Download Congenital

RSPT 2353 – Neonatal/Pediatric Cardiopulmonary Care
Congenital Disorders
Lecture Notes
Reference & Reading: Czverinske Chapter 29 & 31
I. Congenital Anomalies – Pulmonary involvement
a. Diaphragmatic Hernia
 More common on left than right
 Symptoms:
1. Polyhydramnios
2. Cyanosis, respiratory distress
3. Bowel sounds in chest
4. Flat abdomen, scaphoid chest
 Diagnosis
1. Usually prenatal
2. CXR – bowel in chest
3. Depending on degree of herniation there may be a presence of
lung hypoplasia
 Treatment
1. Immediate intubation at birth
2. Placement of NG or OG tube
3. Ventilate patient as necessary
4. UAC & UVC need to be placed
b. Tracheoesophageal Anomalies (TE fistula) – Atresia of the upper
esophagus, usually includes a fistula between the lower esophageal tube &
trachea (most common Fig 29-2 (A)).
 Diagnosis - Suspicions usually arise with:
1. Accumulation of oral secretions
2. Sporadic or continuous respiratory distress
3. Repeated regurgitation
 Treatment – surgical repair
1. Keep bed at 30 degree angle
2. Post-op
c. Omphalocele
 Diagnosis – prenatal
 Treatment
d. Gastroschisis
 Diagnosis – prenatal
 Treatment –
1. Contents during delivery are placed in a protective sac
2. Pedi surgeons will place a silo on defect and reduce until patient
is ready for surgery
3. Patient may or may not require respiratory intervention
4. Post-op
II. Congenital anomalies – Cardiac defects
1
a. Affect about 1 in 100 deliveries. Signs, Symptoms and treatment depend on the
type & degree of defect.
b. Patent Ductus Arteriosus (PDA)
 Pathophysiology –
1. Pulmonary edema
2. With  amount of blood shunting through PDA hypoperfusion
occurs to all postductal organsNEC, and other disorders
3. Keeping the PDA can be important in some heart defects
 Diagnosis
1. Loud grade I or II murmur
2. Echocardiogram with color mapping can indicate presence and
direction of blood flow
3. Pre/post ductal:
a. R-to-L
b. L-to-R
 Treatment
1. Usually self resolve
2. Indomethacin
3. Surgical ligation
c. Atrial Septal Defect (ASD)
 Diagnosis – usually undected
 ASD is desirable in certain situations
d. Ventricular Septal Defect (VSD)
 Diagnosed
 Treatment is usually withheld unless:
1. Failure to thrive
2. CHF that doesn’t respond to tx
2
e. Tetralogy of Fallot
 4 defects:
1. VSD
2. overriding aorta
3. hypertrophy of the RV
4. obstruction to flow through PA
 Cyanosis is a result of  blood flow through PA
 CXR
 Diagnosed by ultrasound
 Cardiac Cathetarization
 Treatment
f.
Transposition of the Great Vessels (TGV) aka TGA
 Aorta arises from R ventricle, PA arises from L ventricle
 Shunt dependant
 Mixing of blood occurs via an ASD, PDA or VSD
 Pt often show signs of CHF
 Diagnosis
 Treatment
1. Balloon septostomy
2. dissection of vessels and placement in correct ventricle
3
g. Coarctation of Aorta
 Constriction of aorta restricts blood flow
 Signs are related to  CO
 Diagnosis
1. If suspected
2. Definitive
 Treatment:
1. Surgical repair
2. PGE
h. Anomalous Venous Return (TAPVR)
 Return of oxygenated blood to R atrium instead of left
 ASD must be present to survive
 Diagnosis: cardiac cathetarization
 Treatment:
1. Immediate cardiac catheterization with balloon septostomy
2. Surgical repair involves placement of pulmonary veins into left
atria
4
i.
Truncus Arteriosus – One large vessel arises from both R & L ventricles with a
large VSD
 Cyanosis usually present, depends on degree of pulmonary perfusion
 Diagnosis
 Treatment
j.
Hypoplastic Heart (Left or Right)
 Right hypoplastic heart
1. non PDA dependent, avoid fluid overload
2. better survival
 Left hypoplastic heart – involves several anomolies
1. coarc, hypoplastic L ventricle, aortic and/or mitral valve stenosis
or atresia
2. Duct dependant
3. Treatment:
a. 2 stage surgical repair: Norwood, Glenn, Fontan
b. Heart transplant
5