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9/6/2016 FETAL CIRCULATION CONGENITAL HEART DISEASES 2 SYSTEMIC AND PULMONARY CIRCULATION CONGENITAL HEART DISEASES 3 1 9/6/2016 CONGENITAL HEART DISEASE (CHD) Incidence: 10 in 1,000 live births Subdivisions: .Acyanotic congenital heart disease .Cyanotic congenital heart disease CONGENITAL HEART DISEASES 4 ACYANOTIC CHD 1. Ventricular septal defect (VSD) 2. Atrial septal defect (ASD) 3. Patent ductus arteriosus (PDA) 4. AV Canal 5. Coarctation of the aorta 6. Aortic stenosis 7. Pulmonary stenosis CONGENITAL HEART DISEASES 5 VENTRICULAR SEPTAL DEFECT Bicuspid aortic valve most common congenital heart defect Excluding bicuspid valve congenital heart defect VSD accounts for 25% of all CHD in childhood. A common concomitant with other forms of congenital heart disease; Truncus arteriosus and tetralogy of Fallot CONGENITAL HEART DISEASES 6 2 9/6/2016 VENTRICULAR SEPTAL DEFECT CONGENITAL HEART DISEASES 7 VENTRICULAR SEPTAL DEFECT CONGENITAL HEART DISEASES 8 VSD AS PRINCIPAL DEFECT 30% of cardiac malformations at birth 20% during childhood 10% during adult life CONGENITAL HEART DISEASES 9 3 9/6/2016 VENTRICULAR SEPTAL DEFECT VSD is the most common cardiac defect associated with chromosomal abnormalities such as trisomy D & E VSD is the most common defect with other anomalies such as esophageal atresia CONGENITAL HEART DISEASES 10 VENTRICULAR SEPTAL DEFECT – PROGNOSIS 75%-80% of small VSDs may close spontaneously by age 10 10%-15% of large VSDs may close spontaneously Generally large VSDs require surgery Loud, holosystolic murmur along the lower left sternal border CONGENITAL HEART DISEASES 11 EISENMENGER SYNDROME A syndrome associated with increased pulmonary vascular resistance (pulmonary hypertension) leading to the right to left shunt in patient with large VSD and or ASD causing cyanosis. CONGENITAL HEART DISEASES 12 4 9/6/2016 ATRIAL SEPTAL DEFECT CONGENITAL HEART DISEASES 13 ATRIAL SEPTAL DEFECT Ostium Primmum ASD: Most commonly associated with Down’s syndrome; the abnormality produces loud pan-systolic murmur due to ostium being located at low level interfering the valvular function of mitral valve and or tricuspid valve. Ostium Secundum ASD: Comprises 70% of all ASD Most common congenital heart disease fount initially in adults excluding bicuspid aortic valve. F>M; resulting in systolic ejection murmur at the left sternal border. CONGENITAL HEART DISEASES 14 ATRIAL SEPTAL DEFECT Sinus Venosus ASD: 10% of all ASD Associated with anomalous pulmonary venous return, because it occurs high on the septum CONGENITAL HEART DISEASES 15 5 9/6/2016 ATRIAL SEPTAL DEFECT CONGENITAL HEART DISEASES 16 PATENT DUCTUS ARTERIOSUS CONGENITAL HEART DISEASES 17 PATENT DUCTUS ARTERIOSUS 1/2500 – 1/5000 live births 2nd and 3rd most common lesions (12-17%) F:M = 2:1 PDA causes continuous “machinery” murmur at left upper sternal border Differential cyanosis develops (clubbed toes, normal fingers) CONGENITAL HEART DISEASES 18 6 9/6/2016 PDA Normal Heart Heart with PDA CONGENITAL HEART DISEASES 19 RIGHT-TO-LEFT SHUNT VSD & PDA are volume loads on the left heart . Increased LV size . Increased LA size . Increased PA size X-ray: LV and LA enlarged . Increased vascularity . Increased PA size EKG: LVH, LAE CONGENITAL HEART DISEASES 20 LEFT-TO-RIGHT SHUNT ASD is a volume load to the right heart . Increased RV size . Increased RA size . Increased PA size X-ray: RV and RA enlarged . Increased vascularity . Increased PA size EKG: Right Axis RVH RAE CONGENITAL HEART DISEASES 21 7 9/6/2016 COARCTATION 17% of Turner’s Syndrome have coarctation M:F = 2:1 single coarctation M:F = 1:1 complex coarctation CONGENITAL HEART DISEASES 22 COARCTATION OF AORTA CONGENITAL HEART DISEASES 23 COARCTATION - COMPLICATIONS 1) Hypertension 2) Congenital bicuspid valve a) Bacterial endocarditis b) Aortic regurgitation c) Calcified aortic stenosis 3) Congestive heart failure CONGENITAL HEART DISEASES 24 8 9/6/2016 COARCTATION – PHYSICAL EXAM 1. Hypertension (primarily systolic) 2. II/VI systolic murmur 3. Absent or decreased femoral pulses with brachial femoral pulse delay 4. Ejection click 5. Notching of the ribs in x-ray CONGENITAL HEART DISEASES 25 CYANOTIC CHD 1) Tetralogy of Fallot (TOF) 2) Total Anomalous Pulmonary Venous Connection 3) Transposition of the Great Arteries 4) Truncus Arteriosus CONGENITAL HEART DISEASES 26 TETRALOGY OF FALLOT CONGENITAL HEART DISEASES 27 9 9/6/2016 TETRALOGY OF FALLOT (TOF) Incidence: • 11% of all congenital cardiac lesions • Most common cause of cyanotic heart disease in older children • 75% of children over four with cyanotic lesions have tetralogy • “boot shaped” heart CONGENITAL HEART DISEASES 28 TETRALOGY OF FALLOT: COMPONENTS 1. Pulmonary stenosis (infundibular) 2. Overriding aorta 3. Ventricular septa defect (VSD) 4. Right Ventricular hypertrophy CONGENITAL HEART DISEASES 29 TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVR) CONGENITAL HEART DISEASES 30 10 9/6/2016 TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVR) Pulmonary veins drain into the right atrium, instead of left atrium X-ray: “snowstorm” appearance CONGENITAL HEART DISEASES 31 TRUNCUS ARTERIOSUS Single arterial trunk originating from the heart that supplies the systemic, pulmonary, and coronary circulations Present in CHF Low survival rate CONGENITAL HEART DISEASES 32 TRUNCUS ARTERIOSUS CONGENITAL HEART DISEASES 33 11 9/6/2016 TRANSPOSITION OF GREATER ARTERIES Pathology: • Aorta arises from right ventricle • Pulmonary artery arises from left ventricle • Need anatomic connection between left and right heart for survival • Abnormal distribution of coronary arteries arising from aorta Incidence: • 10% of all cardiac lesions • Male predominance of 2 to 3:1 • Most common cyanotic heart defect in newborns CONGENITAL HEART DISEASES 34 TRANSPOSITION OF GREATER ARTERIES CONGENITAL HEART DISEASES 35 TRANSPOSITION OF GREAT VESSELS Transposition of Great vessels (TGA) - Most common cyanotic cardiac lesion in neonates - X-ray: “egg on a string” CONGENITAL HEART DISEASES 36 12 9/6/2016 REVIEW QUESTIONS CONGENITAL HEART DISEASES 37 1.THE MOST COMMON ACYANOTIC CONGENITAL HEART DEFECT IN CHILDREN IS: a) Atrial Septal Defect b) Ventricular Septal Defect c) Patent Ductus Arteriosus d) Transposition of the Great Vessels e) Tetralogy of Fallot CONGENITAL HEART DISEASES 38 2. THE MOST COMMON FORM OF CYANOTIC CONGENITAL HEART DISEASE IN CHILDREN, ADOLESCENTS, AND ADULTS IS: a) Tricuspid Atresia b) Univentricular Heart c) Aortic Stenosis d) Tetralogy of Fallot e) Truncus Arteriosus CONGENITAL HEART DISEASES 39 13 9/6/2016 3. THE MOST COMMON ACYANOTIC FORM OF CONGENITAL HEART DISEASE ASSOCIATED WITH HYPERTENSION IN INFANTS, CHILDREN, AND ADULTS IS: a) Coarctation of the Aorta b) Ventricular Septal Defect c) Atrial Septal Defect d) Patent Ductus Arteriosus e) Tricuspid Atresia CONGENITAL HEART DISEASES 40 4.THE MOST COMMON CAUSE OF CYANOTIC CONGENITAL HEART DISEASE IN THE NEWBORN IS: a) Ventricular Septal Defect b) Atrial Septal Defect c) Transposition of the Great Vessels d) Univentricular Heart e) AV Canal CONGENITAL HEART DISEASES 41 5.THE MOST COMMON CARDIAC CAUSE OF DEATH IN THE FIRST WEEK OF LIFE: a. Aortic and Mitral Atresia – Hypo-plastic Left Heart Syndrome b. ASD c. VSD d. Aortic Stenosis e. AV Canal CONGENITAL HEART DISEASES 42 14 9/6/2016 6. CYANOTIC CONGENITAL HEART DEFECT WHICH DEMONSTRATES INCREASE PULMONARY VASCULARITY IN THE NEWBORN IS: a) Pulmonary Atresia with intact septum b) Tricuspid Atresia c) Severe Pulmonary Stenosis d) Transposition of the Great Vessels e) VSD CONGENITAL HEART DISEASES 43 7.THE ANATOMIC ABNORMALITY THAT DETERMINES THE PHYSIOLOGY IN TETRALOGY OF FALLOT IS: a) The size and placement of the ventricular septal defect b) An atrial septal defect c) A right aortic arch d) An overriding aorta e) The degree of valvular and sub-valvular pulmonary stenosis CONGENITAL HEART DISEASES 44 8. PICK ONE THING NOT ASSOCIATED WITH A PATENT DUCTUS ARTERIOSUS: a) Prematurity b) Rubella Syndrome c) Continuous Murmur d) Volume loaded left heart with left atrial and left ventricular dilatation e) Marfan’s Syndrome CONGENITAL HEART DISEASES 45 15 9/6/2016 9. THE PATHOGNOMONIC CLINICAL FINDING IN PATIENTS WITH ATRIAL SEPTAL DEFECT IS: a) Left-to-Right Shunt b) Volume loaded right heart c) Early severe pulmonary vascular disease d) Fixed splitting of the second heart sound e) High altitude pulmonary edema CONGENITAL HEART DISEASES 46 10. THE FOLLOWING IS TRUE OF AORTIC STENOSIS: a) A bicuspid aortic valve may remain stable over time, develop aortic obstruction, or become incompetent b) May present in the newborn period with critically ill neonate c) Is commonly associated with coarctation of the aorta d) All of the above e) None of the above CONGENITAL HEART DISEASES 47 16.
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