Hypertrophic myocardiopathy (II). 24-hour electrocardiographic monitoring and its relation to the echocardiographic characteristics

Seventy-two patients with hypertrophic cardiomyopathy were evaluated by 24 hours of electrocardiographic ambulatory monitoring. The results were related to echocardiographic extent of hypertrophy, left ventricular outflow obstruction at rest, and transmitral flow parameters. Also, the symptoms and functional classification of NYHA were related to ventricular arrhythmias. Ventricular arrhythmias were detected in 68% of our patients: high degree of ventricular ectopia in 26 (36.1%), and ventricular tachycardia in 14 (19.4%). Most important supraventricular arrhythmias were: atrial fibrillation in 7 patients, atrial flutter in one, and supraventricular tachycardia in five. Asymmetric hypertrophy type I of Maron classification showed the lower number of ventricular arrhythmias. High degree of ventricular arrhythmias were more frequent in patients with: apical hypertrophy, worse functional classification of NYHA (I: 20%; II: 50%; III: 80%), and in patients with impaired distensibility detected by Doppler (80.7% vs 56.7%; p less than 0.05). There was no relationship between left ventricular outflow obstruction at rest and ventricular arrhythmias.     

Hypertrophic cardiomyopathy in the Saudi Arabian population: Clinical and echocardiographic characteristics and outcome analysis

Background

Current published literature on hypertrophic cardiomyopathy (HCM) comes primarily from Western populations. There is no published data on clinical and echocardiographic characteristics and long-term outcome of HCM in an Arab population.

Methods

We conducted a retrospective analysis of all patients 16 years or older diagnosed with HCM at our institution. Detailed clinical and echocardiographic data were collected and outcome was analyzed.

Results

A total of 69 patients were identified as having HCM. The mean age was 42 ± 16 years with 71% male patients. All patients were Saudi citizens with Arab ancestry. Details about family history and presenting symptoms were available for 44 and 48 patients consecutively. Nine (18%) patients were asymptomatic and were diagnosed based on abnormal cardiac auscultation. The commonest presenting symptoms were dyspnea with or without chest pain and palpitations occurring in 40 (81%) patients. Only four (9%) of 44 patients had a family history of HCM and /or sudden cardiac death (SCD). The most common ECG abnormality was left ventricular hypertrophy (LVH) present in 60 (86%) patients. The commonest septal hypertrophy morphology was mid-septal (catenoid) in 30 (43%) followed by neutral in 23 (33%), basal septal (sigmoid) in 3 (4%) and apical in 6 (8%) patients. Twenty (28%) patients had evidence of resting left ventricular cavity gradient of ⩾30 mmHg. Eleven (16%) patients had evidence of biventricular hypertrophy. Left ventricular ejection fraction was normal in 65 (94%) patients. Over a median (25–75 percentile) follow-up of 7 years (4.5–10), only three patients died, all of non-cardiac causes. There were no cases of SCD during the follow-up period. Six patients required an implantable cardioverter-defibrillator (ICD); five for primary prevention and one for secondary prevention. Only one patient progressed to end stage dilated cardiomyopathy.

Conclusion

The natural history of hypertrophic cardiomyopathy in the Saudi population appears to be benign with catenoid morphology being the most common septal hypertrophy pattern. Risk of SCD appears to be quite low in this population.     

Hypertrophic cardiomyopathy: two-dimensional echocardiographic score versus clinical and electrocardiographic findings

The severity and site of hypertrophy is important in determining the clinical picture and the natural history of hypertrophic cardiomyopathy (HCM). We evaluated left ventricular hypertrophy by means of two-dimensional echocardiographic score and score index, and correlated these findings with symptoms, electrovector-cardiographic data, and ventricular arrhythmias. A total of 42 patients with HCM were studied by clinical examination, ECG, VCG, M-mode and 2D echocardiography, and 24-h Holter monitoring. The extent and severity of the hypertrophic process were calculated by a score system. The left ventricle was divided into 11 segments and a hypertrophic score (HS) was given to each segment. A hypertrophy score index (HSI) was also calculated by dividing the number of hypertrophied segments by 13. No correlation was found between symptoms and HS and HSI, nor ECG-VCG abnormalities and HS and HSI. A statistically significant relationship between the severity of ventricular arrhythmias and HS and HSI was found (p less than 0.01). The mechanism responsible for ventricular tachyarrhythmias in severe and diffuse hypertrophy might reside in the high intraventricular pressures which produce or worsen areas of myocardial ischemia.     

Hypertrophic myocardiopathy and tetralogy of Fallot. Study of 2 cases

The authors report two observations of concentric hypertrophic cardiomyopathy associated with Fallot's tetralogy in one case and with pulmonary atresia complicated by atrial septal defect in the other. The association of Fallot's tetralogy with hypertrophic cardiomyopathy is rare, as only 5 cases have been reported in the literature. In this connection, the mechanism of myocardial hypertrophy and the therapeutic problems posed by this association are discussed.     

Baseline echocardiographic characteristics of heart failure patients enrolled in a large European multicentre trial (CArdiac REsynchronisation Heart Failure study).

AIMS: Information on the prevalence and clinical, electrocardiographic and echocardiographic inter-relationships of mechanical dyssynchrony among patients with heart failure (HF) and left ventricular systolic dysfunction derives mainly from relatively small studies. The CARE-HF trial provides the opportunity to address these issues in a large population of patients with advanced HF. METHODS AND RESULTS: The CARE-HF trial enrolled patients with New York Heart Association (NYHA) class III or IV HF, with a QRS duration > or =120 ms, left ventricular (LV) ejection fraction (EF) < or =35% and LV end diastolic diameter > or =30 mm/m (height in m). Patients underwent a thorough echocardiographic evaluation, which included assessment of LV structure, systolic function, mitral inflow pattern, right ventricular (RV) dimensions and function, and interventricular mechanical delay (IVMD) as an index of interventricular dyssynchrony. Echocardiographic measurements were made in a Core Laboratory to ensure consistent quantitative analysis. Of the 813 patients enrolled, 735 had a baseline echocardiographic examination suitable for measurement. Overall patients had advanced LV dysfunction (mean EF 25.5%) but few had a restrictive mitral filling pattern (18%) and both the mean RV diameter and RV function were within normal limits. Interventricular dyssynchrony defined as IVMD >40 ms was present in 455 patients (62%). Clinical, electrocardiographic and standard echocardiographic variables were only loosely associated with IVMD. CONCLUSIONS: Interventricular dyssynchrony appears to be an independent characteristic of patients with advanced HF, and is poorly related to clinical, electrocardiographic or standard echocardiographic variable.     

Prognosis and risk evaluation of postoperative coronary patients (PERISCOP). Methodology and study population characteristics

The aim of the PERISCOP study was to evaluate the predictive value of cardiological investigations performed after recent coronary bypass surgery with regards to cardiac event and mortality at one year. The treatment of lipid abnormalities was also analysed. This first article describes the methodology and patient characteristics at inclusion. This prospective national multicenter trial included 2065 patients (86% men) with an average age of 63.1 +/- 9.9 years. The number of diseased vessels was 2.6 +/- 0.6. Preoperative left ventricular function was normal (ejection fraction 60 +/- 13%). Revascularisation was complete in 73% of cases (22% of arterial grafts). The cardiological investigations were performed at Day 20 +/- 10 after surgery. The duration of exercise on stress testing was 429 +/- 170 seconds. It was positive or doubtful in 9% of cases. Ventricular arrhythmias were observed in 6.5% of cases. The blood pressure response was abnormal in 6% of cases. Holter monitoring showed a median number of ventricular extrasystoles over 24 hours of 44. Three per cent of patients had one episode of ventricular tachycardia and 7% had ischaemic episodes. The echocardiographic index of segmental contractility was on average 1.75 (ejection fraction: 52.6%). The lipid analysis performed at one month, under lipid therapy in 34% of cases, showed a total cholesterol level at 1.91 +/- 0.10 g/l, an LDL-cholesterol of 1.27 +/- 0.08 g/l. The therapeutic target (LDL-cholesterol < 1 g/l) was attained in 46% of cases with treatment and in 18% of cases without treatment.     

Hypertrophic cardiomyopathy in three generations of a large Norwegian family. A clinical, echocardiographic, and genetic study

Hypertrophic cardiomyopathy is a heart muscle disease with an obscure aetiology. Data from four generations of a large family (71 members) are presented. The occurrence of hypertrophic cardiomyopathy among members of the two oldest generations was compatible with a pattern of autosomal dominant inheritance. Seven out of 14 siblings in the second generation had definite signs of or were clinically suspected of having hypertrophic cardiomyopathy. The severity and distribution of left ventricular hypertrophy varied, but three (21%) brothers in generation II showed the classic picture of left ventricular outflow obstruction. Four siblings (29%) died suddenly aged 11, 22, 38, and 40 years. A high incidence of the disease would have been expected in the two younger generations (41 members, aged 1-31 years), but only two, a 16 year old boy and a 17 year old girl had signs of asymmetric septal hypertrophy. Current diagnostic procedures, including M mode and cross sectional echocardiography, are not sufficiently sensitive to identify young family members who may have preclinical hypertrophic cardiomyopathy. No evidence for close genetic linkage between a postulated locus for hypertrophic cardiomyopathy and the major histocompatibility complex (antigens HLA-A, HLA-B, and HLA-DR) was found.     

Hypertrophic cardiomyopathy in three generations of a large Norwegian family. A clinical, echocardiographic, and genetic study.

Hypertrophic cardiomyopathy is a heart muscle disease with an obscure aetiology. Data from four generations of a large family (71 members) are presented. The occurrence of hypertrophic cardiomyopathy among members of the two oldest generations was compatible with a pattern of autosomal dominant inheritance. Seven out of 14 siblings in the second generation had definite signs of or were clinically suspected of having hypertrophic cardiomyopathy. The severity and distribution of left ventricular hypertrophy varied, but three (21%) brothers in generation II showed the classic picture of left ventricular outflow obstruction. Four siblings (29%) died suddenly aged 11, 22, 38, and 40 years. A high incidence of the disease would have been expected in the two younger generations (41 members, aged 1-31 years), but only two, a 16 year old boy and a 17 year old girl had signs of asymmetric septal hypertrophy. Current diagnostic procedures, including M mode and cross sectional echocardiography, are not sufficiently sensitive to identify young family members who may have preclinical hypertrophic cardiomyopathy. No evidence for close genetic linkage between a postulated locus for hypertrophic cardiomyopathy and the major histocompatibility complex (antigens HLA-A, HLA-B, and HLA-DR) was found.     

成人双孔二尖瓣畸形的二维及三维超声及临床特点

目的:探讨成人双孔二尖瓣(DOMV)的临床及超声心动图特征。方法:回顾分析经超声心动图诊断双孔二尖瓣13例,分析其临床表现、超声心动图特征及预后。结果:13例成人DOMV中,10例经胸超声心动图明确诊断,3例经食管超声心动图明确诊断。DOMV的典型表现为左心室短轴二尖瓣口水平呈"眼镜"征,四腔心或两腔心切面表现为典型的"海鸥征"。13例成人DOMV中,7例合并先天性心脏病,3例合并心肌梗死。结论:超声心动图可以准确诊断成人先天性双孔二尖瓣畸形。成人DOMV的临床表现与伴随病变相关,多数为偶然发现。     

Non-myxomatous flail mitral valve: clinical and echocardiographic characteristics and long-term clinical outcome

BACKGROUND AND AIM OF THE STUDY: Chordal rupture leading to flail mitral valve and mitral regurgitation (MR) is considered to be caused primarily by myxomatous mitral valve disease. The study aim was to determine the prevalence and clinical and echocardiographic characteristics of non-myxomatous versus myxomatous flail mitral valve. METHODS: A total of 96 patients with flail mitral valve was identified from an echocardiography database and classified as either myxomatous (n = 36; 37%) or non-myxomatous (n = 60; 63%), based on echocardiographic mitral valve anatomy (systolic leaflet buckling). In 10 other patients the etiology was indeterminate. The clinical and echocardiographic characteristics and outcome at five years were compared between groups. RESULTS: Patients with non-myxomatous mitral valve were older than those with myxomatous mitral valve (mean age 76 +/- 9 versus 61 +/- 12 years; p <0.0001), and were more likely to have aortic sclerosis, mitral annulus and papillary muscle calcification (odds ratio 3.6, 95% CI 1.2-10.8, p = 0.02) and to have short duration of symptoms (< or =1 month, p <0.02). There was no inter-group difference in MR severity, but non-myxomatous patients had higher systolic pulmonary artery pressure (52 +/- 16 versus 42 +/- 13 mmHg, p = 0.008). During the five-year follow up period, non-myxomatous patients had a poorer crude survival and survival free from rehospitalization for heart failure (p = 0.02), and were less likely to have mitral valve surgery (p = 0.015). However, these differences were abolished when data were adjusted for age. CONCLUSION: Among patients with flail mitral valve referred for echocardiography, more than half were non-myxomatous in origin, most likely due to wear and tear. Non-myxomatous flail mitral valve was associated with older age, degenerative calcific valvular changes, and more recent onset of symptoms. Age-adjusted survival free of heart failure was similar in both non-myxomatous and myxomatous patients.     

Inoue balloon mitral valvuloplasty: long-term clinical and echocardiographic follow-up of a predominantly unfavourable population.

AIMS: To assess long-term outcome in a typical Western population of predominantly unfavourable patients undergoing Inoue balloon mitral valvuloplasty. Outcome amongst patients has only been undertaken in the medium term. Long-term echocardiographic data in particular are scarce. METHODS: Inoue mitral valvuloplasty was attempted in 106 patients. There were six technical failures; the procedure was therefore completed in 100 patients, who underwent annual clinical and echocardiographic follow-up. RESULTS: Patients were aged 63.5+/-10. 3 years. 82% were female. Unfavourable characteristics included age >65 (52%), NYHA class III or IV (87%), >/=1 significant co-morbidity (63%), atrial fibrillation (82%), previous surgical commissurotomy (25%) and echocardiographic score >8 (59%, mean 8.9+/-2.1). Mitral valve area increased from 0.98+/-0.23 to 1.54+/-0.31 cm(2). There were three major complications. Post-procedure, symptoms improved in 88% of patients. Haemodynamic success (mitral valve area increase >50%, final mitral valve area >1.5 cm(2), mitral regurgitation 50% gain in mitral valve area, mitral valve area <1.5cm (2)) was 98%, 92% and 75% at 1, 3 and 6 years. Pre-procedural predictors of event-free survival were male sex, absence of co-morbidities, lower echocardiographic score and smaller left atrial diameter. CONCLUSIONS: In a Western population with predominantly unfavourable characteristics for mitral valvuloplasty, long-term outcome post-procedure is reasonable. A moderate increase in mitral valve area can be achieved at low procedural risk, and the subsequent rate of restenosis is low. Nonetheless, 6 years after the procedure, half of the patients will have required further intervention or died. For fitter patients willing to accept significant operative risk, mitral valve replacement remains a valuable alternative.     

Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): ventriculographic and echocardiographic features in 30 patients

In 30 of 1,002 consecutive patients who had left heart catheterization and cineangiography for evaluation of either ischemic heart disease or cardiomyopathy the electrocardiogram showed giant negative T waves (greater than 10 mm) associated with high QRS voltage (R wave greater than 26 mm in lead V₅ or the sum of the S wave in lead V₁ and the R wave in lead V₅ 35 mm or more) in the precordial leads despite absence of hypertension or significant coronary artery disease. In all 30 patients a characteristic spade-like configuration (concentric apical hypertrophy) was observed in the right anterior oblique ventriculogram at end-diastole as well as in the long axis two dimensional echocardiogram.The average apical thickness in these patients (24.8 ± 6.6 mm) was significantly greater than that in normal subjects (9.4 ± 3.1 mm) (P < 0.001) or in patients with hypertrophic obstructive cardiomyopathy (14.7 ± 5.0 mm) (P < 0.001). Values for both the mid anterior free wall thickness (13.9 ± 4.1 mm) and the mid posterior free wall thickness (14.3 ± 3.0 mm) were greater than values in normal subjects (8.9 ± 1.8 mm and 8.2 ± 2.0 mm, respectively) (P < 0.001). However, the ratio between the apical and the mid anterior free wall thickness in these 30 patients (1.86 ± 0.53) was significantly greater than the ratio in normal subjects (1.05 ± 0.24), patients with hypertrophic obstructive cardiomyopathy (0.96 ± 0.15) (P < 0.001) and patients with types of nonobstructive hypertrophic cardiomyopathy (1.26 ± 0.24) (P < 0.005) other than apical concentric hypertrophy. Obstruction of the tract did not occur because the upper half of the septem remained rather thin in systole and did not bulge into the left ventricle during systole. Pressure study with proper provocations as well as two dimensional echocardiograms revealed no peak systolic pressure gradient or obstruction within the outflow tract of the left ventricle.It is concluded that these 30 patients have nonobstructive hypertrophic cardiomyopathy with marked concentric hypertrophy in the apex (apical hypertrophic type) and with a different septal shape and contraction pattern from those seen in the obstructive type. This type of hypertrophy appears to be a fairly common type of hypertrophic cardiomyopathy in Japan.     

Significance of electrocardiographic isolated abnormal terminal P-wave force (left atrial abnormality). An echocardiographic and clinical correlation

An abnormal terminal negative deflection in precordial lead V1 (PTFV1) is occasionally present as an isolated electrocardiographic finding. To determine the significance of this, 41 patients with PTFV1 greater than or equal to 0.04 mms were recalled for a repeated electrocardiogram as well as an echocardiographic examination. Patients were classified as cardiovascular normal (n = 15) or abnormal (n = 26). Left atrial enlargement was the most common echocardiographic abnormality found, but represented less than a third of the total. P-wave prolongation (greater than 110 ms) was present in 30 of the 41 patients and 21 of the 26 abnormal patients. This persisted into the second study, while the PTFV1 fell significantly in both the normal and abnormal groups. Among the P-wave abnormalities found, combinations were used to identify patients most likely to have cardiovascular disease. The most discriminating was an initial abnormal PTFV1 in combination with P-wave prolongation (sensitivity, 82%; specificity, 40%; positive predictive value, 70%; and negative predictive value, 55%). Maximal specificity (93%) and positive predictive value (88%) were achieved when P-wave prolongation and PTFV1 greater than or equal to 0.06 mms were present in both studies, although the sensitivity for this criterion was only 27%. The isolated P-wave abnormality described may be helpful in suggesting the presence of underlying cardiovascular disease and indicate the need for further evaluation.     

Comparison of clinical echocardiographic and hemodynamic characteristics of male and female patients who underwent mitral balloon valvuloplasty.

The clinical echocardiographic and hemodynamic characteristics and outcome of male and female patients before and after valvuloplasty were evaluated. The study population consisted of 34 male and 122 female consecutive patients who successfully underwent percutaneous balloon mitral valvuloplasty (PBMV). Clinical, echocardiographic, and hemodynamic variables were compared between male and female patients. Male patients were significantly older than female patients at the time of PBMV (39 years vs 34 years p < 0.05). Mitral valve score was significantly higher in male patients (p < 0.01). The mitral valve area before PBMV in male patients was significantly less than that in female patients (0.97 +/- 0.22 cm2 vs 1.09 +/- 0.25 cm2, respectively, p < 0.05). The pulmonary artery pressures of female patients before PBMV were higher than those of males (48 mm Hg vs 40 mm Hg, respectively, p < 0.05). The restenosis rates in male and female patients at the end of the follow-up period (38 months) were 20% and 9%, respectively (p < 0.05). In conclusion, male patients are older than female patients at the time of the PBMV procedure, and male patients have worse echocardiographic parameters and restenosis rates than female patients with the exception of pre-procedural pulmonary artery pressure.     

The clinical spectrum of IgM-related amyloidosis: a French nationwide retrospective study of 72 patients

Immunoglobulin M (IgM)-related amyloidosis remains a rare and little-known complication of monoclonal IgM-associated disorders. We sought to determine the clinical and laboratory presentation, response to treatment, and outcome of patients with IgM-related amyloidosis in the era of new therapeutic approaches. We conducted a retrospective study in 29 French centers to identify patients with monoclonal IgM and biopsy-proven amyloidosis; we reviewed patients' records and collected relevant clinical and laboratory data.We identified 72 patients with IgM-related amyloidosis. Systemic primary amyloidosis (AL) was present in 64, peritumoral AL in 5, and systemic secondary amyloidosis (AA) in 3 patients. A peculiar pattern of relatively frequent lymph node (31%) and lung (17%) involvement was noted in patients with systemic AL amyloidosis. Response to alkylating agents was poor, with a hematologic response in 37%, a complete remission in 0%, and an organ response in 21%. Response to hematopoietic stem cell transplantation showed a hematologic response in 100% with complete remission in 75% and an organ response in 75%. Purine analogs and rituximab induced a hematologic response in 73% and 60%, respectively, with complete remission in 9% and 0% and an organ response in 55% and 0%, respectively. In multivariate analysis, prognostic factors for survival were serum albumin level < or =3.5 g/dL (p = 0.018) and heart involvement (p = 0.0034). Further prospective studies are needed in patients with IgM-related amyloidosis, with special emphasis on treatment options: hematopoietic stem cell transplantation and purine analogs could represent the most effective therapies. The identification of adverse prognostic factors of survival could be useful for those managing and making treatment decisions for these patients.     

Congenital left atrial aneurysms: clinical characteristics and cross-sectional echocardiographic features

Congenital intrapericardial aneurysm of the left atrial wall is a rare anomaly with protean manifestations. Subtle electrocardiographic and chest radiographic abnormalities often give a clue to this disease. This study describes clinical characteristics, cross-sectional and Doppler echocardiographic features of this anomaly in 4 young male patients.     

Hypertrophic cardiomyopathy. Long-term clinical development in a regional cohort of 243 patients

This retrospective study was undertaken to assess the long-term clinical outcome of hypertrophic cardiomyopathy (HCM) in a regional cohort of 243 patients aged 40.4 years on average at the time of diagnosis and followed up for 12.3 +/- 8.1 years. Forty-one deaths were recorded during the follow-up period directly related to HCM (including 20 sudden deaths and 17 deaths due to cardiac failure), an annual cardiac mortality rate of 1.37%. In multivariate analysis, two factors were associated with extra mortality: occurrence of the first symptoms before the age of 20 (RR x 2.35) (p = 0.006) and NYHA functional classes III: IV at the latest clinical assessment (p = 0.005). The risk of sudden death increased significantly with septal wall thickness: RR x 2.34 (p = 0.05), RR x 3.27 (p = 0.007) and RR x 3.67 (p = 0.02) respectively, for septal thickness equal to or greater than 25, 30 and 35 mm. Eighty-three patients (34%) had major cardiovascular events (sudden death, congestive cardiac failure, cerebrovascular accident) during follow-up. However, at the latest clinical assessment, 79% were relatively unaffected by their disease, without treatment (12%) or with drug therapy alone (60%). In a minority of patients (28%) a more aggressive therapeutic approach was necessary: cardiac pacing (N = 48), implantable cardiac defibrillators (N = 2) myomectomy (N = 27) or cardiac transplantation (N = 6). The authors conclude that HCM is a complex disease, less serious than initially thought in the majority of patients, but the cause of major cardiovascular events and premature deaths which still remain difficult to prevent.