Congenital Abnormalities Of The Heart

Question 1

What are congenital heart abnormalities?

Answer 1

Structural abnormalities of the heart present at birth, which may be hereditary or due to influences during pregnancy.

Question 2

What are the two main causes of congenital heart abnormalities?

Answer 2

Altered embryonic development of a normal structure or failure of a structure to progress beyond an early stage of development.

Question 3

What is the most common congenital heart defect?

Answer 3

Ventricular septal defect (VSD).

Question 4

What is the prevalence of congenital heart defects in live births?

Answer 4

Approximately 0.9% of live births are complicated by cardiovascular malformations.

Question 5

Which congenital heart conditions are more common in females?

Answer 5

Patent ductus arteriosus (PDA), Ebstein’s anomaly, and atrial septal defect (ASD).

Question 6

Which congenital heart conditions are more common in males?

Answer 6

Aortic valve stenosis, coarctation of the aorta, hypoplastic left heart syndrome, pulmonary & tricuspid atresia, and transposition of the great arteries (TGA).

Question 7

What maternal infections are associated with congenital heart defects?

Answer 7

Maternal rubella infection is associated with ASD, VSD, PDA, TOF, and pulmonary stenosis.

Question 8

What genetic condition is associated with atrioventricular septal defects?

Answer 8

Down syndrome (Trisomy 21).

Question 9

What are cyanotic congenital heart lesions?

Answer 9

Congenital heart defects that cause reduced oxygen levels in the blood, leading to cyanosis (e.g., TOF, TGA, pulmonary atresia).

Question 10

What are acyanotic congenital heart lesions?

Answer 10

Congenital heart defects that do not initially cause cyanosis, such as ASD, VSD, and PDA.

Question 11

What is the most common acyanotic heart defect?

Answer 11

Ventricular septal defect (VSD).

Question 12

What is the most common cyanotic heart defect?

Answer 12

Tetralogy of Fallot (TOF).

Question 13

What are the four components of Tetralogy of Fallot?

Answer 13

Overriding aorta, VSD, right ventricular outflow obstruction (pulmonary stenosis), and right ventricular hypertrophy.

Question 14

What congenital heart defect is associated with Turner’s syndrome?

Answer 14

Coarctation of the aorta.

Question 15

What is the classic murmur heard in patent ductus arteriosus (PDA)?

Answer 15

A continuous ‘machinery’ murmur at the upper left sternal border.

Question 16

What condition is associated with a right bundle branch block (RBBB) and a murmur?

Answer 16

Atrial septal defect (ASD).

Question 17

What is the hallmark physical exam finding in coarctation of the aorta?

Answer 17

Radio-femoral delay (weak femoral pulse compared to radial pulse).

Question 18

What is Eisenmenger syndrome?

Answer 18

A condition where a left-to-right shunt reverses to a right-to-left shunt due to pulmonary hypertension.

Question 19

What congenital defect is commonly associated with paradoxical embolism?

Answer 19

Patent foramen ovale (PFO).

Question 20

What is the treatment of choice for large symptomatic VSDs?

Answer 20

Surgical closure with a patch.

Question 21

What are the indications for ASD closure?

Answer 21

Right-sided heart dilation, significant ASD (>5mm), or Qp/Qs >1.5.

Question 22

Which genetic syndrome is associated with pulmonary stenosis and ASD?

Answer 22

Noonan syndrome.

Question 23

What congenital defect is associated with Marfan syndrome?

Answer 23

Aortic root dilation and mitral valve prolapse.

Question 24

What is the gold standard for diagnosing congenital heart defects?

Answer 24

Echocardiography (TTE or TEE).

Question 25

What is the purpose of the Rashkind procedure in TGA?

Answer 25

To create an atrial septal defect for better oxygen mixing.

Question 26

What is the characteristic ECG finding in Ebstein’s anomaly?

Answer 26

Right atrial enlargement and a tall P wave.

Question 27

What is the primary cause of cyanosis in Tetralogy of Fallot?

Answer 27

Right-to-left shunting through the VSD due to severe pulmonary stenosis.

Question 28

What congenital heart defect is commonly seen in DiGeorge syndrome?

Answer 28

Truncus arteriosus.

Question 29

What is the primary mechanism of PDA closure after birth?

Answer 29

A decrease in prostaglandin E2 levels and increased oxygen tension.

Question 30

What medication is used to keep a PDA open in neonates with duct-dependent lesions?

Answer 30

Prostaglandin E1 infusion.

Question 31

What syndrome is associated with supravalvular aortic stenosis?

Answer 31

Williams syndrome.

Question 32

What heart defect is associated with Holt-Oram syndrome?

Answer 32

Secundum atrial septal defect.

Question 33

What congenital defect is a major risk factor for infective endocarditis?

Answer 33

Ventricular septal defect (VSD).

Question 34

What is the most common location of a VSD?

Answer 34

Perimembranous septum.

Question 35

What is the preferred non-surgical intervention for PDA closure?

Answer 35

Percutaneous device closure with a coil or occluder.

Question 36

What is the preferred treatment for critical aortic coarctation?

Answer 36

Surgical resection with end-to-end anastomosis.

Question 37

What is the first-line imaging study for suspected congenital heart disease in adults?

Answer 37

Transthoracic echocardiography (TTE).

Question 38

What congenital heart defect is associated with paradoxical embolism and stroke?

Answer 38

Patent foramen ovale (PFO).

Question 39

What are the long-term complications of untreated congenital heart defects?

Answer 39

Pulmonary hypertension, heart failure, arrhythmias, and Eisenmenger syndrome.

Question 40

What is the survival rate of patients with untreated severe coarctation of the aorta?

Answer 40

50% die before age 30.

Question 41

What congenital heart defect has a strong association with Down syndrome?

Answer 41

Atrioventricular septal defect (AVSD).

Question 42

What congenital heart defect is associated with maternal diabetes?

Answer 42

Transposition of the great arteries (TGA).

Question 43

What is the role of MRI in congenital heart disease evaluation?

Answer 43

Assessing complex heart defects, shunt quantification, and vascular anomalies.

Question 44

What is the classic ECG finding in atrial septal defect (ASD)?

Answer 44

Right axis deviation and incomplete right bundle branch block.

Question 45

What is the classic auscultatory finding in an atrial septal defect?

Answer 45

Wide, fixed splitting of the second heart sound (S2).

Question 46

What congenital heart defect commonly requires a Fontan procedure?

Answer 46

Single ventricle physiology (e.g., tricuspid atresia).

Question 47

What is the primary consequence of an untreated large VSD?

Answer 47

Pulmonary hypertension leading to Eisenmenger syndrome.

Question 48

Which congenital defect has a continuous murmur and differential cyanosis?

Answer 48

Patent ductus arteriosus (PDA) with Eisenmenger syndrome.

Question 49

What syndrome is associated with truncus arteriosus and facial anomalies?

Answer 49

DiGeorge syndrome (22q11 deletion).

Question 50

What is the primary method of diagnosing a sinus venosus ASD?

Answer 50

Transesophageal echocardiography (TEE).

Question 51

What heart defect is associated with a boot-shaped heart on chest X-ray?

Answer 51

Tetralogy of Fallot (TOF).

Question 52

What congenital heart defect has a 'snowman' sign on chest X-ray?

Answer 52

Total anomalous pulmonary venous return (TAPVR).

Question 53

What is the primary treatment for transposition of the great arteries (TGA)?

Answer 53

Arterial switch operation.

Question 54

What congenital heart defect is characterized by severe tricuspid valve displacement?

Answer 54

Ebstein’s anomaly.