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Atypical Kawasaki Disease and Haemophagocytic Lymphohistiocytosis: An Unusual Association with Severe Consequences
Abstracts
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middle and posterior cerebral arteries (MCA and PCA respectively) and stiffness of cerebral vessels, as determined by pulsatility and resistive indices. Cerebrovascular responsiveness (CVR) to a 3-min hypercapnic challenge (95%O2/5%CO2) in the MCA was calculated as a per cent change from the peak velocity achieved during hypercapnia and basal velocity. Results: Both groups were normotensive; pulse pressure was 17% higher in the patient group (P=0.013) compared with controls despite similar systolic blood pressure and arterial compliance. In both the MCA and PCA, the patient group exhibited significantly increased stiffness of the intracranial vessels (16-30% greater in the MCA and 16-37% greater in the PCA). Additionally, the CVR to hypercapnia in the MCA had a relative impairment of 35% in the patient group. Conclusion: Intracranial vascular function is impaired in normotensive patients with a history of CoA. Further investigation in warranted to assess whether these abnormalities in cerebral haemodynamics predispose to stroke and cerebral aneurysm formation. http://dx.doi.org/10.1016/j.hlc.2016.06.722 721 Atypical Kawasaki Disease and Haemophagocytic Lymphohistiocytosis: An Unusual Association with Severe Consequences Z. Wake ∗ , J. Ramsay, P. Walsh, C. Murray Princess Margaret Hospital for Children, Perth, Australia Introduction: Kawasaki disease is a medium-vessel vasculitis most commonly affecting pre-school children. Haemophagocytic lymphohistiocytosis (HLH) is a rare macrophage activation syndrome. Primary HLH is familial, whilst acquired HLH follows significant immunological activation. We describe a case of severe atypical Kawasaki disease complicated by HLH, widespread arterial aneurysms and severe ectasia of the coronary arteries. Case report: A 9-year-old boy presented with fever, headache and acute confusion. Clinical examination revealed a macular rash, peripheral oedema, inflamed mucosa with labial desquamation and fluctuating consciousness consistent with acute encephalopathy. Blood investigations revealed poor renal and hepatic function, significantly elevated inflammatory markers, anaemia, thrombocytopaenia and leukocytosis. Bone marrow biopsy was suggestive of HLH with phagocytosis of erythrocytes. Atypical Kawasaki disease complicated by HLH was suspected and intravenous immunoglobulin (IVIG) administered. Normal coronary arteries were demonstrated on initial echocardiogram. Within days, diffuse ectasia of the right coronary artery (RCA), left coronary artery (LCA) and proximal left anterior descending artery (LAD) was apparent (Z scores 4.93, 7.80 and 7.94 respectively). Further IVIG was given with pulse intravenous methylprednisolone. Serial echocardiograms demonstrated progressive coronary artery dilatation without stenosis to maximum RCA 6.4 mm (Z score 8.59), LCA10.3 mm (Z score 14.49) and LAD 10.2 mm (Z score 19.41). Anticoagulation therapy was escalated from daily Aspirin to Warfarin. CT
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angiogram demonstrated widespread medium vessel disease with fusiform aneurysms of the vertebral, celiac, proximal mesenteric and common iliac arteries. Conclusion: Giant coronary aneurysms are a rare but serious complication of Kawasaki disease, requiring long-term anticoagulation to prevent myocardial hypoperfusion. A high index of clinical suspicion is required to detect coronary artery changes in atypical presentations. http://dx.doi.org/10.1016/j.hlc.2016.06.723 722 Congenital Heart Disease (CHD) Patients Attending Cardiac Rehabilitation – a Comparative Study Between Simple and > Moderate CHD Complexity R. Zecchin 1,2,∗ , J. Baihn 1 , Y. Chai 1 , K. Haeusler 1 , J. Hungerford 1 , G. Lindsay 1 , M. Pettitt 1 , M. Rull 1 , E. Siachico 1 , S. Te Whaiti 1 , J. Thelander 1 , T. Vail 1 , K. McNulty 1 , P. Choudhary 1,2 , D. Tanous 1,2 , C. Dickson 1 , R. Denniss 1,2 1 Western
Sydney LHD, Sydney, Australia West Adult Congenital Heart Centre, Sydney, Australia
2 Sydney
Background: Patients referred with congenital heart disease (CHD) to cardiac rehabilitation (CR) usually have simple (SCHD) complexity defects. Little is known if patients with moderate to severe CHD (MSCHD) benefit from a traditional CR program compared to those with SCHD. Aim: To compare CR outcomes between SCHD and MSCHD patients. Methods: We reviewed prospective clinical data from January 1994 to December 2015 for CHD patient completing CR. This included demographics, severity of CHD, cardiac risk factors, functional capacity, quality of life (QOL), and mortality. Results: This study observed 46 patients (62%) with SCHD (age 47±17 years; 62% male) and 28 patients (38%), who attended on 33 occasions (age 37±12 years; 53% male) with MSCHD. At baseline assessment, there was no significant difference in BMI, functional capacity (SCHD 6MWT n=9, 407±89 metres; EST n= 37, 8±3 METS: MSCHD 6MWT n=16, 392±105 metres; EST n=17, 8.4±3 METS), rates of hyperlipidaemia, hypertension and/or smoking, and use of most cardiac medications. Differences at baseline between the 2 groups were diabetes rate (SCHD 11%: MSCHD 3%, p=0.05), use of oral nitrates (SCHD 0%: MSCHD 12%, p=0.01) and decreased physical-functioning (p=0.05) in the SCHD group on the SF-36. Both groups increased functional capacity using 6MWT post CR (SCHD 15% p<0.001; MSCHD 21%; p=0.05) and/or EST (SCHD 40%; p<0.01: MSCHD 35%, p=0.01). At post program assessment, there was no difference between the 2 groups in QOL. There was no morbidity and/or mortality associated with CR. Conclusion: Patients with SCHD and MSCHD can achieve similar benefits from CR. http://dx.doi.org/10.1016/j.hlc.2016.06.724
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middle and posterior cerebral arteries (MCA and PCA respectively) and stiffness of cerebral vessels, as determined by pulsatility and resistive indices. Cerebrovascular responsiveness (CVR) to a 3-min hypercapnic challenge (95%O2/5%CO2) in the MCA was calculated as a per cent change from the peak velocity achieved during hypercapnia and basal velocity. Results: Both groups were normotensive; pulse pressure was 17% higher in the patient group (P=0.013) compared with controls despite similar systolic blood pressure and arterial compliance. In both the MCA and PCA, the patient group exhibited significantly increased stiffness of the intracranial vessels (16-30% greater in the MCA and 16-37% greater in the PCA). Additionally, the CVR to hypercapnia in the MCA had a relative impairment of 35% in the patient group. Conclusion: Intracranial vascular function is impaired in normotensive patients with a history of CoA. Further investigation in warranted to assess whether these abnormalities in cerebral haemodynamics predispose to stroke and cerebral aneurysm formation. http://dx.doi.org/10.1016/j.hlc.2016.06.722 721 Atypical Kawasaki Disease and Haemophagocytic Lymphohistiocytosis: An Unusual Association with Severe Consequences Z. Wake ∗ , J. Ramsay, P. Walsh, C. Murray Princess Margaret Hospital for Children, Perth, Australia Introduction: Kawasaki disease is a medium-vessel vasculitis most commonly affecting pre-school children. Haemophagocytic lymphohistiocytosis (HLH) is a rare macrophage activation syndrome. Primary HLH is familial, whilst acquired HLH follows significant immunological activation. We describe a case of severe atypical Kawasaki disease complicated by HLH, widespread arterial aneurysms and severe ectasia of the coronary arteries. Case report: A 9-year-old boy presented with fever, headache and acute confusion. Clinical examination revealed a macular rash, peripheral oedema, inflamed mucosa with labial desquamation and fluctuating consciousness consistent with acute encephalopathy. Blood investigations revealed poor renal and hepatic function, significantly elevated inflammatory markers, anaemia, thrombocytopaenia and leukocytosis. Bone marrow biopsy was suggestive of HLH with phagocytosis of erythrocytes. Atypical Kawasaki disease complicated by HLH was suspected and intravenous immunoglobulin (IVIG) administered. Normal coronary arteries were demonstrated on initial echocardiogram. Within days, diffuse ectasia of the right coronary artery (RCA), left coronary artery (LCA) and proximal left anterior descending artery (LAD) was apparent (Z scores 4.93, 7.80 and 7.94 respectively). Further IVIG was given with pulse intravenous methylprednisolone. Serial echocardiograms demonstrated progressive coronary artery dilatation without stenosis to maximum RCA 6.4 mm (Z score 8.59), LCA10.3 mm (Z score 14.49) and LAD 10.2 mm (Z score 19.41). Anticoagulation therapy was escalated from daily Aspirin to Warfarin. CT
.. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. ..
angiogram demonstrated widespread medium vessel disease with fusiform aneurysms of the vertebral, celiac, proximal mesenteric and common iliac arteries. Conclusion: Giant coronary aneurysms are a rare but serious complication of Kawasaki disease, requiring long-term anticoagulation to prevent myocardial hypoperfusion. A high index of clinical suspicion is required to detect coronary artery changes in atypical presentations. http://dx.doi.org/10.1016/j.hlc.2016.06.723 722 Congenital Heart Disease (CHD) Patients Attending Cardiac Rehabilitation – a Comparative Study Between Simple and > Moderate CHD Complexity R. Zecchin 1,2,∗ , J. Baihn 1 , Y. Chai 1 , K. Haeusler 1 , J. Hungerford 1 , G. Lindsay 1 , M. Pettitt 1 , M. Rull 1 , E. Siachico 1 , S. Te Whaiti 1 , J. Thelander 1 , T. Vail 1 , K. McNulty 1 , P. Choudhary 1,2 , D. Tanous 1,2 , C. Dickson 1 , R. Denniss 1,2 1 Western
Sydney LHD, Sydney, Australia West Adult Congenital Heart Centre, Sydney, Australia
2 Sydney
Background: Patients referred with congenital heart disease (CHD) to cardiac rehabilitation (CR) usually have simple (SCHD) complexity defects. Little is known if patients with moderate to severe CHD (MSCHD) benefit from a traditional CR program compared to those with SCHD. Aim: To compare CR outcomes between SCHD and MSCHD patients. Methods: We reviewed prospective clinical data from January 1994 to December 2015 for CHD patient completing CR. This included demographics, severity of CHD, cardiac risk factors, functional capacity, quality of life (QOL), and mortality. Results: This study observed 46 patients (62%) with SCHD (age 47±17 years; 62% male) and 28 patients (38%), who attended on 33 occasions (age 37±12 years; 53% male) with MSCHD. At baseline assessment, there was no significant difference in BMI, functional capacity (SCHD 6MWT n=9, 407±89 metres; EST n= 37, 8±3 METS: MSCHD 6MWT n=16, 392±105 metres; EST n=17, 8.4±3 METS), rates of hyperlipidaemia, hypertension and/or smoking, and use of most cardiac medications. Differences at baseline between the 2 groups were diabetes rate (SCHD 11%: MSCHD 3%, p=0.05), use of oral nitrates (SCHD 0%: MSCHD 12%, p=0.01) and decreased physical-functioning (p=0.05) in the SCHD group on the SF-36. Both groups increased functional capacity using 6MWT post CR (SCHD 15% p<0.001; MSCHD 21%; p=0.05) and/or EST (SCHD 40%; p<0.01: MSCHD 35%, p=0.01). At post program assessment, there was no difference between the 2 groups in QOL. There was no morbidity and/or mortality associated with CR. Conclusion: Patients with SCHD and MSCHD can achieve similar benefits from CR. http://dx.doi.org/10.1016/j.hlc.2016.06.724