title: The right ventricular response to high afterload: comparison between atrial switch procedure, congenitally corrected transposition of the great arteries, and idiopathic pulmonary arterial hypertension.
abstract: BACKGROUND
  We aimed to define differences between systemic right ventricle (RV) in patients with atrial switch procedure for transposition of the great arteries, and congenitally corrected transposition of the great arteries (ccTGA), which remodels early on in life and the subpulmonary RV in patients with idiopathic pulmonary arterial hypertension (iPAH) which remodels later in adulthood to the effects of progressive pulmonary hypertension.
  
  
  METHODS
  Prospective echocardiographic assessment of consecutive patients with atrial switch procedure, ccTGA, and iPAH attending adult congenital heart program. Right ventricular long axis function by M-mode and tissue Doppler imaging; myocardial performance index; and total isovolumic time (t-IVT), ventricular filling time, and ejection time (ET) were studied and compared with normal left ventricle and RV.
  
  
  RESULTS
  Seventy-eight patients (20 atrial switch, 18 ccTGA, 20 iPAH, and 20 normal) were studied. Right ventricular long axis function was most reduced after atrial switch procedure. Diastolic filling and dysfunction varied across the groups, with atrial switch patients having the lowest myocardial early diastolic (Em) and atrial diastolic (Am) velocities and iPAH patients with the longest t-IVT, shortest filling time and ET, and lowest Em/Am, reflecting predominantly late diastolic filling. Patients with ccTGA had better preserved global systemic RV systolic and diastolic indices.
  
  
  CONCLUSIONS
  The RV develops adaptative mechanisms when faced with increased afterload, behaving more like normal left ventricle. This adaptation is closer when present from birth (ccTGA) without facing subsequent surgical insults. In iPAH, the RV adapts poorly, showing prolonged t-IVT and shortened filling and effective ETs, eventually resulting in lower stroke volume and overall poorer prognosis.
authors: Adult Congenital Heart Disease Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College, London, United Kingdom.
authors: Ju-Le
authors: JL
authors: Tan
authors: Daniele
authors: D
authors: Prati
authors: Michael A
authors: MA
authors: Gatzoulis
authors: Derek
authors: D
authors: Gibson
authors: Michael Y
authors: MY
authors: Henein
authors: Wei
authors: W
authors: Li
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology*; D008297:Male; D011446:Prospective Studies; D014188:Transposition of Great Vessels / Q000503:physiopathology* / Q000601:surgery*; D016276:Ventricular Function