Erythrocyte indexes, iron metabolism, and hyperhomocysteinemia in adults with cyanotic congenital cardiac disease

Kaemmerer, Harald; Fratz, Sohrab; Braun, Siegfried; Koelling, Klaus; Eicken, Andreas; Brodherr-Heberlein, Silke; Pietrzik, Klaus; Hess, John

doi:10.1016/j.amjcard.2004.06.014

Cited by 62 publications

(47 citation statements)

References 4 publications

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“…In another sample of patients with Eisenmenger syndrome, 37% were iron deficient (ferritin level <20 ng/mL). 198,199 Pathogenesis Secondary erythrocytosis is an important adaptation to chronic cyanosis that allows adequate oxygen delivery in the setting of decreased oxygen tension. 200 In patients with adequate iron stores, the degree of erythrocytosis is inversely related in a linear fashion to the average oxygen saturation.…”

Section: Cyanotic Chd: Secondary Erythrocytosis and Iron Deficiencymentioning

confidence: 99%

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Lui¹,

Saïdi²,

Bhatt³

et al. 2017

Circulation

177

136

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ABSTRACT:Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

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Section: Cyanotic Chd: Secondary Erythrocytosis and Iron Deficiencymentioning

confidence: 99%

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Lui¹,

Saïdi²,

Bhatt³

et al. 2017

Circulation

177

136

View full text Add to dashboard Cite

show abstract

“…Instead, patients often present with hyperchromia and/or macrocytosis possibly related to coexistent folate or vitamin B deficiency. 76 Hypoglycemia is not uncommon and usually represents an artificially low blood glucose level due to augmented in vitro glycolysis as an effect of the increased red cell mass. This potentially misleading result can be avoided by adding sodium fluoride in the laboratory tube.…”

Section: Routine Laboratory Testingmentioning

confidence: 99%

Pulmonary Vascular Disease in Adults With Congenital Heart Disease

2007

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Abstract-Pulmonary arterial hypertension of variable degree is commonly associated with adult congenital heart disease.Depending on size and location of the underlying cardiac defect as well as on repair status, pulmonary arterial hypertension may present with or without reversed shunting and associated cyanosis (ie, Eisenmenger syndrome). We review available data on etiology, clinical presentation, prognosis, and management strategies of pulmonary arterial hypertension in adult patients with congenital heart disease. In addition, we discuss the numerous complications associated with Eisenmenger syndrome, representing a multisystem disorder. Finally, we present general management strategies and emerging disease-targeting therapies.

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“…More than a third of CCHD patients are iron deficient [68][69][70]. Typical indices of iron deficiency including hypochromia and microcytosis are often absent; exclusion of iron deficiency, therefore, requires a complete iron metabolism workup, including serum ferritin and transferrin levels [68].…”

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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Erythrocyte indexes, iron metabolism, and hyperhomocysteinemia in adults with cyanotic congenital cardiac disease

Cited by 62 publications

References 4 publications

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Pulmonary Vascular Disease in Adults With Congenital Heart Disease

Extra-cardiac manifestations of adult congenital heart disease

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