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Graphical abstract, Highlights • Cor triatriatum dexter (CTD) can complicate percutaneous atrial septal interventions. • Clinically silent (forme fruste) CTD is detectable by new echocardiography techniques. • Using these techniques could improve patient selection and guide interventions.

Radiation treatment of thoracic malignancies is linked with development of valvular disease years after exposure. Given the challenges to surgical treatment related to previous radiation exposure, TAVR has emerged as an alternative. However, outcomes data for patients with history of mediastinal

Myxedema coma can mask cardiac causes of hypotension. Careful assessment is needed once initial therapy fails. A 43-year-old woman with untreated hypothyroidism presented with hypotension, respiratory distress, and somnolence after 6 months of progressive dyspnea and weight gain. She was

The American College of Cardiology Foundation (ACCF), in partnership with key specialty and subspecialty societies, conducted a review of common clinical scenarios where noninvasive vascular testing (venous ultrasound and physiological testing) is frequently considered. The indications (clinical

Peter Alagona, MD[⁎][1] Gerard Aurigemma, MD[‡][2] Javed Butler, MD, MPH[§][3] Don Casey, MD, MPH, MBA[∥][4] Ricardo Cury, MD[#][5] Scott Flamm, MD[¶][6] Tim Gardner, MD[⁎⁎][7] Rajesh Krishnamurthy, MD[††][8] Joseph Messer, MD[⁎][1] Michael W. Rich, MD[‡‡][9] Henry

Steven R. Bailey, MD, FACC, FSCAI, FAHA, Moderator Andrea M. Russo, MD, FACC, FHRS, Writing Group Liaison [⁎][1] Suraj Kapa, MD, Writing Group Liaison Michael B. Alexander, MD, FACC[§][2] Steven R. Bailey, MD, FACC, FSCAI, FAHA[∥][3] Ulrika Birgersdotter-Green, MD, FHRS[∥][3] Alan S.

The past several decades have seen rapid and extensive changes in the practice of cardiology, especially in the innovation and utilization practices of imaging, interventional, and electrophysiology procedures. Enhanced radionuclide imaging techniques, evolution of echocardiography, development of

The American College of Cardiology Foundation (ACCF), Society for Cardiovascular Angiography and Interventions, Society of Thoracic Surgeons, and the American Association for Thoracic Surgery, along with key specialty and subspecialty societies, conducted an update of the appropriate use criteria (AUC) for coronary revascularization frequently considered. In the initial document, 180 clinical scenarios were developed to mimic patient presentations encountered in everyday practice and included information on symptom status, extent of medical therapy, risk level as assessed by noninvasive testing, and coronary anatomy. This update provides a reassessment of clinical scenarios the writing group felt to be affected by significant changes in the medical literature or gaps from prior criteria. The methodology used in this update is similar to the initial document, and the definition of appropriateness was unchanged. The technical panel scored the clinical scenarios on a scale of 1 to 9. Scores of 7 to 9 indicate that revascularization is considered appropriate and likely to improve patients' health outcomes or survival. Scores of 1 to 3 indicate revascularization is considered inappropriate and unlikely to improve health outcomes or survival. Scores in the mid-range (4 to 6) indicate a clinical scenario for which the likelihood that coronary revascularization will improve health outcomes or survival is uncertain. In general, as seen with the prior AUC, the use of coronary revascularization for patients with acute coronary syndromes and combinations of significant symptoms and/or ischemia is appropriate. In contrast, revascularization of asymptomatic patients or patients with low-risk findings on noninvasive testing and minimal medical therapy are viewed less favorably. The technical panel felt that based on recent studies, coronary artery bypass grafting remains an appropriate method of revascularization for patients with high burden of coronary artery disease (CAD). Additionally, percutaneous coronary intervention may have a role in revascularization of patients with high burden of CAD. The primary objective of the appropriate use criteria is to improve physician decision making and patient education regarding expected benefits from revascularization and to guide future research.

Recent advances in the field of left ventricular device support have led to an increased use of left ventricular assist devices (LVADs) in patients with end stage heart disease. The primary imaging modality to monitor patients with LVADs has been echocardiography. The purpose of this review is to highlight the clinical role of echo and other noninvasive imaging modalities in the assessment of cardiac structure and function in patients with pulsatile and continuous flow LVADs. In addition, we discuss the role of imaging with emphasis on echo to detect LVAD dysfunction and device related complications.

Michael H. Picard, MD, FASE, David Adams, RDCS, FASE, S. Michelle Bierig, RDCS, MPH, FASE, JohnM.Dent,MD,FASE, Pamela S.Douglas,MD,FASE,LindaD.Gillam,MD,FASE,AndrewM.Keller,MD,FASE, David J. Malenka, MD, FASE, Frederick A. Masoudi, MD, MSPH, Marti McCulloch, RDCS, FASE, Patricia A. Pellikka, MD, FASE, Priscilla J. Peters, RDCS, FASE, Raymond F. Stainback, MD, FASE, G.Monet Strachan, RDCS, FASE, andWilliam A. Zoghbi,MD, FASE, Boston, Massachusetts; Durham,North Carolina; St. Louis, Missouri; Charlottesville, Virginia; New York, New York; Danbury, Connecticut; Lebanon, New Hampshire; Denver, Colorado; Houston, Texas; Rochester, Minnesota; Pennsauken, New Jersey; San Diego, California

The American College of Cardiology Foundation along with key specialty and subspecialty societies, conducted an appropriate use review of common clinical presentations for stable ischemic heart disease (SIHD) to consider use of stress testing and anatomic diagnostic procedures. This document reflects an updating of the prior Appropriate Use Criteria (AUC) published for radionuclide imaging (RNI), stress echocardiography (Echo), calcium scoring, coronary computed tomography angiography (CCTA), stress cardiac magnetic resonance (CMR), and invasive coronary angiography for SIHD. This is in keeping with the commitment to revise and refine the AUC on a frequent basis. A major innovation in this document is the rating of tests side by side for the same indication. The side-by-side rating removes any concerns about differences in indication or interpretation stemming from prior use of separate documents for each test. However, the ratings were explicitly not competitive rankings due to the limited availability of comparative evidence, patient variability, and range of capabilities available in any given local setting. The indications for this review are limited to the detection and risk assessment of SIHD and were drawn from common applications or anticipated uses, as well as from current clinical practice guidelines. Eighty clinical scenarios were developed by a writing committee and scored by a separate rating panel on a scale of 1 to 9, to designate Appropriate, May Be Appropriate, or Rarely Appropriate use following a modified Delphi process following the recently updated AUC development methodology. The use of some modalities of testing in the initial evaluation of patients with symptoms representing ischemic equivalents, newly diagnosed heart failure, arrhythmias, and syncope was generally found to be Appropriate or May Be Appropriate, except in cases where low pre-test probability or low risk limited the benefit of most testing except exercise electrocardiogram (ECG). Testing for the evaluation of new or worsening symptoms following a prior test or procedure was found to be Appropriate. In addition, testing was found to be Appropriate or May Be Appropriate for patients within 90 days of an abnormal or uncertain prior result. Pre-operative testing was rated Appropriate or May Be Appropriate only for patients who had poor functional capacity and were undergoing vascular or intermediate risk surgery with 1 or more clinical risk factors or an organ transplant. The exercise ECG was suggested as an Appropriate test for cardiac rehabilitation clearance or for exercise prescription purposes. Testing in asymptomatic patients was generally found to be Rarely Appropriate, except for calcium scoring and exercise testing in intermediate and high-risk individuals and either stress or anatomic imaging in higher-risk individuals, which were all rated as May Be Appropriate. All modalities of follow-up testing after a prior test or percutaneous coronary intervention (PCI) within 2 years and within 5 years after coronary artery bypass graft (CABG) in the absence of new symptoms were rated Rarely Appropriate. Pre-operative testing for patients with good functional capacity, prior normal testing within 1 year, or prior to low-risk surgery also were found to be Rarely Appropriate. Imaging for an exercise prescription or prior to the initiation of cardiac rehabilitation was Rarely Appropriate except for cardiac rehabilitation clearance for heart failure patients.

Sinus of Valsalva aneurysms appear to be rare. They occur most frequently in the right sinus of Valsalva (52%) and the noncoronary sinus (33%). More of these aneurysms originate from the right coronary cusp than from the noncoronary cusp. Surgical intervention is usually recommended when symptoms become evident. We report the case of a 34-year-old woman who presented with a congenital, ruptured sinus of Valsalva aneurysm that originated from the noncoronary cusp. Moderate aortic regurgitation was associated with this lesion. Simple, direct patch closure of the ruptured aneurysm resolved the patient's left-to-right shunt and was associated with decreased aortic regurgitation to a degree that valve replacement was not necessary. Only trace residual aortic regurgitation was evident after 3 months, and the patient remained free of symptoms after 6 months. Our observations support the idea that substantial runoff blood flow in the immediate supra-annular region can be responsible for aortic regurgitation in the absence of a notable structural defect in the aortic valve, and that restoring physiologic flow in this region and equalizing aortic-cusp closure pressure can largely or completely resolve aortic insufficiency. Accordingly, valve replacement may not be necessary in all cases of ruptured sinus of Valsalva aneurysms with associated aortic valve regurgitation.

Objectives.This study sought to determine the prognostic yield and utility of transesophageal echocardiography in critically ill patients with unexplained hypotension.Background.Transesophageal echocardiography is increasingly utilized in the intensive care setting and is particularly suited for the evaluation of hypotension; however, the prognostic yield of transesophageal echocardiography in these patients is unknown.Methods.We prospectively studied 61 adult patients in the intensive care unit with sustained (>60 min) unexplained hypotension. Both transthoracic and transesophageal echocardiography were performed, and results were immediately disclosed to the primary physician, who reported any resulting changes in management. Patients were classified on the basis of transesophageal echocardiographic findings into one of three prognostic groups: 1) nonventricular (valvular, pericardial) cardiac limitation to cardiac output; 2) ventricular failure; and 3) noncardiac systemic disease (hypovolemia or low systemic vascular resistance, or both). Primary end points were death or discharge from the intensive care unit.Results.A transesophageal echocardiographic diagnosis of nonventricular limitation to cardiac output was associated with improved survival to discharge from the intensive care unit (81%) versus a diagnosis of ventricular disease (41%) or hypovolemia/ low systemic vascular resistance (44%, p = 0.03). Twenty-nine (64%) of 45 transthoracic echocardiographic studies were inadequate compared with 2 (3%) of 61 transesophageal echocardiographic studies (p < 0.001). Transesophageal echocardiography contributed new clinically significant diagnoses (not seen with transthoracic echocardiography) in 17 patients (28%), leading to operation in 12 (20%).Conclusions.Transesophageal echocardiography makes a clinically important contribution to the diagnosis and management of unexplained hypotension and predicts prognosis in the critical care setting.

Exposing the heart to external beam radiotherapy (EBRT) increases the risk of heart disease sevenfold. We report 23 patients with previous EBRT and critical aortic stenosis (AS) who were treated with transcatheter aortic valve implantation (TAVI). Of the 424 consecutive patients who underwent TAVI

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Isolated ventricular myocardial noncompaction is a cardiomyopathy that is being diagnosed more frequently in patients of all ages because of increased awareness and improvements in imaging methods. It is an extremely rare cause of heart failure in nonagenarians. We describe the case of a man who presented with heart failure for the first time at 90 years of age. The diagnosis was isolated left ventricular noncompaction. Transthoracic echocardiography showed a trabeculated, sponge-like appearance of the left ventricular apical and inferolateral segments. After medical management, the patient was asymptomatic at the 3-month follow-up examination. Knowledge of ventricular noncompaction is increasing within the cardiology community. Patients who have isolated noncompaction with a limited number of involved ventricular segments can live beyond normal life expectancy without developing heart failure. In addition to discussing our patient's case, we briefly review the relevant medical literature.

The American College of Cardiology Foundation, along with key specialty and subspecialty societies, conducted an appropriate use review of common clinical scenarios where cardiac computed tomography (CCT) is frequently considered. The present document is an update to the original CCT/cardiac magnetic resonance appropriateness criteria published in 2006, written to reflect changes in test utilization, to incorporate new clinical data, and to clarify CCT use where omissions or lack of clarity existed in the original criteria.1 The indications for this review were drawn from common applications or anticipated uses, as well as from current clinical practice guidelines. Ninety-three clinical scenarios were developed by a writing group and scored by a separate technical panel on a scale of 1 to 9 to designate appropriate use, inappropriate use, or uncertain use. In general, use of CCT angiography for diagnosis and risk assessment in patients with low or intermediate risk or pretest probability for coronary artery disease was viewed favorably, whereas testing in high-risk patients, routine repeat testing, and general screening in certain clinical scenarios were viewed less favorably. Use of noncontrast computed tomography for calcium scoring was rated as appropriate within intermediate- and selected low-risk patients. Appropriate applications of CCT are also within the category of cardiac structural and functional evaluation. It is anticipated that these results will have an impact on physician decision making, performance, and reimbursement policy, and that they will help guide future research. In an effort to respond to the need for the rational use of imaging services in the delivery of high-quality care, the ACCF has undertaken a process to determine the appropriate use of cardiovascular imaging for selected patient indications. Appropriate use criteria publications reflect an ongoing effort by the ACCF to critically and systematically create, review, and categorize clinical situations where diagnostic tests and procedures …

Contrast echocardiography is recognized to be a safe, effective technique for evaluating the endocardial border and left ventricular function in patients who have suboptimal non-contrast echocardiograms. However, its use in diagnosing right-heart conditions is less well established. Herein, we report our experience with the use of contrast echocardiography for diagnosing 3 distinct right-sided heart conditions (hypokinesis of the right ventricular free wall, severe tricuspid regurgitation, and cardiac tamponade) in patients who had suboptimal echocardiograms. Further studies should be done to validate the use of contrast echocardiography for diagnosing right-heart conditions.

The American College of Cardiology Foundation (ACCF) and the American Society of Echocardiography (ASE) together with key specialty and subspecialty societies, conducted an appropriateness review for stress echocardiography. The review assessed the risks and benefits of stress echocardiography for several indications or clinical scenarios and scored them on a scale of 1 to 9 (based upon methodology developed by the ACCF to assess imaging appropriateness). The upper range (7 to 9) implies that the test is generally acceptable and is a reasonable approach, and the lower range (1 to 3) implies that the test is generally not acceptable and is not a reasonable approach. The midrange (4 to 6) indicates a clinical scenario for which the indication for a stress echocardiogram is uncertain. The indications for this review were drawn from common applications or anticipated uses, as well as from current clinical practice guidelines. Use of stress echocardiography for risk assessment in patients with coronary artery disease (CAD) was viewed favorably, while routine repeat testing and general screening in certain clinical scenarios were viewed less favorably. It is anticipated that these results will have a significant impact on physician decision making and performance, reimbursement policy, and will help guide future research.

Echocardiography is a safe, noninvasive, and widely available method that often provides a definitive anatomic and hemodynamic diagnosis and guides medical management. Although long viewed as clinically “mature,” echocardiography has undergone further “revolutionary” advances during the past 10 years, including not only technologic breakthroughs but also new clinical applications for older techniques. The recent explosion of potential “add-on” techniques presents echocardiographers with challenging questions about what constitutes real clinical progress and what techniques should be incorporated into the standard examination protocol. By becoming familiar with their clinical indications, echocardiographers can use these techniques selectively, on an “as-needed” basis, and thus streamline patient care. Because of increased availability and clinical utility, echocardiography’s usage has greatly expanded in recent years. However, echocardiography demands a major commitment in terms of time, personnel, ongoing training, and technology. An understanding of the physics of ultrasound is essential for performing echocardiography examinations and interpreting their results. A comprehensive transthoracic echocardiography examination includes systematic acquisition of a set of twodimensional and M-mode views, along with spectral Doppler and color Doppler evaluation of the intrathoracic cardiovascular structures (e.g., the myocardium, cardiac valves, pericardium, and great vessels). Transthoracic and transesophageal echocardiography are frequently used as complementary imaging techniques. Accordingly, the advantages and disadvantages of these two methods should be understood. Newer imaging modalities that are being introduced into the standard examination include contrast echocardiography, harmonic imaging modalities, parametric imaging modes, and selected three-dimensional techniques. Echocardiography examinations using standardized and novel imaging modes are frequently applied to a wide range of new clinical treatments, including assessment of left ventricular assist devices. In this growing field, important quality assurance measures have evolved along with imaging techniques to improve the quality of delivered care.

Echocardiography is the primary tool for the noninvasive detection, quantitation, and follow-up assessment of valvular heart disease. No single echocardiography method can be considered a robust “gold standard” for quantification of lesion severity in all situations. Valve lesion severity is often diagnosed by a synthesis of several anatomic, qualitative, and quantitative echocardiography severity indicators. For best practice, laboratories should routinely obtain as many severity parameters as possible, so that adequate scanning and interpretation skills are maintained for accurate diagnoses in difficult cases. Transesophageal echocardiography (TEE) may be needed in conjunction with the transthoracic exam (TTE) for a definitive diagnosis.

Tumors of the heart are uncommon, and they present in diverse and challenging ways. The heart may be the site of a primary tumor or may be invaded secondarily by malignancies that arise in adjacent or remote organs. Tumors—more frequently carcinomas than sarcomas—that involve the heart, pericardium, or both, originate as primary tumors in some other organ 20 to 40 times more often than they originate as primary tumors of the heart. Although less common than secondary tumors, primary tumors of the heart are far more challenging to both the cardiologist and the surgeon. These tumors usually present as intracavitary lesions. Although more than 75% of them are benign, clinical manifestations are common. Intracardiac myxomas are the most common benign tumors of the heart, constituting nearly 50% of all such tumors. Most patients with cardiac myxomas present with one or more effects of a triad of constitutional, embolic, and obstructive manifestations. Systemic tumor embolization occurs in 40% to 50% of patients with left atrial myxomas. Tumor fragments or surface clots most commonly embolize to arteries in the brain, kidneys, and extremities. Histologic examination of emboli recovered from a peripheral artery at operation can lead to diagnosis of an otherwise unsuspected intracardiac myxoma. Tumor embolization to the central nervous system constitutes about half of all embolic events caused by left atrial myxomas. Rhabdomyomas are the most frequent cardiac tumors in infants and children (usually males). These lesions usually present early in life and cause a variety of manifestations, including arrhythmias and sudden death. Lipomatous hypertrophy of the atrial septum is an unencapsulated hyperplasia of adipose tissue and may not represent a true tumor. Caseous mitral annular calcification is a more recently recognized unusual variant of mitral annular calcification that is generally clinically benign. However, its striking incidental appearance on echocardiography can lead to mistaken diagnosis of a tumor or an abscess. Mesotheliomas (lymphangioepitheliomas) of the atrioventricular node are the smallest tumors capable of producing sudden death by causing complete heart block. Recurrent, nonbloody pericardial effusion is common in children with a teratoma of the pericardium. For metastatic lesions, no particular type of malignant tumor predominates except possibly malignant melanoma, which involves the myocardium in more than 50% of cases.

From April 2000 through September 2001, we studied 11 patients with the Jarvik 2000--a left ventricular assist device with an axial-flow pump that provides continuous blood flow--to determine the echocardiographic characteristics. All patients underwent complete echocardiographic examination, including outflow-graft flow evaluation 24 hours after implantation and each month thereafter for the duration of support. Data were obtained at each pump setting (8000-12000 rpm in 1000-rpm increments) and with the pump off. Left ventricular dimensions and shortening fraction and the duration of aortic valve systolic opening decreased as pump speed increased. Although the aortic valve remained closed at higher pump speeds, pump outflow-graft flow remained pulsatile, because of the systolic thrust of the assisted ventricle. Systolic dominance of phasic flow was more pronounced at lower pump speeds, due to normalization of the diseased heart's Starling response. When the aortic valve was closed continuously, echocardiographic contrast (indicating blood stasis) was noted in the aortic root. Because of the pump outflow graft's proximity to the chest wall, device output could be measured independently of cardiac contributions. Mean peak outflow-graft flow velocities were 0.75 +/- 0.30 m/s (systolic) and 0.41 +/- 0. 13 m/s (diastolic). When the pump was turned off briefly there was minimal regurgitation through the device into the left ventricle. This 1st echocardiographic heart function analysis of the Jarvik 2000 confirms that the device unloads the ventricle and increases cardiac output. Cardiac responses to device-speed changes can be evaluated readily with echocardiography in the early and late postoperative period.

Background The bileaflet valve is currently the mechanical replacement valve of choice. Though durable, it does not closely mimic native valve hemodynamics and remains potentially thrombogenic. Methods Prototype trileaflet valves (T1 and T2) were implanted in the mitral position in calves. Group I calves received either a T1 valve (n = 12) or a control bileaflet valve (n = 5); Group II, either a T2 valve (n = 7) or a control bileaflet valve (n = 5). Valve function, perivalvular leakage, and transvalvular pressure gradients were evaluated. Also, long-term prototype leaflet wear was evaluated in vivo in one Group I calf (502 days) and two Group II calves (385 and 366 days). Calves were euthanized and necropsied at study termination, and major organs weighed and examined. Results Valve function was excellent and hematologic parameters remained normal in all calves that survived to study termination. Mean peak transvalvular pressure gradients were 10 ± 7 mm Hg for T1 valves, 6 ± 3 mm Hg for T2 valves, and 12 ± 4 mm Hg for bileaflet control valves. Clinically insignificant valvular regurgitation was observed in both prototypes. Explanted valves showed no thrombus-impaired leaflet motion, except in two T1-fitted calves and one T2-fitted calf. Major organs showed no evidence of clinically significant thromboembolic events. There were no other significant differences between the results of experimental and control groups. Conclusions Prototype trileaflet valves performed safely and effectively in the mitral position in calves, even without long-term anticoagulation. This warrants their evaluation as an equivalent alternative to bileaflet valves.

A 21-year-old man presented with syncope and a heart murmur. Screening echocardiography revealed severe asymmetric septal hypertrophic cardiomyopathy (thickness, 3.7 cm). No resting or provocable left ventricular outflow obstruction was present. Because the patient was not an athlete and performed no regular …