SHA24/077006

Challenging case Shakeel A Qureshi Evelina Children’s Hospital London, UK

Complex pulmonary atresia • A girl now 7 years old • Diagnosed at 4 weeks of age with complex pulmonary atresia, VSD, tiny central pulmonary arteries, MAPCAs 3 to right, 2 to left with multiple stenoses • Had staged unifocalisation of left MAPCAs with 4 mm shunt age 7 months • Unifocalisation of right MAPCAs and reconstruction of central PAs and RV to PA with 17 mm aortic homograft • VSD partly left open

Complex pulmonary atresia • Age 4 years, moderately cyanosed with R to L shunt through VSD • In catheter lab, RV and MPA pressures suprasystemic • Gradient of 40 mm Hg in RUL PA

Complex pulmonary atresia

Not easy to cross RLLPA or LPA

Complex pulmonary atresia

Decided to deal with RULPA first

6 mm cutting balloon through 8 Fr sheath

Complex pulmonary atresia

Balloon carefully withdrawn into sheath

RULPA angio after cutting balloon

Complex pulmonary atresia

Premounted 6 mm x 17 mm Visipro stent in place

Visipro stents • Open cell premounted up to 10 diameter • Can be overexpanded to 14 mm • 8m premounted Visipro dilated to 8 and then 14 mm

Complex pulmonary atresia

Post stent angio – good result

LPA angio – very tortuous branches

Complex pulmonary atresia • Post procedure, MPA pressure still systemic but O2 saturation increased from 80% to 88% • Gradient across RULPA reduced to 15 mm Hg • Maintained on sildenafil and bosentan • Improvement sustained over 2 years follow up • This year, increased cyanosis with sats of 78-80% • Brought back to catheter laboratory

Complex pulmonary atresia • RV and MPA pressures just above systemic

Complex pulmonary atresia

Intimal proliferation within stent Severe RLPA stenosis and tortuous vessel

Complex pulmonary atresia

Cutting balloon 5 mm in RULPA stent

Complex pulmonary atresia

RULPA stent angio after cutting balloon RLLPA is very tortuous and severely stenosed

Complex pulmonary atresia • Distal RLLPA pressure very low • Options for treatment?

Complex pulmonary atresia

Complex pulmonary atresia

5 mm cutting balloon in position Burst pressure 10 atm

Balloon at 10 atm

Complex pulmonary atresia

Balloon at 12 atm

Balloon at 14 atm Waist abolished

Complex pulmonary atresia

Dissection flap inevitable in this situation Final angio after Tyshak balloon inflation for 5 minutes

Complex pulmonary atresia • Post procedure RV and MPA pressure at systemic level • O2 saturations 92 – 94% 2 weeks after discharge • Plan to deal with LPA in the future • This is a difficult anatomy to deal • Best to plan multiple interventions • Plan is to perform CT scan in 2 months and plan further intervention on RLLPA (further cutting balloon and possibly visipro stent