A Hemodynamic Study of Pulmonary Hypertension in Sickle Cell Disease

Parent, Florence; Bachir, Dora; Inamo, Jocelyn; Lionnet, François; Driss, Françoise; Loko, Gylna; Habibi, Anoosha; Bennani, Soumiya; Savale, Laurent; Adnot, Serge; Maître, Bernard; Yaïci, Azzedine; Hajji, Leila; O’Callaghan, Dermot S.; Clerson, Pierre; Girot, Robert; Galactéros, Frédéric; Simonneau, Gérald

doi:10.1056/nejmoa1005565

Cited by 452 publications

(544 citation statements)

References 27 publications

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“…Sickle cell patients with leg ulcers constitute a relatively distinct sub-phenotype, characterized by less frequent hospitalizations for vaso-occlusive pain crisis [22,23], while they are prone to develop pulmonary hypertension [5,[24][25][26][27][28], suggesting that the two complications share a common pathophysiology. In a cohort of 20 SCD patients with leg ulcer who underwent right hearth catheterization for suspected pulmonary hypertension, 15 had confirmation of PAH (personal data, this cohort has been described in Mehari et al (2013) [24].…”

Section: Epidemiologymentioning

confidence: 99%

Critical Reviews: How we treat sickle cell patients with leg ulcers

Minniti

Kato

2015

American J Hematol

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The past five decades have seen an improvement in the mortality and morbidity of sickle cell disease (SCD) because of prophylaxis against infectious complications, improved and expanded red cell transfusions, implementation of hydroxyurea therapy, and advances in supportive care. Now that the majority of patients in the western hemisphere reaches adulthood, end organ diseases are frequent, which include vasculopathic complications such as chronic leg ulcers. The management of patients with leg ulcers requires the hematologist to lead a team of health care professionals, and investigates the presence of associated, but potentially still occult signs of vasculopathy, such as pulmonary hypertension, renal disease, priapism and retinopathy. These complications may be asynchronous, and long term careful screening is indicated, in order to ensure early diagnosis and intervention. It is crucial to address both the immediate consequences of pain, infection and disability, and long term effects on quality of life, employment and stigma associated with chronic ulceration. Recent insights into their pathophysiology may have practical implications. We propose a holistic approach to the management of patients' physical and emotional problems and mechanisms of ulcers formation and delayed healing. An overview of topical and systemic therapies for chronic ulcers is given, with the understanding that wound care therapy is best left to the wound specialists, medical and surgical, with whom the hematologist must keep an open line of communication. In the absence of evidence-based guidelines, our opinion is based on both a critical review of the literature and our personal clinical and research experience. Am. J. Hematol. 91:22-30, 2016. V C 2015 Wiley Periodicals, Inc. Clinical CasesCase 1. A 49 year old African American woman with sickle cell anemia, bilateral hip replacements for avascular necrosis of the femoral head, pulmonary arterial hypertension documented by pulmonary artery catheterization, and history of laser treatment and vitrectomy for proliferative retinopathy and vitreous hemorrhage, presented with a very painful ulcer at the right malleolus. She has had three previous ulcers, on both legs, the first at age 32 years, attributed to excessive standing at her job, no trauma, which had responded to topical care and healed within few months of presentation. At that time she was not taking hydroxyurea, but has been taking it now for longer than 10 years with good fetal hemoglobin response: 17%. This current ulcer developed while she was experiencing high personal stress but no physical trauma. She rarely experiences vaso-occlusive pain crisis and has very infrequent hospitalizations. She was referred to the wound care service and after 7 months of unfruitful therapies, including surgical debridement, MIST ultrasound therapy, antibiotics, and compressions, she was started on monthly transfusions and her ulcer healed 5 months later. She has had consistently elevated serum lactate dehydrogenase (LDH) (>600 IU/L), serum dire...

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Section: Epidemiologymentioning

confidence: 99%

Critical Reviews: How we treat sickle cell patients with leg ulcers

Minniti

Kato

2015

American J Hematol

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“…The prevalence of elevated TRV using echocardiography is estimated to be 25-30% among SCA patients and 10-25% in those with genotype SC 6,8,11 . However, studies have reported that only about 6-11% of patients with TRV ≥ 2.5m/s on ECHO have confirmed pulmonary hypertension on right heart catheterization 12,13 . Notwithstanding, elevated TRV ≥ 2.5m/s in sickle cell patients has been significantly associated with decreased exercise tolerance and increased mortality estimated to be about 40% at 40 months of follow-up and is therefore considered an independent risk factor for death among these patients 6,14,15 .…”

Section: Introductionmentioning

confidence: 99%

Clinical and Laboratory Predictors of Elevated TRV In Sickle Cell Anaemia

Afriyie-Mensah

Dei‐Adomakoh

Forson

et al. 2018

JHOR

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Pulmonary hypertension (PH) has become an increasingly recognized complication in sickle cell anaemia (SCA) and is a major cause of morbidity and mortality. Though the burden of SCA in sub-Saharan Africa is high, there is paucity of data on SCA-associated PH with little or no attention given to it in routine patient care. The current study therefore sought to determine the prevalence of PH and its associated risk factors among adult patients with SCA. This was a cross-sectional study involving 76 clinically stable, hydroxyurea-naive participants. We obtained socio-demographic and clinical history. Measurement of Tricuspid Regurgitant jet Velocity (TRV) was obtained via transthoracic echocardiography and lung function was assessed using spirometry and pulse oximetry. Other investigations were complete blood counts, free plasma haemoglobin, serum urea and creatinine. Twenty-five (32.9%) of study participants had elevated TRV (≥ 2.5m/s) on Doppler echocardiography, which was suggestive of raised pulmonary artery systolic pressure.There were significant associations between elevated TRV and steady-state haemoglobin (p < 0.001), blood urea level (p = 0.030), presence of chronic leg ulcers (p = 0.043) and oxygen saturation (p < 0.001) and these may be identifiable and modifiable risk factors for selective screening with echocardiography in a resource poor setting.

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“…Age, sex, and distribution of hemoglobinopathies of our patients are similar to those of other published cohorts. 3,4 The hemodynamic profile reveals elevated right atrial pressures and mean pulmonary arterial pressure (mPAP), with relatively higher cardiac outputs and lesser elevation in pulmonary vascular resistance (PVR), as typically seen in SCDPH. However, notably, the average cardiac output was lower than that of other cohorts, with or without PH.…”

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confidence: 99%

“…Nargues A. Weir, 1 Rehan Saiyed, 2 Shoaib Alam, 1 Anna Conrey, 2 Himanshu D. Desai, 3 M. Patricia George, 4 Jennifer H. Keeley, 4 Elizabeth S. Klings, 5 Alem Mehari,6 James G. Taylor VI, 2 …”

mentioning

confidence: 99%