Download Single Ventricle Physiology

N EONATAL C ARDIAC I NTENSIVE C ARE
M ANAGEMENT OF THE P OSTOPERATIVE
N EONATAL C ARDIAC S URGICAL PATIENT
ANTHONY C. CHANG, MD, MBA, MPH
MEDICAL DIRECTOR, HEART INSTITUTE
CHILDREN’S HOSPITAL OF ORANGE COUNTY
[email protected]
N EONATAL C ARDIAC I NTENSIVE C ARE
T OP T EN C ONCEPTS
ANTHONY C. CHANG, MD, MBA, MPH
MEDICAL DIRECTOR, HEART INSTITUTE
CHILDREN’S HOSPITAL OF ORANGE COUNTY
[email protected]
PDA in Ductal-dependent Lesions
Systemic Flow
(HLHS)
Pulmonary Flow
(Pulmonary Atresia)
Transposition of the Great Arteries/ Mixing
Cardiac Output in the Neonate
Evolution of Pathophysiology
Supraventricular Tachycardia Types
Reentrant Type
Ectopic Type
Single Ventricle Physiology
Calculation
Qp:Qs =
O2% (Ao –MV)
_____________
O2% (PV – PA)
In single ventricle,
O2% (Ao) = O2% (PA)
O2% (Ao – MV) = 25%
O2% PV = 95%
Single Ventricle Physiology
Calculation
Qp:Qs =
25%
_____________
(95% –O2%Ao)
In single ventricle,
O2% (Ao) = O2% (PA)
O2% (Ao – MV) = 25%
O2% PV = 95%
Single Ventricle Physiology (85%)
Calculation
Qp:Qs =
25%
_____________
(95% – 85%)
=
25%
____
10%
=
2.5: 1
Single Ventricle Physiology (85%)
Calculation
Qp:Qs =
40%
_____________
(95% – 85%)
=
40%
____
10%
=
4: 1 (!)
Single Ventricle Physiology (85%)
Calculation
Qp:Qs =
40%
_____________
(90% – 85%)
=
40%
____
5%
=
8: 1 (!!)
RV/LV Interaction
Examples
Pressure overload
Pulmonary
hypertension
Volume overload
TAPVR/ Ebstein’s
Diastolic dysfunction
Truncus s/p repair
Other
HLHS s/p Norwood
Pulmonary Hypertension and PVR
PAP/PBF = PVR
Pulmonary Artery
Pressure
Pulmonary Blood
Flow
Pulmonary
Vascular
Resistance
VSD
CDH
Cardiopulmonary Interaction
Examples
Lung hypoplasia
Ebstein’s anomaly
Lung disease
Meconium aspiration
Overventilation
Other
TOF with absent PV
Prematurity and Pulmonary Vascular
Resistance
Normal
Premature
Ductal-dependent systemic blood flow
lesions (HLHS) are much more
vulnerable to Qp:Qs imbalance due to
larger PDA.
The only effective mixing site in TGA is at
the atrial level (not PDA) so TGA is not a
ductal-dependent lesion.
The only effective means that a neonate
can increase cardiac output is by heart
rate (not volume or increased inotropy).
Pathophysiology can be progressive
(such as supracardiac TAPVR and
pulmonary venous obstruction).
In any neonate with tachycardia that is
not responding to conventional therapy,
suspect an ectopic mechanism.
In HLHS, the estimated Qp:Qs is usually
underestimated (as there is low cardiac
output and pulmonary venous
desaturation).
RV/LV interaction problems in certain
clinical situations (pulmonary
hypertension, Ebstein’s anomaly, TAPVR,
HLHS, etc).
Pulmonary hypertension in neonatal CHD
and prognosis often relates to whether
pulmonary blood flow is increased or not.
Cardiopulmonary interaction in CHD is
essential especially with ventilation and
lung disease.
Pulmonary vascular resistance falls more
rapidly in premature neonates with
implications for CHD.
Maintain a healthy work/life balance like
the cardiac cycle (systole/diastole).
Work/Life Balance (Cardiac Cycle)
Systole
Diastole
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{
A=sum(Outcome_Treated[,i])
B=length(Outcome_Treated[,i])-A
X=sum(Outcome_Control[,i])
Y=length(Outcome_Control[,i])-X
ABXY[i,]=c(A,B,X,Y)
table_temp=matrix(ABXY[i,],byrow=T,ncol=2)
P_val=rbind(P_val,fisher.test(table_temp)$p)
Conf_int=rbind(Conf_int,fisher.test(table_temp)$conf.int[1:2])
OR=rbind(OR, fisher.test(table_temp)$estimate[[1]])
}
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Moonrise over Laguna Beach, California
Ductal-dependent Systemic Blood Flow
Aortic Stenosis
Coarctation of the aorta
Interrupted aortic arch
Hypoplastic left heart syndrome
Ductal-dependent Pulmonary Blood Flow
Critical pulmonary stenosis
Pulmonary atresia
Tricuspid atresia
Tetralogy of Fallot and pulmonary stenosis/atresia
Ebstein’s anomaly and pulmonary stenosis/atresia
Single ventricle and pulmonary stenosis/atresia
A one-week old neonate who is most
likely to have a pulmonary hypertensive
crisis after cardiac surgery is:
a. Total anomalous pulmonary venous connection
(infracardiac) s/p repair
b. Truncus arteriosus s/p repair
c. Transposition of the great arteries s/p arterial switch
d. Hypoplastic left heart syndrome s/p Norwood
e. Tricuspid atresia with pulmonary atresia s/p shunt
Total Anomalous Pulmonary Venous
Connection
Preoperative problems
Obstructive/ Pulmonary disease
Non-obstructive/ Shunt
Total Anomalous Pulmonary Venous
Connection (Surgery)
Postoperative problems
Pulmonary hypertension
Noncompliant left ventricle
Atrial ectopic tachycardia
Truncus Arteriosus
Preoperative problems
Increased pulmonary blood flow
Biventricular volume overload
Mesenteric ischemia
22q11 microdeletion
Truncus Arteriosus (Surgery)
Postoperative problems
Right ventricular dysfunction
Junctional ectopic tachycardia
Pulmonary hypertension
Coronary ischemia
Transposition of the Great Arteries
(Arterial Switch Operation)
Postoperative problems
Left ventricular ischemia
Pulmonary hypertension
Hypoplastic Left Heart Syndrome
Preoperative problems
Low systemic blood flow
Excessive pulmonary blood flow
Mesenteric ischemia
Hypoxemia
Inadequate atrial septal opening
Hypoplastic Left Heart Syndrome
(Norwood Operation)
Postoperative problems
Ventricular dysfunction
Shunt issues
Residual arch obstruction
Tricuspid atresia
Preoperative problems
Hypoxemia
Tricuspid atresia
(Post-BT Shunt or PA Band)
Postoperative problems
Excessive pulmonary blood
flow
Inadequate pulmonary blood
flow
A one-week old neonate who is most
likely to have a pulmonary hypertensive
crisis after cardiac surgery is:
a. Total anomalous pulmonary venous connection
(infracardiac) s/p repair
b. Truncus arteriosus s/p repair
c. Transposition of the great arteries s/p arterial switch
d. Hypoplastic left heart syndrome s/p Norwood
e. Tricuspid atresia with pulmonary atresia s/p shunt
A one-week old neonate who is most
likely to have a pulmonary hypertensive
crisis after cardiac surgery is:
a. Total anomalous pulmonary venous connection
(infracardiac) s/p repair
b. Truncus arteriosus s/p repair
c. Transposition of the great arteries s/p arterial switch
d. Hypoplastic left heart syndrome s/p Norwood
e. Tricuspid atresia with pulmonary atresia s/p shunt
A one-day old neonate has an arterial
blood gas that shows a base deficit of 12. In addition, the oxygen saturation is
74%. The least likely diagnosis is:
a. Total anomalous pulmonary venous connection
(infracardiac) with obstruction
b. Tetralogy of Fallot with pulmonary atresia
c. Transposition of the great arteries with pulmonary
hypertension
d. Hypoplastic left heart syndrome with a restrictive
ASD
e. Ebstein’s anomaly with pulmonary atresia
Ebstein’s Anomaly
Preoperative problems
Hypoxemia
Inadequate antegrade flow
Lung hypoplasia
Low cardiac output
Supraventricular tachycardia
Ebstein’s Anomaly
(Shunt vs Starne’s Operation)
Postoperative problems
Low cardiac output
RV/LV interaction
Lung hypoplasia
Supraventricular tachycardia
A one-day old neonate has an arterial
blood gas that shows a base deficit of 12. In addition, the oxygen saturation is
74%. The least likely diagnosis is:
a. Total anomalous pulmonary venous connection
(infracardiac) with obstruction
b. Tetralogy of Fallot with pulmonary atresia
c. Transposition of the great arteries with pulmonary
hypertension
d. Hypoplastic left heart syndrome with a restrictive
ASD
e. Ebstein’s anomaly with pulmonary atresia
A one-day old neonate has an arterial
blood gas that shows a base deficit of 12. In addition, the oxygen saturation is
74%. The least likely diagnosis is:
a. Total anomalous pulmonary venous connection
(infracardiac) with obstruction
b. Tetralogy of Fallot with pulmonary atresia
c. Transposition of the great arteries with pulmonary
hypertension
d. Hypoplastic left heart syndrome with a restrictive
ASD
e. Ebstein’s anomaly with pulmonary atresia
A neonate with hypoplastic left heart
syndrome is admitted to ICU on PGE1
with a oxygen saturation of 87%. What is
the estimated Qp:Qs?
a. 3:1
b. 2:1
c. 1:1
d. 0.5:1
e. Cannot be calculated based on only this data
Single Ventricle Physiology
Criteria
Systemic and pulmonary
venous return mix in a
common chamber
and
Systemic and pulmonary
blood flow is
proportional to the
respective resistances
only (no outflow tract
obstruction)
Single Ventricle Physiology (HLHS)
Criteria
Systemic and pulmonary
venous return mix in a
common chamber
and
Systemic and pulmonary
blood flow is
proportional to the
respective resistances
only (no outflow tract
obstruction)
Single Ventricle Physiology (HLHS)
Criteria
Systemic and pulmonary
venous return mix in a
common chamber and
Systemic and pulmonary
blood flow is
proportional to the
respective resistances
only (no outflow tract
obstruction)
Single Ventricle Physiology (HLHS)
Criteria
Systemic and pulmonary
venous return mix in a
common chamber and
Systemic and pulmonary
blood flow is
proportional to the
respective resistances
only (no outflow tract
obstruction) Single Ventricle Physiology (AV Canal)
Criteria
Systemic and pulmonary
venous return mix in a
common chamber
and
Systemic and pulmonary
blood flow is
proportional to the
respective resistances
only (no outflow tract
obstruction)
Single Ventricle Physiology (AV Canal)
Criteria
Systemic and pulmonary
venous return mix in a
common chamber and
Systemic and pulmonary
blood flow is
proportional to the
respective resistances
only (no outflow tract
obstruction)
Single Ventricle Physiology (AV Canal)
Criteria
Systemic and pulmonary
venous return mix in a
common chamber and
Systemic and pulmonary
blood flow is
proportional to the
respective resistances
only (no outflow tract
obstruction) Single Ventricle Physiology
Calculation
Qp:Qs =
O2% (Ao –
MV)
_____________
O2% (PV – PA)
In single ventricle,
O2% (Ao) = O2% (PA)
O2% (Ao – MV) = 25%
O2% PV = 95%
Single Ventricle Physiology
Calculation
Qp:Qs =
25%
_____________
(95% –O2%Ao)
In single ventricle,
O2% (Ao) = O2% (PA)
O2% (Ao – MV) = 25%
O2% PV = 95%
Single Ventricle Physiology (87%)
Calculation
Qp:Qs =
25%
_____________
(95% – 87%)
=
25%
____
8%
=
3: 1
A neonate with hypoplastic left heart
syndrome is admitted to ICU on PGE1
with a oxygen saturation of 87%. What is
the estimated Qp:Qs?
a. 3:1
b. 2:1
c. 1:1
d. 0.5:1
e. Cannot be calculated based on only this data
A neonate with hypoplastic left heart
syndrome is admitted to ICU on PGE1
with a oxygen saturation of 87%. What is
the estimated Qp:Qs?
a. 3:1
b. 2:1
c. 1:1
d. 0.5:1
e. Cannot be calculated based on only this data
Of the following lesions, the one that is
most vulnerable to excessive pulmonary
blood flow (relative to systemic blood
flow) and therefore be in shock is:
a. Hypoplastic left heart syndrome
b. Tetralogy of Fallot with severe pulmonary stenosis
c. Tricuspid atresia with pulmonary atresia
d. Ebstein’s anomaly with functional pulmonary atresia
e. Asplenia with double outlet right ventricle and
common atrioventricular canal
PULMONARY VASCULAR RESISTANCE
Low lung volume leads to
large vessel collapse
High lung volume leads to
capillary compression
PVR lowest at FRC
CARDIAC INTENSIVE CARE
HYPERCYANOTIC SPELL
Decrease in SVR leading to decreased
pulmonary blood flow
CARDIAC INTENSIVE CARE
ELEVATED ATRIAL PRESSURE
CARDIAC INTENSIVE CARE
ELEVATED SVC/RA PRESSURE
Pericardial effusion
TV stenosis/insufficiency
RV disease
RV hypertension
PV disease
Pulmonary embolus
Pulmonary hypertension
Lung pathology
Elevated PVR
Left-sided disease
CARDIAC INTENSIVE CARE
ELEVATED LA PRESSURE
Pericardial effusion
MV stenosis/insufficiency
LV disease
LV hypertension
AoV disease
Systemic hypertension
Dysrhythmias
AV block
Junctional ectopic tachycardia
CARDIAC INTENSIVE CARE
CARDIAC OUTPUT MANIPULATION
CARDIAC INTENSIVE CARE
PRELOAD
Increase in preload (from A to D) increases stroke volume
but at the cost of edema if atrial pressure is > 15mmHg
Reference
Chang AC (2006). Heart Failure in Children and Young Adults. Elsevier.
CARDIAC INTENSIVE CARE
CONTRACTILITY
As inotropic state improves from A to C, stroke volume
increases at any given LA pressure
Reference
Chang AC (2006). Heart Failure in Children and Young Adults. Elsevier.
CARDIAC INTENSIVE CARE
AFTERLOAD
As afterload decreases from C to D as well as from A to
B, stroke volume increases
Reference
Chang AC (2006). Heart Failure in Children and Young Adults. Elsevier.
CARDIAC PHYSIOLOGY
VOLUME AND PRESSURE OVERLOAD
Volume- eccentric hypertrophy with sarcomeres added in
series
Pressure- concentric hypertrophy with sarcomeres added
in parallel
CARDIAC INTENSIVE CARE
QUESTION #1
The shaded area is:
a. Stroke work
b. Stroke volume
c. Ejection fraction
d. Cardiac output
e. None of the above
CARDIAC INTENSIVE CARE
QUESTION #1
The shaded area is:
a. Stroke work
b. Stroke volume
c. Ejection fraction
d. Cardiac output
e. None of the above
CARDIAC PHYSIOLOGY
THE PRESSURE-VOLUME LOOP
CARDIAC PHYSIOLOGY
VOLUME AND PRESSURE OVERLOAD
Volume- increased stroke work but not potential energy
Pressure- increased stroke work and potential energy
CARDIAC INTENSIVE CARE
QUESTION #2
The red lines denote:
a. Decreased inotropy and
increased compliance
b. Increased inotropy and
increased compliance
c. Decreased inotropy and
decreased compliance
d. Increased inotropy and
decreased compliance
e. None of the above
CARDIAC INTENSIVE CARE
QUESTION #2
The red lines denote:
a. Decreased inotropy and
increased compliance
b. Increased inotropy and
increased compliance
c. Decreased inotropy and
decreased compliance
d. Increased inotropy and
decreased compliance
e. None of the above
HEART FAILURE
SYSTOLIC AND DIASTOLIC
DYSFUNCTION
ESPVR line “depressed” towards x-axis
EDPVR line “elevated” towards y-axis
Hemodynamic “vise”
Reference
Chang AC (2006). Heart Failure in Children and Young Adults. Elsevier.
HEART FAILURE
ACUTE AND CHRONIC HEART FAILURE
ESPVR- depresses
EDPVR- elevates
Stroke volume
Reference
Chang AC (2006). Heart Failure in Children and Young Adults. Elsevier.
CARDIAC INTENSIVE CARE
QUESTION #3
The pressure volume loop seen is
most consistent with:
a. Aortic stenosis
b. Mitral stenosis
c. Aortic insufficiency
d. Mitral insufficiency
e. Tricuspid insufficiency
CARDIAC INTENSIVE CARE
QUESTION #3
The pressure volume loop seen is
most consistent with:
a. Aortic stenosis
b. Mitral stenosis
c. Aortic insufficiency
d. Mitral insufficiency
e. Tricuspid insufficiency
CARDIAC PHYSIOLOGY
VALVE STENOSIS [AORTIC]
During ventricular ejection, LV pressure exceeds aortic
pressure.
Stroke volume is decreased; stroke work can be about the
same but potential energy is increased.
CARDIAC PHYSIOLOGY
VALVE STENOSIS [MITRAL]
During ventricular filling (diastole), LA pressure exceeds
LVEDP.
Both stroke volume and stroke work are decreased and
the potential energy is unchanged.
CARDIAC PHYSIOLOGY
VALVE INSUFFICIENCY [AORTIC]
During ventricular relaxation, blood flows backwards
from aorta into to the LV. Pulse pressure widens.
Both stroke volume and stroke work are increased but
potential energy can be about the same.
CARDIAC PHYSIOLOGY
VALVE INSUFFICIENCY [MITRAL]
During systole, LV ejects blood back into the LA as well
as into the aorta. The LAP and the v-wave are increased.
Both stroke volume and stroke work are increased but
potential energy can be about the same.
CARDIAC PHYSIOLOGY
VALVE INSUFFICIENCY [MITRAL]
Acute- increased LA pressure
with pulmonary edema and
decreased cardiac output
Chronic- increased LA size with
little if any pulmonary edema
and normal cardiac output
CARDIAC PHYSIOLOGY
VALVE INSUFFICIENCY [MITRAL]
Compensated vs Decompensated
HEART FAILURE
LEFT-TO-RIGHT SHUNTS
DOUBLE SHUNTS
Qs = 2.45
Qp = 12.25
MV = 70%
Qep = 2.45
PV = 95%
Qp = 12.25
RA = 80%
LA = 95%
Qp = 12.25 L/min
Qs = 2.45 L/min
Qep = 2.45 L/min
L-R shunt = 9.80 L/min
RV = 90%
Qp = 12.25
LV = 95%
Qep = 2.45
PA = 90%
Qp = 12.25
Ao = 95%
Qs = 2.45
Reference
Garson A et al. Science and Practice of Pediatric Cardiology. Elsevier. p.974-975.
HEART FAILURE
LEFT-TO-RIGHT SHUNTS
DOUBLE SHUNTS (ASD ALONE)
Qs = 2.45
Reference
Qp = 4.08
MV = 70%
Qep = 2.45
PV = 95%
Qp = 4.08
RA = 80%
Qp = 4.08
LA = 95%
Qep = 2.45
RV = 80%
Qp = 4.08
LV = 95%
Qep = 2.45
PA = 80%
Qp = 4.08
Ao = 95%
Qs = 2.45
Qp = 4.08 L/min
Qs = 2.45 L/min
Qep = 2.45 L/min
L-R shunt ASD = 1.63
L/min
And since
L-R shunt = 9.80 L/min
Therefore
L-R shunt VSD = 8.17
L/min
Garson A et al. Science and Practice of Pediatric Cardiology. Elsevier. p.974-975.
CARDIAC PHYSIOLOGY
ATRIAL SEPTAL DEFECT
Preoperative Pathophysiology
Left to Right Shunt
Increased Pulmonary Flow
RV Volume overload
CARDIAC INTENSIVE CARE
ATRIAL SEPTAL DEFECT
Preoperative Pathophysiology
Left to Right Shunt
Increased Pulmonary Flow
RV Volume overload
Partial Anomalous PV Return
LV-RA Shunt (Gerbode Defect)
CA-RA Fistula
Cerebral AVM
CARDIAC INTENSIVE CARE
VENTRICULAR SEPTAL DEFECT
Preoperative Pathophysiology
Left to Right Shunt
Increased Pulmonary flow
and Pressure
LV Volume Overload
CARDIAC INTENSIVE CARE
VENTRICULAR SEPTAL DEFECT
Preoperative Pathophysiology
Left to Right Shunt
Increased Pulmonary flow
and Pressure
LV Volume Overload
Patent Ductus Arteriosus
Aortopulmonary Window
CARDIAC INTENSIVE CARE
COMMON ATRIOVENTRICULAR CANAL
Preoperative Pathophysiology
Common Mixing
Increased Pulmonary Blood
Flow and Pressure
AV Valve Regurgitation
Biventricular Volume Overload
Cyanosis
CARDIAC INTENSIVE CARE
COMMON ATRIOVENTRICULAR CANAL
CARDIAC INTENSIVE CARE
TETRALOGY OF FALLOT
CARDIAC INTENSIVE CARE
AORTIC STENOSIS
PREOPERATIVE PATHOPHYSIOLOGY
LV PRESSURE OVERLOAD
LV CONCENTRIC HYPERTROPHY
SUBENDOCARDIAL ISCHEMIA
COEXISTING
LESIONS
CARDIAC INTENSIVE CARE
CARDIAC INTENSIVE CARE
DUCTAL DEPENDENT LESIONS
CARDIAC INTENSIVE CARE
CASE
NEONATE WITH CYANOSIS AT 6 HOURS OF AGE
PULSE OXIMETRY 72% IN RA AND 79% ON OXYGEN
NO RESPIRATORY DISTRESS
CARDIAC INTENSIVE CARE
DUCTAL DEPENDENT LESIONS
PULMONARY BLOOD FLOW
CRITICAL PS
PULMONARY ATRESIA (PAT)
TOF WITH PS OR PAT
SV W/PS OR PAT
TRICUSPID ATRESIA
EBSTEIN’S ANOMALY
CARDIAC INTENSIVE CARE
DUCTAL DEPENDENT LESIONS
PULMONARY BLOOD FLOW
CRITICAL PS
CARDIAC INTENSIVE CARE
TETRALOGY OF FALLOT/PULMONARY ATRESIA
CARDIAC INTENSIVE CARE
CASE
NEONATE WITH CYANOSIS AND SHOCK AT 18 HOURS
OF AGE
PULSE OXIMETRY 79% IN RA AND 81% ON OXYGEN
SEVERE RESPIRATORY DISTRESS
CARDIAC INTENSIVE CARE
HYPOPLASTIC LEFT HEART SYNDROME
PREOPERATIVE PATHOPHYSIOLOGY
SINGLE VENTRICLE PHYSIOLOGY
INCREASED QP:QS
RV VOLUME OVERLOAD
EXTRACARDIAC ISSUES
CARDIAC INTENSIVE CARE
HYPOPLASTIC LEFT HEART SYNDROME
PREOPERATIVE PATHOPHYSIOLOGY
CARDIAC INTENSIVE CARE
MISCELLANEOUS
CARDIAC INTENSIVE CARE
TRANSPOSITION OF THE GREAT ARTERIES
PREOPERATIVE PATHOPHYSIOLOGY
MIXING
ASD (MOST EFFECTIVE)
VSD
PDA
COEXISTING PPHN
(REVERSE DIFFERENTIAL
CYANOSIS)
CARDIAC PHYSIOLOGY
TRANSPOSITION OF THE GREAT ARTERIES
CARDIAC INTENSIVE CARE
TRUNCUS ARTERIOSUS
PEROPERATIVE PATHOPHYSIOLOGY
COMMON MIXING
INCREASED PULMONARY
BLOOD FLOW AND PRESSURE
BIVENTRICULAR VOLUME OVERLOAD
TRUNCAL STENOSIS/ INSUFFICIENCY
EXTRACARDIAC ISSUES
ISCHEMIC BOWEL SYNDROME
MICRODELETION 22Q11
CARDIAC INTENSIVE CARE
MULTIDISCIPLINARY TEAM