GENERAL ANAESTHESIA Tutorial 459

Perioperative Management of
Patients With Pulmonary
Hypertension and Right Ventricular
Dysfunction
Christopher Wood1†
1
Anaesthesia and ICM trainee, Sunderland Royal Hospital, Sunderland, UK

Edited by: Niraj Niranjan, Consultant Anaesthetist, University Hospital of North Durham,
Durham, UK

†Corresponding author e-mail: [email protected]

Published 16 November 2021

KEY POINTS
 Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure 20 mm Hg at rest.
 Perioperative management aims to minimise the effects of anaesthesia and surgery on right ventricular function in
patients at risk of such dysfunction as a result of chronic PH.
 Right ventricular function can be compromised if there is (1) a reduction in right ventricular perfusion if systemic
vascular resistance (SVR) falls and/or (2) a reduction in right ventricular stroke volume if pulmonary vascular
resistance (PVR) increases.
 Falls in SVR can be minimised with the judicious use of vasopressors and intravenous fluids.
 Acute increases in PVR can be minimised by avoiding direct pulmonary vasoconstriction precipitants, namely,
hypoxia, hypercarbia, acidosis and hypothermia.
 Regional anaesthetic techniques, where possible, are often preferable as they are less likely to compromise SVR and
PVR in the manner described above.

INTRODUCTION
Pulmonary hypertension (PH) is defined as a pulmonary artery catheterisation pressure of 20 mm Hg at rest and pulmonary
vascular resistance .3 Wood units (WU).1 Severity is classified as mild (20-40 mm Hg), moderate (40-55 mm Hg) and severe
(.55 mm Hg).
A Joint Task Force of the European Society of Cardiology and European Respiratory Society (ESC/ERS) provided a
classification for the aetiology of PH (Figure 1).
1. Class 1: disease of the pulmonary arterial vasculature
2. Class 2: due to left heart disease
3. Class 3: due to lung disease or hypoxia
4. Class 4: due to pulmonary artery obstruction
5. Class 5: multifactorial aetiologies

An online test is available for self-directed continuous medical education (CME). It is estimated to take 1 hour
to complete. Please record time spent and report this to your accrediting body if you wish to claim CME points. TAKE ONLINE TEST
A certificate will be awarded upon passing the test. Please refer to the accreditation policy here.

Subscribe to ATOTW tutorials by visiting https://resources.wfsahq.org/anaesthesia-tutorial-of-the-week/

Figure 1. Mechanisms of PH. Based on ESC/ERS guidelines for the diagnosis of PH, 2015. RV indicates right ventricle; PA, pulmonary artery;
PV, pulmonary vein; LV, left ventricle.

The European epidemic of PH in the mid-1960s, following widespread use of the amphetamine-like prescription appetite
suppressant Aminorex, resulted in recognition by the World Health Organisation.2
The underlying causes vary between countries, with low-income countries burdened by infective aetiologies such as HIV,
rheumatic heart disease and schistosomiasis, whilst the aetiology in resource-rich, high-income countries is largely secondary
to chronic obstructive pulmonary disease (COPD) and left ventricular failure secondary to arterial disease. The annual
incidence of PH is increasing in the United Kingdom; current estimates predict 8 cases per million people.
Demographically, patients diagnosed with PH are in the fifth decade of life (53 6 14 years) and are more likely to be female (2-
5:1) with class 2 or 3 disease aetiology. The reason for the inequality between sexes remains unclear. Multicentre US-based
registry data (REVEAL Registrye) highlight a racial disparity: 73% Caucasians, 13% African Americans and 9% Hispanic.3
Early disease recognition is often challenging because of nonspecific symptoms, leading to a delay in diagnosis of up to 2
years, with a clear influence on disease prognosis, especially in the case of systemic sclerosis.

Right Ventricular Physiology and Pathophysiology

Right Ventricular Structure and Blood Supply
The right ventricle (RV) pumps deoxygenated blood into the high-volume, low-pressure lung vasculature. This system requires
less stroke work when compared with the left ventricle (LV), with a consequent 6-fold reduction in right ventricular mass.
Usually, the right coronary artery (RCA) supplies the right heart. In the absence of pathology, such as hypertrophy, the
relatively low intraventricular pressure allows flow through the RCA throughout the cardiac cycle, although diastolic flow
predominates in the more distal vessels. The RV perfusion pressure is therefore dependent on the mean arterial pressure
rather than diastolic pressure alone; this differs from the situation with LV perfusion.4

Pulmonary Vascular Resistance

PH may or may not be associated with a raised pulmonary vascular resistance (PVR). Nevertheless, anaesthetic management
is frequently based on avoiding acute increases in PVR that can exacerbate RV dysfunction. Understanding factors that alter
PVR is therefore critical in the perioperative management of these patients.
The pulmonary vasculature is thin walled and easily distensible; resistance is low and dependent on passive physical factors
such as gravity, posture and the pressure differential between alveoli and capillaries. The alveolar-capillary pressure differential
is increased at times of low cardiac output (capillaries easily collapse), lung underexpansion (forces holding alveoli open are
reduced), or lung overexpansion (pulmonary capillaries are compressed). Other factors actively alter PVR by affecting
pulmonary vascular muscle tone (Table 1).

Pathophysiology
Chronic changes in RV afterload, as observed in PH, can negatively impair right coronary perfusion, because of the effect
of myocardial adaptive changes on the coronary vessels, similar to what occurs with systemic hypertension impairing LV

Subscribe to ATOTW tutorials by visiting https://resources.wfsahq.org/anaesthesia-tutorial-of-the-week/

 Raises PVR
Hypoxia
Hypercapnia
Acidosis
Histamine
Serotonin
Sympathetic a-adrenergic fibres
Lowers PVR
Hyperoxia
Hypocapnia
Alkalosis
Nitric oxide
Prostacyclin
Sympathetic b-adrenergic fibres
Parasympathetic cholinergic fibres
Table 1. Factors Affecting Pulmonary Vascular Resistance (PVR)

myocardial perfusion. This may make the RV of a patient with PH more susceptible to ischaemia if perfusion pressure
drops.
In health, the RV can dilate and accommodate higher volumes more easily than the LV does. However, chronic elevation in
pulmonary artery pressure can lead to hypertrophic changes with an increase in RV wall thickness and a reduction in internal
radius. At this stage, the RV responds poorly to acute elevations in pulmonary artery pressure, which may precipitate acute RV
failure secondary to its overdistention, both macroscopically into the LV (via intraventricular septal deviation) and
microscopically due to sarcomere overstretching. Ultimately, both mechanisms lead to a reduction in output from both
ventricles for a given preload, resulting in cardiogenic shock.

PREOPERATIVE ASSESSMENT AND MANAGEMENT

History
Symptoms of chronic PH are often nonspecific, leading to a delay in diagnosis. Symptoms can be classified into 4 categories:
1. Systemic symptoms of reduced cardiac output (eg, dyspnoea and fatigue)
2. Primary cardiac symptoms: RV failure leads to exertional chest pain and syncope with weight gain and fluid overload.
3. Intrathoracic mechanical symptoms: Dilation of the pulmonary artery trunk can lead to compression of the left recurrent
laryngeal nerve, presenting as hoarseness.
4. Symptoms relating to the underlying aetiology (Figure 1)
a. Symptoms suggestive of upper airway disease (obstructive sleep apnoea), chronic respiratory disease (COPD, venous
thromboembolism), connective tissue diseases, endocrine and metabolic disorders
b. History of illicit drug use, alcohol intake, cigarette smoking and HIV risk factors5

Examination
The clinical signs can reflect the following:
1. RV failure: elevated jugular venous pressure and associated signs of fluid overload, such as tender hepatomegaly, ascites
and peripheral oedema
2. Tricuspid valve regurgitation causing a pansystolic murmur and prominent ‘v’ wave on the central venous pressure
waveform
3. Elevated pulmonary artery pressure leading to an increased intensity of the second heart sound (loud P2)

Investigations
Clinical investigations can be used to both screen and diagnose patients with PH. Basic screening investigations should include
an echocardiogram; the 2018 British Society of Echocardiography outlines criteria for diagnosis of PH (Table 2).6 Needless to
say, this echocardiogram can also comment on the size and function of the RV to help delineate the end-organ consequences
of a diagnosis of PH.

Subscribe to ATOTW tutorials by visiting https://resources.wfsahq.org/anaesthesia-tutorial-of-the-week/

 Structure Parameter
Ventricle RV/LV basal diameter ratio .1.0
Flattening of the interventricular septum
PA RV outflow Doppler acceleration time ,105 ms and/or mid-systolic notching.
Early diastolic pulmonary regurgitation velocity .2.2 m/s
PA diameter .25 mm
IVC and right atrium IVC diameter .21 mm with decreased inspiratory collapse (,50% with a sniff)
Right atrial area end-systole diameter .18 cm

Table 2. Diagnostic Criteria for PH During Echocardiography. Echocardiography criteria to suggest PH is determined by
positive parameters from at least 2 groups (ventricle, pulmonary artery, inferior vena cava and right atrium). Adapted with
permission from the British Society of Echocardiography 2018 guidelines.6RV indicates right ventricle; LV, left ventricle; PA,
pulmonary artery; IVC, inferior vena cava

Additional tests that may provide further information include the following:
1. Laboratory biochemistry (cardiac disease may cause associated renal dysfunction, either directly or as a consequence of
pharmacologic treatment for heart failure)
2. Elevated brain natriuretic peptide may be suggestive of RV 6 LV dysfunction
3. Electrocardiography (right axis deviation, right bundle branch block and finally R wave/S wave ratio .1 in lead V1 are
suggestive of RV strain)
4. Chest x-ray (may show evidence of cardiorespiratory pathology and PH with enlargement of central pulmonary vessels and
right atria and ventricular enlargement)
Pulmonary function tests (PFTs) are a noninvasive method of assessing lung function. Patients presenting with PH should
undergo PFTs, especially those with multifactorial (class 5) or lung-specific disease (class 3). Attention should be focused on
reversibility, common airway patterns (forced expiratory volume in 1 second/forced vital capacity ratios) and gas transfer
(diffusing capacity for carbon monoxide) rates.
The gold standard for diagnosis remains right heart catheterisation (RHC; Figure 2).7 Parameters of note during RH are as
follows:
1. Pulmonary
a. Pulmonary artery pressure
b. PVR
c. Pulmonary capillary wedge pressure (Figure 2)
2. Cardiac
a. Right ventricular pressure

Figure 2. Pulmonary artery (PA) catheterisation to calculate (1) mean PA pressure (mPAP) and (2) pulmonary capillary wedge pressure
(PCWP). RV indicates right ventricle.

Subscribe to ATOTW tutorials by visiting https://resources.wfsahq.org/anaesthesia-tutorial-of-the-week/

 b. Right atrial pressure
c. Cardiac output
d. Cardiac index

Cardiopulmonary exercise testing (CPET) remains a fundamental noninvasive and dynamic tool to determine functional
capacity and exercise limitation whilst attaining information regarding metabolic capacity and cardiorespiratory function.8 CPET
can provide supporting information regarding disease patterns but, unlike RHC, will not diagnose PH.
Patients with PH may be unable to achieve adequate perioperative oxygenation, due to impaired cardiac output in the presence
of elevated mean pulmonary artery pressures (mPAPs) and possible RV dysfunction. The most recent European guideline
(2015 ESC/ERS) uses peak VO2 (maximal oxygen consumption in mL/kg/min) and VE/V 0 CO2 (ventilatory equivalents for
carbon dioxide) to categorise PH patients into low-, medium- or high-risk categories, although the document acknowledges that
the incremental ramp protocol used for CPET is not standardised for PH patients.

Chronic Disease Management

In the United Kingdom, chronic disease management for PH is initiated by 1 of 8 tertiary centres when the mPAP is 25 mm
Hg. There are effective drug treatments available specific to each aetiology.
Pulmonary endothelial cells release nitric oxide, which stimulates vascular smooth muscle cells to produce cyclic guanosine
monophosphate (cGMP), an intracellular pathway, which then results in cyclic adenosine monophosphate–mediated
pulmonary vascular smooth muscle cell relaxation.
Basic drug classes for the pharmacologic management of PH include the following:
1. Phosphodiesterase-5 inhibitors (sildenafil): inhibition of this enzyme prevents the breakdown of intracellular cGMP
2. Guanylate cyclase activators (riociguat): stimulates production of cGMP via the same pathway as nitric oxide
3. Prostacyclin receptor agonists (selexipag): these selectively bind the prostaglandin I receptor, a G-protein–coupled receptor
mediating vasodilation.
4. Endothelin receptor antagonist (ERA; ambrisentan, bosentan): ERAs inhibit the action of endothelin-1, an amino acid
produced by endothelial cells that mediates vascular constriction.
Active respiratory agents should be continued throughout the perioperative period, with conversion to inhaled or intravenous
routes when enteral is unavailable.
Other therapies may include diuretics and supplemental oxygen. A suspected prothrombotic state in PH or a thromboembolic
aetiology may mean patients are also treated with anticoagulants, but the heterogeneous nature of PH means this is often
patient specific.

INTRAOPERATIVE CARE
Preoperative Decision Making
It is important that patients are assessed and optimised by a specialist in PH prior to elective surgery; the presence of PH
increases perioperative mortality, so the risks and benefits of surgery need to be balanced against each other.
This may mean a multidisciplinary discussion to identify whether surgery is in the patient’s best interests or not. A condition that
might normally be treated surgically may be treated conservatively if that is the correct decision for that patient. This balance of
risks needs to consider the specialist’s opinion, the patient’s wishes, the surgical pathology and the site and magnitude of
surgery; this decision making is probably as important as the intraoperative management of the patient’s physiology.

Cardiovascular Requirements
The two main intraoperative goals when delivering anaesthesia for patients with PH aim to maintain RV output.
First, to avoid compromising the RV perfusion that takes place more or less continuously during the cardiac cycle, it is essential
to maintain systemic blood pressure. This means maintaining systemic vascular resistance (SVR) by counteracting the
reduction that often occurs in the presence of vasodilating anaesthetic agents. This can be achieved with vasopressors
administered peripherally (metaraminol or phenylephrine) or centrally (eg, noradrenaline or vasopressin).
Noradrenaline theoretically helps maintain RV function via b1 adrenoceptor agonism and b2-mediated PVR reduction, whilst
vasopressin offers a similar potential benefit by appearing to not cause pulmonary vascular constriction. However, these
benefits have not been proven to effect outcomes in clinical trials, and all of the agents have been used safely and effectively in
clinical practice.9

Subscribe to ATOTW tutorials by visiting https://resources.wfsahq.org/anaesthesia-tutorial-of-the-week/

 Technique Advantage Disadvantage
Regional anaesthesia Excellent opioid-sparing analgesia to reduce Not suitable for many types of surgery
pain and reduce opioid-induced
respiratory depression
No cardiorespiratory depression provided Some patients may not be able to maintain
that sedation is either avoided or minimal position, eg, lying flat with significant
respiratory disease
Central neuraxial Excellent opioid-sparing analgesia as above High blocks activating Bezold–Jarisch reflex
or direct sympathetic blockade may be
more poorly tolerated than normal
Not suitable for many types of surgery
General anaesthesia Secure airway with control of ventilation Anaesthetic agents may have a depressant
Reduced risk of increases in intrathoracic cardiovascular and respiratory effects that
pressure intraoperatively (coughing) need monitoring and mitigation
Easily titratable anaesthesia and analgesia
Table 3. Anaesthetic Technique, Advantages and Disadvantages Explained. PONV indicates postoperative nausea and
vomiting

Second, it is important to avoid acute elevations in PVR that will compromise RV ejection. This can occur with preserved RV
function but will be more severe in an impaired ventricle. As mentioned above, if the RV dilates in response to an increased
afterload, it may also lead to interventricular septum distortion, compromising LV ejection.10 PVR can be influenced both
pharmacologically and nonpharmacologically.
Nonpharmacologic measures include avoiding physiological precipitants of pulmonary vasoconstriction such as hypoxia,
hypercapnia (aim PaCO2 4-4.5 kpa), over- and underinflation of lungs (6-8 mL/kg ideal body weight), acidosis, pain and
hypothermia.
Pharmacologic measures include the implementation of pulmonary vasodilator agents that can be administrated by both
inhaled and intravenous routes. Agents include synthetic analogues of prostacyclin (iloprost and epoprostenol) and gases
(nitric oxide); these agents and, more importantly, experience of their use may be available only in specialist centres.
It may also be necessary to directly support cardiac output with an inotropic agent. Calcium sensitisers (eg, levosimendan) and
phosphodiesterase inhibitors (eg, milrinone) may be helpful as they can reduce PVR and therefore be more beneficial in right
heart failure. However, no selective right heart inotrope exists. Note that inotrope use may require concurrent vasopressor
administration to attenuate the vasodilation that occurs with some inotropic agents.

ANAESTHESIA TECHNIQUES
The anaesthetic technique most appropriate for each patient encounter will depend on patient, anaesthetic and surgical factors.
Patient factors obviously include relevant additional medical conditions and patient choice but will also need to take severity of
PH and associated cardiac dysfunction into account.
Anaesthetic and surgical factors include patient positioning, duration of surgery, site of operation and risk of cardiovascular
instability (eg, blood loss); these are clearly applicable with or without PH.
General and regional anaesthesia—be that central neuraxial, peripheral regional or local anaesthesia—are all potential options,
and the key advantages and disadvantages are listed in Table 3. Whilst many of these advantages and disadvantages are
generic, it is the avoidance of any unnecessary cardiac and respiratory depression that is of specific benefit in patients with PH,
more so than in a fit and healthy patient, hence the role of opioid-sparing analgesia, avoidance of excessive sedation or good
control of gas exchange.
Anticoagulation may be common and needs to be considered when opting for certain types of regional anaesthetic techniques.

Regional and Local Anaesthesia

Regional anaesthesia remains a safe and simple option to provide these intraoperative cardiovascular conditions with the
added advantage of good, intra- and postoperative opioid-free analgesia. Clearly, some forms of surgery, such as orthopaedic
or ophthalmic surgery, lend themselves to regional anaesthesia.
These advantages can be negated if regional anaesthesia is accompanied by any adverse effects of accompanying sedation.
The use of sedation should therefore be considered very carefully.

Subscribe to ATOTW tutorials by visiting https://resources.wfsahq.org/anaesthesia-tutorial-of-the-week/

 Component Parameter Goal
Systemic SVR Maintain SVR: rapid reduction can reduce venous return and RV output
SBP 90 mm Hg
MAP 65 mm Hg
PVR/SVR ratio ,0.4
Respiratory Low/normal PVR Maintain. Avoid rapid increases in PVR by preventing hypercarbia, acidosis,
hypoxemia, pain and hypothermia.
Minimize intrathoracic pressures by using Vt ,8 mL/kg ideal body weight if
possible and avoiding excessive positive end-expiratory pressure.
Cardiac Sinus rhythm Maintain (to optimise ventricular filling from atria)
Preload Maintain (as above)
Myocardial depression Avoid
Increased Afterload Avoid (compromises cardiac output)
Cardiac index 2.2 L/min/m2
RAP Lowest possible to maintain a MAP .65 mm Hg
Table 4. Perioperative Haemodynamic Goals and Parameters. Adapted with permission from Pilkington et al.12 SVR indicates
systemic vascular resistance; SBP, systolic blood pressure; MAP, mean arterial pressure; PVR, pulmonary vascular
resistance; Vt, tidal volume; RAP, right atrial pressure

Central Neuraxial Anaesthesia

Neuraxial anaesthesia can be applied safely to PH patients if attention is paid to achieving the cardiovascular goals described
above. Relatively rapid systemic vasodilation can occur, which can compromise right-sided cardiac filling as well as RV
coronary perfusion. If combined with inhibition of high thoracic cardioaccelerator autonomic nerve fibres, it can result in a
profound negative chronotropic and inotropic response. In both instances, cardiovascular impairment in the presence of
elevated mPAPs may lead to reduced respiratory perfusion, acute pulmonary hypertensive crisis and cardiovascular collapse,
so care must be taken with the previously detailed cardiovascular targets.

General Anaesthesia
Regional or local anaesthesia is often a preferred option. However, there are circumstances in which general anaesthesia is the
only choice, and it is still a safe option in PH patients if attention is paid to maintain the goals previously described.
Diligent control of CO2 and thus pH is important and is often more easily achieved via controlled ventilation. This usually
necessitates endotracheal intubation, but for shorter cases in appropriate patients (eg, nonobese, low risk of aspiration, etc), a
suitable second-generation supraglottic airway device (SAD) can be used.
Conversely, spontaneous ventilation techniques, usually but not exclusively involving an SAD, are usually avoided in patients
with PH unless the disease is mild and the procedure is short.
Both intravenous and volatile anaesthetic agents have cardiorespiratory depressant effects, but induction and maintenance of
anaesthesia can be safely achieved with both. All intravenous agents and all inhalational agents given up to a concentration of
1 minimum alveolar concentration have negligible effects on pulmonary vascular resistance.11
All agents reduce ventilatory drive and minute ventilation, but this is prevented by control of ventilation. There is reduced
hypoxic pulmonary vasoconstriction but little direct effect on PVR, apart from with nitrous oxide, which can directly increase
PVR and is therefore best avoided in patients with PH.
Cardiovascular effects vary depending on the agent used, but all generally decrease myocardial contractility, SVR, stroke
volume and blood pressure. There may be differing effects on heart rate depending on the agent and rate of
administration. All of these effects are predictable; appropriate use of monitoring and cardiovascular pharmacological
support can help to ameliorate them and maintain the target of a maintained SVR (for RV perfusion and RV filling from
venous return).
Patients should be monitored as per the standard recommended monitoring techniques described by the Association of
Anaesthetists of Great Britain and Ireland. Additional monitoring will depend on the patient, and the surgery and may include
invasive arterial and/or central venous pressure monitoring, pulmonary artery catheterisation or other cardiac output–
monitoring techniques. Suggested perioperative haemodynamic parameters—when available from invasive monitoring—are
suggested in Table 4.

Subscribe to ATOTW tutorials by visiting https://resources.wfsahq.org/anaesthesia-tutorial-of-the-week/

POSTOPERATIVE CARE
PH carries significant perioperative mortality and morbidity. Patients should be monitored postoperatively in high-dependence
areas. Postoperative goals reflect the cardiorespiratory parameters aimed at preventing elevations in PVR and maintaining
SVR, as described (Table 4).
Good analgesia, ideally using opioid-sparing approaches including nonsedating analgesics (paracetamol, nonsteroidal anti-
inflammatory drugs), and local or regional analgesia should be applied where possible, as this assists in minimising PVR
increases associated with hypoventilation as well as improving overall recovery.
There should also be minimal interruption of existing PH therapies.
Tachyarrhythmias are a common problem in PH patients. Unlike left heart diseases, arrhythmias are usually supraventricular,
with ventricular rhythms being rare. Supraventricular arrhythmias can compromise both long- and short-term prognosis, with a
potential for deterioration into right heart failure. Restoration and maintenance of sinus rhythm is highly desirable, with
prophylactic antiarrhythmics being frequently used. Data regarding individual drugs are lacking, but using an agent with minimal
negative inotropy should be considered.
Rhythms associated with haemodynamic instability should be treated as per resuscitation algorithms, using synchronised
electrical cardioversion as appropriate.

PULMONARY HYPERTENSIVE CRISIS

A pulmonary hypertensive crisis (PHC) is characterised by acute elevation in PVR that overwhelms the ability of the RV to
maintain output against the increased afterload and is associated with significant mortality (.50%).13 This can go on to
negatively affect LV preload, LV cardiac output and diastolic coronary perfusion pressure, leading to myocardial collapse and
cardiogenic shock.
Under normal physiological conditions, RV adaptation and remodelling help maintain RV output. However, intraoperative
physiological changes secondary to physical (eg, patient positioning and pneumoperitoneum) or pharmacologic (anaesthetic or
other drugs) factors can lead to decompensation. Rapid recognition of PHC is essential; whilst signs of cardiogenic shock are
characteristically observed, confirmation generally requires invasive pressure measurements and/or bedside echocardiography
(elevated right atrial and pulmonary pressures with decreasing cardiac output).
Treatment options are supportive or pharmacologic and reflect those previously discussed to control PVR:
1. Supportive
a. Treatment for a specific precipitating cause if possible (eg, pain, hypothermia)
b. Supplemental oxygen
c. Appropriate fluid administration (if hypovolaemia suspected)
d. Ventilatory support to maintain CO2 and control pH if necessary; postoperative respiratory depression can be
multifactorial
2. Pharmacologic
a. Cardiac output can be augmented with inotropic and chronotropic agents
b. Systemic vascular resistance can be augmented with vasopressors
c. Pulmonary vascular vasodilators can be given via the inhaled or intravenous route

SUMMARY
Patients with PH present for both elective and urgent cardiac and noncardiac surgery. Not all patients may have a
preoperative confirmed diagnosis, so clinical assessment may be needed to aid diagnosis and determine the degree
of cardiorespiratory compromise.
Intraoperative management should involve input from a physician experienced in the management of patients with
PH. Postoperative care should be performed in an area of high acuity, with regional opioid-sparing techniques forming
the mainstay of treatment.

REFERENCES
1. Condon DF, Nickel NP, Anderson R, et al. The 6th World Symposium on Pulmonary Hypertension: what’s old is new.
F1000Research. 2019;8(F1000 Faculty Rev-888).

Subscribe to ATOTW tutorials by visiting https://resources.wfsahq.org/anaesthesia-tutorial-of-the-week/

 2. Montani D, Seferian A, Savale L et al. Drug-induced pulmonary arterial hypertension: a recent outbreak. Eur Respir Rev.
2013;22(129):244-250.
3. Frost AE, Badesch DB, Barst RJ, et al. The changing picture of patients with pulmonary arterial hypertension in the United
States: how REVEAL differs from historic and non-US contemporary registries. Chest. 2011;139(1):128-137.
4. Crystal GJ, Pagel PS. Right ventricular perfusion: physiology and clinical implications. Anesthesiology. 2018;128(1):202-
218.
5. Elliot CA, Kiely DG. Pulmonary hypertension. Contin Educ Anaesth Crit Care Pain. 2006;6(1):17-22.
6. Augustine DX, Coates-Bradshaw LD, Willis J, et al. Echocardiographic assessment of pulmonary hypertension: a
guideline protocol from the British Society of Echocardiography. Echo Res Pract. 2018;5(3):G11-G24.
7. Rosenkranz S, Preston IR. Right heart catheterisation: best practice and pitfalls in pulmonary hypertension. Eur Respir
Rev. 2015;24(138):642-652.
8. Farina S, Correale M, Bruno N, et al. The role of cardiopulmonary exercise tests in pulmonary arterial hypertension. Eur
Respir Rev. 2018;27(148):170134.
9. Currigan DA, Hughes RJA, Wright CE, et al. Vasoconstrictor responses to vasopressor agents in human pulmonary and
radial arteries: an in vitro study. Anesthesiology. 2014;121:930-936.
10. Condliffe R, Kiely DG. Critical care management of pulmonary hypertension. BJA Educ. 2017;7(17):228-234.
11. Sarkar MS, Desai PM. Pulmonary hypertension and cardiac anaesthesia: anesthesiologist’s perspective. Ann Card
Anaesth. 2018;21(2):116-122.
12. Pilkington SA, Taboada D, Martinez G. Pulmonary hypertension and its management in patients undergoing non-cardiac
surgery. Anaesthesia. 2015;7(1):56-70.
13. Simon M. Perioperative management of pulmonary hypertensive crisis. Adv Pulm Hypertens. 2013;12(1):38-39.

This work by WFSA is licensed under a Creative Commons Attribution-NonCommercial-NoDerivitives 4.0 International
License. To view this license, visit https://creativecommons.org/licenses/by-nc-nd/4.0/

WFSA Disclaimer
The material and content provided has been set out in good faith for information and educational purposes only and is not intended as a
substitute for the active involvement and judgement of appropriate professional medical and technical personnel. Neither we, the authors, nor
other parties involved in its production make any representations or give any warranties with respect to its accuracy, applicability, or com-
pleteness nor is any responsibility accepted for any adverse effects arising as a result of your reading or viewing this material and content. Any
and all liability directly or indirectly arising from the use of this material and content is disclaimed without reservation.

Subscribe to ATOTW tutorials by visiting https://resources.wfsahq.org/anaesthesia-tutorial-of-the-week/