Background-Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. Methods and Results-Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 wereincluded from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0;© 2015 American Heart Association, Inc.Circulation characterized by apical displacement of the valve or leaflet deformation, respectively. 3 In severe cases of dysplasia, the tricuspid valve orifice may become unguarded. 4,5 Although there is a broad morphologic spectrum, these malformations lead to the same hemodynamic burden, namely, tricuspid regurgitation (TR) and its pathophysiological sequelae. Although older children and adults with EA/TVD may be asymptomatic for years, the diagnosis of EA/TVD in the perinatal period carries a poor prognosis. In the fetus, severe TR may lead to cardiomegaly, hydrops, and arrhythmia, with demise rates as high as 48%.6 Among prenatally diagnosed patients who survive to live birth, hemodynamic instability, cyanosis, and respiratory compromise are common. Although neonatal mortality approached 80 to 85% in early series, 7,8 various single-center series have reported reduced mortality in the past 2 decades, ranging from 17% to 56%. 9-12 Clinical Perspective on p 489Fetal risk factors for perinatal mortality have been identified, including lack of antegrade flow across the pulmonary valve and retrograde duct flow 9-12 and fetal distress. 12However, studies were limited by small sample sizes, with Yu et al 12 reporting the largest series to date with 46 prenatally diagnosed patients. The prognostic value of indices of cardiomegaly, such as the cardiothoracic area (CTA) ratio and the right atrial area index, has been mixed. [9][10][11][12][13] Importantly, hemodynamic factors with potentially important influences on perinatal mortality, such as right ventricular pressure and the presence of pulmonary regurgitation (PR), have not been investigated.Since our understanding of fetuses diagnose...

Chest ultrasound performed by cardiac intensivists allows for an early and accurate diagnosis of abnormal diaphragmatic motion, as evidenced by their ability to predict fluoroscopy findings in pediatric cardiothoracic patients. Training in ultrasound-guided assessment of diaphragmatic motion should be reinforced during pediatric cardiac intensive care fellowship.

Use of dexmedetomidine in patients with CHD and patients following cardiothoracic surgery is not associated with any significant ECG interval abnormalities other than a trend towards lower HR.

BackgroundWe sought to identify cardiovascular magnetic resonance (CMR) parameters associated with successful univentricular to biventricular conversion in patients with small left hearts.MethodsPatients with small left heart structures and a univentricular circulation who underwent CMR prior to biventricular conversion were retrospectively identified and divided into 2 anatomic groups: 1) borderline hypoplastic left heart structures (BHLHS), and 2) right-dominant atrioventricular canal (RDAVC). The primary outcome variable was transplant-free survival with a biventricular circulation.ResultsIn the BHLHS group (n = 22), 16 patients (73%) survived with a biventricular circulation over a median follow-up of 40 months (4–84). Survival was associated with a larger CMR left ventricular (LV) end-diastolic volume (EDV) (p = 0.001), higher LV-to-right ventricle (RV) stroke volume ratio (p < 0.001), and higher mitral-to-tricuspid inflow ratio (p = 0.04). For predicting biventricular survival, the addition of CMR threshold values to echocardiographic LV EDV improved sensitivity from 75% to 93% while maintaining specificity at 100%. In the RDAVC group (n = 10), 9 patients (90%) survived with a biventricular circulation over a median follow-up of 29 months (3–51). The minimum CMR values were a LV EDV of 22 ml/m2 and a LV-to-RV stroke volume ratio of 0.19.ConclusionsIn BHLHS patients, a larger LV EDV, LV-to-RV stroke volume ratio, and mitral-to-tricuspid inflow ratio were associated with successful biventricular conversion. The addition of CMR parameters to echocardiographic measurements improved the sensitivity for predicting successful conversion. In RDAVC patients, the high success rate precluded discriminant analysis, but a range of CMR parameters permitting biventricular conversion were identified.

Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live‐born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23‐center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live‐born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live‐born patients, 7 received comfort care, 11 died <24 hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1–5.0], 95% CI, per m/s; P =0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3–14.2]; P =0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome. Conclusions Among neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High‐risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival.

Cor Triatriatum Dexter (CTD) is a rare congenital anomaly involving the systemic venous valves. Failure of regression of the right‐sided sinus venosus valve leads to abnormal septation of the right atrium and a variety of right atrial and tricuspid valve obstructive lesions. The presentation can be varied ranging from asymptomatic to persistent neonatal cyanosis. We describe a late diagnosis of CTD in a 10‐month‐old male with persistent hypoxia despite balloon valvuloplasty for mild pulmonic valve stenosis with a large secundum atrial septal defect and a mildly hypoplastic right ventricle.

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Free-breathing cine imaging with Cine-Nav yielded comparable image quality and ventricular measurements to BH, and was superior to 3AVG.