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.

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.

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.

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.

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.

Congenital Absence of Left Atrial Appendage Diagnosed by Multimodality Imaging.

A 70-years-old male with a history of hypertension and drug resistant paroxysmal atrial fibrillation (AF) presented to our hospital for catheter ablation to his symptomatic AF. He had no prior surgical or percutaneous procedure to close or exclude the left atrial appendage (LAA). A transesophageal echocardiography (TEE) was performed to rule out intra-cardiac thrombus prior to the ablation procedure. Although the TEE imaging at multiple acquisition angles was obtained, the LAA could not be visualized and an absence of the LAA was suspected. An absence of the LAA was confirmed using cardiac computed tomography (CT), which included 3D reconstruction. Additionally, the LAA was not visualized with left atrium (LA) angiography. During the ablation procedure, 3D voltage mapping in LA was created and no low voltage area or abnormal potential was recorded around the usual root location of the LAA. Successful electrical pulmonary vein isolation was achieved with no major complications. After six months of follow-up, the patient remained in sinus rhythm without any antiarrhythmic drugs and showed no related clinical symptoms. He stopped his anticoagulation therapy due to lack of evidence of AF recurrence and an absence of LAA. Multimodality imaging allowed us to identify the congenital absence of LAA.

Factors influencing bacteraemia in patients with isomerism and CHD: the effects of functional splenic status and antibiotic prophylaxis.

BACKGROUND: "Heterotaxy syndrome", best segregated as isomerism, is characterised by laterality defects of the thoraco-abdominal organs, causing functional impairment. In particular, the spleen is frequently affected, increasing susceptibility to bacteraemia. This study explored factors that may increase the risk of bacteraemia in patients with isomerism. METHODS: We identified patients with CHD and isomerism. Review of outpatient, inpatient, and surgical records was conducted to collect data and determine trends in the cohort. A Cox regression analysis was conducted to determine factors influencing freedom from bacteraemia (Fig 1). RESULTS: We identified 83 patients with CHD and isomerism - 17 (20%) who had documented episodes of bacteraemia with a total of 21 episodes. A majority (86%) were nosocomial. The median age at the time of bacteraemia was 4 months. Although splenic anatomy did appear to influence the risk of bacteraemia in univariate analysis, this significance was lost with multivariate analysis. None of the other factors was significantly associated in either univariate or multivariate analysis. CONCLUSION: Specific factors such as splenic anatomy, atrial appendage isomerism, and antibiotic prophylaxis status are not significantly associated with the risk of bacteraemia in patients with CHD and isomerism. Nosocomial infections represent a majority of bacteraemia in these patients.

Segregating bodily isomerism or heterotaxy: potential echocardiographic correlations of morphological findings.

BACKGROUND: Bodily isomerism, also referred to as heterotaxy, involves predominantly the thoracic organs, although other organs are usually abnormally positioned. Previously assessed on the basis of splenic anatomy, it is now understood that isomerism is better segregated on the basis of atrial appendage morphology. This allows for anticipation of associated findings. We aimed to assess the accuracy of segregation based on the morphology of the atrial appendages and other structures more easily identified by echocardiography. METHODS: We reviewed postmortem specimens of hearts from the archives at four institutions categorised as obtained from patients with "heterotaxy". The cardiac structures were analysed using sequential segmental analysis. Non-cardiac structures were also examined if available. Statistical analyses were performed to compare differences in the settings of right as opposed to left isomerism. RESULTS: Specimens were available from 188 patients. Of these, 57 had left isomerism, and 131 had right isomerism. Atrial appendages were isomeric in all patients. A coronary sinus was found only in left isomerism, whereas a terminal crest, or a Eustachian valve, was found only in right isomerism. Interruption of the inferior caval vein was associated with left isomerism, whereas totally anomalous pulmonary venous connection was associated with right isomerism. CONCLUSION: Isomerism is uniformly segregated on the basis of the morphology of the atrial appendages, itself defined by the extent of the pectinate muscles. Other features such as the presence of a coronary sinus and systemic venous return can further help with such segregation of isomerism.

Inferior and right-sided juxtaposition of the left atrial appendage with an unexpected type of inter-atrial communication.

We have re-investigated an unusual cardiac specimen with juxtaposition of the atrial appendages. The original description dates to 1962, when the autopsy was performed at the Children's Memorial Hospital in Chicago, now Ann & Robert H. Lurie Children's Hospital of Chicago. The heart was subsequently stored in the Farouk S. Idriss Cardiac Registry at the same institution. The specimen shows usual atrial arrangement, but with the morphologically left appendage juxtaposed in a rightward manner, passing behind the heart rather than through the transverse sinus so as to reach its location inferior to the morphologically right appendage. The heart also demonstrated an inter-atrial communication between the cavities of the juxtaposed left appendage and the morphologically right atrium. We provide a detailed description of the morphology, and provide images of this lesion, which to the best of our knowledge has not previously been described.

Obstructive membrane at the base of the left atrial appendage, a multi-imaging approach.

The left atrial appendage (LAA) is a small muscular extension that grows from the anterolateral wall of the left atrium, in the proximity of the left pulmonary veins. The presence of a membrane in the LAA is a rare clinical entity whose origin is not known. Its clinical implication in the genesis of atrial arrhythmias and thromboembolic risk remains unknown. We report a case of an obstructive membrane located at the base of the LAA, found incidentally in a young patient who was initially undergoing a transesophageal echocardiogram prior to an invasive treatment for atrial fibrillation.

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.

Seahorse left atrial appendage diverticula mimicking a coronary fistula.

We report a left atrial appendage diverticulum in close proximity to the left coronary artery, thereby mimicking a fistulous connection between the two structures.

Cor triatriatum or divided atriums: which approach provides the better understanding?

It is frequent, in the current era, to encounter congenital cardiac malformations described in terms of "cor triatriatum". But can hearts be truly found with three atrial chambers? The morphological method, emphasised by Van Praagh et al, states that structures within the heart should be defined on the basis of their most constant components. In the atrial chambers, it is the appendages that are the most constant components, and to the best of our knowledge, hearts can only possess two appendages, which can be of either right or left morphology. The hearts described on the basis of "cor triatriatum", nonetheless, can also be analysed on the basis of division of either the morphologically right or the morphologically left atriums. In this review, we provide a description of cardiac embryology, showing how each of the atrial chambers possesses part of the embryological body, along with an appendage, a vestibule, a venous component, and a septum that separates them. We then show how it is, indeed, the case that the hearts described in terms of "cor triatriatum" can be readily understood on the basis of division of these atrial components. In the right atrium, it is the venous valves that divide the chamber. In the left atrium, it is harder to provide an explanation for the shelf that produces atrial division. We also contrast the classic examples of the divided atrial chambers with the vestibular shelf that produces supravalvar stenosis in the morphologically left atrium, showing that this form of obstruction needs to be distinguished from the fibrous shelves producing intravalvar obstruction.

Congenital left atrial appendage aneurysm associated with a systemic embolism.

A 20 year-old woman presented with systemic embolisation. On subsequent investigation, she was diagnosed with a congenital left atrial appendage aneurysm. Few case reports are reported in the literature. This cardiac malformation presents a diagnostic challenge in patients with cardiomegaly.