Parental Impressions and Perspectives of Efficacy in Prenatal Counseling for Single Ventricle Congenital Heart Disease

Prenatal counseling at the Fetal Heart Program at Children’s Hospital of Philadelphia (CHOP) is offered by a dedicated group of providers including a core team of experienced fetal cardiologists, fetal heart nurse coordinators, and fetal heart social worker. Although some individualized variability may occur, in general, counseling is provided in the following manner. Fetal echocardiographic imaging is performed in a dedicated fetal heart imaging suite, located in a separate facility from the pediatric echocardiography laboratory. In parallel, obstetrical anatomical scan imaging is performed and evaluation offered by a dedicated group of maternal–fetal medicine specialist at CHOP. Following imaging, families are brought into a private consultation room, where findings and counseling by a cardiologist, nurse, and social worker are offered as a team, with cardiologist taking the lead in conversation. The encounter includes face-to-face review of patient history, explanation of normal heart structure and function, and conveyance of how current findings of the cardiac anatomy on fetal echocardiography differ from normal. Visual tools such as diagrams of normal and abnormal cardiac structure, either pre-formatted or drawn uniquely for the condition present, are used to aid in explanation. Counseling includes discussion of impact on delivery plans, postnatal treatment course, surgical options as well as short- and long-term outcomes. Further education, family assessment, and ongoing concerns are addressed by the fetal heart nurse and social worker. Following initial encounter, additional visits occur serially every 4 weeks until delivery, with updated fetal echocardiographic imaging and repeat counseling sessions to supplement and review findings and answer questions. Fetal heart nurse coordinators are in frequent touch with families in response to queries that arise between visits, through phone calls or email communications. Towards the end of gestation, families have an opportunity for a single encounter meeting with a heart surgeon, accompanied by the fetal heart nurse. A tour of the cardiac intensive care unit is offered.

An online survey created in REDcap (research electronic data capture) was distributed to family email addresses recorded in the electronic health record (Epic). The survey consisted of basic demographic information, Likert scales (grade 1–5, with 1 as lowest and 5 as highest score) assessing parental grade with respect to various aspects of prenatal counseling, and open-ended questions to elicit further free text response (Table 1, survey). Survey questions solicited grades from parents concerning various aspects of prenatal counseling and were developed specifically for this study, based on feedback during discussion with families. The first question related to grading the recollection of prenatal counseling. This was followed by requests for assessments of the following: (1) overall impression of quantity of counseling, (2) explanation of the heart defect, (3) preparation for heart surgery, (4) preparation for the hospital course and care, (5) preparation for complications and outcomes of a Fontan circulation, and (6) preparation for neurological, school-related, or behavioral problems. Survey questions then solicited impressions concerning how helpful it was to meet with specific FHP members including (1) fetal cardiologist, (2) fetal heart nurse, (3) fetal heart social worker, and (4) cardiac surgeon.

Only one parent was required to respond and discussion between parents was allowed. The questionnaire was distributed via email to potential candidates 3 times over the course of 2 months to increase response rates. Responding families were assigned a study ID for deidentification purposes. Once a response cohort was identified, chart review was performed to examine patient medical records in a manner blinded to specific survey responses. Data collection included condition variables as well as factors that influence magnitude of burden of care and potentially impact perceptions of prenatal counseling: cardiac diagnosis, length of hospital stays for all stages of surgical palliation, type of Fontan, utilization of ECMO, number of cardiac catheterizations, additional surgeries beyond anticipated, other non-cardiac diagnoses, use of nasogastric feeding tube, and current medication regimen.

Data analysis consisted of creating scores for each Likert scale variable by averaging the numerical ratings (1–5) for the survey response cohort. Paired t-tests were used to explore for significant differences between the survey variables of various aspects of prenatal counseling, and between ratings for FHP counseling team members. A composite impression of overall quality of counseling—a “comprehensive fetal counseling score (FCS)”—was calculated for each participant by averaging their individual Likert scale ratings across all 10 survey questions including those concerning prenatal counseling content (6 questions) and the provider assessments (4 questions).

To explore the influence of postnatal course and disease severity on current impressions of prenatal counseling, the magnitude of burden of disease was assessed and compared across the survey responses. Chart review data analysis consisted of determining 5 indicators of overall burden of disease. These included (1) parental report of their estimate of number of days of initial newborn hospitalization after birth for first stage of care, (2) discharge to home after initial hospitalization with or without a feeding tube, (3) current cardiac medication burden as a reflection of magnitude of care, dichotomized into 2 groups: (a) those who only received aspirin alone or aspirin and enalapril (standard medications) and (b) those who received additional medications, (4) presence of other comorbid conditions or complications, and (5) unexpected interventions such as unanticipated surgeries or cardiac catheterizations. Parental report of days of initial newborn hospitalization was compared with actual medical record recording.

In order to test for possible associations between burden of disease severity and perception of fetal counseling, non-parametric independent t-tests (Independent-Samples, Two-Tailed Mann–Whitney U Test) were used to determine if there were significant differences in composite FCS values between (1) those with and without unexpected cardiac surgeries, (2) those with and without unexpected catheterizations, (3) those with and without comorbid conditions, (4) those who were and were not discharged home with a feeding tube, (5) those who had a standard (aspirin with or without enalapril) or increased (more than aspirin and enalapril) medication regimen, and (6) those who received or did not receive extracorporeal membrane oxygenator (ECMO) support at any point during care. Pearson correlation test was used to determine the association between hospitalization length and comprehensive fetal counseling score. A p value of < 0.05 was considered significant.

In total, 248 families with prenatal diagnosis of single ventricle congenital heart disease meeting study criteria were identified. Of these, 65 responded to the questionnaire (26% response rate) with 6 responding to only a very limited number of survey questions, hence the cohort for complete analysis consisted of 59 subjects. Respondents included 62 mothers (95%) and 3 fathers (5%). Of the overall respondents, 62 (95%) delivered at the CHOP Special Delivery Unit and 3 (5%) delivered elsewhere. Deliveries consisted of 40 (62%) vaginal births and 25 (39%) cesarian section deliveries. Of the 59 respondents with completed surveys, 11 (19%) had a fetus with dominant single left ventricle anatomy and 48 (81%) had a dominant single right ventricle anatomy. With regard to initial palliation, 22 (37%) received the Norwood procedure with a Blalock–Taussig shunt, 29 (49%) received the Norwood procedure with a Sano shunt, 5 (9%) underwent pulmonary arterial banding, 1 (2%) received a ductus arteriosus stent, and 2 (3%) received no initial intervention. Prenatal counseling consisted of 3–5 in-person encounters, with fetal heart nurse coordinator follow-up via email and phone calls throughout the prenatal period. At the time of survey administration, 38 patients (64%) completed the final stage of Fontan palliation; 29 (76%) with extra-cardiac fenestrated conduit; and 9 (24%) a lateral tunnel with fenestration. Average age of the children at the time of parental survey was 4.6 ± 3.3 years (range 1–10 years).

Table 2 shows the mean ± SD values for the survey variables and Fig. 1a–k shows the distribution of survey scores for domains of counseling, FHP provider satisfaction scores, and composite comprehensive fetal counseling score. Memory recollection of fetal counseling scores were generally quite high with no association to the comprehensive fetal counseling score. With regard to preparation for various aspects of care, average scores were highest for explanation of heart condition and lowest for preparation for neurological, school, and behavioral issues. The average level of preparation for complications and outcomes of the Fontan circulation was significantly lower than preparation for surgery (p < 0.01) and hospital course (p < 0.05). The average level of preparation for neurological, school, and behavioral issues was significantly lower than surgery (p < 0.001) and hospital course (p < 0.001).

With regard to satisfaction with various members of the FHP provider team, cardiac surgeon had the highest average score although 12 (20%) families did not meet this team member before birth. Social worker had the lowest average score. When comparing team member scores among the 45 families who met with all providers, the average social worker score was significantly lower than the cardiologist (p < 0.05) and surgeon (p < 0.05).

The average estimated days of hospitalization after birth by parental report is 36 ± 31 days with a range of 5–150 (median 28) days, with one outlier of 341 days. Actual newborn hospitalization was 32 ± 26 days with a range of 5–146 (median 24) days excluding one outlier of 302 days. Parental recollection of initial newborn hospitalization length of stay matched very well to the actual measure of days of hospitalization from the medical record (Fig. 2, Pearson r = 0.953, p < 0.001). A statistically significant negative correlation was found between the composite FCS and parental recollection of length of hospitalization (Fig. 3, Pearson r = − 0.357 p < 0.01).

Table 3 lists the distribution of elements of disease burden assessed in the survey study cohort. The composite FCS for the entire study cohort was a mean of 4.1 ± 0.7. No statistically significant differences were found in the composite FCS between those with or without a feeding tube, those with differing medication regimens, those with additional comorbidities or complications, and those who required unexpected surgeries or catheterizations. However, the composite FCS value was significantly lower for those who had ECMO care versus those who did not (avg 3.3 ± 1.1 vs 4.2 ± 0.5, p = 0.047).