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OVERVIEW
Background
Patent ductus arteriosus (PDA), in which there is a persistent
communication between the descending thoracic aorta and the pulmonary
artery that results from failure of normal physiologic closure of the fetal
ductus (see image below), is one of the more common congenital heart
defects.
Historical information
Galen initially described the ductus arteriosus in the early first century.
Harvey undertook further physiologic study in fetal circulation. However, it
was not until 1888 that Munro conducted the dissection and ligation of the
ductus arteriosus in an infant cadaver, and it would be another 50 years
before Robert E. Gross successfully ligated a patent ductus arteriosus
(PDA) in a 7-year-old child. [1] This was a landmark event in the history of
surgery and heralded the true beginning of the field of congenital heart
surgery. Catheter-based closure of the structure was first performed in
1971.
Anatomy
During fetal life, the ductus arteriosus is a normal structure that allows
most of the blood leaving the right ventricle to bypass the pulmonary
circulation and pass into the descending aorta. Typically, only about 10%
of the right ventricular output passes through the pulmonary vascular bed.
The ductus arteriosus is a remnant of the distal sixth aortic arch and
connects the pulmonary artery at the junction of the main pulmonary artery
and the origin of the left pulmonary artery to the proximal descending
aorta just after the origin of the left subclavian artery. It passes from the
anterior aspect of the pulmonary artery to the posterior aspect of the aorta.
Typically, the ductus has a conical shape with a large aortic end tapering
into the small pulmonary connection. The ductus may take many shapes
and forms, from short and tubular to long and tortuous.
Most typically, the patent ductus arteriosus (PDA) is a left aortic remnant;
however, it can be right-sided or on both the left side and right side.
Although a left ductus arteriosus is a normal structure during normal fetal
development, the presence of a right ductus arteriosus is usually
associated with other congenital abnormalities of the cardiovascular
system, most typically involving the aortic arch or conotruncal
development.
The Krichenko classification of PDA is based on angiography and includes
type A (conical), type B (window), type C (tubular), type D (complex), and
type E (elongated) PDA.
Pathophysiology
The ductus arteriosus is normally patent during fetal life; it is an important
structure in fetal development as it contributes to the flow of blood to the
rest of the fetal organs and structure. From the 6th week of fetal life
onwards, the ductus is responsible for most of the right ventricular outflow,
and it contributes to 60% of the total cardiac output throughout the fetal
life. Only about 5-10% of its outflow passes through the lungs.
If the SVR is high and/or the PVR is low, the flow through the ductus
arteriosus is potentially large. Beginning at the ductus arteriosus, the
course of blood flow (through systole and diastole) in a typical patent
ductus arteriosus (PDA) with pulmonary overcirculation is as follows:
patent ductus arteriosus (PDA), pulmonary arteries, pulmonary capillaries,
pulmonary veins, left atrium, left ventricle, aorta, patent ductus arteriosus
(PDA). Therefore, a large left-to-right shunt through a patent ductus
arteriosus (PDA) results in left atrial and left ventricular enlargement. The
pulmonary veins and the ascending aorta can also be dilated with a
sufficiently large patent ductus arteriosus (PDA). In addition, if little or no
restriction is present at the level of the patent ductus arteriosus (PDA),
pulmonary hypertension results.
Although functional closure usually occurs in the first few hours of life, true
anatomic closure, in which the ductus loses the ability to reopen, may take
several weeks. A second stage of closure related to fibrous proliferation of
the intima is complete in 2-3 weeks.
Vo l u m e - p r e s s u r e r e l a t i o n s h i p s
Further progression of disease is dependent on volume and pressure
relationships, as follows:
Volume = pressure/resistance
High volume yields increasing pulmonary artery pressures,
eventually producing endothelial and muscular changes in the vessel
wall
These changes may eventually lead to pulmonary vascular
obstructive disease (PVOD), a condition of resistance to pulmonary
blood flow that may be irreversible and will preclude definitive repair
Etiology
Genetics
Familial cases of patent ductus arteriosus (PDA) have been recorded, but
a genetic cause has not been determined. In infants born at term who
have a persistent patent ductus arteriosus (PDA), the recurrence rate
among siblings is 5%. Some early evidence suggests that as many as one
third of cases are caused by a recessive trait labeled PDA1, located on
chromosome 12, at least in some populations.
Chromosomal abnormalities
Several chromosomal abnormalities are associated with persistent
patency of the ductus arteriosus. Implicated teratogens include congenital
rubella infection in the first trimester of pregnancy, particularly through 4
weeks' gestation (associated with patent ductus arteriosus [PDA] and
pulmonary artery branch stenosis), fetal alcohol syndrome, maternal
amphetamine use, and maternal phenytoin use.
Prematurity
Prematurity or immaturity of the infant at the time of delivery contributes to
the patency of the ductus. Several factors are involved, including
immaturity of the smooth muscle within the structure or the inability of the
immature lungs to clear the circulating prostaglandins that remain from
gestation. These mechanisms are not fully understood. Conditions that
contribute to low oxygen tension in the blood, such as immature lungs,
coexisting congenital heart defects, and high altitude, are associated with
persistent patency of the ductus.
Other
Other causes include low birth weight (LBW), prostaglandins, high altitude
and low atmospheric oxygen tension, and hypoxia.
Epidemiology
The estimated incidence of patent ductus arteriosus (PDA) in US children
born at term is between 0.02% and 0.006% of live births. This incidence is
increased in children who are born prematurely (20% in premature infants
> 32 weeks' gestation up to 60% in those < 28 weeks' gestation), children
with a history of perinatal asphyxia, and, possibly, children born at high
altitude. In addition, up to 30% of low birth weight infants (< 2500 g)
develop a patent ductus arteriosus (PDA). Siblings also have an increased
incidence. Perinatal asphyxia usually only delays the closure of the
ductus, and, over time, the ductus typically closes without specific therapy.
Prognosis
The prognosis is generally considered excellent in patients in whom the
patent ductus arteriosus (PDA) is the only problem. In premature infants
who have other sequelae of prematurity, these sequelae tend to dictate
prognosis of patent ductus arteriosus (PDA).
Morbidity
Morbidity and mortality rates are directly related to the flow volume
through the ductus arteriosus. A large patent ductus arteriosus (PDA) may
cause congestive heart failure (CHF); if left untreated for a long period,
pulmonary hypertension may develop. Occasionally, the ductus arteriosus
patency can be intermittent.
Premature babies, particularly low birth weight neonates, are more likely
to have problems related to patent ductus arteriosus (PDA). Spontaneous
closure of the patent ductus arteriosus (PDA ) in premature neonates is
common, but respiratory distress and impaired systemic oxygen delivery
(CHF) often drive the need for therapy to effect ductal closure in this
group. Low birth weight neonates with a patent ductus arteriosus (PDA)
are more likely to develop chronic lung disease.
Mortality
No firm statistics exist, but survival rates are decreased in patients with
large shunts. The surgical mortality rate in premature infants ranges from
20% to 41%. With the availability of antibiotics to treat endocarditis and
low-risk surgery and catheter techniques to obliterate the patent ductus
arteriosus (PDA), the mortality rate appears to be quite low except in the
extremely premature infant.
It is estimated that left untreated, the mortality rate for patent ductus
arteriosus (PDA) is 20% by age 20 years, 42% by age 45 years, and 60%
mortality rate by age 60 years. An estimated 0.6% per year undergoes
spontaneous closure.
Clinical Presentation
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