scholarly journals Pheochromocytoma and Paraganglioma in Cyanotic Congenital Heart Disease

2015 ◽  
Vol 100 (4) ◽  
pp. 1325-1334 ◽  
Author(s):  
Alexander R. Opotowsky ◽  
Lilamarie E. Moko ◽  
Jonathan Ginns ◽  
Marlon Rosenbaum ◽  
Matthias Greutmann ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Odds Ratio ◽  
Congenital Heart ◽  
Psychiatric Symptoms ◽  
Clinical Signs ◽  
Cyanotic Congenital Heart Disease ◽  
Genetic Syndromes ◽  
Cross Sectional ◽  
Increased Risk

Context: Aberrant cellular oxygen sensing is a leading theory for development of pheochromocytoma (PHEO) and paraganglioma (PGL). Objective: The objective of the study was to test the hypothesis that chronic hypoxia in patients with cyanotic congenital heart disease (CCHD) increases the risk for PHEO-PGL. Design/Setting/Participants: We investigated the association between CCHD and PHEO-PGL with two complementary studies: study 1) an international consortium was established to identify congenital heart disease (CHD) patients with a 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 noncyanotic CHD and those without CHD using multivariable logistic regression adjusted for age, sex, and genetic PHEO-PGL syndromes. Results: In 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 20 years (range 1–57 y). Cases were young at diagnosis (median 31.5 y, range 15–57 y) and 7 of 18 had multiple tumors (two bilateral PHEO; six multifocal or recurrent PGL), whereas 11 had single tumors (seven PHEO; four PGL). PGLs were abdominal (13 of 17) or head/neck (4 of 17). Cases displayed a noradrenergic biochemical phenotype similar to reported hypoxia-related PHEO-PGL genetic syndromes but without clinical signs of such syndromes. In study 2, hospitalized CCHD patients had an increased likelihood of PHEO-PGL (adjusted odds ratio 6.0, 95% confidence interval 2.6–13.7, P < .0001) compared with those without CHD; patients with noncyanotic CHD had no increased risk (odds ratio 0.9, P = .48). Conclusions: There is a strong link between CCHD and PHEO-PGL. Whether these rare diseases coassociate due to hypoxic stress, common genetic or developmental factors, or some combination requires further investigation.

scholarly journals Nutritional assessment and associated factors in children with congenital heart disease in Ethiopia.

2021 ◽  
Author(s):  
Temesgen Tsega Desta
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Associated Factors ◽  
Congenital Heart ◽  
Nutritional Assessment ◽  
Failure To Thrive ◽  
Cross Sectional ◽  
Care Givers ◽  
Increased Risk

ABSTRACT Infants and children with congenital heart disease exhibit a range of delays in weight gain and growth. In some instances, the delay can be relatively mild, whereas in other cases, cause the failure to thrive. OBJECTIVES To determine the nutritional status and associated factors of pediatric patients with congenital heart disease. MATERIAL AND METHODS A cross sectional analytical study was done over a period of 6months (Feb to Jul 2020). A total of 228 subjects with congenital heart disease that come to the cardiac center during the study period where included until the calculated sample size was attained. Data was collected from patient card and care givers of the children included in the study after obtaining their informed consent using data inquiry sheet. RESULTS A total of 228 children from age 3month to 17yrs. Most of the subjects had acyanotic heart disease accounting for 87.7%. The overall prevalence of wasting, underweight and stunting were 41.3%, 49.1% and 43% respectively. Among this children with congenital heart disease those with PAH were found more likely have wasting compared to those without PAH with an odds of 1.9 (95% CI: 1.0-3.4) and also greater chance of being stunted with an odds of 1.9 (95% CI: 1.0-3.4). children above 5years of age were 2.3 times more likely to be underweight. CONCLUSION Malnutrition is a major problem of patients with CHD. Pulmonary hypertension and older age are associated with increased risk of undernutrition. KEYWORDS: Acyanotic, cyanotic, Pulmonary hypertension, underweight, wasting and stunting.

Association Between Postoperative Dexmedetomidine Use and Arrhythmias in Infants After Cardiac Surgery

2019 ◽  
Vol 10 (4) ◽  
pp. 440-445 ◽  
Author(s):  
Laura A. Ortmann ◽  
Meera Keshary ◽  
Karl Stessy Bisselou ◽  
Shelby Kutty ◽  
Jeremy T. Affolter
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Odds Ratio ◽  
Retrospective Cohort ◽  
Congenital Heart ◽  
Atrioventricular Canal ◽  
Need For Treatment ◽  
Increased Risk ◽  
High Risk Infants ◽  
Complete Atrioventricular

Background: Dexmedetomidine has been suggested as an arrhythmia prophylactic agent after surgery for congenital heart disease due to its heart rate lowering effect, though studies are conflicting. We sought to study the effect of dexmedetomidine in infants that are at highest risk for arrhythmias. Methods: Retrospective cohort study of infants less than six months of age undergoing cardiopulmonary bypass for congenital heart disease. The arrhythmia incidence in the first 48 hours after surgery in infants receiving dexmedetomidine for sedation was compared to those that did not receive dexmedetomidine. Results: A total of 309 patients were included, 206 patients who did not receive dexmedetomidine and 103 patients who did. The incidence of tachyarrhythmias was similar between the non-DEX group and the DEX group (19% vs 15%, P = .34). When adjusted for baseline differences, the non-DEX group did not have an increased risk of postoperative tachyarrhythmias (odds ratio [OR]: 1.4, 95% confidence interval [CI]: 0.5-3.8). The non-DEX group had an increased need for treatment for arrhythmias (18% vs 8%, P = .012). The three lesions with baseline higher risk for arrhythmias (tetralogy of Fallot, transposition of the great arteries, and complete atrioventricular canal) had an increased incidence of tachyarrhythmias in the non-DEX group (34% vs 6%, P = .027). This risk was not significant in multivariate analysis (OR: 2.5, 95% CI: 0.4-15.5). Conclusions: High-risk infants had decreased incidence of tachyarrhythmias when receiving dexmedetomidine, though this was not significant after accounting for baseline differences between groups.

scholarly journals Multiple Crystal Cross-Sectional Echocardiography in the Diagnosis of Cyanotic Congenital Heart Disease

Circulation ◽  
1974 ◽  
Vol 50 (2) ◽  
pp. 230-238 ◽  
Author(s):  
DAVID J. SAHN ◽  
RICHARD TERRY ◽  
ROBERT O'ROURKE ◽  
GEORGE LEOPOLD ◽  
WILLIAM F. FRIEDMAN
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease ◽  
Cross Sectional

scholarly journals Parenting stress mediates the association between cyanotic congenital heart disease and internalising problems in children and adolescents

2019 ◽  
Vol 19 (4) ◽  
pp. 301-309 ◽  
Author(s):  
Ling-Yin Chang ◽  
Shuenn-Nan Chiu ◽  
Chia-Ching Wang ◽  
Wen-Chin Weng ◽  
Hsing-Yi Chang
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Parenting Stress ◽  
Children And Adolescents ◽  
Congenital Heart ◽  
Child Behavior Checklist ◽  
Cyanotic Congenital Heart Disease ◽  
Increased Risk ◽  
Acyanotic Congenital Heart Disease ◽  
Internalising Problems

Background: Children and adolescents with cyanotic congenital heart disease have been found to be at an increased risk of internalising problems. The underlying mechanisms, however, remain uncertain. Aims: To examine the association between cyanotic congenital heart disease and internalising problems and assess whether parenting stress mediates this association. Method: The study sample was 699 children and adolescents (aged 2–17 years) with congenital heart disease (253 with cyanotic congenital heart disease and 446 with acyanotic congenital heart disease) in Taiwan. The Child Behavior Checklist and the Parenting Stress Index were used to assess internalising problems and parenting stress, respectively. A series of multiple regression models was conducted using the SPSS PROCESS procedure to test the association between types of congenital heart disease and internalising problems and the mediating role of parenting stress. A bootstrapping approach was applied to determine the significance of mediation. Results: Compared with acyanotic congenital heart disease, cyanotic congenital heart disease was associated with increased levels of internalising problems (B=2.52, P<0.01), and this association was mediated by parenting stress (B=0.97, 95% bias-corrected bootstrap confidence interval 0.24, 1.75). In particular, parents of children with cyanotic congenital heart disease reported significantly more parenting stress compared with parents of children with acyanotic congenital heart disease (B=4.63, P<0.01), which contributed to elevated levels of internalising problems in the offspring (B=0.21, P<0.001). Conclusion: Cyanotic congenital heart disease conferred risks for internalising problems in children and adolescents, and this association was mediated by parenting stress. Interventions to decrease internalising problems in children and adolescents with cyanotic congenital heart disease may be more effective by targeting parenting stress.

scholarly journals Severe Acute Malnutrition among Unoperated Ethiopian Children with Congenital Heart Disease: A Wake-up Call to Reverse the Situation, A Retrospective Cross-Sectional Study

2020 ◽  
Vol 30 (5) ◽  
Author(s):  
Bruk Assefa ◽  
Henok Tadele
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Severe Acute Malnutrition ◽  
Cross Sectional Study ◽  
Acute Malnutrition ◽  
Sectional Study ◽  
Cross Sectional ◽  
Increased Risk ◽  
Anthropometric Assessment

BACKGROUND፡ Children with Congenital Heart Disease (CHD) are at increased risk for severe acute malnutrition (SAM). We aimed to determine the magnitude and determinants of SAM among children with CHD in a tertiary hospital.METHODS: Retrospective cross-sectional study was conducted among children with CHD between 2016 and 2019. Clinical and anthropometric data were retrieved from medical records. Anthropometric assessment was done by using WHO standard growth curves. Data analysis was done using Statistical Package for Social Sciences V22. Statistical significance was set at p-value<0.05, and multivariable logistic regression was used to determin predictors.RESULTS: There were 2400 pediatric admissions during the study period,CHD accounted for 6.5%(156) of admissions. For review, 141 records were eligible. The gender distribution was comparable, males 51.1% (72). Infants (<12 months) and older children (>12 months) accounted for 57.4% (81) and 42.6% (60) of study subjects, respectively. SAM was documented in 51.8% (73) of the study subjects, [95% CI: 44.7-60.2]. Infants had higher odds of SAM compared to children aged >12 months[adjusted odds ratio (AOR)= 4.48, 95%CI:2.07-9.70]. Anemic children had higher odds for SAM[AOR =3.76, 95% CI:1.54-9.18]. Children without acyanotic CHD with heart failure(HF) were 58% less likely to develop SAM[AOR= 0.42, 95% CI:0.19-0.96].CONCLUSION: The burden of SAM among children with CHD is high. Younger age,anemia and acyanotic CHD with HF predicted SAM.Screening for anemia and targeted anthropometric assessment are recommended for early SAM detection.

Galectin-3 plasma levels in adult congenital heart disease and the pressure overloaded right ventricle: reason matters

2020 ◽  
Vol 14 (13) ◽  
pp. 1197-1205
Author(s):  
Ewa Kowalik ◽  
Beata Kuśmierczyk-Droszcz ◽  
Anna Klisiewicz ◽  
Aleksandra Wróbel ◽  
Anna Lutyńska ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Clinical Status ◽  
Pressure Overload ◽  
Cross Sectional ◽  
Galectin 3 ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Aim: To assess galectin-3 (Gal-3) levels and their relationship with clinical status and right ventricular (RV) performance in adults with RV pressure overload of various mechanisms due to congenital heart disease. Materials & methods: A cross-sectional study was conducted. Patients underwent clinical examination, blood testing and transthoracic echocardiography. Results: The study included 63 patients with congenitally corrected transposition of the great arteries, 41 patients with Eisenmenger syndrome and 20 healthy controls. Gal-3 concentrations were higher in patients compared with controls (7.83 vs 6.11 ng/ml; p = 0.002). Biomarker levels correlated with age, New York Health Association class, N-terminal probrain natriuretic peptide and RV function only in congenitally corrected transposition of the great arteries patients. Conclusion: Gal-3 profile in congenital heart disease patients and pressure-overloaded RV differs according to the cause of pressure overload.

scholarly journals Development of nephropathy in an adult patient after Fontan palliation for cyanotic congenital heart disease

2021 ◽  
Author(s):  
Kaori Hayashi ◽  
Akinori Hashiguchi ◽  
Masako Ikemiyagi ◽  
Hirobumi Tokuyama ◽  
Shu Wakino ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Adult Patient ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease ◽  
Fontan Palliation

scholarly journals A novel haemoglobin variant mimicking cyanotic congenital heart disease

2016 ◽  
pp. bcr2015213615
Author(s):  
Francisco Abecasis ◽  
Inês Marques ◽  
Celeste Bento ◽  
Anabela Ferrão
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease ◽  
Haemoglobin Variant

Assessment of the conjunctival microcirculation in adult patients with cyanotic congenital heart disease compared to healthy controls

2021 ◽  
pp. 104167
Author(s):  
Paul F. Brennan ◽  
Min Jing ◽  
Andrew J. McNeil ◽  
Agnes Awuah ◽  
Jonathan Mailey ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease ◽  
Adult Patients ◽  
Healthy Controls
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