CONGENITAL HEART

DISEASESDr Swati Prashant

MD Paediatrics

Index Medical College, Indore,MP,India

drprahantw@gmail.com.

www.paediatrics4all.com

LEFT TO RIGHT SHUNTS The most common L →R Shunts

are : 1. VSD : 27% 2. ASD : 13 % 3. PDA : 11 % .

ATRIAL SEPTAL DEFECT It constitutes 13 % of all CHD . There is an abnormal

communication between the 2 Atrias .

ASD’ s are of 3 types . 1 Ostium Secundum defect :

70% .Defect is at the fossa Ovalis or rarely superior or Posterior to fossa .

2. Ostium Primum defect : 30% . Defect is

ATRIAL SEPTAL DEFECT Defect is an Endocardial Cushion

defect lying Inferior to fossa . It may be associated with Mitral Valve defect .

3. Sinus Venosus defect : 10% ,associated with defect at entry of SVC in Rt. Atrium .

ATRIAL SEPTAL DEFECT Haemodynamics 1. Oxygenated blood from Lt Atrium ↓ Right Atrium It receives extra blood , causing Right Atrial enlargement ↓ Large volume of Blood passes

through Normal Tricuspid Valve

ATRIAL SEPTAL DEFECT Causing Delayed Diastolic Murmur ( DDM ). ↓ large Volume is received by RV Rt. Ventricle enlarges ( cardiac impulse ↓ Large vol . Thru. Pulmonary

Artery causes Ejection Systolic Murmur & delayed closure of P2 , Therefore A2 --

P2 WIDE split & loud p2 . As age

advances PH OCCURS .

CLINICAL FEATURES Mild effort intolerance Chest infections CCF Rare . Parasternal Impulse A2—P2 Wide split fixed Systolic Thrill & Murmur in P2

area due to flow thru. Pulmonary valve .

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ATRIAL SEPTAL DEFECT Complications are rare After age 20 yrs. PH occurs . ECG---RVH & RBB X-Ray---mild cardiomegaly ,

RAH ,RVH ,PA prominent , plethora.

ATRIAL SEPTAL DEFECT TREATMENT : 1. T/t of Infections , ccf 2. Surgery Common syndromes asso. With

ASD : Down’s Syndrome , Holt Oram

syndrome , Lutembachker , Noonans syndrome .

VENTRICULAR SEPTAL DEFECT It is most common amongst the CHD

. Constitutes 27% of all CHD’s . Location : 90% of VSD are in

Membranous part of the Septum Others occur in Muscular part

& can be multiple . Syndromes: Trisomy 13 - 15 , 17-

18. Absent Radius & Ulna , poly & Syndactyly .

VENTRICULAR SEPTAL DEFECT HAEMODYNAMICS Left → Right shunt . Lt. Ventricle blood →enters Rt.

Ventricle through the defect . At the same time Rt. Ventricle is also

contracting. So the blood is almost directly going to Pulmonary Artery .

Large vol. Thru. PA → CAUSE Ejection Sys. Murmur + delayed P2 , due to delayed empting .Also there is early empting of LV causing early A2 .

VENTRICULAR SEPTAL DEFECT Therefore there is a wide split

A2 P2 . ↑ blood in LA causes LA

ENLARGEMENT. ↑ blood flow thru. Mitral valve

causes DDM at apex . Shunt itself causes PANSYSTOLIC

Murmur as blood is going thru. The shunt in systole ----in Tricuspid area --lt. Sternal border 3,4,5 space .

CLINICAL FEATURES-VENTRICULAR SEPTAL DEFECT Symptomatic around 6 –10 wks. CCF develops . Palpitation , dyspnea on exertion . Frequent chest infections . Wide pulse pressure . Hyperkinetic precordium with

systolic Thrill . Cardiomegaly with Left

ventricular Apex .

VENTRICULAR SEPTAL DEFECT Wide split 2 nd HEART SOUND P2 accentuated Pansystolic Murmur at Lt.

Sternal border ( 3 ,4 ,5th IC SPACE .

ECG : 1) RVH initially & in newborn .

2) IN small & mod . Size VSD ,RVH comes to normal after ↓ of pulmonary resistance .

VENTRICULAR SEPTAL DEFECT 3) In large VSD without PAH

there is LVH 4) In large VSD + PS /PAH : ECG

shows RVH + LVH or purely RVH . X-RAY CHEST 1. LVH—Depends on size of

shunt . 2. Plethora 3. Aorta N or small in size .

VENTRICULAR SEPTAL DEFECT 4. LAH in large shunts . 5. If VSD is small : Heart size

normal, pulmonary vasculature is normal .

6. If VSD + PS : Heart size is normal , normal lung fields .

7. If VSD + PAH : Heart size is normal ,but lung fields are Plethoric .

ASSESSMENT OF SEVERITY Small VSD : PSM + normal P2 ,

disappearance of murmur + ECG becomes Normal .

Large VSD : RV pressure = LV pressure , therefore murmur becomes softer + PAH + accentuated P2

Large VSD + PS : ejection systolic murmur +↑ RV pressure + normal PA pressure + P2 soft

TREATMENT-VENTRICULAR SEPTAL DEFECT Medical : T/t --CCF , Infections ,

Anemia , Endocarditis . Surgery : Indications 1. CCF in infancy not responding to

medical t/t . 2. L→ R shunt is large 3. VSD ( large) + PS / PH or AR . 4. Surgery : contraindicated in PAH

+ reversal of shunt .

VENTRICULAR SEPTAL DEFECT Surgery : Closure of VSD WITH A

Dacron patch , through Rt. Atrial approach .

Surgery is advised if PAH develops , within 2 yrs.

Complications of Surgery : Complete Heart Block , residual

VSD .

PATENT DUCTUS ARTERIOSUS It is a communication between

the Pulmonary Artery & the Aorta .

Aortic attachment is just distal to the Left Subclavian Artery .

Ductus arteriosus is normally present in fetal life .

It closes normally after birth . It constitutes 11% of all cardiac

defects .

HAEMODYNAMICS- PATENT DUCTUS ARTERIOSUS L→R shunt from Aorta to

Pulmonary Artery . Flow is both during systole as

well as Diastole , as pressure is always higher in Aorta with normal Pulm . Artery .

This L →R shunt causes murmur . Murmur starts in systole after 1st HS & Continues in Diastole but with diminished intensity , therefore Continuous murmur.

PATENT DUCTUS ARTERIOSUS LA receives large amt. of

blood ,therefore LA enlarges In size .

↑ blood flow through Mitral valve -> causes accentuated 1st HS + DDM .

LV also receives more blood → overloading → prolongation of lt. Ventricular systole & ↑ in LV size .

Prolonged systole → cause delayed closure of Aortic valve ---late A2 .

PATENT DUCTUS ARTERIOSUS Late A2 causes paradoxical split

in large shunts . Large vol. Coming to Aorta causes

Aortic dilatation ( ascending ) , this causes Ejection click & Ejection systolic murmur , but this is masked by continuous murmur .

CLINICAL FEATURES- PATENT DUCTUS ARTERIOSUS Patient becomes symptomatic

early in life . Develops CCF around 6-10 wks of

life , or even earlier within 7 days of birth with murmur + ccf .

In older children there is effort intolerance , palpitation , chest infections .

PATENT DUCTUS ARTERIOSUS As there IS a leak of blood to

PDA from systemic blood there is a wide pulse pressure + collapsing pulse .

Prominent CAROTID pulsations + features L → R shunt is s/o PDA .

Cardiac impulse & Apex Beat are Hyperkinetic s/o LVH due to ↑ blood Volume .

PATENT DUCTUS ARTERIOSUS Continuous / systolic murmur +

Thrill at Lt. 2nd space . SO IF SHUNT IS LARGE : 1. 1 st HS is accentuated due

to ↑ Mitral flow . 2. 2 nd HS is narrow

/paradoxically split 3. P2 is louder than normal . Continuous murmur best heard

in P2 AREA

PATENT DUCTUS ARTERIOSUS ECG : LVH--- ‘ Q’ & tall ‘T’ waves

are characteristic of Lt . Ventricular vol. Overload .

X-Ray chest : cardiomegaly with LV enlargement .( large shunt -- large size, large shunt --narrow split , small shunt --- no split .)

LA enlarged , Ascending Aorta ( knuckle) prominent .

COURSE & COMPLICATIONS In Newborn & infants ---PH is +nt

at birth causing Ejection syst. Murmur .

Later as PH ↓ the murmur becomes continuous .

CCF same as in VSD . In PDA ,PH later due to flow

develops earlier than VSD . As PH develops later diastolic

component ↓ ,so the murmur becomes Ejection syst. Murmur .

If PH --P2 is loud + DDM +nt If PS --P2 is soft or N + no DDM If L→ R becomes R→L there is no

murmur , but DIFFERENTIAL CYANOSIS is present

In PDA + PH causing reversal .

TREATMENT- PATENT DUCTUS ARTERIOSUS For closure of PDA 1. Indomethacin ( prostaglandin

synthetase inhibitor ) given orally Dose is 0.1 mg /kg / day 12

hourly in 3 doses. Hepatic / Renal / Bleeding tendency----

CI 2. Surgical ligation PDA .

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