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.

Heart in Heart: A Case Report of Giant Left Atrial Appendage Aneurysm.

Left atrial appendage aneurysm (LAAA) is a rare cardiac anomaly. The cause mostly due to congenital, but can be acquired also. Patient may remain asymptomatic or may present with variable symptom. It can predispose to hazardous adverse events, including atrial fibrillation, myocardial infarction, cardiac dysfunction and life-threatening systemic thromboembolism. Simple imaging, electrocardiography and echocardiography can diagnose this rare cardiac anomaly. We are reporting a case who presented to us at 5 years of age with palpitation, chest pain and dizziness with arrythmia that developed one month back; he visited our outpatient department of the National Heart Foundation Hospital & Research Institute Hospital, Dhaka, Bangladesh on 13th February 2020. We diagnosed left atrial appendage aneurysm with mitral valve prolapse with atrial arrhythmia thereafter surgical resection of aneurysmal part along with mitral valve annuloplasty done by mid sternotomy and maze therapy. Postoperative period was uneventful and discharged after 6th post operative day.

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.

Juxtaposition of the Atrial Appendages: A Large Echocardiographic Series.

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.

The left atrial appendage congenital aneurysm in a two-year-old child: a case report.

The left atrial appendage congenital aneurysm is an extremely rare heart defect. The entity can remain asymptomatic for a long time; however, it may lead to life-threatening morbidity or death.

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.

Non-obstructive membrane of left atrial appendage orifice: first paediatric report.

Left atrial appendage membrane is a rare anomaly. Its implications on clinical course are unknown. We report the first paediatric case in association with a congenital heart disease.

Isomerism of the atrial appendages: morphology and terminology.

BACKGROUND: Our aim is to identify the pathognomonic anatomical markers and the best terminology to describe the cardiac malformations associated with absent or multiple spleens, which are known as asplenia or polysplenia syndromes or isomerism. MATERIALS AND METHODS: We have reviewed 65 hearts with isomerism of atrial appendages of the Anatomical Collections of Congenital Heart Disease, Institute of Pathological Anatomy of the University of Padua consisting of 1800 specimens. All the hearts were classified according to sequential segmental classification. RESULTS: The incidence of isomerism was 3.6%. Of the total, 45 hearts with isomerism of right atrial appendages showed bilateral trilobed lungs, short bronchi, and absent spleen. The atrioventricular junction was univentricular in 49% of cases with a common atrioventricular valve in 91%. Pulmonary atresia and double outlet right ventricle were present in 40% and 47% of cases, respectively. Total anomalous pulmonary venous drainage and absent coronary sinus were always present. In 20 hearts with isomerism of left atrial appendages, bilateral bilobed lungs with long bilateral bronchi and multiple spleens were always found. The biventricular atrioventricular connection was present in 65% with a common valve in 30% of the hearts. The ventriculoarterial connection was concordant in 45% of cases, and aortic atresia and pulmonary atresia were both noted in 15% of each. An anomalous symmetric pulmonary venous drainage was observed in 65% of the hearts and interruption of inferior vena cava was found in 75% of cases. CONCLUSIONS: We believe that the appropriate terminology is based on the symmetrical morphology of the atrial appendages. The absence of the coronary sinus and the total anomalous pulmonary venous drainage are the markers of isomerism of the right atrial appendages. Symmetric pulmonary venous drainage and interruption of inferior vena cava are the markers of isomerism of left atrial appendages. In recent years, thanks to the improvement of clinical diagnosis and of surgical techniques these patients have the possibility to survive to adult age.

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.

Absent left atrial appendage: case report and review of the literature.

Congenital absence of left atrial appendage (LAA) is an extremely rare condition and is usually diagnosed incidentally in imaging intended for other purposes. Herein, we report a rare case of absent left atrial appendage in an 80-year-old gentleman who was candidate for radiofrequency catheter ablation procedure for atrial flutter rhythm in whom we observed the absence of left atrial appendage in echocardiographic examination. Computed tomography angiographic examination performed in the evaluation course of the patient was also confirmative of this finding. As there is no data on anticoagulating of patients with absent left atrial appendage, after successful radiofrequency catheter ablation procedure, we continued rivaroxaban per guidelines. The results of a second imaging modality and a thorough medical history are critical for diagnosis of absent left atrial appendage. These steps are required to rule out imitating conditions such as prior surgical/percutaneous exclusion, unusual anatomical features or flush thrombotic exclusion of left atrial appendage. In this case report, we also provide a brief review of the characteristics of 17 cases that have been reported in the literature so far.

Unusual interatrial fistula discovered during device closure of an atrial septal defect.

Atrial septal defect (ASD) closure is a common reason for referral to the cardiac catheterization laboratory. We report a case in which a relatively large Qp:Qs of 1.85:1 was demonstrated in the face of a small secundum ASD. This led to further investigation, ultimately leading to the discovery of an unusual atrial fistula. Rare cases of interatrial tunnels have been described in the literature, however, this is a unique case of a left atrial appendage to right atrial appendage fistula in the setting of a right juxtaposed left atrial appendage.

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 curious case of an absent left atrial appendage.

The left atrial appendage (LAA) in the setting of non-valvular atrial fibrillation (NVAF) is the predominant location for intracardiac thrombus formation. An absent LAA is a very rare congenital cardiac anomaly. We present a case of a 79-year-old female with NVAF, high CHADS2VASC2 score, and high bleeding risk who presented for elective Watchman™ left atrial appendage closure device implant. A pre-procedural transesophageal echocardiography showed an absent LAA. This finding was confirmed with cardiac computed tomography and a left atrial angiogram. The patient was discharged on medical therapy with close outpatient follow-up.