The geometry and heterogeneity of the right ventricle in hypoplastic left heart syndrome makes objective echocardiographic assessment of systolic function challenging. Consequently, subjective echocardiographic assessment of right ventricular (RV) function is still routinely undertaken. The aims of this study were to compare this with magnetic resonance imaging (MRI), investigate the impact of experience and training on the accuracy of subjective assessment, and critically analyze the role of echocardiography to detect impaired systolic function.
A retrospective analysis of prospectively acquired data was performed. Children with hypoplastic left heart syndrome underwent routine preoperative cardiac MRI and echocardiography under the same general anesthetic. Echocardiograms were reviewed, and members of the congenital heart disease team with differing echocardiography experience subjectively graded RV systolic function (good, moderate, or poor). This was compared with MRI-derived ejection fraction.
Twenty-eight patients at different palliative stages were included. Twenty-eight observers were divided into five experience categories (congenital heart disease junior trainees to attending cardiologists). Median agreement was 47.6% (range, 31.4%–58.2%), with the lowest agreement among junior trainees and the highest among attending cardiologists. When used as a screening test for poor RV systolic function, the median sensitivity of echocardiography was 0.89 (range, 0.86–0.96), and median specificity was 0.45 (range, 0.26–0.55). The highest sensitivity was observed among junior trainees but with the lowest specificity. The highest specificity was observed among attending cardiologists (0.55).
Agreement between echocardiographic and MRI RV ejection fraction improves with experience but remains suboptimal. When used as a screening test for poor RV function, echocardiography is sensitive, but specificity is heavily influenced by operator experience.
Hypoplastic left heart syndrome (HLHS) describes a spectrum of underdevelopment of the left heart that renders it incapable of supporting the systemic arterial circulation. The current management approach includes three staged surgeries carried out over the first few years of the child’s life; the resulting circulation is maintained by a systemic right ventricle. Impaired right ventricular (RV) systolic function is associated with poor outcomes, so the early detection of cardiac dysfunction has important implications for future management and prognosis. Assessment of RV function is an essential part of evaluation in both the acute inpatient setting and outpatient review. The geometry and heterogeneity of the single right ventricle in patients with HLHS make reliable and reproducible echocardiographic assessment of systolic function challenging.
The “gold standard” for the measurement of intrinsic RV contractility is by conductance catheterization, but this is not used in routine clinical practice. In the clinical care of patients with HLHS, cardiac magnetic resonance imaging (MRI) is the imaging method used to provide objective measurement of ventricular volumes, ejection fraction (EF), and flow. MRI usually requires general anesthesia or sedation in younger children and is expensive, is not portable, and requires a level of expertise not universally available. Despite these universal limitations of MRI, it is used routinely at our institution to assess this patient group before hemi-Fontan and Fontan surgery, or if there are concerns regarding anatomic or functional complications.
Although several echocardiographic indices have been investigated for the assessment of RV function, there are still significant limitations, and in routine clinical practice, evaluation of RV function is still based predominantly on subjective assessment. Previous studies have attempted to describe established RV functional assessment parameters in patients with HLHS, but small numbers and multiple confounders (operative stage, left ventricular morphology) have meant that these are not necessarily widely applicable. A previous study of subjective assessment of single ventricular function (including single left, single right, and indeterminate ventricles) showed poor correlation between qualitative assessment of function and MRI, with subjective assessment concordant in fewer than half of cases.
The first aim of this study was to investigate the accuracy of subjective echocardiographic assessment of systolic RV function in patients with HLHS, when compared with cardiac MRI–derived EF ( Videos 1–2 [available at www.onlinejase.com ]). As secondary aims, we sought to investigate the impact of observer training and experience on the correlation of echocardiographic and MRI methods, as well as the performance of echocardiography when used as a screening test to detect reduced RV EF measured on MRI.
Ethical and institutional approval was granted and informed consent was obtained from parents or legal guardians. All patients with HLHS (defined as mitral stenosis or atresia with aortic stenosis or atresia and atrioventricular and ventriculoarterial concordance) undergoing cardiac MRI under general anesthesia were included in the study. At our institution, cardiac MRI is routinely used in combination with echocardiography before hemi-Fontan and total cavopulmonary connection as well as for patients after total cavopulmonary connection. Therefore, all patients with HLHS undergo MRI before hemi-Fontan and total cavopulmonary connection. Patients were excluded if parental consent was not given or if there was cardiovascular instability during the MRI scan, precluding additional time for echocardiography. Data were prospectively acquired but retrospectively analyzed for this study.
MRI scans were performed on a Philips 1.5-T Achieva scanner (Philips Medical Systems, Best, The Netherlands) and were reevaluated using ViewForum EWS version 2.0 (Philips Medical Systems, Best, The Netherlands). Two-dimensional steady-state free precession cine imaging oriented to the short axis of the right ventricle was used to calculate ventricular volumes using the disk summation method. End-diastolic and end-systolic contours were manually traced (excluding major trabeculations from the volume) to determine end-diastolic volume, end-systolic volume, stroke volume, and EF. MRI EF was categorized as used in our clinical department: good function (≥50%), moderate function (40%–49%), or poor function (<40%).
Echocardiography was undertaken immediately after the MRI scan under the same general anesthetic to avoid potential physiologic changes. Comprehensive echocardiography was performed on a Philips iE33 ultrasound system (Philips Medical Systems, Andover, MA). Subcostal, apical, long-axis, and short-axis views were obtained, whenever acoustic windows permitted. Two-dimensional cine images (no color) were anonymized and reviewed by a range of observers working within the department of congenital heart disease with varying levels of experience of echocardiography in this patient group. Observers were blinded to the MRI results. Each observer was asked on the basis of the images to categorize global RV systolic function as good, moderate, or poor consistent with the normal subjective description in clinical use at our institution. This method has been used previously to compare echocardiography and MRI in both the biventricular and single-ventricle setting.
Observers were grouped by experience level: residents with <6 months’ exposure to echocardiography ( n = 5), junior fellows with <3 years of training ( n = 6), senior fellows with >3 years of training ( n = 5), cardiac physiologists ( n = 5), and attending cardiologists ( n = 7). Further delineation of the experience of each group is given in the Appendix .
Observers were scored on the basis of the concordance of their visual assessment of RV systolic function on echocardiography with MRI EF (0 if concordant, 1 if a single functional echocardiographic grade different, and 2 if functional echocardiographic grade differed by two grades compared with the MRI scan). For example, if an MRI EF was good (>50%) and the observer rated it as poor echocardiographically, this was considered two grades different. Each observer was given a total score (calculated from the total of grades different); this was then averaged for each observer experience group. Thus, a low score means that there was a high level of agreement between the echocardiographic assessment and MRI, and a high score indicates worse agreement between the echocardiographic and MRI methods. To evaluate variability in assessment in each observer group, intraclass correlation coefficients were calculated within in each observer group (two-way random, absolute agreement).
An important clinical consideration is the ability of echocardiography to accurately detect reduced RV systolic function. If an RV EF of <50% assessed by MRI is regarded as “reduced function,” sensitivity, specificity, positive predictive value, and negative predictive value of subjective echocardiographic function were calculated for each observer group. Echocardiographically assessed moderate or poor function was taken to indicate reduced function.
All MRI scans were analyzed by two observers (H.R.B.-R. and A.J.B.). All echocardiograms were graded for quality by the same two observers. Scans were graded from 1 (poor quality) to 4 (excellent quality). Intraclass correlation coefficients were calculated to assess interobserver variation for MRI analysis (two-way random, absolute agreement).
Twenty-eight patients with HLHS underwent echocardiography under the same general anesthetic as MRI between July 2007 and January 2009. All patients had magnetic resonance and echocardiographic images available for analysis. The demographics and MRI results are shown in Table 1 . Twenty-three patients (82%) had MRI EFs ≥ 50%, four (14%) had MRI EFs of 40% to 49%, and one (4%) had an MRI EF < 40%. The median MRI EF for the whole group was 59% (range, 33%–78%).
|Stage||n (%)||Age (mo)||Weight (kg)||Saturation (%)||EDVi (mL/m 2 )||ESVi (mL/m 2 )||SVi (mL/m 2 )||COi (L/min/m 2 )||EF (%)|
|Post-Norwood ∗||14 (50%)||3 (2–5)||4.98 (3.42–6.83)||78 (72–98)||92 (48–113)||47 (16–57)||46 (26–60)||4.8 (2.2–6.7)||55 (33–63)|
|Post–hemi-Fontan||11 (39%)||27.5 (24–49)||12.85 (10.4–15.4)||84 (70–89)||79 (54–173)||32 (15–77)||51 (34–96)||4.3 (3.6–10)||62.5 (43–78)|
|Post-Fontan||3 (11%)||104 (76–111)||29 (23.6–32.5)||92 (92–95)||70 (52–75)||23 (20–29)||46 (29–50)||3.1 (2.7–4)||60 (60–70)|
Interobserver variability for MRI analysis showed good concordance. Intraclass correlation coefficients were 0.945 (95th confidence interval [CI], 0.741–0.91), 0.952 (95% CI, 0.779–0.984), 0.926 (95% CI, 0.780–0.970), and 0.885 (95% CI, 0.764–0.946) for end-diastolic volume, end-systolic volume, stroke volume, and EF, respectively.
When evaluating the echocardiograms, there was a trend toward improved concordance with increasing experience ( Figure 1 ). Attending cardiologists assessed the grade of function the same as MRI in 58.2% of patients, compared with 55% of senior fellows, 47.1% of cardiac physiologists, 47.6% of junior fellows, and 31.4% of residents. Concordance within each observer group also increased with increasing experience, from 0.674 (95% CI, 0.440–0.831) in residents to 0.876 (95% CI, 0.789–0.936) in attending cardiologists. Twenty-one echocardiograms (75%) were rated as having good to excellent quality clips; all loops were included to reflect the normal clinical situation of varying quality of images. It was noted that concordance with MRI in those with less than good to excellent images was lower than those with good to excellent clips ( Table 2 ).