Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome

Tay, Edgar; Peset, Ana; Papaphylactou, Maria; Inuzuka, Ryo; Alonso‐Gonzalez, Rafael; Giannakoulas, George; Tzifa, Aphrodite; Goletto, Sara; Broberg, Craig S.; Dimopoulos, Konstantinos; Gatzoulis, Michael Α.

doi:10.1016/j.ijcard.2010.05.066

Cited by 118 publications

(64 citation statements)

References 37 publications

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“…5 These observations pointed to the possibility that iron status may influence the expression of pulmonary hypertension in Chuvash polycythemia as well. Furthermore, iron deficiency is common in patients with idiopathic pulmonary hypertension [15][16][17] and pulmonary hypertension secondary to cyanotic congenital heart disease, 18 and is associated with reduced exercise capacity. [16][17] Iron therapy improved exercise capacity in such patients.…”

Section: Introductionmentioning

confidence: 99%

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Pulmonary artery pressure and iron deficiency in patients with upregulation of hypoxia sensing due to homozygous VHLR200W mutation (Chuvash polycythemia)

et al. 2011

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BackgroundPatients with Chuvash polycythemia, (homozygosity for the R200W mutation in the von Hippel Lindau gene (VHL)), have elevated levels of hypoxia inducible factors HIF-1 and HIF-2, often become iron-deficient secondary to phlebotomy, and have elevated estimated pulmonary artery pressure by echocardiography. The objectives of this study were to provide a comprehensive echocardiographic assessment of cardiovascular physiology and to identify clinical, hematologic and cardiovascular risk factors for elevation of tricuspid regurgitation velocity in children and adults with Chuvash polycythemia. Design and MethodsThis cross-sectional observational study of 120 adult and pediatric VHL R200W homozygotes and 31 controls at outpatient facilities in Chuvashia, Russian Federation included echocardiography assessment of pulmonary artery pressure (tricuspid regurgitation velocity), cardiac volume, and systolic and diastolic function, as well as hematologic and clinical parameters. We determined the prevalence and risk factors for elevation of tricuspid regurgitation velocity in this population and its relationship to phlebotomy. ResultsThe age-adjusted mean ± SE tricuspid regurgitation velocity was higher in VHL R200W homozygotes than controls with normal VHL alleles (2.5±0.03 vs. 2.3±0.05 m/sec, P=0.005). The ageadjusted left ventricular diastolic diameter (4.8±0.05 vs. 4.5±0.09 cm, P=0.005) and left atrial diameter (3.4±0.04 vs. 3.2±0.08 cm, P=0.011) were also greater in the VHL R200W homozygotes, consistent with increased blood volume, but the elevation in tricuspid regurgitation velocity persisted after adjustment for these variables. Among VHL R200W homozygotes, phlebotomy therapy was associated with lower serum ferritin concentration, and low ferritin independently predicted higher tricuspid regurgitation velocity (standardized beta=0.29; P=0.009). ConclusionsChildren and adults with Chuvash polycythemia have higher estimated right ventricular systolic pressure, even after adjustment for echocardiography estimates of blood volume. Lower ferritin concentration, which is associated with phlebotomy, independently predicts higher tricuspid regurgitation velocity (www.clinicaltrials.gov identifier NCT00495638).Key words: pulmonary hypertension, tricuspid regurgitation velocity, ferritin, VHL, hypoxia inducible factor.Citation: Sable CA, Aliyu ZY, Dham N, Nouraie M, Sachdev V, Sidenko S, Miasnikova GY, Polyakova LA, Sergueeva AI, Okhotin DJ, Bushuev V, Remaley AT, Niu X, Castro OL, Gladwin MT, Kato GJ, Prchal10 JT, and Gordeuk VR. Pulmonary artery pressure and iron deficiency in patients with upregulation of hypoxia sensing due to homozygous VHLR200W mutation (Chuvash polycythemia). Haematologica 2012;97(2):193-200. doi:10.3324/haematol.2011

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Section: Introductionmentioning

confidence: 99%

“…[16][17] Iron therapy improved exercise capacity in such patients. 18 Congenital polycythemia is a rare disorder that occurs sporadically around the world. It is remarkable to find a large group of affected individuals with a single genotype and a homogeneous ethnic and environmental background, as is found in Chuvashia.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary artery pressure and iron deficiency in patients with upregulation of hypoxia sensing due to homozygous VHLR200W mutation (Chuvash polycythemia)

et al. 2011

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“…29,30 Venesection should only be offered to patients with moderate-to-severe hyperviscosity symptoms (Table 3) due to significant secondary erythrocytosis (Hb level well above 24 g/dL) in the absence of volume depletion/dehydration. 31 Also, venesection can be considered preoperatively for autologous blood donation if the hematocrit level is >65% and for boosting platelet production. 32 Iron deficiency is a common complication in patients with ES.…”

Section: General Management Of Eisenmenger Syndromementioning

confidence: 99%

Eisenmenger syndrome: current perspectives

Nashat¹,

Kempny²,

McCabe³

et al. 2017

RRCC

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Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). It results from a cardiac defect allowing significant systemic-to-pulmonary (left-to-right) shunting, which triggers the development of pulmonary vascular disease (PVD) if the defect is not repaired in a timely fashion. Once severe PVD has developed, the defect cannot be repaired. With advances in pediatric cardiology and surgery, the prevalence of ES is steadily falling in developed countries; nonetheless, there will always be patients who are unsuitable for repair at the time of diagnosis, or emigrating from countries with less advanced healthcare, who will develop ES. ES is a multisystem disorder causing chronic hypoxemia and reduced cardiac output resulting in significant morbidity and mortality. While lung (plus defect repair) or combined heart and lung transplantation is thought be the definitive treatment for ES, transplant organs are a limited resource and long-term results are still suboptimal. PAH pharmacotherapy was, until quite recently, largely directed at symptomatic relief and had no impact on morbidity and mortality. Targeted PAH therapies have recently been proven to be beneficial in various forms of PAH in terms of functional status, progression of disease, and prognosis. Data on the effect of PAH therapies in the ES cohort remain limited, but available studies demonstrate evidence of improvement in symptoms, exercise capacity, and some evidence of survival benefit. ES patients should be followed in specialized centers, by means of an interdisciplinary approach by clinicians experienced in PAH and CHD. However, local physicians working in cardiology, respiratory medicine, primary care, and emergency services are likely to encounter ES patients and need to be aware of the main issues and pitfalls in their care. The authors present an overview of the management of ES, focusing on the most common issues and complications.

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“…Iron deficiency should be avoided in CCHD, with oral iron supplementation as needed to normalize ferritin levels remaining mindful of an overly robust rise in hematocrit [7]. Although physicians are often uncomfortable administering iron in patients with high hematocrit, supplementation has been shown to improve exercise capacity and quality of life in iron-deficient cyanotic patients without increasing hyperviscosity risk [69,72].…”

Section: Hematologic Consequences Of Cchdmentioning

confidence: 99%

Extra-cardiac manifestations of adult congenital heart disease

Gaeta

Ward

Krasuski

2016

Trends in Cardiovascular Medicine

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a b s t r a c tAdvancement in correction or palliation of congenital cardiac lesions has greatly improved the lifespan of congenital heart disease patients, resulting in a rapidly growing adult congenital heart disease (ACHD) population. As this group has increased in number and age, emerging science has highlighted the systemic nature of ACHD. Providers caring for these patients are tasked with long-term management of multiple neurologic, pulmonary, hepatic, renal, and endocrine manifestations that arise as syndromic associations with congenital heart defects or as sequelae of primary structural or hemodynamic abnormalities. In this review, we outline the current understanding and recent research into these extra-cardiac manifestations.Key words: Cardiovascular disease, Adult congenital heart disease, Pulmonary hypertension. Advances in the diagnosis and management of congenital heart disease (CHD) over the past several decades have greatly improved longevity and quality of life in the CHD population. Palliative or corrective interventions are now available for nearly every type of congenital cardiac lesion, and approximately 90% of affected children now survive to adulthood [1]. Adults with CHD (ACHD) already outnumber pediatric CHD patients, and this population is expected to grow by 5% per year [1,2]. Strikingly, the median age of severe CHD patients has increased from 11 years in 1985 to 25 years in 2010 [3]. Continued improvement in CHD interventions means that new (and more complex) populations will likely reach adulthood in the coming years.Practitioners who take care of adults with CHD are often charged with the delivery or coordination of comprehensive care for these complex patients, including the management of manifestations and complications that arise later in life. ACHD has many extra-cardiac effects, both as syndromic associations with congenital heart defects, or more commonly, as sequelae of primary structural or hemodynamic abnormalities. The role of genetic predilection remains undefined for nearly all extra-cardiac complications, though their incidence has clearly risen over the past decades. Indeed, more than a third of hospital admissions for ACHD patients are now for noncardiovascular reasons [4]. Optimal care of ACHD patients, therefore, mandates a thorough understanding of its multiorgan system manifestations. Herein, we will review current understanding of the most common manifestations of ACHD organized by the affected organ systems. Neurologic/neuropsychiatric Cerebrovascular complicationsA recent study quantified the strikingly high rates of stroke in ACHD [5]. Among nearly 30,000 ACHD patients, 8.9% of men http://dx

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Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome

Cited by 118 publications

References 37 publications

Pulmonary artery pressure and iron deficiency in patients with upregulation of hypoxia sensing due to homozygous VHLR200W mutation (Chuvash polycythemia)

Pulmonary artery pressure and iron deficiency in patients with upregulation of hypoxia sensing due to homozygous VHLR200W mutation (Chuvash polycythemia)

Eisenmenger syndrome: current perspectives

Extra-cardiac manifestations of adult congenital heart disease

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