Clinical course of hypertrophic cardiomyopathy in a non selected population. The Experience of the Italian Multicenter Cardiomyopathy Study

BACKGROUND: Most of the information available on the clinical course and prognosis of hypertrophic cardiomyopathy (HCM) is based on data generated from international referral centres and as a result, it constitutes a potentially biased perspective of the disease process in this complex and diverse condition. A multicentric study was therefore set up with the aim of providing information on unselected patient populations with HCM. METHODS: The study group comprised 330 patients from 5 non-referral hospitals (mean age 42 +/- 16 years, M/F 226/104, 74-22%-obstructive, 299-91%-in NYHA class I-II) who were followed up regularly for 9.5 +/- 5.6 years. RESULTS: The vast majority of patients (n = 272, 82%) remained asymptomatic or mildly symptomatic during the follow-up period, whereas the remaining patients (n = 58, 18%) experienced clinical deterioration or died. Of the 18 patients (5%) who died of cardiovascular causes related to hypertrophic cardiomyopathy, 14 had progressive congestive heart failure and only 4 died suddenly. The annual mortality rate for cardiovascular disease was 0.57%, while the mortality rate due to sudden cardiac death was only 0.1%. The cumulative survival rate was 98, 95 and 93%, at 5, 10 and 15 years of follow-up respectively. Atrial fibrillation proved to be a relatively common (n = 81, 24%) and particularly unfavourable clinical feature, with higher mortality rate for cardiovascular causes related to hypertrophic cardiomyopathy. Syncope occurred in 47 patients (14%) but did not appear to have prognostic significance. CONCLUSIONS: In an unselected population, hypertrophic cardiomyopathy had a relatively benign prognosis that was inconsistent with its prior characterization as a generally progressive disorder, based primarily on the experience of selected referral institutions. Sudden unexpected cardiac death was distinctly uncommon, although a sizable proportion of patients, particularly the subset prone to atrial fibrillation, did experience clinical deterioration.     

Hypertrophic cardiomyopathy: spontaneous course

Hypertrophic cardiomyopathy is a relatively rare (prevalence approximately 0.2%), primary myocardial disorder with an autosomal pattern of inheritance, characterized by mostly asymmetric left ventricular hypertrophy with myocyte and myofibrillar disarray. To date, about 34 mutations of the beta-cardiac myosin heavy chain gene have been described and shown to have prognostic implications. The disease has an annual mortality rate of 3%, related to both sudden cardiac death and progressive systolic dysfunction. Not only diastolic but also progressive systolic dysfunction with cavity dilatation occurs in a minority of patients with severe hypertrophy during the long-term course. Sudden death often occurs in young, asymptomatic or mildly symptomatic patients. The degree of hypertrophy and the presence of a pressure gradient are of little prognostic significance. Nonsustained ventricular tachycardia is associated with a poor prognosis in the presence of a history of syncope.     

Peroxidase activity of liver microsomal vitamin D 25-hydroxylase and cytochrome P450 1A2 catalyzes 25-hydroxylation of vitamin D3 and oxidation of dopamine to aminochrome

OBJECTIVES: This study sought to determine the long-term risk of sudden cardiac death in patients with hemodynamically stable sustained ventricular tachycardia complicating coronary artery disease. BACKGROUND: The prognosis and risk of sudden cardiac death in patients with a history of myocardial infarction and ventricular tachyarrhythmias have not been clearly defined. Prior studies are limited by a short follow-up period and by inclusion of patients with heterogeneous cardiac diseases and presenting arrhythmias. METHODS: A retrospective cohort analysis was performed on data from 124 patients, followed up for a mean of 36 +/- 30 months, who received electrophysiologically guided therapy for hemodynamically stable ventricular tachycardia after remote myocardial infarction. RESULTS: Seventy-eight patients were treated pharmacologically (medical group), and 46 patients underwent map-guided subendocardial resection (surgical group). Nine patients (7.3%) died suddenly, 5 (4.0%) died of noncardiac causes, 9 (7.3%) died of a perioperative complication, and 20 (23.4%) died of other cardiac causes. At 1, 2 and 3 years, sudden death occurred at cumulative rates of 2 +/- 1%, 3 +/- 2% and 7 +/- 3%, whereas total mortality was 20 +/- 4%, 28 +/- 4% and 32 +/- 5% (mean +/- SD). Sudden cardiac death (p = 0.047) and total mortality (p = 0.036) were higher in patients with multivessel disease and were similar for both treatment groups. CONCLUSIONS: Although the overall mortality in postinfarction patients presenting with hemodynamically stable ventricular tachycardia treated with electrophysiologically guided antiarrhythmic therapy is high, the risk of sudden death in these patients appears to be low (average 2.4%/year).     

Influence of left ventricular function on survival and mode of death after implantable defibrillator therapy (Cleveland Clinic Foundation and Montefiore Medical Center experience)

To determine the influence of left ventricular (LV) function on survival and mode of death in patients with an implantable cardioverter-defibrillator (ICD), sudden death, surgical mortality, total arrhythmia-related death, total cardiac death and total death were retrospectively evaluated in 377 consecutive patients. The outcomes were also compared between patients with an LV ejection fraction > or = 30% (214 patients, group 1) and < 30% (148 patients, group 2). Surgical mortality was 3.9% (1.8% in group 1, 7% in group 2). During the follow-up of 25 +/- 20 months, actuarial survival rates of all patients at 3 years were 96% for sudden deaths, 81% for total cardiac deaths and 74% for total mortality. When the 2 groups were compared, survival rates of groups 1 and 2 at 3 years, respectively, were 99 and 90% for sudden death (p < 0.05), 97 and 84% for sudden death and surgical mortality (p < 0.01), 94 and 80% for the total arrhythmia-related death (p < 0.001), 88 and 68% for total cardiac death (p < 0.0001), and 81 and 62% for total mortality (p < 0.002). In group 2, 73% of total cardiac deaths within 1 year were causally related to the arrhythmia. Thus, in patients with an ICD, sudden death rates were very low. However, total cardiac death and total death rates were relatively higher. The outcomes of patients with an ICD were strongly influenced by the degree of LV dysfunction.(ABSTRACT TRUNCATED AT 250 WORDS)     

The implantable cardioverter/defibrillator

Despite all advances in the diagnosis and therapy of cardiovascular diseases, the mortality from malignant ventricular tachyarrhythmias is still a major health problem. In addition to established therapeutic strategies in the prevention of sudden cardiac death such as antiarrhythmic drug treatment, catheter ablation or antiarrhythmic drug treatment, cardioverter/defibrillator was introduced to clinical practice in 1980. The number of 50,000 overall implants reflects the current clinical status of the therapy with implantable cardioverter/defibrillators. Significant technical improvements in the defibrillator therapy may contribute to an increase in therapy acceptance. These advances include the introduction of nonthoracotomy lead systems, enhanced defibrillation efficacy, full programmable devices providing tiered electrical therapy, improved diagnostic Holter functions and enhanced arrhythmia detection algorithms. The major present goals of defibrillator therapy are detection and termination of malignant ventricular tachyarrhythmias, prevention of sudden cardiac death, reduction in patient's mortality and improvement in quality of life. The efficacy and safety of defibrillator therapy to prevent sudden arrhythmic death has been proven in several large clinical investigations In patients with this device the annual sudden cardiac death mortality is < 2% even in high-risk patient populations. Compared to sudden cardiac death rate there is a much higher rate of overall cardiac mortality because a defibrillator is not able to prevent nonarrhythmic cardiovascular deaths. There is a clinical impression that cardiovascular mortality is lower in patients treated with an implantable cardioverter/defibrillator compared to patients treated with other therapies. However, there are no results from controlled studies providing scientific evidence that defribillator therapy can decrease overall cardiovascular mortality.(ABSTRACT TRUNCATED AT 250 WORDS)     

Molecular and cellular changes in vein grafts: influence of pulsatile stretch

Sudden cardiac death is frequent in patients with dilated cardiomyopathy. To assess the risk of an arrhythmic event is still difficult. Here the analysis of the heart rate variability offers new possibilities. METHOD: 25 patients (18 males, 7 females, age 53 +/- 9 yrs) with dilated cardiomyopathy were included in the study. Analysis of heart rate variability assessed by time- and frequency-domain measures was determined from Holter recording. The mean follow-up was 18 +/- 5 months. RESULTS: 6 patients died (5 of sudden cardiac death, 1 of heart failure), 1 patient with an implanted defibrillator received an adequate shock. Parameters influenced by low- and mid-frequent oscillations of the heart rate were significantly lower in patients who died suddenly or had adequate shocks. The best predictive parameter was the s.d.RR: all patients with an s.d.RR < 50 ms had lethal arrhythmias whereas the s.d.RR of the surviving patients was > or = 50 ms. No significant difference was found or high frequency parameters, which are mainly influenced by parasympathetic activity. CONCLUSION: The analysis of heart rate variability is of prognostic relevance in patients with dilated cardiomyopathy. Especially the s.d.RR is able to identify patients with a high risk of a sudden cardiac death.     

The impact of reversible gonadal sex steroid suppression on serum leptin concentrations in children with central precocious puberty

OBJECTIVE: Evaluation of clinical course and outcome of hypertrophic cardiomyopathy in a representative Greek population. BACKGROUND: Hypertrophic cardiomyopathy is characterized by unexplained left ventricular hypertrophy and varied clinical expression. Recent studies suggest ethnic differences. MATERIALS AND METHODS: One hundred seventy-four consecutive Greek patients (117 male, 57 female, age 47+/-16 years) from 143 different families were assessed at the Department of Cardiology of the University of Athens, Greece, and the State Cardiac Department, Hippokration Hospital, both located in Athens, Greece. To reduce selection bias, referral was based on disease diagnosis irrespective of clinical status or treatment needs. All patients were examined clinically, echocardiographically, and by ECG ambulatory monitoring at 6-month intervals for a period of 74+/-22 months (range, 8 to 108 months). RESULTS: Most patients (n = 156, 89.7%) were in New York Heart Association (NYHA) functional class I or II. The disease was familial (at least one affected first-degree relative) in 81 of the 143 families (56.6%), and in 19 of these (13.3%) there was familial history of sudden cardiac death. At initial examination, intraventricular septal thickness was 17.3+/-4.1 mm and posterior wall thickness was 13.7+/-3.8 mm and a left ventricular outflow gradient >30 mm Hg was present in 58 patients (33.3%). Similar were the findings during the last examination (17.5+/-4.3 mm, 13.5+/-4.4 mm, and 56 (32.2%, respectively, p = not significant). Episodes of nonsustained ventricular tachycardia were noted in 15 patients (8.6%). There were eight deaths during follow-up: four sudden deaths and four from intractable heart failure. Syncope was reported by all patients who died. The annual mortality in this study was 1%. Syncope and NYHA class were the only predictors of outcome. CONCLUSIONS: In this representative Greek patient cohort with hypertrophic cardiomyopathy, the arrhythmogenic substrate was modest and the clinical course benign. Sudden cardiac death was infrequent and syncope, functional class, and ventricular arrhythmias were the only predictors of a poor outcome.     

Bronchodilator response to salbutamol after chronic dosing with salmeterol or placebo

OBJECTIVES: We report the occurrence of cardiac events during long-term follow-up in patients with hypertrophic cardiomyopathy (HCM) after cardioverter-defibrillator implantation. BACKGROUND: The identification of patients at high risk for sudden death and the prevention of recurrence of sudden death in HCM represents a difficult problem. METHODS: We retrospectively analyzed the occurrence of cardiac events during follow-up of 13 patients with HCM who received an implantable cardioverter-defibrillator (ICD) because of aborted sudden death (n = 10) or sustained ventricular tachycardia (n = 3) (group I). Findings were compared with those in 215 patients with an ICD and other structural heart disease or idiopathic ventricular fibrillation (group II). RESULTS: After a mean (+/-SD) follow-up period of 26+/-18 months, 2 of 13 patients in group I received appropriate shocks. The calculated cumulative incidence of shocks was 21% in group I and 66% in group II after 40 months (p < 0.05). We observed a low incidence of recurrence of ventricular tachycardia/fibrillation during follow-up in patients with HCM. No deaths occurred. CONCLUSIONS: Our data suggest that ventricular tachyarrhythmias may not always be the primary mechanism of syncope and sudden death in patients with HCM. The ICD seems to have a less important impact on prognosis in patients with HCM than in patients with other etiologies of aborted sudden death.     

A population study of the natural history of Wolff-Parkinson-White syndrome in Olmsted County, Minnesota, 1953-1989

BACKGROUND: Virtually all natural history studies of Wolff-Parkinson-White (WPW) syndrome have been case series and, as such, have been constrained by referral biases, skewed age and sex distributions, or brief follow-up periods. The purpose of our study was to examine the natural history, the development of arrhythmias, and the incidence of sudden death in an entire cohort of pediatric and adult WPW patients from a community-based local population. METHODS AND RESULTS: We identified 113 residents of Olmsted County, Minnesota, during the period 1953-1989 using the centralized records-linkage system provided by the Mayo Clinic and the Rochester Epidemiology Program Project. Medical records and ECGs were reviewed to confirm the diagnosis and to establish pathway location by ECG criteria. Follow-up, via record review and telephone interview, was complete in 95% of subjects through 1990. The incidence of newly diagnosed cases was approximately four per 100,000 per year. Preexcitation was not present on the initial ECG of 22% of the cohort. Approximately 50% of the population was asymptomatic at diagnosis, with 30% subsequently having symptoms related to arrhythmia at follow-up. Two sudden cardiac deaths (SCD) occurred over 1,338 patient-years of follow-up, yielding an overall SCD rate of 0.0015 (95% confidence interval, 0.0002-0.0054) per patient-year. No SCD occurred in patients asymptomatic at diagnosis. CONCLUSIONS: The incidence of sudden death in a local community-based population is low and suggests that electrophysiological testing should not be performed routinely in asymptomatic patients with WPW syndrome. Nevertheless, young, asymptomatic patients, particularly those < 40 years old, should return for medical follow-up should symptoms develop.     

A clinical and pathological study of 46 cases of sudden and unexpected death

A series of 46 autopsied adult cases of sudden and unexpected natural death were investigated. In this study, sudden and unexpected death was defined as any death occurring with 24 hours of onset of symptoms in a person with or without probable cause of death suggested by medical history. The cases included 31 males and 15 females aged 26 to 85 years (mean 66.6 years). Age distribution peaked in seventies. The lesions causing sudden and unexpected death according to the most frequent organ systems were, diseases of the heart (acute myocardial infarction with or without old infarct, 20; old myocardial infarction without acute infarction, 2; dilated cardiomyopathy, 2; sarcoidosis, 1; amyloidosis, 2; and valvular disease, 2), the aorta (ruptured aneurysm, 6; dissecting aneurysm, 2), the respiratory tract (pulmonary embolism, 7; pulmonary hypertension, 1), the alimentary tract (intestinal obstruction, 1), and other diseases (cause unknown, 1). The cardiovascular lesions were found in 78.2% of cases autopsied. The sudden and unexpected death caused by acute myocardial infarction was found in 47.8%, and acute myocardial infarction seemed to play a major role in cardiac sudden death in these series. The respiratory lesions were found in 17.4%. Four of seven cases with pulmonary embolism died in two weeks after surgical operation. The most common underlying disease was post-operative condition.     

Sudden cardiac death in the adult with congenital heart disease

Preliminary results of a review of sudden cardiac death in the adult with congenital heart disease were presented at the Canadian Adult Congenital Heart (CACH) Network meeting during the Canadian Cardiovascular Society's annual meeting in October 1994. Of 125 patients who were known to have died, sufficient details were available for 92 to determine the circumstance of death. Sudden death occurred in 23 patients (estimated incidence 5.3 per 1000 patients followed per year) at an average age of 33.5 +/- 11.9 years. Surgical procedures included intracardiac repair in 12, palliative procedures in only six and no cardiac surgery in six. Nine patients with sudden death had Eisenmenger syndrome. Right or left ventricular abnormalities were present in 15 of 21 patients with premorbid echocardiographic evaluation. A prior history of ventricular arrhythmia was available in only three patients. Sudden death is a significant cause of mortality in adults with congenital heart disease. Determination of risk factors will be an important aspect of the patient database under development by the CACH Network.     

Okadaic acid-stimulated degradation of p35, an activator of CDK5, by proteasome in cultured neurons

BACKGROUND: Implantable cardioverter-defibrillators have been infrequently used in children as therapy for resuscitated sudden death and syncope due to ventricular arrhythmias unresponsive to antiarrhythmics. METHODS: The medical records of 5 children with implantable cardioverter-defibrillators were retrospectively reviewed. All patients had experienced syncope and 3 (60%) an out-of-hospital cardiac arrest. Underlying pathology included hypertrophic cardiomyopathy in 2, long QT syndrome in 2, and ventricular arrhythmia after remote repair of congenital heart disease in 1. Open thoracotomy with epicardial lead placement and transvenous endocardial approaches were used. RESULTS: There was no early or late mortality in the 5 pediatric patients undergoing implantable cardioverter-defibrillator placement. Postoperative complications occurred more frequently when open thoracotomy was used for placement. At mean follow-up of 34 months, 4 of the 5 (80%) have received shocks. CONCLUSIONS: Implantable cardioverter-defibrillator is a safe and reliable therapy for children with resuscitated sudden death and syncope due to ventricular tachycardia unresponsive to antiarrhythmics. Transvenous lead placement lowers morbidity and hospital length of stay.     

Long-term results of visually guided left ventricular reconstruction as single therapy to treat ventricular tachycardia associated with postinfarction anteroseptal aneurysm

INTRODUCTION: Postinfarction ventricular tachycardia (VT), anteroseptal aneurysm, and ventricular dysfunction are commonly associated and predict a poor long-term prognosis. Surgical left ventricular reconstruction, which includes double plication of the anterior and septal wall, can improve ventricular function. This article analyzes the long-term efficacy of such a procedure to control recurrence of VT in a group of 50 consecutive patients. METHODS AND RESULTS: The study group consisted of 50 consecutive patients operated on between December 1986 and December 1994. The group comprised 44 men and 6 women. The mean age was 56+/-11 years. All patients had spontaneous VT following an anterior myocardial infarction. Twenty-five patients had two or more episodes of VT (eight presented as cardiac arrest, nine as syncope). Coronary artery disease was limited to the left anterior descending artery in 27 patients. An anteroseptal aneurysm was present in 49 patients. All patients had VT induced by programmed ventricular stimulation before surgery, and left ventricular reconstruction was performed without intraoperative mapping in all cases. Total mortality, VT recurrence, and sudden death rate were the endpoints of the study. In-hospital mortality was 8%. Postoperative left ventricular ejection fraction improved from 0.38 to 0.50 (P<0.05). Only two patients had postoperative inducible VT. Overall survival, VT recurrence rate, and sudden death rate were 73%, 12%, and 10%, respectively, after a median follow-up period of 6.25 years (0 to 8 years). CONCLUSION: Visually guided left ventricular reconstruction with septal and anterior wall plicature can be utilized effectively to treat recurrent VT associated with postinfarction anteroseptal aneurysm.     

Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men

BACKGROUND: Between 1981 and 1988, the Centers for Disease Control and Prevention reported a very high incidence of sudden death among young male Southeast Asians who died unexpectedly during sleep. The pattern of death has long been prevalent in Southeast Asia. We carried out a study to identify the clinical markers for patients at high risk of developing sudden unexplained death syndrome (SUDS) and long-term outcomes. METHODS AND RESULTS: We studied 27 Thai men (mean age, 39.7+/-11 years) referred because they had cardiac arrest due to ventricular fibrillation, usually occurring at night while asleep (n=17), or were suspected to have had symptoms similar to the clinical presentation of SUDS (n=10). We performed cardiac testing, including EPS and cardiac catheterization. The patients were then followed at approximately 3-month intervals; our primary end points were death, ventricular fibrillation, or cardiac arrest. A distinct ECG abnormality divided our patients who had no structural heart disease (except 3 patients with mild left ventricular hypertrophy) into two groups: group 1 (n=16) patients had right bundle-branch block and ST-segment elevation in V1 through V3, and group 2 (n=11) had a normal ECG. Group 1 patients had well-defined electrophysiological abnormalities: group 1 had an abnormally prolonged His-Purkinje conduction time (HV interval, 63+/-11 versus 49+/-6 ms; P=.007). Group 1 had a higher incidence of inducible ventricular fibrillation (93% for group 1 versus 11% for group 2; P=.0002) and a positive signal-averaged ECG (92% for group 1 versus 11% for group 2; P=.002), which was associated with a higher incidence of ventricular fibrillation or death (P=.047). The life-table analysis showed that the group 1 patients had a much greater risk of dying suddenly (P=.05). CONCLUSIONS: Right bundle-branch block and precordial injury pattern in V1 through V3 is common in SUDS patients and represents an arrhythmogenic marker that identifies patients who face an inordinate risk of ventricular fibrillation or sudden death.     

Predictors of sudden cardiac death in hypertrophic cardiomyopathy

Patients with hypertrophic cardiomyopathy (HC) die suddenly. Proposed risk factors for sudden cardiac death (SCD) in HC are youth, a family history of SCD, syncope, and ventricular tachycardia. Hemodynamic variables have not convincingly proved to be risk factors for SCD. Therefore, this study was designed to examine predictors of SCD in a large number of patients with HC during long-term follow-up periods. The relation of studied variables (clinical, electrocardiographic, echocardiographic, hemodynamic, and exercise test findings) to SCD in 309 patients with HC who were initially diagnosed during 1971 through 1994 (mean follow-up 9.4 years) was examined by multivariate analysis. SCD occurred in 28 patients. Independent predictors of SCD were a smaller difference between peak and rest systolic blood pressure during exercise testing (p=0.006), and higher left ventricular outflow tract pressure gradient at rest (p=0.003). Exercise-related SCD occurred in 8 patients and exercise-unrelated SCD in 20 patients (mean age 28 vs 47 years, p <0.05). Thus, patients of exercise-related SCD were younger and had smaller increases in systolic blood pressure during exercise testing, whereas patients with exercise-unrelated SCD were older and had higher left ventricular outflow tract pressure gradient.     

Eisenmenger syndrome. Factors relating to deterioration and death

AIMS: To assess the natural history, risk factors for death and deterioration of patients with Eisenmenger Syndrome. METHODS: The clinical course of 188 patients from three different cardiac centres specialized in adult congenital heart defects, followed for a median period of 31 years, was retrospectively analysed. According to the diagnosis, 64 males and 124 females, mean age at last follow-up 33.02+/-12.8 years, were divided into two groups: simple (128) and complex (60) congenital heart disease. Events analysed were: time variation of Ability Index, pulmonary and cerebral complications, non-cardiac surgery, urate metabolism and renal function, arrhythmias, pregnancy and contraception, medical therapy and transplantation, death from all causes and sudden death. RESULTS: Eighty-four percent of the overall population showed a satisfactory Ability Index (1 and 2) at the first attendance (median age 25 years). Patients with complex heart disease and Eisenmenger reaction had earlier clinical deterioration (18.6+/-11.3 vs 26.7+/-12.2) and shorter survival (25.8+/-7.9 vs 32.5+/-14.6 years). Thirty-eight (20.2%) patients had at least one episode of haemoptysis between 20 and 40 years of age but it did not modify overall survival; 25 (13.2%) had pulmonary thromboembolism at a mean age of 35.2+/-13.4 years. Fifteen (7.9%) had a stroke and 7 (3.7%) a cerebral abscess at a mean age of 31.4+/-15.7 and 24.1+/-4.9 years, respectively. Cerebral complications influenced the quality of life but did not modify survival. Patients who had venesection showed a 2.04 times greater hazard ratio for haemoptysis. Venesection did not reduce cerebral complications and in 20% caused anaemia and iron deficiency. Other non-cardiac surgery with general anaesthesia carried risks (23.5% of deaths). Significant maternal mortality (27%) in relation to pregnancy occurred with constant deterioration in physical status, high incidence of spontaneous abortions (35.8%) and cardiac abnormalities in offspring (20%). Sixty-one patients died during follow-up, mainly by sudden death (29.5%), heart failure (22.9%) and from haemoptysis (11.4%). Eight patients had heart and lung transplantation and five died 1 week to 4 years after transplant. Deterioration in Ability Index (worsening symptoms), age, complex defects, blood creatinine level, right ventricular dysfunction and non-cardiac surgery were variables which affected the prognosis adversely with uni- and multivariate analysis. CONCLUSIONS: patients with Eisenmenger syndrome can survive to the seventh decade with informed medical care and protection from special risks     

Prognostic value of arrhythmogenic markers in systemic hypertension

OBJECTIVE: To evaluate the prognostic value of arrhythmogenic markers in hypertensive patients. DESIGN: Two hundred and fourteen hypertensive patients without symptomatic coronary disease, systolic dysfunction, electrolyte disturbances or anti-arrhythmic therapy were included. Recordings were made of 12-lead standard ECGs with calculations of QT interval dispersion, 24 h Holter ECGs (204 patients), echocardiography (187 patients) and signal-averaged ECGs (125 patients). RESULTS: Baseline data: echocardiographic left ventricular hypertrophy was found in 63 patients (33.7%), non-sustained ventricular tachycardia (Lown class IV b) in 33 patients (16.2%), ventricular late potentials in 27 patients (21.6%). Mortality: after a mean follow-up of 42.4 +/- 26.8 months, global mortality was 11.2% (24 patients), cardiac mortality 7.9% (17 patients), sudden death 4.2% (nine patients). Univariate analysis: predictors of global, cardiac and sudden death were age > or = 65 years, ECG strain pattern, Lown class IV b and QT interval dispersion > 80 ms (P < or = 0.01). Left ventricular mass index was closely related to cardiac mortality (P = 0.002). Multivariate analysis: only Lown class IV b was an independent predictor of global (RR 2.6, 95% CI 1.2-6.0) and cardiac mortality (RR 3.5, 95% CI 1.2-9.7). CONCLUSION: In hypertensive patients, non-sustained ventricular tachycardia has a prognostic value.     

Cervical reconstruction of the supra-aortic trunks: a 16-year experience

PURPOSE: The purpose of this study was to review 182 consecutive cervical reconstructions of supra-aortic trunks, which were performed over a 16-year period. METHODS: A total of 182 innominate, common carotid, or subclavian arteries were reconstructed with a cervical approach in 173 patients aged 23 days to 83 years. Indications included hemispheric (n = 79), vertebrobasilar (n = 56), upper extremity (24), and internal mammary/cardiac ischemia (n = 5), asymptomatic severe common carotid disease (n = 33), or other (n = 3). Primary atherosclerotic innominate (n = 6), common carotid (n = 84), and subclavian (n = 66) lesions underwent reconstruction. Thirty-one operations were performed for multiple trunk involvement, recurrent disease, arteritis, infection, dissection, coarctation, or aneurysm. There were 122 bypass grafting procedures (98 ipsilateral, 24 contralateral) and 60 arterial transpositions. RESULTS: One death (0.5%) and 7 nonfatal strokes (3.8%) occurred, none in patients who were asymptomatic. Perioperative morbidity included four asymptomatic occlusions (2%), 6 myocardial infarctions (3%), 10 pulmonary complications (5%), and 2 graft infections (1%). Follow-up periods ranged from 1 to 190 months (mean, 53 +/- 5 months). Nineteen patients (10%) were lost to follow-up. Fifty-seven late deaths occurred, most from cardiac causes. Seven reconstructions necessitated late revision. The cumulative primary patency rate at 5 and 10 years was 91% +/- 2% and 82% +/- 5%, respectively. The survival rate at 5 years was 72% +/- 4% and at 10 years was 41% +/- 6%. The stroke-free survival rate was 92% +/- 2% at 5 years and 84% +/- 2% at 10 years. CONCLUSION: Cervical reconstruction of symptomatic and asymptomatic supra-aortic trunk lesions carries acceptable death and stroke rates and provides a long-term patient benefit. This should be the preferred approach for asymptomatic lesions and for patients with significant comorbidity because it carries less morbidity than direct transmediastinal aortic-based reconstruction.     

Sudden death in hypertrophic myocardiopathy]

Hypertrophic cardiomyopathy is the most common cause of sudden death in young individuals who are otherwise healthy. Risk of sudden death is highest in patients who are between 14 and 35 years old. Several mechanisms are involved in sudden death: ventricular arrhythmias, supraventricular arrhythmias leading to cardiac collapse, bradycardias and severe ischemia. Many studies have analyzed how to identify high risk patients. The factors that best identify high risk patients are: previous history of sudden death or syncope, induction in adults of sustained ventricular arrhythmias, the presence of non-sustained ventricular tachycardia in symptomatic patients, the presence of ischemia associated with hypotension in children, the presence of mutations in the beta-myosin heavy chain together with a family history of sudden death and a poor left ventricular ejection fraction. Risk stratification should be done on an individualized basis. In those patients in whom a high risk for sudden arrhythmic death is suspected, the only current effective treatment is the implantable defibrillator.     

The stratification and prevention of the arrhythmia risk in nonischemic dilated cardiomyopathy]

The challenge of preventing arrhythmic sudden death is one of the major issues in today's treatment of heart failure. To achieve this ambitious goal, an accurate selection of the candidates for sudden death is needed on the one hand, while on the other hand, the assessment of the real cost/benefit ratio of the implantable cardioverter-defibrillator in selected patients, as compared to ACE inhibitors, beta-blockers and antiarrhythmic drug therapy, should no longer be delayed. As is well known, the incidence of sudden death is higher in ischemic dilated cardiomyopathy than it is in non-ischemic dilated cardiomyopathy. Moreover, tachyarrhythmic sudden death is prevalent in NYHA classes I and II (80%), whereas its incidence is lower (50%) in NYHA classes III and IV, since bradyarrhythmia, electromechanical dissociation and thromboembolic events characterize the other 50% of sudden deaths in patients in the latter NYHA class. The stratification of arrhythmic risk in non-ischemic dilated cardiomyopathy is questionable from any point of view, considering the poor predictive power of invasive and non-invasive indexes. However, some subgroups of high-risk patients should be selected, such as patients waiting for heart transplant or those with a severe disease but without an extreme degree of ventricular dysfunction, in whom the prognosis in terms of pump failure events is better and life expectancy is longer if the risk of arrhythmia is properly assessed and sudden death prevented. Consequently, the ICD implant may be effective in order to pursue the aim of reducing the tachyarrhythmic and bradyarrhythmic mortality in patients with a more severe disease and of minimizing the tachyarrhythmic risk in those with a less severe disease. Further studies will be developed to identify the ideal candidates for ICD implants.