ABSTRACT
Pulmonary hypertension (PH) can occur as either a primary or a secondary process,
and in general, its presence increases overall morbidity and mortality. Importantly,
the majority of prior studies have been in the setting of idiopathic pulmonary arterial
hypertension (IPAH); thus the following discussion focuses on IPAH. Because the majority
of available diagnostic strategies lack sensitivity and specificity, the physician
must maintain a high index of suspicion in considering PAH. This article provides
an overview of the available diagnostic studies for PAH with a particular focus on
hemodynamic assessment. Novel approaches to the often delayed diagnosis of PAH are
being studied and are also discussed here.
KEYWORDS
Pulmonary hypertension - hemodynamic - echocardiogram - diagnosis - right heart catheterization
REFERENCES
1
Romberg E.
Ueber sklerose der lungen arterie.
Dtsch Arch Klin Med.
1891;
48
197-206
2
Rich S, Dantzker D R, Ayres S M et al..
Primary pulmonary hypertension: a national prospective study.
Ann Intern Med.
1987;
107
216-223
3
D'Alonzo G E, Barst R J, Ayres S M et al..
Survival in patients with primary pulmonary hypertension: results from a national
prospective registry.
Ann Intern Med.
1991;
115
343-349
4 Fishman A P. Handbook of Physiology. Bethesda, MD; American Physiological Society
1985
5 West J. Respiratory Physiology: The Essentials. 8th ed. Baltimore; Lippincott Williams
& Wilkins 2008
6
McGoon M, Gutterman D, Steen V American College of Chest Physicians et al.
Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP
evidence-based clinical practice guidelines.
Chest.
2004;
126(1, Suppl)
14S-34S
7
McGoon M D, Krichman A, Farber H W et al..
Design of the REVEAL registry for US patients with pulmonary arterial hypertension.
Mayo Clin Proc.
2008;
83
923-931
8
Bossone E, Paciocco G, Iarussi D et al..
The prognostic role of the ECG in primary pulmonary hypertension.
Chest.
2002;
121
513-518
9
Henkens I R, Gan C T, van Wolferen S A et al..
ECG monitoring of treatment response in pulmonary arterial hypertension patients.
Chest.
2008;
134
1250-1257
10
Ahearn G S, Tapson V F, Rebeiz A, Greenfield Jr J C.
Electrocardiography to define clinical status in primary pulmonary hypertension and
pulmonary arterial hypertension secondary to collagen vascular disease.
Chest.
2002;
122
524-527
11
Algeo S, Morrison D, Ovitt T, Goldman S.
Noninvasive detection of pulmonary hypertension.
Clin Cardiol.
1984;
7
148-156
12
Lupi E, Dumont C, Tejada V M, Horwitz S, Galland F.
A radiologic index of pulmonary arterial hypertension.
Chest.
1975;
68
28-31
13
Teichmann V, Jezek V, Herles F.
Relevance of width of right descending branch of pulmonary artery as a radiological
sign of pulmonary hypertension.
Thorax.
1970;
25
91-96
14
Matthay R A, Schwarz M I, Ellis Jr J H et al..
Pulmonary artery hypertension in chronic obstructive pulmonary disease: determination
by chest radiography.
Invest Radiol.
1981;
16
95-100
15
Chetty K G, Brown S E, Light R W.
Identification of pulmonary hypertension in chronic obstructive pulmonary disease
from routine chest radiographs.
Am Rev Respir Dis.
1982;
126
338-341
16
Schmidt H C, Kauczor H U, Schild H H et al..
Pulmonary hypertension in patients with chronic pulmonary thromboembolism: chest radiograph
and CT evaluation before and after surgery.
Eur Radiol.
1996;
6
817-825
17
Sun X G, Hansen J E, Oudiz R J, Wasserman K.
Pulmonary function in primary pulmonary hypertension.
J Am Coll Cardiol.
2003;
41
1028-1035
18
Meyer F J, Ewert R, Hoeper M M German PPH Study Group et al.
Peripheral airway obstruction in primary pulmonary hypertension.
Thorax.
2002;
57
473-476
19
Meyer F J, Lossnitzer D, Kristen A V et al..
Respiratory muscle dysfunction in idiopathic pulmonary arterial hypertension.
Eur Respir J.
2005;
25
125-130
20
Kabitz H J, Schwoerer A, Bremer H C et al..
Impairment of respiratory muscle function in pulmonary hypertension.
Clin Sci (Lond).
2008;
114
165-171
21
Chang B, Schachna L, White B, Wigley F M, Wise R A.
Natural history of mild-moderate pulmonary hypertension and the risk factors for severe
pulmonary hypertension in scleroderma.
J Rheumatol.
2006;
33
269-274
22
Steen V, Medsger Jr T A.
Predictors of isolated pulmonary hypertension in patients with systemic sclerosis
and limited cutaneous involvement.
Arthritis Rheum.
2003;
48
516-522
23
Launay D, Mouthon L, Hachulla E et al..
Prevalence and characteristics of moderate to severe pulmonary hypertension in systemic
sclerosis with and without interstitial lung disease.
J Rheumatol.
2007;
34
1005-1011
24
Steen V D, Graham G, Conte C, Owens G, Medsger Jr T A.
Isolated diffusing capacity reduction in systemic sclerosis.
Arthritis Rheum.
1992;
35
765-770
25
Hsu V M, Moreyra A E, Wilson A C et al..
Assessment of pulmonary arterial hypertension in patients with systemic sclerosis:
comparison of noninvasive tests with results of right-heart catheterization.
J Rheumatol.
2008;
35
458-465
26
Oudiz R J.
The role of exercise testing in the management of pulmonary arterial hypertension.
Semin Respir Crit Care Med.
2005;
26
379-384
27
Tolle J J, Waxman A B, Van Horn T L, Pappagianopoulos P P, Systrom D M.
Exercise-induced pulmonary arterial hypertension.
Circulation.
2008;
118
2183-2189
28
Dantzker D R, D'Alonzo G E, Bower J S, Popat K, Crevey B J.
Pulmonary gas exchange during exercise in patients with chronic obliterative pulmonary
hypertension.
Am Rev Respir Dis.
1984;
130
412-416
29
Riley M S, Pórszász J, Engelen M P, Brundage B H, Wasserman K.
Gas exchange responses to continuous incremental cycle ergometry exercise in primary
pulmonary hypertension in humans.
Eur J Appl Physiol.
2000;
83
63-70
30
D'Alonzo G E, Gianotti L A, Pohil R L et al..
Comparison of progressive exercise performance of normal subjects and patients with
primary pulmonary hypertension.
Chest.
1987;
92
57-62
31
Dantzker D R, Bower J S.
Mechanisms of gas exchange abnormality in patients with chronic obliterative pulmonary
vascular disease.
J Clin Invest.
1979;
64
1050-1055
32
Hansen J E, Ulubay G, Chow B F, Sun X G, Wasserman K.
Mixed-expired and end-tidal CO2 distinguish between ventilation and perfusion defects during exercise testing in
patients with lung and heart diseases.
Chest.
2007;
132
977-983
33
Sun X G, Hansen J E, Oudiz R J, Wasserman K.
Exercise pathophysiology in patients with primary pulmonary hypertension.
Circulation.
2001;
104
429-435
34
Markowitz D H, Systrom D M.
Diagnosis of pulmonary vascular limit to exercise by cardiopulmonary exercise testing.
J Heart Lung Transplant.
2004;
23
88-95
35
Tolle J J, Waxman A B, Van Horn T L, Pappagianopoulos P P, Systrom D M.
Exercise-induced pulmonary arterial hypertension.
Circulation.
2008;
118
2120-2121
36
Oudiz R J, Roveran G, Hansen J E, Sun X G, Wasserman K.
Effect of sildenafil on ventilatory efficiency and exercise tolerance in pulmonary
hypertension.
Eur J Heart Fail.
2007;
9
917-921
37
Wensel R, Opitz C F, Anker S D et al..
Assessment of survival in patients with primary pulmonary hypertension: importance
of cardiopulmonary exercise testing.
Circulation.
2002;
106
319-324
38
Guyatt G H, Sullivan M J, Thompson P J et al..
The 6-minute walk: a new measure of exercise capacity in patients with chronic heart
failure.
Can Med Assoc J.
1985;
132
919-923
39
Miyamoto S, Nagaya N, Satoh T et al..
Clinical correlates and prognostic significance of six-minute walk test in patients
with primary pulmonary hypertension: comparison with cardiopulmonary exercise testing.
Am J Respir Crit Care Med.
2000;
161(2 Pt 1)
487-492
40
Paciocco G, Martinez F J, Bossone E, Pielsticker E, Gillespie B, Rubenfire M.
Oxygen desaturation on the six-minute walk test and mortality in untreated primary
pulmonary hypertension.
Eur Respir J.
2001;
17
647-652
41
Rafanan A L, Golish J A, Dinner D S, Hague L K, Arroliga A C.
Nocturnal hypoxemia is common in primary pulmonary hypertension.
Chest.
2001;
120
894-899
42
Minai O A, Pandya C M, Golish J A et al..
Predictors of nocturnal oxygen desaturation in pulmonary arterial hypertension.
Chest.
2007;
131
109-117
43
Hoeper M M, Pletz M W, Golpon H, Welte T.
Prognostic value of blood gas analyses in patients with idiopathic pulmonary arterial
hypertension.
Eur Respir J.
2007;
29
944-950
44
Njaman W, Iesaki T, Iwama Y, Takasaki Y, Daida H.
Serum uric acid as a prognostic predictor in pulmonary arterial hypertension with
connective tissue disease.
Int Heart J.
2007;
48
523-532
45
Nagaya N, Uematsu M, Satoh T et al..
Serum uric acid levels correlate with the severity and the mortality of primary pulmonary
hypertension.
Am J Respir Crit Care Med.
1999;
160
487-492
46
Nagaya N, Nishikimi T, Uematsu M et al..
Plasma brain natriuretic peptide as a prognostic indicator in patients with primary
pulmonary hypertension.
Circulation.
2000;
102
865-870
47
Leuchte H H, Holzapfel M, Baumgartner R A et al..
Clinical significance of brain natriuretic peptide in primary pulmonary hypertension.
J Am Coll Cardiol.
2004;
43
764-770
48
Hargett C W, Tapson V F.
Brain natriuretic peptide: diagnostic and therapeutic implications in pulmonary arterial
hypertension.
Semin Respir Crit Care Med.
2005;
26
385-393
49
Gan C T, McCann G P, Marcus J T et al..
NT-proBNP reflects right ventricular structure and function in pulmonary hypertension.
Eur Respir J.
2006;
28
1190-1194
50
Andreassen A K, Wergeland R, Simonsen S, Geiran O, Guevara C, Ueland T.
N-terminal pro-B-type natriuretic peptide as an indicator of disease severity in a
heterogeneous group of patients with chronic precapillary pulmonary hypertension.
Am J Cardiol.
2006;
98
525-529
51
Kampolis C, Plastiras S, Vlachoyiannopoulos P, Moyssakis I, Tzelepis G.
The presence of anti-centromere antibodies may predict progression of estimated pulmonary
arterial systolic pressure in systemic sclerosis.
Scand J Rheumatol.
2008;
37
278-283
52
Shah S J, Thenappan T, Rich S, Tian L, Archer S L, Gomberg-Maitland M.
Association of serum creatinine with abnormal hemodynamics and mortality in pulmonary
arterial hypertension.
Circulation.
2008;
117
2475-2483
53
Chu J W, Kao P N, Faul J L, Doyle R L.
High prevalence of autoimmune thyroid disease in pulmonary arterial hypertension.
Chest.
2002;
122
1668-1673
54
Falcao C A, Alves I C, Chahade W H, Duarte A L, Lucena-Silva N.
Echocardiographic abnormalities and antiphospholipid antibodies in patients with systemic
lupus erythematosus.
Arq Bras Cardiol.
2002;
79
285-291
55
Asherson R A, Higenbottam T W, Dinh Xuan A T, Khamashta M A, Hughes G R.
Pulmonary hypertension in a lupus clinic: experience with twenty-four patients.
J Rheumatol.
1990;
17
1292-1298
56
Rich S, Kieras K, Hart K, Groves B M, Stobo J D, Brundage B H.
Antinuclear antibodies in primary pulmonary hypertension.
J Am Coll Cardiol.
1986;
8
1307-1311
57
Isern R A, Yaneva M, Weiner E et al..
Autoantibodies in patients with primary pulmonary hypertension: association with anti-Ku.
Am J Med.
1992;
93
307-312
58
Lopes A A, Maeda N Y, Gonçalves R C, Bydlowski S P.
Endothelial cell dysfunction correlates differentially with survival in primary and
secondary pulmonary hypertension.
Am Heart J.
2000;
139
618-623
59
Golbin J M, Krowka M J.
Portopulmonary hypertension.
Clin Chest Med.
2007;
28
203-218
, ix
60
Sitbon O.
HIV-related pulmonary arterial hypertension: clinical presentation and management.
AIDS.
2008;
22(Suppl 3)
S55-S62
61
Dorfmüller P, Perros F, Balabanian K, Humbert M.
Inflammation in pulmonary arterial hypertension.
Eur Respir J.
2003;
22
358-363
62
Sanchez O, Marcos E, Perros F et al..
Role of endothelium-derived CC chemokine ligand 2 in idiopathic pulmonary arterial
hypertension.
Am J Respir Crit Care Med.
2007;
176
1041-1047
63
Dorfmüller P, Zarka V, Durand-Gasselin I et al..
Chemokine RANTES in severe pulmonary arterial hypertension.
Am J Respir Crit Care Med.
2002;
165
534-539
64
Bakouboula B, Morel O, Faure A et al..
Procoagulant membrane microparticles correlate with the severity of pulmonary arterial
hypertension.
Am J Respir Crit Care Med.
2008;
177
536-543
65
Miyatake K, Okamoto M, Kinoshita N et al..
Evaluation of tricuspid regurgitation by pulsed Doppler and two-dimensional echocardiography.
Circulation.
1982;
66
777-784
66
Simonson J S, Schiller N B.
Sonospirometry: a new method for noninvasive estimation of mean right atrial pressure
based on two-dimensional echographic measurements of the inferior vena cava during
measured inspiration.
J Am Coll Cardiol.
1988;
11
557-564
67
Ommen S R, Nishimura R A, Hurrell D G, Klarich K W.
Assessment of right atrial pressure with 2-dimensional and Doppler echocardiography:
a simultaneous catheterization and echocardiographic study.
Mayo Clin Proc.
2000;
75
24-29
68
Kircher B J, Himelman R B, Schiller N B.
Noninvasive estimation of right atrial pressure from the inspiratory collapse of the
inferior vena cava.
Am J Cardiol.
1990;
66
493-496
69
Bossone E, Rubenfire M, Bach D S, Ricciardi M, Armstrong W F.
Range of tricuspid regurgitation velocity at rest and during exercise in normal adult
men: implications for the diagnosis of pulmonary hypertension.
J Am Coll Cardiol.
1999;
33
1662-1666
70
McQuillan B M, Picard M H, Leavitt M, Weyman A E.
Clinical correlates and reference intervals for pulmonary artery systolic pressure
among echocardiographically normal subjects.
Circulation.
2001;
104
2797-2802
71
Yock P G, Popp R L.
Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound
in patients with tricuspid regurgitation.
Circulation.
1984;
70
657-662
72
Kitabatake A, Inoue M, Asao M et al..
Noninvasive evaluation of pulmonary hypertension by a pulsed Doppler technique.
Circulation.
1983;
68
302-309
73
Dabestani A, Mahan G, Gardin J M et al..
Evaluation of pulmonary artery pressure and resistance by pulsed Doppler echocardiography.
Am J Cardiol.
1987;
59
662-668
74
Isobe M, Yazaki Y, Takaku F et al..
Prediction of pulmonary arterial pressure in adults by pulsed Doppler echocardiography.
Am J Cardiol.
1986;
57
316-321
75
Willens H J, Chirinos J A, Gomez-Marin O et al..
Noninvasive differentiation of pulmonary arterial and venous hypertension using conventional
and Doppler tissue imaging echocardiography.
J Am Soc Echocardiogr.
2008;
21
715-719
76
Abbas A E, Fortuin F D, Schiller N B, Appleton C P, Moreno C A, Lester S J.
A simple method for noninvasive estimation of pulmonary vascular resistance.
J Am Coll Cardiol.
2003;
41
1021-1027
77
Selimovic N, Rundqvist B, Bergh C H et al..
Assessment of pulmonary vascular resistance by Doppler echocardiography in patients
with pulmonary arterial hypertension.
J Heart Lung Transplant.
2007;
26
927-934
78
Kim Y J, Sohn D W.
Mitral annulus velocity in the estimation of left ventricular filling pressure: prospective
study in 200 patients.
J Am Soc Echocardiogr.
2000;
13
980-985
79
Currie P J, Seward J B, Chan K L et al..
Continuous wave Doppler determination of right ventricular pressure: a simultaneous
Doppler-catheterization study in 127 patients.
J Am Coll Cardiol.
1985;
6
750-756
80
Berger M, Haimowitz A, Van Tosh A, Berdoff R L, Goldberg E.
Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation
using continuous wave Doppler ultrasound.
J Am Coll Cardiol.
1985;
6
359-365
81
Denton C P, Cailes J B, Phillips G D, Wells A U, Black C M, Bois R M.
Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary
hypertension in systemic sclerosis.
Br J Rheumatol.
1997;
36
239-243
82
Shapiro S M, Oudiz R J, Cao T et al..
Primary pulmonary hypertension: improved long-term effects and survival with continuous
intravenous epoprostenol infusion.
J Am Coll Cardiol.
1997;
30
343-349
83
Kim W R, Krowka M J, Plevak D J et al..
Accuracy of Doppler echocardiography in the assessment of pulmonary hypertension in
liver transplant candidates.
Liver Transpl.
2000;
6
453-458
84
Arcasoy S M, Christie J D, Ferrari V A et al..
Echocardiographic assessment of pulmonary hypertension in patients with advanced lung
disease.
Am J Respir Crit Care Med.
2003;
167
735-740
85
Berger M, Hecht S R, Van Tosh A, Lingam U.
Pulsed and continuous wave Doppler echocardiographic assessment of valvular regurgitation
in normal subjects.
J Am Coll Cardiol.
1989;
13
1540-1545
86
Celermajer D S, Marwick T.
Echocardiographic and right heart catheterization techniques in patients with pulmonary
arterial hypertension.
Int J Cardiol.
2008;
125
294-303
87
Aessopos A, Farmakis D, Taktikou H, Loukopoulos D.
Doppler-determined peak systolic tricuspid pressure gradient in persons with normal
pulmonary function and tricuspid regurgitation.
J Am Soc Echocardiogr.
2000;
13
645-649
88
Dib J C, Abergel E, Rovani C, Raffoul H, Diebold B.
The age of the patient should be taken into account when interpreting Doppler assessed
pulmonary artery pressures.
J Am Soc Echocardiogr.
1997;
10
72-73
89
de Divitiis O, Fazio S, Petitto M, Maddalena G, Contaldo F, Mancini M.
Obesity and cardiac function.
Circulation.
1981;
64
477-482
90
Davidson Jr W R, Fee E C.
Influence of aging on pulmonary hemodynamics in a population free of coronary artery
disease.
Am J Cardiol.
1990;
65
1454-1458
91
Ekelund L G, Holmgren A.
Central hemodynamics during exercise.
Circ Res.
1967;
xx-xxi
92
Eysmann S B, Palevsky H I, Reichek N, Hackney K, Douglas P S.
Two-dimensional and Doppler-echocardiographic and cardiac catheterization correlates
of survival in primary pulmonary hypertension.
Circulation.
1989;
80
353-360
93
Raymond R J, Hinderliter A L, Willis P W et al..
Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension.
J Am Coll Cardiol.
2002;
39
1214-1219
94
Lanzarini L, Fontana A, Lucca E, Campana C, Klersy C.
Noninvasive estimation of both systolic and diastolic pulmonary artery pressure from
Doppler analysis of tricuspid regurgitant velocity spectrum in patients with chronic
heart failure.
Am Heart J.
2002;
144
1087-1094
95
Stephen B, Dalal P, Berger M, Schweitzer P, Hecht S.
Noninvasive estimation of pulmonary artery diastolic pressure in patients with tricuspid
regurgitation by Doppler echocardiography.
Chest.
1999;
116
73-77
96
Scapellato F, Temporelli P L, Eleuteri E, Corrà U, Imparato A, Giannuzzi P.
Accurate noninvasive estimation of pulmonary vascular resistance by Doppler echocardiography
in patients with chronic failure heart failure.
J Am Coll Cardiol.
2001;
37
1813-1819
97
Ihlen H, Amlie J P, Dale J et al..
Determination of cardiac output by Doppler echocardiography.
Br Heart J.
1984;
51
54-60
98
Lewis J F, Kuo L C, Nelson J G, Limacher M C, Quinones M A.
Pulsed Doppler echocardiographic determination of stroke volume and cardiac output:
clinical validation of two new methods using the apical window.
Circulation.
1984;
70
425-431
99
Rakowski H, Appleton C, Chan K L et al..
Canadian consensus recommendations for the measurement and reporting of diastolic
dysfunction by echocardiography: from the Investigators of Consensus on Diastolic
Dysfunction by Echocardiography.
J Am Soc Echocardiogr.
1996;
9
736-760
100
Schulman D S, Matthay R A.
The right ventricle in pulmonary disease.
Cardiol Clin.
1992;
10
111-135
101
Tei C, Dujardin K S, Hodge D O et al..
Doppler echocardiographic index for assessment of global right ventricular function.
J Am Soc Echocardiogr.
1996;
9
838-847
102
Ryan T, Petrovic O, Dillon J C, Feigenbaum H, Conley M J, Armstrong W F.
An echocardiographic index for separation of right ventricular volume and pressure
overload.
J Am Coll Cardiol.
1985;
5
918-927
103
Miller D, Farah M G, Liner A, Fox K, Schluchter M, Hoit B D.
The relation between quantitative right ventricular ejection fraction and indices
of tricuspid annular motion and myocardial performance.
J Am Soc Echocardiogr.
2004;
17
443-447
104
Kaul S, Tei C, Hopkins J M, Shah P M.
Assessment of right ventricular function using two-dimensional echocardiography.
Am Heart J.
1984;
107
526-531
105
Forfia P R, Fisher M R, Mathai S C et al..
Tricuspid annular displacement predicts survival in pulmonary hypertension.
Am J Respir Crit Care Med.
2006;
174
1034-1041
106
Alkotob M L, Soltani P, Sheatt M A et al..
Reduced exercise capacity and stress-induced pulmonary hypertension in patients with
scleroderma.
Chest.
2006;
130
176-181
107
Cotrim C, Simões O, Loureiro M J et al..
Stress echocardiography in the evaluation of exercise physiology in patients with
severe arterial pulmonary hypertension: new methodology.
Rev Port Cardiol.
2005;
24
1451-1460
108
Bach D S.
Stress echocardiography for evaluation of hemodynamics: valvular heart disease, prosthetic
valve function, and pulmonary hypertension.
Prog Cardiovasc Dis.
1997;
39
543-554
109
Marwick T H.
Application of stress echocardiography to the evaluation of non-coronary heart disease.
Eur J Echocardiogr.
2000;
1
171-179
110
Collins N, Bastian B, Quiqueree L, Jones C, Morgan R, Reeves G.
Abnormal pulmonary vascular responses in patients registered with a systemic autoimmunity
database: Pulmonary Hypertension Assessment and Screening Evaluation using stress
echocardiography (PHASE-I).
Eur J Echocardiogr.
2006;
7
439-446
111
Steen V, Chou M, Shanmugam V, Mathias M, Kuru T, Morrissey R.
Exercise-induced pulmonary arterial hypertension in patients with systemic sclerosis.
Chest.
2008;
134
146-151
112
Bossone E, Avelar E, Bach D S, Gillespie B, Rubenfire M, Armstrong W F.
Diagnostic value of resting tricuspid regurgitation velocity and right ventricular
ejection flow parameters for the detection of exercise induced pulmonary arterial
hypertension.
Int J Card Imaging.
2000;
16
429-436
113
Oudiz R J, Langleben D.
Cardiac catheterization in pulmonary arterial hypertension: an updated guide to proper
use.
Advances in Pulmonary Hypertension.
2005;
4
15-25
114
Guillinta P, Peterson K L, Ben-Yehuda O.
Cardiac catheterization techniques in pulmonary hypertension.
Cardiol Clin.
2004;
22
401-415
, vi
115 Grossman W. Cardiac Catheterization and Angiography. Philadelphia; Lea & Febiger
1980
116
Swan H J, Ganz W, Forrester J, Marcus H, Diamond G, Chonette D.
Catheterization of the heart in man with use of a flow-directed balloon-tipped catheter.
N Engl J Med.
1970;
283
447-451
117
Rich S, D'Alonzo G E, Dantzker D R, Levy P S.
Magnitude and implications of spontaneous hemodynamic variability in primary pulmonary
hypertension.
Am J Cardiol.
1985;
55
159-163
118
Khalil H H.
Determination of cardiac output in man by a new method based on thermodilution.
Lancet.
1963;
1
1352-1354
119
Yung G L, Fedullo P F, Kinninger K, Johnson W, Channick R N.
Comparison of impedance cardiography to direct Fick and thermodilution cardiac output
determination in pulmonary arterial hypertension.
Congest Heart Fail.
2004;
10(2, Suppl 2)
7-10
120
Kaluski E, Shah M, Kobrin I, Vered Z, Cotter G.
Right heart catheterization: indications, technique, safety, measurements, and alternatives.
HeartDrug.
2003;
3
225-235
121
Fowler N O, Westcott R N, Scott R C.
Normal pressure in the right heart and pulmonary artery.
Am Heart J.
1953;
46
264-267
122
Olschewski H.
Dana Point: what is new in the diagnosis of pulmonary hypertension? [in German].
Dtsch Med Wochenschr.
2008;
133(Suppl 6)
S180-S182
123
McGregor M, Sniderman A.
On pulmonary vascular resistance: the need for more precise definition.
Am J Cardiol.
1985;
55
217-221
124
Naeije R, Torbicki A.
More on the noninvasive diagnosis of pulmonary hypertension: Doppler echocardiography
revisited.
Eur Respir J.
1995;
8
1445-1449
125
Chemla D, Castelain V, Hervé P, Lecarpentier Y, Brimioulle S.
Haemodynamic evaluation of pulmonary hypertension.
Eur Respir J.
2002;
20
1314-1331
126
McLaughlin V V, Shillington A, Rich S.
Survival in primary pulmonary hypertension: the impact of epoprostenol therapy.
Circulation.
2002;
106
1477-1482
127
Sitbon O, Humbert M, Jaïs X et al..
Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension.
Circulation.
2005;
111
3105-3111
128
Rich S, Kaufmann E, Levy P S.
The effect of high doses of calcium-channel blockers on survival in primary pulmonary
hypertension.
N Engl J Med.
1992;
327
76-81
129
Mandel J, Mark E J, Hales C A.
Pulmonary veno-occlusive disease.
Am J Respir Crit Care Med.
2000;
162
1964-1973
130
Ghofrani H A, Wilkins M W, Rich S.
Uncertainties in the diagnosis and treatment of pulmonary arterial hypertension.
Circulation.
2008;
118
1195-1201
131
Komadina K H, Schenk D A, LaVeau P, Duncan C A, Chambers S L.
Interobserver variability in the interpretation of pulmonary artery catheter pressure
tracings.
Chest.
1991;
100
1647-1654
132
Braunwald E, Ross Jr J.
The ventricular end-diastolic pressure: appraisal of its value in the recognition
of ventricular failure in man.
Am J Med.
1963;
34
147-150
133
Nootens M, Wolfkiel C J, Chomka E V, Rich S.
Understanding right and left ventricular systolic function and interactions at rest
and with exercise in primary pulmonary hypertension.
Am J Cardiol.
1995;
75
374-377
134 Reeves J, Groves B M. Approach to Patient with Pulmonary Hypertension. Mount Kisco,
NY; Futura 1984
135 Reeves J TDJ, Grover R F. Pulmonary Circulation during Exercise. New York; Marcel
Dekker 1989
136
Groves B M, Reeves J T, Sutton J R et al..
Operation Everest II: elevated high-altitude pulmonary resistance unresponsive to
oxygen.
J Appl Physiol.
1987;
63
521-530
137
Wagner P D, Gale G E, Moon R E, Torre-Bueno J R, Stolp B W, Saltzman H A.
Pulmonary gas exchange in humans exercising at sea level and simulated altitude.
J Appl Physiol.
1986;
61
260-270
138
Fowler N O.
The normal pulmonary arterial pressure-flow relationships during exercise.
Am J Med.
1969;
47
1-6
139
Bevegard S, Holmgren A, Jonsson B.
The effect of body position on the circulation at rest and during exercise, with special
reference to the influence on the stroke volume.
Acta Physiol Scand.
1960;
49
279-298
140
Brofman B L, Charms B L, Kohn P M, Elder J, Newman R, Rizika M.
Unilateral pulmonary artery occlusion in man; control studies.
J Thorac Surg.
1957;
34
206-227
141
Provencher S, Hervé P, Sitbon O, Humbert M, Simonneau G, Chemla D.
Changes in exercise haemodynamics during treatment in pulmonary arterial hypertension.
Eur Respir J.
2008;
32
393-398
142
Castelain V, Chemla D, Humbert M et al..
Pulmonary artery pressure-flow relations after prostacyclin in primary pulmonary hypertension.
Am J Respir Crit Care Med.
2002;
165
338-340
143
Kessler R, Faller M, Weitzenblum E et al..
“Natural history” of pulmonary hypertension in a series of 131 patients with chronic
obstructive lung disease.
Am J Respir Crit Care Med.
2001;
164
219-224
144
Sherrick A D, Swensen S J, Hartman T E.
Mosaic pattern of lung attenuation on CT scans: frequency among patients with pulmonary
artery hypertension of different causes.
AJR Am J Roentgenol.
1997;
169
79-82
145
Perloff J K, Hart E M, Greaves S M, Miner P D, Child J S.
Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger
syndrome and primary pulmonary hypertension.
Am J Cardiol.
2003;
92
182-187
146
Kuriyama K, Gamsu G, Stern R G, Cann C E, Herfkens R J, Brundage B H.
CT-determined pulmonary artery diameters in predicting pulmonary hypertension.
Invest Radiol.
1984;
19
16-22
147
Haimovici J B, Trotman-Dickenson B, Halpern E F Massachusetts General Hospital Lung
Transplantation Program et al.
Relationship between pulmonary artery diameter at computed tomography and pulmonary
artery pressures at right-sided heart catheterization.
Acad Radiol.
1997;
4
327-334
148
Ng C S, Wells A U, Padley S PA.
A CT sign of chronic pulmonary arterial hypertension: the ratio of main pulmonary
artery to aortic diameter.
J Thorac Imaging.
1999;
14
270-278
149
Hansell D M.
Small-vessel diseases of the lung: CT-pathologic correlates.
Radiology.
2002;
225
639-653
150
Horton M R, Tuder R M.
Primary pulmonary arterial hypertension presenting as diffuse micronodules on CT.
Crit Rev Comput Tomogr.
2004;
45
335-341
151
Nolan R L, McAdams H P, Sporn T A, Roggli V L, Tapson V F, Goodman P C.
Pulmonary cholesterol granulomas in patients with pulmonary artery hypertension: chest
radiographic and CT findings.
AJR Am J Roentgenol.
1999;
172
1317-1319
152
Lippert J L, White C S, Cameron E W, Sun C C, Liang X, Rubin L J.
Pulmonary capillary hemangiomatosis: radiographic appearance.
J Thorac Imaging.
1998;
13
49-51
153
Eksinar S, Gedevanishvili A, Koroglu M et al..
Extrinsic compression of the left main coronary artery in pulmonary hypertension.
JBR-BTR.
2005;
88
190-192
154
Kovacs G, Reiter G, Reiter U, Rienmüller R, Peacock A, Olschewski H.
The emerging role of magnetic resonance imaging in the diagnosis and management of
pulmonary hypertension.
Respiration.
2008;
76
458-470
155
Maceira A M, Prasad S K, Khan M, Pennell D J.
Reference right ventricular systolic and diastolic function normalized to age, gender
and body surface area from steady-state free precession cardiovascular magnetic resonance.
Eur Heart J.
2006;
27
2879-2888
156
Alfakih K, Plein S, Thiele H, Jones T, Ridgway J P, Sivananthan M U.
Normal human left and right ventricular dimensions for MRI as assessed by turbo gradient
echo and steady-state free precession imaging sequences.
J Magn Reson Imaging.
2003;
17
323-329
157
Grothues F, Moon J C, Bellenger N G, Smith G S, Klein H U, Pennell D J.
Interstudy reproducibility of right ventricular volumes, function, and mass with cardiovascular
magnetic resonance.
Am Heart J.
2004;
147
218-223
158
Chatzimavroudis G P, Oshinski J N, Franch R H, Walker P G, Yoganathan A P, Pettigrew R I.
Evaluation of the precision of magnetic resonance phase velocity mapping for blood
flow measurements.
J Cardiovasc Magn Reson.
2001;
3
11-19
159
Gan C T, Lankhaar J W, Westerhof N et al..
Noninvasively assessed pulmonary artery stiffness predicts mortality in pulmonary
arterial hypertension.
Chest.
2007;
132
1906-1912
160
Chin K M, Kingman M, de Lemos J A et al..
Changes in right ventricular structure and function assessed using cardiac magnetic
resonance imaging in bosentan-treated patients with pulmonary arterial hypertension.
Am J Cardiol.
2008;
101
1669-1672
161
Gan C T, Holverda S, Marcus J T et al..
Right ventricular diastolic dysfunction and the acute effects of sildenafil in pulmonary
hypertension patients.
Chest.
2007;
132
11-17
162
Pietra G G, Edwards W D, Kay J M et al..
Histopathology of primary pulmonary hypertension: a qualitative and quantitative study
of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood
Institute, Primary Pulmonary Hypertension Registry.
Circulation.
1989;
80
1198-1206
163
Nicod P, Moser K M.
Primary pulmonary hypertension: the risk and benefit of lung biopsy.
Circulation.
1989;
80
1486-1488
Rajan SaggarM.D.
Division of Pulmonary, Critical Care Medicine, and Hospitalists, David Geffen School
of Medicine at UCLA
18033 Le Conte Ave., 37-131 CHS, Los Angeles, CA 90095-1690
Email: rsaggar@mednet.ucla.edu