Echocardiography vs cardiac magnetic resonance imaging assessment of the systemic right ventricle for patients with d-transposition of the great arteries status post atrial switch.

OBJECTIVE: Patients with Dextro-transposition of the great arteries status post atrial switch (dTGA s/p atrial switch) are "at-risk" for systemic right ventricular (RV) dysfunction. Due to complex RV geometry, echocardiography (Echo) does not allow accurate determination of ejection fraction (EF), but cardiac magnetic resonance imaging (CMR) allows quantitative right ventricular assessment. Measures of ventricular deformation may be precursors to global ventricular dysfunction. The primary aim of this study was to characterize imaging and clinical findings for adult patients with dTGA s/p atrial switch. DESIGN: This was a retrospective cohort study of patients with dTGA s/p atrial switch operation (February 1966 to August 1988) with CMR performed at Children's Hospital of Wisconsin (from September 2005 to May 2015). Eligible patients had clinic visit, Echo, and exercise stress test within 1 year of CMR. RESULTS: This study enrolled twenty-seven patients (16 males, 11 females) with dTGA s/p atrial switch (18 with Mustard operation and 9 with Senning operation; median age 30 years; 74% New York Heart Association class 1 and 26% class 2). Seventy-four percentage had normal RV systolic function (RV EF >45% by CMR). No correlation was observed between Echo strain data and clinical status (EF, exercise endurance, VO2 max, or New York Heart Association class). Cardiac magnetic resonance imaging RV global circumferential strain GCS and RV EF had moderate negative correlation (r = -0.65, P < .001). Global circumferential strain was significantly different for those with RV EF above and below 45%, while global peak longitudinal strain (GLS) was not. Patients had reduced CMR myocardial strain values compared with healthy controls. CONCLUSIONS: Reduced RV CMR GCS (for those with RV EF <45%) suggests that CMR evaluation may enhance early detection of detrimental changes in the systemic RV myocardium.

Human genotyping and an experimental model reveal NPR-C as a possible contributor to morbidity in coarctation of the aorta.

Coarctation of the aorta (CoA) is a common congenital cardiovascular (CV) defect characterized by a stenosis of the descending thoracic aorta. Treatment exists, but many patients develop hypertension (HTN). Identifying the cause of HTN is challenging because of patient variability (e.g., age, follow-up duration, severity) and concurrent CV abnormalities. Our objective was to conduct RNA sequencing of aortic tissue from humans with CoA to identify a candidate gene for mechanistic studies of arterial dysfunction in a rabbit model of CoA devoid of the variability seen with humans. We present the first known evidence of natriuretic peptide receptor C (NPR-C; aka NPR3) downregulation in human aortic sections subjected to high blood pressure (BP) from CoA versus normal BP regions (validated to PCR). These changes in NPR-C, a gene associated with BP and proliferation, were replicated in the rabbit model of CoA. Artery segments from this model were used with human aortic endothelial cells to reveal the functional relevance of altered NPR-C activity. Results showed decreased intracellular calcium ([Ca2+]i) activity to C-type natriuretic peptide (CNP). Normal relaxation induced by CNP and atrial natriuretic peptide was impaired for aortic segments exposed to elevated BP from CoA. Inhibition of NPR-C (M372049) also impaired aortic relaxation and [Ca2+]i activity. Genotyping of NPR-C variants predicted to be damaging revealed that rs146301345 was enriched in our CoA patients, but sample size limited association with HTN. These results may ultimately be used to tailor treatment for CoA based on mechanical stimuli, genotyping, and/or changes in arterial function.

Assessment of quality of life in pediatric patients with pulmonary hypertension.

The aim of this study is to evaluate quality of life in four domains (physical, emotional, social, and school) in pediatric patients with pulmonary hypertension (PH) using a validated survey (PedsQL). This is a prospective cohort study of pediatric patients aged 2-18 years with PH. Parents of all children and patients aged 8-18 years with appropriate developmental capacity completed the PedsQL survey in the clinic. Results were compared with published norms for pediatric patients, those with congenital heart disease (CHD) and cancer. Thirty-three children were enrolled yielding 32 parent and 18 patient self-reports: seven patients were aged 2-4 years; three were aged 5-7 years; 11 were aged 8-12 years, and 12 were aged 13-18 years. Twenty-one patients were classified as World Health Organization (WHO) Group I pulmonary arterial hypertension (PAH), 11 WHO Group III PH due to lung disease, and one WHO Group V with segmental PH. Thirteen patients were NYHA functional class (FC) 1, 12 were FC 2, eight were FC 3, and none were FC 4. The PH cohort had significantly lower scores than healthy children in all domains on both parent and self-report. The PH cohort also had significantly lower scores than patients with CHD (parent report: total, physical, social, school; patient self-report: total, physical, school) and cancer (parent report: school; patient self-report: physical, school). Close to 50% of participants reported at risk scores in each domain. The quality of life in pediatric PH patients assessed by PedsQL revealed functional impairment in multiple domains. Administration of the PedsQL during outpatient encounters may provide an easy, reproducible method to assess quality of life and direct referral for interventional services.

The Impact of Cardiac Motion on Aortic Valve Flow Used in Computational Simulations of the Thoracic Aorta.

Advancements in image-based computational modeling are producing increasingly more realistic representations of vasculature and hemodynamics, but so far have not compensated for cardiac motion when imposing inflow boundary conditions. The effect of cardiac motion on aortic flow is important when assessing sequelae in this region including coarctation of the aorta (CoA) or regurgitant fraction. The objective of this investigation was to develop a method to assess and correct for the influence of cardiac motion on blood flow measurements through the aortic valve (AoV) and to determine its impact on patient-specific local hemodynamics quantified by computational fluid dynamics (CFD). A motion-compensated inflow waveform was imposed into the CFD model of a patient with repaired CoA that accounted for the distance traveled by the basal plane during the cardiac cycle. Time-averaged wall shear stress (TAWSS) and turbulent kinetic energy (TKE) values were compared with CFD results of the same patient using the original waveform. Cardiac motion resulted in underestimation of flow during systole and overestimation during diastole. Influences of inflow waveforms on TAWSS were greatest along the outer wall of the ascending aorta (AscAo) (∼30 dyn/cm2). Differences in TAWSS were more pronounced than those from the model creation or mesh dependence aspects of CFD. TKE was slightly higher for the motion-compensated waveform throughout the aortic arch. These results suggest that accounting for cardiac motion when quantifying blood flow through the AoV can lead to different conclusions for hemodynamic indices, which may be important if these results are ultimately used to predict patient outcomes.

Including aortic valve morphology in computational fluid dynamics simulations: initial findings and application to aortic coarctation.

Computational fluid dynamics (CFD) simulations quantifying thoracic aortic flow patterns have not included disturbances from the aortic valve (AoV). 80% of patients with aortic coarctation (CoA) have a bicuspid aortic valve (BAV) which may cause adverse flow patterns contributing to morbidity. Our objectives were to develop a method to account for the AoV in CFD simulations, and quantify its impact on local hemodynamics. The method developed facilitates segmentation of the AoV, spatiotemporal interpolation of segments, and anatomic positioning of segments at the CFD model inlet. The AoV was included in CFD model examples of a normal (tricuspid AoV) and a post-surgical CoA patient (BAV). Velocity, turbulent kinetic energy (TKE), time-averaged wall shear stress (TAWSS), and oscillatory shear index (OSI) results were compared to equivalent simulations using a plug inlet profile. The plug inlet greatly underestimated TKE for both examples. TAWSS differences extended throughout the thoracic aorta for the CoA BAV, but were limited to the arch for the normal example. OSI differences existed mainly in the ascending aorta for both cases. The impact of AoV can now be included with CFD simulations to identify regions of deleterious hemodynamics thereby advancing simulations of the thoracic aorta one step closer to reality.

Caregiving motivations and developmentally prompted transition for mothers of prematurely born infants.

Parenting transition is a process prompted by infant developmental changes and may be defined by motivations for caregiving and the goals they indicate. Qualitative exploration of neonatal and 1-year feeding experience of 22 mothers of very-low-birth-weight infants revealed 3 types of caregiving-related motivations-nurturing, relating, and shaping quality of life. The clusters of motivations differed between ages and across mothers, suggesting transition in parenting reflective of both infant development and maternal goals. Study with a larger sample is needed to examine change in caregiving motivations and their function in characterizing parenting transitions.

Aortic valve replacement in neonates and infants: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

OBJECTIVE: We sought to describe early outcomes of aortic valve replacement in neonates and infants across a large multicenter cohort. METHODS: Neonates and infants in the Society of Thoracic Surgeons Congenital Heart Surgery Database undergoing nontruncal aortic valve replacement with the Ross-Konno procedure, Ross procedure, or homograft replacement from 2000 to 2009 were included. Preoperative characteristics, operative data, and early outcomes are described. RESULTS: A total of 160 patients (43 neonates, 117 infants) from 47 centers were included. Society of Thoracic Surgeons-defined preoperative risk factors were present in 76 patients (48%) and were most prevalent in neonates (67%) and patients undergoing homograft aortic valve replacement (93%). Concomitant arch repair or mitral valve surgery was performed in 30 patients (19%) and 19 patients (12%), respectively. Postoperative mechanical circulatory support was used in 17 patients (11%). Overall in-hospital mortality was 18% and was highest for neonates (28%) and patients undergoing homograft aortic valve replacement (40%). Concomitant arch repair was associated with higher in-hospital mortality (33% vs 15%, P = .02), whereas concurrent mitral valve surgery was not (21% vs 18%, P = .73). Postoperative mechanical circulatory support was also associated with increased in-hospital mortality (65% vs 13%, P < .0001). CONCLUSIONS: Neonates and infants undergoing aortic valve replacement are a high-risk group, with hospital mortality comparable with some of the highest risk procedures in this age group. The requirement for arch repair or postoperative mechanical circulatory support was associated with an increased risk of death in this cohort.

Internal working models of parenting: motivations of parents of infants with a congenital heart defect.

Drawing on attachment-caregiving theory, we interviewed parents of 24 infants with a complex congenital heart defect (CCHD) about parenting motivations through the first year. Using directed content analysis, 8 categories of motivations, focused either on the baby, the parent-infant relationship, family, self, or tasks were identified at 1, 4 or 6, and 12 months. A matrix of motivations by parent showed family and infant age variations. Motivations illustrated for 5 parents at 1 month suggest that specification of expectations and intentions and clustering of motivations mentioned by a parent would advance study of linkages of parenting internal working models with parenting action.

A continuing education program for hospital and public health nurses to guide families of very low birth-weight infants in caregiving.

Nurses have a critical role in family development of competencies for giving care to very low birth-weight infants, However, current information-based methods of preparation may be inadequate for competency development. This article describes a continuing education program designed to strengthen nurses' support of families in developing caregiving competencies through processes of guided participation. Program effectiveness was explored with: (1) a survey of participant and non-participant nurse satisfaction with family work and with organizational resources and practices; (2) a description of relationship and caregiving competencies for mothers who had and had not received guided participation; and (3) a review of mothers' reports of their experience either with or without guided participation. Organizational arrangements and mechanisms for establishing guided participation practice within an agency, including ongoing reflective supervision sessions, peer collaboration, and documentation of competency development, are discussed.

Guiding mothers' management of health problems of very low birth-weight infants.

OBJECTIVE: Explore the feasibility, usefulness, and outcomes of a pilot program to support mothers in developing competencies for managing health problems of their very low birth-weight (VLBW) infants in partnership with the primary care clinician (PCC). DESIGN: In a randomized study, mothers who received guided participation (GP) and printed guidelines for managing VLBW infant health problems were compared with mothers who received only the guidelines and standard care (GL group). SAMPLE: All mothers (GP = 20; GL = 11) were at least 18 years old and English speaking. Infants were all VLBW (< or =1,500 g). INTERVENTION: GP began during the infant's neonatal intensive care unit stay and continued with public health nurses (PHNs) and a family service clinician through the infant's first 4 postterm months. MEASUREMENTS: Intervention feasibility and usefulness were assessed with maternal and clinician feedback. Outcomes included maternal and clinician appraisal of mothers' use of clinical resources and mothers' perceptions of primary-care quality and the family-PCC relationship. RESULTS: Intervention feasibility and usefulness were supported. GP and GL groups did not differ significantly on outcomes. CONCLUSIONS: Findings indicate a longer intervention period, GP organized by infant problem episodes, and enhancement of the PHN role in the context of interdisciplinary and interagency collaboration.