Case Report
Contained Rupture of Mycotic Aneurysm of the Left Circumflex Coronary Artery in a Child
World Journal for Pediatric and Congenital Heart Surgery 2015, Vol. 6(1) 111-114 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135114551994 pch.sagepub.com
Reena K. Joshi, MD1, Aman Jyoti, MD1, Neeraj Aggarwal, MD2, Mridul Aggarwal, MD2, and Raja Joshi, MCh3
Abstract Coronary artery aneurysm (CAA) is defined as dilatation of a coronary artery segment to a diameter of more than 1.5-fold normal size. Rupture of CAA is a catastrophic event and may result in sudden death or myocardial infarction. We report this unusual case of contained rupture of the left circumflex CAA. Keywords coronary artery surgery, aneurysm (coronary artery, mycotic), pediatric, rupture (coronary artery aneurysm) Submitted April 27, 2014; Accepted August 25, 2014.
Introduction Coronary artery aneurysm (CAA) is a rare, though welldefined, entity that has been described in adults as well as in the pediatric patient population. Coronary artery aneurysm is typically defined as dilatation in the diameter of a coronary artery segment to more than 1.5-fold normal size. Among the etiological factors of CAA, the most common are Kawasaki disease (KD) in children and atherosclerotic coronary artery disease in adults. Rupture of CAA is a catastrophic event and may result in sudden death or myocardial infarction. Because of its unusual presentation and rarity, we report a case of contained rupture of CAA of mycotic etiology in a child.
Case Description An 18-month-old girl weighing 9.3 kg was referred to our facility with a two-week history of fever, diarrhea, and vomiting, despite antibiotic therapy. Cardiomegaly was noted on chest X-ray. On admission, she had a high-grade fever, tachycardia, and extreme irritability. Cough, skin rash, desquamation, or syncope was not observed. Laboratory values included white blood cell count of 19,500/ mL, hematocrit 21%, platelet count 564,000/mL, serum procalcitonin level 2.16ng/mL, C-reactive protein 274 mg/L, and interleukin 6 of 49.84 pg/mL. The blood, urine, and gastric aspirate cultures were sterile. On echocardiography, a 50 60 mm heterogeneous mass surrounded by a large fluid cavity could be seen near the posterior atrioventricular groove. Global and regional cardiac wall motions were normal. Computed tomography (CT) angiography of the thorax
revealed a large, thick-walled, multi-loculated collection surrounding the left ventricle (LV) with an enhancing nodular lesion that appeared to be related to the left circumflex (LCX) coronary artery (Figure 1). Electrocardiogram (ECG) showed no significant abnormalities and biochemical markers of myocardial ischemia were negative. A coronary angiogram confirmed the presence of an isolated saccular aneurysm of the LCX with no coronary artery stenosis (Figure 2). Multivessel involvement was ruled out by CT angiography, fundoscopy, and a vascular Doppler study of carotid and limb arteries. Intravenous immunoglobulin (IVIG), methylprednisolone, and oral aspirin were administered to address the severe degree of inflammation. After 96 hours, marked reduction in the values of inflammatory markers was observed, and gradual resolution of the febrile episodes was also noted. Because of the presence of the coronary artery aneurysm and the multi-loculated collection surrounding the LV, the child was taken to surgery. After induction of anesthesia, midline sternotomy was done and dense fibrous intrapericardial 1 Pediatric Cardiac Anesthesia, Department of Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, New Delhi, India 2 Pediatric Cardiology, Department of Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, New Delhi, India 3 Pediatric Cardiac Surgery, Department of Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, New Delhi, India
Corresponding Author: Reena K. Joshi, Department of Pediatric Cardiac Sciences, Institute of Child Health & Dharamvira Heart Center, Sir Ganga Ram Hospital, New Delhi, India. Email: [email protected]
112
World Journal for Pediatric and Congenital Heart Surgery 6(1)
Abbreviations and Acronyms CAA CPB CT ECG IVIG KD LAD LCX LMCA LV PCICU RCA
coronary artery aneurysm cardiopulmonary bypass computed tomography electrocardiogram intravenous immunoglobulin Kawasaki disease left anterior descending left circumflex left main coronary artery left ventricle pediatric cardiac intensive care unit right coronary artery
Figure 2. Coronary angiogram showing left circumflex (LCx) coronary artery aneurysm ( arrow).
Figure 1. Computed tomography of thorax showing large organized clot containing the extravasation of contrast from left circumflex coronary artery aneurysm located in the posterior atrioventricular groove.
adhesions were encountered. During dissection of the area near the left atrial appendage and the LV, a large collection of chocolate-colored thick fluid was seen in the pericardium. This fluid was sent for Gram stain and aerobic, anaerobic, and fungal cultures. A thick-walled saccular aneurysm of the LCX coronary artery was seen in the posterior atrioventricular groove covered with a large layered thrombus (Figure 3). During dissection of intrapericardial adhesions, bleeding was encountered from the ruptured aneurysm and changes in ECG were observed. Cardiopulmonary bypass (CPB) was quickly established with bicaval and ascending aortic cannulation and moderate hypothermia. Del Nido cardioplegia solution was administered to achieve prompt cardiac arrest. After aneurysmectomy of the external wall of the aneurysm, the posterior wall of the LCX was found to be healthy with normal distal and proximal orifices. Fresh homograft saphenous vein patch aneurysmorrhaphy with 8-0 polypropylene continuous suture
Figure 3. Intraoperative pictures showing the left circumflex coronary artery aneurysm (left panel) and the layered thrombus (right panel).
was done (Figure 4). The patient was subsequently weaned from CPB on dobutamine. She was transferred to the pediatric cardiac intensive care unit (PCICU) and extubated after four hours. Heparin infusion (10 u/kg/h) was started on the same day, with a goal of keeping the activated partial thromboplastin time at 2 to 2.5 times the control value. The next day, oral feeds were started and oral aspirin therapy was also started to prevent platelet aggregation. She had an uneventful postoperative course and was discharged from the PCICU on postoperative day 2. Pathologic examination of tissue from the aneurysmal wall showed layered thrombus. The Gram stain and cultures were positive for Staphylococcus aureus and sensitive to clindamycin. She was given intravenous clindamycin for 28 days. She was discharged on oral ampicillin–cloxacillin combination for two weeks, oral prednisolone (tapered over two weeks), and aspirin. At 14-month follow-up, echocardiography showed no
Joshi et al
Figure 4. Saphenous vein patch aneurysmorrhaphy of left circumflex coronary artery.
Figure 5. Postoperative coronary computed tomography (CT) angiography.
regional wall motion abnormality, and CT angiography revealed normal flow in the LCX coronary artery distal to the repair site with no residual aneurysm (Figure 5).
Comments Coronary artery aneurysm is defined as dilatation of a coronary vessel to 1.5 times the diameter of the adjacent normal vessel. They exist in two forms, fusiform and saccular. Fusiform aneurysms are more common, but saccular aneurysms are more likely to rupture, thrombose, or fistulize. The incidence of CAA in the general population is 0.3% to 4.9%. They are more commonly encountered in males (88.2%). Most CAAs are secondary to other conditions like KD, trauma, polyarteritisnodosa, Takayasu disease, syphilis, atherosclerosis, bacterial infection, septic embolism, dissection, scleroderma, metastatic tumor,
113 and iatrogenic insults after angioplasty or stent placement. Congenital aneurysms are rarer and associated with EhlerDanlos syndrome, Marfan syndrome, cyanotic congenital heart disease, and supravalvar aortic stenosis.1 In the pediatric agegroup, KD is the most common cause of coronary aneurysm. In general, CAAs are most commonly located in the proximal and middle right coronary artery (RCA; 40%-87%), followed by the left anterior descending (LAD; 25%-50%), the circumflex coronary artery (24% to 50%), and left main coronary artery (LMCA; 7%).1 Conversely, CAA in KD has a predilection for the RCA (66%), followed by LMCA (42%), the LAD (38%), and the circumflex coronary artery (28%). In KD, occurrence of aneurysms and associated complications may be diminished if treatment with IVIG, steroids, and aspirin is started early after the onset of the symptoms. Coronary artery aneurysm is managed conservatively with periodic follow-up unless the aneurysm is large and therefore, unlikely to regress and may potentially thrombose or rupture. There is no clear indication concerning the optimal timing of surgery during the acute phase. Aneurysmal dilatations of the coronary artery are prone to spasm, thrombosis, embolism, spontaneous dissection, trauma, and erosion into surrounding structures. Infective aneurysms develop when septic emboli lodge in vasa vasorum and cause arterial wall necrosis. Mycotic aneurysms of coronary arteries (both right and left) have been reported in adults in the setting of infective endocarditis.2,3 Coronary artery aneurysm with mycotic pathology in the pediatric population is extremely rare. Rupture of CAA either into myocardium or into the pericardial cavity is associated with high mortality.4 In our patient, exsanguination and tamponade were prevented by thrombus formation and adhesions at the site of rupture. The surgical treatment of a complicated CAA (thrombosed, occluded, or ruptured) is individualized to the findings. Options include thrombectomy, endarterectomy, ligation, or lateral aneurysmorrhaphy, in addition to bypass of the stenotic coronary artery if present. The objectives of management are restoration of distal blood supply along with vessel wall integrity. Patch repair of the defect or coronary artery graft with saphenous vein or artery have all been reported.5 Kitamura et al have reported better long-term patency with internal thoracic artery graft (77.1% + 1.1%, n ¼ 151) compared to saphenous vein graft (46.2% + 6.3%, n ¼ 126).6 Patch repair of CAA has been reported with good longterm outcomes but almost exclusively in adults.5,7,8 In our case, atypical presentation of KD was initially considered in the differential diagnosis based on the presence of a high-grade fever of more than five days’ duration, thrombocytosis, raised inflammatory markers and favorable response with IVIG, steroids, and aspirin therapy. Points disfavoring it were the absence of classical clinical findings of KD (hyperemic tongue and skin peeling), lack of involvement of proximal parts of coronary arteries, and the intraoperative finding of thick chocolate-colored purulent fluid around the aneurysm, which ultimately tested positive for Staphylococcus aureus. Here we present successful outcome of a ruptured mycotic CAA in a small child with this rather unusual presentation and approach.
114
World Journal for Pediatric and Congenital Heart Surgery 6(1)
Declaration of Conflicting Interests
A case report and brief review of the literature. Texas Heart Inst J. 1994;21(3): 231-235. Berrizbeitia LD, Samuels LE. Ruptured right coronary artery aneurysm presenting as a myocardial mass. Ann Thorac Surg. 2002;73(3): 971-973. Pa¨tila¨ T, Virolainen J, Sipponen J, Heikkila¨ L. Resection and patch repair of a large saccular coronary artery aneurysm at the left main bifurcation. Ann Thorac Surg. 2009;87(1): 297-299. Kitamura S, Kameda Y, Seki T, et al. Long-term outcome of myocardial revascularization in patients with Kawasaki coronary artery disease A multicenter cooperative study. J Thorac Cardiovasc Surg. 1994;107(3): 663-674. Kuwaki K, Morishita K, Abe T. Saphenous vein patch angioplasty for a discrete saccular aneurysm of left anterior descending coronary artery. Ann Thorac Cardiovasc Surg. 2000;6(5): 342-344. Moriyama Y, Hisatomi K, Shimokawa S, Taira A, Arima S. Coronary artery aneurysm repaired with saphenous vein patch plasty. Ann Thorac Surg. 1998;65(2): 561-562.
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
4.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
5.
References
6.
1. Befeler B, Aranda MJ, Embi A, Mullin FL, El-Sherif N, Lazzara R. Coronary artery aneurysms: study of the etiology, clinical course and effect on left ventricle function and prognosis. Am J Med. 1977;62(4): 597-607. 2. Omoto T, Saito K, Kashima T, Kume M, Hosaka S, Kimura S. Mycotic aneurysm of the right coronary artery. Asian CardiovascThorac Ann. 2006;14(4): 331-332. 3. Reece IJ, al Tareif H, Tolia J, Saeed FA. Mycotic aneurysm of the left anterior descending coronary artery after aortic endocarditis.
7.
8.
Contained Rupture of Mycotic Aneurysm of the Left Circumflex Coronary Artery in a Child
World Journal for Pediatric and Congenital Heart Surgery 2015, Vol. 6(1) 111-114 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135114551994 pch.sagepub.com
Reena K. Joshi, MD1, Aman Jyoti, MD1, Neeraj Aggarwal, MD2, Mridul Aggarwal, MD2, and Raja Joshi, MCh3
Abstract Coronary artery aneurysm (CAA) is defined as dilatation of a coronary artery segment to a diameter of more than 1.5-fold normal size. Rupture of CAA is a catastrophic event and may result in sudden death or myocardial infarction. We report this unusual case of contained rupture of the left circumflex CAA. Keywords coronary artery surgery, aneurysm (coronary artery, mycotic), pediatric, rupture (coronary artery aneurysm) Submitted April 27, 2014; Accepted August 25, 2014.
Introduction Coronary artery aneurysm (CAA) is a rare, though welldefined, entity that has been described in adults as well as in the pediatric patient population. Coronary artery aneurysm is typically defined as dilatation in the diameter of a coronary artery segment to more than 1.5-fold normal size. Among the etiological factors of CAA, the most common are Kawasaki disease (KD) in children and atherosclerotic coronary artery disease in adults. Rupture of CAA is a catastrophic event and may result in sudden death or myocardial infarction. Because of its unusual presentation and rarity, we report a case of contained rupture of CAA of mycotic etiology in a child.
Case Description An 18-month-old girl weighing 9.3 kg was referred to our facility with a two-week history of fever, diarrhea, and vomiting, despite antibiotic therapy. Cardiomegaly was noted on chest X-ray. On admission, she had a high-grade fever, tachycardia, and extreme irritability. Cough, skin rash, desquamation, or syncope was not observed. Laboratory values included white blood cell count of 19,500/ mL, hematocrit 21%, platelet count 564,000/mL, serum procalcitonin level 2.16ng/mL, C-reactive protein 274 mg/L, and interleukin 6 of 49.84 pg/mL. The blood, urine, and gastric aspirate cultures were sterile. On echocardiography, a 50 60 mm heterogeneous mass surrounded by a large fluid cavity could be seen near the posterior atrioventricular groove. Global and regional cardiac wall motions were normal. Computed tomography (CT) angiography of the thorax
revealed a large, thick-walled, multi-loculated collection surrounding the left ventricle (LV) with an enhancing nodular lesion that appeared to be related to the left circumflex (LCX) coronary artery (Figure 1). Electrocardiogram (ECG) showed no significant abnormalities and biochemical markers of myocardial ischemia were negative. A coronary angiogram confirmed the presence of an isolated saccular aneurysm of the LCX with no coronary artery stenosis (Figure 2). Multivessel involvement was ruled out by CT angiography, fundoscopy, and a vascular Doppler study of carotid and limb arteries. Intravenous immunoglobulin (IVIG), methylprednisolone, and oral aspirin were administered to address the severe degree of inflammation. After 96 hours, marked reduction in the values of inflammatory markers was observed, and gradual resolution of the febrile episodes was also noted. Because of the presence of the coronary artery aneurysm and the multi-loculated collection surrounding the LV, the child was taken to surgery. After induction of anesthesia, midline sternotomy was done and dense fibrous intrapericardial 1 Pediatric Cardiac Anesthesia, Department of Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, New Delhi, India 2 Pediatric Cardiology, Department of Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, New Delhi, India 3 Pediatric Cardiac Surgery, Department of Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, New Delhi, India
Corresponding Author: Reena K. Joshi, Department of Pediatric Cardiac Sciences, Institute of Child Health & Dharamvira Heart Center, Sir Ganga Ram Hospital, New Delhi, India. Email: [email protected]
112
World Journal for Pediatric and Congenital Heart Surgery 6(1)
Abbreviations and Acronyms CAA CPB CT ECG IVIG KD LAD LCX LMCA LV PCICU RCA
coronary artery aneurysm cardiopulmonary bypass computed tomography electrocardiogram intravenous immunoglobulin Kawasaki disease left anterior descending left circumflex left main coronary artery left ventricle pediatric cardiac intensive care unit right coronary artery
Figure 2. Coronary angiogram showing left circumflex (LCx) coronary artery aneurysm ( arrow).
Figure 1. Computed tomography of thorax showing large organized clot containing the extravasation of contrast from left circumflex coronary artery aneurysm located in the posterior atrioventricular groove.
adhesions were encountered. During dissection of the area near the left atrial appendage and the LV, a large collection of chocolate-colored thick fluid was seen in the pericardium. This fluid was sent for Gram stain and aerobic, anaerobic, and fungal cultures. A thick-walled saccular aneurysm of the LCX coronary artery was seen in the posterior atrioventricular groove covered with a large layered thrombus (Figure 3). During dissection of intrapericardial adhesions, bleeding was encountered from the ruptured aneurysm and changes in ECG were observed. Cardiopulmonary bypass (CPB) was quickly established with bicaval and ascending aortic cannulation and moderate hypothermia. Del Nido cardioplegia solution was administered to achieve prompt cardiac arrest. After aneurysmectomy of the external wall of the aneurysm, the posterior wall of the LCX was found to be healthy with normal distal and proximal orifices. Fresh homograft saphenous vein patch aneurysmorrhaphy with 8-0 polypropylene continuous suture
Figure 3. Intraoperative pictures showing the left circumflex coronary artery aneurysm (left panel) and the layered thrombus (right panel).
was done (Figure 4). The patient was subsequently weaned from CPB on dobutamine. She was transferred to the pediatric cardiac intensive care unit (PCICU) and extubated after four hours. Heparin infusion (10 u/kg/h) was started on the same day, with a goal of keeping the activated partial thromboplastin time at 2 to 2.5 times the control value. The next day, oral feeds were started and oral aspirin therapy was also started to prevent platelet aggregation. She had an uneventful postoperative course and was discharged from the PCICU on postoperative day 2. Pathologic examination of tissue from the aneurysmal wall showed layered thrombus. The Gram stain and cultures were positive for Staphylococcus aureus and sensitive to clindamycin. She was given intravenous clindamycin for 28 days. She was discharged on oral ampicillin–cloxacillin combination for two weeks, oral prednisolone (tapered over two weeks), and aspirin. At 14-month follow-up, echocardiography showed no
Joshi et al
Figure 4. Saphenous vein patch aneurysmorrhaphy of left circumflex coronary artery.
Figure 5. Postoperative coronary computed tomography (CT) angiography.
regional wall motion abnormality, and CT angiography revealed normal flow in the LCX coronary artery distal to the repair site with no residual aneurysm (Figure 5).
Comments Coronary artery aneurysm is defined as dilatation of a coronary vessel to 1.5 times the diameter of the adjacent normal vessel. They exist in two forms, fusiform and saccular. Fusiform aneurysms are more common, but saccular aneurysms are more likely to rupture, thrombose, or fistulize. The incidence of CAA in the general population is 0.3% to 4.9%. They are more commonly encountered in males (88.2%). Most CAAs are secondary to other conditions like KD, trauma, polyarteritisnodosa, Takayasu disease, syphilis, atherosclerosis, bacterial infection, septic embolism, dissection, scleroderma, metastatic tumor,
113 and iatrogenic insults after angioplasty or stent placement. Congenital aneurysms are rarer and associated with EhlerDanlos syndrome, Marfan syndrome, cyanotic congenital heart disease, and supravalvar aortic stenosis.1 In the pediatric agegroup, KD is the most common cause of coronary aneurysm. In general, CAAs are most commonly located in the proximal and middle right coronary artery (RCA; 40%-87%), followed by the left anterior descending (LAD; 25%-50%), the circumflex coronary artery (24% to 50%), and left main coronary artery (LMCA; 7%).1 Conversely, CAA in KD has a predilection for the RCA (66%), followed by LMCA (42%), the LAD (38%), and the circumflex coronary artery (28%). In KD, occurrence of aneurysms and associated complications may be diminished if treatment with IVIG, steroids, and aspirin is started early after the onset of the symptoms. Coronary artery aneurysm is managed conservatively with periodic follow-up unless the aneurysm is large and therefore, unlikely to regress and may potentially thrombose or rupture. There is no clear indication concerning the optimal timing of surgery during the acute phase. Aneurysmal dilatations of the coronary artery are prone to spasm, thrombosis, embolism, spontaneous dissection, trauma, and erosion into surrounding structures. Infective aneurysms develop when septic emboli lodge in vasa vasorum and cause arterial wall necrosis. Mycotic aneurysms of coronary arteries (both right and left) have been reported in adults in the setting of infective endocarditis.2,3 Coronary artery aneurysm with mycotic pathology in the pediatric population is extremely rare. Rupture of CAA either into myocardium or into the pericardial cavity is associated with high mortality.4 In our patient, exsanguination and tamponade were prevented by thrombus formation and adhesions at the site of rupture. The surgical treatment of a complicated CAA (thrombosed, occluded, or ruptured) is individualized to the findings. Options include thrombectomy, endarterectomy, ligation, or lateral aneurysmorrhaphy, in addition to bypass of the stenotic coronary artery if present. The objectives of management are restoration of distal blood supply along with vessel wall integrity. Patch repair of the defect or coronary artery graft with saphenous vein or artery have all been reported.5 Kitamura et al have reported better long-term patency with internal thoracic artery graft (77.1% + 1.1%, n ¼ 151) compared to saphenous vein graft (46.2% + 6.3%, n ¼ 126).6 Patch repair of CAA has been reported with good longterm outcomes but almost exclusively in adults.5,7,8 In our case, atypical presentation of KD was initially considered in the differential diagnosis based on the presence of a high-grade fever of more than five days’ duration, thrombocytosis, raised inflammatory markers and favorable response with IVIG, steroids, and aspirin therapy. Points disfavoring it were the absence of classical clinical findings of KD (hyperemic tongue and skin peeling), lack of involvement of proximal parts of coronary arteries, and the intraoperative finding of thick chocolate-colored purulent fluid around the aneurysm, which ultimately tested positive for Staphylococcus aureus. Here we present successful outcome of a ruptured mycotic CAA in a small child with this rather unusual presentation and approach.
114
World Journal for Pediatric and Congenital Heart Surgery 6(1)
Declaration of Conflicting Interests
A case report and brief review of the literature. Texas Heart Inst J. 1994;21(3): 231-235. Berrizbeitia LD, Samuels LE. Ruptured right coronary artery aneurysm presenting as a myocardial mass. Ann Thorac Surg. 2002;73(3): 971-973. Pa¨tila¨ T, Virolainen J, Sipponen J, Heikkila¨ L. Resection and patch repair of a large saccular coronary artery aneurysm at the left main bifurcation. Ann Thorac Surg. 2009;87(1): 297-299. Kitamura S, Kameda Y, Seki T, et al. Long-term outcome of myocardial revascularization in patients with Kawasaki coronary artery disease A multicenter cooperative study. J Thorac Cardiovasc Surg. 1994;107(3): 663-674. Kuwaki K, Morishita K, Abe T. Saphenous vein patch angioplasty for a discrete saccular aneurysm of left anterior descending coronary artery. Ann Thorac Cardiovasc Surg. 2000;6(5): 342-344. Moriyama Y, Hisatomi K, Shimokawa S, Taira A, Arima S. Coronary artery aneurysm repaired with saphenous vein patch plasty. Ann Thorac Surg. 1998;65(2): 561-562.
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
4.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
5.
References
6.
1. Befeler B, Aranda MJ, Embi A, Mullin FL, El-Sherif N, Lazzara R. Coronary artery aneurysms: study of the etiology, clinical course and effect on left ventricle function and prognosis. Am J Med. 1977;62(4): 597-607. 2. Omoto T, Saito K, Kashima T, Kume M, Hosaka S, Kimura S. Mycotic aneurysm of the right coronary artery. Asian CardiovascThorac Ann. 2006;14(4): 331-332. 3. Reece IJ, al Tareif H, Tolia J, Saeed FA. Mycotic aneurysm of the left anterior descending coronary artery after aortic endocarditis.
7.
8.
