Transient Left Ventricular Dysfunction in Childhood Sickle Cell Disease

Johnson, W. H.; McCrary, R.B.; Mankad, Vipul N.

doi:10.1007/s002469900447

Cited by 11 publications

(8 citation statements)

References 10 publications

(16 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…This is consistent with previous studies which have shown significant correlation between left ventricular size and mass and degree of anemia [22, 24]. A substantial portion of pulmonary artery pressure elevation is likely a consequence of increased cardiac output and stroke volume.…”

Section: Discussionsupporting

confidence: 93%

“…However, the presence of increased preload suggests elevated preload is necessary to provide normal left ventricle shortening. These findings are similar to other studies in SCD [21, 22, 26, 27]. Previous studies have reported that children with SCD have lower load independent measures of systolic function such as velocity of circumferential fiber shortening [24, 27].…”

Section: Discussionsupporting

confidence: 91%

“…Calculations for left ventricular mass index and pulmonary vascular resistance were significantly higher in SCD after adjustment for hemoglobin concentration. We postulate that these findings provide evidence to support an anemia independent process that likely represents the effect of hemolysis on the systemic and pulmonary vascular beds [19, 20, 22, 28, 29]. Hemolysis and hemoglobin were independent predictors of TRV in our prior analysis [30].…”

Section: Discussionmentioning

confidence: 81%

See 2 more Smart Citations

Prospective Echocardiography Assessment of Pulmonary Hypertension and Its Potential Etiologies in Children With Sickle Cell Disease††Conflicts of interest: Dr. Gordeuk has received grants from BioMarin Pharmaceutical Inc., Novato, California, and Actelion Pharmaceuticals Ltd., Allschwil, Switzerland, and is a consultant for Ikaria Holdings, Clinton, New Jersey.

Dham

Ensing

Minniti

et al. 2009

The American Journal of Cardiology

View full text Add to dashboard Cite

Pulmonary hypertension (PH) is associated with adverse outcomes in adults with sickle cell disease (SCD) but its importance in children is less clear. We define the incidence and etiologies of PH in pediatric patients with SCD. Children with SCD (n = 310) and matched controls (n = 54) were prospectively enrolled under basal conditions. Participants underwent echocardiography, pulse oximetry, 6-minute walk, and hematologic testing. Echocardiographic measures were compared between SCD and control subjects before and after adjusting for hemoglobin. Correlation of echocardiographic and clinical parameters was performed. Tricuspid regurgitation velocity (TRV) was elevated compared to controls (2.28 vs. 2.10 m/s, p <0.0001). Increased TRV was associated with left ventricular diastolic diameter, hemoglobin, and estimated left atrial pressure. TRV remained elevated when controlled for left ventricular diameter and left atrial pressure. An echocardiography-derived pulmonary resistance was not significantly different between SCD and control patients, although it was elevated in the SCD subgroup with elevated © 2009 Excerpta Medica, Inc. Published by Elsevier Inc. All rights reserved.Correspondence to: Craig Sable, MD, Children's National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010, Tel: 202-476-2020, Fax: 202-476-5700, Email: csable@cnmc.org. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. TRV. When controlled for hemoglobin, TRV was no longer statistically different, but pulmonary insufficiency velocity, septal wall thickness and estimated pulmonary resistance were statistically higher. TRV, pulmonary insufficiency end diastolic velocity, and markers of increased cardiac output correlated with indicators of adverse functional status including history of acute chest syndrome, stroke, transfusions, and 6-minute walk. In conclusion, children with SCD have mildly increased TRV that correlated with increased cardiac output and left ventricular filling pressures. Hemoglobin adjusted analysis also suggests contribution of primary vascular changes. KeywordsPulmonary hypertension; Tricuspid regurgitation; Pulmonary vascular resistance; Sickle cellIn the present report, we compare multiple echocardiographic variables between children with sickle cell disease (SCD) and age and gender matched controls to assess relative contributions of potential determinants of pulmonary artery pressure in this population. We also examine the relations among TRV, other echocardiographic measurements and clinical parameters among children with SCD to better elucidate the physiology and consequences of echo...

show abstract

Section: Discussionsupporting

confidence: 93%

Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 81%

See 1 more Smart Citation

Prospective Echocardiography Assessment of Pulmonary Hypertension and Its Potential Etiologies in Children With Sickle Cell Disease††Conflicts of interest: Dr. Gordeuk has received grants from BioMarin Pharmaceutical Inc., Novato, California, and Actelion Pharmaceuticals Ltd., Allschwil, Switzerland, and is a consultant for Ikaria Holdings, Clinton, New Jersey.

Dham

Ensing

Minniti

et al. 2009

The American Journal of Cardiology

View full text Add to dashboard Cite

show abstract

“…Despite the stimulus for sickling, several published studies (14,19) do not support the existence of an "ischemic sickle cell cardiomyopathy." In contrast, case reports (20,21) describe clinical evidence of myocardial infarction in pediatric SCA patients, and autopsy findings (22,23) document subendocardial fibrosis proposed to be caused by chronic anemia and increased O 2 demand of a dilated hypertrophied ventricle. Finally, although this study did not address genetic variations or polymorphisims, it is possible that dimensional and functional differences seen in SCA patients, previously ascribed to abnormal loading conditions, may, in fact, represent intrinsic genetic differences in myocardial cytoarchitecture not yet described in association with SCA.…”

Section: Contractilitymentioning

confidence: 77%

Evaluation of Left Ventricular Systolic Function in Pediatric Sickle Cell Anemia Patients Using the End-Systolic Wall Stress-Velocity of Circumferential Fiber Shortening Relationship

Lamers

Ensing

Pignatelli

et al. 2006

Journal of the American College of Cardiology

View full text Add to dashboard Cite

show abstract

“…Only 1 previous report of myocardial dysfunction with signs of myocardial infarction suggests reversibility of ischemia after exchange transfusion. 1,15 We report an adolescent who had previous evidence of myocardial dysfunction with findings suggesting acute myocardial infarction and rapid development of severe cardiac dysfunction and who responded to exchange transfusion. …”

mentioning

confidence: 99%

Myocardial Infarction and Transient Ventricular Dysfunction in an Adolescent With Sickle Cell Disease

Deymann

Goertz

2003

Pediatrics

View full text Add to dashboard Cite

ABSTRACT. We report a case of an adolescent who had sickle cell disease and previous evidence of myocardial damage and presented with abdominal pain and rapid progression to cardiogenic shock and subsequent development of myocardial infarction. To our knowledge, this represents only the second report of a case of acute myocardial ischemia and subsequent infarction resulting transient ventricular dysfunction reported in a child with sickle cell disease successfully treated with exchange transfusion. The pathophysiology of this complication remains unclear, and cardiac complications may remain undetected as lung, bone, and brain infarcts are more common and the pain associated with sickle cell crisis may mask the ischemic symptoms. Multiple factors may contribute to ischemia in addition to the presence of a vaso-occlusive crisis or infection. Acute or chronic myocardial ischemia are probably more prevalent than currently known. V ascular injury that leads to thrombosis and hyperplastic remodeling of large arterial vessels 1 is believed to be the cause of ischemic or embolic stroke in sickle cell disease. Pathologic examination of the affected cerebral vasculature reveals intimal proliferation with a break-up of the elastic lamina. Endothelial cells repopulate denuded areas only in monolayers, and the hyperplastic intima consists of fibroblasts, fibrous tissue, and scattered smooth muscle cells leading to a narrowed vascular lumen and increased fragility of the affected cerebral vessels. In comparison with stroke, myocardial infarction is rare in young adults with sickle cell disease 2-10 and may be attributable in large part to acute microvascular occlusion of small vessels. Epicardial coronary artery disease is notably uncommon in sickle cell disease. Several mechanisms may lead to impairment of microvascular circulation. In addition to impairment of circulation by sickled cells, thrombosis of small vessels is thought to be the result of endothelial damage attributable to shear injury and resulting procoagulant state and embolization of endothelial cells into the microvasculature. 3,11,12 Fibromuscular dysplasia of small cardiac vessels has been demonstrated in patients with sickle cell disease and may explain chronic ischemic changes and apoptosis in myocytes. 3,12 Echocardiographic and electrocardiogram (ECG) changes may be more prevalent in sickle cell disease than currently known. Myocardial necrosis has been observed by autopsy in 1 child with hemoglobin sickle cell disease 13 and has been reported in adults. 3,12 Chronic myocardial dysfunction can occur and may be complicated by volume overload of anemia and coexisting renal and lung disease. 2 Myocardial ischemia may be a cause of chest pain during crisis in adults 8,14 and is a possible cause for chest pain in children with sickle cell disease. Only 1 previous report of myocardial dysfunction with signs of myocardial infarction suggests reversibility of ischemia after exchange transfusion. 1,15 We report an adolescent who had previous evidence of myocardi...

show abstract

Transient Left Ventricular Dysfunction in Childhood Sickle Cell Disease

Cited by 11 publications

References 10 publications

Evaluation of Left Ventricular Systolic Function in Pediatric Sickle Cell Anemia Patients Using the End-Systolic Wall Stress-Velocity of Circumferential Fiber Shortening Relationship

Myocardial Infarction and Transient Ventricular Dysfunction in an Adolescent With Sickle Cell Disease

Contact Info

Product

Resources

About