Myocardial fibrosis by late gadolinium enhancement cardiovascular magnetic resonance in myotonic muscular dystrophy type 1: highly prevalent but not associated with surface conduction abnormality.

BACKGROUND: Conduction disease and arrhythmias represent a major cause of mortality in myotonic muscular dystrophy type 1 (MMD1). Permanent pacemaker (PPM) implantation is the cornerstone of therapy to reduce cardiovascular mortality in MMD1. Cardiovascular magnetic resonance (CMR) studies demonstrate a high prevalence of myocardial fibrosis in MMD1, however the association between CMR myocardial fibrosis with late gadolinium enhancement (CMR-LGE) and surface conduction abnormality is not well established in MMD1. We investigated whether myocardial fibrosis by CMR-LGE is associated with surface conduction abnormalities meeting criteria for PPM implantation according to current guidelines in a cohort of patients with genetically confirmed MMD1. METHODS: Patients with genetically confirmed MMD1 were retrospectively evaluated. 12-lead electrocardiography (ECG) performed within 6 months of CMR was necessary for inclusion. The severity and extent of MMD1 was quantified using a validated Muscular Impairment Rating Scale (MIRS). Based on current guidelines for device-based therapy of cardiac rhythm abnormalities, we defined surface conduction abnormality as the presence of ECG alterations meeting criteria for PPM implant (class I or II indications): PR interval > 200 ms (type I atrioventricular (AV) block) and/or mono or bifascicular block (QRS > 120 ms), or evidence of advanced AV block. Balanced steady-state free precession sequences (bSSFP) were used for assessment of left ventricular (LV) volumes and ejection fraction. MOdified Look-Locker Inversion Recovery (MOLLI) acquisition schemes were used to acquire T1 maps. Patients' charts were reviewed up to 12 months post-CMR for occurrence of PPM implantation. RESULTS: Fifty-two patients (38% male, 41 ± 14 years) were included. Overall, 31 (60%) patients had a surface conduction abnormality and 22 (42%) demonstrated midwall myocardial fibrosis by CMR-LGE. After a median of 57 days from CMR exam, 15 patients (29%) underwent PPM implantation. Subjects with vs. without surface conduction abnormality had significantly longer disease length (15.5 vs. 7.8 years, p = 0.015) and higher disease severity on the MIRS scale (p = 0.041). High prevalence of myocardial fibrosis by CMR-LGE was detected in subjects with and without surface conduction abnormality with no significant difference between the two cohorts (42% vs. 43%, p = 0.999). By multivariate logistic regression analysis, disease length was the only independent variable associated with surface conduction abnormality (OR 1.071, 95%CI 1.003-1.144, p = 0.040); while CMR-LGE was not associated with conduction abnormality (ρ = - 0.009, p = 0.949). CONCLUSIONS: Myocardial fibrosis by CMR-LGE is highly prevalent in MMD1 but not related to surface conduction abnormality meeting current guideline criteria for PPM implantation .

Laser treatment for facial acne scars: A review.

BACKGROUND AND OBJECTIVES: Acne scarring is a widely prevalent condition that can have a negative impact on a patient's quality of life and is often worsened by aging. A number of options are available for the treatment of acne scarring, including retinoids, microdermabrasion, dermal fillers, and surgical techniques such as subcision. The aim of this review is to evaluate the different laser modalities that have been used in peer-reviewed clinical studies for treatment of atrophic acne scars, and summarize current clinical approaches. MATERIALS AND METHODS: A Medline search spanning from 1990 to 2016 was performed on acne scarring. Search terms included "atrophic acne scars," "ablative'', "nonablative," "fractional," "nonfractional," "neodymium," "alexandrite," "pulsed dye" lasers, and results are summarized. RESULTS: Various types of lasers have been evaluated for the treatment of atrophic acne scars. While they are efficacious overall, they differ in terms of side effects and clinical outcomes, depending on patients skin and acne scar type. A new emerging trend is to combine lasers with other energy-based devices and/or topicals. CONCLUSION: Evaluation of the literature examining acne scar treatment with lasers, revealed that clinical outcomes are dependent on various patient factors, including atrophic acne scar subtype, patient skin type, treatment modality, and side-effect profile.

Endoscopic closure of tracheoesophageal fistula for tuberculosis with an over-the-scope-clip.

Surgical management has been the main approach for enteral fistulae. This approach is usually complex due to comorbidities, a wasted nutritional state and anatomical difficulties related to prior multiple interventions. Therefore, endoscopic methods such as clips, self-expanding metal stent (SEMS) and recently, the over scope clip (OTSC®) are increasing in popularity and use. Herein, we present the case of a patient with a HIV infection who was admitted due to respiratory symptoms. Radiological and microbiological studies documented a tracheoesophageal fistula due to tuberculosis (TB) and cytomegalovirus (CMV) infection. Therefore, an esophageal fully-covered stent was placed, which migrated into the stomach. The thoracic surgeons considered an esophagectomy with gastric ascent and muscle patch in the trachea. However, due to his poor nutritional status and comorbidity, an OTSC was placed to treat the fistulae. The patient also received medical treatment with anti-tuberculotics and anti-retrovirals.

The role of stress cardiac magnetic resonance in women.

Coronary artery disease (CAD) is the leading cause of death in women. Nevertheless, extensive evidence demonstrates under-diagnosis and under-treatment of women for suspected or known ischemic heart disease (IHD). Stress cardiac magnetic resonance (CMR) is becoming readily available and offers significant advantages over other stress imaging modalities. The high spatial and temporal resolution of CMR provides the unique ability to identify subendocardial ischemia, viability, and the presence of microvascular disease. Furthermore, CMR is free from ionizing radiation, and image quality is not compromised by attenuation artifacts or patient size. Over the past two decades, evidence-based data have demonstrated the high diagnostic and prognostic performance of stress CMR in the context of IHD, often superior to other stress imaging techniques. Importantly, ad hoc studies confirmed these results in women with known or suspected IHD. Stress CMR warrants consideration as the modality of choice for women requiring an imaging test for ischemia given its strong evidence base, superior test characteristics, comprehensive nature, and unique ability to characterize both epicardial and microvascular disease.

Multimodality Imaging for Selecting Candidates for CRT: Do We Have a Single Alley to Increase Responders?

Cardiac resynchronization therapy has evolved in recent years to provide a reduction of morbidity and mortality for many patients with heart failure. Its application and optimization is an evolving field and its use requires a multidisciplinary approach for patient and device selection, technical preprocedural planning, and optimization. While echocardiography has always been considered the first line for the evaluation of patients, additional imaging techniques have gained increasing evidence in recent years. Today different details about heart anatomy, function, dissynchrony can be investigated by magnetic resonance, cardiac computed tomography, nuclear imaging, and more, with the aim of obtaining clues to reach a maximal response from the electrical therapy. The purpose of this review is to provide a practical analysis of the single and combined use of different imaging techniques in the preoperative and perioperative phases of cardiac resynchronization therapy, underlining their main advantages, limitations, and information provided.

The role of coronary computed tomography angiography in low- and intermediate-risk patients with chest pain: a paradigm shift in modern cardiology.

Chest pain is a common complaint among patients presenting to primary care physicians. The management of chest pain secondary to coronary artery disease is rapidly changing as new evidence increase our knowledge of this complex clinical problem. The 2021 multisociety guidelines developed by the American College of Cardiology and the American Heart Association along with other organizations and imaging societies represent the first international guidelines for the evaluation and diagnosis of patients with acute or stable chest pain. This review will discuss in details the evaluation of low- and intermediate risk subjects presenting with acute and stable chest pain both in the emergency and office settings, providing a practical approach, supported by contemporary evidence, for the management of this important clinical problem leveraging on the central role played by coronary computed tomography angiography as documented by current clinical guidelines and available scientific literature.

[Mitral mass and severe mitral regurgitation in a patient with heart failure: differential diagnosis]. / Massa mitralica ed insufficienza di grado severo in paziente con scompenso cardiaco: diagnosi differenziale.

We describe the case of an 84-year-old woman, hospitalized for acute heart failure, presenting with an intracardiac mass detected on echocardiography. The differential diagnosis and the usefulness of multimodality imaging are discussed.

Serum uric acid and outcomes in patients with chronic heart failure through the whole spectrum of ejection fraction phenotypes: Analysis of the ESC-EORP Heart Failure Long-Term (HF LT) Registry.

BACKGROUND: Retrospective analyses of clinical trials indicate that elevated serum uric acid (sUA) predicts poor outcome in heart failure (HF). Uric acid can contribute to inflammation and microvascular dysfunction, which may differently affect different left ventricular ejection fraction (LVEF) phenotypes. However, role of sUA across LVEF phenotypes is unknown. OBJECTIVES: We investigated sUA association with outcome in a prospective cohort of HF patients stratified according to LVEF. METHODS: Through the Heart Failure Long-Term Registry of the European Society of Cardiology (ESC-EORP-HF-LT), 4,438 outpatients were identified and classified into: reduced (<40% HFrEF), mid-range (40-49% HFmrEF), and preserved (≥50% HFpEF) LVEF. Endpoints were the composite of cardiovascular death/HF hospitalization, and individual components. RESULTS: Median sUA was 6.72 (IQ:5.48-8.20) mg/dl in HFrEF, 6.41 (5.02-7.77) in HFmrEF, and 6.30 (5.20-7.70) in HFpEF. At a median 372-day follow-up, the composite endpoint occurred in 648 (13.1%) patients, with 176 (3.6%) deaths and 538 (10.9%) HF hospitalizations. Compared with lowest sUA quartile (Q), Q-III and Q-IV were significantly associated with the composite endpoint (adjusted HR 1.68: 95% CI 1.11-2.54; 2.46: 95% CI 1.66-3.64, respectively). By univariable analyses, HFrEF and HFmrEF patients in Q-III and Q-IV, and HFpEF patients in Q-IV, showed increased risk for the composite endpoint (P<0.05 for all); after model-adjustment, significant association of sUA with outcome persisted among HFrEF in Q-IV, and HFpEF in Q-III-IV. CONCLUSIONS: In a large, contemporary-treated cohort of HF outpatients, sUA is an independent prognosticator of adverse outcome, which can be appreciated in HErEF and HFpEF patients.

Prevalence of Coronary Artery Calcification on Pre-Atrial Fibrillation Ablation CT Pulmonary Venograms and its Impact on Selection for Statin Therapy.

Atherosclerotic cardiovascular disease (ASCVD) shares many risk factors with atrial fibrillation (AF). Obtaining computed tomography images of the pulmonary veins (CTPV) before AF ablation procedures is common and can incidentally detect coronary artery calcification (CAC). The purpose of this study was to investigate the prevalence of CAC on pre-ablation CTPV, the frequency of CAC reporting on CTPV reports, and its impact on statin therapy among patients hospitalized for AF procedures. We retrospectively evaluated consecutive patients undergoing CTPV and AF procedures from October 2016 to December 2017 in a single-center tertiary hospital. The patients' demographic and clinical characteristics were analyzed. The CAC presence on CTPV was visually assessed. The severity was classified qualitatively. The statin therapy status was evaluated using the patient's admission and discharge medication lists. A total of 638 subjects were included in our study, with 34.5% female. The mean age was 63.3 ± 10.8 years. CAC was detected in 70.1% of all patients, and in 58.1% of patients without a history of ASCVD. When present, CAC was documented in 92.6% of the clinical CTPV reports. While coronary artery atherosclerosis was present in a majority of AF patients, and its presence was widely reported, it was not associated with increased statin therapy at discharge.

Left atrial volume quantification using coronary calcium score scan: Feasibility, reliability and reproducibility analysis of a standardized approach.

BACKGROUND: Left atrial volume (LAV) is an independent prognosticator of cardiovascular events. We investigated whether LAV could be accurately and reliably measured using coronary calcium score (CAC) scan. METHODS: We retrospectively selected consecutive patients that underwent coronary CT angiography (CCTA) and CAC scans. A standardized approach to calculate LAV on images was implemented. The measurements of the LAV on CAC scans and CCTA were performed one to three weeks apart in a random fashion by two readers blinded to the results of each other. The LAV measurements from CAC scan were compared to those from CCTA using correlation analysis. Inter-observer and intra-observer agreement of LAV measurement using CAC scan was evaluated. RESULTS: Final analysis included one hundred subjects, mean age 52 ±â€¯12 years, 48% male. There was a trend of a marginally larger, albeit not clinically significant, mean LAV calculated using CAC scan compared to that using CCTA: 74.3 vs. 71.0 mL: p < 0.001; for reader 1, and 71.7 vs. 71.2 mL p = 0.06 for reader 2, respectively. LAV using CAC scan and CCTA were highly correlated (R = 0.954, p < 0.001 for reader1 and R = 0.945, p < 0.001 for reader 2). There was high reproducibility within each reader with ICC of 0.951 and 0.989 for readers 1 and 2, respectively (p < 0.001). Finally, there was high inter-observer agreement as indicated by R of 0.97 and ICC of 0.96 (p < 0.001 for both). CONCLUSIONS: Quantification of LAV from CAC scan using the proposed standardized approach is feasible, highly reliable and reproducible as compared to CCTA.

Evidence of aldosterone synthesis in human myocardium in acute myocarditis.

BACKGROUND: Myocarditis may be self-limited but has been identified as an important contributor to downstream cardiomyopathy. Aldosterone mediates myocardial damage in various conditions, but has not been considered specifically as a therapeutic target for inflammatory damage in acute myocarditis. We sought to demonstrate local aldosterone synthesis in human myocardium affected by acute myocarditis. METHODS: We evaluated myocardial samples obtained via endomyocardial biopsy (EMB) for expression of CYP11B2, the final and key enzyme for aldosterone synthesis, from patients with acute myocarditis and from stable heart transplant recipients with no evidence of rejection as negative controls. Excised adrenal glands from patients with aldosterone-secreting adenomas were used as positive controls. An experienced cardiovascular pathologist blinded to clinical information rated CYP11B2 stains as negative, positive, or borderline, also recording location of any CYP11B2-positivity. RESULTS: Sixteen patients' EMB samples showing definite acute myocarditis were identified (50% female). CYP11B2 was positive in 13/16 cases (81%), typically showing diffuse intracardiomyocyte cytoplasmic staining, vs. 2/16 borderline stains in transplant controls (p < 0.001 myocarditis vs. negative controls). All 3 adrenalectomy samples stained positive for CYP11B2 (diffuse intracellular staining). Importantly, no myocarditis or transplant patients were on aldosterone antagonist therapy at the time of biopsy. CONCLUSIONS: In this proof-of-concept study, myocardium from patients with acute myocarditis demonstrates evidence and high prevalence of local aldosterone synthesis by immunohistochemistry that showed high accuracy, specificity, and sensitivity. Aldosterone warrants consideration as a specific target for therapy in patients with myocardial damage due to inflammation towards strategies that reduce downstream complications.

Estimation of myocardial fibrosis in humans with dual energy CT.

BACKGROUND: The current clinical standard for in vivo imaging of myocardial fibrosis is contrast-enhanced cardiac magnetic resonance (CMR). We sought to validate a novel non-contrast dual energy computed tomography (DECT) method to estimate myocardial fibrosis in patients undergoing CMR with contrast. METHODS: All subjects underwent non-contrast, prospectively-triggered cardiac DECT on a single source scanner with interleaved acquisition between tube voltages of 80 and 140 kVp. Monochromatic images were reconstructed at 11 energies spanning 40-140 keV; a region of interest (ROI) was drawn in the mid-inferoseptal segment, recording mean attenuation value in the ROI, at each energy level. Comparison was made to data from single energy (70 keV) image data. Linear discriminant analysis (LDA) was performed to compare the predictive capability of single vs. multi-energy inferoseptal segment CT attenuation on myocardial fibrosis by both visually assessed LGE (absent/present fibrosis) and CMR T1 mapping-derived myocardial extracellular volume fraction (ECV). RESULTS: The multi-energy CT/LDA approach performed better than a single energy approach to discriminate among LGE-CMR classes of present/absence myocardial fibrosis severity, demonstrating correct classification rates of 89% and 71%, respectively. The multi-energy CT/LDA approach also performed better in correctly discriminating normal from elevated ECV, doing so in 89% of patients vs. correct distinction of normal/elevated ECV in only 70% using the single energy approach. CONCLUSIONS: Non-contrast cardiac DECT with multi-energy analysis better classifies myocardial fibrosis and extracellular volume compared to what is feasible with non-contrast single energy cardiac CT. These data support further evaluation of this approach to noninvasively assess myocardial fibrosis.

Trimethylamine N-oxide and incident atherosclerotic events in high-risk individuals with diabetes: an ACCORD trial post hoc analysis.

Introduction: Type 2 diabetes mellitus (T2D) confers high atherosclerotic cardiovascular disease (ASCVD) risk. The metabolite trimethylamine N-oxide (TMAO) derived via gut flora has been linked to excess ASCVD. Research design and methods: We analyzed data, biospecimens, and major adverse cardiovascular events (MACEs) from the prospective multicenter randomized Action to Control Cardiovascular Risk in Diabetes (ACCORD) trial to assess its value in 330 high-risk individuals with T2D without evident atherosclerotic disease at enrollment. Results: Incident cardiovascular events occurred in 165 cases; 165 controls matched by age, sex, and treatment arm experienced no incident events during follow-up. Cases and controls (mean age 64.5 years) had similar mean glycated hemoglobin (HbA1c) (8.2%) and mean 10-year ASCVD risk (23.5%); groups also had similar use of statins and antihypertensive medications at baseline and follow-up. Baseline plasma TMAO levels did not differ between groups after adjusting for ASCVD risk score, HbA1c, and estimated glomerular filtration rate, nor did TMAO distinguish patients suffering incident MACE from those who remained event-free. Conclusions: TMAO's prognostic value for incident ASCVD events may be blunted when applied to individuals with T2D with poor glycemic control and high baseline ASCVD risk. These results behoove further translational investigations of unique mechanisms of ASCVD risk in T2D.

Stabilization of Early Duchenne Cardiomyopathy With Aldosterone Inhibition: Results of the Multicenter AIDMD Trial.

Background Duchenne muscular dystrophy incurs nearly universal dilated cardiomyopathy by the third decade of life, preceded by myocardial damage and impaired left ventricular strain by cardiac magnetic resonance. It has been shown that (1) mineralocorticoid receptor antagonist therapy with spironolactone attenuated damage while maintaining function when given early in a mouse model and (2) low-dose eplerenone stabilized left ventricular strain in boys with Duchenne muscular dystrophy and evident myocardial damage but preserved ejection fraction. We hypothesized that moderate-dose spironolactone versus eplerenone would provide similar cardioprotection in this first head-to-head randomized trial of available mineralocorticoid receptor antagonists, the AIDMD (Aldosterone Inhibition in Duchenne Muscular Dystrophy) trial. Methods and Results This was a multicenter, double-blind, randomized, noninferiority trial. Subjects were randomized to eplerenone, 50 mg, or spironolactone, 50 mg, orally once daily for 12 months. The primary outcome was change in left ventricular systolic strain at 12 months. Among 52 enrolled male subjects, aged 14 (interquartile range, 12-18) years, spironolactone was noninferior to eplerenone (∆strain, 0.4 [interquartile range, -0.4 to 0.6] versus 0.2 [interquartile range, -0.2 to 0.7]; P=0.542). Renal and pulmonary function remained stable in both groups, and no subjects experienced serious hyperkalemia. Infrequent adverse events included gynecomastia in one subject in the spironolactone arm and facial rash in one subject in the eplerenone arm. Conclusions In boys with Duchenne muscular dystrophy and preserved left ventricular ejection fraction, spironolactone added to background therapy is noninferior to eplerenone in preserving contractile function. These findings support early mineralocorticoid receptor antagonist therapy as effective and safe in a genetic disease with high cardiomyopathy risk. Clinical Trial Registration URL: http://www.clinicaltrials.gov. Unique identifier: NCT02354352.

T-Wave Abnormality as Electrocardiographic Signature of Myocardial Edema in Non-ST-Elevation Acute Coronary Syndromes.

BACKGROUND: T-wave abnormalities are common during the acute phase of non-ST-segment elevation acute coronary syndromes, but mechanisms underlying their occurrence are unclear. We hypothesized that T-wave abnormalities in the presentation of non-ST-segment elevation acute coronary syndromes correspond to the presence of myocardial edema. METHODS AND RESULTS: Secondary analysis of a previously enrolled prospective cohort of patients presenting with non-ST-segment elevation acute coronary syndromes was conducted. Twelve-lead electrocardiography (ECG) and cardiac magnetic resonance with T2-weighted imaging were acquired before invasive coronary angiography. ECGs were classified dichotomously (ie, ischemic versus normal/nonischemic) and nominally according to patterns of presentation: no ST- or T-wave abnormalities, isolated T-wave abnormality, isolated ST depression, ST depression+T-wave abnormality. Myocardial edema was determined by expert review of T2-weighted images. Of 86 subjects (65% male, 59.4 years), 36 showed normal/nonischemic ECG, 25 isolated T-wave abnormalities, 11 isolated ST depression, and 14 ST depression+T-wave abnormality. Of 30 edema-negative subjects, 24 (80%) had normal/nonischemic ECGs. Isolated T-wave abnormality was significantly more prevalent in edema-positive versus edema-negative subjects (41.1% versus 6.7%, P=0.001). By multivariate analysis, an ischemic ECG showed a strong association with myocardial edema (odds ratio 12.23, 95% confidence interval 3.65-40.94, P<0.0001). Among individual ECG profiles, isolated T-wave abnormality was the single strongest predictor of myocardial edema (odds ratio 23.84, 95% confidence interval 4.30-132, P<0.0001). Isolated T-wave abnormality was highly specific (93%) but insensitive (43%) for detecting myocardial edema. CONCLUSIONS: T-wave abnormalities in the setting of non-ST-segment elevation acute coronary syndromes are related to the presence of myocardial edema. High specificity of this ECG alteration identifies a change in ischemic myocardium associated with worse outcomes that is potentially reversible.

Evidence of impaired microvascular dilatation in preterms with acute respiratory distress syndrome.

BACKGROUND: Evaluating microcirculatory function in severely ill neonates is a relevant, unmet clinical need. Inappropriate peripheral microvascular vasodilatation is thought to contribute to cardiovascular alterations in preterm infants with acute respiratory distress syndrome (ARDS). We directly evaluated microcirculatory function in preterms with ARDS. METHODS: Peripheral microvascular function was assessed in 50 newborns, divided in three groups: preterms with ARDS; at-term newborns with mild-moderate congenital cardiac disease (Cardio group); healthy controls. Skin microvascular perfusion was assessed using an operator-independent, laser-Doppler camera, under basal conditions and during post-ischemic hyperemia, allowing objective quantification of microcirculatory flow reserve (MFR). RESULTS: At baseline, perfusion was similar among the three groups. During post-ischemic phase, microcirculatory perfusion significantly increased in controls compared to baseline (baseline perfusion units [PU] 3.65±1.8 to 4.59±2.1 during hyperemia; p for trend=0.041), whereas in ARDS group perfusion tended to decrease. Comparing results across groups, ARDS showed lower values compared to either controls or Cardio groups (p<0.05). Controls, and to a lesser extent Cardio group, showed recruitable MFR (1.78±1.13 and 1.19±0.30 in controls and Cardio group, respectively). MFR was absent in ARDS (0.88±0.48; p<0.05), documenting impaired microcirculatory response. CONCLUSION: We demonstrate that it is possible to assess, non-invasively and quantitatively, vasodilator response of skin microcirculation to physiological stimuli in neonates. We also documented that microvascular vasodilation is impaired in preterms with ARDS.

Enfermedad de Ménétrier en niños: reporte de dos casos / Two cases of Menétrier's disease in children

Resumen Se presentan 2 casos de enfermedad de Ménétrier (EM) remitidos a nuestra institución por síndrome edematoso. Esta enfermedad de poca prevalencia es una gastropatía hipertrófica perdedora de proteínas que en la mayoría de los casos es de causa desconocida, aunque se ha asociado con procesos infecciosos. Se caracteriza por edema, hipoproteinemia, hipoalbuminemia y, en la infancia, es de carácter benigno y autolimitado.

Abstract We present two cases of Menétrier's Disease (MS) referred to our institution due to edema. The prevalence of this disease is low. It is a hypertrophic gastropathy which causes loss of proteins and which has unknown causes in the majority of cases although it has been associated with infectious processes. It is characterized by edema, hypoproteinemia, hypoalbuminemia. In childhood it is benign and self-limited.