Prognostic significance of echocardiographic deformation imaging in adult congenital heart disease.

BACKGROUND: Due to medical and surgical advancements, the population of adult patients with congenital heart disease (ACHD) is growing. Despite successful therapy, ACHD patients face structural sequalae, placing them at increased risk for heart failure and arrhythmias. Left and right ventricular function are important predictors for adverse clinical outcomes. In acquired heart disease it has been shown that echocardiographic deformation imaging is of superior prognostic value as compared to conventional parameters as ejection fraction. However, in adult congenital heart disease, the clinical significance of deformation imaging has not been systematically assessed and remains unclear. METHODS: According to the Preferred Reporting Items for Systematic Reviews checklist, this systematic review included studies that reported on the prognostic value of echocardiographic left and/or right ventricular strain by 2-dimensional speckle tracking for hard clinical end-points (death, heart failure hospitalization, arrhythmias) in the most frequent forms of adult congenital heart disease. RESULTS: In total, 19 contemporary studies were included. Current data shows that left ventricular and right ventricular global longitudinal strain (GLS) predict heart failure, transplantation, ventricular arrhythmias and mortality in patients with Ebstein's disease and tetralogy of Fallot, and that GLS of the systemic right ventricle predicts heart failure and mortality in patients post atrial switch operation or with a congenitally corrected transposition of the great arteries. CONCLUSIONS: Deformation imaging can potentially impact the clinical decision making in ACHD patients. Further studies are needed to establish disease-specific reference strain values and ranges of impaired strain that would indicate the need for medical or structural intervention.

Surgical Repair of a Sinus of Valsalva Aneurysm.

A surgically challenging case of an unruptured Sinus of Valsalva aneurysm (SoVA) with severe aortic regurgitation (AR) due to cusp prolapse is presented. Sinus reconstruction with a patch cut out from the sinus portion of a Gelweave Valsalva graft (Terumo Vascutek) was performed. Intraoperative measurements showed insufficient effective height of the right coronary cusp; therefore, cusp plication and pericardial patch augmentation of the right coronary cusp were performed with satisfying result.

Infectious stentitis after treatment of coarctation of the aorta: a case report.

BACKGROUND: Aortitis is a rare condition that can be caused by inflammatory or infectious aetiologies. The clinical presentation of aortitis includes a heterogeneous range of symptoms and clinical signs. CASE SUMMARY: We present a 53-year-old man whose medical history included presence of a ventricular septal defect, a bicuspid aortic valve, and coarctation of the aorta. The coarctation was treated with percutaneous stent implantation. One and a half years later, he presented to our hospital with complaints of fatigue, night sweats, and shivers. Physical examination revealed a fever, tachycardia, and hypertension. Imaging studies showed no signs of endocarditis. Positron emission tomography-computed tomography (PET-CT) showed an increase in 18F-fluorodeoxyglucose uptake at the distal end of the stent in the descending aorta. Blood cultures revealed a Streptococcus gordonii and antibiotic treatment was adjusted accordingly. The patients' functional status improved quickly, the fever resolved, and the laboratory markers of inflammation returned to normal. DISCUSSION: Aortitis is extremely rare after stent implantation. Risk factors for aortitis include congenital vascular malformation and stent implantation. Computed tomography is currently the imaging study of choice for aortitis, while PET-CT seems ideal for identification of stent infection. Mortality associated with infectious aortitis ranges from 21% to 44%, with generally higher mortality if managed with antibiotics alone. The differential diagnosis of stent infection should be taken into account in patients presenting with fever and chills after previous stent procedures.

Reparación de la estenosis aislada de la válvula pulmonar: ¿es curativa? / Repaired isolated pulmonary valve stenosis: living happily ever after?

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Quantitative assessment of the entire right ventricle from one acoustic window: an attractive approach.

AIMS: The aim of this prospective study was to evaluate the feasibility and establish normal values of functional right ventricle (RV) parameters as assessed in our four, long-axis view RV model using iRotate echocardiography. Furthermore, we evaluated the potential use of this model in patients with abnormally loaded RVs. METHOD AND RESULTS: One hundred and fifty-five healthy subjects aged 20-72 years (≥28 subjects per decile) were prospectively recruited. We used non-dedicated RV speckle-tracking software to test the feasibility and to establish normal range values of peak systolic global longitudinal RV strain (RV-GLS) from the RV free-walls (septum was excluded). Also normal range values for: dimensions, tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular peak systolic velocity (TDI-S') were established. The feasibility of RV-GLS was 88, 84, and 62%, respectively, in the lateral, inferior, and anterior free wall. Mean RV-GLS normal values were -24.5 ± 4.9% for lateral wall and -25.4 ± 5.0% for anterior wall. Mean RV-GLS in the inferior wall was -23.2 ± 4.4% in the aortic (Ao) view and -20.7 ± 5.0% in the coronal (CV) view. The feasibility of mean RV-GLS was 100% in the anterior, lateral, and inferior walls in abnormally dilated RVs. CONCLUSION: The feasibility of all RV parameters assessed in the four-view iRotate model is good to excellent. Normal values for RV dimension and function of the anterior, inferior, and lateral RV walls have been established. Further studies and dedicated RV speckle-tracking software are warranted to discover the full potential of this new technique.

A Novel 13-Segment Standardized Model for Assessment of Right Ventricular Function Using Two-Dimensional iRotate Echocardiography.

AIMS: The aim of this study was to evaluate the feasibility of transthoracic two-dimensional (2D) iRotate, a new echo modality, to assess the whole right ventricle (RV) from a single transducer position based on anatomic landmarks. METHODS AND RESULTS: The anatomic landmarks were first defined based on three-dimensional echocardiographic datasets using multiplane reconstruction analyses. Thereafter, we included 120 healthy subjects (51% male, age range 21-67 years). Using 2D iRotate, four views of the RV could be acquired based on these landmarks. The anterior, lateral, inferior wall (divided into three segments: basal-mid-apical), and right ventricular outflow tract (RVOT) anterior wall of the RV were determined. The feasibility of visualization of RV segments and tricuspid annular plane systolic excursion (TAPSE) and tissue Doppler imaging (TDI) measurements were assessed. To evaluate this model for diseased RVs, a small pilot study of 20 patients was performed. In 98% of healthy subjects and 100% of patients, iRotate mode was feasible to assess the RV from one single transducer position. In total, 86% and 95%, respectively, of the RV segments could be visualized. The visualization of the RVOT anterior wall was worse 23% and 75%, respectively. TAPSE and TDI measurements on all four views were feasible 93% and 92%, respectively, of the healthy subjects and in 100% of the patients. CONCLUSION: With 2D iRotate, a comprehensive evaluation of the entire normal and diseased RV is feasible from a fixed transducer position based on anatomic landmarks. This is less time-consuming than the multiview approach and enhances accuracy of RV evaluation. Imaging of the RVOT segment remains challenging.

Multiframe registration of real-time three-dimensional echocardiography time series.

Mosaicing of real-time three-dimensional echocardiography (RT3-DE) images aims at extending the field-of-view of overlapping images. Currently available methods discard most of the temporal information available in the time series. We investigate the added value of simultaneous registration of multiple temporal frames using common similarity metrics. We combine RT3-DE images of the left and right ventricles by registration and fusion. The standard approach of registering single frames, either end-diastolic (ED) or end-systolic (ES), is compared with simultaneous registration of multiple time frames, to evaluate the effect of using the information from all images in the metric. A transformation estimating the protocol-specific misalignment is used to initialize the registration. It is shown that multiframe registration can be as accurate as alignment of the images based on manual annotations. Multiframe registration using normalized cross-correlation outperforms any of the single-frame methods. As opposed to expectations, extending the multiframe registration beyond simultaneous use of ED and ES frames does not further improve registration results.

Long-term outcome and quality of life after arterial switch operation: a prospective study with a historical comparison.

AIM.: The study aims to describe the long-term cardiological and psychological results of our first surgical cohort of arterial switch operation (ASO) patients and compare the results with our earlier series of Mustard patients. METHODS.: Twenty-four survivors of ASO operated in our center (1985-1990) were evaluated by electrocardiography, echocardiography, magnetic resonance imaging, exercise testing, 24-hour Holter-monitoring, and health-related quality of life questionnaire. The results were compared with 58 adult Mustard patients who were evaluated in 2001 using the same study protocol. RESULTS.: Arterial switch operation was performed at a median age of 13 days and Mustard operation at 2 years. Median follow-up was 22 years (range 20-25) and 25 years (22-29), respectively. After ASO, survival was better (P =.04). The event-free survival after 22 years was 77% after ASO vs. 44% after Mustard (P =.03). Good systemic ventricular function was present in 93% after ASO vs. 6% after Mustard (P <.01). Exercise capacity in ASO was 85% of predicted, compared with 72% in Mustard patients (P =.01). Aortic regurgitation was found in 21% of ASO patients vs. 16% in Mustard patients. Arterial switch patients vs. Mustard patients reported significantly better quality of life and less somatic complaints. CONCLUSION.: The progression made in surgical treatment for transposition of the great arteries from Mustard to ASO has had a positive impact on survival, cardiac function, exercise capacity, and also self-reported quality of life and somatic complaints. Longer follow-up is warranted to monitor aortic regurgitation.

Regional left ventricular rotation and back-rotation in patients with reverse septal curvature hypertrophic cardiomyopathy.

AIMS: This study sought to investigate regional left ventricular (LV) rotation in patients with hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: The study comprised 44 patients with HCM with a typical reverse septal curvature (age 40 ± 14 years, 33 men) and 44 healthy volunteers (age 39 ± 14 years, 32 men) in whom LV rotation could be assessed at the basal and apical LV level with speckle-tracking echocardiography, using the QLAB Advanced Quantification Software version 6.0 (Philips, Best, The Netherlands). In HCM patients, lower values of initial counter-clockwise rotation at the basal LV level (1.5 ± 1.2 vs. 0.6 ± 0.9°, P < 0.001) were seen, in particular in the septal segment (1.7 ± 1.6 vs. 0.4 ± 0.7°, P < 0.001). After this period, the direction of rotation changed to clockwise with a peak basal rotation of -4.8 ± 2.0° in controls vs. -6.1 ± 2.5° in HCM patients (P < 0.05). Peak basal rotation in HCM patients was in particular higher in the anterior (-6.6 ± 3.0 vs. -4.4 ± 2.4°, P < 0.01) and septal (-5.4 ± 2.6 vs. -3.9 ± 1.9°, P < 0.05) segments. The normalized (corrected for peak basal rotation) global back-rotation rate was lower in HCM patients (4.1 ± 3.1 vs. 6.3 ± 4.9 s(-1), P < 0.05), in particular driven by a lower rate in the septal segment (3.8 ± 2.6 vs. 6.4 ± 4.8 s(-1), P < 0.01). At the apical level, changes in rotation and back-rotation were more homogeneous. CONCLUSION: Changes in rotation and back-rotation at the LV basal level in HCM patients are mainly caused by regional changes in the basal septal and anterior segments, the segments mostly involved in the hypertrophic process.

Right ventricular quantification in clinical practice: two-dimensional vs. three-dimensional echocardiography compared with cardiac magnetic resonance imaging.

AIMS: To establish the additional value of three-dimensional echocardiography (3D echo) for assessment of right ventricular (RV) size and function in patients with congenital heart disease (CHD) in everyday clinical practice, the accuracy and reproducibility of 3D echo were compared with conventional two-dimensional echocardiography (2D echo) and cardiac magnetic resonance (CMR) imaging as reference. METHODS AND RESULTS: Patients with CHD and primarily affected right ventricles (n = 62), patients with CHD and primarily affected left ventricles (LV group, n = 27), and healthy controls (n = 31) were studied. 2D echo-, 3D echo- and CMR data sets were obtained. Moderate correlations were found between RV dimensions by 2D echo and CMR-derived RV end-diastolic volumes (r = 0.32-0.77). The correlations between RV volumes obtained by 3D echo and CMR imaging were better (r = 0.71-0.97) than the 2D echo-derived correlations (P < 0.001). Only the 2D echo-derived RV inlet diameter correlated better in healthy controls than in the RV group. Receiver operating characteristic curve analysis revealed that 3D echo-derived end-diastolic volume best identified RV dysfunction (sensitivity 95% and specificity 100%). The 3D echo-derived measurements were as reproducible as the 2D echo-derived measurements (n = 37, coefficients of variation ranging from 5 to 19%), with tricuspid annular plane systolic excursion being the most reproducible measurement (coefficient of variation of 6%). CONCLUSION: 3D echo improved quantitative RV size and function assessment compared with 2D echo in patients as well as in healthy controls. Everyday clinical use of 3D echo for RV assessment can be reality with the currently available software and provides incremental benefit in assessment of the right ventricle.

Test-retest variability of volumetric right ventricular measurements using real-time three-dimensional echocardiography.

BACKGROUND: Substantial variability in sequential echocardiographic right ventricular (RV) quantification may exist. Interobserver and intraobserver values are well known, but acquisition (test-retest) variability has been rarely assessed. The objective of this study was to determine the test-retest variability of sequential RV volume and ejection fraction (EF) measurements by real-time three-dimensional echocardiography in patients with congenital heart disease and healthy controls. METHODS: Twenty-eight participants (21 patients with congenital heart disease, seven healthy controls; mean age, 30 ± 14 years; 43% men) underwent a series of three echocardiographic studies. To obtain interobserver and intraobserver test-retest variability, two sonographers acquired sequential RV data sets in each participant during one outpatient visit. RV volumetric quantification was done using semiautomated three-dimensional border detection. The variability data were analyzed using correlation coefficients, Bland-Altman analysis, and coefficients of variation. RESULTS: Absolute mean differences for sequential intraobserver acquisitions were 12 ± 12 mL for end-diastolic volume, 7 ± 6 mL for end-systolic volume, and 4 ± 3% for EF. Interobserver and intraobserver test-retest variability, respectively, were 7% and 7% for RV end-diastolic volume, 14% and 7% for end-systolic volume, and 8% and 6% for EF. CONCLUSIONS: Good test-retest variability, besides the practical nature of real-time three-dimensional echocardiography for RV volume and EF assessment, makes it a valuable technique for serial follow-up. Although it may be challenging to diminish all factors that can influence echocardiographic examination for serial follow-up, standardization of RV size and functional measurements should be a goal to produce more interchangeable data.

Diagnostic value of rigid body rotation in noncompaction cardiomyopathy.

BACKGROUND: The diagnosis of noncompaction cardiomyopathy (NCCM) remains subject to controversy. Because NCCM is probably caused by an intrauterine arrest of the myocardial fiber compaction during embryogenesis, it may be anticipated that the myocardial fiber helices, normally causing left ventricular (LV) twist, will also not develop properly. The resultant LV rigid body rotation (RBR) may strengthen the diagnosis of NCCM. The purpose of the current study was to explore the diagnostic value of RBR in a large group of patients with prominent trabeculations. METHODS: The study comprised 15 patients with dilated cardiomyopathy, 52 healthy subjects, and 52 patients with prominent trabeculations, of whom a clinical expert in NCCM defined 34 as having NCCM. LV rotation patterns were determined by speckle-tracking echocardiography and defined as follows: pattern 1A, completely normal rotation (initial counterclockwise basal and clockwise apical rotation, followed by end-systolic clockwise basal and counterclockwise apical rotation); pattern 1B, partly normal rotation (normal end-systolic rotation but absence of initial rotation in the other direction); and pattern 2, RBR (rotation at the basal and apical level predominantly in the same direction). RESULTS: The majority of normal subjects had LV rotation pattern 1A (98%), whereas the 18 subjects with hypertrabeculation not fulfilling diagnostic criteria for NCCM predominantly had pattern 1B (71%), and the 34 patients with NCCM predominantly had pattern 2 (88%). None of the patients with dilated cardiomyopathy showed RBR. Sensitivity and specificity of RBR for differentiating NCCM from "hypertrabeculation" were 88% and 78%, respectively. CONCLUSIONS: RBR is an objective, quantitative, and reproducible functional criterion with good predictive value for the diagnosis of NCCM as determined by expert opinion.

Usefulness of real-time three-dimensional echocardiography to identify right ventricular dysfunction in patients with congenital heart disease.

Because right ventricular (RV) dysfunction predicts a poor outcome in patients with congenital heart disease (CHD), regular monitoring of RV function is indicated. To date, cardiac magnetic resonance (CMR) imaging has been the reference method. A more practical, more accessible, and accurate tool would be preferred. We defined normality regarding RV systolic function using healthy controls and tested the ability of real-time 3-dimensional echocardiographic (RT3DE) findings to identify patients with CHD with RV dysfunction. The cutoff values for the RV volumes and ejection fraction (EF) were derived from the CMR imaging findings from 41 healthy controls (mean age 27 +/- 8 years, 56% men). In 100 patients with varying CHDs (mean age 27 +/- 11 years, 65% men), both RT3DE data sets (iE33) and short-axis CMR imaging (1.5 T) were obtained within 2 hours. The RT3DE and CMR RV volumes and EF were calculated using commercially available software. Receiver operating characteristic curves were created to obtain the sensitivity and specificity of the RT3DE data to identify RV dysfunction. Applying the cutoff values derived from the healthy controls using the CMR data of patients with CHD, we identified 23 patients with an enlarged indexed end-diastolic volume, 29 patients with an enlarged indexed end-systolic volume, and 21 patients with an impaired RVEF. The best cutoff values predicting RV dysfunction using the RT3DE findings were identified (indexed end-diastolic volume >105 ml/m(2), indexed end-systolic volume >54 ml/m(2), and EF <43%). The RT3DE findings revealed 23 patients with impaired RVEF, with 95% sensitivity, 89% specificity, and a negative predictive value of 99%. In conclusion, real-time 3-dimensional echocardiography is a very sensitive tool to identify RV dysfunction in patients with CHD and could be applied clinically to rule out RV dysfunction or to indicate additional quantitative analysis of RV function.

Clinical value of real-time three-dimensional echocardiography for right ventricular quantification in congenital heart disease: validation with cardiac magnetic resonance imaging.

BACKGROUND: The objective of this study was to test the feasibility, accuracy, and reproducibility of the assessment of right ventricular (RV) volumes and ejection fraction (EF) using real-time three-dimensional echocardiographic (RT3DE) imaging in patients with congenital heart disease (CHD), using cardiac magnetic resonance (CMR) as a reference. METHODS: RT3DE data sets and short-axis cine CMR images were obtained in 62 consecutive patients (mean age, 26.9 +/- 10.4 years; 65% men) with various CHDs. RV volumetric quantification was done using semiautomated 3-dimensional border detection for RT3DE images and manual tracing of contours in multiple slices for CMR images. RESULTS: Adequate RV RT3DE data sets could be analyzed in 50 of 62 patients (81%). The time needed for RV acquisition and analysis was less for RT3DE imaging than for CMR (P < .001). Compared with CMR, RT3DE imaging underestimated RV end-diastolic and end-systolic volumes and EF by 34 +/- 65 mL, 11 +/- 55 mL, and 4 +/- 13% (P < .05) with 95% limits of agreement of +/-131 mL, +/-109 mL, and +/-27%, as shown by Bland-Altman analyses, with highly significant correlations (r = 0.93, r = 0.91, and r = 0.74, respectively, P < .001). Interobserver variability was 1 +/- 15%, 6 +/- 17%, and 8 +/- 13% for end-diastolic and end-systolic volumes and EF, respectively. CONCLUSION: In the majority of unselected patients with complex CHD, RT3DE imaging provides a fast and reproducible assessment of RV volumes and EF with fair to good accuracy compared with CMR reference data when using current commercially available hardware and software. Further studies are warranted to confirm our data in similar and other patient populations to establish its use in clinical practice.

Influence of cardiac shape on left ventricular twist.

The dynamic interaction between subendocardial and subepicardial fibre helices in the left ventricle (LV) leads to a twisting deformation, which has an important role in LV function. This study sought to assess the influence of cardiac shape on LV twist in the normal and dilated human heart. The study comprised 45 dilated cardiomyopathy (DCM) patients and 60 for age- and gender-matched healthy volunteers. Speckle tracking echocardiography was used to determine basal and apical LV peak systolic rotation (Rot(max)) and instantaneous LV peak systolic twist (Twist(max)). LV sphericity index was calculated by dividing the LV maximal long-axis internal dimension by the maximal short-axis internal dimension at end-diastole. A parabolic relation between the sphericity index and apical Rot(max) or Twist(max) was identified in the total study population (R(2) = 0.56 and R(2) = 0.54, respectively; both P < 0.001) and healthy volunteers (R(2) = 0.39 and R(2) = 0.25, respectively; both P < 0.001), whereas these relations were linear in DCM patients (R(2) = 0.40 and R(2) = 0.43, respectively; both P < 0.001). In a multivariate analysis, LV sphericity index was the strongest independent predictor of apical Rot(max) and Twist(max). In conclusion, LV apical rotation and twist are significantly influenced by LV configuration. Taking the important function of LV twist into account, this finding highlights the vital influence of cardiac shape on LV systolic function.

Delayed left ventricular untwisting in hypertrophic cardiomyopathy.

BACKGROUND: Almost all patients with hypertrophic cardiomyopathy (HCM) have some degree of left ventricular (LV) diastolic dysfunction. Nevertheless, the pathophysiology remains incompletely characterized. Conceptually, an ideal therapeutic agent should target the underlying mechanisms that cause LV diastolic dysfunction. Assessment of diastolic LV untwisting could potentially be helpful to gain insight into the mechanism of diastolic dysfunction. The purpose of this study was to investigate LV untwisting in patients with HCM and control subjects. METHODS: LV untwisting parameters were assessed using speckle-tracking echocardiography in 75 consecutive patients with HCM and compared with those from 75 healthy control subjects. RESULTS: Untwisting at 5%, 10%, and 15% of diastole was lower in patients with HCM (all P values < .001) compared with control subjects. Peak diastolic untwisting velocity (-92 +/- 32 degrees/s vs -104 +/- 39 degrees/s, P < .05) and untwisting rate from peak systolic twist to mitral valve opening (MVO) (-37 +/- 20 degrees/s vs -46 +/- 22 degrees/s, P < .01) were lower, while the for diastolic duration normalized time-to-peak diastolic untwisting velocity (17 +/- 9% vs 13 +/- 9%, P < .05) was higher in patients with HCM. Untwisting rate from peak systolic twist to MVO was negatively correlated with the E/A ratio (R(2) = 0.15, P < .01). Peak diastolic untwisting velocity and untwisting rate from peak systolic twist to MVO were increased in mild but decreased in moderate and severe diastolic dysfunction compared with control subjects. CONCLUSION: LV untwisting is delayed in HCM, which probably significantly contributes to diastolic dysfunction.

Feasibility and reproducibility of left ventricular rotation parameters measured by speckle tracking echocardiography.

AIMS: This study sought to find the most robust method for left ventricular (LV) rotation measurement by speckle tracking echocardiography (STE) with the new QLAB Advanced Quantification Software (version 6.0, Philips, Best, The Netherlands). METHODS AND RESULTS: The study population consisted of 40 non-selected patients (mean age 48 +/- 18 year, 20 men) and 50 non-selected healthy volunteers (mean age 34 +/- 12 year, 21 men). Feasibility and intra-observer reproducibility of the measurement of LV rotation parameters by STE were assessed for two different methods (Method A: six tracking points placed mid-myocardial and Method B: six tracking points placed endocardial and epicardial forming six myocardial segments). Subsequently, inter-observer and temporal reproducibility of the most robust method were assessed. Complete LV rotation assessment was more feasible with Method A (60 out of 90 subjects, 67% vs. 50 out of 90 subjects, 56%). In the 49 subjects in whom both Methods A and B were feasible, intra-observer reproducibility of LV rotation parameters was better with Method A (variabilities 2 +/- 3 to 10 +/- 9% vs. 2 +/- 4 to 21 +/- 18%). With this method, inter-observer variability varied from 4 +/- 4 to 13 +/- 9% and temporal variability from 4 +/- 6 to 19 +/- 15%. CONCLUSION: The most robust method to assess LV rotation with QLAB software is from the mid-myocardium. This method is feasible in approximately two-thirds of subjects and has good intra-observer, inter-observer, and temporal reproducibility, allowing to study changes over time in LV rotation in an individual patient.