Sonja Bartolome | The University of Texas at Austin (original) (raw)

Papers by Sonja Bartolome

Research paper thumbnail of Chronic thromboembolic pulmonary hypertension: an update for 2018

The purpose of this session is to review the history of our understanding of CTEPH and its pathop... more The purpose of this session is to review the history of our understanding of CTEPH and its pathophysiology and natural history. The diagnostic evaluation of a patient with suspected CTEPH will be reviewed as will current concepts in the surgical, interventional and medical treatment of the disease. Educational Objectives: At the end of this lecture, participants will be able to: A. Define CTEPH and describe its natural history. B. Review the diagnostic evaluation of a patient with suspected CTEPH. C. Describe the approach to intervention or medical therapy for CTEPH. CTEPH 2018 # 2 Definition, Pathophysiology and Epidemiology Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a pre-capillary elevation in pulmonary vascular resistance (PVR) resulting from chronic, "scarred-in" thromboembolic material partially occluding the pulmonary arteries. This vascular obstruction, over time, results in failure of the right side of the heart and early mortality. CTEPH was first described in the early 1900s and was further characterized in an autopsy series from the Massachusetts General Hospital in 1931. On these postmortem examinations, it was noted that the affected patients had large pulmonary artery vascular obstruction, relatively normal pulmonary parenchyma distal to the vascular obstruction, and the presence of extensive bronchial collateral blood flow or "compensatory circulation." [1] Still, it would take until the mid-20th century until imaging and cardiac catheterization techniques allowed the recognition of the diagnosis antemortem. The pathobiology of the disease remains poorly understood, but clinical evidence suggests that the process most certainly begins with an acute pulmonary embolus. (Figure 1) Rather than resolving over time, the embolus develops into organized, fibrotic thromboembolic material which adheres to and incorporates with the pulmonary artery wall. (Figure 2) These lesions tend to recanalize and may appear irregularly shaped, or like "bands" or "webs" inside the pulmonary artery. Histologic examination of the chronic thromboembolic material reveals intimal thickening composed of fibroblasts, collagen, lymphocytes and hemosiderin-laden macrophages. [2] Over time, this physical obstruction to the pulmonary vasculature raises the pulmonary vascular resistance and presents an increased workload to the right ventricle, which is geometrically, CTEPH 2018 # 3

Research paper thumbnail of Activated Protein C Attenuates Microvascular Injury During Systemic Hypoxia

Shock, 2007

In response to hypoxia, an inflammatory cascade is initiated and microvascular injury ensues. Spe... more In response to hypoxia, an inflammatory cascade is initiated and microvascular injury ensues. Specifically, within 10 min, leukocyte adherence to the endothelium begins, and leukocyte emigration and vascular leak soon follow. Activated protein C (APC) has been reported to have both anticoagulant and anti-inflammatory properties. Activated protein C is best described in its role as a treatment for sepsis. However, it has been used, with some success, in experimental models of hypoxic injury. We hypothesized that APC would be protective against microvascular injury during systemic hypoxia. Randomized prospective animal study. Adult male Sprague-Dawley rats. To characterize the microvascular response to APC exposure during hypoxia, four rat groups were used: saline control, APC infusion alone (100 mg/kg bolus), hypoxia alone (10% O2), and simultaneous hypoxia + APC infusion. Measurements of leukocyte adherence (no. per 100-microm venule), leukocyte emigration (no. per 4,000 microm(2)), and venular leak by fluorescein isothiacyanate-labeled albumin (Fo/Fi) were performed during intravital microscopy of the intact venular bed. Leukocyte adherence decreased from 14.5 (+/-1.2) cells/100-microm venule in hypoxic rats to 4.4 (+/-1.5) cells/100-microm venule in those treated with both hypoxic gas and APC infusion (P < 0.001). Similarly, leukocyte emigration in hypoxic rats reached 12.3 (+/- 2.2) cells/4,000-microm(2) venule, but was reduced to 3.5 (+/-0.3) cells/4,000-microm(2) venule (P <.001). Venular permeability to protein was also significantly decreased in the APC-treated group from 0.82 (+/-0.14) to 0.25 (+/-0.14) (P < 0.001). The infusion of APC…

Research paper thumbnail of ISHLT consensus statement: Perioperative management of patients with pulmonary hypertension and right heart failure undergoing surgery

The Journal of Heart and Lung Transplantation

Research paper thumbnail of Health disparities and treatment approaches in portopulmonary hypertension and idiopathic pulmonary arterial hypertension: an analysis of the Pulmonary Hypertension Association Registry

Pulmonary Circulation, 2021

Compared to idiopathic pulmonary arterial hypertension (IPAH), patients with portopulmonary hyper... more Compared to idiopathic pulmonary arterial hypertension (IPAH), patients with portopulmonary hypertension (POPH) have worse survival. Health disparities may contribute to these differences but have not been studied. We sought to compare socioeconomic factors in patients with POPH and IPAH and to determine whether socioeconomic status and/or POPH diagnosis were associated with treatment and health-care utilization. We performed a cross-sectional study of adults enrolled in the Pulmonary Hypertension Association Registry. Patients with IPAH (n = 344) and POPH (n = 57) were compared. Compared with IPAH, patients with POPH were less likely to be college graduates (19.6% vs. 34.9%, p = 0.02) and more likely to be unemployed (54.7% vs. 30.5%, p < 0.001) and have an annual household income below poverty level (45.7% vs. 19.0%, p < 0.001). Patients with POPH had similar functional class, quality of life, 6-min walk distance, and mean pulmonary arterial pressure with a higher cardiac index. Compared with IPAH, patients with POPH were less likely to receive combination therapy (46.4% vs. 62.2%, p = 0.03) and endothelin receptor antagonists (28.6% vs. 55.1%, p < 0.001) at enrollment with similar treatment at follow-up. Patients with POPH had more emergency department visits (1.7 ± 2.1 vs. 0.9 ± 1.2, p = 0.009) and hospitalizations in the six months preceding enrollment (1.5 ± 2.1 vs. 0.8 ± 1.1, p = 0.02). Both POPH diagnosis and lower education level were independently associated with a higher number of emergency department visits. Compared to IPAH, patients with POPH have lower socioeconomic status, are less likely to receive initial combination therapy and endothelin receptor antagonists but have similar treatment at follow-up, and have increased health-care utilization.

Research paper thumbnail of Health Disparities and Treatment Patterns in Portopulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension: An Analysis of the Pulmonary Hypertension Association Registry

B27. UP-TO-DATE PAH ASSESSMENT AND MANAGEMENT, 2020

Research paper thumbnail of Variable Creatinine Levels in Critical Care Patients: A Concerning Knowledge Gap

Journal of Clinical Medicine, 2021

An accurate creatinine (Cr) estimate is pivotal for the assessment of renal function. Both patien... more An accurate creatinine (Cr) estimate is pivotal for the assessment of renal function. Both patient- and practice-spawned factors palliate the test accuracy of serum creatinine (sCr) and can erratically represent actual kidney function. This study evaluated the caregivers’ awareness of enzymatic serum creatinine (E-sCr) assay interfering in dopamine/dobutamine (DD)-infused patient samples and the frequency of such interference in a critical care setting. We conducted an sCr awareness survey among UT Southwestern physicians, nurses, and pharmacists. We then performed a cross-sectional E-sCr comparison against the kinetic Jaffe method using the DD-infused patient samples collected from central venous catheters (CVC), peripherally inserted central catheter (PICC) lines, and the peripheral vein (PV). We retrospectively compared the longitudinal E-sCr results of the CVC/PICC draws with the corresponding blood urea nitrogen (BUN) levels. The survey results show a significant lack of awaren...

Research paper thumbnail of in Parenchymal Lung Disease

The prevalence of pulmonary hypertension (PH) in parenchymal lung disease is common. The pathophy... more The prevalence of pulmonary hypertension (PH) in parenchymal lung disease is common. The pathophysiology of pulmonary hypertension (PH) in parenchymal lung diseases is partially, if not mostly, related to hypoxic pulmonary vasoconstriction. The pathophysiology of PH in chronic obstructive pulmonary disease is poorly described, but likely involves an interaction between hypoxic vasoconstriction, vascular inflammation, and a loss of capillaries in the diseased lung. Restrictive lung diseases are characterized by pulmonary parenchymal abnormalities rather than the obstruction of airflow and are often associated with PH through hypoxic pulmonary vasoconstriction and varying degrees of inflammation. Systemic disorders can also affect the pulmonary parenchyma and be associated with PH. PH treatment is controversial for patients with parenchymal lung disease.

Research paper thumbnail of Long Term Survival and Transplant Rates in Pulmonary Arterial Hypertension Patients Treated with Parenteral Prostacyclin Therapy

Chest, 2020

Pulmonary arterial hypertension (PAH), is a fatal disease with high morbidity and mortality. Curr... more Pulmonary arterial hypertension (PAH), is a fatal disease with high morbidity and mortality. Current guidelines recommend parenteral prostacyclin treatment as mainstay of treatment in severely ill patients.However, there is lack of published data on long term outcomes in these patients. Here we describe long-term outcomes in PAH patients on intravenous/ subcutaneous (IV/SC) therapy. METHODS: We performed a retrospective chart review of all patients initiated on parenteral prostacyclin therapy (IV epoprostenol or IV/SC treprostinil) between 2007 and 2016. Patients with portopulmonary hypertension were excluded. Analyses include survival time (censored at transplant), time from diagnosis to IV therapy start, and time from diagnosis to lung transplantation. Statistical analysis was carried out using NCSS software.

Research paper thumbnail of Transitioning Stable Pulmonary Arterial Hypertension Patients from Parenteral Prostacyclin Therapy to Oral Selexipag

B56. PULMONARY HYPERTENSION CLINICAL TRIALS ENDEAVOR SAFETY, COMBINATION WHAT ELSE?, 2020

Research paper thumbnail of Estrogen Metabolism in Portopulmonary Hypertension (POPH): A Case-Control Study

C52. USE YOUR ILLUSION II: CLINICAL RESEARCH IN PAH, 2019

Research paper thumbnail of Quality of Life of Patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Idiopathic Pulmonary Arterial Hypertension (IPAH): The Pulmonary Hypertension Association Registry (PHAR)

C104. LATEST CLINICAL INVESTIGATIONS IN ACUTE AND CHRONIC THROMBOEMBOLISM DISORDERS OF THE PULMONARY CIRCULATION, 2020

Research paper thumbnail of Long-term outcomes in pulmonary arterial hypertension in the first-line epoprostenol or first-line bosentan era

The Journal of Heart and …, 2010

In a single-center, retrospective, longitudinal cohort, data on right heart catheterization, 6-mi... more In a single-center, retrospective, longitudinal cohort, data on right heart catheterization, 6-minute walk distance (6MWD), disease progression and mortality were collected. Outcomes were assessed in first-line bosentan and first-line epoprostenol patients. To reduce selection bias due to ...

Research paper thumbnail of EXPRESS: Health Related Quality of Life and Hospitalizations In Chronic Thromboembolic Pulmonary Hypertension Versus Idiopathic Pulmonary Arterial Hypertension: An Analysis from the Pulmonary Hypertension Association Registry(PHAR)

Pulmonary Circulation

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, morbid, potentially curable subt... more Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, morbid, potentially curable subtype of pulmonary hypertension that negatively impacts health-related quality of life (HRQoL). Little is known about differences in HRQoL and hospitalization between CTEPH patients and idiopathic pulmonary arterial hypertension (IPAH) patients. Using multivariable linear regression and mixed effects models, we examined differences in HRQoL assessed by emPHasis-10 (E10) and SF-12 between CTEPH and IPAH patients in the Pulmonary Hypertension Association Registry, a prospective multicenter cohort of patients newly evaluated at a Pulmonary Hypertension Care Center. Multivariable negative binomial regression models were used to estimate incidence rate ratios (IRR) for hospitalization amongst the two groups. We included 461 IPAH and 169 CTEPH patients. 21% of CTEPH patients underwent pulmonary thrombendarterectomy (PTE) before the end of follow-up. At baseline, patients with CTEPH had significa...

Research paper thumbnail of Parenteral prostanoids for severe Group 3 pulmonary hypertension with right ventricular dysfunction

Journal of Thoracic Disease

Background: Group 3 pulmonary hypertension (PH) is a common complication in patients with lung di... more Background: Group 3 pulmonary hypertension (PH) is a common complication in patients with lung diseases but there are currently no FDA-approved therapies. The data is conflicting, but a few small studies suggest potential benefits in using Group 1 PH therapies in these patients, particularly in severe PH with right ventricular (RV) dysfunction. Methods: A retrospective cohort study of patients with severe Group 3 PH with RV dysfunction who received parenteral prostanoids from 2007-2018 at our institution was undertaken. Severe PH was defined as mean pulmonary arterial pressure (mPAP) ≥35 mmHg or mPAP 25-34 with cardiac index (CI) <2.4 L/min/m 2. Routine prognostic studies including N-terminal prohormone of brain natriuretic peptide (NT-proBNP), 6-minute walk distance (6MWD), WHO Functional Class assessment, oxygen requirement, arterial oxygen saturation, right ventricular systolic pressure (RVSP) and right heart catheterization (RHC) pressures, were obtained before initiation of parenteral therapy and at first clinical follow-up. Results: Nine patients were included. Five were female (55.6%) with a median [interquartile range (IQR)] of 69 [54-71] years. Median CI was 1.8 (1.6-2.4) L/min/m 2 and median pulmonary vascular resistance (PVR) was 14.7 (10.7-17.1) Wood units (WU). We found no statistically significant improvement in NT-proBNP levels, exercise capacity, or functional class. Resting oxygen requirement worsened from 4 to 6 L/min (P=0.04) and exertional oxygen saturation nadir worsened from 90% to 83% (P=0.01) despite the increase in FiO 2 with exertion. Overall results were heterogenous: several patients demonstrated clinical stabilization, with two undergoing lung transplantation and one showing long-term stability with medical therapy. Symptoms remained severe for most: three patients discontinued prostanoid therapy, choosing to pursue hospice care. Conclusions: We found no statistically significant improvement in NT-proBNP levels, exercise capacity, or functional class, while oxygen requirement at rest and oxygen saturation during exertion significantly worsened. Our results suggest that parenteral prostanoids should not generally be considered in the treatment of Group 3 PH patients.

Research paper thumbnail of Estrogen Signaling and Portopulmonary Hypertension: The Pulmonary Vascular Complications of Liver Disease Study (PVCLD2)

Research paper thumbnail of Pulmonary Hypertension and Chronic Obstructive Pulmonary Disease: When Is It Out of Proportion?

Advances in Pulmonary Hypertension

Patients with chronic obstructive pulmonary disease (COPD) often present with mild pulmonary hype... more Patients with chronic obstructive pulmonary disease (COPD) often present with mild pulmonary hypertension (PH). This finding has been attributed to hypoxic pulmonary vasoconstriction. However, a small proportion of COPD patients will present with moderate or severe elevations in their pulmonary artery pressure (PAP), and these patients appear to have worsened symptoms and survival when compared to patients with milder elevations in PAP. The diagnosis of PH in COPD may be difficult, due to inaccuracies in the echocardiographic estimates of PAP in these patients. Additionally, many patients with COPD will also have comorbid conditions such as diastolic heart failure, systolic heart failure, or obstructive sleep apnea, which may cause increased pulmonary pressures through other mechanisms. Clinical trials investigating the effect of PH-specific therapy for patients with PH and COPD have been small, with mixed results. A careful evaluation for other causes of PH and hemodynamic evaluati...

Research paper thumbnail of Anorexigen-Associated Pulmonary Arterial Hypertension and the Serotonin Hypothesis: A Story Worth Telling

Advances in Pulmonary Hypertension

Diet pills such as aminorex, fenfluramine, and dexfenfluramine have been strongly associated with... more Diet pills such as aminorex, fenfluramine, and dexfenfluramine have been strongly associated with the development of pulmonary arterial hypertension (PAH). Other drugs ostensibly related in function have also been implicated as “likely” associated, including amphetamine, methamphetamine, and the serotonin precursor L-tryptophan. Serotonin signaling is thought to be a mediator of diet pill–associated PAH, and it is also thought to potentially play a significant role in PAH in general. In this article, we review the evidence supporting the serotonin hypothesis in PAH in both contexts, and the potential concerns related to selective serotonin reuptake inhibitors and other medications acting on serotonin signaling pathways.

Research paper thumbnail of A Case of Pulmonary Arterial Hypertension: If Only!

Advances in Pulmonary Hypertension

Research paper thumbnail of BMP 9/10 in Pulmonary Vascular Complications of Liver Disease

American Journal of Respiratory and Critical Care Medicine

Research paper thumbnail of Ambrisentan in portopulmonary hypertension: A multicenter, open label trial

The Journal of Heart and Lung Transplantation

Research paper thumbnail of Chronic thromboembolic pulmonary hypertension: an update for 2018

The purpose of this session is to review the history of our understanding of CTEPH and its pathop... more The purpose of this session is to review the history of our understanding of CTEPH and its pathophysiology and natural history. The diagnostic evaluation of a patient with suspected CTEPH will be reviewed as will current concepts in the surgical, interventional and medical treatment of the disease. Educational Objectives: At the end of this lecture, participants will be able to: A. Define CTEPH and describe its natural history. B. Review the diagnostic evaluation of a patient with suspected CTEPH. C. Describe the approach to intervention or medical therapy for CTEPH. CTEPH 2018 # 2 Definition, Pathophysiology and Epidemiology Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a pre-capillary elevation in pulmonary vascular resistance (PVR) resulting from chronic, "scarred-in" thromboembolic material partially occluding the pulmonary arteries. This vascular obstruction, over time, results in failure of the right side of the heart and early mortality. CTEPH was first described in the early 1900s and was further characterized in an autopsy series from the Massachusetts General Hospital in 1931. On these postmortem examinations, it was noted that the affected patients had large pulmonary artery vascular obstruction, relatively normal pulmonary parenchyma distal to the vascular obstruction, and the presence of extensive bronchial collateral blood flow or "compensatory circulation." [1] Still, it would take until the mid-20th century until imaging and cardiac catheterization techniques allowed the recognition of the diagnosis antemortem. The pathobiology of the disease remains poorly understood, but clinical evidence suggests that the process most certainly begins with an acute pulmonary embolus. (Figure 1) Rather than resolving over time, the embolus develops into organized, fibrotic thromboembolic material which adheres to and incorporates with the pulmonary artery wall. (Figure 2) These lesions tend to recanalize and may appear irregularly shaped, or like "bands" or "webs" inside the pulmonary artery. Histologic examination of the chronic thromboembolic material reveals intimal thickening composed of fibroblasts, collagen, lymphocytes and hemosiderin-laden macrophages. [2] Over time, this physical obstruction to the pulmonary vasculature raises the pulmonary vascular resistance and presents an increased workload to the right ventricle, which is geometrically, CTEPH 2018 # 3

Research paper thumbnail of Activated Protein C Attenuates Microvascular Injury During Systemic Hypoxia

Shock, 2007

In response to hypoxia, an inflammatory cascade is initiated and microvascular injury ensues. Spe... more In response to hypoxia, an inflammatory cascade is initiated and microvascular injury ensues. Specifically, within 10 min, leukocyte adherence to the endothelium begins, and leukocyte emigration and vascular leak soon follow. Activated protein C (APC) has been reported to have both anticoagulant and anti-inflammatory properties. Activated protein C is best described in its role as a treatment for sepsis. However, it has been used, with some success, in experimental models of hypoxic injury. We hypothesized that APC would be protective against microvascular injury during systemic hypoxia. Randomized prospective animal study. Adult male Sprague-Dawley rats. To characterize the microvascular response to APC exposure during hypoxia, four rat groups were used: saline control, APC infusion alone (100 mg/kg bolus), hypoxia alone (10% O2), and simultaneous hypoxia + APC infusion. Measurements of leukocyte adherence (no. per 100-microm venule), leukocyte emigration (no. per 4,000 microm(2)), and venular leak by fluorescein isothiacyanate-labeled albumin (Fo/Fi) were performed during intravital microscopy of the intact venular bed. Leukocyte adherence decreased from 14.5 (+/-1.2) cells/100-microm venule in hypoxic rats to 4.4 (+/-1.5) cells/100-microm venule in those treated with both hypoxic gas and APC infusion (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001). Similarly, leukocyte emigration in hypoxic rats reached 12.3 (+/- 2.2) cells/4,000-microm(2) venule, but was reduced to 3.5 (+/-0.3) cells/4,000-microm(2) venule (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;.001). Venular permeability to protein was also significantly decreased in the APC-treated group from 0.82 (+/-0.14) to 0.25 (+/-0.14) (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001). The infusion of APC…

Research paper thumbnail of ISHLT consensus statement: Perioperative management of patients with pulmonary hypertension and right heart failure undergoing surgery

The Journal of Heart and Lung Transplantation

Research paper thumbnail of Health disparities and treatment approaches in portopulmonary hypertension and idiopathic pulmonary arterial hypertension: an analysis of the Pulmonary Hypertension Association Registry

Pulmonary Circulation, 2021

Compared to idiopathic pulmonary arterial hypertension (IPAH), patients with portopulmonary hyper... more Compared to idiopathic pulmonary arterial hypertension (IPAH), patients with portopulmonary hypertension (POPH) have worse survival. Health disparities may contribute to these differences but have not been studied. We sought to compare socioeconomic factors in patients with POPH and IPAH and to determine whether socioeconomic status and/or POPH diagnosis were associated with treatment and health-care utilization. We performed a cross-sectional study of adults enrolled in the Pulmonary Hypertension Association Registry. Patients with IPAH (n = 344) and POPH (n = 57) were compared. Compared with IPAH, patients with POPH were less likely to be college graduates (19.6% vs. 34.9%, p = 0.02) and more likely to be unemployed (54.7% vs. 30.5%, p < 0.001) and have an annual household income below poverty level (45.7% vs. 19.0%, p < 0.001). Patients with POPH had similar functional class, quality of life, 6-min walk distance, and mean pulmonary arterial pressure with a higher cardiac index. Compared with IPAH, patients with POPH were less likely to receive combination therapy (46.4% vs. 62.2%, p = 0.03) and endothelin receptor antagonists (28.6% vs. 55.1%, p < 0.001) at enrollment with similar treatment at follow-up. Patients with POPH had more emergency department visits (1.7 ± 2.1 vs. 0.9 ± 1.2, p = 0.009) and hospitalizations in the six months preceding enrollment (1.5 ± 2.1 vs. 0.8 ± 1.1, p = 0.02). Both POPH diagnosis and lower education level were independently associated with a higher number of emergency department visits. Compared to IPAH, patients with POPH have lower socioeconomic status, are less likely to receive initial combination therapy and endothelin receptor antagonists but have similar treatment at follow-up, and have increased health-care utilization.

Research paper thumbnail of Health Disparities and Treatment Patterns in Portopulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension: An Analysis of the Pulmonary Hypertension Association Registry

B27. UP-TO-DATE PAH ASSESSMENT AND MANAGEMENT, 2020

Research paper thumbnail of Variable Creatinine Levels in Critical Care Patients: A Concerning Knowledge Gap

Journal of Clinical Medicine, 2021

An accurate creatinine (Cr) estimate is pivotal for the assessment of renal function. Both patien... more An accurate creatinine (Cr) estimate is pivotal for the assessment of renal function. Both patient- and practice-spawned factors palliate the test accuracy of serum creatinine (sCr) and can erratically represent actual kidney function. This study evaluated the caregivers’ awareness of enzymatic serum creatinine (E-sCr) assay interfering in dopamine/dobutamine (DD)-infused patient samples and the frequency of such interference in a critical care setting. We conducted an sCr awareness survey among UT Southwestern physicians, nurses, and pharmacists. We then performed a cross-sectional E-sCr comparison against the kinetic Jaffe method using the DD-infused patient samples collected from central venous catheters (CVC), peripherally inserted central catheter (PICC) lines, and the peripheral vein (PV). We retrospectively compared the longitudinal E-sCr results of the CVC/PICC draws with the corresponding blood urea nitrogen (BUN) levels. The survey results show a significant lack of awaren...

Research paper thumbnail of in Parenchymal Lung Disease

The prevalence of pulmonary hypertension (PH) in parenchymal lung disease is common. The pathophy... more The prevalence of pulmonary hypertension (PH) in parenchymal lung disease is common. The pathophysiology of pulmonary hypertension (PH) in parenchymal lung diseases is partially, if not mostly, related to hypoxic pulmonary vasoconstriction. The pathophysiology of PH in chronic obstructive pulmonary disease is poorly described, but likely involves an interaction between hypoxic vasoconstriction, vascular inflammation, and a loss of capillaries in the diseased lung. Restrictive lung diseases are characterized by pulmonary parenchymal abnormalities rather than the obstruction of airflow and are often associated with PH through hypoxic pulmonary vasoconstriction and varying degrees of inflammation. Systemic disorders can also affect the pulmonary parenchyma and be associated with PH. PH treatment is controversial for patients with parenchymal lung disease.

Research paper thumbnail of Long Term Survival and Transplant Rates in Pulmonary Arterial Hypertension Patients Treated with Parenteral Prostacyclin Therapy

Chest, 2020

Pulmonary arterial hypertension (PAH), is a fatal disease with high morbidity and mortality. Curr... more Pulmonary arterial hypertension (PAH), is a fatal disease with high morbidity and mortality. Current guidelines recommend parenteral prostacyclin treatment as mainstay of treatment in severely ill patients.However, there is lack of published data on long term outcomes in these patients. Here we describe long-term outcomes in PAH patients on intravenous/ subcutaneous (IV/SC) therapy. METHODS: We performed a retrospective chart review of all patients initiated on parenteral prostacyclin therapy (IV epoprostenol or IV/SC treprostinil) between 2007 and 2016. Patients with portopulmonary hypertension were excluded. Analyses include survival time (censored at transplant), time from diagnosis to IV therapy start, and time from diagnosis to lung transplantation. Statistical analysis was carried out using NCSS software.

Research paper thumbnail of Transitioning Stable Pulmonary Arterial Hypertension Patients from Parenteral Prostacyclin Therapy to Oral Selexipag

B56. PULMONARY HYPERTENSION CLINICAL TRIALS ENDEAVOR SAFETY, COMBINATION WHAT ELSE?, 2020

Research paper thumbnail of Estrogen Metabolism in Portopulmonary Hypertension (POPH): A Case-Control Study

C52. USE YOUR ILLUSION II: CLINICAL RESEARCH IN PAH, 2019

Research paper thumbnail of Quality of Life of Patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Idiopathic Pulmonary Arterial Hypertension (IPAH): The Pulmonary Hypertension Association Registry (PHAR)

C104. LATEST CLINICAL INVESTIGATIONS IN ACUTE AND CHRONIC THROMBOEMBOLISM DISORDERS OF THE PULMONARY CIRCULATION, 2020

Research paper thumbnail of Long-term outcomes in pulmonary arterial hypertension in the first-line epoprostenol or first-line bosentan era

The Journal of Heart and …, 2010

In a single-center, retrospective, longitudinal cohort, data on right heart catheterization, 6-mi... more In a single-center, retrospective, longitudinal cohort, data on right heart catheterization, 6-minute walk distance (6MWD), disease progression and mortality were collected. Outcomes were assessed in first-line bosentan and first-line epoprostenol patients. To reduce selection bias due to ...

Research paper thumbnail of EXPRESS: Health Related Quality of Life and Hospitalizations In Chronic Thromboembolic Pulmonary Hypertension Versus Idiopathic Pulmonary Arterial Hypertension: An Analysis from the Pulmonary Hypertension Association Registry(PHAR)

Pulmonary Circulation

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, morbid, potentially curable subt... more Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, morbid, potentially curable subtype of pulmonary hypertension that negatively impacts health-related quality of life (HRQoL). Little is known about differences in HRQoL and hospitalization between CTEPH patients and idiopathic pulmonary arterial hypertension (IPAH) patients. Using multivariable linear regression and mixed effects models, we examined differences in HRQoL assessed by emPHasis-10 (E10) and SF-12 between CTEPH and IPAH patients in the Pulmonary Hypertension Association Registry, a prospective multicenter cohort of patients newly evaluated at a Pulmonary Hypertension Care Center. Multivariable negative binomial regression models were used to estimate incidence rate ratios (IRR) for hospitalization amongst the two groups. We included 461 IPAH and 169 CTEPH patients. 21% of CTEPH patients underwent pulmonary thrombendarterectomy (PTE) before the end of follow-up. At baseline, patients with CTEPH had significa...

Research paper thumbnail of Parenteral prostanoids for severe Group 3 pulmonary hypertension with right ventricular dysfunction

Journal of Thoracic Disease

Background: Group 3 pulmonary hypertension (PH) is a common complication in patients with lung di... more Background: Group 3 pulmonary hypertension (PH) is a common complication in patients with lung diseases but there are currently no FDA-approved therapies. The data is conflicting, but a few small studies suggest potential benefits in using Group 1 PH therapies in these patients, particularly in severe PH with right ventricular (RV) dysfunction. Methods: A retrospective cohort study of patients with severe Group 3 PH with RV dysfunction who received parenteral prostanoids from 2007-2018 at our institution was undertaken. Severe PH was defined as mean pulmonary arterial pressure (mPAP) ≥35 mmHg or mPAP 25-34 with cardiac index (CI) <2.4 L/min/m 2. Routine prognostic studies including N-terminal prohormone of brain natriuretic peptide (NT-proBNP), 6-minute walk distance (6MWD), WHO Functional Class assessment, oxygen requirement, arterial oxygen saturation, right ventricular systolic pressure (RVSP) and right heart catheterization (RHC) pressures, were obtained before initiation of parenteral therapy and at first clinical follow-up. Results: Nine patients were included. Five were female (55.6%) with a median [interquartile range (IQR)] of 69 [54-71] years. Median CI was 1.8 (1.6-2.4) L/min/m 2 and median pulmonary vascular resistance (PVR) was 14.7 (10.7-17.1) Wood units (WU). We found no statistically significant improvement in NT-proBNP levels, exercise capacity, or functional class. Resting oxygen requirement worsened from 4 to 6 L/min (P=0.04) and exertional oxygen saturation nadir worsened from 90% to 83% (P=0.01) despite the increase in FiO 2 with exertion. Overall results were heterogenous: several patients demonstrated clinical stabilization, with two undergoing lung transplantation and one showing long-term stability with medical therapy. Symptoms remained severe for most: three patients discontinued prostanoid therapy, choosing to pursue hospice care. Conclusions: We found no statistically significant improvement in NT-proBNP levels, exercise capacity, or functional class, while oxygen requirement at rest and oxygen saturation during exertion significantly worsened. Our results suggest that parenteral prostanoids should not generally be considered in the treatment of Group 3 PH patients.

Research paper thumbnail of Estrogen Signaling and Portopulmonary Hypertension: The Pulmonary Vascular Complications of Liver Disease Study (PVCLD2)

Research paper thumbnail of Pulmonary Hypertension and Chronic Obstructive Pulmonary Disease: When Is It Out of Proportion?

Advances in Pulmonary Hypertension

Patients with chronic obstructive pulmonary disease (COPD) often present with mild pulmonary hype... more Patients with chronic obstructive pulmonary disease (COPD) often present with mild pulmonary hypertension (PH). This finding has been attributed to hypoxic pulmonary vasoconstriction. However, a small proportion of COPD patients will present with moderate or severe elevations in their pulmonary artery pressure (PAP), and these patients appear to have worsened symptoms and survival when compared to patients with milder elevations in PAP. The diagnosis of PH in COPD may be difficult, due to inaccuracies in the echocardiographic estimates of PAP in these patients. Additionally, many patients with COPD will also have comorbid conditions such as diastolic heart failure, systolic heart failure, or obstructive sleep apnea, which may cause increased pulmonary pressures through other mechanisms. Clinical trials investigating the effect of PH-specific therapy for patients with PH and COPD have been small, with mixed results. A careful evaluation for other causes of PH and hemodynamic evaluati...

Research paper thumbnail of Anorexigen-Associated Pulmonary Arterial Hypertension and the Serotonin Hypothesis: A Story Worth Telling

Advances in Pulmonary Hypertension

Diet pills such as aminorex, fenfluramine, and dexfenfluramine have been strongly associated with... more Diet pills such as aminorex, fenfluramine, and dexfenfluramine have been strongly associated with the development of pulmonary arterial hypertension (PAH). Other drugs ostensibly related in function have also been implicated as “likely” associated, including amphetamine, methamphetamine, and the serotonin precursor L-tryptophan. Serotonin signaling is thought to be a mediator of diet pill–associated PAH, and it is also thought to potentially play a significant role in PAH in general. In this article, we review the evidence supporting the serotonin hypothesis in PAH in both contexts, and the potential concerns related to selective serotonin reuptake inhibitors and other medications acting on serotonin signaling pathways.

Research paper thumbnail of A Case of Pulmonary Arterial Hypertension: If Only!

Advances in Pulmonary Hypertension

Research paper thumbnail of BMP 9/10 in Pulmonary Vascular Complications of Liver Disease

American Journal of Respiratory and Critical Care Medicine

Research paper thumbnail of Ambrisentan in portopulmonary hypertension: A multicenter, open label trial

The Journal of Heart and Lung Transplantation