Atrioventricular plane displacement and regional function to predict outcome in pulmonary arterial hypertension.

To investigate if left and right atrioventricular plane displacement (AVPD) or regional contributions to SV are prognostic for outcome in patients with pulmonary arterial hypertension (PAH). Seventy-one patients with PAH and 20 sex- and age-matched healthy controls underwent CMR. Myocardial borders and RV insertion points were defined at end diastole and end systole in cine short-axis stacks to compute biventricular volumes, lateral (SVlat%) and septal (SVsept%) contribution to stroke volume. Eight atrioventricular points were defined at end diastole and end systole in 2-, 3- and 4-chamber cine long-axis views for computation of AVPD and longitudinal contribution to stroke volume (SVlong%). Cut-off values for survival analysis were defined as two standard deviations above or below the mean of the controls. Outcome was defined as death or lung transplantation. Median follow-up time was 3.6 [IQR 3.7] years. Patients were 57 ± 19 years (65% women) and controls 58 ± 15 years (70% women). Biventricular AVPD, SVlong% and ejection fraction (EF) were lower and SVlat% was higher, while SVsept% was lower in PAH compared with controls. In PAH, transplantation-free survival was lower below cut-off for LV-AVPD (hazard ratio [HR] = 2.1, 95%CI 1.2-3.9, p = 0.02) and RV-AVPD (HR = 9.8, 95%CI 4.6-21.1, p = 0.005). In Cox regression analysis, lower LV-AVPD and RV-AVPD inferred lower transplantation-free survival (LV: HR = 1.16, p = 0.007; RV: HR = 1.11, p = 0.01; per mm decrease). LV-SVlong%, RV-SVlong%, LV-SVlat%, RV-SVlat%, SVsept% and LV- and RVEF did not affect outcome. Low left and right AVPD were associated with outcome in PAH, but regional contributions to stroke volume and EF were not.

Cardiac Magnetic Resonance Imaging in Pulmonary Arterial Hypertension: Ready for Clinical Practice and Guidelines?

PURPOSE OF REVIEW: Pulmonary arterial hypertension (PAH) is a progressive disease with high mortality. A greater understanding of the physiology and function of the cardiovascular system in PAH will help improve survival. This review covers the latest advances within cardiovascular magnetic resonance imaging (CMR) regarding diagnosis, evaluation of treatment, and prognostication of patients with PAH. RECENT FINDINGS: New CMR measures that have been proven relevant in PAH include measures of ventricular and atrial volumes and function, tissue characterization, pulmonary artery velocities, and arterio-ventricular coupling. CMR markers carry prognostic information relevant for clinical care such as treatment response and thereby can affect survival. Future research should investigate if CMR, as a non-invasive method, can improve existing measures or even provide new and better measures in the diagnosis, evaluation of treatment, and determination of prognosis of PAH.

Correction to: Cardiac Magnetic Resonance Imaging in Pulmonary Arterial Hypertension: Ready for Clinical Practice and Guidelines?

The original version of this article contains error in the text.

Right ventricular function parameters in pulmonary hypertension: echocardiography vs. cardiac magnetic resonance.

BACKGROUND: Right ventricular (RV) function is a major determinant of outcome in patients with pulmonary hypertension. Cardiac magnetic resonance (CMR) is gold standard to assess RV ejection fraction (RVEFCMR), however this is a crude measure. New CMR measures of RV function beyond RVEFCMR have emerged, such as RV lateral atrio-ventricular plane displacement (AVPDlat), maximum emptying velocity (S'CMR), RV fractional area change (FACCMR) and feature tracking of the RV free wall (FWSCMR). However, it is not fully elucidated if these CMR measures are in parity with the equivalent echocardiography-derived measurements: tricuspid annular plane systolic excursion (TAPSE), S'-wave velocity (S'echo), RV fractional area change (FACecho) and RV free wall strain (FWSecho). The aim of this study was to compare regional RV function parameters derived from CMR to their echocardiographic equivalents in patients with pulmonary hypertension and to RVEFCMR. METHODS: Fifty-five patients (37 women, 62 ± 15 years) evaluated for pulmonary hypertension underwent CMR and echocardiography. AVPDlat, S'CMR, FACCMR and FWSCMR from cine 4-chamber views were compared to corresponding echocardiographic measures and to RVEFCMR delineated in cine short-axis stack. RESULTS: A strong correlation was demonstrated for FAC whereas the remaining measurements showed moderate correlation. The absolute bias for S' was 2.4 ± 3.0 cm/s (relative bias 24.1 ± 28.3%), TAPSE/AVPDlat 5.5 ± 4.6 mm (33.2 ± 25.2%), FWS 4.4 ± 5.8% (20.2 ± 37.5%) and for FAC 5.1 ± 8.4% (18.5 ± 32.5%). In correlation to RVEFCMR, FACCMR and FWSecho correlated strongly, FACecho, AVPDlat, FWSCMR and TAPSE moderately, whereas S' had only a weak correlation. CONCLUSION: This study has demonstrated a moderate to strong correlation of regional CMR measurements to corresponding echocardiographic measures. However, biases and to some extent wide limits of agreement, exist between the modalities. Consequently, the equivalent measures are not interchangeable at least in patients with pulmonary hypertension. The echocardiographic parameter that showed best correlation with RVEFCMR was FWSecho. At present, FACecho and FWSecho as well as RVEFCMR are the preferred methods to assess and follow up RV function in patients with pulmonary hypertension. Future investigations of the CMR right ventricular measures, beyond RVEF, are warranted.

CMR feature tracking in cardiac asymptomatic systemic sclerosis: Clinical implications.

BACKGROUND: Impaired myocardial deformation has been sporadically described in cardiac asymptomatic systemic sclerosis (SSc). We aimed to study myocardial deformation indices in cardiac asymptomatic SSc patients using cardiac magnetic resonance feature tracking (CMR-FT) and correlate these findings to the phenotypic and autoimmune background. METHODS: Fifty-four cardiac asymptomatic SSc patients (44 females, 56±13 years), with normal routine cardiac assessment and CMR evaluation, including cine and late gadolinium enhancement (LGE) images, were included. SSc patients were compared to 21 sex- and age- matched healthy controls (17 females; 54±19 years). For CMR-FT analysis, a mid-ventricular slice for LV peak systolic radial and circumferential strain and a 4-chamber view for LV/RV peak systolic longitudinal strain were used. RESULTS: Twenty-four patients had diffuse cutaneous SSc and 30 limited cutaneous SSc. Thirteen patients had digital ulcers. Median disease duration was 3.6 years. LV ejection fraction was higher in SSc patients compared to controls (62±6% vs. 59±5%, p = 0.01). Four patients had no LGE examination; in the remaining patients LGE was absent in 74%, while 18% had RV insertion fibrosis and 8% evidence of subendocardial infarction. LV longitudinal strain differed in those with insertion fibrosis (-18.0%) and infarction (-16.7%) compared to no fibrosis (-20.3%, p = 0.04). Patients with SSc had lower RV longitudinal strain and strain rate compared to controls (p<0.001 and p = 0.01, respectively). All other strain and strain rate measurements were non-significant between patients and controls. CONCLUSIONS: In cardiac asymptomatic SSc patients with normal routine functional indices, CMR-FT identifies subclinical presence of insertion fibrosis and/or myocardial infarction by impaired LV longitudinal strain. RV derived longitudinal indices were impaired in the patient group. CMR FT indices did not correlate to the patients' phenotypic and autoimmune features.

Decreased biventricular longitudinal strain in patients with systemic sclerosis is mainly caused by pulmonary hypertension and not by systemic sclerosis per se.

PURPOSE: Patients with pulmonary arterial hypertension (PAH) due to systemic sclerosis (SSc) have high mortality. Left ventricular (LV) peak global longitudinal strain (GLS) is decreased in SSc. It is unknown whether low GLS is due to SSc or PAH. Therefore, our primary aim was to evaluate both LV and right ventricular free wall GLS (RVFW GLS) in SSc, with and without PAH, using cardiac magnetic resonance with feature tracking. Secondary aim was to relate GLS to invasive mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR). METHODS: Thirty-eight patients with SSc, 19 patients with SSc-PAH and 19 healthy controls for comparison, were included. Endocardial and epicardial borders were delineated in cine images (short-axis stack and three long-axis views) for volumetric and strain calculations. RESULTS: Systemic sclerosis-PAH had lower LV and RVFW GLS than SSc (LV: P = 0·01, RV: P<0·001) and controls (LV: P = 0·02; RV: P<0·001), with no difference between SSc and controls. LV strain correlated with mPAP (R = 0·42, P = 0·03) and PVR (R = 0·52, P = 0·006). RVFW GLS correlated with mPAP (R = 0·68, P<0·001) and PVR (R = 0·59, P = 0·001). ROC curves for predicting PAH had AUC 0·73 for LV strain (P = 0·003) and 0·86 for RVFW GLS (P<0·001). CONCLUSIONS: Lower GLS is mainly determined by increased pulmonary pressure and not by SSc per se. Low LV and RVFW GLS are indicative of increased mPAP and PVR, which opens for improved non-invasive methods to select patients eligible for right heart catheterization and to monitor the effects of PAH therapy.