Iron deficiency is associated with adverse outcome in Eisenmenger patients

Bruaene, Alexander Van De; Delcroix, Marion; Pasquet, Agnès; Backer, Julie De; Pauw, Michel De; Naeije, Robert; Vachiéry, Jean‐Luc; Paelinck, Bernard P.; Morissens, Marielle; Budts, Werner

doi:10.1093/eurheartj/ehr130

Cited by 83 publications

(53 citation statements)

References 30 publications

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“…In all of these entities, preliminary data indicate that iron deficiency may be associated with reduced exercise capacity, and perhaps also with a higher mortality, independent of the presence or severity of anaemia [181,182,184,185]. Based on these data, regular monitoring of the iron status should be considered in patients with PAH and detection of an iron deficiency should trigger a search for potential reasons.…”

Section: Anaemia and Iron Statusmentioning

confidence: 99%

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

et al. 2015

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Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.

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Section: Anaemia and Iron Statusmentioning

confidence: 99%

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

et al. 2015

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“…If moderate-to-severe symptoms of hyperviscosity are present, and iron deficiency and dehydration have been excluded, phlebotomy with isovolumic replacement should be performed carefully when the haematocrit is .65% [38]. Iron deficiency has been shown to be associated with a higher risk of adverse outcomes (allcause mortality, transplantation and hospitalisation due to cardiopulmonary causes) in Eisenmenger's syndrome patients [39] and iron replacement therapy improves exercise tolerance and QoL [40]. However, care should be taken in patients with low oxygen saturations to avoid haemoglobin levels becoming too high [39].…”

Section: General Management Of Pah-chdmentioning

confidence: 99%

“…Iron deficiency has been shown to be associated with a higher risk of adverse outcomes (allcause mortality, transplantation and hospitalisation due to cardiopulmonary causes) in Eisenmenger's syndrome patients [39] and iron replacement therapy improves exercise tolerance and QoL [40]. However, care should be taken in patients with low oxygen saturations to avoid haemoglobin levels becoming too high [39]. There are no data to support the use of calcium channel blockers in patients with PAH-CHD and their use must be avoided [3].…”

Section: General Management Of Pah-chdmentioning

confidence: 99%

Pulmonary arterial hypertension associated with congenital heart disease

2012

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Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD), with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD.The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges.This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.

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“…A study of 68 patients by Van De Bruaene et al found that patients with resting oxygen saturations of <83% often reached Hb concentrations in the range that may be considered "at risk" for hyperviscosity symptoms when treated for iron deficiency and suggested closer monitoring for hyperviscosity symptoms in this group. 34 As iron deficiency is strongly related to adverse outcomes, particularly in those on anticoagulation or those having inappropriate venesection, it is important to regularly screen patients for this and treat when appropriate.…”

Section: General Management Of Eisenmenger Syndromementioning

confidence: 99%

“…OACs are associated with an increased risk of iron deficiency anemia and patients should be routinely investigated for this. 34 Also, endothelin receptor antagonists (ERAs) can reduce the efficacy of anticoagulants such as warfarin; therefore, necessitate close monitoring of the international normalized ratio is needed to maintain an adequate therapeutic index. Novel OACs have not been tested in this group and there are concerns regarding the limited availability of direct reversing agents.…”

Section: Oxygen Therapymentioning

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 is associated with adverse outcome in Eisenmenger patients

Cited by 83 publications

References 30 publications

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Pulmonary arterial hypertension associated with congenital heart disease

Eisenmenger syndrome: current perspectives

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