Adrienne H. Kovacs

Best Practices in Managing Transition to Adulthood for Adolescents With Congenital Heart Disease: The Transition Process and Medical and Psychosocial Issues: A Scientific Statement From the American Heart Association

Many children born with complex childhood illnesses that historically caused early death are now surviving into adulthood with the expectation of leading meaningful and productive lives. They will ultimately need to transition their care from pediatric to adult-centered care. Unfortunately, in the absence of structured programs to guide this transition, there is often delayed or inappropriate care, improper timing of the transfer of care, and undue emotional and financial stress on the patients, their families, and the healthcare system. At its worst, and as frequently happens now, patients are lost to appropriate follow-up. In fact, the number of adults with congenital heart disease (CHD) in the United States is rising exponentially and now exceeds 1 000 000.1,–,7 At least half of these patients may have complex CHD. Fewer than 30% of adults with CHD are seen by appropriate specialized providers. Fewer than 15% of these patients, who are seen in specialty adult CHD (ACHD) clinics, have CHD that is classified as severe.8 Thus, adolescents with CHD constitute a growing population of individuals for whom a well-planned and well-executed “transition process” is essential. The goals of a formal transition program are to prepare young adults for transfer of care. It should provide uninterrupted health care that is patient centered, age and developmentally appropriate, flexible, and comprehensive. It should include age-appropriate education about medical conditions and promote skills in communication, decision making, self-care, and self-advocacy.9,–,13 It should foster greater personal and medical independence and a greater sense of control over health, healthcare decisions, and psychosocial environment. The ultimate goal of a transition program is to optimize the quality of life (QOL), life expectancy, and future productivity of young patients.14 We acknowledge that the development of ideal transition programs is a …

Depression and anxiety in adult congenital heart disease: Predictors and prevalence

BACKGROUND Adult congenital heart disease (ACHD) patients face unique medical and social challenges that may contribute to psychological difficulties. The goals of this study were to identify predictors of symptoms of depression and anxiety and evaluate the prevalence of mood and anxiety disorders among North American ACHD patients. METHODS In this cross-sectional study, consecutive patients were recruited from two ACHD outpatient clinics. All patients completed self-report psychosocial measures and a subset was randomly selected to participate in structured clinical interviews. Linear regression models were used to predict symptoms of depression and anxiety. RESULTS A total of 280 patients (mean age=32 years; 52% female) completed self-report measures. Sixty percent had defects of moderate complexity and 31% had defects of great complexity. Significant predictors of depressive symptoms were loneliness (p<0.001), perceived health status (p<0.001), and fear of negative evaluation (p=0.02). Predictors of anxiety symptoms were loneliness (p<0.001) and fear of negative evaluation (p<0.001). Disease severity and functional class did not predict mood or anxiety symptoms. Fifty percent of interviewed patients (29/58) met diagnostic criteria for at least one lifetime mood or anxiety disorder, of whom 39% had never received any mental health treatment. CONCLUSIONS The results confirm an increased risk and under-treatment of mood and anxiety disorders in ACHD patients. Social adjustment and patient-perceived health status were more predictive of depression and anxiety than medical variables. These factors are modifiable and therefore a potential focus of intervention.

The ICD shock and stress management program: A randomized trial of psychosocial treatment to optimize quality of life in ICD patients

Background: Implantable cardioverter defibrillator (ICD) patients potentially face significant psychological distress because of their risk for life‐threatening arrhythmias and the occurrence of ICD shock.

Pregnancy and Contraception in Congenital Heart Disease: What Women Are Not Told

As increased numbers of patients with congenital heart disease (CHD) survive to adulthood more women with CHD are reaching reproductive age. Contraception and pregnancy have now become important issues in this population; however both can be associated with increased risks in women with CHD (1). Other issues such as adverse fetal outcomes in these women and transmission of CHD to offspring must be addressed. Current guidelines for the care of adults with CHD recommend proactive counseling regarding issues of contraception and pregnancy (2-5). The objective of this study was to evaluate whether women with CHD have adequate knowledge regarding risks of contraception and pregnancy. (authors)

Increasing Mortality Burden among Adults with Complex Congenital Heart Disease

BACKGROUND Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease. METHODS Among 12,644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029. RESULTS Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot. CONCLUSIONS Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease.

Gender and sex differences in prevalence of major depression in coronary artery disease patients: A meta-analysis

BACKGROUND Depression is related to increased morbidity and mortality in the general population and among patients with coronary artery disease (CAD). The prevalence of major depression is two-times higher in women than men in the general population, but whether this pattern holds true in the CAD population has not been established. OBJECTIVE To test, through quantitative synthesis, whether women with CAD have a greater prevalence of major depression than men. METHOD MEDLINE, EMBASE, and PsycINFO electronic databases were searched. Authors of key articles were contacted to identify other relevant publications. The titles and abstracts were screened by the first author and the selected full-text articles were independently screened by the first and second authors based on pre-defined inclusion criteria. Major depression had to be diagnosed through structured clinical interviews during cardiac-related hospitalization or post-CAD hospitalization. Meta-analysis was undertaken using the Review Manager 5 software program. All pooled analyses were based on random-effects models. RESULTS Eight eligible cohort and cross-sectional studies reporting data for 2072 participants (509 [24.6%] women) were included. Overall, major depression was observed in 95 (18.7%) women and 187 (12.0%) men. In the pooled analysis, prevalence of major depression was significantly greater in women compared to men (odds ratio=1.77, 95% confidence interval=1.21-2.58, p<.01). Heterogeneity was considered low to moderate (I(2)=36.0%). CONCLUSION Consistent with the general population, the prevalence of major depression is two-times greater in women than men with CAD. Women with CAD may warrant greater emphasis in efforts to identify and treat depression.

End-of-life care in hospitalized adults with complex congenital heart disease: care delayed, care denied:

Adults with congenital heart disease represent a growing patient population. Notwithstanding dramatic improvements in survival and life expectancy over recent decades, many of these patients remain at risk of premature death from progressive heart disease and would benefit from the principles of palliative and end-of-life care. Data on end-of-life care in this patient group are, however, lacking. We report a retrospective study of 48 patients with congenital heart disease who died while admitted to our hospital (mean age at death 37 ± 14 years). We describe circumstances of death, end-of-life discussions, and the provision of end-of-life care. The majority of patients had complex congenital heart disease and were considered to be in the end stage of their disease. Despite this, only a minority of patients had documented end-of-life discussions prior to their terminal admission and most received continuing aggressive medical treatment up to their demise. Advanced palliative and end-of-life care strategies should be developed for and provided to this group of patients, with the dual aims of reduction of unwarranted therapies and enhancement of the quality of death and dying.

Adults with congenital heart disease: psychological needs and treatment preferences.

OBJECTIVE Approximately one-third of adult congenital heart disease (ACHD) patients have mood or anxiety disorders, the majority of which go untreated. The extent to which this group of patients is interested in psychological services is, however, unknown. This study investigated the perceived psychological needs of patients and their mental health treatment preferences. DESIGN Participants completed an anonymous survey with items regarding mental health treatment history, preferences for future treatment, Internet use, and interest in peer support. RESULTS A total of 155 ACHD patients completed study questionnaires (mean age = 39 years; 50% female). Forty percent of patients reported previous mental health treatment. Some 51% of patients (73/142) indicated significant interest in at least 1 of 7 defined areas of psychological treatment (managing mood and/or anxiety, coping with a cardiac condition, stress management, anger management, relationship difficulties, substance use, and smoking cessation). Patients were most interested in stress management and coping with heart disease; one-third of patients (48/141 and 47/141, respectively) expressed high interest in these 2 topics. There was a consistent preference for psychological treatment to be provided over the Internet vs. in person or over the telephone; 94% of the patients had Internet access. In total, 35% of patients (51/145) were interested in receiving peer support. CONCLUSIONS It is not only health providers who recognize the importance of psychological care for ACHD patients. As a group, patients are also interested in psychological treatment and peer support. Programs are encouraged to provide psychological services and maximize opportunities for interactions with other patients.

Knowledge of and preference for advance care planning by adults with congenital heart disease.

Congenital heart disease (CHD) is a chronic illness. Few adults with CHD are cured and those with disease of moderate or great complexity remain at risk of premature death. Current adult CHD guidelines recommend that providers encourage their patients to complete advance directives. We evaluated the prevalence of completed advance directives by and the preference for information about life expectancy of outpatients at a large adult CHD program. Two hundred patients with CHD (52% men, 35 ± 15 years old, range 18 to 79, 81% with disease of moderate or great complexity) completed a survey that assessed knowledge of advance directives and nature of and preferences for advance care planning. Only 5% of patients reported that they had completed advance directives; 56% had never heard of them. However, most patients (87%) reported that they would prefer to have an advance directive available if they were dealing with their own dying and were unable to speak for themselves. Patients who had formally identified substitute decision makers (n = 34) were typically older (47 ± 16 vs 33 ± 13 years, p <0.001) and more likely to have partners (30% vs 6%, p <0.001). Most patients (70%) reported that they wanted general information about the average life expectancy for patients with their heart condition. In conclusion, in contrast to recommendations from published guidelines, advance care planning documents are infrequently completed by outpatients. Health care providers caring for patients with CHD should educate their patients about advance directives and assist them in preparing formal end-of-life-planning documents.

Cardiac Resynchronization Therapy: A Pilot Study Examining Cognitive Change in Patients Before and After Treatment

Cardiovascular patients with reduced cardiovascular output and capacity such as those with congestive heart failure (CHF) have demonstrated cognitive‐related dysfunction. The use of cardiac resynchronization therapy (CRT) is considered standard care for CHF patients who do not improve despite optimal medical therapy. Cardiac resynchronization therapy may improve neurocognitive and psychosocial functioning in patients by increasing cardiac output and cerebral perfusion.