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Peri-operative management of patients with pulmonary hypertension or cardiomyopathy

  • Introduction


    Definition of Terms

    Assessment & Inclusion Criteria


    Pre and Post Procedural Care

    Special Consideration


    Evidence Table



    Children with either PAH and Cardiomyopathy represent a small cohort of patients with severe and progressive disease. General anaesthesia (GA) or procedural sedation (PS) is sometimes required for assessment of disease severity via cardiac catheterization, for insertion/removal of central venous access devices (CVAD’s) to administer long term intravenous medication and for general surgical requirements. General anaesthesia or procedural sedation is used during such interventions for practical reasons in young patients and to facilitate physiological stability under controlled conditions. Whilst survival has improved recently with PAH targeted therapies, mortality under general anaesthesia remains high.

    Patients with PAH or cardiomyopathy are at an increased risk of potential complications including, prolonged hospitalisation, utilisation of intensive care resources and death. Any procedure with anaesthesia needs to be treated with caution due to the potential of PAH crisis/ cardiac decompensation with subsequent cardio-respiratory arrest. 


    The guideline exists to highlight the anaesthetic risk in PAH or Cardiomyopathy patients, and the important steps needed to mitigate this anaesthetic risk in all areas of perioperative care. This includes preoperative anaesthetic evaluation, clear risk stratification, ensuring adequate hydration during fasting, expert anaesthetic care with attention to the stimuli that can provoke a pulmonary hypertension crisis or cardiac decompensation, planning for ECMO standby and routine PICU admission in higher risk patients.

    Identification of risk factors and diligent planning of intervention with specific strategies should ultimately reduce complications and mortality of these complex and unpredictable patients.

    This guideline provides medical, nursing and allied health professionals information and strategies to manage patients with identified risks associated with PAH or cardiomyopathy and the significance of planned interventions requiring anaesthetic agents, to optimize the best outcome for the patient.

    Definition of Terms 

    1. Pulmonary Hypertension (PH):

    Pulmonary Hypertension is defined by an elevation of the mean pulmonary artery pressure of ≥ 20mmHg at rest from any cause, measured on a right heart catheterisation. It is important to appreciate that PH is simply a description of the haemodynamic state of the pulmonary circulation, and many diseases and mechanisms can lead to elevated pulmonary artery pressure.    

    2. Pulmonary Arterial Hypertension (PAH):

    A progressive, debilitating disease characterised by an increase in pulmonary vascular resistance leading to right ventricular failure and death. PAH describes a subgroup that is distinguished by a mean pulmonary artery pressure (PAPm) that exceeds 20mmHg at rest, with normal pulmonary arterial wedge pressure (PAWP) ≤15mmhg and a pulmonary vascular resistance index (PVRI) > 3 U.msq / >3.0 Wood units m2. 

    3. Cardiomyopathy:

    A disease of the myocardium associated with cardiac dysfunction, and is classified into groups as listed below:

    • Dilated Cardiomyopathy: characterised by dilatation and impaired contraction of the left or both ventricles and frequently presents with heart failure. If myocarditis is the cause, spontaneous resolution may be more likely. Patients with dilated cardiomyopathy may have accompanying diastolic dysfunction.
    • Hypertrophic Cardiomyopathy: is characterised by ventricular hypertrophy and usually involves the intraventricular septum and/or the left ventricle and right ventricular free walls.
    • Restrictive Cardiomyopathy: is a rare disease in children with restrictive and reduced filling of one or both ventricles with normal or near normal systolic function.
    • Left ventricular non–compaction: is characterized by deep trabeculations in the ventricular wall, which has defined recessed communication with the main ventricular chamber. This can be associated with systolic dysfunction, hypertrophy or restrictive pathophysiology.

    4. Anaesthesia Care:

    The intra operative monitoring and cardio-respiratory support of the patient is to be conducted by the anaesthetist. Pharmacological management encompasses the following categories of agent (alone or in combination): local anaesthetic, analgesia, sedation, and full general anaesthetic. It also involves level of parental involvement, Child Life Therapy and if required Comfort Kids involvement to assist in alleviating procedural related stress/anxiety (known PH crisis/cardiac decompensation trigger).

    5. Identified at-risk:

    Identified risk factors predispose individuals with PAH or Cardiomyopathy to potential complications including, prolonged hospitalisation, additional therapies and the risk of morbidity and mortality. Any procedure with anaesthesia needs to be treated with caution due to the potential of PAH crisis/cardiac decompensation with subsequent cardio-respiratory arrest or the purposes of this guideline the term “at-risk” pertains to patients identified with an elevated likelihood of experiencing complications across all areas of peri operative care.

    Alert system in place for PAH/PH patients filed under FYI flag.

    Anaesthetic alert for all PAH/ PH patients. Pain/procedural support plan alert identifying patients with procedural related anxiety to help guide clinician directed care and avoid cardiac decompensation.

    Because of the heterogeneity of cardiomyopathy types and severity, the treating physician should exercise their discretion about the application of CPG to their particular patient.

    6. Intervention:

    The procedures undertaken within this patient group most commonly include right heart catheterization (RHC), MRI’s, CT scans, insertion or removal of CVAD’s, dental procedures and other general surgical procedures.

    7. Pulmonary Hypertensive Crisis:

    Apulmonary hypertensive crisis is characterized by a rapid increase in pulmonary vascular resistance (PVR) to the point where pulmonary arterial pressure (PAP) exceeds systemic blood pressure (BP). The resulting right heart failure leads to a decrease in pulmonary blood flow, decreased cardiac output, hypoxia, and biventricular failure4. Known stimuli include hypoxemia, anxiety, hypercapnia, acidemia, hypothermia, vasoconstriction and noxious stimulus including endotracheal intubation that may elevate sympathetic tone. Hypotension and tachycardia are early signs of elevated pulmonary arterial pressure; if there is progression towards a pulmonary hypertensive crisis, the patient may become bradycardic and pale (signifying a drop in cardiac output)5. Other clinical features include abrupt desaturation, systemic hypotension and elevated central venous pressure (CVP). These are ominous signs and may signify an impending cardiac arrest.

    8. ECMO standby: Confirmed availability and allocation of essential ECMO staff prior to commencement of intervention (pre anaesthetic huddle), with extracorporeal membrane oxygenation unit in the operating room for immediate set-up and prime, in case of emergency situation. It is mandatory that a cardiac surgery nurse is scrubbed and ready to rapidly facilitate ECMO support, and a cardiac surgeon or fellow is available as per the Stand-by ECMO Activation Procedure located via the PICU guidelines (

    If ECMO standby is required, Cardiac coordinator to notify essential ECMO staff.  

    9. Brain Natriuretic Peptide (BNP):

    This hormone is released in response to high ventricular filling pressures. BNP is a sensitive, diagnostic marker for heart failure.   

    Assessment and Inclusion Criteria

    Patients with PAH or Cardiomyopathy are at risk of serious cardiorespiratory compromise both during and for a few hours after an anaesthetic. Optimal perioperative management requires good communication between the treating team, anaesthetists, intensivists and the cardiology team. For high risk patients the conversations need to include the cardiac surgeons and perfusionists. It also requires careful risk stratification into Lower or Higher risk.

    Please consider the following when assessing patients: 


    This should include questions directed towards signs and symptoms of heart failure and other recent symptoms, as well as functional status (i.e. ability to undertake age appropriate physical activity).  

    • Onset of symptoms, time when diagnosis made
    Symptoms history:
    • Syncope at rest or with exertion
    • Reduced exercise capacity, sedentary lifestyle
    • Shortness of breath at rest or with minimal exercise
    • Symptoms of arrhythmias and fluid retention
    • Chest pain (often frequent and non-specific)
    • Rate of symptom progression
    • Oxygen Therapy
    • Oral heart failure therapy
    • Chronic epoprostenol infusion
    • Intercurrent inotropic or respiratory support
    Past Anaesthetics:
    • Previous instability with procedures involving sedation or general anaesthesia


    • Resting heart rate, respiratory rate, blood pressure
    • Work of breathing
    • Oxygen saturations (minor intrapulmonary shunting is common with severe PAH)
    • Signs of heart failure (elevated JVP, peripheral oedema, lung creptitations)
    • Poor peripheral perfusion


    Chest X-Ray:

    • Heart size, pulmonary oedema, pleural effusion 

    Echocardiogram (Pulmonary Arterial Hypertension):

    • Right atrial and ventricular size
    • RV wall thickness and systolic function
    • RV systolic pressure estimated from TR velocity
    • RV diastolic pressure estimated from PR velocity
    • Pericardial effusion (more than trivial)
    Echocardiogram (Cardiomyopathy):
    • Left atrial and ventricular size
    • LV ejection fraction <40% or FS <20%
    • More than mild mitral regurgitation
    • Evidence of right ventricular involvement
    • Restrictive pathophysiology
    Recent BNP

    From this information, using the table on the following page the patient’s risk can be stratified to low or higher risk:

    Pulmonary Arterial Hypertension Patients:

    TABLE 1: Risk factor guide for PAH (Reference 9)

    One or more risk factors: will need to consider higher risk pathway.

    For higher risk cases discussion required between Anaesthetist/Cardiologist concerning need for ECMO.

    For further detail please refer to sub title Management



    Age < 5 years  
    Prior instability during anaesthesia  
    Recent or current signs and symptoms of heart failure including syncope  
    Recent symptom progression  
    NYHA 3 or 4 functional status classification  
    Growth impairment  
    Markedly elevated or increasing BNP  
    • Marked RA or RV dilatation,
    • Marked RVH,
    • Severe TR,
    • RV systolic dysfunction,
    • Near systemic RVSP,
    • Pericardial effusion
    Unfavourable haemodynamics at recent RHC;
    • CI <2.5l/min/msq,
    • Mean Rap >10mmHg,
    • PVRI >20U.msq

    Cardiomyopathy Patients:

    TABLE 2: Risk factor guide for Cardiomyopathy 



    Age < 5 years  
    Prior instability during anaesthesia  
    Recent or current signs and symptoms of heart failure  
    Recent symptom progression  
    Resting tachypnoea or tachycardia  
    NYHA 3 or 4 functional status classification  
    Receiving inotropic or respiratory support  
    Growth impairment  
    Markedly elevated or increasing BNP  
    • Marked LA or LV dilatation
    • LVEF <40% of FS <20%
    • Severe mitral regurgitation
    • Marked restrictive physiology

     Assessment and Inclusion Criteria Figure 1: 

    Key Step Lower Risk Pathway Higher Risk Pathway
    Booking AM List AM List
    Assessment PARC Anaesthetic Review in Koala
    Admission Day of Procedure Day Prior to Procedure
    Fasting Access & IV Fluids Access & IV Fluids
    Anaesthetic Prep Ensure NO Available Ensure NO Available
    ECMO Standby No Yes
    Postoperative Overnight Koala Admission
    ≥ 6 hour Rosella Observation if unstable
    ≥ 6 Hour Rosella Observation


    The assessment of the risk pathway is ultimately a decision for the treating physician/team.  It is essential that consultation with treating physicians regarding the level of risk to patients on an individual basis occur, in order to activate this clinical guideline. 


    The overall management plan should minimise stress and maintain haemodynamic conditions as close to baseline, this can be achieved as follows:

    • When booking theatre please notify CNC PH or CNC VAD
    • If high risk patient, book Rosella bed 
      • discuss with ECLS consultant (if ECMO is required) 
      • discussion about ECMO availability for very high risk cases should be made between the Cardiology and Anaesthetic teams,
    • Anaesthetist to network with Cardiac coordinator to schedule timely booking
    • ECMO standby: Patient should be booked on AM theatre list. Anaesthetist to discuss with perfusionist and cardiac surgeon concerning optimal timing of surgery 
    • Cardiac coordinator to book patient in Cardiac Surgery Theatre
    • In the event that the procedure is not performed in a cardiac theatre, the Cardiac Coordinator is still required to confirm availability and allocation of essential ECMO staff. In additional the Anaesthetist in charge and Floor Coordinator requires notification. After hours the Nurse in charge should be notified. 
    • Once booking confirmed CNC to email all persons involved /teams (see table 3 for list of staff) regarding patient procedure and plan, include the need for ECMO standby if necessary.  
    • Cardiac Coordinator to email cardiac surgery op list +/- need for ECMO backup   
    • CNC PH or CNC for VAD to communicate admission plan and process to family via telephone  
    • Finally the assessment of the risk pathway is ultimately a decision for the treating physician/team.

    TABLE 3: Anaesthetic Procedure timeline guide

    CNC PH/ CNC VAD to initiate and plan as below for procedures related to PAH or Cardiomyopathy.

    It is suggested all other teams planning procedures for these patients, are to follow the below guide. 

    Planning Phase 7-14 days pre op 2-7 days pre op 1-2 days pre op
    Liaise with cardiac surgery and cardiology for most appropriate date & timing of intervention.

    Email the following:

    Cardiac coordinator, NUM’s /ANUM’S (PICU, Koala, cardiology, cardiac theatre), ECLS clinical nurse consultant, anaesthetics, perfusionists, haematology, PH or heart failure team, on-call cardiologist, bed manager, catheter lab, elective surgery access manager and child life therapist.

    Any other persons involved in care or procedure.

    Attach CPG to email.

    Document admission into Koala (or primary care unit) diary & discuss with relevant NUM’s. Liaise with cardiac coordinator & bed manager prior to admission regarding high-risk patient to confirm admission and procedure.
    Book procedural date & include high risk criteria into EMR booking by admin staff.   Book Rosella bed post-operatively for high-risk cases and discuss with ECLS consultant. Cardiology fellow or Ward Doctor to complete orders on EMR including medications, tests & IV fluids whilst fasting. Ensure a senior consultant paediatric anaesthetic review of patient and previous anaesthetic interventions pre-operatively.
    Referral to Child Life therapy for procedural sedation cases.   Child Life Therapy to follow up for planning peri-operative management. If applicable FYI flag for pain/procedure support plan will be created. In collaboration with haematology staff, support  timing & cessation of anticoagulation as an outpatient as relevant. Alert ward pharmacist of planned admission.
    Discussion with cardiologist(s) irrespective of whether procedure is cardiac or non-cardiac in nature.   Follow up email to perfusionists re high risk patient height and weight for ECMO circuit. Once admitted, obtain peripheral IV access, pathology (blood group and antibody screen extended expiry) and commence IV hydration once fasting.

    Pre and post procedural care

    The staff whom are allocated for direct care of patient are responsible to ensure below tasks are completed

    Nursing/Medical management

    Pre-operative care:

    • Pathology + blood group and antibody screen extended expiry + Peripheral IV access
    • Ensure IV hydration during fasting period until oral fluids are tolerated post-operatively. To commence IV hydration the night prior to procedure at ¾ maintenance fluid. If on PM list, commence IV hydration when fasting
    • Pre-medication to be determined by Anaesthetics
    • Promotion of a calm environment is important to minimise stress / anxiety which is a known stimuli for cardiac decompensation
    • Continue usual medications whilst fasted unless directed otherwise
    • Both lower and higher risk patients: direct transfer to anaesthetic bay/theatre, by-pass pre-operative holding bay

    Continual bedside monitoring:

    • Heart Rate (ECG)
    • Blood pressure (reportable mean limits documented)
    • Oxygen Saturations (reportable limits documented)

    Causes/ known stimuli for cardiac decompensation:

    • Hypoxemia
    • Anxiety
    • Hypercapnia
    • Acidemia
    • Hypothermia
    • Vasoconstriction
    • Noxious stimulus including endotracheal intubation that may elevate sympathetic tone.
    • Hypotension and tachycardia are early signs of elevated pulmonary arterial pressure; if there is progression towards a pulmonary hypertensive crisis, the patient may become bradycardic and pale (signifying a drop in cardiac output)
    • Other clinical features include abrupt desaturation, systemic hypotension and elevate central venous pressure (CVP)

    Warning signs of deterioration:

    • patient restless or showing signs of discomfort
    • falling saturations
    • systemic hypotension
    • sinus tachycardia or bradycardia
    • pallor or poor peripheral perfusion
    • elevated central venous pressure (CVP)

    Management of cardiac decompensation during a procedure:

    • administer 100% oxygen
    • institute inotropic support
    • initiate nitric oxide (NO) therapy (for PH patients)
    • administer opioid & deepen anaesthesia (for PH patients)
    • correct metabolic & respiratory acidosis; treat hypothermia if present
    • ensure ICU are notified
    • consider requirement for circulatory support if patient not improving

    Post-operative care:

    Continual bedside monitoring:

    • Heart Rate (ECG)
    • Blood pressure (reportable mean limits documented)
    • Oxygen Saturations (reportable limits documented)
    • Lower risk patient: Stage 1 Recovery then transfer to Koala ward when hemodynamically stable.
    • Higher risk patient: Direct transfer from theatre to Rosella/PICU, bypass recovery, for 6 hours post-procedural care and observation for warning signs of acute PH crisis/ cardiac decompensation  
    • Ensure adequate analgesia is ordered and administered to promote optimal patient comfort
    • Ensure IV hydration until tolerating oral fluids post-operatively
    • The patient to return back to pre-procedure baseline prior to discharge or ward transfer

    Process of discharge: treating team or on-call team to assess patient prior to discharge and make appropriate follow-up arrangements 

    Special Considerations

    Intellectual ability and cognitive development with regard to a child’s cooperation should guide the implementation of procedural sedation or administration of general anaesthesia.


    Evidence Table

    The evidence table for this guideline can be viewed here.  


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    5. Taylor MB, Laussen PC. Fundamentals of Management of Acute Postoperative Pulmonary Hypertension. Pediatr Crit Care Med. 2010;11(2):S27-S29.
    6. Beghetti M., Barst R. J., Naeije, R., Rubin, L.J. Pulmonary Arterial Hypertension Related to Congenital Heart Disease. 2006, Elsevier GmbH, Munich. Chapter 12.
    7. Rose M, Norman B, Reducing Anaesthetic Risk in Idiopathic Pulmonary Arterial Hypertension; 2015 Nursing Research & Clinical Innovations Symposium RCH   
    8. Chiletti, R. (Oct, 2020). Stand-by ECMO activation procedure.
    9. Rosenzweig EB, Abman SH, Adatia I, Beghetti M, Bonnet D, Haworth S, Ivy DD, Berger R. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J 2019; 53: 1801916. 


    Please remember to read the disclaimer

    The revision of this nursing guideline was authored by Samantha Lopez, CNC, Cardiology and Kathy Lim, CNC, Cardiology, and approved by the Nursing Clinical Effectiveness Committee. Updated January 2021.