[Show abstract][Hide abstract] ABSTRACT: Objective
Nurse practitioners (NPs) have an established role for delivering competent care to patients in the primary care setting. The aim of this study was to compare satisfaction of patients managed by NPs vs. physicians in the outpatient adult congenital heart disease (CHD) clinic.DesignA prospective study conducted in two outpatient adult CHD clinics to assess patient satisfaction through standardized surveys. Demographic data included CHD diagnosis, age, and New York Heart Association functional class. All patients completed a Short-Form-12 to assess health status.ResultsOf the 371 patients recruited (52% men; median age 29 years, severe CHD complexity 31.5%) and seen by NP (n = 187) or physician (n = 184), physician-managed practices had higher perceived: overall experience, courtesy of provider (P < 0.05) and confidence (trust) in provider (P < 0.1). Overall, patients reported satisfaction with an NP providing care (98%), the NP was able to effectively deal with illness/CHD (95%), and an increased chance (94%) or willingness to see an NP at a future visit. Only 73% reported an understanding of NP training and how an NP differed from a registered nurse. There was a stronger perception of how an NP differed from a physician (83%).Conclusion
Patient satisfaction was high regardless of whether care was provided by physicians or NPs. However, patients appear to make distinctions in what they believe the type of care each practitioner is best at providing. Patient education regarding competence of the different health care providers may continue to improve patient satisfaction.
Full-text · Article · Jun 2015 · Congenital Heart Disease
[Show abstract][Hide abstract] ABSTRACT: Basic knowledge of genetics is essential for understanding genetic testing and counseling. The lack of a written, English language, validated, published measure has limited our ability to evaluate genetic knowledge of patients and families. Here, we begin the psychometric analysis of a true/false genetic knowledge measure. The 18-item measure was completed by parents of children with congenital heart defects (CHD) (n = 465) and adolescents and young adults with CHD (ages 15-25, n = 196) with a mean total correct score of 12.6 (SD=3.5, range 0-18). Utilizing exploratory factor analysis, we determined that one to three correlated factors, or abilities, were captured by our measure. Through confirmatory factor analysis, we determined that the two factor model was the best fit. Although it was necessary to remove two items, the remaining items exhibited adequate psychometric properties in a multidimensional item response theory analysis. Scores for each factor were computed, and a sum-score conversion table was derived. We conclude that this genetic knowledge measure discriminates best at low knowledge levels and is therefore well suited to determine a minimum adequate amount of genetic knowledge. However, further reliability testing and validation in diverse research and clinical settings is needed.
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No preview · Article · May 2015 · Clinical Genetics
[Show abstract][Hide abstract] ABSTRACT: Aberrant cellular oxygen sensing (pseudo-hypoxia) is a leading theory for development of pheochromocytoma (PHEO) and paraganglioma (PGL).
To test the hypothesis that chronic hypoxia in patients with cyanotic congenital heart disease (CCHD, CHD) increases the risk for PHEO-PGL.
We investigated the association between CCHD and PHEO-PGL with 2 complementary studies: Study 1) An international consortium was established to identify patients with PHEO-PGL diagnosis confirmed by pathology, or biochemistry and imaging. Study 2) The 2000-2009 Nationwide Inpatient Survey, a nationally representative discharge database, was used to determine population-based cross-sectional PHEO-PGL frequency in hospitalized CCHD patients compared with non-cyanotic CHD and those without CHD using multivariable logistic regression adjusted for age, sex, and genetic PHEO-PGL syndromes.
Study 1) We identified 20 PHEO-PGL cases, of which 18 had CCHD. Most presented with cardiovascular or psychiatric symptoms. Median cyanosis duration for the CCHD PHEO-PGL cases was 20y (range 1-57y). Cases were young at diagnosis (median 31.5y, range 15-57) and 7/18 had multiple tumors (2 bilateral PHEO; 6 multi-focal or recurrent PGL) while 11 had single tumors (7 PHEO; 4 PGL). PGL were abdominal (13/17) or head/neck (4/17). Cases displayed a noradrenergic biochemical phenotype similar to reported hypoxia-related PHEO-PGL genetic syndromes, but without clinical signs of such syndromes. Study 2) Hospitalized CCHD patients had increased likelihood of PHEO-PGL (adjusted OR=6.0, 95%CI [2.6-13.7], p<0.0001) compared with those without CHD; patients with non-cyanotic CHD had no increased risk (OR=0.9, p=0.48).
There is a strong link between CCHD and PHEO-PGL. Whether these rare diseases co-associate due to hypoxic stress, common genetic or developmental factors, or some combination, requires further investigation.
No preview · Article · Jan 2015 · The Journal of Clinical Endocrinology and Metabolism
[Show abstract][Hide abstract] ABSTRACT: The univentricular heart includes a spectrum of complex cardiac defects that are managed by staged palliative surgical procedures, ultimately resulting in a Fontan procedure. Since 1971, when it was first developed, the procedure has undergone several variations. These patients require lifelong management, including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function, together with multi-organ evaluation. As these patients enter middle age, there is increasing awareness of long-term complications and mortality. This review highlights the concept behind the staged surgical palliations, the unique single ventricle physiology and the long-term complications in this complex cohort of patients.
No preview · Article · Oct 2013 · Circulation Journal
[Show abstract][Hide abstract] ABSTRACT: Background
Adult congenital heart disease (ACHD) clinicians are hampered by the paucity of data to inform clinical decision-making. The objective of this study was to identify priorities for clinical research in ACHD.
A list of 45 research questions was developed by the Alliance for Adult Research in Congenital Cardiology (AARCC), compiled into a survey, and administered to ACHD providers. Patient input was sought via the Adult Congenital Heart Association at community meetings and online forums. The 25 top questions were sent to ACHD providers worldwide via an online survey. Each question was ranked based on perceived priority and weighted based on time spent in ACHD care. The top 10 topics identified are presented and discussed.
The final online survey yielded 139 responses. Top priority questions related to tetralogy of Fallot (timing of pulmonary valve replacement and criteria for primary prevention ICDs), patients with systemic right ventricles (determining the optimal echocardiographic techniques for measuring right ventricular function, and indications for tricuspid valve replacement and primary prevention ICDs), and single ventricle/Fontan patients (role of pulmonary vasodilators, optimal anticoagulation, medical therapy for preservation of ventricular function, treatment for protein losing enteropathy). In addition, establishing criteria to refer ACHD patients for cardiac transplantation was deemed a priority.
The ACHD field is in need of prospective research to address fundamental clinical questions. It is hoped that this methodical consultation process will inform researchers and funding organizations about clinical research topics deemed to be of high priority.
No preview · Article · Jan 2013 · International journal of cardiology
[Show abstract][Hide abstract] ABSTRACT: AimsThere is a fivefold increase in the frequency of intracranial aneurysm (IA) in adults with coarctation of the aorta (CoA). Current guidelines for management of adults with CoA recommend computed tomography angiography (CTA) or magnetic resonance imaging of the intracranial vessels. However, this recommendation has not been universally accepted. The purpose of our study was to prospectively perform CTA of the intracranial vessels in adults with CoA to evaluate the prevalence and identify high-risk features of this complication. Methods and ResultsFrom January 2008 to February 2011, adults 18 years of age with CoA were prospectively enrolled in a screening program with CTA of the intracranial vessels. Analyses of prognostic variables were performed with both Fisher's exact and two sample t-test. Forty-three patients (58% female, 33.55 10.21 years) with CoA completed CTA of the intracranial vessels. Five patients (11%) were found to have IA. Patients with IA were older than those without (45.6 +/- 8.17 vs. 30.89 +/- 7.89, P = 0.0003). There were no statistically significant differences detected between measurements of fasting lipid profiles, C-reactive protein, brain natriuretic peptide, and homocysteine levels among CoA patients with and without IA (P = not significant). Conclusion
Prospective screening of adults with CoA confirmed the increased prevalence of IA but also identified increased age as the sole risk factor. These data suggested that screening is justified particularly in the fourth and fifth decades of life. Further studies are required that focus on the development, natural history, and treatment of IA.
No preview · Article · Dec 2012 · Congenital Heart Disease
[Show abstract][Hide abstract] ABSTRACT: Background:
In studies of adult patients, increased QRS duration and mechanical dyssynchrony have been associated with decreased ventricular function. The aim of this study was to test the hypothesis that similar findings would be present in a population of patients with hypoplastic left heart syndrome (HLHS) after the Fontan procedure.
A retrospective cross-sectional study was conducted. All patients with HLHS after the Fontan procedure were eligible. QRS duration was measured using 12-lead electrocardiography. Echocardiographic measurements of mechanical dyssynchrony included Doppler tissue imaging (DTI) QRS to onset of s' wave difference between the left ventricle and the right ventricle, time to peak strain, time to peak systolic strain rate (SRs), the standard deviation of time to peak strain rate (modified Yu strain), and the standard deviation of time to peak SRs (modified Yu SRs). Right ventricular (RV) functional measurements included DTI s' wave, DTI RV myocardial performance index, global strain, global SRs, and RV fractional area change. Pearson's correlations were performed between the variables.
Thirty-one echocardiographic studies were performed on 26 patients. The median age was 5.3 years (range, 2.5-15.4 years). QRS duration was correlated significantly with global SRs (r = 0.42). Time to peak SRs was correlated significantly with DTI s' wave (r = -0.48) and global SRs (r = 0.37). Modified Yu SRs was correlated significantly with global strain (r = 0.35) and RV fractional area change (r = -0.35).
Both QRS duration and mechanical dyssynchrony were correlated with RV function, albeit weakly. The clinical significance of these findings is intriguing, but only larger studies will determine if these measurements are reliable in guiding treatment options for this complex patient population.
Full-text · Article · Dec 2012 · Journal of the American Society of Echocardiography: official publication of the American Society of Echocardiography
[Show abstract][Hide abstract] ABSTRACT: Coarctation of the aorta (CoA) occurs in 0.04% of the population and accounts for approximately 10% of lesions in adults with congenital heart disease (ACHD). It can occur as an isolated lesion or as a part of a complex defect and is associated with multiple complications, both with and without treatment. Since the first surgical repair in 1944, significant refinement in surgical techniques has expanded the available treatment options. While transcatheter therapy for both native and especially recurrent CoA has become the standard of care for young patients, the use of bare metal stent has been associated with significant complications in adults. Perhaps one of the most important advances in the management of CoA, has been the development of covered stent technology. Covered stents offer the advantage of excluding any stretch-induced wall trauma from the endoluminal aspect of the aorta, particularly in the catastrophic event of aortic rupture which has been reported. Here we describe the indications for and the limitations in the use of covered stents in the treatment of CoA. These may be used as the therapy of choice in patients with native CoA, complications after prior CoA repair, patients with severe and complex CoA lesions, or patients with advanced age.
No preview · Article · Dec 2012 · Progress in Pediatric Cardiology
[Show abstract][Hide abstract] ABSTRACT: Background:
With advances in cardiac care, patients with congenital heart disease, including single ventricle (SV) physiology, now survive into adulthood. These patients often suffer from congestive heart failure (CHF) with overexpression of serum biomarkers. Strain and strain rate (SR) may better describe the myocardial mechanics of a failing SV. Our objective was to determine the correlation between strain/SR and biomarkers in adult patients with SV and CHF.
Adult patients (age ≥8 years) with a SV were enrolled. Strain/SR in a 16-segment model of a SV was measured using 2D speckle echocardiography. Serum levels of interleukin 6, interleukin 8, matrix metalloproteinase 9, procollagen I C-terminal peptide (PCIP), cross-linked carboxy-terminal telopeptide of type I collagen (ICTP), pro-B-type natriuretic peptide, nitrotryrosine, tissue growth factor beta (TGF-β), tumor necrosis factor alpha, vascular endothelial growth factor, and creatinine (Cr) were measured. Patients underwent a complete 6 Minute Walk Test (MWT). Pearson correlation coefficient was used. P <.05 was considered significant.
Ten patients with SV (LV = 7, RV = 3) were enrolled. Mean age was 35.5 years (25-42 years). Mean single ventricular ejection fraction (SVEF) was 47%. ICTP correlated with the basal, mid, and apical anterolateral SR, as well as apical anterior and inferior SR. PCIP correlated with mid anterolateral, basal anteroseptal, and mid inferolateral SR. TGF-β correlated with apical inferior SR. Cr correlated with mid inferior-septal and apical lateral SR. 6 MWT negatively correlated with the apical anterior septum SR.
ICTP, Cr, and PCIP correlated best with segmental SR values. Our results provide a preliminary platform for future studies to follow the results of treatment modalities using strain/SR and biomarkers for CHF in this population.
Full-text · Article · Oct 2012 · Congenital Heart Disease
[Show abstract][Hide abstract] ABSTRACT: Background: Currently >85% of children in the US with congenital heart disease (CHD) survive to adulthood. Patients with repaired Tetralogy
of Fallot (TOF) and critical pulmonary stenosis (PS) frequently present with late sequelae including pulmonary insufficiency, right ventricular (RV)
enlargement and systolic dysfunction. These patients may have left ventricular (LV) systolic dysfunction for no apparent cause. Prior studies have
demonstrated improvement in RV size and function after pulmonary valve replacement (PVR); however changes in LV function post-PVR have rarely
been described. We sought to determine the mid term results on LV function after PVR using cardiac magnetic resonance imaging (CMR).
Methods: Retrospective review of pts (≥16 years) from 1/1/06 to 7/1/11 was performed. Demographics, clinical variables, cardiopulmonary
bypass (CPB) time and post-op medication use were reviewed. Pre and post-PVR CMR scans were analyzed for LV and RV volumes and ejection
fraction (EF). Paired t-test was used to compare pre and post-PVR volumes and EF. Pearson linear correlation and multivariate analysis evaluated the
effect of variables on LVEF.
Results: There were 40 pts who met inclusion criteria (TOF=29, PS=11). Mean age was 29±9 yrs (range:16-53). Each pt had a CMR before (173
days±103) and after PVR (446 days±264). For the entire cohort, the mean LVEF increased from 54±8% to 57±6% (p = 0.023). Pre-PVR, 9 pts had
depressed LVEF (44±5%, range 36-49%). In this group, the LVEF significantly increased post-PVR by 10±7% (p=.004). By multivariate analysis to
assess CMR predictors for improved LVEF post-PVR, the only variable to emerge was low EF pre-PVR (regression coefficient:-.7,R2=.59, p<0.0001).
Demographics, clinical variables, medications, prior pregnancies and CPB time had no effect on LVEF post-PVR.
Conclusion: This is the largest CMR study to describe the mid-term results of LV function after PVR in adults with CHD. Patients with depressed
LVEF pre-PVR had significant improvement in LV function post-PVR. Larger studies are needed to further evaluate predictors and pathophysiologic
mechanisms associated with improved LVEF.
[Show abstract][Hide abstract] ABSTRACT: Bartonella spp. are a known cause of culture-negative endocarditis. We report a 42-year-old male with underlying surgically corrected congenital heart disease who was diagnosed with Bartonella henselae endocarditis involving the right-ventricle-to-pulmonary-artery conduit.
No preview · Article · Mar 2012 · Journal of Cardiac Surgery
[Show abstract][Hide abstract] ABSTRACT: Purpose: Patients with systemic right ventricle (RV) after Fontan procedure are at increased risk for heart failure. In adult studies, increased mechanical and/or electrical dyssynchrony have been associated with decreased ventricular function. The goal of this study was to determine if similar correlations exist in a population of single RV patients status post Fontan.
Methods: All patients with a single RV following Fontan were eligible. Retrospective cross sectional analysis of echocardiograms and electrocardiograms (EKGs) was performed. Electrical dyssynchrony was defined as QRS duration which was measured on a 12 lead EKG. Echocardiographic measurements of mechanical dyssynchrony included TDI QRS to onset of S wave difference between left ventricle (LV) and RV, time to peak strain, time to peak strain rate, modified Yu strain, and modified Yu strain rate. RV function measurements included TDI s wave, TDI RV MPI, global strain, global strain rate and RV fractional area change. Pearson’s correlations were performed between the variables. A p value of <0.05 was considered significant.
Results: Thirty echocardiograms were performed on 27 patients. Twenty one patients had hypoplastic left heart syndrome, four had double-outlet right ventricle, two had unbalanced atrioventricular septal defect. Mean age was 6.7 +/- 4.3 yrs. Mean QRS duration was 97 +/- 21 msec. Mean TDI QRS to onset of S wave difference between LV and RV was 19.1 +/- 15.1 msec, time to peak strain was 357 +/- 51 msec, time to peak strain rate was 190 +/- 41 msec, modified Yu strain was 51.2 +/- 14.9, and modified Yu strain rate was 130.8 +/- 63.1. Mean TDI s wave was 0.05 +/- 0.01 m/s, TDI RV MPI was 0.4 +/- 0.1, global strain rate was -1.03 +/- 0.23 1/s, global strain was -17.3 +/- 3.4% and RV fractional area change was 29.1 +/- 5.8%. QRS duration correlated significantly with global strain (r = 0.41) and global strain rate (r = 0.61). Time to peak strain and strain rate correlated significantly with TDI s wave (r = -0.39, r = -0.54, respectively). Modified Yu strain rate correlated significantly with global strain (r = 0.48), global strain rate (r = 0.54) and RV fractional area change (r = -0.43).
Conclusion: Both electrical and mechanical dyssynchrony correlated with RV function. Electrical dyssynchrony had a slightly greater correlation than mechanical dyssynchrony with RV function. Future studies are needed to determine if correcting one or both of these parameters will improve function in single RV patients.
[Show abstract][Hide abstract] ABSTRACT: Double outlet right ventricle is a heterogeneous congenital defect that encompasses a variety of anatomic aberrations and physiologic consequences. We describe the unusual cardiovascular magnetic resonance anatomic findings and sequelae of a 44-year-old man who underwent biventricular repair of double outlet right ventricle, subpulmonary type, which included tunneling of the ventricular septal defect to the pulmonary artery, right ventricular-to-pulmonary artery conduit and Damus-Kaye-Stansel procedure.