Pamela Heggie, RN BN Clinic Coordinator Northern Alberta Adult Congenital Heart (NAACH) Clinic Mazankowski Heart Institute

Transcription

1 Pamela Heggie, RN BN Clinic Coordinator Northern Alberta Adult Congenital Heart (NAACH) Clinic Mazankowski Heart Institute

2 Brief Overview of Congenital Heart Disease Spectrum disorder Treatment & Impact on Health Care Care Considerations for Adult Congenital Heart Disease (ACHD) Patients Common Complaints Pearls of Practice Resources

3 Congenital heart disease is the most common birth defect Spectrum of Disease Simple Isolated valve disease, Small shunt lesions Moderately Complex CoA, ToF, Shunt/valve combo Highly Complex TGA, Single ventricle, Cyanotic AHA Guidelines for the Management of Adult Congenital Heart Disease 2008 (CACHNet)

4 1. Shunt Lesions: blood can shunt or flow somewhere that it shouldn t.

5 2. Obstructive Lesions: Obstruction of the normal blood flow in, around or out of the heart.

6 3. Complex Lesions: Combination of shunt/obstruction

7 Successful pediatric care= More adult survivors > 90% expected to reach adulthood Repaired but not cured Prevalence is now greater than occurrence 1.8% cardiologists with formal ACHD training (Diagnosis and Management of Adult Congenital Heart Disease, Gatzoulis et al, 2003, 2 nd edition 2011, 3 rd edition coming 2017)

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9 A) Approximately 6,000 people B) Approximately 26,000 people C) Approximately 60,000 people D) Approximately 160,000 people E) Approximately 260,000 people

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11 No longer an oddity Life long care Special concerns for moderate and complex disease patients Common issues

12 Good Prognostic Factors Poor Prognostic Factors Early repair Biventricular heart Systemic ventricle is LV No residual hemodynamic lesions Good functional capacity Early surgical era Pulmonary hypertension Ventricular dysfunction Arrhythmias Multiple lesions

13 No previous cardiac history August 2013 ER- Palpitations EASE Referral Seen by cardiology BP 149/80 BP Tru- 167/87 (ave of 5 readings) Echo in Normal bi-ventricular size/function, mild AR (although AV not optimally visualized) Loud neck bruits, pulsatile neck

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15 A) Holter Monitor B) 24 Hour Ambulatory BP Monitor C) Repeat Echocardiogram D) 4- Limb Blood Pressure E) CT Head and Neck

16 Physical Assessment Findings Systolic ejection click 4 Limb BP Right Arm: 165/88 Right Leg: 129/82 Left Arm: 169/99 Left Leg: 125/87 Radial/Femoral delay Complains of early leg fatigue

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18 A) Atrial Septal Defect B) Isolated Bicuspid Aortic Valve C) Isolated Coarctation of Aorta D) Coarctation of Aorta & Bicuspid Aortic Valve E) Peripheral Vascular Disease

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24 A) True B) False

25 Transposition of the Great Arteries, VSD S/P Balloon Atrial Septostomy (August 1975)

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27 A) Arterial switch repair with VSD closure B) Mustard repair with VSD closure C) Palliative care, no surgical option available

28 TGA; VSD Balloon Atrial Septostomy (August 1975) Closure of VSD and Mustard atrial switch operation (August 1976) Systemic A-V valve (tricuspid) regurgitation TV reconstruction (August 2006) Ventricular dysfunction (2016)

29 Type I Diabetes Mellitus (age 8) Congenital Glaucoma Diabetic Nephropathy Diabetic Neuropathy Tobacco Abuse

30 Quality of Life Paper vs. Reality Works FT, plays in band Complexity of Care Diabetes- Endocrine Renal Disease- Nephrologist CAD Risk- Acquired heart disease Transplant Candidate?

31 Assessment Chronic? Original Diagnosis Residual Lesion Precipitating/Alleviating Factors ECG- normal abnormal Likelihood of CAD cause for CP

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33 Assessment Chronic? Original Diagnosis Residual Lesion Precipitating/Alleviating Factors ECG- normal abnormal Associated with Presyncope or Syncope?

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36 PREVENTION SBE prophylaxis guidelines (AHA 2007) CHD patients are at higher risk PROMPT DIAGNOSIS Know the signs and symptoms Acute or Subacute BCBD- Blood Cultures Before Drugs

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38 A) Send the patient to ER immediately B) Start empiric antibiotics C) Order a CBCD and Electrolytes D) Have blood cultures drawn E) Ask the patient to call their cardiologist

39 Pre-pregnancy counseling Hereditary risk Normal changes of pregnancy Maternal/Fetal risk Maternal Heart Health Clinic

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42 Residual R > L shunt Unrepaired Defect Eisenmenger Syndrome L>R becomes R>L, persistent fixed ^ PHTN Saturations- their normal Relative Anemia IV air filter required for ALL IV infusion

43 Contraception Is oral contraceptive safe? Occupation Long term Acceptance- Military Physical Activity/Sports Life Insurance/Travel Insurance Psychological Health Anxiety, depression Disability/AISH

44 Shaun White X-Games Gold Medal Record Holder Tetralogy of Fallot

45 BP Both upper limbs, use highest arm Saturations Cyanotic->their normal Cyanosis Relative anemia +/- Oxygen IV therapy Micron air filter if a shunt lesion

46 Fever BCBD Single Ventricles (Fontan) Volume Sensitive Notify Questions Call NAACH Clinic Ph Fx ACHD on Call Mon-Fri 08:00-17:00

47 Adult Congenital Heart Disease- A Practical Guide (Gatzoulis et al.)

48 Canadian Adult Congenital Heart Network Find a centre Patient support and information 15 minute learning modules US site, lists all US and Canadian ACHD centers Fellowship Accreditation

49 CACH Network Member Centres St. John's Vancouver Edmonton Calgary Saskatoon Winnipeg Quebec City Halifax Ottawa Montreal (2) Toronto Kingston London Hamilton

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51 Adult Congential Surgical Volumes Adult Congential Surgical Volumes

52 SVR TM 1 TM 2 TM 3 From Heart Disease in Pregnancy, C. Oakley,

53 Native conditions: - Isolated congenital aortic valve disease - Isolated congenital mitral valve disease (except parachute valve, cleft leaflet) - Isolated patent foramen ovale or small atrial septal defect - Isolated small ventricular septal defect (no associated lesions) - Mild pulmonic stenosis Repaired conditions: - Previously ligated or occluded ductus arteriosus - Repaired secundum or sinus venosus atrial septal defect without residua - Repaired ventricular septal defect without residua

54 - Aorto-left ventricular fistulae - Anomalous pulmonary venous drainage (partial or total) - Atrioventricular canal defects (partial or complete) - Coarctation of the aorta - Ebstein's Anomaly - Infundibular right ventricular outflow obstruction of significance - Ostium primum atrial septal defect - Patent ductus arteriosus (not closed) - Pulmonary valve regurgitation (moderate to severe) - Pulmonic valve stenosis (moderate to severe) - Sinus of Valsalva fistula/aneurysm - Sinus venosus atrial septal defect - Subvalvar or supravalvar aortic stenosis (except HOCM = hypertrophic obstructive cardiomyopathy - Tetralogy of Fallot - Ventricular septal defect with --- Absent valve or valves --- Aortic regurgitation --- Coarctation of the aorta --- Mitral Disease --- Right ventricular outflow tract obstruction --- Straddling tricuspid/mitral valve --- Subaortic stenosis

55 - Conduits, valved or nonvalved - Cyanotic congenital heart disease (all forms) - Double-outlet ventricle - Eisenmenger syndrome - Fontan procedure - Mitral Atresia - Single Ventricle (also called double inlet or outlet, common or primitive) - Pulmonary Atresia (all forms) - Pulmonary vascular obstructive diseases - Transposition of the Great Arteries - D Type - Transposition of the Great Arteries - L Type (Congenitally Corrected TGA) - Tricuspid Atresia Truncus arteriosus/hemitruncus - Other abnormalities of atrioventricular or ventriculoarterial connection not included above (i.e. criss-cross heart, isomerism, heterotaxy syndromes)