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.

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.

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.

Longitudinal analysis of systemic ventricular function and atrioventricular valve function after the Norwood procedure.

OBJECTIVES: To evaluate longitudinal systemic ventricular function and atrioventricular valve regurgitation in patients after the neonatal Norwood procedure. METHODS: Serial postoperative echocardiographic images before Fontan completion were assessed in neonates who underwent the Norwood procedure between 2001 and 2020. Ventricular function and atrioventricular valve regurgitation were compared between patients with modified Blalock-Taussig shunt and right ventricle to pulmonary artery conduit. RESULTS: A total of 335 patients were identified including 273 hypoplastic left heart syndrome and 62 of its variants. Median age at Norwood was 8 (7-12) days. Modified Blalock-Taussig shunt was performed in 171 patients and the right ventricle to pulmonary artery conduit in 164 patients. Longitudinal ventricular function and atrioventricular valve regurgitation were evaluated using a total of 4352 echocardiograms. After the Norwood procedure, ventricular function was initially worse (1-30 days) but thereafter better (30 days to stage II) in the right ventricle to pulmonary artery conduit group (P < 0.001). After stage II, the ventricular function was inferior in the right ventricle to the pulmonary artery conduit group (P < 0.001). Atrioventricular valve regurgitation between the Norwood procedure and stage II was more frequent in the modified Blalock-Taussig shunt group (P < 0.001). After stage II, there was no significant difference in atrioventricular valve regurgitation between the groups (P = 0.171). CONCLUSIONS: The effect of shunt type on haemodynamics after the Norwood procedure seems to vary according to the stage of palliation. After the Norwood, the modified Blalock-Taussig shunt is associated with poorer ventricular function and worse atrioventricular valve regurgitation compared to right ventricle to pulmonary artery conduit. Whereas, after stage II, modified Blalock-Taussig shunt is associated with better ventricular function and comparable atrioventricular valve regurgitation, compared to the right ventricle to pulmonary artery conduit.

Comparison of ductus stent versus surgical systemic-to-pulmonary shunt as initial palliation in patients with univentricular heart.

OBJECTIVES: In this study, we aimed to compare infants with univentricular hearts who underwent an initial ductus stenting to those receiving a surgical systemic-to-pulmonary shunt (SPS). METHODS: All infants with univentricular heart and ductal-dependent pulmonary blood flow who underwent initial palliation with either a ductus stenting or a surgical SPS between 2009 and 2022 were reviewed. Outcomes were compared after ductus stenting or SPS including survival, probability of re-interventions and the probability to reach stage II palliations. RESULTS: A total of 130 patients were evaluated, including 49 ductus stenting and 81 SPSs. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19 patients. There was comparable hospital mortality (2.0% stent vs 3.7% surgery, P = 0.91) between the groups, but shorter intensive care unit stay (median 1 vs 7 days, P < 0.01) and shorter hospital stay (median 7 vs 17 days, P < 0.01) were observed in patients with initial ductus stenting, compared to those with SPS. However, acute procedure-related complications were more frequently observed in patients with ductus stenting, compared with those with SPS (20.4 vs 6.2%, P = 0.01), and 10 patients needed a shunt procedure after the initial ductus stent. The cumulative incidence of reaching stage II was similar between ductus stenting and SPS (88.0 vs 90.6% at 12 months, P = 0.735). Pulmonary artery (PA) index (median 194 vs 219 mm2/m2, P = 0.93) at stage II was similar between patients with ductus stenting and SPS. However, the ratio of the left to the right PA index [0.69 (0.45-0.95) vs 0.86 (0.51-0.84), P = 0.015] was higher in patients who reached stage II with surgical shunt physiology, compared with patients with ductus stent physiology. CONCLUSIONS: After initial ductus stenting in infants with univentricular heart, survival is comparable and post-procedural recovery shorter, but more acute stent dysfunctions and lower development of left PA are observed, compared to acute shunt dysfunctions. The less invasive procedure and shorter hospital stay are at the expense of more stent reinterventions.

Impact of aortopulmonary collaterals on adverse events after total cavopulmonary connection.

OBJECTIVES: Effects of aortopulmonary collaterals (APCs) on outcomes after the total cavopulmonary connection (TCPC) are unclear. This study evaluated the incidence of APCs before and after TCPC and analysed the impacts of APCs on adverse outcomes. METHODS: A total of 585 patients, who underwent TCPC from 1994 to 2020 and whose preoperative angiographies were available, were included. Pre-TCPC angiograms in all patients were used for the detection of APCs, and post-TCPC angiograms were evaluated in selected patients. Late adverse events included late death, protein-losing enteropathy (PLE) and plastic bronchitis (PB). RESULTS: The median age at TCPC was 2.3 (1.8-3.4) years with a body weight of 12 (11-14) kg. APCs were found in 210 patients (36%) before TCPC and in 81 (14%) after TCPC. The closure of APCs was performed in 59 patients (10%) before TCPC, in 25 (4.2%) at TCPC and in 59 (10%) after TCPC. The occurrences of APCs before and after TCPC were not associated with short-term or mid-term mortality. The APCs before TCPC were associated with chylothorax (P = 0.025), prolonged chest tube duration (P = 0.021) and PB (P = 0.008). The APCs after TCPC were associated with PLE (P < 0.001) and PB (P < 0.001). With APCs following TCPC, freedom from PLE and PB was lower than without (P < 0.001, P < 0.001). CONCLUSIONS: APCs before TCPC were associated with chylothorax, prolonged chest tube duration and PB. APCs after TCPC were associated with both PLE and PB. The presence of APCs might affect the lymph drainage system and increase the incidence of chylothorax, PLE and PB.

An adult case of giant congenital left atrial wall aneurysm.

Congenital left atrial wall aneurysm is a rare disorder that occurs in a wide range of age groups from infancy to adulthood. Here, we present a case of a congenital left atrial wall aneurysm that was detected in a 19-year-old man who was surgically treated. Although the patient was asymptomatic without any pre-existing conditions, chest radiography performed as part of a routine health examination detected abnormalities in the heart. Contrast-enhanced computed tomography revealed a giant aneurysm measuring 72 mm × 56 mm that extended from the posteroinferior wall of the left atrium to the posterior surface of the left ventricle. Transthoracic echocardiography revealed mild mitral regurgitation. The patient was diagnosed as having a congenital left atrial wall aneurysm associated with mild mitral regurgitation. The aneurysm was resected through median sternotomy under cardiopulmonary bypass with cardioplegic arrest. During surgery, no structural abnormalities were noted in the mitral valve. After surgery, the patient was discharged without complications. Neither recurrence of the aneurysm nor exacerbation of mitral regurgitation was observed at 1 year postoperatively. Learning objective: A congenital left atrial wall aneurysm is a rare disorder. Rupture of the aneurysm is rare. However, when they are left untreated, there are concerns regarding arrhythmias, heart failure, and systemic embolism. Thus, surgical treatment is recommended. Aneurysms are resected under cardiopulmonary bypass. In cases of aneurysms complicated by moderate or severer mitral regurgitation, mitral valve repair is necessary. The prognosis following surgical treatment is favorable.

Surgical Management of Group A ß-Hemolytic Streptococcal Aortic Root Abscess.

This case report presents a pediatric patient with an aortic root abscess caused by group A ß-hemolytic Streptococcus. After aggressive debridement of infected tissue, the continuity between the left ventricular outflow tract and aorta was reconstructed with autologous pericardium, and the aortic valve was replaced with a mechanical prosthesis using the Konno procedure with concomitant mitral valve plasty. We believe that radical resection of the abscess is an effective way to eradicate such a life-threatening infection.

Acute Limb Ischemia in a COVID-19 Patient: A Case Report.

A 63-year-old man with a confirmed case of coronavirus disease 2019 and having complaints of severe pain and paralysis in his right lower limb was transported to our hospital in an ambulance. Because of thrombosis, a computed tomography angiogram revealed the occlusion of right common iliac artery and stenosis of abdominal aorta. Emergency angiography and thrombectomy were performed; after surgery, the patient was managed in the intensive care unit with mechanical ventilation and hemodialysis for renal failure. However, on postoperative day 7, thrombosis recurred, and he died because of multiple organ failure.

[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.

[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.

Effect of Thoracic Gas Volume Changes on Body Composition Assessed by Air Displacement Plethysmography after Rapid Weight Loss and Regain in Elite Collegiate Wrestlers.

We investigated the effect of rapid weight loss (RWL) and weight regain (WR) on thoracic gas volume (VTG) and body composition assessment using air displacement plethysmography (ADP) in male wrestlers. Eight male elite collegiate wrestlers completed a RWL regimen (6% of body mass) over a 53-h period, which was followed by a 13-h WR period. ADP was used at three time points (baseline (T1), post-RWL (T2) and post-WR (T3)) according to the manufacturer's testing recommendations. The total body water and bone mineral content were estimated using the stable isotope dilution method and dual energy X-ray absorptiometry, respectively, at the same time points. Body composition was assessed with two-component (2C) or four-component (4C) models using either the measured VTG (mVTG) or predicted VTG (pVTG). Measured VTG increased from T1 to T2 (0.36 ± 0.31 L, p < 0.05) and decreased from T2 to T3 (-0.29 ± 0.15 L, p < 0.01). However, the changes in fat mass and fat free mass, which were calculated by both 2C and 4C models, were not significantly different when compared between calculations using mVTG and those using pVTG. Our results indicate that VTG significantly changes during RWL and WR, but both measured and predicted VTG can be used to assess changes in body composition during RWL and WR.

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.

Expectations and limitations after bilateral pulmonary artery banding.

OBJECTIVES: Bilateral pulmonary artery banding (bil-PAB) has been developed as a part of a hybrid procedure for hypoplastic left heart syndrome (HLHS). We use this procedure for palliation of complex congenital heart disease, particularly in patients with arch anomaly. We reviewed our experience with bil-PAB. METHODS: Sixty-six consecutive cases between 2003 and 2014 were reviewed retrospectively. RESULTS: Fifty-one patients (77%) had single ventricle physiology (HLHS: 37, HLHS variant: 13, others: 1). Twelve patients had biventricular physiology [5 interrupted aortic arch with ventricular septal defect (VSD); 4 coarctation of the aorta with VSD; 2 truncus arteriosus; 1 other]. In 3 patients, it was difficult to determine if the physiology was single ventricle or biventricle due to borderline left ventricle size. Age and body weight at the time of operation were 4.3 ± 3.4 days and 2.7 ± 0.5 kg, respectively. Overall hospital mortality was 11% (7/66). Of the 42 patients from 2010, only 1 with multiple anomalies died between bil-PAB and the second-stage operation. Thirteen patients (20%) required reoperation of bil-PAB. The mean waiting time for the next operation was 44.3 ± 42.8 days. The body weight at the second-stage operation was 3.2 ± 0.8 kg. Seven (11%) patients developed patent ductus arteriosus obstruction during the waiting period. Twenty-four patients were 2.5 kg or less; 20 patients in this group progressed to the second-stage operation and of these 19 gained body weight. Forty-two patients were above 2.5 kg, but of these only 23 patients gained body weight. Ten patients underwent bil-PAB because of shock and 8 of these recovered from shock. In 2 borderline cases, the patients underwent the Norwood procedure and 1 patient had arch repair and VSD closure. CONCLUSIONS: Bil-PAB could be applied to a wide variety of complex diseases. Our mortality rates with bil-PAB improved significantly post-2010. Good indications for bil-PAB were shock and situations where it was unclear whether the physiology was single ventricle or biventricular. Body weight gain was difficult to predict, but patients weighing less than 2.5 kg could be expected to gain body weight after the procedure.

Bulge-like asymmetric heterodye clustering in DNA duplex results in efficient quenching of background emission based on the maximized excitonic interaction.

Asymmetric dye clusters with a single fluorophore (Cy3) and multiple quenchers (4'-methylthioazobenzene-4-carboxylate, methyl red, and 4'-dimethylamino-2-nitroazobenzene-4-carboxylate) were prepared. The dye and one-to-five quenchers were tethered through D-threoninol to opposite strands of a DNA duplex. NMR analysis revealed that the clusters with a single fluorophore and two quenchers formed a sandwich-like structure (antiparallel H-aggregates). The melting temperatures of all the heteroclusters were almost the same, although structural distortion should become larger, as the number of quenchers increased. An asymmetric heterocluster of a single fluorophore and two quenchers showed larger excitonic interaction (i.e., hypochromicity of Cy3), than did a single Cy3 and a single quencher. Due to the larger exciton coupling between the dyes, the 1:2 heterocluster suppressed the background emission more efficiently than the 1:1 cluster. However, more quenchers did not enhance quenching efficiency due to the saturation of exciton coupling with two quenchers. Finally, this asymmetric 1:2 heterocluster was introduced into the stem region of a molecular beacon (MB; also known as an in-stem MB) targeting the fusion site in the L6 BCR-ABL fusion gene. With this MB design, the signal/background ratio was as high as 68 due to efficient suppression of background emission resulting from the maximized excitonic interaction.