Atrial Defect Research Articles

Total resection via right mini-thoracotomy for left atrial myxoma in juvenile Carney complex: a case report

Abstract Background Carney complex is a rare syndrome characterized by skin pigmentation, endocrine disorders, and myxomas. It is particularly notorious for its tendency to exhibit aggressive cardiac myxomas. Herein, we present a case of a juvenile female patient diagnosed with Carney complex who underwent a right lateral mini-thoracotomy. Case presentation A 13-year-old girl presented with sudden-onset left hemiplegia and dysarthria. Magnetic resonance imaging revealed multiple areas of restricted diffusion. Echocardiography identified a tumor in the left atrium, suspected to be related to Carney complex based on her medical history and physical examination findings. Surgery was performed via right lateral mini-thoracotomy, which minimized the risk of embolism and ensured a cosmetically favorable outcome. The left atrial wall defect was repaired with autologous pericardium. At 3 years postoperatively, follow-up echocardiography indicated no tumor recurrence and normal cardiac function. Conclusions Ongoing follow-ups are essential due to the aggressive nature of the Carney complex and its high recurrence rates. Right lateral mini-thoracotomy offers the advantage of avoiding re-sternotomy and minimizing adhesion dissection, making it the optimal choice for this case.

Donor Posterior Atrial Flap Rotation for Left Atrial Cuff Reconstruction in Lung Transplantation.

We describe in detail a novel surgical technique for repairing donor anterior left atrial wall defect in vivo by rotating the posterior atrial flap during lung transplantation. This method can safely and effectively address the most common type of donor left atrial cuff defect: the anterior wall defect, with the intact posterior wall, typically retained when the donor heart is also used. During atrial cuff anastomosis in lung transplantation, the excess donor posterior atrial wall is trimmed into an atrial flap. After the posterior wall anastomosis is completed, the atrial flap is rotated 180° and used as a patch for anterior wall reconstruction anastomosis. After restoring blood flow, the flow rate of the pulmonary vein is normal and smooth as confirmed by transesophageal echocardiography. Compared with the traditional patch reconstruction method, the new method effectively reduces the atrial cuff anastomosis time. Our results showed no significant difference in pulmonary vein obstruction after reconstruction. Pulmonary artery systolic blood pressure was significantly lower and pulmonary function improved postoperatively in all groups, with no significant differences among the groups. The new technique provides a feasible strategy for the reconstruction of left atrial cuff defects and can improve the effective utilization rate of donor lungs.

Correlation between Epsilon Wave and Late Potentials in Arrhythmogenic Right Ventricular Cardiomyopathy-Do Late Potentials Define the Epsilon Wave?

Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder characterised by progressive fibrosis predominantly of the right ventricular (RV) myocardium, resulting in life-threatening arrhythmias and heart failure. The diagnosis is challenging due to a wide spectrum of clinical symptoms. The important role of ECG was covered in the current diagnostic criteria. The role of the epsilon wave (EW) is still under discussion. Aim: The aim of the study was to examine a potential association between the EW and late ventricular potentials (LPs) in ARVC patients (pts). The correlation between RV dilatation or dysfunction and LPs/EW was also analysed. Methods: The ARVC group consisted of 81 pts (53 men, aged 20-78 years) fulfilling 2010 International Task Force Criteria. 12-lead ECG, LPs, Holter, and ECHO were performed in all pts. The presence of EW was analysed in ECG by 3 investigators. LPs were detected by signal-averaged ECG (SAECG). SAECG was considered positive for LPs when at least two of the three following criteria were met: (1) the filtered QRS duration (fQRS) ≥ 114 msec; (2) the duration of the final QRS fragment in which low-amplitude signals lower than 40 μV are recorded (LAS-40 > 38 msec); and (3) the root mean square amplitude of the last 40 milliseconds of the fQRS complex (RMS-40 < 20 μV). The results were compared with a reference group consisting of 53 patients with RV damage in the course of atrial septum defect (ASD) or Ebstein's Anomaly (EA). Results: In the ARVC group, a significant relationship was observed between the occurrence of EW and the presence of LPs. EW was more common in the LP+ than in the LP- patients (48.1% vs. 6.9%, p < 0001; OR 12.5; 95% CI [2.691-58.063]). In ARVC pts, RVOT > 36 mm, RVIT > 41 mm, and RV S' < 9 cm/s were observed significantly more often in the LPs+ than in the LPs- group (OR [95% CI]: 8.3 [2.9-1.5], 6.4 [2.2-19.0] and 3.6 [1.1-12.2], respectively). In the ARVC group, any of fQRS > 114 ms, LAS > 38 ms, and RMS < 20 μV were significantly more frequent in EW+ pts. In multivariate analysis, the independent factors of the EW were LAS-40 and RV S'. In the LPs- subgroup, RVOT > 36 mm was more frequent in ASD/EA than in ARVC (70.4% vs. 25%, p = 0.002). Similarly, in the LPs- subgroup, RVIT > 41 mm was encountered more frequently in ASD/EA than in ARVC (85.2% vs. 48.3%, p = 0.004). Conclusions: In ARVC, there is an association between EW and LPs, with both probably resulting from the same process of fibrofatty substitution of the RV myocardium. Although RV dilatation is common in ASD and EA, it does not correlate with LPs.

Patterns and Complications of Congenital Heart Disease in Adolescents and Adults in Ethiopia.

Congenital heart disease (CHD) encompasses morphofunctional anomalies in the heart and circulatory system present at birth, which may not become apparent until later in life. In Ethiopia, there needs to be more understanding of the prevalence, patterns, and associated complications of CHD malformations. This study aimed to investigate the patterns and complications of CHDs among patients receiving follow-up care at a specialized university referral hospital in Ethiopia. A hospital-based cross-sectional study was conducted on 199 patients with CHDs to assess the patterns and complications of defects. Retrospective data were collected from 16,972 patients who had follow-ups at a cardiac clinic in 2021 using medical records, and a statistical analysis was performed with SPSS version 24. The most prevalent types of CHDs in our study population were atrial septal defects (ASDs) at 41.2% (82 cases), ventricular septal defects (VSDs) at 26.6% (53 cases), and patent ductus arteriosus (PDAs) at 9.5% (19 cases). Complications related to CHDs were observed in 69.3% (138) of patients, with 30.7% (61) experiencing a single complication and 39.2% (87) experiencing multiple complications. This study found a higher prevalence of CHDs in females (77.8%) compared to males, a trend consistent across various atrial and ventricular defect types. Individuals aged 15 to 25 years exhibited the highest incidence of ASD and VSD. Moreover, CHD-related anomalies were present in 69.3% of the patients studied.

Evaluating the Efficacy and Safety of Antihypertensive Drugs in the Treatment of Congenital Heart Disease in Paediatric Patients: A Comprehensive Study

CHD is the most common of all congenital anomalies causing mortality among children, characterized by structural abnormalities present at birth that vary in severity and complexity. Increased cardiac workload due to CHD may stipulate a growth delay in the affected children. The complexity of treatment is further compounded by medication errors, and hence, rigorous strategies of evaluation and management become necessary. This study was conducted to determine antihypertensive drug consumption patterns and to evaluate their therapeutic efficacy among 92 patients with CHD to meet the evaluation criteria, including appropriateness of treatment for the condition of the patient, adherence to indications, accuracy of dosage, and drug use practice. Females constituted the highest proportion of patients at 53.26 percent, infants were the most common age group at 43.48 percent, and Atrial seal defect accounted for 22.5 percent of the CHD patients. The antihypertensive drugs predominantly prescribed are Furosemide, used by 44.9% of the subjects, followed by Captopril, used by 30.3%, and Spironolactone, used by 10.1%. Therapy was evaluated for its effectiveness; appropriateness, both for the patient's indications and drug usage, was found to be very high, at 98.9% and 97.8%, respectively, whereas adherence to prescribed dosage was relatively lower at 62.7%. This study points toward further challenges in the management of antihypertensive treatment among children with CHD. It needs combined efforts by a pediatric cardiologist, pharmacist, nurse, and caregiver for total care to reduce the risk from treatment. Continuous monitoring and alterations in the plan of treatment, coupled with education, could be significant in optimizing medication adherence to improve long-term health outcomes in CHD children. The research done in this field, at large, improves pediatric cardiology practice to reduce morbidity and mortality rates associated with congenital heart disease around the world.

Minimally invasive cardiac surgeries in 2021: annual report by Japanese society of minimally invasive cardiac surgery.

Although minimally invasive cardiac surgery (MICS) procedures are widely performed throughout Japan, nationwide data regarding treated cases are limited. Up-to-date results for cardiovascular surgery are vital for quality control in clinical practice. Presented here is the 2021 annual report based on data from the Japan Cardiovascular Surgery Database (JCVSD). Records noted in the JCVSD of patients who underwent mitral valve surgery, aortic valve replacement (AVR), coronary artery bypass grafting (CABG), atrial septum defect (ASD) closure, or cardiac tumor resection via right or left minithoracotomy, as well as thoracoscopic- or port-assisted, or robotic-assisted approaches, in 2021 were examined. Perioperative parameters including mortality and morbidity was evaluated. The 30-day and in-hospital mortalities for isolated mitral valve repair (n = 1211) were 0.1% and 0.2%, respectively, while those for all mitral valve repair (n = 2017) were 0.05% and 0.2%, respectively. More than 100 facilities were found to perform fewer than five MICS mitral valve surgery cases per year. As for MICS-AVR, 30-day and in-hospital mortalities for isolated AVR (n = 818) were 0.5% and 0.5%, respectively, while those for all AVR (n = 987) were 0.6% and 1.1%, respectively. Additionally, those for MICS-CABG (n = 400) were 0.8% and 0.5%, respectively. Those for ASD (n = 183) and cardiac tumor (n = 96), were 0.5% and 0.5%, respectively, and 0% and 1.0%, respectively. This is the first report of MICS results of procedures performed in Japan based on the 2021 JCVSD data. Additional results obtained with a similar data collection method are expected and details on MICS are being collected starting 2024.

P-772 Assisted reproductive technology and the risk of minor congenital heart defects in the newborn

Abstract Study question Are children conceived through assisted reproductive technology (ART) at higher risk of minor congenital heart defects (CHD) compared with naturally conceived (NC) children? Summary answer Contrary to previous studies children conceived through ART had similar risk of minor CHDs compared with children conceived naturally. What is known already Congenital heart defects (CHD) may precipitate cardiac arrhythmias, lead to thromboembolic events, culminate in heart failure or even sudden cardiac demise. A systematic review and meta-analysis showed an elevated odds ratio (OR) for diagnosis of any CHD among children conceived through in-vitro fertilization (IVF) or intracytoplasmic sperm injection (ICSI) compared with NC children. Other studies indicate that ART is associated with an increased arterial blood pressure and vascular dysfunction in the pulmonary and systemic arterial vasculature. This association persists throughout childhood, adolescence, and early adulthood in individuals conceived through ART; this indicates a lasting impact on the cardiovascular circulatory system. Study design, size, duration The present study is a sub-study to the Copenhagen Baby Heart Study (CBHS), a prospective population-based cohort study of &amp;gt; 25 000 children born in the three main maternity wards in Copenhagen, Denmark, in 2016–2018. Transthoracic echocardiography (TTE) was performed on all children within 60 days of delivery and diagnosis of CHD was reviewed by a specialist in paediatric cardiology. Information regarding parental health, pregnancy, delivery, and the newborn was collected through Danish patient registries. Participants/materials, setting, methods This study includes all children conceived by ART in the CBHS (n = 1 784), and a matched group (1:4 by maternal age at birth) of NC children (n = 7 141). Exclusion criteria were genetic or chromosomal syndromes and heart surgery. Minor CHD was defined as an atrial (ASD) or ventricular septum defects (VSD), bicuspid aortic valve, coarctation of the aorta, pulmonary stenosis, or patent ductus arteriosus. We calculated ORs using logistic regression analyses. Main results and the role of chance The mean maternal age at birth was 34.9 (standard deviation [SD] ±4.4) and 35.1 years (SD ± 4.6) and the newborn mean age at TTE was 12.1 (SD ± 9.3) and 12.2 days (SD ± 9.4) in the ART and NC population, respectively. Parental CHD was registered in 40 (2.2 %) children in the ART group and 121 (1.7 %) in the NC group. Overall, 165 (9.2%) children conceived by ART and 611 (8.6%) NC children were diagnosed with a minor CHD. The most prevalent CHDs were VSD and ASD which accounted for 38.8 % and 35.2 % in children with CHD in the ART group, respectively, and 36.8 % and 33.9 % in children with CHD in the NC group. The unadjusted OR for minor CHD was 1.09 (95% CI 0.91–1.30). Adjustments for maternal age and parental CHD did not change the OR. There were no differences in ORs when stratifying by minor CHD type. We stratified by the method of ART: ICSI and IVF and frozen embryo transfer and fresh embryo transfer, and calculated risk of minor CHD comparing the methods and with NC children. We found no difference in any ORs, crude or adjusted. Limitations, reasons for caution Children born preterm, and children with severe minor and major CHDs, were underrepresented in the cohort indicating parents of such children chose not to participate in a research project that would include another hospital visit. Residual confounding is plausible because parental CHD diagnoses were retrieved from patient registries. Wider implications of the findings The number of children conceived through ART is increasing each year and previous research have found an increased risk of CHD in children conceived through ART. Our findings indicate no increased risk of minor CHD, providing comforting and significant information for advising individuals considering or undergoing ART treatment. Trial registration number NCT02753348

Multimodality Cardiovascular Imaging for Totally Video-Guided Thorascopic Cardiac Surgery.

Totally video-guided thorascopic cardiac surgery (TVTCS) represents one of the most minimally invasive access routes to the heart. Its feasibility and safety can be guaranteed by an experienced surgeon with skilled operative techniques under the guidance of a video signal via thoracoscopy and the imaging from transesophageal echocardiography. At present, this surgical approach has been applied for atrioventricular valve disease, atrial septum defects plus and partial anomalous pulmonary venous drainage, cardiac tumors, hypertrophic obstructive cardiomyopathy, aortic valve disease, and atrial fibrillation. Multimodality cardiovascular imaging, including echocardiography, X-ray, computed tomography (CT), magnetic resonance imaging (MRI) and cardiac catheterization, provides morphologic characteristics and function status of the cardiovascular system and a comprehensive view of the target anatomy. In this review, the benefits of multimodality cardiovascular imaging are summarized for the clinical practice of TVTCS, including the preoperative preparation, intraoperative guidance and postoperative supervision. The disease categories are also individually reviewed on the basis of multimodality cardiovascular imaging, to ensure the feasibility and safety for TVTCS. Cardiovascular imaging technologies not only confirm who is a candidate for this surgical technique, but also provide technical support during the procedure and for postop follow to assess the clinical outcomes. Multimodality cardiovascular imaging is instrumental to provide the requirements to solve the problems for conduction of TVTCS; and to provide individualized protocols with high-resolution and real-time dynamic imaging fusion.

Original Article--Clinical and Procedural Characteristics of Successful Transcatheter Device Closure of Ostium Secundum Atrial Septal Defect in Symptomatic Children Weighing

This retrospective study sought to determine the feasibility of transcatheter atrial septal defect device closure in patients less than 15 kg, as well as to assess complication rates and the reasons for unsuccessful device closure. In general, the risks associated with transcatheter atrial septal defect device closure are believed and reported to be relatively low, but the evidence stems from trials involving adults and older children. Current guidelines do not recommend atrial defect closure in device closure in children <15 kg, due to limited data available for feasibility and safety of device closure in this group of patients. Retrospective review of all patients who underwent elective transcatheter closure of ostium secundum atrial septal defect between September 2013 to February 2022. We excluded all children above 15 kg, as well as those with complex congenital heart defects. Major and minor complications were predefined and indications for referral were evaluated. We identified 81 patients meeting criteria with a median procedural age of 3 years (1 year-8 years), and median weight of 12 kg (4-15 kg). Successful device closure was achieved in 95.1% (77/81) and in 4.9% (4/81), the procedure was aborted. There was 1 major (1.2%) and 1 minor (1.2%) complication, total complication rate (2.4%). 100% of the referrals had right heart enlargement and exertional dyspnoea, 18.5% had recurrent lower respiratory tract infection and 9.9% had failure to thrive. Rate of resolution of residual shunt was 95.1%. at post-procedure day 1 and 98.8% at post-procedure 3 and 6 months respectively. Percutaneous atrial septal defect closure can be done effectively and safely in symptomatic children weighing less than 15 kg in experienced centres. However, deferral for closure until the historically established timeline of around 4-5 years of age should be strongly considered in asymptomatic children.

Pulmonary arteriovenous malformations in patients with previous brain abscess: a cross-sectional population-based study.

Pulmonary arteriovenous malformations (PAVMs) may cause recurrent brain abscess. The primary aim was to determine the prevalence of PAVM amongst survivors of brain abscess. The proportion with cardiac right-to-left shunts was also assessed post hoc. This was a cross-sectional population-based study of adult (≥18 years) survivors of cryptogenic bacterial brain abscess in Denmark from 2007 through 2016. Patients were invited for bubble-echocardiography to detect vascular right-to-left shunting and, if abnormal, subsequent computed tomography thorax for diagnosis of PAVM. Data are presented as n/N (%) or median with interquartile range (IQR). Study participation was accepted by 47/157 (30%) eligible patients amongst whom two did not appear for scheduled bubble-echocardiography. The median age of participants was 54 years (IQR 45-62) and 19/57 (33%) were females compared with 59 years (IQR 48-68, p = 0.05) and 41/85 females (48%, p = 0.22) in non-participants. Bubble-echocardiography was suggestive of shunt in 10/45 (22%) participants and PAVM was subsequently confirmed by computed tomography in one patient with grade 1 shunting. The corresponding prevalence of PAVM was 2% (95% confidence interval 0.06-11.8) amongst all examined participants. Another 9/45 (20%) were diagnosed with patent in persistent foramen ovale (n = 8) or atrial septum defect (n = 1), which is comparable with the overall prevalence of 25% amongst adults in the Danish background population. Undiagnosed PAVM amongst adult survivors of cryptogenic bacterial brain abscess is rare but may be considered in select patients. The prevalence of cardiac right-to-left shunts amongst brain abscess patients corresponds to the prevalence in the general population.

ECPR for prolonged Pediatric Cardiac Arrest, an outcome without major neurological compromise

Pediatric in-hospital cardiac arrest (IHCA) has been reported in 1–3% of pediatric intensive care unit (ICU) admissions and up to 6% of children admissions to the cardiac ICU. In the last 25 years, the survival to hospital discharge after pediatric IHCA has improved from 9% to 13.7% up to 35%. The improvement in outcomes was attributed in part to the application of ECMO as a rescue strategy when prolonged conventional CPR cannot restore spontaneous circulation. We report a case of a 4-month-old patient with a history of ventricular and septal defects, with left to right shunt and enlargement of left heart chambers that underwent surgery for the closure of the atrial and septal defects, and experienced complications that led to the use of ECMO in response to a prolonged cardiac arrest.

Retracted: Total Thoracoscopic versus Robotic Surgery for Repair of Atrial Septum Defect: A Propensity Matching Score Analysis

Retracted: Total Thoracoscopic versus Robotic Surgery for Repair of Atrial Septum Defect: A Propensity Matching Score Analysis

Comparison of ultrafast and fast track extubation after secundum atrial septal defect surgery in pediatric age group

Bacground Patients who underwent ultrafast track on the operating table and fast track extubation in the pediatric intensive care unit for 2 to 6 h after secundum atrial septal defect surgery in the pediatric age group were compared. Methods Between January 2013 and February 2017, 60 pediatric patients (24 boys, 36 girls; Mean age 7.5 ± 4.6 years) whose secundum atrial defect was closed were retrospectively analyzed. The patients were separated as those who were extubated on the operating table (Group1,n = 28) and those extubated in the pediatric intensive care unit within 2-6 h postoperatively (Group2,n = 32). Results No difference was found in demographic data and preoperative catheter information between the groups. Cardiopulmonary bypass time was 20(18–25)/27.5(20–30)minutes (p:0.001), the cross-clamp time was 10(10–15)/15(11–20)minutes(p:0.004), the postoperative drainage amount was 50(25-50)/60(32.5–100)ml(p:0.013), the length of stay in the intensive care unit was 1(1-–1)/1(1–2)day(p:0.025), the length of stay after intensive care was 3(2–3)/3(3–4)days(p:0.001) and the total hospital stay was 4(3–4)/5(4–5.5) days (p < 0.001), which were respectively shorter for the group 1 compared to 2. Postoperative blood product replacement, positive inotrope support, pericardial effusion, mortality, and morbidity were not detected in either groups. Conclusion In this study, it was observed that the UFT extubation was safe for the patients who were operated for secundum ASD, in the pediatric age group, and had a cross-clamp time not exceeding 15 min. It was found that the amount of drainage, length of stay in the intensive care unit, post-intensive care unit, and the total hospital stay of patients extubated on the operating table were shorter.

A Patient Undergoing Laparoscopic Radical Prostatectomy and Atrial Defect Closure Simultaneously: A Case Report

Background: Prostatectomy offers the greatest potential for a definitive cure for localized prostate cancer. Closure devices for secundum atrial septal defects have a good safety and efficacy profile. Simultaneous operation of the two types of surgery has not been reported to date. Case Presentation: A 65-year-old man was admitted to receive laparoscopic radical prostatectomy for prostate cancer under general anesthesia. The chest CT imaging unexpectedly showed that the heart and the pulmonary artery were obviously enlarged. Subsequently, a secundum atrial septal defect (ASD) with left-to-right shunt measuring 27*23mm was observed on transthoracic echocardiography (TTE). After a multi-disciplinary team (MDT) consisting of departments of urology, cardiac surgery, and anesthesiology was assembled to discuss the surgical procedure, an atrial septal defect closure device was scheduled to be implanted, followed by laparoscopic radical prostatectomy. The patient underwent both surgeries and was successfully discharged from the hospital. Conclusions: Adequate perioperative assessment and management are crucial for patients. We should pay more attention to patients with cardiac disease undergoing both cardiac and non-cardiac surgery.

New method for quantification of atrial septum defect shunting by echocardiography

Abstract Background Atrial septum defect (ASD) is a common congenital heart disease. Echocardiography is the preferred method for detection and characterization of ASD. Accurate quantification of shunting is pivotal, but often requires right heart catheterization (RHC). In the present study, we introduce a new non-invasive method for accurate quantification of the ASD shunt by combined transesophageal- (TEE) and transthoracic (TTE) echocardiography. Shunt by RHC was used as a reference method. Method In 22 adults with secundum ASD, left ventricular (LV) stroke volume (SV) was measured by the pulsed Doppler method using 2D TTE. The shunt volume passing through the ASD was quantified as the ASD area measured by planimetry from 3D TEE, multiplied with the time velocity integral (TVI) of the PW Doppler obtained at the center of the ASD by TEE. Finally, the ratio of the pulmonary to systemic shunt (Qp:Qs) was calculated as (LVSV+ASDvolume)/LVSV. Shunt by RHC was assessed by the Fick’s principle. Results Mean shunt was 2.2±0.7 (±SD) by echocardiography and 2.1±0.7 by RHC, with a mean difference of -0.1±0.4 (Figure 1). The correlation between shunt measured by RHC and echocardiography was strong (r=0.86, p&amp;lt;0.0001; Figure 2). Conclusion The present study demonstrates that ASD shunting can be quantified accurately by combining TTE and TEE, suggesting a new non-invasive approach in settings where RHC is not feasible or available.Figures

Elevation of CISD2 attenuates atrial aging

Abstract Background Age-related atrial cardiomyopathy is characterized by conduction disturbance, structural fibrosis as well as cardiac remodeling. Atrial cardiomyopathy promotes the development of atrial fibrillation, which is associated with a 5-fold increase in the risk of stroke. However, the molecular mechanisms underlying age-associated deterioration of atrial function remain unclear. Cisd2 is a mitochondrial outer membrane protein that regulates intracellular calcium homeostasis and mitochondrial integrity. Importantly, Cisd2 is a pro-longevity gene in mammals. Purpose In this study, we investigate the role of Cisd2 in atrial cardiomyopathy during aging. Methods CISD2 expression and atrial conductance were examined in different age human surgical samples. Atrial conductance was assessed and validated by immunofluorescence and Transmission electron microscope in Cisd2 knockout and overexpressing transgenic mice at young and old age. The mitochondrial functions and calcium homeostasis were investigated in the atrial HL-1 cell lines with Cisd2KO or re-expression. Transcriptomic analysis of mice atrial tissues was performed. Results The age-associated decrease of atrial Cisd2 protein, in human and mice, were associated with atrial conductance disturbance and intercalated disc disorganization. Furthermore, Cisd2 deficiency disrupts calcium homeostasis via dysregulation of Serca2a and Stim1 resulting in an increased level of basal cytosolic Ca2+ and mitochondrial Ca2+ overload in HL-1 atrial cardiomyocytes. Most strikingly, in Cisd2 transgenic mice, a persistently high level of Cisd2 is sufficient to delay age-associated atrial myopathy and attenuate age-related atrial structural defects and functional decline. In addition, it results in a younger atrial transcriptome pattern during old age. Conclusions The mitochondrial-associated membrane-bounded CDGSH iron-sulfur domain-containing protein 2 (CISD2) protein regulates cytosolic calcium homeostasis and mitochondrial integrity. Interestingly, an age-dependent decrease of atrial Cisd2 protein has been detected during aging in both humans and mice. Our findings indicate that decreased atrial Cisd2 is one of the causes of atrial myopathy during cardiac aging. A persistently high level of atrial Cisd2 delays atrial myopathy during aging and ameliorates age-related atrial dysfunction.

Correlation between epsilon wave and late potentials in arrythmogenic right ventricular cardiomyopathy. Do late potentials define the epsilon wave?

Abstract Introduction Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder characterised by progressive fibrosis predominantly of the right ventricular (RV) myocardium resulting in life-threatening arrhythmias and heart failure. The diagnosis is challenging due to a wide spectrum of clinical symptoms. The important role of ECG was covered in the current diagnostic criteria. The role of epsilon wave (EW) is still under discussion. Aim The aim of the study was to correlate the presence of EW with late ventricular potentials (LPs) in ARVC patients (pts). The correlation between RV dilatation /dysfunction and LPs/EW was also analysed. Methods The ARVC group consisted of 81 pts (53 men, aged 20-78 years) fulfilling 2010 International Task Force Criteria. 12-lead ECG, LPs, Holter, ECHO were performed in all pts. The presence of EW, defined as low-amplitude deflection observed between the end of the QRS complex and the onset of the T wave was analysed in ECG by 3 investigators. LPs were defined as low-amplitude, high-frequency waveforms at the end of the QRS complex on signal-averaged ECG (SAECG). SAECG was considered positive for LPs when at least two of the three following criteria were met: filtered QRS duration (fQRS) &amp;gt;114 ms, root-mean-square voltage in the terminal 40 ms (RMS 40) &amp;lt;20 uV, or low amplitude (&amp;lt;40 µV) signal (LAS 40) duration &amp;gt;38 ms. The results were compared with a reference group consisting of 53 pts with RV damage in the course of atrial septum defect (ASD) (29 pts, 8 men, aged 22-80 years) or Ebstein Anomaly (EA) (24 pts, 11 men, aged 23-55 years). Results In the ARVC group, a significant relationship was observed between the occurrence of EW and the presence of LPs. EW was more common in LP+ than in LP- patients (48.1% vs 6.9%, p&amp;lt;0001; OR 12.5). In ARVC a relationship was also found between the presence of LPs and increased RV inflow tract (RVIT) and RV outflow tract (RVOT) dimensions, as well as a reduced RV S' wave value. RVOT &amp;gt;36 mm, RVIT &amp;gt;41 mm and RV S’ &amp;lt;9 cm/s were observed significantly more often in the LPs+ than in LPs- group (OR: 8.3, 6.4 and 3.6, respectively). In the ARVC group, any of fQRS &amp;gt;114 ms, LAS &amp;gt;38 ms, and RMS &amp;lt;20 uV were significantly more frequent in EW+ pts. In multivariate analysis, the only independent factor of the EW was fQRS (OR: 1.043 [1.020-1.067], p&amp;lt;0.001; AUC: 0.852). In the LPs- subgroup, RVOT &amp;gt;36 mm was more frequent in ASD/EA than in ARVC (70.4% vs 25%, p=0.002). Similarly, in the LPs- subgroup, RVIT &amp;gt;41 mm was encountered more frequently in ASD/EA than in ARVC (85.2% vs 48.3%, p=0.004). Conclusions In ARVC, there is an association between EW and LPs, with both probably resulting from the same process of fibrofatty substitution of the RV myocardium. Although RV dilatation is common in ASD and EA, it does not correlate with LPs, possibly due to a lower degree of fibrosis in ASD/EA compared to ARVC.

Long-term prognosis of elderly patients undergoing atrial septum defect closure: are we acting too late?

Abstract Introduction Atrial septum defects (ASD) are the third most common congenital heart defect affecting adults and often goes unrecognized due to the absence of significant symptoms and unspecific signs at physical examination. Yet, long-term exposure to chronic right heart volume overload can have deleterious effects, with almost all patients becoming symptomatic by the fifth or sixth decade. However, whether ASD closure in an elderly patient affects their average life expectancy (ALE) is still unknown. Aim To assess the relationship between ASD closure (surgical or percutaneous) and ALE in a Portuguese cohort of patients older than 65 years old. Methods Single-centre retrospective study including all consecutive patients older than 65 years old that underwent isolated ASD closure (surgical or percutaneous) between January 1998 and December 2020. Baseline characteristics were assessed as well as the predicted ALE (as determined per pre-defined national ALE tables) for every given patient at the time of the ASD closure. Results A total of 37 patients with a mean age at closure of 69±5 years, 76% female, were included. Most patients presented with heart failure (62%), 14% with ischemic stroke, and 51% had an atrial arrythmia. 22% patients had severe tricuspid regurgitation, the mean systolic pulmonary artery pressure (SPAP) was 50±11mmHg and a median Qp/Qs of 2.1 [1.2-2.5]. Five patients were treated surgically. The mean size of the ASD was 21±7mm and those treated surgically had a higher mean size (24±7mm vs 21±9mm). During the mean FUP of 9.3±5.2 years, 16 patients died (37%). The mean age of death was 79.4±5.8 years and did not differ significantly from the expected mean ALE of 84.3±1.3 years (p=0.304). Patients who did not reach the predicted ALE at the time of the procedure (N=12, 33%) had higher SPAP (58±10 vs 46±8mmHg; p=0.001) and presented more frequently with severe tricuspid regurgitation (42 vs 12%, p=0.040) (figure 1A). There was no difference in terms of mortality regarding method of closure (p=0.096). Conclusion Overall survival of elderly patients that underwent late ASD closure did not differ significantly from the expected ALE, nevertheless one-third of the patients died prematurely. A premature death was associated with higher SPAP and a more severe tricuspid regurgitation. These results suggest that late-ASD closure does not overcome the effects of long-term right chamber overload and pulmonary hypertension, thus arguing in favour of earlier intervention.

An unexpected case of right heart dilation in the elderly

Atrial septal defects (ASD) represent the most common congenital heart defect diagnosed in adulthood. Adults with an ASD are often asymptomatic, but sometimes may present with non-specific symptoms such as dyspnea on exertion or exercise intolerance. Isolated sinus venosus atrial defect is an extremely rare anomaly. Sinus venosus defects occur more commonly in the superior (rather than inferior) portion of the embryologic sinus venosus and commonly occur with partial anomalous pulmonary venous return, particularly of the right upper pulmonary vein. We describe the case of an 80-year-old man with an undiagnosed, hemodynamically significant superior sinus venosus type of ASD who presented with persistent dyspnea and hypoxia after COVID-19 disease. Although cardiac magnetic resonance represents the gold standard for the morpho-functional evaluation of ASDs, transesophageal echocardiography is an accessible method for diagnosing the superior sinus venosus type of ASD and three-dimensional transesophageal echocardiography is useful for obtaining an "en face" view of the ASD and important surrounding structures.

Lower Extremity Rare Preaxial Polydactyly. A Case Report and Literature Review

The condition known as pediatric foot polydactyly can manifest itself in a broad range of malformations, from a single extra digit that is only connected to the rest of the foot by a thin band of connective tissue to intricate central foot duplications that involve the duplication of tarsal bones. The presentation of preaxial polydactyly of the foot is crucial to understand, even though it is quite uncommon. This is because in over half of the cases, several congenital malformations, such as syndactyly and atrial septum defects, have been described. The result of surgical reconstruction should be a foot that is stable, mobile, and pain-free, with five aesthetically pleasing toes. This should allow the patient to wear standard footwear and walk without experiencing any discomfort.