Cerebrovascular and cardiovascular disease burden in patients with hereditary hemorrhagic telangiectasia

Farooqui, Mudassir; Suriya, Sajid; Qeadan, Fares; Vigil, Catherine; Wegele, Ashley; Agrebi, I.; Quadri, Syed A.; Robinson, Myranda; Rodriguez-Lopez, Josanna; Ortega‐Gutiérrez, Santiago; Zafar, Atif

doi:10.1007/s10072-021-05135-z

Cited by 3 publications

(1 citation statement)

References 23 publications

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“…Left‐sided heart disease (clinical group 2) or lung diseases and/or hypoxia (clinical group 3) are the most common etiologies of PH in the general population; while likely under‐reported, signs and symptoms of these pathologies are also prevalent in a significant portion of patients with HHT. 33 , 109 , 110 Left‐sided heart disease as well as restrictive and obstructive lung disease have been observed in patients with HHT and PH. 34 Patients with HHT are at an elevated risk of myocardial infarction due to paradoxical embolism through pulmonary AVMs, leading to subsequent cardiomyopathy.…”

Section: Additional Etiologies Of Pulmonary Hypertension In Hereditar...mentioning

confidence: 99%

Pulmonary hypertension in hereditary hemorrhagic telangiectasia: A clinical review

Mathavan,

Reddy

et al. 2023

Pulm. circ.

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Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant hereditary disorder characterized by recurrent spontaneous epistaxis, mucocutaneous telangiectasias, and solid organ arteriovenous malformations (AVMs). Pulmonary hypertension (PH) is an increasingly recognized complication in patients with HHT, most often precipitated by high‐output heart failure in the presence of hepatic AVMs as well as pulmonary arterial hypertension in the form of a proliferative vasculopathy. The presence of PH in patients with HHT is associated with significant elevations in rates of morbidity and mortality. Additionally, there is growing recognition of a thromboembolic propensity in this population that increases the risk of chronic thromboembolic PH, posing unique clinical considerations regarding the use of anticoagulation. Patients with HHT are also at risk of PH due to disorders commonly seen in the general population, including left‐sided heart and lung disease. The etiology of PH in HHT is multifaceted and complex; the diagnostic approach and treatment strategies must consider the underlying pathophysiology of HHT. This comprehensive review summarizes current knowledge of PH in HHT, detailing the pathogenesis of known etiologies, diagnostic evaluation, and suggested treatment modalities as well as emerging therapies that may be of future interest.

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Section: Additional Etiologies Of Pulmonary Hypertension In Hereditar...mentioning

confidence: 99%

Pulmonary hypertension in hereditary hemorrhagic telangiectasia: A clinical review

Mathavan,

Reddy

et al. 2023

Pulm. circ.

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Neurologic conditions in Hereditary Hemorrhagic Telangiectasia with pulmonary arteriovenous malformations: Database study

Lau

Agarwal

Vandermeer

et al. 2023

Canadian Journal of Respiratory, Critical Care, and Sleep Medic

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An Unusual Cause of Right Heart Dysfunction and High Output Heart Failure in a Young Woman

Ordoñez

Marín

Crespo

et al. 2022

JCDD

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A 35-year-old female presented to our emergency department with clinical signs of acute heart failure. Clinical workup identified severe right heart (RH) dilation and dysfunction with a crossing membrane structure in the right atrium. Right heart catheterization confirmed high output heart failure (HOHF), pulmonary hypertension (PH), and left-to-right blood shunting followed by the documentation of multiple liver and pulmonary arteriovenous malformations (AVMs). Hereditary Hemorrhagic Telangiectasia (HHT) diagnosis was made according to clinical criteria and was established as the cause of HOHF because of chronic volume overload from systemic to pulmonary shunts. With this illustrative case, we aim to discuss the broad spectrum of clinical manifestations of HHT and the unusual phenotype of HOHF secondary to HHT. This case also highlights the broad diagnosis of atrial echocardiographic abnormalities and cardiac structural distortion secondary to high output that can be misleading at imaging evaluation.

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Cerebrovascular and cardiovascular disease burden in patients with hereditary hemorrhagic telangiectasia

Cited by 3 publications

References 23 publications

Pulmonary hypertension in hereditary hemorrhagic telangiectasia: A clinical review

Pulmonary hypertension in hereditary hemorrhagic telangiectasia: A clinical review

Neurologic conditions in Hereditary Hemorrhagic Telangiectasia with pulmonary arteriovenous malformations: Database study

An Unusual Cause of Right Heart Dysfunction and High Output Heart Failure in a Young Woman

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