Equilibrium phase contrast-enhanced magnetic resonance angiography of the thoracic aorta and heart using balanced T1 relaxation-enhanced steady-state.

BACKGROUND: Three-dimensional (3D) contrast-enhanced magnetic resonance angiography (CEMRA) is routinely used for vascular evaluation. With existing techniques for CEMRA, diagnostic image quality is only obtained during the first pass of the contrast agent or shortly thereafter, whereas angiographic quality tends to be poor when imaging is delayed to the equilibrium phase. We hypothesized that prolonged blood pool contrast enhancement could be obtained by imaging with a balanced T1 relaxation-enhanced steady-state (bT1RESS) pulse sequence, which combines 3D balanced steady-state free precession (bSSFP) with a saturation recovery magnetization preparation to impart T1 weighting and suppress background tissues. An electrocardiographic-gated, two-dimensional-accelerated version with isotropic 1.1-mm spatial resolution was evaluated for breath-hold equilibrium phase CEMRA of the thoracic aorta and heart. METHODS: The study was approved by the institutional review board. Twenty-one subjects were imaged using unenhanced 3D bSSFP, time-resolved CEMRA, first-pass gated CEMRA, followed by early and late equilibrium phase gated CEMRA and bT1RESS. Nine additional subjects were imaged using equilibrium phase 3D bSSFP and bT1RESS. Images were evaluated for image quality, aortic root sharpness, and visualization of the coronary artery origins, as well as using standard quantitative measures. RESULTS: Equilibrium phase bT1RESS provided better image quality, aortic root sharpness, and coronary artery origin visualization than gated CEMRA (P < 0.05), and improved image quality and aortic root sharpness versus unenhanced 3D bSSFP (P < 0.05). It provided significantly larger apparent signal-to-noise and apparent contrast-to-noise ratio values than gated CEMRA and unenhanced 3D bSSFP (P < 0.05) and provided ninefold better fluid suppression than equilibrium phase 3D bSSFP. Aortic diameter and main pulmonary artery diameter measurements obtained with bT1RESS and first-pass gated CEMRA strongly correlated (P < 0.05). CONCLUSIONS: We found that using bT1RESS greatly prolongs the useful duration of blood pool contrast enhancement while improving angiographic image quality compared with standard CEMRA techniques. Although further study is needed, potential advantages for vascular imaging include eliminating the current requirement for first-pass imaging along with better reliability and accuracy for a wide range of cardiovascular applications.

Science to Practice: Can Functional MR Imaging Be Useful in the Evaluation of Cardiorenal Syndrome?

Functional magnetic resonance (MR) imaging of the kidneys has gained interest recently, especially in the detection of early changes in acute kidney injury or to predict progression of chronic kidney disease (CKD). The application of these methods to cardiorenal syndrome (CRS) is novel. CRS is widely accepted as a complex clinical problem routinely faced by clinicians. In this issue, Chang et al ( 1 ) present their preliminary experience applying blood oxygen level-dependent (BOLD) MR imaging to the kidneys in mice with experimental myocardial infarction. They showed that R2* in the kidney increases after induced myocardial infarction and that the response was higher in animals with larger infarcts and over time. The authors also for the first time correlated the BOLD MR imaging findings against hypoxia-inducible factor-1α (HIF-1α) expression, an independent marker of renal hypoxia. In addition, they showed evidence for renal injury by using a kidney injury marker, kidney injury molecule-1 (KIM-1). The results of their study support the use of renal BOLD MR imaging in subjects with heart failure, in whom the risk of subsequent renal ischemia and/or hypoxia is known to exist. These results, along with those of other recent reports ( 2 ), suggest that functional imaging methods could play a key role in evaluating changes in both the primary and secondary organs involved in complex disease processes such as CRS. Availability of such methods could facilitate translation to the clinic and improve the mechanistic understanding of the complicated and interrelated pathophysiology.

Transseptal transcatheter mitral valve-in-valve: A step by step guide from preprocedural planning to postprocedural care.

Transcatheter mitral valve replacement has been successfully performed with the use of aortic transcatheter heart valves in hundreds of patients worldwide with severe dysfunction of a degenerated mitral bioprosthesis and high surgical risk for repeat operation. The delivery approach in the vast majority of the mitral valve-in-valve procedures has been transapical. Although the transseptal approach may be more technically challenging, it is less invasive and may be preferred by patients. Data from case series and a large international registry suggest that patients treated with transseptal mitral valve-in-valve have faster recovery, more improvement in left ventricular ejection fraction and possibly lower mortality compared with patients treated with transapical approach. A prospective clinical trial, the MITRAL trial (Mitral Implantation of TRAnscatheter vaLves) is evaluating the safety and feasibility of transvenous transseptal mitral valve-in-valve. The experience from this trial has allowed us to improve our procedural approach. In anticipation of a wider adoption of the transseptal approach for mitral valve-in-valve, we describe our current method step-by-step from planning the procedure through postprocedural management. This is an evolving technique that has changed with experience and the transition to newer generation transcatheter heart valve devices. We discuss the use of cardiac computed tomography for planning the procedure including transseptal puncture and valve size selection, provide procedural and technical tips, and discuss postprocedural care.

Cardiovascular cine imaging and flow evaluation using Fast Interrupted Steady-State (FISS) magnetic resonance.

BACKGROUND: Existing cine imaging techniques rely on balanced steady-state free precession (bSSFP) or spoiled gradient-echo readouts, each of which has limitations. For instance, with bSSFP, artifacts occur from rapid through-plane flow and off-resonance effects. We hypothesized that a prototype cine technique, radial fast interrupted steady-state (FISS), could overcome these limitations. The technique was compared with standard cine bSSFP for cardiac function, coronary artery conspicuity, and aortic valve morphology. Given its advantageous properties, we further hypothesized that the cine FISS technique, in combination with arterial spin labeling (ASL), could provide an alternative to phase contrast for visualizing in-plane flow patterns within the aorta and branch vessels. MAIN BODY: The study was IRB-approved and subjects provided consent. Breath-hold cine FISS and bSSFP were acquired using similar imaging parameters. There was no significant difference in biplane left ventricular ejection fraction or cardiac image quality between the two techniques. Compared with cine bSSFP, cine FISS demonstrated a marked decrease in fat signal which improved conspicuity of the coronary arteries, while suppression of through-plane flow artifact on thin-slice cine FISS images improved visualization of the aortic valve. Banding artifacts in the subcutaneous tissues were reduced. In healthy subjects, dynamic flow patterns were well visualized in the aorta, coronary and renal arteries using cine FISS ASL, even when the slice was substantially thicker than the vessel diameter. CONCLUSION: Cine FISS demonstrates several benefits for cardiovascular imaging compared with cine bSSFP, including better suppression of fat signal and reduced artifacts from through-plane flow and off-resonance effects. The main drawback is a slight (~ 20%) decrease in temporal resolution. In addition, preliminary results suggest that cine FISS ASL provides a potential alternative to phase contrast techniques for in-plane flow quantification, while enabling an efficient, visually-appealing, semi-projective display of blood flow patterns throughout the course of an artery and its branches.

Short-term results of alcohol septal ablation as a bail-out strategy to treat severe left ventricular outflow tract obstruction after transcatheter mitral valve replacement in patients with severe mitral annular calcification.

OBJECTIVES: To evaluate the outcomes of the early experience of percutaneous alcohol septal ablation in patients with severe left ventricular outflow tract (LVOT) obstruction post transcatheter mitral valve replacement (TMVR). BACKGROUND: Severe LVOT obstruction with hemodynamic compromise is a complication of TMVR associated with high mortality. Percutaneous alcohol septal ablation has recently been described as a therapeutic option in this setting. METHODS: Multicenter retrospective review of clinical outcomes of patients undergoing alcohol septal ablation to treat LVOT obstruction after TMVR for severe mitral stenosis with severe mitral annular calcification. RESULTS: Six patients underwent percutaneous alcohol septal ablation to treat LVOT obstruction post-TMVR at six different centers. Five patients had immediate significant reduction in LVOT obstruction with improvement in hemodynamic status while one had persistent LVOT gradient but hemodynamic instability improved. The first patient died on postoperative day 4 due to complete heart block. One patient had initial improvement in LVOT gradient with recurrence on postoperative day 1 thought to be secondary to septal edema, was treated with surgical removal of the transcatheter valve and resection of the anterior mitral leaflet followed by transatrial TMVR and died 3 weeks later due to multi-organ failure. The remaining four patients improved clinically after alcohol septal ablation, were discharged from the hospital and were clinically stable at 30-day follow-up. CONCLUSIONS: Percutaneous alcohol ablation provides acute relief of TMVR-induced LVOT obstruction when septal hypertrophy is a contributing factor. This may be a safer alternative to bail-out surgery in this extremely high-risk patient population. © 2017 Wiley Periodicals, Inc.

Improved dark blood imaging of the heart using radial balanced steady-state free precession.

BACKGROUND: Dark blood imaging of the heart is conventionally performed using a breath-hold, dual-inversion Cartesian fast spin-echo pulse sequence. Our aim was to develop a faster, more flexible approach that would be less motion-sensitive and provide better image quality. For this purpose, we implemented a prototype radial balanced steady-state free precession (bSSFP) pulse sequence. METHODS: The study was approved by the institutional review board. Six healthy volunteers and 27 subjects undergoing clinically-indicated cardiovascular magnetic resonance (CMR) were imaged using dark blood Cartesian fast spin-echo and radial bSSFP. For patient studies, overall image quality, fat suppression and blood nulling were scored on a 5-point Likert scale. The quality of visualization of the right and left ventricular free walls and septum were individually scored. Streaking and ghosting artifacts were noted, as well as signal dropout in the free wall of the left ventricle. RESULTS: In volunteer studies, radial bSSFP showed less degradation by cardiac or respiratory motion than fast spin-echo as indicated by visual analysis and calculation of the temporal signal-to-noise ratio. The least motion sensitivity and maximal imaging efficiency were achieved with a single-shot radial bSSFP acquisition using only 35 views (temporal resolution = 95 ms). In patient studies, radial bSSFP images showed fewer motion artifacts and were judged to provide better myocardial visibility, including depiction of the right ventricular free wall, than fast spin-echo. CONCLUSIONS: Dual-inversion radial bSSFP provides the benefits of diminished sensitivity to image artifacts from respiratory or cardiac motion, better myocardial visibility, and improved imaging efficiency compared with standard-of-care Cartesian fast spin-echo for dark blood imaging of the heart.

High-risk coronary plaque at coronary CT angiography is associated with nonalcoholic fatty liver disease, independent of coronary plaque and stenosis burden: results from the ROMICAT II trial.

PURPOSE: To determine the association between nonalcoholic fatty liver disease (NAFLD) and the presence of high-risk coronary atherosclerotic plaque as assessed with coronary computed tomographic (CT) angiography. MATERIALS AND METHODS: This study was approved by the local ethics committees; informed consent was obtained. Patients randomized to the coronary CT angiography arm of the Rule Out Myocardial Infarction using Computer Assisted Tomography, or ROMICAT, II trial who underwent both nonenhanced CT to assess calcium score and contrast material-enhanced coronary CT angiography were included. Readers assessed coronary CT angiography images for the presence of coronary plaque, significant stenosis (≥50%), and high-risk plaque features (positive remodeling, CT attenuation < 30 HU, napkin-ring sign, spotty calcium). NAFLD was defined as hepatic steatosis at nonenhanced CT (liver minus spleen CT attenuation < 1 HU) without evidence of clinical liver disease, liver cirrhosis, or alcohol abuse. To determine the association between high-risk plaque and NAFLD, univariable and multivariable logistic regression analyses were performed, with high-risk plaque as a dependent variable and NAFLD, traditional risk factors, and extent of coronary atherosclerosis as independent variables. RESULTS: Overall, 182 (40.9%) of 445 patients had CT evidence of NAFLD. High-risk plaque was more frequent in patients with NAFLD than in patients without NAFLD (59.3% vs 19.0%, respectively; P < .001). The association between NAFLD and high-risk plaque (odds ratio, 2.13; 95% confidence interval: 1.18, 3.85) persisted after adjusting for the extent and severity of coronary atherosclerosis and traditional risk factors. CONCLUSION: NAFLD is associated with advanced high-risk coronary plaque, independent of traditional cardiovascular risk factors and the extent and severity of coronary artery disease.

Guideline-Based Statin Eligibility, Coronary Artery Calcification, and Cardiovascular Events.

IMPORTANCE: The 2013 American College of Cardiology/American Heart Association (ACC/AHA) guidelines for cholesterol management defined new eligibility criteria for statin therapy. However, it is unclear whether this approach improves identification of adults at higher risk of cardiovascular events. OBJECTIVE: To determine whether the ACC/AHA guidelines improve identification of individuals who develop incident cardiovascular disease (CVD) and/or have coronary artery calcification (CAC) compared with the National Cholesterol Education Program's 2004 Updated Third Report of the Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults (ATP III) guidelines. DESIGN, SETTING, AND PARTICIPANTS: Longitudinal community-based cohort study, with participants for this investigation drawn from the offspring and third-generation cohorts of the Framingham Heart Study. Participants underwent multidetector computed tomography for CAC between 2002 and 2005 and were followed up for a median of 9.4 years for incident CVD. EXPOSURES: Statin eligibility was determined based on Framingham risk factors and low-density lipoprotein thresholds for ATP III, whereas the pooled cohort calculator was used for ACC/AHA. MAIN OUTCOMES AND MEASURES: The primary outcome was incident CVD (myocardial infarction, death due to coronary heart disease [CHD], or ischemic stroke). Secondary outcomes were CHD and CAC (as measured by the Agatston score). RESULTS: Among 2435 statin-naive participants (mean age, 51.3 [SD, 8.6] years; 56% female), 39% (941/2435) were statin eligible by ACC/AHA compared with 14% (348/2435) by ATP III (P < .001). There were 74 incident CVD events (40 nonfatal myocardial infarctions, 31 nonfatal ischemic strokes, and 3 fatal CHD events). Participants who were statin eligible by ACC/AHA had increased hazard ratios for incident CVD compared with those eligible by ATP III: 6.8 (95% CI, 3.8-11.9) vs 3.1 (95% CI, 1.9-5.0), respectively (P<.001). Similar results were seen for CVD in participants with intermediate Framingham Risk Scores and for CHD. Participants who were newly statin eligible (n = 593 [24%]) had an incident CVD rate of 5.7%, yielding a number needed to treat of 39 to 58. Participants with CAC were more likely to be statin eligible by ACC/AHA than by ATP III: CAC score >0 (n = 1015): 63% vs 23%; CAC score >100 (n = 376): 80% vs 32%; and CAC score >300 (n = 186): 85% vs 34% (all P < .001). A CAC score of 0 identified a low-risk group among ACC/AHA statin-eligible participants (306/941 [33%]) with a CVD rate of 1.6%. CONCLUSIONS AND RELEVANCE: In this community-based primary prevention cohort, the ACC/AHA guidelines for determining statin eligibility, compared with the ATP III, were associated with greater accuracy and efficiency in identifying increased risk of incident CVD and subclinical coronary artery disease, particularly in intermediate-risk participants.

Effect of the 2010 task force criteria on reclassification of cardiovascular magnetic resonance criteria for arrhythmogenic right ventricular cardiomyopathy.

BACKGROUND: We sought to evaluate the effect of application of the revised 2010 Task Force Criteria (TFC) on the prevalence of major and minor Cardiovascular Magnetic Resonance (CMR) criteria for Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) versus application of the original 1994 TFC. We also assessed the utility of MRI to identify alternative diagnoses for patients referred for ARVC evaluation. METHODS: 968 consecutive patients referred to our institution for CMR with clinical suspicion of ARVC from 1995 to 2010, were evaluated for the presence of major and minor CMR criteria per the 1994 and 2010 ARVC TFC. CMR criteria included right ventricle (RV) dilatation, reduced RV ejection fraction, RV aneurysm, or regional RV wall motion abnormalities. When quantitative measures of RV size and function were not available, and in whom abnormal size or function was reported, a repeat quantitative analysis by 2 qualified CMR physicians in consensus. RESULTS: Of 968 patients, 220 (22.7%) fulfilled either a major or a minor 1994 TFC, and 25 (2.6%) fulfilled any of the 2010 TFC criterion. Among patients meeting any 1994 criteria, only 25 (11.4%) met at least one 2010 criterion. All patients who fulfilled a 2010 criteria also satisfied at least one 1994 criterion. Per the 2010 TFC, 21 (2.2%) patients met major criteria and 4 (0.4%) patients fulfilled at least one minor criterion. Eight patients meeting 1994 minor criteria were reclassified as satisfying 2010 major criteria, while 4 patients fulfilling 1994 major criteria were reclassified to only minor or no criteria under the 2010 TFC.Eighty-nine (9.2%) patients had alternative cardiac diagnoses, including 43 (4.4%) with clinically significant potential ARVC mimics. These included cardiac sarcoidosis, RV volume overload conditions, and other cardiomyopathies. CONCLUSIONS: Application of the 2010 TFC resulted in reduction of total patients meeting any diagnostic CMR criteria for ARVC from 22.7% to 2.6% versus the 1994 TFC. CMR identified alternative cardiac diagnoses in 9.2% of patients, and 4.4% of the diagnoses were potential mimics of ARVC.

A systematic review and meta-analysis of left atrial strain in hypertrophic cardiomyopathy and its prognostic utility.

Despite advances in noninvasive imaging modalities to identify atrial fibrillation (AF) risk in Hypertrophic Cardiomyopathy (HCM), there is a paucity of evidence concerning the impact of low Left Atrial strain (LAS) on AF and major adverse cardiac events (MACE) incidence in these patients. This study investigated the diagnostic and prognostic significance of LAS in predicting AF and MACE in HCM. Findings revealed lower LA reservoir (MD: -11.79, 95% CI -14.83, -8.74; p<0.00001), booster (MD: -4.10, 95% CI -6.29, -1.91; p=0.0002), and conduit (MD: -7.52, 95% CI -9.39, -5.65; p<0.00001) strains in HCM patients versus healthy controls, and also indicated a significant association between low LA reservoir/conduit/booster strain and the development of new AF as well as MACE prevalence in HCM patients. The results from this study suggest the valuable role of LA strain in HCM and its utility in predicting the development of new AF and cardiac events in HCM patients.

Temporal trends and procedural safety of transcatheter mitral valve repair with mitraclip in patients with hypertrophic cardiomyopathy: Insights from the national inpatient sample.

Data on utilization and safety of mitral Transcatheter Edge-to-Edge Repair (TEER) among hypertrophic cardiomyopathy (HCM) patients is limited. Our study aimed to assess the national utilization, safety, and clinical outcomes of TEER procedures among HCM patients using a nationwide real-world cohort. HCM patients undergoing TEER hospitalizations between 2015-2020 were identified using ICD-10 (International Classification of Diseases, (ICD-10-CM/PCS). HCM-TEER and HCM No-TEER formed the two comparison groups. Demographic characteristics, baseline comorbidities, procedural complications, inpatient mortality, length of stay (LOS), and cost of hospitalization were compared between the propensity-matched cohorts. Numeric values of 10 or less were not reported per NIS data use agreements. A total of 39,625 weighted cases of TEER were identified from 2015-2020. Of the included patients, 335 patients had the HCM diagnosis. The median age of the HCM-TEER group was 74 (70-79) vs. 79 (72-85) for the no-TEER cohort. The TEER procedure was more frequently performed among Caucasians (86.57%) and females (53.73%). The TEER procedure among HCM patients had similar in-hospital mortality (Adjusted odds ratio: aOR 1.50, 95% CI [0.68-3.29]; p = 0.30) and net adverse cardiac events (NACE) (aOR 1.16, 95% CI [0.73-1.85]; p = 0.51). TEER among HCM was associated with higher odds of gastrointestinal/hematological (aOR 2.33, 95% CI [1.29-4.19]; p = 0.003) complications. However, the odds of cardiac complications (aOR 0.57, 95% CI [0.33-0.96]; p = 0.03) were not higher. The median length of stay was similar in both the groups (median: 2 vs. 2, p = 0.74), although TEER among HCM was associated with higher costs of hospitalization ($44729.36 vs. $40513.82, p < 0.01). TEER is a minimally invasive procedure and could be a safe option for symptomatic HCM patients with significant MR who are poor surgical candidates. Mitral TEER among HCM has been increasingly utilized in recent years in the United States more commonly in obstructive HCM and is associated with no difference in mortality and net adverse cardiac events but higher odds for gastrointestinal/hematological complications than non-HCM patients.

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.

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.

5-Year Prospective Evaluation of Mitral Valve-in-Valve, Valve-in-Ring, and Valve-in-MAC Outcomes: MITRAL Trial Final Results.

BACKGROUND: The MITRAL (Mitral Implantation of Transcatheter Valves) trial is the first prospective trial to evaluate the safety and feasibility of balloon-expandable aortic transcatheter heart valves in patients with failed surgical bioprostheses or annuloplasty rings and severe mitral annular calcification treated with mitral valve-in-valve (MViV), valve-in-ring (MViR), or valve-in-mitral annular calcification (ViMAC). OBJECTIVES: The aim of this study was to evaluate 5-year outcomes among these patients. METHODS: A multicenter prospective study was conducted among patients at high surgical risk at 13 U.S. sites. Patients underwent MViV (n = 30), MViR (n = 30), or ViMAC (n = 31) and were followed annually for 5 years. Kansas City Cardiomyopathy Questionnaire scores were obtained at baseline and follow-up visits. Echocardiograms were analyzed at independent core laboratories. RESULTS: A total of 91 patients underwent transcatheter mitral valve replacement (February 2015 to December 2017). The mean age was 74.3 ± 8.9 years. At 5-year follow-up, the lowest all-cause mortality was observed in the MViV group (21.4%), 94.7% of patients were in NYHA functional class I or II, and the mean mitral gradient was 6.6 ± 2.5 mm Hg. The MViR and ViMAC groups had higher all-cause mortality (65.5% and 67.9%), most survivors were in NYHA functional classes I and II (50% and 55.6%), and mean mitral gradients remained stable (5.8 ± 0.1 and 6.7 ± 2.5 mm Hg). Significant improvements in Kansas City Cardiomyopathy Questionnaire scores were observed when all 3 arms were pooled. CONCLUSIONS: MViV, MViR, and ViMAC procedures were associated with sustained improvement of heart failure symptoms and quality of life among survivors at 5 years. Transcatheter heart valve function remained stable in all 3 groups. Patients treated with MViV had excellent survival at 5 years, whereas survival was lower in the MViR and ViMAC groups, consistent with underlying disease severity. Patients with more residual mitral regurgitation had higher mortality.

A case series of desmoplakin cardiomyopathy: a mimic of viral myocarditis.

Background: Clinical features and imaging presentation of myocarditis can overlap with other inflammatory or arrhythmogenic cardiomyopathies. Desmoplakin (DSP) is an important structural cardiac protein. Mutations in the DSP gene are associated with a variant of arrhythmogenic right ventricular cardiomyopathy (ARVC). Interestingly, this distinct genetic cardiomyopathy can also present with a myocardial inflammation and fibrosis pattern that may mimic other forms of myocarditis including viral myocarditis, which can raise a clinical challenge. We report two cases of DSP cardiomyopathy, which were initially thought to represent coronavirus disease of 2019 (COVID-19) myocarditis. Case summary: First patient is a 21-your-old woman with no past medical history but family history of presumed 'viral myocarditis' and ventricular tachycardia in her brother. She presented with acute chest pain and elevated cardiac enzymes. She tested positive for COVID-19 and given the suspicion for possible COVID-19 related acute myocarditis, cardiac magnetic resonance imaging obtained and revealed regional wall motion abnormalities, several areas of subepicardial and pericardial late gadolinium enhancement (LGE). Ambulatory cardiac monitoring showed runs of non-sustained ventricular tachycardia and considering her family history of arrhythmogenic myocarditis, genetic testing was performed that was positive for a likely pathogenic heterozygous mutation of DSP gene. She declined the recommended implantable cardioverter defibrillator (ICD).Second patient is a 34-year-old physician with no significant past medical history who works at a COVID-19 unit and presented with syncope and was found to have ventricular tachycardia. Echocardiogram revealed severely dilated left ventricle and globally depressed systolic function with left ventricular ejection fraction of 20%. Coronary computed tomography angiography showed no evidence of coronary atherosclerosis. Cardiac magnetic resonance imaging revealed several areas of mid myocardial and pericardial LGE. Subcutaneous ICD was implanted and an endomyocardial biopsy had evidence of lymphocytic myocarditis and adipose tissue infiltration of the myocardium. Genetic testing revealed pathogenic heterozygous DSP mutation. He underwent epicardial ablation for the episodes of ventricular tachycardia despite medical therapy. He was able to return to work and has not had any further episodes of arrhythmia. Conclusion: Mutations in the DSP gene are associated with left dominant arrhythmogenic cardiomyopathy, which is a variant of ARVC. Beside left ventricular systolic dysfunction and ventricular tachyarrhythmias, carriers of these mutations may present with episodes of chest pain associated with elevated cardiac enzymes and cardiac imaging findings indistinguishable from other forms of acute myocarditis including viral myocarditis. Currently, there are no guidelines for diagnosis and treatment of this entity.

Impact of Tafamidis on Survival in a Real-World Community-Based Cohort.

Tafamidis is the only therapy shown to improve survival in transthyretin cardiac amyloidosis (ATTR) based on randomized controlled trial data. We sought to evaluate the impact of tafamidis on survival in a real-world community-based cohort. This was a prospective observational cohort study that included consecutive patients with confirmed ATTR based on biopsy or TcPYP imaging. Baseline characteristics were compared between patients taking tafamidis vs not, and Kaplan-Meier survival analysis was performed to compare survival between these groups. We examined the reasons that ATTR patients were not on tafamidis. Of 107 ATTR patients, median age was 83.9 years, 79% were men, and 63 (59%) of them were on tafamidis. Demographics and baseline cardiovascular risk factors did not differ significantly between those on vs off tafamidis, although there was a higher proportion of NYHA Class III or IV heart failure in those off tafamidis (76% vs 57%, P < 0.01). The most common reasons patients were not on tafamidis included delays in obtaining the drug or financial barriers (59%) and NYHA Class IV heart failure (19.5%). Patients taking tafamidis had a significantly higher median survival compared to those not on tafamidis (median survival 6.70 vs 1.43 years, P < 0.0001). Our study demonstrates significantly improved survival in ATTR patients taking tafamidis. Barriers exist to tafamidis initiation including delayed access and affordability, and efforts should be made to improve patient access.

Management of Patients With Severe Mitral Annular Calcification: JACC State-of-the-Art Review.

Mitral annular calcification (MAC) is a common and challenging pathologic condition, especially in the context of an aging society. Surgical mitral valve intervention in patients with MAC is difficult, with varying approaches to the calcified annular anatomy, and the advent of transcatheter valve interventions has provided additional treatment options. Advanced imaging provides the foundation for heart team discussions and management decisions concerning individual patients. This review focuses on the prognosis of, preoperative planning for, and management strategies for patients with MAC.

2-Year Outcomes of Transcatheter Mitral Valve Replacement in Patients With Annular Calcification, Rings, and Bioprostheses.

BACKGROUND: The MITRAL (Mitral Implantation of Transcatheter Valves) trial is the first prospective study for valve-in-mitral annular calcification (ViMAC), mitral valve-in-ring (MViR), and mitral valve-in-valve (MViV) using balloon-expandable aortic transcatheter heart valves. Procedural outcomes beyond 1 year are not well described. OBJECTIVES: This study evaluated 2-year outcomes in ViMAC, MViR, and MViV in the MITRAL trial. METHODS: This multicenter prospective study enrolled patients with severe MAC, prior failed mitral annuloplasty ring repair, or prior failed bioprosthetic MV replacement who were at high surgical risk at 13 U.S. sites. RESULTS: Between February 1, 2015, and December 31, 2017, 91 patients were enrolled (31 with ViMAC, 30 with MViR, and 30 with MViV). In the ViMAC group, 2-year all-cause mortality was 39.3%, 66.7% were New York Heart Association (NYHA) functional class I-II, and mean MV gradient was 5.6 ± 2.0 mm Hg. In the MViR group, 2-year all-cause mortality was 50%, 65% were NYHA functional class I-II, and mean MV gradient was 6.5 ± 2.7 mm Hg. In the MViV group, 2-year all-cause mortality was 6.7%, 85% were NYHA functional class I-II, and mean MV gradient was 6.9 ± 2.4 mm Hg. At 2 years, all patients had ≤mild mitral regurgitation and survivors in all 3 arms showed sustained improvement in Kansas City Cardiomyopathy Questionnaire scores compared to baseline. CONCLUSIONS: Use of balloon-expandable aortic transcatheter heart valves in selected patients with severe MAC, failed annuloplasty ring, and bioprosthetic MV dysfunction is associated with improvements in symptoms, quality of life, and stable prosthesis function at 2-year follow-up. Between 1 and 2 years, the MViR group experienced higher mortality rates than the MViV and ViMAC groups.