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Nationwide registry data of patients with single-ventricle physiology have been rarely reported. The Medical Aid Program for Chronic Pediatric Diseases of Specified Categories (Japan) has contributed to the financial support of medical expense for patients younger than 20 years with chronic paediatric disease, and almost all children in Japan who require disease-specific treatment voluntarily apply to this programme. The epidemiology and medium- to long-term outcome of patients following a Fontan procedure were investigated using the database. The usefulness of this epidemiologic investigation in identifying real-world objectives and clinical applications was also examined. A total of 2862 patients who underwent a Fontan operation were identified from 18,589 patients with chronic heart disease registered to the medical aid programme. The details of symptoms, treatment, and somatic growth were evaluated, from which we were able to clarify the nationwide data regarding the current status of post-Fontan patients younger than 20 years. This study elucidated the current status of post-Fontan patients under 20 years of age in Japan. Data analysis of the Medical Aid Program for Chronic Pediatric Diseases of Specified Categories cohort provided useful information towards understanding the comprehensive status of patients with chronic heart disease and contributed to improved disease management.
Although larger stent placement could be effective for pulmonary vein stenosis, stents extrusion tends to occur due to caliber narrowing, small landing area, and the slippery nature. We placed stents with diameter ≧8 mm for four stenotic lesions using the “half-uncovered technique”. All stents were precisely placed and successfully resolved the stenosis. This technique allows us to avoid extrusion and to perform safe and effective dilatation when placing larger stents for pulmonary vein stenosis.
CHDs occasionally have coronary complications; however, stent implantation is technically difficult in small children. We reported a successful drug-eluting stent implantation into the congenital anomalous coronary artery in a 6-year-old boy. This treatment is useful for rescuing coronary stenosis, and dual antiplatelet therapy is important to prevent stent restenosis.
Static balloon atrial septostomy is a widely accepted intervention for children with CHD. Successful surgical palliation is creating increasing numbers of adult CHD patients who need subsequent left heart intervention requiring transseptal access. In these patients, the interatrial septum is usually thick and fibrotic because of a previous open heart surgery or catheter intervention, and conventional transseptal puncture may be unsuccessful. Static balloon atrial septostomy to access the left atrium may facilitate intervention via the interatrial septum in such situations. The purpose of this study was to investigate the usefulness and the safety of static balloon atrial septostomy, and the evolution of an iatrogenic atrial septal defect post procedure in adult CHD.
We retrospectively reviewed six procedures in five adults with CHD and collected demographic characteristics, details of the procedures, clinical outcome, and size changes of the iatrogenic atrial septal defect.
The mean age at the time of the procedure was 35 years. The intended primary interventions were pulmonary vein isolation, stenting for pulmonary vein obstruction, and catheter ablation for focal atrial tachycardia. All static balloon atrial septostomies were effective, and the left heart interventions were successfully achieved via transseptal sheaths. There were no major complications associated with the static balloon atrial septostomy. There were no adverse clinical outcomes related to iatrogenic atrial septal defect, and the size of the defects regressed over time in all cases.
Static balloon atrial septostomy can be a safe and useful technique in adult CHD patients needing left heart procedures. The thick interatrial septum found in postoperative patients may reduce the risk of persistent iatrogenic atrial septal defect.
Only few reports have described successful simultaneous transcatheter intervention for CHD in infants. We present an infant with secundum atrial septal defect complicated by valvular pulmonary stenosis. Percutaneous transcatheter pulmonary valvuloplasty was performed first, followed by transcatheter closure of the secundum atrial septal defect uneventfully. Simultaneous transcatheter correction is an effective therapeutic option even in infants.
Providing care for children with cardiac disease remains a challenge in developing countries.
Materials and methods
Since 2001, a team of paediatric cardiologists has been visiting Mongolia to perform children's cardiac catheterisation, as well as screening children for cardiac disease. Considering the limited medical resources, as well as cost, risk, and benefit, we focused our activities on diagnostic consultation by echocardiography, transcatheter closure of persistent arterial duct, balloon dilation of valvar pulmonary stenosis and aortic coarctation, and diagnostic catheterisation.
Up to 2011, we have completed echocardiography in 1200 patients; diagnostic catheterisations in 59 patients; and catheter interventions in 255 patients, including 224 for persistent arterial duct, 23 for valvar pulmonary stenosis, 6 for aortic coarctation, and a few others. We have visited 14 rural areas to screen for children's cardiac diseases. A total of 131 persistent ducts were closed with coils and a further 93 with a duct occluder. Migration of the coil or occluder to the pulmonary artery occurred once for each device. Pulmonary valvuloplasty was successful in 23 patients and a coarctation was effectively dilated in six patients.
The limited healthcare resources in developing countries such as Mongolia make catheter interventional procedures an attractive alternative to surgery in treating children with simple but critical congenital heart diseases. Introduction of the duct occluder extended the application of transcatheter occlusion of persistent arterial duct in Mongolia to larger vessels and avoided the expense of coil occlusion using multiple coils.
We analyzed retrospectively the relationship between coagulation profile, and either hepatic function or hemodynamics, in patients who had undergone a Fontan-type procedure, comparing them, first, with a control group of 12 patients without significant hemodynamic abnormality, and, second, with a group of 14 patients who had not undergone a Fontan procedure, but whose mean right atrial pressure exceeded 8 mmHg. Follow-up catheterization had been performed in all 30 patients submitted to the Fontan-type operation. Prothrombin time, and factor XIII, were significantly lower in those who had undergone the Fontan procedure than in the other groups. Those submitted to the Fontan operation also had lower levels of protein C than controls, and their levels of plasminogen were lower than the patients with high right atrial pressure. Both aspartate aminotransferase and alanine aminotransferase were higher in those undergoing the Fontan procedure than in the other groups, while gamma-glutamyltranspeptidase in these patients was higher than in the control group. Mean right atrial pressure was highest in those under-going the Fontan procedure, while cardiac index was lowest. Prothrombin time was correlated to some extent with aspartate aminotransferase, mean right atrial pressure, and cardiac index. Protein C correlated with both aspartate aminotransferase and mean right atrial pressure, while factor XIII correlated with alanine aminotransferase, mean right atrial pressure, and cardiac index. Aspartate aminotransferase, alanine aminotransferase, and gamma-glutamyltranspeptidase, parameters of hepatic function, correlated significantly with mean right atrial pressure. In those who had undergone the Fontan procedure, decreased synthesis of pro-and anti-coagulant factors is a risk factor for both thrombosis and bleeding. Abnormal hemodynamics, in the absence of a right sided pumping chamber, may predispose to subclinical hepatic dysfunction, leading to selective disturbances of protein synthesis.
We studied factors that predict in children catecholamine induced vasoconstriction of the persistent arterial duct. Epinephrine at 2.0–10.0 (4.9 ± 1.3, mean ± standard deviation) μg/kg was injected intravenously into 30 patients with persistent arterial duct whose age ranged from 1 to 91 (27 ± 26) months. In 11 patients aged 10–66 (34 ± 23) months (responders), a continuous murmur had almost completely disappeared on auscultation, and both the diameter and the shunt flow area of the arterial duct had become smaller as shown by Doppler echocardiography. On the other hand, there was no such change in 19 patients aged 1–91 (23 ± 27) months (non-responders). Although there was no significant difference in the age, the body weight, the minimal diameter of the arterial duct, or the doses of epinephrine between responders and non-responders, the gestational age at birth was slightly less (p=0.09) and the birth weight was significantly smaller in responders than in non-responders (p<0.05). The persistent arterial duct of those who had a history of low birth weight always responded to epinephrine. In infants younger than 6 months, the persistent arterial duct was never constricted by epinephrine regardless of the birth weight and gestational age. In low birth weight infants, the vascular smooth muscle of the persistent arterial duct can usually constrict beyond infancy. There may be some age dependent difference in responsiveness to epinephrine until 6 months of age.
A 41-day-old boy was transferred to our department with severe congestive heart failure. Digital subtraction counter current aortography, and antegrade aortography, revealed coarctation of a persistent right fifth aortic arch, stenosis of the origin of an aberrant left subclavian artery, and sequestration of the lower lobe of the right lung.
As his heart failure seemed to be caused not only by pressure overload to the left ventricle following coarctation, but also by the volume load associated with the shunting effect of the pulmonary sequestration, we performed transcatheter balloon dilation of the coarctation and the origin of the aberrant subclavian artery, together with embolization of the aberrant pulmonary artery. His heart failure responded dramatically to these procedures.
An aortic valve with four leaflets is a rare congenital malformation. It is usually diagnosed unexpectedly at autopsy, during replacement of the aortic valve, or by aortic angiography. Transthoracic cross-sectional echocardiography, however, can also delineate the structure of the aortic valve, and demonstrate the presence of valves with four leaflets
On the assumption that the diameter of the reference vessel might determine the thickness of neointimal coverage of stents placed in the pulmonary arteries, we analyzed the angiograms of 28 lesions in 17 patients who underwent follow-up cardiac catheterization. Excluding 2 lesions where late stenosis was determined mainly by recoil of greater than 30%, we investigated the neointimal thickness of 26 lesions in 16 patients. Several factors that might contribute to late re-stenosis were also analyzed. Age and body weight at implantation ranged from 0.8 to 20 years, with a median of 6 years, and from 6.8 to 77.5 kg, with a median of 17.6 kg. Follow-up interval was from 6 to 15 months, with a median of 6 months.
There was a significant increase in diameter, as well as a reduction in pressure gradient, immediately after the implantation of stents. Although there was no significant difference between the achieved diameter and the diameter of the stent at follow-up, the diameter of the lesion at follow-up was significantly smaller than the diameter achieved by stenting. The increase in the pressure gradient at follow-up was slightly greater in 4 lesions where the late reduction in diameter was greater than 30% than in the 18 lesions where this was less than 30% (p = 0.05). The diameter of the reference vessel, and the diameter by stenting correlated with the late reduction in diameter. All lesions with diameter reduced greater than 30% had a reference diameter of less than 6.1 mm, and a diameter achieved by stenting of less than 6.5 mm. Late loss in luminal diameter directly correlated with the thickness of the neointimal coverage. In conclusion, close observation should be mandatory following implantation of stents in small pulmonary arteries.
A 3-year-old boy developed acute obstruction of a left modified Blalock-Taussig shunt following selective power injection of contrast in the shunt. Balloon dilation was ineffective due to rapid recoil of the narrowed segment, but implantation of a stent effectively abolished the obstruction. The obstruction itself may have been produced by an intimal flap caused by the power injection.
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.
Objectives: We evaluated the efficacy of percutaneous transluminal coronary angioplasty for anastomotic stenosis after coronary arterial bypass grafting using the internal thoracic artery in patients with coronary arterial lesions due to Kawasaki disease. Subjects and Methods: From July 1997 to April 2000, four boys and one girl underwent percutaneous transluminal coronary angioplasty for 6 anastomotic lesions following coronary arterial bypass grafting using the left or right internal thoracic artery. Progressive severe stenosis of the grafts in the follow-up angiograms after grafting, and evidence of ischemia, were regarded as indications for percutaneous transluminal coronary angioplasty. Age at coronary angioplasty ranged from 4.2 to 16.7 years, with a median of 6.9 years, while the interval from operation ranged from 0.3 to 3.0 years, with a median of 1.1 years. The diameter of the balloon catheter employed varied from 1.5 to 2.5 mm, and the pressure of inflation ranged from 8 to 16 atmospheres. Results: The degree of stenosis decreased from 63 to 99%, with a median of 88%, to 0 to 40%, with a median of 17% immediately after angioplasty. A follow-up angiogram either 3 months or 1 year later revealed no restenosis in any patient. Conclusion: Percutaneous transluminal coronary angioplasty is a feasible and useful procedure for treating anastomotic stenosis following coronary arterial bypass grafting using the internal thoracic artery in patients with coronary arterial lesions due to Kawasaki disease.
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