Acyanotic heart disease& Fetal circulation

Guided by, Dr.K.Sivakanthan Consultant Paediatrician Dr.Arulnithy Consultant Cardiologist

FETAL CIRCULATION

The Placenta Facilitates gas and nutrient exchange between maternal and fetal blood.

Umbilical Circulation

Umbilical vein carries oxygenated blood and nutrients from the placenta. Pair of umbilical arteries carry deoxygenated blood & wastes to placenta

Oxygenated blood(PO2 30-35mmHg) returning from the placenta flows to the fetus through the umbilical vein. Approximately 50% of the umbilicus venous blood enters the hepatic circulation. Whereas the rest bypasses the liver & joins the IVC via the ductus venosus. This combined lowerbody +umbilical venous bloodflow(PO2 26-28mmHg) enters the right atrium. Blood is directed across the foramen ovalae into the left atrium,then flows into the left ventricle& ejected into the aorta.

 Fetal SVC blood(less oxygenated PO2 12-14mmHg) enters the right atrium& traversus the tricuspid valve &flows primarily to the right ventricle. From the right ventricle it is ejected into the pulmonary artery. Only about 10% of R.V outflow enters the lung. The major portion of this bypasses the lungs & flows through the DUCTUS ARTRIOSUS into the descending aorta to perfuse the lowerpart of the body.

After that it returns to the placenta via the 2 umbilical arteries.

 Approximately 65% of descending aorta blood flow returns to the placenta,the remaining 35% perfuses the fetal organ & tissues.

Note:Upper part of the fetal body is perfused exclusively from the L.V with the blood that has a slightly higher PO2 than the blood perfusing the lower part of the fetal body(derived mostly from the R.V)

What happens at birth?

The change from fetal to postnatal circulation happens very quickly. Changes are initiated by babys first breath.

 First Breath Umbilical vessels are clamped and cut This lowers the pressure system of the placenta from fetal circulation
No placental function pO2 and pCO2 and pH Causes infant to inhale Infant has to generate large negative pressure to overcome resistances Fluid expelled on first exhalation Alveoli expand increases O2 and causes vasodialation All of this to decrease Pulmonary vascular resistance.

Pressures in the right heart& left side Foramen ovale closes Closed foramen ovale promotes more pulmonary Blood flow.
Aortic pressure >pulmonary Artery pressure so shunting via ductus aretriosus decreases until fully closes Left ventricle is now coupled to the high resistance systemic circulationwall thickness&mass Right ventricle is coupled to the low resistance pulmonary circulationwall thickness&mass In fetus-LV pumps blood to the upper part of the body After birth-it delivers to the entire system.

Foramen ovale

Closes shortly after birth, fuses completely in first year. Ductus arteriousus Closes soon after birth, becomes ligamentum arteriousum in about 3 months. Ductus venosus Ligamentum venosum

Umbilical arteries
Umbilical vein

Medial umbilical ligaments

Ligamentum teres

 When congenital structural defects are superimposed on these dramatic physiologic changes,they often impede this smooth transition&markedly increase the burden. Ductus arteriosus&foramen ovalae may remain patent in certain congenital cardiac lesions. Patency of the pathwayslifesaving pathway for blood to bypass a congenital defect.eg 1),A patent ductus in pulmonary atresia or coarctation of aorta. 2) Foramen ovalae in transposition of greatvessels May provide additional stress to the circulation. eg,PDA in a premature infant Pathway of right-left shunting in infants with pulmonary hypertension.

ACYANOTIC HEART DISEASE

ACYANOTIC HEART DISEASE

Left to right shunts Atrial septal defect Ventricular septal defect Patent ductus arteriosus

Obstructive Aortic stenosis Coarctation of the aorta Pulmonary stenosis

ATRIAL SEPTAL DEFECT

 It is an abnormal communication between the 2 atria. Can occur any portion of the atrium septum (secundum,primum,sinus venosus) Females>Males 2 Main types 1. Ostium secundum(75%) 2. Ostium primum

Common form Ostium secundum:-involves fossa ovalis in the atrial midseptum Can also be superior/postreior to the fossa ovalis. Ostium primum defect(endocardial cushion defect)-is situated inferior to the fossa ovalis. Associated with a cleft in the antrior leaflet of mitral valve with or without a cleft in the septal tricuspid leaflet.

Pathophysiology
The degree of L-R shunting is dependent on the :* size of the defect * Relative vascular resistance in the pul &sys circulations * Relative compliance of the R&L ventricles In large defects,aconsiderable shunt of oxygenated blood flows from the L-R atrium & is added to the usual venous return to the R.A & is pumped by the R.V to the lungs. In infants-R.V is muscular & less compliant,thus limiting the LRshunt As the infant becomes older-Pulmonaryvascular resistance dropsR.Vwall becomes thinner &L-R shunt Due to the large blood flow-Enlargement of R.A&R.V &dilatation of the pulmonary artery. L.A may be enlarged,the L.V & aorta normal in size.

Haemodynamics
Leaking of oxygenated blood from the L-R atrium at a minor pressure difference between the 2 atria.-silent in auscultation. R.atrium receives blood from SVC,IVC &shunted bloodR.A enlargeslarge volume passes through a normal tricuspid valve-Delayed diastolic murmur R.V enlarges to accommodate the large volume passes through a normal pulmonary valve-Pulmonary ejection murmur &prologed ejection phase P.valve closes later-Delayed P2

CLINICAL FEATURES
Symptoms Asymptomatic Recurrent chest infections/wheeze Heart failure Arrythmias(4th decade onward)

Signs S1-normal S2-Fixed & widely split Ejection systolic murmur-best heard at the upper left sternal edge. With a partial AVSD-an apical pansystooic murmur from AV regurgitation

INVESTIGATIONS
Chest X ray-May show cardiomegaly enlarged pulmonary arteries pulmonary vascular markings

 ECGSecundum ASD-partial bundle branch block(may occur in normal children) Right axis deviation due to the R.V enlargement. Partial AVSD-Left axis deviation(Superior QRS axis)

EchocardiographyR.V volume overload Location & size of the defect. Shunt is confirmed by color flow doppler

TREATMENT
Medical management Treating chest infections Infective endocarditis-rare unless associated with floppy mitral vale Surgical management Surgical or transcatheter device closure-advised for all symptomatic &asymptomatic patients. Timing for Sx-After the 1st year&before the entry into school

PROGNOSIS
May close spontaneously Secundum ASD- well tolerated during childhood Results after surgical or device closure-Excellent

Ventricular septal defect

VENTRICULAR SEPTAL DEFECT

The lesion consists of an abnormal opening between the right & left ventricles which may vary in size from miniscule hole to complete absence of the septum,resultig in a common ventricle.

Incidence

Most common cardiac malformation. Accounts for 25% of congenital heart diseases. Often accompanied by other cardiac defects.

PATHOPHYSIOLOGY
Depends on the size of VSD & Pulmonary vascular resistance in relation to systemic vascular resistance. Smaller communication present-RESTRICTIVE VSDRight ventricular pressure is normal. Higher pressure in the L.V drives the shunt left to right. Larger defects(>1cm2)-NONRESTRICTIVE VSDRight &Left Ventricular pressure is equalised. Direction of shunt & Magnitude is determined by the ratio of pulmonary to systemic vascular resistance.

CLINICAL MANIFESTATIONS
. Physical signs and symptoms depend on the size of the lesion& the presence of associated cardiac lesions. Clinical symptoms are usually not seen at birth-continued high pulmonary vascular resistance in the newborn. Infants with moderate to large defects will become symptomatic within the first few weeks of life. Children with small defects will remain asymptomatic.

Small lesions are associated with Loud PSM because of high pressure velocity Up to 60% close spontaneously by 5yrs Surgical closure is not usually undertaken

Large defects Present with heart failure in infancy There is

Failure to thrive Breathlessness Poor feeding

Signs Precordium is hyperkinetic with a systolic thrill at the left sternal border S1 & S2 are masked by a pansystolic murmur at left sternal edge. S2-widely split &variable with accenuated P2 Loud pansystolic murmur heard at the left sternal border Maximum intensity in the 3rd ,4th or 5th intercostal space.

COMPLICATIONS
Congestive heart failure. Growth failure ,especially in infancy. Bacterial endocarditis. Irregular heart beat or rhythm. Complete heart block . Pulmonary artery hypertension. Aortic insufficiency.

DIAGNOSTICS
Chest X Rays---looks to see if there is a large heart with fluids in the lungs. ECG--- shows signs of an enlarged left ventricle. Echocardiogram---used to make definitive diagnosis.

Cardiac catheterisation---(rarely needed unless there are concerns of high blood pressure in the lungs,in which case surgery to close the defect is generally not recommended.
MRI of the heart---used to find out how much blood is getting to the lungs.

Chest x ray
Small VSDs, the chest radiograph is usually normal

Large VSD: The presence of right ventricular hypertrophy, olegeimic lung fields (pulmonary hypertension or an associated pulmonic stenosis), gross cardiomegaly with prominence of both ventricles, the left atrium.

ECHOCARDIOGRAM

TREATMENT
Medical management To control congestive cardiac failure Treatment of repeated chest infections. Prevention &treatment of Infective endocarditis. The patients should be followed caefully to assess the devlopment of either pulmonic stenosis,pulmonary areterial hypertension or aortic regurgitation.

 Surgical treatment
Indicated Congestive cardiac failure occurs in infancy &is not responding to medical management. The left to right shunt is large(pulmonary flow more than twice the systemic flow0 If associated with pulmonary stenosis,pulmonary arterial hypertension/aortic regurgitation.

Note:It is not indicated * In patients with a small ventricular septal defect *Patients who have developed pulmonary arterial hypertension of a severity that a right to left shunt has appeared.

Closure of the ventricular septal defect with the use of dacron patch can performed through the right atrium or through a right ventriculotomy

Major complications of ventricular defect surgery Complete heart block Bifascicular block Reopened or residual ventricular septal defect.

Patent ductus arteriosus

It is a communication between the pulmonary artery& the aorta. Ductus Arteriosus-present in all fetus it closes functionally&anatomically. Persistant of ductus arteriosus, which allows oxygen-rich (red) blood that should go to the body to re-circulate through the lungs .

IN GROSS

Haemodynamics
Left to right shunt occurs both during systole &diastole. Continous murmur

Large amount of blood passes through pulmonary artey to left atrium & through mitral valve.

Accentuated S1 Mitral delayed DIASTOLIC murmur

 Large flow through left Late A2 ventricle-Delayed closure Paradoxically split of aortic valve. S2 Dilatation of ascending aorta. Aortic ejection click. Aortic ejection systolic murmur.

Clinical features
May become symptomatic in early life& develop

congestive cardiac failure. Older chidren present withFrequent chest infections fatigue sweating rapid breathing heavy breathing congested breathing Not interest in feeding, or tiring while feeding poor weight gain

ON EXAMINATION
Inspection Prominent Carotid pulsations Hyperkinetic & left ventricular type of apical impulse
Palpation Systolic or continous thrill at 2nd lt interspace

AUSCULTATION Accentuated s1 Narrowly or paradoxically split s2

Continous/gibsons/train-in-tunnel murmur best heard in infraclavicular region

Mitral delayed diastolic murmur

Diagnosis
1)CXR Cardiomegaly Left atrium enlargement Left ventricle enlargement Prominent aortic knuckle Pulmonary plethora 2)ECG Normal axis with lt ventricular hypertrophy

MANAGEMENT
MEDICAL INDOMETHACIN 0.1 mg/kg/dose,orally,bd for three doses contraindications-Hepatic or Renal insufficiency Bleeding tendency Digoxin for increasing working capacity of heart Diuretics to reduce preload on heart

SURGICAL REPAIR :DEVICES

REPAIR

AORTIC STENOSIS

Types
Congenital aortic stenosis (more common)

Valvular (70%) Degeneration of bicuspid valve Subvalvular (30%) Supravalvular

Acquired aortic stenosis Rheumatic valvulitis

Almost invariably associated with mitral valve disease

Fibrocalcific senile aortic stenosis

Degenerative

Location
Supravalvular Subvalvular Valvular

Valvular Congenital bicuspid valve-Most common cause in young More prone to calcification Develop symptoms in their 30s&40s. Unicuspid valve.

Schematic of AV

Tricuspid (left) and Bicuspid (right) Aortic Valve.

Subvalvular AS
Below the aortic valve. Discrete membrane or fibromuscular shelf obstructing the Left ventricle outflow. May also be due to markedly hypertrophied ventricular septum in association with hypertrophic cardiomyopathy. Aortic regurgitation frequently accompanies

Supravalvular
Ascending aorta superior to the aortic valve is narrowed Least common. May be sporadic,familial or associated with Williams syndrome. Elfin facies Hypercalcemia Peripheral pulmonic stenosis Mental retardation.

Pathophysiology
When the aortic valve becomes progressively stenotic ,a pressure gradient is created between the left vetricle& the aorta. Initially the left ventricle compensates by thickening of its wall in order to maintain adequate systolic function&to overcome the afterload-concentric hypertrophyIn the later stages, the left ventricle dilates due to increased wall stress, and the systolic function deteriorates & leads to left ventricle failure.

Hemodynamics

Clinical Presentation
Asymptomatic for many years but heart murmur detected on physical exam Classical triad:exertional dyspnea, angina, syncope Occasionally pt with end stage aortic stenosis and LV dysfunction present with anasarca and cardiac cachexia

Palpation Narrow pulse pressure.-most severe Apical impulse-forcible or heaving A systolic thrill is palpable at the 2nd right intercostal space,suprasternal notch,& the carotid arteries. Auscultation S1-normal S2-A2 delayed P2-normal S3-with left ventricular failure S4-audible in severe aortic stenosis Ejection systolic murmur starting after the ejection click reaches a peak in middiastole. In severe Aortic stenosis-the peak gets delayed.

Aortic Stenosis: Physical Findings

S1 S2 Mild-Moderate

S1

S2 Severe

Diagnosis
ECG-reveals left ventricular hypertrophy. Presence of ST & T wave -severe aortic stenosis. Thorasic roentgenogramValvular AS-normal sized heart,dilated ascending aorta. Supravalvular&subvalvular AS-may be normal.

EchocardiographyBest noninvasive test for evaluate the aortic valve anatomy&function. May also show LV hypertrophy,thickened &immobile aortic valve&dilated aortic root. Doppler excellent for assessment of severity

 Chest x rayShowing calcific aortic valve In longstanding disease, enlarged left ventricle and atrium.

Treatment
Symptoms should be carefully evaluated. BP &pulse pressure on each visit. Careful evaluation of the 2nd heart sound &its record should be maintained. An ECG should be obtain every 6months to a year. Doppler Echo can be used to quantitate the gradient at each visit. The patients as a rule should be discouraged from outdoorgames,athletics,competitive sports&strenous exercises.

Balloon Aortic valvuloplasty-non operative relief of Aortic obstruction.

Surgical treatment
2 main operations-Aortic valvotomy &Aortic valve replacement.

Bioprosthetic valve

Disc valve

Coarctation of the Aorta

FUNCTION OF THE AORTA

It is the bodys main artery.

It distributes oxygen-rich blood to all body parts except lungs.

 It is a narrowing of part of the aorta between the upper part of the body artery branches and the branches of the lower body. 98% occurs just below the origin of the left subclavian artery at the origin of ductus arteriosus Males>Females May be a feature of Turner syndrome

 Juxtaductal coarctation(adult type/Post ductal) Narrowing of the descending aorta just beyond the remnant of ductus arteriosus. 95% of all coarctation cases. Net left-right shunting occurs. 70% of patients have bicuspid valve. Collaterals develop. Tubular hypoplasia(infantile/Preductal) More diffuse narrowing of transverse aorta&aortic isthmus. Proximal to patent ductus arteriosus.

Haemodynamics
Preductal(infantile)&Postductal(adult)coarctation-depends on the absence or presence of collateral anastomosing vessels. Isthmus-Portion of the aorta distal to the left subclavian.A& before the portion where the ductus arteriosus joins-at birth most narrowest part of the aorta. In Normal fetal lifeOutflow of R.V Ductus Arteriosus Descending aorta Most of the L.V outflow Innominate,L.Carotid & Subclavian.A In Fetus: Preductal Coarctation does not interfere with normal haemodynamics. collaterals are not formed.

Postductal coarctation-interferes with the R.v output reaching the descending Aorta. Stimulates collaterals even in the fetal life. After birthR.V output Pulmonary vessels No flow from pulmonary .A Descending Aorta. In preductal coarctation no collaterals ,the neonate become symptomatic immediately. In postductal coarctation-already have some collaterals & are spared from developing

Clinical features
Usually there arent any symptoms, but if obstruction is severe, you may: Not tolerate exercise. Have headache. Have leg pain after exertion. Have chest pain or palpitation. Dizziness, shortness of breath, nosebleed, cold feet or legs. Pounding headache

 Disparity in pulsation & Blood pressure in the arms & legs Lower limb pulses are weak Bounding pulses in the upperlimbs. Radiofemoral delay.

DIAGNOSIS
Test to diagnose this condition may include: Physical exam Take blood pressure in arms and legs Check pulse in the femoral area Chest x-ray Electrocardiography MRI of the chest Cardiac Catheterization and aortography Doppler ultrasound of the aorta

Complications
Congestive heart failure-infancy Infective endocarditis-Wall of the aorta distal to the coarctation Or the bicuspid aortic valve. Rupture of berry intracranial aneurysm. Rupture of an intercostal aneurysm. Dissection of aorta.

TREATMENT
Surgery is usually recommended The narrowed part of the aorta will be removed. If the problem area is small, the two free ends of the aorta may be reconnected. This procedure is called Anastomosis. If a large part of the aorta is removed, a Dacron Graft (synthetic material) is used to fill the gap. In some cases, balloon angioplasty may be done instead of surgery.

Pulmonic stenosis

Located at the valvular or subvalvular(infundibular pulmonic stenosis) Uncommonly pulmonic stenosis may be in the pulmonart artery above the valve(supravalvular) or in the main Right & Left branches(Right &left main branch stenosis),the peripheral branches(peripheral pulmonic stenosis) Constitutes approximately 2% of congenital cardiac defects.

pathophysiology
Obstruction to the outflow from the right ventricle to the pulmonary artery systolic pressure & wall stress

Hypertrophy of the right ventricle Arterial O2 saturation will be normal ,unless an intracardiac communication(VSD/ASD)

haemodynamics
Flow across the narrow pulmonary valve-pulmonary ejection systolic murmur. Right ventricle sytole is prolonged-delayed closure of the pulmonic component of the S2,this results in widely split S2. Split is variable becoming wider in inspiration. Width of splitting in expiration& duration of the systolic murmur are directly related to the severity of obstruction.

Clinical features
Symptoms Most patients are asymptomatic. In severe pulmonary stenosis-exertional dyspnoea palpitation easy fatiguability angina like chest pain(rarely) Signs Characteristically having a round face& hypertelorisim. Port-wine angiomatous malformation-skin Absence of cyanosis-except in severe pulmonary stenosis with right to left shunt at atria level.

Left parasternal heave.-hypertrophied R.V Palpable systolic thrill-2nd&3rd left intercostal space. S1-normal S2-P2 delayed&softer widely split with normal movement S3-RV failure S4-with severe stenosis. Murmur-Ejection systolic murmur.

Diagnosis
ECG-Right axis deviation &Right ventricular hypertrophy. Thorasic roentgenogram-normal sized heart,normal pulmonary vasculature,post stenotic dilatation. Echocardiogram-site Doppler-assess the severity of the obstruction.

Infundibular pulmonary stenosis is separated from valvular pulmonic stenosis *Absence of click in infundibular type *Absence of post stenotic dilatation in the thorasic roentgenogram. *A relatively lower point of maximum intensity of the systolic murmur in the 3rd&4th left interspace.

Treatment
Mild pulmonary stenosis often does not require treatment. Moderate or severe stenosis is treated with repair of the obstructed valve.

At cardiac catheterisation75% or more of systemic systolic pressure-should be operated <50%-can be followed without operation.
Balloon pulmonary valvuloplasty-Rx of choice for isolated valvular pulmonary stenosis.