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Future Directions for Guidelines

Future Priorities for Guidelines

Adult BAVM screening (how, when)
Pulmonary Hypertension and HOCF
Antiangiogenic dosing, maintenance strategy
Antiangiogenics propranolol, lenalidomide and pomalidomide, somatostatine analogues...
Re-screening and when to stop screening due to age
Multidisciplinary team care (care models and utilization; care outside of center versus in a center and impact on outcomes) - no care, non-disease specific care, disease specific care in nonspecialized centers by nonspecialized providers, care in nonspecialized centers by specialized providers, integrated care model - Should integrated care or nonintegrated care be used.
Long term outcomes
Manage anemia, GI and epistaxis to better define grading or severity
Cerebral AVM’s in adults
Updating current recommendations where new research that has dramatically improved treatment

Future Priorities for Research

The expert panel identified many areas for further research in HHT, which are outlined below. Thematically, these areas fall into several categories: improved analysis of existing retrospective data and collection of harmonized prospective data through multicenter collaboration, standardization of diagnostic and treatment algorithms, technology development, technique refinement, and an increased focus on prenatal and pediatric disease detection and treatment.


  1. Sensitivity and specificity of Curacao criteria; incorporate genetic testing into criteria
  2. App based / phone-based diagnosis of skin lesions

Screening Methods

  1. Full body imaging exam for HHT
  2. Optimal MRI imaging protocols for HHT-Optimized full-body imaging for HHT diagnosis and surveillance (brain, lungs, liver, etc).
  3. Value of screening programs


  1. Review of Curacao Criteria-diagnosis in children
  2. Pediatric rescreening strategies
  3. Brain AVMs in children with HHT e.g. prevalence of hemorrhage, success of various treatment modalities; This could include collecting MRI data on children and combining case series data from multiple centers
  4. Natural history of PAVM in children
  5. Outcomes for PAVM treatment in children
  6. Clarifying screening yield and disease course in pediatric patients though multi-center collaborations


  1. Prenatal imaging: Fetal MRI and ultrasound to detect pulmonary AVM, brain VM, Liver VM, SMAD4 features
  2. Development of recommendations for Prenatal screening for PAVM and BAVM with advanced prenatal ultrasound and fetal MRI in pregnant patients
  3. Does prenatal screening for pulmonary AVM and brain VM with advanced prenatal ultrasound and fetal MRI in pregnant patients, increase the detection rate of pulmonary AVM/brain VM during the antenatal period through the first year of life?

Anemia and Blood Loss

  1. Algorithmic approach to anemia: Quantification of total bleeding (GI, epistaxis), scoring system-Development of a hematologic support score (HSS) that is a precise measurement of total burden of chronic HHT-associated bleeding; HHT related bleeding scoring system specific to anemia
  2. Impact of chronic anemia in HHT patients beyond the hematologic effects (neurocognitive, musculoskeletal function, heart failure)
  3. New endoscopic techniques of hemostasis

Quality of Life

  1. Exercise and improvement in HHT symptoms
  2.  Can we improve morbidity/mortality by targeting specific things: hemoglobin level, ESS, ferritin level


  1. Costs of epistaxis treatment
  2. Evaluate the effectiveness of embolization of the nasal arteries for patients in  life threatening situations or for those who have failed all surgical modalities
  3. Develop a validated tool to record bleeding frequency, severity and quality of life in patients with HHT.
  4. Long-term outcomes of laser and sclerotherapy
  5. Validate patient-based outcome measures for epistaxis
  6. Validate clinimetric system for grading nasal telangiectasias
  7. Evaluate effectiveness of nasal packing

Brain AVM

  1. Develop standardized brain imaging protocols
  2. When to repeat brain screening
  3. Pooled data on: de novo and enlarging brain VMs in patients of all ages
  4. Pooled data on manifestations of HHT (e.g., children with brain VMs or central nervous system hemorrhages)
  5. Risk of VM in pregnancy

Spinal AVM

  1. Pooled data on spinal AVM


  1. Genotype phenotype correlation in HHT
  2. Unknown genes that yield HHT phenotype


  1. Sac embolization/venous embolization in PAVM
  2. Management of systemic artery reperfusion
  3. Growth rates of pulmonary AVM by noninvasive imaging
  4. Outcomes for pulmonary AVM treatment in children
  5. Follow up of pulmonary AVM based on TTCE, how often and when to stop?
  6. IV filter recommendations
  7. Should treatment be repeated multiple times, or does this cause more AVMs to form?
  8. Long-term effects of pulmonary AVM treatment (incidence of arterial reperfusion and how to treat it

Gastrointestinal Bleeding

  1. Systemic treatments for GI bleeding .

Liver VMs

  1. Natural history of liver VM in adults
  2. Ultrasound versus MRI in liver VM flow and portal vein shunting
  3. Treatment of asymptomatic liver VM with high cardiac output to prevent progression to HOCF
  4. Targeted embolization/interventional treatment of liver VM
  5. Determinants of hepatic phenotype
  6. Outcomes in screened versus non-screened patients (liver VM)
  7. Overlap between Liver AVM and pulmonary hypertension
  8. Mechanisms of non-response to antiangiogenics in liver VMs (vessel caliber, other)
  9. Study prevalence of symptomatic liver VM in persons with an Endoglin mutation
  10. Does screening for Liver AVMs change outcomes?

Women and HHT

  1. Heavy Menstrual periods and incidence of uterine AVM’s/ telangiectasia's/AVM
  2. Incidence of miscarriages in women with HHT
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