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The Journal of Heart and Lung Transplantation, Vol 33, No 2, February 2014
Cause of death in patients with pulmonary arterial hypertension Harrison W. Farber, MD,a and Dave P. Miller, MSb From the aPulmonary Center, Boston University School of Medicine, Boston, Massachusetts; and the bBiostatistics & Programming, ICON Clinical Research, San Francisco, California.
We thank Drs Tonelli and Dweik for their interest in our study,1 as well as their observations and questions. We are pleased that the depth of their single-center study2 complements the breadth of the 55-site REVEAL analysis. We completely agree that adjudicating deaths as pulmonary arterial hypertension (PAH)-related vs non–PAH-related is a complex task; in the absence of a full chart review and an adjudication committee, it is frequently impossible to determine with confidence whether the categorization is accurate. In addition to the PAH-related deaths, the mortality rate for so-called non–PAH-related deaths vastly exceeds the expected mortality for patients who do not have PAH; this finding questions the meaning and accuracy of the two categories. We therefore applaud Tonelli and Dweik for utilizing a PAH-contributed category, which was much larger than the unrelated category. We presented the REVEAL data as both PAH-related deaths
and all deaths, because this distinction is difficult to make retrospectively and impossible to make at the time a clinical decision is required for a living patient. Determining and understanding the reasons for lack of treatment with systemic prostanoids is extremely important; unfortunately, REVEAL was not designed to capture these data. Finally, classifying deaths as non–PAH-related when they are at least PAHattributable may obscure rather than inform this issue; as such, it is clear that more work is needed to understand treatment decisions, both those that are not made and those not made.
Disclosure statement The authors have no conflicts of interest to disclose.
References 1. Farber HW, Miller DP, Meltzer LA, et al. Treatment of patients with pulmonary arterial hypertension at the time of death or deterioration to function class IV: insights from the REVEAL registry. J Heart Lung Transplant 2013;32:1114-22. 2. Tonelli AR, Arelli V, Minai OA, et al. Causes and circumstances of death in pulmonary arterial hypertension. Am J Respir Crit Care Med 2013;188:365-9.
The Journal of Heart and Lung Transplantation, Vol 33, No 2, February 2014
Cause of death in patients with pulmonary arterial hypertension Harrison W. Farber, MD,a and Dave P. Miller, MSb From the aPulmonary Center, Boston University School of Medicine, Boston, Massachusetts; and the bBiostatistics & Programming, ICON Clinical Research, San Francisco, California.
We thank Drs Tonelli and Dweik for their interest in our study,1 as well as their observations and questions. We are pleased that the depth of their single-center study2 complements the breadth of the 55-site REVEAL analysis. We completely agree that adjudicating deaths as pulmonary arterial hypertension (PAH)-related vs non–PAH-related is a complex task; in the absence of a full chart review and an adjudication committee, it is frequently impossible to determine with confidence whether the categorization is accurate. In addition to the PAH-related deaths, the mortality rate for so-called non–PAH-related deaths vastly exceeds the expected mortality for patients who do not have PAH; this finding questions the meaning and accuracy of the two categories. We therefore applaud Tonelli and Dweik for utilizing a PAH-contributed category, which was much larger than the unrelated category. We presented the REVEAL data as both PAH-related deaths
and all deaths, because this distinction is difficult to make retrospectively and impossible to make at the time a clinical decision is required for a living patient. Determining and understanding the reasons for lack of treatment with systemic prostanoids is extremely important; unfortunately, REVEAL was not designed to capture these data. Finally, classifying deaths as non–PAH-related when they are at least PAHattributable may obscure rather than inform this issue; as such, it is clear that more work is needed to understand treatment decisions, both those that are not made and those not made.
Disclosure statement The authors have no conflicts of interest to disclose.
References 1. Farber HW, Miller DP, Meltzer LA, et al. Treatment of patients with pulmonary arterial hypertension at the time of death or deterioration to function class IV: insights from the REVEAL registry. J Heart Lung Transplant 2013;32:1114-22. 2. Tonelli AR, Arelli V, Minai OA, et al. Causes and circumstances of death in pulmonary arterial hypertension. Am J Respir Crit Care Med 2013;188:365-9.
