Congenital heart diseases in adults

1. CONGENITAL HEART DISEASES IN
ADULTS
Dr anoop k r

3. ATRIAL SEPTAL DEFECT
 One third of adult congenital heart disease
 Types:
 1. Ostium secondum at the region of fossa ovalis
(75%)
 2. Ostium primum (15%)
 3. Sinus venosus (10%)

5.  Magnitude of shunt depends on the size of ASD,
ventricular diastolic properties and relative
impedence of pulmonary and systemic circulations
 Increased pulmonary blood flow and dilatation of the
atria, right ventricle, and pulmonary arteries
 Develops pulmonary arterial hypertension
 Reversal of the shunt

6.  Clinical features:
 fatigue or dyspnea on exertion.
 supraventricular arrhythmias, right heart failure,
paradoxical embolism, or recurrent pulmonary infections
may prompt the patient to seek medical attention
 Prominent right ventricular impulse
 Palpable pulmonary artery pulsation
 Systolic thrill
 S1 normal or split
 S2 Widely split and fixed
 A systolic ejection murmur, audible in the second left
intercostal space, peaks in mid-systole, ends before the
second heart sound (increased flow across pulmonary
valve)

7.  Mid diastolic rumbling murmur at 4th left intercostal
space ( flow across tricuspid valve)
 As pulmonary hypertension develops..
 Pulmonary and tricuspid flow murmurs decreases
 P2 loud
 Systolic ejection sound at pulmonary area
 Diastolic murmur of pulmonary regurgitation
 ….Cyanosis .. Clubbing– Reversal of shunt

8.  ECG:
 Right axis deviation, rSr pattern right precordial
leads
 Atrial ectopics or first degree heart block- Sinus
venosus ASD
 Ostium primum: Left axis deviation, incomplete
RBBB
 Atrial arrhythmias, including atrial fibrillation and
supraventricular tachycardia, may appear

9.  On chest radiography,
 Prominent RA, RV
 Pulmonary arteries and a peripheral pulmonary
vascular pattern of “shunt vascularity” (in which the
small pulmonary arteries are especially well
visualized in the periphery of both lungs).

10.  ECHO:
 Dilated pulmonary artery, RA,RV with paradoxical
ventricular motion
 Better visualization with color flow imaging, contrast
echo
 Transesophagial echo: sinus venosus defects
 Catheterization :to determine the magnitude and
direction of shunting, as well as whether pulmonary
hypertension is present and, if so, its severity

11. treatment
 ASD with significant left to right shunting ie
pulmonary to systemic flow ratio > 1.5:1
 supportive care
 Treatment of arrythmia and infections
 Infective endocarditis prophylaxis not indicated

12. VENTRICULAR SEPTAL DEFECT
 Most common congenital cardiac abnormality in
infants and children
 Types: 1. perimembreanous (most common)
 2. Muscular – inlet, tabecular, outlet

14. Natural history
 Depending on size and location
 Small muscular VSD- spontaneous closure
 Large– unlikely
 Initial L—R shunt
 Increase in pulmonary vascular resistance
 Pulmonary hypertension and shunt reversal

15.  Clinical features
 PSM over 3d or 4th ICS
 Mid diastolic flow murmur across mitral valve
 Features of pulmonary hypertension develops as
vascular resistance increases
 ECG: Biventricular enlargement with or withour
RBBB
 X- ray: LV enlargement with pulmonary plethora

16. Treatment
 Surgical closure
 If pulmonary vascular resistance is 1/3 or less than
systemic, progression of pulmonary HTN is unusual
 Operation is indicated when pulmonary to systemic
flow ratio >1.5:1 without high pulmonary resistance (
Pulmonary vascular resistance <2/3 of systemic
resistance)

17. PATENT DUCTUS ARTERIOSUS

18.  L– R shunt
 10% of congenital heart disease
 Functional closure occurs soon after birth;
anatomical closure is complete in 2-3 weeks after
birth
 More seen in prematurity, perinatal hypoxemia, high
altitude, maternal rubella

19. Clinical features
 bounding peripheral arterial pulses
 widened pulse pressure
 hyperdynamic left ventricular impulse
 A continuous “machinery” murmur, audible in the
second left anterior intercostal space, begins shortly
after the first heart sound, peaks in intensity at or
immediately after the second heart sound (thereby
obscuring it), and declines in intensity during
diastole.
 Flow murmurs through mitral or aortic valves

20.  Pulmonary vascular resistance increases …
 Features of pulmonary hypertension
 Shunt reversal…
 Differential cyanosis and clubbing

22.  ECG: Left atrial enlargement, Left ventricular
enlargement (volume overload), Right ventricular
enlargement ( pressure overload)
 Confirmation by 2D echo and doppler studies

23.  Complications:
 Calcification and rupture
 Infective endocarditis
 Pulmonary hypertension and Eisenmenger’s
 Treatment: Surgical ligation

24. COARCTATION OF AORTA
 Narrowing of aortic lumen
 Upper part of body is overperfused
 Lower part is underperfused
 Types: 1. pre ductal
 2. Post ductal (most common)
 Extensive collaterals to lower part of body develops

27.  Associations: gonadal dysgenesis (e.g., Turner’s
syndrome), bicuspid aortic valve, ventricular septal
defect, patent ductus arteriosus, mitral stenosis or
regurgitation, or aneurysms of the circle of Willis

28.  Clinical features:
 Upper limb BP> lower limb
 Feeble LL pulses
 Radio-femoral delay
 Systolic thrill in suprasternal notch
 Left ventricular enlargement
 Systolic ejection click
 ESM at left sternal border and back
 Continuous murmur over interscapular or
infrascapular areas

29.  ECG: LVH
 X- Ray: Dilated left subclavian and ascending aorta
 “3” Sign: Indentation of aorta at the site of
coarctation and pre and post stenotic dialatation
 Notching of 3d to 9th ribs due to inferior rib erosion
by dilated collaterals
 Conformation: 2D echo and doppler, TEE, MR or CT
angiography

30.  Complications:
 Cerebral aneurisms and hemorrhage
 Aortic dissection and rupture
 Premature coronary atherosclerosis
 Aortic valve failure, LVF
 Infective endarteritis

31.  Treatment:
 Percutaneous catheter baloon dilatation with stent
placement
 Surgical correction

32.  Other obstructive lesions:
 Congenital aortic stenosis– valvular, associated with
bicuspid valves
 Pulmonary stenosis with intact ventricular septum:
 MC cause of isolated RVOTO
 Can be A/W congenital Rubella
 Exertional dyspnoea, fatigue
 RVH, delayed P2, RV S4, Prominent A vaves in JVP
 Hepatic pulsations, LPH
 Pulmonary ESM, PSM of TR

33.  Treatment: Catheter balloon valvuloplasty

34. TETRALOGY OF FALLOT
 Malaligned VSD
 Right ventricular outflow obstruction
 Aortic override of VSD
 Right ventricular hypertophy due to right ventricular
response to aortic pressure via large VSD

36.  R-L shunt
 Cyanosis from birth
 Severity is proportionate to Right ventricular outflow
obstruction
 decrease in systemic vascular resistance increases
right-to-left shunting, whereas an increase in
systemic resistance decreases right-to-left shunting.

37. Clinical features
 Cyanosis from birth
 hypoxic “spells,” characterized by tachypnea
and hyperpnea, followed by worsening cyanosis
and, in some cases, loss of consciousness,
seizures, cerebrovascular accidents in infancy
 dyspnea and limited tolerance of exercise
 erythrocytosis, hyperviscosity, abnormalities of
hemostasis, cerebral abscesses or stroke
 endocarditis

38.  Right ventricular lift or tap
 Systolic thrill pulmonary area
 Single S1
 Aortic ejection click may be heared
 Systolic ejection murmur, audible along the left
sternal border, is caused by the obstruction of right
ventricular outflow
 Intensity is inversely proportional to severity

39.  ECG: RVH, Right axis deviation
 X- ray: “bootshaped,” with an upturned right
ventricular apex and a concave main pulmonary
arterial segment
 Arterial oxygen desaturation
 Compensatory erythrocytosis
 Confirmation: 2D echo, Angiography

41.  Treatment:
 Complete surgical correction: closure of the
ventricular septal defect and relief of right ventricular
outflow obstruction
 At present, palliative shunting or balloon pulmonary
valvuloplasty is performed only in severely ill infants
for whom complete repair is unsuitable

42.  Complications:
 Polycythemia and hyperviscosity
 Cerebral abscess
 Infective endocarditis
 CCF occurs if infective endocarditis, anemia,
pregnancy, systemic hypertension, AR, Stenosis of
aortic valve, Pulmonary atresia

43. Ebstein’s Anomaly
 Downward displacement of tricuspid valve into RV
due to anomalous attachment of TV leaflets
 Hypoplastic RV
 Progressive cyanosis (right to left shunt)
 Tricuspid regurgitation and RV dysfunction
 Paroxysmal atrial tachyarrythmias
 Atrio- ventricular byepass tracts– WPW syndrome

45. Transposition of the Great Arteries