The prognostic role of the ECG in primary pulmonary hypertension

BACKGROUND/RATIONALE: Doppler echocardiography and invasive hemodynamic parameters reflective of right ventricular failure are associated with a poor prognosis in patients with primary pulmonary hypertension (PPH). The aims of the present study were to examine whether ECG features in patients with PPH are associated with a decrease in survival, and to determine the value of the ECG in risk stratification. METHODS/RESULTS: We analyzed the ECG, New York Heart Association (NYHA) class, and hemodynamic parameters in 51 untreated patients with PPH (88% women; mean age, 41.7 years; 79% NYHA classes III and IV) evaluated between 1992 and 1998. Subsequent treatment included epoprostenol in 37 patients, calcium channel blockers in 10 patients, epoprostenol and atrial septostomy in 2 patients, and lung transplant in 3 patients. As of 1999, 16 patients had died. Based on Kaplan-Meier estimates, median survival was > 6.5 years and estimated survival at 1 year, 3 years, and 5 years was 86%, 71%, and 57%, respectively. Significant predictors of decreased survival by Cox regression analysis include pulmonary vascular resistance (PVR; hazard ratio [HR], 1.11 per Wood unit), cardiac index (HR, 0.22 per L/min/m(2)), p wave amplitude in lead II (HR, 3.06 per mm), p > or = 0.25 mV in lead II (HR, 2.77), qR in V(1) (HR, 3.55), and World Health Organization criteria for right ventricular hypertrophy (HR, 4.26). After controlling for PVR, the HRs attributable to the ECG criteria were only slightly diminished. NYHA class and pulmonary artery pressures did not correlate with a decrease in survival. CONCLUSIONS: ECG parameters reflective of physiologic and anatomic abnormalities in the right ventricle are associated with decreased survival in patients with PPH, and may be useful for deciding therapeutic choices including the timing for lung transplantation listing.     

高血压所致左心室肥厚与肥厚型心肌病体表心电图和超声心动图的鉴别要点

目的 比较高血压所致左心室肥厚与肥厚型心肌病（HCM）患者体表心电图及超声心动图参数之间的差异，探讨体表心电图对二者鉴别诊断的临床应用价值。 方法 收集高血压致左心肥厚（H-LVH）患者，HCM患者及正常对照各49例。常规测量心电图中的P波时限、QTC时限，QRS波时限、异常q波数量、R波及S波振幅和ST-T与QRS波方向一致率，超声心动图中测量E/A、左室射血分数（LVEF）、室间隔厚度、左房内径（LA）、左心室收缩末期左右径（LVs左右径）、左心室舒张末期左右径（LVd左右径）、左心室收缩末期前后径（LVs前后径）、左心室舒张末期前后径（LVd前后径）、左心室收缩末期长轴（LVs长轴）、左心室舒张末期长轴（LVd长轴）等参数，比较3组之间的差异。 结果 HCM组患者与H-LVH组患者比较，最大左心室室壁厚度显著增厚，LVEF显著升高（均P<0.05），而HCM每博排出量（SV）、LVs左右径、LVd左右径、LVs前后径、LVd前后径、LVd长径显著小于H-LVH（均P<0.05）；心电图提示，HCM组患者II，III，Avf V1 V2导联R波与S波电压之和，QTC时限显著高于H-LVH组患者（P<0.01）；HCM患者异常Q波数量以及III、aVR导联ST-T与QRS波方向一致率显著高于H-LVH组（P<0.01）。 结论 超声心动图可从左心室最大室壁厚度及肥厚特征鉴别HCM患者和H-LVH患者；体表心电图鉴别二者需结合QRS波振幅之和，QTC时限，II、V4导联异常Q波数量及导联ST-T与QRS波方向在V2、V3、V6导联的一致率4个参数。     

脊髓性肌萎缩症的基因研究

目的 研究我国Ⅰ～Ⅳ型脊髓性肌萎缩症（SMA）患者运动神经元生存基因（SMN）及神经细胞凋亡抑制蛋白（NAIP）基因外显子的缺失情况,以探讨此两种基因与SMA表型之间的关系。方法 应用PCR法检测45例Ⅰ～Ⅳ型SMA患者、30例表型正常的SMA直系亲属及30例正常对照的SMIN基因第7、8号外显子和NAIP基因第5、6号外显子缺失情况。结论 7例Ⅰ型和Ⅱ型SMA患者中6例纯合缺失SMN基因外显子7和8,1例纯合缺失外显子7而保留外显子8;8例Ⅲ型SMA患者仅1例缺失外显子7和8,余7例均无SMN基因的缺失;成人型（Ⅳ型）SMA未检测到SMN基因缺失;45例Ⅰ～Ⅳ型SMA患者均未检测到NAIP基因外显子5和（或）6的缺失。结论 Ⅰ型、Ⅱ型SMA可通过SMN基因第7、8号外显子的检测进行确诊,Ⅲ型SMA患者SMN基因缺失率低,故通过检测SMN基因7、8外显子进行基因诊断尚需谨慎,Ⅳ型SMA未检测到SMN基因缺失,发病可能与SMN基因缺失无关;NAIP基因在SMA发病中的作用尚不清楚。     

Clinical significance of autoantibodies to soluble liver antigen in autoimmune hepatitis.

BACKGROUND/AIMS: Classification of autoimmune hepatitis (AIH) into different subgroups according to autoantibody status has been proposed: type I (ANA/SMA), type II (LKM-1) and type III (anti-SLA). However, whether type III AIH forms a clinically distinct disease entity remains controversial. The aim of this study was to evaluate the subclassification of AIH into ANA/SMA and anti-SLA positive patients with regard to clinical, biochemical and histologic differences. METHODS: Ninety-seven consecutive patients with a well-documented long-term course of AIH with ANA/SMA and/or anti-SLA autoantibodies were studied. Clinical, biochemical and histological features of patients with ANA/SMA and/or anti-SLA autoantibodies were compared in a secondary analysis of data acquired prospectively. RESULTS: Anti-SLA autoantibodies were found in 21.6% of patients. Anti-SLA-positive patients tended to have lower transaminases (mean: 153 vs. 247 IU/l), gamma-globulins (25 vs. 31%) and bilirubin (1.8 vs. 3.3 mg/dl) in comparison to ANA/SMA positive patients, but there was a large overlap. HLA-type A1 B8 was more frequent in anti-SLA positive patients, while there was no difference in HLA DR3 and DR4 allotype. Response to immunosuppressive therapy was excellent, but relapse occurred frequently. Diagnosis of anti-SLA positive AIH was often delayed (mean: 68 months from first elevation of transaminases) since testing for anti-SLA autoantibodies is currently not generally available. CONCLUSIONS: ANA/SMA and anti-SLA positive patients share most clinical, biochemical, histologic and prognostic features. Distinction between type I and type III AIH is therefore clinically not helpful. However, testing for anti-SLA autoantibodies helps in the diagnosis of AIH in many patients who may otherwise be misdiagnosed.     

Hypertensive cardiopathy. Study of cardiac performance]

Twenty-two hypertensive patients with normal coronary arteriograms underwent a complete study of left ventricular performance. The patients were classified into three groups according to the findings on X-ray and ECG investigation: group I--ECG normal, X-ray normal (6 cases); group II--ECG showed left atrial hypertrophy, X-ray normal (7 cases); group III-ECG showed left ventricular hypertrophy, X-ray showed cardiac dilatation (9 cases). The results of this study were as follows:--cardiac ouptut was lowered in groups II and III;--end-diastolic volume was lowered in group II and raised in group III;--LVEDP was raised in groups II and III;--V max and VEC max were lower in groups II and III than in group I;--left ventricular compliance was diminished in groups I and II. Myocardial rigidity of the left ventricle was similar in the three groups. Hypertensive heart disease is therefore characterised by early changes in compliance of the left ventricle. These are associated with geometrical changes in the ventricle: disturbance of the volume/mass ratio of the left ventricle. These findings have therapeutic implications for the importance of beta-blockers.     

Evaluating the Effectiveness of Diabetes Shared Medical Appointments (SMAs) as Implemented in Five Veterans Affairs Health Systems: a Multi-site Cluster Randomized Pragmatic Trial

ObjectiveTo examine whether diabetes shared medical appointments (SMAs) implemented as part of usual clinical practice in diverse health systems are more effective than usual care in improving and sustaining A1c improvements.Research Design and MethodsA multi-site cluster randomized pragmatic trial examining implementation in clinical practice of diabetes SMAs in five Veterans Affairs (VA) health systems was conducted from 2016 to 2020 among 1537 adults with type 2 diabetes and elevated A1cs. Eligible patients were randomly assigned to either: (1) invitation to participate in a series of SMAs totaling 8–9 h; or (2) continuation of usual care. Relative change in A1c (primary outcome) and in systolic blood pressure, insulin starts, statin starts, and anti-hypertensive medication classes (secondary outcomes) were measured as part of usual clinical care at baseline, at 6 months and at 12 months (~7 months after conclusion of the final SMA in four of five sites). We examined outcomes in three samples of SMA participants: all those scheduled for a SMA, those attending at least one SMA, and those attending at least half of SMAs.ResultsBaseline mean A1c was 9.0%. Participants scheduled for an SMA achieved A1c reductions 0.35% points greater than the control group between baseline and 6-months follow up (p = .001). Those who attended at least one SMA achieved reductions 0.42 % points greater (p < .001), and those who attended at least half of scheduled SMAs achieved reductions 0.53 % points greater (p < .001) than the control group. At 12-month follow-up, the three SMA analysis samples achieved reductions from baseline ranging from 0.16 % points (p = 0.12) to 0.29 % points (p = .06) greater than the control group.ConclusionsDiabetes SMAs as implemented in real-life diverse clinical practices improve glycemic control more than usual care immediately after the SMAs, but relative gains are not maintained. Our findings suggest the need for further study of whether a longer term SMA model or other follow-up strategies would sustain relative clinical improvements associated with this intervention.Trial RegistrationClinicalTrials.gov ID {"type":"clinical-trial","attrs":{"text":"NCT02132676","term_id":"NCT02132676"}}NCT02132676Supplementary InformationThe online version contains supplementary material available at 10.1007/s11606-020-06570-y.KEY WORDS: shared medical appointment, peer support, disease management, implementation, diabetes mellitus, pragmatic clinical trial     

Sudden cardiac death risk factors in patients with heart failure treated with carvedilol

BACKGROUND: Chronic heart failure (CHF) is associated with a high risk of sudden cardiac death (SCD). Most frequently SCD occurs in patients with NYHA class II and III. AIM: To evaluate the influence of prolonged carvedilol therapy on SCD risk in CHF patients. METHODS: The study included 86 patients (81 men and 5 women) aged 56.8+/-9.19 (35-70) years with CHF in NYHA class II and III receiving an ACE inhibitor and diuretics but not beta-blockers. At baseline and after 12 months of carvedilol therapy the following risk factors for SCD were analysed: in angiography - occluded infarct-related artery; in echocardiography - left ventricular ejection fraction (LVEF) <30%, volume of the left ventricle (LVEDV) >140 ml; in ECG at rest - sinus heart rate (HRs) >75/min, sustained atrial fibrillation, increased QTc; in 24-hour ECG recording - complex arrhythmia, blunted heart rate variability (SDNN <100 ms) and abnormal turbulence parameters (TO and TS or one of them); in signal-averaged ECG - late ventricular potentials and prolonged fQRS >114 ms. The analysis of SCD risk factors in basic examination in patients who suddenly died was also performed. RESULTS: During one-year carvedilol therapy heart transplantation was performed in 2 patients; 5 patients died. At 12 months the following risk factors for SCD were significantly changed: HRs >75/min (50 vs. 16 patients, p=0.006), LVEF <30% (37 vs. 14 patients, p=0.01), SDNN <100 ms (19 vs. 9 patients, p=0.04). At 12 months the number of risk factors for SCD in each patient was significantly reduced (p=0.001). In patients who suddenly died we found a greater amount of SCD risk factors in basic examination (7 vs. 5) as compared to alive patients. CONCLUSIONS: Prolonged beta-adrenergic blockade reduces risk of sudden cardiac death through significant LVEF increase, reduction of HR at rest and improvement of HRV.     

Four cases of Fabry's disease mimicking hypertrophic cardiomyopathy

Four patients with Fabry's disease diagnosed by right ventricular endomyocardial biopsy had cardiac manifestations simulating hypertrophic cardiomyopathy (HCM). Case 1: A 51-year-old woman, whose elder sister had congestive heart failure, was hospitalized for exertional dyspnea and cardiomegaly. Her electrocardiogram (ECG) showed a short PQ interval (0.10 sec) and left ventricular hypertrophy. Her echocardiogram (Echo) showed moderate symmetrical hypertrophy of the left ventricle (IVST/PWT = 18 mm/17 mm). Case 2: A 32-year-old woman, whose elder sister had an abnormal ECG, was hospitalized for the ECG abnormalities consisting of a short PQ interval (0.10 sec) and ST-T changes in the left precordial leads. The Echo revealed mild symmetrical hypertrophy of the left ventricle (IVST = 13 mm, PWT = 13 mm). Case 3: A 44-year-old man was hospitalized for his ECG suggestive of left ventricular hypertrophy, and his Echo showed asymmetrical septal hypertrophy (ASH; IVST = 22 mm). Case 4: A 51-year-old man was hospitalized for his ECG showing high voltage in the left precordial leads, and his Echo showed ASH (IVST = 20 mm). The cardiac histopathological findings of these cases included cytoplasmic vacuolization by light microscopy, and electron-dense deposits consisting of parallel or concentric lamellae with periodic spacing, suggesting Fabry's disease. The urinary glycolipids of Case 1 were increased biochemically; then the diagnosis of Fabry's disease was confirmed. Cardiac hypertrophy in Fabry's disease has many aspects, because the histopathological changes and clinical manifestations are determined by genetic factors. It was concluded that Fabry's disease may be concealed in some patients with the clinical diagnosis of HCM.     

Long-term outcomes of left bundle branch block in high-risk survivors of acute myocardial infarction: The VALIANT experience

BACKGROUND: In survivors of myocardial infarction (MI), new left bundle branch block (LBBB) is associated with adverse outcomes, but its impact is not well described in post-MI patients with left ventricular (LV) systolic dysfunction and/or heart failure (HF). OBJECTIVES: The aim of this study was to determine if new LBBB is an independent predictor of long-term fatal and nonfatal outcomes in high-risk survivors of MI by reviewing data from the VALsartan In Acute myocardial iNfarcTion (VALIANT) trial. METHODS: In VALIANT, 14,703 patients with LV systolic dysfunction and/or HF were randomized to valsartan, captopril, or both a mean of 5 days after MI. Baseline ECG data were available from 14,259 patients. We assessed the predictive value of new LBBB for death and major cardiovascular outcomes after 3 years, adjusting for multiple baseline covariates including LV ejection fraction. RESULTS: At follow-up, patients with new LBBB (608 [4.2%]) compared with patients without new LBBB had more comorbidities and increased adjusted risk of death (hazard ratio [HR] 1.3, 95% confidence interval [CI] 1.2-1.6), cardiovascular death (HR 1.4, 95% CI 1.2-1.7), HF (HR 1.3, 95% CI 1.1-1.6), MI (HR 1.5, 95% CI 1.2-1.9), and the composite of death, HF, or MI (HR 1.4, 95% CI 1.2-1.6). CONCLUSION: In post-MI survivors with LV systolic dysfunction and/or HF, new LBBB was an independent predictor of all major adverse cardiovascular outcomes during long-term follow-up. This readily available ECG marker should be considered a major risk factor for long-term cardiovascular complications in high-risk patients after MI.     

Systemic onset juvenile idiopathic arthritis: a retrospective study of 80 consecutive patients followed for 10 years

OBJECTIVE: To investigate the relationships between systemic onset juvenile idiopathic arthritis disease activity, course of the disease, and functional class according to Steinbrocker. METHODS: The records of all children with systemic onset juvenile arthritis (JA) according to the American College of Rheumatology criteria attending our center since 1971 with a minimum followup period of 3 years were reviewed. A cohort of 80 consecutive patients entered the study: 42 males, 38 females, mean age at onset 6.3 years (range 0.7-16), mean followup period 10.7 years (range 3-33). The cumulative duration of the active periods (CDAP) in months was calculated for every patient. RESULTS: Three patterns of disease course were apparent: monocyclic (subtype I), intermittent (subtype II), and persistent (subtype III). At the last control the functional class and disease activity status were evaluated. In all subtype I patients (9 cases) the disease was in remission and no patient was in class II, III, or IV. In subtype II patients (27 cases), 16 were inactive or in remission and 6 in class III. In subtype III (44 cases) 21 were inactive or in remission and 17 were in class III or IV. The equation relating the Steinbrocker class to the CDAP was calculated considering the functional outcome as the dependent variable. The linear regression equation y = 0.0083 x + 1.266 was found with a correlation coefficient r = 0.586 (p < 0.0001). The majority of our patients were treated with disease modifying antirheumatic drugs, which in many cases were effective in reducing the duration of the active phases of disease. CONCLUSION: Systemic onset JA may present with different clinical courses; the functional outcome is always good in subtype I (monocyclic), but can be poor in subtypes II and III. The severity of disability evaluated according to Steinbrocker classes is dependent on the cumulative duration of the active periods of the disease.     

Effect of chronic pressure and volume stress on the function of the right ventricle

Biplane right ventricular angiography was performed in 36 patients with chronic pressure overload of the right ventricle; 12 patients additionally had tricuspid insufficiency (TI). There were 4 subgroups: patients with systolic pulmonary artery pressure less than or equal to 40 mm Hg without (group I, n = 10) and with TI (group II, n = 6), as well as patients with systolic pulmonary artery pressure greater than 40 mm Hg without (group III, n = 14) and with TI (group IV, n = 6). Compared with the normal volumes of groups I and III, a significant increase in end-diastolic right ventricular volumes (p less than 0.01) was found in groups II and IV with 112.2 +/- 22.3 ml/m2 and 116.3 +/- 27.4 ml/m2, respectively. In both groups II and IV end-systolic volumes were also significantly increased, with 51.0 +/- 10.3 ml/m2 in group II and 49.7 +/- 11.3 ml/m2 in group IV. Right ventricular ejection fraction was 53.8 +/- 11.9% in group II, 57.3 +/- 8.5% in group III and 57.8 +/- 7.3% in group IV. There was no significant difference between the ejection fraction of these groups in comparison to the normal ejection fraction of group I with 63.4 +/- 10.9%. The results suggest that the right ventricle can compensate for moderate chronic pressure and volume overload using the Frank-Starling mechanism. Overall right ventricular dysfunction is not determined primarily by the loading conditions alone. Local myocardial and septal involvement is suspected to be an important determinant of right ventricular function.     

Genotype-phenotype relationship in Brugada syndrome: electrocardiographic features differentiate SCN5A-related patients from non-SCN5A-related patients

OBJECTIVES: We have tested whether a genotype-phenotype relationship exists in Brugada syndrome (BS) by trying to distinguish BS patients with (carriers) and those without (non-carriers) a mutation in the gene encoding the cardiac sodium channel (SCN5A) using clinical parameters. BACKGROUND: Brugada syndrome is an inherited cardiac disease characterized by a varying degree of ST-segment elevation in the right precordial leads and (non)specific conduction disorders. In a minority of patients, SCN5A mutations can be found. Genetic heterogeneity has been demonstrated, but other causally related genes await identification. If a genotype-phenotype relationship exists, this might facilitate screening. METHODS: In a multi-center study, we have collected data on demographics, clinical history, family history, electrocardiogram (ECG) parameters, His to ventricle interval (HV), and ECG parameters after pharmacologic challenge with I(Na) blocking drugs for BS patients with (n = 23), or those without (n = 54), an identified SCN5A mutation. RESULTS: No differences were found in demographics, clinical history, or family history. Carriers had a significantly longer PQ interval on the baseline ECG and a significantly longer HV time. A PQ interval of > or =210 ms and an HV interval > or =60 ms seem to be predictive for the presence of an SCN5A mutation. After I(Na) blocking drugs, carriers had significantly longer PQ and QRS intervals and more increase in QRS duration. CONCLUSIONS: We observed significantly longer conduction intervals on baseline ECG in patients with established SCN5A mutations (PQ and HV interval and, upon class I drugs, more QRS increase). These results concur with the observed loss of function of mutated BS-related sodium channels. Brugada syndrome patients with, and those without, an SCN5A mutation can be differentiated by phenotypical differences.     

The early cardiac involvement in patients with β-thalassemia major

BackgroundCardiac complications, heart failure and arrhythmias remain the major causes of death in thalassemia major.AimTo detect the early cardiac involvement in β-thalassemic patients.Patients and methods56 Patients (pts) with β-thalassemia major and transfusion burden ?12 times/year (age 6–16 years) were included in our study, classified into three groups according to serum ferritin, group I: 21 pts with ferritin level <2500 ng/ml, group II: 23 pts with ferritin level 2500–5000 and group III: 12 pts with ferritin level >5000 ng/ml. They were subjected to detailed clinical evaluation, routine laboratory investigations, serum ferritin level, ECG {corrected QT intervals (QTc) and QT dispersion(QTd)}, echocardiography for measurement of left atrial (LA) active emptying fraction, Systolic (peak systolic wave, Q–S peak duration) and diastolic functions of left ventricle using standard and tissue Doppler imaging (TDI).ResultsGroups III and II showed a significant increase in LV septal and posterior wall thickness than group I while QTc and QTd were increased significantly only in group III compared to group I (P = 0.00, 0.01). LV diastolic function and LA active emptying fraction were significantly impaired in group III and II compared to group I while LV systolic function parameters by TDI were impaired significantly in group III compared to group I with insignificant difference by standard echocardiography.ConclusionThe increase in LV septal and posterior wall thickness precedes ECG changes. Also LV diastolic dysfunction and impaired LA active emptying fraction precede LV systolic dysfunction.     

Prevalence and prognostic significance of atrial fibrillation in outpatients with heart failure due to left ventricular systolic dysfunction

INTRODUCTION: Atrial fibrillation (AF) is common in patients with heart failure (HF) due to left ventricular systolic dysfunction (LVSD), with conflicting prognostic data. The aim of our study was to assess the prevalence and incidence of AF in patients with HF and to determine the prognostic impact of baseline AF and the development of new onset AF. METHODS AND RESULTS: We included 1019 outpatients with HF due to LVSD; follow-up time ranged from 3 to 64 months. At baseline 26.4% of patients had AF. Of the 284 patients with a follow-up ECG and baseline SR, 18.7% developed new onset AF. Patients with AF were older (p<0.001), more often male (p=0.04), and more likely to have a history of stroke (p=0.03), but were less likely to have IHD (p<0.001). Baseline rhythm was independent of LVEF and NYHA-class. Baseline AF was associated with increased all-cause mortality (HR 1.38; CI 1.07-1.78, p=0.01) and all-cause mortality/hospitalisation (HR 1.43; CI 1.22-1.68, p<0.001). When adjusted for baseline covariates, baseline AF was independently associated with an increased risk of experiencing the combined endpoint (HR 1.29; CI 1.05-1.58; p=0.02), but did not predict all-cause mortality. By multivariable analyses, new-onset AF was associated with increased risk of all-cause mortality/hospitalisation (HR 1.45; CI 1.05-2.00; p=0.02). CONCLUSION: In outpatients with HF due to LVSD, AF is a common co-morbidity, which adversely affects morbidity and mortality outcomes.     

The hypoplastic right heart complex Clinical, hemodynamic, pathologic and surgical considerations

The term hypoplastic right heart complex is proposed to define a diversity of anomalies of the right ventricle and the tricuspid and pulmonary valves. Based on the angiocardio-graphic and pathologic findings in 10 patients, the cases were divided into four types: Type I, moderate-sized right ventricular cavity (functioning right ventricle), moderate tricuspid stenosis, well formed pulmonary valve but with orifice either stenotic or atretic (4 cases). Type II, small right ventricle (nonfunctioning), rudimentary tricuspid valve and atretic pulmonary valve (3 cases). Type III, same as type I but in association with ventricular septal defect (2 cases). Type IV, isolated hypoplasia of the right ventricle (1 case). The patients ranged in age from a few days to 10 months. Cyanosis, with or without congestive heart failure, was present in all. The electrocardiogram was not characteristic. Hemodynamic studies were available in 5 patients. The right ventricular pressure was higher than systemic pressure in type I, slightly elevated in type II and normal in type IV. A large diastolic gradient was measured across the tricuspid valve in types I and II. In type III no studies were available. The angiocardiogram was of prime value in differentiating among the four types. Successful surgical management depends on an accurate delineation of the various malformed structures.     

Heart rate variability in left ventricular hypertrophy and heart failure, and the effects of beta-blockade. A non-spectral analysis of heart rate variability in the frequency domain and in the time domain.

An analysis of heart rate (HR) variability (HRV) was based on quantifying the number and amplitude of HR fluctuations over long (8-30 cycles) and short (2-4 cycles) sequences of acceleration and deceleration-forming oscillations. The 'product' (number times amplitude) is an equivalent of the power spectrum in the frequency domain. In the time domain, positive correlations with HR were found for the number of long (L) sympathetic-mediated oscillations, whereas they were negative for short (S) vagally-mediated oscillations. The L/S ratio, an index of the autonomic nervous system (ANS) balance, closely paralleled the circadian HR values. HRV was studied in the ambulatory ECG of three groups of 15 normal adults (group I), 13 patients with left ventricular hypertrophy (LVH, group II) and 13 patients with heart failure (HF, group III). In basic conditions the mean HR was 77.1 +/- 1.9 beats min-1 (mean +/- SEM) in group I, 76.8 +/- 3.3 in II, 79.5 +/- 3.5 in III (P = NS). The different types of oscillations had smaller 24-h average values of the product in groups II and III than in group I, but the trends did not reach significance. However, the pooled 24 hourly values of the L/S ratio in group I (1.17 +/- 0.09) were lower than in group II (1.33 +/- 0.06, P less than 0.05 at Ancova) and higher than in group III (1.06 +/- 0.09, P less than 0.001). A 3-day treatment with acebutolol non-uniformly slowed the mean HR:--9.5% in group I, --18.1% in II and -19.1% in III (P less than 0.001), and uniformly diminished the L/S ratio by 17% to 20% (P less than 0.01). In conclusion, the sympathetic predominance of the ANS balance in LVH and HF is reflected by the beta-blockade induced HR decrease that is twice as marked in patients as in controls. In basic conditions HRV tends to be depressed in LVH and even more in HF. The ANS imbalance, however, has different modalities depending on the presence of HF, probably because of the different status of beta-adrenergic receptors in this condition.     

Quantitative evaluation of right ventricle function by transthoracic echocardiography in childhood congenital heart disease patients with pulmonary hypertension

Introduction and Objective: The present study aims to quantitatively evaluate the right ventricle (RV) function by means of transthoracic echocardiography in normal children and childhood congenital heart disease patients with pulmonary hypertension. Patients and Methods: This study was conducted in a cohort including 40 healthy children and 30 pediatric patients with pulmonary hypertension who were diagnosed under close surveillance at the study center between October 2009 and November 2010. Results: Statistically significant differences were found between the patient and control groups for the right ventricle myocardial performance index (RVMPI), the left ventricle myocardial performance index (LVMPI), the tricuspid valve systolic flow velocity (Ts), the ratio of systolic pulmonary artery pressure to the right ventricle outflow tract systolic flow velocity time integral (sPAP/RVOT VTI), and the ratio of systolic pulmonary artery pressure to right ventricle outflow tract systolic flow velocity time integral × heart rate (sPAP/[RVOT VTI×HR]). When the children were divided into three groups based on their pulmonary vascular resistance significant differences emerged that predicted an increasing severity of RV dysfunction. Significant differences were also observed for the RVMPI, the LVMPI, and the Ts as well as for echocardiographic pulmonary flow (Qp) and systemic flow (Qs). Discussion: The present study demonstrates that echocardiographic parameters can be used for the quantitative detection of RV dysfunction in childhood congenital heart disease patients with high pulmonary artery pressure (systolic, diastolic, and mean) or pulmonary vascular resistance.     

Evaluation of muscle strength in patients with spinal muscular atrophy

The first aim of this study was to evaluate the muscular strength in order to document the strength scores for patients with spinal muscular atrophy (SMA). The second aim was to analyze the differences in average strength score among the musculatures at various body parts. Twenty-five patients with type II SMA (mean age of 13.7 +/- 7.0 years) and 15 patients with type III SMA (mean age of 13.7 +/- 8.2 years) were enrolled to undergo a muscle strength evaluation using the method of manual muscle testing. For each patient, the strength of 71 muscle groups were graded using a numerical ordinal scale to obtain individual muscle group scores, average scores of the arms, legs, the musculatures around the limbs' joints, the arm flexors and extensors, and leg flexors and extensors. The data were analyzed using a statistic model of multivariate analysis of variance and paired t-test. All data were administrated respectively for type II and type III SMA. The results showed that in both types of patients, the trunk muscles and the musculatures around the hips possessed lower strength scores, whereas elbow flexors, wrist flexors and extensors, finger flexors, and diaphragms exhibited relatively higher strength scores. In type II SMA the average scores of the musculatures of the left side arms was lower than those of the right side at the joints of scapulas, elbows and wrists. Our data also showed the arm flexor scores were higher than the arm extensor scores in both types, whereas significant higher score of the leg flexor in comparison to the leg extensor was only found in the patients with type II SMA. The profiles of muscle strength and strength differences over various body parts in the patients with type II SMA as well as the type III SMA were obtained from this study.     

Relationship between left ventricular geometry and left ventricular systolic and diastolic functions in patients with chronic severe aortic regurgitation

Background: Chronic aortic regurgitation (AR) is a form of volume overload inducing left ventricle (LV) dilatation. Myocardial fibrosis, apoptosis, progressive LV dilatation, and eventually LV dysfunction are seen with the progression of disease. The aim of the study was to assess the relation between LV geometry and LV systolic and diastolic functions in patients with chronic severe AR. Methods: The study population consisted of 88 patients with chronic severe AR and 42 healthy controls. The LV ejection fraction (LVEF) was calculated. Subjects were divided as Group I (controls, n = 42), Group II (LVEF > 50%, n = 47), and Group III (LVEF < 50%, n = 41). Transmitral early and late diastolic velocities and deceleration time were measured. The annular systolic (Sa) and diastolic (Ea and Aa) velocities were recorded. Diastolic function was classified as normal, impaired relaxation (IR), pseudonormalization (PN), and restrictive pattern (RP). Results: The LVEF was similar in Group I and II, while significantly lower in Group III. Sa velocity was progressively decreasing, but LV long- and short-axis diameters were increasing from Group I to Group III. Forty-six, 31 and 11 patients had IR, PN, and RP, respectively. LV long-axis systolic and diastolic diameters were significantly increasing, while LVEF and Sa velocity were significantly decreasing from patients with IR to patients with RP. The LV long-axis diastolic diameter is independently associated with LV systolic and diastolic functions. Conclusions: The LV long-axis diastolic diameter is closely related with LV systolic and diastolic functions in patients with chronic severe AR.     

Implementing shared medical appointments for heart failure patients in a community cardiology practice: A pilot study

Objective

To examine patient and staff satisfaction, billing charges, and programmatic feasibility of shared medical appointments (SMA) in a nurse practitioner-managed heart failure (HF) clinic in a community cardiology practice.

Methods

Twenty patients were scheduled among four SMAs for this pilot study. All aspects of a usual clinic appointment were utilized during the SMA, but an additional 20-minute teaching session was presented. All patients completed a satisfaction questionnaire. The office staff completed satisfaction questionnaires about the SMA. Billing charges, cancellations and missed appointments without cancellation for SMAs were compared to usual clinic appointment days.

Results

Satisfaction was high among patients and office staff. Rates for no-shows were similar for SMAs versus usual appointments (15% versus 5.5%), but there were fewer cancellations among SMAs (0% versus 28%). This may be a reflection of the personalized appointment reminder calls that were made to the patients scheduled for SMAs. Billing charges were not significantly different for SMAs versus usual appointments.

Conclusion

SMAs are a feasible option in a community cardiology practice.