Predictors of liver cirrhosis and hepatocellular carcinoma among perioperative survivors of the Fontan operation.

OBJECTIVE: Fontan-associated liver disease (FALD) is widely recognised as a common complication in patients long after the Fontan operation. However, data on the predictors of FALD that can guide its screening and management are lacking. The present study aimed to identify the predictors of liver cirrhosis (LC) and hepatocellular carcinoma (HCC) in post-Fontan patients. METHODS: This was a multi-institutional retrospective cohort study. Clinical data of all perioperative survivors of Fontan operation before 2011 who underwent postoperative catheterisation were collected through a retrospective chart review. RESULTS: A total of 1117 patients (538 women, 48.2%) underwent their first Fontan operation at a median age of 3.4 years. Postoperative cardiac catheterisation was conducted at a median of 1.0 year. During a median follow-up period of 10.3 years, 67 patients (6.0%) died; 181 (16.2%) were diagnosed with liver fibrosis, 67 (6.0%) with LC, 54 (4.8%) with focal nodular hyperplasia and 7 (0.6%) with HCC. On multivariable analysis, high central venous pressure (CVP) (HR, 1.28 (95% CI 1.01 to 1.63) per 3 mm Hg; p=0.042) and severe atrioventricular valve regurgitation (HR, 6.02 (95% CI 1.53 to 23.77); p=0.010) at the postoperative catheterisation were identified as independent predictors of LC/HCC. CONCLUSIONS: Patients with high CVP and/or severe atrioventricular valve regurgitation approximately 1 year after the Fontan operation are at increased risk of developing advanced liver disease in the long term. Whether therapeutic interventions to reduce CVP and atrioventricular valve regurgitation decrease the incidence of advanced liver disease requires further elucidation.

Vascular occlusion with 0.035-inch hydrogel expandable coils in congenital heart diseases and vascular anomalies.

BACKGROUND: We present our experience with transcatheter vascular occlusion using 0.035-inch hydrogel expandable coils, which has been reported only in a few cases in the pediatric cardiology fields. METHODS: This study is a retrospective analysis of all patients who underwent transcatheter embolization with 0.035-inch hydrogel coils at the Department of Pediatrics, Okayama University Hospital, between October 2018 and September 2020. RESULTS: Twenty patients with a median age of 5.1 years (0.05-26.0 years) and a median weight of 13.8 kg (3.0-56.8 kg) were included. A total of fifty-four 0.035-inch hydrogel coils, including 35 Azur 35 and nineteen Azur CX 35 coils (Terumo, Tokyo, Japan), were successfully deployed in 22 target vessels. The target vessels consisted of 10 aortopulmonary collaterals, 8 veno-venous collaterals, and 4 pulmonary arteriovenous malformations. We achieved technical success in all the target vessels. In total, the mean target vessel diameter was 4.4 mm, the mean number of 0.035-inch hydrogel coils was 2.5 per vessel. The mean device to vessel ratio was 1.6 for the anchor coil and 1.2 for the additional coil. Post-implantation angiograms revealed that the primary occlusion rate was 18/22 (82%). There were no periprocedural complications. CONCLUSIONS: The 0.035-inch hydrogel expandable coils are effective and safe in patients with congenital heart disease and vascular anomalies. These occlusion devices could be valuable options for interventional pediatric cardiologists.

Stepwise Treatment for Heterotaxy Syndrome and Functional Single Ventricle Complicated by Infra-Cardiac Total Anomalous Pulmonary Venous Connection with Ductus Venosus Stent Placement and Subsequent Occlusion.

Even today, when the surgical outcome of congenital heart disease in the neonatal period has improved, the prognosis for heterotaxy syndrome and functional single ventricle complicated with total anomalous pulmonary venous connection (TAPVC), especially the infra-cardiac type, is catastrophic. We describe a strategy that combines percutaneous ductus venosus (DV) stent placement and occlusion after TAPVC repair to ensure survival from initial surgery to bidirectional cavopulmonary shunt (BCPS) procedure and facilitate subsequent treatment. Three consecutive patients with heterotaxy syndrome and functional single ventricle complicated by infra-cardiac TAPVC treated with our own strategy were retrospectively studied. In two infants, DV stent placement was performed on the day of birth. In one case at 11 days of age. The risk of pulmonary vein obstruction was reduced, and on-pump surgery, including TAPVC repair, was performed on a standby basis. Since the rapid increase in hepatic enzymes occurred on postoperative day 0 to 1 in all cases, percutaneous stent occlusion was performed until postoperative day 3. The procedure improved liver function. One patient died due to severe atrioventricular valve regurgitation, one case underwent BCPS, and one patient was waiting to undergo. DV stent placement can avoid TAPVC repair in the early neonatal period. After TAPVC repair, the portosystemic shunt remained, resulting in hepatic dysfunction, but this could be improved by stent and vertical vein occlusion. A series of stepwise treatments can be useful to help such critically ill infants survive the high-risk neonatal period and achieve good BCPS circulation.

Higher preoperative Qp/Qs ratio is associated with lower preoperative regional cerebral oxygen saturation in children with ventricular septal defect.

PURPOSE: The relationship between regional cerebral oxygen saturation (rSO2) and the amount of left-to-right shunt in ventricular septal defect (VSD) patients has not yet been investigated. The purpose of this study was to identify the association of preoperative pulmonary to systemic blood flow (Qp/Qs) ratio and preoperative rSO2 in patients with VSD. METHODS: We retrospectively evaluated 49 VSD surgical closure candidates at a single institution. Preoperative Qp/Qs ratio was compared with rSO2 measurements at the time of VSD closure surgery. RESULTS: Forty-nine were eligible for the final analysis. The median age at surgery was 6 (interquartile range [IQR]: 3, 12) months, and 36.7% were male. Atrial septal defects coexisted in 51.0%. There were no genetic abnormalities except trisomy 21 in 32.6% of the patients. Pulmonary hypertension was found in 42.8%. The median Qp/Qs ratio, calculated based on catheter testing results before the surgery, was 2.7 (IQR: 2.1, 3.7). Postoperative rSO2 was significantly higher than preoperative values (52.2 ± 12.9, 63.5 ± 13.1%, p < 0.001). There was an inverse relationship of Qp/Qs and preoperative cerebral rSO2 (r = - 0.11, p = 0.02). CONCLUSION: A higher Qp/Qs ratio was associated with a lower preoperative cerebral rSO2 in pediatric patients with VSD.

Perioperative Milrinone Infusion Improves One-Year Survival After the Norwood-Sano Procedure.

OBJECTIVES: The aim of this study was to investigate whether milrinone infusion improved one-year survival in patients who underwent the Norwood-Sano procedure. DESIGN: A retrospective observational study. SETTING: A single-institution university hospital. PARTICIPANTS: Children who underwent the Norwood-Sano procedure from January 2008 to December 2014. INTERVENTIONS: Patients were categorized into two groups: group E+D, who received routine epinephrine and dopamine infusion, and group M, who received routine milrinone infusion for intra- and postoperative inotropic support. MEASUREMENTS AND MAIN RESULTS: The primary outcome of this study was one-year survival after the Norwood-Sano procedure. A total of 45 patients were included (group E+D, 22; group M, 23). One-year survival in group M was significantly higher than that in group E+D (95.7% [22/23] v 72.7% [16/22], p = 0.03). A Kaplan-Meier curve also showed that one-year survival in group M was significantly higher than that in group E+D (p = 0.04), from the result of the log-rank test. The number of patients who had any arrhythmias in the intensive care unit (ICU) was significantly lower in group M than in group E+D (21.7% [5/23] v 50% [11/22], p = 0.03). The duration of ICU stay did not have statistical difference between groups (group M 19; interquartile range [IQR], 15-28) v group E+D 19.5 (IQR, 16.3-35.5) days, p = 0.57). CONCLUSIONS: Perioperative milrinone infusion improved the mortality after the Norwood-Sano procedure. Potential advantages of milrinone compared with epinephrine are fewer arrhythmias and better systemic perfusion, which could decrease lethal cardiac events in the ICU.

Balloon atrial septostomy in hypoplastic left heart syndrome with restrictive atrial septum.

BACKGROUND: Rashkind balloon atrial septostomy (BAS) can be challenging in infants with hypoplastic left heart syndrome (HLHS) and small atrial septal defect (ASD). METHODS: We retrospectively reviewed all infants with HLHS who underwent surgery and BAS between January 2006 and December 2015. The infants were divided into three groups: no BAS; catheter BAS; and open AS. Infants who underwent catheter BAS were divided into two groups based on atrial septal anatomy: standard and complex. RESULTS: Of the 70 patients, 57 (81%) underwent Glenn surgery. Subsequently, a significant difference in survival was observed: 86% (44/51), 91% (10/11), and 25% (2/8) in the no BAS, catheter BAS, and open AS groups, respectively (P = 0.0002). No significant difference was seen between the no BAS and the catheter BAS groups (P = 1.0). In the 56 patients who underwent catheterization after surgery, no intergroup differences in mean pulmonary artery pressure, pulmonary vascular resistance, or pulmonary artery index were found. We classified catheter BAS into standard (n = 5) and complex (n = 5) based on ASD location, and septum thickness. All patients in the standard group underwent complete Rashkind BAS, but in the complex group, only one patient underwent complete Rashkind BAS, with the remaining requiring initial static BAS (P = 0.048). Following septostomy, ASD size, ASD flow, and percutaneous oxygen saturation (SpO2 ) were not significantly different between the two groups. CONCLUSIONS: Catheter BAS is effective in infants with HLHS and a restrictive atrial septum. Infants with standard or complex atrial septum can achieve equivalent outcomes despite more patients often requiring static BAS.

Morphological and Hemodynamic Effectiveness of Stenting for Pulmonary Artery Stenosis　- Subanalysis of JPIC Stent Survey.

BACKGROUND: Percutaneous stenting for branch pulmonary artery stenosis is an established interventional choice in congenital heart disease. The apparent morphologic change in the vessel diameter often differs from the hemodynamic result. METHODS AND RESULTS: We performed a subanalysis of the data from the Japanese Society of Pediatric Interventional Cardiology (JPIC) stent survey. The factors that may have contributed to morphologic effectiveness included reference vessel diameter (RVD), minimum lumen diameter (MLD) and percent diameter stenosis (%DS) and the relation between morphologic and hemodynamic effectiveness was evaluated in 206 lesions treated with stenting. We defined a "50% increase in MLD" as "morphologically effective", while "achievement of either a reduced pressure gradient greater than 50% or an increase of perfusion ratio to the affected side to the contralateral side greater than 20%" as "hemodynamically effective". Morphologic effectiveness was achieved in 84% of patients. Before stenting, %DS was significantly larger, while RVD was smaller in the "effective" group than in the "non-effective" group. The cutoff value for effective stenting was 51% for %DS and 14.7 mm for RVD before stenting. Hemodynamic effectiveness was obtained more often in the "morphologic effective" group. CONCLUSIONS: RVD and %DS were the 2 main contributors to acute morphologic effectiveness. There was a significant relationship between "morphologic effectiveness" and "hemodynamic effectiveness", judging from increased perfusion of the affected lung and/or decreased pressure gradient. (Circ J 2016; 80: 1852-1856).

A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum.

OBJECTIVES: To achieve the growth of right-sided heart structures, our choice of the first palliation for patients with pulmonary atresia and intact ventricular septum (PA-IVS) includes a modified Blalock-Taussig shunt (BTS) with pulmonary valvotomy. We sought to analyse the impact of the first palliation on the growth of right-sided heart structures and factors associated with a choice of definitive surgical procedure. METHODS: Fifty patients with PA-IVS who underwent a staged surgical approach from 1991 to 2012 were retrospectively reviewed. RESULTS: Right ventricular (RV)-coronary artery fistulas were seen in 42% of patients at the time of birth. All 50 patients had a modified BTS with pulmonary valvotomy. Six patients died after the first palliation or inter-stage. Thirty patients achieved a biventricular repair (BVR group), 6 patients had a 1 + 1/2 ventricular repair (1 + 1/2V group) and 5 patients had a Fontan completion (Fontan group). After modified BTS with pulmonary valvotomy, tricuspid valve z-score did not increase in any of the group (BVR: pre -2.79 vs post -2.24, 1 + 1/2V: pre -5.25 vs post -6.69, Fontan: pre -6.82 vs post -7.94). Normalized RV end-diastolic volume increased only in BVR group after modified BTS with pulmonary valvotomy (BVR: pre 32% vs post 64%, 1 + 1/2V: pre 43% vs post 42%, Fontan: pre 29% vs post 32%). Major RV-coronary artery fistula was a strong factor with proceeding single-ventricle palliation [BVR: 4/30 (13%) patients, 1 + 1/2V: 1/6 (17%) and Fontan: 4/5 (80%)]. CONCLUSIONS: Tricuspid valve growth was not obtained by modified BTS with pulmonary valvotomy; therefore, tricuspid valve size at birth appeared to be a predictor for achieving BVR. Proportionate RV growth was seen only in patients who achieved BVR. However, RV growth was not seen in patients having 1 + 1/2 ventricular repair. Major RV-coronary artery fistula was a strong predictor for proceeding single-ventricle palliation.

Current trends in stenting for aortic coarctation in Japan: Subanalysis of Japanese Society of Pediatric Interventional Cardiology (JPIC) stent survey.

BACKGROUND: Stenting for aortic coarctation (CoA) has been accepted as an alternative to surgery for adolescents and adults, but only a few case have been reported in Japan. The purpose of this study was to provide a detailed review of Japanese national data on stenting of CoA. METHODS: In a subanalysis of the data of the Japanese Society of Pediatric Interventional Cardiology (JPIC), we identified 35 patients with CoA who underwent stenting. We analyzed procedural characteristics including factors that may have contributed to hemodynamic effectiveness, and we compared these parameters between the patients under and over 15 years of age. RESULTS: The mean ratio of balloon diameter/minimum lumen diameter (MLD) before stenting was 1.7 (range, 1.2-4.0), and the mean difference between the balloon diameter and the reference vessel diameter was -0.7 mm (range, -5.0 to +3.0 mm). %MLD/balloon diameter, which was defined as [(balloon diameter - MLD after dilation)/balloon diameter] × 100 predicted achievement of <10 mmHg pressure gradient after stenting. The sensitivity and the specificity of its cut-off of 7% were 93% and 47% (AUC, 0.7), respectively. There was no statistical difference between the two age groups under and over 15 years of age, in terms of selection criteria of stent size, balloon type used for deployment and immediate angiographic and hemodynamic result. CONCLUSIONS: Stenting for CoA was clinically effective with few complications in Japan, even in patients not fully grown.

Stenting for pulmonary artery stenosis complicated by univentricular physiology: subanalysis of JPIC stent survey.

BACKGROUND AND PURPOSE: Stent implantation is an important treatment option for pulmonary artery stenosis (PS), even if complicated by univentricular physiology (UVP). However, there is paucity of evidence concerning not only its hemodynamic and morphologic indications but also on markers for its optimal target attainment in UVP. The purpose of this study was to evaluate the acute outcome and factors associated with efficacy of stenting for PS complicating UVP. METHODS AND SUBJECTS: A subanalysis was performed using the data of the Japanese Society of Pediatric Interventional Cardiology (JPIC) stent survey. We analyzed the morphologic and hemodynamic data of 11 patients with UVP who underwent stenting for PS. We defined "a 50% increase in the minimum lumen diameter (MLD)" as "morphologically effective," and "an achievement of 0 mmHg pressure gradient" as "hemodynamically effective." We analyzed the success rate for each criterion and determined factors which may have contributed to hemodynamic effectiveness. RESULTS: Stenting was morphologically effective in all patients, while it was hemodynamically effective in 6/11 (55%). The percent diameter stenosis after stenting was significantly lower in the "hemodynamically effective" group (2.5 ± 5.5% vs 19.6 ± 13.1%, p=0.017). The cutoff value of percent diameter stenosis after stenting to "hemodynamically effective" was 14.6%; the sensitivity was 80% and the specificity was 100% (area under the curve 0.825, p=0.021). CONCLUSIONS: The percent diameter stenosis after stenting significantly contributed to achieving a "0 mmHg" pressure gradient, while in order to achieve a "0 mmHg" pressure gradient, the residual percent diameter stenosis should be less than around 15%.

Double-barrel Damus-Kaye-Stansel operation is better than end-to-side Damus-Kaye-Stansel operation for preserving the pulmonary valve function: the importance of preserving the shape of the pulmonary sinus.

OBJECTIVE: The Damus-Kaye-Stansel operation sometimes results in deteriorating semilunar valve insufficiency. We verified the semilunar valve function after the Damus-Kaye-Stansel operation and compared the end-to-side Damus-Kaye-Stansel with the double-barrel Damus-Kaye-Stansel. METHODS: Forty-seven patients who underwent the Damus-Kaye-Stansel operation between June 1993 and August 2008 were retrospectively reviewed. Any patient who underwent a Norwood-type operation was excluded. The median age at operation was 19 months (range, 0-276 months). Forty-five patients were Fontan candidates. Thirty-nine patients underwent pulmonary artery banding before the Damus-Kaye-Stansel operation. Twenty-two patients had undergone an arch repair previously. The semilunar valve function was evaluated by echocardiography. RESULTS: Thirteen patients underwent the end-to-side Damus-Kaye-Stansel operation, and 34 patients underwent the double-barrel Damus-Kaye-Stansel operation. The mean follow-up period was 71 ± 50 months (range, 1-188 months). Although there were 4 deaths, no death was related to the Damus-Kaye-Stansel procedure. Two of the patients with early death could not undergo a postoperative evaluation of the semilunar valves. The semilunar valve regurgitation mildly deteriorated in 7 patients (pulmonary regurgitation in 5 patients and aortic regurgitation in 2 patients). Pulmonary regurgitation deteriorated from none to mild in 1 patient, none to trivial in 2 patients, and trivial to mild in 2 patients. Both deteriorations in aortic regurgitation ranged from none to trivial. Semilunar valve regurgitation did not affect patients' circulatory condition. The end-to-side Damus-Kaye-Stansel operation more frequently caused a deterioration in pulmonary regurgitation than the double-barrel Damus-Kaye-Stansel operation (4/11 vs 1/34, P = .001). No surgical intervention for a systemic ventricular outflow obstruction was observed in the follow-up period. CONCLUSIONS: The double-barrel Damus-Kaye-Stansel operation was found to be superior to the end-to-side Damus-Kaye-Stansel operation for the prevention of postoperative pulmonary regurgitation.

Growth of the lateral tunnel in patients who underwent a total cavopulmonary connection at less than 5 years of age.

OBJECTIVE: A lateral tunnel-total cavopulmonary connection (LT-TCPC) using a right atrial (RA) free wall is the first choice of treatment for patients with a small body weight in this institute. Whether the growth of the LT is appropriate or not according to the growth of the body remains controversial. In addition, the optimal initial diameter of an LT is unknown. The purpose of this study was to verify the growth of the LT. METHODS: Ninety-one patients of a total of 267 TCPC cases underwent an LT-TCPC at less than 5 years of age in this institute between March 1991 and June 2008. The data on 47 of the 91 patients, which were available to investigate the LT growth, were retrospectively reviewed. The mean age at LT-TCPC was 37+/-11 months (16-57 months). The mean body weight at TCPC was 12.4+/-2.4 kg (7.6-20.0 kg). The initial LT diameter was determined with Hegar's sizer of the estimated half-pulmonary arterial (PA) diameter, which is a diameter that results in half of the dimension of the normal pulmonary valve. The measured maximum LT diameter (mm) divided by the estimated half-PA diameter (mm) was considered as the LT index. The size of the LT was evaluated using either echocardiography or angiography. RESULTS: The mean follow-up period was 7.4+/-3.5 years (1.6-13.5 years). The LT index was initially 2.0+/-0.7 (1.3-4.5), 2.0+/-0.4 (1.3-3.2) at 1 year after operation, 2.1+/-0.5 (1.5-3.2) at 5 years after operation, 1.9+/-0.4 (1.5-2.8) at 10 years after operation and 2.1+/-0.5 (1.6-2.5) at 13 years after operation, respectively. CONCLUSIONS: LT growth suitable for the body growth can be expected. Although there was some variation in the initial LT diameter, the LT index tended to converge at 2.0 with growth.

The impact of the length between the top of the interventricular septum and the aortic valve on the indications for a biventricular repair in patients with a transposition of the great arteries or a double outlet right ventricle.

The purpose of this study was to establish a useful cut-off level for performing an original Rastelli-type operation in patients with transposition of the great arteries (TGA)/ventricular septal defect (VSD) or double outlet right ventricle (DORV). A total of 43 patients with TGA/VSD or DORV who underwent an original Rastelli-type operation in this institute between March 1993 and January 2009 were reviewed retrospectively. These patients were divided into two groups using the length between the top of the interventricular septum and the aortic valve (IVS-AV length); Group A; IVS-AV length <80% of normal left ventricular end-diastolic diameter (LVDd). Group B; IVS-AV length > or =80% of normal LVDd. Group A had a significantly better survival than Group B (100% vs. 56%, P=0.001). The cardiac event-free survival were 89.1% at 7.2 years in Group A and 26.3% at 8.4 years in Group B (P<0.0001). The Group B had a higher incidence of left ventricular outflow tract obstruction (LVOTO; 3% vs. 33%, P=0.02). The IVS-AV length was found to be a significant risk factor for mortality and LVOTO. The IVS-AV length should, therefore, be taken into consideration when selecting the optimal surgical procedures for these patients.

Clinical outcome of the Fontan operation in patients with impaired ventricular function.

OBJECTIVE: Although a staged Fontan strategy allows for an excellent outcome in high-risk patients, an impaired ventricular function remains a significant factor of early/late mortality and morbidity. This study evaluated the clinical outcome of the Fontan operation in patients with impaired ventricular function. METHODS: A retrospective review was performed on 217 patients who had undergone the Fontan operation between 1991 and 2007. RESULTS: Twenty-nine (13%) of the 217 patients had an impaired ventricular function (ejection fraction (EF) <50%). The median age at the time of the operation was 3 (range: 1-31 years) years. There were five adult patients. The ventricular morphology was right in 20 patients (including five hypoplastic left heart syndrome (HLHS)) and others (left and two-ventricle) in nine patients. Heterotaxy syndrome was present in eight patients. Previous surgical interventions included bidirectional Glenn anastomoses in 24, modified Blalock-Taussig shunts in two and pulmonary artery banding in two. The preoperative EF was 43+/-6%. Significant (moderate or severe) atrioventricular valve regurgitation was noted in four patients. The percutaneous oxygen saturation (SpO(2)) was 82+/-5%. The pulmonary artery pressure and pulmonary artery index were 11+/-3 mmHg and 296+/-102 mm(2)m(-2), respectively. All 29 patients underwent the Fontan operation without any early mortality. There were two late mortalities and two re-operations. EF was maintained at 59+/-15% at a median follow-up of 7.5 (range: 1-19) years. The percent normal systemic ventricular end-diastolic volume decreased from 174+/-95% to 124+/-39% (p<0.05). The SpO(2) increased to 92+/-2%. The mean cardiothoracic ratio in chest X-ray and B-type natriuretic peptide were 51% (range: 35-68%) and 22 pgml(-1) (range: 9-382 pgml(-1)), respectively. Three patients developed congestive heart failure, seven had arrhythmia and two developed protein-losing enteropathy. The New York Heart Association (NYHA) class functional class is I in 21 patients, II in five and III in one. CONCLUSIONS: Acceptable clinical outcomes were observed at an intermediate follow-up of the Fontan operation in patients with an impaired ventricular function.

Differences in right and left ventricular remodeling after transcatheter closure of atrial septal defect among adults.

OBJECTIVES: To evaluate acute cardiac remodeling after transcatheter closure of atrial septal defect (ASD) in adult patients. BACKGROUND: In adult patients with ASD, longer periods of cardiac adaptation should be expected after the procedure due to long-standing RV volume overload and subsequent changes in the pulmonary vasculature. There are limited reports about this remodeling in adult patients. METHODS: We prospectively enrolled 17 adults (mean age 58.4 +/- 17.3 years) who underwent successful transcatheter closure of their ASDs from August 2005 to July 2006. We performed routine transthoracic echocardiographic studies, including LV and RV myocardial performance indices, or Tei indices, and plasma brain natriuretic peptide (BNP) sampling before closure of the ASD, and 1 day, 1 month, and 3 months after closure. RESULTS: We found (1) LV end diastolic diameter increased, and RVEDD decreased markedly after the closure; (2) differences existed in LV and RV adaptation. While LV Tei index improved soon after the procedure, RV Tei index worsened until 1 month after the procedure, then recovered by the 3 month follow-up visit; and (3) BNP elevated 1 day after closure of the ASD and declined by the 1-month follow-up visit. CONCLUSION: "Shrinkage" of the RV and "expansion" of the LV occurred soon after the procedure, even in elderly patients. Device closure of ASDs caused rapid improvement of LV function, but RV function underwent transient deterioration, probably due to delayed changes in RV ventricular mass in the face of acute volume reduction in this aged cohort.

Changes in the aortic and pulmonary diameters in a fetus with hypoplastic left heart syndrome.

We diagnosed hypoplastic left heart syndrome in a 26-week-old fetus using fetal echocardiography. Color Doppler ultrasonography was helpful for evaluating the structural abnormalities. The diameters of the aorta and the pulmonary artery were measured periodically from 26 to 38 weeks of gestation. Aortic diameter was below the normal range throughout gestation. The diameter of the pulmonary artery was normal at 26 weeks of gestation but gradually dilated and was abnormally dilated after the 36th week of gestation. Here we discuss the cause of enlarged pulmonary artery in fetal hypoplastic left heart syndrome.