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To determine whether continuous systemic perfusion is of effective use when establishing primary repair of the aortic obstruction and associated cardiac malformations.

To determine the clinical features in patients with double inlet right ventricle (DIRV) and separate atrioventricular (AV) valves in terms of the Fontan procedure.

To determine the efficacy of coronary artery bypass grafting (CABG) in young patients with coronary-arterial obstructive disease subsequent to Kawasaki disease.

To evaluate the results after total cavopulmonary connection (TCPC) in small children, our clinical experience was retrospectively reviewed.

To determine the efficacy in the intermediate term of artificial cords inserted in children with congenital mitral regurgitation.

To determine whether or not the ventricular outflow tracts can be efficiently constructed in patients with double outlet right ventricle with subpulmonary ventricular septal defect by the Kawashima intraventricular rerouting in which the morphologically right ventricular outlet is divided into two, one for the systemic and the other for the pulmonary circulations.

Background Few studies have investigated the clinical advantages of surgical correction with the morphologic left ventricle (MLV) instead of the morphologic right ventricle as a systemic ventricle (SV) in patients with congenital heart disease. Methods Twenty-four healthy control subjects (group A1), 6 patients with isolated congenitally corrected transposition of the great arteries (TGA) (group A2) , 16 patients with TGA who had undergone an arterial switch operation (group B1), 18 patients with TGA who had undergone a venous switch operation (group B2), 9 patients with atrioventricular and ventriculoarterial discordance who had undergone a double switch operation (group C1), and 6 patients with atrioventricular and ventriculoarterial discordance who had undergone a conventional external conduit operation from the MLV to the pulmonary artery (group C2), performed treadmill exercise testing. Their heart rate (HR), oxygen uptake (V@?O"2), and oxygen pulse (O"2 pulse), which reflects individual stroke volume, were measured, and contractile function was assessed by echocardiography. Results The peak HR for the patients after a definitive operation were significantly lower than that in group A1 and was correlated with peak V@?O"2 (r = .67, P < .0001). The peak V@?O"2 and peak O"2 pulse for the groups A2 and B2 were significantly lower than those for the groups A1 and B1, respectively. The peak O"2 pulse data were strongly correlated with those of peak V@?O "2 (r = 0.91, P < .0001). The left ventricular ejection fraction was significantly lower in groups B1 and C1 than in group A1 and was correlated with peak V@?O "2 (r = .50, P < .01). No significant differences in V@?O"2, HR, and O "2 pulse at peak exercise were observed between groups C1 and C2. Conclusions Chronotropic incompetence and an impaired response of the stroke volume of the MRV during exercise are partly responsible for the reduced exercise capacity in groups A2 and B2 compared with groups with the MLV as an SV, and the SV function at rest is also related to exercise capacity. Superiority of the double-switch operation compared with the conventional conduit operation was not observed. A longer-term follow-up is necessary before the advantages of these 2 operations can be compared. (Am Heart J 1999;137:1185-94.)

To minimize deleterious postoperative influences of cardiopulmonary bypass on the pulmonary circulation immediately after the Fontan type procedure, total cavopulmonary connection was achieved without use of cardiopulmonary bypass.

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 THORAC CARDIOVASC SURG 1995; 109: 530-5)

Unifocalization, a surgical technique to unifocalize the pulmonary blood supply in patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries, is a useful preparative operation to extend the indication for corrective surgery. The preoperative and postoperative pulmonary angiograms of 51 patients (aged 3 months-26 years at first unifocalization, 29 males, 22 females), who underwent a total of 96 unifocalizations from December 1985 to July 1994, were studied to assess the effectiveness of each procedure of unifocalization. The procedures of unifocalization were ligation (9), angioplasty (6), direct anastomosis (25), bridging (6), additional central pulmonary artery creation (9), central pulmonary artery creation (36) and thrombectomy (5). Altogether 125 anastomoses were made, and the patency of 101 (80.2%) anastomoses was confirmed, 24 (19.2%) were shown to be occluded. The patency rate of the anastomoses between xenograft rolls and intrapulmonary arteries was 79.5% (70/88), while that between native central pulmonary arteries and intrapulmonary arteries was 83.8% (31/37; N. S.). The patency rate of the anastomoses involving intrahilar arteries was 88.0% (44/50), while that for the group involving extrahilar arteries only was 71.9% (46/64; P < 0.05). It is concluded that 1) unifocalization with the reconstruction of central pulmonary arteries using a pericardial roll is a useful method for patients with absent or hypoplastic central pulmonary arteries, 2) it is preferable to divide the fissures of lung in anastomosing pulmonary arteries of arborization abnormalities.

We examined the arrangement of the coronary arterial and cardiac venous systems in 46 specimens with discordant atrioventricular connections so as to identify any structural abnormalities and to consider their surgical implications in terms of anatomical biventricular repair. Grossly abnormal arterial courses were seen in 11 hearts (24%). A substantial branch supplying the morphologically right ventricular outflow tract, which could restrict a ventriculotomy, was found in 61% of cases. The coronary sinus received all the morphologically right ventricular veins, as well as the posterior interventricular vein, in 40 hearts, this pattern being in contrast to the pattern in the normal heart. The morphologically left ventricular and anterior interventricular veins, all of which drain via the coronary sinus in the normal heart, were frequently connected independently to the morphologically right atrium in the specimens with discordant connections, the drainage occurring through the spaces between the pectinate muscles. These direct drainages are at risk of potential damage either by extensive intra-atrial maneuvers or by postoperative intraatrial thrombosis. It is predicted, therefore, that surgical results can be improved still further when account is taken of this vascular anatomy of the heart itself.

In patients with isomeric atrial appendages, regurgitation of atrioventricular valves is recognized clinically as one of the risk factors that militate against successful achievement of definitive repairs. To determine whether this reflected anatomic features, we investigated 91 specimens with atrioventricular septal defect that had a common atrioventricular valve and biventricular atrioventricular connections. Of these specimens, 35 had isomeric right appendages, 23 showed isomeric left appendages, and 33 had usual atrial arrangement. We measured either the size or location of the supporting papillary muscles and the circumference of the mural leaflet within the morphologically systemic ventricle, as well as the length of outlet, inlet, and so-called scooped dimensions of the muscular ventricular septum. Presence of a solitary papillary muscle, or deviation of the attachments of the papillary muscles, was more frequent in hearts with isomeric right appendages. Values for the diameter and lengths of the papillary muscles were significantly smaller in hearts with isomeric right appendages compared with those with usual atrial arrangement (p < 0.001), as were the distances between the papillary muscles (p < 0.002) and the circumference of the mural leaflet (p < 0.001). The proportional length of ventricular outlet was longer in the setting of isomeric right appendages than in the other groups (p < 0.001), whereas the extent of septal scooping showed no differences among these three groups. We conclude that these structural features could be factors in the known insufficiency of the common atrioventricular valve and the ventricular dysfunction in patients with isomeric right appendages. (J THORAC CARDIOVASC SURG 1995;110:445-52)

Although absence of the coronary sinus is widely recognized in hearts with isomerism of the right atrial appendages, little attention has been paid to the fashion of the venous return from the heart itself. In this study, the arrangement of coronary venous return was investigated in 99 specimens with isomeric right and 49 with isomeric left appendages. In the normal heart, the coronary veins consist of a circumflex component within the atrioventricular groove and longitudinal components on the ventricular mass. The circumflex venous system was seen in 44 hearts with isomerism of left appendages (90%), but 23 of these hearts lacked the anatomic features of the coronary sinus. Circumflex veins were entirely lacking in the other 10% of hearts with isomeric left appendages and in all those with isomeric right appendages. In these hearts, longitudinal veins drained independently into the atria in three patterns. The first was a direct connection, with the venous orifice opening between the trabeculations of the atrial wall immediately having crossed the atrioventricular groove. The second was a crooked return, with the vein running an intramural course along the atrioventricular groove. The third was a distant connection, reaching superiorly to the smooth-walled atrial component after running an intramural course. Intramural courses were seen in 19% of the longitudinal veins, such veins being found in 62% of all hearts with no circumflex venous system. These findings, which to the best of our knowledge have never previously been recognized in detail, almost certainly have potential surgical significance. (J THORAC CARDIOVASC SURG 1995;110:436-44)

Postoperative conditions after a Fontan-type operation, particularly as they affect results in the early term, are thought to depend on factors such as the state of pulmonary circulation and ventricular function. In this study, we attempted to determine the factors that influence ventricular characteristics in the middle term after Fontan-type procedures. Catheterization was performed at a mean of 15 months after operation in 57 patients with univentricular atrioventricular connection who underwent the operation between 1.0 and 22.6 years of age. End-diastolic volume, end-systolic volume, ejection fraction, and end-diastolic pressure of the systemic ventricle were analyzed together with an estimation of the systemic flow index. These parameters were influenced significantly by the presence of atrioventricular valve insufficiency. The morphologically left ventricle showed a better ejection fraction than did the morphologically right ventricle, whereas the systemic flow index was greater in patients undergoing total cavopulmonary connection than in those receiving an atriopulmonary connection. Young age was significantly associated with a better postoperative contractility, whereas the potential for impaired ventricular compliance was suggested in several patients undergoing operation after 4 years of age. On the basis of our results, we conclude that total cavopulmonary connection performed at a young age should be the surgical procedure of choice and that atrioventricular insufficiency must be treated properly at, and even after, the initial definitive repair. (J T HORAC C ARDIOVASC S URG 1995;110:405-15)

To extend the indications for corrective operation in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, surgical procedures were done to unify the blood sources for pulmonary perfusion. Since December 1985, 50 patients have undergone unifocalization at ages from 2 months to 26 years with a mean of 6 +/- 7 years. In total, 84 staged unifocalization procedures and 5 other palliative procedures were done in 49 patients. These included several operative procedures: simple ligation of major aortopulmonary collateral arteries in 8; pulmonary angioplasty in 29 including reconstruction of the pulmonary arterial tree by direct anastomosis or interposition between the central pulmonary arteries and the intrapulmonary arteries; construction of artificial central pulmonary arteries with use of a xenograft pericardial tube graft in 36 with no native central pulmonary arteries detected; and construction of supplemental central pulmonary arteries also with use of a pericardial tube graft in 10. The pericardial tube graft, if used, was anastomosed to the intrapulmonary arteries on one end and connected to a prosthetic tube on the other end so as to perfuse the reconstructed pulmonary arteries. The anastomosis was made inside the lung through the divided interlobar fissure. Five patients died after operation among those undergoing these 89 preparative operative procedures. Deaths were related either to bleeding caused by anticoagulation therapy administered to prevent thrombosis within the xenograft pericardial tube graft used or to progressive congestive heart failure as a result of an excessive amount of pulmonary blood flow. Twenty-six patients have undergone intracardiac repair after previous unifocalization. In 16 patients the artificial central pulmonary arteries surgically constructed were connected to each other and then an external conduit was placed. In another patient, intracardiac repair and unifocalization could be concomitantly achieved via a median sternotomy. The right ventricle to left ventricle systolic pressure ratio immediately after intracardiac repair in 27 patients ranged from 0.24 to 0.91 with a mean of 0.54 +/- 0.17. One patient (4%) died shortly after intracardiac repair because of thrombosis within the pulmonary arteries. Postoperative catheterization showed that pulmonary vascular resistance was correlated significantly with the number of pulmonary vascular segments functioning rather than with the condition of the central pulmonary arteries. We conclude that surgical unifocalization is a feasible procedure before subsequent intracardiac repair, even in patients with critically hypoplastic or absent central pulmonary arteries. (J T horac Cardiovasc Surg 1996;112:392-402)

To determine factors affecting postoperative pulmonary circulation in patients with major systemic-to-pulmonary collateral arteries.

To clarify the precise anatomic arrangement of the annular attachment of a common atrioventricular valve in the setting of visceral heterotaxy.