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We reviewed the long-term outcome of children with hypertrophic cardiomyopathy (HCM) based on the type.We reviewed the medical records of 100 patients (male 54 female 46) with HCM at our hospital between 1977 and 2015. The survival and cardiac event-free survival rates were calculated by the Kaplan-Meier method.The age at the time of the diagnosis ranged from 0 to 15 years with a median of 8 years. The number of patients with Noonan syndrome and hypertrophic obstructive cardiomyopathy (HOCM), idiopathic HCM (i-HCM), and secondary HCM (s-HCM) was 13, 13, 65, and 9 respectively. A dilated phase of HCM occurred in 24 patients. Nineteen (79 %) of the 24 patients died of heart failure, and two underwent a heart transplantation. Eight (33 %) of the 24 patients had s-HCM. The median age when a dilated phase occurred was 15 years old, and the median interval from the initial diagnosis to the dilated phase was 8 years. The median time from the diagnosis of a dilated phase to death was 1.6 years. Sudden death and implantable cardioverter defibrillator implantations occurred in 6 and 11 patients at around 15 years old, respectively. The 20-year survival rates were as follows: Noonan syndrome 84 %; HOCM 82 %; i-HCM 71 %; and s-HCM 17 %. Overall, the survival rates at 10, 20, and 30 years were 83 % (95 % confidence interval 73-89), 69 % (58-78), and 63 % (50-74), respectively. The overall cardiac event-free survival rates at 10, 20, and 30 years were 57 % (47-67), 39 % (31-50), and 32 % (21-44), respectively.The long-term outcome in children with HCM was poor, and the outcome of s-HCM was very poor. The occurrence of a dilated phase worsened the outcome in HCM patients. Sudden death and d-HCM often occurred at around 15 years old.

The study aimed to investigate the long-term influence of atrial switch on post-Rastelli hemodynamic condition. Of 112 patients with transposition of the great arteries (TGA) or TGA-type double outlet right ventricle, ventricular septal defect (VSD), and pulmonary stenosis (PS) who underwent intra-cardiac repair between 1979 and 2018, 50 patients with levo-TGA underwent atrial switch and Rastelli as an anatomic repair and 62 patients with dextro-TGA underwent Rastelli. Postoperative outcomes were retrospectively compared. The median follow-up durations were 20.1 years (interquartile range: 4.3, 32.4) in the Rastelli group and 15.3 years (7.1, 23.0) in the atrial switch plus Rastelli group (p = 0.19). Sex, age, and weight at operation were similar in both groups. Overall survival rates at 30 years were 69.8% in the Rastelli group and 80.1% in the atrial switch plus Rastelli group (p = 0.18). The atrial switch plus Rastelli group required more frequent catheter interventions (p 0.001), mainly for caval obstruction (n = 8) and atrial arrhythmia (n = 6). Medication was more frequent in the atrial switch plus Rastelli group (p = 0.009). Exercise capacity was similarly reduced in two groups. Protein-losing enteropathy (PLE) occurred in three long-term survivors in the atrial switch plus Rastelli group (p = 0.07). Concomitantly performed atrial switch operation did not affect long-term survival and exercise capacity after Rastelli procedure. However, the occurrence of PLE, a frequent need for medication, and catheter interventions after atrial switch plus Rastelli may result from atrial switch under the post-Rastelli condition.

The case of a 14-year-old girl with acute myocardial infarction due to coronary artery aneurysms (CAAs) of unknown origin, which resembled coronary artery lesions caused by Kawasaki disease, is reported. She was transferred to our hospital due to chest pain with ST-T elevation. She had no history of Kawasaki disease. On the first admission, she was misdiagnosed with acute myocarditis. Then, 54 days later, she experienced chest pain with exertional dyspnea. Her electrocardiogram showed negative T waves in the chest leads. A CAA of the left coronary artery was suspected on two-dimensional echocardiography. Coronary angiograms showed 90% stenosis and multiple CAAs of the left anterior descending artery and the bifurcation of the left coronary artery. Both the right coronary artery and left circumflex artery were occluded. A left ventriculogram showed dyskinesis and an aneurysm at the apex. She underwent triple-vessel coronary artery bypass grafting, and her symptoms improved. In addition, an intracranial aneurysm was also found on cerebral angiography. There were no specific laboratory findings other than SS-A antibodies. It was suspected that the weakness of the vessels was related to the disease. It may have been a different disease that was never previously detected, but her CAAs were Kawasaki-like CAAs. Learning objective A timely precise diagnosis of acute myocardial infarction is unlikely to be made in children because they are rare. The present patient was initially misdiagnosed as having acute myocarditis, because the coronary arteries could not be detected by two-dimensional echocardiography. Either computed tomographic angiography or magnetic resonance angiography is recommended in patients with ST-T abnormalities on the electrocardiogram if it is difficult to identify the coronary arteries. In this case, the patient had a rare coronary artery disease in which the cause of the coronary artery aneurysms was unknown.

BACKGROUND Pregnant women with a Fontan circulation have a high risk of obstetric complications, such as preterm delivery and small for gestational age (SGA), which may be affected by low blood flow to the placenta and hypoxia. This study investigated placental pathology in a Fontan circulation.Methods and Results:Eighteen pregnancies in 11 women with a Fontan circulation were reviewed. Pregnancy outcomes showed 9 miscarriages and 9 live births, with 4 preterm deliveries. Five neonates were SGA (

The long-term outcomes of a total cavopulmonary connection (TCPC) with an extracardiac conduit (ECC) for patients with apicocaval juxtaposition (ACJ) remain unclear.A total of 38 patients with ACJ who underwent TCPC with ECC between 1998 and 2014 were enrolled in this study. For 19 patients with a superior vena cava - inferior vena cava contralateral position, a long-curved route rounding the opposite side of the apex was selected (CC group). For 11 patients with a superior vena cava-inferior vena cava ipsilateral position, a long-curved route was principally selected (IC group); however, a short, straight route was selected for 8 patients because there was sufficient space behind the ventricular apex (IS group).Follow-up was completed in all patients, with a mean follow-up duration of 13.2 ± 4.9 years. The angles of the caudal conduit anastomosis site measured from the frontal view of cineangiography had significantly straightened in the CC group from 1 year to 15 years (P.05) and in the IC group from 1 year to 10 years (P.05). There were 2 late mortalities and 6 reoperations during follow-up. Overall survival and freedom from reoperation rates at 15 years were 95% and 82%, respectively. There were no conduit-related or route-related complications such as death, reoperations, pulmonary venous obstructions, conduit obstructions, or pulmonary arteriovenous malformations in any of the groups.Even though chronologic geometric changes of curved ECCs were observed, TCPC with ECC for patients with ACJ can be safely applied without conduit- or route-related complications in long-term follow-up.

OBJECTIVES The present study was conducted to investigate the decrease in left ventricular stroke volume index (LVSVI) that is caused by pulmonary regurgitation-induced right heart dysfunction and its clinical implications before and after pulmonary valve replacement (PVR). METHODS Between January 2010 and December 2019, 30 adults who underwent surgical PVR for chronic pulmonary regurgitation with right ventricular dilation late after tetralogy of Fallot (TOF) repair were included. All patients were evaluated using cardiac magnetic resonance before PVR. The median interval from TOF repair to PVR was 29 [25th, 75th percentile: 25, 37] years. The median pulmonary regurgitation fraction and right ventricular end-diastolic volume index were 56 [48, 66] % and 203 [187, 239] ml/m2. Twenty-three patients (76.7%) were re-evaluated 1 year after PVR. RESULTS Before PVR, the median LVSVI was 40 [35, 46] ml/beat/m2. A lower LVSVI was associated with a longer interval from TOF repair to PVR (r = −0.40, P = 0.029) and a lower right ventricular ejection fraction (r = 0.52, P = 0.004). A lower LVSVI was not associated with a higher right ventricular end-diastolic volume index. LVSVI remained unchanged after PVR. The patients were subdivided into Normal-stroke volume index (SVI) and Subnormal-SVI groups using the preoperative LVSVI cut-off value of 35 mL/beat/m2. Compared with the Normal-SVI group, the Subnormal-SVI group had a higher incidence of ablation therapy before PVR (4.7 vs 2.3 patient-years, P = 0.044). After PVR, LVSVI in the Subnormal-SVI group was still lower (40 [34, 42] vs 44 [42, 47] ml/beat/m2, P = 0.038) despite the right ventricular end-diastolic volume index normalization. There was no difference in the clinical event incidence between the 2 groups during the follow-up period. Brain natriuretic peptide level in the Subnormal-SVI group was higher within 3 years after PVR (P = 0.046). CONCLUSIONS Reduced left ventricular stroke volume did not fully recover after PVR. PVR for patients with repaired TOF should be performed before the left ventricular stroke volume begins to decrease.

The objectives of this study was to compare the long-term outcomes of anatomic repair using atrial switch with the Rastelli procedure versus physiological repair with left ventricle-to-pulmonary artery conduit for patients with levo-transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction.Of patients with levo-transposition of the great arteries who underwent biventricular repair between 1978 and 2001, 31 hospital survivors after anatomic repair of atrial switch and the Rastelli (anatomic group) and 14 hospital survivors after physiological repair with left ventricle-to-pulmonary artery conduit (physiological group) were enrolled. Survival rates, reoperation rates, and most recent conditions were compared.The overall survival rate at 20 years was 79.7% (95% CI, 66.4%-95.6%) in the anatomic group and 85.1% (95% CI, 68.0%-100%) in the physiological group (P = .87). The reoperation rate at 10 years was 19.8% (95% CI, 5.6%-34.0%) in the anatomic group and 52.0% (95% CI, 25.0%-79.1%) in the physiological group (P = .067). Only patients in the physiological group underwent systemic tricuspid valve replacement. The anatomic group showed a better cardiac index at catheterization (2.79 ± 0.75 L/min/mPreservation of the left ventricle as the systemic ventricle using anatomic repair contributes to better cardiopulmonary condition compared with physiological repair.

Failed Fontan Patients with high cardiac output (CO) heart failure (HF) might have vasodilatory syndrome and markedly high mortality rates. The aim of this study was to review the clinical effects of vasoconstrictor therapy (VCT) for failed Fontan hemodynamics. We retrospectively reviewed 10 consecutive patients with Fontan failure (median age, 33 years) and high CO–HF who had received VCT. The hemodynamics were characterized by high central venous pressure (CVP: median, 16 mm Hg), low systolic blood pressure (median, 83 mm Hg), low systemic vascular resistance (median, 8.8 U·m2), high cardiac index (median, 4.6 L/min/m2), and low arterial oxygen saturation (median, 89%). VCT included intravenous noradrenaline infusion for five unstable patients, oral midodrine administration for nine stable patients, and both for four patients. After VCT introduction with a median interval of 1.7 months, the median systolic blood pressure (102 mm Hg, p = 0.004), arterial oxygen saturation (90%, p = 0.03), and systemic vascular resistance (12.1 U·m2, p = 0.13) increased without significant changes in CVP or cardiac index. After a median follow-up of 21 months, the number of readmissions per year decreased from 4 (1–11) to 1 (0–9) (p = 0.25), and there were no VCT-related complications; however, five patients (50%) developed hepatic encephalopathy, and six patients (60%) eventually died. VCT was safely introduced and could prevent the rapidly deteriorating Fontan hemodynamics. VCT could be an effective therapeutic strategy for failed Fontan patients with high CO–HF.

Background Congenital heart disease (CHD) is often diagnosed prenatally using fetal echocardiography, but few studies have evaluated the accuracy of these fetal cardiac diagnoses in detail. We investigated the discrepancy between pre- and postnatal diagnoses of CHD and the impact of discrepant diagnoses.Methods and Results:This retrospective study at a tertiary institution included data from the medical records of 207 neonates with prenatally diagnosed CHD admitted to the cardiac neonatal intensive care unit between January 2011 and December 2016. Pre- and postnatal diagnoses of CHD differed in 12% of neonates. Coarctation of the aorta and ventricular septal defects were the most frequent causes of discrepant diagnosis. Unexpected treatments were added to 38% of discrepant diagnostic cases. However, discrepant diagnoses did not adversely affect the clinical course. The 9% of the 207 neonates who required invasive intervention within 24 h of delivery were accurately diagnosed prenatally. Conclusions Pre- and postnatal diagnoses differed in only a few neonates, with differences not adversely affecting the clinical course. Neonates who required invasive intervention immediately after delivery were accurately diagnosed prenatally. Prenatal diagnosis thus seems to contribute to improved prognosis in neonates with CHD.

Background A stiffened, dilated ascending aorta may represent an important predictor of cardiovascular mortality, and has been reported in patients with congenital heart disease, including single ventricle. However, the serial conformational changes and determinants of reduced distensibility in ascending aorta have not been clarified. Methods This retrospective study investigated 115 postoperative Fontan survivors (median age at Fontan: 3.7 years). All patients underwent cardiac catheterization before and 1, 5, 10, and 15 years after the Fontan operation. We measured Z-scores for diameters and stiffness indexes (β) of the ascending aorta and descending aorta from angiograms. We also reviewed the clinical profiles, hemodynamic parameters, and exercise capacities of patients and compared them with results from 47 control subjects. Results Fontan survivors displayed significantly larger Z-score and β of the ascending aorta from before to 15 years after surgery than controls, whereas values for the descending aorta were comparable. Z-score for the ascending aorta was decreased, but β was elevated significantly according to the trend test. In multivariable analysis, β of the ascending aorta at 15 years after Fontan operation and its increasing trend were associated with older age at Fontan operation and elevated ventricular end-diastolic pressure. Reduced exercise capacity also correlated with stiffening of the ascending aorta. Conclusions Fontan survivors showed progressive stiffening and relatively slow growth of the dilated ascending aorta. Progressive stiffening of the ascending aorta may be coupled to diastolic dysfunction and reduced exercise capacity, suggesting the importance of lifelong management of subclinical Fontan pathophysiology.

Several studies have reported aortic dilation and increased stiffness of the ascending aorta in patients after repair of congenital heart disease (CHD), which may be a predominant cardiovascular risk. However, the clinical significance has not been described in detail. In this retrospective study, 175 repaired patients with complex CHD achieving biventricular circulation and age-matched 39 control subjects were reviewed (median age: 14.9 and 15.7 years, respectively). We measured the diameters of the ascending aorta and descending aorta from catheterization angiograms to yield Z-scores and stiffness indexes (β) using diameter fluctuations corresponding to pulsatile pressures. Clinical profile, peak oxygen uptake during the cardiopulmonary exercise test, and incidence of unscheduled hospitalization during follow-up was also reviewed. Compared with controls, patients with complex CHD, except for those with aortic coarctation, exhibited significant dilation and increased stiffness of the aortic root and ascending aorta, but not of the descending aorta. In this CHD population (n = 147, including 112 conotruncal anomalies), exercise capacities correlated independently with the diameter Z-score and stiffness index of the ascending aorta along with the history of repetitive thoracotomies, reduced forced vital capacity, and right ventricular hypertension. During a follow-up period (median 15.6 years), either dilation (Z-score >3.5) or increased stiffness (β >6.0) of the ascending aorta stratified morbidity, but no synergistic impact was detected. In conclusion, in repaired patients with complex CHD, a stiffened and dilated ascending aorta was frequently found, exerting significant adverse impacts on diminished exercise capacity and morbidity.

Reoperation after pediatric mitral valve replacement (MVR) is inevitable due to patient-prosthesis mismatch (PPM) associated with somatic growth. We analyzed potential metrics for PPM and outcomes of redo MVR for valve upsizing. Between 1999 and 2018, 15 children without obstructive left heart lesions other than mitral stenosis underwent initial MVR with a 16-mm ATS-Advanced Performance valve. We analyzed hemodynamic data from 28 postoperative catheterizations and concomitant echocardiograms. The median age and body weight at initial MVR were 4.9 months (25th, 75th percentile: 3.6, 6.6) and 5.9 kg (5.0, 7.3). Redo MVR was planned when patients had congestive heart failure and postcapillary pulmonary hypertension (PH) due to PPM: systolic pulmonary arterial pressure (SPAP)35 mm Hg and pulmonary capillary wedge pressure (PCWP)15 mm Hg on catheterization. Indexed effective orifice area (iEOA) and mean transmitral pressure gradient (TMPG) were strongly correlated with SPAP (r = -0.72, P0.001 and r = 0.75, P0.001) and PCWP (r = -082, P0.001 and r = 0.84, P0.001). Cut-off values for detecting postcapillary PH due to PPM were 1.0 cm

OBJECTIVES The aim of this study was to review a single institution’s experience with EXCOR Paediatric implantation. METHODS Patients RESULTS Overall median age and weight for all 20 children at implantation were 10.8 (interquartile range, 7.9–33.2) months and 6.3 (4.6–10.2) kg. Ten patients (50%) weighed 20 months. One patient died. Seven patients underwent heart transplant. Heart transplant has not been performed in the last 1.5 years. Five patients were weaned from EXCOR support after native myocardial recovery, including a patient with dilated cardiomyopathy who recovered after 24 months of EXCOR support. Major complication-free survival at 6, 12 and 18 months were 79.3%, 49.6% and 38.6%, respectively. Body weight CONCLUSIONS Survival during EXCOR Paediatric support was good, but it prolonged the wait time for a heart transplant. The number of major complications increased over time and was not negligible, especially in small children.

Background: Assessment of plasma volume status (PVS) is essential for the management of patients with heart failure (HF). We aimed to elucidate the clinical features of PVS in patients with Fontan circulation (FC). Methods: Plasma volume (PV) was measured via pulse dye densitometry (icg-PV) and compared with the calculated PV (c-PV) in 114 patients with FC and 32 with biventricular circulation (BVC). We derived the PVS (%) [{(c-PV－ideal-PV)/ideal-PV} ​× ​100] and elucidated its clinical associations in 275 consecutive patients with FC and 493 with BVC. Results: The c-PV correlated with the icg-PV in FC (r ​= ​0.70) and BVC (r ​= ​0.67) (p ​

OBJECTIVE The aim of this study was to assess outcomes of the deferred Norwood strategy, i.e. planned Norwood following routine bilateral pulmonary artery banding and ductal stenting or continuous prostaglandin E1 administration. METHODS Forty-five patients with hypoplastic left heart syndrome and its variants treated with the deferred Norwood strategy between 2012 and 2021 were enrolled. Mid-term outcomes were retrospectively reviewed. The median follow-up period after Norwood in hospital survivors was 4.6 years (interquartile range: 1.9, 6.8). RESULTS Fourteen patients (31.1%) had no risk factors. The median age and weight at Norwood were 1.8 months (0.9, 3.5) and 3.1 kg (2.7, 3.6). Transplant-free survival at 6 years was 84.5%. Birth weight ≤2.5 kg and systemic atrioventricular valve (SAVV) regurgitation ≥ moderate after birth were not risk factors for mortality; body weight at Norwood ≤2.5 kg, however, was a risk factor [hazard ratio (HR), 11.3; 95% confidence interval (CI), 1.2–11; P = 0.036]. Twenty-two (48.9%) underwent Fontan with no mortalities, and 7 (15.5%) are awaiting Fontan. Freedom from SAVV surgery at 5 years was 53.1%. SAVV regurgitation ≥ moderate after birth was a risk factor for SAVV surgery (HR, 16; 95% CI, 3.6–71; P < 0.001); however, ductal stenting had a protective effect against SAVV surgery (HR, 0.09; 95% CI, 0.01–0.68; P = 0.019). Freedom from both surgical and catheter-based pulmonary artery intervention at 3 years was 27.1% CONCLUSIONS Although deferred Norwood provided acceptable intermediate-term survival, the Fontan completion rate was unsatisfactory. SAVV surgery and pulmonary artery intervention were frequently required.

Given the rarity of significant first-degree atrioventricular block (AVB) after Occlutech Figulla Flex II atrial septal defect occluder (OFF II, Occlutech International AB, Helsingborg, Sweden) placement, its management and predictors of recovery have not been established. We report a 9-year-old boy who developed significant first-degree AVB a day after OFF II deployment. Thereafter, oral prednisolone was initiated at 1.5 mg/kg/day. On day 2 of steroid treatment, normal sinus rhythm without conduction delays was occasionally observed, while the prolonged PR interval on the exercise stress test was ameliorated. After 5 days of steroid administration, normal sinus rhythm without conduction delays was completely achieved. An increase in the frequency of normal sinus rhythm and the absence of conduction delays on electrocardiogram monitoring and exercise stress test play an important role in predicting recovery from prolonged PR interval. Thus, steroid treatment can be effective and should be considered for patients who develop first-degree AVB after undergoing OFF II placement for transcatheter device closure of atrial septal defect.

Objectives: Despite the high prevalence of menstrual problems (MPs), their associations with hemodynamics and their prognostic value in women with congenital heart disease (WCHD) remain unknown. Methods: We retrospectively evaluated characteristics related to menstruation (age at menarche, menstrual cycle, and menstrual blood loss) in 143 consecutive WCHD, and investigated the associations of these characteristics with hemodynamics and prognosis. Results: A total of 132 WCHD were analyzed after excluding 11 patients in menopause. Diagnoses of abnormal onset of menarche (delayed menarche or primary amenorrhea) (AOM) and menstrual cycle disorders (MCDs) were made in 11 (8.3%) and 64 (48.5%) patients, respectively. Cyanotic CHD, non-left systemic ventricle, multiple surgeries before menarche, and high central venous pressure (CVP) during puberty (≥13 ​mmHg) were associated with AOM (p ​

Background Although breastfeeding can cause an increase in the maternal cardiac load, little is known about how it affects the postpartum clinical course among women with congenital heart disease (CHD). The aim of this study was to evaluate whether breastfeeding affects the incidence of postpartum cardiovascular events and the levels of brain natriuretic peptide (BNP) among mothers with CHD. Methods A review of 153 pregnancies in 153 women with CHD was conducted. Postpartum outcomes, including late postpartum cardiovascular events and BNP levels, were analysed by breastfeeding status. Results Ninety-three were primarily breastfeeding (the breastfed-group) and 60 were primarily formula feeding (the formula-group). The formula-group had a significantly higher rate of women with severe conditions, defined as class II-III and III of the modified World Health Organization (WHO) classification of maternal cardiovascular risk, than the breastfed-group (p = 0.03). Late postpartum cardiovascular events occurred in 11 women and not feeding status, but medication use, was the only correlated factor for the events in multivariate analysis. The values of BNP in the third trimester (29.2 pg/mL vs 32.8 pg/mL; p = 0.15) and at one-month postpartum (23.7 pg/mL vs 24.9 pg/mL; p = 0.26) were not statistically different between the breastfed-group and the formula-group. Even when limited to women with the modified WHO class III disease, BNP values were not significantly different between the two groups. Conclusions Among postpartum women with CHD, the cardiovascular event rates and BNP values may not be affected by feeding status, but mostly by disease severity.

To predict the prognosis of infants with congenital heart disease, accurate prenatal diagnosis of structural abnormality and heart failure are both necessary. The aim of this study was to investigate whether cardiovascular profile (CVP) and biophysical profile (BP) scores are useful for predicting prognosis in infants with congenital heart defect (CHD).A retrospective review of singletons prenatally diagnosed with CHD at a tertiary pediatric cardiac center between 2011 and 2015 was undertaken.A total of 202 patients with CHD were analyzed. Perinatal and infant deaths occurred in 16 (7.9%) and 10 cases (5.0%), respectively. Infants with the last CVP score ≤ 5 had 18.7-fold higher perinatal mortality than those with a last CVP score 5 (P 0.01). Infants with a last BP score ≤ 6 had 18.7-fold higher perinatal mortality than those with a last BP score 6 (P 0.01). Infants with a CVP score decrease in utero had 4.5-fold higher infant mortality than those with an increase or no change (P 0.01). Multivariate analysis showed that single-ventricle physiology, pre-term birth at37 weeks of gestation, last CVP score ≤ 5, and last BP score ≤ 6 were independent predictors of perinatal mortality. Single-ventricle physiology and a CVP score decrease were independent predictors of infant mortality.CVP and BP scores are useful for predicting perinatal prognosis in infants with CHD. A CVP score decrease in utero is associated with infant mortality, suggesting that serial CVP score assessment may be useful for management planning.

OBJECTIVESTo review the long-term surgical outcomes of ventricular septation for double-inlet left ventricle and reconsider the possibility of ventricular septation as an option of surgical treatments.METHODSBetween 1978 and 1994, 22 patients with double-inlet left ventricle underwent ventricular septation. The mean age at operation was 5.3 years (range 0–22 years). Follow-up was carried out in 20 of 22 patients (91%) and the mean follow-up period was 14.7 years (range 0–39 years).RESULTSActuarial survival and reoperation-free survival rates at 30 years were 49% and 21%, respectively. To date, 8 patients have been followed up. Among them, atrioventricular valve replacement and permanent pacemaker were required in 4 and 7 patients, respectively. Late cardiac catheter examination at 25.5 years after surgery showed that the median cardiac index was 2.6 l/min/m2 (range 2.1–3.4 l/min/m2), left ventricular end-diastolic pressure was 7 mmHg (range 4–11 mmHg), left ventricular ejection fraction was 50% (range 27–63%), right ventricular ejection fraction was 53% (range 31–66%) and central venous pressure was 6 mmHg (range 4–11 mmHg). At the latest follow-up, the New York Heart Association Functional Classification was I for 5 patients, II for 2 patients and III for 1 patient. The median peak oxygen uptake was 52.9% (range 44.1–93.5%).CONCLUSIONSSome patients with double-inlet left ventricle were able to maintain low central venous pressure and a sufficient cardiac index long after ventricular septation. Although the single ventricle strategy remains a first-line treatment, ventricular septation can be a surgical treatment option.

The frequency of newborns with congenital heart disease (CHD) is approximately 1% in the general population; however, the recurrence rate of CHD in mothers with CHD differs in ethnicity and reports. We therefore aimed to determine the prevalence of CHD among neonates born to mothers with CHD in our institute in Japan. We reviewed the medical charts of 803 neonates delivered by 529 women with CHD at the National Cerebral and Cardiovascular Center from 1982 to 2016. They included isolated ventricular septal defect (VSD,31.4%), isolated atrial septal defect (ASD, 23.3%), tetralogy of Fallot (TOF,10.6%). We defined CHD in neonates as being diagnosed within 1 month of birth. We estimated that the average rate of the CHD recurrence was 3.1%. The recurrence ratios in each maternal CHD were 8.6%, 7.1%, 6.2%, 4.8%, 3.6%, and 1.5% for PS, CoA, TOF, atrioventricular septal defect, VSD, and ASD, respectively. The rate of CHD in offsprings whose mothers have CHD was 3 times greater than that of mothers with healthy hearts. Almost half of neonates with CHD had the same phenotype as their mother in our series. Especially, PS and CoA were closely related to the type of maternal CHD.

Arterial switch operation (ASO) for dextro-transposition of the great arteries (d-TGA) has gradually replaced the atrial switch operation and has become the standard operation. To date, the outcomes of pregnant women with d-TGA after this new operation have not been investigated. In this study, we investigated the impact of ASO on pregnant outcomes and mid-term prognosis in women with d-TGA and compared with the atrial switch operation through the literature review.There were 20 pregnancies in 10 women with d-TGA after ASO and 6 resulted in abortion. Among 14 successful pregnancies in 10 women, 11 pregnancies achieved the term delivery and 3 pregnancies, including 1 twin pregnancy, resulted in preterm labor. Maternal cardiovascular events occurred in 4 (heart failure and arrhythmias in 3 and arrhythmia in 1), and all were controllable with medications. Risk factors for the peripartum cardiac events were older age at ASO and delivery, and higher concentration of brain natriuretic peptide (BNP) at first trimester (p0.05). In 7-60 month-follow-up after delivery, no case showed deterioration of functional class and systemic ventricular function. According to the literature review, women after ASO demonstrated a better prognosis than those after the atrial switch operation.The majority of women with d-TGA after ASO tolerated pregnancy and delivery well. The older age at ASO, an elderly pregnancy, and higher BNP levels at the first trimester were possibly risk factors of peripartum cardiovascular events among the group. The literature reviews and this study may indicate the advantage of systemic left ventricle compared with systemic right ventricle in long-term outcomes after delivery.

Objective: Surgical outcomes of biventricular repair for hearts with hypoplastic left ventricle with congenital mitral valve stenosis are described. Serial changes of left ventricular geometry and clinical features after biventricular repair were reviewed. Methods: Eight patients with hypoplastic left ventricle and congenital mitral valve stenosis who underwent first surgical intervention for biventricular circulation in neonatal or infantile period between 2001 and 2014 comprise the study population. Serial change in left ventricular end-diastolic diameter, left ventricular mass index, and relative wall thickness after biventricular repair were evaluated by two-dimensional echocardiography. Results: The median Z-scores of left ventricular end-diastolic diameter and mitral valve diameter before the first surgical intervention were −3.0 (range, −4.8 to −2.0) and −1.0 (−2.9 to 2.1), respectively. Mitral valves were surgically treated in five patients; they were replaced in two and repaired in three patients. Left ventricular end-diastolic diameter Z-score at five years after biventricular repair was 0.1 (−3.0 to 1.0), which was significantly larger than before first surgical intervention ( P = .005). Left ventricular mass index, on the other hand, did not change, but relative wall thickness significantly decreased ( P = .009). Postoperative catheter study showed pulmonary hypertension with high left ventricular end-diastolic pressure in more than half of survivors. Conclusions: Left ventricle increased in size after the biventricular repair with appropriate mitral valve procedures before progression of pulmonary hypertension. Left ventricular mass, however, did not accompany the increase. Some patients may have suffered from mild, but certain restrictive left ventricular physiology and subsequent pulmonary hypertension as the result of abnormal remodeling process of the myocardium.

OBJECTIVES The aim of this study was to identify the impact of bilateral bidirectional Glenn (BBDG) anastomosis on staged Fontan strategy and late Fontan circulation. METHODS Of 267 patients who underwent bidirectional Glenn prior to Fontan completion between 1989 and 2013, 62 patients (23%) who underwent BBDG were enrolled in this study. Age at operation was 0.84 years (25th–75th percentile: 0.58–1.39). Thirty-three patients had heterotaxy syndrome (53%). The mean follow-up period was 12.7 ± 8.1 (max. 30.6) years. RESULTS The overall survival rate at 15 years was 73%. Although 49 patients (79.0%) went on to Fontan completion, 12 patients (19.4%) died without achieving it. Thrombus formation and poor development in a central pulmonary artery were not observed, but obstruction of the superior vena cava (SVC) occurred in 8 patients (13%), mainly those with right atrial isomerism (P = 0.037). SVC obstruction was not, however, a risk factor for mortality (P = 0.097) or Fontan completion (P = 0.41). The shape of BBDG anastomosis, symmetricity of pulmonary blood flow, impingement of caval blood flow returning from the superior and inferior vena cavae or coexisting interrupted inferior vena cava with azygos or hemi-azygos continuation did not affect late Fontan outcomes, such as overall survival, freedom from protein-losing enteropathy or pulmonary arterio-venous malformation rates. CONCLUSIONS SVC obstruction after BBDG frequently occurred, mainly in patients with right atrial isomerism; however, its direct impact on prognosis or achieving Fontan completion was not identified. Once Fontan circulation was established, the arrangement of the Fontan pathway did not affect late Fontan outcomes.

The study objectives were to reconfirm the superiority of the pulmonary valve-sparing procedure versus the transannular patch procedure for repair of tetralogy of Fallot and to evaluate the influence of a right ventriculotomy in the pulmonary valve-sparing procedure.Between 1978 and 2003, 440 patients (aged10 years) underwent tetralogy of Fallot repair. Of these patients, 242 (55.0%) underwent the transannular patch procedure, 106 (24.1%) underwent the pulmonary valve-sparing procedure without right ventriculotomy, and 92 (20.9%) underwent the pulmonary valve-sparing procedure with right ventriculotomy. End points focused on adverse events and included all-cause mortality, reoperation, catheter intervention, and symptomatic arrhythmias. To compare the outcomes of pulmonary valve sparing with and without right ventriculotomy, inverse probability weighting was applied to adjust for potential confounding factors.The median follow-up period was 20.3 years (interquartile range, 10.7-27.6). In all cohorts, the pulmonary valve-sparing procedure was the independent factor that reduced adverse events after tetralogy of Fallot repair (hazard ratio, 0.47; 95% confidence interval, 0.23-0.94;Right ventriculotomy for tetralogy of Fallot repair with pulmonary valve-sparing did not increase major adverse events. However, negative impacts on current status cannot be ignored.

Background Liver cancer (LC) is a serious late complication after the Fontan operation. However, the incidence, predictors, and prognosis remain unknown. The purpose of our study was to determine these clinical characteristics. Methods and Results We assessed liver function in 339 consecutive patients who had undergone the Fontan procedure from 2005 to 2019. LC was histologically diagnosed in 10 patients after a median period of 2.9 years (range: 0.3–13.8; median age: 29.9 years [range: 14.4–41.5 years]; overall median post–Fontan procedure follow‐up: 25.6 years [range: 13–32.1 years]), and the annual incidence was 0.89%. Over the entire post‐Fontan follow‐up period, the annual incidences of new‐onset LC in the second, third, and fourth decades were 0.14%, 0.43%, and 8.83%, respectively. The patients with LC had longer follow‐up periods, higher levels of AFP (α‐fetoprotein), and higher values of liver fibrosis indices ( P P P Conclusions The LC incidence rapidly increased ≥30 years after the Fontan procedure, and liver fibrosis indices and AFP were predictive of new‐onset LC. These LC‐predictive markers should be monitored closely and mandatorily for early LC detection and better prognosis.

The study aimed to assess the long-term outcomes after fenestration closure in patients at risk for Fontan failure. Of 119 patients who underwent Fontan operation between 1995 and 2004, fenestration was not created in 89 patients (NF group) and created in 30 patients with hypoplastic left heart syndrome, heterotaxy syndrome, high pulmonary arterial pressure, high systemic ventricular end-diastolic pressure, low ventricular ejection fraction, or atrioventricular valve regurgitation. All fenestrations were closed spontaneously or by catheter/surgical interventions, excepting two patients, and therefore, they were excluded. In fenestration group, patients with pre-Fontan mean pulmonary arterial pressure ≥ 15 mmHg or systemic atrioventricular valve regurgitation ≥ moderate were classified as high-risk Fontan candidates (F-HR group, n = 16), and the remaining patients were as standard-risk (F-SR group, n = 12). Protein-losing enteropathy-free survival rates did not differ among the three groups (p = 0.72). Serial follow-up catheter examinations after Fontan operation were completed in 69 patients in NF group and 11 patients in both F-SR and F-HR groups. Cardiac index and pulmonary vascular resistance significantly and similarly decreased over time in all groups, though the F-HR group showed lowest arterial oxygen saturation, lowest cardiac index, and highest pulmonary vascular resistance. The F-HR group also showed much veno-venous collaterals (p = 0.049), low peak oxygen consumption (p = 0.019), and low anaerobic threshold (p = 0.023) as compared to those in the F-SR group. In F-HR group, cyanosis remained after fenestration closure due to transformation from fenestration to veno-venous collaterals, which resulted in elevation of pulmonary vascular resistance, low cardiac index, and deterioration of exercise tolerance.

Heterograft and artificial materials have been used for extracardiac conduit implantation to create right ventricular (RV) to pulmonary artery (PA) continuity for biventricular repair in Japan because of the limited availability of homograft valves. However, few studies have examined morphological changes and number of candidates for transcatheter pulmonary valve implantation (TPVI) in which the conduit includes more than one type of material. Overall, 88 patients who underwent biventricular repair with an external conduit were included in this evaluation. Based on catheterization data and surgical records, we estimated morphological change in the RV outflow tract for each material and the number of candidates for Melody valve implantation based on premarket approval application criteria established by the U.S. Food and Drug Administration. There were 63 candidates for TPVI (72%, 63/88). Median anteroposterior and lateral diameter of the RV outflow tract was 20.4 mm (range 9.0–41.5) and 17.8 mm (range 9.5–34.9), respectively. Bovine pericardium tended to dilate by 11.2%. Polytetrafluoroethylene (ePTFE), homograft, and Dacron polyethylene terephthalate (PET) tended to become stenotic by 11.1%, 28.0%, and 13.4%, respectively. While ePTFE (27/33, 82%) and Dacron PET (2/2, 100%) were highly suitable for TPVI, bovine pericardium (32/48, 67%) was less suitable. In Japan, many patients with hemodynamic indications for TPVI following extracardiac conduit implantation to create RV to PA continuity may also meet the morphological indications.

Background: Left ventricular noncompaction (LVNC) is a hereditary type of cardiomyopathy. Although it is associated with high morbidity and mortality, the related ion channel gene variants in children have not been fully investigated. This study aimed to elucidate the ion channel genetic landscape of LVNC and identify genotype-phenotype correlations in a large Japanese cohort. Methods: We enrolled 206 children with LVNC from 2002 to 2017 in Japan. LVNC was classified as follows: LVNC with congenital heart defects, arrhythmia, dilated phenotype, or normal function. In the enrolled patients, 182 genes associated with cardiomyopathy were screened using next-generation sequencing. Results: We identified 99 pathogenic variants in 40 genes in 87 patients. Of the pathogenic variants, 8.8% were in genes associated with channelopathies, 27% were in sarcomere genes, and 11.5% were in mitochondrial genes. Ion channel gene variants were mostly associated with the arrhythmia classification, whereas sarcomere and mitochondrial gene variants were associated with the dilated phenotype. Echocardiography revealed that the group with ion channel gene variants had almost normal LV ejection fraction and LV diastolic diameter Z scores. Fragmented QRS, old age, and an arrhythmia phenotype were the most significant risk factors for ventricular tachycardia ( P =0.165, 0.0428, and 0.0074, respectively). Moreover, the group with ion channel variants exhibited a greater risk of a higher prevalence of arrhythmias such as ventricular tachycardia, rather than congestive heart failure. Conclusions: This is the first study that focused on genotype-phenotype correlations in a large pediatric LVNC patient cohort with ion channel gene variants that were determined using next-generation sequencing. Ion channel gene variants were strongly correlated with arrhythmia phenotypes. Genetic testing and phenotype specification allow for appropriate medical management of specific LVNC targets.

Background Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy, associated with high morbidity and mortality, but the role of genetics in cases of fetal-onset has not been fully evaluated. The goal of this study was to identify the genetic background in LVNC fetal-onset patients using next-generation sequencing (NGS). Methods Thirty-three fetal-onset Japanese probands with LVNC (20 males and 13 females) were enrolled. In the enrolled patients, 81 genes associated with cardiomyopathy were screened using next-generation sequencing (NGS) retrospectively. Results Twenty-three patients had congestive heart failure (CHF), and six patients had arrhythmias. Prominent trabeculations were mostly observed in lateral LV, posterior LV, and apex of LV in patients with LVNC. Twelve died; three patients experienced intrauterine death or termination of pregnancy. Overall, 15 variants were found among eight genes in 16 patients. Seven variants were detected in MYH7 and two in TPM1. Sarcomere gene variants accounted for 75.0%. A multivariable proportional hazards model revealed that CHF at diagnosis and a higher ratio of the noncompacted layer/compacted layer in the LV posterior wall were independent risk factors for death in LVNC fetal-onset patients (odds ratio = 4.26 × 106 and 1.36 × 108, p = 0.0075 and 0.0005, respectively). Conclusions The present study is the first report focusing on genetic background combined with clinical features in LVNC fetal-onset patients using NGS. Sarcomere variants were most commonly identified in fetal-onset patients, and greater attention should be paid to fetal-onset patients with LVNC having prominent trabeculations in the LV because they are more likely to develop CHF.

To clarify ventricular function in patients with asymptomatic coronary artery occlusion (ACAO) after Kawasaki disease (KD).Methods and Results:We enrolled 65 patients with coronary artery lesions who had undergone cardiac magnetic resonance (CMR). Median age at CMR was 29 years. CMR was performed to evaluate only the transmural extent of late gadolinium enhancement (LGE) and ejection fraction (EF). Based on the depth of LGE, it was classified into 5 groups: 0% (G0), 1-25% (G1), 26-50% (G2), 51-75% (G3), and 76-100% (G4). We investigated the relationship of the degree of LGE and EF. Further, we also evaluated the EF among 3 groups [ACAO, myocardial infarction (MI), and noncoronary artery occlusion (Non-CO)]. The grade of LGE and the LVEF (mean±SD, %) were as follows: G0 (n=24, 52.6±4.8), G1 (n=13, 50.8±4.4), G2 (n=15, 49.1±5.6), G3 (n=9, 30.9±9.1), and G4 (n=9, 27.7±6.8). LVEF in patients with G3 and G4 was significantly low (P0.05). LVEF (%) in patients with ACAO, MI, and Non-CO were 50.5±4.8 (n=38), 33.6±10.8 (n=17), and 53.0±5.7 (n=10), respectively. LVEF in the MI group was significantly low (P0.0001).LGE50% can lead to LV dysfunction. The transmural extent of LGE in most of the study patients with ACAO was ≤50% and they had subendocardial infarction, with preserved LV function.

Electrical disparity can induce inefficient cardiac performance, representing an uncoordinated wall motion at an earlier activated ventricular wall: an early shortening followed by a systolic rebound stretch. Although regional contractility and distensibility modulate this pathological motion, the effect of a morphological factor has not been emphasized. Our strain analysis in 62 patients with single ventricle revealed that those with an activation delay in 60–70% of ventricular wall area suffered from cardiac dysfunction and mechanical discoordination along with prolonged QRS duration. A computational simulation with a two-compartment ventricular model also suggested that the ventricle with an activation delay in 70% of the total volume was most vulnerable to a large activation delay, accompanied by an uncoordinated motion at an earlier activated wall. Taken together, the ratio of the delayed ventricular wall has a significant impact on the pathophysiology due to an activation delay, potentially highlighting an indicator of cardiac dysfunction.

Hereditary hemorrhagic telangiectasia (HHT) is a hereditary disorder that causes refractory nasal bleeding, arteriovenous malformations in the lung (pulmonary arteriovenous fistula: PAVF), central nervous system (CNS-AVF), and liver (hepatic AVF). HHT is caused by genetic abnormalities in endoglin (ENG), ACVRL1 (ALK1), and other rare genes (e.g., SMAD4). The relationship between these gene mutations and the affected organs remains unclear.

Objectives The objective of this study was to assess the utility of 3D printed heart models of congenital heart disease for preoperative surgical simulation. Methods Twenty patient-specific 3D models were created between March 2015 and August 2017. All operations were performed by a young consultant surgeon who had no prior experience with complex biventricular repair. All 15 patients with balanced ventricles had outflow tract malformations (double-outlet right ventricle in 7 patients, congenitally corrected transposition of great arteries in 5, transposition of great arteries in 1, interrupted aortic arch Type B in 1, tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries in 1). One patient had hypoplastic left heart complex, and the remaining 4 patients had a functional single ventricle. The median age at operation was 1.4 (range 0.1-5.9) years. Based on a multislice computed tomography data set, the 3D models were made of polyurethane resins using stereolithography as the printing technology and vacuum casting as the manufacturing method. Results All but 4 patients with a functional single ventricle underwent complete biventricular repair. The median cardiopulmonary bypass time and aortic cross-clamp time were 345 (110-570) min and 114 (35-293) min, respectively. During the median follow-up period of 1.3 (0.1-2.5) years, no mortality was observed. None of the patients experienced surgical heart block or systemic ventricular outflow tract obstruction. Conclusions Three-dimensional printed heart models showed potential utility, especially in understanding the relationship between intraventricular communications and great vessels, as well as in simulation for creating intracardiac pathways.

For neonates with right atrial isomerism (RAI), functional single ventricle (f-SV), and obstructive total anomalous pulmonary venous connection (TAPVC), primary TAPVC repair (TAPVCR) has a poor outcome. At our hospital, the survival rate at 1 year of such neonates undergoing primary TAPVCR between 1999 and 2010 (TAPVCR group) was 30% (3/10). Most deceased cases suffered from capillary leak syndrome and unstable pulmonary resistance after the surgeries. We sought to determine whether less invasive primary draining vein stenting (DVS) improved the outcome of these neonates. We investigated outcomes in consecutive nine such neonates (median gestational age 38 weeks, birth weight 2.8 kg, females 4) who underwent primary DVS with 6-mm-diameter Palmaz® Genesis® stents at our hospital between 2007 and 2017 (DVS group). Eight patients underwent subsequent surgeries to adjust the pulmonary flow after decreased pulmonary resistance. The survival rate at 1 year after the first interventions in the DVS group improved to 77% (7/9), although there was a difference between the interventional eras of the two groups. Of the seven patients who underwent multiple stent redilations with a larger balloon or additional stenting in other sites until the next stage of surgery at a median age of 8 months, four received a bidirectional Glenn (BDG) shunt and TAPVCR and three underwent TAPVCR, with two of those cases reaching BDG. Less invasive primary DVS improved the outcome of neonates with RAI, f-SV, and obstructive TAPVC, with many reaching BDG. Patient selection to advance toward Fontan is thought to further improve the outcome.

Background Heart failure (HF) is an important complication in adults with congenital heart disease (CHD), but because only a few studies have focused on acute HF hospitalization in adults with CHD, we study aimed to define the clinical characteristics of such patients and examine the differences in acute HF between adults with CHD and acquired heart disease.Methods and Results:We retrospectively evaluated 50 adults with CHD admitted for treatment of acute HF and compared their data with those from Japanese HF registries. Patient mean age was 37±15 years and 58% were male. In total, 86% of the patients had complex forms of CHD and 62% had undergone corrective surgery, including the Fontan procedure; 66% of patients showed right heart hemodynamic abnormality. In-hospital mortality was 4%, which was comparable to the Japanese HF registries. Survival rate was 93% at 1 year and 75% at 3 years, which was similarly poor to the rates of HF secondary to acquired heart disease. Conclusions We clarified the clinical characteristics of adults with CHD requiring HF hospitalization. Young adults with complex CHD were hospitalized for management of acute right HF. Short-term and mid-term outcomes were similarly poor compared with acute HF secondary to acquired heart disease.

This study reviewed late clinical features after Kawashima operation to confirm the impact of scheduled subsequent early Fontan completion. Of the 17 consecutive patients who underwent the Kawashima operation between 1987 and 2010, 11 underwent the procedure as inter-stage palliation (scheduled Fontan group). Ten of these patients underwent subsequent early Fontan completion after a median interval of 0.6 years. The remaining 6 patients underwent the Kawashima operation initially as definitive surgery (non-scheduled group). Late Fontan completion was performed in 4 of these patients as salvage surgery to prevent progression of hypoxia after a median interval of 6.6 years. All patients completed follow-up; the mean follow-up period was 12 ± 7 years (range 0.6–28.2). Cumulative survival at 10 years was 66% in the non-scheduled group and 79% in the scheduled group (p = 0.66). Pulmonary arteriovenous malformations developed after Kawashima operation in all 4 patients without antegrade pulmonary blood flow in the non-scheduled group but in only 2 of 11 patients in the scheduled group, both of which completely resolved after Fontan completion. In the scheduled Fontan group, 3 patients developed venovenous malformations between the Fontan pathway and the pulmonary veins or atrium after Fontan completion. Issues related to pulmonary arteriovenous malformations after Kawashima operation resolved with early scheduled Fontan completion. However, data on long-term outcomes are limited and the risk of death continues throughout early life. For unsuitable Fontan candidates, the Kawashima operation with antegrade pulmonary blood flow may provide definitive palliation.

Objective To review long-term respiratory outcomes for tetralogy of Fallot and absent pulmonary valve (TOF/APV) in respiratory symptomatic populations. Methods Of 25 consecutive patients undergoing primary total correction for TOF/APV between 1987 and 2016, Sixteen patients (64%) with a preoperative respiratory disturbance were enrolled. The median age at operation was 1.9 months old, including 4 neonates and 12 infants. Ten patients (62.5%) preoperatively necessitated mechanical ventilator support. During operation, dilated central pulmonary arteries (cPAs) were plicated and retracted anteriorly in all patients, except for the first patient of the study cohort. VSD was completely closed, and the right ventricular outflow tract was reconstructed with a handmade valved conduit before 1990, or a transannular patch with a handmade monocusp after 1991. Results The actuarial survival rate at 20 years was 86.7%. One patient without cPAs plication and another requiring mechanical ventilator support right after his delivery died. The median duration of postoperative mechanical ventilator support was 14 days (range, 1–183). Readmission for respiratory disturbance after discharge was frequently observed before five years of age, however, no patients were readmitted to the hospital after six years of age, during the median follow-up period of 14.7 years, with a maximum of 27.2 years. Although persistent dilatation of cPAs was common, respiratory symptoms never recurred. Conclusions Prognostic and respiratory outcomes after primary total correction for TOF/APV in respiratory symptomatic populations were good. Successful relief of tracheal/main bronchial compression by primary total correction at the neonatal or infantile period provided delayed, but nonrecurrent improvement of respiratory disturbances.