Dr. aso faeq salih

Pediatric cadiologist

2013-2014

Congenital heart disease

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 : 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 DuctusArteriosus ( 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.