Cardiopulmonary bypass for surgical correction of congenital heart disease in children with sickle cell disease: a case series

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.

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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 ◽

10.1542/peds.33.4.562 ◽

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.

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

Stroke ◽

10.1161/str.45.suppl_1.wmp113 ◽

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.

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Modified ultrafiltration in surgical correction of congenital heart disease with cardiopulmonary bypass

Perfusion ◽

10.1191/0267659103pf629oa ◽

2003 ◽

Vol 18 (1_suppl) ◽

pp. 61-68 ◽

Cited By ~ 9

Author(s):

Miguel A Maluf

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Cardiopulmonary Bypass ◽

Congenital Heart ◽

Surgical Correction ◽

Modified Technique ◽

Homogeneous Groups ◽

Volume Balance ◽

Water Accumulation ◽

Total Body

The surgical correction of congenital heart disease using haemodilution and hypothermia with cardiopulmonary bypass (CPB) may expose patients to tissue ischaemia and initiate a systemic inflammatory response, increasing the total body water and inducing impairment, especially of heart, lung and brain function. It is possible to use ultrafiltration during CPB in the rewarming phase to remove water accumulation in the third space (conventional ultrafiltration). The reduced volume of prime used in children and the ability only to filter the reservoir blood during CPB led the Great Ormond Street Group to modify the method of ultrafiltration with regards to the placement of the filter and the timing of filtration (post-CPB). The main advantage of the modified technique is the ultrafiltration of the patient. A prospective nonrandomized study has been conducted to compare conventional with conventional+modified ultrafiltration. From January 1996 to March 1998, 41 patients underwent correction of congenital heart disease and were submitted to a comparative study (homogeneous groups), using either the conventional or the conventional+modified ultrafiltration techniques. There were no technical complications, no patient required mediastinal re-exploration due to bleeding and it was possible to close all the chests. There were significant differences in the ultrafiltrate volume balance (143.39 /54.3 versus 2279 /71.4 mL; P B /0.001) but there were no significant differences in clinical postoperative evolution between the conventional and the conventional+modified ultrafiltration.

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Modified ultrafiltration in surgical correction of congenital heart disease with cardiopulmonary bypass

Perfusion ◽

10.1191/267659103pf629oa ◽

2003 ◽

Vol 18 (1) ◽

pp. 61-68 ◽

Cited By ~ 1

Author(s):

Miguel A Maluf

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Cardiopulmonary Bypass ◽

Congenital Heart ◽

Surgical Correction

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Pushing the envelope: a treat and repair strategy for patients with advanced pulmonary hypertension associated with congenital heart disease

Pulmonary Circulation ◽

10.1177/2045893217726086 ◽

2017 ◽

Vol 7 (3) ◽

pp. 747-751 ◽

Cited By ~ 4

Author(s):

Rebecca Johnson Kameny ◽

Elizabeth Colglazier ◽

Hythem Nawaytou ◽

Phillip Moore ◽

V. Mohan Reddy ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Case Series ◽

Surgical Correction ◽

Complex Congenital Heart Disease ◽

Pulmonary Vascular Disease ◽

Pulmonary Hemodynamics ◽

Operative Strategy ◽

Repair Strategy

Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease as a consequence of altered pulmonary hemodynamics with increased pulmonary blood flow and pressure. The development of pulmonary vascular disease (PVD) in this patient population is an important concern in determining operative strategy. Early, definitive surgical repair, when possible, is the best therapy to prevent and treat PVD. However, this is not possible in some patients because they either presented late, after the development of PVD, or they have complex lesions not amenable to one-step surgical correction, including patients with single ventricle physiology, who have a continuing risk of developing PVD. These patients represent an important, high-risk subgroup and many have been considered inoperable. We present a case series of two patients with complex congenital heart disease and advanced PVD who successfully underwent a treat and repair strategy with aggressive PAH therapies before surgical correction. Both patients had normalization of pulmonary vascular resistance prior to surgical correction. Caution is warranted in applying this strategy broadly and long-term follow-up for these patients is crucial. However, this treat and repair strategy may allow for favorable outcomes among some patients who previously had no therapeutic options.

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Cardiac transplantation in adults with congenital heart disease: A single center case series

Clinical Transplantation ◽

10.1111/ctr.14430 ◽

2021 ◽

Author(s):

Christopher A. Heid ◽

Raghav Chandra ◽

Charles Liu ◽

Jessica Pruszynski ◽

Mitri K. Khoury ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Cardiac Transplantation ◽

Congenital Heart ◽

Case Series ◽

Single Center

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Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa320 ◽

2020 ◽

Author(s):

Sarah Blissett ◽

David Blusztein ◽

Vaikom S Mahadevan

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Side Effects ◽

Arterial Hypertension ◽

Exercise Capacity ◽

Congenital Heart ◽

Pulmonary Veins ◽

Case Series ◽

Pulmonary Arterial

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

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A systematic review examining the clinical and health-care outcomes for congenital heart disease patients using home monitoring programmes

Journal of Telemedicine and Telecare ◽

10.1177/1357633x20984052 ◽

2021 ◽

pp. 1357633X2098405

Author(s):

Rachel Crawford ◽

Ciara Hughes ◽

Sonyia McFadden ◽

Jacqui Crawford

Keyword(s):

Systematic Review ◽

Health Care ◽

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Case Series ◽

Home Monitoring ◽

Risk Of Bias ◽

Health Care Outcomes ◽

Care Outcomes

Objectives This review aimed to present the clinical and health-care outcomes for patients with congenital heart disease (CHD) who use home monitoring technologies. Methods Five databases were systematically searched from inception to November 2020 for quantitative studies in this area. Data were extracted using a pre-formatted data-collection table which included information on participants, interventions, outcome measures and results. Risk of bias was determined using the Cochrane Risk of Bias 2 tool for randomised controlled trials (RCTs), the Newcastle–Ottawa Quality Assessment Scale for cohort studies and the Institute of Health Economics quality appraisal checklist for case-series studies. Data synthesis: Twenty-two studies were included in this systematic review, which included four RCTs, 12 cohort studies and six case-series studies. Seventeen studies reported on mortality rates, with 59% reporting that home monitoring programmes were associated with either a significant reduction or trend for lower mortality and 12% reporting that mortality trended higher. Fourteen studies reported on unplanned readmissions/health-care resource use, with 29% of studies reporting that this outcome was significantly decreased or trended lower with home monitoring and 21% reported an increase. Impact on treatment was reported in 15 studies, with 67% of studies finding that either treatment was undertaken significantly earlier or significantly more interventions were undertaken in the home monitoring groups. Conclusion The use of home monitoring programmes may be beneficial in reducing mortality, enabling earlier and more timely detection and treatment of CHD complication. However, currently, this evidence is limited due to weakness in study designs.

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