title: Hemodynamics of patients developing pulmonary arterial hypertension after shunt closure.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) after shunt closure is associated with a poor prognosis. The aim of this study was to assess retrospectively the hemodynamics of patients developing PAH after shunt closure.
  
  
  METHODS
  Hemodynamic data obtained by right heart catheterization (RHC) performed at baseline and after shunt closure were analyzed.
  
  
  RESULTS
  Twenty-two patients, 13 with atrial septal defect (ASD), 6 with ventricular septal defect (VSD), 1 with patent ductus arteriosus, 1 with both ASD and VSD, and 1 with complete atrio-ventricular canal have been considered. The mean age at closure was 25.3±20.1 years (range of 3 months to 56.7 years), and the mean age at PAH diagnosis was 37.0±20.8 years (range of 5 to 61.2 years). The time delay between shunt closure and PAH diagnosis was 140.2±100.2 months. At baseline RHC, hemodynamic data were as follows: pulmonary vascular resistance (PVR) of 8.6±2.6 Wood units, PVR index (PVRi) of 10.1±2.7 Wood units∗m(2), mean pulmonary arterial pressure of 43.7±9.7 mmHg, PVR to systemic vascular resistance ratio (PVR/SVR) of 0.70±0.23, and Qp/Qs of 1.6±0.4. In particular, 18/22 (81%) had PVR≥5 Wood units, 21/22 (95%) PVRi≥6 Wood units∗m(2), 21/22 (95%) PVR/SVR≥0.33, and 11/22 (50%) Qp/Qs≤1.5. During the follow-up, 5/22 (22%) patients died and one patient underwent successful double lung transplantation.
  
  
  CONCLUSIONS
  High baseline values of PVR (≥5 Wood units), PVRi (≥6 Wood units∗m(2)) and PVR/SVR (≥0.33) are common findings in patients who develop PAH late after shunt closure. Large prospective clinical trials are needed to establish the safe limits for shunt closure.
authors.affiliation: Department of Cardiology, Second University of Naples, Monaldi Hospital, Naples, Italy. Electronic address: mic.dalto@tin.it.
authors.forename: Michele
authors.initials: M
authors.lastname: D'Alto
authors.forename: Emanuele
authors.initials: E
authors.lastname: Romeo
authors.forename: Paola
authors.initials: P
authors.lastname: Argiento
authors.forename: Anna
authors.initials: A
authors.lastname: Correra
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Santoro
authors.forename: Gianpiero
authors.initials: G
authors.lastname: Gaio
authors.forename: Berardo
authors.initials: B
authors.lastname: Sarubbi
authors.forename: Raffaele
authors.initials: R
authors.lastname: Calabrò
authors.forename: Maria Giovanna
authors.initials: MG
authors.lastname: Russo
mesh_terms: D000293:Adolescent; D000328:Adult; D006328:Cardiac Catheterization / Q000009:adverse effects*; D002648:Child; D002675:Child, Preschool; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000503:physiopathology* / Q000601:surgery*; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology*; D007223:Infant; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology*; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: Congenital heart diseases; Hemodynamics; Pulmonary hypertension