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4 Congenital Heart Disease

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4 Congenital Heart Disease

  1. 1. Congenital Heart Disease, (CHD) mbbs.weebly.com
  2. 2. Scientific medicine begins one of the most gifted pupils of Fabricius (1578-1657), to form the general picture of what we today call the circulation of the blood. But even he had no clear idea of the circulation in the region of the capillaries. William Harvey
  3. 3. Prof. Dr. Ludwig Rehn 1896 closure of a stab would in the right ventricle in Frankfurt am Main by Prof. Dr. Ludwig Rehn. Patient developed empyema but eventually survived.
  4. 4. Ferdinand Sauerbruch (1875-1951) A negative-pressure chamber enabling the safe opening of the chest while conducting a pneumothorax was deviced After series of test on animals, on October 1, 1903
  5. 6. Cardiac Catheterization Werner Forssmann August 29 , 1904 – June 1 , 1979 ) First Cardiac Catheterization in 1929 received The Nobel Prize in Medicine 1956
  6. 8. ( 1898 - 1986 ) was an American cardiologist , working in Baltimore and Boston, who founded the field of pediatric cardiology. Notably, she helped develop the Blalock-Taussig shunt in cooperation with Dr. Alfred Blalock and Vivien Thomas , to treat blue baby syndrome Helen Brooke Taussig, M.D.
  7. 9. the book- Congenital Malformations of the Heart in 1947 Blalock-Taussig shunt , first performed on an 11-month old baby girl on November 29,1944.
  8. 10. In 1959, she was one of the first women to be awarded a full professorship at Johns Hopkins University
  9. 11. Extracardiac Repair and Palliation <ul><li>PDA R.GROSS 08/26/38 </li></ul><ul><li>COARCT. C.CRAFORD 10/01/44 </li></ul><ul><li>S-P SHUNT BLALOCK-TAUSSIG 11/29/44 </li></ul><ul><li>PA BAND DAMMON-MULLER 1952 </li></ul>
  10. 12. PDA R.GROSS 08/26/38
  11. 14. First successful surgical repair of the heart on September 2 , 1952 by under hypothermia performed surgeries using cross-circulation, in which to take up the pumping and oxygenation functions of the patient as he was being operated on March 26 , 1954 Walton Lillehei ( 1918 – 1999 )
  12. 15. Jacqueline Noonan (1921-) Genetics of Noonan syndrome
  13. 16. Noonan Syndrome <ul><li>Noonan JA. Hypertelorism with Turner phenotype. Am J Dis Child.1968;116:373-380 </li></ul>
  14. 17. PGE & Imaging <ul><li>Early ’ 70s-M-mode echo in CHD </li></ul><ul><li>1974-First 2D images </li></ul><ul><li>1975-First reports of PGE use </li></ul><ul><li>1977-Axial Angiography </li></ul><ul><li>1978-First reports of subxiphoid 2D imaging in CHD </li></ul><ul><li>1980s-Doppler studies, fetal studies </li></ul><ul><li>Late ’ 80s-90s-TEE, intraoperative studies </li></ul>
  15. 18. New Advances <ul><li>Cardiac Imaging: </li></ul><ul><ul><li>Echocardiography </li></ul></ul><ul><ul><li>Cardiac MRI/CT </li></ul></ul><ul><li>Cardiac Intervention (Cath lab) </li></ul><ul><li>Heart Failure: </li></ul><ul><ul><li>Medications </li></ul></ul><ul><ul><li>Biventricular Pacemaker (synchronization) </li></ul></ul><ul><ul><li>Heart Transplantation/Surgery (assist devices) </li></ul></ul><ul><li>Gene Therapy … </li></ul>
  16. 19. Pediatric Cardiology: Summary <ul><li>1938-Essentially no Rx for CHD </li></ul><ul><ul><li>Pioneers-pathologists, cardiologists, surgeons, imaging experts, intensivists, interventionalists </li></ul></ul><ul><li>2008-Rx for virtually all CHD, BUT mort./morb.: </li></ul><ul><ul><li>ventricular function </li></ul></ul><ul><ul><li>arrhythmia </li></ul></ul><ul><ul><li>cns </li></ul></ul><ul><ul><li>valves/conduits </li></ul></ul><ul><ul><li>pulm hypertension </li></ul></ul><ul><li>New frontiers-causation, gene-based Rx, CHF Rx, Arrhythmia Rx, imaging, safety of CPB, PVR </li></ul><ul><li>An incredibly exciting run--much for the future </li></ul>
  17. 21. Beginner (pioneer ) in China 刘薇廷教授 陈为敏教授
  18. 22. Controlled Cross-circulation 3/26/54-7/19/55 <ul><li>NO. MORT. <2Y MORT. </li></ul><ul><li>VSD 28 8(28%) 16 6(37%) </li></ul><ul><li>TOF 11 4(36%) 5 3(60%) </li></ul><ul><li>CAVC 4 3(75%) 3 2(67%) </li></ul><ul><li>IPS 2 0 </li></ul><ul><li>45 15(33%) 24 11(49%) </li></ul>
  19. 23. Early Open Heart Surgery With Heart-Lung Machine <ul><li>C.Dennis 1952 2/2 Died </li></ul><ul><li>J.Gibbon 1953 5/6 Died </li></ul><ul><li>J.Helmsworth 1953 1/1 Died </li></ul><ul><li>D.Dodrill 1953 2/2 Died </li></ul><ul><li>G.Clowes 1953 2/2 Died </li></ul><ul><li>W.Mustard 1953/54 5/5 Died </li></ul><ul><li>17/18 94.5% Died </li></ul>
  20. 24. Definition <ul><li>CHD : the maldevelopment of heart and great vessels to cause abnormalities when the embryo period </li></ul>—— existing when being born —— incidence:0.5~1 % —— The heart: complex hollow organ —— More common : -> in children: CHD -> In adult : athero-sclerotic heart disease
  21. 25. The Cardiovascular formation Embryo development of the heart <ul><li>the primitive cardiac tube forms </li></ul><ul><li>During the first month of </li></ul><ul><li>Gestation (the second week) </li></ul><ul><li>Sinoatrium: SA </li></ul><ul><li>primitive ventricle: V </li></ul><ul><li>bulbus cordis: BC </li></ul><ul><li>Conotruncus: TA </li></ul><ul><li>2 weeks after insemination </li></ul><ul><li>formation start </li></ul><ul><li>4 weeks circulation start </li></ul><ul><li>8 weeks four chamber heart </li></ul>
  22. 26. The separation of the atrioventricular canal -> endocardial cushions -> tricuspid/ mitral inlets Formation of the separation of the heart , valves, arterial and venous system <ul><li>Sinoatrium -> right and left atria </li></ul><ul><li>primitive ventricle/ bulbus cordis </li></ul><ul><li>-> right and left ventricle </li></ul><ul><li>truncus -> aorta and pulmonary </li></ul><ul><li>The separation of the </li></ul><ul><li>atrium and ventricular </li></ul><ul><li>Formation of the </li></ul><ul><li>membranous part of </li></ul><ul><li>interventricular septum </li></ul>
  23. 27. Cardiac development Formation of a single Heart tube Formation of the heart loop
  24. 28. Cardiac development Out flow tract septation Septation of four chambers
  25. 29. 原发房隔 原发孔 继发房隔 原发房隔 继发孔 继发房隔 Atrial septation
  26. 30. Fetal development One month 5th week 6th week 8th week
  27. 32. Fetal and postnatal circulations 胎儿血循环与出生后改变
  28. 33. normal blood circulation way
  29. 34. Diagrammatic representation of fetal circulation
  30. 35. Fetal circulation
  31. 36. 通过动脉导管 通过卵圆孔 血氧含量较高 血氧含量较低 通过静脉导管 Fetal circulation 下腔静脉 右心房 右心室 左心房 左心室 升主动脉 心脑及上半身 肺动脉 降主动脉 下半身 上半身静脉血 上腔静脉 脐静脉动脉血 门静脉静脉血 母体 下半身静脉血 肺循环
  32. 37. <ul><li>Gas and nutritional requirements exchange occurs in the placenta </li></ul><ul><li>Pulmonary blood flow is very low </li></ul><ul><li>Foramen ovale and ductus arteriosus are opened </li></ul><ul><li>The blood saturation is higher in up limbs and head </li></ul>Fetal Circulation
  33. 38. <ul><li>Pulmonary ventilation, and respiration, pulmonary vascular resistance decreases rapidly, systemic blood saturation increased </li></ul><ul><li>The ductus arteriosus closed by its contraction of muscular wall, which resulted by bradykinin </li></ul><ul><li>By increasing in pulmonary blood flow, pulmonary venous return increased, combined with a decrease in pressure within the right atrium, resulted in a closing of foramen ovale </li></ul>Postnatal Circulations
  34. 39. Close of the circulation in infant after birth <ul><li>Foramen ovale(PFO) </li></ul><ul><li>—— Functional close: after birth </li></ul><ul><li>—— Anatomically close: 5 ~ 7 months </li></ul><ul><li>Ductus arteriosus(PDA) </li></ul><ul><li>—— Functional close: within 24h after birth (term infant 80%) </li></ul><ul><li>—— Anatomically close: 3 month (80%) </li></ul><ul><ul><li>one year (95%) </li></ul></ul><ul><li>Ductus venosus(PDV) </li></ul><ul><li>—— atresia to forming ligaments </li></ul><ul><li>Placenta : source of oxygen for the fetus </li></ul>
  35. 40. To compare circulation between prior and after birth 返回 A : fetal period B : after birth Gas exchange by matrix systemic circulation Gas exchange by pulmonary circulation Blood oxygen content : Mixing or upper: heart /brain ﹥ lower body Separation of the venous and arterial blood PFO/PDA/PDV Close The same pressure of the aterail and pulmonary High pulmonary resistance The lower of the pulmonary pressure and resistance The burden of the right ventricle higher The burden of the left ventricle higher A B
  36. 41. <ul><li>CHD general introduction 先天性心脏病总论 </li></ul>
  37. 42. Etiology <ul><li>Chromosomal abnormalities(10~12%) : </li></ul><ul><li>extra some or deletion of a chromosome </li></ul><ul><li>-> trisomy syndrome:Down’s (21/13/18), Turner’s </li></ul><ul><li>Single gene abnormalities (1~2%): </li></ul><ul><li>-> Marfan’s syndrome </li></ul><ul><li>Multiple gene </li></ul><ul><li>Maternal factors (1~2%). : </li></ul><ul><li>-> maternal infection </li></ul><ul><li>-> metabolic disease </li></ul><ul><li>-> drug ingestion </li></ul><ul><li>-> Alcoholism drug abuse </li></ul><ul><li>No specific cause(85%) </li></ul><ul><li>-> Surrounding environment </li></ul>
  38. 43. Incidence of Congenital Heart Disease <ul><li>0.8 – 1 % of all newborns, i n China, alone 250000 children with CHD are born each year </li></ul><ul><li>The CHD are the first causes of child mortality from 1-4 years old children until now . 30% of cases may die within first year of life </li></ul><ul><li>> 50-60% diagnosed within first month of life </li></ul><ul><li>Increasing number diagnosed prenatally </li></ul><ul><li>(>50% of complex lesions) </li></ul>
  39. 44. Relative Frequency of CHD <ul><li>Ventricular septal defect 25-30% </li></ul><ul><li>Atrial septal defect (secundum) 6-8% </li></ul><ul><li>Patent ductus arteriosus 6-8% </li></ul><ul><li>Coarctation of aorta 5-7% </li></ul><ul><li>Tetralogy of Fallot 5-7% </li></ul><ul><li>Pulmonary valve stenosis 5-7% </li></ul><ul><li>Aortic valve stenosis 4-7% </li></ul><ul><li>Transposition of great arteries 3-5% </li></ul>
  40. 45. Diagnostic approaches <ul><li>History: </li></ul><ul><li>Gestation </li></ul><ul><li>Family </li></ul><ul><li>Personal :feeding/dyspnea/ </li></ul><ul><li>delayed growth/sweating </li></ul><ul><li>Age </li></ul><ul><li>Physical examination: </li></ul><ul><li>general manifestation: </li></ul><ul><li>cardiac examination: </li></ul>BP Edema Respiratory Cyanosis/clubbing Abdomen: liver/spleen Extremities Pulse Ox /cyanosis HR/RR <ul><li>Cardiac : </li></ul><ul><li>Apex /Apex impulse </li></ul><ul><li>Thrill /Murmur: </li></ul><ul><li>—— location/character </li></ul><ul><li>Rhythm/Sound </li></ul>Inspection Palpation Percussion Auscultation
  41. 46. 患儿,男, 3 岁, TOF ,中央性青紫( + ) 患儿,女, 5 岁 单心室、单心房, 中央性青紫( + )
  42. 47. Clubbing fingers (toes)
  43. 48. Knee-chest Position Child with a cyanotic heart defect squats (assumes a knee-chest position) to relieve cyanotic spells. Some times called “tet” spells. Ball & Bindler Nurse puts infant in knee-chest position. Whaley & Wong
  44. 49. Specific examination: imaging/ hemodynamics/intervention <ul><li>Invasiveness examination: </li></ul><ul><li>X-ray </li></ul><ul><li>Electrocardiogram, ECG </li></ul><ul><li>Echocardiography, ECHO: fetal / esophageal /cardiovascular </li></ul><ul><li>—— Mainstay </li></ul><ul><li>Electron-beam CT/ Magnetic Resonance Imaging ,( MRI) </li></ul><ul><li>Invade examination: </li></ul><ul><li>Cardiac catheterization and angiography </li></ul><ul><li>Endocardium biopsy </li></ul><ul><li>Cardiac electrophysiologic </li></ul><ul><li>others </li></ul>
  45. 50. Physical Exam <ul><li>Ausculatation- The Sounds </li></ul><ul><ul><li>S1 </li></ul></ul><ul><ul><ul><li>Closure of the A-V valves </li></ul></ul></ul><ul><ul><ul><li>Single sound in early isovolumic ventricular contraction </li></ul></ul></ul><ul><ul><ul><li>Best heard in tricuspid and mitral areas </li></ul></ul></ul>
  46. 51. Physical Exam <ul><li>Auscultation </li></ul><ul><ul><li>S2 </li></ul></ul><ul><ul><ul><li>Closure of the semilunar valves </li></ul></ul></ul><ul><ul><ul><li>Physiologic splitting results from increased right heart filling and decreased left heart filling during inspiration </li></ul></ul></ul><ul><ul><ul><li>Pathologic splitting </li></ul></ul></ul>
  47. 52. auscultation <ul><ul><li>Mitral valve </li></ul></ul><ul><ul><li>Pulmonary valve </li></ul></ul><ul><ul><li>Aortic valve </li></ul></ul><ul><ul><li>Tricuspid </li></ul></ul><ul><li>Component of auscultation </li></ul><ul><ul><li>Heart rate </li></ul></ul><ul><ul><li>Heart thythm </li></ul></ul><ul><ul><li>Heart sounds </li></ul></ul><ul><ul><li>Muemurs:position/grade/phase/character/conduction </li></ul></ul><ul><li>Commonly used area of auscultation </li></ul>
  48. 53. Physical Exam <ul><li>Auscultation of Murmurs </li></ul><ul><ul><li>Murmurs are audible sound waves resulting from turbulent blood flow </li></ul></ul><ul><ul><li>Classification of murmurs </li></ul></ul><ul><ul><ul><li>Timing </li></ul></ul></ul><ul><ul><ul><li>Intensity </li></ul></ul></ul><ul><ul><ul><li>Location on chest wall </li></ul></ul></ul><ul><ul><ul><li>Duration </li></ul></ul></ul><ul><ul><ul><li>Configuration </li></ul></ul></ul><ul><ul><ul><li>Quality </li></ul></ul></ul><ul><ul><ul><li>Pitch </li></ul></ul></ul>
  49. 54. Types of Murmurs <ul><li>Systolic </li></ul><ul><ul><li>Begin with S1 and end before S2 </li></ul></ul><ul><ul><li>Classified as holosystolic, ejection, early and late systolic </li></ul></ul><ul><li>Diastolic </li></ul><ul><ul><li>Occur in the period between closure of semilunar valves and subsequent closure of A-V valves </li></ul></ul><ul><ul><li>Classified as early, mid and late diastolic </li></ul></ul><ul><li>Continuous </li></ul><ul><ul><li>Not confined to systole or diastole </li></ul></ul>
  50. 55. Types of Murmurs <ul><li>Continuous murmurs </li></ul><ul><ul><li>Begin in systole and extend up to diastole without interruption </li></ul></ul><ul><ul><li>Result from blood from a higher pressure chamber or vessel to a lower system with a persistent pressure gradient between these areas </li></ul></ul><ul><ul><ul><li>PDA </li></ul></ul></ul><ul><ul><ul><li>Left to right shunts </li></ul></ul></ul><ul><ul><ul><li>Venous hum </li></ul></ul></ul>
  51. 56. Types of Murmurs <ul><li>Systolic murmurs </li></ul><ul><ul><li>pansystolic murmurs </li></ul></ul><ul><ul><ul><li>Starts with S1 and extends up to A2/P2 </li></ul></ul></ul><ul><ul><ul><ul><li>Mitral regurgitation </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Ventricular septal defect </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Tricuspid regurgitation </li></ul></ul></ul></ul><ul><ul><li>Systolic Ejection Murmurs </li></ul></ul><ul><ul><ul><li>Ejection murmurs </li></ul></ul></ul><ul><ul><ul><ul><li>Valvular, supravalvular or subvalvular aortic stenosis </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Pulmonic stenosis </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Innocent Murmurs </li></ul></ul></ul></ul>
  52. 57. What do you hear?
  53. 58. Purposes of Imaging <ul><li>Anatomic-pathologic diagnosis </li></ul><ul><li>Hemodynamic assessment </li></ul><ul><li>(velocity, flow, pressure, stress-strain) </li></ul><ul><li>Volume, function, wall motion, torsion </li></ul><ul><li>Coronary perfusion / Metabolism </li></ul><ul><li>Tissue characterization </li></ul>
  54. 59. Chest X-ray test <ul><li>Heart size, shape </li></ul><ul><li>pulmonary vascularity </li></ul><ul><li>Cardiothoracic ratio </li></ul><ul><li>Hilar pulsation </li></ul><ul><li>Situs perversus </li></ul>
  55. 60. Image of heart at anteroposterior position
  56. 61. The X-ray diagnosis ?
  57. 62. Electrocardiogram, ( ECG ) <ul><li>Diagnostic value: </li></ul><ul><li>Arrhythmia </li></ul><ul><li>The situation of cardiac conduction </li></ul><ul><li>H ypertrophy of atrium and ventricle </li></ul><ul><li>Myocardial ischemia </li></ul><ul><li>Speciality of Infantile ECG </li></ul><ul><li>heart rate and age are inverse proportion in children </li></ul><ul><li>The QRS wave of right ventric take predominant in Infant </li></ul><ul><li>T wave is comparatively large in variation </li></ul>
  58. 63. ECG
  59. 64. Echocardiography , (ECHO) <ul><li>Easy, non-invasive, accurate, real-time , </li></ul><ul><li>Anatomic and physiologic information </li></ul><ul><li>Changed practice of pediatric cardiology </li></ul>
  60. 65. Echocardiography - Modalities <ul><li>M-mode / 2-D / 3-D </li></ul><ul><li>Doppler / color Doppler </li></ul><ul><li>Trans-thoracic, trans-esophageal, </li></ul><ul><li>trans-abdominal, trans-vaginal, </li></ul><ul><li>intra-cardiac, intra-vascular </li></ul>
  61. 66. M - ECHO
  62. 67. Echocardiography
  63. 68. Echocardiography
  64. 69. B/Color /Doppler ECHO
  65. 70. New Development in Echo <ul><li>Imaging: edge detection/auto-measurement </li></ul><ul><li>Doppler: 3-D flow / stress-strain </li></ul><ul><li>Contrast echo: coronary perfusion </li></ul><ul><li>Fetal Echo: >16 weeks </li></ul>
  66. 71. 3-D Echocardiography
  67. 76. Purposes of Catheterization <ul><li>Anatomic diagnosis </li></ul><ul><li>Hemodynamic assessment </li></ul><ul><li>Interventional procedure </li></ul>
  68. 77. Equipment <ul><li>Biplane monitor / Cine with digital subtraction </li></ul><ul><li>Patient monitoring : EKG, BP, pulse oximeter </li></ul><ul><li>Physiologic signal amplifier and recording device </li></ul><ul><li>Blood gas, O 2 consumption, Dye/ Thermodilution </li></ul><ul><li>Emergency treatment tools : </li></ul><ul><li>Room for Others : anesthesia, echo, exercise </li></ul>
  69. 78. Catheterization Room Fluoroscopic Monitor
  70. 81. Cardioangiography
  71. 83. <ul><li>1. left to right shunt ( potential cyanotic type ) </li></ul><ul><li>-左向右分流型(潜伏紫绀型) VSD 、 ASD 、 PDA </li></ul><ul><li>2. right to left shunt ( cyanotic type ) </li></ul><ul><li>-右向左分流型(紫绀型) </li></ul><ul><li>TOF D-TGA </li></ul><ul><li>3. without shunt ( Acyanotic type ) </li></ul><ul><li>- 无分流型(无紫绀型) PS 、 AS </li></ul>Classification of CHD
  72. 84. Electrophysiologic Study
  73. 85. Other Imaging Tools <ul><li>Magnetic Resonance Imaging (MRI) </li></ul><ul><li>CT / Electron-beam CT (EBCT) </li></ul><ul><li>Radionuclide / SPECT </li></ul><ul><li>Positron Emission Tomography </li></ul>
  74. 86. Magnetic Resonance Imaging <ul><li>Sectional still image/ cine image/ 3-D </li></ul><ul><li>Flow information / volume flow </li></ul><ul><li>Less window dependant / post-op study, older age / functional evaluation </li></ul>
  75. 87. Magnetic Resonance Imaging Spin echo Gradient echo Velocity encoded
  76. 88. MRI and coarctation
  77. 89. MRI and TOF
  78. 90. Computerized Tomography
  79. 91. CT and Aortic arch anomalies
  80. 92. Radionuclide Study
  81. 93. Radionuclide Study