Volume 22: Issue 1

BOSENTAN DECREASES RIGHT VENTRICULAR SYSTOLIC PRESSURE AND PRO-BRAIN NATRIURETIC PEPTIDE LEVELS IN INFANTS WITH PULMONARY HYPERTENSION

Lily M. Landry

Department of Pediatrics, Division of Pediatric Cardiology University of Mississippi Medical Center, Jackson, MS, USA

Andrew C. Burks

School of Medicine, University of Mississippi Medical Center, Jackson, MS, USA

Onyekachukwu Osakwe

Department of Pediatrics, Division of Pediatric Cardiology, University of Mississippi Medical Center, Jackson, MS, USA

Jarrod D. Knudson

Department of Pediatrics, Division of Pediatric Critical Care, University of Mississippi Medical Center, Jackson, MS, USA

Christopher L. Jenks

Department of Pediatrics, Division of Pediatric Critical Care, University of Mississippi Medical Center,

Pulmonary arterial hypertension (PAH) is a devastating disease mediated by vasoconstriction and vascular remodeling of the pulmonary vasculature. Current therapies target the imbalance of vasoconstrictors and vasorelaxants in 3 pathways: nitric oxide, prostacyclin, and endothelin. While these have extended lifespans for PAH patients, significant morbidity and mortality remains. Notably, the progress in PAH therapy for over a decade has utilized these same 3 pathways. Fortunately, several new treatment options utilizing different mechanisms are emerging and will be reviewed here.

Background

For decades, pulmonary hypertension (PH) used to be defined by a mean pulmonary artery pressure (mPAP) ≥25 mm Hg; however, this criterion was not based on data that were systematically collected. With the availability of contemporary datasets however, it was evident that the upper limit of normal mPAP was ∼20 mm Hg, which is also the level of mPAP above which adverse outcomes increase. In addition, it is now evident that the specificity of mPAP >20 mm Hg to denote precapillary pulmonary vascular disease could be enhanced by adding pulmonary vascular resistance (PVR) to the precapillary PH definition. Finally, after characterizing large groups of normal individuals, akin to observations for mPAP, it was recently demonstrated that a PVR of ∼2.0 Wood units (WU) is the upper limit of normal, and the lower level associated with all-cause mortality in at-risk patients.

Clinical Implications

The current hemodynamic criteria for PH are positioned to capture more patients compared to the classical definition, with particular implications for earlier diagnosis. Importantly, pulmonary vasodilator therapies have not been tested adequately in patients with mPAP <25 mm Hg or PVR between 2 to 3 WU and, thus, should not be administered in these patients. Mild PH is an active focus of clinical trial design; at present, these patients should be referred to expert PH centers earlier for individualized therapeutic planning.

Conclusions

The revised definition of precapillary PH uses a PVR threshold of >2 WU. This value is evidence-based, and exceeding this threshold is associated with adverse clinical outcomes. This revision places focus on early diagnosis, close monitoring, and consideration for certain treatments. Further studies are needed that test the efficacy and safety of pulmonary arterial hypertension-specific therapy in precapillary PH patients with PVR 2 to 3 WU.

Comprehensive serial risk assessment in pulmonary arterial hypertension has shown to determine prognosis, monitor disease progression, and guide treatment decisions. The treatment goal is to achieve a low-risk status, which is associated with lower mortality rate. However, use of formal risk assessment in clinical practice has been inconsistent due to numerous barriers related to the multivariable nature of the scores. This publication reviews strategies to increase risk evaluation in daily clinical practice, while emphasizing the role of the RN and APRN in implementing risk assessment calculation and skillful communication to the patient-family dyad to promote open dialogue with shared decision making and improved patient outcomes.

Full Issue PDF

Guest Editor’s Memo

PHA 2022 International Pulmonary Hypertension Conference Abstracts

BOSENTAN DECREASES RIGHT VENTRICULAR SYSTOLIC PRESSURE AND PRO-BRAIN NATRIURETIC PEPTIDE LEVELS IN INFANTS WITH PULMONARY HYPERTENSION

The Future of PAH Treatment

POINT: Is It Time to Lower the Cut-off for Increased Pulmonary Vascular Resistance? Yes

Background

Clinical Implications

Conclusions

COUNTERPOINT: Pulmonary Vascular Resistance 2.0— Shedding Light or Casting Shadows?

PH Roundtable: Pros and Cons of the 2022 ERS/ESC Guidelines: Practicality vs Real World View

PH Professional Network: Real-World Implementation: Nursing Role in Balancing the Art and Science of PAH Risk Assessment

Conference Abstracts

Guest Editor’s Memo

A Roundtable Discussion Addressing Patient-Centered Care and Quality of Life in Patients With Pulmonary Arterial Hypertension

Editor’s Memo

Pulmonary Hypertension Associated With Parenchymal Lung Disease