Faculty Bibliography

Background: Atrial switch operations for dextro-transposition of the great arteries (d-TGA) were supplanted by more physiologic arterial switch operations in the late 1980s, but it is important to recognize the complications faced by aging patients who underwent the older procedure. Case: A 35-year-old woman with d-TGA who underwent the Senning atrial switch procedure as an infant has had stable NYHA Class I functional status, with occasional palpitations but no significant exertional symptoms. Stress testing demonstrated mildly reduced exercise capacity and mild oxygen desaturation at peak exercise. CMR and TTE revealed mild enlargement and normal function of the systemic RV, normal size and function of the subpulmonic LV, moderate TR, and a baffle leak. There was also marked systolic anterior motion (SAM) of the mitral valve, leading to both severe left ventricular outflow tract obstruction (LVOTO) and severe MR in the subpulmonic ventricle. Peak systolic LVOT velocity was 4.5 m/s and peak MR velocity was 5 m/s, corresponding to peak gradients of 80 mm Hg and 100 mm Hg, respectively. Decision-making: There is no consensus on optimal treatment for subpulmonic LVOTO. The dynamic nature of our patient's LVOTO suggests that it might be relieved pharmacologically, and she is being trialed on a beta blocker prior to considering surgery. Importantly, the role of ventricular interdependence cannot be overlooked in patients with inverted ventricular morphologies. Literature on patients with congenitally corrected TGA (cc-TGA) who underwent pulmonary banding or have RV-to-pulmonary artery conduits suggests that relief of LVOTO and reduction of LV pressures may paradoxically lead to RV failure and worsened TR. The mechanism is thought to be related to the advantageous role of the interventricular septum being pushed towards the systemic RV by the pressurized subpulmonic LV, reducing RV sphericity and improving tricuspid valve coaptation. Conclusion(s): Characterization of LVOTO is important for prognosis, pharmacologic management and surgical planning in patients with a systemic RV. Treatment decisions must balance the risk that good relief of LVOTO may actually precipitate systemic RV failure.2019 American College of Cardiology Foundation. All rights reserved

Cardiovascular disease complicating pregnancy is rising in prevalence secondary to advanced maternal age, cardiovascular risk factors, and the successful management of congenital heart disease conditions. The physiological changes of pregnancy may alter drug properties affecting both mother and fetus. Familiarity with both physiological and pharmacological attributes is key for the successful management of pregnant women with cardiac disease. This review summarizes the published data, available guidelines, and recommendations for use of cardiovascular medications during pregnancy. Care of the pregnant woman with cardiovascular disease requires a multidisciplinary team approach with members from cardiology, maternal fetal medicine, anesthesia, and nursing.

OBJECTIVES/OBJECTIVE:To determine whether use of a stylus user input device (UID) would be superior to a mouse for CMR segmentation. METHODS:Twenty-five consecutive clinical cardiac magnetic resonance (CMR) examinations were selected. Image analysis was independently performed by four observers. Manual tracing of left (LV) and right (RV) ventricular endocardial contours was performed twice in 10 randomly assigned sessions, each session using only one UID. Segmentation time and the ventricular function variables were recorded. The mean segmentation time and time reduction were calculated for each method. Intraclass correlation coefficients (ICC) and Bland-Altman plots of function variables were used to assess intra- and interobserver variability and agreement between methods. Observers completed a Likert-type questionnaire. RESULTS:The mean segmentation time (in seconds) was significantly less with the stylus compared to the mouse, averaging 206±108 versus 308±125 (p<0.001) and 225±140 versus 353±162 (p<0.001) for LV and RV segmentation, respectively. The intra- and interobserver agreement rates were excellent (ICC≥0.75) regardless of the UID. There was an excellent agreement between measurements derived from manual segmentation using different UIDs (ICC≥0.75), with few exceptions. Observers preferred the stylus. CONCLUSION/CONCLUSIONS:The study shows a significant reduction in segmentation time using the stylus, a subjective preference, and excellent agreement between the methods. KEY POINTS/CONCLUSIONS:• Using a stylus for MRI ventricular segmentation is faster compared to mouse • A stylus is easier to use and results in less fatigue • There is excellent agreement between stylus and mouse UIDs.

Background: Tricuspid regurgitation (TR) in patients with congenitally corrected transposition of the great arteries (cc-TGA) has been shown to be a major risk factor for adverse outcomes. Pulmonary arterial banding (PAB) procedure shifts the interventricular septum (IVS) towards the systemic ventricle (SV). We hypothesized that the tenting height (TH) of the tricuspid valve (TV) decreases after PAB as a marker of the improved TV competency. Methods: Clinical and echocardiographic analysis of patients with cc-TGA referred for PAB procedure was performed. TV Measurements included TH, TV annular dimensions, TR degree and sphericity index (SI). TH was measured between the coaptation point and the true TV annular plane or the expected plane such as in Ebstein-like TV. Results: 17 patients (70% males) were referred to PAB surgery at a mean age of 3.15+/-4.2 years. Median interval between pre and postsurgery echoes was 293 (IQR 112,386) days. Overall, PAB resulted in a significant decrease in TH [both true (6.8+/-2.2 to5.6+/-1.6 cm; p=0.03) and expected (8.6+/-3.4 to 7.2+/-3.4 cm; p=0.01)]. In patients with mild and above degree of TR (n=10), PAB resulted in significant decrease in TR (by ~1 grade; p=0.026), SV size (by ~1 class; p=0.02) and increase in SI (3.94+/-1.1 to 4.74+/-1.1; p=0.001) (table/image). TV annular dimensions did not significantly change. Conclusions: By shifting the IVS, PAB elongates the SV, resulting in reconfiguration of the TV and reduction in TH with resultant decrease in the degree of TR in patients with cc-TGA. (Figure presented)

BACKGROUND: Anatomic features of obstructive hypertrophic cardiomyopathy are septal hypertrophy, elongated mitral leaflets, and anterior displacement of the papillary muscles. In addition to extended myectomy, the resect-plicate-release operation adds horizontal plication of the anterior mitral leaflet (AML) and release of the anterolateral papillary muscle (APM) in selected patients. The aim of this study was to test the hypotheses that (1) preoperative findings would be associated with procedures applied, (2) anatomic corrections would be observable postoperatively, and (3) there would be consistently good physiologic outcomes. METHODS: A retrospective study was conducted of patients with obstructive hypertrophic cardiomyopathy who had adequate echocardiograms before and 9.5 +/- 12 months after the resect-plicate-release operation was performed from 2006 to 2012. RESULTS: Seventy-seven patients underwent myectomy, 50 AML plication, and 50 APM release. Patients who underwent plication had longer AMLs (32 +/- 4 vs 28 +/- 4 mm; P < .004). Anterior extension of the APM was more common with papillary muscle release (86% vs 62%, P < .04). Twenty-seven (35%) had septal thickness