Surgical repair for congenital cardiac disease has evolved tremendously over the last two decades. One of the major advances in pediatric cardiac surgery is the ability to perform a complete repair in infancy, or even in the neonatal period, for many forms of malformations that previously were treated only by palliative procedures. For instance, infants with tetralogy of Fallot used to be treated initially with an aortopulmonary shunt, then undergo complete repair in later childhood.1–4 Many centers now perform primary repair in infancy.5–7 Infants with ventricular septal defects can be palliated by banding the pulmonary trunk, then undergo a complete intracardiac repair months or years later. In many cases today, both one-stage and two-stage approaches are considered. The options for treatment, determined jointly by the surgeon and cardiologist, are presented to the parents. Little is known, however, regarding the preferences of parents and families for a one-stage complete repair as opposed to a two-stage approach, with palliation first followed by complete repair. We conducted this study to explore parental preferences for surgical strategies in children undergoing cardiac surgery.
Methods
The protocol was approved by an Institutional Review Board of the Harbor-UCLA Research and Education Institute. Participants were recruited from the clinic for pediatric cardiology at the Harbor-UCLA Medical Center. Eligible candidates were parents or primary caregivers of children who were referred to cardiology for evaluation of heart murmur or chest pain, and were determined by a cardiologist to have no cardiac abnormality.
Design of the study
The study was conducted using face-to-face interviews. The interviews took place in a private, quiet, room in the clinic. The participants were first told that the study explored the preferences of parents for surgical treatment of heart disease. We informed the participants that the questions asked in the interview were hypothetical. They were asked, nonetheless, to regard the scenarios as real as possible while considering answers to the questions. Graphs were used to assist understanding of the concept of mortality rates. Participants were encouraged to ask for more explanation as needed to ensure understanding of the questions, and were given sufficient time to contemplate the answer to each question.
Each participant was given a hypothetical scenario that his/her child was born with a hole in the heart, such as a ventricular septal defect, that requires an elective operation. The cardiac defect can be treated by two surgical strategies: a one-stage repair to close the hole by an open heart operation, or a two-stage approach with an operation, such as banding of the pulmonary trunk, for initial palliation, and then open heart surgery to close the hole one year after the initial palliation. The risk of mortality for the one-stage repair was set at 5%. The mortality rates of the two-stage surgery were set at four scenarios to determine how the varying rates for the combined two-stage approach and the first stage operation of the two-stage approach influence parental decision concerning a one versus two-stage surgical strategy. Two scenarios had the combined mortality rate for the two-staged approach set at 4%, lower than the rate for the one-stage approach, and the rates for the first stage were set at 1% and 3%, respectively. The other two scenarios had the rate for the two-staged approach the same as for the one-stage strategy, and those for the first stage were set at 1% and 3%, respectively. The participants were informed that the only difference among the scenarios was the mortality rate. For each scenario, the participants were asked to choose between the one and two-stage approaches.
Statistical analysis
Descriptive continuous data are presented as mean plus and minus standard deviations. Continuous variables were compared using Student's t test, and categorical variables were compared using the chi square test. Logistic regression models were used to determine the variables associated with the decision for one- versus two-stage strategy in each of the 4 scenarios. Dependent variables in the logistic regressions were the decision for one- versus two-stage strategy in different scenarios. Independent variables in the models were age of the participant, at older or younger than 30 years, gender, ethnicity as white versus non-white, none, public, or private insurance, education at elementary school or lower as opposed to higher than elementary school, primary language as English or non-English, owning a vehicle as opposed to travel by public transportation, and distance from home to our hospital of less than or greater than 15 miles. A p value less than 0.05 was considered statistically significant. Statistical analyses were performed using SPSS 10.1 for Windows (SPSS Inc., Chicago, IL).
Results
We interviewed 103 parents or adult guardians of children referred to our clinic. Of the interviews, 74 (72%) were conducted in Spanish, and 29 (28%) were in English. The predominance of Spanish interviews is due to a large Hispanic population seen at the Harbor-UCLA Medical Center. The mean age of the participants was 33 ± 7.7 years old, with a median of 33 years, and a range from 18 to 48 years. Among the participants, 82 (80%) were female and 21 (20%) were male. Of their relation to the child seen in the clinic, 78 (76%) were mother, 21 (20%) were father, 2 were aunt, and 2 were older sister. In regard to insurance, 47 (46%) participants had Medi-Cal (California Medicaid program), 8 had managed care health plans, 9 had other coverage, and 39 (38%) had no insurance coverage.
When the two-stage combined mortality rate was lower than that set for the one-stage repair, participants proved more likely to choose the two-stage approach if the mortality rate for the first stage was 1% as compared to 3% (57% and 44%, respectively, p = 0.04). When the combined mortality rate for the two-staged approach was the same as that for one-stage at 5%, 42% of participants chose the two-stage approach when the mortality rate for the first stage was 1%, and 34% of participants chose the two-stage approach when this rate for the first stage was 3% (p = 0.24). These results are illustrated in Figure 1.
Figure 1.
The graph shows the percentage of patients choosing the two-staged surgical approach. The X-axis denotes different levels of the combined mortality rate. The first stage surgical mortality rate for white columns was 1%, and for shaded columns was 3%. The mortality rate for single staged surgery was set at 5%. (MR: mortality rate; *p < 0.05.)
On analyzing the characteristics of participants in their choice of the one-stage versus two-stage approach for surgical repair using multiple logistic regression, we found that age, gender, race, primary language, education, transportation and distance to hospital were not associated with the choice of surgical approach. When the combined two-stage mortality was the same as one-stage repair, the odds ratio for choosing two-stage among participants with no insurance was 0.71 when the first stage mortality was 1% (p = 0.03), and was 0.66 when the first stage mortality was 3% (p = 0.02) comparing to participants with insurance as the reference group, with an odds ratio of 1.
Discussion
Advances in early diagnosis and surgical treatment in the past two decades have changed the practice of surgical management of infants with congenital cardiac disease. Open heart surgery in the neonatal period, such as the arterial switch operation for complete transposition, or the Norwood operation for hypoplastic left heart syndrome, has been adopted by many pediatric cardiac centers since the mid-1980s. Successes in these neonatal procedures have also inspired some surgeons to perform corrective surgery at an early age for some cardiac defects that used to be treated initially by palliative surgery. For example, infants with large ventricular septal defects may undergo closure of the defect at an early age, instead of being palliated by banding of the pulmonary trunk, followed by later closure of the defect. Cyanotic infants with tetralogy of Fallot may undergo intracardiac repair in the neonatal period or early infancy instead of being palliated by an aortopulmonary shunt first, then intracardiac repair later. For patients with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries, there are advocates for one-stage complete repair, and advocates for stepwise palliation towards complete repair.8, 9
The decision for corrective versus palliative surgery for neonates and infants with congenital cardiac disease frequently depends on institutional experience and the preference of the surgeons and cardiologists. Centers with more experience in neonatal surgery are more likely to perform early corrective surgery rather than palliative procedures. Although both one-stage and two-stage approaches may be presented to the parents, the decision of the parents is very likely to be influenced by how the case is presented, and the recommendations of the surgeon and the cardiologist. To the best of our knowledge, parental preference for a one-staged versus a two-staged repair has not previously been investigated in systematic fashion. Since it may be very difficult to study parental preference prospectively in real patients, we used standardized case scenarios to explore the parental decision-making process.
It is understandable that, when the two-stage combined mortality rate is the same as the one-stage repair, most parents chose the one-stage repair regardless of the mortality rate for the first or second stage in the combined approach. The mortality rate for the first stage also appears to be important, however, when the combined mortality rate is lower than that of the one-staged repair. When the combined mortality is lower than that for one-staged repair and the rate is low for the first stage, it is more likely that parents will choose the two-staged approach. The explanation for such a finding is not entirely clear, albeit that the mortality rate for the first stage palliation is, indeed, usually lower than that for the subsequent complete repair in real life.
When the combined mortality rate was set at the same as that for one-staged repair, we found that availability of insurance was an important variable in determining the choice of a one- versus two-staged approach. People without insurance may consider the potential problems with financial coverage for the operations, and therefore, are more likely to choose one-stage repair. Insurance, nonetheless, was not a significant determinant of decision-making when the combined mortality rate was lower than that for one-staged repair. Based on these results, we speculate that, when deciding on a one- versus two-staged approach, parents will first compare the combined mortality rate with that of the one-staged repair. If there is no difference in mortality, other factors, such as insurance, may become important in the decision-making for the surgical approach.
We recognize the limitations of our investigation. Studies have shown that, in the field of medicine, the words used to describe the frequency and probability of events may vary widely, and be interpreted differently by different individuals.10 Verbal specification of frequency and probability using numbers, therefore, is better than using descriptive terms. In the present study, we used very specific numbers, such as 5% mortality rate, and used graphs to assist participants in their understanding of these numerical concepts. Nonetheless, it is possible that not all patients were able to appreciate the difference between a 4% and a 5% mortality rate. We encouraged participants to ask questions if they did not fully understand, but we did not test their understanding of the questions.
The participants in the study were parents of children without cardiac disease. Parents of children with cardiac disease were not recruited specifically to avoid potential confusion and psychological distress induced by the interview. In keeping with this, we used hypothetical scenarios, and asked the participants to think about the questions as real as possible. Furthermore, we did not ask the participants why they chose one approach over the other. More studies are needed to investigate further the decision-making processes of the parents.
The study was conducted in an urban public hospital, and the majority of participants was mother, Spanish-speaking, and uninsured or publicly insured. These characteristics may make extrapolation of our results to other populations difficult. Patients of different cultures may respond to the questions differently. Responses of patients in a health care system providing universal coverage, such as the United Kingdom, may also differ from responses of patients with various types of insurance coverage or no coverage in the United States.
In conclusion, we have shown that parental preference is an important consideration when planning for surgical repair of children with congenital cardiac disease. If the combined mortality rate for a two-stage approach is the same as for one-staged repair, more parents choose the option of a single stage. If the combined mortality is lower than that for one-staged repair, parents are more likely to choose the two-stage repair if the mortality for the first stage is lower. The age, gender, race, education and primary language of the parents are not associated with their decisions. When the mortality rates for the two approaches are the same, however, people without insurance are more likely to choose one-staged repair.
Primary Funding Sources: This study was supported, in part, by a research grant from the Harbor-UCLA Research and Education Institute, and grants (RR 00425 and 1 K23 RR17041-01) from National Center for Research Resources, National Institutes of Health.
