diagnosis and early management of the infant with suspected congenital heart disease
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Diagnosis and Early Management of the Infant with Suspected Congenital Heart Disease. Introduction. Congenital heart disease occurs in 1% of live-born infants Almost 1/2 of all cases of congenital heart disease are diagnosed during the 1st week of life - PowerPoint PPT PresentationTRANSCRIPT
Diagnosis and Early Management of the Infant with
Suspected Congenital Heart Disease
Introduction
• Congenital heart disease occurs in 1% of live-born infants
• Almost 1/2 of all cases of congenital heart disease are diagnosed during the 1st week of life
• The most frequently occuring anomalies seen during the 1st week are: PDA, D-transposition of the great arteries, hypoplastic left heart syndrome, TOF, and pulmonary atresia
Indications for Fetal Echocardiography
Maternal Risk Factors Associated With Congenital Heart Disease
• Congenital heart disease
• Cardiac teratogen exposure– Lithium
– Amphetamines
– Alcohol
– Anticonvulsants: phenytoin, valproic acid, carbamazepine, and trimethadione
– Isotretinoin
Maternal Metabolic Disorders or Infection
• Diabetes mellitus
• PKU
• Hyperthyroidism
• Lupus, collagen vascular disease
• Rubella, CMV, Coxsackie, Parvovirus
Fetal Risk Factors Associated With Congenital Heart Disease
• Trisomies, Turner’s syndrome, abnormal karyotype
• Congenital malformations: duodenal atresia, TEF, omphalocele, diaphragmatic hernia, renal dysgenesis, and hydrocephalus
• Fetal arrhythmias• IUGR• Nonimmune hydrops• ?2 vessel cord
Cyanosis• Etiology: CV, pulmonary, airway obstruction,
neurological, neuromuscular, or hematological (methemoglobinemia or polycythemia)
• Infants can appear cyanotic when the deoxygenated Hgb concentration is at least 3g/dL; it is not related to the percent saturated
• 2 babies with sats of 80%: one with a hgb of 20g/dL and 4g/dL of desaturated hgb will be cyanotic, but an anemic infant with 10g/dL with 2g/dL deoxygenated hgb will not be cyanotic
Evaluation• ABC’s• PE: murmur, pulses, precordium, respiratory
status, HSM, color, capillary refill• 4 ext BPs: if SBP >10mmHg in right hand
compared to lower ext, concerning for arch anomaly (though if normal may not rule it out)
• Pre/post ductal saturations: if see a difference >5%, concerning for PPHN or left heart abnormalities
Evaluation (Continued)• Hyperoxia test: baseline pre-ductal ABG when
infant in room air, then repeat on 100% FiO2• Reason for ABG and not just sats: with a saturation
of 100%, you can have a PaO2 of 80 or 300; very different
• CXR: cardiomegaly; normal, increased, or decreased pulmonary vascularity
• EKG• Echo
Interpretation of hyperoxia test: From Harriet Lane Handbook FiO2= 0.21 PaO2 FiO2 =1.00 PaO2 PaCO2 (%sats) (%sats) Normal 70 (95%) >200 (100%) 35 Pulmonary Dz 50 (85%) >150 (100%) 50 Neurologic Dz 50 (85%) >150 (100%) 50 Methemoglobinemia 70 (95%) >200 (100%) 35 Cardiac Dz Separate circulation (TGA no VSD) <40 (<75%) <50 (85%) 35 Restricted PBF ( TA +PS, PA, PS + no VSD, TOF) < 40 (<75%) <50 (<85) 35 Complete mix no restricted PBF 50 (85%) <150 (<100%) 35 (Truncus, TAPVR, Single Vent,
TGA +VSD, TA no PA or PS) PPHN Preductal Post ductal PFO no R->L shunt 70 (95%) <40 (<75%) Variable 35-50 PFO + R->L shunt <40 (<75%) <40 (75%) Variable 35-50
Specific Heart Disease Abnormalities
Cyanotic With Decreased Pulmonary Blood Flow
• Tetrology of Fallot
• Ebsteins Anomaly
• Tricuspid Atresia with PA or PS
• Pulmonary atresia with intact septum
• Critical pulmonic stenosis
• PPHN
Right Sided Obstructive Lesions
• Cyanosis• No respiratory distress• Normal pulses and perfusion• Single second heart sound• Murmur• Moderate to marked hypoxemia• CXR: normal to large sized heart, decreased
pulmonary blood flow (PBF)
Tetralogy of Fallot
Tetrology of Fallot
Ebstein’s Anomaly
Ebstein’s Anomaly
Tricuspid Atresia
Tricuspid Atresia
EKG : QRS axis
•Tricuspid atresia with PS or PA with intact ventricular septum: superior (0— -90)•Critical PS or PA : 0 to 90 degree quadrant•TOF and TOF with PA: 90-180 degree quadrant
Cyanotic With Increased Pulmonary Blood Flow
• d-Transposition of the great vessels• Truncus arteriosus• Total anomalous pulmonary venous return,
above diaphragm• Single ventricle• Endocardial cushion defect
Inadequate Mixing Lesions
• Cyanosis• Mild tachypnea• Normal pulses• Single heart sound• Murmur• ABG: marked hypoxemia, + acidosis• CXR: cardiomegaly, normal or increased
PBF
Transposition of the Great Arteries
d - Transposition of the Great Vessels
Truncus Arteriosus
Truncus Arteriosus
Lesions with Poor Gas Exchange
• Cyanosis
• Marked tachypnea
• Fair perfusion, normal pulses
• May or may not have a single heart sound
• May or may not have a murmur
• CXR: normal heart size, pulmonary congestion
Total Anomalous Pulmonary Venous Return
Total Anomalous Pulmonary Venous Return
Left Sided Obstructive Lesions
• Coarctation of aorta, interrupted aortic arch
• Hypoplastic left heart syndrome
• Aortic stenosis
• Mitral stenosis
• Total anomalous pulmonary venous return, below diaphragm
Left Sided Obstructive Lesions
• Grey or ashen color• Tachypnea• Poor perfusion• Decreased pulses/differential pulses• Single second heart sound• Murmur + gallop• Hepatomegaly• ABG: metabolic acidosis• CXR: cardiomegaly with increased PBF
Coarctation of the Aorta
Hypoplastic Left Heart Syndrome
Hypoplastic Left Heart Syndrome
Aortic Stenosis
Acyanotic With Increased Pulmonary Blood Flow
• VSD
• ASD
• PDA
• Endocardial cushion defect
Ventricular Septal Defect
Ventricular Septal Defect
Atrial Septal Defect
Atrioventricular Canal
Patent Ductus Arteriosus
Initial Stabilization
• ABC’s: Volume resuscitation, ionotorpic support, correction of metabolic acidosis, r/o sepsis
• Intubate if needed, titrate Fi02 to keep Sp02 80%-85% to prevent pulmonary overcirculation
• Placement of umbilical lines• Infants who present in shock within the first 3
weeks of life, consider ductal dependent lesions• Use of PGE1 (0.025 to 0.1mcg/kg/min)
Stabilization for Transport
• Reliable vascular access• Intubation if on PGE1, OG placement• Oxygen delivery, Sp02• Monitor HR, tissue perfusion, blood
pressure, and acid-base status• Calcium and glucose status (increased risk
for DiGeorge)
Prostaglandin E1
• Failure to respond: diagnosis incorrect, older infant with unresponsive ductus, ductus absent, obstructed pulmonary venous return
• Clinical deterioration after PGE1: obstructed blood flow out of pulmonary veins or left atrium, HLHS with restrictive FO, TGA with intact ventricular septum and restrictive FO, obstructed TAPVR, mitral atresia with restrictive FO
PGE 1 - Side Effects• Common: Apnea, fever, leukocytosis,
cutaneous flushing, and bradycardia.
• Uncommon: seizures, hypoventilation, hypotension, tachycardia, cardiac arrest, sepsis, diarrhea, DIC, fever
• Rare: urticaria, bronchospasm, hemorrhage*, hypoglycemia, and hypocalcemia
*inhibits platelet aggregation