Satoshi Yazaki

Impact of the Evolution of the Fontan Operation on Early and Late Mortality: A Single-Center Experience of 405 Patients Over 3 Decades

BACKGROUND Postoperative mortality has decreased in patients undergoing the Fontan operation, and the determinants of such mortality may also have changed significantly. METHODS We conducted a study intended to focus on clarifying the determinants of mortality in 405 consecutive patients who had undergone a Fontan operation (62 patients after an atriopulmonary connection, 105 after an intra-atrial rerouting, and 238 patients after an extracardiac rerouting) between 1979 and 2010. RESULTS The overall 1-year, 5-year, 10-year, and 15-year rates of survival were 87.1%, 84.6%, 83.4%, and 81.6%, respectively, and the type of procedure as well as heterotaxy syndrome, ventricular ejection fraction, and atrioventricular valve repair at the time of the Fontan operation were independent predictors of overall mortality (p < 0.05 for all). Heterotaxy syndrome and atrioventricular (AV) valve repair were independent predictors of early (less than 6 months postoperative) mortality and the type of procedure was an independent predictor of late (6 or more months postoperative) mortality (p < 0.05 to 0.01). In the era of intra-atrial rerouting, heterotaxy syndrome was the only independent predictor of total, early, and late mortality (p < 0.05 for all), whereas a low ejection fraction, AV valve repair, and repair of a total anomalous pulmonary vein connection, rather than heterotaxy syndrome, were independent predictors of total or early mortality or both (p < 0.05 for all) in the era of extracardiac rerouting. CONCLUSIONS Even in the modern era of extracardiac rerouting in the Fontan operation, a low ventricular ejection fraction, AV valve dysfunction, or a total anomalous pulmonary vein connection remain significant risk factors for mortality in patients with a single-ventricle physiology.

Changes in causes of sudden deaths by decade in patients with coronary arterial lesions due to Kawasaki disease

Over a 25-year period, we encountered 12 patients who died suddenly with coronary arterial lesions due to Kawasaki disease. We report their clinical course, and analyze the happenings of their deaths. Of the 12 patients, 10 were dead on arrival at hospital. Their age at death ranged from 13 months to 27 years, with a median of 16 years, and the interval from the onset of Kawasaki disease to death ranged from 2 months to 24 years. In 4 patients, death was found to be due to myocardial infarction, while in the remaining 8, it could not be determined. In 7 patients, coronary angiograms obtained less than 4 months after the acute onset of Kawasaki disease showed lesions bilaterally, most being giant aneurysms. Myocardial infarction had occurred in 6 patients prior to their death. In 1 patient of the late 1970s, who collapsed after running, cardiac sequels had not been suspected prior to autopsy. During the 1980s, 3 infants with bilateral giant aneurysms died within a year of the initial onset of Kawasaki disease, with acute myocardial infarction being the cause in 2 of them. In the late 1990s, and the 2000s, 5 patients died suddenly with left ventricular dysfunction, their ejection fractions being less than 40 percent more than 20 years after the initial onset of Kawasaki disease. Prior to their sudden deaths, they had had no cardiac events for many years, but had suffered previous myocardial infarctions. Multifocal premature ventricular contractions, and non-sustained ventricular tachycardia, are probable risk factors in such patients. Careful follow-up, checking for ventricular arrhythmia, is needed to prevent sudden death in patients suffering left ventricular dysfunction in the setting of Kawasaki disease.

Haemodynamic characteristics before and after the onset of protein losing enteropathy in patients after the Fontan operation

OBJECTIVES Determinant risk factors for developing protein losing enteropathy (PLE), including haemodynamics, remain unclear in patients after the Fontan operation. METHODS Our purpose was to characterize the serial PLE haemodynamics before and after the onset and to determine the risk factors based on the cardiac catheterization-based analysis. RESULTS Of 354 Fontan survivors who had undergone postoperative cardiac catheterizations, we experienced 26 PLE patients during the follow-up. Non-left ventricular morphology systemic ventricle, functional one-lung pulmonary circulation and an early postoperative high central venous pressure (CVP) were associated with the PLE onset and the high CVP (odds ratio (OR) = 1.19 per 1 mmHg, 95% confidence interval (CI) 1.04-1.37, especially ≥12 mmHg, OR = 3.09, 95% CI 1.25-7.64, P < 0.05 for both) and one-lung pulmonary circulation (OR = 10.0-10.5, P < 0.001) independently predicted the onset. At the time of the PLE onset, a Fontan route stenosis/obstruction, arrhythmias, ventricular dysfunction/heart failure and pulmonary arterio-venous fistulae were demonstrated in 10 (38%), 8 (31%), 4 (15%) and 3 (12%) patients, respectively. When compared with 56 excellent Fontan survivors, the high CVP, ventricular end-diastolic pressure, and pulmonary artery resistance, and the low arterial oxygen saturation, systemic artery pressure, and ventricular ejection fraction characterized the pre-PLE Fontan haemodynamics (P < 0.05-0.0001). However, the following intensive treatments reduced the CVP, systemic artery pressure and cardiac output (P < 0.05-0.01), resulting in haemodynamics no different from those of the excellent survivors, except for the low systemic pressure (P < 0.0001). CONCLUSIONS The pre-PLE haemodynamics was characterized by several impaired haemodynamics, while those after PLE only by a low systemic pressure. A high early postoperative CVP was the only haemodynamic predictor for a new onset of PLE. Strict selective criteria for the operation and strategies to eliminate CVP-raising factors are mandatory to prevent a new onset of PLE.

Feasibility and clinical impact of transcatheter closure of interatrial communications after a fenestrated Fontan procedure: Medium-term outcomes

This study was to review an institutional experience with transcatheter closure of Fontan fenestrations and its impact on clinical care.

The influence of morphological changes in amplatzer device on the atrial and aortic walls following transcatheter closure of atrial septal defects.

OBJECTIVE This study prospectively investigated morphological changes in Amplatzer Septal Occluder (ASO) over time and the influences of these changes on the atrial and aortic walls after atrial septal defect (ASD) closure. METHODS Between August 2005 and December 2007, 78 patients with ASD were treated with ASO devices and changes in the device shape, the device thickness, and relations of the discs to the atrial and aortic walls over time were evaluated by transesophageal echocardiography immediately and 3-12 months after deployment. RESULTS The maximum unstretched ASD diameter was 16.2 +/- 4.8 mm and the device diameter selected was 20.6 +/- 5.5 mm. At the time of last follow-up, the device thickness decreased by 17-33%, 6 of 26 devices with a flare shape on the aortic side developed a closed shape, and the relations of the discs to the anterior atrial and aortic walls changed from touching to intermittent compression in 14 of the 78 cases. In these 14 cases, the aortic rim was significantly smaller, the number of flared device shapes on the aortic side/the number of closed shapes immediately after deployment was significantly larger, and the maximum device thickness at the middle part was significantly more decreased than those in other cases. CONCLUSION As the device becomes thinner, loses its flexibility, and often changes from a flare-to-closed shape on the aortic side over time, the edges of ASO can start to compress the atrial and aortic walls. However, erosion was not recognized in these cases.

The clinical characteristics of sudden cardiac arrest in asymptomatic patients with congenital heart disease

Sudden cardiac arrest (SCA) is a major cause of death in patients with congenital heart disease (CHD). Systemic ventricular dysfunction is a reported risk factor for SCA. We retrospectively analyzed the medical records of 46 patients (age >6 years) who experienced SCA. The following underlying cardiac defects were observed: biventricular repair with affected subpulmonary right ventricle (n = 18, 39 %), biventricular repair with systemic right ventricle and Eisenmenger syndrome (n = 6 each, 13 %), Fontan circulation and unrepaired CHD (n = 5 each, 11 %), and others (n = 6, 13 %). Twenty-one patients (46 %) had no history of arrhythmias, and 21 of 43 (49 %) showed systemic ventricular ejection fraction >55 %. According to the New York Heart Association classification, 18 patients (39 %) were class I and 28 (61 %) were class II/III. SCA occurred at a younger age in class I (16 ± 5 years) than in the other classes (23 ± 10 years; P = 0.004). QRS duration was similar between the groups (136 ± 38 vs. 141 ± 50 ms; P not significant). Seven patients in class I (15 % of all SCAs) had no history of arrhythmias or features of hemodynamic abnormalities. The proportion of patients with biventricular repair and affected subpulmonary right ventricle was higher than that of patients with other defects, and the majority of SCA patients had more complicated defects than a simple repaired ventricular septal defect or an atrial septal defect. No symptoms of heart failure, history of arrhythmias, or features of hemodynamic abnormalities were observed in 15 % of the patients who experienced SCA. Prolonged QRS duration might be a predictor of SCA even in asymptomatic CHD patients. Prevention of SCA in CHD patients may require more detailed evaluation than is typically considered necessary.

Coil occlusion of PDA in patients younger than 1 year: Risk factors for adverse events

BACKGROUND Transcatheter occlusion of infantile patent ductus arteriosus (PDA) challenges the interventionist. PURPOSE To analyze the risk factors for adverse events from this procedure in patients younger than 12 months. SUBJECTS We retrospectively analyzed data on 32 patients younger than 12 months in whom transcatheter coil occlusion of a PDA was attempted. Ages ranged from 1 to 11 (median, 7) months and body weight from 1.2 to 10 (6.0) kg. The minimum ductal diameter ranged from 1.0 to 4.6 (3.3) mm and pulmonary to systemic flow ratio from 0.7 to 12.5 (2.2). Major adverse events were defined as those requiring surgery, while minor adverse events included transient hemolysis not needing treatment, coil migration with successful transcatheter retrieval, and mild left pulmonary artery (PA) stenosis. We determined whether any factors such as age, body weight, minimum PDA diameter, angiographic type, operator, and use of a 0.052-in. Gianturco coil related to the occurrence of adverse events. RESULTS In two patients, coils could not be deployed in the appropriate position. They underwent surgery following transcatheter retrieval of coils. Coils were successfully deployed in the other 30 patients, however, one patient developed persistent hemolysis that required surgical retrieval of the coils and closure. PDA was completely closed in the other 29 patients (clinical success rate, 91%). Thus, there were three major adverse events, while minor adverse events occurred in five patients. Univariate analysis did not identify any single factor that contributed to either major, minor, or total adverse events. However, all major adverse events occurred in patients under 6 months and less than 6 kg body weight with a minimum duct diameter of more than 3.5 mm. CONCLUSION Transcatheter coil occlusion of PDA is feasible in selected patients younger than 1 year. A minimum diameter more than 3.5 mm in patients under 6 kg may be a risk factor for major adverse events.

Management of ostium secundum atrial septal defect in the era of percutaneous trans-catheter device closure: 7-Year experience at a single institution

OBJECTIVES This study aimed to review the single institutional experience of the repair of secundum atrial septal defect (ASD) after the initiation of percutaneous trans-catheter device closure, to confirm the current management strategy and outcomes. METHODS From August 2005 to December 2012, a total of 1026 (659 females, age 27±21 years) consecutive patients underwent the repair of ASD. Including eight patients who converted to surgical repair, 317 patients (31%) underwent surgical repair and 709 (69%) underwent trans-catheter device closure. RESULTS An embolized device into the left atrium was surgically retrieved in one patient soon after trans-catheter device closure without any postoperative complications. The other patient developed left atrium to aorta fistula due to late erosion, and required the removal of implanted device and patch closure of fistula and ASD 3 months after trans-catheter device closure. Whereas serious central nerve system complications occurred in three patients after the surgical repair including a 75-year-old patient with postoperative transient atrial fibrillation who subsequently developed aspiration pneumonia and died; there were no mortalities and no morbidities associated with cranial nerve function after trans-catheter device closure. A number of patients approached through partial sternotomy with limited skin incision have increased per year, and the length of skin incision was 5.1±1.2cm in pediatric patients weighing less than 15kg (n=40), 6.9±1.9cm in the remaining pediatric patients (n=91), and 10.0±2.5cm in young adult females (n=10). CONCLUSION Percutaneous trans-catheter ASD closure was safely performed under the support of a surgical team. The cosmetic outcome of surgical closure is improving after initiation of partial sternotomy via limited skin incision for the pediatric population and young adult females. Prior to the treatment, the physicians must thoroughly inform patients and families of the advantages and disadvantages of both treatment options.

Potential goals for the dimensions of the pulmonary arteries and aorta with stenting after the Fontan operation

The purpose of this study was to clarify desired stent sizes for stenotic lesions in the post‐Fontan circulation. Using angiograms from 22 patients before and at late follow‐up (≥ 15 years) after the Fontan operation, we measured the maximum diameters of the proximal pulmonary arteries (PA) and the descending aorta. The diameters of the PA ipsilateral to the inferior vena cava, contralateral to the inferior vena cava, and descending aorta after the Fontan were 10.6–22.6 (15.8 ± 3.3), 8.0–19.1 (12.9 ± 3.1), and 12.1–18.9 (15.8 ± 2.0) mm, respectively, while the percent of normal predicted diameters (% N) were 55%–104% (70% ± 14%), 38%–99% (66% ± 17%), and 46%–74% (60% ± 7%), respectively. Despite somatic growth, the % N of all vessel diameters decreased significantly after the Fontan operation. In conclusion, smaller‐sized stents should be acceptable for both the pulmonary artery and descending aorta in the Fontan circulation. Cathet Cardiovasc Intervent 2002;56:246–253.

Late neointimal proliferation following implantation of stents for relief of pulmonary arterial stenosis.

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.