Search

We aimed to determine quantitative sacroiliac (SI) joint magnetic resonance imaging (MRI) cutoffs for active and structural lesions that will be incorporated as imaging domains in classification criteria of axial disease in juvenile spondyloarthritis (SpA). MRI scans from an international cross‐section of juvenile SpA patients were reviewed by 6 musculoskeletal imaging experts blinded to clinical details. Raters globally assessed the presence/absence of lesions typical of axial SpA and performed SI joint quadrant‐ or joint‐based scoring. Sensitivity and specificity of lesion cutoffs were calculated using a rater majority (≥4 of 6 raters) on a global assessment of the presence/absence of active or structural lesions typical of axial SpA with high confidence as the reference standard. Cutoffs were validated in an independent cohort. Imaging from 243 subjects, 61% male, median age 14.9 years, had sequences available for detailed MRI scoring. Optimal cutoffs for defining lesions typical of axial disease in juvenile SpA were: 1) inflammatory lesion: bone marrow edema in ≥3 SI joint quadrants across all SI joint MRI slices (sensitivity 98.6%, specificity 96.5%); 2) structural lesions: erosion in ≥3 quadrants or sclerosis or fat lesion in ≥2 SI joint quadrants or backfill or ankylosis in ≥2 joint halves across all SI joint MRI slices (sensitivity 98.6%, specificity 95.5%). Sensitivity and specificity of the optimal cutoffs in the validation cohort were excellent. We propose data‐driven cutoffs for active inflammatory and structural lesions on MRI typical of axial disease in juvenile SpA that have high specificity and sensitivity using central imaging global assessment as the reference standard and excellent reliability.

A diagnostic strategy for assessment of childhood interstitial lung disease is necessary for understanding and categorizing these conditions and facilitating improved patient care.

Introduction:Pulmonary regurgitation after surgical repair of tetralogy of Fallot (rToF) can result in progressive right ventricular (RV) dilatation and biventricular dysfunction leading to adverse clinical outcomes. Although pulmonary valve replacement (PVR) is considered to be critical for preservation of ventricular function, effects of PVR on ventricular strain are unknown. We aimed to assess the impact of PVR on ventricular mechanics in patients with rToF.Methods:Fifty five rToF patients who underwent cardiac magnetic resonance imaging (CMR) prior to and after PVR were assessed retrospectively. Global radial strain (GRS), global circumferential strain (GCS), and global longitudinal strain (GLS) were calculated by feature tracking analysis using combinations of short axis (sax) and 4-chamber view (4cv) images. Global measures of biventricular performance by CMR were also calculated.Results:Mean age at CMR prior to PVR was 19.2 ? 8.0 years old (yo), at PVR was 22.3 ? 7.5 yo, and at CMR after PVR was 26.4 ? 4.6 years old. Following PVR, RV GRS and GLS decreased (p < 0.001 and < 0.01, respectively), while LV GRS and GCS also significantly decreased (p < 0.01 and 0.02, respectively) (Fig. 1). As expected, following PVR, pulmonary regurgitation fraction (PRF), heart rate (HR), RV end-diastolic volume (EDV), RV end-systolic volume (ESV) and RV mass significantly declined with no significant change in RV ejection fraction (EF). In addition, left ventricular (LV) ESV, EDV, and mass significantly increased with no significant change in LVEF and LV cardiac index (CI) after PVR, demonstrating a beneficial ventricular-ventricular interaction (Table 1).Conclusions:Paradoxical decrease in biventricular strain is seen in rToF patients after PVR. This is most likely due to decreased need for RV contractility after abolition of RV volume overload and a more efficient geometry of LV contraction. Follow up strain studies will be needed for long term effects.

Introduction:Aortopulmonary collateral (APC) formation in single ventricle physiology is well known and associated with high morbidity which may include adverse neurodevelopmental outcome (NDO) because of the established inverse relationship between cerebral blood flow (CBF) and APC flow. How CBF changes in relation to APC flow as Fontan patients age remains unknown.Hypothesis:As Fontan patients age, APC flow decreases resulting in increased CBF.Methods:Patients with single ventricle after Fontan underwent serial cardiac magnetic resonance (CMR) 6-9 months after Fontan surgery and were followed up longitudinally 5-7 years later. CBF and APC flows were calculated using the phase contrast CMR in internal jugular veins, cavae, aorta (Ao), and pulmonary arteries and veins. Bivariate linear regression models for change in CBF indexed to BSA (?CBFBSA) and indexed to Ao flow (?CBFAo) were estimated. Statistical significance was taken at the 0.05 level.Results:Twenty-four (71% male) patients were enrolled. Mean age at Fontan was 3.37 years (SD: 1.31). A statistically significant inverse relationship between the change in CBF (?CBF) and the change in APC flow (?APC) was found (between ?CBFAoand ?APCBSA: estimate= -5.28, standard error [SE] = 2.47, p=0.043; between ?CBFAoand ?APCAo: estimate= -0.27, SE= 0.13, p=0.045). (Figure1 and 2). That is to say that as patients age, if their APC flow decreased, their CBF increased.Conclusions:Decreasing APC flow after Fontan results in increasing CBF, but this process can take years. Interventions to alter APC flow may improve CBF and thus potentially improve NDO; prospective investigation with a larger patient population is warranted.

Background:In the past 2 decades patients living with Fontan physiology have had improved life expectancy but continued high rates of morbidity. Alterations in flow equilibrium within this circulation are known to place patients at risk for Fontan morbidity. However little is known regarding how blood flow through the Fontan circuit changes over time. This study uses serial cardiac magnetic resonance (CMR) data to describe longitudinal trends in palliated single ventricle circulation.Methods:This was a retrospective single center study of all patients who underwent Fontan completion from 2008 to 2017 and underwent serial CMRs at ? 2 of the following time points: prior to Fontan, 3-9 months after Fontan and >1 year after Fontan. Flow was measured in the right (RSVC) and left superior vena cava (LSVC), inferior vena cava (IVC), Native Aorta, Neo-Aorta, right (RPA) and left pulmonary artery (LPA) indexed to BSA. Trends were studied using linear mixed effects models with random intercepts.Results:Sixty-three patients were studied; 18 had serial CMRs at 3 time points and 4 patients had MRIs at 4 time points. There was a linear increase in RPA and LPA flow and a linear decrease in SVC flow with time from Fontan (P=0.016 RPA, 0.009 LPA, 0.0001 SVC). Both neo-aortic and IVC flow decreased sharply in the first 1-2 years post Fontan however IVC flow increased after 2 years. This non-linear trend was statistically significant (P<0.0001 IVC, 0.0004 Neo Aorta).Systemic to pulmonary collateral flow decreased in a non-linear fashion the first year after Fontan (p < 0.0001).Conclusion:Serial data demonstrate specific longitudinal trends in vascular flow after conversion to a total cavopulmonary circulation. Early Fontan physiology is characterized a sharp decrease in IVC and neo-aortic flow, and linear increase in pulmonary artery flow over time. This data provides a framework with which serial CMR data can be used to identify patients who have abnormal adaptation to the Fontan circulation.

Introduction:Single right ventricular (SRV) morphology has been associated with adverse outcomes in Fontan patients. The impact of ventricular morphology on ventricular strain with the imposition of Fontan physiology is not well known. We sought to evaluate contractile function in single ventricle patients prior to and after Fontan surgery.Methods:SRV (n = 38) and single left ventricular (SLV) morphology (n = 11) underwent cardiac magnetic resonance imaging (CMR) prior to and after Fontan surgery. Global radial strain (GRS) and global circumferential strain (GCS) were calculated by feature tracking using the short axis and 4 chamber view images. Ejection Fraction (EF) and atrioventricular valve regurgitation (AVVR) were also measured.Results:Age at CMR prior to Fontan was 3.1 ? 1.3 years old (yo), at Fontan surgery was 3.4 ? 1.2 yo, and at CMR after Fontan was 5.8 ? 2.7 yo. GRS of SRV were significantly lower than SLV GRS both prior to (24.3 vs. 32.3%, p= 0.046) and after (21.8 vs. 30.6%, p= 0.03) Fontan. Although it didn?t reach statistical significance, mean SRV GCS were lower both prior to and after Fontan than SLV GCS. Both SRV GRS and SRV GCS deteriorated significantly after Fontan in contrast to SLV GRS and SLV GCS which did not (GRS; p< 0.01 and 0.61, GCS; p< 0.01 and 0.71, respectively) (Fig. 1A). There were no significant differences in EF between RV and LV both prior to and after Fontan, and EF didn?t deteriorate significantly after Fontan. While both GRS and GCS were not correlated with AVVR, EF was negatively correlated with AVVR both prior to (r = -0.30, p= 0.03) and after (r = -0.42, p< 0.01) Fontan (Fig. 1B).Conclusions:SRV strain are lower than SLV?s both prior to and after Fontan and deteriorates after Fontan surgery, whereas SLV strain does not. Our study suggests that strain measures can detect ventricular deterioration earlier than EF. In addition, the inverse correlation between AVVR and EF suggests that the single ventricle has lost ability to compensate for AVVR.

Introduction:Survivors of tetralogy of Fallot repair (TOF) can be left residua which may subject them to adverse outcomes. A large single center cohort with uniform surgery and imaging using cardiac magnetic resonance (CMR) measures may be able to predict these complications.Methods:Retrospective, single center study of all TOF survivors who underwent CMR from 2005-November 2017. CMR variables were extracted from reports or images analyzed. Medical and surgical history were obtained. Associations were tested using logistic or linear regression.Results:A total 667 TOF survivors underwent CMR (median 14.5 yo); 56% were male, 68% had routine TOF, 21% had TOF with pulmonary atresia, and 5% had TOF with absent pulmonary valve. Most (70%) had a transannular patch. With a mean followup of 4.8 +3.9 years, numerous CMR measures predicted adverse outcomes (Table). As in a previous multicenter trial, higher RV mass/volume was a significant risk factor for death, however, we also found that lower RV ejection fraction (EF) and left pulmonary artery (LPA) flow, were also significantly associated with death. Not surprisingly, higher RV volumes (in absolute terms and relative to the left ventricle (LV)) were associated with pulmonary valve replacement (PVR), however, lower RV EF was also associated with PVR. LV EF was associated with ventricular tachycardia (VT). For exercise, higher RV mass/volume predicted lower anaerobic threshold (? -140, P=.04) and higher LV volumes (? 1.1, P<.02) and cardiac index (CI) (? 14.2, P=.05) predicted greater maximum work. No risk factors were identified for hospitalization or pacemaker placement.Conclusions:In a large single center study, multiple CMR measures were associated with adverse outcomes in TOF survivors. RV mass/volume, EF and LPA flow were associated with greater odds of death; RV mass/volume also predicted lower anaerobic threshold. LV EF was associated with greater odds of VT. LV volumes and CI predicted greater maximum work.