Can Isolated Discordant Atrioventricular Connections Survive Without Interatrial or Interventricular Shunt?

Clinical presentation of isolated discordant atrioventricular connections is akin to transposition of the great arteries. In the absence of a significant intracardiac shunt, profound cyanosis is expected at birth. We report one such 5-month-old infant who had only mild cyanosis. The left-sided tricuspid valve straddled the interventricular septum with a closed interventricular communication, a type of "Double Outlet Left Atrium with three atrioventricular valves," which provided the necessary "left to right" shunt while severe regurgitation through the straddling part and a patent ductus arteriosus provided the effective pulmonary blood flow.

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.

Aortic root abscess and anomalous aortic origin of right coronary artery in a 5-year-old child.

BACKGROUND: Aortic root abscess is a rare complication of infective endocarditis in children. CASE REPORT: A 5-year-old boy with infective endocarditis of aortic valve and an anterior aortic root abscess was found to have anomalous aortic origin of right coronary artery from the left coronary sinus on computed tomography scan with contrast. RESULT: He was managed surgically by "patch and prosthesis" approach and required aortic root enlargement. Since anomalous aortic origin of right coronary artery is a relative contraindication for a Konno-Rastan aortic root enlargement, a Manouguian procedure was performed. CONCLUSION: Anterior aortic root abscesses are rare, the co-existence of anomalous aortic origin of right coronary artery is rarer still and such cases requiring aortic root enlargement are possibly best served by a Manouguian procedure.

Concordant Ventriculoarterial Connections With Abnormally Related Arterial Trunks (Anatomically Corrected Malposition) Associated With Right Ventricular Outflow Tract Obstruction by Accessory Valvar Tissue.

Anatomically corrected malposition of the great arteries is a rare conotruncal anomaly that is often confused with congenitally corrected transposition of the great arteries. This case report describes the successful surgical management of an adolescent with severe right ventricular outflow obstruction caused by accessory tricuspid valve tissue in the presence of situs inversus with atrioventricular and ventriculoarterial concordance with malposed great arteries (anatomically corrected malposition) {I,L,D}.

Double outlet right atrium-A "bridge" across the bridging leaflets.

INTRODUCTION: Mal-alignment between the inter-atrial septum (IAS) and the inter-ventricular septum (IVS) during cardiac embryogenesis results in abnormal atrioventricular (AV) connections ranging from doubleoulet left atrium (DOLA) to double outlet right atrium (DORA)1. CASE REPORT: We report DORA resulting from partial override of the interatrial septum (IAS) across the cleft between the bridging leaflets of the left atrioventricular (AV) valve. RESULTS: Successful surgical management with preservation of the orifice is described, leading to an unusual type of double orifice left AV valve. DISCUSSION: The mal-alignment of the interatrial septum, resulting in it bridging the bridging leaflets, leads to an additional orifice between the RA and the LV and a unique form of right to left shunt. DORA can cause cyanosis depending on the size of the shunt and the compliance of the ventricles. Our patient was acyanotic, possibly due to the small size of the orifice and low LV compliance secondary to hypertrophy of the LV. CONCLUSION: our case adds to the spectrum of atrial septal mal-alignment variant of DORA in the setting of AVSD without atrial and ventricular components, with RA to LV communication occurring via the smaller orifice of a left atrioventricular valve divided by the abnormal insertion of a partially overriding, mal-aligned atrial septum; a mechanism not described previously - the 'bridge' across the bridging leaflets!

A rare cause of wheezing in a child with pulmonary atresia.

The determination of the exact cause for symptomatic airway obstruction in pediatric patients not responding to medication can be a clinical dilemma. Very rarely external vascular compressions can produce airway obstruction symptoms unresponsive to usual bronchodilator medications. The successful management of a child with pulmonary atresia and an innominate artery compression syndrome with respiratory compromise due to tracheal compression is described.