Successful ductal stent implantation for initial palliation in two patients with aortic atresia and interrupted aortic arch.

The coexistence of aortic valve atresia and interrupted aortic arch are an extremely rare condition. In this pathology, blood flow to the ascending aorta and coronary arteries should be provided through the ductus arteriosus or collaterals originating from the descending aorta. In rare cases where bilateral ductus arteriosus is present, they can provide circulation. Here, we report two cases in which coronary arteries and ascending aorta were supplied by one ductus arteriosus and distal systemic circulation is supplied by a second ductus arteriosus in one patient and a collateral artery in the other. Initial palliation was successfully performed by bilateral pulmonary artery banding and transcatheter ductal stent implantation in both cases.

Relationship between myocardial bridging and fatal ventricular arrhythmias in patients with hypertrophic cardiomyopathy: the HCM-MB study.

BACKGROUND: Myocardial bridging (MB) and hypertrophic cardiomyopathy (HCM) are associated with the risk of fatal ventricular arrhythmias (VAs). The goal of the study was to determine the relationship between MB and fatal VAs in HCM patients with implantable cardiac defibrillators (ICD). METHODS: A total of 108 HCM patients (mean age: 46.6 ± 13.6 years; male: 73) were enrolled in this retrospective study. All patients underwent transthoracic echocardiography and coronary computed tomography angiography. Fatal VAs including sustained ventricular tachycardia and ventricular fibrillation were documented in ICD records. RESULTS: There were documented fatal VAs in 29 (26.8%) patients during a mean follow-up time of 71.3 ± 30.9 months. Compared with the other groups, the fatal VA group had a higher incidence of the following: presence of MB (82.8 vs. 38%, p < 0.001), deep MB (62.1 vs. 6.3%, p < 0.001), very deep MB (24.1 vs. 0%, p < 0.001), long MB (65.5 vs. 11.4%, p < 0.001), presence of > 1 MB (17.2 vs. 0%, p = 0.001), and MB of the left anterior descending artery (79.3 vs. 17.7%, p < 0.001) . Sudden cardiac death (SCD) risk score (hazard ratio: 1.194; 95% CI: 1.071-1.330; p = 0.001) and presence of MB (hazard ratio: 3.815; 95% CI: 1.41-10.284; p = 0.008) were found to be independent predictors of fatal VAs in HCM patients. CONCLUSIONS: The current data suggest that the SCD risk score and presence of MB were independent risk factors for fatal VAs in patients with HCM. In addition to conventional risk factors, the coronary anatomical course can provide clinicians with valuable information when assessing the risk of fatal VAs in HCM patients.

Structural Changesofthe Right Fibrous Trigone as a Risk Factor for Conduction Disturbance After Transcatheter Aortic Valve Implantation.

BACKGROUND: The right fibrous trigone, which the His bundle penetrates, is part of the subaortic area adjacent to the membranous septum. Structural alterations of the right fibrous trigone may cause conduction disturbance as a result of compression in this area after transcatheter aortic valve implantation. This study analyzed the hypothesis of whether imaging parameters of the RFT could predict the risk of conduction disturbance after transcatheter aortic valve implantation. METHODS: We retrospectively examined 209 patients who underwent transfemoral transcatheter aortic valve implantationat a tertiary cardiac center. The different valve models were divided into 2 groups: self-expanding valve and balloon-expandable valve. Using pre-procedure computed tomography, we evaluated for the alterations of the right fibrous trigone. RESULTS: New conduction disturbance was seen in 75 of 209 (35.8%) patients. Receiver operating characteristics plots displayed a right fibrous trigone density of -6 Hounsfieldunit for SEV and -16 Hounsfield unit for balloon-expandable valve as the optimal cut- off points for prediction conduction disturbance. In multiple regression analyses, the high density of RFT emerged as an independent predictor of conduction disturbance in both the self-expanding valve (odds ratio: 1.01, 95% CI: 1.01 to 1.02, P = 0.035) and bal- loon-expandable valve (odds ratio: 1.01, 95% CI: 1.01 to 1.03, P = 0.017) groups. A shorter membranous septum length and a greater implantation depth were also found to be significantly associated with a higher incidence of conduction disturbance in both trans- catheter aortic valve implantation groups. CONCLUSION: High density of right fibrous trigone is independently associated with con- duction disturbance after transcatheter aortic valve implantation, and its pre-proce- dure computed tomography evaluation can help predict the new-onset of conduction disturbance.

Comparison of echocardiography and 320-row multidetector computed tomography for the diagnosis of congenital heart disease in children.

INTRODUCTION: Echocardiography (echo) is the primary non-invasive imaging modality for the assessment of congenital heart disease (CHD). Computed tomography angiography (CTA) also has potential to examine the anatomy of complex heart anomalies as well as extracardiac involvement. OBJECTIVES: The aim of this study is to determine the impact of new CTA technology in the diagnosis of CHD and to compare echo and CTA in terms of diagnostic accuracy. METHODS: Forty-five patients who underwent preoperative echo and CTA assessment in the intensive care unit were included in this study. The results were assessed for three main types of CHD (cardiac malformations, cardiac-major vessel connections and major vessels). The main groups were also divided into subgroups according to surgical features in order to assess them more objectively. Imaging methods were compared for diagnostic accuracy, sensitivity and specificity, while surgical findings were accepted as the gold standard. RESULTS: Patients' median age and weight were two months (three days-eight years) and 12 kg (2.5-60 kg), respectively. In 45 operated cases, 205 subgroup malformations were assessed. Diagnostic accuracy was significantly greater in echo (echo vs. CTA: 98.4% and 96.2% [chi-square=6.4, p=0.011]). During surgery, 84 cardiac malformations (echo vs. CTA: 97.4% and 95.1% [chi-square=4.9, p=0.03]), 47 cardiac-major vessel connections (echo vs. CTA: 98.3% and 95.4% [chi-square=7.5, p=0.03]), and 74 major vessel malformations (echo vs. CTA: 96% and 98% [chi-square=1.8, p=0.48]) were confirmed. CONCLUSION: Echocardiography and CTA are imaging methods with high diagnostic accuracy in children with CHD. The use of echocardiography together with CTA, especially for the visualization of extracardiac anatomy, provides additional information for clinicians.

A rare cause of abnormal pulmonary venous drainage: septum primum malposition.

OBJECTIVES: This study aimed to evaluate the clinical features of patients with septum primum malposition, imaging tools used for diagnosis, and their effects on the surgical approach. MATERIALS AND METHODS: Patients diagnosed with septum primum malposition in our paediatric cardiac centre between 1 January, 2015 and 1 January, 2019 were included in the study. In all patients, the age, reason for admission, transthoracic echocardiography, cardiac multidetector CT angiography findings, and subsequent surgical data were evaluated. RESULTS: Fifteen patients were diagnosed with septum primum malposition during the study period. The median age was 12 months (2 months-10 years). Six patients were left isomeric, and the rest were situs solitus; 80% of the patients (n = 12) had additional secundum atrial septal defect. There was cardiac pathology in 46% of the patients (n = 7) in addition to the abnormal pulmonary venous drainage, ventricular septal defect (n = 3), left ventricularhypoplasia (n = 2), cortriatriatum sinister (n = 2), double outlet right ventricle (n = 1), and atrioventricular septal defect (n = 1). There was bilateral superior caval vein in three patients, right-sided superior caval vein in 11 patients, and left-sided superior caval vein in one patient. All three patients with total abnormal pulmonary venous drainage were left atrial isomeric. There were differences between the results of transthoracic echocardiography and CT angiographies in two patients. The surgical strategy was changed in three patients after the preoperative diagnosis of septum primum malposition. CONCLUSION: Septum primum malposition should be kept in mind during the imaging of complex CHDs specifically during the segmental analysis of the pathologies with heterotaxy syndromes; it should be differentiated from other aetiologies of abnormal pulmonary venous drainage as accurate diagnosis would facilitate the ideal surgery in these complex pathologies requiring a detailed preoperative preparation.

Low-Dose Computed Tomographic Imaging of Partial Anomalous Pulmonary Venous Connection in Children.

BACKGROUND: In this study, we aimed to determine lobar distribution, drainage sites, and associated cardiovascular anomalies of partial anomalous pulmonary venous connection in pediatric patients using low-dose multidetector computed tomographic angiography. METHODS: Sixty-one cases (27 female, mean age: 4.7 years) with partial anomalous pulmonary venous connection diagnosed by multidetector computed tomographic angiography were included in this study. In all patients, multidetector computed tomographic angiography examinations were performed using dual-source 256-slice scanner without sedation. RESULTS: In 61 patients, 73 anomalous pulmonary veins were detected, 56 (77%) of them were right-sided and 17 (23%) were left-sided. Of 56 right-sided anomalous pulmonary veins in 49 patients, 38 (68%) drained into superior vena cava, eight (14%) into atriocaval junction, six (11%) into inferior vena cava, three (5%) into right atrium, and one (2%) into levoatriocardinal vein. Of 17 left-sided anomalous pulmonary veins in 12 patients, 16 (94%) drained into left innominate vein, and one (6%) into coronary sinus. Only seven (12%) patients had isolated partial anomalous pulmonary venous connection, whereas 54 (88%) patients had additional cardiovascular anomalies. The most common (66%) associated anomaly is atrial septal defect. The overall mean effective radiation dose was 1.12 mSv (range: 0.15-7.41 mSv), and it was 0.58 mSv (range: 0.15-0.73) in the patients younger than one-year old. CONCLUSIONS: The presence and course of the anomalous pulmonary veins and associated cardiovascular anomalies can be reliably detected by dual-source 256-slice multidetector computed tomographic angiography with low radiation doses.

Total Anomalous Pulmonary Venous Connection in Children: Preoperative Evaluation with Low-Dose Multidetector Computed Tomographic Angiography.

We report the results of our retrospective evaluation, from February 2011 through August 2014, of the anatomic features of total anomalous pulmonary venous connection (TAPVC) and its associated cardiovascular anomalies in a pediatric population. In all 43 patients under study (23 female; average age, 9 mo [range, 4 d-7.1 yr]), these examinations had been performed with a dual-source 256-detector scanner. The type of TAPVC, the presence of obstruction, and the association with other cardiovascular anomalies were investigated and recorded. In accordance with the absence or presence of these accompanying anomalies, patients were subdivided into 2 groups: isolated and complex. In the 43 patients, 22 (51%) TAPVCs were supracardiac, 10 (23%) were cardiac, 6 (14%) were infracardiac, and 5 (12%) were mixed. Obstruction was detected in 7 patients. Seventeen patients were in the isolated group and 26 in the complex group. The diagnostic agreements between multidetector computed tomographic angiographic and surgical results were 100% in both the isolated and complex groups. The overall average effective radiation dose was 0.66 mSv (range, 0.15-1.11 mSv); and it was 0.52 mSv (range, 0.12-0.72 mSv) in patients younger than 1 year of age. We conclude that computed tomographic angiography with a dual-source 256-slice multidetector scanner is a reliable imaging method that enables, despite lower radiation doses, the detailed and comprehensive anatomic imaging of TAPVC in neonates and children.

Multidetector Computed Tomographic Angiography Imaging of Congenital Pulmonary Venous Anomalies: A Pictorial Review.

Congenital pulmonary venous anomalies are not uncommon that can occur either in isolation or in association with different forms of congenital heart disease. Clinical presentation of these anomalies may vary from the relatively benign single anomalous partial pulmonary venous return to life-threatening critical obstructed total anomalous pulmonary venous return. Accurate delineation of these anomalies and accompanied cardiovascular anomalies are crucial to guide decision making in these patients. Low-dose high-pitch dual-source 256-detector multidetector computed tomographic angiography is a fast and reliable imaging modality allowing comprehensive noninvasive anatomic imaging in neonates and children with congenital pulmonary venous anomalies with lower radiation doses and should be preferred for these patients after transthoracic echocardiography.

Assessment of children with vascular ring.

BACKGROUND: Vascular rings may cause pressure on the trachea and/or esophagus of varying degree, resulting in symptoms. This study assessed the presentation symptoms, diagnostic methods and treatment results after surgery in children with vascular ring. METHODS: Symptomatic vascular ring patients undergoing surgery between January 2010 and August 2014 at Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, were retrospectively assessed. The presentation symptoms, demographic characteristics, and diagnostic tests were evaluated. Operative data and postoperative follow up, complications and problems were also examined in detail. RESULTS: Twenty-one patients underwent surgery, 13 (63%) of whom were male. Median age was 12 months (range, 1 month-8 years). Among these patients, 62% (n = 13) had double aortic arch, 24% (n = 5) had right-sided aortic arch + ligamentum arteriosum, 10% (n = 2) had left-sided aortic arch + aberrant right subclavian artery, and 4% (n = 1) had pulmonary sling abnormality. Computed tomography (CT) angiography was performed in 17/21 patients. All the patients were successfully operated on. In the early postoperative period, two patients had chylothorax and one had nerve paralysis of nervus laryngeus recurrens. One patient died in the early period, and two patients had ongoing stridor on follow up. CONCLUSIONS: CT angiography is effective for the differential diagnosis and visualization of vascular ring abnormality. In such cases, full recovery can be assured with early diagnosis and surgery.

A rare pathology: Levoatriocardinal vein.

OBJECTIVE: Levoatriocardinal vein (LACV) is a rare cardiac pathology that represents a connection between the pulmonary venous and cardinal systems. The aim of the present study was to discuss morphological and clinical characteristics, as well as diagnostic methods, of experience with LACV. METHODS: Records of 11 patients (4 male, 7 female; mean age 79±1.83 days; range 1-390 days) diagnosed with LACV between 2010 and 2014 were retrospectively reviewed. Presence of LACV was confirmed with echocardiography. The primary obstructive lesion associated with cardiac defects and the integrity of the interatrial septum was identified in each patient with left-sided obstruction. RESULTS: Mean weight was 4.4±0.4 kg (range: 2-8). Age at presentation was under 1 year in 82% of patients. Nine patients had left-sided obstruction, and 2 had normal intracardiac anatomy and pulmonary venous return. In patients with left-sided obstruction, LACV was initially demonstrated with echocardiographic evaluation, performed in apical 4-chamber, high parasternal, and subcostal views. Atrial septum was restrictive or intact in patients with left-sided obstructions. LACV originated directly from the left atrium in all patients. CONCLUSION: Levoatriocardinal vein is an extremely rare cardiac pathology, presenting almost exclusively in patients with left-sided obstructive lesions. In patients with left-sided obstructions, LACV must be kept in mind. It may also present in patients with normal intracardiac anatomy and pulmonary venous return.

Robot-Assisted Repair of Right Partial Anomalous Pulmonary Venous Return.

Partial anomalous venous return from the right lung to the right atrium is a rare congenital heart anomaly in adults. We report a 20-year-old female, who presented with right partial anomalous pulmonary venous return and an associated inferior atrial septal defect. A complex repair was successfully done through a right atriotomy approach using a robotic surgical system. doi: 10.1111/jocs.12753 (J Card Surg 2016;31:394-397).

Serum sclerostin levels, arteriovenous fistula calcification and 2-years all-cause mortality in prevalent hemodialysis patients.

BACKGROUND: Bone and mineral abnormalities, and cardiovascular calcification are associated with increased cardiovascular mortality in patients with chronic kidney disease (CKD). Recent studies have implicated Wnt signaling pathway in the pathogenesis of bone metabolism and vascular calcification. Sclerostin is a soluble inhibitor of Wnt signaling pathway and has been shown to be associated with decreased bone turnover and vascular calcification in CKD patients. OBJECTIVES: The aim was to investigate whether the circulating levels of sclerostin are associated with all-cause mortality in prevalent hemodialysis patients. METHODS: Data are prospectively collected for 24 months for survival analysis in 350 prevalent hemodialysis patients. At baseline, serum sclerostin levels were measured and arteriovenous fistula calcification was detected by using a 64-detector computerized tomographic scanner. RESULTS: During the follow-up, 84 (24%) patients died. Patients who died had higher serum sclerostin levels. Kaplan-Meier curve revealed that patients with increasing tertiles of serum sclerostin levels at baseline, had a worse survival. In the multivariate Cox regression analysis age, albumin, and presence of arteriovenous fistula calcification, but not sclerostin levels, were found to be independent predictors of survival in maintenance hemodialysis patients. CONCLUSION: Further clinical studies with longer follow-up are needed to clarify the impact of serum sclerostin levels on morbidity and mortality of maintenance hemodialysis patients. CLINICAL TRIAL REGISTRATION NUMBER: The study was performed as a post hoc survival analysis of the patients involved in a single-center prospective trial investigating the association between serum sclerostin levels and arteriovenous fistula calcification and patency [Balci M, et al. Herz 2015;40:289-97] with a Clinicaltrials.gov number: NCT01382966.

Independent association between serum sclerostin levels and carotid artery atherosclerosis in prevalent haemodialysis patients.

BACKGROUND: Sclerostin is a soluble inhibitor of the Wnt signalling pathway and has been shown to be associated with decreased bone turnover and vascular and/or valvular calcification in patients with chronic kidney disease. Common carotid artery intima-media thickness (CIMT) assessment and common carotid artery (CCA) plaque identification with ultrasound imaging are well-recognized tools for the identification and monitoring of atherosclerosis. The aim of the present study was to investigate whether the circulating levels of sclerostin might be associated with carotid artery atherosclerosis in prevalent haemodialysis patients. METHODS: In this cross-sectional study, serum sclerostin concentrations were measured using a commercially available enzyme-linked immunosorbent assay kit. CIMT was measured and carotid plaques were identified by B-mode and Doppler ultrasound imaging. RESULTS: One hundred and twenty-two prevalent haemodialysis patients were involved in the study. Serum sclerostin levels were higher in patients with plaques in CCA than patients free of plaques (227 ± 166 versus 117 ± 91 pmol/L, P = 0.016). A significant correlation was recorded between serum sclerostin levels and CIMT (r = 0.459, P < 0.0001). In the multiple regression analysis, sclerostin concentrations were one of the independent factors that remained significantly associated with CIMT. CONCLUSION: Sclerostin is independently associated with CIMT although further studies are needed.