My Nightmare Case Double Intervention in a Case of Atrial Septal Defect, Secundum Type, and Severe Pulmonary Stenosis

T, a five-year-old baby girl, weighing 13 kg, was diagnosed
as a case of Atrial Septal Defect (ASD) secundum type
with severe pulmonary valve stenosis.

History:

1. Failure to thrive
2. Cyanosis on crying.
3. Exertional dyspnoea.

Investigations:

1. ECG – Right bundle branch block, Right
ventricular hypertrophy, Right atrial hypertrophy.
2. CXR- Right Atrium enlarged, apex uplifted.
3. Colour Doppler Echo: Large ASD 2° size 14mm. Severe pulmonary valve stenosis. PPG 65 mmHg. Pulmonary valve annulus 10mm.

Management plan:

ASD device closure and pulmonary valvoplasty.

Procedure:

The patient was taken into the catheterization laboratory after proper pre-cath evaluation and investigation. Right
ventricular angiography showed thickened and domed pulmonary valve with an annulus of 10mm. Pulmonary valve
annulus was 10mm. Pulmonary balloon valvoplasty was done with 12mm 4cm that balloon. Then a super stiff wire was placed in the left upper pulmonary vein and ASD size was measured with 24mm amplatzer sizing balloon. Waist appeared in the balloon was 18mm. A decision was taken to use a 20mm amplatzer septal occluder to close the ASD. A delivery sheath was placed inside left atrium over the wire and dilator and wire removed. There was no free flow of blood through the sheath so the sheath was slightly withdrawn but there was still not free flow. Ultimately the sheath was withdrawn back to RA, flushed properly with saline and then repositioned again inside the LA with the help of wire and dilator. A device was loaded to the loader and forwarded through sheath towards LA. Immediately patient developed ST elevation in ECG, bradycardia, and hypotension. Delivery sheath with a device was rapidly pulled back to inferior vena cava. A patient was resuscitated with injection atropine, adrenaline, heparin, the patient also developed apnoea for a while and was intubated. Transthoracic echocardiography was performed throughout the procedure and LA cavity was found very small to have enough space to open the LA disk freely. A patient was settled within half an hour. Then an improvised technique was used to deploy the ASD device. The patient was heparinized again with 100ml/kg heparin. A delivery sheath was placed again in Right Atrium. A left atrial disk was open to RA until it took the shape of onion and device was de-aired inside RA again. Then the whole system was pushed softly to LA keeping the head of the onion-shaped device towards the gap of the atrial septum. The LA disk was then rapidly opened and pulled down against the atrial septum. Then right atrial disk was released. This time there was no ECG or hemodynamic change. Tug test was done and the device was released. Echocardiography next morning showed no residual ASD and mild residual Pulmonary valve stenosis with PPG 20mmHg. A patient was discharged 24 hours after the procedure with the advice of tablet ecosprin 90 mg once daily for 6 months. Follow up appointment was given accordingly.