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Article ; Online: Upfront triple therapy for pulmonary arterial hypertension: is three a crowd or critical mass?

McGoon, Michael D

2014 Volume 43, Issue 6, Page(s) 1556–1559

MeSH term(s)	Antihypertensive Agents/administration & dosage ; Female ; Humans ; Hypertension, Pulmonary/drug therapy ; Male
Chemical Substances	Antihypertensive Agents
Language	English
Publishing date	2014-06
Publishing country	England
Document type	Comment ; Editorial
ZDB-ID	639359-7
ISSN	1399-3003 ; 0903-1936
ISSN (online)	1399-3003
ISSN	0903-1936
DOI	10.1183/09031936.00039314
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Article ; Online: 69-year-old woman with ascites, hypoxia, and weight loss.

Prasad, Megha / Wilson, Michael E / McGoon, Michael D

Mayo Clinic proceedings

2013 Volume 88, Issue 12, Page(s) 1475–1479

MeSH term(s)	Aged ; Ascites/etiology ; Cardiac Catheterization ; Diagnosis, Differential ; Echocardiography ; Female ; Humans ; Hypertension, Pulmonary/complications ; Hypertension, Pulmonary/diagnosis ; Hypoxia/etiology ; Oxygen/administration & dosage ; Piperazines/administration & dosage ; Purines/administration & dosage ; Scleroderma, Limited/complications ; Scleroderma, Limited/diagnosis ; Sildenafil Citrate ; Sulfones/administration & dosage ; Vasodilator Agents/administration & dosage ; Weight Loss
Chemical Substances	Piperazines ; Purines ; Sulfones ; Vasodilator Agents ; Sildenafil Citrate (BW9B0ZE037) ; Oxygen (S88TT14065)
Language	English
Publishing date	2013-12
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	124027-4
ISSN	1942-5546 ; 0025-6196
ISSN (online)	1942-5546
ISSN	0025-6196
DOI	10.1016/j.mayocp.2013.04.028
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Article: Diagnostic dilemmas in pulmonary hypertension.

Frantz, Robert P / McGoon, Michael D

Heart failure clinics

2012 Volume 8, Issue 3, Page(s) 331–352

Abstract: Dilemmas persist in the screening, assessment, and follow-up of patients with pulmonary hypertension, relating to issues of whom and how to screen, how to resolve ambiguities in the clinical classification of patients with multiple potential substrates ... ...

Abstract	Dilemmas persist in the screening, assessment, and follow-up of patients with pulmonary hypertension, relating to issues of whom and how to screen, how to resolve ambiguities in the clinical classification of patients with multiple potential substrates of pulmonary vascular disease, how to interpret test results, how to integrate multiple clinical parameters into a global diagnosis, how to use ambiguous test results, how to determine disease severity and prognosis, and how to monitor patients on treatment. This article describes how to incorporate available information into the diagnostic process, and where lack of concrete data should impose caution in patient management.
MeSH term(s)	Biomarkers ; Dyspnea ; Hemodynamics ; Humans ; Hypertension, Pulmonary/diagnosis ; Hypertension, Pulmonary/pathology ; Hypertension, Pulmonary/therapy ; Lung Diseases ; Mass Screening/methods ; Prognosis ; Pulmonary Artery/pathology ; Pulmonary Veins/pathology ; Sleep Apnea Syndromes/pathology ; Ventricular Function, Right
Chemical Substances	Biomarkers
Language	English
Publishing date	2012-07
Publishing country	United States
Document type	Journal Article
ZDB-ID	2212019-1
ISSN	1551-7136
ISSN	1551-7136
DOI	10.1016/j.hfc.2012.04.006
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Article ; Online: Thrombocytopenia independently predicts death in idiopathic PAH.

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

Heart & lung : the journal of critical care

2018 Volume 48, Issue 1, Page(s) 34–38

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 ... ...

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 × 10 Results: The median platelet count was 209 × 10 Conclusion: Thrombocytopenia in the context of iPAH portends a poor prognosis and is a simple independent factor to consider in judging severity of disease.
MeSH term(s)	Adult ; Cause of Death/trends ; Echocardiography ; Familial Primary Pulmonary Hypertension/complications ; Familial Primary Pulmonary Hypertension/mortality ; Familial Primary Pulmonary Hypertension/physiopathology ; Female ; Follow-Up Studies ; Hemodynamics/physiology ; Humans ; Male ; Middle Aged ; Platelet Count ; Prevalence ; Prognosis ; Survival Rate/trends ; Thrombocytopenia/blood ; Thrombocytopenia/complications ; Thrombocytopenia/epidemiology ; United States/epidemiology
Language	English
Publishing date	2018-10-06
Publishing country	United States
Document type	Journal Article
ZDB-ID	193129-5
ISSN	1527-3288 ; 0147-9563
ISSN (online)	1527-3288
ISSN	0147-9563
DOI	10.1016/j.hrtlng.2018.08.009
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Article ; Online: The 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: a practical chronicle of progress.

Lau, Edmund M T / Tamura, Yuichi / McGoon, Michael D / Sitbon, Olivier

The European respiratory journal

2015 Volume 46, Issue 4, Page(s) 879–882

MeSH term(s)	Algorithms ; Decision Support Techniques ; Europe ; Global Health ; Humans ; Hypertension, Pulmonary/diagnosis ; Hypertension, Pulmonary/therapy ; Practice Guidelines as Topic ; Pulmonary Medicine/methods ; Pulmonary Medicine/standards ; Pulmonary Medicine/trends ; Societies, Medical ; World Health Organization
Language	English
Publishing date	2015-10
Publishing country	England
Document type	Editorial
ZDB-ID	639359-7
ISSN	1399-3003 ; 0903-1936
ISSN (online)	1399-3003
ISSN	0903-1936
DOI	10.1183/13993003.01177-2015
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Article ; Online: Pulmonary Hypertension in Hereditary Hemorrhagic Telangiectasia.

Lyle, Melissa A / Fenstad, Eric R / McGoon, Michael D / Frantz, Robert P / Krowka, Michael J / Kane, Garvan C / Swanson, Karen L

Chest

2016 Volume 149, Issue 2, Page(s) 362–371

Abstract: Background: A subset of patients with hereditary hemorrhagic telangiectasia (HHT) develops pulmonary hypertension (PH) by mechanisms including pulmonary arterial hypertension, high flow, and elevated pulmonary arterial wedge pressure (PAWP). We aimed to ...

Abstract	Background: A subset of patients with hereditary hemorrhagic telangiectasia (HHT) develops pulmonary hypertension (PH) by mechanisms including pulmonary arterial hypertension, high flow, and elevated pulmonary arterial wedge pressure (PAWP). We aimed to describe echocardiographic and hemodynamic characteristics of patients with coexisting HHT and PH. Methods: We conducted a single-center cohort study of patients with confirmed HHT who underwent right-sided heart catheterization (RHC) and transthoracic two-dimensional echocardiography for suspected PH between June 1, 2003 and September 1, 2013 at Mayo Clinic Rochester, Minnesota. Results: Of 38 patients with confirmed HHT who underwent RHC and echocardiography, 28 (74%) had a mean pulmonary artery pressure (MPAP) ≥ 25 mm Hg. Of those 28, 12 (43%) had pulmonary arterial hypertension. Two patients had normal PAWP and pulmonary vascular resistance (PVR), with PH secondary to either an atrial septal defect or high cardiac flow. Fourteen patients (50%) had elevated PAWP (≥ 15 mm Hg), nine with evidence of high flow. RHC in all 28 patients demonstrated a MPAP of 41 ± 11 mm Hg, PAWP of 17 ± 10 mm Hg, and PVR of 4.5 ± 4.2 Wood units. Echocardiography demonstrated moderate/severe right ventricular dysfunction in nine patients (32%). The presence of PH trended toward worse survival (P = .06). Conclusions: PH in patients with HHT occurs by different mechanisms, and there is a trend toward worse survival in patients who develop PH despite the mechanism. The equal predilection toward all subtypes of PH illustrates the necessity of RHC to clarify the hemodynamics.
MeSH term(s)	Cardiac Catheterization ; Female ; Folic Acid/analogs & derivatives ; Follow-Up Studies ; Humans ; Hypertension, Pulmonary/diagnosis ; Hypertension, Pulmonary/etiology ; Hypertension, Pulmonary/physiopathology ; Male ; Middle Aged ; Pulmonary Wedge Pressure/physiology ; Retrospective Studies ; Telangiectasia, Hereditary Hemorrhagic/complications ; Vascular Resistance/physiology ; Ventricular Function, Right/physiology ; Vinca Alkaloids
Chemical Substances	EC 0489 ; Vinca Alkaloids ; Folic Acid (935E97BOY8)
Language	English
Publishing date	2016-02
Publishing country	United States
Document type	Journal Article
ZDB-ID	1032552-9
ISSN	1931-3543 ; 0012-3692
ISSN (online)	1931-3543
ISSN	0012-3692
DOI	10.1378/chest.15-0535
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Article ; Online: The importance of patient perspectives in pulmonary hypertension.

McGoon, Michael D / Ferrari, Pisana / Armstrong, Iain / Denis, Migdalia / Howard, Luke S / Lowe, Gabi / Mehta, Sanjay / Murakami, Noriko / Wong, Brad A

The European respiratory journal

2019 Volume 53, Issue 1

Abstract: The assessment of objective measurement of cardiopulmonary status has helped us achieve better clinical outcomes for patients and develop new therapies through to the point of market access; however, patient surveys indicate that more can be done to ... ...

Abstract	The assessment of objective measurement of cardiopulmonary status has helped us achieve better clinical outcomes for patients and develop new therapies through to the point of market access; however, patient surveys indicate that more can be done to improve holistic care and patient engagement. In this multidisciplinary review, we examine how clinical teams can acknowledge and embrace the individual patient's perspective, and thus improve the care for individual patients suffering from pulmonary hypertension by cultivating the importance and relevance of health-related quality of life in direct clinical care. At the individual level, patients should be provided with access to accredited specialist centres which provide a multidisciplinary approach where there is a culture focused on narrative medicine, quality of life, shared decision making and timely access to palliative care, and where there is participation in education. On a larger scale, we call for the development, expansion and promotion of patient associations to support patients and carers, lobby for access to best care and treatments, and provide input into the development of clinical trials and registries, focusing on the patients' perspective.
MeSH term(s)	Health Knowledge, Attitudes, Practice ; Humans ; Hypertension, Pulmonary/psychology ; Hypertension, Pulmonary/therapy ; Palliative Care/methods ; Patient Participation ; Quality of Life/psychology
Language	English
Publishing date	2019-01-24
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	639359-7
ISSN	1399-3003 ; 0903-1936
ISSN (online)	1399-3003
ISSN	0903-1936
DOI	10.1183/13993003.01919-2018
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Article ; Online: Predicting Survival in Patients With Pulmonary Arterial Hypertension: The REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies.

Benza, Raymond L / Gomberg-Maitland, Mardi / Elliott, C Greg / Farber, Harrison W / Foreman, Aimee J / Frost, Adaani E / McGoon, Michael D / Pasta, David J / Selej, Mona / Burger, Charles D / Frantz, Robert P

Chest

2019 Volume 156, Issue 2, Page(s) 323–337

Abstract: Background: Pulmonary arterial hypertension is a progressive, fatal disease. Published treatment guidelines recommend treatment escalation on the basis of regular patient assessment with the goal of achieving or maintaining low-risk status. Various ... ...

Abstract	Background: Pulmonary arterial hypertension is a progressive, fatal disease. Published treatment guidelines recommend treatment escalation on the basis of regular patient assessment with the goal of achieving or maintaining low-risk status. Various strategies are available to determine risk status. This analysis describes an update of the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk calculator (REVEAL 2.0) and compares it with recently published European Society of Cardiology/Respiratory Society guideline-derived risk assessment strategies. Methods: A subpopulation from the US-based registry REVEAL that survived ≥ 1 year postenrollment (baseline for this cohort) was analyzed. For REVEAL 2.0, point values and cutpoints were reassessed, and new variables were evaluated. The Kaplan-Meier method was used to estimate survival at 12 months postbaseline; discrimination was quantified using the c-statistic. Mortality estimates and discrimination were compared between REVEAL 2.0 and Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) and French Pulmonary Hypertension Registry (FPHR) risk assessment strategies. For this comparison, a three-category REVEAL 2.0 score was computed in which patients were classified as low-, intermediate-, or high-risk. Results: REVEAL 2.0 demonstrated similar discrimination as the original calculator in this subpopulation (c-statistic = 0.76 vs 0.74), provided excellent separation of risk among the risk categories, and predicted clinical worsening as well as mortality in patients who were followed ≥ 1 year. The REVEAL 2.0 three-category score had greater discrimination (c-statistic = 0.73) than COMPERA (c-statistic = 0.62) or FPHR (c-statistic = 0.64). Compared with REVEAL 2.0, COMPERA and FPHR both underestimated and overestimated risk. Conclusions: REVEAL 2.0 demonstrates greater risk discrimination than the COMPERA and FPHR risk assessment strategies in patients enrolled in REVEAL. After external validation, the REVEAL 2.0 calculator can assist clinicians and patients in making informed treatment decisions on the basis of individual risk profiles. Trial registry: ClinicalTrials.gov; No. NCT00370214; URL: www.clinicaltrials.gov.
MeSH term(s)	Adult ; Aged ; Cohort Studies ; Female ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Predictive Value of Tests ; Pulmonary Arterial Hypertension/mortality ; Registries ; Risk Assessment ; Survival Rate
Language	English
Publishing date	2019-02-14
Publishing country	United States
Document type	Comparative Study ; Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1032552-9
ISSN	1931-3543 ; 0012-3692
ISSN (online)	1931-3543
ISSN	0012-3692
DOI	10.1016/j.chest.2019.02.004
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Article ; Online: Four- and seven-year outcomes of patients with congenital heart disease-associated pulmonary arterial hypertension (from the REVEAL Registry).

Barst, Robyn J / Ivy, D Dunbar / Foreman, Aimee J / McGoon, Michael D / Rosenzweig, Erika B

The American journal of cardiology

2014 Volume 113, Issue 1, Page(s) 147–155

Abstract: Uncorrected congenital heart disease (CHD) frequently leads to pulmonary arterial hypertension (PAH), the most severe form of which is Eisenmenger syndrome (ES). We compared patients with idiopathic or heritable PAH (IPAH or HPAH; n = 1,626) against ... ...

Abstract	Uncorrected congenital heart disease (CHD) frequently leads to pulmonary arterial hypertension (PAH), the most severe form of which is Eisenmenger syndrome (ES). We compared patients with idiopathic or heritable PAH (IPAH or HPAH; n = 1,626) against those with CHD-associated PAH (n = 353) who were enrolled in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL Registry). Of patients with CHD-associated PAH, 151 had ES. Compared with the IPAH or HPAH cohort, the ES cohort had greater systemic blood flow (2 ± 1 vs 3 ± 2 L/min/m(2), p <0.001), lower mean right atrial pressure (10 ± 6 vs 7 ± 4 mm Hg, p <0.001), higher mean pulmonary artery pressure (53 ± 14 vs 65 ± 17 mm Hg, p <0.001), higher pulmonary vascular resistance index (22 ± 12 vs 32 ± 31 Wood units × m(2), p <0.001), and lower systemic arterial oxygen saturation at rest (92 ± 11% vs 84 ± 13%, p <0.001). At 4 years from enrollment and 7 years from diagnosis, survival rate was similar between IPAH or HPAH and CHD-associated PAH cohorts. For the overall CHD-associated PAH cohort, longer 6-minute walk distance, lower mean right atrial pressure, brain natriuretic peptide level <50 pg/ml, and the presence of acute vasoreactivity were predictors of survival at 4 years from enrollment; younger age and lower mean right atrial pressure were predictors of survival at 7 years from diagnosis. In conclusion, these observations support predicted physiologic differences (e.g., hemodynamics) between patients with IPAH or HPAH and patients with CHD-associated PAH, with or without a systemic-pulmonary shunt. These differences, however, did not translate into significantly improved 4- and 7-year survival rates in patients with ES versus IPAH or HPAH and CHD-associated PAH.
MeSH term(s)	Adult ; Cardiac Catheterization ; Familial Primary Pulmonary Hypertension ; Female ; Follow-Up Studies ; Heart Defects, Congenital/complications ; Heart Defects, Congenital/diagnosis ; Heart Defects, Congenital/epidemiology ; Hemodynamics/physiology ; Humans ; Hypertension, Pulmonary/epidemiology ; Hypertension, Pulmonary/etiology ; Hypertension, Pulmonary/physiopathology ; Incidence ; Male ; Prognosis ; Registries ; Retrospective Studies ; Survival Rate/trends ; Time Factors ; United States/epidemiology ; Young Adult
Language	English
Publishing date	2014-01-01
Publishing country	United States
Document type	Comparative Study ; Journal Article ; Multicenter Study ; Research Support, Non-U.S. Gov't
ZDB-ID	80014-4
ISSN	1879-1913 ; 0002-9149
ISSN (online)	1879-1913
ISSN	0002-9149
DOI	10.1016/j.amjcard.2013.09.032
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Article ; Online: Pulmonary hypertension: diagnosis and management.

McGoon, Michael D / Kane, Garvan C

Mayo Clinic proceedings

2009 Volume 84, Issue 2, Page(s) 191–207

Abstract: Pulmonary arterial hypertension is a progressive, symptomatic, and ultimately fatal disorder for which substantial advances in treatment have been made during the past decade. Effective management requires timely recognition and accurate diagnosis of the ...

Abstract	Pulmonary arterial hypertension is a progressive, symptomatic, and ultimately fatal disorder for which substantial advances in treatment have been made during the past decade. Effective management requires timely recognition and accurate diagnosis of the disorder and appropriate selection among therapeutic alternatives. Despite progress in treatment, obstacles remain that impede the achievement of optimal outcomes. The current article provides an overview of the pathobiologic mechanisms of pulmonary arterial hypertension, including genetic substrates and molecular and cellular mechanisms, and describes the clinical manifestations and classification of pulmonary arterial hypertension. The article also reviews established approaches to evaluation and treatment, with emphasis on the appropriate application of calcium channel blockers, prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase 5 inhibitors. In addition, the authors discuss unresolved issues that may complicate patient management, such as the clinical importance of mild or exercise-related pulmonary arterial hypertension, and they identify avenues by which treatment may advance in the future through the use of combination treatment, outcomes assessment, and exploration of alternative pharmacologic strategies.
MeSH term(s)	Activin Receptors, Type II/genetics ; Algorithms ; Angina, Unstable/etiology ; Antihypertensive Agents/therapeutic use ; Bone Morphogenetic Protein Receptors, Type II/genetics ; Calcium Channel Blockers/therapeutic use ; Drug Therapy, Combination ; Endothelin Receptor Antagonists ; Exercise ; Female ; Humans ; Hypertension, Pulmonary/diagnosis ; Hypertension, Pulmonary/genetics ; Hypertension, Pulmonary/physiopathology ; Hypertension, Pulmonary/therapy ; Insurance Coverage ; Insurance, Health ; Phosphodiesterase Inhibitors/therapeutic use ; Pregnancy ; Severity of Illness Index ; Syncope/etiology
Chemical Substances	Antihypertensive Agents ; Calcium Channel Blockers ; Endothelin Receptor Antagonists ; Phosphodiesterase Inhibitors ; ACVRL1 protein, human (EC 2.7.11.30) ; Activin Receptors, Type II (EC 2.7.11.30) ; BMPR2 protein, human (EC 2.7.11.30) ; Bone Morphogenetic Protein Receptors, Type II (EC 2.7.11.30)
Language	English
Publishing date	2009-02-05
Publishing country	England
Document type	Journal Article
ZDB-ID	124027-4
ISSN	1942-5546 ; 0025-6196
ISSN (online)	1942-5546
ISSN	0025-6196
DOI	10.4065/84.2.191
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