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39 week/M by PA with note of occasional arrhythmia NICU Rotation SGD . 07 January 2009 Interns Belandres, Bombase, D. Chan, Chu, Francisco Circumstances of Delivery Born Full Term 37 1/7 weeks by LMP 39 weeks by PA 2500g AGA Cephalic via primary LSCS due to NRFS PRENATAL: Maternal Profile (+) 9 x PNCU’s c/o a midwife’s clinic (-) cough/colds, HTN, BA, PTB, DM/GDM, PROM, alcohol intake, hep B, drugs, smoking Course of Labor, Resuscitation 14 hours PTC, (+) watery vaginal d/c with irregular mild uterine contractions. On admission, FHT noted to be 124/min, regularly irregular. On delivery, thermoregulation, suctioning, tactile stimulation done/given (-) meconium staining Resuscitation Further thermoregulation, suctioning, tactile stimulation done/given; 30 secs Good cry, tone and color noted. HR 130s, RR 40, Apgar 9. 4 mins supportive care HR 130s, RR 40. Apgar 9. Occasional arrhythmia noted. O2 sats taken: 98-99% Post-natal course Roomed-in, monitored Persistent arrhythmia noted Admitted to NICU 2 Course in the NICU Day 1 Was received awake, active, comfortable, HR 122-134 irregular, RR 50-60, O2Sat 98%. AP, DHS, (-) murmurs, good pulses hooked to cardiac monitor (+) occ’l PVC’s noted thermoregulation done, feeding with EBM started, 3xBM noted, good UO A>> r/o CHD, PDA Course in the NICU Day 2 Feeding increased (-) murmurs on auscultation Good cry, activity Day 3-5 (-) PVC’s on monitor (-) murmurs good cry/activity. Course in the NICU Day 6 still (-) PVC’s 15-L ECG done>> (N) good suck noted sent home Discharge PE pink conjunctivate, anicteric sclerae, (-) molding/caput, anterior fontanelle open and soft, (-) overlap of sutures, (-) anterior neck mass, (-) CLAD, head circumference 33cm equal chest expansion, (-) alar flaring, (-) retractions, clear breath sounds, chest circumference 31.4cm adynamic precordium, (-) heaves/thrills, normal rate/regular rhythm, (-) murmurs appreciated Discharge PE slightly globular soft abdomen, nonpalpable liver edge, (-) masses, umbilical cord stump clean and dry penile shaft straight, testes (B) descended peripheral pulses full and equal, pink color, skin with good moisture and turgor, patent DUCTUS arteriosus the ductus arteriosus shunts right ventricular outflow to the distal aorta facilitating bypass of the lungs by 90% of the outflow; lung arterial circulation is still vasoconstricted perfuses lower part of the fetal body (upper part, including coronaries and cerebral perfused by LV output) Effectively a RL shunt * If RV output (flow to the lungs) is compromised by other (congenital) heart disease, can be a critical, essential shunt the ductus arteriosus Effectively a RL shunt * If RV output (flow to the lungs) is compromised by other (congenital) heart disease, can be a critical, essential shunt Medial layer with circularly arranged smooth muscle Patency = low oxygen tension + endogenously produced prostaglandins (maternal NSAID use can close it during fetal life and compromise fetal CV function) bifurcation of the pulmonary artery aorta just distal to the left subclavian artery closure of the ductus At birth, changes in pressure gradient favors RV output lungs Pulmonary vascular pressure DEcreases with lung expansion Systemic vascular resistance INcreases with loss of placental circulation (which is low resistance) (systemic > pulmonary) Flow in the ductus becomes L R High arterial PO2 constricts the ductus closes over days closure of the ductus Functionally complete in ½ of neonates by the 24th hour of life (10th to 18th hour) 100% by the 4th day of life PATENT ductus arteriosus note again… Medial layer with circularly arranged smooth muscle Patency = low oxygen tension + endogenously produced prostaglandins PERSISTENT PATENCY … in term infants: deficient muscular and endothelial layers Persistence beyond 4 days usually will be unresponsive to pharmacologic therapy … in PRE term infants: hypoxia and immaturity Risk Factors Infant Prematurity Chromosomal abnormalities (Down) Females : Males 2:1 Hypoxia Asphyxia Coexistence with other congenital heart diseases (facilitates survival) Right side outflow stenosis or atresia (TOF, Tricuspid or Pulmonary atresia) Aortic coarctation TGA or TAPVR RDS/surfactant therapy Maternal Rubella infection early in the pregnancy Pathophysiology Consequences of a L to R shunt Hypoxia and Congestive Heart Failure Later in life, increase in pulmonary pressure pulmonary hypertension scarring and poor O2 exchange increasing pressure of R side/circulation R to L shunt = Eisenmengerization Severity, significance related to size and ratio of pulmonary : systemic vascular resistance Smaller and lower ratio = less Signs and Symptoms Patient may be cyanotic, with bounding peripheral pulses and wide pulse pressure in diastole, blood runs off pulmonary artery Murmur “machinery,” “rolling thunder” after onset of 1st sound wanes in late diastole 2nd L ICS, sternal border, infraclavicular areas Increases and becomes more continuous with decreasing pulmonary vascular resistance With increasing PVR, diastolic component wanes Heart Size / Dynamic Precordium / Heaves Increasing, with increasing PDA size 2nd L interspace thrill radiating to the L clavicle Diagnostics ECG NORMAL with small LR shunt LVH, Bi-VH if large CXR pulmonary artery prominence Increasing cardiac size with greater LR shunting 2D Echo larger LA and LV dimensions Retrograde turbulent flow in the PA on doppler, as well as retrograde flow in the aortic during diastole Visualization on Cardiac Catheterization Therapeutics MEDICAL Prostaglandin inhibition (10-14 days of life, more for premature infants), intravenous agents Indomethacin 0.1 to 0.25 mg/kg q12 for 3 doses or Ibuprofen 10 mg/kg IV for first dose, then 5 mg/kg IV for second and third doses after 24 and 48 h, respectively Fluid restriction theoretically, to decrease fluid conveyed by the shunt and the load on the pulmonary circulation evidence not fully supportive Optimum oxygenation (support) Therapeutics CARDIAC CATHETERIZATION Occlusion with coils (2.5-3mm) Even partial occlusion can lead to closure thrombus formation further reduces the shunt SURGICAL Ligation; approach: thoracotomy I: Congestive heart failure, failure of medical therapy (note also risk of bacterial endocarditis) Low-risk, but complications involve nerve and vessel ligations thank you.