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Clinical Guidelines (Nursing)

Peri-operative management of patients with pulmonary hypertension or cardiomyopathy

  • Note: This guideline is currently under review.  



    Definition of Terms

    Assessment & Inclusion Criteria


    Special Consideration


    Evidence Table


    Companion Documents


    Children with 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. 

    The overall complication rate of children undergoing cardiac catheterization with general anaesthesia has been described, but few studies assess the impact of pulmonary arterial hypertension in children undergoing anaesthesia.  In 2009 a review was undertaken at RCH to determine the incidence of mortality following anaesthesia and to determine the incidence and nature of anaesthesia-related mortality in practice across over 100,000 cases1.  There were 10 anaesthesia-related deaths, with 5 (50%) cases having pulmonary hypertension.  Whilst survival has improved in the recent era with PAH targeted therapies, mortality under general anaesthesia remains high.  

    In the past 10 years there have been ongoing modifications to clinical practice for patients with known PAH and cardiomyopathy. These include avoidance of general anaesthesia where possible, prior treatment with targeted therapies and involvement of the anaesthetic and ICU teams prior to any procedure requiring GA or PS. The identification of potential risk factors should ultimately be incorporated into clinical guidelines and protocols to further reduce the risk for this difficult and unpredictable group of patients2.  Interventions should ideally be performed in a centre of excellence in which cardiologists, anaesthetists, nursing and ancillary staff are well acquainted with caring for this at-risk patient group3.


    This guideline has been developed for the entire peri-operative process including ward admission, pre-operative hold, theatre, recovery room and post-operative hospitalisation. Identification of risk factors and diligent planning of intervention with specific strategies should ultimately reduce complications and mortality of these complex and unpredictable patients.

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

    Definition of Terms 

    1. Pulmonary Arterial Hypertension (PAH)
      A progressive, debilitating disease characterised by an increase in pulmonary vascular resistance leading to right ventricular failure and death. PAH is defined as the presence of a mean pulmonary artery pressure (PAP) that exceeds 25mmHg at rest.  Prediction of prognosis in each individual is difficult, but certain risk factors are strongly and independently associated with survival. There are varying forms of PAH, including the primary form of the condition, termed as idiopathic PAH or familial PAH.  Pulmonary Arterial Hypertension can also be associated with other conditions including congenital heart disease, portal hypertension and connective tissue disorders (Nice, 2013).See Appendix A.

    2. 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. Causes can include myocarditis and 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. 

    3. Anaesthesia Care:
      This is broad and not restricted to pharmacological anaesthesia alone.  The assessment of the patient is performed by the anaesthetist; this often requires liaison with the cardiology team. The anaesthetist also coordinates the non- pharmacological management of this patient group peri-operatively, including “Comfort kids” techniques and the level of parental involvement.  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.

    4. Identified at-risk:
      Identified risk factors predispose individuals with PAH or Cardiomyopathy to potential complications including, prolonged hospitalisation, utilization of intensive care resources and death. Any procedure with anaesthesia needs to be treated with caution due to the potential of PAH crisis 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 

    5. 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.

    6. Pulmonary Hypertensive Crisis:
      A pulmonary 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.   

    Assessment and Inclusion Criteria

    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.
    Patients with pulmonary arterial hypertension or cardiomyopathy with the following criteria:

    • Newly diagnosed or symptomatic patients with pulmonary arterial hypertension or cardiomyopathy
    • Severe clinical and / or echocardiographic disease * (see score tables below)
    • Congestive heart failure, low cardiac output, hypotension, resting tachycardia, poor exercise capacity 
    • Known arrhythmias, particularly non-sustained ventricular tachycardia (VT)
    • Known instability related to prior interventions with anaesthesia
    • Those patients NOT receiving optimal targeted therapy
    • Receiving supplemental oxygen

    Patients assessed to have moderately to severely elevated right ventricular systolic pressure (RVSP), right atrial enlargement, pericardial effusion and displacement of the intraventricular septum should be identified as potentially high risk and particularly unpredictable patients under anaesthesia associated with adverse outcomes6.

    To help stratify the patients into different risk categories, the following criteria are to be utilised:

    PAH patients: Score system (✔) - medium risk ≤2; high risk ≤3

    Table 1: Score tables 

     Pleural / pericardial effusion  
     Treated with Epoprostenol infusion (Flolan/Veletri)  
     Severe RV / RA dilatation  
     RV Fractional Area Change < 40%  
     RVSP > 50% systemic   
     Syncope /  exercise intolerance / chest pain / arrhythmias  
     Signs of right heart failure (RHF)  
     Cardiac index < 2.0 L/min/m2  

    Cardiomyopathy:  Score system (✔) - medium risk ≤2; high risk ≥3

     RISK CRITERIA Tick (✔) 
     Treated with IV inotropes  
     Severe LV / LA dilatation  
     Tachypnoea at rest  
     Tachycardia at rest  
     LV ejection fraction < 40%  


    Streamline patients according to risk criteria cumulative score, individual treating cardiologist preference and decision at team conference as follows:
    Medium risk (cumulative score ≤ 2) PH / Cardiomyopathy patient:
    • cardiac dysfunction is present but not severe 
    • appropriate medical therapy has been instituted

    During procedures patient may tolerate procedure well but has an increased risk of instability.
    High risk (cumulative score ≥ 3 ) PH/ Cardiomyopathy patient: 
    • Symptomatic
    • severe cardiac dysfunction
    • medical therapy not yet instituted or at therapeutic dose  
    During procedures patient may not tolerate procedure and has a defined risk of instability during procedure.

    Assessment and Inclusion Criteria

    FIGURE 1:

    PHA Figure 1

    The overall management plan should minimise stress and maintain haemodynamic conditions as close to baseline, by providing adequate anaesthesia, to avoid stimuli that may trigger a PH crisis or exacerbation of cardiac dysfunction, and maintain stability. At time of discharge from Rosella or Koala to the primary care unit, the patient’s condition should have returned to their pre intervention baseline status. 

    Although not always possible, interventions with anaesthesia should be planned on the morning theatre list.  Anaesthesia management should be provided by a Paediatric Consultant Anaesthetist with appropriate expertise in the care of patients with PAH and Cardiomyopathy, to oversee both non pharmacological and pharmacological anaesthesia.  Subsequently, in the event of unexpected complications, appropriate treatment can be administered in a prompt and effective manner. 

    At time of booking by administration staff, include in free text statement detailing the need for ECMO standby if necessary. A letter or telephone call detailing admission plan will be offered to the family following booking by secretary.

    TABLE 2: Pre-Operative planning phase

    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 (PICU, Koala, cardiology, cardiac theatre,) PICU care manager, anaesthetics, haematology, theatre staff, catheter lab, perfusionists, on-call cardiologist, bed manager, play specialist. Attach CPG to email

    Document admission into Koala (or primary care unit) diary & discuss with relevant NUM’s

    Liaise with cardiac coordinator & bed manage prior to admission regarding high-risk patient

    Book procedural date & include high risk criteria into EMR booking by admin staff.

    Cardiology fellow to complete orders on EMR including medications, tests & IV fluids whilst fasting

    Ensure a cardiac anaesthetic review of patient and previous anaesthetic interventions pre-operatively. 

    Referral to play therapy for procedural sedation cases

    Play therapy  follow up for planning peri-operative management 

    In collaboration with haematology staff, support  timing & cessation of anticoagulation as an outpatient as relevant

    Alert ward pharmacist of planned admission 

    Peer review discussion with cardiologist(s) irrespective of whether procedure is cardiac or non-cardiac nature

    Book Rosella bed post-operatively for high-risk cases

    Follow up email to perfusionists re high risk patient Ht & Wt for ECMO circuit

    Once admitted, gain peripheral IV access and commence IV hydration once fasting. 


    TABLE 3: Peri-operative phase 

    Day of surgery (for all cases, both elective and emergency)

    Post-operative care

    Ensure IV hydration until tolerating oral fluids post-operatively

    Continual bedside monitoring:
    • Heart Rate ECG
    • Blood pressure (reportable limits documented)
    • Oxygen saturations (reportable limits documented)

    Ensure adequate pre-medication and promote a calm environment. Administer usual PAH medications whilst fasted. Direct transfer to anaesthetic bay from Koala pre-operatively.

    Ensure adequate sedation/analgesia is ordered and administered to promote optimal patient comfort.  

    For cases performed with PS in theatre, the CNC’s will be present to promote adequate oxygenation, sedation and clinical ambience throughout the procedure for adolescents receiving PS.

    Warning signs of PH crisis:
    • abrupt desaturation
    • systemic hypotension/pallor
    • sinus tachycardia or bradycardia
    • elevated central venous pressure (CVP)

    Direct transfer from theatre to Rosella, bypassing recovery, for 4 - 6 hours post-procedural care & observation for warning signs of acute PH crisis for high-risk patient

    If intubation is required post-operatively in the unstable patient:

    • Consider appropriate medical staff perform intubation. Liaise with consultant anaesthetist and consultant cardiologist, prior to intubation. 

    Communicate with Rosella ANUM/team if unplanned transfer to Rosella is required directly from theatre.

    Management of PH crisis:

    • administer 100% oxygen
    • institute inotropic support
    • initiate nitric oxide (NO) therapy
    • administer opioid & adequate anaesthesia
    • correct metabolic & respiratory acidosis, and treat hypothermia, if present.


    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.  


    1. Van der griend BF, Lister NA, McKenzie IM, Martin N, Ragg PG, Sheppard SJ, Davidson AJ. Postoperative mortality in children after 101,885 anesthetics and a tertiary pediatric hospital. Anesth Analg 2011; 112: 1440-4
    2. Taylor CJ, Derrick G, McEwan A, Haworth SG, Sury MRJ. Risk of cardiac catheterization under anaesthesia in children with pulmonary hypertension. Br J Anaesth 2007; 98: 657-61.
    3. WA Zuckerman, ME Turner, J Kerstein, A Torres, JA Vincent, U Krishnan, D Kerstein, EB Rosenzweig. Safety of cardiac catheterization at a center specializing in the care of patients with pulmonary arterial hypertension. Pulmonary circulation 2013;  3(4):831-39
    4. Carmosino MJ, Friesen RH,  Doran A, Ivy DD. Perioperative Complications in Children with Pulmonary Hypertension Undergoing Noncardiac Surgery or Cardiac Catheterization. Anesth Analg. 2007 March; 104(3): 521–527. 
    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   

    Companion documents


    Please remember to read the disclaimer

    The development of this nursing guideline was coordinated by Michelle Rose, Clinical Nurse Consultant, Cardiology and Bronwyn Norman, RN, Cardiology, and approved by the Nursing Clinical Effectiveness Committee. Updated September 2016.