PortoSinusoidal Vascular Diseases and Systemic Sclerosis
PSVD With or Without SScPorto-Sinusoidal Vascular Disorders (PSVD) is a heterogeneous clinico-pathological entity characterized by alterations in small liver vessels. "Nodular regenerative hyperplasia (NRH)" is the most common PSVD. The most frequent liver disease associated with Systemic sclerosis (SSc) is primary biliary cholangitis reported in 2 to 22% of cases. NRH prevalence estimated to 1.4%, may be underestimated as NRH diagnosis is histologic and usually suspected in case of complications due to portal hypertension. Few data are available about NRH associated with SSc resulting in a lack of knowledge of the characteristics and outcome of these patients.
Cardiovascular Longitudinal ALSPAC Research Investigations Following Hypertensive Pregnancy in Young...
Young AdultHypertension in Pregnancy4 moreThe purpose of this study is to understand more about why young people who were born to a hypertensive pregnancy may have increased risk of high blood pressure and are often at increased risk of heart and blood vessel disease later in life.
Pediatric Pulmonary Hypertension Network (PPHNet) Informatics Registry
Pulmonary Vascular DiseasePulmonary Arterial HypertensionPatients are being asked to be in this research study because medical researchers hope that by gathering information about a large number of children with pulmonary hypertension over time, their understanding of the disease process will increase and lead to better treatment. Investigators believe that pulmonary hypertension in children is different than pulmonary hypertension in adults and this study will help us understand those differences.
The "Virtual" Multicenter Spontaneous Coronary Artery Dissection (SCAD) Registry
Spontaneous Coronary Artery DissectionSCADThe primary goal of this project is to describe the clinical and physiologic characteristics of Spontaneous Coronary Artery Dissections (SCAD) in order to increase awareness, understanding, treatment and prevention of a potentially fatal cardiovascular event. This study will be a retrospective and prospective review of medical course and current health of men and women with SCAD.
Early Detection of Vaping-related Vascular Diseases
VapingBlood Vessel InjuryThe purpose of this study is to determine how vaping affects blood vessels, in particular if early damage occurs in the lung vessels.
Perioperative Three-dimensional Echocardiography in Cardioanesthesia.
Cardiac DiseaseVascular DiseasesThe aim of this study is to investigate the significance of intraoperative echocardiography on the surgical course and association with patient outcome. Of particular interest is the three-dimensional assessment of cardiac and valvular function. The patient course from surgery to the time of hospital discharge will be followed up.
Long-term Outcomes, Recurrence Rates, and Treatment Needs in Polypoidal Choroidal Vasculopathy (PCV)...
Polypoidal Choroidal VasculopathyTo determine long-term outcomes, recurrence rates, and treatment needs in Polypoidal Choroidal Vasculopathy (PCV). Specifically, to describe the long-term outcomes, recurrence rates and treatment patterns PCV patients 5 years after first presentation. This study will also evaluate whether polyp closure within the first 12 months of therapy is associated with better long-term visual outcome and will also determine the risk of disease development in the fellow eye
Samples From Human Subjects to Facilitate Basic, Translational and Clinical Research
Endothelial DysfunctionInflammation in Cardiopulmonary and Vascular Disease States1 moreBackground: This study is designed to provide samples to help us study the genes your blood cells are making as well as the proteins, sugars, fats, vitamins and other metabolites found in your blood or urine. Blood samples may also be collected to make special cells. These are called induced pluripotent stem cells or iPSCs. Pluripotent stem cells are cells that can be converted into any type of cell. Researchers want to study in the lab iPSCs that are derived from blood samples. Objective: To collect samples to help study genes, proteins, sugars, fats, vitamins, and other metabolites found in blood or urine. Eligibility: Healthy volunteers and patients ages 18 and older Design: First-time research study participants at NIH will have an initial visit for this study that should last no more than 1 hour. All other visits should last 20 30 minutes. Participants will undergo a limited history and physical exam. Participants may have routine blood and urine tests. If participants are giving a blood sample, they must have a hemoglobin level checked in the past 12 months to make sure it is safe for them to give a blood sample for research. Participants may have a venous blood collection. They may do this at several visits. They will lie on a recliner or couch or sit in a chair. A needle will be placed into a vein in the hand or arm, using sterile techniques. Blood will be withdrawn into multiple syringes or tubes. Participants may be asked to provide urine in an appropriate container...
Influence of Age, Weight and Ethnic Background on Blood Pressure
Cardiovascular DiseasesVascular Diseases1 moreHypertension, also known as high blood pressure, is a chronic medical condition, in which the blood pressure is elevated. This is a common condition, which can lead to severe complications such as cardiovascular disease, heart attack, stroke and kidney disease, if not detected and treated early. Accumulating evidence suggests that the incidence of hypertension varies according to age, ethnicity and obesity. In order to obtain an in-depth knowledge of the pathophysiological mechanisms of hypertension, we aim to investigate the haemodynamic and biochemical correlates of elevated blood pressure across the adult age-span, and determine the extent to which body size and ethnicity impact on these associations. We also wish to investigate the impact of hypertension on key target organs (end-organ damage). The primary objective of this study is to compare the mechanisms regulating blood pressure in hypertensive and non-hypertensive participants across the adult age span, and to assess the influence of body mass index and ethnic background on these mechanisms. Secondary objectives are to investigate the association between blood pressure and cardiovascular physiology across the adult age span at rest and during sub-maximal exercise and to investigate the impact of blood pressure haemodynamics on key organs including the arteries and heart by assessing end-organ damage such as endothelial function, arterial structure and left ventricular mass/function. This study will be a combined case-control and cross-sectional study describing the procedures and time commitment required to investigate our scientific aims. This is a single centre study that will be conducted in a secondary care environment. Both male and females aged 18 and over and that are able provide informed consent will be considered for this study. People who are pregnant, currently receiving dialysis, illness with a life expectancy <1 year, current active malignancy and cannot provide informed consent are ineligible for this study. The study will be open for five years and each patient will complete a maximum of four visits in a 12 month period. Participants will complete a variety of non-invasive physiological assessments of their cardiovascular system and lung function. There will be some minimally invasive procedures completed, including a blood test and assessment of small artery endothelial function which involves insertion of a small needle under local anesthetic.
Complement Prospective Evaluation of Thrombotic Microangiopathy on Endothelium
Thrombotic MicroangiopathiesHemolytic Uremic Syndrome2 moreThrombotic microangiopathy (TMA) is a severe and life-threatening condition, often affecting the kidneys and brain. It can occur on the background of various clinical conditions. Dysregulation of the alternative pathway of complement may be the etiological factor and this type of TMA is classified, according to the current nomenclature, as primary atypical hemolytic uremic syndrome (HUS). Half the patients with primary atypical HUS present with rare variants in complement genes, although coexisting conditions are often needed for the TMA to become manifest. In patients with secondary atypical HUS, certain coexisting conditions appear to drive the disease and treatment should target the underlying condition to remit the TMA. Recently, the investigators demonstrated, by using a novel in-house developed functional endothelial cell-based test, that complement dysregulation and overactivation is the dominant cause of disease and its sequelae in a subset of patients with secondary atypical HUS, having impact on treatment and prognosis. The investigators did first prove this concept in patients presenting with TMA and hypertensive emergency. A prospective study is needed to further corroborate these findings along the spectrum of TMA. The investigators hypothesize that their functional endothelial cell-based test, the so-called "HMEC" test, can better categorizes the TMA into different groups with potential therapeutic and prognostic implications. Thus, paving the road to the ultimate goal of precision medicine.