THE CARDIOVASCULARSYSTEM  

Dr. Talaat Ali SabeehAl-Jarrah

Pediatric Cardiologist

ACYANOTIC CONGENITAL

HEART DISEASE

Acyanotic Congenital Heart Disease(Left-to-Right Shunt Lesions)

1-Ventricular septal defects2-Atrial septal defects3-Atrio-ventricular septal defects ( ECD)5-Patent ductus arteriosus 6-PS.:mild,moderate.7-Aortic stenosis 8-Mitral stenosis/incompetence9-Coarctation of aorta

Common Cyanotic LesionsDecreased flow:Tetralogy of Fallot Tricuspid Atresia Sever PS Ebstein’s anamoly  Increased Flow: Transposition of great vessles Truncus Arteriosus Hypoplastic left heart Single ventricleTAPVR .

  

Prevalence  I-Cyanotic: 22%II-Acyanotic: 68%

VSD 30%ASD 7-8%PDA 7-8%TOF 7-8%PS 7-8%AS 5% 

Question:How you can differentiate cyanosis due to cardiac cause from pulmonary (lung) cause?

A: Hyperoxia Test

Neonates with cyanotic congenital heart disease usually do not have significantly raised arterial PaO2 during administration of 100% oxygen.

Atrial Septal Defect

Atrial Septal DefectASD is an defect in the atrial septum

permitting free communication of blood between the atria. Seen in 7-8 % of all CHD.

There are 3 major types:Secundum ASD – at the Fossa Ovalis, most

common.Primum ASD – lower in position & is a form

of ASVD, MV cleft. Sinus Venosus ASD – high in the atrial

septum, associated with partial anomalous venous return & the least common.

Clinical Signs & SymptomsRarely : signs of CHF or other CV symptoms.Most are asymptomatic but may have easy

fatigability or mild growth failure.Cyanosis does not occur unless pulmonary

HTN .Hyperactive precordium,RV heave,fixed

widely splitS2.II-III/VI systolic ejection murmur @ LSB.Mid-diastolic murmur heard over LLSB.

Treatment:1-Surgical or catherization laboratory closure is generally recommended for secundum ASD w/ a Qp:Qs ratio >2:1.2-Closure is performed electively between ages 2 & 5 yrs to avoid late complications.3-Surgical correction is done earlier in children w/ CHF or significant Pulm HTN

4-Once pulmonary HTN w/ shunt reversal occurs this is considered too late.

Mortality is < 1%. 

Question. Is endocarditis prophylaxis required for ASD?

Answer: NO

Ventricular Septal Defect

VSD abnormal opening in IVS,so

communication between the RV& LV. Accounts for 30 % of CHD.

Types1-Perimembranous (or membranous) – Most

common.2-Infundibular (subpulmonary or supracristal

VSD) – involves the RV outflow tract.3-Muscular VSD – can be single or multiple.4-AVSD – inlet VSD, almost always involves

AV valvular abnormalities.

Ventricular Septal Defect

Hemodynamics The left to right shunt ,leads to elevated RV & pulmonary

pressures & volume hypertrophy of the LA & LV.Clinical Signs & Symptoms1- Small - moderate VSD, 3-6mm, are usually asymptomatic

and 50% will close spontaneously by age 2yrs.2- Moderate – large VSD, almost always have symptoms and

will require surgical repairII-IV /VI harsh HSH murmur LSB, more prominent with

small VSD, maybe absent with a very Large VSD.• Prominent P2, Diastolic murmur.• CHF, FTT, Respiratory infections, exercise intolerancehyperactive PrecordiumSymptoms develop between 1 – 6 months

 

Treatment• Small VSD - no surgical intervention, no

physical restrictions, just reassurance and periodic follow-up and IE prophylaxis.

• Symptomatic VSD - Medical treatment initially.

Indications for Surgical Closure:1-Large VSD with medically uncontrolled

symptomatology & continued FTT.2-Ages 6-12 mo with large VSD & pulmonary

hypertension3-Age > 24 mo with Qp:Qs ratio > 2:1.4-Supracristal VSD of any size, secondary to risk

of developing AV insufficiency.

Atrioventricular Septal Defect

Atrioventricular Septal DefectIncomplete fusion the the ECD, which help to

form the lower portion of the atrial septum, the membranous portion of the ventricular septum and the septal leaflets of the triscupid and mitral valves. They account for 4% OF ALL CHD.

Question. What genetic disease is AVSD more commonly seen in?

Answer: Down’s Syndrome (Trisomy 21), Seen in 20-25% of cases.

TypesComplete FormIncomplete Form

Clinical Signs & Symptoms Complete type : same large VSD.Incomplete type: same ASD.TreatmentSurgery is always required.Treat congestive symptoms.Pulmonary banding may be required in premature

infants or infants < 5 kg.Correction is done during infancy to avoid

irreversible pulmonary vascular disease (Eisenmenger `s syndrome).

Mortality low with incomplete 1-2% & as high as 5% with complete AVSD.

 

Patent Ductus arteriosus

Patent Ductus arteriosus PDA – Persistence of the normal fetal vessel that joins the PA to the Aorta. Normally closes in the 1st few wk of life. Accounts for 7-8% of all CHD Female : Male ratio of 2:1 . Often associated with coarctation & VSD. Question. What TORCH infection is PDA associated with? Answer: Rubella

Hemodynamics Higher aortic pressure, blood shunts L to R through ductus from Aorta to PA. Extent of the shunt depends on size of the ductus & PVR. Small PDA, pressures in PA, RV, RA are normal. Large PDA, PA pressures are increasing.

Clinical Signs & Symptoms Small PDA’s are usually asymptomatic Large PDA’s : catabolic state Widened pulse pressure Enlarged heart, prominent apical impulse Classic continuous machinary systolic murmur

PDA Treatment

Indomethacin, inhibitor of prostaglandin synthesis can be used in premature infants.

PDA requires Surgical or Transcatheter closure.

Closure is required treatment heart failure & to prevent pulmonary vascular disease.

Usually done by surgical ligation or division and suturing

Mortality is < 1%.

Obstructive lesions  

1-Pulmonary Stenosis2-Aortic Stenosis3-Coarctation of the AortaPulmonary StenosisPulmonary Stenosis is obstruction in the region of either the

pulmonary valve or the subpulmonary ventricular outflow tract.Accounts for 7-8% of all CHD.Most cases are isolated lesionsCan present w/or w/o an intact ventricular septum.Question. What syndrome is PS associated with?Answer: Noonan’s Syndrome, secondary to valve dysplasia.

Hemodynamics RV pressure hypertrophy RV failure.RV pressures maybe > systemic pressure(LV pressure).Cyanosis is indicative of Critical PS.  

Clinical Signs & SymptomsDepends on the severity of obstruction.Asymptomatic w/ mild PS< 40mmHg,but in

Moderate:40-70mmHg & Sever : > 70mmHg.Prominent jugular a-wave, RV lift(heave)Split 2nd hrt sound w/ a delayEjection click, and Ejection systolic murmur.Heart failure & cyanosis seen in severe casesTreatmentMild PS no intervention required, follow-up.Mod-severe : require relieve of stenosis.Balloon valvuloplasty, treatment of choice.Surgical valvotomy is also a consideration, but

rarely needed.

Aortic Stenosis

Aortic Stenosisobstruction to the outflow from the left ventricle at or near

the aortic valve that causes a systolic pressure gradient of more than 10mmHg. Accounts for 5% of CHD.

Types1-Valvular – Most common.2-Subvalvular(subaortic) – involves the left outflow tract.3-Supravalvular – involves the ascending aorta is the least

common.Question. Which syndrome with Supravalvular AS?Answer: Williams SyndromeHemodynamic: Pressure hypertrophy of the LV and LA .Mild AS 20-40mmHGModerate AS 40-60mmHg Sever AS > 60mmHg

Clinical Signs & Symptoms

Mild AS : asymptomatic.Moderate AS :chest pain, dypsnea on exertion, dizziness & syncope.Severe AS : weak pulses, left sided heart failure, Sudden Death.LV thrust at the Apex.Systolic thrill on Rt.base/suprasternal notch.Ejection click ,and Ejection systolic murmur ,radiation to the

carotids.

TreatmentBecause surgery does not offer a cure ,it is reserved for patients with

symptoms and a resting gradient of 60-80mmHg.For subaortic stenosis it is reserved for gradients of 40-50mmHg

because of it’s rapidly progressive nature.Balloon valvuloplasty is the treatment of choice.Treatment Aortic insufficiency & re-stenosis is likely after surgery

and may require valve replacement.Activity should not be restricted in Mild AS.Mod-severe AS, no competitive sports.

Coarcotation Of Aorta

Coarctation- is narrowing of the aorta at varying points anywhere from the transverse arch to the iliac bifurcation.

98% of coarctations are juxtaductal Male: Female ratio 3:1. Accounts for 7 % of all CHD.Question: What other heart anomaly is coarctation

associated with?Answer: Bicuspid aortic valve, seen in > 70% of

cases.Question: What genetic syndrome is coarctation seen

in?Answer: Turner’s Syndrome.Hemodynamics Obstruction of left ventricular outflow pressure

hypertrophy of the LV.

Coarcotation Of Aorta

Clinical Signs & Symptoms

Classic signs: absence,weakness or delayed femoral pulses.Higher BP in the upper extremities than lower extremities.90% have systolic hypertension of the upper extremities.Pulse discrepancy between Rt. & Lt arms.With severe coarc. hypoperfusion, acidosis, HF and shock.II/VI systolic ejection murmur @ LSB.Cardiomegaly, rib notching on X-ray. TreatmentWith severe coarctation maintaining the ductus with

prostaglandin E is essential.Native:Surgical intervention, to prevent LV dysfunction.Angioplasty is used by some centers.

Re-coarctation can occur, balloon angioplasty is the procedure of choise.

Thanks