Bhawna Arya

Utility of novel fetal echocardiographic morphometric measures of the aortic arch in the diagnosis of neonatal coarctation of the aorta.

Prenatal diagnosis of neonatal coarctation of the aorta (CoA) is challenging; there is a high false–positive rate, yet 60–80% are not identified prenatally. We aimed to identify novel fetal echocardiographic measures to improve prenatal identification of CoA.

Response to comment on “Utility of novel fetal echocardiographic morphometric measures of the aortic arch in the diagnosis of neonatal coarctation of the aorta”

I thank Dr. Devore for bringing this error to the attention of the authors, editors of Prenatal Diagnosis, and the readership, especially those investigators who choose to utilize these promising measurements. We have reviewed our linear regression equation and have confirmed that the equation as currently published predicts the probability of not having coarctation of the aorta (CoA) instead of having CoA. The correct equation for computing the probability of having CoA is “(1/(1 + exp)−F).” This error was caught early in our manuscript preparation, but unfortunately, the figure with the erroneous equation was submitted in the final revisions submitted to the journal.

Prenatal physiologic findings and postnatal advanced imaging in the management of absent pulmonary valve syndrome with intact ventricular septum

Absent pulmonary valve syndrome is a congenital heart defect usually associated with tetralogy of Fallot. Rarely, absent pulmonary valve syndrome can occur independently of tetralogy of Fallot and presents with an intact ventricular septum and a patent ductus arteriosus. This case report describes the prenatal diagnosis of absent pulmonary valve syndrome with intact ventricular septum via echocardiogram and the postnatal management of the resulting physiologic effects secondary to this rare congenital heart disease.

The role of regional prenatal cardiac screening for congenital heart disease: A single center experience

BACKGROUND Accurate prenatal diagnosis of congenital heart disease (CHD) allows for appropriate delivery and postnatal management. Geographic constraints limit access to fetal cardiology subspecialists. In our approach, general pediatric cardiologists are first line in regional prenatal cardiac screening. We aim to demonstrate the utility of this approach in diagnosing CHD requiring cardiac interventions within 30 days of life. METHODS This is a retrospective review of fetal echocardiograms performed at Seattle Childrens Hospital regional cardiology sites (SCH-RC) from December 2008 to December 2015. Referrals to Seattle Childrens Hospital Prenatal Program (SCH-PNP) were evaluated for referral timing, indication, diagnostic accuracy, and postnatal care. Diagnostic accuracy was determined using the initial postnatal echocardiogram as the gold standard. Major discrepancy was defined as one resulting in change in surgical management. RESULTS Of 699 fetuses evaluated at regional sites throughout Washington and Alaska, a small subset (n = 48; 6.9%) required referral to SCH-PNP. Need for relocation was confirmed in 31 subjects, of which 27 required cardiac intervention within 30 days of life. Of those not referred to SCH-PNP (n = 643, 91.9%), none required neonatal cardiac intervention. There were 22 regional diagnostic discrepancies (31% major, 7% minor). Referral to SCH-PNP improved diagnostic accuracy (2% major, 0% minor). CONCLUSIONS Regional prenatal cardiac screening demonstrated 100% sensitivity and 98.9% specificity for identifying critical CHD. Utilizing regional pediatric cardiologists as first line in prenatal screening in geographically remote regions may improve access to care and outcomes in neonates with critical CHD while improving resource utilization.

Diagnostic Approach in Fetal Coarctation of the Aorta: A Cost-Utility Analysis

Background: Coarctation of the aorta (CoA) is difficult to diagnose by fetal echocardiogram (F‐Echo), often requiring multiple F‐Echos during gestation and neonatal echocardiograms (N‐Echos) after birth. Furthermore, CoA is the most common ductal‐dependent lesion missed on routine physical exam. Objectives: We sought to determine the most cost‐effective diagnostic approach in caring for infants in whom an initial F‐Echo is concerning for CoA. Methods: Four paradigms for management after initial F‐Echo could not rule out CoA were compared, with a single paradigm involving additional F‐Echos: (1) multiple F‐Echos for diagnostic clarity and performance of N‐Echo on neonates with remaining high suspicion for CoA on F‐Echos (prenatal‐multiple), (2) no further F‐Echo and performance of N‐Echo on neonates with high suspicion for CoA on initial F‐Echo (postnatal‐selective), (3) no further F‐Echo and performance of N‐Echo on all neonates (postnatal‐all), and (4) no further F‐Echo or N‐Echo with reliance on routine physical exam to identify afflicted infants (postnatal‐none). Decision analysis models were constructed. Probabilities dictating clinical course and costs were calculated using our institutions study population. The utility‐state values were derived from existing literature. The measure of effectiveness was quality‐adjusted life years. To represent societal perspectives, cost was defined as hospital reimbursement payments. Results: From 2007 to 2014 at our institution, 92 patients were diagnosed with CoA and met the inclusion criteria for this study. These patients presented to care either through prenatal diagnosis (n = 31), postnatal examination findings while clinically well (n = 41), or after clinical deterioration in extremis (n = 20), with one patient subsequently dying. Presenting in extremis was associated with a 20% increase in the cost of their subsequent care and with a 51% increase in length of hospital stay. Postnatal‐none was the least effective paradigm but also the least costly, thus forming the baseline model. Of the three other diagnostic approaches modeled, Postnatal‐all was the cost‐effective paradigm, maximizing utility due to avoidance of high‐cost/low‐utility disease states such as presentation in extremis and death. Prenatal‐multiple was the next most effective but was also the most expensive. Conclusions: Echocardiography is the screening gold standard in avoiding the devastating clinical manifestations of a missed CoA. When a diagnosis of CoA cannot be ruled out on initial F‐Echo, the most cost‐effective approach is performance of N‐Echo on all neonates with no further prenatal evaluation. HighlightsPrenatal echocardiographic concern for coarctation of the aorta is a frequent diagnostic challenge for pediatric cardiologists.Faced with diagnostic uncertainty, providers often repeat fetal echocardiograms throughout gestation, yet doing so often does not add diagnostic clarity.This study illustrates that the optimally cost‐effective strategy is to defer further fetal echocardiograms after an initially inconclusive study, instead obtaining a definitive postnatal echocardiogram on all infants with concern remaining.