Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome

Background

Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited.

Evisceration with autogenous scleral graft and bioceramic implantation within the modified scleral shell: 133 cases over 17 years

Introduction: To present long-term follow-up data on evisceration performed with autogenous scleral grafting and ceramic implantation in a modified scleral shell.

Methods: This was a retrospective analysis of all consecutive eviscerations performed in the Department of Ophthalmology, Montpellier University Hospital, France, between February 1998 and October 2015. For all patients, the technique used was a conventional anterior evisceration after total keratectomy, disinsertion of the medial rectus muscle, sectioning of the optic nerve and excision of sclera centered on the papilla. The scleral graft was then sutured just behind the sutured keratectomy, and the bioceramic implant was inserted by posterior way in the scleral shell. Demographic characteristics, implant size and type, cosmetic results from pictures of all patients and complications were recorded. This study was performed with Ethics Review Committee Approval, and in compliance with the Declaration of Helsinki.

Results: In total, 133 patients (36.6% women) were identified during the study period. The mean (SD) implant size was 17.32 (1.84) mm. The median follow-up after evisceration was 57.43 (24.7, 68.3) months. Two cases of implant exposure (1.5%) were recorded. For 24 patients (17.9%), additional surgeries were performed for ptosis (2.2%), conjunctival cyst (1.5%), or post-evisceration socket syndrome (6.7%). Cosmetics results were excellent for 50.1% of cases, good for 33.3% and fair for 16.6%; using a grading scale based on the superior sulcus deformity.

Conclusion: Evisceration with autogenous scleral grafting and ceramic implantation can result in a high volume of restoration, good cosmetic results, and low risk of exposure of the implant.     

Inferior Vena Cava-Tricuspid Annulus Isthmus is a Critical Site of Unidirectional Block During the Induction of Common Atrial Flutter

Background: Until recently no clinical studies had reported precise right atrium (RA) mapping when performing induction of atrial flutter (AFI). We studied the mode of tachycardia initiation in 16 patients (pts) referred for radiofrequency (RF) AFl ablation. AFl induction was performed at the beginning of the procedure (n = 10), or after previous AFl termination during RF delivery (n = 6). Detailed analysis of AFl initiation was provided by duodecapolar (Halo) and multipolar catheters positioned in the peritricuspidian region at the lateral right atrial wall (LRA), the inferior vena cavatricuspid annulus (IVC-TA) isthmus and the interatrial septum. Induction was obtained during incremental pacing (IAP) (15 pts) or programmed stimulation (1 pt) from the proximal coronary sinus (PCS).Results: Atrial flutter with counterclockwise (CCW) RA rotation was induced in all pts by PCS pacing. During PCS IAP, at long pacing cycle lengths, impulse propagated in a clockwise (CW) direction through the IVC-TA isthmus and then upward at low (L) LRA. This led to a collision at the mid LRA with another wave front propagating in a CCW direction at the septum. IAP from PCS induced a progressive delay of propagation at the IVC-TA isthmus resulting in a prolongation of the PCS-Mid Isthmus interval from 85±29 to 151±42 msec. At same pacing cycle lengths (CL), the PCS-HLRA interval was comparatively less prolonged, from 75±12 to 105±18 msec, p = 0.0007. This preferential slowing of conduction between PCS and mid isthmus, during IAP from PCS, was associated with a displacement of the zone of collision to the Low LRA. Finally a CW functional block occurred at the IVC-TA isthmus and CCW AFl was induced through a period of transient concealed entrainment. The paced CL required to initiate flutter ranged from 290 to 180 msec and the mean CL of induced atrial flutter was 254±27 msec.Conclusions: The IVC-TA isthmus has decremental properties and exhibits wenckebach phenomenon during incremental PCS pacing. Initiation of a counterclockwise flutter by PCS pacing is associated with appearance of a functional unidirectional block at the IVC-TA isthmus.     

Mutations of the Imprinted CDKN1C Gene as a Cause of the Overgrowth Beckwith–Wiedemann Syndrome: Clinical Spectrum and Functional Characterization

Beckwith–Wiedemann syndrome (BWS) is an imprinting disorder associating macroglossia, abdominal wall defects, visceromegaly, and a high risk of childhood tumor. Molecular anomalies are mostly epigenetic; however, mutations of CDKN1C are implicated in 8% of cases, including both sporadic and familial forms. We aimed to describe the phenotype of BWS patients with CDKN1C mutations and develop a functional test for CDKN1C mutations. For each propositus, we sequenced the three exons and intron–exon boundaries of CDKN1C in patients presenting a BWS phenotype, including abdominal wall defects, without 11p15 methylation defects. We developed a functional test based on flow cytometry. We identified 37 mutations in 38 pedigrees (50 patients and seven fetuses). Analysis of parental samples when available showed that all mutations tested but one was inherited from the mother. The four missense mutations led to a less severe phenotype (lower frequency of exomphalos) than the other 33 mutations. The following four tumors occurred: one neuroblastoma, one ganglioneuroblastoma, one melanoma, and one acute lymphoid leukemia. Cases of BWS caused by CDKN1C mutations are not rare. CDKN1C sequencing should be performed for BWS patients presenting with abdominal wall defects or cleft palate without 11p15 methylation defects or body asymmetry, or in familial cases of BWS.     

Cardiac CT: Technological Advances in Hardware,Software, and Machine Learning Applications

Purpose of Review

Multidetector row computed tomography (CT) allows noninvasive imaging of the heart and coronary arteries. The purpose of this review is to briefly summarize recent advances in CT hardware and software technology, and machine learning applications for cardiovascular imaging.

Recent Findings

In the last decades, there have been significant improvements in CT hardware focusing on faster gantry rotation resulting in improved temporal resolution. Concurrent hardware improvements include improved spatial resolution and higher coverage of the patient, enabling faster acquisition. Advances in cardiac CT software include methods for measurement of noninvasive FFR, coronary plaque characterization, and adipose tissue characteristics around the heart. Machine learning approaches using cardiac CT have been shown to improve both risk of prognosis and lesion-specific ischemia.

Summary

Recent advances in CT hardware and software have expanded the clinical utility of CT for cardiovascular imaging. In the next decades, continued advances can be anticipated in these areas, and in machine learning applications in cardiac CT, as they are incorporated into clinical routine for image acquisition, image analysis, and prediction of patient outcomes.

     

In-stent fractional flow reserve variations and related optical coherence tomography findings: the FFR–OCT co-registration study

We sought to assess in-stent variations in fractional flow reserve (FFR) in patients with previous percutaneous coronary intervention (PCI) and to associate any drop in FFR with findings by optical coherence tomography (OCT) imaging. Suboptimal post-PCI FFR values were previously associated with poor outcomes. It is not known to which extent in-stent pressure loss contributes to reduced FFR. In this single-arm observational study, 26 patients who previously underwent PCI with drug-eluting stent or scaffold implantation were enrolled. Motorized FFR pullback during continuous intravenous adenosine infusion and OCT assessments was performed. Post-PCI FFR?<?0.94 was defined as suboptimal. At a median of 63 days after PCI (interquartile range: 59–64 days), 18 out of 26 patients (72%) had suboptimal FFR. The in-stent drop in FFR was significantly higher in patients with suboptimal FFR vs. patients with optimal FFR (0.08?±?0.07 vs. 0.01?±?0.02, p?<?0.001). Receiver operating characteristic curve analysis showed that an in-stent FFR variation of >?0.03 was associated with suboptimal FFR. In patients with suboptimal FFR, the OCT analyses revealed higher mean neointimal area (respectively: 1.06?±?0.80 vs. 0.51?±?0.23 mm²; p?=?0.018) and higher neointimal thickness of covered struts (respectively 0.11?±?0.07 vs. 0.06?±?0.01 mm; p?=?0.021). Suboptimal FFR values following stent-implantation are mainly caused by significant in-stent pressure loss during hyperemia. This finding is associated to a larger neointimal proliferation.     

Novel Z Scores to Correct Biases Due to Ventricular Volume Indexing to Body Surface Area in Adolescents and Young Adults

BackgroundReference values for cardiac magnetic resonance imaging (cMRI) in children and young adults are scarce. This leads to risk stratification of patients with congenital heart diseases being based on volumes indexed to body surface area (BSA). We aimed to produce cMRI Z score equations for ventricular volumes in children and young adults and to test whether indexing to BSA resulted in an incorrect assessment of ventricular dilation according to sex, body composition, and growth.MethodsWe retrospectively included 372 subjects aged < 26 years with either normal hearts or conditions with no impact on ventricular volumes (reference group), and 205 subjects with repaired tetralogy of Fallot (TOF) aged < 26 years. We generated Z score equations by means of multivariable regression modelling. Right ventricular dilation was assessed with the use of Z scores and compared with indexing to BSA in TOF subjects.ResultsVentricular volume Z scores were independent from age, sex, and anthropometric measurements, although volumes indexed to BSA showed significant residual association with sex and body size. In TOF subjects, indexing overestimated dilation in growing children and underestimated dilation in female compared with male subjects, and in overweight compared with lean subjects.ConclusionsIndexed ventricular volumes measured with cMRI did not completely adjust for body size and resulted in a differential error in the assessment of ventricular dilation according to sex and body size. Our proposed Z score equations solved this problem. Future studies should evaluate if ventricular volumes expressed as Z scores have a better prognostic value than volumes indexed to BSA.