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.

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.

A Newly Detected Left Ventricular Mass Following A Complex Intracardiac Repair.

Appearance of unexpected masses in the chambers of the heart during cardiac surgery can be intriguing. We report the case of a mass in the left ventricle that appeared at the time of separation from cardiopulmonary bypass in a child after a complex intracardiac repair. The child presented for surgery to a tertiary care hospital in Muscat, Oman, in 2022. Prior to the surgical repair the mass was not appreciated by echocardiography. An intraventricular baffle was used to divert left ventricular blood flow towards the outflow tract, after which an intraventricular "mass" was observed. Intraoperative transoesophageal echocardiography identified the mass as a portion of the interventricular septum that was located between the inlet and outlet ventricular septal defects.

Troubleshooting During Temporary Epicardial Lead Implantation in a Child with an Erosive Twiddler's Syndrome and Multiple Sternotomies: A case report.

Temporary epicardial cardiac pacing in patients with bradyarrhythmias may be used as a bridge to implantation of a permanent pacemaker. The temporary epicardial lead placement may sometimes necessitate a sternotomy that may pose a challenge in patients who have had multiple earlier sternotomies. The difficulty in accessing the epicardium for urgent implantation of temporary epicardial pacing leads depends on the extent of adhesions in such patients. We report an 8-year-8-month-old girl with a pacemaker with an extruded pulse generator and difficult myocardial access due to 5 prior transsternal procedures. The child presented to a tertiary care hospital in Muscat, Oman, in 2021. A trouble-shooting technique was adopted to achieve temporary epicardial pacing to provide time for a course of antibiotic therapy administration. A permanent transvenous pulse generator system was implanted after 7 days of temporary pacing.

Device Closure of Superior Sinus Venosus Atrial Septal Defects: A single centre experience.

Sinus venosus atrial septal defects present a wide variety of anatomical features and are frequently associated with partial anomalous pulmonary venous drainage of one or more right pulmonary veins. Surgical correction used to be the standard treatment. In recent times, transcatheter correction of superior sinus venosus atrial septal defects has come into vogue. The transcatheter closure of these defects with covered stents at a tertiary care centre in Oman between 2018 and 2023 is reported.

The Sinus Venosus Veno-Venous Bridge: Not a septal defect.

This review provides an update on the morphology of the sinus venosus defect. It was earlier believed that a 'common wall' separated the right pulmonary veins from the superior caval vein. In the sinus venosus defects, this wall was absent. Current evidence shows that the superior rim of the oval fossa, rather than forming a second septum or representing a common wall, is an infolding between the walls of the caval veins and the right pulmonary veins. The sinus venosus defect is caused by the anomalous connection of one or more pulmonary veins to a systemic vein. However, the pulmonary vein(s) retain their left atrial connections, leading to a veno-venous bridge that allows interatrial shunting outside the oval fossa. True atrial septal defects are located within the oval fossa or in the anteo-inferior buttress, while sinus venosus defects, ostium defects and coronary sinus defects are morphologically distinct from them.

Sudden Cardiac Death in Patients Under 49 Years Including Adolescents: A single-centre study from Oman.

Objectives: This study aimed to identify the incidence of sudden cardiac death (SCD0 in adult patients under the age of 49 years, including adolescents with an out-of-hospital cardiac arrest that presented to the emergency department of a tertiary care hospital. Methods: This retrospective cross-sectional study was conducted at the Royal Hospital, Muscat, Oman, between January 2015 and December 2019. All patients with out-of-hospital cardiac arrest were enrolled. The incidence of SCD was evaluated. Information about the patient's demographic data, the site of cardiac arrest, the mode of arrival, the duration of pre-arrest symptoms and if cardiopulmonary resuscitation was performed was gathered. Survival data at 3-year follow-up was obtained. Results: A total of 117 out of 769 (15%) patients met the criteria for SCD. Male gender was predominant, with a median age of 33 years. In about 79.5% of the patients, cardiac arrest was witnessed. Only 43 patients (36.8%) received cardiopulmonary resuscitation at the arrest site; 21 patients (17.9%) had a shockable rhythm and 96 patients (82.1%) had a non-shockable rhythm. Spontaneous circulation was returned in 15 patients (12.8%). Nine patients (7.7%) were discharged from the hospital and 8 (6.8%) survived at least 36 months. Conclusion: The study findings indicate the prevalence of SCD among patients who experienced a cardiac arrest outside the hospital. Unfortunately, only a small number of patients were able to survive in the long term. By implementing preemptive screening for individuals and their families, it may be possible to prevent SCD and improve outcomes for those affected.

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.

Anaesthetic Management of Pulmonary Metastasectomy in a Patient Infected with Novel Corona Virus SARS-CoV-2.

Prioritisation of individual patients for thoracic surgeries gained importance during the current COVID-19 pandemic to ensure optimal utilisation of resources. We report a 76-year-old-male patient who presented to a tertiary care hospital in Muscat, Oman, 2020, with bilateral pulmonary metastasis. The patient underwent an urgent pulmonary metastasectomy with successful anaesthesia management in an elderly patient despite him testing positive for COVID-19 twice via real-time reverse transcription-polymerase chain reaction. At the 3-month follow-up some pulmonary abnormalities persisted but the patient was recovering well. This report discusses the rationale to perform surgery in this case and highlights the precautions taken for reducing aerosol generation during the various stages of anaesthesia.

Diagnostic lacunae and implications of an automated implantable cardioverter defibrillator implantation in a child with type 3 Long QT (LQT3) syndrome.

A diagnosis of congenital long QT interval syndrome based on history and electrocardiogram was made in a child in the absence of readily available genetic testing. A genotype 3 (LQT3) was suspected after exclusion of other variants as the child was non-responsive to beta-blocker and sodium channel blocker medication. As the child continues to show episodic bradycardia, polymorphic ventricular ectopy, and T-wave alternans, a single-chamber automated implantable cardioverter-defibrillator implantation was done successfully. This report highlights how the diagnosis of LQT3 was arrived at as well as the anesthetic challenges in the management of patients with LQTS.

Bronchial decompression following repair of absent pulmonary valve: Fine-tuning by procedural fiberoptic bronchoscopy.

Marked aneurysmal dilation of the central and branch pulmonary arteries in utero in patients with tetralogy of Fallot with absent pulmonary valve can often exhibit extrinsic compression of the trachea and bronchi. The major morbidity in these patients remains postoperative ventilation issues. This case report highlights the role of intraoperative bronchoscopy in providing guidance for obtaining optimal bronchial decompression that was achieved by an initial pulmonary arteriopexy followed by an aortopexy.

Real-time confirmation of tracheal decompression.

AIMS: The primary objective was to highlight the role of intraoperative bronchoscopic guidance during the management of central airway obstruction. MATERIALS & METHODS: A 6-month-old child presented with recurrent chest infections due to innominate artery compression of distal trachea. Aortopexy was performed under real-time bronchoscopic guidance. RESULTS: Intraoperative bronchoscopic guidance helped in confirm a successful release of the central airway obstruction. DISCUSSION: When operations similar to aortopexy are performed for release of airway obstructions, intraoperative bronchoscopic guidance is a valuable tool for confirming a successful outcome. CONCLUSION: The successful surgical management of central airway obstruction due to an innominate artery compression of the distal trachea under real-time bronchoscopic guidance in a 6-month-old child is described.

How Could Aortic Atresia With Interrupted Aortic Arch Survive? About a Neonatal Repair on Two Ventricles.

Aortic atresia with interrupted aortic arch (IAA) is an exceptional condition. In absence of associated lesion, the flow to the brain and the retrograde flow to the coronary arteries can only come from vessels on the descending aorta. This flow needs to be large enough to supply the brain and the myocardium. The only IAA type compatible with survival is type C where the flow from descending aorta could be insured by the left carotid and the left vertebral artery, branch of the left subclavian artery. Only one such a case was described in the literature. All the other surviving cases had an associated lesion including: a double aortic arch, an aorto-pulmonary window (AP window), an aberrant right subclavian artery or a bilateral patent ductus arteriosus (PDA). We report a case of aortic atresia with IAA type B associated with a double aortic arch that underwent a successful bi-ventricular one-stage neonatal Norwood-Rastelli repair and is doing well after 18 months.

Pulmonary Hemorrhage After Arterial Switch Operation.

Major aortopulmonary collateral arteries can influence the postoperative course of arterial switch operation, with heart failure being the common clinical presentation. A relatively rare presentation of an aortopulmonary collateral artery in the form of persistent postoperative pulmonary hemorrhage was encountered after an uneventful arterial switch operation in a neonate with transposition of the great arteries, intact interventricular septum, and situs inversus totalis. The aortopulmonary collateral artery was coil embolized with a successful outcome.