PJB

dr Amaliah Harumi Karim

3 tanda penting yang perlu diperhatikan pada
bayi PJB
Sianosis
Penurunan perfusi sistemik
Takipnea

BBL dengan PJB biasanya mempunyai 1 dari 4 gejala ini = murmur

asimptomatik, sianosis, HF, syok
Neonatus dengan murmur asimptom
Bising transient akibat aliran darah pada duktus arteriosus yang
sedang menutup. Terdengar dalam jam2 pertama.

Bising bisa asimtom, dan simtomatik

 Lesi stenotik
katup pulmonal
simtomatik
Lesi stenotik
katup aorta
bising
VSD
asimptomatik
AVSD
 Lanjutan catatan
The increase in circulating catecholamines, combined
The term heart failure is a misnomer, however; total
To maintain this high level of left with the
left ventricular output is actually several times greater
ventricular output, heart rate and stroke volume are increased work of breathing, results in an elevation in
than
increased, total body
normal, although much of this output is ineffective
mediated by an increase in sympathetic nervous oxygen consumption, often beyond the oxygen
because it
system activity transport ability
returns directly to the lungs.
of the circulation.

Remodeling
of the heart occurs, with predominantly dilatation
and a lesser
Sympathetic activation leads to the additional
degree of hypertrophy. If left untreated, pulmonary
symptoms of sweating and irritability and the
vascular resistance
imbalance between
eventually begins to rise and, by several years of age,
oxygen supply and demand lead to failure to thrive
the
shunt volume will decrease and eventually reverse to
right to left

Additional lesions that impose a volume load on the heart
include regurgitant lesions ( Chapter 422 ) and the
cardiomyopathies
( Chapter 433 )
Regurgitation through the AV valves is most
commonly encountered in patients with partial or
complete AV
septal defects (AV canal, endocardial cushion defects). In
these
lesions, the combination of a left-to-right shunt with AV
valve
regurgitation increases the volume load on the heart and
often
leads to more severe symptoms.
Lesions Resulting in Increased Pressure Load
The pathophysiologic common
denominator of these lesions is
an obstruction to normal blood fl ow
The most frequent are obstructions to
ventricular outfl ow: valvular pulmonic
stenosis,
valvular aortic stenosis, and coarctation
of the aorta
Unless the obstruction is severe,
cardiac output will be maintained and
the clinical symptoms of
heart failure will be either subtle or
absent
Less common are obstruction to
ventricular infl ow: tricuspid
or mitral stenosis, cor triatriatum and
obstruction of the
pulmonary veins
Ventricular outfl ow obstruction can
occur at
the valve, below the valve (double-
chambered right ventricle,
subaortic membrane), or above it
(branch pulmonary stenosis or
supravalvular aortic stenosis).
 This compensation
predominantly involves an increase in cardiac wall thickness
(hypertrophy), but in later stages it also involves dilatation and
can progress to ventricular dilation and failure.
 The clinical picture is different when obstruction to outfl ow
is severe, which is usually encountered in the immediate newborn
period. The infant may become critically ill within several hours
of birth. Severe pulmonic stenosis in the newborn period (critical
pulmonic stenosis) results in signs of right-sided heart failure
(hepatomegaly, peripheral edema), as well as cyanosis from rightto-
left shunting across the foramen ovale
 Severe aortic stenosis
in the newborn period (critical aortic stenosis) is characterized
by signs of left-sided heart failure (pulmonary edema, poor perfusion)
and right-sided failure (hepatomegaly, peripheral edema),
and it may progress rapidly to total circulatory collapse. In older
children, severe pulmonic stenosis leads to symptoms of rightsided
heart failure, but not to cyanosis unless a pathway persists
for right-to-left shunting (e.g., patency of the foramen ovale).
 Coarctation of the aorta in older children and adolescents is
usually manifested as upper body hypertension and diminished
pulses in the lower extremities. In the immediate newborn period,
however, the clinical presentation of coarctation may be delayed
because of the presence of a patent ductus arteriosus. In these
patients, the open aortic end of the ductus may serve as a conduit
for blood fl ow to partially bypass the obstruction. These infants
then become symptomatic, often dramatically, when the ductus
fi nally closes, usually within the 1st 2 mo of life.
Atrial Septal Defect
ASD
Atrial septal defects (ASDs) can occur in any portion of the atrial
septum (secundum, primum, or sinus venosus)