(See also Overview of Congenital Cardiovascular Anomalies Overview of Congenital Cardiovascular Anomalies Congenital heart disease is the most common congenital anomaly, occurring in almost 1% of live births ( 1). Among birth defects, congenital heart disease is the leading cause of infant mortality... read more .)
Patent ductus arteriosus accounts for 5 to 10% of congenital heart anomalies; the male:female ratio is 1:3. PDA is very common among premature infants (present in about 45% with birth weight < 1750 g and in 70 to 80% with birth weight < 1200 g). About one third of PDAs will close spontaneously, even in extremely low birth weight infants. When persistent in premature infants, a significant PDA can result in heart failure Heart failure Congenital heart disease is the most common congenital anomaly, occurring in almost 1% of live births ( 1). Among birth defects, congenital heart disease is the leading cause of infant mortality... read more , exacerbation of lung disease of prematurity, pulmonary hemorrhage, renal insufficiency, feeding intolerance, necrotizing enterocolitis Necrotizing Enterocolitis Necrotizing enterocolitis is an acquired disease, primarily of preterm or sick neonates, characterized by mucosal or even deeper intestinal necrosis. It is the most common gastrointestinal emergency... read more , and even death.
Pathophysiology of PDA
The ductus arteriosus is a normal connection between the pulmonary artery and aorta; it is necessary for proper fetal circulation. At birth, the rise in PaO2 and decline in prostaglandin concentration cause closure of the ductus arteriosus, typically beginning within the first 10 to 15 hours of life. If this normal process does not occur, the ductus arteriosus will remain patent (see figure Patent ductus arteriosus Patent ductus arteriosus ).
Patent ductus arteriosus
Pulmonary blood flow, LA and LV volumes, and ascending AO volume are increased.
AO = aorta; LA = left atrium; LV = left ventricle;
PA = pulmonary artery.
Physiologic consequences depend on ductal size. A small ductus rarely causes symptoms. A large ductus causes a large left-to-right shunt Left-to-right shunts Congenital heart disease is the most common congenital anomaly, occurring in almost 1% of live births ( 1). Among birth defects, congenital heart disease is the leading cause of infant mortality... read more . Over time, a large shunt results in left heart enlargement, pulmonary artery hypertension, and elevated pulmonary vascular resistance, ultimately leading to Eisenmenger syndrome Eisenmenger Syndrome Eisenmenger syndrome is a complication of uncorrected large intracardiac or aortic to pulmonary artery left-to-right shunts. Increased pulmonary resistance may develop over time, eventually... read more .
Symptoms and Signs of PDA
Clinical presentation depends on patent ductus arteriosus size and gestational age at delivery. Infants and children with a small PDA are generally asymptomatic; infants with a large PDA present with signs of heart failure Heart failure Congenital heart disease is the most common congenital anomaly, occurring in almost 1% of live births ( 1). Among birth defects, congenital heart disease is the leading cause of infant mortality... read more (eg, failure to thrive, poor feeding, tachypnea, dyspnea with feeding, tachycardia). Premature infants may present with respiratory distress, apnea, worsening mechanical ventilation requirements, or other serious complications (eg, necrotizing enterocolitis Necrotizing Enterocolitis Necrotizing enterocolitis is an acquired disease, primarily of preterm or sick neonates, characterized by mucosal or even deeper intestinal necrosis. It is the most common gastrointestinal emergency... read more ). Signs of heart failure occur earlier in premature infants than in full-term infants and may be more severe. A large ductal shunt in a premature infant often is a major contributor to the severity of the lung disease of prematurity.
Most children with a small PDA have normal 1st and 2nd heart sounds and peripheral pulses. A grade 1 to 3/6 continuous murmur is heard best in the upper left sternal border (see table Heart Murmur Intensity Heart Murmur Intensity ). The murmur extends from systole to beyond the 2nd heart sound (S2) into diastole and typically has a different pitch in systole and diastole.
Full-term infants with a significant PDA shunt have full or bounding peripheral pulses with a wide pulse pressure. A grade 1 to 4/6 continuous murmur is characteristic. If the murmur is loud, it has a “machinery sounding” quality. An apical diastolic rumble (due to high flow across the mitral valve) or gallop rhythm may be audible if there is a large left-to-right shunt or heart failure develops.
Premature infants with a significant shunt have bounding pulses and a hyperdynamic precordium. A heart murmur occurs in the pulmonary area; the murmur may be continuous, systolic with a short diastolic component, or only systolic, depending on the pulmonary artery pressure. Some infants have no audible heart murmur.
Diagnosis of PDA
Chest x-ray and ECG
Diagnosis is suggested by clinical examination, supported by chest x-ray and ECG, and established by 2-dimensional echocardiography with color flow and Doppler studies.
Chest x-ray and ECG are typically normal if the patent ductus arteriosus is small. If the shunt is significant, chest x-ray shows prominence of the left atrium, left ventricle, and ascending aorta and increased pulmonary vascular markings; ECG may show left ventricular hypertrophy.
Echocardiography provides important information about the hemodynamic significance of a PDA by assessing a number of parameters, including the
Size of the PDA (often compared to the left pulmonary artery size)
Flow velocity in the PDA
Presence of left heart enlargement
Presence of diastolic reversal of flow in the descending aorta
Presence of diastolic antegrade flow in the left pulmonary artery
Cardiac catheterization is not necessary unless used for therapy.
Treatment of PDA
Supportive medical therapy
In symptomatic premature infants, cyclo-oxygenase (COX) inhibitor therapy (eg, indomethacin, ibuprofen lysine)
Sometimes transcatheter closure or surgical repair
Typical medical management of patent ductus arteriosus includes fluid restriction, a diuretic (usually a thiazide), maintenance of hematocrit ≥ 35 %, providing a neutral thermal environment, and, for ventilated patients, use of positive end-expiratory pressure (PEEP) to improve gas exchange.
Treatment differs depending on whether the infant is premature or full term.
PDA treatment in premature infants
Fluid restriction may facilitate ductal closure.
In premature infants without respiratory or other compromise, a patent ductus arteriosus is typically not treated.
In premature infants with a hemodynamically significant PDA and compromised respiratory status, the PDA can sometimes be closed by using a COX inhibitor (either ibuprofen lysine or indomethacin [see table Indomethacin Dosing Guidelines Indomethacin Dosing Guidelines* for doses]). COX inhibitors work by blocking the production of prostaglandins. Three doses of indomethacin are given IV every 12 to 24 hours based on urine output; doses are withheld if urine output is < 0.6 mL/kg/hour. An alternative is ibuprofen lysine 10 mg/kg orally followed by 2 doses of 5 mg/kg at 24-hour intervals.
In the past, if fluid restriction and/or a COX inhibitor was unsuccessful, the PDA was ligated surgically. Over the past decade, it has been recognized that this nonselective approach to PDA therapy has not resulted in better long-term outcomes. More recent efforts have focused on better defining the subgroup of patients with a hemodynamically significant PDA in whom surgery is more likely to be beneficial. Echocardiography plays an important role in this determination of hemodynamic significance.
A few centers have successfully used transcatheter closure of PDAs in preterm infants < 2 kg.
PDA treatment in full-term infants
In full-term infants, COX inhibitors are usually ineffective.
Transcatheter closure has become the treatment of choice for PDA in children > 1 year, and some authors consider transcatheter closure to be the preferred route in term neonates and young infants as well. A variety of catheter-delivered occlusion devices are available (eg, coils, septal duct occluder).
In infants < 1 year who have ductal anatomy unfavorable for transcatheter closure, surgical division and ligation may be preferred over the transcatheter approach. For a patent ductus arteriosus with a shunt large enough to cause symptoms of heart failure or pulmonary hypertension, closure should be done after medical stabilization. For a persistent PDA without heart failure or pulmonary hypertension, closure can be done electively any time after 1 year. Delaying the procedure minimizes the risk of a vascular complication and allows time for spontaneous closure.
Outcomes after PDA closure are excellent.
Endocarditis prophylaxis Prevention Infective endocarditis is infection of the endocardium, usually with bacteria (commonly, streptococci or staphylococci) or fungi. It may cause fever, heart murmurs, petechiae, anemia, embolic... read more is not needed preoperatively and is required only for the first 6 months after closure or if there is a residual defect adjacent to a transcatheter-placed device or surgical material.
Patent ductus arteriosus (PDA) is a persistence after birth of the normal fetal connection (ductus arteriosus) between the aorta and pulmonary artery, resulting in a left-to-right shunt.
Manifestations depend on the size of the PDA and the age of the child, but a continuous murmur is characteristic and, if loud, has a “machinery sounding” quality.
Premature infants may have respiratory distress or other serious complications (eg, necrotizing enterocolitis).
Over time, a large shunt causes left heart enlargement, pulmonary artery hypertension, and elevated pulmonary vascular resistance, ultimately leading to Eisenmenger syndrome if untreated.
For premature infants with hemodynamically significant PDA, give a cyclo-oxygenase (COX) inhibitor (eg, ibuprofen lysine or indomethacin). Surgical closure may benefit patients with a hemodynamically significant PDA in whom medical therapy has failed.
For full-term infants and older children, COX inhibitors are usually ineffective, but a catheter-delivered occlusion device or surgery typically provides long-term correction of this anomaly.
The following are some English-language resources that may be useful. Please note that The Manual is not responsible for the content of these resources.
American Heart Association: Common Heart Defects: Provides overview of common congenital heart defects for parents and caregivers
American Heart Association: Infective Endocarditis: Provides an overview of infective endocarditis, including summarizing prophylactic antibiotic use, for patients and caregivers