title: Management of pulmonary arterial hypertension during pregnancy: a retrospective, multicenter experience.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) is a rare disease with a predilection for young women that is associated with right ventricular failure and premature death. PAH can complicate pregnancy with hemodynamic instability or sudden death during parturition and postpartum. Our aim was to examine the impact of PAH on pregnancy outcomes in the modern era.
  
  
  METHODS
  We conducted a retrospective evaluation of pregnant patients with PAH managed between 1999 and 2009 at five US medical centers. Patient demographics, medical therapies, hemodynamic measurements, manner of delivery, anesthetic administration, and outcomes were assessed.
  
  
  RESULTS
  Among 18 patients with PAH, 12 continued pregnancy and six underwent pregnancy termination. Right ventricular systolic pressure in patients managed to parturition was 82 ± 5 mm Hg and in patients with pregnancy termination was 90 ± 16 mm Hg. Six patients underwent pregnancy termination at mean gestational age of 13 ± 1.0 weeks with no maternal deaths or complications. Twelve patients elected to continue their pregnancy and were hospitalized at 29 ± 1.4 weeks. PAH-specific therapy was administered to nine (75%) at time of delivery consisting of sildenafil, IV prostanoids, or combination therapy. All parturients underwent Cesarean section at 34 weeks with one in-hospital death and one additional death 2 months postpartum for maternal mortality of 16.7%.
  
  
  CONCLUSIONS
  Compared with earlier reports, maternal morbidity and mortality among pregnant women with PAH was reduced, yet maternal complications remain significant and patients should continue to be counseled to avoid pregnancy.
authors.affiliation: Division of Pulmonary/Critical Care Medicine, The University of Texas Medical Branch at Galveston, Galveston, TX. Electronic address: aduarte@utmb.edu.
authors.forename: Alexander G
authors.initials: AG
authors.lastname: Duarte
authors.affiliation: Division of Pulmonary/Critical Care Medicine, The University of Texas Medical Branch at Galveston, Galveston, TX.
authors.forename: Shibu
authors.initials: S
authors.lastname: Thomas
authors.affiliation: Pulmonary/Critical Care Medicine, Baylor College of Medicine, Houston, TX.
authors.forename: Zeenat
authors.initials: Z
authors.lastname: Safdar
authors.affiliation: Pulmonary/Critical Care Medicine, The University of Texas Southwestern Medical Center, Dallas, TX.
authors.forename: Fernando
authors.initials: F
authors.lastname: Torres
authors.affiliation: Department of Obstetrics and Gynecology and Department of Anesthesiology, The University of Texas Medical Branch at Galveston, Galveston, TX.
authors.forename: Luis D
authors.initials: LD
authors.lastname: Pacheco
authors.affiliation: Arizona Pulmonary Specialists Ltd, Phoenix, AZ.
authors.forename: Jeremy
authors.initials: J
authors.lastname: Feldman
authors.affiliation: Pulmonary and Critical Care Medicine, LSU Health New Orleans, New Orleans, LA.
authors.forename: Bennet
authors.initials: B
authors.lastname: deBoisblanc
mesh_terms: D000328:Adult; D000777:Anesthetics / Q000008:administration & dosage; D001794:Blood Pressure / Q000187:drug effects / Q000502:physiology; D002585:Cesarean Section; D019468:Disease Management; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality* / Q000503:physiopathology / Q000628:therapy*; D010879:Piperazines / Q000494:pharmacology / Q000627:therapeutic use; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000401:mortality* / Q000503:physiopathology / Q000628:therapy*; D011256:Pregnancy Outcome; D011453:Prostaglandins / Q000494:pharmacology / Q000627:therapeutic use; D011687:Purines / Q000494:pharmacology / Q000627:therapeutic use; D012189:Retrospective Studies; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000494:pharmacology / Q000627:therapeutic use; D015996:Survival Rate; D014665:Vasodilator Agents / Q000494:pharmacology / Q000627:therapeutic use
chemical_list: D000777:Anesthetics; D010879:Piperazines; D011453:Prostaglandins; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate