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Congenital heart disease systemic diseases (such as acute rheumatic carditis and viral myocarditis) Cardiomyopathy Tachyarrhythmi post

cardiac surgery

Congenital heart disease are heart problems that develop before birth. They can occur in the heart's chambers, valves or blood vessels. A baby may be born with only one defect or with several defects. Of the dozens of heart defects, some are mild and may need little or no medical treatment even through adulthood. Other types of congenital heart defects are life-threatening, either immediately to the newborn or over time

Commonly

presents with Central cyanosis Heart failure Sudden collapse Heart murmur

Bluish discoloration of lips and mucous membrane Confirmed by pulse oxymetry(spo2<85%)or ABG Must be distinguished from peripheral cyanosis; look into oral mucouse membrane, tongue and conjunctiva Caused by excess deoxygenated haemoglobin(>5Gm/dL)

Cyanotic heart diseases- obstructed pulmonary flow(TOF,pulmonary atresia - common mixing(single ventricle,truncus arteriosus, tricuspid atresia, total anomalous pulmonary venous drainage(TAPVD)) Primary pulmonary diseases Persistent pulmonary hypertension of newborn(PPHN) Central nervous depression Severe polycythaemia methaemoglobinaemia

Clinical presentation may mimic pulmonary diseases or sepsis.


Tachypnoea Tachycardia Hepatomegaly Weak pulses

Can be difficult to be distinguished from sepsis or metabolic disorders.


Hypotension Metabolic acidosis Extreme cyanosis oliguria

Identification of a heart murmur during routine newborn examination is common Absence of heart murmur does not exclude serious structural heart problems(e.g TGA,TAPVD,AVSD) Heart murmur can be innocent or pathological

Pulmonary flow murmur Transient systolic murmur from closing ductus arteriosus Transient systolic murmur from tricuspid regurgitation due to high pulmonary vascular resistance

History
Antenatal scan(cardiac malformation,fetal arrhytmias,hydrops) Family history of congenital heart disease Maternal illness:diabetis,rubella,teratogenic medications Perinatal problems:prematurity,meconium aspiration,perinatal asphyxia

Physical examination
Dysmorphism Central cyanosis Differential cyanosis(spo2 lower limb<upper limbs) Tachypnoea Weak or unequal pulses Heart murmur hepatomegaly

Investigations
Chest x-ray
Cardiomegally Oligaemic lungs-TOF,pulmonary atresia Plethoric lungs-TGA,truncus arteriosus Pulmonary oedema-obstructed TAPVD Abnormal abdominal situs,dextrocardia-heterotaxy syndrome

Hyperoxia test

administer 100% oxygen in headbox at 15L/min for 15 mins.ABG taken from right radial artery. - CHD : po2<100mmhg;rise in po2 is <20mmhg - in severe lung diseases &PPHN,po2 can be <100mmhg Echocardiography

cause

History/sign

cxr

ABG

Hyperoxia test

echo

Cyanotic heart disease

No/mild respiratory distress+heart murmur

Abnormal heart size/pulmonary

Low po2 Normal pco2

No rise in po2

Usually diagnostic

Primary lung disease

Respiratory distress

Abnormal lungs

Low po2 High pco2

Po2<100mmhg

normal

Persistent pulmonary hypertension

Suggestive history(MAS,asphyxia, Sepsis)

May be abnormal lungs

Right arm po2>than lower limbs

Can be inclonclusive

Right to left shunt acro PFO or PDA

Initial stabilization-secure airway,adequate ventilation,circulatory support Correct the metabolic acidosis,electrolyte,hypoglycaemia:prevent hypothermia Empirical IV antibiotics Early cardiology consultation IV prostaglandin E infusion if ductdepentdant lession suspected

Starting dose 10-40ng/kg/min;maintainance 2-10 ng/kg/min

Left to right shunt


Obstructive lessions

Atrial septal defect(ASD) Ventricular septal defect(VSD) Patent ductus arteriosus(PDA) Atrioventricular septal defects(AVSD)

Cyanotic heart lession


Pulmonary stenosis Coarctation of aorta(CoA) Tetralogy Of Fallot(TOF) Transposition Of Great Arteries(TGA) Pulmonary atresia with intact ventricular septum Truncus arteriosus TOF with pulmonary atresia Single ventricle Total anomalous pulmonary venous drainage

Atrial septal defect is a hole that occurs when the muscular wall (septum) separating the top two chambers of the heart (right and left atria) doesn't close properly. This allows oxygen-rich blood from the left atrium to flow into the right atrium, where it mixes with blood that doesn't have adequate oxygen in it. Blood from the left atrium should normally flow into the left ventricle and on to the aorta and the rest of the body.

Small defect- no treatment Large defect- elective closure 4-5 years age Contraindication for closure-established pulmonary vascular disease with reversal of shunt.

Sometimes called a hole in the heart, this defect the most common congenital heart defect occurs when the muscular wall (septum) separating the bottom chambers of the heart (right and left ventricles) doesn't fully form. The hole allows oxygen-rich blood to leak from the left ventricle into the right ventricle, instead of moving into the aorta and on to the body. In the right ventricle, the oxygen-rich blood mixes with blood that doesn't have enough oxygen in it

Small defect

Moderate defects

No treatment;high rate of spontaneous closure SBE prophylaxis Yearly follow up for aortic valve prolapse,regurgitation Surgical closure indicated if prolapsed aortice valve

Large defects

Anti failure therapy if heart failure Surgical closer if: heart failure not controlled by medical therapy,persistent cardiomegally on cxr,elevated pulmonary arterial pressure,aortic valve prolapse or regurgitation Early primary surgical closure Pulmonary artery banding followed by VSD closure in multiple VSDs

Before birth, a blood vessel called the ductusarteriosus connects the pulmonary artery the artery carrying blood to your lungs and the aorta, the large artery that carries blood away from the heart. Before a baby is born, the ductusarteriosus allows blood to bypass the lungs because the baby receives oxygen through the placenta and umbilical cord. The ductusarteriosus normally closes within soon after birth. If the ductusarteriosus remains open (patent), some blood that should flow through the body goes to the lungs

Small PDA
No treatment if there is no murmur If murmur present-elective closure as risk of endocarditis

Moderate to large PDA


Anti failure therapy if heart failure Timing,method of closure (surgical vs transcatheter)depends on symptom severity,size of PDA and body weight.

This is a combination of defects, including a large hole in the center of the heart and a single common valve instead of the separate tricuspid and mitral valves. Also called atrioventricularcanal defect, this defect is classified by whether it's only partial (involving only the upper chambers of the heart), or complete (in which blood can travel freely among all four chambers of the heart). Both forms of the defect allow extra blood to circulate to the lungs, causing the heart to enlarge

Partial AVSD(ASD primum)


Elective surgical repair at 4-5 years old;early if symptomatic or severe AV valve regurgitation

Complete AVSD
Primary surgical repair<6 months age to prevent pulmonary vascular disease. In selected patients-e.g with severe AV valve regurgitation and older patients,conservative treatment is an option as surgical outcomes are poor

In this condition, blood flow from one of the heart's bottom chambers, the right ventricle, to the pulmonary artery is slowed by narrowing at the pulmonary valve. When there's narrowing (stenosis), the right ventricle must pump harder to get blood into the artery that carries blood to the lungs (pulmonary artery). Pulmonary valve stenosis may occur along with other defects, such as thickening of the muscle of the right ventricle below the valve.

Mild(peak systolic gradient<50mmhg)


No treatment

Moderate-severe(gradient>50mmhg)
Transcatheter balloon valvuloplasty is treatment of choice

Neonatal critical PS
Characterized with cyanosis and RV dysfunction Temporary stabilization with IV prostaglandin E infusion Early transcatheter balloon valvuloplasty

This is a narrowing (coarctation) in a portion of the heart's main artery (aorta). Coarctation forces the heart to pump harder to get blood through the aorta and on to the rest of the body. Coarctation of the aorta can cause several lifethreatening complications, including severe high blood pressure, a bulge in the aorta that can burst (aortic aneurysm), infection of the heart (endocarditis), brain hemorrhage, stroke, heart failure and premature coronary artery disease.

Neonatal severe CoA


Frequently associated with large malaligned vsd and intractable heart failure Sick infants require temporary stabilization Early surgical repair

Asymptomatic/ older children with discrete CoA


Presents with incidental hypertension or heart murmur Choice of treatment(primary transcatheter balloon angioplasty,stent implantation,or surgical repair)

This defect is a combination of four (tetralogy) heart defects. The four defects typically are ventricular septal defect (VSD), pulmonary valve stenosis, a misplaced aorta and a thickened right ventricular wall (right ventricular hypertrophy). They usually result in an insufficient amount of oxygenated blood reaching the body. Complications of tetralogy of Fallot include cyanosis sometimes called "blue baby syndrome," since the lips, fingers and toes may have a bluish tinge from lack of oxygen as well as poor eating, physical inability to exercise, irregular heartbeats (arrhythmias), delayed growth and development, and stroke

Most TOFs suitable for single repair at 1 to 2 years age. Indications for modified Blalock Taussig shunt
Hypercyanotic spells or severe cyanosis<6 months age when child is too young for total repair Small pulmonary arteries Anomalous coronary artery crossing in front of right ventricular outflow tract

Following surgical repair,patients need life-long follow up for late right ventricular dysfunction; some may require pulmonary valve replacement

With this defect, the positions of the aorta the main artery leading away from the heart and the pulmonary artery, which leads to the lungs, are reversed (transposed). The aorta and pulmonary artery are, together, sometimes referred to as the great arteries. In transposition of the great arteries, the aorta arises from the right ventricle instead of the left ventricle and the pulmonary artery arises from the left ventricle instead of the right. This prevents nourishing oxygenated blood from reaching the body.

Simple TGA

TGA with VSD

IV prostaglandin E infussion promotes intercirculatory mixing at PDA Early balloon atrial septostomy(BAS) if restrictive interatrial communication Surgical repair of choice:atrial switch operation at 2 to 4 weeks age Left ventricular regression may occur if repair not performed within 4 weeks of life Do not usually require intervention durin early neonatal period Elective one-stage arterial switch operation +VSD clossure before 3 months age

TGA with VSD and PS

Blalock Taussig shunt during infancy followed by Rastelli repair at 4 to 6 years age.

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital cardiac lesion characterized by heterogeneous right ventricular development, an imperforate pulmonary valve, and possible extensive ventriculocoronary connections. Prognosis and management depend on the degree of right ventricular hypoplasia (including tricuspid valve hypoplasia) and the dependency of the myocardial blood supply on abnormal communications between the right ventricle and coronary arteries. These 2 factors are the most important prognostic determinants.

IV prostaglandin E infusion to maintain ductal patency in early neonatal period Further management strategy depends on the degree of right ventricular hypoplasia

This is a defect in which the normally separate pulmonary artery and aorta merge into one single large vessel (truncus) arising from the two bottom chambers of the heart (right and left ventricles). Many people who have this defect also have a large ventricular septal defect, which turns the right and left ventricles into a single chamber. This allows red oxygenated blood and blue unoxygenated blood to mix. Too much blood may flow to the lungs, flooding them and making it difficult to breathe. It can also result in life-threatening pulmonary hypertension high blood pressure in the lungs

Surgical repair (VSD closure and RV to PA conduit) before 3 months of age

IV prostaglandin E infusion is often required during early neonatal period Further management strategy depends on the anatomy of the pulmonary arteries and presence of aortapulmonary collaterals

Total anomalous pulmonary venous connection (TAPVC) consists of an abnormality of blood flow in which all 4 pulmonary veins drain into systemic veins or the right atrium with or without pulmonary venous obstruction. Systemic and pulmonary venous blood mix in the right atrium. An arterial defect or foramen ovale(part of the complex) is important in left ventricular output both in fetal and in newborn circulation

4 major anatomic types : supracardiac ,cardiac, infracardiac, and mixed Management strategy depends on presence of pulmonary venous obstruction
Presents with respiratory distress and heart failure Initial stabilization : oxygen,diuretics,positive pressure ventilation Surgical repair immediately after initial stabilization Early surgical repair required

Obstructed pulmonary venous drainage (frequent in infracardiac type)

Unobstructed pulmonary venous drainage