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Topic Critical Congenital Heart Disease
Critical Congenital Heart Disease (CCHD) refers to several heart defects present at birth that require intervention. Some key points: - CCHD includes defects where blood flow depends on an open ductus arteriosus after birth, such as Tetralogy of Fallot. - Clinical presentation varies but may include cyanosis, heart murmur, respiratory distress. Diagnosis involves tests like echocardiogram, EKG, chest x-ray. - Management depends on the specific defect but may include prostaglandin E1 to keep the ductus arteriosus open, then surgery to repair the anatomical issues. Early detection through newborn pulse oximetry screening can help identify cases
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Topic Critical Congenital Heart Disease
- 1. Critical Congenital Heart Disease SasathornTharapoom Ubolwan Chansingthong 5th year medical student Advisor Yasinee Apiraknapanon M.D. Department of Pediatric, Naresuan University Hospital
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- 4. Flow Chart ofFetal Circulation
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- 6. 1. Placenta Removal2. Breathing Lung Expansion Pulmonary Artery Dilatation PVR Ductus Venosus Closure Flow to RA RA Pressure PBF LA Pressure Close Foramen Ovale SVR Flow to LA PDA Flow PaO2 Circulatory Adjustment After Birth
- 7. 1. Placenta Removal2. Breathing Lung Expansion Pulmonary Artery dilatation PVRPBF SVR PDA flow PaO2 Closure of Ductus Arteriosus PGE Lt. to Rt. Rt. to Lt. Shunt Ductal tissue contraction Close Ductus Arteriosus
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- 9. Fetal vs. InfantCirculation • Fetal • Low pressure system • Right to left shunting • Lungs non-functional • Increased pulmonary resistance • Decreased systemic resistance • Infant • High pressure system • Left to right blood flow • Lungs functional • Decreased pulmonary resistance • Increased systemic resistance
- 10. Approach to Critical CongenitalHeart Disease (CCHD)
- 11. Classification of CCHD •Ductal dependent for pulmonary blood flow • Ductal dependent for systemic blood flow • Ductal dependent for blood mixed • Total anomalous pulmonary venous return with obstruction
- 12. Clinical Presentation in CCHD •Can be asymptomatic in newborn period • Tachypnea o Many other etiologies • Absent or weak femoral pulses o Only occurs after the PDA has closed • Murmur o Occur in up to 77% of newborns, often not pathologic o Some CCHD lesions do not have murmurs o Some forms of CCHD do not have murmur until pulmonary vascular resistance falls • Cyanosis from hypoxia • Shock
- 13. Clinical Presentation in CCHD Clinical •Ductal dependent for pulmonary blood flow • Ductal dependent for blood mixed • Total anomalous pulmonary venous return with obstruction • Cyanosis from hypoxia • + Tachypnea, heart murmur • Ductal dependent for systemic blood flow • Shock • Absent or weak femoral pulses (PDA closed) • + Tachypnea, cyanosis, heart murmur
- 14. Cyanosis • Visual recognitionof hypoxia – not easily detected • Dependent on hemoglobin levels • Central cyanosis : deoxygenated Hb ≥ 5𝑔/𝑑𝑙 • Peripheral cyanosis : poor perfusion • Differential cyanosis : preductal SpO2 > postductal SpO2 • Reversed-differential cyanosis : postductal SpO2 > preductal SpO2
- 15. Cyanosis in Newborn •Heart : CCHD • Lungs o Parenchyma : MAS, RDS, pneumonia, atelectasis etc. o Airway : choanal atresia, Tracheal stenosis, etc. o Extrinsic lung compression : pneumothorax, pleural effusion, diaphragmatic hernia, etc. o Hypoventilation : CNS/neuromuscular disease, sedation, sepsis etc. • PPHN • Abnormal hemoglobin : methemoglobinemia • Metabolic : hypothermia, hypoglycemia, neonatal sepsis (poor tissue perfusion)
- 16. Initial Evaluation and Management •ABC : airway, breathing and circulation • Oxygen supplement : keep SpO2 75-85% • PGE1 • Pre and postductal SpO2 o Differential cyanosis (preductal SpO2 > postductal SpO2) : PPHN, severe coarctation of aorta with PDA o Reverse differential cyanosis (postductal SpO2 > preductal SpO2) : D-TGA with pulmonary hypertension, D-TGA with coarctation of aorta • Hyperoxia test ในผู้ป่วยที่เขียว
- 17. Acute Mx. • ABC Hx,PE • CCHD : heart murmur, cardiomegaly hepatomegaly • PPHN : respiratory distress, labile cyanosis Inv. • CXR • Hct, blood glucose, ABG • Hyperoxia test, hyperventilation, pre-post duct PaO2
- 18. EKG •Suspected CCHD PGE1 •Can’t excludeductal dependent lesion •Consult & supportive treatment
- 19. Test Method Result(ABG) Suggest Hyperoxia Exposed to 100% FiO2 for 5-10 min • PaO2 increases to >100mmHg • PaO2 increases to < 20mmHg • Pulmonary parenchymal disease • PPHN/CCHD Hyperoxia- Hyperventilation Mechanical ventilator with FiO2 100% RR 100-150 bpm • PaO2 increases to >100mmHg without hyperventilation • PaO2 increases at a critical PCO2 often to <25mmHg • No increase in PaO2 despite hyperventilation • Pulmonary parenchymal disease • PPHN • CCHD
- 20. Test Method Result(ABG) Suggest Simultaneous preductal- postductal PO2 Compare PO2 of right arm/shoulder to that lower abdomen/extremities • Preductal PO2 ≥15 + postductal PO2 • Rt. to Lt. shunt at PDA
- 21. Central Cyanosis Hyperoxia test NotImproved Hyperoxia-Hyperventilation test Improved Improved Not Improved PPHN CCHDEcho
- 22. Pulse Oximetry forCCHD (CCHD Screening) • Pulse oximetry can identify hypoxemia that is not visible clinically • Hypoxemia occurs in many forms of CHD • Can also identify differences in pre and post ductal saturations • Pulse oximetry is quick, easy, and safe
- 24. Primary Targets for Screening •Hypoplastic Left Heart Syndrome • Pulmonary Atresia • Tetrology of Fallot • Total Anomalous Pulmonary Venous Return • Transposition of Great Arteries • Tricuspid Atresia • Truncus Arteriosus
- 25. Routine Pulse Oximetry Screening PROSCONS • Earlier detection of CHD, earlier treatment • Decrease in mortality • Detection of other conditions (PPHN, PNX, PNA, early sepsis) • Low risk of harm, non- invasive • Cost? • False reassurance if negative screen • False Positives • Parental and staff anxiety • Elevated use of healthcare • Cost?
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- 27. Ductal Dependent Pulmonary BloodFlow • Obstruction to pulmonary blood flow • most common causes : right-sided obstructive lesion • Need PDA for pulmonary blood flow • Presentation o Progressive cyanosis o PE : single S2 + murmur o Chest X-ray : decreased PVM
- 28. Ductal Dependent Pulmonary BloodFlow • Common lesions o Tetralogy of Fallot o Double outlet right ventricle with pulmonary stenosis o Single ventricle with pulmonary stenosis o Pulmonary atresia with intact ventricular septum o Pulmonary atresia with VSD o Tricuspid atresia with pulmonary stenosis
- 29. Management • PGE1 0.05-1mcg/kg/min : increase PBF • Stabilizes infant : until surgical intervention • Assisted ventilation and increased environmental oxygen • SpO2 75-85% are adequate
- 30. PGE1 • Dose 0.05-1mcg/kg/min oStarting dose of PGE1 is 0.05 mcg/kg/min IV o If no improvement, ↑ dose to 0.1mcg/kg/min o usual maintenances dose of PGE1 is 0.025 mcg/kg/min • Complications of PGE1: o Apnea : direct effect of PGE1 on the CNS • Need assisted ventilation immediately after PGE1 has been started o Hypotension : peripheral vasodilation, decreased cardiac output • continuous measurement of arterial blood pressure o Fever o Irritability, abnormal EEG and seizures o Diarrhea o hypertrophy of gastric mucosa : longer term effects
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- 32. TOF • 5-10% ofCongenital heart disease • Most common cyanotic heart disease • Conotruncal maldevelopment • Anterior displacement of conus septum
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- 35. 1 1 1 0.4 0.6 0.6 0.6 1 Qp : Qs 0.6: 1 Left heart Decreased volume Normal pressure Right heart Normal volume Increased pressure
- 36. TOF o RVOT obstruction oRV pressure ~ LV pressure o Rt. to Lt. shunt (through VSD) cyanosis • Severity depends on : RVOT obstruction, collateral vessel
- 37. Signs & Symptoms •Central Cyanosis • Dyspnea on exertion • Squatting position • Clubbing fingers • Severe cyanosis : tachypnea, single S2 (severe PS) • SEM at mid LUSB (turbulent flow ผ่าน RVOT) • No VSD murmur (pressure RV ~LV) • Hypoxic spells : severe cyanosis, hyperpnoea, PS murmur เบาสั้นลง • Acyanotic TOF (pink TOF) : heart murmur
- 38. Vicious Cycle ofHypoxic Spell (Systemic vascular resistance)
- 39. Investigation • Chest X-ray •EKG • Echocardiogram • Cardiac catheterization
- 40. CXR • Pulmonary vascularmarking • Boot shaped heart (RVH)
- 41. Right axis deviation, RVH: Tall R wave with upright T wave in right chest lead, Deep S wave in left chest lead
- 42. Echocardiogram • VSD • Overridingaorta • RVOT obstruction : infundibular stenosis/valvular PS • Turbulent flow • peak flow velocity pressure gradient • Main pulmonary artery and bifurcation characteristics • Coronary artery abnormality • Others abnormality : ASD, PDA, collateral vessels
- 43. Cardiac Catheterization • Forsurgery • Distal pulmonary artery, collateral vessels, abnormal coronary artery • Hemodynamic o Ascending aorta : desaturation
- 44. Treatment • Medical treatment: hypoxic spell • Surgery repair anatomical defect : indication o Decreased exercise tolerance o Increased hypoxic spells o Polycythemia
- 45. Hypoxic Spells Management 1.Calm down 2. Knee chest position ( systemic vascular resistance , systemic venous return) 3. Oxygen supplement 4. MO 0.1 mg/kg ( systemic venous return) 5. IV Fluid (correct dehydration) 6. NaHCO3 1-2 mEq/kg ( acidosis when spell >10-15 min) 7. Propranolol 0.1mg/kg + NSS up to 10 ml IV in 5-10min ( infundibular spasm) 8. Correct anemia 9. Phenylephrine HCl 10. Propranolol 1-2MKday o 3-4 times/day IV (prevent spell)
- 46. Surgical Management • Severecyanosis (Hct >65%) : palliative surgery (systemic to pulmonary shunt) o At age 6 month echocardiogram, cardiac catheterization ประเมินขนาด PA o ถ้า PAขนาดใหญ่พอ total correction/เล็กทา shunt เพิ่ม • Moderate cyanosis : พิจารณาผ่าตัดตามขนาด PA • Mild cyanosis : total correction (สามารถทาได้เนื่องจาก PA มี ขนาดใหญ่พอ) o VSD closure o ขยายทางเดินเลือดจาก RV Lungs
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- 49. Pulmonary Atresia withVSD • Extremely severe form of TOF • Incomplete septation conus cordis VSD • Abnormal septation of truncus arteriosus atretic pulmonic valve, abnormal pulmonary trunk • 70% PBF ผ่าน PDA, 30% PBF จาก systemic collateral artery
- 50. Lung bud Paired dorsala. Intersegmental a. Pul. vas. plx. 6th aortic arch Pulmonary trunk RPA,LPA PA Bronchial a. Dorsal a. collateral
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- 54. Signs & Symptoms •Cyanosis and dyspnea in a few hours/days after birth • O2sat 90-92% (nonsevere hypoxia) • Single S2 • No murmur • SEM/continuous murmur of PDA at LUSB
- 55. Investigation • EKG :right axis deviation, RVH • CXR : decreased PBF, concave/flat pulmonary trunk • Echocardiogram : o PDA, collateral a. o VSD o Overriding aorta o ไม่พบ pulmonary outflow จาก RV o Atretic/hypoplastic pulmonary trunk o Cardiac catheterization : collateral artery
- 56. Treatment • Severe cyanosis oPGE1 0.05-1 mcg/kg/min IV 0.01-0.03 mcg/kg/min o Systemic to pulmonary shunt/PDA stenting • Surgery o Palliative surgery 2-3 ปีก่อนทา total correction o Total correction : at age 3-5 year • VSD closure • Ligate systemic to pulmonary shunt
- 57. Double Outlet Right Ventricle(DORV) with Pulmonary Stenosis
- 58. DORV • Cyanotic heartdisease • 0.8-1% of congenital heart disease • Aorta and pulmonary artery arise from RV • No arteries arise from LV
- 59. DORV • Conotruncal maldevelopment •Incomplete septation of conus cordis • the aortic and mitral valves are separated by a smooth muscular conus • No leftward shift of LVOT structure
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- 61. Subaortic VSD คล้าย Large VSD(Left to right shunt มาก) เขียวไม่มาก O2sat 90-96% Heart failure + PHT
- 62. คล้าย d-TGA withVSD เขียวมาก Heart failure + PHT Subpulmonic VSD
- 63. Doubly committed VSD Remoted VSD คล้าย Large VSD เขียวมากกว่าsubaortic น้อยกว่า subpulmonic Heart failure + PHT
- 64.
- 65. Signs & SymptomsDORV/PS • Hypoxia cyanosis dyspnea • Severity depends on : severity of PS, relationship with VSD • PE : o Single S2 o SEM at LUSB (PS murmur) may transmit to back
- 66. Investigations • CXR o Normalheart size o Decreased pulmonary vascular marking as in TOF • EKG o Right axis deviation o RVH ± RAE (in severe PS) • Echocardiogram o Relationship of VSD and great arteries o No LV outflow o VSD o Turbulent flow ผ่าน subvalvular, pulmonary valve
- 67. Treatment • Treat hypoxicspell • Surgery o Small PA, severe PS, severe cyanosis • Palliative Blallock-Taussig shunt correction surgery o Subaortic VSD/doubly committed VSD • ปิด VSD ด้วย patch/graft • แก้ไข PS (เหมือน TOF) o Subpulmonic VSD/remote VSD • ปิด VSD แบบ intraventricular tunnel • Homograft valve conduit ระหว่าง RVOT, MPA o DORV with PS with restrictive VSD • ขยาย VSD intraventricular tunnel repair
- 68. Tricuspid Atresia with PulmonaryStenosis
- 69. Tricuspid Atresia with PulmonaryStenosis • SVC/IVC RA TR obstruction need associated lesion RV • Associated lesion o PDA o PFO o ASD o VSD o Others : TGA, PS
- 70. . Normal related Greatarteries d-TGA PA PS No PS
- 72. Sings & Symptoms •Severity depends on PBF (ถ้ามากจะเขียวน้อย) • Cyanosis and dyspnea in 1st month after birth • Hepatomegaly • Heart sound : o Single S2 o SEM at LUSB (PS/PDA/relative PS) o SEM mid LSB (restrictive VSD)
- 73. Investigation • Chest X-ray oProminent right atrial border o Decreased pulmonary vasculature • EKG • Echocardiogram o RAE, PFO/ASD, atrial septum โป่งไปทางซ้าย o Hypoplastic RV o Tricuspid valve ไม่มีรูเปิดลงไป RV
- 74. EKG Left superior QRSaxis RAE, LVH
- 75. Treatment • Supportive treatment •Treat hypoxic spell • PGE1 • เลือดไปปอดน้อย (PA/PS), เขียวมาก : systemic to pulmonary shunt • Age 6 month o Cardiac catheterization : PVR, PAP evaluation o Bidirectional superior cavopulmonary connection (Bidirectional Glenn shunt) : SVC PA • Age 3 year : Fontan type operation : SVC, IVC PA
- 76. Pulmonary Atresia with IntactVentricular Septum (PA/IVS)
- 77. PA/IVS • 1% ofCHD • Well-formed RV, tricuspid valve • Inflammatory process at pulmonic valve fibrosis • Hypoplastic RV • Need PDA, PFO • Tripatite : TR ร่วมบ่อย • Severity : PDA, PVR 2. Outlet 1. Inlet 3. Trabecular DA
- 78. RVO obstruction RVsystolic pressure แรงดันย้อนกลับจาก RV ไป Coronary sinusoid pressure injury effect to coronary a. coronary a.: Intimal, medial thickening Proximal coronary a. stenosis Myocardium
- 79. Sings & Symptoms •Cyanosis and dyspnea in a few hours/days after birth • Normal lung sound • Hepatomegaly (flow ผ่าน PFO ไม่สะดวก RA pressure สูง) • Heart sound : o Single S2 o Most no murmur o Systolic murmur (flow ผ่าน PDA ที่กาลังจะปิด) o PSM/SEM of tricuspid regurgitation น้อย
- 80. CXR • Normal heartsize • Decreased PVM • Concave Pulmonary trunk • Prominent RA border
- 81. Left axis deviation,RAE, LVH
- 82. Echocardiogram • RAE • HypoplasticRV • Atrial septum โป่งไปทาง LA • Right to left shunt ผ่าน PFO • TR • Coronary sinusoid • Ventriculocoronary artery connection in RV • PA, small MPS/RPA/LPA, PDA • Right to left shunt ผ่าน PDA
- 83. Treatment • PGE1 0.05-1mcg/kg/min • Definitive repair : เลือกตามลักษณะ RV o Biventricular repair o Univentricular repair o One and a half ventricle repair o Transplantation • Catheter intervention o Tripatite, tricuspid valve, RV ขนาดใหญ่พอ
- 84. Single Ventricle with PulmonaryStenosis
- 85. Single Ventricle • 1%of CHD • ความผิดปกติของการเคลื่อนย้าย AV canal • Double inlet ventricle
- 88. Signs & Symptoms •Hypoxia cyanosis dyspnea • Severity depends on : severity of PS • PE : o Single S2 o SEM at LUSB (PS murmur)
- 89. Investigation • CXR o Normalheart size o Normal/decreased pulmonary vasculature • EKG o Superior axis deviation (common AV valve) o Ventricular hypertrophy o Abnormal Q wave • ECHO o Ventricular morphology o AV valve o Associated cardiac anomaly : e.g. PS, PA
- 90. Right superior axis DeepS in V1, V2, V4 Deep Q in V5, V6
- 91. Treatment • PGE1 • Surgery oSystemic to pulmonary artery shunt o Bidirectional Glenn operation at 6-8 month o Fontan type operation at 3-5 year
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- 93. Ductal Dependent Systemic BloodFlow • Critically obstructed systemic circulation • Neonate who present with signs of poor systemic circulation need to be rapidly evaluation and categorized as having CHD or other diagnosis such as sepsis or metabolic disease • Neonate with congenital obstructive left heart syndrome or left ventricular outflow tract will usually present in the first few weeks of life as their ductal dependent systemic circulation become compromised with the closure of the PDA
- 94. Ductal Dependent Systemic BloodFlow • Finding in neonates with cardiogenic shock o Tachycardia o Hypotension o Diaphoresis o Poor perfusion (dusky, mottled) o Oliguria o Acidosis
- 95. Management • Maintain anairway (they can quickly tire and apnea) • Place the patient on oxygen and cardio-pulmonary monitoring (be sure to have a bag valve mask and oxygen already flowing) • Vital signs should include pre & post ductal pulse oximetry and blood pressure • Immediate two IV/IO access points
- 96. Management • When concernedabout cardiogenic shock only 10 mL/kg of crystalloid should be initially be given • EKG and portable CXR should be done as soon as possible • Bedside initial lab : glucose, CBC, basic metabolic panel, blood culture and ABG • Hyperoxia test
- 97. Management • Neonate whothought to have a ductal dependent lesion is to immediately start an infusion of PGE1 to re-open/maintain the ductus 0.05-0.1 mcg/kg/min IV route o Major side effect is apnea • If not impossible to immediately rule out septic shock, antibiotics should be given as soon as possible. With plan LP as soon as the pt. is stabilized • Consult pediatric cardiologist and echocardiogram
- 98. Differential Diagnosis • Aorticstenosis • Coarctation of the aorta • Interruption of the aortic arch • Hypoplastic left heart syndrome (HLHS)
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- 102. Critical Aortic stenosisin NB เลือดไป aorta น้อย LA pressure สูง Pulmonary congestion เลือดบางส่วนผ่าน foramen ovale มี ductus arteriosus เลือดจาก Pulmonary artery จึงไป aorta ได้ ทารกจึงมี cardiac output เพียงพอ ไม่เหนื่อย Rt to Lt shunting
- 103. Clinical Manifestation • CriticalAS in neonate o Tachypnea o Irritable o Poor feeding o Cold extremity
- 104. Physical Examination • Systolicthrill at RUSB, suprasternal notch, carotid artery both side • SEM at LUPSB, RUPSB radiate to neck • Valvular AS : systolic ejection click จาก stenotic aortic valve • Supravalvular AS : BP Right arm > Left arm and lower extremities (Coanda effect) • Subvalvular AS : hypertrophic valve cusp Aortic regurgitation diastolic blowing murmur at LUPSB
- 105. Investigation • ECG: o Nonsevere AS : normal EKG or LVH o Severe AS : LVH and strain pattern • Chest X-ray o Cardiomegaly • Echocardiogram • Cardiac catheterization
- 106. Treatment • Valvular AS: o Asymptomatic Mild Moderate AS : observe • if doppler echo peak pressure gradient ≥ 70 mmHg or • peak velocity > 4.2 m/s o Severe valvular AS • Start with balloon aortic valvuloplasty • Surgical if cannot or fail cardiac cath Cardiac catheterization and balloon aortic valvuloplasty Indication for Balloon aortic valvuloplasty 1. critical AS in neonate 2. peak gradient > 60 mmHg 3. Symptoms (angina, syncope, and/or dyspnea on exertion) and a transvalvular peak- to-peak aortic valve gradient at catheterization greater than 50 mmHg
- 107. Treatment • Supravalvular ASand subvalvular AS : only surgery • Valvular AS : o Normal size of aortic annulus : open aortic valvulotomy under inflow occlusion or under cardiopulmonary bypass o Small aortic annulus : Ross-Konno or Ross’s operation • Supravalvular AS : o Dilate ascending aorta under cardiopulmonary bypass • Subvalvular AS : o Myectomy
- 109. Coarctation of TheAorta
- 111.
- 112. Development of Coarctation •Abnormal development of left 4th and 6th aortic arches • No real impact prior to birth due to presence of PDA unless there is fetal closure
- 113. Development of Coarctation Ductustissue theory Hemodynamic theory • Due to a migration of ductus smooth muscle cells into the periductal aorta with subsequent constriction and narrowing of the aortic lumen • Evident when ductus closes • Reduced intrauterine blood flow causes underdevelopment of aortic arch • Results from reduced volume of blood flow through the fetal aortic arch and isthmus
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- 115. Fetus LV pressure=RV pressure Due toductus arteriosus Neonate ductus arteriosus closed LV pressure increase LV pressure overload proximal artery HT Upper ext. hypertension Lower ext. hypotension
- 116. Ductus closure LV pressureoverload : immediately Pulmonary congestion low output : distal to coarctation Severe HF with oliguria
- 117. Clinical Manifestation • Asymptomatic oPersistent patent ductus arteriosus (PDA) o Not severe coarctation • Pale, irritable, diaphoretic, and dyspneic with absent femoral pulses • Heart failure and/or shock when the PDA closes o Severe coarctation
- 118. Physical Examination • Absentor delayed femoral pulse (when compared with the brachial pulse). • Murmur o May associated with other cardiac defects • Differential cyanosis o Severe coarctation of the aorta o Large PDA with a right-to-left shunt into the descending thoracic aorta • Hepatomegaly
- 119. Investigation • ECG: o Isolatedcoarctation normal EKG or LVH o Coarctation with VSD and PDA RVH or combined ventricular hypertrophy • Chest X-ray o Cardiomegaly o Figure of 3 o E sign or reverse 3 sign (Barium swallow) • Echocardiogram • Cardiac catheterization
- 121. Treatment • Medical treatment oClinical heart failure : inotropic drug, diuretic drug before surgery • Surgical treatment o Asymptomatic isolated coarctation if hypertension • Coarctation repair • Balloon angiography • Coarctation stenting o Coarctation with VSD • small VSD : coarctation repair alone • Large VSD : coarctation repair with pulmonary artery banding • Staged repair
- 124.
- 126. LV Prox. IAA RV PA PDA Distal IAA O2 sat upper ext. > lower ext. PDA closure => Decrease pulmonary vascular resistant Increase pulmonary blood flow BP upper>lower ext.
- 127. Large VSD RV pressure= LV pressure PDA closure Decrease pulmonary vascular resistant Increase pulmonary blood flow Heart failure and Pulmonary congestion Lower extremities - Low output - Acidosis - Oliguria
- 128. Clinical Manifestation • WhenPDA was closed interrupted aortic arch may present with o Dyspnea o Tachypnea o Cold extremities
- 129. Physical Examination • Tachypnea •Dyspnea • Tachycardia • Weak or absent femoral pulses • Differential cyanosis • Crepitation both lung • S2P loud • SEM grade 2-3 at mid left sternal border • Hepatomegaly
- 130. Investigation • EKG o RVH(upright T wave and tall R wave) • Chest X-ray o Cardiomegaly o Increased pulmonary vascular marking o Pulmonary venous congestion • Echocardiogram • Cardiac catheterization
- 131. Treatment • Medical treatment oClinical heart failure : inotropic drug, diuretic drug before surgery • Surgical treatment
- 133.
- 135. LA PFO RA RV PA PDA aorta Mixed blood
- 136. PDA closure Decrease pulmonary vascularresistant Increase pulmonary blood flow Decrease systemic blood flow Low output&Shock
- 137. Clinical Manifestation –With ASD • Typically have a "honeymoon" period (adequate systemic perfusion) • As the PDA begins to close, infants become symptomatic o Systemic perfusion : diminished peripheral pulses o Pulmonary blood flow hypotension, acidosis, and respiratory distress o Symptoms can rapidly progress from cyanosis, increased respiratory distress, and poor feeding to heart failure and cardiogenic shock
- 138. Clinical Manifestation – WithoutASD • Severe cyanosis and respiratory distress • Quickly develop cardiogenic shock and die
- 139. Physical Examination • Tachypnea •Dyspnea • Tachycardia • Weak or absent peripheral pulses • Hypotension • Blood pressure upper = lower ext. • Mild cyanosis • Crepitation both lungs • Active precordium • Single and loud second heart sound • SEM grade1-2 at left sternal border • Hepatomegaly
- 140. Investigation • EKG o RVH(m/c) or LVH (rare) • Chest X-Ray o Cardiomegaly o Pulmonary venous congestion • Echocardiogram • Cardiac catheterization
- 141. Treatment • Medical treatment oClinical heart failure : inotropic drug, diuretic drug before surgery o Avoid oxygen if no indication (can cause pulmonary vasodilation) • Surgical treatment o The three steps consist of the following procedures: • Norwood procedure • Bi-directional Glenn operation • Fontan operation
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- 146.
- 148. D-TGA RTSystemic LungLT Parallel circulation AortaPA PDA ASD VSD
- 149. I-TGA RA LA Corrected TGA AortaPALVRV Systemic Lung
- 150. Decrease pulmonary vascular resistant DecreasePA pressure Aorta PDA PA Lung Oxygenated blood LA If PDA closure Progressive cyanosis LA PFO RA RV aorta systemic
- 151. VSD Ventricular systole Rt Lt Ventricular diastole Lt Rt Bidirectional flow by VSD
- 152. Clinical Manifestation • Cyanosis •Tachypnea • Signs of congestive heart failure
- 153. Physical Examination • SimpleTGA o Tachypnea with cyanosis o Single S2 o SEM at LUPSB • TGA with VSD o Tachypnea with cyanosis o Single S2 o SEM at LUPSB o Crepitation both lungs (heart failure)
- 154. Investigation • EKG :RVH • Chest X-ray o Cardiomegaly o Pulmonary venous congestion : increase pulmonary vasculature o Egg on a string
- 155. Investigation • Echocardiogram • Cardiaccatheterization
- 156. Treatment • Medical treatment oIf cyanosis after administer PGE1 o Balloon atrial septostomy (BAS) o Clinical heart failure • Inotropic drug • Diuretic drug
- 157. Treatment • Surgical treatment oAll patient o Atrial switch operation (ASO)
- 159. Total Anomalous Pulmonary VenousReturn (TAPVR)
- 160. Lung bud Intraparenchymal pulmonary v. เชื่อมกับ commonpulmonary v. (ยื่นจาก LA) • Splanchnic plexus lung bud • Venous drainage : Umbilico - vitelline & cardinal venous system Pulmonary vascular bed • Pulmonary plexus & splanchnic plexus obliterate • Pulmonary venous drainage ต่อกับ common pulmonary vein Common pulmonary vein รวมกับ LA Pulmonary vein เทเข้า posterior wall ของ LA TAPVR Cor triatrium
- 161. Cardiac type CardiactypeSupracardiac Infracardiac Mixed
- 163. Signs & Symptoms •TAPVR without pulmonary venous obstruction o แรกคลอดเหมือนปกติ แต่ SpO2 90% o 3-6 month : เขียว หายใจเร็วแรง น้าหนักขึ้นน้อย ติดเชื้อทางเดินหายใจบ่อยๆ o Mild cyanosis, undernourished, tachypnea, dyspnea o Tachycardia bulging precordium with palpable right ventricular impulse o Widely fixed split S2 , louder S2P, SEM gr 2-3/6 at LUSB, S3 gallop + diastolic rumbling murmur at LLSB o hepatomegary
- 164. Signs & Symptoms •TAPVR with pulmonary venous obstruction o เขียว หายใจเหนื่อยเมื่อแรกคลอด o Moderate - severe cyanosis, tachypnea with retraction o Lungs: crepitation o Loud S2P ± murmur (Systolic murmur at LSB) o Hepatomegaly
- 165. CXR • TAPVR without pulmonaryvenous obstruction o Cardiomegaly o Increased PVM
- 166. EKG • TAPVR withoutpulmonary venous obstruction o RVH : rSR’/ tall R in right chest lead o RAE • TAPVR with pulmonary venous obstruction o RVH : T upright in V4R, V1/ tall R in right chest lead
- 167. Echocardiogram • RAE • RVH •Atrial septum โป่งไปทาง LA • ไม่พบ pulmonary vein เทเข้า LA แต่เทเข้า SVC/IVC/Coronary sinus • Right to left flow ผ่าน PFO • Pulmonary venous confluence ผ่านเข้า systemic vein
- 168. Treatment • Medical treatment: ควบคุม heart failure o TAPVR without PVO : digitalis, diuretic o TAPVR with PVO, pulmonary edema : ETT on ventilator ให้ O2, PEEP • Surgical treatment o เชื่อม pulmonary vein & LA โดยให้ anastomosis กว้างที่สุด o TAPVR with PVO : ควรผ่าอย่างรีบด่วน o TAPVR without PVO : ผ่าเมื่อผู้ป่วยพร้อม ทิ้งไว้นานจะ heart fail รุนแรงขึ้น
- 169.
- 170.
- 171.