The Society for Cardiovascular Magnetic Resonance Registry at 150,000.

OBJECTIVES: To summarize the status of the SCMR Registry at 150,000 exams. BACKGROUND: Cardiovascular magnetic resonance (CMR) is increasingly utilized to evaluate expanding cardiovascular conditions. The SCMR Registry is a central repository for real-world clinical data to support cardiovascular research, including those relating to outcomes, quality improvement, and machine learning. The SCMR Registry is built on a regulatory-compliant, cloud-based infrastructure that houses searchable content and Digital Imaging and Communications in Medicine (DICOM) images. METHODS: The processes for data security, data submission, and research access are outlined. We interrogated the Registry and present a summary of its contents. RESULTS: Data were compiled from 154,458 CMR scans across 20 United States sites, containing 299,622,066 total images (~100 terabytes of storage). The human subjects had an average age of 58 years (range 1 month to >90 years old), were 44% female, 72% Caucasian, and had a mortality rate of 8%. The most common indication was cardiomyopathy (27%), and most frequently used current procedural terminology (CPT) code was 75561 (35%). Macrocyclic gadolinium-based contrast agents represented 89% of contrast utilization after 2015. Short-axis cines were performed in 99% of scans, short-axis LGE in 66%, and stress perfusion sequences in 30%. Mortality data demonstrated increased mortality in patients with left ventricular ejection fraction (LVEF) < 35%, the presence of wall motion abnormalities, stress perfusion defects, and infarct late gadolinium enhancement (LGE), compared to those without these markers. There were 456,678 patient-years of all-cause mortality follow-up, with a median follow-up time of 3.6 years. CONCLUSIONS: The vision of the SCMR Registry is to promote evidence-based utilization of CMR through a collaborative effort by providing a web mechanism for centers to securely upload de-identified data and images for research, education, and quality control. The Registry quantifies changing practice over time and supports large-scale real-world multicenter observational studies of prognostic utility. CONDENSED ABSTRACT: The SCMR Registry is a central regulatory-compliant cloud-based repository for real-world clinical data and DICOM images for multicenter cardiovascular research, including outcomes-based data. The Registry contains 299,622,066 DICOM images and 456,678 patient-years follow-up. Data compiled from 154,458 CMR scans across 20 US sites demonstrated cardiomyopathy as the most common indication and 89% macrocyclic gadolinium contrast utilization after 2015. There was an overall mortality rate of 8%, with higher rates in those with LVEF<35%, abnormal wall motion, ischemia presence, or infarct LGE. The Registry aims to promote evidence-based CMR utilization through a collaborative effort to positively impact cardiovascular outcomes.

Artificial intelligence based left ventricular ejection fraction and global longitudinal strain in cardiac amyloidosis.

BACKGROUND: Assessment of left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) plays a key role in the diagnosis of cardiac amyloidosis (CA). However, manual measurements are time consuming and prone to variability. We aimed to assess whether fully automated artificial intelligence (AI) calculation of LVEF and GLS provide similar estimates and can identify abnormalities in agreement with conventional manual methods, in patients with pre-clinical and clinical CA. METHODS: We identified 51 patients (age 80 ± 10 years, 53% male) with confirmed CA according to guidelines, who underwent echocardiography before and/or at the time of CA diagnosis (median (IQR) time between observations 3.87 (1.93, 5.44 years). LVEF and GLS were quantified from the apical 2- and 4-chamber views using both manual and fully automated methods (EchoGo Core 2.0, Ultromics). Inter-technique agreement was assessed using linear regression and Bland-Altman analyses and two-way ANOVA. The diagnostic accuracy and time for detecting abnormalities (defined as LVEF ≤ 50% and GLS ≥ -15.1%, respectively) using AI was assessed by comparisons to manual measurements as a reference. RESULTS: There were no significant differences in manual and automated LVEF and GLS values in either pre-CA (p = .791 and p = .105, respectively) or at diagnosis (p = .463 and p = .722). The two methods showed strong correlation on both the pre-CA (r = .78 and r = .83) and CA echoes (r = .74 and r = .80) for LVEF and GLS, respectively. The sensitivity and specificity of AI-derived indices for detecting abnormal LVEF were 83% and 86%, respectively, in the pre-CA echo and 70% and 79% at CA diagnosis. The sensitivity and specificity of AI-derived indices for detecting abnormal GLS was 82% and 86% in the pre-CA echo and 100% and 67% at the time of CA diagnosis. There was no significant difference in the relationship between LVEF (p = .99) and GLS (p = .19) and time to abnormality between the two methods. CONCLUSION: Fully automated AI-calculated LVEF and GLS are comparable to manual measurements in patients pre-CA and at the time of CA diagnosis. The widespread implementation of automated LVEF and GLS may allow for more rapid assessment in different disease states with comparable accuracy and reproducibility to manual methods.

Imaging-Based Risk Stratification for Recurrence Risk in Women with a History of Peripartum Cardiomyopathy.

OBJECTIVE: Peripartum cardiomyopathy (PPCM) affects 1:1,000 U.S. pregnancies, and while many recover from the disease, the risk of recurrence in subsequent pregnancy (SSP) is high. This study aims to evaluate the utility of left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) to predict the risk of recurrence of PPCM in SSP. STUDY DESIGN: We retrospectively evaluated outcomes in women with a history of PPCM and SSP at a large-volume cardioobstetrics program (2008-2019). RESULTS: There were 18 women who had incident PPCM and pursued SSP. Of 24 pregnancies in these women, 8 (33%) were complicated by the development of recurrent PPCM. LVEF ≥ 52% or GLS ≤ -16 was associated with a low risk of recurrent PPCM. CONCLUSION: Approximately one-third of women with PPCM developed recurrent PPCM in SSP. LVEF and GLS on prepregnancy echocardiography may predict the risk of recurrence. Additional studies evaluating risk for recurrence are required to better understand which women are the safest to consider SSP. KEY POINTS: · Peripartum cardiomyopathy affects 1:1000 US pregnancies.. · Approximately one third of women with a history of peripartum cardiomyopathy developed recurrent disease in a subsequent pregnancy.. · A left ventricular ejection fraction ≥52% or global longitudinal strain ≤-16 on echocardiogram is associated with a low risk of recurrence..

Contemporary Understandings of Cardiovascular Disease After Cancer Radiotherapy: a Focus on Ischemic Heart Disease.

PURPOSE OF REVIEW: Radiation-induced cardiovascular disease, including coronary artery disease, is a well-known sequela of radiation therapy and represents a significant source of morbidity and mortality for cancer survivors. This review examines current literature and guidelines to care for this growing population of cancer survivors. RECENT FINDINGS: The development of radiation-induced ischemic heart disease following radiation can lead even to early cardiotoxicities, inclusive of coronary artery disease, which limit cancer treatment outcomes. These coronary lesions tend to be diffuse, complex, and proximal. Early detection with multimodality imaging and targeted intervention is required to minimize these risks. Early awareness, detection, and management of radiation-induced cardiovascular disease are paramount as cancer survivorship continues to grow.

Personalized Approach to Cancer Treatment-Related Cardiomyopathy.

PURPOSE OF REVIEW: Cancer treatment-related cardiotoxicity (CTRC) represents a significant cause of morbidity and mortality worldwide. The purpose of our review is to summarize the epidemiology, natural history, and pathophysiology of cardiotoxicity-related to cancer treatment. We also summarize appropriate screening, surveillance, and management of CTRC. While cardiotoxicity is characteristically associated with anthracyclines, HER2-B antagonists, and radiation therapy (XRT), there is growing recognition of toxicity with immune checkpoint inhibitors (ICI), tyrosine kinase inhibitors, and proteasome inhibitors. RECENT FINDINGS: Patients at risk for cardiotoxicity should be screened based on available guidelines, generally with serial echocardiograms. The role of medical heart failure (HF) therapies is controversial in patients with asymptomatic left ventricular dysfunction but may be considered in some instances. Once symptomatic HF has developed, treatment should be in accordance with ACC/AHA guidelines. The goal in caring for patients receiving cancer treatment is to optimize cardiac function and prevent interruptions in potentially lifesaving cancer treatment.

Hypertension and Heart Failure.

Hypertensive heart disease represents a spectrum of illnesses from uncontrolled hypertension to heart failure. The authors discuss the natural history and pathogenesis of heart failure owing to hypertensive heart disease, reviewing the important role of left ventricular hypertrophy as the inciting process leading to diastolic dysfunction and heart failure with preserved ejection fraction. They describe the various mechanisms by which a subset of patients ultimately develops systolic heart failure. They discuss management strategies for hypertensive heart disease at all stages of the disease process. Treatment in the initial stages before onset of heart failure may result in regression of disease.

ATTR Gene Variants in HCM.

Hypertrophic cardiomyopathy is the most common inherited cardiomyopathy, with a prevalence of 1:200 to 1:500. Cardiac amyloidosis, another cardiomyopathy caused by myocardial deposition of abnormally folded TTR protein, can be acquired or hereditary. The presence of pathogenic TTR gene variants in patients with phenotypic HCM is an underrecognized and clinically important entity.

Fibrosing Mediastinitis Caused by Histoplasmosis in an Adolescent.

Fibrosing mediastinitis (FM) is a rare, potentially progressive disease resulting from an idiosyncratic immune response to a variety of stimuli that lead to fibrous infiltration of the mediastinum and possible narrowing of the bronchovascular structures. We report an unusual case of FM in a pediatric patient presenting as myopericarditis and progressing to pericardial thickening and encasement of the mediastinal vascular structures needing surgical intervention. Imaging, including transthoracic echocardiography, cardiac computed tomography, and cardiac magnetic resonance played a crucial role in the diagnosis, assessment, and follow-up. Contrast-enhanced computed tomography can be especially helpful to demonstrate potential findings associated with FM.

Prevalence of valvular heart disease in cardiac amyloidosis and impact on survival.

BACKGROUND: Limited data exists on the prognostic impact of valvular heart disease in cardiac amyloidosis (CA). We therefore sought to define the prevalence of valvular disease in patients with CA and assess the effects of significant valve disease on survival. METHODS: This multi-center retrospective cohort study included consecutive patients with confirmed transthyretin (TTR) or light chain (AL) amyloidosis. Echocardiographic data closest to the date of amyloid diagnosis was reviewed, and severity was graded according to ASE guidelines. Kaplan-Meier survival analysis was performed to compare survival between patients with moderate or greater valve disease against those with mild or less disease. RESULTS: We included 345 patients (median age 76 years; 73 % men; 110 AL, 235TTR). The median survival for the total patient cohort with cardiac amyloidosis was 2.92 years, with 30 % of patients surviving at five years after their diagnosis. Median survival comparing AL vs ATTR was 2.58 years vs 2.82 years (p = 0.67) The most common valvular abnormalities in the total cohort were mitral (62 %) and tricuspid (66 %).regurgitation There was a statistically significant difference in median survival between patients with no or mild MR compared to those with moderate or severe MR (2.92 years vs 3.35 years, p = 0.0047) (Fig. 5). There was a statistically significant difference in median survival in patients with no or mild TR compared to those with moderate or severe TR (3.35 years vs 2.3 years, p = 0.015). CONCLUSION: Our study demonstrates a significant prevalence of mitral and tricuspid regurgitation in CA, with patients with moderate to severe MR and TR having a poorer prognosis.

Echocardiographic Detection of Regional Wall Motion Abnormalities Using Artificial Intelligence Compared to Human Readers.

BACKGROUND: Although regional wall motion abnormality (RWMA) detection is foundational to transthoracic echocardiography, current methods are prone to interobserver variability. We aimed to develop a deep learning (DL) model for RWMA assessment and compare it to expert and novice readers. METHODS: We used 15,746 transthoracic echocardiography studies-including 25,529 apical videos-which were split into training, validation, and test datasets. A convolutional neural network was trained and validated using apical 2-, 3-, and 4-chamber videos to predict the presence of RWMA in 7 regions defined by coronary perfusion territories, using the ground truth derived from clinical transthoracic echocardiography reports. Within the test cohort, DL model accuracy was compared to 6 expert and 3 novice readers using F1 score evaluation, with the ground truth of RWMA defined by expert readers. Significance between the DL model and novices was assessed using the permutation test. RESULTS: Within the test cohort, the DL model accurately identified any RWMA with an area under the curve of 0.96 (0.92-0.98). The mean F1 scores of the experts and the DL model were numerically similar for 6 of 7 regions: anterior (86 vs 84), anterolateral (80 vs 74), inferolateral (83 vs 87), inferoseptal (86 vs 86), apical (88 vs 87), inferior (79 vs 81), and any RWMA (90 vs 94), respectively, while in the anteroseptal region, the F1 score of the DL model was lower than the experts (75 vs 89). Using F1 scores, the DL model outperformed both novices 1 (P = .002) and 2 (P = .02) for the detection of any RWMA. CONCLUSIONS: Deep learning provides accurate detection of RWMA, which was comparable to experts and outperformed a majority of novices. Deep learning may improve the efficiency of RWMA assessment and serve as a teaching tool for novices.

Limitations of apical sparing pattern in cardiac amyloidosis: a multicentre echocardiographic study.

AIMS: Although impaired left ventricular (LV) global longitudinal strain (GLS) with apical sparing is a feature of cardiac amyloidosis (CA), its diagnostic accuracy has varied across studies. We aimed to determine the ability of apical sparing ratio (ASR) and most common echocardiographic parameters to differentiate patients with confirmed CA from those with clinical and/or echocardiographic suspicion of CA but with this diagnosis ruled out. METHODS AND RESULTS: We identified 544 patients with confirmed CA and 200 controls (CTRLs) as defined above (CTRL patients). Measurements from transthoracic echocardiograms were performed using artificial intelligence software (Us2.AI, Singapore) and audited by an experienced echocardiographer. Receiver operating characteristic curve analysis was used to evaluate the diagnostic performance and optimal cut-offs for the differentiation of CA patients from CTRL patients. Additionally, a group of 174 healthy subjects (healthy CTRL) was included to provide insight on how patients and healthy CTRLs differed echocardiographically. LV GLS was more impaired (-13.9 ± 4.6% vs. -15.9 ± 2.7%, P < 0.0005), and ASR was higher (2.4 ± 1.2 vs. 1.7 ± 0.9, P < 0.0005) in the CA group vs. CTRL patients. Relative wall thickness and ASR were the most accurate parameters for differentiating CA from CTRL patients [area under the curve (AUC): 0.77 and 0.74, respectively]. However, even with the optimal cut-off of 1.67, ASR was only 72% sensitive and 66% specific for CA, indicating the presence of apical sparing in 32% of CTRL patients and even in 6% healthy subjects. CONCLUSION: Apical sparing did not prove to be a CA-specific biomarker for accurate identification of CA, when compared with clinically similar CTRLs with no CA.

Cardiovascular computed tomography for the detection of quadricuspid aortic valve: A case report.

Aortic regurgitation (AR) represents a significant cause of morbidity and mortality. Due to its low cost and widespread availability, echocardiography remains the frontline for aortic valve (AV) assessment. However, poor sonographic windows may limit the assessment of valve morphology with this technique. Cardiovascular computed tomography (CCT) is increasingly utilized prior to structural AV interventions. Due to its excellent spatial resolution, CCT provides exceptional characterization of aortic leaflets. Accordingly, we present a case of a quadricuspid valve diagnosed by CCT. Here, CCT led to a new diagnosis of quadricuspid valve, highlighting the potential for CCT for the characterization of aortic leaflet morphology. CCT may be particularly useful in patients with contraindications to transesophageal echocardiography or those undergoing structural or robotic interventions.

Noninvasive assessment of left ventricular end-diastolic pressure using machine learning-derived phasic left atrial strain.

AIMS: While transthoracic echocardiography (TTE) assessment of left ventricular end-diastolic pressure (LVEDP) is critically important, the current paradigm is subject to error and indeterminate classification. Recently, peak left atrial strain (LAS) was found to be associated with LVEDP. We aimed to test the hypothesis that integration of the entire LAS time curve into a single parameter could improve the accuracy of peak LAS in the noninvasive assessment of LVEDP with TTE. METHODS AND RESULTS: We retrospectively identified 294 patients who underwent left heart catheterization and TTE within 24 h. LAS curves were trained using machine learning (100 patients) to detect LVEDP ≥ 15 mmHg, yielding the novel parameter LAS index (LASi). The accuracy of LASi was subsequently validated (194 patients), side by side with peak LAS and ASE/EACVI guidelines, against invasive filling pressures. Within the validation cohort, invasive LVEDP was elevated in 116 (59.8%) patients. The overall accuracy of LASi, peak LAS, and American Society of Echocardiography/European Association for Cardiovascular Imaging (ASE/EACVI) algorithm was 79, 75, and 76%, respectively (excluding 37 patients with indeterminate diastolic function by ASE/EACVI guidelines). When the number of LASi indeterminates (defined by near-zero LASi values) was matched to the ASE/EACVI guidelines (n = 37), the accuracy of LASi improved to 87%. Importantly, among the 37 patients with ASE/EACVI-indeterminate diastolic function, LASi had an accuracy of 81%, compared with 76% for peak LAS. CONCLUSION: LASi allows the detection of elevated LVEDP using invasive measurements as a reference, at least as accurately as peak LAS and current diastolic function guideline algorithm, with the advantage of no indeterminate classifications in patients with measurable LAS.

Preservation of Circumferential and Radial Left Ventricular Function as a Mitigating Mechanism for Impaired Longitudinal Strain in Early Cardiac Amyloidosis.

BACKGROUND: In patients with cardiac amyloidosis (CA), left ventricular ejection fraction (LVEF) is frequently preserved, despite commonly reduced global longitudinal strain (GLS). We hypothesized that nonlongitudinal contraction may initially serve as a mitigating mechanism to maintain cardiac output and studied the relationship between global circumferential (GCS) and radial (GRS) strain with LVEF and extracellular volume (ECV), a marker of amyloid burden. METHODS: Patients with CA who underwent cardiac magnetic resonance (CMR; n = 140, 70.7 ± 11.5 years, 66% male) or echocardiography (n = 67, 71 ± 13 years, 66% male) and normal controls (CMR, n = 20; echocardiography, n = 45) were retrospectively identified, and GCS, GLS, and GRS were quantified using feature-tracking CMR or speckle-tracking echocardiography and compared between CA patients with preserved and reduced LVEF (CAHFpEF, CAHFrEF) and controls. The prevalence of impaired strain (magnitudes <2.5th percentile of the controls) was compared between CAHFpEF and CAHFrEF and between ECV quartiles. RESULTS: While echocardiography-derived GLS was impaired in both CAHFpEF (-13.4% ± 3.1%, P < .003) and CAHFrEF (-9.1% ± 3.2%, P < .003), compared with controls (-20.8% ± 2.4%), GCS was more impaired in CAHFrEF compared with both controls (-15.6% ± 5.0% vs -32.3% ± 3.3%, P < .003) and CAHFpEF (-30.4% ± 5.7%, P < .003) and did not differ between CAHFpEF and controls (P = .24). The prevalence of abnormal CMR-derived GCS (P < .0001) and GRS (P < .0001) but not GLS (P = .054) varied significantly across ECV quartiles. CONCLUSIONS: Among CA patients with preserved LVEF, preserved GCS and GRS, despite near-universally impaired GLS, may be explained by an initial predominantly subendocardial involvement, where mostly longitudinal fibers are located. If confirmed in future studies, these findings may facilitate identification of patients with early stages of CA, when treatments may be most effective.

Non-invasive diagnosis of transthyretin cardiac amyloidosis utilizing typical late gadolinium enhancement pattern on cardiac magnetic resonance and light chains.

AIMS: While cardiac magnetic resonance (CMR) is often obtained early in the evaluation of suspected cardiac amyloidosis (CA), it currently cannot be utilized to differentiate immunoglobulin (AL) and transthyretin (ATTR) CA. We aimed to determine whether a novel CMR and light-chain biomarker-based algorithm could accurately diagnose ATTR-CA. METHODS AND RESULTS: Patients with confirmed AL or ATTR-CA with typical late gadolinium enhancement (LGE) and Look-Locker pattern for CA on CMR were retrospectively identified at three academic medical centres. Comprehensive light-chain analysis including free light chains, serum, and urine electrophoresis/immunofixation was performed. The diagnostic accuracy of the typical CMR pattern for CA in combination with negative light chains for the diagnosis of ATTR-CA was determined both in the entire cohort and in the subset of patients with invasive tissue biopsy as the gold standard. A total of 147 patients (age 70 ± 11, 76% male, 51% black) were identified: 89 ATTR-CA and 58 AL-CA. Light-chain biomarkers were abnormal in 81 (55%) patients. Within the entire cohort, the sensitivity and specificity of a typical LGE and Look-Locker CMR pattern and negative light chains for ATTR-CA was 73 and 98%, respectively. Within the subset with biopsy-confirmed subtype, the CMR and light-chain algorithm were 69% sensitive and 98% specific. CONCLUSION: The combination of a typical LGE and Look-Locker pattern on CMR with negative light chains is highly specific for ATTR-CA. The successful non-invasive diagnosis of ATTR-CA using CMR has the potential to reduce diagnostic and therapeutic delays and healthcare costs for many patients.

Normative values of the aortic valve area and Doppler measurements using two-dimensional transthoracic echocardiography: results from the Multicentre World Alliance of Societies of Echocardiography Study.

AIMS: Aortic valve area (AVA) used for echocardiographic assessment of aortic stenosis (AS) has been traditionally interpreted independently of sex, age and race. As differences in normal values might impact clinical decision-making, we aimed to establish sex-, age- and race-specific normative values for AVA and Doppler parameters using data from the World Alliance Societies of Echocardiography (WASE) Study. METHODS AND RESULTS: Two-dimensional transthoracic echocardiographic studies were obtained from 1903 healthy adult subjects (48% women). Measurements of the left ventricular outflow tract (LVOT) diameter and Doppler parameters, including AV and LVOT velocity time integrals (VTIs), AV mean pressure gradient, peak velocity, were obtained according to ASE/EACVI guidelines. AVA was calculated using the continuity equation. Compared with men, women had smaller LVOT diameters and AVA values, and higher AV peak velocities and mean gradients (all P < 0.05). LVOT and AV VTI were significantly higher in women (P < 0.05), and both parameters increased with age in both sexes. AVA differences persisted after indexing to body surface area. According to the current diagnostic criteria, 13.5% of women would have been considered to have mild AS and 1.4% moderate AS. LVOT diameter and AVA were lower in older subjects, both men and women, and were lower in Asians, compared with whites and blacks. CONCLUSION: WASE data provide clinically relevant information about significant differences in normal AVA and Doppler parameters according to sex, age, and race. The implementation of this information into clinical practice should involve development of specific normative values for each ethnic group using standardized methodology.

Extensive pulmonary venous stenoses as a complication of radiofrequency catheter ablation for atrial fibrillation.

Although pulmonary veins stenosis (PVS) is a well documented complication of radiofrequency-catheter ablation (RFCA) of atrial fibrillation (AF), simultaneous involvement of multiple PVs is extremely rare. We present the case of a 69 years-old male patient, with prior medical history of persistent AF, who had been treated with RFCA two years ago. After RFCA, he started with shortness of breath and needed hospitalization for bilateral pneumonia. One year after the procedure, he was on home oxygen, but still referred dyspnea, cough and hemoptysis. A transthoracic echocardiogram showed moderate right ventricular (RV) systolic dysfunction and elevated RV systolic pressure. Dedicated cardiac tomography for PV assessment revealed severe narrowing and pre-stenotic engorgement of all 5 PVs, with subtotal ostial occlusion of both the left lower and right middle PVs. PV angiography confirmed the diagnosis. Only the left and right upper PV were able to be wire-crossed and stented, with substantial reductions in stenosis from 90 % to 10 %. After 3 months of follow-up, the patient improved substantially, and home O2 was withdrawn.

Prevalence and haemodynamic profiles of pulmonary hypertension in cardiac amyloidosis.

OBJECTIVES: While cardiac amyloidosis (CA) classically involves the left ventricle (LV), less is known about its impact on the right ventricle (RV) and pulmonary vasculature. We performed a retrospective analysis to identify the prevalence and types of pulmonary hypertension (PH) profiles in CA and to determine haemodynamic and cardiovascular magnetic resonance (CMR) predictors of major adverse cardiovascular events (MACE). METHODS: Patients with CA who underwent CMR and right heart catheterisation (RHC) within 1 year between 2010 and 2019 were included. Patients were assigned the following haemodynamic profiles based on RHC: no PH, precapillary PH, isolated postcapillary PH (IPCPH), or combined precapillary and postcapillary PH (CPCPH). The relationship between PH profile and MACE (death, heart failure hospitalisation) was assessed using survival analysis. CMR and RV parameters were correlated with MACE using Cox-regression analysis. RESULTS: A total of 52 patients were included (age 69±9 years, 85% men). RHC was performed during biopsy in 44 (85%) and for clinical indications in 8 (15%) patients. Rates of no PH, precapillary PH, IPCPH and CPCPH were 5 (10%), 3 (6%), 29 (55%) and 15 (29%), respectively. Haemodynamic PH profile did not correlate with risk of death (p=0.98) or MACE (p=0.67). Transpulmonary gradient (TPG) (HR 0.88, CI 0.80 to 0.97), RV, (HR 0.95, CI 0.92 to 0.98) and LV ejection fraction (HR 0.95, CI 0.92 to 0.98) were significantly associated with MACE. CONCLUSIONS: PH is highly prevalent in CA, even at the time of diagnosis. While IPCPH was most common, CPCPH is not infrequent. TPG and RV ejection fraction (RVEF) are prognostic markers in this population.

Atrial function and geometry differences in transthyretin versus immunoglobulin light chain amyloidosis: a cardiac magnetic resonance study.

To determine the differences in left atrial (LA) function and geometry assessed by cardiac magnetic resonance (CMR) between transthyretin (ATTR) and immunoglobulin light chain (AL) cardiac amyloidosis (CA). We performed a retrospective analysis of 54 consecutive patients (68.5% male, mean age 67 ± 11 years) with confirmed CA (24 ATTR, 30 AL) who underwent comprehensive CMR examinations. LA structural and functional assessment including LA volume, LA sphericity index, and LA strain parameters were compared between both subtypes. In addition, 15 age-matched controls were compared to all groups. Patients with ATTR-CA were older (73 ± 9 vs. 62 ± 10 years, p < 0.001) and more likely to be male (83.3% vs. 56.7%, p = 0.036) when compared to AL-CA. No significant difference existed in LA maximum volume and LA sphericity index between ATTR-CA and AL-CA. LA minimum volumes were larger in ATTR-CA when compared with AL-CA. There was a significant difference in LA function with worse strain values in ATTR vs AL: left atrial reservoir [7.4 (6.3-12.8) in ATTR vs. 13.8 (6.90-24.8) in AL, p = 0.017] and booster strains [3.6 (2.6-5.5) in ATTR vs. 5.2 (3.6-12.1) in AL, p = 0.039]. After adjusting for age, LA reservoir remained significantly lower in ATTR-CA compared to AL-CA (p = 0.03), but not LA booster (p = 0.16). We demonstrate novel differences in LA function between ATTR-CA and AL-CA despite similar LA geometry. Our findings of more impaired LA function in ATTR may offer insight into higher AF burden in these patients.

The Utility of Noninvasive PET/CT Myocardial Perfusion Imaging in Adult Liver Transplant Candidates.

Background: The optimal cardiovascular (CV) risk stratification in liver transplant (LT) candidates remains unclear. The aim of this study was to evaluate concordance of findings between dobutamine stress echocardiography (DSE), positron emission tomography/computed tomography myocardial perfusion imaging (PET/CT MPI), and left heart catheterization in adult LT candidates. Methods: Data on 234 consecutive adult LT candidates from February 2015 to June 2018 with PET/CT MPI were reviewed. Adverse CV outcomes were adjudicated via chart review by a board-certified cardiologist. Results: Median age was 60.8, body mass index 30.2 kg/m2, and model of end-stage liver disease-sodium 14; 61% were male, and 54% had diabetes. Thirty-seven percent had nonalcoholic steatohepatitis and 29% alcohol-related liver disease. Sixty-five percent of patients had a DSE, of which 41% were nondiagnostic. No factors were independently associated with having a nondiagnostic DSE. The median global myocardial flow reserve correlated positively with hemoglobin and negatively with model of end-stage liver disease-sodium, age, ejection fraction, and body mass index. Moderate/high-risk MPIs were associated with older age and known CV disease. In patients with 2 cardiac testing modalities, findings were concordant in 87%. Eleven of 53 LT recipients experienced an adverse CV outcome, but no independent predictors were identified for this outcome. Conclusions: Results of different cardiac risk-stratification modalities were concordant across modalities the majority of the time in LT candidates, although these findings were not independently correlated with risk of post-LT CV outcomes. Given the high rates of nondiagnostic DSEs in this population, PET/CT MPI may be the preferred CV risk-stratification modality in older patients and those with known CV disease.