In Pediatric Heart Disease: Understanding Post-Fontan Risks, Improving Parental Bonding, and Video Telemedicine

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Photo of crowd during conference A panel of experts in pediatric heart disease shared the latest findings from their research at a large national conference hosted by the Cardiac Center at Children’s Hospital of Philadelphia (CHOP) in partnership with Children’s Hospital Los Angeles. Cardiology 2019, the 22nd Annual Update on Pediatric and Congenital Cardiovascular Disease, occurred Feb. 13 to Feb. 17 in Huntington Beach. Organizers of the conference highlighted five finalists for best scientific abstracts. Below is a series of brief recaps of these important studies.

Small, Unrepaired Atrial Septal Defects Lead to Decreased Lifespan, More Chronic Diseases and Higher Levels of Anxiety and Stress

Using the Danish National Patient Registry, researchers identified 723 patients with small, unrepaired atrial septal defects (ASD) dating between 1953 and 2011. Of those patients, 182 had died. The average lifespan of patients with ASD was 63 years compared with the average Danish lifespan of 81 years. The study also found that 38.2 percent of ASD patients had some form of chronic illness, compared with 26.9 percent in the general population. Chronic lung disease was four times more common in ASD patients. More than 25 percent of ASD patients often felt nervous or stressed, compared with 16.9 percent in the general population. In light of these findings, the researchers suggest that current guidelines for intervention and follow-up may need to be revised in order to improve the lives of these patients.

Sebastian Udholm MD, et al, Aarhus University Hospital; Lifelong Burden of Small Unrepaired Atrial Septal Defect: Results from the Danish National Patient Registry (abstract 21)

Simple Changes for Newborns with Congenital Heart Disease Can Help Increase Parental Bonding Time and Reduce Unnecessary Interventions

Maternal-infant bonding time immediately after birth can provide benefits for both the newborn and mother. However, for infants born with congenital heart disease, delivery teams may be unfamiliar with the expected course, necessary (or unnecessary) treatments in the immediate period after birth, and whether bonding time is appropriate. In this study, researchers looked at 386 patients prenatally diagnosed with what is known as “ductal dependent” congenital heart disease from 2005 to 2017. In this severe type of heart disease, surgical or other types of intervention are required in the newborn period, but infants are typically stable for a period of time immediately after birth. The researchers created a tool that allowed cardiologists caring for the mother prenatally to better communicate with the delivery team about the infant’s diagnosis, expected course immediately after birth, acceptable oxygen levels, and whether a brief period of bonding was encouraged prior to initiation of necessary treatments. The tool was implemented in 2014. Researchers found that there was a decrease in infants who received oxygen or other perhaps unnecessary respiratory treatments from 38 percent to 26 percent, while those who were able to participate in bonding time increased from 22 percent to 74 percent. There were no documented adverse events. The researchers concluded that this easily implemented tool decreased unnecessary respiratory intervention and increased maternal-infant bonding.

Ian Thomas, et al, University of Michigan; Impact of Documentation on Respiratory Intervention and Parental Bonding among Newborns with Congenital Heart Disease (abstract 69)

Video Telemedicine Could Help Monitor Which Single Ventricle Infants Need Hospital Readmission*

Researchers at Children’s Mercy Kansas City developed the Cardiac High Acuity Monitoring program (CHAMP) which utilizes daily videos to monitor interstage single ventricle infants (SVI) at home. To assess if this program could be expanded, the clinical team tested an objective video clinical scoring system to identify SVI at risk for clinical deterioration and predict unplanned hospital readmissions (UHA). The study showed that video scoring was feasible for 91.6 percent of all observations. Interstage SVI demonstrated significantly higher scores shortly prior to admissions, and this new scoring system provided 89 percent sensitivity and 97 percent specificity predicting UHA. Dr. Aly and her team recommend conducting multicenter, prospective validation of this tool.

*Dr. Aly was awarded the Outstanding Investigator Award.

Doaa Aly, et al, Children’s Mercy Kansas City; Ability of Video Telemedicine to Predict Unplanned Hospital Readmissions for Single Ventricle Infants (abstract 91)

Systemic to Pulmonary Arterial Collateral Embolization Improves Outcomes for Acute Post-Fontan Patients

Systemic to pulmonary arterial collaterals are common after superior cavo-pulmonary anastomosis in single ventricle patients, and a higher burden of collateral flow is associated with worse acute post-Fontan clinical outcomes. The researchers wanted to analyze whether collateral embolization performed prior to Fontan completion would improve outcomes in these patients. A group of 32 embolized patients was compared with a historical control group of non-embolized patients with similar pre-Fontan collateral burdens. Embolized patients had a shorter post-Fontan hospital stay and lower risk of chest tube duration over 10 days. The researchers conclude these findings support embolization of collaterals prior to Fontan in patients with a high burden as it may improve clinical outcomes.

Emmanuelle Favilla, et al, Children’s Hospital of Philadelphia; Acute Post-Fontan Clinical Outcomes in Single Ventricle Patients Following Systemic to Pulmonary Arterial Collateral Embolization (abstract 148)

Clinical Factors Identified in Early Adolescent Fontan Patients That Help Predict Better Exercise Capacity

Peak minute oxygen consumption (PVO2) is frequently used to characterize exercise capacity in patients with a Fontan circulation. The magnitude of PVO2 decline may predict increased morbidity and hospitalization. Researchers looked at “top performers” in the 95th percentile of PVO2 among these patients to see what clinical factors might predict better long-term outcomes. The study found that in early adolescence in patients with a Fontan circulation, top performers in this cohort tend to be younger, female, and have lab values reflecting healthier hepatic, endocrine, renal, and hematologic organ systems. The researchers recommend longitudinal follow-up to see how exercise capacity and multi-organ performance fare as patients pass through adolescence. They also recommend investigating whether aggressive exercise rehabilitation in pre-adolescence can sustain wellness in these high-performing individuals or improve cardiovascular health.

Scott Weinreb, et al, Children’s Hospital of Philadelphia; Clinical Characteristics Associated with Exercise Performance in the Fontan Circulation: What Defines Our “Super-Star” Top Performers?  (abstract 203)

Contact: Natalie Solimeo, The Children’s Hospital of Philadelphia, 267-426-6246 or

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