Utilizing Fetal Echocardiography to Risk Stratify and Predict Neonatal Outcomes in Fetuses Diagnosed with Congenital Heart Disease.

OBJECTIVE: Risk stratification of fetuses diagnosed with congenital heart disease (CHD) helps provide a delivery plan and prepare families and medical teams on expected course in the delivery room. Our aim was to assess the accuracy of echocardiographically determined risk-stratification assignments in predicting postnatal cardiac outcomes beyond the delivery room. STUDY DESIGN: This was a retrospective study at a single center evaluating all fetuses with CHD who were risk-stratified by echocardiographically determined level of care (LOC) assignment, ranging from 1a (lowest risk) to 4 (highest risk). All data were collected from January 1, 2017, to November 1, 2021. Outcomes included any unexpected cardiac interventions and neonatal clinical outcomes including in-hospital mortality, the need for prostaglandins or inotropes, and defined critical illness. These outcomes were assessed for each LOC assignment by Fisher's exact test. RESULTS: Out of 817 patients assigned a LOC, a total of 747 fetuses were included in our final cohort with a separate subanalysis of 70 fetuses diagnosed with coarctation of the aorta. The sensitivity and specificity were high for all LOC levels in predicting delivery room needs (93-100%). Higher LOC levels (3-4) had a lower positive predictive value (66-67%) indicating a high false-positive rate. Subjects with higher LOC assignments had a greater frequency of critical illness, hospital mortality, need for inotropes, need for neonatal surgical or catheterization interventions, and need for prostaglandins (p < 0.001 for all outcomes). A post-hoc analysis reviewing LOC assignments revealed a greater tendency to over-assign LOC at higher assignments (19% for LOC 3 and 4) compared to lower assignments (4% for LOC 1 and 2). CONCLUSION: Risk stratification based on fetal echocardiography can predict neonatal clinical outcomes and acuity of postnatal management needs. However, there is greater variability in expected clinical events and an expected degree of false positives for those with higher LOC assignments. KEY POINTS: · Risk stratification utilizing fetal echocardiography can be used to predict neonatal needs.. · Complex heart disease has lower positive predictive value in predicting postnatal clinical needs.. · There is a tendency to over-assign risk of acute hemodynamic instability for complex heart disease.. · False positives are expected when planning high-risk deliveries to avoid compromising situations..

Diagnostic Approach to Recurrent Intracardiac Masses That Present With Systemic Symptoms.

Cardiac masses are difficult to diagnose in the pediatric population, especially in the setting of systemic symptoms. Although multiple imaging modalities are available to characterize cardiac masses, it is important to consider a different diagnostic approach in the setting of recurrent cardiac masses and nonspecific systemic symptoms. We present a case involving a previously healthy adolescent with multiple hospitalizations because of persistent fevers, cachexia, and recurrent cardiac masses. Echocardiography and cardiac computed tomography imaging suggested endocarditis, but the patient failed to respond to multiple intravenous antibiotic treatments. He developed recurrent cardiac masses in the right atrium and right ventricle that were debulked and biopsied. The biopsy did not yield a conclusive diagnosis. The patient returned to the hospital with hemoptysis and large pulmonary pseudoaneurysms that had to be occluded during cardiac catheterization. Given his constellation of symptoms and improvement with steroids during surgical procedures, he was ultimately diagnosed with a variant of Behcet's disease known as Hughes-Stovin syndrome. His symptoms resolved completely with steroids and immunosuppression therapy. Our report reveals the limitations of the standard diagnostic approach toward cardiac masses and the importance of considering response to treatment as a clue to the etiology of an unusual cardiac mass.

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.