Neonatal Cardiology
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Neonatal Cardiology

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Neonatal Cardiology Neonatal Cardiology Presentation Transcript

  • Diagnosis and Management of the Neonate With Critical Congenital Heart Disease Department of Pediatrics National Naval Medical Center 15 April 03
  • 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
  • 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
  • Normal Fetal Echocardiogram: Four Chamber View
  • Ebstein’s Anomaly
  • 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
  • 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
  • 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
  • Stabilization and Transport
    • PGE 1 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
  • Side effects of PGE 1
    • 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
  • 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)
  • 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
  • Evaluation of Other Organ Systems
    • CNS: CNS anomalies and ischemic injury
    • GI: risk for NEC
    • Renal: 3-6% incidence of urinary tract anomalies
  • 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
  • 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
  • Hypoplastic Left Heart Syndrome
  • 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
  • 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.
  • 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
  • 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.
  • 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%
  • 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
  • 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
  • Hypoplastic Left Heart Syndrome: Fenestrated Fontan Procedure
  • Transplant in Hypoplastic Left Heart Syndrome
    • Issues of waiting for donor heart
    • Excellent operative results
    • Limited donor availability
    • Issues of life long immunosuppresion
  • Coarctation of the Aorta
  • Critical Pulmonary Valve Stenosis
  • Critical Pulmonary Valve Stenosis: Tricuspid Regurgitation
  •  
  • Ebstein Anomaly
  • D-transposition of the Great Arteries
  • Arterial Switch Procedure for D-TGA
  • Total Anomalous Pulmonary Venous Connection
  • Total Anomalous Pulmonary Venous Connection With Obstruction
  • Total Anomalous Pulmonary Venous Connection With Obstruction