Methods

Following approval from our institutional review board, our study had three phases, which took place between 1 October 2001 and 1 May 2002. The first phase consisted of setting up focus groups with parents of children who had undergone repair of congenital heart disease at an age less than 28 days between January 2001 and September 2001. The focus groups were held in October 2001. The purpose of the focus groups was to generate issues to be included in a semi-structured interview during the third phase of the study.¹⁰ The focus of the discussion was the decisions needed to be made by the parents for repair of the congenital cardiac malformation, as well as the potential impact of the timing of diagnosis. The discussions were audio-taped, transcribed, and then the transcripts were analyzed by two different raters to generate themes. The themes were generated out of phrases frequently used by the parents. The themes of the focus groups were analyzed and then used for construction of the semi-structured interview tool (see Table 1).

All English-speaking parents of neonates undergoing surgery at our institution between 1 November 2001 and 1 May 2002 were eligible for the second and third phases of the study. For the second phase, the quantitative portion of the investigation, three constructs were chosen to characterize the levels of stress suffered by the parents, specifically anxiety, optimism, and the perception of experiences of life. We quantified levels of anxiety using the Spielberger State/Trait Anxiety Inventory.¹¹ This measure taps both dispositional (trait) anxiety, and situational (state) anxiety. The life orientation test was used to quantify optimism. This tool assesses dispositional optimism irrespective of the stresses of life, and can be used to predict how people will respond to stress.¹² The life experiences survey was used to measure perceptions of the experiences of life as positive or negative.¹³ The instruments were used pre-operatively to detect possible differences in the groups before the additional stress of the peri-operative period. Each instrument was coded only with a number, and was scored only at completion of the study.

The third phase of the study consisted of post-operative semi-structured interviews with the parents. Verbal consent was obtained on tape at the beginning of all interviews. All of the interviews were done by the principal investigator (K.S.H.) and were tape recorded, transcribed, and then analyzed. The interviews and the instruments were both coded by number to ensure confidentiality, with the list of names maintained independently from the data. Each transcript was coded independently by two raters (including K.S.H.) trained in the coding categories, with codes assigned to the data using NVivo software.¹⁴ Transcripts were compared to ensure that codes were being applied consistently. There was 75% agreement between coders at the first comparison. Where there were differences in the codes applied to the same selections of text, the rationale for applying a code was discussed, and agreement was reached on the most appropriate code.

Table 1. Semi-structured interview tool.

Results

For the first phase, of a total of 45 eligible families, 5 families (10 adults) came to the first focus group; and 9 adults, representing 6 families, came to the second focus group, giving a total of 19 adults. The themes discussed in the focus groups were the perception of active choice for the baby to have surgery, and the effect of stress on the understanding of the complex congenital cardiac malformations afflicting the baby. The themes from the focus group were used to generate the semi-structured interview tool, and to code the interviews. The themes were choice, stress, understanding, and focus. Choice refers to whether or not the parents felt as though they made a genuine choice for their baby to undergo surgery, a component of voluntariness. Stress refers to the description by the parents of their emotional state. Understanding reflects the parental appreciation of the information that they received. Focus is the ability, as described by the parents, to process specific information despite overwhelming stress and anxiety.

For the second and third phases, of 41 eligible neonates, parents of 31 agreed to participate. The racial breakdown of the neonates included 21 Caucasians, 6 African-American, 3 Latinos, and 1 Asian neonate. The diagnoses of the 31 neonates are shown in Figure 1. For the pre-operative questionnaires, 52 of 62 possible parents chose to participate, 30 mothers and 22 fathers. A two-tailed independent group t-test was used, analyzing mothers and fathers separately, to test for differences in the pre-operative measures as a function of the timing of diagnosis.

At the time of surgery, there were no statistically significant differences between the mothers of neonates diagnosed prenatally as opposed to postnatally in terms of anxiety, optimism, or perception of the experiences of life (Fig. 2, Table 2). Fathers of neonates who were diagnosed prenatally, however, had less anxiety, were more optimistic, and reported fewer negative life events compared to fathers of those neonates diagnosed after birth (Fig. 3, Table 2).

In the third phase, we enrolled 44 parents of 31 babies, all parents being interviewed between the first and seventh post-operative day, with the mean number interviewed on the third post-operative day. The 14 mothers of prenatally diagnosed neonates were generally diagnosed between the 22nd and 24th weeks of gestation. Their perception was that, between diagnosis and delivery, they moved from a period of grief and mourning to a period of active planning. In the case of 13 mothers of postnatally diagnosed neonates, there was the added stress of being in the postpartum state. These mothers felt a variety of emotions, but their perception was that they were able to focus to make decisions, and to advocate for their babies.

The 10 fathers of neonates diagnosed prior to birth ran the spectrum from being overwhelmed with grief to being more detached until the baby was born. These fathers stated concern about the financial impact on their family; in contrast to the fathers of neonates diagnosed postnatally. In this cohort, the seven fathers of neonates diagnosed postnatally with critical malformations did not feel that non-intervention was an option; yet they wanted information prior to making their final decisions. These fathers had the additional stress of worrying about the mother and the baby being in different hospitals.

With regard to informed consent, parents of both groups of neonates felt as though they made a genuine informed choice for their baby to have surgery. They understood that the baby could not survive without surgery, and thus chose surgery to keep the baby alive. The parents of neonates with hypoplastic left heart syndrome were different from the other parents in this study because of the number of “options” available for their children. At other institutions, those who received a prenatal diagnosis were offered the choices of termination, staged reconstruction, cardiac transplantation, non-intervention subsequent to birth. It is of interest that non-intervention was not given as an option for diagnoses other than hypoplastic left heart syndrome. Those who received their prenatal counseling at this institution were offered either termination or staged reconstruction as the options presented. Despite the variety in the options presented to the parents of neonates with hypoplastic left heart syndrome, they had similar quality of informed consent to the other parents. All of the parents understood that their neonate would die without intervention, and, thus, chose staged reconstruction.

The parents of prenatally diagnosed neonates may have had a more sophisticated understanding of the process of the disease, but both groups of parents perceived that they understood the consequences of not having surgery. In the qualitative interviews undertaken within 1 week of surgery, none of the participating parents expressed regret about their decision to opt for surgery. All of the parents included in the study felt satisfied with the decision they made for the baby to have surgery, regardless of whether they made the decision 3 months in advance or shortly before surgery.

Figure 1. Diagnoses of the neonates.

Figure 2. Results of the mothers’ pre-operative psychological instruments.

Figure 3. Results of the fathers’ pre-operative psychological instruments.

Table 2. Means and standard deviations on quantitative measures by parental role and timing of diagnosis.

Discussion

Prenatal diagnosis of critical congenital cardiac disease has various physiological effects for the baby; and may improve the health and outcome of the neonate.¹⁵ Hypoplastic left heart syndrome is a subgroup within complex congenital heart disease that necessitates special consideration given the different choices, specifically palliative surgery, cardiac transplantation, and non-intervention, and the variability in outcome.^16–18 To the best of our knowledge, ours is the first study to evaluate the impact of prenatal diagnosis on parental decision-making.

In this small pilot study, the quantitative data revealed fathers of neonates in whom the diagnosis has been made prior to birth were less anxious, more optimistic, and perceived fewer negative life events than did those who learnt of the illness of their child after birth. One reason for this could be that the fathers of those diagnosed prenatally have more time to make a plan for action, both for their jobs, and for the care of their other children. The fathers may use this element of control as a coping skill. An alternate explanation is that fathers with less anxiety and more optimism may be more likely to pursue prenatal testing. No differences were seen when mothers of prenatally diagnosed neonates were compared to mothers of neonates diagnosed after birth. This could be because both groups of mothers were equally distressed at the time of surgery.

The qualitative data suggested that, irrespective of the timing of the diagnosis, both sets of parents felt as though they made a genuine decision for their baby to undergo surgical repair of the congenital cardiac malformation. The parents felt as though they understood, and then actively decided, for the baby to undergo surgery. Both groups felt satisfied with the decisions they made.

Our investigation is novel and unique in that it examined informed consent in close proximity, less than 1 week, from the decision to proceed with cardiothoracic surgery. The population with complex malformations was chosen specifically because of the urgency to make a decision for surgery prior to the death of the baby. This is in contrast to previous retrospective studies of informed consent, which suggested deficiencies in informed consent in parents of neonates with life-threatening conditions, such as the Euricon trial of extra-corporeal membrane oxygenation.⁶ In the Euricon trial, parents had similar decisions to make as in our study, since non-intervention was associated with a high likelihood of mortality. Our study suggests that parents of critically ill children may adequately weigh options and make decisions on the behalf of their child.

A strength of our study is that we interviewed parents shortly after the decision for surgery. Other studies investigating the quality of informed consent have all involved a much longer time lag between the parental decision and the research interview. The Euricon study, which examined randomized controlled trials involving neonates, included interviews done up to a year after the decision of the parents to enroll their neonate in the trial. In addition, the deficiencies identified in competence, information, understanding, and voluntariness were based on the subjective recall of both parents and physicians.⁶ Recall bias from newer knowledge about the allocation of treatment allocation may impact on studies of informed consent carried out retrospectively.¹⁹ Other studies dispute that stress has a negative impact on informed consent, suggesting rather that parents assessed the probability and magnitude of risks and benefits.^{20, 21} Fathers, in particular, have to worry about the postpartum mother in addition to the baby.²²

There are several limitations to our pilot study. The proximity of the interview to the date of surgery, seeking to avoid the impact of surgical outcome on parental recall, separates our study from others related to informed consent, but does leave it open to other parental bias. Parents may be biased by the condition of the baby, or by comments made by the surgeons, such as “surgery went well”. The difference in trait anxiety between the two groups of fathers is another limitation, because they are not the same at baseline. The wide variety of cardiac diagnoses is another limitation, because the emotional responses to the anticipated clinical courses may be quite different. This heterogeneity also limits the comparison between the groups of neonates diagnosed postnatally as opposed to prenatally. There is also an inherent selection bias, secondary to incomplete recruitment for all three phases of the study. Our selected population included only the parents of neonates diagnosed prenatally who chose to continue the pregnancy, and to have the surgery carried out at our institution. Other limitations of our study include lack of objective testing of parental understanding and institutional bias. Those who chose non-intervention did not present to our center. Non-intervention is generally not presented as an option for parents who receive counseling at our institution, unless there are multiple congenital anomalies, genetic syndromes, or severe hypoxic ischemic encephalopathy.

In summary, therefore, our study validates our hypothesis that prenatal diagnosis of a critical congenital cardiac malformation may enhance paternal adjustment, and that from the parental perspective, stress, and urgency do not impair decision-making about neonatal cardiac surgery.