Faculty Bibliography

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.

• RHD is a rare cause of severe valvular AS. • Rheumatic MS typically accompanies rheumatic AV disease. • 2D and 3D echocardiography are essential in the assessment of rheumatic AS. • CCT has become crucial in planning therapeutic procedures for AS.

• Precise LAA anatomy must be established for LAA occlusion device selection. • We have developed the TUPLE maneuver, an acronym for “tilt up and turn left”. • The TUPLE maneuver facilitates LAA device selection and intraprocedural guidance.

INTRODUCTION/BACKGROUND:Gram-negative organisms of the AACEK group, formerly known as HACEK, rarely cause endocarditis. CASE SERIES/METHODS:We present three cases of bacterial endocarditis, involving native and prosthetic valves, caused by AACEK organisms. In two patients, Cardiobacterium hominis was the responsible organism, and in a third, Aggregatibacter aphrophilus was implicated. A dental source of infection was identified in two patients, and in all three patients, the presentation of endocarditis was subacute. DISCUSSION/CONCLUSIONS:This case series highlights the indolent nature of infection with the AACEK organisms. It also demonstrates the crucial role of multimodality imaging, especially transesophageal echocardiography, in the diagnosis of AACEk endocarditis of both native and prosthetic valves, and in delineating the extent of abscess in those with prosthetic valve infection.

Infective endocarditis (IE) is a life-threatening disease associated with in-hospital mortality of nearly one in five cases. IE can destroy valvular tissue, which may rarely progress to aneurysm formation, most commonly at the anterior leaflet in instances of mitral valve involvement. We present a remarkable case of a patient with IE and a rare complication of a ruptured aneurysm of the posterior leaflet of the mitral valve. Two- and Three-dimensional transesophageal echocardiography, intra-operative videography, and histopathologic analysis revealed disruption at this unusual location-at the junction of the P2 and P3 scallops, surrounded by an annular abscess.

Mitral regurgitation (MR) is one of the most commonly encountered valvular lesions in clinical practice. MR can be either primary (degenerative) or secondary (functional) depending on the etiology of MR and the pathology of the mitral valve (MV). Echocardiography is the primary diagnostic tool for MR and is key in determining this etiology as well as MR severity. While clinicians usually turn to 2 Dimensional echocardiography as first-line imaging, 3 Dimensional echocardiography (3DE) has continually shown to be superior in terms of describing MV anatomy and pathology. This review article elaborates on 3DE techniques, modalities, and advances in software. Furthermore, the article demonstrates how 3DE has reformed MR evaluation and has played a vital role in determining patient management.

Partial anomalous pulmonary venous return (PAPVR) comprises a group of congenital cardiovascular anomalies associated with pulmonary venous flow directly or indirectly into the right atrium. Scimitar syndrome is a variant of PAPVR in which the right lung is drained by right pulmonary veins connected anomalously to the inferior vena cava. Surgery is the definitive treatment for scimitar syndrome. However, it is not always necessary as many patients are asymptomatic, have small left-to-right shunts, and enjoy a normal life expectancy without surgery. We report multimodality imaging in four adults with scimitar syndrome and the implications for management of this rare syndrome.