Introduction
Anomalous left coronary artery from pulmonary artery is a rarely encountered congenital cardiac anomaly in adults, with an estimated incidence of 0.01%. Reference Kandzari, Harrison and Behar1 When diagnosed in infancy it is associated with high mortality, but extensive collaterals permit rare adult survival despite coronary steal. Reference Yau, Singh, Halpern and Fischman2 Patients at older age present with angina, dyspnoea, or sudden death complicating ventricular arrhythmia. Reference Frapier, Leclercq, Bodino and Chaptal3 While treatment usually consists of surgical repair, there are a few case reports of alternative transcatheter management instead. We report improvement of perfusion abnormality after transcatheter proximal left coronary occlusion in a symptomatic adult with inducible ischaemia.
Case details
A 48-year-old female presented with exertional chest pain; ECG (Figure 1) showed sinus rhythm with left axis deviation with intraventricular conduction defect.
EKG – with sinus rhythm, left axis deviation, and intraventricular conduction defect. No pathological Q waves seen in anterolateral leads.
Echocardiogram diagnosed an anomalous left coronary artery arising from the pulmonary artery with normal ventricular function which was further confirmed by CT (Figure 2). Exercise stress showed large perfusion defects (Figure 3).
ECHO still image in parasternal short-axis view at the level of pulmonary valve, showing coronary flow retrogradely directed into posterior pulmonary cusp (blue arrow) collaterals in RVOT septum.
(a) and (b) CT images in coronary arterial phase and 3D reconstruction image of coronary vessels, showing left coronary artery draining into pulmonary cusp and dilated right coronary artery.
Methods
The case was discussed with surgical team, and it was decided to surgically ligate left coronary artery. Patient denied surgical procedure but agreed for transcatheter left coronary artery occlusion. A rotational right coronary angiography identified 40° left anterior oblique projection to profile the coronary away from the pulmonary artery. 5F Judkins right catheter was tracked into left coronary artery from the pulmonary sinus after tracking 0.032″ angle tip hydrophilic wire (Terumo). After this step, 0.032″ angle tip hydrophilic wire was exchanged with 0.035″ angle tip Teflon wire. Another 0.014″ Grand slam coronary wire was parked distally inleft circumflex artery for better support. 8F Guiding Judkins right catheter was deeply throated with the buddy wire technique into LMCA. 14 X 12 mm Lifetech Konar-MFO device was deployed to occlude the origin of vessel at the pulmonary end. Device was released safely after confirming the position on echocardiogram. After releasing the device, it was realised that mistakenly the device was loaded in reverse manner (waist diameter on right side is smaller than waist diameter in left side). We have deployed the device in a way that smaller waist was towards the high-pressure side, and larger waist towards low-pressure side, which eventually resulted in deployment of the device in reverse manner (Figure 4a–i).
Fluoroscopic images of device deployment. (a,b) Coronary angiogram views in RAO and LAO views, respectively, profiling ecstatic coronary arteries with LMCA draining in to PA. (c) PTCA wire placed in LAD. (d) Grand slam in the circumflex. (e) 0.035” wire parked in proximal left circumflex artery for support with PTCA wire in distal LAD. (f) Konar-MF device being deployed into coronary. (g,h) Check coronary angiogram post-device deployment in LAO and RAO views, respectively. (i) Post-device deployment.
Patient was shifted to ICU after the procedure, and close follow-up was done with echocardiogram along with continuous ECG monitoring. The next morning, it was noted that device got embolised into left pulmonary artery (Figure 5a–c). Patient was shifted to Cath lab immediately with a plan to retrieve the device back with snare. Device was snared back from LPA without any complication.
Retrieval of embolised device. (a) Device embolisation in to LPA confirmed with check pulmonary angiography. (b) Device snared and brought down to IVC. (c) Device tracked into the delivery sheath in the right iliac region.
This time a bigger device was considered to close this defect, and the devices considered were 16 mm vascular plug, 18 X 16 PDA occluder, and 18 mm muscular VSD occluder. The same steps were repeated as done previously, but a bigger (8,9F) long sheath could not be advanced into the desired site. 8F guiding JR was the only hardware that was tracked successfully into the desired site. Finally, it was decided to deploy the Lifetech 14 X 12 Konar-MFO device with desired orientation (larger disc towards high-pressure site and smaller disc towards low-pressure site as this was the only device that can be delivered with 8F guiding JR among the available devices (Figure 6a–c).
Redeployment of Konar-MFO device in reverse manner with bulky retention disc into coronary end. (a, b) Device redeployment through JR guiding catheter in LMCA confirming occlusion with check coronary angiogram. (c) Post-device deployment.
Device was released safely after confirming the position on echocardiogram.
Results
Patient showed improvement in intraventricular conduction defect and QTc duration, and also no significant ischaemic changes were noted (Figure 7).
ECG post-procedure, with normal sinus rhythm, left axis deviation, intraventricular conduction delay. No ischaemic changes observed.
Repeat CT done after 6 months revealed device in situ with no focal aneurysmal dilation of the distal LMCA and also good opacification of the left coronaries noted through collaterals (Figure 8).
CT coronary angiography post-device occlusion of LMCA: left – 3D reconstruction image of coronaries with device in situ at LMCA–PA junction, middle and right: coronal CT image with arterial phase showing device in situ.
Myocardial perfusion scintigraphy was done after 6 months of procedure which showed improvement in contractile function and perfusion of left ventricle (Figure 9).
Showing Tc sestamibi myocardial perfusion scintigraphy scan showing decreased tracer uptake in the LAD territory.
Discussion
Anomalous left coronary artery from pulmonary artery was first described by Abbott in a 60-year-old woman in 1908. Symptoms and timing of presentation of anomalous left coronary artery from pulmonary artery are variable, depending on the adequacy of the collateralised left coronary artery circulation and pulmonary artery pressures. Patients with satisfactory collateralisation may have a variable onset of symptoms. It can be a good point for discussion that it is always better to have two coronary systems with reimplantation of coronary artery. We have observed that in older patients with very well-collateralised circulation, the ischaemia is more due to coronary steal. Reference Egorova, Ewert, Hadamitzky and Eicken4– Reference Hauser6 Myocardial steal and consequent myocardial ischaemia from left to right shunting along with left ventricular dysfunction may be associated with large interconnections as seen in our patient. Dual coronary perfusion with an additional bypass graft has its advantages; however, only left coronary occlusion may be considered in view of high surgical risks, graft occlusions on follow-up, slow-flow in venous grafts, and non-suitability of mammary grafts. Long-term follow-up of adults, post-transcatheter strategy of management holds the key to considering it as true surgical alternative. Most importantly, the patient was not at all ready for any surgical treatment and was desperate only to have transcatheter treatment. The plan was to close the anomalous left coronary artery from pulmonary artery at PA end with a device and to watch for any signs of low coronary perfusion. If at all needed, there is always a possibility of doing off-pump LIMA to LAD graft. This plan has been discussed with surgical team and explained to patient for which we all agreed. With the advancement of available hardware and devices, more and more structural congenital and acquired heart defects are now amenable for transcatheter approach.Reference Kenny7 Before this case, Sivakumar KReference Sagar and Sivakumar8 and team have closed the similar lesion with device and proved long-term safety. They have also documented improvement in symptoms and perfusion of myocardium. In our case, also perfusion has improved to almost normal with significant improvement in LV function.
