scholarly journals Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children

2016 ◽  
Vol 43 (6) ◽  
pp. 509-513 ◽  
Author(s):  
Glen J. Iannucci ◽  
Olufolake A. Adisa ◽  
Matthew E. Oster ◽  
Michael McConnell ◽  
William T. Mahle
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Congenital Heart ◽  
Cyanotic Congenital Heart Disease ◽  
Cell Disease ◽  
Long Term Outcome ◽  
Cerebrovascular Accidents

Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo–17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction. The surviving patient had developmental delays. On the basis of this series, we suggest mitigating hypoxemia, and thus the risk of stroke, in patients who have sickle cell disease and cyanotic congenital heart disease. Potential therapies include chronic blood transfusions, hydroxyurea, earlier surgical correction to reduce the duration of hypoxemia, and heart or bone marrow transplantation.

THE COEXISTENCE OF SICKLE CELL DISEASE AND CONGENITAL HEART DISEASE: A REPORT OF THREE CASES, WITH REPAIR UNDER CARDIO-PULMONARY BY-PASS IN TWO

PEDIATRICS ◽  
1964 ◽  
Vol 33 (4) ◽  
pp. 562-570
Author(s):  
Leonard C. Harris ◽  
Mary Ellen Haggard ◽  
Luther B. Travis
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Congenital Heart ◽  
Septal Defect ◽  
Pulmonary Stenosis ◽  
Severe Case ◽  
Cell Disease ◽  
Hemoglobin S

Sickle cell disease, characterized by protean manifestations, has been confused frequently with rheumatic fever and congenital heart disease. Though it has been described in combination with rheumatic mitral stenosis, little on no consideration has been given to the association of sickle cell disease with congenital heart disease. This communication describes the occurrence of hemoglobin S disease in combination with congenital heart disease in three patients, the lesions being an atrial septal defect of the secundum type in one and pulmonary stenosis in two other patients. The congenital cardiac abnormalities were repaired under cardiopulmonary by-pass in the case of atrio-septal defect and the more severe case of pulmonary stenosis. Preparation for surgery consisted in the suppression of hemoglobin S formation by blood transfusions. During cardiopulmonary by-pass, further dilution of the hemoglobin S cells occurred so that their concentration in the patients' blood was negligible. Following surgery, it was necessary to administer greater amounts of intravenous fluid than usual to allow for the reduced ability to concentrate urine. Convalescence was unremarkable in each case.

Abstract W MP113: Transient Ischemic Attacks Requiring Hospitalization of Children in the United States: Kids’ Inpatient Database 2003-2009

Stroke ◽  
2014 ◽  
Vol 45 (suppl_1) ◽  
Author(s):  
Malik M Adil ◽  
Adnan I Qureshi ◽  
Lauren A Beslow ◽  
Lori C Jordan
Keyword(s):  
Risk Factors ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ischemic Stroke ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Congenital Heart ◽  
Transient Ischemic Attacks ◽  
Cell Disease ◽  
Inpatient Database

Objective: To assess the prevalence of and risk factors for transient ischemic attacks requiring hospitalization in children in a large national sample. Methods: Using the Healthcare Cost and Utilization Project Kids’ Inpatient Database, ICD-9 code (435.XX) identified children 1-18 year(s) admitted for TIA from 2003-2009. Descriptive analysis was performed to identify patient characteristics; and trend analysis was performed to determine any change in annual average hospitalization days from 2003-2009 utilizing the Cochran-Armitage trend test. Results: TIA was the primary diagnosis for hospitalization in 531 children over the 3-year sample (Table). Important secondary diagnoses during the same hospital admission included sickle cell disease (20%), congenital heart disease (11%), migraine (12%), moyamoya disease (10%) and stroke (4%). Anemia, coagulopathy, diabetes, hypertension, and obesity were rare co-morbid conditions, each noted in ≤6% of children. TIAs were more common in adolescents with 67% occurring in those aged 11-18 years. The mean length of stay decreased from 3.0 days (95% confidence interval (CI) 2.4 -3.6) in 2003 to 2.3 days (95% CI 2.0-2.7) in 2009 (p<0.04). No children died; 97% were discharged to home. During the same time period 2590 children were admitted with ischemic stroke; therefore 4.8 children with ischemic stroke were admitted for every child with TIA. Conclusion: Recognized risk factors for TIA including sickle cell disease, congenital heart disease, moyamoya, recent stroke and migraine were present in <60% of children. Admissions for ischemic stroke were about 5-fold more common than TIA in children. Further study is required to understand the risk of stroke after TIA in children to guide appropriate evaluation and treatment.

scholarly journals Contemporary Patients With Congenital Heart Disease: Uniform Atrial Tachycardia Substrates Allow for Clear Ablation End Points With Improved Long-Term Outcome

2021 ◽  
Author(s):  
Charlotte Brouwer ◽  
Joachim Hebe ◽  
Peter Lukac ◽  
Jan-Hendrik Nürnberg ◽  
Jens Cosedis Nielsen ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Atrial Tachycardia ◽  
Congenital Heart ◽  
Reentrant Tachycardia ◽  
Term Outcome ◽  
End Points ◽  
Long Term Outcome ◽  
Procedural Success

Background: Poor outcome after atrial tachycardia (AT) radiofrequency catheter ablation (RFCA) in repaired congenital heart disease (CHD) has been attributed to CHD complexity. This may not apply to contemporary patients. The objective of our study was to assess outcome after RFCA for AT in contemporary patients with CHD according to prior atrial surgery and predefined procedural end points. Methods: Patients with CHD referred for AT RFCA to 3 European centers were classified as no atrial surgery/cannulation only, limited or extensive prior atrial surgery. Procedural success was predefined as termination and nonreinducibility for focal AT and bidirectional block across ablation lines for intra-atrial reentrant tachycardia and after empirical substrate ablation for noninducible patients. Patients were followed for AT recurrence and mortality. Results: Ablation was performed in 290 patients (41±17 years, 59% male; 3-dimensional mapping 89%, irrigated tip catheters 90%, transbaffle access 15%). In 197, 233 AT were targeted (196 intra-atrial reentrant tachycardia [64% cavotricuspid (mitral) isthmus–dependent, 33% systemic-venous incision–dependent] and 37 focal AT). In 93 noninducible patients, empirical substrate ablation was performed. Procedural success was achieved in 209 (84%) patients. AT recurred in 148 (54%) 10 (interquartile range, 0–25) months after RFCA. AT-free survival was significantly better in patients with no atrial repair/cannulation only and in patients with complete procedural success independently of CHD complexity. From 94 patients undergoing reablation, the initially targeted substrate had recovered in 64%. Conclusions: In contemporary patients with CHD, outcome after AT ablation is associated with presence of prior atrial surgery and achievement of predefined procedural end points rather than CHD complexity. Techniques to improve lesion durability are likely to further improve long-term outcome.

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