Basic concepts to Understand Congenital Heart Disease and their Repair p
Basic concepts to Understand Congenital Heart Disease and their R Repair i •...
Basic concepts to Understand Congenital Heart Disease and their Repair p
Basic concepts to Understand Congenital Heart Disease and their R Repair i • No Relationships to disclose
FRANCISCO J. GENSINI, MD Assistant Professor of Surgery Division of Congenital Cardiac Surgery University of Rochester, SUNY Health Science Center and CROUSE Medical Center
Normal Cardiac Anatomyy • 4 cavities • 2 sides with 2 cavities each • Interatrial and interventricular septa • 2 Atrioventricular valves • 2 Semilunar valves
Normal Intra cardiac Blood Flow
Pulmonary valve Right
Left
Aortic valve
2
1 Interatrial Septum Tricuspid valve
4 3
Mitral valve Inter ventricular S t Septum
Right Heart
Left Heart
Orienting the Heart Aorta
BASE
Right Atrium
Pulmonary Artery Left L ft Pulmonary P l veins
Right Ventricle
Left Anterior descending coronary (LAD) Left Ventricle
APEX
Surgical g view of the Heart Ascending Aorta Right Atrium Atri m
Cardiopulmonary Bypass Circuit
M i Main Pulmonary artery
Reservoir
Oxygenator
Right Ventricle
Bubble Filter Arterial pump head Heat Exchanger Oxygen
Suction pumps
Cardioplegia pump
Cardiopulmonary p y Bypass yp p pump p in the OR
Bubble Filter
Diagnosis: Trans thoracic Echo
Suction pumps
Venous Reservoir
Oxygenator
Cardioplegia C di l i pump
Arterial pump
Transesophageal p g Echo
Other Diagnostic tests
Cardiac MRI
Cardiac Catheterization
Congenital Heart Disease
Most common Congenital Cardiac defects 1. Ventricular Septal Defect (VSD) : 20% isolated 50% with associated anomalies
4. Tetralogy of Fallot (TOF) : 8-10% 5 T 5. Transposition iti off Great G t Arteries A t i (TGA) : 8-10% 8 10%
• ASD
6. PDA: 5-10% (higher in prematures).
• VSD • AVSD
7 Coarctation of the Aorta: 5 7. 5-8% 8% 8. Hypoplastic Left Heart Syndrome (HLHS) : 5% 9 Atrioventricular Septal Defect (AVSD) : 3-4% 9. 3 4%
•
Truncus
•
TAPVC
•
10. TOF-Pulmonary Atresia –MAPCAs: 2%
TGA + VSD •
TA type C
Congenital Heart Disease
Pulmonary artery Banding
ASD
Increased Pulmonary Blood Flow
VSD
Decreased Pulmonary Blood Flow
Other type of Lesions
• PDA
• TOF
• DORV
• AP Window
• LVOTO
• ASD
• TOF + PA + MAPCAs
• VSD
• PA + IVS
• HLHS
• AVSD
• PV stenosis
• ALCAPA
PDA
Palliative and Repair procedures for Increased Pulmonary circulation
•
•
Truncus
•
TAPVC TGA + VSD
•
TA type C
• TA type A •TGA+VSD+PS
• CoAo
Palliative procedures to increase Pulmonary blood flow
Cyanotic Heart defects Venous Blood is reaching the systemic circulation • Tetralogy of Fallot (TOF) • Transposition of Great Arteries (TGA) • Tricuspid Atresia (TA) • Truncus Arteriosus • Total Anomalous Pulmonary venous return (TAPVR) • Hypoplastic Left Heart syndrome (HLHS)
Left-to-Right Shunting Lesion: Definition • Communication C i ti b between t left l ft heart h t structure t t and d right i ht heart structure allowing blood flow (shunting) from the left heart to the right g heart • No obstruction to right heart blood flow • 2 well-formed ventricles
Left-to-Right Left to Right Shunt Pathophysiology: Pulmonary Vascular Resistance Neonatal Period : -
High PVR
-
No pressure gradient
-
Minimal shunt
-
Asymptomatic
-
Absent murmur
PVR starts to decrease for following g days y ((2-6 weeks))
Left-to-Right Shunting Lesion: Pathophysiology • Decreasing PVR – Increased shunting g with pulmonary over-circulation and
Left-to-Right Shunting Lesion: Long-term Effects • Chronic Volume Overload – Arrhythmia y a – Systolic / Diastolic Dysfunction
volume load (CHF) – Audible murmur – Tachypnea yp / dyspnea y p
• Pulmonary Vascular Disease • Eisenmenger Eisenmenger’s s Syndrome
– Failure to thrive
Left-to-Right g Shunting g Lesion: Timing of Surgical Repair • Technical feasibility • Size • Co-morbidity C bidit
• • • •
Symptomatic state: Shunt volume Potential for spontaneous closure Potential for irreversible pulmonary vascular disease Potential for or presence of related pathophysiology • Endocarditis risk • Prolapse aortic leaflet with VSD
Calculation of Shunt Volume: Fi k P Fick Principle i i l Shunt Volume = Pulmonary Blood flow (Qp) Systemic Blood flow (Qs)
Qp = VO2 PV sat - PA sat
Qs =
VO2 Ao sat - MVO2
Calculation of Shunt Volume: Fick Principle p Qp =
Qs =
I di ti Indications ffor Surgery: S Qp/Qs Q /Q
VO2 PV sat - PA sat VO2 Ao sat -MVO2
Qp/Qs = Ao sat - MVO2
• < 1.5 :1
No surgery g y
• 1.5 - 2 :1
Consider surgery
• > 2 :1
Needs Surgical repair
PV sat – PA sat
Estimation of Shunt Magnitude 1. Chamber dilatation – ASD:
RA and RV
– VSD:
LA and LV
– PDA:
LA and LV
– AVCD:
All
2. Flow velocity
P l Pulmonary V Vascular l Di Disease • Compensatory response to increased pulmonary blood flow • Progressive process: medial hypertrophy and intimal hyperplasia: initially reversible •
Eventually irreversible
• Rate of progression variable • Lesion • Genetic factors
Irreversible Pulmonary Vascular Disease
• Complete AVCD
9 -12 months
• Large VSD
1-2 years
• Large PDA
1-2 years
• ASD
30 - 40 years
Sec ASD
VSD
Technical ec ca Feasibility
Uncomplicated U co p ca ed
Mild d to o moderately complicated
Mildly dy complicated
Complicated Co p ca ed
Symptomatic Sy po a c state
Usua y Usually Asymptomatic
So e es Sometimes symptomatic sy po a c symptomatic
