Added Value of Live 3-Dimensional Transesophageal Echocardiography During Procedures in Fontan Patients.

3D transesophageal echocardiography (3D-TEE) has proven useful and accurate during some operating room (OR), interventional cardiac catheterization (Cath), and electrophysiologic (EP) procedures. The use of 3D-TEE during similar procedures in patients who have undergone Fontan surgery and its additional value have not been previously reported. To determine if live 3D-TEE during procedures post Fontan has added value, 3D-TEEs in 58 post-Fontan patients over a 5-year study period were reviewed. Additional information provided by 3D-TEE (over 2D-TEE) was classified into the following: A: new information which changed/refined the plan and 0: no new important information. Pre- and post-bypass OR 3D-TEEs were counted as one study. A total of 67 3D-TEEs (41 Cath, 13 OR, and 13 EP procedures) were performed. Median age was 14 years (6-39 years). Median weight was 47 kg (21-109 kg). In Cath procedures, only 2/41 (5%) were graded A (R to L atrial level shunt [Fontan leak], n = 1; thrombus in pulmonary artery stump, n = 1). In the OR, 6/13 (46%) were graded A (atrioventricular valvuloplasty, n = 1; neo-aortic valvuloplasty, n = 1; relief of systemic and pulmonary venous outflow obstruction, n = 2 and n = 2; respectively). In EP procedures, 4/13 (31%) were graded A (thrombus, n = 3; mapping for lead placement to assist in multisite pacing for dyssynchrony, n = 1). 3D-TEE of Fontan improved visualization and frequently added value in the OR/EP lab and may be helpful in select catheterization cases. Future studies with a larger sample could build on this data to identify when 3D-TEE will be most useful.

First Intracardiac Kaposiform Hemangioendothelioma in an Infant Resolved With Sirolimus: A Case Report.

INTRODUCTION: Kaposiform hemangioendothelioma (KHE) is a rare and aggressive vascular tumor that can be associated with a consumptive coagulopathy and thrombocytopenia (Kasabach-Merritt phenomenon). Only one case of an intracardiac KHE has been reported which was treated with surgical excision and then expectant management. CASE PRESENTATION: We present a patient with an intracardiac KHE which presented as a large mass surrounding the atria, pulmonary veins, superior vena cava, and infiltrating the atrial septum with moderate compression of the superior vena cava and mild compression of the pulmonary veins. This tumor clinically presented as persistent tachypnea and was unresponsive to conventional therapy with vincristine and steroids but responded dramatically to Sirolimus with almost complete regression on follow-up. CONCLUSIONS: None of the current treatments for KHE, alone or in combination therapy have been found to be effective in a uniform or reproducible manner. Well designed, preferably randomized trials are required for a better understanding of the appropriate dosage and duration as well as response to treatment and a consensus of first and second line therapies.

Three-dimensional analysis of regional right ventricular shape and function in repaired tetralogy of Fallot using cardiovascular magnetic resonance.

BACKGROUND: Patients with surgically repaired tetralogy of Fallot (rTOF) often have residual pulmonic valve regurgitation, leading to abnormal remodeling and dysfunction of the right ventricle often requiring pulmonic valve replacement. We tested the hypothesis that 3D analysis of right ventricular (RV) shape and function may reveal differences in regional adaptive remodeling that occurs in patients with rTOF, depending on whether a transannular patch (TAP) was utilized. METHODS: Forty patients with rTOF who underwent cardiac magnetic resonance imaging (1.5 T), including 20 with and 20 without TAP, and 10 normal controls were studied. Images were analyzed to measure RV endocardial curvature and global and regional volume and function. RESULTS: RV ejection fraction (EF) was 42 ±â€¯11% in TAP and 38 ±â€¯9% in no-TAP (p = 0.19), both lower than 54 ±â€¯3% in controls (p < 0.01). Left ventricular (LV) EF was 54 ±â€¯9% in TAP, 54 ±â€¯8% in no-TAP (p = 0.87) and 61 ±â€¯16% in controls (both p < 0.01). Indexed LV end-diastolic volumes were higher in no-TAP than in TAP subgroup (p = 0.02). With TAP, mid RV septum showed lower curvature during diastole (less convex), than the mid and apical free walls and free wall adjacent to the RV outflow tract (RVOT; more convex). There were no differences in curvature during systole between rTOF subgroups but mid and RVOT free walls showed higher curvature versus controls. CONCLUSIONS: This is the first study to comprehensively describe the influence of TAP on changes in regional RV shape in patients with rTOF. Understanding these differences may help guide therapeutic options for residual pulmonary valve regurgitation in rTOF patients.

Three-Dimensional Echocardiographic En Face Views of Ventricular Septal Defects: Feasibility, Accuracy, Imaging Protocols and Reference Image Collection.

BACKGROUND: Ventricular septal defect (VSD) is the most common congenital cardiac anomaly. Accurate assessment is critical for planning treatment. Recent advances in three-dimensional (3D) echocardiography have improved image quality and ease of use. METHODS: The feasibility and accuracy of three specific 3D echocardiographic protocols to demonstrate en face views of VSDs were analyzed in a retrospective review of 100 consecutive patients. Sixty-four patients underwent transthoracic echocardiography and 36 transesophageal echocardiography. Types of VSDs included 34 muscular, 32 perimembranous, 18 malaligned, 11 inlet, four outlet, and one acquired. Ages ranged from 1 day to 77 years, and body weights from 3 to 92 kg. Three-dimensional echocardiographic full-volume mode with standard XYZ and adjustable plane cropping, 3D full-volume mode with iCrop, and narrow-sector live 3D protocols were compared for feasibility and accuracy to obtain a diagnostic-quality en face view of a VSD. RESULTS: The success rate for obtaining a high-quality en face image for the three protocols was 100% for full-volume mode with iCrop, 97% for full-volume standard mode, and 94% for narrow-sector live 3D mode. The ability of both full-volume mode with iCrop and full-volume standard mode to demonstrate a VSD was slightly better than that of narrow-sector live 3D mode (P < .001 for both vs narrow-sector live 3D mode). In all patients, the type, size, and location of the VSD were demonstrated accurately by two or more of the protocols. CONCLUSIONS: Three-dimensional echocardiography of VSDs is feasible and accurate in most patients using defined protocols. The protocols are described and illustrated in detail, and a reference 3D image collection is presented.