Eisenmenger Syndrome: A Multisystem Disorder—Do Not Destabilize the Balanced but Fragile Physiology

Chaix, Marie-A; Diller, Gerhard‐Paul; Khairy, Paul; Oechslin, Erwin

doi:10.1016/j.cjca.2019.10.002

Cited by 34 publications

(43 citation statements)

References 75 publications

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“…ES associated with haematological abnormalities promote a complex paradox with increased risk of bleeding and thrombosis. Currently, there is no recommendation for anticoagulation treatment, except for special cases of arrhythmias and thromboembolic events, which are very rare in paediatric ES [ 97 , 105 , 106 ].…”

Section: Group 1 Ph ( Table 1 )mentioning

confidence: 99%

Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist

2020

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Pulmonary hypertensive vascular disease (PHVD), and pulmonary hypertension (PH), which is a broader term, are severe conditions associated with high morbidity and mortality at all ages. Treatment guidelines in childhood are widely adopted from adult data and experience, though big differences may exist regarding aetiology, concomitant conditions and presentation. Over the past few years, paediatric aspects have been incorporated into the common guidelines, which currently address both children and adults with pulmonary hypertension (PH). There are multiple facets of PH in the context of cardiac conditions in childhood. Apart from Eisenmenger syndrome (ES), the broad spectrum of congenital heart disease (CHD) comprises PH in failing Fontan physiology, as well as segmental PH. In this review we provide current data and novel aspects on the pathophysiological background and individual management concepts of these conditions. Moreover, we focus on paediatric left heart failure with PH and its challenging issues, including end stage treatment options, such as mechanical support and paediatric transplantation. PH in the context of rare congenital disorders, such as Scimitar Syndrome and sickle cell disease is discussed. Based on current data, we provide an overview on multiple underlying mechanisms of PH involved in these conditions, and different management strategies in children and adulthood. In addition, we summarize the paediatric aspects and the pros and cons of the recently updated definitions of PH. This review provides deeper insights into some challenging conditions of paediatric PH in order to improve current knowledge and care for children and young adults.

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Section: Group 1 Ph ( Table 1 )mentioning

confidence: 99%

Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist

2020

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“…The first of these groups include the most frequent aetiologies at paediatric stage, which are congenital heart diseases with left-to-right shunt and increased pulmonary overload, which leads to pre-capillary antegrade PH [4]. Heath and Edwards developed a histopathological classification of PH [5] in six grades, of which the last three usually show irreversible tissue damage and clinically present as Eisenmenger syndrome [6]. This syndrome is characterised by the installation of cardiopulmonary failure and right-to-left (inverted) shunting, with subsequent clinical onset of cyanosis.…”

Section: Introductionmentioning

confidence: 99%

Hyperbaric oxygen effects on the alveoli-capillary unit in a murine model of pulmonary arterial hypertension

et al. 2020

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Introduction: Pulmonary hypertension (PH) is an increase in the normal value of systolic pressure of the pulmonary artery, frequently due to congenital heart diseases being its treatment the correction of this defects; alternatively, hyperbaric oxygen (HBO) has shown beneficial effects facilitating neovascularisation and decreased vascular resistance. An experimental study was designed to determine the effect of HBO on the alveoli-capillary unit in a murine model of irreversible pulmonary hypertension (IPAH) induced with monocrotaline. Material and methods: Four groups of 10 Wistar rats each were considered: A (control), B (HBO), C (IPAH), and D (IPAH+HBO). HBO was administered at 2 atm, 1 h daily, for 15 days. At the end of this time, systolic pulmonary artery pressure (PAP) was measured, followed by histologically quantification of the number of cells, vascular buttons, and neoformation vessels employing the immunochemistry detection of hypoxia-induced factor-1 (HIF-1), vascular endothelial growth factor (VEGF), and CD34, respectively. Results: Histologically, this matches with an increase of the average number of positive HIF-1 cells, and an increase in VEGF-positive vascular buttons in group D rats compared with those of group C. The number of CD34-positive neovessels was similar in groups C and D, but in the latter group the number of capillary vessels was more significant. Conclusions: This suggests that angiogenesis and vasculogenesis were induced by HIF-1, and it has been achieved due to an early effect of HBO therapy. Presumably, neovessels were formed between days 5 and 10 following HBO therapy, but more experimental studies are required to test this hypothesis.

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“…Eisenmenger syndrome refers to the sequelae of untreated congenital heart defects (CHD) that lead to pulmonary hypertension [1][2][3]. Due to prolonged left-to-right shunt caused by a CHD, right-sided pulmonary pressures increase along with pulmonary vascular resistance, which causes shunt reversal leading to cyanosis [4].…”

Section: Introductionmentioning

confidence: 99%

Secundum Atrial Septal Defect With Early Presentation of Eisenmenger Syndrome and Right-Heart Failure: A Rare Case Report and Literature Review

Malik¹,

Ikram²,

Kamal³

et al. 2020

Cureus

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Eisenmenger syndrome, the most advanced form of pulmonary arterial hypertension (PAH), poses a considerable risk to the survival and quality of life of patients. It is more commonly seen in large intra-cardiac defects like ventricular septal defects (VSD) or patent ductus arteriosus (PDA), and rarely in atrial septal defects (ASD). Early diagnosis is the single most important step in the definitive management of the condition; otherwise, only conservative treatment can be offered. In this report, we present the case of a 20-year-old female patient diagnosed with Eisenmenger syndrome secondary to a large secundum ASD. The patient responded well to medical treatment.

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Eisenmenger Syndrome: A Multisystem Disorder—Do Not Destabilize the Balanced but Fragile Physiology

Cited by 34 publications

References 75 publications

Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist

Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist

Hyperbaric oxygen effects on the alveoli-capillary unit in a murine model of pulmonary arterial hypertension

Secundum Atrial Septal Defect With Early Presentation of Eisenmenger Syndrome and Right-Heart Failure: A Rare Case Report and Literature Review

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