Management of cyanotic congenital heart diseae3


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  • Management of cyanotic congenital heart diseae3

    2. 2. 5 “T’s” • m/c cyanotic lesions of the newborn : –TOF –TGA –TA –TAPVC –Tricuspid Atresia
    3. 3. EARLY LIFE-THREATENING PRESENTATION 1.SHOCK 2.CYANOSIS 3.HEART FAILURE • Shock is managed with initial attention to advanced life support such as establishing airway, maintenance of ventilation. • Vascular acess with volume resuscitation. • Inotropic support with the goal of improving cardiac output & tissue perfusion.
    4. 4. CYANOSIS • Normal closure of the PDA in the first days of life can precipitate profound cyanosis in the following scenarios: • When the PDA is the only mechanism of pulmonary blood flow, such as in patients with critically obstructive right heart lesions (eg, critical pulmonary stenosis/atresia,tricuspid atresia.) • When the PDA supplies the majority of systemic circulation in critically obstructive left heart lesions (including hypoplastic left heart syndrome and critical aortic valve stenosis). With ductal closure, these patients will present with decreased peripheral perfusion. • When the PDA provides mixing between parallel pulmonary and systemic circulations (transposition of the great arteries).
    5. 5. PGE1 • The neonate who fails a hyperoxia test is likely to have a PDA dependent systemic or pulmonary circulation should receive PGE1 till anatomical diagnosis is established. • PGE1is started at a dose of 0.05-0.1u/kg/min • May be given upto 0.4u/kg/min • When the desired effect achieved may be reduced to 0.025u/kg/min. • Apnoea may occur .
    6. 6. Time of onset of symptoms in CHD (HART FAILURE)  At birth: HLHS, large A-V fistula, pulmonary atresia  1st wk: TGA, TAPVR  1-4 wk: COA with associated anomalies, critical AS  4-6 wk: endocardial cushion defect 6 wk-4 mth: large VSD, large PDA
    7. 7. General measures: Propped up Oxygen Adequate calories - human milk fortifiers, fat modular products, low osmolarity products Salt restriction Bed rest
    8. 8. Medical management Diuretics - 1st line of drugs - ↓ pre-load - Do not improve CO or myocardial contractility - Hypokalemia and hypochloremic alkalosis Inotropic agents -Digoxin -Dobutamine -Dopamine -Amrinone /milrinone Afterload ↓ agents Dilators: Arteriolar- Veno- Mixed-
    9. 9. How to dizitalize the heart ? 1. Baseline ECG & Serum electrolytes 2. Calculate the oral digoxin dosage : Age Total dizitalizing dose(μg/kg) Maintenance dose(μg/kg/D) Prematures 20 5 Newborns 30 8 < 2yrs 40-50 10-12 > 2yrs 30-40 8-10 Maintenance dose is 25% of the total dig.dose in 2 divided doses I.V. dose is 75% of the oral dose. 3. Give one half of the TDD immediately ,then 1/4th & then the final 1/4th at 6- to 8-hr intervals. 4. Start the maintenance dose 12hrs after the final TDD but before this do ECG
    10. 10. Other ionotropes: Phosphodiesterase inhibitors: Milrinone/amrinone • Low cardiac output refractory to standard therapy • After open heart surgery • Adjunct to DA / Dobutamine • S/E-thrombocytopenia Adrenergic agents: Dopamine • Inotropic,peripheral vesodilatation, increased renal blood flow-natriuresis • 5mcg/kg/min • In higher doses- peripheral vesoconstriction Dobutamine •2.5-40mcg/kg/min •Dose is gradually increased Surgery: (depends on the type of defect)  Blalock Taussig shunt  Balloon septoplasty  Mustard Senning  Jatene’s switch
    11. 11. Late Complications of CCHD • Polycythemia & Hyperviscosity syndrome. • Clubbing. • CNS complications. • Bleeding Disorders. • Hypoxic Spells and Squatting. • Depressed Intelligent Quotient. • Hyperuricemia and Gout. • Failure to thrive • Psychosocial adjustment
    12. 12. SUGGESTED STEPS IN THE MANAGEMENT • Pulse Oximetry. • ABG : confirm or reject central cyanosis. • Hyperoxia test : helps separate cardiac causes of cyanosis from pulmonary. • Chest x-ray : may reveal pulmonary causes of cyanosis and the urgency of the problem. hint at the cardiac defect. • ECG if cardiac origin of cyanosis is suspected. ECHO DOPPLER STUDY
    13. 13. Treatment of complications • Polycythemia& hyperviscosity: symptomatic pt’s (having headache,dizziness,visual disturbances) should have phlebotomy . • Iron replacement. • Bleeding disorder lead to plt Count& function, PT &APTT, lower levels of coagulation factors: plt transfusion,FFP, vit- k, cryoprecipitate can be used.
    14. 14. CNS Complications: high hematocrit or iron deficient RBC’S predispose these pt’s to brain abcess, stroke. Rt to Lt shunt may lead to embolization ,increase chance of infection. Gouty arthritis & Hyperuricemia can be managed with colchicin, probenecid& allopurinol. FOLLOW UP: Should directed towards care of underlying heart condition ,symptoms of hyperviscosity, change in exercise tolerance,saturation level, prophylaxis of IE,influenza, pneumococcus infection. In stable pt’s yearly follow up is recommended & should include vaccination, yearly blood work up(CBC,ferritin,clotting profile,renal function,uricacid.)& regular echo doppler studies .
    15. 15. Peak incidence of 2-4 months Characterized by: Hyperapnea (Rapid and deep respirations) Irritability and prolonged crying Inc cyanosis Decreased heart murmur Hypoxic Spell/ Hypercyanotic Spells/Blue Spells (“TET Spell”) Pathophysiology Lower SVR or inc resistance of RVOT can increase the R-L shunt ↓ Stimulates the respiratory center to produce hyperapnea ↓ Results in an increase in systemic venous return In turn, increases R-L shunt through VSD.
    16. 16. Management: Cyanotic Spell: • Moist O2 inhalation • Knee Chest • Morphin Sulphate 0.1 -0.2mg/kg IM/IV • I.V. Propranolol (0.5 mg/kg) • NaHCo3 : 1 mEq / kg I.V. • Phenylephrine 0.02 mg/kg • Ketamine 1-3 mg/kg I.V. • Gen. Anaesthesia • Emergency Surgical intervention
    17. 17. TOF 1. Non-restrictive VSD 2. RVOT Obstruction 3. RVH 4. Overriding aorta
    18. 18. TOF - treatment MEDICAL : • Recognition & treatment of cyanotic spells. • Ballon dilation of RVOT (used in the past). • IE prophylaxis. • Detection of relative Iron deficiency, iron deficient are more suscetible for CVA
    19. 19. Surgery for TOF • Shunt operation to increase PBF. • Many one prefers primary repair. • Shunts preferred in – 1. Neonates with TOF & PA. 2. Infants with hypoplastic Pulmonary annulus. 3. Children with hypoplastic PA. 4. Medically un-managable hypoxic spells. 5. Infants <2.5kg.
    20. 20. Shunts – various locations Modified ballock-taussing is the preferred one
    21. 21. Definitive repair : • Early surgery generally preferred. • Saturation <75% , occurrence of cyanotic spell considered indication for surgery. • Symptomatic with favourable RVOT & PA have repair after3- 4months. • Mildly cyanotic who had shunt surgery may have repair after 1-2 yrs. • Patch closure of VSD, RVOT widening, PA vulvotomy(avoiding palcement of patch)
    22. 22. TGA Defect to permit mixing of 2 circulations- ASD, VSD, PDA.
    23. 23. Medical : 1. PGE1 – to keep Patent ductus , untill time of surgery. 2. CHF Rx 3. Before surgery, cardiac catheterization and a balloon atrial septostomy (i.e., the Rashkind procedure) often carried out to have some flexibility in planning surgery Surgical : Palliative: Balloon Artrial Septostomy delay in surgery necessary Def: Arterial Switch operation A. Atrial baffle operation – Mustard and Senning operations. B. Ventricular level(VSD +PS) – Rastelli operation(intraventricular tunnel) C. Gr. Art level – (Physiological + Anatomical Correction) Jantene oper done usually in 1st 2wks of life.
    24. 24. Tricuspid Atresia
    25. 25. Tricuspid Atresia – T/t • PGE IV infusion to maintain patent ductus. • Most require a palliative shunt before definitive FONTAN OPERATION. • Blalock-Taussig shunt in infancy – systemic to pulmonary arterial shunt – Provide stable blood flow to the lungs – A gortex tube is sewen between the subclavian artery and the right pulmonary artery
    26. 26. • BIDERECTIONAL GLENN SHUNT : SVC is connected to the RPA ( end to side anastomosis ) • IVC continues to be connected to the heart. • HEMI FONTAN operation : • Modified fontan operation is the definitive choice usually done at 1.5-3 yr – directs the whole systemic venous blood to pulmonary arteries without intervening heart chambers.
    27. 27. Fontan Procedure • Redirects IVC to lungs
    28. 28. Truncus Arteriosus
    29. 29. Management of PTA In1st few wks most of infants can be managed with anticongestive medication;as PVR falls HF worsens & surgery is indicated in1st few months • surgical : various modification of Rastelli operation. • For all types VSD is closed in such a way that LV ejects into truncus.PA are separated from truncus & continuity is established betwn PA &RV.
    30. 30. Total Anomalous Pulmonary Venous Return • The pulmonary veins drain into the RA or its venous tributaries rather than the LA • A interatrial communication (ASD or PFO) is necessary for survival • Pulmonary venous return reaches the RA – Systemic and pulmonary venous blood are completely mixed
    31. 31. 4 Types 1. Supracardiac Common pulmonary vein drains into the SVC via the left SVC and left innominate vein.
    32. 32. 2. Cardiac • The common PV drains into the coronary sinus
    33. 33. 3. Infracardiac • The common PV drains into the portal vein, ductous venosus, hepatic vein, or IVC. 4.Mixedtype :combination of other types
    34. 34. Clinical Signs for Obstructed Veins • Profound desaturation • Acidosis • PGE1 administration does not improve oxygenation because elevated pulmonary pressures in the right side of the heart (due to obstructed pulmonary outflow) will result in right to left shunting across an open ductus further decreasing arterial saturation.
    35. 35. Treatment • Digitalis and diuretics to treat heart failure • Intubation and inc PEEP for those with severe pulm over load • Corrective surgery: pulmonary venous confluence is aanastomosed directly to LA, & ASD is closed • All infants with PV obstruction should be operated soon after diagnosis.If surgery cannot be done immediately ECMO may be required to maintain oxygenation. • With out obstruction, but with HF not managable , operation 4-6 month age
    36. 36. Pulmonary Atresia/VSD Management • Medical: ? PDA dependent – PGE1 • Surgical: – Palliative=shunt – Definitive= staged repair (Unifocalization) – + RV-PA conduit
    37. 37. • RV size? Coronary sinusoids present or not. • PE: • Severe cyanosis/ tachypnea • S2 single • No murmur • CXR: • RAE • Decrease PVM • ECG: • RAE, LVH
    38. 38. Pulmonary atresia with intact septum • Medical : – PGE1 – Radiofrequency perforation of PV. • Surgical: – Open pulmonary valvotomy+ palliative Blallock-shunt – (in the neonatal period). – If good RV size : Biventricular repair (at 1 year of age). – If small RV size ± sinusoids + RVDCC : – BT- shunt in neonatal period – Bidirectinol Glenn (at 6 months of age). – Total Cavo Pulmonary Connection (TCPC) – (at 4 years of age)
    39. 39. Ebstein Anomaly
    40. 40. Ebstein management • Medical : – Mild : nothing – CHF : Diuretics – Severe cyanosis : PGE1 – SVT : adenosine, b-blockers are 1st line preventive drugs. • Surgical : – Palliative shunt – Definitive repair : – If good RV size : biventricular repair ( TV repair ) a) Danielson repair b) Carpentier repair – If small RV----- Univentricular repair strategy
    41. 41. INDICATION of operation
    42. 42. Single Ventricle • ↑Pulm. Blood flow: No PS Lt-→ Rt shunt Like large VSD • ↓Pulm. Blood flow: PS Rt-→Lt shunt Like Fallot
    43. 43. Single Ventricle Management • With ↓pulm. Blood flow: – Medical: PGE1 – Surgical : – - Blalock shunt - Bidirectional Glenn - Total Cavo Pulmonary Connection (TCPC)
    44. 44. Double Outlet RV
    45. 45. Double Outlet RV • ? position of VSD ?PS • Sub – aortic VSD+ no PS: like large VSD CHF • Subaortic VSD+PS: like Fallot • Sub-pulmonic VSD (Tassig-Bing): like TGA • Doubly commited VSD
    46. 46. Thank You