Mitral regurgitation assessment by cardiovascular magnetic resonance imaging during continuous in-scanner exercise: a feasibility study.

PURPOSE: Exercise imaging using current modalities can be challenging. This was patient focused study to establish the feasibility and reproducibility of exercise-cardiovascular magnetic resonance imaging (EX-CMR) acquired during continuous in-scanner exercise in asymptomatic patients with primary mitral regurgitation (MR). METHODS: This was a prospective, feasibility study. Biventricular volumes/function, aortic flow volume, MR volume (MR-Rvol) and regurgitant fraction (MR-RF) were assessed at rest and during low- (Low-EX) and moderate-intensity exercise (Mod-EX) in asymptomatic patients with primary MR. RESULTS: Twenty-five patients completed EX-CMR without complications. Whilst there were no significant changes in the left ventricular (LV) volumes, there was a significant increase in the LVEF (rest 63 ± 5% vs. Mod-EX 68 ± 6%;p = 0.01). There was a significant reduction in the right ventricular (RV) end-systolic volume (rest 68 ml(60-75) vs. Mod-EX 46 ml(39-59);p < 0.001) and a significant increase in the RV ejection fraction (rest 55 ± 5% vs. Mod-EX 65 ± 8%;p < 0.001). Whilst overall, there were no significant group changes in the MR-Rvol and MR-RF, individual responses were variable, with MR-Rvol increasing by ≥ 15 ml in 4(16%) patients and decreasing by ≥ 15 ml in 9(36%) of patients. The intra- and inter-observer reproducibility of LV volumes and aortic flow measurements were excellent, including at Mod-EX. CONCLUSION: EX-CMR is feasible and reproducible in patients with primary MR. During exercise, there is an increase in the LV and RV ejection fraction, reduction in the RV end-systolic volume and a variable response of MR-Rvol and MR-RF. Understanding the individual variability in MR-Rvol and MR-RF during physiological exercise may be clinically important.

Cardiac reverse remodeling in primary mitral regurgitation: mitral valve replacement vs. mitral valve repair.

BACKGROUND: When feasible, guidelines recommend mitral valve repair (MVr) over mitral valve replacement (MVR) to treat primary mitral regurgitation (MR), based upon historic outcome studies and transthoracic echocardiography (TTE) reverse remodeling studies. Cardiovascular magnetic resonance (CMR) offers reference standard biventricular assessment with superior MR quantification compared to TTE. Using serial CMR in primary MR patients, we aimed to investigate cardiac reverse remodeling and residual MR post-MVr vs MVR with chordal preservation. METHODS: 83 patients with ≥ moderate-severe MR on TTE were prospectively recruited. 6-min walk tests (6MWT) and CMR imaging including cine imaging, aortic/pulmonary through-plane phase contrast imaging, T1 maps and late-gadolinium-enhanced (LGE) imaging were performed at baseline and 6 months after mitral surgery or watchful waiting (control group). RESULTS: 72 patients completed follow-up (Controls = 20, MVr = 30 and MVR = 22). Surgical groups demonstrated comparable baseline cardiac indices and co-morbidities. At 6-months, MVr and MVR groups demonstrated comparable improvements in 6MWT distances (+ 57 ± 54 m vs + 64 ± 76 m respectively, p = 1), reduced indexed left ventricular end-diastolic volumes (LVEDVi; - 29 ± 21 ml/m2 vs - 37 ± 22 ml/m2 respectively, p = 0.584) and left atrial volumes (- 23 ± 30 ml/m2 and - 39 ± 26 ml/m2 respectively, p = 0.545). At 6-months, compared with controls, right ventricular ejection fraction was poorer post-MVr (47 ± 6.1% vs 53 ± 8.0% respectively, p = 0.01) compared to post-MVR (50 ± 5.7% vs 53 ± 8.0% respectively, p = 0.698). MVR resulted in lower residual MR-regurgitant fraction (RF) than MVr (12 ± 8.0% vs 21 ± 11% respectively, p = 0.022). Baseline and follow-up indices of diffuse and focal myocardial fibrosis (Native T1 relaxation times, extra-cellular volume and quantified LGE respectively) were comparable between groups. Stepwise multiple linear regression of indexed variables in the surgical groups demonstrated baseline indexed mitral regurgitant volume as the sole multivariate predictor of left ventricular (LV) end-diastolic reverse remodelling, baseline LVEDVi as the most significant independent multivariate predictor of follow-up LVEDVi, baseline indexed LV end-systolic volume as the sole multivariate predictor of follow-up LV ejection fraction and undergoing MVR (vs MVr) as the most significant (p < 0.001) baseline multivariate predictor of lower residual MR. CONCLUSION: In primary MR, MVR with chordal preservation may offer comparable cardiac reverse remodeling and functional benefits at 6-months when compared to MVr. Larger, multicenter CMR studies are required, which if the findings are confirmed could impact future surgical practice.

The Use and Efficacy of FFR-CT: Real-World Multicenter Audit of Clinical Data With Cost Analysis.

BACKGROUND: Fractional flow reserve-computed tomography (FFR-CT) is endorsed by UK and U.S. chest pain guidelines, but its clinical effectiveness and cost benefit in real-world practice are unknown. OBJECTIVES: The purpose of this study was to audit the use of FFR-CT in clinical practice against England's National Institute for Health and Care Excellence guidance and assess its diagnostic accuracy and cost. METHODS: A multicenter audit was undertaken covering the 3 years when FFR-CT was centrally funded in England. For coronary computed tomographic angiograms (CCTAs) submitted for FFR-CT analysis, centers provided data on symptoms, CCTA and FFR-CT findings, and subsequent management. Audit standards included using FFR-CT only in patients with stable chest pain and equivocal stenosis (50%-69%). Diagnostic accuracy was evaluated against invasive FFR, when performed. Follow-up for nonfatal myocardial infarction and all-cause mortality was undertaken. The cost of an FFR-CT strategy was compared to alternative stress imaging pathways using cost analysis modeling. RESULTS: A total of 2,298 CCTAs from 12 centers underwent FFR-CT analysis. Stable chest pain was the main symptom in 77%, and 40% had equivocal stenosis. Positive and negative predictive values of FFR-CT were 49% and 76%, respectively. A total of 46 events (2%) occurred over a mean follow-up period of 17 months; FFR-CT (cutoff: 0.80) was not predictive. The FFR-CT strategy costs £2,102 per patient compared with an average of £1,411 for stress imaging. CONCLUSIONS: In clinical practice, the National Institute for Health and Care Excellence criteria for using FFR-CT were met in three-fourths of patients for symptoms and 40% for stenosis. FFR-CT had a low positive predictive value, making its use potentially more expensive than conventional stress imaging strategies.

Atrial secondary mitral regurgitation: prevalence, characteristics, management, and long-term outcomes.

BACKGROUND: The prevalence, clinical characteristics, management and long-term outcomes of patients with atrial secondary mitral regurgitation (ASMR) are not well described. METHODS: We performed a retrospective, observational study of consecutive patients with grade III/IV MR determined by transthoracic echocardiography. The aetiology of MR was grouped as being either primary (due to degenerative mitral valve disease), ventricular SMR (VSMR: due to left ventricular dilatation/dysfunction), ASMR (due to LA dilatation), or other. RESULTS: A total of 388 individuals were identified who had grade III/IV MR; of whom 37 (9.5%) had ASMR, 113 (29.1%) had VSMR, 193 had primary MR (49.7%), and 45 (11.6%) were classified as having other causes. Compared to MR of other subtypes, patients with ASMR were on average older (median age 82 [74-87] years, p < 0.001), were more likely to be female (67.6%, p = 0.004) and usually had atrial fibrillation (83.8%, p = 0.001). All-cause mortality was highest in patients with ASMR (p < 0.001), but similar to that in patients with VSMR once adjusted for age and sex (hazard ratio [HR] 0.81, 95% confidence interval [CI] 0.52-1.25). Hospitalisation for worsening heart failure was more commonly observed in those with ASMR or VSMR (p < 0.001) although was similar between these groups when age and sex were accounted for (HR 0.74, 95% CI 0.34-1.58). For patients with ASMR, the only variables associated with outcomes were age and co-morbidities. CONCLUSIONS: ASMR is a prevalent and distinct disease process associated with a poor prognosis, with much of this related to older age and co-morbidities.

A case report of primary meningococcal pericarditis secondary to Neisseria meningitidis in a young female patient.

Pericarditis is responsible for approximately 5 % of emergency admissions due to chest pain. Pericarditis secondary to Neisseria meningitidis (meningococci) was originally reported in 1918, and remains a rare diagnosis. We report a case of primary meningococcal pericarditis presenting with non-specific symptoms, illustrating the importance of considering rarer causes of pericardial effusion. A previously fit and well 23-year-old female presented to her local hospital with a 2-day history of feeling generally unwell with myalgia and fevers and was initially discharged. Four days following discharge the patient re-presented with worsening symptoms. A Computed Tomography Pulmonary Angiogram (CTPA) demonstrated a large pericardial effusion with subsequent bedside echocardiogram confirming a global pericardial effusion of up to 3 cm. This required drainage, with blood cultures and pericardial fluid showing polymerase chain reaction positivity for Neisseria meningitidis, serogroup B. Our report describes a rare case of Primary Meningococcal Pericarditis secondary to serotype B meningococcal infection. The European Society of Cardiology propose criteria that warrant hospital admission and an aetiology search for certain patients with pericardial disease. These criteria provide a useful framework to help select those minority of patients in whom a more serious underlying cause is present. Blood cultures provide vital information to allow us to complete a thorough aetiological search and empirical antibiotics can cloud the clinical picture, making it harder to identify causative organisms. To aid the early administration of appropriate therapy, it may be pertinent to recommend a low threshold for taking blood cultures in patients with pyrexia and pericarditis or pericardial effusion.

An enormous right atrial myxoma highlights the role of echocardiography in heart failure pathways: a case report.

BACKGROUND: Myxomata are rare, benign, primary tumours of the heart which can present with a variety of symptoms depending on size, location, and mobility. Here, we report a case of enormous right atrial myxoma, obliterating the right atrial and right ventricular cavities presenting with symptoms of heart failure. CASE SUMMARY: A 66-year-old Caucasian female presented to primary care with symptoms of right heart failure and was found to have elevated N-terminal pro B-type natriuretic peptide of 2829 ng/L (normal value <125 ng/L). The patient was referred for urgent evaluation to the integrated heart failure service at our institution. Echocardiography revealed an enormous mobile mass attached to the right atrial septum, extending into the right ventricle and inferior vena cava measuring 90 × 42 mm. The patient underwent urgent surgical resection. Perioperative transoesophageal echocardiography demonstrated severe tricuspid regurgitation, which was treated with tricuspid annuloplasty ring. The patient made an uneventful recovery and was discharged. Subsequent imaging showed a reduction in right ventricular dimensions and improved systolic function. DISCUSSION: This case serves to remind us of the critical role of echocardiography in the diagnosis and management of people with breathlessness and raised natriuretic peptides. Therapies for heart failure are guided by ejection fraction, therefore timely and accurate diagnosis is critical. Moreover, as in this case, echocardiography can also identify other features of critical relevance to patient care.

Cardiovascular outcomes in systemic sclerosis with abnormal cardiovascular MRI and serum cardiac biomarkers.

OBJECTIVES: To explore the prognostic value of subclinical cardiovascular (CV) imaging measures and serum cardiac biomarkers in systemic sclerosis (SSc) for the development of CV outcomes of primary heart involvement (pHI). METHODS: Patients with SSc with no clinical SSc-pHI and no history of heart disease underwent cardiovascular magnetic resonance (CMR) imaging, and measurement of serum high-sensitivity-troponin I (hs-TnI) and N-terminal-pro-brain natriuretic peptide (NT-proBNP). Follow-up clinical and CV outcome data were recorded. CV outcomes were defined as myocarditis, arrhythmia and/or echocardiographic functional impairment including systolic dysfunction and/or diastolic dysfunction. RESULTS: Seventy-four patients with a median (IQR) age of 57 (49, 63) years, 32% diffuse cutaneous SSc, 39% interstitial lung disease, 30% Scl70+ were followed up for median (IQR) 22 (15, 54) months. Ten patients developed CV outcomes, comprising one patient with myocarditis and systolic dysfunction and nine arrhythmias: three non-sustained ventricular tachycardia and six supraventricular arrhythmias. The probability of CV outcomes was considerably higher in those with NT-proBNP >125 pg/mL versus normal NT-proBNP (X2=4.47, p=0.035). Trend for poorer time-to-event was noted in those with higher extracellular volume (ECV; indicating diffuse fibrosis) and hs-TnI levels versus those with normal values (X2=2.659, p=0.103; X2=2.530, p=0.112, respectively). In a predictive model, NT-proBNP >125 pg/mL associated with CV outcomes (OR=5.335, p=0.040), with a trend observed for ECV >29% (OR=4.347, p=0.073). CONCLUSION: These data indicate standard serum cardiac biomarkers (notably NT-proBNP) and CMR indices of myocardial fibrosis associate with adverse CV outcomes in SSc. This forms the basis to develop a prognostic model in larger, longitudinal studies.

A 30-Year-Old Man With Primary Cardiac Angiosarcoma.

A previously fit and well 30-year-old man presented with palpitations, fever, and pleuritic chest pain. Multimodality imaging and histopathology confirmed the diagnosis of primary cardiac angiosarcoma. We present the details of the presentation, diagnostic process using multimodality imaging, and clinical management. (Level of Difficulty: Beginner.).

Outpatient management of heart valve disease following the COVID-19 pandemic: implications for present and future care.

The established processes for ensuring safe outpatient surveillance of patients with known heart valve disease (HVD), echocardiography for patients referred with new murmurs and timely delivery of surgical or transcatheter treatment for patients with severe disease have all been significantly impacted by the novel coronavirus pandemic. This has created a large backlog of work and upstaging of disease with consequent increases in risk and cost of treatment and potential for worse long-term outcomes. As countries emerge from lockdown but with COVID-19 endemic in society, precautions remain that restrict 'normal' practice. In this article, we propose a methodology for restructuring services for patients with HVD and provide recommendations pertaining to frequency of follow-up and use of echocardiography at present. It will be almost impossible to practice exactly as we did prior to the pandemic; thus, it is essential to prioritise patients with the greatest clinical need, such as those with symptomatic severe HVD. Local procedural waiting times will need to be considered, in addition to usual clinical characteristics in determining whether patients requiring intervention would be better suited having surgical or transcatheter treatment. We present guidance on the identification of stable patients with HVD that could have follow-up deferred safely and suggest certain patients that could be discharged from follow-up if waiting lists are triaged with appropriate clinical input. Finally, we propose that novel models of working enforced by the pandemic-such as increased use of virtual clinics-should be further developed and evaluated.

Trans-catheter aortic valve implantation without contrast using the Lotus mechanically-expanded heart valve.

Trans-catheter aortic valve implantation (TAVI) has become an established treatment for inoperable and high-surgical risk patients with severe, symptomatic aortic stenosis (AS). Post-procedural acute kidney injury (AKI) is a frequent complication following TAVI and is associated with increased mortality. Patients with pre-existing chronic renal impairment are at particularly high risk. The etiology of post-TAVI AKI is multi-factorial, but the principal procedural issues are contrast-induced nephropathy, and renal hypoperfusion secondary to intra-procedural hypotension. We report a case of a TAVI in an 80-year-old patient with severe AS and significant chronic kidney disease (CKD), which was carried out without the use of contrast and with minimal procedural hypotension. Pre-procedural imaging was carried out using 3D trans-esophageal echocardiography (TEE) rather than computed tomography (CT) to avoid contrast administration. The Lotus valve (Boston Scientific, Marlborough, MA, USA) was chosen due to a number of design features which minimize both the need for contrast injection and procedural hypotension during valve positioning and deployment. The procedure was carried out successfully and produced an excellent result with no decline in renal function. We believe that the approach of using TEE and the mechanically-expanded Lotus valve illustrates an important therapeutic approach in patients with severe CKD. .

Multimodality imaging for the quantitative assessment of mitral regurgitation.

The natural history of mitral regurgitation (MR) results in significant morbidity and mortality. Innovations in non-invasive imaging have provided new insights into the pathophysiology and quantification of MR, in addition to early detection of left ventricular (LV) dysfunction and prognostic assessment in asymptomatic patients. Transthoracic (TTE) and transesophageal (TOE) echocardiography are the mainstay for diagnosis, assessment and serial surveillance. However, the advance from 2D to 3D imaging leads to improved assessment and characterization of mitral valve (MV) disease. Cardiovascular magnetic resonance (CMR) is increasingly used for MR quantitation and can provide an alternative imaging method if echocardiography is suboptimal or inconclusive. Other techniques such as exercise echocardiography, tissue Doppler imaging and speckle-tracking echocardiography can further offer complementary information on prognosis. This review summarises the current evidence for state-of-the-art cardiovascular imaging for the investigation of MR. Whilst advanced echocardiographic techniques are superior in the evaluation of complex MV anatomy, CMR appears the most accurate technique for the quantification of MR severity. Integration of multimodality imaging for the assessment of MR utilises the advantages of each imaging technique and offers the most comprehensive assessment of MR.

Consensus best practice pathway of the UK Systemic Sclerosis Study group: management of cardiac disease in systemic sclerosis.

Objective: Cardiac disease in SSc can manifest in various ways and is associated with a poor prognosis. There is little evidence on how best to detect and manage cardiac disease in SSc. Our objective was to produce an expert consensus best practice pathway for the management of cardiac disease in SSc. Methods: The UK Systemic Sclerosis Study Group set up several working groups to develop a number of consensus best practice pathways for the management of SSc-specific complications, including cardiac disease. A multidisciplinary task force was convened. The guidelines were partly informed by a comprehensive literature review. Results: A best practice pathway for cardiac disease (with a focus on primary cardiac disease) in SSc is presented, including approaches for early detection and standard pharmacological and device therapies. Due to the benefits, shared care and a multidisciplinary approach are recommended. A future research agenda has been formulated in response to the relative lack of understanding of the natural history of primary cardiac disease that was highlighted by the initiative. Conclusion: The physician should be alert to the possibility of cardiac disease in SSc; it is best managed within a multidisciplinary team including both rheumatologists and cardiologists. This pathway provides a reference for all physicians managing patients with SSc.

Severe aortic stenosis, critical coronary artery disease, and transfusion-dependent angiodysplasia - A management conundrum.

The combination of severe aortic stenosis, bleeding due to Heyde's syndrome and critical coronary artery disease presents a management challenge. We report the case of an 86-year-old who underwent percutaneous coronary intervention to the right coronary artery, simultaneous balloon aortic valvuloplasty to treat aortic stenosis and address bleeding from Heyde's syndrome, and subsequent staged transcatheter aortic valve implantation. .

Recurrent ischaemic mitral regurgitation post mitral annuloplasty due to suture dehiscence evaluated using real time three dimensional transoesophageal echocardiography.

Ischaemic mitral regurgitation after myocardial infarction results from geometric changes in left ventricular shape and displacement of papillary muscles with resultant tethering and incomplete leaflet coaptation of mitral leaflets. Post mitral valve repair, both valve apparatus related factors such as persistent leaflet tethering and progressive left ventricular adverse remodelling and procedure related factors such as ring dehiscence are important causes of recurrent mitral regurgitation after initial undersized mitral ring annuloplasty. Three-dimensional echocardiography is a novel clinical tool that has the potential to provide additional anatomical and functional information regarding the mechanism of recurrent mitral regurgitation post mitral valve repair that is complementary to standard two dimensional transoesophageal echocardiography thus helping guide the most appropriate subsequent therapeutic intervention.

Real-time 3D transesophageal echocardiography for the evaluation of rheumatic mitral stenosis.

OBJECTIVES: The aims of this study were: 1) to assess the feasibility and reliability of performing mitral valve area (MVA) measurements in patients with rheumatic mitral valve stenosis (RhMS) using real-time 3-dimensional transesophageal echocardiography (3DTEE) planimetry (MVA(3D)); 2) to compare MVA(3D) with conventional techniques: 2-dimensional (2D) planimetry (MVA(2D)), pressure half-time (MVA(PHT)), and continuity equation (MVA(CON)); and 3) to evaluate the degree of mitral commissural fusion. BACKGROUND: 3DTEE is a novel technique that provides excellent image quality of the mitral valve. Real-time 3DTEE is a relatively recent enhancement of this technique. To date, there have been no feasibility studies investigating the utility of real-time 3DTEE in the assessment of RhMS. METHODS: Forty-three consecutive patients referred for echocardiographic evaluation of RhMS and suitability for percutaneous mitral valvuloplasty were assessed using 2D transthoracic echocardiography and real-time 3DTEE. MVA(3D), MVA(2D), MVA(PHT), MVA(CON), and the degree of commissural fusion were evaluated. RESULTS: MVA(3D) assessment was possible in 41 patients (95%). MVA(3D) measurements were significantly lower compared with MVA(2D) (mean difference: -0.16 ± 0.22; n=25, p<0.005) and MVA(PHT) (mean difference: -0.23 ± 0.28 cm(2); n=39, p<0.0001) but marginally greater than MVA(CON) (mean difference: 0.05 ± 0.22 cm(2); n=24, p=0.82). MVA(3D) demonstrated best agreement with MVA(CON) (intraclass correlation coefficient [ICC] 0.83), followed by MVA(2D) (ICC 0.79) and MVA(PHT) (ICC 0.58). Interobserver and intraobserver agreement was excellent for MVA(3D), with ICCs of 0.93 and 0.96, respectively. Excellent commissural evaluation was possible in all patients using 3DTEE. Compared with 3DTEE, underestimation of the degree of commissural fusion using 2D transthoracic echocardiography was observed in 19%, with weak agreement between methods (κ<0.4). CONCLUSIONS: MVA planimetry is feasible in the majority of patients with RhMS using 3DTEE, with excellent reproducibility, and compares favorably with established methods. Three-dimensional transesophageal echocardiography allows excellent assessment of commissural fusion.

Prognostic indicators in patients presenting with acute cardiogenic pulmonary edema treated with CPAP: it's not the acid that matters, it's back to basics.

Several prognostic markers have been identified for patients admitted with acute cardiogenic pulmonary edema. Most of the markers are based on clinical risk scores. Unlike hypercapnic respiratory failure, acidosis is not an adverse predictor in these patients. Hemodynamic variables that assess pathophysiological mechanisms may be more helpful to guide appropriate management.