Diagnosis and Management of the Neonate With Critical Congenital Heart Disease - PowerPoint PPT Presentation

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Diagnosis and Management of the Neonate With Critical Congenital Heart Disease
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Diagnosis and Management of the Neonate With Critical Congenital Heart Disease

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  1. Diagnosis and Management of the Neonate With Critical Congenital Heart Disease Department of Pediatrics National Naval Medical Center 15 April 03

  2. Neonate With Critical CHD • Prenatal evaluation • Initial neonatal evaluation and management • Stabilization and transport • Confirmation of the diagnosis • Preoperative evaluation of non-cardiac organ systems • Timing and type of surgery • Lesion specific management

  3. Prenatal Assessment • Obstetric history • Genetic evaluations • Prenatal ultrasound • Fetal echocardiography • 60% of cardiology admissions at CHOP prenatally diagnosed • 49% of HLHS admissions at Children’s Hospital of Wisconsin were prenatally diagnosed

  4. Normal Fetal Echocardiogram: Four Chamber View

  5. Ebstein’s Anomaly

  6. Critical CHD: Initial Evaluation and Management • ABC’s • Oxygen (judicial) to saturations of 80-85% • Place umbilical lines • PGE (0.025-0.1 micrograms/Kg/min) • History • Complete physical with four extremity BP’s • Pre and post-ductal oxygen saturations • Hyperoxia test • CXR • EKG • Echocardiogram

  7. Suspected CHD: Initial Evaluation and Management • Pre and post-ductal oxygen saturations • If pre-ductal sat higher than post-ductal sat (differential cyanosis) • Left heart abnormalities (such as aortic arch hypoplasia, critical aortic stenosis, interrupted aortic arch) • Persistent pulmonary hypertension • If post-ductal sat higher than pre-ductal (reverse differential cyanosis) • TGA with CoA or TGA with IAA • TGA with supersystemic pulmonary vascular resistance

  8. Stabilization and Transport • ABC’s • Place lines (UVC, UAC) • Check and administer glucose and calcium as needed • If severe respiratory distress, shock, or severe cyanosis: sedate, paralyze, intubate, and mechanically ventilate to oxygen sats of 80-85%. Place NG tube. • Check ABG’s • Sepsis evaluation. Antibiotics

  9. Stabilization and Transport • PGE1 lowest dose possible (0.025 micrograms/kg/min) • Judicious use of pressors • Dopamine and Dobutamine • Milrinone • Consider use of 2-3% CO2 in ventilated patients with left sided obstructive lesions

  10. Side effects of PGE1 • More common in premature infants • Clinical deterioration if pulmonary venous obstruction present • HLHS with restrictive/intact atrial septum • TGA with intact ventricular septum and a restrictive/intact atrial septum • TAPVR with obstruction • Apnea • Hypotension

  11. Confirmation of the Diagnosis • Echocardiography • primary diagnostic modality for anatomic definition • Not “non-invasive” in sick newborn • Cardiac catheterization • Rarely indicated to confirm diagnosis • Therapeutic (interventional procedures)

  12. Genetic Evaluation • Genetic • Trisomies 13, 18, 21 • Monosomy X (Turner’s syndrome): Coarctation • 22q11 Deletion (DiGeorge syndrome): Conotruncal abnormalities • 7q11 Deletion (Williams syndrome) • Single gene defects (Noonan’s, Holt-Oram, Ellis-van Crevald, Alagille) • Unknown cause • Vacterl • Charge

  13. Evaluation of Other Organ Systems • CNS: CNS anomalies and ischemic injury • GI: risk for NEC • Renal: 3-6% incidence of urinary tract anomalies

  14. Timing and Type of Surgery • Cardiac catheterization procedures • Balloon atrial septostomy • Balloon valvuloplasty • Balloon angioplasty • Open versus Closed • Palliative versus Corrective • Trend towards early, corrective surgery, even in preterm or low birth weight infants

  15. Critical CHD: Lesion Specific Management • Ductal dependent for systemic blood flow • HLHS management • Ductal dependent for pulmonary blood flow • D-transposition of the great arteries • Total anomalous pulmonary venous connection with obstruction

  16. Hypoplastic Left Heart Syndrome

  17. Hypoplastic Left Heart Syndrome: • Pathology: aortic atresia/severe stenosis, mitral atresia/severe stenosis, hypoplastic left ventricle and aortic arch. • 1.5% of congenital heart defects. Most common cause of cardiac related neonatal mortality. • Ductal dependent for systemic blood flow at birth • Patients may have associated chromosomal or developmental abnormalities

  18. Hypoplastic Left Heart Syndrome: Clinical Presentation • May be diagnosed by fetal ultrasound. Prognostic issues: atrial septal position, size of foramen ovale (if restrictive, pulmonary venous obstruction) • Classic presentation: cardiogenic shock, poor perfusion, decreased pulses, profound metabolic acidosis. May have systolic murmur. • Diagnosis: echocardiogram. CXR and EKG are non-specific.

  19. Hypoplastic Left Heart Syndrome: Initial Medical Management • Prostaglandin E1 0.025 to 0.2 micrograms/kg/min- watch for side effects • Room air ventilation: ideal ABG ph 7.4/ pco2 40/ po2 40 • Inhaled CO2 to manipulate pulmonary vascular resistance? • Watch the use of pressors- may be harmful

  20. Hypoplastic Left Heart Syndrome: Stage One Norwood • Performed in neonatal period • Procedure: MPA divided; distally MPA closed with patch; hypoplastic aortic arch reconstructed and anastomosed to the proximal MPA with homograft augmentation; atrial septosomy; systemic shunt placed.

  21. Hypoplastic Left Heart Syndrome: S/P Stage One Norwood • Surgical issues: • Unobstructed aortic arch • Adequate atrial septectomy • Balanced pulmonary and systemic blood flow (Qp:Qs 1:1) • Survival at major centers: 80%

  22. Hypoplastic Left Heart Syndrome: HemiFontan Procedure • Shunt ligated, superior vena cava anastomosis to pulmonary artery, pulmonary arteries augmented, flap of tissue closes SVC-RA junction • Performed around 6 months of age following Norwood • Volume load on right ventricle removed • Excellent survival statistics

  23. Hypoplastic Left Heart Syndrome: Fontan Procedure • Performed around 18-24 months • Venous and systemic circulations are separated • Survival: excellent • Long term issues: RV function, arrhythmias

  24. Hypoplastic Left Heart Syndrome: Fenestrated Fontan Procedure

  25. Transplant in Hypoplastic Left Heart Syndrome • Issues of waiting for donor heart • Excellent operative results • Limited donor availability • Issues of life long immunosuppresion

  26. Coarctation of the Aorta

  27. Critical Pulmonary Valve Stenosis

  28. Critical Pulmonary Valve Stenosis: Tricuspid Regurgitation

  29. Ebstein Anomaly

  30. D-transposition of the Great Arteries

  31. Arterial Switch Procedure for D-TGA

  32. Total Anomalous Pulmonary Venous Connection

  33. Total Anomalous Pulmonary Venous Connection With Obstruction

  34. Total Anomalous Pulmonary Venous Connection With Obstruction