Midterm prognosis following total correction of tetralogy of fallot in adult patients.

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.

Short-term outcomes following total correction of tetralogy of fallot in adult patients.

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.

Electrocardiographic manifestations of pulmonary stenosis versus pulmonary hypertension.

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.

Percutaneous closure of an atrial septal defect in adult patients with congenitally corrected transposition of the great arteries.

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.

Percutaneous Transcatheter Closure of Post-Myocardial Infarction Ventricular Septal Rupture.

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.

The Treatment Dilemma of Arteriopathy in Takayasu Arteritis- A State-of-the-Art Approach.

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.

Atrial septal defect device closure and concurrent atrio-fascicular Mahaim ablation in adult patient with Ebstein's anomaly.

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.

Prevalence of Structural Heart Diseases Detected by Handheld Echocardiographic Device in School-Age Children in Iran: The SHED LIGHT Study.

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.

Safety of Aortic Coarctation Treatment in Patients with Turner Syndrome: A Single-Country Case Series and Literature Review.

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.

Displacement of all three leaflets of tricuspid valve: A rare variant of Ebstein anomaly.

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.

Balloon-Expandable Cheatham-Platinum Stents Versus Self-Expandable Nitinol Stents in Coarctation of Aorta: A Randomized Controlled Trial.

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.

Iatrogenic partial diversion of inferior vena cava to left atrium after surgical closure of atrial septal defect.

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.

Quality of life in adult patients with congenital heart disease: Results of a double-center study.

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.

Fast vs. ultraslow thrombolytic infusion regimens in patients with obstructive mechanical prosthetic valve thrombosis: a pilot randomized clinical trial.

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 (>75% fall in the obstructive gradient by transthoracic echocardiography, <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 Transcatheter Tricuspid Valve-in-Valve Technique in Degenerated Bioprostheses Without Fluoroscopic Radiopaque Landmarks.

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.

Current Endovascular Approach in Adult Patients with Pulmonary Vein Stenosis: A State-of-the-Art Approach.

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.

Trans-catheter closure of large PDA in adult patients with Amplatzer device: case series.

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.