Lisa W. Howley

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.

Enteral feeding in neonates with prostaglandin-dependent congenital cardiac disease: international survey on current trends and variations in practice

BACKGROUND The benefits of early enteral feeding in neonates are well known and yet the optimal pre-operative nutrition of prostaglandin-dependent infants with congenital cardiac disease remains ill-defined. This survey delineates international nutritional practices and trends with this population. MATERIALS AND METHODS Paediatric practitioners responded to an Internet-based survey that explored assessment of feeding practices, criteria for feeding readiness, medication dosing, concurrent feeding with umbilical catheters, and the observed incidence of feeding intolerance. Documented nutritional strategies were not correlated with patient outcomes. RESULTS A total of 200 caregivers responded to the survey. Fewer United States caregivers (56%) reported routine pre-operative enteral feeding in prostaglandin-dependent infants when compared with caregivers outside the United States of America (93%). Of those respondents willing to feed, approximately two-thirds did not base their decision on the ductal flow direction. Numerous and heterogeneous parameters were reported to assess feeding readiness. Many caregivers report scepticism with regard to enteral feeding safety in neonates with an umbilical artery catheter, and to a lesser extent in the presence of an umbilical venous catheter. In summary, there is a prevailing lack of consensus regarding pre-operative enteral nutrition to prostaglandin-dependent neonates. CONCLUSIONS This survey demonstrates noticeable variations in pre-operative nutritional practices between providers from around the world. Arguments that support or refute this practice have little support in the medical literature. Future studies should aim to demonstrate the safety of such practice and compare the outcomes of prostaglandin-dependent neonates who were pre-operatively enterally fed with those who were not.

Changes in fetal cardiac axis between 8 and 15 weeks' gestation

To document changes in the normal embryonic/fetal cardiac axis in the late first and early second trimesters of pregnancy.

Flow patterns in the ductus arteriosus during open fetal myelomeningocele repair

The objective of this study is to perform a longitudinal evaluation of blood flow patterns in the ductus arteriosus (DA) during the perioperative period in fetal myelomeningocele (MMC) surgical patients.

Ex-utero intrapartum treatment as a novel bridging strategy to surgery in hypoplastic left heart syndrome with intact atrial septum-cross-circulation revisited.

Hypoplastic left heart syndromewith an intact or nearly intact septum (HLHS-IS/NIS) constitutes 6%-20% of hypoplastic left heart syndrome cases and represents themost severe form of the disease. Unless there is a pathway for pulmonary venous decompression, hemodynamic instability and death ensue within minutes to hours after birth. Survival depends upon emergent relief of this obstruction, either by operative or interventional techniques. The optimal strategy to bridge a newborn to a stable circulation remains undefined. We describe a novel EXIT (ex-utero intrapartum treatment) strategy using maternal-placental support to bridge a fetus with HLHS-IS/NIS to palliative intervention. A 26-year-old mother was referred for a fetal echocardiogram (at 30 weeks gestation), and the diagnosis of HLHSIS/NIS (with aortic atresia, mitral stenosis) was confirmed (Figure 1, A). The family elected to proceed with fetal intervention, which was performed (at 31 weeks) in another institution. This intervention was complicated by interatrial stent malposition with no demonstrable flow across the septum, and pleural effusion (Figure 1, B). As delivery neared, the parents requested aggressive management. Although our usual approach at birth is immediate catheter intervention, this strategy would be complicated by the oblique stent position in the right atrium. An EXIT to achieve cardiopulmonary bypass (CPB) was proposed to allow the fetus to remain on placental support while undergoing median sternotomy and initiation of CPB. The strategy was the consensus from a multidisciplinary fetal care conference as the safest means of maintaining hemodynamic stability before establishing interatrial communication. After the mother suffered premature rupture of membranes, a multidisciplinary team was mobilized for an emergent EXIT procedure. General anesthesia was induced using

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.

Right Atrial Dysfunction in the Fetus with Severely Regurgitant Tricuspid Valve Disease: A Potential Source of Cardiovascular Compromise

Background: In severe right heart obstruction (RHO), redistribution of cardiac output to the left ventricle (LV) is well tolerated by the fetal circulation. Although the same should be true of severely regurgitant tricuspid valve disease (rTVD) with reduced or no output from the right ventricle, affected fetuses more frequently develop hydrops or suffer intrauterine demise. We hypothesized that right atrium (RA) function is altered in rTVD but not in RHO, which could contribute to differences in outcomes. Methods: Multi‐institutional retrospective review of fetal echocardiograms performed over a 10‐year period on fetuses with rTVD (Ebsteins anomaly, tricuspid valve dysplasia) or RHO (pulmonary atresia/intact ventricular septum, tricuspid atresia) and a healthy fetal control group. Offline velocity vector imaging and Doppler measurements of RA size and function and LV function were made. Results: Thirty‐four fetuses with rTVD, 40 with RHO, and 79 controls were compared. The rTVD fetuses had the largest RA size and lowest RA expansion index, fractional area of change, and RA indexed filling and emptying rates compared with fetuses with RHO and controls. The rTVD fetuses had the shortest LV ejection time and increased Tei index with a normal LV ejection fraction. RA dilation (odds ratio, 1.27; 95% CI, 1.05–1.54) and reduced indexed emptying rate (odds ratio, 2.49; 95% CI, 1.07–5.81) were associated with fetal or neonatal demise. Conclusions: Fetal rTVD is characterized by more severe RA dilation and dysfunction compared with fetal RHO and control groups. RA dysfunction may be an important contributor to reduced ventricular filling and output, potentially playing a critical role in the worsened outcomes observed in fetal rTVD. HighlightsThis is the first study to examine intrinsic right atrial function using velocity vector imaging in fetuses with and without right heart disease.Fetuses with regurgitant tricuspid valve disease, including Ebsteins anomaly and tricuspid valve dysplasia, have evidence of impaired right atrial function characterized by increased global size, decreased emptying fraction and reservoir function, and reduced indexed emptying and filling rates.Impaired right atrial function in fetuses with regurgitant tricuspid valve disease may compromise fetal cardiac output, potentially contributing to the higher rate of poor outcomes in this population.

Prominent coronary artery flow with normal coronary artery anatomy is a rare but ominous harbinger of poor outcome in the fetus.

Abstract Objectives: “Heart sparing” refers to prominent antegrade fetal coronary artery (CA) blood flow readily visualized by color Doppler and is a harbinger of poor outcome in growth restricted fetus, but little is known of the features and presentation of heart sparing in normally grown fetuses. Our objective was to describe heart sparing effects in normally grown fetuses, and compare the presentation and outcome of heart sparing between fetuses with growth restriction and those who were normally grown. Methods: In a series of fetuses with prominent antegrade CA flow, we assessed Doppler flow profiles in the aortic isthmus, ductus venosus (DV), umbilical vein (UV), umbilical artery (UA) and middle cerebral artery (MCA). We calculated MCA and UA systolic/diastolic ratios and the cerebral placental ratio, and measured fetal biometry. We evaluated cardiac function using the myocardial performance index (MPI) and the cardiovascular profile score (CVPS). Results: Ten fetuses with heart sparing had normal DV flow at 24–36.6 (mean 30.9) weeks of gestation. Five had growth restriction (Group 1); 4/5 had normal MPI and CVPS, and one died. Five were normally grown (Group 2); 5/5 had elevated MPI and decreased CVPS, of these 2 died in utero and one died immediately after birth despite urgent delivery. Coronary arteries were normal after birth or autopsy. Conclusions: Heart sparing confers a poor prognosis in fetal growth restriction and in normally grown fetuses with cardiac dysfunction. We suggest CA flow be assessed in all high-risk fetuses.

Simultaneous recording of pulsed‐wave Doppler signals in innominate vein and transverse aortic arch: new technique to evaluate atrioventricular conduction and fetal heart rhythm

Fetal heart rhythm abnormalities are common. Simultaneous pulsed Doppler interrogation of the superior vena cava (SVC) and ascending aorta (AAo) is widely used to analyze fetal arrhythmias. However, the SVC/AAo Doppler technique can be limited by a suboptimal angle of interrogation and poor visualization of atrial systole in the SVC. We present our experience with a novel Doppler technique using simultaneously recorded pulsed wave Doppler signals in the innominate vein (InnV) and transverse aortic arch (Ao) from an axial view of the fetal thorax. Advantages of the InnV/Ao Doppler technique include improved acquisition feasibility and a near 0-degree angle of insonation of the InnV, improving visualization of atrial systolic events.

Impact of Objective Echocardiographic Criteria for Timing of Congenital Diaphragmatic Hernia Repair

Objective To assess the impact of specific echocardiographic criteria for timing of congenital diaphragmatic hernia repair on the incidence of acute postoperative clinical decompensation from pulmonary hypertensive crisis and/or acute respiratory decompensation, with secondary outcomes including survival to discharge, duration of ventilator support, and length of hospitalization. Study design The multidisciplinary congenital diaphragmatic hernia management team instituted a protocol in 2012 requiring the specific criterion of echocardiogram‐estimated pulmonary artery pressure ≤80% systemic blood pressure before repairing congenital diaphragmatic hernias. A retrospective review of 77 neonatal patients with Bochdalek hernias repaired between 2008 and 2015 were reviewed: group 1 included patients repaired before protocol implementation (n = 25) and group 2 included patients repaired after implementation (n = 52). Results The groups had similar baseline characteristics. Postoperative decompensation occurred less often in group 2 compared with group 1 (17% vs 48%, P = .01). Adjusted analysis accounting for repair type, liver herniation, and prematurity yielded similar results (15% vs 37%, P = .04). Group 2 displayed a trend toward improved survival to 30 days postoperatively, though this did not reach statistical significance (94% vs 80%, P = .06). Patient survival to discharge, duration of ventilator support, and length of hospitalization were not different between groups. Conclusions The implementation of a protocol requiring echocardiogram‐estimated pulmonary arterial pressure ≤80% of systemic pressure before congenital diaphragmatic hernia repair may reduce the incidence of acute postoperative decompensation, although there was no difference in longer‐term secondary outcomes, including survival to discharge.