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ABSTRACT: Patients with isolated congenital complete atrioventricular block (CCAVB) occasionally develop dilated cardiomyopathy (DCM), despite early pacemaker implantation. However, the etiology of the DCM and its relationship to permanent ventricular pacing are not fully understood. Twenty-five patients with CCAVB underwent (99m) technetium (Tc) myocardial perfusion scintigraphy. Five patients were studied before and after pacing, providing a total of 30 image sets, which were divided into three groups; group 1: CCAVB before pacemaker implantation (PMI) (n = 11); group 2: CCAVB after PMI who did not subsequently develop DCM (n = 13); group 3: CCAVB after PMI who subsequently developed DCM (n = 6). Perfusion defects on single-photon-emission computed tomography (SPECT) were identified in group 1, 0 of 11 patients; group 2, 85% of patients; and group 3, 100% of patients. In groups 2 and 3, in patients with right ventricular pacing, the perfusion defects were mainly in the septum or between the apex and septum. On 20 segments' polar maps, the distribution of %uptake showed a similar pattern in groups 2 and 3, the degree of decreased %uptake and the number of segments with decreased %uptake being more severe in group 3. "Artificial" left bundle branch block (LBBB) pattern myocardial contraction induced by right ventricular pacing decreased myocardial perfusion around the apex and septum. Some patients with CCAVB will develop left ventricular dysfunction caused by artificial LBBB-induced interventricular asynchrony.
European Journal of Pediatrics 03/2008; 167(2):183-8. · 1.88 Impact Factor
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ABSTRACT: Few investigators have examined the outcome of patients with isolated congenital complete atrioventricular block (CCAVB) paced from the neonatal period. The present study follows the clinical course and describes the outcome of patients who have been paced with CCAVB since they were neonates.
The medical records of 20 patients with CCAVB paced as neonates between 1981 and 2006 were retrospectively studied and reviewed; 18 were diagnosed in utero and 2 at birth. SS-A antibodies were detected in 9 of 14 (64%) mothers tested. The median age at follow-up was 5.6 years (range, 2 days to 21.9 years). Three (15%) of the early neonates died and 3 (15%) died later: 2 from dilated cardiomyopathy (DCM) and 1 from pneumonia. The total mortality rate was 30%. The cumulative probability of survival at 10 years was 73%. Of the 14 survivors, 4 developed DCM, representing a total morbidity of 30%. The cumulative probability of freedom from DCM at 10 years was 59%.
Patients with CCAVB who undergo pacing as neonates have high mortality and high morbidity because of DCM. Left ventricular pacing or resynchronization can benefit patients who develop DCM and left ventricular epicardial pacing is recommended for neonates with CCAVB.
Circulation Journal 02/2008; 72(1):81-7. · 3.77 Impact Factor
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Ken Watanabe,
Toshio Nishikimi,
Motoki Takamuro,
Kenji Yasuda,
Yuichi Ishikawa,
Saori Tanabe,
Osamu Yamada,
Toshikatsu Yagihara,
Shinichi Suga,
Kenji Kangawa,
Hiroaki Matsuoka,
Shigeyuki Echigo
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ABSTRACT: We investigated the pathophysiological significance of molecular forms of adrenomedullin (AM) in patients after the Fontan procedure.
Plasma concentrations of mature AM (AM-m), an active form, glycine-extended AM (AM-Gly), an inactive form, and total AM (AM-T: AM-m+AM-Gly) were measured by specific immunoradiometric assay in the femoral vein, pulmonary artery and femoral artery of 29 consecutive patients after the Fontan procedure. The eleven patients who had history of Kawasaki disease and have normal coronary and hemodynamics served as control.
Patients who underwent Fontan procedure had significantly higher venous concentrations of AM-T, AM-Gly, and AM-m than age-matched normal controls (AM-T, 12.0+/-3.3 vs. 9.6+/-2.0; AM-Gly, 10.4+/-3.0 vs. 8.5+/-1.6; AM-m, 1.6+/-0.7 vs. 1.0+/-0.6 pmol/l, each p<0.05). In patients with Fontan procedure, there were no differences in plasma AM-T, AM-Gly or AM-m levels between the femoral vein and pulmonary artery, however, there was a significant step-down in the AM-m levels, but not in plasma AM-T or AM-Gly levels, between the pulmonary artery and femoral artery (1.3+/-0.6 to 1.0+/-0.6, p<0.05). The venous concentrations of AM-m correlated negatively with systemic blood flow (cardiac output) (r=-0.46, p<0.05).
Results suggest that in Fontan circulation plasma AM-m is increased in parallel with those of AM-T and AM-Gly and that AM-m is extracted in the lung. Extracted AM-m may be involved in the regulation of pulmonary arterial tonus, although further studies are necessary to elucidate the exact role of AM in Fontan circulation.
Regulatory Peptides 06/2007; 141(1-3):129-34. · 2.11 Impact Factor
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Pediatrics International 09/2005; 47(4):466-8. · 0.63 Impact Factor
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ABSTRACT: Impaired cardiac autonomic nervous activities and increased neurohumoral activities (CANA, NHA) characterize Fontan patients. However, the clinical significance of these changes is not clearly understood. Our purpose was to clarify the clinical significance of the CANA and NHA in stable Fontan patients.
We divided 22 atriopulmonary connection (APC) and 75 total cavopulmonary connection (TCPC) patients into 4 subgroups according to New York Heart Association (NYHA) class (1.8+/-0.6) and measured various CANA and NHA indices. All NHA indices were elevated in the symptomatic patients (P<0.001). Natriuretic peptides were higher in the APC than in the TCPC patients, and the hemodynamics showed no correlation with brain natriuretic peptide in the APC patients. Low arterial oxygen saturation and impaired hemodynamics greatly influenced all elevated NHA indices (P<0.01), except for plasma renin activity, in the TCPC patients. Impaired CANA indices did not relate to NYHA class, although surgeries were associated with lower heart rate variability. In addition to poor correlation between NHA and CANA, age and ventricular morphology had no impact on all CANA and NHA indices, except for high norepinephrine in right ventricular Fontan patients.
Although symptomatic Fontan patients exhibit higher NHA, CANA is not related to either NYHA class or NHA. APC itself is responsible for higher natriuretic peptides, and arterial oxygen desaturation has a great impact on elevated NHA in the TCPC patients. These characteristics of the NHA and CANA differ from those of heart failure patients with biventricular physiology.
Circulation 11/2004; 110(17):2601-8. · 14.74 Impact Factor
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ABSTRACT: We studied hemodynamic changes during simulated ventricular tachycardia using ventricular pacing. Hemodynamic deterioration during pacing is more significant in patients after right ventricular outflow tract reconstruction, especially in adults, than after closure of a ventricular septal defect. The cardiac autonomic nervous system has a significant impact on hemodynamics during simulated ventricular tachycardia.
The American Journal of Cardiology 07/2004; 93(11):1445-8, A12. · 3.37 Impact Factor
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Hideshi Tomita,
Satoshi Yazaki,
Kohji Kimura,
George Hayashi,
Hideki Fujita,
Yoko Okada, Ken Watanabe,
Ken-ichi Kurosaki,
Yasuo Ono,
Toshikatsu Yagihara,
Shigeyuki Echigo
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ABSTRACT: We performed balloon angioplasty in three patients with postoperative coarctation in the transverse arch adjacent to the left common carotid artery. The age at arch reconstruction was 5, 6, and 2 days, while the interval between operation and balloon dilatation was 59 days, 87 days, and 12 months, respectively. Two balloons, one in the stenosis and the other in the left common carotid artery, were introduced over a wire sequentially and inflated simultaneously until the waist of the balloon in the arch disappeared. After balloon dilatation, a significant reduction in the peak-to-peak pressure gradient and an increase in vessel diameters were observed in all patients. Further growth of the transverse arch was documented at follow-up in two patients. No aneurysm has been detected in any patients. We believe that placing a protective balloon in the neck vessel increases safety during balloon dilatation of coarctation in the transverse arch.
Catheterization and Cardiovascular Interventions 01/2004; 60(4):529-33. · 2.29 Impact Factor
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ABSTRACT: We implanted either large or medium Palmaz stents, or a Palmaz Corinthian stent, in various stenotic vessels, such as the pulmonary arteries, pulmonary veins, aorta, or superior caval vein. Using angiograms, we measured the diameter of the stenotic vessel before or after the implantation, the minimal diameter of the lumen, the minimal diameter of the largest fully expanded balloon used to expand the stent, and the diameter immediately after withdrawal of the balloon. The minimal diameter of the fully expanded balloon, and the minimal diameter of the lumen subsequent to expansion, were 8.2 +/- 2.4, and 7.7 +/- 2.3 mm, giving an absolute recoil of 0.5 +/- 0.4 mm, and a proportional recoil of 7 +/- 4%. There was no significant difference in either the absolute or proportional recoil for any of the stents, or for any of the different stenotic vessels. The proportional recoil correlated linearly with the minimal diameter of the lumen prior to the procedure, and with the ratio of the stenosis to the balloon, while the diameter of the stenotic vessels, the minimal diameter of the largest fully expanded balloon, the proportional stenosis prior to the procedure, and the ratio of the balloon to the diameter of the stenotic vessel, had no significant correlation with proportional recoil. The proportional recoil exceeded more than one-tenth when the minimal diameter of the lumen prior to the dilation was less than 3 mm, or the ratio of the balloon to the stenosis was greater than 3.0. An absolute recoil of around 1 mm is common when a large or medium Palmaz, or a Palmaz Corinthian stent, is implanted in great vessels. Balloons with a diameter of approximately one-tenth greater than that of the adjacent vessel may be needed if the minimal diameter of the lumen is small prior to the procedure.
Cardiology in the Young 01/2004; 13(6):519-25. · 0.76 Impact Factor
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ABSTRACT: In the present study, 0.2-0.6 mg/kg (0.4+/-0.2, mean +/- standard deviation) indomethacin was administered intravenously to close a patent ductus arteriosus in 13 infants with co-existing congenital heart defects whose ages ranged from 3 to 48 (14+/-14) days. All of them were hemodynamically ductus-independent and symptomatic. Echocardiography demonstrated that the ductus had closed in 8 infants, aged 3-33 (12+/-10) days (responders), but had not closed in 5 infants aged 6-48 (19 +/-19) days (non-responders). There was no significant difference between the responders and non-responders in their age, body weight, minimal diameter of the duct, dose of indomethacin, gestational age, birthweight, or Apgar score. One possible major complication might be associated with indomethacin. However, intravenous indomethacin should be considered prior to surgical ligation as one option for infants with a symptomatic patent ductus arteriosus complicated by other congenital heart defects.
Circulation Journal 10/2003; 67(9):750-2. · 3.77 Impact Factor
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ABSTRACT: The outcome of stent implantation and redilatation was investigated in 4 pediatric patients with 7 stenotic lesions of the pulmonary vein (PV), paying particular attention to late neointimal proliferation. The minimal diameter of the lesions increased from 0.8-3.6 (2.3+/-1.1) mm to 3.6-8.4 (5.1+/-1.6) mm immediately after stent implantation. Although the pressure gradient across the lesion was not measured in patient 4, in patients 1-3, it decreased from 4-34 (18+/-13) mmHg to 3-15 (7+/-4) mmHg. Except for case 4, who achieved a lesion diameter of 8.4 mm after initial stent dilatation, the other 6 lesions all restenosed within a few months, with an increasing pressure gradient. One patient with multiple PV stenoses associated with persistent severe pulmonary hypertension died suddenly. However, repeat dilatations were effective in all other lesions. Furthermore, in one lesion in patient 1, no serious restenosis developed for 20 months after the lesion was dilated up to 5.6 mm. Although further follow-up is mandatory, the final stent diameter within a vessel may determine long-term patency, and aggressive redilatation may be crucial for successful therapy of such a difficult disease.
Circulation Journal 04/2003; 67(3):187-90. · 3.77 Impact Factor
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ABSTRACT: Functional pulmonary atresia must be distinguished from anatomical atresia, which has an intact ventricular septum, to avoid inappropriate treatment, but there is a paucity of data regarding the echocardiographic features that differentiate these conditions. Echocardiographic findings in 5 neonates with functional atresia were compared to those in 5 with anatomical atresia. The left and right ventricular end-diastolic dimensions (LVDd, RVDd), percent of normal predicted LVDd, RVDd/LVDd, tricuspid valve ring diameter (TVD), percent of normal predicted TVD, grade of tricuspid regurgitation (TR), peak TR velocity, pulmonary valve ring diameter (PVD), percent of normal predicted PVD, the minimum diameter of the ductus and the peak velocity through it (PDA velocity) were measured. In addition, systolic pulmonary (PAp) and right ventricular pressure (RVp) from either PDA velocity or TR velocity, and calculated PAp/RVp were also estimated. There were significant differences in RVDd/LVDd, %TVD, and peak TR velocity between the 2 groups. All functional patients showed RVDd/LVDd >0.6, %TVD >100%, estimated RVp <50mmHg, PAp/RVp >0.85, and peak TR velocity <4m/s, whereas the findings in anatomical atresia patients were completely the opposite. In conclusion, a large RVDd/LVDd, TVD, PAp/RVp, low RVp and small TR velocity all suggest functional rather than anatomical pulmonary atresia, although there may be some exceptions such as severe Ebstein anomaly.
Circulation Journal 07/2002; 66(7):665-7. · 3.77 Impact Factor
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Hideshi Tomita MD,
Kohji Kimura MD,
Kenichi Kurosaki MD,
Yoko Okada MD,
Ken Watanabe MD,
Kenji Yasuda MD,
Satoshi Hasegawa MD,
George Hayashi MD,
Yasuo Ono MD,
Toshikatsu Yagihara MD, [......],
Kohji Kimura,
Kenichi Kurosaki,
Yoko Okada, Ken Watanabe,
Kenji Yasuda,
Satoshi Hasegawa,
George Hayashi,
Yasuo Ono,
Toshikatsu Yagihara,
Shigeyuki Echigo
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ABSTRACT: A female infant developed severe aortic coarctation 42 days after a Norwood operation. Balloon dilatation was ineffective due to rapid recoil of the narrowing, but stent implantation completely abolished coarctation. Although the patient died because of low systemic output with high pulmonary flow 62 days after stent implantation, an autopsy showed that the implanted stent was widely patent without any restenosis. Transcatheter stent implantation should be considered for coarctation in selected situations even in early infancy. Cathet Cardiovasc Intervent 2001;54:239–241. © 2001 Wiley-Liss, Inc.
Catheterization and Cardiovascular Interventions 09/2001; 54(2):239 - 241. · 2.29 Impact Factor
