Coronary Angiography and Interventions in Patients With Hereditary Hemorrhagic Telangiectasia

Publication date: Available online 11 November 2017 Source:Revista Española de Cardiología (English Edition) Author(s): Andreas S. Triantafyllis, Andreas S. Kalogeropoulos, Keir McCutcheon, Walter Desmet, Johan Bennett
Source: Revista Espanola de Cardiologia - Category: Cardiology Source Type: research

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We present a 10 year old boy with syncope who was found to have long-QT syndrome and severe pulmonary hypertension (PH) both in the absence of a secondary cause, to our knowledge this is the first report with this unusual coexistence. His genetic tests were positive for hereditary hemorrhagic telangiectasia and long QT syndrome (LQTS) without any family history of PH or LQTS. We demonstrated that digital subtraction pulmonary angiography was more useful compared to CT angiogram to demonstrate pulmonary vascular changes which correlated with a no-response to acute vasoreactivity testing during right heart catheterization. H...
Source: Cardiovascular and Hematological Agents in Medicinal Chemistry - Category: Cardiology Tags: Cardiovasc Hematol Agents Med Chem Source Type: research
Publication date: December 2018Source: Revista Española de Cardiología (English Edition), Volume 71, Issue 12Author(s): Andreas S. Triantafyllis, Andreas S. Kalogeropoulos, Keir McCutcheon, Walter Desmet, Johan Bennett
Source: Revista Espanola de Cardiologia - Category: Cardiology Source Type: research
This article describes three hereditary conditions known to be associated with arteriovenous malformation (AVM), along with their clinical and imaging features and angiographic angioarchitecture. Hereditary hemorrhagic telangiectasia, capillary malformation–AVM (CM–AVM), and PTEN tumor hamartoma syndrome are conditions with autosomal dominant inheritance, caused by mutations in different molecular pathways, which frequently present with symptomatic AVMs. Imaging biomarkers, including sites of predilection, angioarchitecture, and tissue overgrowth patterns, are helpful in identifying these patients and selecting...
Source: Seminars in Interventional Radiology - Category: Radiology Authors: Tags: Review Article Source Type: research
We report a case of a 62-year-old woman who received a liver transplant 19 years previously for end-stage liver disease due to hereditary hemorrhagic telangiectasia and fibropolycystic liver disease. During long-term follow-up 8 years after the liver transplant, de novo vascular lesions were detected with magnetic resonance imaging and magnetic resonance angiography. Hepatic vascular lesions had slowly progressed, despite no symptoms. To our knowledge, there are few reports in the English literature of de novo vascular lesions after liver transplant in patients with hepatic telangiectasias. PMID: 28004998 [PubMed - as...
Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation - Category: Transplant Surgery Authors: Tags: Exp Clin Transplant Source Type: research
Abstract: A 5-year-old boy was admitted due to shortness of breath. Blood gas analysis showed hypoxemia. However, thoracic and abdominal CT, brain MRI, and MR angiography were all normal. A 99mTc-MAA pulmonary scintigraphy revealed right-to-left shunting of the blood. Further genetic analysis showed the mutations in the activin receptor-like kinase 1 gene, and a diagnosis of hereditary hemorrhagic telangiectasia was made.
Source: Clinical Nuclear Medicine - Category: Nuclear Medicine Tags: Interesting Images Source Type: research
This study reports the experience of a referral medical center with the use of coils and Amplatzer plugs for treating PAVMs in patients with hereditary hemorrhagic telangiectasia. Methods The study was approved by the Institutional Review Board with waiver of informed consent. The cohort included all patients who underwent PAVM embolization in 2004–2014 for whom follow-up imaging scans were available. The medical files were retrospectively reviewed for background data, embolization method (coils, Amplatzer plugs, both), and complications. Re-canalization of treated PAVMs wa...
Source: CardioVascular and Interventional Radiology - Category: Radiology Source Type: research
Publication date: Available online 19 May 2016 Source:Journal of the American College of Radiology Author(s): Michael Hanley, Osmanuddin Ahmed, Ankur Chandra, Kenneth L. Gage, Marie D. Gerhard-Herman, Michael Ginsburg, Heather L. Gornik, Pamela T. Johnson, Isabel B. Oliva, Thomas Ptak, Michael L. Steigner, Richard Strax, Frank J. Rybicki, Karin E. Dill Pulmonary arteriovenous malformations are often included in the differential diagnosis of common clinical presentations, including hypoxemia, hemoptysis, brain abscesses, and paradoxical stroke, as well as affecting 30% to 50% of patients with hereditary hemo...
Source: Journal of the American College of Radiology - Category: Radiology Source Type: research
We present a woman who presented with neurological symptoms due to a cerebral abscess. On further evaluation, bilateral pulmonary AVMs were identified. The patient was diagnosed with HHT, based on positive family history and multiple cerebral AVMs recognized on subsequent catheter angiogram, in addition to the presence of bilateral pulmonary AVMs. Craniotomy with drainage of the brain abscess and endovascular embolization of the pulmonary AVMs was offered to the patient. As a preembolization work-up, the patient underwent nuclear lung perfusion scan with technetium-99m macroaggregated albumin (Tc-99m MAA) to assess the rig...
Source: Annals of Thoracic Medicine - Category: Respiratory Medicine Authors: Source Type: research
Conclusions For asymptomatic AVMs discovered incidentally without angiographic bleeding risk, we propose a therapeutic abstention. In case of AVM rupture and bleeding, the other “associated” AVMs (discovered through a complete angiographic assessment) should also be treated if they are not located in an eloquent area and if the treatment does not present technical difficulties. AVMs with a history of bleeding or associated to angiographic risks have to be treated more aggressively.
Source: Acta Neurochirurgica - Category: Neurosurgery Source Type: research
CONCLUSIONS: Spinal fistulae in HHT patients are usually type IV perimedullary fistulae. Both endovascular and surgical treatments appeared to be effective in treating these lesions. However, it is clear that endovascular therapy has become the preferred treatment modality. PMID: 26823330 [PubMed - as supplied by publisher]
Source: Interventional Neuroradiology - Category: Radiology Tags: Interv Neuroradiol Source Type: research
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