged approach is difficult. For meaningful comparison between these 2 approaches, randomized allocation of patients with pulmonary atresia with VSD and MAPCAs to single-staged or staged repair would be required.
In summary, the results of our study indicate that children with pulmonary atresia with VSD and MAPCAs can be successfully treated using the staged repair. In our experience the staged repair yields a relatively low mortality with good functional results. There remains room for improvement in the requirement for surgical reoperation. As our experience with this approach has evolved, we have observed a significant reduction in the associated mortality and morbidity. We anticipate an improved quality of life with reduced need for postoperative surgical and interventional catheterization in patients treated with this approach in the future.
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