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.

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.

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.

Pubertal status of children with congenital heart disease.

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.

Self-Expandable Stent for Repairing Coarctation of the Left-Circumferential Aortic Arch with Right-sided Descending Aorta and Aberrant Right Subclavian Artery with Kommerell's Aneurysm.

Endovascular treatment offers a great advantage in the management of main arteries stenoses. However, simultaneous presence of a group of anomalies may complicate the situation. Here we present a case of 21-year-old man with aortic coarctation. Radiographic imaging and angiography demonstrated aortic coarctation of the left-circumferential aortic arch, right-sided descending aorta, and Kommerell's diverticulum at the origin of right subclavian artery. These anomalies have rarely been reported to concurrently exist in the same case and the treatment is challenging. Percutaneous treatment for repair of aortic coarctation was successfully performed with deployment of self-expanding nitinol stents. Follow-up demonstrated the correction of blood pressure and improvement of the symptoms. It appears that deployment of self-expandable nitinol stents present a viable option for the management of coarcted aorta in patients having all or some of these anomalies together.

Fibrinolytic therapy for mechanical pulmonary valve thrombosis.

Treatment of prosthetic heart valve thrombosis using intravenous thrombolytics, although an acceptable alternative to surgery, is not complication free, and the literature has a dearth of data on the subject. This study analyzed the results of fibrinolytic treatment (FT) among a single-center group of patients with mechanical pulmonary valve thrombosis. Between 2000 and 2013, 23 consecutive patients with 25 episodes of pulmonary valve thrombosis received FT. The diagnosis of mechanical pulmonary valve thrombosis was established by fluoroscopy and echocardiography. Streptokinase (SK) was used in 24 cases and alteplase in 1 case. The FT was continued a second day for 14 patients (58.3%), a third day for 1 patient, and a fourth day for 1 patient. Echocardiography and fluoroscopy were performed every day until improvement of malfunction was achieved. Of the 23 patients, 19 had complete resolution of hemodynamic abnormalities after FT, 1 had partial resolution, and 2 showed no change. No patient had major complications. Five minor complications were detected, namely, fever, nausea, thrombophlebitis, epistaxi, and pain. Seven patients (30%) experienced recurrence of thrombosis, whereas four patients had surgery (biological pulmonary valve replacement) without re-thrombolytic therapy, one patient was treated with Alteplase, one patient received SK, and one patient received intense anticoagulation using heparin and warfarin. Overall, FT had a success rate of 84%. The results indicate that regardless of the time to pulmonary valve replacement and echocardiographic and fluoroscopic findings, FT was effective in most cases of mechanical pulmonary valve thrombosis. The efficacy increased with second-day thrombolytic therapy. Major complications were not common after lytic therapy for mechanical pulmonary valve thrombosis.

Anomalous origin of the right pulmonary artery from the ascending aorta accompanied by absent pulmonary valve syndrome and right-sided aortic arch: a rare case in adult congenital heart disease.

We present a rare congenital heart disease in a 20-year-old man with anomalous origin of the right pulmonary artery from the ascending aorta, accompanied by absent pulmonary valve syndrome, and a right-sided aortic arch suspected initially in transthoracic echocardiography and subsequently confirmed by cardiac catheterisation and computed tomography angiography.

L-carnitine supplementation decreases lipid peroxidation and improves cardiopulmonary function in broiler chickens reared at high altitude.

An experiment was carried out to examine the effects of L-carnitine supplementation on growth performance and cardiopulmonary function of broiler chickens reared at high altitude (2100 m above sea level). A total of 96 day-old male chicks (Cobb 500) were randomly assigned into two dietary treatments containing 0 (control group) and 200 mg/kg L-carnitine. The experimental diets were fed for a period of 42 days consisting of the starting (days 1 to 21) and growing periods (days 21 to 42). Nutrient requirements of chickens met the NRC (1994) recommendations. The results showed that dietary L-carnitine had no significant influence on body weight gain, feed intake and feed conversion ratio. L-carnitine reduced plasma concentration of malondialdehyde, packed cell volume (PCV) and abdominal fat deposition compared to the control (P < 0.05). A significant (P < 0.05) decrease was observed in the right to total ventricular weight ratio (RV:TV) in birds receiving L-carnitine when compared to the control. Supplementation of L-carnitine increased plasma nitric oxide and immune responsiveness, which manifested in an increased toe-web thickness index measured at 24 h following the injection of phytohaemagglutinin P. In conclusion, supplementation of L-carnitine had beneficial effects on preventing lipid peroxidation and pulmonary hypertension in broiler chickens raised at high altitude.

Exploring the role of non-coding RNAs in atrial septal defect pathogenesis: A systematic review.

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.

Uncommon manifestation of Ebstein anomaly: A case report of apical displacement involving all tricuspid valve leaflets.

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.

Clinical outcomes of transcatheter closure of congenital coronary artery fistula in 28 cases.

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.

Ultraslow low-dose thrombolytic therapy in patients with acute mechanical prosthetic heart valve thrombosis - A prospective cohort study.

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.

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.

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.

A Case of Unexplained Warfarin Resistance: A Case Report and Literature Review.

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.

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.

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.