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Pulmonary vascular volume ratio measured by cardiac computed tomography in children and young adults with congenital heart disease: comparison with lung perfusion scintigraphy.

Pediatr Radiol. 2017 Nov;47(12):1580-1587

Authors: Goo HW, Park SH

BACKGROUND: Lung perfusion scintigraphy is regarded as the gold standard for evaluating differential lung perfusion ratio in congenital heart disease.
OBJECTIVE: To compare cardiac CT with lung perfusion scintigraphy for estimated pulmonary vascular volume ratio in patients with congenital heart disease.
MATERIALS AND METHODS: We included 52 children and young adults (median age 4 years, range 2 months to 28 years; 31 males) with congenital heart disease who underwent cardiac CT and lung perfusion scintigraphy without an interim surgical or transcatheter intervention and within 1 year. We calculated the right and left pulmonary vascular volumes using threshold-based CT volumetry. Then we compared right pulmonary vascular volume percentages at cardiac CT with right lung perfusion percentages at lung perfusion scintigraphy by using paired t-test and Bland-Altman analysis.
RESULTS: The right pulmonary vascular volume percentages at cardiac CT (66.3 ± 14.0%) were significantly smaller than the right lung perfusion percentages at lung perfusion scintigraphy (69.1 ± 15.0%; P=0.001). Bland-Altman analysis showed a mean difference of -2.8 ± 5.8% and 95% limits of agreement (-14.1%, 8.5%) between these two variables.
CONCLUSION: Cardiac CT, in a single examination, can offer pulmonary vascular volume ratio in addition to pulmonary artery anatomy essential for evaluating peripheral pulmonary artery stenosis in patients with congenital heart disease. However there is a wide range of agreement between cardiac CT and lung perfusion scintigraphy.

PMID: 28646273 [PubMed - indexed for MEDLINE]

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Is having a job a protective factor? Employment status and state of medical care as subjectively perceived by adults with CHD in Germany.

Cardiol Young. 2017 Aug;27(6):1110-1117

Authors: Helm PC, Sticker EJ, Keuchen R, Koerten MA, Diller GP, Tutarel O, Bauer UMM

BACKGROUND: Most patients born with CHD nowadays reach adulthood, and thus quality of life, life situation, and state of medical care aspects are gaining importance in the current era. The present study aimed to investigate whether patients' assessment depends on their means of occupation. The findings are expected to be helpful in optimising care and for developing individual treatment plans.
METHODS: The present study was based on an online survey conducted in cooperation with patient organisations. Participants were recruited from the database of the German National Register for Congenital Heart Defects. In total, 1828 individuals (777 males, 1051 females) took part. Participants were asked to rate aspects such their state of health on a six-tier scale (1=worst specification). Response behaviour was measured against the background of occupational details.
RESULTS: Training for or pursuing a profession was found to be significantly associated with participants' rating of five of the six examined aspects (p<0.05). Sex seemed to play an important part in four of the six aspects.
CONCLUSIONS: An optimal treatment plan for adults with CHD should always consider aspects such as sex and employment status. To work out such an optimal and individual treatment plan for each adult CHD patient, an objective tool to measure patients' actual CHD-specific knowledge precluding socially accepted response bias would be very useful.

PMID: 27830637 [PubMed - indexed for MEDLINE]

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Congenital coronary artery anomalies in adults: review of 111 cases from a single-centre experience.

Cardiol Young. 2017 Aug;27(6):1041-1050

Authors: Çanga Y, Güvenç TS, Karataş MB, Çalık AN, Onuk T, Tanık VO, Güngör B, Bolca O

BACKGROUND: Coronary artery anomalies are a heterogeneous group of congenital disorders presenting with a wide spectrum of symptoms, ranging from vague chest pain to sudden cardiac death. Despite available data, there is no consensus about the classification, nomenclature, and outcomes of coronary anomalies in the normally connected heart. In this study, we aimed to investigate clinical and angiographic characteristics of coronary arterial anomalies, as well as the frequency of atherosclerotic involvement in anomalous coronaries, diagnosed at a tertiary referral centre.
METHODS: We retrospectively reviewed coronary angiograms performed between 2011 and 2015 for the presence of a coronary anomaly. A total of 111 patients with a final diagnosis of coronary anomaly were included in the study group. We also recruited 110 age- and sex-matched patients who underwent coronary angiography because of symptomatic coronary artery disease as controls.
RESULTS: Among 36,893 coronary angiograms, 111 (0.30%) major coronary anomalies were found. Compared with controls, the prevalence of significant atherosclerotic coronary disease was lower in patients with coronary anomalies and stable symptoms (p=0.02); however, the prevalence of significant coronary atherosclerosis was similar among patients admitted with unstable angina or myocardial infarction (p>0.05). Compared with controls, patients with an anomalous left anterior descending coronary artery had significantly less atherosclerotic involvement than those in whom the left anterior descending artery was not anomalous (p=0.005).
CONCLUSIONS: Although coronary artery anomalies are cited as a cause for myocardial ischaemia, atherosclerotic coronary artery disease is also frequent and may offer an alternative explanation to ischaemic symptoms. No predisposition to accelerated atherosclerosis was found, however, and atherosclerotic involvement was less frequent in some anomalous vessels.

PMID: 27842619 [PubMed - indexed for MEDLINE]

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Transthoracic echocardiography study of congenitally corrected transposition of the great arteries.

J Clin Ultrasound. 2017 Jul 08;45(6):375-379

Authors: Singh A, Singh G, Dhaliwal TS, Singh M

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart defect, occurring in only 0.5% of patients exhibiting congenital heart defects. Because it is not seen in a routine transthoracic echocardiography (TTE) examination, CCTGA may be missed. The echocardiography diagnosis of CCTGA is difficult and not well explained in most textbooks. The present report provides key sonographic images that contribute to the diagnosis. In the present case, the patient had no prior history of heart disease. TTE revealed that the patient had CCTGA and early tricuspid regurgitation with right ventricle enlargement and hypertrophy. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:375-379, 2017.

PMID: 28182287 [PubMed - indexed for MEDLINE]

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Effect of iloprost on biomarkers in patients with congenital heart disease-pulmonary arterial hypertension.

Clin Exp Pharmacol Physiol. 2017 Sep;44(9):914-923

Authors: Li XY, Zheng Y, Long Y, Zhang X, Zhang L, Tian D, Zhou D, Lv QZ

Some biomarkers play important roles in the endothelial dysfunction of patients with pulmonary arterial hypertension (PAH), including nitric oxide (NO), endothelin-1 (ET-1), asymmetric dimethylarginine (ADMA), galectin-3 (Gal-3), B-type natriuretic peptide (BNP), and uric acid (UA). However, studies on these biomarkers in pulmonary artery blood in congenital heart disease-PAH (CHD-PAH) and the effect of iloprost on the regulation of biomarkers are lacking. This study investigated potential CHD-PAH biomarkers and their association with the severity of disease. The effect of iloprost on the regulation of these biomarkers was also studied. A total of 31 patients with CHD-PAH were enrolled. Seven with positive effects of iloprost (the average reduction in mPAP 11.13±1.73 mm Hg) and 19 with negative effects of iloprost (the average reduction in mPAP 4.21±4.87 mm Hg; iloprost positive group [IPG] vs iloprost negative group [ING], P<.01) and five age-matched controls were studied. The pulmonary artery blood sample was collected before and after inhaling iloprost, and the plasma concentrations of Gal-3, ADMA, ET-1, and NO were measured. A significant positive linear relationship was observed between mPAP and plasma ET-1, BNP, ADMA, and UA levels in all patients with CHD-PAH. ET-1, ADMA, BNP, and UA levels had a significant linear relationship with mean pulmonary arterial pressure, which could be used to predict the severity of CHD-PAH. ET-1 might be a potential biomarker to pre-evaluate the effect of iloprost on CHD-PAH. Iloprost could affect the expression of Gal-3 and, therefore, the process of fibrosis could be influenced by iloprost.

PMID: 28608969 [PubMed - indexed for MEDLINE]

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Prevalence of HCV Infection in Adults with Congenital Heart Disease and Treatment with Direct Antiviral Agents.

South Med J. 2018 03;111(3):137-141

Authors: Gade AR, Patel M, West DR, Abrams GA

OBJECTIVES: Hepatitis C virus (HCV) infection affects >3% of the US population, which over time can lead to cirrhosis and hepatocellular carcinoma. The lack of a reliable screening method for HCV before 1992 resulted in a higher prevalence of the virus in adults with congenital heart disease who underwent corrective surgery that required blood transfusions. Direct-acting antiviral agents such as sofosbuvir/ledipasvir have significantly increased the efficacy of HCV therapy, although use of these medications in adults with congenital heart disease has not been described.
METHODS: Ours was a retrospective study of 188 adults with congenital heart conditions who had cardiac surgery before 1992. These patients were screened for HCV using HCV antibody followed by HCV RNA if the screening test was positive.
RESULTS: Of the 188 adults, 116 (43% male patients, 24-70 years) were screened for the HCV antibody, demonstrating that 104 individuals were negative and 12 subjects were positive for the virus. Subsequently, further testing for the presence of HCV demonstrated 11 of 12 were infected, with an overall prevalence of 9.5%. Five individuals chose to be treated with sofosbuvir/ledipasvir and 5 of 5 have successfully cleared the virus and are considered cured.
CONCLUSIONS: Adults with congenital heart disease who underwent cardiac surgery before 1992 warrant being screened for HCV, and, if testing positive, may be considered for therapy using direct-acting antiviral agents with close monitoring for cardiac complications.

PMID: 29505646 [PubMed - indexed for MEDLINE]

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Abdominal Imaging Surveillance in Adult Patients After Fontan Procedure: Risk of Chronic Liver Disease and Hepatocellular Carcinoma.

Curr Probl Diagn Radiol. 2018 Jan - Feb;47(1):19-22

Authors: Nandwana SB, Olaiya B, Cox K, Sahu A, Mittal P

PURPOSE: To assess the prevalence of chronic liver disease (CLD) and hepatocellular carcinoma (HCC) in adult patients who had surveillance imaging after Fontan procedure.
METHODS: Institutional review board-approved, Health Insurance Portability and Accountability Act-compliant retrospective study evaluated electronic medical records including radiology reports and clinical notes for adult patients after Fontan procedure between January 1993 and January 2016. Abdominal ultrasound, computed tomography, and magnetic resonance imaging reports were reviewed for changes of CLD and HCC. Existence of concomitant viral hepatitis was also recorded.
RESULTS: A total of 145 patients (male: 78 and female: 67) had surveillance imaging after Fontan procedure. In all, 78% (113/145) had ongoing imaging surveillance (median follow-up 3.05 years, IQR: 0.75-5.3 years); 19% (21/113) had an initial normal study and remained normal throughout follow-up; 19% (21/113) had an initial normal study with subsequent imaging reporting changes of CLD; and 62% (71/113) had existing changes of CLD on initial study. HCC was identified in 5 patients (median 22 years post-Fontan, IQR: 10-29 years), 4 of which had a normal initial study. Only 1 patient with HCC had concomitant viral hepatitis C infection.
CONCLUSION: Radiologists should be aware that CLD is exceedingly common in post-Fontan cardiac physiology, and surveillance imaging is warranted given the risk of HCC.

PMID: 28602501 [PubMed - indexed for MEDLINE]

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Experimental Study of Anisotropic Stress/Strain Relationships of Aortic and Pulmonary Artery Homografts and Synthetic Vascular Grafts.

J Biomech Eng. 2017 Oct 01;139(10):

Authors: Jia Y, Qiao Y, Ricardo Argueta-Morales I, Maung A, Norfleet J, Bai Y, Divo E, Kassab AJ, DeCampli WM

Homografts and synthetic grafts are used in surgery for congenital heart disease (CHD). Determining these materials' mechanical properties will aid in understanding tissue behavior when subjected to abnormal CHD hemodynamics. Homograft tissue samples from anterior/posterior aspects, of ascending/descending aorta (AA, DA), innominate artery (IA), left subclavian artery (LScA), left common carotid artery (LCCA), main/left/right pulmonary artery (MPA, LPA, RPA), and synthetic vascular grafts, were obtained in three orientations: circumferential, diagonal (45 deg relative to circumferential direction), and longitudinal. Samples were subjected to uniaxial tensile testing (UTT). True strain-Cauchy stress curves were individually fitted for each orientation to calibrate Fung model. Then, they were used to calibrate anisotropic Holzapfel-Gasser model (R2 > 0.95). Most samples demonstrated a nonlinear hyperelastic strain-stress response to UTT. Stiffness (measured by tangent modulus at different strains) in all orientations were compared and shown as contour plots. For each vessel segment at all strain levels, stiffness was not significantly different among aspects and orientations. For synthetic grafts, stiffness was significantly different among orientations (p < 0.042). Aorta is significantly stiffer than pulmonary artery at 10% strain, comparing all orientations, aspects, and regions (p = 0.0001). Synthetic grafts are significantly stiffer than aortic and pulmonary homografts at all strain levels (p < 0.046). Aortic, pulmonary artery, and synthetic grafts exhibit hyperelastic biomechanical behavior with anisotropic effect. Differences in mechanical properties among vascular grafts may affect native tissue behavior and ventricular/arterial mechanical coupling, and increase the risk of deformation due to abnormal CHD hemodynamics.

PMID: 28753691 [PubMed - indexed for MEDLINE]

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[Long-term outcome and prognostic factors in pregnant women with pulmonary arterial hypertension associated with congenital heart disease].

Zhonghua Nei Ke Za Zhi. 2017 Nov 01;56(11):827-832

Authors: Ou QT, Lu JK, Zhang J, Chen Y, Li Q, Zhang JL

Objective: To investigate the perinatal outcome, risk factors and long-term outcome of pregnancy complicated with pulmonary arterial hypertension(PAH) and congenital heart diseases (CHD). Methods: Clinical data of 110 pregnant women who were diagnosed as PAH-CHD were retrospectively analyzed in the Department of Obstetrics and Gynecology and Surgical Intensive Care Unit at Beijing Anzhen Hospital from 2004 to 2013. The survival and treatment status were followed up. Results: 110 subjects consisted of 11 mild PAH, 33 moderate and 66 severe ones. The incidences of deterioration in New York Heart Association (NYHA) classes (≥2) during pregnancy, respiratory failure, pulmonary hypertension crisis and arrhythmia were 25.5% (28/110), 7.3% (8/110), 10.0% (11/110), 10.0% (11/110) respectively. Among them, the difference of deterioration in NYHA classes (≥2) during pregnancy among the three groups was statistically significant. A total of 8 (7.3%) maternal deaths occurred during hospitalization, all of whom were severe PAH cases. Multivariate analysis showed that pulmonary artery systolic pressure was a risk factor of perioperative death (OR=1.042, P=0.005). There were 55 cases (50.0%) of term delivery, and 35 cases (31.8%) of iatrogenic abortion. The proportion of term delivery in the severe PAH group was significantly lower. The proportion of iatrogenic abortion and small for gestational age infant (SGA) were higher in severe group. The incidence of neonatal malformations was 8.0% (6/75). The follow-up rate was 61.8% (63/102). Sudden death was reported in a parturient a few days after discharge. The remaining 62 patients survived during follow-up, while 53 patients (85.5%) were functional class (FC) Ⅰ-Ⅱ, 9 (14.5%) were FC Ⅲ-Ⅳ at follow-up. The cardiac function deterioration during pregnancy was not significantly correlated with long-term deterioration (P=0.767). Conclusions: Perinatal mortality and the incidence of maternal and fetal adverse events were high in pregnancy with PAH-CHD. Pulmonary artery systolic pressure is a major risk factor for perioperative mortality in pregnant women. PAH-CHD woman had good overall outcome after puerperium.

PMID: 29136712 [PubMed - indexed for MEDLINE]

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Exploring the relationship between disease-related knowledge and health risk behaviours in young people with congenital heart disease.

Eur J Cardiovasc Nurs. 2016 Jun;15(4):231-40

Authors: Janssens A, Goossens E, Luyckx K, Budts W, Gewillig M, Moons P, i-DETACH investigators

BACKGROUND: In order to prevent cardiac complications, young people with congenital heart disease (CHD) should conduct heart-healthy behaviours. Therefore, they are assumed to have a good understanding of their disease. However, empirical data on the relationship between disease-related knowledge and health behaviours in this population is lacking.
AIMS: This study aimed (a) to describe the health risk behaviours of young people with CHD; (b) to describe their level of disease-related knowledge; and (c) to explore the relationship between the level of disease-related knowledge and health risk behaviours.
METHODS: Data were collected in 429 young people with CHD. Health risk behaviours were assessed using the Health Behaviour Scale for CHD and the Baecke questionnaire. Disease-related knowledge was evaluated using the Leuven Knowledge Questionnaire for CHD. An overall health behaviour risk score, a total knowledge score and eight thematic knowledge subscale scores were calculated. Eight relationships between knowledge and health risk behaviours were tested.
RESULTS: Patients presented moderate to good overall health behaviour. Disease-related knowledge was found to be relatively poor. Furthermore, better understanding of endocarditis was significantly associated with the performance of annual dental visits.
CONCLUSIONS: This study was the first to explore the relationship between disease-related knowledge and health risk behaviours in young people with CHD. Little evidence, however, was found to support this relationship. Additional studies are needed to evaluate the effectiveness of other strategies altering the health-compromising behaviours of these patients.

PMID: 25550312 [PubMed - indexed for MEDLINE]