Background: Although the modified Blalock-Taussig shunt remains the mainstay method of palliation for augmenting pulmonary blood flow in various congenital heart diseases, the shunt must be carefully designed to achieve the best outcomes. This study investigated the effect of shunt configuration on pulmonary artery growth and growth discrepancy. Methods: Twenty patients with successful modified Blalock-Taussig shunt takedown were analyzed. Pulmonary artery and shunt characteristics were obtained using computed tomography scans. Differences in the baseline and follow-up diameter ratios and growth in the ipsilateral and contralateral arteries were calculated. The angle between the shunt and pulmonary artery, as well as the distance from the main pulmonary artery bifurcation, were measured. Correlations between pulmonary arteries and shunt configurations were analyzed. Results: The median interval time between shunt placement and takedown was 154.5 days (interquartile range, 113.25-276.25 days). Follow-up values of the ipsilateral-to-contralateral pulmonary artery diameter ratio showed no significant correlation with the shunt angle (ρ=0.429, p=0.126) or distance (ρ=0.110, p=0.645). The shunt angle and distance from the main pulmonary bifurcation showed no significant correlation (ρ=-0.373, p=0.189). Pulmonary artery growth was negatively correlated with shunt angle (ipsilateral, ρ=-0.565 and p=0.035; contralateral, ρ=-0.578 and p=0.030), but not with distance (ipsilateral, ρ=-0.065 and p=0.786; contralateral, ρ=-0.130 and p=0.586). Conclusion: Shunt configuration had no significant effect on growth imbalance. The angle and distance of the shunt showed no significant correlation with each other. A more vertical shunt was associated with significant pulmonary artery growth. We suggest a more vertical graft design for improved pulmonary artery growth.
Modified Blalock-Taussig operation remains the standard technique of systemic-to-pulmonary artery shunt in patients of congenital heart disease with decreased pulmonary blood flow. We reviewed the 41 patients who had been performed modified Blalock-Taussig operation from March 1985 to Feb. 1988, and angiographically measured pulmonary artery size before and after shunt, and calculated pulmonary artery index [PAI] and ratio of left and right pulmonary artery to descending aorta[[LPA+RPA]/dAo]. The mean duration of palliation after shunt operation was 624 days and mean age of the patient at shunt operation was 3.59 years. Mean PAI increased significantly from 131.15*67.11 mm2/M2 preoperatively to 232.70*84.46 mm2/M2 postoperatively. Mean ratio of right and left pulmonary artery to descending aorta also increased significantly from 1.48*0.40 preoperatively to 1.92*0.50 postoperatively. All patients manifested clinical improvement; there was mean decrease in hematocrit of 8.95%, mean increase in arterial oxygen saturation of 11.08%. Pulmonary arterial growth was not influenced by age at operation, initial pulmonary artery size, or graft size, but significantly influenced by antegrade flow. The patients who have some antegrade flow were in more increase of PAI. There were no linear correlation between change of PAI, change of [[LPA+RPA]/dAo], SaO2, and duration. But, according to Scatterplot between change of PAI and duration, some complex correlation was suggested and mean PAI was decreased after 2-year palliation. We concluded that modified Blalock-Taussig operation is excellent palliative surgery for pulmonary artery growth especially on the patient who have some antegrade flow, and the proper duration of palliation was about 2 years.
Pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries [abbreviated as PA+VSD+MAPCA in the following] has limited the success of attempts at accurate diagnosis and complete surgical repair. From April 1986 to September 1990, 23 patients with PA+VSD+MAPCA among 96 patients of PA+VSD in Seoul National University Children’s Hospital were encountered. The group comprised 14 male and 9 female patients with ages ranging from 17 days to 177 months [mean 49.6 months]. We operated one stage total repair on good pulmonary artery sized two patients by R.E.V. [Reparation a l’etage ventriculaire] and Rastelli operation respectively. And the 11 patients who had independent MAPCAs and hypoplastic central pulmonary artery were dealt with unifocalization and modified Blalock-Taussig Shunt and followed by second stage repair in 3 patients later. We successfully had managed 7 patients whose MAPCAs could be ligated with modified Blalock-Taussig Shunt and followed by second stage repair in 3 patients with R.E.V or Rastelli operation. Recently, three obstruction after 11 unifocalization procedures made us to try palliative right ventricle-pulmonary artery conduit operation by Gore-Tex vascular graft interposition under cardiopulmonary bypass. And so we managed another 3 patients with these procedures for the purpose of pulmonary artery growth whose central pulmonary artery were severely hypoplastic. We experienced one death after second stage repair whose central pulmonary artery was created by 12mm Gore-Tex vascular graft and was unifocalized.
Ten patients with tetralogy of Fallot were studied angiocardiographically before a modified Blalock-Taussig shunt and again 25*3.2 months after the previous shunt. All of ten patients had patent previous shunt at the time of follow up examination. Pre-and postoperative diameters of left and right pulmonary artery and descending aorta were measured and pulmonary artery index [the sum of the crossectional areas of the right and left pulmonary arteries standardized by the body surface area] was calculated. The ratio of mean diameter of left and right pulmonary arteries to the diameter of the descending aorta [LPA+RPA/2xDA] was increased postoperatively by 0.20*0.068 [p=0.020]. Mean PAI [pulmonary artery index] increased from 283.8*178.4 mm/m2 BSA to 345.8~144.5 mm/m2 BSA after shunt operation [p=0.019]. This results suggested that the modified Blalock-Taussig shunt was effective to help growth of the pulmonary arteries in most cases of the study populations but the ones with the PAI>233mm*/m* BSA appeared less benefited by Blalock Taussig shunt. Calculation of PAI could be an aid to making a decision whether to perform a one stage corrective surgical procedure or a palliative shunt procedure in the patient with small pulmonary arteries.
The possible relationship between pulmonary vascular pathologic changes and an increased pulmonary blood flow and pulmonary blood pressure has been the subject of investigation for many years. In an attempts to study the effects of increased pulmonary blood flow. long-term observations have been made in neonatal goats in which the total pulmonary vascular bed was restricted by means of ligation of left pulmonary artery, thereby diverting the total right ventricular output through the right lung. The left pulmonary artery and patent ductus arteriosus were ligated in 6 neonatal goats of under 3 weeks of age, and the goats were put to death at interval between 1 and 7 months of age. Pulmonary arterial development in both right and left lungs was studied by applying quantitative morphometric techniques, and compared with control group of goats between 1 and 6 months of age. The axial pulmonary artery and its branches were larger in the right lung than in the control group in all animals, and they were abnormally small in the left lung. In the right lung, arteries smaller than 50\ulcornerm showed abnormal increase in `% wall thickness’ in postoperative 2,3,5 and 6th months[p<0.05]. The proportion of non-muscular arteries was over 50% at postoperative 1st month in both right and left lungs, but an increase in proportion of partially muscular and wholly muscular arteries occurred thereafter. The ratio of alveoli/arteries was lower than normal in the right lung of postoperative 1st month[p<0.05], but was elevated thereafter. The failure to perfuse one pulmonary artery in neonatal goats changed growth and development of both lungs.
Nho, Un Seok;Kim, Yeo Hyang;Hyun, Myung Chul;Lee, Sang Bum
Clinical and Experimental Pediatrics
/
v.46
no.2
/
pp.167-172
/
2003
Purpose : Pulmonary vascular hypertension is a common problem in congenital heart disease, the most common cardiac condition in childhood. However, the mechanisms responsible for this pathologic change, treatment, and prevention are poorly understood. Therefore, we studied the gene expression of vascular endothelial growth factor(VEGF) by using a hypoxic model of the pulmonary artery smooth muscle cells. Methods : The main pulmonary artery and its proximal branches of a 6 wk old Fischer rat were excised. They were cut into multiple small pieces and suspended in DMEM medium supplemented with 20% fetal bovine serum and incubated in 5% $CO_2$-95% air atmosphere. The smooth muscle cells were confirmed by immunostaining with smooth muscle myosin and ${\alpha}$-smooth muscle actin antibodies. The VEGF gene expression in the hypoxic group was compared with the one in control the group as well as the one in the starved group by RT-PCR and Northern blot hybridization. Results : There was no statistically significant difference among the control, hypoxic and starved groups. Conclusion : There are few studies of pulmonary vascular hypertension at the molecular level in Korea. Therefore, we studied the expression of VEGF gene in hypoxic pulmonary vascular smooth muscle cells. Further studies will be needed to find the difference between newly born and adult rats, or human and rat pulmonary vascular smooth muscle cells in gene expression. We hope that the study will lead to a better understanding of pulmonary vascular hypertension.
Background: Modified Blalock-Taussig shunt using 3mm or 3.5mm PTFE graft has been performed in patients with small body weight or in candidates for single ventricle palliation. However, there are few reports concerning clinical outcomes in terms of pulmonary artery growth and shunt patency rate after shunt operations using such a small graft. Material and Method: Twenty-five patients rate after shunt operations using 3 or 3.5 mm sized grafts from September 1996 to August 1999. We retrospectively assessed the pulmonary artery growth and the shunt patency rate by reviewing the pre-and post-operative pulmonary angiograms. The risk factors for late death and second shunt operations were also analyzed. To assess the presence of any correlation between body weight and selection of the graft size, regression analysis was done in 81 cases of shunt operations performed during the same period. Result: There were 1(4%) early death and 5(20%) late deaths. The survivors were followed up for an average of 7.34 months. The pulmonary artery index increased significantly from 129$\pm$66$\textrm{mm}^2$/$m^2$ to 213$\pm$114 $\textrm{mm}^2$/$m^2$(p=0.002). The shunt patency rate assessed at postoperative 2, 4, 6 and 8 months were 82.5%, 77%, 73% and 42% respectively with a marked decline between 6 and 8 months. Asplenia was a frequent finding for the patients with late death although the incidence failed to reach statistical significance(p=0.078). Pre-operative diagnosis of PA with VSD was found to be a statistically significant risk factor for a second shunt operation(p=0.01). Body weight(a) at operation and graft size(b) used in the shunt operations revealed strong correlation and could be expressed by the following formula; b=0.128a + 3.233. Conclusion: Adequate growth of pulmonary artery and satisfactory early patency rate could be obtained by modified Blalock-Taussing shunt using 3mm or 3.5mm graft. However, during 6 to 8 months after shunt operations, the patency rate fell sharply, which implicates that close observation and early intervention are mandatory in this period.
Background: Pulmonary atresia (PA) with ventricular septal defect has various morphology of pulmonary arteries and pulmonary blood flow sources, so pulmonary arterial hypoplasia and arborization abnormality make this anomaly difficult to manage surgically. In cases associated with juxtaductal stenosis, we evaluated the change of the pulmonary arterial and juxtaductal stenotic site after shunt operations, and would like to find useful information in surgical planning and methodology of these patients. Material and Method: Among 59 cases diagnosed as PA with ventricular septal defect associated with juxtaductal stenosis, 29 cases who had cardiac catheterization before and after shunt operation were selected from July, 1991 to July, 1996. In 10 cases of right shunt operation(Group I) and 19 cases of left shunt operation (Group II), the diameters of the descending aorta, both pulmonary arteries, and the juxtaductal stenosis site were measured before and after the shunt operation. Result: In both Group I and II, the pre- and postoperative ratio of diameters of the ipsilateral pulmonary artery to the descending aorta was from 0.78${\pm}$0.31 units to 1.01${\pm}$0.26 units and from 0.67${\pm}$0.18 units to 0.84${\pm}$0.27 units respectively, showing a signigicant increase. The contralateral pulmonary artery index was increased from 0.92${\pm}$0.28 units to 1.05${\pm}$0.15 units and from 0.94${\pm}$0.27 units to 1.08${\pm}$0.37 units respectively, but could not be confirmed statistically. In both groups, the change of juxtaductal stenosis showed an aggravating tendency but of no statistical significance from 0.43${\pm}$0.27 units to 0.39${\pm}$0.25 units and from 0.32${\pm}$0.10 units to 0.30${\pm}$0.16 units respectively, and we experienced 2 total obstruction in Group II. Because the increased pulmonary blood flow by shunt operation has a favorable effect to the pulmonary arterial growth, the shunt operation is a recommended treatment in patients with hypoplastic pulmonary arteries. But in PA with ventricular septal defects, the change of juxtaductal stenosis is very important. In conclusion, the growth of ipsilateral (shunt site) pulmonary artery was promoted by shunt operation, but there is a tendency for the juxtaductal stenosis to be aggravated. And we experienced 2 total obstruction in Group II. Conclusion: Thus, in cases operated with shunt method, much careful postoperative follow up study including angiographic evaluation is needed, and after the shunt operation on the side of pulmonary artery associated with juxtaductal stenosis, early precise planning for total correction is recommended.
Double-outlet left ventricle(DOLV) is a rare congenital cardiac malformation, defined as the origin of both the aorta and the pulmonary artery being entirely or predominantly above the morphologically left ventricle, which is difficult to diagnose accurately. A 3-year old male was admitted for cyanosis and dyspnea. At the age of 2 months, he had undergone pulmonary artery banding and coarctoplasty. He was diagnosed as DOLV with subaortic ventricular septal defect(VSD). Biventricular repair was achieved by patch closure of VSD, primary closure of PFO, and pulmonary trunk translocation from left ventricle to right ventricle. The advantages of this procedure using native tissue for right ventricular outflow tract reconstruction are growth potential and preserved valve function, which contribute to a decreased likelihood of reoperation related to the right ventricular dysfunction related to pulmonary insufficiency.
A five-month-old boy who had undergone previously transcatheter balloon atrioseptostomy at 3 days of age for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis underwent pulmonary root translocation with the Lecompte maneuver. This operation has the advantages of maintaining pulmonary valve function, preserving the capacity for growth, and avoiding problems inherent to the right ventricular to pulmonary artery conduit. This patient progressed well for 9 months postoperatively and we report this case of pulmonary root translocation with the Lecompte maneuver.
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