Dr. Talaat Ali Sabeeh Al-Jarrah Pediatric Cardiologis t

Acyanotic Congenital Heart Disease (Left-to-Right Shunt Lesions) 1-Ventricular septal defects 2-Atrial septal defects 3-Atrio-ventricular septal defects ( ECD) 5-Patent ductus arteriosus 6-PS.:mild,moderate. 7-Aortic stenosis 8-Mitral stenosis/incompetence 9-Coarctation of aorta

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

Prevalence I-Cyanotic: 22% II-Acyanotic: 68% VSD 30% ASD 7-8% PDA7-8% 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

ASD 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 & Symptoms Rarely : 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 LSB. Mid-diastolic murmur heard over LLSB.

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. Types 1-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 & Symptoms 1- 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 repair II-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 intolerance hyperactive Precordium Symptoms 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 hypertension 3-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

Incomplete 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 % of cases. Types Complete Form Incomplete Form

Clinical Signs & Symptoms Complete type : same large VSD. Incomplete type: same ASD. Treatment Surgery 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

PDA – Persistence of the normal fetal vessel that joins the PA to the Aorta. Normally closes in the 1 st 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 Stenosis 2-Aortic Stenosis 3-Coarctation of the Aorta Pulmonary Stenosis Pulmonary 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 lesions Can 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 & Symptoms Depends on the severity of obstruction. Asymptomatic w/ mild PS 70mmHg. Prominent jugular a-wave, RV lift(heave) Split 2 nd hrt sound w/ a delay Ejection click, and Ejection systolic murmur. Heart failure & cyanosis seen in severe cases Treatment Mild 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

obstruction 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. Types 1-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 Syndrome Hemodynamic: Pressure hypertrophy of the LV and LA. Mild AS 20-40mmHG Moderate 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. Treatment Because 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 LSB. Cardiomegaly, rib notching on X-ray. Treatment With 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.