Long-Term Surgical Outcomes of Patients With Isomeric Right and Left Atrial Appendages.

Objectives: To compare the long-term outcomes of biventricular, univentricular, and so-called one-and-one-half ventricular repairs in patients with left and right isomerism. Methods: Surgical correction was undertaken, between 2000 and 2021, in 198 patients with right, and 233 with left isomerism. Results: The median age at operation was 24 days (interquartile range [IQR]: 18-45) and 60 days (IQR: 29-360) for those with right and left isomerism, respectively. Multidetector computed-tomographic angiocardiography demonstrated more than half of those with right isomerism had superior caval venous abnormalities, and one-third had a functionally univentricular heart. Almost four-fifths of those with left isomerism had an interrupted inferior caval vein, and one-third had complete atrioventricular septal defect. Biventricular repair was achieved in two-thirds of those with left isomerism, but under one-quarter with right isomerism (P < .001). Hazard regression for mortality revealed odds for prematurity at 5.5, pulmonary atresia at 2.81, atrioventricular septal defect with a common valvar orifice at 2.28, parachute mitral valve at 3.73, interrupted inferior caval vein at 0.53, and functionally univentricular heart with a totally anomalous pulmonary venous connection at 3.77. At a median follow-up of 124 months, the probability of survival was 87% for those with left, and 77% for those with right isomerism (P = .006). Conclusions: Multimodality imaging characterizes and delineates the relevant anatomical details, facilitating surgical management of individuals with isomeric atrial appendages. Continuing higher mortality despite surgical intervention in those with right isomerism points to the need for the reassessment of strategies for management.

Left atrial appendage aneurysm enlarged in the neonatal period.

We describe a newborn with a congenital left atrial appendage aneurysm. The aneurysm size did not change prenatally. However, it rapidly enlarged after birth. MRI was useful for assessing the aneurysm extent and exact size, and for diagnosis. Respiratory distress and feeding difficulties appeared, and surgery was performed. These symptoms disappeared post-operatively. The patient is alive without complications or recurrence.

A giant right atrial appendage aneurysm in an infant: A case report.

Right atrial appendage aneurysms (RAAAs) are extremely rare in cardiac anomalies. According to the literature, a few dozen cases have been reported thus far, among which only four cases were infants or neonates. Here, we report an infant with a giant RAAA and severe symptoms. The RAAA was diagnosed by echocardiography and surgically resected under cardiopulmonary bypass (CPB). The role of transesophageal echocardiography was very important during aneurysm resection surgery, which helped surgeons to plan surgical procedures during surgery and evaluate the surgical effect postoperatively.

The Apical View Sign for Easier Identification of Left Juxtaposition of the Right Atrial Appendage.

Left juxtaposition of the right atrial appendage (RAA) is a rare congenital cardiac malformation, where both atrial appendages are located leftward of the great arteries. Despite well-described echocardiographic markers, this diagnosis remains challenging to establish, especially for the novice imager. We describe the apical view sign in three patients with juxtaposed RAA. We noted that the normal left atrial appendage was visualized from the apical four-chamber or equivalent coronal view at the level of the atrioventricular valves.

Left Atrial Appendage Aneurysm Characterized by Multimodal Imaging.

We present a four-year-old female with an incidental finding of a congenital left atrial appendage aneurysm who underwent surgical resection with excellent results. This case highlights the importance of multimodal imaging in the diagnosis and characterization of this rare condition.

Right Atrial Appendage Aneurysm Resection to Cure Aneurysm-Related Atrial Tachyarrhythmia.

Congenital right atrial appendage aneurysm (RAAA) is an extremely rare malformation that can coexist with atrial tachyarrhythmia. There is no consensus on treatment for this condition. This research aimed to investigate the clinical characteristics and efficacy of surgical resection to treat atrial tachyarrhythmia originating from RAAA in children. Four RAAA children diagnosed with atrial tachyarrhythmia at the age of 1-5.25 years weighing 8.3-17.1 kg were discussed in this retrospective study. Patients underwent various treatments, included electrocardiogram (ECG) and echocardiography, antiarrhythmic medication therapy, radiofrequency catheter ablation (RFCA), surgical resection of RAAA and pathological examinations. The results from these treatments along with clinical features of patients were analyzed. The incidence of RAAA in patients with atrial tachycardia originating from the right or left atrial appendages (RAA or LAA) was 7.3% (4/55). The prevalence of RAAA in the RAA was 12.5% (4/32). Atrial tachyarrhythmia was identified both prenatally (26 and 36 weeks of gestational age) and postnatally (1 and 4 months after birth), with two patients per group, respectively. The RAAAs condition in two patients with atrial tachycardia (AT), concomitant atrial flutter (AF) and atrial fibrillation (Af) was identified using echocardiogram. Although, RAAA in two patients with mono AT was unidentified in echocardiogram and failed to be identified in the procedure of RFCA, RAAA was confirmed during surgical resection of the RAA. Multiple pre-surgical antiarrhythmic medications combined therapy used to treat all four patients showed either no effect at all or was only partially effective. The original atrial tachyarrhythmia was successfully abolished after RAAA surgical resection in four patients. AT originating from new foci was established in two patients post-surgically. The conditions of these two patients were successfully reverted and normal sinus rhythm maintained in the application of antiarrhythmic medications. These results confirmed the efficacy of RAAA surgical resection. The pathology study showed cystic dilation in parts of the atrial cavity, fibrosis of the cyst wall, generalized fibrosis of atrial myocardium with myocardium atrophy and cystic dilation. RAAA is prone to misdiagnosis by echocardiogram. Atrial tachyarrhythmia in patients with RAAA is usually resistant to antiarrhythmic medication therapy and RFCA. Surgical resection of RAAA is a safe and effective option that is minimally invasive.

A rare giant congenital left atrial appendage aneurysm in a 1-day-old newborn.

Congenital left atrial appendage aneurysm (LAAA) is a very rare condition and occurs as a result of congenital dysplasia of musculi pectinate. These patients may be asymptomatic and/or may present with dyspnea, and thromboembolic events. The most common complications are life-threatening thromboembolic events and supraventricular tachyarrhythmias. Transthoracic echocardiography plays a very important role in the diagnosis of LAAA. Herein, we present a rare case of giant congenital LAAA.

Five chambered heart: case of a huge left atrial appendage aneurysm.

Aneurysmal enlargement of the left atrial appendage is an extremely rare pathology and can predispose to adverse events, including cardiac arrest, respiratory distress, arrhythmia, heart failure, systemic thromboembolism, or rupture. It is usually diagnosed incidentally or after the occurrence of atrial tachyarrhythmias or thrombotic events in the second to fourth decades of life. We describe a rare case of a symptomatic giant congenital left atrial appendage aneurysm (LAAA) in a 26-year-old man presenting with neurologic event, in whom surgical resection of the aneurysm was successfully performed. This is the largest LAAA reported in the literature so far.

Isolated Right Juxtaposition of the Atrial Appendages in Adult With Bicuspid Aortic Valve Stenosis.

We report the case of a 30-year-old male who had symptomatic bicuspid aortic valve stenosis. Operative findings unexpectedly revealed right juxtaposition of the atrial appendages. This is a rare association, as juxtaposition of atrial appendages is generally associated with more complex forms of congenital heart disease. The patient underwent successful surgery with uneventful postoperative course.

Inverted left atrial appendage masquerading as a left atrial mass.

An inverted left atrial appendage after cardiac surgery is a rare finding and can be misinterpreted as a thrombus, mass, or vegetation. We report a case where intraoperative transesophageal echocardiography assisted in making an accurate diagnosis.

LA mass shadow after removal of LA myxoma.

Inverted left atrial tissue is mostly identified when the heart is empty while coming off bypass. During echocardiography a new, echodense mobile mass in the left atrium without attachment is visualized. Such a picture can easily produce not only confusion in the diagnosis but also mitral valve obstruction, hemodynamic instability, and a possibility of appendage necrosis. Inability to identify such inverted tissue can lead to unwanted interventions with additional cardiopulmonary bypass time.

Left Isomerism of the Atrial Appendages With Sinus Venosus Defect and Anomalous Systemic Venous Drainage.

We present a case with left isomerism, interruption of the inferior caval vein, anomalous systemic venous drainage with partially anomalous pulmonary venous drainage, and a sinus venosus type of atrial septal defect in the absence of a right superior caval vein. This report is of interest because of a rare combination of these anomalies, which was accurately diagnosed and successfully repaired.

Atrial Switch Operation in a Late Presenter With d-Transposed Great Arteries, Juxtaposed Atrial Appendages, and Bilateral Superior Caval Veins.

A 26-year-old patient with d-transposition of great arteries (d-TGA), bilateral superior vena cava, and juxtaposed atrial appendages underwent a successful atrial switch operation. It is extremely uncommon to encounter a previously unpalliated patient with d-TGA at this age. Unusual morphologic features in this patient necessitated technical modifications to successfully accomplish an atrial switch procedure.

Surgical Treatment of Dextroversion, Isolated Persistent Left Superior Vena Cava Draining Into the Left Atrium.

We report a rare case of dextroversion accompanied with atrial septal defect (ASD), persistent left superior vena cava with absent right superior vena cava in a four-year-old male. A polytetrafluoroethylene (PTFE) graft as an extracardiac conduit was used to connect the persistent left superior vena cava (PLSVC) with the right atrial appendage.