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Morning Report

Morning Report. Thursday, August 9 th 2012. Semantic Qualifiers. Illness Script. Predisposing Conditions Age, gender, preceding events (trauma, viral illness, etc), medication use, past medical history (diagnoses, surgeries, etc) Pathophysiological Insult

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Morning Report

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  1. Morning Report Thursday, August 9th 2012

  2. Semantic Qualifiers

  3. Illness Script • Predisposing Conditions • Age, gender, preceding events (trauma, viral illness, etc), medication use, past medical history (diagnoses, surgeries, etc) • Pathophysiological Insult • What is physically happening in the body, organisms involved, etc. • Clinical Manifestations • Signs and symptoms • Labs and imaging

  4. Normal ECHO: 4 chamber

  5. Our patient’s ECHO: 4 chamber

  6. Normal ECHO: long axis

  7. Our patient’s ECHO: long axis

  8. Hypoplastic Left Heart Syndrome (HLHS): Predisposing conditions • Males > Females (1.5:1) • Most commonly occurs sporadically in otherwise healthy individuals • Can be associated with: • Turner’s syndrome (XO), Jacobsen’s syndrome (deletion of distal 11q), trisomy 13, and trisomy 18 • Most common form of single ventricle heart disease • (Prematurity) • (Low birth weight)

  9. HLHS: Pathophysiology • Spectrum of cardiac malformations with normally related great arteries • Includes: • Underdevelopment of the left heart • Significant hypoplasia of the left ventricle • Atresia, stenosis or hypoplasia of the AV and/or MV • Hypoplasia of the ascending aorta and arch • Cause is unknown • ? Intrauterine infarction, infection, selective left ventricular cardiomyopathy, altered blood flow through the left heart

  10. Spectrum of HLHS Most severe    less severe

  11. HLHS: Pathophysiology • Right ventricle supports both pulmonary and systemic circulation • Survival is DEPENDENT on a patent ductusarteriosus (PDA) and a non-restrictive atrialseptall defect (ASD)

  12. HLHS: Clinical Manifestations • At birth: typically no signs/symptoms (due to open ductus, high pulmonary vascular resistance, patent foramen ovale) • Adequately oxygenated systemic blood flow: lungs  LA  PFO  RA  RV  pulmonary artery  PDA  aorta • A restrictive PFO (or ASD) results in early severe cyanosis and acidosis (10% of cases) • As PDA closes and pulmonary vascular resistance lowers: • Decreased systemic perfusion/increased pulmonary perfusion • Can rapidly progress to heart failure and shock

  13. HLHS: Clinical Manifestations • Cyanosis: varying degree • Does not correct with supplemental O2 • No murmur or SEM • Loud S2 (absent aortic valve and pulm HTN) • As heart failure develops**: • Cool extremities, diminished peripheral pulses, hypotension • irritability, tachypnea, retractions, decreased volume of feedings, edema, hepatomegaly, JVD, gallop

  14. Initial Management • KEEP THE PDA OPEN!!!** • Prostin 0.05mcg/kg/min • Watch for apnea, hypotension • Ensure adequate ASD flow • If not adequate  atrialseptoplasty • Treat heart failure • Diuretics, inotropic agents • May need mechanical ventilation

  15. Surgical Management • Staged palliative procedure (preferred) • First stage (as neonate): Norwood • Second stage (at 4-6 months): bidirectional Glenn • Third stage (at18-30months): Fontan • Cardiac transplant (less common)

  16. Thank you!!!Noon Conference: Formula (Dr. Riojas)

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