Static cutoffs or tables for the diagnosis of hypertension? Effect on identification of organ damage in youths with obesity.

BACKGROUND AND AIM: Recently, the European Society of Cardiology task force released a Consensus document (ESC-CD) on pediatric hypertension (HTN) supporting the use of normative tables (age range 6-16 years) for the diagnosis of HTN, while the Hypertension Canada Guidelines (HTN-CGs) proposed static cutoffs. We aimed to assess the prevalence of HTN by ESC-CD or HTN-CGs and their association with glomerular function and left ventricular (LV) geometry in youths with overweight/obesity (OW/OB). METHODS AND RESULTS: Data of 3446 youths were analyzed. HTN by was defined using normative tables (ESC-CD) or static cutoffs of BP ≥ 120/80 in children (age <12 years) and ≥130/85 mmHg in adolescents (age ≥12 years) (HTN-CGs). Mildly reduced glomerular filtration rate was defined by GFR <90 ≥ 60 mL/min/1.73 m2. Concentric LV hypertrophy (cLVH) was assessed in 500 youths and defined by LVH and high relative wall thickness as proposed by ESC-CD. Prevalence of HTN was 27.9% by ESC-CD and 22.7% by HTN-CGs. The association with mildly reduced glomerular filtration rate was significant only in hypertensive adolescents classified by HTN-CGs [Odds Ratio (OR), 95%Cl] 2.16 (1.44-3.24), whereas the association with cLVH was significant using both criteria: children OR 2.18 (1.29-3.67) by ESC-CD and 2.27 (1.32-3.89) by HTN-CGs; adolescents OR 2.62 (1.17-5.84) by ESC-CD and 2.83 (1.14-7.02) by HTN-CGs. CONCLUSION: Although static cutoffs may represent a simplification for HTN identification, tables by ESC-CD detect a higher number of hypertensive youths before a clear appearance of glomerular impairment, which offers advantages in terms of primary cardiovascular prevention.

Hypertension in children and adolescents.

Definition and management of arterial hypertension in children and adolescents are uncertain, due to different positions of current guidelines. The European Society of Cardiology task-force, constituted by Associations and Councils with interest in arterial hypertension, has reviewed current literature and evidence, to produce a Consensus Document focused on aspects of hypertension in the age range of 6-16 years, including definition, methods of measurement of blood pressure, clinical evaluation, assessment of hypertension-mediated target organ damage, evaluation of possible vascular, renal and hormonal causes, assessment and management of concomitant risk factors with specific attention for obesity, and anti-hypertensive strategies, especially focused on life-style modifications. The Consensus Panel also suggests aspects that should be studied with high priority, including generation of multi-ethnic sex, age and height specific European normative tables, implementation of randomized clinical trials on different diagnostic and therapeutic aspects, and long-term cohort studies to link with adult cardiovascular risk. Finally, suggestions for the successful implementation of the contents of the present Consensus document are also given.

Sex differences in arterial hypertension.

There is strong evidence that sex chromosomes and sex hormones influence blood pressure (BP) regulation, distribution of cardiovascular (CV) risk factors and co-morbidities differentially in females and males with essential arterial hypertension. The risk for CV disease increases at a lower BP level in females than in males, suggesting that sex-specific thresholds for diagnosis of hypertension may be reasonable. However, due to paucity of data, in particularly from specifically designed clinical trials, it is not yet known whether hypertension should be differently managed in females and males, including treatment goals and choice and dosages of antihypertensive drugs. Accordingly, this consensus document was conceived to provide a comprehensive overview of current knowledge on sex differences in essential hypertension including BP development over the life course, development of hypertension, pathophysiologic mechanisms regulating BP, interaction of BP with CV risk factors and co-morbidities, hypertension-mediated organ damage in the heart and the arteries, impact on incident CV disease, and differences in the effect of antihypertensive treatment. The consensus document also highlights areas where focused research is needed to advance sex-specific prevention and management of hypertension.

Computational Modeling of Drying of Pharmaceutical Wet Granules in a Fluidized Bed Dryer Using Coupled CFD-DEM Approach.

Drying of wet granules in a fluidized bed dryer is an important part of the pharmaceutical tablet manufacturing process. Complicated gas-solid flow patterns appear in the fluidized bed dryer, and interphase momentum, heat, and mass transfer happen during the drying process. A coupled computational fluid dynamics (CFD)-discrete element method (DEM)-based approach was used to model the drying process of pharmaceutical wet granules in a fluidized bed dryer. The evaporation of water from the surfaces of the particles and the cohesion force between the particles due to the formation of liquid bridges between the particles were also considered in this model. The model was validated by comparing the model predictions with the experimental data available from the literatures. The validated model was used to investigate the drying kinetics of the wet granules in the fluidized bed dryer. The results from numerical simulations showed that the dynamics and rate of increase of temperature of wet particles were considerably different from those of dry particles. Finally, the model was used to investigate the effects of inlet air velocity and inlet air temperature on the drying process. The model predicted increase in drying rate with the increase of inlet air velocity and inlet air temperature. This model can help not only to understand the multiphase multicomponent flow in fluidized bed dryer but also to optimize the drying process in the fluidized bed dryer.

Elevated blood pressure, cardiometabolic risk and target organ damage in youth with overweight and obesity.

BACKGROUND AND AIM: To compare cardiometabolic risk profile and preclinical signs of target organ damage in youth with normal and elevated blood pressure (BP), according to the American Academy of Pediatrics (AAP) guidelines. METHODS AND RESULTS: This cross-sectional multicenter study included 2739 youth (5-17 year-old; 170 normal-weight, 610 overweight and 1959 with obesity) defined non hypertensive by the AAP guidelines. Anthropometric, biochemical and liver ultrasound data were available in the whole population; carotid artery ultrasound and echocardiographic assessments were available respectively in 427 and 264 youth. Elevated BP was defined as BP ≥ 90th to <95th percentile for age, gender and height in children or BP ≥ 120/80 to <130/80 in adolescents. The overall prevalence of elevated BP was 18.3%, and significantly increased from normal-weight to obese youth. Young people with elevated BP showed higher levels of body mass index (BMI), insulin resistance and a higher prevalence of liver steatosis (45% vs 36%, p < 0.0001) than normotensive youth, whilst they did not differ for the other cardiometabolic risk factors, neither for carotid intima media thickness or left ventricular mass. Compared with normotensive youth, individuals with elevated BP had an odds ratio (95%Cl) of 3.60 (2.00-6.46) for overweight/obesity, 1.46 (1.19-1.78) for insulin-resistance and 1.45 (1.19-1.77) for liver steatosis, controlling for centers, age and prepubertal stage. The odds for insulin resistance and liver steatosis persisted elevated after correction for BMI-SDS. CONCLUSION: Compared to normotensive youth, elevated BP is associated with increased BMI, insulin resistance and liver steatosis, without significant target organ damage.

Myocardial mechano-energetic efficiency and insulin resistance in non-diabetic members of the Strong Heart Study cohort.

BACKGROUND: Myocardial energetic efficiency (MEE), is a strong predictor of CV events in hypertensive patient and is reduced in patients with diabetes and metabolic syndrome. We hypothesized that severity of insulin resistance (by HOMA-IR) negatively influences MEE in participants from the Strong Heart Study (SHS). METHODS: We selected non-diabetic participants (n = 3128, 47 ± 17 years, 1807 women, 1447 obese, 870 hypertensive) free of cardiovascular (CV) disease, by merging two cohorts (Strong Heart Study and Strong Heart Family Study, age range 18-93). MEE was estimated as stroke work (SW = systolic blood pressure [SBP] × stroke volume [SV])/"double product" of SBP × heart rate (HR), as an estimate of O2 consumption, which can be simplified as SV/HR ratio and expressed in ml/sec. Due to the strong correlation, MEE was normalized by left ventricular (LV) mass (MEEi). RESULTS: Linear trend analyses showed that with increasing quartiles of HOMA-IR patients were older, more likely to be women, obese and hypertensive, with a trend toward a worse lipid profile (all p for trend < 0.001), progressive increase in LV mass index, stroke index and cardiac index and decline of wall mechanics (all p < 0.0001). In multivariable regression, after adjusting for confounders, and including a kinship coefficient to correct for relatedness, MEEi was negatively associated with HOMA-IR, independently of significant associations with age, sex, blood pressure, lipid profile and central obesity (all p < 0.0001). CONCLUSIONS: Severity of insulin resistance has significant and independent negative impact on myocardial mechano-energetic efficiency in nondiabetic individual from a population study of American Indians. Trial registration number NCT00005134, Name of registry: Strong Heart Study, URL of registry: https://clinicaltrials.gov/ct2/show/NCT00005134 , Date of registration: May 25, 2000, Date of enrolment of the first participant to the trial: September 1988.

Prognostic impact of increased pulse pressure/stroke index in a registry of hypertensive patients: the Campania Salute Network.

Objectives: Increased arterial stiffness is associated with advanced arteriosclerosis, abnormal left ventricular (LV) geometry and function. Whether increased arterial stiffness is associated with incident cardiovascular (CV) event (MACE), independent of other markers of target organ damage needs to be clarified. Methods: We selected hypertensive participants of the Campania Salute Network free of prevalent CV disease, with available echocardiogram and carotid ultrasound, ejection fraction ≥50%, and ≤ stage III Chronic Kidney Disease (n = 6907). Median follow-up was 63 months. End-point was incident MACE (fatal and non-fatal stroke and myocardial infarction, sudden cardiac death, carotid stenting and heart failure requiring hospitalization). Arterial stiffness was assessed from ratio of brachial pulse pressure/stroke index (i.e. normalized for body height in meter to 2.04 power) (PP/SVi). High PP/SVi (n = 980) was defined as >95th sex-specific percentile of the normal distribution from a reference normal population (>2.63/>2.82 mmHg/ml in men and women, respectively). Results: Patients with high PP/SVi were more likely to be women, older, diabetic, with higher systolic blood pressure (BP) and heart rate, more LV concentric geometry, left atrial dilatation and more carotid plaque (all p < .01). At given increase in SVi, patients with high PP/SVi exhibited two-fold increase in PP compared to normal PP/SVi. In Cox regression, patients with high PP/SVi had 63% increased hazard of MACE [95% CI (1.02-2.59) p = .04], independently of significant effect of older age, male sex, carotid plaque and less frequent anti-RAS therapy. Conclusions: In treated hypertensive patients, high PP/SVi predicted increased rate of MACE, independent of common confounders.

Determinants of decline of renal function in treated hypertensive patients: the Campania Salute Network.

Background: Hypertension is a leading cause of chronic kidney disease (CKD) and a decrease in glomerular filtration rate (GFR) is associated with a higher prevalence of hypertension and an increased proportion of suboptimal blood pressure (BP) control. Methods: To investigate characteristics associated with GFR decline, we selected 4539 hypertensive patients from the Campania Salute Network (mean age 53 ± 11 years) with at least 3 years of follow-up (FU) and no more than Stage III CKD. GFR was calculated at baseline and at the last available visit using the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation. GFR decline was defined as a ≥30% decrease from initial GFR for patients in Stage III CKD or by a composite ≥30% decrease from baseline and a final value of <60 for those < with Stage III or higher CKD. Results: At a mean FU of 7.5 years, 432 patients (10%) presented with GFR decline. Those patients were older, more likely to be diabetic, with lower GFR and ejection fraction, higher systolic and lower diastolic BP and higher left ventricular (LV) mass and relative wall thickness at baseline; during FU, patients with GFR decline exhibited higher systolic BP, took more drugs and developed more atrial fibrillation (all P < 0.02). The probability of GFR decline was independently associated with older age, prevalent diabetes, baseline lower GFR, higher systolic BP during FU, FU duration, increased LV mass and incident AF with no impact from antihypertensive and antiplatelet medications. Conclusions: During antihypertensive therapy, kidney function declines in patients with initially lower GFR, increased LV mass and suboptimal BP control during FU.

2018 Practice Guidelines for the management of arterial hypertension of the European Society of Cardiology and the European Society of Hypertension.

These practice guidelines on the management of arterial hypertension are a concise summary of the more extensive ones prepared by the Task Force jointly appointed by the European Society of Hypertension and the European Society of Cardiology. These guidelines have been prepared on the basis of the best available evidence on all issues deserving recommendations; their role must be educational and not prescriptive or coercive for the management of individual subjects who may differ widely in their personal, medical and cultural characteristics. The members of the Task Force have participated independently in the preparation of these guidelines, drawing on their academic and clinical experience and by objective examination and interpretation of all available literature. A disclosure of their potential conflict of interest is reported on the websites of the ESH and the ESC.

Assessment of left atrial size in addition to focused cardiopulmonary ultrasound improves diagnostic accuracy of acute heart failure in the Emergency Department.

BACKGROUND: Acute dyspnea is one of the main reasons for admission to Emergency Department (ED). Availability of ultraminiaturized pocket ultrasound devices (PUD) adds diagnostic power to the clinical examination. The aim of this study was to identify an integrated ultrasound approach for diagnosis of acute heart failure (acute HF), using PUD and combining evaluation from lung, heart and inferior vena cava (IVC). METHODS: We included 102 patients presenting to the ED of "Antonio Cardarelli" Hospital in Naples (Italy) for acute dyspnea (AD). All patients underwent integrated ultrasound examination (IUE) of lung-heart-IVC, using PUD. The gold standard was the final diagnosis determined by two expert reviewers: acute heart failure (acute HF) or noncardiac dyspnea. We used 2 × 2 contingency tables to analyze sensitivity, specificity, positive and negative predictive value and accuracy of the three ultrasonic methods, and their combinations for the diagnosis of acute HF, comparing with the final, validated diagnosis. RESULTS: Lung ultrasound (LUS) alone exhibited a good sensitivity (100%) and specificity (82%) and had the highest accuracy (89%) among single modalities (heart and IVC) for the diagnosis of acute HF. The highest accuracy among all methods (96%) was obtained by the combination of positive LUS and either dilated left atrium or EF ≤ 40% or both (all P < .01 vs single modalities). CONCLUSION: In patients presenting to ED, IUE using PUD is a useful extension of clinical examination and has a reliable diagnostic discriminant ability in the immediate evaluation of acute dyspnea. The combination of positive LUS with focused cardiac ultrasonography (FoCUS), including also dilated left atrium, substantially extends the spectrum of recognizable acute HF.

A challenging diagnosis of dyspnea: A case report of contralateral reexpansion pulmonary edema.

Reexpansion pulmonary edema (RPE) is an uncommon complication of thoracentesis or chest drainage. It occurs in the ipsilateral or contralateral lung. Causes, pathogenesis and therapy are not well understood especially for contralateral RPE. We describe a case of fatal contralateral RPE in a 59-years-old woman with right lung cancer underwent ultrasound-guided thoracentesis for massive pleural effusion and severe dyspnea. Pathogenesis of contralateral RPE is probably multifactorial and in this case is mostly due to the overperfusion of the healthy lung and consequent capillary damage. The right therapy for this condition is not known.

Target organ damage and incident type 2 diabetes mellitus: the Strong Heart Study.

BACKGROUND: Recent analyses in a registry of hypertensive patients suggested that preceding left ventricular (LV) hypertrophy (LVH) and/or carotid atherosclerosis are associated with incident type 2 diabetes, independent of confounders. We assess the relation between prevalent cardio-renal target organ damage (TOD) and subsequent incident type 2 diabetes in a population-based study with high prevalence of obesity. METHODS: We selected 2887 non-diabetic participants from two cohorts of the Strong Heart Study (SHS). Clinical exam, laboratory tests and echocardiograms were performed. Adjudicated TODs were LVH, left atrium (LA) dilatation, and high urine albumin/creatinine ratio (UACR). Multivariable logistic regression models were used to identify variables responsible for the association between initial TODs and incident diabetes at 4-year follow-up (FU). RESULTS: After 4 years, 297 new cases of diabetes (10%) were identified, 216 of whom exhibited baseline impaired fasting glucose (IFG, 73%, p < 0.0001). Participants developing type 2 diabetes exhibited higher inflammatory markers, fat-free mass and adipose mass and higher prevalence of initial LVH and LA dilatation than those without (both p < 0.04). In multivariable logistic regression, controlling for age, sex, family relatedness, presence of arterial hypertension and IFG, all three indicators of TOD predicted incident diabetes (all p < 0.01). However, the effects of TOD was offset when body fat and inflammatory markers were introduced into the model. CONCLUSIONS: In this population-based study with high prevalence of obesity, TOD precedes clinical appearance of type 2 diabetes and is related to the preceding metabolic status, body composition and inflammatory status. Trial registration Trial registration number: NCT00005134, Name of registry: Strong Heart Study, URL of registry: https://clinicaltrials.gov/ct2/show/NCT00005134, Date of registration: May 25, 2000, Date of enrolment of the first participant to the trial: September 1988.

Hypertension and cardiac arrhythmias: a consensus document from the European Heart Rhythm Association (EHRA) and ESC Council on Hypertension, endorsed by the Heart Rhythm Society (HRS), Asia-Pacific Heart Rhythm Society (APHRS) and Sociedad Latinoamericana de Estimulación Cardíaca y Electrofisiología (SOLEACE).

Hypertension is a common cardiovascular risk factor leading to heart failure (HF), coronary artery disease, stroke, peripheral artery disease and chronic renal insufficiency. Hypertensive heart disease can manifest as many cardiac arrhythmias, most commonly being atrial fibrillation (AF). Both supraventricular and ventricular arrhythmias may occur in hypertensive patients, especially in those with left ventricular hypertrophy (LVH) or HF. Also, some of the antihypertensive drugs commonly used to reduce blood pressure, such as thiazide diuretics, may result in electrolyte abnormalities (e.g. hypokalaemia, hypomagnesemia), further contributing to arrhythmias, whereas effective control of blood pressure may prevent the development of the arrhythmias such as AF. In recognizing this close relationship between hypertension and arrhythmias, the European Heart Rhythm Association (EHRA) and the European Society of Cardiology (ESC) Council on Hypertension convened a Task Force, with representation from the Heart Rhythm Society (HRS), Asia-Pacific Heart Rhythm Society (APHRS), and Sociedad Latinoamericana de Estimulación Cardíaca y Electrofisiología (SOLEACE), with the remit to comprehensively review the available evidence to publish a joint consensus document on hypertension and cardiac arrhythmias, and to provide up-to-date consensus recommendations for use in clinical practice. The ultimate judgment regarding care of a particular patient must be made by the healthcare provider and the patient in light of all of the circumstances presented by that patient.

Diagnostic performance of multi-organ ultrasound with pocket-sized device in the management of acute dyspnea.

BACKGROUND: The availability of ultra-miniaturized pocket ultrasound devices (PUD) adds diagnostic power to the clinical examination. Information on accuracy of ultrasound with handheld units in immediate differential diagnosis in emergency department (ED) is poor. The aim of this study is to test the usefulness and accuracy of lung ultrasound (LUS) alone or combined with ultrasound of the heart and inferior vena cava (IVC) using a PUD for the differential diagnosis of acute dyspnea (AD). METHODS: We included 68 patients presenting to the ED of "Maurizio Bufalini" Hospital in Cesena (Italy) for AD. All patients underwent integrated ultrasound examination (IUE) of lung-heart-IVC, using PUD. The series was divided into patients with dyspnea of cardiac or non-cardiac origin. We used 2 × 2 contingency tables to analyze sensitivity, specificity, positive predictive value and negative predictive value of the three ultrasonic methods and their various combinations for the diagnosis of cardiogenic dyspnea (CD), comparing with the final diagnosis made by an independent emergency physician. RESULTS: LUS alone exhibited a good sensitivity (92.6%) and specificity (80.5%). The highest accuracy (90%) for the diagnosis of CD was obtained with the combination of LUS and one of the other two methods (heart or IVC). CONCLUSIONS: The IUE with PUD is a useful extension of the clinical examination, can be readily available at the bedside or in ambulance, requires few minutes and has a reliable diagnostic discriminant ability in the setting of AD.

Higher pulse pressure/stroke volume index is associated with impaired outcome in hypertensive patients with left ventricular hypertrophy the LIFE study.

We tested the prognostic impact of a marker of arterial stiffness, pulse pressure/stroke volume index (PP/SVi), in patients with hypertension and left ventricular (LV) hypertrophy. We used data from 866 patients randomized to losartan or atenolol-based antihypertensive treatment, over a median of 4.8 years, in the Losartan Intervention For Endpoint reduction in hypertension (LIFE) study. The association of PP/SVi with outcomes was tested in Cox regression analyses and reported as hazard ratio (HR) and 95% confidence intervals (CI). In multivariate regression, reduction of PP/SVi was independently associated with male gender, reduction in systolic blood pressure (BP) and relative wall thickness and with an increase in left ventricular ejection fraction (all p < .05). After adjusting for confounders, higher baseline PP/SVi predicted a 38% higher hazard of combined major fatal and non-fatal cardiovascular events (95% CI 1.04-1.84), and higher hazard of cardiovascular mortality (HR 2.35 (95% CI 1.59-3.48) and stroke (HR 1.45 (95% CI 1.06-1.99) (all p < .05). Higher PP/SVi also predicts higher rate of hospitalization for HF (HR 2.15 (95% CI 1.48-3.12) and a 52% higher hazard of all-cause mortality (95% CI 1.10-2.09) (both p < .05). In hypertensive patients with electrocardiographic LV hypertrophy, higher PP/SVi was associated with increased cardiovascular morbidity and mortality.

Value of Combined Circumferential and Longitudinal Left Ventricular Systolic Dysfunction to Predict Adverse Outcome in Patients with Asymptomatic Aortic Stenosis.

BACKGROUND AND AIM OF THE STUDY: Patients with asymptomatic aortic stenosis (AS) may have left ventricular systolic dysfunction (LVSD) defined as an impairment of the circumferential and/or longitudinal (C&L) myocardial fibers, despite a preserved left ventricular ejection fraction (LVEF). An assessment was made as to whether the combined LVSD of C&L fibers has a prognostic impact in asymptomatic AS. METHODS: A total of 200 asymptomatic AS patients was analyzed. Midwall shortening and mitral annular peak systolic velocity were considered as indices of C&L function and classified as low if <16.5% and <8.5 cm/s, respectively. The primary outcome was a composite of major cardiovascular events (MACE), including aortic valve-related and ischemic cardiovascular-related events. RESULTS: During a 25-month follow up period, MACE occurred in 69 patients (35%),while 46 of 72 patients (64%) had C&L LVSD and 23 of 128 patients (18%) had not (p <0.001). Cox analysis identified C&L LVSD as an independent MACE predictor, together with aortic transvalvular peak gradient, E/E' ratio and excessive left ventricular mass. C&L-LVSD also predicted the occurrence of aortic valve-related events and ischemic cardiovascular-related events analyzed separately. A receiver operating characteristic curve analysis showed that the area under the curve (AUC) for C&L LVSD in predicting MACE was 0.77, significantly higher (p = 0.002, z-statistic) than the AUCs of C&L fibers considered individually (0.64 and 0.63, respectively). CONCLUSION: C&L-LVSD provides additional prognostic information into traditional risk factors for patients with asymptomatic AS.

Early markers of right heart involvement in regular smokers by Pocket Size Imaging Device.

PURPOSE: To test the diagnostic power of Pocket Size Imaging Device (PSID) in detecting early signs of right heart (RH) involvement in regular smokers (RS) free of overt cardiac involvement. METHODS: One-hundred-forty-three regular smokers and 51 healthy controls, comparable for age and sex, underwent physical exam (PE), PSID exam and standard echocardiography. Based on a simplified Boston score, ≥1 of clinical signs (jugular venous distension, hepatomegaly, peripheral pitting oedema and abnormal pulmonary sounds) were considered indicative of RH involvement. A composite score (1 to 4) obtained by summing the points of four quantitative RH abnormalities detectable by PSID (inferior vena cava [IVC] dilatation, reduced IVC respiratory variation, right ventricular dilatation and right atrial dilatation), was generated and ≥1 of PSID abnormal signs was considered indicative of RH involvement. RESULTS: Boston score was not significantly different between the two groups. By using PSID, smokers exhibited greater IVC diameter (p < 0.0001), right atrial diameter (p < 0.002) and higher PSID score (p < 0.005) than controls. Compared to PE, the additional diagnostic power of PSID (≥1 abnormal sign of both Boston and PSID score) was 44.9% in smokers. By dividing smokers in tertiles according to number of cigarettes per day, the third tertile showed the largest values of both IVC and right atrial dimension. Differences were confirmed by standard echocardiography. Reproducibility of PSID measurements and concordance of linear measurements between PSID and standard echo measurements was very good except for concordance of right ventricular basal diameter. CONCLUSIONS: PSID detects early ultrasound signs of RH involvement in regular otherwise healthy smokers in comparison with PE.

Left ventricular diastolic dysfunction in type I Gaucher disease: an echo Doppler study.

Type I Gaucher disease (GD1) is an autosomal recessive lysosomal storage disease characterized by multiorgan damage. Left ventricular (LV) involvement has been rarely reported. Accordingly, the aim of the study was to evaluate LV geometry and function in a series of patients with GD1. Eighteen patients with GD1, 18 age- and sex-matched normal controls, and 18 age- and sex-matched hypertensive patients (HTN) were compared by standard echo Doppler examination. LV mass index, relative wall thickness and ejection fraction, transmitral E/A ratio, E velocity deceleration time (DT), atrial filling fraction (AFF = time-velocity integral of A velocity/time-velocity integral of total diastole × 100), E/e' ratio, and left atrial volume index were determined. Nine GD1 patients also exhibited arterial hypertension. The intergroup difference of LV mass index and relative wall thickness was not significant. Transmitral E/A ratio was lower in HTN than in normal controls and GD1 (P < 0.05). GD1 exhibited longer DT than NC and HTN (P = 0.009). AFF was higher in GD1 and HTN compared to NC (P = 0.034). After adjustment for heart rate, GD1 was associated with longer DT (P < 0.001) and greater AFF (P = 0.036), while HTN was associated only with AFF (P = 0.013). No interaction was found between GD1 and HTN. In conclusion, GD1 is associated with subclinical LV diastolic dysfunction, which is independent of the coexistence of arterial hypertension. Subclinical LV impaired relaxation in the context of myocardial infiltrative damage could be the mechanism underlying these alterations.

Combined circumferential and longitudinal left ventricular systolic dysfunction in patients with asymptomatic aortic stenosis.

BACKGROUND: Early detection of left ventricular (LV) systolic dysfunction is pivotal in the management of patients with aortic stenosis (AS). LV circumferential and/or longitudinal shortening may be impaired in these patients despite LV ejection fraction is preserved. We focused on prevalence and factors associated with combined impairment of circumferential and longitudinal shortening (C&L) in asymptomatic AS patients. METHODS: Echocardiographic and clinical data from 200 patients with asymptomatic AS of any degree without history of heart failure and normal LV ejection fraction were analyzed. C&L were evaluated by mid-wall shortening (MS) and tissue Doppler mitral annular peak systolic velocity (S'), and classified low if <16.5% and if <8.5 cm/sec, respectively (10th percentiles of controls). RESULTS: Combined C&L dysfunction was detected in 72 patients (36%). The variables associated with this condition were higher LV mass (OR 1.02 [CI 1.01-1.04], P = 0.03), concentric LV geometry (OR 4.30 [CI 1.79-10.34], P = 0.001), increasing pulmonary artery wedge pressure (by E/e' ratio; OR 1.11 [CI 1.04-1.19], P = 0.001). The relation of MS and peak S' was linear and slightly significant in the whole population (r = 0.23; F statistic=0.001), absent in patients with C&L dysfunction (r = 0.04; F = ns), negative (linear model) in the subgroup of patients without C&L dysfunction (r = -0.22; F = 0.02). CONCLUSIONS: C&L dysfunction is present in more than one-third of patients with asymptomatic AS and is associated with concentric LV geometry and higher degree of diastolic dysfunction. The relation between MS and peak S' largely varies in the subgroups with different C&L function.