This document discusses congenital heart disease in adults. It notes that 1 million adults in the US have congenital heart disease, with 20,000 more reaching adulthood each year due to increased survival of children with CHD. Common adult presentations of CHD include effort dyspnea, atrial fibrillation, and right heart failure. The document reviews the pathophysiology, clinical features, diagnostic evaluation, and management of various CHD lesions that may present in adulthood, such as atrial septal defects, ventricular septal defects, patent ductus arteriosus, tetralogy of Fallot, Ebstein's anomaly, and coarctation of the aorta. Surgical and percutaneous interventions are discussed

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)