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Introduction: Persistent left superior vena cava (PLSVC) is one of the major sources of triggers and drivers of atrial fibrillation (AF). There has been no established PLSVC ablation procedure to eliminate the arrhythmogenicity along the entire length of PLSVC. Methods and Results: A 70‐year‐old woman with a history of two previous catheter ablations for AF, mitral valvuloplasty, and an unroofed coronary sinus‐type atrial septal defect closure underwent the redo AF ablations. The AF trigger and driver were identified within the patient's enlarged PLSVC. The AF was treated by complete PLSVC free wall isolation. Conclusion: Complete PLSVC free wall isolation may be an effective ablation method to eliminate the arrhythmogenicity along the entire length of the PLSVC. [ABSTRACT FROM AUTHOR]

Introduction: Management of transvenous leads in patients with congenital heart disease (CHD) can be complicated by venous obstructions and residual shunts. We present our experience performing concurrent lead extraction and dilation/stenting of venous pathways, including patients with complete venous obstruction. Methods: All cases of concurrent lead extraction and recanalization of vena cavae/baffles between 2017 and 2021 at Boston Children's Hospital were retrospectively included and reviewed for safety and efficacy. Results: Eight patients, 4 female, median 38.5 years of age (range 16.7−49 years) and 81.6 kg weight (range 41.3−97.8 kg) at time of procedure were included. All patients had CHD, a majority (n = 7) having transposition of the great arteries palliated via atrial switch. All leads were removed in their entirety, with most patients having two leads extracted (n = 7). Median lead dwell time was 13.8 years (range 3.6−35.3 years). Three patients had complete obstructions, three required stenting of their innominate veins and three required recanalization of their femoral vessels. Median procedure time was 9.8 h (range 5.4−12.8 h). Complications included blood transfusion (n = 2), arrhythmia (n = 3), pleural effusion (n = 1), and pressure ulcer (n = 1). There were no cardiac perforations, venous tears, or deaths. Conclusion: Lead extraction along with dilation and stenting of venous anomalies, though long in duration, proved effective with minimal complications. This combined procedure can safely and effectively resolve complete obstructions secondary to transvenous leads. [ABSTRACT FROM AUTHOR]

Objective: Small extracellular vesicle (sEV)‐mediated intercellular communication is increasingly the key for the understanding of venous malformations (VMs). This study aims to clarify the detailed changes of sEVs in VMs. Subjects and Methods: Fifteen VM patients without treatment history and twelve healthy donors were enrolled in the study. sEVs were isolated from both fresh lesions and cell supernatant, and were examined by western blotting, nanoparticle tracking analysis and transmission electron microscopy. Western blot analysis, immunohistochemistry and immunofluorescence were adopted to screening candidate regulator of sEV size. Specific inhibitors and siRNA were employed to validate the role of dysregulated p‐AKT/vacuolar protein sorting‐associated protein 4B (VPS4B) signaling on the size of sEVs in endothelial cells. Results: The size of sEVs derived from both VM lesion tissues and cell model was significantly increased. VPS4B, whose expression level was mostly significantly downregulated in VM endothelial cells, was responsible for the size change of sEVs. Targeting abnormal AKT activation corrected the size change of sEVs by recovering the expression level of VPS4B. Conclusion: Downregulated VPS4B in endothelial cells, resulted from abnormally activated AKT signaling, contributed to the increased size of sEVs in VMs. [ABSTRACT FROM AUTHOR]

A 50‐year‐old woman underwent catheter ablation for atrial fibrillation. Preoperative computed tomography revealed a left‐sided variant of the right top pulmonary vein (PV) and a persistent left superior vena cava. The right top PV was successfully isolated through a wide antral circumferential ablation line simultaneously with the right PVs. [ABSTRACT FROM AUTHOR]

Background: Persistent left superior vena cava (PLSVC) is the most prevalent form of thoracic venous abnormality and can serve as a significant arrhythmogenic source in atrial fibrillation (AF). Methods and results: Among the 3950 patients who underwent radiofrequency ablation for AF between September 2014 to April 2020, 17 patients (mean age 59.4 ± 8.0 years, 64.7% male) with PLSVC were identified. Among them, nine patients (52.9%) had a prior history of pulmonary vein isolation (PVI) alone. Eight out of nine patients who experienced AF recurrence underwent PLSVC isolation with or without pulmonary vein (PV) reconnection. For the remaining eight patients (47.1%), PVI plus PLSVC isolation were performed during the index procedure. Ectopy originating from PLSVC was documented in 11 patients (64.7%) and successful PLSVC isolation was achieved in 16 patients (94.1%). After a median follow‐up of 28.3 months, freedom from AF/ atrial tachycardia (AT) was observed in 13 patients (76.5%). Conclusion: Empirical PLSVC isolation beyond PVI appears to be a feasible and safe strategy to prevent AF recurrence in patients with concomitant PLSVC. [ABSTRACT FROM AUTHOR]

The persistent left superior vena cava (PLSVC) is a common venous abnormality. However, malformation of the bilateral inferior venae cava (IVC) is extremely rare, with an incidence rate of.3%. IVC malformation is associated most frequently with heart defects and isomerism and often has a poor prognosis. We presented a case of vascular malformations in the fetus of bilateral caval veins with the interruption of the left‐sided venous return with hemiazygos continuation in presence of a right‐sided inferior caval vein. Also noted were the PLSVC and a dilated right heart with a widened pulmonary trunk. In this case, there were no heart defects or chromosomal abnormalities, and the newborn postpartum was in a good condition. [ABSTRACT FROM AUTHOR]

Background: The occurrence of Isolated left subclavian artery (ILSA) is relatively rare, ILSA is caused by the persistence of the dorsal segment of the sixth left arch, with regression of the fourth arch artery and interruption of the left dorsal aorta at the distal end of the seventh intersegmental artery on the left side during embryonic development. The left subclavian artery is connected to the pulmonary artery through an arterial duct, which can be closed or unobstructed. This abnormality can lead to congenital subclavian steal syndrome and vertebrobasilar artery insufficiency. Case presentation: We reported three fetuses with ILSA and intracardiac malformation. Among them, one case was suspected to be diagnosed with ILSA by echocardiography, while the other two cases were not diagnosed, but were accidentally discovered during autopsy. We have also conducted a literature review of its prenatal screening, diagnosis, management, and outcomes. Our three cases were tested by WES‐Trio (whole exome sequencing). Worldwide, the ILSA cases reported in English literature have not been detected by WES. And likely pathogenic results were found in our two cases. Although it could not explain the intracardiac malformation we found, it will help to explore the etiology in the future. Conclusions: Prenatal echocardiography detection and diagnosis of ILSA is a new challenge, which has different effects on the prognosis of the fetus. When finding intracardiac malformation with right aortic arch, we need to perform an unconventional view of ultrasound scanning and combine with CDFI to find the origin of the left subclavian artery. Although we cannot find the cause of the disease temporarily, but our genetic results can help prenatal genetic counseling. [ABSTRACT FROM AUTHOR]

Although the ductal anomalous origin of the pulmonary artery (DOPA) constitutes a rare heart anomaly, this malformation has a high mortality rate due to the rapid development of pulmonary hypertension(PTH) and right heart failure. Case Presentation: We report a case of DOPA, in which ductus arteriosus originated from the left pulmonary artery. This article summarizes the embryogenesis, clinical manifestations, complications and prognosis, diagnosis and experience, and treatment strategies of DOPA. The most fundamental sonographic finding was the lack of confluence at the bifurcation of the main pulmonary artery. Scanning upper mediastinum views is essential for the diagnosis. In addition, three‐dimensional echocardiography with high‐definition flow imaging and spatio‐temporal image correlation technique facilitates the identification of the anomalous origin of the pulmonary artery. It should be considered a complementary modality in fetal cardiac examinations. Although rare, DOPA can be diagnosed prenatally, usually at the three‐vessel view (3VV). The early diagnosis of DOPA thus can prevent the potentially devastating effects of PHT and right heart failure. [ABSTRACT FROM AUTHOR]

Introduction: Anomalous cardiac veins are not rare and pulmonary vein (PV) isolation for atrial fibrillation (AF) treatment should include these veins. Pulsed‐field ablation (PFA) is a novel technology for AF ablation with excellent efficacy and safety profile. In this case series, we describe our first experience of isolation of anomalous cardiac veins using PFA in patients with AF. Methods: We report a series of patients with congenital anomalies of the cardiac veins and AF, treated with PFA. All patients underwent cardiac computed tomography for procedural planning. Results: We included five patients (four males). Anomalous cardiac veins included a connection of a left common ostium to the coronary sinus, a partial and complete drainage of the right superior PV into the superior vena cava (SVC) with and without additional atrial septal defect, a persistent left SVC and an anomalous posterior PV. All anomalous PVs were isolated using PFA. No phrenic nerve palsy or other complications occurred. PFA of an abnormal right superior PV draining into the distal SVC was possible without affecting the sinus node. After a median of 4 months, four patients were free of recurrence. One patient had recurrent AF and perimitral reentry tachycardia, probably facilitated by PFA in the mitral isthmus region during isolation of an anomalous connection of the left common ostium to the coronary sinus. Conclusions: Using systematic preprocedural imaging and three‐dimensional‐electroanatomic mapping, the currently available PFA system seems well suited, efficient, and versatile for the treatment of AF in patients with anomalous cardiac veins. [ABSTRACT FROM AUTHOR]

Introduction: Persistent left superior vena cava (PLSVC) is accompanied by enlarged coronary sinus (CS) and deformation of the triangle of Koch. This makes anatomical evaluation of the atrioventricular (AV) nodal pathways difficult. Methods: We attempted cryoablation of retrograde fast pathway located in the enlarged CS roof of PLSVC for slow‐fast AV nodal reentrant tachycardia (AVNRT) induced by inadvertent antegrade fast pathway elimination during ablation of left atrial tachycardia. Results: Slow‐fast AVNRT was successfully eliminated without AV block progression. Conclusions: This is the first case of successful retrograde fast pathway ablation of the CS ostial roof for slow‐fast AVNRT with PLSVC. [ABSTRACT FROM AUTHOR]

Abernethy malformation, also known as congenital extrahepatic shunt, is a rare anomaly, which is characterized by partial or complete diversion of the portal blood into the systemic venous circulation. The clinical manifestations of Abernethy malformation during childhood include neonatal cholestasis, failure to thrive, mental retardation, and other congenital defects. We report a case of Abernethy malformation Type II in a 9‐year‐old boy, whose left ventricle was slightly enlarged because of several major aortopulmonary collateral arteries (MAPCAs) but laboratory examinations were normal 5 years earlier. The characteristics of congenital heart disease in patients with Abernethy malformation are discussed. We propose that physicians should be aware of the possibility of Abernethy malformation in children with enlargement of the left ventricular due to systemic‐pulmonary collateral circulation. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to examine the application value of high‐definition live (HDlive) flow combined with spatiotemporal image correlation (STIC) in the diagnosis of fetal inferior vena cava malformation (IVCM). Methods: Twenty cases of IVCMs were diagnosed using two‐dimensional HDlive flow and HDlive flow combined with STIC and retrospectively analyzed to examine the impact of using HDlive flow combined with STIC in the diagnosis of IVCM. Results: HDlive flow combined with STIC detected one case of duplicated IVC, four cases of left IVC (two cases with complex malformations), and 15 cases of interrupted IVC (two cases of isolated IVC disconnection, five cases with left atrial heterogeneous syndrome, and eight cases with other complex malformations). Conclusion: HDlive flow combined with STIC can help in the diagnosis of IVCM, and this technique has important clinical value. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 32 year old pregnant woman whose fetus was diagnosed with Partial Anomalous Pulmonary Venous Connection (PAPVC) during a fetal echocardiography at 33 weeks. It is reported that the baby, born without symptoms, had postnatal follow-ups confirming the closure of Persistent Foramen Ovale (PFO). It highlights the challenges in prenatally diagnosing isolated PAPVC and underscores the significance of thorough fetal echocardiographic assessments.

A 61‐year‐old male presented with symptoms of decompensated heart failure and cardiogenic shock. Transthoracic and transesophageal echocardiography showed severely impaired left ventricular (LV) systolic function (LVEF of 20–25%), bicuspid aortic valve with moderate aortic insufficiency and no significant stenosis, dilated coronary sinus and a tortuous vascular structure in the left‐sided atrioventricular groove. Cardiac computed tomography confirmed the diagnosis of persistent left superior vena cava and a giant coronary artery fistula to the coronary sinus. Cardiac magnetic resonance illustrated non‐specific late gadolinium enhancement in the mid‐wall of the septum. The patient was treated medically and with cardiac re‐synchronization therapy. [ABSTRACT FROM AUTHOR]

Background: Transvenous lead extraction (TLE) is rising in parallel to cardiac implantable electronic device implantations. Persistent left side superior vena cava (PLSVC) is a relatively common anatomical variant in the healthy population; TLE in patients with a PLSVC is rare. Method: Data were collated from 6 European TLE institutes of 10 patients who had undergone lead extraction with a PLSVC. Patient demographics, procedural challenges and outcomes were reported. Results: Ten patients aged 73.4 ± 7.8 years (60% male) underwent TLE of 20 leads (3 left ventricle, 10 right ventricle, 7 right atrium) with dwell time of 82.95 ± 39.1 months. Of the 10 cases, 4 had an infection indication and 5 were biventricular system extractions; 25% of the extracted leads were defibrillator leads. The majority of the procedures were completed in the cardiac catheterization suite (80%) under general anaesthesia (60%) by cardiologists (80%) using a rotational powered sheath (65%). The Tandem approach was used successfully in 3 cases. Complete procedural success was obtained in 100% of cases in the absence of complications within 127.4 ± 74.7 min. There was no 30‐day mortality. Conclusion: TLE in PLSVC is feasible albeit rare. Standard extraction techniques in experienced hands are associated with favorable outcomes; the Tandem procedure may be an additional technique to improve the safety and efficacy of TLE in PLSVC. [ABSTRACT FROM AUTHOR]

Persistent left superior vena cava (PLSVC) poses technical challenges to implantation of transvenous cardiac implantable electronic devices. His‐bundle pacing is a physiologic pacing strategy to avoid or treat pacing‐induced cardiomyopathy. We report a case of His‐bundle lead implantation in a patient with PLSVC, absent right SVC, and pacing‐induced cardiomyopathy. [ABSTRACT FROM AUTHOR]

Background: Little is known on the trends of aeromedical retrieval (AR) during social isolation. Aim: To compare the pre, lockdown, and post‐lockdown AR patient characteristics during a period of Coronavirus 2019 (COVID‐19) social isolation. Methods: An observational study with retrospective data collection, consisting of AR between 26 January and 23 June 2020. Results: There were 16 981 AR consisting of 1983 (11.7%) primary evacuations and 14 998 (88.3%) inter‐hospital transfers, with a population median age of 52 years (interquartile range 29.0–69.0), with 49.0% (n = 8283) of the cohort being male and 38.0% (n = 6399) being female. There were six confirmed and 230 suspected cases of COVID‐19, with the majority of cases (n = 134; 58.3%) in the social isolation period. As compared to pre‐restriction, the odds of retrieval for the restriction and post‐restriction period differed across time between the major diagnostic groups. This included, an increase in cardiovascular retrieval for both restriction and post‐restriction periods (odds ratio (OR) 1.12, 95% confidence interval (CI) 1.02–1.24 and OR 1.18 95%, CI 1.08–1.30 respectively), increases in neoplasm in the post restriction period (OR 1.31, 95% CI 1.04–1.64) and increases for congenital conditions in the restriction period (OR 2.56, 95% CI 1.39–4.71). Cardiovascular and congenital conditions had increased rates of priority 1 patients in the restriction and post restriction periods. There was a decrease in endocrine and metabolic disease retrievals in the restriction period (OR 0.72, 95% CI 0.53–0.98). There were lower odds during the post‐restriction period for retrievals of the respiratory system (OR 0.78, 95% CI 0.67–0.93), and disease of the skin (OR 0.78, 95% CI 0.6–1.0). Distribution between the 2019 and 2020 time periods differed (P < 0.05), with the lockdown period resulting in a significant reduction in activity. Conclusion: The lockdown period resulted in increased AR rates of circulatory and congenital conditions. [ABSTRACT FROM AUTHOR]

Aim: To review our experience with the prenatal diagnosis of congenital portosystemic shunt (CPSS). Methods: This is a retrospective study of CPSS cases examined at an ultrasonographic tertiary referral center from 2013 to 2019. The anatomical origin and drainage of the shunt were assessed. Feto‐maternal clinical characteristics and long‐term outcomes were investigated via medical files and telephone interviews with the mothers. Results: Eleven cases were reviewed. Based on the anatomical origins, before or after portal vein division, cases were classified into extrahepatic portosystemic shunt (EHPSS, n = 3, 27.3%) and intrahepatic portosystemic shunt (IHPSS, n = 8, 72.7%). Additional abnormalities were also observed in the EHPSS (n = 2, 66.7%) and IHPSS (n = 3, 37.5%) groups. Intrauterine growth restriction was the most common abnormality (n = 4, 80%). The median age of the pregnant women was 31.9 years (range 26 ~ 43 years). Most cases (n = 8, 72.7%) were diagnosed in the third trimester, and the median gestational age was 31+5 weeks (range 24 ~ 36+6 weeks). Three cases underwent karyotype examinations, and one had trisomy 13. The median time after birth was 2 years (range 0.7 ~ 5.7 years). The overall postnatal live‐birth rate was 60% (6/10), not including one case with no data on pregnancy outcome. The mothers of the six live births indicated that their children were in excellent health. Conclusion: This study indicates that prenatal CPSS diagnosis is feasible, especially in the third trimester. IHPSS is more common than EHPSS. Complicated cases most often occur with EHPSS. Intrauterine growth restriction is the most common concomitant abnormality. The prognosis of most cases is good. [ABSTRACT FROM AUTHOR]

Platypnea‐Orthodeoxia syndrome (POS) is a rare clinical syndrome characterized by shortness of breath in the upright position that is relieved with supine positioning. We present a rare case of persistent left superior vena cava (PLSVC) draining into the left atrium causing a right‐to‐left shunt and subsequent POS. A 30‐year‐old female with a past medical history of hypertrophic cardiomyopathy, congenital Long QT syndrome and a left‐sided dual chamber implantable cardioverter‐defibrillator (ICD) presented with dyspnea and lightheadedness. Prior to presentation, the patient underwent a left‐sided ICD extraction due to ICD lead infection and re‐implantation from the right side through the cephalic vein. After further investigation, it was concluded that the PLSVC resulted in a physiological right‐to‐left shunting causing POS, with resolution of her symptoms after surgical ligation. To our knowledge, this is the first case report of PLSVC presenting with POS without anatomical intracardiac shunts following iatrogenic right superior vena cava (RSVC) obstruction. [ABSTRACT FROM AUTHOR]

Objective: To determine the key sonographic features for the diagnosis of sirenomelia in the first trimester of pregnancy.Methods: Cases of sirenomelia from several prenatal diagnosis centers were retrospectively identified and reviewed. The diagnosis was established through the detection of fused lower limbs. Additional sonographic findings were also noted.Results: A total of 12 cases were collected. The most striking sonographic finding was the detection of malformed lower limbs, which appeared to be fused and in an atypical position. Nuchal translucency thickness was mildly increased in three cases (25%). An abdominal cyst, representing the dilated blind-ending bowel, was noted in seven cases (58%). Color flow imaging detected a single umbilical artery in six cases (50%) and the associated intra-abdominal vascular anomalies in three cases (25%). No cases of aneuploidy were detected. The pregnancy was terminated in nine cases (75%) and intrauterine demise occurred in the remaining three cases (25%).Conclusions: The sonographic detection of abnormal lower limbs or an intra-abdominal cyst located laterally during the first-trimester scan may be warning signs of sirenomelia. This should prompt a detailed examination of the fetal lower body and intra-abdominal anatomy, including the main abdominal vessels, in order to look for additional confirmatory findings. [ABSTRACT FROM AUTHOR]

The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety, and systematizing treatment, employing evidence‐based medicine (EBM) techniques and aimed at improvement of the outcomes. Clinical questions (CQs) were decided based on the important clinical issues. For document retrieval, key words for literature searches were set for each CQ and literature published from 1980 to the end of September 2014 was searched in Pubmed, Cochrane Library, and Japana Centra Revuo Medicina (JCRM). The strengths of evidence and recommendations acquired by systematic reviews were determined following the Medical Information Network Distribution System (MINDS) technique. A total of 33 CQs were used to compile recommendations and the subjects included efficacy of resection, sclerotherapy/embolization, drug therapy, laser therapy, radiotherapy, and other conservative treatment, differences in appropriate treatment due to the location of lesions and among symptoms, appropriate timing of treatment and tests, and pathological diagnosis deciding the diagnosis. Thus, the Japanese Clinical Practice Guidelines for Vascular Anomalies 2017 have been prepared as the evidence‐based guidelines for the management of vascular anomalies. [ABSTRACT FROM AUTHOR]

Vascular malformations arising from the wall of the external jugular vein are rare and appeared most commonly in pediatric population. Here, we present a case of vascular malformation in the left external jugular vein diagnosed in a fetus during third trimester ultrasound. This is the first described case in prenatal diagnosis. [ABSTRACT FROM AUTHOR]

A correction is presented to the article regarding the classification of Abernethy malformation in the discussion section, correcting it from "type 2 CEPS" to "type 1b CEPS" in El-Medany et al. (2022).

Background: 4D flow MRI shows great potential in neurovascular disorders such as stenosis, atherosclerotic disease, aneurysms, and vascular malformations. Its widespread application in the neurovascular system requires evidence of good test-retest multicenter reproducibility.Purpose: To assess the multicenter reproducibility, test-retest reliability and interobserver dependence of 4D flow MRI in measurements of cerebral blood flow/velocity in main intracranial vessels.Study Type: Prospective study.Subjects: Ten healthy subjects underwent 4D flow scans at three different centers. All subjects were scanned twice at 2 different days at each center.Field Strength/sequence: 3.0 T; 4D flow sequence.Assessment: Multicenter reproducibility, test-retest reliability and interobserver agreement for measurements of the blood flow and peak velocity from five regions of interest were assessed (bilateral internal carotid arteries, bilateral medial cerebral arteries, and sagittal sinus).Statistical Test: A Shapiro-Wilks test was conducted to assess normality of measurements in each scan. Coefficient of variances (CVs) was computed to evaluate intra- and intersite variances of all measurements. The multicenter reproducibility was assessed by two-way mixed intraclass correlation coefficient (ICC). A Bland-Altman plot and Pearson correlation were used to evaluate test-retest reliability. ICC was calculated to assess interobserver agreements.Results: All P-values for Shapiro-Wilks tests were greater than 0.05, which indicated the normality of all measurements. Both intra- and intersite CVs were lower than 12%. There was good test-retest reliability for both blood flow and peak velocity of all ROIs (r = 0.75-0.94). In addition, high multicenter reproducibility was detected (ICC = 0.77-0.96, all P < 0.001). The results of these measurements also showed great interobserver agreement (all ICC > 0.9 and all P < 0.001).Data Conclusion: High multicenter reproducibility and test-retest reliability was shown for 4D flow in the measurements of blood flow and peak velocity of intracranial vessels. In addition, these measurements showed great interobserver agreement.Level Of Evidence: 2 Technical Efficacy: Stage 1 J. Magn. Reson. Imaging 2019;49:1543-1552. [ABSTRACT FROM AUTHOR]

Objectives: To determine whether an abnormal orientation of the abdominal or hepatic vasculature and an abnormal gallbladder position on prenatal ultrasound (US) imaging are associated with intrathoracic liver herniation and postnatal outcomes in cases of congenital diaphragmatic hernia (CDH). Methods: Children who underwent prenatal US examinations and postnatal CDH repair at our institution were eligible. Prenatal US images were reviewed, and the orientation of the superior mesenteric artery (SMA) and hepatic veins as well as gallbladder position were recorded. Findings were correlated with prenatal US measurements (lung‐to‐head ratio and calculated observed‐to‐expected lung‐to‐head ratio) and postnatal outcomes, including intrathoracic liver herniation, an extracorporeal membrane oxygenation (ECMO) requirement, and mortality. Results: A total of 175 patients met inclusion criteria. The SMA was shown in 168 cases and had a cephalad orientation in 95.4% (161 of 168), which was not associated with outcome measures and represented bowel herniation. A cephalad orientation of the hepatic veins was identified in 52.6% (90 of 171) and was associated with intrathoracic liver herniation, an ECMO requirement, and mortality (P < .01). In right‐sided CDH, the gallbladder was intrathoracic in 91.3% (21 of 23). In left‐sided CDH, an abnormal gallbladder position was seen in 51.3% (76/152) and was associated with intrathoracic liver herniation, an ECMO requirement, mortality, and lower lung‐to‐head ratio and observed‐to‐expected lung‐to‐head ratio values. When combined, abnormal hepatic vein and gallbladder positions showed good sensitivity and specificity in predicting intrathoracic liver herniation (area under the curve, 0.93). Conclusions: Abnormal SMA, hepatic vein, and gallbladder positions can be used to improve prenatal characterization of CDH. Accurate depiction of these structures on prenatal US images may aid in patient counseling and postnatal management. [ABSTRACT FROM AUTHOR]

Background: Klippel–Trenaunay syndrome (KTS) and related overgrowth syndromes are rare conditions that often present to academic pediatric units and multidisciplinary vascular anomaly clinics. These conditions are commonly associated with abnormalities of the superficial and deep venous structures and carry an increased risk of venous thromboembolism. Methods: A retrospective chart review was completed of all patients treated at Johns Hopkins All Children's Hospital with endovenous laser ablation therapy (EVLT) for management of dilated embryonic veins in the setting of limb overgrowth. Results: Three patients with limb overgrowth underwent EVLT between 2015 and 2017. All patients had successful occlusion of the targeted veins on post‐procedure imaging. One patient developed a cellulitis successfully treated with oral antibiotics. Conclusions: Endovenous laser ablation therapy is a well‐tolerated and safe procedure for prophylactic closure of abnormal superficial embryonic veins. This study supports the use of this technique in managing increased thromboembolic risk in this unique patient population and recognizes the need for a long‐term study to determine its efficacy. [ABSTRACT FROM AUTHOR]

Congenital portosystemic shunts (CPSS) are defined as abnormal communications between the portal venous system and the systemic circulation. Ultrasound examination on day 6 depicted a shunt between the left hepatic vein and a branch arising from the left portal vein, and another shunt between the portal vein and the middle hepatic vein (Figure). GRAPH: Videoclip S2 Color Doppler ultrasound of fetal abdomen at 33 + 0 weeks' gestation, showing shunts between inferior left portal vein and left hepatic vein, and between medial left portal vein and right hepatic vein. [Extracted from the article]

This report was about a 60‐year‐old asymptomatic female patient who presented to our clinic with an anterior mediastinal mass found on routine chest computed tomography (CT). Chest CT revealed an irregular poorly enhanced anterior mediastinal mass which showed signs of infiltration to adjacent structures with sparse calcifications. The preliminary diagnosis was thymic carcinoma. The patient underwent extended thymectomy via median sternotomy and complete excision of the tumor. A small draining vein to the left brachiocephalic vein and phleboliths were identified in the tumor. A definitive diagnosis was made of mediastinal venolymphatic malformation (VLM). The patient had an uneventful clinical course and was discharged without further complication. This report highlights that it is possible to misdiagnose mediastinal VLM as thymic carcinoma and could serve as a useful reminder to physicians in the future. [ABSTRACT FROM AUTHOR]

Abstract: We describe a rare case of right aortic arch with bilateral arterial duct and disconnected left pulmonary artery. Prenatal diagnosis allowed to plan delivery at the referral center and to maintain perfusion of the disconnected pulmonary artery through prostaglandin E1 infusion until surgical reconnection was performed. Early postnatal treatment avoided functional loss of the left lung that would have happened if malformation had not been identified before birth. [ABSTRACT FROM AUTHOR]

Abstract: Objectives: Patients with acute headache increasingly undergo CT‐angiography (CTA) to evaluate underlying vascular causes. The aim of this study is to determine clinical and non‐contrast CT (NCCT) criteria to select patients who might benefit from CTA. Methods: We retrospectively included patients with acute headache who presented to the emergency department of an academic medical center and large regional teaching hospital and underwent NCCT and CTA. We identified factors that increased the probability of finding a vascular abnormality on CTA, performed multivariable regression analyses and determined discrimination with the c‐statistic. Results: A total of 384 patients underwent NCCT and CTA due to acute headache. NCCT was abnormal in 194 patients. Among these, we found abnormalities in 116 cases of which 99 aneurysms. In the remaining 190 with normal NCCT we found abnormalities in 12 cases; four unruptured aneurysms, three cerebral venous thrombosis’, two reversible cerebral vasoconstriction syndromes, two cervical arterial dissections and one cerebellar infarction. In multivariable analysis abnormal NCCT, lowered consciousness and presentation within 6 hr of headache onset were independently associated with abnormal CTA. The c‐statistic of abnormal NCCT alone was 0.80 (95% CI: 0.75–0.80), that also including the other two variables was 0.84 (95% CI: 0.80–0.88). If NCCT was normal no other factors could help identify patients at risk for abnormalities. Conclusions: In patients with acute headache abnormal NCCT is the strongest predictor of a vascular abnormality on CTA. If NCCT is normal no other predictors increase the probability of finding an abnormality on CTA and diagnostic yield is low. [ABSTRACT FROM AUTHOR]

The fifth Åland Island meeting on von Willebrand disease (VWD) was held on the Åland Islands, Finland, from 22 to 24 September 2016—90 years after the first case of VWD was diagnosed in a patient from the Åland Islands in 1926. This meeting brought together experts in the field of VWD to share knowledge and expertise on current trends and challenges in VWD. Topics included the storage and release of von Willebrand factor (VWF), epidemiology and diagnostics in VWD, treatment of VWD, angiogenesis and VWF inhibitors. [ABSTRACT FROM AUTHOR]

Abstract: Phacomatosis pigmentovascularis is characterized by coexistent extensive cutaneous vascular (capillary) and pigmentary anomalies. We describe a 2‐month‐old infant presenting with classic features of phacomatosis pigmentovascularis 2b (phacomatosis cesioflammea). He was also found to have hitherto unreported associations in the form of extensive venous anomalies presenting as striking abdominal wall varicosities and persistent left superior vena cava and natal tooth. [ABSTRACT FROM AUTHOR]

Abstract: Background: Cutaneous venous malformation (VM) can be associated with internal vascular anomalies. Our objective was to investigate the frequency of internal vascular anomalies in patients with an isolated genital venous malformation to assess the utility of screening for internal findings. Methods: We retrospectively reviewed our Vascular Anomalies Center database for patients with a focal genital venous malformation presenting between 1999 and 2016. Abdominal and pelvic imaging reports were reviewed for internal vascular anomalies. Endoscopy reports were also reviewed when available. Results: A focal genital venous malformation was found in 22 patients (14 female, 8 male). Ten (45%) had a venous malformation of at least one internal structure, most commonly the pelvic floor (n = 6), colon (n = 5), urethra (n = 4), and/or bladder (n = 3). Eight experienced hematuria, hematochezia, and/or rectorrhagia secondary to their internal venous malformation. In patients with internal venous malformations, the mean age of symptom presentation was 7.3 years (range 1‐22 years). Two patients had malformed inferior mesenteric and portal veins visible using ultrasonography and magnetic resonance imaging. They required surgical intervention to prevent thrombosis and decrease urogenital and gastrointestinal bleeding. Conclusion: Nearly half of our patients with a focal genital venous malformation had internal venous anomalies. Physicians should suspect urogenital or gastrointestinal venous malformations in patients with a focal genital venous malformation, especially if they develop hematuria, hematochezia, or rectorrhagia. Significant mesenteric venous trunk anomalies can also occur. Because these require surgical intervention, early recognition is important. We recommend that all patients with a focal genital venous malformation undergo abdominal and pelvic ultrasound to evaluate for internal venous anomalies. [ABSTRACT FROM AUTHOR]

Abstract: Bleomycin‐induced flagellate erythema is a rare but typical skin toxicity of bleomycin. We report the case of a boy with a left foot venous malformation who developed this skin rash after two sessions of bleomycin intralesional injection. We discuss the mechanism and characteristics of this reaction to bleomycin, which is usually benign and self‐limited. We conclude that, although rare, flagellate pigmentation can occur when bleomycin is used as a sclerosant in children. [ABSTRACT FROM AUTHOR]

Objective: Brain arteriovenous malformations (AVMs) are the most common cause of nontraumatic intracerebral hemorrhage in young adults. The genesis of brain AVM remains enigmatic. We investigated microRNA (miRNA) expression and its contribution to the pathogenesis of brain AVMs.Methods: We used a large-scale miRNA analysis of 16 samples including AVMs, hemangioblastoma, and controls to identify a distinct AVM miRNA signature. AVM smooth muscle cells (AVMSMCs) were isolated and identified by flow cytometry and immunohistochemistry, and candidate miRNAs were then tested in these cells. Migration, tube formation, and CCK-8-induced proliferation assays were used to test the effect of the miRNAs on phenotypic properties of AVMSMCs. A quantitative proteomics approach was used to identify protein expression changes in AVMSMCs treated with miRNA mimics.Results: A distinct AVM miRNA signature comprising a large portion of lowly expressed miRNAs was identified. Among these miRNAs, miR-137 and miR-195* levels were significantly decreased in AVMs and constituent AVMSMCs. Experimentally elevating the level of these microRNAs inhibited AVMSMC migration, tube formation, and survival in vitro and the formation of vascular rings in vivo. Proteomics showed the protein expression signature of AVMSMCs and identified downstream proteins regulated by miR-137 and miR-195* that were key signaling proteins involved in vessel development.Interpretation: Our results indicate that miR-137 and miR-195* act as vasculogenic suppressors in AVMs by altering phenotypic properties of AVMSMCs, and that the absence of miR-137 and miR-195* expression leads to abnormal vasculogenesis. Ann Neurol 2017;82:371-384. [ABSTRACT FROM AUTHOR]

Venous malformations are slow-flow congenital vascular malformations that enlarge as the child ages and may be associated with localised intravascular coagulation, a consumptive coagulopathy characterised by elevated D-dimer and decreased fibrinogen levels. The authors review the known correlations between localised intravascular coagulation and venous malformation number, size and planes involved, and call attention to the concept of the progression of localised intravascular coagulopathy as the child ages and their venous malformations enlarge. The authors also discuss the identified therapeutic options for its investigation, management and treatment, including compression garments, anti-coagulation therapy, sclerotherapy, endovascular laser, surgical excision and sirolimus (rapamycin). Evidence for protocol improvements that may be instigated for the optimal physical and medical therapy of venous malformations complicated by localised intravascular coagulopathy is reviewed. [ABSTRACT FROM AUTHOR]

Background The association of anaemia with aortic stenosis ( AS) has been recognised for over 50 years; however, although there have been several sporadic reports, there are few data on the prevalence of this syndrome. Aims We sought to compare the prevalence of anaemia in adults with AS with that of controls who had undergone coronary artery bypass grafting ( CABG). Methods We conducted a retrospective cohort study comparing pre-procedural levels of haemoglobin in 1537 adults who underwent aortic valve replacement ( AVR) for AS with 8025 contemporaneous patients who had CABG. We hypothesised that the prevalence of anaemia in patients with AS would be significantly higher than in the control group. Results A total of 30.1% in the AVR group was anaemic compared to 16.2% in the CABG group. The mean haemoglobin concentration measured across the whole population was significantly lower (132 g/L) in AVR patients than in those who underwent CABG (138 g/L). In a multivariable model, haemoglobin levels varied significantly by treatment group, gender and age. The adjusted marginal mean haemoglobin value was 135.6 g/L in AVR patients compared to 137.3 g/L in CABG patients. Conclusions The prevalence of anaemia was significantly greater in patients with AS than in a contemporaneous cohort that underwent CABG. This may indicate that Heyde syndrome is more common than has been generally appreciated and should be considered in the evaluation of anaemia in patients with AS. [ABSTRACT FROM AUTHOR]

The purpose of this study was to examine the effect of venous occlusion per se on forearm muscle blood flow, as determined by the near-infrared spectroscopy (NIRS) venous occlusion technique (NIRS-VOT). NIRS data were obtained from the flexor digitorum superficialis (FDS) muscle on the dominant arm of 16 young, ostensibly healthy participants (14 men and two women; 30 ± 6 year; 73 ± 7 kg). Participants completed a series of five venous occlusion trials while seated at rest, and a series of 12 venous occlusion trials during a reactive hyperaemia induced by 5 min of forearm arterial occlusion. The NIRS-VOT was used to assess FDS muscle blood flow ( [ABSTRACT FROM AUTHOR]

Objectives: To analyze a population of fetuses with prenatally diagnosed right aortic arch (RAA).Methods: Retrospective study of fetuses with RAA diagnosed prenatally between 2011 and 2015 in two referral centers.Results: Right aortic arch was found in 4.4% (46/1036) of fetuses with cardiovascular abnormalities (CVA). As an isolated anomaly, RAA was present in 30.4% of cases; in 32.6%, other CVA were detected; in 23.9%, CVA and extracardiac anomalies; and in 13.1%, only extracardiac malformations. The most common noncardiac abnormalities were thymus hypoplasia/aplasia (7/17), of which six had deletion 22q.11.2. In another three fetuses, trisomy 21 was present. One intrauterine fetal death occurred at 41 weeks of pregnancy, and two fetuses died after birth. In six of 18 infants with known follow-up, symptoms of dysphagia were reported, of which four infants underwent surgical intervention. In 12 infants, an isolated RAA was clinically silent.Conclusions: The diagnosis of RAA is an indication for a detailed examination of cardiac and noncardiac structures, including the thymus. It is advisable to consider genetic testing, together with the assessment of deletion 22q11.2, especially in the case of accompanying defects. The prognosis depends on underlying cardiac and extracardiac anomalies and possibly coexisting genetic defects. Isolated anomalies are asymptomatic. © 2017 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Objectives: The aim of the study was to assess the associated prenatal findings, genetic anomalies with chromosomal microarray analysis (CMA) and postnatal outcome of fetal right aortic arch (RAA).Methods: This retrospective study reviewed 92 fetuses diagnosed with RAA and the findings of CMA using Affymetrix CytoScan HD array in our institution between 2013 and 2016.Results: Postnatal data were not available for six cases, and genetic data were not available for 26 cases. Tetralogy of the Fallot was the most frequently associated anomaly. Among the 60 fetuses with known karyotype, one was 46, X, Yqh+, der(13)t(8;13)(q22.3;q33.2), one was 47, XYY and the remaining were normal. Our study showed that CMA could detect uncertain significant copy number variants in 5.2% of fetal RAA and pathogenic copy number variants in 5.2%, all of which were microdeletion in chromosome 22q11.21. The genetic anomalies, gestational age at delivery and postnatal death were not significantly different between RAA-no intracardiac anomalies and RAA-intracardiac anomalies group. One infant with aberrant left subclavian artery needed to perform a surgery for respiratory symptom.Conclusions: A right aortic arch is associated with 22q11.2 deletion syndrome in approximately 5% of cases, and, therefore, prenatal testing, preferably using CMA, should be offered. © 2017 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

A 2-months-old male German shepherd puppy was referred for regurgitation and delayed growth. Radiographic and endoscopic investigations revealed a precardiac megaoesophagus and oesophageal constriction at the level of the heart base. At post-mortem examination, a specific form of persistent right aortic arch characterized by an aberrant left subclavian artery in combination with a ligamentum arteriosum originating at the aberrant left subclavian artery ( PRAA- SA- LA) was detected. A complete-type persistent left cranial vena cava ( PLCVC) was also observed. This is the first report describing the association between PRAA- SA- LA and PLCVC in a dog with megaoesophagus. [ABSTRACT FROM AUTHOR]