Hitoshi Shirotani

Postoperative Long-Term Results in Total Correction of Tetralogy of Fallot: Hemodynamics and Cardiac Function

Late results were assessed in 63 patients who underwent complete repair of tetralogy of Fallot. These patients were divided into four groups. Group I-A included 13 who had a transannular patch with a monocusp, and whose cross-sectional area index (CSAI) was less than 2.5 cm2/m2; group I-B included 11 with the patch and with a CSAI greater than 2.5 cm2/m2. Group II included 27 patients who underwent pulmonary valvotomy. Group III included 9 who had not undergone valvotomy and 3 who had undergone pulmonary valve replacement. Late death occurred in 1 patient, and reoperation was done on 3. Mild pulmonary stenosis (PS) was present in 73%, moderate PS in 17%, and severe PS in 10% of the patients. A significant pulmonary regurgitation (PR) of grade 3 or 4 was present in 33% of the patients in group I-A, and in 87% of group I-B, 17% of group II, and 0% of group III. Right ventricular end-diastolic volume was normal in patients with a PR of grade 2 or less, and it was higher in patients who had a significant PR. The ejection fraction was generally decreased, regardless of the grade of PR. Left ventricular function was normal in those with a PR of grade 3 or less and was impaired in those with a PR of grade 4. Thus, late postoperative hemodynamics and ventricular function were excellent in patients with a mild PS and a PR of grade 2 or less and it was poor in those with a moderate PS and a significant PR.(ABSTRACT TRUNCATED AT 250 WORDS)

Right ventricular outflow tract prosthesis in total correction of tetralogy of Fallot.

Right ventricular outflow tract obstruction was relieved by placing outflow patches across the pulmonary annulus in 39 of 195 patients who underwent total correction of tetralogy of Fallot. The mortality rate in these 39 patients was 12.8%, which did not differ significantly from the overall mortality of 11.3% (p = 1.00). The ratio of the pulse pressure to pulmonary artery systolic pressure as an index of pulmonary insufficiency was dependent on the cross-sectional area index (CSAI) of the pulmonary annulus after enlargement, as shown in the regression equation Y = I - 0.63/(X - 0.07) (r = 0.79, p <0.05). When the CSAI was 2.5 cm2/m2 or less and a single cusp was mounted on the outflow patch, the pulmonary insufficiency was negligible and the right ventricular end-diastolic pressure was 10 mm Hg or less. In patients without outflow patches, the right ventricular-to-pulmonary arterial systolic pressure gradient and the right ventricular-toaortic systolic pressure ratio 1 month after surgery was dependent on the CSAI, as shown in the regression equations Y = 54.0/X2 + 5.6 (r = 0.76, p < 0.01) and Y = 0.42/X2 + 0.36 (r = 0.72, p < 0.01), respectively. These two equations may also be applied in the case of patients with outflow patches with a single cusp. Thus, when the outflow patch is used, the CSAI must be larger than 1.75 cm2/m2 and less than 2.5 cm2/m2, and a single cusp should be mounted on the outflow patch.

Postoperative Size of the Right Ventricular Outflow Tract and Optimal Age in Complete Repair of Tetralogy of Fallot

Abstract One hundred forty-three patients underwent complete repair of tetralogy of Fallot with an overall mortality of 14.7%. The mortality rate correlated with the preoperative pulmonary artery to aorta (PA/Ao) diameter ratio but not with age. A retrospective study revealed that for success, the postoperative pulmonary annulus should be over 1.75 cm 2 per square meter of body surface area (BSA) in patients with a BSA of less than 0.6 m 2 at operation. The younger the patient, the lower was the ratio of right ventricular to aortic systolic pressure, even when the cross-sectional area index (CSAI) of the pulmonary annulus was the same. Even with application of an outflow patch, pulmonary regurgitation was negligible when the CSAI was less than 2.6 cm 2 /m 2 . The pulmonary vascular response to increased blood flow was excellent in younger patients. Residual ventricular septal defect and recurrent pulmonary stenosis were unrelated to age. Thus, for symptomatic patients, even infants, we recommend that complete repair be attempted when the PA/Ao diameter ratio is over 0.3. For patients in whom this ratio is less than 0.3, operation should be undertaken when the average diameter index of the arterial pathway to the right upper lobe is above 4 mm/m 2 . Should this index be less than 4 mm/m 2 , a two-stage operation is recommended.

Bivalvation with bridging for common atrioventricular valve regurgitation in right isomerism

A child with regurgitation in the common atrioventricular valve associated with complex heart disease underwent bivalvation with bridging for common atrioventricular valve regurgitation and arterial-pulmonary shunt for low pulmonary blood flow. Postoperative cardiac catheterization and color Doppler echocardiography revealed elimination of atrioventricular valve regurgitation and ventricular enlargement, reflecting an increase in pulmonary artery blood flow. We describe the concept and technique of bivalvation with bridging for common atrioventricular valve regurgitation.

Semilunar Valve Replacement with a Cylindrical Valve

Abstract A cylindrical valve was designed to prevent regurgitation of the semilunar valve. The valve is made of a sheet of polytetrafluoroethylene (PTFE) or porcine pericardium, and has three cusps and three commissures. The diameter of the valve is equal to the height of the cusps. We have used these valves in pulmonary stenosis after Jatenes operation and total correction of tetralogy of Fallot, and for truncal valve regurgitation. Regurgitation was trivial on color Doppler echocardiography in all cases. Advantages in comparison with the implantation of commercially available artificial valves include the ability to insert a larger size and no compression of the valve ring when closing the sternum. Outflow tract obstruction does not occur even when the valve is implanted in a small infant. In the present report, we describe this simple technique.

Thromboembolic Pulmonary Hypertension Due to Disseminated Fibromuscular Dysplasia

We present two patients with thromboembolic pulmonary hypertension associated with unusual complications probably caused by disseminated fibromuscular dysplasia (FMD) or FMD-like vascular lesions. Intimal fibroplasia, which is typical of the vascular lesions associated with FMD, was observed in both patients. The presence of such intimal lesions suggests that there was a systemic factor that caused the formation of recurrent thrombi in the systemic vessels in these patients. These cases are the first ones reported in which an association between FMD and pulmonary hypertension has been observed. The pathogenesis of the thrombi in our patients was thought to be recurrent pulmonary thromboembolisms resulting from FMD.

Transposition of the great arteries with posterior aorta: detection by two-dimensional echocardiography.

SummaryTwo-dimensional echocardiographic features in a case of transposition of the great arteries (TGA) with the posterior aorta are described. The unusual arrangement of the great arteries and the presence of bilateral conuses and aortic-mitral fibrous continuity were clearly demonstrated by angiocardiography and then confirmed at surgery.

Two-Cusp Plasty for the Right Ventricular Outflow Tract in Complete Repair of Tetralogy of Fallot

A procedure termed two-cusp plasty, which prevents pulmonary regurgitation in complete repair of tetralogy of Fallot, is described.

Reconstruction of pulmonary artery with substitute valve: with special reference to size of conduit and valve

Operative risk factors and postoperative late results were evaluated in 26 patients undergoing pulmonary artery reconstruction with a substitute valve. Seventeen extracardiac conduits bearing a valve were used in 16 patients and an in situ pulmonary valve insertion was carried out in the other 10. The surgical results were influenced by complexity of the underlying cardiac lesions and pulmonary vascular status, with a high mortality rate in patients with several cardiac defects including single ventricle, asplenia syndrome, complete atrioventricular canal etc. The mortality rate was 6% in patients with an immediate post-repair Ppv/sv of less than 0.75 and 77.8% in those with a Ppv/sv over 0.75. Postoperative Ppv/sv was mainly regulated by valve area index and a close correlation was obtained for the regression equation Ppv/sv = 0.41/(VAI)2 + 0.36 (r = -0.61, p less than 0.05). To obtain excellent hemodynamics with a Ppv/sv of less than 0.50, valve area index should be over 1.7 cm2/M2, and to eliminate re-implantation of the conduit after reaching adulthood, the diameter of the conduit should be 18 mm or more and the valve size 23 A, or more when a SJM valve is used.