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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 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.

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.

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.

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.

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.

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.

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.

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.

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.

Objectives: Although venography guidance is helpful for central venous catheter placement, it is sometimes difficult to place a peripheral intravenous cannula for enhancement. We designed a new technique for establishing femoral venous access using venography guidance in the return phase of peripheral arteriography. This new technique was named arterial injection venous return guidance. Here we assessed the efficacy and safety of arterial injection venous return guidance. Methods: We reviewed data of 29 infants less than 6 months old undergoing catheter intervention at our institute in 2014. Of the 29 patients, femoral venous cannulation was performed using arterial injection venous return guidance in 5 patients, venography in 20 patients, and the landmark method in 4 patients. The technical success rates and incidence of complications were compared. Results: The overall success rates were 100% in the arterial injection venous return-guided and venography-guided groups. The mean procedure duration and mean contrast material injection time were similar between the groups. The contrast effect on the femoral vein in the arterial injection venous return-guided group was lower than that in the venography-guided group, but adequate for surgery. The overall complication rate was 17%, and obstruction of previously placed intravenous catheters was the most common complication. Conclusions: Therefore, the arterial injection venous return guidance technique was as safe and efficient as venography for establishing venous access.

OBJECTIVES The aim of this study was to reveal the serial changes in tricuspid regurgitation (TR) after anatomic repair for congenitally corrected transposition of great arteries. METHODS Between 1995 and 2018, 48 patients underwent anatomic repair (atrial/arterial switch in 14 patients, atrial switch and Rastelli in 34 patients). The mean age and weight of the patients during anatomic repair was 33 (interquartile range 21.8–62.1) months and 12 (10.3–16.3) kg. The preoperative TR was less than mild in 15 patients (31.3%), mild-to-moderate in 29 patients (60.4%) and more-than-moderate in 4 patients (8.3%). Ebsteinoid dysplasia of the tricuspid valve (TV) was observed in 7 patients (14.6%). During the study period, no patient underwent TV surgery or bidirectional Glenn anastomosis at the time of anatomic repair. RESULTS There was 1 in-hospital death and 1 late death. The follow-up was completed by other surviving patients, with a median follow-up period of 12.1 years (5.9–18.1). The overall survival, reoperation-free survival and freedom from permanent pacemaker implantation rate at 15 years were 94.3%, 74.3% and 81.5%, respectively. The mean TR grade was 2.0 (1.0–2.6) preoperatively, 2.0 (1.0–2.0) at 1 year, 2.0 (2.0–2.0) at 5 years and 2.0 (2.0–2.0) at 10 years after anatomic repair. A Cox proportional hazards model showed that association of Ebsteinoid dysplasia of the TV, type of anatomic repair and previous pulmonary artery banding did not affect freedom from death or the more-than-moderate TR rate. There were 2 patients who underwent TV surgery after the anatomic repair for severe TR; TV repair was successfully done for 1 patient, the other required semi-closure of TV and one and one-half ventricle conversion. CONCLUSIONS TR remained subclinical or improved in the majority of patients after anatomic repair without TV repair. However, there were a few patients whose TR progressed to severe or massive, then required TV surgery after anatomic repair. Although exposure was difficult, TR was sometimes repairable following atrial switch, otherwise, one and one-half ventricle repair conversion would be the choice of treatment.

OBJECTIVES Our goal was to assess the efficacy of managing pulmonary blood flow from the Norwood procedure with a right ventricle-to-pulmonary artery (RV–PA) conduit until stage 2 palliation (S2P). METHODS Among 48 consecutive patients undergoing the Norwood procedure between 2008 and 2018, 40 (83.3%) patients who survived to discharge were included in this study. The primary diagnosis was hypoplastic left heart syndrome in 28 (70%) patients and hypoplastic left heart syndrome variant in 12 (30%) patients. All patients received bilateral pulmonary artery banding. The median age and weight at the time of the Norwood procedure were 41 (25th–75th percentiles: 27–89) days and 3.2 (2.7–3.9) kg, respectively. In keeping with institutional strategy, S2P was undertaken when body weight exceeded 5.0 kg, and normal gross motor development was confirmed. RESULTS The RV–PA conduit was clipped in 28 (70%) patients during the perioperative period of the Norwood procedure, then partial unclipping was performed in 8 (20%) patients and full unclipping was performed in 20 (50%) patients. Before S2P, the median pulmonary-to-systemic blood flow ratio was 1.0 (0.7–1.3). The median age and weight at the time of S2P were 10.7 (9.0–12.9) months and 6.3 (5.5–7.1) kg, respectively. The survival rate 5 years after Norwood discharge was 85.3%. Pre-S2P pulmonary-to-systemic blood flow ratio was linearly correlated with greater interstage changes in systemic atrioventricular valve regurgitation (R2 = 0.223, P = 0.004). CONCLUSIONS Interstage management of pulmonary blood flow by RV–PA conduit clipping and gradual unclipping provided good interstage outcomes. The median pulmonary-to-systemic blood flow ratio could be controlled to 1.0 at pre-S2P catheter examination.

OBJECTIVES The aim of this study was to identify the long-term therapeutic effect of total cavopulmonary connection (TCPC) conversion with an extracardiac conduit. METHODS Between 1991 and 2014, 36 patients underwent TCPC conversion with an extracardiac conduit. Half of these patients were diagnosed with tricuspid atresia or its variant. The left ventricle was dominant in 26 patients (72.2%). Median age at conversion and interval from initial Fontan operation to conversion were 24.1 years (interquartile range 18.9–29.2) and 17.8 years (15.4–20.9), respectively. Surgical cryoablation was concomitantly performed in 32 patients (88.9%). Cardiac catheter examination was performed preoperatively (36 patients, 100%) and at 1 year (31 patients, 86%), 5 years (25 patients, 69%) and 10 years (13 patients, 36%) after TCPC conversion. Symptom-limited treadmill exercise with expired gas analysis was performed preoperatively (32 patients, 88.9%) and at 1 year (27 patients, 75.0%), 5 years (20 patients, 55.6%) and 10 years (12 patients, 33.3%) after conversion. RESULTS All patients received follow-up; the mean follow-up period was 8.2 ± 4.8 years. Actuarial survival rate, protein-losing enteropathy-free survival rate and rate of survival with sinus rhythm maintenance at 10 years were 79.2%, 67.8% and 48.5%, respectively. The survival curve declined steeply when the duration of Fontan circulation exceeded 25 years. New cases of protein-losing enteropathy developed postoperatively in 2 patients. Permanent pacemakers were implanted in 12 patients (33%), but atrial tachyarrhythmia was not sustained in any of the remaining patients. Pulmonary arterial pressure (11.0 ± 3.1 to 9.5 ± 3.6 mmHg, P = 0.003), pulmonary vascular resistance (2.1 ± 0.7 to 1.3 ± 0.5 WU/m2, P CONCLUSIONS Owing to its anti-arrhythmic effect and Fontan pathway recruitment effect, TCPC conversion with an extracardiac conduit prevented the natural decline of exercise tolerance that is seen in classic Fontan patients.

Although adverse effects of phrenic nerve palsy (PNP) on early Fontan circulation have been reported, detailed late impact remains unclear.Of 218 patients undergoing extracardiac total cavopulmonary connection between 1995 and 2008, 160 who all underwent cardiac catheter examination, spirometry, and exercise capacity testing 10 years after the operation were enrolled. The cohort was divided into 2 groups: with (N = 21) or without PNP (control group, N = 139). The patients with PNP were further divided into the recovered PNP group (n = 10) and the persistent PNP group (n = 11). All but 2 patients who developed PNP (90.9%) underwent diaphragmatic plication. There was no difference in hemodynamic indices at pre-Fontan evaluation among the three groups.Ten years after the Fontan procedure, the averaged forced vital capacity was 81% ± 18% of predicted in the control group, 86% ± 17% in the recovered PNP group, and 56% ± 12% in the persistent PNP group (P.001). Peak oxygen consumption was linearly correlated to the forced vital capacity (r = 0.222, P = .009). There was no significant difference in the peak oxygen consumption between groups. Significant veno-venous collaterals into the diaphragm from lower body to pulmonary vein(s) or atria more frequently developed in patients who underwent diaphragmatic plication compared with those who did not (P.001).Persistent PNP resulted in reduced forced vital capacity; however, its influence on exercise intolerance could not be identified. Diaphragmatic plication should be reserved for patients who experience clinically significant respiratory or hemodynamic sequelae of PNP.

To evaluate the significance of natriuretic peptide (NP) levels in fetal arrhythmia. Cardiovascular profile (CVP) scores and umbilical vein (UV) NP levels at birth were compared by different fetal arrhythmia statuses. Fetal tachyarrhythmia (n = 22), bradyarrhythmia (n = 12), extrasystole (n = 12) and controls (n = 127) were enrolled in this study. Fetal antiarrhythmic therapy was performed in fetuses with tachyarrhythmia (n = 18) and bradyarrhythmia (n = 5). Fetal arrhythmias were divided into three groups: group A (arrhythmia controlled at birth, n = 17), Group B (arrhythmia uncontrolled at birth, n = 9) and Group C (fetal therapy not indicated, n = 20). Group B had significantly lower CVP scores and higher NP levels than the other two groups and controls (p UV NP concentrations reflect the severity of fetal arrhythmia and responses to fetal therapy.

Background Outcomes of surgical intervention for severe tricuspid regurgitation related to Ebstein anomaly or tricuspid valve dysplasia in the neonatal period, particularly when associated with pulmonary atresia, are extremely poor. However, owing to emerging innovative surgical techniques, such as cone reconstruction, outcomes of tricuspid valve plasty in the neonatal period have gradually improved. Methods The study retrospectively reviewed the medical records of 12 neonates who were diagnosed with severe tricuspid regurgitation and pulmonary atresia related to Ebstein anomaly (n = 9) or isolated tricuspid valve dysplasia (n = 3) between 2000 and 2013. Results The first 6 patients underwent palliative therapy in anticipation of future functional single-ventricle palliation (Starnes operation). Biventricular repair was performed in the 6 patients born after 2012. As tricuspid valve plasty, cone reconstruction has been applied since 2013. Five patients underwent a Starnes operation, and 5 patients underwent biventricular repair, including 4 cone reconstructions. Four of the 5 patients who underwent a Starnes operation died in-hospital; the remaining patient underwent a Fontan operation at age 2 years. Three of the 5 patients who underwent biventricular repair survived. On echocardiogram, the 3 survivors who were treated with biventricular repair had a preoperative tricuspid regurgitation flow velocity greater than 3.0 m/s. Conclusions Biventricular repair with cone reconstruction can be applied for severe tricuspid regurgitation due to neonatal Ebstein anomaly or tricuspid valve dysplasia with associated pulmonary atresia. A tricuspid regurgitation flow velocity greater than 3.0 m/s may be an indicator of successful biventricular repair.

BACKGROUND We have previously demonstrated that umbilical cord plasma natriuretic peptide (NP) levels reflect the severity of heart failure (HF) in fetuses with congenital heart defects (CHD). The aim of this study was to evaluate the significance of amniotic fluid (AF) NP levels in the assessment of HF in fetuses with CHD or arrhythmia. Methods and Results: This was a prospective observational study at a tertiary pediatric cardiac center. A total of 95 singletons with CHD or arrhythmia, and 96 controls from 2012 to 2015 were analyzed. AF concentrations of atrial NP (ANP), B-type NP (BNP) and N-terminal pro-B-type NP (NT-proBNP) at birth were compared with ultrasonographic assessment of fetal HF using the cardiovascular profile (CVP) score. Multivariate analysis showed that a CVP score ≤5 and preterm birth are independently associated with high AF NT-proBNP levels. AF NT-proBNP levels of fetuses with CHD or arrhythmia inversely correlated with CVP score (P for trend

Left ventricular noncompaction (LVNC) is a distinct cardiomyopathy that is morphologically characterized by a two-layered myocardium, numerous prominent trabeculations, and deep intertrabecular recesses communicating with the left ventricular cavity. We present a case report regarding the identification of a new mutation in TNNI3 in a patient with LVNC using next-generation sequencing. A 13-year-old girl who had no family history of cardiac disease was hospitalized with dyspnea after exercise and electrocardiographic abnormalities during a school screening. Based on her clinical features, she was diagnosed with LVNC. Via genetic analysis, a TNNI3 heterozygous missense variant was identified in the proband. Although mutations in TNNI3 have been reported in patients with hypertrophic cardiomyopathy and restrictive cardiomyopathy, this is the first report of a mutation in this gene in a patient with LVNC.

Background Poor left ventricular (LV) growth and diastolic dysfunction long after simple total anomalous pulmonary venous connection (TAPVC) repair has been well documented and is believed to originate from insufficient preoperative volume preload. The objective of the study was to confirm these findings. Methods Of 61 patients undergoing simple TAPVC repair between 1996 and 2016, 42 patients undergoing postoperative catheter examinations were enrolled. The mean age at the time of repair was 39 ± 117 days. Postoperative catheter examinations were conducted at a mean duration of 1.1 years after the repair. LV end-diastolic volume (LVEDV) was calculated by biplane cineangiography with the use of Simpson's method as a Graham modification. Results LV ejection fraction, LV end-diastolic pressure (LVEDP), and cardiac index were 72% ± 5%, 10.3 ± 2.7 mm Hg, and 4.0 ± 0.7 L •·min−1 • m−2, respectively. LVEDV was 102% ± 16% of the predictive normal value, and it strongly correlated with the predictive normal value of LV end-diastolic diameter (LVEDd) calculated by two-dimensional echocardiography (R2 = 0.29, p = 0.005). LVEDd maintained a normal range thereafter during the entire follow-up period. LVEDP correlated linearly with age at the time of TAPVC repair (R2 = 0.18, p = 0.007) but not with LVEDV (p = 0.67). Conclusions LV size maintained a normal range after the repair of simple TAPVC. High LVEDP was frequently observed a year after repair; it did not correlate with LV size but had a strong negative correlation with age at the time of repair.

Objectives To assess the feasibility of hybrid Stage I palliation consisting of bilateral pulmonary artery bandings and ductal stenting for patients with 2 ventricular cavities and hypoplastic left heart structures. Methods Eleven consecutive patients who underwent hybrid Stage I palliation between 2010 and 2017 were enrolled. The diagnoses were interrupted aortic arch/coarctation of the aorta, ventricular septal defect and significant left ventricular (LV) outflow tract obstruction in 5 patients, critical aortic stenosis and reduced LV contraction in 3 patients and hypoplastic left heart complex in 3 patients. Results The median age at definitive surgery was 12 months (range 6-22 months). During the mean follow-up period of 24 months (range 9-83 months) following the definitive surgery, there was 1 death. Two patients with interrupted aortic arch/coarctation of the aorta did not undergo the Yasui operation but underwent arch repair and ventricular septal defect closure after the growth of the aortic valve and LV outflow tract. For 2 of the 3 patients with critical aortic stenosis, biventricular repair was performed. Of the 3 patients with hypoplastic left heart complex, 2 patients showed growth of the mitral valve and left ventricle following LV rehabilitation by balloon pulmonary artery dilatation or surgical debanding of the banded pulmonary arteries and subsequently underwent biventricular repair, which resulted in 1 death. Conclusions Hybrid Stage I palliation would be a safe and beneficial treatment for patients with 2 ventricles, as a bridge to decide whether and how to achieve a biventricular repair and whether it should be preceded by a preliminary LV rehabilitation.

OBJECTIVES: Common atrioventricular valve (CAVV) regurgitation is widely known as a risk factor for mortality and Fontan completion in patients with functional single ventricle. Hence, we reviewed our surgical experience with CAVV plasty in Fontan candidates. METHODS: Staged Fontan strategy and extracardiac total cavopulmonary connection as Fontan modification were our principal approaches in 1995. Since then, 38 consecutive Fontan candidates (21 males, median weight at operation was 7.0 kg and median age was 17 months old) underwent CAVV plasty. Right atrial isomerism was associated with 24 patients. The initial CAVV plasty was performed before inter-stage bidirectional Glenn (BDG) in 3 patients, at BDG in 23, before Fontan in 4 and during Fontan in 8. Since 1995, the modified Alfieri technique with a tailed, expanded, polytetrafluoroethylene tube as a bridging strip was the procedure for repair and 27 patients underwent the procedure. The mean follow-up period was 7.1 years (range 0–17 years). RESULTS: Actuarial survival and freedom from CAVV replacement rates at 1, 5 and 10 years were 81, 70 and 67% and 89, 85 and 75%, respectively. Seven patients ultimately underwent CAVV replacement with one death. Twenty-three of the 38 patients completed Fontan operation (61%). Association with total anomalous pulmonary venous connection (P=0.01) and CAVV plasty before BDG (P= 0.05) were risk factors for mortality. CONCLUSIONS: CAVV plasty for patients with functional single ventricle is still challenging; however, the aggressive and repeated surgical intervention may contribute to provide better life-prognosis. The ventricular volume unloading effect of BDG without additional pulmonary blood flow or Fontan operation did not contribute to maintain CAVV function. Therefore, there would not be any hesitation for CAVV replacement to control CAVVR in the setting of systemic ventricular failure. Although the statistically significant therapeutic superiority of the modified Alfieri technique was not shown so far, further follow-up may reveal the advantage of this easy and simple technique.