Quantitative Assessment of Regional Pulmonary Transit Times in Pulmonary Hypertension.

BACKGROUND: Pulmonary hypertension (PH) contributes to restricted flow through the pulmonary circulation characterized by elevated mean pulmonary artery pressure acquired from invasive right heart catheterization (RHC). MRI may provide a noninvasive alternative for diagnosis and characterization of PH. PURPOSE: To characterize PH via quantification of regional pulmonary transit times (rPTT). STUDY TYPE: Retrospective. POPULATION: A total of 43 patients (58% female); 24 controls (33% female). RHC-confirmed patients classified as World Health Organization (WHO) subgroups 1-4. FIELD STRENGTH/SEQUENCE: A 1.5 T/time-resolved contrast-enhanced MR Angiography (CE-MRA). ASSESSMENT: CE-MRA data volumes were combined into a 4D matrix (3D resolution + time). Contrast agent arrival time was defined as the peak in the signal-intensity curve generated for each voxel. Average arrival times within a vessel region of interest (ROI) were normalized to the main pulmonary artery ROI (t0 ) for eight regions to define rPTT for all subjects. Subgroup analysis included grouping the four arterial and four venous regions. Intraclass correlation analysis completed for reproducibility. STATISTICAL TESTS: Analysis of covariance with age as covariate. A priori Student's t-tests or Wilcoxon rank-sum test; α = 0.05. Results compared to controls unless noted. Significant without listing P value. ICC ran as two-way absolute agreement model with two observers. RESULTS: PH patients demonstrated elevated rPTT in all vascular regions; average rPTT increase in arterial and venous branches was 0.85 ± 0.15 seconds (47.7%) and 1.0 ± 0.18 seconds (16.9%), respectively. Arterial rPTT was increased for all WHO subgroups; venous regions were elevated for subgroups 2 and 4 (group 1, P = 0.86; group 3, P = 0.32). No significant rPTT differences were found between subgroups (P = 0.094-0.94). Individual vessel ICC values ranged from 0.58 to 0.97. DATA CONCLUSION: Noninvasive assessment of PH using standard-of-care time-resolved CE-MRA can detect increased rPTT in PH patients of varying phenotypes compared to controls. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY: Stage 3.

Evaluation of Pulmonary Hypertension Using 4D Flow MRI.

BACKGROUND: Cardiac magnetic resonance imaging (MRI) is becoming an alternative to right heart catheterization (RHC) for evaluating pulmonary hypertension (PH). A need exists to further evaluate cardiac MRI's ability to characterize PH. PURPOSE: To evaluate the potential for four-dimensional (4D) flow MRI-derived pulmonary artery velocities to characterize PH. STUDY TYPE: Prospective case-control. POPULATION: Fifty-four PH patients (56% female); 25 controls (36% female). FIELD STRENGTH/SEQUENCE: 1.5 T; gradient recalled echo 4D flow and balanced steady-state free precession cardiac cine. ASSESSMENT: RHC was used to derive patients' pulmonary vascular resistance (PVR). 4D flow measured blood velocities at the main, left, and right pulmonary arteries (MPA, LPA, and RPA); cine measured ejection fraction, end diastolic, and end systolic volumes (EF, EDV, and ESV). EDV and ESV were normalized (indexed) to body surface area (ESVI and EDVI). Parameters were evaluated between, and within, PH subgroups: pulmonary arterial hypertension (PAH); PH due to left heart disease (PH-LHD)/chronic lung disease (PH-CLD)/or chronic thrombo-emboli (CTE-PH). STATISTICAL TESTS: Analysis of variance and Kruskal-Wallis tests compared parameters between subgroups. Pearson's r assessed velocity, PVR, and volume correlations. Significance definition: P < 0.05. RESULTS: PAH peak and mean velocities were significantly lower than in controls at the LPA (36 ± 12 cm/second and 20 ± 4 cm/second vs. 59 ± 15 cm/second and 32 ± 9 cm/second). At the RPA, mean velocities were significantly lower in PAH vs. controls (27 ± 6 cm/second vs. 40 ± 9 cm/second). Peak velocities significantly correlated with right ventricular EF at the MPA (r = 0.286), RPA (r = 0.400), and LPA (r = 0.401). Peak velocity significantly correlated with right ventricular ESVI at the RPA (r = -0.355) and LPA (r = -0.316). Significant correlations between peak velocities and PVR were moderate at the LPA in PAH (r = -0.641) and in PH-LHD (r = -0.606) patients, and at the MPA in PH-CLD (r = -0.728). CTE-PH showed non-significant correlations between peak velocity and PVR at all locations. DATA CONCLUSION: Preliminary findings suggest 4D flow can identify PAH and track PVR changes. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY: Stage 5.

MRA of the Supraaortic Vasculature: Comparison of Gadobutrol and Gadoterate Meglumine at 1.5 T.

BACKGROUND: Gadobutrol (GB) and gadoterate meglumine (GM) are contrast agents used for contrast-enhanced magnetic resonance angiography (CEMRA). Supraaortic vasculature (SAV) CEMRAs are used to evaluate stroke risk and neurologic symptoms. There is a need to compare the SAV CEMRA image quality obtained with GB and GM. PURPOSE: To intra-individually compare MRA images obtained with equimolar GB and GM at 1.5 T in the SAV. STUDY TYPE: Prospective, crossover. POPULATION: Twenty-eight subjects (54 ± 13 years; 17 female). FIELD STRENGTH/SEQUENCE: 1.5 T; three-dimensional (3D) gradient recalled echo. ASSESSMENT: Quantitative image quality was measured by normalized signal intensity (SIn ) [SIn  = SI blood/SD blood] and contrast ratio (CR) [CR = SI blood/SI muscle], determined by an observer (JWC) with 1 year of vascular imaging experience. Three radiologists (AS, PA, and MU) with (5, 5, and 6 years of) vascular imaging experience evaluated image quality by Likert-scale ratings (of image impression, wall conspicuity, and artifact absence). STATISTICAL TESTS: SIn and CR were compared with paired t-tests or Wilcoxon signed-rank tests and Bland-Altman plots. Qualitative ratings were compared with Wilcoxon signed-rank test. RESULTS: No significant difference in SIn was found between GB and GM. CRs with GB were significantly higher than GM at the right common carotid (6.9 ± 2.5 vs. 4.8 ± 1), left internal carotid (7.3 ± 2 vs. 4.4 ± 1.2), right internal carotid (7.7 ± 2.2 vs. 5 ± 1.1), and left vertebral (6.6 ± 2.2 vs. 4.5 ± 1.1) arteries. Bland-Altman plots showed relatively greater differences between GB and GM at higher CRs and SIn s. GM showed significantly higher artifact than GB (3.56 ± 0.52 vs. 3.36 ± 0.46) and significantly lower overall image quality (10.73 ± 1.45 vs. 11.26 ± 1.58) at the left vertebral artery. DATA CONCLUSION: At 1.5 T and equimolar demonstration, GB (0.1 mL/kg, i.e., 0.1 mmol/kg) showed higher CRs in the SAV compared to GM (0.2 mL/kg, i.e., 0.1 mmol/kg) at most vessels. Subjective image quality was not significantly different between the two agents for most vessels. LEVEL OF EVIDENCE: 2 TECHNICAL EFFICACY: Stage 2.

Potential Prognostic Value of Native T1 in Pulmonary Hypertension Patients.

Native T1, extracellular volume fraction (ECV), and late gadolinium enhancement (LGE) characterize myocardial tissue and relate to patient prognosis in a variety of diseases, including pulmonary hypertension. The purpose of this study was to evaluate if left ventricle (LV) fibrosis measurements have prognostic value for cardiac outcomes in pulmonary hypertension subgroups. 54 patients with suspected pulmonary hypertension underwent right-heart catheterization and were classified into pulmonary hypertension subgroups: pre-capillary component (PreCompPH) and isolated post-capillary (IpcPH). Cardiac magnetic resonance imaging (MRI) scans were performed with the acquisition of balanced cine steady-state free precession, native T1, and LGE pulse sequences to measure cardiac volumes and myocardial fibrosis. Associations between cardiac events and cardiac MRI measurements were analyzed within PreCompPH and IpcPH patients. IpcPH: LV native T1 was higher in patients who experienced a cardiac event within two years vs. those who did not. In patients with LV native T1 > 1050 ms, the rate of cardiac events was higher. ECV and quantitative LGE did not differ between groups. PreCompPH: native T1, ECV, and quantitative/qualitative LGE did not differ between patients who experienced a cardiac event within two years vs. those who did not. LV native T1 may have potential value for forecasting cardiac events in IpcPH, but not in PreCompPH, patients.

Combined modality PET/MR for the detection of severe large vessel vasculitis.

BACKGROUND: Large vessel vasculitis (LVV) can be characterized based on symptom severity, and this characterization helps clinicians decide upon treatment approach. Our aim was to compare the imaging findings of combined modality positron emission tomography/magnetic resonance (PET/MR) and inflammatory markers between severe and non-severe LVV. A retrospective query was performed to identify all patients with LVV who underwent PET/MR at our institution between January 2015 and January 2021. RESULTS: Eleven patients (nine females; age 62.2 ± 16.4 years) underwent 15 PET/MR scans. Positivity was defined by findings indicative of active LVV on each modality: PET positive if vessel metabolic activity > liver metabolic activity; MR positive if wall thickening or contrast enhancement. When positive PET or positive MR findings were considered a positive scan, LVV patients with severe disease (n = 9 scans) showed a higher number of positive scans (n = 9) compared to the number of positive scans in non-severe patients (n = 3) (p < 0.05). The sensitivity and specificity for the detection of severe LVV were 1.00 and 0.50, respectively. When only the presence of both positive PET and positive MR findings were considered a positive scan, inflammatory marker levels were not significantly different between severe and non-severe LVV groups (severe: erythrocyte sedimentation rate (ESR) = 9.8 ± 10.6 mm/h; C-reactive protein (CRP) = 0.6 ± 0.4 mg/dL) (non-severe: ESR = 14.3 ± 22.4 mm/h; CRP = 0.5 ± 0.6 mg/dL). Blood- and liver-normalized maximum standardized uptake values were not significantly different between severe and non-severe patients (1.4 ± 0.3 vs 1.5 ± 0.4; 1.1 ± 0.4 vs 1.0 ± 0.3, respectively). CONCLUSIONS: Because of the differences observed, PET/MR appears to be better suited to facilitate the characterization of LVV as severe or non-severe compared to inflammatory marker measurements and quantitative measurements of metabolic activity. Qualitative assessment of PET and MR positivity by 18F-fluorodeoxyglucose PET/MR may be able to supplement clinical symptoms-based LVV classification decisions and may be helpful when clinical symptoms overlap with other disease processes.

Left Ventricular Fibrosis Assessment by Native T1, ECV, and LGE in Pulmonary Hypertension Patients.

Cardiac magnetic resonance imaging (MRI) is emerging as an alternative to right heart catheterization for the evaluation of pulmonary hypertension (PH) patients. The aim of this study was to compare cardiac MRI-derived left ventricle fibrosis indices between pre-capillary PH (PrePH) and isolated post-capillary PH (IpcPH) patients and assess their associations with measures of ventricle function. Global and segmental late gadolinium enhancement (LGE), longitudinal relaxation time (native T1) maps, and extracellular volume fraction (ECV) were compared among healthy controls (N = 25; 37% female; 52 ± 13 years), PH patients (N = 48; 60% female; 60 ± 14 years), and PH subgroups (PrePH: N = 29; 65% female; 55 ± 12 years, IpcPH: N = 19; 53% female; 66 ± 13 years). Cardiac cine measured ejection fraction, end diastolic, and end systolic volumes and were assessed for correlations with fibrosis. LGE mural location was qualitatively assessed on a segmental basis for all subjects. PrePH patients had elevated (apical-, mid-antero-, and mid-infero) septal left ventricle native T1 values (1080 ± 74 ms, 1077 ± 39 ms, and 1082 ± 47 ms) compared to IpcPH patients (1028 ± 53 ms, 1046 ± 36 ms, 1051 ± 44 ms) (p < 0.05). PrePH had a higher amount of insertional point LGE (69%) and LGE patterns characteristic of non-vascular fibrosis (77%) compared to IpcPH (37% and 46%, respectively) (p < 0.05; p < 0.05). Assessment of global LGE, native T1, and ECV burdens did not show a statistically significant difference between PrePH (1.9 ± 2.7%, 1056.2 ± 36.3 ms, 31.2 ± 3.7%) and IpcPH (2.7 ± 2.7%, 1042.4 ± 28.1 ms, 30.7 ± 4.7%) (p = 0.102; p = 0.229 p = 0.756). Global native T1 and ECV were higher in patients (1050.9 ± 33.8 and 31.0 ± 4.1%) than controls (28.2 ± 3.7% and 1012.9 ± 29.4 ms) (p < 0.05). Cardiac MRI-based tissue characterization may augment understanding of cardiac involvement and become a tool to facilitate PH patient classification.

Pediatric facial kaposiform hemangioendothelioma: a case report and review of the literature.

Cutaneous kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are vascular tumors that are often misdiagnosed. Treatment urgency and type varies depending on which tumor-type is diagnosed, because of the differing rates of progression to Kasabach-Merritt phenomenon. An 11-month-old male presented with a facial mass and biopsy results favoring a diagnosis of TA. The diagnosis of KHE, was later favored over TA given the overall clinical picture of initial rapid growth. However, serial imaging showed no subsequent growth, suggesting that clinically diagnosed KHE, may follow a benign clinical course when biopsy results suggest a diagnosis of TA.