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Cyanotic congenital heart disease

Cyanotic congenital heart disease. 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.

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Cyanotic congenital heart disease

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  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. Allows for nearly full saturation of Hb at reduced PO2 Left shift alkalosis, fetal Hb Right shift acidosis, hypercarbia, hyperthermia Oxygen-Hemoglobin Dissociation Curve

  10. Not enough oxygen in Oxygen “mal-absorption” Too much oxygen out Differential Diagnosis of cyanosis

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

  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 UE<LE 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. Ebstein’s 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 Ebstein’s 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

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