Post-graduate presentation on Acyanotic congenital heart disease

1. Acyanotic Congenital
Heart Diseases
Dr. Abdul Kareem
1st year DNB Resident
Department of General Medicine
Rohini Super-Speciality Hospital

2. Classification of Congenital Heart Disease
Congenital Heart Disease
Cyanotic
Increased pulmonary
blood flow
Decreased
pulmonary blood flow
Acyanotic
• Tetrology of Fallot (ToF)
• Ebstein anomaly
• Pulmonary atresia
• Tricuspid atresia
• Transposition of great arteries (TGA)
• Truncus arteriosus
• Single ventricle
• Total anomalous pulmonary venous
return (TAPVC)
• Hypoplastic left heart syndrome (HLHS)

3. Classification of Congenital Heart Disease
Congenital Heart Disease
Cyanotic Acyanotic
With shunt (Left to right) / Increased
pulmonary blood flow Without shunt (Outflow obstruction)
• Atrial Septal Defect (ASD)
• Ventricular Septal Defect (VSD)
• Patent Ductus Arteriosus (PDA)
• Atrio-ventricular septal defect (AVSD)
• Coarctation of Aorta (CoA)
• Pulmonary Stenosis (PS)
• Aortic Stenosis (AS)

4. Atrial Septal
Defect
(ASD)

5. In the presence of an atrial septal
defect, the difference in
compliance between the (RA+ RV)
as compared to the (LA+LV),
combined with the size of the
defect itself, allows for a “shunt” of
flow (“y”) of “red” (oxygenated)
blood from the left side of the heart
to the right side (deoxygenated).
Systemic venous return of pure
deoxygenated blood (“x”) is
increased by the oxygenated
shunted blood (“y”) to increase
volume of blood (“x + y”) in the RA,
RV, and total blood flow
to the lungs. If the volume or the
sequelae of the shunted blood is
suffcient, RA and RV can dilate
(hashed lines), and arrhythmias or
shortness of breath (and
occasionally pulmonary
hypertension) can ensue.
Jameson, J. L. (2018). Harrison’s Principles of Internal Medicine (20th ed.). New York: McGraw-Hill

6. Atrial Septal Defect
 Pathophysiology:
 Left-to-right shunting is determined by the size of the defect and diastolic
properties of the heart
 ↑ Age → DM, HTN, atherosclerosis → ↓ compliance of left chambers →
↑ shunting → ↑ symptoms
 Symptoms:
 Mostly asymptomatic till late adulthood, often incidentally diagnosed
 Exercise intolerance, arrhythmia, dyspnea with exertion
 Children: Failure to thrive, frequent respiratory infections

7. Atrial Septal Defect
 Physical Examination:
 Classical finding: Wide, fixed splitting of S2
 Due to prolonged RV ejection & increased pulmonary artery capacitance
 Murmurs:
 Increased blood flow across pulmonary valve → systolic ejection murmur at 3rd ICS
 Increased blood flow across tricuspid valve → mid-diastolic murmur at lower right
sternum
 Right ventricular heave (due to RVH)

8. ASD: Auscultation

9. ASD: Investigations
 Chest X-ray:
 Enlarged heart
 Enlarged pulmonary artery
 Increased pulmonary vascular markings, central plethora
 ECG:
 Right axis deviation in secundum defect
 Hallmark of primum defect is Left axis deviation
 RVH, RBBB patterns may be seen
 Echocardiography:
 RVH
 Valve anatomy, flow direction

10. ASD: Complications
 Right heart dilation:
 Risk factor for progression toward symptomatic right heart failure, atrial arrhythmias, and
potential development of pulmonary hypertension
 Therefore, these patients must be offered ASD closure
 Pulmonary hypertension:
 Pulmonary vascular disease leasing to pul. hypertension occurs in 10% of cases
 Eisenmenger syndrome (ES) is a rare complication

11. ASD: Types
 Secundum type:
 Most common type
 In the region of fossa ovalis
 Different from patent foramen ovale (PFO),
which is the persistence of the flap valve of
fossa ovalis
 Can be closed percutaneously, except if large in
size, inadequate tissue rims or associated
TAPVC

12. ASD: Types
 Primum ASD:
 Deficiency in AV canal portion of the atrial
septum
 Always associated with abnormal development
of AV valves, most commonly resulting in cleft in
mitral valve
 Coronary sinus defect:
 Rare, opening between coronary sinus & left atrium

13. ASD: Types
 Sinus venosus defect:
 Not a defect in the atrial septum
 Defect between right superior vena caval-atrial
junction & right upper pulmonary veins
 Or less commonly, the inferior vena caval-atrial
junction and the right lower pulmonary veins
 Management:
 Percutaneous closure for secundum ASDs
 Surgical closure is required for primum ASDs,
coronary sinus & sinus venosus defects.

14. Ventricular
Septal
Defect
(VSD)

15. Ventricular Septal Defect
 Most common congenital heart defect recognized at birth
 But only 10% of CHD in adult as high rate of spontaneous closure of small
VSDs in early years of life
 Large VSDs – cause symptoms of heart failure & poor somatic growth, have a
higher risk of pulmonary hypertension & Eisenmenger syndrome

16. In the presence of a ventricular
septal defect, the difference in
pressure and outflow resistance in
systole (and the difference in
compliance in diastole) between
the RV and LV, combined with the
size of the defect itself, allow for a
“shunt” of flow (“y”) of “red”
(oxygenated) blood from the left
side of the heart to the right side
(deoxygenated). Systemic venous
return of pure deoxygenated blood
(“x”) is increased by the
oxygenated shunted blood
(“y”) to increase volume of blood
(“x + y”) through the outflow of the
RV into the lungs, and in the left
atrium and left ventricle. If the
volume or the sequelae of the
shunted blood is suffcient, LA and
LV can dilate (hashed lines), and
arrhythmias or shortness of breath
(and occasionally pulmonary
hypertension) can ensue.
Jameson, J. L. (2018). Harrison’s Principles of Internal Medicine (20th ed.). New York: McGraw-Hill

17. Ventricular Septal Defect
 Pathophysiology:
 Left-to-right shunt as long as pulmonary vascular resistance is lower than system
(may reverse if increased – Eisenmenger syndrome)
 Clinical Features:
 Mostly asymptomatic
 Growth failure, recurrent respiratory infections
 Congestive heart failure
 Risk of infective endocarditis increased

18. Ventricular Septal Defect
 Clinical Examination:
 Parasternal thrill
 Pansystolic murmur at lower left sternal edge (loud if small defect)
 Large VSD → Increased flow across pulmonary valve → Ejection systolic
murmur
 Loud P2 if pulmonary hypertension develops

19. VSD: Classification
 Membranous type:
 Most common, also called peri-membranous or outlet
type
 Muscular type:
 Often pressure and flow restricted, resulting in no
significant hemodynamic consequence
 Atrioventricular canal defects:
 Also referred to as inlet defects
 Located in the crux of the heart
 Associated with abnormalities of the AV valve leaflets

20. VSD: Classification
 Sub-pulmonary type:
 Also known as conal septal defects
 Commonly associated with prolapse of the right
coronary cusp and aortic insufficiency

21. VSD: Investigations
 Chest X-ray:
 Cardiomegaly, enlarged LA & LV
 Enlarged PA, increased pulmonary vascular markings
 Pulmonary edema may be seen
 ECG:
 Extreme left axis is characteristic, biventricular hypertrophy
 Echocardiography:
 Determines chamber sizes and pressures
 Cardiac catheterization:
 Oxygen content, PA pressure & size, number of defects

22. VSD: Management
 Majority close spontaneously before 1 year of age, less than 10% require
surgery
 Treatment:
 Surgical closure before pulmonary vascular changes become irreversible
 Endocarditis prophylaxis
 Heart failure management: Diuretics, ACEIs
 Prognosis:
 Outcome for adults with small VSDs without evidence of ventricular dilation or pulmonary
hypertension is generally excellent

23. Patent Ductus
Arteriosus
(PDA)

24. In the presence of a patent ductus
arteriosus, the difference in
pressure and resistance in both
systole and diastole between
the pulmonary arteries and the
aorta, combined with the size of
the ductus itself, allow for a “shunt”
of flow (“y”) of “red” (oxygenated)
blood from the aorta to the
pulmonary arteries
(deoxygenated). Systemic venous
return of pure deoxygenated blood
(“x”) is increased by the
oxygenated shunted blood (“y”) to
increase volume of blood (“x + y”)
in the lungs, the left atrium, the left
ventricle, and out the aortic valve.
If the volume or the sequelae of
the shunted blood is suffcient, LA
and LV can dilate (hashed lines),
and arrhythmias or shortness of
breath (and occasionally
pulmonary hypertension) can
ensue.
Jameson, J. L. (2018). Harrison’s Principles of Internal Medicine (20th ed.). New York: McGraw-Hill

25. Jameson, J. L. (2018). Harrison’s Principles of Internal Medicine (20th ed.). New York: McGraw-Hill

26. Patent Ductus Arteriosus
 Courses between the aortic isthmus and the origin of one of the branch
pulmonary arteries
 Small PDAs → often silent to auscultation, and do not cause hemodynamic
changes
 Large PDAs will lead to left heart dilation and may lead to chronically elevated
pulmonary vascular resistance, including the potential for Eisenmenger
Syndrome.
 Clinical features:
 Depend on size & direction of flow
 Slow growth, recurrent LRTIs

27. Patent Ductus Arteriosus
 Classic continuous “machinery” murmur:
 Best heard below left clavicle
 Typically extends from systole past S2 into diastole, reflecting flow turbulence
and gradient between the aorta and the pulmonary arteries (resulting in L→R
shunting)
 Bounding pulse

28. Patent Ductus Arteriosus
 Investigations:
 Chest X-ray: Cardiomegaly, increased pulmonary vascularity
 ECG: Biventricular hypertrophy
 Echocardiography: visualizes PDA, Doppler shows turbulence
 Cardiac catheterization: Shows PA pressures & oxygen saturations
 Treatment:
 Infective endocarditis prophylaxis as long as duct is patent
 Indomethacin, a PG-E1 inhibitor, may close the PDA
 Surgical ligation / coiling / clipping / division may be done

29. Eisenmenger
Syndrome

30. Eisenmenger Syndrome
 Final common pathway for all significant left to right
shunts
 Unrestricted pulmonary blood flow leads to
pulmonary vaso-occlusive disease (PVOD) after
which reversal of shunt occurs and cyanosis
develops
 Generally need Qp : Qs ratio of > 2:1 (pulmonary to
systemic blood flow ratio determined by Doppler)
 Management:

32. Classification of Congenital Heart Disease
Congenital Heart Disease
Cyanotic Acyanotic
With shunt (Left to right) / Increased
pulmonary blood flow Without shunt (Outflow obstruction)
• Atrial Septal Defect (ASD)
• Ventricular Septal Defect (VSD)
• Patent Ductus Arteriosus (PDA)
• Atrio-ventricular septal defect (AVSD)
• Coarctation of Aorta (CoA)
• Pulmonary Stenosis (PS)
• Aortic Stenosis (AS)

33. Coarctation
of Aorta
(CoA)

34. Coarctation of Aorta
 Shelf-like obstruction at the level of the
descending aorta that passes just posterior
to the junction of the main and left PA
 98% are at a segment adjacent to ductus
arteriosus

35. Coarctation of Aorta
 Poor prognosis if unrepaired
 Physical Examination:
 Lower extremity blood pressure and pulses are lower than, and delayed in timing,
to the upper extremity (unless significant aortic collaterals have developed)
 A continuous murmur over the scapula may be present, due to the collateral blood
flow
 Chest X-ray: Rib-notching is pathognomonic

36. Coarctation of Aorta
Rib-notching
on chest x-ray

37. Coarctation of Aorta  Associations:
 Bicuspid aortic valve (typically with right-left
commissural fusion) is a common association.
 Descending or aortic aneurysm / enlargement
 LV diastolic & systolic heart failure
 Accelerated coronary or cerebral
atherosclerosis
 Cerebral aneurysm formation
 Recurrence of coarctation after repair
 Turner syndrome in females (short stature,
webbed neck, lymphedema, primary
amenorrhea)

38. Coarctation of Aorta
 Surgical correction:
 Patch aortoplasty with removal of segment
and end-to-end anastomosis
 Bypass tube grafting around segment
 Prognosis:
 Good prognosis on surgical repair, but remain at
risk for systemic hypertension, premature
atherosclerosis, LV failure, as well as aortic
aneurysm, dissection, and recurrent coarctation

39. Others
 Other obstructive acyanotic congenital heart diseases include pulmonary stenosis &
aortic stenosis
 Pulmonary stenosis:
 No symptoms in mild or moderate lesions. Cyanosis, RVH, right heart failure seen with
severe lesions.
 High pitched systolic ejection murmur heard, loudest at 2nd left ICS. Ejection click often
present
 Oligemic lung fields on chest x-ray
 Aortic stenosis:
 Most common obstructive type, usually asymptomatic in children
 May cause severe heart failure in infants
 Harsh systolic murmur and thrill along left sternal border, systolic ejection click heard.

40. Thank you