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Right Heart

Catheterization for the


Diagnosis of Pulmonary
Hypertension
Controversies and Practical Issues
Michele D’Alto, MD, PhD, FESCa,*,1,
Konstantinos Dimopoulos, MD, MSc, PhDb,
John Gerard Coghlan, MD, FRCPc, Gabor Kovacs, MD, PhDd,
Stephan Rosenkranz, MD, PhDe, Robert Naeije, MD, PhDf

KEYWORDS
 Pulmonary hypertension  Heart catheterization  Hemodynamics

KEY POINTS
 Right heart catheterization (RHC) is the gold standard for the diagnosis and classification of pulmo-
nary hypertension.
 RHC is used to assess the response to therapy specific to pulmonary arterial hypertension and
guide clinical decision-making.
 Significant expertise is required for safely performing an RHC and for the acquisition of reliable and
reproducible information.

BACKGROUND vasoreactivity testing in selected patients.1


Indeed, the definition of PH is based strictly on
Right heart catheterization (RHC) is the gold stan- invasive hemodynamics: mean pulmonary arterial
dard for assessing pulmonary hemodynamics and pressure (mPAP) greater than or equal to 25 mm
is mandatory for confirming the diagnosis of pul- Hg by RHC measured at rest. Considering that
monary hypertension (PH), assessing the severity the upper limit of normal for mPAP is 20 mmHg,
of hemodynamic impairment, and performing it is still debated the meaning and the clinical

No grant support or any potential conflicts of interest, including related consultancies, shareholdings and
funding grants.
a
Department of Cardiology, Second University of Naples, Monaldi Hospital, piazzale E. Ruggieri, Naples
80131, Italy; b Department of Cardiology, Adult Congenital Heart Centre, Royal Brompton Hospital, Imperial
College, Sidney Street, London SW3 6NP, UK; c Department of Cardiology, Royal Free Hospital, Pond Street,
London NW3 2QG, UK; d Department of Internal Medicine, Medical University of Graz, Ludwig Boltzmann
heartfailure.theclinics.com

Institute for Lung Vascular Research Graz, Stiftingtalstrasse 24, Graz 8010, Austria; e Department of Cardiology
and Cologne Cardiovascular Research Center (CCRC), Heart Center, University of Cologne, Kerpener Street. 62,
Köln 50937, Germany; f Department of Cardiology, Erasme University Hospital, University of Brussels, Route de
Lennik 808, Brussels 1070, Belgium
1
This author takes responsibility for all aspects of the reliability and freedom from bias of the data presented
and their discussed interpretation.
* Corresponding author. Via Tino di Camaino, 6, Naples 80128, Italy.
E-mail address: mic.dalto@tin.it

Heart Failure Clin 14 (2018) 467–477


https://doi.org/10.1016/j.hfc.2018.03.011
1551-7136/18/Ó 2018 Elsevier Inc. All rights reserved.
468 D’Alto et al

implication of values between 21 and 24 mmHg. catheterization (eg, for obtaining left ventricular
Precapillary PH is defined as a pulmonary artery end-diastolic pressure [LVEDP] when PAWP is
wedge pressure (PAWP) less than or equal to suboptimal). Complications include hemothorax
15 mm Hg, and postcapillary PH as a PAWP and pneumothorax, which are less likely with an
greater than 15 mm Hg. Among postcapillary PH, ultrasound-guided approach.4 Indeed, although in
isolated postcapillary PH (Ipc-PH) is defined as a most subjects the internal jugular vein is located
diastolic pulmonary gradient (DPG) less than lateral to the carotid artery, there is a high degree
7 mm Hg and/or a pulmonary vascular resistance of variability. In 22.5% of patients the internal jugu-
(PVR) less than or equal to 3 Wood units (WU); lar vein may be anterior and in 5.5% it is medial to
combined postcapillary and precapillary PH the carotid artery.6 An arm approach (cephalic or
(Cpc-PH) is defined as a DPG greater than or equal basilica vein) is often preferred by patients because
to 7 mm Hg and/or a PVR greater than 3 WU.1 the procedure is similar to venous cannulation,
Nevertheless, the role of DPG remain controver- even though ultrasound guidance is often required
sial. Pulmonary arterial hypertension (PAH) is char- to access deeper veins. A (hydrophilic) guidewire
acterized by the presence of precapillary PH and may be needed to navigate the cephalic-
PVR greater than 3 WU, in the absence of other subclavian junction, which may be tortuous.
causes of precapillary PH, such as PH due to
lung diseases, or chronic thromboembolic PH.1
Accurate classification of PH patients is essen- CATHETERS USED FOR RIGHT HEART
tial for their management and can only be CATHETERIZATION
achieved by invasive means. The interpretation
of invasive hemodynamics should always take The gold standard for pressure and pulmonary
into consideration the clinical picture and imaging blood flow measurement is the high-fidelity micro-
findings.1 RHC can be a challenging procedure in manometer-tipped catheter and the direct Fick
PH patients and requires expertise, attention to method, respectively. Currently, fluid-filled, flow-
detail, and meticulous collection of data. To obtain directed thermodilution catheters are widely used,
accurate and reproducible information and mini- albeit with some error.7–13 The Swan-Ganz
mize the risks related to the procedure, RHC balloon-tipped floatation catheter is an end-hole
should be limited to specialist centers and opera- catheter, which may have an additional lumen ter-
tors with training and expertise in this specific pro- minating in a proximal side port and a thermistor
cedure and condition.2 (temperature monitor) at the tip for calculating car-
diac output (CO) by the thermodilution method.14
VASCULAR ACCESS The use of the Swan-Ganz catheter expanded
rapidly in the 1970s in critically ill and high-risk sur-
Although any systemic large vein may be used for gery patients but declined thereafter when random-
venous access when performing RHC,3–5 the ized controlled trials failed to demonstrate a benefit
femoral and internal jugular veins are most from its use, showing an increase in complica-
commonly used in clinical practice. The cephalic tions.15,16 Current indications include cardiogenic
or basilic vein is preferred in some centers and is shock and the diagnosis and follow-up of PH
particularly helpful in patients who are dyspneic at (Box 1).17
rest (eg, those with severe respiratory disease) In few patients with severe PH, advancing the
and do not tolerate the supine position. The femoral Swan-Ganz catheter to the pulmonary artery and
access is easily compressible and allows access PAWP position may be challenging because of
through a large patent foramen ovale (PFO) to marked dilatation of right heart chambers and ves-
obtain direct pulmonary venous and left atrial mea- sels, or the presence of severe tricuspid or pulmo-
surements. Moreover, left heart catheterization can nary valve regurgitation. Several tricks can be used
be performed simultaneously. Disadvantages to overcome such difficulties (eg, use of standard
include the need for fluoroscopy, difficulties in of hydrophilic guidewires, coiling the catheter in
reaching the pulmonary artery, and the need for the right atrium). Other catheters can be used to
bedrest postprocedure. Vascular access complica- access the pulmonary arteries but are not able to
tions (eg, pseudoaneurysm, arteriovenous fistula, provide a reliable PAWP (Table 1). The Berman
or retroperitoneal bleeding) are more likely when catheter, a balloon-tipped, blind-end angiographic
arterial and venous access are both obtained. catheter, has several holes proximal (or distal) to
The internal jugular vein allows easy access to the balloon, and does not allow thermodilution or
the pulmonary artery, often not requiring imaging. PAWP measurements. Non–flow-directed cathe-
Crossing PFOs through this access is not easy ters, such as the multipurpose or pigtail catheter,
and alternative access is required for left heart may be used to access the pulmonary arteries
Heart Catheterization for Pulmonary Hypertension 469

Box 1 located, assuming there is no obstruction or signif-


Current indications for use of the Swan-Ganz icant flow within the catheter.20,21 The specific
catheter gravities for blood and mercury are 1.055 and
13.6, respectively; therefore, a blood column of
 Cardiogenic shock during supportive therapy 1 cm is equivalent to a mercury column of
 Discordant right and left ventricular failure 0.78 mm. Hence, a zero level set 5 cm above (or
 Severe chronic heart failure requiring below) the midthoracic level results in underesti-
inotropic, vasopressor, and vasodilator mation (or overestimation) of all pressures by
therapy approximately 4 mm Hg. This may significantly
affect management of patients with borderline
 Suspected pseudosepsis (high CO, low sys-
temic vascular resistance, elevated right atrial hemodynamics.
and pulmonary capillary wedge pressures) An ideal zero reference should be independent
of chest diameter and insensitive to changes in
 Potentially reversible systolic heart failure,
body position: this defines the hydrostatic indiffer-
such as fulminant myocarditis and peripar-
tum cardiomyopathy ence point, that is, the location in the circulatory
system where changes in body position do not
 Differential diagnosis of PH affect pressure measurements.20–23 Guyton and
 Assessment of the effects of therapy in pa- Greganti20 suggested that the level of the tricuspid
tients with precapillary and mixed types of PH valve was the most anatomically adequate zero
 Workup for cardiac, lung, and liver transplan- reference point. Right atrial pressure represents
tation (including hepatic wedge pressure) the outflow pressure of the systemic venous return
and the inflow pressure of the heart and is, thus,
Adapted from Chatterjee K. The Swan-Ganz catheters:
past, present, and future. A viewpoint. Circulation regulated by the coupling of these 2 systems. As
2009;119:151; with permission. such, it should remain stable with changes in
body position. As a consequence, for many years,
it was recommended that the zero level should be
set at the level of the right atrium, or of the
but cannot provide accurate PAWP measurements tricuspid valve, or 5 cm below the anterior chest
unless exchanged for a Swan-Ganz catheter. surface.
Recently, the intersection of the midthoracic
CALIBRATION AND ZEROING frontal plane with the transverse plane passing
through the fourth anterior intercostal space and
Fluid-filled catheters require static and dynamic the midsagittal plane was suggested as the stan-
calibration. Static calibration is through standard dardized zero reference point (Fig. 1).12 However,
zero leveling, followed by raising the catheter tip Kovacs and colleagues24 found that the center of
10 cm and measuring 1 kPa. Dynamic calibration the left atrium was best described by the mid-
is practically limited to the fast flush test18,19 to thoracic level. Given that left atrial pressure
detect underdamping or overdamping. Too slow a (or PAWP) is the most pivotal measure in RHC
decay of pressure after sudden interruption of flush- for PH (differentiating between precapillary and
ing indicates overdamping due to insufficient flush- postcapillary PH), current consensus is that the
ing and/or excessively long tubing. Persistent midthoracic level should be used as zero reference
spiking oscillations in the pressure trace indicate point. Whether this anatomic landmark correctly
underdamping, which can be controlled by insertion reflects the mid-left atrium in nonsupine patients
of a small bubble in the tubing system. The fre- remains to be confirmed.
quency response of the Swan-Ganz catheter
(<12 Hz) is, in theory, insufficient for assessing rapid PRESSURE MEASUREMENTS
changes in intravascular pressures; however, this is
acceptable for waveform analysis in clinical prac- Essential measurements and calculations per-
tice. Most monitoring systems currently filter at formed during RHC are reported in Box 2. This
8 Hz to correct for underdamping but may introduce article focuses on PAWP, which is among the
error through overdamping of pressure signals. most important, yet controversial, hemodynamic
A wrong zero level set is among the most com- parameters in PH.
mon mistakes and a major confounding factor dur-
Pulmonary Artery Wedge Pressure
ing RHC. All pressure measurements during RHC
are the difference between the pressure at the Accurate measurement of left atrial pressure is
chosen zero level and the pressure within the car- essential for distinguishing precapillary from post-
diac chamber or vessel where the catheter tip is capillary PH, hence identifying patients with left
470 D’Alto et al

Table 1
Catheters for heart catheterization

Type of Catheter Balloon-Tipped End-Hole Thermodilution Possible


Swan-Ganz U U U

Berman U  

Multipurpose  U 

Pigtail  U 

U, yes; , no.

heart disease (eg, heart failure with preserved there is often a prominent swing in intrathoracic
ejection fraction [HFpEF]) who should not receive pressure affecting intracardiac pressures.12,27 In
PAH therapies.25 PAWP is commonly used as a such patients, pressures should probably be aver-
surrogate of left atrial pressure, whereas LVEDP aged over the entire respiratory cycle.1 Moreover,
is used when an accurate PAWP cannot be ob- a retrospective study28 involving 329 subjects un-
tained.26 The PAWP is obtained using a Swan- dergoing right heart catheterization for suspected
Ganz catheter with the balloon inflated in a branch PH showed that a significant proportion of the sub-
pulmonary artery, preventing blood flow or the jects with precapillary PH phenotype had end-
transmission of pressure from the proximal pulmo- expiratory PAWP greater than 15 mm Hg. These
nary arteries. The static column of blood transmits data support the conclusion that PAWP averaged
left atrial pressure to the catheter tip, providing a throughout respiration may be a more accurate
reliable estimate of left atrial pressure (Fig. 2A). measurement.
The European Society of Cardiology (ESC) and Achieving a safe and stable balloon occlusion
the European Respiratory Society (ERS) guidelines position may be difficult and requires expertise
recommend that all pressures are recorded as the to avoid underwedging or overwedging (Figs. 2
mean of 3 to 5 measurements obtained at the end and 3). The catheter should be advanced to the
of normal expiration (avoiding breath holding or wedge position, with the balloon partially or fully
Valsalva maneuver).1 There are, however, situa- inflated to allow the catheter to find a suitable
tions in which this recommendation may not be vessel. Inflation of the balloon while the catheter
applicable; for example, patients with chronic is positioned distally should be avoided because
obstructive pulmonary disease (COPD), in whom rupture of a small pulmonary vessel can lead to
Heart Catheterization for Pulmonary Hypertension 471

Box 2
Essential measurements and calculations of
invasive hemodynamic parameters by right
heart catheterization.

Measurement
 Heart rate
 Systemic blood pressure
 Right atrial pressure
 Right ventricular systolic and end-diastolic
pressures
 Systolic, diastolic, and mPAP
 Mean PAWP and PAWP V wave
 O2 saturations in superior vena cava (high
and low), inferior vena cava, right atrium,
right ventricle, pulmonary artery, systemic ar-
tery, left atrium and pulmonary veins (when
Fig. 1. Phlebostatic axis (red line) introduced by possible), and PO2 if the patient is on supple-
Winsor and Burch23: an axis running through the tho- mental O2 with a fraction of inspired oxygen
rax at the junction of a transverse plane (green) pass- (FiO2) greater than 0.30
ing through the fourth anterior intercostal space with  Thermodilution or Fick CO (preferably direct)
a frontal plane (blue) passing midway between the
posterior surface of the body and the base of the xi- Calculations
phoid process of the sternum. Suggested reference  CO or index
point (red point) defined by the intersection of the
 Transpulmonary pressure gradient
frontal plane (blue) at the midthoracic level, the
transverse plane (green) at the level of the fourth  Pulmonary vascular resistance or index
anterior intercostal space, and the midsagittal plane  DPG
(yellow). (Adapted from Kovacs G, Avian A, Pienn M,
et al. Reading pulmonary vascular pressure tracings.  Response to acute vasodilator
How to handle the problems of zero leveling and
respiratory swings. Am J Respir Crit Care Med
2014;190:253; Reprinted with permission of the Amer- LVEDP.25 A gradient between PAWP and LVEDP of
ican Thoracic Society. Copyright Ó 2017 American 3 to 4 mm Hg was previously reported by a
Thoracic Society.) large-scale study on 3926 subjects,10 and has been
built into the recommended cutoff values for the
catastrophic lung hemorrhage. Methods for diagnosis of diastolic heart failure.29
ensuring that an accurate PAWP is obtained The interpretation of PAWP should occur within
include (1) PAWP should be equal to or lower the clinical context and in accordance with noninva-
than diastolic pulmonary arterial pressure (PAP), sive information; for example, a significantly
(2) the PAWP waveform must exhibit clear A and enlarged left atrium on echocardiography is unlikely
V waves, (3) a respiratory swing should be visible, to be associated with a low PAWP, unless the pa-
(4) the catheter tip position must be stable on fluo- tients is heavily diuresed.1 Indeed, PAWP is not a
roscopy, (5) blood sampling from the distal lumen constant value but a dynamic parameter affected
of the catheter should detect an oxygen (O2) satu- by factors such as afterload and fluid balance. In
ration in the occlusion position similar or higher many patients with left heart disease, PAWP can
than the systemic saturations,26 and (6) hold-up be lowered to less than 15 mm Hg with intense
of contrast in the distal circulation should occur. diuretic treatment or fluid restriction.30 In the Regis-
However, forceful aspiration or injection of try to Evaluate Early and Long-term Pulmonary Arte-
contrast or saline through the end-hole in the oc- rial Hypertension Disease Management (REVEAL)
clusion position should be avoided. database, 10% of patients with an initial PAWP of
When PAWP seems unreliable, or there is discrep- 12 mm Hg had a follow-up PAWP of 16 mm Hg,
ancy with clinical and imaging data, an LVEDP should whereas 50% of patients with an initial PAWP of
be obtained.1 End-expiratory PAWP is almost iden- 16 mm Hg had a follow-up PAWP of 12 mm Hg.31
tical to end-expiratory LVEDP in PH patients, Unfortunately, we still lack a robust definition of
whereas the electronic mean PAWP throughout the optimal fluid status. RHC should, ideally, be per-
respiratory cycle underestimates end-expiratory formed in well-compensated patients.
472 D’Alto et al

Fig. 2. Different inflated balloon positions and PAWP tracing. (A) Correct position. (B–D) Incorrect positioning,
causing overestimation of PAWP due to partial occlusion of the lumen from insufficient balloon inflation (B),
due to contact between the catheter tip and the balloon from excessive balloon inflation (C), or contact between
the the catheter tip and the vessel (D).

The optimal PAWP threshold for distinguishing standardization is necessary before fluid challenge
precapillary from postcapillary PH remains a matter or exercise hemodynamics can be used in routine
of debate. Although current guidelines recommend clinical practice.
a threshold of 15 mm Hg,1 values between 12 and
18 mm Hg have also been suggested.29,32 In the CARDIAC OUTPUT
REVEAL registry,32 a cutoff of 18 mm Hg was
used to avoid misclassifying older patients with In clinical practice, CO is frequently assessed by
PAH or patients with pulmonary vascular disease either the indirect Fick method or thermodilution.
and a minor degree of left ventricular diastolic In terms of accuracy and reproducibility, the direct
dysfunction. Such a high cutoff risks misclassifying Fick method is preferable to thermodilution, which
HFpEF patients as PAH, whereas a lower PAWP in turn is preferable to the indirect Fick method.
threshold (eg, 12 mm Hg) carries the opposite risk.33 The Fick principle is based on the observation
Exercise and volume challenge have been that the total uptake (or release) of a substance
proposed as tools for uncovering latent HFpEF in by the peripheral tissues is equal to the product
patients with borderline hemodynamics.34–38 of blood flow to the peripheral tissues and the
Recently, D’Alto and colleagues38 investigated arterial-venous concentration difference (gradient)
the clinical relevance of a fluid challenge systemat- of the substance. When determining CO, the
ically performed during standard RHC in 212 substance most commonly measured is O2.
consecutive subjects referred for evaluation of Pulmonary blood flow and, hence, CO in
PH. A cutoff value of 18 mm Hg after fluid loading, patients without intracardiac shunts is calculated
in addition to clinical and echocardiographic measuring O2 consumption per unit time (VO2)
parameters, allowed reclassification of 6% to 8% and arteriovenous O2 difference (difference of
of subjects with precapillary PH or normal hemo- O2 content in the pulmonary veins and the pulmo-
dynamics at baseline. Further validation and nary artery):
Heart Catheterization for Pulmonary Hypertension 473

Fig. 3. Example of overwedging due to balloon overinflation. The PAWP must be equal or lower than diastolic
pulmonary pressure. PAWP is not reliable because it is similar to systolic pulmonary pressure.

VO2 Thermodilution is based on the same principles


CO 5
arteriovenous O2 difference as dye dilution: a known quantity of an indicator is
injected into the blood circulation; blood flow and
In the absence of intracardiac right-to-left blood volume are calculated by measuring the
shunting, sampling of peripheral arterial blood is concentration of the indicator downstream at a
a surrogate for pulmonary venous saturations. In distal arterial site. A bolus of saline colder than
patients who are on supplemental O2 (fraction the patient’s blood (10 mL at 4 C) is injected into
of inspired oxygen [FiO2] >0.30), PO2 (blood gas) the proximal port of a pulmonary artery catheter
should also be measured to account for diluted O2. located in the right atrium. The cold saline mixes
Determination of O2 uptake is more complex. with the blood and reaches the pulmonary artery
Ideally, VO2 should be measured either by col- where a thermistor located at the tip of the cath-
lecting the patient’s exhaled air over several mi- eter records the change in blood temperature.
nutes (Douglas mask), or using a mouthpiece The area under the curve and average rate of
and integrating ventilation and O2 content differ- change in temperature is converted into an esti-
ence between inspired and expired air (direct mate of CO. Thermodilution is quite reliable, even
Fick method). In most centers, an assumed in patients with very low CO and/or severe
value for VO2 is taken from nomograms (indirect tricuspid regurgitation32,40; it should be avoided
Fick).39
474 D’Alto et al

in patients with intracardiac shunts. It is recom- a DPG greater than or equal to 7 mm Hg and a
mended to average 3 to 5 thermodilution CO mea- PVR greater than 3 WU is a more robust definition
surements with less than 10% variability. of Cpc-PH.42,43 PVR is also particularly important
in patients with intracardiac shunts, in whom an in-
PULMONARY VASCULAR RESISTANCE crease in PAP may be a combination of pulmonary
vascular disease and raised pulmonary blood flow
PVR is essential for the diagnosis of PAH and for (see later discussion).
prognostication.1 It is calculated as the ratio of
the transpulmonary gradient (mPAP minus left
atrial or PAWP) and pulmonary blood flow (CO in ASSESSMENT OF CONGENITAL SHUNTS
patients without shunts) (Box 3). Together with The first step during cardiac catheterization in
the DPG (the difference between diastolic PAP congenital heart disease (CHD) is serial oximetry
and PAWP), PVR is particularly useful in identifying in the systemic veins (pulmonary veins and sys-
patients in group 2 PH, who may suffer from Cpc- temic arteries), the right (and left) heart, and the
PH.41 The threshold of 7 mm Hg for DPG and 3 WU pulmonary circulation. This should include mea-
for PVR are suggested by current ESC-ERS guide- surements in the superior vena cava and inferior
lines for differentiating Cpc-PH from Ipc-PH.1 It vena cava to assess for partial anomalous pulmo-
has been suggested that the combination of both nary venous return or sinus venosus atrial septal
defects. Combined right and left cardiac catheter-
ization is often performed in CHD patients to ac-
Box 3 quire systemic pressures and saturations and to
Useful formulas for the assessment of calculate the ratio between pulmonary and sys-
pulmonary vascular hemodynamics temic artery pressures and resistances, especially
if shunt closure is contemplated.9
Qpulmonic 5 VO2/[O2capacity  (PVsat – PAsat)/100] Blood samples for shunt calculation are prefer-
Qsystemic 5 VO2/[O2capacity  (Aosat – MVsat)/100] ably taken in the resting state without supple-
Qeffective 5 VO2/[O2capacity  (PVsat – MVsat)/100] mental O2 because a raised mixed venous
saturation makes shunt detection challenging. In
Qpulmonic: blood flow over the pulmonary
physiologically significant left-to-right shunts, a
circulation
greater than 7% step-up in O2 saturation can be
Qsystemic: blood flow over the systemic observed from 1 chamber to the next. Accurate
circulation estimation of mixed venous saturations is impor-
Qeffective: nonshunted flow carried from sys- tant, especially in atrial septal defects. At rest,
temic to pulmonic capillary beds most of the CO is received by the brain and the
O2capacity of 1 g of hemoglobin (Hb) 5 1.34 mL heart, thus mixed venous O2 saturation may be
O2capacity: O2 carrying capacity 5 Hb calculated as follows:
(g/dL)  saturated O2  1.34 1 0.003 (PaO2) Mixed venous O2 saturation
PVsat: pulmonary venous saturation
PAsat: pulmonary arterial saturation 3  SVC SatO2 11  IVC SatO2
5
Aosat: aortic saturation
4

MVsat: mixed venous saturation where SVC is superior vena cava and IVC is infe-
rior vena cava. Right-to-left shunting is detected
Shunt flow in 1 minute as a step-down in O2 saturations between the
Right-to-left shunt 5 Qsystemic – Qeffective pulmonary veins (accessible through an intracar-
Left-to-right shunt 5 Qpulmonic – Qeffective diac defect or PFO) and the left heart chambers.
This can also be encountered in PH patients
Shunt fractions without CHD who shunt through a PFO. If a pul-
Qpulmonic/Qsystemic (Qp/Qs) 5 (Aosat – MVsat)/ monary vein cannot be sampled, pulmonary
(PVsat – PAsat) vein saturation may be estimated as 96% to
Pulmonic shunt fraction (the fraction of 97% in room air in the absence of airways dis-
pulmonic flow due to left-to-right ease (eg, severe COPD). Accurate estimation of
shunting) 5 (PAsat – MVsat)/(PVsat – MVsat) pulmonary and systemic blood flow and resis-
Systemic shunt fraction (the fraction of systemic
tances is essential for the management of pa-
flow due to right-to-left shunting) 5 (PVsat – tients with PAH related to CHD, who should be
Aosat)/(PVsat – MVsat) assessed in centers with expertise in both PH
and adult CHD.
Heart Catheterization for Pulmonary Hypertension 475

VASOREACTIVITY TESTING
Table 2
Pulmonary vasoreactivity testing is aimed at iden- Possible complications related to right heart
tifying patients with idiopathic, heritable, or drug- catheterization
induced PAH who may respond to high-dose cal-
Related to Hematoma at the puncture
cium channel blockers. In other PH patients, this venous site
test is not indicated because the likelihood of a access Vagal reaction with
sustained response to calcium channel blockers bradycardia and
is extremely low. Vasoreactivity testing should be hypotension
performed at the time of the first RHC. A positive Pneumothorax
acute response is defined as a reduction in Arteriovenous fistula
mPAP greater than or equal to 10 mm Hg, reaching Puncture of the carotid,
an absolute mPAP less than or equal to 40 mm Hg, femoral, or brachial artery
with an increased or unchanged CO. Approxi- Hypertensive crisis during
puncture
mately only 5% to 10% of patients with idiopathic,
Phrenic nerve injury
heritable, or drug-induced PAH meet these Brachial plexus injury
criteria. Vasoreactivity is used in CHD for different Air embolism
indications (assessment of operability) with a Chylothorax
different definition of response. Hemothorax
Related to Rupture of pulmonary vessel
RHC or perforation of cardiac
COMPLICATIONS chamber wall
Supraventricular tachycardia
In patients with PH, RHC has been associated with
Ventricular tachycardia
serious, potentially fatal complications (Table 2), Vagal reaction with
especially in older studies from the 1980s and bradycardia and
1990s.1 In a long-term retrospective follow-up hypotension
study on 120 subjects, Fuster and colleagues44 re- Systemic hypotension
ported a catheterization-related mortality of 4.2%. Transient ischemic attack
In the series of the National Institutes of Health Hypertensive crisis
registry obtained between 1981 and 1985, no fatal Chest pain and hemoptysis or
events were reported during 187 procedures; hemothorax after balloon
however, the rate of major complications related inflation
Pulmonary embolism
to RHC was 5.3%.45 The largest study evaluating
Knots in the catheter
adverse events of RHC in subjects with PH in the complicating removal
modern era was a multicenter survey including Transient right bundle branch
experienced centers from Europe and the United block (risk of complete
States.2 The overall number of serious adverse atrioventricular block if
events in 7218 procedures was 76 (1.1%). The preexistent left bundle
most frequent complications were related to branch block)
venous access (eg, hematoma, vagal reaction Related to Systemic hypotension
with bradycardia and hypotension after the punc- vasoreactivity Bronchospasm during
ture, pneumothorax), followed by arrhythmias testing prostanoid inhalation
and hypotensive episodes related to vasoreactiv- Related to Hypertensive crisis and
ity testing. Almost all of the complications were pulmonary pulmonary edema after
mild to moderate and resolved either spontane- angiography contrast injection
ously or after appropriate intervention. There Second-degree
were 4 fatal events, with a procedure-related mor- atrioventricular
block after dye
tality of 0.055%.
injection
RHC is more technically demanding and risky in Chest pain after dye injection
children. In a retrospective audit in 70 children with Vomiting after dye
PH, resuscitation and death occurred in 4.3% and injection
1.4% of cases, respectively.46 Recent insight from
Adapted from Hoeper MM, Lee SH, Voswinckel R, et al.
the Global Tracking Outcomes and Practice in Pe- Complications of right heart catheterization procedures
diatric Pulmonary Hypertension (TOPP) Registry in patients with pulmonary hypertension in experi-
confirmed a higher complication rate in the pediat- enced centers. J Am Coll Cardiol 2006;48(12):2550;
ric population than in adults. Complications with permission.
occurred in 5.9% of 908 heart catheterization
476 D’Alto et al

procedures, including 5 (0.6%) deaths, and were 10. Halpern SD, Taichman DB. Misclassification of
related to the general anesthesia and a higher pre- pulmonary hypertension due to reliance on pulmo-
procedural functional class.47 nary capillary wedge pressure rather than left ven-
tricular end-diastolic pressure. Chest 2009;136:
37–43.
SUMMARY
11. Rich S, D’Alonzo GE, Dantzker DR, et al. Magnitude
RHC is the gold standard for measuring pulmonary and implications of spontaneous hemodynamic vari-
hemodynamics and is mandatory for establishing ability in primary pulmonary hypertension. Am J Car-
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