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Improved survival in limited scleroderma-related pulmonary artery hypertension

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Abstract

Reportedly, patients with scleroderma-related pulmonary hypertension (SSc-PAH) respond poorly to new vasoactive drugs (NVD). Forty-nine SSc-PAH patients underwent right heart catheterization (RHC) and, according to NVD availability, divided as follows: Group 1 (n = 23, from 1999 to 2004, poor availability), and Group 2 (n = 26, from 2005 to 2010, good availability). Before diagnostic RHC, NVD had been given to 30 % of the patients in Group 1, and 58 % of those in Group 2 (p = 0.049). At diagnosis, patients in Group 1 had greater heart dilatation (p < 0.01), higher mean pulmonary artery pressure (p < 0.05), lower pulmonary artery capacitance (p < 0.05), and lower carbon monoxide lung diffusing capacity (DLco, p < 0.05) than those in Group 2. At a median follow-up time of 15.5 months, DLco further decreased in Group 1 (p < 0.05), whereas cardiac index increased in Group 2 (p < 0.05). At 36 months of follow-up, 72.4 % of the patients in Group 2 were still alive as opposed to 30.4 % in Group 1 (p = 0.02). In multivariate analysis, DLco and mixed venous oxygen saturation (SvO2) were independent predictors of survival. A value of DLco <7.2 mL/mmHg/min was associated with a hazard ratio (HR) of 5.3 (p < 0.001); for SvO2 <63.8 %, the HR was 3.7 (p < 0.01).NVD have beneficial effects in patients with SSc-PAH. Both DLco and SvO2 are predictors of survival and may assist in planning treatment.

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Abbreviations

MRC:

Medical Research Council dyspnea scale

DLco:

Carbon monoxide diffusing capacity

SvO2 :

Mixed venous blood saturation

FIO2-RHC:

Oxygen inhaled fraction at right heart catheterization

Pv50 :

Mixed venous partial pressure 50 % saturated

O2ER:

Oxygen extraction ratio

PFT:

Pulmonary function tests

eGFR:

Estimated glomerular filtration rate

mSAP:

Mean systolic arterial pressure

mRAP:

Mean right atrium pressure

mPAP:

Mean pulmonary artery pressure

PP:

Pressure pulse

CO:

Cardiac output

CI:

Cardiac index

SVI:

Stroke volume index

PVR:

Pulmonary vascular resistance

SV/PP:

Pulmonary capacitance

NVD:

New vasoactive drugs for PAH

ERA:

Endothelin receptor antagonist

PDE5-I:

Phosphodiesterase type-5 inhibitor

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Acknowledgments

The authors thank Dr Michele Emdin for his thoughtful review and advice, and Dr. Edoardo Airò for the retrieval of the data.

Conflict of interest

None.

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Authors and Affiliations

  1. Fondazione Regione Toscana “G. Monasterio”, Via G. Moruzzi 1, Ghezzano, 56124, Pisa, Italy

    Carlo Marini, Bruno Formichi, Carolina Bauleo, Claudio Michelassi, Renato Prediletto, Giosuè Catapano, Simonetta Monti, Francesca Mannucci, Luigi Emilio Pastormerlo & Alberto Giannoni

  2. Istituto di Fisiologia Clinica del CNR, Via G. Moruzzi 1, Ghezzano, 56124, Pisa, Italy

    Bruno Formichi, Claudio Michelassi, Renato Prediletto & Simonetta Monti

  3. Dipartimento Cardio-Toracico e Vascolare, Università degli Studi, Via Paradisa 2, Pisa, Italy

    Carlo Marini, Roberta Pancani & Carlo Giuntini

  4. Dipartimento di Medicina Interna, Università degli Studi, Via Roma 67, 56126, Pisa, Italy

    Antonio Tavoni & Anna D’Ascanio

  5. Dipartimento di Area Critica Medico Chirurgica, Università degli Studi, VialeMorganti 85, 50134, Florence, Italy

    Massimo Miniati

Authors
  1. Carlo Marini
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Correspondence to Carlo Marini.

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Marini, C., Formichi, B., Bauleo, C. et al. Improved survival in limited scleroderma-related pulmonary artery hypertension. Intern Emerg Med 9, 385–396 (2014). https://doi.org/10.1007/s11739-013-0900-7

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  • DOI: https://doi.org/10.1007/s11739-013-0900-7

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