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Cardiac Surgical Anomalies in the Neonate

Catherine Bull M.S.N, P.N.P-C Clinical Coordinator, Pediatric and Adult Congenital Cardiac Surgical Services Department of Cardiothoracic Surgery NYU Medical Center. Cardiac Surgical Anomalies in the Neonate. What Is Qp:Qs?. Qp Pulmonary blood flow ~ 1 cup

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Cardiac Surgical Anomalies in the Neonate

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  1. Catherine Bull M.S.N, P.N.P-C Clinical Coordinator, Pediatric and Adult Congenital Cardiac Surgical Services Department of Cardiothoracic Surgery NYU Medical Center Cardiac Surgical Anomalies in the Neonate

  2. What Is Qp:Qs? Qp • Pulmonary blood flow ~ 1 cup Qp:Qs ratio is the amount of blood going to the lungs compared to the amount of blood going to the body. Qp : Qs (LUNGS : BODY) Qp : Qs 1 : 1 Qs • Systemic blood flow ~ 1 cup

  3. Complex Mixing Lesions Blood flow to the body or the lungs is not 100% ductal dependent

  4. Complex Mixing Lesions Transposition of the Great Arteries Total Anomalous Venous return Truncus Arteriosus

  5. Transposition of the Great Arteries NORMAL • TGA : • Aorta arises from the anatomic RV • PA arises from the anatomical LV

  6. D-TGA

  7. D-TGA • Parallel circulations • Mixing can occur at PFO, PDA or VSD • Most mixing occurs at the PFO • Without a mixing: • cyanosis, hypercarbia, tachypnea, tachycardia and acidosis may occur n

  8. D-TGA NORMAL • To increase SaO2 you must increase mixing: • PGE to open duct • BAS • Volume • Oxygen PDAPFO

  9. Balloon AtrialSeptostomy

  10. D-TGA Repair • Arterial Switch • The aorta and the PA are transected and the coronary arteries are removed. • The aorta and the coronary arteries and attached to the neoaortic root • PA is attached to the neopulmonary root

  11. Arterial Switch Operation

  12. ASO: Postop • Decreased LV function • Coronary ischemia • Nitroglycerine/Heparin • Usually a surgical problem • In older patients with IVS whose LV only exposed to pulmonary pressures pre-operatively • Decreased cardiac output • Arrhythmias

  13. TAPVR NORMAL • Failure of pulmonary veins to connect to the to the LA • Blood from both the systemic and pulmonary venous systems return to the RA • the RA, RV & pulmonary arteries enlarge to compensate for the increased volume • An ASD is essential for CO and always present

  14. TAPVR Types NORMAL • Supracardiac: pulmonary veins attach to SVC.

  15. TAPVR Types NORMAL • Cardiac: pulmonary veins attach directly to the heart via RA or coronary sinus

  16. TAPVR Types NORMAL • Infracardiac: pulmonary veins attach below the diaphragm. Prone to obstruction.

  17. TAPVR Supracardiac • Unobstructed: • May be asymptomatic at first • CHF, FTT & frequent upper respiratory infections will occur • Obstructed: • Profound cyanosis within the first few hours of life • Typical “ground glass” CXR • No PGE • Surgical Emergency Infracardiac

  18. Obstructed TAPVR • CXR: • Typical “ground glass” appearance of lung fields • Small heart

  19. Supracardiac TAPVR Repair Attach pulmonary vein confluence to posterior LA and close the ASD Ligatethe vertical vein

  20. Cardiac TAPVR Repair • Create a large ASD and baffle veins from the RA to LA

  21. TAPVR to Coronary Sinus Repair Unroof coronary sinus and baffle pulmonary venous return to the LA

  22. Infracardiac TAPVR Repair • Attach pulmonary vein confluence to posterior LA • Ligatethe vertical vein • Close the ASD

  23. TAPVR Postop • Low cardiac output: • Noncompliant LV. Treated w/ inotropes. • Avoid aggressive volume overload-unresolved LA hypertension & PHTN. • PHTN: • r/o pulmonary venous obstruction. Ventilation, O2, NO & sedation to decrease PVR. • Respiratory failure: • Due to obstructed veins preop and resultant pulmonary vascular congestion. • Treated with mechanical ventilation, paralysis, sedation, PEEP & possibly ECMO. • Re obstruction • occurs in 10% of patients-usually obstructed infracardiac type

  24. Truncus NORMAL • CHF, mild cyanosis & FFT within the first month of life. • Can develop pulmonary hypertension by 3 months.

  25. Truncus Repair • The pulmonary arteries are excised from the truncus • RV to PA conduit placed • VSD is closed in a manner in which the truncal valve recieves blood from the left ventricle

  26. Truncus: Postop • PHTN: • preop overcirculation results in PA pressures=>Paralyze, sedate, O2 and NO. • Low CO: • RV dysfunction=> volume (need a high CVP), inotropes & vasodilators. • Cyanosis: • RL PFO =>will resolve with RV function.

  27. Right Sided Heart Lesions Pulmonary blood flow is ductal dependant

  28. Right Sided Heart Lesions Regular Pulmonary Atresia Real Pulmonary Atresia Pulmonary Atresia w/ MAPCA’s Tetralogy of Fallot Pulmonary Atresia w/ Intact Ventricular Septum Tricuspid Atresia

  29. Tetrology of Fallot • TOF is characterized by 4 cardiac anomalies: • Ventricular septal defect • Pulmonary stenosis or pulmonary atresia/right ventricular outflow tract obstruction (RVOTO) • Overriding aorta • Right ventricular hypertrophy

  30. TOF NORMAL • Hemodynamics depends on the amount of PS and the size of the VSD • Severe PS: Cyanotic • RL shunt • Qp<Qs • Mild PS: Pink Tet • LR shunt • Qp>Qs • CHF

  31. TOF Repair • Patch closure of VSD • Relieve RVOTO • Resect muscle below the valve • Enlarge the pulmonary artery above the valve OR • transannular patch with removal of valve

  32. TOF: Transannular Patch

  33. TOF: Post-op • RV dysfunction • Inotropes • May have pleural effusions (esp. right) and ascites • Junctional Eptopic Tachycardia • prevention • keep HR low with cooling, no chronotropic drugs, sedation • Amiodarone • Cyanosis • due to right to left shunt across PFO if present • Pulmonary insufficiency • all patients with transannular patch • Residual VSD • not well tolerated • Residual RVOTO • well tolerated

  34. TOF w/ PA • Pulmonary valve & main pulmonary artery are atretic • Pulmonary blood flow is supplied by • PDA (most common) • multiple aortopulmonary collateral arteries (MAPCAs) TOF/PA TOF/PA w/ MAPAC’s

  35. TOF/PA Repair • Blalock-Taussig Shunt followed by full repair later in infancy

  36. PA w/ IVS: Anatomy NORMAL • Pulmonary valve atresia with no VSD • Hypoplastic RV (Variable) • Size of the RV is determined by the size of the TV • High RV pressure • RV sinusoids (Variable) • May form due to high RV pressure. • Steal coronary blood flow from CA

  37. PA w/ IVS: Anatomy RV Sinusoids

  38. PA w/ IVS • Need to do 2 things: establish pulmonary blood flow and get the RV to grow • Pulmonary valve balloon angioplasty • Works best when the leaflets of valve are only fused • BTS • Transannular patch

  39. PA w/ IVS • 2 Ventricle Repair (Adequate RV without sinusoids) • Balloon angioplasty • +/- BTS • 1 ½ Ventricle Repair (Borderline RV +/-sinusoids) • +/- Balloon angioplasty • BTS • +/- Transannular patch • Single Ventricle Repair (Inadequate RV +/- sinusoids) • BTS

  40. PA w/ IVS 1 ½ Ventricle Repair • Borderline RV +/-sinusoids: • BTS & transannular patch to allow pulmonary insufficiencyand subsequent RV growth. • Adequate RV growth: complete repair • Inadequate RV growth: Bidirectional Glenn procedure (1 ½ ventricle repair)

  41. Tricuspid Atresia NORMAL • The tricuspid valve is absent w/ no communication between the RA and RV • Results in RV and PA hypoplasia and a single left ventricle

  42. Tricuspid Atresia Types • No VSD, PA, hypoplastic RV • Pulmonary blood flow is ductal dependant • Small VSD, hypoplastic PV, hypoplastic RV (most common) • Pulmonary blood flow is ductal dependant • Large VSD, small to adequate PV & RV • Qp:Qs is variable

  43. Tricuspid Atresia • Cyanosis, hypoxemia and metabolic acidosis usually occur within the first few days of life if pulmonary blood flow is not adequate and the PDA closes. Qp<Qs • Management strategies should be aimed at balancing pulmonary and systemic blood flow to maintain Qp=Qs.

  44. Tricuspid Atresia • Blalock Taussig Shunt • Pallative shunt between the right innominate artery and the RPA that provides pulmonary blood flow. • Variable Qp:Qs

  45. Left Sided Heart Lesions Blood flow to the body is ductal dependant

  46. Left Sided Heart Lesions Coarctation of the Aorta Interrupted Aortic Arch Hypoplastic Left Heart Syndrome

  47. Interrupted Aortic Arch NORMAL • Systemic blood flow is ductal dependant • Blood to the upper body comes from the LV & aorta: pre-ductalsats are higher • Blood to the lower part of the body comes from the PA and PDA: post-ductalsats are lower • Decreased peripheral perfusion & metabolic acidosis if duct closes

  48. IAA Types • Type A= away • Type B • Type C= close

  49. IAA Repair • End-to-end anastomosis w/ PDA ligation

  50. IAA: Postop • Pulmonary hypertension • Issues related to DiGeorge Syndrome • Recurrent laryngeal nerve palsy • Pherenic nerve damage • Recurrent stenosis at site of repair

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