Biventricular Ejection Fraction Research Articles

Unexplained exertional dyspnea caused by low ventricular filling pressures: results from clinical invasive cardiopulmonary exercise testing.

To determine whether low ventricular filling pressures are a clinically relevant etiology of unexplained dyspnea on exertion, a database of 619 consecutive, clinically indicated invasive cardiopulmonary exercise tests (iCPETs) was reviewed to identify patients with low maximum aerobic capacity (V̇o2max) due to inadequate peak cardiac output (Qtmax) with normal biventricular ejection fractions and without pulmonary hypertension (impaired: n = 49, V̇o2max = 53% predicted [interquartile range (IQR): 47%-64%], Qtmax = 72% predicted [62%-76%]). These were compared to patients with a normal exercise response (normal: n = 28, V̇o2max = 86% predicted [84%-97%], Qtmax = 108% predicted [97%-115%]). Before exercise, all patients received up to 2 L of intravenous normal saline to target an upright pulmonary capillary wedge pressure (PCWP) of ≥5 mmHg. Despite this treatment, biventricular filling pressures at peak exercise were lower in the impaired group than in the normal group (right atrial pressure [RAP]: 6 [IQR: 5-8] vs. 9 [7-10] mmHg, P = 0.004; PCWP: 12 [10-16] vs. 17 [14-19] mmHg, P < 0.001), associated with decreased stroke volume (SV) augmentation with exercise (+13 ± 10 [standard deviation (SD)] vs. +18 ± 10 mL/m(2), P = 0.014). A review of hemodynamic data from 23 patients with low RAP on an initial iCPET who underwent a second iCPET after saline infusion (2.0 ± 0.5 L) demonstrated that 16 of 23 patients responded with increases in Qtmax ([+24% predicted [IQR: 14%-34%]), V̇o2max (+10% predicted [7%-12%]), and maximum SV (+26% ± 17% [SD]). These data suggest that inadequate ventricular filling related to low venous pressure is a clinically relevant cause of exercise intolerance.

Comparison of right and left ventricular function and size in Duchenne muscular dystrophy

IntroductionRight ventricular (RV) size and function in Duchenne muscular dystrophy (DMD) have not been well described. Using cardiac magnetic resonance (CMR) imaging we describe the relationship of RV and left ventricular (LV) size and function in a large DMD cohort. MethodsLatest CMR scans of 272 patients consecutively seen at a single tertiary referral center (2011–2014) with skeletal muscle biopsy confirmed DMD were included. 1.5 and 3 Tesla CMR scanners were used. Biventricular ejection fraction (EF), end-diastolic volume index (EDVI), mass and mass index were compared across categories of LVEF. ResultsMean age was 13.5±4.9 years. 71% had normal (≥55%) LVEF while mild (EF 45–54%), moderate (EF 30–44%), and severe LV dysfunction (EF <30%) was present in 20%, 6% and 3% respectively. The correlation between RVEF and LVEF was weak. Even in patients with severe LV dysfunction, RVEF (49.7%±12.9%) was relatively preserved. There were no significant differences in RVEDVI and RV mass index across categories of LV function. ConclusionIn a large DMD cohort, RVEF was relatively preserved and RV size was preserved across categories of LV dysfunction.

Ventricular and pulmonary vascular remodeling induced by pulmonary overflow in a chronic model of pretricuspid shunt

Current preclinical models of pulmonary arterial hypertension do not reproduce the clinical characteristics of congenital heart anomalies. Aortocaval shunt is relevant to a variety of clinical conditions. The pathophysiology and possible determination of pulmonary hypertension in this model are still undefined. A method to create a standardized and reproducible aortocaval shunt was developed in rats. After creation of the shunt, the animals were followed up for 20 weeks and a sham laparotomy was used as a control. The chronic effects of volume overload on the right and left ventricles and pulmonary hemodynamic modifications were evaluated by biventricular catheterization, echocardiography, and magnetic resonance. Pulmonary vascular changes were defined by histology. An increased right ventricular end-diastolic area was confirmed by echocardiography. Left ventricular overload and decreased biventricular ejection fraction were demonstrated by magnetic resonance after 20 weeks in the shunt group compared with the controls (left ventricle, 50% ± 5% vs 62% ± 3%, P = .029; right ventricle, 53% ± 2% vs 65% ± 2%, P = .036). Preload recruitable stroke work of left and right ventricles decreased after 20 weeks in shunt rats (left ventricle: 36 ± 7 vs 98 ± 5, P = .004; right ventricle: 19 ± 2 vs 32 ± 9, P = .047). At the same time point, catheterization showed that effective pulmonary arterial elastance was increased only in the shunt group (1.29 ± 0.20 vs 0.14 ± 0.06 mm Hg/μL; P = .004). Histology showed medial hypertrophy, small artery luminal narrowing, and occlusion. The aortocaval shunt model reliably produces right ventricular volume overload and secondary pulmonary hypertension. Due to a combination of left ventricular dysfunction and pulmonary overflow, the pulmonary hypertension produced shows features similar to those found in patients with chronic atrial-level shunt.

Impaired Right and Left Ventricular Function in Asymptomatic Children with Repaired Tetralogy of Fallot by Two‐Dimensional Speckle Tracking Echocardiography Study

Early detection of right ventricular (RV) and left ventricular (LV) dysfunction in patients with repaired tetralogy of Fallot (TOF) is essential because dysfunction is correlated with a poor clinical outcome. The aim of this study was to assess RV and LV function in asymptomatic children with repaired TOF by two-dimensional ultrasound speckle tracking echocardiography (STE). Fifty-six asymptomatic children with a preserved biventricular ejection fraction (EF) after repair of TOF and 35 healthy control subjects were studied. RV and LV strain and strain rate were measured by STE. RVEF and pulmonary regurgitation (PR) were assessed using cardiac magnetic resonance imaging. Compared with the control subjects, RV regional longitudinal strain and strain rate and global longitudinal strain (GLS) and strain rate (GLSR) were impaired in children with repaired TOF. Likewise, LV circumferential and radial strain and strain rate were reduced in patients with TOF. In contrast, longitudinal strain and strain rate did not differ between the groups. RV and LV GLSR were correlated with postoperative follow-up period (r1 = -0.44; r2 = -0.48). RV GLS and GLSR were associated with RVEF (r1 = 0.64; r2 = 0.60) and PR (r1 = -0.48; r2 = -0.49). LV circumferential strain rate was related to PR (r = -0.45). STE can identify abnormalities that may represent early impairment of RV and LV systolic function in postoperative TOF patients with a preserved EF. PR is associated with decreased biventricular performance in repaired patients. STE-derived strain and strain rate may be useful indices for detecting the early deterioration of biventricular performance in patients with TOF.

Titin: a phenotype-genotype descriptive comparison of dilated cardiomyopathy

Background Cardiovascular magnetic resonance (CMR) imaging is the gold standard imaging modality for characterization of dilated cardiomyopathy (DCM). We recently identified genetic variants that truncate the giant protein Titin (TTN) accounting for up to 25% of DCM cases: a discovery that will increase the utility of genetic testing and family screening in DCM. However, the clinical consequences of TTN truncating variants (TTNtv) are unknown. Here, we integrated the power of CMR and capacity of next generation sequencing (NGS) to assess the relationship between genotype and phenotype in a non-ischemic DCM population. Methods 319 patients referred for characterization of DCM were recruited and underwent quantitative CMR study with detailed phenotyping (Siemens Avanto or Siemens Sonata 1.5T scanners). Additional clinical data were collected. TTN genotypes were determined by target enrichment using SureSelect and NGS sequencing using the SOLiD 5500 platform. Results TTN truncating variants (TTNtv) were identified in 13% (n = 42) of cases, the commonest genetic cause of DCM. Table 1 compares the CMR and clinical characteristics of TTNtv-positive and TTNtv-negative cohorts. TTNtv-positive DCM patients had lower left ventricular (LV) ejection fraction (EF) (p = 0.02), thinner LV walls (p < 0.02) and higher incidence of sustained ventricular tachycardia (VT) (p = 0.001) which was independent of EF. No statistically significant difference in the prevalence of midwall fibrosis existed between the two groups. Multivariate linear regression models that considered age, sex, TTNtv presence and position (distance from the protein N-terminus) indicated that TTNtv position was significantly correlated with biventricular ejection fraction and indexed stroke volumes such that more distal truncations had worse indices of cardiac function. For example, a C-terminal TTNtv would be associated with an absolute reduction in LV EF of 18 ± 7%, (p = 0.005), as compared with an N-terminal TTNtv. Conclusions These data highlight the prevalence of TTNtv in this unselected DCM cohort and suggest that TTNtvpositive DCM patients may benefit from tailored clinical management. Sustained VT, LV wall thickness and LVEF influence DCM outcomes. The increased risk of sustained VT in TTNtv-positive DCM may warrant a lower threshold for device therapy, as recommended for DCM caused by LMNA mutations. As distal TTNtv were associated with worse LV function, more aggressive clinical management may be appropriate in patients carrying these variants. The phenotypic expression of genetic substrates can be highly variable, and CMR is central to the accurate phenotypic description of this common yet heterogenous condition. Enhanced molecular understanding may lead to strategies to alter disease course beyond current pharmacological and device based therapy.

New insights into the mechanisms involved in B-type natriuretic peptide elevation and its prognostic value in septic patients.

IntroductionElevated plasma B-type natriuretic peptide (BNP) levels in patients with critical sepsis (severe sepsis and septic shock) may indicate septic cardiomyopathy. However, multiple heterogeneous conditions may also be involved in increased BNP level. In addition, the prognostic value of BNP in sepsis remains debatable. In this study, we sought to discover potential independent determinants of BNP elevation in critical sepsis. The prognostic value of BNP was also evaluated.MethodsIn this observational study, we enrolled mechanically ventilated, critically septic patients requiring hemodynamic monitoring through a pulmonary artery catheter. All clinical, laboratory and survival data were prospectively collected. Plasma BNP concentrations were measured daily for five consecutive days. Septic cardiomyopathy was assessed on day 1 on the basis of left and right ventricular ejection fractions (EF) derived from echocardiography and thermodilution, respectively. Mortality was recorded at day 28.ResultsA total of 42 patients with severe sepsis (N = 12) and septic shock (N = 30) were ultimately enrolled. Daily BNP levels were significantly elevated in septic shock patients compared with those with severe sepsis (P ≤0.002). Critical illness severity (assessed by Acute Physiology and Chronic Health Evaluation II and maximum Sequential Organ Failure Assessment scores), and peak noradrenaline dose on day 1 were independent determinants of BNP elevation (P <0.05). Biventricular EFs were inversely correlated with longitudinal BNP measurements (P <0.05), but not independently. Pulmonary capillary wedge pressures (PCWP) and volume expansion showed no correlation with BNP. In septic shock, increased central venous pressure (CVP) and CVP/PCWP ratio were independently associated with early BNP values (P <0.05).Twenty-eight-day mortality was 47.6% (20 of 42 patients). Daily BNP values poorly predicted outcome; BNP on day 1 > 800 pg/ml (the best cutoff point) fairly predicted mortality, with a sensitivity%, specificity% and area under the curve values of 65, 64 and 0.70, respectively (95% confidence interval = 0.54 to 0.86; P = 0.03). Plasma BNP levels declined faster in survivors than in nonsurvivors in both critical sepsis and septic shock (P ≤0.002). In septic shock, a BNP/CVP ratio >126 pg/mmHg/ml on day 2 and inability to reduce BNP <500 pg/ml implied increased mortality (P ≤0.036).ConclusionsThe severity of critical illness, rather than septic cardiomyopathy, is probably the major determinant of BNP elevation in patients with critical sepsis. Daily BNP values are of limited prognostic value in predicting 28-day mortality; however, fast BNP decline over time and a decrease in BNP <500 pg/ml may imply a favorable outcome.

A case of biventricular involvement of Takotsubo cardiomyopathy: 3D echocardiographic imaging

An 86-year-old woman was admitted to our institution exhibiting drowsy mentality due to hyperkalemia. Electrocardiography revealed symmetric T wave inversions at the anterior, inferior, and lateral leads (Fig. 1a). Threedimensional echocardiography (iE33 xMATRIX with X5-1 transducer, QLAB software analysis using Cardiac 3D Quantification Advanced; Philips, Eindhoven, The Netherlands) revealed akinetic apical segments and hyperkinetic basal segments of the left ventricle (Fig. 1b, Video 1 in Supplementary Material) and dyskinetic apical segments of the right ventricle with a mildly decreased biventricular ejection fraction (Fig. 1c, Video 2 in Supplementary Material). Her coronary angiogram was normal. After 3 weeks, follow-up echocardiography showed normal wall motion and a good ejection fraction of the biventricle. Biventricular involvement of Takotsubo cardiomyopathy is not uncommon if close attention is paid to right ventricular function [1–3]. Three-dimensional echocardiography is a useful tool to detect right ventricular involvement of Takotsubo cardiomyopathy and to estimate its function and morphology at the same time as assessing left ventricle.

MRI Survey In Transfusion-Dependent and Non-Transfusion-Dependent MDS Patients

IntroductionSeveral studies have shown cardiac diseases as causes of death in myelodisplastic (MDS) patients receiving transfusions. So iron overload may be considered an independent negative prognostic factor. There are few and rather contradictory studies using Magnetic Resonance Imaging (MRI) in the evaluation of myelodysplastic syndromes. We report the baseline MRI findings at the end of the recruitment in the MIOMED (Myocardial Iron Overload in MyElodysplastic Diseases) study. In particular, we investigated myocardial iron overload (MIO), hepatic iron overload and biventricular functional parameters in MDS patients, outlying the differences between transfusion dependent and non transfusion dependent patients. MethodsMIOMED is an observational, MRI multicentre study in low and intermediate-1 risk MDS patients who have not received regular iron chelation therapy. Out of the 51 MDS patients enrolled, 48 underwent the baseline MRI exam. Mean age was 71.7±8.5 years and 17 patients were females. Hepatic T2* values were assessed in a homogeneous tissue area and converted into liver iron concentration (LIC). MIO was assessed using a multislice multiecho T2* approach. Biventricular function parameters were quantified by cine sequences. ResultsThe mean global heart T2* was 38.7±8.3 ms while the mean LIC was 7.6±8.8 mg/g/dw. Global heart T2* values were not significantly correlated with LIC or serum ferritin levels while a significant association between LIC and serum ferritin was detected (R=0.689; P<0.0001).Thirty-two (66.6%) patients were non-transfusion dependent while 16 patients were transfusion-dependent. The two groups were homogeneous for age, sex and hemoglobin levels but transfusion-dependent patients had significantly higher serum ferritin levels (1612±864 vs 711±430; P<0.0001). The percentage of patients with detectable hepatic iron (LIC≥3 mg/g/dw) was significantly higher in the transfusion-dependent group (Figure 1, left). Mean LIC was 14.4±11.1 mg/g/dw in the transfusion-dependent group and 4.2±4.6 mg/g/dw in the non-transfusion-dependent group (P<0.0001). A significant heart iron (global heart T2* value <20 ms) was found in two patients, in both patients an heterogeneous pattern (some segments with T2* values >20 ms and other segments with T2* values <20 ms) was detected. Out of two patients with significant heart iron one patient was not transfused and he did not show significant hepatic iron (LIC=2.12 mg/g/dw). The other one patient was regularly transfused and he received sporadically (less than two weeks/month) chelation treatment with deferoxamine in the 2 years before the MRI. The global heart T2* (Figure 1, right), the pattern of iron burden and the number of segments with T2*<20 ms were comparable between the two groups. Biventricular end-diastolic volume index, biventricular ejection fraction and left ventricular (LV) mass index were comparable between the two groups. [Display omitted] ConclusionsAs expected, regularly transfused MDS patients showed significantly higher levels of hepatic iron overload, that, however, was present in almost the 30% of non-transfusion-dependent patients, mainly due to increased intestinal iron and augmented erythropoiesis. MIO is not frequent in MDS patients and it is not correlated with LIC and serum ferritin levels. Conversely, MIO can be present also in non-transfusion dependent patients and in absence of detectable hepatic iron. These data remark the importance to check directly for heart iron with a more sensitive segmental approach avoiding to estimate heart iron burden from indirect indicators such as LIC, serum ferritin or transfusion state. Disclosures:No relevant conflicts of interest to declare.

Hypothyroidism and Cardiac Complications In Thalassemia Major Patients

IntroductionIn the non-thalassemic population hypothyroidism has been associated with an increased risk of cardiac disease while the link thyroid-heart disease has been little explored in thalassemia major (TM). This retrospective cohort study aimed to systematically evaluate in a large historical cohort of TM in the cardiovascular magnetic resonance (CMR) era whether hypothyroidism was associated with a higher risk of heart complications (heart failure, arrhythmias and pulmonary hypertension). MethodsFrom a cohort of 957 TM patients who underwent CMR for myocardial iron overload (MIO) assessment, quantification of biventricular function and detection of myocardial fibrosis within the MIOT network (Myocardial Iron Overload in Thalassemia), we identified 115 (12%) hypothyroid patients. Each hypothyroid patient was matched by sex and age (at the time of the CMR) with two non-hypothyroid patients, creating 115 triples. A cardiac event was considered valid if diagnosed at an age older than the hypothyroidism's onset age for the hypothyroid patient in the belonging triple. ResultsHypothyroid and non-hypothyroid patients had comparable MIO, but hypothyroid patients showed significantly lower biventricular stroke volume index, ejection fraction and left ventricular cardiac index. Accordingly, the prevalence of overall heart dysfunction (LV, RV or both) was higher in hypothyroid patients (43.5% vs 33.5%, P=0.0314). Hypothyroid patients had a significant higher frequency of heart failure (19.1% vs 9.1%, P=0.003) and arrhythmias (11.3% vs 4.3%; P=0.003).Figure1 shows odds ratios (OR) estimating the relationship between hypothyroidism and cardiac involvement. Hypothyroid patients had a significant higher risk of heart dysfunction, heart failure and arrhythmias, also adjusting for the endocrine co-morbidity. [Display omitted] ConclusionsHypothyroidism seems to increase the risk for heart failure, arrhythmias and heart dysfunction in TM patients. Our data confirm the link thyroid-heart disease also in TM patients and they stress the need to prevent hypothyroidism in this population. Disclosures:No relevant conflicts of interest to declare.

Myocardial and Hepatic Iron Overload and Cardiac Function In Sickle/Thalassemia Patients Of Italian Origin

IntroductionSickle-thalassemia results from the combined heterozygosity for sickle-cell and β-thalassemia genes. This study evaluates myocardial and hepatic iron overload and cardiac function in Italian patients and explores their correlation with transfusions, age and sex. MethodsFifty-nine sickle-thalassemia patients (29 males, mean age 35.6±14.1 years), enrolled in the MIOT network underwent magnetic resonance imaging (MRI). T2* value for all 16 myocardial segments and global heart T2* value were calculated. Hepatic T2* value was converted into liver iron concentration (LIC). Cine images were acquired to quantify biventricular volumes and ejection fraction (EF). Results55 (93%) patients had all segmental T2* values normal (>20 ms). Of the 4 patients with abnormal segmental T2* values, all showed an heterogeneous myocardial iron overload (some segments with T2*>20 ms and other with T2*<20 ms) and only one had a global T2*<20 ms. The mean global heart T2* value was 34.4±6.2 ms. The mean LIC was 5.9±6.5 mg/g/dw and 30 patients (50.8%) had a pathological value (≥ 3 mg/g dw). There was a statistically significant positive correlation between global heart T2* and age but with poor linearity (R=0.368; P=0.004) and there was not a significant correlation between age and LIC.Males and females had comparable global heart T2* values and LIC values.Twenty patients were regularly transfused, 32 received sporadic transfusions while 7 were not transfused. The comparison among the three groups is shown in Table 1. We did not find significant differences in the global heart T2* value while patients regularly transfused had significantly higher LIC than sporadically transfused patients. Biventricular volumes indexed by body surface area and ejection fractions were comparable among the groups.Table 1TransfusionsPNo (N=7)Sporadic (N=32)Regular (N=20)Global heart T2* (ms)33.4±7.332.4±6.335.5±5.40.425Pts with global heart T2*<20 ms, N (%)0 (0)0 (0)1 (5.0)0.458MRI LIC (mg/g dw)3.6±3.33.6±2.910.5±8.90.001Pts with MRI LIC ≥ 3 mg/g/dw , N (%)3 (42.9)11 (34.4)16 (80.0)0.004Left ventricular end-diastolic volume index (ml/m2)93.3±40.693.8±23.890.4±15.90.910Left ventricular end-systolic volume index (ml/m2)40.2±30.939.8±17.233.5±7.70.365Left ventricular mass index (g/m2)66.5±12.561.7±14.861.2±22.60.709Left ventricular ejection fraction (%)66.6±7.159.9±7.962.3±7.70.168Right ventricular end-diastolic volume index (ml/m2)81.9±45.085.9±23.783.5±13.90.916Right ventricular end-systolic volume index (ml/m2)36.2±13.134.9±14.729.4±7.70.249Right ventricular ejection fraction (%)47.8±19.852.1±16.553.8±12.10.342 ConclusionsIn respect of MIO, the sickle/thalassemia patients are similar to patients with homozygous SCD for which iron overloading is relatively rare. Hepatic iron overload may develop also in no regularly-transfused patients, maybe due to increased absorption of iron from the digestive tract, characteristic of both SCD and thalassemia intermedia patients. This finding underlines the importance to monitor by MRI also no regularly transfused sickle/thalassemia patients. Disclosures:No relevant conflicts of interest to declare.

Left and Right Ventricular Wall Motion Abnormalities In Thalassemia Major Patients

IntroductionLeft ventricular (LV) and right ventricular (RV) wall motion abnormalities can be detected through a qualitative analysis of cine magnetic resonance (MR) images. Moreover, MR is the gold standard technique for the evaluation of myocardial iron overload (MIO), biventricular global systolic function and myocardial fibrosis. We investigated the relationships between LV movement abnormalities and MIO, LV function and myocardial fibrosis as well as between RV motion and function in thalassemia major (TM) patients. MethodsCMR was performed in 1092 TM patients (537 male; 30.6±8.5 years) enrolled in the Myocardial Iron Overload in Thalassemia Network. Cine images were acquired to evaluate wall motion and to quantify biventricular volumes and ejection fraction (EF). For MIO assessment, a T2* multislice approach was used. To detect myocardial fibrosis, late gadolinium enhanced (LGE) images were acquired. For the LV the 16-segment model of the AHA/ACC was taken into account during image analysis: wall motion, T2* value and presence/absence of enhancing area were evaluated for each segment. ResultsAbnormal motion of LV was found in 66 (6%) patients (60 hypokinetic and 6 dyskinetic). Table 1 shows the comparison between TM patients with normal and abnormal LV motion. Patients with abnormal LV motion were older and had significantly lower global T2* value and significantly higher number of segments with T2*<20 ms. Left volumes and mass indexed by body surface area were significantly higher in patients with abnormal LV motion while the EF was significantly lower. LGE areas were detected in 196 patients (18%) and were predominantly located in the mid-ventricular septum. There was a significant correlation between LGE and abnormal LV motion.Table 1Abnormal LV motionNormal LV motionPAge33.1 ± 8.330.4 ± 8.50.014Sex (M/F)37/29500/5260.248Global heart T2* (ms)22.5 ± 14.730.4 ± 8.50.001N. of segments with T2* < 20 ms8.6 ± 7.54.6 ± 6.1<0.0001Global heart T2* <20 ms, N (%)38 (57.6)376 (36.6)0.001LV end-diastolic volume index (ml/m2)99.0 ± 24.587.5 ± 18.9<0.0001LV end-systolic volume index (ml/m2)49.3 ± 18.734.0 ± 11.6<0.0001LV mass index (g/m2)66.3 ± 14.158.4 ± 13.3<0.0001LV ejection fraction (%)51.1 ± 9.161.7 ± 6.6<0.0001LGE, N (%)34 (51.5)162 (15.8)<0.0001Abnormal motion of the RV was found in 35 (3.2%) patients (29 hypokinetic, 5 dyskinetic and 1 akynetic). Table 2 shows the comparison between TM patients with normal and abnormal RV motion. Patients with abnormal RV motion were older and they were more frequently males. Right volumes were significantly higher in patients with abnormal RV motion while the EF was significantly lower.Table 2Abnormal RV motionNormal RV motionPAge33.9 ± 5.930.5 ± 8.60.013Sex (M/F)27/8510/5470.001RV end-diastolic volume index (ml/m2)110.4 ± 48.283.4 ± 19.2<0.0001RV end-systolic volume index (ml/m2)61.5 ± 29.632.5 ± 11.4<0.0001RV ejection fraction (%)44.9 ± 10.161.4 ± 7.7<0.0001Abnormal LV motion was not correlated with abnormal RV motion. Seventeen patients showed movement abnormalities in both ventricles. ConclusionsMovement abnormalities in the left ventricle were not really frequent in TM patients but were associated with age, MIO, LV dilation and dysfunction, and myocardial fibrosis. Movement abnormalities in the right ventricle were less frequent compared to the left ventricle, but were associated with age, sex , RV dilation and dysfunction. Disclosures:No relevant conflicts of interest to declare.

1081Constrictive Pericarditis in the Modern Cardiology Era Quantitative Analysis of Constrictive Physiology using Four-Dimensional MRI

Objectives: Accurate diagnosis of constrictive pericarditis (CP) is a well-recognized clinical challenge. Since magnetic resonance imaging (MRI) provides high resolution assessment of ventricular volumes, we sought to investigate left ventricular (LV) and right ventricular (RV) time-volume curve using four-dimensional MRI (4D MRI) in patients with CP. Methods: Fourteen patients with CP who had undergone surgery for pericardiectomy and 10 normal subjects were included. Three dimensional MRI covering the whole myocardium in the short axis projection was performed. LV and RV volumes were evaluated over the whole cardiac cycle to generate a four-dimensional MRI dataset for assessment of ventricular time volume curve yielding systolic function and filling parameters: end-diastolic and end-systolic volume, ejection fraction, filling time, early and late filling volume, E/A ratio, peak filling rate (PFR), and time to peak filling rate (TPFR). Results: In patients compared with normal subjects, LV and RV end-diastolic and end-systolic volumes were decreased (all P < 0.05), but biventricular ejection fraction was similar. The filling time of both ventricles was significantly shorter (both P < 0.001). In early diastole, LV and RV filling volumes were increased (P = 0.004, P < 0.001; respectively), in late diastole; however, LV and RV filling volumes were reduced (both P < 0.001). Biventricular E/A ratio and PFR were higher (all P < 0.001) and TPFR of the right ventricle was lower (P = 0.02) in patients compared with normal subjects. Conclusion: In patients with CP quantitative 4D MRI analysis provides reduction in end-diastolic and end-systolic volumes and demonstrated a typical hemodynamic response. In early diastole both ventricles have an increased filling, in late diastole, however; the constricted physiology allowed only a slight ventricular filling.

Normal Ventricular Functional Reference Parameters on Magnetic Resonance Imaging in Healthy Children

We aimed to research right and left ventricular functional reference values on magnetic resonance imaging in healthy children. Echocardiographically normal sixty healthy children were performed cardiac MRI between January 2009 and June 2010. Biventricular volumes, left myocardial mass, septal thickness, diameter of the ascending aorta and main pulmonary artery were calculated. It was investigated whether there was a difference or not between ventricular volumes and sexes, and the relationship age and body surface area with functional parameter results. The minimum, maximum, mean values of every parameters were determined in all children, boys, girls, and in the groups of age 8-12 and 13-18. There was a statistically significant difference in the left ventricular end-systolic diameter results between the sexes. There were statistical significant differences in parameter results except biventricular cardiac output and ejection fraction, right ventricular end-diastolic and end-systolic diameter between the age groups when the children were grouped as 8-12 and 13-18 years by ignoring their sexes. When it was compared the mean value of normal functional parameters after normalized to body surface area; the mean values of all parameters were higher in males, but there was a significant difference in only between right ventricular end-diastolic volumes. Biventricular cardiac output mean values were higher in the age group of 8-12 years.Consequently, we determined the mean, minimum, and maximum values of normal ventricular volumes, left myocardial mass, septal thickness, diameter of the ascending aorta and main pulmonary artery in the 8-18 age group to be used as a reference in all diagnostic and follow-up stage.

Reference ranges for biventricular volumes and ejection fraction and for left ventricular mass in adult thalassemia intermedia patients without myocardial iron overload

Background Thalassemia intermedia (TI) patients were shown to have significantly higher cardiac output and cardiac volumes with respect to thalassemia major (TM) patients. So, to compare biventricular parameters in TI patients with established ranges from TM may be misleading. The aim of this study was to establish the ranges for normal biventricular volumes and ejection fraction (EF) and for left ventricular (LV) mass assessed by cardiovascular magnetic resonance (CMR) in TI.

Feasibility of Real-Time Three-Dimensional Echocardiography for the Assessment of Distorted Biventricular Systolic Function in Patients with Cor Pulmonale

BackgroundThis study was to investigate the feasibility of real-time 3-dimensional echocardiography (RT3DE) for the analysis of biventricular ejection fractions and volume measurements in patients with cor pulmonale and the correlations of RT3DE results with 64-slice multi-detector cardiac computed tomography (64-MDCT) results.MethodsThis study included a total of 22 patients (59.3 ± 16.6 years of age; 10 males and 12 females) who showed flattening or reverse curvature of the interventricular septum and severe pulmonary hypertension [mean right ventricular (RV) systolic pressure = 66.8 ± 19.7 mmHg] on 2-dimensional transthoracic echocardiography due to cor pulmonale. Biventricular end-diastolic and end-systolic volumes were measured by RT3DE and 64-MDCT. The severity of D-shaped deformation was evaluated by using left ventricular (LV) eccentricity index (ratio of diameters parallel/perpendicular to the interventricular septum on parasternal short axis images of the papillary muscle level).ResultsThere were moderate correlations between biventricular volumes measured by RT3DE and 64-MDCT except for LV end-systolic volume (59.8 ± 17.1 vs. 73.2 ± 20.2 mL, r = 0.652, p = 0.001 for LV end-diastolic volume; 30.6 ± 9.1 vs. 30.8 ± 12.5 mL, r = 0.361, p = 0.099 for LV end-systolic volume; 110.1 ± 42.9 vs. 171.1 ± 55.3 mL, r = 0.545, p = 0.009 for RV end-diastolic volume; and 80.9 ± 35.0 vs. 128.7 ± 45.1 mL, r = 0.549, p = 0.005 for RV end-systolic volume respectively).ConclusionThis study suggests that RT3DE may be a modest method for measuring distorted biventricular end-systolic and end-diastolic volumes in patients with cor pulmonale.

Evaluation of Cardiac Function by Magnetic Resonance Imaging During the Follow-Up of Patients With Kawasaki Disease

Although histopathologic studies suggest persistent myocardial abnormalities after Kawasaki disease (KD), the long-term effects on cardiac function remain to be revealed. We investigated biventricular volumes, function, and the presence of myocardial fibrosis by cardiac magnetic resonance imaging during long-term follow-up of KD. Sixty patients with a history of KD (mean age, 16.9 years; 67% men; median interval after KD onset, 11.6 years) and 20 healthy control subjects (mean age, 17.9 years; 55% men) 12 to 24 years of age underwent cardiac magnetic resonance imaging. Biventricular end-diastolic volume, end-systolic volume, stroke volume, and ejection fraction were determined. Volumetric measurements were indexed for body surface area. Late contrast enhancement was used to detect areas of myocardial fibrosis. Biventricular volumes and function did not differ significantly between patients and control subjects. There were also no significant differences between patients with and without a history of left ventricular dysfunction resulting from KD-associated myocarditis or between patients with and without coronary artery aneurysms. Only those with prior ischemic heart disease had a significantly lower left ventricular ejection fraction compared with unaffected KD cases (left ventricular ejection fraction, 51% versus 57%; P=0.012). Late contrast enhancement was observed in only 2 patients with severe coronary artery aneurysms and was typical for myocardial infarction. In this cardiac magnetic resonance imaging study evaluating the cardiac function of patients with KD at long-term follow-up, we did not observe a difference in cardiac function between KD patients and control subjects, except for a subgroup of patients with ischemic heart disease as a result of severe coronary artery pathology.

Ethnic differences in ventricular hypertrabeculation on cardiac MRI in elite football players

Left ventricular (LV) trabeculation may be more pronounced in ethnic African than in Caucasian (European) athletes, leading to possible incorrect diagnosis of left ventricular non-compaction cardiomyopathy (LVNC). This study investigates ethnic differences in LV hypertrabeculation amongst elite athletes with cardiac magnetic resonance (CMR) and electrocardiography (ECG). 38 elite male football (soccer) players (mean age 23.0, range 19-34years, 28/38 European, 10/38 African) underwent CMR and ECG. Hypertrabeculation was assessed using the ratio of non-compacted to compacted myocardium (NC/C ratio) on long-axis and short-axis segments. ECGs were systematically rated. No significant differences were seen in ventricular volumes, wall mass or E/A ratio, whereas biventricular ejection fraction (EF) was significantly lower in African athletes (European/African athletes LVEF 55/50%, p = 0.02; RVEF 51/48%, p = 0.05). Average NC/C ratio was greater in African athletes but only significantly at mid-ventricular level (European/African athletes: apical 0.91/1.00, p = 0.65; mid-ventricular 0.89/1.45, p < 0.05; basal 0.40/0.46, p = 0.67). ECG readings demonstrated no significant group differences, and no correlation between ECG anomalies and hypertrabeculation. A greater degree of LV hypertrabeculation is seen in healthy African athletes, combined with biventricular EF reduction at rest. Recognition of this phenomenon is necessary to avoid misdiagnosis of LVNC.

QRS pattern and improvement in right and left ventricular function after cardiac resynchronization therapy: a radionuclide study

BackgroundPredicting response to cardiac resynchronization therapy (CRT) remains a challenge. We evaluated the role of baseline QRS pattern to predict response in terms of improvement in biventricular ejection fraction (EF).MethodsConsecutive patients (pts) undergoing CRT implantation underwent radionuclide angiography at baseline and at mid-term follow-up. The relationship between baseline QRS pattern and mechanical dyssynchrony using phase analysis was evaluated. Changes in left and right ventricular EF (LVEF and RVEF) were analyzed with regard to baseline QRS pattern.ResultsWe enrolled 56 pts, 32 with left bundle branch block (LBBB), 4 with right bundle branch block (RBBB) and 20 with non-specific intraventricular conduction disturbance (IVCD). A total of 48 pts completed follow-up. LBBB pts had significantly greater improvement in LVEF compared to RBBB or non-specific IVCD pts (+9.6 ± 10.9% vs. +2.6 ± 7.6%, p = 0.003). Response (defined as ≥ 5% increase in LVEF) was observed in 68% of LBBB vs. 24% of non-specific IVCD pts (p = 0.006). None of the RBBB pts were responders. RVEF was significantly improved in LBBB (+5.0 ± 9.0%, p = 0.007), but not in non-specific IVCD and RBBB pts (+0.4 ± 5.8%, p = 0.76). At multivariate analysis, LBBB was the only predictor of LVEF response (OR, 7.45; 95% CI 1.80-30.94; p = 0.006), but not QRS duration or extent of mechanical dyssynchrony.ConclusionsPresence of a LBBB is a marker of a positive response to CRT in terms of biventricular improvement. Pts with non-LBBB pattern show significantly less benefit from CRT than those with LBBB.

Bi-atrial function and its relation with biventricular function and clinical parameters in patients operated for tetralogy of Fallot

Background Biventricular size and function have been studied extensively in patients after tetralogy of Fallot (TOF) repair, but little is known about atrial size and function. The atria play a crucial role in ventricular filling during diastole, and abnormalities in atrial size and function may reflect ventricular diastolic dysfunction. Diastolic dysfunction may precede systolic dysfunction and may therefore play an important role in the early detection of ventricular dysfunction. Aim We assessed bi-atrial size and function in patients after TOF repair, and evaluated relationships with biventricular systolic and diastolic function, and clinical parameters. Methods 51 patients (21±8 years) and 30 healthy controls (31±7 years) were included and underwent magnetic resonance imaging. Patients also underwent exercise testing, and biomarker assessment. Bi-atrial and biventricular size, systolic and diastolic function were assessed from timevolume curves (figure 1) and time-volume-change curves. Results In patients, right atrial (RA) minimal volume (min.vol.), RA pump function, and RA late emptying fraction were increased (RA min.vol.: 34±8 ml/m 2 (patients) vs. 28±8 ml/m 2 (controls), p=0.001); RA reservoir function, RA early emptying fraction, and the RA early-to-late-emptying ratio (E/A ratio) were decreased (RA E/A ratio: 1.1 ±0.5 (patients) vs. 1.9±0.9 (controls), p<0.001). Patients had larger right ventricular (RV) volumes, lower biventricular ejection fraction and prolonged biventricular deceleration time (Dt) (RV Dt: 0.24±0.10 (patients) vs. 0.13±0.04 (controls), p<0.001). Left atrial (LA) maximal volume and LA early emptying fraction were decreased. Left ventricular (LV) E/A-filling ratio was increased (LV E/A-ratio: 4.2±3.7 (patients) vs. 2.7±1.3 (controls), p=0.008). Patients with end-diastolic forward flow (EDFF) had larger RA and RV size, higher RA pump function (RA

Impact of Gender and Age on Cardiovascular Function Late After Repair of Tetralogy of Fallot

Background— The impact of gender and age on cardiac function by cardiac magnetic resonance (CMR) in repaired tetralogy of Fallot (TOF) is unknown, which limits the value of currently discussed volumetric thresholds and the accuracy of individual follow-up. Methods and Results— In a nationwide, prospective, 14-center study, 407 consecutive patients with repaired TOF (age, 17.9±8.3 years; range, 8–59 years; 226 male patients) underwent standardized CMR ventricular volumetry and flow quantification (pulmonary artery/ascending aorta). There were no sex differences for age at TOF repair, type of repair, number of prior repair palliations or reinterventions after repair, pulmonary regurgitation fraction, and maximal gradient across the right ventricular outflow tract. Biventricular volumes and mass (indexed to body surface area), available in 380 of 407 patients, respectively, were higher in male patients ( P &lt;0.003), but biventricular ejection fraction was higher in female patients ( P &lt;0.012). As opposed to reported data of healthy populations, sex-specific reference percentiles computed for an age range of 8 to 40 years (lambda-mu-sigma method) demonstrated (1) an increase of end-diastolic and end-systolic left ventricular volumes, particularly in female patients; (2) an increase of end-systolic right ventricular volumes in both sexes; and (3) a decrease of biventricular ejection fraction in male patients, whereas in female patients, only right ventricular ejection fraction decreased. Conclusions— Significant gender differences of biventricular volumes, function, and mass by CMR exist late after repair of TOF, suggesting that age and gender cannot be ignored when discussing thresholds. Gender-specific percentiles may present a more relevant framework of reference for an individual patient at a given age and suggest a gradual decline of biventricular systolic function over time. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT00266188.