Rationale and design of the CONFIRM2 (Quantitative COroNary CT Angiography Evaluation For Evaluation of Clinical Outcomes: An InteRnational, Multicenter Registry) study.

BACKGROUND: In the last 15 years, large registries and several randomized clinical trials have demonstrated the diagnostic and prognostic value of coronary computed tomography angiography (CCTA). Advances in CT scanner technology and developments of analytic tools now enable accurate quantification of coronary artery disease (CAD), including total coronary plaque volume and low attenuation plaque volume. The primary aim of CONFIRM2, (Quantitative COroNary CT Angiography Evaluation For Evaluation of Clinical Outcomes: An InteRnational, Multicenter Registry) is to perform comprehensive quantification of CCTA findings, including coronary, non-coronary cardiac, non-cardiac vascular, non-cardiac findings, and relate them to clinical variables and cardiovascular clinical outcomes. DESIGN: CONFIRM2 is a multicenter, international observational cohort study designed to evaluate multidimensional associations between quantitative phenotype of cardiovascular disease and future adverse clinical outcomes in subjects undergoing clinically indicated CCTA. The targeted population is heterogenous and includes patients undergoing CCTA for atherosclerotic evaluation, valvular heart disease, congenital heart disease or pre-procedural evaluation. Automated software will be utilized for quantification of coronary plaque, stenosis, vascular morphology and cardiac structures for rapid and reproducible tissue characterization. Up to 30,000 patients will be included from up to 50 international multi-continental clinical CCTA sites and followed for 3-4 years. SUMMARY: CONFIRM2 is one of the largest CCTA studies to establish the clinical value of a multiparametric approach to quantify the phenotype of cardiovascular disease by CCTA using automated imaging solutions.

Incidental Coronary Artery Calcium on Chest CT in Persons Without Known Atherosclerotic Cardiovascular Disease.

This cross-sectional study examines the expected prevalence of coronary artery calcium (CAC) on chest computed tomography (CT) in people without clinical atherosclerotic cardiovascular disease (ASCVD) by age, sex, and race and ethnicity.

Preferences and Attitudes of Cardiologists in Management of Patients with Cancer.

Background: With recent improvements in survival of cancer patients and common use of high-value care at end of life, the management of cardiovascular disease (CVD) in patients with cancer is increasingly important. To our knowledge, there are no current U.S. data examining how the presence and extent of cancer influence cardiologists' decision making for common cardiovascular conditions. Methods: An anonymous online vignette-based survey of cardiologists was conducted at five U.S. institutions investigating how the extent of gastrointestinal and thoracic malignancies (prior/localized, metastatic) would influence treatment recommendations for atrial fibrillation (AF), aortic stenosis, unstable angina (UA), and obstructive coronary artery disease (CAD). Results: Thirty-three percent (86/259) of cardiologists completed the survey between September and November 2019. Participants were 67% male, 51% below age 40, and 58% had five or more years of clinical experience. Majority of cardiologists practiced at teaching hospitals (72%) and were noninterventional (63%). Cardiologists were more likely to recommend procedural interventions for patients with localized cancer than for those with metastatic disease: AF (left atrial appendage occlusion: 20% vs. 8%), atrial stenosis (aortic valve repair: 83% vs. 11%), UA (left heart catheter: 70% vs. 27%), and obstructive CAD (percutaneous coronary intervention: 81% vs. 38%). In patients with metastatic cancer, most cardiologists sought an oncology (82%) or a palliative care (69%) consultation. However, a persistent trend of undertreatment in patients with localized cancers and overtreatment in patients with end-of-life disease was apparent. Conclusions: Cardiologists were less likely to recommend invasive cardiovascular therapies to patients with metastatic cancer. This preference pattern likely reflects the influence of comorbidities and quality of life expectation on cardiologists' treatment recommendations but may also be related to the stigma of advanced cancer. Better communication between cardiologists and oncologists is necessary to provide a personalized care of patients with cancer and CVD that would maximize treatment benefit with least morbidity.

Increased RV:LV ratio on chest CT-angiogram in COVID-19 is a marker of adverse outcomes.

BACKGROUND: Right ventricular (RV) dilation has been used to predict adverse outcomes in acute pulmonary conditions. It has been used to categorize the severity of novel coronavirus infection (COVID-19) infection. Our study aimed to use chest CT-angiogram (CTA) to assess if increased RV dilation, quantified as an increased RV:LV (left ventricle) ratio, is associated with adverse outcomes in the COVID-19 infection, and if it occurs out of proportion to lung parenchymal disease. RESULTS: We reviewed clinical, laboratory, and chest CTA findings in COVID-19 patients (n = 100), and two control groups: normal subjects (n = 10) and subjects with organizing pneumonia (n = 10). On a chest CTA, we measured basal dimensions of the RV and LV in a focused 4-chamber view, and dimensions of pulmonary artery (PA) and aorta (AO) at the PA bifurcation level. Among the COVID-19 cohort, a higher RV:LV ratio was correlated with adverse outcomes, defined as ICU admission, intubation, or death. In patients with adverse outcomes, the RV:LV ratio was 1.06 ± 0.10, versus 0.95 ± 0.15 in patients without adverse outcomes. Among the adverse outcomes group, compared to the control subjects with organizing pneumonia, the lung parenchymal damage was lower (22.6 ± 9.0 vs. 32.7 ± 6.6), yet the RV:LV ratio was higher (1.06 ± 0.14 vs. 0.89 ± 0.07). In ROC analysis, RV:LV ratio had an AUC = 0.707 with an optimal cutoff of RV:LV ≥ 1.1 as a predictor of adverse outcomes. In a validation cohort (n = 25), an RV:LV ≥ 1.1 as a cutoff predicted adverse outcomes with an odds ratio of 76:1. CONCLUSIONS: In COVID-19 patients, RV:LV ratio ≥ 1.1 on CTA chest is correlated with adverse outcomes. RV dilation in COVID-19 is out of proportion to parenchymal lung damage, pointing toward a vascular and/or thrombotic injury in the lungs.

A Rare Case of Primary Pericardial Schwannoma.

Schwannomas are typically benign, indolent neoplasms. Primary pericardial schwannomas are extremely rare and arise from the cardiac plexus and vagus nerve innervating the heart. Few case reports have been documented to date. Pericardial schwannomas are difficult to diagnose at plain radiography or transthoracic echocardiography, often leading to further characterization with either CT or MRI. Biopsy is required for definitive diagnosis. A case of primary pericardial schwannoma of the posterior pericardium with concerns for compression of the left atrium and left ventricle is presented. © RSNA, 2021.

Technical and clinical study of x-ray-based surface echo probe tracking using an attached fiducial apparatus.

PURPOSE: Several types of structural heart intervention (SHI) use information from multiple imaging modalities to complete an interventional task. For example, in transcatheter aortic valve replacement (TAVR), placement and deployment of a bioprosthetic aortic valve in the aorta is primarily guided by x-ray fluoroscopy (XRF), and echocardiography provides visualization of cardiac anatomy and blood flow. However, simultaneous interpretation of independent x-ray and echo displays remains a challenge for the interventionalist. The purpose of this work was to develop a novel echo/x-ray co-registration solution in which volumetric transthoracic echo (TTE) is transformed to the x-ray coordinate system by tracking the three-dimensional (3D) pose of a probe fiducial attachment from its appearance in two-dimensional (2D) x-ray images. METHODS: A fiducial attachment for a commercial TTE probe consisting of rings of high-contrast ball bearings was designed and fabricated. The 3D pose (position and orientation) of the fiducial attachment is estimated from a 2D x-ray image using an algorithm in which a virtual point cloud model of the attachment is iteratively rotated, translated, and forward-projected onto the image until the average sum-of-squares of grayscale values at the projected points is minimized. Fiducial registration error (FRE) and target registration error (TRE) of this approach were evaluated in phantom studies using TAVR-relevant gantry orientations and four standard acoustic windows for the TTE probe. A patient study was conducted to assess the clinical suitability of the fiducial attachment prototype during TTE imaging of patients undergoing SHI. TTE image quality for the task of guiding a transcatheter procedure was evaluated in a reviewer study. RESULTS: The 3D FRE ranged from 0.32 ± 0.03 mm (mean ± SD) to 1.31 ± 0.05 mm, depending on C-arm orientation and probe acoustic window. The 3D TRE ranged from 1.06 ± 0.03 mm to 2.42 ± 0.06 mm. Fiducial pose estimation was stable when >75% of the fiducial markers were visible in the x-ray image. A panel of reviewers graded the presentation of heart valves in TTE images from 48 SHI patients. While valve presentation did not differ significantly between acoustic windows (P > 0.05), the mitral valve did achieve a significantly higher image quality compared to the aortic and tricuspid valves (P < 0.001). Overall, reviewers perceived sufficient image quality in 76.5% of images of the mitral valve, 54.9% of images of the aortic valve, and 48.6% of images of the tricuspid valve. CONCLUSIONS: Fiducial-based tracking of a commercial TTE probe is compatible with clinical SHI workflows and yields 3D target registration error of less than 2.5 mm for a variety of x-ray gantry geometries and echo probe acoustic windows. Although TTE image quality with respect to target valve anatomy was sufficient for the majority of cases examined, prescreening of patients for sufficient TTE quality would be helpful.

It hurts to swallow! Pseudoachalasia resulting from attempted transcatheter occlusion of a giant congenital coronary artery fistula.

Congenital coronary artery fistula (CAF) is a rare anomaly that can cause heart failure and myocardial ischemia. In recent decades, transcatheter approaches to occlude CAF have emerged as minimally invasive alternatives to surgical ligation. Reported complications with transcatheter CAF occlusion include device embolization and dissection. We report the first case of attempted transcatheter occlusion of a giant CAF that resulted in severe pseudoachalasia.

Performance of Dynamic Automated CT Annular Measurements Compared to Standard Manual Measurements for Transcatheter Aortic Valve Replacement Sizing.

PURPOSE: We sought to determine the performance of an automated computed tomography (CT) software that provides dynamic annular measurements of all available cardiac phases for transcatheter aortic valve replacement (TAVR) sizing as compared to the standard single manual measurement. MATERIALS AND METHODS: In 60 TAVR patients (84±7 years, 60% male) who underwent pre-procedural CT scans, we measured the aortic annular diameters, perimeter, and area using (1) the dynamic automated CT measurements and (2) standard single manual measurement from the cardiac phase of maximum systolic opening by visual estimate. RESULTS: The automated software was successful in providing annular measurements in 43/60 (72%) of cases, with the remainder requiring semi-automated contours. The maximum dynamic automated values were predominantly in systole (46/60[77%] for diameter, 44/60[73%] for perimeter, 48/60[80%] for area), and was a different phase from the standard manual phase in 46/60 (77%) cases. The maximum dynamic automated annular values were larger than the standard manual values measured (Δdiameter 0.35 mm, p=0.04; Δperimeter 1.71 mm, p<0.001; Δarea 15.6 mm2, p<0.001). When comparing standard manual to the same phase by automated measurements, while there was no difference in annular mean diameter (p=0.80), perimeter and area were larger with the automated measurements (Δperimeter 0.95 mm, p=0.002; Δarea 10.8 mm2, p=0.03). However, the maximum automated measurements were consistently larger than the same phase automated measurements (Δdiameter 0.13 mm, p<0.001; Δperimeter 0.42 mm, p<0.001; Δarea 4.4 mm2, p<0.001). CONCLUSIONS: Automated maximum dynamic CT annular measurements provide larger values than standard manual and same phase automated measurements.

Relationship of soluble ST2 to pulmonary hypertension severity in patients undergoing cardiac resynchronization therapy.

BACKGROUND: Pulmonary hypertension (PH) is an adverse prognostic marker in patients undergoing cardiac resynchronization therapy (CRT). We sought to determine the relation of biomarkers of fibrosis [soluble ST2 (sST2), galectin-3], wall stretch [amino terminal pro-brain natriuretic peptide (NT-proBNP)], and necrosis [high-sensitivity troponin-I (hsTnI)] to PH severity in CRT patients. METHODS: Biomarkers and right ventricular systolic pressure (RVSP) were measured at CRT implant and 6-month later (n=111). PH was categorized into 3 groups based on RVSP: no (<35 mmHg), mild-moderate (35-60 mmHg), and severe (>60 mmHg). Patients were categorized as progressors (worsened PH), persistent PH (no change) and regressors (improved PH). Endpoints were 6-month CRT response and 2-year major adverse cardiac event (MACE). RESULTS: RVSP was associated with CRT nonresponse (P=0.02) and MACE (P=0.03). Severe PH patients had 5-fold increase risk for CRT nonresponse (OR 5.0, P=0.04) and MACE (HR 5.7, P=0.04) over non-PH patients. Progressors and persistent PH patients had >2-fold odds for CRT non-response (OR 2.8, P=0.45) and >11-fold increase in MACE compared to no PH patients or regressors (HR 11.6, P=0.02). Only NT-proBNP and sST2 were discernable between PH groups, with graded increase based on PH severity (both P≤0.02), and lower values in regressors versus non-regressors (both P≤0.01). Levels of sST2 decreased at 6 months in regressors (15 ng/mL, P=0.03) and increased slightly (3-8 ng/mL) in non-regressors, without difference for NT-proBNP (P=0.08). CONCLUSIONS: sST2 levels are related with PH severity in CRT patients. Serial sST2 changes after CRT implant suggests potential role to monitor PH after CRT.

Cardiorenal status using amino-terminal pro-brain natriuretic peptide and cystatin C on cardiac resynchronization therapy outcomes: From the BIOCRT Study.

BACKGROUND: Cardiorenal syndrome comprises a heterogeneous group of disorders characterized by acute or chronic cardiac and renal dysfunction. OBJECTIVE: The purpose of this study was to determine the effect of cardiorenal status using a dual-marker strategy with amino-terminal pro-brain natriuretic peptide (NT-proBNP) and cystatin C on cardiac resynchronization therapy (CRT) outcomes. METHODS: In 92 patients (age 66 ± 13 years; 80% male; left ventricular ejection fraction 26% ± 7%), NT-proBNP and cystatin C levels were measured at CRT implantation and at 1 month. NT-proBNP >1000 pg/mL and cystatin C >1 mg/L were considered high. Baseline cardiorenal patients were defined as having high NT-proBNP and cystatin C. At 1 month, CRT patients were categorized as (1) irreversible cardiorenal if cystatin C was persistently high; (2) progressive cardiorenal with transition from low to high cystatin C; (3) reversible cardiorenal with transition from high to low cystatin C; and (4) "normal" with stable low cystatin C. Outcomes were 6-month clinical and echocardiographic CRT response and 2 -year major adverse cardiovascular event (MACE). RESULTS: Compared to patients with low NT-proBNP and cystatin C, cardiorenal patients had >9-fold increase risk of CRT nonresponse (odds ratio uncompensated 9.0; compensated 36.4; both P ≤.004) and >6-fold risk of MACE (hazard ratio uncompensated 8.5; P = .005). Compared to "normal" and reversible patients (referent), irreversible patients had a 9-fold increase for CRT nonresponse (odds ratio 9.1; P <.001) and had >4-fold risk of MACE (adjusted hazard ratio 5.1; P <.001). Irreversible patients were most likely echocardiographic CRT nonresponders. CONCLUSION: Cardiorenal status by NT-proBNP and cystatin C can identify high-risk CRT patients, and those with both elevated concentrations have worse prognosis.

Bayesian reasoning in residents' preliminary diagnoses.

Whether and when humans in general, and physicians in particular, use their beliefs about base rates in Bayesian reasoning tasks is a long-standing question. Unfortunately, previous research on whether doctors use their beliefs about the prevalence of diseases in diagnostic judgments has critical limitations. In this study, we assessed whether residents' beliefs about the prevalence of a disease are associated with their judgments of the likelihood of the disease in diagnosis, and whether residents' beliefs about the prevalence of diseases change across the 3 years of residency. Residents were presented with five ambiguous vignettes typical of patients presenting on the inpatient general medicine services. For each vignette, the residents judged the likelihood of five or six possible diagnoses. Afterward, they judged the prevalence within the general medicine services of all the diseases in the vignettes. Most importantly, residents who believed a disease to be more prevalent tended to rate the disease as more likely in the vignette cases, suggesting a rational tendency to incorporate their beliefs about disease prevalence into their diagnostic likelihood judgments. In addition, the residents' prevalence judgments for each disease were assessed over the 3 years of residency. The precision of the prevalence estimates increased across the 3 years of residency, though the accuracy of the prevalence estimates did not. These results imply that residents do have a rational tendency to use prevalence beliefs for diagnosis, and this finding also contributes to a larger question of whether humans intuitively use base rates for making judgments.

Incremental value of cystatin C over conventional renal metrics for predicting clinical response and outcomes in cardiac resynchronization therapy: The BIOCRT study.

BACKGROUND: Despite the benefit of CRT in select patients with heart failure (HF), there remains significant need for predicting those at risk for adverse outcomes for this effective but costly therapy. CysC, an emerging marker of renal function, is predictive of worsening symptoms and mortality in patients with HF. This study assessed the utility of baseline and serial measures of cystatin C (CysC), compared to conventional creatinine-based measures of renal function (estimated glomerular filtration rate, eGFR), in predicting clinical outcomes following cardiac resynchronization therapy (CRT). METHODS: In 133 patients, we measured peripheral venous (PV) and coronary sinus (CS) CysC concentrations and peripheral creatinine levels at the time of CRT implant. Study endpoints included clinical response to CRT at 6 months and major adverse cardiac events (MACE) at 2 years. RESULTS: While all 3 renal metrics were predictive of MACE (all adjusted p ≤ 0.02), only CysC was associated with CRT non-response at 6 months (adjusted odds ratio 3.6, p = 0.02). CysC improved prediction of CRT non-response (p ≤ 0.003) in net reclassification index analysis compared to models utilizing standard renal metrics. Serial CysC > 1mg/L was associated with 6-month CRT non-response and reduced 6-minute walk distance as well as 2-year MACE (all p ≤ 0.04). CONCLUSION: In patients undergoing CRT, CysC demonstrated incremental benefit in the prediction of CRT non-response when compared to standard metrics of renal function. Baseline and serial measures of elevated CysC were predictive of CRT non-response and functional status at 6 months as well as long-term clinical outcomes.

Use of coronary artery calcium scanning beyond coronary computed tomographic angiography in the emergency department evaluation for acute chest pain: the ROMICAT II trial.

BACKGROUND: Whether a coronary artery calcium (CAC) scan provides added value to coronary computed tomographic angiography (CCTA) in emergency department patients with acute chest pain remains unsettled. We sought to determine the value of CAC scan in patients with acute chest pain undergoing CCTA. METHODS AND RESULTS: In the multicenter Rule Out Myocardial Infarction using Computer-Assisted Tomography (ROMICAT) II trial, we enrolled low-intermediate risk emergency department patients with symptoms suggesting acute coronary syndrome (ACS). In this prespecified subanalysis of 473 patients (54±8 years, 53% men) who underwent both CAC scanning and CCTA, the ACS rate was 8%. Overall, 53% of patients had CAC=0 of whom 2 (0.8%) developed ACS, whereas 7% had CAC>400 with 49% whom developed ACS. C-statistic of CAC>0 was 0.76, whereas that using the optimal cut point of CAC≥22 was 0.81. Continuous CAC score had lower discriminatory capacity than CCTA (c-statistic, 0.86 versus 0.92; P=0.03). Compared with CCTA alone, there was no benefit combining CAC score with CCTA (c-statistic, 0.93; P=0.88) or with selective CCTA strategies after initial CAC>0 or optimal cut point CAC≥22 (P≥0.09). Mean radiation dose from CAC acquisition was 1.4±0.7 mSv. Higher CAC scores resulted in more nondiagnostic CCTA studies although the majority remained interpretable. CONCLUSIONS: In emergency department patients with acute chest pain, CAC score does not provide incremental value beyond CCTA for ACS diagnosis. CAC=0 does not exclude ACS, nor a high CAC score preclude interpretation of CCTA in most patients. Thus, CAC results should not influence the decision to proceed with CCTA, and the decision to perform a CAC scan should be balanced with the additional radiation exposure required. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01084239.

Analysis of ventricular function by CT.

The assessment of ventricular function, cardiac chamber dimensions, and ventricular mass is fundamental for clinical diagnosis, risk assessment, therapeutic decisions, and prognosis in patients with cardiac disease. Although cardiac CT is a noninvasive imaging technique often used for the assessment of coronary artery disease, it can also be used to obtain important data about left and right ventricular function and morphology. In this review, we will discuss the clinical indications for the use of cardiac CT for ventricular analysis, review the evidence on the assessment of ventricular function compared with existing imaging modalities such cardiac magnetic resonance imaging and echocardiography, provide a typical cardiac CT protocol for image acquisition and postprocessing for ventricular analysis, and provide step-by-step instructions to acquire multiplanar cardiac views for ventricular assessment from the standard axial, coronal, and sagittal planes. Furthermore, both qualitative and quantitative assessments of ventricular function as well as sample reporting are detailed.

Steroid exposure, acute coronary syndrome, and inflammatory bowel disease: insights into the inflammatory milieu.

BACKGROUND: Steroids are anti-inflammatory agents commonly used to treat inflammatory bowel disease. Inflammation plays a critical role in the pathophysiology of both inflammatory bowel disease and acute coronary syndrome. We examined the relationship between steroid use in patients with inflammatory bowel disease and acute coronary syndrome. METHODS: In 177 patients with inflammatory bowel disease (mean age 67 years, 75% male, 44% Crohn's disease, 56% ulcerative colitis), we performed a 1:2 case-control study matched for age, sex, and inflammatory bowel disease type, and compared 59 patients with inflammatory bowel disease with acute coronary syndrome to 118 patients with inflammatory bowel disease without acute coronary syndrome. Steroid use was defined as current or prior exposure. Acute coronary syndrome was defined as myocardial infarction or unstable angina, confirmed by cardiac biomarkers and coronary angiography. RESULTS: In patients with inflammatory bowel disease, 34% with acute coronary syndrome had exposure to steroids, vs 58% without acute coronary syndrome (P < .01). Steroid exposure reduced the adjusted odds of acute coronary syndrome by 82% (odds ratio [OR] 0.39; 95% confidence interval [CI], 0.20-0.74; adjusted OR 0.18; 95% CI, 0.06-0.51) in patients with inflammatory bowel disease, 77% in Crohn's disease (OR 0.36; 95% CI, 0.14-0.92; adjusted OR 0.23; 95% CI, 0.06-0.98), and 78% in ulcerative colitis (OR 0.41; 95% CI, 0.16-1.04; adjusted OR 0.22; 95% CI, 0.06-0.90). There was no association between other inflammatory bowel disease medications and acute coronary syndrome. CONCLUSIONS: In patients with inflammatory bowel disease, steroid use significantly reduces the odds of acute coronary syndrome. These findings provide further mechanistic insight into the inflammatory processes involved in inflammatory bowel disease and acute coronary syndrome.

A look at statin cost-effectiveness in view of the 2013 ACC/AHA cholesterol management guidelines.

The 2013 cholesterol management guidelines presented a major shift in recommendations on which patients at risk of cardiovascular disease should be treated and how to treat them. Implementation of the guidelines is estimated to increase substantially the number of people who would be eligible for statin therapy. As the medical community considers the broad population impact of the new cholesterol guidelines, the issue of cost-effectiveness plays a role. This review covers the basic fundamentals of cost-effectiveness analysis and summarizes the key cost-effectiveness studies that relate to the new cholesterol guidelines.

Referral of patients with pulmonary hypertension diagnoses to tertiary pulmonary hypertension centers: the multicenter RePHerral study.

IMPORTANCE: Pulmonary hypertension (PH) is a fatal disease. Although the prognosis of pulmonary arterial hypertension (PAH) has improved with targeted therapies, the outcome is dependent on early detection and an accurate diagnosis. OBJECTIVE: To determine the accuracy of PH diagnoses in patients referred to PH centers and the frequency of PAH-specific medication use despite an uncertain or incorrect diagnosis. DESIGN: Multicenter, descriptive, cross-sectional study. During a 10-month period in 2010 and 2011, data on newly referred patients were collected and entered into a secure Internet database. SETTING: Three large tertiary PH centers. PARTICIPANTS: One hundred forty consecutive patients newly referred to PH centers were invited to participate, and all consented to do so. RESULTS: Of 140 patients referred with a mean age of 56 years, 95 (68%) were referred by cardiologists or pulmonologists and 86 (61%) had disease classified as World Health Organization functional class III or IV. Fifty-six of the prereferral diagnoses (40%) were PAH, 42 (30%) unknown, and 22 (16%) PH secondary to lung disease or hypoxia. Of the 98 patients who received a definitive diagnosis before referral, 32 (33%) received a misdiagnosis. Fifty-nine patients underwent catheterization of the right and/or left side of the heart for the first time at the tertiary center. Of the 38 patients who underwent catheterization of the right side alone, 14 (37%) received a different diagnosis after undergoing the procedure; of the 21 patients who underwent catheterization of both sides of the heart, 11 (52%) received a different diagnosis after undergoing the procedures. Forty-two patients (30%) had started receiving PAH-specific medications before referral, with 24 of the prescriptions (57%) contrary to published guidelines. CONCLUSIONS AND RELEVANCE: Patients referred to PH centers for diagnosis and treatment are often referred late (with functional class III or IV disease), receive misdiagnoses, and are inappropriately prescribed medications. A reevaluation of educational efforts is required to improve awareness and the care and outcome of patients diagnosed as having PH.

Lipid effects of antihypertensive medications.

Thiazide diuretics and beta-blockers are first-line therapies for hypertension unless there are compelling indications for other drug classes. Diuretics and beta-blockers, however, may worsen dyslipidemia and glucose tolerance whereas antihypertensive agents in other drug classes may have neutral or beneficial effects. Initial clinical trials of antihypertensive regimens suggested that blood pressure lowering was the most important aspect of therapy and that the adverse effects on lipids and glucose tolerance did not impact clinical outcomes. Newer trials, however, question this finding and implicate these pleotropic effects as contributing to the results of the trials. Patients with cardiometabolic risk factors may have compelling indications for agents that inhibit the renin-angiotensin-aldosterone system, relegating diuretics and beta-blockers to third-line therapy.