PATENT DUCTUS ARTERIOSUS BASICS DESCRIPTION Patent ductus arteriosus (PDA) is the failure of the ductus arteriosus to close

after birth. 75% of time occurs as isolated defect. System(s) affected: Cardiovascular Genetics: No Mendelian inheritance. 1% chance of PDA in infant if one parent affected. Incidence/Prevalence in USA: 8/1000 live births Predominant age: Infancy Predominant sex: Female > Male (2-3:1) SIGNS & SYMPTOMS Children Failure to grow Recurrent respiratory infections Easy fatigability Dyspnea on exertion Adult Leg fatigue Fatigue Shortness of breath Angina Syncope Signs (left-to-right shunt) Rough systolic murmur Continuous machinery

murmur Thrill at left upper sternal border Bounding pulse with wide pulse pressure Prominent, displaced apical impulse Systolic ejection click Diastolic flow murmur (across mitral valve) Excessive sweating Tachypnea, tachycardia, rales if failure ensues Signs (right-to-left shunt) Cyanosis, especially lower extremities Clubbing Diastolic Graham-Steele murmur (pulmonic insufficiency) Right ventricular heave Polycythemia CAUSES Prematurity Congenital Hypoxia Prostaglandins RISK FACTORS Premature birth High altitudes Maternal rubella

 Coexisting cardiac anomalies Any condition resulting in hypoxia (pulmonary, hematologic, etc.) DIAGNOSIS DIFFERENTIAL DIAGNOSIS Venous hum Total anomalous pulmonary venous return Ruptured sinus of Valsalva Arteriovenous communications Anomalous origin of left coronary artery from pulmonary artery Absence or atresia of pulmonary valve Aortic insufficiency with ventricular septal defect Peripheral pulmonary stenosis (maternal rubella) Truncus arteriosus Aortopulmonary fenestration Coronary artery fistula LABORATORY Arterial blood gas Drugs that may alter lab results: None Disorders that may alter lab results: None PATHOLOGICAL FINDINGS Left ventricular and atrial enlargement Patent ductus may have abnormal intima (maternal rubella) SPECIAL TESTS ECG in children and adults may show left ventricle and left atrial hypertrophy ECG in infants usually normal IMAGING Echocardiography/Doppler Contrast echocardiography Radionuclide angiography Magnetic resonance imaging (MRI) Chest x-ray usually normal in infants Chest x-ray in children and adults (shunt vascularity, calcifications, left ventricle and left atrial enlargement, dilated ascending aorta, dilated pulmonary arteries) DIAGNOSTIC PROCEDURES Cardiac catheterization and angiography - will demonstrate the shunt and determine the amount of shunt, pulmonary pressures, and other coexisting cardiac abnormalities Echocardiography - left atrial enlargement Doppler - displays direction of shunt and size of the patent ductus TREATMENT APPROPRIATE HEALTH CARE Inpatient surgery GENERAL MEASURES Small, asymptomatic shunts may not need closure Pulmonary support Oxygen to correct hypoxia Sodium and fluid restriction Correction of anemia (hematocrit > 45) SURGICAL MEASURES Surgical transection and ligation for moderate/large shunts Transfemoral catheter technique to occlude PDA with foam plastic plug or double umbrella ACTIVITY As tolerated DIET No special diet

PATIENT EDUCATION Discuss prematurity and explain different treatments of premature infants and full-term infants MEDICATIONS DRUG(S) OF CHOICE Ibuprofen 10mg/kg on day 3 of life, 5mg/kg/day for 2 days. Oxygen Diuretics Antibiotic prophylaxis if not surgically repaired Contraindications: To treatment with indomethacin Renal dysfunction Overt bleeding Shock Necrotizing enterocolitis Myocardial ischemia Precautions: With indomethacin treatment - oliguria, hyponatremia Significant possible interactions: Refer to manufacturers profile of each drug ALTERNATIVE DRUGS Indomethacin 0.2-0.25 mg/kg/dose IV preferred. Repeat every 12-24 hours x 3 doses. (Decreased efficacy in term infants; not effective in children or adults.) Alprostadil FOLLOW-UP PATIENT MONITORING Annual, routine follow-up after closure Shunts that have not been closed should be followed more closely PREVENTION/AVOIDANCE N/A POSSIBLE COMPLICATIONS Left heart failure Pulmonary hypertension Right heart hypertrophy and failure Eisenmengers physiology Bacterial endocarditis Myocardial ischemia Necrotizing enterocolitis EXPECTED COURSE/PROGNOSIS Spontaneous closure after 3 months is rare Before 3 months, closure in premature infants is 75% Before 3 months, closure in term infants is 40% Best postoperative results if closed before age 3 years Increased pulmonary vascular resistance and pulmonary hypertension more common if closed after age 3 years No firm statistics but decreased survival for large shunts MISCELLANEOUS ASSOCIATED CONDITIONS Coarctation of the aorta Pulmonary valve stenosis or atresia Peripheral pulmonary stenosis (maternal rubella) Aortic stenosis Ventricular septal defect Necrotizing enterocolitis Club feet, cataracts, blindness, systemic arterial stenosis (associated with maternal rubella)

AGE-RELATED FACTORS Moderate to large shunts usually diagnosed in infancy or childhood. Small shunts occasionally diagnosed in adults. Pediatric: Symptoms and signs depend largely on size of shunt Some infants with coexisting cardiac anomalies benefit temporarily from a patent ductus to provide shunting to the lungs (right heart obstructions) or periphery (coarctation of the aorta). This benefit is short lived, so definitive treatment should proceed as soon as feasible Geriatric: Good results expected with repair age 50-70 years Others: N/A PREGNANCY Women with small to moderate sized ductus and left-toright shunt can expect an uncomplicated pregnancy High risk in those with high pulmonary resistance and right-to-left shunt SYNONYMS Aorticopulmonary shunt Aorticopulmonary communication ICD-9-CM 747.0 Patent ductus arteriosus SEE ALSO OTHER NOTES No need for antibiotic prophylaxis after surgical repair ABBREVIATIONS N/A REFERENCES Adams FH, Emmanouilides GC, Riemenschneider TA: Moss Heart Disease in Infants, Children and Adolescents. 5th Ed. Baltimore, Williams & Wilkins, 1995 Braunwald E, ed. Heart Disease: A Textbook of Cardiovascular Medicine. 5th Ed. Philadelphia, W.B. Saunders Co., 1996 Makowitz JS, et al: Transcatheter versus surgical closure of patent ductus arteriosus. New Engl J Med 1994;330(14);1014 Overneire BV, Smets K, Lecoutere D, et al. A Comparison of Ibuprofen and Indomethacin for Closure of Patent Ductus Arteriosus. N Engl J Med 2000 Sep 7 (to be published)