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Congenital heart disease Dr. aso faeq salih Pediatric cadiologist 2013-2014 Ventricular Septal ( VSD ) Defect Most common cardiac malformation 25-30 % Types of VSD : According to position perimembranous , inlet , muscular . According to size small , medium , large . Membranous : most common , are usually single ,( called peri membranous ) may extend into adjacent muscle Muscular : mid portion of septum to the apex . Single or multiple (Swiss cheese septum ) Inlet : At level of both Av valve s Size of defect : Small (restrictive ) : Trivial L R shunt . (LV pressure > RV ) Normal pulmonary arterial &RV pressure . Normal cardiac chambers . Large (non restrictive ) : > aortic annulus RV, LV pressure equalizes . Direction & magnitude of shunt determined by ratio of pulmonary to systemic vascular resistance . RV , pulmonary arterial hypertension . Main pulmonary artery , LA , LV are enlarged Medium will be in between Pathophysiology : Clinical features : Varies according to : size of defect , pulmonary blood flow & pressure . Small VSD : Most often asymptomatic . Loud , harsh , blowing , holosystolic murmur heard best over LLSB frequently accompanied by thrill . Large VSD : Dyspnea , feeding difficulties , poor growth , profuse perspiration , recurrent chest infection & cardiac failure in early infancy . Cyanosis usually absent , duskiness noted during crying or infection . Physical signs : Prominent L precordium , palpable para sternal lift . Lateral displacement of apex beet , apical thrust . Holosystolic murmur ( less harsh , more blowing ). Pulmonary component of S2 may be increased pulmonary hypertension Investigations : CXR : Small VSD : normal or minimal cardiomegaly . borderline increase in pul. Vasculature . Large VSD : gross cardiomegaly ( RV , LV, LA PA ). prominent pulmonary vascularity . ECG: Small VSD : normal or may suggest LV hypertrophy Large VSD: biventricular hypertrophy P- wave notched or peaked . Echocardiography : Cardiac catheterization Treatment : Small VSD: Reassurance & encourage to live normal life with no restriction of activities . Protection against infective endocarditis . Regular follow – up Large VSD : Aim of treatment : Control the symptoms of H.F . Prevent the development of pulmonary vascular disease . Surgical closure of defect : Indications : 1. Patient at any age with large defect in whom clinical symptoms , FTT cannot be controlled medically . 2. Supracristal VSD . 3. VSD complicated with AR or subvalvular PS Complication of surgery : Residual defect . Heart block . Prognosis & complications : Small VSD : Spontaneous closure : 30 – 50 % most often during first 2 years of live ( small muscular are > likely to close ( up to 80 % ) than membranous (up to 35 % ) . Most often asymptomatic . Infective endocarditis . Moderate – Large VSD : • Early & successful therapy may become smaller & • • • • • • up to 8 % may close completely . Repeated episodes of chest infection . H.F & FTT . Pulmonary HT & evidence of pulmonary vascular disease . Eisen menger complex . Aortic valve regurgitation Acquired infundibular pulmonary stenos is . Patent Ductus Arteriosus ( PDA) 6 – 8 % of CHD , F:M 2 : 1 Ass. With maternal rubella infection in early pregnancy . Common problem in premature infants . Ductus Arteriosus : Fetal life , patency of Ductus is maintained by : Relaxant effect of low O2 tension . Prosta glandines (E2) . •In full term neonates , once Po2 passing through Ductus reaches 50 mmHg Ductal wall constricts . Functional closure of Ductus 10 – 15 hrs. in normal neonate , anatomical occlusion 4 m of age Ligamentum arteriosum Pathophysiology : Types &clinical manifestations : Small PDA : Usually asymptomatic . Normal cardiac size . Pressure within PA , RA & RV are normal . Large PDA : PA pressure may be elevated to a systemic pressure . Risk of pulmonary vascular disease . Often symptomatic ( HF & growth retardation ). Bounding peripheral pulsations . Wide pulse pressure . Moderate – gross cardiomegaly . heaving apical impulse. Thrill (systolic ) max. in 2nd L ICS +/_ radiation . Machinery continuous murmur max. in 2nd L ICS . Investigations : CXR : Small PDA : normal . Large PDA : moderate – gross cardiomegaly ( LV , LA ). Prominent intra pul. Vascular marking . normal or prominent aortic knob . ECG : Small normal. Large LV or biventricular hypertrophy. Echocardiography : Cardiac Catheterization : Prognosis & complications : Small PDA : May live a normal span with a few or no symptoms . Spontaneous closure after infancy is extremely rare. Infective endocarditis . Large PDA : HF in early infancy , FTT . Infective endocarditis . Pulmonary or systemic emboli . Treatment : Surgery : Ligation & division of Ductus , preferably before 1st year of live . Trans catheter closure of defect.