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1、A Quick Tour of Congenital Heart Disease,Chris Longhurst, MD Tuesday, October 2, 2018,Introduction,Present in 0.8% of North American and European children Most common category of congenital structural malformation Commonly divided into noncyanotic (L R) and cyanotic (R L) categories based on directi
2、on of shunting,Relative Frequency of Lesions,Ventricular septal defect 25-30 Atrial septal defect (secundum) 6-8 Patent ductus arteriosus 6-8 Coarctation of aorta 5-7 Tetralogy of Fallot 5-7 Pulmonary valve stenosis 5-7 Aortic valve stenosis 4-7 Transposition of great arteries 3-5 Hypoplastic left v
3、entricle 1-3 Hypoplastic right ventricle 1-3 Truncus arteriosus 1-2 Total anomalous pulm venous return 1-2 Tricuspid atresia 1-2 Double-outlet right ventricle 1-2 Others 5-10,Noncyanotic CHD (L R),Atrial septal defects (ASD) Ventricular septal defects (VSD) Patent ductus arteriosus (PDA) Obstruction
4、 to blood flow Pulmonic stenosis (PS) Aortic stenosis (AS) Aortic coarctation,Atrial Septal Defect,Most commonly asymptomatic Essentials of diagnosis: Right ventricular heave S2 widely split and usually fixed Grade I-III/VI systolic murmur at the pulmonary area Widely radiating systolic murmur mimic
5、king PPS in infancy Cardiac enlargement on CXR,Atrial Septal Defect,Atrial Septal Defect,Three major types Ostium secundum most common In the middle of the septum in the region of the foramen ovale Ostium primum Low position Form of AV septal defect Sinus venosus Least common Positioed high in the a
6、trial septum Frequently associated with PAPVR,Atrial Septal Defect,Treatment Closure generally recommended when ratio of pulmonary to systemic blood flow (qP/qS) is 2:1 Operation performed electively between ages 1 and 3 years Previously surgical; now often closed interventionally,Atrial Septal Defe
7、ct,Ventricular Septal Defect,Single most common congenital heart malformation, accounting for almost 30% of all CHD Defects can occur in both the membranous portion of the septum (most common) and the muscular portion,Ventricular Septal Defect,Ventricular Septal Defect,Three major types Small, hemod
8、ynamically insignificant Between 80% and 85% of all VSDs 2:1 typically undergo surgical repair at 3-6 mo,Patent Ductus Arteriosus,Persistence of normal fetal vessel joining the pulmonary artery to the aorta Closes spontaneously in normal term infants at 3-5 days of age Epi facts Accounts for about 1
9、0% of all cases of CHD Higher incidence of PDA in infants born at high altitudes ( 10,000 feet) More common in females,Patent Ductus Arteriosus,Accounts for about 10% of all cases of CHD Higher incidence of PDA in infants born at high altitudes (over 10,000 feet) More common in females,Patent Ductus
10、 Arteriosus,Patent Ductus Arteriosus,Clinical findings and course depend on size of the shunt and the degree of associated pulmonary hypertension,Patent Ductus Arteriosus,Pulses are bounding and pulse pressure is widened Characteristically has a rough “machinery” murmur which peaks at S2 and becomes
11、 a decrescendo murmur and fades before the S1,Patent Ductus Arteriosus,Treatment consists of surgical correction when the PDA is large except in patients with pulmonary vascular obstructive disease Transcatheter closure of small defects has become standard therapy In preterm infants indomethacin is
12、used (80-90% success in infants 1200 grams),Cyanotic CHD (R L),Tetralogy of Fallot (TOF) Tricuspid atresia (TA) Total anomalous pulmonary venous return (TAPVR) Truncus arteriosus Transposition of the great vessels Hypoplastic left heart syndrome (HLH) Pulmonary atresia (PA) / critical PS Double outl
13、et right ventricle (DORV),Tetralogy of Fallot,“Cyanosis, especially in the adult, is the result of a small number of cardiac malformations well determined. Oneis much more frequent than the others. This malformation consists of a true anatomopathologic type represented by the following tetralogy: (1
14、) Stenosis of the pulmonary artery; (2) Interventricular communication; (3) Deviation of the origin of the aorta to the right; and (4) Hypertrophy, almost always concentric in type, of the right ventricle. Failure of obliteration of the foramen ovale may occasionally be added in a wholly accessory manner.” Fallot, tienne-Louis-Arthur. Contribution to the pathologic anatomy of morbus caeruleus (cardiac cyanosis). Marseilles Med. 1888; 25:418-20.,
