Congenital Heart disease and haemodynamics Flashcards
Causes of raised pulmonary capillary wedge pressure (PCWP)
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
Constrictive vs. restrictive cardiomyopathy
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)
Definition of pulmonary hypertension
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
Vascular resistance
Pulmonary vascular resistance = PAP - Left atrial pressure(PCWP) / cardiac output
Systemic vascular resistance = Aortic pressure - Right atrial pressure / cardiac output
Management of pulmonary arterial hypertension
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
Types of atrial septal defect
– 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%)
Fick’s equation to determine shunt size
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
Indications and contraindications to ASD closure
Indications:
– Symptoms
– Haemodynamically significant shunt
RV enlargement
Qp:Qs > 1.5
– Paradoxical embolism
– Platypnea‐orthodeoxia syndrome
Contraindications:
- Severe pulmonary arterial hypertension
Complications of ASD
- Paradoxical embolism
- Congestive heart failure, right heart failure
- Pulmonary hypertension
- SVTs
Ventricular Septal Defect subtypes
– Membranous 80%
– Subarterial 6%
subpulmonary
doubly committed
juxta‐arterial
outlet
supracristal
– Muscular 20%
– AV Canal type
Inlet
Complications of VSD and indications for closure
Left ventricular failure
Right ventricular failure
Pulmonary hypertension
Close if
- Qp:Qs >2
- CCF
- pulmonary hypertension
- RV tract outlet obstruction, aortic regurgitation, IE
Patent Ductus Arteriosus clinical signs
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
Ventricular septal defect clinical signs
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
Hypertrophic cardiomyopathy signs
Bifid carotid pulse
‘a’ wave in JVP
Double apical impulse
ESM (diamond) along left sternal border accentuated by Valsalva manoeuvre
S4
Atrial septal defect clinical signs
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
Pulmonary stenosis clinical signs
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
Coarctation of aorta clinical signs and associations
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
Eisenmenger syndrome clincal signs
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)
Management associated with Eisenmenger syndrome
Erythrocytosis, venesection if HCT >65%
Consequential hyperviscosity syndrome
Infective endocarditis risk
Pulmonary hypertension management
Gallstones and gout
SCD
Heart failure
Stroke
Complications and management of coarctation
Complications
- Heart failure
- Stroke
- Aortic dissection
Management
- Stent if hypertensive and peak to peak gradient difference >20mmHg
- Angiogram at 1yr, MRI every 5yrs
Features of tetralogy of Fallot
- Unrestrictive VSD
- Right ventricular outflow tract obstruction
- Subpulmonary stenosis or Pulmonary atresia
- Overriding aorta
- RVH
Complications of atrial procedure for transposition of great arteries
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
Complications for great vessel switch for TGA
Coronary compromise
Neo‐AR
Neo‐aortic dilation
Neo‐PS or Neo‐PR
PA stenoses (LeCompte)
Features of Ebstein anomaly
Enlarged right atrium
Displaced tricuspid valve annulus
Atrial septal defect
Cyanosis
Accessory pathways
Issues post tetralogy of Fallot repair
Pulmonary regurgitation
RV outlet obstruction
LV dysfunction
Increased risk SCD - VT
Palliative procedures for tetralogy of Fallot
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
Complications of Fontan procedure
• ‘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
Cardiac risks in pregnancy
– 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
Patent foramen ovale closure
Orthodeoxia-platypnoea
Decompression illness (if wanting to dive)
Stroke - RESPECT trial benefit of closure for cryptogenic stroke
Insufficient evidence for migraines
Endocarditis prophylaxis indications
– 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
Pulmonary hypertension clinical signs
Loud P2
Early diastolic murmur at LUSE
Left parasternal heaveEjection click after S1 in pulmonary area
