Acute vasodilator testing: An opportunity to advance the precision care of pulmonary hypertension

Meghan M Cirulis1, Lynda O Jorgensen2, Ronald W Day2

  • 1Division of Pulmonary Medicine (Fellow), Department of Internal Medicine, University of Utah, 50 North Medical Drive, Salt Lake City, UT, 84132, USA.

Insights

A child with severe pulmonary arterial hypertension showed immediate normalization of pulmonary arterial pressure with nitric oxide. This acute response to inhaled nitric oxide persisted, suggesting its value in personalized pulmonary hypertension treatment.

Area of Science:

  • Cardiology
  • Pulmonology
  • Pediatrics

Background:

  • Pulmonary arterial hypertension (PAH) is a severe condition characterized by high blood pressure in the lung arteries.
  • Accurate assessment of pulmonary vascular response is crucial for effective PAH management.

Observation:

  • An 8-year-old child with severe PAH experienced immediate normalization of pulmonary arterial pressure during inhaled nitric oxide administration.
  • This acute responsiveness to nitric oxide was maintained throughout the patient's life.

Findings:

  • The patient's acute vasodilator testing responses correlated with long-term medication efficacy.
  • Inhaled nitric oxide demonstrated a consistent and sustained effect on pulmonary arterial pressure.

Implications:

  • Acute vasodilator testing, particularly with inhaled nitric oxide, can predict long-term treatment responses in PAH.
  • This approach may enhance precision medicine strategies for pediatric and adult pulmonary hypertension patients.

Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
591
Pulmonary Function Tests01:25

Pulmonary Function Tests

Pulmonary Function Tests (PFTs)
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
767
Acute Kidney Injury V: Interprofessional Care01:20

Acute Kidney Injury V: Interprofessional Care

Acute Kidney Injury (AKI) requires a collaborative healthcare approach to restore renal function and prevent complications. Essential management strategies involve monitoring fluid and electrolyte balance, adjusting medications, initiating dialysis when necessary, and providing nutritional support.Fluid and Electrolyte ManagementFluid Monitoring: Regularly monitoring body weight, central venous pressure, and urine output helps detect fluid imbalances early. Patient intake and output are...
305
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

Phosphodiesterase 5 (PDE5) inhibitors are potent enzymes that function to hydrolyze cyclic nucleotides to their corresponding 5' monophosphates. Their unique biochemical properties have been applied in treating Pulmonary Arterial Hypertension (PAH).
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...
577
Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care01:29

Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care

Diagnosing Pulmonary EmbolismDiagnosing pulmonary embolism (PE) involves clinical assessment and advanced imaging tests. The preferred diagnostic tool is the spiral (helical) CT scan or CT angiography (CTA), which uses intravenous contrast media to visualize the pulmonary vasculature and identify emboli.A ventilation-perfusion (V/Q) scan is an alternative for patients unable to receive contrast media. This scan includes both perfusion and ventilation scanning. Perfusion scanning involves...
349
Acute Coronary Syndrome IV: Interprofessional Care01:28

Acute Coronary Syndrome IV: Interprofessional Care

IntroductionThe management of Acute Coronary Syndrome (ACS) aims to minimize myocardial damage, preserve myocardial function, and prevent complications.Initial ManagementInpatient management involves continuous cardiac monitoring, preferably in an ICU, focusing on blood pressure, serum sodium, potassium, and creatinine levels, and urine output. Ongoing pharmacologic management is crucial for stabilizing the patient.Supplemental Oxygen: Administer supplemental oxygen if oxygen saturation is...
230