The Heart in Sickle Cell Anemia

Covitz, Wesley; Espeland, Mark A.; Gallagher, Dianne; Hellenbrand, William E.; Leff, Sanford; Talner, Norman S.

doi:10.1378/chest.108.5.1214

Cited by 121 publications

(37 citation statements)

References 26 publications

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“…Chronic anemia, hypoxia, local ischemia due to sickling and iron overload [16] could explain this dilatation. These results are in agreement with several reports [1,17,18,19]. After L -carnitine therapy, patients with initial abnormal left atrial dimensions showed improvement, but the difference did not reach a statistical significance (p = 0.08).…”

Section: Discussionsupporting

confidence: 92%

Diastolic Dysfunction and Pulmonary Hypertension in Sickle Cell Anemia: Is There a Role for <i>L</i>-Carnitine Treatment?

et al. 2006

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Clinical manifestations of cardiovascular abnormalities in patients with sickle cell (SC) anemia are well documented. Many variables were assessed in our study before and after administration of L-carnitine to randomly selected 37 SC disease (SCD) children for a period of 6 months. Variables such as weight, height, serum ferritin levels, units of blood transfused and the number of veno-occlusive crises all showed significant improvement after the 6 months of therapy with L-carnitine. Our study also showed that cardiac diastolic function and pulmonary hypertention are common in pediatric SCD patients. These two disorders showed some improvement after L-carnitine administration. Therefore, L-carnitine deserves a rigorous large-scale randomized clinical trial to evaluate its potential benefits as treatment for SCD patients with cardiac complications.

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Section: Discussionsupporting

confidence: 92%

Diastolic Dysfunction and Pulmonary Hypertension in Sickle Cell Anemia: Is There a Role for <i>L</i>-Carnitine Treatment?

et al. 2006

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“…2 Most patients with SCD have normal left ventricular function, but myocardial dysfunction may occur and can be complicated by coexisting chronic renal failure, cor pulmonale, volume overload of anemia or myocardial ischemia. 7,8 More than 2 years after chronic renal failure, our patient developed severe dilated cardiomyopathy without myocardial ischemia or epicardial vessel disease. The relation between cardiomyopathy and SCD is not clear; cardiovascular manifestations of SCD have been attributed to chronic hemolytic anemia accompanied by an increased ventricular pre-load with the development over time of cardiomegaly with ventricle dilation and evidence of cardiac dysfunction.…”

Section: Discussionmentioning

confidence: 81%

“…6 Cardiac involvement includes various types of complications. 2,7 Pulmonary hypertension is common and associated with increased morbidity and mortality. 3 Cardiac chamber structural abnormalities include right and left ventricular dilation and left ventricular hypertrophy.…”

Section: Discussionmentioning

confidence: 99%

Successful Combined Heart and Kidney Transplantation in a Patient With Sickle-cell Anemia

Audard¹,

Grimbert²,

Kirsch³

et al. 2006

The Journal of Heart and Lung Transplantation

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“…While systolic function was normal in both groups, the echocardiographic estimate of LV filling pressure, lateral E/e′, was significantly associated with TRV and LAV z-score but not with LVED z-score or LVMi (Figure 2, Figure S3 and Table S2), which indicates that patients with TRV≥2.5m/sec have features of restrictive physiology. Next, we examined whether the association between LAV, diastolic dysfunction and TRV is also observed with LVED or LVMi, which are directly related to anemia(17,18). As anticipated, LAV z-score, LVED z-score and LVMi were associated with the degree of anemia, reticulocyte count, LDH, and bilirubin (Table 4).…”

Section: Resultsmentioning

confidence: 99%

“…This suggests that LA enlargement precedes enlargement of other chambers in SCD. In addition, LV dilation and LVH are known adaptive responses to anemia(18), and these worsen over time in SCD(17,28,30). Thus, LVED enlargement and LVH were more noticeable in meta-analysis results, compared to our data from a younger cohort of SCD patients than most studies in the meta-analysis.…”

Section: Discussionmentioning

confidence: 99%

Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease

Niss¹,

Quinn²,

Lane³

et al. 2016

JACC: Cardiovascular Imaging

115

137

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Objective To identify a unifying cardiac pathophysiology that explains the cardiac pathology in SCD. Background Cardiopulmonary complications, the leading cause of adult mortality in sickle cell disease (SCD), are associated with heart chamber dilation, diastolic dysfunction, elevated tricuspid regurgitant jet velocity (TRV) and pulmonary hypertension (PH). However, no unifying cardiac pathophysiology has been identified to explain these findings. Methods In a two-part study, we first examined SCD patients who underwent screening echocardiography during steady state at our institution. We then conducted a meta-analysis of cardiac studies in SCD. Results In the 134 SCD patients studied (median age 11 years), a significant enlargement of the left atrial volume was present (z-score 3.1, P=0.002), shortening fraction (SF) was normal (37.6 ± 4.7%), and lateral and septal ratios of mitral velocity to early diastolic velocity of the mitral annulus (E/e′) were severely abnormal in 8% and 14% of patients, respectively, indicating impaired diastolic function. Both TRV and lateral E/e′ correlated with enlarged left atrial volume in SCD (P=0.003 and P=0.006, respectively). Meta-analysis of 68 studies confirmed significant left atrial diameter enlargement in SCD patients compared to controls, evidence of diastolic dysfunction and enlarged left ventricular end-diastolic dimension with normal SF. The majority of patients with catheter-confirmed PH had mild pulmonary venous hypertension consistent with restrictive cardiac physiology. Conclusions Patients with SCD have a unique cardiomyopathy with restrictive physiology that is superimposed on hyperdynamic physiology, and is characterized by diastolic dysfunction, left atrial dilation and normal systolic function. This results in mild, secondary, pulmonary venous hypertension and elevated TRV. Sudden death is common in other forms of restrictive cardiomyopathy. Our finding of this unique restrictive cardiomyopathy may explain the increased mortality and sudden death seen in patients with SCD with mildly elevated TRV.

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The Heart in Sickle Cell Anemia

Cited by 121 publications

References 26 publications

Diastolic Dysfunction and Pulmonary Hypertension in Sickle Cell Anemia: Is There a Role for <i>L</i>-Carnitine Treatment?

Diastolic Dysfunction and Pulmonary Hypertension in Sickle Cell Anemia: Is There a Role for <i>L</i>-Carnitine Treatment?

Successful Combined Heart and Kidney Transplantation in a Patient With Sickle-cell Anemia

Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease

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