Association Between Thrombus Histopathology and Hemodynamic Outcomes Among Patients With Chronic Thromboembolic Pulmonary Hypertension Undergoing Pulmonary Endarterectomy.

Background: Pulmonary endarterectomy (PEA) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH). Although most have normalization of pulmonary artery (PA) pressures, development of residual pulmonary hypertension (RPH) is challenging to predict.

Research Question: Among patients with CTEPH undergoing PEA, is there a relationship between specimen histopathology and postoperative hemodynamics?

Study Design And Methods: In this single-center retrospective cohort study, patients with CTEPH who underwent PEA were classified by thrombus chronicity: organized (chronic), organizing (subacute), or mixed (combined organizing and organized).

Hereditary hemorrhagic telangiectasia may be the most morbid inherited bleeding disorder in women.

Hereditary hemorrhagic telangiectasia (HHT) is the second-most common inherited bleeding disorder (BD) worldwide and remains without approved therapies. HHT causes serious mucosal bleeding resulting in severe iron-deficiency anemia, major psychosocial complications, and visceral arteriovenous malformations in the brain, lung, and liver, which can cause life-threatening hemorrhagic complications. No study has examined the relative morbidity of HHT and von Willebrand disease (VWD), which is the most common inherited BD in women.

Elevations in Norclobazam Concentrations and Altered Mental Status in CYP2C19 Poor Metabolizer Phenotype: A Case Report.

Clobazam is a 1,5-benzodiazepine frequently used as an adjunctive agent for refractory seizures and status epilepticus. Clobazam undergoes metabolism to an active metabolite norclobazam which is subsequently hydroxylated by CYP2C19, a cytochrome with several pharmacogenetic variants. Patients with poor metabolizer phenotypes may have elevated norclobazam levels and subsequent adverse effects.

Anticoagulation and antiplatelet therapy in hereditary hemorrhagic telangiectasia: A scoping review.

Introduction: Data describing safety and tolerability of anticoagulation and antiplatelet therapy in hereditary hemorrhagic telangiectasia (HHT), the second-most-common inherited bleeding disorder, is limited.

Methods: We performed a scoping review, searching MEDLINE and EMBASE from inception to March 2023 for eligible studies reporting detailed clinical data describing antithrombotic use in HHT. Data extracted included study design, patient population, and characteristics and outcomes of antithrombotic therapy.

Safety, tolerability, and effectiveness of anticoagulation and antiplatelet therapy in hereditary hemorrhagic telangiectasia.

Background: Antithrombotic therapy (anticoagulation and antiplatelet therapy) is frequently needed in patients with hereditary hemorrhagic telangiectasia (HHT); however, data describing and guiding its use are very limited.

Objectives: To investigate the safety, tolerability, and effectiveness of antithrombotic therapy in HHT in a cohort large enough to compare agents, evaluate for baseline predictors of premature discontinuation, and evaluate hematologic support requirements and healthcare utilization before and after antithrombitc therapy initiation.

Methods: We performed a multicenter observational cohort study characterizing the outcomes of antithrombic therapy in adults with HHT.

Perfusion imaging heterogeneity during NO inhalation distinguishes pulmonary arterial hypertension (PAH) from healthy subjects and has potential as an imaging biomarker.

Background: Without aggressive treatment, pulmonary arterial hypertension (PAH) has a 5-year mortality of approximately 40%. A patient's response to vasodilators at diagnosis impacts the therapeutic options and prognosis. We hypothesized that analyzing perfusion images acquired before and during vasodilation could identify characteristic differences between PAH and control subjects.

Safety of macitentan for the treatment of pulmonary hypertension: Real-world experience from the OPsumit® USers Registry (OPUS) and OPsumit® Historical USers cohort (OrPHeUS).

Macitentan is an oral endothelin receptor antagonist for the management of pulmonary arterial hypertension (PAH). The OPsumit® USers Registry (OPUS) and the OPsumit® Historical USers cohort (OrPHeUS) medical chart review provide real-world data for patients newly initiating macitentan. This study aims to describe the characteristics, safety profile, and clinical outcomes of PAH patients newly treated with macitentan in the combined OPUS/OrPHeUS data set.

Inhaled high dose nitric oxide is a safe and effective respiratory treatment in spontaneous breathing hospitalized patients with COVID-19 pneumonia.

Background: Inhaled nitric oxide (NO) is a selective pulmonary vasodilator. In-vitro studies report that NO donors can inhibit replication of SARS-CoV-2. This multicenter study evaluated the feasibility and effects of high-dose inhaled NO in non-intubated spontaneously breathing patients with Coronavirus disease-2019 (COVID-19).

Treatment of pulmonary hypertension in patients with Hereditary Hemorrhagic Telangiectasia - A case series and systematic review.

Rationale: Pulmonary Arterial Hypertension (PAH), a rare complication of HHT is associated with poor outcome. There are no trials to date that have investigated whether pulmonary vasodilator therapy improves hemodynamics or survival in this disease.

Objective: To determine whether pulmonary vasodilator therapy improves survival, exercise capacity, or hemodynamics in HHT patients with pre-capillary PH.

Cerebrovascular and cardiovascular disease burden in patients with hereditary hemorrhagic telangiectasia.

Introduction: Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disease with prevalence of approximately 1 in 5000-10,000. We evaluated the prevalence and association of cerebrovascular and cardiovascular comorbidities in HHT patients using national database.

Methods: Retrospective observational study was performed using National Inpatient Sampling (NIS) database for the year 2014.

Room for improvement in pulmonary capillary wedge pressure reporting: a review of hemodynamic tracings at a large academic medical center.

To describe the frequency with which pulmonary capillary wedge pressure measurements, obtained during right heart catheterization, are falsely elevated and to educate operators on techniques to improve accuracy of pulmonary capillary wedge pressure reporting. Failure to completely occlude pulmonary artery branch vessels during balloon inflation can lead to falsely elevated, "incomplete" pulmonary capillary wedge pressures. Balloon deflation prior to catheter retraction may result in catheter advancement into smaller branch vessels, yielding an inadvertent but more accurate alternative pulmonary capillary wedge pressure.

Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia.

Description: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications.

An international, multicenter study of intravenous bevacizumab for bleeding in hereditary hemorrhagic telangiectasia: the InHIBIT-Bleed study.

Hereditary hemorrhagic telangiectasia (HHT, Osler-Weber-Rendu disease) is a rare multisystem vascular disorder causing chronic gastrointestinal bleeding, epistaxis, and severe anemia. Bevacizumab, an anti-vascular endothelial growth factor antibody, may be effective to treat bleeding in HHT. This international, multicenter, retrospective study evaluated the use of systemic bevacizumab to treat HHT-associated bleeding and anemia at 12 HHT treatment centers.

Ammonia Predicts Hepatic Involvement and Pulmonary Hypertension in Patients With Hereditary Hemorrhagic Telangiectasia.

Introduction: Hepatic involvement in hereditary hemorrhagic telangiectasia (HHT) is common and can be associated with severe clinical consequences, including portal hypertension, cardiac failure, and encephalopathy. However, there are no reliable clinical predictors of hepatic involvement and its associated complications, limiting appropriate identification of these patients. In this work, we define the utility of serum ammonia and liver biochemical tests (LFTs) in predicting hepatic HHT involvement and its complications.

Diagnosis, Treatment and Follow Up of Acute Pulmonary Embolism: Consensus Practice from the PERT Consortium.

Pulmonary embolism (PE) is a life-threatening condition and a leading cause of morbidity and mortality. There have been many advances in the field of PE in the last few years, requiring a careful assessment of their impact on patient care. However, variations in recommendations by different clinical guidelines, as well as lack of robust clinical trials, make clinical decisions challenging.

Correction to: Changes in treatment and outcomes after creation of a pulmonary embolism response team (PERT), a 10-year analysis.

The original version of the article unfortunately contained an error in conflict of interest. This erratum is published with the correct conflict of interest.

Changes in treatment and outcomes after creation of a pulmonary embolism response team (PERT), a 10-year analysis.

Multidisciplinary pulmonary embolism response teams (PERTs) are being implemented to improve care of patients with life-threatening PE. We sought to determine how the creation of PERT affects treatment and outcomes of patients with serious PE. A pre- and post-intervention study was performed using an interrupted time series design, to compare patients with PE before (2006-2012) and after (2012-2016) implementation of PERT at a university hospital.

Surgical Management of Acute and Chronic Pulmonary Embolism.

Surgical pulmonary embolectomy and pulmonary thromboendarterectomy are well-established treatment strategies for patients with acute and chronic pulmonary embolism, respectively. For both procedures, techniques and outcomes have evolved considerably over the past decades. Patients with massive and submassive acute pulmonary embolism are at risk for rapid decline owing to right ventricular failure and shock.

Quantifying the learning curve for pulmonary thromboendarterectomy.

Background: Pulmonary thromboendarterectomy (PTE) is an effective treatment for chronic thromboembolic pulmonary hypertension (CTEPH), but is a technically challenging operation for cardiothoracic surgeons. Starting a new program allows an opportunity to define a learning curve for PTE.

Methods: A retrospective case review was performed of 134 consecutive PTEs performed from 1998 to 2016 at a single institution.

Health Disparities in Patients with Pulmonary Arterial Hypertension: A Blueprint for Action. An Official American Thoracic Society Statement.

Background: Health disparities have a major impact in the quality of life and clinical care received by minorities in the United States. Pulmonary arterial hypertension (PAH) is a rare cardiopulmonary disorder that affects children and adults and that, if untreated, results in premature death. The impact of health disparities in the diagnosis, treatment, and clinical outcome of patients with PAH has not been systematically investigated.