CASE SUMMARY
A 14 years of age boy presented to the cardiologist with complaints of
dyspnea and angina on exertion and palpitations for the past one year.
Clinically, there was no cyanosis. Chest X-Ray showed cardiomegaly with
enlargement of left-side chambers of the heart and a prominent main
pulmonary artery(MPA) impression. His resting ECG was essentially normal
with no ST-T changes. On echocardiography(Figure-1), there was an
enlarged Left atrium, Left ventricle and MPA with severe pulmonary
artery hypertension(PAH). There was a large type-I APW of 24mm.
CT-Angiography(Figure-2b) was done which confirmed the above findings.
On the catheterization study(Figure-2a), pulmonary artery pressures were
at systemic levels but reversible with 100% Oxygen. MPA was dilated(3.5
cm) and was seen compressing the left main coronary artery(LMCA). The
closure was attempted with a 24mm VSD device and 34mm ASD
device(Amplatzer St.Jude Medical, USA) but was unsuccessful despite
multiple attempts. Also, there were transient ST-T changes while
deploying the device, hence the procedure was abandoned and was referred
for surgical closure of APW. Under normothermic cardiopulmonary bypass,
aortic root cardioplegia was given after snaring both pulmonary arteries
with a LV vent in-situ. A trans-window vertical incision was used for
opening the defect(Figure-3a, Supplementary-Video-1). The right
pulmonary artery(RPA) (Figure-3b) and coronary ostia(Figure-3c) were
away from the defect. Polytetrafluoroethylene(PTFE) patch was used to
close the window with the anterior sandwich technique. The LMCA was seen
arising eccentrically to the left and a little higher than usual but the
caliber was found to be adequate on putting a Garrett probe(Scanlan
Memory Garett). Post cardiopulmonary bypass, PA was found to be soft,
less distensible and PA pressures were 50% of the systemic pressures.
The postoperative course was uneventful and the patient was discharged
on day 5.