Transcript Neonatal Cardiology

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

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
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
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 1824 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