BackgroundMarfan syndrome is associated with ventricular arrhythmia but risk factors including FBN1 mutation characteristics require elucidation.Methods and ResultsWe performed an observational cohort study of 80 consecutive adults (30 men, 50 women aged 42±15 years) with Marfan syndrome caused by FBN1 mutations. We assessed ventricular arrhythmia on baseline ambulatory electrocardiography as >10 premature ventricular complexes per hour (>10 PVC/h), as ventricular couplets (Couplet), or as non-sustained ventricular tachycardia (nsVT), and during 31±18 months of follow-up as ventricular tachycardia (VT) events (VTE) such as sudden cardiac death (SCD), and sustained ventricular tachycardia (sVT). We identified >10 PVC/h in 28 (35%), Couplet/nsVT in 32 (40%), and VTE in 6 patients (8%), including 3 with SCD (4%). PVC>10/h, Couplet/nsVT, and VTE exhibited increased N-terminal pro–brain natriuretic peptide serum levels(P<.001). All arrhythmias related to increased NT-proBNP (P<.001), where PVC>10/h and Couplet/nsVT also related to increased indexed end-systolic LV diameters (P = .024 and P = .020), to moderate mitral valve regurgitation (P = .018 and P = .003), and to prolonged QTc intervals (P = .001 and P = .006), respectively. Moreover, VTE related to mutations in exons 24–32 (P = .021). Kaplan–Meier analysis corroborated an association of VTE with increased NT-proBNP (P<.001) and with mutations in exons 24–32 (P<.001).ConclusionsMarfan syndrome with causative FBN1 mutations is associated with an increased risk for arrhythmia, and affected persons may require life-long monitoring. Ventricular arrhythmia on electrocardiography, signs of myocardial dysfunction and mutations in exons 24–32 may be risk factors of VTE.
However, with the increasing application of transvenous ICDs and higher patients' life expectancy, long-term technical difficulties, such as lead failure or device infection, have become important issues in clinical practice. [2][3][4] In addition, complications during lead placement, such as lead dislocation, perforation, or pneumothorax, bear a substantial perioperative risk. 5 The subcutaneous ICD (S-ICD) offers an alternative, novel approach to avoid lead-associated complications. 6 A first report from a single center has shown a promising device performance, with a sensitivity of 100% to detect ventricular tachyarrhythmia and 100% cardioversion efficacy. 7 Here, we report an initial multicenter experience with patients offered the S-ICD for both primary and secondary preventions of sudden cardiac death. Clinical Perspective on p 919 Methods PatientsThe study included all 40 consecutive patients (42±15 years [range, 19-73 Correspondence to Ali Aydin, MD, Department of Cardiology/Electrophysiology, University Heart Center, University Medical Center HamburgEppendorf, Martinistr, 5220246 Hamburg, Germany. E-mail aydin@uke.de Background-Recently, subcutaneous implantable cardioverter-defibrillator (S-ICD) has become available. The aim of our study was to assess the efficacy of S-ICD in a clinical setting. Table 1. Of note, the majority of our patients had an indication for secondary prevention (n=23; 58%). Methods and Results-Between Patient SelectionFiles of all patients receiving an S-ICD between June 2010 and July 2011 at the participating institutions were reviewed for this report. Patients were selected for an S-ICD if they fulfilled the indication criteria specified by the American College of Cardiology/ American Heart Association/European Society of Cardiology guidelines for primary or secondary prevention of sudden cardiac death. 1According to the recommendations given by the manufacturer, the S-ICD was not implanted in patients with symptomatic bradycardia, incessant ventricular tachycardia, or documented spontaneous, frequently recurring ventricular tachycardia that was reliably terminated with antitachycardia pacing. Patients with pacemakers were also classified as not suitable for an S-ICD. ECG morphology was preoperatively screened in accordance with the recommendations of the manufacturer to exclude patients with atrioventricular block, bundle branch block, and long-QT interval. S-ICD therapy was offered to all patients who fulfilled the criteria mentioned above; therefore, the patients' preference was 1 decision factor on transvenous ICD versus S-ICD. Also, patients with previous transvenous ICDs and an indication for removal were given the choice for another transvenous ICD or the S-ICD. Magnetic resonance tomography or transthoracic echocardiography was performed to assess left ventricular ejection fraction. Subcutaneous ICD SystemA commercially available S-ICD system (pulse generator model SQ-RX 1010 and subcutaneous lead model Q-Trak 3010, both from Cameron Health Inc, San Clemente, CA) wa...
AF is common in patients with HCM who need a CRM device. More than 50% of these patients develop de novo AF that was predominantly subclinical in our cohort.
ABSTRACT:In this study, the circulus arteriosus cerebri of the ground squirrel (Spermophilus citellus) was investigated. Five ground squirrels were used as subjects. Coloured latex was injected from the left ventriculi of the hearts of all the squirrels. When the vertebral arteries of two of the animals were ligatured, it was found that there was no internal carotid artery. After careful dissection, the circulus arteriosus cerebri (the circle of Willis) was investigated. The right and left vertebral arteries gave rise to the caudal cerebellar artery before forming the basilar artery. The basilar artery formed the caudal communicans artery that was the caudal part of the circulus arteriosus cerebri on the pontocrural groove (sulcus pontocruralis). The caudal, medial, rostral cerebellar, the common root formed by the caudal cerebral and choroid arteries, the rostral choroid, the rostral and medial cerebral arteries arose from the vertebral, basilar and caudal communicans arteries and dispersed to the cerebrum and cerebellum from caudal to cranial. The termination and the branches of the rostral cerebral artery in ground squirrels varied. It was observed that the internal carotid artery does not supply the circulus arteriosus cerebri in ground squirrels. Keywords: morphology; circulus arteriosus cerebri; brain; ground squirrel (Spermophilus citellus)The rodents (Rodentia) which are the widest order of placental mammals, comprise more than half of all described mammals. The ground squirrels (Spermophilus citellus) are representatives of the Sciuridae family that constitutes a group of the order Rodentia (Karol, 1963;Weichert, 1970;Kuru, 1987;Demirsoy, 1992).There have been many investigations on the vascularisation of the arteries which supply blood to the brain. Studies have been carried out in rats (Brown, 1966;Green, 1968), rats and mice (Firbas et al., 1973), mouse (Cook, 1965;Wiland, 1974;Szczurkowski et al., 2007), Guinea pigs (Ocal and Ozer, 1992), Guinea pigs and rabbits (Popesko et al., 1990), rabbits (Brehmer and Beleites, 1988), dogs (Miller et al., 1964), cats (McClure et al., 1973), porcupine (Aydin et al., 2005), Red squirrels (Aydin, 2008), Mongolian gerbils (Klachinka et al., 2008) and mole-rats . According to our knowledge there are no investigations on the circulus arteriosus cerebri of the ground squirrel (Spermophilus citellus) and this is the first study on this subject in ground squirrels.The purpose of this study was to document arteries that constitute the circulus arteriosus cerebri in the ground squirrel (Spermophilus citellus). MATERIAL AND METHODSFive adult ground squirrels, trapped by farmers, were used. After they were anaesthetized with penthathol (6 ml/kg), the cavum thoracis of all animals were opened and a 5 mm diameter, 7 cm long plastic pipe was placed into the left cardiac ventricle. The arterial blood was drained and red coloured latex was injected into the left ventriculi through this pipe. To see whether the internal carotid artery existed, the right and left vertebral arter...
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