0
Good afternoon
SEMINAR ON CYANOTIC  HEART DISEASES            Presented by:               B.Kalpana        M.Sc (N) 2nd yeara
EMBRYOLOGY   OF  HEART
LOCATION OF CARDIOGENIC AREA                        19 days                      1. Neural plate                      2. P...
Formation of endocardial tubes -20 days                                                            Formation of           ...
FORMATION OF CARDIAC LOOP -23 DAYS
BENDING OF PRIMITIVE HEART TUBE-            24 DAYS
VENTRICULAR LOOPING    END WEEK 4            NORMAL : Loop            to the RIGHT:            Levocardia!            ABNO...
FROM TUBE TO FOUR CHAMBERS      INTERNAL VIEW
ENDOCARDIAL CUSHION: 80         DAYS
HEART FORMATION CON’T Weeks  5-8   SVC, IVC form   Right and Left Atrium divide   Ventricles start to form   Aorta an...
TRANSITION FROM THE FETALCIRCULATION    Pulmonary vascular resistance falls    Ductus venosus and ductus arteriosus     ...
CYANOTIC HEART DISEASE Cyanotic   heart disease is a heart defect, present at birth (congenital), that results in low blo...
CYANOSIS IN CHILDREN   Central cyanosis:    Cyanosis of the tongue,mucous membranes and      peripheral skin, it is neces...
CAUSES OF CENTRAL CYANOSISB) LUNG DISEASE             D) CNS DEPRESSIONa)   RDS                     a) IVHb)   Pneumonia  ...
RADIOLOGICAL FEATURES   CXR may exclude non cardiac causes of cyanosis e.g. RDS. .    Meconium aspiration, Diaphgramatic ...
AUSCULTATIONHEART SOUNDS:    S1 is normally accentuated in newborns    S2 split is normally heard as a slurring rather t...
MURMURS   SYSTOLIC EJECTION MURMURS    May be heard in the first hours of life.     Usually due to ventricular obstructio...
Examination of CVS in the newborn1.Look for cyanosis and dysmorphology
Feel thepulsesespeciallybrachialandfemoral
Look at the respiratory pattern and for         evidence of recession
Feel the precordium for     hyperactivity     and for thrills
Locatetheapexbeat
Feel forhepatomegally
Listencarefully
Do what is necessary tocalm the baby down!
Listen at the back for radiation of murmurs
3 months old- severe failure to thrive
Special tests-echocardiography
PULMONARY VEINS - COLOUR DOPPLER                         Subcostal view                          Apical view
TETRALOGY OF   FALLOT
TETRALOGY OF FALLOT (TOF) Named   by Etienne-Louis Arthur Fallot in 1888 Approximately 10% of all complex CHD Single de...
DEFINITION   Tetralogy of Fallot is the result of unequal division of    the conus. Four alterations result:     Narrowe...
FOUR MALFORMATIONS IN TOF                   A.Pulmonic stenosis                   B.Overriding aorta                   C.V...
CAUSES Unknown Methylene  tetrahydrofolate reductase  gene (Portugal study) Maternal rubella during pregnancy Down syn...
SIGNS AND SYMPTOMSCyanosis-blue color to the skin, which gets worse when the baby is upset
 Squating position
Clubbing   of fingers
CONT…………..Failure  to gain weightPoor developmentDifficulty in feeding
PATHOPHYSIOLOGY   Pulmonary stenosisA  narrowing of the right ventricular outflow  tract and occur at the pulmonary valv...
OVERRIDING OF AORTA   An aortic valve with biventricular connection,    that is, it is situated above the ventricular sep...
   The aortic root can be displaced toward    the front (anteriorly) or directly above the    septal defect, but it is al...
VENTRICULAR SEPTAL DEFECT A hole between the two bottom chambers  (ventricles) of the heart. The defect is centered arou...
RIGHT VENTRICULAR HYPERTROPHY The  right ventricle is more muscular than  normal, causing a characteristic boot-  shaped ...
ADDITIONAL ANAMOLIES stenosis of the left pulmonary artery, 40% of patients a bicuspid pulmonary valve, 40% of patients...
DIAGNOSIS OF TETRALOGY OF FALLOT History –cyanosis,feeding Physical examination:       Single accent. S2       ESM   ...
CONTD… Echocardiography-ductus   arteriosus,  VSD,ASD MRI- belineation of Aorta, RVOT,VSD  RV hypertrophy Cardiac cathe...
SOME PICTURES OF TETRALOGY OF FALLOT
CYANOTIC OR TET SPELLAnxietyOver sweating (hyperventilation)Sudden increase in cyanosis
CYANOTIC SPELLSSpasm of        decrease SVR           cryingRVOT                 Increase R…..L shuntingIncrease systemic ...
CYANOTIC SPELLS   Increase systemic vascular resistance                                                Squat/Knee chest p...
TOF     MANAGEMENT Medical   :   Correct iron deficiency anemia   Correct polycythemia   B-Blocker   Analgesics-to re...
FACTORS THAT INCREASE THE RISK FORSURGERY Low birth weight Pulmonary artery atresia Major associated anomalies Multipl...
CONTRAINDICATIONS FOR SURGERY The presence of an anomalous coronary artery Very low birth weight Small pulmonary arteri...
POTTS SHUNT   A side to side anastomosis of pulmonary artery with    aorta is created   The Potts shunt has been abandon...
 It consists of constructing a shunt between the  ascending aorta and right pulmonary artery The Waterston shunt is some...
SURGICAL MANAGEMENT Surgical:     Palliative = Blalock-Taussig shunt       for small PA’s     Definitive= Total correct...
GOAL OF PALLIATIVE SURGERY   The goals of palliation for tetralogy of Fallot (TOF)    are to increase pulmonary blood flo...
ADVANTAGES OF MODIFIEDB-T SHUNT preservation of the subclavian artery, suitability for use on either side, good relief ...
CORRECTIVE SURGERY Primary correction is the ideal operation for treatment of tetralogy of Fallot (TOF) and is usually pe...
CORRECTIVE SURGERY Before     cardiopulmonary     bypass    is initiated, previously placed systemic-to- pulmonary artery...
SURGICAL COMPLICATIONS   Early postoperative complications following repair    of tetralogy of Fallot (TOF) include the c...
TRANSPOSITION OF GREAT VESSELS The   hallmark of tranposition of great arteries is ventriculoarterial discordance, in whi...
TRANSPOSITION OF THE        GREAT ARTERIES (TGA) Complete TGA or D-TGA   Embryological inversion of the great    arterie...
CAUSESUnknownAbnormal  persistence of the subaorticconus with resorption or underdevelopment of the subpulmonaryconus(in...
PATHOPHYSIOLOGY Atrialseptal defect Ventricular septal defect Patent ductus arteriosus
DIAGNOSIS OF TGA Transposition of the great arteries with intact  ventricular septum: Prominent and progressive  cyanosis...
CONTD… Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction:...
MEDICATIONSInotropic    agents Digoxin Loop diuretics Prostaglandins-alprostadil
SURGICAL CAREArterial switch procedure  It represents an repair and           establishes        ventriculoarterial       ...
TGA MANAGEMENT Medical:     PGE1     O2 (3L/minute)     Correct :       acidosis ,hypoglycemia.       electrolyte di...
SURGICAL REPAIR Balloon  atrial septostomy   Developed by Rashkind (1965)   Enlarges the atrial communication Atrial s...
SURGICAL REPAIR Arterial   switch     Performed first by Jatene (1976)     Great arteries transected and reattached to ...
   Atrial Switch There are two types of atrial switch    operations - the MUSTARD operation, and the    SENNING operation...
 AtrialSwitch Using pericardium (Mustard) or  flaps created from the atrial septum and wall  (Senning), a "baffle" is con...
 Definitive Procedures Rastelli  procedure. Pulmo artery transected distal to its  valve and proximal end is oversewn.  ...
EBSTEIN’S ANAMOLY  Congenital defect Origins of  septal or posterior leaflets, or  both, are displaced downward  into RV L...
EBSTEIN’S ANOMALY The   tricuspid valve is abnormal and inserts well down into the RV. There is often severe trisuspid re...
Physical ExaminationCyanosis and clubbing - Varying degrees ofcyanosis at various times in life and transientworsening wit...
Arterial pulses Usually normal Diminished volumeHeart sounds First heart sound widely split with loud tricuspid component ...
ADDITIONAL HEART SOUNDS AND MURMURS     Third and fourth heart sounds commonly  present, even in the absence of congestiv...
Chest  X-Ray: Normal findings Cardiomegaly ( Rounded or Box- like contour ) Small aortic root and main pulmonary artery s...
Wall to wallheartEbstein’sanomaly
12-LEAD EKG: Rhythm -Usually normal sinus findings Intermittently SVT, paroxysmal SVT, atrial flutter, atrial fibrillation...
ECG from a patient with Ebsteinsanomaly showing huge P waves andlow amplitude QRS waves. RBBB and Twave inversion are not ...
 Echocardiogram:Echocardiogram standard for diagnosis.M-mode Paradoxical septal motion Dilated right ventricle Delayed...
CONTD…   Two-dimensional Apical  displacement of the septal leaflet of  greater than 8 mm/m 2 – Abnormalities in morpho...
Doppler   studies Varying degrees of tricuspid regurgitation Excludes associated shunts
ASSESSMENT OF SEVERITY AND SURGICAL       OPTIONS BY ECHOCARDIOGRAPHY Functional   right ventricular area less  than 35% ...
CONTD… Magnitude   of leaflet deformity and  dysplasia Aneurysmal dilatation of right  ventricular outflow tract (right ...
   Cardiac Catheterization:Cardiac Catheterization Rarely performed today Confirms echocardiographic findings Can reveal ...
 Complications: Congestive heart failure Sudden cardiac death Bacterial endocarditis Brain abscess Paradoxical embol...
MEDICAL MANAGEMENT Antibiotic prophylaxis for infective endocarditis Medical therapy for heart failure - Angiotensin-  c...
   Factors associated with lower likelihood    of success include the following:    Accessory pathways located along the...
   Indications for surgery are generally as follows:      New York Heart Association (NYHA) class I-II       heart failu...
SURGICAL MANAGEMENT Approaches: Approaches Tricuspid valve repair is preferred over  valve replacement Bioprosthetic val...
 Repair of TV & Closure of ASD: Repair of TV & Closure of ASD Usual preparation  for operation & anaesthesia CPB Two ven...
 Palliative           procedures include creation of atrial septal defect, closure of tricuspid valve with plication of t...
TOTAL  ANAMOLOUS  PULMONARYVENOUS RETURN
TAPVRDefinition    -No direct communication exists between     the      pulmonary veins and the LA.    -Instead, they dra...
TOTALLY ANOMALOUS PULMONARYVENOUS DRAINAGE(INFRADIAPHRAGMATIC)   Allfour pulmonary veins    drain to the right side.    B...
Pathophysiology1)Mixing of oxygenated and deoxygenated blood before or at  the level of the right atrium (total mixing les...
CLINICAL MANIFESTATION1)Severe obstruction to pulmonary venous return,   -In neonatal period: Cyanosis and severe tachypne...
SUPRACARDIACTAPVD
DIAGNOSISX-RAY            -
In most cases without obstruction       :the heart is enlarged,   the pulmonary artery and right    ventricle are prominen...
ECHOCARDIOGRAPHY: ESSENTIAL FOR DIAGNOSISA large right ventricle :the pattern of abnormal pulmonary venous connections.A v...
TREATMENT   Medical1.Intensive anticongestive measures with digitalis and diuretics ->in patient without pulmonary venous...
NATURAL HISTORY1.CHF occurs in both types of TAPVR with  growth retardation and repeated  pneumonias2.Without surgical rep...
TRICUSPID ATRESIA
Types of tricuspid atresia Type 1       Type2        Type 3• The        • The        • The  great        great        grea...
Diagnosis of Tricuspidatresia    History      Physical examination        Investigations
CyanosisGrowth  retardationNasal flaringMuscle retraction*Brain abscess and bacterial endocarditis- headache, seizures...
 Cyanosis                           P Digital clubbing JVP distension                     H                            ...
INVESTIGATIONS Lab    studies-        Chest X-rayCBP-polycythemia   Cardiomegaly,right                    atrial enlarge...
CARDIAC CATHETERIZATIONStastusof ductus arteriosusASD if present ATRIAL SEPTOSTOMY
Infants with               Infants with decreased                increased      Palliative pulmonary blood flow           ...
SURGICAL CARE Blalock-Taussig   ShuntDefinition: A subclavian artery to pulmonary artery anastamosis.Advantages: Predicta...
   Disadvantages:    Inadequate flow is possible, stenosis of the    anastomosis is possible due to increased tension on ...
GLENN PROCEDURE   Definition:                   Advantages:    A superior vena cava           Pulmonary hypertension    ...
GLENN PROCEDUREDisadvantages:                       Uses:  Cyanosis may increase by            Used to bypass the right  ...
FONTAN OPERATION The  right atrium is connected to the pulmonary artery directly. Used when it is not possible to have a ...
INTERRUPTEDAORTIC ARCH
InterruptedAortic Arch
TYPES OFINTERRUPTEDAORTIC ARCH
Type ATYPE BTYPE C
TYPE A IAAThe arch interruption occurs distal to the origin of the left       subclavian artery.
TYPE B IAAThe interruption occursdistal to the origin of the  left common carotid           artery.
TYPE C IAAThe interruption occursproximal to the origin ofthe left common carotid         artery
PATHOPHYSIOLOGY With  an interrupted aortic arch, venous  blood returning to the heart goes to the  lungs and returns to ...
CONTD… Arteries   that come off the Aorta distal to the obstruction get deoxygenated blood from the right ventricle. This...
INTERRUPTED AORTICARCH/COARCTATION Heart failure/collapse
RASTELLI OPERATION  An example of a repair of  complex CHD with two  ventricles. This one had a  big VSD, TGA and PS.  Th...
SEVERAL    PARAMETERS SHOULD BE METTO ENSURE A SUCCESSFUL OUTCOME.    The candidate should be aged 4 years or     older. ...
Nursing management ofclient with cyanotic heart          disease
Helping family memebers to adjust           Proiding pre operative care           Preoperative assessmentNursing      Preo...
NURSING DIAGNOSISImpaired   gas exchange R/T altered pulmonary blood flowAltered cardiac output R/T specific anatomic de...
CONT………. Altered nutrition : Less than body  requirement R/T excessive energy  demands Increased Potential for infection...
USEFUL HINTS Large male baby with rapid, shallow abdominal breathing:                             D-TGA Upper body blue,...
Thank you
Cyanotic heart diseases
Cyanotic heart diseases
Cyanotic heart diseases
Cyanotic heart diseases
Cyanotic heart diseases
Cyanotic heart diseases
Cyanotic heart diseases
Cyanotic heart diseases
Cyanotic heart diseases
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Cyanotic heart diseases

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Transcript of "Cyanotic heart diseases"

  1. 1. Good afternoon
  2. 2. SEMINAR ON CYANOTIC HEART DISEASES Presented by: B.Kalpana M.Sc (N) 2nd yeara
  3. 3. EMBRYOLOGY OF HEART
  4. 4. LOCATION OF CARDIOGENIC AREA 19 days 1. Neural plate 2. Primitive node 3. Primitive streak 4. Cut edge of amnion 5. Angiogenetic cell clusters
  5. 5. Formation of endocardial tubes -20 days Formation of primitive tube-21 days Development of regions in primitive heart Tube -22 days
  6. 6. FORMATION OF CARDIAC LOOP -23 DAYS
  7. 7. BENDING OF PRIMITIVE HEART TUBE- 24 DAYS
  8. 8. VENTRICULAR LOOPING END WEEK 4 NORMAL : Loop to the RIGHT: Levocardia! ABNORMAL: Loop to the LEFT: Dextrocardia!
  9. 9. FROM TUBE TO FOUR CHAMBERS INTERNAL VIEW
  10. 10. ENDOCARDIAL CUSHION: 80 DAYS
  11. 11. HEART FORMATION CON’T Weeks 5-8  SVC, IVC form  Right and Left Atrium divide  Ventricles start to form  Aorta and pulmonary arteries Weeks 8-12  Ventricles and mitral and tricuspid valves  Aorta and pulmonary artery, aortic arch Week 12:Fetal Circulation begins
  12. 12. TRANSITION FROM THE FETALCIRCULATION  Pulmonary vascular resistance falls  Ductus venosus and ductus arteriosus close  Right-to-left shunting through foramen ovale ceases Timing of these events determines the timing of presentation of congenital heart defects
  13. 13. CYANOTIC HEART DISEASE Cyanotic heart disease is a heart defect, present at birth (congenital), that results in low blood oxygen levels. There may be more than one defect.
  14. 14. CYANOSIS IN CHILDREN Central cyanosis: Cyanosis of the tongue,mucous membranes and peripheral skin, it is necessary to have >3g/dl of reduced Hb to have it. Peripheral cyanosis: It is visible only in the skin of the extremities with normal arterial saturation due to vasomotor instability,ex. cold environment.
  15. 15. CAUSES OF CENTRAL CYANOSISB) LUNG DISEASE D) CNS DEPRESSIONa) RDS a) IVHb) Pneumonia b) Perinatal asphyxiac) Pneumothorax c) Heavy maternal sedationd) Pleural effusione) Diaphragmatic herniaf) T.E.FistulaC) PERSISTENT PULMONARY E) MISCELLANOUS HYPERTENSION a) shock & sepsis b) Hypoglycemia c) Methemoglobinemia d) Neuromuscular conditions ( Werdnig – Hoffman)
  16. 16. RADIOLOGICAL FEATURES CXR may exclude non cardiac causes of cyanosis e.g. RDS. . Meconium aspiration, Diaphgramatic hernia, Pneumothorax Pulmonary Vascular Markings Decreased Increased Heart Size Heart Size Normal Increased Increased( “Boot shaped”) (“ Wall-to-Wall”) TOF Ebstein (“ egg-on-end”) D-TGA Aortic Arch Mediastinum Abdominal Situs
  17. 17. AUSCULTATIONHEART SOUNDS:  S1 is normally accentuated in newborns  S2 split is normally heard as a slurring rather than a distinct split.  S2 is single in many cyanotic lesions especially in, D-TGA, TOF.  S2 is widely split in TAPVR, Critical PS.
  18. 18. MURMURS SYSTOLIC EJECTION MURMURS May be heard in the first hours of life. Usually due to ventricular obstruction e.g. AS., PS., TOF DIASTOLIC MURMURS: Rarely heard in newborns Early diastolic murmurs heard in Truncus arteriosus , TOF with absent pulmonary valve Continuous: Continuous murmurs are caused by AV fistulas (not PDA) ABSENT: Silent hearts often characteristic of Tricuspid atresia , Pulmonary atresia & D-TGA.
  19. 19. Examination of CVS in the newborn1.Look for cyanosis and dysmorphology
  20. 20. Feel thepulsesespeciallybrachialandfemoral
  21. 21. Look at the respiratory pattern and for evidence of recession
  22. 22. Feel the precordium for hyperactivity and for thrills
  23. 23. Locatetheapexbeat
  24. 24. Feel forhepatomegally
  25. 25. Listencarefully
  26. 26. Do what is necessary tocalm the baby down!
  27. 27. Listen at the back for radiation of murmurs
  28. 28. 3 months old- severe failure to thrive
  29. 29. Special tests-echocardiography
  30. 30. PULMONARY VEINS - COLOUR DOPPLER Subcostal view Apical view
  31. 31. TETRALOGY OF FALLOT
  32. 32. TETRALOGY OF FALLOT (TOF) Named by Etienne-Louis Arthur Fallot in 1888 Approximately 10% of all complex CHD Single developmental error of the terminal portion of the spiral truncoconal septum Four distinct components: subpulmonic stenosis, VSD, overriding aorta, and RV hypertrophy Often accompanied by other anomalies
  33. 33. DEFINITION Tetralogy of Fallot is the result of unequal division of the conus. Four alterations result:  Narrower right ventricle outflow region  Defect in the interventricular septum  An aorta that overrides directly above the septal defect  The right ventricular wall hypertrophies.
  34. 34. FOUR MALFORMATIONS IN TOF A.Pulmonic stenosis B.Overriding aorta C.Ventricular septal defect (VSD) D.Right ventricular hypertrophy
  35. 35. CAUSES Unknown Methylene tetrahydrofolate reductase gene (Portugal study) Maternal rubella during pregnancy Down syndrome CATCH 22(cardiac defects, thymic hypoplasia,cleft palate, hypocalcemia) Right ventricular out flow tract obstruction Deletion of chromosome band 22q11
  36. 36. SIGNS AND SYMPTOMSCyanosis-blue color to the skin, which gets worse when the baby is upset
  37. 37.  Squating position
  38. 38. Clubbing of fingers
  39. 39. CONT…………..Failure to gain weightPoor developmentDifficulty in feeding
  40. 40. PATHOPHYSIOLOGY Pulmonary stenosisA narrowing of the right ventricular outflow tract and occur at the pulmonary valve (valvular stenosis) or just below the pulmonary valve (infundibular stenosis). Caused by overgrowth of the heart muscle wall (hypertrophy of the septo parietal trabeculae). These events finally leads to overriding of aorta.
  41. 41. OVERRIDING OF AORTA An aortic valve with biventricular connection, that is, it is situated above the ventricular septal defect and connected to both the right and the left ventricle. The degree to which the aorta is attached to the right ventricle is referred to as its degree of "override."
  42. 42.  The aortic root can be displaced toward the front (anteriorly) or directly above the septal defect, but it is always abnormally located to the right of the root of the pulmonary artery.
  43. 43. VENTRICULAR SEPTAL DEFECT A hole between the two bottom chambers (ventricles) of the heart. The defect is centered around the most superior aspect of the ventricular septum (the outlet septum), and in the majority of cases is single and large. In some cases thickening of the septum (septal hypertrophy) can narrow the margins of the defect.[
  44. 44. RIGHT VENTRICULAR HYPERTROPHY The right ventricle is more muscular than normal, causing a characteristic boot- shaped (coeur-en-sabot) appearance as seen by chest X-ray. Due to the misarrangement of the external ventricular septum, the right ventricular wall increases in size to deal with the increased obstruction to the right outflow tract. This feature is now generally agreed to be a secondary anomaly, as the level of hypertrophy generally increases with age.
  45. 45. ADDITIONAL ANAMOLIES stenosis of the left pulmonary artery, 40% of patients a bicuspid pulmonary valve, 40% of patients right-sided aortic arch, 25% of patients coronary artery anomalies, 10% of patients a foramen ovale or atrial septal defect, in which case the syndrome is sometimes called a pentalogy of Fallot, atrioventricular septal defectpartially or totally anomalous pulmonary venous returnforked ribs and scoliosis.
  46. 46. DIAGNOSIS OF TETRALOGY OF FALLOT History –cyanosis,feeding Physical examination:  Single accent. S2  ESM CXR: boot shaped heart (coeur en sabot) ECG: RVH Hematocrit-elevated ABG and Oximetry
  47. 47. CONTD… Echocardiography-ductus arteriosus, VSD,ASD MRI- belineation of Aorta, RVOT,VSD RV hypertrophy Cardiac catheterization: -assessment of pulmonary annulus size&pulmonary artery -RVOT,VSD -coronary artery abnormalities
  48. 48. SOME PICTURES OF TETRALOGY OF FALLOT
  49. 49. CYANOTIC OR TET SPELLAnxietyOver sweating (hyperventilation)Sudden increase in cyanosis
  50. 50. CYANOTIC SPELLSSpasm of decrease SVR cryingRVOT Increase R…..L shuntingIncrease systemic venous return DecreaseO2 Increase CO2 Decrease pH Tachypneea
  51. 51. CYANOTIC SPELLS Increase systemic vascular resistance Squat/Knee chest position Ketamine 1-2mg/kg IV Neosynephrine 0.02mg/kg IV Tachycardia Propranolol 0.1mg/ Kg IV Release of infundibular spasm Irritability Morphine 0.2mg/ Kg S.C or IM Hypoxia Oxygen Dehydration Volume Acidosis NaHco3 1mEq/ Kg IV
  52. 52. TOF MANAGEMENT Medical :  Correct iron deficiency anemia  Correct polycythemia  B-Blocker  Analgesics-to reduce the ventilatory drive. eg:morphine sulfate  Alpha-adrenergic agonists-to improve hemodynamic status by improving the myocardial contractility and increasing heart rate resulting in increased cardiac output. eg:phenylephrine
  53. 53. FACTORS THAT INCREASE THE RISK FORSURGERY Low birth weight Pulmonary artery atresia Major associated anomalies Multiple previous surgeries Absent pulmonary valve syndrome Young or old age Severe annular hypoplasia Small pulmonary arteries High peak RV–to–left ventricular pressure ratio Multiple VSDs Coexisting cardiac anomalies
  54. 54. CONTRAINDICATIONS FOR SURGERY The presence of an anomalous coronary artery Very low birth weight Small pulmonary arteries Multiple VSDs Multiple coexisting intracardiac malformations
  55. 55. POTTS SHUNT A side to side anastomosis of pulmonary artery with aorta is created The Potts shunt has been abandoned because of a tendency toward increased pulmonary blood flow and increasing difficulty with takedown at the time of corrective surgery.
  56. 56.  It consists of constructing a shunt between the ascending aorta and right pulmonary artery The Waterston shunt is sometimes used, but it also increases pulmonary artery blood flow. This shunt is more related to pulmonary artery stenosis, which generally requires reconstruction.
  57. 57. SURGICAL MANAGEMENT Surgical:  Palliative = Blalock-Taussig shunt for small PA’s  Definitive= Total correction
  58. 58. GOAL OF PALLIATIVE SURGERY The goals of palliation for tetralogy of Fallot (TOF) are to increase pulmonary blood flow independent of ductal patency and to allow pulmonary artery growth and even total correction.
  59. 59. ADVANTAGES OF MODIFIEDB-T SHUNT preservation of the subclavian artery, suitability for use on either side, good relief of cyanosis, easier control and closure at time of primary repair, excellent patency rate, and decreased incidence of iatrogenic pulmonary/systemic artery trauma.
  60. 60. CORRECTIVE SURGERY Primary correction is the ideal operation for treatment of tetralogy of Fallot (TOF) and is usually performed under cardiopulmonary bypass (CPB). The aims of the surgery are to close the ventricular septal defect (VSD), resect the area of infundibular stenosis, and relieve the right ventricular (RV) outflow tract obstruction (RVOTO).
  61. 61. CORRECTIVE SURGERY Before cardiopulmonary bypass is initiated, previously placed systemic-to- pulmonary artery shunts are isolated and taken down. Patients then undergo cardiopulmonary bypass. Associated anomalies, such as atrial septal defect (ASD) or patent foramen ovale, are closed.
  62. 62. SURGICAL COMPLICATIONS Early postoperative complications following repair of tetralogy of Fallot (TOF) include the creation of heart block and residual ventricular septal defects (VSDs). Ventricular arrhythmias are more common and are reportedly the most frequent cause of late postoperative death. Sudden death from ventricular arrhythmias has been reported in 0.5% of individuals within 10 years of repair.
  63. 63. TRANSPOSITION OF GREAT VESSELS The hallmark of tranposition of great arteries is ventriculoarterial discordance, in which the aorta arises from the morphologic right ventricle and pulmonary artery arises from the morphologic left ventricle
  64. 64. TRANSPOSITION OF THE GREAT ARTERIES (TGA) Complete TGA or D-TGA  Embryological inversion of the great arteries  Ventriculoarterial discordance Congenitally corrected TGA or L-TGA  Embryological inversion of the ventricles  Atrioventricular & ventriculoarterial discordance (double discordance)
  65. 65. CAUSESUnknownAbnormal persistence of the subaorticconus with resorption or underdevelopment of the subpulmonaryconus(infundibulum)
  66. 66. PATHOPHYSIOLOGY Atrialseptal defect Ventricular septal defect Patent ductus arteriosus
  67. 67. DIAGNOSIS OF TGA Transposition of the great arteries with intact ventricular septum: Prominent and progressive cyanosis within the first 24 hours of life is the usual finding in infants. Transposition of the great arteries with large ventricular septal defect: Infants may not initially manifest symptoms of heart disease, although mild cyanosis (particularly when crying) is often noted. Signs of congestive heart failure (tachypnea, tachycardia, diaphoresis, and failure to gain weight) may become evident over the first 3-6 weeks as pulmonary blood flow increases.
  68. 68. CONTD… Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: Infants often present with extreme cyanosis at birth,
  69. 69. MEDICATIONSInotropic agents Digoxin Loop diuretics Prostaglandins-alprostadil
  70. 70. SURGICAL CAREArterial switch procedure It represents an repair and establishes ventriculoarterial concordance
  71. 71. TGA MANAGEMENT Medical:  PGE1  O2 (3L/minute)  Correct :  acidosis ,hypoglycemia.  electrolyte disturbances. Transcatheter :  BAS Surgical:  Arterial switch (Jatene operation) at 7-15 days  Atrial switch ( Senning operation) at 6-9 months
  72. 72. SURGICAL REPAIR Balloon atrial septostomy  Developed by Rashkind (1965)  Enlarges the atrial communication Atrial switch  Performed first by Senning (1958) and later modified by Mustard (1964)  Atrial baffle is created to direct venous return to the contralateral ventricle
  73. 73. SURGICAL REPAIR Arterial switch  Performed first by Jatene (1976)  Great arteries transected and reattached to appropriate AV valve  Coronary ostia also transplanted  Surgical treatment of choice  Excellent outcomes so far
  74. 74.  Atrial Switch There are two types of atrial switch operations - the MUSTARD operation, and the SENNING operation. Both are similar in principle, but differ in technique. The atrial switch operation is an open heart procedure and is carried out with the assistance of a heart-lung machine. The right atrium is opened, and the wall between the atria is fully removed.
  75. 75.  AtrialSwitch Using pericardium (Mustard) or flaps created from the atrial septum and wall (Senning), a "baffle" is constructed directing blood from the veins in the right atrium towards the left ventricle. The same baffle also directs blood from pulmonary veins to the right ventricle. The circulation is therefore restored to normal in a functional sense. 
  76. 76.  Definitive Procedures Rastelli procedure. Pulmo artery transected distal to its valve and proximal end is oversewn. Rt. Ventricle incised high along its outflow tract. Intraventricular dacron prosthetic tunnel is created between the edge of septal defect and aortic orifice.
  77. 77. EBSTEIN’S ANAMOLY Congenital defect Origins of septal or posterior leaflets, or both, are displaced downward into RV Leaflets are variably deformed Atrialization of right ventricle Anterior leaflet is enlarged and sail-like
  78. 78. 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.
  79. 79. Physical ExaminationCyanosis and clubbing - Varying degrees ofcyanosis at various times in life and transientworsening with arrhythmiasPrecordial asymmetry-Usually left parasternalprominence and occasionally right parasternalprominence Absent left parasternal ( ie , rightventricular) lift an important negative signJugular venous pulse May be normal Large aand v waves late in the course of the disease,with development of right heart failure
  80. 80. Arterial pulses Usually normal Diminished volumeHeart sounds First heart sound widely split with loud tricuspid component Mitral component may be soft or absent in the presence of prolonged PR interval. Second heart sound usually is normal widely split when the pulmonary component is delayed due to RBBB.
  81. 81. ADDITIONAL HEART SOUNDS AND MURMURS Third and fourth heart sounds commonly present, even in the absence of congestive heart failure (CHF). Summation of third and fourth heart sounds, especially with prolonged PR interval, can mimic an early diastolic murmur. The holosystolic murmur of tricuspid regurgitation at the lower left parasternal area and sometimes at the apex murmur intensity and duration increase during inspiration. 
  82. 82. Chest X-Ray: Normal findings Cardiomegaly ( Rounded or Box- like contour ) Small aortic root and main pulmonary artery shadow Decreased pulmonary vasculature Large right atrium
  83. 83. Wall to wallheartEbstein’sanomaly
  84. 84. 12-LEAD EKG: Rhythm -Usually normal sinus findings Intermittently SVT, paroxysmal SVT, atrial flutter, atrial fibrillation, ventricular tachycardia Abnormal P waves consistent with right atrial enlargement PR interval -Most commonly prolonged May be normal or short in patients with WPW (B) syndrome QRS complex -RBBB Low voltage in many patients
  85. 85. ECG from a patient with Ebsteinsanomaly showing huge P waves andlow amplitude QRS waves. RBBB and Twave inversion are not present on thisECG.
  86. 86.  Echocardiogram:Echocardiogram standard for diagnosis.M-mode Paradoxical septal motion Dilated right ventricle Delayed closure of tricuspid valve leaflets more than 65 milliseconds after mitral valve closure
  87. 87. CONTD… Two-dimensional Apical displacement of the septal leaflet of greater than 8 mm/m 2 – Abnormalities in morphology and septal attachment of the septal and anterior tricuspid leaflets Eccentric leaflet coaptation Dilated right atrium Dilated right ventricle with decreased contractile performance Various left heart structural abnormalities
  88. 88. Doppler studies Varying degrees of tricuspid regurgitation Excludes associated shunts
  89. 89. ASSESSMENT OF SEVERITY AND SURGICAL OPTIONS BY ECHOCARDIOGRAPHY Functional right ventricular area less than 35% of total right ventricular area or an atrialized to functional right ventricular ratio greater than 0.5 associated with unfavorable prognosis Functional right ventricular size Degree of septal leaflet displacement Amount of leaflet tethering
  90. 90. CONTD… Magnitude of leaflet deformity and dysplasia Aneurysmal dilatation of right ventricular outflow tract (right ventricular outflow tract-to-aortic root ratio of >2:1 on parasternal short axis view) Moderate-to-severe tricuspid regurgitation
  91. 91.  Cardiac Catheterization:Cardiac Catheterization Rarely performed today Confirms echocardiographic findings Can reveal right ventricular electrical activity on the intracardiac ECG with simultaneous right atrial pressure and waveform when the catheter is withdrawn from the right ventricle, back across the tricuspid valve into the right atrium
  92. 92.  Complications: Congestive heart failure Sudden cardiac death Bacterial endocarditis Brain abscess Paradoxical embolism Transient ischemic attacks Stroke
  93. 93. MEDICAL MANAGEMENT Antibiotic prophylaxis for infective endocarditis Medical therapy for heart failure - Angiotensin- converting enzyme (ACE) inhibitors, diuretics, and digoxin Arrhythmia treatment - Medical treatments such as anti-arrhythmic drugs or radiofrequency ablation of the accessory pathways  Curative therapy of SVT with radiofrequency ablation is currently the treatment of choice.  The success rate is lower than that in patients without significant structural heart disease.
  94. 94.  Factors associated with lower likelihood of success include the following: Accessory pathways located along the atrialized right ventricle Multiple accessory pathways Complex geometry of the pathways Abnormal morphology of the endocardial action potentials in this region
  95. 95.  Indications for surgery are generally as follows:  New York Heart Association (NYHA) class I-II heart failure with worsening symptoms or with a cardiothoracic ratio of 0.65 or greater[6]  NYHA class III-IV heart failure  History of paradoxical embolism  Significant cyanosis with arterial O2 saturation of 80% or less and/or polycythemia with hemoglobin of 16 g/dL or more  Arrhythmias refractory to medical and radiofrequency ablation
  96. 96. SURGICAL MANAGEMENT Approaches: Approaches Tricuspid valve repair is preferred over valve replacement Bioprosthetic valves are preferred over mechanical prosthetic valves. The atrialized portion of the right ventricle can be resected surgically, and the markedly dilated, thin- walled right atrium can be resected. Associated septal defects may be closed
  97. 97.  Repair of TV & Closure of ASD: Repair of TV & Closure of ASD Usual preparation for operation & anaesthesia CPB Two venous cannulae Assess atrialized portion for paradoxical movement Body temperature about 25 o C RA incised parallel to AV groove
  98. 98.  Palliative procedures include creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through aortopulmonary shunt.
  99. 99. TOTAL ANAMOLOUS PULMONARYVENOUS RETURN
  100. 100. TAPVRDefinition -No direct communication exists between the pulmonary veins and the LA. -Instead, they drain anomalously into the systemic venous tributaries or into the RA. Epidemiology -1% of all congenital heart defects. -There is a marked male preponderance for the infracardiac type (male/female ratio of 4:1)
  101. 101. TOTALLY ANOMALOUS PULMONARYVENOUS DRAINAGE(INFRADIAPHRAGMATIC)  Allfour 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.
  102. 102. Pathophysiology1)Mixing of oxygenated and deoxygenated blood before or at the level of the right atrium (total mixing lesion). -Cyanosis -Right atrial blood either passes into the right ventricle and pulmonary artery or passes through an atrial septal defect (ASD) or patent foramen ovale into the left atrium. -Enlarged RA,RV, PA, small or normal LA, LV2) Obstructed pulmonary venous return -severe pulmonary congestion,pulmonary hypertension, hypoxia3) Restriction of PFO or ASD: -diminished LV preload->low cardiac output, small left side heart
  103. 103. CLINICAL MANIFESTATION1)Severe obstruction to pulmonary venous return, -In neonatal period: Cyanosis and severe tachypnea are prominent, but murmurs may not be present. -Severely ill and fail to respond to mechanical ventilation.2)Mild or moderate obstruction to pulmonary venous return and a large left-to-right shunt. - Heart failure in early life - Pulmonary artery hypertension->severely ill. - Systolic murmurs :the left sternal border, gallop rhythm -Cyanosis: mild.3)No pulmonary venous obstruction -No pulmonary hypertension these patients -less likely to be severely symptomatic during infancy. -Clinical cyanosis: mild.
  104. 104. SUPRACARDIACTAPVD
  105. 105. DIAGNOSISX-RAY -
  106. 106. In most cases without obstruction :the heart is enlarged, the pulmonary artery and right ventricle are prominent, and pulmonary vascularity is increased. In neonates with marked pulmonary venous obstruction :a perihilar pattern of pulmonary edema and a small heart
  107. 107. ECHOCARDIOGRAPHY: ESSENTIAL FOR DIAGNOSISA large right ventricle :the pattern of abnormal pulmonary venous connections.A vessel in the abdomen with Doppler venous flow away from the heart is pathognomonic of TAPVR belowthe diaphragm.Shunting occurs almost exclusively from right to left at the atrial level
  108. 108. TREATMENT Medical1.Intensive anticongestive measures with digitalis and diuretics ->in patient without pulmonary venous obstruction2.Metabolic acidosis should be corrected3.In infants with severe pulmonary edema(in infracardiac type and other type) ->ventilator support with oxygen and positive end-expiratory pressure4.PGE1; In patient with PHT (controversial), ->PGE1:increase systemic flow by keeping ductus open. ->In the infacardiac type, PGE1->maintaining the ductus venosus open5.If the size of the interatrial communication appears small and immediate surgery is not indicated ->balloon atrial septostomy or blade septostomy may be performed
  109. 109. NATURAL HISTORY1.CHF occurs in both types of TAPVR with growth retardation and repeated pneumonias2.Without surgical repair, two thirds of the infants without obstruction die before reaching 1 years of age.(d/t superimposed pneumonia)3.Patients with infracardiac type rarely survive for longer than a few weeks without surgery. Most die before 2months of age.
  110. 110. TRICUSPID ATRESIA
  111. 111. Types of tricuspid atresia Type 1 Type2 Type 3• The • The • The great great great arteries arteries arteries are are d- are l- related transpos transpos normally ed ed
  112. 112. Diagnosis of Tricuspidatresia History Physical examination Investigations
  113. 113. CyanosisGrowth retardationNasal flaringMuscle retraction*Brain abscess and bacterial endocarditis- headache, seizures and neurologic deficit
  114. 114.  Cyanosis P Digital clubbing JVP distension H Y Peripheral pulses Apical impulse displaced First heart sound increased S Second heart sound –split Continuous cardiac murmur- I 80%cases C A L examination
  115. 115. INVESTIGATIONS Lab studies-  Chest X-rayCBP-polycythemia Cardiomegaly,right atrial enlargement Echo  ECG Tall T waves-atrialPresence of ASD enlargement,first degree AV block
  116. 116. CARDIAC CATHETERIZATIONStastusof ductus arteriosusASD if present ATRIAL SEPTOSTOMY
  117. 117. Infants with Infants with decreased increased Palliative pulmonary blood flow pulmonary procedures blood flow Severe Severe Surgicalhypoxemia- congestive proceduresprostagland heart in E failure- digitalis and diuretic therapy
  118. 118. SURGICAL CARE Blalock-Taussig ShuntDefinition: A subclavian artery to pulmonary artery anastamosis.Advantages: Predictability of flow, shunt may grow with the child, post-op congestive heart failure is less common than with other procedures, graft thrombosis is uncommon.
  119. 119.  Disadvantages: Inadequate flow is possible, stenosis of the anastomosis is possible due to increased tension on the vessel, possible injury to the phrenic nerve, pulmonary hypertension is possible, small diameter of the subclavian may result in worsened hypoximia. Uses: Tetralogy of Fallot - can restore partial blood flow in the obstruction of the pulmonary circulation. Tricuspid Atresia - improves oxygenation of the desaturated blood due to the the shunt to the pulmonary circulation. Pulmonary Atresia - creates a shunt between systemic and pulmonary circulations
  120. 120. GLENN PROCEDURE Definition:  Advantages: A superior vena cava Pulmonary hypertension (SVC) to pulmonary is rare (blood is being artery anastomosis. shunted under low This is an end to side venous pressure), does anastomosis where the not increase the volume right pulmonary artery of work on the heart is divided at the distal (amount of blood end and is attached to returned to the heart is the side of the SVC. unchanged).
  121. 121. GLENN PROCEDUREDisadvantages:  Uses: Cyanosis may increase by Used to bypass the right the decreased perfusion to heart, therefore bothlungs, may call for a second palliative procedure becomes useful in such as Blalock-Taussig. anomalies where right Polycythemia secondary to side obstruction occurs, hypoximia may increase such as tricuspid atresia blood viscosity and or tricuspid stenosis. decrease flow through the pulmonary vascular bed thus decreasing oxygen saturations. Shunt is only effective if the child weighs over 8 kg.
  122. 122. FONTAN OPERATION The right atrium is connected to the pulmonary artery directly. Used when it is not possible to have a two ventricle repair (this case has tricuspid atresia). Pulmonary vascular resistance must be low. It is done at age 4 years or so.
  123. 123. INTERRUPTEDAORTIC ARCH
  124. 124. InterruptedAortic Arch
  125. 125. TYPES OFINTERRUPTEDAORTIC ARCH
  126. 126. Type ATYPE BTYPE C
  127. 127. TYPE A IAAThe arch interruption occurs distal to the origin of the left subclavian artery.
  128. 128. TYPE B IAAThe interruption occursdistal to the origin of the left common carotid artery.
  129. 129. TYPE C IAAThe interruption occursproximal to the origin ofthe left common carotid artery
  130. 130. PATHOPHYSIOLOGY With an interrupted aortic arch, venous blood returning to the heart goes to the lungs and returns to the left side of the heart as oxygenated blood, so it can go to the body through the aorta. However, oxygenated blood leaving the left ventricle to the Aorta, only goes to vessels proximal to the interruption. These vessels usually go to the upper body and the head.
  131. 131. CONTD… Arteries that come off the Aorta distal to the obstruction get deoxygenated blood from the right ventricle. This blood gets to the descending Aorta through a PDA. Repair of this lesion entails connecting the ascending and descending Aorta and ligating the PDA.
  132. 132. INTERRUPTED AORTICARCH/COARCTATION Heart failure/collapse
  133. 133. RASTELLI OPERATION  An example of a repair of complex CHD with two ventricles. This one had a big VSD, TGA and PS. The severe sub- pulmonary stenosis precluded an arterial switch (it would become the neo-aortic valve), so a conduit joins RV to PA, and the VSD is patched.
  134. 134. SEVERAL PARAMETERS SHOULD BE METTO ENSURE A SUCCESSFUL OUTCOME.  The candidate should be aged 4 years or older.  A right atrium of normal volume and normal caval drainage should be present.  Sinus rhythm should be present.  Mean pulmonary artery pressure should be low (ie, < 15 mm Hg), as should mean pulmonary arteriolar resistance.  The pulmonary artery-to-aorta diameter ratio should be greater than 0.75.
  135. 135. Nursing management ofclient with cyanotic heart disease
  136. 136. Helping family memebers to adjust Proiding pre operative care Preoperative assessmentNursing Preoprative teaching care Providing post oprative care Post operative assessment and management
  137. 137. NURSING DIAGNOSISImpaired gas exchange R/T altered pulmonary blood flowAltered cardiac output R/T specific anatomic defectActivity intolerance R/T O2 in blood & tissues Fluid volume excess with CHF
  138. 138. CONT………. Altered nutrition : Less than body requirement R/T excessive energy demands Increased Potential for infection R/TCHF Anxiety related to procedures & hospitalization Developmental delay R/T energy , inadequate nutrition Alterations in parenting
  139. 139. USEFUL HINTS Large male baby with rapid, shallow abdominal breathing: D-TGA Upper body blue, lower body pink; seen in : D-GA+PDA.COA Only cyanotic newborn who has a thrill: Tricuspid atresia. Ejection click is often heard in : Severe PS, HLHS Systolic ejection murmurs in first hours of life: TOF, PS, AS Silent heart characteristic of : D-TGA, Pulmonary atresia. Pulse oximetry& ABG should be obtained from the RIGHT arm. ECG showing LEFT axis deviation: Tricuspid atresia
  140. 140. Thank you
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