Although idiopathic cardiomyopathies are prognostically important and are a common indication for cardiac transplantation in all age groups, the incidence and age distribution of idiopathic ...cardiomyopathies in a well-defined pediatric population have been poorly characterized. A retrospective study was carried out in Finland in 1980-1991 to obtain information on the epidemiology of childhood cardiomyopathies. The medical records of all patients aged birth to 20 years with cardiomyopathy from the five university hospitals and 16 central hospitals covering the entire country were reviewed. Moreover, data on causes of death from the Finnish National Census Bureau were examined. Of the 808 potential cases screened, 118 infants, children, and adolescents, representing an average age-specific population of 1.4 million, were definitely identified as having idiopathic cardiomyopathy. The average annual occurrence of new cases was 0.65 per 100,000 population (95% confidence interval (CI) 0.53-0.79). If the 15 cases diagnosed only after death during the 12-year study period were included, the occurrence increased to 0.74 per 100,000 population per year. Fifty-six new cases of dilated cardiomyopathy and 40 new cases of hypertrophic cardiomyopathy were diagnosed during the study period, giving average annual occurrences of 0.34/100,000/year (95% CI 0.26-0.44) and 0.24/100,000/year (95% CI 0.17-0.33) for new cases of dilated and hypertrophic cardiomyopathies, respectively. At the end of 1991, the prevalence of dilated cardiomyopathy was 2.6/100,000 (95% CI 1.8-3.6) and that for hypertrophic cardiomyopathy was 2.9/100,000 (95% CI 2.0-4.0). The number of new cases of dilated cardiomyopathy per year increased over the study period, whereas the annual occurrence of hypertrophic cardiomyopathy remained relatively constant. Marked variability was seen in occurrence among the different age groups of children with dilated cardiomyopathy, suggesting that different pathophysiologic mechanisms, and possibly etiologies, may exist in different age groups.
Dilated cardiomyopathy (DCM) is a common disorder characterized by cardiac dilation and reduced systolic function. To identify a cardiomyopathy gene, we studied a family with DCM associated with ...sinus node dysfunction, supraventricular tachyarrhythmias, conduction delay, and stroke. A general linkage approach was used to localize the disease gene in this family. Linkage to D3S2303 was identified with a two-point lod score of 6.09 at a recombination fraction of 0.00. Haplotype analyses mapped this locus to a 30 cM region of chromosome 3p22-p25, excluding candidate genes encoding a G-protein (GNAI2), calcium channel (CACNL1A2), sodium channel (SCN5A), and inositol triphosphate receptor (ITPR1). These data indicate that a gene causing DCM associated with rhythm and conduction abnormalities is located on chromosome 3p, and represent the first step toward disease gene identification.
To explore the relationship among heart rate turbulence (HRT), QT dispersion (QTd) and heart function in patients with dilated cardiomyopathy (DCM) and assess its clinical value.
A total of 81 DCM ...patients with ventricular premature contraction (VPC) were divided into two groups according to heart function: Group A (NYHA class I-II, n=34) and Group B (NYHA class III-IV, n=47). Thirty out-patient control cases were chosen from those undergoing regular physical examination. 24 hour holter was performed to monitor turbulence onset (TO) and turbulence slope (TS). Electrocardiogram (ECG) was used to assess QTd. Meanwhile left ventricular ejection fraction (LVEF), left ventricular end-diastolic dimension (LVEDD), E and A-wave peak velocities, E/A were measured by echocardiogram. After a comparison of all indicators in each group, an investigation was conducted to discern the relationship among HRT, QTd and heart function.
Compared with normal group, TO significantly increased in DCM A and B group: 0.38 (-0.99-1.85
To assess cardiac twist in dilated cardiomyopathy (DCM) patients using echocardiography velocity vector imaging (VVI) and to explore the clinical application value of VVI in evaluating cardiac twist.
...Thirty-three normal subjects and 30 DCM patients were enrolled. Echocardiographs of parasternal left ventricle basal, papillary muscle level and apical short axis plane, apical four-, two-chamber plane were obtained respectively. Systolic maximal rotation degree, peak rotation velocity, circumferential strain (CS), time to peak rotation velocity (TPRV), peak un-rotation velocity of end diastole and end isovolumic relaxation period in subendocardium were measured by VVI software.
(1) In the normal group, left ventricle performed systolic wring motion with counterclockwise rotation at the apex and clockwise rotation at the base as seen from the apex, while with transient counterclockwise rotation at the base and clockwise rotation at the apex in isovolumic relaxation period. The papillary level rotation form was not constant For the dominant rotation action of the apex, the whole cardiac twist form was counterclockwise. (2) Compared with the control group, 4 DCM patients cardiac twist pattern changed: two showed both counterclockwise rotation of the base and the apex, one represented both clockwise rotation of the base and the apex, another performed the base rotated counterclockwise and the apex rotated clockwise. (3) All rotation and twist parameters of other 26 DCM patients decreased, especially at the apical level: LVtw:7.34 degrees +/- 3.65 degrees vs. 17.01 degrees +/- 4.81 degrees, LVtor: (0.09 +/- 0.04) degrees/mm vs. (0.23 +/- 0.06) degrees/mm, torsion rate: (60.23 +/- 23.67) degrees/s vs. (148.24 +/- 56.23) degrees/s, untwisting rate (0.37 +/- 0.19) degrees/m vs. (0.59 +/- 0.33%)/m, basal CS: (-8.09 +/- 2.73)% vs. (-19.49 +/- 5.51)% (P = 0.013), apical CS: (-8.94 +/- 5.90)% vs. (-27.49 +/- 9.53)% (P = 0.000), basal rotation angle: (-3.60 +/- 2.38) vs. (-6.28 +/- 3.05) (P = 0.014), apical rotation angle: (5.80 +/- 3.55) degrees vs. (11.02 +/- 3.33) degrees (P = 0.001). (4) The apical TPRV in DCM group were longer than the control group represented rotational dyssynchrony in DCM patients (400.26 ms +/- 70.15 ms vs 328.13 ms +/- 66.95 ms, P = 0.008). LVtw correlated positively well with EF (r = 0.489, P < 0.05).
(1) Cardiac twist function was diffusely impaired in DCM patients and it contributed to the global cardiac dysfunction. (2) Cardiac twist pattern changed in some of DCM patients. (3) VVI can objectively reflect cardiac twist function in DCM patients.
To explore the effect of early statin therapy (atorvastatin and simvastatin) on mortality in patients with non-ischemic dilated cardiomyopathy.
A retrospective study was conducted at a single center. ...A total of 315 patients with non-ischemic dilated cardiomyopathy, admitted into our hospital from January 2002 to December 2008, were enrolled. The association of statin therapy at the initial hospitalization with all-cause mortality was evaluated. The median follow-up period was 45.1 months.
By the single-factor analysis, we found that the follow-up mortality was 17.2% in statin group and it was significantly lower than 37.4% of non-statin users (P = 0.003); in patients with worsening cardiac function NYHA III - IV, the mortality of statin group was 17.2% while a much higher mortality of 47.4% was found in non-statin users (P = 0.003); in patients with NYHA I - II, no significant difference was found in mortality between two groups. By the multi-factor analysis adjusting for age, gender, history of hypertension,
It is unknown whether basal release of endothelium-derived nitric oxide in the coronary artery is altered in heart failure in humans. The aim of the present study was to evaluate the effect of ...inhibition of nitric oxide synthesis on basal tone of the conduit and resistance coronary arteries in awake patients. Coronary blood flow velocity (Doppler guide wire) and coronary arterial diameter (quantitative coronary angiography) were measured in 14 patients with heart failure caused by nonischemic left ventricular dysfunction (7 idiopathic dilated cardiomyopathy and 7 valvular insufficiency) and 7 patients with normal ventricular function (controls). Intracoronary N -monomethyl-L-arginine (L-NMMA), an inhibitor of nitric oxide synthesis, at graded doses decreased coronary blood flow in both groups. However, the magnitude of flow reduction was smaller in patients with heart failure than in control patients (P < .0001). The magnitude of coronary blood flow reduction in response to L-NMMA inversely correlated to indexes of left ventricular contractile function (P < .01) but was not affected by the cause of heart failure. Constriction of the large epicardial coronary artery with L-NMMA also tended to be attenuated in patients with heart failure. In summary, vasoconstricting response to L-NMMA was blunted in the coronary resistance artery in heart failure in vivo. These findings suggest that basal release of nitric oxide in the coronary circulation is decreased in patients with heart failure. (Hypertension. 1997;30part 1:50-56.)
Background:
It has been suggested that a genetic polymorphism in the angiotensin II type 1 receptor gene (ATRG) and the ACE gene DD genotype might have a synergistic influence on the risk of ...developing cardiovascular disease.
Aims:
To study the possible interaction between polymorphisms in the ACE gene and the ATRG, regarding survival and left ventricular function.
Methods:
Polymorphism of the two genes was studied in a population‐based cohort of 194 patients with idiopathic heart failure, recruited from the western part of Sweden 1985–1988. The patients were investigated by echocardiography. The survival status was checked during the 7‐year follow‐up period.
Results:
Although there was no statistically significant additive risk of the ATRG polymorphism, patients carrying the ACE gene DD genotype in combination with a C allele of the ATRG tended to have a poorer prognosis. DD + AA, OR 1.24 (95% CI 0.67–2.32, P = 0.49); DD + AC, OR 1.64 (95% CI 0.95–2.83, P = 0.08); DD + CC, OR 3.54 95% CI 0.78–16.1, P = 0.10); DD + AC/CC, OR 1.84 (95% CI 1.10–3.08, P = 0.02). Patients with the DD + AC/CC genotypes tended to have lower ejection fraction and increased left ventricular mass.
Conclusions:
There was a trend toward a worse prognosis in patients with the combination of a C‐allele in the ATRG and the ACE gene DD genotype, suggesting an interaction of these two genetic polymorophisms on disease severity.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
To evaluate the postsystolic shortening (PSS) of different segments of left ventricle (LV) and its meanings in dilated cardiomyopathy (DCM).
Twenty-two normal controls and 14 DCM patients underwent ...tissue velocity imaging (TVI) to obtain the regional velocity profiles of 18 segments of LV. The peak velocities of isovolumic contraction phase (V(IC)), systolic phase (V(S)), and PSS (V(PSS)), the time of V(PSS) (T(PSS)) was measured and the ratio of V(PSS) to V(IC) (V(PSS)/V(IC)), and ratio of V(PSS) to V(S) (V(PSS)/V(S)) were calculated. The active and passive PSS were compared by the standard of V(PSS)/V(IC).
Physiologic PSS was detected only in minority segments of normal subjects and pathologic PSS was detected in all segments of DCM patients. Compared with the physiologic PSS, The V(PSS), V(PSS)/V(IC), and V(PSS)/V(S) of the pathological PSS were increased and the T(PSS) of pathologic velocity of PSS (V(PSS)) were prolonged. Compared with the passive PSS segments, the V(PSS) and V(PSS)/V(S) of active PSS we
We investigated the interrelations between surface electrocardiographic changes and clinical outcomes in children with idiopathic dilated cardiomyopathy (DCMP). 33 patients (19 boys, 14 girls) were ...classified into two groups; group I (15) who were in poor clinical status or dead; and group II (18) who showed good clinical status. Group I had larger LV dimensions compared to group II (Gr I vs. Gr II; LVEDD, 52 +/-11 vs. 42+/-7 (mm); LVESD, 43+/-12 vs. 30+/-5 (mm); p<0.05). QRS duration was prolonged in Gr I compared to Gr II and normal (Gr I, 84+/-28; Gr II, 66+/-12; normal control, 67+/-9). The QRS duration was correlated with the dimensions of left ventricle (LV). Corrected QT and JT interval and dispersions of QT in the DCMP group showed a significant difference compared to the normal control, however there was no significant difference between Gr I and II. In conclusion, QRS duration was correlated with ventricular dimension and clinical outcome in children with idiopathic dilated cardiomyopathy. Irrespective of increased ventricular inhomogeneity, QT dispersion could not be used to predict long-term prognosis.
The authors present the successful use of the Impella recover 100 intracardiac left ventricular assist device in a 55-year old man suffering from end-stage ischemic cardiomyopathy. The pump was ...implanted preoff pump coronary artery bypass grafting (OPCAB) and it was successfully weaned 5 days postoperatively. In addition, an intra-aortic balloon pump (IABP) was implanted to convert the non-pulsatile flow of the Impella into a pulsatile flow. In this paper benefits and risks of this technique are shown.
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