AJR Teaching File: High-Output Cardiac Failure in a Patient with a History of Hereditary Hemorrhagic Telangiectasia

High-output cardiac failure secondary to hepatic involvement is a rare complication of hereditary hemorrhagic telangiectasia (HHT). Here we report a 43-year-old woman who presented at 29 weeks gestation of her second pregnancy with complications of right-sided heart failure and preterm labor. After delivery via cesarean section, the patient was found to have intrahepatic arteriovenous malformations through non-invasive imaging. Subsequently, a family history of vascular malformations and epistaxis was elucidated and a diagnosis of HHT was made. This case is presented, along with a review of the literature and discussion of hepatic involvement in HHT with particular focus on the pregnant patient.

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High-Output Cardiac Failure Due to Hereditary Hemorrhagic Telangiectasia: A Case of an Extra-Cardiac Left to Right Shunt

International Journal of Angiology ◽

10.1055/s-0035-1568878 ◽

2016 ◽

Vol 26 (02) ◽

pp. 125-129

Author(s):

Patrick Wu ◽

Amber Horwith ◽

Stephanie Mai ◽

Milind Parikh ◽

Gaurav Tyagi ◽

...

Keyword(s):

Cardiac Failure ◽

Hereditary Hemorrhagic Telangiectasia ◽

Arteriovenous Malformations ◽

Rare Complication ◽

Differential Diagnoses ◽

Cardiac Manifestations ◽

High Output

AbstractHigh-output cardiac failure is a rare complication of hereditary hemorrhagic telangiectasia and can potentially be mistaken for other entities. We present a case of high-output cardiac failure because of large hepatic arteriovenous malformations, review the literature regarding the cardiac manifestations of the disease, and discuss the possible differential diagnoses.

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Systemic bevacizumab for high-output cardiac failure in hereditary hemorrhagic telangiectasia: an international survey of HHT centers

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-019-1239-6 ◽

2019 ◽

Vol 14 (1) ◽

Cited By ~ 5

Author(s):

Hanny Al-Samkari ◽

Hasan A. Albitar ◽

Scott E. Olitsky ◽

Marianne S. Clancy ◽

Vivek N. Iyer

Keyword(s):

Adverse Events ◽

Cardiac Failure ◽

Hereditary Hemorrhagic Telangiectasia ◽

Current Treatment ◽

Induction Treatment ◽

Published Data ◽

Centers Of Excellence ◽

Treatment Practices ◽

Angiogenic Therapy ◽

High Output

Abstract Background Systemic bevacizumab is a novel targeted anti-angiogenic therapy for high-output cardiac failure (HOCF) in hereditary hemorrhagic telangiectasia (HHT) but published data is limited. This survey-based study measured physician-reported safety, effectiveness and current treatment practices for systemic bevacizumab in HHT-HOCF. Methods A 27-item survey was sent to center directors of 31 international HHT Centers of Excellence. Results Response rate was 74% with centers reporting 150 total patients receiving systemic bevacizumab for HHT-HOCF. Approximately two-thirds of centers had treated ≥5 patients. All centers utilize a 5 mg/kg dose for induction treatment and most administer 6 doses (range, 4–6) every 2 weeks, although maintenance regimens varied considerably. Center directors reported bevacizumab to be effective, with 55% reporting significant improvement in cardiac index and HOCF symptoms in most patients treated with bevacizumab, although normalization of cardiac parameters was uncommon. Adverse events were uncommon with three-quarters of centers reporting adverse event rates < 10%. Discontinuation for adverse events or ineffectiveness was rare. Bevacizumab was typically administered by hematologists and pulmonologists (50 and 39% of centers, respectively), with highly variable thresholds for initiation. Although half the centers reported difficulty with the insurance approval process, 70% of centers were ultimately able to obtain coverage for most or all of their patients. Conclusions Systemic bevacizumab is a widely-used therapy for HHT-HOCF with reasonable safety and effectiveness. HHT centers appear to vary considerably in maintenance treatment practices and disease severity thresholds for initiation of bevacizumab in HHT-related HOCF.

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High Output Cardiac Failure Resolving after Repair of AV Fistula in a Six-Month-Old

Case Reports in Vascular Medicine ◽

10.1155/2016/8564081 ◽

2016 ◽

Vol 2016 ◽

pp. 1-4

Author(s):

Uygar Teomete ◽

Rubee Anne Gugol ◽

Holly Neville ◽

Ozgur Dandin ◽

Ming-Lon Young

Keyword(s):

Blood Pressure ◽

Heart Rate ◽

Pulmonary Hypertension ◽

High Risk ◽

Cardiac Failure ◽

Pulmonary Artery Hypertension ◽

Hemodynamic Effect ◽

Av Fistula ◽

History Of ◽

High Output

Background. Acquired AVF in pediatrics are commonly caused by iatrogenic means, including arterial or venous punctures. These fistulae can cause great hemodynamic stress on the heart as soon as they are created.Case. A six-month-old 25-week gestation infant was referred for respiratory distress. Initial exam revealed tachypnea, tachycardia, and hypertension. There was a bruit noted on her left arm. An ultrasound showed an arteriovenous fistula. Its location, however, precluded intervention because of the high risk for limb-loss. An echocardiogram showed evidence of pulmonary hypertension that was treated with sildenafil and furosemide. However, no improvement was seen. On temporary manual occlusion of the fistula, the patient was noted to have increased her blood pressure and decreased her heart rate, suggesting significant hemodynamic effect of the fistula. The fistula was subsequently ligated and the patient clinically and echocardiographically improved.Conclusion. A patient in high output cardiac failure or pulmonary artery hypertension, especially prematüre patients with preexisting lung disease, should be probed for history of multiple punctures, trauma, or surgery and should have prompt evaluation for AVF. If it can be diagnosed and repaired, most of the cases have been shown to decrease the stress on the heart and reverse the pathologic hemodynamics.

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High output cardiac failure in 3 patients with hereditary hemorrhagic telangiectasia and hepatic vascular malformations, evaluation of treatment

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-020-01583-6 ◽

2020 ◽

Vol 15 (1) ◽

Author(s):

Lilian B. Olsen ◽

Anette D. Kjeldsen ◽

Mikael K. Poulsen ◽

Jens Kjeldsen ◽

Annette D. Fialla

Keyword(s):

Liver Transplantation ◽

Orthotopic Liver Transplantation ◽

Cardiac Failure ◽

Hereditary Hemorrhagic Telangiectasia ◽

Arteriovenous Malformations ◽

Vascular Malformations ◽

Symptom Relief ◽

Case Series ◽

Hospital Treatment ◽

High Output

Abstract Background This report addresses how patients with hereditary hemorrhagic telangiectasia (HHT) and high output cardiac failure (HOCF) due to hepatic vascular malformations, should be evaluated and could be treated. HHT is a genetic disorder, leading to vascular abnormalities with potentially serious clinical implications. In the liver, arteriovenous malformations occur in more than 70% of patients, but only about 8% present clinical symptoms such as HOCF with pulmonary hypertension and less commonly portal hypertension, biliary ischemia and hepatic encephalopathy. Results Three female patients with HHT type 2 and HOCF caused by severe arteriovenous malformations in the liver are presented in this case series. The patients were seen at the HHT-Centre at Odense University Hospital. Treatment with either orthotopic liver transplantation (one patient) or bevacizumab (two patients) was initiated. All patients experienced marked symptom relief and objective improvement. New York Heart Association—class were improved, ascites, peripheral edema and hence diuretic treatment was markedly reduced or discontinued in all three patients. Bevacizumab also resulted in notable effects on epistaxis and anemia. Conclusion Our findings substantiate the importance of identification of symptomatic arteriovenous malformations in the liver in patients with HHT. Bevacizumab may possibly, as suggested in this case series and supported by previous case studies, postpone the time to orthotopic liver transplantation or even make it unnecessary. Bevacizumab represents a promising new treatment option, which should be investigated further in clinical trials.

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