Correlation of Acute Prostacyclin Response in Primary (Unexplained) Pulmonary Hypertension with Efficacy of Treatment with Calcium Channel Blockers and Survival

Groves, Bertron Μ.; Badesch, David B.; Turkevich, Darya; Ditchey, Roy V.; Donnellan, Kathleen; Wynne, Kristine; Robertson, Alastair D.; Long, Walker; Voelkel, Norbert F.; Reeves, John Τ.

doi:10.1007/978-1-4615-2397-0_23

Cited by 17 publications

(24 citation statements)

References 18 publications

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“…However, it is interesting to note that overall, 15 of 67 (22%) adult patients with idiopathic or familial PAH demonstrated vasoreactivity, even though they presented with severe elevations of mean PAP and pulmonary vascular resistance. This is comparable to the proportion of idiopathic PAH patients reported previously who meet the current consensus definition of vasoreactivity 6,22,32 (Data Supplement, Part D). Nevertheless, it is possible that vasoreactivity is present in individuals with BMPR2 nonsynonymous variants when they are identified earlier in the course of their disease, particularly if BMPR2 nonsynonymous variants induce proliferation of pulmonary vascular smooth muscle cells followed by secondary activation and injury of pulmonary artery endothelial cells that produce obstruction of pulmonary arteries.…”

Section: Discussionsupporting

confidence: 85%

“…Vasoreactivity tests were performed with vasodilators titrated to doses proven to identify PAH patients with marked vasoreactivity. [22][23][24]32,33 Although multiple drugs were used, there is general consensus that vasoreactivity is independent of the vasodilator. 8,32 …”

Section: Discussionmentioning

confidence: 99%

“…The dose of epoprostenol, adenosine, nifedipine, nitroprusside, prostaglandin E2, or phentolamine was increased until either an intolerable side effect occurred or vasoreactivity was observed. 22 Nitric oxide was inhaled, and the hemodynamic response was measured shortly thereafter according to local institutional protocols. 23,24 Data as reported in the medical record and stored in the database from the first complete test of vasoreactivity for each patient were analyzed.…”

Section: Vasoreactivity Testingmentioning

confidence: 99%

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Relationship of BMPR2 Mutations to Vasoreactivity in Pulmonary Arterial Hypertension

et al. 2006

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 99%

Section: Vasoreactivity Testingmentioning

confidence: 99%

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Relationship of BMPR2 Mutations to Vasoreactivity in Pulmonary Arterial Hypertension

et al. 2006

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“…Uncontrolled studies have suggested that extended treatment with calcium channel blockers (CCBs) prolongs survival in rare cases of acutely vasoresponsive patients [1,2].…”

mentioning

confidence: 99%

Iloprost for pulmonary vasodilator testing in idiopathic pulmonary arterial hypertension

Jing

Jiang²,

Zy³

et al. 2009

Eur Respir J

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The aim of this study was to confirm the utility of aerosolised iloprost for identifying long-term responders to calcium channel blockers (CCBs) in patients with idiopathic pulmonary arterial hypertension (IPAH).While undergoing right heart catheterisation, 74 patients with IPAH sequentially received incremental infusions of adenosine and aerosolised iloprost. The effects of the two vasodilators on haemodynamic parameters were recorded. All acute responders identified by aerosolised iloprost were subsequently treated with high doses of a CCB and were re-evaluated after 12 months.Both adenosine and iloprost produced significant decreases in mean pulmonary arterial pressure and pulmonary vascular resistance, and significant increases in cardiac index. Adverse effects were experienced by 35 out of the 74 patients with adenosine, but by only two with iloprost. Aerosolised iloprost identified more acute responders than infused adenosine (10 versus eight, respectively) according to the criteria recommended in recent consensus guidelines. Nine responders identified by iloprost were followed-up after 12 months of high-dose CCB therapy. Five had normal or near-normal haemodynamics and a World Health Organization functional classification of I or II after 12 months.Aerosolised iloprost is an appropriate new agent to identify long-term responders to CCBs in patients with IPAH. It is as effective in this regard as infused adenosine but is better tolerated.

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“…The response of a given patient with severe PH to acute vasodilator treatment is critically important because patients that show a clear haemodynamic response to acute prostacyclin [23,24], adenosine [25] or inhaled NO [26] tend to respond, at least initially, to treatment with calcium antagonists [27].…”

Section: Recommendation For a Classification Based On Associated Condmentioning

confidence: 99%

Severe pulmonary hypertensive diseases: a perspective

Voelkel¹,

Tuder²

1999

Eur Respir J

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Severe pulmonary hypertensive diseases: a perspective. N.F. Voelkel, R.M. Tuder. #ERS Journals Ltd 1999. ABSTRACT: Clinically "severe pulmonary hypertension" is a group of diseases. The nomenclature of pulmonary hypertensive disorders is confusing since terms like "primary", "idiopathic", "unexplained" and "plexogenic" are often used interchangeably.In this Point of view or perspective the authors challenge the validity of the traditional pathohistological classification as it had been provided by HEATH and EDWARDS [1]. A classification that is focused on histological and clinical association is proposed and a recommendation to reorder "Severe pulmonary hypertension" in an association-morphology-function context is provided.The purpose of this "perspective" is to stimulate a discussion which hopefully will lead to a change in the presently used nomenclature and classification of pulmonary hypertensive disorders.

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Correlation of Acute Prostacyclin Response in Primary (Unexplained) Pulmonary Hypertension with Efficacy of Treatment with Calcium Channel Blockers and Survival

Cited by 17 publications

References 18 publications

Relationship of BMPR2 Mutations to Vasoreactivity in Pulmonary Arterial Hypertension

Relationship of BMPR2 Mutations to Vasoreactivity in Pulmonary Arterial Hypertension

Iloprost for pulmonary vasodilator testing in idiopathic pulmonary arterial hypertension

Severe pulmonary hypertensive diseases: a perspective

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