Prognostic Value of Reduced Heart Rate Reserve during Exercise in Hypertrophic Cardiomyopathy.

BACKGROUND: Sympathetic dysfunction can be evaluated by heart rate reserve (HRR) with exercise test. OBJECTIVES: To determine the value of HRR in predicting outcome of patients with hypertrophic cardiomyopathy (HCM). METHODS: We enrolled 917 HCM patients (age = 49 ± 15 years, 516 men) assessed with exercise stress echocardiography (ESE) in 11 centres. ESE modality was semi-supine bicycle in 51 patients (6%), upright bicycle in 476 (52%), and treadmill in 390 (42%). During ESE, we assessed left ventricular outflow tract obstruction (LVOTO), stress-induced new regional wall motion abnormalities (RWMA), and HRR (peak/rest heart rate, HR). By selection, all patients completed the follow-up. Mortality was the predetermined outcome measure Results: During ESE, RWMA occurred in 22 patients (2.4%) and LVOTO (≥50 mmHg) in 281 (30.4%). HRR was 1.90 ± 0.40 (lowest quartile ≤ 1.61, highest quartile > 2.13). Higher resting heart rate (odds ratio 1.027, 95% CI: 1.018-1.036, p < 0.001), older age (odds ratio 1.021, 95% CI: 1.009-1.033, p < 0.001), lower exercise tolerance (mets, odds ratio 0.761, 95% CI: 0.708-0.817, p < 0.001) and resting LVOTO (odds ratio 1.504, 95% CI: 1.043-2.170, p = 0.029) predicted a reduced HRR. During a median follow-up of 89 months (interquartile range: 36-145 months), 90 all-cause deaths occurred. At multivariable analysis, lowest quartile HRR (Hazard ratio 2.354, 95% CI 1.116-4.968 p = 0.025) and RWMA (Hazard ratio 3.279, 95% CI 1.441-7.461 p = 0.004) independently predicted death, in addition to age (Hazard ratio 1.064, 95% CI 1.043-1.085 p < 0.001) and maximal wall thickness (Hazard ratio 1.081, 95% CI 1.037-1.128, p < 0.001). CONCLUSIONS: A blunted HRR during ESE predicts survival independently of RWMA in HCM patients.

Prognostic role of stress echocardiography in hypertrophic cardiomyopathy: The International Stress Echo Registry.

BACKGROUND: Stress echo (SE) may have a role in the outcome in patients with hypertrophic cardiomyopathy (HCM). OBJECTIVES: The aim was to assess the prognostic value of SE in a retrospective multicenter study in HCM. METHODS: We enrolled 706 HCM patients. The employed stress was exercise (n=608) and/or vasodilator (n=146, dipyridamole in 98 and adenosine in 48). We defined SE positivity according to clinical/hemodynamic criteria including: symptoms (all stress modalities), exercise-induced hypotension (failure to increase or fall >20mmHg, exercise) and exercise-induced left ventricular outflow tract obstruction (left ventricular outflow tract obstruction >50mmHg); and ischemic criteria, such as new wall motion abnormalities (new wall motion abnormality) and/or reduction of coronary flow reserve velocity (CFVR≤2.0) on left anterior descending coronary artery with vasodilator stress assessed in 116 patients. All patients completed the clinical follow-up. RESULTS: Positive SE showed more frequently CFVR reduction, exercise-induced hypotension, left ventricular outflow tract obstruction, and symptoms (38, 23, 20 and 15% respectively), but new wall motion abnormality only in 6%. During a median follow-up of 49months 180 events were observed, including 40 deaths. Clinical/hemodynamic criteria did not predict outcome (X2 0.599, p=0.598), whereas ischemia-related SE criteria (X2: 111.120, p<0.0001) was significantly related to outcome. Similarly, mortality was predicted with SE ischemic-criteria (X2 16.645, p<0.0001). CONCLUSIONS: SE has an important prognostic significance in HCM patients, with ischemia-related end-points showing greater predictive accuracy than hemodynamic endpoints. New wall motion abnormalities and impairment of CFVR should be specifically included in SE protocols for HCM.

Circulating miR-29a, among other up-regulated microRNAs, is the only biomarker for both hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy.

OBJECTIVES: The purpose of this paper was to determine whether microRNAs (miRNAs) involved in myocardial remodeling were differentially expressed in the blood of hypertrophic cardiomyopathy (HCM) patients, and whether circulating miRNAs correlated with the degree of left ventricular hypertrophy and fibrosis. BACKGROUND: miRNAs-small, noncoding ribonucleic acids (RNAs) that regulate gene expression by inhibiting RNA translation-modulate cellular function. Myocardial miRNAs modulate processes such as cardiomyocyte (CM) hypertrophy, excitation-contraction coupling, and apoptosis; non-CM-specific miRNAs regulate myocardial vascularization and fibrosis. Recently, the possibility that circulating miRNAs may be biomarkers of cardiovascular disease has been raised. METHODS: Forty-one HCM patients were characterized with conventional transthoracic echocardiography and cardiac magnetic resonance. Peripheral plasma levels of 21 miRNAs were assessed by quantitative real-time polymerase chain reaction and were compared with levels in a control group of 41 age- and sex-matched blood donors. RESULTS: Twelve miRNAs (miR-27a, -199a-5p, -26a, -145, -133a, -143, -199a-3p, -126-3p, -29a, -155, -30a, and -21) were significantly increased in HCM plasma. However, only 3 miRNAs (miR-199a-5p, -27a, and -29a) correlated with hypertrophy; more importantly, only miR-29a correlated also with fibrosis. CONCLUSIONS: Our data suggest that cardiac remodeling associated with HCM determines a significant release of miRNAs into the bloodstream: the circulating levels of both cardiac- and non-cardiac-specific miRNAs are significantly increased in the plasma of HCM patients. However, correlation with left ventricular hypertrophy parameters holds true for only a few miRNAs (i.e., miR-199a-5p, -27a, and -29a), whereas only miR-29a is significantly associated with both hypertrophy and fibrosis, identifying it as a potential biomarker for myocardial remodeling assessment in HCM.

Speckle-tracking analysis based on 2D echocardiography does not reliably measure left ventricular torsion.

PURPOSE: Worldwide left ventricular (LV) twist is measured by 2D speckle tracking acquiring apical short axis at a LV level where papillary muscles are no longer visible; however, we hypothesized that this methodological recommendation is not enough accurate to obtain a reliable measurement of apical rotation. METHODS: We measured twist and untwist rate in 30 healthy subjects by following the earlier method. By 3D echocardiography, we identified two LV apex levels: (i) 3D Apex, defined as the last apical slice at which LV cavity was visible; (ii) 2D Apex, defined as the level where diameters are equal to those of apical LV short axis used for twist analysis in the same subject. The ratio between the distance of 2D Apex and 3D Apex from LV base was calculated and expressed as percentage (2D Apex/3D Apex). RESULTS: 2D Apex/3D Apex was strongly related to the magnitude of twist and untwisting rate (r = 0·82, P<0·001; r = -0·46, P = 0·015, respectively). The only determinant of twist was 2D Apex/3D Apex (r(2)  = 0·68; r = 0·82; F ratio: 52·6, P<0·001); whereas untwisting rate was influenced by 2D Apex/3D Apex and age (r(2)  = 0·42; r = 0·65; F ratio: 7·7; P = 0·003 for 2D Apex/3D Apex; and P = 0·011 for age). CONCLUSIONS: Left ventricular apical level acquisition, even when recorded in a standard manner, determines variability of twist mechanics measurements. Thus, current anatomical markers used to identify LV apex for twist analysis are not reliable and need different standardization. 3D echocardiography may help in such standardization.

Percutaneous treatment of patients with heart diseases: selection, guidance and follow-up. A review.

Aortic stenosis and mitral regurgitation, patent foramen ovale, interatrial septal defect, atrial fibrillation and perivalvular leak, are now amenable to percutaneous treatment. These percutaneous procedures require the use of Transthoracic (TTE), Transesophageal (TEE) and/or Intracardiac echocardiography (ICE). This paper provides an overview of the different percutaneous interventions, trying to provide a systematic and comprehensive approach for selection, guidance and follow-up of patients undergoing these procedures, illustrating the key role of 2D echocardiography.

Aortic valve sclerosis in patients with peripheral and/or coronary arterial disease.

BACKGROUND: Aortic valve sclerosis (AVS) is a marker of cardiovascular risk; its prevalence increases in elderly and in patients with hypertension and/or coronary arterial disease (CAD). There are no data available in patients with peripheral arterial disease (PAD) and with both CAD and PAD. METHODS: To investigate the presence of AVS, 57 patients with stable CAD, 38 with PAD, and 62 with CAD + PAD where studied by echocardiography. RESULTS: The prevalence of AVS progressively increased within groups (P = 0.005). The prevalence of AVS in PAD doubled that in CAD group (42.1% vs. 22.8%, P < 0.05). PAD patients had a 4.634 (95% CI: 1.02-17.88; P = 0.026) fold increased risk of AVS compared to CAD. Also CAD + PAD group had a higher prevalence of aortic sclerosis when compared to CAD group (50.8% vs. 22.8%, P = 0.001). CAD + PAD showed a 3.799 (95% CI: 1.26-11.45; P < 0 .01) fold greater risk of aortic sclerosis than CAD group. There were no differences in AVS prevalence between CAD + PAD and PAD group (50.8% vs. 42.1%; P = 0.36). Age was related to AVS in both analysis (PAD vs. CAD and CAD + PAD vs. CAD: OR = 1.09, 95% CI: 1.02-1.16, P = 0.011 and OR = 1.13, 95% CI: 1.07-1.21; P < 0.001) but no classical cardiovascular risk factors. CONCLUSIONS: PAD patients have an elevated prevalence of AVS greater than CAD patients. In patients with both disease, the prevalence of AVS is similar to that of patients with PAD alone.

Echocardiography in patients with hypertrophic cardiomyopathy: usefulness of old and new techniques in the diagnosis and pathophysiological assessment.

Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiomyopathy. The identification of patients with HCM is sometimes still a challenge. Moreover, the pathophysiology of the disease is complex because of left ventricular hyper-contractile state, diastolic dysfunction, ischemia and obstruction which can be coexistent in the same patient. In this review, we discuss the current and emerging echocardiographic methodology that can help physicians in the correct diagnostic and pathophysiological assessment of patients with HCM.

Myocardial fibrosis and diastolic dysfunction in patients on chronic haemodialysis.

BACKGROUND: Left ventricular (LV) diastolic dysfunction is linked to myocardial collagen content in many cardiac diseases. There are no data regarding such relationship in patients with end-stage renal disease (ESRD) undergoing haemodialysis. METHODS: Twenty-five patients with ESRD undergoing haemodialysis were studied by echocardiography. LV diastolic function was investigated by Doppler echocardiography, by analysing LV filling velocities at rest and during loading manoeuvres, which represent an estimate of LV filling pressure. According to the Doppler pattern, LV filling pressure in a given patient was judged to be normal or slightly increased or to be moderately or severely increased. The presence of myocardial fibrosis was estimated by ultrasound tissue characterization with integrated backscatter, which in diastole correlates with the collagen content of the myocardium. RESULTS: Integrated backscatter was higher in patients with moderate or severely increased than in patients with normal or slightly increased LV filling pressure (integrated backscatter: 51.0 +/- 9.8 vs 41.6 +/- 5.6%; P = 0.008). Integrated backscatter was a strong and independent determinant of diastolic dysfunction (odds ratio = 1.212; P = 0.040). CONCLUSION: Our data support the hypothesis that, in a selected population of patients with ESRD undergoing haemodialysis, myocardial fibrosis is associated with LV diastolic myocardial properties.

Thrombosis of mechanical valve prosthesis in patient with recent Caesarean delivery.

We present a case of a mechanical mitral valve thrombosis in a 37-year-old woman occurred 2 days after a Caesarean delivery. The patient stopped warfarin and initiated low-molecular-weight heparin 1 week before the programmed delivery. Subsequently the diagnosis of thrombosis, heparin infusion was started however unsuccessfully and eventually patient was referred for cardiac surgery.

Resolution of established cardiac hypertrophy and fibrosis and prevention of systolic dysfunction in a transgenic rabbit model of human cardiomyopathy through thiol-sensitive mechanisms.

BACKGROUND: Cardiac hypertrophy, the clinical hallmark of hypertrophic cardiomyopathy (HCM), is a major determinant of morbidity and mortality not only in HCM but also in a number of cardiovascular diseases. There is no effective therapy for HCM and generally for cardiac hypertrophy. Myocardial oxidative stress and thiol-sensitive signaling molecules are implicated in pathogenesis of hypertrophy and fibrosis. We posit that treatment with N-acetylcysteine, a precursor of glutathione, the largest intracellular thiol pool against oxidative stress, could reverse cardiac hypertrophy and fibrosis in HCM. METHODS AND RESULTS: We treated 2-year-old beta-myosin heavy-chain Q403 transgenic rabbits with established cardiac hypertrophy and preserved systolic function with N-acetylcysteine or a placebo for 12 months (n=10 per group). Transgenic rabbits in the placebo group had cardiac hypertrophy, fibrosis, systolic dysfunction, increased oxidized to total glutathione ratio, higher levels of activated thiol-sensitive active protein kinase G, dephosphorylated nuclear factor of activated T cells (NFATc1) and phospho-p38, and reduced levels of glutathiolated cardiac alpha-actin. Treatment with N-acetylcysteine restored oxidized to total glutathione ratio, normalized levels of glutathiolated cardiac alpha-actin, reversed cardiac and myocyte hypertrophy and interstitial fibrosis, reduced the propensity for ventricular arrhythmias, prevented cardiac dysfunction, restored myocardial levels of active protein kinase G, and dephosphorylated NFATc1 and phospho-p38. CONCLUSIONS: Treatment with N-acetylcysteine, a safe prodrug against oxidation, reversed established cardiac phenotype in a transgenic rabbit model of human HCM. Because there is no effective pharmacological therapy for HCM and given that hypertrophy, fibrosis, and cardiac dysfunction are common and major predictors of clinical outcomes, the findings could have implications in various cardiovascular disorders.

Prognostic significance of left atrial volume dilatation in patients with hypertrophic cardiomyopathy.

To evaluate the prognostic role of left atrial (LA) volume in hypertrophic cardiomyopathy (HCM), LA volume was measured at baseline and during follow-up in 140 patients with HCM. Unfavorable outcome, defined as occurrence of sudden death, heart transplantation, or invasive reduction of obstruction, developed in 16 patients. In patients with enlarged LA volume (>27 mL/m(2)), there was an increased risk for unfavorable outcome (P = .0152). Patients with normal LA volume at baseline in whom volume increased more than 3 mL per year (fast dilating LA volume) had a worse prognosis than patients with normal and stable volume (P < .001) and similar to patients with dilated LA volume at baseline (P = not significant). LA volume dilated at baseline, fast dilating LA volume, and New York Heart Association functional class were independent predictors of unfavorable outcome development (odds ratio: 11.453; P = .021, P = 2.019, P = .020, respectively). The assessment of LA volume at baseline and during follow-up adds information regarding prognosis in patients with HCM.

Diastolic function and BNP changes during exercise predict oxygen consumption in chronic heart failure patients.

UNLABELLED: B-type natriuretic peptide (BNP) is a diagnostic and prognostic marker in heart failure (CHF) patients. AIM: To assess the relation between BNP, diastolic function and exercise capacity in CHF patients. METHODS: Fifty CHF patients underwent cardiopulmonary exercise testing. BNP levels were determined at baseline and at peak exercise. Patients were divided in two groups: with lower (<14 ml/kg/min) or higher (>or=14 ml/kg/min) peak oxygen consumption (VO(2)). RESULTS: Seventeen patients with lower peak VO(2) showed larger incidence of restrictive pattern of the transmitral flow (7/17 vs 4/33, p =0.036). E/Ea ratio was inversely related with peak VO(2) (r =-0.419, p =0.004) and directly related with BNP levels at baseline (r =0.449, p =0.001) and at peak exercise (r =0.475, p =0.001). LV ejection fraction was similar in the two groups. Independent predictors of exercise tolerance were E/Ea ratio (p =0.003), lg BNP at baseline (p =0.034) and increase in lg BNP during exercise (p =0.038). CONCLUSIONS: In CHF patients, E/Ea ratio is a predictor of exercise tolerance and is related with BNP level at rest and at peak exercise.

Abnormal blood-pressure response to exercise and oxygen consumption in patients with hypertrophic cardiomyopathy.

BACKGROUND: Abnormal blood-pressure response during exercise occurs in about one third of patients with hypertrophic cardiomyopathy (HCM), and it has been associated with a high risk of sudden cardiac death. We assessed the hemodynamics of exercise in HCM patients with abnormal blood-pressure response by using ambulatory radionuclide monitoring (VEST) of left-ventricular (LV) function, and exercise tolerance by oxygen consumption. METHODS: Twenty-two HCM patients underwent treadmill exercise during VEST monitoring. A cardiopulmonary exercise test was performed a few days after. The VEST data were averaged for 1 minute. Stroke volume, cardiac output, and systemic vascular resistance were expressed as percent of baseline. Exercise tolerance was assessed as maximal oxygen consumption. RESULTS: In eight HCM patients (36%) with an abnormal blood-pressure response, end-systolic volume increased more (52% +/- 21% vs 31% +/- 28%, P = .012), and the ejection fraction (-31% +/- 17% vs -14% +/- 22%, P = .029) and stroke volume (-21% +/- 21% vs 3% +/- 28%, P = .026) fell more, than in patients with normal response. Cardiac output increased less in the former patients (49% +/- 44% vs 94% +/- 44%, P = .012). Systemic vascular resistance decreased similarly, irrespective of blood-pressure response (-28% +/- 26% vs -34% +/- 26%, P = N.S.). Percent of maximal predicted oxygen consumption was lower in HCM patients with an abnormal blood-pressure response (63% +/- 11% vs 78% +/- 15%, P = .025). CONCLUSIONS: In HCM patients, abnormal blood-pressure response was associated with exercise-induced LV systolic dysfunction and impairment in oxygen consumption. This may cause hemodynamic instability, associated with a high risk of sudden cardiac death.

Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy.

CONTEXT: Recently, the implantable cardioverter-defibrillator (ICD) has been promoted for prevention of sudden death in hypertrophic cardiomyopathy (HCM). However, the effectiveness and appropriate selection of patients for this therapy is incompletely resolved. OBJECTIVE: To study the relationship between clinical risk profile and incidence and efficacy of ICD intervention in HCM. DESIGN, SETTING, AND PATIENTS: Multicenter registry study of ICDs implanted between 1986 and 2003 in 506 unrelated patients with HCM. Patients were judged to be at high risk for sudden death; had received ICDs; underwent evaluation at 42 referral and nonreferral institutions in the United States, Europe, and Australia; and had a mean follow-up of 3.7 (SD, 2.8) years. Measured risk factors for sudden death included family history of sudden death, massive left ventricular hypertrophy, nonsustained ventricular tachycardia on Holter monitoring, and unexplained prior syncope. MAIN OUTCOME MEASURE: Appropriate ICD intervention terminating ventricular tachycardia or fibrillation. RESULTS: The 506 patients were predominately young (mean age, 42 [SD, 17] years) at implantation, and most (439 [87%]) had no or only mildly limiting symptoms. ICD interventions appropriately terminated ventricular tachycardia/fibrillation in 103 patients (20%). Intervention rates were 10.6% per year for secondary prevention after cardiac arrest (5-year cumulative probability, 39% [SD, 5%]), and 3.6% per year for primary prevention (5-year probability, 17% [SD, 2%]). Time to first appropriate discharge was up to 10 years, with a 27% (SD, 7%) probability 5 years or more after implantation. For primary prevention, 18 of the 51 patients with appropriate ICD interventions (35%) had undergone implantation for only a single risk factor; likelihood of appropriate discharge was similar in patients with 1, 2, or 3 or more risk markers (3.83, 2.65, and 4.82 per 100 person-years, respectively; P = .77). The single sudden death due to an arrhythmia (in the absence of advanced heart failure) resulted from ICD malfunction. ICD complications included inappropriate shocks in 136 patients (27%). CONCLUSIONS: In a high-risk HCM cohort, ICD interventions for life-threatening ventricular tachyarrhythmias were frequent and highly effective in restoring normal rhythm. An important proportion of ICD discharges occurred in primary prevention patients who had undergone implantation for a single risk factor. Therefore, a single marker of high risk for sudden death may be sufficient to justify consideration for prophylactic defibrillator implantation in selected patients with HCM.

Myocardial texture in hypertrophic cardiomyopathy.

BACKGROUND: This study was conceived to assess associations between integrated backscatter signal at end diastole (IBS) and diastolic properties in patients with hypertrophic cardiomyopathy. METHODS: In 46 patients with hypertrophic cardiomyopathy, septal IBS was calculated by both applying an appropriate regression correction (IBSc) and by relating it to pericardial reflectivity (IBSp). Difference in duration between transmitral forward and pulmonary venous backward velocities (A-Ar) was measured as an estimate of passive diastolic filling. In all, 38 patients underwent ambulatory electrocardiogram monitoring for 48 hours. RESULTS: IBS inversely correlated to both A-Ar (IBSc, r = -.522, P < .001; IBSp, r = -.302, P = .041) and mitral peak velocity at atrial contraction (IBSc, r = -.464, P = .002; IBSp, r = -.413, P = .004). Moreover, IBS was greater in patients with sustained or nonsustained ventricular tachycardia (IBSc, 28.5 +/- 3.8 vs 25.4 +/- 3.8 dB, P = .034). CONCLUSIONS: Septal IBS correlates with Doppler parameters of left ventricular chamber stiffness in patients with hypertrophic cardiomyopathy. Increased IBS is associated with presence of ventricular arrhythmias.

Prognostic significance of left atrial size in patients with hypertrophic cardiomyopathy (from the Italian Registry for Hypertrophic Cardiomyopathy).

This study assessed left atrial (LA) dimension as a potential predictor of outcome in hypertrophic cardiomyopathy (HC). From the Italian Registry for Hypertrophic Cardiomyopathy, 1,491 patients (mean age 47 +/- 17 years; 61% men; 19% obstructive), followed for 9.4 +/- 7.4 years after the initial echocardiographic evaluation, constituted the study group. The mean LA transverse dimension was 43 +/- 9 mm and was larger in patients with severe symptoms (48 +/- 9 mm for New York Heart Association classes III and IV vs 42 +/- 9 mm for classes I and II, p <0.001), atrial fibrillation (47 +/- 9 vs 42 +/- 8 mm in sinus rhythm, p <0.001), and left ventricular outflow obstruction (46 +/- 9 mm for >or=30 mm Hg at rest vs 42 +/- 9 mm for <30 mm Hg at rest, p <0.001). On univariate analysis, each 5-mm increase in LA size was associated with a hazard ratio (HR) of 1.2 for all-cause mortality (p <0.0001). On multivariate analysis, a LA dimension >48 mm (the 75th percentile) had a HR of 1.9 for all-cause mortality (p = 0.008), 2.0 for cardiovascular death (p = 0.014), and 3.1 for death related to heart failure (p = 0.008) but was unassociated with sudden death (p = 0.81). Similar results were obtained after the exclusion of patients with atrial fibrillation (HR 1.7, p = 0.008) or outflow obstruction (HR 1.8, p = 0.003). The predictive power of LA dimension >48 mm was also validated in an independent HC cohort from the United States, with similar HRs (1.8 for all-cause mortality, p = 0.019). In conclusion, in a large cohort of patients with HC from a nationwide registry, a marked increase in LA dimension were predictive of long-term outcome, independent of co-existent atrial fibrillation or outflow obstruction. LA dimension is a novel and independent marker of prognosis in HC, particularly relevant to the identification of patients at risk for death related to heart failure.

Effect of hypertrophy on left ventricular diastolic function in patients with hypertrophic cardiomyopathy.

BACKGROUND.: Hypertrophic cardiomyopathy (HCM) is characterized by asymmetric LV hypertrophy (LVH) and impairment in diastolic function. We assess the relationship between LVH and invasive indexes of diastolic function. METHODS.: 21 HCM patients underwent cardiac catheterization to assess pulmonary capillary wedge pressure, LV end-diastolic pressure (measured by microtip catheters), and LV volumes (calculated by simultaneous radionuclide angiography). We calculated from LV pressure the time constant of isovolumetric relaxation (τ, variable asymptote method, ms), and from LV pressure and volume the constant of chamber stiffness (k, ml(-1)). LVH was assessed by different indexes: maximal wall thickness, number of hypertrophied LV segments, LVH index, and Wigle's score. Results. Wigle's score was directly related to pulmonary capillary Wedge pressure (r=0.436, p=0.048), peak V wave of pulmonary capillary wedge pressure (r=0.503, p=0.024), LV end-diastolic pressure (r=0.643, p=0.002) and k (r=0.564, p=0.015). HCM patients were divided into 2 groups according to Wigle's score: 10 with mild or moderate LVH (< 8), and 11 with severe LVH (≥ 8). HCM patients with severe LVH showed a higher pulmonary capillary Wedge pressure (15.1±7.2 vs 9.5±2.4, p=0.033), peak V wave of pulmonary capillary wedge pressure (20.7±4.6 vs 14.6±4.9, p=0.011), LV end-diastolic pressure (23.9±10.9 vs 10.6±2.5, p=0.002), k (0.0465±0.032 vs 0.015±0.007, p=0.022) and LV outflow tract gradient (72±36 mmHg vs 29±30 mmHg, p=0.01).τ was similar in the two groups. Other indexes of LVH were not related to diastolic function. CONCLUSIONS.: Wigle's score is the only index of LVH that relates to invasive indices of diastolic function.

The Italian Registry for hypertrophic cardiomyopathy: a nationwide survey.

BACKGROUND: National registries are advocated as instrumental to the solution of rarity-related problems for patients with hypertrophic cardiomyopathy (HCM), including limited access to advanced treatment options. Thus, an Italian Registry for HCM was created to assess the clinical profile and the level of care nationwide of patients with HCM. METHODS: Cardiology centers over the national territory were recruited to provide clinical data of all patients with HCM ever seen at each institution. The enrollment period was from May 2000 to May 2002. RESULTS: The registry enrolled 1677 patients from 40 institutions. Most (69%) were followed at referral centers, whereas 31% were from community centers with intermediate-low patient flow. Patients diagnosed after routine medical examinations or familial screenings were 39%. Most patients were male (62%), in their fourth to sixth decade of life, and in New York Heart Association class I to II (89%); 24% had resting left ventricular obstruction and 18% had atrial fibrillation. During a 9.7-year average follow-up, cardiovascular mortality was 1%/y, mostly because of heart failure, with no significant change over the last 3 decades; sudden death was less common (0.4%/y). Only 4% of patients received a defibrillator; 14% of the 401 patients with LV outflow obstruction underwent invasive relief of obstruction; and <1% were offered genetic analyses or counseling. CONCLUSIONS: The Italian Registry represents the first comprehensive attempt to evaluate the clinical impact and management of HCM at a national level. Findings underscore the role of screening strategies for an early diagnosis and suggest limited use of the advanced therapeutic options for HCM.

Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy.

OBJECTIVES: This study sought to determine the impact of surgical myectomy on long-term survival in hypertrophic cardiomyopathy (HCM). BACKGROUND: Left ventricular (LV) outflow tract obstruction in HCM increases the likelihood of heart failure and cardiovascular death. Although surgical myectomy is the primary treatment for amelioration of outflow obstruction and advanced drug-refractory heart failure symptoms, its impact on long-term survival remains unresolved. METHODS: Total and HCM-related mortality were compared in three subgroups comprised of 1,337 consecutive HCM patients evaluated from 1983 to 2001: 1) surgical myectomy (n = 289); 2) LV outflow obstruction without operation (n = 228); and 3) nonobstructive (n = 820). Mean follow-up duration was 6 +/- 6 years. RESULTS: Including two operative deaths (procedural mortality, 0.8%), 1-, 5-, and 10-year overall survival after myectomy was 98%, 96%, and 83%, respectively, and did not differ from that of the general U.S. population matched for age and gender (p = 0.2) nor from patients with nonobstructive HCM (p = 0.8). Compared to nonoperated obstructive HCM patients, myectomy patients experienced superior survival free from all-cause mortality (98%, 96%, and 83% vs. 90%, 79%, and 61%, respectively; p < 0.001), HCM-related mortality (99%, 98%, and 95% vs. 94%, 89%, and 73%, respectively; p < 0.001), and sudden cardiac death (100%, 99%, and 99% vs. 97%, 93%, and 89%, respectively; p = 0.003). Multivariate analysis showed myectomy to have a strong, independent association with survival (hazard ratio 0.43; p < 0.001). CONCLUSIONS: Surgical myectomy performed to relieve outflow obstruction and severe symptoms in HCM was associated with long-term survival equivalent to that of the general population, and superior to obstructive HCM without operation. In this retrospective study, septal myectomy seems to reduce mortality risk in severely symptomatic patients with obstructive HCM.