CHAIRPERSON: DR B.L BHARDWAJ

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

Genetic : as in downs , noonans , holt-oram, williams etc

Environmental : maternal rubella , drugs like thalidomide , isotretinoin

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

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

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

• 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

• 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.

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

First described by roger in 1879 Term” maladie de roger” : refers to

small asymptomatic VSD

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

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

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

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

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

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

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

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

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

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

4 features• Malalignment VSD• Overriding Aorta• Pulmonic Stenosis• RVH

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

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

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

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

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

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

ONLY SURGICAL

•PALLIATIVE SURGERY

•DEFINITIVE SURGERY

TREATMENT OPTIONSTREATMENT OPTIONS

Atrialization of RV, sail-like TV, TR

50% ASD/PFO 50% ECG evidence of

WPW Age at presentation

varies from childhoodadulthood and depends on factors such as severity of TR, Pulm Vascular resistance in newborn, and associated abnormalities such as ASD

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

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

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

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

Low voltage Peaked p waves in lead 2 and v1:

reflect rt atrial enlargement Prolonged PR interval RBBB common in adults

Pediatric• murmur

Adult (unrepaired with ASD)• atrial arrhythmias• murmur• cyanosis

RL shunt • exercise intolerance

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

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

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

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

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)