Atrioventricular valve surgery in patients with univentricular heart and two separate atrioventricular valves.

OBJECTIVES: Atrioventricular valve regurgitation in patients with univentricular heart is a well-known risk factor for adverse outcomes and atrioventricular valve repair remains a particular surgical challenge. METHODS: We reviewed all surgical atrioventricular valve procedures in patients with univentricular heart and two separate atrioventricular valves who underwent surgical palliation. Endpoints of the study were reoperation-free survival and cumulative incidence of reoperation. RESULTS: Between 1994 and 2021, 202 patients with univentricular heart and two separate atrioventricular valve morphology underwent surgical palliation, with 15.8% (32/202) requiring atrioventricular valve surgery. Primary diagnoses were double inlet left ventricle (n = 14, 43.8%), double outlet right ventricle (n = 7, 21.9%), and congenitally corrected transposition of the great arteries (n = 7, 21.9%). Median weight at valve surgery was 10.6 kg (interquartile range, 7.9-18.9). Isolated left or right atrioventricular valve surgery was required in nine (28.1%) and 22 patients (68.8%), respectively. Concomitant left and right atrioventricular valve surgery was performed in one patient (3.1%). Closure of the left valve was conducted in four patients (12.5%) and closure of the right valve in three (9.4%). Operative and late mortality were 3.1% and 9.7%, respectively. Reoperation-free survival and cumulative incidence of reoperation at 10 years after surgery were 62.3% (standard error of the mean: 6.9) and 30.9% (standard error of the mean: 9.6), respectively. CONCLUSIONS: In patients with univentricular heart and two separate atrioventricular valves, surgical intervention on these valves is required in a minority of patients and is associated with low mortality but high incidence of reoperation.

Impact of early postoperative haemodynamic and laboratory parameters on outcome after the Fontan procedure.

OBJECTIVE: To identify early postoperative haemodynamic and laboratory parameters predicting outcomes following total cavopulmonary connection. METHODS: Patients who underwent total cavopulmonary connection between 2012 and 2021 were evaluated. Serial values of mean pulmonary artery pressure, mean arterial pressure, peripheral oxygen saturation, and lactate levels were collected. The influence of these variables on morbidities was analyzed. Cut-off values were calculated using the receiver operating characteristic analysis. RESULTS: A total of 249 patients were included. All patients had previous bidirectional cavopulmonary shunt. Median age and weight at total cavopulmonary connection were 2.2 (1.8-2.7) years and 11.7 (10.7-13.4) kg, respectively. All patients were extubated in the ICU at a median of 3 (2-5) hours after ICU admission. Postoperative pulmonary artery pressure, around 12 hours after extubation, was significantly associated with chest tube drainage (p = 0.048), chylothorax (p = 0.021), ascites (p = 0.016), and adverse events (p = 0.028). Receiver operating characteristic analysis revealed a cut-off value of 13-15 mmHg for chest tube drainage and chylothorax and 17 mmHg for ascites and adverse events. Mean arterial pressure 1 hour after extubation was associated with prolonged chest tube drainage (p = 0.015) and adverse events (p = 0.008). Peripheral oxygen saturation 6 hours after extubation (p = 0.003) was associated with chest tube duration and peripheral oxygen saturation 1 hour after extubation (p < 0.001) was associated with ascites. Lactate levels on 2nd postoperative day (p = 0.022) were associated with ascites and lactate levels on 1st postoperative day (p = 0.009) were associated with adverse events. CONCLUSIONS: Higher pulmonary artery pressure, lower mean arterial pressure, lower peripheral oxygen saturation, and higher lactate in early postoperative period, around 12 hours after extubation, predicted in-hospital and post-discharge adverse events following total cavopulmonary connection.

Outcomes after bidirectional cavopulmonary shunt with antegrade pulmonary blood flow in high-risk patients.

OBJECTIVE: We have left antegrade pulmonary blood flow (APBF) at bidirectional cavopulmonary shunt (BCPS) only for high-risk patients. This study evaluates the indication and the outcomes of patients with APBF, compared to those without APBF. METHODS: Patients with APBF after BCPS were identified among patients who underwent BCPS between 1997 and 2022. Outcomes of patients with and without APBF after BCPS were compared. RESULTS: APBF was open in 38 (8.2%) of 461 patients. Median age (7.7 versus 6.3 months, p = 0.55) and weight (5.6 versus 6.1 kg, p = 0.75) at BCPS were similar in both groups. The most frequent indication for APBF was high pulmonary artery pressure (PAP) in 14 patients, followed by hypoxaemia in 10, and hypoplastic left pulmonary artery in 8. The source of APBF was the pulmonary trunk in 10 patients and the aortopulmonary shunt in 28. Median hospital stay after BCPS was longer (22 versus 14 days, p = 0.018) and hospital mortality was higher (10.5 versus 2.1%, p = 0.003) in patients with APBF compared to those without APBF. However, 448 hospital survivors showed similar survival after discharge following BCPS (p = 0.224). Survival after total cavopulmonary connection (TCPC) was similar between the groups (p = 0.753), although patients with APBF were older at TCPC compared to those without (3.9 versus 2.2 years, p = 0.010). CONCLUSION: APBF was left in 8% following BCPS in high-risk patients, mainly due to preoperative high PAP. Hospital survivors after BCPS demonstrated comparable survival in patients with and without APBF. Adding APBF at BCPS might be a useful option for high-risk patients.

Surgical outcome of the borderline hypoplastic left ventricle: impact of the left ventricle rehabilitation strategy.

OBJECTIVE: This study aims to assess the surgical outcome of borderline hypoplastic left ventricle before and after the induction of the left ventricle rehabilitation strategy. METHODS: A retrospective review investigated patients with borderline hypoplastic left ventricle who underwent surgical intervention between 2012 and 2022. The patient cohort was stratified into two groups based on the initiation of left ventricle rehabilitation: an early-era group (E group, 2012-2017) and a late-era group (L group, 2018-2022). Left ventricle rehabilitation was defined as palliation combined with other procedures aimed at promoting left ventricular growth such as restriction of atrial septal defect, relief of inflow/outflow obstructive lesions, and resection of endocardial fibroelastosis. RESULTS: A total of 58 patients were included. Primary diagnosis included 12 hypoplastic left heart syndromes, 11 critical aortic valve stenosis, and others. A total of 9 patients underwent left ventricle rehabilitation, 8 of whom underwent restriction of atrial septal defect. As for clinical outcomes, 9 of 23 patients achieved biventricular repair in the E group, whereas in the L group, 27 of 35 patients achieved biventricular repair (39% vs. 77%, p = 0.004). Mortality did not differ statistically between the two groups (log-rank test p = 0.182). As for the changes after left ventricle rehabilitation, left ventricular growth was observed in 8 of 9 patients. The left ventricular end-diastolic volume index (from 11.4 to 30.1 ml/m2, p = 0.017) and left ventricular apex-to-right ventricular apex ratio (from 86 to 106 %, p = 0.014) significantly increased after left ventricle rehabilitation. CONCLUSIONS: The introduction of the left ventricle rehabilitation strategy resulted in an increased proportion of patients achieving biventricular repair without a concomitant increase in mortality. Left ventricle rehabilitation was associated with enhanced left ventricular growth and the formation of a well-defined left ventricle apex. Our study underscores the significance of left ventricle rehabilitation strategies facilitating successful biventricular repair. The data suggest establishing restrictive atrial communication may be a key factor in promoting left ventricular growth.

Serum albumin levels as a predictive biomarker for low-load resistance training programs' effects on muscle thickness in the community-dwelling elderly Japanese population: interventional study result.

BACKGROUND: Resistance training has been recommended as an effective measure against age-related loss of muscle mass and muscle strength, called sarcopenia, even in older adults. However, despite subjecting each participant to the same training program, the training effect solely depended on the individual. This study aimed to evaluate whether certain blood parameters influenced the effect of a low-load resistance training program on muscle thickness in the community-dwelling elderly population. METHODS: Sixty-nine community-dwelling Japanese (49 women and 20 men) subjects aged 69.4 ± 6.5 years were included. Low-load resistance training was performed twice a week for 12 weeks. Muscle thickness at the anterior aspects of the thigh (AT) was measured using a B-mode ultrasound device, and 22 blood parameter levels were assessed before and after the program. We checked the first quartile value of each parameter to establish cutoff values, and participants were divided into low or normal groups for each parameter. RESULTS: A low-load resistance training program significantly increased muscle thickness at the AT. The interaction between time and groups was examined at low (< 4.1 g/dL) versus normal (≥ 4.1 g/dL) serum albumin (Alb) levels. Although there was no difference in muscle thickness at the AT before the training intervention, the hypertrophic effects were higher in the normal serum Alb level group than in the low serum Alb level group. The binomial logistic regression analysis showed that participants in the low serum Alb group had an odds ratio of 7.08 for decreased muscle thickness at the AT. The effect of a low-load resistance training program on lower limb muscle thickness appears to be limited in participants with low serum Alb levels before training interventions. CONCLUSIONS: Serum Alb level may act as a biomarker to predict the effects of low-load resistance training programs on muscle hypertrophy in elderly individuals. TRIAL REGISTRATION: This study was retrospectively registered in UMIN-Clinical Trial Registry (CTR), ID: UMIN000042759 (date of registration, 14 Dec 2020).

The 30-s chair stand test can be a useful tool for screening sarcopenia in elderly Japanese participants.

BACKGROUND: Low muscle strength has been focused on as an essential characteristic of sarcopenia, and the 30-s chair stand test (CS-30) could be a particularly useful test for assessing muscle strength. While it is speculated to be a beneficial tool for the assessment of sarcopenia, this remains to be verified. In this study, we examined the reliability and optimal diagnostic score of the CS-30 for assessing sarcopenia in elderly Japanese participants. METHODS: This cross-sectional study included 678 participants (443 females and 235 males) who underwent the test for sarcopenia as per the Asian Working Group for Sarcopenia (AWGS) 2019, the CS-30 test, and the isometric knee-extension muscle strength test. ROC analysis was used to estimate the optimal CS-30 scores at which sarcopenia was detected. RESULTS: CS-30 scores were positively associated with sarcopenia (OR: 0.88; 95% CI:0.82-0.93). The AUC of the CS-30 for sarcopenia definition were 0.84 (p < 0.001) for females and 0.80 (p < 0.001) for males. The optimal number of stands in the CS-30 that predicted sarcopenia was 15 for females (sensitivity, 76.4%; specificity, 76.8%) and 17 for males (sensitivity, 75.0%; specificity, 71.7%). CONCLUSIONS: The CS-30 was found to be a reliable test for sarcopenia screening in the elderly Japanese population.

Site-Specific Muscle Loss in the Abdomen and Anterior Thigh in Elderly Males with Locomotive Syndrome.

Although locomotive syndrome (LS) is a condition of reduced mobility, little information is available regarding the loss of site-specific skeletal muscle mass. The aim of the present study is to examine site-specific muscle loss in elderly males with LS. A total of 100 men ranging in age from 65 to 74 years were divided into two groups (LS and non-LS) using LS risk tests including the stand-up test, two-step test, and the 25-question geriatric locomotive function scale Muscle thickness (MTH) at eight sites-anterior and posterior thigh (AT and PT, respectively), anterior and posterior lower leg (AL and PL, respectively), rectus abdominis (RA), anterior and posterior upper arm (AU and PU, respectively), and anterior forearm (AF)-was evaluated using B-mode ultrasound. Furthermore, the 30-s chair stand test (CS-30), 10-m walking time, zig-zag walking time, and sit-up test were assessed as physical functions. There were no significant differences in age and body mass index between the LS and non-LS groups. The percentage of skeletal muscle was lower in the LS group than in the non-LS group. Although there were no differences in the MTH of AU, PU, AF, PT, Al and PL, site-specific muscle loss was observed at RA and AT in the LS group. CS-30, 10-m walking time, zig-zag walking time, and sit-up test in the LS group were all worse than those in the non-LS group. The MTHs of RA and AT were both correlated to those physical functions. In conclusion, the LS group had site-specific muscle loss and worse physical functions. This study suggests that site-specific changes may be associated with age-related physical functions. These results may suggest what the essential characteristics of LS are.

Muscle Size and Strength of the Lower Body in Supervised and in Combined Supervised and Unsupervised Low-Load Resistance Training.

This study aimed to clarify whether low-load resistance training at a low frequency (twice a week) using body weight and elastic band improves muscle size, muscle strength, and physical functions and to compare the training effects between supervised training and a combination of supervised and unsupervised training in untrained older adults. Fifty-one older adults (ages: 57-75 years) selected to either a supervised (S) training group (n = 34) or a combined supervised and unsupervised (SU) group (n = 17). Both groups performed low-load resistance training composed of nine exercises for 12 weeks. The S group participated in supervised exercise sessions twice a week, and the SU group performed a supervised exercise session once a week and an unsupervised exercise session at home also once a week. For muscle thicknesses in the anterior aspects of the forearm, upper arm, and thigh and the posterior aspect of the thigh, group × time interactions were observed (p < 0.05). The hypertrophic effects were higher in the S group. Isometric knee extension strength and physical functions increased similarly in both groups. Low-load resistance training using body weight and elastic band twice a week for 12 weeks induces muscle hypertrophy and increases muscle strength and physical functions in older adults. Although the muscle hypertrophic effects are greater in the S group than in the SU group, the other effects were similar between the groups.

[Mid-aortic Syndrome Requiring Surgical Intervention during Infancy;Report of a Case].

Mid-aortic syndrome (MAS) is a very rare disease characterized by stenosis from the distal of the thoracic aorta to the abdominal aorta, in many case it is found as a result of hypertension and the like, and it needs surgical intervention in early childhood to adolescence. Here, we report a case of MAS which recognized prominent left ventricular myocardial hypertrophy from the early stage and needed surgical intervention in the infancy. We selected patch angioplasty using expanded polytetrafluoro ethylene( ePTFE) graft, and after surgery pressure gradient was disappeared.

[Midterm Outcomes and Therapeutic Effects of Total Cavopulmonary Connection (TCPC) Conversion].

Total cavopulmonary connection (TCPC) conversion prevents late complications after the atrio-pulmonary-Fontan procedure. However, the outcomes and effects of TCPC conversion are not fully known. We performed TCPC conversion in 31 patients (2004~2017). Concomitantly, we performed anti-arrhythmia surgery in 28 patients( 90%), atrioventricular valve surgery in 4, and pacemaker implantation in 2. There were no perioperative deaths, but one late death occurred due to protein-losing gastroenteropathy. Five-year survival was 96.8%. Eleven patients were readmitted for various reasons, including arrhythmia in 7 and heart failure in 1. The 5-year cardiac event-free rate was 67.7%. The cardiac index was significantly improved:1.58 l/min/m2 before to 3.57 l/min/m2 after surgery ( p=0.0075). Surgical and midterm outcomes of TCPC conversion were favorable. In the perioperative and late periods, therapeutic intervention was usually for atrial arrhythmia. This study demonstrated that TCPC conversion is an effective therapeutic procedure.

Effects of a progressive walking program on the risk of developing locomotive syndrome in elderly Japanese people: a single-arm trial.

[Purpose] This study aimed to identify the efficacy of a progressive walking program on the risk of developing locomotive syndrome among untrained elderly Japanese people. [Participants and Methods] Twenty-four untrained elderly individuals (68 ± 4 years) completed a 17-week progressive walking program. The stand-up, two-step tests and the 25-question geriatric locomotive function scale were used to assess the risk of locomotive syndrome at baseline, the 8-week midpoint (2 months), and the 17-week endpoint (4 months). Maximal isometric muscle strength of the knee extensors and flexors were measured using a dynamometer with the hip joint angle at 90° of flexion and physical function (the 30-s sit-to-stand, sit-up, 10-meter walk, and grip strength) were evaluated. [Results] The 4-month walking program significantly improved the two-step test and geriatric locomotive function scale scores. This may be attributable to the improvement in knee flexor strength and physical function. [Conclusion] A 4-month program of progressive walking effectively lowered the risk of developing locomotive syndrome in elderly Japanese people by improving knee flexor muscle strength and physical function.

Hypoxia affects tissue oxygenation differently in the thigh and calf muscles during incremental running.

PURPOSE: The present study was performed to determine the impact of hypoxia on working muscle oxygenation during incremental running, and to compare tissue oxygenation between the thigh and calf muscles. METHODS: Nine distance runners and triathletes performed incremental running tests to exhaustion under normoxic and hypoxic conditions (fraction of inspired oxygen = 0.15). Peak pulmonary oxygen uptake ([Formula: see text]) and tissue oxygen saturation (StO2) were measured simultaneously in both the vastus lateralis and medial gastrocnemius. RESULTS: Hypoxia significantly decreased peak running speed and [Formula: see text] (p < 0.01). During incremental running, StO2 in the vastus lateralis decreased almost linearly, and the rate of decrease from warm-up (180 m min-1) to [Formula: see text] was significantly greater than in the medial gastrocnemius under both normoxic and hypoxic conditions (p < 0.01). StO2 in both muscles was significantly decreased under hypoxic compared with normoxic conditions at all running speeds (p < 0.01). The rate at which StO2 was decreased by hypoxia was greater in the vastus lateralis as the running speed increased, whereas it changed little in the medial gastrocnemius. CONCLUSIONS: These results suggest that the thigh is more deoxygenated than the calf under hypoxic conditions, and that the effects of hypoxia on tissue oxygenation differ between these two muscles during incremental running.

Tissue Blood Volume Parameters Measured by Continuous-Wave and Spatially Resolved NIRS Show Different Changes During Prolonged Cycling Exercise.

Near-infrared spectroscopy (NIRS) shows two types of tissue blood volume (BV) parameters: the total hemoglobin concentration (cHb; assessed by continuous-wave NIRS) and the normalized tissue hemoglobin index (nTHI; evaluated by spatially resolved NIRS). This study was performed to investigate the differences between cHb and nTHI, estimated by calibration using acute reduction of BV at the onset of exercise. Seven active male volunteers (age: 22  ±  4 years) performed a prolonged (60-min) cycling exercise test at 65% or 70% of the peak oxygen uptake. During the tests, cHb and nTHI from the vastus lateralis muscle were monitored. The two parameters were expressed relative to the resting value of 100% and the minimum value of 0% at the onset of exercise. The value of cHb started to increase after arriving at the minimum value and continued until the end of exercise. Similar to cHb, nTHI increased acutely from the lowest level, but the parameter remained almost unchanged from ~5 min throughout the test. Therefore, the two parameters interacted significantly. Moreover, in comparison with the same exercise duration, cHb was significantly higher than nTHI from the 28th min to the end of the test. These results suggest that cHb and nTHI in working muscle are not synchronized during prolonged exercise, especially after ~30 min. The differences between the two BV parameters are likely partly attributable to other factors, such as the increase in skin blood flow.

Delayed Onset of Reoxygenation in Inactive Muscles After High-Intensity Exercise.

Oxygenation, measured by near-infrared spectroscopy (NIRS), in inactive muscles decreases during high-intensity exercise because of the decrease of oxygen supply. However, there have been few reports regarding recovery of inactive muscle oxygenation after exercise. This study was performed to examine reoxygenation in the biceps brachii muscle (BB) after supramaximal cycling exercise. Six active young male volunteers (age: 22  ± â€…3 years, peak oxygen uptake (VO2peak): 53.8  ± â€…5.4 mL/kg/min, mean  ± â€…S.D.) performed warm-up exercise, followed by cycling exercise at 140% of VO2peak for 30 s and then at 0 watt for 4 min (recovery exercise). Tissue oxygen saturation (StO2) in the vastus lateralis muscle (VL) and BB was monitored by spatially resolved NIRS throughout the test. The decrease rate of StO2 during exercise was 24.7  ± â€…7.5 p.p. in VL and 15.1  ± â€…8.2 p.p. in BB (N.S.). StO2 in VL increased immediately after the end of exercise, but StO2 in BB decreased continuously for 12.7  ± â€…7.8 s after exercise. Moreover, the time of half-recovery from the minimum to peak values after exercise was significantly (P â€…< â€…0.05) longer at StO2 in BB (39.5  ± â€…12.2 s) than VL (25.2  ± â€…6.0 s). These results suggest that the recovery of microvascular oxygen supply and blood flow in inactive muscles does not start immediately after exercise.

Do Two Tissue Blood Volume Parameters Measured by Different Near-Infrared Spectroscopy Methods Show the Same Dynamics During Incremental Running?

UNLABELLED: Both the change in total hemoglobin concentration (cHb), assessed by near-infrared continuous-wave spectroscopy (NIR-CWS), and the normalized tissue hemoglobin index (nTHI), assessed by NIR spatially resolved spectroscopy (NIR-SRS), were used to quantify changes in tissue blood volume. However, it is possible that these parameters may show different changes because of the different measurement systems. The present study aimed to compare changes in cHb and nTHI in working muscles, which were selected for measurement because the parameters changed dynamically. METHODS: After a standing rest, seven male runners (age 24±3 years, mean±S.D.) performed an incremental running exercise test on a treadmill (inclination=1%) from 180 to 300 m min(-1). During the tests, cHb and nTHI were monitored from the vastus lateralis (VL) and medial gastrocnemius (GM) muscles. These parameters were relatively evaluated from the minimal to maximal values through the test. RESULTS: When the exercise began, cHb and nTHI quickly decreased and then gradually increased during running. In comparison with both VL and GM, there was significant interaction between cHb and nTHI. CONCLUSIONS: The present results suggest that cHb and nTHI in working muscles are not always synchronized, particularly at the onset of exercise and at high intensities. Although cHb was previously used as the change of tissue blood volume, it is implied that tissue blood volume assessed by cHb is overestimated.

¹³C MRS reveals a small diurnal variation in the glycogen content of human thigh muscle.

There is marked diurnal variation in the glycogen content of skeletal muscles of animals, but few studies have addressed such variations in human muscles. (13)C MRS can be used to noninvasively measure the glycogen content of human skeletal muscle, but no study has explored the diurnal variations in this parameter. This study aimed to investigate whether a diurnal variation in glycogen content occurs in human muscles and, if so, to what extent it can be identified using (13)C MRS. Six male volunteers were instructed to maintain their normal diet and not to perform strenuous exercise for at least 3 days before and during the experiment. Muscle glycogen and blood glucose concentrations were measured six times in 24 h under normal conditions in these subjects. The glycogen content in the thigh muscle was determined noninvasively by natural abundance (13)C MRS using a clinical MR system at 3 T. Nutritional analysis revealed that the subjects' mean carbohydrate intake was 463 ± 137 g, being approximately 6.8 ± 2.4 g/kg body weight. The average sleeping time was 5.9 ± 1.0 h. The glycogen content in the thigh muscle at the starting point was 64.8 ± 20.6 mM. Although absolute and relative individual variations in muscle glycogen content were 7.0 ± 2.1 mM and 11.3 ± 4.6%, respectively, no significant difference in glycogen content was observed among the different time points. This study demonstrates that normal food intake (not fat and/or carbohydrate rich), sleep and other daily activities have a negligible influence on thigh muscle glycogen content, and that the diurnal variation of the glycogen content in human muscles is markedly smaller than that in animal muscles. Moreover, the present results also support the reproducibility and availability of (13)C MRS for the evaluation of the glycogen content in human muscles.

Molecular design of Cy3 derivative for highly sensitive in-stem molecular beacon and its application to the wash-free FISH.

We herein describe a novel in-stem molecular beacon (ISMB) containing multiple Cy3-quencher pairs on d-threoninol scaffolds in the stem region. The designed Cy3 derivative was not significantly quenched by the adjacent nucleobases, self-quenching of the fluorophore was minimal, and the fluorophore did not severely destabilize the duplex. Using newly designed Cy3, we synthesized ISMBs containing two Cy3 moieties. The signal to background ratio of the ISMB containing two Cy3 moieties was above 100, whereas that with one Cy3 was 30. A Cy3-derivative containing ISMB used in a fluorescence in situ hybridization (FISH) detected endogenous ß-actin mRNA in fixed cells without need for washing procedures.

Impact of low birth weight on staged single-ventricle palliation.

BACKGROUND: To assess the impact of low birth weight on early and late outcomes after staged palliation for single ventricle. METHODS: Patients after stage 1 palliation for single ventricle in our institution were retrospectively included and divided into two weight groups: 2.5 kg or less (low birth weight) and more than 2.5 kg. The impact of low birth weight on mortality and on the progression to further palliation stages (bidirectional Glenn, stage 2, and total cavopulmonary connection, stage 3) was assessed. RESULTS: A total of 452 patients were included. Patients with low birth weight (n = 37, 8 %) had more frequently associated prematurity and extracardiac anomalies. Early and inter-stage mortality after stage 1 was higher in patients with low birth weight, so that less of these patients reached the next palliation stage (57 % vs. 77 %, p = 0.01, and 38 % vs. 56 %, p = 0.05, for stage 2 and stage 3, respectively). After 5 years, overall survival was inferior in patients with low birth weight (48 % vs. 73 %, p < 0.001). Survival conditioned by stage 2 palliation was inferior in patients with low birth weight compared to the reference group (76 % vs. 89 % after 5 years, p = 0.04). Low birth weight was a risk factor for death in most patients' subgroups, inclusive those with restricted pulmonary blood flow after a systemic-to-pulmonary shunt procedure. CONCLUSIONS: During staged palliation of single-ventricle physiology, low birth weight has a detrimental impact on survival extending to beyond stage 2. This study calls for increased vigilance of these patients beyond the first interstage.

Comparison of pulmonary artery growth between ductus stent and systemic-to-pulmonary shunt as single-ventricle palliation.

OBJECTIVE: We aimed to compare the pulmonary artery (PA) growth between infants with univentricular hearts who underwent a ductus stenting (DS) and those who received a systemic to pulmonary shunt (SPS) as an initial palliation. METHODS: All infants with ductal-dependent pulmonary blood flow who underwent initial palliation with either a DS or SPS between 2009 and 2022 in our institution were reviewed. PA development was compared between the groups using the PA index and the symmetry index. RESULTS: A total of 130 patients were evaluated including 49 patients after DS and 81 after SPS. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19. At stage II palliation, PA index (p=0.926), right PA index (p=0.692), left PA index (p=0.297) and the symmetry index (p=0.650) were similar between the groups. At stage III Fontan completion, PA index (p=0.115), right PA index (p=0.868), and the symmetry index (p=0.144) were similar between the groups. However, left PA index (60 vs. 74mm2/m2, p=0.015) was lower, and the incidence of veno-venous collaterals (24.2 vs. 7.8%, p=0.036) was higher in DS group compared to SPS group. Freedom from PA interventions between stage II and III palliation was lower in DS group compared to SPS group (p=0.009). CONCLUSIONS: In patients after DS in infants with univentricular heart, freedom from PA interventions after stage II was lower and the left PA index was smaller, compared to those after SPS. Long-term outcomes after the Fontan procedure should be addressed in patients after DS.

Impact of aorto-pulmonary and veno-venous collaterals on the onset of plastic bronchitis following the Fontan procedure.

OBJECTIVE: This study aimed to clarify the association of the aorto-pulmonary/veno-venous collaterals in patients with plastic bronchitis after the Fontan procedure. METHODS: All patients who underwent total cavopulmonary connection from 1994 to 2022 were reviewed. Aorto-pulmonary/veno-venous collaterals were detected using angiograms. The impact of aorto-pulmonary/veno-venous collaterals as well as other variables on the onset of plastic bronchitis were evaluated. RESULTS: A total of 635 patients were included. Plastic bronchitis was observed in 15 (2.4%) patients and the median duration between total cavopulmonary connection and the onset of plastic bronchitis was 1.1 years. Freedom from plastic bronchitis at 5 and 10 years was 97.5 and 96.5%, respectively. As for the association of PB and APCs/VVCs, patients with PB had an increased incidence of post-TCPC APCs (60 vs. 14%, p<0.001) and VVCs (53 vs. 14%, p<0.001). The freedom from plastic bronchitis was the lowest in the patients who were associated with both aortopulmonary collaterals and veno-venous collaterals after total cavopulmonary connection. Aortopulmonary collaterals after total cavopulmonary connection (p=0.002, hazard ratio: 5.729), veno-venous collaterals after total cavopulmonary connection (p=0.016, hazard ratio: 3.527), and chylothorax (p=0.019, hazard ratio: 3.662) were identified as independently associated factors with the onset of plastic bronchitis. CONCLUSIONS: The occurrence of post-Fontan aortopulmonary and veno-venous collaterals was significantly associated with the development of plastic bronchitis. Treatment or mitigation of collaterals may represent an opportunity to prevent the development of plastic bronchitis, a remaining source of post-Fontan excess morbidity and mortality.