Usefulness of Transcranial Doppler for Detecting Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia

Orthotopic Liver Transplantation for Hereditary Hemorrhagic Telangiectasia and MEN Type I Syndrome - Case Report and Review of Literature

“…PAVMs expose HHT patients to a wide range of potential complications caused by the rightto-left shunt: transient ischemic attack (10%), stroke (10-19%), hypoxemia, air embolism and secondary polycythaemia and brain abscess (5-19%) (44). This is caused by septic emboli in the deoxygenated blood entering the brain via paradoxical embolism (45). It is noteworthy that neurological manifestations are more common in those with pulmonary arteriovenous fistula (AVF) than in those with cerebrovascular malformations.…”

Some Practical Points About Patent Foramen Ovale Conditions that May Not Be Covered in the Rest of the Book

“…If a patient has hereditary hemorrhagic telangiectasia, then the incidence of pulmonary AVM is 30%, but these cases are infrequent. 10 I would caution physicians who are interested in this field to be skeptical of the echocardiographic data that does not compare the frequency of finding a PFO with evidence based on a right heart catheterization (ie, ability to pass a guidewire across the atrial septum), which should be the correct in vivo gold standard for PFO diagnosis. I hope that the echocardiographic societies will reassess their guideline statements, as TEE (the current accepted gold standard by echocardiographic societies) can miss or misdiagnose a PFO in 10% of cases.…”

Multimodal monitoring: practical recommendations (dos and don'ts) in challenging situations and uncertainty

“…A similar phenomenon allows the detection of paradoxical emboli when evaluating for the presence of a patent foramen ovale (PFO) in patients presenting with a cryptogenic stroke ( 44 ). Hereditary hemorrhagic telangiectasia (HHT) has been commonly associated with PAVF, so TCD may also be a useful tool for screening this patient population ( 45 ). As intracranial vessels are being insonated, patients are injected with agitated saline to identify microemboli presenting as HITS.…”