Right Ventricular Strain in Patients With Ductal-Dependent Tetralogy of Fallot.

BACKGROUND: Right ventricular (RV) dysfunction is an independent predictor of poor outcomes in patients with tetralogy of Fallot (TOF), and global longitudinal strain (GLS) is a well-validated echocardiographic technique to measure RV function. Although trends in RV GLS have been examined in patients with TOF, they have not been studied specifically in those with ductal-dependent TOF, a group in which there is not a clear consensus on the best surgical strategy. The aim of this study was to assess the midterm trajectory of RV GLS in patients with ductal-dependent TOF, drivers of this trajectory, and differences in RV GLS between repair strategies. METHODS: This was a retrospective two-center cohort study of patients with ductal-dependent TOF who underwent repair. Ductal dependence was defined as being initiated on prostaglandin therapy and/or undergoing surgical intervention on or before 30 days of life. RV GLS was measured on echocardiography preoperatively, early after complete repair, and at 1 and 2 years of age. RV GLS was trended over time and compared between surgical strategies and with control subjects. Mixed-effects linear regression models were used to evaluate the factors associated with changes in RV GLS over time. RESULTS: Forty-four patients with ductal-dependent TOF were included in the study, of whom 33 (75%) underwent primary complete repair and 11 (25%) underwent staged repair. Complete TOF repair was performed at a median of 7 days in the primary-repair group and 178 days in the staged-repair group. RV GLS improved over time from post-complete repair echocardiography through 2 years of age (-17.4% [interquartile range, -15.5% to -18.9%] vs -21.5% [interquartile range, -18.0% to -23.3%], P < .001). However, compared with age-matched control subjects, patients had worse RV GLS at all time points. There was no difference in RV GLS between the staged and primary complete repair groups at 2-year follow-up. Shorter intensive care unit length of stay after complete repair was independently associated with improvement in RV GLS over time. Strain improved by 0.07% (95% CI, 0.01 to 0.12) for each fewer day in the intensive care unit (P = .03). CONCLUSIONS: RV GLS improves over time among patients with ductal-dependent TOF, though it is consistently reduced compared with control subjects, suggesting an altered deformation pattern in patients with ductal-dependent TOF. There was no difference in RV GLS between the primary- and staged-repair groups at midterm follow-up, suggesting that repair strategy is not a risk factor for worse RV strain in the mid postoperative period. A shorter complete-repair intensive care unit length of stay is associated with an improved trajectory of RV GLS.

Post-operative troponin levels and left ventricular function in patients with d-transposition of the great arteries following the arterial switch operation.

The aim of this study was to assess the significance of post-operative troponin levels as a surrogate for left ventricular (LV) dysfunction measured by global longitudinal strain (GLS) in patients with dextro-transposition of the great arteries (d-TGA) who undergo an arterial switch operation (ASO), and to explore the LV GLS recovery in the mid-term follow-up period. Seventy-eight neonates were included, of whom 41 had troponin-I measurements and 37 had troponin-T measurements. The primary outcome of LV GLS was assessed and compared with healthy controls at the pre-operative stage and time of discharge, 3 months, 6 months and 12 months of age. Secondary outcomes included deaths or transplantations and other clinical markers such as length of hospital stay. D-TGA patients had worse LV GLS post-operatively compared to age-matched controls (p < 0.01) which improved by 12 months of age (p = 0.53). No association was found between changes in troponin-I or troponin-T levels and LV GLS at the time of discharge (r = 0.4, p = 0.64 and r = -0.5, p = 0.91, respectively). In addition, there were no deaths or transplantations in this cohort over a period of 12 months. LV GLS appears to worsen in the early post-operative period for d-TGA patients who undergo neonatal ASO but this recovers through the first post-operative year. Troponin levels have limited value in predicting early or midterm LV dysfunction and recovery.

Current State of Fetal Heart Disease Counseling and Training: Room for Improvement? : Endorsed by the Fetal Heart Society.

We sought to describe the fellowship experiences and current practice habits of pediatric cardiologists who counsel patients with fetal heart disease (FHD) and to identify fellowship experiences related to FHD counseling perceived as valuable by respondents as well as opportunities for improvement. A cross-sectional survey of attending pediatric cardiologists who care for patients with FHD was performed. The respondents' demographics, fellowship experiences related to FHD counseling, reflections on fellowship training, and current practice habits were collected. The Fetal Heart Society endorsed this survey. There were 164 survey responses. 56% of respondents did not have 4th-year subspecialty training in fetal cardiology. Observing and performing FHD counseling were the most commonly used methods of training, with the highest perceived effectiveness. The number of counseling sessions observed and performed correlated moderately with confidence in FHD counseling skills at fellowship graduation. Extracardiac pathology and neurodevelopment were the least frequently addressed topics in fellowship training and in current practice. Fewer than 50% of respondents received formal education and feedback in counseling techniques during fellowship training. A significant proportion of practicing pediatric cardiologists provide FHD counseling with only standard categorical training. This highlights the potential importance of expanding FHD counseling education into categorical fellowship curricula. We suggest increasing opportunities for fellows to perform FHD counseling and receive feedback as this is a valued and beneficial experience during training. A formalized curriculum including extracardiac pathology and neurodevelopment and the use of evidence-based workshops in counseling techniques may address identified gaps in fellowship education.

Midterm Outcomes and Cardiac Magnetic Resonance Imaging following Multisystem Inflammatory Syndrome in Children.

At midterm follow-up visits performed at a median of 7 months (IQR 6.0-8.4 months), 16 patients with multisystem inflammatory syndrome in children had resolution of left ventricular dysfunction and most had resolution of coronary aneurysms. On cardiovascular magnetic resonance imaging, no patients had late gadolinium enhancement.