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.

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.

Echo Rounds: Partial Anomalous Systemic and Pulmonary Venous Return.

Anomalous drainage of a right-sided superior vena cava to the left atrium is a rare variant of systemic venous return anomalies. During the course of investigation for a heart murmur, an asymptomatic child was found to have a superior sinus venosus atrial septal defect with partial anomalous systemic and pulmonary venous drainage. Despite the right superior vena cava overriding the upper end of the atrial septum and draining into the left atrium, the child did not exhibit hypoxemia. Saline contrast transesophageal echocardiography suggested an explanation for the absence of hypoxemia.

Acquired right atrial-to-left ventricular shunt in an adult patient after several years of a senning procedure for D-transposition of great arteries.

This report describes the case of an 18-year-old post-Senning patient noted to have a residual ventricular septal defect and severe tricuspid regurgitation together with a shunt from the right atrium to the left ventricle detected several years after surgery.

VACTERL association with double-chambered left ventricle: A rare occurrence.

VACTERL association is a non-random association of birth defects of unknown etiology derived from structures of embryonic mesoderm. The common cardiac defects seen with VACTERL association are ventricular septal defects, atrial septal defects, and tetralogy of Fallot. We present a 2-year-old child with VACTERL association in whom we detected double-chambered left ventricle on transthoracic echocardiography.

Giant superior vena caval aneurysm in a post-Glenn patient.

Aneurysms of mediastinal systemic veins are extremely rare, usually asymptomatic and incidentally diagnosed during chest radiography. We describe the case of a giant superior vena caval aneurysm in a 14-year old male following Glenn surgery and discuss its complications and management.

Early migration of an Amplatzer muscular ventricular septal defect occluder device causing severe tricuspid valve obstruction.

Percutaneous closure of perimembranous and muscular ventricular septal defects is becoming well established worldwide. We present the case of a 15-year-old girl with postoperative tetrology of Fallot and a residual ventricular septal defect that was closed with Amplatzer device complicated by early device migration and severe tricuspid valve obstruction.

Coronary Artery Fistula with Heart Failure in Early Infancy.

Heart failure in early infancy is commonly caused by lesions leading to pulmonary over circulation secondary to left-to-right shunt. This case report describes an unusual cause of significant left-to-right shunt in a 2 months old infant presenting with congestive heart failure, which was diagnosed with transthoracic echocardiography. In this infant, transthoracic echocardiography with Doppler color flow mapping allowed direct visualization of a large right coronary artery to right ventricular fistula that was surgically corrected successfully.