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. Simple CHD
Unrepaired:
Repaired:
Repaired/Unrepaired:
Small ASD
PDA
Isolated AoV
Small VSD
ASD
Isolated MV
Mild PS
VSD
PFO
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
ACHAHeart.org

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 At least annual visit at ACHD program
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
TOF – Pulmonary valve insufficiency; RV-PA conduit stenosis, regurgitation
TGA s/p arterial switch – may have aortic valve insufficiency
TGA s/p senning – systemic ventricular dysfunction, baffle leak, arrhythmia
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
Fontan revision
Aortic stenosis
Mustartd/Senning baffle obstruction
Aortic root enlargement
Arrhythmia interventions:
Coarctation of aorta
Pulmonary regurgitation/stenosis
ICD/pacemakers
Ablations
RV to PA conduit failure
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. RN Tidbit: WATCH FOR AIR
Eisenmenger Syndrome
RN Tidbit: WATCH FOR AIR
BUBBLES IN IV LINES!!!

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
RN Tidbit: NO BP IN ARM
WITH A CLASSIC BT
SHUNT!!!

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
Melody valve needs 22 fr venous introducer
Coarctation stents must be done in peds cath lab – size of stents, bi-plane cameras

18. Coarctation of Aorta Diagnosed any time in life
4 extremity BP helpful in diagnosis and monitoring
Commonly associated with bicuspid aortic valve
Describe defect, diagnosed as infant, teen or adult. How diagnosed…
Narrowing of aorta

19. RN Tidbit: NO BP IN ARM WITH A SUBCLAVIAN FLAP REPAIR!
Repair of coarctation
Surgical Intervention
Catheter intervention
RN Tidbit: NO BP IN ARM
WITH A SUBCLAVIAN
FLAP REPAIR!
Coarc repair and reintervention

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

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
THANK YOU!!!