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Post-operative Management
Following the Unifocalization
Procedure in Patients with
TOF/PA/MAPCAs:
The Stanford Experience
Sandra Staveski RN, PhD, CPNP-AC, CNS, CCRN
Assistant Professor, Research in Patient Services/Heart Institute
Cincinnati Children’s Hospital Medical Center
Formerly Nurse Practitioner in Cardiovascular ICU at Lucile Packard Children’s
Hospital Stanford
No disclosures
Objectives
• Anatomical and physiologic considerations
• ICU course following the unifocalization
procedure
• Standardized management and daily goal
directed therapy
• Specific areas of nursing focus
Anatomical Considerations
• Less common, complex subset of TOF
– TOF/PA ~ 20% of TOF*; 30-65% have MAPCAs+
• Pulmonary blood supply:
– Collateral-dependent
– Severe abnormalities of native pulmonary arteries
– Marked heterogeneity between patients
• MAPCAs
– Derived from splanchnic vascular plexus
– May supply all pulmonary blood flow, be part of “dual
supply", or supply a single lung segment
– Propensity for stenosis++ *Baltimore-Washington Infant Study 1981-89
+Shimazaki
Y 1990
++Ma et al., 2014
Unifocalization Concept
Surgical reconstruction of the
pulmonary arterial tree, incorporating as
many lung segments as possible, by
maximal recruitment of true pulmonary
arteries and MAPCAs
Haworth and Macartney,1980
Malhotra and Hanley, 2009
Conceptual Goals
• To create a single-compartment,
unobstructed, low-pressure pulmonary
circulation in a separated, 2-ventricle
circulation as early as possible
• Achieve normal circulatory physiology
– Low RV systolic pressure (< 50% LVp)
•
•
•
•
Maximize lung segments incorporated
Minimize pulmonary vascular resistance
Eliminate VSD shunt
Eliminate proximal RV outflow obstruction
Bull & Somerville et al., 1995
Pulmonary Vascular Beds of
Children with TOF/PA/MAPCAs
• “Microclimates”
– Hypoperfused
– High pressure, high flow
– Normal
• Morphology and physiology determines
surgical approach and surgical approach
determines post-operative management
ICU Course Following
Unifocalization Surgery
Problems encountered - Cardiac
• Low cardiac output syndrome (LCOS)
– Often long cardiopulmonary bypass (CPB) times
– Pharmacologic support of RV function
• Monitor for tachycardia, high filling pressures
(RAp > 10-12 mmHg), and poor perfusion
– Dysrhythmias (sinus tachycardia, JET)
• Fever control (inflammation from CPB)
– Bleeding (suture lines, long CPB, coagulopathy)
• Tolerate mild permissive hypotension
– RV lines (relationship between RVp and SBP)
• Monitor measures of cardiac output
– Physical exam, bicarbonate, lactate, urine output
Bronicki, & Chang, 2011
– NIRS
ICU Course Following
Unifocalization Surgery
Problems encountered - Cardiac
• Treatment
– Low dose epinephrine and milrinone infusions
– Delayed sternal closure
– Normalization of pH and correction of alveolar
hypoxia with titration of end-expiratory pressure to
achieve a normal functional residual capacity
• Ventilation increases RV afterload
• Acidosis and hypoxemia increases RV afterload
– Inhaled nitric oxide (iNO)
• Judicious use of analgesia and sedation
– Chemical paralysis
Bronicki, & Chang, 2011
ICU Course Following
Unifocalization Surgery
Problems encountered – Pulmonary
Figure 1: Differen al Diagnosis
New or resolving
pulmonary
hemorrhage
Diaphragm
paralysis
Obstruc ve
breathing pa ern
Lung parenchymal
disease (contusion,
hematoma,
pneumonia,
atelectasis, RPE)
Airway
compression
Upper airway
obstruc on
Possible
Prolonged
Respiratory
Failure
Hemodynamic
altera ons
Asija et al., 2014
Stanford Pathway for TOF/PA/MAPCAs
ICU Course Following
Unifocalization Surgery
Problems encountered - Pulmonary
• Pulmonary reperfusion edema
– Incidence ~ 50%
– Severity of stenosis and bilateral unifocalizations are
associated with development of reperfusion injury
– Does not affect duration of respiratory failure and
mechanical ventilation
• Clinically self-limiting and does not appear to involve
vascular injury
Maskatia et al., 2012
Asija et al., 2013
Asija et al., 2014
Left-sided Lung Reperfusion Injury
PRE-OP
EARLY POST-OP
POD 3
POD 4
POD 1
POD 2
DISCHARGE
ICU Course Following
Unifocalization Surgery
Problems encountered - Pulmonary
• Treatment
– Judicious ventilation strategies
• Permissive hypercarbia
– Pulmonary toilet
– Diuretic therapy
ICU Course Following
Unifocalization Surgery
Problems encountered - Pulmonary
• Pulmonary hemorrhage
– Related to extensive dissection
• Other parenchymal processes
– Contusion, hematoma, altectasis, pneumonia
*Asija et al., 2013
ICU Course Following
Unifocalization Surgery
Problems encountered - Pulmonary
• Bronchospasm
– Frequent post-op complication
– > 70% of patients receive treatment for significant
airflow limitation and wheezing
• This is thought to be caused by the extensive dissection and
disruption of lymphatics and blood vessels around the
bronchopulmonary tree
• Treatment
– Ventilation strategies
– Pulmicort, albuterol (Continuous, ATC, and PRN)
– Analgesia, sedation, and at times chemical paralysis
Asija et al., 2013
Maskatia et al., 2012
ICU Course Following
Unifocalization Surgery
Problems encountered - Variety
• Phrenic nerve injury
– Hilar dissection, more frequent in reoperations
• Prolonged pleural fluid drainage
– Likely related to lymphatic disruption from
extensive mediastinal dissection
• Infection
– Increased incidence of 22q11 microdeletion,
DiGeorge syndrome and associated immune
dysfunction
• Acute, severe hepatic ischemia
– Low incidence (1-1.5%), but high mortality rate
– Causative factor(s) unclear; no cases > 10 years
ICU Course Following
Unifocalization Surgery
• Defense….
– Handwashing
– De-intensifying
– Getting them up and
moving
– Pulmonary toilet
– Early intervention with
infectious disease
processes
Standardize Post-operative
Management
CVICU Clinical Pathway Guideline (CPG) for patients with TOF/PA/MAPCAs
Inclusion Criteria: All patients undergoing unifocalization surgery age > or = to 3 months with no other significant cardiac
abnormalities. Includes patients undergoing partial unifocalization surgery via right or left lateral thoracotomy, with or without CPB.
Includes patients with delayed sternal closure, though the guidelines are initiated on the day of sternal closure.
CPG members- Ritu Asija, Catherine Krawczeski, Julie Bushnell (CVICU), Frank Hanley (CT surgery), Frandics Chan (Radiology), Sumit
Bhargava (Pulm), Stan Perry (Cath), Anna Messner (ENT), Vickie Arnold (RT), Alaina Kipps (3W), Chandra Ramamoorthy (CV anesth)
Monitoring
Cardiac
Resp
Fluids
Diagnostics
Medications/ IV
therapy
POD 0
Vital signs per
CVICU routine
CR monitoring
Central venous line
LA line and RV
line (if complete
repair)
Arterial line and
pacing wires secure
Breath sounds
CT drainage
Measure I/O, Foley
CXR
CBC, cardiac
metabolic panel
(CMP), coags,
ABG q 2 hr, lactate
q 2 until < 2
Antibiotic x 24
hours * [1],
milrinone [2],
epinephrine
dopamine **
fentanyl infusion
sedative infusion
(midazolam or
dexmetetomidine)
POD 1
Vital signs per
CVICU routine
CR monitoring
Central venous line
LA line and RV
line (if complete
repair)
Arterial line and
pacing wires secure
Breath sounds
CT drainage
Measure I/O, Foley
CXR
CBC, CMP, ABG q
6 and prn, lactate
prn
POD 2
Vital signs per
CVICU routine
CR monitoring
Central venous line
LA line and RV
line (if complete
repair)
Arterial line and
pacing wires secure
Breath sounds
CT drainage
Measure I/O, Foley
CXR
BMP, Ca, Mg,
PO4, ABG q 12 and
prn
POD 3
Vital signs per
CVICU routine
CR monitoring
Central venous line
Arterial line and
pacing wires secure
POD 4
Vital signs per
CVICU routine
CR monitoring
Central venous line
Arterial line and
pacing wires secure
POD 5
Vital signs per
CVICU routine
CR monitoring
Central venous line
Arterial line and
pacing wires secure
Breath sounds
CT drainage
Measure I/O
CXR
BMP, Ca, Mg,
PO4, ABG q 12 and
prn
Breath sounds
CT drainage
Measure I/O
CXR
BMP, Ca, Mg,
PO4, ABG q 12 and
prn
Breath sounds
CT drainage
Measure I/O
CXR
BMP, Ca, Mg, PO4
milrinone
epinephrine
dopamine
morphine infusion
sedative infusion
(midazolam or
dexmetetomidine)
acetaminophen atc
Zantac and any
milrinone
dopamine
furosemide and
additional diuretic
if required to
achieve negative
fluid balance
morphine infusion
sedative infusion
furosemide
acetaminophen atc
morphine prn
Zantac and any
other home antireflux meds
If patient has signs
of airflow
obstruction,
furosemide
acetaminophen atc
ibuprofen prn
morphine prn
Zantac and any
other home antireflux meds
If patient has signs
of airflow
furosemide
acetaminophen prn
ibuprofen prn
morphine prn
Zantac and any
other home antireflux meds
If patient has signs
of airflow
Asija et al., 2014
Stanford Pathway for TOF/PA/MAPCAs
Daily Goal Directed Therapy
Daily Goals
POD 0
POD 1
POD 2
POD 3
Cardiac and
respiratory
stability
Initiate
diuresis
with goal
negative
fluid
balance for
next
morning
Wean
ventilator
Continue
Continue
diuresis
diuresis
Discontinue Extubate
intracardiac
lines
Wean
ventilator
Wean
inotropes
POD 4
POD5
Nutrition
Pulmonary
toilet
Wean
NIPPV as
tolerated
Nutrition
Pulmonary
toilet
Wean
NIPPV as
tolerated
Asija et al., 2014
Stanford Pathway for TOF/PA/MAPCAs
Specific Areas for Nursing
Focus
• Keeping everyone’s eye on the prize
– Personal patient safety officer
– Nursing assessment is key!
• Establishment of physical and
occupational therapy
– Feeding evaluation
– Movement/activities of daily living
• Get them up and moving as soon as it is safe
• Pulmonary toilet
Specific Areas for Nursing
Focus
• Nutrition
– Early enteral feeds
• Comfort management
– Standardized comfort management protocols
– Pharmacologic and nonpharmacologic
supports
• Family education
– Starts pre-operatively and goes throughout
their child’s hospitalization
Important Considerations with
Family Education
• “Tending the garden” – pulmonary
vascular bed (long-term management)
– Growth and development of pulmonary bed is commensurate
with visceral growth
– Lung perfusion scans
• Hospital discharge
• Before further surgical interventions
– Surveillance cardiac catheterization (6 months to 1
year) to assess the pulmonary vasculature
– Echocardiograms should be done routinely to monitor
Asija et al., 2014
right ventricular function
Hanley, 2010
Mainwaring et al., 2013
Important Considerations with
Family Education
• Children undergone unifocalization
surgery without intracardiac repair have
aortopulmonary shunts providing PBF
– At risk for specific complications
• Thrombosis or pulmonary overcirculation
• Require close monitoring for any changes in their
oxygen saturation or ventricular overload
Asija et al., 2014
Important Considerations with
Family Education
• Complete intracardiac repair
– Monitored for recurrent PA stenosis, right
ventricle-to-pulmonary artery conduit stenosis
or insufficiency, and RV hypertension/dilation
• Endocarditis prophylaxis
– 6 months after repair, and beyond that for
patients who have repairs that include a prior
episode of endocarditis, and have residual
intracardiac shunt who remain cyanotic or have
patch leaks
Asija et al., 2014
Thank you!
I would like to acknowledge the work and
support of my colleagues at Lucile Packard
Children’s Hospital Stanford
Frank Hanley MD
V. Mohan Reddy MD
Stephen Roth MD, MPH
Ritu Asija MD