scholarly journals Hereditary Hemorrhagic Telangiectasia Presenting as High Output Cardiac Failure during Pregnancy

2009 ◽  
Vol 2009 ◽  
pp. 1-3 ◽  
Author(s):  
Tareq Goussous ◽  
Alex Haynes ◽  
Katherine Najarian ◽  
Marcos Daccarett ◽  
Shukri David
Keyword(s):  
Cardiac Failure ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Preterm Labor ◽  
Vascular Malformations ◽  
Rare Complication ◽  
Pregnant Patient ◽  
Non Invasive ◽  
Hepatic Involvement ◽  
History Of ◽  
High Output

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.

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

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.

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

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.

scholarly journals High-output cardiac failure secondary to high-output arteriovenous fistula: investigations, management and definitive treatment

2020 ◽  
Vol 13 (2) ◽  
pp. e233669 ◽  
Author(s):  
James Leon Hartley ◽  
Asheesh Sharma ◽  
Lamis Taha ◽  
Thomas Hestletine
Keyword(s):  
Pulmonary Hypertension ◽  
Arteriovenous Fistula ◽  
Cardiac Failure ◽  
Rare Complication ◽  
Definitive Treatment ◽  
Progressive Dyspnoea ◽  
Dialysis Fistula ◽  
End Stage ◽  
Haemodialysis Treatment ◽  
High Output

A 32 year-old woman was admitted to our institution with progressive dyspnoea. Her medical history was notable for end-stage renal failure secondary to chronic pyelonephritis, and she had undergone a cadaveric renal transplant in 2010. This had been preceded by haemodialysis treatment via a radiocephalic arteriovenous fistula. Her diagnostic evaluation was remarkable for pulmonary hypertension. A subsequent doppler ultrasound of her arteriovenous fistula revealed a blood flow of 3 L/min. This is consistent with a high output fistula. Echocardiography demonstrated an improvement in pulmonary artery pressure with occlusion of the fistula. After multidisciplinary discussion, a decision was made to surgically tie off her fistula. The patient experienced immediate improvement in her shortness of breath along with resolution of pulmonary hypertension on echocardiography. This case highlights the rare complication of high output cardiac failure from a dialysis fistula and its successful surgical management.

Infection in the Pregnant Patient

2016 ◽  
Author(s):  
Vanessa Vasquez
Keyword(s):  
Social History ◽  
Preterm Labor ◽  
Labor History ◽  
Pregnant Patient ◽  
Vaginal Fluid ◽  
Uterine Contractility ◽  
Transmitted Infection ◽  
Sexually Transmitted ◽  
History Of ◽  
High Degree

Infections in pregnancy can result in significant complications for both the mother and fetus and can increase the risk of preterm labor. Fever in a pregnant woman also raises concern for its associated risk of preterm birth due to the release of prostaglandins and cytokines that stimulate uterine contractility. Infection can be passed to the neonate hematogenously or ascend from the genital tract. Treatment during pregnancy creates problems, as many antimicrobials cross the placenta and may have a teratogenic risk. Prophylaxis, vaccination, a high degree of suspicion, and early intervention can help improve morbidity and mortality. The pregnant patient should be asked important questions that include history of uterine tenderness and leakage of vaginal fluid, exposure to or symptoms of sexually transmitted infection, previous preterm labor, history of pregnancy complications, and a thorough social history.

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

2019 ◽  
Vol 14 (1) ◽  
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.

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

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.

scholarly journals Scalp congenital hemangioma with associated high-output cardiac failure in a premature infant: Case report and review of literature

2016 ◽  
Vol 23 (1) ◽  
pp. 102-106 ◽  
Author(s):  
Sumedh S Shah ◽  
Brian M Snelling ◽  
Samir Sur ◽  
Alexandra R Ramnath ◽  
Emmalee S Bandstra ◽  
...  
Keyword(s):  
Premature Infant ◽  
Cardiac Failure ◽  
Neonatal Intensive Care ◽  
Vascular Malformations ◽  
Arterial Embolization ◽  
Venous Drainage ◽  
Occipital Artery ◽  
Sagittal Sinus ◽  
Doppler Ultrasound Imaging ◽  
High Output

Introduction Scalp congenital hemangiomas (CHs) are rare vascular malformations among infants; they can be associated with an array of complications, including cardiac and cosmetic issues. Here, we report the endovascular treatment of a premature infant with a suspected large right parietal scalp hemangioma and associated high-output cardiac failure. Case description A two-day-old female premature infant (29 weeks gestational age; 1330 g birth weight) was referred by the neonatologists to our department for consultation and potential treatment of a large right parietal CH causing abrupt hypotension and high-output cardiac failure. Doppler ultrasound imaging at bedside revealed areas of arterial-venous shunting from the scalp and the presence of a superior sagittal sinus waveform, consistent with intracranial venous drainage. To alleviate cardiac dysfunction secondary to this lesion, trans-arterial embolization via n-butyl cyanoacrylate (nBCA) glue and deployment of detachable coils was performed via umbilical artery to occlude the right superficial temporal and occipital artery branches supplying the CH. Following treatment, the infant continued to require ventilator management, vasopressor support, and correction of coagulopathy, but by post-operative day two, her condition improved remarkably and the mass size began decreasing. The patient was discharged after a relatively uncomplicated subsequent 2½-month course in the neonatal intensive care unit. Conclusion Endovascular therapy proved effective and safe in treating cardiac failure associated with scalp CH, despite potential complications associated with neuro-interventional surgery in premature infants. Appropriate consideration in this patient population should be given to factors including blood loss, contrast use, radiation exposure, operative time, and possible intra-/post-operative complications.

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