16) Congenital heart diseases -ASD, VSD

Question 1

what are the clinical features of atrial septal defect ?

Answer 1

asymptomatic up to 30 years

frequent respiratory infections in children

failure to thrive

complaints associates with pulmonary hypertension
right heart failure

Question 2

what are the physical findings in those individuals with atrial septal defect ?

Answer 2

Widely fixed split OF s2 second heart sound (S2) over the second left ICS,- pulmonary zone

systolic ejection murmur in pulmonary auscultary zone (2 intercostal space left parasternal ) - some pulmonary valve stenosis

Low-pitched mid diastolic murmur in tricuspid auscultatory zone - due to increased flow though the tricuspid valve

Right-ventricular S3 gallop

Question 3

what are the diagnostic findings in atrial septal defect ?

Answer 3

ECHO - dilated right atria and ventricle
paradoxiacl septal movemnet in hemodynamically significant

doppler view
or we can use contrast echo - to see the contrast bubles being shunted left to right

Trasn esophageal echo - we can see a direct visualisation

ecg - in ostium secindum - we see right axis deviation
with incomlete or complete rbbb

in ostium primum - we see left axis deviation
with complete or incomlete RBBB

chest x ray - we see dilated pulmonary arteries
cardiomegaly

Question 4

what is eisenmenger syndrome ?

Answer 4

long-standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus)

causes pulmonary hypertension

and eventual reversal of the shunt into a cyanotic right-to-left shunt.

Question 5

INVASIVE ASSESSMENT OF THE INTERATRIAL SEPTAL DEFECT

Answer 5

cardiac catheterisation :
oxohemometry
jump in SaО2 > 7 % in LA

SaО2 in RV and PA > 80 %

Manometry -ELEVATED pressures in
PA,-92mmhg
RV,-92mmhg
RA - 90 mmhg

Quantitative assessment:
• PBF : SBF < 1.5 (in low
pressures) hemodynamically insignificant LR shunting
• PBF : SBF > 1.5 hemodynamically significant LR shunting
• PBF : SBF > 2.0 absolute indication for correction

PBF more than SBF =it means pulmonary hypervolemia

Question 6

what is SBF ?

Answer 6

О2 consumption /

О2 (Ао) – О2 (mixed venous blood)

Question 7

what is bpf ?

Answer 7

О2 consumption

О2 (pulmonary vein) – О2 (pulmonary artery)

Question 8

what are associated with atrial septal defect secundum ?

Answer 8

5-10% in combination with pulmonary stenosis

• 10% combination of anomalous inflow of pulmonary veins (mainly in sinus venosus type)
• 2-8% in combination with mitral stenosis (Lutembacher’s syndrome)

Question 9

what are the treatment method for atrial septal defect ?

Answer 9

in children - spontaneous closure

Hemodynamically insignificant defect – Conservative, symptomatic

Hemodynamically significant defect

Interventional methods
Use of CardioSEAL, Amplatzer (including closure of patent foramen ovale, a common sourse of paradoxical embolism which is the cause of strokes in young people).

– Surgical closure

Eisenmenger’s syndrome – heart lung transplant

Question 10

what are the complication of all types of atrial septal defects ?

Answer 10

Paradoxical embolism
venous thromboembolus passes through a shunt from the inferior vena cava entering into the arterial circulation

and stroke and infraction

Question 11

what are the causes for all types of atrial septal defect ?

Answer 11

Down syndrome

Fetal alcohol syndrome

Question 12

what are the associations with ASD1 (PRIMUM TYPE)

Answer 12

Equally prevalent in men and women
in secundum it’s majorly more women

usually isolated from other heart defects

Relatively earlier development of pulmonary hypertension and cardiomegaly

Mitral regurgitation often present

Often supraventricular arrhythmias

Often AV-block

Question 13

what is the evolution of atrial septal defect

Answer 13

volume overload and dilation of the right atrium and ventricle

tricuspid an pulmonary annuli may dilate and becomeincompettent

pulmonary arteries dilate - so does pulmonary vein

flow related pulmonary artery hypertension

medial hypertrophy of pulmonary arteries and muscularisation of arterioles - pulmonary vascular obstructive disease

reversion shunt- Eisenmenger syndrome

Question 14

what are other ASD?

Answer 14

COMMON (SINGLE) ATRIUM
• Complete lack of interatrial septum
• Clinical features similar to those in large interatrial septum defect

COMMON AV-CANAL
• Distally positioned ASD1 -partial 
• Proximally situated VSD - complete
• Fissure of anterior mitral leaflet
• Fissure of septal tricuspid leaflet

Question 15

symptoms of COMMON AV-CANAL

Answer 15

Large L-to-R shunt at atrial and ventricular level

pulmonary hypertension

Symptoms include difficulty breathing (dyspnoea) and bluish discoloration on skin and lips (cyanosis). A newborn baby will show signs of heart failure such as edema, fatigue, wheezing, sweating and irregular heartbeat.

Question 16

diagnosis of COMMON AV-CANAL

Answer 16

ЕCG
– biatrial hypertrophy
right atrium enlargmnet - p pulmonate
lead 2 - with amplitude of 2,5mm, and v1 - 1.5mm

p - mitrale
lead 2 - we see notched p wave - 40ms between each peak
and the whole p wave duration is more than 120ms

in v1 terminal portion of biphasic P wave = 1mm deep and 40ms wide

• biventrcular hypertrophy
  katz watchel phenomena
  Large biphasic QRS complexes (tall R waves + deep S waves) in V2-5

– Left axis deviation

– Various degrees of AV-block

• Echo in different modalities – direct
visualization of the defect

• Cardiac catheterization – definitive lesions evaluation and assessing the hemodynamic changes

Question 17

what are the physical findings of ventricular septal defect ?

Answer 17

palpation
widened and uplifting apical heart beat
thrill at the fourth left intercostal space
palpable p2

auscultation 
holosystolic murmurs - heard best in 3rd and 4 th intercostal space 
radial propagation 
and starts immediately after S1 
band shaped 
crosses A2 

mid diastolic murmur over cardiac apex (mitral auscutatroy zone - 5th intercostal space mid clavicular ) - increase flow through the mitral valve

pathologicaly split S2

pathological left ventricular S3 -increased atrial pressure leading to increased flow rates, as seen in congestive heart failure, which is the most common cause of a S3

Question 18

WHAT ARE THE SIGNS FOR HEMODYNAMICALLY Significant VSD ?

Answer 18

pulmonary arterial hypertension

ECG - RA and RV hypertrophy

or ECG - volume overloadd LV hypertrophy

Question 19

in cardiac catheterisation one of the diagnostic invasive assessment tools for intraventricular septal defect what do we find ?

Answer 19

elevated pressure in the RV - 88
PA - 87
LA - 93

jump in sa02 by more than 5 percent in rv

SaO2 - in RV and PA more than 80 percent

PBF over SBF = pulmonary hypervolemia

Question 20

what are the diagnostic modalities for ventricular septal defect ?

Answer 20

echo
pulse doppler

ecg
medium and large size effects

LV hypertrophy : increase in amplitude of qrs , left axis deviation and left atrial enlargnet sign - p mitral

signs of rv hypertrophy - vright axis deviation , p pulmonate , PR prolongation , complete or incomplete right bundle branch block

chest x ray enhanced pulmonary vasculature
left atrial and ventricular enlargements
later stages - enlarged right ventricle and pulmonary artery

Question 21

what are the clinical features of VSD ?

Answer 21

small asymptomatic

medium to large
heart failure in 2-3 months

Question 22

what are the causes of VSD

Answer 22

down syndrome , edward , pat au
TORCH
maternal risk fctors- diabetes and smoking

Question 23

what is the treatment of VSD

Answer 23

symptomatic and large defects

surgical repair
in children less than 1 yr indicating pulmonary hypertension

in older children
Special modifications of dual- sided occluders – CardioSEAL, STARFlex and Amplatzer (often not suitable for the more frequent perimembranous VSD)

if eisenmenger syndrome has occurred - heart lung transplant or lung heart transplant

Question 24

what are the complications of VSD ?

Answer 24

arrhythmia
heart failure
eisenmenger syndrome

Question 25

what are the causes for patent ductus arteriosus ?

Answer 25

prematurity
rubella infections during first trim
alcohol consumption whilst pregnant
Down sydruome

Question 26

what is the patent ductus arteries do hemodynamically ?

Answer 26

persistent communication between the aorta to the pulmonary artery
effectively making a left to right shunt - this causes volume overload on pulmonary vessels - strain on right ventricle - leading to heart failure

Question 27

what are the clinical features with patent ductus arteriosus ?

Answer 27

small

large - failure to thrive
heart failure symptoms

Question 28

what are the physical findings for patent ductus arteriosus ?

Answer 28

palpation
laterally displaced apical pulse
BOUNDING PERIPHERAL PULSE - wide pulse pressure

AUSCULTATION
machien like murmur of systolic and diastolic period

onset is after s1 and passes over s2 and end before the next S1
heard best at the 2-3 left intercostal space parasternally
propagation towards left subclavian space , left axilla and left paravertebrla space

ECHO

ANGIOGRAPHY
in right catheterisation -passes through pulmonary artery to aorta - typical shape of TREBLE CLEF
direct visualisation if injected with contrast dye

invasive assessment through cardiac catheterisation :
slightly increase pa pressure
leap in sa02 of more than 5 percent in PA
sao2 in pa more than 80 percent
bff is more than SBF

chest x ray
prominent pulmonary artery

Question 29

what are the complications of PDA?

Answer 29

frequent lung infections

infective endocarditis

left and right ventricle failure

Question 30

what is the treatment for PDA?

Answer 30

= pharmacological closure in premature infants with infusion of indomethacin and ibuprofen

small ductus
diameter less than 2.5mm
occlusion with special wire coil through a catheter - gianturco coils

large ducts
diameter more tha 2.5mm
amplatzer or cardioseal system