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LetterCorrespondence

Right Ventricular Function in Systemic Sclerosis-associated Pulmonary Arterial Hypertension

MARTIN KOESTENBERGER and WILLIAM RAVEKES
The Journal of Rheumatology January 2013, 40 (1) 90; DOI: https://doi.org/10.3899/jrheum.120966
MARTIN KOESTENBERGER
Division of Pediatric Cardiology, Department of Pediatrics, Medical University Graz, Graz, Austria;
PhD, MD
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  • For correspondence: Martin.Koestenberger@medunigraz.at koestenbergerm@gmx.at
WILLIAM RAVEKES
MD
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To the Editor:

We read with interest the article “Tricuspid annular plane systolic excursion is a robust outcome measure in systemic sclerosis-associated pulmonary arterial hypertension” by Mathai, et al1. This excellent article describes the current need for a more detailed investigation of the right ventricle (RV). The authors clearly state that there is a need for a detailed evaluation of RV function measurements, e.g., the tricuspid annular plane systolic excursion (TAPSE), in a population with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH)1.

Forfia, et al2 have also described the excellent sensitivity, reproducibility, and clinical utility of the TAPSE in various forms of PAH. For the convenience of readers and especially for centers performing detailed echocardiographic investigations of the RV, we add that our group has published normal TAPSE values for pediatric and adolescent patients3. In addition, we have described decreased TAPSE values for pediatric patients with PAH secondary to congenital heart diseases (PAH-CHD)4. We thank the authors for addressing the need for careful and systematic evaluation of the RV, especially in patients with different forms of PAH.

RV function should be carefully investigated in patients with systemic diseases known to influence the RV function and diseases that can lead to secondary PAH, including many rheumatologic disorders. RV function should also be investigated in patients with CHD and primary PAH. We hope that with more available normal reference values for this population that quantification of RV function in PAH will become easily available to all sonographers.

REFERENCES

  1. 1.↵
    1. Mathai SC,
    2. Sibley CT,
    3. Forfia PR,
    4. Mudd JO,
    5. Fisher MR,
    6. Tedford RJ,
    7. et al.
    Tricuspid annular plane systolic excursion is a robust outcome measure in systemic sclerosis-associated pulmonary arterial hypertension. J Rheumatol 2011;38:2410–8.
    OpenUrlAbstract/FREE Full Text
  2. 2.↵
    1. Forfia PR,
    2. Fisher MR,
    3. Mathai SC,
    4. Housten-Harris T,
    5. Hemnes AR,
    6. Borlaug BA,
    7. et al.
    Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 2006;174:1034–41.
    OpenUrlCrossRefPubMed
  3. 3.↵
    1. Koestenberger M,
    2. Ravekes W,
    3. Everett AD,
    4. Stueger HP,
    5. Heinzl B,
    6. Gamillscheg A,
    7. et al.
    Right ventricular function in infants, children and adolescents: Reference values of the tricuspid annular plane systolic excursion (TAPSE) in 640 healthy patients and calculation of z score values. J Am Soc Echocardiogr 2009;22:715–9.
    OpenUrlCrossRefPubMed
  4. 4.↵
    1. Koestenberger M,
    2. Nagel B,
    3. Avian A,
    4. Ravekes W,
    5. Sorantin E,
    6. Cvirn G,
    7. et al.
    Systolic right ventricular function in children and young adults with pulmonary artery hypertension secondary to congenital heart disease and tetralogy of Fallot: Tricuspid annular plane systolic excursion (TAPSE) and magnetic resonance imaging data. Congenit Heart Dis 2012;7:250–8.
    OpenUrlCrossRefPubMed
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1 Jan 2013
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Right Ventricular Function in Systemic Sclerosis-associated Pulmonary Arterial Hypertension
MARTIN KOESTENBERGER, WILLIAM RAVEKES
The Journal of Rheumatology Jan 2013, 40 (1) 90; DOI: 10.3899/jrheum.120966

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Right Ventricular Function in Systemic Sclerosis-associated Pulmonary Arterial Hypertension
MARTIN KOESTENBERGER, WILLIAM RAVEKES
The Journal of Rheumatology Jan 2013, 40 (1) 90; DOI: 10.3899/jrheum.120966
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