Heart failure in the adult Ebstein patient

Schultz, K.; Haeffele, Christiane

doi:10.1007/s10741-020-09930-2

Cited by 8 publications

(8 citation statements)

References 53 publications

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“…Ebstein's anomaly can present at any age and tends to be severe in younger patients and mild in older patients. However, even in milder forms, patients often eventually develop easy fatigability and right heart failure, as in the present case ( 7 ). Our patient had not developed overt heart failure until this admission, probably because she had no other cardiac defects and only developed hypertension very recently.…”

Section: Discussionsupporting

confidence: 55%

Moyamoya Disease Complicating Ebstein's Anomaly

Goto¹,

Okamoto

Sato

et al. 2022

Intern. Med.

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Ebstein's anomaly is an uncommon congenital disorder affecting the tricuspid valve. We herein report a 38-year-old woman who experienced consciousness and sensory disturbance during treatment for heart failure caused by Ebstein's anomaly. Urgent magnetic resonance imaging and cerebral angiography demonstrated acute cerebral infarction and internal carotid artery obstruction with the development of collateral arteries. We diagnosed her with multiple cerebral infarctions due to moyamoya disease. Ebstein's anomaly concomitant with moyamoya disease is extremely rare. However, we should consider the possibility of this rare but important concurrence when treating patients with heart failure due to Ebstein's anomaly to avoid excessive diuresis and vasodilation and irreversible brain injury.

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Section: Discussionsupporting

confidence: 55%

Moyamoya Disease Complicating Ebstein's Anomaly

Goto¹,

Okamoto

Sato

et al. 2022

Intern. Med.

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“…Furthermore, additional structural abnormalities such as valve regurgitation may be common risk factors for both AF and HF. Lastly, AF may also represent a sign of deteriorating haemodynamics 27. Therefore, it is of utmost importance to identify potential reversible causes and to develop management strategies that interrupt this vicious circle before it becomes irreversible.…”

Section: Discussionmentioning

confidence: 99%

“…Specifically, AF was independently associated with HF hospitalisation. The pathophysiology of HF in EA is multifactorial and derives from complex interactions between the structurally and haemodynamically abnormal left and right chambers 27. Furthermore, the role of the left heart in HF development is expected to augment due to increased survival and accordingly higher prevalence of comorbidities.…”

Section: Discussionmentioning

confidence: 99%

Prognostic implications of atrial fibrillation in adults with Ebstein anomaly

Miguel

Madhavan

Miranda

et al. 2022

Heart

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ObjectiveSupraventricular arrhythmias are common in adults with Ebstein anomaly (EA). However, there are limited data about prognostic implications of atrial fibrillation (AF) in this population. Accordingly, our aim was to assess the clinical profile and burden of AF in adults with EA, and the relationship between AF and outcomes.Methods and resultsSix hundred eighty-two consecutive adults with a median age of 36 (24–49) years from Mayo Clinic, Minnesota, USA, between 2003 and 2020 were included. Sustained episodes of AF, clinical, echocardiographic, rhythm and surgical data were collected. Prevalence of AF at baseline was 18% (126 patients); the first episode occurred at a mean age of 43±17 years. Patients with AF were older, were more likely men, and had hypertension, renal dysfunction, cardiac devices, and more advanced right-sided and left-sided remodelling. During a median follow-up of 156 (81–240) months, 62 patients (11%) developed incident AF. At the last encounter, prevalence of AF was 28% (188 patients); of those, 63 (34%) had recurrent AF. Hospitalisation for heart failure (HF) occurred in 51 patients (7%). AF (HR 2.32, 95% CI 1.18 to 4.47; p=0.01) was independently associated with hospitalisation for HF. All-cause death occurred in 53 patients (8%); it was more frequent in those with AF in the univariable analysis, although it did not remain significant in the multivariable analysis.ConclusionsAF in EA develops at relatively young ages with one-third of the cohort exhibiting a recurrent pattern. Patients with AF had a higher prevalence of comorbidities and worse right-sided and left-sided cardiac remodelling. AF was independently associated with HF hospitalisation.

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“…44 Experimental heart failure in rats, canines, and rabbits produces carotid chemoreflex hypersensitivity leading to increased tonic levels of sympathetic outflow under normoxic conditions, enhanced sympathetic and ventilatory responses to acute hypoxemia, and vasoconstriction of locomotor muscle vasculature during exercise. 45 46 This form of CB plasticity is critically dependent on increases in locally produced angiotensin II, upregulation of angiotensin II type 1 receptors, increased expression of NADPH oxidase subunits, and enhanced superoxide production. 47 Thus, the CHF-induced increase in CB sensitivity is caused by a shift in redox balance toward oxidative stress.…”

Section: Chemoreceptorsmentioning

confidence: 99%

Control of Breathing

Dempsey

Welch

2023

Semin Respir Crit Care Med

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Substantial advances have been made recently into the discovery of fundamental mechanisms underlying the neural control of breathing and even some inroads into translating these findings to treating breathing disorders. Here, we review several of these advances, starting with an appreciation of the importance of V̇A:V̇CO2:PaCO2 relationships, then summarizing our current understanding of the mechanisms and neural pathways for central rhythm generation, chemoreception, exercise hyperpnea, plasticity, and sleep-state effects on ventilatory control. We apply these fundamental principles to consider the pathophysiology of ventilatory control attending hypersensitized chemoreception in select cardiorespiratory diseases, the pathogenesis of sleep-disordered breathing, and the exertional hyperventilation and dyspnea associated with aging and chronic diseases. These examples underscore the critical importance that many ventilatory control issues play in disease pathogenesis, diagnosis, and treatment.

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Heart failure in the adult Ebstein patient

Cited by 8 publications

References 53 publications

Moyamoya Disease Complicating Ebstein's Anomaly

Moyamoya Disease Complicating Ebstein's Anomaly

Prognostic implications of atrial fibrillation in adults with Ebstein anomaly

Control of Breathing

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