Adult Congenital Heart Disease Program

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Adult Congenital Heart Disease Program

• Division of Cardiology
• Dr. Simon Jackson

Queen Elizabeth II Health Sciences Center
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An overview of Adult Congenital Heart Disease
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And.a practical approach to murmurs
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Objectives

• To gain an awareness of the resources available
  for adults with congenital heart disease
• To understand specific problems unique to the
  ACHD population
• Medical complications of cyanotic heart disease
• Psycho-social implications (impact of living with
  chronic disease)
• To understand the principle of SBE prophylaxis,
  and which ACHD patients DO NOT require
• To gain a practical approach to the assessment of
  murmurs

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Why talk about ACHD?

• To increase your awareness of the complexity of
  care for these young patients
• To review some of the complex psycho-social
  problems related to chronic illness and impact on
  a normal life
• To learn the specific complications of long term
  cyanotic heart disease

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Adult Congenital Population

• North America -800,000 adults
• with a congenital cardiac anomaly
• Approximately 1400 new patients
• with significant congenital heart defects will
  become adults in Canada each year

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Adult Congenital Population Subtypes

• Those who have received curative surgery
• Those who have had palliative procedures to
  prolong life and improve its quality
• Those for whom no treatment is available and care
  is purely supportive
• Those in whom a diagnosis and plan of action have
  not yet been established

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Goals Adult Congenital Heart Program

• Provide comprehensive multi-disciplinary care to
  the Adult Congenital patient population of the
  Maritime provinces
• Provide training opportunities in the area of
  Adult Congenital Heart Disease
• Provide education and support for adult
  congenital heart disease patients, their families
  and their primary care physicians
• Contribute to clinical research in ACHD

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Adult Congenital Heart Program QE II HSC

• Three adult Cardiologists
• One rotating pediatric Cardiologist
• One Nurse - Coordinator

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Consultative Support

• Social work
• Psychology
• Genetic Assessment
• Obstetrics/Gynecology Electrophysiology
• CV Surgery
• Anesthesia

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QE II HSC ACHD
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QEII HSC ACHD Program
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Non-Cardiac Issues of ACHD Population

• Young population with associated co-morbidities
• Chronic illness
• Pregnancy and contraception
• Vocational counseling
• Un-insurability
• Uncertainty of the future

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Patient Education

• Despite its lifelong illness most adolescents and
  young adults with CHD have inadequate knowledge
  about their cardiac conditions.
• Patients need appropriate information and
  education to enable independent decision-making
  about choices in care.

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Co-Morbidities

• Syndromes
• Post-pump complications
• Stroke
• Cognitive decline
• Respiratory disorders
• Neurologic sequlae

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Psychological Issues

• Adults with CHD face special challenges
• Employment
• Inter-personal relationships
• Assuming responsibility for their own health care
• Family planning

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Psychological Issues

• Long-term disturbances range from frank
  psychiatric disorders to subclinical distress and
  abnormalities in neurocognitive functioning
  secondary to
• chronic illness disability
• parental overprotection
• ischemia/hypoxia from operations

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Uncertainty and Insurability

• Many patients are un-insurable
• Limits opportunities with work, business, owning
  a home
• Reinforces concerns about mortality

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Pregnancy

• Health of the mother
• Health of the fetus
• Risk of congenital disease in the offspring

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Pregnancy

• Pregnant women with CHD should be managed by the
  patient's obstetrician and ACHD cardiologist
  concurrently.
• Post partum, the patients with CHD may need
  ICU/monitoring facilities even for relatively
  minor procedures or uncomplicated deliveries

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QE II Health Sciences Pregnancies 1995
- 2002

• 1995 - 17(MRC Pregnancy Study)
  
• 1996 - 14
• 1997 - 4
• 1998 - 17

• 1999 - 17
• 2000 - 21
• 2001 - 22
• 2002 - 17

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1988-2001 SummaryQE II HSC

• 81 pregnancies
• 57 Live births (70.4)
• 13 lost to followup, 4 TA, 6 SA, 1 stillbirth
• 7 (12 of live births) congenital defects
• gt20 (35 of live births) obstetrical events
• gt22 (39 of live births) cardiac events

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Pregnancy Contraindications

• Pulmonary hypertension
• Severe LV (systemic) dysfunction
• Marfan syndrome with dilated aortic root (gt 4 cm)
  
• Cyanotic heart disease

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Contraception

• OCP may be contra-indicated
• IUDs increased risk of bacterial endocarditis
• Permanent sterilization risk of the anesthetic

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Medical Issues

• Endocarditis prevention
• Other surgeries
• Specific conditions
• Eisenmengers syndrome

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Endocarditis

• Endovascular infection on some structure within
  the heart, usually a heart valve
• High morbidity and significant mortality

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Dental Care

• Regular dental care often in a hospital setting
  is mandatory for adult patients with CHD to
  decrease the likelihood of infective endocarditis
• Endocarditis prophylaxis is recommended for all
  dental work except for 'fillings above the gum
  line'

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Endocarditis Prophylaxis

• Education and good dental hygeine
• Cardiac conditions are stratified into
• high
• moderate
• negligible risk categories

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1997 ACC/AHA Recommendations

• High Risk
• those who have prosthetic heart valves
• a previous history of endocarditis
• complex cyanotic congenital heart disease, or
  surgically constructed systemic pulmonary shunts
  or conduits

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1997 ACC/AHARecommendations

• For oral or dental procedures the initial
  amoxicillin dose is reduced to 2 gm 1 hour pre
  procedure
• Clindamycin 600mg is offered to penecillin
  allergic patients

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1997 ACC/AHA Recommendations

• For gastrointestinal or genitourinary procedures,
  the prophylactic regimens have been simplified
• High Risk IV ampicillin 2gm plus gentamycin 1.5
  mg/kg then po amoxil or iv ampicillin
• Moderate risk 2 gm po amoxil 1 hour pre

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Non-Cardiac Surgery

• Performance of any surgical procedures in some
  adult patients with CHD carries a greater risk
  than in the normal population.
• Evaluation in a referral centre prior to surgery
  is recommended
• Specific concerns regarding SBE prophylaxis, air
  filters, risk DVT/PE and need for understaning of
  physiology of lesion

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Physiologic Consequences of Cyanosis

• Secondary erythrocytosis is normal (Hb 200-220)
• Stimulus is low oxygen saturation leading to
  increase erythropoetin leading to increased red
  cell mass
• Compensation to increase oxygen carried in the
  blood

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Non-Cardiac Sequelae of Erythrocytosis and
Cyanosis

• Abnormal hemostasis,
• Easy bruising, epistaxis, gingival bleeding
• bleeding or traumatic bleeding (including
  peri-operative bleeding)
• Spontaneous intravascular thrombosis

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Non-Cardiac Sequelae of Erythrocytosis and
Cyanosis

• Hyperuricemia with gout, urate nephropathy
• Glomerular sclerosis, secondary to cyanosis, is
  usually manifest initially as proteinuria
• Hypertrophic osteoarthropathy
• Gallstones and cholecystitis

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Hyperviscosity

• Viscosity of blood increases with disorders of
  increased cellular mass
• thick blood clogs up capillaries leading to
  symptoms
• Headache, lethargy, confusion, vision change
  progressing to stroke
• Removal of RBCs is an effective treatment

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Care of the Cyanotic Patient

• Iron deficiency (anemia) is a crisis!
• Reduced oxygen carrying capacity and reduced
  deformability of the red cells leads to increased
  symptoms and possibly an increased risk of
  stroke.

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Plebotomy

• Rarely required for the secondary increase in RBC
  mass in cyanotic heart disease
• Should only be done with adequte replacement of
  volume
• Can lead to iron deficiency anemia

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ACHD

• Know SBE prophylaxis
• The non-medical issues of ACHD
• Physiologic effects of cyanosis

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Question 1

• Aortic insufficiency produces a
• Systolic ejection murmur
• Diastolic ejection murmur
• Diastolic rumble
• Diastolic decresendo murmur

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Question 2

• Tricuspid insufficiency produces a
• Systolic ejection murmur
• Systolic rumble murmur
• Diastolic rumble
• Systolic regurgitant murmur

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Question 3

• Pulmonary stenosis produces a
• Systolic ejection murmur
• Diastolic decrescendo murmur
• Diastolic rumble
• Systolic regurgitant murmur

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Question 4

• Mitral stenosis produces a
• Diastolic rumble
• Systolic rumble
• Systolic regurgitant murmur
• Diastolic decrescendo murmur

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Practical Approach to Murmurs

• Know what valves are open or closed in systole
  and diastole
• Decide if the murmur is systolic or diastolic
• Decide if the murmur character is
• Ejection
• Regurgitant
• Decrescendo
• Rumble
• Decide where is the murmur heard best
• Put it all together

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Know what valves are open or closed in
systole?

• In systole (ventricles ejecting blood)
• AV and PV are open and
• the MV and TV are closed
• In diastole (ventricles being filled)
• MV and TV are open while
• the AV and PV are closed

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Facts you need to know

• Stenotic (narrowed) valves make noise when they
  should be normally open (not opened enough)
• Regurgitant (leaky, insufficient) valves make
  noise when they should be closed (valves are
  leaking blood the wrong way)

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Is the murmur systolic?

• Time the murmur with the pulse
• Murmurs heard when the pulse can be felt are
  systolic

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Facts Ejection Murmurs

• Ejection murmurs are always systolic (blood is
  ejected in systole)
• Ejection murmurs peak and (almost) always fall in
  intensity
• This means they begin after S1 and end (almost)
  always before S2
• Ejection murmurs arise from the aortic valve or
  pulmonary valve (or less commonly from the LV or
  RV outflow tracts)

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Regurgitant Murmurs

• Regurgitant murmurs means blood is regurgitating
  (going the wrong way)
• Regurgitant murmurs are high pitched (the flow is
  from an area of high pressure to an area of much
  lower pressure)
• Systolic regurgitant murmurs are (almost) always
  holosystolic ( pansystolic) and begin with S1
  and end with S2
• Examples are
• mitral insuffiency
• tricuspid insufficiency.
• A VSD is another cause.

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Facts Diastolic Murmurs

• Diastolic murmurs can be
• Decrescendo high pitch, intensity decreasing
  during diastole, due to insufficiency of AV or PV
  
• Rumbles low pitched, localized, heard with
  bell, related to low pressure flow across a
  narrowed valve, (mitral stenosis, tricuspid
  stenosis)

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Final Facts

• Know the areas where the murmurs are heard best
• Aortic stenosis Aortic area
• Pulmonary stenosis Pulmonary area
• Tricuspid stenosis Tricuspid area
• Mitral stenosis Mitral area (apex)

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Final Facts

• Know the areas where the murmurs are heard
• Aortic insufficiency Left sternal edge
• Pulmonary insufficiency Pulmonary area
• Tricuspid insufficiency Tricuspid area
• Mitral insufficiency Mitral area, axilla,
  rarely to aorta

Not where expected
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Case 1 Putting it together

• You hear a systolic ejection murmur loudest in
  the upper right sternal border
• Ejection murmurs come when a valve is not opened
  properly (stenotic)
• This is the aortic area
• This is the murmur of aortic stenosis

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Putting it together Case 2

• You hear a systolic murmur loudest in the apex
  which is regurgitant
• What is regurgitant? What does it mean?
• What valves should be closed in systole?
• What area is this?
• This is the murmur of mitral insufficiency

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Putting it together Case 3

• You hear a diastolic murmur loudest at the apex
  which is low pitched, and localized.
• What is another name for a low pitched murmur?
  What does it imply?
• What valves should be open in diastole?
• What area is this?
• This is the murmur of mitral stenosis

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Putting it together Case 4

• You hear a diastolic murmur loudest at the left
  sternal border which is high pitched and
  decreases in intensity during diastole.
• What is another name this type of murmur? What
  does it imply?
• What valves should be closed in diastole?
• What area is this?
• This is the murmur of aortic insufficiency

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Describe the murmurs for the following lesions

• Pulmonary stenosis
• Pulmonary insufficiency
• Tricuspid stenosis
• Tricuspid insufficiency

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How else do we sort out murmurs?

• Associated findings (intensity of heart sounds,
  associated sounds, palpation of heart)
• Radiation of murmur
• Effects of respiration, dynamic maneuvers
• Pattern recognition
• Knowledge of the disease process effecting the
  heart