Initial validation of a healthcare needs scale for young people with congenital heart disease.

AIM: To validate the initial psychometric properties of a Healthcare Needs Scale for Youth with Congenital Heart Disease. BACKGROUND: As the number of patients with congenital heart disease surviving to adulthood increases, the transitional healthcare needs for adolescents and young adults with congenital heart disease require investigation. However, few tools comprehensively identify the healthcare needs of youth with congenital heart disease. DESIGN: A cross-sectional study was employed to examine the psychometric properties of the Healthcare Needs Scale for Youth with Congenital Heart Disease. METHODS: The sample consisted of 500 patients with congenital heart disease, aged 15-24 years, from paediatric cardiology departments and covered the period from March-August 2015. The patients completed the 25-item Healthcare Needs Scale for Youth with Congenital Heart Disease, the questionnaire on health needs for adolescents and the WHO Quality of Life-BREF. Reliability and construct, concurrent, predictive and known-group validity were examined. RESULTS: The Healthcare Needs Scale for Youth with Congenital Heart Disease includes three dimensions, namely health management, health policy and individual and interpersonal relationships, which consist of 25 items. It demonstrated excellent internal consistency and sound construct, concurrent, predictive and known-group validity. CONCLUSION: The Healthcare Needs Scale for Youth with Congenital Heart Disease is a psychometrically robust measure of the healthcare needs of youth with congenital heart disease. It has the potential to provide nurses with a means to assess and identify the concerns of youth with congenital heart disease and to help them achieve a successful transition to adult care.

Between invisible defects and visible impact: the life experiences of adolescents and young adults with congenital heart disease.

AIM: To describe the life experiences of adolescents and young adults with congenital heart disease. BACKGROUND: Owing to medical advances, most children with congenital heart disease are expected to survive into adulthood. The transitional development from adolescence to adult is the critical period for fostering self-care. DESIGN: Descriptive phenomenological study. METHODS: Thirty-five patients of 15-24 years old with congenital heart disease were recruited from paediatric cardiology clinics by purposive sampling. They were individually interviewed between October 2012-February 2013 using a semi-structured interview guideline and joined adult congenital heart disease clinics at two medical centres in northern Taiwan. The data were analysed using descriptive phenomenological method developed by Giorgi. FINDINGS: The essence of the life experience of adolescents and young adults with congenital heart disease involves a dynamic process of moving between invisible defects and coexistence with the disease. Six themes emerged: (1) invisible defects: the existence of imperfect understanding; (2) conflict: interpersonal frustrations; (3) imbalance: the loss of self-balance; (4) suffering: increasing anxiety; (5) encounters: meeting needs; and (6) coexistence: positive coping strategies. CONCLUSION: As patients with congenital heart disease transition from adolescence into adulthood, they must learn about their disease, overcome frustration and anxiety and develop self-care strategies for coexisting with congenital heart disease. Results of this study may serve as clinical care guidelines for adolescents and young adults with congenital heart disease and give a reference for developing transitional intervention strategies.

Clinical implications of dextrocardia based on four visceroatrial situs studies.

BACKGROUND: Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. METHODS: We retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs. RESULTS: Dextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage. CONCLUSION: Our study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.

Health-promoting lifestyle and life satisfaction in full-time employed adults with congenital heart disease: grit as a mediator.

AIMS: Enhancing life satisfaction is vital for adults with congenital heart disease (ACHD). Life satisfaction potentially correlates with a health-promoting lifestyle and grit. However, the mediating role of grit-a trait marked by perseverance and long-term goal-oriented passion-between a health-promoting lifestyle and life satisfaction in ACHD remains unverified. This study aimed to examine the relationships between health-promoting lifestyle, grit, and life satisfaction in full-time employed ACHD and to confirm the mediating role of grit. METHODS AND RESULTS: A total of 181 full-time employed ACHD aged 20-59 years with patient-reported New York Heart Association class I-IV were recruited from two medical centers in northern Taiwan. Participants completed questionnaires, including the short version of the Chinese Health-Promoting Lifestyle Profile scale, Grit-10 scale, and Satisfaction with Life Scale between February and December 2022. Data analysis employed the Hayes PROCESS macro. Health-promoting lifestyle, grit, and life satisfaction were discovered to be positively correlated. Overall grit score was a partial mediator between a health-promoting lifestyle and life satisfaction, accounting for 20% of the total variation. Of the grit domains, perseverance served as a partial mediator between a health-promoting lifestyle and life satisfaction, accounting for 32% of the total variation, whereas passion had a nonsignificant mediation effect. CONCLUSION: A health-promoting lifestyle boosts ACHD individuals' life satisfaction through grit, particularly perseverance. Integrating grit into such a lifestyle could benefit from comprehending support systems and influential factors aiding grit development for enhanced life satisfaction.

The morphogenesis and associated anomalous pulmonary venous drainage in sinus venosus defect.

BACKGROUND: Sinus venosus defect (SVD) is an unusual type of interatrial communication (IAC) and is virtually always associated with partial anomalous pulmonary venous drainage (PAPVD) of the right pulmonary veins (RPV) to the superior vena cava (SVC) or right atrium (RA). However, its definite morphogenesis is still elusive, and diagnostic fallibility continues. METHODS: We conducted a retrospective review of the echocardiograms, cardiac catheterization data, computed tomographic findings, and surgical notes of 44 children with surgery-confirmed isolated SVD from 1977 to 2016. We investigated the location of the IAC and its boundaries within the atrial septum and its anatomic relationship with the adjacent structures, including the anomalously draining RPV. We also tried to explore any possible associated abnormalities which might be implicated in the morphogenesis of SVD. RESULTS: Two distinct types of IAC were defined. Forty patients had an IAC that was located posterosuperior to the intact fossa ovalis (superior type), and all were associated with PAPVD of the right upper and often the right middle pulmonary veins to the SVC. The remaining 4 patients had an IAC that was located posterior to the intact fossa ovalis (inferior type), and all were associated with PAPVD of all the RPV to the RA. Another consistently associated abnormality was a defect between the anomalously draining RPV posteriorly and the SVC or RA anteriorly. All these 44 patients underwent successful surgical baffling the associated PAPVD via the IAC into the left atrium. CONCLUSION: A defect between the RPV posteriorly and the SVC or RA anteriorly will result in SVD, and an unusual type of IAC, and PAPVD of the RPV to the SVC or RA. The IAC is not a true atrial septal defect in the atrial septum proper, but it actually represents the left atrial orifice of the unroofed RPV.

The rare association of truncus arteriosus with a cervical double aortic arch presenting with left main bronchial compression.

Truncus arteriosus, a double aortic arch, and a cervical aortic arch are all rare cardiovascular anomalies. We experienced a unique female newborn with the rare combination of truncus arteriosus with a cervical double aortic arch, which probably resulted from abnormal persistence of the bilateral 2nd or 3rd rather than the 4th embryonic aortic arches and failure of regression of the right 8th somitic segment of the right dorsal aorta. She presented with respiratory distress soon after birth, which was initially attributed to the vascular ring and hypertensive pulmonary arteries. Our inability to relieve her respiratory compromise by surgical division of the vascular ring and main pulmonary artery banding prompted the diagnosis of left main bronchial compression caused by a posteriorly displaced dilated ascending aorta that compressed the right pulmonary artery and left main bronchus against the descending aorta. The patient then underwent successful left main bronchus stent implantation. We speculate the cervical double aortic arch is redundant in nature and is a loose ring that may not cause tracheal compression. Nevertheless, a posteriorly displaced dilated ascending aorta in patients with truncus arteriosus may compress the right pulmonary artery and the main bronchus on the side of the aortic arch against the descending aorta.

A positive perspective of knowledge, attitude, and practices for health-promoting behaviors of adolescents with congenital heart disease.

BACKGROUND: Health-promoting behaviors could serve as a major strategy to optimize long-term outcomes for adolescents with congenital heart disease. The associations assessed from a positive perspective of knowledge, attitudes, and practice model would potentially cultivate health-promoting behaviors during adolescence. AIM: The purpose of this study was to examine the relationships between disease knowledge, resilience, family functioning, and health-promoting behaviors in adolescents with congenital heart disease. METHODS: A total of 320 adolescents with congenital heart disease who were aged 12-18 years were recruited from pediatric cardiology outpatient departments, and participated in a cross-sectional survey. The participants completed the Leuven Knowledge Questionnaire for Congenital Heart Disease; Haase Adolescent Resilience in Illness Scale; Family Adaptability, Partnership, Growth, Affection, and Resolve; and Adolescent Health Promotion scales. The collected data were analyzed using descriptive statistics and three multiple regression models. RESULTS: Greater knowledge of prevention of complications and higher resilience had a more powerful effect in enhancing health-promoting behaviors. Having symptoms and moderate or severe family dysfunction were significantly more negatively predictive of health-promoting behaviors than not having symptoms and positive family function. The third model explained 40% of the variance in engaging in health-promoting behaviors among adolescents with congenital heart disease. CONCLUSION: The findings of this study provide new insights into the role of disease knowledge, resilience, and family functioning in the health-promoting behavior of adolescents with congenital heart disease. Continued efforts are required to plan family care programs that promote the acquisition of sufficient disease knowledge and the development of resilience for adolescents with congenital heart disease.

Healthcare needs of adolescents with congenital heart disease transitioning into adulthood: a Delphi survey of patients, parents, and healthcare providers.

BACKGROUND: The increasing survival of children with congenital heart disease (CHD) challenges healthcare systems regarding how to manage the many health needs of patients undergoing transitional care. A comprehensive understanding of the perspectives of patients, parents, and healthcare providers is required. OBJECTIVE: This study systematically identified the healthcare needs of adolescents with CHD transitioning into young adults by collecting the perspectives of patients, parents, and healthcare providers. METHODS: A sample of CHD patients ( n = 29), parents of children with CHD ( n = 29), and healthcare providers ( n = 16) completed the two-round Delphi study, and 64 healthcare needs were identified. The central tendency and the level of dispersion were computed in order to establish a consensus. RESULTS: A consensus was reached on 25 healthcare needs including health, family, individual, interpersonal interaction, and policy dimensions, which were classified as important with a moderate to high level of agreement by all three groups. The three groups were strongly agreed that "encouraging the patient to learn health self-management" and "cultivating a positive attitude toward the illness" were very important. The opinions of the three groups differed significantly on 12 needs ( p < 0.05) related to health, family, and policy dimensions. CONCLUSION: A consensus was reached on the needs that were identified as being potentially valid measures of the healthcare needs of adolescents with CHD transitioning into young adults. The identified needs can serve as the basis for establishing a transitional health passport and developing a clinical intervention for adolescents with CHD transitioning into young adults.

Effectiveness of Cognitive-behavioral Program on Pain and Fear in School-aged Children Undergoing Intravenous Placement.

PURPOSE: This study aimed to evaluate the effects of cognitive-behavioral program on pain and medical fear in hospitalized school-aged children receiving intravenous (IV) placement. METHODS: This study used an quasi-experimental design. Thirty-five participants were assigned to the experimental group and 33 to the control group in the acute internal medicine ward of a children's hospital. The cognitive-behavioral program entailed having the patients read an educational photo book about IV placement before the procedure and having them watch their favorite music video during the procedure. The outcome measures were numeric rating scales for pain intensity and fear during the procedure. RESULTS: After applying the cognitive-behavioral program, the mean scores on pain and fear decreased in the experimental group. However, the difference in pain intensity between these two groups was nonsignificant. The intensity of fear in the experimental group was significantly lower than that in the control group. CONCLUSION: In this study, the cognitive-behavioral program used with school-aged hospitalized children promoted less fear during IV placement. The results of this study can serve as a reference for empirical nursing care and as care guidance for clinical IV injections involving children.

Ischemic retinopathy and uveitis in a patient with tetralogy of Fallot.

PURPOSE: To describe ischemic retinopathy in a patient with tetralogy of Fallot. DESIGN: Interventional case report. TESTING: Clinical and imaging evaluation. MAIN OUTCOME MEASURES: Clinical, imaging, and laboratory findings in a patient with tetralogy of Fallot. RESULTS: A 20-year-old female patient with tetralogy of Fallot had progressive visual loss of 3 weeks duration. Bilateral examination revealed dilated, tortuous, conjunctival vessels; prominent anterior chamber reaction; iris neovascularization; posterior synechia; retinal vascular tortuosity in both eyes; and inferior exudative retinal detachment. Fluorescein angiography revealed delayed retinal and choroidal filling. The working diagnosis was ischemic retinopathy with uveitis. The patient was treated for 6 months with a high-dose oral corticosteroid combined with a topical corticosteroid, a topical mydriatic, and panretinal photocoagulation. Conjunctival, vascular congestion subsided with a decrease in anterior chamber reaction. The inferior, exudative retinal detachment resolved, and vision was restored. CONCLUSIONS: Retinal ischemic syndrome combined with uveitis can develop in patients with tetralogy of Fallot. Treatment can restore vision in such patients.

Diaphragmatic paralysis caused by malposition of chest tube placement after pediatric cardiac surgery.

Diaphragmatic paralysis is a recognized complication after pediatric cardiac surgery. It is universally acknowledged that direct phrenic nerve injury during surgery is the etiology. However, we experienced two unusual cases of diaphragmatic paralysis following malposition of chest tube placement after pediatric cardiac surgery. The malposition of too deeply placed chest tube with resultant phrenic nerve injury was presumably the underlying cause. One patient underwent successful diaphragmatic plication due to intractable respiratory distress. The other was asymptomatic. Our report highlights the previously unreported complication of chest tube-induced phrenic nerve injury following its malposition after pediatric cardiac surgery. Prompt recognition and correction of tube malposition or selection of a softer chest tube probably can ameliorate the problem.

Physical self-concept and its link to cardiopulmonary exercise tolerance among adolescents with mild congenital heart disease.

BACKGROUND: Due to medical advances, most children with congenital heart disease (CHD) are expected to survive into adulthood. Establishing adequate physical self-concept and cardiopulmonary tolerance during the adolescent period can primarily enhance overall well-being. AIM: The purpose of this study was to undertake a gender-specific evaluation of the domain of physical self-concept among adolescents with mild CHD, and to examine the relationships between physical self-concept and cardiopulmonary exercise tolerance among adolescents with mild CHD. METHODS: Four hundred and thirteen adolescents 12-20 years of age, whose cardiologists had not recommended any limitation of exercise, completed Physical Self-Description Questionnaires and three-minute step tests in two outpatient cardiology departments. RESULTS: The male participants had significantly greater scores in measures of overall physical self-concept, competence in sports, physical appearance, body fat, physical activity, endurance, and strength than did the female participants. More than 80% of the participants had at least an average cardiopulmonary exercise tolerance index. The perception of not being 'too fat' and being more physically active were significant correlates of better cardiopulmonary exercise tolerance for adolescents with mild CHD. CONCLUSIONS: The results provided evidence for gender-specific evaluation of domains of physical self-concept among adolescents with mild CHD. The three-minute step test to measure cardiopulmonary exercise tolerance in adolescents with mild CHD may be an appropriate objective measure for use in future research. Continued efforts are needed in early intervention to promote cardiopulmonary exercise tolerance.

Natural history and risk stratification of discrete subaortic stenosis in children: an echocardiographic study.

BACKGROUND AND PURPOSE: Discrete subaortic stenosis (DSS) is considered an acquired cardiac defect. However, its clinical course and pathogenesis have not been well defined. This study used echocardiography to investigate the natural history of DSS and identify the possible anatomic abnormalities leading to its development. METHODS: We reviewed the medical records of 12 children with a diagnosis of DSS between 1988 and 2002. Data on the age at initial diagnosis of DSS was collected and the sequential changes of left ventricular outflow tract obstruction (LVOTO) and aortic regurgitation (AR) were analyzed by serial echocardiographic studies. Patients were divided into 2 subgroups according to the latest or presurgical (in patients with operations for DSS) Doppler-derived peak instantaneous left ventricular outflow tract gradient (deltaP; cut-off point: deltaP 50 mm Hg, our institutional criterion of operation for DSS). The indexed mitral valve (MV)-aortic valve (AV) distance, aortoseptal angle (ASA), indexed DSS-AV distance, and whether the anterior MV leaflet was involved were also determined. The DSS patients were compared with an age- and lesion-matched control group (12 patients). RESULTS: The mean age at initial diagnosis of DSS was 4.9 +/- 3.7 years. Nine children had disease characterized by milder LVOTO (latest deltaP < 50 mm Hg) and slower progression of LVOTO (mean increase of deltaP, 7.1 +/- 4.4 mm Hg/year) and AR ( 50 mm Hg) and more rapid progression of LVOTO (mean increase of deltaP, 27.8 +/- 8.4 mm Hg/year) and AR (>/= grade II). Compared with the control group, the study group had a significantly longer indexed MV-AV distance and a steeper ASA. Compared with the patients with milder disease, patients with more severe disease had a significantly shorter indexed DSS-AV distance and more frequent involvement of the anterior MV leaflet. CONCLUSIONS: These data indicate that DSS may present with 2 distinct clinical courses. A shorter indexed DSS-AV distance and the involvement of the anterior MV leaflet might be predictive of more rapidly progressive DSS. Longer indexed MV-AV distance and steeper ASA might be useful to evaluate primary cardiac defects and thus to identify patients who are at risk for developing DSS.

Social-cognitive determinants of exercise behaviour among adolescents with mild congenital heart disease.

BACKGROUND: Nowadays, most adolescents with mild congenital heart disease (CHD) undergo medical or surgical correction in early childhood for their congenital anomalies. There is a need to examine the determinants of exercise behaviour among adolescents (CHD) who are able to exercise. AIMS: The aims of this study were to examine determinants of exercise among adolescents with mild CHD, including personal beliefs about exercise, interpersonal influences on exercise, and availability of physical environments for exercise, based on social-cognitive theory. METHOD: A repeated-measures study was conducted at three medical centres in Taiwan. All participants, 126 adolescents with CHD aged 12 to 18 years, had a cardiologist's recommendation of exercise with no limits or limits only on vigorous exercise. Self-administered questionnaires were completed during a summer vacation and again in the fall semester of school along with a 7-day exercise log. The data were analysed using structured equation modelling. RESULTS: The effect of interpersonal influences on total exercise and moderate-to-vigorous exercise was mediated by personal exercise beliefs. Peer influences had significant effects on moderate-to-vigorous exercise through the mediator of perceived exercise self-efficacy. There was no significant difference in the determinants of exercise behaviour during two periods. Personal exercise beliefs, particularly perceived exercise self-efficacy, played the most important role in determining the performance of moderate-to-vigorous exercise. CONCLUSIONS: The social-cognitive determinants may serve as a clinical reference for promoting exercise among adolescents with mild CHD.

Cardiac troponin I release after transcatheter atrial septal defect closure correlated with the ratio of the occluder size to body surface area.

BACKGROUND: Cardiac troponin I (cTnI) is a very specific and sensitive marker of myocardial injury. The degree of myocardial injury associated with transcatheter atrial septal defect (ASD) closure in children is unknown. METHODS: In a longitudinal study on children with ASD, cTnI serum concentrations were measured after transcatheter ASD closure. Implantation success, complications, and latest patient follow-up were described. RESULTS: We inserted 73 Amplatzer septal occluders in 73 patients. Of these, we excluded two patients in whom the device embolized to the right ventricle the day after deployment. The median age was 4.5 years (range, 1.1-18.0) with 20 boys and 51 girls (male:female ratio, 1:2.6). The mean ASD size was 17 ± 7 mm, and device size ranged from 7 mm to 38 mm. The Amplatzer size/body surface area ratio was validated by demonstrating positive correlation with cTnI elevation. In children who had a successful attempt, 30 samples had a cTnI value higher than 1.0 µg/L l at 6 hours after procedure. Six patients had a significant release of cTnI greater than normal limits (mean level of 1.51 ± 0.26 µg/L). CONCLUSION: In our study, transcatheter ASD closure induced minor myocardial lesion, the extent of which depended on the ratio of the occluder size to body surface area (p<0.05) but not on the patient's weight or preprocedural left ventricular ejection fraction.

Management of patent ductus arteriosus in term or near-term neonates with respiratory distress.

BACKGROUND: Respiratory distress and patent ductus arteriosus (PDA) in neonates are mutually perpetuating. Contrary to the situation in premature infants, the recognition, clinical relevance and optimal management of PDA in full-term neonates are unclear. The present study aimed to identify PDA as a possible cause of respiratory distress in term and near-term neonates, and to examine the clinical responsiveness of PDA to different treatment modalities in mature-gestational-age neonates. METHODS: Patients with gestational ages of over 34 weeks were included in this retrospective chart review; they had PDA as the sole recognizable cause of respiratory distress and were free of all other diseases. Clinical responsiveness to different regimens, including conservative treatment, drug therapy with preload reduction and inotropic agent with or without the addition of indomethacin, and surgical intervention were analyzed. RESULTS: Forty-four neonates qualified for this study. Six received no treatment and their cardiorespiratory symptoms resolved within 1 week (regimen A). Symptoms in 11 neonates were relieved after use of diuretic and inotropic agents (regimen B). Twelve neonates became asymptomatic without further intervention after indomethacin treatment in addition to preload reduction and inotropes (regimen C). A total of 15 of the 44 infants underwent PDA ligation (regimen D) due to persistent heart failure following regimens B or C, but had speedy resolution of respiratory symptoms following surgery. There were significant differences in birth body weight and hemodynamic variation based on left atrium to aortic root dimensional ratio between the treatment (regimens B, C and D) and non-treatment (regimen A) groups (p < 0.05). CONCLUSION: PDA plays an important role in prolonging respiratory distress in term or near-term neonates. Although most infants respond to noninvasive medical treatment, surgical ligation during the neonatal period is warranted in certain mature infants. Surgical treatment should be considered in patients with smaller birth body weights and those with increased left atrium to aortic root dimensional ratios.