Pre operative evaluation of Single ventricle Disease
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Transcript of Pre operative evaluation of Single ventricle Disease
PRE OPERATIVE EVALUATION OF SINGLE VENTRICLEDr. Shahreen KabirFCPS (Paediatrics)Paediatric Cardiac ProgrammeNational Heart Foundation, Dhaka
Single Ventricle (or Univentricular heart) is considered a
cardiac malformation in which both atria connect to only one
ventricular chamber by either two separate AV Valve ( Double
Inlet) or a common AV Valve ( common inlet).
k
Ref- Congenital Heart Defect A.F Conro
Single Ventricle - congenital cardiac
malformations that lack two completely well
developed ventricles, and in which functionally there
is only a single ventricular chamber that supports
both pulmonary and systemic circulations.
Single Ventricle
PhysiologicalAnatomical
Single Ventricle:
Tricuspid Atresia
HLHS
DILV, DIRV
Unbalanced AV Canal Defect
DORV
Tricuspid Atresia
HLHS
DILV
DORV
Unbalanced AV CANAL DEFECT
Univentricluar Atrioventricular Connection
Presentation of Single Ventricle Dis:
Cyanosis
Irritability
Respiratory Distress
Feeding Difficulties
Not growing well
End organ failure ( CNS, Renal, etc)
Without PSWith PS
Physical Assessment
Vitals:
SpO2
Pulse, feeble in HLHS and Co arc
BP
Perfusion
Periphery for warmth
Cyanosis
Clubbing
Polycythemia
FTT
Respiratory- findings of collapse, consolidation
Precordium- findings depends upon the basic disease, presence and absence of PS
The Stages of Palliation:
Stage I : MBT/ PA Banding
Stage II: BD Glenn
Stage III: FONTAN
The 10 Commandements of FONTAN procedure
1. Minimum age, 4 years
2. Sinus rhythm
3. Normal caval drainage
4. Right atrium of normal volume
5. Mean pulmonary artery pressure ≤ 15 mm Hg
6. Pulmonary arterial resistance < 4 Wood/m2
7. Pulmonary-artery-to-aorta-diameter ratio ≥ 0.75
8. Normal ventricular functions (ejection fraction > 0.6)
9. Competent left atrioventricular valve
10. No impairing effects of previous shunts
Cardiovascular Workup
Chest X-ray-
Normal
Heart size (depends if the pt is in failure or not)
Pulmonary Vascularity or
Tricuspid Atresia
HLHS
ECG- acc to disease
Arrhythmia
LAD w LVH, 1st degree AV Block ( Tric Atr)
RAD, RVH, Tall P wave( HLHS)
Echocardiographic Evaluation
Basic diagnosis
Basic Anatomy
Atrial Volume
IAS mixing or restrictive?
SVC, IVC size, their draining, ratio.
AV valve morphology, allignment, regurgitation
MV, TV ratio
Ventricles, LV or RV morphology, rudimentary one
VSD size
LV EF, LV EDP
PA size, branching, anatomy.
PAH or PS
Pre FONTAN Cath:
PA Anatomy
PA Pressure ( <15mmHg is desirable)
PVR ( <4Wood Unit is desirable)
RV to PA gradient
Pulm Venous Return
LV EDP
Functioning of prev shunts
Any AVM
Before Proceeding to Fontan ( Stage III Palliation)
Improve clinical symptoms: cyanosis, CHF
Provide optimal pulmonary artery architecture and low PVR
Preserve systolic and diastolic ventricular function
Preserve atrio-ventricular valve function
Relieve systemic ventricular outflow tract obstruction
Provide anatomic setup for a definitive Fontan repair
Ideal saturation:
Age 3 to 4 mo
Mean PA pressure <15mmHg
PVR < 4 Wood units
Surgically repairable PA hypoplasia or discrete
stenosis present
Target SpO2: 78~85%
TAKE HOME MESSAGE
Meticulous pre operative assessment should be done before patient selection.
LOW PVR
GOOD LV FUNCTION
NO AV VALVE REGURG
THANKS FOR PATIENT HEARING!