A 19 year old tribal girl presented with history of progressive effort dyspnoea of five years duration with bluish discoloration of nails and lips for the past three years. By means of clinical examination and multimodality imaging which included transthoracic echocardiography, cardiac CT scan, cardiac MRI scan and invasive cath study, she was diagnosed to have a biventricular non compaction with heart failure and a pulmonary arteriovenous malformation. She had deep cyanosis due to right to left shunt at two levels, through the stretched open PFO due to severe RV dysfunction and through the pulmonary arteriovenous malformation.
The case was discussed in the heart team meeting. Device closure of AVM to observe the improvement in saturation was the only feasible option as surgical lobectomy followed by a bidirectional Glenn was not approved as the LVEF was only 35%. We decided for selective closure of the two feeders, as a common trunk which gives rise to the two feeders could not be identified.
Device closure of one feeder of pulmonary AVM was done initially with a AmplatzTM Vascular Plug II(AVP II)(Abbott Cardiovascular, Plymouth, Minnesotta, USA) resulting in improvement in saturation to 88% from baseline value of 72%(Figure 1). But, repeated attempts for introducing sheaths or 5F Judgkins Right(JR) 3.5 guiding catheter(Cordis, Santa Clara, California) into the other feeder from pulmonary arterial side was futile due to acute angulation at the origin of the second feeder. Stiff wires could not be introduced deep inside the feeder. Even a 4F catheter could not be negotiated deep into the feeder of AVM over exchange length coronary wires. An innovative technique of veno venous loop creation was done for introducing the guiding catheter into the other feeder. Using a 5F JR 3.5 guiding catheter a curved tip Terumo wire (Terumo Medical Corporation, Somerset, New Jersey) was introduced through inferior vena cava into right atrium and through patent foramen ovale into left atrium. The wire was then carefully introduced through the left lower pulmonary vein into the AVM and was manipulated through the AVM into the arterial feeder which was difficult to enter during previous attempts. By careful manipulations the wire could be advanced into the right pulmonary artery which was then snared out through the other femoral vein creating the veno venous loop(Figure 2,3). ( Wire through right femoral vein -> inferior venacava -> RA -> PFO -> left atrium -> pulmonary vein -> AVM -> arterial feeder ->pulmonary artery -> snared out -> RV -> RA -> inferior vena cava -> Left Femoral vein ). After loop creation the guiding catheter could be negotiated into the arterial feeder. A second AVP II device was deployed(Figure 4). After device closure the saturation improved on table to 92%. Patient was discharged with guideline directed medical management for heart failure. On follow up patient reported significant symptomatic improvement despite persistence of mild cyanosis. Repeat contrast echo showed persistence of right to left shunt at the level of ASD/PFO. There was mild improvement in LV systolic function with persisting restrictive RV filling pattern.