PATENT DUCTUS ARTERIOSUS

The ductus arteriosus is a normal fetal connection between the left PA and the
descending aorta. During fetal life, blood flow is shunted away from the lungs
through the ductus arteriosus and directly into the systemic circulation. PDAs are
common in premature neonates who weigh less than 1,500 g. They account for 5% to
10% of !Ds, e"cluding premature neonates.
Pathophysiology and Etiology
During fetal life, the ductus arteriosus allows blood to bypass the pulmonary
circulation #fetus recei$es o"ygen from the placenta% and flow directly into the
systemic circulation.
After birth, the ductus arteriosus is no longer needed. &unctional closure
usually occurs within '( hours after birth. Anatomic closure is completed by
age ) to * wee+s.
,hen the ductus arteriosus fails to close, blood from the aorta #high pressure%
flows into the low-pressure PA, resulting in pulmonary o$ercirculation.
.ncreased pulmonary blood flow leads to a $olume-loaded /0.
Clinical Presentation
1mall to 2oderate-1i3ed PDA
4sually asymptomatic.
/arge PDA
!&, tachypnea, fre5uent respiratory tract infections.
Poor weight gain, failure to thri$e.
&eeding difficulties.
Decreased e"ercise tolerance.
Diagnostic Evaluation
Auscultation6 continuous murmur heard best at left upper sternal border.
!yperacti$e precordium with large PDAs.
,ide pulse pressure7 bounding pulses.
hest 8-ray6 $aries7 normal or cardiomegaly with increased pulmonary
$ascular mar+ings.
9:6 $aries7 normal or /0!.
Two-dimensional echocardiogram with Doppler study and color flow mapping
to $isuali3e the PDA with left-to-right blood flow.
ardiac catheteri3ation is not needed for the initial diagnosis.
Manageent
.n the symptomatic premature neonate6 indomethacin gi$en ..0.
2edical management6
o 2onitor growth and de$elopment.
o ;eassess for spontaneous PDA closure.
o .ncrease caloric inta+e as needed for normal weight gain.
o Diuretics6 furosemide #/asi"%, spironolactone #Aldactone%.
o .nfecti$e endocarditis prophyla"is for < months after surgery or coil
occlusion.
ardiac catheteri3ation6
o &or small PDAs coil occlusion.
o &or larger PDAs a closure de$ice may be used.
1urgical management through PDA ligation.
Coplications
!&, pulmonary edema.
.nfecti$e endocarditis.
Pulmonary hypertension=pulmonary $ascular occlusi$e disease.
;ecurrent pneumonia.
ATRIA! SEPTA! DE"ECT
A1D is an abnormal communication between the left and right atrias. A1Ds account
for >% of !Ds. There are three types6
?stium secundum A1D6 the most common type of A1D7 abnormal opening in
the middle of the atrial septum.
?stium primum A1D6 abnormal opening at the bottom of the atrial septum7
increased association with cleft mitral $al$e and atrio$entricular defects.
1inus $enosus A1D6 abnormal opening at the top of the atrial septum7
increased association with partial anomalous pulmonary $enous return.
Pathophysiology and Etiology
@lood flows from the higher-pressure left atrium across the A1D into the
lower-pressure right atrium #left-to-right shunt%.
.ncreased blood return to the right heart leads to right $entricular $olume
o$erload and right $entricular dilation.
.ncreased pulmonary blood flow leads to ele$ated pulmonary artery pressures.
Clinical Mani#estations
4sually asymptomatic.
linical symptoms $ary depending on type of associated defects6
o !& #usually not until the third or fourth decade of life%.
o &re5uent upper respiratory infections #4;.s%.
o Poor weight gain.
o Decreased e"ercise tolerance.
Diagnostic Evaluation
Auscultation6 soft systolic eAection murmur heard best at the left upper sternal
border7 widely split, fi"ed second heart sound.
hest 8-ray6 $aries7 normal to right atrial and $entricular dilation, increased
pulmonary mar+ings.
9:6 $aries7 right a"is de$iation and mild ;0! or right bundle-branch bloc+.
Two-dimensional echocardiogram with Doppler study and color flow mapping
to identify the site of the A1D and associated lesions and document left-to-
right flow across the atrial septum.
ardiac catheteri3ation usually not needed for initial diagnosis7 performed if
defect can be closed using an atrial occlusion de$ice #de$ice can be used only
in ostium secundum defects%.
Manageent
2edical management6
o 2onitor and reassess #spontaneous closure rate is small but may occur
up to age )%.
o Treatment with anticongesti$e therapy #digo"in and /asi"% may be
necessary if signs of !& are present #usually not until third to fourth
decade of life if A1D unrepaired%.
o .nfecti$e endocarditis prophyla"is for < months after surgery or atrial
occlusion de$ise is used.
ardiac catheteri3ation for placement of an atrial occlusion de$ice for ostium
secundam defects.
1urgical inter$ention6
o Primary repair6 suture closure of the A1D.
o Patch repair of the A1D.
Coplications
!& #rare%.
.nfecti$e endocarditis.
9mbolic stro+e.
Pulmonary hypertension.
Atrial arrhythmias.
$ENTRICU!AR SEPTA! DE"ECT
A 01D is an abnormal communication between the right and left $entricles. .t is the
most common type of congenital heart defect, accounting for appro"imately )5% of
all !Ds. 01Ds $ary in the si3e #small and restricti$e to large and nonrestricti$e
defect%, number #single $ersus multiple%, and type #perimembranous or muscular%.
Pathophysiology and Etiology
@lood flows from the high-pressure left $entricle across the 01D into the low-
pressure right $entricle and into the PA, resulting in pulmonary
o$ercirculation.
A left-to-right shunt because of a 01D results in increased right $entricular
pressure and increased PA pressure.
The increased pulmonary $enous return to the left side of the heart results in
left atrial dilation.
/ong-standing pulmonary o$ercirculation causes a change in the pulmonary
arterial bed, leading to increased pulmonary $ascular resistance. !igh
pulmonary $ascular resistance #P0;% can re$erse the blood flow pattern that
leads to a right-to-left shunt across the 01D #9isenmengerBs syndrome%,
resulting in cyanosis. ?nce this de$elops, the child is no longer a candidate for
surgical repair.
Clinical Mani#estations
1mall 01DsCDEusually asymptomatic7 high spontaneous closure rate during
the first year of life.
/arge 01Ds.
o !&6 tachypnea, tachycardia, e"cessi$e sweating associated with
feeding, hepatomegaly.
o &re5uent 4;.s.
o Poor weight gain, failure to thri$e.
o &eeding difficulties.
o Decreased e"ercise tolerance.
Diagnostic Evaluation
Auscultation6 harsh systolic regurgitant murmur heard best at the lower left
sternal border #//1@%7 systolic thrill felt at //1@, narrowly split 1
)
.
hest 8-ray6 $aries7 normal or cardiomegaly and increased pulmonary
$ascular mar+ings. Pulmonary $ascular mar+ings are directly proportionate to
the amount of left-to-right shunting.
9:6 $aries7 normal to bi$entricular hypertrophy.
Two-dimensional echocardiogram with Doppler study and color flow mapping
to identify the si3e, number, and sites of the defects, estimate pulmonary artery
pressure, and identify associated lesions.
ardiac catheteri3ation usually not needed for initial diagnosis7 may be needed
to calculate the si3e of the shunt or to assess P0;. 2ay be performed if defect
can be closed using a $entricular occlusion de$ice #de$ice can be used only in
muscular defects%.
Manageent
1mall 01D
2edical management6
o 4sually no anticongesti$e therapy is needed.
o .nfecti$e endocarditis prophyla"is for < months after surgical
implantation of a $entricular occlusion de$ice.
ardiac catheteri3ation for placement of a $entricular occlusion de$ice for
muscular defects #for Fp6Fs G )61%.
1urgical inter$ention is usually not necessary.
2oderate to /arge 01D
2edical 2anagement6
o !& management6 digo"in and diuretics #furosemide, spironolactone%
and afterload reduction.
o A$oid o"ygen7 o"ygen is a potent pulmonary $asodilator and will
increase blood flow into the PA.
o .ncrease caloric inta+e6 fortify formula or breast mil+ to ma+e )' to *0
cal=o3 formula7 supplemental nasogastric feeds as needed.
o .nfecti$e endocarditis prophyla"is for < months after
surgery=$entricular de$ice occluder.
ardiac catheteri3ation for placement of a $entricular occlusion de$ice for
muscular defects #for Fp6Fs G )61%.
;efer for surgical inter$ention.
o 4sually repaired before age 1.
o ?ne-stage approach6 preferred surgical plan7 patch closure of 01D.
o Two-stage approach6 first surgery is to band the PA to restrict
pulmonary blood flow7 second surgery is to patch close the 01D and
remo$e the PA band.
/ong-Term &ollow-4p
2onitor $entricular function.
2onitor for subaortic membrane and double-chamber ;0.
Coplications
!&.
&re5uent 4;.s.
&ailure to thri$e7 poor weight gain.
.nfecti$e endocarditis.
9isenmengerBs syndrome.
Pulmonary hypertension.
Aortic insufficiency.