A novel cardiovascular magnetic resonance risk score for predicting mortality following surgical aortic valve replacement.

The increasing prevalence of patients with aortic stenosis worldwide highlights a clinical need for improved and accurate prediction of clinical outcomes following surgery. We investigated patient demographic and cardiovascular magnetic resonance (CMR) characteristics to formulate a dedicated risk score estimating long-term survival following surgery. We recruited consecutive patients undergoing CMR with gadolinium administration prior to surgical aortic valve replacement from 2003 to 2016 in two UK centres. The outcome was overall mortality. A total of 250 patients were included (68 ± 12 years, male 185 (60%), with pre-operative mean aortic valve area 0.93 ± 0.32cm2, LVEF 62 ± 17%) and followed for 6.0 ± 3.3 years. Sixty-one deaths occurred, with 10-year mortality of 23.6%. Multivariable analysis showed that increasing age (HR 1.04, P = 0.005), use of antiplatelet therapy (HR 0.54, P = 0.027), presence of infarction or midwall late gadolinium enhancement (HR 1.52 and HR 2.14 respectively, combined P = 0.12), higher indexed left ventricular stroke volume (HR 0.98, P = 0.043) and higher left atrial ejection fraction (HR 0.98, P = 0.083) associated with mortality and developed a risk score with good discrimination. This is the first dedicated risk prediction score for patients with aortic stenosis undergoing surgical aortic valve replacement providing an individualised estimate for overall mortality. This model can help clinicians individualising medical and surgical care.Trial Registration ClinicalTrials.gov Identifier: NCT00930735 and ClinicalTrials.gov Identifier: NCT01755936.

Corrigendum to: A case of pseudo-pseudo Meigs' syndrome.

[This corrects the article DOI: 10.1093/omcr/omy136.][This corrects the article DOI: 10.1093/omcr/omy136.].

A case of pseudo-pseudo Meigs' syndrome.

Here we report a case of a patient with systemic lupus erythematosus presenting with pseudo-pseudo Meigs' syndrome (PPMS): a triad of pleural effusion, ascites and raised CA-125. There have only been nine other cases reported in the literature. To our knowledge, this is the first to have an oesophago-gastro-duodenoscopy and liver biopsy as part of the diagnostic work up. Its mechanism of action is not yet fully understood but PPMS is a treatable condition that is responsive to immunosuppression. It is therefore important to consider it in patients presenting like this, where alternative diagnoses, including malignancy, have been ruled out.

Osteoprotegerin and Myocardial Fibrosis in Patients with Aortic Stenosis.

Left ventricular myocardial fibrosis in patients with aortic stenosis (AS) confers worse prognosis. Plasma osteoprotegerin (OPG), a cytokine from the TNF receptor family, correlates with the degree of valve calcification in AS, reflecting the activity of the tissue RANKL/RANK/OPG (receptor activator of nuclear factor κΒ ligand/RANK/osteoprotegerin) axis, and is associated with poorer outcomes in AS. Its association with myocardial fibrosis is unknown. We hypothesised that OPG levels would reflect the extent of myocardial fibrosis in AS. We included 110 consecutive patients with AS who had undergone late-gadolinium contrast enhanced cardiovascular magnetic resonance (LGE-CMR). Patients were characterised according to pattern of fibrosis (no fibrosis, midwall fibrosis, or chronic myocardial infarction fibrosis). Serum OPG was measured with ELISA and compared between groups defined by valve stenosis severity. Some 36 patients had no fibrosis, 38 had midwall fibrosis, and 36 had chronic infarction. Patients with midwall fibrosis did not have higher levels of OPG compared to those without fibrosis (6.78 vs. 5.25 pmol/L, p = 0.12). There was no difference between those with midwall or chronic myocardial infarction fibrosis (6.78 vs. 6.97 pmol/L, p = 0.27). However, OPG levels in patients with chronic myocardial infarction fibrosis were significantly higher than those without fibrosis (p = 0.005).

Myocardial Scar and Mortality in Severe Aortic Stenosis.

BACKGROUND: Aortic valve replacement (AVR) for aortic stenosis is timed primarily on the development of symptoms, but late surgery can result in irreversible myocardial dysfunction and additional risk. The aim of this study was to determine whether the presence of focal myocardial scar preoperatively was associated with long-term mortality. METHODS: In a longitudinal observational outcome study, survival analysis was performed in patients with severe aortic stenosis listed for valve intervention at 6 UK cardiothoracic centers. Patients underwent preprocedural echocardiography (for valve severity assessment) and cardiovascular magnetic resonance for ventricular volumes, function and scar quantification between January 2003 and May 2015. Myocardial scar was categorized into 3 patterns (none, infarct, or noninfarct patterns) and quantified with the full width at half-maximum method as percentage of the left ventricle. All-cause mortality and cardiovascular mortality were tracked for a minimum of 2 years. RESULTS: Six hundred seventy-four patients with severe aortic stenosis (age, 75±14 years; 63% male; aortic valve area, 0.38±0.14 cm2/m2; mean gradient, 46±18 mm Hg; left ventricular ejection fraction, 61.0±16.7%) were included. Scar was present in 51% (18% infarct pattern, 33% noninfarct). Management was surgical AVR (n=399) or transcatheter AVR (n=275). During follow-up (median, 3.6 years), 145 patients (21.5%) died (52 after surgical AVR, 93 after transcatheter AVR). In multivariable analysis, the factors independently associated with all-cause mortality were age (hazard ratio [HR], 1.50; 95% CI, 1.11-2.04; P=0.009, scaled by epochs of 10 years), Society of Thoracic Surgeons score (HR, 1.12; 95% CI, 1.03-1.22; P=0.007), and scar presence (HR, 2.39; 95% CI, 1.40-4.05; P=0.001). Scar independently predicted all-cause (26.4% versus 12.9%; P<0.001) and cardiovascular (15.0% versus 4.8%; P<0.001) mortality, regardless of intervention (transcatheter AVR, P=0.002; surgical AVR, P=0.026 [all-cause mortality]). Every 1% increase in left ventricular myocardial scar burden was associated with 11% higher all-cause mortality hazard (HR, 1.11; 95% CI, 1.05-1.17; P<0.001) and 8% higher cardiovascular mortality hazard (HR, 1.08; 95% CI, 1.01-1.17; P<0.001). CONCLUSIONS: In patients with severe aortic stenosis, late gadolinium enhancement on cardiovascular magnetic resonance was independently associated with mortality; its presence was associated with a 2-fold higher late mortality.

A case of Takotsubo cardiomyopathy after chemotherapy.

Here we present the case of a patient with diffuse large B-cell lymphoma who was admitted to hospital for an elective autologous peripheral blood stem cell transplant after cytotoxic treatment with lomustine, cytarabine, cyclophosphomide and etoposide (LACE). On the final day of chemotherapeutic treatment, she developed sudden onset dyspnoea. Electrocardiography confirmed acute antero-lateral T-wave inversion. She went onto have coronary angiography that demonstrated unobstructed coronary arteries. Left ventriculography demonstrated apical ballooning, consistent with Takotsubo (stress) cardiomyopathy. The link between chemotherapy and Takotsubo cardiomyopathy has become increasingly recognized in recent years, although causality remains to be established and the mechanism of action is not yet fully understood.

18F-sodium fluoride uptake is a marker of active calcification and disease progression in patients with aortic stenosis.

BACKGROUND: 18F-Sodium fluoride (18F-NaF) and 18F-fluorodeoxyglucose (18F-FDG) are promising novel biomarkers of disease activity in aortic stenosis. We compared 18F-NaF and 18F-FDG uptake with histological characterization of the aortic valve and assessed whether they predicted disease progression. METHODS AND RESULTS: Thirty patients with aortic stenosis underwent combined positron emission and computed tomography using 18F-NaF and 18F-FDG radiotracers. In 12 patients undergoing aortic valve replacement surgery (10 for each tracer), radiotracer uptake (mean tissue/ BACKGROUND: =0.65; P=0.04) and osteocalcin (r=0.68; P=0.03) immunohistochemistry. There was no significant correlation between 18F-FDG uptake and CD68 staining (r=-0.43; P=0.22). After 1 year, aortic valve calcification increased from 314 (193-540) to 365 (207-934) AU (P<0.01). Baseline 18F-NaF uptake correlated closely with the change in calcium score (r=0.66; P<0.01), and this improved further (r=0.75; P<0.01) when 18F-NaF uptake overlying computed tomography-defined macrocalcification was excluded. No significant correlation was noted between valvular 18F-FDG uptake and change in calcium score (r=-0.11; P=0.66). CONCLUSIONS: 18F-NaF uptake identifies active tissue calcification and predicts disease progression in patients with calcific aortic stenosis. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT01358513.

Anterior shoulder instability associated with coracoid nonunion in patients with a seizure disorder.

BACKGROUND: An association between coracoid fracture and glenohumeral instability with both a seizure disorder and the subsequent development of coracoid nonunion has not previously been recognized. This is clinically important as patients with a seizure disorder and glenohumeral instability frequently require a primary osseous reconstructive procedure, such as coracoid osteotomy and transfer to the anterior glenoid rim (the Bristow-Latarjet procedure), to address glenoid osseous deficiency. We report on coracoid fracture nonunion in five patients with a seizure disorder and anterior glenohumeral instability and discuss the implications for surgical treatment. METHODS: Coracoid fracture was specifically sought on three-dimensional reconstructions of computed tomography scans in a consecutive series of 234 patients presenting to our unit with recurrent anterior instability. In addition to demographic data, we specifically sought information on any history of shoulder injury, the mechanism of injury, or previous seizure activity in these patients. In patients with a coracoid fracture or nonunion viewed to be at high risk of failure with a soft-tissue procedure, an open osseous reconstructive procedure was performed. The type of operative procedure was determined by the location of the nonunion. RESULTS: We identified six coracoid fracture nonunions in association with anterior glenohumeral instability in five patients (mean age, 26.8 years; range, twenty-four to thirty years). All patients had instability occurring in association with seizures. In the four shoulders with the anatomic location of the coracoid nonunion at its so-called elbow, a standard Bristow-Latarjet procedure was performed. In the two shoulders in which the nonunion was more distal, an Eden-Hybbinette procedure was performed. CONCLUSIONS: We recommend having a high index of suspicion of coracoid fracture when treating patients with a seizure disorder who have anterior glenohumeral instability. In these patients, preoperative computed tomographic images allow the diagnosis of a coracoid nonunion to be made prior to surgery and help to determine whether there is sufficient intact coracoid bone to allow a Bristow-Latarjet procedure to be performed.