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Concept: Left ventricular hypertrophy


These pediatric hypertension guidelines are an update to the 2004 “Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents.” Significant changes in these guidelines include (1) the replacement of the term “prehypertension” with the term “elevated blood pressure,” (2) new normative pediatric blood pressure (BP) tables based on normal-weight children, (3) a simplified screening table for identifying BPs needing further evaluation, (4) a simplified BP classification in adolescents ≥13 years of age that aligns with the forthcoming American Heart Association and American College of Cardiology adult BP guidelines, (5) a more limited recommendation to perform screening BP measurements only at preventive care visits, (6) streamlined recommendations on the initial evaluation and management of abnormal BPs, (7) an expanded role for ambulatory BP monitoring in the diagnosis and management of pediatric hypertension, and (8) revised recommendations on when to perform echocardiography in the evaluation of newly diagnosed hypertensive pediatric patients (generally only before medication initiation), along with a revised definition of left ventricular hypertrophy. These guidelines include 30 Key Action Statements and 27 additional recommendations derived from a comprehensive review of almost 15 000 published articles between January 2004 and July 2016. Each Key Action Statement includes level of evidence, benefit-harm relationship, and strength of recommendation. This clinical practice guideline, endorsed by the American Heart Association, is intended to foster a patient- and family-centered approach to care, reduce unnecessary and costly medical interventions, improve patient diagnoses and outcomes, support implementation, and provide direction for future research.

Concepts: Myocardial infarction, Hypertension, Cardiology, Stroke, Blood pressure, Left ventricular hypertrophy, Orthostatic hypotension, Prehypertension


Controversial data exist about the long-term results of aortic coarctation (AC) repair. This study explored the prevalence and predictors of left ventricular (LV) hypertrophy, late hypertension, and hypertensive response to exercise in 48 subjects (age, 15.1 ± 9.7 years) currently followed in the authors' tertiary care hospital after successful AC repair. Data on medical history, clinical examination, rest and exercise echocardiography, and ambulatory blood pressure monitoring were collected. The time from AC repair to follow-up evaluation was 12.9 ± 9.2 years. The prevalence of LV hypertrophy ranged from 23 to 38 %, based on the criteria used to identify LV hypertrophy, and that of concentric geometry was 17 %. One sixth of the patients without residual hypertension experienced late-onset hypertension. One fourth of those who remained normotensive without medication showed a hypertensive response to exercise. Age at AC repair was the strongest independent predictor of LV hypertrophy, defined using indexation either for body surface area (odds ratio [OR], 1.03; p = 0.0090) or for height (OR 1.02; p = 0.029), and it was the only predictor of late hypertension (OR 1.06; p = 0.0023) and hypertensive response to exercise (OR 1.09; p = 0.029). The risk of LV hypertrophy was 25 % for repair at the age of 3.4 years but rose to 50 and 75 % for repair at the ages of 5.9 and 8.4 years, respectively. Similar increases were found for the risk of late-onset hypertension and hypertensive response to exercise. A considerable risk of LV hypertrophy, late hypertension, and hypertensive response to exercise exists after successful AC repair. Older age at intervention is the most important predictor of these complications.

Concepts: Myocardial infarction, Atherosclerosis, Hypertension, Cardiology, Stroke, Blood pressure, Left ventricular hypertrophy, Cortisol


The classic Morrow technique for hypertrophic obstructive cardiomyopathy (HOCM) in patients with simultaneous obstruction of left ventricular (LV) midcavity and right ventricular outflow tract (RVOT) combined with extreme left ventricular hypertrophy, is not effective. A new technique for HOCM surgical correction in patients with severe hypertrophy is proposed.

Concepts: Cardiology, Cardiomyopathy, Left-wing politics, Left ventricular hypertrophy, Hypertrophic cardiomyopathy, Political spectrum, Hypertrophy


: We determined whether left ventricular hypertrophy (LVH) which exceeds that predicted from workload [inappropriate LV mass (LVMinappr)] is associated with reduced left ventricle (LV) systolic chamber function independent of and more closely than absolute or indexed left ventricular mass (LVM).

Concepts: Cardiology, Left ventricular hypertrophy, Right ventricle, Ventricle, Ventricular hypertrophy, Left ventricle


BackgroundTo determine the prognostic value of various self-blood pressure (BP) monitoring (SBPM) cutoff at the time of diagnosis.MethodsCohort of 466 newly diagnosed and never-treated hypertensive patients. At baseline and at 1 year, the patients underwent a physical examination, clinic BP (CBP), SBPM, and ambulatory BP monitoring (ABPM), fasting blood and urine analysis, electrocardiogram (ECG), and retinography. The diagnosis of hypertension was made based on CBP average of two readings, separated by 2 min, taken over three different days, with results ≥140/90 mm Hg. At 1-year follow-up, target organ damage (TOD) evolution was classified as favorable or unfavorable.ResultsMean age was 57.4 years, 56.8% were men. Adjusted multivariate analysis showed that hypertensive patients with baseline SBPM <135/85 mm Hg had a more favorable evolution of left ventricular hypertrophy (LVH) (odds ratio (OR): 1.9; 95% confidence interval (CI): 1.5-2.5), high urinary albumin excretion rate (UAER) (OR: 6.9; 95% CI: 3.4-14.4), and more favorable amount of TOD evolution (OR: 1.7; 95% CI: 1.4-2.0) than those with baseline SBPM ≥135/85 mm Hg. Patients with baseline SBPM <130/80 mm Hg, or <125/80 mm Hg had a more favorable evolution of the amount of TOD (OR: 2.7; 95% CI: 2.0-3.6, and OR: 2.9; 95% CI: 2.1-4.1, respectively) at 1 year than those with baseline SBPM <135/85 mm Hg.ConclusionsBaseline SBPM values <130/80 mm Hg is associated with better evolution of amount of TOD than SBPM values <135/85 mm Hg. These results would support a clinical trial to test a SBPM threshold <130/80 as an optimal pressure not needing pharmacological treatment among those with CBP ≥140/90.American Journal of Hypertension 2012; doi:10.1038/ajh.2012.126.

Concepts: Nephrology, Hypertension, Urine, Cardiology, Left ventricular hypertrophy, Retina, Ventricular hypertrophy, End organ damage


Angiotensin-converting enzyme inhibitors (ACEIs) are the first-line therapy for the treatment of hypertension. However, not all ACEIs are equal. Delapril is a nonsulfhydryl ACEI with unique properties. Delapril has a high lipophilicity and weak bradykinin potentiating action. As a result, delapril has a more potent inhibition capacity of vascular wall angiotensin-converting enzyme activity and a lower incidence of cough than enalapril or captopril. With regard to efficacy, delapril has a long-lasting antihypertensive effect with a trough/peak ratio that is in the upper range of different ACEIs and a positively high smoothness index. Thus, delapril effectively and smoothly reduces blood pressure over 24 h. Moreover, the benefits of delapril are not limited to hypertensive patients, but also in those with microalbuminuria, left ventricular hypertrophy, myocardial infarction or heart failure; delapril appears to be effective and well tolerated.

Concepts: Myocardial infarction, Hypertension, Cardiology, Heart failure, Blood pressure, Left ventricular hypertrophy, ACE inhibitor, Captopril


Left ventricular hypertrophy (LVH) regression is an important issue in hypertensive patients. Patients with LVH who had received the angiotensin receptor blocker (ARB) treatment for 8 weeks and had not reached the target blood pressure level were enrolled in the study. Patients were assigned to either losartan (50 mg)/hydrochlorothiazide (HCTZ, 12.5 mg) group or ARB + CCB group (usual dose of ARB and calcium channel blocker, CCB). After 48 weeks, LV mass index was found to be reduced significantly in the losartan/HCTZ group but not in the ARB + CCB group. These results suggest that combination therapy of an ARB and diuretic has greater potential to cause regression compared with an ARB and CCB.

Concepts: Hypertension, Cardiology, Blood pressure, Left ventricular hypertrophy, Angiotensin, Renin-angiotensin system, Angiotensin II receptor antagonist, Angiotensin receptor


BACKGROUND: Most sudden deaths during sports occur in general population practising recreational sports and according to legislation, a forensic autopsy is required. Most are sudden cardiac deaths but the incidence of specific pathologies differs in reported series according to autopsy methods or diagnostic criteria. The purpose of this work is to analyse the pathology of sports-related sudden deaths in a large forensic series from Spain studied according with cardiovascular pathology criteria. MATERIALS AND RESULTS: We have reviewed the sudden deaths occurred during sports studied at our institution between 1995 and 2010 in which a complete autopsy was performed with exhaustive cardiac examination and toxicological analysis. Out of 8862 sudden deaths studied, 168 (1.8%) were related to sports; age was between 9 and 69 (average 36.6±15.6y); 163 were males and 5 females. Only 3 were professional athletes. Most frequent sports associated to sudden death were cycling (29%), soccer (25.5%), running (8.9%) and gymnastics (6.5%). In 49 cases (29.1%) there were some personal pathological antecedents or familial sudden deaths. Causes of death were: coronary atherosclerotic disease, 85 (50.5%) (74 over 35 years old); arrhythmogenic cardiomyopathy, 13 (7.7%); hypertrophic cardiomyopathy, 12 (7.1%); idiopathic left ventricular hypertrophy, 7 (4.1%); congenital coronary anomalies, 7 (4.1%); myocarditis, 6 (3.5%); aortic stenosis, 5 (2.9%); and other, 11 (6.5%). Myocardial diseases were the most frequent under 35 years old. No cause of death was found in 19 (11.3%) (all under 30 years old) what stresses the necessity of including molecular techniques in forensic autopsies.

Concepts: Death, Atherosclerosis, Pathology, Cardiology, Heart, Left ventricular hypertrophy, Hypertrophic cardiomyopathy, Autopsy


-Left ventricular (LV) hypertrophy (LVH, high LV mass) is traditionally classified as concentric or eccentric based on LV relative wall thickness. We evaluated the prediction of subsequent adverse events in a new 4-group LVH classification based on LV dilatation (high LV end-diastolic volume [EDV] index) and concentricity (LVM/EDV((2/3))) in hypertensive patients.

Concepts: Hypertension, Cardiology, Left ventricular hypertrophy, Geometry, Ventricular hypertrophy, Aortic insufficiency, Cardiomegaly


PurposePompe disease results from lysosomal acid α-glucosidase (GAA) deficiency and its associated glycogen accumulation and muscle damage. Alglucosidase alfa (recombinant human GAA (rhGAA)) received approval in 2006 as a treatment for Pompe disease at the 160 L production scale. In 2010, larger-scale rhGAA was approved for patients up to 8 years old without cardiomyopathy. NCT01526785 evaluated 4,000 L rhGAA efficacy/safety in US infantile- or late-onset Pompe disease (IOPD, LOPD) patients up to 1 year old transitioned from 160 L rhGAA.MethodsA total of 113 patients (87 with IOPD; 26 with LOPD) received 4,000 L rhGAA for 52 weeks dosed the same as previous 160 L rhGAA. Efficacy was calculated as the percentage of patients stable/improved at week 52 (without death, new requirement for invasive ventilation, left ventricular mass z-score increase >1 if baseline was >2, upright forced vital capacity decrease ≥15% predicted, or Gross Motor Function Measure-88 decrease ≥8 percentage points). Safety evaluation included an extension ≤20 months.ResultsWeek 52 data was available for 104 patients, 100 of whom entered the extension. At week 52, 87/104 (83.7%) were stable/improved. Overall survival was 98.1% overall, 97.6% IOPD, 100% LOPD; 92.4% remained invasive ventilator-free (93.4% IOPD, 88.7% LOPD). Thirty-five patients had infusion-associated reactions. Eight IOPD patients died of drug-unrelated causes.ConclusionsMost Pompe disease patients were clinically stable/improved after transitioning to 4,000 L rhGAA. Safety profiles of both rhGAA forms were consistent.Genetics in Medicine advance online publication, 22 March 2018; doi:10.1038/gim.2018.2.

Concepts: Death, Respiratory physiology, Left ventricular hypertrophy, Standard deviation, Glycogen storage disease, Percentage point, Glycogen storage disease type II, Vital capacity