Stéphanie M. Levasseur

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era A Multicenter Study

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 were included 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; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). Conclusion— In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

Referral for fetal echocardiography is associated with increased maternal anxiety

Background. Referral for fetal echocardiography (fECHO) is an acute stressor that may induce significant maternal anxiety. To promote good clinical management of expectant mothers in this situation, including adequate screening for possible psychiatric interventions, data are needed regarding the psychosocial functioning of women scheduled for fECHO procedures. Objective. To investigate the association between fECHO and maternal anxiety. Methods. Pregnant women answered two questionnaires before first fECHO. The Spielberger State-Trait Anxiety Inventory (STAI) assessed how subjects feel “now” (state) versus how they “usually feel” (trait). Separate state and trait anxiety scores were calculated; scores were compared between the study cohort and a gestational age-matched historical cohort of 31 pregnant women who did not undergo fECHO. A second questionnaire developed by the investigators ascertained pregnancy specific concerns and characteristics. Results. Forty subjects were enrolled. The mean state score of the fECHO cohort (42.1 ± 15.1) differed from the historical cohort (32.8 ± 11.3; p = 0.006); however there was no difference between trait scores (34.7 ± 10.8 vs. 35.4 ± 12.8; p = 0.8). A multivariate linear regression model controlling for race and maternal age demonstrated that fECHO was a strong independent predictor of maternal state anxiety score (p = 0.004, β = 10.4). Conclusions. Pregnant women presenting for fECHO report high anxiety levels compared with women not presenting for fECHO. Clinician awareness and sensitivity are recommended and further investigation of modifiers of anxiety in this high risk group should be explored.

Parents of children with congenital heart disease prefer more information than cardiologists provide

OBJECTIVES To determine whether pediatric cardiologists and parents of older children with congenital heart disease (CHD) share similar expectations regarding the education and counseling that should be provided to parents of children with CHD in both the prenatal and neonatal period. DESIGN Consenting parents of older children with CHD (age >3 years) and cardiologists ranked the importance of education topics on a scale of 1 (unimportant) to 10 (very important). The rankings of parents and cardiologists were compared using Students t-test. RESULTS We had 38 cardiologists and 41 parents complete the questionnaire. There was a statistically significant difference in rankings between cardiologist and parents of children with CHD (P<0.03). Parents consistently ranked topics as more important than cardiologists with a mean difference in rank score of 0.85 ±0.3. In the prenatal period, the most significant differences between parents and cardiologists were noted for information related to the childs quality of life. For neonatal counseling, the most significant differences were noted for information regarding follow-up care and the parents ability to describe the childs CHD to medical personnel. CONCLUSION Parents of older children with CHD would prefer to receive more counseling and education in the prenatal and newborn period than cardiologists perceive is wanted.

Erratum to: Aiming to Preserve Pulmonary Valve Function in Tetralogy of Fallot Repair: Comparing a New Approach to Traditional Management

Pulmonary valve (PV) incompetence following transannular patch (TAP) repair of tetralogy of Fallot (TOF) results in long-term morbidity and mortality. Valve-sparing repairs have recently gained recognition; however, they may be associated with residual pulmonary stenosis (PS) in patients with small PV z scores. We sought to determine whether a repair that increases the PV annulus and augments the valve leaflet with a biomaterial would result in annular growth and in longer duration of valve competence compared with TAP. Eighty patients (median age 136 days, range 4–350) who underwent surgical repair of TOF between 2010 and 2014 were included in the study. Patients were divided into three groups based on the PV intervention: balloon dilation/valvotomy (n = 29), valve-sparing transannular repair (VSTAR) (n = 19) and TAP (n = 32). Intraoperative, early postoperative and midterm follow-up echocardiographic data (median 19 months, range 1–59) were obtained. The primary outcomes were the presence and severity of pulmonary regurgitation and/or PS. Compared with TAP, VSTAR patients demonstrated significantly less severe PR with 100 % freedom of severe PR immediately post-op (vs. 0 % in TAP), 60 % at 6 months and 20 % at 20 months. There were no differences in PS between VSTAR and TAP at follow-up. A subgroup analysis of the VSTAR group was performed. PV z scores were calculated and fit to a random effects model. Patient data fit the model closely, predicting a reproducible increase in valve annulus size over time. With better short-term and comparable midterm results, VSTAR may be appropriate for TOF repair in patients with small PV that would conventionally require a TAP.

Heart sounds at home: feasibility of an ambulatory fetal heart rhythm surveillance program for anti-SSA-positive pregnancies

Objective:Fetuses exposed to anti-SSA (Sjögren’s) antibodies are at risk of developing irreversible complete atrioventricular block (CAVB), resulting in death or permanent cardiac pacing. Anti-inflammatory treatment during the transition period from normal heart rhythm (fetal heart rhythm (FHR)) to CAVB (emergent CAVB) can restore sinus rhythm, but detection of emergent CAVB is challenging, because it can develop in ⩽24 h. We tested the feasibility of a new technique that relies on home FHR monitoring by the mother, to surveil for emergent CAVB.Study Design:We recruited anti-SSA-positive mothers at 16 to 18 weeks gestation (baseline) from 8 centers and instructed them to monitor FHR two times a day until 26 weeks, using a Doppler device at home. FHR was also surveilled by weekly or every other week fetal echo. If FHR was irregular, the mother underwent additional fetal echo. We compared maternal stress/anxiety before and after monitoring. Postnatally, infants underwent a 12-lead electrocardiogram.Results:Among 133 recruited, 125 (94%) enrolled. Among those enrolled, 96% completed the study. Reasons for withdrawal (n=5) were as follows: termination of pregnancy, monitoring too time consuming or moved away. During home monitoring, 9 (7.5%) mothers detected irregular FHR diagnosed by fetal echo as normal (false positive, n=2) or benign atrial arrhythmia (n=7). No CAVB was undetected or developed after monitoring. Questionnaire analysis indicated mothers felt comforted by the experience and would monitor again in future pregnancies.Conclusion:These data suggest ambulatory FHR surveillance of anti-SSA-positive pregnancies is feasible, has a low false positive rate and is empowering to mothers.

Survival of Three Neonates With Congenital Diaphragmatic Hernia and d-Transposition of the Great Arteries.

Survival is significantly compromised in infants born with congenital diaphragmatic hernia and major cardiac anomalies. Mortality is highest when congenital diaphragmatic hernia occurs in association with d-transposition of the great arteries. We present three infants with congenital diaphragmatic hernia associated with d-transposition of the great arteries from a single institution. All three infants survived to discharge after surgical repair/palliation of both the diaphragmatic hernia and heart defect and are doing well at last follow-up. The clinical course and management of these three patients are described.

Applicability of the modified Duke criteria for the diagnosis of infective endocarditis in children with and without heart disease

We sought to assess whether children with pre-existing heart disease treated for infective endocarditis (IE) were less likely to fulfill the modified Duke criteria for definite IE than those without heart disease. While the modified Duke criteria are widely accepted in research and clinical diagnosis, their applicability in diagnosing IE in children is not as well studied. We performed a retrospective single-center study of children treated for IE from January 1999-December 2009 and compared the proportion of children with and without heart disease who fulfilled modified Duke criteria for definite IE. We also examined these criteria in children with cyanotic heart disease, hospital-acquired IE, or recent cardiac surgery. Fifty (60%) of 84 children treated for IE met modified Duke criteria for definite IE. The proportion of children with and without heart disease who fulfilled modified Duke criteria for definite IE was not significantly different (38/68, 56%, versus 12/16, 75%, respectively, p = 0.26). Patients with cyanotic heart disease or hospital-associated IE were as likely to meet definite criteria as those without. However, children with early postoperative IE were less likely than those with late postoperative or without cardiac surgery to meet definite IE criteria (12/31, 39%, versus 38/53, 72%, p = 0.005). Only 60% of children treated for IE met modified Duke criteria for definite IE. Children with early postoperative IE were least likely to fulfill these criteria. Future studies should evaluate potential strategies to improve the diagnosis of IE in children.

Outcomes of cardiac surgery in patients less than 2.5 kg: impact of patient-dependent and patient-independent variables

OBJECTIVE A recent Society of Thoracic Surgeons database study showed that low weight (<2.5 kg) at surgery was associated with high operative mortality (16%). We sought to assess the outcomes after cardiac repair in patients weighing <2.5 kg versus 2.5 to 4.5 kg in an institution with a dedicated neonatal cardiac program and to determine the potential role played by prematurity, the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) risk categories, uni/biventricular pathway, and surgical timing. METHODS We analyzed the outcomes (hospital mortality, early reintervention, postoperative length of stay, mortality [at the last follow-up point]) in patients weighing <2.5 kg at surgery (n = 146; group 1) and 2.5 to 4.5 kg (n = 622; group 2), who had undergone open or closed cardiac repairs from January 2006 to December 2012 at our institution. The statistical analysis was stratified by prematurity, STAT risk category, uni/biventricular pathway, and usual versus delayed surgical timing. Univariate versus multivariate risk analysis was performed. The mean follow-up was 21.6 ± 25.6 months. RESULTS Hospital mortality in group 1 was 10.9% (n = 16) versus 4.8% (n = 30) in group 2 (P = .007). The postoperative length of stay and early unplanned reintervention rate were similar between the 2 groups. Late mortality in group 1 was 0.7% (n = 1). In group 1, early outcomes were independent of the STAT risk category, uni/biventricular pathway, or surgical timing compared with group 2. A lower gestational age at birth was an independent risk factor for early mortality in group 1. CONCLUSIONS A dedicated multidisciplinary neonatal cardiac program can yield good outcomes for neonates and infants weighing <2.5 kg independently of the STAT risk category and uni/biventricular pathway. A lower gestational age at birth was an independent risk factor for hospital mortality.

Fetal Myocardial Strain Abnormalities in Pulmonary Atresia with Intact Ventricular Septum

Global and regional myocardial deformation have not been well described in fetuses with pulmonary atresia and intact ventricular septum (PA/IVS). Speckle‐tracking echocardiography (STE), an angle‐independent technique for assessing global and regional strain, may be a more sensitive way of determining ventricular systolic dysfunction compared with traditional 2D echocardiography. The aim of this study was to assess myocardial deformation in fetuses with PA/IVS compared with control fetuses and to determine if, in fetuses with PA/IVS, strain differs between those with and those without right ventricle‐dependent coronary circulation (RVDCC).

Cardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair

Background: Repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) is a challenging operation increasingly being performed as a complete, primary repair in infancy. Previous studies have focused on perioperative outcomes; however, midterm valve function, ventricular function, and residual obstruction have received little attention. Methods: We retrospectively reviewed 20 patients who underwent CAVC/TOF repair (January 2005 to December 2014). A two-patch repair was used in all patients to correct the CAVC defect. Tetralogy of Fallot repair included transannular patch in 11 (65%) patients and valve-sparing in 6 (35%) patients. Results: The average age at surgery was 72 ± 122 weeks, 40% were male, and 80% had trisomy 21. Mean echo follow-up was 3.0 ± 3.0 years. There were no in-hospital or late mortalities. The rate of reoperation was 20%. At the latest follow-up, moderate left atrioventricular valve regurgitation was present in three (15%) patients and mild stenosis present in seven (35%) patients. One (5%) patient had moderate right ventricular outflow tract (RVOT) obstruction. The valve-sparing population was smaller at the time of surgery than the non-valve-sparing cohort (body surface area: 0.28 ± 0.04 vs 0.42 ± 0.11, P = .002) and less likely to have had a previous shunt (0% vs 64%, P = .01). Among the valve-sparing patients (six), at the latest follow-up, moderate pulmonary insufficiency was present in two (33%) patients. Conclusion: Repair of CAVC concomitant with TOF can be performed with low mortality and acceptable perioperative morbidity. Management of the RVOT remains a challenge for the long term.