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Congenital

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									                           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


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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




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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




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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




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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



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