title: Cardiac remodelling amongst adults with various aetiologies of pulmonary arterial hypertension including Eisenmenger syndrome-implications on survival and the role of right ventricular transverse strain.
abstract: OBJECTIVE
  Survival in pulmonary arterial hypertension (PAH) and Eisenmenger syndrome (ES) relates to right ventricular (RV) function. Little is known about differences of ventricular function between ES patients and those suffering from other PAH aetiologies. In this study, we compared global ventricular function assessed by speckle-tracking in adult patients with ES, other PAH aetiologies, or healthy controls; and assessed the relationship between ventricular function and survival.
  
  
  RESULTS
  We performed a prospective cohort study recruiting 83 adult PAH patients (43 ES and 40 other PAH aetiologies patients) and 37 controls between March 2011 and June 2015. Patients with complex congenital heart disease were excluded. Fifty-three patients (63.9%) were in NYHA functional class ≥III at baseline and 60 (72.3%) were on advanced therapies. Mean RV peak longitudinal strain was -16.3 ± 7% in ES, lower compared with healthy controls (P < 0.001) but similar to other PAH aetiologies (P = 0.6). Mean RV peak transverse strain was +26.1 ± 17% in ES, lower than in controls (P < 0.001) but higher than in other PAH aetiologies (P < 0.001). No difference was observed between ES and other PAH in LV circumferential and longitudinal strain. Over a median follow-up of 22.6 months (3.3-32.2), 22 (26.5%) patients died all from cardio-pulmonary causes. ES and RV peak transverse strain were independent predictors of survival. RV peak transverse strain ≤22% identified patients with a 14-fold increased risk of death.
  
  
  CONCLUSIONS
  Right ventricular remodelling differs between adults with ES and other PAH aetiologies. ES and increased RV free wall transverse strain are associated with better survival.
authors.affiliation: Cardiology Department, Hôpital Pasteur, CHU de Nice, Avenue de la Voie Romaine, CS 51069-06001 Nice, France.|Faculté de Médecine, Université de Nice Sophia-Antipolis, Nice, France.|Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.|NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Pamela
authors.initials: P
authors.lastname: Moceri
authors.affiliation: Cardiology Department, Hôpital Pasteur, CHU de Nice, Avenue de la Voie Romaine, CS 51069-06001 Nice, France.
authors.forename: Priscille
authors.initials: P
authors.lastname: Bouvier
authors.affiliation: Cardiology Department, Hôpital Pasteur, CHU de Nice, Avenue de la Voie Romaine, CS 51069-06001 Nice, France.|Faculté de Médecine, Université de Nice Sophia-Antipolis, Nice, France.
authors.forename: Delphine
authors.initials: D
authors.lastname: Baudouy
authors.affiliation: Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.|NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.affiliation: Cardiology Department, Hôpital Pasteur, CHU de Nice, Avenue de la Voie Romaine, CS 51069-06001 Nice, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Cerboni
authors.affiliation: Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.|NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Wort
authors.affiliation: Cardiology Department, Hôpital Pasteur, CHU de Nice, Avenue de la Voie Romaine, CS 51069-06001 Nice, France.
authors.forename: Denis
authors.initials: D
authors.lastname: Doyen
authors.affiliation: Cardiology Department, Hôpital Pasteur, CHU de Nice, Avenue de la Voie Romaine, CS 51069-06001 Nice, France.|Faculté de Médecine, Université de Nice Sophia-Antipolis, Nice, France.
authors.forename: Elie-Dan
authors.initials: ED
authors.lastname: Schouver
authors.affiliation: Cardiology Department, Hôpital Pasteur, CHU de Nice, Avenue de la Voie Romaine, CS 51069-06001 Nice, France.|Faculté de Médecine, Université de Nice Sophia-Antipolis, Nice, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Gibelin
authors.affiliation: Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.|Department of Echocardiography, Royal Brompton Hospital, London, UK.
authors.forename: Roxy
authors.initials: R
authors.lastname: Senior
authors.affiliation: Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.|NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
authors.affiliation: Cardiology Department, Hôpital Pasteur, CHU de Nice, Avenue de la Voie Romaine, CS 51069-06001 Nice, France.|Faculté de Médecine, Université de Nice Sophia-Antipolis, Nice, France.
authors.forename: Emile
authors.initials: E
authors.lastname: Ferrari
authors.affiliation: Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.|NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College School of Medicine, London, UK.|Department of Echocardiography, Royal Brompton Hospital, London, UK.
authors.forename: Wei
authors.initials: W
authors.lastname: Li
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D015150:Echocardiography, Doppler / Q000379:methods*; D004541:Eisenmenger Complex / Q000150:complications* / Q000000981:diagnostic imaging* / Q000401:mortality; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000209:etiology* / Q000401:mortality; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D011446:Prospective Studies; D015996:Survival Rate; D020257:Ventricular Remodeling
keywords: congenital; echocardiography; eisenmenger syndrome; heart defects; hypertension; pulmonary; speckle-tracking imaging