Gwen R. Rempel

Diagnosis and Treatment of Fetal Cardiac Disease A Scientific Statement From the American Heart Association

Background— The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. Methods and Results— A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin–twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. Conclusions— Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care.

Safeguarding precarious survival: Parenting children who have life-threatening heart disease

The purpose of this study is to describe the process of parenting a child with life-threatening heart disease. Despite advances in pediatric cardiac sciences, hypoplastic left heart syndrome remains difficult and controversial to treat. The Norwood surgical approach is a developing technology, and little is known about how mothers and fathers experience parenting a child who survives this treatment. Constructivist grounded theory informed this Canadian study that involved multiple interactive interviews with 9 mothers and 7 fathers of children with hypoplastic left heart syndrome who were at various stages of treatment. Extraordinary parenting in a context of uncertainty was evident as parents simultaneously safeguarded their childs precarious survival as well as their own survival. As technologically advanced treatment contributes to the survival of children with complex health conditions, health care professionals must consider how to promote and support parenting strategies that benefit the new survivors of technology as well as their families.

Interactive Use of Genograms and Ecomaps in Family Caregiving Research

This article argues for the concurrent and comparative use of genograms and ecomaps in family caregiving research. A genogram is a graphic portrayal of the composition and structure of ones family and an ecomap is a graphic portrayal of personal and family social relationships. Although development and utilization of genograms and ecomaps is rooted in clinical practice with families, as research tools they provide data that can enhance the researchers understanding of family member experiences. In qualitative research of the supportive and nonsupportive interactions experienced by male family caregivers, the interactive use of genograms and ecomaps (a) facilitated increased understanding of social networks as a context for caregiving, (b) promoted a relational process between researcher and participant, and (c) uncovered findings such as unrealized potential in the participants social network that may not be revealed with the use of the genogram or ecomap alone, or the noncomparative use of both.

Parents' Perspectives on Decision Making After Antenatal Diagnosis of Congenital Heart Disease

OBJECTIVE To discover and describe how prospective parents make decisions when they learn of their babys congenital heart disease (CHD) during pregnancy, and to provide professionals with direction for their interactions with these families. DESIGN AND METHOD Qualitative analysis informed by symbolic interactionism. SETTING A tertiary care womens health center that provided referral services for a province with a population of 4 million. PARTICIPANTS Mothers and fathers of 19 babies with antenatally diagnosed CHD participated in interviews during pregnancy and after the birth of their baby. Thirty-four interviews were analyzed for common themes and distinguishing characteristics of antenatal decision making. RESULTS Parents approached their antenatal decisions regarding further testing and continuation of the pregnancy as their first parenting decisions. They made their decisions with differing degrees of apparent ease or deliberation, and some parents more readily sought the opinion of professionals. The offered opinions offended some parents, even though the professionals may have intended the information as descriptive of options, not suggestive of a particular decision. CONCLUSION Although advances in technology have enabled diagnosis of CHD antenatally, health care professionals, including nurses, must elicit each parents particular perspective, be cognizant of their professional influence, and actively support parents from the time of the antenatal diagnosis.

Healthcare transition for youth with heart disease: a clinical trial

Objectives Adolescents with heart disease have complex health needs and require lifelong cardiology follow-up. Interventions to facilitate paediatric to adult healthcare transition are recommended, although outcomes are unknown. We sought to determine the impact of a transition intervention on improving knowledge and self-management skills among this population. Methods We conducted a clinical trial of 15–17 year olds with moderate or complex congenital heart disease (CHD) or cardiomyopathy. Participants were systematically allocated to either usual care (controls) or a 1 h nurse-led one-on-one teaching session about their heart. Allocation was determined by week of attendance in the cardiology clinic. The primary outcome was change in Transition Readiness Assessment Questionnaire (TRAQ) score at 6 months, possible scores ranging from 1 (low) to 5 (optimal). Cardiac knowledge (MyHeart score, range 0–100) was a secondary outcome. Analysis was intention to treat. Results Of 58 participants (48% female), 52 had CHD and 6 had cardiomyopathy. 27 were allocated to the intervention group; 3 declined the intervention and received usual care. When comparing the intervention group with the usual care group at 6 months postintervention, the mean self-management TRAQ score was 3.59 (±0.83) vs 3.16 (±1.05), respectively (p=0.048, adjusted for baseline score); the mean self-advocacy TRAQ score was 4.38 (±0.56) vs 4.01 (±0.95) (p=0.18) and the mean MyHeart score was 75% (±15) vs 61% (±25) (p=0.019). Conclusions A 1 h nurse-led transition intervention resulted in a significant improvement in self-management and cardiac knowledge scores. An educational intervention should be routine for youth with congenital or acquired heart disease. Trial registration number NCT01286480

Parenting under pressure: a grounded theory of parenting young children with life-threatening congenital heart disease.

AIM To report a grounded theory study to describe the process of parenting young children who have survived hypoplastic left heart syndrome to inform parent-focused interventions. BACKGROUND Technological advances in paediatric cardiology worldwide have improved the survival rates for young children with hypoplastic left heart syndrome who undergo staged surgical palliation. These children, however, are at risk for life-threatening complications and parents are charged with the responsibility to monitor their children at home with minimal support and guidance from healthcare professionals once home. DESIGN A constructivist grounded theory study. METHOD The study was conducted in 2006-2008. Participants were 25 parents (15 mothers, 10 fathers) and 28 grandparents (17 grandmothers, 11 grandfathers) of 15 young children (6 months-4·5 years) who had undergone the Sano surgical approach for hypoplastic left heart syndrome. The 53 interviews were digitally recorded, transcribed and analysed using open and focused coding, constant comparative analysis and memoing. FINDINGS A process of Parenting under Pressure emerged that was characterized by four overlapping and re-emerging phases: (1) realizing and adjusting to the inconceivable; (2) growing increasingly attached; (3) watching for and accommodating the unexpected; and (4) encountering new challenges. CONCLUSIONS In-depth understanding of the phases of Parenting under Pressure provides direction for nurses to support parents of children who survive hypoplastic left heart syndrome. Interventions that help carers of children with complex health conditions move through the phases of our Parenting under Pressure process may help them safeguard the survival of their children, and their own survival as parents as they manage multiple demands.

Is ''Treat your child normally'' helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome?

BACKGROUND Developing technology affords children with complex congenitally malformed hearts a chance for survival. Parents gratefully pursue life-saving options on behalf of their children, despite the risks to the life of their child, and uncertainty about outcomes. Little is known about how mothers and fathers experience parenting a child whose new state as a survivor may include less than optimal developmental sequels. METHOD Our study involved multiple interactive interviews with 9 mothers and 7 fathers of infants and preschool children with hypoplastic left heart syndrome who had survived the Norwood surgical approach. Qualitative methodology included grounded theory methods of simultaneous collection and analysis of data, and we used open and selective coding of transcribed interviews. RESULTS Parents used normalization in the context of uncertainty regarding the ongoing survival of their child. Parents described their underweight children as being on their own growth curve, and viewed their developmental progress, however delayed, as reason for celebration, as they had been prepared for their child to die. CONCLUSION There is growing evidence that children with congenitally malformed hearts who require surgical intervention during the first year of life may experience developmental delay. The use of normalization by their parents may be effective in decreasing their worry regarding the uncertain future faced by their child, but may negatively affect the developmental progress of the child if they do not seek resources to assist development. Advice from paediatric specialists for parents to view their children as normal needs to be balanced with assistance for parents to access services to support optimal growth and development of their child.

Risk factors for loss to follow-up among children and young adults with congenital heart disease

OBJECTIVE To identify risk factors for loss to cardiology follow-up among children and young adults with congenital heart disease. METHODS We used a matched case-control design. Cases were born before January, 2001 with moderate or complex congenital heart disease and were previously followed up in the paediatric or adult cardiology clinic, but not seen for 3 years or longer. Controls had been seen within 3 years. Controls were matched 3:1 to cases by year of birth and congenital heart disease lesion. Medical records were reviewed for potential risk factors for loss to follow-up. A subset of cases and controls participated in recorded telephone interviews. RESULTS A total of 74 cases (66% male) were compared with 222 controls (61% male). A history of missed cardiology appointments was predictive of loss to follow-up for 3 years or longer (odds ratio 13.0, 95% confidence interval 3.3-51.7). Variables protective from loss to follow-up were higher family income (odds ratio 0.87 per

Interpreter-facilitated cross-language interviews: a research note

10,000 increase, 0.77-0.98), cardiac catheterisation within 5 years (odds ratio 0.2, 95% confidence interval 0.1-0.6), and chart documentation of the need for cardiology follow-up (odds ratio 0.4, 95% confidence interval 0.2-0.8). Cases lacked awareness of the importance of follow-up and identified primary care physicians as their primary source of information about the heart, rather than cardiologists. Unlike cases, controls had methods to remember appointments. CONCLUSIONS A history of one or more missed cardiology appointments predicted loss to follow-up for 3 or more years, as did lack of awareness of the need for follow-up. Higher family income, recent catheterisations, and medical record documentation of the need for follow-up were protective.

Parenting children with hypoplastic left heart syndrome: Finding a balance

This research note focuses on interpreter-facilitated cross-language qualitative interviews. Although researchers have written about strategies and procedures for working with interpreters, rarely have they offered adequate detail to determine the relative merits of various approaches, and little attention has been paid to the influence that interpreters have on the validity of qualitative data. We advance this body of literature by describing and critically examining the strategies and procedures we used to work with an interpreter to conduct qualitative interviews with Mandarin-speaking grandparents who participated in our study of intergenerational social support during the transition to parenthood. In addition, we examine the influence that our strategies and procedures had on the data generation process and on the validity of the data. Drawing on our experiences, we argue that with adequate preparation, validity checks, and the supplementary strategies that we describe in this article, an interpreter-facilitated interview approach to generating data in cross-language studies can be an effective alternative to more commonly used and more laborious and expensive translation practices.