Heart and Blood Diseases

Diseases and Abnormalities at or Before Birth

All Conditions

Nervous System Diseases

Neoplasms

Rare Diseases

Arteriovenous Malformations

Congenital Abnormalities

Intracranial Arteriovenous Malformations

Hemangioma

Vascular Malformations

Cardiovascular Abnormalities

Vascular Diseases

Neoplasms, Vascular Tissue

Neoplasms by Histologic Type

Cerebrovascular Disorders

Brain Diseases

Central Nervous System Diseases

Central Nervous System Vascular Malformations

Nervous System Malformations

Intracranial Arterial Diseases

Intracranial Arteriovenous Malformation

SQUID™

A non-interventional multicentric study for patients with a cerebral AVM for which endovascular treatment is indicated.

This observational study will collect data about the treatment of ruptured, unruptured, untreated or endovascular previously treated AVM's.

Primary endpoint is safety. Safety will be assessed with the number of procedure-related complications occurring during the endovascular treatment until 30 days after the final embolization.

Secondary endpoint is efficacy. Efficacy will be assessed by angiography and clinical outcome assessments (mRS).

For each patient enrolled, data will be collected at inclusion, at each embolization session and at 6 ± 1 months after the end of the endovascular treatment phase.

Treatment of Cerebral Arteriovenous Malformations With SQUID™ Liquid Embolic Agent

The safety is assessed with the number of procedure-related complications occurring during endovascular treatment until 30 days after the final embolization session. Procedure-related complications include complications/adverse events related to the procedure or to the device such as but not limited to hemorrhagic and ischemic complications.

Change of mRS score at 6 months after treatment phase is assessed compared to baseline mRS score.

The degree of the predefined targeted portion of the AVM's occlusion will be assessed angiographically.

Archer Research

Emboflu

Symptoms and General Pathology

Digestive System Diseases

Respiratory Tract (Lung and Bronchial) Diseases

Ear, Nose, and Throat Diseases

Blood and Lymph Conditions

Hemorrhage

Gastrointestinal Hemorrhage

Telangiectasis

Epistaxis

Telangiectasia, Hereditary Hemorrhagic

Gastrointestinal Diseases

Nose Diseases

Respiratory Tract Diseases

Otorhinolaryngologic Diseases

Signs and Symptoms, Respiratory

Hemostatic Disorders

Blood Coagulation Disorders

Hemorrhagic Disorders

Hematologic Diseases

Hereditary Hemorrhagic Telangiectasia

Those diagnosed with HHT based on the Curacao diagnostic criteria or genetic testing

Melissa A Dickey, MSN

Nolie Krock, MSc

Sayeh Jafari

Victoria Rueda

Justin McWilliams, MD

Lucas Cusumano, MD

Lindsay Park, MS

Steven Hetts, MD

Miles Conrad, MD

Johan Allingmon

Peter Hountras, MD

Katharine Henderson

Jeffrey Pollak, MD

Melissa James, RN

James Gossage, MD

Madeleine Macy, BS

Carolena Othman, MS

Josanna Rodriguez-Lopez, MD

Greg Schwichtenberg

Sue Ann Donlinger

Vivek Iyer, MD

Melissa Beasley, AA

Kristine Kempf

Murali Chakinala, MD

Kelsey Weddig, RN, BSN

Charles Murphy, MD

Karen Smith, RN

Kristi Kirkland

Raj Kasthuri, MBBS

Lori Russell, RN

Eleanor Lottsfeldt, BA

Mark Chesnutt, MD

Claire Kaufman, MD

Ala Streater, BS

Isaac Elysee, MS

Theodore Drivas, MD

Mhd Wisam Alnablsi, MD

John Battaile, MD

An Lu, MD

Cassidy Sion, RN, BSN

Kevin Whitehead, MD

The Comprehensive HHT Outcomes Registry of the United States (CHORUS) is an observational registry of patients diagnosed with Hereditary Hemorrhagic Telangiectasia (HHT). The purpose of this study is to better understand HHT, the symptoms and complications it causes, and the impact the disease has on people's lives. The investigators will collect long-term information about the participant, allowing us to understand how the disease changes over time, and what factors can influence those changes. Ultimately, this should help improve treatments for the disease.

Another important goal of the study is to provide a way to contact people to participate in future clinical trials and other research. The registry will be a centralized resource for recruitment for clinical trials. People in the registry will not be obligated to join any of these additional studies, but if interested, can agree to be contacted if they may be eligible for a study.

Participants will:

* Be asked to provide permission to collect information from their medical records, including things like demographic information, diagnosis information, family history, test results, treatment information, symptoms, complications, lifestyle and other relevant medical information.
* Be asked study-related questions by phone or at a clinic visit.
* Be asked study-related questions every year after enrollment for up to 10 years or until the study ends. A member of the study team will communicate with participants by phone or at clinic visits to collect information regarding any changes to their health over the previous year/s including new test results, treatment information, symptoms, and complications from HHT.

The Comprehensive HHT Outcomes Registry of the United States (CHORUS) is a research initiative led by the HHT Foundation International, Inc. ("Cure HHT"). The study focuses on Hereditary Hemorrhagic Telangiectasia (HHT), a rare genetic disorder characterized by the development of abnormal blood vessels in various organs of the body, including the brain, spine, lungs, liver, GI tract, skin, nasal mucosa, and oral cavity. The prevalence of HHT is estimated to be 1 in 5000, affecting children and adults. These abnormal blood vessels can lead to acute and chronic bleeding, stroke, heart failure, and death. Treatments are currently predominately limited to managing complications, while approximately 90% of adults have ongoing symptoms, despite the best surgical and medical therapies. With recent drug developments related to angiogenesis, there is hope for effective novel therapies.

A natural history registry for HHT has significant implications for improving the understanding and management of this rare genetic disorder. The purpose of this study is to better understand HHT, the symptoms and complications it causes ("outcomes"), and to understand how the disease impacts people's lives. The investigators hypothesize that a natural history registry for HHT will improve our understanding of the disease, lead to better management of patients, and ultimately, contribute to developing novel therapies to treat this disease.

The investigators will collaborate with multiple HHT Centers of Excellence across the U.S. to establish a comprehensive registry of HHT patients. The study aims to enroll approximately 10,000 HHT patients over a 10-year period. Longitudinal data will be collected both retrospectively and prospectively, with a focus on increasing the understanding of this rare disease, accelerating the development of new diagnostic and treatment options, and working collaboratively with clinicians who care for individuals with HHT to identify and address gaps in the system of care, especially those from underserved populations.

The study aims to serve as a centralized resource for future clinical trials and research in HHT. Data security and confidentiality are prioritized, and participants have the option to withdraw from the study at any time. The study is funded by the U.S. Department of Health Resources and Service Administration (HRSA) through a grant awarded to Cure HHT.

Comprehensive HHT Outcomes Registry of the United States (CHORUS)

CHORUS: Comprehensive HHT (Hereditary Hemorrhagic Telangiectasia) Outcomes Registry of the United States

Utilizing the "HHT Baseline Assessment Scale," this measure focuses on recruiting a diverse cohort of HHT patients across North America. Comprehensive baseline clinical, demographic, and lifestyle data will be collected and entered into CHORUS. The data, aggregated as mean change from baseline using the scale, will serve as a foundational resource for future clinical translational studies, addressing the urgent need for natural history data in HHT.

Utilizing the "HHT Clinical Outcomes Scale," this measure aims to prospectively and longitudinally assess the clinical outcomes of HHT patients. Determinants such as demographic factors, environmental influences, lifestyle choices, comorbidities, medications, HHT-genotype, and organ vascular malformations (VMs) will be measured. The data, aggregated as mean change from baseline using the scale, will enhance understanding and accelerate the development of new diagnostic and treatment options for HHT.

Description: Utilizing the "HHT Clinical Outcomes Scale," this measure aims to prospectively and longitudinally assess severe complications in HHT. This measure focuses on measuring rates of severe complications in HHT. Determinants of these complications will be characterized, and the data will be aggregated as mean change from baseline using the index, providing valuable insights into the disease progression.

Description: Utilizing the "Epistaxis Severity Scale," this measure aims to characterize epistaxis in adults with HHT, considering aspects such as frequency, duration, intensity, and variability. Outcome determinants related to epistaxis will be elucidated, and the data will be aggregated as mean change from baseline using the scale to inform comprehensive patient care.

Utilizing the "Organ VMs Assessment Tool," this measure involves the prospective measurement of the development and growth of organ vascular malformations (VMs) in HHT patients over time. Outcome determinants related to these VMs will be investigated, and the data will be aggregated as mean change from baseline using the tool, contributing to a better understanding of disease progression.

Utilizing the "HHT Treatment Outcomes Scale," this measure involves the prospective measurement of treatment outcomes for HHT and its clinical manifestations. Treatment outcome determinants will be characterized, and the data will be aggregated as mean change from baseline using the scale to guide effective management strategies for patients with HHT.

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Treatment of Cerebral Arteriovenous Malformations With SQUID™ Liquid Embolic Agent	NCT02602990	Cerebral Arteri...	SQUID™	18 Years -	Emboflu
Comprehensive HHT Outcomes Registry of the United States (CHORUS)	NCT06259292	Hereditary Hemo... Arteriovenous M... Telangiectasia Epistaxis GastroIntestina... Cerebral Arteri... Vascular Malfor...		-	Cure HHT
Treatment of Cerebral Arteriovenous Malformations With SQUID™ Liquid Embolic Agent	NCT02602990	Cerebral Arteri...	SQUID™	18 Years -	Emboflu
Comprehensive HHT Outcomes Registry of the United States (CHORUS)	NCT06259292	Hereditary Hemo... Arteriovenous M... Telangiectasia Epistaxis GastroIntestina... Cerebral Arteri... Vascular Malfor...		-	Cure HHT

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