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A left-sided hepatic vein connected to the coronary sinus is reported in a case of a 22-month-old boy with isomerism of the left atrial appendages, complete atrioventricular septal defect and azygous continuation of the inferior caval vein. The diagnosis of the anomalous hepatic vein was made intraoperatively and successful biventricular repair has been accomplished. To the best of our knowledge, this is the first communication on this peculiar entity diagnosed during life, notwithstanding Nabarro's description of a similar autopsy finding in 1903. Aspects of the development of this rare entity are discussed.
We describe two patients with right isomerism, corrected with a fenestrated Fontan operation, who suffered severe progressive cyanosis. Cardiac catheterisation in both revealed a massive right-to-left shunt from the inferior caval vein, through the liver, to a hepatic vein draining directly to the left side of the intra-atrial baffle. The anomalous vein was successfully ligated in both patients.
We report obstruction of the reconstructed left ventricular outflow tract diagnosed 18 months after an arterial switch operation that included closure of a ventricular septal defect. We speculate that either turbulences created by the patch used to close the ventricular septal defect, or postoperative modifications of ventricular geometry, were responsible for producing this unusual complication.
the techniques of computational fluid dynamics are among the most powerful tools available to engineers dealing with the motion of fluids and the exchange of mass, momentum, and energy. they have recently been shown to have an increasing number of applications to the human cardiovascular system, including the fluid dynamics of surgical reconstruction of congenitally malformed parts of the cardiovascular system. in vitro models are the alternative laboratory tools with which to study fluid dynamics. the advantages of computational fluid dynamics over the in vitro models are the easy quantification of haemodynamic variables, such as rates of flow, pressure, and distribution of shear stress, and changes in geometric and fluid dynamics parameters. furthermore, using computational fluid dynamics allows the development of three-dimensional models to reproduce both the complex anatomy of the investigated region and the details of the surgical reconstruction, especially with the recent developments in magnetic resonance imaging. on the basis of the results, it is possible quantitatively to evaluate the surgical correction. this technology, which benefits greatly from the continuous improvement in hardware and software, enables cardiovascular experts and bioengineers to look at the fluid dynamics of various cardiovascular regions with increasing sophistication.
among the operations which lead to a partial rerouting of the systemic venous return into the pulmonary arteries, the bidirectional cavopulmonary anastomosis is frequently utilised in the staged surgical management of patients with functionally univentricular hearts. whereas some surgeons insist on closing any other source of pulmonary blood flow while performing a bidirectional cavopulmonary anastomosis, others maintain patency of either a stenosed native pulmonary outflow tract, or of a systemic-to-pulmonary arterial shunt. it remains controversial as to whether an additional source of pulmonary arterial blood flow can safely and usefully be left at the time of the bidirectional cavopulmonary anastomosis to increase systemic saturation, or whether it should be eliminated to reduce the volume load on the ventricle.
the norwood procedure involves three separate stages of operative corrections. the first stage involves re-fashioning the pulmonary trunk into a neo-aorta so that it is possible to establish an unrestricted systemic circulation. an interpositional, or systemic-to-pulmonary arterial, shunt is then created between the neo-aorta and the pulmonary arteries to allow pulmonary perfusion and gas exchange. two of the available options for the systemic-to-pulmonary shunt are the central shunt and the right modified blalock-taussig shunt. in the setting of a central shunt, pulmonary perfusion is derived from a conduit placed between the pulmonary arterial bed and the neo-aorta whereas, in the modified blalock-taussig shunt, the conduit is interposed between one of the pulmonary arteries and the brachiocephalic artery. in subsequent stages, pulmonary perfusion is provided directly by deoxygenated blood. this is achieved by connecting, first, the superior caval vein, and then the inferior caval vein, to the pulmonary arteries. it is usually during the second stage that the systemic-to-pulmonary shunt is removed.
The appropriate timing of intervention in patients with chronic aortic incompetence allows recovery of ventricular function. We sought to determine the optimal timing of the Ross procedure for chronic aortic incompetence in young patients. We retrospectively analysed case notes, and measured pre- and postoperative echocardiographic indexes of left ventricular function, in patients who had undergone the Ross procedure for chronic aortic incompetence. Methods and results: We found 21 patients with preoperative and postoperative data suitable for analysis. Their age at operation ranged from 5.6 to 26 years, with a median of 13.8 years, and the duration of follow-up was from 0.5 to 6.8 years, with a median of 2.4 years. The preoperative left ventricular end-diastolic dimension was converted to a z-score, and this was used as a threshold to divide the population. Using the threshold of a preoperative left ventricular z-score of more than 3 to divide the population did not show any difference in postoperative parameters of left ventricular function. Significant differences were found postoperatively, however, in both the left ventricular z-score and the ratio of left ventricular end-diastolic radius to posterior wall thickness in diastole, with a cutoff preoperative threshold z-score greater than 4. Conclusion: The increase in the ratio of left ventricular end-diastolic radius to the thickness of the posterior wall in diastole would suggest that there is disruption of left ventricular short axis architecture and myocardial contractile function when intervention is postponed. The significantly larger left ventricular dimension at end-diastole, despite the reduction in volume loading post surgery, may also demonstrate irreversible structural changes. Our data would suggest that recovery of left ventricular function is less likely when the left ventricular z-score has reached the value of 4, and that, ideally, intervention should be performed when the z-score approaches or exceeds 3.