Diese Präsentation wurde erfolgreich gemeldet.
Wir verwenden Ihre LinkedIn Profilangaben und Informationen zu Ihren Aktivitäten, um Anzeigen zu personalisieren und Ihnen relevantere Inhalte anzuzeigen. Sie können Ihre Anzeigeneinstellungen jederzeit ändern.

Approach to acyanotic congenital heart diseases

6.439 Aufrufe

Veröffentlicht am


Veröffentlicht in: Gesundheit & Medizin
  • Follow the link, new dating source: ❶❶❶ http://bit.ly/2Q98JRS ❶❶❶
    Sind Sie sicher, dass Sie …  Ja  Nein
    Ihre Nachricht erscheint hier
  • Dating direct: ❤❤❤ http://bit.ly/2Q98JRS ❤❤❤
    Sind Sie sicher, dass Sie …  Ja  Nein
    Ihre Nachricht erscheint hier
  • 7 Sacred Signs from the Universe, learn more...  http://t.cn/AiuvUCDd
    Sind Sie sicher, dass Sie …  Ja  Nein
    Ihre Nachricht erscheint hier
  • Hear The Angels Sing: Listen to this free musical composition to clear away all the negativity in your life and welcome in miracles! Download your complimentary "Angel Soundscape" now. ◆◆◆ https://bit.ly/30Ju5r6
    Sind Sie sicher, dass Sie …  Ja  Nein
    Ihre Nachricht erscheint hier
  • best presentation on chd
    Sind Sie sicher, dass Sie …  Ja  Nein
    Ihre Nachricht erscheint hier

Approach to acyanotic congenital heart diseases

  2. 2.  Congenital cardiovascular defects, also known as congenital heart defects, are structural problems that arise from abnormal formation of the heart or major blood vessels. Defects range in severity from tiny pinholes between chambers that may resolve spontaneously to major malformations that can require multiple surgical procedures before school age and may result in death in utero, in infancy, or in childhood. AMERICAN HEART ASSOCIATION INTRODUCTION
  3. 3. Congenital heart disease is a problem with the heart's structure and function that is present at birth. DEFINITION
  4. 4.  Approximately 5 to 8 per 1000 live births .  About 2 or 3 in 1000 infants will be symptomatic during the first year of life with significant heart diseases that will require treatment.  CHD is the major cause of death in the first year of life.  There are more than 35 well recognized cardiac defects the most common heart anomaly is VSD. INCIDENCE
  5. 5. MULTIFACTORIAL GENETIC - 90% ENVIRONMENTAL - 10% PREDISPOSING FACTORS - Drugs / abortificient ! Infection ! Radiation ! Systemic disease (maternal) Etiology
  6. 6. The systematic approach to CHD includes:-  1.Detailed antenatal and postnatal history.  2.Systematic physical examination: Which includes general examination, followed by detailed examination of the cardiovascular system by inspection, palpation, percussion and auscultation. The examination of the respiratory system and abdomen should be done in a methodical manner.  3. Electrocardiography (ECG)  4. Chest X-ray  5. Echocardiography  6.Computed tomography (CT) angiogram and cardiac magnetic resonance imaging (MRI) in selected cases  7.Invasive procedures like cardiac catheterization and angiocardiography, if needed.
  7. 7. Approach to CHD  Is the Heart Disease Congenital? CONGENITAL 1. Recognition early in age 2. Murmurs more parasternally 3. Presence of central cyanosis 4. Presence of extra-cardiac anomalies ACQUIRED 1. H/o. joint pains, fever 2. Apical murmurs
  8. 8. Does the child have Heart Disease? NADAS Criteria  MAJOR 1. Systolic murmur Gr. III or > intensity 2. Diastolic murmur 3. Cyanosis 4. CHF  MINOR 1. Systolic murmur Gr. II intensity 2. Abnormal 2nd Sound 3. Abnormal ECG 4. Abnormal Chest X Ray 5. Abnormal Blood Pressure
  9. 9. ACYANOTIC vs CYANOTIC  Normal saturations are >94%.  Between 93 and 85 human eye cannot detect desaturation.  Below 85% we can visually diagnose cyanosis.
  10. 10.  Central cyanosis is characterized by blueness of the tongue and oral mucosa.  Central cyanosis most likely is related to cardiac or respiratory disease.  If the source of cyanosis is cardiac, it is constant.  Children in shock also may appear cyanotic owing to venous stasis, right-to-left intrapulmonary shunting, and increased peripheral oxygen extraction.
  11. 11. Classification CHD Acyanotic Cyanotic Left-to-right shunts Outflow obstruction - Ventricular Septal Defect (VSD) - Patent Ductus Arteriosus (PDA) - Atrial Septal Defect (ASD - Pulmonary Stenosis - Aortic Stenosis -Coarctation of aorta • Teralogy of fallot • Tricuspid atresia • Transposition of great vessels • Truncus arteriosus • Total anomalous pulmonary venous return (TAPVR) • Ebstein’s anomaly
  12. 12. Lesions % of all Lesions - Ventricular septal defect 25-30 - Atrial septal defect (Secundum) 10 - Patent ductus arteriosus 10 - Coarctation of aorta 7 - Tetralogy of Fallot 6 - Pulmonary Valve Stenosis 5-7 - Aortic Valve Stenosis 4-7 - Transposition of great arteries 4 - 0thers 20
  13. 13.  Acyanotic congenital heart diseases - present with signs of congestive heart failure and/or heart murmurs that are heard during physical examination and can manifest any time during infancy or early childhood.  Most of these patients present during the first 6 months of life.
  14. 14. PRESENTING COMPLAINTS  1.Feeding difficulties  2. Tachypnea  3. Sweating  4. Subcostal recession  5. Recurrent respiratory infections  6. Growth impairment in the infant  7. Exercise intolerance  8. Easy fatigability or murmur in the older child  The magnitude of the shunt or the severity of the obstruction determines the clinical presentation and symptoms.
  15. 15. FEEDING DIFFICULTIES  It is a common symptom in significant acyanotic CHDs.  In infants, feeding itself is a form of exercise or effort.  An infant with heart failure has the inability to complete feeds within 15 to 20 minutes, sucks less volume of milk (< 3.5 ounces per feeding), gets tired easily and takes frequent feeds (less than 2 hours).  As the feeds are inadequate they become irritable and cry excessively. This suck-rest-suck cycle continues round the clock.  As pulmonary vascular resistance declines, irritability and fussiness with feeding may indicate angina and ischemia in a child with an anomalous left coronary artery from the pulmonary artery.
  16. 16. RESPIRATORY DISTRESS  It is the most prominent sign of heart failure caused by significant lefttoright shunting in infancy.  Symptoms of respiratory distress include tachypnea or rapid breathing (respiratory rate > 60/min in newborn and > 50/min in infants) and intercostal and subcostal chest retractions.  In very sick infants, grunting and nasal flaring can also be present.  Unlabored tachypnea often accompanies cyanotic heart disease, whereas grunting and dyspnea are associated with left-sided obstructive lesions or respiratory illness.  Leftsided heart failure produces tachypnea with or without dyspnea.
  17. 17. Easy Fatigability  In an infant, it is seen as poor ability to suck and feed.  In older children, heart failure may be manifested as exercise intolerance; difficulty in keeping up with peers during sports or need for a nap after coming home from school and poor growth.
  18. 18. Repeated Lower Respiratory infections  Recurrent pneumonia - two or more episodes of RTI in one year or more than three in any time frame requiring hospitalization or IV antibiotics.  Frequent upper respiratory tract infection are not related to CHD.
  19. 19. Growth Retardation or Failure to Thrive  Failure to thrive is defined as weight < 3rd percentile for age.  Infants should gain about 20 g/day.  A normal infant can grow while receiving 100 calories/kg/day; infants whose growth is impaired by heart disease typically require 130 to 140 calories/kg/day  Acyanotic patients - weight is more affected due to high catabolic rate & poor feeding due to dyspnea ,fatigue.  Cyanotic patients – disturbances in both height and weight.
  20. 20. CHEST PAIN  Chest pain or angina is rare, but not unknown in infants and children.  Pain associated with palpitations, dizziness and panic attacks - mitral valve prolapse (Barlow syndrome).  LVOT obstruction – stenosis of the aortic valve, subaortic valve area, supra-aortic valve area or coarctation of the aorta, may present with chest pain and associated with dizziness and fatigue - due to demand-supply mismatch resulting in myocardial ischemia.  Chest pain in association with exercise intolerance and fatigue - hypertrophic or dilated cardiomyopathy.  Chest pain due to myocardial ischemia - abnormal coronary artery anatomy, including congenital anomalies of the coronary artery, coronary artery fistulas and stenosis or atresia of the coronary artery ostium and have been recognized in infants with an aberrant left coronary artery.
  21. 21. Syncope  History of syncope on mild to moderate exertion indicates severe aortic stenosis, hypertrophic cardiomyopathy (HOCM), severe pulmonary hypertension or complete transposition of the great vessels (CTGV).  In CTGV , syncope is due to significant bradycardia.  long QT syndrome – A uncommon cause of syncope.
  22. 22. Peripheral Edema  Pretibial and presacral edema are late developments in the child with congestive circulatory failure, apparently due to the difference in tissue turgor.  When peripheral edema due to heart failure does develop in an infant, it first appears periorbitally, and is usually preceded by other manifestations such as tachypnea, tachycardia, dyspnea and liver enlargement.
  23. 23.  Sweat on forehead – low cardiac output – increased sympathetic activity- seen in CHF.
  24. 24. Family History  If one parent has a congenital heart anomaly, the risk of the child having one – 10 percent.  When a first cousin has a congenital heart anomaly, the risk of a sibling having one is approximately 2 percent.  With no family history of CHD, if the firstborn has a congenital heart lesion, the risk of a second child having a congenital heart lesion is 2 to 3 percent.
  25. 25. Maternal History
  26. 26. BIRTH WEIGHT  HIGH BIRTH WEIGHT – TGA  LOW BIRTH WEIGHT – Intra uterine infections,drugs( alcohol)
  27. 27. CYANOSIS  onset –at birth / several days after birth  Severity of cyanosis  Permanent /paroxysmal nature  Parts of body that is cyanotic  Whether cyanosis becomes worse after feeding  Cyanotic spells – time of appearance , duration of spell, frequency of spell  Should be differentiate from breath holding spell  History of squatting.
  28. 28. HEART MURMUR  Heard within few hours after birth – stenotic lesion / AV valve regurgitation, small left to right shunt lesions as their flow characteristics is independent of PVR changes.  Murmurs of large left to right shunts are delyed due to slow regression of pulmonary vascular resistence.  Murmur that is first noticed in a routine examination of a healthy boy – innocent
  29. 29. How to assess PBF  SYMPTOMS –  Increased respiratory rate, retractions ,  Increased respiratory infections,  Sweating while feeding  Failure to thrive  Harrison sulcus  Infants with lesions associated with shunting at the ventricular or great vessel level are generally more symptomatic than those with only atrial-level shunting..
  30. 30. GENERAL APPEARANCE  A wide spectrum of extracardiac malformations occur in 15-45% of cases with CHD.  Extracardiac malformations can give a clue towards certain CHD.
  31. 31. PULSE  Character of the pulse – 1. Bounding pulses – PDA , BAV with severe AR , large systemic AV Fistulas , persistent truncus arteriosus. 2. Pulsus parvus et tardus- severe AS. 3. Weak ,thready pulse – cardiac failure /circulatory shock ,myocarditis, cardiomyopathy, legs in patients with CO A, arm pulses on the side of classic BT shunt / subclavian flap angiplasty.  Difference between upper and lower limb pulses – CO A/ interrupted aortic arch.  Discrepancy in both radial pulses - supravalvular aortic stenosis, aortic isthmus stenosis or pre-ductal CoA with the left subclavian arising below the coarctation & the aortoarteritis with obstruction to left subclavian artery.
  32. 32. BLOOD PRESSURE  Upper extremity hypertension – first sign of CoA.  A narrow pulse pressure - low cardiac output or severe aortic stenosis.  Pulse pressure widens in conditions with an elevated cardiac output (anemia and anxiety) or with abnormal runoff of blood from the aorta during diastole (PDA or aortic insufficiency).
  33. 33. Jugular Venous Pressure  The JVP is difficult to interpret in newborns and infants (due to short neck and tachycardia).  ASD- V ≥ A – large shunt A> V – small shunt , associated PHT , LV failure increased mean pressure – LV dysfunction.  VSD- JVP elevated with incresed A and V in patients with moderately restrictive ,non restrictive VSD eith CHF. EISENMENGER – normal/ small dominant A wave.  PDA – same  AS – increase of A wave amplitude in the absence of PHT.
  34. 34.  PS – prominent A wave and a presystolic liver pulse , With the advent of right ventricular failure and tricuspid regurgitation, the ʻvʼ wave increases, the Y descent becomes brisk and the liver manifests presystolic and systolic pulsations.
  35. 35. INSPECTION  Precordial bulge – suggests chronic cardiac enlargement  Pectus excavatum – pumonary systolic murmur and cardiomegaly in PA view  Harrisons groove – large left to right shunts leading to HF > 2 months .  left precordial bulge - left atrial enlargement in post- tricuspid shunts.  The upper sternum may bulge in children with a large left- to-right shunt and pulmonary hypertension or with elevated pulmonary venous pressure. Seen after 3 months of age.  In older patients with right ventricular(RV) hypertrophy secondary to pulmonary hypertension there
  36. 36.  Hyper active precordium – heart diseases with volume overload – large left to right shunts , AV valve regurgitation.  Left parasternal pulsations and lift can be seen in large atrial septal defects and in cases of RV hypertrophy.  In patients with PDA, aortopulmonary window, aortic insufficiency, aortic stenosis and CoA, suprasternal pulsations can be visible.
  37. 37. GENERAL APPEARANCE - SCAR  Right thoracotomy - right Blalock Thomas Taussig shunt, atrial septal defect repair, or mitral valve surgery.  Midline sternotomy - most heart operations, especially if cardiopulmonary bypass is used.  A left thoracotomy - patent ductus ligation, coarctation repair, and placement of a left Blalock Thomas Taussig shunt.
  38. 38. Apex Beat  In infants and children below 4 years, the apex beat is located in the fourth intercostal space (ICS).  Between 4 and 7 years it can be either in the fourth or fifth ICS and thereafter in the fifth ICS.  The displacement of the apex beat laterally or inferiorly indicates cardiac enlargement.  A hyperdynamic apical impulse is seen in volume overload conditions like post-tricuspid shunts and  A sustained heaving apical impulse is seen in pressure overload conditions like in LVOTO.
  39. 39. Thrill  Thrills at the lower sternal border are more likely to be associated with VSDs than mitral or tricuspid regurgitation.  Thrills at the right upper sternal border (RUSB) or suprasternal notch are most likely to be due to severe aortic stenosis.  Other less common cause of thrill in the suprasternal notch is pulmonary stenosis.  Diastolic thrills are less common
  40. 40. Auscultation  loud S1 can occur with increased flow across the AV valves from large lefttoright shunts, such as ASD,VSD,PDA.  Normal splitting: - small VSD, mild aortic or pulmonic stenosis  Wide splitting-  Volume overload (e.g., ASD, PAPVR)  Pressure overload (e.g., PS)  Electrical delay (e.g., RBBB)  Early aortic closure (e.g., MR)  Occasional normal child.
  41. 41.  Paradoxical splitting or reversed splitting – PDA, severe aortic stenosis, complete left bundle branch block, WPW syndrome Type 2.  Single S2  Pulmonary hypertension  One semilunar valve (e.g., pulmonary atresia, aortic atresia, persistent truncus arteriosus)  P2 not audible (e.g., TGA, TOF, severe PS)  Severe AS  Occasional normal child  Narrowly Split S2  Pulmonary hypertension  AS
  42. 42. Third Heart Sound  The S3 - low-frequency sound in early diastole and is related to rapid filling of the ventricle .  It is best heard at the apex or lower left sternal border. It is commonly heard in normal children and young adults.  A loud S3 is abnormal and is audible in conditions with dilated ventricles and decreased ventricular compliance (e.g., large-shunt VSD or CHF).  When tachycardia is present, it forms a “Kentucky” gallop.
  43. 43. Fourth Heart Sound or Atrial Sound  The S4 is a relatively low-frequency sound of late diastole (i.e., presystole) and is rare in infants and children.  When present, it is always pathologic and is seen in conditions with decreased ventricular compliance or CHF.  With tachycardia, it forms a “Tennessee” gallop.
  44. 44. SYSTOLIC and DIASTOLIC SOUNDS  An ejection click (or ejection sound) follows the S1 very closely and occurs at the time of the ventricular ejection's onset. It is usually audible at the base (either side of the upper sternal border), whereas the split S1 is usually audible at the lower left sternal border. 1. Stenosis of semilunar valves (e.g., PS or AS). 2. Dilated great arteries, which are seen in systemic or pulmonary hypertension, idiopathic dilatation of the PA, TOF (in which the aorta is dilated), and persistent truncus arteriosus.  Midsystolic click with or without a late systolic murmur is heard at the apex in mitral valve prolapse(MVP)
  45. 45. SYSTOLIC MURMURS  Mid-systolic murmurs- crescendo-decrescendo or diamond-shaped murmurs-  Bicuspid aortic valve - murmur is brief with an aortic ejection sound. The murmur is best heard over the right second interspace with little or no radiation.  valvular aortic stenosis- the maximum intensity is appreciated over the right second interspace; murmur is harsh and rough and a thrill may be present. The murmur radiates up into the neck and over both carotid arteries. In patients with aortic stenosis, the longer and later peaking murmur is usually associated with hemodynamically significant obstruction; The intensity of the murmur is variable and may not correlate with the severity of stenosis.
  46. 46.  In supravalvular aortic stenosis, the murmur may be loudest at a slightly higher location than in valvular aortic stenosis.  In subvalvular left ventricular outflow obstruction (hypertrophic cardiomyopathy), the maximum intensity of the murmur is usually located along the lower left sternal border or over the cardiac apex. It radiates poorly to the base and neck.
  48. 48.  The murmur of valvular pulmonary stenosis is harsh and best heard over the left second interspace and radiates to the left side of the neck and is frequently accompanied by a palpable thrill.  A pulmonary ejection sound at the onset of the murmur may be heard and S2 is widely split with a decreased intensity of P2.  The murmur duration correlates reasonably well with the severity.
  49. 49. Holosystolic (pansystolic) murmurs  This type of murmur is typically heard in AV valve regurgitation.  Mitral and tricuspid regurgitation murmurs are high pitched, with variable intensity and blowing quality.
  50. 50. Diastolic Murmurs  Diastolic murmurs are usually abnormal and may be early, mid or late diastolic.  Early diastolic murmurs - aortic and pulmonary regurgitation.  Mid-late diastolic murmurs occur due to stenosis or increased flow across the mitral or the tricuspid valves. These murmurs can occur in mitral stenosis, tricuspid stenosis, or as flow murmurs of ASD, VSD and PDA.  Late diastolic (presystolic) murmurs - pathological narrowing of the AV valves.  The pulmonary regurgitation murmur in patients with normal pulmonary artery pressure, is low-pitched and early diastolic because of the low-pressure gradient.  In patients with pulmonary hypertension, the murmur, known as the Graham-Steell murmur is heard, which is also early diastolic but is high pitched and decrescendo, because of the high pressure gradient between the pulmonary artery and the right ventricle in diastole.
  51. 51.  The continuous murmur from aortopulmonary communications is loudest around the S2.  The arterial continuous murmur is characterized by a more pronounced systolic component while a venous continuous murmur is characterized by a more pronounced diastolic component.
  52. 52.  Abdominal Examination-  Normally liver is palpable (2–3 cm below costal margin) at midclavicular line up to 4 to 5 years of the age, after that it remains palpable up to 1 cm till late childhood. If liver is palpable further downwards and the infant is irritable it probably indicates presence of congestive heart failure.  If the liver is in midline and palpable symmetrically, it indicates cardiac malpositions and underlying complex heart disease.  The site of the gastric fundus is detected by percussion, which determines visceral situs.  Spleen is not normally palpable; if palpable it indicates possibility of infective endocarditis.  Respiratory System Examination  Unlike adults, where crepitations is commonly basal, in neonates and infants it is more diffusely heard and often
  53. 53. CXR  Feature of increased PBF – 1. Visibility of pulmonary vascularity in lateral 1/3rd 2. Vascularity extending below diaphragm. 3. End on views – 3 on oneside / 5 on both sides 4. Trachea /right MPA >1.5 in children <12 yrs 5. Hilar haze in lateral views. 6.Ratio between The end on vessel diameter to that of accompanying Bronchus Normally it is 1.2:1 and in plethora it is ≥1.5:1. 7.The size of the RDPA in nomally < 14 mm and if it increased (> 16 mm in male and >14 mm in female), it indicates a shunt lesion.  In normal children diameter of RDPA is less than that of trachea. If the ratio of RDPA to trachea is more than 1 it indicates significant left to right shunt.
  54. 54.  Plethora occurs in left to right shunts, admixture lesions and transposition of the great arteries (d- TGA) without PS.  Increased pulmonary blood flow in an acyanotic child - ASD, VSD, PDA, ECD, partial anomalous pulmonary venous return (PAPVR).
  55. 55.  Prominent Main Pulmonary Artery Segment-  1. Poststenotic dilatation (e.g., pulmonary valve stenosis)  2. Increased blood flow through the PA (e.g., atrial septal defect [ASD], ventricular septal defect [VSD])  3. Increased pressure in the PA (e.g., pulmonary hypertension)  4. Occasional normal finding in adolescents, especially girls  Dilatation of the Aorta- patent ductus arteriosus (PDA), coarctation of the aorta (COA), Marfan
  56. 56.  PULMONARY VENOUS CONGESTION  Pulmonary venous congestion is characterized by a hazy and indistinct margin of the pulmonary vasculature.  This is caused by pulmonary venous hypertension secondary to left ventricular failure or obstruction to pulmonary venous drainage (e.g., mitral stenosis, TAPVR, cor triatriatum).  NORMAL PULMONARY VASCULATURE  Pulmonary vascularity is normal in patients with obstructive lesions such as pulmonary stenosis or aortic stenosis.  Patients with small left-to-right shunt lesions also show normal pulmonary vascular markings.
  57. 57.  Associated anomalies -hypoplasia of the right lung and right pulmonary artery, sequestration of right lung tissue receiving arterial supply from the aorta, and other congenital heart diseases such as VSD, PDA, COA, or TOF.
  58. 58. ASD - CXR  1. increased PBF  2. dilated MPA, both PA  3. right atrail enlargement  LAE – rare , seen in older adults with AF , associated MS  RVH – acute angle at apex  LVH – if MR is present in OP- ASD.  scimitar syndrome- A vertical vascular shadow along the lower right cardiac border - PAPVR from the lower lobe ,pulmonary veins drain in to IVC.  SV –ASD – localized dilatation of SVC at junction with RA.
  59. 59. SCIMITAR SIGN • The scimitar sign is produced by an anomalous pulmonary vein that drains any or all of the lobes of the right lung.
  60. 60. VSD  SMALL VSD - N , mild dilatation of pulmonary trunk  MOD VSD - cardiomegaly , enlarged LA – SPLAYING OF CARINA., increased PBF features  LARGE VSD – cradiomegaly , globular silhoutte- LA,LV,RV ,occ RA dilatation  With increasing PVR , left to right shunt decresese , cardiac size decrease , but enlargement of pulmonary trunk persists.  VSD with AR – LVH is disproportionate to shunt with dilated aortic root.  VSD Is a part of many congenital defects. So Xray feature is suggestive of the original defect that VSD.
  61. 61. PATENT DUCTUS ARTERIOSUS  Enlargement of the left heart chambers.  Enlargement of the ascending aorta or aortic arch.  Pulmonary plethora with enlarged central and peripheral artery.  Filling up of the angle between the aortic arch and the pulmonary artery : Most specific sign. • Possible PDA calcification in adults.
  62. 62. CO A  In a heavily exposed film, the precoarctation and postcoarctation dilatation of the aorta may be seen as a “figure of 3.”  This may be confirmed by a barium esophagogram with E-shaped indentation.  Rib notching is a specific finding of COA in an older child (usually older than 5 years) and is usually found between the fourth and eighth ribs.
  63. 63. Left anterior oblique view of chest, obtained with barium esophagography  Unilateral rib notching : ◦ left rib:anamolous origin of right subclavian artery distal to coarctation ◦ Right rib:if it involves origin of left subclavian artery
  64. 64. DDS FOR RIB NOTCHING  Chronic superior venacava obstruction  Chronic inferior venacava obstruction  Aortoarteritis  Intercostal AV malformations  Post BT shunt
  66. 66. ECG
  67. 67. ASD
  68. 68. Severity of shunt Size of Shunt Mild Moderate Severe Qp/Qs <1.4 1.4 – 2 >2 RAE No Yes Yes RV volume overload No Yes Yes N rsr’ rsR’/evidence of PAH Crochetage No No Yes Evidence of PAH No No Yes R’ > 7mm in V1
  69. 69. CROCHETAGE  In inferior leads, a notch near the apex of the R wave,coined “crochetage,” has been correlated with ASD  Seen in 73.1%, specificity - 92% (all 3 inf leads),86.1%(atleast 1 inf lead)
  70. 70. ASSOCIATED CONDITIONS  Lutembacher’s syndrome – LAE,RVH more common,Atrial arrhythmias more common
  71. 71.  TAPVR-resembles OS ASD,PR prolonged, AF in older persons  PFO – no hemodynamic changes ECG is normal or shows crochetage  Common Atrium – Absent atrial septum ECG shows features of combination of OS, OP, Sinus venosus ASD
  72. 72. 2. VSD  Small shunt –Normal / mild LVH  Moderate shunt - LVH, LAH (±)  Large shunt - BVH, LAH  Katzwatchal phenomenon- R+S in mid precordial leads >60 / >50 in single lead  Inlet VSD – LAD , counter clock wise loop  PVOD (Eisenmenger's syndrome) – RVH, RAE, R in v1, RAD >120.  SIZE OF THE SHUNT – 1.restrictive – N / Rsr in v1 2. mod restrictive – volume overloaded LV with BVH ,LAE 3. large non restrictive – BVH ,LAE , PAH  Number of VSDs- multiple VSD – left axis deviation with clockwise loop.  GERBODE defect – RAE with LV volume overload.  VSD with AR – deep Q ,tall R , deep inverted T ,coved ST segment in lateral precordial leads.
  73. 73. SITE OF VSD Axis Loop Inlet LAD Counterclockwise Multiple muscular LAD Clockwise L TGA with VSD LAD Clockwise DORV with VSD LAD Counter clockwise
  77. 77. AV SEPTAL DEFECTS  Marked left to right shunt  Early development of PAH  Associated MR determines outcomes
  78. 78. AV SEPTAL DEFECTS  Characteristic finding :  - left axis deviation  - QRS vector counter clockwise loop Mech : due to posterior and inferior displacement of the AV node and the left bundle  S waves in leads II, III, and aVF have a characteristically notched upstroke  QRS pattern in right leads resembles OS-ASD-Due to longer than normal Rt bundle
  79. 79. PDA  Diagnosis by -  LV Volume overload  LAE  +/- RV pressure overload  QRS axis – normal
  80. 80. SHUNT SEVERITY  Small –normal  Moderate – LAE + LV Vol overload q & Tall R in V5-6,deep S in V1-2, T remain upright  Large – Biatrial enlargement + BV hypertrophy  Eisenmenger – RAE + RVH
  81. 81. COMPLICATIONS  PAH  BUT Q IN V5-V6 PERSIST EVEN AFTER DEVELOPMENT OF PAH  PR interval prolongation -10 – 20%  AF – in older persons with large shunt
  82. 82. ASSOCIATED CONDITIONS  RAD – Neonates , LAD- Rubella, sometimes superior axis  CoA – LVH with secondary ST- T changes
  83. 83. Pulmonary stenosis  Obstruction to RVOT increases afterload on RV – RV systolic overload  RVH With or without RAE  Good correlation with hemodynamics and ECG
  84. 84.  Normal ht of R in V1 =<8 mm in infancy, <10 mm in children  Persistent upright T waves in V1 & V2 after 4 days age indicates RVH
  85. 85. SEVERITY OF PS MILD MODERATE SEVERE P Wave Sharp pointed/peaked P waves <2.5 mm in II & V1 Sharp pointed/peaked P waves >2.5 mm in II & V1 Sharp pointed/peaked P waves >5 mm in II & V1 QRS Axis 40 to 60(N) 100 to 120 120 to 150 QRS voltage Tall R in V1/V2,but R/S <1 <10 – 15mm Tall R in V1/V2,but R/S >4:1,<20mm or rsR’/Notched R’ Shift of transition zone to left Tall R /qR in V1/V2,but R/S >>4,with ht >20mm Shift of transition zone to left QR in V1 T wave Inverted in V1/V2 only Upright T in V1 –V2 after 4 days may be only sign Inverted upto V4-V5, In 50% cases upright in right leads Deep symmetric inversions,upto V6 T inversions II,III,AVF
  86. 86. RVSP MEASUREMENTS • Ht of R in V1/V4R x 5,between 2 – 20 yrs age, if pure R wave is present. • Ht of R in V1 + 27 • Ht of R in V1 x 3 + 47 • Pressure gradient across pulmonary valve= 10.5(ISM)+2.6 (S1)+S2 Score+T score ISM=intensity of systolic murmur(gr 1-6) S1=S in lead I in mm S2 score= -10 if P2 is normal,+2 if P2 is audible but diminished,+15 if P2 is inaudible T score= +15 if T in V1 is diphasic with R in V1 >10mm,otherwise T score = 0
  87. 87. TYPES OF PS • Valvular PS – As described • Sub/Supra valvular –same • Sub infindibular PS/DCRV –Degree of RVH in V1-3 less than anticipated, Upright T in V3R may be the only finding in 40% cases • Dysplastic Pulmonary valvular stenosis as a part of Noonan syndrome –Extreme axis deviation, QRS prolonged and splintered, QS in inferior and left precordial leads
  88. 88. Pure PS  RAD,But not marked  Tall R in Rt precordial leads  Deeply inverted symmetric T waves TOF(VSD + PS)  Marked RAD  Tall R only in V1,with sudden transition  Less deeply inverted T waves/upright T in V1 & V2  EXCEPTION-  Pentology –LA or LV enlargement  Triology –marked increase in RVSP ~PS + LAE  Pulmonary atresia –marked increase in RVSP
  89. 89. 4. AS  Mild to moderate- Normal or LVH  Severe - LVH with or without “strain” 5. COA  Infants younger than 6 mo- RBBB or RVH  Older children- LVH, normal, or RBBB 6. Anomalous origin of the left coronary artery from the PA - Myocardial infarction, anterolateral
  90. 90. COR TRIATRIATUM  RAE, RVH,RAD are common  LAE may be seen due to prolonged conduction in proximal accessory chamber
  91. 91. REGURGITANT LESIONS  CONGENITAL PR  Normal in mild-mod cases  RV volume overload in severe cases  AF unlikely  CONGENITAL AR  LV volume overload with LV q waves
  92. 92. APPROACH Acyanotic congenital heart disease> Step -1-see which chamber is enlarged Step -2-suppose it is RV Step-3-is it volume overload(rsr’/rsR’)or pressure overload(monophasic R/qR) Step-4- • Volume overload-ASD • Pressure overload-PS, Infantile coarctation,cor triatriatum & congenital MS-
  93. 93. Step-2-suppose it is LV Step-3-is it LVH alone/BVH Step-4-LVH alone- Step-5-volume/pressure Step-6- • LV Volume overload-Moderately restrictive VSD, PDA • LV Pressure overload-coarctation of aorta/congenital AS/interrupted aortic arch/critical PS of infancy
  94. 94. Step-4-BVH-nonrestrictive VSD, large PDA, AP window ,L-TGA Step -5- q in lateral leads/v1 Step -6- q in v1,absent in lateral leads-L TGA q in lateral leads-simple VSD,PDA
  95. 95. Introduction- ASD  Incidence is 1 child per 1,500 live births  More frequent in females than males by about 2:1  Most ASDs occur sporadically; however, a few families have the defect as a genetic abnormality.  Mutation in-TBX5, NKX2.5 , GATA4, Myosin heavy chain 6 Samanek M et al. Pediatr Cardiol1999;20:411–7
  96. 96.  Small ASD – ASYMPTOMATIC  MODERATE ASD- symptoms starts at 2nd decade  Symptoms after 40 yrs – 1. Decrese in LV distensibility -> increased left to right shunt 2. Age related increase in atrial arhhythmias – precipitates CHF 3. Development of PAH .  By 4th decade 50% will became symptomatic, 5th decade – 75% will become symptomatic.  Depending on ASD size and PBF – 7% will develop Eisenmengerization in 1st decade and 8% in 2nd decade.  Infective endocarditis does not occur in patients with isolated ASDs.
  97. 97.  SURGICAL CLOSURE - 1. Primum septal defects, sinus venosus and coronary sinus defects and defects with associated anomalous pulmonary venous drainage. 2. Secundum atrial defects that are larger than 38 mm in diameter or defects that have insufficient rims (< 5 mm) are also not suitable for transcatheter device closure.  DEVICE CLOSURE-  Devices available for clinical use have included the Sideris buttoned device, the Angel Wings device, the CardioSEAL device, and the Amplatzer ASD Occlusion Device.  There must be enough rim (4 mm) of septal tissue around the defect for appropriate placement of the device.  The patients are administered aspirin 81 mg/day for 6 months.
  98. 98. VSD  The incidence of spontaneous closure in perimembranous and muscular VSDs is high, while it is low in outlet defects and inlet defects do not close.  60 percent close before 3 years and 90 percent by 8 years of age.  Surgery for Ventricular Septal Defect-
  99. 99.  RECOMMENDATIONS FOR DEVICE CLOSURE OF MUSCULAR VSDS-  Class IIa - Class IIA is reasonable for infants who weigh ≥ 5 kg, children and adolescents with hemodynamically significant muscular ventricular septal defect (MVSD) to undergo percutaneous VSD device closure (level of evidence: B).
  100. 100.  Exclusion criteria - Weight less than 3.0 kg (unless the hybrid perventricular approach is used); distance of less than 4 mm between the VSD and the aortic, pulmonic, mitral or tricuspid valves.  Except the subpulmonic and inlet VSDs, most of the other VSDs can be closed without surgery.
  101. 101. PDA  INDICATIONS FOR PDA CLOSURE – Premature Infants  PDA closure is indicated in all patients less than 10 days of age with symptomatic PDA, who had no response to indomethacin and for those who are more than 10 days old.  Symptomatic PDA is defined as respiratory rate of more than 70 per minute, heart rate more than 160 per minute, liver enlargement more than 3 cm below costal margin and cardiomegaly more than 60 percent on chest X-ray. Children and Adults  Symptomatic and even asymptomatic PDA should be closed to avoid CHF, pulmonary vascular disease, IE and aneurysm formation.
  102. 102.  Gianturco coils- The coil diameter should be at least two times the minimum PDA diameter and yet fit within the ampulla of the ductus.  The immediate success rate using single or multiple coils ranges from 67 to 95 percent, almost invariably related to ductus size.  Although ducts as large as 7 mm have been closed with coils, a larger ductus diameter is an unfavorable factor.  Sideris Buttoned Device-  Gianturco-Grifka Vascular Occlusion Device- ideal for long tubular PDA  Duct-occlud and Nit-occlud Device- for <2 mm and < 6 mm PDA s  Amplatzer duct occluder (ADO)- a device is chosen so that the diameter of the pulmonary arterial end of the device is 2 to 3 mm larger than the narrowest diameter of the ductus (usually the pulmonary end of the ductus)
  103. 103.  Indications for Surgery – 1. Failure of catheter based closure or anatomy of ductus not suitable (Type C). 2. Preterm infants with contraindications or failure of pharmacotherapy. 3. Calcific PDA, aneurysmal ductus, hypertensive ductus, inflamed ductus. 4. Recurrent ductus, giant ductus. 5. PDA with complex CHD.
  104. 104. Aortic Stenosis: Timing of Intervention  For infants and older children 1. Left ventricular dysfunction: Immediate intervention by balloon dilatation, irrespective of gradients (Class I). 2. Normal left ventricular function: Balloon dilatation if any of these present:  i. Gradient greater than 80 mm Hg peak and 50 mm Hg mean by echo-Doppler (Class I).  ii. ST-T changes in ECG with peak gradient of greater than 50 mm Hg (Class I).  iii. Symptoms due to AS with peak gradient of greater than 50 mm Hg (Class IIa). In case of doubt about severity/symptoms, an exercise test may be done for older children (Class IIb).  For neonates: Balloon dilatation if symptomatic or if there is evidence of left ventricular dysfunction/mild left ventricular hypoplasia (Class I), or if Doppler gradient (peak) greater than 75 mm Hg (Class IIa).
  105. 105.  The balloon size used should not exceed the aortic annular size.  A successful valvotomy is defined as fall in gradients of more that 50 percent of predilatation values.  surgical Intervention-  In children valve replacement is always difficult as adequate size of prosthesis may not be available.  Ross procedure remains the choice for small infants but there might be gross aortic and pulmonary annulus mismatch and hence it may not be very successful.  Aortic valve repair is still not a very preferred mode of treatment due to the poor outcome in long run.
  106. 106. PS NATURAL HISTORY  The severity of stenosis is usually not progressive in mild PS.  over 95% of patients who had an initial Doppler gradient less than 25 mm Hg were free of cardiac operation over a 25-year period. The severity tends to progress with age in moderate or severe PS.  Indications for the Procedure- Currently pulmonary balloon valvuloplasty is the first line of treatment for pulmonary valve stenosis at any age. 1. Any symptomatic patients , asymptomatic patients with severe obstruction & 2. Patients with moderate obstruction should undergo elective valvuloplasty, if the right ventricular pressure is ≥ 50 percent of systemic or higher, 3. Patient with echocardiographic peak instantaneous gradient of > 40 mm Hg 4. Clinically significant pulmonary valvar obstruction in the presence of RV dysfunction
  107. 107.  A balloon is chosen that is 20 to 40 percent larger than the angiographically measured pulmonary valve annulus and it is positioned over a guidewire with the valve at its midpoint  Surgical Technique - Indications 1. Dysplastic pulmonary valve with valve ring hypoplasia. 2. Fixed infundibular and supravalvar stenosis with pulmonary valvar stenosis.
  108. 108. Natural history- CO A  Bimodal presentation.  produces significant symptoms in early infancy and after age 20 to 30 years.  Neonates with severe coarctation become acutely symptomatic when the ductus closes.  Most who survive the hazards of infancy reach adulthood  25% die by age 20 years,  50% die by age 30 years  75% die by age 50 years.  Survival has been reported at age 74 years and 76 years. natural history of
  109. 109. CO A  Indications for treatment- 1. In infants with hemodynamic compromise . 2. In older patients a consistent systolic blood pressure gradient between the arms and legs of more than 20 mm Hg 3. Systemic hypertension at rest or following provocation with exercise greater than the 97th percentile for age along with evidence of important luminal stenosis. Surgical treatments- 1. End-to-End Anastomosis Including Extended Repair 2. Subclavian Flap Repair 3. Interposition Grafts, Bypass Tube, Extra-anatomic Bypass Grafts 4. Patch Aortoplasty Interventional treatments-  1. Balloon Angioplasty-sick hemodynamically compromised infantas a bridge to definitive surgical repair , children weighing less than 25 kg when faced with significant residual or early recurrence of gradient  2. Stent Placement- Covered stents – preferred where possible.
  110. 110. RSOV  M:F- 4:1  More in asians  Presentation  Acute tearing chest pain/interactable sob  Small shunt –asymptomatic – 20%  Small shunt – incidious onset sob – 45%  Large shunt – acute hf – 35% natural history of
  111. 111. RSOV natural history of acynotic chd  Involvement of  RCC – 67%  NCC - 25%  LCC – 8%  Rupture in to  RV - 70-75%  RA – 15-20%  LA – 5-10%  LV - <5% ASSOCIATED WITH-  Vsd – 30-40% - Sub pulmonic  AR – 39%  Prolapse of cusps due to vsd  BAV  Rvot obs -10 %  BAV - 15-20%
  112. 112.  Device closure should not be attempted in - 1.Patients with an aneurysm which has ruptured into the pulmonary artery and left ventricle, 2.Presence of associated lesions such as as VSD and aortic regurgitation, 3. Aneurysmal opening within 5mm of coronary ostia, 4.Right-to-left shunting across the defect with systemic saturation less than 94 percent, 5. Patients with pulmonary vascular resistance greater than 7 Wood units and 6. Significant right ventricle/left ventricle dysfunction with left ventricular ejection fraction less than 30 percent
  113. 113. THANK YOU