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).

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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!