Catheterization and Cardiovascular Interventions 85:847–849 (2015)
Case Report Iatrogenic Left Pulmonary Artery to Left Atrium Fistula Riad Abou Zahr, MD, William E. Hellenbrand, MD, and Jeremy D. Asnes, MD Trans-catheter balloon angioplasty is a well-established treatment modality for pulmonary artery (PA) stenosis in children with congenital heart disease. We report a case of an unusual complication where a fistula developed between the left PA and the left atrium during balloon angioplasty in a patient with history of tetralogy of Fallot. This was successfully treated with placement of a covered stent. VC 2014 Wiley Periodicals, Inc. Key words: balloon angioplasty; complications; pediatric catheterization/intervention; fistula - shunts
INTRODUCTION
Trans-catheter balloon angioplasty is a wellestablished treatment modality for pulmonary artery (PA) stenosis in children with congenital heart disease. Although first instituted a little over 3 decades ago, it is currently considered the intervention of choice for stenosis of branch pulmonary arteries. Similar to other invasive procedures, balloon angioplasty carries certain risks and complications. The risk of significant trauma to the pulmonary arterial wall is 1–3% [1,2]. This may range from small confined extravasations to arterial ruptures leading to catastrophic events. We report a case of an unusual complication where a fistula developed between the left pulmonary artery (LPA) and the left atrium (LA) during balloon angioplasty in a patient with history of tetralogy of Fallot. We discuss some of the complications of this procedure and management options.
CASE
This is a case of a 3-month-old male infant with history of pulmonary atresia with ventricular septal defect and confluent central pulmonary arteries supplied exclusively by a patent ductus arteriosus. The LPA was hypoplastic. He underwent complete surgical repair at 6 days of life which consisted of ventricular septal defect patch closure, a long transannular patch from the right ventricular outflow tract into the main PA, a separate LPA patch augmentation for stenosis of the proximal LPA, and partial closure of a secundum atrial septal defect. C 2014 Wiley Periodicals, Inc. V
He was left with residual proximal LPA stenosis. At 8 weeks of age he underwent repeat cardiac catheterization at which time complete proximal occlusion of the LPA was diagnosed. The LPA was crossed with a wire and successfully balloon dilated to a diameter of 3.5 mm using a 6 mm balloon. The patient was treated with lovenox and later transitioned to aspirin for thrombosis prevention given his narrow LPA. His partial atrial septal defect had spontaneously closed. At 3-months-of-age he was found to have recurrent severe LPA stenosis and was referred again for interventional catheterization. Angiography demonstrated a large right PA and a stenotic origin of the LPA with a diameter of 2.5 1.9 mm2 (Fig. 1). The diameter of the LPA distal to the stenosis was 3.4 3.2 mm2. Following initial angioplasty with a 7 mm/2 cm Z-Med II balloon inflated to 17 atmospheres for 5 sec, there was Additional Supporting Information may be found in the online version of this article. Department of Pediatrics, Section of Pediatric Cardiology, Yale University School of Medicine, New Haven, Connecticut Conflict of interest: Nothing to report. *Correspondence to: Riad Abou Zahr; 302 LLCI Building, 333 Cedar Street, Department of Pediatrics, section of Pediatric Cardiology, Yale University School of Medicine, New Haven, CT 06520-8064. E-mail: [email protected] Received 14 May 2014; Revision accepted 19 September 2014 DOI: 10.1002/ccd.25687 Published online 26 September 2014 in Wiley Online Library (wileyonlinelibrary.com)
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Abou Zahr et al.
Fig. 1. AP with caudal/LAO angulation (left) and Lateral (right) projections: proximal LPA stenosis (AP, antero-posterior; LAO, left anterior oblique; LPA, left pulmonary artery).
mild residual stenosis with a proximal LPA diameter of 5.3 mm. Due to the initial severe nature of the stenosis and the rapid development of recurrent stenosis twice previously additional angioplasty was performed with an 8 mm/2 cm Z-Med II balloon that was maximally inflated for 5 sec. A follow up LPA angiogram demonstrated resolution of proximal stenosis with a proximal LPA diameter of 8.1 mm but a tear was seen in the middle of the stenotic area at the underside of the LPA with minimal contrast extravasation. No contrast was seen in the pleural space or lung parenchyma and no blood was retrieved on suctioning of the endotracheal tube. The patient remained hemodynamically stable. Repeat LPA angiography 10 min later demonstrated contrast entering the LA from the LPA (Fig. 2 and supporting information media: lateral). The creation of an LPA-LA fistula was confirmed by echocardiography: injection of agitated saline into the LPA resulted in the immediate appearance of bubbles in the LA and left ventricle. Due to concern for possible jailing of the left upper lobe branch PA we initially attempted to seal the fistula by implanting a Genesis 8 mm/12 mm stent in the proximal LPA with the hope that surrounding scar tissue would compress and occlude the fistula. However, the leak persisted after implant. Thus an iCast 7 mm/16 mm covered stent was remounted on a 8 mm/2 cm SDS balloon (the largest diameter 16 mm long iCast stent is 7 mm) and was implanted within Genesis stent. Repeat angiography demonstrated complete closure of the LPALA fistula with preserved flow to the left upper lobe PA (supporting information media: covered stent). The patient tolerated the procedure with no hemodynamic instability and is doing well in clinical follow up. Several months later, he underwent repeat catheterization and the stent was further expanded to match somatic growth without difficulty. DISCUSSION
Trans-catheter balloon angioplasty of the pulmonary arteries in children was first performed in the early
Fig. 2. AP with caudal/LAO angulation (left) and lateral (right) projections: Black arrow-head denotes the fistula from the proximal LPA to the LA. White arrow-heads show drainage into the LA (LPA, left pulmonary artery; LA, left atrium).
1980s after successful experimentation with lambs [3,4]. Currently, it is considered the treatment of choice for branch PA stenosis, particularly in infants and young children with a history of PA surgery [1]. Such patients often require serial angioplasties to adequately address this problem. Successful angioplasty results in tearing of the intimal and medial layers of the arterial wall which increases luminal cross-sectional area and may allow for vessel remodeling [5]. Using balloons with diameters two to four times the diameter of the stenosis but smaller than twice the diameter of the nonstenotic part of the vessel is needed to achieve a good and safe result [6]. Low pressure balloons are successful in disrupting the vessel wall in about 60% of cases [1]. The use of noncompliant high pressure balloons increased success rates to about 70% [7]. Recently, the use of cutting balloons in resistant cases, proved more effective than high pressure balloons with similar safety profile [8]. Reported complications of PA angioplasty range from small confined extravasations to life threatening events including death [1]. A recent multicenter study found about 2.7% incidence of significant trauma to the pulmonary arteries during balloon angioplasty and stent placement [2]. This was most often caused by excessive tearing of the arterial wall. Traumatic tears most frequently occur distal to the stenosis where the vessel wall is thinner [9]. Baker et al. found that elevated main PA pressure (pulmonary hypertension) and, to a lesser extent, history of recent cardiac surgery (less than 6 weeks prior to catheterization) were the most important risk factors leading to serious PA trauma [9]. In their experience, coil occlusion of a damaged vessel was lifesaving in some instances. To our knowledge, ours is the first reported case of LPA to LA fistula formation during balloon angioplasty of the LPA. Formation of such a fistula requires disruption of both the LPA wall and the LA free wall. Both the LA and the LPA are posterior structures. The
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Balloon Angioplasty Complication
LPA, after taking off from the main pulmonary trunk, runs superior to the LA, the left upper pulmonary vein and the left main bronchus and then descends anterior to the descending aorta and enters the oblique fissure. We believe that in our patient the prior surgical manipulation and pericardial patching of the LPA resulted in dense adhesions between the LPA and left atrial roof. During balloon dilation LPA wall disruption most likely occurred at or close to the patch site. The adherent LA wall tore along with the surrounding scar tissue resulting in a communication between these two structures. While congenital communications between the pulmonary arteries and the LA do rarely occur—more commonly between the right PA and the LA—this was certainly an unanticipated complication of balloon angioplasty [10]. This complication may have been avoided by primary stent angioplasty, which does not require over distention of the PA to achieve a durable result. However, primary stent angioplasty in infancy most often necessitates the use of stents that cannot achieve adult size and thus commits patients to future surgical intervention. CONCLUSION
Balloon dilation of branch PA stenosis in infants and small children is a relatively high-risk procedure. This report adds a new and unanticipated complication to the list of potential pitfalls associated with PA angioplasty. Furthermore, this unusual complication and its treatment highlight the utility of small size covered stents in the congenital catheterization laboratory. Although commercially available in the United States, iCast covered stents are not approved by the Food and Drug Administration for use in the pulmonary arteries but rather are approved for the treatment of tracheobronchial strictures. Despite the lack of a specific
849
indication for use, these stents should be included in the congenital catheterization laboratory inventory to aid in the treatment of complications such as the one described here. REFERENCES 1. Bergersen L, Lock JE. What is the current option of first choice for treatment of pulmonary arterial stenosis? Cardiol Young 2006;16:329–338. 2. Holzer RJ, Gauvreau K, Kreutzer J, Leahy R, Murphy J, Lock JE, Cheatham JP, Bergersen L. Balloon angioplasty and stenting of branch pulmonary arteries: adverse events and procedural characteristics: Results of a multi-institutional registry. Circ Cardiovasc Interv 2011;4:287–296. 3. Lock JE, Niemi T, Einzig S, Amplatz K, Burke B, Bass JL. Transvenous angioplasty of experimental branch pulmonary artery stenosis in newborn lambs. Circulation 1981;64:886–893. 4. Lock JE, Castaneda-Zuniga WR, Fuhrman BP, Bass JL. Balloon dilation angioplasty of hypoplastic and stenotic pulmonary arteries. Circulation 1983;67:962–967. 5. Edwards BS, Lucas RV, Jr., Lock JE, Edwards JE. Morphologic changes in the pulmonary arteries after percutaneous balloon angioplasty for pulmonary arterial stenosis. Circulation 1985;71: 195–201. 6. Perry SB, Keane JF, Lock JE. Interventional catheterization in pediatric congenital and acquired heart disease. Am J Cardiol 1988;61:109G–117G. 7. Gentles TL, Lock JE, Perry SB. High pressure balloon angioplasty for branch pulmonary artery stenosis: Early experience. J Am Coll Cardiol 1993;22:867–872. 8. Bergersen L, Gauvreau K, Justino H, Nugent A, Rome J, Kreutzer J, Rhodes J, Nykanen D, Zahn E, Latson L, Moore P, Lock J, Jenkins K. Randomized trial of cutting balloon compared with high-pressure angioplasty for the treatment of resistant pulmonary artery stenosis. Circulation 2011;124:2388–2396. 9. Baker CM, McGowan FX, Jr., Keane JF, Lock JE. Pulmonary artery trauma due to balloon dilation: Recognition, avoidance and management. J Am Coll Cardiol 2000;36:1684–1690. 10. Monge MC, Russell HM, Popescu AR, Robinson JD. Right pulmonary artery to left atrial fistula in a neonate: Case report and review of the literature. World J Pediatr Congenit Heart Surg 2014;5:306–310.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Case Report Iatrogenic Left Pulmonary Artery to Left Atrium Fistula Riad Abou Zahr, MD, William E. Hellenbrand, MD, and Jeremy D. Asnes, MD Trans-catheter balloon angioplasty is a well-established treatment modality for pulmonary artery (PA) stenosis in children with congenital heart disease. We report a case of an unusual complication where a fistula developed between the left PA and the left atrium during balloon angioplasty in a patient with history of tetralogy of Fallot. This was successfully treated with placement of a covered stent. VC 2014 Wiley Periodicals, Inc. Key words: balloon angioplasty; complications; pediatric catheterization/intervention; fistula - shunts
INTRODUCTION
Trans-catheter balloon angioplasty is a wellestablished treatment modality for pulmonary artery (PA) stenosis in children with congenital heart disease. Although first instituted a little over 3 decades ago, it is currently considered the intervention of choice for stenosis of branch pulmonary arteries. Similar to other invasive procedures, balloon angioplasty carries certain risks and complications. The risk of significant trauma to the pulmonary arterial wall is 1–3% [1,2]. This may range from small confined extravasations to arterial ruptures leading to catastrophic events. We report a case of an unusual complication where a fistula developed between the left pulmonary artery (LPA) and the left atrium (LA) during balloon angioplasty in a patient with history of tetralogy of Fallot. We discuss some of the complications of this procedure and management options.
CASE
This is a case of a 3-month-old male infant with history of pulmonary atresia with ventricular septal defect and confluent central pulmonary arteries supplied exclusively by a patent ductus arteriosus. The LPA was hypoplastic. He underwent complete surgical repair at 6 days of life which consisted of ventricular septal defect patch closure, a long transannular patch from the right ventricular outflow tract into the main PA, a separate LPA patch augmentation for stenosis of the proximal LPA, and partial closure of a secundum atrial septal defect. C 2014 Wiley Periodicals, Inc. V
He was left with residual proximal LPA stenosis. At 8 weeks of age he underwent repeat cardiac catheterization at which time complete proximal occlusion of the LPA was diagnosed. The LPA was crossed with a wire and successfully balloon dilated to a diameter of 3.5 mm using a 6 mm balloon. The patient was treated with lovenox and later transitioned to aspirin for thrombosis prevention given his narrow LPA. His partial atrial septal defect had spontaneously closed. At 3-months-of-age he was found to have recurrent severe LPA stenosis and was referred again for interventional catheterization. Angiography demonstrated a large right PA and a stenotic origin of the LPA with a diameter of 2.5 1.9 mm2 (Fig. 1). The diameter of the LPA distal to the stenosis was 3.4 3.2 mm2. Following initial angioplasty with a 7 mm/2 cm Z-Med II balloon inflated to 17 atmospheres for 5 sec, there was Additional Supporting Information may be found in the online version of this article. Department of Pediatrics, Section of Pediatric Cardiology, Yale University School of Medicine, New Haven, Connecticut Conflict of interest: Nothing to report. *Correspondence to: Riad Abou Zahr; 302 LLCI Building, 333 Cedar Street, Department of Pediatrics, section of Pediatric Cardiology, Yale University School of Medicine, New Haven, CT 06520-8064. E-mail: [email protected] Received 14 May 2014; Revision accepted 19 September 2014 DOI: 10.1002/ccd.25687 Published online 26 September 2014 in Wiley Online Library (wileyonlinelibrary.com)
848
Abou Zahr et al.
Fig. 1. AP with caudal/LAO angulation (left) and Lateral (right) projections: proximal LPA stenosis (AP, antero-posterior; LAO, left anterior oblique; LPA, left pulmonary artery).
mild residual stenosis with a proximal LPA diameter of 5.3 mm. Due to the initial severe nature of the stenosis and the rapid development of recurrent stenosis twice previously additional angioplasty was performed with an 8 mm/2 cm Z-Med II balloon that was maximally inflated for 5 sec. A follow up LPA angiogram demonstrated resolution of proximal stenosis with a proximal LPA diameter of 8.1 mm but a tear was seen in the middle of the stenotic area at the underside of the LPA with minimal contrast extravasation. No contrast was seen in the pleural space or lung parenchyma and no blood was retrieved on suctioning of the endotracheal tube. The patient remained hemodynamically stable. Repeat LPA angiography 10 min later demonstrated contrast entering the LA from the LPA (Fig. 2 and supporting information media: lateral). The creation of an LPA-LA fistula was confirmed by echocardiography: injection of agitated saline into the LPA resulted in the immediate appearance of bubbles in the LA and left ventricle. Due to concern for possible jailing of the left upper lobe branch PA we initially attempted to seal the fistula by implanting a Genesis 8 mm/12 mm stent in the proximal LPA with the hope that surrounding scar tissue would compress and occlude the fistula. However, the leak persisted after implant. Thus an iCast 7 mm/16 mm covered stent was remounted on a 8 mm/2 cm SDS balloon (the largest diameter 16 mm long iCast stent is 7 mm) and was implanted within Genesis stent. Repeat angiography demonstrated complete closure of the LPALA fistula with preserved flow to the left upper lobe PA (supporting information media: covered stent). The patient tolerated the procedure with no hemodynamic instability and is doing well in clinical follow up. Several months later, he underwent repeat catheterization and the stent was further expanded to match somatic growth without difficulty. DISCUSSION
Trans-catheter balloon angioplasty of the pulmonary arteries in children was first performed in the early
Fig. 2. AP with caudal/LAO angulation (left) and lateral (right) projections: Black arrow-head denotes the fistula from the proximal LPA to the LA. White arrow-heads show drainage into the LA (LPA, left pulmonary artery; LA, left atrium).
1980s after successful experimentation with lambs [3,4]. Currently, it is considered the treatment of choice for branch PA stenosis, particularly in infants and young children with a history of PA surgery [1]. Such patients often require serial angioplasties to adequately address this problem. Successful angioplasty results in tearing of the intimal and medial layers of the arterial wall which increases luminal cross-sectional area and may allow for vessel remodeling [5]. Using balloons with diameters two to four times the diameter of the stenosis but smaller than twice the diameter of the nonstenotic part of the vessel is needed to achieve a good and safe result [6]. Low pressure balloons are successful in disrupting the vessel wall in about 60% of cases [1]. The use of noncompliant high pressure balloons increased success rates to about 70% [7]. Recently, the use of cutting balloons in resistant cases, proved more effective than high pressure balloons with similar safety profile [8]. Reported complications of PA angioplasty range from small confined extravasations to life threatening events including death [1]. A recent multicenter study found about 2.7% incidence of significant trauma to the pulmonary arteries during balloon angioplasty and stent placement [2]. This was most often caused by excessive tearing of the arterial wall. Traumatic tears most frequently occur distal to the stenosis where the vessel wall is thinner [9]. Baker et al. found that elevated main PA pressure (pulmonary hypertension) and, to a lesser extent, history of recent cardiac surgery (less than 6 weeks prior to catheterization) were the most important risk factors leading to serious PA trauma [9]. In their experience, coil occlusion of a damaged vessel was lifesaving in some instances. To our knowledge, ours is the first reported case of LPA to LA fistula formation during balloon angioplasty of the LPA. Formation of such a fistula requires disruption of both the LPA wall and the LA free wall. Both the LA and the LPA are posterior structures. The
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Balloon Angioplasty Complication
LPA, after taking off from the main pulmonary trunk, runs superior to the LA, the left upper pulmonary vein and the left main bronchus and then descends anterior to the descending aorta and enters the oblique fissure. We believe that in our patient the prior surgical manipulation and pericardial patching of the LPA resulted in dense adhesions between the LPA and left atrial roof. During balloon dilation LPA wall disruption most likely occurred at or close to the patch site. The adherent LA wall tore along with the surrounding scar tissue resulting in a communication between these two structures. While congenital communications between the pulmonary arteries and the LA do rarely occur—more commonly between the right PA and the LA—this was certainly an unanticipated complication of balloon angioplasty [10]. This complication may have been avoided by primary stent angioplasty, which does not require over distention of the PA to achieve a durable result. However, primary stent angioplasty in infancy most often necessitates the use of stents that cannot achieve adult size and thus commits patients to future surgical intervention. CONCLUSION
Balloon dilation of branch PA stenosis in infants and small children is a relatively high-risk procedure. This report adds a new and unanticipated complication to the list of potential pitfalls associated with PA angioplasty. Furthermore, this unusual complication and its treatment highlight the utility of small size covered stents in the congenital catheterization laboratory. Although commercially available in the United States, iCast covered stents are not approved by the Food and Drug Administration for use in the pulmonary arteries but rather are approved for the treatment of tracheobronchial strictures. Despite the lack of a specific
849
indication for use, these stents should be included in the congenital catheterization laboratory inventory to aid in the treatment of complications such as the one described here. REFERENCES 1. Bergersen L, Lock JE. What is the current option of first choice for treatment of pulmonary arterial stenosis? Cardiol Young 2006;16:329–338. 2. Holzer RJ, Gauvreau K, Kreutzer J, Leahy R, Murphy J, Lock JE, Cheatham JP, Bergersen L. Balloon angioplasty and stenting of branch pulmonary arteries: adverse events and procedural characteristics: Results of a multi-institutional registry. Circ Cardiovasc Interv 2011;4:287–296. 3. Lock JE, Niemi T, Einzig S, Amplatz K, Burke B, Bass JL. Transvenous angioplasty of experimental branch pulmonary artery stenosis in newborn lambs. Circulation 1981;64:886–893. 4. Lock JE, Castaneda-Zuniga WR, Fuhrman BP, Bass JL. Balloon dilation angioplasty of hypoplastic and stenotic pulmonary arteries. Circulation 1983;67:962–967. 5. Edwards BS, Lucas RV, Jr., Lock JE, Edwards JE. Morphologic changes in the pulmonary arteries after percutaneous balloon angioplasty for pulmonary arterial stenosis. Circulation 1985;71: 195–201. 6. Perry SB, Keane JF, Lock JE. Interventional catheterization in pediatric congenital and acquired heart disease. Am J Cardiol 1988;61:109G–117G. 7. Gentles TL, Lock JE, Perry SB. High pressure balloon angioplasty for branch pulmonary artery stenosis: Early experience. J Am Coll Cardiol 1993;22:867–872. 8. Bergersen L, Gauvreau K, Justino H, Nugent A, Rome J, Kreutzer J, Rhodes J, Nykanen D, Zahn E, Latson L, Moore P, Lock J, Jenkins K. Randomized trial of cutting balloon compared with high-pressure angioplasty for the treatment of resistant pulmonary artery stenosis. Circulation 2011;124:2388–2396. 9. Baker CM, McGowan FX, Jr., Keane JF, Lock JE. Pulmonary artery trauma due to balloon dilation: Recognition, avoidance and management. J Am Coll Cardiol 2000;36:1684–1690. 10. Monge MC, Russell HM, Popescu AR, Robinson JD. Right pulmonary artery to left atrial fistula in a neonate: Case report and review of the literature. World J Pediatr Congenit Heart Surg 2014;5:306–310.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
