Although commonly performed, optimal techniques, strategies, and content to achieve the most effective prenatal counseling have not been explored. We investigate the efficacy of prenatal counseling via survey feedback of parents of children with prenatally diagnosed single ventricle. Grades of counseling using a Likert scale (1–5) were solicited to assess: 1) overall impression of quantity of counseling, 2) explanation of the heart defect, 3) preparation for heart surgery, 4) preparation for hospital course and care, 5) preparation for complications and outcomes of a Fontan circulation, and 6) preparation for neurological, school related or behavioral problems. Impressions were solicited concerning specific providers. A comprehensive fetal counseling score was calculated for each participant. Burden of care including length of hospitalization was explored as impacting prenatal counseling grades. There were 59 survey respondents. Average age of the children at the time of survey was 4.6 ± 3.3 years (range 1–10 years). Highest grades were for explanation of the heart condition, with lowest grades for preparation for neurological, school related or behavioral problems. Cardiac surgeon received the highest with social worker lowest grade for provider. Negative correlation was found between the composite fetal counseling score and parental recollection of length of hospitalization (Pearson r = − 0.357, p < 0.01). Prenatal counseling for neurological, school related and behavioral problems in single ventricle is deficient. Further studies analyzing prenatal counseling techniques and content, can help improve upon the delivery of this important aspect of prenatal care.

Transit from fetal to newborn life is well tolerated in most forms of congenital heart disease, however some are immediately unstable at birth and at risk for demise once separated from placental circulation. Through a strategy designed as Immediate Postpartum Access to Cardiac Therapy (IMPACT), fetuses are born via Caesarean section in a cardiac operating room, expediently moved to an adjacent hybrid suite for catheter-based or surgical intervention, and managed by a highly skilled, dedicated multidisciplinary team of providers. We report our experience with the IMPACT procedure and outcomes. We reviewed all IMPACT procedures since program's inception. Candidacy for IMPACT was considered in high-risk patients in five physiologic categories: 1) obstruction of pulmonary venous return (e.g., hypoplastic left heart/intact atrial septum), 2) inadequate mixing with hypoxemia (e.g., transposition of the great arteries/restrictive foramen ovale), 3) inadequate heart rate (e.g., complete heart block), 4) right-sided heart failure (e.g., severe Ebstein's), or 5) other (e.g., pulmonary arteriovenous fistula). Electronic medical records of newborn and mother were reviewed. A cohort of 96 subjects was identified. Pregnancy complications included HELLP (1), pre-eclampsia (2) and hypertension (2). IMPACT involved first Caesarean delivery in 77 (81%) with 4 (5%) maternal operative complications including: postoperative bleeding requiring surgery (1), anemia with transfusion (1), intraoperative chest pain and desaturations (1), and pneumonia (1). There was no maternal mortality. Fetal hydrops was associated with neonatal mortality (p-value 0.02). Newborn survival to discharge was 65%, ranging 44% in Category 5 to 100% in Category 2. The IMPACT strategy orchestrates numerous services to provide opportunity for optimising outcomes in a high-risk fetal population with a low maternal complication rate. Despite anticipated neonatal demise, nearly two in three survive to hospital discharge.

Introduction: Fetuses with heterotaxy syndrome (HS) and congenital heart disease continue to have worse outcomes compared to other lesions. We sought to explore differences between asplenia and polysplenia subtypes and to identify risk factors for poor outcome in the current era. Methods: All cases of fetal HS seen at our center between 1/2005 and 3/2018 were included in this study. Prenatal echocardiographic parameters and clinical outcomes were recorded. Findings were compared between the asplenia and polysplenia subtypes via Chi-square or Fisher’s exact analysis. Univariate logistic regression was utilized to identify prenatal variables predictive of fetal demise. Postnatal survival was examined with Kaplan-Meier curves and univariate Cox proportional hazards models. Results: The cohort consisted of 155 subjects: 86 with asplenia and 69 with polysplenia. There were 36 with biventricular (2V) and 119 with univentricular (1V) physiology. Asplenia subjects were more likely to have 1V physiology, common atrioventricular canal (CAVC), conotruncal anomaly, and pulmonary venous obstruction (all p<0.001). Polysplenia subjects were more likely to have systemic venous anomalies (p<0.001), complete heart block (CHB, p<0.001), ventricular dysfunction (p=.005), and hydrops (p=0.03). There were 105 subjects born alive, 7 fetal demises, 40 terminations, and 3 lost to follow-up prior to birth. Postnatal follow-up data were available for 96/105 (91%) subjects with median follow-up of 5.5 years (IQR 1.2-10.5). Estimated 5-year survival was 72% (95% CI 62-80) for the entire cohort and there was no difference in overall postnatal survival between polysplenia and asplenia subtypes (p=0.24). CHB and ventricular dysfunction were significant risk factors for both fetal demise and postnatal death. Pulmonary venous obstruction and CAVC were additional risk factors for postnatal death. Conclusions: Fetuses with HS continue to have high rates of postnatal mortality, particularly if CHB, ventricular dysfunction, or pulmonary venous obstruction are identified. CHB and ventricular dysfunction are also significant risk factors for fetal demise. Further research is needed to improve management of CHB and pulmonary venous obstruction in this high-risk group.

Prenatal diagnosis is optimal for detecting congenital heart disease (CHD), however it can generate stress, depression and anxiety in pregnant mothers. Elevated maternal cortisol due to the psychological impact of the diagnosis may impart physiologic effects on the fetus. We hypothesise that measures of maternal stress, depression and anxiety are associated with maternal salivary cortisol as a biomarker of stress in women carrying a fetus with CHD. We evaluated women carrying a fetus with CHD and a control group of pregnant women with a normal fetus. Between 32-36 weeks' gestation, awakening, peak and evening salivary cortisol samples were collected in each of two days prior to an investigational study visit, which included fetal echo and maternal psychometric testing. Participants included 43 cases and 20 normal controls. Traumatic stress and state anxiety were significantly higher in cases in comparison to controls (p=0.007, p=0.021). Mean depression scores were higher for case subjects but did not reach statistical significance. Average of day 1 and day 2 evening salivary cortisol was significantly higher for cases in comparison to controls (p<0.05). Day 1 Area Under the Curve-Increase (AUC-I), a derivative measure characterising the magnitude of cortisol response, was positively associated with anxiety (p<0.05) and with depression in cases (p<0.05). No association was found between psychometric testing or cortisol and the fetal echo spectral Doppler parameters of middle cerebral, umbilical or maternal uterine pulsatility indices. Prenatal diagnosis of congenital heart disease is associated with higher maternal evening cortisol levels in comparison to controls. Maternal anxiety and depression influence salivary cortisol when carrying a fetus with CHD. Maternal psychological state as a consequence of prenatal diagnosis of CHD can impact maternal hormonal physiology, which may influence fetal development. Further study is warranted in looking at the relationship between maternal cortisol and fetal/neonatal outcomes.

BACKGROUND:We sought to describe current outcomes and risk factors for mortality for fetuses diagnosed with absent pulmonary valve syndrome (APV). Fetuses with APV were divided into two cohorts, those with underlying tetralogy of Fallot (TOF/APV) and those without underlying TOF and either an intact ventricular septum or small ventricular septal defect (APV/IVS). METHODS:The fetal echocardiographic database was reviewed from January 1, 2001, until June 1, 2010, and all subjects with a diagnosis of APV were included. Multiple clinical and fetal echocardiographic measurements were recorded. Statistical analysis was performed by χ2 analysis and t tests. Survival analysis was performed by Kaplan-Meier analysis. Significant relationships between variables were explored by regression analysis. Significance was set at p=0.05. RESULTS:The cohort consisted of 15 fetuses with TOF/APV and 6 fetuses with APV/IVS. There were no fetal demises in either cohort. Survival to birth was 71% in the TOF/APV cohort and 83% in the APV/IVS cohort (p=0.62). Of subjects born alive, survival was 80% for both cohorts (p=0.95). However, in the APV/IVS cohort, transplantation-free survival was only 20%. Underlying single-ventricle physiology strongly predicted those who underwent heart transplantation (p=0.003, R2=0.50). For the entire APV cohort, left ventricular dysfunction (p=0.005, R2=0.41) and a higher pulmonary artery valve-to-aortic valve ratio (p=0.02, R2=0.34) predicted mortality. CONCLUSIONS:Postnatal outcomes continue to improve for fetuses with APV syndrome. Left ventricular dysfunction and higher pulmonary artery valve-to-aortic valve ratio accurately predict postnatal mortality for fetuses with APV.

Prenatal diagnosis of congenital heart disease (CHD) has many benefits but can also generate increased stress and anxiety for pregnant mothers. Alteration of maternal cortisol patterns may mediate stress effects to the fetus. Our objective was to assess if traumatic stress is positively associated with maternal salivary cortisol as a biomarker of stress in pregnant women carrying a fetus with CHD. In addition, we analyzed whether depression, anxiety, and severity of CHD moderate cortisol levels in this population. This was a prospective, case-control study. Case subjects were women carrying a fetus with CHD potentially requiring operative or catheter-based care within the first 6 months of life; controls were women referred for fetal echocardiography with no cardiac abnormalities detected. Awakening, peak and evening salivary cortisol samples were collected on the two days prior to the investigational study visit at 32-36 weeks gestation, at which all underwent fetal echocardiography and testing for traumatic stress (Inventory of Events Scale, IES), anxiety (State-Trait Anxiety Inventory, STAI), and depression (Beck Depression Inventory, BDI). Scores for IES, STAI for state anxiety and BDI were higher for case subjects (n=29) in comparison to controls (n=13), but did not reach statistical significance. Awakening cortisol levels on the day prior to the study visit correlated with increased traumatic stress (p=0.04), state anxiety (p=0.02) and depression (p=0.04) (See table). Fetal CHD severity was not associated with cortisol levels. Increased maternal cortisol is associated with clinically important maternal traumatic stress, anxiety and depression in pregnant women between 32-36 weeks gestation. Maternal salivary cortisol may function as a biomarker for stress in those carrying a fetus with CHD. The physiological impact of increased maternal cortisol on fetal and postnatal well being is worthy of further exploration.Tabled 1Psychological testing scores and awakening cortisol levels Open table in a new tab

Prenatal diagnosis of congenital heart disease (CHD) can lead to maternal stress during pregnancy. Time between initial prenatal diagnosis and birth creates an opportunity for anxiety, promoting increased maternal stress. We sought to investigate the effect of a sustained protocol of serial prenatal counseling on maternal stress following prenatal diagnosis of CHD. Psychometric self-report instruments were administered to mothers carrying a fetus with CHD and measured: 1) traumatic stress (Impact of Events Scale-Revised, IES-R), 2) depression (Beck Depression Index II, BDI), and 3) anxiety (State-Trait Anxiety Index, STAI). Serial assessment was performed at 3 encounters. Cut-off score of > 24 on IES-R indicated clinically important post-traumatic stress. Serial prenatal counseling in all cases was performed every 4 weeks from initial encounter until delivery. Counseling sessions included a physician, nurse coordinator and social worker and utilized a protocol approach with review of diagnosis, implications, management strategies, short and long-term outcomes in addition to other support. Supplemental counseling between nurse coordinator and patient through phone and email was performed in between visits. Piecewise linear mixed models was used for analyzing the change in IES-R over time. 59 mothers completed initial measures and 32 pregnant women were serially evaluated at 3 gestational intervals (time 1: 27 + 3 wks; time 2: 31 + 3 wks; time 3: 34 + 3 wks). At first evaluation, clinically important traumatic distress was seen in 39%, depression in 22%, and state anxiety in 31%. There was a significant decrease in the percentage of scores above the clinical cutoff for traumatic distress at each visit (IES-R > 24 at time 1 = 39%; time 2 = 32%; time 3 = 22%, P < 0.01). Post-traumatic stress, depression and anxiety are common after prenatal diagnosis of CHD. Multiple encounters after initial prenatal diagnosis of CHD allows for serial prenatal counseling and focused education which reduces maternal traumatic distress.

Objective To determine whether prenatal diagnosis of congenital heart disease (CHD) increases maternal stress.Study design Self-report instruments were administered to mothers carrying a fetus with CHD. Domains included: (1) traumatic stress (Impact of Events Scale-Revised); (2) depression (Beck Depression Index II); and (3) anxiety (State-Trait Anxiety Index). Modifiers included: (1) coping skills (COPE Inventory); (2) partner satisfaction (Dyadic Adjustment Scale); and (3) demographics. Multivariate linear regression models were used to assess relationships between stress measures and modifiers.Results Fifty-nine mothers (gestational age 27 +/- 3 weeks) completed all measures. Clinically important traumatic distress was seen in 39%, depression in 22%, and state anxiety in 31%. Lower partner satisfaction was associated with higher depression (P < .01) and higher anxiety (P < .01). After controlling for partner satisfaction and income, " denial" was most associated with increased traumatic stress, anxiety, and depression (P < .01).Conclusions Posttraumatic stress, depression, and anxiety are common after prenatal diagnosis of CHD. Healthy partner relationships and positive coping mechanisms can act as buffers. (J Pediatr 2013;162:302-7).

To review our experience with the prenatal diagnosis of hypoplastic left heart syndrome (HLHS). Our goal was to establish the benchmark for perinatal and early surgical outcome in the current era, from a center with an aggressive surgical approach and a cohort with a high level of intention‐to‐treat.

Absence of the semilunar valve leaflets is an uncommon cardiac congenital anomaly. Typically, absence of the pulmonary valve leaflets (APVL) is associated with tetralogy of Fallot (ToF) severe pulmonary insufficiency, pulmonary artery dilation and respiratory failure at birth. However, APVL can also occur in other forms of congenital heart disease and little is known about absent aortic valve leaflets (AAVL). Review our experience with prenatal diagnosis and outcome of APVL and AAVL. Methods: Our database was searched; 15 cases were identified between 2001–8; 13 APVL and 2 AAVL. Anatomical features, diagnostic accuracy and outcome were reviewed. Mean GA at initial evaluation was 26 ± 6 wks, range 14 to 34. Of 2 with AAVL, 1 had cystic hygroma, Turner's syndrome and died at 15 wks. The other has interrupted aortic arch type B and is still in utero. Of the 13 with APVL, 9 had classic ToF; 4 had other findings: tricuspid atresia, VSD and complete heart block (n = 1), tricuspid atresia, intact ventricular septum (n = 1), aorto-pulmonary window and intact ventricular septum (n = 1), and tricuspid stenosis, intact ventricular septum and subaortic stenosis (n = 1). Fetal echocardiography was highly reliable and accurate in comparison to postanatal imaging. For all 15 fetuses there were a total of 3 terminations (TAB), one intra-uterine demise and 2 are still in-utero. Of the 5/9 with classic ToF findings who were live-born, 1 died due to respiratory failure/prematurity, 4 survived surgery and are alive, one of these with tracheostomy. Of the 4 with APVL and non-ToF anatomy 1 died before surgery, 1 had heart transplant, and 2 survived surgical palliation but both have tracheostomy. Of 8 live-born in our series, only 3 are repaired and breathing spontaneously. APVL and AAVL are uncommon anomalies, when diagnosed prenatally, have relatively poor outcome. APVL has the highest morbidity and mortality when associated with non-ToF type of disease.

Transition from prenatal to postnatal life imposes the demands of respiration and altered cardiac load on the newborn. Some forms of congenital heart disease while stable in-utero can become unstable at birth, with separation from placental circulation resulting in neonatal demise. We sought to determine if fetuses at risk could have improved outcome with a programmatic effort of delivery and immediate intervention at the earliest point in time following birth. A multidisciplinary team of obstetricians, maternal-fetal medicine specialists, anesthesiologists, cardiologists, surgeons and nurses was put together to facilitate immediate postpartum access to cardiac therapy (IMPACT procedure). Organizational efforts focused on (1) Caesarian section delivery in our cardiac operating room suite (2) infant resuscitation and stabilization by cardiothoracic anesthesiologists, and (3) catheter/surgical intervention within the first few minutes of life following separation from placental circulation. Sixteen fetuses in which postnatal outcome was anticipated to be extremely poor were delivered via IMPACT procedure;12 high-risk hypoplastic left heart syndrome (HLHS), 3 complete heart block (CHB) with early hydrops fetalis, and 1 giant pulmonary arteriovenous malformation (PAVM). Of these, 9 survived to hospital discharge (56%). All are doing well at 6-36 months following delivery. The IMPACT procedure improves outcome for severe cardiovascular anomalies at risk for death at placental separation. Organization of a multidisciplinary team focused on offering high-level care for both mother and fetus in a single locale can positively influence outcome for these unique fetuses at risk.Tabled 1IndicationNAliveHLHS with intact atrial septum106Single ventricle, heterotaxy with CHB21Maternal lupus mediated CHB11HLHS with anomalous pulmonary veins10HLHS with severe ventricular dysfunction10Giant PAVM11 Open table in a new tab