Background & AimsHereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder frequently associated with hepatic vascular malformations (HVMs).Up to 78% of patients develop HVMs, which may lead to haemodynamic alterations, increased cardiac preload, and J o u r n a l P r e -p r o o f progression to high-output heart failure (HOHF).This study aimed to determine the prevalence of HOHF and assess the relationship between HVM severity, stroke volume, and cardiac index in patients with HHT.A secondary aim was to explore the haemodynamic mechanisms underlying HOHF and evaluate the usefulness of transthoracic echocardiography (TTE). MethodsIn this cohort study, all adult patients with HHT registered in the national Danish HHT database and residing in the regions of Zealand and Southern Denmark were invited to participate.Participants underwent clinical evaluation, hepatic Doppler ultrasound, and TTE.HVM severity was graded according to the Buscarini classification from 0 to 4. ResultsOf 203 eligible patients, 152 (75%) participated.The mean age was 51 years (range 18-84), and 57.9% were female.HVMs were identified in 103 patients (67.8%), of whom 24 (15.8%) had grade 4 HVMs.HOHF was present in 10 patients (6.6%), nine of whom (90%) had grade 4 HVMs.Cardiac index measured by TTE correlated positively with HVM severity ( = 0.36, p < 0.001), with a mean increase of 0.81 L/min/m between HVM grades 0-1 and grade 4 (95% CI 0.48-1.13). ConclusionsHigh output heart failure was present in 6.6% of patients and occurred predominantly in those with severe hepatic vascular malformations.Stroke volume and cardiac index increased in parallel with hepatic vascular malformation severity.Transthoracic echocardiography appears to be a useful non-invasive tool for identifying patients at risk of haemodynamic complications. Impact and implicationsIncreasing hepatic vascular malformation severity in hereditary haemorrhagic telangiectasia was associated with a higher cardiac index and clarified the haemodynamic basis of clinical high-output states.These findings may help clinicians identify patients at the greatest risk of this complication, even before symptom manifestation, supporting the use of transthoracic echocardiography alongside hepatic Doppler ultrasound for monitoring and early J o u r n a l P r e -p r o o f intervention. Highlights Hepatic vascular malformations were found in 67.8% of HHT patients. Cardiac index increased progressively with hepatic vascular malformation severity. High-output heart failure occurred primarily in patients with severe vascular malformations. Transthoracic echocardiography may aid early detection of haemodynamic complications. High-output heart failure was identified in 6.6 % of patients.
HAAHR et al. (Wed,) studied this question.