Anomalous origin of the left circumflex artery from the pulmonary artery associated with non-compaction of the left ventricle: usefulness of multimodality imaging-a case report.

Background: The anomalous origin of the left circumflex artery from the pulmonary artery (ACXAPA) is a very rare coronary anomaly. Only a few cases have been reported until today, from incidental findings to autopsy reports after sudden cardiac death. Case summary: We report here for the first time the case of a man, previously monitored for asymptomatic left ventricular non-compaction cardiomyopathy, who presented with non-ST myocardial infarction and was diagnosed with ACXAPA. Complementary tests confirmed ischaemia in the corresponding territory, and the patient was referred to surgery for reimplantation of the circumflex artery. Discussion: Left ventricular non-compaction cardiomyopathy is a rare congenital cardiomyopathy whose association with coronary anomalies, not with ACXAPA, had previously been described until now. A related embryological origin could potentially explain this association. The management of a coronary anomaly should indicate dedicated multimodality cardiac imaging in order to not disregard the association with underlying cardiomyopathy.

Left Atrial Remodeling Following ST-Segment-Elevation Myocardial Infarction Correlates With Infarct Size and Age Older Than 70 Years.

Background Following myocardial infarction, left ventricular remodeling (LVR) is associated with heart failure and cardiac death. At the same time, left atrial (LA) remodeling (LAR) is an essential part of the outcome of a wide spectrum of cardiac conditions. The authors sought to evaluate the correlates of LAR and its relationships with LVR after myocardial infarction. Methods and Results This is a retrospective analysis of 320 of 443 patients enrolled for study of LVR after ST-elevation myocardial infarction. Left ventricular (LV) volumes, infarct size and LA volume index were assessed by cardiac magnetic resonance imaging during index hospitalization (day 6 [interquartile range, 4-8]) and after a 3-month follow-up. LAR was studied using a linear mixed model for repeated measurements. Overall, there was a decrease in LA volume index between 6 days and 3 months (43.9±10.4 mL versus 42.8±11.1 mL, P=0.003). Patients with changes in LA volume index >8% over time were older, with greater body mass index, lower LV ejection fraction, and larger infarct size. Unadjusted predictors of LAR were age older than 70 years, infarct size, anterior infarction, time to reperfusion, history of hypertension, LV end-diastolic volume, and heart failure at day 6. Independent correlates were age older than 70 years (3.24±1.33, P=0.015) and infarct size (2.16±0.72 per 10% LV, P<0.001). LA remodeling was correlated with LV remodeling (r=0.372, P<0.001), but neither LA nor LV volumes at day 6 were related to LVR or LAR, respectively. Conclusions The authors found LA changes to occur in the months after myocardial infarction, with an overall decrease in LA volumes. While LAR coincided with LVR, the correlates for LAR were age older than 70 years and larger infarct size.

Prognostic value of cardiovascular magnetic resonance T1 mapping and extracellular volume fraction in nonischemic dilated cardiomyopathy.

BACKGROUND: Heart failure- (HF) and arrhythmia-related complications are the main causes of morbidity and mortality in patients with nonischemic dilated cardiomyopathy (NIDCM). Cardiovascular magnetic resonance (CMR) imaging is a noninvasive tool for risk stratification based on fibrosis assessment. Diffuse interstitial fibrosis in NIDCM may be a limitation for fibrosis assessment through late gadolinium enhancement (LGE), which might be overcome through quantitative T1 and extracellular volume (ECV) assessment. T1 and ECV prognostic value for arrhythmia-related events remain poorly investigated. We asked whether T1 and ECV have a prognostic value in NIDCM patients. METHODS: This prospective multicenter study analyzed 225 patients with NIDCM confirmed by CMR who were followed up for 2 years. CMR evaluation included LGE, native T1 mapping and ECV values. The primary endpoint was the occurrence of a major adverse cardiovascular event (MACE) which was divided in two groups: HF-related events and arrhythmia-related events. Optimal cutoffs for prediction of MACE occurrence were calculated for all CMR quantitative values. RESULTS: Fifty-eight patients (26%) developed a MACE during follow-up, 42 patients (19%) with HF-related events and 16 patients (7%) arrhythmia-related events. T1 Z-score (p = 0.008) and global ECV (p = 0.001) were associated with HF-related events occurrence, in addition to left ventricular ejection fraction (p < 0.001). ECV > 32.1% (optimal cutoff) remained the only CMR independent predictor of HF-related events occurrence (HR 2.15 [1.14-4.07], p = 0.018). In the arrhythmia-related events group, patients had increased native T1 Z-score and ECV values, with both T1 Z-score > 4.2 and ECV > 30.5% (optimal cutoffs) being independent predictors of arrhythmia-related events occurrence (respectively, HR 2.86 [1.06-7.68], p = 0.037 and HR 2.72 [1.01-7.36], p = 0.049). CONCLUSIONS: ECV was the sole independent predictive factor for both HF- and arrhythmia-related events in NIDCM patients. Native T1 was also an independent predictor in arrhythmia-related events occurrence. The addition of ECV and more importantly native T1 in the decision-making algorithm may improve arrhythmia risk stratification in NIDCM patients. Trial registration NCT02352129. Registered 2nd February 2015-Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT02352129.

Preservation of the Aortic Root During Type A Aortic Dissection Surgery: An Effective Strategy?

BACKGROUND: Management of the aortic root during acute Type A aortic dissection (TAAD) repair remains controversial in term of long-term evolution and reoperation. The aim of this study was to assess the long-term outcomes of the aortic root after conservative management during primary surgery. METHODS: One hundred sixty-four consecutive patients were included in this monocentric retrospective study. The primary endpoint was reoperation on the aortic root during long-term follow-up. Forty-six patients had aortic root replacement (ARR) and 118 had supracoronary aortic replacement (SCR). The 10-year survival, occurrence of significant aortic regurgitation, and radiologic aortic root dilatation in each group were assessed during follow-up. RESULTS: Patients from ARR group were younger than those from SCR group (p < 0.0001). Median follow-ups of ARR group and SCR group are 4.4 (interquartile range [IR]: 2.6-8.3) and 6.15 (IR: 2.8-10.53) years, respectively. Reoperation of the aortic root during long-term follow-up was similar in both groups (ARR group: 5.1%, SCR group: 3.3%, p = 0.636). The 10-year survivals of ARR and SCR groups were 64.8 ± 12.3% and 46.3 ± 5.8% (p = 0.012), respectively. Long-term significant aortic regurgitation occurred in one patient (1.7%) and seven patients (7.6%) of the ARR and SCR groups (p = 0.176), respectively. Radiologic aortic root diameters in the SCR group were similar between postoperative period and follow-up studies (p = 0.58). Reoperation on the distal aorta (p = 0.012) and patent radiologic false lumen of the descending aorta (p = 0.043) were independent risk factors of late death. CONCLUSION: SCR is an effective technique for primary TAAD surgery and does not increase the rate of late reoperation on the aortic root.

Renal function after partial nephrectomy following intra-arterial embolization of renal tumors.

Laparoscopic Partial Nephrectomy (LPN) after intra-arterial Embolization of renal tumors (LPNE) in a hybrid operating room allows renal tumor enucleation without dissection and clamping of the renal pedicle. The purpose was to assess the potential negative impact of embolization on the renal function. This prospective monocentric study included all patients treated with LPNE between May 2015 and June 2019. Clinical data was collected and incorporated into the UroCCR database (NCT03293563). Glomerular Filtration Rate (GFR) and Computed Tomography Renal Volume (CTRV) were compared before and after 6 months following LPNE. The mean post-operative GFR was 86.6 mL/min (SD 22.9). The mean GFR loss was 9.4% (SD 15.1) and the median renal parenchyma loss was 21 mL (SD 20.6). Using a threshold of 25% GFR loss, age was the only significant predictive factor of renal function impairment according to bivariate (59.5 vs 69.3 years, p = 0.017) and multivariable analysis (OR 1.075, CI 1-1.2], p = 0.05). Significant renal function impairment was not correlated with the renal parenchymal volume loss (OR 0.987, CI [0.95-1.02], p = 0.435). Renal function impairment after LPNE seems to be comparable to other techniques of partial nephrectomy.

Is an ischemic origin in MINOCA patients predictable?

This study sought to identify parameters that could guide towards an ischemic origin in patients hospitalized for myocardial infarction (MI) with non-obstructive coronary arteries (MINOCA). MINOCA is challenging in clinical practice, as the pathophysiology is multifaceted. A total of 135 patients with MINOCA who underwent cardiovascular magnetic resonance imaging (CMR) in a single tertiary University Hospital, were retrospectively included. The study cohort was classified into 4 groups according to the CMR diagnosis (i.e., myocarditis, myocardial infarction, Takotsubo cardiomyopathy, normal or uncommon diagnosis). According to the CMR, 62% had myocarditis, 14.1% myocardial infarction, 4.4% of Takotsubo and 19.3% showed a normal CMR or uncommon diagnoses. In the multivariate analysis, three criteria were independently correlated with the underlying diagnosis of myocardial infarction: (1) the absence of inflammatory response (HR: 5.71 IC95% [1.79-18.28]; p = 0.002), (2) the presence of coronary atheroma in invasive coronary angiography (HR: 6.56 IC95% [2.27-18.92]; p = 0.001) and (3) a peak of troponin ratio elevated than normal levels of 150 (HR: 4.12 IC95% [1.45-11.65]; p = 0.01). The prevalence of myocardial infarction in MINOCA was 4.9% in the absence of these three criteria, 3.4% with one of the criteria present, 34.5% with two criteria present and 71.4% with all three criteria. The negative predictive value for MI was 96% in the presence of at least two criteria. Our study shows that the absence of inflammatory response, a high troponin and the presence of angiographic coronary atheroma are independently correlated with a myocardial infarction underlying cause of MINOCA.

Early arterial calcification does not correlate with bone loss in pseudoxanthoma elasticum.

BACKGROUND AND AIMS: Pseudoxanthoma elasticum (PXE; OMIM 264800, prevalence 1/25,000 to 1/50,000) is an autosomal recessive multisystem disease due to deficiency in ABCC6, an ATP-binding cassette, sub-family C transporter. The PXE phenotype is mainly characterized by progressive ectopic calcification of connective tissues (namely skin, retinal Bruch's membrane and peripheral arteries) but the impact of PXE on bone structure is currently unknown. The present study sought to investigate bone mineralization and its potential link with vascular calcification in a large cohort of PXE patients with inherited mutations of the ABCC6 gene. METHODS AND RESULTS: 96 patients (61 women) matching the PXE criteria participated in this study. Their clinical history and status and bone biological markers were collected. Bone mineral density (BMD) was measured by dual energy X-ray absorptiometry and expressed as T- and Z-scores. Osteoporotic fractures were identified by X-ray, and coronary (CAC) and lower limb arterial calcification (LLAC) scores were determined by CT scan. RESULTS: 44% of the women were menopausal. Osteopenia was disclosed in 46% (17 women) while 23% (9 women) exhibited osteoporosis, 3 with severe osteoporosis. Fractures of an osteoporotic nature were authenticated in 3 patients (1 woman). Markers of bone remodelling processes (CTX, BSAP and osteocalcin) were within the normal range for our laboratory standards. Severe vitamin D deficiency (<25nmol/L) was found in 15%, while 51% exhibited no vitamin D deficiency (vitamin D≥50nmol/L). LLAC and CAC scores were significantly higher in the patients with a low T- and/or Z-score, although this difference disappeared in multivariate analysis with age as a confounding factor. There was no significant difference in LLAC and CAC between PXE patients with and without osteoporotic fractures. There was no statistically significant association between BMD, LLAC and CAC and any of the bone remodelling factors. CONCLUSIONS: This is the first report on the bone mineralization process in PXE patients. Our data shows that PXE patients are not markedly prone to exaggerated bone demineralization and fracture risk, and prevalence of osteoporosis remains within the normal range for the general population. Furthermore, the relationships between LLAC, but not CAC, and BMD with age are similar to those observed in the general population. Therefore, despite its pivotal role in ectopic calcification, ABCC6 deficiency does not interfere with the bone-vascular axis. The lack of PXE-related disturbances between BMD and arterial calcification also supports vitamin D supplementation in PXE patients with vitamin D deficiency. ClinicalTrials.gov Identifier: NCT01446393.

Relation of Quantity of Subepicardial Adipose Tissue to Infarct Size in Patients With ST-Elevation Myocardial Infarction.

According to the so-called obesity paradox, obesity might present a protective role in patients with myocardial infarction. We aimed to assess the influence of the epicardial adipose tissue (EAT) volume on cardiac healing and remodeling in patients with acute ST-elevation myocardial infarction. We prospectively included 193 consecutive patients presenting a first STEMI without known coronary artery disease. Cardiac magnetic resonance imaging was performed at baseline and after a 3-month follow-up. EAT volume was computed, and the population was divided into quartiles: the highest quartile of EAT defining the high EAT group (h-EAT). h-EAT was associated with increased body mass index, higher rate of history of hypertension, and smaller infarct size at initial CMR assessment (18.3 ± 11.9% vs 23 ± 13.7% of total left ventricular [LV] mass, p = 0.041). Moreover, microvascular obstruction was less frequent in the h-EAT group (36.2% vs 59.3%, p = 0.006). There were no differences in LV ejection fraction (LVEF), LV volumes, systolic wall stress, coronary artery burden, and clinical events during the index hospitalization between the EAT groups at baseline and at follow-up. Linear regression analysis showed h-EAT to be associated with smaller infarct size at baseline (ß coefficient = -3.25 [95% CI -5.89 to -0.61], p = 0.016). h-EAT also modified positively the effect of infarct size on LV remodeling, as assessed by the change in LVEF (p = 0.046). In conclusion, h-EAT was paradoxically related to smaller infarct size and acted as an effect modifier in the relation between the extent of infarct size and LVEF changes. Patients with higher extent of EAT presented better cardiac healing.

Influence of stentless versus stented valves on ventricular remodeling assessed at 6 months by magnetic resonance imaging and long-term follow-up.

BACKGROUND: To compare the effect of stented versus stentless bioprostheses on left ventricular remodeling and assess their impact on long-term survival. METHODS: From January 2002 to December 2009, 62 severe aortic stenosis patients without coronary artery disease were randomized for bioprosthetic aortic valve replacement. After randomization, a cross-over was possible based on intraoperative data. Ventricular remodeling was studied by cardiovascular magnetic resonance imaging six months after surgery. Long-term survival was assessed by telephone survey. RESULTS: Thirty-five patients received a porcine Mosaïc® Medtronic bioprosthesis (Stented Group; Medtronic, Minneapolis, MN, USA) inserted using the usual supra-annular technique and 27 received a porcine Freestyle® Medtronic bioprosthesis (Stentless Group) inserted in the subcoronary position. Mean age was 75±3 and 73±4 years in the stentless and stented group, respectively. Nine patients who should have been implanted with a stentless bioprosthesis received a stented bioprosthesis for anatomical reasons. At 6 months, the left ventricular mass (LVM) decreased significantly in both groups (Stentless Group: 214.6±56.1g and 156.3±23g and Stented Group: 237±75.7g and 181±53.3g, respectively after surgery and at 6 months), this decrease was significantly greater in the stentless group (p=0.026). Reserve and coronary flow were increased in both groups at 6 months. Mean follow-up duration was 6.6±3.0 years and 7.2±4.0 years in the stentless and stented group, respectively. The 5-year actuarial survival was 87.5±11.7% and 82.5±17.1% for the stentless and stented group, respectively (p=0.81). CONCLUSION: Porcine stentless prosthesis results in a better LVM regression than a stented valve at 6 months without changing the long-term survival.

Uterine Artery Embolization for Retained Products of Conception with Marked Vascularity: A Safe and Efficient First-Line Treatment.

OBJECTIVE: To report our clinical practice regarding a case series of retained products of conception (RPOC) with marked vascularity (MV) managed with selective uterine artery embolization (UAE) as first-line treatment. METHODS: This was a monocentric, retrospective study of 31 consecutive cases of RPOC with MV diagnosed by Doppler ultrasound in the context of postpartum/postabortal bleeding. The primary outcome was the absence of rebleeding following embolization. RESULTS: RPOC with MV occurred after abortion in 27 out of 31 patients (87%). The time elapsed between delivery/abortion and UAE ranged from 1 to 210 days (mean 55.7 ± 45 days). Primary clinical success was achieved in 23 women (74.2%) following a single embolization. In total, 27 out of 31 women (87%) had been exclusively managed by UAE with conservative success. Although procedural success was achieved in this number, six women had a further procedure to evacuate RPOC despite procedural success. Large uterine arteriovenous (AV) shunts associated with RPOC were observed in five cases (16.1%), among which two were successfully treated after a single UAE and one after two UAEs, while hysterectomy was performed in the last two cases despite two and three UAE procedures respectively. RPOC was histologically proven in ten cases (32.2%) including four out of five cases of uterine AV shunt. CONCLUSION: RPOC with MV can present with large uterine AV shunt, particularly in case of late management. Uterine artery embolization is an effective and safe first-line treatment, and should be evaluated for this indication in larger prospective trials.

First pass perfusion imaging to improve the assessment of left ventricular thrombus following a myocardial infarction.

PURPOSE: To assess whether a first-pass perfusion sequence (FPP) improved the detection of left ventricular thrombus (LVT). MATERIALS AND METHODS: Three hundred and twenty-nine patients with a first STEMI were prospectively included to undergo cardiac magnetic resonance (CMR) at baseline and after a 3-month follow-up. A CMR delayed analysis was performed by three blinded examiners (2 CMR experts and 1 novice) according to a two-step reading protocol. First, an analysis was performed on cine CMR and late gadolinium enhancement (routine stage). Then, the FPP stage was performed following initial protocol along with a FPP sequence. RESULTS: LVT was found in 31 out of a total of 638 (4.9%) CMR scans, affecting 30 (9.1%) individuals. All were located in the left ventricular apex. The FPP stage improved significantly the LVT diagnosis for all readers, in 10 and 13 cases (32% and 42%) of LVT suspicion for the experts and 16 cases (41%) for the novice. Respectively 1, 2 and 6 LVT were not detected during the routine stage by the CMR experts and the novice. For the novice, the FPP stage improved diagnosis sensitivity from 78.1 to 91.2%. CONCLUSIONS: The prevalence of LVT following a myocardial infarction reached 9.1% and increased with the reading of FPP sequence. The FPP stage improved expert diagnostic certitude and the novice's abilities to reach expert level.

A semi-automated method for measuring the evolution of both lumen area and blood flow in carotid from Phase Contrast MRI.

Phase-Contrast (PC) velocimetry Magnetic Resonance Imaging (MRI) is a useful modality to explore cardiovascular pathologies, but requires the automatic segmentation of vessels and the measurement of both lumen area and blood flow evolutions. In this paper, we propose a semi-automated method for extracting lumen boundaries of the carotid artery and compute both lumen area and blood flow evolutions over the cardiac cycle. This method uses narrow band region-based active contours in order to correctly capture the lumen boundary without being corrupted by surrounding structures. This approach is compared to traditional edge-based active contours, considered in related works, which significantly underestimate lumen area and blood flow. Experiments are performed using both a sequence of a homemade phantom and sequences of 20 real carotids, including a comparison with manual segmentation performed by a radiologist expert. Results obtained on the phantom sequence show that the edge-based approach leads to an underestimate of carotid lumen area and related flows of respectively 18.68% and 4.95%. This appears significantly larger than weak errors obtained using the region-based approach (respectively 2.73% and 1.23%). Benefits appear even better on the real sequences. The edge-based approach leads to underestimates of 40.88% for areas and 13.39% for blood flows, compared to limited errors of 7.41% and 4.6% with our method. Experiments also illustrate the high variability and therefore the lack of reliability of manual segmentation.

Predictive Factors of Pericardial Effusion After a First Acute Myocardial Infarction and Successful Reperfusion.

The aim of the study was to identify the determinants of pericardial effusion (PE) after a first myocardial infarction (MI). Cardiac magnetic resonance enables early analysis of multiple post-MI parameters; 193 patients with a first ST-elevation MI admitted to the Angers University Hospital (France) were enrolled prospectively. Cardiac magnetic resonance was performed at baseline (median of 5 days [4 to 7]) and repeated at a 3-month follow-up to investigate left ventricular (LV) volumes, LV ejection fraction, infarct size, microvascular obstruction (MVO), systolic wall stress (SWS), and PE presence and extent. A 1-year follow-up was also performed. Overall, 113 patients (58.5%) showed a PE with a median size of 31.6 ± 24.0 ml in the event that a PE was present. Patients with PE typically presented larger initial infarct sizes and LV volumes, and higher SWS, with more depressed LV ejection fraction and more frequent MVO and pleural effusions. Patients with PE exhibited higher rates of heart failure during hospitalization. At follow-up, there was no relevant PE, with no pericardiocentesis required. The multivariate analysis revealed SWS (odds ratio [OR] 1.092 [95% CI 1.007 to 1.184], p = 0.042), infarct size (OR 1.048 [95% CI 1.014 to 1.083], p = 0.003), and MVO extent (OR 1.274 [95% CI 1.028 to 1.579], p = 0.018) to be independent predictors for PE presence and volume. One patient died of LV free wall rupture during initial hospitalization, with only "small" PE found. In conclusion, infarct size, MVO, and SWS were independently related to PE presence and volume. Post-MI PE was found in 58.5% of cases, being regressive at follow-up. Among these patients with early reperfusion and optimal medical therapy, PE volume did not seem to be related to future clinical events.

End-systolic wall stress predicts post-discharge heart failure after acute myocardial infarction.

BACKGROUND: Compensatory mechanisms activated after myocardial infarction include an increase in systolic wall stress (SWS) and activation of the neurohormonal system. Nevertheless, left ventricular ejection fraction (LVEF) and infarct size are the established primary predictors of outcome after ST-segment elevation myocardial infarction. AIMS: To assess the relative impact of various cardiac magnetic resonance (CMR) imaging variables, such as infarct size, LVEF and SWS, on pre- and post-discharge heart failure (HF). METHODS: CMR was performed in a prospective study involving 169 patients with first ST-segment elevation myocardial infarction. Common CMR findings, such as SWS, were computed. RESULTS: Mean SWS was 16.3±5.1×10(3)N·m(-2), and was systematically higher in patients exhibiting either pre- or post-discharge HF (18.9±5.7 and 21.3±7.6×10(3) N·m(-2), respectively). SWS was moderately related to initial infarct size (r=0.405; P <0.001). In total, 28 patients presented with HF during the hospitalization phase and 14 during follow-up, with a median time of event of 93 days (25th-75th percentiles, 29-139.25 days). The univariate predictors of HF were age, LVEF, infarct size, SWS, microvascular obstruction, anterior infarction and heart rate at admission. Multivariable analysis revealed infarct size and age to be the predictors of predischarge HF, while SWS and heart rate at admission predicted post-discharge HF. The greatest SWS quartile provided a negative predictive value of 95.9%. CONCLUSION: Regardless of LVEF and infarct size, SWS was shown to be an independent predictor of post-discharge HF after ST-segment elevation myocardial infarction.

Worsening of Obstructive Sleep Apnea Associated with Catheter-Related Superior Vena Cava Syndrome.

ABSTRACT: There is growing evidence that fluid accumulation in the neck contributes to the pathogenesis of obstructive sleep apnea (OSA). We describe a case of catheter-related superior v ena cava (SVC) thrombosis revealed by rapid onset of typical symptoms of OSA. A marked improvement in OSA severity was observed after central venous catheter removal, anticoagulant therapy, and SVC angioplasty.

The contribution of arterial calcification to peripheral arterial disease in pseudoxanthoma elasticum.

BACKGROUND AND AIMS: The contribution of arterial calcification (AC) in peripheral arterial disease (PAD) and arterial wall compressibility is a matter of debate. Pseudoxanthoma elasticum (PXE), an inherited metabolic disease due to ABCC6 gene mutations, combines elastic fiber fragmentation and calcification in various soft tissues including the arterial wall. Since AC is associated with PAD, a frequent complication of PXE, we sought to determine the role of AC in PAD and arterial wall compressibility in this group of patients. METHODS AND RESULTS: Arterial compressibility and patency were determined by ankle-brachial pressure index (ABI) in a cohort of 71 PXE patients (mean age 48 ± SD 14 yrs, 45 women) and compared to 30 controls without PAD. Lower limb arterial calcification (LLAC) was determined by non-contrast enhanced helicoidal CT-scan. A calcification score (Ca-score) was computed for the femoral, popliteal and sub-popliteal artery segments of both legs. Forty patients with PXE had an ABI<0.90 and none had an ABI>1.40. LLAC increased with age, significantly more in PXE subjects than controls. A negative association was found between LLAC and ABI (r = -0.363, p = 0.002). The LLAC was independently associated with PXE and age, and ABI was not linked to cardiovascular risk factors. CONCLUSIONS: The presence of AC was associated with PAD and PXE without affecting arterial compressibility. PAD in PXE patients is probably due to proximal obstructive lesions developing independently from cardiovascular risk factors.

Longitudinal strain is a marker of microvascular obstruction and infarct size in patients with acute ST-segment elevation myocardial infarction.

OBJECTIVES: We assessed the value of speckle tracking imaging performed early after a first ST-segment elevation myocardial infarction (STEMI) in order to predict infarct size and functional recovery at 3-month follow-up. METHODS: 44 patients with STEMI who underwent revascularization within 12 h of symptom onset were prospectively enrolled. Echocardiography was performed 3.9 ± 1.2 days after myocardial reperfusion, assessing circumferential (CGS), radial (RGS), and longitudinal global (GLS) strains. Late gadolinium-enhanced cardiac magnetic imaging (CMR), for assessing cardiac function, infarct size, and microvascular obstruction (MVO), was conducted 5.6 ± 2.5 days and 99.4 ± 4.6 days after myocardial reperfusion. RESULTS: GLS was evaluable in 97% of the patients, while CGS and RGS could be assessed in 85%. Infarct size significantly correlated with GLS (R = 0.601, p<0.001), RGS (R = -0.405, p = 0.010), CGS (R = 0.526, p = 0.001), ejection fraction (R = -0.699, p<0.001), wall motion score index (WMSI) (R = 0.539, p = 0.001), and left atrial volume (R = 0.510, p<0.001). Baseline ejection fraction and GLS were independent predictors of 3-month infarct size. MVO mass significantly correlated with GLS (R = 0.376, p = 0.010), WMSI (R = 0.387, p = 0.011), and ejection fraction (R = -0.389, p = 0.011). In multivariate analysis, GLS was the only independent predictor of MVO mass (p = 0.015). Longitudinal strain >-6.0% within the infarcted area exhibited 96% specificity and 61% sensitivity for predicting the persistence of akinesia (≥ 3 segments) at 3-month follow-up. CONCLUSIONS: Speckle-tracking strain imaging performed early after a STEMI is easy-to-use as a marker for persistent akinetic territories at 3 months. In addition, GLS correlated significantly with MVO and final infarct size, both parameters being relevant post-MI prognostic factors, usually obtained via CMR.

CMR assessment after a transapical-transcatheter aortic valve implantation.

AIMS: To describe the time course of myocardial scarring after transapical-transcatheter aortic valve implantation (TA-TAVI) with the Edwards SAPIEN XT™ and the Edwards SAPIEN™ prosthesis in a 3-month follow-up study using cardiac magnetic resonance imaging (CMR). METHODS: In 20 TA-TAVI patients, CMR was performed at discharge and 3 months (3M). Cine-MRI was used for left ventricular (LV) functional assessment, and late gadolinium enhancement (LGE) imaging was employed for detecting the presence of myocardial scarring. Special attention was given to any artifacts caused by the prosthesis, which were consequently defined using a three-grade artifact scale. RESULTS: We systematically reported the presence of small LGE hyperintensity relating to the apical segment, with no variation found between discharge and 3M (2.8±1.6g vs. 2.35±1.1g). LV ejection fraction, end-diastolic, and end-systolic volumes did not significantly vary. A small area of apical akinesia was observed, with no improvement at follow-up. Whereas the Edwards SAPIEN XT™ prosthesis and the Edwards SAPIEN™ prosthesis are both constituted by metallic stenting structure, the Edwards SAPIEN™ was responsible for a larger signal void, thus potentially limiting the diagnostic performance of CMR. CONCLUSIONS: CMR may be performed safely in the context of TA-TAVI. The presence of a very small apical infarction correlating with focal akinesia was observed. As expected, the Edwards SAPIEN XT™ prosthesis was shown to be particularly suitable for CMR assessment.

The vascular phenotype in Pseudoxanthoma elasticum and related disorders: contribution of a genetic disease to the understanding of vascular calcification.

Vascular calcification is a complex and dynamic process occurring in various physiological conditions such as aging and exercise or in acquired metabolic disorders like diabetes or chronic renal insufficiency. Arterial calcifications are also observed in several genetic diseases revealing the important role of unbalanced or defective anti- or pro-calcifying factors. Pseudoxanthoma elasticum (PXE) is an inherited disease (OMIM 264800) characterized by elastic fiber fragmentation and calcification in various soft conjunctive tissues including the skin, eyes, and arterial media. The PXE disease results from mutations in the ABCC6 gene, encoding an ATP-binding cassette transporter primarily expressed in the liver, kidneys suggesting that it is a prototypic metabolic soft-tissue calcifying disease of genetic origin. The clinical expression of the PXE arterial disease is characterized by an increased risk for coronary (myocardial infarction), cerebral (aneurysm and stroke), and lower limb peripheral artery disease. However, the structural and functional changes in the arterial wall induced by PXE are still unexplained. The use of a recombinant mouse model inactivated for the Abcc6 gene is an important tool for the understanding of the PXE pathophysiology although the vascular impact in this model remains limited to date. Overlapping of the PXE phenotype with other inherited calcifying diseases could bring important informations to our comprehension of the PXE disease.