ACHD Flashcards
ASD
Associated with Flutter, diastolic and systolic flow, more systolic as TV moves down and draws blood across ASD.
Primum - 15% of ASDs - low on septum, involving AV valves, surgical repair only
Secundum - 75% of ASDs - middle of septum. Usually percutaneously closable
Sinus Venosus ASD - 5%. high on septum. Associated with PAPVD (overarching PV). Surgical repair
Indications to close:
1. RV dilatation
2. Qp:Qs >1.5:1
3. Stroke
PH develops in <5% ASD
VSD
Muscular - entire rim is muscle
Membranous - between the inlet / outlet (perimembranous if extends into muscle). Can cause AR - Venturi effect causes prolapse of cusps
Doubly Committed - Infundibulum involving outflow tracts of LV and RV
*VSDs shunt in systole - results in direct flow to PA (as RV also in systole), so higher LV pre-load and LV dilatation.
*Close VSD if PVR <5 WU
Indications to close:
1. LV dilatation
2. Large shunt (Qp:Qs ≥1.5:1
3. Aortic Regurgitation
4. Recurrent endocarditis
AVSD
- Complete disruption of endocardial cushion - absence of centre of heart endocardium
- Partial - only ASD or VSD present (90% in non Down’s) or complete (75% in Downs)
- Common AV valve - usually with 5 cusps
- Complications - left AV valve regurgitation, conduction system disease
Calculations
PVR = mPAP - PCWP / Qp
Mixed Venous Sats = 2(SVC) + IVC / 3
Qp:Qs = Ao Sats - mixed venous sats / PV Sats - PA sats
Tetralogy of Fallot
- Over-riding aorta
- Stenotic pulmonary valve
- VSD
- RV hypertrophy
Ventricular patch repair and RVOT reconstruction
More severe pulmonary stenosis / atresia - increased R->L flow through VSD
Reduced LV preload -> “spelling” unconscious episodes with crying , exertion
ECG - RBBB
Long term - atrial and ventricular arrhythmias, right heart failure, aortic dilatation
Poor prognosis: high grade ectopics on Holter, QRSd>180, age at time of surgery, era of surgery
Sudden death 2.5% per decade of surgery
Transposition of the Great Arteries
- Pulmonary Artery arises from LV and Aorta arises from RV
- Universally fatal without PDA, ASD or VAS as 2 parallel circulations
- Previous repair - Mustard or Senning Procedures - atrial switch - ++atrial arrhythmias, systemic RV failure
- Modern repair - arterial switch operation - coronary arteries re-implanted on buttons - long term AV, PV and RVOT disease, coronary stenosis
Bicuspid Aortic Valve
*1-2% of population
*Peak surgery 60-80
*Abnormal aorta - histology similar to Marfans
Subvalvular / Supravalvular aortic Stenosis
Subaortic Stenosis
- Discrete fibrous muscular ring - can extend into the AMVL
- 6.5% of congenital heart disease. Associated with ASD, VSD and Shone complex
- Progressive disease, can lead to AR
- Can lead to AV damage, ventricular dysfunction, IE and sudden death
- Operate when instantaneous gradient >50mmHg or mean gradient >30mmHg
- Rx - surgery. Resection of fibrous ring and muscular base on L septal surface
- Complications - AV or MV damage, heart block, VSD
Supravalvular
- Wlliams syndrome, friendly, LD, renal artery stenosis, other aortopathy, accelerated CAD
Coarctation of the Aorta
- Commonly at isthmus - PDA insertion site
- Often present with HTN
- Dilated ascending aorta
- 3 sign on CXR, rib notching
- Radiofemoral delay
- Bruit in L infrascapular area
- BP difference in arms
- Forward diastolic flow, decreased pulsatility in abdominal aorta
Indication for intervention: peak to peak ≥20mmHg or radiological evidence of collaterals
Recurrent, discrete coarctation - catheter intervention
Long segment or arch hypoplasia - surgery
Rx: End to end repair, subclavian flap, conduit
Rx in adults - coarctation stent
Fontan Circulation
- Unventricular circulation created during staged surgery for patients with rudimentary RV or LV (hypoplastic left heart syndrome, tricuspid atresia, heterotaxia)
- Norwood Procedure (to form aorta)
- Glen procedure- IVC and SVC to PA
- Total cavopulmonary connection (Fontan Completion)
- Passive pulmonary flow - sensitive to rises in PVR
- Risk of VTE. Old Fontan repairs with atrial appendage used get anticoagulation
- Protein losing enteropathy - poor prognosis
- VTE, Fontan Failure, Arrhythmias, Sudden Death over time
Eisenmenger’s Snydrome
- Post tricuspid shunts leads to pulmonary vascular disease, pulmonary HTN and shunt reversal
- Cyanosis
- Chronic hypoxia leads to secondary erythrocytosis. This leads to high haemoatocrit and thrombocytopaenia (compensatory mechanism). Leads to bleeding tendency. Low ferritin as high red cell production
- Tendency to bleed and to clot. Aim to avoid anticoagulation
- Leads to hypertrophic osteoarthropathy - clubbing, arthralgia, ossifying periositis
- Renal and liver disease due to prolonged cyanosis
- Complications - arrhythmias, pulmonary bleeding
Rx - as PAH. Entothelin antagonists, PDE-5i, prostacyclin analogues (+ selexipag)
Ebstein’s Anomaly
Apically displaced and rotated TV
Atrialised portion of RV
Spectrum of severity, severe end have essentially no functional RV - need Fontan
Cone operation for milder cases
Atrial arrhythmias - AF/AVNRT and AVRT
Other ACHD considerations
- Atrial tachycardia common. Suspect in patients with HR >90. Often need shock. Avoid positive pressure ventilation in Fontan
- MVO2 - helps guide surgery
Anomalous Coronary Arteries
LCA from PA - surgery - bad prognosis
If no symptoms or ischaemia, still do surgery in anomalous aortic origin of LCA if:
- Intramural course
- Slit like orifice
- Acute angle take off
- Orifice >1cm above STJ
