Cardioneuroablation for pediatric patients with functional sinus node dysfunction and paroxysmal atrioventricular block.

INTRODUCTION: Severe transitory episodes of bradycardia with subsequent syncope in children are common, and generally portend a benign prognosis. Rarely, patients may experience prolonged asystolic episodes secondary to significant sinus pauses (SP) or paroxysmal atrioventricular block (AVB). Cardioneuroablation (CNA) is a catheter-based intervention, used to identify and ablate the epicardial ganglionated plexi (GP), which results in disruption of the vagal-mediated parasympathetic input to the sinus and atrioventricular node. OBJECTIVE: Describe the methodology and role of CNA for treatment of pediatric patients with functional AVB or SP. METHODS: This is a single-center, case series study. Patients with SP or AVB, 21 years of age or younger, who underwent CNA between 2015 and 2021 were included. CNA was performed via anatomically guided and high-frequency stimulation methods. RESULTS: Six patients were included. The median age was 18.9 years (range 12.3-20.9 years), 33% female. Two patients had prolonged SP, two had paroxysmal AVB, and two had both SP and AVB. Four patients had prior syncope. The median longest pause was 8.9 s (range 3.9-16.8) with 11 total documented pauses (range 2-231) during the 6 months pre-CNA. Post-CNA, the median longest pause was 1.3 s (range 0.8-2.2) with one documented SP after termination of atrial tachycardia at the 3-month follow-up. At 6 months, the median longest pause was 1.1 s (0.8-1.3) with 0 documented pauses. No patients had syncope post-CNA. CONCLUSION: CNA may be an effective alternative to pacemaker implantation in pediatric patients with syncope or significant symptoms secondary to functional SP or AVB.

Prominent crista terminalis mimicking a right atrial mass: a systematic literature review and meta-analysis.

The crista terminalis is an anatomical structure localized on the posterolateral wall of the right atrium (RA). We performed a systematic review of the literature and meta-analysis concerning cases of unusual prominent crista terminalis mimicking RA mass. Moreover, we described the differential diagnosis of cardiac masses with the use of echocardiography, computed tomography, and cardiac magnetic resonance (CMR). We also emphasize the potential importance of this structure in electrophysiological procedures, including its role in exaggerated arrhythmias. Prominent crista terminalis may be a potential obstacle during invasive cardiac procedures or catheter ablation target. In analyzed cases, the crista terminalis was often erroneously interpreted as pathologic and at first confused with a thrombus or tumor during transthoracic echocardiography examination. The correct final diagnoses were mostly made with used transesophageal echocardiography or CMR. The most important imaging findings suggestive of prominent crista terminalis rather than tumor were a similar echogenicity/intensity with adjacent myocardium, the location on posterolateral wall of the RA, the phasic change in size, and no enhancement after contrast injection. We describe up to date and detailed imaging features for the differential diagnostics of selected intracardiac masses using various imaging techniques, including multimodality cardiac imaging. Familiarity with the anatomy and the imaging findings of the prominent crista terminalis will reduce misdiagnosis and avoid additional tests and unwarranted clinical interventions, while in patients considered for invasive cardiac procedures it might increase their efficacy and safety.

Clinical Presentation and Therapy of Total Anomalous Pulmonary Venous Return.

Total anomalous pulmonary venous return (TAPVR) is rare (accounting for about 1% of all CHD) and can occur as a single lesion or in combination with other types of CHD (such as heterotaxy or HLHS). TAPVR is defined as an abnormal connection where all pulmonary veins do not drain into the left atrium but into the right atrium either directly or through a vein that is connected to the right atrium. TAPVR can be divided into four anatomic groups (Fig. 32.1): (1) supracardiac (about 55%), (2) cardiac (about 30%), (3) infracardiac (about 13%), and (4) mixed (very rare). In addition, it can be divided into two physiological types: nonobstructed and obstructed. Embryologically, all pulmonary veins usually connect to a pulmonary venous confluence that connects to the left atrium. If this connection does not occur, the pulmonary venous confluence connects to a systemic vein instead.

Rare case of total anomalous pulmonary venous return into the right atrium in situs solitus.

We report an uncommon case report of total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein's ostium without a sinus venosus defect, in situs solitus, without vertical vein or a posterior pulmonary venous confluence.

Vein of Galen Aneurysmal Malformation with Anomalous Right Superior Vena Cava to the Left Atrium Leading to Atypical Clinical and Echocardiographic Findings.

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1 week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4 months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM.

Midline crossing right pulmonary veins with dual drainage to the left atrium.

We report the exceptional case of transcatheter treatment of a partial anomalous pulmonary venous drainage of the right lung to the innominate vein and dual drainage to the left atrium.

Idiopathic enlargement of the right atrium masking left atrial aneurysm in a neonate.

An idiopathic enlargement of the right atrium is an extremely rare cardiac malformation. There are no established guidelines for the management of this disease, especially concerning medical versus surgical therapeutic approach and the timing for an operation. We report in this case about a neonate that first was treated conservatively until the age of 5 month and finally got an operative resection of the aneurysm. After surgery, unexpected complications occurred. A second aneurysm in the left atrium was demasked. Furthermore, a progressive dilatation of both atrial chambers after resection required regular follow-up and ongoing evaluation of treatment.

Anomalous systemic venous connection to left atrium in a heart with usual atrial arrangement.

Anomalous systemic venous connection to left atrium is rare anomaly. Previously published cases described this anatomy in patients with left isomerism. Depending on the size of the atrial septal defect, patients usually present with varying degrees of cyanosis and right heart hypoplasia. Here, we report a case of anomalous systemic venous connection to left atrium in a newborn with the usual atrial arrangement.

A black box congenital heart defect: Anomalous superior vena cava drainage into the left atrium.

A 25-year-old pregnant woman with low oxygen saturation due to unclear congenital heart disease was admitted. During work-up the rare anomaly of superior vena cava drainage to the left atrium was diagnosed by transthoracic bubble test with agitated saline. The pathology was later confirmed by contrast multislice computed tomography angiography after delivery.

A rare congenital anomaly: Anomalous fibromuscular cord of the left atrium.

BACKGROUND: Left atrial anomalous fibromuscular cord is a rare congenital anomaly, which exists in a small proportion of the general population. Although its clinical significance remains largely unknown, it is generally considered a benign entity. We report a case of incidental finding of left atrial fibromuscular cord without structural cardiac abnormalities or hemodynamic obstruction. METHODS AND RESULTS: A-39-year-old female presented with palpitations for more than 10 years. Electrocardiogram and laboratory tests showed no unremarkable. Transthoracic echocardiography revealed an abnormal linear structure connecting the interatrial septum and the left atrial free wall, color Doppler flow imaging did not show hemodynamic obstruction. Cardiac contrast-enhanced computed tomography images showed the string-like structure associated with calcification, connecting the interatrial septum and the ridge around the orifice of the left inferior pulmonary vein. Sagittal multiplanar reconstructed image showed a dot-like structure located in the left atrial cavity. DISCUSSION: Left atrial anomalous fibromuscular cord is a rare congenital anomaly, which is also known as left atrial anomalous fibromuscular cord, left atrial false tendon, accessory chordae tendineae, or left atrial aberrant band. The clinical significance is unclear. Some cases have been reported that the fibromuscular cord, which do not have pathological significance. It has also been reported that it may be associated with supraventricular arrhythmias, patent foramen ovale, and Chiai's network. In some patients, attachment to the mitral chord can lead to mitral valve insufficiency and murmur. Nevertheless, a detailed understanding the anomalous anatomical characteristics of the anomalous cord may help us to better predict an unexpected difficulty in catheter manipulation, and potential arrhythmogenicity. CONCLUSION: Transthoracic echocardiography and cardiac computed tomography angiography have an important imaging value for the diagnosis of the left atrial anomalous fibromuscular cord, including its origin, course, or whether associated with other cardiovascular malformations.

An aberrant draining of the anterior interventricular part of the great cardiac vein into the superior vena cava.

BACKGROUND: Abnormalities in drainage of the great cardiac vein (GCV) are interesting due to its rarity and likely to be underreported, with most cases found incidentally in cardiac imaging and autopsy studies. We report a case with anomalous drainage of the GCV into the LA, and the rest of the cardiac veins are draining normally. METHODS AND RESULTS: A 60-year-old male presented with heart palpitations for half a month. Electrocardiogram and laboratory tests showed no abnormalities. He was recommended for coronary computed tomography angiography (CCTA). The maximum intensity projection image of CCTA showed that the GCV draining into the left atrium, the rest of the cardiac veins, and coronary vein sinus were draining into the right atrium normally. Volume-rendered image of coronary CT angiography showed that the GCV originated in the upper third of the anterior interventricular sulcus and drained directly into the left atrium. DISCUSSION: Abnormalities in drainage of the GCV are interesting due to its rarity and likely to be underreported. Only a few cases have been reported that the aberrant drainage of the GCV, with draining into the anterior cardiac veins, the left internal thoracic vein, the superior vena cava, the right atrium, and the LA. The abnormality of GCV is an often neglected aspect of CCTA imaging, it can be better displayed in the venous phase of coronary catheter angiography. The awareness of which may be critically important for procedures that require venous access such as coronary surgery requiring retrograde cardioplegia, surgical ablation of aberrant conducting pathways, pacemaker insertion, and valves surgery. CONCLUSION: This variant of the GCV is interesting due to its rarity. CCTA has important diagnostic imaging value in abnormalities of the origin, course, and termination of the GCVs, the variant can be better displayed in the venous phase of coronary catheter angiography.

Atresia of the coronary sinus ostium with persistent left superior caval vein.

We describe a case of a 33-year-old woman with congenitally corrected transposition where computed tomography angiography incidentally detected ostial atresia of the coronary sinus with dilatation of the terminal parts of the middle cardiac vein and great cardiac vein and retrograde drainage of the coronary sinus into the persistent left superior caval vein, the intercommunicating vein, then to the right superior caval vein, and ultimately into the right atrium.

A hitherto unreported combination of pulmonary stenosis, single coronary artery anomaly, and coronary sinus to left atrial communication.

BACKGROUND: We report a hitherto unreported combination of pulmonary stenosis, single coronary artery anomaly and coronary sinus to left atrial communication. Our case highlights the important value of coronary computed tomographic angiography and transthoracic echocardiography for the diagnosis of such anomalies and guidance for proper management. METHODS AND RESULTS: A 64-year-old male presented chest tightness and shortness of breath for 2 days. Transthoracic echocardiography revealed a thickened pulmonary valve leaflet and subvalvular outflow tract stenosis, colour flow Doppler showed a significant accelerated blood flow in the pulmonary artery cavity originating from the subvalvular outflow tract, continuous wave Doppler revealed the transpulmonary valvular pressure gradient of 63mmHg. Computed tomographic angiography image reveals thickened pulmonary valve leaflets and subvalvular outflow tract stenosis, single coronary artery anomaly and levoatriocardinal vein. The patient underwent percutaneous pulmonary valve balloon dilatation, the post-procedural course was uneventful. DISCUSSION: Pulmonary stenosis can occur as part of more congenital cardiac malformations or as rare primary isolated pulmonary stenosis, which includes the valvular, sub-valvular, or supra-valvular pulmonary stenosis. Single coronary artery anomalies are very rare, anomalous right coronary artery originates from proximal to mid-left anterior descending coronary artery is one such single coronary artery anomaly, in most cases, it is asymptomatic, diagnosed incidentally, and a benign entity has a better prognosis except if the right coronary artery is passing between the aorta and pulmonary artery. This course of the right coronary artery anomaly is malignant. Coronary sinus to left atrial communication includes a direct or indirect communication. The direct communication is described as a partial or complete absence of the roof between the coronary sinus and left atrium, as it is well known as the unroofed coronary sinus syndrome. The indirect communication is an anomalous bridging vein communicating the coronary sinus to the left atrium, which can be distinguished from classical unroofed coronary sinus syndrome. The venous collateral channel communication between the coronary sinus to the left atrium by a bridging vein is also categorized as a variant type of unroofed coronary sinus syndrome. Understanding coronary venous variations has significant clinical implications particularly in the realm of electrophysiology. The anatomical variations can have important consequences for procedures such as biventricular pacing and trans-coronary vein ablations. CONCLUSION: Pulmonary stenosis combined with single coronary artery anomaly and bridging vein communication between the coronary sinus and the left atrium is an extremely rare. Coronary computed tomographic angiography and transthoracic echocardiographyplay an important role the diagnosis of such anomalies and guidance for clinical Treatment.

Giant right atrium, what does it hide? Case presentation.

BACKGROUND: Malformation of the right atrium is a rare cardiac abnormality and is usually reported as isolated malformation in the literature. CASE PRESENTATION: Prenatal giant atrial dilatation in an asymptomatic infant was treated surgically at 18 months of age, due to potential risk of thrombosis and arrhythmias. Post-surgical echocardiographic images illustrate residual atrial elevated pressure. CONCLUSIONS: Sometimes, as seems in our case, right atrial dilatation hides an associated restrictive right ventricle.

Electrocardiogram and left atrial abnormality: Design of an observational study to clarify diagnostic criteria.

BACKGROUND: The criteria applied for diagnosis of left atrial (LA) abnormality using electrocardiogram (ECG) have high specificity but low sensitivity. In fact, some authors have suggested classifying P-wave anomalies associated with LA abnormality and interatrial block as "atrial abnormalities." The most widely known ECG criteria for LA abnormality include P-wave duration, morphology and voltage of P wave in inferior leads, presence of P-wave terminal force in V1 (PtfV1 ), and P-wave axis and area. PtfV1 has also been reported to vary according to misplacement of the V1 and V2 electrodes. METHODS: The objective of this observational cohort study is to determine the degree of correlation between ECG criteria for LA abnormality and left atrium volume and functionality, as determined by speckle tracking echocardiography. The study also aims to investigate the correlation between these echocardiographic parameters and PtfV1 value by placing the V1 and V2 electrodes in the second, third, and fourth intercostal spaces. RESULTS AND CONCLUSIONS: Our results could help to clarify whether the decrease in left atrial deformity, which is currently considered a surrogate target of fibrosis, correlates better with ECG criteria for LA abnormality than atrial volumes.

Gerbode defect and left ventricular-aortic discontinuity in prosthetic valve endocarditis.

Communication between the left ventricle and right atrium is known as the Gerbode defect. The defect is usually congenital but can be acquired secondary to infective endocarditis. Left ventricular-aortic discontinuity is another serious complication of extensive infective endocarditis. Here, we report a rare case of prosthetic valve endocarditis complicated with both acquired Gerbode defect and left ventricular-aortic discontinuity. We successfully performed reconstructive surgery involving patch closure of the Gerbode defect and reconstruction of the circumferential left ventricular outflow tract with a xenopericardial patch, followed by supra-annular aortic valve replacement with the Solo Smart bovine pericardial stentless valve.

Right superior vena cava draining into the left atrium in a patient with sinus venosus defect.

Right superior vena cava draining into the left atrium is an extremely rare anomaly of systemic venous return. It can be isolated or it can be associated with other congenital heart defects, thus clinical presentation may vary. A case of a 9-year-old asymptomatic patient with sinus venous defect and bilateral superior vena cava with the right-sided draining into the left atrium is described.

P-Wave Changes Associated with Chiari Network in the Right Atrium.

The Chiari network (CN) is a mobile, net-like structure occasionally present in the right atrium, near the opening of the inferior vena cava and coronary sinus. While typically asymptomatic, it may contribute to thromboembolism or right atrial pathologies. Here, we hypothesized that existing differences in P-wave morphology on electrocardiograms (ECG) may be associated with atrial conduction changes. Seventy-one children with a CN were recruited and matched to 60 healthy controls. P-wave duration, P-wave amplitude, P dispersion (Pd), QRS, PR, QT, and QTc (calculated with Bazett formula) intervals were measured and compared. Between the control and the patient groups, the mean P-wave duration was 78.1 ms and 88.7 ms, P amplitude was 1.3 mm and 1.1 mm, and Pd was 18.9 and 35.5 ms, respectively. These differences were statistically significant across all measurements (p < 0.05). Atrial conduction may be affected in patients with CN, and these patients may then develop atrial arrhythmia.

The coronary sinus interatrial connection with total unroofing coronary sinus discovered late after correction of secundum atrial septal defect.

A coronary sinus (CS) interatrial connection is a rare congenital anomaly which can be in various types from atrial septal defect type unroofing CS to the total absence of the CS. The pathology usually accompanies the left superior caval vein (LSCV) draining to CS and in case of its absence directly to the left atrium (LA). We present a 53-year-old woman after surgical correction of a secundum atrial septal defect in 1974, with a CS interatrial connection and paroxysmal atrial fibrillation (PAF). She gave a history of PAF and was admitted to our clinic due to progressive exertional intolerance, peripheral edema, and mild hypoxia (SatO2 92%) with subsequent cyanosis. Transthoracic echocardiography showed a left-to-right shunt in the posteroinferior part of the atrial septum. Computed tomography revealed a persistent LSCV draining directly into the LA, the absence of the CS, and cardiac veins draining into the LA. The right atrium (RA) and the LA were connected via a tunnel with a visible contrast passage from the left to the right side-the persisting mouth of the coronary sinus. The patient was qualified for surgical correction. A glutaraldehyde-treated autologous pericardial patch was used to construct the tunnel connecting the LSVC and the RA. The second part of the patch was used to close the atrial communication at the inferior vena cava level. The patient had an uncomplicated postoperative course and is now classified in New York Heart Association Class II.

Thoracoatrial discordance in a case of twisted atrioventricular connections: A unique configuration of visceroatrial arrangement.

We present a case of a 12-year-old boy with discordance between the atrial arrangement and the thoracoabdominal arrangement in the setting of twisted atrioventricular connections. This case highlights the importance of a separate description of all visceral organs and venous drainage for an accurate description of visceroatrial arrangement in cases of congenital heart defects.