In this section
Note: This guideline is currently under review.
Definition of Terms
Assessment & Inclusion Criteria
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.
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:
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
Cardiomyopathy: Score system (✔) - medium risk ≤2; high risk ≥3
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 institutedDuring 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.
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
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)
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:
Communicate with Rosella ANUM/team if unplanned transfer to Rosella is required directly from theatre.
Management of PH crisis:
Intellectual ability and cognitive development with regard to a child’s cooperation should guide the implementation of procedural sedation or administration of general anaesthesia.
The evidence table for this guideline can be
Appendix A: TABLE 1. UPDATED CLINICAL CLASSIFICATION OF PULMONARY HYPERTENSION FROM DANA POINT, 2008 (1)
Please remember to
read the disclaimer
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.