Motofumi Iemura

Kurume University, Kurume, Fukuoka-ken, Japan

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Publications (19)80.42 Total impact

  • Article: Thirty-year follow-up of carnitine supplementation in two siblings with hypertrophic cardiomyopathy caused by primary systemic carnitine deficiency.
    International journal of cardiology 12/2011; 159(1):e14-5. · 7.08 Impact Factor
  • Article: Persistent coronary arterial inflammation in a patient long after the onset of Kawasaki disease.
    International journal of cardiology 11/2011; 154(2):193-4. · 7.08 Impact Factor
  • Article: Long-term prognosis of patients with Kawasaki disease complicated by giant coronary aneurysms: a single-institution experience.
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    ABSTRACT: Some patients with Kawasaki disease develop giant coronary aneurysms and coronary stenosis, leading to ischemic heart disease. The aim of this study was to determine the long-term prognosis of patients with Kawasaki disease with giant aneurysms. From our institutional database, 76 patients (57 men and 19 women) who developed giant aneurysms after January 1, 1972, were identified. Information on patient demographics, catheter and surgical interventions, and most recent status was collected from medical charts and patients' contacts. From these data, we calculated the survival rate and cumulative coronary intervention rate. The average age at onset was 2.9±2.9 years, and the median observational period was 19 years. During this period, 7 patients died and 1 patient underwent a heart transplantation, resulting in 95%, 88%, and 88% survival rates at 10, 20, and 30 years after the onset of KD, respectively. On the other hand, catheter and surgical coronary interventions (median, 1 intervention; range, 1 to 7 interventions) were performed to alleviate coronary ischemia in 46 patients (61%) at 1 month to 21 years (mode at 1 month) after onset, resulting in 28%, 43%, and 59% cumulative coronary intervention rates at 5, 15, and 25 years after onset, respectively. The long-term survival of patients with Kawasaki disease complicated by giant coronary aneurysms is moderately good with multiple catheter and surgical interventions. Further research should focus on the prevention of coronary vascular remodeling and on the indications for and effectiveness of percutaneous and surgical coronary interventions.
    Circulation 05/2011; 123(17):1836-42. · 14.74 Impact Factor
  • Article: Microembolic signals measured by transcranial Doppler during transcatheter closure of atrial septal defect using the Amplatzer septal occluder.
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    ABSTRACT: To determine the frequency and factors associated with increase in microembolic signals during transcatheter closure of atrial septal defect using the Amplatzer septal occluder. During the procedure in 16 patients, we measured microembolic signals using transcranial Doppler. Procedure time was divided into five periods: right cardiac catheterisation; left cardiac catheterisation; left cardiac angiocardiography; sizing and long sheath placement; device placement and release. We compared numbers of microembolic signals among the five periods and identified factors associated with them. Mean size of septal occluder was 16 millimetres in diameter. Total number of microembolic signals was a median of 31.5, ranging from 3 to 113. Microembolic signals in three periods, left cardiac catheterisation; sizing, and long sheath placement; and device placement and release, were not significantly different from one another, but were significantly higher than those in the remaining two periods, right cardiac catheterisation and left cardiac angiocardiography (median was 9 in left cardiac catheterisation; 6 in sizing and long sheath placement; 6.5 in device placement and release, versus 0 in right cardiac catheterisation and 1 in left cardiac angiocardiography, p less than 0.05, respectively). Importantly, the time for device manipulation positively correlated with total number of microembolic signals (r equals 0.77, p less than 0.001), although fluoroscopic time, age, or size of septal occluder did not. Transcatheter closure of atrial septal defect using the Amplatzer septal occluder produces microemboli, especially during device placement. To minimise the risk of systemic embolism, we must decrease the time for device manipulation.
    Cardiology in the Young 04/2011; 21(2):182-6. · 0.76 Impact Factor
  • Article: Increased plasma type B natriuretic peptide in the acute phase of Kawasaki disease.
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    ABSTRACT: The aim of this study was to identify possible factors associated with type-B natriuretic peptide (BNP) production in the acute phase of Kawasaki disease (KD). Subjects were 54 patients with KD (KD group [KDG]) and 18 age-matched controls (control group [CG]). We evaluated left ventricular function using multi-modal echocardiography and determined blood chemistry including BNP, white blood cell count, C-reactive protein (CRP), and interleukin (IL)-6 in the KDG. We compared echocardiographic parameters between the KDG and the CG and determined the correlation between log (BNP) and echocardiographic parameters, white blood cell count, CRP, and IL-6 in the KDG. The KDG showed high BNP (169.6 ± 529.6 pg/ml) despite preserved left ventricular function indicated by no significant difference in left ventricular ejection fraction (72.2 ± 9.2 vs 71.2 ± 7.8 %), z-score of left ventricular diastolic dimension (0.8 ± 1.3 vs 0.9 ± 0.8 SD), and Tei index (0.29 ± 0.09 vs 0.30 ± 0.06) between the KDG and the CG. However, left ventricular ejection fraction (r =-0.44, P= .001) and left ventricular end-diastolic dimension (r = 0.30, P < .05) significantly correlated with log (BNP). On the other hand, the KDG showed high CRP (89.7 ± 55.6 mg/l) and high IL-6 (242.2 ± 243.5 pg/ml), and CRP (r = 0.60, P < 0.0001) and IL-6 (r = 0.78, P < 0.0001) significantly correlated with log (BNP). Multiple stepwise regression analysis identified IL-6 (r = 0.77, P < 0.0001) most significantly correlated with log (BNP). In acute KD, BNP significantly increases, despite well-preserved global left ventricular function, and inflammation might be associated with this increased BNP.
    Pediatrics International 03/2011; 53(5):736-41. · 0.63 Impact Factor
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    Article: Entrapment of the left coronary artery ostium by the aortic valve leaflet, promoting myocardial ischemia.
    Journal of the American College of Cardiology 11/2010; 56(20):1679. · 14.16 Impact Factor
  • Article: Health-related quality of life in adolescents and young adults with a history of Kawasaki disease.
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    ABSTRACT: To investigate the health-related quality of life (HRQOL) in adolescents and young adults with Kawasaki disease (KD). We conducted a cross-sectional observational study. Patients were eligible for this study when they were > or =16 years of age and had received a diagnosis of KD > or =5 years before the start of this study. The patients were divided in 3 groups according to their coronary status: normal, aneurysms, and giant aneurysms/ischemia. A self-administered questionnaire (Medical Outcome Study Short Form 36) was mailed to the patients to evaluate the HRQOL. Of 624 total questionnaires mailed, 377 were delivered, and 250 were returned. The response rates of the normal, aneurysm, and giant aneurysms/ischemia groups were 33%, 62%, and 72% of all eligible patients, respectively. All subscale scoress of HRQOL, except vitality and role-emotional (limitations in the usual role activities because of emotional problems) in patients with KD were significantly higher than scores from the national norms. The HRQOL of adolescents and young adults with a history of KD is favorable. However, long-term follow-up is necessary, because the general health perceptions of older patients tended to be lower.
    The Journal of pediatrics 12/2009; 156(3):439-43. · 4.02 Impact Factor
  • Article: Progressive left coronary stenosis after rotablator ablation appreciated by Doppler echocardiography.
    Shintaro Kishimoto, Kenji Suda, Motofumi Iemura
    Pediatric Cardiology 09/2009; 30(8):1190-1. · 1.30 Impact Factor
  • Article: Impact of intravenous immunoglobulin infusion on longitudinal left ventricular performance in patients with acute Kawasaki disease of usual course.
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    ABSTRACT: To determine the acute change in cardiac performance after intravenous immunoglobulin infusion (IVIG) in patients with acute Kawasaki disease (KD). Subjects were 33 patients with KD who were treated with IVIG 2 g/kg and recovered without coronary artery lesion and 27 controls. Subjects underwent combined two-dimensional, Doppler, and tissue Doppler echocardiographic (TDI) studies. In KD, these echocardiographic studies were performed before IVIG, 48 h after IVIG, and in convalescence. Echocardiographic variables were compared between KD and controls as well as among 3 time points in KD. Before IVIG, KD showed significantly higher peak aortic velocity and shorter aortic ejection time as results of tachycardia and significantly lower E' (p<0.04) but significantly higher E/E' (p<0.02). After IVIG, patients with KD became afebrile and showed significantly lower TDI indices such as S', E', and, A' and isovolumic acceleration (IVA) (163+/-56 vs. 208+/-70 cm/s(2), p<0.01) with higher TDI-derived Tei index (0.50+/-0.10 vs. 0.44+/-0.06, p<0.02) than controls. These differences tended to disappear in convalescence. In analysis of repeated measurements, except for hemodynamic changes associated with tachycardia, S' (7.9+/-1.3 vs. 7.0+/-1.1 vs. 7.4+/-0.9 cm/s, p<0.001), IVA (227+/-72 vs. 163+/-56 vs. 180+/-63, p<0.05), and A' (7.7+/-3.0 vs. 5.6+/-1.3 vs. 6.7+/-2.3 cm/s, p<0.001) were significantly different among these time points. In patients with acute KD with usual course, IVIG induced transient sub-clinical longitudinal left ventricular dysfunction.
    Journal of Cardiology 08/2009; 54(1):45-51. · 1.28 Impact Factor
  • Article: Portal-systemic encephalopathy after Fontan-type operation in patient with polysplenia syndrome.
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    ABSTRACT: An 18-year-old patient, who had polysplenia and single ventricle, presented with altered mental status 9 years after a Fontan-type operation and pacemaker implantation. He underwent replacement of common atrioventricular valve and aortic valve plasty 1 year previously and has been placed on multiple medications including beta-blocker for his poor ventricular function. Blood chemistry revealed hyperammonemia of 2420 microg/l as a cause of this altered mental status disturbance. Superior mesenteric arteriography revealed large portal-systemic shunts in venous phase as a cause of hyperammonemia. To control blood ammonia level, we placed him on low protein diet, oral polymixin B, and lactulose instead of closing shunt with device. This case illustrates that portal-systemic shunt may result in hyperammonemia leading to altered mental status long after a Fontan-type operation.
    European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 05/2009; 35(6):1083-5. · 2.40 Impact Factor
  • Article: Warfarin therapy for giant aneurysm prevents myocardial infarction in Kawasaki disease.
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    ABSTRACT: We retrospectively investigated the effect of warfarin therapy in improving the clinical outcome of Kawasaki disease (KD) patients with giant coronary aneurysms (GAs). We followed 2350 KD patients from 1973 to 2004. The GAs (> or =8 mm in diameter) were diagnosed by coronary angiography. Sixty-eight patients (54 males and 14 females) were retrospectively studied. Patients were divided into two groups. One group consisted of 19 patients with 33 branches treated with a combination of low-dose aspirin and warfarin (target international normalized ratio: 1.5 - 2.5 IU). The second group consisted of 49 patients with 102 branches treated with low-dose aspirin without warfarin. The incidence of myocardial infarction was significantly less in the combination therapy group than in patients treated with aspirin without warfarin (1 patient vs. 16 patients, p < 0.05). Sudden death occurred in seven patients taking aspirin without warfarin, but none of the patients receiving warfarin died. No major bleeding events occurred in either group. Combination therapy of warfarin with aspirin is associated with a decreased risk of myocardial infarction in KD patients with GAs.
    Pediatric Cardiology 04/2008; 29(2):398-401. · 1.30 Impact Factor
  • Article: Effect of revision of Japanese diagnostic criterion for fever in Kawasaki disease on treatment and cardiovascular outcome.
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    ABSTRACT: The aim of this study was to investigate the effect of a revision of the fever criterion for initial intravenous immunoglobulin (IVIG) treatment, and cardiovascular sequelae, in the new Japanese diagnostic criteria for Kawasaki disease. Patients who were reported in the 16th and 18th nationwide surveys in Japan were analyzed. New criteria group comprised patients who received the diagnosis of Kawasaki disease in the 18th nationwide survey (n=18,789). Old criteria group was comprised patients who received their diagnosis in the 16(th) nationwide survey (n=15,017). The difference between the new and old criteria for complete cases was only 1%. The proportion of patients who were treated with IVIG within 4 days of illness onset in the new criteria group was significantly lower than in the old criteria group (27.7% vs 30.7%). Multivariate logistic regression analysis identified criteria sex, age, recurrence, diagnosis, last day of initial IVIG and additional IVIG treatment as significant independent factors for cardiovascular sequelae. The guideline revision improves diagnostic sensitivity somewhat, but reflects the clinical reality of the disease that approximately 30% of patients are treated with IVIG within 4 days of illness onset.
    Circulation Journal 12/2007; 71(11):1791-3. · 3.77 Impact Factor
  • Article: [New diagnostic methods and modalities for congenital heart disease].
    Motofumi Iemura
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    ABSTRACT: Ultrasound imaging of cardiovascular structures has progressed rapidly from standard surface techniques to real-time 3-dimensional echocardiography and intracardiac echocardiography. Especially, 3-dimensional echocardiography is having a significant impact on the evaluation of congenital heart diseases (CHD). However, the present system of prenatal screening for CHD in Japan is not as efficient as it is in some Western countries. For this reason, the number of patient referrals for fetal echocardiography and the fetal diagnosis of CHD increased year after year. Gestational age at referral and at the diagnosis of CHD has increased rapidly in recent years. To improve the patient's prognosis, we should form more simple prenatal primary screening programs for CHD. In addition, we require the progression of the diagnostic modalities and familiarize these new diagnostic methods and modalities.
    Kyobu geka. The Japanese journal of thoracic surgery 08/2007; 60(8 Suppl):667-73.
  • Article: Prediction of resistance to intravenous immunoglobulin treatment in patients with Kawasaki disease.
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    ABSTRACT: The objective of this study was to find the predictors and generate a prediction score of resistance to intravenous immunoglobulin (IVIG) in patients with Kawasaki disease (KD). Patients diagnosed as having KD were sampled when they received initial high-dose IVIG treatment (2 g/kg dose) within 9 days of illness (n = 320). These patients were divided into 2 groups: the resistance (n = 41) and the responder (n = 279). The following data were obtained and compared between resistance and responder: age, sex, illness days at initial treatment, and laboratory data. Multivariate logistic regression analysis identified age, illness days, platelet count, alanine aminotransferase (ALT), and C-reactive protein (CRP) as significant predictors for resistance to IVIG. We generated prediction score assigning 1 point for (1) infants less than 6 months old, (2) before 4 days of illness, (3) platelet count <or= 30 x 10(10)/L, (4) CRP >or= 8 mg/dL, as well as 2 points for (5) ALT >or= 80 IU/L. Using a cut-off point of 3 and more with this prediction score, we could identify the IVIG-resistant group with 78% sensitivity and 76% specificity. Resistance to IVIG treatment can be predicted using age, illness days, platelet count, ALT, and CRP. Randomized, multicenter clinical trials are necessary to create a new strategy to treat these high-risk patients.
    Journal of Pediatrics 08/2006; 149(2):237-40. · 4.11 Impact Factor
  • Article: Transthoracic echocardiography-assisted valve perforation in pulmonary atresia.
    Pediatrics International 01/2005; 46(6):748-50. · 0.63 Impact Factor
  • Article: Sequential follow-up results of catheter intervention for coronary artery lesions after Kawasaki disease: quantitative coronary artery angiography and intravascular ultrasound imaging study.
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    ABSTRACT: The purpose of this study was to assess the sequential follow-up results of catheter intervention in Kawasaki disease by use of quantitative coronary angiography (QCA) and intravascular ultrasound imaging. Catheter intervention was performed on 23 stenotic lesions in 22 patients (aged 2 to 24 years). Percutaneous balloon angioplasty (PBA) was performed in 4 patients, stent implantation in 7, percutaneous transluminal coronary rotational ablation (PTCRA) in 10, and a combination of PTCRA with stent implantation in 2. A total of 21 lesions (91%) were successfully dilated by catheter intervention without major or minor complications. One patient immediately underwent coronary artery bypass grafting (CABG) surgery because stent implantation failed to resolve his lesion. At 4 to 6 months after catheter intervention, 2 restenotic lesions (9%) were detected by QCA in 2 patients who had undergone PBA, and these patients subsequently underwent CABG surgery. In 6 months to 3 years after catheter intervention, no patients showed evidence of ischemic findings. At 3 to 4 years after catheter intervention, QCA and intravascular ultrasound studies were performed on 15 lesions in 14 patients. Two restenotic lesions (13%) were detected by QCA in 2 patients. One of the 2 had stent implantation and underwent CABG surgery, and the other had undergone PTCRA and underwent re-PTCRA. Thirteen patients demonstrated no ischemic findings at 3 to 8 years after catheter intervention. Catheter intervention for Kawasaki disease can be accomplished and can be effective in the short term, but the long-term efficacy should be verified by further study.
    Circulation 07/2002; 105(25):3004-10. · 14.74 Impact Factor
  • Article: Re‐treatment for immune globulin‐resistant Kawasaki disease: A comparative study of additional immune globulin and steroid pulse therapy
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    ABSTRACT: Abstract Background: We compared the efficacy and safety of additional intravenous immune globulin (IVIG) therapy with steroid pulse therapy in patients with IVIG-resistant Kawasaki disease.Methods: Two-hundred and sixty-two consecutive patients had been treated with a single dose of IVIG (2 g/kg) and aspirin (30 mg/kg per day). Thirty-five patients (13.4%) were not clinical responders to the initial IVIG treatment. They received an additional IVIG treatment (1 g/kg) within 48 h after the initial treatment. Seventeen patients (6.5%) did not respond to the additional IVIG treatment. We randomly divided these patients into two groups: group 1 consisted of eight patients who were treated with a single additional dose of IVIG (1 g/kg), while group 2 consisted of nine patients who were treated with steroid pulse therapy.Results: The IVIG-resistant patients had a high incidence of coronary artery lesions (CAL; 48.6%). Five patients (62.5%) in group 1 had CAL, including two patients who each had a giant aneurysm and three patients who each had a small aneurysm. Seven patients (77.8%) in group 2 had CAL, including two patients who each had a giant aneurysm, two patients who each had a small coronary aneurysm and three patients who each showed transient dilatation during steroid pulse therapy. There was no significant difference in the incidence of CAL between the two groups. The duration of high fever in group 2 (1.4±0.7 days) was significantly shorter than in group 1 (4.8±3.4 days; P<0.05). The medical costs for the treatment of patients in group 2 (¥113 012±22 084) were significantly lower than those for group 1 (¥144 194±12 914; P<0.05).Conclusions: Steroid pulse therapy may be useful in the treatment of patients with IVIG-resistant Kawasaki disease who experience prolonged fever. However, transient dilatation of the coronary artery is observed during steroid pulse therapy, so careful echocardiographic examination should be performed for those patients receiving steroid pulse therapy for the sake of early detection of coronary artery abnormalities.
    Pediatrics International 06/2001; 43(3):211 - 217. · 0.63 Impact Factor
  • Article: Simultaneous Double or Triple Coil Technique for Closure of Moderate Sized (± 3.0 mm) Patent Ductus Arteriosus
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    ABSTRACT: One important complication of coil occlusion of patent ductus arteriosus using the conventional Gianturco coil is migration of coils into peripheral vessels. Especially in patients having relatively larger size ductus, the risk for such complication could be increased. In this regard, a detachable coil may have some technical benefits in performing coil occlusion and reducing the incidence of complications such as migration of coil. Based on our clinical experiences, we describe the clinical efficacy of a simultaneous double or triple coil occlusion technique using the Cook detachable coil system to close the ductus arteriosus, especially in patients whose ductus diameter more than 3.0 mm. (J Interven Cardial 2001;14:91–96)
    Journal of Interventional Cardiology 01/2001; 14(1):91 - 96. · 1.18 Impact Factor
  • Article: Selective high dose gamma‐globulin treatment in Kawasaki disease: Assessment of clinical aspects and cost effectiveness
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    ABSTRACT: Background: High-dose intravenous gamma-globulin (IVGG) plus aspirin (ASA) treatment is effective in preventing coronary artery complications in acute Kawasaki disease (KD). However, gamma-globulin is very expensive, especially in Japan. Furthermore the indication for IVGG treatment and the optimal dose of gamma-globulin remain controversial.Objectives: To examine these two issues, we used Harada’s scoring system to investigate whether a single 2 g/kg dose therapy has any advantage over the 5 day 400 mg/kg per day therapy.Methods: We studied 203 patients with KD who had no coronary artery complications on admission. Of these, 145 patients scored 4 or more on Harada score within the first 9 days of illness and were treated with IVGG treatment. Using a random number table, 72 patients were selected to receive a single 2 g/kg dose (2 g group), while the remaining 73 patients were treated with 400 mg/kg per day for 5 consecutive days (400 mg group). Those who had a Harada score of three or less received no IVGG (non-IVGG group) treatment (58 patients).Results: The incidence rate of coronary artery complications in the 2 g group was significantly lower than in the 400 mg group. The duration of high fever, positive duration of C-reactive protein and the number of hospital days in the 2 g group were each significantly shorter than in the 400 mg group. The total medical expense in the 2 g group was significantly lower than in the 400 mg group. There were no coronary artery complications in the non-IVGG group.Conclusions: It was found to be clinically more effective and more cost effective to select a patient by Harada’s scoring system and, where a score of four or more was obtained, to administer a single 2 g/kg intravenous dose of gamma-globulin for acute KD.
    Pediatrics International 01/1999; 41(1):1 - 7. · 0.63 Impact Factor