1) A 1-month-old female baby presented with a cough for 1 week. On examination, a heart murmur was detected, which prompted an echocardiogram. 2) Atrioventricular septal defects (AVSDs) can be partial, complete, transitional, or intermediate based on the connections between the atrial and ventricular septa. 3) Cardiac catheterization may be needed to assess for pulmonary hypertension and can show characteristic features like the "gooseneck malformation".

1. ATRIOVENTRICULAR SEPTAL DEFECT

2.  1 month female baby delivered by LSCS
History of cough for x 1week
No cyanosis, tachypnea
For which her parents took her to pediatrician , who notice murmur and referred for ECHO

6. DISCUSSION

12. CLASSIFICATION OF AVSD
Partial: When 2 bridging leaflets are joined each other by a tongue of tissue dividing AV valve into two separate
orifices. Thus, partial AVSD has primum defect , 2 distinct mitral and tricuspid AV annuli and cleft mitral
leaflet is present invariably.
Complete: When bridging leaflets are free , guarding the opening of both the atria to the respective ventricle as a
common opening .Thus in complete AVSD primum ASD is contiguous with VSD and common AV valve
has single annulus
Transitional : Subtype of partial AVSD .Additional finding is a small inlet VSD that is partially occluded by dense
chordal attachment to the septum
Intermediate: Subtype of complete AVSD that has distinct right and left AV valve orifice despite having one common
annulus

29. CARDIAC CATHETERIZATION AND ANGIOGRAPHY
 Indicated when there is strong ECHO based evidence of severe PTHN.
 Catheter usually course low in the septum, has typical curve on fluoroscopy and may enter LV
 Best angiographic feature is the gooseneck malformation.Body of goose is produced by abnormal parietal
attachment of the left component of the common Av junction.

33.  Patient of partial AVSD and minimal AV Regurgitation do well. AF is important important cause of late morbidity
and mortality.

34. MANAGEMENT OF AVSD
 All requires surgery except rare patients with small septal defect and complete AV valves.
 Medical therapy Digoxin and diuretics to stabilize the patients with large shunt and cardiac failure in early
infancy
 Feeding by gastric tube sometimes necessary to provide adequate Caloric intake.
 Aim of medical therapy is to postpone surgery in symptomatic infants (preferably until 6months of age,<5kg).
 Young infants very high pulmonary vascular resistance , oxygen is occasionally given continuously during the
last weeks prior to surgery to reduce the incidence of postoperative pulmonary hypertensive crises.

35. ASSESSMENT OF OPERABILITY
INOPERABLE CASES
 CLINICAL EXAMINATION: Complete AVSD with normal venous drainage and with cyanosis and clubbing in the
absence of Right ventricular outflow track obstruction.
 ECHO: Dilated PAs, dominant RV, compressed appearing LV and on Chest X ray absence of plethora.
 Cardiac Cath: Raised and fixed PVR (>8 wood units /m
2
) with little and no fall after 100% O2 administration.
 Angiogram in LAO .If the dye appears earlier in pulmonary artery and density is more than aorta, it indicates
significant L TO R shunt (operability)