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- 1. PRESENTATION TOPIC: CYANOTIC CONGENITALHEART DISEASE • PREPARED BY: • FATIMA ZAINAB KAMARA ID 22044 • ZAINAB KADIATU KAMARA ID 22045 • MODULE: PEDIATRICS • LECTURER: DR AMADU JALLOH
- 2. INTRODUCTION • Cyanotic congenitalheart disease {CCHD} encompasses a group of congenital heart defects that lead to a low level of oxygen in the blood, resulting in cyanosis{ a bluish tint in the skin, lips, and nails}. This happens because these defects allow deoxygenated {blue} blood to bypass the lugs, mixing with oxygenated blood, or because blood has difficulty reaching the lugs for oxygenation.
- 3. TYPES OF CYANOTIC CONGENITALHEART DISEASE Tetralogy of Fallot {TOF} Transposition of the Great Arteries {TGA} Tricuspid Atresia Total Anomalus Pulmonary Venous Connection {TAPCV} Truncus Arteriosus
- 4. Tetralogy of Fallot Tetralogyof fallot {TOF} is the most common type of cyanotic congenital heart disease. This condition is characterized by the combination of four defects: Ventricular Septal defect Pulmonary Stenosis Dextroposition of the aorta so that it overrides the ventricular septum Right ventricular hypertrophy
- 5. PATHOPHYSIOLOGY • The initialdefect in TOF is a narrowing of the right ventricular outflow tract into the pulmonary artery {Pulmonary Stenosis}. • Prevents deoxygenated blood from entering the pulmonary circuit. • In response to this outflow obstruction RV pressure is high as the LV, a right to left shunt appear{VSD}. • VSD allows shunting of the blood between ventricles.
- 6. CONT • The aortaoverrides the VSD • Mixed blood immediately exist the heart • Severity and clinical consequences of TOF is the degree of right ventricular outflow obstruction.
- 7. Clinical features • Cyanosis,tet spell • Dyspnea • Clubbing of the fingers and toes • Slow weight gain {Poor growth}
- 8. Diagnosis • History taking •Physical examination to elicit • Continuous murmur • Chest X ray (IOC)
- 9.
- 10. Management • Treatment ofTOF depends on the severity of the RVOT obstruction. Infants with severe tetralogy require urgent medical treatment and surgical intervention in the neonatal period. Therapy is aimed at providing an immediate increase in pulmonary blood flow to prevent the sequelae of severe hypoxia. • Pott’s procedure: the upper descending aorta is anastomosed with the left pulmonary artery.
- 11. CONT • Brock’s procedure:Pulmonary valvotomy done to correct PS The IV administration of prostaglandin E1 (PGE1 ; 0.01-0.20μg/kg/min)
- 12. Complications • Bacterial endocarditis •Seizure • Brain Thrombosis • Brain abscess • Death
- 13. TRICUSPID ATRESIA Congenital absenceof the tricuspid valve is called tricuspid atresia. There is an absence of right atrioventricular connection. The right ventricle is hypoplastic {undersized or absent}. This condition is associated with PS and PD. There is complete mixing of unoxygenated and oxygenated blood in the left Atrium and so have a mandatory ASD. A PDA or VSD is necessary for PBF and survival.
- 14. • Pulmonary bloodflow (and thus the degree of cyanosis) depends on the size of the VSD and the presence and severity of any associated pulmonic stenosis. • In patients with tricuspid atresia and transposition of the great arteries (TGA) , LV blood flows directly into the pulmonary artery, whereas systemic blood must traverse the VSD and right ventricle to reach the aorta. • In these patients, pulmonary blood flow is usually massively increased and heart failure develops early. If the VSD is restrictive, aortic blood flow may be compromised. • Coarctation of the aorta is often noted in this setting.
- 15. Clinical Manifestation • Cyanosis •Difficulty in breathing • Clubbing of fingers and toes • Polycythemia
- 16. Diagnosis • Chest Xray • ECHO • ECG
- 17. Management • Initial Management Oxygentherapy Administration of prostaglandins E1 Surgical management {BT shunt, Glenn, and Fontan procedures}
- 18.
- 19. Transposition of theGreat Arteries • Transposition of the great arteries, or vessels, a common cyanotic congenital anomaly, accounts for approximately 5% of all congenital heart disease. • Complete separation of systemic and pulmonary circulation • In this anomaly, the systemic veins return normally to the right atrium and the pulmonary veins return to the left atrium. • The connections between the atria and ventricles are also normal (atrioventricular concordance ). • The aorta arises from the right ventricle and the pulmonary artery from the left ventricle • Now survival depends on mixing of blood with PDA
- 20. Clinical Manifestation • Timeof presentation depends on weather ventricular septum is intact or VSD is present. • Patient with TGA with intact septum are cyanotic at birth with CHF occuring in first week of life. • Patients with TGA with VSD presents with mild cyanosis and CHF occur in 6-8weeks ( or 4-10 weeks). • Cyanosis • Tachypnoea • hypoxemia
- 21. DIAGNOSIS • Clinical Presentation •Physical Examination
- 22. Investigations • ECHO • ECG •ABG • Chest X-ray
- 23. Management Medical Management • PGE1like Alprostadil administration which will keep the Ductus Arteriosus (DA) open temporarily ( transient improvement) Surgical management • Ballon artrial septostomy- emergency procedure • Definitive surgery : arterial switch operation or JATENE REPAIR
- 24. Complications • Hypoxemia • Acidosis •Heart Failure • Pulmonary Hypertention • Arrhythmias • Death
- 25. Total anomalous pulmonary venousconnection (TAPVC) • Pulmonary veins instead of draining into left atrium, drain into either • Superior Vena Cava • Right Atrium • Inferior Vena Cava/ Portal vein/ Hepatic veins
- 26. TYPES • Supracardiac TAPVC •Cardiac TAPVC • Infracardiac TAPVC • Supracardiac TAPVC is the most type • Whereas Infracardiac is the most obstructed type.
- 27. Clinical manifestation Cyanosis Respiratory Distress{Tachypnea} Failure to thrive
- 28. Diagnosis • History taking •Clinical Examination Cyanosis is always present even with supplemental oxygen. Examination may reveal signs of right heart overload, such as a prominent right ventricular impulse and possibly a widely spilt and fixed S2{second heart sound}.
- 29. Investigations • Echocardiography • ECG •ECHO • Chest X-ray
- 30. Management • Oxygen therapy Inotropicsupport Surgical Procedures Postoperative Management Long term followup
- 31. Complications • Onset ofCCF occurs at 4-10 weeks of life • Respiratory distress syndrome • Pneumonia • Hypoplastic left heart syndrome
- 32. TRUNCUS ARTERIOSUS • Intruncus arteriosus, a single arterial trunk (truncus arteriosus) arises from the heart and supplies the systemic, pulmonary, and coronary circulations. • A VSD is always present, with the truncus overriding the defect and receiving blood from both the right and left ventricles. • The number of truncal valve cusps varies from 2 to as many as 6, and the valve may be stenotic, regurgitant, or both.
- 33. • The pulmonaryarteries can arise together from the posterior left side of the persistent truncus arteriosus and then divide into left and right pulmonary arteries (type I ). • In types II and III truncus arteriosus, no main pulmonary artery is present, and the right and left pulmonary arteries arise from separate orifices on the posterior (type II ) or lateral (type III ) aspects of the truncus arteriosus. • Type IV truncus is a term no longer used because, in this case, there is no identifiable connection between the heart and pulmonary arteries, and pulmonary blood flow is derived from major aortopulmonary collateral arteries arising from the transverse or descending aorta; this is essentially a form of pulmonary atresia.
- 34. Clinical Manifestations • Boundingpulse • Cyanosis • Systolic ejection murmur • An apical mid-diastolic rumbling murmur
- 35. Diagnosis • ECG • Echocardiography •Angiography • Chest X ray
- 36. Treatment • In thefirst few weeks of life, many of these infants can be managed with anticongestive medications, as PVR falls, heart failure symptoms worsen and surgery is indicated, usually within the 1st few mo. • Delay of surgery much beyond this time period may increase the likelihood of pulmonary vascular disease; many centers now perform routine neonatal repair at the time of diagnosis. • Transcatheter stent-valve.
- 37. Complications • Eisenmenger syndrome •Heart failure
- 38.