Giant coronary artery aneurysm in the atrial septum.

A man in his 60s with paroxysmal atrial fibrillation was scheduled for a catheter ablation but was admitted to our department after contrast-enhanced CT showed a large homogeneous right atrial mass (52×52 mm) as well as a dilated right coronary artery (RCA). Coronary artery angiography showed a large fistula from the RCA to the mass in the right atrium. A giant coronary artery aneurysm was suspected and a surgical resection was performed. The mass was attached to the atrial septal wall and was palpated in the right atrium with a feeding artery from the RCA. The final diagnosis was an extremely rare case of giant coronary artery aneurysm originating from the RCA. The surgery was successful, and the patient was discharged 30 days later.

Unexpected diagnosis: large hemangioma in the interatrial septum.

BACKGROUND: Cardiac hemangiomas are very uncommon benign primary tumors. They are usually located preferentially in the right atrium and their location in the interatrial septum is extremely rare. CASE PRESENTATION: We report the case of a 41-year-old patient who was admitted due to a stroke. The transthoracic echocardiogram revealed a large mass in the right atrium adhered to the interatrial septum. Suspecting an atrial myxoma, surgical intervention was performed confirming that the mass extended within the thickness of the interatrial septum, protruding into the right atrial cavity. The histologic report confirmed a hemangioma. CONCLUSIONS: Cardiac hemangiomas are rare primary tumors and are usually misdiagnosed as other cardiac tumors. Histopathological examination is essential for a definitive diagnosis.

Papillary fibroelastoma originating from the atrial septum touching the mitral valve leading to infective endocarditis: a case report.

BACKGROUND: Cardiac papillary fibroelastoma is a rare benign tumor, which is often mistaken for a vegetation. Predominantly asymptomatic, it can cause life-threatening complications. Although rare, mobile papillary fibroelastoma movement between affected valves may hamper valve closure and damage the valve, leading to valvular regurgitation. Endothelial damage increases the risk of developing infective endocarditis. We report a rare case of a highly mobile papillary fibroelastoma originating from the atrial septum touching the mitral valve, leading to mitral regurgitation and, eventually, infective endocarditis. CASE PRESENTATION: A 26-year-old woman with suspected infective endocarditis was referred to us from a previous hospital after having experienced intermittent fever for a month. Before the fever, she had been experiencing exertional dyspnea. In addition, she had undergone a cesarean section two weeks before this admission. A transthoracic echocardiogram showed a mobile mass originating from the atrial septum touching the mitral valve with severe mitral regurgitation. Computed tomography revealed an occluded right profunda femoris artery with an embolus. Infective endocarditis associated with a mobile vegetation with high embolic risk was diagnosed, and urgent surgery was performed. Following the surgery, examinations revealed papillary fibroelastoma originating from the atrial septum and infective endocarditis of the mitral valve. The histopathological examination confirmed that a mass initially thought to be a mobile vegetation was a papillary fibroelastoma. The postoperative course was uneventful except for pericarditis. There has been no recurrence of infective endocarditis or papillary fibroelastoma. CONCLUSIONS: The highly mobile papillary fibroelastoma was thought to have caused both chronic mitral regurgitation and infective endocarditis. Mobile papillary fibroelastomas can cause endothelial damage to nearby valves and predispose patients to infective endocarditis.

[Lipomatous Hypertrophy of Interatrial Septum in Patient with Venous Thrombus:Report of a Case].

A 64-year-old female underwent a computed tomography (CT) examination of the chest because of pneumonia, which revealed lipomatous hypertrophy of the atrial septum. During medical treatment for pneumonia, venous thrombus of the superior vena cava occurred and then disappeared following apixaban administration. The patient refused surgery at that time, thus follow-up examinations include CT scans were conducted at the outpatient clinic. Two years later, the tumor became larger, and this time she accepted surgery. Under total cardiopulmonary bypass with beating heart a 5.0×3.5 cm tumor was resected. Because of its location very close to superior vena cava, upper area of the right atrium was augmented using a bovine pericardium. Following surgery, sick sinus syndrome developed and pacemaker implantation was performed. Thereafter, the patient had a smooth recovery and no recurrence is noted one year since.

Leftward and Posterior Deviation of the Septum Primum Predicts Morbidity in Patients with Hypoplastic Left Heart Syndrome.

Leftward posterior deviation of the atrial septum primum (LDSP) has been reported in up to 64% of patients with hypoplastic left heart syndrome (HLHS) but there are no published data on its impact on neonatal outcomes. We reviewed the prevalence of LDSP and its correlation with neonatal outcomes in our institution. This was a single-center retrospective study of neonates with HLHS from 2001 to 2019. Echocardiograms were reviewed and the presence or absence of LDSP was noted. To quantify the degree of deviation in patients with LDSP, a new measurement, the deviation index (DI) was calculated using both the subcostal long and short-axis views. Of ninety-four patients with HLHS, fifty-seven (61%) patients were noted to have LDSP. There was no statistically significant difference in gestational age (GA), birth weight (BW), or mortality between patients with and without LDSP. Patients with LDSP had an increased incidence of unplanned reoperation (p < 0.01), post-operative cardiac catheterization (p < 0.05), and post-operative infection (p < 0.05). After correction for GA, BW, HLHS subtype, and type of surgery, LDSP predicted reoperation (OR = 3.6, p < 0.01), catheterization (OR = 2.7, p = 0.05), and infection (OR = 3.4, p < 0.05). Higher degree of deviation predicted reoperation (DI > 0.17), catheterization (DI > 0.07), and infection (DI > 0.12). There was excellent inter-observer reproducibility of the DI (ICCabsolute-agreement = 0.82, ICCconsistency = 0.90). Patients with LDSP have a higher prevalence of post-operative morbidity. The degree of deviation was found to be predictive of post-operative complications. Pre-operative echocardiographic evaluation of LDSP in patients with HLHS may be helpful in risk stratification and counseling.

Lipomatous Hypertrophy of the Interatrial Septum in a Patient with Chronic Dyspnea and Episodes of Paroxysmal Atrial Fibrillation: A Case Report.

Lipomatous Hypertrophy of the Interatrial Septum (LHIS) is an unusual condition usually found as an incidental finding on echocardiography. It can create a mass-like bulge typically sparing the fossa ovalis. We describe a 73-year-old overweight Cypriot woman, who visited our clinic in November 2018 and was complaining of dyspnea on exertion, chest tightness, and two episodes of paroxysmal atrial fibrillation for the last eight months. A big sessile mass was detected in the interatrial septum measuring 3.7×4.7 cm during transoesophageal echocardiography. The patient underwent resection of the mass and pericardial patch reconstruction of the defect with significant improvement of her clinical status during the follow-up period. The histopathological analysis suggested LHIS. In patients with persistent cardiac symptoms, a prompt resection is a useful option, although surgery is generally considered unnecessary in this clinical setting. Preprint of this article is available on: https://www.authorea.com/users/380290/articles/496351-a-patient-with-chronic-dyspnea-and-episodes-of-paroxysmal-atrial-fibrillation-in-the-presence-of-a-right-atrial-mass.

[Efficacy of fenestrated atrial septal defect occulders on pulmonary hypertension dogs].

Objective: To explore the short-term efficacy of fenestrated atrial septal defect (ASD) occulders in the treatment of pulmonary arterial hypertension (PAH). Methods: Thirty-six healthy dogs were divided into the balloon atrial septostomy (BAS)+fenestrated ASD occulders group (n=12), BAS group (n=12) and non-septostomy group (n=12). PAH was induced by intra-atrial injection of dehydrogenized monocrotaline (1.5 mg/kg) in all dogs. Animals in the BAS+fenestrated ASD occulders group underwent atrial septal puncture and fenestrated ASD occulders implantation. Animals in the BAS group underwent balloon atrial septostomy. The non-septostomy group received no surgical intervention. The hemodynamic indexes and blood N-terminal pro-B-type natriuretic peptide (NT-proBNP) of dogs were measured before modeling, 2 months after modeling, 1, 3, and 6 months after surgery, respectively. Echocardiography was performed to observe the patency of the shunt and atrial septostomy of the dogs in the BAS+fenestrated ASD occulders group and BAS group at 1, 3, and 6 months after surgery. Three dogs were sacrificed in each group at 1, 3, and 6 months after surgery, respectively. Atrial septal tissue and fenestrated ASD occulders were removed to observe the patency and endothelialization of the device. Lung tissues were obtained for hematoxylin-eosin (HE) staining to observe the inflammatory cells infiltration and the thickening and narrowing of the pulmonary arterials. Results: Among 36 dogs, 2 dogs died within 24 hours after modeling, and 34 dogs were assigned to BAS+fenestrated ASD occulders group (n=12), BAS group (n=11), and non-septostomy group (n=11). Compared with BAS group, the average right atrial pressure (mRAP) and NT-proBNP of dogs in the BAS+fenestrated ASD occulders group were significantly reduced at 3 months after surgery (P<0.05), and the cardiac output (CO) was significantly increased at 6 months after surgery, arterial oxygen saturation (SaO2) was also significantly reduced (P<0.05). Compared with non-septostomy group, dogs in the BAS+fenestrated ASD occulders group had significantly lower mRAP and NT-proBNP at 1, 3, and 6 months after surgery (P<0.05), and higher CO and lower SaO2 at 6 months after surgery (P<0.05). Compared with the non-septostomy group, the dogs in the BAS group had significantly lower mRAP and NT-proBNP at 1 month after surgery (P<0.05), and there was no significant difference on mRAP and NT-proBNP at 3 and 6 months after surgery (P>0.05). Echocardiography showed that there was a minimal right-to-left shunt in the atrial septum in the BAS group at 1 month after the surgery, and the ostomy was closed in all the dogs in the BAS group at 3 months after the surgery. There was still a clear right-to-left shunt in the dogs of BAS+fenestrated ASD occulders group. The shunt was well formed and satisfactory endothelialization was observed at 1, 3 and 6 months after surgery. The results of HE staining showed that the pulmonary arterials were significantly thickened, stenosis and collapse occurred in the non-septostomy group. Pulmonary microvascular stenosis and inflammatory cell infiltration in the pulmonary arterials were observed in the non-septostomy group. Pulmonary arterial histological results were comparable between BAS+fenestrated ASD occulders group and non-septostomy group at 6 months after surgery. Conclusions: The fenestrated ASD occulder has the advantage of maintaining the open fistula hole for a longer time compared with simple balloon dilation. The fenestrated ASD occulder can improve cardiac function, and it is safe and feasible to treat PAH in this animal model.

Association of atrial septal fenestration with outcomes after atrioventricular septal defect repair.

OBJECTIVE: During repair of atrioventricular septal defect (AVSD), surgeons might leave an atrial level shunt when concerned about postoperative physiology, or as part of routine practice. However, the association of fenestration with outcomes is unclear. We sought to determine factors associated with mortality after biventricular repair of AVSD. METHODS: We included 581 patients enrolled from 32 Congenital Heart Surgeons' Society institutions from January 1, 2012, to June 1, 2020 in the Congenital Heart Surgeons' Society AVSD cohort. Parametric multiphase hazard analysis was used to identify factors associated with mortality. A random effect model was used to account for possible intersite variability in mortality. RESULTS: An atrial fenestration was placed during repair in 133/581 (23%) patients. Overall 5-year survival after repair was 91%. Patients who had fenestration had an 83% 5-year survival versus 93% for those not fenestrated (P < .001). Variables associated with mortality in multivariable hazard analysis included institutional diagnosis of ventricular unbalance (hazard ratio [HR], 2.7 [95% confidence interval (CI): 1.5-4.9]; P = .003), preoperative mechanical ventilation (HR, 4.1 [95% CI, 1.3-13.1]; P = .02), atrial fenestration (HR, 2.8 [95% CI, 1.5-4.9]; P < .001), and reoperation for ventricular septal defect (HR, 4.0 [95% CI, 1.3-13.1]; P = .002). There was no difference in measures of ventricular unbalance for comparisons of fenestrated with nonfenestrated patients. No significant interinstitution variability in mortality was observed on the basis of the random effect model (P = .7). CONCLUSIONS: An atrial communication at biventricular repair of AVSD is associated with significantly reduced long-term survival after adjusting for other known associated factors, including unbalance. These findings might challenge the routine practice of fenestration.

18F-FDG PET/CT helps rule out malignancy in lipomatous hypertrophy of the interatrial septum with atypical MRI manifestations, to avoid unnecessary surgical treatment.

Lipomatous hypertrophy of the interatrial septum (LHIS) is a rare benign heart disease characterized by excessive deposition of adipose tissue in the atrial septum with sparing fossa ovalis, which demonstrates a characteristic hourglass/dumbbell configuration. We reported a case of LHIS with inhomogenous characteristic on CMR and persistently no FDG uptake on 18F-FDG PET-CT, which is contrary to a few previous cases. This single case report suggests that in case LHIS present in-homogenous characteristic on CMR, metabolic PET imaging could be used as a complementary imaging model to decrease the concern of lipomatous neoplasms, thereby avoid unnecessary surgical therapy.

[Lipomatous Hypertrophy of the Interatrial Septum Preoperatively Misdiagnosed as Right Atrial Lipoma].

A 73-year-old man with a right atrial tumor and a distal aortic arch aneurysm was scheduled for tumor resection and total arch replacement (TAR). Although the tumor was diagnosed as an intraatrial lipoma preoperatively, it could not be differentiated from an infiltrating lipoma on the basis of intraoperative pathological examination. We decided to completely excise the tumor. However, we found intraoperatively that the tumor was contiguous with the epicardial fat tissue of the heart, strongly suggesting a lipomatous hypertrophy of the interatrial septum (LHIS). Thus, we discontinued the resection, reconstructed the atrial septum and right atrial wall, and performed TAR with a frozen elephant trunk technique. Although the patient required temporary continuous hemodiafiltration because of his preoperative renal dysfunction, he was discharged 30 days postoperatively. If LHIS was the preoperative diagnosis, only an incisional biopsy was needed for diagnosing this case. However, LHIS is quite rare in Japan. Therefore, awareness of the disease and meticulous analysis of preoperative heart imaging are important to avoid an unnecessary surgery.

LIPOMATOUS HYPERTROPHY OF THE INTERATRIAL SEPTUM - A BENIGN HEART ANOMALY CAUSING UNEXPECTED PROBLEM IN ELECTROPHYSIOLOGY (CASE REPORT).

Lipomatous Hypertrophy of the Interatrial Septum (LHIS) is an unusual and benign condition characterized by the excessive deposition of adipose tissue in the interatrial septum, which is most often detected as an incidental finding on echocardiography. The classic finding is a homogenous, bi-lobed configuration of the interatrial septum with sparing of the fossa ovalis. LHIS has been associated with various atrial arrhythmias, including multifocal atrial tachycardia, multiple premature atrial contractions, atrial fibrillation and rarely sudden death. The prevalence of LHIS is estimated to be between 1-8%. The incidence increases with age, body mass and chronic corticosteroid therapy. There may be a higher incidence in women. Here the authors describe a case report of a 73 year-old obese female who visited the cardiology department for planned cryoablation of paroxysmal atrial fibrillation. Difficulties raised during transseptal punction, a bidimensional tranthoracic echocardiography (TTE) showed the typical findings of LHIS. A 73 year-old asymptomatic woman was found to have an incidental cardiac mass, TTE findings were consistent with lipomatous hypertrophy of the interatrial septum. Given the characteristic appearance on TTE, biopsy or surgery was not indicated, the procedure of cryoablation has been stopped and the patient was managed conservatively.

Use of a cerebral protection device to facilitate pulmonary vein isolation in a patient with left atrial mass arising from the interatrial septum.

Sentinel cerebral embolic protection devices (CPDs) may limit periprocedural cerebrovascular events by preventing micro and macro-embolization to the brain, and has been used in many cardiology and radiology procedures. We hereby report the use of a Sentinel CPD to facilitate safe and effective atrial fibrillation ablation in a patient with a left atrial mass arising from the interatrial septum.

Totally anomalous pulmonary venous connection with intact atrial septum.

BACKGROUND: Total anomalous pulmonary venous connection with intact interatrial septum is extremely rare. AIMS: In these patients, a right to left shunt through a ventricular septal defect or a patent ductus arteriosus is mandatory to maintain the systemic circulation. Mechanical or physiological restriction of shunting through these pathways results in rapid clinical deterioration. DISCUSSION: We describe the anatomical findings with surgical repair in one such 10-day-old baby.

Interatrial septal tachycardias following atrial fibrillation ablation or cardiac surgery: Electrophysiological features and ablation outcomes.

BACKGROUND: Interatrial septal tachycardias (IAS-ATs) following atrial fibrillation (AF) ablation or cardiac surgery are rare, and their management is challenging. OBJECTIVE: The purpose of this study was to investigate the electrophysiological features and outcomes associated with catheter ablation of IAS-AT. METHODS: We screened 338 patients undergoing catheter ablation of ATs following AF ablation or cardiac surgery. Diagnosis of IAS-AT was based on activation mapping and analysis of response to atrial overdrive pacing. RESULTS: Twenty-nine patients (9%) had IAS-AT (cycle length [CL] 311 ± 104 ms); 16 (55%) had prior AF ablation procedures (median 3; range 1-5), 3 (10%) had prior surgical maze, and 12 (41%) had prior cardiac surgery (including atrial septal defect surgical repair in 5 and left atrial myxoma resection in 1). IAS substrate abnormalities were documented in all patients. Activation mapping always demonstrated a diffuse early IAS breakout with centrifugal biatrial activation, and atrial overdrive pacing showed a good postpacing interval (equal or within 25 ms of the AT CL) only at 1 or 2 anatomically opposite IAS sites in all cases. Ablation was acutely successful in 27 patients (93%) (from only the right IAS in 2, only the left IAS in 9, both IAS sides with sequential ablation in 13, and both IAS sides with bipolar ablation in 3). After median follow-up of 15 (6-52) months, 17 patients (59%) remained free from recurrent arrhythmias. CONCLUSION: IAS-ATs are rare and typically occur in patients with evidence of IAS substrate abnormalities and prior cardiac surgery. Catheter ablation can be challenging and may require sequential unipolar ablation or bipolar ablation.

Hypoplastic Left Heart: Stage-I Will be Performed Interventionally, Soon.

The hypoplasia of left-sided heart structures shows great variability and complexity. What the many variants have in common is that their heart structures are neither fully developed before nor after birth. Fetuses and newborns require an individual therapy depending on anatomy and function of the heart. Fetal interventions focus on improving left heart structures by catheter-based interventions and maternal hyperoxygenation which promotes growth as the left ventricular preload and blood flow within the cavity increase. Stage-I management of newborns with single ventricle physiology is usually based on the Norwood/Sano surgery or the Hybrid approach. Two more steps are required to ultimately achieve a Fontan circulation. Some centers also use the Hybrid approach for subsequent Norwood operation beyond the neonatal period. After the Hybrid approach, a comprehensive stage-II or corrective surgery is performed, the latter if a bi-ventricular circulation is possible. With progressively improved catheter-based interventions, particularly ductal stenting and manipulations of the atrial septum, the next advance is to develop a bespoke flow restrictor that can be easily inserted into the branches of the pulmonary artery. The main goal is to avoid complex heart operations under general anesthesia, followed by substantial intensive care in the neonatal period, especially for patients with complex heart defects. Based on the current state of the art of surgical treatment of hypoplastic left heart syndrome and variants with the Norwood surgery or the Hybrid approach, our main focus is on an alternative percutaneous transcatheter technique in the sense of a completely non-surgical stage-I approach.

Safety of Transseptal Puncture for Access to the Left Atrium in Infants and Children.

Transseptal puncture (TSP) is a standard procedure to obtain access to the left heart. However, data on TSP in infants and children particularly with congenital heart defects (CHD) is sparse. Safety and efficacy of TSP in infants and children < 18 years with normal cardiac anatomy and with CHD were assessed. 327 TSP were performed in a total of 300 individuals < 18 years from 10/2002 to 09/2018 in our tertiary pediatric referral center. Median age at TSP was 11.9 years (IQR 7.8-15; range: first day of life to 17.9 years). 13 subjects were < 1 year. Median body weight was 43.8 kg (IQR 26.9-60; range: 1.8-121 kg). CHD was present in 28/327 (8.6%) procedures. TSP could be successfully performed in 323/327 (98.8%) procedures and was abandoned in 4 procedures due to imminent or incurred complications. Major complications occurred in 4 patients. 3 of these 4 subjects were ≤ 1 year of age and required TSP for enlargement of a restrictive atrial septal defect in complex CHD. Two of these babies deceased within 48 h after TSP attempt. The third baby needed urgent surgery in the cath lab. Pericardial effusion requiring drainage was noted in the forth patient (> 1 year) who was discharged well later. Minor complications emerged in 5 patients. The youngest of these individuals (0.3 years, 5.8 kg) developed small pericardial effusion after anterograde ballon valvuloplasty for critical aortic stenosis. The remaining 4/5 patients developed small pericardial effusion after ablation of a left-sided accessory atrioventricular pathway (6.1-12.2 years, 15.6-34.0 kg). TSP for access to the left heart was safe and effective in children and adolescents > 1 year of age. However, TSP was a high-risk procedure in small infants with a restrictive interatrial septum with need for enlargement of interatrial communication.

Elective Non-Urgent Balloon-Atrial Septostomy in Infants with d-Transposition of the Great Arteries Does Not Eliminate the Need for PGE1 Therapy at the Time of Arterial Switch Operation.

Once a mainstay in the treatment of neonates with d-transposition of the great arteries (d-TGA), the application of balloon atrial septostomy (BAS) in the d-TGA population has become more selective. Currently, there is no clear evidence for or against a selective BAS strategy. The aims of this single-center retrospective study were to determine the incidence of BAS in the neonatal d-TGA population in the current era, to measure the rate of procedural success, and to compare the outcomes and complication rates of patients who underwent BAS to those who underwent neonatal ASO alone. Between 2012 and 2018, 147 patients with d-TGA underwent initial medical management and ASO, 73 of which underwent BAS. The percentage of patients that underwent BAS decreased from 73 to 33% over the study time period. In patients with d-TGA with intact ventricular septum, 33% of patients remained off of PGE1 at the time of surgery regardless of BAS. In d-TGA with ventricular septal defect, 85.7% of those that underwent BAS and 54.1% of those who did not remained off of PGE1 at the time of surgery, however, this difference did not reach statistical significance. In this single institution retrospective cohort of patients with d-TGA, the performance of a technically successful balloon atrial septostomy did not eliminate the need for PGE1 therapy at the time of definitive ASO. This was true regardless of the presence or absence of a ventricular septal defect.