Computational fluid dynamics modeling aiding surgical planning in a toddler with Parkes Weber syndrome.

Parkes Weber syndrome is a fast-flow and slow-flow vascular anomaly with limb overgrowth that can lead to congestive heart failure and limb ischemia. Current management strategies have focused on symptom management with focal embolization. A pediatric case with early onset heart failure is reported. We discuss the use of computational fluid dynamics (CFD) modeling to guide a surgical management strategy in a toddler with an MAP2K1 mutation.

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 Children's Hospital regional cardiology sites (SCH-RC) from December 2008 to December 2015. Referrals to Seattle Children's 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.

Anomalous aortic origin of coronary arteries from the opposite sinus: a critical appraisal of risk.

BACKGROUND: Anomalous aortic origin of the coronary artery (AAOCA) from the opposite sinus of Valsalva with an interarterial course has received much attention due to its association with sudden death in otherwise healthy individuals. AAOCA is relatively common and may have significant public health implications. While our knowledge of its pathophysiology and natural history remains incomplete, an emphasis has been placed on surgical correction. DISCUSSION: In 2005 we published a review examining the rates of sudden death with AAOCA, as well as complications of surgical management. Evidence now points even more strongly to lower rates of sudden death, while surgical outcomes data now better documents associated risks. SUMMARY: Armed with this updated information, we agree with the need for a national registry to better track patients with AAOCA. We submit that the risks of surgical management outweigh any benefits in the asymptomatic patient with anomalous right coronary artery, and expectant management should also be strongly considered even in asymptomatic patients with anomalous left coronary artery.