Evaluation of Cardiac Functions of Patients with Benign Joint Hypermobility Syndrome

We sought to investigate whether echocardiography with tissue Doppler imaging identifies myocardial dysfunction in children with benign joint hypermobility syndrome (BJHS). This cross-sectional study enrolled 75 children with BJHS and 70 healthy children. We performed detailed echocardiography in individuals with BJHS without inherited connective tissue disorders. Any congenital or acquired cardiac disease was excluded by clinical and echocardiographic examination. Both groups were similar in terms of age, sex, and body mass index. The diameter of the aortic annulus and sinus valsalva were wider in patients with BJHS. There was no significant differences in ejection fraction or mitral and tricuspid annular plane systolic excursion between the two groups. Pulsed-wave Doppler-derived E/A ratios in mitral and tricuspid valves were similar in both groups. Deceleration time of early mitral inflow was prolonged in patients with BJHS. Mitral and tricuspid annulus Ea velocity were significantly lower in children with BJHS. Ea, Aa, and Ea/Aa ratios in the interventricular septum, left ventricle posterior wall, and right ventricle free wall were lower in patients with BJHS than in the control group. The E/Ea ratio was greater in patients with BJHS than in the control group. Isovolumic relaxation time and right-ventricular (RV) and left-ventricular (LV) myocardial performance indices (MPIs) were greater in patients with BJHS. This study showed the diastolic dysfunction in patients with BJHS. In addition, we detected increased LV and RV MPI. We believe that BJHS may affect proteins of the myocardial cytoskeleton and extracellular matrix.     

The Forgotten Chamber: Right-Ventricular Functions in Juvenile Idiopathic Arthritis

Cardiac involvement, such as pericarditis, myocarditis, and endocarditis, is seen in juvenile idiopathic arthritis. Although there have been some reports about right-ventricular systolic and diastolic functions of adults with rheumatoid arthritis and left-ventricular systolic and diastolic functions of children with JIA, there have been no studies about RV functions of children with JIA. The aim of this study was to determine RV functions in children with JIA. We performed conventional echocardiography and tissue Doppler imaging measurements of the right ventricle of patients with JIA. All patients were in sinus rhythm at the time of examination without overt LV heart failure and with normal LV ejection fraction. Fifty-five children with the diagnosis of JIA and 33 healthy control subjects were included in the study. Peak systolic, early, and late diastolic tricuspid annular velocities were significantly decreased in JIA patients compared with healthy controls (p < 0.05). Isovolumic accelaration (IVA), as a measure of myocardial acceleration during isovolumic contraction of the right ventricle, was also significantly lower in JIA patients than in healthy controls (p < 0.05). RV systolic and diastolic functions, in addition to the previously shown LV functions, are affected in JIA patients. IVA decreases in JIA patients and may be used as an alternative, noninvasive parameter for the assessment of RV systolic function in children with JIA.     

Ventricular diastolic filling indices in pulmonary stenosis

Children with valvar pulmonary stenosis have right ventricular diastolic filling abnormalities that may be due to either right ventricular hypertrophy or right ventricular outflow obstruction. In order to investigate the reason for this abnormality, 23 consecutive cases with pulmonary stenosis (mean age 7.94 +/- 3.33 years) undergoing transluminal pulmonary balloon valvuloplasty without significant tricuspid or pulmonary valvar regurgitation were studied prospectively. Right ventricular diastolic filling indices and pulmonary valvar systolic gradients were measured in these children one day before and after pulmonary balloon valvuloplasty and were re-examined six months later. Right ventricular diastolic indices based on rapid early diastolic filling peak velocity (peak E), peak velocity during atrial contraction (peak A), and ratio of E/A were determined by pulsed Doppler echocardiography. In conclusion, right ventricular diastolic filling indices in patients with pulmonary stenosis did not improve after pulmonary balloon valvuloplasty in the first day but when re-examined by the sixth month there was a significant improvement. These data suggest that diastolic filling abnormalities are more likely a result of right ventricular hypertrophy than of right ventricular outflow obstruction.     

小儿急性风湿热的实验室与心脏器械检查及分析

通过有关实验室与心脏器械检查,以发现急性风湿热(ARF)诊断的新指标。方法对114例 ARF患儿,检查抗链球菌溶血素“O”(ASO),其中60例做了咽部A组β溶血性链球菌快速鉴定(GABHSRA), 且对二者阳性率进行对比;将114例的肌酸激酶同功酶(CK-MB)、多普勒超声心动图,与健康儿童对比。 结果ARF患儿GABHSRA阳性率为90％(54/60),显著高于ASO阳性率65.8％(75/114)。114例ARF患儿 CK-MB升高者46.5％,α-羟丁酸脱氢酶升高者32.7％;超声心动图左房增大者23.7％,左室扩大者16.7％,右 室扩大者7.1％;二尖瓣增厚者13.2％,主动脉瓣增厚者8.8％;多普勒超声心动图检查二尖瓣返流者29.8％, 主动脉瓣返流者9.6％,与健康儿童相比,差异均有显著性意义。34例有二尖瓣返流患儿中29例二尖瓣返流流 速时间积分增大。结论GABHSRA、心肌酶和多普勒超声心动图检查有助于ARF的诊断与病情了解。     

The Efficacy of Cardiac Findings in Assessing the Outcome in Preterms with Bronchopulmonary Dysplasia

Objectives

To evaluate if cardiac dysfunctions are important in assessing the outcome in newborns with Bronchopulmonary Dysplasia (BPD), by evaluating cardiac functions with N-terminal prohormone of brain natriüretic peptide (NT-proBNP) levels, M-mode and tissue doppler echocardiography at 6–12 mo of age.

Methods

Twenty eight patients were retrospectively classified as mild, moderate and severe according to the diagnostic criterias for BPD. All cases were assessed with standard M-mode, tissue doppler echocardiography and NT-proBNP levels. Control group consisted of 28 healthy infants, having similar postnatal ages as patients and were assessed with standard M-mode and tissue doppler echocardiography.

Results

The age of patients with BPD was 9.8?±?2.3 mo and control group was 9.5?±?2.6 mo. There was no significant difference between the postnatal ages of two groups (p?>?0.05). Neither pulmonary hypertension nor pulmonary/tricuspid regurgitation was detected. The M-mode echocardiography measurements did not differ between patients and control group (p?>?0.05). Tissue doppler echocardiography, tricuspid valve medial segment early diastolic myocardial relaxation velocity (TME’) measurements of patients were found significantly lower, peak transtricuspid filling velocity in the early diastole (TE)/TME’ ratios and isovolumetric relaxation time (IVRT) measurements were found significantly higher than control group (p?<?0.05). Tricuspid E, TE/TLE’ (Tricuspid valve lateral segment early diastolic myocardial relaxation velocity), TE/RVLE’(Right ventricular lateral segment early diastolic myocardial relaxation velocity), TE/TME’ levels were also found as significantly abnormal in patients with severe BPD. A significant correlation was found between right ventricular diastolic disfunctions and severity of BPD (p?<?0.05). No statistically significant difference was found between NT-proBNP levels, BPD stages and tissue doppler echocardiography measurements (p?>?0.05).

Conclusions

This is the first study evaluating cardiac findings in patients with BPD by tissue doppler echocardiography and NT-proBNP at the same time. On the basis of cardiac evaluations, tissue doppler echocardiography measurements were found as significant and specific for the early assessment of right ventricular diastolic disfunctions.     

Cardiovascular Effects of Acute Bronchiolitis

ABSTRACT. Twenty-one children with normal hearts were studied during acute bronchiolitis. Doppler echocardiography showed tricuspid valve regurgitation in 11 patients, many of whom had evidence of raised pulmonary artery systolic pressure. Serial studies in those with severe infection showed that tricuspid regurgitation disappears with clinical improvement.     

Cardiovascular effects of acute bronchiolitis

Twenty-one children with normal hearts were studied during acute bronchiolitis. Doppler echocardiography showed tricuspid valve regurgitation in 11 patients, many of whom had evidence of raised pulmonary artery systolic pressure. Serial studies in those with severe infection showed that tricuspid regurgitation disappears with clinical improvement.     

Noninvasive cardiac evaluation of young patient with cystic fibrosis

Cardiac involvement was evaluated by echocardiography in 26 young cystic fibrosis patients. The mean age was 48.4 months (range 3 months to 15 years). The findings were compared with 26 age- and sex-matched children without a history of cardiopulmonary complaints. All patients had normal values of left ventricular ejection fraction and fractional shortening. Interventricular septal and posterior left ventricular wall thicknesses were similar to control group but right ventricular free wall thickness was found greater than in the control group. Abnormal septal motion was documented in six patients. Right ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in two patients and there was a negative correlation with clinical Shwachman scores (r: -0.55). Left ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in five patients. For both mitral and tricuspid valves, the mean ratios of peak velocity during passive filling (E) phase of diastole to peak velocity during atrial contraction (A) phase were found significantly lower than in the control group (p < 0.05). Early diastolic peak velocity was similar to that in the control group but late atrial peak velocity was higher in the patient group (p < 0.05). Isovolumic relaxation time was found the same as in the control group. We conclude that cardiac changes in diastolic and systolic functions begin at very young ages in cystic fibrosis patients.     

Ratio of Early Diastolic Tricuspid Inflow to Tricuspid Lateral Annulus Velocity Reflects Pulmonary Regurgitation Severity but Not Right Ventricular Diastolic Function in Children With Repaired Tetralogy of Fallot

The current study assessed relationships between the ratio of early diastolic tricuspid inflow to tricuspid lateral annular velocity (tricuspid E/e′) and right ventricular (RV) function in children after tetralogy of Fallot (TOF) repair. The RV function of 25 asymptomatic children with surgically repaired TOF (age 3.3 ± 2.0 years) was assessed by echocardiography and cardiac catheterization. Right ventricular end-diastolic pressure and volume (RVEDP and RVEDV), systolic pressure, and ejection fraction, as well as mean pulmonary arterial pressure, mean right atrial pressure (RAP), and the severity of both pulmonary regurgitation (PR) and tricuspid regurgitation (TR) were assessed in terms of the contribution to tricuspid E/e′. Univariate analysis discovered a relationship between tricuspid E/e′ and RVEDV (R ² = 0172), pressure half-time of PR (PR-PHT) (R ² = 0.173), and TR grade (R ² = 0.145) (p < 0.01 for each). After multivariate adjustment, PR-PHT was significantly associated with tricuspid E/e′ (β = 0.210; p < 0.001). Tricuspid E/e′ was not significantly associated with RVEDP or RAP. In conclusion, tricuspid E/e′ does not indicate RV diastolic function but reflects the severity of PR in asymptomatic children after TOF repair.     

Tissue Doppler Echocardiography Before and After the Surgical Reconstruction of an Insufficient Mitral Valve in a Patient with Fontan Circulation

We report a patient with tricuspid atresia after total cavopulmonary connection who developed significant mitral valve regurgitation. Doppler myocardial echocardiography tracings showed "pseudonormalization" of diastolic velocities. After successful surgical reconstruction of the mitral valve, the diastolic myocardial velocities changed to the typical Fontan physiology with predominant late diastolic ventricular filling.     

Echocardiographic Evaluation of Systolic and Diastolic Function in Postoperative Coarctation Patients

Postoperative coarctation (CoA) patients are often found to have signs of persistent myocardial dysfunction. M-mode echocardiography was performed to study left ventricular (LV) size, mass, and systolic function and Doppler ultrasonography to study LV filling and flow velocity in the LV outflow tract and aorta in 28 ``healthy' postoperative CoA patients (5–21 years) and 28 age- and sex-matched controls. The early (E) and late (A) diastolic transmitral velocities were significantly higher in the patient group than in the controls (p < 0.05). Other diastolic parameters (isovolumic relaxation time, E/A ratio, and deceleration time of the early diastolic velocity) were similar in the two groups (p= NS). The left atrial diameter, LV wall average, end-diastolic diameter of the LV and LV mass were higher in the patients than controls (p < 0.05). The patients also had a higher stroke volume, cardiac output, and cardiac index than the controls (p < 0.01). The fractional shortening was similar in the two groups (p= NS). Blood flow velocities in both the LV outflow tract and aorta were higher in the patients than the controls (p < 0.0002). We found LV hypertrophy with signs of a hyperdynamic circulation (increased cardiac index and stroke volume) in our patients. An increase in A is associated with LV hypertrophy and seems to be a sensitive marker of diastolic abnormality. The rise in E is paradoxical but has been seen in other studies. The increased blood flow velocity in the LV outflow tract suggests obstruction to LV outflow and increased flow in the descending aorta is due to residual coarctation.     

Noninvasive evaluation of systolic pressures of pulmonary artery and right ventricle using contrast-enhanced doppler echocardiography: Comparative study using sonicated albumin or glucose solution

Evaluation of pulmonary arterial pressure is essential for the diagnosis and management of patients with congenital heart disease; it is usually done by cardiac catheterization. An alternative, noninvasive method may be clinically more useful. The purpose of this study was to assess the usefulness of the noninvasive determination of systolic pressure of the pulmonary artery and right ventricle by contrast-enhanced Doppler echocardiography. We selected 30 pediatric patients (28 with trivial or nonsignificant tricuspid regurgitant Doppler signals and 2 with significant tricuspid regurgitant Doppler signals) aged 2 months to 21 years. The flow velocity of tricuspid regurgitation was measured with continuous-wave Doppler of the right ventricular inflow view or left parasternal or apical four-chamber view before and after injection of two types of contrast medium (hand-agitated 5% glucose or sonicated albumin). The systolic pressure of the pulmonary artery was assessed as the estimated right ventricular systolic pressure (albumin method) minus the peak pressure gradient across the pulmonary valve (nonenhanced Doppler method). After injection of hand-agitated 5% glucose and sonicated albumin, trivial tricuspid regurgitation signals were enhanced in 25 of 28 patients (89%). In two patients, spectral envelopes were well defined enough to obtain the peak systolic velocity of the tricuspid regurgitation jet without contrast medium injection. Peak velocity was not altered by injection of contrast medium in these patients. There was significant correlation between the estimation by contrast-enhanced Doppler using hand-agitated 5% glucose and the cardiac catheterization measurement of the transtricuspid pressure gradient (r= 0.88). The transtricuspid pressure gradients obtained by continuous-wave Doppler during sonicated albumin enhancement corresponded closely to those measured by cardiac catheterization (r = 0.95). Pulmonary arterial and right ventricular systolic pressures measured by Doppler using sonicated albumin and those obtained by cardiac catheterization were highly correlated (right ventricle, r = 0.96; pulmonary artery, r = 0.95). In conclusion, this technique may be a valuable noninvasive method for determining accurate right ventricular and pulmonary arterial systolic pressures.     

The right ventricle in Fabry disease

AIM: Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease. METHODS: Echocardiographic examination of the right and left ventricle was carried out in 129 patients (80 women and 49 men) with Fabry disease. RESULTS: RV hypertrophy was present in 46 patients (35.7%). Of these patients, 13 showed signs of severely depressed right systolic function (tricuspid annulus movement < 10 mm and a prolonged RV pre-ejection period/pulmonary ejection time ratio) and six patients showed additional severe depression of parameters of diastolic function (pseudo-normal or restrictive RV filling pattems). Those patients with RV hypertrophy and severely compromised systolic and diastolic function had the highest LV masses (92 +/- 11.7 g/m(2.7)). CONCLUSION: RV involvement is common in Fabry disease and ultimately progresses to severe systolic and diastolic RV dysfunction. These findings might explain why patients with preserved LV function can develop clinical features such as reduced exercise capacity, organomegaly and lymphoedema.     

The right ventricle in Fabry disease

Aim: Left ventricular (LV) hypertrophy is a common feature in Fabry disease-related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease. Methods: Echocardiographic examination of the right and left ventricle was carried out in 129 patients (80 women and 49 men) with Fabry disease. Results: RV hypertrophy was present in 46 patients (35.7%). Of these patients, 13 showed signs of severely depressed right systolic function (tricuspid annulus movement <10 mm and a prolonged RV pre-ejection period/pulmonary ejection time ratio) and six patients showed additional severe depression of parameters of diastolic function (pseudo-normal or restrictive RV filling patterns). Those patients with RV hypertrophy and severely compromised systolic and diastolic function had the highest LV masses (92±11.7 g/m^2.7).
Conclusion: RV involvement is common in Fabry disease and ultimately progresses to severe systolic and diastolic RV dysfunction. These findings might explain why patients with preserved LV function can develop clinical features such as reduced exercise capacity, organomegaly and lymphoedema.     

Left Ventricular Diastolic Dysfunction in Children and Young Adults with Marfan Syndrome

Adults with Marfan syndrome (MFS) demonstrate abnormal aortic elastic properties manifest by decreased aortic distensibility and increased aortic stiffness. Left ventricular (LV) diastolic dysfunction has been reported in adults with MFS. The objective of this study was to assess the frequency of LV diastolic dysfunction in a group of children and young adults with MFS and to determine whether diastolic dysfunction is associated with hemodynamic alterations of the aorta. Review of echocardiographic findings in 40 patients with MFS was performed to assess LV size, systolic function, isovolumic relaxation time (IVRT), mitral inflow velocities, deceleration time (DT) of mitral E wave, and aortic root dimension. No patient had significant valvar disease or was on any cardiac medication at the time of study. A group of 40 age and sex-matched healthy subjects undergoing echocardiography served as controls. Significant differences in LV diastolic function were found between MFS patients and controls. MFS patients had prolonged DT and IVRT and decreased mitral E/A ratio, suggesting impaired LV relaxation. No relationship between aortic root dimension and diastolic performance was identified. Left ventricular diastolic dysfunction may be an early marker of myocardial involvement in young MFS patients occurring independently of aortic root dilatation.     

Mitochondrial myopathy,encephalopathy, lactic acidosis,and stroke-like episodes (MELAS) decrease in diastolic left ventricular function assessed by echocardiography

Summary Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) are known to be associated with cardiomyopathy. Systolic and diastolic left ventricular functions were assessed by M-mode and Doppler echocardiography in four patients with MELAS and in 14 normal controls. The interventricular septal thickness and left ventricular posterior wall thickness were greater (11.0±1.6 mm vs. 5.8±0.7 mm and 11.0±2.2 mm vs. 5.9±0.8 mm) in patients with MELAS than in a control group. Parameters of systolic left ventricular functions (ejection fraction, shortening fraction, systolic time intervals, and mean Vcf) and left ventricular dimensions were not significantly different between the two groups. To assess the diastolic function, blood flow velocity across the mitral valve was measured by Doppler echocardiography and various indexes were obtained. In patients with MELAS, the impairment of diastolic left ventricular filling was demonstrated by decrease in the following indexes: peak flow velocity in the early passive filling period (E) (0.76±0.10 m/s vs. 0.94±0.09 m/s), integrated velocity for total E (10.2±1.3 vs. 13.0±0.9), the ratio of E and late atrial filling integrated velocities (1.72±0.06 vs. 2.49±0.29).     

Early detection of doxorubicin and daunorubicin cardiotoxicity by echocardiography: Diastolic versus systolic parameters

Doxorubicin and daunorubicin are effective anticancer agents in children, however, their therapeutic value is limited by myocardial cardiotoxicity. In 14 children (median age 5,0 years, range 3–12) prospective studies were performed using pulsed Doppler echocardiography to assess the changes in left ventricular systolic and diastolic filling dynamics. None of these children developed cardiomyopathy. M-mode echocardiographic systolic parameters and Doppler transmitral flow velocities were analysed at baseline, after a cumulative anthracycline dose of 138±26 mg/m² (second examination) and after 240±15 mg/m² (third examination). At the second examination the acceleration time/ejection time ratio was significantly reduced (P<0.01), but this was no longer evident at the third examination. There was no significant change of peak velocity over aortic valve, preejection period and change of velocity over time. In contrast, three diastolic parameters changed significantly; the late over early inflow velocity (P<0.05), mitral valve late time velocity integral (P<0.01 at the second andP<0.05 at the third examination) and the ratio A-TVI/TVI (P<0.025 andP<0.01). At the third examination the velocity of the A wave was also significantly increased.Conclusion In anthracyline treated children left ventricular diastolic function deteriorates before systolic function. Diastolic function parameters should be used rather than systolic parameters to monitor these patients.     

Tissue Doppler,speckling tracking and four-dimensional echocardiographic assessment of right ventricular function in children with dilated cardiomyopathy

BACKGROUNDRight ventricular (RV) function is frequently overlooked during dilated cardiomyopathy (DCM) evaluation. AIMTo evaluate RV function in children with idiopathic DCM using relatively recent echocardiographic modalities. METHODSWe prospectively studied the cardiac function in 50 children with idiopathic DCM and 50 healthy children as a control group, using four-dimensional echocardiography (4-DE), Tissue Doppler Imaging (TDI), and two-dimensional-speckles tracking echocardiography (2-D-STE). RV EF was measured by 4-DE. RESULTSThe auto left (LV) ejection fractions (EF) measured by 2-D-STE were significantly lower in the patients'' group than in the control. The sphericity index was also significantly lower in children with DCM than in the control. RV EF measured by 4-DE was significantly lower in the patient''s group than the control. RV S wave, e´/a'' ratio, myocardial performance index (MPI), and tricuspid annular plane systolic excursion (TAPSE) were significantly impaired in children with DCM than in control. Both LV and RV global longitudinal strains (GLS) were significantly reduced in children with DCM than in control. RVGLS was significantly associated with the duration since diagnosis, tricuspid annulus S wave, RV MPI, and TAPSE, but not with the age of the patients, RV EF, or e´/a'' ratio. CONCLUSIONThere was impairment of the RV LGS and other systolic and diastolic parameters in children with DCM. STE and TDI can help to detect the early decline of RV function.     

Echocardiographic findings in endomyocardial fibrosis.

An 18-month-old infant diagnosed as having endomyocardial fibrosis by echocardiography is presented. Most patients with endomyocardial fibrosis reported in the literature are either older children or adults. To our knowledge, our patient was the youngest ever to have been reported. Echocardiographic studies showed obliteration of the left ventricular apex and increased echo reflectance at the left ventricular endocardium and subendocardium. The left atrium and right ventricle were significantly enlarged. Doppler echocardiography showed minimal mitral, but significant tricuspid regurgitation. In regard to the contribution of echocardiography in the diagnosis, we recommend this method for suspected cases. Contrary to the other patients reported, there was no thickening of the atrioventricular valves. Mitral valve insufficiency was related to the restriction of the ventricular filling rather than to valve involvement occurring with the disease.     

Systolic and diastolic function in children with chronic renal failure

BACKGROUND: Cardiovascular complications are the most frequent cause of death in patients with end-stage renal failure (ESRF). We aimed to investigate systolic and diastolic functions in children with ESRF. METHODS: Thirty-nine children with ESRF (17 on continuous ambulatory peritoneal dialysis (CAPD), eight on hemodialysis and 14 on predialysis) were examined to assess systolic and diastolic functions by echocardiography and ultrasound Doppler. Left ventricular systolic and diastolic functions were measured both in patients and age-matched healthy controls (n = 20) and the indices of cardiac performance were compared. RESULTS: Increased left ventricular mass index (LVMI) and decreased volume/mass ratio with normal systolic left ventricular function was found in patients, as compared with controls. Left ventricular diastolic dysfunction was observed in dialysis patients. In most of these patients, left ventricular isovolumic relaxation time was prolonged, except in CAPD patients. The peak of late diastolic flow (A) velocities were increased with a reduction of the early diastolic flow velocity (E)--the E/A ratio. The E velocities were unchanged in all patients as compared with controls. Our data indicated an abnormality of myocardial relaxation in patients with ESRF. We found no relationship between E/A ratio and LVMI. Among three groups of patients, the LVMI and diastolic abnormalities were highest in the hemodialysis group indicative of poor control of hypervolemia and hypertension. CONCLUSIONS: The technique of CAPD has some advantages as a renal replacement therapy for preserving cardiac functions as compared with hemodialysis. However, it must be remembered that patients with hemodialysis have features that effects cardiac status, such as higher volume load and higher afterload (hypertension).