Virtually Delivered Psychosocial Intervention for Prenatally Diagnosed Congenital Heart Disease: Feasibility and Acceptability of HEARTPrep.

Prenatal diagnosis of congenital heart disease (CHD) often leads to anxiety, depression, and traumatic stress in expectant mothers, with long-term implications for the child and family. However, psychosocial intervention is rarely incorporated into prenatal care. HEARTPrep is a virtually delivered psychosocial intervention aimed at reducing distress and social isolation and increasing parenting self-efficacy and hope for mothers expecting a baby with CHD to promote long-term child/family well-being. This study evaluated the feasibility and acceptability of HEARTPrep. Participants were mothers receiving cardiology care for a fetal CHD diagnosis. Partners could participate with the mother. HEARTPrep was delivered through a mobile app and telehealth. Feasibility was assessed through enrollment/retention rates. Acceptability was assessed through 20 Likert-scale and five open-ended questions. Of 39 recruited mothers, 35 (90%) enrolled. Half of partners (48%) also participated. Twenty-seven of 35 enrolled mothers (77%) completed HEARTPrep. On a scale from 0 (Not at All) to 4 (Very), mean item acceptability scores ranged from 3.5 to 3.9. Mothers reported HEARTPrep helped them feel less distressed (mean: 3.74), less alone (3.84), more prepared (3.89), and more hopeful (3.84). Opportunities to process emotions, develop coping skills, learn with their partner, navigate relationships, understand they are not alone, connect with peer support, access resources, and prepare for stressors were described as helpful. HEARTPrep is feasible and acceptable for mothers expecting a baby with CHD. Future research will evaluate its efficacy in preventing/reducing maternal mental health problems and improving postnatal clinical outcomes.

The impact of body weight on the diagnosis of aortic dilation-misdiagnosis in overweight and underweight groups.

BACKGROUND: Body surface area (BSA)-indexed Z-scores are used to assess the ascending aorta (AAo) and diagnose aortic dilation (AoD) in children. BSA is directly related to body weight and corresponds to body mass index (BMI). We hypothesized extremes in BMI alter interpretation of aortic size in pediatric patients with AoD. METHODS: We reviewed all echocardiograms with a diagnosis of AoD performed at our institution from January 2013 through June 2013. Those with an age <2 or >20 years, history of aortic root surgery, or inadequate images were excluded. The aorta was measured by standard methods at the sinus of Valsalva, sinotubular junction, and proximal AAo. Using subject age, height, and gender, hypothetical weights for each subject were calculated to provide BMIs corresponding to the 5th, 50th, 85th, and 95th percentiles. The derived weights were then used to determine hypothetical BSA, and Z-scores were calculated for the subject's aortic diameters in each BMI group. RESULTS: A total of 153 patients met inclusion criteria. Mean age was 11.1±4.6 years (68% male). Mean height was 142.7±27.9 cm, mean weight 44.6±24.8 kg, and mean true BMI was the 62nd centile. Significant differences in all aortic dimension Z-scores were found among normal and underweight, overweight, and obese BMI groups (P<.001 for all comparisons), respectively. CONCLUSION: Using current recommended methods, AoD will be missed in overweight and obese patients and overdiagnosed in underweight patients. For children of normal weight, a Z-score based on BSA may be reliable. As obesity rates increase, weight-independent Z-scores must be developed.

The importance of small for gestational age in the risk assessment of infants with critical congenital heart disease.

BACKGROUND: Infants with critical congenital heart disease who require cardiothoracic surgical intervention may have significant post-operative mortality and morbidity. Infants who are small for gestational age <10th percentile with foetal growth restriction may have end-organ dysfunction that may predispose them to increased morbidity or mortality. METHODS: A single-institution retrospective review was performed in 230 infants with congenital heart disease who had cardiothoracic surgical intervention <60 days of age. Pre-, peri-, and post-operative morbidity and mortality markers were collected along with demographics and anthropometric measurements. RESULTS: There were 230 infants, 57 (23.3%) small for gestational age and 173 (70.6%) appropriate for gestational age. No significant difference was noted in pre-operative markers - gestational age, age at surgery, corrected gestational age, Society for Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality score; or post-operative factors - length of stay, ventilation days, arrhythmias, need for extracorporeal membrane oxygenation, vocal cord dysfunction, hearing loss; or end-organ dysfunction - gastro-intestinal, renal, central nervous system, or genetic. Small for gestational age infants were more likely to have failed vision tests (p = 0.006). Small for gestational age infants were more likely to have increased 30-day (p = 0.005) and discharge mortality (p = 0.035). Small for gestational age infants with normal birth weight (>2500 g) were also at increased risk of 30-day mortality compared with appropriate for gestational age infants (p = 0.045). CONCLUSIONS: Small for gestational age infants with congenital heart disease who undergo cardiothoracic surgery <60 days of age have increased risk of mortality and failed vision screening. Assessment of foetal growth restriction as part of routine pre-operative screening may be beneficial.

Long-term management of adults with conotruncal lesions: the diagnostic approach at All Children's Hospital.

Improved survival in children with complex congenital cardiac disease, such as conotruncal abnormalities, has created a sub-population of children and young adults who need comprehensive multi-disciplinary long-term follow-up. Routine surveillance with comprehensive screening for structural heart disease, functional heart disease, thromboembolic disease, arrhythmias, and associated end-organ dysfunction is important. Future research will better define the care plans for routine surveillance in patients with conotruncal abnormalities.

Partnering With Stakeholders to Inform the Co-Design of a Psychosocial Intervention for Prenatally Diagnosed Congenital Heart Disease.

Input from diverse stakeholders is critical to the process of designing healthcare interventions. This study applied a novel mixed-methods, stakeholder-engaged approach to co-design a psychosocial intervention for mothers expecting a baby with congenital heart disease (CHD) and their partners to promote family wellbeing. The research team included parents and clinicians from 8 health systems. Participants were 41 diverse parents of children with prenatally diagnosed CHD across the 8 health systems. Qualitative data were collected through online crowdsourcing and quantitative data were collected through electronic surveys to inform intervention co-design. Phases of intervention co-design were: (I) Engage stakeholders in selection of intervention goals/outcomes; (II) Engage stakeholders in selection of intervention elements; (III) Obtain stakeholder input to increase intervention uptake/utility; (IV) Obtain stakeholder input on aspects of intervention design; and (V) Obtain stakeholder input on selection of outcome measures. Parent participants anticipated the resulting intervention, HEARTPrep, would be acceptable, useful, and feasible for parents expecting a baby with CHD. This model of intervention co-design could be used for the development of healthcare interventions across chronic diseases.

Long-term management of patients with hypoplastic left heart syndrome: the diagnostic approach at All Children's Hospital.

Improved survival in children with hypoplastic left heart syndrome has created a sub-population of children and young adults who are living with functionally univentricular physiology. Routine surveillance with comprehensive screening for structural cardiac disease, functional cardiac disease, arrhythmias, thromboembolic disease, and associated dysfunction of end organs is important. Future directives will better define the plans of care for routine surveillance in patients with hypoplastic left heart syndrome.

A 22-Month-Old Girl With 3 Weeks of Dyspnea.

CASE PRESENTATION: A previously healthy 22-month-old girl presented to the ED with a 3-week history of dyspnea on exertion. A chest radiograph showed a right upper-lobe opacity suspicious for pneumonia (Fig 1A). The patient was prescribed amoxicillin but returned to the ED 7 days later with cough and persistent dyspnea and tachypnea. At that time, a repeat chest radiograph was concerning for worsening pneumonia (Fig 1B). Treatment with azithromycin and albuterol was initiated, and amoxicillin was discontinued. Her symptoms briefly improved; however, she returned to the ED 10 days later because of worsening cough and tachypnea, and a 2-day history of increased irritability, decreased oral intake, decreased urine output, and intermittent perioral cyanosis. She was afebrile throughout this period per parent report and vital sign documentation at each ED visit.

Frequency of Development of Aortic Valve Disease in Unrepaired Perimembranous Ventricular Septal Defects.

We sought to determine the natural history of aortic valve disease in patients with unrepaired perimembranous ventricular septal defects (pVSDs) and to identify echocardiographic parameters predictive of increased risk of surgical repair of pVSD because of aortic valve disease. We retrospectively analyzed all echocardiograms of patients with a diagnosis of pVSD at our institution from January 1999 to January 2015. All available echocardiographic data were collected. Patients were excluded if there was another structural cardiac anomaly other than bicuspid aortic valve, small patent foramen ovale, or ductus arteriosus. The prevalences of aortic valve prolapse and regurgitation, as well as aortic valve disease progression, were determined. A total of 2,114 echocardiograms from 657 patients with unrepaired pVSD were reviewed. Median age at the time of echocardiogram was 1.9 years (interquartile range [IQR] 0.2 to 5.4). Median duration of follow-up was 1.7 years (IQR 0.2 to 7.4). pVSD-associated aortic valve disease prompted surgical intervention in 1.5% (10 of 657) of patients. Median age at the time of surgery was 4.8 years (IQR 1.7 to 8.4). A pVSD-to-aortic annulus diameter ratio of 0.66 ± 0.05 was present in 90% (9 of 10) of patients who underwent surgical closure because of pVSD-associated aortic valve disease. In conclusion, pVSD-associated aortic valve disease is uncommon, and progression of aortic regurgitation is rare. These data suggest that the majority of patients with pVSD do not require frequent follow-up and that frequent follow-up can be saved for a subset with echocardiographic markers placing them at higher risk of aortic valve diseases.