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  • Holt-Oram- can have absent radius or thumb, triphalangeal thumb or more severe limb defects. 95% not associated with any chromosomal defect
  • Vsd

    2. 2. Ventricular Septal Defect Henri Roger was the first man to describe a ventricular septal defect, in 1879 he wrote: “A developmental defect of the heart occurs from which cyanosis does not ensue in spite of the fact that a communication exists between the cavities of the two ventricles and in spite of the fact that the admixture of venous blood and arterial blood occurs. This congenital defect, which is even compatible with long life, is a simple one. It comprises a defect in the interventricular septum”
    3. 3. INTRODUCTION Isolated VSD - most commonly recognized CHD 2- per 1000 live birth Forms 20 % of all CHD 50 % when associated with other major defects . 75-80% of small VSD’s close spontaneously by late childhood 10-15% of large VSD’s close spontaneously 60% of defects close before age 3, and 90% before age 8
    4. 4. HISTORICAL ASPECT Roger in 1879 - first described Eisenmenger – 1897 - autopsy finding Pathophysiology by Abbott (1936) & Selzer (1949) . 1952 – Muller and Danman- pulmonary artery band 1954 - Lillehei and associates – First vsd repair 1956 – Dushane – Transventricular / Stirling – Transatrial 1961 – Kirklin- Repair of VSD in infants 1976 - Baratt-Boyes – deep hypothermia & circulatory arrest
    5. 5. The primitive cardiac tube has five zones: the arterial trunk the bulbus cordis ) the ventricle the atrium and the sinus venosus V B V D A SV
    6. 6. A V
    7. 7. EMBRYOLOGY VSD occurs during the first 8 weeks of foetal life 3 components – Interventricular muscular partition - Endocardial cushions - Bulbar ridges that separate great vessels .
    8. 8. Four cushions (AVC) have developed at the A/V junction; the superior and inferior cushions will meet to divide the AV orifice (AVO) into the tricuspid and mitral valves. The inferior septal crest (VS) will aim to meet the divided valve where the cushions fuse.
    9. 9. Formation of IV septum  IV septum upwards from the floor of the bulboventricular cavity,division into rt and lt halves.It meets fused AV cushions and partially fuse with them.  Two ridges rt and lt arise in conical upper part of bulboventricular cavity.fuse with each other to form bulbar septum.  Gap persists between the two-filled by proliferation of tissue from the AV cushions.
    10. 10. Membranous IV septum Ant part separates rt and left ventricles. Post part separates rt atrium and left ventricle. This is because the interatrial and interventricular septum don’t meet in the midline.
    11. 11. Ventricular Septum R Membranous Muscular Spiral (Aorticopulmonary)
    12. 12. What if?.............. - then you get the truncal septum fails to fuse with the septal crest? - perimembraneous VSD the truncal septum is deviated to the PA side? - tetralogy of Fallot the truncal septum fails to develop? - truncus arteriosus the ventricular septum fails to reach the AV valve? - AV septal defects the arterial trunk stays over the RV but does divide? - double outlet RV the aortic valve pushes up and right instead of the pulmonary? - transposition of the great vessels the ventricles fail to centralise over the AV valve - double inlet left ventricle (commonest form of single ventricle) the loop is to the left? - ventricular inversion (RV on the left, LV on the right)
    13. 13. Associated Defects Left Heart Defects – Aortic stenosis – Coarctation of the aorta Right Heart Defects – Tetrology of Fallot – Double Outlet Right Ventricle Truncus Arteriosus Some single ventricle (e.g. Tricuspid atresia, double inlet left ventricle)
    14. 14. Chromosomal Disorders associated with VSD Trisomy 21: 40% of T21 will have VSD Trisomy 13, 18: 18% of T13, 31% of T18 will have VSD 22q11 deletion: – Tetrology of Fallot is most common anomaly – VSD with or without aortic arch anomaly is second most common Holt-Oram (Hand-heart syndrome): TBX5 gene found on Chromosome 12 Recurrence risk for VSD based on parental VSD – Paternal 2% – Maternal 6-10%
    15. 15. The Ventricular Septum 1. Membranous 2. Outflow 3. Trabecular septum 4. Inflow 5. Subarterial / Supracristal
    16. 16. The Ventricular Septum
    17. 17. Ventricular Septum The membranous septum-The septal leaflet of the tricuspid valve divides the membranous septum into 2 components, the pars atrioventricularis and the pars interventricularis.1 The muscular septum is a nonplanar structure that can be divided into inlet, trabecular, and infundibular components. An inlet VSD has no muscular rim between the defect and the atrioventricular valve annulus. The trabecular septum is the largest part of the interventricular septum. The location of defects in the trabecular septum can be classified as anterior, midmuscular, apical, and posterior The infundibular septum separates the right and left ventricular outflow tracts. On the right side, it is bordered by the line from the membranous septum to the papillary muscle of the conus inferiorly and the semilunar valves superiorly.
    18. 18. Nomenclature / Classification TYPE IConal,Supracristal, Infundibular,Subarterial TYPE II – Paramembranous TYPE IIIInlet/ AV canal type Type IV – Muscular
    19. 19. CLASSIFICATION ROBERT ANDERSON Perimembranous Muscular Doubly committed Juxta arterial defects Van Pragh AV canal type Muscular VSDs Conoventricular Conal
    20. 20. Lesion Size • Restrictive VSD – < 0.5 cm2 (Smaller than Ao valve orifice area) – Small L to R shunt – Normal RV output – 75% spontaneously close < 2yrs • Non-restrictive VSD – > 1.0 cm2 (Equal to or greater than to Ao valve orifice area) – Equal RV and LV pressures – Large hemodynamically significant L to R shunt – Rarely close spontaneously
    21. 21. Based on size 1. Size 1) Large : 2/3rd of aortic annular size or > 15mm or > 1cm/sq.m of BSA Peak RV sys = LV sys pressure 2) Moderate : half of aortic annular size 5 to 15mm RV pressure to ½ of LV Qp/Os>2.0 3) Small : One third of aortic annular size insufficient size to raise RV pressure & Qp/Qs < 1.75
    22. 22. TYPE I VSD Conal,Supracristal,Infundibular, Subarterial Maldevelopment of bulbotruncal system Located within infundibular portion of RVOT Superior margin – no muscular tissue Inferior margin – defect is muscular Can extend upto right or sometimes non-coronary cusps of the aortic valve Conduction system is not in surgical proximity
    23. 23. Doubly committed subarterial VSD
    24. 24. TYPE II VSD Also called Conoventricular defects. Most common (80%) Margins include membranous septum or remnant May have extensions into inlet, outlet or trabecular septum Postero-inferior margin very close to the antero-septal commissure of the Tricuspid valve Can extend upto non-coronary cusp of aortic valve Danger area- inferior and posterior region of defect
    25. 25. Membranous VSD
    26. 26. TYPE III VSD AV Canal type / Inlet VSDs Form about – 5% of all VSDs Located posteriorly – subjacent to TV septal leaflet in inlet portion Superior border- may extend to the annulus of tricuspid valve Conduction system at risk – close proximity to AV node Guide- apical area of Triangle of koch Common bundle courses around infr. aspect of defect
    27. 27. Endocardial Cushion (Inlet VSD)
    28. 28. INLET VSD
    29. 29. TYPE IV VSD Muscle tissue all around the defect May be either anterior, in the inlet septum, mid-muscular or apical Classification according to location is important because it determines the approach for surgical closure. – Inlet and mid-muscular ----- RA approach – Anterior ------- Rt. Ventriculotomy – Apical ------ May require left ventriculotomy May be Single/ multiple Swiss Cheese VSD
    30. 30. MUSCULAR VSD
    31. 31. Pathophysiology Two determinants – Size of defect – Pulmonary vascular resistance These determine – Pressure gradient across VSD – Shunt volume across VSD After birth PVR falls ------ Large flow across shunt if large VSD Causes increased PA pressure (initially flow related), increased PV return, hence LA enlargement and LV overload PH initially flow related and reversible
    32. 32. Pathophysiology Later ----- Intimal proliferation and medial hypertrophy leads to fixed irreversible PH Flow through the lungs decreases as PVR increases, hence shunt volume decreases Eventually PVR > SVR, hence R  L shunt across VSD Cyanosis  Eisenmengerised VSD Shunt calculated by Fick’s principle Qp/Qs Aortic O2 % sat - Central Venous O2 % sat Pulm. Vein O2 % sat – Pulm. Art O2 % sat With small VSDs, there is resistance to flow across the VSD hence Qp/Qs is rarely > 1.5 With moderate VSDs, Qp/Qs is between 1.5 and 2.5, and is less likely to cause pulm vasc disease
    34. 34. HEATH- EDWARD CLASSIFICATION Grade I - hypertrophy of the media of small muscular arteries and arterioles. Grade II - intimal cellular proliferation in addition to medial hypertrophy. Grade III - progressive intimal proliferation and concentric fibrosis. Grade IV - "plexiform lesions" Grade V - angiomatous and cavernous lesions and hyalinization of intimal fibrosis. Grade VI - necrotizing arteritis.
    35. 35. Natural History Spontaneous closure is known, primarily with perimembranous and muscular VSDs. Subarterial and inlet VSDs rarely close – Chances differ with age at detection At 1 month  80% of large VSDs close At 6 months  50% At 12 months  25%
    36. 36. Natural History Patients with large vsd- symptom develop soon after birth. CHF manifested by- dyspnea/rptd.pulmonary infn/hepatomegaly/sweating/failure to thrive. Irreversible pulmonary vascular disease after 1-2 yrs of age. Some children with isolated vsd develop Subpulmonic stenosis- pt. not at risk of pulmonary vascular disease
    37. 37. VSD IN ADULT SVT – AF prevalent with increasing age. VSD+ AR – High risk of bacterial endocarditis Right sided failure- due to pulmonary stenosis Left sided failure- in pts. of aortic valve prolapse. Eisenmenger complex- 2nd & 3rd decade of life Pregnancy- spontaneous abortion/small-for-date babies Mortality- 27% by 20 years & 69% by 60 years.
    38. 38. CLINICAL FEATURES Grade I Small ventricular septal defect (less than 1.5 cm2) Patient is asymptomatic. Murmur can be present since a few days after birth. Grade II Frequent respiratory tract infections. CHF (rare). Cyanosis is absent even during exercise. Functional aerobic capacity is usually moderately reduced with early fatigability but unusual CHF. Grade III More frequent respiratory tract infections. Defective growth. Moderate cyanosis at times with exertion Congestive heart failure frequent in the first years of life (one of the most frequent causes of CHF during the first year of life). Functional capacity markedly reduced. Grade IV or Eisenmenger Complex
    39. 39. EISENMENGER COMPLEX • Infants with Eisenmenger may become easily fatigued, especially during crying spells and at feeding time • Low tolerance for extra exertion • Shortness of Breath (dyspnea) and/or rapid breathing • Fainting (syncope) • Difficulty eating, breathing or sucking • Poor weight gain • Slow growth or other physical retardation
    40. 40. CLINICAL FINDINGS • Pulse pressure is relatively wide • Precordium is hyperkinetic with a systolic thrill at LSB • S1&S2 are masked by a PSM at Lt.sternal border ,max. intensity of the murmur is best heard at 3rd,4th&5th Lt interspace.Also well heard at the 2nd space but not conducted beyond apex • Lt. 2nd space –widely split &variable accentuated P2 • Delayed diastolic murmur at the apex &S3 • Presence of mid-diastolic ,low pitched rumble at the apex is caused by increased flow across the mitral valve &indicates Qp:Qs=2:1/greater • Maladie de Roger –small VSD presenting in older children as a loud PSM w/o other significant hemodynamic changes
    41. 41. ECG Size of Defect Results Small restrictive VSDs Normal tracing Medium-sized VSDs Broad, notched P wave characteristic of left atrial overload • Signs of LV volume overload — deep Q and tall R waves with tall T waves in leads V5 and V6 • Signs of atrial fibrillation are often present Large VSDs Right ventricular hypertrophy with right-axis deviation. With further progression, the ECG shows biventricular hypertrophy; P waves may be notched or peaked.
    42. 42. ECG May show right/left or combined ventricular hypertrophy Presence of RAD represents elevated RVP and PAP Postoperative RBBB is common CHEST X-RAY Cardiomegaly : proportional to the volume overload. Mainly LV, LA and RV enlargement. Increased pulmonary blood flow, PAH. Unless LA is significantly enlarged its difficult to differentiate from ASD. RV may not be as enlarged as anticipated as it receives the shunt into its outflow tract.
    44. 44. 42-year-old woman with Eisenmenger complex, demonstrating atrial fibrillation with right axis deviation, right ventricular hypertrophy, right bundle-branch block, and premature ventricular beat.
    45. 45. Ventricular septal defect in a 7-month-old. Frontal(A)and lateral (B) views of the chest show moderate cardiac enlargement including right atrial, right ventricular, and left atrial enlargement with posterior displacement of the left main stem bronchus (arrow in B) and increased pulmonary
    46. 46. 2D -ECHO Determine vsd location LV outflow morphology Aortic valve involvement AV valve chordal attachment Septal trabeculations may cause multiple reflections and obscure small defects Prominent bulging of tissue into the RV : aneurysmal perimembranous VSD
    47. 47. 2D-ECHO
    48. 48. Supracristal VSD, with pulm outflow tract obstruction
    49. 49. Cardiac catheterization Identification of Multiple VSDs Cardiac catheterization can quantify shunt volume and pulmonary arterial resistance. Step-up in oxygen saturation may be detected in the pulmonary artery rather than in the right ventricular cavity because of streaming of the shunted blood into the pulmonic trunk. If aortic valve prolapse is significant, left-to-right shunting by oximetry may be fairly unremarkable, because the ventricular septal defect (VSD) in such cases is partially obstructed.
    50. 50. CATH GRADING Grade I Right atrium and right ventricular pressures are normal, due to the low volume shunt. Oxymetry can be misleading, showing only a mild step up in oxygen saturation at ventricular level. The passage to the left ventricle with the catheter is often possible. Grade II Elevation of right ventricular pressure and pulmonary hyperdynamics hypertension (moderate) due to large pulmonary flow. Blood oxygen measurements will show typical right ventricular oxygen step up. If the defect is located over the Crista Supraventricularis the step up can only be seen at the pulmonary artery level. (The maximal normal step up between vena cava and atrium is 2 Vol % and between RV and MPA 0.5 Vol %; any difference over such figures must be considered abnormal.) . Grade III The pressure tends to equalize between right and left ventricles but with a still predominant left to right shunt. Significant blood oxygen step up is noted. With exertion, the normal peripheral arterial oxygen saturation is reduced. Significant pulmonary hypertension exists (close to systemic). There is marked R.V.H. Grade IV: Eisenmenger Complex
    51. 51. Angiocardiography Contrast injected into LV will localise the site & size of the defect Contrast into the pulmonary artery will demonstrate the L-R shunt. Gerbode defect : RA opacifies from the LV injection
    52. 52. perimembranous ventricular septal defects
    53. 53. Muscular ventricular septal defects
    54. 54. Subarterial VSD
    55. 55. Selective left ventricular angiogram in right anterior oblique view showing a bulge (arrowheads) of the outlet septum with a subpulmonary ventricular septal defect (arrow). Pierli C et al. Heart 2001;86:e6-e6 Copyright © BMJ Publishing Group Ltd & British Cardiovascular Society. All rights reserved.
    56. 56. CT APICAL MUSCULAR VSD MRI Flow jet (*) across the defect into the right ventricle, indicating a left-to-right shunt.
    57. 57. TREATMENT PROTOCOL SMALL VSD - No medication or surgery if asymptomatic – 75-80% close by 2 years . Observation MODERATE / LARGE VSD - Treatment of CHF Determining when to repair INTERVENTION Decompensated CHF Compensated CHF with: – Large hemodynamically significant VSD - L to R shunting with Qp/Qs > 2:1, even if asymptomatic, ideally before 1 year – Growth failure, unresponsive to medical therapy is an indication for surgery
    58. 58. Surgical correction has to be done before irreversible damage to pulmonary vasculature occurs.
    59. 59. Indications for intervention Significant VSD: symptomatic without irreversible pulmonary HTN * Qp/Qs > 1.5 * PA systolic pressure > 50 mm Hg * Increased LV and LA size * Deteriorating LV function Perimembranous VSD with more than mild AR + recurrent endocarditis. Subarterial VSD - High incidence of aortic valve prolapse/ AI Children without irreversible pulmonary HTN * significant symptoms failing to respond to medication * elective surgery (performed between 3 ~ 9 m/o) Pulmonary HTN * PA resistance < 7 Wood units * Net left-to-right shunt of at least 1.5 * Irreversible
    60. 60. SURGICAL CONSIDERATION Preoperative VSD location Avoidance of injury to conduction pathways. Operative technique needed to secure the closure 5 operative approaches - RIGHT ATRIAL TRANSPULMONARY TRANSAORTIC RIGHT VENTRICULAR LEFT VENTRICULAR
    61. 61. RIGHT ATRIAL APPROACH Most frequently used Used for – Paramembranous/ Inlet /Muscular/LV & RA types APICAL & SWISS Cheese defect – limited left apical ventricular incision may be required
    62. 62. Shallow Stitching Close to the Rim of the Ventricular Septal Defect Eliminates Injury to the Right Bundle Branch
    63. 63. VSD
    64. 64. TRANSPULMONARY ARTERY Used for repair of Conal ( Supracristal vsd) Conal Vsd – importance of patch closure Treacherous situation- Prolapsed aortic valve leaflet partially occlude the defect. Avoiding injury to Aortic valve leaflet . Repairing superior aspect of VSD Combined approach for AI may
    65. 65. TRANSAORTIC APPROACH Need for concomitant correction – aortic valvuloplasty / valvar or subvalvar stenosis. Incision- curved incision over Aortic valve commisure Absence of superior muscular or fibrous rim of the defect. Used for DORV with subaortic VSD
    66. 66. RV APPROACH 2 types- TRANSVERSE/ VERTICAL Important to examine epicardial coronary artery distribution Indications- inaccesibility from rt .atrium /pulmonary artery - defect extending into infundibular septum - presence of obstructive infundibular muscle bundles - difficulty exposing inferior margin of conal defect Patch closure by RV approach
    67. 67. Apical Muscular VSD Patch Closure via RVtomy (A) Trabeculations overlying the VSD are taken down. (B) Interrupted pledgetted sutures are placed full thickness at the superior margin of the defect, maintaining the pledgets on the left ventricular side (C) Closure of the VSD with a Dacron patch
    68. 68. LV APPROACH Rarely used Vertical / Transverse incision Limited to certain type of trabecular VSDs- multiple apical,swiss cheese Easier to patch from LV side because of smooth septum LV incision avoided to prevent long term ventricular dysfunction.
    69. 69. Interventional Options Percutaneous Device Closure – Muscular VSDs can typically be closed percutaneously
    70. 70. Flap Valve Double Patch Closure • Flap valve double patch closure of Ventricular Septal Defects in children with Increased Pulmonary Vascular Resistance
    71. 71. Much more to come Are we all still awake?
    72. 72. QUESTIONS?
    73. 73. Thank You