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Background: Covered stent correction for a sinus venosus atrial septal defect (SVASD) was first performed in 2009. This innovative approach was initially viewed as experimental and was reserved for highly selected patients with unusual anatomic variants. In 2016, increasing numbers of procedures began to be performed, and in several centers, it is now offered as a standard of care option alongside surgical repair. However, covered stent correction for SVASD is not recognized by regulatory authorities, and in the minds of many pediatric and adult congenital cardiologists and surgeons, the condition is viewed as treatable only by cardiac surgery with cardiopulmonary bypass., Methods: In April 2023, all centers identified from international conferences, publications, and colleague networks to be undertaking covered stent correction for SVASD were invited to participate in a retrospective audit of their procedures., Results: Data were received on 381 patients from 54 units over a 12-year period with 90% of procedures being performed over the past 5 years. Balloon-expandable stents (8 types) were used in the majority; self-expanding stents (4 types) were used in 4.5%. The commonest stent was the 10-zig covered Cheatham Platinum stent in 62% of cases. In 10 procedures, the stent embolized requiring surgical retrieval and repair of the defect, resulting in technically successful implantation in 371 of 381 (97.4%). Major complications (surgical drainage of tamponade, pacemaker implantation, surgery for pulmonary vein occlusion, and late stent removal) occurred in 5 patients (1.3%). Repeat catheterization to correct residual leaks was required in 7 patients (1.8%). Thus, 359 of 381 patients (94.2%) had successful correction without major complications or additional catheter interventions., Conclusions: This article details the exponential uptake of covered stent correction for SVASD during the past 5 years. Cardiopulmonary bypass was avoided in the majority of patients, and major complications were infrequent. Prospective registries with standardized definitions, inclusion criteria, and follow-up and comparative studies with surgery are now required to help support the extension of covered stent correction as an alternative standard-of-care option for patients with an SVASD.

Background: While surgery still remains the gold standard treatment for mechanical prosthetic valve thrombosis (MPVT) by many guidelines, the ultraslow low-dose thrombolytic regimen has been reported as a promising alternative., Methods: In this prospective single-center cohort, patients with acute MPVT were treated with an ultraslow low-dose thrombolytic regimen consisting of 25 mg infusion of recombinant tissue-type plasminogen activator (rtPA) over 25 h. The regimen could be repeated in case of failure until resolution/occurrence of adverse events or a maximum cumulative dose of 150 mg. The primary outcome was the complete MPVT resolution rate; other outcomes included first-dose success rate, major bleeding, thromboembolic events, mortality, and total thrombolytic dose/duration., Results: Between April 2018 to January 2024, 135 episodes of acute MPVT were treated with an ultraslow low-dose thrombolytic regimen in 118 patients. In 118/135 (87.4 %) episodes, right-sided prosthetic valve was involved. Complete success was achieved in 88.1 % of cases, with 39.5 % responding after the first dose. The median total dose was 50 mg over a median of 30 h. Only one fatal intracranial hemorrhage occurred (0.7 %), with no other bleeding or thromboembolic complications., Conclusion: The ultraslow low-dose thrombolytic regimen appears to exhibit high efficacy and acceptable safety in treating acute MPVT. Further large clinical trials are essential for validating these preliminary findings., Competing Interests: Declaration of competing interest The authors declare that they have no competing interests., (Copyright © 2024. Published by Elsevier B.V.)

Background: Extensive research has recognized the significant roles of non-coding RNAs (ncRNAs) in various cellular pathophysiological processes and their association with diverse diseases, including atrial septal defect (ASD), one of the most prevalent congenital heart diseases. This systematic review aims to explore the intricate involvement and significance of ncRNAs in the pathogenesis and progression of ASD., Methods: Four databases (PubMed, Embase, Scopus, and the Web of Science) were searched systematically up to June 19, 2023, with no year restriction. The risk of bias assessment was evaluated using the Newcastle-Ottawa scale., Results: The present systematic review included thirteen studies with a collective study population of 874 individuals diagnosed with ASD, 21 parents of ASD patients, and 22 pregnant women carrying ASD fetuses. Our analysis revealed evidence linking five long ncRNAs (STX18-AS1, HOTAIR, AA709223, BX478947, and Moshe) and several microRNAs (hsa-miR-19a, hsa-miR-19b, hsa-miR-375, hsa-miR-29c, miR-29, miR-143/145, miR-17-92, miR-106b-25, and miR-503/424, miR-9, miR-30a, miR-196a2, miR-139-5p, hsa-let-7a, hsa-let-7b, and hsa-miR-486) to ASD progression, corresponding to previous studies., Conclusions: NcRNAs play a crucial role in unraveling the underlying mechanisms of ASD, contributing to both biomarker discovery and therapeutic advancements. This systematic review sheds light on the mechanisms of action of key ncRNAs involved in ASD progression, providing valuable insights for future research in this field., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 AmiRsardari et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Key Clinical Message: This case report presents an uncommon variant of Ebstein anomaly, where all three tricuspid valve leaflets exhibited apical displacement-a rare finding. It illustrates the complexities in diagnosing and managing such atypical presentations, with successful surgical correction through annuloplasty. The report adds valuable insights to the limited literature on this congenital heart disease., Abstract: Ebstein anomaly (EA), a rare congenital heart disorder, presents with diverse clinical spectrums. This case report explores a distinctive manifestation of EA, where all three tricuspid valve (TV) leaflets exhibited apical displacement, highlighting a novel aspect in the presentation of this condition. A 44-year-old woman, under long-term medical surveillance for EA, showcased an atypical clinical trajectory marked by the apical displacement of all TV leaflets, which is uncommon in EA. Despite a predominantly asymptomatic course, recent exacerbation of symptoms prompted further evaluation. Diagnostic modalities, including echocardiography and cardiac magnetic resonance imaging, revealed severe tricuspid regurgitation concomitant with unprecedented apical displacement of the anterior, septal, and posterior tricuspid leaflets. The displacement of the anterior leaflet was contrary to typical embryonic valvular formation expectations, indicating a unique presentation within EA. The patient underwent annuloplasty surgery, which successfully rectified the anomalous TV architecture. Postoperative evaluation demonstrated mild residual tricuspid regurgitation, and the patient was discharged in stable condition. This case underscores the variability in EA presentations and accentuates the significance of tailored surgical interventions. The observation of apical displacement involving all TV leaflets adds a unique dimension to the existing EA literature, reinforcing the need for careful diagnosis and personalized treatment approaches., Competing Interests: The authors declare no conflicts of interest., (© 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.)

Most cases of congenital coronary artery fistula (CAF) resolve spontaneously, symptomatic patients with severe shunting require surgical intervention. Our aim is to evaluate success rate and outcome of CAFs treatment using transcatheter interventional methods.This retrospective study conducted on 28 CAF patients who were referred to Rajaie Cardiovascular Medical and Research Center in Tehran between 2015 and 2020. Baseline characteristics were collected by assessing hospital records, and patients were followed up annually for long-term evaluation. All of 28 patients gone throughtranscatheter closure of CAF. In 23 patient's it was proximal type (82.1%) and in 5 patients was distal type (17.9%). In 11 patients, the fistula originated from the RCA (39.3%) and in 11 patients, it originated from the LAD and Diagonal. Most common drainage site was the pulmonary artery (82.1%). Coil used in 23 patients(82.1%). PDA occluder (7.1%) for 2 patients. VSD occluder for one patient (3.6%) and VSD+PDA occluder combination was used for one patient (3.6%). Procedure failure was in only one patient. Non-significant remaining shunt in the injection immediately after the procedure was seen in 4 patients (14.3%), which was reduced during the follow-up. None of the patients had significant shunt or clinical symptoms during long-term follow-up. As for complications, fistula dissection occurred in only one patient.The transcatheter interventional approach for the treatment of CAFs leads to favorable long-term results., Competing Interests: All authors declare no competing interests., (© 2024 The Author(s).)

Objectives: Tetralogy of Fallot (TOF) is a common congenital heart disease which should be corrected. The recommended time for the Tetralogy of Fallot Total Correction (TFTC) surgery is during the infancy for the possible difficulties during the surgery and the related issues. However, sometimes TOF is diagnosed and managed during the adulthood., Methods: This study is a descriptive and retrospective one which included all patients who underwent TFTC at the age of 15-year and older in 10 years (between the years 2010 and 2020) to identify short-term (in-hospital mortality, ICU stay, postoperative bleeding, respiratory complications after the surgery such as pulmonary edema, pneumonia, etc.) and one-year (left ventricle ejection fraction (LVEF), right ventricle (RV) ejection fraction, the severity of tricuspid and aortic regurgitation after surgery) outcomes. All data were taken from medical records at Rajaie Cardiovascular Medical and Research Center. Data were analyzed using SPSS 22., Results: 94 patients with the mean ± SD age of 26.7 ± 9.6 years were enrolled. Most of them were male (59.6%) (P-value: 0.009). In-hospital mortality in our study were 5.3%. Tricuspid regurgitation (TR) was significantly resolved after the surgery (P-value: 0.006). Of 17 (18.1%) patients with small or hypoplastic pulmonary artery (PA) branches, 14 patients had acceptable PA branch size after surgery., Conclusion: TFTC at an older age is safe with acceptable results. Age is not a contraindication for TFTC and surgery should be recommended if the patients are diagnosed with TOF in adulthood. Also, the TOF diagnosis should be considered in adult patients with suspicious signs and symptoms., (© 2023. The Author(s).)

Background: Right ventricular hypertrophy can be caused by conditions such as pulmonary stenosis and pulmonary hypertension. ECG is a readily available and affordable test, the aim of this study was the evaluation of the electrocardiographic aspects of pulmonary stenosis, and pulmonary hypertension., Methods: A list of patients diagnosed with isolated pulmonary stenosis and pulmonary hypertension patients hospitalized and treated between 2019 and 2021 were extracted from the hospital archives. Furthermore, the ECG of the patients was analyzed in terms of the prevalence of the variables in the study using FECG Caliper software. Finally, the data of 93 patients (in both groups) were analyzed., Results: In this study, 46 patients were in the severe pulmonary stenosis group, and 49 were in the severe or moderate-to-severe pulmonary hypertension group. The heart rate in the pulmonary hypertension group was significantly higher. R/S > 1 in precordial leads differs between the two groups and higher amplitude R wave in V1(p-value = 0.05). in the pulmonary stenosis group. While in the pulmonary hypertension group, R wave growth occurs later, and this ratio is greater than one after V4. Bundle block in the form of RBBB(p-value <0.001) and maximum QRS duration is more in the pulmonary stenosis group(p-value = 0.001)., Conclusion: Our findings show the different strains of the right ventricle in two groups. It can be concluded that the effects of severe pulmonary stenosis on the ECG are more on the QRS wave and in the form of a block, while severe pulmonary hypertension affects the ST segment and T wave., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Tetralogy of Fallot is a common congenital heart disease characterized by cyanosis. The primary treatment approach involves corrective surgery typically performed within the first year of life to achieve complete resolution. However, certain patients may undergo surgery at an older age. This study seeks to assess the efficacy of surgery by examining the midterm outcomes of total correction of Tetralogy of Fallot when performed in older individuals., Methods: This interventional-longitudinal study focused on patients who underwent complete surgery to correct tetralogy of Fallot at an advanced age of over 15 years. All of the participants were referred to the Shahid Rajaei Heart and Vascular Center, which is a referral center for congenital heart diseases in Iran, between 2010 and 2020. The surgical procedures for these patients involved primary total correction of tetralogy of Fallot or surgery following by shunt implantation. Prior to the surgery, the necessary information was gathered from the patients' medical records. The patients were then monitored over a 5-year period, during which they received regular check-ups from cardiologist with fellowship in adult congenital heart disease., Results: A total of 94 participants were enrolled in the study, with an average age of 26.7 ± 9.6 years. Notably, the majority of the participants were male. The study reported a late mortality rate of 3.2%. Furthermore, 17 patients, constituting 18% of the cohort, underwent a secondary surgical procedure. This secondary surgery encompassed 14 cases of Pulmonary Valve Replacement (14.8%) and 3 cases of Ventricular Septal Defect repair (3.1%)., Conclusion: While the optimal age for total correction of Tetralogy of Fallot is conventionally considered to be within the first year of life, this study demonstrated that surgical intervention performed at a later stage of life can yield favorable midterm prognoses. It is imperative to emphasize that individuals unable to undergo surgery at the ideal age due to a multitude of factors should not be deprived of the potential benefits associated with surgical intervention., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Khajali, Mohammadi, Toloueitabar, Maleki, Saedi, nourouzi, Mazloum-Zadeh, Chenaghloo, Jalali, Tatari and Aliramezany.)

Adjusting the exact warfarin dose has always been challenging since it has a narrow therapeutic window. Numerous factors, including poor drug compliance, drug-drug interactions, and malabsorption syndromes, affect the warfarin plasma concentration, leading to oversensitivity or resistance to warfarin. Patients who need more than 15 mg/d of warfarin for maintained anticoagulant effects are considered warfarin resistant. We describe a 62-year-old man referred to our center with bruising on his feet in June 2021. The patient had a history of valve replacement (mechanical prosthetic valves in 2013), hypothyroidism, and atrial fibrillation. He presented with warfarin resistance (first noticed in 2013) and did not reach the desired warfarin therapeutic effect despite receiving 60 mg of warfarin daily. Upon admission, the patient was on warfarin (100 mg/d) with an international normalized ratio (INR) of 1.5. He underwent laboratory and molecular genetic tests, which showed no mutation in the CYP2C9 and VKORC1, the genes associated with warfarin resistance. A stepwise diagnosis is required to identify the underlying cause. Assessing the patient's compliance, drug history, dietary habits, malabsorption diseases, and genetics may be necessary. We evaluated these possible reasons for resistance and found no correlation. The patient's warfarin intake was monitored closely to reach the INR therapeutic target of 3-3.5. He decided to leave the hospital with personal consent. He was discharged with a cardiologist referral and 24 warfarin tablets daily (120 mg/d) with an INR of 1.8. The patient was followed up 6 months and 2 years after discharge and was on the same daily dose of warfarin as at discharge, with no complications., (Copyright© 2023 Tehran University of Medical Sciences. Published by Tehran University of Medical Sciences.)

Post-myocardial infarction ventricular septal rupture is the most catastrophic mechanical complication with high morbidity and mortality, reaching 94% in patients treated conservatively and 47% in patients undergoing cardiac surgery procedures (30-day mortality). In this review article we tried to review trans-catheter closure approach and explain elaborately device selection based on real-world patients in this fatal complication., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Congenitally corrected transposition of great arteries is a rare anomaly which are responsible for 0.5% of all CHDs and can be associated with other congenital cardiac abnormalities. Association of congenitally corrected transposition of great arteries and isolated atrial septal defect is a very rare condition, and management of this association is challenging. In this paper, we describe three patients with congenitally corrected transposition of great arteries and isolated atrial septal defect who were admitted to our clinic and all of them underwent percutaneous closure of defect. From 2017 to 2020, we visited three patients with congenitally corrected transposition of great arteries and isolated atrial septal defect. Our patients' ages ranged from 28 to 38 years. All of them underwent percutaneous atrial septal defect device closure without any complications. Patients were discharged from hospital in good condition with a daily dose of Aspirin 80 mg and Plavix 75 mg. For all of them, follow-up echocardiography was performed the day after the procedure at 1, 3, and 6 months later and showed the function of the right-sided left ventricle improvement and the severity of the mitral regurgitation was reduced. Furthermore, clinical evaluation also indicated functional class improvement. Although the cases of percutaneous transcatheter closure are few and cannot be regarded as strong evidence to recommend this procedure, the outcomes are promising and can demonstrate that this approach is practical.

Introduction: Prevalence of congenital heart disease (CHD) has increased in recent years, and patients with CHD have to deal with sequelae of the multiple medical and surgical treatments that can affect different aspects of their life which could be reflected in their quality of life (QoL). In Iran, to the best of our knowledge, QoL of adult patients with CHD has not been investigated, so this study aimed to investigate the QoL of adult patients with CHD referred to two Iranian outpatient settings., Methods: In 2022, a double center, cross-sectional study was performed on adult patients with CHD receiving out-patient care at Besat Clinic in Kerman, Iran, and Clinic of Shahid Rajaee Hospital in Tehran, Iran. Inclusion criteria were documented diagnosis of CHD based on guidelines, ejection fraction of above 45% and age of 18-55 years. We recruited a sample of 120 individuals using a simple random sampling method. At the day of referring to the adult congenital heart diseases clinic, after ensuring their written consent to participate in the study, we asked patients to fill in two questionnaires of demographic questionnaire and Persian version of the Ferrans and Powers Quality of Life Index. Data were analyzed using descriptive statistics and chi square via SPSS-22., Results: A total of 101 patients with a mean age of 31.05 years participated in the study. Demographic and socio-economic factors had no significant relationship with the patients' QoL. But factors related to the disease were significantly different among QoL groups ( P -value > 0.05)., Discussion: Adults with CHD had a low QoL, which is not related to their demographic and socio-economic factors. That is, the existence of the disease alone and its accompanying complications can affect the QoL of these people. Hence, the mental health of adult patients with CHD should be monitored during their routine cardiac care., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Khajali, Sayyadi, Ansari and Aliramezany.)

Takayasu Arteritis (TA) is a chronic idiopathic granulomatous pan-arteritis affecting the pulmonary artery, the aorta, and its principal derived branches. The majority of TA patients are female (82.9%-97.0%). Due to the inflammatory character of the illness, arterial stenosis therapy must be treated differently than the atherosclerosis process. In this review paper, we outline a strategy using real-world challenging cases., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Coronary artery fistulas (CAFs) are accounted as the most congenital coronary anomalies. As their natural course is progressive dilatation of the feeder arteries and the fistula tract, so, catastrophic complications are frequently illustrated by aging. Even in those with asymptomatic small fistulas, close follow-up is mandatory to prevent subsequent sequelas. In patients with medium or large-sized fistulas, irrespective of symptoms, closure (either by surgical ligation or transcatheter closure) is recommended. In the current era of advances in the equipment and devices and also innovations in percutaneous closure techniques and preprocedural imaging, TCC is now regarded as the preferred strategy for CAF closure except in some high-risk cases. So, by appropriate case selection modalities, pre-procedural planning, and determining the closure techniques, recent small case-series studies have been reported good final angiographic and clinical results by TCC. In this article, we have introduced several transcatheter closure techniques by details; also, we have recommended more multi-center trials with long-term clinical follow-up to address the best treatment options in these patients., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Nonetheless, in principle, bioprosthetic heart valves are often preferred to mechanical valves in that not only do they obviate the need for high-level systemic anticoagulation and the attendant bleeding risks, but also higher rates of prosthetic valve thrombosis have been reported observed in the tricuspid position. The transcatheter tricuspid valve-in-valve (TVIV) procedure has expanded the horizons of cardiac surgery by allowing the implantation of stent-valves within degenerated bioprostheses in older adults and even young patients as the reported rate of the mortality of redo surgery in antecedent studies is at least 37%. Fluoroscopic guidance can assist the operator in implanting a bioprosthetic valve by radiopaque findings in the ideal position and depth; nonetheless, the complete radiolucency of some bioprosthetic valves represents a significant hurdle. In this state-of-the-art manuscript, we decided to deeply explain the appropriate heart-team approach in this scenario., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Whereas most patent ductus arteriosus (PDAs) are identified and treated in the childhood, and the long term outcome studies have approved the outstanding results (positive cardiac reverse remodeling) in these group of patients, the outcome of percutaneous PDA closure in adults is not comprehensible. The aim of this retrospective cohort study which consecutively recruited 121 adult patients (age >16 years) who underwent the transcatheter closure (TCC) of PDAs between 2012 and 2018 is, evaluation at least 1-year follow-up of adult patients after PDA closure and compare the hemodynamic and the cardiac reverse remodeling findings with the previous studies in adults and children., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Aims: Thrombolysis is an alternative to surgery for mechanical prosthetic valve thrombosis (MPVT). Randomized clinical trials have yet to test the safety and efficacy of a proposed ultraslow thrombolytic infusion regimen., Methods and Results: This single-centre, open-label, pilot randomized clinical trial randomized adult patients with acute obstructive MPVT to an ultraslow thrombolytic regimen [25 mg of recombinant tissue-type plasminogen activator (rtPA) infused in 25 h] and a fast thrombolytic regimen (50 mg of rtPA infused in 6 h). If thrombolysis failed, a repeated dose of 25 mg of rtPA for 6 h was administered in both groups up to a cumulative dose of 150 mg or the occurrence of a complication. The primary outcome was a complete MPVT resolution (&gt;75% fall in the obstructive gradient by transthoracic echocardiography, &lt;10° limitation in opening and closing valve motion angles by fluoroscopy, and symptom improvement). The key safety outcome was a Bleeding Academic Research Consortium type III or V major bleeding. Overall, 120 patients, including 63 (52.5%) women, at a mean age of 36.3 ± 15.3 years, were randomized. Complete thrombolysis success was achieved in 51 patients (85.0%) in the ultraslow-regimen group and 47 patients (78.3%) in the fast-regimen group [odds ratio 1.58; 95% confidence interval (CI) 0.25-1.63; P = 0.34]. One case of transient ischaemic attack and three cases of intracranial haemorrhage (absolute risk difference -6.6%; 95%CI -12% -0.3%; P = 0.07) were observed only in the fast-regimen group., Conclusion: The ultraslow thrombolytic regimen conferred a high thrombosis resolution rate without major complications. Such findings should be replicated in more adequately powered trials., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background: Coarctation of the aorta is a common cardiac disease in Turner syndrome. Evidence indicates that surgery and balloon angioplasty in infants and small children do not have any added risk of mortality or complication in these patients. Stenting in older patients may, however, pose higher risks of arterial wall injury and mortality., Methods: In this case series, we describe 15 patients with coarctation of the aorta in Turner syndrome: 9 received stenting, 4 underwent surgery, and 2 were treated via balloon angioplasty., Results: Dissection occurred in 2 patients after stenting: 1 in the aorta and the other in the external femoral artery. Both were managed promptly without any mortality or serious damage, one percutaneously and the other surgically., Conclusions: Awareness of increased risks and preparedness for prompt interventions in case of an acute arterial wall injury are recommended when coarctation stenting is done for a patient with Turner syndrome., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Ebstein's anomaly is an uncommon congenital malformation which might be associated with atrial septal defect and atrio-fascicular Mahaim. Here, we report a known case of Ebstein's anomaly with atrial septal defect and concomitant atrio-fascicular Mahaim pathway who underwent atrial septal defect device closure and concurrent ablation of accessory pathway., (© 2022 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.)

Background: Structural heart disease (SHD) has great impacts on healthcare systems, creating further public health concerns. Proper data are scant regarding the magnitude of the affected population by SHD., Objectives: This study aimed to determine the prevalence of SHD among children and adolescents in an Iranian population., Methods: In this population-based study, a multistage cluster-random sampling was used to choose schools from the Tehran urban area. All students were examined using a handheld Vscan device by echocardiographer, and the results were concurrently supervised and interpreted by cardiologists. All the major findings were reevaluated in hospital clinics., Results: Of 15,130 students (6-18 years, 52.2% boys) who were examined, the prevalence of individuals with congenital heart disease (CHD) and cardiomyopathy was 152 (10.046 per 1,000 persons) and 9 (0.595 per 1,000 persons), respectively. The prevalence of definite and borderline rheumatic heart disease (RHD) was 30 (2 per 1,000 persons) and 113 (7.5 per 1,000 persons), correspondingly. Non-rheumatic valvular heart disease (VHD) was also detected in 465 (30.7 per 1,000 persons) students. Of all the pathologies, only 39 (25.6%) cases with CHD and 1 (0.007%) cases with RHD had already been diagnosed. Parental consanguinity was the strongest predictor of CHD and SHD (odds ratio [OR]: 1.907, 95% CI, 1.358 to 2.680; P < 0.001 and OR, 1.855, 95% CI, 1.334 to 2.579; P < 0.001, respectively). The female sex (OR, 1.262, 95% CI, 1.013 to 1.573; P = 0.038) and fathers' low literacy (OR, 1.872, 95% CI, 1.068 to 3.281; P = 0.029) were the strongest predictors of non-rheumatic VHD and RHD, correspondingly., Conclusions: The implementation of echocardiographic examinations for detecting SHD among young population is feasible which detected SHD prevalence in our population comparable to previous reports. Further studies are required to delineate its economic aspects for community-based screening., Competing Interests: SH reports grants from the NIMAD, during the conduct of the study. All other authors have none to declare., (Copyright: © 2022 The Author(s).)

Regarding to more invasive treatment of atrial arrhythmia (atrial fibrillation ablation) and pulmonary vein isolation (PVI), the rate of acquired pulmonary vein stenosis (PVS) is increasing and at present, PV ablation for AF has become the principal cause of PVS in adult patients. On the other way, by improvement in procedural techniques, equipment, and the experience of the operators, the incidence of PVS has been decreased. There is some controversy about the manner of follow-up of these patients and in most centers, just symptomatic patients are considered for imaging and treatment. Almost always, those with PV stenosis more than 70% or multiple PV involvement become symptomatic and if give them up without treatment, pulmonary symptoms and finally irreversible pulmonary hypertension will occurred. So, intensive pursue after the procedure is highly recommended. Whereas in pediatric patients with congenital or acquired PVS, the best treatment approach is surgery, in adult patients, the preferred type of treatment is the transcatheter intervention with high acute success rate. In this present review, we have scrutinized about the diagnostic modalities, the indications for intervention, the diverse treatment strategies, and principally clarify an accurate stepwise approach during transcatheter procedure., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: CHD influences many aspects of life in affected individuals. Puberty, a major aspect of development, is a concern for patients and families., Objectives: We investigated pubertal status in children and adolescents with CHD., Methods: Patients with CHD aged 6-18 were enrolled. Cardiac diagnoses were confirmed using history, examination, and paraclinical tools including echocardiography. An endocrinologist determined pubertal stages, and the second Tanner stages for pubarche (P2), thelarche (B2), and gonadarche (G2) were considered as the pubertal onset. A study with a large sample size on pubertal onset in a normal population was used for comparison., Results: Totally, 451 patients (228 girls and 223 boys) at a median (10th-90th percentile) age of 10.79 (8.02-14.28) years for the girls and 10.72 (8.05-14.03) years for the boys were enrolled. The median (10th-90th percentile) ages at B2 and P2 in the girls with CHD were 10.77 (9.55-12.68) and 10.53 (9.39-12.28) years, respectively, which were higher than the median ages of 9.74 (8.23-11.94) and 10.49 (8.86-12.17) years in the normal girls.The median (10th-90th percentile) ages at G2 and P2 in the boys with CHD were 11.04 (8.85-13.23) and 11.88 (9.78-13.46) years, correspondingly, which were higher than the median ages of 9.01 (6.00-11.84) and 10.34 (6.84-13.10) years in the normal boys., Conclusions: Pubertal onset could be delayed in children with CHD when compared with the normal population.

Ebstein anomaly, a rare congenital heart disease, is defined as displacement of hinge points of septal or posterior tricuspid leaflets but not anterior leaflet. Here, we report the case of a 35-year-old woman with an extremely rare pattern of Ebstein anomaly (EA) with all three tricuspid leaflets displaced downward to the apex., Competing Interests: The authors have no conflicts of interest to declare., (© 2022 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.)

Objectives: This study sought to compare the safety and efficacy of the balloon-expandable stent (BES) and the self-expandable stent (SES) in the endovascular treatment of coarctation of aorta., Background: Coarctoplasty with stents has conferred promising results. Although several nonrandomized studies have approved the safety and efficacy of the BES and the SES, no high-quality evidence exists for this comparison., Methods: In the present open-label, parallel-group, blinded endpoint randomized pilot clinical trial, adult patients with de novo native aortic coarctation were randomized into Cheatham-platinum BES and uncovered nitinol SES groups. The primary outcome of the study was a composite of procedural and vascular complications. The secondary outcomes of the study consisted of the incidence of aortic recoarctation, thoracic aortic aneurysm/pseudoaneurysm formation, and residual hypertension at a 12-month follow-up., Results: Among 105 patients who were screened between January 2017 and December 2019, 92 eligible patients (32 women [34.8%]) with a median age of 30 years (IQR: 20-36 years) were randomized equally into the BES and SES groups. The composite of procedural and vascular complications occurred in 10.9% of the BES group and 2.2% of the SES group (odds ratio: 0.18; 95% CI: 0.02-1.62; P = 0.20). Aortic recoarctation occurred in 5 patients (5.4%), 3 patients (6.5%) in the BES group and 2 patients (4.3%) in the SES group (odds ratio: 0.65; 95% CI: 0.10-4.09; P = 0.64). Only 1 patient (1.1%) was complicated by aortic pseudoaneurysm. Hypertension control was achieved in 50% of the study population, with an equal distribution in the 2 study groups at the 12-month follow-up., Conclusions: Both the BES and the SES were safe and effective in the treatment of native coarctation., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Atrial septal defects (ASDs) are one of the most common congenital cardiac abnormalities. Repair of these defects is a quite secure and routine operation. The most common complications were postoperative atrial arrhythmias, sinus arrhythmia, and atrioventricular (AV) blocks requiring pacemaker implantation, mediastinal bleeding, and transient ischemic attacks (TIAs) or strokes. Iatrogenic diversion of the inferior vena cava (IVC) to the left atrium (LA) during surgical closure of an ASD is a very rare complication., Case Report: We reported a patient who had a history of cardiac surgery in another center at the age of seven and was introduced to our clinic with complaints of dyspnea and cyanosis of extremities on exertion. She underwent surgery in our center with diagnosis of iatrogenic diversion of IVC to LA., Conclusion: The most common mechanism suggested is a large eustachian valve being mistaken for the inferior rim of the ASD., (© 2022 Isfahan Cardiovascular Research Center & Isfahan University of Medical Sciences.)

Ductus arteriosus is a physiological structure if not closed after birth, may lead to many complications. Today, trans-catheter closure of patent ductus arteriosus with Occluder devices is the preferred method. Surgical ligation is used only in certain cases such as large symptomatic patent ductus arteriosus in very small infants and premature babies; unfavourable structure of the duct or economic considerations. In this article, we described haemodynamic and morphological characteristics of five patients with large patent ductus arteriosus which were occluded with Amplatzer device.From 23 January, 2010 to 31 July, 2018, five patients referred to our clinic with large patent ductus arteriosus and pulmonary arterial hypertension for further evaluation. After assessing them with various diagnostic methods, we decided to close defect with ventricular septal defect Occluder device. Patients aged 21-44 years and one of them was male. Ductus closure was successfully done with ventricular septal defect Occluder device. Closure was successful for all of them but in one case, whose device was embolized to pulmonary artery after 24 hr and he underwent surgery.Trans-catheter closure of large patent ductus arteriosus in adult patients with pulmonary hypertension is feasible. Despite the fact that complications may occur even with the most experienced hands, the 'double disk' Amplatzer ventricular septal defect muscular Occluder could be advantageous in this setting.

Background: Ebstein's anomaly is one of the challenging congenital heart diseases (CHDs) that is presented with different anatomical and clinical symptoms. For this reason, patients with this complication require exact diagnostic methods and appropriate treatment approaches. In addition, multiple cardiac defects accompany this anomaly., Case Report: In this study, we describe a rare associated defect in an adult patient with Ebstein's anomaly and proper surgical methods performed for her., Conclusion: The most important issue in adult patients with Ebstein's anomaly is to pay close attention to the presence of associated anomalies, in which careful examination and use of para-clinical methods is very helpful. Furthermore, accurate diagnosis of the associated defects determines the treatment and surgery of patients., (© 2021 Isfahan Cardiovascular Research Center & Isfahan University of Medical Sciences.)

Background: Peripheral pulmonary artery stenosis (PPAS) is a rare and underdiagnosed phenomenon that is reported infrequently in adult patients. Most patients with PPAS have concomitant congenital heart diseases, a history of palliative surgical therapies during childhood, or syndromic characteristics. Acquired cases are rare, and they are underestimated in adulthood and managed inappropriately., Case Presentation: This case series describes 3 adult patients with PPAS of diverse etiologies and discusses their underlying causes, diagnostic modalities, and treatment strategies., Conclusions: In patients with PPAS, sufficient heed should be paid to endovascular interventions such as balloon dilation and primary or bailout stenting, not least vis-à-vis the type and size of balloons or stents as well as complications and preventive strategies., (© 2021. The Author(s).)

Background: Secundum-type atrial septal defects (ASD) constitute 8% to 10% of congenital heart defect. Secundum ASDs can be closed either percutaneously or surgically. However, ASD device closure has proven to be technically safe and feasible; it is not free of complications. These complications include device embolization/malposition which have been reported in 3.5% of cases, arrhythmia, and pericardial effusion in 2.6% and 0.5-1.5% respectively, device thrombus, residual shunting, and impingement of the device on the adjacent structures., Case Presentation: We introduce three patients with secundum ASD who had preexisting pericardial effusion, device closure was performed for them, and after the procedure, the effusion size progressed significantly. We used multimodality imaging to diagnose the cause of pericardial effusion (PE). Cardiac erosion was diagnosed in one of the patients that managed surgically. We did not found any specific procedure-related cause for worsening the pericardial effusion in the other two patients., Conclusion: Several reasons include procedure-related complication and other systemic causes should be considered in patients who develop pericardial effusion after trans catheter closure of ASDs.

A 23-year-old woman with history of tetralogy of Fallot presented with new hypertension and constitutional symptoms first diagnosed as coartation of aorta based on primary imaging but that turned out to be Takayasu arteritis after more evaluation by laboratory data and complementary imaging modalities . < Learning objective: Inflammatory vasculitis involving large vessels such as Takayasu arteritis might mimic congenital structural heart diseases like aortic coarctation in rare cases due to post inflammatory strictures in thoracic aorta and aortic arch main branches. This report demonstrates the importance of history taking, physical examination, and using different imaging modalities for the most accurate diagnosis.>., Competing Interests: The authors do not have any conflict of interest., (© 2021 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

Aberrant systemic artery to the lung without evidence of sequestration is a rare congenital anomaly. Treatment of choice for this anomaly is controversial. A 27-year-old man with bicuspid aortic valve (AV) and a history of AV replacement was admitted to our center with hemoptysis. Aberrant systemic artery to the lung without sequestration was diagnosed. Endovascular closure of aberrant vessel with septal occluder was performed. After 2 years the previous device was embolized to the distal part and he came back with hemoptysis. The second endovascular procedure was performed with a larger device with a successful result. There are surgical and interventional treatment options for systemic arterialization to a normal lung without sequestration. In this case we used Amplatzer septal occluder (St. Jude Medical, Minneapolis, MN, USA) for occlusion of the aberrant vessel. < Learning objective: Systemic arterialization of the lung is a rare congenital anomaly, the treatment of choice for this anomaly is controversial, using septal occluder may be a useful method for management of this anomaly. Here we report a patient with this rare anomaly who had an educational clinical course. The type of management performed is not reported in other articles with similar cases.>., Competing Interests: No potential conflict of interest relevant to this article was reported., (© 2021 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

An interrupted aortic arch (IAA) is a rare type of congenital heart disease, and few patients survived into the adulthood. Surgical reconstruction is still the recommended approach, despite its complexity and considerable complication. In addition, patients with IAA usually suffer from several other important congenital heart anomalies, which increase the complexity of surgical management of IAA. Although endovascular therapy has replaced surgery in the treatment of the majority of non-IAA, its applicability in IAA is still matter of debate. In the present review, we have discussed about various therapeutic solutions of IAA, and present a stepwise approach for its endovascular management., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Familial hypercholesterolemia (FH) is a common, yet underdiagnosed, genetic disorder characterized by lifelong elevated low-density lipoprotein cholesterol levels, which can increase the risk of early-onset coronary artery disease (CAD). In the present study, we screened the nucleotide variations of the LDLR and PCSK9 genes, as well as a part of the APOB gene, in Iranian patients with FH and premature CAD to find the genetic cause of the disorder. Fifteen unrelated individuals with a clinical diagnosis of FH and premature CAD were recruited. Direct DNA sequencing was applied to screen the whole coding exons and exon-intron boundaries of the LDLR and PCSK9 genes and the main parts of their introns, together with exon 26 of the APOB gene. The pathogenicity of the identified mutations was investigated via either segregation analyses in the family or in silico predictive software. Six different point mutations (p.Cys148Tyr, p.Cys216Tyr, p.Cys302Trp, p.Cys338Trp, p.Leu479Gln, and p.G593Afs ∗ 72) in LDLR and a double mutation (p.Asp172His and p.Ala53Val) in both LDLR and PCSK9 genes were identified in seven families with clinically diagnosed FH (43%), whereas no pathogenic mutations were found in eight families with clinically diagnosed FH. This study is the first to identify 1 pathogenic mutation in the LDLR gene (c.1014C > G [p.Cys338Trp]) and to cosegregate it from the affected individual in the family. No mutations were found in the APOB gene, whereas several silent mutations/polymorphisms were identified in the LDLR and PCSK9 genes. Genetic testing and reports on nucleotide alterations in the Iranian population are still limited. Our findings not only further confirm the significant role of FH in the incidence of premature CAD but also enlarge the spectrum of LDLR and PCSK9 variations and exhibit the heterogeneity of FH in Iranians. In patients with no mutation in the examined genes, the disease could be begotten either by a polygenic cause or by gene defects occurring in other related genes and regions not targeted in this study., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Moradi, Maleki, Ghaemmaghami, Khajali, Noohi, Moghadam, Kalyinia, Mowla, Seidah and Malakootian.)

Superior cavopulmonary anastomosis is a type of palliative cardiac surgeries that usually done in children with cyanotic and complex congenital heart disease who have single ventricle profile. BDG shunt is staged palliation procedure for single ventricle patients who are candidates for total cavopulmonary connection (TCPC). Sometimes the surgeon misses ligating or intentionally leaves the azygos vein as a fenestration or emergency exit. This allows an abnormal flow from the superior vena cava (SVC into azygos vein). These patients can present progressive desaturation, chest tightness, progressive dyspnea, edema and shortness of breath. Therapeutic options include observation, surgical ligation and trans catheter closure. Because of high risks and extra traumas of surgery and greater chance for difficulties and the feasibility of trans catheter therapy, it is done in some centers as a method of choice., (© 2021 The Author(s).)

Fontan operation is a reliable palliative surgery for patients with single ventricle physiology. Still, the development of complication is common; one of these complications that need to interventional approach is veno-venous collaterals between systemic and pulmonary veins. A 16-yearoldgirl with a history of modified Fontan operation at 9 years ago was referred with progressive cyanosis and dyspnea on exertion. In contrast trans-thoracic echocardiography (TTE), no fenestration was seen in Fontan circulation. Cardiac magnetic resonance revealed partial anomalous pulmonary vein connection (PAPVC) from left upper pulmonary vein to vertical vein and then into the in nominate vein and SVC with the reverse flow from superior vena cava (SVC) to left upper pulmonary vein(LUPV). This anomalous vein became severe engorged and tortuous. Possibly, LUPV and the verticalvein was dilated gradually as a result of increased pressure in the Fontan circuit. Finally, she underwent successful coil embolization in the midpart of the vertical vein. The oxygen saturation increased from80% to 93%., (© 2021 The Author(s).)