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

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.

High-output heart failure associated with primary plasma cell leukaemia due to arteriovenous shunting: a case report

Background Primary plasma cell leukaemia is rarely associated with high-output heart failure, and the underlying mechanism is not well understood. We encountered a rare case of high-output heart failure caused by primary plasma cell leukaemia. Its underlying mechanism was clarified through imaging studies. Case Summary A 49-year-old man with no specific medical history was admitted to our hospital because of heart failure that did not improve with diuretic therapy. His condition was diagnosed as high-output heart failure and primary plasma cell leukaemia after admission. Extensive bone involvement in primary plasma cell leukaemia and arteriovenous shunts in the same lesion were suspected after various imaging studies. The first cycle of chemotherapy with bortezomib, adriamycin, and dexamethasone led to remission of primary plasma cell leukaemia and improved heart failure symptoms. The patient received further chemotherapy in addition to autologous peripheral blood stem cell transplantation and maintenance therapy and had no recurrence of pPCL or heart failure for 1 year to date. Discussion : Primary plasma cell leukaemia can be associated with high-output heart failure, which is caused by arteriovenous shunting at the lesion site with diffuse bone involvement. Imaging studies may lead to the early diagnosis of aetiology and treatment of patients with high-output heart failure associated with primary plasma cell leukaemia.

Abstract P155: Etiology And Complications Associated With High-output Heart Failure; A National Readmission Database Study.

Background: The literature on the etiologies and complications of high-output heart failure (HOHF) is limited. Objective: To study the causes and complications related to HOHF in the United States (US). Methods: Data from the national readmissions database (NRD) sample that constitutes 49.1% of the stratified sample of all hospitals in the United States, representing more than 95% of the national population were analyzed for hospitalizations with primary diagnosis of HOHF for the years 2017-2018. Etiology associated with HOHF were extracted using ICD-10 codes. Results: A total of 2,107 index hospitalizations (mean age 62.2 ± 19.1) with primary diagnosis of HOHF were recorded in the NRD for the years 2017-2018. The most common causes of HOHF include sepsis 204 (9.7%), leukemia 53 (2.5%), arteriovenous fistula 13 (0.6%), liver cirrhosis 155 (7.4%), Hyperthyroidism 133 (6.3%), thalassemia 23 (1.14%), sickle cell disease 71 (3.35%), morbid obesity 188 (8.95%), COPD 406 (19.3%), myeloproliferative disorders 166 (7.87%). Among the HOHF group, major complications include acute ischemic stroke (42 or 2%), acute kidney injury (593 or 28.1%), hypertensive emergency (74 or 3.5%), atrial fibrillation (409 or 19.4%), ventricular tachycardia/fibrillation (77 or 3.7%), and conduction block (81 or 3.8%) and ST-Elevation myocardial infarction (11 or 0.5%). A total of 83 (3.9%) patients had died during the inpatient hospitalization. Out of the remaining 2,024 patients, a significant portion (62 or 3.1%) required readmission within 30 days. Conclusion: HOHF is an under-reported cardiovascular complication associated with non-cardiovascular disorders. HOHF is associated with significant 30-day readmissions and mortality rates. Proper management of the underlying etiology can prevent the development of HOHF and associated complications. Keywords: cirrhosis; hemodynamics; obesity, leukemia, myeloproliferative disorders, ST-Elevation myocardial infarction (STEMI).

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

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.

Hepatic hemangioma: A case of spontaneous involution

Hepatic hemangiomas are benign, well-circumscribed tumors often found incidentally on imaging. They are estimated to be prevalent in 0.4-20% of the population. While usually solitary lesions, these tumors tend to not cause any issues for patients. However, when they grow to be larger than 5 cm, there can be associated symptoms including pain, nausea, or early satiety from compression on adjacent structures. There can be more rare, serious complications including hemobilia, rupture, coagulopathy, or high output heart failure seen in tumors that are typically larger than 10 cm. While small hepatic hemangiomas (<5 cm) that do not cause symptoms do not need to be followed, ones that are greater than 5 cm should be followed at least once to ensure that they are stable in size, or more regularly if they are growing. Symptomatic large hepatic hemangiomas can be intervened on with a number of procedures if needed. These include hepatectomy, enucleation, transcatheter arterial embolization, radiofrequency ablation, and in rare cases, liver transplant.