Cyanotic congenital heart disease

1
Cyanotic congenital heart disease
2
Case Presentation

• Term male infant delivered by spontaneous vaginal
  delivery and appears cyanotic at birth
• respiratory rate 70 bpm, baby has grunting and
  nasal flaring with chest retractions
• Heart murmur on exam
• ABG pH 7.32 PaCO2 45 PaO2 35

3
Case Presentation

• What is happening?
• Have you seen this problem?
• What is causing her problem?
• What can we do about it?

4
Cyanosis defined

• Bluish discoloration of skin or mucous membranes
• Presence of 5g/dL of deoxyhemoglobin
• Low flow areas with increased oxygen extraction
  have more deoxyhemoglobin
• High flow areas with less extraction should not
  have enough deoxyhemoglobin to appear cyanotic
• Under normal circumstances you should not be able
  to extract enough O2 to have 5 g/dL
  deoxyhemoglobin running through the tongue and
  gums (lips OK when cold)

5
Cyanosis Peripheral v. Central

• Peripheral
• Response to cool temperatures
• part of normal transition
• may last 72hr
• May also represent poor cardiac output
• poor perfusion
• anemia
• Central
• Multiple causes

6
Cyanosis
7
Cyanosis

• Cyanosis is dependent on HCT and Sat
• Florescent light makes cyanosis hard to see.
• Except in the extreme, cyanosis is not obvious
• Look at the the tongue and the gums
• Any question, check a pulse ox

8
Terms

• Oxygen tension (PO2)
• Partial pressure of oxygen in the blood (mm Hg)
• Measured on an ABG machine
• Oxygen dissolved in plasma
• 0.003 ml O2/mm Hg/dl plasma
• Oxygen saturation (SO2)
• Amount of oxygen actually combined with
  hemoglobin as a percent of total oxygen that
  could be bound to hemoglobin
• Measured by saturation monitor (pulse-oximeter)
• 1.34ml O2/g Hb

9
Oxygen-Hemoglobin Dissociation Curve

• Allows for nearly full saturation of Hb at
  reduced PO2
• Left shift
• alkalosis, fetal Hb
• Right shift
• acidosis, hypercarbia, hyperthermia

10
Differential Diagnosis of cyanosis

• Not enough oxygen in
• Oxygen mal-absorption
• Too much oxygen out

11
Not enough oxygen in

• Apnea
• neurologic and drugs
• Diffusion barrier
• RDS, aspiration, pneumonia
• Obstruction
• pneumothorax, head position

12
Oxygen mal-absorption

• Shunting lesions
• cardiac
• non-cardiac (like PPHN)
• Hematologic
• methemoglobinemia
• carboxyhemoglobinemia

13
Too much oxygen out

• High oxygen consumption
• sepsis
• low flow, high extraction
• acrocyanosis
• hyperviscosity/polycythemia
• extravasated (e.g. bruising)

14
Neonatal Circulation

• Baby separated from placenta
• Baby breathes and lungs expand
• Closure of ductus arteriosus and foramen ovale

15
Persistent Pulmonary Hypertension

• High resistance in pulmonary vessels
• Persistent R ? L shunts
• ductal
• atrial
• intra-pulmonary
• Poor heart function

16
The Five Ts of Cyanotic Congenital Heart Disease

• Transposition of the great arteries
• Tetralogy of Fallot
• Truncus arteriosus
• Total Anomalous Pulmonary Venous Return
• Tricuspid Atresia

17
Normal Anatomy

• normal

18
Tetralogy of Fallot (TOF)

• Overriding Aorta
• Ventricular septal defect
• Pulmonary stenosis
• Right ventricular hypertrophy

19
Tetralogy of Fallot

• Boot shape
• RVH lifting apex
• loss of PA knob

20
Fallots with pulmonary atresia

• Blue. Will need a systemic- pulmonary shunt.

21
Transposition of Great Arteries - no PPHN

• Comfortably tachypneic (usually big) child with
  oxygen saturation 50-6- on room air or oxygen

22
Transposed Great Arteries

• Blue. Presents with cyanosis when the duct closes.

23
Transposition of Great Arteries

• Egg on a string
• alignment of PA and Ao narrows the mediastinum

24
Total Anomalous Pulmonary Venous Return (TAPVR)

• Type 1 (supracardiac)
• 50 with snowman
• can have UEltLE saturation
• Type 2 (cardiac)
• Type 3 (infracardiac)
• all can obstruct, infracardiac almost always does

25
Totally anomalous pulmonary venous drainage
(infradiaphragmatic)

• All four pulmonary veins drain to the right side.
  Below the diaphragm they are always obstructed.
  Infant presents in first days with cyanosis,
  circulatory and respiratory failure and collapse.

26
Total Anomalous Pulmonary Venous Return

• Snowman

27
Ebsteins anomaly

• The tricuspid valve is abnormal and inserts well
  down into the RV. There is often severe trisuspid
  regurgitation, which can lead to death in the
  fetus or infant. Usually also with ASD so
  right-to-left flow results in cyanosis.

28
Wall to wall heart Ebsteins anomaly
29
DiGeorge syndrome

• Thymic aplasia (T-cell, immune problems)
• Hypocalcaemia (hypothyroid gland aplasia,
  seizures)
• Dysmorphism (unusual facial appearance)
• Outflow tract abnormalities in the heart

30
Tricuspid Atresia

• DiGeorge syndrome with low calcium seen in 1/3 of
  the cases

31
(No Transcript)