Chd Introduction And Approach 1

Question 1

Chd common in boys and girls ?

Answer 1

Boys-tga and left sided obstructive lesions like aortic stenosis and coarctation of aorta 65%…
Girls-vsd,asd,pda,ps…

Question 2

Common chd in girls ?

Answer 2

Vap mnemonic
Vsd,asd,pda,ps

Question 3

Maternal conditions associated with chd?

Answer 3

2-4% chd Assosciated with maternal conditions or teratogen influences…
Dps-delhi public school
Maternal diabetes Mellitus,phenylketonuria,sle,torch infections…
Mother takes anticonvulsant,vitamin a derivatives,lithium,ethanol,warfarin,thalidomide

Question 4

Peripheral pulmonary stenosis is seen in which syndromes?
Mnemonic is wan

Answer 4

Williams syndrome-elastin gene-supra valvular aortic stenosis-elfin facies,hypercalcemia
Alagille syndrome-jagged 1gene-tof-bile duct hypoplasia
Noonan syndrome-ptpn 11gene-short stature,dysmorphic facies-asd,pda,hcm

Question 5

Short stature with chd conditions are ?

Answer 5

Noonan syndrome-ptpn11gene-pulmonary stenosis ,pda,asd,hcm-dysmorphic facies
Turner syndrome-44x0-coa,aortic stenosis -renal anomalies,puberty failure

Question 6

Conditions Assosciated with asd?

Answer 6

Familial asd with heart block-nkx2.5 gene -asd,heart block
Familial asd with out heart block-gata4 gene -asd
Holt oram syndrome -asd-tbx 5 gene ,limb defects

Question 7

DiGeorge syndrome also known as shprintzen syndrome (velocardiofacial syndrome )
Deletion-

Answer 7

22q11.2 deletion
Prevalence-1 in 4000 live births
Tbx1 gene is a transcription factor involved in outflow tract development
Conotruncal defects-tof,truncus arteriosus,dorv
Branchial arch defects-coa,interrupted aortic arch,right arch
Catch 22-cardiac defects,abnormal facies,thymic aplasia,cleft palate,hypocalcemia…
Other features-hypertelorism,bulbous nose,protrusion of frontal eminence

Question 8

Catch 22 full form ?

Answer 8

Cardiac defects , abnormal facies, thymic aplasia , cleft palate , hypocalcemia

Question 9

If one child is affected with
Asd, vsd-recurrence risk in next child is ?
C tga-

Answer 9

Asd, vsd-3to 4%
Ctga-5.8%

Question 10

If mother has
Asd-
Vsd-
Coa-
What is recurrence risk in child ?

Answer 10

Asd-4 to 14%
Vsd-6 to 17%
Coa-12 to 20%

Question 11

Relationship between Hemoglobin concentration and cyanosis ?
Why in polycythemic patients develop cyanosis at high spo2 level?
Why anemic patients develop cyanosis at low spo2 levels ?

Answer 11

More hemoglobin → More deoxygenated hemoglobin at the same oxygen saturation → Cyanosis appears at a higher oxygen saturation.

Let’s compare a normal person vs. a polycythemic person at the same oxygen saturation.

Example: Oxygen Saturation = 75%

This means that 25% of hemoglobin is deoxygenated.

1. Normal Hemoglobin (15 g/dL)• Deoxygenated Hb =

15 \times 0.25 = 3.75 \text{ g/dL}

•	This is below the 5 g/dL cyanosis threshold, so no cyanosis yet.

2. Polycythemia (20 g/dL)• Deoxygenated Hb =

20 \times 0.25 = 5 \text{ g/dL}

•	This reaches the cyanosis threshold → Cyanosis appears.

Key Insight

•	Even though both individuals have the same oxygen saturation (75%), the person with polycythemia has more total hemoglobin.
•	Since more total hemoglobin is present, more absolute deoxygenated hemoglobin accumulates, crossing the 5 g/dL threshold earlier than in a person with normal Hb.

Question 12

Central cyanosis definition?
Causes cyanotic chd?

Answer 12

Bluish discolouration of nails ,skin and mucous membrane due to intra cardiac shunting of blood from right to left side of heart.
Causes:- 5t’s they are
Tricuspid atresia
Tga,truncus arteriosus
Tof,tapvc,single ventricle

Question 13

Central cyanosis with heart murmur with decreased pulmonary blood flow ?
Examples?
Presentation?

Answer 13

Tricuspid atresia,tof,tga,ctga,dorv,avsd ,single ventricle along with pulmonary stenosis and vsd in all causes

Presentation :- due to decreased pulmonary blood flow so deoxygenated blood goes to aorta so prominent cyanosis present,silent tachypnea due to low o2,cyanotic spells if child is >2 to 3 months of age…

Question 14

Cyanotic chd with increased pulmonary blood flow causes?
Presentation?

Answer 14

Causes:- tga with vsd ,dorv with vsd,tricuspid atresia with vsd

Presentation:-congestive heart failure ,relatively mild cyanosis,presents at 2 to 6 weeks after birth.

Question 15

Duct dependent pulmonary blood flow ,duct dependant systemic blood flow and duct dependent mixing ….tell the examples for dat ?

Answer 15

Duct dependent blood flow-critical pulmonary stenosis
Presentation-severe cyanosis ,silent tachypnea ,adequate perfusion initially …after prostaglandin given—>spo2 increases…

Duct dependant systemic blood systemic blood flow-critical aortic stenosis and hlhs…
Presentation-cardiac failure,low cardiac output,shock and poor perfusion,oliguria,abdominal distension—->after prostaglandin given oliguria subsides…

Duct dependant systemic blood mixing—-tga with intact ivs,truncus arteriosus,unobstructed tapvc…
In tga with intact ivs—mixing occurs at asd level or ductus arteriosus level—-so after ductus closure baby develops cyanosis ,silent tachypnea with out respiratory distress…