Papers by Manatomo Toyono
Abstract 2263: Preoperative Regurgitant Volume Can Predict “Unexpected” Left Ventricular Dysfunction After Mitral Valve Repair for Chronic Mitral Regurgitation
Circulation, Oct 16, 2007
Background: Left ventricular (LV) dysfunction after mitral valve (MV) repair in patients with chr... more Background: Left ventricular (LV) dysfunction after mitral valve (MV) repair in patients with chronic mitral regurgitation (MR) carries a poor prognosis. It has been previously described that LV dysfunction could be predicted using preoperative echocardiographic parameters such as LV size and ejection fraction (EF). However, we encounter MR patients who have unexpected postoperative LV dysfunction despite preoperatively normal LV size and function. Methods: We retrospectively investigated pre- and post-operative (median 4th postoperative day) echocardiograms of 248 consecutive patients who underwent MV repair from June through December 2005 for degenerative MR without other heart complications except tricuspid regurgitation. Among these patients, 48 patients were excluded because of inadequate echo quality for regurgitant volume (RVol) quantitation. Our study consisted of the remaining 200 patients (mean age 57±13 years, 140 male) in whom we could clearly determine the preoperative RVol from either quantitative Doppler and/or proximal isovelocity surface area method. Results: After MV repair, the median (interquartile range) EF was significantly decreased from 68 (61–75) to 54 (41–63) % ( P P 60% and LVDs P P P P Conclusion: A very large regurgitant volume was a significant predictor of postoperative LV dysfunction after MV repair despite preoperatively normal LV size and function. Quantification of MR volume is strongly recommended for earlier indication of MV repair in patients with severe MR.
Doppler-derived preoperative mitral regurgitation volume predicts postoperative left ventricular dysfunction after mitral valve repair
American Heart Journal, May 1, 2009
Unexpected postoperative left ventricular (LV) dysfunction after valve repair for mitral regurgit... more Unexpected postoperative left ventricular (LV) dysfunction after valve repair for mitral regurgitation (MR) occurs in some patients with normal preoperative LV function. Identification of factors that predispose to such LV dysfunction would enhance our understanding of the indications and outcomes of surgery. We retrospectively analyzed pre- and postoperative (median fourth day) echocardiograms of 174 patients undergoing valve repair for pure and isolated MR. Preoperative MR volume was quantified by the quantitative Doppler and/or proximal isovelocity surface area method. There was an incremental predictive value of MR quantification over the current recommendations (global chi(2) from 48.14 to 81.57, P < .001; Hosmer-Lemeshow test, P = .98), for postoperative LV dysfunction, defined as ejection fraction <50%. The independent predictors were MR volume and LV end-systolic dimension (P < .001 and P = .01, respectively). Sixty-nine patients underwent surgery before development of the current surgical criteria, namely, symptoms, atrial fibrillation, preoperative LV dysfunction, or pulmonary hypertension. Of…
Paradoxical Relationship Between B-type Natriuretic Peptide and Pulmonary Vascular Resistance in Patients with Ventricular Septal Defect and Concomitant Severe Pulmonary Hypertension
Pediatric Cardiology, Sep 5, 2007
B-type natriuretic peptide (BNP) reflects volume overload on left ventricle and pulmonary hyperte... more B-type natriuretic peptide (BNP) reflects volume overload on left ventricle and pulmonary hypertension (PH) in patients with ventricular septal defect (VSD). Pulmonary vascular resistance (PVR) has been reported to correlate positively with BNP in VSD patients with various degrees of PH. We aimed to investigate the relationship between PVR and BNP in VSD patients with severe PH. We examined 24 subjects with VSD concomitant severe PH aged from 2 months to 17 years (median: 4 months). The ratio of pulmonary to systemic pressure (Pp/Ps), the ratio of pulmonary to systemic flow (Qp/Qs), the ratio of pulmonary to systemic resistance (Rp/Rs), and PVR were determined by cardiac catheterization. PVR and Rp/Rs ranged from 1.6 to 15.5 (mean: 5.7 +/- 3.9) Wood unit . m(2) and 0.1 to 0.8 (mean: 0.4 +/- 0.2), respectively. BNP ranged from 5.5 to 69 (mean: 31 +/- 19) pg/ml. Negative correlations were observed between BNP and PVR (r = -0.56, p = 0.004) and BNP and Rp/Rs (r = -0.51, p = 0.01). BNP was significantly lower (<10 pg/ml) in VSD patients with Eisenmenger physiology as compared with the others (p = 0.003). We should draw attention to evaluate BNP values in VSD patients with severe PH.
Usefulness of Left Ventricular Shape to Predict the Early Recovery of Left Ventricular Function After Isolated Aortic Valve Replacement for Aortic Valve Stenosis
American Journal of Cardiology, Dec 1, 2008
Improvement in left ventricular (LV) systolic function after aortic valve replacement (AVR) has b... more Improvement in left ventricular (LV) systolic function after aortic valve replacement (AVR) has been observed in patients with aortic valve stenosis (AS). However, the factors that predict such recovery remain unclear. We sought to identify the predictive value of the LV spherical shape for LV functional recovery after "isolated" AVR in patients with severe AS and LV dysfunction. We examined 90 patients with severe AS and LV systolic dysfunction by echocardiography before and after AVR. Patients with known coronary artery disease, significant aortic or mitral regurgitation, and other cardiac surgery were excluded. LV end-diastolic and end-systolic volumes indexes and ejection fraction (EF) were measured by the Simpson method. LV mass index was calculated by the area-length method. LV end-diastolic and end-systolic sphericity were calculated as the ratio of the minor axis to the major axis of the left ventricle in apical 4-chamber view. The postoperative EF was significantly associated with preoperative EF, end-diastolic and end-systolic volumes indexes, LV mass index, and end-diastolic and end-systolic sphericity (all p <0.001). Multivariate analysis revealed that preoperative EF, end-systolic volume index, and end-diastolic sphericity were independent parameters predicting postoperative EF. The sensitivity and specificity in predicting normalization of EF…
Pediatric Research, Apr 1, 1994
Expanding coronary aneurysm in the late phase of Kawasaki disease
Pediatrics International, Feb 1, 2012
A case of MERRF associated with chronic pancreatitis
Neuromuscular Disorders, Apr 1, 2001
We report the first case to our knowledge of chronic pancreatitis associated with mitochondrial e... more We report the first case to our knowledge of chronic pancreatitis associated with mitochondrial encephalopathy with the A8344G mitochondrial DNA (mtDNA) mutation. This 10-year-old-girl had suffered from recurrent abdominal pain with elevated serum amylase and lipase since the age of 6, and easy fatigability, tremor and astatic seizures since the age of 8. A biopsy of quadriceps muscle revealed ragged-red-fibers and cytochrome c oxidase deficiency. Analysis of mtDNA in peripheral blood identified an A8344G mutation in the mitochondrial tRNA(Lys) gene. Taken together with physical signs of myoclonic seizures and cerebellar dysfunction, we diagnosed her as myoclonic epilepsy with ragged-red fibers associated with chronic pancreatitis. Although no association between mitochondrial disease and pancreatitis has yet been established, this case suggests it is necessary to consider the participation of mitochondrial abnormality in the pathogenesis of recurrent pancreatitis.
Transcatheter Occlusion of a Giant Coronary Artery Fistula in a Neonate
American Journal of Case Reports, Aug 9, 2021
Patient: Female, newborn Final Diagnosis: Coronary arterial fistula Symptoms: Respiratory distres... more Patient: Female, newborn Final Diagnosis: Coronary arterial fistula Symptoms: Respiratory distress Medication: — Clinical Procedure: — Specialty: Cardiology • Pediatrics and Neonatology Objective: Congenital defects/diseases Background: Although large coronary artery fistulas are uncommon; they lead to substantial over-circulation in the pulmonary vascular beds and left heart system. Fistula occlusions are achieved via surgical or transcatheter technique; however, reports on successful outcomes of transcatheter treatment during the neonatal period are limited. Case Report: A female infant was born at the gestational age of 37 weeks with a birth weight of 2615 grams via normal vaginal delivery. Cardiac auscultation revealed a loud continuous murmur emanating from the fourth right intercostal space. A right coronary artery-to-right ventricle fistula was confirmed using transthoracic echocardiography. The newborn developed respiratory distress 3 days after birth and was administered continuous positive airway pressure to assist breathing. On day 8, the ventilator was used through tracheal intubation due to gradual worsening of dyspnea. A 6-mm Amplatzer Vascular Plug 4 (AGA Medical Corporation, Plymouth, MN) was chosen, as the minimum diameter of the coronary artery fistula was 5 mm. In view of the risk of myocar-dial ischemia with additional devices, the procedure was stopped despite persistent shunting. The newborn’s clinical condition significantly improved following the procedure and she was eventually weaned off ventilator support. Conclusions: A self-expanding occlusion device was useful for relieving this life-threatening condition. Complete elimination of shunting is not always necessary, to avoid compromising myocardial circulation.
Case reports in genetics, Aug 29, 2020
Genitopatellar syndrome (GPS) is a rare autosomal dominant disorder caused by de novo pathogenic ... more Genitopatellar syndrome (GPS) is a rare autosomal dominant disorder caused by de novo pathogenic variants in the KAT6B gene. It is characterized by genital abnormalities, patellar hypoplasia/agenesis, flexion contractures of the hips and knees, corpus callosum agenesis with microcephaly, and hydronephrosis and/or multiple renal cysts. More than half of patients with GPS have congenital heart defects, mostly atrial and/or ventricular septal defects, patent foramen ovale, and patent ductus arteriosus. We report a case of a Japanese neonate with a de novo heterozygous c.3769_3772delTCTA pathogenic variant in the KAT6B gene who presented with a cardiac intramural cavity of the ventricular septum at birth. e cavity unexpectedly disappeared at 1 month of age, but trabecular septal thinning and flash remained. e features of the cavity were not consistent with those of congenital ventricular diverticulum or aneurysm, and its identity and prognosis are still unclear. Because patients with GPS may exhibit various forms of cardiac malformation, careful cardiac examination and follow-up are required from birth in cases of suspected GPS.
European Heart Journal, Oct 1, 2019
Circulation journal, 2013
Analysis of Coronary Arterial Aneurysm Regression in Patients With Kawasaki Disease by Aneurysm Severity: Factors Associated With Regression
Journal of the American Heart Association
Background Coronary arterial aneurysms (CAAs) associated with Kawasaki disease (KD) significantly... more Background Coronary arterial aneurysms (CAAs) associated with Kawasaki disease (KD) significantly affect prognosis. However, the clinical course of CAAs and factors associated with CAA regression have not been well analyzed. Methods and Results The cohort of the Z‐Score 2nd Project Stage study, a multicenter, retrospective, cohort study involving 44 institutions in Japan including 1006 patients with KD, was examined. CAAs were classified by the z score of their internal diameter in the acute phase: small ( z <5), medium (5≤ z <10), and large ( z ≥10). The lower limit of small CAA was based on the Japanese Ministry of Health, Labour and Welfare criteria. In the right coronary artery, the CAA regression rates 10 years after diagnosis were 95.5% for small, 83.2% for medium, and 36.3% for large. In the proximal left anterior descending artery, the regression rates 10 years after diagnosis were 95.3% for small, 80.1% for medium, and 28.8% for large. Cox regression analysis showed t...
Abstract O.57: Coronary Circulation Assessed by Transthoracic Echocardiography during Exercise Test is Impaired in Patients after Kawasaki Disease Even with Regressed Coronary Arterial Lesions
Circulation, Apr 28, 2015
Background: Coronary flow reserve (CFR) has important clinical implications for the evaluation of... more Background: Coronary flow reserve (CFR) has important clinical implications for the evaluation of coronary circulation including Kawasaki disease (KD). Pharmacological vasodilation is generally used to induce hyperemia for the assessment of CFR; however, exercise test provides more physiological stress. Objectives: This study sought to assess CFR during exercise in patients after KD with regressed coronary arterial lesions (CALs) by transthoracic echocardiography (TTE). Methods: Twenty KD patients were studied, comprising 8 patients with regressed CALs in the left anterior descending coronary artery (LAD) and 12 patients without CALs (median ages; 10 and 9 years, respectively). Fourteen age-matched healthy subjects were also studied as controls. We obtained peak diastolic coronary flow velocity (CFV) of the LAD by pulsed-Doppler TTE at rest and at submaximal exercise on supine ergometer. CFR was calculated as the ratio of exercise to rest CFVs. Results: The CFV measurements were obtained in all the subjects. There was no significant difference in the CFVs among the patients with regressed CALs, those without CALs and the controls (30 ± 7 vs. 31 ± 10 vs. 28 ± 8 cm/s, respectively). The CFVs increased during exercise in the patients without CALs and the controls (51 ± 11 and 49 ± 10 cm/s, respectively, both p <0.05). In consequence, the CFR was lower in the patients with regressed CALs compared with those without CALs and the controls (1.3 ± 0.2 vs. 1.7 ± 0.2 and 1.7 ± 0.2, respectively, p <0.05). Conclusions: Exercise test demonstrated impaired CFR in KD patients with regressed CALs. It suggests that these patients have risk of future myocardial ischemic events.
Abstract 8322: Obesity-Related Alteration of Arterial Compliance Associated with Concentric Left Ventricular Hypertrophy Begins to Appear in Childhood
Circulation, Nov 22, 2011
Abstract O.54: Alteration of Left Ventricular Performance and Aortic Elastic Properties in Patients After Kawasaki Disease With Coronary Artery Aneurysm Even Without Cardiac Ischemia
Circulation, 2015
Introduction: Recent studies have suggested that patients after Kawasaki disease (KD) have altere... more Introduction: Recent studies have suggested that patients after Kawasaki disease (KD) have altered arterial stiffness. However, there is little evidence of the relationship between arterial stiffness and left ventricular (LV) global function of KD patients without cardiac ischemic region. Hypothesis: We hypothesized that the alteration in the elastic properties of the ascending aorta may influence both LV function. Methods: Sixty one patients after KD (age, 6.1±4.0 years) were studied, comprising 15patients with CAAs and 46 patients without CAA. All the patients with CAAs showed no cardiac ischemic region confirmed by myocardial perfusion SPECT. Using pulsed Doppler echocardiography combined with Doppler tissue imaging, mitral peak velocities during early diastole (E) and LV peak myocardial velocities during early diastole (e’) and systole (s’) were measured. The ratio of E/e’ was used as an index of filling pressure of the LV. From Doppler tissue imaging, Doppler-derived index of c...
Abstract 5794: Geometric Changes of Mitral Annulus Assessed by Real-Time 3-Dimensional Transesophageal Echocardiography
Circulation, 2008
Background: The mitral annular (MA) geometric changes have been reported in patients with various... more Background: The mitral annular (MA) geometric changes have been reported in patients with various cardiac diseases such as atrial fibrillation (Af), mitral regurgitation (MR) and dilated cardiomyopathy (DCM). The advances of real-time 3D transesophageal echocardiography (TEE) enable us to analyze the MA geometry more accurately and reliably than 3D transthoracic echocardiography (TTE). We sought to determine the independent predictors for MA geometric changes in patients with Af, significant MR, and DCM by 3D TEE. Methods: We examined 32 subjects by 3D TEE and 2D TTE; 6 with lone Af, 9 with mitral valve prolapse (MVP), 3 with organic MR, 6 with DCM, and 8 normal subjects. Left ventricular (LV) end-diastolic and end-systolic volume indices (EDVI and ESVI), ejection fraction (EF), left atrial volume index (LAVI), and MR severity were assessed by 2D TTE. We measured MA area index, commissural length, and MA height (Figure 1 ). For the index of the saddle-shaped MA geometry, MA shape in...
Abstract 10397: Higher Waist Circumference is the Start of Cardiovascular Structural and Functional Changes in Childhood Obesity
Circulation, 2015
Background: Higher waist circumference (WC) is associated with cardiovascular disease, however, l... more Background: Higher waist circumference (WC) is associated with cardiovascular disease, however, little is known about the association between WC and cardiovascular functional change in children. The purpose of this study is to examine the relationships between numbers of cardiovascular risk factors and left ventricular (LV) geometry and function in children. Methods: Echocardiography was performed in 311 children aged 7 to 13 years. Left atrial (LA) diameter, LV end-diastolic volume, ejection fraction, mass, mass-to-volume ratio, and carotid artery intima media-thickness (IMT) were measured. Transmitral peak flow velocities during early and late diastole and mitral annular myocardial velocities during early (Em) and late diastole (Am) were measured. LA systolic force and aortic stiffness were calculated. Lipids, uric acid, fasting glucose, insulin, high sensitive CRP, and homeostasis model assessment for insulin resistance (HOMA-IR) were also assessed. Subjects were divided into 4 g...
Abstract 16669: Left Atrial Function After Closure of Atrial Septal Defect in Adults: Comparison Between Transcatheter Closure and Surgical Closure
Circulation, 2018
Introduction: Transcatheter closure is offered as an alternative to surgery for the majority of p... more Introduction: Transcatheter closure is offered as an alternative to surgery for the majority of patients with secundum atrial septal defect (ASD). However, little is known about the effect of a mec...
Abstract 2263: Preoperative Regurgitant Volume Can Predict “Unexpected” Left Ventricular Dysfunction After Mitral Valve Repair for Chronic Mitral Regurgitation
Circulation, 2007
Background: Left ventricular (LV) dysfunction after mitral valve (MV) repair in patients with chr... more Background: Left ventricular (LV) dysfunction after mitral valve (MV) repair in patients with chronic mitral regurgitation (MR) carries a poor prognosis. It has been previously described that LV dysfunction could be predicted using preoperative echocardiographic parameters such as LV size and ejection fraction (EF). However, we encounter MR patients who have unexpected postoperative LV dysfunction despite preoperatively normal LV size and function. Methods: We retrospectively investigated pre- and post-operative (median 4th postoperative day) echocardiograms of 248 consecutive patients who underwent MV repair from June through December 2005 for degenerative MR without other heart complications except tricuspid regurgitation. Among these patients, 48 patients were excluded because of inadequate echo quality for regurgitant volume (RVol) quantitation. Our study consisted of the remaining 200 patients (mean age 57±13 years, 140 male) in whom we could clearly determine the preoperative ...
Case Reports in Genetics, 2020
Genitopatellar syndrome (GPS) is a rare autosomal dominant disorder caused by de novo pathogenic ... more Genitopatellar syndrome (GPS) is a rare autosomal dominant disorder caused by de novo pathogenic variants in the KAT6B gene. It is characterized by genital abnormalities, patellar hypoplasia/agenesis, flexion contractures of the hips and knees, corpus callosum agenesis with microcephaly, and hydronephrosis and/or multiple renal cysts. More than half of patients with GPS have congenital heart defects, mostly atrial and/or ventricular septal defects, patent foramen ovale, and patent ductus arteriosus. We report a case of a Japanese neonate with a de novo heterozygous c.3769_3772delTCTA pathogenic variant in the KAT6B gene who presented with a cardiac intramural cavity of the ventricular septum at birth. The cavity unexpectedly disappeared at 1 month of age, but trabecular septal thinning and flash remained. The features of the cavity were not consistent with those of congenital ventricular diverticulum or aneurysm, and its identity and prognosis are still unclear. Because patients wit...
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Papers by Manatomo Toyono