Faculty Bibliography

BACKGROUND:Raghib syndrome is a congenital anomaly that is caused by a persistent left superior vena cava draining into the left atrium, unroofed coronary sinus, and atrial septal defect. It can lead to cyanosis, cryptogenic strokes, pulmonary hypertension, and arrythmias. CASE SUMMARY/METHODS:A 60-year-old woman presented with palpitations due to atrial flutter. Cardiac imaging, including computed tomography, transesophageal echocardiography, and magnetic resonance imaging, confirmed bilateral superior vena cavae, with the left superior vena cava draining into the left atrium and minimal right-to-left shunting (3%). She was managed with anticoagulation and underwent regular follow-up without complications. DISCUSSION/CONCLUSIONS:Diagnosis of Raghib syndrome relies on multimodality imaging. In cases with minimal symptoms and left-to-right shunting, conservative management with biannual follow-up may be sufficient. TAKE-HOME MESSAGES/CONCLUSIONS:Multimodality imaging is crucial in confirming anatomy and characterizing flow dynamics in cyanotic congenital heart disease. In patients with Raghib syndrome without cyanosis, conservative management with anticoagulation may be a safe alternative to surgical intervention.

BACKGROUND:Buckling of a transesophageal echocardiography (TEE) probe is a rare but potentially life-threatening complication that carries a significant risk of esophageal perforation. CASE SUMMARY/METHODS:We report 3 cases of buckled TEE probes that were promptly recognized, diagnosed, and managed using multimodal imaging. In each instance, the location of the buckled probe was confirmed with bedside imaging. Subsequently, the patient was taken to the interventional suite for advancement of the probe to the stomach under fluoroscopic guidance. Finally, the buckled TEE probe was straightened and removed successfully from the patient without difficulty. DISCUSSION/CONCLUSIONS:Early recognition of buckled TEE probes and employment of a structured, algorithmic management approach are essential to optimizing outcomes and preventing associated morbidity and mortality. TAKE HOME MESSAGES/CONCLUSIONS:Buckling of the TEE probe requires a high index of suspicion and prompt diagnosis to prevent catastrophic complications such as esophageal perforation. Advancement of the TEE probe in the stomach under fluoroscopic guidance facilitates straightening and removal of the probe without the risk of esophageal perforation.

As the number of mitral valve transcatheter edge-to-edge repair procedures increases, there are catheter operators and interventional echocardiography operators performing these procedures with variable expertise. Expert imaging is paramount for the success of these procedures and requires knowledge of mitral valve anatomy, the ability to quickly manipulate two-dimensional (2D) and three-dimensional (3D) images in real time, and sufficient procedural experience to anticipate challenges and offer imaging solutions. There are currently no standard algorithms for the interventional echocardiographer to direct a successful mitral valve transcatheter edge-to-edge repair procedure. This guideline sets forth imaging views and the standard procedural steps and defines the imaging content that must be communicated using 2D, biplane, 3D volume, and/or multiplanar reconstruction 3D formats.

UNLABELLED:Oral anticoagulation (OAC) remains the primary means of stroke prevention in patients with atrial fibrillation (AF). However, there are patients at greater risk of bleeding or who have experienced major bleeding, whereby long term OAC is relatively contraindicated. Additionally, up to 55% of eligible AF patients do not utilize OAC. Thus, transcatheter left atrial appendage occlusion devices (LAAOD) present an attractive alternative to mitigate stroke risk. Randomized trials have demonstrated the noninferiority of LAAOD to OAC in reducing stroke risk. However, treatment with LAAODs presents its own risks, including the risk of device-related thrombus (DRT). In this report, we describe an unusual case of a patient with a small DRT initially identified on transesophageal echocardiography (TEE) 6 weeks after implantation of a Watchman FLX (Boston Scientific, Marlborough, MA, USA) LAAOD that was appropriately treated and resolved on TEE 6 months after implantation. However, a massive late DRT recurred 2 years after implantation. This case highlights the importance of continued device monitoring for unfavorable evolution of DRT beyond the currently recommended 45-day to 1-year monitoring period, and especially after suspected thrombus resolution. Furthermore, it underlines the importance of developing newer generation LAAOD that reduces DRT risk. LEARNING OBJECTIVE/UNASSIGNED:Left atrial appendage occlusion devices can mitigate stroke risk in atrial fibrillation patients with contraindications to oral anticoagulation. However, these devices present with their own risks, including the risk of device-related thrombus (DRT). This case highlights the importance of continued device monitoring for unfavorable evolution of DRT beyond the recommended 45-day to 1-year monitoring period.

An ultrasound artifact is a feature in an ultrasound image that does not accurately represent the true anatomy or pathology. Cardiac ultrasound artifacts are common and inevitable findings as they originate from the physical properties of ultrasound. Additionally, artifacts may occur due to interference from external equipment and devices producing ultrasound waves. This document provides a uniform and structured approach to managing ultrasound artifacts, including the appearance of the artifact on the image, the mechanism behind the artifact generation, the clinical impact of the artifact on the diagnosis and management of the patient, examples of real cases, and how the artifact can be avoided or mitigated. In addition to true artifacts, we also discuss a series of artifact-like phenomena. Everyone involved in performing or interpreting cardiac ultrasound should be familiar with artifacts and their potential for misdiagnosis, which in some instances may lead to serious clinical consequences. Despite continued improvements in ultrasound imaging technologies, artifacts remain common in all echocardiographic modes, including two-dimensional, spectral, and color Doppler, as well as three-dimensional echocardiography.

PURPOSE OF REVIEW:This review summarizes the current and evolving landscape of multimodality imaging for percutaneous endocardial left atrial appendage occlusion (LAAO) across three phases: preprocedural planning, periprocedural guidance, and postprocedural follow-up. RECENT FINDINGS:Transesophageal echocardiography (TEE) remains the primary imaging modality to assess the left atrial appendage (LAA) in all three phases of clinical care surrounding LAAO, though many innovations have led to the rise of cardiac computed tomography (CCT) and intracardiac echocardiography (ICE) as powerful adjuncts and alternatives to TEE. Advances in CCT technology such as fusion imaging and three-dimensional (3D) modeling have contributed to the emergence of CCT as an alternative to TEE for preprocedural evaluation. Intraprocedurally, real-time 3D ICE offers similar real-time guidance to TEE with similar procedural efficacy while employing a simplified workflow that can reduce procedural times and staffing needs. Post-procedural assessment with TEE or CCT is essential for evaluating device stability and complications such as peridevice leak or device-related thrombus. SUMMARY:Multimodality imaging of LAAO is a rapidly evolving field, and thoughtful integration of TEE, CCT, and ICE is critical to optimize LAAO outcomes and minimize procedural complications.

OBJECTIVE/UNASSIGNED:During septal myectomy, once the heart is arrested and drained of blood on cardiopulmonary bypass, transesophageal echocardiography can no longer assess septal thickness. In the present study, we evaluated the effectiveness of on-pump intracardiac echocardiography (OPIE) for real-time intraoperative septal thickness assessment in patients with preoperative thickness ≤2.0 cm. Our hypothesis was that OPIE measurements would be conconcordant with the pre- and postcardiopulmonary bypass transesophageal echocardiography measurements that are at present the primary operative guides. METHODS/UNASSIGNED:We retrospectively reviewed patients with hypertrophic cardiomyopathy and septal thickness ≤2.0 cm on transthoracic echocardiography who underwent septal myectomy from July 2017 to July 2024. The OPIE probe was introduced into the left-ventricular chamber during cardioplegic arrest, with repeated measurements to assess the depth and adequacy of resection. Septal thickness was evaluated pre-myectomy using transthoracic echocardiography, cardiac magnetic resonance imaging, transesophageal echocardiography, and OPIE. Lin's concordance correlation coefficients and Bland-Altman analyses were used to evaluate agreement between modalities. RESULTS/UNASSIGNED:A total of 220 patients were included with preoperative thickness ≤2.0, 56 of whom underwent myectomy with OPIE guidance. Preresection transesophageal echocardiography and OPIE demonstrated the strongest agreement of all the imaging modalities (Lin's concordance correlation coefficient, 0.81; 95% CI, 0.72-0.88), with minimal bias (-0.73) and the narrowest limits of agreement (-3.76, +2.31]. OPIE-derived resection thickness estimates were tightly clustered. In the OPIE cohort, there was 1 ventricular septal defect (1.8%) and no 30-day mortality. CONCLUSIONS/UNASSIGNED:OPIE is a reliable tool for intraoperative assessment of septal thickness, particularly in patients with mild hypertrophy.

• Less-invasive interventions for moderate-to-severe, symptomatic MR are emerging. • Direct measures of MR are unreliable after intervention due to the altered orifice. • Indirect measures of MR when used together can assess procedural success. • 2D, color, and spectral Doppler indirectly evaluate MR postop. • More validation is needed to link these indirect markers to long-term outcomes.

Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart malformations, characterized by four pathological features: right ventricular outflow tract obstruction/pulmonic stenosis, a ventricular septal defect, an overriding aorta, and right ventricular hypertrophy. It was initially partially defined by Nicholas Steno in the 17th century and completely described by Étienne-Louis Arthur Fallot and Maude Abbott in the 19th and 20th centuries. The advances in multimodality imaging and innovative surgical and transcatheter techniques have led to advances in the management of TOF. While initial management in the mid-20th century favored palliative procedures in infancy followed by complete surgical repair, data now support an early complete surgical repair in infancy. The major post-repair complication is the development of significant pulmonary regurgitation, necessitating either surgical or transcatheter valve replacement. Multimodality imaging is essential to the initial identification of TOF, preoperative planning, and post-procedural complication assessment. In this review, we provide a historical perspective of the discovery and clinical management of TOF from the 1600s to the present day, as well as the role of multimodality imaging in TOF management.

BACKGROUND:Diagnosis and management of multivalve disease could be challenging for clinicians. Rheumatic heart disease is a well-known etiology of multivalve disease. CASE SUMMARY/METHODS:A 51-year-old male with a history of rheumatic heart disease was referred to rule out infectious endocarditis. Three-dimensional (3D) transesophageal echocardiography (TEE) showed significant stenosis of the tricuspid valve, moderate tricuspid regurgitation, rheumatic mitral valve disease, and a bicuspid aortic valve. DISCUSSION/CONCLUSIONS:Transthoracic echocardiography is the modality of choice for evaluation of cardiac valves and quantification of cardiac chambers. However, due to the complex nature of multivalve disease, using a complementary imaging technique such as 3D TEE becomes crucial in many patients. Tricuspid stenosis as the dominant valvular lesion in rheumatic heart disease is rare and almost always occurs in the presence of mitral valve disease. We present a unique case of rheumatic heart disease with the involvement of mitral and tricuspid valves in the presence of a bicuspid aortic valve, in which tricuspid stenosis is the predominant lesion. We also discuss the important role of cardiac imaging and 3D TEE in patient decision making. TAKE-HOME MESSAGES/CONCLUSIONS:Tricuspid stenosis as the dominant valvular lesion in rheumatic heart disease is rare and almost always occurs in the presence of mitral valve disease. Transthoracic echocardiography remains the modality of choice for evaluation of the tricuspid valve. 3D TEE can play an important role in reaching a final management decision.