Clinical Features and Diagnosis of Pulmonary Hypertension in Adults

29/9/2014 Clinical features and diagnosis of pulmonary hypertension in adults
Official reprint from UpToDate®

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Clinical features and diagnosis of pulmonary hypertension in adults
Authors Section Editor Deputy Editor

Lewis J Rubin, MD Jess Mandel, MD Geraldine Finlay, MD
William Hopkins, MD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Aug 2014. | This topic last updated: Apr 25, 2014.
INTRODUCTION — Patients suspected of having pulmonary hypertension (PH) undergo extensive diagnostic testing. The purpose of diagnostic testing is to confirm that PH exists and
identify the underlying cause.
The clinical features, diagnostic evaluation, and diagnostic criteria for PH are reviewed here. Classification, epidemiology, etiologies, pathogenesis, natural history, treatment, and prognosis
are discussed separately. (See "Overview of pulmonary hypertension in adults" and "Pathogenesis of pulmonary hypertension" and "Treatment of pulmonary hypertension in adults".)
CLINICAL MANIFESTATIONS — Symptoms and signs of pulmonary hypertension (PH) may be difficult to recognize because they are nonspecific. Initially, patients present with
exertional dyspnea and fatigue. Because PH is progressive, the presentation evolves over time so that patients may eventually develop the signs and symptoms of severe pulmonary
hypertension with overt right ventricular failure (eg, exertional chest pain or syncope and congestion including peripheral edema, ascites, and pleural effusion). The diagnosis is often
delayed because the presenting features of PH are frequently attributed incorrectly to age, deconditioning, or a coexisting or alternate medical condition. As a result, PH is often not
suspected until symptoms become severe or serious. It has been estimated that more than 20 percent of patients have symptoms of PH for longer than two years before it is recognized
[1]. This is particularly prevalent among patients younger than 36 years and in those with co-existing medical conditions.
Symptoms — The initial symptoms of PH are the result of an inability to adequately increase cardiac output during exercise [2,3]. These include exertional dyspnea, lethargy, and fatigue.
Additional symptoms emerge as the PH progresses and right ventricular hypertrophy and failure develop. (See "Cor pulmonale", section on 'Symptoms'.)
These include the following:
● Exertional chest pain (ie, angina) is usually due to subendocardial hypoperfusion caused by increased right ventricular wall stress and myocardial oxygen demand. However, it is
occasionally caused by dynamic compression of the left main coronary artery by an enlarged pulmonary artery; this risk is greatest for patients with a pulmonary artery trunk at least
40 mm in diameter [4-6].
● Exertional syncope is due to the inability to increase cardiac output during activity or reflex bradycardia that is secondary to mechanoreceptor activation in the right ventricle.
● Peripheral edema is due to right ventricular failure, increased right sided filling cardiac pressures, and extracellular volume expansion.
● Anorexia and/or abdominal pain in the right upper quadrant due to passive hepatic congestion.
Uncommon symptoms include cough, hemoptysis, and hoarseness (Ortner's syndrome). The hoarseness is caused by compression of the left recurrent laryngeal nerve by a dilated main
pulmonary artery.
Examination — Patients with PH may develop the following physical signs as they progress from PH alone to PH associated with right ventricular failure:
● The initial physical finding of PH is usually increased intensity of the pulmonic component of the second heart sound, which may become palpable. The second heart sound is
narrowly split or single in patients with preserved right ventricular function. (See "Cor pulmonale", section on 'Clinical manifestations' and "Auscultation of heart sounds" and
"Auscultation of cardiac murmurs" and "Examination of the precordial pulsation".)
● As right ventricular hypertrophy develops, a prominent A wave may emerge within the jugular venous pulse. This may be accompanied by a right-sided fourth heart sound and either a
left parasternal heave or a downward subxiphoid thrust. (See "Examination of the jugular venous pulse".)
● Right ventricle failure (or a right bundle branch block) widens the splitting of the second heart sound. Auscultation may reveal a holosystolic murmur of tricuspid regurgitation and,
occasionally, systolic ejection murmur and, in more severe disease, a diastolic pulmonic regurgitation murmur. These right-sided murmurs and gallops are augmented with
inspiration. The systemic venous hypertension caused by right ventricular failure can lead to a variety of findings, such as elevated jugular venous pressure, a right ventricular third
heart sound, and a prominent V wave in the jugular venous pulse. Hepatomegaly, a pulsatile liver, peripheral edema, ascites, and pleural effusion may also exist [7,8].
Some patients with PH due to severe chronic obstructive pulmonary disease (COPD) develop edema even in the absence of right heart failure [9]. The pathogenesis of edema in such
patients is not well understood and is discussed in detail separately [10,11]. (See "Cor pulmonale".)
Some patients may also have the signs and symptoms of the underlying cause of PH (eg, connective tissue disease, heart failure, COPD).
DIAGNOSTIC EVALUATION — Diagnostic testing is indicated whenever pulmonary hypertension (PH) is suspected. The purpose of the diagnostic testing is to confirm that PH exists,
determine its severity, and identify its cause (algorithm 1). Our approach is described below.
Our approach — We suggest beginning the evaluation with an echocardiogram [12]. (See 'Echocardiography' below.)
When the echocardiogram does not suggest PH, clinicians need to be guided by clinical suspicion. The diagnostic evaluation should be directed toward alternative diagnoses if the clinical
suspicion for PH is low, whereas right heart catheterization should be considered if the clinical suspicion for PH is still high.
When the echocardiogram is suggestive of PH, clinicians should determine whether or not there is enough left heart disease on the echocardiogram to explain the degree of estimated PH:
● Patients with enough left heart disease on the echocardiogram to explain the degree of estimated PH do not require further testing to determine the etiology of PH. This may be more
obvious in patients with severe left ventricular systolic dysfunction or severe mitral or aortic valve disease. It may be less obvious in patients with diastolic dysfunction or diastolic
heart failure (heart failure with preserved ejection fraction). If there is uncertainty, right and left heart catheterization may be helpful. (See 'Right heart catheterization' below and
"Cardiac catheterization techniques: Normal hemodynamics".)
● Patients who have no left heart disease, left heart disease that seems insufficient to explain the degree of estimated PH, or a potential second etiology, should undergo additional
diagnostic testing to look for an alternative cause of the PH. The testing may include pulmonary function testing, ventilation-perfusion scanning, overnight oximetry, polysomnography,
and/or laboratory testing (eg, autoimmune serologies, HIV serology, and liver function tests). The sequence of testing should be prioritized according to the history and physical
examination. As an example, pulmonary function testing is an appropriate initial test in a heavy smoker. (See 'Diagnostic tests' below.)
For patients in whom chronic lung disease is suspected, right heart catheterization is usually reserved for those in whom the severity of PH on echocardiogram is not explained by the
severity of their underlying lung disease or for those in whom an alternate etiology is suspected. When pulmonary dysfunction is severe enough to explain the degree of PH on
echocardiogram, right heart catheterization is not indicated.
Right heart catheterization is indicated to confirm the PH and assess its severity, although this is often done at the time that therapy is initiated. Exercise testing may be performed
as well, to determine the functional severity of disease (table 1). (See 'Right heart catheterization' below and "Treatment of pulmonary hypertension in adults".)
Diagnostic tests
Chest radiograph — The classic chest radiograph of a patient with PH shows enlargement of the central pulmonary arteries with attenuation of the peripheral vessels, resulting in
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oligemic lung fields (image 1 and image 2). Right ventricular enlargement (diminished retrosternal space) and right atrial dilatation (prominent right heart border) may also be seen.
Occasionally, the underlying cause of the PH is apparent on the chest radiograph (eg, interstitial lung disease).
Electrocardiography — The electrocardiogram (ECG) of a patient with PH may demonstrate signs of right ventricular hypertrophy or strain (waveform 1), including right axis deviation,
an R wave/S wave ratio greater than one in lead V1, incomplete or complete right bundle branch block, or increased P wave amplitude in lead II (P pulmonale) due to right atrial enlargement
(figure 1 and waveform 2). Most ECG signs are specific but not sensitive for the detection of right ventricular disease. ECG changes do not correlate with disease severity or prognosis
[13,14].
Echocardiography — Echocardiography is performed to estimate the pulmonary artery systolic pressure and to assess right ventricular size, thickness, and function (image 3). In
addition, echocardiography can evaluate right atrial size, left ventricular systolic and diastolic function, and valve function, while detecting pericardial effusions and intracardiac shunts
(movie 1 and movie 2) [15,16].
Echocardiography uses Doppler ultrasound to estimate the pulmonary artery systolic pressure. This technique takes advantage of the tricuspid regurgitation that usually exists. The
maximum tricuspid regurgitant jet velocity is recorded and the pulmonary artery systolic pressure (PASP) is then calculated:
PASP = (4 x [TRV]2) + RAP
where TRV is the maximum tricuspid regurgitant jet velocity and RAP is the right atrial pressure estimated from the size and respiratory variation of flow in the inferior vena cava. Doppler
echocardiography is limited when an adequate tricuspid regurgitant jet cannot be identified. (See "Principles of Doppler echocardiography".)
Patients with PH may have echocardiographic signs of right ventricular pressure overload, including paradoxical bulging of the septum into the left ventricle during systole and hypertrophy
of the right ventricular free wall and trabeculae (image 4 and movie 3). As the right ventricle fails, there is dilation and hypokinesis, septal flattening, right atrial dilation, and tricuspid
regurgitation (movie 4). The tricuspid regurgitation is not due to an intrinsic abnormality of the tricuspid valve; it is a secondary manifestation of dilation of the tricuspid annulus and right
ventricle (ie, functional tricuspid regurgitation) (movie 5 and movie 6) [17]. Other findings associated with pulmonary hypertension are pulmonic insufficiency and midsystolic closure of the
pulmonic valve [18,19]. The echocardiographic findings of PH are summarized in the figure (figure 2).
Based upon a Doppler echocardiographic study, it can be determined if PH is likely, unlikely, or possible [20]:
● PH is likely if the PASP is >50 and the TRV is >3.4
● PH is unlikely if the PASP is ≤36, the TRV is ≤2.8, and there are no other suggestive findings
● PH is possible with other combinations of findings
Doppler echocardiography may be misleading in the assessment of patients with suspected pulmonary hypertension [21,22], especially when an inadequate tricuspid regurgitant jet is over-
interpreted. This was illustrated by an observational study of 65 patients with various types of PH [21]. The pulmonary arterial pressure estimated by Doppler echocardiography was at least
10 mmHg higher or lower than that obtained by right heart catheterization in 48 percent of patients and, in many cases, the difference in pulmonary artery pressure determined by the two
modalities was significantly greater than 10 mmHg. Overestimation and underestimation of pulmonary arterial pressure occurred with similar frequency, although the magnitude of the
underestimation was greater. A major limitation of the study was that catheterization and Doppler echocardiography were not performed simultaneously.
We believe that there should be a low threshold to evaluate patients with suspected pulmonary hypertension via right heart catheterization. Despite its limitations, Doppler
echocardiography detects PH with greater accuracy than clinical history and physical examination [23,24].
Pulmonary function tests — Pulmonary function tests (PFTs) are performed to identify and characterize underlying lung disease that may be contributing to PH. An obstructive
pattern is suggestive of COPD, while restrictive disease suggests interstitial lung disease, neuromuscular weakness, or chest wall disease. (See "Overview of pulmonary function testing in
adults".)
It is usually severe interstitial lung disease (with lung volumes below 50 percent of predicted) or obstructive lung disease that produces PH. In most circumstances, PH should not be
attributed to lung disease if the PFTs are only mildly abnormal since PH itself can cause PFT abnormalities. As an example, a study of 79 patients with idiopathic pulmonary arterial
hypertension (IPAH) demonstrated that more than half of patients had a mild to moderate decrease of forced expiratory volume in one second (FEV1) or forced vital capacity (FVC)
compared to age and sex matched controls [25]. The diffusing capacity for carbon monoxide (DLCO) is usually decreased in PH [25,26].
Overnight oximetry — Nocturnal oxyhemoglobin desaturation can be identified by overnight oximetry. It is common in patients with PH and may prompt supplemental oxygen therapy
during sleep [27]. Overnight oximetry is not an acceptable diagnostic test for sleep related breathing disorders, such as obstructive sleep apnea. (See "Treatment of pulmonary
hypertension in adults", section on 'Oxygen therapy' and "Out-of-center sleep testing for obstructive sleep apnea in adults", section on 'Pulse oximetry'.)
Polysomnography — Polysomnography is the gold standard diagnostic test for sleep related breathing disorders, such as obstructive sleep apnea (OSA). It should be performed when
the clinical suspicion for OSA is high, or the results of overnight oximetry are discordant with clinical expectation. (See "Out-of-center sleep testing for obstructive sleep apnea in adults"
and "Polysomnography in obstructive sleep apnea in adults".)
V/Q scan — Ventilation-perfusion (V/Q) scanning is the preferred imaging study to evaluate patients for chronic thromboembolic disease (chronic thromboembolic pulmonary
hypertension [CTEPH]). A normal V/Q scan accurately excludes chronic thromboembolic disease with a sensitivity of 96 to 97 percent and a specificity of 90 to 95 percent [28]. When the
V/Q scan suggests that chronic thromboembolic disease exists, pulmonary angiography is necessary to confirm the positive V/Q scan and to define the extent of disease (image 5). V/Q
scans are an important part of the diagnostic evaluation because PH due to chronic thromboembolic disease is potentially reversible with surgery. (See "Clinical manifestations and
diagnosis of chronic thromboembolic pulmonary hypertension".)
Laboratory tests — Blood tests performed during the diagnostic evaluation of PH include:
● HIV serology to screen for HIV-associated PH

● Liver function tests to screen for portopulmonary hypertension
● Antinuclear antibody (ANA) to screen for PAH due to connective tissue diseases; consider rheumatoid factor (RF) and antineutrophil cytoplasmic antibody (ANCA) if clinically
indicated
In the appropriate clinical setting, laboratory studies looking for evidence of chronic hemolytic anemia (eg, sickle cell disease) or schistosomiasis are appropriate. (See "Diagnosis of sickle
cell disorders" and "Diagnosis of schistosomiasis".)
N-terminal pro-brain natriuretic peptide (NT-proBNP) is the precursor of brain natriuretic peptide (BNP). Both peptides are released from the myocardial tissue of the right and left ventricle
when stretched. NT-proBNP and BNP are helpful in diagnosing heart failure. However, they do not distinguish between left or right ventricular failure. They also play a prognostic role in
patients with PH, although accuracy diminishes if renal dysfunction exists [29-32].
Exercise testing — Exercise testing is usually performed using the six-minute walk test (6MWT), stress echocardiography, or cardiopulmonary exercise testing. The latter can be
performed with gas exchange measurements, echocardiography, and/or right heart catheterization.
Exercise testing during the diagnostic evaluation of PH serves several purposes:
● Screens for alternative causes of the patient's symptoms
● Detects exercised-induced PH, which may be an intermediate stage that exists between normal and resting PH [33,34]
● Determines the patient's World Health Organization (WHO) functional class [35], which guides therapy (table 1)
● Establishes a baseline from which the response to therapy can be measured [36,37]
● Provides prognostic information, since a longer distance walked during the 6MWT is associated with longer survival [35]
Right heart catheterization — Right heart catheterization is necessary to confirm the diagnosis of PH (see 'Diagnostic criteria' below) and accurately determine the severity of the
hemodynamic derangements. Hemodynamic values of normal recumbent adults are listed in the table (table 2).
Right heart catheterization is also helpful in distinguishing patients who have PH due to left heart disease, such as systolic dysfunction, diastolic dysfunction, or valvular heart disease
(pulmonary venous hypertension) (table 3) [38]. Such patients have a mean pulmonary capillary wedge pressure (PCWP) ≥15 mmHg, as measured by right heart catheterization. However,
the mean PCWP alone is insufficient to definitively diagnose PH due to left heart disease because it may be falsely elevated due to dilatation of the pulmonary arteries and incomplete
"wedging" of the balloon catheter. To avoid this dilemma, patients with a mean PCWP ≥15 mmHg should have their left heart filling pressure directly assessed by measuring the left
ventricular end diastolic pressure (LVEDP) via left heart catheterization. Failure to do so means patients may be incorrectly diagnosed as having PH due to left heart disease, which has
important therapeutic implications. (See "Treatment of pulmonary hypertension in adults".)
The risk of incorrectly diagnosing patients with PH due to left heart disease on the basis of a falsely elevated mean PCWP was demonstrated by a retrospective analysis of hemodynamic
data from 3926 patients with PH who underwent simultaneous right and left heart catheterization [39]. Among the 3346 patients who had a mean PCWP >15 mmHg, 152 patients (5
percent) had a normal left ventricular filling pressure, defined as a LVEDP ≤15 mmHg. The authors of the study focused on how frequently the mean PCWP was ≤15 mmHg when the
LVEDP was >15 mmHg, but it should be emphasized that the mean PCWP is virtually always less than the LVEDP because the latter reflects the pressure at the end of left atrial
contraction.
An additional benefit of right heart catheterization is that the presence and/or severity of a congenital or acquired left-to-right shunt can be confirmed when noninvasive studies are not
definitive.
DIAGNOSTIC CRITERIA — The definitive diagnosis of pulmonary hypertension (PH) requires right heart catheterization (RHC). PH is confirmed when the mean pulmonary artery pressure
is ≥25 mmHg at rest [40]. A mean pulmonary artery pressure of 8 to 20 mmHg at rest is considered normal, while a mean pulmonary artery pressure of 21 to 24 mmHg at rest has
uncertain clinical implications.
Clinical studies and additional information provided by RHC are necessary to then classify the patient into an appropriate World Health Organization (WHO) PH category. The WHO
classification of PH is based upon etiology and mechanism and classifies PH into the five groups listed below (table 3) [38,41]. Pulmonary arterial hypertension (PAH) refers to group 1
PAH. Pulmonary hypertension (PH) refers to any of group 2 through group 5 PH and is also used when referring to all five groups collectively. (See "Overview of pulmonary hypertension in
adults".)
● Group 1 – PAH
● Group 2 – PH due to left heart disease
● Group 3 – PH due to chronic lung disease and/or hypoxemia
● Group 4 – Chronic thromboembolic pulmonary hypertension (CTEPH)
● Group 5 – PH due to unclear multifactorial mechanisms
Group 1 - Pulmonary arterial hypertension — The diagnosis of PAH requires right heart catheterization (RHC) to demonstrate a mean pulmonary artery pressure ≥25 mmHg at rest.
Several additional criteria to exclude the remaining categories of PH must also be met:
● Mean pulmonary capillary wedge pressure <15 mmHg (to exclude PH due to left heart disease [ie, group 2 PH])
● Chronic lung diseases and other causes of hypoxemia are mild or absent (to exclude PH owing to left lung disease or hypoxemia [ie, group 3 PH])
● Venous thromboembolic disease is absent (to exclude chronic thromboembolic PH [ie, group 4 PH])
● Certain miscellaneous disorders are absent, including systemic disorders (eg, sarcoidosis), hematologic disorders (eg, myeloproliferative diseases), and metabolic disorders (eg,
glycogen storage disease). The purpose is to exclude PH with unclear multifactorial mechanisms (group 5 PH).
The diagnosis of PAH is also supported by an increased pulmonary vascular resistance and transpulmonary gradient (the difference between the mean pulmonary artery pressure and the
mean pulmonary capillary wedge pressure).
Group 2 - PH due to left heart disease — For patients in whom RHC is performed for suspected PH due to left heart disease (PH-LHD), most clinicians agree on the following RHC
criteria to confirm PH-LHD: mean pulmonary arterial pressure (mPAP) ≥25 mmHg, pulmonary artery wedge pressure (PAWP) ≥15 mmHg and a normal or reduced cardiac output. Important
adjunct information is the presence of left atrial enlargement on an echocardiogram. Left atrial enlargement is a “biomarker” for the presence of chronic left atrial hypertension.
Importantly, patients who have passive elevations in the pulmonary artery pressure (isolated post-capillary PH) need to be distinguished from those with reactive pulmonary artery pressures
that are “out of proportion” to the severity of the underlying cardiac function (ie, combined pre-and post-capillary PH). Patients in the combined group may be pathogenetically different to
those with isolated post-capillary hypertension and, whenever possible, these patients should be referred to a specialized center for individualized patient care and for consideration of
medical therapy for PAH or enrollment in a clinical trial.
Group 3 - PH due to chronic lung disease and/or hypoxemia — The diagnosis of PH due to chronic lung disease and/or hypoxemia is made by the demonstration of PH on RHC or
echocardiogram and evidence of moderate to severe lung dysfunction and/or hypoxemia. All other causes of PH should also be ruled out.
Group 4 - PH due to chronic thromboembolic pulmonary hypertension — Chronic thromboembolic pulmonary hypertension (CTEPH) is diagnosed by PH on RHC that occurs in the
presence of thromboembolic occlusion of the proximal or distal pulmonary vasculature and in the absence of other causes of PH. (See "Clinical manifestations and diagnosis of chronic
thromboembolic pulmonary hypertension", section on 'Diagnostic criteria'.)
Group 5 - PH due to mulifactorial mechanisms — Patients with PH who do not clearly fit into a WHO classification or have combined etiologies may fall into this group (eg, sickle cell
disease).
DIFFERENTIAL DIAGNOSIS — The differential diagnosis of early PH is wide because there are many causes of exertional dyspnea, which is the most common initial symptom. The
evaluation and differential diagnosis of dyspnea is described separately. (See "Approach to the patient with dyspnea".)
Once the PH progresses to right ventricular hypertrophy and failure (ie, peripheral edema, exertional chest pain, exertional syncope, and right upper quadrant pain), the differential diagnosis
becomes considerably narrower:
● Left-sided heart failure – Left ventricular systolic and diastolic heart failure are well-known causes of peripheral edema, right upper quadrant pain due to hepatic congestion, and
syncope due to arrhythmias or insufficient cardiac output. Exertional chest pain may also occur. Left-sided heart failure and PH can be distinguished via echocardiography and/or right
and left heart catheterization. (See "Evaluation of the patient with suspected heart failure".)
● Coronary artery disease – Myocardial ischemia is the most common cause of exertional chest pain and it can also cause ischemia-induced arrhythmias with exertional syncope.
Coronary artery disease is identified by stress testing and/or left heart catheterization. (See "Stress testing for the diagnosis of coronary heart disease".)
● Liver disease – Acute and chronic liver diseases cause peripheral edema and the former can also cause right upper quadrant pain. Liver disease can be detected by liver function
testing and right upper quadrant ultrasound. Liver biopsy is the gold standard, but it is seldom necessary.
● Budd-Chiari syndrome – Budd-Chiari syndrome refers to thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. It causes peripheral edema due to
venous outflow obstruction and right upper quadrant pain due to hepatic congestion. Budd-Chiari syndrome can be identified by Doppler ultrasonography, computed tomography (CT),
magnetic resonance imaging (MRI), or venography. (See "Budd-Chiari syndrome: Epidemiology, clinical manifestations, and diagnosis".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, “The Basics” and “Beyond the Basics.” The Basics patient education pieces are written in
plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who
want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are
written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a
variety of subjects by searching on “patient info” and the keyword(s) of interest.)
● Basics topics (see "Patient information: Pulmonary hypertension in adults (The Basics)")
SUMMARY AND RECOMMENDATIONS
● Patients with pulmonary hypertension (PH) initially present with exertional dyspnea and fatigue, that progress to develop the signs and symptoms of severe PH or right ventricular
failure (eg, exertional chest pain or syncope, and congestion including peripheral edema, ascites, and pleural effusion). The diagnosis is often delayed because the presenting
features of PH are frequently attributed incorrectly to age, deconditioning, or a coexisting or alternate medical condition. (See 'Clinical manifestations' above.)
● Diagnostic testing is indicated whenever PH is suspected. The purpose of the diagnostic testing is to confirm that PH exists, determine its severity, and identify its cause. (See
'Diagnostic evaluation' above.)
● We suggest beginning with an echocardiogram (algorithm 1):
• Echocardiogram is NOT suggestive of PH – For patients whose echocardiogram is NOT suggestive of PH, the diagnostic evaluation should be guided by clinical suspicion. If the
clinical suspicion for PH is low, evaluation of the patient's symptoms should be directed toward alternative diagnoses. Alternatively, if the clinical suspicion for PH remains high
despite the echocardiographic findings, right heart catheterization should be performed. (See 'Diagnostic evaluation' above.)
• Echocardiogram is suggestive of PH – No further testing for PH is required if there is enough left heart disease on the echocardiogram to explain the degree of estimated PH.
However, additional diagnostic testing is required if there is either no evidence of left heart disease or the extent of left heart disease seems insufficient to explain the degree of
estimated PH. (See 'Diagnostic evaluation' above.) The sequence of testing should be prioritized according to the patient's history and physical examination. (See 'Diagnostic
tests' above.)
● Diagnosis of PH requires right heart catheterization (RHC). PH is confirmed when the mean pulmonary artery pressure is ≥25 mmHg at rest. Clinical studies and additional information
provided by RHC are necessary to then classify the patient into an appropriate World Health Organization (WHO) PH category (group 1 through 5). (See 'Diagnostic criteria' above.)
● Pulmonary arterial hypertension (PAH) is one type of PH. The diagnosis of PAH requires right heart catheterization and that several criteria be met (see 'Diagnostic criteria' above):
• Mean pulmonary artery pressure is ≥25 mmHg at rest
• Mean pulmonary capillary wedge pressure <15 mmHg
• Chronic lung diseases and other causes of hypoxemia are mild or absent
• Venous thromboembolic disease is absent
• Certain miscellaneous disorders are absent, including systemic disorders (eg, sarcoidosis), hematologic disorders (eg, myeloproliferative diseases), and metabolic disorders
(eg, glycogen storage disease)
● The differential diagnosis of early PH is wide because there are many causes of exertional dyspnea, which is the most common initial symptom. However, once the PH progresses to
right ventricular hypertrophy and failure (ie, peripheral edema, exertional chest pain, exertional syncope, and right upper quadrant pain), the differential diagnosis becomes
considerably narrower and includes left-sided heart failure, coronary artery disease, liver disease, and Budd-Chiari syndrome. (See 'Differential diagnosis' above.)
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31. Blyth KG, Groenning BA, Mark PB, et al. NT-proBNP can be used to detect right ventricular systolic dysfunction in pulmonary hypertension. Eur Respir J 2007; 29:737.
32. Leuchte HH, El Nounou M, Tuerpe JC, et al. N-terminal pro-brain natriuretic peptide and renal insufficiency as predictors of mortality in pulmonary hypertension. Chest 2007; 131:402.
33. Tolle JJ, Waxman AB, Van Horn TL, et al. Exercise-induced pulmonary arterial hypertension. Circulation 2008; 118:2183.
34. Kovacs G, Berghold A, Scheidl S, Olschewski H. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J 2009; 34:888.
35. Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54:S55.
36. Grünig E, Lichtblau M, Ehlken N, et al. Safety and efficacy of exercise training in various forms of pulmonary hypertension. Eur Respir J 2012; 40:84.
37. Mathai SC, Puhan MA, Lam D, Wise RA. The minimal important difference in the 6-minute walk test for patients with pulmonary arterial hypertension. Am J Respir Crit Care Med
2012; 186:428.
38. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013; 62:D34.
39. Halpern SD, Taichman DB. Misclassification of pulmonary hypertension due to reliance on pulmonary capillary wedge pressure rather than left ventricular end-diastolic pressure.
Chest 2009; 136:37.
40. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 2013; 62:D42.
41. Rich S (ed). Executive summary from the World Symposium on Primary Pulmonary Hypertension, Evian, France, September 6-10, 1998, co-sponsored by The World Health
Organization.
Topic 8249 Version 14.0
GRAPHICS
Algorithm for investigation of suspected pulmonary

hypertension
PH: pulmonary hypertension; PAH: pulmonary arterial hypertension; ANA:

antinuclear antibody; RF: rheumatoid factor; ANCA: antineutrophil cytoplasmic
antibody.
Graphic 72553 Version 2.0
World Health Organization (WHO) functional classification for pulmonary hypertension
Class WHO functional classification
I Patients with pulmonary hypertension but without resulting limitations of physical activity. Ordinary physical activity does not cause undue fatigue or
dyspnea, chest pain, or heart syncope.
II Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity results in
undue fatigue or dyspnea, chest pain, or heart syncope.
III Patients with pulmonary hypertension resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary physical
activity causes undue fatigue or dyspnea, chest pain, or heart syncope.
IV Patients with pulmonary hypertension resulting in inability to carry on any physical activity without symptoms. These patients manifest signs of right
heart failure. Dyspnea and/or fatigue may be present even at rest. Discomfort is increased by physical activity.
Data from: Rich, S. Primary pulmonary hypertension: executive summary. Evian, France. World Health Organization, 1998.
Pulmonary hypertension
This plain frontal chest radiograph from a 50-year-old male

demonstrates prominence of the interstitial pulmonary markings with
enlargement of the right and left ventricle and the right atrium (black
arrow). Additionally, large central, but attenuated peripheral
pulmonary arteries are noted (white arrow), all features characteristic
of pulmonary hypertension.
Photo courtesy of Jonathan Kruskal, MD.
Normal chest radiograph
Posteroanterior view of a normal chest radiograph.
Courtesy of Carol M Black, MD.
Pulmonary hypertension
Chest radiograph in lateral view showing decreased retrosternal space

(arrow) due to right ventricular enlargement in pulmonary
hypertension.
Courtesy of Sven Paulin, MD.
Normal lateral chest radiograph
Courtesy of Steven Weinberger, MD.
Electrocardiogram showing right ventricular hypertrophy
Right ventricular hypertrophy due, in this case, to idiopathic pulmonary

arterial hypertension. The characteristic features include marked right axis deviation
(+210º which is equal to -150º), tall R wave in V1 (as part of a qR complex), delayed
precordial transition zone with prominent S waves in leads V5 and V6, inverted T waves
and ST depression in V1 to V3 consistent with right ventricular "strain", and peaked P
waves in lead II consistent with concomitant right atrial enlargement.
Courtesy of Ary Goldberger, MD.
Normal ECG
Normal electrocardiogram showing normal sinus rhythm at a rate of 75 beats/min, a PR

interval of 0.14 sec, a QRS interval of 0.10 sec, and a QRS axis of approximately 75°.
Courtesy of Ary Goldberger, MD.
http://www.uptodate.com.consultaremota.upb.edu.co/contents/clinical-features-and-diagnosis-of-pulmonary-hypertension-in-adults?topicKey=PULM%2F8249&… 10/19
Right atrial enlargement
The left panel shows the normal pattern of atrial activation as it would
appear on lead II of the electrocardiogram: activation of the right atrium
(RA) occurs first, followed by left atrial (LA) activation. The right panel
shows the pattern in a patient with right atrial enlargement: delayed
activation of the right atrium, due to dilatation, hypertrophy, scarring, or a
conduction abnormality, results in simultaneous activation of the right and
left atria. The synchronous electrical activity has an additive effect upon
the surface ECG, resulting in a relatively narrow P wave which is of
increased amplitude (P pulmonale). The amplitude of the P wave may
become significantly increased in the presence of right atrial hypertrophy; it
also becomes peaked due to the increase in amount of depolarized tissue.
Electrocardiograms showing atrial enlargement
P wave morphology with atrial enlargement in leads I, II, and V1. The P
waves in left atrial enlargement (left panel) are wide (>0.12 sec) and
notched in leads I and II and the terminal segment has a negative
deflection that is deep and delayed in V1. In right atrial enlargement
(middle panel), the P wave amplitude is increased (0.28 mV) in lead II.
Biatrial enlargement (right panel) has characteristics of both atrial
abnormalities: the P wave amplitude (0.22 mV) and duration (0.12 sec)
are increased in lead II and there is deep terminal negativity in V1.
Courtesy of Morton Arnsdorf, MD.
Echocardiogram apical 4-chamber with and without

saline contrast showing enlargement of the right
heart chambers
Panel A: Apical four-chamber view from a patient with severe

idiopathic pulmonary arterial hypertension associated with tricuspid
regurgitation. There is a large apex-forming right ventricle (RV), large
right atrium (RA), and small left ventricle (LV) and left atrium (LA).
Panel B: Agitated saline contrast is injected intravenously and results
in RA and RV opacification; four bubbles are seen in the LV (arrow),
possibly due to right-to-left flow across a patent foramen ovale.
Panel C: Similar echocardiographic findings can be seen in patients
with primary volume overload of the right ventricle. This panel
shows an apical four chamber view from a patient with a large left-to-
right shunt due to an atrial septal defect (ASD). The RV is apex-
forming but the RV and RA are not as large as in panel A.
Panel D: Contrast is injected intravenously and a few bubbles are
seen in the LV; more importantly, there is a prominent negative
contrast (nc) effect due to opacified atrial blood.
Echocardiogram short axis showing RV pressure

overload in advanced pulmonary hypertension
The short axis view at the level of the mitral chordae from a patient
with advanced pulmonary hypertension shows substantial morphologic
changes, including severe hypertrophy of the right ventricular (RV)
wall, dilation of the RV chamber and hypertrophy of the right side of
the septum. The septum is flattened, strongly suggesting pressure
overload in the RV; this septal shape imparts a "D shape" to the left
ventricle (LV) which has relatively thin walls.
Representation of echocardiographic findings in

severe pulmonary hypertension
Schematic representation of two-dimensional and Doppler

echocardiographic changes in severe pulmonary hypertension. The
main findings are right ventricular enlargement (RVE), right ventricular
hypertrophy (RVH), right atrial enlargement (RAE), functional tricuspid
regurgitation (TR) with a high velocity regurgitant jet by Doppler (TR
jet), and a mid-systolic notch on the pulmonary artery Doppler flow
tracing (PA flow). The interventricular septum is shifted toward the
left ventricular cavity, resulting in flattening of the ventricular septum
particularly during systole; this flattening is most evident in the short-
axis left ventricular cavity view (not shown).
Adapted from: Otto C. Cardiomyopathies, hypertensive and pulmonary heart

disease. In: Textbook of Clinical Echocardiography, Otto C, Pearlman AS (Eds),
Saunders, Philadelphia, 1995.
Idiopathic pulmonary arterial hypertension
This angiogram, obtained after injection of contrast material into the

proximal right pulmonary artery, demonstrates a large central
pulmonary artery with marked attenuation of the peripheral pulmonary
arteries with no intraluminal filling defects to suggest the presence of
emboli.
Photo courtesy of Jonathan Kruskal, MD.
Hemodynamic values of normal recumbent adults
Mean Range
Cardiac index, liters/min/m2 3.4 2.8-4.2
Stroke volume index, ml/m2/beat 47 30-65
Arteriovenous oxygen difference, ml per liter of blood 38 30-48
Arterial saturation, percent 98 94-100
Pressure*, mmHg
Left ventricle
Systolic 130 90-140
End-diastolic 7 4-12
Left atrium
Maximum 13 6-20
Minimum 3 -2-+9
Mean 7 4-12
Pulmonary artery wedge ("PC")

Maximum 16 9-23
Minimum 6 1-12
Mean 9 6-15
Pulmonary artery
Systolic 24 15-28
Diastolic 10 5-16
Mean 16 10-22
Right ventricle
Systolic 24 15-28
End-diastolic 4 0-8
Right atrium
Maximum 7 2-14
Minimum 2 -2-+6
Mean 4 -1-+8
Venae cavae
Maximum 7 2-14
Minimum 5 0-8
Mean 6 1-10
End-diastolic volume index

Left ventricular, ml/m 2 70 50-90
Resistance, dyn•s/cm5
Total systemic 1150 900-1400
Systemic arteriolar 850 600-900
Total pulmonary 200 150-250
Pulmonary arteriolar 70 45-120
* Baseline for pressure measurements one-half of anteroposterior chest diameter. 1 mmHg = 133.332 Pascal (PA) = 0.133 kPa.
Reproduced with permission from: Hurst JW, Rackley CE, Sonnenblick EH, Wenger NK. The Heart: Arteries and veins. 7th ed. McGraw-Hill, Inc, New York 1990. Copyright ©
1990 McGraw-Hill Companies, Inc.
Clinical classification of pulmonary hypertension (NICE, 2013)
1. Pulmonary arterial hypertension (PAH)
1.1. Idiopathic PAH
1.2. Heritable
1.2.1. BMPR2
1.2.2. ALK1, endoglin, SMAD9, C AV1, KC NK3
1.2.3. Unknown
1.3. Drug- and toxin-induced
1.4. Associated with

1.4.1. C onnective tissue diseases
1.4.2. HIV infection
1.4.3. Portal hypertension
1.4.4. C ongenital heart diseases
1.4.5. Schistosomiasis
1′. Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)
1′′. Persistent pulmonary hypertension of the newborn (PPHN)
2. Pulmonary hypertension owing to left heart disease
2.1. Left ventricular systolic dysfunction
2.2. Left ventricular diastolic dysfunction
2.3. Valvular disease
2.4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
3. Pulmonary hypertension owing to lung diseases and/or hypoxia
3.1. Chronic obstructive pulmonary disease
3.2. Interstitial lung disease
3.3. Other pulmonary diseases with mixed restrictive and obstructive pattern
3.4. Sleep-disordered breathing
3.5. Alveolar hypoventilation disorders
3.6. Chronic exposure to high altitude
3.7. Developmental abnormalities
4. Chronic thromboembolic pulmonary hypertension (CTEPH)
5. Pulmonary hypertension with unclear multifactorial mechanisms
5.1. Hematologic disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy
5.2. Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis: lymphangioleiomyomatosis, neurofibromatosis, vasculitis
5.3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
5.4. Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental pulmonary hypertension
ALK1: activin receptor-like kinase type 1; SMAD9: mothers against decapentaplegic 9; CAV1: caveolin1; KKCNK3: potassium channel super family K member-3;
BMPR2: bone morphogenetic protein receptor type 2; HIV: human immunodeficiency virus.
Original figure modified for this publication. Simonneau, G, Gatzoulis, M, Adiata, I, et al. Updated Clinical Classification of Pulmonary Hypertension. J Am Coll Cardiol 2013;
62:S34. Illustration used with the permission of Elsevier Inc. All rights reserved.
Disclosures
Disclosures: Lew is J Rubin, MD Consultant/Advisory Boards: Actelion; United Therapeutics; Gilead; GeNO [pulmonary hypertension
(macitentan, selexipag, treprostinil, beraprost, ambrisentan, nitric oxide)]. Equity Ow nership/Stock Options: GeNO [inhaled NO system].
William Hopkins, MD Speaker's Bureau: Merck [Atherosclerosis]; Actelion [Pulmonary Hypertension (Bosentan, Opsimut, Epoprostenol,
Iloprost)]; Bayer [Pulmonary Hypertension (Riociguat)]. Jess Mandel, MD Nothing to disclose. Geraldine Finlay, MD Employee of
UpToDate, Inc.
Contributor disclosures are review ed for conflicts of interest by the editorial group. When found, these are addressed by vetting through
a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced
content is required of all authors and must conform to UpToDate standards of evidence.
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Clinical Features and Diagnosis of Pulmonary Hypertension in Adults

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29/9/2014 Clinical features and diagnosis of pulmonary hypertension in adults

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Clinical features and diagnosis of pulmonary hypertension in adults

Authors Section Editor Deputy Editor

These include the following:

PASP = (4 x [TRV]2) + RAP

● PH is likely if the PASP is >50 and the TRV is >3.4

● PH is possible with other combinations of findings

● HIV serology to screen for HIV-associated PH

Exercise testing during the diagnostic evaluation of PH serves several purposes:

● Screens for alternative causes of the patient's symptoms

SUMMARY AND RECOMMENDATIONS

● We suggest beginning with an echocardiogram (algorithm 1):

• Mean pulmonary artery pressure is ≥25 mmHg at rest

• Mean pulmonary capillary wedge pressure <15 mmHg

• Venous thromboembolic disease is absent

Use of UpToDate is subject to the Subscription and License Agreement.

Topic 8249 Version 14.0

Algorithm for investigation of suspected pulmonary

PH: pulmonary hypertension; PAH: pulmonary arterial hypertension; ANA:

Graphic 72553 Version 2.0

World Health Organization (WHO) functional classification for pulmonary hypertension

Class WHO functional classification

Graphic 62080 Version 1.0

This plain frontal chest radiograph from a 50-year-old male

Photo courtesy of Jonathan Kruskal, MD.

Graphic 69446 Version 2.0

Normal chest radiograph

Posteroanterior view of a normal chest radiograph.

Courtesy of Carol M Black, MD.

Graphic 65576 Version 1.0

Chest radiograph in lateral view showing decreased retrosternal space

Courtesy of Sven Paulin, MD.

Graphic 72063 Version 2.0

Normal lateral chest radiograph

Courtesy of Steven Weinberger, MD.

Graphic 57909 Version 1.0

Electrocardiogram showing right ventricular hypertrophy

Right ventricular hypertrophy due, in this case, to idiopathic pulmonary

Courtesy of Ary Goldberger, MD.

Graphic 67622 Version 4.0

Normal electrocardiogram showing normal sinus rhythm at a rate of 75 beats/min, a PR

Courtesy of Ary Goldberger, MD.

Graphic 76183 Version 3.0

Right atrial enlargement

Graphic 63181 Version 1.0

Electrocardiograms showing atrial enlargement

Courtesy of Morton Arnsdorf, MD.

Graphic 64928 Version 3.0

Echocardiogram apical 4-chamber with and without

Panel A: Apical four-chamber view from a patient with severe

Graphic 80275 Version 4.0

Echocardiogram short axis showing RV pressure

Graphic 71065 Version 4.0

Representation of echocardiographic findings in

Schematic representation of two-dimensional and Doppler

Adapted from: Otto C. Cardiomyopathies, hypertensive and pulmonary heart

Graphic 77640 Version 4.0

Idiopathic pulmonary arterial hypertension

This angiogram, obtained after injection of contrast material into the

Photo courtesy of Jonathan Kruskal, MD.

Graphic 67815 Version 2.0

Hemodynamic values of normal recumbent adults