Paediatric & end of life consideration

Question 1

True / False

1 in 5 palliative care patients will experience tachyarrhythmias in the last weeks of their life.

Answer 1

True.

Question 2

True / False

In lieu of programming, ICD shock therapy can be terminated by placing a magnet over the device.

Answer 2

True.

Question 3

True / False

End of life issues should be discussed at the time of implant.

Answer 3

True.

Question 4

True / False

The following statement is a class I indication for end of life ICD consideration.

‘Patients with refractory HF symptoms, refractory sustained VA, or nearing the end of life from other illness, clinicians should discuss ICD shock deactivation and consider the patients’ goals and preferences’.

Answer 4

True.

Question 5

True / False

The following statement is a class I indication for end of life ICD consideration.

‘ICD implantation or replacement, and during advance care planning, patients should be informed that their ICD shock therapy can be deactivated at any time if it is consistent with their goals and preferences’.

Answer 5

True.

Question 6

True / False

Cardiac pacemakers are usually placed in the subpectoral region for paediatrics.

Answer 6

False.

Typically placed in the abdomen. Abdominal fat helps protect the pacemaker during falls, knocks and bumps that are part and parcel of childhood activity. Also less likely for twiddlers syndrome.

Question 7

True / False

Most infants <15kg receive endocardial leads.

Answer 7

False.

Most receive epicardial leads as they’re easier to remove. Remember its likely the patient will have multiple revisions over their 60+ year lifetime.

Question 8

True / False

Paediactric pacing is mainly performed in the setting of SND.

Answer 8

False.

It’s mainly performed in the setting of congenital or post-surgical complete heart block and less frequently in some surgical patients with sinus node dysfunction.

Question 9

Epicardial leads in children are associated with

1. Higher chronic stimulation threshold
2. Higher lead failures and fractures
3. Early depletion of battery life

Desipte this, list the two reasons why these leads are used.

Answer 9

1. Preserves venous access for later in life
2. Easier to remove leads

Question 10

True / False

LV apical > RV apical as an epicardial pacing site for paediactrics.

Answer 10

True.

Less dyssnchrony, better haemodynamics, less progression to HF.

Question 11

True / False

Paediatric venous obstruction post PPM lead implantation is related to the ratio of cross-sectional lead area to the body surface area at implantation.

Answer 11

True.

Question 12

Why is it wise to always perform a venography in paediatric patients?

Answer 12

Large proportion of implant cases will present with congenital defects.

Thus venography will highlight potentially complex anatomy.

Question 13

List 3 paediatric cardiac anatomy abnormalities which increase implant difficulty.

Answer 13

1. Structural heart defects (TOF etc)
2. surgical repairs (Fontan, Mustard, Senning)
3. Synthetic septal patches (PFO closure)
4. Atrial baffles
5. Conduits
6. Absence of appendages
7. Obstructed venous channels
8. Persistent left superior vena cava
9. Extensive surgical fibrosis

Question 14

True / False

Endocardial pacing is not an option in patients with single ventricles.

Answer 14

True.

Access is eliminated from the systemic veins after the extracardiac conduit Fontan procedure.

Question 15

True / False

AV synchrony can add up to 15% to the paediatric cardiac output.

Answer 15

True.

Question 16

True / False

Paediatrics have lower resting and peak heart rates than do adults.

Answer 16

False.

Typically much higher than adults.

Question 17

True / False

Resting HR between 120-150bpm and peak rates >200bpm are not uncommon in paediatric patients.

Answer 17

True.

Majority of PPMs can pace at rates up to 180bpm.

Question 18

True / False

Mismatch between peak HR and the generators ability to track this HR is of no concern in paediatrics.

Answer 18

False.

Limits to MTR can result in reduced exercise tolerance, pVO2 and anaerobic threshold.

Question 19

True / False

Higher heart rates can have a negative effect on battery longevity.

Answer 19

True.

More stimulation = faster battery depletion.

Question 20

True / False

Dual chamber pacemakers are generally reserved for patients >25Kg.

Answer 20

True.

Due to size constraints a single chamber PPM will likely be used in smaller patients.

Question 21

List the 3 main implant approaches for abdominal pacemakers.

Answer 21

1. Sternotomy
2. Thoracotomy
3. Subxiphoid approach

Question 22

Which lead type is most likely to fail in paediatric patients?

1. Conventional epicardial
2. Steroid-eluting epicardial
3. Steroid-eluting endocardial
4. Conventional endocardial

Answer 22

Conventional epicardial leads.

No significant differences observed between the other 3 types.

Question 23

Why are active fixation leads preferable to passive fixation in paediatrics.

Answer 23

Easier to remove / revise.

High likelihood of revision as ~50% of leads will have failed by the 15yr mark.

Question 24

What is an atrial loop and why is it used when implanting paediatric endocardial ventricular leads?

Answer 24

Employing extra redundancy of the ventricular lead such that it forms a loop in the atrium.

This is to ensure better long term outcomes as the redundant slack will be used as the patient grows.

Question 25

# True / False Paediatric patients comprise \<1% of all PPM implants.

Answer 25

True. ## Footnote *Indeed paediatric implant centres may only implant circa 10 systems per year.*

Question 26

Describe what is meant by antibody positive vs. antibody negative for paediatric AVB.

Answer 26

* Antibody positive - Fetus develops AVB respondent to maternal antibodies / single stranded RNA crossing placenta and damaging conduction system. * Antibody negative - Develops later in life, AVB is more likely to be a progressive disease.

Question 27

# True / False Most common cause of AVB in congenital patients is respondent to damage from cardiac surgery.

Answer 27

True.

Question 28

# True / False Paediatric incidence of AVB post surgery is \<2%.

Answer 28

True.

Question 29

# True / False Typically paediatric patients with transient AVB will recover \<5 days.

Answer 29

False. ## Footnote *If truly transient, patient will most likely recover within 10 days. After 10 days PPM insertion is likely treatment.*

Question 30

# True / False After \<7yrs of paediatric pacing, the following may be observed with epicardial implants. 1. ~13% partial venous occlusion 2. ~12% complete venous occlusion

Answer 30

False - these statistics are true of transvenous implants. Epicardial does not use venous access. ## Footnote *Bar-Cohen Y, Berul CI, Alexander ME, et al. Age, size, and lead factors alone do not predict venous obstruction in children and young adults with transvenous lead systems. J Cardiovasc Electrophysiol 2006.*

Question 31

# True / False Venous stenosis is always asymptomatic.

Answer 31

False. ## Footnote *May present with venous congestion and swelling of the ipsilateral arm.*

Question 32

# True / False Paed endocardial systems have similar complications as adults, however the incidence is much lower.

Answer 32

False - incidence of complication in paediatrics is far higher. ## Footnote *Smaller size, more complex anatomy, fewer numbers, less experienced operators, more active lifestyle are all recognised contributors.*

Question 33

# True / False VVIR is the most common pacing mode in small children.

Answer 33

True. ## Footnote *Children tolerate AV dyssnchrony better than adults and there's a preference to implant the least amount of hardware possible early in a childs life. Thus single chamber devices are standard.*

Question 34

# True / False Unpaced postoperative heart block carries a high mortality in paediatric patients.

Answer 34

True.

Question 35

Why should RVp should be minimised in paediatrics and how can it be achieved in SR systems?

Answer 35

Higher rates = More RVp over time. This is potentially determinental as per MOST study findings. ## Footnote *Higher pacing % could increase risk of pacemaker syndrome and ventricular dysfunction. Thus program a lower HR and/or remove rate response function to encourate intrinsic conduction. Important to balance HR and respondent exercise tolerance with Vp%.*

Question 36

List 3 reasons why CRT outcomes in paediatrics poor.

Answer 36

1. Paeds rarely present with LBBB and QRS \>150ms, which are markers of CRT success 2. CRT in adults is mainly ischemic based, in paeds its congenital or progressive cardiomyopathies 3. CRT isn't shown to benefit the failing RV, which is the most likely ventricle to fail in congenital scenarios

Question 37

# True / False Paediatric patients most likely to benefit from CRT are those with impaired LV function who are being paced from the RV (Thus presenting with LBBB morphology on ECG).

Answer 37

True.

Question 38

# Yes / No Can patients/guardians request discontinuation of tachy/brady therapy, even when not terminally ill?

Answer 38

Yes. ## Footnote *It is unethical to provide any treatment against a persons will.*

Question 39

# True / False All patients are asked about their wishes to cease device therapy at the time of implantation.

Answer 39

False. ## Footnote *Only a small minority of patients are asked this question, when in fact all patients should be asked to ensure appropriate action is taken should the need arise.*

Question 40

What should be ascertained when any request to end critical treatment is requested.

Answer 40

Cognitive competence and the ability to comprehend the consequences of changing device settings. ## Footnote *E.g. The difference between ending tachycardia therapy and/or pacing therapy in a dependent patient.*

Question 41

# True / False Withdrawal of Tach/Brady therapy is not akin to withdrawal of mechanical ventilators, heamodialysis or other life support machines and should never be performed.

Answer 41

False.

Question 42

# True / False Withdrawal of Tach/Brady therapy is likened to assisted dying, analogous to voluntary euthanasia.

Answer 42

False.

Question 43

# True / False A person's underlying condition is deemed the cause of death following treatment withdrawl.

Answer 43

True.

Question 44

# True / False It is unethical to withdraw treatment that becomes part of the patients self - I.e. organ transplant.

Answer 44

True. ## Footnote *This does not apply cardiac devices.*

Question 45

# True / False From an ethical and legal standpoint, the following statement is true. 'Refusing cardiac device implant and withdrawal of cardiac device therapy are one and the same'.

Answer 45

True.

Question 46

What should be done if personal and professional opinions differ between clinician and patient with respect to withdrawal of therapy?

Answer 46

HRS guidelines state clinicians are obligated to arrange for alternative provision of care in cases of conscientious objection that cannot be resolved.

Question 47

# True / False Withdrawing bradycardia support is unlikely to result in instantaneous death but rather symptoms respondent to inadequate cardiac output like dizziness and syncope.

Answer 47

True.

Question 48

# True / False In patients with a do not resuscitate order in force, ICD deactivation should be seriously considered.

Answer 48

True.

Question 49

# True / False An active pacemaker will not affect the timing or circumstances of death as a patient typically becomes acidotic before cardiac arrest, rendering the pacemaker ineffective.

Answer 49

True.

Question 50

# True / False Cardiac device deactivation is an extension of patients' self-determination regarding their own treatment.

Answer 50

True.