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Cyanotic CHD
This document provides a case scenario of a 4-week-old male infant presenting with cyanosis after feeding or crying. On examination, the infant has purplish lips, hands and feet, a grade III/VI systolic murmur, and tests revealing low oxygen levels that increase slightly with oxygen. The document discusses possible diagnoses of congenital cyanotic heart disease such as tetralogy of Fallot and asks what the diagnosis could be. It then provides an overview of tetralogy of Fallot and its characteristics.
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Cyanotic CHD
- 1. Welcome to Seminar Dr.Maimuna Sayeed Dr. Md. Tariqul Islam
- 2. Case Scenario History • 4weeks old male • Gets bluish after feeding or crying • Previously well, full-term baby • The family history was negative
- 3. Case Scenario (Contd.) Physical •Vigorous male, growing appropriately • HR= 135, RR= 30, normal BP, no fever • Lungs clear to auscultation b/l, no wheezes, rhonchi, rales • Purplish lips, hands and feet • Grade III/VI systolic murmur loudest at lower left sternal border • Liver was 1.5cm below right costal margin and a normal spleen • Peripheral pulses equal in upper/lower extremities, cap refill time < 3sec
- 4. Case Scenario (Contd.) Workup •PaO2 of 38mm Hg and a hyperoxia test showed increase to 48mm Hg • Electrocardiogram showed RVH
- 5.
- 6. What could bethe diagnosis
- 7. Congenital Cyanotic HeartDisease (Tetralogy of Fallot)
- 8. Congenital Cyanotic HeartDisease
- 9. Overview • Cyanosis innewborn • Approach to Cyanotic CHD
- 10. Cyanosis in Newborn •It is the bluish colouration of skin and mucous membrane due to increased deoxygenated haemoglobin in blood. • Cyanosis becomes visible when there is >3-5g/dl of deoxygenated haemoglobin. - T. L . Gomella, 2013
- 11. Why Cyanosis Develops? •The degree of cyanosis depends on both oxygen saturation and haemoglobin concentration. • Cyanosis can be a sign of severe cardiac, respiratory or neurologic compromise. • Cyanosis can also be caused by a reduced blood oxygen – carrying capacity secondary to an abnormal form of hemoglobin such as methemoglobinemia.
- 12. Causes of Cyanosisin Newborn A. Respiratory Diseases: i. Parenchymal: • Transient Tachypnea of Newborn (TTN) • Hyaline membrane Disease (HMD) • Aspiration - Meconium, blood, mucus or milk • Pneumonia • Pulmonary – Haemorrhage – Edema
- 13. Causes of Cyanosisin New-born (Contd.) ii. Non-Parenchymal: • Airway obstruction – mucous plug – choanal atresia • External compression of the lungs – any air leaks – pleural effusion • Congenital defects – congenital diaphragmatic hernia – hypoplastic lung – adenomatoid malformation – diaphragm abnormality
- 14. Causes of Cyanosisin Newborn (Contd.) B. Cardiac causes: • Transposition of Great Arteries (TGA) • Tetralogy of Fallot (TOF) • Total Anomalous Pulmonary Venous Return (TAPVR) • Truncus arteriosus • Tricuspid atresia • Ebstien’s anomaly • Single ventricle • Hypoplastic Left Heart • Double outlet right ventricle • Persistent Pul HTN of Newborn (PPHN) FiveTs
- 15. Causes of Cyanosisin New-born (Contd.) C. CNS cause: • Hypoxic Ischemic Encephalopathy • Congenital Hydrocephalus • Seizure • Periventricular-intraventricular hemorrhage D. Miscellaneous: • Methemoglobinemia • Polycythemia • Hypothermia • Hypoglycemia • Sepsis
- 16. Making the diagnosis ImmediateQuestions: • Does the infant have respiratory distress? • Does the infant have a murmur? • Was the infant cyanotic at birth? • Is the cyanosis continuous, intermittent, cyclical, sudden in onset, or occurring only with feeding or crying?
- 17. Making the diagnosis(Contd.) • Has the baby had the recommended pulse oximetry screening for CHD? • Is there differential cyanosis? • What is the prenatal & delivery history?
- 18. Diagnosis • After throughhistory and physical examination, investigations according to suspicion. • Treatment according to cause.
- 19. Approach For CongenitalHeart Disease In Newborn
- 20. Epidemiology of CongenitalHeart Diseases • Structural Congenital Heart Disease is 6-8 per 1000 live births • Major cause of death during 1st year of life after prematurity (3/1000 live births) - John P. Cloherty, Manual of Neonatal Care: 7th Edition • Congenital heart defects account for 27% of infant deaths worldwide. - American Heart association Update, 2013
- 21. Estimated causes ofneonatal mortality around the year 2010 in Bangladesh Data source: Bangladesh-specific mortality estimates (Liu et al. 2012).
- 22. Congenital Heart Diseasedocumentation By NNPD In Bangladesh, June 2014 – July, 2015
- 23. Etiology of CHD Factorsassociated with increased incidence: High risk of CHD in children: Prenatal factors: Genetic factors: • Maternal rubella • Radiation • Alcoholism • Age >40 yrs • Insulin dependent diabetes • Fetal intra uterine cardiac viral disease • A sibling with a heart defect • A parent with CHD • Chromosomal aberration e.g. Down’s syndrome • Born with other congenital anomalies
- 24. Common Presentations OfCHD • Cyanosis • CHF • Asymptomatic heart murmur • Arrhythmia
- 25. Relevant Clues FromHistory For CHD Family History: The presence of a congenital heart lesion in a first-degree relative can have considerable diagnostic relevance. The recurrence rate in a first-degree relative is 1% to 4%. If there are two affected first-degree relatives, the recurrence rate is 3% to 12%. E.g. ventricular septal defect, patent ductus arteriosus, atrial septal defect, and tetralogy of Fallot - Arthur J Mos. 1992
- 26. History Maternal & PerinatalHistory- • Maternal DM (Ventricular septal hypertrophy) • Maternal Rubella (PDA) • Maternal exposure to alcohol or drugs
- 27. Physical Examination • Dysmorphicface (Trisomies 13, 18, 21) • Colour- Pallor or cyanosis • Signs of respiratory distress like tachypnea, chest indrawing • Poor perfusions • Pulse volume- bounding (PDA) or Diminished pulses in the lower extremities (COA) • Blood pressure is ≥10 mmHg in arms than legs in COA
- 28. Physical Examination (Contd.) •Abnormal heart rate (may be high or low than normal) • Abnormal precordial activity (Dextrocardia, cardiac enlargement, ventricular impulse) • Abnormal S2 splitting • A single S2 occurs- – Aortic atresia – Pulmonary atresia (PA) – Truncus arteriosus (TAC) – Severe pulmonary stenosis (PS)
- 29. Physical Examination (Contd.) •Abnormal extra heart sounds (gallop, pericardial friction rub) • Pathologic murmurs (should be distinguished from innocent murmur) • Hepatomegaly • Extracardiac abnormalities
- 30. Diagnostic Tools • ChestX-ray • Electrocardiogram • Echocardiogram • Arterial Blood Gas • Hyperoxia test • Pulse oxymetry
- 31. Cyanotic heart disease ↑Pulmonary Flow ↓ Pulmonary Flow • TGA • TOF • Single Ventricle • Pulmonary Atresia • Truncus Arteriosus • Tricuspid Atresia • TAPVR w/o Obstruction • TAPVR With Obstruction • Single Ventricle • Double Outlet Right Ventricle • Hypoplastic Left Heart Syndrome • Ebstein Anomaly (of Tricuspid Valve)
- 32. Transposition of theGreat Arteries
- 33. Complete Transposition ofthe Great Arteries • 5% of all CHD • Male: Female = 3:1 • Most common cyanotic condition that requires hospitalization in the first two weeks of life • Aorta arises from the right ventricle • Pulmonary artery arises from the left ventricle • Defect to permit mixing of two circulations- ASD, VSD, PDA. • VSD is present in 50% of cases, necessary for survival
- 36. Clinical Symptoms No mixinglesion and restrictive PFO • Profound hypoxia • Rapid deterioration • Death in first hours of life • Absent respiratory symptoms or limited to tachypnea • Single second heart sound, no murmurs
- 37. Clinical Symptoms (Contd.) Mixinglesion present (VSD or large PDA) • Large vigorous infant • Cyanotic • Little to no respiratory distress • Most likely to develop CHF in first 3-4 months of life
- 38. Diagnosis CXR • Egg shapedcardiac silhouette • Narrow superior mediastinum ECG • Normal or RVH Echocardiography • Transposed ventricular & arterial connection
- 40. Management • Prostaglandin toestablish patency of the ductus arteriosus • Therapeutic balloon atrial septostomy (Rashkind Procedure) if surgery is not going to be performed immediately • Improves mixing and pulmonary venous return at the atrial level • Definitive Surgery: – Arterial switch operation (Jatene) - usually within first 2 weeks – Mustard operation
- 41.
- 42. Tricuspid Atresia (Contd.) •Represents about 2% of structural heart lesion • Tricuspid valve fails to develop • Hypoplasia of right heart • Venous blood from right atrium depends on open ASD or PFO, VSD, PDA
- 44. Tricuspid Atresia (Contd.) ClinicalFindings • Cyanosis at birth • Single S2 • Systolic murmur along left lower sternal border Diagnosis • Chest X-Ray: Oligemic lung fields
- 45. Tricuspid Atresia (Contd.) •ECG – Left axis deviation – Reveals left ventricular hypertrophy • Echocardiography – Absence of tricuspid valve
- 46. Tricuspid Atresia: Tx •PGE1 administration necessary • Rashkind Balloon atrial septostomy • Pulmonary artery banding • Glenn shunt Superior and inferior vena cava are connected directly to the pulmonary arteries - Between 3-6 month • Fontan procedure Anastomosis between Right atrium & pulmonary artery - Between 1.5 – 3 year of age
- 48.
- 49. Truncus Arteriosus (Contd.) •Failure of primitive truncus arteriosus to divide into aorta and pulmonary Artery • It constitute 1% of CHD • VSD almost always present • 4 types: According to arise of Pulmonary Artery from common trunk
- 51. Truncus Arteriosus (Contd.) ClinicalFindings: • Minimal cyanosis at birth • Congestive Heart failure develops in 2-3 weeks • Bounding pulses, pulse pressure widened • Loud, single S2 • Systolic murmur heard at left sternal border Diagnosis: • ECG reveals biventricular hypertrophy • Echocardiography: • Demonstrates the large truncal artery overriding the VSD and the pattern of origin of the branch pulmonary arteries
- 52. Truncus Arteriosus (Contd.) CXR •Cardiomegaly • Increased pulmonary vascularity
- 54. Truncus Arteriosus: Tx Surgicalrepair (Rastelli repair) • At 2 to 3 months of age • Closing VSD • Separation of pulmonary arteries from truncus • Placing conduit between right ventricle and pulmonary arteries
- 55.
- 56. Tetralogy of Fallot(Contd.) • Constitute 10% of CHD • Only 20% of TOF are cyanotic at neonatal period • Variable Degree of Pulmonary stenosis and size of VSD determine present degree of Cyanosis
- 58. Tetralogy of Fallot(Contd.) Pathophysiology: Increased resistance by the pulmonary stenosis causes deoxygenated systemic venous return to be diverted from RV, through VSD to the overriding aorta and systemic circulation systemic hypoxemia and cyanosis
- 61. Tetralogy of Fallot(Contd.) Symptoms: • Dyspnea on exertion or when crying • Tet spells: irritability, cyanosis, hyperventilation and sometimes syncope or convulsions due to cerebral hypoxemia.
- 62. Tetralogy of Fallot(Contd.) Patients learn to alleviate symptoms by squatting which increases systemic resistance and decreases the right-to- left shunt and directs more blood to the pulmonary circulation.
- 64. Tetralogy of Fallot(Contd.) Physical exam: • Clubbing of the fingers and toes • Single S2 • Systolic ejection murmur heard at the upper left sternal border Lab Studies: • CXR: prominent RV • ECG: RVH, right axis deviation • ECHO: displays and quantifies extent of RV outflow tract obstruction
- 66. Tetralogy of Fallot(Contd.) Complication: • Hyper cyanotic spell • Severe Polycythemia • Cerebral thrombosis • Cerebral abscess • Infective endocarditis • Heart failure • Delayed growth, development and puberty
- 67. Tetralogy of Fallot(Contd.) Management: • Correction of acidosis • IV infusion of Prostaglandin E1 followed by palliative surgery • Correction of anemia by iron therapy • Correction of dehydration. • Treatment of cyanotic spell • Treatment of polycythemia: Blood may be replaced by plasma, dextran or saline after phlebotomy. • Prophylactic antibiotic
- 68. Cyanotic Spell -Treatment • Knee chest position • Oxygen inhalation • Morphine: 50-100 µg/kg (sc, im, iv) • Propranolol: 20 µg/kg iv • Phenylephrine: 20 µg/kg iv • Heavy sedation/anesthesia • Assisted Ventilation
- 69. Definitive surgical Palliative shunt procedure ModifiedBallot- Taussing (Gore- Tex interposing) shunt Between the subclavian artery and ipsilateral pulmonary artery Complete surgical repair Patch closure of the VSD Widening of the RVOT Pulmonary valvectomy
- 71. Total Anomalous PulmonaryVenous Return
- 72. Pathophysiology • The pulmonaryveins drain into the RA or its venous tributaries rather than the LA • A interatrial communication (ASD or PFO) is necessary for survival • Systemic and pulmonary venous blood are completely mixed thus produces cyanosis
- 73. Types of TAPVR •Supracardiac (50%): Common pulmonary vein drains into the SVC via vertical vein • Cardiac(25%): The common PV drains into the right atrium and/or the coronary sinus • Infracardiac(20%): The common PV drains into the IVC or one of its major tributaries often via ductus venosus • Mixed(5%)
- 74. Features Unobstructed PVR • Mildto moderate desaturation, signs of CHF • Systolic murmur heard at the LSB Obstructed PVR • Severe cyanosis • Respiratory distress • Infants are severely ill • Fail to respond to mechanical ventilation
- 75. Diagnosis Chest X-Ray: • Snowmanor figure of 8 appearance • Cardiomegaly • Plethoric lung field
- 77. Diagnosis (Contd.) ECG: • RVH Echo: •RVH • Identify pattern of abnormal pulmonary venous connection
- 78. Treatment • Digitalis anddiuretics to treat heart failure • Corrective surgery
- 79.
- 80. Hypoplastic Left Heart •2-3% of all CHD • Presents first week of life, as PDA closes symptoms develop • Ductal dependant systemic blood flow
- 83. Clinical Feature • Cyanosis •Poor perfusion • Absent or weak pulse • Right parasternal heave • Systolic murmur
- 84. Diagnosis • Chest X-Ray –Cardiomegaly – Plethoric • ECG – RVH • ECHO – Absence or hypoplasia of mitral valve & aortic root – small left atrium & left ventricle
- 85. Treatment of HypoplasticLeft Heart • Correction of acidosis & hypoglycemia • PGE1 • Balloon dilatation of atrial septum • Norwood procedure • Sano procedure
- 86.
- 88. Clinical Feature • Cyanosis •Poor peripheral perfusion • The first heart sound is normal • The second heart sound is single & loud • A systolic ejection murmur
- 89. Diagnosis (Contd.) Chest radiography: •With pulmonary stenosis, the cardiac silhouette is normal to mildly enlarged. Pulmonary vascularity is not increased. • With arch obstruction, the cardiac silhouette is usually at least mildly enlarged. Pulmonary vascularity usually is increased.
- 90. Diagnosis ECG: Ventricular hypertrophy Echocardiography: Two-dimensional echocardiographyand Doppler analysis is diagnostic for single ventricle.
- 91. Treatment Surgery: • If pulmonarystenosis is severe, a Blalock- Taussing aortopulmonary shunt • If pulmonary blood flow is unrestricted, pulmonary arterial banding • The bidirectional Glenn shunt followed by a modified Fontan operation • If sub-aortic stenosis anastomosing the proximal pulmonary artery to the side of the ascending aorta (Damus-Stansel Kaye operation).
- 92. Double Outlet RightVentricle
- 94. Pathophysiology • Both greatarteries arise from the right ventricle in association with a nonrestrictive VSD. • Left-to-right shunting across the VSD results in pulmonary over circulation, pulmonary hypertension, and congestive heart failure. • Pulmonary stenosis results in right-to-left shunting and cyanosis.
- 95. Symptoms • Baby tireseasily, especially when feeding • Cyanosis • Congestive heart failure • Single S2 • Systolic murmur
- 96. Diagnosis Chest X-Ray • DORVwithout PS – Cardiomegaly – plethora • DORV with PS – Heart is normal – pulmonary vascular is oligemic
- 97. Diagnosis (Contd.) ECG Right axisdeviation with Right, Left or bi ventricular hypertrophy Echo The location and size of VSD The position of aorta and pulmonary artery out from ventricle
- 98. Treatment • Pulmonary arterialbanding followed by surgical correction • Intraventricular tunnel repair • Switch repair ( arterial, atrial ) Taussing- Bing heart • Rastelli operation
- 99.
- 101. Pathophysiology • Downward displacementof an abnormal tricuspid valve into the right ventricle • Right ventricle divided into 2 parts - Arterialized part & normal ventricular myocardium • Functional pulmonary atresia
- 102. Clinical Features • Cyanosis •Cardiac dysrhythmia • Gallop rhythm • Hollo systolic murmur
- 103. Diagnosis • CXR: Cardiomegalywith huge RA • ECG: Right bundle branch block, tall p wave, prolonged PR interval • ECHO: Displacement of tricuspid valve, dilated Right atrium, Right ventricular outflow tract obstruction
- 105. Treatment • Neonate withsevere hypoxemia: PGE1 • Starness procedure : Surgical patch closure of tricuspid valve, atrial septectomy, placement of a aortopulmonary shunt • Glenn shunt • Fontan operation
- 106. Take Home Message CyanoticCongenital heart disease in the newborn is a unique and complex problem faced by both neonatologists and cardiologists as it requires skilful handling and balancing of both neonatal issues as well as cardiac physiology. So rapid diagnosis and appropriate management is the key to reduce mortality and morbidity in this delicate newborn population.
- 107.