Echocardiographic assessment of right ventricular function and right ventriculoarterial coupling in tricuspid regurgitation.

Echocardiographic evaluation of the cardiopulmonary unit is difficult in case of tricuspid regurgitation (TR) and combined echocardiographic parameters could be useful. This study aimed to assess the variation of simple and combined echocardiographic parameters analysing the cardiopulmonary unit according to the severity of TR. TR was graded according to Hahn's classification in 179 patients. Classical morphological, function and load parameters analysing right ventricle were assessed. Combined parameters of function and load; morphology and load; and morphology-load-function index were calculated. We used ROC curve analysis to analyze the diagnostic value of echocardiographic parameters to predict potential high or low surgical risk of mortality according to TRISCORE in 82 patients. Simple parameters were significatively different among groups with a nonlinear progression between the 5 levels of severity of TR. Combined parameters were also significatively different among groups. Among them, myomechanical index (MMI = RV-RA mean pressure gradient x RVFWS/indexed RAED area × 10-2) and morphology-load-function index (MLF = RVED length/area x TR TVI x RVFWS) had a linear progression between the 5 groups and had the best predictive value for TRISCORE high and low risk. Combined parameters are relevant to evaluate cardiopulmonary unit in patients with various degrees of TR, especially when combining morphology, function, and load parameters, and are potentially interesting in their prognostic assessment, as shown by the good predictive value for TRISCORE risk.

Advanced myocardial deformation echocardiography for evaluation of the athlete's heart: Functional and mechanistic analysis.

BACKGROUND: Assessment of the athlete's heart is challenging because of a phenotypic overlap between reactive physiological adaptation and pathological remodelling. The potential value of myocardial deformation remains controversial in identifying early cardiomyopathy. AIM: To identify the echocardiographic phenotype of athletes using advanced two-dimensional speckle tracking imaging, and to define predictive factors of subtle left ventricular systolic dysfunction. METHODS: In total, 191 healthy male athletes who underwent a preparticipation medical evaluation at Nancy University Hospital between 2013 and 2020 were included. Clinical and echocardiographic data were compared with 161 healthy male subjects from the STANISLAS cohort. Borderline global longitudinal strain value was defined as<17.5%. RESULTS: Athletes demonstrated lower left ventricular ejection fraction (57.9±5.3% vs. 62.6±6.4%; P<0.01) and lower global longitudinal strain (17.5±2.2% vs. 21.1±2.1%; P<0.01). No significant differences were found between athletes with and without a borderline global longitudinal strain value regarding clinical characteristics, structural echocardiographic features and exercise capacity. A borderline global longitudinal strain value was associated with a lower endocardial global longitudinal strain (18.8±1.2% vs. 22.7±1.9%; P=0.02), a lower epicardial global longitudinal strain (14.0±1.1% vs. 16.6±1.2%; P<0.01) and a higher endocardial/epicardial global longitudinal strain ratio (1.36±0.07 vs. 1.32±0.06; P<0.01). No significant difference was found regarding mechanical dispersion (P=0.46). CONCLUSIONS: Borderline global longitudinal strain value in athletes does not appear to be related to structural remodelling, mechanical dispersion or exercise capacity. The athlete's heart is characterized by a specific myocardial deformation pattern with a more pronounced epicardial layer strain impairment.

Factors Associated with Myocardial Uptake on Oncologic Somatostatin PET Investigations and Differentiation from Myocardial Uptake of Acute Myocarditis.

Myocardial somatostatin PET uptake is observed not only in most patients with acute myocarditis (AM) but also in some oncology patients referred for routine somatostatin PET. This raises concerns about the specificity of somatostatin PET for detecting myocarditis. The current study aims to identify factors associated with the detection of myocardial uptake on somatostatin PET scans recorded for oncology indications and differential PET criteria that characterize myocardial uptake in AM patients. Methods: We analyzed factors associated with the detection of myocardial [68Ga]Ga-DOTATOC uptake in 508 [68Ga]Ga-DOTATOC PET scans from 178 patients, performed for confirmed or suspected oncologic disease (Onc-PET) and PET criteria that could differentiate myocardial [68Ga]Ga-DOTATOC uptake in 31 patients with MRI-ascertained AM (AM-PET) from that in the Onc-PET group. Results: Significant myocardial uptake was detected in 137 (26.9%) Onc-PET scans and was independently associated with somatostatin analog treatment (exp(ß), 0.805; 95% CI, 0.728-0.890; P < 0.001) and age (exp(ß), 1.005; 95% CI, 1.001-1.009; P = 0.012). A comparable model was selected for predicting the myocardial-to-blood SUVmax ratio using somatostatin analog treatment (P < 0.001) and history of coronary artery disease (P = 0.022). Myocardial uptake was detected in 12.9% (25/193) of Onc-PET scans from patients treated with somatostatin analogs but in 43.4% (59/136) of untreated patients over the median age of 64 y. Myocardial uptake was apparent in all 31 AM-PET scans, with volume and intensity of uptake dramatically higher than in the 137 Onc-PET scans showing myocardial uptake. A myocardial-to-blood SUVmax ratio threshold of 2.20 provided a sensitivity of 87% (27/31) and a specificity of 88% (44/50) for differentiating myocardial uptake between the AM-PET group and an Onc-PET group restricted to patients with clinical characteristics comparable to those of patients in the AM-PET group (≤64 y of age, no coronary artery disease history, and no somatostatin agonists). A myocardial uptake volume threshold of 18 cm3 provided comparable diagnostic accuracy (sensitivity, 84% [26/31]; specificity, 94% [47/50]). Conclusion: Myocardial uptake was detected in 26.9% of somatostatin PET scans recorded for oncology indications. This rate was decreased by somatostatin analog treatments and increased in older individuals. However, somatostatin PET scans, analyzed with the quantitative criterion of uptake intensity or volume, are able to identify AM and to differentiate it from myocardial uptake of other origins.

Advent of severe mitral regurgitation in surgical ventricular restoration and ventricular septal rupture repair.

BACKGROUND: Per-procedural severe mitral regurgitation is a rare complication in concomitant surgical ventricular restoration and postinfarction ventricular septal rupture repair. It is challenging to discover the underlying etiology and adopt an appropriate strategy, in particular, in a high-risk patient. CASE PRESENTATION: Semi-emergent surgical ventricular restoration combined with ventricular septal rupture closure and coronary artery bypassing was performed in a 67-year-old male patient. Severe mitral regurgitation was detected after the weaning of cardiopulmonary bypass. Two key questions arose in the management of this condition: did the regurgitation exist previously and was dissimulated by significant left-to-right shunt, or it occurred secondarily to the Dor procedure? Which was the better management strategy, chordal-sparing mitral valve replacement or mitral plasty? We believed that severe mitral regurgitation was under-estimated pre-operatively and we performed an downsizing annuloplasty to treat mitral regurgitation. The outcomes were promising and the patient did well in follow-up. CONCLUSIONS: Our case brought out an open discussion on the etiology and therapeutic strategies of this complicated condition.

Myocardial deformation in malignant mitral valve prolapse: A shifting paradigm to dynamic mitral valve-ventricular interactions.

Objectives: This study sought to assess the value of myocardial deformation using strain echocardiography in patients with mitral valve prolapse (MVP) and severe ventricular arrhythmia and to evaluate its impact on rhythmic risk stratification. Background: MVP is a common valvular affection with an overly benign course. Unpredictably, selected patients will present severe ventricular arrhythmia. Methods: Patients with MVP as the only cause of aborted SCD (MVP-aSCD: ventricular fibrillation and monomorphic and polymorphic ventricular tachycardia) with no other obvious reversible cause were identified. Nonconsecutive patients referred for the echocardiographic evaluation of MVP were enrolled as a control cohort and dichotomized according to the presence or absence of premature ventricular contractions (MVP-PVC or MVP-No PVC, respectively). All patients had a comprehensive strain assessment of mechanical dispersion (MD), postsystolic shortening, and postsystolic index (PSI). Results: A total of 260 patients were enrolled (20 MVP-aSCD, 54 MVP-PVC, and 186 MVP-No PVC). Deformation pattern discrepancies were observed with a higher PSI value in MVP-aSCD than that in MVP-PVC (4.6 ± 2.0 vs. 2.9 ± 3.7, p = 0.014) and a higher MD value than that in MVP-No PVC (46.0 ± 13.0 vs. 36.4 ± 10.8, p = 0.002). In addition, PSI and MD increased the prediction of severe ventricular arrhythmia on top of classical risk factors in MVP. Net reclassification improvement was 61% (p = 0.008) for PSI and 71% (p = 0.001) for MD. Conclusions: In MVP, myocardial deformation analysis with strain echocardiography identified specific contraction patterns with postsystolic shortening leading to increased values of PSI and MD, translating the importance of mitral valve-myocardial interactions in the arrhythmogenesis of severe ventricular arrhythmia. Strain echocardiography may provide important implications for rhythmic risk stratification in MVP.

Machine Learning-Based Phenogrouping in MVP Identifies Profiles Associated With Myocardial Fibrosis and Cardiovascular Events.

BACKGROUND: Structural changes and myocardial fibrosis quantification by cardiac imaging have become increasingly important to predict cardiovascular events in patients with mitral valve prolapse (MVP). In this setting, it is likely that an unsupervised approach using machine learning may improve their risk assessment. OBJECTIVES: This study used machine learning to improve the risk assessment of patients with MVP by identifying echocardiographic phenotypes and their respective association with myocardial fibrosis and prognosis. METHODS: Clusters were constructed using echocardiographic variables in a bicentric cohort of patients with MVP (n = 429, age 54 ± 15 years) and subsequently investigated for their association with myocardial fibrosis (assessed by cardiac magnetic resonance) and cardiovascular outcomes. RESULTS: Mitral regurgitation (MR) was severe in 195 (45%) patients. Four clusters were identified: cluster 1 comprised no remodeling with mainly mild MR, cluster 2 was a transitional cluster, cluster 3 included significant left ventricular (LV) and left atrial (LA) remodeling with severe MR, and cluster 4 included remodeling with a drop in LV systolic strain. Clusters 3 and 4 featured more myocardial fibrosis than clusters 1 and 2 (P < 0.0001) and were associated with higher rates of cardiovascular events. Cluster analysis significantly improved diagnostic accuracy over conventional analysis. The decision tree identified the severity of MR along with LV systolic strain <21% and indexed LA volume >42 mL/m2 as the 3 most relevant variables to correctly classify participants into 1 of the echocardiographic profiles. CONCLUSIONS: Clustering enabled the identification of 4 clusters with distinct echocardiographic LV and LA remodeling profiles associated with myocardial fibrosis and clinical outcomes. Our findings suggest that a simple algorithm based on only 3 key variables (severity of MR, LV systolic strain, and indexed LA volume) may help risk stratification and decision making in patients with MVP. (Genetic and Phenotypic Characteristics of Mitral Valve Prolapse, NCT03884426; Myocardial Characterization of Arrhythmogenic Mitral Valve Prolapse [MVP STAMP], NCT02879825).

Aortic regurgitation: multimodal assessment of quantification and impact.

BACKGROUND: The assessment of severity of aortic regurgitation (AR) by transthoracic echocardiography (TTE) remains challenging in routine practice. Contemporary guidelines recommend cardiovascular magnetic resonance imaging (CMR) in patients with significant disease and suboptimal TTE images. The objective of this study was to assess the role of CMR in the evaluation of severity of AR and to compare both modalities in the quantification of regurgitation and left ventricular volumes. METHODS: Fifty consecutive patients who had isolated chronic AR and who underwent TTE and CMR within an interval of less than three months between May 2009 and June 2020 were included. The main indication for CMR was difficulties in quantifying AR, either because of lack of multiparametric analysis (only one method possible) or because of discrepancies in the different methods by TTE. RESULTS: In 25 patients, precise grading of AR was not possible by echocardiography. Among them, CMR finally detected seven patients with mild AR, 11 with moderate AR and seven with severe AR. For the 25 patients who had AR quantification by TTE, there was concordance between TTE and CMR in only seven patients (28%), and the AR was re-graded by CMR in 18 patients, including eight patients with severe AR by TTE and moderate AR by CMR. The concordance between TTE and CMR was weakly significant (intraclass correlation coefficient = 0.39, 95% confidence interval: 0.003-0.67, p = 0.02). There was a moderate correlation between left ventricular volumes measured by TTE and by CMR (left ventricular end-diastolic volume: r = 0.57; p = 0.01; left ventricular end-systolic volume: r = 0.47, p = 0.01) but regurgitant volumes were not correlated (r = 0.04; p = 0.8). No TTE parameter of quantification was correlated with regurgitant volume measured by CMR. CONCLUSIONS: The concordance of AR quantification by CMR and TTE was weak. CMR re-graded some patients with severe AR by TTE into moderate AR. This should motivate practitioners to systematically assess all significant AR by CMR in order to improve quantification and optimise clinical management.

Detection of acute myocarditis by ECG-triggered PET imaging of somatostatin receptors compared to cardiac magnetic resonance: preliminary results.

Somatostatin receptors are overexpressed by inflammatory cells but not by cardiac cells, under normal conditions. This study assesses the detection of acute myocarditis by the ECG-triggered digital-PET imaging of somatostatin receptors (68Ga-DOTATOC-PET), as compared to Cardiac Magnetic Resonance (CMR) imaging, which is the reference diagnostic method in this setting. METHODS: Fourteen CMR-defined acute myocarditis patients had a first 15-minutes ECG-triggered 68Ga-DOTATOC PET recording, 4.4 ± 3.0 days from peak troponin, and 10 had a second 4.3 ± 0.3 months later. Myocardial/blood SUVmax ratio was analyzed relative to the normal upper limit of 2.18, which had been previously determined from oncology 68Ga-DOTATOC-PET recordings of patients with a similar age range as the myocarditis patients. RESULTS: An increased myocardial 68Ga-DOTATOC uptake relative to blood activity was invariably observed during the acute phase. SUVmax ratio exceeded 2.18 in all patients during the acute phase but also in 3/10 patients at 4-months, at a time when there were no more signs of active inflammation on CMR. A residual myocardial 68Ga-DOTATOC uptake was still observed on all gated-PET cine loops at 4-months. CONCLUSION: These preliminary results suggest that 68Ga-DOTATOC ECG-triggered digital-PET may be as sensitive as CMR at detecting myocarditis during the acute phase and more sensitive at later stages.

Staphylococcus aureus endocarditis: Identifying prognostic factors using a method derived from morbidity and mortality conferences.

Objectives: Lethality of Staphylococcus aureus (Sa) infective endocarditis (IE) is high and might be due to yet unidentified prognostic factors. The aim of this study was to search for new potential prognostic factors and assess their prognostic value in SaIE. Materials and methods: We used a two-step exploratory approach. First, using a qualitative approach derived from mortality and morbidity conferences, we conducted a review of the medical records of 30 patients with SaIE (15 deceased and 15 survivors), randomly extracted from an IE cohort database (NCT03295045), to detect new factors of possible prognostic interest. Second, we collected quantitative data for these factors in the entire set of SaIE patients and used multivariate Cox models to estimate their prognostic value. Results: A total of 134 patients with modified Duke definite SaIE were included, 64 of whom died during follow-up. Of the 56 candidate prognostic factors identified at the first step, 3 had a significant prognostic value in multivariate analysis: the prior use of non-steroidal anti-inflammatory drugs [aHR 3.60, 95% CI (1.59-8.15), p = 0.002]; the non-performance of valve surgery when indicated [aHR 1.85, 95% CI (1.01-3.39), p = 0.046]; and the decrease of vegetation size on antibiotic treatment [aHR 0.34, 95% CI (0.12-0.97), p = 0.044]. Conclusion: We identified three potential SaIE prognostic factors. These results, if externally validated, might eventually help improve the management of patients with SaIE.

Characteristics of patients with infective endocarditis and no underlying cardiac conditions.

BACKGROUND: Infective endocarditis (IE) typically occurs in patients with underlying cardiac conditions (UCC). Little is known about IE in patients without UCC. We aimed to describe the clinical, microbiological and imaging characteristics, management, and in-hospital mortality of IE patients without UCC. METHODS: We analysed the data of patients with definite IE included in an observatory between 1st January 2009 and 31st December 2019. We described patients without UCC compared to those with UCC. RESULTS: Of 1502 IE patients, 475 (31.6%) had no UCC. They were younger (median 64.0 [19.0-101.0] vs. 70.0 [18.0-104.0] years, p < .001), more often on chronic haemodialysis (5.5% vs. 2.7%, p = .008), and had more often malignancy (22.5% vs. 17.3%, p = .017), immune deficiency (10.3% vs. 6.4%, p = .008), and an indwelling central venous line (14.5% vs. 7.0%, p < .001). They more often developed cerebral complications (34.7% vs. 27.5%, p = .004) and extracerebral embolism (48.6% vs. 36.1%, p < .001). Causative microorganisms were less often coagulase negative staphylococci (5.9% vs. 10.8%, p = .002) or enterococci (10.3% vs. 15.0%, p = .014) and more often group D streptococci (14.1% vs. 10.0%, p = .020). Vegetations were more common (92.8% vs. 77.0%, p < .001) and larger (14.0 [1.0-87.0], vs. 12.0 [0.5-60.0] mm, p = .002). They had more valve perforation or valve regurgitation (67.4% vs. 53.0%, p < .001) and underwent valve surgery more often (53.5% vs. 36.3%, p < .001). In-hospital mortality did not significantly differ between groups. CONCLUSION: Patients with IE and no UCC were younger than those with UCC, had specific comorbidities and portals of entry, and a more severe disease course.

Long-Lasting Myocardial and Skeletal Muscle Damage Evidenced by Serial CMR During the First Year in COVID-19 Patients From the First Wave.

Introduction: This observational CMR study aims to characterize left-ventricular (LV) damage, which may be specifically attributed to COVID-19 and is distant in time from the acute phase, through serial CMR performed during the first year in patients with no prior cardiac disease. Methods: This study included consecutive patients without any prior history of cardiac disease but with a peak troponin-Ic > 50 ng/ml at the time of the first COVID-wave. All had a CMR in the first months after the acute phase, and some had an additional CMR at the end of the first year to monitor LV function, remodeling, and abnormalities evocative of myositis and myocarditis - i.e., increased T1/T2 relaxation times, increased extracellular volume (ECV), and delayed contrast enhancement. Results: Nineteen consecutively admitted COVID-19 patients (17 men, median age 66 [57-71] years) were included. Eight (42%) had hypertension, six (32%) were obese, and 16 (84%) had suffered an acute respiratory distress syndrome. The 1st CMR, recorded at a median 3.2 [interquartile range: 2.6-3.9] months from the troponin peak, showed (1) LV concentric remodeling in 12 patients (63%), (2) myocardial tissue abnormalities in 11 (58%), including 9 increased myocardial ECVs, and (3) 14 (74%) increased ECVs from shoulder skeletal muscles. The 2nd CMR, obtained at 11.1 [11.0-11.7] months from the troponin peak in 13 patients, showed unchanged LV function and remodeling but a return to normal or below the normal range for all ECVs of the myocardium and skeletal muscles. Conclusion: Many patients with no history of cardiac disease but for whom an increase in blood troponin-Ic ascertained COVID-19 induced myocardial damage exhibited signs of persistent extracellular edema at a median 3-months from the troponin peak, affecting the myocardium and skeletal muscles, which resolved within a one-year time frame. Associations with long-COVID symptoms need to be investigated on a larger scale now. Clinical Trial Registration: NCT04753762 on the ClinicalTrials.gov site.

Arterial and Cardiac Remodeling Associated With Extra Weight Gain in an Isolated Abdominal Obesity Cohort.

Introduction: This study aims to assess the changes in cardiovascular remodeling attributable to bodyweight gain in a middle-aged abdominal obesity cohort. A remodeling worsening might explain the increase in cardiovascular risk associated with a dynamic of weight gain. Methods: Seventy-five middle-aged subjects (56 ± 5 years, 38 women) with abdominal obesity and no known cardiovascular disease underwent MRI-based examinations at baseline and at a 6.1 ± 1.2-year follow-up to monitor cardiovascular remodeling and hemodynamic variables, most notably the effective arterial elastance (Ea). Ea is a proxy of the arterial load that must be overcome during left ventricular (LV) ejection, with increased EA resulting in concentric LV remodeling. Results: Sixteen obese subjects had significant weight gain (>7%) during follow-up (WG+), whereas the 59 other individuals did not (WG-). WG+ and WG- exhibited significant differences in the baseline to follow-up evolutions of several hemodynamic parameters, notably diastolic and mean blood pressures (for mean blood pressure, WG+: +9.3 ± 10.9 mmHg vs. WG-: +1.7 ± 11.8 mmHg, p = 0.022), heart rate (WG+: +0.6 ± 9.4 min-1 vs. -8.9 ± 11.5 min-1, p = 0.003), LV concentric remodeling index (WG: +0.08 ± 0.16 g.mL-1 vs. WG-: -0.02 ± 0.13 g.mL-1, p = 0.018) and Ea (WG+: +0.20 ± 0.28 mL mmHg-1 vs. WG-: +0.01 ± 0.30 mL mmHg-1, p = 0.021). The evolution of the LV concentric remodeling index and Ea were also strongly correlated in the overall obese population (p < 0.001, R2 = 0.31). Conclusions: A weight gain dynamic is accompanied by increases in arterial load and load-related concentric LV remodeling in an isolated abdominal obesity cohort. This remodeling could have a significant impact on cardiovascular risk.

Adherence to diagnostic and therapeutic practice guidelines for suspected cardiac implantable electronic device infections.

BACKGROUND: Despite guidelines describing the optimal diagnostic and therapeutic procedures for patients with suspected cardiac implantable electronic device (CIED) infections, their management is often challenging. AIMS: To describe our diagnostic and therapeutic practices for suspected CIED infection, and to compare them with European Heart Rhythm Association (EHRA) guidelines. METHODS: Patients hospitalized in the tertiary care Nancy University Hospital for suspected CIED infection from 2014 to 2019 were included retrospectively. We applied the EHRA classification of CIED infection, and compared diagnostic and therapeutic management with the EHRA guidelines. RESULTS: Among 184 patients (mean age 72.3±12.4 years), 137 had a proven infection of the lead (by transthoracic echocardiography/transoesophageal echocardiography, 18F-fluorodesoxyglucose positron emission tomography/computed tomography or positive culture of the lead) or an isolated pocket infection without proof of lead infection, and 47 had no proof of CIED infection. According to the EHRA classification, CIED infection was considered as definite in 145 patients and possible in 31 and was excluded in eight patients. Regarding recommended diagnostic procedures, blood cultures were performed in 90.8%, transthoracic echocardiography in 97.8%, transoesophageal echocardiography in 85.9%, 18F-fluorodesoxyglucose positron emission tomography/computed tomography in 50.5% and imaging for embolisms in 78.3% of the patients. Compared with therapeutic recommendations for the 145 cases of definite CIED infection, device removal was performed in 96 patients (66.2%) and antibiotic therapy was prescribed in 130 (89.7%), with a duration equal to or longer than that recommended in 105 (72.4%) of the patients. CONCLUSION: This study underlines the difficulties in following theoretical guidelines in daily practice, where both technical and human considerations interfere with their strict appliance.

Treatment with Delta-9-tetrahydrocannabinol/cannabidiol in Multiple Sclerosis: Influence on the Autonomy Profile according to the International Classification of Functioning, Disability and Health.

Multiple sclerosis (MS) is the most common cause of non-traumatic neurological disability in young adults. It has effects at different levels: physical, emotional, psychological, cognitive and social, with a great variety of signs and symptoms. In particular, spasticity contributes to reducing the motor performance of patients with MS, causing pain, reduction in distance walked and limitations in social life. We present the case of a 39-year-old woman with MS. She was treated with delta-9-tetrahydrocannabinol/cannabidiol and the outcome was assessed with the International Classification of Functioning Disability and Health core set framework. LEARNING POINTS: The clinical presentation of multiple sclerosis (MS) is heterogeneous but very often lower limb spasticity leads to severe disability.The use of nabiximols improved spasticity control and motor performance in walking, and also had a larger effect in improving activity and participation in personal relationships.Appropriate assessment of MS cases, through the ICF framework, may demonstrate further effects of nabiximols on patient capacity and performance.

A Giant Abdominal Aortic Aneurysm Revealing a Marfan Syndrome With a New FBN1 Mutation.

Marfan syndrome is a connective tissue disease that rarely presents first with peripheral aortic aneurysms. We highlight the case of a young man with Marfan syndrome presenting with an abdominal aortic aneurysm due to a heterozygous fibrillin-1 gene mutation.

Neurofibromatosis Type 1 with Highly Active Relapsing-Remitting Multiple Sclerosis (RRMS).

Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disease which confers an increased risk of malignant tumour development. Relapsing remitting multiple sclerosis (RRMS) is an inflammatory demyelinating disease of the central nervous system. The coexistence of multiple sclerosis and NF1 is rare but has been reported. Here, we describe the case of a 31-year-old man with NF1 and subacute walking problems with proximal pain in the lower limbs who was successfully treated with natalizumab. LEARNING POINTS: The coexistence of multiple sclerosis (MS) and neurofibromatosis type 1 (NF1) is very rare but has been described in the literature.Follow-up of patients with NF1 is important as the early detection and management of MS can prevent further disability.Appropriate treatment and physical therapy can improve the patient's activity and social life.

Replacement Myocardial Fibrosis in Patients With Mitral Valve Prolapse: Relation to Mitral Regurgitation, Ventricular Remodeling, and Arrhythmia.

BACKGROUND: Mitral valve prolapse (MVP) is a frequent disease that can be complicated by mitral regurgitation (MR), heart failure, arterial embolism, rhythm disorders, and death. Left ventricular (LV) replacement myocardial fibrosis, a marker of maladaptive remodeling, has been described in patients with MVP, but the implications of this finding remain scarcely explored. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance in patients with MVP. METHODS: Four hundred patients (53±15 years of age, 55% male) with MVP (trace to severe MR by echocardiography) from 2 centers, who underwent a comprehensive echocardiography and LGE cardiac magnetic resonance, were included. Correlates of replacement myocardial fibrosis (LGE+), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events (cardiac death, heart failure, new-onset atrial fibrillation, arterial embolism, and life-threatening ventricular arrhythmia). RESULTS: Replacement myocardial fibrosis (LGE+) was observed in 110 patients (28%; 91 with myocardial wall including 71 with basal inferolateral wall, 29 with papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate MR, and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45% versus 26%, P<0.0001). In trace-mild MR, despite the absence of significant volume overload, abnormal LV dilatation was observed in 16% of patients and ventricular arrhythmia in 25%. Correlates of LGE+ in multivariable analysis were LV mass (odds ratio, 1.01 [95% CI, 1.002-1.017], P=0.009) and moderate-severe MR (odds ratio, 2.28 [95% CI, 1.21-4.31], P=0.011). LGE+ was associated with worse 4-year cardiovascular event-free survival (49.6±11.7 in LGE+ versus 73.3±6.5% in LGE-, P<0.0001). In a stepwise multivariable Cox model, MR volume and LGE+ (hazard ratio, 2.6 [1.4-4.9], P=0.002) were associated with poor outcome. CONCLUSIONS: LV replacement myocardial fibrosis is frequent in patients with MVP; is associated with mitral valve apparatus alteration, more dilated LV, MR grade, and ventricular arrhythmia; and is independently associated with cardiovascular events. These findings suggest an MVP-related myocardial disease. Last, cardiac magnetic resonance provides additional information to echocardiography in MVP.

Relationship Between Left Ventricular Ejection Fraction Variation and Systemic Vascular Resistance: A Prospective Cardiovascular Magnetic Resonance Study.

Introduction: This cardiovascular magnetic resonance (CMR) study aims to determine whether changes in systemic vascular resistance (SVR), obtained from CMR flow sequences, might explain the significant long-term changes in left ventricular (LV) ejection fraction (EF) observed in subjects with no cardiac disease history. Methods: Cohort subjects without any known cardiac disease but with high rates of hypertension and obesity, underwent CMR with phase-contrast sequences both at baseline and at a median follow-up of 5.2 years. Longitudinal changes in EF were analyzed for any concomitant changes in blood pressure and vascular function, notably the indexed SVR given by the formula: mean brachial blood pressure / cardiac output x body surface area. Results: A total of 118 subjects (53 ± 12 years, 52% women) were included, 26% had hypertension, and 52% were obese. Eighteen (15%) had significant EF variations between baseline and follow-up (7 increased EF and 11 decreased EF). Longitudinal changes in EF were inversely related to concomitant changes in mean and diastolic blood pressures (p = 0.030 and p = 0.027, respectively) and much more significantly to SVR (p < 0.001). On average, these SVR changes were -8.08 ± 9.21 and +8.14 ± 8.28 mmHg.min.m2.L-1, respectively, in subjects with significant increases and decreases in EF, and 3.32 ± 7.53 mmHg.min.m2.L-1 in subjects with a stable EF (overall p < 0.001). Conclusions: Significant EF variations are not uncommon during the long-term CMR follow-up of populations with no evident health issues except for uncomplicated hypertension and obesity. However, most of these variations are linked to SVR changes and may therefore be unrelated to any intrinsic change in LV contractility. This underscores the benefits of specifically assessing LV afterload when EF is monitored in populations at risk of vascular dysfunction. Clinical Trial Registration: ClinicalTrials.gov, identifier: NCT01716819 and NCT02430805.