Liezl Domingo, H. Sonali Magdo, Ronald W. Day
University of Utah and Primary Children’s Hospital.
United States
Pediatric Cardiology
Pediatr Cardiol 2018; 39: 501-508
DOI: 10.1007/s00246-017-1780-9
Abstract
Results of acute pulmonary vasodilator testing (AVT) and the outcome of medical therapy have not been described in patients with segmental pulmonary vascular disease (SPVD). We sought to compare the pulmonary vasodilatory effects of oxygen, oxygen with nitric oxide, and diltiazem, and to describe the clinical course of patients with SPVD and pulmonary hypertension. A retrospective review of 16 patients with pulmonary hypertension and SPVD involving 2-3 major lung segments who underwent AVT between January 2000 and December 2015 was performed. Baseline hemodynamic measurements were obtained with patients breathing ≤ 30% oxygen. AVT was performed using 100% oxygen, 100% oxygen with 20 ppm nitric oxide, 21-35% oxygen, and 21-35% oxygen with intravenous diltiazem. The events associated with their long-term care were described. Nine of 16 patients were acutely responsive during AVT using the Sitbon criteria. The change in mean pulmonary artery pressure with oxygen or oxygen with nitric oxide (19 ± 12 mmHg) was significantly greater than the change with diltiazem (7 ± 5 mmHg). Pulmonary vasodilator therapy was initiated or escalated after AVT in 12 patients. Five patients subsequently experienced a decrease in mean pulmonary artery pressure or normalization in B-type natriuretic peptide. Three patients experienced adverse events associated with therapy. The actuarial survival was 94% over a period of 1-20 years. This study suggests that AVT can be used to identify patients with SPVD who are reactive to oxygen, oxygen with nitric oxide, and diltiazem. Clinical improvement was temporally associated with pulmonary vasodilator therapy in some patients with few adverse effects.
Category
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins. Efficacy or Lack of Efficacy
Length of Life Associated with Pulmonary Venous Obstruction
Diagnostic Testing. Invasive Medical Therapy for Pulmonary Hypertension Associated with Pulmonary Venous Obstruction
Medical Therapy. Adverse Effects or Lack of Adverse Effects
Year of Publication: 2018
Age Focus: Pediatric
Article Type: Retrospective Observational Cohort Studies (>10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No