Juxtaposition of the Atrial Appendages: A Clinical Series of 22 Patients

Abstract

Because the outcome of a large clinical series of patients with juxtaposition of the atrial appendages (JAA) has not previously been reported, a retrospective study was performed on patients diagnosed with JAA at a tertiary medical center. Patients with JAA were identified through a computerized database search, and echocardiograms and medical records of patients with JAA were reviewed. Twenty-two patients with JAA were identified, with an overall incidence of 0.28%. All but 2 patients were diagnosed prospectively with JAA by echocardiography. The lesion-specific incidences and associated lesions were similar to those of large autopsy and surgical series. Abnormal conotruncal anatomy was more frequently seen with juxtaposition of the right atrial appendage (JRAA) vs juxtaposition of the left atrial appendage (JLAA) (14/15 vs 4/7), as was atrial outlet obstruction (6/15 vs 2/7). JLAA was more frequently associated with complex atrioventricular anatomy (3/7 vs 1/15). Patients with JAA underwent single ventricle palliation in 11/22 cases with 6 deaths; biventricular repair was performed in 8/22 cases with no deaths. Surgical outcomes for patients with JRAA and JLAA were similar, and survival was predominantly influenced by suitability for biventricular repair.

Echocardiographic Assessment of Juxtaposition of the Right Atrial Appendage in Children with Congenital Heart Disease

“…Juxtaposition of the atrial appendage is a rare congenital malformation, including JRAA and JLAA 1,2 . Lai and colleagues reported 15 cases (68.2%) with JRAA and 7 cases (31.8%) with JLAA 3 . In our study, the prevalence of JRAA was 0.30% in patients with congenital heart disease.…”

“…Cardiac catheterization and angiographic examination were performed in all 10,880 children with congenital heart disease using GE LC‐LP digital subtraction angiography (DSA) (General Electric Medical Systems, Seattle, WA, USA). Left atrial morphology was assessed following pulmonary artery angiography, and right atrial morphology was assessed following selective right atrial angiography 3 . MRI scans were performed in 6,050 of the children.…”

“…In 1996, juxtaposition of the atrial appendages was classified by Van Praagh and colleagues into either juxtaposition of the right atrial appendage (JRAA) or juxtaposition of the left atrial appendage (JLAA) 1,2 . The prevalence of juxtaposition of the atrial appendages has been reported to be 0.28% in patients with congenital heart disease 3,4 . This rare anomaly is usually an incidental finding during surgery or angiocardiography for other cardiac abnormalities and does not typically cause significant hemodynamic disturbances.…”

Left Atrial Appendage Occlusion for Stroke Prevention

“…(12) In a study by Veinot et al which included 500 autopsy cases, 54% of LAA had two lobes and 34% had > 3three lobes with the lobes often lying in different planes. (11) Congenital juxtaposition and isomerism of the atrial appendages are associated with rare congenital anomalies (13)(14)(15). There are similarities between LAA myocardial and ventricular myocardial cells, and the LAA cells resemble skeletal muscle.…”

Multimodality Imaging Guidelines of Patients with Transposition of the Great Arteries: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography

“…26 Conversely, >50% of patients with juxtaposition of the atrial appendages have TGA. 26,28 Because of its unusual appearance, leftward juxtaposition sometimes mimics a moderate or large atrial communication. In leftward juxtaposition of the atrial appendages, a subxiphoid frontal (long-axis) sweep shows the atrial septum in a perpendicular orientation to the diaphragm ( Figure 7, Video 8; available at www.onlinejase.com) (in the normal heart, the atrial septum is parallel to the diaphragm in this view).…”