Congenital Flashcards
Relation of Normal PA to Aorta
ALS- Anterior,left, Superior; PALS - pulmonary artery is ALS
Mitral pulmonary continuity is seen in
TGA
—- has double conus
DORV
Ostium secundum is formed in septum
Primum;
Ostium primum is at lower end of Septum primum
Limbus is formed by Septum—
Secundum.
So will be on RA side
Floor of fossa ovalis is formed by
Septum Primum
Remnant of foremen ovale on the right side is called Fossa ovalis
In coarctation resting arm leg gradient >______is considered if more than
20 mm Hg at rest
After exercise > 40 mm Hg is abnormal
What percentage of cases both subclavians arise after coarctation
3-4%
So BP in all limbs will be equal
Rib notching I coarctation
Notching of posterior 1/3 of 3-8th ribs
Indication for coarctation intervention in children is
peak INSTANTANEOUS gradient more than
20 mm Hg
Role of balloon angioplasty in coarctation
- Balloon angioplasty is an alternative to surgical repair for “older infants & young children”(greater than four months) with native discrete coarctation
- Stent placement has replaced balloon angioplasty as the procedure of choice in “older children and adults “with native coarctation.
- It remains the preferred intervention for all patients with isolated recoarctation regardless of age
Stent placement is not preferred in coarctation if body weight is
Less than 25
The features of REcoarctation absent in children
Hypertension and Headaches are usually absent
Indications for intervention in re coarctation
- Hypertension
- Instantaneous gradient more than 20
- Presence of collaterals
Balloon angioplasty vs Surgery in coarctation risks
Aneurysm and re coarctation more with balloon
Indications for Pulmonary valve replacement
RF>25% measured by CMR
and 2 or more of
RV volume criteria(3 in no); EF, RVOT aneurysm
or Clinical criteria of exercise intolerance,HF,Syncope,sustained VT
ASD may be closed if PVR is
Less than 5 Woods unit
Others the efficacy is uncertain
If Pulmonary artery pressure >2/3 of Systemic pressure should not do
Largest TV leaflet in Ebsteins is
ATL
Called ‘SAIL LIKE’
Indications for surgery in Ebsteins
Cyanosis- spo2-<90%
RV failure….(to be refined)
Deteriorating exercise capacity
Embolism
REC- RV failure, Embolism, Exercise, Cyanosis
Precautions with Venous P valve for PR
Upsize by 3-4 mm
Check for LMCA occlusion with balloon occlusion of RVOT -RAO caudal and lateral views
Deploy proximal part of stent fast to avoid hypotension
Murmur of moderate VSD is evident………..days of birth
Within 2-3 days
Katz Wachtel phenomenon is
Large biphasic QRS in V2-4.
QRS 50 mm or more
Due to biventricular hypertrophy
Least common type of VSD
Supracristal or Doubly committed
AR common
Crista Supraventricularis can be considered synonymous with
Infundibular or Conus Ventricular Septum
It’s the portion of septum seperating Tricuspid and Pulmonary valves
Supracristal VSD is in this part. Usually the term is reserved for Defects lying immediately below the Pulmonary Valve. Defect in conus septum is also technically supracristal
Supracristal septum supports the…..
Muscular support to Aortic Valve esp RCC
Unlike the ……VSD the supracristal VSD does not lie near……..
Peri membranous
Tricuspid Valve
VSD closure in children if PAP more than
50% of Systemic arterial pressure
Double chambered RV is
DCRV- A form of septated RV with a proximal and distal chamber ( inlet /outlet)due to hypertrophied or abnormally located muscular bands
A/w VSD, PS, Subaortic Sinus etc
Should we close small VSDs
Only if history of IE, or Valve prolapse and AR
Normal course of LCA in relation to Pulmonary Artery
Passes behind the MPA
…….color in LCA in PSAX is abnormal and s/o ALCAPA
BLUE
Classically ALCAPA presents at
2-6 wks of age
Prominent RCA in a child suspect
ALCAPA
Incidence of sudden death in adult type ALCAPA
80-90% sudden death at a mean age of 35 yrs in adult type( better collateral circulation)
In ALCAPA ……is often misdiagnosed as LCA
Transverse Sinus of Pericardium
Echogenic papillary muscles in a child may suggest
ALCAPA
RSOV most commonly arises from
RCC
Reason for LVH +LAD in ALCAPA in older children
Due to hypertrophy of basal posterior wall compensating for the scarred and thinned anterolateral wall
Incidence of CHD excluding MVP and Bicuspid Aortic Valve
0.5-0.8%
BAV-1-2%
MVP-4-6%
Most common CHD is
VSD
25% of CHD
Sizing of VSD
In relation to Aortic annulus
<25%- Small
>50% - Large
In between- moderate sized
Last portion of Ventricular septum to close during development
Peri membranous septum
ie why Perimembranous VSD is most common
Other names of Outflow VSD
Supracristal
Subpulmonic
Doubly committed subarterial
Gasuls VSD develops in………,,type of VSDs
Peri membranous VSDs
Difference in diastolic flow in a VSD versus RSOV
VSD diastolic flow peaks in late diastole
Most common congenital cardiac anomaly
BAV
Upto 2%
Other cardiac anomalies a/w BAV
COARCTATION
AORTOPATHY
Asd/Vsd
Life expectancy in BAV
Same as general population
Risk factors for rapid progression of Aortic dilatation in BAV
Hypertension
Male sex
Old age
Valve disease
Most common type of BAV is
Anterior- Posterior (AP) type - fusion of RCC +LCC
Second type is Right-Left type (RL)- fusion of RCC+NCC
RL Type is more associated with Aortopathy
Echo probe position for Proximal Ascending Aorta
One intercostal space above the PLAX
In BAV Aortopathy Most centers recommend intervention when Aortic size is >
50-55’mm independent of Valve disease
Can be done at 45-50 if
- Undergoing Aortic valve surgery or
- Growth > 5mm in 6 months
2013 JACC imaging
Spontaneous closure of ASD happens
Defects less than 8 mm in younger patients around 90% close
Uptodate 2018
Phlebotomy in Eisenmenger only if Hb and PCV is
> 20 or > 65
Hemoptysis is cause of death in ….% of Eisenmenger patients
11-29%
Circulation 2007 Jun 19
