Aortic Insufficiency
Increase in pulse
pressure, LV becomes dilated and hypertropied
Pulmonary congestion and
edema are common
Clinical Feature
Cerebral insufficiency,
dizziness, pulsating headaches, dyspnea, increased pulse pressure,
and pulmonary congestion
and edema. Coronary insufficiency mat occur
Left heart failure followed
by right heart failure
Surgical replacement with
a prosthetic valve is treatment.
Aortic Stenosis
Increase in the systolic
pressure within the left ventricle
Clinical Feature
Dizziness and fainting and
angina pectoris are common. Easily fatigability exertional
dyspnea and palpitations
may also occur.
Sudden death occurs in approximately
30% of cases
Valve replacement is the
treatment of choice.
Supravalvular or Subvalvular or Idiopathic hypertrophic
subaortic stenosis
Aortopulmonary Window
Defect in which there is
a opening between the aorta; near it's valve; and the pulmonary artery.
This window functions as
a patent ductus arteriosus pathology and clinical features
are the same.
Most cases shunt is large
and increased pulmonary hypertension.
Operative closure early
is indicated, Correction contraindicated if shunt is right to left.
Atrial Septal Defect
Most common defect in adults.
Three types:
1) Ostium primum- occurs low in septum and frequently associated with partial
or complete cleft in medial mitral valve
2) Ostium secundum- most common type usually located in the central portion
of atrial septum.
3) Sinus venosus- located high on the atrial septum near opening of SVC,
occasionally anomalous drainage associated
Clinical Features
Some pulmonary hypertension
may develop no cyanosis until right to left shunt occurs.
Common atrium a secundum
defect involving almost all of septum is rare.
Cardiac Tamponade
Compression of the heart
due to the collection of fluid (blood) within the pericardium
Venous return to the right
ventricle during diastole is restricted.
Increased right ventricular
end diastolic pressure
Removal of blood from pericardium
usually results in dramatic recovery.
Cardiomyopathies
Term used for myocardial
disease of unknown etiology
Four classifications
1) Hypertrophic-subdivided into conditions with or without obstruction.
2) Congestive-characterized by congestive heart failure
3) Obliterative- conditions in which the cavity of ventricle obliterated
by abnormal tissue
4) Restrictive- restricts ventricular filling extremely rare
Coarctation of the Aorta
Most commonly found in the
thoracic aorta distal to subclavian artery but may be located anywhere
in aorta.
Constriction can be pre
or post ductal.
Constriction usually produces
arterial hypertension and left ventricular hypertrophy in proximal artery.
Distal artery will
show hypotension blood supply by collateral.
Clinical Features
Exertional dyspnea,
frank congestive heart failure, hypertension, claudication in the low extremities.
Defect repaired by resection
of the coarctation and resuturing the aorta. Small tube graft may be used
for large repair.
Cor Triatriatum
Relatively rare congenital
defect in which the left atrium is divided by a septum. commonly
located transversely, divides the left atrium into two chambers.
Posterior chamber receives
all of the flow from the pulmonary veins, a small opening or
openings connect the two left atrial chambers and offer high resistance
to blood flow.
Clinical Feature
Pulmonary venous hypertension,
pulmonary arterial hypertension, and right ventricular
hypertrophy. Difficulty in breathing; exertional dyspnea and orthopnea
Surgical correction involves
excision of the septum between the left atrial chambers.
Corrected Transposition
Defect of the great vessels
are transposed but the ventricles are also transposed.
Blood circulates in normal
fashion venous blood passes through a mitral valve.
Systemic blood passes through
a tricuspid valve then out aorta.
Clinical Features
most of time no problem
unless associated defects VSD, pulmonary stenosis, MV
abnormalities and disturbance
in the A-V conduction system.
Surgical Jatene
or Le Compte
Double Outlet
When ventricular septum so
grossly deformed that it appears to be absent. ASD and transposition commonly
found
Surgical correction is difficult and unsuccessful
in most cases.
Double outlet right ventricle both aorta and pulmonary artery leave RV may appear to be Tetralogy of
Fallot or VSD
Sometime referred to as
partial transposition.
Ebstein's Malformation
Part of the septal and posterior
leaflets are attached directly to the right ventricular wall
Divides the RV into two
parts; proximal RV (atrialized part) and distal or functional RV
Patent foramen ovale or
ASD common finding.
Tricuspid insufficiency
common and occasionally tricuspid stenosis is also present
Clinical Feature
Dyspnea, cyanosis
and clubbing are common. Cardiac arrhythmias common.
70% pts untreated die before
age 20 many suddenly.
Valvuloplasty indicated
in only 30%
Endocardial Cushion Defects
Two ridges of embryonic
tissue which fuse and close the atrioventricular canal and the septal cusps
of the mitral and
tricuspid valves.
Three types:
1) Ostium primum defect
2) Ostium primum defect is present and clefts in both the mitral and tricuspid leaflets. Small
bridge of tissue interrupts
this cleft and prevents it from becoming a common valve. A small VSD may
be present.
3) Ostium primum defect and continuous cleft through both the mitral and
tricuspid common valve to both sides.
This is called atrioventricular communis (A-V canal)
Clinical Features
Dyspnea and fatigue
common as are respiratory infections.
70% infants with defect
die within the 1st yr of life.
ASD repaired by direct closure.
Clefts are sutured closed.
Hypoplastic Left Heart Syndrome
Underdeveloped left side
of the heart.
Aortic valve atresia, extreme
hypoplasia of the aorta, mitral valve atresia or hypoplasia
Foramen ovale usually opened
and a patent ductus arteriosus along with intact ventricular
septum
Fourth most common defect
accounts for 25% of cardiac deaths during the 1st month.
Staged Norwood
procedure only treatment.
Left Ventricle-Right Atrial Communication
A high VSD connecting the
left ventricle with the right atrium surgical correction by direct closure
Mitral Insufficiency
Incomplete closure or absence
of a mitral valve.
Increased filling in left
ventricle which results in dilation and hypertrophy.
Peak pressure in atrium
can reach 50-70 mmHg
Increased pulmonary artery
and RV systolic pressures
Clinical Feature
Palpitations, fatigue,
orthopnea, and pulmonary edema secondary to LV volume overload
Right heart failure may
occur
Treatment is prosthetic valve replacement usually.
Mitral Stenosis
Most common valvular defect.
Rise in left atrial pressure
due to slowed flow to ventricle during diastole
Clinical Feature
Palpitations, weakness,
orthopnea, dyspnea, pulmonary edema, and hemolysis common
Edema and cyanosis may occur
in advanced stages.
Usually valve replacement
or mitral commissurotomy are repair.
Patent Ductus
Arteriosus
Should form ligamentum arteriosum
within 12 days of birth.
Clinical Features
Left to right Shunt;
dyspnea, fatigue, diminished growth, and cardiac failure if the shunt is
large.
Subacute bacterial endocarditis
may develop.
Could be right to left shunt
if pulmonary resistance is higher than systemic; cyanosis and fatigue main
complaints.
Ductus is closed by ligation
and/or complete division, When shunt is right to left surgical correction
contraindicated.
Pseudotruncus
Defect which includes hypoplasia
or absence of the main pulmonary artery. Pulmonary
circulation is dependent upon a patent ductus arteriosus and VSD
Clinical feature
Resemble those of
tricuspid atresia, dyspnea, fatigability, frequent respiratory infections,
cyanosis, hypoxia, and cardiac failure are all common findings.
Surgical correction involves
closing the VSD and patent ductus arteriosus, Rastelli
procedure.
Pulmonary Atresia
Valvular pulmonary atresia
is the failure of the development of the pulmonary valve and normal development
of the right ventricular chamber.
Arterial pulmonary atresia
is failure of embryologic development of the main pulmonary artery.
Circulation depends on the
patent foramen ovale or the atrial septal defect and a patent ductus arteriosus.
Clinical Features
Severe cyanosis,
clubbing, dyspnea, and fatigue are common.
Complications include subacute
bacterial endocarditis, brain abscess, embolism, and cerebral vascular accidents.
Surgical correction depends
on the RV development
If right ventricle is normal a simple pulmonary valvulotomy may be performed.
Right ventricular chamber hypoplastic with a normal pulmonary artery (
Cooley, Blalock-Taussig,
Potts, Glenn Shunts,
or the Rashkind procedure
Main Pulmonary artery hypoplastic Fontan procedure
Right ventricle normal pulmonary artery hypoplastic the Rastelli
is effective
Pulmonary Stenosis
Compromises 10-15% of congenital
defects.
Four types can occur
1) Valvular Stenosis
2) Infundibular stenosis (or combination of the two)
3) Stenosis of the pulmonary artery (Supravalvular stenosis or coarctation of the PA)
4) Peripheral pulmonary stenosis
Causes obstruction of blood
from the RV increasing the work load of the right ventricle.
Pulmonary Stenosis may be described
by RV pressure as the following:
1) mild 45mmHg or less
2) moderate 46-89 mmHg
3) severe 90 mmHg or more
Clinical Features
Exertional dyspnea, and
frank cardiac failure.
systolic pressure gradient
of 60mmHg or more indication of operation.
If pure valvular fused commissures
are incised by direct vision
If infundibular hypertrophy
in the pulmonary outflow tract is resected.
Tetralogy of Fallot
Consists of four distinct features:
1) Pulmonary stenosis
2) VSD
3) Overriding aorta
4) Right ventricular hypertrophy
If ASD present Pentalogy of Fallot
Clinical Features
Cyanosis and clubbing
common, Clotting abnormalities; hypoxic episodes, cerebral thrombosis,
brain abscess
and pulmonary hemorrhage
are other occurrences
Surgical palliation has been recommended for small
infants
1) Blalock-Taussig
or 2) Potts
Total Anomalous Pulmonary Venous
Return
When four pulmonary veins
return blood to the right side of the heart. Intracardiac defect
must be present for left side circulation. Usually a ASD. Can also be partial
left called
Partial anomalous pulmonary return.
Can fall into three categories.
1) Supracardiac- most common type 50% pulmonary vein empties into left SVC,
then innominate vein and finally into the SVC.
2) Cardiac- 30% return frequently empties into the coronary sinus, occasionally
R. atrium
3) Infracardiac- 10% return into a common venous trunk that descends and
enters the IVC
or portal vein below the diaphragm.
Clinical Feature
Cyanosis characteristic
of all types. Cardiac failure and pulmonary hypertension are consequential.
Surgical treatment aimed
at redirecting the venous return to the left side.
Transposition of the Great Vessels
Complete transposition the aorta arises from the right ventricle and is
located anterior
to the pulmonary artery, pulmonary artery arises from the left ventricle and is posterior to
the aorta. When this is present, normal circulation is interrupted and blood circulates
around
the systemic circulation without passing through the pulmonary circulation.
A patent ductus
arteriosus, patent foramen ovale are present in 50% of the cases, most
common VSD.
Clinical Features
cyanosis, frequent respiratory
infections, spells of hypoxia, or anoxia, myocardial infarction
pulmonary hypertension,
and congestive failure.
Surgical correction Blalock-Hanlon
Total correction of this
defect involves rearranging the atrial septum so that the atrial
chamber receiving blood from the superior and inferior vena cava will empty
into the
ventricle
from which the pulmonary artery leaves, same for the systemic
system.
This rearranging procedure is the Mustard or Senning
Tricuspid Atresia
Absence of tricuspid valve,
prevents normal heart circulation. Blood must flow through a
ASD or patent foramen ovale
VSD or patent ductus arteriosus must be present for blood to get to
pulmonary vessels.
Clinical Feature
high mortality rate, 50% die within six months.
Severe cyanosis, clubbing, dyspnea, and fatigue common, as is right heart
failure.
Complications include subacute bacterial endocarditis, brain abscess, embolism,
and cerebrovascular accidents.
Surgical correction usually limited to increasing pulmonary blood flow
1) Cooley shunt
2) Blalock-
Taussig
3) Potts
4) Glenn procedure
5) Rashkind
Tricuspid Insufficiency
Extremely rare finding;
congenital or rheumatic disease, trauma or endocarditis occasionally
cause tricuspid insufficiency.
Clinical Feature
Edema and ascites are usually present, also hepatomegaly, and splenomegaly.
Primary cardiac defect must be corrected. Repair is usually limited to
valvuloplasty.
Tricuspid Stenosis
Rare instances is congenital.
almost always due to rheumatic fever.
Tricuspid commissures fuse
and fibrose.
Clinical Features
right heart failure
symptoms; edema, systemic venous hypertension, and ascites.
Usually corrected by valvuloplasty
unless a valve leaflet has been destroyed, in this case
valve replacement is necessary
Truncus Arteriosus
VSD with a single vessel,
single ventricular valve. Pulmonary arteries may arise separately
or from a common stem.
Clinical Features
PVR compared to SVR
largely determines the clinical picture. If PVR low in comparison
then pulmonary flow is normal or increased. cardiac failure.
Dyspnea, fatigability, frequent
respiratory infections, cyanosis, hypoxia, clubbing are all
frequent findings.
Surgical correction involves
the closing of VSD, removing the pulmonary arteries from the
wall of the truncus, and inserting a woven Dacron tube graft which incorporates
a
prosthetic valve between RV and left pulmonary artery. Rastelli
Ventricular Septal Defect
Four
types: top to bottom of septal wall
1) located between the crista supraventricularis and the pulmonary valve
2) just caudal to the crista supraventricularis
3) Beneath the septal leaflet of the tricuspid valve in an area where the
A-V conduction bundle is susceptible to injury.
4) located in the muscular septum near the apex of the right ventricle
Clinical Features
Size and pulmonary
resistance determine the volume of blood shunted, increased pulmonary blood
flow increased PVR
When PVR becomes more pronounced
(PVR higher than SVR) right to left shunt occurs once this happens surgical
correction uniformly unsuccessful. Correction may be carried out by direct
closure
Repair procedures:
Blalock-Hanlon- does not involve CPB involves
excising a portion of the atrial septum
Blalock-Taussig- right subclavian
to pulmonary artery
Cooley
- aorta to right pulmonary artery
Fontan- right atrium to the pulmonary
artery
Glenn- superior vena cava and right pulmonary artery.
Jatene- descending aorta and main pulmonary
artery are transected into normal fashion.
Le Compte- vessels transected but the ascending
aorta is behind the pulmonary bifurcation.
Mustard- switching of the atrium in transposition
of the great vessels by inflow inversion
Norwood- right atrium to pulmonary (modified Fontan) intra
atrial baffle
Potts- aortic to left pulmonary artery
Rashkind- pulling a balloon catheter
through left atrium into right atrium Cath lab procedure (Atrial Septostomy)
Rastelli- tube graft right ventricle
and pulmonary artery
Senning- switching of the atrium in transposition
of the great vessels by inflow inversion.