The influence of age on gender-specific differences in the left ventricular cavity size and contractility in patients with hypertrophic cardiomyopathy

BACKGROUND: The aim of the study was to assess gender-specific differences in left ventricular cavity size, contractility and left ventricular outflow tract obstruction in younger and older subgroups of patients with hypertrophic cardiomyopathy. METHODS: We studied retrospectively 153 referred patients with hypertrophic cardiomyopathy (89 males and 64 females). The echocardiographically measured left ventricular end-systolic, end-diastolic dimensions, fractional shortening and occurrence of left ventricular outflow tract gradient were compared between sexes in subgroups of patients 50 years of age. RESULTS: In younger patients with hypertrophic cardiomyopathy, left ventricular end-diastolic and end-systolic dimensions were significantly smaller in females than males (41.9+/-5.8 vs. 44.7+/-5 mm P<0.01 23.4+/-5 vs. 25.2+/-5.4 mm P<0.05, respectively). Fractional shortening was comparable in both sexes (44.7+/-7.5 vs. 43.7+/-8.2% P>0.05). The left ventricular outflow tract gradient occurred in females as frequently as in males (13.3 vs. 17.6% P>0.05). In older patients with hypertrophic cardiomyopathy, left ventricular end-diastolic and end-systolic dimensions were also significantly smaller in females than males (42.5+/-6 vs. 46.3+/-3.2 mm P<0.02; 25.7+/-4.8 vs. 28.6+/-3.7 mm P<0.01, respectively). In contrast to the younger group, the fractional shortening was significantly higher in females than males (44.4+/-6.8 vs. 38.2+/-7.3% P<0.02). The left ventricular outflow tract gradient occurred in females more frequently than in males (63.2 vs. 20.8% P<0.02). CONCLUSIONS: In patients with hypertrophic cardiomyopathy, the gender-based differences in the absolute value of left ventricular cavity size persisted with aging. In older females left ventricular contractility was higher and left ventricular outflow tract gradient occurred more frequently than in males. In younger patients with hypertophic cardiomyopathy these sex-based differences were absent. The gender-specific differences in the parameters of left ventricular systolic function became apparent with increasing age.     

Clinical characteristics and outcomes of hypertrophic cardiomyopathy in Taiwan--a tertiary center experience

BACKGROUND: Most information concerning hypertrophic cardiomyopathy has been derived from western countries and Japan. HYPOTHESIS: Patients with hypertrophic cardiomyopathy in Taiwan may have a distinct morphology and clinical picture. METHODS: Information from 163 consecutive patients with hypertrophic cardiomyopathy at a medical center in Taiwan from 1990 through 2005 was retrospectively collected. The diagnosis of hypertrophic cardiomyopathy required echocardiographic demonstration of left ventricular hypertrophy (wall thickness >or= mm during diastole) in a specific region or with diffuse distribution. Follow-up information was obtained from medical records. RESULTS: Among 163 patients (male, 52%), the mean follow-up period was 5.3 +/- 4.1 years. Men had nearly a threefold increase in prevalence of apical hypertrophic cardiomyopathy (23.8% vs. 8.9%, p = 0.03), younger onset of initial evaluation (57.2 +/- 12.9 vs. 64.8 +/- 11.3, p < 0.001), and lower prevalence of hypertrophic obstructive cardiomyopathy (33.3% vs. 63.3%, p < 0.001) compared to women. Fifty-eight patients (35.6%) experienced cardiovascular events, of which pulmonary edema and paroxysmal atrial fibrillation were the most common (19.7% and 12.3%, respectively). The annual cardiovascular mortality rate was 0.8%. In multivariate analysis, left ventricular outflow obstruction [odds ratio (OR): 4.92, p = 0.001], atrial fibrillation (OR: 3.53, p = 0.014), and female gender (OR: 2.99, p = 0.043) were independent predictors of mortality. CONCLUSIONS: Hypertrophic cardiomyopathy did not significantly increase cardiovascular mortality rate, but over one-third of patients with hypertrophic cardiomyopathy experienced cardiovascular events. High prevalence of left ventricular outflow obstruction especially in elderly women was observed. Left ventricular outflow obstruction, atrial fibrillation, and female gender were predictors of mortality.     

Clinical and morphologic features of hypertrophic cardiomyopathy in elderly patients 85 years or older.

We studied the distinctive morphology of the left ventricle (LV) and attempted to relate advanced age and hypertension to this characteristic feature in elderly patients with hypertrophic cardiomyopathy (HC). Fourteen elderly patients > or = 85 years old (mean age 90 +/- 5 years) with HC were compared with 45 young patients < or = 40 years (mean age 34 +/- 4 years) with this disease. More mild hypertension in the elderly (10/14, 71%) than in the young (0%), and more syncope in the young (10/45, 22%) than in the elderly (0%) were observed. Echocardiography showed that the elderly patients had relatively mild LV wall thickening, generally confined to the septum (elderly vs young: 18 +/- 4 vs 25 +/- 8 mm, p < 0.001), with more basal septal bulging (elderly vs young: 12/14, 86% vs 0%, p < 0.001) and anterior septal hypertrophy of LV (elderly vs young: 11/14, 79% vs 0%, p < 0.001). Elderly patients with mild hypertension showed a predominantly basal septal bulging (10/10, 100%) and anterior septal hypertrophy of LV (9/10, 90%). HC in elderly patients > or = 85 years old has a striking LV morphology. Mild hypertension and advanced age may contribute to the distinctive geometry.     

Verapamil improves the pacing-induced vasodilatation in symptomatic patients with hypertrophic cardiomyopathy

The purpose of the study was to assess the effect of verapamil on the response of diastolic coronary flow velocity and coronary vascular resistance to pacing in symptomatic patients with hypertrophic cardiomyopathy. In 14 patients with hypertrophic cardiomyopathy, the coronary flow velocity was detected in the left anterior descending coronary artery using transthoracic Doppler echocardiography. The peak diastolic coronary flow velocity and coronary vascular resistance was measured at baseline and during pacing. Changes of these parameters induced by the pacing (expressed as the percentage of baseline values) were compared on verapamil treatment and after verapamil withdrawal. The same measurements were obtained in ten control subjects. The results show that, in hypertrophic cardiomyopathy patients, increase in coronary flow velocity during pacing was significantly higher on than off verapamil therapy (64.8+/-32.5 vs. 41.1+/-21.3%, P<0.05). In control subjects, pacing-induced increase in coronary flow velocity was comparable to changes in coronary flow velocity in hypertrophic cardiomyopathy patients receiving verapamil (80.2+/-18.4 vs. 64.8+/-32.5%, P>0.05). After verapamil withdrawal in hypertrophic cardiomyopathy patients, coronary flow velocity increase during pacing was significantly lower than in control subjects (41.1+/-21.3 vs. 80.2+/-18.4%, P<0.05). During pacing the coronary vascular resistance decreased more on verapamil than after drug withdrawal (-34.7+/-11.7 vs. -24.6+/-12.9%, P<0.05). In control subjects the coronary vascular resistance decreased during pacing -38.6+/-6.3% to similar extent as in hypertrophic cardiomyopathy patients on verapamil. We can conclude that endothelium-dependent vasodilatation during pacing was impaired in symptomatic patients with hypertrophic cardiomyopathy. Verapamil treatment was able to restore adequate vasodilator response to pacing stress.     

Community-acquired bacterial meningitis in older people

OBJECTIVES: To describe clinical features of bacterial meningitis in older people. DESIGN: Cohort study. SETTING: Hospitals in the Netherlands. PARTICIPANTS: Patients aged over 16 with community-acquired bacterial meningitis, confirmed using cerebrospinal fluid culture. MEASUREMENTS: Data were collected prospectively. The cohort was dichotomized with respect to age (>or=60 vs 17-59). RESULTS: Two hundred fifty-seven of 696 episodes of community-acquired bacterial meningitis (37%) occurred in elderly patients and 439 (63%) in younger adults. Older people more often presented with the triad of fever, neck stiffness, and altered mental status than younger adults (58% vs 36%; P<.001). In older people, meningitis was due to Streptococcus pneumoniae in 176 episodes (68%). In younger adults, Neisseria meningitidis was the most common pathogen, responsible for 221 episodes (50%). Elderly patients more often developed complications than younger adults (72% vs 57%; P<.001), which resulted in a higher mortality rate (34% vs 13%; P<.001). Older people tended to die more often from cardiorespiratory failure (25% vs 11%; P=.06), whereas younger adults more often died from brain herniation (23% vs 2%; P=.004). CONCLUSION: Elderly patients with bacterial meningitis often present with classic symptoms of bacterial meningitis. Bacterial meningitis within this age group is predominantly due to S. pneumoniae and is associated with high morbidity and mortality rates. Whereas older people die frequently of cardiorespiratory failure, younger adults more often die of brain herniation.     

Comparison of clinical, morphological, and prognostic features in hypertrophic cardiomyopathy between Japanese and western patients

Apical hypertrophic cardiomyopathy appears to be more common in Japan than in the West. Explanations for this difference include variable methods and criteria for the diagnosis. To assess morphological, clinical, and prognostic differences, 45 consecutive Japanese and 45 age- and gender-matched Western patients with hypertrophic cardiomyopathy were evaluated in two referral institutions by the same individuals. The diagnosis of hypertrophic cardiomyopathy was based on the echocardiographic demonstration of unexplained left ventricular hypertrophy. Patients were aged 8 to 64 years (mean 50); there were 66 males and 24 females. The pattern of left ventricular hypertrophy was similar in Japanese and Western patients: asymmetric septal 64 vs. 76%, concentric 22 vs. 13%, and apical 13 vs. 11% (p = NS). The incidence of an echocardiographic or Doppler calculated left ventricular gradient of > 30 mmHg was similar (11 vs. 18%; p = NS). The maximal left ventricular wall thickness was greater in Western patients (23 +/- 7 vs. 20 +/- 4 mm; p = 0.03), but was not different when adjusted for body surface area. Clinical features including incidence of family history and ventricular tachycardia during 24-h ambulatory electrocardiography were similar. During follow-up (4.9 +/- 4.0 years for Western vs. 4.4 +/- 2.0 years for Japanese), disease-related mortality was worse in Western patients (p < 0.05; 10 versus 2 patients). This evaluation, using the same diagnostic methods and criteria, reveals a worse prognosis in Western patients despite a similar clinical and morphological spectrum of hypertrophic cardiomyopathy.     

老年人肥厚型心肌病

目的 探讨我国老年人肥厚性心肌病(HCM)患者的临床特点. 方法 连续入选2002年9月至2008年9月在我院注册的HCM患者711例.记录临床资料,分析年龄≥65岁与非老年患者的差异. 结果 711例HCM患者中老年人共103例(14.5%),与非老年患者比较,老年HCM患者中女性较多(52.5%与29.0%,P<0.01),并存疾病多(64.1%与25.0%,P<0.01),心房颤动发生率高(21.4%与14.0%,P<0.05).心功能不全是老年人HCM最常见的症状,年龄(r=0.213,P<0.05)、左心房扩大(r=0.308,P<0.01)和心房颤动(r=0.362,P<0.01)是影响心功能的主要因素.24%患者有1个以上心原性猝死主要风险因素,以晕厥最常见. 结论 老年HCM患者具有并存疾病和心房颤动发生率高、心功能更差等特点,老年患者HCM相关的心原性猝死风险评价应予以重视.     

Comparison of outcome of paramedic-witnessed cardiac arrest in patients younger and older than 70 years

To obtain further information concerning differences in the mechanism of out-of-hospital cardiac arrest between elderly and younger patients, 381 consecutive patients who experienced out-of-hospital cardiac arrest, and whose arrest was witnessed by paramedics, were studied. In 91% of cases the arrest occurred at the time the patient's cardiac rhythm was monitored. Patients were divided into 2 age groups: elderly patients were greater than 70 years (187) and younger patients were less than 70 years (194). Elderly patients more commonly had a past history of heart failure (25 vs 10%, p less than 0.003) and were more commonly taking digoxin (40 vs 20%, p less than 0.005) and diuretics (35 vs 25%, p less than 0.004). Before the cardiac arrest, elderly patients were more likely to be complaining of dyspnea (53 vs 40%, p less than 0.009), whereas younger patients were more likely to complain of chest pain (27 vs 13%, p less than 0.001). Forty-two percent of younger patients demonstrated ventricular fibrillation as the initial out-of-hospital rhythm associated with the arrest, compared to only 22% of elderly patients (p less than 0.001). Besides patient age, initial cardiac rhythm varied according to the patient's complaint preceding the arrest. Sixty-eight percent of patients with chest pain demonstrated ventricular fibrillation, whereas only 21% of patients with dyspnea demonstrated ventricular fibrillation. Elderly patients could be as successfully resuscitated as younger patients; however, 24% of younger patients survived, compared to only 10% of elderly patients (p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)     

Electrocardiographic findings in patients with hypertrophic cardiomyopathy. Relation to presenting features and prognosis

The relation of ECG findings to presenting features and prognosis was evaluated in 125 consecutive patients with hypertrophic cardiomyopathy (HC). Seventy-nine men and 46 women (mean age, 34 +/- 7 years) were studied since 1970. Most ECG features were similar in patients with and without a left ventricular outflow tract gradient. Those with obstruction had a higher prevalence of left ventricular hypertrophy according to ECG voltage criteria (54% vs. 28%, p less than 0.01), whereas higher grade ventricular arrhythmias were more common in patients without an outflow gradient (20% vs. 7%, p less than 0.05). The prevalence of ECG abnormalities was also similar in younger (less than or equal to 14 years) and older patients (greater than 14 years), and only repolarization abnormalities were more frequently detected in the older age group (56% vs. 32%, p less than 0.025). Stratification of patients according to the clinical state revealed that those who had moderate to severe functional limitation had a higher prevalence of atrial fibrillation than asymptomatic or mildly symptomatic patients (24% vs. 1%, p less than 0.001). There were no significant differences in most hemodynamic variables among patients dichotomized according to any specific ECG abnormality. Only patients with atrial fibrillation had significantly higher right ventricular end-diastolic pressure (10 +/- 7 vs. 6 +/- 4 mmHg, p less than 0.01), lower systolic index (22 +/- 8 vs. 37 +/- 15 ml/m2; beat, p less than 0.02) and lower ejection fraction (53 +/- 8 vs. 64 +/- 10%, p less than 0.001) than those in sinus rhythm.(ABSTRACT TRUNCATED AT 250 WORDS)     

Unexplained pulmonary hypertension in elderly patients

BACKGROUND: Idiopathic pulmonary arterial hypertension (IPAH) preferentially affects young women. However, a subset of patients with IPAH is elderly. Our objective was to compare elderly (age >/= 65 years) vs younger persons with unexplained pulmonary hypertension (PH) and a presumptive diagnosis of IPAH. METHODS: Clinical, echocardiographic, hemodynamic, and survival data were collected on consecutive patients with suspected IPAH after evaluation in a large tertiary center PH clinic. RESULTS: Of 197 patients (mean age +/- SD, 52 +/- 16 years; 80% female), 48 patients (24%) were elderly. Elderly and younger patients had similar symptom severity, systolic pulmonary artery (PA) pressure (82.7 +/- 20.3 mm Hg vs 86.9 +/- 18.8 mm Hg, respectively; p = 0.21), and severity of right ventricular enlargement and dysfunction. Elderly patients had higher pulmonary capillary wedge pressure (PCWP) [15.3 +/- 7.3 mm Hg vs 11.1 +/- 5.3 mm Hg; p < 0.0001] and more frequently failed (56%) to meet hemodynamic criteria for IPAH (PH with PCWP < 15 mm Hg) than did younger patients (19%). Elderly patients also had higher systemic systolic (p < 0.0001) and pulse (p < 0.0001) pressures and more cardiovascular disease. Among those patients with normal PCWP, elderly patients had worse survival than young patients (p = 0.007). Among those patients with elevated PCWP, elderly patients had lower PA pressures (p = 0.04) and better survival (p = 0.02). CONCLUSIONS: Elderly patients with clinically suspected IPAH often fail to meet hemodynamic criteria for IPAH due to elevated PCWP. Studies to define the proper diagnostic strategy and the safety and efficacy of pulmonary vasodilators in elderly patients with unexplained PH are needed.     

Coronary artery stenting in the elderly: short-term outcome and long-term angiographic and clinical follow-up

Objectives. This study sought to compare the short- and long-term outcomes of elderly patients undergoing coronary artery stenting with those of younger patients and to determine the long-term clinical outcome and survival of elderly patients post stent implantation.Background. Elderly patients undergoing coronary revascularization are considered a high-risk group. Few data exist that relate the results of stenting in treating coronary artery disease in the elderly population.Methods. All elderly patients ≥75 years of age who underwent coronary artery stenting between March 1993 and July 1997 (n = 137) at our center were compared to the patients <75 who underwent coronary artery stenting during the same time period (n = 2,551). Long-term clinical follow-up and survival were determined for the elderly group.Results. Elderly patients presented with lower ejection fractions (54% vs. 58%, p = 0.0001), more unstable angina (47% vs. 28%, p = 0.0001), and more multivessel disease (78% vs. 62%, p = 0.0001) than younger patients. These older patients had higher rates of procedure related complications including procedural myocardial infarction (MI) (2.9% vs. 1.7%, p = 0.2), emergency CABG (3.7% vs. 1.4%, p = 0.04), and death (2.2% vs. 0.12%, p = 0.0001). Angiographic follow-up, obtained in both groups, demonstrated significantly higher restenosis rates in the elderly versus younger patients (47% vs. 28%, p = 0.0007). Longer term clinical follow-up, which was obtained only in the elderly group, showed that at a mean follow-up period of 12 months post coronary stenting, elderly survival free from death, MI, revascularization and angina was 54% and that their overall survival was 91%. Subanalysis of the elderly patients who died showed much higher incidence of combined unstable angina (80%), prior MI (60%), lower ejection fraction (46%), multivessel disease (100%) and complex lesions (100%) than the overall group.Conclusions. Elderly patients who undergo coronary artery stenting have significantly higher rates of procedural complications and worse six month outcomes than younger patients, especially those who present with combined unstable angina, history of MI, EF < 50%, multivessel disease and complex lesions. Overall survival in the elderly population at 12 months postcoronary artery stenting was 91% and event-free survival was 54%.     

Comprehensive analysis of the beta-myosin heavy chain gene in 389 unrelated patients with hypertrophic cardiomyopathy

OBJECTIVES: We sought to determine the prevalence and phenotype of beta-myosin heavy chain gene MYH7 mutations in a large cohort of unrelated patients with hypertrophic cardiomyopathy (HCM). BACKGROUND: Hypertrophic cardiomyopathy is a heterogeneous cardiac disease. MYH7 mutations are one of the most common genetic causes of HCM and have been associated with severe hypertrophy, young age of diagnosis, and high risk of sudden cardiac death. However, these clinical findings from large, family studies have not been confirmed in a large unrelated cohort. METHODS: Deoxyribonucleic (DNA) samples obtained from 389 HCM outpatients seen at this tertiary referral center were analyzed for mutations, using polymerase chain reaction, denaturing high-performance liquid chromatography, and DNA sequencing for all 38 protein-coding exons of MYH7. Clinical data were extracted from patient records blinded to patient genotype. RESULTS: Fifty-eight patients (15%) harbored 40 different mutations in MYH7. Compared with HCM patients without MYH7 mutations, HCM patients with MYH7 were younger at diagnosis (32.9 vs. 42.7 years, p = 0.0002), had more hypertrophy (left ventricular wall thickness of 24.2 vs. 21.1 mm, p = 0.0009), and more frequently underwent myectomy (60% vs. 38%, p = 0.002). The HCM patients with MYH7 mutations more often had a family history of HCM (43% vs. 29%, p = 0.006), but there was no difference in family history of sudden death (16% vs. 14%, p = NS). CONCLUSIONS: In this setting, HCM patients with MYH7 were diagnosed at a younger age and had more hypertrophy, but they had no greater frequency of sudden death among first-degree relatives. Although these associations may prove useful for targeted gene screening, caution should be exercised in terms of using pathogenic status in risk stratification.     

Cumulative exercise-induced left ventricular systolic and diastolic dysfunction in hypertrophic cardiomyopathy

The phenomenon of cumulative exercise-induced left ventricular function impairment was studied in 40 patients with non-obstructive hypertrophic cardiomyopathy with resting normal left ventricular function and no increase in ejection fraction on exercise. All patients underwent two symptom-limited exercise tests one-hour apart. Cumulative myocardial dysfunction was seen in 13 patients (group I) but not in the remaining 27 patients (group II). During follow-up, group I showed more commonly than group II a deterioration in symptoms (67% vs 22%, P=0.025) and left ventricular function (50% vs 9%, P=0.019). In conclusion, cumulative exercise-induced myocardial dysfunction can occur in hypertrophic cardiomyopathy and may be associated with clinical deterioration and worse outcome.     

Intraventricular Systolic Flow Mapping in Hypertrophic Cardiomyopathy

Ninety-two consecutive patients with hypertrophic cardiomyopathy were studied with pulsed and continuous-wave Doppler and color flow imaging to assess the intraventricular systolic flow profile from apex to base and compare it with that obtained in normals and in patients with aortic stenosis and systemic hypertension. Hypertrophic cardiomyopathy patients had higher intraventricular blood flow velocities (cm/sec) from apex to base compared with normals and aortic stenosis and systemic hypertension patients (apex: 41.5 ± 17.3 vs 24 ± 1.9,26.1 ± 2.9, and 26.4 ± 3.3; papillary muscles: 95.4 ± 66.5 vs 41.9 ± 4.9, 46.2 ± 3.4, and 46.4 ± 5.7; outflow tract: 249.3 ± 176.2 vs 66.9 ± 8.4, 64.1 ± 10.8, and 66 ± 9.5, respectively) (P < 0.001). Eighty-six (93%) hypertrophic cardiomyopathy patients showed an abnormal intraventricular systolic color flow pattern at one or more sites but none of the patients with aortic stenosis or systemic hypertension or normal controls. Of those, 65 (71%) showed one or more variant (mosaic) flow, all of whom had intraventricular gradients, while 75 showed abnormal aliased flow at a site other than the subaortic area. It is concluded that patients with hypertrophic cardiomyopathy often exhibit an abnormal spatial distribution of the intraventricular systolic flow velocity profile compared with normals and patients with secondary forms of ventricular hypertrophy that can readily be recognized with color flow imaging. This could improve the sometimes difficult separation of hypertrophic cardiomyopathy patients from secondary hypertrophy.     

Clinical features of hypertrophic cardiomyopathy in the young

BACKGROUND: We analysed the experience with hypertrophic cardiomyopathy in two paediatric centres to establish the differences from older patients. METHODS: Out of 45 young patients seen from 1974 to 1999, we included 38. Criterions for exclusion were secondary forms, or association with severe congenital cardiac disease which could alter the outcome. RESULTS: The patients presented at the age of 5.7 years, and were followed for 7.0 years. The 34 patients referred because of a murmur or cardiomegaly were older than the four with heart failure, presenting at 6.2 as opposed to 2.1 years of age, p = 0.08. Of the patients, 29 (76%) had primary cardiomyopathy, while 9 (24%) had secondary forms associated with Noonan's and LEOPARD syndromes. Familial tendency was ascertained in 7 patients (18%). The septal thickness in mm/m2 at presentation was greater in patients under 2 years than in older children (29 vs 18, p = 0.02). Obstructive hypertrophic cardiomyopathy was found in 17 patients (45%), with six of these having mild associated congenital cardiac defects. Nine had symptomatic arrhythmias. Overall, treatment was medical in 31, with DDD pacing used in 5, and surgery, radiofrequency ablation, and transplantation in one patient each. Total mortality was 24%, at a rate of 4.3% per year. Four patients died in heart failure and 5 had sudden death. Those in failure were significantly younger (p = 0.01). CONCLUSIONS: Hypertrophic cardiomyopathy in the young is characterized by referral for murmur or heart failure; frequent secondary forms; the obstructive variant being as common as the non-obstructive form; a mortality rate similar to that for adults attending tertiary centres; and less frequent familial forms than in older populations.     

Increased cardiovascular response to epinephrine in hypertrophic cardiomyopathy

To study the role of catecholamines in the pathogenesis of hypertrophic cardiomyopathy (HCM), hemodynamic responses to low and high dose infusions of epinephrine (0.037 and 0.074 micrograms/Kg/min) were compared between 21 patients with nonobstructive HCM and 21 healthy controls, matched for age and sex. During low dose infusion, patients with HCM showed significantly greater responses (p less than 0.05) than controls in echocardiographic left ventricular (LV) end-systolic dimension (-8 +/- 1% vs -4 +/- 1%, mean +/- SEM), fractional shortening (12 +/- 2% vs 7 +/- 1%) and peak systolic velocity of the LV posterior wall (32 +/- 5% vs 15 +/- 4%), but the differences disappeared during high dose infusion. Thus, patients with HCM started to respond to epinephrine earlier than controls and seemed to have an increased sensitivity of beta-adrenergic receptors in the cardiovascular system. As the augmented responses were more evident in younger patients (less than 35 years) who manifested frequent familial occurrences of HCM, the increased sensitivity to catecholamine was postulated to be genetically determined and to be related to the abnormal myocardial hypertrophy of HCM.     

Epidemiology of cardiomyopathies in children and adolescents: a retrospective study over the last 10 years

We conducted a retrospective study at the Department of Paediatric Cardiology of the University Hospital Centre Rebro, Zagreb, over the period from 1988 to 1998, so as to assess the epidemiology of childhood cardiomyopathies. The patients were categorized according to the guidelines of the Task Force on Cardiomyopathies of the World Health Organization and the International Society and Federation of Cardiology. We identified 121 infants, children and adolescents as having cardiomyopathy, giving an average occurrence for all cardiomyopathies of 38.81 for each 10,000 patients examined in our outpatient clinics for paediatric cardiology. Of the patients, 50 were female (41.3%) and 71 were male (58.7%). The cardiomyopathy was of the dilated variant in 52 patients (42.9%), with 43 patients (35.5%) having hypertrophic cardiomyopathy, and 6 patients (4.8%) identified with restrictive cardiomyopathy. We encountered no patients with arrhythmogenic right ventricular cardiomyopathy. In nine patients (7.4%), it proved impossible to classify the cardiomyopathy. We placed 11 patients (9.0%) in the group of specific cardiomyopathies. Most of those with dilated cardiomyopathy had been diagnosed prior to the age of 3 years (RR 1.9, 95% CI 1.4-2.47). There were no statistically significant differences in the incidences of dilated as compared to hypertrophic cardiomyopathy (Z 0.923, p = 0.1779), but we encountered a significantly lower occurrence of restrictive cardiomyopathy (Z 6.044, p < 0.001). Of those with hypertrophic cardiomyopathy, 15 patients (34.8%) had the asymmetric variant, while 28 patients (65.2%) exhibited the concentric form. During the period of follow-up, 10 patients died, 4 with dilated cardiomyopathy, 4 with hypertrophic cardiomyopathy, 1 with restrictive cardiomyopathy, and 1 with a specific cardiomyopathy. We encountered 12 (9.9%) patients who, besides cardiomyopathies, also suffered from neuromuscular disorders. Most of these had dilated cardiomyopathy. Mitochondrial disorders, in contrast, were more frequently found in patients with hypertrophic cardiomyopathy.     

Clinical evaluation of the prehospital phase of acute myocardial infarction in the elderly

In order to clarify the clinical characteristics of the prehospital phase of acute myocardial infarction (AMI) in the elderly, we studied 92 elderly (65 years old or more) and 41 younger patients with first AMI. Fifty eight elderly (63.1%) and 30 younger (72.7%) patients had typical symptoms such as chest pain at onset. There were 5 elderly cases who had no symptom, although all of the younger had some symptoms at onset. More than 70% of the younger cases developed their symptoms either between 6 am and noon or between 6 pm and midnight, whereas the elderly showed no such tendency. Intervals between the onset of symptoms and hospital admission (admission time) averaged 6.8 hours (hr) in the younger and 7.7 hr in the elderly. Approximately one half of the elderly and a quarter of the younger showed admission time more than 6 hr, respectively. Elderly cases with atypical symptoms tended to have a longer admission time than younger cases (7.2 vs 3.1 hr), although there was no significant difference between the elderly and the younger with typical symptoms (8.0 vs 7.9 hr). Younger patients with heart failure had significantly shorter admission time than those without heart failure (3.6 vs 8.6 hr). However, the elderly with heart failure showed a prolonged admission time (6.1 hr). Although there was no difference on admission time between survivors and non-survivors within 28 days after admission in the elderly (7.5 vs 8.4 hr), the elderly non-survivors with atypical symptoms had the longest admission time (13.0 hr).(ABSTRACT TRUNCATED AT 250 WORDS)     

Clinical characteristics of and long-term outcome in Chinese patients with hypertrophic cardiomyopathy

BACKGROUND: Data on the phenotypical pattern and natural history of hypertrophic cardiomyopathy in Chinese patients are very limited. The purpose of this study was to describe the clinical characteristics of and long-term outcome in Chinese patients with hypertrophic cardiomyopathy. METHODS: We evaluated 118 Chinese patients (62 male) who were diagnosed with hypertrophic cardiomyopathy at Queen Mary Hospital from 1973 to 2002. Diagnosis was based on the demonstration of left ventricular hypertrophy (wall thickness > or =15 mm during diastole), either in a specific region or with diffuse distribution, using echocardiography or magnetic resonance imaging. Clinical predictors of major cardiovascular events related to hypertrophic cardiomyopathy (cardiovascular death, potentially fatal cardiac arrhythmia, and refractory heart failure requiring cardiac transplantation or percutaneous alcohol septal ablation) were evaluated with univariate and multivariate Cox proportional hazards regression models. RESULTS: The mean (+/- SD) age at presentation was 54 +/- 18 years. During a mean follow-up of 5.8 +/- 4.3 years (range, 1 to 29 years) from presentation, major cardiovascular events related to hypertrophic cardiomyopathy occurred in 19 patients (16%), including 9 deaths. Annual cardiovascular mortality was 1.6%. Fifty-five patients (47%) had one or more cardiovascular complications related to hypertrophic cardiomyopathy, of which atrial fibrillation was the most common (35%, n = 41). The most common type of hypertrophic cardiomyopathy was the apical variant (41%, n = 49). In multivariate analysis, female sex was the only independent predictor of major cardiovascular events related to hypertrophic cardiomyopathy (hazard ratio = 5.86; 95% confidence interval: 1.77 to 7.21; P = 0.007). CONCLUSION: Hypertrophic cardiomyopathy in Chinese patients is characterized by late onset of presentation, a high incidence of the apical form of the condition, and adverse clinical outcome in female patients, which suggest a different phenotypical pattern than in white patients.     

Hypertrophic cardiomyopathy and associated coronary artery disease

Eighty-five consecutive patients with hypertrophic cardiomyopathy underwent hemodynamic evaluation and coronary arteriography to determine, in each case, the incidence and importance of coronary artery disease (CAD). Sixteen patients (19%) had >60% narrowing of the luminal diameter of one or more coronary arteries. Our findings revealed that patients with CAD were significantly older (mean, 64 years) than patients without CAD (mean, 42 years) and had a higher incidence of angina pectoris (81% versus 44%). Left ventricular end-diastolic pressures were lower in patients with CAD (mean, 17 mm Hg) than in those without CAD (mean, 23 mm Hg). The electrocardiographic findings were similar in both groups. There was no operative mortality in either group. In five patients with severe stenosis of the left anterior descending coronary artery (>90%), no collaterals to that artery were seen. In two, filling of the narrowed artery appeared slower than usual. Our study indicates that in patients older than 45 years of age with hypertrophic cardiomyopathy, the incidence of CAD is significantly higher, and warrants coronary arteriography prior to consideration for cardiac surgery. In this study, patients with hypertrophic cardiomyopathy and CAD who underwent corrective surgery for hypertrophic cardiomyopathy concomitantly with coronary artery bypass surgery have had low operative and long-term mortality with long-lasting symptomatic improvement.