Right ventricular abnormalities in sickle cell anemia: Evidence of a progressive increase in pulmonary vascular resistance

Qureshi, Naveen; Joyce, James J.; Qi, Ning; Chang, Ruey‐Kang R.

doi:10.1016/j.jpeds.2005.12.055

Cited by 33 publications

(24 citation statements)

References 33 publications

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“…Most recently, it has been recognised that 30% of adults with sickle cell disease develop pulmonary hypertension, which is strongly associated with a high rate of sudden death (Gladwin et al, 2004). Similar rates of pulmonary hypertension have been observed in children (Suell et al, 2005;Qureshi et al, 2006). The severity of these complications warrants the institution of therapeutic interventions that prevent and eliminate these life-threatening events.…”

Section: Discussionmentioning

confidence: 96%

Matched‐related donor transplantation for sickle cell disease: report from the Center for International Blood and Transplant Research

Panepinto¹,

Walters²,

Carreras³

et al. 2007

Br J Haematol

162

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SummaryWe report outcomes after myeloablative haematopoietic cell transplantation (HCT) from human leucocyte antigen (HLA)‐matched sibling donors in 67 patients with sickle cell disease transplanted between 1989 and 2002. The most common indications for transplantation were stroke and recurrent vaso‐occlusive crisis in 38% and 37% of patients respectively. The median age at transplantation was 10 years and 67% of patients had received >10 red blood cell transfusions before HCT. Twenty‐seven percent of patients had a poor performance score at transplantation. Ninety‐four percent received busulfan and cyclophosphamide‐containing conditioning regimens and bone marrow was the predominant source of donor cells. Most patients achieved haematopoietic recovery and no deaths occurred during the early post‐transplant period. Rates of acute and chronic graft‐versus‐host disease were 10% and 22% respectively. Sixty‐four of 67 patients are alive with 5‐year probabilities of disease‐free and overall survival of 85% and 97% respectively. Nine patients had graft failure with recovery of sickle erythropoiesis, eight of who had recurrent sickle‐related events. This report confirms and extends earlier reports that HCT from HLA‐matched related donors offers a very high survival rate, with few transplant‐related complications and the elimination of sickle‐related complications in the majority of patients who undergo this therapy.

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Section: Discussionmentioning

confidence: 96%

Matched‐related donor transplantation for sickle cell disease: report from the Center for International Blood and Transplant Research

Panepinto¹,

Walters²,

Carreras³

et al. 2007

Br J Haematol

162

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“…In addition, alteration of the left and right heart cavities (Zilberman et al, 2007;Eddine et al, 2009) have been evidenced by standard echocardiography and by pulsed wave tissue Doppler imaging (TDI) in SCD patients. Left ventricular (LV) diastolic dysfunction is present in about 18% of SCD adult patients and is an independent predictor of death (Sachdev et al, 2007) while right ventricular (RV) hypertrophy is seen in 25% of the SCD paediatric population (Qureshi et al, 2006).…”

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confidence: 99%

Pulmonary hypertension in sickle cell disease children under 10 years of age

et al. 2010

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SummaryDespite the finding of elevated Tricuspid Regurgitant Velocity (TRV) in children below 5 years of age, the prevalence and evolution of Pulmonary Hypertension (PH) in young children with sickle cell disease (SCD) are unclear. In order to identify predictive factors of precocious PH development, SCD children ‡3 years old, at steady state, underwent annual echocardiography and Tissue Doppler Imaging (TDI). Patients receiving chronic transfusion were excluded. Thirty-seven of seventy-five patients were ‡3 years, with measurable TRV. In our young population (mean age 6AE2 years) of mainly African, HbS/HbS patients, 8/37 (21AE6%) had TRV ‡2AE5 m/s, 8% being only 3 years old. Significant correlation was found between precocious TRV elevation and high platelet and reticulocyte counts and frequent acute chest syndromes (ACS). In multivariate analysis, ACS was the only variable predicting TRV ‡2AE5 m/s. TDI of the 37 patients showed signs of diastolic dysfunction of the left ventricle. At follow-up all eight patients with high TRV displayed further increase and seven more developed TRV ‡2AE5 m/s. PH seems to begin in children earlier than expected. Factors involved in its early onset might be different from the ones causing its development in older children or adults. African children might benefit from early screening and re-assessment once a year.

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“…In the setting of chronic anaemia cardiac output is raised mainly by an increase in stroke volume, which prolongs ETc along with a slight change in heart rate (Naveen Qureshi et al). 19 In our study, we found that 32 out of 138 sickle cell patients (23.18%) had PH. Out of the 32 patients, 25 (18.1%) had mild, 7 (5.1%) had moderate and 0 patients had severe PH.…”

Section: Discussionmentioning

confidence: 50%

“…Years of clinical and subclinical haemolysis and vaso-occlusive episodes in sickle cell anaemia may lead to an obstructive pulmonary vasculopathy with a gradual increase in pulmonary vascular resistance and RV afterload, reflected in a steady increase in RV mass index out of proportion to the chronic anaemia-induced RV preload (Naveen Qureshi et al). 19 Significantly, higher levels of RV-Etc was noted in SS subjects compared to AS subjects (p < 0.0001). Mean values in AS subjects was 336.34 ± 18.52 msec and in SS subjects was 376.62 ± 24.89 msec.…”

Section: Discussionmentioning

confidence: 86%

“…Moreover, stroke volume is increased principally by raising the diastolic volume with only a minimal change in ejection fraction, which explains why RV-FAC remains in the normal range (Naveen Qureshi et al). 19 Comparison of RV-FW MI between AS and SS subjects was performed using Student's unpaired "t" test. Significantly, higher levels of this parameter were noted in SS subjects compared to AS subjects (p < 0.0001).…”

Section: Discussionmentioning

confidence: 99%

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Echocardiographic Study of Right Ventricular Abnormalities in Adult Patients of Sickle Cell Anaemia

Ahmad¹,

Toppo²,

Varma³

2017

jemds

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BACKGROUNDSickle Cell Disorder (SCD) is the first diagnosed disease that was linked to the haemoglobin protein. It is an autosomal recessive disorder that occurs throughout the world. The cardiac effects of this chronic illness on the left ventricle have been extensively investigated in adults. However, fewer adult studies and only one study that included adolescents have investigated any aspect of the impact of SCA on the Right Ventricle (RV).

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Right ventricular abnormalities in sickle cell anemia: Evidence of a progressive increase in pulmonary vascular resistance

Cited by 33 publications

References 33 publications

Matched‐related donor transplantation for sickle cell disease: report from the Center for International Blood and Transplant Research

Matched‐related donor transplantation for sickle cell disease: report from the Center for International Blood and Transplant Research

Pulmonary hypertension in sickle cell disease children under 10 years of age

Echocardiographic Study of Right Ventricular Abnormalities in Adult Patients of Sickle Cell Anaemia

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