Edmundo Arteaga

Value of Real Time Three-Dimensional Echocardiography in Patients with Hypertrophic Cardiomyopathy: Comparison with Two-Dimensional Echocardiography and Magnetic Resonance Imaging

Real time three‐dimensional echocardiography (RT3DE) has been demonstrated to be an accurate technique to quantify left ventricular (LV) volumes and function in different patient populations. We sought to determine the value of RT3DE for evaluating patients with hypertrophic cardiomyopathy (HCM), in comparison with cardiac magnetic resonance imaging (MRI). Methods: We studied 20 consecutive patients with HCM who underwent two‐dimensional echocardiography (2DE), RT3DE, and MRI. Parameters analyzed by echocardiography and MRI included: wall thickness, LV volumes, ejection fraction (LVEF), mass, geometric index, and dyssynchrony index. Statistical analysis was performed by Lin agreement coefficient, Pearson linear correlation and Bland‐Altman model. Results: There was excellent agreement between 2DE and RT3DE (Rc = 0.92), 2DE and MRI (Rc = 0.85), and RT3DE and MRI (Rc = 0.90) for linear measurements. Agreement indexes for LV end‐diastolic and end‐systolic volumes were Rc = 0.91 and Rc = 0.91 between 2DE and RT3DE, Rc = 0.94 and Rc = 0.95 between RT3DE and MRI, and Rc = 0.89 and Rc = 0.88 between 2DE and MRI, respectively. Satisfactory agreement was observed between 2DE and RT3DE (Rc = 0.75), RT3DE and MRI (Rc = 0.83), and 2DE and MRI (Rc = 0.73) for determining LVEF, with a mild underestimation of LVEF by 2DE, and smaller variability between RT3DE and MRI. Regarding LV mass, excellent agreement was observed between RT3DE and MRI (Rc = 0.96), with bias of−6.3 g (limits of concordance = 42.22 to−54.73 g). Conclusion: In patients with HCM, RT3DE demonstrated superior performance than 2DE for the evaluation of myocardial hypertrophy, LV volumes, LVEF, and LV mass.

Obstructive Sleep Apnea Is Common and Independently Associated With Atrial Fibrillation in Patients With Hypertrophic Cardiomyopathy

BACKGROUND Hypertrophic cardiomyopathy (HCM) is associated with arrhythmias and cardiovascular death. Left atrial enlargement and atrial fibrillation (AF) are considered markers for death due to heart failure in patients with HCM. Obstructive sleep apnea (OSA) is independently associated with heart remodeling and arrhythmias in other populations. We hypothesized that OSA is common and is associated with heart remodeling and AF in patients with HCM. METHODS We evaluated 80 consecutive stable patients with a confirmed diagnosis of HCM by sleep questionnaire, blood tests, echocardiography, and sleep study (overnight respiratory monitoring). RESULTS OSA (apnea-hypopnea index [AHI] > 15 events/h) was present in 32 patients (40%). Patients with OSA were significantly older (56 [41-64] vs 38.5 [30-53] years, P < .001) and presented higher BMI (28.2 +/- 3.5 vs 25.2 +/- 5.2 kg/m(2), P < .01) and increased left atrial diameter (45 [42-52.8] vs 41 [39-47] mm, P = .01) and aorta diameter (34 [30-37] vs 29 [28-32] mm, P < .001), compared with patients without OSA. Stepwise multiple linear regression showed that the AHI (P = .05) and BMI (P = .06) were associated with left atrial diameter. The AHI was the only variable associated with aorta diameter (P = .01). AF was present in 31% vs 6% of patients with and without OSA, respectively (P < .01). OSA (P = .03) and left atrial diameter (P = .03) were the only factors independently associated with AF. CONCLUSIONS OSA is highly prevalent in patients with HCM and it is associated with left atrial and aortic enlargement. OSA is independently associated with AF, a risk factor for cardiovascular death in this population.

Primary Neoplasms of the Heart. Clinical and Histological Presentation of 50 Cases

OBJECTIVE To analyze clinical and histologic findings of 50 patients with primary neoplasms of the heart in a tertiary referral center. METHODS 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). RESULTS 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. CONCLUSION Primary cardiac tumors are a rare entity with diverse clinical and histologic findings, requiring, therefore, a high level of clinical suspicion.

Management of aortic dissection that involves the right coronary artery

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.

Self‐Expanding Endovascular Stent‐Graft Implant for Treatment of Descending Aortic Diseases

AbstractBackground: 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. Methods: 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). Results: 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. Conclusions: 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.

Relationship between outflow obstruction and left ventricular functional impairment in hypertrophic cardiomyopathy: a Doppler echocardiographic study.

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 ≥ 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.

Sleep Quality and Quality of Life in Patients with Hypertrophic Cardiomyopathy

Objectives: To evaluate clinical predictors of poor sleep quality and quality of life (QOL) in patients with hypertrophic cardiomyopathy (HCM). Methods: 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. Results: 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. Conclusion: 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.

Myocardial fibrosis in patients with hypertrophic cardiomyopathy and high risk for sudden death

FUNDAMENTO: A estratificacao de risco para morte subita na cardiomiopatia hipertrofica (CMH), continua a ser um verdadeiro desafio devido a grande heterogeneidade da sua apresentacao, em que a maioria dos individuos permanecem assintomaticos por toda sua vida e outros apresentam a morte subita como primeiro sintoma. Recentes trabalhos vem sugerindo que a fibrose miocardica pode constituir-se em um importante substrato para as arritmias ventriculares malignas, responsaveis pela morte subita nesta doenca. OBJETIVO: Avaliacao da prevalencia e quantificacao da fibrose miocardica (FM), em pacientes com CMH com alto risco ou recuperados de morte subita, portadores de cardiodesfibrilador implantavel (CDI). METODOS: Vinte e oito pacientes com CMH portadores de CDI foram submetidos a tomografia computadorizada com multiplos detectores, para realizacao da tecnica de realce tardio, e avaliacao da fibrose miocardica. RESULTADOS: 96% dos pacientes apresentavam fibrose miocardica (20,38 ± 15,55 gramas) correspondendo a 15,96 ± 10,20% da massa miocardica total. A FM foi significativamente mais prevalente que os demais fatores de risco classicos para morte subita. CONCLUSAO: Concluimos que existe uma alta prevalencia de fibrose miocardica em pacientes com cardiomiopatia hipertrofica de alto risco ou recuperados de morte subita, como neste grupo - portadores de cardiodesfibrilador implantavel. A maior prevalencia da fibrose miocardica comparada aos fatores de risco de pior prognostico levantam a hipotese de que a fibrose miocardica possa ser um importante substrato potencialmente necessario na genese das arritmias desencadeadoras da morte subita.BACKGROUND The stratification of risk for sudden death in hypertrophic cardiomyopathy (HCM) continues to be a true challenge due to the great heterogeneity of this diseases presentation, as most individuals remain asymptomatic during their entire lives and others present sudden death as first symptom. Recent studies have suggested that myocardial fibrosis may represent an important substrate for the malignant ventricular arrhythmias, that are responsible for the cases of sudden death related to this disease. OBJECTIVE To assess the prevalence and quantification of myocardial fibrosis (MF) in hypertrophic cardiomyopathy (HCM) patients with implantablecardioverter - defibrillator (ICD) indicated due to their high risk or recovered from cardiac sudden death. METHODS Twenty-eight HCM patients with ICD were submitted to multidetector computed tomography to assess myocardial fibrosis by delayed enhancement technique. RESULTS Myocardial fibrosis was present in 96% of these HCM patients with (20.38 +/- 15.55 g) comprising 15.96 +/- 10.20% of the total myocardial mass. MF was observed in a significantly higher prevalence as compared to other classical risk factors for sudden death. CONCLUSION It is possible to conclude that there is a high prevalence of myocardial fibrosis in hypertrophic cardiomyopathy patients with high-risk or recovered from cardiac sudden death, like those with clinical indication to implantable cardioverter -defibrillator. The higher prevalence of myocardial fibrosis in comparison to classical risk factors of worse prognosis raise the hypothesis that the myocardial fibrosis may be an important substrate in the genesis of lifethreatening arrhythmias in these high risk HCM population.

Cardiomiopatia hipertrófica: importância dos eventos arrítmicos em pacientes com risco de morte súbita

OBJECTIVE: To evaluate, in patients with hypertrophic cardiomyopathy and risk for SCD who underwent implantable cardioverter-defibrillator (ICD) implantation: a- the occurrence of arrhythmic events; b- the occurrence of clinical events and their correlation with arrhythmic events; c- the occurrence of ICD shock therapy and clinical and functional correlations; d- clinical and functional predictors of prognosis. METHODS: Twenty six patients with hypertrophic cardiomyopathy and risk factors for SCD undergoing ICD implantation from May, 2000 to January, 2004 (mean follow-up = 20 months) were studied. Fourteen patients (53.8%) were females and the mean age was 42.7 years. ICD was indicated for primary prevention of sudden cardiac death in 16 patients (61.5%), and for secondary prevention in 10 patients (38.5%). Twenty patients (76.9%) presented syncope prior to ICD implantation; half of them were related to ventricular fibrillation or sustained ventricular tachycardia, 15 (57.7%) had a history of familial sudden death, 12 patients (46.2%) had nonsustained ventricular tachycardia on the 24-hour Holter monitoring, and five (19.2%) had an interventricular septal thickness greater than 30 mm. RESULTS: Four shock therapies were recorded by the ICD in potentially lethal arrhythmias (three patients with sustained ventricular tachycardia and one patient with ventricular fibrillation) during the follow-up. One death occurred, probably due to a thromboembolic stroke. Four patients had recurrence of syncope with no arrhythmic event recorded by the ICD. The statistical analysis showed a significant difference in early ICD shock therapy in patients whose interventricular septal thickness was greater than 30 mm. CONCLUSION: 1- occurrence of arrhythmic events in 50% of the patients; most of them (62%) were ventricular tachycardia, whether sustained (31%) or nonsustained (31%); in the remaining patients paroxysmal supraventricular tachycardia was observed. 2- recurrent syncope in the minority of the patients (16%), however not associated with the presence of arrhythmic events. 3- the presence of an interventricular septal thickness greater than 30 mm in the echocardiogram was associated with early shock therapy (p = 0.003). 4- absence of clinical or functional predictors.

Clinical Meaning of Ascites in Patients with Endomyocardial Fibrosis

OBJECTIVE To evaluate the clinical meaning of ascites and the main features of patients with ascites and endomyocardial fibrosis. METHODS We studied 166 patients with endomyocardial fibrosis (mean age 37 years, 114 women) treated over the last 20 years. Ventriculography findings, surgery or necropsy confirmed the diagnosis in all patients. Most patients belonged to New York Heart Association Functional Class III/IV (134, 83.7%). Eighty-one (50.6%) had biventricular, 28 (17.5%) had right ventricular, and 51 (31.8%) had left ventricular involvement. During follow-up, 56 patients died. RESULTS Ascites was present in 67 (41.8%) patients, and right ventricular involvement was present in 59 (88%). In the comparison between patients with or without ascites, those with ascites had higher mortality (49.2% and 24.7%, respectively). Patients with ascites had a higher incidence of edema (95% vs. 43%), hepatomegaly (5.8cm vs. 4.1cm), mean right atrium pressure (19.3 vs. 12mmHg), and final right ventricle diastolic pressure (18.7 vs. 12.9mmHg). Also, patients with ascites had a longer history of illness (5.1 and 3.9 years, respectively) and had atrial fibrillation more frequently (44.7% vs. 30.1%). CONCLUSION Ascites was observed in less than 50% of cases of endomyocardial fibrosis and was associated with greater involvement of the right ventricle and with a longer duration of the disease, thus being a characteristic of a worse prognosis.