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Cardiology for RACP > Congenital Heart disease and haemodynamics > Flashcards

Congenital Heart disease and haemodynamics Flashcards

1
Q

Causes of raised pulmonary capillary wedge pressure (PCWP)

A

Diastolic HF (HFpEF) - PCWP ~ LEDP

Mitral stenosis - PCWP >LEDP

Atrial myxoma - PCWP >LEDP

Pulmonary vein stenosis (post catheter ablation for AF) - PCWP > LEDP

Cor triatriatrium PCWP >LEDP

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2
Q

Constrictive vs. restrictive cardiomyopathy

A

Constrictive cardiomyopathy

  • Prominent x and y descents, equal a and v waves (M wave) on JVP
  • Kausmul’s sign (rise JVP pressure on inspiration)
  • LV-RV interdependence (discordance between peak RV and LV systolic pressures, particularly during inspiration)

Restrictive cardiomyopathy

  • Prominent y descent
  • equal drop in PCWP and left diastolic pressure during inspiration (in contrast to CC, whereby increased intrathoracic pressure decreases PCWP only)
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3
Q

Definition of pulmonary hypertension

A

Definition:

mPAP > 25mmHg – Normal mPAP < 20

– Mild: mPAP > 25

– Moderate: mPAP > 35

– Severe: mPAP > 45

1: Small arterioles – Idiopathic – Congenital – HIV – Drugs
2: Left heart – Systolic, diastolic, valves
3: Hypoxaemia – Lung disease
4: Thromboembolic
5: Multifactorial

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4
Q

Vascular resistance

A

Pulmonary vascular resistance = PAP - Left atrial pressure(PCWP) / cardiac output

Systemic vascular resistance = Aortic pressure - Right atrial pressure / cardiac output

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5
Q

Management of pulmonary arterial hypertension

A

Type 1:

  • Vasoactive patients (positive vasoactive test during PWCP testing >10mmHg drop) - long acting calcium channel blocker promote survival
  • Patients with NYHA II and III symptoms - endothelin antagonist and/or nitric oxide agonist (riociguat) or combination (ambrisentan and sildenafil) and/or selexapag (non-prostanoid IP agonist)
  • 2 former CI in pregnancy
  • Patients with NYHA IV symptoms - IV prostanoid therapy (epoprostenol, iloprost)
  • Atrial septostomy with refractory symptoms and RHF
  • Lung transplant
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6
Q

Types of atrial septal defect

A

– Ostium Primum = partial AVSD (15‐20%)

– More common in Down Syndrome

– Ostium Secundum (75%)

– Amenable to percutaneous closure

– More common in females (65‐75%)

– Sinus venosus (5‐10%)

Superior vena cava (most common) and inferior vena cava types

Associated with anomalous pulmonary venous return

– Coronary Sinus (sinoseptal) (<1%)

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7
Q

Fick’s equation to determine shunt size

A

Ratio of pulmonary blood flow : systemic blood flow

(Aortic O2 sat - Mixed venous return sat) / ( pulmonary vein O2 sat - pulmonary artery O2 sat)

Atrial septal defect - increased sats expected from the right atrium and hence right ventricle and pulmonary artery

Ventricular septal defect - increased sats expected from right ventricle and hence pulmonary artery

Patent ductus arteriosus - increased sats from pulmonary artery only

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8
Q

Indications and contraindications to ASD closure

A

Indications:

– Symptoms

– Haemodynamically significant shunt

RV enlargement

Qp:Qs > 1.5

– Paradoxical embolism

– Platypnea‐orthodeoxia syndrome

Contraindications:

  • Severe pulmonary arterial hypertension
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9
Q

Complications of ASD

A
  • Paradoxical embolism
  • Congestive heart failure, right heart failure
  • Pulmonary hypertension
  • SVTs
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10
Q

Ventricular Septal Defect subtypes

A

– Membranous 80%

– Subarterial 6%

subpulmonary

doubly committed

juxta‐arterial

outlet

supracristal

– Muscular 20%

– AV Canal type

Inlet

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11
Q

Complications of VSD and indications for closure

A

Left ventricular failure

Right ventricular failure

Pulmonary hypertension

Close if

  • Qp:Qs >2
  • CCF
  • pulmonary hypertension
  • RV tract outlet obstruction, aortic regurgitation, IE
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12
Q

Patent Ductus Arteriosus clinical signs

A

Collapsing pulse

Heaving apex beat

Systolic and diastolic thrill in left second interspace

Pansystolic ‘machinery’ murmur extending into diastole, beginning after S1 and peaking at S2 (not heard)

Differential cyanosis (in toes not fingers)

Reverse split S2

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13
Q

Ventricular septal defect clinical signs

A

Normal pulse

Left ventricular impulse

Pansystolic murmur at LLSE and apex

Thrill

Decrescendo diastolic murmur (aortic valve annulus opening)

Plus signs of : +/- pulmonary hypertension, CCF, aortic regurgitation, pulmonary stenosis

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14
Q

Hypertrophic cardiomyopathy signs

A

Bifid carotid pulse

‘a’ wave in JVP

Double apical impulse

ESM (diamond) along left sternal border accentuated by Valsalva manoeuvre

S4

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15
Q

Atrial septal defect clinical signs

A

Wide fixed S2

Parasternal heave

Ejection systolic flow murmur in the second left and third intercostal space

Congenital defect of thumb (Holt-Oram)

Plus signs of pulmonary hypertension

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16
Q

Pulmonary stenosis clinical signs

A

Normal pulse

Large ‘a’ wave on JVP

Left parasternal impulse

Ejection systolic click that increases on inspiration

Soft S2

ESM in LUSE, radiating to left shoulder and lung

Central cyanosis and clubbing

17
Q

Coarctation of aorta clinical signs and associations

A

Systolic BP of upper limbs elevated as compared to lower limbs; but diastolic pressures are similar

Reduced left sided radial pulse

Weak femoral pulses

Systolic thrill at suprasternal notch

Harsh systolic ejection murmur along the left sternal border, clavicles and back

Associated with:

  • Bicuspid aorta (ESM, ejection click after S1)
  • Turner syndrome
  • Berry aneurysms
  • VSD
  • PDA
  • Mitral regurgitation or stenosis
18
Q

Eisenmenger syndrome clincal signs

A

Clubbing of fingers and central cyanosis

Prominent a waves and large v waves on JVP

Parasternal heave and palpable P2

Pansystolic murmur LLSE

Pulmonary ejection click

Early diastolic murmur of pulmonary regurgitation

Pulmonary hypertension

Associations/complications/causes:

  • HF
  • ASD (wide split S2, systolic murmur L) 3rd intercostal space)
  • PDA (machinery murmur through both systole and diastole, reverse split S2)
  • VSD (harsh pansystolic murmur left sternal border w/ thrill)
  • Tricuspid regurgitation (large v waves, pulsatile liver, LLSE pansystolic murmur)
19
Q

Management associated with Eisenmenger syndrome

A

Erythrocytosis, venesection if HCT >65%

Consequential hyperviscosity syndrome

Infective endocarditis risk

Pulmonary hypertension management

Gallstones and gout

SCD

Heart failure

Stroke

20
Q

Complications and management of coarctation

A

Complications

  • Heart failure
  • Stroke
  • Aortic dissection

Management

  • Stent if hypertensive and peak to peak gradient difference >20mmHg
  • Angiogram at 1yr, MRI every 5yrs
21
Q

Features of tetralogy of Fallot

A
  • Unrestrictive VSD
  • Right ventricular outflow tract obstruction
  • Subpulmonary stenosis or Pulmonary atresia
  • Overriding aorta
  • RVH
22
Q

Complications of atrial procedure for transposition of great arteries

A

80% survive >20 years Issues in adulthood:

• Heart failure

– ? Failure of AV coupling with an inability to increase SV with exercise or dobutamine

– ? RV failure

• Pulmonary HT

– ? ’set up’ from birth • Arrhythmias, sudden death

• Baffle leak, obstruction

23
Q

Complications for great vessel switch for TGA

A

Coronary compromise

Neo‐AR

Neo‐aortic dilation

Neo‐PS or Neo‐PR

PA stenoses (LeCompte)

24
Q

Features of Ebstein anomaly

A

Enlarged right atrium

Displaced tricuspid valve annulus

Atrial septal defect

Cyanosis

Accessory pathways

25
Q

Issues post tetralogy of Fallot repair

A

Pulmonary regurgitation

RV outlet obstruction

LV dysfunction

Increased risk SCD - VT

26
Q

Palliative procedures for tetralogy of Fallot

A

Blalock-Tussig shunt - subclavian to pulmonary trunck

Waterston and Pott’s shunts - ascending and descending aortic to pulmonary artery shunts

Fontan operation - IVC to pulmonary artery, bypassing the right heart

27
Q

Complications of Fontan procedure

A

• ‘Fontan failure’ (esp. if ventricular dysfunction, AVVR)

– Low output or Heart failure syndrome

– PLE (stool alpha 1 antitrypsin)

  • Arrhythmias, sinus node dysfunction (epicardial PPM)
  • Thromboembolism

– Fontan -> PE (Fontan failure)

– Systemic -stroke

• Hypoxia (should have near normal saturations)

– Veno‐venous collaterals

– AVMs

28
Q

Cardiac risks in pregnancy

A

– Severe PHT

– Eisenmenger syndrome

– Cardiomyopathy

NYHA 2+, EF 40mm

– Previous severe peripartum CM

  • Severe cardiac obstructive lesions AS,PS, MS
  • Marfan syndrome with aortic root >40mm
29
Q

Patent foramen ovale closure

A

Orthodeoxia-platypnoea

Decompression illness (if wanting to dive)

Stroke - RESPECT trial benefit of closure for cryptogenic stroke

Insufficient evidence for migraines

30
Q

Endocarditis prophylaxis indications

A

– Prosthetic cardiac valve

– Congenital heart disease

Unrepaired cyanotic

Repaired CHD with residual defect at site of patch/device (no endothelialisation)

(Repaired CHD – surgery or device ‐ for first 6 months)

– Previous endocarditis

– Cardiac transplant patients with valvular disease

31
Q

Pulmonary hypertension clinical signs

A

Loud P2

Early diastolic murmur at LUSE

Left parasternal heaveEjection click after S1 in pulmonary area

Cardiology for RACP (6 decks)

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