Congenital heart disease Dr. aso faeq salih Pediatric cadiologist 2013-2014

Ventricular Septal Defect ( VSD )

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 : Physical signs : 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 : ECG: 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 : Patient at any age with large defect in whom clinical symptoms , FTT cannot be controlled medically . Supracristal VSD . 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 Ductus Arteriosus : 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.