Cardiac metastases from neuroendocrine neoplasms: complementary role of SSTR PET/CT and cardiac MRI.

BACKGROUND: Cardiac metastases from neuroendocrine neoplasms (NENs) are being detected with increasing frequency, although the optimal imaging strategy remains unclear. We performed a single-center retrospective study to explore the role of somatostatin receptor positron emission tomography/computed tomography (SSTR PET/CT) and cardiac magnetic resonance imaging (CMR) in NEN cardiac metastases, determine the degree of concordance between the findings of these imaging modalities, and examine the advantages and disadvantages of each imaging technique. A secondary aim was to determine if cardiac metastases were associated with adverse cardiac events during peptide receptor radionuclide therapy (PRRT). METHODS AND RESULTS: 19 patients with NEN cardiac metastases were identified. A retrospective review of electronic medical records was performed, and if available SSTR PET/CT and CMR were blindly re-reviewed by imaging specialists, documenting the number and location of cardiac metastases. All 19 patients had SSTR PET/CT, and 10/19 patients had CMR. SSTR PET/CT identified more metastases than CMR. When identified on CMR, metastases were more accurately localized. 12/19 patients received PRRT, with no cardiac adverse effects. CONCLUSION: SSTR PET/CT and CMR are complementary investigations in the imaging of NEN cardiac metastases. SSTR PET/CT appears more sensitive for lesion detection, and CMR offers better lesion characterization. Both investigations present useful information for the planning of treatment including PRRT, which was administered safely.

Clinical characteristics of Aboriginal and Torres Strait Islander emergency department patients with suspected acute coronary syndrome.

OBJECTIVES: To describe the demographics, presentation characteristics, clinical features and cardiac outcomes for Aboriginal and Torres Strait Islander patients who present to a regional cardiac referral centre ED with suspected acute coronary syndrome (ACS). METHODS: This was a single-centre observational study conducted at a regional referral hospital in Far North Queensland, Australia from November 2017 to September 2018 and January 2019 to December 2019. Study participants were 278 Aboriginal and Torres Strait Islander people presenting to an ED and investigated for suspected ACS. The main outcome measure was the proportion of patients with ACS at index presentation and differences in characteristics between those with and without ACS. RESULTS: ACS at presentation was diagnosed in 38.1% of patients (n = 106). The mean age of patients with ACS was 53.5 years (SD 9.5) compared with 48.7 years (SD 12.1) in those without ACS (P = 0.001). Patients with ACS were more likely to be male (63.2% vs 39.0%, P < 0.001), smokers (70.6% vs 52.3%, P = 0.002), have diabetes (56.6% vs 38.4%, P = 0.003) and have renal impairment (24.5% vs 10.5%, P = 0.002). CONCLUSIONS: Aboriginal and Torres Strait Islander patients with suspected ACS have a high burden of traditional cardiac risk factors, regardless of whether they are eventually diagnosed with ACS. These patients may benefit from assessment for coronary artery disease regardless of age at presentation.

Emergency Department Assessment of Suspected Acute Coronary Syndrome Using the IMPACT Pathway in Aboriginal and Torres Strait Islander People.

OBJECTIVES: The Improved Assessment of Chest pain Trial (IMPACT) pathway is an accelerated strategy for the assessment of emergency patients presenting with suspected acute coronary syndrome (ACS). The objective of this study was to report outcomes for Aboriginal and Torres Strait Islander patients deemed low-, intermediate-, or high-risk according to this pathway. DESIGN: This was a prospective observational trial conducted between November 2017 and December 2019. SETTING: Regional hospital in Far North Queensland. PARTICIPANTS: Aboriginal and Torres Strait Islander people presenting to the Emergency Department with suspected ACS were asked to participate. Participants were stratified as low-, intermediate- or high-risk of ACS according to the IMPACT pathway. High-and intermediate risk patients were managed according to the IMPACT pathway. Management of low-risk patients included additional inpatient cardiac testing, which was not part of the original IMPACT pathway. MAIN OUTCOME MEASURES: The primary outcome was acute coronary syndrome within 30-days. Secondary outcomes included length of stay and prevalence of objective testing. RESULTS: A total of 155 participants were classified as either at low-risk (n=18 11.6%), intermediate-risk (n=87 56.1%), or high-risk (n=50 32.3%) of ACS. Thirty-day (30-day) ACS occurred in 29 (18.6%) patients, which included 26 (52.0%) high-risk patients and three (3.4%) intermediate-risk patients. No patients in the low-risk group were diagnosed with ACS during their index presentation or by 30-days. Median hospital length-of-stay was 11.9 hours (interquartile range [IQR] 5.3-20.2 hrs) for low- and 15.5 hours (IQR 5.9-29.2 hrs) for intermediate-risk patients. CONCLUSION: The IMPACT pathway, which has been associated with reduced LOS in other settings, could be safely implemented for patients of Aboriginal and Torres Strait Islander origin, classifying two-thirds as low- or intermediate risk. However, a clinically significant proportion of Aboriginal and Torres Strait Islander patients experience cardiac events, which supports the need to provide early objective testing for coronary artery disease.

Widespread Introduction of a High-Sensitivity Troponin Assay: Assessing the Impact on Patients and Health Services.

Adoption of High-sensitivity troponin (hs-cTn) assays by hospitals worldwide is increasing. We sought to determine the effects of a simultaneous state-wide hs-cTn assay introduction on the implementing health service. A quasi-experimental pre-post design was used. Participants included all adult patients presenting to 21 Australian hospitals who had troponin testing commenced within the Emergency Department (ED). Data were collected for 124,357 episodes of care between 30 April 2018 and 23 April 2019; six months pre- and six months post-implementation of the assay. The primary outcome was hospital length of stay (LOS). Secondary outcomes included ED LOS, 90-day cardiovascular mortality, elevated troponin, diagnosis of acute myocardial infarction (AMI), admission to a cardiology ward, invasive cardiac procedures, and total hospital costs. Following hs-cTn implementation, there was a 1.9-h (95% CI: -2.9 to -1.0 h) reduction in overall LOS. This equated to a cost saving of over 9 million Australian dollars per year. There was no increase in diagnosis of AMI, invasive cardiac procedures or ward admissions. The use of hs-cTn assays facilitates important benefits for health services by enabling more rapid evaluation protocols within the ED. This benefit may be considerable given the large cohort of emergency patients with possible ACS.

Hypertrophic Cardiomyopathy: Challenging the Status Quo?

Hypertrophic cardiomyopathy (HCM) is the most common cardiovascular genetic disorder. While our mechanistic understanding has been informed by elegant gene discovery studies that led to the term "disease of the sarcomere", more recent investigations have challenged the single-gene hypothesis. Multimodality imaging has allowed better phenotyping to facilitate early diagnosis, identify treatable phenocopies, and guide management. While HCM remains an important cause of sudden death, recent studies have reported a substantial cumulative burden of heart failure and atrial fibrillation in middle-aged and older individuals. Nonetheless, improvements in risk stratification have allowed early intervention to transition HCM from being a common cause of sudden death in the young to a treatable chronic disease.

Implementing change: evaluating the Accelerated Chest pain Risk Evaluation (ACRE) project.

OBJECTIVE: To evaluate hospital length of stay (LOS) and admission rates before and after implementation of an evidence-based, accelerated diagnostic protocol (ADP) for patients presenting to emergency departments (EDs) with chest pain. DESIGN: Quasi-experimental design, with interrupted time series analysis for the period October 2013 - November 2015. Setting, participants: Adults presenting with chest pain to EDs of 16 public hospitals in Queensland. INTERVENTION: Implementation of the ADP by structured clinical re-design. MAIN OUTCOME MEASURES: Primary outcome: hospital LOS. SECONDARY OUTCOMES: ED LOS, hospital admission rate, proportion of patients identified as being at low risk of an acute coronary syndrome (ACS). RESULTS: Outcomes were recorded for 30 769 patients presenting before and 23 699 presenting after implementation of the ADP. Following implementation, 21.3% of patients were identified by the ADP as being at low risk for an ACS. Following implementation of the ADP, mean hospital LOS fell from 57.7 to 47.3 hours (rate ratio [RR], 0.82; 95% CI, 0.74-0.91) and mean ED LOS for all patients presenting with chest pain fell from 292 to 256 minutes (RR, 0.80; 95% CI, 0.72-0.89). The hospital admission rate fell from 68.3% (95% CI, 59.3-78.5%) to 54.9% (95% CI, 44.7-67.6%; P < 0.01). The estimated release in financial capacity amounted to $2.3 million as the result of reduced ED LOS and $11.2 million through fewer hospital admissions. CONCLUSIONS: Implementing an evidence-based ADP for assessing patients with chest pain was feasible across a range of hospital types, and achieved a substantial release of health service capacity through reductions in hospital admissions and ED LOS.

Sex Differences in Phenotypes of Bicuspid Aortic Valve and Aortopathy: Insights From a Large Multicenter, International Registry.

BACKGROUND: This large multicenter, international bicuspid aortic valve (BAV) registry aimed to define the sex differences in prevalence, valve morphology, dysfunction (aortic stenosis/regurgitation), aortopathy, and complications (endocarditis and aortic dissection). METHODS AND RESULTS: Demographic, clinical, and echocardiographic data at first presentation of 1992 patients with BAV (71.5% men) were retrospectively analyzed. BAV morphology and valve function were assessed; aortopathy configuration was defined as isolated dilatation of the sinus of Valsalva or sinotubular junction, isolated dilatation of the ascending aorta distal to the sinotubular junction, or diffuse dilatation of the aortic root and ascending aorta. New cases of endocarditis and aortic dissection were recorded. There were no significant sex differences regarding BAV morphology and frequency of normal valve function. When presenting with moderate/severe aortic valve dysfunction, men had more frequent aortic regurgitation than women (33.8% versus 22.2%, P<0.001), whereas women were more likely to have aortic stenosis (34.5% versus 44.1%, P<0.001). Men had more frequently isolated dilatation of the sinus of Valsalva or sinotubular junction (14.2% versus 6.7%, P<0.001) and diffuse dilatation of the aortic root and ascending aorta (16.2% versus 7.3%, P<0.001) than women. Endocarditis (4.5% versus 2.5%, P=0.037) and aortic dissections (0.5% versus 0%, P<0.001) occurred more frequently in men. CONCLUSIONS: Although there is a male predominance among patients with BAV, men with BAV had more frequently moderate/severe aortic regurgitation at first presentation compared with women, whereas women presented more often with moderate/severe aortic stenosis compared with men. Furthermore, men had more frequent aortopathy than women.

Prognostic Implications of Raphe in Bicuspid Aortic Valve Anatomy.

Importance: Little is known about the association between bicuspid aortic valve (BAV) morphologic findings and the degree of valvular dysfunction, presence of aortopathy, and complications, including aortic valve surgery, aortic dissection, and all-cause mortality. Objective: To investigate the association between BAV morphologic findings (raphe vs nonraphe) and the degree of valve dysfunction, presence of aortopathy, and prognosis (including need for aortic valve surgery, aortic dissection, and all-cause mortality). Design, Setting, and Participants: In this large international multicenter registry of patients with BAV treated at tertiary referral centers, 2118 patients with BAV were evaluated. Patients referred for echocardiography from June 1, 1991, through November 31, 2015, were included in the study. Exposures: Clinical and echocardiographic data were analyzed retrospectively. The morphologic BAV findings were categorized according to the Sievers and Schmidtke classification. Aortic valve function was divided into normal, regurgitation, or stenosis. Patterns of BAV aortopathy included the following: type 1, dilation of the ascending aorta and aortic root; type 2, isolated dilation of the ascending aorta; and type 3, isolated dilation of the sinus of Valsalva and/or sinotubular junction. Main Outcomes and Measures: Association between the presence and location of raphe and the risk of significant (moderate and severe) aortic valve dysfunction and aortic dilation and/or dissection. Results: Of the 2118 patients (mean [SD] age, 47 [18] years; 1525 [72.0%] male), 1881 (88.8%) had BAV with fusion raphe, whereas 237 (11.2%) had BAV without raphe. Bicuspid aortic valves with raphe had a significantly higher prevalence of valve dysfunction, with a significantly higher frequency of aortic regurgitation (622 [33.1%] vs 57 [24.1%], P < .001) and aortic stenosis (728 [38.7%] vs 51 [21.5%], P < .001). Furthermore, aortic valve replacement event rates were significantly higher among patients with BAV with raphe (364 [19.9%] at 1 year, 393 [21.4%] at 2 years, and 447 [24.4%] at 5 years) vs patients without raphe (30 [14.0%] at 1 year, 32 [15.0%] at 2 years, and 40 [18.0%] at 5 years) (P = .02). In addition, the all-cause mortality event rates were significantly higher among patients with BAV with raphe (77 [5.1%] at 1 year, 87 [6.2%] at 2 years, and 110 [9.5%] at 5 years) vs patients without raphe (2 [1.8%] at 1 year, 3 [3.0%] at 2 years, and 5 [4.4%] at 5 years) (P = .03). However, on multivariable analysis, the presence of raphe was not significantly associated with all-cause mortality. Conclusions and Relevance: In this large multicenter, international BAV registry, the presence of raphe was associated with a higher prevalence of significant aortic stenosis and regurgitation. The presence of raphe was also associated with increased rates of aortic valve and aortic surgery. Although patients with BAV and raphe had higher mortality rates than patients without, the presence of a raphe was not independently associated with increased all-cause mortality.

Clinical predictors of genetic testing outcomes in hypertrophic cardiomyopathy.

PURPOSE: Genetic testing for hypertrophic cardiomyopathy has been commercially available for almost a decade; however, low mutation detection rate and cost have hindered uptake. This study sought to identify clinical variables that can predict probands with hypertrophic cardiomyopathy in whom a pathogenic mutation will be identified. METHODS: Probands attending specialized cardiac genetic clinics across Australia over a 10-year period (2002-2011), who met clinical diagnostic criteria for hypertrophic cardiomyopathy and who underwent genetic testing for hypertrophic cardiomyopathy were included. Clinical, family history, and genotype information were collected. RESULTS: A total of 265 unrelated individuals with hypertrophic cardiomyopathy were included, with 138 (52%) having at least one mutation identified. The mutation detection rate was significantly higher in the probands with hypertrophic cardiomyopathy with an established family history of disease (72 vs. 29%, P < 0.0001), and a positive family history of sudden cardiac death further increased the detection rate (89 vs. 59%, P < 0.0001). Multivariate analysis identified female gender, increased left-ventricular wall thickness, family history of hypertrophic cardiomyopathy, and family history of sudden cardiac death as being associated with greatest chance of identifying a gene mutation. Multiple mutation carriers (n = 16, 6%) were more likely to have suffered an out-of-hospital cardiac arrest or sudden cardiac death (31 vs. 7%, P = 0.012). CONCLUSION: Family history is a key clinical predictor of a positive genetic diagnosis and has direct clinical relevance, particularly in the pretest genetic counseling setting.