Aortic dilation in adults with repaired tetralogy of Fallot: a single-centre study.

BACKGROUND: Tetralogy of Fallot is the most prevalent cyanotic CHD. With the advent of advanced surgical methods, the majority of tetralogy of Fallot patients reach adulthood. However, many need re-intervention for the residual anomalies including residual right ventricular outflow obstruction, pulmonary regurgitation, residual ventricular septal defects, and progressive aortic dilatation. Aortic dilation could lead to aortic regurgitation or dissection requiring surgical correction. In the current study, we aimed to determine the prevalence and outcomes of aortic root dilatation in adults with repaired tetralogy of Fallot in our tertiary care centre. METHODS: In this retrospective study, 730 consecutive patients with history of repaired tetralogy of Fallot were included. Aortic diameter at the level of annulus, the sinus of Valsalva, sinotubular junction, and the ascending aorta as measured by echocardiography were evaluated. Prevalence of outcomes necessitating re-intervention including aortic regurgitation and dissection were recorded. RESULTS: The mean size of annulus, sinus of Valsalva, sinotubular-junction, and ascending aorta in the latest available echocardiography of patients were 2.4+/-0.4 cm, 3.3+/-0.5 cm, 2.9+/-0.5cm, and 3.2+/-0.5cm, respectively. Prevalence of dilatation of sinus of Valsalva, dilation of Ascending aorta, sinotubular-junction, and aortic annulus was 28.7%, 21%, 8.3%, and 1 %, respectively. Five patients had severe aortic regurgitation (0.6%) and underwent surgical repair. One of these patients presented with acute aortic dissection. CONCLUSION: Aortic dilation is common in tetralogy of Fallot but prevalence of redo surgery for aortic dilation, regurgitation, and adverse events including acute dissection is low.

Incidental detection of ruptured sinus of Valsalva aneurysm and coronary abnormality in a patient with penetrating chest trauma.

Aortic sinus of Valsalva aneurysm is a rare congenital or acquired anomaly with a potential for rupture into adjacent cardiac chambers. Ruptured aneurysm of the sinus of Valsalva leads to volume overload of the receiving chamber and should be repaired as soon as the diagnosis is made. Here, we present a young patient with history of chest trauma leading to discovery of an asymptomatic ruptured sinus of Valsalva aneurysm.

Cardiac remodeling after atrial septal defects device closure.

BACKGROUND: Trans-catheter device closure of secundum type atrial septal defects (ASDs) has now become the treatment of choice. Device closure affects the hemodynamics and function of the heart. In the current study, we investigated the remodeling of the heart as assessed by advanced echocardiographic parameters following successful device closure. METHODS: A prospective cohort study was conducted and 45 patients who were eligible for ASD device closure were evaluated for volumetric, functional, Doppler, strain, and strain rate data of left and right atrium and ventricle pre-procedural and 48 h post-procedure RESULTS: In a prospective cohort study 45 patients who were eligible for ASD device closure; atrial and ventricular volumetric, functional, Doppler, strain, and strain rate data were recorded and compared pre-procedurally and 48 h post-procedure.We found significant changes in the left ventricular (LV) end diastolic volume index (p-value = .03), right ventricular (RV) diameter (p-value = <.001), left atrial (LA) volume index (p-value = .05), right atrial (RA) volume index (p-value = .001), and right and left sided E/e' ratio with a p-value of .001 and .004, respectively. Our findings showed a significant reduction in the strains of the right and left atria and the right ventricular free wall after ASD device closure. LV global longitudinal strain decreased after the procedure but did not reach statistical significance. CONCLUSION: ASD patients have impaired global strains compared to normal defined ranges. LA, RA, and RV strains show significant reduction after device closure. Decline in LA function following closure was greater in those with larger ASDs. In adult patients undergoing the procedure, abnormal LA function is a clinically relevant issue demanding pre and post- procedural precautions and treatment.

Case Report of a 33-year-old with Ebstein malformation of tricuspid valve and hypertrophic cardiomyopathy.

Ebstein malformation of tricuspid valve is a congenital disease of tricuspid valve with associated right ventricular cardiomyopathy. Hypertrophic cardiomyopathy is a form of inherited left ventricular cardiomyopathy caused by sarcomeric protein gene mutations with inherent risks of sudden cardiac death. Here we report a rare case with co-occurrence of Ebstein malformation of tricuspid valve and hypertrophic cardiomyopathy in a young patient.

Transhepatic device closure of large atrial septal defect.

Transcatheter closure of secundum-type atrial septal defects has become the treatment of choice in the majority of cases. Femoral venous access is the standard rout for device implantation. Anatomic abnormalities of venous system including interrupted inferior caval vein with azygous continuation can make the percutaneous procedure more complicated. In such instances, alternative methods of transjugular or transhepatic approach or surgical repair should be considered. We present the case of a 50-year-old male with secundum-type atrial septal defect and a rare form of segmental interruption of inferior caval vein and describe successful atrial septal defect closure through transfemoral approach.

Left ventricular intra-myocardial dissection after myocardial infarction.

Left ventricular (LV) intra-myocardial dissection or dissecting hematoma is a rare complication of myocardial infarction that could occur in the acute phase, during remodeling process and even after coronary revascularization. LV intra-myocardial dissection has a high mortality, and the best management strategy remains controversial. Here, we present a case of dissection of left ventricle late after anterior myocardial infarction diagnosed by multimodality imaging.

Concomitant cardiac and hepatic hemangiomas.

Primary tumors of the heart are rare with a reported incidence of about 0.002% to 0.3% at autopsy. A cardiac hemangioma is a form of benign primary cardiac tumor that often presents with atypical clinical symptoms. Hemangiomas are generally isolated lesions. Here, we report a patient with previous hepatic hemangioma who later was found to have a large coexistent cardiac hemangioma presenting with cardiac compressive symptoms.

The role of echocardiography in anomalous origin of coronary artery from pulmonary artery (ALCAPA): Simple tool for a complex diagnosis.

Anomalous origin of coronary artery from pulmonary artery (ALCAPA) is a rare coronary anomaly usually presenting in the first year of life. Adult presentation suggests a wide range of differential diagnosis such as myocarditis, dilated cardiomyopathy, and coronary artery disease. We have presented here the major distinctive echocardiographic features of ALCAPA through 4 cases. Since echocardiography is often used as the first imaging modality in these clinical scenarios, recognizing the characteristics of ALCAPA in a routine echocardiographic examination can transform a readily available, low-cost tool into a valuable discriminative modality.

Peri-mitral ventriculoatrial fistula after blunt thoracic trauma.

Blunt trauma to the chest can damage any thoracic organ. Heart valve damage and particularly mitral apparatus occurs relatively rare, and the patients are expected to become acutely symptomatic. However, in patients with multiple traumas, other dominant severe damages tend to draw away the attention of the physicians resulting in neglect of less salient heart injuries. Here, we present a patient with history of blunt chest trauma and incidental finding of posttraumatic severe mitral regurgitation.

A novel PKP2 mutation and intrafamilial phenotypic variability in ARVC/D.

Background: Arrhythmogenic ventricular cardiomyopathy (AVC) is an inherited cardiac disorder affecting 1 in 1000 individuals worldwide. The mean diagnosed age of disease is 31 years. In this article, an Iranian family reported that they were affected by ARVC due to a novel PKP2 mutation. Methods: Clinical evaluations, 12-lead ECG, CMR, and signal-averaged ECG were performed. After DNA extraction, genetic testing was done, and PCR-sequencing was applied to find causal mutations. Segregation analysis was also performed for the family. Results: ARVC criteria were documented in the patients. Genetic testing revealed a novel chain termination mutation (p.Tyr168Ter) in PKP2 gene; this mutation was transmitted from the mother to her 23-year-old son, but only the son was affected with ARVC. Conclusion: Modifier genes were indicated using interactome analysis of Plakophilin 2 protein (PKP2); they might have led to phenotypic variability through cellular mechanisms, such as nonsense-mediated mRNA decay. At least, 9 proteins were identified that might have affected Plakophilin 2 protein function, and consequently, rationalizing this intrafamilial phenotypic variability. This study highlighted the role of modifier genes involved in ARVC as well as the major role of PKP2 mutation in developing the disease in our population.

Autosomal Recessive Nonsyndromic Arrhythmogenic Right Ventricular Cardiomyopathy without Cutaneous Involvements: A Novel Mutation.

The arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic disease frequently associated with desmosomal mutations, mainly attributed to dominant mutations in the Plakophilin-2 (PKP2) gene. Naxos and Carvajal are the syndromic forms of ARVD/C due to recessive mutations. Herein, we report an autosomal recessive form of nonsyndromic ARVD/C caused by a mutation in the PKP2 gene. After examination and implementation of diagnostic modalities, the definite diagnosis of ARVD/C was confirmed by detection of ventricular tachycardia with a left bundle branch configuration and a superior axis, T-wave inversion in right precordial leads (i.e., V1-V3) in a 12-lead electrocardiogram, and a right ventricle outflow tract dilatation. Neither cutaneous involvement nor other abnormalities were observed. Genetic testing was performed during which an intronic mutation of c.2577+1G>T in the PKP2 gene was observed homozygously. The c.2577+1G>T disrupts PKP2 mRNA splicing and causes a nonsyndromic form of ARVD/C.

How canola and sunflower oils affect lipid profile and anthropometric parameters of participants with dyslipidemia.

Background: Restricted intakes of saturated and trans-fatty acids and replacement with poly or monounsaturated fatty acids are emphasized in healthy diets. This study evaluates the effects of a six-month consumption of canola oil compared to sunflower oil on lipid profile and anthropometric parameters of people affected by dyslipidemia. Methods: This randomized controlled trial was conducted on 96 patients with dyslipidemia, who were randomly assigned into canola oil or the sunflower oil groups. The participants were instructed to record the contents of their daily meals, beverages, fruits, and snacks a day before treatment, at the second clinic visit, in the third month, and at the end of study (i.e., six months). Lipid profile and anthropometric parameters were compared between the two groups. Student t-test or Mann Whitney U test was used for statistical comparisons of variables between groups. Multivariate analysis was performed to adjust the confounding factor effects. Results: Of the enrolled participants, 44 (45.8%) were on sunflower oil diet and 52 (54.2%) on canola oil diet. We observed no change in anthropometric parameters and thus no significant difference between the two groups (p>0.05). Significant reductions in LDL-C (p<0.001), total cholesterol (p<0.001) and triglyceride levels (p<0.001), and significant elevation in HDL-C (p=0.008) were observed in canola oil group, as well as those who used sunflower oil. Conclusion: Dietary fats in the form of canola oil or sunflower oil effectively lower the serum cholesterol, LDL-C and triglyceride concentrations. They also result in an increase in serum concentration of HDL-C. These oils, however, did not modify general anthropometric parameters.

A rare association of the absence of left pulmonary artery with ventricular septal defect, pulmonary hypertension, and more interestingly, patent ductus arteriosus in an adult patient: Case report and literature review.

Key Clinical Message: In this study, we introduced one of the rarest concomitants of the absence of left pulmonary artery (LPA), which was seen in our patient along with patent ductus arteriosus (PDA) and ventricular septal defect (VSD). Abstract: Unilateral absence of pulmonary artery (UAPA) is a congenital heart disease in association with other abnormalities such as tetralogy of Fallot and septal defects or isolated in 30% of cases and occurs in the right lung in two thirds of cases. Our case is a 33-year-old man who was hospitalized with symptoms of cough, shortness of breath, and hemoptysis. The echocardiography revealed a large ventricular septal defect, absent left pulmonary artery, and severe pulmonary hypertension (PH) along with patent ductus arteriosus. These findings were confirmed by CT angiography. This association has rarely been found in past studies. Due to PH and pulmonary infection, the patient was treated with intravenous prostaglandin and antibiotics. However, in cases of timely diagnosis and treatment of UAPA, fatal complications such as pulmonary hypertension, morbidity, and mortality are reduced. This case emphasizes the importance of awareness of this abnormality and its associated anomalies to enable early diagnosis and treatment.

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.

Right ventricular echocardiographic remodeling after pulmonary valve replacement in repaired Tetralogy of Fallot.

BACKGROUND: Many patients with repaired tetralogy of Fallot require reoperation in the medium to long-term for residual pulmonary valve regurgitation. Best timing for pulmonary valve replacement remains controversial. A balance needs to be found between protecting the patients from permanent right ventricular damage and insertion of a prosthetic valve with its inherent issues. In the current study we sought to investigate the right and left ventricular functional recovery following valve replacement in our tertiary care institution. RESULTS: In a retrospective cross-sectional study patients with history of total correction of tetralogy of Fallot who had undergone pulmonary valve replacement due to severe pulmonary regurgitation between 2003-2018 were evaluated for post intervention right and left ventricular functional recovery. Clinical and full echocardiographic data before and after the surgery were recorded and compared. There was statistically significant improvement in RV size and function post pulmonary valve replacement. There was no statistically significant improvement in left ventricular systolic function. Twenty percent of patient had persisting severe right ventricular enlargement at least twelve months post-surgery. No patient had fully normalized right ventricular size and function in follow-up. CONCLUSIONS: Pulmonary valve replacement leads to improvement in right ventricular size and function in patients with repaired tetralogy of Fallot. However normalization of functional parameters did not occur and the majority of the patients have residual right and left ventricular dysfunction following redo valve replacement surgery.

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.

Multiple congenital visceral abnormalities as a rare cause of pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare, progressive disorder. PAH is caused by a wide spectrum of pathologies but the cause remains undetermined on many occasions and patients are classified in the idiopathic group. CASE PRESENTATION: Here we report a young woman with rare congenital visceral abnormalities presenting with severe pulmonary hypertension. CONCLUSIONS: Pulmonary hypertension is a complex disorder. Search for uncommon conditions that lead to pulmonary hypertension is necessary to determine the best management options.

Safety and feasibility of simultaneous left and right heart catheterization via single-arm arterial and venous access.

BACKGROUND: Catheterization via the radial artery has become the method of choice for evaluation of the coronary arteries; however in patients requiring simultaneous coronary and right heart catheterization upper extremity access is not commonly used. AIMS: In the present study we aimed to assess whether simultaneous left and right heart catheterization via the radial artery and antecubital vein might increase the vascular access site complications. METHODS: In this prospective cohort study, 49 patients with congenital and valvular heart disease requiring both left and right heart catheterization were enrolled, and rates of vascular access complications, including radial artery obstruction (RAO), were compared to 49 subjects in the control group who underwent catheterization only via radial artery access. RESULTS: No major vascular complications occurred in the study population. Post-procedural radial artery obstruction was detected in 14 patients (28.5%) in the venous-radial artery group and 7 (14.2%) in the control group (P = 0.09). Age, sex, body mass index, wrist circumference, hemoglobin concentrations, and history of smoking, diabetes mellitus, hypertension, and anticoagulant use did not affect the rate of radial pulse perseverance. However, shorter times of radial compression device removal were significantly related to lower rates of radial artery obstruction in both groups. CONCLUSION: Right and left heart catheterization via an upper-extremity route in patients with congenital and valvular heart disease is safe and practical with greater patient comfort and no serious vascular complications. A longer duration of radial compression is an important predictor of RAO, regardless of using additional upper-extremity venous access.

A giant aneurysm of sinus of Valsalva presenting with frequent syncopal attacks.

Sinus of Valsalva aneurysm is a rare congenital or acquired aortic root anomaly. The aneurysm has the potential to compress the adjacent structures or rupture to other cardiac chambers with fatal consequences. Patients might be asymptomatic or present with nonspecific cardiac symptoms. Once the diagnosis is made, prompt surgical repair is generally required. Here, we present a young woman with frequent syncope who was found to have a giant sinus of Valsalva aneurysm as the underlying etiology.