Non-cyanotic

congenital heart disease

Non-cyanotic Congenital Heart

Disease
Medical Cadet
Weerayut Wiriyabanditkul
 Outline
Non-cyanotic congenital heart disease
1. Lesions Resulting Normal Pulmonary Blood Flow
2. Lesions Resulting Increased Pulmonary Blood Flow
3. Other Anomalies
 Non-cyanotic Congenital Heart Disease

1. Lesions Resulting Normal Pulmonary Blood Flow

1. Congenital valvar aortic stenosis
2. Coarctation of aorta
3. Cor Triatriatum
2. Lesions Resulting Increased Pulmonary Blood Flow
1. Ventricular septal defect
2. Atrial septal defect
3. Patent ductus arteriosus
4. Rupture aneurysm of sinus of valsava
5. Interrupted of aortic arch
6. Truncus arteriosus
3. Other Anomalies
1. Vascular ring
2. Vascular sling
 IAA
PDA

TA
ASD
Rupture
anuerysm of VSD
sinus of
valsava

1. Lesions Resulting
Increased Pulmonary
Blood Flow
 Cor tratraitum
Congenital
aortic stenosis
Coarctation
of aorta

2. Lesions Resulting
Normal Pulmonary
Blood Flow
3. Other Anomalies
1.Lesions Resulting Increased Pulmonary Blood Flow

1. Ventricular septal defect

2. Atrial septal defect
3. Patent ductus arteriosus
4. Rupture aneurysm of sinus of valsava
5. Interrupted of aortic arch
6. Truncus arteriosus
1. Ventricular Septal Defect
 Definition
• Hole between left and right ventricle
• Most common congenital heart disease 30-
40%
• Most common type is Membranous type
• But Perimembranous type 80% Surgery
 Ventricular Septal Defect
• magnitude of the shunt is determined by
– the size, not the location
– the level of pulmonary vascular resistance
(PVR).
 Ventricular Septal Defect

• Small defect a large resistance at defect

– shunt does not depend on the level of PVR.
• With a large VSD resistance is minimal
– shunt depends on the level of PVR.
– The lower the PVR the magnitude of shunt↑.
 Pansystolic

• enlargement of the main PA, LA, and LV, as well as an

increase in pulmonary vascular markings
• Enlargement of the LA, LV, PA and increased pulmonary
vascular markings
The difference between VSD and ASD
- presence of left atrial enlargement
– The similarities between VSD and patent ductus
arteriosus (PDA) : an enlarged LA and LV
 Ventricular Septal Defect
• most common form of congenital heart
defect
• ventricular septum divided into
– a small membranous portion
– a large muscular portion : three components—
the inlet septum, the trabecular septum, and
the outlet septum
 Perimembranous VSD Supracristal/Infundib
ular type

Atrioventricular canal
Muscular VSD
 Epidemiology
• 20-30%
• 80% of perimembranous type require surgery.
 CLINICAL MANIFESTATIONS

History
1.small VSD asymptomatic with normal growth and
development   
2.moderate to large VSD
– delayed growth and development
– decreased exercise tolerance
– repeated pulmonary infections
– CHF :common during infancy   
3.long-standing pulmonary hypertension  history of
cyanosis + decreased level of activity
 Physical Examination

1.small VSDs  acyanotic.

large VSDs  poor weight gain or signs of CHF.
Eisenmenger's syndrome Cyanosis and clubbing
2. systolic thrill may be present at the lower left sternal
border.
Precordial bulge and hyperactivity are present with a
large-shunt VSD.
 NATURAL HISTORY

1.Spontaneous closure occurs in 30% to 40%

with membranous VSDs and muscular VSDs
during the first 6 months of life.
2.CHF develops in infants with large VSDs, but
usually not until 6 to 8 weeks of age.
 NATURAL HISTORY
3.Pulmonary vascular obstructive disease may
begin to develop as early as 6 to 12 months
resulting right-to-left shunt usually does
not develop until the teenage years.
4. Infective endocarditis rarely occurs.
 X-ray Studies

1.Cardiomegaly LA, LV, Pulmonary vascular

markings increase.
2.In pulmonary vascular obstructive disease,
 PA and the hilar PAs enlarge heart size is
usually normal.
Echocardiogram
 MANAGEMENT

Medical
1.Treatment of CHF, if it develops, is indicated
with digoxin and diuretics
2.No exercise restriction is required in the
absence of pulmonary hypertension.
 3.Maintenance of good dental hygiene and
antibiotic prophylaxis against infective
endocarditis
 Surgical
Indications and Timing
• Small infants who have large VSDs and develop CHF
and growth retardation are  first Manage with
digoxin and diuretics.
• If growth failure cannot be improved by medical
therapy
• Older infants with large VSDs + evidence of elevated
pulmonary vascular resistance should be operated on
as soon as possible
 Procedure
• Requiring use of CPB + mod. Hypothermia +
cardioplegia
• Patch repair through right atrial approach. (minimally
invasive surgical techniques with smaller skin
incisions ) Cosmetic
• Left ventriculotomy in apical muscular VSD.
• Percutaneous Amplatzer device
Percutaneous Amplatzer device
2. Atrial Septal Defect
 Definition
• Opening in the interatrial septum that enable
themixing of blood from systemic and
pulmonary venous
• 10-15% of all congenital heart disease
• Most common type is Ostium secundum 80%
• More common in females
 PATHOLOGY
• There are three types of ASDs
1. Ostium secundum defect is the most
common type of ASD(50%-70% )
2. Ostium primum defects (30%)
3. Sinus venosus defect ( 10%)
 Atrial Septal Defect
• magnitude of the left-to-right shunt is
determined by
– size of the defect
– compliance of RV and LV
• The compliance of RV > LV  left-to-right
shunt
 Large defect
Relative TS

Systolic ejection

Wide and fixed split

S2

• the RA, RV, and main PA and its branches have two arrows 
dilated
 CLINICAL MANIFESTATIONS

History
• Infants and children with ASDs are usually
asymptomatic.
 CLINICAL MANIFESTATIONS

Physical Examination
1. A relatively slender body build is typical. 2.  A
widely split and fixed S2 and a grade 2-3/6 systolic
ejection murmur.
With a large left-to-right shunt, a mid-diastolic
rumble resulting from relative tricuspid stenosis at
the lower left sternal border.
3. The typical auscultatory findings may be absent in
infants
 X-ray Studies

1. Cardiomegaly with enlargement of the RA

and RV is present.
2. A prominent pulmonary artery segment
and increased pulmonary vascular
markings can be seen.
• CXR : reveal enlargement of the RA, RV, and PA, as well as an
increase in pulmonary vascular markings
 RV enlargement

RA enlargement

• The heart is mildly enlarged, with involvement of the right atrium (best
seen in the posteroanterior view) and the right ventricle (best seen in the
lateral view with obliteration of the retrosternal space). Pulmonary
vascularity is increased, and the main pulmonary artery segment is
slightly prominent.
 Atrial Septal Defect

• Absence of left atrial enlargement is one of

the helpful x-ray signs for differentiating an
ASD from VSD
 Electrocardiography
• Right axis deviation
• mild right ventricular hypertrophy (RVH)
 Surgical Closure
Indications
- A left-to-right shunt with a pulmonary-to-systemic
blood flow ratio ( p/ s) of ≥1.5:1 indicates the need for
surgical closure.
- a smaller shunt to be a surgical indication because of
the risk of paradoxical embolization and
cerebrovascular accident.
 Surgical Closure
Timing.
- Surgery is usually delayed until the patient is 3 to 4
years of age because the possibility of spontaneous
closure exists.
- surgery is performed during infancy if CHF does not
respond to medical management
 Surgical Closure
• Procedure.
• a midsternal incision under cardiopulmonary
bypass by either a simple suture or a pericardial
or Teflon patch.
• Recently, so-called minimally invasive cardiac
surgical techniques with smaller skin incisions
have become popular, especially for female
patients.
3.Patent Ductus Arteriosus
 Definition
• Connection (patent) from main or left
pulmonary artery to upper descending
thoracic aorta
• 1:2000 births
• Increase dramaticlly in prematurity
• Female > male 2:1
• PDA usually closed in 48 hours after birth.
Upper descending
thoracic aorta
connect with
Proximal part of
pulmonary artery
 Patent Ductus Arteriosus

• Hemodynamics of PDA are similar to VSD

• left-to-right shunt is determined
Ductus is small
– the resistance by the ductus (diameter, length,
and tortuosity)
Ductus is large
– level of PVR
 Patent Ductus Arteriosus

• 5% - 10% of all congenital heart defects

PATHOLOGY
1.There is a persistent patency of a normal
fetal structure between the left PA and the
descending aorta,
2.The ductus is usually cone shaped with a
small orifice to the PA
 CLINICAL MANIFESTATIONS

History
1.usually asymptomatic
2.lower respiratory tract infection, atelectasis
and CHF
(accompanied by tachypnea and poor weight gain)
 Physical Examination
1.Tachycardia and exertional dyspnea. With pulmonary vascular
obstructive disease, a right-to-left ductal shunt results in
cyanosis only in the lower half of the body
2.The precordium is hyperactive. A systolic thrill may be
present at the upper left sternal border. Bounding
peripheral pulses with wide pulse pressure.  
3.The P2 is usually normal, A grade 1-4/6 continuous
(“machinery”) murmur is best audible at the left
infraclavicular area
4. Elevated jugular venous pressure and Liver enlargement.
 Electrocardiography.

• similar to those for VSD

• LVH is seen with small to moderate PDA.
• CVH(Combined ventricular hypertrophy) is
seen with large PDA. If pulmonary vascular
obstructive disease develops, RVH may be
seen.
 X-ray Studies.
• similar to those of VSD.
1.normal with a small-shunt PDA.   
2.Cardiomegaly of varying degrees occurs with enlargement of
the LA, LV, and ascending aorta. Pulmonary vascular markings
are increased.   
3.With pulmonary vascular obstructive disease, the heart size is
normal, with a marked prominence of the PA segment and
hilar vessels.
CXR of PDA are indistinguishable
from VSD.
 MANAGEMENT

Medical
1. Indomethacin is effective in premature infant 
Inhibition of Prostaglandin synthesis
Indomethacin is ineffective in term infants with PDA
and should not be used.  
2.No exercise restriction is needed (absence of
pulmonary hypertension)   
 Nonsurgical Closure.
• Transluminal placement of various occlusive
devices. (PDA<3cm.) (better in older than age5)
The advantages
– no need for general anesthesia
– shorter hospital stay and convalescent period,
– elimination of a thoracotomy scar.
Disadvantages and potential complications
– peripheral coil embolization,
– left PA stenosis, and femoral vessel occlusion
These techniques may not be
applicable in very young infants.
 Surgical Closure

Indications
1. Premature baby with CHF and Medication failure or
contraindicated with medication(Such as azothemia,
evidence of bowel ischemia, thrombocytopenia,
intracerebral or other hemorrhage and sepsis.)
2. Size of PDA >=3 cm
• The presence of pulmonary vascular obstructive
disease is a contraindication to surgery.
 Procedure
• Standard procedure
– Triple ligation of the ductus with permanent suture
through left posterolateral thoracotomy without
cardiopulmonary bypass
– Patch closure
– Division and oversewing
• Recently, a smaller incision with video-assisted thoracoscopy
Triple ligation
Division and oversewing

Patch closure
 Prognosis
Mortality rate
Premature : very low
Older infants and children : less than 1%
Bleeding, chylothorax, vocal cord paralysis that
need reopened is uncommon.
Thank you
4. Ruptured aneurysm of sinus
valsava
 Definition
• Congenital aneurysm of the sinuses of Valsava
• Common at right sinus through the middle of
noncoronary sinus]
• May perforate into the right heart chamber
• Aortocardiac fistula
 Clinical features
• Signs and symptoms
– Rupture of the sinus aneurysm 35% acute
symptom
• Sudden breathlessness
• Precordial pain
– Predisposing factor to rupture
• Heavy exertion
• Bacterial endocarditis
• Marfan’s syndrome
 Clinical features
• Examination
– Continuous murmur
– Widened aortic pulse pressure
– Elevated jugular venous pressure with prominent
V wave from tricuspid incompetence
 Investigation
• Electrocardiogram
– Left ventricular hypertrophy
– May Biventricular hypertrophy
• Chest X-ray
– Lung congestion (LeftRight to shunt)
– Aortic root not enlargement
• Echocardiogram
– Enough for diagnosis
• Cardiac catheterization
– Associated anomaly
 Surgery
• Indication
– Acute rupture sinus of Valsava  Congestive heart failure
– Prolong rupture  congestive heart failure
– Associated anomaly : VSD, aortic incompetence
• Operation
– Repair sinus of valsava under cardiopulmonary bypass by
Dacron patch
– Closure VSD and Repair aortic incompetence (if there is
associated anomaly)
5. Interrupted of aortic arch
 Definition
• Interrupted aortic arch is complete luminal
and anatomical discontinuity between two
segments of the aortic arch. Largeventricular
septal defect is nearly always present.
• Rare defect
 Clinical features
• Signs and symptoms
– Severe congestive heart failure
– Metabolic acidosis
– Anuria
• Examination
– Diminished femoral pulse
– Cardiac murmur
 Investigation
EKG
- not specific
Chest X-ray
- Gross cardiomegaly and pulmonary congestion
Echocardiography
- made for operative intervention
- diagnosis
Cardiac catheterization
- unsure diagnosis
 Management
PGE1 is infused to maintain ductal patency and
correct acidosis
Hemodynamic status should be optimized with
mechanical ventilation and inotropic support.
Increase pulmonary resistance Directed blood
into systemic circulation by decreasing
fractional inspired oxygen and avoiding
hyperventilation
 Surgery
Indication
- When diagnosis is Interrupted of aortic arch is
indication for surgery.
Operation
- Complete surgical repair of IAA
Result
- Operative mortality is less than 10%
6. Truncus arteriosus
 Definition
• Rare anomaly
• a single arterial conduit that arises from the
heart, overrides the ventricular septum, and
supplies the pulmonary, systemic, and
coronary circulations.
 Type
• Type I is the same as A1.
• Types II and III are grouped as a single type A2
because they are not significantly distinct
embryologically or therapeutically.
• Type A3 denotes unilateral pulmonary artery with
collateral supply to the contralateral lung
(hemitruncus).
• Type A4 is truncus associated with interrupted
aortic arch (13% of all cases of truncus arteriosus)
 Clinical features
• Signs and symptoms
– Severe congestive heart failure
– Cyanosis
• Examination
– Systolic murmur or continuous murmur at left
parasternal border
 Investigation
EKG
- Bilateral ventricular hypertrophy
Chest X-ray
- Gross cardiomegaly and pulmonary congestion
Echocardiography
- Definitive diagnosis
Cardiac catheterization
- Do in unsure diagnosis
 Surgery
• Indication
– When diagnosis Truncus arteriosus is indication
Can control CHF  third months of life
Can not control CHF  Immediate surgery
• Operative Technique
1. Use cardiopulmonary bypass
2. Repair VSD
3. Reconstruction pulmonary a. and aorta by Homograft
valve conduit
4. Valve replacement
 Outcome
• Survival rate = 91% in younger than 6 months
• Risk factor for poor prognosis and
perioperative death
– Severe truncal regurgitation
– IAA
– Coexistiong coronary anomaly
– Age younger than 100 days
2.Lesions Resulting Normal Pulmonary Blood
Flow
1. Congenital valvar aortic stenosis
2. Coarctation of aorta
3. Cor Triatriatum
1. Congenital Valvar Aortic
Stenosis
 Definition
• Obstruction at valve level caused by imperfect
cusp development with leaflet thickening and
fusion
 Pathophysiology
– LVOT  LVH  LA hypertension  increased
pulmonary vascular resistance
–  RVH
– Circulatory collapse when PDA closed  cyanosis
 Clinical features
• Signs and symptoms
– History of poor feeding
– Pallor
– Perspiration
– Dyspnea
• Examination
– Systolic ejection murmur
– Absent if severe left ventricular failure
 Investigation
• EKG
– Left ventricular hypertrophy +/- Right ventricular
hypertrophy
• Chest X-ray
– Cardiomegaly with pulmonary congestion
• Echocardiogram
– Confirm diagnosis and identify other associated anomaly
• Cardiac catheterization
– Measurement of systolic gradient to calculate valvular area
 Indication for surgery
Indication
1. Transvalvular gradient >50 mmHg +
Symptomatic : syncope, CHF, angina
2. Transvalvular gradient 50-75 mmHg + EKG
LV stain and ischemia
3. Association defect
Contraindication : hypoplastic LV
 Surgery
• Indication for surgery
– Circulatory collapse from acisosis
– Fail medical treatment (Prostaglandin E1)
– Fail intervention (Balloon valvuoplasty of aortic
valve)
• Operation
– Surgical valvulotomy under cardiopulmonary
bypass
2. COARCTATION OF THE AORTA
 Definition
• Luminal narrowing of the aorta Obstruction
blood flow
• Most commonly located distal to the left
subclavian artery
• Extensive collateral  intercostals &
Mammary arteries
 Pathophysiology
• LV outflow tract obstruction
– Pulmonary over circulation
– Later Biventricular failure
• Proximal systemic hypertension from
– Mechanical obstruction to ventricula ejection
– RAAS
• Persistent hypertension
• Complication : circle of willis aneurysm, aortic
dissection, risk MI
 Clinical manifestation
• S/S
– Left ventricular failure
• Physical examination
– Decrease femoral pulse
– Hyperdynamic precordium
 cyanosis

Differential cyanosis
 COARCTATION OF THE AORTA

• On chest x-ray films

– poststenotic dilatation of the descending aorta 
figure-of-3 sign on the plain film
– Rib notching
• E-shaped on the barium esophagogram
• Echocardiogram
– Narrowed aortic segment
– Pressure gradient on the stenotic
 COARCTATION OF THE AORTA

• The ECG shows LVH because of a pressure

overload on the LV.
• In newborns and small infants, RVH or right
bundle branch block (RBBB) is commonly
seen, but LVH is not
Arteriography
• A, In symptomatic infants, associated defects are frequently found
(VSD, aortic and mitral valve abnormalities and hypoplasia of the
ascending and transverse aortas ).
• B In asymptomatic children, the coarctation is usually an isolated
lesion
 Management

Medical
1.Prostaglandin E1  reopen the ductus arteriosus and
establish flow
2.Intensive anticongestive measures with short-acting
inotropic agents (e.g., dopamine, dobutamine),
diuretics, and oxygen
3.Balloon angioplasty useful procedure for sick
infants in whom standard surgical management
carries a high risk.
 Surgery
• Indication
– If diagnosis coarctation is indication for surgery
Procedures.
• segment of coarctation is resected
• The distal subclavian artery is divided, and the
flap of the proximal portion
• Patch aortoplasty
Main major complication is aneurysm
Surgical Procedure
3. Cor triatriatum
 Definition
• Rare anomaly
• the presence of a fibromuscular diaphragm
that partitions the left atrium into two
chambers:
– Superior chamber : receives drainage from the
pulmonary veins.
– Inferior chamber : communicates with the
mitral valve and the left ventricle
A. Common chamber draining to right atrium directly.
B. B. Common chamber draining into systemic venous
circulation via anomalous vein.
 Clinical feature
Signs and symptoms
- Low cardiac output : fatigue, dyspnea,
decrease peripheral pulse, not growth
Physical examination
- loud pulmonary S2 sound
- a right ventricular heave, as well as jugular
venous distention and hepatomegaly
 Investigation
• EKG
– Right axis deviation, right atrial enlargement and
right ventricular hypertrophy.
• Chest x-ray
– heart size, pulmonary edema.
• Echocardiogram
– Diagnosis
• Cardiac catheterization
– If suggest other anomaly.
 Surgery
• Indication
– Severe pulmonary edema and pulmonary
hypertension.
• Operation
1.Cardiopulmonary bypass and cardioplegic arrest
2.Excised membrane between chamber and true left
atrium.
 3. Other anomaly
1. Vascular ring
2. Vascular sling
1. Vascular ring
 Definition
• Abnormal of great vessels(aortic arch and
branches) that compress trachea or/and
esophagus.
   Right aortic arch with mirror-image branching and
Double aortic arch retroesophageal ligamentum arteriosum.

Right aortic arch with aberrant left subclavian artery Left aortic arch with right descending aorta
and left ligamentum arteriosum. and right ligamentum arteriosum.
 Clinical features
• Signs and symptoms
– Difficult to feeding  reflux and respiratory
infection
– May stridor respiratory distress.
 Investigation
• Chest X-ray
– Normal or right aortic arch
• Echocardiogram
– Diagnosis vascular ring or other disease.
• CT scan
– If + contrast better
• Cardiac catheterization
– If others anomaly
 Surgery
• Indication
– If significant obstructive symptoms
• Operation
– Left thoracotomy
– Excision ligamentum arteriosum
– Excision fibrous band
2. Vascular sling
 Definition
• Anomalous origin of the left pulmonary artery from
the posterior aspect of the right pulmonary artery.
• The anomalous left pulmonary artery courses over
the right mainstem bronchus and then from right to
left, posterior to the trachea or carina and anterior to
the esophagus, to reach the hilum of the left lung
• Compression caused by the sling can produce
obstructive emphysema and/or atelectasis of the
right as well as the left lung.
 Clinical features
• Signs and symptoms
– 90% severe symptoms  Wheezing and stridor
 Investigation
• Chest X-ray
– Anterior bowing of Right main bronchus has
deviated of lower trachea and carina to the left
side.
– Left hilum is lower than normal.
– Unequal aeration of both lungs +/- atelectesis
• Esophagogram
– Lateral : Anterior indentation of esophagus at
carina level.
 Investigation
• Noninvasive imaging
– Echocardiogram, CT scan, MRI
• Cardiac catheterization
– If need confirm diagnosis.
– Others congenital anomaly.
 Surgery
• Indication
– If significant obstructive symptoms
• Operation
– Reimplantation of the left pulmonary artery with
or without cardiopulmonary bypass.
– Tracheal resection and relocation of the left
pulmonary artery.
Thank you
for your
attention!!