- Email: [email protected]
Kawasaki’s disease: morphology of coronary artery aneurysms
CORRESPONDENCE
187
myocardial infarction as a consequence of Kawasaki’s disease in a 29-year-old female. The patient was referred to our hospital one hour after the onset of symptoms of acute myocardial infarction. Abnormal Q waves appeared on electrocardiograph (ECG). The patient had no history of coronary artery disease and she had been well until just before the myocardial infarction. Coronary angiography showed multiple proximal obstructions and aneurysmal dilations of the left main (LMCA) and right coronary arteries (RCA). A balloon angioplasty was performed as a life saving procedure. Cardiogenic shock developed after the surgical procedure of myocardial revascularisation and one day after admission the patient underwent heart transplantation. At present, the patient is alive and in good clinical condition. On gross examination, the explanted heart showed multiple coronary aneurysms involving LMCA and RCA (Fig. 1, 2) complicated by thrombosis of the LMCA aneurysm and massive myocardial infarction (Fig. 3). No atherosclerotic change was noted and the coronary ostia were found to be patent and in normal position.
Fig. 1 Right upper view of the cardiectomy specimen, showing in detail the aneurysm of the left main coronary artery (LMCA) and two smaller aneurysms of the right coronary artery (RCA; A1, A2). The right ventricle (RV) has been sectioned.
Kawasaki’s disease: morphology of coronary artery aneurysms Sir, The aetiology of Kawasaki’s disease (KD) remains unknown, although bacterial toxins and autoimmunity may be involved in its pathogenesis. Cardiac sequelae from KD involvement are the most important manifestations of KD, and coronary artery abnormalities include ectasia, non-giant and giant aneurysms, with ventricular dysfunction.1 We describe a case of coronary aneurysms and acute
Fig. 2 Upper view of the heart, showing mitral (M) and tricuspid (TR) valve orifices encircled by the dissected coronary artery tree.
188
CORRESPONDENCE
Pathology (2007), 39(1), February
such as revascularisation techniques or heart transplantation, that has a different impact according to the proximal or distal site of the coronary aneurysms. Edgardo Bonacina* Antonio Brucato{ Maurizio Vertemati{ Departments of *Pathology and {Internal Medicine, Niguarda Hospital, Milan, and {Department of Endocrinology, Faculty of Pharmacy, University of Milan, Italy Contact Dr M. Vertemati. Email: [email protected] Fig. 3 Longitudinal section of the left ventricle, showing myocardial infarction (MI) and thrombosis of the left main coronary artery (LMCA) aneurysm.
Histological findings mainly consisted of luminal obstruction by organised thrombus superimposed to intimal fibromuscular thickening of the wall of the aneurysms of involved coronary arteries. Multiple foci of mononuclear inflammatory activity were found in the wall of the aneurysms. These histological findings were consistent with post-arteritis aneurysms in KD. A retrospective study of the clinical records of the patient revealed two previous hospitalisations at the ages of 4 and 9 years for high fever complicated by muco-cutaneous rash with cervical lymphadenopathy and splenomegaly. These clinical findings, in retrospect, were consistent with KD. The mechanism of coronary artery stenosis in KD is not well defined, depending on acute occlusion by massive thrombus formation in the coronary aneurysms or progression of marked intimal thickening often associated with calcification, that is similar to an arteriosclerotic lesion. Coronary artery aneurysms can lead to acute myocardial infarction or sudden death.2,3 Obstruction generally occurs in the proximal portion of the major coronary arteries. In our patient, myocardial revascularisation could not restore adequate ventricular function because of the extent of the acute myocardial infarction, which involved anatomically about 70% of the left ventricular mass and caused the development of cardiogenic shock. Typical myocardial revascularisation damage, consisting of massive interstitial haemorrhage and/or contraction band necrosis of myocites, could be observed histologically. Different surgical treatment, such as aortocoronary bypass surgery and percutaneous transluminal coronary angioplasty have been proposed.4,5 Moreover, cardiac transplantation for severe ischaemic heart disease as a sequela of Kawasaki’s disease has been proposed in the subgroup of patients who are not candidates for revascularisation because of distal coronary stenosis or aneurysms, and/or those with severe irreversible myocardial dysfunction.6 Our patient had selective proximal involvement of coronary arteries, and the indication for heart transplantation was the irreversible myocardial dysfunction. Correlation of anatomical knowledge of coronary substrates with coronary artery involvement can disclose a better understanding of the clinical sequelae in KD. This can be useful for the planning of interventional treatment,
1. Fujiwara H, Hamashima Y. Pathology of the heart in Kawasaki disease. Pediatrics 1978; 61: 100–7. 2. Shulman ST, DeInocencio J, Hirsch R. Kawasaki disease. Pediatr Clin North Am 1995; 42: 1205–22. 3. Kato H, Ichinose E, Kawasaki T. Myocardial infarction in Kawasaki disease. J Pediatr 1986; 108: 923–27. 4. Kitamura S, Kawachi K, Seki T, et al. Bilateral internal mammary artery grafts for coronary artery grafts for coronary artery bypass operations in children. J Thorac Cardiovasc Surg 1990; 99: 708–15. 5. Ino T, Akimoto K, Ohkubo M, et al. Application of percutaneous transluminal coronary angioplasty to coronary arterial stenosis in Kawasaki disease. Circulation 1996; 93: 1709–15. 6. Checchia PA, Pahl E, Shaddy Re, et al. Cardiac transplantation for Kawasaki disease. Pediatrics 1997; 100: 695–9.
DOI: 10.1080/00313020601123920
187
myocardial infarction as a consequence of Kawasaki’s disease in a 29-year-old female. The patient was referred to our hospital one hour after the onset of symptoms of acute myocardial infarction. Abnormal Q waves appeared on electrocardiograph (ECG). The patient had no history of coronary artery disease and she had been well until just before the myocardial infarction. Coronary angiography showed multiple proximal obstructions and aneurysmal dilations of the left main (LMCA) and right coronary arteries (RCA). A balloon angioplasty was performed as a life saving procedure. Cardiogenic shock developed after the surgical procedure of myocardial revascularisation and one day after admission the patient underwent heart transplantation. At present, the patient is alive and in good clinical condition. On gross examination, the explanted heart showed multiple coronary aneurysms involving LMCA and RCA (Fig. 1, 2) complicated by thrombosis of the LMCA aneurysm and massive myocardial infarction (Fig. 3). No atherosclerotic change was noted and the coronary ostia were found to be patent and in normal position.
Fig. 1 Right upper view of the cardiectomy specimen, showing in detail the aneurysm of the left main coronary artery (LMCA) and two smaller aneurysms of the right coronary artery (RCA; A1, A2). The right ventricle (RV) has been sectioned.
Kawasaki’s disease: morphology of coronary artery aneurysms Sir, The aetiology of Kawasaki’s disease (KD) remains unknown, although bacterial toxins and autoimmunity may be involved in its pathogenesis. Cardiac sequelae from KD involvement are the most important manifestations of KD, and coronary artery abnormalities include ectasia, non-giant and giant aneurysms, with ventricular dysfunction.1 We describe a case of coronary aneurysms and acute
Fig. 2 Upper view of the heart, showing mitral (M) and tricuspid (TR) valve orifices encircled by the dissected coronary artery tree.
188
CORRESPONDENCE
Pathology (2007), 39(1), February
such as revascularisation techniques or heart transplantation, that has a different impact according to the proximal or distal site of the coronary aneurysms. Edgardo Bonacina* Antonio Brucato{ Maurizio Vertemati{ Departments of *Pathology and {Internal Medicine, Niguarda Hospital, Milan, and {Department of Endocrinology, Faculty of Pharmacy, University of Milan, Italy Contact Dr M. Vertemati. Email: [email protected] Fig. 3 Longitudinal section of the left ventricle, showing myocardial infarction (MI) and thrombosis of the left main coronary artery (LMCA) aneurysm.
Histological findings mainly consisted of luminal obstruction by organised thrombus superimposed to intimal fibromuscular thickening of the wall of the aneurysms of involved coronary arteries. Multiple foci of mononuclear inflammatory activity were found in the wall of the aneurysms. These histological findings were consistent with post-arteritis aneurysms in KD. A retrospective study of the clinical records of the patient revealed two previous hospitalisations at the ages of 4 and 9 years for high fever complicated by muco-cutaneous rash with cervical lymphadenopathy and splenomegaly. These clinical findings, in retrospect, were consistent with KD. The mechanism of coronary artery stenosis in KD is not well defined, depending on acute occlusion by massive thrombus formation in the coronary aneurysms or progression of marked intimal thickening often associated with calcification, that is similar to an arteriosclerotic lesion. Coronary artery aneurysms can lead to acute myocardial infarction or sudden death.2,3 Obstruction generally occurs in the proximal portion of the major coronary arteries. In our patient, myocardial revascularisation could not restore adequate ventricular function because of the extent of the acute myocardial infarction, which involved anatomically about 70% of the left ventricular mass and caused the development of cardiogenic shock. Typical myocardial revascularisation damage, consisting of massive interstitial haemorrhage and/or contraction band necrosis of myocites, could be observed histologically. Different surgical treatment, such as aortocoronary bypass surgery and percutaneous transluminal coronary angioplasty have been proposed.4,5 Moreover, cardiac transplantation for severe ischaemic heart disease as a sequela of Kawasaki’s disease has been proposed in the subgroup of patients who are not candidates for revascularisation because of distal coronary stenosis or aneurysms, and/or those with severe irreversible myocardial dysfunction.6 Our patient had selective proximal involvement of coronary arteries, and the indication for heart transplantation was the irreversible myocardial dysfunction. Correlation of anatomical knowledge of coronary substrates with coronary artery involvement can disclose a better understanding of the clinical sequelae in KD. This can be useful for the planning of interventional treatment,
1. Fujiwara H, Hamashima Y. Pathology of the heart in Kawasaki disease. Pediatrics 1978; 61: 100–7. 2. Shulman ST, DeInocencio J, Hirsch R. Kawasaki disease. Pediatr Clin North Am 1995; 42: 1205–22. 3. Kato H, Ichinose E, Kawasaki T. Myocardial infarction in Kawasaki disease. J Pediatr 1986; 108: 923–27. 4. Kitamura S, Kawachi K, Seki T, et al. Bilateral internal mammary artery grafts for coronary artery grafts for coronary artery bypass operations in children. J Thorac Cardiovasc Surg 1990; 99: 708–15. 5. Ino T, Akimoto K, Ohkubo M, et al. Application of percutaneous transluminal coronary angioplasty to coronary arterial stenosis in Kawasaki disease. Circulation 1996; 93: 1709–15. 6. Checchia PA, Pahl E, Shaddy Re, et al. Cardiac transplantation for Kawasaki disease. Pediatrics 1997; 100: 695–9.
DOI: 10.1080/00313020601123920