High-Output Heart Failure Contributing to Recurrent Epistaxis Kiesselbach Area Syndrome in a Patient With Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare genetic blood disorder that leads to abnormal bleeding due to absent capillaries and multiple abnormal blood vessels known as arteriovenous malformations. A feature of HHT is high-output heart failure due to multiple arteriovenous malformations. High-output heart failure can lead to recurrent epistaxis Kiesselbach area syndrome (REKAS), further exacerbating heart failure through increased blood loss and resultant anemia. We report a patient with HHT who presented with high-output heart failure contributing to REKAS. In patients with REKAS, we propose if anemia is present, REKAS can be avoided by correcting the anemia by increasing the hemoglobin level to greater than 9 to 10 g/dL. This decreases hyperdynamic circulation and reduces pressure in the blood vessels of the nose.

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Severe Hypoxemia Caused by High-Output Heart Failure and Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia

JACC: Case Reports ◽

10.1016/j.jaccas.2021.10.008 ◽

2021 ◽

Vol 3 (17) ◽

pp. 1863-1868

Author(s):

Hiroaki Yamamoto ◽

Chieko Itamoto ◽

Tomomi Yamaguchi ◽

Tomoki Koshyo

Keyword(s):

Heart Failure ◽

Hereditary Hemorrhagic Telangiectasia ◽

Arteriovenous Malformations ◽

Pulmonary Arteriovenous Malformations ◽

Severe Hypoxemia ◽

High Output Heart Failure ◽

High Output

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Predictors of heart failure symptoms in hereditary hemorrhagic telangiectasia patients with hepatic arteriovenous malformations

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-021-02109-4 ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Lucas R. Cusumano ◽

Joseph A. Tesoriero ◽

Craig B. Wilsen ◽

James Sayre ◽

Matthew Quirk ◽

...

Keyword(s):

Heart Failure ◽

Hepatic Artery ◽

Hereditary Hemorrhagic Telangiectasia ◽

Arteriovenous Malformations ◽

Multiple Logistic Regression Analysis ◽

Laboratory Data ◽

Hemoglobin Level ◽

Hepatic Involvement ◽

Asymptomatic Patients ◽

High Output Heart Failure

Abstract Background Hepatic arteriovenous malformations (AVMs) in hereditary hemorrhagic telangiectasia (HHT) patients are most commonly hepatic artery to hepatic venous shunts which can result in high-output heart failure. This condition can be debilitating and is a leading cause of liver transplantation in HHT patients. However, it is not known what characteristics can discriminate between asymptomatic patients and those who will develop heart failure symptoms. Results 176 patients with HHT were evaluated with computed tomography angiography (CTA) between April 2004 and February 2019 at our HHT Center of Excellence. 63/176 (35.8%) patients were found to have hepatic AVMs on CTA. 18 of these patients were excluded because of the presence of another condition which could confound evaluation of heart failure symptoms. In the remaining 45 patients included in our cohort, 25/45 (55.6%) patients were classified as asymptomatic and 20/45 (44.4%) were classified as symptomatic, and these groups were compared. In symptomatic patients, mean common hepatic artery (CHA) diameter was significantly higher (11.1 versus 8.4 mm) and mean hemoglobin levels were significantly lower (10.7 vs 12.6 g/dL). A stepwise multiple logistic regression analysis demonstrated that both CHA diameter and hemoglobin level were independent predictors of heart failure symptoms with ORs of 2.554 (95% CI 1.372–4.754) and 0.489 (95% CI 0.299–0.799), respectively. The receiver operator characteristic (ROC) curve of our analysis demonstrated an AUC of 0.906 (95% CI 0.816–0.996), sensitivity 80.0% (95% CI 55.7–93.4%), and specificity 75.0% (95% CI 52.9–89.4%). Conclusions CTA is an effective and easily reproducible method to evaluate hepatic involvement of HHT. Utilizing CTA, clinical, and laboratory data we determined CHA diameter and hemoglobin level were independent predictors of heart failure symptoms.

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Predictors of Heart Failure Symptoms in Hereditary Hemorrhagic Telangiectasia Patients With Hepatic Arteriovenous Malformations

10.21203/rs.3.rs-824554/v1 ◽

2021 ◽

Author(s):

Lucas Russell Cusumano ◽

Joseph A Tesoriero ◽

Craig B Wilsen ◽

James Sayre ◽

Matthew Quirk ◽

...

Keyword(s):

Heart Failure ◽

Hepatic Artery ◽

Hereditary Hemorrhagic Telangiectasia ◽

Arteriovenous Malformations ◽

Multiple Logistic Regression Analysis ◽

Laboratory Data ◽

Hemoglobin Level ◽

Hepatic Involvement ◽

Asymptomatic Patients ◽

High Output Heart Failure

Abstract BackgroundHepatic arteriovenous malformations (AVMs) in hereditary hemorrhagic telangiectasia (HHT) patients are most commonly hepatic artery to hepatic venous shunts which can result in high-output heart failure. This condition can be debilitating and is a leading cause of liver transplantation in HHT patients. However, it is not known what characteristics can discriminate between asymptomatic patients and those who will develop heart failure symptoms.Results176 patients with HHT were evaluated with computed tomography angiography (CTA) between April 2004 and February 2019 at our HHT Center of Excellence. 63/176 (35.8%) patients were found to have hepatic AVMs on CTA. 18 of these patients were excluded because of the presence of another condition which could confound evaluation of heart failure symptoms. In the remaining 45 patients included in our cohort, 25/45 (55.6%) patients were classified as asymptomatic and 20/45 (44.4%) were classified as symptomatic, and these groups were compared. In symptomatic patients, mean common hepatic artery (CHA) diameter was significantly higher (11.1 versus 8.4 mm) and mean hemoglobin levels were significantly lower (10.7 vs 12.6 g/dL). A stepwise multiple logistic regression analysis demonstrated that both CHA diameter and hemoglobin level were independent predictors of heart failure symptoms with ORs of 2.554 (95% CI 1.372-4.754) and 0.489 (95% CI 0.299-0.799), respectively. The receiver operator characteristic (ROC) curve of our analysis demonstrated an AUC of 0.906 (95% CI 0.816–0.996), sensitivity 80.0% (95% CI 55.7-93.4%), and specificity 75.0% (95% CI 52.9-89.4%).ConclusionsCTA is an effective and easily reproducible method to evaluate hepatic involvement of HHT. Utilizing CTA, clinical, and laboratory data we determined CHA diameter and hemoglobin level were independent predictors of heart failure symptoms.

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