student

1. Practical Nursing
Considerations for Caring for
the Adult Congenital Heart
Patient
Kristi Ryan, APN

2. Objectives
• Understand practical nursing considerations for
caring for the adult patient with congenital heart
disease
• Common congenital heart complications that bring
patients to the hospital
• What makes these patients different?... Beyond
the anatomy and physiology

3. Normal Cardiac Anatomy

4. Cardiac Pressures

5. ACHD Defects
• Classifications: Simple,
Moderately complex,
and Highly complex
• ACHA (Adult Congenital
Heart Association)
ACHAHeart.org

6. Unrepaired:
• Small ASD
• Small VSD
• Mild PS
Repaired:
• PDA
• ASD
• VSD
Repaired/Unrepaired:
• Isolated AoV
• Isolated MV
• PFO
Simple CHD
ACHAHeart.org
One visit to ACHD program then can be followed by
general cardiologist or congenital cardiologist
Any new issues should be re-evaluated by ACHD clinic

7. Moderately complex CHD
• ALCAPA
• P/TAPVR
• AV Canal
• Sinus venosus ASD
• Coarctation
• Ebstein’s anomaly
• RVOTO
• PV regurgitation(> mod)
• PV stenosis (> mod)
• Subvalvar or supravalvar AS
• Tetralogy of Fallot
• VSD with valve problem or
obstructions
Follow up every 2 years or more with ACHD program
ACHAHeart.org

8. Highly Complex CHD
• Eisenmenger syndrome
• All cyanotic patients
• L-TGA
• DORV
• Mitral atresia
• Pulmonary atresia
• Shone’s complex
• Single ventricle
• D-TGA
• Tricuspid atresia
• Truncus
• Heterotaxy
• s/p BT shunt
• s/p conduit
• s/p double switch
• Fontan
• Mustard/Senning
• Norwood
• Rastelli
At least annual visit at ACHD program
ACHAHeart.org

9. Common Adult Lesions
CHD diagnosed in adulthood
• Atrial Septal Defect (ASD)
• Coarctation of Aorta
• Congenitally Corrected Transposition (L-TGA)
• Patent foramen ovale

10. Re-operation/Intervention
• Aortic regurgitation
• Aortic stenosis
• Aortic root enlargement
• Coarctation of aorta
• Pulmonary
regurgitation/stenosis
• RV to PA conduit failure
• Fontan revision
• Mustartd/Senning baffle
obstruction
• Arrhythmia
interventions:
– ICD/pacemakers
– Ablations
– Cardioversions

11. Atrial Septal Defects (ASD)

12. ASD
• ~1/3 diagnosed as an adult
• Multiple types of ASDs:
– PFO, secundum, primum, sinus venosus
• Symptoms and presentation depends
– Size of hole
– Location
– Any other defects, comorbidities
– Age
• Closure: cath lab vs. surgery
• Eisenmenger Syndrome- long term complication if
unrepaired

13. Eisenmenger Syndrome

14. Tetralogy of Fallot (TOF)
Four abnormalities:
• Ventricular septal
defect (VSD)
• RV outflow tract
obstruction
(Subpulmonary
stenosis)
• Right ventricular
hypertrophy
• Overriding aorta

15. TOF: Initial Palliation
• Need to establish
pulmonary blood
flow
• Classic BT Shunt
ligated the
subclavian artery

16. TOF: Surgical Repair
Valve Sparing Repair RV to PA Conduit

17. Adult RV to PA Replacement
Transcatheter valve replacement
– Melody Transcatheter Pulmonary Valve
– Edwards Sapien XT Pulmonary Valve
Surgical replacement
– Redo sternotomy

18. Coarctation of Aorta
Narrowing of aorta
• Diagnosed any time
in life
• 4 extremity BP helpful
in diagnosis and
monitoring
• Commonly associated
with bicuspid aortic
valve

19. Repair of coarctation
Surgical Intervention Catheter intervention

20. Where will you see them?
• Emergency room
• Operating room and post op (CVICU)
• Cardiology and medical admissions
• Labor & delivery
• Psychology admissions

21. Reason for hospitalization
Shikhar Agarwal et al. J Am Heart Assoc 2016;5:e002330
Complex ACHD Simple ACHD
(ASD/PFO excluded)
Simple ASD/PFO
• CHF
• Respiratory
disorders
• Arrhythmias
• Valve disease
• Coronary artery
disease
• Arrhythmias
• CVA- 26%
• Arrhythmia

22. Tips for admitting ACHD
• Identify patient as ACHD patient
– Were they born with the defect?
– Do they have a scar in the middle of chest or a
thoracotomy?
– Did they see a cardiologist as a child?
• Consult the ACHD team!
• Only ~10% of ACHD patients in the US are
currently getting the ACHD care that is
recommended

23. Why are they different?
• Increased incidence of comorbidities: obesity,
hypertension, smoking, kidney disease
• Psychological impact of- depression and anxiety
more common
• Social considerations
• Quality of life

24. Summary
• ACHD patients are special
• There are some nursing considerations that must
be considered- the key is ASSESSMENT and
consultation!
• Nearly all congenital heart patients need life long
follow up with an ACHD specialist. Many have
been lost to follow up. They may show up in your
office, ED or department.
• There are lots of resources available to you. Never
hesitate to call our office.

25. “…I think those of us who have had life-threatening
illness have been blessed with a knowledge of how
precious life is.”
-Dylan Henricks
“To be able to survive in spite of poor odds, there has
to be a strong will and a passion for life.”
-Tara Shane
“As a child, I was very ashamed of all my scars and
never wanted anyone to see them. As I’ve gotten
older, I’ve realized that without those scars I wouldn’t
be alive today and able to do the great things I’ve
done.”
-Kayla Pepmeyer

26. Kristi Ryan, APN
Adult Congenital Heart
Childrens Hospital of Illinois
OSF St. Francis
Kristi.n.ryan@osfhealthcare.org
309-624-3893