In this section
Definition of Terms
Assessment & Inclusion Criteria
Pre and Post Procedural Care
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.
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
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.
A disease of the myocardium associated with cardiac dysfunction, and is classified into groups as listed below:
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).
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.
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.
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
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.
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.
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).
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.
TABLE 2: Risk factor guide for
Assessment and Inclusion
Criteria Figure 1:
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:
TABLE 3: Anaesthetic Procedure timeline
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.
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.
The staff whom are allocated for direct
care of patient are responsible to ensure below tasks are completed
Continual bedside monitoring:
Causes/ known stimuli for cardiac decompensation:
Warning signs of deterioration:
Management of cardiac decompensation during a procedure:
Process of discharge: treating team or on-call team to assess patient prior to discharge and make appropriate follow-up arrangements
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 for this guideline can be viewed here.
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.