1 / 17

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

ona
Download Presentation

Morning Report

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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)

More Related