Edmundo Arteaga

Myocardial fibrosis (MF) occurs in up to 80% of subjects with asymptomatic or mildly symptomatic hypertrophic cardiomyopathy (HCM) and can constitute an arrhythmogenic substrate for re-entrant, life-threatening ventricular arrhythmias in predisposed persons. The aim was to investigate whether MF detected by delayed enhancement cardiac CT is predictive of ventricular tachycardia (VT) and fibrillation (VF) that require appropriate therapy by an implantable cardioverter defibrillator (ICD) in patients with HCM. Twenty-six patients with HCM with previously (for at least 1 year) implanted ICD underwent MF evaluation by cardiac CT. MF was quantified by myocardial delayed enhanced cardiac CT. Data on ICD firing were recorded every 3 months after ICD implantation. Risk factors for sudden cardiac death in patients with HCM were evaluated in all patients. MF was present in 25 of 26 patients (96%) with mean fibrosis mass of 20.5 ± 15.8 g. Patients with appropriate ICD shocks for VF/VT had significantly greater MF mass than patients without (29.10 ± 19.13 g vs 13.57 ± 8.31 g; P = .01). For a MF mass of at least 18 g, sensitivity and specificity for appropriate ICD firing were 73% (95% CI, 49%-88%) and 71% (95% CI, 56%-81%), respectively. Kaplan-Meier curves indicated a significantly greater VF/VT event rate in patients with MF mass ≥18 g than in patients with MF <18 g (P = .02). In the Cox regression analysis, the amount of MF was independently associated with VF/VT in ICD-stored electrograms. The mass of MF detected by cardiac CT in patients with HCM at high risk of sudden death was associated with appropriate ICD firings.

Chagas' disease is a known dilated form of cardiomyopathy. However, a great number of patients, although showing electrocardiographic (ECG) well-recognized changes, maintain normal ventricular chamber dimensions and are asymptomatic. The aim of the present study was to objectively characterize functional capacity in asymptomatic patients with Chagas' disease and normal left ventricular function. Eighteen asymptomatic male patients with Chagas' disease, aged 33+/-6 years, were selected for the study. All showed ECG changes typical of the disease, as well as left ventricular fractional shortening (LVFS) greater than 0.30 on M-mode 2-dimensionally guided echocardiography. Twenty sedentary normal male patients, aged 29+/-6 years, served as controls. Both groups were submitted to ergoespirometry testing for assessment of exercise functional capacity. Patients with Chagas' disease, when compared with controls, showed lower (P < .001) maximal O2 consumption (VO2max, 24.3+/-4.2 v 37.0+/-5.4 mL x kg(-1) x min(-1) respectively); O2 pulse rate (PO2max, 10.5+/-1.4 v 15.1+/-2.5 mL/beat, respectively); maximal ventilation (VEmax, 50.1+/-13.5 v 113.0+/-17.6 L x min(-1), respectively); anaerobic threshold of maximal O2 consumption (VO2-AT, 15.8+/-3.6 v 24.6+/-4.7 mL x kg(-1) x min(-1), respectively); and maximal heart rate (HRmax, 154+/-21 v 186+/-7 beat x min(-1), respectively). Asymptomatic patients with Chagas' disease, although presenting normal left ventricular systolic function at rest, display a substantial impairment of exercise functional capacity.

... Sao Paulo Medical School, Sp, Bra) ARTEAGA E (Univ. Sao Paulo Medical School, Sp, Bra) BUCK P (Univ. Sao Paulo Medical School, Sp, Bra) RABELLO R (Univ. Sao Paulo Medical School, Sp, Bra) MADY C (Univ. Sao Paulo Medical School, Sp, Bra). Journal Title;J Card Fail. ...

We report a case of a 26-year-old woman who presented to our hospital with arrhythmia and heart failure. She had an incessant supraventricular tachycardia, which was not reversible with electrical cardioversion. Echocardiogram showed a severe LV systolic and diastolic dysfunction. After radiofrequency catheter ablation, LV function returned to normal. This article is intended to show a case with tachycardiomyopathy, which is considered the most frequently unrecognized curable cause of heart failure, and to demonstrate that early treatment allows the recovery to a normal LV systolic and diastolic function, preventing irreversible structural cardiac damage. It is very likely that some patients with idiopathic dilated cardiomyopathy and chronic atrial fibrillation or other chronic arrhythmia actually have a curable tachycardiomyopathy.

Left ventricular outflow tract (LVOT) obstruction is predictive of a worse outcome in hypertrophic cardiomyopathy (HCM). In a detailed Doppler echocardiographic study of 178 selected HCM patients, the group of patients (n = 73) with the obstructive form (resting peak gradient > or = 30 mmHg) presented more hypertrophy and poorer systolic and diastolic left ventricular (LV) functions than the HCM group (n = 105) without obstruction. LVOT peak gradient was positively correlated with hypertrophy (P < 0.0001) and negatively to tissue Doppler mitral annulus systolic (P = 0.0001) and early diastolic (P < 0.0001) velocities. The gradient significantly correlated with E/Ea ratio (r = 0.67; P < 0.0001). By multiple regression, LVOT gradient was related to E/Ea, LV maximal thickness and left atrial size. In comparison with patients without obstruction, patients with obstruction presented greater hypertrophy (P < 0.0001), lower systolic and early diastolic mitral annulus velocities (both P < 0.0001), higher E/Ea ratio (P < 0.0001) and higher global function index (P < 0.0001). In HCM, beyond the effects on hypertrophy, LVOT obstruction is an independent determinant of LV functional abnormalities.

A global function index (GFI) derived from tissue Doppler imaging (TDI) has been proposed to improve the diagnosis of hypertrophic cardiomyopathy (HCM). We aimed to evaluate the usefulness of this index in a large selected HCM population. GFI =[E/Ea]/Sa, was calculated at mitral annulus lateral and septal borders in 164 HCM patients and in 40 healthy volunteers. Group comparisons and correlations between GFI and other variables were performed. Of the 164 patients, 69 (42%) had a peak gradient >30 mmHg in the left ventricle outflow tract (LVOT). GFI (lateral or septal) was not normally distributed. There were differences among controls, obstructive HCM, and nonobstructive HCM (P < 0.0001), but significant overlap of GFI values were observed between groups. GFI was correlated to septal thickness (r = 0.44; P < 0.0001), left atrial diameter (r = 0.52; P < 0.0001), and LVOT gradient (r = 0.58; P < 0.0001). In a selected HCM population, GFI was limited by its asymmetrical distribution and significant overlap of values between groups. Further studies are necessary to verify the reliability of GFI in the clinical practice and its position among other tissue Doppler indices.

... Paulo Harada, Fernando Morita, Edmundo Arteaga, Afonso Akio Shiozaki, Jussara Bianchi Castelli Instituto do Coração (InCor) - HC-FMUSP, São Paulo, SP - Brasil Paciente do sexo feminino de 19 anos de idade, com diagnóstico de cardiomiopatia hipertrófica, foi ...

The hypertrophic cardiomyopathy is a primary cardiac disease, characterized by the hypertrophy of the left ventricle, without dilatation, in the absence of any other cardiac or systemic disease that may lead to the myocardial hypertrophy 1 . The estimate prevalence of the disease in ...

To analyze clinical and histologic findings of 50 patients with primary neoplasms of the heart in a tertiary referral center. From 1980 to 1998, we retrospectively analyzed 50 patients, 32 of whom were females, whose ages ranged from 9 to 73 years (mean age = 44.16+/-18 years). Most tumors were located in the left side of the heart (72%), myxoma being the most common (84%) histologic type. The other histologic types found were as follows: fibroma (4%), lipoma (2%), rhabdomyosarcoma (2%), hemangioma (2%), sarcoma (2%), angiosarcoma (2%), and lymphoma (2%). Diagnosis was established by echocardiography in 94% of the cases. Clinical findings were as follows: dyspnea (36%), weight loss (20%), palpitations (18%), chest pain (16%), fever (8%), and arthralgia (6%). All patients with thromboembolic phenomena (10%) had left atrial myxoma. Approximately 20% of the patients were asymptomatic at the initial clinical assessment. Primary cardiac tumors are a rare entity with diverse clinical and histologic findings, requiring, therefore, a high level of clinical suspicion.

During a diagnostic investigation in a 40-year-old male with pericardial effusion associated with hypothyroidism, cholesterol pericarditis was detected. We report a brief review on the etiopathogeny, clinical findings, and therapeutical possibilities of this entity.

To characterize patients with neoplastic pericardial disease diagnosed by clinical presentation, complementary test findings, and the histological type of tumor. Twenty-six patients with neoplastic pericardial disease were retrospectively analyzed. Clinical manifestations and abnormalities in chest roentgenograms and electrocardiograms were frequent, but were not specific. Most patients underwent surgery. There was a high positivity of the pericardial biopsy when associated with the cytological analysis of the pericardial liquid used to determine the histological type of the tumor, particularly when the procedure was performed with the aid of pericardioscopy. The correct diagnosis of neoplastic pericardial disease involves suspicious but nonspecific findings during clinical examination and in screen tests. The suspicious findings must be confirmed through more invasive diagnostic approaches, in particular pericardioscopy with biopsy and cytological study.

In hypertrophic cardiomyopathy (HC), diastolic dysfunction of the left ventricle is a prominent feature caused by myocardial hypertrophy and fibrosis. Angiotensin II has trophic and profibrotic effects on the heart, and the blockade of angiotensin II receptors reverses hypertrophy and fibrosis in human cardiac diseases and in animal HC. This study investigated the short-term (6 months) effects of losartan 100 mg/day in 20 patients with nonobstructive HC, with an emphasis on left ventricular (LV) diastolic dysfunction, compared with 10 patients with HC who were not treated. At the final evaluation, significant changes were observed in the losartan group: a left atrial diameter decrease (p<0.0001), a tissue Doppler early (Ea) mitral annulus diastolic velocity increase (p=0.003) and an E/Ea ratio decrease (p=0.0002), and a significant decrease in plasma levels of the aminoterminal fragment of pro-brain natriuretic peptide (NT-pro-BNP) from a median of 860 to 606 pg/ml (p=0.001). A significant correlation was found between percentage changes in NT-pro-BNP and the E/Ea ratio from baseline to 6 months (r=0.61, p=0.002). In the 2 groups, echocardiographic LV wall and cavity measures did not change. In conclusion, in selected patients with nonobstructive HC, losartan during a 6-month period improved LV diastolic function.

Selected patients with hypertrophic cardiomyopathy (HCM) have 3% to 4% annual mortality as compared to only 0.5% to 1.5% in nonselected patients. Our aim was to evaluate survival and prognostic factors in HCM in patients in a tertiary care center. From 1980 to 1997, 214 patients were prospectively studied, with a mean follow-up of 7 years (range 1-25 years); there were 102 male and 112 female patients, aged 37 +/- 16 years (range 3-76 years). All patients had 12-lead electrocardiogram, 24-hour Holter monitor, and surface echocardiography. Univariate analysis was performed for known adverse factors such as young age, family history, syncope, functional class, atrial fibrillation, ventricular hypertrophy, left ventricular outflow tract obstruction, and nonsustained ventricular tachycardia. There were 22 deaths (10%), 15 directly related to HCM (sudden in 11). The cumulative survival rates were 94.5% at 5 years, 91% at 10 years, and 87.9% at 15 years. The annual mortality rate was 1%. Only New York Heart Association functional class III/IV and maximal ventricular wall thickness >30 mm were associated with HCM-related cardiac death. We concluded that even a referred population of HCM patients may have a relatively benign outcome. Prognosis is related to advanced functional class and degree of left ventricular hypertrophy.

Plasma B-type natriuretic peptide (BNP) is a sensitive functional marker in heart disease including hypertrophic cardiomyopathy (HCM). The utility of plasma amino-terminal pro-BNP (NT-proBNP) quantification in heart disease has been investigated, but there are no published data regarding this test in HCM. Plasma NT-proBNP was assessed in 71 patients with HCM and in 40 healthy subjects. Symptomatic status was assessed according to the New York Heart Association classification. M-mode and Doppler echocardiographic data were obtained in all patients and healthy subjects to study their correlations and comparisons (Spearman and Mann-Whitney tests). Median NT-proBNP was 848 pg/mL in patients and 28 pg/mL in the control group (P < .0001). Patients in New York Heart Association functional class I/II had a median NT-proBNP of 669 pg/mL as compared with 3357 pg/mL for patients in class III/IV (P < .0001). Amino-terminal pro-BNP levels correlated positively with left atrial diameter (r = 0.40, P = .0005), septal thickness (r = 0.35, P = .002), and mitral flow velocity/mitral annulus velocity (E/Ea) ratio (r = 0.42, P < .0001). There was a weak correlation with obstruction (r = 0.23, P = .05), and a significant difference in the medians was observed between obstructive (1651 pg/mL) and nonobstructive (669 pg/mL) HCM groups (P = .01). Patients with Doppler E/Ea ratios > or = 10 had higher NT-proBNP levels than patients with E/Ea < 10 (P < .0001). Multivariate analysis showed that NT-proBNP correlated independently with left atrial diameter (P < .01), hypertrophy (P < .01), and E/Ea (P < .01). In HCM, plasma NT-proBNP levels are elevated and correlate positively with symptoms of heart failure, hypertrophy severity, and Doppler echocardiographic signs of left ventricular diastolic dysfunction. Further studies are necessary to assess the usefulness of the test in clinical practice and its role as a prognostic marker.

The aim of this study was to analyze the relationships among exercise capacity (EC), hypertrophy, and diastolic function in nonobstructive hypertrophic cardiomyopathy (NOHCM). Twenty-seven patients with NOHCM were studied. Left ventricular hypertrophy (LVH) was determined by appropriate echocardiographic indexes. For diastolic function evaluation, the following were measured: the early (E) and late (A) waves, E/A, and deceleration time of E of the mitral flow; the systolic (S), diastolic (D), and atrial reversal (AR) waves, S/D, and the atrial systolic filling fraction of the pulmonary vein flow; and the early (Ea), late (Aa) waves, Ea/Aa, and E/Ea by tissue Doppler imaging. The difference between the duration of AR and A waves (DurAR - DurA), the peak VO2, and anaerobic threshold (AT) were also determined. In these patients, the E/Ea ratio was 8.9 +/- 3.2 and DurAR - DurA was 22.6 +/- 32.6 milliseconds. The peak VO2 and AT correlated with D (r = 0.55, P = .003 and .51, P = .007, respectively) and Ea/Aa (r = 0.56, P = .007 and .45, P = .03, respectively). There was no correlation between EC and LVH. Patients with NOHCM demonstrated evidences of elevated left ventricular (LV) end-diastolic pressure with normal filling pressure. EC compromise may be attributed to relaxation changes with inadequate filling of the left ventricle.

Experimental studies demonstrate that infection with trypanosoma cruzi causes vasculitis. The inflammatory lesion process could hypothetically lead to decreased distensibility of large and small arteries in advanced Chagas' disease. We tested this hypothesis. We evaluated carotid-femoral pulse-wave velocity (PWV) in 53 Chagas' disease patients compared with 31 healthy volunteers (control group). The 53 patients were classified into 3 groups: 1) 16 with indeterminate form of Chagas' disease; 2) 18 with Chagas' disease, electrocardiographic abnormalities, and normal systolic function; 3) 19 with Chagas' disease, systolic dysfunction, and mild-to-moderate congestive heart failure. No difference was noted between the 4 groups regarding carotid-femoral PWV (8.4 +/- 1.1 vs 8.2 +/- 1.5 vs 8.2 +/- 1.4 vs 8.7 +/- 1.6 m/s, P = 0.6) or pulse pressure (39.5 +/- 7.6 vs 39.3 +/- 8.1 vs 39.5 +/- 7.4 vs 39.7 +/- 6.9 mm Hg, P = 0.9). A positive, significant, similar correlation o...

Real-time 3-dimensional echocardiography is a recently developed imaging technique that provides unique information on spatial geometry in real time. We described an asymptomatic patient with hypertrophic obstructive cardiomyopathy for whom 3-dimensional echocardiography was performed after intravenous injection of perfluorocarbon-filled microbubbles. It resulted in enhancement of the left ventricular endocardial border delineation and myocardial perfusion in the hypertrophic septum. A clear visualization of the entire course of the left anterior descending coronary artery and its septal perforator branches was obtained. This case illustrates the potential of real-time 3-dimensional echocardiography to improve the anatomic evaluation of coronary arteries, especially when combined with contrast agents.

A report on the follow-up of 11 patients who suffered from aortic dissection involving the right coronary artery and who underwent surgical treatment is reported. In two patients, the left coronary ostia was also affected. In seven patients, the dissection was acute and in four, chronic. The ascending aorta was substituted by a Dacron graft in all patients, and right coronary artery saphenous vein bypass or Gore-Tex graft to the coronary ostia or right coronary artery was performed in nine, and reimplantation of both dissected coronary in two. There were three early postoperative deaths (27.3%) caused by low-output syndrome and myocardial infarction. There were two late deaths. The six surviving patients were followed-up from 78 to 96 months (mean 83 months). This experience suggests that although carrying a high risk, the involvement of the coronary ostia in aortic dissection can be successfully managed if made before irreversible complications arise.

Aneurysms and dissections involving the descending thoracic aorta and the distal portion of the aortic arch are difficult to resolve surgically. The introduction of endovascular self-expanding stent-grafts has simplified the operation. Given the complications associated with their peripheral placement, we explored the feasibility of surgical insertion. Thirteen patients underwent surgical insertion of a stent-graft into the aortic arch via longitudinal aortotomy. Six patients had aneurysms (ruptured in two, and seven dissections (acute in two, ruptured in one). Five patients also underwent associated procedures including aortic valve replacement (one), ascending aorta replacement (two), arch replacement (one), and coronary artery bypass (one). There was one intraoperative death due to ascending aortic dissection, and two hospital deaths due to multiple complications. Of ten patients discharged, one died 3 months postoperatively. The remaining survivors are well, and imaging studies confirmed adequate correction of the aortic disease. The use of this technique simplifies the operation and treatment of particular cases of aortic disease. The observed morbidity and mortality are due to factors independent of the technique.

To evaluate clinical predictors of poor sleep quality and quality of life (QOL) in patients with hypertrophic cardiomyopathy (HCM). Consecutive stable patients with HCM were evaluated for the risk of obstructive sleep apnea (OSA) by the Berlin Questionnaire, daytime sleepiness by the Epworth Sleepiness Scale, sleep quality by the Pittsburgh Sleep Questionnaire Index and QOL by the Minnesota Living with Heart Failure Questionnaire. Asymptomatic subjects without HCM were used as controls. We studied 84 patients with HCM and 42 controls who were similar with regard to gender (49 vs. 50% males), age [52 (38-62) vs. 47 (33-58) years] and body mass index (27 ± 4 vs. 27 ± 5). HCM diagnosis, high risk for OSA and female gender were independently associated with poor sleep quality in the entire population. Among patients with HCM, poor QOL was independently associated with poor sleep quality, New York Heart Association functional class and diuretic therapy. Poor sleep quality is very common in patients with HCM and may have a negative impact on the QOL, which in turn is an important marker of prognosis in patients with cardiomyopathies.