D i a g n o s i s an d M a n a g e m e n t

of Pulmonary Hypertension
a n d R i g h t Ven t r i c u l a r Fa i l u re i n
t h e C a rd i o v a s c u l a r In t e n s i v e C a re
Unit
Anika Vaidy, MDa, Oisin O’Corragain, b
MB, BCh, BAO ,
Anjali Vaidya, MDa,*

KEYWORDS
 Pulmonary hypertension  Right ventricular failure  Critical care  Hemodynamics
 Right heart physiology

KEY POINTS
 Pulmonary hypertension encompasses a broad range of causes, associated conditions,
hemodynamic profiles, and complex physiology that often manifest in the critical care
setting.
 Successful diagnosis and management of pulmonary hypertension and right heart failure
rely on clinical integration of physiology and right heart function including echocardiogra-
phy and hemodynamics.
 Right ventricular failure can be a result of varying abnormalities in preload, afterload, and
intrinsic myocardial dysfunction, all of which require distinct management strategies.
 It is crucial to avoid common pitfalls in critical care management, which may include peri-
cardiocentesis, volume loading, and mechanical circulatory support in PAH.

PULMONARY HYPERTENSION AND HEMODYNAMIC ASSESSMENT

Pulmonary hypertension (PH) is categorized into 5 World Health Organization (WHO)

groups (Table 1) and defined by a mean pulmonary artery (PA) pressure greater
than 20 mm Hg. This can be further hemodynamically categorized into 3 distinct en-
tities. Precapillary PH has pulmonary capillary wedge pressure (PCWP) 15 mm Hg
and pulmonary vascular resistance (PVR) > 2 Wood units (WU), and postcapillary
PH has PCWP > 15 mm Hg with PVR  2 WU. Combined postcapillary and

a
Pulmonary Hypertension, Right Heart Failure, CTEPH Program, Division of Cardiology, Temple
University Hospital, 9th floor Parkinson Pavilion, 3401 North Broad Street, Philadelphia, PA
19140, USA; b Pulmonary and Critical Care, Temple University Hospital
* Corresponding author.
E-mail address: Anjali.Vaidya@tuhs.temple.edu

Crit Care Clin 40 (2024) 121–135

https://doi.org/10.1016/j.ccc.2023.05.003 criticalcare.theclinics.com
0749-0704/24/ª 2023 The Authors. Published by Elsevier Inc. This is an open access article under
the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
122 Vaidy et al

Table 1
Classification of pulmonary hypertension

Group 1 (PAH) 1.1. Idiopathic

Group 2 (associated with
left heart disease)
Group 3 (associated with
lung disease and/or
hypoxia)
Group 4 (associated
with pulmonary
artery obstructions)
Group 5 (associated
with unclear and/or
multifactorial
mechanisms)
1.1.1. Nonresponders at vasoreactivity testing
1.1.2. Acute responders at vasoreactivity testing
1.2. Heritable
1.3. Associated with drugs and toxins
1.4. Associated with:
1.4.1. Connective tissue disease
1.4.2. HIV infection
1.4.3. Portal hypertension
1.4.4. Congenital heart disease
1.4.5. Schistosomiasis
1.5. PAH with features of venous/capillary pulmonary
veno-occlusive disease and pulmonary capillary
hemangiomatosis (PVOD/PCH) involvement
1.6. Persistent PH of the newborn
2.1. Heart failure:
2.1.1. With preserved ejection fraction
2.1.2. With reduced or mildly reduced ejection fraction
2.2. Valvular heart disease
2.3. Congenital/acquired cardiovascular
conditions leading to postcapillary PH
3.1. Obstructive lung disease or emphysema
3.2. Restrictive lung disease
3.3. Lung disease with mixed restrictive/obstructive pattern
3.4. Hypoventilation syndromes
3.5. Hypoxia without lung disease (eg, high altitude)
3.6. Developmental lung disorders
4.1. Chronic thrombo-embolic PH
4.2. Other pulmonary artery obstructions
5.1. Hematologic disorders
5.2. Systemic disorders
5.3. Metabolic disorders
5.4. Chronic renal failure with or without hemodialysis
5.5. Pulmonary tumor thrombotic
microangiopathy
5.6. Fibrosing mediastinitis

Adapted from Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis
and treatment of pulmonary hypertension [published correction appears in Eur Heart J. 2023 Apr
17;44(15):1312]. Eur Heart J. 2022;43(38):3618-3731; with permission.

precapillary PH (Cpc-PH) is the third distinct entity in which there are PCWP > 15 mm
Hg and PVR > 2 WU.1,2
Consideration of these hemodynamic phenotypes, combined with physiologic cir-
cumstances, is paramount, particularly in the intensive care setting, where clinical
instability can transiently impact this assessment. Hypoxemia, positive pressure venti-
lation, vasopressors, inotropes, arrhythmia, intubation, and sedation can all signifi-
cantly, and transiently, impact cardiopulmonary hemodynamics.

Right Heart Catheterization

Right heart catheterization (RHC) is the diagnostic gold standard when performed accu-
rately and with avoidance of common pitfalls in data acquisition and interpretation.
 Pulmonary Hypertension and Right Ventricular Failure in Critical Care 123

Level transducer positioning and zeroing to atmospheric pressure should be

confirmed, while obtaining hemodynamics at end expiration and avoiding waveform
dampening. Accurate determination of PCWP is often challenging in precapillary
PH. It is common for the balloon to “under-occlude” in enlarged and poorly compliant
PA branches, thus overestimating the true PCWP, falsely lowering the calculated PVR,
and misdiagnosing the true PH phenotype.3 A PCWP saturation can confirm accurate
measurement, as it should be within 5% of systemic oxygen saturation. Lower values
should prompt repeat attempts to wedge correctly or direct measurement of left ven-
tricular end diastolic pressure.

Echocardiography
Although RHC remains the gold standard for diagnosis of PH, echocardiography has
emerged as a validated tool in PH hemodynamic assessment and can be deployed
noninvasively and quickly in an intensive care unit (ICU) setting before RHC may be
safely or readily available.
In addition to estimated pulmonary arterial systolic pressure (PASP), echocardiog-
raphy can classify the hemodynamic phenotype. For example, right ventricular (RV)
enlargement, low RV base/apex ratio, dysfunction, systolic intraventricular septal flat-
tening, significant tricuspid regurgitation (TR), and a “notched” right ventricular outflow
tract (RVOT) pulse-wave Doppler profile are predictive of PVR elevation rather than
postcapillary PH.4–6
Opotowsky and colleagues7 and Vaidya and colleagues8 have both presented sim-
ple echocardiographic scores that accurately predict PH hemodynamics and help
distinguish precapillary PH from postcapillary PH. Direct review of echo-Doppler im-
ages that reveal normal left atrial (LA) size, low transmitral and tissue Doppler E:e’ ra-
tio, and a “notched” RVOT or reduced acceleration time predicts precapillary PH.
Conversely, when the LA is at least moderately enlarged, E:e’ is elevated, and the
RVOT is not “notched”, this is consistent with postcapillary PH.7 Vaidya and col-
leagues8 validated a simplified virtual echocardiography screening tool based on LA
size, E:e’ ratio, and presence of systolic interventricular septal flattening. This score
proved far more reliable in distinguishing precapillary and postcapillary PH than esti-
mated PASP and is easily derived from information within the standard echocardiog-
raphy report without relying on direct visualization and interpretation of echo-Doppler
images.
Examples of representative precapillary PH and postcapillary PH echo-Doppler im-
ages are seen in Fig. 1. These screening tools should be incorporated into initial PH
assessment in an ICU setting to assist in rapidly triaging unstable patients.

WORLD HEALTH ORGANIZATION GROUP 1

WHO group 1 includes pulmonary arterial hypertension (PAH), defined hemodynami-

cally as precapillary PH. This includes idiopathic, heritable, toxin-induced, and PAH
related to connective tissue disease, portal hypertension, HIV, and congenital heart
disease.1

Diagnosis
Symptoms include exertional dyspnea, fatigue, chest pain, fluid retention, and syncope.
A known associated condition with these symptoms should raise suspicion for PAH.
Physical examination reveals right heart disease manifesting with elevated jugular
venous pressure (JVP), parasternal heave, accentuated second heart sound, TR,
and peripheral edema. Low-volume arterial pulses can indicate low cardiac output.
124 Vaidy et al

Fig. 1. Echo-Doppler findings representing postcapillary PH and precapillary PH high-

lighting differences in right ventricle shape and LA size (A, E), transmitral E:e’ Doppler
pattern (B, F), interventricular septum position in systole (C, G), and RVOT pulse-wave
Doppler shape (D, H).

An electrocardiogram (ECG) often reveals right-axis deviation, right atrial enlarge-

ment, right heart strain, or right ventricular hypertrophy (RVH). Chest radiograph
may identify right atrial and RV enlargement, and prominent pulmonary arteries.
As described, echocardiography and RHC should be used together to diagnose and
assess the severity of the PH syndrome.
Management
Risk stratification should occur at the time of diagnosis and serially thereafter.1 This
includes evaluation of functional class, exercise capacity with 6-minute walk distance
(6MWD), clinical right heart failure, B-type natriuretic peptide, echocardiography, right
atrial pressure, and cardiac index.
The most important concept in PAH treatment, in the ICU or otherwise, is that of RV-
PA coupling, with the goal to medically decrease the PVR to a point where the right
heart can function near normally.5 Recent work by D’Alto and colleagues5 describes
right heart reverse remodeling, reductions in RV size, and normalization of RV systolic
function, all closely linked to improved WHO functional status, exercise capacity, and
survival.
Prostacyclins, phosphodiesterase 5 inhibitors (PDE5i), endothelin receptor antago-
nists, and soluble guanylate cyclase stimulators comprise the 4 classes of PAH med-
ical therapies, each with distinct nuances regarding titration and side-effect profiles,
warranting PH expertise when used. Recent guidelines1 recommend referral to PH
accredited centers for care and treating most PAH with upfront dual therapy and addi-
tion of prostacyclin if unable to achieve a low- or intermediate-level risk. When medical
therapy is maximized, lung transplantation should be considered.

WORLD HEALTH ORGANIZATION GROUP 2

WHO group 2 PH includes isolated postcapillary PH (Ipc-PH) from left heart disease
(LHD) from systolic heart failure, valvular disease, or heart failure with preserved
 Pulmonary Hypertension and Right Ventricular Failure in Critical Care 125

ejection fraction and accounts for 65% to 80% of PH. Among patients with LHD, the
presence of PH is associated with high mortality.
Cpc-PH, includes which PVR elevation has implications on RV size and function, is
associated with a worse prognosis than Ipc-PH.9

Diagnosis
Suspicion for PH should be heightened based on orthopnea and known associated
left heart conditions. Physical examination may demonstrate pulmonary rales, left-
sided gallops or murmurs, an abnormal apical impulse, and a prolonged elevation in
systolic blood pressure during Valsalva.
RHC, performed accurately as described above, should be incorporated with the
patient’s clinical features and echocardiographic findings to make a final diagnosis
of PH-LHD.

Management
Optimizing the underlying LHD is the mainstay of WHO group 2 PH management,
including guideline-recommended treatments with diuretics, systemic afterload
reduction, neurohormonal antagonists as indicated, and consideration for advanced
left heart failure management strategies.
PH medical therapy increasing flow to a dysfunctional left heart can cause pulmo-
nary edema, which can be dangerous. The FIRST trial10 demonstrated increased mor-
tality with epoprostenol in PH-LHD from systolic heart failure.
In select patients with severe RV dysfunction in one study of Cpc-PH with
preserved ejection fraction, sildenafil improved PVR, cardiac output, peak
oxygen consumption, 6MWD, and reduced heart failure hospitalizations.11
However, the long-term benefits remain unknown and have yet to be studied in a
randomized controlled trial, and although guidelines recommend against PAH
therapy in Ipc-PH, they do not recommend for or against the use of PDE5is in
Cpc-PH.1

WORLD HEALTH ORGANIZATION GROUP 3

Group 3 encompasses PH in restrictive and obstructive lung disease, hypoventilation

syndromes, sleep disorders, hypoxia from high altitude, and lung developmental dis-
orders.1 This is a precapillary phenotype, although Cpc-PH may occur given overlap-
ping lung and LHD risk factors.
Hypoxia and acidosis stimulate pulmonary vasoconstriction and hyperviscosity;
airway destruction involves the vascular bed. Hyperinflation causes direct
vascular compression, and sympathetic activation predisposes to volume retention.

Diagnosis
Digital clubbing, “barrel chest,” facial plethora, and cyanosis may be seen. Patients
with PAH typically have modest hypoxia; thus, the need for significant oxygen supple-
mentation should raise suspicion for lung disease or right-to-left shunting. Although
positive pressure ventilation augments JVP, this should not discourage a thorough ex-
amination in the ICU.
Computed tomography (CT) characterizes lung disease and may reveal RVH
or an enlarged PA. Mosaic attenuation on CT can indicate air trapping or hypoper-
fusion (in the setting of chronic thromboembolic disease or long-standing
arteriopathy).
126 Vaidy et al

Management
It is important in the ICU to distinguish acute RV dilation and dysfunction (from
acute hypoxia and hypercapnia causing pulmonary vasoconstriction, vascular oc-
clusion during critical illness, or low tidal volumes or overdistention in mechanical
ventilation contributing to RV afterload) from a longer-standing, afterload-depen-
dent PH syndrome. Acute respiratory distress syndrome (ARDS) may be consid-
ered an archetype of acute RV dysfunction, seen in greater than 20% of
patients,12 and management should focus primarily on the underlying cause.
Inhaled nitric oxide and epoprostenol have been studied in ARDS without
improvement.13,14
The use of PH therapy in chronic lung disease can worsen hypoxia via worsening
ventilation-perfusion (V/Q) matching, although those with severe right heart failure
may be considered.15 Patients with a low cardiac output may experience an improve-
ment in oxygen delivery, which outweighs any changes in V/Q relationships or preex-
isting diffusion impairments. PH therapy in group 3 PH has been studied with mixed
results; however, many previous studies included patients without PH or mild
disease.16,17

WORLD HEALTH ORGANIZATION GROUP 4

PH owing to PA obstruction is typically from chronic thromboembolic pulmonary hy-

pertension (CTEPH), but may also include sarcoma or other malignancies, such as PA
stenosis, vasculitis, and fibrosing mediastinitis.1,18

Diagnosis
History may include prior venous thromboembolism, hypercoagulable disorders, ma-
lignancy, red blood cell dyscrasias, splenectomy, long-term central venous catheter
(CVC) or pacemaker, and pelvic vein compression from uterine fibroids or May-
Thurner anatomy.19,20 Physical examination findings may reveal pulmonary bruits
and skin findings in keeping with postthrombotic syndrome.
V/Q scintigraphy is most sensitive for CTEPH.21 CT angiography anatomically
delineates thromboembolic disease, revealing lining clot with occlusive defects,
webbing, poststenotic dilations, systemic to pulmonary collateralization, mosaic
attenuation, subpleural wedge, or curvilinear defects representing infarction.22
A falsely high PCWP may result from the inability to reach a wedged position owing
to thromboembolic disease. Catheter-directed digital subtraction pulmonary angiog-
raphy further characterizes thromboembolic disease. Venography is also indicated for
patients for whom there is a suspicion of pelvic or lower-extremity venous
compression.22

Management
Treatment of CTEPH includes pulmonary thromboendarterectomy, balloon pulmonary
angioplasty, and medical management, as determined by an experienced and expert
multidisciplinary CTEPH team.

WORLD HEALTH ORGANIZATION GROUP 5

Group 5 disease includes multifactorial PH,1 including glycogen and lysosomal

storage, thyroid, and hematologic disorders, sarcoidosis, and Langerhans cell his-
tiocytosis. They may display precapillary, postcapillary, or Cpc-PH via diverse
mechanisms.
 Pulmonary Hypertension and Right Ventricular Failure in Critical Care 127

Management
Treatment of patients with group 5 disease should focus on maintenance of euvolemia
and optimizing the underlying disease. Improved PASP has been reported in patients
treated for hyperthyroidism.23 Exchange transfusions may improve PH associated
with SCD.24 Patients with arteriovenous dialysis access with an increased cardiac
output may be considered for access ligation.25
There are no Food and Drug Administration–approved medications for group 5 PH.

RIGHT VENTRICULAR FAILURE IN THE INTENSIVE CARE UNIT

Causes of RV failure in the ICU can be categorized into 3 main physiologic

perturbations: afterload, inadequate myocardial contractility, and preload. Although
all 3 parameters may be targeted for therapy, they should be emphasized
according to which of these serves as the mechanistic culprit for each case of
RV failure.
Managing RV failure related to PH should emphasize optimizing right-sided
filling pressures and decreasing RV afterload. In addition, immediate restoration
of sinus rhythm is paramount in the context of atrial arrhythmia, as the contribu-
tion of atrial systole comprises up to one-half of overall right heart function in
PAH.26

Right Ventricular Afterload

Elevated RV afterload (PVR) is increasingly the dominant culprit for RV failure in the
ICU, as is the case in most forms of PH, including WHO groups 1, 3, and 4. Elevated
PVR results in increased RV wall stress, leading to cardiac myocyte hypertrophy,
increased wall thickness, and augmented contractility to maintain stroke volume.
With disease progression, however, the RV eventually dilates and loses function
such that it “uncouples” from the pulmonary circulation, unable to match the degree
of RV afterload, leading to right heart failure.
Management should include minimizing common physiologic insults of critical
illness in the ICU that will increase pulmonary vascular constriction. These include cor-
recting hypoxia, hypercapnia, and maintaining lung volumes that are near functional
residual capacity, for this is when PVR is at its lowest.27,28
When pulmonary vascular disease is significant, RV dysfunction and failure often
persist despite optimizing these factors, and administration of PH medical therapy
may be used. Although specific data in the ICU setting are limited for these therapies,
their use may be warranted under the care of PH expertise familiar with the nuances
of each medication. For example, in the context of intrinsic lung disease and hypox-
ia, V/Q mismatch may be induced. If a patient has a low systemic vascular resis-
tance, the use of systemic prostacyclin therapy, the soluble guanylate cyclase
stimulator riociguat, or PDE5i medications may further lower blood pressure. Paren-
teral prostacyclin therapy may also increase platelet dysfunction and consumption,
thus leading to bleeding complications in the ICU. If a patient is on oral treprostinil at
home, the requirement that it be given with meals may necessitate transition to
parenteral use in the ICU. Inhaled epoprostenol and nitric oxide have a rapid onset
of action with a short half-life, making them a suitable option in the critical care
setting. Endothelin receptor antagonists cannot be crushed or split, limiting use
via enteral feeding tubes in the ICU; they should also be used cautiously in the
context of liver dysfunction, which may be common in the context of shock in the
ICU.29
128 Vaidy et al

Right Ventricular Preload

The RV is not universally preload dependent. Adequate right-sided filling pressure is
vital in maintaining cardiac output, specifically when intrinsic RV contractility is
impaired. This is generally not the case when RV failure is a result of PH.
Proper fluid management lies at the core of successful management of RV failure
and depends on the mechanism of RV failure. For example, when RV dysfunction is
a result of a right-sided myocardial infarction, volume resuscitation may be warranted,
as RV end-diastolic pressure often needs to be higher than normal (10–14 mm Hg) to
maintain cardiac stroke volume.30
However, when RV failure is a consequence of increased RV afterload (PVR), vol-
ume resuscitation is contraindicated and leads to increased intraventricular septal
shift, impaired LV diastolic filling, worsening cardiac output, hypotension, shock,
and cardiorenal syndrome.31 These cases generally require decongestion with
diuresis, even in the context of cardiorenal syndrome, to unload the failing RV
and interrupt the cascade of right heart failure worsening shock. Therefore, delin-
eating the primary insult in each case should guide tailored therapy to each specific
patient.

Right Ventricular Contractility

The RV with intrinsic loss of contractility is a distinct mechanism of RV failure, in
contrast to the failing RV that is chronically exposed to high afterload (which is intrin-
sically hypercontractile owing to compensatory myocardial mechanisms, including
hypertrophy). Classically, RV failure from loss of intrinsic contractility has been
described as RV myocardial infarction from acute coronary syndrome but may be a
result of an intrinsic cardiomyopathy, such as arrhythmogenic RV cardiomyopathy,
heritable cardiomyopathy, or other causes.
In these cases, management should focus on the underlying disorder, such as
revascularization and restoring coronary perfusion or inotropic support for the RV.
In patients who have refractory loss of RV contractility, with preserved left heart func-
tion and normal RV afterload, right heart mechanical support can be considered.
At times, loss of RV contractility is combined with derangements in preload and
afterload.31 As the RV dilates, there is overstretching of the myocardium that places
the myocytes at a mechanical disadvantage. As mentioned previously, increases in
preload and afterload will increase free wall tension and oxygen demand, worsening
LV filling and cardiac output. In these cases, afterload and preload should be opti-
mized to increase RV contractility.
When medical therapy for RV failure in the ICU fails to correct the disease, mechan-
ical support may be considered as a bridge to recovery or intervention. Venoarterial
extracorporeal membrane oxygenation (ECMO) can be used to stabilize patients
who have refractory elevated RV afterload, such as advanced stage PAH or a massive
pulmonary embolism.32 This can also be successfully used in patients who have RV
failure alongside left heart failure disease.33

INTENSIVE CARE UNIT PEARLS: MYTH BUSTERS AND AVOIDING PITFALLS

Pericardial Effusion in Pulmonary Arterial Hypertension

A pericardial effusion in PAH has long been recognized as a marker of higher clinical
risk and worse prognosis.1 This may result when the severity of right heart failure and
chronic congestion is such that it impairs cardiac venous drainage via the coronary si-
nus into the right atrium, thus leading to a transudative leak into the pericardial space.
 Pulmonary Hypertension and Right Ventricular Failure in Critical Care 129

This pericardial consequence from RV failure is similar in mechanism to peripheral

pitting edema seen from impaired systemic venous return. Thus, the risk in PAH is
related to what the effusion represents physiologically, rather than its impending he-
modynamic consequences, as is usually the case with pericardial effusion.34 A lack
of RV diastolic collapse is a result of RV hypertrophy and right-sided pressures that
exceed the pericardial pressure.
Importantly, although a PAH patient with dyspnea, elevated JVP, tachycardia, and
low cardiac output with a large pericardial effusion may tempt a pericardiocentesis,
multiple reports have demonstrated the risk of such practice. For example, Hemnes
and colleagues35 reported poor outcomes in patients who had large pericardial effu-
sions and underwent pericardial drainage. One patient died within an hour of the peri-
cardiocentesis. Removal of the pericardial effusion can worsen RV dilation and
intraventricular septal flattening, further leading to a sudden and catastrophic
compression of left heart with consequent cardiovascular collapse. Pericardiocente-
sis in the context of severe RV dysfunction with PAH is contraindicated, and the man-
agement should instead focus on treating the PAH (Fig. 2). With PAH therapy and
improvement of right heart performance, the pericardial effusion subsequently also
improves.

Preload Optimization in Right Ventricular Failure

As mentioned above, a common misconception is that RV failure should always be
managed with volume resuscitation. Although this may be true in isolated cases of
intrinsic RV contractility impairment, most RV failure is not preload dependent and
is instead exacerbated by volume overload (Fig. 2). In these patients, increases in pre-
load can further distend the RV and increase wall tension, leading to impaired LV
filling.36 Particularly in severe RV failure, often in conjunction with significant TR, renal
venous congestion impairs renal perfusion pressure, further worsening kidney injury.
In such patients, even if already presenting with some degree of cardiorenal syn-
drome, intravenous loop diuretics should be administered. In refractory cases, use
of ultrafiltration or renal replacement therapy may be warranted.

Fig. 2. Critical care pitfalls to avoid in RV failure.

130 Vaidy et al

Percutaneous Right Ventricular Assist Devices in Right Ventricular Failure

In the contemporary era, there is a growing interest in the use of mechanical support
with right ventricular assist devices (RVAD). The TandemHeart RVAD: LivaNova, Inc.
(London), Impella RP:Abiomed (Danvers, MA), and Protek Duo: LivaNova, Inc. (Lon-
don) are current percutaneous options. It is, however, paramount to recognize the lim-
itation of this type of mechanical circulatory support device in patients with PH and to
understand when this type of treatment strategy will be of benefit.
An RVAD redirects flow from the RV into the PA. Therefore, when RV failure results
from severely elevated RV afterload, such as PAH, this device does not bypass the
true circulatory lesion, and the high PVR will still impede forward flow (Fig. 2).37 In
addition, complications related to large-bore percutaneous access, hematologic
and infectious complications of the indwelling device lend toward deleterious effects
for which the risk-benefit profile generally does not justify such use in critically ill PH
patients in the ICU. Cases of RV dysfunction from contractility impairment, without de-
rangements in RV afterload, best predict a favorable outcome with use of a percuta-
neous RVAD.

Immediate and Continuous Invasive Hemodynamic Monitoring

Although RHC provides a direct and complete hemodynamic profile, and CVCs pro-
vide central venous pressure (CVP) and oximetry (CVO2) to assess therapeutic
response, there are limitations to their immediate and continuous use in RV failure
in the ICU. In profound cardiogenic shock from RV failure, supine positioning for
vascular access and placement of a PA catheter can impair ventilation and worsen
oxygenation. Supine positioning decreases pulmonary function and lung volumes in
patients compared with upright positioning.38,39 There is displacement of the dia-
phragm upward, particularly in patients with obesity or ascites, which further limits
effective ventilation and promotes basilar and posterior atelectasis. Pulmonary blood
flow and dorsal-ventral fluid shifts while supine alters V/Q relationships across West
zones of the lung. Chest compliance may also be worsened in volume overloaded
states owing to chest wall edema. Taken together, when there is minimal cardiopul-
monary reserve, supine positioning for jugular venous access can worsen hypoxia
and acidosis, leading to acute increases in PVR, increased myocardial demand, and
hemodynamic collapse. In addition, acquiring jugular venous access, which may inad-
vertently manipulate the carotid bulb, cause bradycardia, or stimulate a vagal
response, can lead to hemodynamic collapse and circulatory arrest in severe RV fail-
ure with cardiogenic shock.
As such, invasive hemodynamic assessment may need to wait until initial clinical
stabilization is achieved. A careful clinical assessment with history, physical examina-
tion, and echocardiography can provide insight into a patient’s hemodynamic profile
as described previously, helping predict precapillary PH versus postcapillary PH. If se-
vere precapillary PH with RV failure and cardiogenic shock is clearly characterized by
history, examination, and echo-Doppler findings, it may be appropriate to initially sta-
bilize a patient with medical therapy before safely placing a CVC or PA catheter.
Once a PA catheter confirms precapillary PH with normal left heart filling pressures,
if there is no echo-Doppler or clinical concern for a propensity for left heart failure, an
indwelling PA catheter is not likely indicated. Ongoing monitoring of CVP and CVO2 to
monitor responses to therapy is of value40; however, continuous PA or PCW pressure
measurements are not necessary for acute ICU management. Rather, it can further
raise the risk of infection, bleeding, or PA rupture if the balloon tip is accidentally
inflated in the wedge position of a stiff and poorly compliant PA, and inadvertent
 Pulmonary Hypertension and Right Ventricular Failure in Critical Care 131

clinician response to strikingly elevated PA pressures as the target for therapy, rather
than CVP and cardiac output.

Intubation for Invasive Positive Pressure Ventilation

Respiratory failure is a common feature of patients with acute RV failure. Hypoxemia,
hypercapnia, and atelectasis increase PVR, thus are important targets for optimiza-
tion. Although positive pressure ventilation and peri-intubation instability are associ-
ated with adverse hemodynamics, morbidity, and mortality,41 invasive mechanical
ventilation should not be delayed when required.
Positive airway pressure decreases RV preload owing to alterations in the mean
systemic venous pressure and right atrial pressure gradient, and increased compres-
sion of the hepatic veins and superior vena cava. Increased intrathoracic and transpul-
monary pressure increases RV afterload, particularly at higher tidal volumes, higher
positive end-expiratory pressure, and native lung hyperinflation.
These effects apply to both noninvasive ventilation (NIV) and invasive ventilation,
with decreased cardiac output noted in ambulatory patients with PH using NIV.42 De-
lays in appropriate invasive mechanical ventilation can lead to increased complica-
tions and worsened outcomes.43–45 Should a critically ill patient with RV failure
require NIV, a time-limited trial should be considered while formulating a comprehen-
sive intubation plan.
Intubation adds additional deleterious hemodynamic effects to right heart function.
The described effects of supine positioning may be mitigated by ramp or incline posi-
tioning. High-flow nasal oxygen may minimize hypoxia during intubation, although
peri-intubation lung de-recruitment remains a danger. Induction with anesthetic
agents, notably propofol, can decrease myocardial contractility and cause systemic
vasodilation, leading to decreased coronary perfusion, increased oxygen demand,
RV ischemia, and cardiac arrest. Invasive blood pressure monitoring and vasopressor
support are crucial before consideration of intubation. Induction with minimal sedation
or awake intubation is preferred,46 avoiding agents with myocardial depressant prop-
erties.47 Extracorporeal support should be on standby if available, or awake ECMO
may be considered.

SUMMARY

PH and RV failure in the ICU remain a broad category of clinical phenotypes that
require expert assessment and management. It is crucial to use history, physical ex-
amination, echo-Doppler, ECG, and chest imaging to quickly characterize an unstable
patient’s likely PH cause and hemodynamic profile. RHC, when able to be performed
safely and accurately, remains the standard for hemodynamic diagnosis.
It is similarly essential to manage RV failure and preload optimization in the ICU
based on distinct physiologic mechanisms of RV failure. When secondary to afterload
(PH with elevated PVR), PH medical therapy should be deployed with diuresis to
normal CVP. This is distinct from intrinsic RV contractility dysfunction (such as RV
infarct or cardiomyopathy), which may instead be more dependent on preload and
warrant inotropic or mechanical support.
Common pitfalls should be avoided when managing PH and RV failure in the ICU,
including pericardiocentesis for hemodynamically mediated pericardial effusion in
PAH, attempts at volume resuscitation when the RV is not dependent on preload,
percutaneous RVAD in the RV that is failing from high afterload, immediate and contin-
uous invasive monitoring with PA catheter in critically ill patients, and intubation for
invasive positive pressure ventilation without adequate preparation and optimization.
132 Vaidy et al

Urgent consultation or referral to centers with expertise in PH and RV failure is para-

mount in the care of this complex overlap of syndromes in the cardiovascular ICU.

CLINICS CARE POINTS

 Pulmonary hypertension in the critical care setting should be clinically assessed using history,
physical exam, and noninvasive data to characterize into the various diagnostic groups.
 Echocardiography with Doppler can be used safely and noninvasively to quickly assess right
heart function and characterize the underlying hemodynamic profile of pulmonary
hypertension related to PVR elevation or left heart disease.
 When safely available, invasive hemodynamic assessment should be obtained using central
venous catheter or right heart catheterization.
 Right heart failure may manifest from derangements in preload, afterload (such as
pulmonary hypertension), or intrinsic myocardial dysfunction (such as myocardial
infarction), each warranting distinct management strategies.
 In PH due to high afterload, such as PAH, it is important to avoid common pitfalls of excessive
volume loading, pericardiocentesis, or mechanical circulatory support.
 Invasive hemodynamic monitoring, invasive positive pressure ventilation, and clinical
management in the critical care setting should be managed with urgent consultation or
referral to centers with expertise in pulmonary hypertension and right heart failure.

DISCLOSURE

The authors have nothing to disclose.

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