Multimodality imaging for the diagnosis of left ventricular apical hypoplasia: A case presentation and review of the literatures.

Left ventricular apical hypoplasia is a rare malformation recently described congenital abnormality characterized by: (1) truncation of the left ventricle, with the septum projecting toward the right ventricle; (2) abnormal papillary muscle originating from the flattened left ventricular apex; (3) a narrow right ventricle encompassing the periapical area of the left ventricle; (4) fatty infiltration of the apex of the left ventricle. We reported a case of LVAH and reviewed the patient's clinical presentation. And its morphologic characteristics were revealed by multimodality imaging, including echocardiography and cardiac magnetic resonance imaging. Additionally, we reviewed 41 cases from 32 reports to summarize the pathogenesis and analyzed the imaging manifestations of LVAH in this study, aiming to provide new ideas for the diagnosis and clinical management of LVAH patients.

Contrast Echocardiography in Heart Failure: Update 2023.

PURPOSE OF REVIEW: The application of ultrasound-enhancing agents (contrast agents) has improved the accuracy and reproducibility of echocardiography. The review focuses on the currently approved and evolving indications for contrast echocardiography in patients with heart failure, specifically examining clinical studies conducted after the publication of the guidelines in 2017 and 2018. RECENT FINDINGS: The current ASE/EACVI recommendations for contrast echocardiography are based on its accuracy and reproducibility in comparison to non-enhanced echocardiography or other imaging modalities like cardiac MRI. However, tissue characterization remains limited with contrast echocardiography. During the last few years, several studies have demonstrated the clinical impact of using contrast agents on the management of patients with heart failure. There is growing evidence on the benefit of using contrast echocardiography in critically ill patients where echocardiography without contrast agents is often suboptimal and other imaging methods are less feasible. There is no risk of worsening renal function after the administration of ultrasound-enhancing agents, and these agents can be administered even in patients with end-stage renal disease. Contrast echocardiography has become a valuable tool for first-line imaging of patients with heart failure across the spectrum of patients with chronic heart failure to critically ill patients.

The value of ultrasound enhancing agents in the echocardiographic acquisition of pulmonary artery systolic pressure: An invasive to non-invasive correlation study.

PURPOSE: Right heart catheterization (RHC) is the gold standard for the assessment of pulmonary artery systolic pressures (PASP). Despite high utilization of echocardiography for the non-invasive assessment of PASP, the data comparing real-time non-invasive echocardiographic PASP with invasive PASP is limited. Furthermore, evidence regarding the utility and diagnostic accuracy of ultrasound enhancing agents (UEA) for non-invasive PASP assessment is lacking. To evaluate the accuracy of non-invasive PASP assessment with real-time invasive measures and the incremental benefit of UEA in this setting. METHODS: This was a prospective cohort study of 90 patients, undergoing clinically indicated RHC for hemodynamic assessment. All patients underwent a limited echocardiogram during RHC. Tricuspid regurgitant velocity (TRV) was measured on unenhanced echo, in the setting of centrally administrated agitated saline, then as either centrally administered or peripherally administered UEA. RESULTS: Of the 90 patients enrolled in our study, 41% had pulmonary hypertension. The overall mean PASP measured by RHC was 32.8 mmHg (+/- 11.3 mmHg). Unenhanced echocardiograms had a moderate correlation with invasive PASP (r = 0.57; p = < 0.001) which improved to a strong correlation with administration of agitated saline (r = 0.75; p = < 0.001) or centrally administered UEA (r = 0.77; p = < 0.001), with the best correlation noted with peripherally administered UEA (r = 0.83; p = < 0.001). Against invasive PASP, agitated saline enhanced PASP had the lowest bias (0.12mmHg; -15.6 to 15.8mmHg) when compared with all other non-invasive measures of PASP. CONCLUSIONS: Unenhanced echocardiographic estimation of TRV was found to have a poorer correlation with invasively measured PASP when compared to agitated saline and centrally administered UEA. Agitated saline enhanced PASP demonstrated the lowest bias with invasive PASP when compared to other non-invasive measures of PASP.

Efficacy and safety of use of ultrasound enhancing agent in patients hospitalized with COVID-19.

PURPOSE: The efficacy and safety of ultrasound enhancing agent (UEA) was unknown in the COVID-19 hospitalized patients. We set out to establish the utility of UEA and its safety profile. METHODS: A retrospective observational study of prospectively assessed hospitalized patients referred for transthoracic echocardiography (TTE) for suspected cardiac pathology due to COVID-19. The indications and subsequent ability to answer the indications for all TTE were reviewed, as well as impact on diagnosis and management. UEA safety was considered through 48 h mortality. RESULTS: From a total of 364 patients (mean age 64.8yrs, 64% males) hospitalized with COVID-19 with TTE requested, an indication could be identified in 363, and 61 required administration of UEA. Standard TTE was able to answer the original indication in 275 (75.8%) patients. This was increased to 322 (88.7%) patients, a relative increase of 17.1%, with the use of UEA (p < 0.001). There was subsequent change in diagnosis in 22 out of 61 (36%) patients receiving UEA and change in management in 13 out of 61 (21.3%). There was no significant increase in 48 h (p = 0.14) mortality with UEA use. The patient population of TTE with UEA versus TTE without UEA differed in having a higher incidence of left ventricular systolic dysfunction, right ventricular dilatation, and self-defined white ethnicity. CONCLUSION: The use of UEA in COVID-19 hospitalized patients, including those who were critically ill, provided incremental information when compared to TTE without UEA resulting in both changes in diagnosis and management plan and appears to be safe.

Ultrasound-Enhancing Agent Safely Enhances Left Ventricular Visualization by Transthoracic Echocardiography in Patients on ECMO Support.

OBJECTIVE: The authors investigated if the use of ultrasound-enhancing agents (UEA) can safely improve left ventricular (LV) image quality by transthoracic echocardiography (TTE) in patients on extracorporeal membrane oxygenation (ECMO). DESIGN AND SETTING: This study was performed in a tertiary cardiothoracic and ECMO center in London, United Kingdom. PARTICIPANTS: The authors included 18 prospectively identified consecutive patients requiring TEE supported on peripherally implanted ECMO. INTERVENTION AND MEASUREMENTS: TTE was performed before and after the UEA administration. The authors assessed the LV image quality using the biplane (apical-4-chamber and apical-2-chamber views) endocardial border definition index (1 = good, 2 = suboptimal, 3 = poor, and 4 = unavailable), as well as the feasibility of LV ejection fraction (LVEF) measurement. The authors also gathered sequential clinical information for the next 24 hours. MAIN RESULTS: The patients' median age was 47 years (35, 65), and 5 (28%) were women. The biplane endocardial border definition index improved from the suboptimal to the good range (2.167 [1.812, 3.042] v 1.500 [1.417, 1.792], p = 0.0004) after the use of UEA. The feasibility of LVEF tripled from 25% (n = 5) to 83% (n = 15) (p = 0.0008) with UEA use. The UEA did not set off the bubble alarm and did not impact clinical or ECMO parameters. CONCLUSION: The use of UEA significantly improved the quality of LV biplane images by transthoracic echocardiography, transforming them from suboptimal to good in patients supported with peripherally implanted ECMO. UEA use tripled the feasibility of measuring LVEF by TTE without affecting clinical and ECMO parameters.

An imaging artifact unmasked by contrast-enhanced transesophageal echocardiography.

Performing transesophageal imaging of the left atrial appendage is key before cardioversion for atrial fibrillation. Ultrasound artifacts may induce misinterpretation and decrease in confidence for thrombus exclusion.

Novel Utilization of Ultrasound Enhancing Agents in Complex Congenital Heart Disease Following Superior Cavopulmonary Connection.

BACKGROUND: Children with single-ventricle congenital heart disease typically undergo a superior cavopulmonary connection (SCPC) as the second stage in their surgical palliation. Postoperatively, stenoses of the SCPC and branch pulmonary arteries can occur. If there are clinical concerns and echocardiography is insufficient for diagnosis, patients undergo invasive evaluation with exposure to radiation and anesthesia. The use of ultrasound enhancing agents (UEAs) to improve echocardiographic diagnostic capabilities has not previously been described in this population. METHODS: A single-center, retrospective case review was conducted of children who underwent echocardiography with UEA, following SCPC, from March 1, 2020, to April 15, 2022, at the Children's Hospital of Philadelphia. Twenty-two patients with hypoxemia or concern for obstruction following SCPC underwent UEA echocardiography. Extracted clinical data included patient demographics, echocardiographic images, angiography, surgical and transcatheter interventions, as well as available follow-up data. RESULTS: Six of the 22 UEA echocardiograms demonstrated stenosis or occlusion of either the SCPC or a pulmonary artery. All six patients underwent cardiac catheterization and five required intervention. Angioplasty was performed in each case with one requiring subsequent surgical revision. Sixteen of 22 UEA echocardiograms demonstrated no evidence of stenosis. Ten of these 16 patients improved, while six experienced persistent hypoxemia prompting referral for cardiac catheterization. Angiography confirmed the UEA echocardiographic findings (absence of stenosis) in four of these six patients. There were no adverse reactions related to UEA administration. CONCLUSIONS: Echocardiography with UEAs is a valuable and safe adjunctive imaging modality following SCPC, particularly when two-dimensional and color imaging is limited. This novel application of UEAs in complex patients with congenital heart disease provides an "angiogram-like" image, better delineating vessel walls and improving assessment of postoperative obstruction. As experience with UEAs increases in the congenital heart disease population, there may be opportunities to decrease invasive and costly procedures, while expediting the care of patients in need of intervention.

Safety findings after intravenous administration of sulfur hexafluoride microbubbles to 463,434 examinations at 24 centers.

OBJECTIVES: We aimed to evaluate the safety of the ultrasound contrast agent sulfur hexafluoride microbubbles in a large group of patients referred for routine contrast-enhanced ultrasound (CEUS). METHODS: A retrospective assessment was made of all patients that received sulfur hexafluoride microbubbles intravenously for CEUS at 24 centers between January 2006 and April 2019. Patient demographic details, examination type, and the dose of sulfur hexafluoride microbubbles administered were recorded with specific adverse events (AEs) documentation tools at each center. All AEs were recorded as serious or non-serious. Non-serious AEs were classified by intensity as mild, moderate, or severe according to ACR criteria. The frequencies of AEs across patient subgroups were compared using the chi-square test. RESULTS: A total of 463,434 examinations were evaluated. Overall, 157 AEs (153 [0.033%] non-serious; 4 [0.001%] serious) were reported after sulfur hexafluoride microbubbles administration, giving an AE frequency of 0.034% (157/463,434). Among the non-serious AEs, 66 (0.014%) were mild, 70 (0.015%) moderate, and 17 (0.004%) severe in intensity. The liver was the most common examination site, presenting an AE frequency of 0.026%. The highest AE frequency (0.092%) was for patients undergoing CEUS for vascular disease. There were no significant gender differences in either the total number or the severity of non-serious AEs (chi-square = 2.497, p = 0.287). The onset of AEs occurred within 30 min of sulfur hexafluoride microbubbles administration in 91% of cases. CONCLUSION: The frequency of AEs to sulfur hexafluoride microbubbles is very low and severe reactions are rare, confirming that sulfur hexafluoride microbubbles are appropriate for routine CEUS applications. KEY POINT: • The frequency of AEs to sulfur hexafluoride microbubbles is very low and severe reactions are rare.

The "Lightbulb" Sign: A Novel Echocardiographic Finding Using Ultrasound Enhancing Agent in Fulminant COVID-19-Related Myocarditis.

We report a case of fulminant COVID-19-related myocarditis requiring venoarterial extracorporeal membrane oxygenation where the use of an ultrasound-enhancing agent demonstrated a previously undescribed echocardiographic finding, the "lightbulb" sign. This sign potentially represents a new area for the use of an ultrasound enhancing agent in the echocardiographic diagnosis of myocarditis.

CEUS cardiac exam protocols International Contrast Ultrasound Society (ICUS) recommendations.

The present CEUS Cardiac Exam Protocols represent the first effort to promulgate a standard set of protocols for optimal administration of ultrasound enhancing agents (UEAs) in echocardiography, based on more than two decades of experience in the use of UEAs for cardiac imaging. The protocols reflect current clinical CEUS practice in many modern echocardiography laboratories throughout the world. Specific attention is given to preparation and dosing of three UEAs that have been approved by the United States Food and Drug Administration (FDA) and additional regulatory bodies in Europe, the Americas and Asia-Pacific. Consistent with professional society guidelines (J Am Soc Echocardiogr 31:241-274, 2018; J Am Soc Echocardiogr 27:797-810, 2014; Eur Heart J Cardiovasc Imaging 18:1205, 2017), these protocols cover unapproved "off-label" uses of UEAs-including stress echocardiography and myocardial perfusion imaging-in addition to approved uses. Accordingly, these protocols may differ from information provided in product labels, which are generally based on studies performed prior to product approval and may not always reflect state of the art clinical practice or guidelines.

Selective Enhancement of Swine Myocardium with a Novel Ultrasound Enhancing Agent During Transthoracic Echocardiography.

Ultrasound enhancing agents are approved to delineate the endocardial border and opacify the left ventricle cavity (LVC). We present a nested phase change agent (NPCA) designed to enable selective myocardial enhancement without enhancing the LVC by employing a dual-activation mechanism dependent on sufficient ultrasound intensity and the microenvironment of the myocardium. Swine received bolus injections of NPCA while echocardiograms were collected and processed to determine background-subtracted acoustic intensities (AI) in the LVC and septal myocardium. At mechanical index (MI) ≥ 0.8, the NPCA enhanced the myocardium selectively (p < 0.001) while the LVC remained at baseline AI. A 5-mL bolus of NPCA enhanced swine myocardium and enhancement persisted for > 5 min at 1.4 MI, while hemodynamics and EKG remained normal. Our findings demonstrate that the NPCA enhances swine myocardium selectively without enhancing the LVC. The NPCA could have utility for functional and structural echocardiographic studies with clinical ultrasound using standard settings.

Routine Use of Contrast on Admission Transthoracic Echocardiography for Heart Failure Reduces the Rate of Repeat Echocardiography during Index Admission.

BACKGROUND: The authors retrospectively evaluated the impact of ultrasound enhancing agent (UEA) use in the first transthoracic echocardiographic (TTE) examination, regardless of baseline image quality, on the number of repeat TTEs and length of stay (LOS) during a heart failure (HF) admission. METHODS: There were 9,115 HF admissions associated with admission TTE examinations over a 4-year period (5,337 men; mean age, 67.6 ± 15.0 years). Patients were grouped into those who received UEAs (contrast group) in the first TTE study and those who did not (noncontrast group). Repeat TTE examinations were classified as justified if performed for concrete clinical indications during hospitalization. RESULTS: In the 9,115 admissions for HF (5,600 in the contrast group, 3,515 in the noncontrast group), 927 patients underwent repeat TTE studies (505 in the contrast group, 422 in the noncontrast group), which were considered justified in 823 patients. Of the 104 patients who underwent unjustified repeat TTE studies, 80 (76.7%) belonged to the noncontrast group and 24 to the contrast group. Also, UEA use increased from 50.4% in 2014 to 74.3%, and the rate of unjustified repeat studies decreased from 1.3% to 0.9%. The rates of unjustified repeat TTE imaging were 2.3% and 0.4% (in the noncontrast and contrast groups, respectively), and patients in the contrast group were less likely to undergo unjustified repeat examinations (odds ratio, 0.18; 95% CI, 0.12-0.29; P < .0001). The mean LOS was significantly lower in the contrast group (9.5 ± 10.5 vs 11.1 ± 13.7 days). The use of UEA in the first TTE study was also associated with reduced LOS (linear regression, ß1 = -0.47, P = .036), with 20% lower odds for odds of prolonged (>6 days) LOS. CONCLUSIONS: The routine use of UEA in the first TTE examination for HF irrespective of image quality is associated with reduced unjustified repeat TTE testing and may reduce LOS during an index HF admission.

Taking the time to get it bright: Use of ultrasound enhancing agent redirects clinical course of an unstable patient.

The use of ultrasound enhancing agents (UEA) during echocardiography helps to optimize visualization in technically difficult studies, with improved left ventricular opacification and endocardial border definition. The use of these agents may often unveil critical data that drastically alter clinical decision making. Despite the potential clinical benefits of UEA and known safety data, clinicians are still sometimes reluctant to take the time to use UEAs in unstable patients. Herein, we demonstrate a challenging case of a patient with late presentation myocardial infarction, complicated with cardiogenic shock and pseudoaneurysm formation that was not observed in non-contrast images, emblematically demonstrating the value of UEA in selected patients. .

Effect of Intermittent High-Mechanical Index Impulses on Left Ventricular Strain.

BACKGROUND: Intermittent high-mechanical index (MI) impulses from a transthoracic ultrasound transducer are recommended for regional wall motion analysis and assessment of myocardial perfusion following intravenous administration of ultrasound enhancing agents (UEAs). High-MI impulses (>1.0) applied in this setting have also been shown to increase microvascular blood flow through a purinergic signaling pathway, but their effects on left ventricular (LV) myocardial function are unknown. Therefore, the aim of this study was to investigate the effect of transthoracic intermittent high-MI impulses during intravenous UEA infusion in patients with normal and abnormal resting systolic function. METHODS: Fifty patients referred for echocardiography to evaluate LV systolic function during continuous infusion of UEAs (Definity 3% infusion) were prospectively assigned to low-MI (<0.2) imaging alone (group 1) or low-MI (<0.2) imaging with intermittent high-MI impulses (five frames, 1.8 MHz, MI = 1.0-1.2) applied at least two times in each apical window to clear myocardial contrast (group 2). Global longitudinal strain (GLS) measurements were obtained at baseline before UEA administration and at 5-min intervals up to 10-min after infusion completion. RESULTS: There were no differences between groups with respect to age, gender, resting GLS, biplane LV ejection fraction, or cardiac risk factors. Resting GLS in group 1 was -15.5 ± 5.2% before UEA infusion and -15.5 ± 5.4% at 10 min after UEA infusion. In comparison, GLS increased in group 2 (-15.3 ± 5.0 before infusion and -16.8 ± 4.8% at 10 min, P < .00001). Improvements in GLS were seen in patients with normal and abnormal systolic function. Regional analysis demonstrated that the increase in strain in patients with abnormal LV ejection fractions was primarily in the apical segments (-12.0 ± 2.7% before infusion and -13.4 ± 3.4% at 10 min, P = .001). CONCLUSIONS: High-MI impulses during infusion of a commercially available contrast agent can improve LV systolic function and may have therapeutic effect in patients with LV dysfunction.