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Step 3- cardiovascular disease > Congenital heart diseases and valvular disease > Flashcards

Congenital heart diseases and valvular disease Flashcards

1
Q

Associations of VSD

A

Holt-Oram syndrome

Intrauterine infections

Down syndrome
Tetralogy of Fallot
AVSD
Transposition of great vessels

Maternal diabetes

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2
Q

Holt-Oram syndrome
- gene mutation
- features

A

TBX5 gene
- autosomal dominant

Cardiac septal defects- ASD.
Upper limb defects- carpal bones

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3
Q

ASD associations

A

Fetal alcohol syndrome
Down syndrome
Intrauterine infections

Holt-Oram syndrome (hand-heart syndrome)

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4
Q

VSD heart murmur

A

Holosystolic murmur @ lower left sternal border
- increases with afterload (handgrip)

Mid-diastolic murmur

Loud S2 (pulmonary hypertension)

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5
Q

ASD heart murmur

A

Systolic ejection murmur

Wide fixed split S2 in 2nd left intercostal space

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6
Q

VSD ECG changes

A

LV and RV hypertrophy

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7
Q

ASD ECG changes

A

RV hypertrophy
SVTs
PR prolongation
P pulmonale- peaked, narrow P wave

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8
Q

VSD CXR features

A

Increased pulmonary vascular markings
Left atrial/ventricular enlargement

Later- enlarged right ventricular and pulmonary artery

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9
Q

Most common type of ASD

A

Ostium secundum

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10
Q

Surgical indications of ASD

A

Large shunt (Qp:Qs >1.5:1)
Right atrial/ ventricular hypertrophy
Heart failure
Paradoxical emobilism
Platypnoea-orthodeoxia syndrome

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11
Q

Platypnoea-orthodeoxia syndrome

A

Right-to-left shunt causing dyspnoea + cyanosis when in upright position

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12
Q

Prognosis of ASD- spontaneous closure

A

40% by age 5

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13
Q

Genetic causes of VSD

A

Down syndrome
Edward syndrome
Patau syndrome

Cri-du-chat syndrome
Apert syndrome

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14
Q

aquired causes of VSD

A

Post-MI
Aortic valve replacement

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15
Q

When is surgical management of VSD/ ASD contraindicated?

A

Severe pulmonary hypertension/ Eisenmenger syndrome

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16
Q

AVSD associations

A

Down syndrome- strongest

Gestational diabetes

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17
Q

PFO associated with what syndrome?

A

Loeys-Dietz
- marfanoid habitus
- associated with aortic aneurysm + dissection
- features: hypertelorism (wide eyes), cleft palate, easy bruising, keloids, tortuous arteries

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18
Q

Best initial study for PFO

A

TTE with agitated saline
- TEE more sensitive

Transcranial doppled with agitated saline- could detect microbubbles in cerebral arteries

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19
Q

Complications of PFO

A

Migraine with aura
Ischaemic stroke/ TIA
Paradoxical emb0lism/ systemic embolism

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20
Q

Risk factors for PDA

A

Prematurity

Maternal factors:
- Rubella
- Alcohol
- Phenytoin
- Prostaglandin use

Chromosomal trisomies

Resp distress syndrome

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21
Q

Clinical features of PDA

A

Large PDA:
- failure to thrve
- heart failure
- bounding pulse
- wide pulse pressure
- laterally displaced apical impulse

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22
Q

A machinery, loud continuous murmur loudest in the left infraclavicular region (S2) is associated with what congenital heart defect?

A

Patent ductus arteriosus

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23
Q

Contraindications of pharamacological closure of PDA

A

Ductal dependent- cyanotic diseases
Persistent pulmonary hypertension with right-left shunt
Oliguria
Thrombocytopenia
Recent haemorrhage
Necrotising enterocolitis

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24
Q

Pharmacological Rx of PDA

A

inhibition of prostaglandins:
- Indomethacin
- Ibuprofen

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25
Coarctation of aorta associations
Turner syndrome Bicuspid valve VSD PDA acquired- Takayasu arteritis, severe arteroscleorisis
26
Clinical features of coarctation of aorta
Lower limb cyanosis Brachial-femoral delay/ different BP Lower limb claudication Laterally displaced apical pulse Tinnitus, headache, epistaxis SEVERE, ductal closure: shock, multi-organ failure
27
Coarctation of aorta heart murmur
Left posterior hemithorax- systolic ejection murmur Left infraclavicular/ interscapular- continuous murmur
28
CXR features of coarctation of aorta
Figure of 3- hourglass shape of aortas - dilated aorta - dilated left subclavian artery Rib notching- colternal internal thoracic/ intercostal arteries, ribs 3-8
29
Rx of critical and non-critical coarctation of aortia
Neonates - Prosglandin E1 infusion (aprostadil) Non-critical - stent, surgery - ballon angioplasty- adults
30
Monitoring in co-arctation of aorta
Screen for: - re-stenosis - aortic aneursym - aortic dissection
31
co-arctation of aorta complications
Secondary hypertension Aortic dissection/ rupture Berry aneurysm- cerebral haemorrhage HF infective endocarditis
32
Pulmonary valve stenosis is associated with what genetic syndrome?
Noonan syndrome - PTPN11 gene, chromosome 12 - short stature, webbed neck, hypertelorism, cardiac defects
33
Rx of critical and non-critical pulmonary valve stenosis
Crtical - Prostaglandin infusion to maintain PDA Non-critical - Balloon valvuloplasty/ valve replacement
34
Features of tetrology of fallot
RVOTO from pulmonary stensosis Right ventricular hypertrophy VSD Overriding aorta
35
Cyanotic congenital heart diseases
- Tetralogy of fallot - Transposition of great vessels - Ebstein anomaly - Pesisten truncus arteriosis - Hypoplastic left heart syndrome - Total anomalous pulmonary venous return - Tricuspid valve atresia Eisenmenger syndrome from ASD, VSD
36
Tetrology of fallot associations
Genetic - DiGeorge syndrome - Down syndrome Maternal expsoure - Diabetes - Alcohol - Phenylketonuria
37
Tetrology of fallot cardiac murmur
Systolic ejection murmur at left upper sternal border Single S2 RV heave/ thrill
38
Boot shaped heart is associated with...
Tetrology of fallot
39
Management of acute hypoxia in TOF
High flow O2 Squatting/ knee to chest= increases systemic vascular resistant Further: - IV Na bicarb for metabolic acidosis - IVF - Vasopressors- phenylephrine - IV beta-blockers
40
TOF prognosis
50% 3-year survival With surgery- >90% 25-year survival
41
TOF definitive treatment
Surgery within 1st year - VSD repair- patch closure - Resection of obstructure right infundibular structure Palliative shunt if surgery not possible
42
Egg on a string heart on CXR is characteristic of what congenital heart defect?
Transposition of great vessels
43
RV hypoplasia and RA dilation with central cyanosis is characteristic of what congenital heart defect?
Pulmonary valve atresia
44
Non-immune hydrops fetalis, wolff-parkinson white syndrome and loud S1 is associated with what congenital heart defect?
Ebstein anomaly
45
Pathophysiology of total anomalous venous return
Connecting vein between venous circulation and pulmonary vein= oxygenation blood into right atrium
46
Heterotaxy is associated with what congenital heart defect?
Total anomalous venous return - associated with asplenia (right isomerism) heterotaxy= abnormal right-left axis (i.e. 2 right lungs, 2 spleens, 2 right atriums)
47
Etiology- persistent truncus arteriosus
Failure of neural crest cells to migrate to cardiac outflow tract= incomplete aorticupulmonary septum
48
DiGeorge syndrome is associated with what cardiac defects?
Tetralogy of Fallot Persitent truncus arterosus VSD, ASD
49
Hypoplastic left heart syndrome associations
Trisomy 13, 18 Jacobsen syndrome Turner syndrome
50
Risk factors for aortic regurg
Bicuspid aortic valve Aortic valve stenosis/ caclinosis Rheumatic fever Aortic dilation - CTD - Chronic hpetension - Aoritis - Thoracic aortic aneurysm
51
Taube sign is associated with what valvular heart disease?
Aoritc regurg - pistol-shot sounds in femoral artery
52
Quincke sign and De Musset sign is associated with what valvular heart disease
Aortic regurg Quincke sign- visible capillary pulse when pressure applied to fingertip De Musset sign- rhyhtmic head nodding/ bobbing with heartbeat
53
What mumur... High-pitched, blowing, decrescendo early diastolic murmur - worsens with squatting/ handgrip
Aortic regurg
54
Indications for surgical management of AR
Acute, severe AR Symptomatic chronic sever AR Asymptomatic chronic severe AR with - LVEF <55% - LVESD >50mm
55
Serial echos in AR
Mild- 3-5 years moderate= 1-2 years Severe= 6-12 months
56
Most common cause of aortic valve stenosis
Aortic valve sclerosis
57
Aortic valve stenosis causes
Aortic sclerosis Bicuspid aortic valve Rheumatic fever
58
Harsh crescendo-decrescendo later systolic murmur with - S4 - ejection systolic click what valve disease?
aortic stenosis
59
Stage B aortic valve stenosis (progressive) - mild aortic valve area transaortic velocity mean aortic pressure gradient
Aortic valve area - 1.5-2.9cm2 Transaortic velocity - 2-2.9m/s Mean aortic pressure gradient - 10-19mmHg
60
Stage B aortic valve stenosis (progressive) - moderate - aortic valve area - transaortic velocity - mean aortic pressure gradient
Aortic valve area - 1.0-1.4 cm2 Transaortic velocity - 3-3.9 m/s Mean aortic pressure gradient - 20-39 mmHg
61
Stage c aortic valve stenosis - severe - aortic valve area - transaortic velocity - mean aortic pressure gradient
Aortic valve area - <1cm 2 Transaortic velocity - >4 m/s Mean aortic pressure gradient - >40 mm Hg
62
Additional evaluation in aortic stenosis (outside of echo)
Low dose dobutamine stress testing (Stage 5, LVEF >50%) Exercise testing- Stage C1/2 Cardiac catherisation Coronary angiogram- preop
63
Anticoagulation in aortic valve replacement
Mechanical valve - Warfarin +/- antiplatelet - IN 2.5-3.5 if >1 risk factor for TE or older valves Bioprostehtic valve - antiplatelet +/- warfarin (3-6 months after SAVR/TAVR)
64
Mitral regurg causes
Primary - degenerative: prolapse, annular calcification, ruptured chordae tendinae - infective endocarditis - Ischaemic- post-mi papillary muscle rutpure secondary - CAD - dialted CMP
65
Holosystolic murmur (high-pitched, blowing) Radiating to axilla
Mitral regurg Also - quiet s1 - s3 (severe)
66
Mitral valve prolapse associations
CTD - marfan - Elhers-Danlos - osetogenesis imperfecta Fragile X syndrome MI Acute rheumatic disease Endocarditis ADPCKD
67
Mid-systolic click +/- High mid-late systolic murmur what murmur?
mitral valve prolapse
68
Mitral valve proplase complications
AF Ventricular ectopics TIA/ stroke Infective endocarditis Right heart failure
69
Severe mitral stenosis- mitral area
MVA <1.5 cm2
70
Most preferred procedure for severe MS
Percutaneous mitral valve blloon commissurotomy
71
Eosphageal compression and recurrent laryngeal nerve palsy is associated with what valvular heart disease?
Mitral valve stenosis
72

Step 3- cardiovascular disease (8 decks)

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