BACKGROUND: The comparative outcomes of subcutaneous implantable cardioverter-defibrillator (S-ICD) and transvenous ICD (T-ICD) have not been well studied. The aim of this study was to evaluate the safety and efficacy of currently available S-ICD and T-ICD. METHODS: The study included 86 patients who received an S-ICD and 1:1 matched to those who received single-chamber T-ICD by gender, age, diagnosis, left ventricular ejection fraction (LVEF), and implant year. The clinical outcomes and implant complications were compared between the two groups. RESULTS: The mean age of the 172 patients was 45 years, and 129 (75%) were male. The most common cardiac condition was hypertrophic cardiomyopathy (HCM, 37.8%). The mean LVEF was 50%. At a mean follow-up of 23 months, the appropriate and inappropriate ICD therapy rate were 1.2% vs. 4.7% (χ = 1.854, P = 0.368) and 9.3% vs. 3.5% (χ = 2.428, P = 0.211) in S-ICD and T-ICD groups respectively. There were no significant differences in device-related major and minor complications between the two groups (7.0% vs. 3.5%, χ = 1.055, P = 0.496). The S-ICD group had higher T-wave oversensing than T-ICD group (9.3% vs. 0%, χ = 8.390, P = 0.007). Sixty-five patients had HCM (32 in S-ICD and 33 in T-ICD). The incidence of major complications was not significantly different between the two groups. CONCLUSIONS The efficacy of an S-ICD is comparable to that of T-ICD, especially in a dominantly HCM patient population. The S-ICD is associated with fewer major complications demanding reoperation.
Adult ; Cardiomyopathy, Hypertrophic ; physiopathology ; therapy ; Death, Sudden, Cardiac ; prevention & control ; Defibrillators, Implantable ; Electrocardiography ; Female ; Humans ; Male ; Middle Aged ; Tachycardia, Ventricular ; physiopathology ; therapy

OBJECTIVE: To evaluate the influence of papillary muscles and trabeculae on left ventricular (LV) cardiovascular magnetic resonance (CMR) analysis using three methods of cavity delineation (classic or modified inclusion methods, and the exclusion method) in patients with hypertrophic cardiomyopathy (HCM). MATERIALS AND METHODS: This retrospective study included 20 consecutive HCM patients who underwent 1.5-T CMR imaging with short-axis cine stacks of the entire LV. LV measurements were performed using three different methods of manual cavity delineation of the endocardial and epicardial contours: method A, presumed endocardial boundary as seen on short-axis cine images; method B, including solely the cavity and closely adjacent trabeculae; or method C, excluding papillary muscles and trabeculae. Ascending aorta forward flow was measured as reference for LV-stroke volume (SV). Interobserver reproducibility was assessed using intraclass correlation coefficients. RESULTS: Method A showed larger end-diastole and end-systole volumes (largest percentage differences of 25% and 68%, respectively, p < 0.05), compared with method C. The ejection fraction was 55.7 +/- 6.9% for method A, 68.6 +/- 8.4% for B, and 71.7 +/- 7.0% for C (p < 0.001). Mean mass was also significantly different: 164.6 +/- 47.4 g for A, 176.5 +/- 50.5 g for B, and 199.6 +/- 53.2 g for C (p < 0.001). LV-SV error was largest with method B (p < 0.001). No difference in interobserver agreement was observed (p > 0.05). CONCLUSION: In HCM patients, LV measurements are strikingly different dependent on whether papillary muscles and trabeculae are included or excluded. Therefore, a consistent method of LV cavity delineation may be crucial during longitudinal follow-up to avoid misinterpretation and erroneous clinical decision-making.
Adult ; Aged ; Cardiomyopathy, Hypertrophic/*pathology ; Female ; Heart Ventricles/physiopathology/*radiography ; Humans ; *Magnetic Resonance Imaging, Cine ; Male ; Middle Aged ; Papillary Muscles/*physiopathology ; Retrospective Studies ; Stroke Volume/physiology ; Systole/physiology

No abstract available.
Adolescent ; Adrenergic beta-Antagonists/therapeutic use ; Calcium Channel Blockers/therapeutic use ; Cardiomyopathy, Hypertrophic, Familial/complications/genetics/*pathology/physiopathology/therapy ; Electric Countershock ; Electrocardiography ; Female ; Genetic Predisposition to Disease ; Heart Failure/etiology/therapy ; Heart Septum/drug effects/*pathology/physiopathology/ultrasonography ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pedigree ; Phenotype ; Tachycardia, Ventricular/etiology/therapy ; Treatment Outcome ; Ventricular Outflow Obstruction/etiology

β-myosin heavy chain mutations are the most frequently identified basis for hypertrophic cardiomyopathy (HCM). A transgenic mouse model (αMHC(403)) has been extensively used to study various mechanistic aspects of HCM. There is general skepticism whether mouse and human disease features are similar. Herein we compare morphologic and functional characteristics, and disease evolution, in a transgenic mouse and a single family with a MHC mutation. Ten male αMHC(403) transgenic mice (at t-5 weeks, -12 weeks, and -24 weeks) and 10 HCM patients from the same family with a β-myosin heavy chain mutation were enrolled. Morphometric, conventional echocardiographic, tissue Doppler and strain analytic characteristics of transgenic mice and HCM patients were assessed. Ten male transgenic mice (αMHC(403)) were examined at ages -5 weeks, -12 weeks, and -24 weeks. In the transgenic mice, aging was associated with a significant increase in septal (0.59±0.06 vs. 0.64±0.05 vs. 0.69±0.11 mm, P<0.01) and anterior wall thickness (0.58±0.1 vs. 0.62±0.07 vs. 0.80±0.16 mm, P<0.001), which was coincident with a significant decrease in circumferential strain (-22%±4% vs. -20%±3% vs. -19%±3%, P=0.03), global longitudinal strain (-19%±3% vs. -17%±2% vs. -16%±3%, P=0.001) and E/A ratio (1.9±0.3 vs. 1.7±0.3 vs. 1.4±0.3, P=0.01). The HCM patients were classified into 1st generation (n=6; mean age 53±6 years), and 2nd generation (n=4; mean age 32±8 years). Septal thickness (2.2±0.9 vs. 1.4±0.1 cm, P<0.05), left atrial (LA) volume (62±16 vs. 41±5 mL, P=0.03), E/A ratio (0.77±0.21 vs. 1.1±0.1, P=0.01), E/e' ratio (25±10 vs. 12±2, P=0.03), global left ventricular (LV) strain (-14%±3% vs. -20%±3%, P=0.01) and global LV early diastolic strain rate (0.76±0.17 s(-1) vs. 1.3±0.2 s-1, P=0.01) were significantly worse in the older generation. In β-myosin heavy chain mutations, transgenic mice and humans have similar progression in morphologic and functional abnormalities. The αMHC(403) transgenic mouse model closely recapitulates human disease.
Adult ; Age Factors ; Aging ; Animals ; Cardiomyopathy, Hypertrophic, Familial ; genetics ; physiopathology ; Cross-Sectional Studies ; Disease Models, Animal ; Echocardiography, Doppler ; Female ; Heart ; physiopathology ; Humans ; Male ; Mice, Transgenic ; Middle Aged ; Myocardium ; metabolism ; pathology ; Myosin Heavy Chains ; genetics ; Phenotype ; Species Specificity ; Young Adult

OBJECTIVETo analyze the endocardial, myocardial, and epicardial longitudinal systolic strain (LSsys) in the left ventricle (LV) segments and walls in patients with cardiac involvement due to primary amyloidosis (AL-CA) and hypertrophic cardiomyopathy (HCM).

METHODSTwenty patients with biopsy-proven AL-CA, 20 with asymmetric HCM, and 20 age-matched healthy volunteers were analyzed for their clinical characteristics and underwent conventional echocardiography for evaluating LV wall thickness, left atrial and ventricle size, systolic and diastolic function and 2-dimensional velocity vector imaging for evaluating the endocardial, myocardial and epicardial LSsys of the LV segments and walls. AL-CA and HCM patients also underwent cardiac magnetic resonance to evaluate the late gadolinium enhancement (LGE) features.

RESULTSCompared with the control group, AL-CA and HCM groups, with similar clinical symptoms and physical signs, both showed increased LV wall thickness, left atrial diameter, E/A ratio, septal E/e' ratio and the prevalence of granular sparkling. LV segments and walls endocardial LSsys were significantly lower in AL-CA patients than in HCM patients and the control subjects. The endocardial-epicardial LSsys difference in all the left ventricle walls were significantly smaller in AL-CA group than in the control group, but this difference appeared variable in HCM group. The LGE also presented with different features in AL-CA and HCM: AL-CA group showed subendocardial LGE in almost all the LV walls, but HCM group showed patchy LGE with a regional, multifocal distribution.

CONCLUSIONAL-CA is characterized by a significantly reduced endocardial LSsys in the LV segments and an uniform decrease of the endocardial-epicardial LSsys difference in all the LV walls, but the changes in HCM appear variable, and 2-dimensional velocity vector imaging is therefore a useful modality to differentiate AL-CA from HCM.

Amyloidosis ; diagnosis ; Cardiomyopathy, Hypertrophic ; diagnosis ; Diagnosis, Differential ; Diastole ; Echocardiography ; Heart Ventricles ; physiopathology ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Systole

OBJECTIVETo assess left ventricular (LV) regional diastolic function in patients with hypertrophic cardiomyopathy (HCM) using single-beat real-time three-dimensional echocardiography (RT-3DE).

METHODSSixty-nine patients with HCM (LV ejection fraction≥45%) and 50 normal control subjects underwent examinations by two-dimensional echocardiography (2DE) and RT-3DE. The parameters analyzed by RT-3DE included the end diastolic sphericity index (EDSI), diastolic dyssynchrony index (DDI), dispersion end diastole [DISPED, including late (DDI-late) and early (DDI-early) diastole, dispersion end diastole (DISPED-late), and dispersion early diastole (DISPED-early)]. The patients were divided into 3 groups with impaired relaxation (n=23), pseudonormal filling (n=37), and restrictive filling (n=9).

RESULTSDDI and DISPED increased with the severity of diastolic dysfunction. In end diastole, DDI-late and DISPED-late were abnormal in cases with severe diastolic dysfunction (7.95∓2.75 and 26.76∓17.19, respectively, P<0.0001) but normal in cases with mild diastolic dysfunction. In early diastole, DDI-early and DISPED-early both increased in cases of mild (8.57∓2.24 and 25.44∓6.31, respectively, P<0.0001) and moderate (9.56∓4.66 and 35.42∓14.19, respectively, P<0.0001) diastolic dysfunction.

CONCLUSIONIn patients with HCM, dyssynchrony in early diastole is more obvious than that in late diastole and shows also a stronger correlation with diastolic dysfunction. This preclinical lesion can be detected by single-beat RT-3DE.

Adult ; Cardiomyopathy, Hypertrophic ; diagnostic imaging ; physiopathology ; Case-Control Studies ; Diastole ; Echocardiography, Three-Dimensional ; Female ; Humans ; Male ; Middle Aged ; Ventricular Dysfunction, Left ; diagnostic imaging ; physiopathology

Medically refractory ventricular tachycardia (VT) storm can be controlled with radiofrequency catheter ablation (RFCA), however, it may be difficult to control in some patients with hemodynamic overload. We experienced a patient with intractable VT storm controlled by hemodynamic unloading. The patient had mid-septal hypertrophic cardiomyopathy with an implantable cardioverter defibrillator (ICD) back-up. Because of the severe mid-septal hypertrophy, his left ventricle (LV) had an hourglass-like morphology and showed apical ballooning; the focus of VT was at the border of apical ballooning. Although we performed VT ablation because of electrical storm with multiple ICD shocks, VT recurred 1 hour after procedure. As the post-RFCA monomorphic VT was refractory to anti-tachycardia pacing or ICD shock, we reduced the hemodynamic overload of LV with beta-blockade, hydration, and sedation. VT spontaneously stopped 1.5 hours later and the patient has remained free of VT for 24 months with beta-blockade alone. In patients with VT storm refractory to antiarrhythmic drugs or RFCA, the mechanism of mechano-electrical feedback should be considered and hemodynamic unloading may be an essential component of treatment.
Cardiomyopathy, Hypertrophic/complications/diagnosis/*physiopathology/therapy ; Catheter Ablation ; Electrocardiography ; Gated Blood-Pool Imaging ; Heart Catheterization ; Humans ; Male ; Middle Aged ; Tachycardia, Ventricular/diagnosis/etiology/*physiopathology/therapy ; Takotsubo Cardiomyopathy/complications/diagnosis/*physiopathology/therapy

OBJECTIVE: To investigate the changes of collagen fibers and the expression of osteopontin in the left ventricle in cases of hypertrophic cardiomyopathy (HCM), along with the significance of their potential forensic application. METHODS: Fifteen cases of HCM, 15 cases of coronary heart disease with cardiac hypertrophy and 20 cases of traffic accidents were selected as HCM group, coronary heart disease group and control group, respectively. Collagen volume fraction and osteopontin expression were observed and compared by HE staining, Masson trichrome staining and immunohistochemistry methods. Imaging and statistical methods were used for quantitative analysis. RESULTS: Collagen volume fraction in left ventricle of HCM and coronary heart disease were significantly higher than that in the control group (P < 0.05), which was not significantly different between the HCM group and the coronary heart disease group. The integral light density value of osteopontin in left ventricular cardiomyocytes of the HCM group and the coronary heart disease group were significantly higher than that of the control group (P< 0.05), and the value of the HCM group was also significantly higher than that of coronary heart disease group (P < 0.05). CONCLUSION The increased contents of collagen fibers and the overexpression of osteopontin may play an important role in myocardial fibrosis, and they can be used as markers in aid of diagnosing sudden death due to HCM.
Cardiomyopathy, Hypertrophic/physiopathology* ; Case-Control Studies ; Collagen/metabolism* ; Coronary Disease/physiopathology* ; Death, Sudden, Cardiac/etiology* ; Female ; Fibrosis ; Forensic Pathology ; Heart Ventricles/pathology* ; Humans ; Immunohistochemistry ; Male ; Myocardium/pathology* ; Osteopontin/metabolism* ; Staining and Labeling

The case is a 54-year-old man with hypertrophic cardiomyopathy, mid-ventricular obstruction, apical aneurysm, and recurrence sustained monomorphic ventricular tachycardia (VT). A coronary angiogram revealed myocardial bridging located in the middle of the left anterior descending coronary artery (LAD), and the left ventriculogram showed an hour-glass appearance of the left ventricular cavity. There was a significant pressure gradient of 60 mmHg across the mid-ventricular obliteration at rest. A successful myectomy of the inappropriate hypertrophy myocardium and excision of the apical aneurysm were performed. Pathologic analysis demonstrated fibrosis in the apical aneurysm and thickened and narrowed vessels in the adjacent area. During the follow-up of eighteen months, the patient remained clinically stable and free from arrhythmic recurrence.
Cardiomyopathy, Hypertrophic ; diagnosis ; physiopathology ; Female ; Heart Aneurysm ; physiopathology ; Humans ; Male ; Middle Aged ; Tachycardia, Ventricular ; physiopathology

We report a case of acute left ventricular failure at one hour after transcatheter closure of a secundum atrial septal defect (ASD) in a 28-year-old man with hypertrophic cardiomyopathy. Afforded noninvasive mechanical ventilation and the administration of intravenous morphine and high doses of furosemide, the patient exhibited improvement of his clinical condition, reduction of pulmonary congestion at chest X-ray, and satisfactory blood gas analyses in twelve hours. Twenty-four hours later, the patient received oral administration of furosemide and metoprolol. After 7 days the patient was discharged in good clinical condition. At follow-up at 12 months, the patient had remained symptomatically improved from NYHA Class III symptoms before the procedure to Class II symptoms. There was no latent arrhythmia at the follow-up examination. Follow-up transthoracic echocardiography estimated an improvement of the left ventricular function. So, transcatheter closure of a secundum ASD in a patient with hypertrophic cardiomyopathy is feasible, and a thorough understanding of the hemodynamic condition of ASD and hypertrophic cardiomyopathy will reduce the complication of ASD closure.
Adult ; Cardiac Catheterization ; Cardiomyopathy, Hypertrophic ; physiopathology ; therapy ; Echocardiography ; Heart Failure ; diagnosis ; pathology ; Heart Septal Defects, Atrial ; physiopathology ; therapy ; Humans ; Male ; Ventricular Function, Left