Thrombocytopenia independently predicts death in idiopathic PAH

Rachel J. Le, Carolyn M. Larsen, Eric R. Fenstad, Robert B. McCully, Robert Frantz, Michael D. McGoon, Garvan M Kane

Research output: Contribution to journalArticle

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Abstract

Background: Pulmonary arterial hypertension (PAH) is a progressive vascular disorder with a high mortality. Clinical experience and small case series suggest thrombocytopenia may be frequent in this population and associated with a poor prognosis. We sought to estimate the prevalence of thrombocytopenia in patients with PAH and characterize its association with disease characteristics and patient outcome. Methods: Single center cohort study of 714 incident adult patients with Group 1 PH who were evaluated for baseline platelet count at the time of diagnosis. Pts were stratified into three groups: normal platelet count (>150 × 109/L), Grade 1 thrombocytopenia (75–149 × 109/L) and Grade 2–4 thrombocytopenia (<75 × 109/L). Results: The median platelet count was 209 × 109/L (IQR 163, 264). There were 572 (80%) pts without thrombocytopenia, 107 (15%) with Grade 1 and 35 (5%) with Grade 2–4 thrombocytopenia. The median pt age was 55 years (IQR 44–65) with no difference between platelet groups (p = 0.85). Men were more likely to have thrombocytopenia (62, 34%) than women (80, 15%, p < 0.0001). Thrombocytopenia was frequent with portopulmonary PAH (84%) as opposed to idiopathic PAH (iPAH; 14%) or connective tissue disease associated PAH (12%). Platelet counts were not associated with functional class symptoms, the degree of right ventricular enlargement or dysfunction or tricuspid regurgitation by echocardiography. Invasive hemodynamics of right atrial pressure, mean pulmonary artery pressure and pulmonary vascular resistance were also similar between platelet groups. Thrombocytopenia was associated with higher mortality in iPAH patients (age- and sex-adjusted 5 year mortality [HR 1.95 (1.20, 3.08) p = 0.008] but not in other etiology groups. In a multivariate model of iPAH patients (adjusted for age, sex, DLCO, PVR, creatinine and 6MW distance) thrombocytopenia was most predictive of 5-year mortality [HR 1.68 (1.32, 2.12), p < 0.0001]. Conclusion: Thrombocytopenia in the context of iPAH portends a poor prognosis and is a simple independent factor to consider in judging severity of disease.

Original languageEnglish (US)
Pages (from-to)34-38
Number of pages5
JournalHeart and Lung
Volume48
Issue number1
DOIs
StatePublished - Jan 1 2019

Fingerprint

Thrombocytopenia
Platelet Count
Pulmonary Hypertension
Mortality
Blood Platelets
Familial Primary Pulmonary Hypertension
Tricuspid Valve Insufficiency
Connective Tissue Diseases
Atrial Pressure
Vascular Resistance
Pulmonary Artery
Blood Vessels
Echocardiography
Creatinine
Cohort Studies
Hemodynamics
Pressure

Keywords

  • Mortality
  • Prediction
  • Pulmonary hypertension
  • Thrombocytopenia

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Thrombocytopenia independently predicts death in idiopathic PAH. / Le, Rachel J.; Larsen, Carolyn M.; Fenstad, Eric R.; McCully, Robert B.; Frantz, Robert; McGoon, Michael D.; Kane, Garvan M.

In: Heart and Lung, Vol. 48, No. 1, 01.01.2019, p. 34-38.

Research output: Contribution to journalArticle

Le, RJ, Larsen, CM, Fenstad, ER, McCully, RB, Frantz, R, McGoon, MD & Kane, GM 2019, 'Thrombocytopenia independently predicts death in idiopathic PAH', Heart and Lung, vol. 48, no. 1, pp. 34-38. https://doi.org/10.1016/j.hrtlng.2018.08.009
Le, Rachel J. ; Larsen, Carolyn M. ; Fenstad, Eric R. ; McCully, Robert B. ; Frantz, Robert ; McGoon, Michael D. ; Kane, Garvan M. / Thrombocytopenia independently predicts death in idiopathic PAH. In: Heart and Lung. 2019 ; Vol. 48, No. 1. pp. 34-38.
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abstract = "Background: Pulmonary arterial hypertension (PAH) is a progressive vascular disorder with a high mortality. Clinical experience and small case series suggest thrombocytopenia may be frequent in this population and associated with a poor prognosis. We sought to estimate the prevalence of thrombocytopenia in patients with PAH and characterize its association with disease characteristics and patient outcome. Methods: Single center cohort study of 714 incident adult patients with Group 1 PH who were evaluated for baseline platelet count at the time of diagnosis. Pts were stratified into three groups: normal platelet count (>150 × 109/L), Grade 1 thrombocytopenia (75–149 × 109/L) and Grade 2–4 thrombocytopenia (<75 × 109/L). Results: The median platelet count was 209 × 109/L (IQR 163, 264). There were 572 (80{\%}) pts without thrombocytopenia, 107 (15{\%}) with Grade 1 and 35 (5{\%}) with Grade 2–4 thrombocytopenia. The median pt age was 55 years (IQR 44–65) with no difference between platelet groups (p = 0.85). Men were more likely to have thrombocytopenia (62, 34{\%}) than women (80, 15{\%}, p < 0.0001). Thrombocytopenia was frequent with portopulmonary PAH (84{\%}) as opposed to idiopathic PAH (iPAH; 14{\%}) or connective tissue disease associated PAH (12{\%}). Platelet counts were not associated with functional class symptoms, the degree of right ventricular enlargement or dysfunction or tricuspid regurgitation by echocardiography. Invasive hemodynamics of right atrial pressure, mean pulmonary artery pressure and pulmonary vascular resistance were also similar between platelet groups. Thrombocytopenia was associated with higher mortality in iPAH patients (age- and sex-adjusted 5 year mortality [HR 1.95 (1.20, 3.08) p = 0.008] but not in other etiology groups. In a multivariate model of iPAH patients (adjusted for age, sex, DLCO, PVR, creatinine and 6MW distance) thrombocytopenia was most predictive of 5-year mortality [HR 1.68 (1.32, 2.12), p < 0.0001]. Conclusion: Thrombocytopenia in the context of iPAH portends a poor prognosis and is a simple independent factor to consider in judging severity of disease.",
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AU - Le, Rachel J.

AU - Larsen, Carolyn M.

AU - Fenstad, Eric R.

AU - McCully, Robert B.

AU - Frantz, Robert

AU - McGoon, Michael D.

AU - Kane, Garvan M

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N2 - Background: Pulmonary arterial hypertension (PAH) is a progressive vascular disorder with a high mortality. Clinical experience and small case series suggest thrombocytopenia may be frequent in this population and associated with a poor prognosis. We sought to estimate the prevalence of thrombocytopenia in patients with PAH and characterize its association with disease characteristics and patient outcome. Methods: Single center cohort study of 714 incident adult patients with Group 1 PH who were evaluated for baseline platelet count at the time of diagnosis. Pts were stratified into three groups: normal platelet count (>150 × 109/L), Grade 1 thrombocytopenia (75–149 × 109/L) and Grade 2–4 thrombocytopenia (<75 × 109/L). Results: The median platelet count was 209 × 109/L (IQR 163, 264). There were 572 (80%) pts without thrombocytopenia, 107 (15%) with Grade 1 and 35 (5%) with Grade 2–4 thrombocytopenia. The median pt age was 55 years (IQR 44–65) with no difference between platelet groups (p = 0.85). Men were more likely to have thrombocytopenia (62, 34%) than women (80, 15%, p < 0.0001). Thrombocytopenia was frequent with portopulmonary PAH (84%) as opposed to idiopathic PAH (iPAH; 14%) or connective tissue disease associated PAH (12%). Platelet counts were not associated with functional class symptoms, the degree of right ventricular enlargement or dysfunction or tricuspid regurgitation by echocardiography. Invasive hemodynamics of right atrial pressure, mean pulmonary artery pressure and pulmonary vascular resistance were also similar between platelet groups. Thrombocytopenia was associated with higher mortality in iPAH patients (age- and sex-adjusted 5 year mortality [HR 1.95 (1.20, 3.08) p = 0.008] but not in other etiology groups. In a multivariate model of iPAH patients (adjusted for age, sex, DLCO, PVR, creatinine and 6MW distance) thrombocytopenia was most predictive of 5-year mortality [HR 1.68 (1.32, 2.12), p < 0.0001]. Conclusion: Thrombocytopenia in the context of iPAH portends a poor prognosis and is a simple independent factor to consider in judging severity of disease.

AB - Background: Pulmonary arterial hypertension (PAH) is a progressive vascular disorder with a high mortality. Clinical experience and small case series suggest thrombocytopenia may be frequent in this population and associated with a poor prognosis. We sought to estimate the prevalence of thrombocytopenia in patients with PAH and characterize its association with disease characteristics and patient outcome. Methods: Single center cohort study of 714 incident adult patients with Group 1 PH who were evaluated for baseline platelet count at the time of diagnosis. Pts were stratified into three groups: normal platelet count (>150 × 109/L), Grade 1 thrombocytopenia (75–149 × 109/L) and Grade 2–4 thrombocytopenia (<75 × 109/L). Results: The median platelet count was 209 × 109/L (IQR 163, 264). There were 572 (80%) pts without thrombocytopenia, 107 (15%) with Grade 1 and 35 (5%) with Grade 2–4 thrombocytopenia. The median pt age was 55 years (IQR 44–65) with no difference between platelet groups (p = 0.85). Men were more likely to have thrombocytopenia (62, 34%) than women (80, 15%, p < 0.0001). Thrombocytopenia was frequent with portopulmonary PAH (84%) as opposed to idiopathic PAH (iPAH; 14%) or connective tissue disease associated PAH (12%). Platelet counts were not associated with functional class symptoms, the degree of right ventricular enlargement or dysfunction or tricuspid regurgitation by echocardiography. Invasive hemodynamics of right atrial pressure, mean pulmonary artery pressure and pulmonary vascular resistance were also similar between platelet groups. Thrombocytopenia was associated with higher mortality in iPAH patients (age- and sex-adjusted 5 year mortality [HR 1.95 (1.20, 3.08) p = 0.008] but not in other etiology groups. In a multivariate model of iPAH patients (adjusted for age, sex, DLCO, PVR, creatinine and 6MW distance) thrombocytopenia was most predictive of 5-year mortality [HR 1.68 (1.32, 2.12), p < 0.0001]. Conclusion: Thrombocytopenia in the context of iPAH portends a poor prognosis and is a simple independent factor to consider in judging severity of disease.

KW - Mortality

KW - Prediction

KW - Pulmonary hypertension

KW - Thrombocytopenia

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