The Older Main Artery Transposition And Ventricular Septal Defect And Severe Pulmonary Hypertension Basis And Clinical Research

Background:It is widely accepted that patients older than 6 months with dextro-transposition of the great arteries and nonrestrictive ventricular septal defect or Taussig-Bing anomaly and severe pulmonary hypertension usually are not indicated for arterial switch operation or even repair. This study aimed to evaluate the midterm results of arterial switch operation in these selected patients.Methods:The records of 86 patients older than 6 months with dextro-transposition of the great arteries and nonrestrictive ventricular septal defect or Taussig-Bing anomaly and severe pulmonary hypertension undergoing arterial switch operation at our institution from 2000 to 2008 were reviewed retrospectively.80 survivors were followed up.Results:There were 6 hospital deaths (7.0%,95%confidence limit,1.6-12.4%). From 2006 to 2008,46 were operated on without death. The median duration of follow-up was 42 months.2 late deaths occurred. Latest follow-up data showed that 2.8% of survivors were in NYHA class II and 97.2%in class I.Conclusions:Midterm results of arterial switch operation for patients older than 6 months with dextro-transposition of the great arteries and nonrestrictive ventricular septal defect or Taussig-Bing anomaly and severe pulmonary hypertension are excellent in current era. PartⅡLung biopsy findings in previously inoperable patients with severe pulmonary hypertension associated with congenital heart diseaseBackground:Congenital heart disease with near-systemic pulmonary arterial pressures, previously thought to have irreversible pulmonary vascular disease (PVD), have been successfully corrected at our institution recently. Whether the PVD is reversible remains unknown. This study aimed to examine the nature of the pulmonary arterial vessels in these selective patients.Methods:All patients with congenital heart disease and severe pulmonary hypertension (PH) were selected using Diagnostic-treatment to undergo radical repair (n=49). Lung biopsy specimens were obtained during operation. The nature of PVD was determined by Heath-Edwards classification system. All specimens were quantitatively analyzed by calculating percentage media wall area, percentage media wall thickness and arteriole density.Results:Transcutaneous oxygen saturation of all selected patients increased significantly after Diagnostic-treatment (P﹤0.001). There were no operative deaths. Mean pulmonary artery pressure and pulmonary vascular resistance regressed significantly postoperatively (P<0.001). The incidence of postoperative PH was 59.2%(29/49). Of 49 selected patients with severe PH,38 (77.6%) showed gradeⅠchange,5 (10.2%) showed gradeⅡchange,4 (8.2%) showed gradeⅢchange and only 2 (4%) showed gradeⅣchange with plexiform lesion. The percentage media wall area, percentage media wall thickness and arteriole density were significantly increased in patients associated with PH than in normal subjects (P﹤0.001). Follow-up data showed the reversal of PVD in these 2 patients with plexiform lesions.Conclusions:The PVD in these selective patients with congenital heart disease and severe PH using a Diagnostic-treatment-and-Repair strategy is generally reversible and these patients are operable in current era. Part III Comparison of pulmonary vascular disease in congenital heart disease with left-to-right shunts to transposition of great arteries with ventricular septal defectBackground:Congenital heart disease (CHD) with near-systemic pulmonary arterial pressures has been successfully corrected recently. This study aimed to examine the nature of the pulmonary arterial vessels in these selective patients and compare pulmonary vascular disease (PVD) in left-to-right shunt CHD to transposition of great arteries with ventricular septal defect.Methods:Lung biopsy specimens were obtained from patients selected using a Diagnostic-treatment-and-Repair strategy. Group I (n=38) comprised patients with left-to-right shunt CHD and severe PH; Group II (n=11) consisted of older patients with transposition of great arteries with ventricular septal defect and severe PH. Autopsy cases of normal cardiovascular system and pulmonary circulation were studied in Group III (n=6). The nature of the pulmonary arterial vessel was determined by Heath-Edwards classification system. All specimens were quantitatively analyzed.Results:In Group I (n=38),31 showed grade I change,3 showed grade II change,3 owed grade III change and only 1 showed grade IV change. In Group II (n=11),7 showed grade I change,2 showed grade II change,1 showed grade III change and only 1 showed grade IV change. Follow-up data showed the reversal of PVD in these 2 with plexiform lesions. Percentage media wall thickness, percentage media wall area and arteriole density in Group I and Group II were significantly higher than those in Group III. Percentage media wall area in Group II was significantly higher than that in Group I; significant differences of percentage media wall thickness and arteriole density could not be found between Group I and Group II.Conclusions:PVD in these selected_patients with CHD and severe PH using a Diagnostic-treatment-and-Repair strategy is generally reversible and the changes of PVD in patients with transposition of great arteries with ventricular septal defect are similar to those in left-to-right shunt CHD.