congenital heart disease

1. CHAIRPERSON: DR B.L
BHARDWAJ

2.  US: 1,000,000 adults with congenital heart dz
 20,000 more patients reach adolescents yearly
 Incidence increasing due to increased survival
of children with CHD

3.  Genetic : as in downs , noonans , holt-
oram, williams etc
 Environmental : maternal rubella , drugs
like thalidomide , isotretinoin

4.  Hyperviscosity syndrome : headache ,
dizziness , nausea , paresthesias due to
increased erythrocytosis bcoz of
cyanosis
 Bleeding tendencies
 Paradoxical embolisation , brain abscess
 Proteinuria , hyperuricemia
 Hypertrophic osteoarthropathy causing
arthralgias and bone pain

5.  Atrial Septal Defect
 Ventricular septal defect
 PDA
 TOF
 Eisenmenger
 Ebstein anomaly
 Pulm. Stenosis with intact ventr. septum
 Aortic stenosis
 COA
.

6.  4 possibilities:
 1..if ecg reveals LAD of >30 deg :
suggests ostium primum defect
 2..pt may hav a floppy mitral valve
 3..ostium secundum defect assoc with
rheumatic MR
 4..secundum ASD rarely assoc with cleft
mitral valve

7. • Effort dyspnea is seen in 30% of patients
by 3rd
decade and over 75% of patients by
the fifth decade
• SVT (atrial fibrillation or flutter) and right-
sided heart failure develop by age 40 in
10-20% of patients

8. • The chest radiograph shows large pulmonary arteries, increased
pulmonary vascularity, an enlarged RA and RV, and a small aortic
knob with all pre-tricuspid cardiac left-to-right shunts.

9.  Percutaneous Closure
• only for secundum (contra in others)
• adequate superior/inferior rim around ASD
 Surgical Closure
• Good prognosis:
 closure age < 25, PA pressure <40
 If >25 or PA>40, decreased survival due to CHF,
stroke, and afib

10.  First described by roger in 1879
 Term” maladie de roger” : refers to small
asymptomatic VSD

11.  Most common CHD in children (25%)
 Isolated VSD found in only 10% of adults
with CHD
 75-80% of small VSD’s close spontaneously
by late childhood
 10-15% of large VSD’s close spontaneously
 60% of defects close before age 3, and 90%
before age 8
 Risk factors for decreased survival for
unoperated patients include:
• Cardiomegaly on CXR, Elevated PASP (>50
mmHg), and CV symptoms

12.  Perimembranous defect (70-80%)
• Less likely to be associated with other defects
• Highest rate of spontaneous closure
 Muscular or apical defects (5-20%)
• Typically occur in isolation
• High spontaneous closure rates unless multiple defects r present
 AV-Canal type (5-8%)
• Rarely close spontaneously, commonly seen in Trisomy 21
• Usually large & associated with abnormal AV valve
 Supracristal or subaortic defects (5-7%)
• Often small but need closure due to associated AR

13.  Arterial pulse is often normal
 There may be a systolic thrill on palpation of the
precordium (maximal in lt 3rd
or 4th
ICS)
 Holosystolic, high frequency murmur (grade 4-
6/6) with small VSD and normal PAP
 Once PAP increases above the systemic
pressures the holosystolic murmur disappears
 Increase flow across pulmonary valve causes a
SEM
 A loud P2 component is heard in this setting

14.  May be normal but often shows LVH and LAE
 Presence of RAD represents elevated RVP and PAP
 Postoperative RBBB is common

15.  Cardiomegaly with LAE and LVE will be seen with large L to R shunts
 A large defect associated with a small heart and oligemic lung fields
should raise the suspicion of pulmonary vascular disease

16.  Hemodynamic severity grading of isolated VSDs in
adults:
• Small: Qp:Qs <1.4, and pulmonary to aortic systolic pressure
<0.3
• Moderate: Qp:Qs = 1.4-2.2, and systolic pressure ratio >0.3
• Large: Qp:Qs >2.2, and systolic pressure ratio >0.3
• Eisenmenger: Qp:Qs <1.5 and systolic pressure ratio >0.9

17.  Clinical severity grading:
• Small: Causes negligible hemodynamic changes. LV size normal
w/o PHTN
• Moderate: Causes LV and LA enlargment, and usually some
PHTN (reversible)
• Large: Results in pulmonary vascular obstructive disease and
Eisenmenger physiology unless there is coexistent RVOTO

18.  When repair is performed in the first two years of life,
asymptomatic adult survival with normal growth and
development can be anticipated
 When surgery is undertaken in older children, a late
postopeartive increase in LV chamber size, together with
decreased systolic function is seen
 Development of late postoperative PHTN is largely
determined by the age at surgery and preoperative PVR
 Risk of SBE persists and requires prophylaxis

19.  Indications for intervention: Geade C, Level IV
• Presence of a significant VSD (symptomatic QP/QS = 2/1, PASP
> 50 mmHg), deteriorating ventricular fx due to volume (LV) or
pressure (RV) overload
• Significant RVOTO (pk to pk gradient of > 50 mmHg, or
instantaneous gradient >70 mmHg)
• Perimembranous or doubly committed VSD with more than mild
AR
• Hx of endocarditis especially if recurrent

20.  Successful closure is associated with excellent survival if
ventricular fx is normal. Elevated PAP preop may
progress, regress, or remain the same postop
 A. fib may occur, especially if there has been
longstanding volume overload of the left heart
 Pregnancy is well tolerated in women with small or
moderate VSD and in women with repaired VSD
 Pregnancy is contraindicated in women with
Eisenmenger syndrome due to both high maternal
(>50%) and fetal (~60%) mortality

21.  4 features
• Malalignment VSD
• Overriding Aorta
• Pulmonic Stenosis
• RVH

24.  OBSTRUCTION TO FLOW OF
DEOXYGENATED BLOOD FROM
THE RIGHT VENTRICLE TO THE
PULMONARY ARTERY
 DECREASED OXYGENATION DUE
TO POOR PERFUSION OF THE
BLOOD
ALTERED PHYSIOLOGYALTERED PHYSIOLOGY

25.  Rt-lt shunting across vsd
 Degree of cyanosis reflects the severity
of RVOTO and level of SVR
 Severity of cyanosis is directly
propotional to severity of pulmonic
stenosis

26.  MORE SHUNTING ACROSS THE VSD
– MORE DESATURATION OF
SYSTEMIC BLOOD – PERIPHERAL
ACIDOSIS – FUTHER SYSTEMIC
VASODILATATION – FURTHER
DECREASE IN SVR – VICIOUS CYCLE

27.  Variable cyanosis
 Rt ventricular impulse and systolic thrill
along left sternal border
 Repaired
• RVOT obstruction
• Pulmonary or tricuspid regurgitation
• LV/RV dysfunction
• Atrial/ventricular arrhythmias
 Unrepaired
• Significant morbidity
• Consider later repair

28.  Since rt ventricle is effectively
decompressed by VSD , CCF never
occurs....exceptions to this rule are:
 1.anemia
 2.infective endocarditis
 3.syst HTN
 4. aortic or pulm.Valve regurgitation

29.  ECG: shows rt axis deviation with rt
ventricular and rt atrial hypertrophy
 With repaired TOF : complete RBBB is the
rule
 QRS width : reflects degree of rt
ventricular dilation, when >180
millisec,is a risk factor for sustained VT
 CHEST XRAY : COEUR EN SABOT

30.  ONLY SURGICAL
• PALLIATIVE SURGERY
• DEFINITIVE SURGERY
TREATMENT OPTIONSTREATMENT OPTIONS

32.  Atrialization of RV, sail-like
TV,TR
 50% ASD/PFO
 50% ECG evidence of
WPW
 Age at presentation varies
from
childhoodadulthood and
depends on factors such as
severity of TR, Pulm
Vascular resistance in
newborn, and associated
abnormalities such as ASD

35.  Commonly associated with:
• ASD or PFO (90%)
• VSD, AV canal defect
• Pulmonary stenosis/atresia (20-25%)
• Wolff-Parkinson-White
 Syndromes:
• Down, Marfan, Noonan, Cornelia de Lange

36.  Heart sounds
• First heart sound widely split with loud tricuspid
component
• Second heart sound usually is normal but may
be widely split due to RBBB
• Third and fourth heart sounds commonly present
 Murmurs
• Holosystolic murmur of tricuspid regurgitation

37.  Cyanosis
• Due to R  L shunt at atrial level
 Fatigue and dyspnea
• Secondary to RV failure and decreased LV
ejection fraction
 Palpitations and sudden cardiac death
 Incidental murmur
 Paradoxic embolism

38.  Due to right atrial enlargement and high
prevalence of accessory pathways
 30-50% have evidence of WPW
secondary to the atrialized RV tissue
 Mapping and ablation are difficult
• Atrial dilation disrupts anatomic landmarks
• Accessory pathways are often multiple

40.  Low voltage
 Peaked p waves in lead 2 and v1: reflect
rt atrial enlargement
 Prolonged PR interval
 RBBB common in adults

41.  Pediatric
• murmur
 Adult (unrepaired with ASD)
• atrial arrhythmias
• murmur
• cyanosis
 RL shunt
• exercise intolerance

42.  Narrowing in proximal descending aorta
 May be long/tubular but most commonly
discrete ridge
 Natural hx: poor prognosis if unrepaired
• Aortic Aneurysm/dissection
• CHF
• Premature CADz

43. Pathophysiology
 Narrowed aorta produces increased left
ventricular afterload and wall stress, left
ventricular hypertrophy, and congestive
heart failure.
 Systemic perfusion is dependent on the
ductal flow and collateralization in severe
coarctation

44.  Associated pathology
 1. Collateral circulation
 * Inflow : primary from branches of both subclavian arteries
 . internal mammary artery . vertebral artery
 . costocervical trunk . thyrocervical trunk
 * Outflow : into descending aorta, two pairs of intercostal
arteries
 2. Aneurysm formation of intercostal arteries
 * 3rd, & 4th rib notching * rare before 10 years of age
 3. Coronary artery dilatation and tortuosity
 * due to LVH
 4. Aortic valve
 * bicuspid (27-45%) * stenosis ( 6 - 7%)
 5. Intracranial aneurysm
 * berry type intracranial aneurysm in some patients
 6. Associated cardiac anomaly
 * 85% of neonates presenting COA

46.  Most repaired, but adult presentation may be:
• Upper limb HTN
• murmur (continuous or systolic murmur heard
in back or SEM/ejection click of bicuspid AV)
 weak/delayed LE pulses
 Interscapular systolic murmur and widespread
crescendo-decrescendo murmurs due to
collaterals
 Rib notching on CXR pathognomonic
 ECG : shows RV hypertrophy, rt axis deviation

48.  Despite surgery, patients still have significant
morbidity/mortality with average age 38
 Up to 70% of repaired patients still go on to
develop HTN, pathology not well understood
 Recurrence in 8-54% of repairs, can undergo
repeat surgery or balloon angioplasty
 Aortic Aneurysm/ruputure may occur despite
successful repair and correction of HTN (freq
around anastomosis site on patch repair – 30%
in one study)