Assessment of left atrial deformation in patients with total anomalous pulmonary venous connection by two-dimensional speckle-tracking echocardiography.

BACKGROUND: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease of newborns characterized by impaired left ventricle growth and diastolic dysfunction. We hypothesized that the patients with TAPVC reduced blood flow into the left heart prenatally could affect left atrium (LA) not just growth but function. We compared the age-related changes in LA deformation using two-dimensional speckle-tracking echocardiography (2DSTE) in Patients with TAPVC. METHOD: This single-center, retrospective cohort study was conducted on consecutive isolated TAPVC patients who underwent neonatal surgery between January 1, 2009 and January 1, 2022. The LA datasets in TAPVC patients were analyzed before surgery (n = 28) and follow-ups at 1-2 (n = 24) and 5-7 years of age (n = 13) and compared with those of age-matched healthy controls (January 2009-2022). The LA strain (ε), indicating LA function, was analyzed using QLAB represented by reservoir (εR), conduit (εCD), and contractile (εCT) strains. LA pressure was evaluated by periodic follow-up catheterization after repair. RESULTS: Compared to the controls, the TAPVC patients had significantly smaller LA maximum volume preoperatively, and with age, the LA maximal volumes reached normal levels, while the LA minimal volumes were larger. All 2DSTE-determined LA strains showed significant reductions at all time points in the TAPVC group compared to those in the control (median εR, εCD, and εCT; before surgery: 17.0% vs. 26.0%, 12.9% vs. 15.9%, and 6.3% vs. 10.4%; follow-up at 1-2 years: 30.0% vs. 45.7%, 23.2% vs. 29.6%, and 6.1% vs. 16.3%; follow-up at 5-7 years: 31.2% vs. 43.1%, 25.0% vs. 31.2%, and 5.2% vs. 10.8%, respectively; p < 0.05). Only εCT did not represented a significant change over time even though after correction of blood flow (median εCT: 6.0% → 5.9%). Patients with pulmonary venous obstruction (PVO) at birth showed significantly decreased εR and εCD and higher LA pressure compared to those without PVO. CONCLUSION: This study showed that nevertheless maximum volume of LA was recovered within the normal range, reduced LA strains, especially contractile function lasted from birth even after repair in Patients with TAPVC.

Microvascular Endothelial Function Assessed Using Peripheral Arterial Tonometry in Adolescents with Repaired Congenital Heart Disease.

Atherosclerosis can develop in adult patients with congenital heart disease (CHD) and should be given attention. Endothelial function is well known as a predictor of the development of atherosclerosis but has not been well investigated in patients with repaired CHD. This study aimed to clarify the endothelial function and its relationship with clinical backgrounds and parameters in adolescents with various types of repaired CHDs. Endothelial function was evaluated using peripheral arterial tonometry (PAT). The reactive hyperemia index (RHI) was evaluated and compared between adolescents with repaired CHD and those in the control group. The relationship between the clinical background and parameters was also investigated in patients with repaired CHD. Forty-eight patients with repaired CHD (age 14.0 ± 3.3 years) and 114 healthy volunteers were included in this study. Patients with repaired CHD comprised 16 with repaired non-cyanotic CHD, 14 with repaired tetralogy of Fallot, and 18 who underwent the Fontan procedure. RHI in the repaired CHD group was significantly lower than in the control group. There was no significant correlation between the RHI and blood biochemical markers, such as uric acid, creatine, and brain natriuretic peptide levels. The RHI was significantly higher in patients taking angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) than in those not taking them. Endothelial function was impaired in adolescents with repaired CHD compared to that in the control group. Microvascular endothelial dysfunction developed even in adolescents with simple non-cyanotic CHD.

Hemoptysis due to progressive scoliosis associated with congenital heart disease: a case report.

BACKGROUND: Patients with congenital heart disease (CHD) are associated with an increased incidence of scoliosis, often with severe progression. We report a case of hemoptysis caused by rapid scoliosis progression subsequent to surgery for CHD that was successfully managed by surgical curve correction following coil embolization. CASE PRESENTATION: A 14-year-old girl with scoliosis had undergone open heart surgery for CHD at the age of 1 year. She was first noted to have scoliosis at 12 years of age, which began to progress rapidly. At age 13, her main thoracic curve Cobb angle was 46°, and hemoptysis with high pulmonary vein pressure due to vertebral rotation was detected. Nine months after coil embolization, she received posterior spinal fusion from T5 to L2 for scoliosis correction. Postoperatively, her pulmonary vein diameter was enlarged, with no detectable signs of hemoptysis. CONCLUSIONS: We encountered a case of hemoptysis caused by advanced scoliosis after cardiac surgery that was successfully treated by correction of the scoliotic curve following coil embolization. Patients with secondary scoliosis after surgery for CHD should be carefully monitored for the possibility of cardiovascular system deterioration.

Clinical impact of subcutaneous treprostinil in trisomy 21 patient with pulmonary arterial hypertension associated with CHD.

Subcutaneous treprostinil is commonly used to improve idiopathic pulmonary arterial hypertension in children. However, its effectiveness has not been reported in trisomy 21. We report the case of 9-year-old boy in trisomy 21 with CHD-pulmonary artery hypertension after surgical correction of CHD. Haemodynamics and exercise capacity dramatically improved with a transition from oral selexipag to subcutaneous treprostinil.

Novel Urinary Biomarkers for Acute Kidney Injury and Prediction of Clinical Outcomes After Pediatric Cardiac Surgery.

Acute kidney injury (AKI) is a serious complication of pediatric cardiac surgery, with high morbidity and mortality. We aimed to evaluate the perioperative risk factors for AKI, and the validity of novel diagnostic urinary biomarkers after pediatric cardiac surgery. We analyzed 103 consecutive pediatric patients (≤ 18 years old), who underwent cardiac surgery. AKI was defined by ≥ 50% increase in serum creatinine levels from baseline. Urinary liver-type fatty acid binding protein (L-FABP) and neutrophil gelatinase-associated lipocalin (NGAL) were measured postoperatively at the intensive care unit (ICU) admission, subsequently at 4, 12, and 24 h. Areas under the receiver-operating characteristic curves (AUC) were calculated at each assessment time. AKI had developed in 47 patients (45.6%) by the second postoperative day. Univentricular status, aortic cross-clamping time, and intraoperative fluid balance were independently associated with AKI (p = 0.02, 0.01 and 0.01, respectively). Urinary L-FABP and NGAL were significantly higher in the AKI group at each point (p < 0.05). The predictive abilities of both biomarkers (AUC = 0.78-0.90) at ICU admission and 4 h after were especially high. The patients with L-FABP greater than the cutoff value at ICU admission and 4 h after ICU admission had significantly longer intubation and hospitalization periods (p < 0.05). Those with elevated NGAL levels at admission, and 4 h and 24 h after ICU admission, had significantly longer intubation, ICU stay, and hospitalization (p < 0.05). L-FABP and NGAL can be useful biomarkers for detecting early AKI after pediatric cardiac surgery and predicting adverse clinical outcomes.

Establishment of a Healthcare System for Patients With Adult Congenital Heart Disease in Collaboration With Children's Hospital　- The Nagano Model.

BACKGROUND: Despite the best efforts of pediatricians, healthcare for adult patients with congenital heart disease (ACHD) has proven challenging because of the increased numbers. This study presents the process of establishing an ACHD care system as a collaborative effort between Shinshu University Hospital and Nagano Children's Hospital. Methods and Results: Establishing an outpatient clinic for transition, a cooperation agreement for in-patient care between the 2 hospitals, and quality management of diagnostic imaging and educational meetings for adult cardiologists were the 3 major challenges. Of the 99 patients who visited the transition clinic in the children's hospital between May 2014 and December 2016, 3 returned to the pediatrician's clinic. Between June 2013 and December 2017, 273 patients visited the ACHD center in Shinshu University Hospital. Until December 2017, mortality and fatal arrhythmia were noted in 3 and 2 cases, respectively. Catheter ablation for arrhythmia was performed in 12 cases, and 4 cases of pregnancy with moderate/severe ACHD or estimated as high risk were managed with healthy livebirths. Surgical interventions for moderate/severe ACHD were performed in collaboration with the children's hospital or Sakakibara Heart Institute. CONCLUSIONS: Patients were successfully transferred to adult cardiology departments. Surgical and nonsurgical interventions for ACHD were provided. Collaboration between adult and pediatric cardiologists assists in the establishment of healthcare systems for ACHD.

Neo-aortic insufficiency late after staged reconstruction for hypoplastic left heart syndrome: impact of differences in initial palliative procedures.

The neo-aortic insufficiency in patients with hypoplastic left heart syndrome is an important sequela. We assessed the risks of the neo-aortic valve deterioration by the difference of initial palliations: Group I underwent primary Norwood (Nw) with systemic-to-pulmonary artery shunt (SPS), Group II underwent bilateral pulmonary artery banding (bPAB) and subsequent Nw with SPS (bPAB-Nw/SPS), Group III underwent bPAB and subsequent Nw with bidirectional Glenn (BDG) procedure (bPAB-Nw/BDG). The neo-aortic valve z score changes over time did not reach statistical significance in all groups (p = 0.43 for Group I, 0.20 for Group II, and 0.30 for Group III). The degree of neo-aortic valve insufficiency did not change significantly over time during this period except for Group III (p = 0.34 for Group I, 0.20 for Group II, and 0.02 for Group III). On the other hand, dimensions of the neo-aortic annulus and degrees of neo-aortic insufficiency did not differ significantly among the 3 groups at any pre-determined time. The presence or absence of incision into the sino-tubular junction at Nw did not affect the late neo-aortic valve z score or insufficiency. These data indicate that the difference of initial palliative procedures does not affect late neo-aortic valve insufficiency in Nw survivors. Because valve failure may develop in longer follow-up, further observation should be conducted.

Development of suction force during early diastole from the left atrium to the left ventricle in infants, children, and adolescents.

Although the suction force that moves blood into the left ventricle during early diastole is thought to play an important role in diastolic function, there have been a few studies of this phenomenon in normal children. Suction force is measured as the intraventricular pressure difference (IVPD) and intraventricular pressure gradient (IVPG), which is calculated as IVPD divided by left ventricular length. The purpose of this study was to determine the suction force in infants, children, and adolescents using IVPD and IVPG. We included 120 normal children categorized into five groups based on age: G1 (0-2 years), G2 (3-5 years), G3 (6-8 years), G4 (9-11 years), and G5 (12-16 years). The total, basal, and mid-apical IVPD and IVPG were calculated using color M-mode Doppler imaging of the mitral valve inflow using the Euler equation. The total IVPD increased with age from G1 to G5 (1.75 + 0.51 vs. 2.95 + 0.72 mmHg, respectively; p < 0.001), due to an increase in mid-apical IVPD with constant basal IVPD. Although total IVPG was constant, mid-apical IVPG was larger in G5 than in G1 (0.21 + 0.06 vs. 0.16 + 0.07 mmHg/cm, respectively; p = 0.006). Total, basal, and mid-apical IVPDs were significantly correlated with age and the parameters of heart size and mitral annular e'. Mid-apical IVPG correlated with age and e' positively, but basal IVPG did with age negatively and did not with e'. The suction force increased at the mid-apical segment, correlating with increasing heart size and developing left ventricular relaxation, even after adjustment for left ventricular length.

Measurement of Aortic Valve Coaptation and Effective Height Using Echocardiography in Patients with Ventricular Septal Defects and Aortic Valve Prolapse.

Decreased coaptation height in adults has been identified as a marker of early valve failure. We evaluated aortic valve coaptation and effective height in healthy children and in children with a ventricular septal defect (VSD) with aortic cusp prolapse (ACP), using echocardiography. We included 45 subjects with VSD with ACP, 27 did not develop aortic regurgitation (AR) by ACP and 18 developed AR by ACP, and 83 healthy children as controls. Aortic root anatomy was estimated using the parasternal long-axis view. We measured the diameter of aortic valve (AV), coaptation height (CH), and effective height (EH) of the aortic valve. We defined the ACH (CH/AV ratio) and AEH (EH/AV ratio) indices as follows: [Formula: see text]. There were significant differences in ACH and AEH between the groups (control vs VSD with ACP vs VSD with ACP and AR, median ACH [%], 35.1 vs 32.0 vs 22.1; median AEH [%], 52.0 vs 48.0 vs 34.4, respectively; P < 0.01]). Intra-cardiac repair (ICR) was performed in 15 cases. Significant increases were observed in ACH and AEH before and after ICR (median ACH [%], before: 27.0, after: 32.7, P < 0.05; median AEH (%), before 38.5, after 45.8, P < 0.05). Measurement of ACH and AEH may allow direct and non-invasive assessment of the severity of VSD with ACP, which could aid clinicians in determining the need and timing for surgical intervention.

Assessment of the developmental change in the left atrial volume using real time three-dimensional echocardiography.

AIMS: Real time 3D echocardiography (RT3DE) has been applied for the assessment of left atrial (LA) function in patients with adult heart disease; however, LA function is not well known in children. We aimed at determining the normal range of LA volume (LAV) using RT3DE and the feasibility and reproducibility of this method in healthy subjects and at elucidating the developmental changes in the LAV with aging. METHODS AND RESULTS: In this study, 359 healthy people (mean age, 23.9 ± 21.3; range, 0.1-76.4 years) were enrolled. We performed transthoracic RT3DE and measured the maximum and minimum LAV. Simultaneously, we measured the LAV using the 2D biplane Simpson's method. Inter-observer and intra-observer variability and the agreement of LAV measurements between RT3DE and 2DE were assessed in a subset of subjects. The RT3DE feasibility for LAV measurement was 93%. Both maximum and minimum LAVs exponentially increased with age and linearly increased with increasing of body surface area (BSA). The LA distensibility, which demonstrates LA reservoir function, decreased with age and BSA. The LAVs measured by RT3DE were significantly smaller than those measured by the 2D biplane Simpson's method. The 3D volumetric method had favorable intra-observer and inter-observer agreement. CONCLUSION: The reference values of LAV from early infancy to adulthood were obtained using RT3DE, which could be useful for future studies in children with congenital heart disease. RT3DE is a reproducible method and a feasible tool for evaluating the LAV in children. LA reservoir function is likely to decrease with age and increasing of body size.

Guidance for performance, utilization, and education of cardiac and lung point-of-care ultrasonography from the Japanese Society of Echocardiography.

In recent years, bedside ultrasound examinations have been used in many clinical departments and are called point-of-care ultrasound (POCUS). Regarding POCUS in the cardiac field, a protocol called focus (focused) cardiac ultrasound (FoCUS) has been developed in Europe and the United States, is being used clinically, and an educational syllabus has been created. According to them, FoCUS is defined as a point-of-care cardiac ultrasound examination using standardized limited sections and protocols. FoCUS is primarily intended to be performed by non-cardiologists, and in order to avoid making mistakes in judgment, it is important to be familiar with its limitations and it is necessary to understand pathological conditions that can only be diagnosed using conventional comprehensive echocardiography. The Japanese Society of Echocardiography has edited this clinical guideline because we believe that FoCUS should be used effectively and appropriately in Japan, and that appropriate education is essential to popularize FoCUS in Japan. Furthermore, lung POCUS has recently come into clinical use. Lung POCUS is useful for the diagnosis and follow-up of heart failure when used in conjunction with FoCUS, and is especially useful in primary care where chest X-rays are not available. The working group that created this manual agreed that it is desirable to educate patients about lung POCUS in conjunction with FoCUS, so we decided to include the basic techniques of lung POCUS and how to use them in this manuscript.

Multiple atrial septal defects closure with a single device using radiofrequency energy-assisted wire atrial septostomy: A new method.

Percutaneous closure of multiple atrial septal defects can be more challenging. It is often discussed whether a single or dual device closure is appropriate for two or more large atrial septal defects with insufficient distance between defects. In this case, we used radiofrequency energy-assisted wire atrial septostomy to break intervening tissue between two adjacent oval fossa defects, thereby combining them into a single hole and facilitating device closure using a single device. This technique could be considered in patients with multiple adjacent secundum defects separated by intervening tissue.

Normal range of left ventricular 2-dimensional strain: Japanese Ultrasound Speckle Tracking of the Left Ventricle (JUSTICE) study.

BACKGROUND: To determine the normal range of left ventricular (LV) 2- dimensional (2-D) strain and vendor-specific differences, a multicenter prospective 2-D strain study endorsed by the Japanese Society of Echocardiography was conducted. METHODS AND RESULTS: 2-D speckle tracking analysis was performed on 817 healthy subjects (age range, 0-88 years); the images included 3 LV short axis and 3 apical views using an ultrasound system from 1 of the 3 different vendors (V(1), n=333; V(2), n=330; V(3), n=337). With the 2-D speckle tracking software from each vendor, radial, circumferential and longitudinal strain were measured using an 18-segment model. Inter-vendor variability was also assessed in a subset of subjects. The feasibility for 2-D strain measurements was different among the 3 vendors (V(1), 83%; V(2), 70%; V(3), 88%, P<0.01). The global radial (V(1), 54.6±12.6%; V(2), 36.3±8.2%; V(3), 51.4±8.0%), circumferential (V(1), -22.8±2.9%; V(2), -22.2±3.2%; V(3), -30.5±3.8%), and longitudinal (V(1), -21.3±2.1%; V(2), -18.9±2.5%; V(3), -19.9±2.4%) strain measurements were significantly different for each of the vendors. Segmental strain was also different between the 3 vendors. On inter-vendor analysis, vendor agreement ranged from mild to moderate. CONCLUSIONS: Reference values are provided for normal 2-D strain for 3 different ultrasound vendors. Due to a low inter-vendor agreement, 2-D strain data are not interchangeable when conducting a longitudinal follow-up or a cross-sectional assessment of LV function.

[Successful application of cholangioscope as an intravascular endoscope in hybrid operation for a child].

We performed hybrid operation on a 3-year-old boy with thrombosis in the pulmonary arterial conduit which had been implanted concomitantly at the time of Fontan operation. We used a cholangioscope as a substitute of intravascular endoscope. It visualized the organized thrombus and the suture line in the conduit. Hybrid operation was successfully performed based on the detailed findings gained by cholangioscopy.

Left ventricular torsion and strain in patients with repaired tetralogy of Fallot assessed by speckle tracking imaging.

BACKGROUND: In patients with tetralogy of Fallot (TOF), left ventricular (LV) dysfunction is an important factor associated with poor clinical outcome. OBJECTIVE: The purpose of this study was to investigate the torsion and multidirectional strain of the LV in patients with TOF. METHODS: Echocardiographic images were prospectively acquired in 29 patients who underwent TOF repair (age range, 5-25 years) and in 29 normal controls. Torsion and circumferential and longitudinal strain of the LV were assessed using speckle tracking imaging. RESULTS: The torsion in patients was smaller compared to that in the controls due to small apical rotation and/or inverse basal rotation (P < 0.01). Torsion and untwisting rates decreased with increasing age (R = 0.37, P < 0.05). Basal circumferential strain and strain rate (SR) at systole and diastole decreased with age (R = 0.58; R = 0.57; R = 0.57, all P < 0.001) and were smaller in patients compared to those in the controls (all P < 0.01). Septal longitudinal strain and SR at systole and diastole decreased with age (R = 0.52; R = 0.62; R = 0.71, all P < 0.001) and were smaller than those of the controls (P < 0.01), although lateral longitudinal strain and SR were relatively maintained. CONCLUSION: Abnormal torsion and strain pattern of the LV were observed in patients without symptoms of cardiac failure. Assessment of torsion and strain is a very sensitive tool to detect the early deterioration of LV function in patients with TOF.

[Pulmonary valve replacement due to ectopic ossification on expanded polytetrafluoroethylene cusp after tetralogy of Fallot repair; report of a case].

Operative correction of tetralogy of Fallot has been performed for more than 50 years and well established. To date, the literature on reoperation has focused and indications and the long-term outcome have not been well defined. We herein report a case of pulmonary valve replacement due to ectopic ossification on expanded polytetrafluoroethylene (ePTFE) cusp in a female child who had undergone right ventricular outflow tract reconstruction for tetralogy of Fallot with pulmonary atresia. Because of severe right ventricular dilatation with end diastolic volume of 186 ml/m2 on cardiac catheterization, redo operation was planned for functional recovery of the right ventricle. Previous transannlar patch was removed and pulmonary valve replacement was performed using CEP 23 mm. A hard calcification was observed around the cusp of transannular patch. Pathological findings revealed ectopic ossification with osteoblast colonization around the ePTFE cusp. This is the 1st report of ectopic ossification on ePTFE.

[Acute coronary syndrome due to abnormal fibrous tissue growth after Aubert-Imai modification for transposition of the great arteries].

We report a female infant with acute coronary syndrome after Aubert-Imai modification for transposition of the great arteries. Two months postoperatively, she visited the emergency room because of perspiration and tachypnea. She was diagnosed with acute coronary syndrome by blood chemistry examination. Cardiac catheterization revealed Aubert route stenosis, and emergency operation was performed. The lumen around the aortopulmonary window was almost totally occluded by fibrous tissue extending from the equine pericardial patch. This fibrous tissue was completely resected and the aortopulmonary window was deepened to enlarge the coronary ostium. We recommend a close follow-up including angiography after these procedures.

[Evaluation of cerebral oxygenation changes with clamping of the left superior vena cava by near-infrared spectroscopy].

OBJECTIVE: The left superior vena cava (LSVC) is often complicated with congenital heart defect. Although we simply clamp LSVC during cardio-pulmonary bypass (CPB), appropriateness of this technique has not been clarified. We noninvasively evaluate cerebral tissue oxygenation while the clamping of LSVC under CPB by near-infrared spectroscopy (NIRS). METHODS: Six children (3 male and 3 female; aged 1.0 +/- 0.6 year) undergoing open heart surgery were studied. The NIRO 300 was incorporated into an established multimodal monitoring system. Tissue oxygenation index (TOI), oxyhemoglobin (O2Hb), and deoxyhemoglobin (HHb) changes were assessed and compared with LSVC pressure. RESULTS: There were no significant changes in cerebral oxygen delivery after LSVC clamp. LSVC pressure increased from 7.3 +/- 1.8 mmHg to 20.1 +/- 2.6 just after LSVC clamp, but gradually decreased without any maneuver. CONCLUSION: These data demonstrated that LSVC could be safely clamped when LSVC pressure was under 30 mmHg.

[Long-term outcome of aortic valve replacement with annular enlargement].

BACKGROUND: The choice of appropriate treatment in children with aortic valvular lesions remains controversial. The purpose of this study is to assess early and late outcomes of aortic valve replacement with annular enlargement in children. METHODS: A retrospective study was conducted in 16 consecutive patients aged 0.26 to 15.9 years operated on between 1993 and 2008. Thirteen children underwent aortic valve replacement with Konno procedure (mechanical valve: 12, homograft: 1), 2 children underwent Ross procedure, and the last child underwent Nicks procedure with mechanical valve replacement. All patients undergoing mechanical valve replacement were given warfarin with a monthly international normalized ratio (INR) control. RESULTS: Overall early mortality was 12.5% (2 cases). Emergency operation was performed in these cases because of infectious endocarditis and acute cardiac failure. However, cardiopulmonary bypass (CPB) weaning could not be obtained. There was 1 late death 5 months after mechanical valve replacement. The patient developed methicillin-resistant Staphylococcus aureus (MRSA) sepsis after cleft palate repair. Reoperation was needed in 1 case. CONCLUSION: We conclude that mechanical valve replacement with aortic annular enlargement is an acceptable treatment in children. It is associated with acceptable mortality and low incidence of late events, and provides long-term survival.

[Results of Norwood type operation for interrupted aortic arch complex; surgical decision making].

BACKGROUND: Interrupted aortic arch (IAA) is associated with a multitude of lesions ranging from isolated ventricular septal defect to hypoplastic left ventricle or severe subaortic stenosis (SAS). Left ventricular outflow obstruction such as SAS continues to be an important factor for deciding the surgical procedure between univentricular and biventricular repairs. METHODS: A retrospective study was conducted in 8 consecutive infants aged 14 to 117 days and operated on between 2004 and 2009. Seven patients had undergone bilateral pulmonary artery banding for pulmonary high flow regulation. All patients underwent Norwood-type operation (4 with systemic to pulmonary artery shunt, 3 with right ventricle to pulmonary artery shunt, and 1 with bidirectional Glenn shunt). RESULTS: One patient died 2 months after surgery due to respiratory failure. The others were discharged in a good condition. One patient underwent Rastelli-type operation and biventricular circulation was achieved. The other 6 patients were all Fontan candidates. CONCLUSION: Satisfactory initial palliation can be achieved by Norwood-type operation for IAA with severe SAS or hypoplastic left ventricular-aortic complex.