417: PSYCHIATRIC DISEASE CHARACTERIZATION OF PULMONARY HYPERTENSION BY WHO CLASSIFICATION

Right heart catheterization is an essential diagnostic modality in the evaluation of pulmonary hypertension. The coronavirus disease 2019 pandemic has resulted in deferral of elective procedures including right heart catheterization. The benefits of proceeding with right heart catheterization, such as further characterization of hemodynamic subtype and severity of pulmonary hypertension, initiation of targeted pulmonary arterial hypertension therapy, as well as further hemodynamic testing, need to be carefully balanced with the risk of potentially exposing both patients and health care personnel to coronavirus disease 2019 infection. This review article aims to provide best clinical practices for safely performing right heart catheterization in pulmonary hypertension patients during the coronavirus disease 2019 pandemic.

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Induction and Characterization of Pulmonary Hypertension in Mice using the Hypoxia/SU5416 Model

Journal of Visualized Experiments ◽

10.3791/59252 ◽

2020 ◽

Author(s):

Olympia Bikou ◽

Roger J. Hajjar ◽

Lahouaria Hadri ◽

Yassine Sassi

Keyword(s):

Pulmonary Hypertension

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PULMONARY HYPERTENSION IN PAEDIATRIC PATIENTS: DATA FROM THE COMPERA-KIDS REGISTRY

Archives of Disease in Childhood ◽

10.1136/archdischild-2015-310148.51 ◽

2015 ◽

Vol 101 (1) ◽

pp. e1.47-e1

Author(s):

Matthias Gorenflo ◽

David Pittrow ◽

Dörte Huscher ◽

Victoria Ziesenitz ◽

Joseph Pattathu ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Treatment Options ◽

Anticoagulation Therapy ◽

Right Heart Catheterization ◽

Functional Class ◽

Right Atrial ◽

Heart Catheterization ◽

Study Cohort ◽

Paediatric Patients

BackgroundPulmonary hypertension (PH/PAH) can have many possible causes in childhood. The aim of the COMPERA registry is the characterization of patients of all ages with PH/PAH and their treatment patterns.MethodsSince June 2013, paediatric patients can be included in the COMPERA registry (ClinTrials.gov: NCT01347216) which has originally been established for adult patients with pulmonary hypertension in 2007.ResultsUntil 2015, 78 patients <18 years (47 Pat <6 years) with pulmonary hypertension were enrolled, of whom 65.4% had PAH due to congenital heart disease (PAH-CHD), 25.6% had idiopathic PAH (iPAH), 3 had persistent PH of the newborn, 2 had PH associated with interstitial lung disease, and two had other PH. The patients were 6.1±6.0 years old, 52.6% girls; NYHA functional class I/II in 55.0%, and III in 42.3%. Mean disease duration after diagnosis was 37.7±55.8 months. Right heart catheterization data were available for 82.1% of the patients. Mean pulmonary artery pressure was 42.8±19.4 mmHg, right atrial pressure was 8.0±8.8 mmHg, cardiac index was 3.6±1.2 l/min/m2. Monotherapy was received by 65.4% of the patients whereas 33.4% of the patients had combination therapy. Phosphodiesterase-5 inhibitors (PDE5I) were administered to 76.9% of the patients, 35.9% of the patients received endothelin receptor antagonists and 3.8% received prostacyclins. About 24% of patients received anticoagulation therapy.ConclusionThe most common form of PH in this study cohort is PAH-CHD, followed by iPAH. Treatment options for children primarily comprise PDE5I. Only a small number of paediatric patients receive anticoagulation therapy.

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Experimental intravascular hemolysis induces hemodynamic and pathological pulmonary hypertension: association with accelerated purine metabolism

Pulmonary Circulation ◽

10.1177/2045894018791557 ◽

2018 ◽

Vol 8 (3) ◽

pp. 204589401879155 ◽

Cited By ~ 5

Author(s):

Victor P. Bilan ◽

Frank Schneider ◽

Enrico M. Novelli ◽

Eric E. Kelley ◽

Sruti Shiva ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Rat Model ◽

Vascular Remodeling ◽

Purine Nucleoside Phosphorylase ◽

Autologous Blood ◽

Systolic Pressure ◽

Per Se ◽

Parenchymal Injury

Pulmonary hypertension (PH) is emerging as a serious complication associated with hemolytic disorders, and plexiform lesions (PXL) have been reported in patients with sickle cell disease (SCD). We hypothesized that repetitive hemolysis per se induces PH and angioproliferative vasculopathy and evaluated a new mechanism for hemolysis-associated PH (HA-PH) that involves the release of adenosine deaminase (ADA) and purine nucleoside phosphorylase (PNP) from erythrocytes. In healthy rats, repetitive administration of hemolyzed autologous blood (HAB) for 10 days produced reversible pulmonary parenchymal injury and vascular remodeling and PH. Moreover, the combination of a single dose of Sugen-5416 (SU, 200 mg/kg) and 10-day HAB treatment resulted in severe and progressive obliterative PH and formation of PXL (Day 26, right ventricular peak systolic pressure (mmHg): 26.1 ± 1.1, 41.5 ± 0.5 and 85.1 ± 5.9 in untreated, HAB treated and SU+HAB treated rats, respectively). In rats, repetitive administration of HAB increased plasma ADA activity and reduced urinary adenosine levels. Similarly, SCD patients had higher plasma ADA and PNP activity and accelerated adenosine, inosine, and guanosine metabolism than healthy controls. Our study provides evidence that hemolysis per se leads to the development of angioproliferative PH. We also report the development of a rat model of HA-PH that closely mimics pulmonary vasculopathy seen in patients with HA-PH. Finally, this study suggests that in hemolytic diseases released ADA and PNP may increase the risk of PH, likely by abolishing the vasoprotective effects of adenosine, inosine and guanosine. Further characterization of this new rat model of hemolysis-induced angioproliferative PH and additional studies of the role of purines metabolism in HA-PH are warranted.

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