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Transcript
CONGENITAL HEART DISEASE 1- Zahra Asghari 2- Samira Baniasadi moghadam 3-Mahtab Dolatabadi 4- Fahime Gohari 5-Aida Mirkazemi 6- Azade Izadi 7- Sanaz khosravi Ghareche • approximately 0.8% of all live births are complicated by congenital cardiac abnormalities. • Congenital heart disease is a major cause of • infant morbidity and mortality. • As a result of advances in pediatric cardiology and cardiothoracic surgery, approximately 85% of infants born with congenital heart disease can be expected to survive into adulthood. • Most cases of congenital heart disease occur sporadically. Etiology • without a known specific cause. • Genetic abnormalities are responsible for a proportion of cases and may contribute to cases occurring sporadically as well • Environmental factors. • Symptoms can develop shortly after birth when transition from fetal to adult circulation represents a new dependence on biventricular circulation with a pulmonary circuit.→begin of symptoms • The isolated pulmonary and systemic circulations: •①D: the ductus arteriosus and the foramen ovale. •②L :Hypertrophy of the morphologic right ventricle in L transposition of the great arteries is sufficient to compensate for systemic vascular resistance and maintain normal perfusion for years with symptoms often developing when the systemic ventricle fails. • Still other lesions may develop in adulthood when degenerative changes, such as stenosis of apreviously well-functioning bicuspid aortic valve, are superimposed on an initial lesion. • congenital defects may : ★Go undetected throughout life →small ASDs ★resolve spontaneously →small muscular VSDs Septal defects ATRIAL SEPTAI DEFECTS (ASD) • ASDs are some of the most common congenital defects, representing 10%to l7% • a higher prevalence in women (6o%) • Defects are classified according to their location in the interatrial septum : • 1- primary ostium defect : 20%, defect in atrioventricular junction usually, associated with a cleft mitral valve and mitral failure rarely, great ASD, single VA valve→defect in VA septum 2-secundary ostium defect:60%,defect in fossa ovalis • uncomplicated ASDs →blood shunts hom the left to the right atrium • The magnitude of the shunting is determined by the size of the defect and the compliance of the Ieft and right ventricles. • Small ASDs :the increased blood flow in the right atrium without sequelae and no significant hemodynamic compromise of the right heart. • Large ASDs: the right atrium and right ventricle dilate to accommodate the increased volume of shunted blood. Pressurein the pulmonary artery increases econdary to the increased volume of blood. Symptoms • Most patients with ASD are asymptomatic until adult. • when symptoms such as fatigue, dyspnea, and poor exercise tolerance develop, secondary to right ventricular dysfunction. • atrial fibrillation, especially after 50 years of age • 5%Irreversible pulmonary vascular obstruction resulting in right-to-left shunting and cyanosis (Eisenmenger syndrome) • Stroke or transient ischemic attack in order to paradoxical emboli Physical exam • Parasternal RV impulse • Widely and fixed split S2 • Ejection murmur across pulmonic Diagnosis • Two dimensional and color Doppler echocardiography • Transesophagial echocardiography • Electerocardiography • MRI • Cardiac catheterization • Angiography Treatment • closure, even in the absence of symptoms, pulmonary hypertension,… Significant pulmonary hypertension is a contraindication to ASD closure. • devise Amplatzer • Primum and sinus venosus defects should be surgically closed. Antibiotic prophylaxis for infective endocarditis is not required for small ASDs or patent foramen ovale or after ASD closure. Ventricular septal defect (VSD) • VSD is a common congenital abnormality in newborns.(1 in 500 births) VSD’s classification • • • • Supracristal vsd Perimembranous vsd(most common:80%) Atrioventricular canal vsd Muscular vsd • If the defect is small: Right ventricular size & function are normal and pulmonary vascular resistance dose not increase. • If the defect is large: The right ventricle dilates to accommodate the increased volume & pulmonary blood flow increases. Eisenmenger syndrome • • • • • • Pulmonary vascular obstruction Pulmonary artery HTN Reversal of interventricular shunt Systemic desaturation Cyanosis clubbing Physical examination • • • • Hyperdynamic precordium Palpable thrill along the left sternal border Holosystolic murmur Loud p2 Diagnosis • • • • Tow dimensional echocardiography Doppler echocardiography MRI CT scan Treatment: • Sutures • Prosthetic patch • Percutaneous device closure(muscular) Congenital AS & bicuspid aortic valve: • Valvalr stenosis is most often secondary to a bicuspid aortiv valve. • Congenital left ventricular outflow obstruction occurs at valvular,subvalvular or supravalvular. Bicuspid aortic stenosis: as with acquired aortic stenosis: chest pain,syncope,congestive heart failure. Other complications: sudden death & infective endocaditis. Physical examination: • Ejection quality murmur at the left sternal border. • Early systolic ejection click • Decrescendo diastolic murmur of aortic insufficiency Diagnosis • Two –dimensional echocardiography • Doppler echocardiography Treatment: • • • • Percutaneous valvuloplasty Aortic valve replacement Antibiotic prophylaxis Oral hygiene Sub aortic stenosis: • Firs diagnosed in adulthood • A discrete,fibrous diaghragm Supravalvar aortic stenosis(svsa): • Loss-of-function mutations in the extracellular matrix protein,elastin,is responsible. • It is often part of a syndrome with hypercalcemia,multiple skeletal-vasculardevelopemental abnormalities. PULMONIC VALVE STENOSIS • Pulmonic valve stenosis is the most common cause of obstruction to right ventricular outflow • usually occurs as an isolated congenital lesion • Fusion of the pulmonary leaflets creates the pressureoverloaded state and results in right ventricular hypertrophy • Unless the valve is severely stenotic at birth, most affected persons live a normal life until adolescence or young adulthood • Patients with mild-to-moderate stenosis are usually asymptomatic and rarely have complications associated with the defect • Patients with moderate-to-sever obstruction often exhibit progressive fatigue and dyspnea • If right ventricular dysfunction occurs, then symptoms and signs of right-sided ventricular heart failure may be present. Physical examination • • • • • • The patient with severe stenosis has a right ventricular lift on palpation of the precordium The S I sound is usually normal and is followed by an opening click that becomes louder with expiration The P2 sound becomes softer and is delayed as the severity of the stenosis increase The characteristic murmur of pulmonic stenosis is a systolic ejection murmur heard best at the left upper sternal border, which increases with inspiration A late-peaking murmur indicates more severe stenosis A prominent jugular venous a wave and right-sided 54 sound may also be present in patients with severe obstruction to right ventricular outflow treatment • For asymptomatic patients with mild pulmonic stenosis, therapy is limited to endocarditis prophylaxis • Patients with moderate stenosis (peak gradient >50mmHg) are likely to develop symptoms and require intervention over time and should be treated even in the absence of symptoms • Patients with severe obstruction (peak gradient >80mmHg) also require intervention • In children and adults with isolated pulmonic stenosis , percutaneous balloon valvuloplasty is a suitable therapeutic option • Valve replacement is rarely necessary Ebstein's a nomaly • • • • • • • A rare condition (0.50loo f patients with congenital heart disease) characterized by apical displacement of the tricuspid valve into the right ventricle The basal portion of the right ventricle forms part of the right atrium and leaves a small functional right ventricle The tricuspid leaflets are often dysplastic and may partially adhere to the interventricular septum or right ventricular free wall The degree of right ventricular dysfunction depends on the size of the functioning right ventricle and the severity of the tricuspid regurgitation Frequently develops in adulthood A patient foramen ovale or ostium secundum ASD is present in more than 50% of patients and may result in right-to-left shunt flow as right atrial pressure increases Supraventricular arrhythmias are common Diseases of Aorta • Coarctation of the aorta: • Coarrctation of the aorta is a firotic narrowing of the aortic lumen Diagnosis The diagnosis of coarctation may be made by • tow-dimensional and Doppler echocardiography. Repair in adults is recommended at at the • time of diagnosis.Surgical repair is standard in many center,although primary percutaneous treatment with balloon angioplasty. Patent Ductus Arteriosus A persistent communication between the • aorta and pulmonary artery is the result of the failure of the ductus arteriosus to close. Diagnosis And Treatment Tow_dimensional and doppller • Percutaneous device closure • Surgery for the PDA that is too large • TETRALOGY OF FALLOT DEFINITION • Tetralogy of fallot is the most common cyanotic congenital heart lesion in adults and represent 10% of all congenital heart defects ETIOLOGY • Malalignment of the aorticopulmonary septum that divides the truncus arteriosus into the aorta and pulmonary artery during development. FOUR COMPONENTS OF TETRALOGY • Overriding of the aorta in relation to the ventricular septum • Right ventricular outflow obstruction, which may be: valvular subvalvular supravalvular or combination of all threes • Membranous VSD • Right ventricular hypertrophy • The presence of right ventricular outflow obstruction is protective,preventing volume and pressure overload of the pulmonary circulation,which would result in fixed pulmonary hypertension. • If pulmonic stenosisi is mild, right - to - left shunt flow is minimal,and the patient remains acyanotic (Pink tetralogy) • The degree of right - to - left shunt flow depends on the degree of RV outflow obstruction. • Tetralogy may also be associated with ASD, muscular VSD, right aortic arch, and other coronary anomalies. • A chromosomal deletion (22q11) is found in 15% of cases, particulary in those whit associated anomalies. TREATMENT • Surgical correction • Palliative surgery Surgical correction • Usually performed during infancy or childhood • Involves relife of right ventricular obstruction and patch closure of the VSD After reparative surgery • • • • Residual polmunary stenosis or regurgitation Aortic insuffiency Residual VSD Aneurysms of the right ventricular outflow tract • Sustained arrhythmias supraventricular ventricular • Prolongation of the QRS duration (to > 180 ms) on the surface electrocardiographic study is a marker for increased risk for ventricular tachycardia and sudden death. Palliative surgery • Performed in childhood to improve pulmonary blood flow • Creation of a shunt between the systemic and pulmonary circulation (e,g., subclavian artery to ipsilateral pulmonary artery [ blalock – taussig shunt ] • Palliative surgery caused long-term palliaton of hypoxia. Palliative surgery complications • Patient may outgrow their shunts, or the shunts may spontaneously close and may lead to progressive cyanosis. • If the shunt is too large, the increased volum of blood into the pulmonary circulation and left heart may result in pulmonary congestion and progress to irreversible pulmonary vascular obstruction. Cardiovascular Disease Congenital Heart Disease Complete transposition of the great arteries • Known as D-transposition • 5-7 % of congenital heart disease • The most common cyanotic congenital heart disease in the newborn • The aorta arises from the RV and the pulmonary artery arises from the left • 90% mortality rate in the first year of life without correction • Associated defects: VSD, left ventricular outflow tract stenosis, coarctation of the aorta Procedures for D-TGA • Atrial switch procedure (e.g. Mustard or Senning procedures) • Arterial switch procedure (Jatene procedure) D-TGA Atrial switch procedure Arterial switch procedure Corrected transposition of the great arteries • Known as L-transposition • The anatomic RV lies on the left and ejects oxygenated blood into an anteriorly displaced aorta and the anatomic LV lies on the right and ejects blood into the posteriorly displaced pulmonary artery • Not cyanotic • Quite uncommon, 0.5% of congenital heart disease • Associated anomalies: atrioventricular nodal block, VSD and Ebstein’s anomaly Single ventricle • When a single ventricle supports both the pulmonary and systemic circulation. As such, tricuspid atresia, double-inlet left ventricle with VSD, and large atrioventricular septal defect • Poor prognosis without repair, median survival of 14 years of age • Cyanosis and functional limitations The Fontan procedure Eisenmenger Syndrome • In 1958,Paul Wood used the term Eisenmenger complex to describe the combination of a large VSD with systemic pulmonary pressures and reversed or bidirectional shunt • A VSD , a PDA , or an ASD could all rezult in Eisenmenger phisiologic characteristic. • The defect size generali exceeds 1.5 diameter for VSD , with about half the diameter for PDA and twice that diameter for ASD . • Complicaitions include hyperviscosity syndrome , hemorahge or thrombosis , arrythmias and sudden death ,endocarditis and cerebral abscenss ,verticular dysfunction , hyperuricemia and goat and renal impairment , among others. • Patient with Eisenmenger syndrome have achived a delicat balance and management of such patients should respect that balance.prevention of complicationsis the preferred sterategy.