Genetic mutation analysis in Japanese patients with non-syndromic congenital heart disease.

Congenital heart disease (CHD) is the most common birth defect occurring in humans and some transcriptional factors have been identified as causative. However, additional mutation analysis of these genes is necessary to develop effective diagnostic and medical treatment methods. We conducted sequence analysis of the coding regions of NKX2.5, GATA4, TBX1, TBX5, TBX20, CFC1 and ZIC3 in 111 Japanese patients with non-syndromic CHD and 9 of their relatives. All patient samples were also analyzed by multiplex ligation-dependent probe amplification using probes involved in chromosome deletion related to CHD. Five novel variations of TBX5, GATA4 and TBX20 were detected in 6 of the patients, whereas none were found in 200 controls. The TBX5 variation p.Pro108Thr, located in the T-box domain, was identified in a patient with tricuspid atresia, an exon-intron boundary variation of GATA4 (IVS4+5G>A) was detected in a Tetralogy of Fallot patient and an 8p23 microdeletion was detected in one patient with atrioventricular septal defect and psychomotor delay. A total of seven non-synonymous polymorphisms were found in the patients and controls. Accumulation of novel variations of genes involving the cardiac development may be required for better understanding of CHD.

Systemic ventricular morphology-associated increased QRS duration compromises the ventricular mechano-electrical and energetic properties long-term after the Fontan operation.

OBJECTIVES: To evaluate the impact of systemic ventricular (SV) morphology on mechano-electrical interaction (MEI) and compare the results with clinical profiles in Fontan patients. BACKGROUND: Abnormal ventricular MEI causes systolic dysfunction of the right and left ventricles (RV, LV). METHODS: We evaluated serial changes (up to 15 years post-Fontan operation) in QRS duration (QRS-d, ms), catheterization-based hemodynamics and SV performance in 77 patients and compared them with 36 referents. RESULTS: Preoperative QRS-d was wider in the Fontan patients (86+/-14 vs. 64+/-9, p<0.001) and steadily increased for the next 15 years (107+/-25 vs. 85+/-9, p<0.001). QRS-d was wider in non-LV type SV patients than those with LV morphology (p<0.05) with a positive association with age at repair (p<0.001). SV contractility (Ees) improved in the RV and LV groups (p<0.05), during the next 10-year Ees, ventriculoarterial coupling (Ea/Ees) and ventricular efficiency (SW/PVA) were superior in the LV group. QRS-d and its long-term change correlated with the corresponding SV volume and change (p<0.01), respectively, while the immediate postoperative volume reduction did not result in the QRS-d shortening. QRS-d correlated with Ees, Ea/Ees, SW/PVA, and peak oxygen uptake (VO(2)) (p<0.001) and QRS-d was one of the main determinants of peak VO(2) (r=-0.48, p<0.001). CONCLUSIONS: SV morphology-associated wide QRS-d compromises the MEI as well as energetic properties long-term after the Fontan operation, especially in non-LV type patients.

Long-term outcome of isolated congenital complete atrioventricular block pacing since neonatal period: experience at a single Japanese institution.

BACKGROUND: 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. METHODS AND RESULTS: 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%. CONCLUSIONS: 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.

Competition between native flow and graft flow after coronary artery bypass grafting. Impact on indications for coronary artery bypass grafting for localized stenosis with giant aneurysms due to Kawasaki disease.

We report the postoperative course of native and graft flow after coronary artery bypass grafting (CABG) in two patients with giant aneurysms and localized stenosis due to Kawasaki disease (KD). Although both patients had undergone CABG to the left anterior descending artery (LAD) with the left internal thoracic artery (ITA), at 5 and 10 years old, respectively, the ITA grafts were occluded 1 month postsurgery. However, when the two patients suffered complete occlusion of the native LAD more than 10 years after surgery, angiograms showed that the ITA grafts had reopened. We believe that this postoperative course reflects competition between the native artery flow and graft flow after CABG. CABG in patients with severely delayed coronary flows or recurrence of thrombus in giant aneurysms was ineffective in preventing myocardial infarction or damage. We conclude that CABG in giant aneurysm without significant localized stenosis should be avoided.

Risk factors for in-hospital mortality during infective endocarditis in patients with congenital heart disease.

Despite developments in preventative and medical therapy, infective endocarditis (IE) carries a high rate of mortality. Risk factors for mortality are unknown in pediatric and adult patients with congenital heart disease (CHD). We determined the risk factors for in-hospital mortality in pediatric and adult patients with CHD. A retrospective observational cohort study was conducted from January 1997 to December 2001 in Japan. Of the 239 patients for whom complete data were available, 216 patients with CHD were identified. Outcomes were alive or deceased. The proposed modified Duke's criteria identified 137 patients, aged 1 month to 62 years with a median of 12 years, with IE. In-hospital mortality was 10%. Four risk factors were independently associated with mortality by stepwise logistic regression analysis: (1) vegetation size > or =20 mm (odds ratio 40.6, 95% confidence interval 2.42 to 681); (2) age <1 year (odds ratio 19.5, 95% confidence interval 1.74 to 219); (3) presence of heart failure (odds ratio 7.16, 95% confidence ratio 1.34 to 38.4); and (4) Staphylococcus aureus as a causative organism (odds ratio 5.68, 95% confidence interval 1.16 to 27.9). Surgical intervention emerged as a predictive factor for lower in-hospital mortality (odds ratio 0.045, 95% confidence interval 0.003 to 0.70) by stepwise logistic regression analysis. In conclusion, surgical intervention, which decreases the risk of in-hospital mortality, should always be considered.

Myocardial scintigraphy after pacemaker implantation for congenital complete atrioventricular block.

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.

Electrocardiographic and clinical characteristics of idiopathic restrictive cardiomyopathy in children.

BACKGROUND: Idiopathic restrictive cardiomyopathy (RCM) is not a single disease and is rare. METHODS AND RESULTS: The clinical features and clinical course of 12 pediatric patients with RCM seen between 1978 and 2005 were retrospectively analyzed. The age at diagnosis ranged from 4 months to 12 years (median 4 years). The age of 7 patients diagnosed because of an abnormal electrocardiogram (ECG) ranged from 4 to 12 years. Three infants less than 2 years old presented with cardiomegaly. Obliquely elevated ST-T segments and the late peak T waves on 12-lead ECG were present in 8 patients (67%). Three patients with ST depression during exercise had no perfusion defects on radioisotope myocardial perfusion imaging. Two patients underwent orthotopic heart transplantation. Of the remaining 10 patients, 7 have died: 4 died suddenly and 3 died of right heart failure. Three patients with a hypertrophic left ventricular wall had a prolonged survival. The probability of survival at 1, 2 and 3 years was 78%, 52% and 26%, respectively. CONCLUSIONS: Obliquely elevated ST-T segments and the late peak T wave on ECG are characteristic, and reflect the restrictive physiology, which may indicate abnormalities of repolarization of ventricular muscle. The mode of death was either heart failure from pulmonary hypertension or sudden death from presumed ventricular arrhythmia.

Comparison of late post-operative cardiopulmonary responses in the Fontan versus ventricular septation for double-inlet left ventricular repair.

Ventricular septation (VS) and the Fontan procedure are alternatives for definitive repair in patients with double-inlet left ventricle; although VS is theoretically preferable, the current preference in practice is the Fontan procedure. However, the long-term outcomes of both procedures remain unclear. To address this issue, cardiopulmonary responses during exercise were measured in patients with double-inlet left ventricle, and the impact of the type of procedure performed, Fontan or VS, on long-term exercise capacity and late postoperative clinical profiles was assessed. Fourteen post-Fontan patients (mean age 17+/-6 years) and 13 VS patients (mean age 19+/-4 years) underwent exercise testing. Of the 13 VS patients, 5 required atrioventricular valve replacement (AVVR), and 7 required pacemaker implantation. Although no difference in peak oxygen uptake was found between the VS and Fontan patients, peak oxygen uptake was higher in VS patients without AVVR (30+/-8 ml/kg/min) than in VS patients with AVVR (19+/-1 ml/kg/min) and Fontan patients (22+/-6 ml/kg/min) (p<0.01). There was no significant difference in peak oxygen uptake between the VS patients with and without pacemaker implantation (p=0.09). The clinical profiles of the VS and Fontan patients were similar in terms of medication and freedom from tachyarrhythmias or reoperations during the follow-up period. In conclusion, the data suggest that VS without AVVR provides excellent future exercise capacity in selected patients with double-inlet left ventricle.

Long-term patency of internal thoracic artery grafts for coronary artery stenosis due to Kawasaki disease: comparison of early with recent results in small children.

BACKGROUND: The results of coronary artery bypass grafting using the internal thoracic artery (ITA) had been reported less satisfactory in patients < 12 years old with coronary artery lesions caused by Kawasaki disease. METHODS: Since 1983, 67 patients have undergone this operation in our hospital; their age at operation ranged from 1 to 59 years (median 11 years), and the total number of ITA grafts was 95. The interval from operation to latest graft patency as confirmed by angiogram or echocardiogram ranged from 4 months to 23 years (median 8 years). For analysis of graft patency rates, patients were divided into 4 groups based on year of coronary artery bypass grafting and age when grafted. The groups based on year were from 1983 to 1993 (early) and from 1994 to 2006 (later), whereas the age groups were age at operation < 12 years and age at operation > or = 12 years. From 1999, percutaneous transluminal balloon angioplasty was performed for postoperative anastomotic stenosis. RESULTS: Percutaneous transluminal balloon angioplasty for anastomotic stenosis in ITA graft was performed in 6 patients. When the age at operation was < 12 years, the 10-year patency rate in the later period was 94.4% (n = 18), significantly > the 70.0% (n = 30) seen in the earlier period (P < .05). CONCLUSION: Recent results of ITA grafts in patients < 12 years old have improved through the application of appropriate indications and percutaneous transluminal balloon angioplasty for anastomotic stenosis. Once good flow in the ITA 1 year after surgery is confirmed, graft patency will persist > 20 years.

Heart rate dynamics during and after exercise in postoperative congenital heart disease patients. Their relation to cardiac autonomic nervous activity and intrinsic sinus node dysfunction.

BACKGROUND: Abnormal exercise-related heart rate (HR) dynamics, that is, blunted exercise HR response, lower peak HR, and delayed HR recovery after exercise, are associated with high morbidity and mortality in adults with acquired and congenital heart disease (CHD). However, the precise mechanisms underlying the abnormal HR dynamics remain unclear. OBJECTIVES: The purpose of this study is to evaluate the precise contribution of cardiac autonomic nervous activity (CANA) and sinus node function on exercise-related HR dynamics in postoperative patients with CHD. METHODS: We analyzed our previous data in 53 postoperative patients with CHD who had undergone pharmacologic evaluation, including intrinsic HR, and compared the results with HR dynamics. RESULTS: Intrinsic HR (84% +/- 11%) was lower than the expected value and independently correlated with resting HR (P < .05). Univariate analysis demonstrated that all CANA indices significantly correlated with most HR dynamic parameters. On multivariate analysis, basal parasympathetic nervous activity significantly influenced all HR dynamics (P < .05-.0001), except for peak HR, whereas postsynaptic beta sensitivity of the sinus node significantly influenced all HR dynamics (P < .05-.001), except for early HR recovery. Resting plasma norepinephrine significantly correlated with all HR dynamics (P < .05-.001), except for resting HR. CONCLUSIONS: Lower resting and peak HRs are independently associated with intrinsic sinus node dysfunction and abnormal sympathetic CANA, respectively. A blunted HR increase and delayed early HR recovery are independently associated with impaired sympathetic and parasympathetic CANAs with the greater influence on a blunted exercise HR increase.

Hybrid therapy for rapid enlargement of hibernating coronary arteriovenous fistulas.

The use of hybrid therapy for recurrent multiple coronary arteriovenous fistulas in a 56-year-old woman is reported. The patient underwent surgical closure of a coronary arteriovenous fistula of the right coronary artery under cardiopulmonary bypass at 47 years of age. Reoperation was required 9 years later for recurrence of the same fistula. It was divided under a beating heart. Early postoperative angiography showed complete occlusion of the right coronary fistula. However, hibernating fistulas of the left circumflex artery, which had been left untouched because of insignificant shunt with no remarkable change for 9 years, increased in size rapidly. Transcatheter embolization was successfully performed for these residual fistulas.

Possible role of adrenomedullin in the regulation of Fontan circulation: mature form of plasma adrenomedullin is extracted in the lung in patients with Fontan procedure.

OBJECTIVE: We investigated the pathophysiological significance of molecular forms of adrenomedullin (AM) in patients after the Fontan procedure. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Impact of central hypercapnic chemosensitivity on enhanced ventilation in patients after the Fontan operation.

BACKGROUND: Central hypercapnic chemosensitivity (Chemo) influences the enhanced ventilatory and sympathetic responses in heart failure patients; however, its influence on these responses in Fontan patients is unknown. OBJECTIVES: To measure Chemo and compare the results with rest and exercise ventilatory characteristics in Fontan patients. METHODS AND RESULTS: We measured Chemo (l/min/mmHg), hemodynamics, pulmonary function, cardiac autonomic nervous and neurohumoral activities and compared the results with the ventilatory response during exercise in 42 Fontan patients and 12 referents. Chemo did not differ significantly between the Fontan patients (1.5 +/- 0.9) and referents (1.3 +/- 0.4). However, a higher Chemo in addition to lower resting arterial oxygen saturation (SaO(2)) and higher dead space ventilation (Vd/Vt) independently determined a higher resting minute ventilation (VE) and, except for the Chemo, these factors also independently determined the higher resting ventilatory equivalent for carbon dioxide output (VE/VCO(2)) (p<0.05-0.001). At peak exercise, the higher Chemo as well as the higher peak Vd/Vt and aerobic exercise capacity independently determined the higher peak VE and VE/VCO(2) (p<0.01-0.001). Among cardiac autonomic and neurohumoral activities, only the higher plasma norepinephrine concentration was associated with higher Chemo in Fontan patients (r=0.40, p<0.01) and age was correlated positively with Chemo in the high Chemo (> or =2.1) Fontan patients (n=10). CONCLUSIONS: In addition to lower SaO(2) and higher Vd/Vt, an increased Chemo associated with sympathetic activation has a significant impact on accelerated rest and exercise ventilation in some Fontan patients, especially in adult patients.

Clinical features in adults with coronary arterial lesions caused by presumed Kawasaki disease.

There exists a population of adults with undiagnosed coronary arterial lesions due to Kawasaki disease occurring before 1967. We report the clinical features in 6 adult males with coronary arterial lesions caused by presumed Kawasaki disease, whose dates of birth ranged from 1945 to 1963. The age of the diagnosed coronary arterial lesions due to presumed Kawasaki disease ranged from 26 to 48 years. In 4 patients, there was a history of probable Kawasaki disease. The presenting features were chest pain in 2; syncope in 1, an abnormal electrocardiogram in 2; a history of presumed Kawasaki disease in 1, and symptomatic myocardial infarction in the final patient. Coronary angiograms revealed multi-vessel disease in 5 patients, with segmental stenosis in 5, and calcified giant aneurysms in the proximal portion of the coronary arteries also in 5. Low left ventricular ejection fractions of less than 40% were found in 3. Of the patients, 3 had undergone coronary arterial bypass grafting. A defibrillator had been implanted in 2 because of rapid ventricular tachycardia with syncope induced during electrophysiologic studies. We conclude that, in patients with multi-vessel disease or left ventricular dysfunction caused by presumed Kawasaki disease, symptoms and serious cardiac events occur in adult life with the onset of ageing, although the patients had been asymptomatic for many years after the onset of Kawasaki disease itself.

Hemodynamic deterioration during simulated supraventricular tachycardia in patients after the Fontan operation.

BACKGROUND: Supraventricular tachycardia (SVT) is becoming of great concern for patients long after the Fontan operation and arterial blood pressure (BP) decreases during SVT may be associated with their morbidity and mortality. METHODS: We investigated the BP during supraventricular pacing (SVP) to simulate SVT in these patients. We measured femoral venous pressure (FVP) and plasma norepinephrine (NE) during SVP at 150 and 180 per minute in 20 Fontan patients and recorded the initial BP fall, its recovery and steady-state BP. RESULTS: Wide QRS duration had a significant impact on a greater BP decline and increases in steady-state FVP and NE at 150 SVP. Changes in BP, FVP and NE were greater at 180 SVP and in adult patients. In addition to a greater impact of baseline BP and age on the initial BP drop and steady-state BP (p<0.01); older age at Fontan repair, lower ventricular ejection fraction and heart rate variability were major determinants of greater BP fluctuation during 180 SVP (p<0.05). Patients with a history of SVT (n=5) exhibited a greater initial BP drop and increase in FVP during 180 SVP (p<0.01). The type of Fontan procedure had no influence on BP dynamics during SVP. CONCLUSIONS: In Fontan patients, wide QRS duration causes a greater hemodynamic fluctuation at slower SVT. Higher baseline BP and age, impaired ventricular function, late Fontan repair and a history of SVT were major determinants of hemodynamic deterioration during faster SVT.

Predictive factors for long-term prognosis in adults with cyanotic congenital heart disease--Japanese multi-center study.

OBJECTIVE: Adults with cyanotic congenital heart disease (CCHD) are associated with a significant incidence of morbid events and premature deaths that may be predicted during childhood. We aimed to identify predictive factors related to long-term prognosis through a Japanese multi-center cross-sectional study. METHODS: Data were collected from 253 adults with CCHD (126 men; age 28 (18 to 56) years) from 15 participating centers between 1998 and 2003. Laboratory data such as cardiothoracic ratio (CTR), percutaneous oxygen saturation (SpO2), hematocrit levels (Ht) and platelet counts (Pl-c) at the age of 15 years were collected for predictive factor analysis for death and cardiovascular and systematic events. Predictive factors were determined by multivariate Cox regression analysis. RESULTS: After a mean follow-up of 21 (0-42) years, 23 patients died with a median age of 29 (18-54) years (heart failure in 8, sudden in 6 and systematic complications in 9). Survival since 18 years of age was 91% and 84% at 10 and 20 years, respectively. Significant predictive factor for death was Pl-c<130x10(9)/l and for renal failure (n=7) was Ht>65%. 162 patients were hospitalized and predictors for hospitalization due to heart failure (n=45) were common atrioventricular canal CTR>60% and Pl-c<100x10(9)/l and that due to arrhythmias (n=44) were systematic right ventricle and CTR>60%. CONCLUSIONS: This multi-center study provides an objective basis of assessing the long-term prognosis in patients with CCHD. These data are useful in making decisions regarding medical management and in favorably altering the non-operative course of the disease.

Cardiorespiratory responses to exercise after anatomic repair of atrioventricular discordance with abnormal ventriculoarterial connection.

We evaluated exercise tolerance and cardiorespiratory responses to exercise in patients with atrioventricular discordance (AVD) and abnormal ventriculoarterial connection after anatomic repair. Cardiopulmonary treadmill exercise testing with gas measurement was done 62 times in 19 patients with AVD who had undergone anatomic repair at the National Cardiovascular Center. Exercise duration, oxygen uptake (V(O2)) and heart rate at anaerobic threshold and peak, and oxygen pulse during exercise were significantly lower in patients with AVD after anatomic repair than in controls. Carbon dioxide ventilatory equivalent during exercise was worse in patients with AVD after anatomic repair than in controls. Percentage peak V(O2) significantly correlated positively with percentage peak oxygen pulse and tended to correlate positively with the heart rate increments. Patients with AVD after anatomic repair exhibit impaired responses of heart rate and oxygen pulse with lower exercise capacity. Careful attention should be paid to patients with AVD after anatomic repair in terms of their functional capacity as well as other postoperative complications.

Prevalence of arrhythmias and their risk factors mid- and long-term after the arterial switch operation.

Early results of the arterial switch operation (ASO) for transposition of the great arteries (TGA) are good, but there are few mid- and long-term data on postoperative arrhythmias, especially in Japan. In this study, clinical data on 624 1-year survivors who had an ASO between 1976 and 1995 were collected from six institutes in Japan up to October 2002. Sixty (9.6%) 1-year survivors had significant arrhythmias. Bradycardia occurred in 22 patients, including complete atrioventricular block (CAVB) in 12, sick sinus syndrome (SSS) in 6, and second-degree atrioventricular block in 4. Syncope developed in 2 with CAVB and 2 with SSS. Ten patients with bradycardia underwent permanent pacemaker implantation. Supraveutricular tachycardia (SVT) was seen in 25 patients, including paroxysmal supraventricular tachycardia in 16, atrial flutter in 7, and atrial fibrillation in 2. Six patients with SVT received antiarrhythmic medication. SVT was transient in 20 and persistent in 5. Ventricular arrhythmias occurred in 13 patients, including nonsustained ventricular tachycardia in 5, paroxysmal ventricular contractions with couplets in 5, ventricular flutter in 2, and sustained ventricular tachycardia in 1. Four patients with ventricular arrhythmias received antiarrhythmic medication. Of the study patients, 8 died 1 year or more after ASO. Death was directly related to arrhythmia in 1 patient and was due to nonsustained ventricular tachycardia with severe congestive heart failure. The presence of a ventricular septal defect (VSD) was a risk factor for postoperative arrhythmia. Patients with TGA and VSD had more arrhythmias than those with TGA and an intact ventricular septum (13.7 vs 8.7%, p < 0.05), and this was especially true for CAVB (3.9% vs 1.0%, p < 0.05). In 36 patients clearly documented time onset of postoperative arrhythmia arrhythmia developed in 18 (50%) after less than 1 year and in 15 (42%) after more than 5 years. In summary serious arrhythmias after ASO were uncommon, but postoperative arrhythmias, such as unpaced CAVB, SSS, and VT, were related to morbidity and mortality. VSD was a risk factor for postoperative arrhythmia, especially CAVB. Approximately half of the arrhythmias developed late. Lifelong monitoring with respect to arrhythmia is needed for patients after ASO.

Percutaneous transluminal coronary rotational atherectomy for localized stenosis caused by Kawasaki disease.

We report the results of percutaneous transluminal coronary rotational atherectomy (PTCRA) for localized stenosis caused by Kawasaki disease (KD) in children. Five males and a females, aged 5-15 years old (median, 9), underwent PTCRA. The interval from the onset of KD to PTCRA ranged from 5 to 12 years (median, 9). The target vessels were the left anterior descending artery (three patients), the left circumflex (two patients), and the right coronary artery (one patient). The immediate results of PTCRA were successful in all patients, and the mean stenosis degree improved from 89 +/- 10% to 27 +/- 12%. In follow-up coronary angiograms within 1 year, four vessels were restenosed, including two with complete occlusion. A 15-year-old male has had good patency for 4 years after undergoing re-PTCRA for restenosis using a larger burr size. PTCRA is feasible for severe localized stenosis with calcification caused by KD in children and the immediate results are good. However, restenosis often occurred within 1 year after PTCRA in small children and PTCRA is not always appropriate for them. When the use of a larger burr is possible, good patency can be expected and can be maintained by close follow-up and re-PTCRA.