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330
Key Questions in CONGENITAL CARDIAC SURGERY
33 Describe the principles of monitoring the neurological
status of children on the paediatric intensive care unit
Although the best method to assess a child’s neurological status is
•
to allow them to rouse from sedation, this is not always possible or
appropriate in some situations.
All children on the PICU receive standard neurological monitoring,
•
including:
a) age-specific regular neurological observations, such as
Glasgow Coma Scale (GCS) and pupillary responses;
b) sedation, COMFORT-B and pain scores;
c) sedation breaks to assess neurology;
d) clinical examination of the neurological system.
Additional tools for use at the beside include:
•
a) bispectral index (BIS) monitor — to assess the depth of
anaesthesia;
b) cerebral function-analysing monitor (CFAM) — which records
an amplitude integrated electroencephalogram (aEEG);
c) formal electroencephalography (EEG) — which can be carried
out at the bedside but requires specialist neurophysiology
interpretation;
d) cranial ultrasound imaging — which is useful in infants with an
open fontanelle. It allows rapid detection of significant
intraventricular haemorrhage and can suggest severe
ischaemia, but can often miss smaller changes.
Other imaging modalities include:
•
a) computed tomography (CT) — which is useful in an
emergency situation, as it is quick to perform and gives good
rapid information about bleeding and infarction. It may,
however, miss some subtle pathologies and requires radiation
to a child’s brain;
b) magnetic resonance imaging (MRI) — which provides much
more detailed information about brain matter, particularly
hypoxic brain injury and does not involve radiation. It does,
however, take a lot longer to perform, with less access to the
patient during the scan, so is only suitable for
haemodynamically stable patients. In addition, it cannot be
used in those with contraindications, such as with a certain
type of pacemaker
áå=ëáíì
.
8 Paediatric cardiac intensive care
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34 What are the principles of point-of-care ultrasound?
Point-of-care ultrasound (POCUS) refers to a portable bedside
•
ultrasound performed by the intensivist to scan relevant body
systems.
It differs from radiology ultrasonography or cardiology
•
echocardiography in that it is designed to answer a pre-defined
question with a yes or no answer, and it is not intended to provide
detailed anatomical and physiological assessments.
The advantages of POCUS over traditional imaging include being
•
immediately available to the attending clinician at the patient’s
bedside, allowing real-time pathological changes to be seen with no
lag time. In addition, it can be repeated frequently to monitor
progression or assess the impact of an intervention, and improves
the safety and accuracy of certain interventional procedures.
Many body systems can be scanned using the POCUS binary
•
questions, including:
a) cardiac POCUS:
i) is the ventricle contracting well?
ii) is the ventricle empty or full?
iii) is there a pericardial effusion?
b) respiratory POCUS:
i) is there pulmonary oedema?
ii) is there a pleural effusion?
iii) is there a pneumothorax?
iv) is there a pleural effusion?
v) is there consolidation?
c) abdominal POCUS:
i) is there ascites?
331
In the cardiac population, a particularly useful role of respiratory
•
POCUS is the assessment of extravascular lung water content and
pulmonary oedema, either prior to extubation or on presentation.
In patients with pulmonary oedema, extensive B-lines with a normal
•
pleural line can be seen. These B-lines resolve in real time with
resolution of the oedema, unlike the chest X-ray changes which can
lag behind the clinical picture (Figure 11).
332
Key Questions in CONGENITAL CARDIAC SURGERY
A
Subcutaneous tissue
Pleural line
Rib shadowing with dropout
either side of the image
A-line and fascia reflections
B
Subcutaneous tissue
Pleural line
B-lines (multiple)
Rib shadowing (bat wing)
Figure 11. Lung point-of-care ultrasound taken from the anterior chest
wall demonstrating: A) normal lung and B) pulmonary oedema.
8 Paediatric cardiac intensive care
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35 Describe the principles of nutrition for cardiac patients
on the paediatric intensive care unit
The nutritional status of the child plays an integral part in the
•
metabolic response to injury and critical illness.
Malnutrition has been associated with increased mortality and
•
morbidity, including longer PICU stay. Poor nutrition in a cardiac child
can prevent growth and make further surgery more difficult. Nutrition
should be optimised pre-operatively, if possible.
Regular reviews by dieticians are vital to ensure metabolic demands
•
are being met and appropriate monitoring is taking place.
Overfeeding can also cause complications, which is why daily energy
requirements should be calculated accurately (Table 8).
Table 8. Complications of malnutrition and overfeeding.
bССЙЕнл=зС=йззк=емнкбнбзе
• Impaired immunity
• Increased risk of infection
• Impaired wound healing
• Constipation/ileus
• Specific vitamin/mineral deficiency
• Hypoproteinaemia and oedema
Establishing sufficient nutritional support and delivering estimated
•
energy requirements can be challenging due to fluid restrictions,
feed interruptions (such as for procedures), gastric intolerances
(such as ileus) and gastrointestinal pathology (such as ischaemic
colitis).
Factors affecting nutrition on the PICU include:
•
a) increased metabolic requirements, such as pyrexia, sepsis;
b) poor intake, such as fluid restriction;
c) increased losses, such as poor absorption, sweating;
d) increased exertion, such as respiratory effort.
bССЙЕнл=зС=зоЙкСЙЙЗбеЦ
• Fluid overload and oedema
• Increased risk of infection
• Diarrhoea
• Increased gastric losses/poor absorption
• Obesity
333
Early enteral feeding is preferable despite the presence of critical
•
illness to maintain gut integrity and reduce the risk of ileus.
334
Key Questions in CONGENITAL CARDIAC SURGERY
Few contraindications for enteral feeding exist and include
•
necrotising enterocolitis (NEC), ischaemic colitis, gastrointestinal
bleeding or mechanical bowel obstruction.
Multiple inotropic support is not an absolute contraindication but
•
nutrition should be carefully reviewed in the context of the whole
clinical picture.
Certain children are considered high risk for abdominal pathology or
•
feeding complications and should be fed cautiously. Broadly, these
are children who have or have had compromised systemic blood flow
and present concerns regarding splanchnic hypoperfusion. These
children are at significantly higher risk of developing ischaemic colitis
and include:
a) cardiac lesions with low systemic output, such as HLHS,
critical aortic stenosis, interrupted aortic arch and coarctation
of the aorta;
b) prolonged cardiopulmonary bypass and aortic cross-clamp
times, particularly in small infants;
c) children on ECMO;
d) previous abdominal pathology;
e) long-term poor nutrition, where there is a risk of refeeding
syndrome.
High-risk children can be fed cautiously after a period of stability.
•
When this is not feasible, parenteral nutrition should be considered
if enteral nutrition has been suspended for 7 days. Early parenteral
nutrition has been associated with poorer outcomes. Many PICUs
have high-risk feeding protocols.
There needs to be a low threshold for stopping feeds, performing an
•
abdominal X-ray or ultrasound, and obtaining a general surgical
review for early detection of ischaemic colitis in these children.
Parenteral nutrition, although vital for some patients, is not without
•
disadvantages, and includes:
a) venous thrombosis and difficulties obtaining long-term IV
access;
b) line sepsis;
c) metabolic abnormalities or electrolyte imbalances;
d) regular blood sampling, including renal and liver function,
lipids, amino acids and triglycerides;
e) imbalance of trace elements;
f) financial implications.
8 Paediatric cardiac intensive care
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36 What considerations must be given to the child who is
dying?
The end-of-life care for a child and their family on the PICU is a
•
difficult time for all involved.
Unexpected death is a very different scenario compared to the
•
situation where the perceived best interest of the child rests with
redirection of care towards symptom management and an end-of-life
care pathway.
Prior to discussing the topic with the family, it is important to obtain
•
agreement of the multidisciplinary team. The consensus decision
needs to be reached that treatment aimed at continuing life is no
longer in the best interest of the child.
These can be extremely difficult and complex discussions and
•
decisions to be made, and they may involve second opinions, ethical
and legal reviews, and escalation to senior hospital management.
It is important for the family to understand the clinical situation. Time
•
is important and this is not a topic that can be rushed.
Some important considerations include:
•
a) involvement of the palliative care services;
b) organ donation, when clinically indicated;
c) memory boxes, such as handprints, footprints and
photographs, to support parents through the process;
d) supporting religious beliefs and requirements are paramount;
e) careful consideration for a post-mortem;
f) recording of all phases of this process, as well as routine
clinical practice and a record of communication are vital, as
legal investigations may arise;
g) performing any relevant investigations to give a diagnosis (e.g.
skin and muscle biopsies).
335
When it comes to withdrawing life-sustaining treatment, it is
•
important to consider:
a) where this will happen — home, hospice or hospital;
b) when this will happen;
c) who will be present from the family and the PICU team;
d) symptom management plans.
After the child has died, it is important to consider:
•
a) emotional support for the team involved and relevant debriefs;
b) reporting and legal documentation, such as death certification;
Key Questions in CONGENITAL CARDIAC SURGERY
c) ongoing follow-up and support for the family;
d) any outstanding investigation results to follow up, such as
genetic reports.
Acknowledgements
The chapter authors would like to thank Dr. Donna Austin BN(Hons)
RN(Child) PhD and Dr. Iain Macintosh MBChB FRCPCH FFICM for their
review and critical appraisal of this chapter.
Recommended reading
336
1. Bronicki RA, Penny DJ, Anas NG, Fuhrman B. Cardiopulmonary interactions.
`êáí=`~êÉ=jÉÇ=
2. Chandler HK, Kirsch R. Management of the low cardiac output syndrome following
surgery for congenital heart disease.
3. Hardwick JA, Griksaitis MJ. Fifteen-minute consultation: point of care ultrasound in the
management of paediatric shock.
41.
4. Hoffman TM, Wernovsky G, Atz AM, Kulik TJ, Nelson DP, Chang AC, Bailey JM,
Akbary A, Kocsis JF, Kaczmarek R, Spray TL, Wessel DL. Efficacy and safety of
milrinone in preventing low cardiac output syndrome in infants and children after
corrective surgery for congenital heart disease.
5. Jenkins KJ, Gauvreau K, Newburger JW, Spray TL, Moller JH, Iezzoni LI. Consensus-
based method for risk adjustment for surgery for congenital heart disease.
`~кЗбзо~лЕ=pмкЦ
6. Kaestner M, Schranz D, Warnecke G, Apitz C, Hansmann G, Miera O. Pulmonary
hypertension in the intensive care unit. Expert consensus statement on the diagnosis
and treatment of paediatric pulmonary hypertension. The European Paediatric
Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.
102 Suppl 2: ii57-66.
7. Pittman JAL, Ping JS, Mark, JB. Arterial and central venous pressure monitoring.
^еЙлнЬЙлбзд=`дбе
8. Tharakan JA, Sukulal K. Post-cardiac surgery junctional ectopic tachycardia: a ‘hit and
run’ tachyarrhythmia as yet unchecked.
2016; 17(8 Suppl 1): S182-93.
`мкк=`~кЗбзд=oЙо
^êÅÜ=aáë= `ÜáäÇ=bÇìÅ=mê~Åí=bÇ
2002; 123(1): 110-8.
2004; 42(1): 13-30.
^ее=mЙЗб~нк=`~кЗбзд
2016; 12(2): 107-11.
`бкЕмд~нбзе
2003; 107(7): 996-1002.
2021; 106(3): 136-
2014; 7(1): 25-8.
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eÉ~êí=
mÉÇá~íê
2016;
fåí
Chapter 9
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Adult congenital cardiac intensive care
Miriam Conway, Susanna Price
1 Where should critically ill adult congenital heart
disease patients be managed?
The American Heart Association (AHA) and European Society of
•
Cardiology (ESC) advise that adult congenital heart disease (ACHD)
patients are managed in specialist centres where they have access
to specific resources and expertise.
The physiology of the critically ill ACHD patient can be complex and
•
expert input is essential when managing these individuals.
Where transfer/admission to a specialist centre is precluded due to
•
the clinical status of the patient, expert advice should be sought until
transfer is possible, as they may need surgical intervention (for
example in endocarditis), but their management prior to transfer may
still require ACHD expertise.
337
2 What are the most common indications for ICU
admission in the adult congenital heart disease
population?
The commonest indication for admission to the ICU is recovery post-
•
cardiac surgery.
Medical acute/emergency admissions are similar to those in acquired
•
cardiac disease, although there are additional indications for
admission that reflect the higher incidence of right-sided pathology in
this patient population. The most common indications for medical
admission to intensive care include:
a) arrhythmia/cardiac arrest;
b) left ventricular failure;
c) endocarditis;
d) right ventricular failure;
e) haemoptysis;
f) syncope.
338
Key Questions in CONGENITAL CARDIAC SURGERY
3 What are the risk factors for increased perioperative
mortality and morbidity in the adult congenital heart
disease patient population?
Patients with complex congenital lesions carry a significantly higher
•
postoperative mortality than those with simple lesions despite having
similar APACHE II scores (Figure 1).
Figure 1. Surgical scoring and risk stratification in adult congenital heart
disease (ACHD) patients. The graphs compare the observed outcomes of
surgery in ACHD patients shown as a function of pre-operative risk
assessment using the: A) Parsonnet; B) Logistic EuroSCORE; and C)
Ontario systems. Scores are divided into low, fair, moderate, high and
extremely high-risk categories. * = p<0.05.
Perioperative mortality is increased in the presence of pre-operative
•
abnormalities, including deranged thyroid function tests, and
elevated serum creatinine and bilirubin levels.
9 Adult congenital cardiac intensive care
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4 What are the main differences between congenital
cardiac intensive care and non-congenital cardiac
intensive care?
The normal physiological status of an ACHD patient is often
•
significantly different from those with acquired cardiac disease.
The relatively high incidence of right ventricular dysfunction presents
•
a particular challenge, as it is associated with renal and hepatic
impairment, gastrointestinal dysfunction, and the need for prolonged
respiratory support and intensive care admission.
Whilst standard management principles do apply to many acutely
•
unwell ACHD patients, certain trends in intensive care
management may not be well tolerated by this patient population,
including:
a) volume resuscitation;
b) systemic oxygen delivery;
c) sedation;
d) use of vasoactive drugs;
e) application of positive airway pressure ventilation.
339
Considering how supportive and therapeutic interventions may
•
affect the patient, anticipating potential pitfalls is important in every
case.
5 What are the principles in managing a critically ill
ACHD patient?
The first step in assessing a critically ill ACHD patient (as with every
•
critically ill patient) is to know the indication for admission.
In addition to the standard principles relating to the assessment and
•
management of the critically ill patient, there are a number of
additional factors when assessing the ACHD patient that should be
considered in turn, including:
a) knowledge of the underlying cardiopulmonary anatomy;
b) understanding the normal physiology of the patient;
c) appreciation of how any supportive or therapeutic interventions
may affect the circulation;
d) anticipating the potential pitfalls related to critical care
monitoring, investigations and interventions.
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