Evaluation of the total hydrodynamic energy loss using 4D flow MRI in a case with Fontan failure.

Fontan Failure (FF) is a common problem for single-ventricle patients as they reach adulthood. Although several mechanisms may cause FF, an optimized blood flow stream through the surgical conduits is essential to avoid excessive energy loss (EL). Recent clinical studies showed EL is related to the quality of life, exercise capacity, and hepatic function since the single-ventricle feeds pulmonary and systemic circulation serially. 4D flow MRI effectively estimates EL in Fontan circulation and allows clinicians to compare the effectiveness of the treatment strategy concerning pre-intervention. Here, we present 26-year-old women with FF who had normal cardiac catheterization findings and were treated according to high EL definitions that are measured through 4D flow MRI.

High AST/ALT Ratio Is Associated with Cardiac Involvement in Acute COVID-19 Patients.

Background and Objectives: We aimed to assess the effect of AST/ALT ratio on echocardiographic and cardiac magnetic resonance imaging (CMRI) parameters after COVID-19 patients recover. Materials and Methods: 87 patients with COVID-19 were included in the study. The patients were hospitalized with COVID-19 pneumonia, but the patients did not need intensive care unit follow-up or non-invasive mechanical ventilation support. After a discharge and two weeks following the positive swab test result, patients were considered eligible if they had any symptoms. Transthoracic echocardiography (TTE) was performed within 24 h prior to CMRI. The median value of AST/ALT ratio was found, and the study population was divided into two subgroups based on the median AST/ALT ratio value. The clinical features, blood test, TTE and CMRI results were compared between subgroups. Results: C-reactive protein, D-dimer and fibrinogen were found to be significantly higher in patients with high AST/ALT ratio. LVEF, TAPSE, S', and FAC were significantly lower in patients with high AST/ALT ratio. LV-GLS were significantly lower in patients with high AST/ALT ratio. In CMRI, native T1 mapping signal, native T2 mapping signal and extracellular volume raised significantly in patients with high AST/ALT ratio. Right ventricle stroke volume and right ventricle ejection fraction were significantly lower in patients with high AST/ALT ratio, but right ventricle end systolic volume was significantly higher in patients with high AST/ALT ratio. Conclusion: High AST/ALT ratio is related to impaired right ventricular function parameters with CMRI and echocardiography after recovery from acute COVID-19. Assessment of AST/ALT ratio at hospital admission may be used to assess the risk of cardiac involvement in COVID-19 disease, and these patients may require closer follow-up during and after the course of COVID-19.

Surgical repair of the anomalous aortic origin of the coronary arteries: a single-center experience.

OBJECTIVE: Anomalous aortic origin of the coronary artery is a rare congenital cardiac anomaly. The aim of this study was to present our experience with patients who underwent surgery for the anomalous aortic origin of the coronary artery. METHODS: This was a retrospective review of our experience with patients who had anomalous aortic origin of the coronary artery from 2019 to 2022. RESULTS: Seven patients were managed for anomalous aortic origin of the coronary artery, including five males and two females. The median age of the patients were 16 years (IQR, 14.5-26 years). Five patients had anomalous aortic origin of the right coronary artery and two patients had anomalous aortic origin of the left coronary artery. Five patients were treated surgically, one patient refused surgical treatment despite myocardial ischaemia symptoms, and the other one was not operated because she had no symptoms. Two patients underwent pulmonary root anterior translocation and left main coronary artery unroofing procedure, one patient underwent right coronary artery unroofing procedure, one patient underwent pulmonary artery lateral translocation procedure, and the last patient underwent right coronary artery osteal translocation procedure. The post-operative mortality or myocardial infarction was not observed in any patient. Patients were followed for a median of 10 months (IQR, 6.75-20.5 months) after repair. CONCLUSION: The data suggest that surgical repair of anomalous aortic origin of the coronary artery can be performed confidently and can be very effective for relieving myocardial ischaemia symptoms. Different surgical techniques can be used in anomalous aortic origin of the coronary artery according to the course and origin of the coronary arteries. To the our knowledge, pulmonary root anterior translocation and coronary artery unroofing procedure were performed for the first time in the literature.

Surgical and transcatheter pulmonary valve replacement in patients with repaired tetralogy of Fallot: cardiac magnetic resonance imaging characteristics and morphology of right ventricular outflow tract.

BACKGROUND: Pulmonary valve replacement is recommended in patients with repaired tetralogy of Fallot based on cardiac magnetic resonance imaging (MRI) criteria. This procedure is performed by surgical or transcatheter approaches. OBJECTIVE: We aimed to investigate the differences in preprocedural MRI characteristics (volume, function, strain) and morphology of the right ventricular outflow tract and branch pulmonary arteries in patients for whom surgical or transcatheter pulmonary valve replacement was planned. MATERIALS AND METHODS: Cardiac MRI of 166 patients with tetralogy of Fallot were analyzed. Of these, 36 patients for whom pulmonary valve replacement was planned were included. Magnetic resonance imaging characteristics, right ventricular outflow tract morphology, branch pulmonary artery flow distribution and diameter were compared between surgical and transcatheter groups. Spearman correlation and Kruskal-Wallis tests were performed. RESULTS: Circumferential and radial MRI strain for the right ventricle were lower in the surgical group (P=0.045 and P=0.046, respectively). The diameter of the left pulmonary artery was significantly lower (P=0.021) and branch pulmonary artery flow and diameter ratio were higher (P=0.044 and P = 0.002, respectively) in the transcatheter group. There was a significant correlation between right ventricular outflow tract morphology and right ventricular end-diastolic volume index and global circumferential and radial MRI strain (P=0.046, P=0.046 and P= 0.049, respectively). CONCLUSION: Preprocedural MRI strain, right-to-left pulmonary artery flow, diameter ratio and morphological features of the right ventricular outflow tract were significantly different between the two groups. A transcatheter approach may be recommended for patients with branch pulmonary artery stenosis, since both pulmonary valve replacement and branch pulmonary artery stenting can be performed in the same session.

Experience of 500 cardiovascular magnetic resonance imaging and systematic analysis of cases.

OBJECTIVE: Cardiovascular magnetic resonance imaging (MRI) is a widely accepted reference imaging technique in routine cardiology clinics in many centers due to its advantages in providing preferable functional, morphologic information. However, there is little information about national experience in clinical application and findings of cardiovascular MRI. The objective of this study was to demonstrate the clinical and demographic characteristics of patients admitted to our cardiac imaging department. METHODS: A total of 500 cardiovascular MRI examinations performed between 2016 and 2019 were enrolled in this retrospective study. Clinical indications, demographic, and cardiovascular MRI findings of the patients were retrospectively evaluated. RESULTS: Five hundred patients (M/F=301/199) were included in this retrospective, single center study. The majority of the examinations were performed for the assessment of congenital heart disease (n=254, 50.8%). The other indications were for myocardial disease (n=160, 32%), cardiac mass (n=44, 8.8%), valvular heart disease (n=20, 4%), magnetic resonance angiography (n=12, 2.4% for aorta and pulmonary artery [n=9, 1.8%] and for coronary arteries [n=3, 0.6%]), and vasculitis (n=7, 1.7%), pericardial disease (n=3, 0.6%). Minor complication was seen during the contrast agent injection in three patients (0.06%). CONCLUSION: Cardiovascular MRI is a reliable and accurate imaging tool in identifying the various cardiac pathology with widely accepted use in the clinical area. Our single-center experience of 500 cases demonstrates the varieties of clinical indications in daily practice that may contribute to the national data pool.

Cardiac magnetic resonance T2* mapping in patients with COVID-19 pneumonia is associated with serum ferritin level?

The coronavirus disease of 2019 (COVID-19)-related myocardial injury is an increasingly recognized complication and cardiac magnetic resonance imaging (MRI) has become the most commonly used non-invasive imaging technique for myocardial involvement. This study aims to assess myocardial structure by T2*-mapping which is a non-invasive gold-standard imaging tool for the assessment of cardiac iron deposition in patients with COVID-19 pneumonia without significant cardiac symptoms. Twenty-five patients with COVID-19 pneumonia and 20 healthy subjects were prospectively enrolled.Cardiac volume and function parameters, myocardial native-T1, and T2*-mapping were measured. The association of serum ferritin level and myocardial mapping was analyzed. There was no difference in terms of cardiac volume and function parameters. The T2*-mapping values were lower in patients with COVID-19 compared to controls (35.37 [IQR 31.67-41.20] ms vs. 43.98 [IQR 41.97-46.88] ms; p < 0.0001), while no significant difference was found in terms of native-T1 mapping value(p = 0.701). There was a positive correlation with T2*mapping and native-T1 mapping values (r = 0.522, p = 0.007) and negative correlation with serum ferritin values (r = - 0.653, p = 0.000), while no correlation between cardiac native-T1 mapping and serum ferritin level. Negative correlation between serum ferritin level and T2*-mapping values in COVID-19 patients may provide a non-contrast-enhanced alternative to assess tissue structural changes in patients with COVID-19. T2*-mapping may provide a non-contrast-enhanced alternative to assess tissue alterations in patients with COVID-19. Adding T2*-mapping cardiac MRI in patients with myocardial pathologies would improve the revealing of underlying mechanisms. Further in vivo and ex vivo animal or human studies designed with larger patient cohorts should be planned.

Association of Epicardial Adipose Tissue Thickness with Cardiovascular Risk in Acromegaly.

Objective: Acromegaly is a rare disease associated with increased mortality. Reports on coronary artery disease in acromegaly are controversial. This study aimed to investigate the possible association of epicardial adipose tissue thickness with cardiovascular risk in patients with acromegaly. Methods: The study included 38 patients followed up with the diagnosis of acromegaly and 29 healthy controls. Patients with acromegaly were divided into controlled and uncontrolled acromegaly groups based on insulin-like growth factor-1 levels. Epicardial adipose tissue thickness measurements were obtained from chest computed tomography, and laboratory data were extracted from patient files. Results: Twenty-nine patients (76.3%) had controlled acromegaly. Eleven patients with acromegaly had diabetes mellitus (28.9%), 18 (47.4%) had hypertension, and 27 (71%) had a concomitant chronic disease. Epicardial adipose tissue thickness was significantly increased in the acromegaly group (p<0.001). No significant difference was observed between the controlled and uncontrolled acromegaly groups in terms of the epicardial adipose tissue thickness. Age was the only parameter that was significantly correlated with the epicardial adipose tissue thickness. When the Framingham risk score was calculated, the 10-year cardiovascular risk of patients with acromegaly was 5.63%. Conclusions: The epicardial adipose tissue thickness is increased in acromegaly. However, this increase may not have clinical relevance in terms of cardiovascular risk.

Non-invasive Assessment of Subclinical Renal Parenchymal Changes in Chronic Hepatitis B Virus By T1 Mapping Magnetic Resonance Imaging.

Background: Renal parenchymal changes are seen in chronic hepatitis B virus (HBV) infection, and its disease diagnosis should be confirmed by renal biopsy, which is an invasive technique. Apparent-T1 mapping magnetic resonance imaging (MRI) is an established imaging technique that assesses subclinical tissue injury without using a contrast agent. Aims: To investigate the early stage subclinical renal changes without apparent renal dysfunction in patients with chronic HBV infection by renal apparent-T1 mapping MRI. Study Design: A cross-sectional study. Methods: This study included 45 participants with normal kidney function, wherein 25 have biopsy-proven chronic HBV hepatitis and 20 are healthy individuals. Liver and kidney biochemical tests were performed within 1 month before the MRI scan, and the estimated glomerular filtration rate was calculated by diet modification in renal disease formula. Breath-hold, electrocardiogram-gated Modified Look-Locker Imaging sequence was acquired in the coronal plane without contrast agent administration. Apparent-T1 mapping value was measured by manually drawing a region of interest in six points for both kidneys by two observers. Apparent-T1 mapping values were compared between the two groups. Results: The mean apparent-T1 mapping values of the kidneys were significantly higher in patients with chronic HBV infection compared to the control group (1445 ± 129 ms vs. 1306 ± 115 ms, P = 0.003). Inter-class correlation coefficient measurement analysis showed excellent agreement. Conclusion: Renal apparent-T1 mapping MRI may help show the early stage of renal parenchymal disease without apparent renal dysfunction in chronic HBV infection.

A novel Fontan Y-graft for interrupted inferior vena cava and azygos continuation.

OBJECTIVES: To evaluate the hemodynamicdynamic advantage of a new Fontan surgical template that is intended for complex single-ventricle patients with interrupted inferior vena cava-azygos and hemi-azygos continuation. The new technique has emerged from a comprehensive pre-surgical simulation campaign conducted to facilitate a balanced hepatic flow and somatic Fontan pathway growth after Kawashima procedure. METHODS: For 9 patients, aged 2 to18 years, majority having poor preoperative oxygen saturation, a pre-surgical computational fluid dynamics customization is conducted. Both the traditional Fontan pathways and the proposed novel Y-graft templates are considered. Numerical model was validated against in vivo phase-contrast magnetic resonance imaging data and in vitro experiments. RESULTS: The proposed template is selected and executed for 6 out of the 9 patients based on its predicted superior hemodynamic performance. Pre-surgical simulations performed for this cohort indicated that flow from the hepatic veins (HEP) do not reach to the desired lung. The novel Y-graft template, customized via a right- or left-sided displacement of the total cavopulmonary connection anastomosis location resulted a drastic increase in HEP flow to the desired lung. Orientation of HEP to azygos direct shunt is found to be important as it can alter the flow pattern from 38% in the caudally located direct shunt to 3% in the cranial configuration with significantly reversed flow. The postoperative measurements prove that oxygen saturation increased significantly (P-value = 0.00009) to normal levels in 1 year follow-up. CONCLUSIONS: The new Y-graft template, if customized for the individual patient, is a viable alternative to the traditional surgical pathways. This template addresses the competing hemodynamic design factors of low physiological venous pressure, high postoperative oxygen saturation, low energy loss and balanced hepatic growth factor distribution possibly assuring adequate lung development. DATE AND NUMBER OF IRB APPROVAL: 25 October 2019, 280011928-604.01.01.

Liver parenchymal changes and association with cardiac magnetic resonance imaging findings in repaired tetralogy of Fallot: an intravoxel incoherent motion magnetic resonance imaging study.

BACKGROUND: Liver disease can develop in repaired tetralogy of Fallot (TOF) from hepatic congestion caused by volume and pressure overload of the right ventricle. Noninvasive assessment of the liver is important for diagnosing and managing children with TOF. OBJECTIVE: To evaluate subclinical hepatic changes without liver function test abnormality in adolescents with repaired TOF using intravoxel incoherent motion (IVIM) MRI and cardiac MRI findings. MATERIALS AND METHODS: We included 106 young adults (75 with repaired TOF and 31 healthy individuals) in the study. Liver IVIM MRI examinations were performed with 10 b values (0, 10, 20, 30, 50, 80, 100, 200, 400, 800 s/mm2). Two observers measured IVIM MRI parameters D true, D* and f, as well as apparent diffusion coefficient (ADC) values in liver segments 5-8. RESULTS: D* and f values were significantly lower in adolescents with TOF (P = 0.003 vs. P = 0.05, respectively). ADC values were higher in adolescents with TOF (P = 0.005). However, we found no significant difference between adolescents with and without TOF in terms of Dtrue (P = 0.53). There was a significant correlation between f value and right ventricular ejection fraction. The intraclass correlation coefficient (ICC) analysis of the two observers showed substantial-to-excellent agreement for D, f, D true and ADC (0.7, 0.8, 0.9 and 0.8, respectively). CONCLUSION: The results of our study suggest that impaired microperfusion with increased ADC values in adolescents with repaired TOF reflect hepatic congestion rather than fibrosis. Hepatic congestion characterized by decreased ADC values can be easily differentiated before fibrotic changes occur by using IVIM MRI to assess diffusion and microcapillary perfusion separately.

Evaluation of electrocardiography, echocardiography and cardiac T2* for cardiac complications in beta thalassemia major.

Cardiac complications such as heart failure and arrhythmias caused by "iron-induced" cardiomyopathy are considered as the primary cause of death in the patients with ß-thalassemia major. The aim of this study was to evaluate electrocardiography, echocardiography according cardiac T2* and ferritin findings of patients followed-up for ß-thalassemia major, and to investigate the importance of these findings for early detection of cardiac complications. The study included 41 patients and 25 healthy individuals with matched age and gender. The cardiac T2* results revealed a cardiac iron load below 20 ms in 12 (29.27%) patients, and above 20 ms in 29 (70.73%) patients. All electrocardiography parameters significantly increased in the patient group when compared to the control group (p < 0.05). All parameters except P wave segment in electrocardiography and T peak-end/QT ratio were significantly higher in the group with cardiac T2* < 20 ms than the group with cardiac T2* > 20 ms (p < 0.05). Intraventricular septum thickness, left ventricular posterior wall thickness, left ventricular mass and left ventricular mass index detected by echocardiography were significantly higher in the group with T2* < 20 ms (p < 0.05). Electrocardiography, echocardiography, cardiac T2* and ferritin findings should be carefully evaluated in these patients in order to detect early signs of cardiac complications.

Cardiac MRI in surgically repaired tetralogy of Fallot: Our initial experience.

OBJECTIVE: Pulmonary regurgitation (PR) required pulmonary valve replacement (PVR) is usually seen after surgically repaired tetralogy of Fallot (TOF). Assessment by cardiac magnetic resonance imaging (CMR) plays a crucial role in the decision of PVR. Herein, we presented our 3-year interdisciplinary CMR experience in the assessment of repaired TOF. METHODS: CMR examinations of 196 patients with repaired TOF performed between 2016 and 2018 were enrolled in this retrospective study. Only 165 were included in the study. CMR findings were assessed according to the American College of Cardiology/American Heart Association guideline and recommendations of Geva. RESULTS: Among those 165 patients (median age 14 years [mean age 15.62±7.42 years], M/F=114/61; 1.86/1), 73 patients were found eligible for PVR (59 patients for transcatheter while 14 patients for surgical). The mean QRS duration was 170.2±16.89 ms. On CMR assessment, mean indexed right ventricular end-diastolic volume, end-systolic volume, right, and left ventricular ejection fraction were 187.64±45.07 ml/m2, 39.90±6.60%, and 47.83±6.12%, respectively. The PR fraction was as 50.10±2.54% and 2.25±1.92. Balloon dilatation and/or stenting of branch pulmonary arteries in 12 patients and ventricular septal defect closure in four patients were performed at the same session of percutaneous PVR. At the time of the surgical PVR, repair of partial anomalous pulmonary venous return in one patient, ventricular septal defect in two patients, and subaortic membrane in one patient were performed. An implantable cardioverter-defibrillator was also performed in one patient. CONCLUSION: Our CMR experience has the largest patient population in our country and may contribute to the national data pool. We believe that our collaborative experience between radiologists, cardiologists, and cardiovascular surgeons may also enhance the use of CMR in determining the appropriate technique or timing for PVR.

Epiphrenic Diverticulum in an Infant with Congenital Esophageal Stenosis Associated with Esophageal Atresia.

Congenital esophageal stenosis (CES) is a rare congenital disorder which may be isolated or associated with esophageal atresia (EA). It courses with esophageal outflow tract obstruction. Esophageal epiphrenic diverticulae are esophageal outpouchings above diaphragm which develop because of an underlying esophageal motor disorder. We present an infant who had CES associated with EA detected during the course of routine follow-up. She underwent several sessions of esophageal balloon dilatations for CES with some symptomatic improvement. However, an epiphrenic diverticulum (ED) developed during the course of treatment which was detected by further investigations. A surgical excision was performed with a successful outcome. An ED developed secondary to CES has not been previously reported. We have highlighted the diagnostic modalities employed, treatment options, and clinical follow-up for these two rare conditions in children.

Left-sided Poland's syndrome associated with dextrocardia.

Poland's syndrome is a rare congenital anomaly accompanied by the absence of the pectoralis major, and the ipsilateral upper-limb and chest wall deformities. Hypoplasia of the breast, agenesis of the ipsilateral rib cartilage, athelia, and ipsilateral developmental finger anomalies such as syndactyly can also be seen. In the literature, only 56 cases of dextrocardia and left-sided Poland's syndrome have been described. Herein, a case of left-sided Poland's syndrome coexisting with dextrocardia and nasal hemangioma was presented.

Takayasu Arteritis Presenting as Renovascular Hypertension and Renal Failure in a Patient with Factor VII Deficiency.

A 23-year-old female patient with factor VII (FVII) deficiency was admitted with severe hypertension and renal failure. Brachial arterial pressures were 230/120 and 220/115 mm/Hg on the right and left arms, respectively. There was no blood pressure difference between the arms. Renal artery Doppler ultrasonography revealed bilateral severe renal artery stenosis (RAS). Contrast-enhanced magnetic resonance imaging angiography (CE-MRA) revealed severe mural irregularities, contrast enhancement in the aorta and its branches, and long-segment stenosis starting in the abdominal aorta and extending into the proximal renal arteries. The diagnosis of Takayasu arteritis (TA) complicated by RAS in a patient with FVII deficiency was established. This is the first case of concomitant TA and factor VII deficiency in the literature. In conclusion, TA complicated with RAS should be kept in mind in the etiology of secondary hypertension, even when there is no blood pressure difference between the arms in patients. CE-MRA is an accurate, sensitive, and safe imaging method for diagnosing vasculitis, even in the early phases of the disease, and should be considered for evaluating the activity and response to treatment in patients with TA.

Serum heparanase levels are associated with endothelial dysfunction in patients with obstructive sleep apnea.

BACKGROUND AND AIM: Obstructive sleep apnea syndrome (OSAS) is well-known to be associated with high risk for cardiovascular (CV) diseases. Heparanase has been recently shown to be related to increased inflammation and vulnerability of the atherosclerotic plaques. Herein we aimed to investigate the relationships between OSAS, heparanase and endothelial dysfunction. MATERIALS AND METHODS: A total of 120 patients with varying severity of OSAS and 31 controls without OSAS were enrolled. Flow-mediated dilatation (FMD) was measured as an indicator of endothelial dysfunction. Serum heparanase levels were measured with ELISA. RESULTS: Serum heparanase levels increased in a stepwise fashion from controls to patients with more severe OSAS. When FMD was compared with controls and various degrees of severity of OSAS, a stepwise decrease in FMD was observed. Serum heparanase levels were found to be significantly associated with apnea hypopnea index (AHI) (r = .57, P < .001) and FMD (r= -.37, P < .001) in patients with OSAS. Serum heparanase levels were significantly associated with hemoglobin-A1c and body mass index in patients with OSAS. Serum heparanase and uric acid levels were independent predictors of FMD in linear regression analysis (R2 = .506, P < .001; P < .001 and P = .001 respectively). CONCLUSIONS: Serum heparanase levels were significantly increased in patients with OSAS and associated with the severity of OSAS (AHI) and endothelial dysfunction (FMD). Increased heparanase activity in OSAS may be related to increased cardiovascular risk in patients with OSAS.

Contrast-induced acute kidney injury: A review of practical points.

Contrast-induced acute kidney injury (CI-AKI) is one of the most common causes of AKI in clinical practice. CI-AKI has been found to be strongly associated with morbidity and mortality of the patients. Furthermore, CI-AKI may not be always reversible and it may be associated with the development of chronic kidney disease. Pathophysiology of CI-AKI is not exactly understood and there is no consensus on the preventive strategies. CI-AKI is an active research area thus clinicians should be updated periodically about this topic. In this review, we aimed to discuss the indications of contrast-enhanced imaging, types of contrast media and their impact on nephrotoxicity, major pathophysiological mechanisms, risk factors and preventive strategies of CI-AKI and alternative non-contrast-enhanced imaging methods.