Guidelines for the Evaluation of Prosthetic Valve Function With Cardiovascular Imaging: A Report From the American Society of Echocardiography Developed in Collaboration With the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography.

In patients with significant cardiac valvular disease, intervention with either valve repair or valve replacement may be inevitable. Although valve repair is frequently performed, especially for mitral and tricuspid regurgitation, valve replacement remains common, particularly in adults. Diagnostic methods are often needed to assess the function of the prosthesis. Echocardiography is the first-line method for noninvasive evaluation of prosthetic valve function. The transthoracic approach is complemented with two-dimensional and three-dimensional transesophageal echocardiography for further refinement of valve morphology and function when needed. More recently, advances in computed tomography and cardiac magnetic resonance have enhanced their roles in evaluating valvular heart disease. This document offers a review of the echocardiographic techniques used and provides recommendations and general guidelines for evaluation of prosthetic valve function on the basis of the scientific literature and consensus of a panel of experts. This guideline discusses the role of advanced imaging with transesophageal echocardiography, cardiac computed tomography, and cardiac magnetic resonance in evaluating prosthetic valve structure, function, and regurgitation. It replaces the 2009 American Society of Echocardiography guideline on prosthetic valves and complements the 2019 guideline on the evaluation of valvular regurgitation after percutaneous valve repair or replacement.

End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.

BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

Quantification of Resting Myocardial Blood Flow Using Rubidum82 Positron Emission Tomography in Regions with MRI-Confirmed Myocardial Scar.

Background: Resting myocardial blood flow (rMBF) within regions of myocardial scar as measured by positron emission tomography (PET) has not yet been assessed with the radiotracer Rubidium82 (Rb82) or correlated with scar thickness. Cardiac magnetic resonance imaging (cMRI) offers high spatial resolution and identifies myocardial scar with late gadolinium enhancement (LGE). Using Rb82 PET, we sought to characterize rMBF in regions of myocardial scar of varying thicknesses determined by cMRI. Methods/Results: Patients with a history of myocardial infarction, a resting Rb82 PET study and a cMRI were identified. On cMRI, regions of infarction, defined as >50% LGE with akinesis, were sub-categorized as 50-75% LGE or >75% LGE, corresponding with increasing transmural scar thickness. PET zones of infarct based on size and %LGE by cMRI were quantified for mean and minimum rMBF. Mean rMBF (cc/min/g) in infarct zones with >75% LGE was 0.32±0.07 with a minimum rMBF of 0.19±0.03. In infarct zones with 50-75% LGE, rMBF was 0.45±0.14 (50-75% vs. >75%, p=0.002). Conclusions: We identified rMBF within cMRI confirmed regions of myocardial scar of varying thicknesses. rMBF has an inverse relationship with the extent of LGE on cMRI, with the most severe regions (>75% LGE) having mean and minimal rMBF (cc/min/g) of 0.32±0.07 and 0.19±0.03, respectively.

Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries.

BACKGROUND: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes. OBJECTIVES: The authors aimed to determine factors associated with survival in a large cohort of such individuals. METHODS: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS). RESULTS: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography. CONCLUSIONS: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk.

Profile of Destructive Ocular Surgery and its Indications: A Twelve-Year Review from Eastern Nepal.

BACKGROUND: Destructive ocular surgeries are performed for many conditions ranging from trauma to tumours, where the eyes cannot be salvaged. The objective of our research was to study the profile of destructive ocular surgery and their indications. METHODS: This retrospective study reviewed all patients who underwent evisceration, enucleation, and exenteration at B.P. Koirala Institute of Health Sciences, a tertiary eye hospital in Eastern Nepal, between January 2008 and December 2019. Medical records on patient demographics, type of surgery performed, and an indication of surgery during the study period were reviewed. RESULTS: One hundred thirty-four patients underwent destructive ocular surgeries. The median age of patients undergoing surgery was 14.5 (3-50) years. Children aged ten years or less accounted for 46.3% of the total patients. Fifty-two percent were male. The left eye was affected in more than half of the cases (56.7%). Enucleation was the most performed destructive ocular surgery (76 cases, 56.7%). Intraocular and ocular adnexal malignancy was the most common overall indication (62 cases, 46.3%). Ocular infection (19 cases, 41.3%) and trauma (15 cases, 32.6%) were the most common indication of evisceration. Retinoblastoma accounted for most cases of enucleation (43 cases, 56.6%). Malignancy was the only indication of exenteration (12 cases, 100%). CONCLUSIONS: Enucleation was the most common destructive ocular surgery. Malignancy accounted for most of the cases of destructive eye surgery, followed by ocular infection. Ocular infection and trauma were the most common indication of evisceration, whereas retinoblastoma and eyelid malignancy were responsible for most of the cases of enucleation and exenteration, respectively.

Orbital Lymphoma Masquerading as Orbital Cellulitis.

BACKGROUND: Orbital lymphomas are primarily non-Hodgkin type and can originate from the eyelids, extraocular muscles, soft tissue orbital adnexa, conjunctiva, or lacrimal glands. Orbital malignancies often represent a diagnostic dilemma for clinicians given their varying and atypical presentations. OBJECTIVE: To report a case of orbital lymphoma mimicking orbital cellulitis. CASE: A 66-year-old male patient presented with sudden onset of painful proptosis with visual impairment in the left eye for 15 days. On ocular examination, best-corrected visual acuity was 6/12 in the right eye and 2/60 in the left eye, abaxial proptosis with hypertropia, swollen and erythematous eyelids, restricted extraocular movement in all cardinal position of gaze, conjunctival congestion with chemosis and tortuous vessels, sluggish pupillary reaction, and chorioretinal folds in the inferior quadrants. The case was diagnosed as left eye orbital cellulitis, and the patient was treated with broad-spectrum intravenous antibiotics and oral steroids. No clinically discernible response was noted despite 7 days of antibiotics and steroids. Contrast-enhanced computed tomography (CECT) orbit showed features suggestive of orbital lymphoma involving the ipsilateral maxillary and ethmoid sinuses. ENT consultation with diagnostic nasal endoscopy and biopsy was done. Histopathological reports showed features of non-Hodgkin lymphoma. CONCLUSION: Orbital malignancies masquerading as orbital cellulitis can pose a diagnostic dilemma. A multidisciplinary approach involving ENT consultation, radiological investigation, and pathological sampling can help achieve a timely diagnosis and appropriate management.

Subconjunctival Mass as Rare Presentation of Even Rarer Intraocular Medulloepithelioma: A Case Report.

Medulloepithelioma is a rare childhood embryonal tumor arising from the non-pigmented ciliary epithelium of the pars plicata. We report a case of an 11-year-old male who presented with painless loss of vision of the right eye for the last three years and progressively increasing mass on the superior aspect of the globe for the last three months. On ocular examination, a firm, non-tender mass of 4cm x 3cm was noted in the superior aspect of the globe. CT-Scan of the orbit was suggestive of a foreign body with a haemorrhage or infection. The patient underwent enucleation with minimal manipulation. Histopathological examination of the enucleated globe revealed medulloepithelioma. The intraocular medulloepithelioma presentation is often late and masquerading, which may lead to extraocular extension and metastasis and ultimately unfavorable prognosis.

Impact of Preinfection Left Ventricular Ejection Fraction on Outcomes in COVID-19 Infection.

Coronavirus disease 2019 (COVID-19) has high infectivity and causes extensive morbidity and mortality. Cardiovascular disease is a risk factor for adverse outcomes in COVID-19, but baseline left ventricular ejection fraction (LVEF) in particular has not been evaluated thoroughly in this context. We analyzed patients in our state's largest health system who were diagnosed with COVID-19 between March 20 and May 15, 2020. Inclusion required an available echocardiogram within 1 year prior to diagnosis. The primary outcome was all-cause mortality. LVEF was analyzed both as a continuous variable and using a cutoff of 40%. Among 396 patients (67 ± 16 years, 191 [48%] male, 235 [59%] Black, 59 [15%] LVEF ≤40%), 289 (73%) required hospital admission, and 116 (29%) died during 85 ± 63 days of follow-up. Echocardiograms, performed a median of 57 (IQR 11-122) days prior to COVID-19 diagnosis, showed a similar distribution of LVEF between survivors and decedents (P = 0.84). Receiver operator characteristic analysis revealed no predictive ability of LVEF for mortality, and there was no difference in survival among those with LVEF ≤40% versus >40% (P = 0.49). Multivariable analysis did not change these relationships. Similarly, there was no difference in LVEF based on whether the patient required hospital admission (56 ± 13 vs 55 ± 13, P = 0.38), and patients with a depressed LVEF did not require admission more frequently than their preserved-LVEF peers (P = 0.87). A premorbid history of dyspnea consistent with symptomatic heart failure was not associated with mortality (P = 0.74). Among patients diagnosed with COVID-19, pre-COVID-19 LVEF was not a risk factor for death or hospitalization.

Optimizing cardiovascular imaging in Staphylococcus aureus endocarditis.

INTRODUCTION: The shift toward value-based health care drives physicians to examine opportunities to optimize use of healthcare resources. There is discordance between providers' use of cardiovascular imaging (CVI) in assessing patients for infective endocarditis (IE) with Staphylococcus aureus bacteremia (SAB). An evidence-based algorithm was created to minimize variation of CVI use. The primary objective was to ensure sensitivity of the algorithm to recommend CVI in patients suspected of IE. METHODS: A retrospective review evaluated patients at Ochsner Medical Center who developed SAB between 1/1/13 and 12/31/14. Predefined patient demographics, use of CVI, outcomes, and 12-week follow-up for readmission after first positive blood culture were collected from chart review. The created algorithm was applied retrospectively to determine its sensitivity and specificity in recommending the right CVI test. RESULTS: 181 patients admitted were admitted with SAB, of which 114 (63%) were male. There were 115 TTEs and 55 TEEs performed. Out of 15 patients diagnosed with IE, 3 were found on TTE and 12 were found on TEE. The algorithm would have recommended a TEE in all 15 patients who had high-risk features for IE and a true diagnosis of IE, suggesting a sensitivity of 100% and specificity of 74.7% for the algorithm to have recommended a highly sensitive CVI modality. CONCLUSION: This algorithm optimizes CVI for diagnosing IE in patients with SAB. As healthcare adapts to a value-based system, use of best-practice algorithms will promote consistency in practice among providers and help optimize patient outcomes and use of resources.

Dimensionless index of the mitral valve for evaluation of degenerative mitral stenosis.

PURPOSE: Degenerative mitral stenosis (DMS) is an increasingly recognized cause of mitral stenosis. The goal of this study was to compare echocardiographic differences between DMS and rheumatic mitral stenosis (RMS), identify echocardiographic variables reflective of DMS severity, and propose a dimensionless mitral stenosis index (DMSI) for assessment of DMS severity. METHODS: This is a single-center, retrospective cohort study. We included patients with at least mild MS and a mean transmitral pressure gradient (TMPG) ≥4 mm Hg. Mitral valve area by the continuity equation (MVACEQ ) was used as an independent reference. The DMSI was calculated as follows: DMSI = VTILVOT / VTIMV. All-cause mortality data were collected retrospectively. RESULTS: A total of 64 patients with DMS and 24 patients with RMS were identified. MVACEQ was larger in patients with DMS (1.43 ± 0.4 cm2 ) than RMS (0.9 ± 0.3 cm2 ) by ~0.5 cm2 (P = <.001), and mean TMPG was lower in the DMS group (6.0 ± 2 vs 7.9 ± 3 mm Hg, P = .003). A DMSI of ≤0.50 and ≤0.351 was associated with MVACEQ ≤1.5 and MVACEQ ≤1.0 cm2 (P < .001), respectively. With the progression of DMS from severe to very severe, there was a significant drop in DMSI. There was a nonsignificant trend toward worse survival in patients with MVACEQ ≤1.0 cm2 and DMSI ≤0.35, suggesting severe stenosis severity. CONCLUSION: Our results show that TMPG correlates poorly with MVA in patients with DMS. Proposed DMSI may serve as a simple echocardiographic indicator of hemodynamically significant DMS.

Left Atrial Posterior Wall Thrombus After Posterior Wall Ablation.

Background: Posterior wall isolation for recurrent atrial arrhythmia is a commonly used technique to achieve long-term freedom from atrial fibrillation. Despite the widespread use of posterior wall isolation, its long-term effects on left atrial function are unknown. Specifically, the effect of isolated atrial walls on stasis and risk of thrombus has not been established. We present the case of a patient who developed a left atrial posterior wall thrombus after a posterior wall isolation attempt. Case Report: A 67-year-old female with a complex electrophysiologic history was found to have a left atrial posterior wall thrombus when she presented for a third ablation attempt for drug-refractory macroreentrant left atrial tachycardia 5 weeks after a posterior wall isolation attempt. The patient had a number of risk factors that could have been associated with the unusually located thrombus: hypertension, low ejection fraction, mitral valve disease, and recurrence and sustained duration of symptomatic atrial fibrillation. After the patient had 3 weeks of anticoagulation treatment, transesophageal echocardiography showed no left atrial thrombus, and she underwent successful reisolation of the posterior wall. The third ablation was successful, and the patient developed no complications of stroke, transient ischemic attack, or systemic embolization throughout her treatment course. Conclusion: To our knowledge, this case is the second report of a left atrial posterior wall thrombus in this setting. The patient's complex and specific set of risk factors likely led to this rare finding. Although left atrial posterior wall thrombus after ablation is rare, in patients with specific risks or a combination of factors that could lead to such a clot, visualizing the left atrium in these patients may be beneficial to minimize the risk of systemic embolization.

Pregnancy Management in a Patient With Congenital Heart Disease and Severe Right Ventricle to Pulmonary Artery Conduit Stenosis.

Background: Pregnancy causes multiple hemodynamic changes that place significant stress on the cardiovascular system. With advancements in medical care, individuals with complex congenital heart disease are living into their childbearing years. Much remains to be understood about the effects and management of pregnancy in individuals with complex congenital heart disease. Case Report: We describe the management and delivery of a 29-year-old pregnant female with repaired tetralogy of Fallot or ventricular septal defect with pulmonary atresia. The patient presented at 21 weeks' gestation with New York Heart Association class II symptoms and pulmonary conduit stenosis, with a mean gradient of 52 mmHg. At 36.5 weeks' gestation, she developed severe pulmonary conduit stenosis with a mean gradient of >75 mmHg. The patient was admitted at 37 weeks' gestation for planned delivery. After a successful cesarean section and bilateral tubal ligation, the patient had an uncomplicated postoperative course. She was scheduled for follow-up for severe conduit stenosis at 6 weeks postpartum to discuss management options. Conclusion: Management of a pregnant patient with adult congenital heart disease should involve risk stratification for complications (commonly congestive heart failure exacerbation and arrhythmias) using tools such as the modified World Health Organization pregnancy risk classification. Based on the risk category, decisions must be made about frequency of follow-up, anesthesia, and mode of delivery. Patients in moderate to high-risk stratification should be managed by a multidisciplinary team at a specialty center, and all patients should undergo an anesthesia consultation prior to delivery. The decision for vaginal or cesarean delivery should be made on a case-by-case basis with consideration given to patient preference. Patients with asymptomatic moderate to severe pulmonic stenosis can be managed conservatively with appropriate follow-up and cardiac imaging, allowing intervention to be completed after delivery.

First in human: the effects of biventricular pacing on cardiac output in severe pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) carries high morbidity and mortality despite available treatment options. In severe PAH, right ventricular (RV) diastolic pressure overload leads to interventricular septal bowing, hindering of left ventricular diastolic filling and reduced cardiac output (CO). Some animal studies suggest that pacing may mitigate this effect. We hypothesized that eliminating late diastole via ventricular pacing could improve CO in human subjects with severe PAH. Using minimal to no sedation, we performed transvenous acute biventricular (BiV) pacing and right heart catheterization in six patients with symptomatic PAH. Hemodynamic measurements were taken at baseline and during BiV pacing at various 20-ms intervals of V-V timing. We compared baseline CO to (1) CO while pacing the RV first by 80 ms (mimicking RV-only pacing), and then to (2) CO during pacing at the V-V timing that resulted in the highest CO. All participants were female, PASP 74 ± 14 mmHg, QRS duration 104 ± 20 ms. Compared with baseline, the CO decreased when the RV was paced first by 80 ms (7.2 ± 1.0 vs. 6.2 ± 1.1 L/min, p = 0.028). Pacing with optimal V-V timing produced CO similar to baseline (7.2 ± 1.0 vs. 7.4 ± 1.4, p = 0.92). Two patients (33%) met the predefined endpoint of a 15% increase in CO during pacing at the optimal V-V timing. In symptomatic PAH, V-V optimized acute BiV pacing does not consistently improve CO. However, acute BiV pacing did improve CO in a subset of this cohort. Further research is needed to identify predictors of response to cardiac resynchronization therapy in this population.

Impact of appropriate use criteria for transesophageal echocardiograms on clinically meaningful care.

INTRODUCTION: The aim of this study was to evaluate appropriateness of transesophageal echocardiography (TEE) studies based on 2011 Appropriate Use Criteria (AUC) for Echocardiography and its impact on patient management. METHODS AND RESULTS: In a retrospective analysis of 100 inpatient TEEs, 85% of the TEEs ordered were determined to be appropriate, 9% were inappropriate, and 6% were uncertain. Inter-observer differences in the determination of AUC were seen in 24% of the studies, requiring a senior-level cardiologist to make the final determination of AUC score. The variance in interpretation by experts is concerning for how that might translate into differences in clinical practice. Of all TEEs, only 63% led to an active change in care, including changes in medications or procedures, while 37% did not. We found a statistically significant difference between cardiologists' and non-cardiologists' orders for TEE having an impact on patient's clinical care (41% vs 22%, respectively, P < 0.05) (Table ). While not statistically significant, a trend toward clinical change was observed in the appropriate vs inappropriate TEEs (70% vs 44%, respectively, P = 0.06). CONCLUSION: Ideally, the role of a diagnostic cardiovascular imaging test, such as a TEE, is to influence clinical care, if it is ordered appropriately on the right patient. While the AUC guides clinicians on the appropriate use of cardiovascular imaging, it is broadly written and offers room for interpretation to encompass variety of clinical scenarios. Clinical care paths that utilize AUC and standardize use of multidisciplinary institutional resources offer opportunity for optimal clinical impact and patient care.

Echocardiography in Congenital Heart Disease.

As the prevalence of adult congenital heart disease continues to grow secondary to advances in surgical and diagnostic techniques, it is important for a physician to supplement their examinations with non-invasive imaging techniques to assess their patients. Although a number of these patients have regular cardiology followup, some may be new patients that do not even know their cardiac history. Echocardiography has proven to be a useful tool for this purpose and its utility has expanded drastically with the development of better technology and newer techniques. In this article, we highlight some of these advancements including 2D echocardiography, agitated saline, contrast echocardiography, stress, and 4D, in addition to how each modality can help assess key aspects of the structure and function of a congenital heart defect.

Prognostic Implications of Left Ventricular Hypertrophy.

Left ventricular hypertrophy (LVH) was one of the earliest studied echocardiographic characteristics of the left ventricle. As the myriad of measurable metrics has multiplied over recent years, this reliable and relevant variable can often be overlooked. In this paper, we discuss appropriate techniques for accurate analysis, underlying pathophysiology, and the contributions from various risk factors. The prognostic implications of LVH on stroke, serious arrhythmias, and sudden cardiac death are reviewed. Finally, we examine the effect of therapy to reduce LVH and the resultant clinical outcomes.