Total anomalous pulmonary venous connection

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Total anomalous pulmonary venous connection

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Total anomalous pulmonary venous connection

  1. 1. TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION Fifth most common cause of cyanotic CHD
  2. 2. TAPVC IS CYANOTIC CHD
  3. 3. TIMELINE • 1798 :1st described by Wilson • 1956:1st ICR by Lewis and Varco In 1798, the Philosophical Transactions of the Royal Society of London published “A description of a very unusual formation of the human heart.” A 1942 review of 100 cases of anomalous pulmonary venous connections included 35 examples that were total, a term that applies when all four pulmonary veins connect anomalously to a systemic venous tributary of the right atrium or to the right atrium proper but have no connection to the left atrium. The malformation is isolated in approximately two thirds of patients so afflicted occurs in approximately four to six per 100,000 live births,57 and accounts for about 2% of deaths from congenital heart disease in the first year of life.
  4. 4. RT3D • http://www.youtube.com/watch?feature=player_embedded&v=RMIKq92dfHE#t=101 The Pulmonary Veins, which carry blood back to the heart after it has circulated through the lungs, are not connected to the left atrium. Instead they are connected to one of the veins from the main circulation so that the blood returning from the lungs drains back to the right side of the heart. The affected babies may be blue or show signs of heart failure. Most of them require surgical repair in the newborn period
  5. 5. INCIDENCE 0.6 to 1.2 per 10,000 live births 0.7 and 1.5 percent of all CHD A strong male preponderance of 3:1 Birth weight was less than 2500 g in 16.2% Gestational age was less than 38 weeks in 18.9% Intrauterine growth retardation occurred in 26.8% 68% of these patients were diagnosed as neonates
  6. 6. WHAT CAUSES IT • NOT KNOWN
  7. 7. EMBRYOLOGY NORMAL TAPVC  Lung buds are formed from the primitive foregut  Lung buds veins drain as splanchnic plexus initially drains into the common cardinal and umbilicovitelline venous systems  Splanchnic plexus differentiates into the primitive pulmonary vascular bed  Primitive left atrium forms a primordial evagination (common pulmonary vein) that grows into and joins the pulmonary portion of the splanchnic plexus  Primitive pulmonary venous system separates from the cardinal and umbilicovitelline veins  Common pulmonary vein become the two right and two left pulmonary veins, each of which enters the LA through a separate orifice  Failure of the left atrium to link to the pulmonary venous plexus, which results in the retention of connections to the primitive cardinal and umbilicovitelline drainage systems  The anatomic variants of TAPVC are dependent upon which connections are retained  The cardinal venous system provides connections to the innominate vein, right atrium, superior vena cava, or azygous vein  Umbilicovitelline system to the portal or hepatic vein, or inferior vena cava.
  8. 8. WHERE DOES ANOMALOUS PV GOES
  9. 9. NATURAL COURSE Severe obstruction —Die within the first month. Restrictive interatrial communication — mortality rate of about 80 percent in the first year of life out of CHF/FTT/LRTI Unobstructed —Some only mild symptoms with exertion, but most develop progressive RHF and PAH
  10. 10. CLASSIFICATION Classifications take into account three features: (1) the pathway by which pulmonary venous blood reaches the right atrium; (2) the presence or absence of obstruction along the course of the pathway; and (3) the nature of the interatrial communication. The most widely used clinical classification recognizes supradiaphragmatic connections with or without obstruction and infradiaphragmatic or infracardiac connections that are always obstructed.
  11. 11. The most common classification system was originally described by Darling et al. consists of four types : Supracardiac : Cardiac : Infracardiac :Mixed
  12. 12. SUPRACARDIAC:SNOWMAN SIGN- 49%
  13. 13. INFRADIAPHRAGMATIC :OBSTRUCTIVE[GROUND GLASS]-25%
  14. 14. CORONARY SINUS[CARDIAC] :16%
  15. 15. MIXED:9%
  16. 16. MALPOSITION OF SEPTUM PRIMUM
  17. 17. PATHOPHYSIOLOGY UNOBSTRUCTIVE  Admixture of pulmonary and systemic venous flow  RA and RV volume loading  RV pressure load if ASD restricted  PBF increases  PAH  LA and LV under filled  Most have PFO  OBSTRUCTIVE       Post capillary pulmonary venous congestion increased pulmonary lymphatic flow Reflex pulmonary arterial vasoconstriction Increase in PVR Decrease in PBF A lower volume of saturated blood in the venous mixture  Decrease in the CO  Worse systemic oxygen saturation
  18. 18. EQUALIZATION OF 4CHAMBERS SATURATION PRESSURE NONOBSTRUCTIVE TAPVC CONSTRICITIVE PERICARDITIS
  19. 19. LEVEL OF OBSTRUCTION SUPRACARDIA C CARDIAC  Hemodynamic Drainage to RA vise site obstruction  CC to VV junction  SVC/AZYGOS DRAINAGE SITE  Restrictive ASD INFRACARDIAC Drainage to portal or IVC
  20. 20. WHO COULD REPLACED ANGIOGRAM? Echocardiography  CT MR angiography
  21. 21. ASSOCIATED • Single ventricles • Heterotaxy with asplenia or polysplenia https://circ.ahajournals.org/content/122/25/2718.full
  22. 22. PHYSICAL EXAMINATION UNOBSTRUCTED         FS S2:RV over load right ventricular heave ESM :^PBF EDM :^ TV blood flow S3 gallop Hepatomegaly :RHF Tachypnea Variable degrees of cyanosis OBSTRUCTED [starts at birth]        P2: loud ESM :less Continuous murmur at obstruction Cyanosis Hypotension/low volume Hepatomegaly Respiratory distress
  23. 23. DD Atrial Septal Defect Hypoplastic Left Heart Syndrome Mitral Stenosis, Congenital Single Ventricle Transposition of the Great Arteries Truncus Arteriosus
  24. 24. TREATMENT UNOBSTRUCTED surgical redirection can be performed within the first month of life  PGE1  BAS  surgery should be undertaken emergently.
  25. 25. SURGICAL OUTCOMES https://circ.ahajournals.org/content/122/25/2718.full
  26. 26. ONLY STUDENTS CAN SMELL External link https://circ.ahajournals.org/content/122/25/2718.full

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