Presentation on theme: "Cyanotic Heart Disease; Overview of Management"— Presentation transcript:
1Cyanotic Heart Disease; Overview of Management ByDr. Ahmad ShakerMD Cardiology
2DefinitionCyanotic Heart Disease is a defect or group of defects in the structure or function of the heart or the great vessels, present at birth, consisting of abnormal blood flow from the right to the left part of the circulatory system (either at the level of the atria, the ventricles, or the great vessels).This abnormal communication (called right-to-left shunt) results in poor oxygenation of the body and therefore cyanosis
3Causes of Cyanotic Heart Disease: –Tetralogy of Fallot–Transposition of the great arteries (D-TGA)–Single ventricle–Truncus arteriosus–Total anomalous pulmonary venous connections–Ebstein’s anomaly–Eisenmenger’s disease
4Ductal Independent Mixing Lesions: -Truncus Arteriosus .-d-Transposition of Great Arteries.-Total Anomalous Pulmonary Venous Connection without Obstruction (TAPVC).Ductal-Dependent Pulmonary Blood flow:-Tricuspid Atresia-Pulmonic Atresia with Intact Ventricular Septum.-Tetralogy of Fallot.-Ebstein’s Anomaly.
5Increased Pulmonary Blood Flow Decreased Pulmonary Blood Flow Lesions with Ductal Dependent Systemic Blood Flow:-Hypoplastic Left Heart Syndrome (HLHS).-Interrupted Aortic Arch.-Total Anomalous Pulmonary Venous Connection with Obstruction.Increased Pulmonary Blood FlowDecreased Pulmonary Blood FlowObstruction to Systemic Blood FlowTotal Anomalous Pulmonary Venous ReturnTruncus ArteriosisTetralogy of FallotTransposition of the Great ArteriesTricuspid AtresiaHypoplastic Left Heart Syndrome
6Symptoms & Signs Cyanosis. Dyspnea. Failure to thrive, or failure to grow properlyFatigueSquatting position after physical activity to relieve breathlessness.Hypoxic spells, characterized by:-Anxiety Hyperventilation.-Sudden increase in cyanosis.Syncope.Chest pain, Arrythmias.CHF.
7DiagnosisClinical:-Upper left sternal border ejection murmur of RV outflow tract obstruction TOF.-Newborns present with severe cyanosis and a continuous murmur of ductal flow pulmonary valve atresia and ductus arteriosus-dependent pulmonary blood flow .-Present immediately after birth with severe cyanosis that progresses rapidly to metabolic acidosis TGA.
8Days to weeks after birth with heart failure and mild hypoxemia, A hyperdynamic precordium, wide pulse pressure, a normal S1 with a frequent ejection click, and a loud, usually single S2 are characteristic persistant truncus.In persistent truncus, Heart murmurs vary and may include a flow murmur at the base, a loud regurgitant murmur at the lower left sternal border, and a mid-diastolic mitral flow murmur. With truncal valve insufficiency, a high-pitched diastolic murmur over the mid-sternum is present
10-Right axis deviation and RV hypertrophy on ECG ---- TFO.
11-Combined ventricular hypertrophy ----- Truncus. -Right axis deviation and Right Ventricular Hypertrophy ---- Total APVD.-Superior axis and L V Hypertrophy ----TV atresia.-LV hypertrophy, leftward axis --- Pulmonic Atresia with Intact Ventricular Septum-Right Bundle Branch Block, Delta Waves due to WPW syndrome --- Ebestine’s Anomaly.
12TGARight ventricular hypertrophy, right axis deviation .
54Complications Heart failure and hypoxic spells. Polycythemia and increased coagulation.Stroke.Infective endocarditis.Brain abscess.Hemoptysis.Impaired growth.Pulmonary hypertension.
55PrognosisMost children with congenital heart defects can be helped by surgery even if the defect is severe. Death rates attributable to congenital cardiovascular defects are only about two per 100,000, but they are considerably higher for infants under one year old. For infants under one year, the death rate is about 60 per 100,000.
56Treatment: *‘tet’ spells: treatment 1. ‘knee-chest position’ or over parent's shoulder withknees bent2. supplemental oxygen (effectiveness is questionable inthe absence of pulmonary blood flow)3. sedation: intravenous or subcutaneous morphine,0.1 mg/kg4. intravascular volume expansion5. prolonged cyanosis: an alpha agonist (phenylephrine,5-10 mcg/kg IV)6. for prevention of spells: propranolol (0.5-1 mg/kg poQID)
57*The infants need to be monitored because of the resultant polycythemia, which may lead tohyperviscosity. Hyperviscous blood flows poorlythrough the circulatory bed and results in poortissue perfusion.*If blood flow to the systemic or pulmonic circulationis not sufficient to sustain life, prostaglandin E1(PGE1) (0.05 to 0.1 µg/kg/min IV) can beadministered to maintain patency of the ductusarteriosus.*When a PGE1 infusion is being administered, bloodpressure must be monitored and hypotensioncorrected.
58Tetralogy of Fallot*Corrective operations are often performed by 18months of age or earlier if the child has recurrenthypoxic episodes or progressive cyanosis.*If pulmonary stenosis is severe, and supplementalblood flow through the ductus arteriosus is requiredto support oxygenation during the neonatal period.*A palliative shunt is often placed, the most commonof which is the Blalock-Taussig shunt. The shuntingprocedure involves anastomosis of the subclavianartery to the pulmonary artery, which will direct bloodfrom the systemic circuit into the pulmonary bed andimprove pulmonary blood flow.
59*Corrective surgery involves closing the VSD with a patch, relieving the right ventricular outflowobstruction, and closing any previous palliativeshunts.*Early corrective surgery is preferable to a palliativeprocedure and can be performed in infancy if thepulmonary arteries are of sufficient size and thecoronary artery connection is in a normal position.*Without surgery, life expectancy is markedlyreduced. Even with successful surgery, heart block,aneurysm formation, and late sudden death arepossible.
60Transposition of the Great Vessels *The arterial switch procedure is the surgicalintervention of choice, since it returns blood flow toits normal pattern. This procedure involves cuttingthe great vessels above the valves and switchingtheir positions with reimplantation of the coronaryarteries.*When corrective surgery is not possible, a palliativeballoon or surgical septostomy is performed.*If a balloon septostomy is not possible or noteffective, a surgical septostomy (Blalock-Hanlonoperation) can be performed.
61*Corrective surgery involves partitioning the atrium and dividing the chamber into a front and a back section (Atrial Switch).*Systemic venous blood is redirected in front of the partition toward the left ventricle and pulmonary venous blood is directed behind the partition toward the right ventricle.*This partition can be made of a synthetic material (Mustard procedure) or of the child’s atrial septum (Senning procedure).*The most common long-term complications of the Mustard and Senning procedures are arrhythmias
62Truncus Arteriosus *Palliative pulmonary banding can be performed to improve the condition and development ofpulmonary vascular disease until reparativeprocedures can be performed.*Since banding increases mortality and complicatesof corrective surgery, the corrective surgery is usuallyattempted in infancy. Correction involves closingthe VSD so the left ventricle empties into the runcus.*The pulmonary arteries are removed from the truncusand a conduit is inserted between the right ventricleand the pulmonary arteries (Rastelli procedure).
63Total Anomalous Pulmonary Venous Return *Surgery is usually indicated soon after the diagnosisis confirmed. This operation involves theanastomosis of the pulmonary veins to the leftatrium, closure of the ASD, and division of theanomalous connection.*Without surgery, the prognosis is poor.*Even if surgery is performed within days of birth,infants who have severe cyanosis and poor cardiacoutput before repair have high postoperativemortality. Connections above the diaphragm have abetter prognosis than connections below thediaphragm.
64Tricuspid Atresia *If pulmonary blood flow is minimal, palliative surgery is frequently necessary soon after birth. Aconnection is usually made between the subclavianartery and the pulmonary artery (Blalock-Taussig).*Enlargement of the interatrial defect may benecessary to facilitate flow to the left atrium from theright.*Corrective surgery usually involves a Fontanprocedure, an anastomosis between the pulmonaryartery and the right atrium. Any previous shuntprocedure will be closed and the ASD patched.
65Hypoplastic Left Heart Syndrome *Staged surgical repair of HLHS is still in its infancy and themortality rate is high, but it does offer some hope for survival.*The first surgery—the Norwood procedure—is performedduring the neonatal period. A portion of the main pulmonaryartery is anastomosed to the aorta, a shunt is performed toincrease the pulmonary blood flow, and a large atrial septaldefect is created.*In the second stage, the volume load on the right ventricle isreduced.*The final repair is a modified Fontan procedure, in whichdeoxygenated blood from the lower part of the body is directedto the right pulmonary artery shunt to mix with blood returningfrom the head and upper body and flow into the systemiccirculation.*Heart transplantation is also an option for infants with HLHS.