Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10-year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow-up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P=0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1-30.0; P=0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.

Culture, teamwork and engagement: 40 years of finding the right ingredients.

PURPOSE OF REVIEW: Changing demands in healthcare, new leadership structures and physician preferences on work-life balance have made culture, teamwork and engagement vitally important for the long-term success of medical practices. With recent emphasis placed on culture, teamwork and engagement, leaders have had to acquire management skills that extend beyond the scope of being a successful clinician, educator and researcher. RECENT FINDINGS: Over the last two decades, experts throughout the business and medical fields have worked to define culture. Furthermore, these authors have shown that success in businesses is often rooted in a strong organizational culture. Large surveys have confirmed that physicians value culture. They may join or leave a practice based on the culture. Furthermore, creating, defining and preserving culture requires leaders to be exemplary citizens and to inspire colleagues to be engaged. SUMMARY: Practices and businesses that have been shown to have a strong culture with engaged employees form stronger teams which ultimately confers a competitive advantage. In the current era, culture fit should be considered during any hire. An organization's unique culture actively needs to be taught and cultivated by leaders. Employees will become more engaged and adopt a practice's culture with education and by following the example of others.

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study.

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.

Temporal Trends in Utilization of Transthoracic Echocardiography for Common Outpatient Pediatric Cardiology Diagnoses over the Past 15 Years.

BACKGROUND: No data exist regarding the temporal trends in utilization of transthoracic echocardiography (TTE) in an outpatient pediatric cardiology setting. This study evaluates the trends in utilization of TTE for common diagnoses known to have low diagnostic yield and the factors influencing these trends. METHODS: Patients evaluated at our pediatric cardiology clinics from January 2000 to December 2014 and discharged with final diagnoses of innocent murmur, noncardiac chest pain, benign syncope, and palpitations were included. Variables collected retrospectively included patient age, sex, insurance type, distance from clinic, and ordering physician's years of experience since fellowship. RESULTS: Of the 74,881 patients seen by 35 physicians, 36,053 (48.1%) had a TTE. The TTE rates increased from the beginning of 2000 to the end of 2004 (5.2% per year; P < .001) and then steadily declined until the end of 2014 (1.6% per year; P < .001). Utilization for noncardiac chest pain remained the highest, and use in infants increased significantly during the study period (P < .001). After adjusting for all other factors, the following variables were associated with higher TTE utilization: younger age, males, Medicaid insurance, increased distance from clinic, and being seen by less experienced physicians. Temporal trends persisted after adjusting for all these factors. CONCLUSIONS: After an initial surge in TTE utilization from 2000 to 2004, there was a steady decline. This study identifies some important factors influencing these trends. This information could help design quality interventions, but additional factors need to be explored since the trends persist despite adjusting for these factors.

Results of Primary Repair Versus Shunt Palliation in Ductal Dependent Infants With Pulmonary Atresia and Ventricular Septal Defect.

BACKGROUND: The 2 management strategies of neonates born with ductal-dependent pulmonary atresia and ventricular septal defect (PAVSD) include single stage primary biventricular repair (BVR) or staged palliation with modified Blalock-Taussig shunt (BTS) followed by second stage repair. Each approach is associated with specific benefits and drawbacks. We report outcomes of those 2 different strategies. METHODS: Between 2002 and 2012, 86 neonates with ductal-dependent PAVSD underwent surgery using primary repair (BVR group: n = 28, 33%) or shunt palliation (BTS group: n = 58, 67%). Early and late results were compared between the 2 groups. RESULTS: Median age was 6 days (interquartile range [IQR] 3-17) and median weight was 2.8 kg (IQR 2.5-3.3) with 27 patients (31%) 2.5 kg or less. Associated risk factors such as prematurity 36 weeks or less and genetic or extra-cardiac malformations were present in 30% and 40% of patients, respectively. Hospital mortality occurred in 5 (5.8%) patients (1 [3.6%] for BVR versus 4 [6.9%] for BTS, p = 1.00). Overall 8-year survival was 76.5% (85.5% for BVR versus 72.2% for BTS, p = 0.189). On multivariable analysis, risk factors for mortality were genetic or extra-cardiac malformations (hazard ratio [HR], 2.8 (95% confidence interval [CI] 1.7% to 16.0%), p = 0.036) and postoperative extracorporeal membrane oxygenation (ECMO) (HR, 4.0 [95% CI, 1.1% to 14.4), p = 0.039). Freedom from right ventricular outflow tract reoperation after achievement of repair was 63.2% at 8 years (52.4% for BVR versus 70.2% for BTS, p = 0.170). On multivariable analysis, risk factors for reoperation were the use of conduit (HR, 8.7 [95% CI, 1.1% to 65.8%], p = 0.037) and prematurity (HR, 2.8 [95% CI, 1.1% to 7.2%], p = 0.028). CONCLUSIONS: Primary BVR of neonates with ductal-dependent PAVSD is associated with a trend for improved survival due to hospital and interstage mortality with the staged approach. Genetic or extra-cardiac malformations are common and are associated with worse survival.

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.

Lower extremity edema in a child due to pectus excavatum.

A previously healthy 11-year-old girl was referred for pediatric cardiology evaluation because of the development of progressive bilateral lower extremity swelling over the course of 2 years. Her prior workup had included a negative result for proteinuria and a negative ultrasound for deep venous thrombosis. On physical examination, in addition to her edema, she was found to have a severe pectus excavatum deformity, which prompted cardiac magnetic resonance imaging. This study demonstrated compression of the inferior vena cava. She underwent uneventful pectus repair with use of a modified Ravitch procedure and experienced complete resolution of her lower extremity edema.

Who should be referred? An evaluation of referral indications for fetal echocardiography in the detection of structural congenital heart disease.

Although fetal echocardiography is a useful tool for earlier detection of congenital heart disease (CHD), no clear, evidence-based appropriate use criteria exist. This study aimed to determine those referral indications most predictive of CHD. A retrospective cohort study of fetal echocardiograms was conducted at a single tertiary care center. Binomial proportion tests were used to compare likelihoods of structural CHD detection based on primary indication with the general population prevalence of 0.8%. Fetal echocardiograms of 2,380 pregnancies were performed, with CHD detected in 29.3% of these pregnancies. Patients referred for suspected CHD on screening ultrasound had the highest prevalence of CHD. Of the women without a history of cardiac anomalies shown on screening ultrasound, those with extracardiac anomalies or known chromosomal abnormalities had the highest prevalence of CHD. Screening ultrasound currently is the most useful method for determining who should be referred for fetal echocardiography, with a high prevalence of CHD found. For women with normal cardiac screening ultrasound results, fetal factors such as extracardiac anomalies and chromosomal abnormalities are more predictive of CHD than maternal or familial factors.

Relation of prenatal diagnosis with one-year survival rate for infants with congenital heart disease.

Prenatal diagnosis of congenital heart defects (CHDs) is increasingly common, but it is still unclear whether it translates to improved postoperative outcomes. We performed a retrospective cohort study of all infants (aged <1 year) who underwent surgery for CHDs from 2006 to 2011 at a single institution. Primary outcomes were in-hospital and 1-year mortality rates. Secondary outcomes were readmission within 30 days of discharge, postoperative length of intensive care unit and hospital stay, unplanned reoperation, and extracorporeal membrane oxygenation use. We used chi-square analyses, Wilcoxon rank-sum tests, Kaplan-Meier survival curves, and adjusted Cox proportional hazards models to compare outcomes. Of the 1,642 patients with CHDs, 539 (33%) were diagnosed prenatally. Patients with prenatal diagnoses were of a younger age and less weight at the time of surgery, had greater Risk Adjustment for Congenital Heart Surgery scores, and were more likely to be white, to have an identified syndrome, or to be born at term. Compared with those diagnosed postnatally, those diagnosed prenatally had a significantly higher unadjusted 1-year mortality rate (11% vs 5.5%, respectively, p = 0.03). Controlling for weight, surgical severity, race, age at surgery, prematurity, and the presence or absence of genetic syndrome, patients with prenatal diagnoses had significantly greater mortality at 1 year (adjusted hazard ratio 1.5, p = 0.03), as well as significantly longer intensive care unit and hospital stays. Infants with CHDs diagnosed prenatally had worse outcomes compared with those diagnosed postnatally. Prenatal diagnosis likely captures patients with more severe phenotypes within given surgical risk categories and even within diagnoses and thus may be an important prognostic factor when counseling families.

Pulmonary hypertension in Kawasaki disease.

This report describes the case of two pediatric patients who demonstrated echocardiographic evidence of pulmonary hypertension (PH) during the acute phase of Kawasaki disease. The etiology of PH development in this setting is currently unknown, but the authors hypothesize that pulmonary vasculitis may play a significant role. Fortunately, the PH appeared to be self-limited and resolved in both cases with routine treatment of Kawasaki disease.

Maternal antibody-associated fetal second-degree heart block and atrial flutter: case report and review.

Exposure to maternal anti-Ro (SS-A) and anti-La (SS-B) antibodies is a well-described risk factor for the development of fetal atrioventricular (AV) block. The role of maternal fluorinated steroids in the treatment and prevention of antibody-mediated fetal AV block is controversial. Fetal atrial flutter has rarely been described in association with maternal antibodies. This report describes a case of fetal exposure to maternal anti-Ro antibodies with associated second-degree AV block and atrial flutter. Interestingly, the reported patient had 2:1 AV conduction during both normal atrial rates (consistent with AV node conduction disease) and episodes of flutter (consistent with physiologic AV node functionality). The fetus was treated with transplacental digoxin and dexamethasone, which resolved both rhythm disturbances. The case report is followed by a brief discussion of AV block and atrial flutter associated with maternal antibody exposure.

Primary congenital infantile fibrosarcoma of the heart: the first confirmed case.

We believe this case represents the first confirmed congenital infantile fibrosarcoma (CIFS) of the heart. We describe an infant with congenital infantile fibrosarcoma of the heart who was treated with chemotherapy and who had a life-threatening complication develop that was successfully corrected by surgery. This demonstrates an unusual presentation, remarkable imaging, and diagnostic and treatment dilemmas. More significantly, this emphasizes the importance of a multidisciplinary approach for successful treatment of congenital infantile fibrosarcoma.