Acyanotic Congenital Heart Diseases; 1. Left-to-right shunts a. Ventricular Septal Defect(VSD) b. Atrial Septal Defect(ASD) c. Patent Ductus Arteriosus(PDA) d. Atrioventricular Septal Defect(AVSD) e. Aortopulmonary window * Eisenmenger Syndrome – The shunt becomes right-to-left 2. Left-sided obstructive lesions a. Coarctation of the Aorta(COA) b. Congenital Aortic Stenosis c. Mitral Stenosis d. Interrupted Aortic Arch Cyanotic Congenital Heart Diseases; 1. Right-to-left shunts a. Tetralogy of Fallot b. Pulmonary stenosis c. Pulmonary atresia d. Tricuspid atresia e. Ebstein’s anomaly 2. Complete mixed lesions a. Transposition of the great vessels b. Double outlet right ventricle(DORV) c. Total anomalous pulmonary venous return d. Truncus arteriosus e. Hypoplastic left heart syndrome

1. Congenital Heart Diseases
in Newborns
W. P. Rivindu H. Wickramanayake
Group no. 04a
6th Year 1st Semester – 2020 April
Tbilisi State Medical University, Georgia

2. 1. Left-to-right shunts
a. Ventricular Septal Defect(VSD)
b. Atrial Septal Defect(ASD)
c. Patent Ductus Arteriosus(PDA)
d. Atrioventricular Septal Defect(AVSD)
e. Aortopulmonary window
* Eisenmenger Syndrome – The shunt becomes right-to-left
2. Left-sided obstructive lesions
a. Coarctation of the Aorta(COA)
b. Congenital Aortic Stenosis
c. Mitral Stenosis
d. Interrupted Aortic Arch
I. Acyanotic Congenital Heart Diseases

3. 1. Right-to-left shunts
a. Tetralogy of Fallot
b. Pulmonary stenosis
c. Pulmonary atresia
d. Tricuspid atresia
e. Ebstein’s anomaly
2. Complete mixed lesions
a. Transposition of the great vessels
b. Double outlet right ventricle(DORV)
c. Total anomalous pulmonary venous return
d. Truncus arteriosus
e. Hypoplastic left heart syndrome
II. Cyanotic Congenital Heart Diseases

4. • A communication(or multiple communications) between the right
and left ventricles.
I. 1. a. Ventricular Septal Defect(VSD)

5. Based on Lesion Size;
● Restrictive VSD
– < 0.5 cm2 (Smaller than Ao valve orifice area)
– Small Left to Right shunt
– Normal RV output
– 75% spontaneously close < 2yrs
● Non-restrictive VSD
– > 1.0 cm2 (Equal to or greater than to Ao valve orifice area)
– Equal RV and LV pressures
– Large hemodynamically significant L to R shunt
– Rarely close spontaneously

6. ● Mostly asymptomatic at birth, may manifest weeks later or remain
asymptomatic throughout life.
● Most self resolve
● Larger lesions may lead to LV overload and HF
● Medication – digoxin, furosemide, captopril, enalapril
● May require surgical intervention
● Timing of the Surgery;
i. <3 months – If symptomatic
ii. 3-6 months – Symptomatic, Growth failure,
Increasing pulmonary artery hypertension(PAH)
iii. >6 months – Primarily based on PAH
iv. Wait till 1 year, if no PAH

7. I. 1. b. Atrial Septal Defect(ASD)
• A communication or opening between the atria that result in
shunting of blood between the two chambers.
Types
• Ostium Secundum (75%)
• Ostium Primum (15–20%)
• Sinus venosus (5–10%)
• Coronary sinusASDs

8. • Fatigue (tiredness)
• Tiring easily during physical
activity
• Shortness of breath
• A buildup of blood and fluid
in the lungs
• A buildup of fluid in the feet,
ankles, and legs
• Sweating
• Rapid breathing
• Poor growth
Signs and Symptoms
• Wide, fixed splitting of S2 (delayed
closure of pulmonic valve with
reduced respiratory variation)
• Midsystolic pulmonary flow or
ejection murmur
– Usually over 2nd intercostalspace
– Peaks in early-to-mid systole, ends
before S2
• Palpable RV heave
• Mid-diastolic murmurs are the result
of augmented tricuspid flow.
Physical Exam Findings

9. ● Symptoms range from none to HF.
● Distinct from patent foramen ovale in that septa are missing tissue
rather than perfused.
● O2 saturation is increased in RA,RV, and pulmonary artery.
● May lead to paradoxical emboli(systemic venous emboli use ASD to
bypass lungs and become systemic arterial emboli).
● Associated with Down syndrome.
● No specific medical treatment. Surgical intervention in indicated.

10. ● Median sternotomy with direct closure of small to moderate defect
● Larger defects closed with autologous pericardium or syntethic
patches like polyester polymer (Dacron) or polytetrafluoroethylene
(PTFE)
● Minimally invasive techniques with hemisternotomy and limited
thoracotomy is to improve cosmetic outcome
● Percutaneous Transcatheter Closure via femoral vein
- success is as good as 96% in good hands
Timing of surgery
• Ideally: 3-5 years
• Usually before 25 years
• Elderly: Improves morbidity and mortality

11. • A communication between the pulmonary artery and the aortic arch
distal to the left subclavian artery.
• Patent ductus arteriosus (PDA) is the failure of the fetal ductus
arteriosus to close after birth.
• Male:Female ratio is 1:3
• Higher incidence in infants born at high altitudes(>10,000 feet)
• Usually closes within 24-72 hours after birth
• Closure of the ductus may be delayed, or not occur at all in preterm
infants
• PDA  Pulmonary blood flow, Pulmonary congestion, workload of
the RV  Pulmonary VR and workload of the RV
I. 1. c. Patent Ductus Arteriosus(PDA)

12. Clinical Findings
● Continuous “machine-like” heart murmur
● Depend on size of the shunt and the degree
of associated pulmonary HTN.
● Asymptomatic if small ductus
● Large ductus – frequent lower RTIs, CHF,
poor weight gain
● Patency is maintained by PGE synthesis and low O2 tension.
Diagnoses
● Thoracic radiography -“triple knuckle”, cardiomegaly, distended pulmonary
vessels, +/- pulmonary edema
● ECG – Signs of left sided enlargement, +/- arrhythmias
● Echo – B-mode or Doppler, for specific Dx. Of PDA

13. ● Medical management : Indomethacin, Antiprostaglandin agents,
Aspirin, Ibuprofen and Mefanaic acid.
● Surgical closure;
● When the PDA is large except in pts. with pulmonary vascular
obstructive disease.
● Transcatheter closure of small defects has become the standard
therapy
● Transarterial occlusion of PDA --
Coil Occlusion Amplatz Canine Duct Occluder

14. I. 1. e. Aorticopulmonary Window
● This leads to pulmonary
overcirculation, and rapidly
progressive congestive heart failure,
pulmonary hypertension
● There must be two distinct and
separate semilunar valves before this
diagnosis can be made.
● A large, nonrestrictive communication between the proximal ascending
aorta and the pulmonary artery,
● resulting in a high-flow arterial level left-to-right shunt.

15. ● DDx. – Large PDAs, Truncus Arteriosus, VSD + AR ruptured
aneurysms of sinus of valsalva.
● Tx. – Divison by direct suture, Patch closure
Eisenmenger Syndrome
● Uncorrected left-to-right shunt(VSD,ASD,PDA)  pulmonary
blood flow  pathologic remodeling of vasculature  pulmonary
arterial HTN.
● RVH occurs to compensate  shunt becomes right-to-left.
● Causes late cyanosis, Clubbing, and Polycythemia

16. ● A congenital narrowing of upper
descending thoracic aorta adjacent to the
site of attachment of ductus arteriosus.
● Narrowed aorta produces increased LV
afterload and wall stress, LV Hypertrophy,
and CHF.
● Systemic perfusion is dependent on the
ductal flow and collateralization in sever
coarctation.
I. 2. a. Coarctation of Aorta

17. • Depends on patency of PDA
• Shock and HF
• Metabolic disturbances
• Hypothermia
• Hypoglycemia
• Hypo perfusion
• Renal failure
Findings in Infants

18. Management
●Medical – Intial stabilization, Ionotropic drugs, Prostaglandin E1 IV
●Surgical – 1. Subclavian flap aortoplasty
2. End-to-end anastomosis
3. Patch angioplasty and graft replacement

19. I. 2. b. Aortic Stenosis
● A narrowing that obstructs blood flow from the left ventricle, leading
to left ventricular hypertrophy and/or aortic insufficiency.
● Because the valve is thickened, it becomes stiffer and heavier,
● The aorta narrow, smaller lumen
● Increased turbulence of blood flow
● The dynamic between pressure, flow, resistance change.
- Pressure increases
- Flow decreases
- Resistance increases
● Cardiac output is increased
● LV is enlarged.

20. Types
• Valvar
• Subaortic
• Supravalvar
Signs and Symptoms
• Shortness of breath with no activity
• Chest pain(left) relieved with rest, worse
with activity
• Dizziness and/or syncope
• Narrow pulse pressure
• Palpitations
• Failure to gain weight, Poor feeding
Treatment
• Valve replacement
• Valvuloplasty
• Medication to reduce BP

21. I. 2. d. Interrupted Aortic Arch(IAA)
● Congenital absence of a portion of the aortic arch.
● There are three types of IAA, and they are labeled according to the
site of the interruption. Management
• Prostaglandin
• Surgical repair

22. II. 1. a. Tetralogy of Fallot
● A congenital heart defect characterized by the association of four
cardiac abnormalities;
1. Pulmonary infundibular stenosis(most important
determinant for prognosis)
2. Right Ventricular Hypertrophy(RVH)
3. Overriding Aorta
4. VSD
● Pulmonary stenosis forces right-to-left flow across VSD  RVH,
“tet spells”(often caused by crying, fever, and exercise due to
exacerbation of RV outflow obstruction).
● Caused by anterosuperior displacement of the infundibular septum.
● Most common cause of cyanosis in early childhood

24. • Depends upon the size of VSD and degree of RV flow obstruction.
• Blue baby(Cyanosis of lips and nail beds with crying and exertion)
• Getting tired easily with exertion
• Difficulty in feeding
• Signs of chronic hypoxemia
• Harsh systolic murmur, often accomplished by a palpable thrill.
• Boot-shaper heart on radiographs(due to poor development of
pulmonary artery)
• “Tet spells” – Spasms or contraction of a band of muscle in RV just
under Pulmonary valve less blood flow into lungs when narrows. As a
result O2 delivery becomes further reduced causing spell.
Findings

25. • Squatting – Increases Systemic Vascular Resistance(SVR) which
diminishes the right-to-left shunt and increases the pulmonary blood
flow. In turn improving the Cyanosis
• Clubbing of finger and toes
• Associated with 22q11 syndromes
Management
● Monitor for Hypoxemia, Hb levels and Ht values.
● Balloon dilatation of RV outflow tract.
● Knee chest position – to increase aortic resistance in spells
● Oxygen, Beta blockers(i.e.-Propanolol) in spells
● Acute episodes of spells – Rapid IV morphine to reduce ventilator drive,
● And Epinephrine, Phenyleprine, NE to increase BP

26. Surgical
• Blalock-Taussig Operation
- Connection between the right subclavian
artery, and the right pulmonary artery,
- which increases the amount of oxygenated
blood reaching the lungs, relieving cyanosis.
• Pott’s shunt
- Descending aorta is anastomosed to the
pulmonary artery
• Waterstont’s shunt
- Ascending aorta – Right pulmonary artery
anastomosis

27. II. 1. b. Pulmonary Stenosis
• A narrowing that obstructs blood flow from the right ventricle.
• Types;
1. Valvular – The valve leaflets are thickened and/or narrowed-
fusion of valves leave a small eccentric hole in the middle.
2. Supravalvular – Pulmonary artery just above the pulmonary
valve is narrowed
3. Subvalvular(infundibular) – The muscle under the valve area is
thickened, narrowing the outflow tract from the RV.
4. Branch peripheral – The right or left pulmonary atery is
narrowed or both may be narrowed.

28. • Usually asymptomatic and in normal health.
• Systolic ejection murmur
• Cyanosis
• In an older child, severe pulmonary valve stenosis may cause easy
fatigue or shortness of breath with physical exertion.
• Severe pulmonary valve stenosis rarely results in right ventricular
failure or sudden death.
• The echocardiogram is the most important non-invasive test to
detect and evaluate pulmonary valve stenosis.
• Cardiac catheterization is an invasive technique that enables
physicians to accurately measure the degree of pulmonary stenosis

29. ● Mild Pulmonary Stenosis
- Requires no surgical
intervention.
- These infants and
children are examined by cardiologists
at regular intervals for signs of
progression of the stenosis.
Treatment
● Moderate To Severe Pulmonary Stenosis
- Pulmonary Balloon Valvuloplasty
- Surgical Valvotomy

30. ● Pulmonary Atresia with an Intact Ventricular Septum (PAwith
IVS) includes a spectrum of defects that can include disorders of
the tricuspid valve, right ventricle and coronary circulation.
● The term ‘atresia’ indicates failure of the pulmonary valve or
pulmonary trunk vessel to develop; therefore there is no
connection between the right ventricle and pulmonary artery.
II. 1. c. Pulmonary Atresia

31. Findings
• Single second heart sound
• Systolic murmur due to tricuspid regurgitation
• Silent precordium
• “machinery-like” murmur due to PDA
• RA enlargement, Cardiomegaly[Tricuspid Regurgitation]
• Paucity of pulmonary vascularity on CTX
• RA enlargement  Tall P waves in leads I, II and AVF
• Prostaglandin infusion
• Oxygen
• Maintenance of acid-base balance
• Palliative / Corrective Surgery
Management

32. II. 1. d. Tricuspid Atresia
• The failure of development of the tricuspid valve, resulting in
a lack of direct communication between the RA and the RV.
• Incidence : 0.06 per 1000 live births
• Muscular type
• Membraneous type
• Valvar type
• Palliative surgery
• Reconstructive surgery

33. • A rare congenital defect of
the tricuspid valve.
• The tricuspid valve leaflets
do not attach normally to
the tricuspid valve annulus.
• The leaflets are dysplastic
and the septal and posterior
leaflets are downwardly
displaced, adhering to the
RV septum.
II. 1. e. Ebstein Anomaly

34. • Atresia Of Tricuspid Valve
• No communication between RAAND RV
• RV id underdeveloped.
• Systemic venous blood received by RA
• Enters LA through PFO or ASD
Pathophysiology

35. • Mixing of systemic and pulmonary blood
• Enters LV
• Blood enters RV through VSD
• From RV blood enters Pulm trunk

36. • Blood enters pulm trunk via PDA
• Increased pulmonary blood flow
• LA and LV hypertrophy
CHF

37. Clinical Features
Decreased Pulmonary Flow 90%
• Severe cyanosis, hypoxemia, and acidosis
• LV apical impulse
• Waves in jugular venous pulse
• Pulmonary oligemia
• May have central cyanosis,
• Tachypnea or hyperpnea
Increased Pulmonary Flow
• May not appear cyanotic but may present with signs of heart
failure later in infancy
• Pulmonary plethora present with symptoms of dyspnea, fatigue,
difficulty feeding, and perspiration, which are suggestive of
congestive heart failure.
• Cyanosis is minimal

38. • Holosystolic type of murmur at the lower sternal border,
suggestive of VSD,
• Problems related to chronic cyanosis, such as
• Clubbing,
• Polycythemia, relative anemia,
• Stroke, brain abscess,
• Coagulation abnormalities,
Investigation
• History and PE
• Pulse oximetry
• ABG
• Hb and hematocrit
• An intravenous infusion of PGE1
• 0.03-0.1 mcg/kg/min to open the ductus arteriosus
• Anticongestive therapy with digoxin, diuretics
• Surgical - Palliative & Tricuspid Valvuoplasty
Management

39. II. 2. a. Transposition of Great Vessels
More common
in infants of
diabetic
mothers

42. Findings
• Prominent and progressive cyanosis within the first 24hrs of
life.
• Tachypnea
• Tachycardia
• Diaphoresis
• Failure to gain weight
• A single or narrowly split, diminished second heart sound
• Systolic ejection murmur may be present
• The classic “Egg on a string” appearance in approximately
one third of patients on CTX

43. Management
• Initial treatment consists of maintaining ductal patency with
continuous IV PGE1 infusion to promote pulmonary blood flow.
• Antibiotic prophylaxis for Endocarditis
• Fluid replacement
• Bicarbonate administration – Acidosis
• Mechanical ventilation
• Surgical;
• Rashkind procedure(Palliative)
• Arterial Switch procedure(Corrective)

44. II. 2. b. Double Outlet Right Ventricle(DORV)
• Spans a wide spectrum of
physiology from Tetrology of
Fallot to Transposition of the
Great Arteries.
• DORV is a complex cardiac
defect where both great
vessels (aorta and pulmonary
artery), either completely or
nearly completely arise from
the right ventricle

45. 1. a. DORV with subaortic VSD and without PS
b. DORV with subaortic VSD and pulmonary stenosis
2. DORV with subpulmonic VSD (Taussig-Bing anomaly)
3. DORV with doubly committed VSD
4. DORV with remote VSD
Types

46. Management

47. • Results from the failure of the pulmonary veins to join normally to
the left atrium during fetal cardiopulmonary development.
II. 2. c. Total Anomalous Pulmonary Venous
Return (T-APVR)
Types;
• Supracardiac
• Cardiac
• Infradiaphramatic
• Mixed

49. • Chest X-ray ;
• Figure of 8 or snow-man appearance
• Ground-glass appearance
Findings

50. Emergency medical management
• Immediate endotracheal intubation and hyperventilation with 100% oxygen to
a PaCO2 of ˂ 30 mm Hg and correction of pH.
• Induced respiratory alkalosis decreases pulmonary vascular resistance and
improves oxygenation.
• Metabolic acidosis should be treated with NaHCO3 or Tromethamine
(THAM) infusions
• Isoproterenol has special merit for inotropic support in obstructed TAPVC
because it has pulmonary vasodilatory properties (0.1 microgm/kg/min for
24-48 hrs).
• PGE1 infusion given to maintain patency of ductus venosus to decompress
the pulmonary veins in obstructed TAPVC.

52. Surgery
various approaches for surgery-
1. Posterior approach
2. Right atrial approach
3. Superior approach

53. II. 2. d. Truncus Arteriosus
• A rare congenital heart defect in which a single great vessel arises
from the heart, giving rise to the coronary, systemic and pulmonary
arteries.
Findings
• Cyanosis at birth
• Early Congecstive cardiac failure
• Failure to thrive
• RTIs
• Bounding peripheral pulses, wide PP
• Single S2
• Harsh Systolic regurgitant murmur – VSD
• Truncal valve regurgitation

54. Management
• Treat CCF – Diuretics, Digoxin
• Ensure O2 delivery – Intubation and Mechanical ventilation
• Hypocalcemia correction in syndromic
• PGE Intravenously
• Surgical;
• Closure of VSD
• Reconstruction of the RV outflow tract
• Committing the common arterial trunk to the LV

55. • Identified as a small,
underdeveloped left
ventricle usually with
aortic and/or mitral
valve atresia or stenosis
and hypoplasia of the
ascending aorta.
II. 2. e. Hypoplastic Left Heart Syndrome
(HLHS)

57. • Stage I Norwood
• Bi-directional Glenn
• Fontan
Surgery

58. • Congenital complete heart
block is the most common
cardiac cause of fetal and
neonatal bradycardia.
• It is a disorder of
conduction due to
abnormality in the AV node
with total dissociation
between atrial and
ventricular contractions.
Congenital Complete Heart Block

59. • Isoproterenol – to increase ventricular rate
• Transventricular cardiac pacing
• Caesarean section if FHR < 50 bpm or hydrops fetalis
• Close monitoring
• Digitalis
• Diuretics
• Intraventricular pacing if HR <50 bpm during waking hours
Management