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- The Mission Hospital,Immon Kalyan Sarani, Sector IIC, Bidhannagar, Durgapur, West Bengal 713212
Common In CHD
VSD
- Ventricular septal defect is a hole in the wall that separates the right and left ventricles of the heart. Ventricular septal defect is one of the most common congenital (present from birth) heart defects. It occurs in nearly half of all children with congenital heart disease.
- The baby may have no symptoms and the hole can close over time as the wall continues to grow after birth.
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If the hole is large, too much blood will be pumped to the lungs. This can lead to heart failure – poor weight gain, recurrent respiratory infections, excessive sweating while feeds.
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If the hole is small, it may not be detected for years and only discovered in adulthood.
ASD
- Atrial septal defect is a hole between the two upper chambers of heart.
- 3 types of ASDs.
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Primum defects are usually associated with AV canal defect.
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Secundum defect are most common and mostly amenable for device closure.
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Sinus venosus defects are rare and usually associated with PAPVC.
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Secundum defects less than 3 mm usually closes by 3-6 monts.
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3-8mm defects: 80% closes by 2 years of age.
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More than 8 mm defects rarely closes.
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Ideal time for closure is after 2 years of age.
PDA
- This is the persistence of normal connection between the pulmonary artery and aorta from the fetal life till yhe newborn period and beyond.
- More common in premature babies.
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Needs closure if persists more than 3 months of age.
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More common in female babies.
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Large defects presents with heart failure symptoms and warrants closure.
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Usually closed by device closure.
AV CANAL DEFECT/ ECD
- Endocardial cushion defect (ECD) - The walls separating all four chambers of the heart are poorly formed or absent. Also, the valves separating the upper and lower chambers of the heart have defects during formation.
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Complete ECD - 70% are associated with Down’s Syndrome
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Needs early repair between 3-6 months.
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Partial ECD – always needs closure, usually by 2-4 years of age.
PAPVC
- Partial anomalous pulmonary venous connection (PAPVC)
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Mostly associated with ASDs, Scimitar syndrome.
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Repair done by 2-5 years of age
TOF
- Tetralogy of Fallot (TOF) is the most common cyanotic Congenital heart defect characterized by four specific cardiac defects:
- pulmonary stenosis which is narrowing of the exit from the right ventricle.
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a ventricular septal defect which is a hole allowing blood to flow between the two ventricles.
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right ventricular hypertrophy which is thickening of the right ventricular muscle
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an overriding aorta which is where the aorta expands to allow blood from both ventricles to enter.
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At birth, children may be asymptomatic or present with many severe symptoms. Later in infancy, there are typically episodes of bluish colour to the skin due to a lack of sufficient oxygenation, known as cyanosis.When affected babies cry or have a bowel movement, they may undergo a "tet spell" where they turn cyanotic, have difficulty breathing, become limp, and occasionally lose consciousness.Other symptoms may include a heart murmur, finger clubbing, and easy tiring upon breastfeeding.
TGA
- D-Transposition of the great arteries(D-TGA) is a cyanotic heart defect in which the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. This switch causes deoxygenated blood from the right heart to be pumped immediately through the aorta and circulated throughout the body and the heart itself, bypassing the lungs altogether. In this same condition, the left heart continuously pumps oxygenated blood back into the lungs through the pulmonary artery, instead of out into the body's circulation as it normally would. In effect, two separate "parallel" circulatory systems are created. It is a serious cyanotic congenital heart defect (CHD) because the newborn infant turns blue (cyanotic) from the lack of oxygen
- Needs urgent operation within a month.
TAPVC
- Total anomalous pulmonary venous connection(TAPVC) is a rare cyanotic congenital heart defect in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation (Normally, pulmonary veins return oxygenated blood from the lungs to the left atrium where it can then be pumped to the rest of the body). A patent foramen ovale/ patent ductus arteriosus/an atrial septal defect must be present, or else the condition is fatal due to a lack of systemic blood flow.
- In some cases, it can be detected prenatally.
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There are four variants:
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Supracardiac (50%): blood drains to one of the innominate veins (brachiocephalic veins) or the superior vena cava,
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Cardiac (20%), where blood drains into coronary sinus or directly into right atrium;
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Infradiaphragmatic (20%), where blood drains into portal or hepatic veins and a
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mixed (10%) variant
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Should be operated in neonatal age group
TRICUSPID ATRESIA
- Tricuspid atresia is a form of cyanotic congenital heart disease whereby there is a complete absence of the tricuspid valve.Therefore, there is an absence of right atrioventricular connection.This leads to a hypoplastic (undersized) or absent right ventricl. It causes the systemic circulation to be filled with relatively deoxygenated blood. The causes of tricuspid atresia are unknown.
- An atrial septal defect(ASD) must be present to fill the left atrium and the left ventricle with blood. Since there is a lack of a right ventricle, there must also be a way to pump blood into the pulmonary artery. This can be accomplished by a ventricular septal defect (VSD) connecting the left ventricle to the pulmonary artery or by a patent ductus arteriosus (PDA) connecting the aorta to the pulmonary artery.
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In the latter case, prostaglandin E1 is used to maintain the PDA connection until emergency corrective surgery can be completed. As oxygenated blood is mixed with deoxygenated blood in both cases, there is a reduction in the oxygen-carrying capacity.
TRUNCUS ARTERIOSUS
- Truncus arteriosus is a birth defect of the heart. It occurs when the blood vessel coming out of the heart in the developing baby fails to separate completely during development, leaving a connection between the aorta and pulmonary artery. There are several different types of truncus, depending on how the arteries remain connected. There is also usually a hole between the bottom two chambers of the heart (ventricles) called a VSD.
- In babies with a truncus arteriosus, oxygen-poor blood and oxygen-rich blood are mixed together as blood flows to the lungs and the rest of the body. As a result, too much blood goes to the lungs and the heart works harder to pump blood to the rest of the body
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Now-a-days many cases are amenable for transcatheter device closure
RSOV
- A sinus of valsalva aneurysm (SOVA) is an abnormal dilatation of the aortic root located between the aortic valve annulus and the sinotubular junction.
- It occurs as a consequence of weakness of the elastic lamina at the junction of the aortic media and the annulus fibrosis
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The estimated rate of Sinus of Valsalva aneurysm is approximately 0.09% of the general population.
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SOVAs comprise up to 3.5% of all congenital heart defects.
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Sinus of Valsalva aneurysms usually affect the right coronary sinus, followed by the noncoronary sinus, and finally the left coronary sinus.
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Males are four times more likely to be affected than females.
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There is a higher reported incidence in Asian groups
RSOV DEVICE CLOSURE
- Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly which has potential for spontaneous rupture into other cardiac chambers or the pericardial space
- It can either be repaired surgically or by device closure
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Now-a-days many cases are amenable for transcatheter device closure
PULMONARY AV FISTULA
- Despite most patients being asymptomatic, the connection between the venous and arterial system can lead to dyspnea (due to right-to-left shunting), as well as embolic events (due to paradoxical emboli). Although it is assumed that vascular defects are present at birth, they are seldom manifested clinically until adult life, when the vessels have been subjected to pressure over several decades. Clinically a murmur or bruit may be audible over the lesion (especially if peripheral). There is a highly variable age of presentation from infant to old age, although most are present within the first three decades of life.
- Most commonly closed in complex cyanotic congenital heart diseases before heart surgeries.
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Sometimes to treat and prevent haemoptysis, these collaterals are being closed.
AORTOPULMONARY WINDOW
- Babies with an aortopulmonary window have a hole in between the aorta and pulmonary artery.
- Because of this hole, blood from the aorta flows into the pulmonary artery, and as a result too much blood flows to the lungs. This causes high blood pressure in the lungs (a condition called pulmonary hypertension) and congestive heart failure.
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The bigger the defect, the more blood that is able to enter the pulmonary artery.
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Aortopulmonary window is very rare. It accounts for less than 1% of all congenital heart defects.
Most common syndromes associated with CHDs
- DiGeorge syndrome
- Down syndrome
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Marfan syndrome
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Noonan syndrome
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Edwards syndrome
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Trisomy 13
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Turner syndrome