Curt J. Daniels

Hypoplastic Left Heart Syndrome : Current Considerations and Expectations

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.

Reopening After Successful Coil Occlusion for Patent Ductus Arteriosus

OBJECTIVES This study was performed to determine the frequency of patent ductus arteriosus (PDA) reopening and the factors that may predict reopening after successful coil occlusion. BACKGROUND Transcatheter coil occlusion is a widely used and accepted method to close a PDA. After documented successful coil occlusion, we found PDAs that reopened. We hypothesized that specific factors are involved in those that reopened. METHODS All patients who underwent percutaneous transarterial PDA coil occlusion were studied. Successful coil occlusion was documented. PDA reopening was determined when Doppler-echocardiography (DE) performed after the procedure was negative for PDA flow but at follow-up demonstrated PDA shunting. Patients with a reopened PDA were compared with all other patients in evaluating independent variables. RESULTS Coil occlusion for PDA was attempted in 22 patients. Clinical success was achieved in 20 patients (91%), and DE was negative for PDA shunting in 19 patients (90%). At follow-up, five patients demonstrated reopening. The PDA minimal diameter was 1.4 +/- 0.5 mm (mean +/- SD) for the reopened group and 1.2 +/- 0.7 mm for the other patients. The PDA length was 2.9 +/- 1.9 mm for the reopened group and 7.1 +/- 3.2 mm for all other patients. All those with type B PDA were in the reopened group. When independent variables were compared between groups, only PDA length and type B PDA predicted reopening (p < 0.05). CONCLUSIONS PDA reopening may occur after successful coil occlusion. Short PDA length and type B PDA are associated with reopening. The data suggest that in such anatomy, alternative strategies to the current coil occlusion technique should be considered.

Acute pulmonary toxicity in an infant from intravenous amiodarone

Intravenous amiodarone is an effective treatment for supraventricular and ventricular tachyarrhythmias. We report a case of acute pulmonary toxicity in an infant from intravenous amiodarone and describe the clinical evaluation and laboratory studies leading to the diagnosis.

The care of adults with congenital heart disease across the globe: Current assessment and future perspective

The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.

Emerging Research Directions in Adult Congenital Heart Disease: A Report From an NHLBI/ACHA Working Group.

Congenital heart disease (CHD) is the most common birth defect, affecting about 0.8% of live births. Advances in recent decades have allowed >85% of children with CHD to survive to adulthood, creating a growing population of adults with CHD. Little information exists regarding survival, demographics, late outcomes, and comorbidities in this emerging group, and multiple barriers impede research in adult CHD. The National Heart, Lung, and Blood Institute and the Adult Congenital Heart Association convened a multidisciplinary working group to identify high-impact research questions in adult CHD. This report summarizes the meeting discussions in the broad areas of CHD-related heart failure, vascular disease, and multisystem complications. High-priority subtopics identified included heart failure in tetralogy of Fallot, mechanical circulatory support/transplantation, sudden cardiac death, vascular outcomes in coarctation of the aorta, late outcomes in single-ventricle disease, cognitive and psychiatric issues, and pregnancy.

Rate of inducible ventricular arrhythmia in adults with congenital heart disease

Patients with adult congenital heart disease are at increased risk of ventricular arrhythmia (VA) and sudden cardiac death, although no clear predictors have been found. Ventricular programmed stimulation has been shown to predict clinical ventricular tachycardia and sudden death events, but the role of screening electrophysiology studies (S-EPSs) in this population remains poorly defined. Therefore, we sought to determine the prevalence of inducible VA and to evaluate the clinical predictors in a heterogeneous group of patients with adult congenital heart disease (> or =18 years old) undergoing S-EPSs at preoperative or interventional cardiac catheterization. Studies for the primary evaluation of clinical VA were excluded. The demographic, clinical, and diagnostic findings were compared between the patients with positive and negative findings. From 2005 to 2009, 80 patients (mean age 30 +/- 9 years) underwent S-EPSs, and 23 had inducible VA. The diagnoses for those with studies positive for VA included tetralogy of Fallot (n = 12), d-transposition of the great arteries (n = 6), pulmonary stenosis (n = 2), double outlet right ventricle (n = 1), double inlet left ventricle (n = 1), and Ebsteins anomaly (n = 1). Men were significantly more likely to have a S-EPS positive for VA (p = 0.015). Increasing QRS duration, decreasing peak oxygen uptake (percentage of predicted), and ventricular fibrosis with cardiovascular magnetic resonance imaging were significantly associated with studies positive for VA (p <0.05). Combined fibrosis and a peak oxygen uptake <80% of predicted had 100% sensitivity for positive VA findings. In conclusion, almost 30% of those with adult congenital heart disease undergoing S-EPSs had inducible VA. A prolonged QRS duration, diminished exercise capacity, and the presence of ventricular fibrosis were significantly associated with findings positive for VA and might improve patient selection for screening evaluations.

Pulmonary Hypertension Idiopathic Pulmonary Fibrosis: A Dastardly Duo

Abstract:Pulmonary hypertension (PH) is a well-recognized complication of interstitial lung disease, including idiopathic pulmonary fibrosis (IPF). The underlying pathogenesis was initially hypothesized to be inflammatory but now is characterized as an over exuberant fibroproliferative process. The prevalence of PH in the setting of IPF has not been well described in the literature, with a reported occurrence from 32% to 85%. Diagnostically, recognizing underlying PH in the setting of IPF remains challenging because of nonspecific clinical symptoms and unrevealing ancillary testing. A high degree of clinical suspicion is paramount. The only reliable diagnostic tool for PH is right heart catheterization. The treatment of PH, in patients with IPF, is based on multiple factors, including disease severity, functional status and degree of hypoxemia. Medications currently approved to treat PH have been administered for PH in the setting of IPF, such as phosphodiesterase-5 inhibitors, nonselective endothelin receptor antagonists and prostacyclin analogues. The treatment of PH in the setting of IPF may also be difficult due to worsening ventilation-perfusion mismatch induced by selective pulmonary artery vasodilator therapy. Lung transplantation should be considered with patients refractory to pharmacological treatment. Identification of PH in IPF patients is crucial, as functional status and prognosis are greatly reduced. Given the high mortality rate and propensity for acute decompensation, IPF and PH patients should be evaluated for transplant early in their disease course.

State-of-the-Art PaperSports and Exercise Cardiology in the United States: Cardiovascular Specialists as Members of the Athlete Healthcare Team

In recent years, athletic participation has more than doubled in all major demographic groups, while simultaneously, children and adults with established heart disease desire participation in sports and exercise. Despite conferring favorable long-term effects on well-being and survival, exercise can be associated with risk of adverse events in the short term. Complex individual cardiovascular (CV) demands and adaptations imposed by exercise present distinct challenges to the cardiologist asked to evaluate athletes. Here, we describe the evolution of sports and exercise cardiology as a unique discipline within the continuum of CV specialties, provide the rationale for tailoring of CV care to athletes and exercising individuals, define the role of the CV specialist within the athlete care team, and lay the foundation for the development of Sports and Exercise Cardiology in the United States. In 2011, the American College of Cardiology launched the Section of Sports and Exercise Cardiology. Membership has grown from 150 to over 4,000 members in just 2 short years, indicating marked interest from the CV community to advance the integration of sports and exercise cardiology into mainstream CV care. Although the current athlete CV care model has distinct limitations, here, we have outlined a new paradigm of care for the American athlete and exercising individual. By practicing and promoting this new paradigm, we believe we will enhance the CV care of athletes of all ages, and serve the greater athletic community and our nation as a whole, by allowing safest participation in sports and physical activity for all individuals who seek this lifestyle.

Erectile dysfunction in men with congenital heart disease.

There has been a tremendous focus on gynecologic health with the increasing number of women with congenital heart disease (CHD). Conversely, experience in providing sexual health screening to men with CHD is lacking. The purpose of this study was to identify sexual health issues in men with CHD. An anonymous survey was distributed to men in our adult CHD clinic. Age, type of CHD, medications, detailed sexual history including cardiovascular symptoms with intercourse, erectile dysfunction (ED), and willingness to discuss ED with a physician was obtained. A logistic regression analysis was performed to analyze the relation between medications, CHD complexity, and ED; 86 men completed the survey with a mean age of 34 +/- 10 years. The prevalence of ED was 38%. Men were treated with cardiac medications including beta blockers (BB; 24%), angiotensin-converting enzyme (ACE) inhibitors (8%), calcium-channel blockers (6%), and combination BB and ACE inhibitor therapy (16%). Men on BB were 3.13 times more likely to report ED (p = 0.045). Complexity of CHD did not increase the likelihood of ED symptoms (p >0.10). Although most men attributed symptoms to their underlying CHD, most were willing to discuss issues of ED with their cardiologist. In conclusion, ED is prevalent in young men with CHD. Although an emotional component may contribute, certain medications may exacerbate symptoms.

Emotional Functioning of Adolescents and Adults with Congenital Heart Disease: A Meta-Analysis

OBJECTIVE This study aimed to quantitatively compare findings of emotional functioning across studies of adolescents and adults with congenital heart disease (CHD) through meta-analysis. DESIGN The current meta-analysis included 22 studies of adolescent and adult survivors of CHD who completed measures of emotional functioning. Effect sizes were represented by Hedges g. Heterogeneity was calculated and possible moderators (i.e., lesion severity, age, study location, study quality) were examined. RESULTS Overall, adolescent and adult survivors of CHD did not differ in emotional functioning from healthy controls or normative data. However, significant heterogeneity was found, and there was a trend for degree of lesion severity to moderate emotional functioning. Further analysis of lesion severity indicated that individuals with moderate lesions reported better emotional functioning than controls/normative data. Limitations in existing literature precluded examination of patient age as a moderator. Study location and quality did not explain a significant portion of the variance in effects. CONCLUSIONS Findings suggest that differences in emotional functioning may exist across lesion severities, and individuals with moderately severe lesions are emotionally thriving. Given the diversity within CHD lesion classifications, future studies should include other indicators of disease severity, such as measures of morbidity, to determine how disease may affect emotional functioning among survivors of CHD. Furthermore, authors and journals need to ensure that research is reported in enough detail to facilitate meta-analysis, a critically important tool in answering discrepancies in the literature.