Clinical characteristics and prognosis of patients with hypertrophic cardiomyopathy and heart failure with preserved ejection fraction.

BACKGROUND: Whether heart failure with preserved ejection fraction (HFpEF) is associated with an increased risk of developing systolic dysfunction and a poor prognosis in hypertrophic cardiomyopathy (HCM) patients is unknown. OBJECTIVE: We aimed to assess risk factors for the development of end-stage (ES) heart failure (HF) (ejection fraction < 50%) and compare the prognosis of different HF phenotypes. METHODS: This retrospective study was conducted on patients with HCM in China between January 2009 and February 2023. Patients were stratified into three different groups: HCM-non-HF, HCM-HFpEF and HCM-heart failure with reduced ejection fraction (HCM-HFrEF). The primary outcome was a composite of major adverse cardiac events (MACEs), including all-cause deaths, HF hospitalization, sudden cardiac death and ventricular tachycardia. RESULTS: Of 3,620 HCM patients enrolled, 1,553 (42.9%) had non-HF, 1,666 (46.0%) had HFpEF, and 579 patients (11.1%) had HFrEF at baseline. During the median follow-up period of 4.0 years (IQR 1.4-9.4 years), patients with HCM-HFpEF exhibited a higher incidence of ES-HF than those with HCM-non-HF (12.4% vs. 2.7%, P < 0.001). HFpEF was an independent risk factor for ES-HF development (HR 3.84, 2.54-5.80, P < 0.001). MACEs occurred in 26.9% with a higher incidence in HCM-HFpEF than HCM-non-HF (36.6% vs 12.2%, P < 0.001). HFpEF was an independent predictor of MACEs (HR 2.13, 1.75-2.59, P < 0.001). CONCLUSIONS: HFpEF is common in HCM. Compared to non-HF, it increases the risk of LVEF decline and poor prognosis. It may aid in risk stratification and need close echocardiography follow-up.

Ventricular septal defect following mitral valve replacement.

Mitral valve surgery (MVS), with repair preferred to replacement, is a common procedure for the treatment of severe primary mitral regurgitation related to leaflet prolapse. Structural complications after MVS include left ventricular outflow obstruction, paravalvular leak and atrial septal defect. Intraoperative transoesophageal echocardiography and predischarge transthoracic echocardiography (TTE) specifically screen for these complications. Ventricular septal defect (VSD), a known complication after aortic valve surgery, is rarely reported after MVS. Recently, unsuccessful valvuloplasty prior to replacement was suggested as a risk factor. We present such a case and explore mechanisms with advanced cardiac imaging. In this case, the patient was found to have an elongated membranous septum that likely predisposed her to septal injury. Finally, we provide guidance on specific transoesophageal/transthoracic echocardiography views to avoid a missed diagnosis.

Impact of lung transplantation on diastolic dysfunction in recipients with pretransplant pulmonary hypertension.

OBJECTIVE: Pulmonary hypertension can cause left ventricular diastolic dysfunction through ventricular interdependence. Moreover, diastolic dysfunction has been linked to adverse outcomes after lung transplant. The impact of lung transplant on diastolic dysfunction in recipients with pretransplant pulmonary hypertension is not defined. In this cohort, we aimed to assess the prevalence of diastolic dysfunction, the change in diastolic dysfunction after lung transplant, and the impact of diastolic dysfunction on lung transplant outcomes. METHODS: In a large, single-center database from January 2011 to September 2021, single or bilateral lung transplant recipients with pulmonary hypertension (mean pulmonary artery pressure > 20 mm Hg) were retrospectively identified. Those without a pre- or post-transplant echocardiogram within 1 year were excluded. Diastolic dysfunction was diagnosed and graded according to the American Society of Echocardiography 2016 guideline on assessment of diastolic dysfunction (present, absent, indeterminate). McNemar's test was used to examine association between diastolic dysfunction pre- and post-transplant. Kaplan-Meier and Cox regression analysis were used to assess associations between pre-lung transplant diastolic dysfunction and post-lung transplant 1-year outcomes, including mortality, major adverse cardiac events, and bronchiolitis obliterans syndrome grade 1 or higher-free survival. RESULTS: Of 476 primary lung transplant recipients, 205 with pulmonary hypertension formed the study cohort (mean age, 56.6 ± 11.9 years, men 61.5%, mean pulmonary artery pressure 30.5 ± 9.8 mm Hg, left ventricular ejection fraction < 55% 9 [4.3%]). Pretransplant, diastolic dysfunction was present in 93 patients (45.4%) (grade I = 8, II = 84, III = 1), absent in 16 patients (7.8%), and indeterminate in 89 patients (43.4%), and 7 patients (3.4%) had missing data. Post-transplant, diastolic dysfunction was present in 7 patients (3.4%) (grade I = 2, II = 5, III = 0), absent in 164 patients (80.0%), and indeterminate in 15 patients (7.3%), and 19 patients (9.3%) had missing data. For those with diastolic dysfunction grades in both time periods (n = 180), there was a significant decrease in diastolic dysfunction post-transplant (148/169 patients with resolved diastolic dysfunction; McNemar's test P < .001). Pretransplant diastolic dysfunction was not associated with major adverse cardiac events (hazard ratio [HR], 1.08, 95% CI, 0.72-1.62; P = .71), bronchiolitis obliterans syndrome-free survival (HR, 0.67, 95% CI, 0.39-1.56; P = .15), or mortality (HR, 0.70, 95% CI, 0.33-1.46; P = .34) at 1 year. CONCLUSIONS: Diastolic dysfunction is highly prevalent in lung transplant candidates with normal left ventricular systolic function and pulmonary hypertension, and resolves in most patients after lung transplant regardless of patient characteristics. Pre-lung transplant diastolic dysfunction was not associated with adverse lung or cardiac outcomes after lung transplant. Collectively, these findings suggest that the presence of diastolic dysfunction in lung transplant recipients with pulmonary hypertension has no prognostic significance, and as such diastolic dysfunction and the associated clinical syndrome of heart failure with preserved ejection fraction should not be considered a relative contraindication to lung transplant in such patients.

Performance of Computed Tomographic Angiography-Based Aortic Valve Area for Assessment of Aortic Stenosis.

Background A total of 40% of patients with severe aortic stenosis (AS) have low-gradient AS, raising uncertainty about AS severity. Aortic valve calcification, measured by computed tomography (CT), is guideline-endorsed to aid in such cases. The performance of different CT-derived aortic valve areas (AVAs) is less well studied. Methods and Results Consecutive adult patients with presumed moderate and severe AS based on echocardiography (AVA measured by continuity equation on echocardiography <1.5 cm2) who underwent cardiac CT were identified retrospectively. AVAs, measured by direct planimetry on CT (AVACT) and by a hybrid approach (AVA measured in a hybrid manner with echocardiography and CT [AVAHybrid]), were measured. Sex-specific aortic valve calcification thresholds (≥1200 Agatston units in women and ≥2000 Agatston units in men) were applied to adjudicate severe or nonsevere AS. A total of 215 patients (38.0% women; mean±SD age, 78±8 years) were included: normal flow, 59.5%; and low flow, 40.5%. Among the different thresholds for AVACT and AVAHybrid, diagnostic performance was the best for AVACT <1.2 cm2 (sensitivity, 85%; specificity, 26%; and accuracy, 72%), with no significant difference by flow status. The percentage of patients with correctly classified AS severity (correctly classified severe AS+correctly classified moderate AS) was as follows; AVA measured by continuity equation on echocardiography <1.0 cm2, 77%; AVACT <1.2 cm2, 73%; AVACT <1.0 cm2, 58%; AVAHybrid <1.2 cm2, 59%; and AVAHybrid <1.0 cm2, 45%. AVACT cut points of 1.52 cm2 for normal flow and 1.56 cm2 for low flow, provided 95% specificity for excluding severe AS. Conclusions CT-derived AVAs have poor discrimination for AS severity. Using an AVACT <1.2-cm2 threshold to define severe AS can produce significant error. Larger AVACT thresholds improve specificity.

Integrated smoking cessation and mood management following acute coronary syndrome: Protocol for the post-acute cardiac event smoking (PACES) trial.

BACKGROUND: Approximately 400,000 people who smoke cigarettes survive Acute Coronary Syndrome (ACS; unstable angina, ST and non-ST elevation myocardial infarction) each year in the US. Continued smoking following ACS is an independent predictor of mortality. Depressed mood post-ACS is also predictive of mortality, and smokers with depressed mood are less likely to abstain from smoking following an ACS. A single, integrated treatment targeting depressed mood and smoking could be effective in reducing post-ACS mortality. METHOD/DESIGN: The overall aim of the current study is to conduct a fully powered efficacy trial enrolling 324 smokers with ACS and randomizing them to 12 weeks of an integrated smoking cessation and mood management treatment [Behavioral Activation Treatment for Cardiac Smokers (BAT-CS)] or control (smoking cessation and general health education). Both groups will be offered 8 weeks of the nicotine patch if medically cleared. Counseling in both arms will be provided by tobacco treatment specialists. Follow-up assessments will be conducted at end-of-treatment (12-weeks) and 6, 9, and 12 months after hospital discharge. We will track major adverse cardiac events and all-cause mortality for 36 months post-discharge. Primary outcomes are depressed mood and biochemically validated 7-day point prevalence abstinence from smoking over 12 months. DISCUSSION: Results of this study will inform smoking cessation treatments post-ACS and provide unique data on the impact of depressed mood on success of post-ACS health behavior change attempts. TRIAL REGISTRATION: ClinicalTrials.gov, NCT03413423. Registered 29 January 2018. https://beta. CLINICALTRIALS: gov/study/NCT03413423 .

Clinical Outcome After Left Ventricular Thrombus Resolution: Who Needs Long-Term or Lifetime Use of Anticoagulants?

Background Patients with left ventricular thrombus (LVT) resolution can have LVT recurrence and risk for thromboembolism. However, these outcomes after LVT resolution are not well known. We aimed to assess the prevalence, risk factors, and clinical outcomes for LVT recurrence in patients with LVT resolution to inform follow-up and treatment. Methods and Results Patients with LVT resolution were identified retrospectively from a large echocardiography database between January 2009 and May 2022. Participants had echocardiograms at 3 time points, including baseline at LVT diagnosis, at LVT resolution, and a follow-up for identification of LVT recurrence. The cumulative LVT recurrence rate was estimated by the Kaplan-Meier method, and predictors of LVT recurrence were evaluated using Cox regression analysis. Among 115 patients with LVT resolution, 28 (24.3%) had LVT recurrence at a median follow-up of 1.2 (0.5-2.8) years. LV aneurysm (hazard ratio [HR], 2.59 [95% CI, 1.20-5.58], P=0.015) and anticoagulant use (HR, 0.12 [95% CI, 0.04-0.41], P=0.001) were predictors of LVT recurrence on multivariable analysis. Patients with an LV aneurysm who did not receive any anticoagulation demonstrated an LVT recurrence rate of 69.5%, whereas those without an LV aneurysm who received anticoagulation had a recurrence rate of 0%. Patients with LVT recurrence had a higher incidence of an embolic event (10.7% versus 1.1%, P=0.016). Conclusions LVT recurrence after LVT resolution is common, especially in those with an LV aneurysm, and is associated with a higher embolic risk. Continued anticoagulation is protective against LVT recurrence, although bleeding risk needs to be considered. These findings can inform follow-up and treatment of patients with documented LVT resolution.

New-onset postoperative atrial fibrillation after mitral valve surgery: Determinants and the effect on survival.

Objective: Mitral valve surgery (MVS) carries substantial risk of postoperative atrial fibrillation (PAF). Identifying patients who benefit from prophylactic left atrial appendage amputation (LAAA) or maze is ill-defined. To guide such interventions, we determined preoperative predictors of PAF and investigated 3-year survival of patients with PAF. Methods: We performed a retrospective analysis of patients undergoing isolated MVS (N = 670) between 2011 and 2021. Patients with preoperative atrial fibrillation, LAAA or pulmonary vein isolation were excluded. Patient characteristics were compared between those without PAF and those who developed transient or prolonged PAF. Predictors of any PAF and prolonged PAF were identified using multivariable regression analysis. Results: In total, 504 patients without preoperative atrial fibrillation underwent isolated MVS. Of them, 303 patients (60.2%) developed PAF; 138 (27.3%) developed transient and 165 (32.7%) developed prolonged (beyond 30 days) PAF. Patients with PAF were older (65.7 vs 54.3 years, P < .001), with larger left atria (4.8 vs 4.3 cm, P < .001), greater prevalence of hypertension (60% vs 47.8%, P < .05), and were New York Heart Association class III/IV (36% vs 8.5%, P < .001). Independent predictors of PAF included left atria volume index (odds ratio [OR], 1.02; P < .003), older age (OR, 1.04; P < .001), heart failure (OR, 6.73; P < .001), and sternotomy (OR, 2.19; P < .002). Age, heart failure, and sternotomy were independent predictors of prolonged PAF. Patients with PAF had greater mortality at 3 years compared with those without PAF (5.3% vs 0.5%, P < .005). On multivariable analysis, PAF was associated with increased mortality (hazard ratio, 7.81; P < .046). Conclusions: PAF is common after MVS and associated with late mortality. Older age, advanced heart failure, and sternotomy are associated with prolonged PAF. These factors may identify patients who would benefit from prophylactic LAAA or ablation during MVS.

Incidental coronary calcium in cancer patients treated with anthracycline and/or trastuzumab.

AIMS: Cancer patients are at increased risk of cardiovascular disease (CVD) after treatment with potentially cardiotoxic treatments. Many cancer patients undergo non-gated chest computed tomography (NCCT) for cancer staging prior to treatment. We aimed to assess whether coronary artery calcification on NCCT predicts CVD risk in cancer patients. METHODS AND RESULTS: Six hundred and three patients (mean age: 61.3 years, 30.8% male) with either breast cancer, lymphoma, or sarcoma were identified retrospectively. Primary endpoint was a major adverse cardiac event (MACE) composite including non-fatal myocardial infarction, new heart failure (HF) diagnosis, HF hospitalization, and cardiac death, with Fine-Gray analysis for non-cardiac death as competing risk. Secondary endpoints included a coronary composite and a HF composite. Coronary artery calcification was present in 194 (32.2%) and clinically reported in 85 (43.8%) patients. At a median follow-up of 5.3 years, 256 (42.5%) patients died of non-cardiac causes. Coronary artery calcification presence or extent was not an independent predictor of MACE [sub-distribution hazards ratio (SHR) 1.28; 0.73-2.27]. Coronary artery calcification extent was a significant predictor of the coronary composite outcome (SHR per two-fold increase 1.14; 1.01-1.28), but not of the HF composite outcome (SHR per two-fold increase 1.04; 0.95-1.14). CONCLUSION: Coronary artery calcification detected incidentally on NCCT scans in cancer patients is prevalent and often not reported. Coronary artery calcification presence or extent did not independently predict MACE. Coronary artery calcification extent was independently associated with increased risk of CAD events but not HF events.

Impact of pectoralis muscle loss on cardiac outcome and survival in Cancer patients who received anthracycline based chemotherapy: retrospective study.

INTRODUCTION: The impact of pectoralis muscle mass index (PMI) on cardiac events is not well studied in cancer patients, especially in those who have received chemotherapy with high potential cardiac toxicity such as anthracyclines. METHODS: Individuals aged ≥18 years with a diagnosis of breast cancer, sarcoma, or lymphoma who received anthracycline-based chemotherapy at the University of Minnesota MHealth Fairview between 2009 and 2014. Eligible patients had to have two CT scans: a baseline CT scan within 6 months prior to chemotherapy and a follow-up CT scan within 2 years after treatment. The PMI was calculated as the right pectoralis muscle area indexed to height squared. Multivariable linear regression was used to analyze factors associated with PMI at follow-up, overall mortality, and major cardiac events (MACE). RESULTS: A total of 474 patients (breast cancer 192; lymphoma 184; sarcoma 98) participated with a median age of 61 years at the time of baseline CT scan; 161 (34%) were male. Almost all patients received anthracyclines except 12% who received trastuzumab only. The median baseline PMI was 5.8 cm2/m2 (4.9, 7.7) which decreased 10.5% after chemotherapy, to 5.2 cm2/m2 (4.4, 6.4). Baseline PMI was not significantly associated with OS, but we detected lower risks of MACE with larger PMI at baseline. Greater baseline PMI was associated with greater follow-up PMI, but also with greater relative PMI loss. Female gender, older age, and history of smoking were also associated with greater PMI losses. CONCLUSION: Greater pre-treatment pectoralis muscle index in patients treated with anthracyclines have a lower risk of MACE. Early identification of sarcopenia using PMI could trigger proactive engagement for intervention and risk-stratified therapies.

A rare case report of severe cardiomyopathy associated with myotonic dystrophy type 2.

Background: Myotonic dystrophies (DM) are multi-systemic diseases characterized by muscle weakness and myotonia. Despite a growing appreciation for the cardiovascular manifestations in myotonic dystrophy type 1 (DM1), cardiac involvement in myotonic dystrophy type 2 (DM2) has been less well characterized. In patients with DM2, cardiomyopathy has rarely been described. Case summary: This case report describes a rare case of DM2 associated cardiomyopathy. A 56-year-old male with DM2 who presented with palpitations and fatigue. Cardiac magnetic resonance (CMR) imaging confirmed a severely enlarged left ventricular cavity with a left ventricular ejection fraction of 28% consistent with severely reduced global systolic function. The lateral wall epicardium exhibited late gadolinium enhancement in a pattern seen in myotonic dystrophy-related cardiomyopathy. Discussion: This case highlights the potential for significant cardiovascular involvement in DM2, as well as the importance of screening, including CMR imaging, and therapy in the myotonic dystrophy patient population.

Prognostic impact of identifying etiology of prosthetic valve dysfunction with CT.

BACKGROUND: In patients with prosthetic heart valves (PHV), there are distinct treatment implications based on prosthetic valve dysfunction (PVD) etiology. We investigated whether evaluation for PVD etiology on computed tomography (CT) has prognostic value for adverse clinical outcomes. METHODS: Consecutive patients with suspected PVD that had a clinically indicated contrast chest CT and echocardiogram done within 1 year of each other were identified retrospectively from the Prosthetic Heart Valve CT Registry at the University of Minnesota. CTs and echocardiograms were assessed for potential PVD etiologies of pannus, structural valve degeneration (SVD) and thrombus, as per standard guidelines. Kaplan-Meier and Cox regression analyses were performed to assess association with a composite outcome of reoperation and all-cause mortality. RESULTS: 132 patients (51.5% male, mean age 62.1 â€‹± â€‹19.3 years) with suspected PVD were included. There were 97 tissue valves, 31 mechanical valves and 4 transcatheter valves. The location of the valve was as follows: 72 aortic, 45 mitral, 8 tricuspid, and 7 pulmonic. A PVD etiology was diagnosed on CT in 80 (60.6%) patients, and on echocardiography in 45 (34.1%) patients, largely driven by a diagnosis of SVD on both modalities. Significant univariate predictors of the composite outcome included CT diagnosis of SVD (P â€‹< â€‹0.001), echocardiography diagnosis of SVD (P â€‹< â€‹0.001), degree of prosthetic stenosis (P â€‹< â€‹0.001) and degree of prosthetic regurgitation (P â€‹< â€‹0.001). On multivariable analyses adjusted for age, sex, left ventricular function, degree of prosthetic stenosis and degree of prosthetic regurgitation, CT diagnosis of SVD was significantly associated with the composite outcome (HR: 1.79, 1.09-2.95) whereas echocardiography diagnosis of SVD was not (HR: 1.56, 0.98-2.46). CONCLUSION: In patients with suspected PVD, CT assessment of SVD had prognostic significance for hard outcomes. CT should be considered in the diagnostic evaluation of patients with suspected PVD.

Long-term prognostic value of right ventricular dysfunction on cardiovascular magnetic resonance imaging in anthracycline-treated cancer survivors.

AIMS: We aimed to determine the prevalence of right ventricular (RV) systolic dysfunction on cardiovascular magnetic resonance imaging (CMR) and its impact on long-term adverse outcomes in a large cohort of cancer survivors treated with anthracycline-based chemotherapy. METHODS AND RESULTS: Consecutive cancer survivors treated with anthracyclines who underwent clinical CMR for suspected anthracycline-related cardiomyopathy were studied. The primary endpoint was a composite of all-cause death or major adverse cardiac events (MACE): heart failure hospitalization, heart transplantation, ventricular assist device implantation, resuscitated cardiac arrest, or life-threatening ventricular arrhythmia. The secondary endpoints were all-cause death, and cardiac death or MACE. Among 249 survivors who underwent CMR at a median of 2.9 years after cancer treatment, RV systolic dysfunction was present in 54 (21.7%). Of these, 50 (92.6%) had an abnormal left ventricular ejection fraction (LVEF). At a median follow-up time after the CMR of 2.7 years, 105 survivors experienced the primary endpoint. On Kaplan-Meier analyses, the cumulative incidence of the primary endpoint was significantly higher in survivors with abnormal RVEF compared with those with normal RVEF (P = 0.002). However, on Cox multivariable analyses, RVEF was not associated with the primary endpoint (HR 1.04 per 5% decrease; 95% CI 0.93-1.17; P = 0.46) after adjustment for non-imaging variables and LVEF. RVEF was also not associated with the secondary endpoints. CONCLUSION: Among anthracycline-treated cancer survivors undergoing CMR for suspected cardiotoxicity, RV systolic dysfunction was present in one in five cases, accompanied by LV systolic dysfunction in nearly all cases, and was not independently associated with long-term outcomes.

Prognostic Role of Paravalvular Spread on Computed Tomography in Suspected Prosthetic Valve Endocarditis.

We aimed to assess the prognostic value of computed tomography assessment of paravalvular spread in suspected prosthetic valve endocarditis.

Interplay of CT Coronary Plaque Characteristics and Ischemia to Predict Adverse Outcomes.

In 134 patients with stable coronary artery disease, high-risk plaque features at CT and physiologic stenosis were found to act in a synergistic fashion to predict adverse outcomes.

Sex Differences in Patients With Suspected Cardiac Sarcoidosis Assessed by Cardiovascular Magnetic Resonance Imaging.

Background: There are few data on sex differences in suspected cardiac sarcoidosis. Methods: Consecutive patients with histologically proven sarcoidosis and suspected cardiac involvement were studied. We investigated sex differences in presenting features, cardiac involvement, and the long-term incidence of a primary composite end point of all-cause death or significant ventricular arrhythmia and secondary end points of all-cause death and significant ventricular arrhythmia. Results: Among 324 patients, 163 (50.3%) were female and 161 (49.7%) were male patients. Female patients had a greater prevalence of chest pain (37.4% versus 23.6%; P=0.010) and palpitations (39.3% versus 26.1%; P=0.016) than male patients but not dyspnea, presyncope, syncope, or arrhythmias at presentation. Female patients had a lower prevalence of late gadolinium enhancement on cardiovascular magnetic resonance imaging (20.2% versus 35.4%; P=0.003) and less often met criteria for a clinical diagnosis of cardiac sarcoidosis (Heart Rhythm Society consensus criteria, 22.7% versus 36.0%; P=0.012 and 2016 Japanese Circulation Society guideline criteria, 8.0% versus 19.3%; P=0.005), indicating lesser cardiac involvement. However, the long-term incidence of all-cause death or significant ventricular arrhythmia was not different between female and male patients (23.2% versus 23.2%; P=0.46). Among the secondary end points, the incidence of all-cause death was not different between female and male patients (20.7% versus 14.3%; P=0.51), while female patients had a lower incidence of significant ventricular arrhythmia compared with male patients (4.3% versus 13.0%; P=0.022). On multivariable analyses, sex was not associated with the primary end point (hazard ratio for female patients, 1.36 [95% CI, 0.77­2.43]; P=0.29). Conclusions: We observed distinct sex differences in patients with suspected cardiac sarcoidosis. A paradox was identified wherein female patients had a greater prevalence of chest pain and palpitations than male patients, but lesser cardiac involvement, and a similar long-term incidence of all-cause death or significant ventricular arrhythmia.

Variable and Severe Phenotypic Expression of the "Lebanese Allele" in Two Sisters with Familial Hypercholesterolemia.

The "Lebanese allele" {LDLR c.2043 C>A (p.cys681X)} is a nonsense mutation in the low-density lipoprotein receptor (LDLR) gene that results in a truncated non-functioning LDLR protein. We report two sisters of Lebanese descent who presented with familial hypercholesterolemia (FH) and were both heterozygous for the Lebanese allele, but had very distinct LDL-C levels and clinical phenotypes. Whereas one of the sisters had LDL-C in the expected range of Heterozygous FH (HeFH) with the Lebanese allele (LDL-C of 292 mg/dl), the other sister had a more severe LDL-C phenotype in the Homozygous FH (HoFH) range (LDL-C of 520 mg/dl) along with manifest atherosclerosis. Surprisingly, she did not demonstrate a compound heterozygote or double heterozygote status. We discuss different mechanisms that are purported to play a role in modifying the phenotype of FH, including different variants and polygenic modifiers. HeFH patients with the Lebanese allele can have a wide spectrum of LDL-C levels that range from the typical heterozygous to homozygous phenotypes.

Impact of 2016 SCCT/STR guidelines for coronary artery calcium scoring of noncardiac chest CT scans on lung cancer screening CT reporting.

The 2016 SCCT/STR guideline for coronary artery calcification (CAC) scoring on non-cardiac chest CT (NCCT) scans explicitly calls for the reporting of CAC. Whether the publication of the 2016 SCCT/STR guideline has had any impact on CAC reporting in lung cancer screening (LCS) scans has not been investigated. Consecutive patients with a LCS scan were identified from the University of Minnesota LCS registry and evaluated for CAC reporting in 3 separate cohorts: 6 months before, 6 months after, and 1 year after the publication of the 2016 SCCT/STR guideline. Scans were evaluated for CAC and quantified using the Agatston method. CAC reporting, downstream testing and initiation of preventive therapy were assessed. Among 614 patients (50% male, mean age 64.1 ± 6.0 years), CAC was present in 460 (74.9%) with a median Agatston score of 62 (IQR 0, 230). Of these, 196 (31.9%) had a CAC score of 1-100, 125 (20.4%) had 101-300, and 118 (19.2%) had > 300. Overall, CAC was reported in 325 (70.7%) patients with CAC present. CAC reporting relative to publication of the 2016 SCCT/STR guideline was as follows: 6 months prior-74.1%, 6 months after-64.6%, and 1 year after-77.5%. In the 308 patients with a new diagnosis of sub-clinical CAD based on CAC presence, 6 (1.9%) patients were referred to cardiology, and 15 (4.9%) patients underwent testing for obstructive CAD. Only 6 (1.9%) and 9 (2.9%) patients were newly started on aspirin and statin respectively. CAC detected incidentally on lung cancer screening CT scans is prevalent, and rarely acted upon clinically. CAC reporting is fairly high, and publication of the 2016 SCCT/STR guideline for CAC scoring on NCCT scans did not have any significant impact on CAC reporting.