Amjad Kouatli MD. FAAP. FACC. Consultant Pediatric Cardiologist King Faisal Specialist Hospital and Research Center Jeddah Anatomy of Ductus Arteriosus Large channel found in mammalian fetuses.
Connects the main pulmonary artery to descending aorta.
Its media consists mainly of smooth muscles compared to elastic fibres in MPA, DAO. Carries 60% of combined vent. output
Diverts blood from high resistance pulmonary circulation to low resistance descending aorta and placental circulation.
PGE 1 and PGI 2 formed intramurally and in placenta maintain ductal patency in fetal life Physiology of Ductus Arteriosus Functional closure In 12 hr., contraction of medial smooth muscles due to PO 2 & PGE 1 .
Anatomical closure In 3 wk., replacement of muscle fibres with fibrosis creating ligamentum arteriosus.
Silent without hemodynamic compromise to either pulmonary or systemic circulation Post Natal Closure of PDA Before Birth After Birth If ductal closure causes significant decrease in systemic circulation, the condition is called ductus dependent systemic blood flow
If ductal closure causes significant decrease in pulmonary circulation, the condition is called ductus dependent pulmonary blood flow DUCTUS ARTERIOSUS DEPENDENT SYSTEMIC BLOOD FLOW Lesions characterized by the entire or part of the systemic blood flow depends solely on the patency of the ductus arteriosus.
Coarctation of Aorta (severe) Interrupted Aortic Arch Hypoplastic Left Heart Critical Coarctation IAA between LCC, LSC Interrupted Aortic Arch Hypoplastic Left Heart Clinical Presentation Normal birth weight and initial examination
Symptoms start suddenly when the duct closes.
40% are symptomatic first 2 days of life.
Tachypnea, dyspnea, grunting, flaring, ashen colour, and cyanosis.
Hyperactive precordium, tachycardia, hypotension, weak or absent femoral pulses, single S2, systolic heart murmur. Investigations Laboratory Metabolic acidosis, hypoglycemia hyperkalemia.
ECG: RAD, RVH
Chest X ray: mild cardiomegaly mild to severe increased pulmonary vascular markings.
Aortic Atresia Hypoplastic LV Echocardiogram Interrupted Aortic Arch Echocardiogram Critical Coarctation DUCTUS ARTERIOSUS DEPENDENT PULMONARY BLOOD FLOW Hypoxic lesions characterized by the pulmonary blood flow depends solely on the patency of the ductus arteriosus.
Pulmonary atresia Severe pulmonary stenosis TOF with severe pulmonary stenosis TOF Critical PS Pulmonary Atresia Clinical Presentation Normal birth weight and initial examination
Symptoms start suddenly when the duct closes.
Progressive cyanosis, tachypnea.
Hyperactive precordium, tachycardia, single S2, TR systolic murmur, normal pulses. Investigations Laboratory hypoxemia not responding to O 2
hypocarbia (hyperventilation).
ECG: LAD, LVH .
Chest X ray; mild to severe cardiomegaly, decrease in pulmonary vascular markings. Pulmonary Atresia Intact ventricular septum Hypoplastic RV Echocardiogram Critical Pulmonary Stenosis Treatment Prostaglandin E 1
IV via a large vein, 0.05- 0.1 ug/kg/min
Side effects: apnea 10%, hypotension, inhibition of platelets aggregation, fever, diarrhea, flushing, bradycardia, seizures, arrhythmia.
Therapeutic response is judged Femoral pulses restoration, pH in DDSBF Resolved cyanosis in DDPBF Treatment cont. Correction of metabolic acidosis with sodium bicarbonate, acidosis decreases actin and myosin coupling.
Correction of hypoglycemia and hypothermia.
Surgical or transcatheter intervention:
Palliation: Complete correction: Norwood Hybrid HLHS BT Shunt PDA Stent PA-IVS RV injection, AP view Critical PS, Severe TR RV hypertrophy RV injection, lateral view PV annulus 4.4 mm Effective opening < 1 mm Treatment cont. Cardiac Catheterization For balloon valvuloplasty in critical PS 0.014 wire crossed Pulmonary valve Balloon inflated Waist is visible Balloon inflated Waist disappeared Pre Balloon dilatation Decreased pulmonary blood flow RA dilatation After Balloon dilatation More blood to lungs Decreased RA size Treatment cont. Oxygenation, Ventilation Oxygen and hyperventilation are excellent pulmonary vasodilator leading to decrease pulmonary vascular resistance.
Hypo oxygenate and hypo ventilate in ductus dependent systemic blood flow.
Oxygenate and ventilate in ductus dependent pulmonary blood flow. PVR = SVR PVR SVR 10 5 2.5 2.5 5 5 60% 100% 80 % 2 60% Effect of oxygen on pulmonary and systemic circulation. Oxygen decreases PVR 10 2 4 4 2 8 60% 100% 92 % 2.5 5 100% 5 Summary Ductus dependent congenital heart disease should be considered in every newborn presenting with shock or cyanosis physical examination shows hyperactive precordium, heart murmur, or weak pulses
Prognosis improves dramatically with early diagnosis, early infusion of Prostaglandin and early understanding of the disease physiology