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Survival in pulmonary hypertension in Spain: insights from the Spanish registry

Pilar Escribano-Subias, Isabel Blanco, Manuel López-Meseguer, Carmen Jimenez Lopez-Guarch, Antonio Roman, Pilar Morales, María Jesús Castillo-Palma, Javier Segovia, Miguel A. Gómez-Sanchez, Joan Albert Barberà on behalf of the REHAP investigators
European Respiratory Journal 2012 40: 596-603; DOI: 10.1183/09031936.00101211
Pilar Escribano-Subias
*Cardiology Dept, Hospital Universitario 12 de Octubre-REDINSCOR and
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  • For correspondence: pilar.escribano@telefonica.net
Isabel Blanco
#Pneumology Dept, Hospital Clínic-CIBERES
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Manuel López-Meseguer
¶Pneumology Dept, Hospital Universitario Vall d'Hebrón-CIBERES, Barcelona
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Carmen Jimenez Lopez-Guarch
*Cardiology Dept, Hospital Universitario 12 de Octubre-REDINSCOR and
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Antonio Roman
¶Pneumology Dept, Hospital Universitario Vall d'Hebrón-CIBERES, Barcelona
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Pilar Morales
+Pneumology Dept, Hospital Universitario La Fe, Valencia
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María Jesús Castillo-Palma
§Internal Medicine Dept, Hospital Universitario Virgen del Rocío, Seville, Spain
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Javier Segovia
fCardiology Dept, Hospital Universitario Puerta de Hierro, Madrid
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Miguel A. Gómez-Sanchez
*Cardiology Dept, Hospital Universitario 12 de Octubre-REDINSCOR and
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Joan Albert Barberà
#Pneumology Dept, Hospital Clínic-CIBERES
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  • Figure 1–
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    Figure 1–

    Kaplan–Meier estimates of 5-yr survival from time of diagnosis in different pulmonary hypertension subtypes. IPAH: idiopathic pulmonary arterial hypertension; CTD: connective tissue disease; CTEPH: chronic thromboembolic pulmonary hypertension; PoPH: portopulmonary hypertension; PVOD: pulmonary veno-occlusive disease. The p-value for the overall comparison is <0.001. #: compared with IPAH.

  • Figure 2–
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    Figure 2–

    a) Observed and estimated survival (National Institutes of Health (NIH) and Pulmonary Hypertension Connection (PHC) equations) of idiopathic pulmonary arterial hypertension (IPAH) nonresponders in the Spanish Registry of Pulmonary Arterial Hypertension (REHAP) registry. b) Observed and estimated survival (French equations) of IPAH cases diagnosed <48 months before June 30, 2008 in the REHAP registry.

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  • Table 1– Clinical and haemodynamic data at the time of diagnosis of pulmonary hypertension in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH)
    PAHCTEPH
    Subjects n866162
    Age yrs45±1761±15*
    Males %2940*
    Time from symptoms to diagnosis yrs3.5±6.12.7±4.3*
    WHO FC %
     I–II3123
     III5868
     IV119
    MeanPpa mmHg54±1647±13*
    Cardiac index L·min−1·m−22.6±0.92.3±0.5*
    PVR Wood units12±610±5*
    Pra mmHg9±58±6
    6MWD m363±120317±112*
    • Data are presented as mean±sd, unless otherwise stated. WHO: World Health Organization; FC: functional class; Ppa: pulmonary artery pressure; PVR: pulmonary vascular resistance; Pra: right atrial pressure; 6MWD: 6-min walk distance. *: p<0.05 compared with PAH.

  • Table 2– 6-min walk distance (6MWD) and pulmonary haemodynamics in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) according to functional class (FC)
    PAH#ANOVA p-valueCTEPH¶ANOVA p-value
    WHO FCI–IIIIIIVI–IIIIIIV
    6MWD m442±97343±111*255±98*<0.001379±111309±10*210±82*<0.001
    PVR Wood units9±513±6*13±7*<0.0019±510±512±60.083
    Pra mmHg8±59±511±6*0.0047±58±513±7*0.001
    • Data are presented as mean±sd, unless otherwise stated. WHO: World Health Organization; PVR: pulmonary vascular resistance; Pra: right atrial pressure. #: n=866; ¶: n=162. *: p<0.05 for differences between FC I–II versus FC III and FC IV groups for both aetiologies.

  • Table 3– Clinical and haemodynamic data at the time of diagnosis in pulmonary arterial hypertension (PAH) subtypes and chronic thromboembolic pulmonary hypertension (CTEPH)
    PAHCTEPHANOVA p-value
    IPAHCTDCHDHIVPoPHTOSPVOD
    Subjects n (%)314 (30)157 (15)167 (16)54 (5)61 (6)33 (3.2)15 (1.5)162 (16)
    Age yrs46±1854±15 *36±17*39±5*52±10*39±13*51±1561±15*<0.001
    Males %2710*2742*49312240*<0.001
    6MWD m382±117309±115*365±101416±126396±109372±139314±127317±112*<0.001
    WHO FC %
     I–II2921384053132223<0.001
     III5964544046724468
     IV1115819216339
    Haemodynamics
     Mean Ppa mmHg55±1547±13*68±19*52±1149±13*67±17*55±1147±13*<0.001
     Pra mmHg8±59±6*9±68±68±58±59±48±50.577
     Cardiac index L·min−1·m−22.4±0.72.6±0.93.0±0.9*2.4±0.63.2±0.9*2.2±0.52.2±0.52.3±0.5<0.001
     PVR Wood units12±611±6*13±712±67±3*17±8*13±610±5*<0.001
     Sv,O2 %62±959±1166±10*60±1069±7*59±857±1761±10<0.001
    • Data are presented as mean±sd, unless otherwise stated. IPAH: idiopathic PAH; CTD: connective tissue disease; CHD: congenital heart disease; PoPH: portopulmonary hypertension; TOS: toxic oil syndrome; PVOD: veno-occlusive disease; 6MWD: 6-min walk distance; WHO: World Health Organization; FC: functional class; Ppa: pulmonary artery pressure; Pra: right atrial pressure; PVR: pulmonary vascular resistance; Sv,O2: mixed-venous oxygen saturation. *: p<0.05 compared with IPAH.

  • Table 4– Survival rates in the Spanish Registry of Pulmonary Arterial Hypertension (REHAP) registry
    Subjects n1 yr3 yrs5 yrs
    All patients1028877565
    Diagnosed before July 2007861877565
    Diagnosed after July 20071678874
    CTEPH162937565
    IPAH314897768
    • Data are presented as %, unless otherwise stated. CTEPH: chronic thromboembolic pulmonary hypertension; IPAH: idiopathic pulmonary arterial hypertension.

  • Table 5– Multivariate predictors of death in Cox's proportional hazards analysis
    All patientsPatients with IPAH
    HR (95% CI)p-valueHR (95% CI)p-value
    Male sex1.38 (1.03–1.83)0.032.25 ( 1.31–3.72)0.002
    WHO FC III–IV1.32 (0.95–1.84)0.1342.39 (1.64–4.92)0.018
    Pra 5 mmHg1.30 (1.15–1.47)<0.0011.46 (1.11–1.91)0.007
    Cardiac index 1 L·min−1·m−2 0.61 (0.51–0.75)<0.0010.68 (0.44–1.05)0.079
    Subtype of PH0.010
     IPAHReference
     CTD1.78 (0.19–2.64)0.005
     CHD0.86 (0.51–1.46)0.583
     CTEPH1.08 (0.71–1.66)0.720
     HIV1.05 (0.57–1.94)0.883
     PoPH3.70 (2.28–6.03)<0.001
     TOS0.69 (0.37–1.28)0.238
     PVOD3.11 (1.25–7.74)0.015
     ≥2 aetiologies1.21 (0.44–2.80)0.710
     Others1.49 (0.79–2.81)0.213
    • IPAH: idiopathic pulmonary arterial hypertension; HR: hazard ratio; WHO: World Health Organization; FC: functional class; Pra: right atrial pressure; PH: pulmonary hypertension; CTD: connective tissue disease; CHD: congenital heart disease; CTEPH: chronic thromboembolic pulmonary hypertension; PoPH: portopulmonary hypertension; TOS: toxic oil syndrome; PVOD: pulmonary veno-occlusive disease.

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Survival in pulmonary hypertension in Spain: insights from the Spanish registry
Pilar Escribano-Subias, Isabel Blanco, Manuel López-Meseguer, Carmen Jimenez Lopez-Guarch, Antonio Roman, Pilar Morales, María Jesús Castillo-Palma, Javier Segovia, Miguel A. Gómez-Sanchez, Joan Albert Barberà
European Respiratory Journal Sep 2012, 40 (3) 596-603; DOI: 10.1183/09031936.00101211

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Survival in pulmonary hypertension in Spain: insights from the Spanish registry
Pilar Escribano-Subias, Isabel Blanco, Manuel López-Meseguer, Carmen Jimenez Lopez-Guarch, Antonio Roman, Pilar Morales, María Jesús Castillo-Palma, Javier Segovia, Miguel A. Gómez-Sanchez, Joan Albert Barberà
European Respiratory Journal Sep 2012, 40 (3) 596-603; DOI: 10.1183/09031936.00101211
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