Faculty Bibliography

BACKGROUND: Pulmonary artery intimal spindle cell sarcomas are rare and carry with them a poor prognosis and high rate of recurrence. In extremely rare cases, this tumor can infiltrate the pulmonic valve and manifest as adult-onset pulmonic stenosis. CASE PRESENTATION: We report an unusual case of a patient with symptomatic, adult-onset severe pulmonic stenosis who was referred for possible balloon valvuloplasty but was subsequently found to have pulmonary artery intimal sarcoma infiltrating the pulmonary valve leading to progressive exertional dyspnea. CONCLUSION: The presence of adult-onset pulmonic stenosis should prompt the clinician to investigate further as most cases of pulmonic stenosis are congenital in nature and present early in life. Careful diagnostic evaluation in concert with multimodal imaging should take place to arrive at the correct and challenging diagnosis of sarcoma-induced adult-onset severe pulmonic stenosis. Given the poor prognosis and rapid progression of disease, early diagnosis is crucial.

This paper provides a comprehensive overview of 3D transesophageal echocardiography still images and movies of mechanical mitral valves, mitral bioprostheses, and mitral valve repairs. Alongside these visual descriptions, the historical overview of surgical and percutaneous mitral valve intervention is described with the special emphasis on the incremental value of 3D transesophageal echocardiography (3DTEE). For each mitral valve intervention, 2D echocardiography, chest x-ray, and fluoroscopy images corresponding to 3DTEE are given. In addition, key references on echocardiographic imaging of individual valves and procedures are enumerated in accompanying figures and tables.

Background: Patients with severe aortic stenosis in the setting of low gradient and preserved left ventricular ejection fraction (LVEF) remain an area of clinical uncertainty. Methods: Retrospective chart review identified 209 patients who underwent transcatheter aortic valve replacement (TAVR) between September 2014 and September 2015. Of these patients, 3 (1.4%) were excluded due to procedural indication other than severe aortic stenosis and 41 (20%) were excluded due to reduced LVEF (<50%). Of the remaining 165 patients with aortic valve area <1 cm2, 77 (47%) had either a peak velocity <4.0 m2 or mean gradient <40 mmHg (LG group) and 88 (53%) had both peak velocity >4.0 m2 and mean gradient >40 mmHg (HG group) across the AV. Outcomes were defined by the valve academic research consortium 2 criteria when applicable and compared between the LG and HG groups via Fisher's exact test. Median follow-up was 367 days. Continuous data are shown as median [interquartile range] and categorical data are shown as proportions. Results: The 30-day mortality risk as assessed by Society of Thoracic Surgery score was not significantly different between the LG and HG groups (5.9% [3.5-8.1] vs 6.2% [4.4-7.6], p=0.45). There were no significant differences in outcomes (Table). Conclusion: In a high-volume center, patients undergoing TAVR for severe AS with LG preserved LVEF have no significant difference in adverse outcomes, both in-hospital and on 1-year follow-up, when compared to patients with HG preserved LVEF. (Figure Presented)

Pectus excavatum is one of the most common congenital chest wall deformities. The degree of sternal depression, which may result in compression of the right heart by the chest wall, is variable. While typically asymptomatic, there are various symptoms that can result from severe pectus excavatum. We report on a patient with severe pectus excavatum leading to dynamic obstruction of the right ventricular outflow tract in the seated position.

Aortic root thrombus is an uncommon complication of continuous-flow left ventricular assist devices (LVAD). We present the case of a 71-year-old man with ischemic cardiomyopathy who underwent destination therapy HeartMate II LVAD placement. Eighteen months later, he presented with a cerebrovascular accident followed by myocardial infarction. Transesophageal echocardiography revealed an aortic root thrombus spanning the left and noncoronary cusps and obliterating the left main coronary artery. We discuss the incidence, risk factors, and management of aortic root thrombus in LVAD patients. To our knowledge, this is the first report of three-dimensional echocardiography used to characterize an LVAD-associated aortic root thrombus.

We report a rare case of progressive left ventricular outflow tract (LVOT) obstruction after percutaneous device closure of a mechanical prosthetic mitral valve (MV) paravalvular leak (PVL) in the region of aortomitral curtain in a patient who also had small mechanical aortic valve prosthesis with patient-prosthesis mismatch.

PURPOSE OF REVIEW: Functional mitral regurgitation (FMR) is a common complication of left ventricular dysfunction. It is now recognized as an important clinical entity and an independent predictor of poor prognosis in cardiomyopathy patients. In this review, we provide a comprehensive summary of the pathophysiology, latest imaging modalities, and diagnostic criteria for FMR. Additionally, we discuss the recent literature on the continuously evolving surgical and percutaneous treatment options. RECENT FINDINGS: The criteria for quantification of FMR on echocardiography were updated and are distinct from organic mitral regurgitation in the most recent American College of Cardiology/American Heart Association 2014 valve guidelines. Furthermore, the evolving role of MitraClip for potential treatment of FMR offers exciting prospects to treat high-risk symptomatic patients. SUMMARY: Our review serves to consolidate the current diagnostic and treatment modalities for FMR and provide a contemporary resource for clinicians while treating patients. Additionally, we identify the gaps present in our knowledge of FMR to guide further clinical investigation.