PATENT DUCTUS ARTERIOSUS PDA

Patent ductus arteriosus (PDA) is a condition in which the ductus arteriosus does not close. (The word "patent" means open.) The ductus arteriosus is a blood vessel that allows blood to go around the baby's lungs before birth. Soon after the infant is born and the lungs fill with air, the ductus arteriosus is no longer needed. It usually closes in a couple of days after birth. PDA leads to abnormal blood flow between the aorta and pulmonary artery, two major blood vessels that carry blood from the heart.

Anatomy The ligamentum arteriosum is a piece of fibrous tissue that develops from the ductusarteriosus (DA). It takes shape within three weeks of a person's birth and remains a feature in the normal adult heart. The term ligamentum arteriosum is the Latin phrase for the arterialligament. Some people describe the ligamentum arteriosum as a non-functional bit of the ductusarteriosus after embryonic formation. Others, however, cite the vestige as contributing to the rupture of the aorta the body's largest artery during major trauma. This is because it keeps the artery in place when it springs back to its original position during a rapid deceleration. As a result, the aorta may break open. During prenatal development, the heart of the fetus has a small hole or passage called the ductus arteriosus. Also called the ductus Botalli, it connects the pulmonary artery, which transports deogygenated blood from the heart to the lungs and the area of the aorta, referred to as the aortic arch. In the fetus, however, the DA is meant for permitting a significant amount of blood from the heart's right ventricle to pass around the fluid-filled lungs. Within 12 to 24 hours after birth, however, the DA begins to close a process that is typically completed within three weeks. The ligamentum arteriosum is what is left after this closure, and is connected to the the aortic arch and the pulmonary artery. The pulmonary artery is located to the arterial ligament's inferomedial, or lower middle, area. The aortic arch is posterosuperior to the ligament, or located at its back and above it. Lateral to the ligamentum arteriosum is the vagus nerve, which is the 10th of the 12 pairedcranial nerves. For this reason, it is also called cranial nerve X. Also lateral to the vestige is the recurrent laryngeal nerve, or Galen's nerve, that springs from the vagus nerve on the left. The laryngeal nerve hooks around the ligament from the rear where part of the aorta is located, then it goes up to the voice box after which it is named. Anteriorally, or frontally, the ligamentum arteriosum is near the surface of a network of nerves that innervate the heart from its base. The tangle is called the cardiac plexus. Also to the front of the ligamentum arteriosum is the phrenic nerve, which mainly emerges from the fourth cervical nerve (C4).

Causes, incidence, and risk factors PDA affects girls more often than boys. The condition is more common in premature infants and those with neonatal respiratory distress syndrome. Infants with genetic disorders, such as Down syndrome, and whose mothers had rubella during pregnancy are at higher risk for PDA. PDA is common in babies with congenital heart problems, such as hypoplastic left heart syndrome, transposition of the great vessels, and pulmonary stenosis. Pathology The ductus arteriosus originates embryologicaly from the left aortic arch artery therefore it connects the left pulmonary artery to the aortic arch opposite to the left subclavian artery. Patent ductus arteriosus when it closes it usually does so beginning from the pulmonary artery end, leaving a diverticulum in the aortic site which eventually closes. The patent ductus arteriosus goes into the left pulmonary artery even in right aortic arch. Rarely does it originates from the right pulmonary artery. In tetralogy of Fallot the patent ductus arteriosus is usually absent or small. In pulmonary atresia the patent ductus arteriosus is small because in-utero the blood shunts left-to-right in small volumes to the lungs. In premature babies the patent ductus arteriosus closes later than it would in term babies. However, if the prematurity is taken into consideration it closes about the same time. Patent ductus arteriosus closes with high oxygen tension which explains why at high altitude the patent ductus arteriosus remain patent for a longer time. Oxygen concentration above atmospheric level does not assist in closing the patent ductus arteriosus faster then it would naturally. The etiology of higher incidence of patent ductus arteriosus in maternal rubella infection is not clear but it could be due to tissue changes in the ductus arteriosus similar to those observed in right pulmonary artery and left pulmonary artery branches. Indomethicin if given to the mother at pre-term will cause the patent ductus arteriosus to constrict. This is due to interference with the arachidonic derivative causing lack of Prostaglandin. Supplemental oxygen will cause the patent ductus arteriosus to close in full term but not in premature babies. On the other hand,

prostaglandin is better at keeping the patent ductus arteriosus open in premature babies versus full term babies. Polycythemia in patent ductus arteriosus with pulmonary vascular obstructive disease and right-to-left shunting tends to be more so than other cyanotic congenital heart disease. This is thought to be because of increased cyanotic blood flow to the kidneys triggering the mechanism for polycythemia.

Pathopysiology

The murmur in patent ductus arteriosus is characterized as being systolic and diastolic with a crescendo and decrescendo pattern, peaking at around the closure of the aortic valve (A2). Due to the left-to-right shunting at the patent ductus arteriosus the pulmonary venous return will be increased causing dilatation of the left atrium and stretching of the patent foramen ovale with more left-to-right shunting at the atrial level worsening congestive heart failure. The patent ductus arteriosus murmur is best heard over the second left intercostal space. Clicking noises during the murmur gives the characteristic machinery quality of the patent ductus arteriosus murmur. Murmurs mimicking patent ductus arteriosus include AP window, venous hum, ruptured sinus of Valsalva (aorta to RA, RV or LA shunts), coronary artery to ventricular cavity fistula and tetralogy of Fallot with pulmonary atresia and large collaterals. Patients present with congestive heart failure, this include easy fatigability (poor feeding in infants), shortness of breath, pallor sweating and cool extremities with exertion. Signs include bounding pulses, increase left ventricular apical impulse, thrill, continuous murmur and pulmonary sounds consistent with pulmonary edema such as rales and wheezing.

Symptoms A small PDA may not cause any symptoms. However, some infants may have symptoms such as:

Fast breathing Poor feeding habits Rapid pulse Shortness of breath Sweating while feeding

Tiring very easily Poor growth

Signs and tests Babies with PDA often have a heart murmur that can be heard with a stethoscope. However, in premature infants, a heart murmur may not be heard. The health care provider may suspect the condition if the infant has breathing or feeding problems soon after birth. Changes may be seen on chest x-rays. The diagnosis is confirmed with an echocardiogram. Sometimes, a small PDA may not be diagnosed until later in childhood. Treatment If the rest of the baby's heart and blood flow is normal or close to normal, the goal is to close the PDA. If the baby has certain other heart problems or defects, keeping the ductus arteriosus open may be lifesaving. Medicine may be used to stop it from closing. Sometimes, a PDA may close on its own. In premature babies it often closes within the first 2 years of life. In full-term infants, a PDA rarely closes on its own after the first few weeks. When treatment is needed, medications such as indomethacin or a special form of ibuprofen are often the first choice. Medicines can work very well for some newborns, with few side effects. The earlier treatment is given, the more likely it is to succeed. If these measures do not work or can't be used, the baby may need to have a medical procedure. A transcatheter device closure is a procedure that uses a thin, hollow tube placed into a blood vessel. The doctor passes a small metal coil or other blocking device through the catheter to the site of the PDA. This blocks blood flow through the vessel. These coils can help the baby avoid surgery. Surgery may be needed if the catheter procedure does not work or it cannot be used. Surgery involves making a small cut between the ribs to repair the PDA. Surgery has risks, however. Weigh the possible benefits and risks with your health care provider before choosing surgery.

Expectations (prognosis) If a small PDA stays open, the baby may eventually develop heart symptoms. Babies with a larger PDA could develop heart problems such as heart failure, high blood pressure in the arteries of the lungs, or an infection of the inner lining of the heart if the PDA does not close Reference 1. Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 9th ed. Philadelphia, Pa:Saunders Elsevier; 2011:chap 65.