Systemic venous anomalies in a child with a vein of Galen.

We describe our findings in a child with a vein of Galen malformation, in whom the right superior caval and the azygos veins drained into the roof of the morphologically left atrium. A persistent left superior caval vein drained into the morphologically right atrium through the coronary sinus. The additional presence of dual brachiocephalic veins permitted the deployment of a multifunctional ventricular septal defect occluder device to occlude the right superior caval vein, correcting the right-to-left shunt. This also prevented azygos venous drainage into the left atrium.

Surgical Repair of Common Arterial Trunk With Ventriculoarterial Septal Defect and Dual Orifice Truncal Valve.

We present a case of a rare example of a ventriculo-arterial septal defect found in a patient with a common arterial trunk, with balanced aortic and pulmonary components, but with separate valvar orifices within the common truncal valve. We managed the lesion using a two-patch approach. Performing a palliative procedure to relieve the elevated right ventricular pressure aided in the preservation of the pulmonary component of the common valve. We validated the success of the technique using postoperative computerized tomography and four-dimensional flow magnetic resonance imaging.

Complex Atrial Baffling Procedures in Left Isomerism With Right- and Left-Hand Topology.

We describe complex atrial baffling procedures in the setting of left isomerism with right-hand as opposed to left-handed ventricular topology. An appropriate understanding of the connections of the systemic and pulmonary veins, along with the internal atrial anatomy, as revealed using 3D printing, allowed for successful biventricular repair.

An Unusual Presentation of Double-Outlet Right Atrium.

We describe an unusual example of double-outlet right atrium with separate atrioventricular junctions. The straddling and overriding tricuspid valve had two orifices, and the mitral valve was morphologically normal. An appropriate understanding of the morphology of the atrioventricular junctions, the valves, and the subvalvar apparatus, along with the location of the atrioventricular conduction axis, allowed for successful biventricular repair.

Co-axial Endotracheal Tube Technique for Achieving One Lung Ventilation in a Toddler With a Space Occupying Malignant Tumor of the Right Lung.

Achieving one-lung ventilation in pediatrics is often challenging. In caring for these patients, the anesthesiologist must consider the child's age and size, underlying tracheobronchial anatomy, equipment availability, urgency of procedure, and as well as the experience level of the anesthesiologist. This report describes a "tube-inside-tube" technique that was adopted for providing one-lung ventilation in a toddler. The method described here involved railroading a smaller endotracheal tube over a flexible intubation video endoscope into the left mainstem bronchus coaxially through a larger endotracheal tube placed in the trachea. The technique achieved effective left-lung ventilation and isolation of the operative right lung during surgical resection of a malignant mesenchymal tumor. On completion of the procedure, double-lung ventilation could be established through the endotracheal tube in the trachea after the retraction of the video endobronchial tube.

Clinical Variables Specific to Timing of Tracheal Extubation Following Pediatric Cardiac Surgery.

BACKGROUND: The primary objective of this study was to identify specific factors in pediatric cardiac surgical patients when tracheal extubation was performed on the operating table after completion of open-heart surgery (Group-1), postoperatively in the intensive care unit within 6 h (Group-II) or after 6 h (Group-III). The causes of failed extubation, the presence of chromosomal disorders in addition to arterial blood gas analysis parameters at the time of tracheal extubation, and the duration of intensive care unit stay were also evaluated in each group. METHODS: In addition to the three groups, Groups I and II were combined as a "fast-track" extubation group. The demographic data, Risk Adjustment for Congenital Heart Surgery (RACHS-1) score, the Society of Thoracic Surgeons - European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Category (STAT Mortality Category), cardiopulmonary bypass (CPB) time, aortic cross-clamp (ACC) time, and vasoactive-inotropic score (VIS) at the time of tracheal extubation along with data related to secondary objectives were recorded for each patient. RESULTS: A significant association was found by bivariate analysis between clinical variables and for both operating table and fast-track extubation in terms of age, weight, RACHS-1 score, STAT category, CPB and ACC time, and VIS. A multivariate-adjusted analysis showed weight, lower STAT category, CPB time, and VIS were independent predictors for operating table and fast-track extubation. CONCLUSIONS: Younger age, lower weight, higher RACHS-1, STAT category, and VIS, along with longer CPB and ACC, are associated with delay in the timing of tracheal extubation in pediatric cardiac surgical patients.

Cause of profound hypoxemia following a bilateral bidirectional Glenn shunt: Clue suggested by agitated saline echo contrast.

Following a bilateral bidirectional Glenn shunt, a child had persistent hypoxemia. Agitated saline contrast injection into the jugular vein during transesophageal echocardiography displayed a rapid appearance of saline particles in the cardiac chambers suggesting the presence of pulmonary arteriovenous malformations. However, the clinical picture was not in agreement and an angiographic contrast injection during an immediate cardiac catheterization revealed the underlying pathology which was immediately corrected surgically.

Surgical repair of interrupted right-sided cervical aortic arch with hypoplasia of the descending thoracic aorta in a child with PHACE syndrome.

Obstruction of a right cervical aortic arch in association with hypoplasia of the descending aorta is a rare congenital cardiac malformation. We report the case of a 6-month-old boy with posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies and eye anomalies (PHACE) syndrome and interruption of a right-sided cervical aortic arch. The descending thoracic aorta in the child had a long hypoplastic segment and the patient also had small ventricular septal defect and pulmonary valve stenosis. The surgical technique of reconstruction of the aortic arch and the descending thoracic aorta through a median sternotomy is described.

Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.

So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect. It is the anomalous pulmonary venous connections that then create a venovenous bridge, which permits the systemic venous tributaries to drain into the morphologically left atrium, even though they may be predominantly connected to the right atrium. More rarely, it is feasible for the primary atrial septum to develop so as to leave the systemic venous sinus in direct connection with the body of the morphologically left, rather than the morphologically right, atrium. We report a series of patients potentially falling into the category of anomalous systemic venous connections. The findings show a spectrum from partially to totally anomalous connections, with some better interpreted on the basis of anomalous drainage. Included in our cases, nonetheless, is an autopsied example of totally anomalous systemic venous connection produced by an abnormal location of the primary atrial septum. We discuss the potential morphogenesis for this finding. We emphasize the distinction that needs to be made between anomalous systemic venous connections and anomalous systemic venous drainage.

Propofol and Kearns-Sayre Syndrome: An idiographic approach.

With the focus on an idiographic approach whereby the observations incorporated the various dimensions of individual functioning 'top-down' to 'bottom-up', this case report describes the successful management of a 14-year-old girl with Kearns-Sayre syndrome and Dyggve-Melchior-Clausen disease requiring a transvenous permanent pacemaker implantation for complete heart block. The patient presented to a tertiary care centre in Muscat, Oman, in 2023 seeking consultation. The current idiographic approach appears to have a heuristic value for 2 interrelated reasons. Firstly, it is unlikely that even tertiary care units can accrue such rare presentations and scrutinise them under nomothetic approach. Secondly, by employing the idiographic approach that is capable of examining each case in-depth, the aspiration for good health and well-being may come to the forefront. To the best of the authors' knowledge this is the first published idiographic report in anaesthesia care.