Approach
• Consider Dx in pts w/ sudden onset cyanosis, hypoxemia, &/or shock, typically in the 1st 1–2 wk of life, though some pts present weeks to years later
• Differentiate cyanotic vs. noncyanotic, & ductal vs. nonductal dependent congenital heart dz
• Hyperoxygenation test: Compare ABG on RA & on 100% O2 for 10 min, PO2 of >250 excludes hypoxia 2/2 congenital heart dz
• Give PGE1 to any pt w/ suspected ductal-dependent lesion & circulatory compromise
Definition
• Cyanotic lesions: Congenital cardiac dz w/ right-to-left shunt
• Ductal dependent lesions: Congenital cardiac dz in which fetal life depends on a PDA, either from impaired systemic or pulmonary blood flow
History
• Cyanosis, fussy baby, poor feeding
Findings
• ↓ O2 sat, cyanosis, ↓ BP, cardiac murmur, hepatomegaly, check 4-extremity BPs
Evaluation
• ABG, response to O2, CXR, ECG, echo
Treatment
• O2, consider PGE1 (alprostadil): 0.05–0.1 μg/kg/min if ductal-dependent lesion suspected, side effects: Bradycardia, hyperthermia, hypotension & apnea
• Inotropic support w/ milrinone, dopamine, or dobutamine & intubation prn
Disposition
• Cardiology consult, ±cardiac surgery consult, admit
Pearls
• Pts w/ ductal-dependent lesions p/w circulatory failure, usually during 1–2 wk of life
• Acyanotic lesions may p/w CHF
TETRALOGY OF FALLOT
Approach
• Recognize/tx Tet spells
Definition
• PA stenosis, VSD, RV hypertrophy, & deviation of aortic origin to the right (overriding); degree of severity dictated by degree of RV outflow tract obstruction
History
• Presentation usually w/i 1st few years of life, though occ. into adulthood
• Cyanosis (often during feeds), ↓ PO intake, agitation, ↑ RR; ↑ sxs w/ exercise, szs, CVA
• “Tet” spell: Infundibulum spasm → ↑ RV outflow obstruction → cyanosis, respiratory distress
Findings
• ↓ O2 sat, systolic ejection murmur, cyanosis, squatting pt
Evaluation
• See above, ECG (RAD, RVH, RAE, RBBB), CXR (boot-shaped heart), CBC, VBG
Treatment
• See above, 100% O2, calm child, bring knees to chest; consider morphine; correct hypovolemia, hypoglycemia, consider propranolol, phenylephrine, intubation
Disposition
• Cardiology, cardiac surgery consult, admit
Pearl
• Onset determined by slowly ↑ infundibulum hypertrophy → ↑ RV outflow tract obstruction → ↑ RV hypertrophy → ↑ right-to-left shunt; thus presentations at later age have poorer long-term outcomes