Point of Care Ultrasonography Assessment During Pulmonary Hypertension Clinic
POCUS Assessment During Ambulatory PAH ClinicBackground: Changes occurring in the pulmonary vascularity imparts changes to the right heart in patients with pulmonary arterial hypertension (PAH). Importantly changes in RV function correlates with severity of disease and patient outcome. This affords and opportunity to assess patients longitudinally through point of care ultrasonography. Investigators objective was to measure the percentage and the rate of patients management change, represented by change in treatment or assessment of patients in the PAH clinic, and to compared management change rate between patients with PAH who underwent point of care ultrasonography (POCUS) assessment in addition to the routine evaluation, and a control group to which the investigators did not add a test with POCUS in addition to the routine evaluation in the clinic. Methods: Consecutive patients from the PAH clinic in two medical centers were randomized to the POCUS assessment group and the non-POCUS group. The POCUS group received lung, heart, and vascular ultrasound assessments. All other assessments were the same between both groups. Management changes were documented during each of the patients' visits over a three-month interval.
Non-invasive Diagnostics of Pulmonary Hypertension With Dual Energy Computed Tomography
Pulmonary Arterial HypertensionThe aim of the study is to determine relevant hemodynamic parameters for the diagnostics of pulmonary arterial hypertension (PAH) by dynamic contrast enhanced dual-energy CT (DE-CT). In this prospective study the investigators validate DE-CT results of patients against hemodynamic parameters from right heart catheterisation and control the results by other clinical investigations. The investigators expect that using this non-invasive method, parameters relevant for the diagnosis of the patients with PAH, like pulmonary blood volume, blood flow and perfusion heterogeneity, can be determined.
Quantifying the Presence of Lung Disease and Pulmonary Hypertension in Children With Sickle Cell...
Sickle Cell DiseaseThe proposed research study is a cross-sectional study enrolling young children with sickle cell disease between 5 and 12 years of age. They will be screened as outpatients for consent to perform pulmonary function testing (PFT) and echocardiography. In addition, the degree of bronchodilator response will be assessed at each session. To estimate presence of pulmonary hypertension, echocardiography will be performed at the time of PFT measures. Study Design: Enroll children aged 5 to 12 years of age with sickle cell disease (HbSS, HbSC, HbS beta plus thalassemia, HbS beta zero thalassemia, and HbS OArab) who are established patients within the Duke Pediatric Sickle Cell Clinic. Perform a chart review of all enrolled subjects to obtain specific details regarding birth history, nutritional status (weight, height), family history, sickle cell genotype, parental smoking history, recent laboratory parameters, parental smoking history, any concurrent conditions (atopy, asthma, airway anomaly), history of sickle cell complications and prescribed medications. Perform spirometry and plethysmography with the administration of albuterol. Before or after completion the PFT session, the patient will have echocardiography in the PFT lab area Using medical record information, determine number of hospitalizations for any pulmonary symptoms indicative of acute chest syndrome (ACS) (dyspnea, fever, wheezing, hypoxia, cough, chest pain). In addition, we will track any respiratory or cardiac symptoms or therapies for each subject 6 years after enrollment up to age 18 years using the registry. As standard of care, refer any child identified as having lung disease or pulmonary hypertension to a pediatric pulmonologist and/or cardiologist for monitoring, treatment and ongoing care.
Novel MRI Techniques in the Evaluation of Pulmonary Vascular Disease
Pulmonary HypertensionThe diagnosis of a patient with pulmonary hypertension (PH) requires many investigations. At present cardiac catheterisation is the cornerstone investigation in these patients where it is used to establish disease severity and estimate prognosis. It is an invasive procedure which is expensive and not without risk to the patient. Despite the multitude of tests performed, identifying those patients with PH who have a poor diagnosis can be difficult. The aim of this study is to improve the assessment of patients with PH using novel magnetic resonance techniques.
Non-invasive Diagnostics of Pulmonary Hypertension With Dual Energy Computed Tomography
Pulmonary Arterial HypertensionThe aim of the study is to determine relevant hemodynamic parameters for the diagnostics of pulmonary arterial hypertension (PAH) by dynamic contrast enhanced dual-energy CT (DE-CT). In this pilot study the investigators investigate patients who underwent right heart catheterisation with DE-CT to determine hemodynamic parameters and control the results by other clinical investigations. The investigators expect that using this non-invasive method, parameters relevant for the diagnosis of the patients with PAH, like pulmonary blood volume, blood flow and perfusion heterogeneity, can be determined.
Sleep-Disordered Breathing in Chronic Thromboembolic Pulmonary Hypertension
Chronic Thromboembolic Pulmonary HypertensionSleep Disordered BreathingThere is evidence for a high prevalence of Sleep Disorder Breathing (SDB) in patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH). Central Sleep Apnea, Cheyne-Stokes Breathing and Obstructive Sleep Apnea appear to occur in CTEPH. However, there is no information on the impact of CTEPH treatment modalities on concomitant SDB. Furthermore, the use of PAP therapy in CTEPH has not yet been investigated. CTEPH is a rare and serious disease and there may be a bidirectional association of SDB and CTEPH. This study plans to investigate the prevalence of SDB in CTEPH and compare it to datasets of large epidemiological studies on SDB. Furthermore, the impact of CTEPH treatment on SDB will be analyzed and CTEPH patients for possible PAP treatment will be defined.
Noninvasive Cardiac Output Monitoring in Pulmonary Hypertension
Pulmonary HypertensionComparison of Ultrasonic Cardiac Output Monitor (USCOM) system with Fick and thermodilution methods during right heart catheterization in patients with pulmonary hypertension.
Acute Exposure to Simulated Hypoxia on Exercise Capacity
Pulmonary HypertensionRandomized crossover trial in patients with Pulmonary Hypertension (PAH, CTEPH) to assess the acute response to simulated altitude (FiO2:15.1, equivalent to 2500m above sea level) in constant loaded exercise capacity.
Acute Excposure of High Altitude on ECG and Non-invasive Blood Pressure.
Pulmonary HypertensionRandomized crossover trial in patients with Pulmonary Hypertension (PAH, CTEPH) to assess the acute response of High Altitude (Säntis, 2500m above sea level) on ECG repolarizations and non-invasive blood pressure measurements by Finapres® NOVA Technology.
Acute Exposure of Simulated Hypoxia on Blood and Tissue Oxygenation (aBGA, NIRS)
Pulmonary HypertensionRandomized crossover trial in patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary Hypertension (CTEPH) to assess the acute response to simulated altitude (FiO2:15.1, equivalent to 2500m above sea level) on blood and tissue oxygenation (ABGA, near infrared spectroscopy)