CARDIAC

MALFORMATION

Submitted to:
Meljoy P. Quitoriano
Submitted by:
Enjel. L. Vinluan
Left to right Shunts

Ventricular septal
defect
Pathophysiology
  is shunt creation between the right and
left ventricles. The amount of blood
shunt and the direction of the shunted
blood determine the hemodynamic
significance of the VSD. These factors
are governed by the size, location of the
VSD and pulmonary vascular resistance.

Signs and Symptoms

 shortness of breath, fast breathing,
paleness, failure to gain weight, fast
heart rate, sweat while feeding, frequent
respiratory infection.

Management/treatment
 It usually is performed as an open-heart procedure with a chest
incision and should be performed by a surgeon who specializes in
adult congenital heart defect.
Atrial Septal Defect
Pathophysiology
 allow communication between the systemic and the pulmonary
circulations. The nature of the shunt, including direction and
magnitude of blood flow, depends on both the size of
the defect and the relative atrial pressures, which relate to the
compliances of the left and right ventricles.
 Signs and Symptoms
 shortness of breath, especially
when exercising, fatigue, swelling
of legs, feet or abdomen, heart
palpation, or skipped beats,
stroke, heart murmur, a
whooshing sound that can be
heard through a stethoscope.

Management/Treatment
 Open-heart surgery.
This type of surgery is done under
general anesthesia and requires
the use of a heart-lung machine. Through an incision in the chest,
surgeons use patches to close the hole. This procedure is the only
way to repair primum, sinus venosus and coronary sinus atrial
defects.

Patent Ductus
Arteriosus
Pathophysiology
 low fetal systemic arterial oxygen tension (PaO2) and elevated
circulating PGs (in addition to PGs made within the ductus wall
itself) play a significant role in keeping the lumen of the ductus
arteriosus patent. This is necessary for fetal circulation and
survival. After a full-term birth, the ductus closes within 24–48 h
of delivery
 Signs and Symptoms
 Fast breathing, working hard to
breath. Premature infants may
need increased oxygen help
breathing from ventilator. Poor
feeding and poor weight gain.
Tiring easily. Sweating with
exertion, such as while feeding.

Management/Treatment
 Closure of the patent ductus
arteriosus (PDA) is stimulated by
administration of prostaglandin
synthesis inhibitors, such as
indomethacin or aspirin, which is effective in premature infants
(see Pharmacologic Management and Medication). Indomethacin
(0.1 mg/kg body weight) is administered orally at 8-hour intervals.

Right to left shunts

Tetralogy of Fallot
Pathophysiology
 depends on the degree of right ventricular outflow obstruction. A
mild obstruction may result in a net left-to-right shunt through the
VSD; a severe obstruction causes a right-to-left shunt, resulting in
 low systemic arterial saturation
(cyanosis) that is unresponsive
to supplemental oxygen.
Signs and Symptoms
 Early cyanosis is a symptom of
a right-to-left shunt. A right-to-
left shunt results in decreased
blood flow through the
pulmonary system, leading to
decreased blood oxygen levels
(hypoxemia). Hypoxemia
manifests as cyanosis, causing
"blue babies."
Management/Treatment
 Surgery is the only effective treatment for tetralogy of Fallot.
Surgical options include intracardiac repair or a temporary
procedure that uses a shunt. However, most babies and older
children have intracardiac repair.

Transposition of the Great

Vessels

Pathophysiology
 Narrowing of the arteries that supply blood to the heart
(coronary arteries) Heart rhythm abnormalities (arrhythmias) Heart
muscle weakness or stiffness leading to heart failure. Narrowed
 connections where the great
vessels are connected.
Signs and Symptoms
 Early cyanosis is a symptom of
a right-to-left shunt. A right-to-
left shunt results in
decreased blood flow through the
pulmonary system, leading to
decreased blood oxygen levels
(hypoxemia). Hypoxemia
manifests as cyanosis, causing
"blue babies”
Management/Treatment
 Initial treatment of transposition
of the great arteries consists of maintaining ductal patency with
continuous intravenous (IV) prostaglandin E1 (PgE1) infusion to
promote pulmonary blood flow, increase left atrial pressure, and
promote left-to-right inter circulatory mixing at the atrial level.

Tricuspid Atresia

Pathophysiology
 Patients might have ventricular septal defects, pulmonary
valvular pathology, or transposition of the great arteries. Patients
with tricuspid atresia do not have any communication between the
right atrium and right ventricle.
 Signs and Symptoms
 blue tinge to the skin and lips
(cyanosis, difficulty breathing,
tiring easily, especially during
feedings, slow growth and poor
weight gain.

Management/Treatment
 Initial management surrounds
the stabilization of the ...
failure with pulmonary over-
circulation needs to
be treated ... is only one
functional ventricle (the left ventricle), via a systemic
to pulmonary artery shunt, usually via the right subclavian and
the right pulmonary artery.

Truncus Arteriosus
Pathophysiology
 truncus  is typified by pulmonary over circulation and systemic
ventricular volume overload. Outflow from both ventricles is
directed into the common arterial trunk.

Signs and Symptoms

  A right-to-left shunt results in
decreased blood flow through
the pulmonary system,
leading to decreased blood
oxygen levels (hypoxemia).
Hypoxemia manifests as
cyanosis, causing "blue
babies."

Management/Treatment
 treated with surgery to repair
the heart defect. Surgery is
usually performed in the
neonatal period (1-2 weeks
after birth). During the surgery, the ventricular septal defect (hole
in the wall between the right and left ventricles) is closed with a
patch.

Stenotic Lesions

Pulmonary stenosis
Pathophysiology
 the flow of blood from the right ventricle to the pulmonary artery
is obstructed by narrowing at the pulmonary valve. The greater the
constriction, the harder the right ventricle must pump to force
blood into the pulmonary artery.
 Signs and Symptoms
 Heart murmur an abnormal
whooshing sound heard using a
stethoscope, caused by turbulent
blood flow, fatigue, shortness of
breath, especially during
exertion, chest pain, loss of
consciousness (fainting)
Management/Treatment
 Most severe cases of pulmonic
stenosis can be treated with a
balloon valvuloplasty during
heart catheterization. With this
procedure, a doctor threads an
unopened balloon through the pulmonary valve and inflates it to
open the valve. Valve replacement involves using an artificial
valve or a valve from a donor.
Aortic Stenosis
Pathophysiology
 may be defined as narrowing of the aortic valve, due primarily to a
combination of progressive fibrosis and calcification of the matrix,
with consequent increase in valve stiffness, progressive reductions
in valve area and concomitant increases in left ventricular afterload
and work.

Signs and Symptoms

  Chest pain, rapid, fluttering
heartbeat, trouble breathing or
feeling short of breath, feeling
dizzy or light-headed, even
fainting. Difficulty walking short
distances, swollen ankles or feet,
difficulty sleeping or needing to
sleep or needing to sleep sitting
up.

Management/Treatment
 treatment for aortic stenosis in
adults is aortic valve
replacement, performed
surgically or percutaneously. The development of symptoms due
to aortic stenosis provides a clear indication for replacement.

Coarctation Of the Aorta

Pathophysiology
 Coarctation of the aorta imposes significant afterload on the left
ventricle (LV), which results in increased wall stress and
compensatory ventricular hypertrophy. The afterload may be
 imposed acutely, as occurs following closure of the ductus
arteriosus in neonates with severe coarctation,
Signs and Symptoms
 High blood pressure, headache, muscle weakness, leg cramps or
cold feet, nosebleed, chest pain.

Management/Treatment
 Your doctor might treat your coarctation by cutting across the
narrowed area of the aorta and then attaching a patch of synthetic
material to widen the blood vessel. Patch aortoplasty is useful if
the coarctation involves a long segment of the aorta.