2Congenital cyanotic heart disease Under supervision ofProf.Dr/ Mariam Abu-ShadyProfessor of pediatrics and neonatologyFaculty of medicine for girlsAl-Azhar UniversityDr.Marwa El-Hady Abd ElmoneimAssisted lecturer of pediatricBy:Amira Mohammad AhmedAmira farag biomyOmnia shams godaa
3CONTENT Definition of Congenital cyanotic heart disease Evaluation of Criticlly Ill Neonate with CyanosisClassification of Congenital cyanotic heart diseaseTetralogy of FallotPulmonary atresia with VSDDouble outlet right ventricle with PSPulmonary atresiaTricuspid atresiaEbstein's Anomaly
4Congenital cyanotic heart disease It is abnormality in cardio circulatory structure that present at birth. CHD occurs in about 8/1000 live births, and those with critical CHD are 2/1000 live births. Prompt diagnosis and treatment of CHD significantly decrease mortality and prevent secondary damage to other organ systems.
5Evaluation of Criticlly Ill Neonate with Cyanosis hyperoxiaABGECGCXRclinicalcausepasslow po2High pco2normaldiagnosticdistressrespiratoryfailLow po2Normal or low pco2May be abnormalAbnormal heart size and vascular marking.Distress less commonOther cardiac fiding.cardiacSlow breathing .Cyanosis improve on stimulationneurologic
6Classification Cyanotic CHD Decreased pulmonary blood flow TOFPulmonary atresia with VSDDouble-Outlet Right VentriclePulmonary atresia without VSDTricuspid atresiaEbstein anomalyIncreased pulmonary blood flowTGATruncus arteriosusDORV without PSTAPVRSingle ventricale
7Criteria of Cyanotic CHD with decrease pulmonary blood flow Central cyanosisclubbingGrowth retardationDecreased Pulmonary component of S2Chest x ray show lung oligaemiaECG show right ventricular hypertrophy or left ventricular hypertrophyEchocardiography : Diagnosticcardiac catheterization; Diagnostic, done preoperative
8Tetralogy of FallotIt is a condition caused by a combination of four heart defects that are present at birth. These defects are:Pulmonary Infundibular StenosisOverriding aortaventricular septal defect (VSD)Right ventricular hypertrophy,
10Tetralogy of Fallot hypercyanotic spells: Sometimes, babies with tetralogy of Fallot will suddenly develop worsen of central cyanosis after crying, feeding or having a bowel movement. It is treated by placing child in the knee-to-chest position, oxygen,morphine and propranol.systolic thrill over left sternal borderSingle second soundEjection systolic murmur(PS)Treated by:Total correction or The Black-Taussig operation
12Pulmonary atresia with VSD Pulmonary atresia with ventricular septal defect (PA-VSD) is a cyanotic congenital heart disease characterized by underdevelopment of the right ventricular (RV) outflow tract with atresia of the pulmonary valve and a large ventricular septal defect (VSD),
14Pulmonary atresia with VSD A prominent a wave in the jugular pulse may be found.auscultation:systolic murmur usually is audible along the lower left sternal border.A continuous murmur is best heard over the upper chest in the presence of a PDA.If systemic-to-pulmonary collateral arteries are present, continuous murmurs may be diffusely audible over the entire chest and back.Treatment:prostaglandin E2 is often required to keep the ductus arteriosus openMost babies will need a 'Shunt' operation during infancy
15Double outlet right ventricle with PS Double outlet right ventricle (DORV) is a congenital heart disease in which the aorta rises from the right ventricle instead of from the left ventricleBoth the pulmonary artery and aorta come from the same pumping chamber. No arteries arise from the left ventricle .
17Double outlet right ventricle with PS initial medical management consists of increasing the fraction of inspired oxygen (FIO2), which may be up to 100%. This decreases pulmonary vascular resistance, thereby increasing the amount of blood flow in the lungsSurgical treatmentcreate a tunnel through the VSD. The tunnel connects to the aorta.
18Pulmonary atresiaPulmonary atresia with intact ventricular septum (PA/IVS) is a condition in which the valve that allows blood to flow from right ventricle of heart to lungs (pulmonary valve) hasn't formed properly or is closed (atresia). PA/IVS is a rare congenital heart disease present from the early stages of a baby's heart development
20Pulmonary atresia systolic thrill over pulmonary area prostaglandin E1 is usually used to help the blood move into the lungs.Open heart surgery to repair or replace the valve
21Tricuspid atresiaTricuspid atresia is the third most common form of cyanotic congenital heart disease, The deformity consists of a complete lack of formation of the tricuspid valve with absence of direct connection between the right atrium and right ventricle.
23Tricuspid atresia single second sound giant A wave murmer of VSD(+PDA) prostaglandin E2 is often required to keep the ductus arteriosus open in the early neonatal period until surgery can be performed.Surgical treatment:The Fontan operation
24Ebstein's AnomalyEbstein's anomaly is a condition in which the tricuspid valve is malformed and sits lower than normal in the right ventricle. This condition is associated with right ventricle dysfunction, and commonly atrial septal defect or patent foramen ovale.
26Ebstein's Anomaly Jugular venous pulse Large a and v waves late in the course of the disease,First heart sound is widely split with loud tricuspid componentThird and fourthheart sounds are commonly presentMedical CareAntibiotic prophylaxis for infective endocarditisMedical therapy for heart failure - Angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxinSurgical CareSurgical care includes correction of the underlying tricuspid valve and right ventricular abnormalities heart sounds are commonly present
27Critaria CCHD with increased pulmonary blood flow Poor feedingRecurrent chest infectionRecurrent heart failureIncrease P2Chest x ray show plethora
28Transposition of the great vessels Def:Transposition of the great vessels is acongenital heart defect in which The two major vessels that carry blood away from the heart -- the aorta and the pulmonary artery -- are switched (transposed).
31Total anomalous pulmonary venous return Def:Total anomalous pulmonary venous return is acongenital heart disease) in which none of the four veins that take blood from the lungs to the heart is attached to the left atrium (left upper chamber of the heart)
33Double outlet right ventricle with out pulmonary stenosis
34InvestigationChest x-rays :no pulmonary stenosis will cause increase pulmonary blood flow resulting in cardiomegaly with increase pulmonary vascular markings. The mediastinum may be narrow due to malposed great vessels.
36Truncus arteriosus (TA) is an uncommon congenital cardiovascular anomaly that is characterized by a single arterial trunk arising from the normally formed ventricles by means of a single semilunar valve (ie, truncal valve).
38Classification.Truncus arteriosus type I is characterized by origin of a single pulmonary trunk from the left lateral aspect of the common trunk, with branching of the left and right pulmonary arteries from the pulmonary trunk..Truncus arteriosus type II is characterized by separate but proximate origins of the left and right pulmonary arterial branches from the posterolateral aspect of the common arterial trunk.
39Type3,4.In truncus arteriosus type III, the branch pulmonary arteries originate independently from the common arterial trunk or aortic arch, most often from the left and right lateral aspects of the trunk. This occasionally occurs with origin of one pulmonary artery from the underside of the aortic arch, usually from a ductus arteriosus..Type IV truncus arteriosus, originally proposed by Collett and Edwards as a form of the lesion with neither pulmonary arterial branch arising from the common trunk, is now recognized to be a form of pulmonary atresia with ventricular septal defect rather than truncus arteriosus.
43Heterotaxy SyndromeHeterotaxy syndrome is a rare birth defect that involves the positionof the heart and other organs. nt
44“right atrial isomerism” and “left atrial isomerism” In a normal heart, the left atrial appendage looks different than the right. In heterotaxy syndrome, the two appendages look similar.
45AspleniaAsplenia or right atrial isomerism: Children with this condition have multiple heart defects. They may have septal defects and problems with heart valves, particularly the pulmonary valve. The spleen may be absent (asplenia), and the liver and other organs may be on the wrong side of the body.
46polyspleniaPolysplenia or left atrial isomerism: Children with this condition may have septal defects as well as problems with heart valves and the heart’s electrical system. The spleen may be absent, or there may be several small spleens (polysplenia), instead of one spleen.
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