Catheterization and Cardiovascular Interventions 85:1017–1020 (2015)

Percutaneous Left Main Coronary Artery Stent for Acute Myocardial Ischemia after Repaired ALCAPA Anna Hallbergson,1,2* MD, PhD and Jonathan J. Rome,1 MD Percutaneous coronary artery stent angioplasty is rare in the pediatric population but can be a life-saving by rapidly reestablishing flow to an obstructed coronary artery. It is a technically challenging and high-risk procedure in infants and further limited by the need for future surgical intervention. We report of an infant with anomalous left coronary artery from the pulmonary artery who underwent acutely successful surgical reimplantation of the left coronary artery onto the ascending aorta. One month later, she developed acute myocardial ischemia and emergent catheterization diagnosed near-total occlusion of the left coronary artery. We implanted a 2.5 mm coronary stent in the left main coronary artery with reestablishment of flow. The patient’s left ventricular systolic function recovered within 4 weeks and repeat angiography 3 months later showed complete normalization of the entire left coronary artery system. The patient weighed 3 kg and was < 6 weeks of age at the time of stent implantation which to our knowledge is the smallest and youngest reported patient to undergo coronary stent angioplasty. This case supports the feasibility of this procedure in infants as a temporizing solution to hemodynamic instability from myocardial ischemia due to coronary artery stenosis. The left ventricular systolic function remained normal at 7 months after stent placement and the patient was clinically well from a cardiac perspective. VC 2014 Wiley Periodicals, Inc.

Key words: coronary artery disease; left main coronary disease; pediatric intervention; percutaneous coronary intervention (PCI)

INTRODUCTION

CASE

Percutaneous coronary interventions, including stent placement, in pediatric patients are rare despite their well-established role in adults with acute coronary syndrome and myocardial infarction. Limitations to stent angioplasty in infants include the small size of the coronary arteries, related procedural difficulty, and risk of adverse events [1]. Expected somatic growth and concern about long-term outcome of stents on vascular integrity in infant coronaries appropriately limit their use. However, there are important clinical scenarios when percutaneous coronary stent angioplasty may be a life-saving option in infants, such as obstruction from anatomic distortion or compression of a coronary artery following cardiac surgery or catheterization resulting in acute hemodynamic instability from myocardial ischemia. In addition, it could be a temporizing strategy in children with subacute presentation of coronary artery stenosis if they are poor candidates for surgical reconstruction. Prior reports support the feasibility of coronary artery stent angioplasty in infants and children as a strategy for short-or long-term bridging for surgical revascularization or transplantation [2–4]. To our knowledge, our patient is the smallest (3 kg) and youngest (5 weeks) reported patient to undergo successful coronary stent angioplasty.

The patient was born a 2.9 kg term product of an uncomplicated pregnancy and delivery to a 27-year-old healthy mother with good prenatal care. Severe hypoglycemia presenting on day of life 1 was refractory to standard treatment. Further testing raised concern for congenital hyperinsulinism which prompted transfer to our center. On day of life 7 ECG demonstrated pathologic Q waves in the inferior leads and diffuse ST segment changes. Echocardiogram demonstrated normal

C 2014 Wiley Periodicals, Inc. V

1

Division of Cardiology, The Children’s Hospital of Philadelphia, Perelman School of Medicine and the University of Pennsylvania, Philadelphia, Pennsylvania 2 Children’s Heart Center, Skane University Hospital, Lund 221 85, Sweden Conflict of interest: Nothing to report. *Correspondence to: Anna Hallbergson, Children’s Heart Center, Skane University Hospital, 221 85 Lund, Sweden. E-mail: [email protected] Received 20 June 2014; Revision accepted 30 November 2014 DOI: 10.1002/ccd.25771 Published online 3 February 2015 in Wiley Online Library (wileyonlinelibrary.com)

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intracardiac anatomy, significant regional wall motion abnormality with marked hypokinesis of the left ventricular (LV) free wall. An aortic root angiogram confirmed the diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA). The patient underwent operative reimplantation of the anomalous left coronary (LCA) onto the aorta with reconstruction of the main pulmonary artery. The anomalous coronary arose high on the main pulmonary artery (above the sinotubular junction near the right pulmonary artery origin) which required the coronary button to be reimplanted high onto the ascending aorta. A small piece of homograft was used as a hood on the reimplanted left coronary artery to avoid kinking. Post-operative echocardiograms showed normalization of LV systolic function and mild mitral regurgitation (MR), however right ventricular (RV) pressure was estimated to be suprasystemic and branch pulmonary artery stenosis was noted. Following initial periods of hemodynamic instability, the patient was extubated 3 weeks after surgery. The patient developed significant tachypnea 4 weeks postoperatively. Echocardiogram demonstrated absence of anterograde flow in the reimplanted LCA, severely diminished LV systolic function with near lack of LV free wall motion, and moderate to severe MR with echo-bright papillary muscles. Severe acute elevation in serum troponin was also consistent with myocardial ischemia. Emergent catheterization revealed bilaterally elevated filling pressures and severe RV hypertension (110/18 mm Hg) due to bilateral central PA stenosis with a diffusely hypoplastic distal pulmonary arterial tree. There was virtual occlusion of the proximal left main coronary artery (LMCA), with a string-like appearance to the entire left coronary tree (Fig. 1). On initial angiograms, the left anterior descending artery was not visualized. In preparation for stent angioplasty via femoral artery access, a 4-Fr long hydrophilic sheath was modified with a right coronary artery curve using heat. Together with a 4-Fr Judkins right coronary catheter this was used to engage the ostium of the LMCA. An 0.01400 intermediate support wire was advanced into the LMCA to the circumflex coronary artery. The coronary catheter was removed and a 2.5 mm  8 mm MiniVision coronary stent was advanced over the wire and deployed in the LMCA proximal to its bifurcation. Post-intervention angiography showed reestablished flow to both the circumflex and anterior descending coronary arteries both of which remained of profoundly diminutive in size (Fig. 1). Following stent placement LV systolic function rapidly improved though it remained moderately depressed. Stent thromboprophylaxis was initiated with aspirin (2–4 mg/kg/day) and clopidogrel (0.2–0.4 mg/

Fig. 1. Serial angiography demonstrating initial occlusion of the left coronary artery with reconstitution of flow following stent placement. Aortic root angiogram (A, B) and selective left coronary angiography (C, D) prior to coronary artery stent placement. Arrows in A–C indicate the threadlike left main coronary artery. Following engagement of the left coronary os and placement of a wire in the circumflex coronary artery (D, arrow), a threadlike left anterior descending artery is visualized. Aortic root angiogram immediately post stent placement (E, F) and 3.5 months (G, H) after stent placement showing complete resolution of flow in the left coronary artery. In panels (E–H), the arrows point at the circumflex and the arrowheads point at the left anterior descending artery. The hood of homograft used in the surgical repair can be seen as a protrusion of the ascending aorta wall just distal to the sinotubular junction in multiple projections.

kg/day). The patient was treated with lovenox for femoral artery thrombosis. Additional medical management included afterload reduction as well as beta blocker,

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

Coronary Artery Stent after ALCAPA Repair

and diuretic therapy. LV systolic function had normalized 4 weeks after stent placement. Cardiac catheterization 3 months after stent angioplasty showed normal cardiac output, a small right to left shunt, normal bilateral filling pressures, and persistent severe RV hypertension (RV 115/8, aorta 90/ 35 mm Hg) due to severe peripheral pulmonary stenosis. There was no significant supravalvar pulmonary stenosis (a well-described complication following ALCAPA repair). Both central pulmonary arteries responded well to angioplasty using high-pressure balloons with associated reduction in RV pressure (RV 90/10, aorta 110/42 mm Hg). Coronary angiography showed no significant in-stent stenosis and complete normalization of the left coronary vasculature (Fig. 1). The most recent echocardiogram performed as an outpatient 7 months after stent placement showed normal LV function, flow in the stented LCA, RV hypertension, and bilateral pulmonary artery stenosis. Extensive genetic testing has been unrevealing, including negative testing for Williams syndrome. DISCUSSION

This case demonstrates the feasibility of percutaneous coronary artery stent implantation in a small infant, in this case a 3 kg 5-week-old infant with repaired ALCAPA. The patient developed cardiovascular collapse due to near-total occlusion of the reimplanted left coronary artery 1 month after surgery. Stent angioplasty allowed for recovery of severely depressed LV systolic function which remained normal at 7 month follow-up. To our knowledge this is the smallest patient with reported coronary artery stenting and the first report of coronary artery stenting in an infant < 6 weeks of age. There have been a number of prior reports of coronary stent angioplasty in children, the majority were also placed following ALCAPA repair [2,4–6]. In one series, 4 children ranging from 7 to 15 months in age were treated including a 5 kg infant who, similar to our patient, developed stenosis of the reimplanted LMCA one month after ALCAPA repair. Another case series of percutaneous coronary stent implantation in 6 children aged 8–18 years suggested particular effectiveness in postsurgical coronary compression [3]. The optimal prophylaxis for prevention of coronary artery stent thrombosis in infants is not known. We presume that the small diameter of this stent increases risk for this potentially fatal complication. Dual antiplatelet agent therapy (aspirin and a platelet P2Y12 receptor blocker), the standard in adults following placement of either bare-metal or drug-eluding coronary artery stents [7], seems the most reasonable strategy. In

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a case series of infants weighing 5–12 kg with bare metal stents ranging from 2 to 3 mm in diameter, dual antiplatelet therapy was used in three infants and aspirin alone in one. Of the four patients, the infant treated with aspirin alone developed acute stent thrombosis and died 5 days after placement [2]. We elected to treat our patient with dual anti-platelet therapy (aspirin and clopidogrel) in addition to the anticoagulation with enoxaparin which she was receiving for a femoral vessel thrombus. Limitations to percutaneous coronary stent angioplasty in small infants include technical challenges, high procedural risk, and the likely need for subsequent surgical removal with coronary artery repair. In addition, the small femoral arteries in infants typically limit the sheath and catheter sizes to 3–4 Fr with associated limitation in catheter varieties. In our patient a modified long sheath served in lieu of a coronary guide catheter; this sheath together with the coronary catheter provided the necessary angle needed to engage the obstructed coronary artery and advance a coronary guidewire. Once the wire was in place, sheath position was stable enough to allow stent delivery and stent angiograpy. The margin for error in these tiny obstructed vessels is quite small and the risk of vessel injury during intervention is likely high. As noted, the small size of infants’ coronary arteries only allow for 2 to 3 mm diameter coronary stents which cannot be subsequently dilated to accommodate the size of adult coronary arteries. Therefore, stent angioplasty in infants should only be viewed as a temporizing measure with anticipated need for future surgical removal and coronary artery repair. This patient had a previously unreported association of ALCAPA, peripheral pulmonary artery stenosis, and congenital hyperinsulinism. Infants with congenital hyperinsulinism have been reported to have an increased incidence of cardiac structural lesions and hypertrophic cardiomyopathy [8,9], however coronary or pulmonary artery abnormalities have not been previously reported.

CONCLUSION

Percutaneous coronary artery stent implantation in infants is likely to remain a rare procedure. However, multiple case reports support its feasibility for management of acute coronary ischemia due to postoperative coronary artery obstruction. In this 3 kg patient with cardiovascular collapse due to a nearly totally occluded left coronary artery following repaired ALCAPA, we report complete recovery of ventricular function which persisted at 7 month follow-up and complete size and flow reconstitution of the left coronary artery system.

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

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The long-term outcome of coronary arteries that have been stented in infancy remains unknown. REFERENCES 1. Bergersen L, Marshall AC, Gauvreau K, Beekman R, Hirsch R, Foerster S, Balzer D, Vincent J, Hellenbr W, Holzer R, Cheatham J, Moore J, Lock JE, Jenkins K. Adverse event rates in congenital cardiac catheterization—A multi-center experience. Catheter Cardiovasc Interv 2010;75:389–400. 2. Bratincsak A, Salkini A, El-Said HG, Moore JW. Percutaneous stent implantation into coronary arteries in infants. Catheter Cardiovasc Interv 2012;79:303–311. 3. Schneider M, Wiebe W, Hraska V, Zartner P Coronary interventions in congenital heart diseases: From preterm to young adult patients. J Interv Cardiol 2013;26:287–294. 4. Schneider AE, Johnson JN, Taggart NW, Cabalka AK, Hagler DJ, Reeder GS, Cetta F. Percutaneous coronary intervention in pediatric and adolescent patients. Congenit Heart Dis 2014;9:228–234.

5. De Lezo JS, Pan M, Herrera C. Combined percutaneous revascularization and cell therapy after failed repair of anomalous origin of left coronary artery from pulmonary artery. Catheter Cardiovasc Interv 2009;73:833–837. 6. Chrysant GS, Balzer D, Taniuchi M. Left main stem coronary artery stenting in a 3-month-old child after anomalous left coronary artery from pulmonary artery repair. Pediatr Cardiol 2005; 26:734–736. 7. Brilakis ES, Patel VG, Banerjee S. Medical management after coronary stent implantation: A review. JAMA 2013;310:189– 198. 8. Banerjee I, Avatapalle B, Petkar A, Skae M, Padidela R, Ehtisham S, Patel L, Rigby L, Flanagan S, Ellard S, Jones C, Ciotti G, Malaiya N, Clayton PE. The association of cardiac ventricular hypertrophy with congenital hyperinsulinism. Eur J Endocrinol 2012;167:619–624. 9. Huang T, Kelly A, Becker SA, Cohen MS, Stanley CA. Hypertrophic cardiomyopathy in neonates with congenital hyperinsulinism. Arch Dis Child Fetal Neonatal Ed 2013;98:F351–F354.

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

Percutaneous left main coronary artery stent for acute myocardial ischemia after repaired ALCAPA.

Percutaneous coronary artery stent angioplasty is rare in the pediatric population but can be a life-saving by rapidly reestablishing flow to an obstr...
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