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Congenital Heart Disease

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Title: Congenital Heart Disease


1
Congenital Heart Disease
  • J.B. Handler, M.D.
  • Physician Assistant Program
  • University of New England

2
Abbreviations
  • ASD- atrial septal defect
  • VSD- ventricular septal defect
  • PDA- patent ductus arteriosus
  • PS- pulmonic stenosis
  • HF- heart failure aka CHF- congestive heart
    failure
  • SAP- systolic arterial pressure
  • RV- right ventricle
  • LV- left ventricle
  • PA- pulmonary artery
  • PAH- pulmonary arterial hypertension
  • LVSP- left ventricular systolic pressure
  • PFO- patent foramen ovale
  • LAE- left atrial enlargement
  • PVOD- pulmonary vascular obstructive/occlusive
    disease
  • PuVR- pulmonary vascular resistance
  • SVR- systemic vascular resistance (same as TPR,
    PVR)
  • CO- cardiac output
  • PAPVC- partial anomolous pulmonary venous
    connection
  • Sx- symptoms
  • Qp/Qs- pulmonary blood flow/systemic blood flow
  • LLSB- lower left sternal border
  • RVSP- right ventricular systolic pressure
  • PFO- patent foramen ovale
  • MCA- middle cerebral artery

3
Incidence and Etiology
  • 8 per thousand births- one third with critical
    disease.
  • Majority (gt80 survive to adulthood)

4
Incidence -Specific Defects
  • VSD - 28
  • Pulmonic Stenosis - 9.5
  • Tetralogy of Fallot- 8-10 (complex ConHD)
  • PDA - 8.7
  • ASD - 6.7
  • Coarctation of the Aorta - 4.4
  • Aortic Stenosis - 4.4
  • Congenital Coronary Anomalies - 1.2

5
Complications-CHD
  • HF (aka CHF) May be early in life depending on
    the defect and its severity. HF as adult with
    milder forms of congenital heart disease if not
    corrected.
  • Cyanosis Common with defects that result in R?L
    shunting of blood. Also occurs in presence of
    severe hypoxemia from other causes (e.g severe
    HF).
  • Clubbing of fingers occurs when cyanosis is long
    standing.
  • Hypoxemia from HF responds to O2 hypoxemia from
    R?L shunting does not.

6
Clubbing of Fingers
Images.google.com
7
Complications of CHD
  • Polycythemia Hct gt60 common with R?L shunting
    and associated chronic hypoxemia.
  • Paradoxical Emboli Venous thrombus ends up in
    systemic circulation. See below.
  • Stroke
  • Polycythemia from R-L shunting can lead to direct
    intracranial thrombosis.
  • Paradoxical embolus as noted.
  • Retardation of growth

8
Additional Complications-CHD
  • Pulmonary Arterial Hypertension (PAH)-Direct
    transmission of SAP to RV or PA via a large
    communication (e.g. VSD, PDA).
  • Pulmonary Vascular Obstructive Disease
    (PVOD)-Destruction of pulmonary vascular
    (arteriolar) bed in presence of continuous
    pressure overload (much less common with volume
    overload alone) results in marked increase in
    PuVR and further elevation of PAP.

9
Ventricular Septal Defect
  • An opening in that part of the ventricular septum
    that separates the two ventricles.
  • 80 involve the thin membranous septum.
  • 20 involve the muscular septum.
  • Isolated vs complex lesions
  • Assoc. conditions Coarct. of the Aorta, ASD,
    PDA, sub-aortic stenosis

10
VSD
Images.google.com
11
Abnormal Physiology (VSD)
  • Small restrictive VSD large resistance to flow
    through small hole normal RVP and PAP small L
    to R shunt well tolerated.
  • Mod/large VSDs allow varying transmission of LVP
    into the RV?PA. PAH common and PVOD develops
    over time. Large defects result in LV dilation
    and failure.

12
Clinical Manifestations (VSD)
  • History Varies depending on size of defect.
  • Small VSD- no symptoms murmur appears within 36
    hrs of birth intensity may change with age.
  • Larger defects- HF early in life surgical repair
    indicated.
  • Small defect- Possible systolic thrill at LLSB
    nl S2 harsh holosystolic murmur along LSB.
  • Large defect signs and symptoms of heart
    failure.

13
Investigative Findings (VSD)
  • CxR variable findings reflect severity of
    shunting. When severe, cardiomegaly, enlarged
    PA, HF.
  • ECG variable findings depending on severity and
    duration.
  • Echo-Doppler - diagnostic identifies the size
    and location of the defect and presence of
    shunting RV and PA pressures can be estimated .

14
Natural History (VSD)
  • Majority of VSDs are small 24 close
    spontaneously by 18 mos, 50 by 4 yrs, more by 10
    yrs. Larger defects may become smaller but do
    not close.
  • HF occurs in 80 of infants with large VSD. Risk
    of PVOD is high in moderate to large defects.
  • Risk of endocarditis if defect remains open
    (regardless of size). But. endocarditis
    prophylaxis with antibiotics no longer indicated
    risk of antibiotics outweighs benefits.

15
Management (VSD)
  • Medical with small VSD need regular follow up
    periodic echo-doppler study will confirm closure.
  • Moderate and large VSD treat HF as in adults
    surgical repair once HF improved.
  • Timing of surgery dependent on severity of shunt,
    LV function and PAP closure in early childhood
    years when PAP remains elevated.
  • With most VSDs primary closure or a patch can be
    placed surgically with lt1 mortality normal life
    expectancy if done early in life, before
    developing PVOD. Catheter based techniques for
    closure are evolving?promising.

16
VSD Long Term Complications
  • If PAH continues over time ? progressive,
    irreversible PVOD develops and surgery carries
    high mortality, with little if any benefit.
  • In presence of significant PVOD PuVR and PAP
    rise dramatically. This can lead to shunt
    reversal?R to L shunting? hypoxemia and Rt sided
    heart failure (Eisenmengers physiology/complex).

17
Case 1
  • A 31 y/o professional football player returns
    from Hawaii (long plane flight) following the Pro
    Bowl (2004). On returning home he has sudden
    onset of numbness and weakness in his left arm
    (LA) and leg (LL) along with a small visual field
    defect.
  • PE Healthy man, anxious. Neuro ?sensation and
    strength in LAgtLL ?reflexes on left side. Legs
    bruising of rt calf and thigh.
  • MRI rt hemispheric stroke (MCA territory)
  • Venous ultrasound Inconclusive ? thrombus in
    RLE
  • What is going on here?

18
Atrial Septal Defect
  • A through and through communication between the
    atria at the septal level.
  • Pathology Large enough defect to allow free
    communication between the atria.
  • Most common form (previously undetected) of CHD
    in adults female to male ratio is 21.
  • Atrial septum formed by fusion of 2 overlapping
    planes of tissue during fetal development. Most
    ASDs occur in mid septum due to lack of tissue
    for overlap.

Lack of fusion occurs in up to 25 of adults
leaving a patent foramen ovale, a potential
space/opening between the two atria.
19
Patent Foramen Ovale
20
ASD
Images.google.com
21
Anatomic Types of ASD
  • Ostium secundum -defect in mid septum at the
    fossa ovalis (80) from incomplete development.
  • Associated partial anomalous pulmonary venous
    connection is not uncommon MVP present in some.
  • Ostium primum- defect in lower atrial septum
    usually associated with additional defects.
  • Sinus venosus defect (6) - defect high in the
    atrial septum.

22
Conditions Common to all ASDs
  • RA, RV and PA enlarge - volume overload
  • Pulmonary HTN usually occurs late (3rd or 4th
    decade) if lesion goes undetected up to that time
    in life result of chronic volume overload x
    years.
  • PVOD uncommon
  • Why left to right shunting?

23
Abnormal Physiology (ASD)
  • L to R shunting at atrial level due to
  • Rt atrium more distensible than left
  • RV more compliant than LV
  • PuVR ltSVR
  • Hemodynamic burden RV volume overload and
    increased pulmonary blood flow well tolerated
    for many years.

24
Clinical Presentation (ASD)
  • Majority of children are asymptomatic
  • Symptoms when present include fatigue, dyspnea,
    decreased stamina and usually begin in early
    20s.
  • Most adults become increasingly symptomatic by
    3rd or 4th decade fatigue, dyspnea and atrial
    arrhythmias (Afib).
  • Paradoxical emboli can result in stroke Tedi
    Bruschi, NE Patriots. Venous embolus?RA through
    PFO ?LA?LV?Rt carotid?MCA.

Patent foramen ovale incomplete fusion of
atrial septum (tiny defect) allows clot to pass
from RA to LA
25
Physical Exam (ASD)
  • Hyperdynamic RV (lift) RV volume increase leads
    to ?contraction via Starling mechanism.
  • S1 accentuated at LLSB
  • S2 widely split through inspiration/expiration
    RV ejection is delayed from volume overload.
  • Grade II-III midsystolic creshendo-decreshendo
    mumur, at upper LSB reflects increased blood flow
    across pulmonic valve. Present during childhood.

26
Investigative Findings
  • ECG rsR pattern in Rt precordial leads with
    mildly widened QRS (incomplete RBBB) arrhythmias
    common in adults-Afib, Aflutter.
  • Echo-Doppler RV volume overload enlarged RV,
    RA 2D echo and doppler identify the defect and
    semi quantitate the shunt.
  • Cardiac cath Measurement of RV/PA pressures
    quantification of shunting identification of
    anomalous pulmonary veins if present. Closure of
    ASD often performed percutaneously using
    catheters/devices.

27
Natural History and Prognosis
  • Defect often missed in childhood?listen for
    murmurs.
  • A systolic ejection murmur is the usual reason
    for further evaluation (echocardiogram or
    consult).
  • Sx often begin in late teens and 20s if large
    ASD
  • PA pressures start to rise in early 20s
  • Incidence of Atrial Fibrillation and Flutter
    increases each decade.
  • Heart failure (Rt sided) and premature death
    occur in adults without surgery.

28
Management (ASD)
  • Once Sx present (includes paradoxical emboli) ?
    surgical/catheter closure.
  • If no Sx? surgical/catheter closure recommended
    if QpQs is gt 1.51, or if PAH present.
  • Transcatheter closure devices (double umbrella)
    applicable to patients with smaller defect.

QpQs- pulmonary to systemic blood flow
29
Surgical Management (ASD)
  • Direct suture closure or pericardial patch.
  • If present, partial anomalous pulmonary veins are
    re-routed to the left atrium.
  • Surgical risk is very low (lt1 mortality).
    Closure is highly recommended in pre-school or
    pre-adolescent years.

30
Pulmonic Stenosis
  • Pathology Dome shaped stenosis of the PV most
    common form. RV develops concentric hypertrophy
    and reflects degree of obstruction at the
    valvular level.
  • Can be associated with other congenital defects
    such as VSD (see Tetralogy of Fallot, below).

31
Pulmonic Stenosis
Images.google.com
32
Abnormal Physiology (PS)
  • PV area must be reduced by 60 or more to be
    hemodynamically significant.
  • Peak systolic gradient gt 40mmHg - moderate PS
  • Peak systolic gradient gt 75mmHg -severe PS
  • Major hemodynamic burden is Rt ventricular
    pressure overload. Expected ECG finding?
  • RV failure occurs with severe obstruction,
    resulting in decreased CO and related Sx and
    signs.

33
Clinical Manifestations (PS)
  • Occurs 10 all congenital lesions most infants
    and children asymptomatic unless obstruction is
    severe- DOE, fatigue.
  • Physical exam
  • Systolic thrill-suprasternal notch prominent RV
    impulse upper LSB.
  • Early systolic click upper LSB.
  • Murmur is loud (Gr 3-4), harsh,
    crescendo-decrescendo at upper LSB radiating
    towards clavical and louder with inspiration.
    Duration of murmur correlates with severity of
    obstruction.

34
Investigative Findings (PS)
  • CxR-usually normal
  • ECG- Mild - normal severe- RVH
  • Echo/Doppler - Identifies obstruction estimates
    severity of PS.
  • Cath- usually not needed to make diagnosis but
    performed for treatment purposes
  • Baloon valvuloplasty opens stenotic valve.

35
Natural History/Prognosis
  • Mild to moderate stenosis - well tolerated
    frequent follow up and echo/doppler necessary as
    progressive PS may develop over time.
  • Severe stenosis - poor prognosis without
    intervention RV failure develops with premature
    death in adults.

36
Management of PS
  • Infants with severe PS - valvuloplasty.
  • In children and adults timing of valvuloplasty
    dependent on gradientNo intervention for
    gradient lt 25mm.Valvuloplasty always indicated
    for gradient gt75mm.
  • Ballon valvuloplasty has replaced surgery as a
    first approach.

37
PS Balloon Valvuloplasty
38
Patent Ductus Arteriosus
Images.google.com
39
Patent Ductus Arteriosus (PDA)
  • Persistent patency of the vessel that normally
    connects the pulmonary arterial system and the
    aorta in the fetus.
  • PDA normally closes within 2-3 days after birth.
    It runs from the origin of the LPA to the lower
    aortic arch just beyond the left subclavian
    artery.
  • Ductus often remains open in pre-term deliveries.
  • Important to differentiate from post-term PDA

40
Abnormal Physiology (PDA)
  • Small ductus- high resistance to flow well
    tolerated small left to right shunt.
  • Moderate ductus- elevated PAP, significant
    shunting.
  • Large ductus- Ao and PA in free communication
    equal pressures with marked left to right
    shunting, pulmonary congestion, LV dysfunction
    and failure, and development of PVOD.

41
Clinical Manifestations (PDA)
  • History- maternal exposure to rubella premature
    deliveries.
  • Symptoms variable with large shunt, HF develops
    in first weeks of life.
  • PE - Systolic thrill over PA in suprasternal
    notch and LSB apical and RV impulse
    increased.Murmur is a continuous (through
    systole and diastole) machinery murmur Gr IV or
    louder at LSB (3rd and 4th ICS) and below
    clavicle-peaks near S2.

42
Additional Findings
  • Dependent on size of ductus/degree of shunting
  • CxR- increased LA, LV, pulmonary vascularity
    (shunt vascularity).
  • ECG LAE/LAA and LVH.
  • Echo-doppler - LAE, LVE and LVH shunt may be
    visualized by 2D echo/doppler.
  • Cardiac MRI and CT also useful in identifying PDA

LAA- left atrial abnormality
43
Natural History/Management
  • Complications include endocarditis, HF, PAH,
    PVOD, and sudden death.
  • Ultimate goal - closure of the ductus.In
    premature infants treatment with Indomethacin is
    1st line therapy?constriction of ductus.
  • Surgical or catheter closure are safe and
    effective when ductus remains open.

44
Coarctation of the Aorta
  • 8-9 of all infants presenting with CHD.
  • Discreet narrowing of the distal segment of the
    aortic arch, just distal to the origin of the
    subclavian artery.
  • Coarctation causes obstruction to outflow to the
    lower half of the body. Principle cardiovascular
    abnormalities
  • LVH due to pressure overload
  • Arterial hypertension

45
Coarctation of the Aorta
Allreferhealth.com
Images.google.com
46
Abnormal Physiology (Coarct)
  • Systolic and diastolic pressures above the
    coarctation are elevated below reduced.
  • A secondary form of HTN
  • Prominent collateral circulation to the lower
    body develops via the internal mammary and
    subcostal arteries (rib notching on CxR).

47
Clinical Manifestations
  • 50 present as infants with HF. Concomitant VSD
    often present.
  • In older children Sx include fatigue, dyspnea and
    claudication in legs while running.
  • Hypertension in childhood is a red flag for
    secondary hypertension.
  • Consider also Renal artery stenosis

48
Clinical Manifestations
  • PE In older children and adults- differential
    blood pressure between arms and legs a measured
    difference gt 10mmHg systolic is diagnostic.
  • Majority of patients will develop marked HTN to
    upper part of body -high renin HTN due to
    decreased perfusion of kidneys.
  • Upper body well developed legs very thin.

49
Additional Findings
  • CxR LV prominent HF-infants notching of
    inferior margins of ribs in adolesence.
  • ECG LVH.
  • Echo-doppler Suprasternal imaging may show the
    coarct LVH, LV dysfunction.
  • Cardiac MRI and CT also useful for coarctation
  • Cardiac cath Pressure differential across the
    coarct with angiographic visualization and any
    associated lesions defined.

50
Natural Hx and Progression
  • 50 of infants will present with HF and respond
    well to medical treatment.
  • Hypertension develops with age and often persists
    if surgical correction is performed after age 6.
    Significant coarcts, if uncorrected, result in
    premature death, often by age 50.
  • Surgery Direct resection/repair if possible
    adequate collaterals crucial for safe repair- if
    absent, lower body paralysis can occur due to
    interrupted blood flow to spinal cord during
    surgery. Stenting via catheters is being
    investigated/used as an option.

51
Tetralogy of Fallot
  • 8-10 of all congenital defects- complex lesion
  • Biventricular origin of the Aorta
  • Large VSD
  • Obstruction to pulmonary blood flow
  • RVH

52
Tetralogy of Fallot
Movie Something The Lord Made details the
first heart surgery done in the US for TOF.
Images.google.com
53
Sudden Cardiac Death in the Young
  • Overall incidence low 600 cases/yr.
  • Structural cardiac abnormalities in 90.
  • 40 occur in children with surgically treated
    CHD.
  • Majority of SD in the young presents as the 1st
    manifestation of cardiac disease in otherwise
    healthy appearing individuals.

54
Etiologies - SCD in the Young
  • Myocarditis (unrecognized)
  • Hypertrophic Cardiomyopathy
  • Congenital Coronary Anomalies
  • Coronary Artery Disease (CAD)
  • Conduction system abnormalities- Brugada
    syndrome, others.
  • Mitral Valve Prolapse- very rare
  • Aortic dissection or rupture often associated
    with connective tissue abnormalities (Marfans
    syndrome), Marfanoid body habitus.

55
SCD in Competitive Athletes
  • Extremely rare 25/yr. in USA
  • Hypertrophic Cardiomyopathy most common cause in
    athletes lt35 yrs.
  • Congenital coronary anomalies
  • Aortic rupture associated with Marfans and other
    connective tissue diseases.
  • CHD present in lt 10

56
Screening and Prevention
  • Because myocarditis is often silent, and
    associated with common viruses, strenuous
    physical exertion and athletic competition should
    be avoided in individuals with symptomatic viral
    symptoms or who are febrile.
  • Detailed histories must be obtained during sports
    physicals- family history of sudden death
    history of chest pain, dizzyness, syncope or
    dyspnea presence of any cardiac risk factors.

57
  • A thorough exam should include observation for
    connective tissue abnormalities, body habitus,
    pectus deformity, etc.
  • Cardiac exam should include thorough evaluation
    of murmurs including provocative maneuvers.
  • ECGs and Echocardiography/Doppler should be
    obtained when structural cardiac pathology is
    suspected. Could make a difference between life
    and death.
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