Figure 1 - uploaded by Mary J Rice
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Schematic diagrams illustrating the anatomy and echocardiographic planes of section seen in left juxtapostion of the atrial appendages. A, Anterior view of the heart showing the right atrial appendage (RAA) lying posterior and to the left of the transposed great arteries (Ao = aorta; PA = pulmonary artery) and anterior and superior to the left atrial appendage (LAA). B, The right atrium and juxtaposed right atrial appendage have been unroofed. Note anteriorly that the floor of the right atrial appendage is oriented within the heart from right to left in a horizontal plane. A short-axis plane of section (plane drawn through the atria) demonstrates the abnormal anatomy visualized by a parasternal shortaxis echocardiographic scan. An inset (above) shows the resultant
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Left juxtaposition of the atrial appendages is usually associated with cyanotic congenital heart disease. Recognition of this rare anomaly is important before therapeutic or surgical procedures that involve the atrial septum can be undertaken (for example, septostomy, the Mustard or Senning operation and the Fontan anastomosis). The diagnosis of le...
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Introduction: Partial anomalous pulmonary venous connection (PAPVC) is a rare entity. Only 10% of these are left sided. An intact atrial septum is further uncommon.
Presentation of case: We present a case of left sided PAPVC with no atrial septal defect (ASD), who presented with effort intolerance and an unremarkable physical examination. Computed...
Citations
... 4 Case Reports in Cardiology RAA. Juxtaposed atrial appendages can also be confused with ASD if the abnormal septal configuration is not recognized or carefully delineated [5,9,10]. Due to the need for a timely and definitive diagnosis, further testing was conducted. ...
Several congenital anomalies of the right atrial appendage (RAA) have been described including aneurysm, herniation (in association with a pericardial defect), and left juxtaposition. The left juxtaposition of the RAA (LJRAA), first described by Birmingham in 1893 and subsequently introduced by Dixon in 1954, is usually associated with complex cardiac malformations such as obstruction of the left ventricular outflow tract. In this case report, we will describe an unusual variant of LJRAA in the absence of any other cardiac defects, which was initially misinterpreted as an aortic dissection. The correct diagnosis was made after careful reinterpretation and the use of multiple imaging modalities as highlighted.
... In this view, the anterior atrial septum is formed by the floor of the RAA as it courses to the left behind the great arteries. [12] During balloon septostomy, it is difficult to maneuver the balloon catheter across the abnormally oriented interatrial septum. The leftward positioned RAA might lead to incorrect catheter positioning, thereby making BAS a more risky procedure. ...
... The leftward positioned RAA might lead to incorrect catheter positioning, thereby making BAS a more risky procedure. [12] Procedure Once vascular access is obtained via femoral or umbilical veins, heparin at 100 IU/Kg is administered. The balloon catheter is guided into the right atrium (RA) through inferior vena cava (IVC) under echocardiographic and fluoroscopic monitoring [Video 1]. ...
An unrestricted interatrial septal orifice is required in many heart diseases in order to maintain hemodynamics. A variety of interventions have been described to achieve the above goal. This review summarizes each of them briefly. Of these, balloon atrial septostomy and interatrial stenting are the most commonly performed procedures. Echocardiography plays a pivotal role not only in the preprocedural evaluation and patient selection but also in intraprocedural guidance. The outcome of the procedure can also be evaluated immediately by echo on table allowing further attempts or changes in strategy. This review highlights the role of echocardiography in improving accuracy, feasibility, and safety of these procedures.
... On fetal evaluation, the three-vessel view may be particularly helpful in identifying both appendages on the same side of the great arteries. On transthoracic echocardiography, both fourchamber views and short-axis views are helpful (Figs. 1, 2, 3, 4, Videos 1-3) [8][9][10]. In leftward juxtaposition of the right atrial appendage, subcostal four-chamber sweeps of the atria will demonstrate the typical plane of atrial septal orientation posterior-inferiorly, but the septum "disappears" as one passes through the septum tangentially antero-superiorly, imaging the right atrium-juxtaposed appendage continuum extending "wall-to-wall." ...
Juxtaposition of the atrial appendages is known to occur in specific congenital heart lesions. Recognition of these variants is critical in balloon atrial septostomy and atrial switch operations. There remains little clinical data on the prevalence and associations of these lesions. This is the largest echocardiographic study of juxtaposition of the atrial appendages. This is a retrospective study using the Mt. Sinai echocardiogram database (EchoLAN), which contains echocardiogram reports performed or reviewed at Mt. Sinai Hospital (New York, NY) between 1992 and 2019. Each report was reviewed for associated intracardiac and extracardiac anomalies and the prevalence of juxtaposed atrial appendage among specific diagnosis, including tricuspid atresia, transposition of the great arteries, and double outlet right ventricle, was calculated. Descriptive and analytical statistics were performed as applicable, including Fisher’s exact test, with p value < 0.05 considered statistically significant. Forty-nine patients had juxtaposed atrial appendages (1.2/1000 patients): Thirty-eight had LJRAA (0.9 per 1000 patients) and eleven had RJLAA (0.3 per 1000 patients). LJRAA was seen in 22% of tricuspid atresia, 6% of d-TGA, 1% of l-TGA, and 5% of DORV. RJLAA was seen in 2% of DORV, and none of the other lesions. Of associated lesions, dextrocardia, mesocardia, transposition, tricuspid atresia, double inlet left ventricle, and pulmonary valve stenosis were more likely to be present in LJRAA, while bicuspid aortic valve and left ventricular hypoplasia were more likely to be present in RJLAA. This study reaffirms our understanding of juxtaposition of the atrial appendages, its prevalence, and clinical importance.
... One of the earliest descriptions of the echocardiographic techniques used to identify juxtaposition of the atrial appendages was published in 1983 by Rice and colleagues from the Mayo Clinic. 2 They identified the combination of the parasternal short-axis plane at the level of the great arteries and the apical 4-chamber view as most helpful in elucidating juxtaposition of the atrial appendages. In 2010, Zhang and colleagues 3 from the Shanghai Children's Medical Center retrospectively analyzed the echocardiographic findings of 10,880 patients with congenital heart disease who also underwent a cardiac catheterization. ...
... [2,3] There are several case reports of LJAA associated with complex cardiac anomalies diagnosed either at autopsy or imaged by echocardiography. [4] There are very few reports of RJAA in the literature. [5] We report three cases of RJAA in children associated with congenital cardiac anomalies. ...
... Echocardiographic imaging of JAA is reported by Rice et al. [4] We encountered RJAA in three cases of congenital cardiac anomaly identified by transthoracic echocardiography. The right atrial appendage is short, fat, and broad based and best seen in subxiphoid sagittal view. ...
Right juxtaposition of atrial appendages are rare and associated with congenital heart defects. We report 3 cases of right juxtaposition of atrial appendages (RJAA) diagnosed by transthoracic echocardiography. The sub-xiphoid sagittal view, parasternal short axis and apical four chambered views demonstrated RJAA. All 3 cases with RJAA were associated with congenital heart disease. Diagnosis was confirmed by CT angiogram and intra-operative findings in two children who underwent surgical correction. Right juxtaposition of atrial appendages is a rare association with congenital heart defects. Transthoracic echocardiographic assessment may give clues to the diagnosis.
... Prior identification has implications during cardiac catheterizations and surgery. [8] In all hearts with atrial appendage juxtapositions, the juxtaposed atrial appendage always lies above the native atrial appendage. In the commoner left juxtaposition, the native left atrial appendage lies below the juxtaposed right atrial appendage. ...
... If this is not recognized during cardiac surgery, the orifi ce of the juxtaposed atrial appendage may be mistaken for an atrial septal defect and it may be closed with a patch. [8] In the echocardiographic images of patients with juxtaposition of atrial appendages, the plane of atrial septum in the anterior part is parallel to the anterior chest wall; but in the posterior part it is normally oriented. [8] This horizontal orientation of the anterior atrial septum is the most diagnostic echocardiographic observation in this anomaly. ...
... [8] In the echocardiographic images of patients with juxtaposition of atrial appendages, the plane of atrial septum in the anterior part is parallel to the anterior chest wall; but in the posterior part it is normally oriented. [8] This horizontal orientation of the anterior atrial septum is the most diagnostic echocardiographic observation in this anomaly. Since the plane of the interatrial septum is different in these patients, occluder devices used to close secundum atrial septal defects that are located in the anterior portion of the atrial septum may have a different device orientation on echocardiography and fl uoroscopy. ...
ABSTRACT
If the atrial appendages lie adjacent to each other on same side of the great arteries, instead of encircling their roots, they are referred as juxtaposed. Right juxtaposition of atrial appendages is less common than left juxtaposition. The images demonstrate the classical radiological, echocardiographic, and surgical images of juxtaposed atrial appendages. Their clinical incidence, associations, and relevance during interventional and surgical procedures are discussed.
Keywords: Atrial appendages, juxtaposition, imaging
... It also helps in preoperative planning in Senning, Mustard, or Fontan procedures. [4] The diagnostic TTE feature of a left juxtaposed right atrial appendage is the alteration of the plane of the interatrial septum on a high parasternal shortaxis view. [4] The diagnosis of JAA can be made by ...
... [4] The diagnostic TTE feature of a left juxtaposed right atrial appendage is the alteration of the plane of the interatrial septum on a high parasternal shortaxis view. [4] The diagnosis of JAA can be made by ...
... 3,4 La cavidad auricular derecha pequeña, vista en casos de conexión ventrículo arterial anómala, crea dificultades técnicas durante la cirugía en el manejo de la aurícula; por otro lado, la posición izquierda cercana de las orejuelas ha facilitado la anastomosis entre ellas y al parecer este es un procedimiento seguro, efectivo y menos invasivo que la septectomía Blalok-Hanlon. 5,6 La yuxtaposición izquierda de la orejuela de la aurícula derecha se halla más frecuentemente en casos de anomalías troncoconales y de obstrucción en la salida de la aurícula derecha, como en la estenosis tricuspídea o en ausencia de conexión atrioventricular derecha (atresia tricuspídea clásica). 7,8 Esta condición es la que se presentó en nuestro caso; y se debe tener en cuenta al ser descubierta durante la cirugía de reparación biventricular de transposición de grandes arterias por su frecuente asociación con anomalía en la válvula tricúspide. ...
We present the first case of left juxtaposition of the right atrial appendages that has been seen at the Children's Hospital of the state of Sonora. This anomaly was associated with a complex congenital heart defect, i.e. absence of the right atrioventricular connection and transposition of the great arteries. The two-dimensional echocardiogram is the usual study to reach a clinical diagnoses, but it may also be an incidental of finding during surgery or during autopsy, in ocurred in our case. Its timely diagnosis may have implications in the surgical treatment and in therapeutic cardiac catheterization.
... 3,4 La cavidad auricular derecha pequeña, vista en casos de conexión ventrículo arterial anómala, crea dificultades técnicas durante la cirugía en el manejo de la aurícula; por otro lado, la posición izquierda cercana de las orejuelas ha facilitado la anastomosis entre ellas y al parecer este es un procedimiento seguro, efectivo y menos invasivo que la septectomía Blalok-Hanlon. 5,6 La yuxtaposición izquierda de la orejuela de la aurícula derecha se halla más frecuentemente en casos de anomalías troncoconales y de obstrucción en la salida de la aurícula derecha, como en la estenosis tricuspídea o en ausencia de conexión atrioventricular derecha (atresia tricuspídea clásica). 7,8 Esta condición es la que se presentó en nuestro caso; y se debe tener en cuenta al ser descubierta durante la cirugía de reparación biventricular de transposición de grandes arterias por su frecuente asociación con anomalía en la válvula tricúspide. ...
We present the first case of left juxtaposition of the right atrial appendages that has been seen at the Children's Hospital of the state of Sonora. This anomaly was associated with a complex congenital heart defect, i.e. absence of the right atrioventricular connection and transposition of the great arteries. The two-dimensional echocardiogram is the usual study to reach a clinical diagnoses, but it may also be an incidental of finding during surgery or during autopsy, in ocurred in our case. Its timely diagnosis may have implications in the surgical treatment and in therapeutic cardiac catheterization.
... (2) abnormal spatial orientation of the atrial septum; and (3) posterior deviation of the malaligned septum secundum to the left atrium. The first two features were described previously by Rice et al. [6] and the third by Chin et al. [2]. All suggest that two-dimensional echocardiography is reliable and reproducible in the noninvasive diagnosis of left JAA. ...
We found that echocardiography in cases of left juxtaposed atrial appendage (JAA) consistently featured a malpositioned right atrial appendage, abnormal spatial orientation of the atrial septum, and posterior deviation of the septum secundum toward the left atrium. The qualitative displacement of the septum secundum together with the posterior and leftward displacement of the right atrial appendage (with respect to the great arteries) prompted us to pose questions concerning the morphogenesis of left JAA and the implications for surgery. The importance of precatheterization and preoperative recognition of left JAA in dextrotransposition of the great arteries by echocardiography cannot be overemphasized when planning a balloon atrial septostomy or a biventricular repair.
