ypertrophic cardiomyopathy (HCM) is the most common cause of sudden death (SD) in young people, including trained athletes. 1 The highest risk for SD in HCM has been associated with any of the following noninvasive clinical markers: 2-6 prior cardiac arrest or spontaneous sustained ventricular tachycardia (VT); family history of premature SD; syncope; nonsustained ventricular tachycardia (NSVT) on ambulatory (Holter) ECG recordings; abnormal blood pressure response (ABPR) to exercise; and extreme left ventricular (LV) hypertrophy with maximum wall thickness ≥3 cm. There are limited reports on echocardiographic predictors of adverse outcomes in patients with HCM, and all these are confined to pediatric patients. 7,8 Conventional Doppler echocardiographic indices of LV function are unreliable in predicting clinical outcome in patients with HCM, primarily because of their dependence on loading conditions. 9,10 Tissue Doppler (TD) velocities are relatively load-independent and have been used in studies of diastolic function in HCM. [11][12][13][14][15][16][17] The purpose of the present study was to determine whether TD velocities can predict the risk of sustained VT, cardiac arrest, implantable cardioverter defibrillator (ICD)-discharge, or SD in patients with HCM. Circulation Journal Vol.71, June 2007 Methods PatientsWe prospectively studied 105 consecutive patients with HCM who were assessed from February 1992 to October 2006 at AHEPA Hospital, Thessaloniki, Greece, and who had completed all noninvasive tests for risk stratification assessment (ie, personal and family history, clinical evaluation, 12-lead ECG, transthoracic echocardiography, 24-h ECG monitoring, and symptom-limited upright exercise test). This protocol has been applied in all patients with HCM since 2002. In 7 patients who were first evaluated before 2002, baseline data were considered to be those obtained after 2002. The study inclusion criterion was the presence of normal sinus rhythm at the time of TD imaging study. Ninety six patients met these criteria and comprised our study cohort. Nine patients were excluded due to chronic atrial fibrillation. All patients had a heart rate <90 beats/min at the time of first evaluation. Seven patients (7.2%) had a LV fractional shortening ≤25% at the time of first evaluation, suggesting LV remodeling with systolic impairment. 18,19 The diagnosis of HCM was based on LV maximum wall thickness (MWT) ≥15 mm on echocardiography, in the absence of any other cause capable of producing such hypertrophy. 20,21 HCM was also considered present in patients with MWT 13 or 14 mm in the presence of a positive family history for HCM and/or ECG changes compatible with HCM. The follow-up period started from the time of the initial assessment, even if the diagnosis preceded initial patient evaluation in hospital. All patients gave written informed consent and the protocol of this study was reviewed and Circ J 2007; 71: 897 -903 (Received January 24, 2007; revised manuscript received March 7, 2007; accepted March 12, 2007 ...
Systemic sclerosis (SSc) is a connective tissue disease, which may lead to elevated pulmonary arterial pressure due to pulmonary arterial hypertension and/or left ventricular diastolic dysfunction. Uric acid (UA) has been shown to be elevated in patients with pulmonary hypertension (PH) and heart failure. We aimed to investigate the potent relationship between serum UA and pulmonary pressure as well as functional capacity in patients with SSc. We studied 66 patients (mean age 57.7±12.1 years, 63 women), presenting with SSc. Systolic pulmonary artery pressure assessed by echocardiography, lung function tests, six-minute walk test (6MWT) and serum UA levels were recorded in all patients. In 24 (36%) patients the diagnosis of PH was established by echocardiography (systolic pulmonary artery pressure ≥40mmHg). PH patients had higher UA serum levels compared to patients without PH (5.1±2.1 mg/dl vs 4.2±0.9 mg/dl, p = 0.04). Amongst PH patients, UA values were inversely correlated with the SMWT distance (r = -0.51, p = 0.01). Serum UA values increased in proportion to the functional capacity PH in patients with scleroderma. Further investigations in prospective studies will unfold in detail the pathophysiological significance of UA in SSc patients with PH, and determine its role as a prognostic marker in the assessment and monitoring of the disease.
Early detection of pulmonary hypertension (PH) in patients with systemic sclerosis (SSc) is essential as it leads to substantial morbidity and mortality irrespective of its etiology. The aim of our study was to determine whether noninvasive biochemical and/or echocardiographic indices can predict the presence of PH in these patients. We prospectively studied 66 patients (mean age of 57.7 +/- 12.1 years, 63 women) with SSc without clinical manifestations of heart failure. All patients underwent standard and tissue Doppler echocardiography. Plasma N-terminal pro-B type natriuretic peptide (NT-proBNP) and asymmetric dimethylarginine (ADMA) levels were also measured. In 24 (36%) patients, the diagnosis of PH was established by echocardiography (systolic pulmonary artery pressure value > or =40 mmHg). Left atrial (LA) volume, NT-proBNP, ADMA, ratio of early transmitral filling velocity to early diastolic velocity of the mitral annulus (mitral E/E (m)), and right ventricular myocardial performance index (MPI) were univariate predictors of PH. In multivariate analysis, NT-proBNP, LA volume, and right ventricular MPI were independent predictors of PH in SSc patients. LA volume and NT-proBNP may be useful noninvasive markers for the prediction of elevated pulmonary artery pressure in patients with SSc. These parameters should be considered when assessing this population for risk stratification and for identification of patients demanding further investigation and institution of specific therapy for the disease at the time when it is most likely to be effective.
hronic heart failure (CHF) has emerged as the most prevalent cause of mortality, morbidity and hospitalization in industrialized countries over the past years. 1 Despite the advances in medical and invasive treatment of CHF, its incidence is expected to rise in the future because of improved survival rates following acute myocardial infarction (MI), which is the principal etiology of CHF in >70% of patients. 2 Traditionally, great emphasis was put on evaluation of the various etiologic factors of this syndrome. Differentiation between ischemic cardiomyopathy (ICM) and idiopathic dilated cardiomyopathy (DCM) has important therapeutic implications because the former may gain substantial benefit from coronary revascularization. 3 Conventional echocardiography is commonly used for the evaluation of left ventricular (LV) and right ventricular (RV) function, but does not provide detailed information about myocardial systolic and diastolic properties in the regional segments. Tissue Doppler echocardiography (TDE) is a promising ultrasonographic technique that quantitatively measures the velocity of the myocardium in systole and diastole. 4 Traditionally, the principal target of the cardiologist is the evaluation of the LV function, with little interest in the RV. In spite of the fact that several recent studies have used the TDE technique in the assessment of RV function in various clinical conditions, 5,6 the literature on the RV and its impact on the pathophysiologic processes is limited. However, there is a growing body of evidence that RV function is a powerful predictor of mortality in patients with CHF. 7 We hypothesized that ICM and DCM differ in terms of RV function. Thus, the aim of our study was to investigate the RV function in patients with ICM and DCM using pulsed wave TDE and to compare the TDE parameters of the RV among the patients with ischemic and idiopathic dilated causes of LV dysfunction. Methods Study PopulationWe prospectively studied 82 consecutive patients with CHF who had undergone an echocardiographic examination at the AHEPA Hospital (Thessaloniki, Greece). CHF was defined as the presence of heart failure symptoms plus a dilated (>55 mm in end-diastolic diameter) LV with a depressed (<40%) ejection fraction of uncertain origin. The inclusion criteria were: (a) good quality echocardiographic imaging of tricuspid and mitral annular motion, (b) adequate tricuspid valve regurgitation Doppler signal in order to assess pulmonary artery systolic pressure, (c) sinus rhythm on electrocardiography (ECG), and (d) stable clinical condition. The following were considered as exclusion criteria:
BackgroundHypertrophic cardiomyopathy (HCM) is an extremely heterogeneous disease. An under recognized and very often missed subgroup within this broad spectrum concerns patients with left ventricular (LV) apical aneurysms in the absence of coronary artery disease.Case presentationWe describe a case of HCM with midventricular obstruction and apical aneurysm formation in 3 patients coming from a single family. This HCM pattern was detected by 2D-echocardiography and confirmed by cardiac magnetic resonance imaging. A cardioverter defibrillator was implanted in one of the patients because of non-sustained ventricular tachycardia detected in 24-h Holter monitoring and an abrupt drop in systolic blood pressure during maximal exercise test. The defibrillator activated 8 months after implantation by suppression of a ventricular tachycardia providing anti-tachycardia pacing. The patient died due to refractory heart failure 2 years after initial evaluation. The rest of the patients are stable after a 2.5-y follow-up period.ConclusionThe detection of apical aneurysm by echocardiography in HCM patients may be complicated. Ventricular tachycardia arising from the scarred aneurysm wall may often occur predisposing to sudden death.
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