Structural examination of human heart specimens at the microscopic level is a prerequisite for understanding congenital heart diseases. It is desirable not to destroy or alter the properties of such specimens because of their scarcity. However, many of the currently available imaging techniques either destroy the specimen through sectioning or alter the chemical and mechanical properties of the specimen through staining and contrast agent injection. As a result, subsequent studies may not be possible. X-ray phase-contrast tomography is an imaging modality for biological soft tissues that does not destroy or alter the properties of the specimen. The feasibility of X-ray phase-contrast tomography for the structural examination of heart specimens was tested using infantile and fetal heart specimens without congenital diseases. X-ray phase-contrast tomography was carried out at the SPring-8 synchrotron radiation facility using the Talbot grating interferometer at the bending magnet beamline BL20B2 to visualize the structure of five non-pretreated whole heart specimens obtained by autopsy. High-resolution, three-dimensional images were obtained for all specimens. The images clearly showed the myocardial structure, coronary vessels, and conduction bundle. X-ray phase-contrast tomography allows high-resolution, three-dimensional imaging of human heart specimens. Intact imaging using X-ray phase-contrast tomography can contribute to further structural investigation of heart specimens with congenital heart diseases.
Among the later modifications of the Fontan type procedure, a significant alteration was introduced by de Leval and associates when they described the total cavopulmonary connection (TCPC). Although current results of TCPC have encouraged us to extend this procedure to high risk patients, risk factors influencing surgical outcome after TCPC have not been evaluated. We review our experiences with TCPC to identify which risk factors may have had a considerable impact on the outcome of patients undergoing TCPC and to clarify selection criteria of high-risk Fontan candidates for TCPC.We retrospectively reviewed the medical and surgical records of all 76 patients who underwent TCPC between July 1988 and August 2000. A cross-sectional review of these patients was undertaken.There were eight early deaths and four late deaths. In a Fisher's exact test, the following variables were associated with an increased early mortality after TCPC: systemic ventricular morphology (right ventricle), ejection fraction of the systemic ventricle less than 60%, and prolonged cardiopulmonary bypass time (240 min or longer). The log-rank test demonstrated that heterotaxy syndrome, moderate to severe atrioventricular valve regurgitation, prolonged cardiopulmonary bypass time (240 min or longer), and prolonged aortic cross clamp time (70 min or longer) were associated with late mortality after TCPC. Six deaths occurred in eight (75%) patients who had six or more risk factors, whereas six deaths (9%) occurred in those who had five or fewer.Patients with complex cardiac anomalies who have six or more risk factors should be excluded from TCPC candidates.
We herein report the case of a neonate with pulmonary atresia with intact ventricular septum, right ventricular hypoplasia and severe aortic stenosis. Repetitive aortic valve surgeries in early infancy resulted in early relapse of the aortic stenosis due to the small aortic annulus, bicuspid configuration and duct-dependent pulmonary circulation. The infant underwent a successful mini-Konno procedure using a 10 mm handmade valve and bidirectional Glenn shunt at 6 months of age.
Surgical repair of total anomalous pulmonary venous connection (TAPVC) in patients with right atrial isomerism is associated with a significant risk of recurrent pulmonary venous obstruction (PVO). We evaluate the effect of sutureless repair to reduce the risk of recurrent PVO. Since November 2007, five patients, including three neonates, with right atrial isomerism underwent sutureless repair of TAPVC. The sutureless repair was used in three neonates as an initial procedure and in two infants as a procedure for postrepair PVO. Under deep hypothermic circulatory arrest or low flow cardiopulmonary bypass, pulmonary vein (PV) was incised as long as possible. The atrial wall was partially resected and anastomosed to the pericardial wall around the incised PV. There were no early deaths. No patients showed recurrence of PVO. There was one late death. Two patients underwent a bidirectional Glenn shunt after the sutureless repair. The pulmonary venous confluence was confirmed to be left open at the time of the Glenn surgery. The sutureless technique may be useful not only for postrepair PVO but also for non-operated TAPVC in neonates with right atrial isomerism.
In the modern era, advances in open-heart surgery as the rst choice for treating many congenital cardiac defects have decreased markedly as indications for palliative procedures.However, palliative surgery is still needed for the selected patients.Furthermore, the indications for palliative procedures have expanded far beyond the original concept.e main goals of palliation are to alter the hemodynamic physiology, i.e., to control the pulmonary blood ow, particularly the normally high neonatal pulmonary vascular resistance.Implantation of a systemic-pulmonary artery shunt (SP shunt) and pulmonary artery banding (PAB) are the two primary methods of palliation.e physiology of circulation incorporating an SP shunt is complex, particularly lesions within a single ventricle.Consequently, the SP shunt is still associated with a signi cant incidence of adverse outcomes.A clear understanding of the anatomy and physiology is important.PAB also continues to occupy an important place in surgical treatment for very young infants for whom cardiopulmonary bypass is associated with a great risk.e role of PAB is to reduce pulmonary blood over ow to prevent or reverse cardiac failure and to provide long-term protection against irreversible pulmonary hypertension.Moreover, several cardiac defects can bene t from early PAB, especially when preparation and training of the le ventricle is necessary.Bilateral PAB is also an attractive and e ective method for the control of pulmonary blood ow in ductal-dependent lesions.
