A 16-year-old male with complete congenital atrioventricular block since 1 year old was treated by epicardial pacemaker lead implantation. At 9 years old, he was diagnosed with supravalvular pulmonary artery stenosis resulting from epicardial pacemaker leads with cardiac strangulation as the possible cause. Transient ST change was seen only in the exercise tolerance test, with no chest pain, indicating that myocardial ischemia was absent. The deterioration status of supravalvular pulmonary artery stenosis was assessed by catheterization. With his arms raised, he was injected with a small amount of contrast media, suddenly, he lost consciousness. Electrocardiography showed ST segment alterations after frequent premature ventricular contractions, followed by junc-tional bradycardia that rapidly progressed to asystole. Hence, transient ischemia caused by arm raising or frequent premature ventricular contractions and deterioration of the coronary artery compression was suspected. The leads were then removed in an urgent surgery. In conclusion, contrast-enhanced computed tomography should be conducted in patients with cardiac strangulation, and earlier lead exchange should be performed because of the rapid deterioration of myocardial ischemia.

Patients with Down’s syndrome (DS) are generally regarded as not being good candidates for the Fontan procedure. However, detailed hemodynamic changes over time are not fully clarified. A retrospective chart review of all patients with DS who underwent the Fontan procedure and 5 times that number of Fontan patients without DS performed in Fukuoka Children’s Hospital and Kyushu University Hospital. Seven Fontan patients with DS were identified, and 35 Fontan patients without DS were recruited. During the mean observational periods of 14.7 years and 15.0 years (DS and non-DS, respectively) after the Fontan procedure, only one DS patient died. Central venous pressure (CVP) and transpulmonary pressure gradient significantly increased, and arterial oxygen saturation significantly decreased over time in DS patients after the Fontan procedure compared with those without DS. CVP in DS patients after the Fontan procedure increased over time compared with non-DS patients. Better management including the efficacy of Pulmonary arterial hypertension-specific therapy should be clarified in further studies.

An intractable pleural effusion is a common comorbidity of a Fontan operation, occasionally leading to undesirable outcomes. The preventive effect of aortopulmonary collateral (APC) coil embolization against a pleural effusion before a Fontan operation is still controversial. This is a retrospective single-center study; among 227 Fontan cases, 57 cases with complete MRI data were analyzed at first. Factors associated with the duration of pleural drainage (median: 6 (2-41) days) and that of postoperative hospital stay (median: 25 (14-91) days) were analyzed using a multiple regression analysis. The pulmonary artery index (PAI; Nakata index) was associated with both the pleural drainage duration (P < 0.05. r(2) = 0.17) and postoperative hospital stay (P < 0.05, r(2) = 0.10). Thereafter, all the 227 patients were classified into the following three groups: Group A (12 patients in whom the embolization was performed within 30 days before the Fontan surgery), Group B (131 patients in whom the embolization was performed more than 30 days before the Fontan surgery), and Group C (84 patients in whom the embolization was not performed). Patients in Group A were found to be associated with the shortest length of both periods (P < 0.05). Lower PAI values were related to a prolonged pleural drainage duration and postoperative hospital stay. APC coil embolizations may reduce the risk if they are performed shortly (less than 30 days) before the operation.

Background: Recently, Fontan operation using extracardiac conduit (EC) has grown to be the most common procedure. However, as the existing history is relatively short, prevalence rates of complications have not been clarified.Methods: A retrospective chart review of 644 consecutive patients undergoing Fontan operation with EC was performed in Fukuoka Children's Hospital and Kyushu University Hospital.Results: " The 10-year and 20-year survival rates were 96.8% and 91.9%, while the 10-year and 20-year freedom rates from Fontan failure were 92.3% and 90.5%. The 20-year cumulative incidence of tachyarrhythmia, bradyarrhythmia, stroke, hemoptysis, acute decompensated heart failure (ADHF), and protein-losing enteropathy (PLE) were 6.6%, 4.0%, 4.9%, 3.1%, 15.0% and 3.9%, respectively. Those with bradyarrhythmia, ADHF, and hemoptysis had a significantly worse outcome in developing Fontan failure. Risk factors for these complications included hypoplastic left heart syndrome, heterotaxy syndrome, previous surgical pulmonary arterial plasty, pacemaker implantation, atrioventricular valvular replacement, higher age at the Fontan procedure, fenestration of the conduit, lower oxygen saturation, higher Fontan pressure, smaller pulmonary arterial size, and moderate to severe atrioventricular valvular regurgitation after the Fontan procedure.Conclusions: Though the long-term survival is acceptable, substantial prevalence of complications was shown among patients receiving Fontan operation with EC. Since some of the complications were associated with developing Fontan failure, they should be monitored carefully.

In the Fontan circulation, there is a substantial degree of systemic-to-pulmonary collateral flow (SPCF), which can be measured by cardiac magnetic resonance (CMR). However, the correlation between the degree of SPCF and long-term outcomes is not fully understood. We retrospectively studied 321 patients who underwent the Fontan procedure and CMR at a single center. Using CMR, we calculated SPCF as pulmonary blood flow − systemic blood flow. %SPCF was defined as SPCF ÷ pulmonary blood flow. The mean age of patients at CMR was 14.3 ± 7.5 years. The average %SPCF was 13.0% ± 11.0%. With a multivariate analysis, %SPCF was significantly correlated with time (i.e., the longer the time period since the Fontan procedure, the lower the %SPCF) (p = 0.006), previous total anomalous pulmonary vein drainage (p = 0.007), a low pulmonary artery index (Nakata index) before the Fontan procedure (p = 0.04), and older age at the time of the Fontan procedure (p = 0.002). Regarding the findings after the Fontan procedure, %SPCF was significantly correlated with ventricular end-diastolic volume (p < 0.001), ventricular end-systolic volume (p < 0.001), central venous pressure (p < 0.001), plasma brain natriuretic peptide concentration (p < 0.001), hemoptysis (p = 0.009), and poor New York Heart Association functional class (p = 0.007). SPCF was correlated with clinical condition after the Fontan procedure. The importance of sufficient growth of the pulmonary vascular bed should be emphasized because the development of SPCF is believed to result from the poor condition of the pulmonary circulation.

BACKGROUND Liver stiffness on ultrasound shear-wave elastography (SWE) reflects central venous pressure (CVP) in adult patients with heart failure, but the association of liver stiffness on SWE with CVP in pediatric patients is not clear. The present study evaluated whether liver stiffness on SWE is useful as a non-invasive indicator of CVP in pediatric patients.Methods and Results:Liver stiffness was measured using ultrasound SWE in 79 patients aged <20 years with congenital heart diseases. None of the patients was found to have liver disease. Correlations between liver stiffness and other clinical variables, including CVP, were analyzed. CVP was the only factor independently and significantly correlated with liver stiffness in multivariate analysis. However, variables related to hepatic fibrosis did not correlate with liver stiffness. CONCLUSIONS Liver stiffness on ultrasound SWE is useful as a non-invasive indicator of CVP in children with heart diseases.

BACKGROUND:Pacemaker implantation in patients with single ventricle is associated with poor outcomes.OBJECTIVE:The purpose of this study was to determine the reasons for the poor outcomes of pacemaker implantation.METHODS:We performed a retrospective chart review of patients with single ventricle who had undergone permanent pacemaker implantation. Patients were categorized into 3 groups based on the site of pacing and the proportion of ventricular pacing (VP) as follows: (1) atrial pacing group with atrial pacing only (n = 11); (2) low VP group with low daily VP proportion (<50%; n = 12); and (3) high VP group with high daily VP proportion (≥50%; n = 15). Pacing leads were placed at the epicardium in all patients.RESULTS:No patients in the atrial pacing or low VP groups died, whereas the survival rate in the high VP group was 58.9% and 39.3% at 10 and 20 years, respectively, after pacemaker implantation. Among the post-Fontan patients, plasma brain natriuretic peptide (BNP) levels significantly increased with the proportion of VP: 11.7, 20.3, and 28.4 pg/mL in the atrial pacing, low VP, and high VP groups, respectively (P = 0.04). In the high VP group, the plasma BNP level was significantly lower in patients with an apical pacing lead than in those with a nonapical pacing lead (27.0 pg/mL vs 82.8 pg/mL, respectively; P = .03).CONCLUSION:A higher proportion of VP was associated with poor outcome and higher plasma BNP levels, probably due to ventricular dyssynchrony. In epicardial ventricular pacing, apical pacing is better to avoid the increase in ventricular stress and plasma BNP level.

The exercise tolerance of Fontan patients is poorer than that of healthy people. Some previous studies reported that exercise tolerance can be improved in this population by cardiac rehabilitation. This study aimed to determine the factors associated with peak oxygen uptake (VO2) and to subsequently clarify the correlation between participation in sports club activities and variables related to exercise tolerance. Cardiopulmonary exercise tests were performed by 115 Fontan patients aged between 6 and 20 years. The patients completed questionnaires on their daily physical activities including participation in extracurricular sports clubs in middle (junior high school) and high school. Peak VO2 had a significant negative correlation with age and a positive correlation with hemoglobin, stroke volume, and resting tidal volume in the entire study group. Additionally, the sports club participants who were middle and high school students had significantly higher peak VO2 and resting tidal volume. Exercise habits including participation in sports club activities may promote exercise tolerance by improving respiratory function in Fontan adolescents.

Background Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear. Purpose We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants. Method and results We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ± 7 years old, follow-up: 18 ± 6 years). The New York Heart Association (NYHA) functional class I and II was 51% and 42%, respectively. During the follow-up period of 4.1 ± 1.6 years, 33 patients died, and the 5-year survival rate was 93.5%. The mode of death was heart failure in 11 patients (34%), arrhythmia or sudden death in 8 (24%), cancer in 5 (15%), perioperative problems and hemostatic problems in 4 each (12% for each), and infection in 1 (3%). Left isomerism, prior hospitalization, protein losing enteropathy (PLE), pulmonary arteriovenous fistulae, NYHA functional class, impaired hemodynamics, hyponatremia, hepatorenal dysfunction, and use of diuretics were associated with a high mortality rate (p < 0.05–0.0001). Further, PLE (hazard ratio [HR]: 14.4), left isomerism (HR: 3.5), and NYHA (HR: 2.4) independently predicted a high 5-year high mortality (p < 0.05 for all). The incidence of cancer-related mortality increased markedly with age >40 years. Conclusions Majority of the Japanese adult Fontan survivors had good functional status, with an acceptable 5-year survival rate. However, the significant prevalence of non-cardiac mortality highlights Fontan pathophysiology as a multi-organ disease that requires a multidisciplinary management strategy to improve the long-term outcome.

背景：単心室疾患群では，体肺動脈側副血管（APCA）の増加がFontan術後の合併症につながるため，術前にコイル塞栓を行うことがある．その目標については術後のAPCAの推移も考慮することが必要である．

Background: Although progress in perioperative management and surgical treatment has improved the survival rate of patients with hypoplastic left heart syndrome (HLHS), the Fontan completion rate remains low and prognosis is poor.Analysis of prognostic factors is challenging because HLHS complicates hemodynamics in patients with typical HLHS without ventricular septal defect and nontypical HLHS.This study aimed to categorize typical HLHS into four groups with combinations of stenosis/atresia of the mitral valve/aortic valve and to investigate survival rates and mortality risk factors to improve Fontan completion and prognosis.Methods: We enrolled 119 patients with HLHS [mitral valve atresia (MA)/aortic valve atresia (AA): 61 patients; MA/aortic valve stenosis (AS): 4; mitral valve stenosis (MS)/AA: 24; and MS/AS: 30)].After excluding the MA/AS group because of a small number of patients, we investigated the survival rate, number of mortalities, and cause of death in the MA/AA, MS/AA, and MS/AS groups by using prenatal diagnosis, MS/AA, low birth weight, atrioventricular valvular insufficiency, noncardiac complications, restricted foramen ovale, and length of intensive care unit stay following initial surgery as variables.Results: The overall 5-year survival rate for typical HLHS was 58％, which significantly varied by subtype (MS/ AS: 72％; MA/AA: 59％; and MS/AA: 33％; p＝0.002).Multivariate analysis identified MS/AA (p＝0.002) and positive prenatal diagnosis (p＝0.03) as significant prognostic risk factors. Conclusion:In typical HLHS, MS/AA was found to be an adverse prognostic factor.Because prognosis after total cavopulmonary connection was similar to those of other groups, preoperative diagnosis and treatment strategies before right heart bypass must be improved.

Most patients with single-ventricle (SV) heart disease can now reach adulthood after Fontan operation. Acute decompensated heart failure (ADHF) is one of the serious complications after Fontan operation, however, its incidence and outcome are not well understood. The study aims to investigate the incidence and clinical characteristics of patients diagnosed with ADHF requiring hospitalization after Fontan procedure in our institutions. A retrospective analysis of 655 patients (387 males) who have completed Fontan procedure in Fukuoka Children's Hospital (FCH) between 1984 and 2015 and followed at FCH and Kyushu University Hospital were conducted. The Fontan procedures included extracardiac conduit (82.7%), lateral tunnel (13.4%), atriopulmonary connection (2.3%), and others (1.5%). The mean observation period after Fontan procedure was 9.8 ± 6.8 years (0 - 31.6 years). Twenty-one patients (3.2%) were hospitalized due to right-sided ADHF with a mean time of 9.4 ± 6.6 years after Fontan procedure. Among them, 7 patients (1.1%) died and the most common cause of death was multiple organ failure. By univariate analysis, older age at Fontan operation, SV dysfunction, significant atrioventricular valve regurgitation, and asplenia were associated with the development of ADHF. In conclusion, ADHF is less common, but it remains a devastating complication after Fontal procedure.

With the advancement of congenital heart disease management, most patients after Fontan procedure can now reach adulthood. However, the long-term outcome and risk factors for major adverse events a...

resonance imaging (MRI) confirmed the severity of the atrioventricular valve regurgitation, and repair was scheduled.On attempt to clarify the morphology of the valve and etiology of the regurgitation prior to surgery, this was difficult to visualize on transthoracic and transesophageal echocardiography (3-D echocardiography was unavailable because of the patient's low weight: 17 kg).MRI was also unable to show the fine structure of the valve.Subsequently, dual-source 128-slice multidetector computed tomography (CT; Siemens SOMATOM Definition Flash) and 4-D imaging were performed.Data were reconstructed in 20 separate phase increments encompassing the entire cardiac cycle, ranging ollowing Fontan procedure, an 8-year-old boy was diagnosed with severe atrioventricular valve regurgitation and poor ventricular function at routine followup on transthoracic echocardiography.He was previously diagnosed with tricuspid atresia with ventriculoarterial discordance (type IIc) and completed a staged Fontan operation with Damus-Kaye-Stansel anastomosis at 3 years of age.His parents stated that he had substantially reduced tolerance to exercise compared with his classmates.On cardiopulmonary exercise test, peak V ˙O2 was 21.3 ml/kg/min, which was 50% of the normal level according to the sex-and age-matched institutional reference value.Cardiac catheterization and magnetic

Background High pulmonary vascular resistance (PVR) may be a risk factor for early and late mortality in both Glen shunt and Fontan operation patients. Furthermore, PVR may increase long after the Fontan operation. Whether pulmonary vasodilators such as phosphodiesterase 5 inhibitors can decrease PVR in patients with single ventricular physiology remains undetermined. Methods and results This was a prospective, multicenter study. Patients with single ventricular physiology who have a PVR index higher than 2.5 Wood units·㎡ (WU) were enrolled. Cardiac catheterization was performed before and after administration of sildenafil in all patients. After the Fontan operation, a six minute walk test (6 MWT) was also performed. A total of 42 patients were enrolled. PVR was significantly decreased in each stage of single ventricular physiology after sildenafil administration: from 4.3 ± 1.5 WU to 2.1 ± 0.6 WU (p < 0.01) in patients before a Glenn shunt, from 3.2 ± 0.5 WU to 1.6 ± 0.6 WU (p < 0.001) in patients after a Glenn shunt, and from 3.9 ± 1.7 WU to 2.3 ± 0.8 WU (p < 0.001) in patients after Fontan. In patients after Fontan, the 6 MWT increased from 416 ± 74 m to 485 ± 72 m (p < 0.01), and NYHA functional class improved significantly (p < 0.05) after sildenafil administration. No major side effects were observed in any patients. Conclusions Sildenafil reduced PVR in patients with single ventricle physiology. Sildenafil increased exercise capacity and improved NYHA functional class in patients after a Fontan operation. This implies that pulmonary vasodilation is a potential therapeutic target in selected patients with elevated PVR with single ventricle physiology. Long-term clinical significance warrants further study.

BACKGROUND:Home oxygen therapy (HOT) is used to adapt patients to the bidirectional Glenn (BDG) physiology. However, the precise cardiovascular effect of oxygen inhalation is still unknown. We used phase-contrast MRI to evaluate the cardiovascular effects of oxygen inhalation in young patients with BDG physiology. METHODS AND RESULTS:The 56 sessions of cardiac MRI were performed in 36 patients with BDG circulation. Oxygen saturation (SpO2) and heart rate (HR) were monitored under both room air and nasal 100% oxygen inhalation, and the blood flow volumes of the ascending aorta (AA), superior vena cava (SVC), and inferior vena cava (IVC) were measured by phase-contrast MRI. Systemic-to-pulmonary collateral flow (SPCF) volumes were calculated by subtracting the sum of flow volumes through the SVC and IVC from the flow volume through the AA, and used for further comparative examination. Under nasal oxygen inhalation, SpO2significantly increased from 82% to 89%, while HR decreased from 115 to 110 beats/min. AA (5.0 vs. 4.9 L·min(-1)·m(-2)), SVC (1.85 vs. 1.77 L·min(-1)·m(-2)), and systemic blood flow volume (=SVC+IVC) significantly decreased (3.60 vs. 3.46 L·min(-1)·m(-2)). In contrast, SPCF and the pulmonary-to-systemic blood flow ratio (Qp/Qs) remained unchanged. CONCLUSIONS:Oxygen inhalation improved arterial blood oxygenation and lowered HR in patients with BDG circulation without an increase in Qp/Qs. HOT would be protective of the cardiovascular system in patients with BDG circulation. (Circ J 2016; 80: 1378-1385).

Background: In patients with single ventricular physiology, risk of Fontan operation increases with higher pulmonary vascular resistance (PVR). Furthermore, in patients after Fontan operation, even...

TCPC術直後から洞機能不全，接合部調律となったEbstein奇形の3歳10ヵ月，女児例を報告する．術後28日まで暫定的心房ペーシングを行い，洞機能回復を促す目的で術後23日からシロスタゾールを，術後37日からテオフィリンを投与した．その後，覚醒時はほぼ洞調律となったが，夜間の大半は接合部調律で，朝，浮腫や末梢冷感を認めた．心エコー図パルスドプラ法による左室流入血流速波形でA波が消失し，肺静脈血流速波形はS波の減少とPVA波の増高を認めた．房室同期を失った結果，心房ブースターポンプ機能およびリザーバー機能が低下したことが示唆された．心臓カテーテル検査における経食道心房ペーシング時と接合部調律時の循環動態の比較では，平均中心静脈圧は両者でほぼ同値であったが，接合部調律では中心静脈圧波形は棘波を呈した．また，接合部調律では体血圧および心係数は低下した．永久ペースメーカ植込術により心不全症状は改善した．房室同期の重要性を再確認するとともに，より質の高いFontan循環を目指したリズム管理を積極的に検討する必要があると考えられた．

Background: In patients with single ventricular physiology, risk of Fontan operation increases with higher pulmonary vascular resistance (PVR). Furthermore, in patients after Fontan operation, even...