Adherence and Exercise Capacity Improvements of Patients With Adult Congenital Heart Disease Participating in Cardiac Rehabilitation.

Background As the number of adults with congenital heart disease increases because of therapeutic advances, cardiac rehabilitation (CR) is increasingly being used in this population after cardiac procedures or for reduced exercise tolerance. We aim to describe the adherence and exercise capacity improvements of patients with adult congenital heart disease (ACHD) in CR. Methods and Results This retrospective study included patients with ACHD in CR at New York University Langone Rusk Rehabilitation from 2013 to 2020. We collected data on patient characteristics, number of sessions attended, and functional testing results. Pre-CR and post-CR metabolic equivalent task, exercise time, and maximal oxygen uptake were assessed. In total, 89 patients with ACHD (mean age, 39.0 years; 54.0% women) participated in CR. Referral indications were reduced exercise tolerance for 42.7% and post-cardiac procedure (transcatheter or surgical) for the remainder. Mean number of sessions attended was 24.2, and 42 participants (47.2%) completed all 36 CR sessions. Among participants who completed the program as well as pre-CR and post-CR functional testing, metabolic equivalent task increased by 1.3 (95% CI, 0.7-1.9; baseline mean, 8.1), exercise time increased by 66.4 seconds (95% CI, 21.4-111.4 seconds; baseline mean, 536.1 seconds), and maximal oxygen uptake increased by 2.5 mL/kg per minute (95% CI, 0.7-4.2 mL/kg per minute; baseline mean, 20.2 mL/kg per minute). Conclusions On average, patients with ACHD who completed CR experienced improvements in exercise capacity. Efforts to increase adherence would allow more patients with ACHD to benefit.

Lone aortic insufficiency and conduction disease: a marker of reactive arthritis.

A 48-year-old male with history of chronic arthritis and uveitis presented with 1 year of progressively reduced exercise capacity and nonexertional chest pain. Physical examination was consistent with severe aortic insufficiency. An electrocardiogram demonstrated sinus rhythm with first degree atrioventricular block. Transthoracic and transesophageal echocardiography demonstrated severe lone central aortic insufficiency of a trileaflet valve due to leaflet thickening, retraction of leaflet margins and mild aortic root dilation in the setting of left ventricular dilatation. In addition, computed tomographic angiography revealed a small focal aneurysm of the distal transverse arch. He was found to be positive for the immunogenetic marker HLA-B27. The patient subsequently underwent uncomplicated mechanical aortic valve replacement. The diagnosis of HLA-B27 associated cardiac disease should be entertained in any individual with lone aortic insufficiency, especially if accompanied by conduction disease.

Fatal acute necrotizing eosinophilic myocarditis temporally related to use of adalimumab in a patient with relapsing polychondritis.

Tumor necrosis factor α (TNF-α) antagonists are being increasingly used as maintenance therapies for rheumatic diseases, and therefore knowledge of their adverse effects is important. We report a case of fatal acute necrotizing eosinophilic myocarditis temporally related to use of a second course of the TNF-α antagonist, adalimumab. A 51-year-old woman with relapsing polychondritis took adalimumab 2 weeks before presenting with acute myocarditis. Within hours of presentation to the emergency department, she had cardiac arrest due to fulminant heart failure. Autopsy demonstrated necrotizing eosinophilic myocarditis. This is a rare cause of fulminant heart failure. This is the first report of a TNF-α antagonist potentially associated with acute necrotizing eosinophilic myocarditis.

Cardiac arrest following anaesthetic induction in a world-class bodybuilder.

This is a report of a 41-year-old professional male bodybuilder with a history of active anabolic-androgenic steroid abuse and a normal echocardiogram two years prior to admission who experienced a near-fatal arrhythmia during anaesthetic induction for elective orthopaedic surgery. The patient had severe concentric left ventricular hypertrophy, diffuse left ventricular hypokinesis, decreased ejection fraction and inducible monomorphic ventricular tachycardia. A single-chamber cardioverter/defibrillator was inserted.

Isolated subclavian artery aneurysm: evaluation by transesophageal echocardiography.

A 50-year-old man was evaluated following a motor vehicle accident. Chest X-ray showed a widened mediastinum. Transesophageal echocardiography was helpful in identifying the left subclavian artery and in demonstrating an isolated subclavian artery aneurysm. The TEE findings correlated well with the results of chest CT. Using TEE for the identification of the aortic branches in patients with chest trauma may be critical.

Bilateral pulmonary artery compression and obstruction by tumor: diagnosis by unusual Doppler flow patterns.

Pulmonary artery obstruction may be caused by tumor within or external to the arteries. Presented here is a patient with life-threatening compromise in pulmonary flow that was caused by a pulmonary neoplasm. The Doppler echocardiogram showed subtotal narrowing of the right pulmonary artery and total occlusion of the left pulmonary artery. In addition, the beneficial effects of chemotherapy were documented by Doppler.

Effect of treatment on the incidence of stroke and other emboli in 519 patients with severe thoracic aortic plaque.

Severe aortic plaques seen on transesophageal echocardiography (TEE) are a high-risk cause of stroke and peripheral embolization. Evidence to guide therapy is lacking. Retrospective information was obtained regarding the occurrence of embolic events (stroke, transient ischemic attacks, or peripheral emboli) in 519 patients with severe thoracic aortic plaque seen on TEE since 1988. Treatment with statins, warfarin, or antiplatelet medications was noted. Treatment was not randomized. In a matched-paired analysis, each patient taking each class of therapy was matched for age, gender, previous embolic event, hypertension, diabetes, congestive failure, and atrial fibrillation to someone not taking that medication. Multivariate analysis was also performed. An embolic event occurred in 111 patients (21%). Multivariate analysis showed that statin use was independently protective against recurrent events (p = 0.0001). Matched analysis also showed a protective effect of statins (p = 0.0004; absolute risk reduction 17%, relative risk reduction 59%, number needed to treat [n = 6]). No protective effect was found for warfarin or antiplatelet drugs. The odds ratio for embolic events was 0.3 (95% confidence interval [CI] 0.2 to 0.6) for statin therapy, 0.7 (95% CI 0.4 to 1.2) for warfarin, and 1.4 (95% CI 0.8 to 2.4) for antiplatelet agents. Thus, there is a protective effect of statin therapy, and no significant benefit of warfarin or antiplatelet drugs on the incidence of stroke and other embolic events in patients with severe thoracic aortic plaque on TEE.

The risk of the development of aortic stenosis in patients with "benign" aortic valve thickening.

BACKGROUND: Aortic valve thickening (AVT) without aortic stenosis (AS) is common and was often considered benign. However, it has recently been found to be associated with increased morbidity and mortality. It is unknown whether patients with AVT are at risk for the development of AS. METHODS: Our echocardiography database from 1987 to 1993 was searched for cases of AVT with at least 1 year of echocardiographic follow-up. The risk of the development of AS was compared in patients with and without AVT. RESULTS: There were 2131 patients with AVT and at least 1 year of echocardiographic follow-up. Aortic stenosis developed in 338 patients (15.9%) (mild, 10.5%; moderate, 2.9%; and severe, 2.5%). Multivariate analysis, including age, left ventricular hypertrophy, and mitral annular calcification, revealed that only mitral annular calcification was independently and significantly associated with progression to AS. CONCLUSIONS: Aortic valve thickening without stenosis is common, and it may progress to significant AS. It is possible that this development of AS may be responsible for some of the increased morbidity and mortality in patients with AVT.