Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Paediatric cardiology > Congenital diagnosis and management > Flashcards

Congenital diagnosis and management Flashcards

1
Q

Describe tricuspid atresia

A

Tricuspid valve never develops = no connection between RA and RV
Associated with ASD + large VSD + PDA
RV is hypoplastic (no flow no grow!)
Cyanotic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Describe circulation in tricuspid atresia

A

SVR enters RA, crosses ASD, mixes with PVR in LA, through MV (often hypoplastic) into LV

From LV, blood almost freely flows through VSD into hypoplastic RV

From the ‘common’ ventricles it can go to either (1) Aorta or (2) Pulmonary artery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Why is it ‘good’ to have PS in tricuspid atresia

A

We are ‘hoping’ for a concomitant degree of pulmonary stenosis, because pulmonary circulation is much lower resistance than systemic circulation, therefore if no PS there will be preferential blood flow into the pulmonary side

This worsens systemic perfusion and leads to pulmonary over-circulation, remodelling, and therefore pHTN.

Importantly, if pHTN develops, this would preclude Fontain palliation!!

On the other hand, if there is LV outflow tract obstruction this can also lead to systemic hypoperfusion and encourage preferential pulmonary circulation, similarly worsening the situation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Key management principles in tricuspid atresia

A
  • Avoid too little systemic outflow
  • Avoid pulmonary overcirculation
  • Saturations and mixing of blood
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Neonatal management of tricuspid atresia

A

Depends on principles of (1) managing the balance of pulmonary/systemic flow, and (2) managing O2 sats

  1. Maintain open duct - to improve pulmonary flow if there is excessive PS and therefore improve circulation to lungs
  2. Atrial septostomy - if no ASD, also to improve pulmonary flow if there is excessive PS and therefore improve circulation to lungs
  3. PA band - increase pulmonary vascular resistance and therefore essentially ‘create’ PS and avoid over-circulation to lung
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Fontain Stage 1 timing and details

A

0-6 months

Norwood procedure
o Close PDA
o Create or widen ASD
o Create neo-aorta by re-rooting PV+proxPA into aorta - Damus-Kaye-Stansel (DSK) anastomosis
o Create new venous flow into PA with Blalock-Thomas-Taussig shunt (R SubC to PA) or Sano shunt (RV to PA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

End result of Stage 1 Fontan (Norwood procedure)

A

 The heart pumps blood into one single common root
 Blood flow to PA comes from a shunt from the aorta or RV
 There is still atrial mixing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the BTT & Sano shunts

A

BTT - R SubC to PA
Sano - RV to PA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Fontan Stage 2 details

A

Bidirectional Glenn
o BTS/SanoS removed
o SVC cut above RA connection
o Connect SVC to PA directly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

End result of Fontan Stage 2 (Bidirectional Glenn)

A

 Blood flow to the lungs is now entirely via the SVC
 There is still atrial mixing from IVC supply, but SpO2 improved

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Fontan Stage 3 details

A

Usually at 2-5y

IVC connection to the pulmonary artery made

 Extracardiac = SVC to PA, IVC to PA. RA is entirely bypassed
 Total cavo-pulmonary conduit = connect SVC and IVC to PA; make a small fenestration in IVC to re-enter RA (to protect pulmonary circulation).
 Close previous BT/Sano shunt

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Classic Fontan risks

A

Massive RA dilation leading to significant atrial tachycardias; specifically intra-atrial re-entry tachycardia

High blood stasis leading to thrombus risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

In addition to normal Fontan, what did ‘Classic Fontan’ entail

A

Not done anymore

= connect RA to PA, and close ASD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

List ASD types with %

A
  • Secundum ASD (80%)
  • Primum ASD (15%);
  • Superior sinus venosus defect (5%)
  • Inferior sinus venosus defect (<1%)
  • Unroofed coronary sinus (<1%)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Shunting in ASD

A

ALL start as L>R shunt, with worsening RVF, these can reverse (R>L) = Eisenmenger’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Secundum ASD

A

located in the region of the fossa ovalis and its surrounding (= patent foramen ovale)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Primum ASD

A

== atrioventricular septal defect (AVSD) with communication on the atrial level only. located near the crux, AV valves are typically malformed (total AV canal defect), resulting in various degrees of regurgitation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Superior sinus venosus ASD

A

located near the superior vena cava (SVC) entry, associated with partial or complete connection of right pulmonary veins to SVC/RA`

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Inferior sinus venosus ASD

A

located near the inferior vena cava (IVC) entry].

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Unroofed coronary sinus ASD

A

separation from the left atrium (LA) can be partially or completely missing.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Typical history in ASD

A
  • Often asymptomatic until adulthood
  • Symptoms beyond 4th decade: exertional SOB, reduced functional capacity, RHF then cyanosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Exam in ASD

A
  • Ejection systolic murmur with fixed split S2 (inspiration and expiration)
  • Cyanosis if shunt reversal
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Investigation in ASD

A

Echo: quantify shunt volume and severity, size of ASD, TOE might be required for secundum defects (posterior) +/- CMRI

P Cath if suspicion of raised PAP +/- exercise testing if suspect PAH
o Can measure QpQs on cath using oximetry values using Fick principle
 VO2 = (CaO2 - CvO2) * Q
 Qp:Qs = (SatAorta-SatSVC)/(SatPulmonaryV- SatPulmonaryArt)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Management of ASD

A
  • Optimize medical therapy
  • If: RV overload or paradoxical embolism OR PAH, closure
  • Eisenmenger’s is beyond possible closure – at that stage, closure of shunt will make patient sicker as it will further increase pulmonary pressures by closing the ‘outlet’ into the L side
  • Patients who do not undergo closure have worse outcomes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Closure of ASD types

A

o Surgical closure if complex lesion / primum ASD or decompensated
o Device closure if secundum ASD with amenable morphology (needs to have good margins for closure device with 5mm buffer area around)
o If PAH present (PVR>1/3 SVR, PASP>1/2 SBP), needs to be carefully considered w/ ACHD/PH team

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Five types of VSD

A

Classified based on location
- Type 1 = subaortic
- Type 2 = in membranous septum (perimembranous, paramembranous, conoventricular)
- Type 3 = inlet (often within AVSD)
- Type 4 = muscular (towards ventricular apex)
- Gerbode = left ventricular to right atrial communication due to absence of AV septum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Typical history in VSD in children

A

Asymptomatic or symptomatic depending on size and where.

Large VSDs might cause fatigue, sweaty in feeds, recurrent RTIs, CHF and failure to thrive.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Adult with VSD - typical history

A

With advancing age, the following problems might occur:
- Double chamber RV (mostly perimembranous, due to the jet lesion on RV endothelium)
- Prolapse of RCC of aortic valve leading to significant AR due to unstable septum
- Arrhythmias
- Late LV dysfunction, heart block or heart failure
- Endocarditis risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Exam findings in VSD

A

Small defect: blood flowing through VSD results in loud harsh blowing high pitched pansystolic murmur. May be associated with parasternal thrill.

Large defect: parasternal heave due to RVH, systolic murmur softer. Additional diastolic murmur due to increased flow through the mitral valve.

Intensity of murmur inversely proportional to severity of the shunt. Harsh, holosystolic murmur

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

harsh blowing high pitched pansystolic murmur. May be associated with parasternal thrill.

A

Small VSD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

R parasternal heave, soft systolic murmur

A

Large VSD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Investigations for VSD

A

Doppler with echo demonstrating flow across septum.

CXR may show cardiomegaly, prominent pulmonary vasculature

ECG may show RVH/LVH

VO2 testing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Rule of thumb re: which ventricle is strained by pre- vs post-tricuspid shunt

A

Pre-tricuspid shunt - LV primarily loaded - generally do not develop pHTN / RV failure

Post-tricuspid shunt - RV primarily loaded - greater risk of pHTN / RV failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Pre-tricuspid shunt examples

A

ASD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Post-tricuspid shunt examples

A

AVSD
VSD
APW
PDA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Indications for intervention in VSD

A

o RCC prolapse
o PAH (PVR>3WU) – careful balance, if PAH severe might be too high risk for closure
o QpQs >1.5 or <1 (ratio of total pulmonary blood flow to total systemic blood flow, normal = 1, L:R shunt >1, whereas R>L shunt <1)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Anatomy in TAPVR

A
  • The PV do not connect to the LA, but connect to the SVC (or IVC, or innominate)
  • TAPVR HAS to be associated with ASD (most sinus venosus) - allows movement of blood in to LV
  • Therefore blood flow IVC > RA > RV > PA > PV > RA > LA > Body (purple)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Blood flow in TAPVR

A

IVC > RA > RV > PA > PV > RA > LA > Body (purple)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Timing of surgery in TAPVR

A

Neonatal period

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Spectrum of APVR

A

Can go anywhere from:

One single anomalous pulmonary vein return (e.g, into IVC, Scimitar syndrome), which leads to a degree of increased RV preload but usually does not require intervention

to

ALL pulmonary veins connect to right-sided circulation, which is a duct-dependent lesion and a cyanotic congenital heart disease requiring intervention in the neonatal period

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Types of surgery in TAPVR

A

Overall principle is intracaval baffling of anomalous PV return back to LA

OR

direct reimplantation of anomalous PV to LA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What is a Warden procedure

A

For: sinus venosus ASD + TAPVR

Involves:
1. Transection of SVC above the origin of PV
2. Mouth of SVC redirected with intracardiac baffle through ASD to shuttle blood from PV through SVC base into LA
3. Upper SVC segment directly connected to RA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Describe a PDA

A

Persistent communication between the proximal left PA and the descending aorta just distal to the left subclavian artery.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Typical presentation of PDA in adults

A

In adults with no known ACHD, usually isolated finding
PDA originally results in L–R shunt and LV and LA volume overload

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

‘machinery-like’ murmur in late diastole and early systole, sounds like a steam train, ff-p-ff p. Loudest at LUSE.

A

PDA

46
Q

PDA murmur

A

‘machinery-like’ murmur in late diastole and early systole, sounds like a steam train, ff-p-ff p. Loudest at LUSE.

47
Q

Indications for intervention in PDA

A
  • LV volume overload
  • PAH (PVR>3WU) or large L>R shunt (QpQs >1.5)
48
Q

Are pregnancy and sports ok in people with PDA

A

Unless LV overload or PAH, sports and pregnancy ok

49
Q

Physiology behind duct open/closing

A

Fetal patency ensured by ductal production of PGE2 but this falls at birth and DA should close within 2 weeks.

50
Q

Timing and types of closure

A

Normal closure at 15h, some have it after first breath (funcitonal closure, contraciton of SM).

True anatomical closure takes weeks (permanent closure, proliferation of fibroblasts).

51
Q

Associations of PDA

A

Prematurity, perinatal distress, fluid therapy, hypoxia.

May have congenital rubella, chromosomal abnormality, fetal alcohol syndrome, maternal amphetamines, FHx.

52
Q

History in PDA in child

A
  • Small: asymptomatic
  • Medium: low exercise tolerance, breathless, hoarse cry/cough as child, LRTI comon and alectasis/pneumonia.
  • Large: symptoms of HF, dyspnea and failure to thrive. Neonatal aopnea/bradycardia.
53
Q

Examination in PDA

A
  • Premature: rough systolic murmur in left sternal border. Bounding pulses.
  • Small: normal pulses, BP continuous machinery murmur and thrill below left clavicle (pressure gradient between aorta and pulmonary vessel.
  • Medium: wide pulse pressure, bounding peripheral pulse, LRTI.
  • Large: absent thrill, murmur in systole only as pressures of aorta and arteries are equal in diastole.
  • CHF: tachycardia, tachypnoea, respiratory distress, displaced apex, cool periph.
54
Q

Investigations in PDA

A

CXR: cardiomegaly, signs of HF
ECG: LVH
Doppler Echo: diagnostic to confirm patency

55
Q

Management of PDA

A

Preterm: conservative if asymptomatic, monitor, fluid restrict, PGE2 inhibitor to encourage closure

Surgical ligation if symptomatic

In term children, minimal access closure in cathlab ~ >1yo

56
Q

Potential long term risks of PDA

A

High risk of PDA associated endocarditis, preterm (IVH, bronchopulmonary dysplasia, CHF).

pHTN + Eisenmenger syndrome.

Emboli. Untreated mortality 20%, with closure minimal

57
Q

Describe HLHS

A

Underdevelopment or complete absence of L side of the heart, with large ASD usually or other form of shunt

Managed neonatally using Fontan procedure (or modifications thereof)

58
Q

What is the Fontan procedure useful for

A

broadly applicable to multiple congenital abnormalities characterised by malformation or lack of one or more chambers of the heart

59
Q

Final circulation in Fontan (example in HLHS)

A

o Dexoygenated blood from body into SVC/IVS direct into P trunk (Passive flow)
o Through lungs
o Oxygenated blood returned from lungs to hypoplastic LA
o Flows through ASD into RA
o Into RV through TV
o RV pumps into aorta

60
Q

Typical findings on examination of Fontan patient

A

RV heave
Mild non pulsatile JV distension

High JVP + Hepatomegaly -> think of Fontan obstruction or failure

61
Q

Long term outcomes of Fontan: adolescence

A

Uneventful course in childhood and adolescence usually

62
Q

Long term outcomes of Fontan: adulthood

A

progressive decline in exercise performance and heart failure, cyanosis, chronic venous insufficiency, and development of important arrhythmias, especially in patients with a classical Fontan operation

63
Q

Long term outcomes of Fontan: arrhythmias

A

By 10 years after a Fontan operation, ∼20% of patients have supraventricular tachyarrhythmias (including typically IART and atrial flutter, but also AF and focal AT)

Especially high risk in ‘Classic’ (Right atrium to pulmonary artery) Fontan

64
Q

Long-term outcomes of Fontan: abdominal

A

Liver disease: hepatic congestion and severe fibrosis, portal hypertension and risk of hepatocellular carcinoma

Protein losing enteropathy: diagnosed by low serum albumin and elevated α1-antitrypsin levels in the stool. Poor progrnosis

Plastic bronchitis and lymphatic dysfunction

65
Q

Description of CoA

A

Narrowing of aorta which can occur as discrete stenosis or as a long, hypoplastic aortic (arch) segment

Typically, CoA is located in the area where the ductus arteriosus inserts, and only in rare cases occurs ectopically (ascending, descending, or abdominal aorta)

Defined by its relation to the ductus arterious.

Preductal, ductal or potductal.

66
Q

CoA associated diseases

A
  • BAV (up to 85%)
  • Ascending aortic aneurysm
  • SubAS, or SupraAS,
  • (Supra)mitral valve stenosis (including parachute mitral valve),
  • Shone complex (supravalvular mitral membrane, parachute mitral valve, muscular or membranous subvalvular aortic stenosis and coarctation of aorta)
  • Turner syndrome and Williams–Beuren syndrome.
  • Extracardiac vascular anomalies - anomalous origin of the right subclavian artery (in 4–5% of cases), collateral arterial circulation, and intracerebral aneurysms (in up to 10%)
67
Q

Symptoms of CoA

A

Headache, nosebleeds, dizziness, tinnitus, shortness of breath, abdominal angina, claudication, and cold feet.

Signs of left heart failure

Intracranial haemorrhage (from associated berry aneurysm), IE, aortic rupture/dissection, premature coronary and cerebral artery disease, and associated heart defects

68
Q

Aetiology of CoA

A

Failure of normal development of the left 4th and 6th aortic arches (6/40)

Related to Turner, rubella, VSD, PDA, hypoplastic left heart (HLH), interruption of AA.

69
Q

Symptoms of CoA in children

A

Neonatal: in ductal/postductal forms, when ductus arteriosus closes, the sudden decrease in outflow leads to pressure build in the LV, leading to sudden LVF (dyspnea, pulmonary oedema etc).

Infant: symptoms depend on severity and collaterals. Preductal has good collaterals, postductal less. May be asymptomatic until adolescence if good collaterals.

70
Q

Exam findings in CoA

A

Nodding type head movements with HR, delayed or absent femoral pulse, RR delay, difference in BP in both arms, (>20mmHg).

Loud S1 and narrow split S2, collateral flow/coarctation murmur.

71
Q

Investigations in CoA`

A

CXR: cardiomegaly, increased pulmonary markings in LVF. Hypoplastic aortic knob with dilated poststenotic segment.

ECG may show LVH.
Doppler echocardiogram is diagnostic.

72
Q

Management of CoA

A

Medical: infusion of PGE1 in neonates to maintain open DA. Palliate symptoms of LVF with fluid restriction and diuretics. Blood pressure control

Surgical: resection of the coarctation, end to end anastomosis or subclavian artery flap repair. 10% re-coarctation.

Balloon angioplasty: alternative to surgery

73
Q

Mechanism of hypertension in CoA

A

CoA -> decreased systemic BP -> low blood flow to renal -> low perfusion pressure of tubule cells -> high renin production -> high aldosterone -> Na/H2o reabsorb -> HTN.

74
Q

Prognosis in CoA

A

Hypertension if untreated. May cause aortic dissection, aneurysm, and CAD.

Untreated do not survive past 50 typically. With correction 20y survival 91%.

75
Q

Indications for intervention in CoA

A

> 50% narrowing, peak gradient >20mmmHg, or arterial hypertension

76
Q

Describe TGA

A

TGA is characterized by AV concordance and ventriculo-arterial discordance:

Ao from RV
PA from LV

77
Q

Simple TGA

A

TGA is called simple in the absence of associated congenital anomalies (except PFO and PDA)
o Duct dependent!

78
Q

Complex TGA

A

TGA is called complex in the presence of associated anomalies
o VSD (∼45%), LVOTO (∼25%), and CoA (∼5%).

79
Q

ccTGA / l-TGA

A

Additionally ventricular switch (RV on L side, LV on R side)

Usually with dextrocardia (20%)

‘Congenitally corrected’ - do not need neonatal intervention, this is not cyanotic or duct dependent

80
Q

Features of d-TGA

A

o Aorta is RIGHTward and ANTERIOR
o 5% ACHD
o More boys, infants of diabetic mother
o 50% have PFO+PDA only (simple); 50% true complex TGA

81
Q

Key facts about associated lesions in complex TGA

A

 5% significant LVOTO (‘subpulmonary’), 40% VSD
 NB after switch, ‘subpulmonary’ LVOTO this will become true ‘subaortic’ LVOTO
 If VSD, cyanosis better, HF worse
 If no VSD, cyanosis worse, HF better

82
Q

Outcomes in simple vs complex TGA

A

Long-term outcome of complex TGA is, regardless of the type of surgical repair, worse than that of simple TGA.

83
Q

Progression of surgical procedures - 1970s

A

1970-90s: Mustard/Senning ‘atrial switch’ repairs, baffle venous blood from SVC/IVC through into LA and into LV -> to PA

Effectively leads to a subpulmonic LV and subaortic RV

Long term issues with RV failure, arrhythmias due to high RA preload

84
Q

Progression of surgical procedures - 1990s

A

1990s - Arterial switch procedures - Switch of arterial trunks and coronary arteries across to match the right ventricle

85
Q

Typical procedure for Complex TGA

A

Rastelli-type repair

86
Q

Key ‘red flag’ history + exam findings in post-atrial repair TGA patient that hint complication

A
  • Swollen head and neck: superior baffle obstruction
  • Swollen legs/abdomen: inferior baffle obstruction
  • ESM: subpulmonary outflow tract obstruction
  • PSM (regurgitant): TR II to RV dilation
  • AR from neo-aortic root filation
87
Q

Management post-atrial switch

A
  • Diuretics if RV overload
  • No evidence for prognostic HF meds
  • EP input for arrhythmias
  • PAH management
  • Difficult to benchmark progression / failure of subaortic RV ?? reference ranges for EF!
88
Q

% dTGA post repair and Fontan who develop HF

A
  • 22% of dTGA and 40% of Fontain
89
Q

% HT in US due to CHD

A

Proportion of transplant for ACHD is growing, and between 2010-12 was 4% of HT

90
Q

Study looking at likelihood of CHD patients receiving a transplant

A
  • Melanie Averick J Heart Lung transpl – pt with ACHD less likely to receive a transplant even when divided by initial listing status
91
Q

Study looking at on list survival of ACHD patients

A
  • JACC 2016 – patients with ACHD who are listed at top urgency 1a are more likely to die or be delisted compared to on achd conterparts
92
Q

Barriers to transplant in CHD population

A

policies, sensitization, mental health, size matching in young people, anatomical consideration

93
Q

Study on outcomes of transplant for CHD vs non CHD

A

ACHD patients who are transplanted have worse early mortality compared to non-achd, but if stratified after 1 year, those with ACHD perform significantly better

More recent data has shown much closer survival – likely that in historical data there was significant bias due to delay in referral of patients (which remains an issue)

94
Q

Key factors contributing to why ACHD transplant outcomes are better at high volume centres

A

o Anaesthetic considerations: poor tolerance to anaesthetics and fluid shifts
o Operative considerations: multiple sternotomies, anatomy
o Post-op mx considerations: restoration of ‘normal’ circulation can be associated with significant organ dysfunction e.g., kidney injury, abnormal LFTs, slow ventilation weans
o Regionalisation can help improve outcomes

95
Q

Potential interventions to improve survival and symptoms while listed for tx for ACHD patients

A
  • Is there systemic AV valve regurgitation that reduces SV -Can be intervened on
  • Is there arrhythmia - Ablation is safe but should be done with appropriate planning and experience
  • Treating complications – PLE ?midodrine (based on case series)
  • Remember that GDMT is not really applicable in ACHD ?what is ‘normal’ in a systemic RV?

CONSIDER additional procedures = additional risk of surgical interventions = extra sternotomies (make HT higher risk) + more sensitization

96
Q

Specific considerations in Fontan HTx

A
  • Assessing for requirement of liver / kidney transplant
  • Assessment of pulmonary pressures is v important
  • High sensitization because often multiple operations
  • Re-sternotomy x4! – might require femoral bypass (but this is associated with higher mortality as it implies longer bypass time)
  • Planning of connections (e.g., in dextrocardia with TGA, L sided SVC) can require specific ACHD surgeons
  • High HLA sensitization eg 90% - makes patients high priority in some places and low priority in others
97
Q

Treatment for AVSD/VSD

A

Supportive: antibiotic prophylaxis if dental surgery, medical treatment of symptoms of CHF e.g., diuretic

Surgical: closure prevents RVH and arrhythmias. Repair of associated anomalies at the same time. This usually occurs at 2-5y unless severe MR.

AVSD/ASD: closure is via sternotomy approach, fixing of the defect with pericardial tissue autologously or with Dacron/PTEF patches. Needs extracorporeal support.

Percutaneous transcatheter can be done for secundum ASD

Complete AVSD: 3/12, requires tx of pulmonary vascular resistance if present at birth

98
Q

ECG and CXR findings for ASD/AVSD

A

CXR: RAH, RVH, prominent PA and increase in vascular markings
ECG can show RAD, IDHB, RBBB, RAH/RVH.

99
Q

ASD examination in children:

A

Left to right shunt -> pink and breathless.

Murmurs due to high volume of flow (blood from left enters right = high RV preload) across small right-sided valves at high pressure. Not due to the shunt itself.

Can hear both an ejection systolic murmur (pulmonary valve) and a mid diastolic murmur (tricuspid valve).

Fixed splitting of S2 due to high P pressures II to high Rv preload

100
Q

AVSD examination in children

A

Mixed shunt -> blue and breathless.

Atrial + ventricular components, therefore presents with pulmonary HTN and a large VSD.

May be cyanosed at birth due to retrograde flow through PDA

Murmur audible in the first few weeks, but sometimes cavity os big that no murmur heard

101
Q

Exam findings in pulmonary stenosis

A

Mild/moderate stenosis: child pink, RV heave, systolic thrill. ESM. S1 with ejection click and s2 widely split. ESM loudest in P area (left sternal border upper). Intensity NOT related to severity.

Severe stenosis or atresia (duct dependent): cyanosis, heart failure with tricuspid insufficiency, giant A waves in JVP, hepatomegaly and pulsatile liver. S4.

102
Q

Investigations in PS

A

CXR: normal heart size, RA dilation, PA dilation post stenosis, low pulmonary blood flow.

Echo is diagnostic, Doppler, determine the amount of stenosis.

ECG may be normal

103
Q

PS Management

A

Mild: conservative, follow up with echo

Moderate: if symptomatic, treat as severe.

Severe: surgical intervention, minimally invasive baloon pulmonary valvuloplasty. May do open aproach, valvotomy with inflow occlusion, hyopthermia and cardiopulmonary bypass).

104
Q

TOF definition

A

Cyanotic cardiac malformation encompassing these four:
* Large VSD
* Infundibular and valvular pulmonary stenosis
* Right ventricualr hypertrophy
* Overriding of aorta relative to ventricular septum (superior to VSD)
`

105
Q

Aetiology of TOF

A

Complex abnormalities due to abnormal development of right ventricular infindibulum.

Related to fetal hydantoin, CBZ, fetal alcohol, DiGeorige, trisomy 21.

106
Q

Haemodynamic consequences of TOF that cause R>L shunting

A

PS/RVOTO -> high RV pressure -> RVH + increase R-L shunting -> deoxygenated blood in circulation

Hypoxic spells occur in times of high R>L shunting

107
Q

Examination in TOF children

A

Birth: cyanotic. Low BW

Infant: hypoxic spells, harsh ESM at left sternal edge (PS), loud single second heart sound, parasternal heave for RVH

Child: often adopt squatting position in spells to increase systemic vascular resistance, decreasing R-L shunt.
Signs of HF, developmental delay.

108
Q

CXR findings in TOF

A

CXR may show RVH, boot shaped heart, low lung markings and vascularity. ECG. (RAD, RVH, RBBB

109
Q

Management of TOF

A

Treat cyanotic spells: soothe, knee chest position, calm increase venous return. If prolonged, IV morphine for sedation.

Corrective surgical intervention: VSD patch to close, treat other associated anomalies too. Repair at >1y.

Preoperative stabilisation with PGE2 to keep PDA open.

110
Q

Prognosis in TOF

A

Hypoxia, MI, ischaemia in brain, secondary polycythaemia (therefore leading to thrombotic events), infective endocarditis, cerebral abscess, delayed growth and puberty.

Pre surgery 30% mortality in first year of life, 75% by 10y. Now, 90% survive to be adults. .

Paediatric cardiology (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions