[Left Main Trunk Orifice Reconstruction Using Aortic flap and Autologous Pulmonary Arterial Patch for Severe Left Main Trunk Stenosis After Arterial Switch Operation].

A 6-year-old boy with left main trunk (LMT) stenosis, who had undergone arterial switch operation (ASO) for transposition of the great arteries( TGA) before 6 years, underwent LMT orifice reconstruction. Coronary angiography showed severe stenosis of LMT, already when he was hospitalized with heart failure after 3 months of ASO. He was stable with oral treatment, therefore we performed the LMT reconstruction, before starting school, in terms of the risk of coronary ischemic event. We reconstructed the LMT ostium using an aortic flap and autologous pulmonary arterial patch. The postoperative computed tomography showed neither stenosis nor kinking at the repair site of LMT. He remains asymptomatic for over 6 months. We have reported that this method is effective to repair coronary artery anomalies (CAAs), since using an aortic flap. We could form a coronary artery floor, which is affixed to the aortic wall. This method allows us to change the coronary orifice position and the angle, so it is very useful method not for only CAAs, but also for coronary stenosis after ASO.

[Midterm Outcomes of Pulmonary Valve Leaflet Augmentation using Fresh Autologous Pericardium for Transannular Patch Repair of Tetralogy of Fallot].

BACKGROUND: Although valve sparing (VS) for patients with smaller pulmonary valves has been increasing, transannular patch repair( TAP) accounts for more than half of the total tetralogy of Fallot corrections worldwide. We use fresh autologous pericardial patches to perform a modified TAP procedure with pulmonary valve leaflet augmentation as proposed by Sung et al. We aimed to explore the early and midterm outcomes of this procedure. METHODS: We retrospectively reviewed 37 patients( group TAP:12;group VS:25) who underwent total tetralogy of Fallot corrections from April 2018 to December 2021. RESULTS: No midterm mortality was observed at a median observation period of 20.4 months in both groups. The midterm rates of freedom from moderate or more pulmonary regurgitation( PR) were 64.2 % and 21.4% in group TAP and 100% and 100% in group VS at 1 and 3 years, respectively( p<0.001). The midterm rates of freedom from pulmonary stenosis reintervention were 100% and 100% in group TAP and 96% and 96% in group VS at 1 and 3 years, respectively( p=0.51). CONCLUSIONS: TAP showed acceptable midterm survival and reintervention rate. Longer follow-up is essential considering the significantly higher PR in the postoperative period in group TAP.

Apex rotation as a risk factor for total anomalous pulmonary connection repair in single ventricle.

BACKGROUND: The high incidence of postoperative pulmonary venous obstruction (PVO) is a major mortality-associated concern in patients with right atrial isomerism and extracardiac total anomalous pulmonary venous connection (TAPVC). We evaluated new anatomical risk factors for reducing the space behind the heart after TAPVC repair. METHODS: Eighteen patients who underwent TAPVC repair between 2014 and 2020 were enrolled. Sutureless technique was used in 12 patients and conventional repair in six patients. The angle between the line perpendicular to the vertebral body and that from the vertebral body to the apex was defined as the "vertebral-apex angle (V-A angle)." The ratio of postoperative and preoperative angles, indicating the apex's lateral rotation, was compared between patients with and without PVO. RESULTS: The median (interquartile range) age and body weight at repair were 102 (79-176) days and 3.8 (2.6-4.8) kg, respectively. The 1-year survival rate was 83% (median follow-up, 29 [11-36] months). PVO occurred in seven patients (39%), who showed an obstruction of one or two branches in the apex side. The postoperative V-A angle (46° [45°-50°] vs. 36° [29°-38°], p = 0.001) and the ratio of postoperative and preoperative V-A angles (1.27 [1.24-1.42] vs. 1.03 [0.98-1.07], p = 0.001) were significantly higher in the PVO group than in the non-PVO group. The cut-off values of the postoperative V-A angle and ratio were 41° and 1.17, respectively. CONCLUSION: A postoperative rotation of the heart apex into the ipsilateral thorax was a risk factor for branch PVO after TAPVC repair.

[Modified Carpentier Technique is Useful Method for Tricuspid Regurgitation in Hypoplastic Left Heart Syndrome].

Management of tricuspid regurgitation( TR) before right ventricular dysfunction is critical in patients with hypoplastic left heart syndrome (HLHS);however, appropriate tricuspid valvuloplasty (TVP) is challenging. We report a TVP technique for TR in a 4-year-old girl with HLHS, who had undergone Norwood operation, bidirectional cavopulmonary shunt, and TVP. Preoperative echocardiography revealed the etiology of TR as anterior leaflet prolapse, annulus dilatation, and relative tethering of the septal leaflet. We performed surgical reconstruction of the anterior leaflet with artificial chordae. Before annuloplasty, the posterior leaflet and a part of the septal leaflet were detached from the annulus with a 1 mm margin using the Key-Reed technique. Furthermore, the posterior leaflet was slid to augment the septal leaflet. We managed to regulate the TR by enlarging the septal leaflet, thus increasing the coaptation zone. We believe that this technique will be useful for TR with annulus dilatation in HLHS.

[Atrial Septal Hematoma after Repair of Ventricular Septal Defect;Report of a Case].

A 3-month-old female with ventricular septal defect underwent definitive repair. In the operation, a venting tube was inserted through the incised patent foramen ovale. The ventricular septal defect was closed using a patch and the incised patent foramen ovale was directly closed. A transesophageal echography showed no abnormal findings in the operating room. On 1st postoperative day, the patient's heart rate and pressure of the superior vena cava increased while blood pressure decreased. A transthoracic echocardiography revealed an atrial septal hematoma. After conservative therapy, the heart rate and pressure of the superior vena cava gradually decreased while blood pressure increased. The atrial septal hematoma disappeared spontaneously. The patient was discharged on 16th postoperative day. The atrial septal hematoma appears to have a good prognosis, but sometimes can be poor resulting and death. Meticulous and constant observation is required to detect an atrial septal hematoma.

[Surgical strategy and the results for hypoplastic left heart syndrome].

OBJECTIVE: We retrospectively reviewed our surgical results in patients with hypoplastic left heart syndrome( HLHS) to investigate the influence of surgical strategy on outcome. METHODS: Seventy-seven patients with classic HLHS were involved in this study. For the initial palliation, 23 patients underwent Norwood operation with modified Blalock-Taussig (BT) shunt, 23 patients underwent Norwood operation with right ventricle to pulmonary artery( RV-PA) shunt and 31 patients underwent bilateral pulmonary artery banding (BPAB). Surgical results, freedom from Fontan operation, hemodynamic data and incidence of complication after Fontan operation were compared between the 3 groups. RESULTS: BPAB group had more preoperative risk factors than Norwood group. Total actuarial survival was 62.4% at 1 year and 58.2% at 3 and 5 years, and there was no difference between the groups. Freedom from Fontan completion was 59.4% at 3 years and 48.7% at 5 years without difference between the groups. The incidence of intervention for pulmonary artery stenosis was higher in Norwood with RV-PA shunt group(52.9%). Hemodynamic data obtained by cardiac catheterization were similar in the 3 groups, however, end-systolic elastance, which represents ventricular contractility, was lower in Norwood with RV-PA group. CONCLUSIONS: Surgical results of Norwood with BT shunt and Norwood with RV-PA shunt were comparably satisfactory, however, there was possible concern of reduced ventricular contractility in RV-PA shunt group. BPAB was a effective and useful initial palliation in high risk cases.

[Aortic and mitral valve replacement via right thoracotomy in the case of a patient with severe heart failure following right pneumonectomy].

We report a case of aortic valve replacement (AVR) and mitral valve replacement (MVR) in a patient with severe left ventricular dysfunction who had undergone right pneumonectomy for lung cancer 14 years previously. A 64-year-old man had cathecolamine-dependent heart failure due to bicuspid aortic valve stenosis, functional mitral valve regurgitation and impaired left ventricular function (left ventricular ejection fraction 13%)because of dilated cardiomyopathy. AVR and MVR were performed using St. Jude Medical mechanical valves with right thoracotomy because the heart had shifted to the right side. Poor left ventricular function and postoperative atrial fibrillation made it difficult to control low-output syndrome, but prolonged use of intra-aortic balloon pumping improved it gradually. Intraaortic balloon pumping( IABP) was removed 34 days after the operation, the respirator was removed with tracheotomy 63 days postoperatively, and the patient was discharged on the 177th postoperative day. Careful preoperative assessment and perioperative control of heart failure are important components of successful clinical management.

Configuration of the neoaortic root after chimney reconstruction in the Norwood procedure.

OBJECTIVES: After staged reconstruction for hypoplastic left heart syndrome, the neoaortic root tends to dilate, and the incidence of significant neoaortic valve insufficiency increases with time. This study aimed to evaluate the mid-term outcomes of the neoaortic root geometries and valve function after chimney reconstruction in the Norwood procedure. METHODS: Between 2013 and 2021, 20 consecutive patients who underwent chimney reconstruction during the Norwood procedure for hypoplastic left heart syndrome and its variants in our institution were enrolled. The actual diameters of the following points were measured, and Z-scores were calculated based on the normal aortic root geometries using the long axis view of echocardiography at the pre-Norwood stage and the lateral view of angiography at pre-Glenn, pre-Fontan, post-Fontan and follow-up (age 5-6 years) stages: neoaortic valve annulus; sinus of Valsalva; sinotubular junction; and ascending aorta just proximal to the anastomosis to the aortic arch. The degree of neoaortic valve regurgitation was evaluated by echocardiography at each stage. RESULTS: The median follow-up period was 3.9 years. Neoaortic roots after chimney reconstruction were spared from progressive dilation over time. With growth, the conical configuration of the neoaortic roots was preserved without geometrical distortion. The Z-scores of the annulus, sinus of Valsalva, sinotubular junction and ascending aorta ranged roughly from 4 to 6, 4 to 6, 2 to 4 and 0 to 2, respectively. All neoaortic valves at each stage had mild or no regurgitation. CONCLUSIONS: Chimney reconstruction prevented neoaortic root dilation and avoided significant neoaortic valve regurgitation in the mid-term. These neoaortic dimensions with smooth flow profiles in the neoaorta after chimney reconstruction may have contributed to the current results. Further studies are needed to clarify the long-term outcomes.

External Stenting for Bronchomalacia Involving the Bronchus Intermedius.

In recent years, external stenting has been used as a reliable method to relieve airway compression of the trachea and main bronchi in young children with acceptable age-proportional airway growth. However, to our knowledge, little literature supporting this approach for the distal airway has been published. A 1-year-old girl with absent pulmonary valve syndrome who had recurrent respiratory infections was diagnosed with bronchomalacia. She underwent external stenting; the infections disappeared postoperatively. This case describes a successful external stenting for malacia of the bronchus intermedius, which exhibited short- and mid-term safety and effectiveness.

Surgical Angioplasty for a Single Coronary Artery With Myocardial Bridging.

Single coronary artery with myocardial bridging is rare but associated with the risk of sudden cardiac death in children, yet there is no standardized treatment approach. We report a 6-year-old girl with chest pain having a single coronary artery with complete myocardial bridging of the left main coronary artery (modified Lipton type RII-S) branching from the right coronary artery at an acute angle. Coronary angioplasty using an in situ aortic flap and an autologous pulmonary arterial patch combined with myocardial unroofing was successfully performed for the left main coronary artery. The patient remains healthy for over 3 years without any exercise restriction.

Re-Norwood operation using femoral artery cannulation.

Re-Norwood operation is technically difficult to perform and is a high risk procedure due to the underlying hypocardiac function. Herein, we describe our successful re-Norwood operation approach in a 6-month old infant with persistent severe cyanosis and aortic re-coarctation. Our procedure was performed using femoral artery cannulation to protect cerebrospinal and lower body perfusion. Safe reopening of the chest was achieved, despite strong adhesions due to prior surgeries. Our repair and anastomosis techniques are described in detail. Cardiac circulation and function improved post-surgery. The patient was maintained on anti-heart failure drug therapy after surgery while awaiting a Glenn procedure.

Outcomes of bidirectional cavopulmonary shunt in patients younger than 4 months of age.

OBJECTIVES: Bidirectional cavopulmonary shunt (BCPS) has played an important role in the staged Fontan approach; however, the timing remains controversial, especially in younger patients. Therefore, we examined the outcomes of BCPS in infants younger than 3 months of age. METHODS: From 2004 to 2018, 120 patients underwent BCPS at <4 months of age (younger group). For reference, we also reviewed the data from 204 patients who had undergone the BCPS procedure during the same period at more than 4 months of age (older group). RESULTS: The median age and body weight at the time of the BCPS were 102 days and 4.2 kg for the younger group versus 196 days and 6.3 kg for the older group, respectively. Forty-eight patients (14.8%, 48 of 324; 16 in the younger group, 32 in the older group) had primary BCPS; the remaining 276 (104 in younger group, 172 in older group) had various forms of single-ventricle palliation before the BCPS procedure. Although preoperatively, 7 patients required extracorporeal membrane oxygenation (ECMO) support due to haemodynamic instability, they were successfully weaned from ECMO through haemodynamic benefits after BCPS. The 10-year actual survival rate (Kaplan-Meier) was 89% in the younger group and 86% in the older group (P = 0.55). Atrioventricular valve regurgitation (AVVR) was identified as a factor associated with hospital deaths in the younger group (P = 0.009), and much older age at BCPS was associated with late deaths in the older group (P = 0.027). CONCLUSIONS: In this study population, early performance of BCPS is applicable for patients who have undergone prior palliation and for those in whom primary BCPS is the first surgical intervention, even for patients with haemodynamic instabilities.

Long-term outcomes of total correction for isolated total anomalous pulmonary venous connection: lessons from 50-years' experience.

OBJECTIVES: Isolated total anomalous pulmonary venous connection (TAPVC) is a relatively rare congenital cardiac defect, while pulmonary venous obstruction (PVO) is associated with poor prognosis. We reviewed the long-term outcome of total correction for isolated TAPVC at our institute and analysed the risk factors for mortality and morbidity. METHODS: A total of 290 isolated TAPVC patients evaluated between 1965 and 2016 were divided into 2 groups: Group Early (n = 151) underwent surgery before 1989; Group Late (n = 139) underwent surgery after 1990. The mean age at operation was 10.4 ± 30.2 months (range 0 day to 23 years), and the mean body weight was 5.5 ± 6.0 kg (range 1.6-48 kg). Group Late included more patients with the infracardiac type of TAPVC and preferably used the posterior approach. RESULTS: There were 53 hospital deaths and 16 late deaths. Postoperative PVO was recognized in 28 patients. The mean follow-up time was 18.2 ± 9.7 years (range 2 months to 42.4 years). The actuarial survival rate was improved to 87.8% at 20 years in Group Late. Multivariable analysis revealed that death rate was significantly increased in patients of Group Early, with a body weight <2 kg and with postoperative PVO (P < 0.0001, P = 0.0041, P = 0.0003, respectively). Reoperations were performed 27 times in 22 patients (PVO repair, 11; staged repair, 4 and others, 12). PVO repair was performed at a mean of 2.5 ± 1.6 months later. The actuarial freedom from reoperation rates were 88.8% and 83.2% at 20 and 30 years, respectively. Multivariable analysis revealed that the risk of reoperation was associated with mixed-type TAPVC and postoperative PVO (P = 0.0064 and P < 0.0001, respectively). CONCLUSIONS: Long-term surgical outcomes of isolated TAPVC have improved over the past 25 years. Postoperative PVO, the mixed-type TAPVC and a body weight <2 kg might be the important factors contributing to mortality and morbidity.

Usefulness of intraoperative continuous infusion of tranexamic acid during emergency surgery for type A acute aortic dissection.

PURPOSE: We investigated the influence of intraoperative continuous tranexamic acid (TA) infusion on the amount of blood transfusion required in emergency surgery for type A acute aortic dissection. METHODS: The study was based on the data of 55 consecutive patients who underwent surgery for type A acute aortic dissection. The patients were divided into 2 groups for comparison: Group T, consisting of 26 patients who received intraoperative continuous infusion of TA, and Group N, consisting of 29 patients who did not receive TA infusion during the surgery. RESULTS: The mean amounts of blood transfusion required during and after surgery were compared between the 2 groups: they were 10.5 ± 8.7 and 16.2 ± 10.0 units of mannitol-adenine-phosphate-added red cell concentrate, 9.3 ± 8.6 and 17.1 ± 10.0 units of fresh frozen plasma, and 20.4 ± 12.2 and 29.7 ± 14.9 units of platelet concentrate, respectively, in Groups T and N. Thus, the amount of each of these blood products required was significantly reduced in Group T. CONCLUSIONS: During emergency surgery for type A acute aortic dissection, continuous infusion of TA resulted in a significant reduction in the amount of blood transfusion required.

Durability of aortic valve preservation with root reconstruction for acute type A aortic dissection.

OBJECTIVES: We evaluated the durability of aortic valve preservation with root reconstruction for acute type A aortic dissection (AAAD). METHODS: From January 2002 to March 2011, 140 patients [70 males, 68 ± 12 (SD) years] underwent emergency operation for AAAD. The aortic valve was preserved and one or more Valsalva sinuses were reconstructed. Techniques used for reconstruction were valve resuspension and additional reinforcement of the aortic root with Teflon felt patches, and gelatin-resorcinol-formaldehyde-glue (GRF-glue) was used for mending the dissection. The mean follow-up period was 44.0 ± 26.2 months. We classified the degree of aortic regurgitation (AR) into four grades (0, 1+, 2+ and 3+) using echocardiography. Based on a retrospective analysis of pre-operative echocardiographic findings, the 127 survivors were divided into two groups: group 1 (G1) included 98 patients with 0 or 1+ AR, and group 2 (G2) 29 patients with 2+ or 3+ AR. In addition, we measured the post-operative native aortic root dimension of AAAD patients with use of echocardiography or CT scan. RESULTS: The operative mortality rate was 9.3% (13/140). Freedom from aortic root re-operation was 100%. Aortic root pseudoaneurysm formation and severe AR requiring aortic valve replacement did not occur. Pre-operative AR of 0.2 ± 0.4 in G1 did not deteriorate (0.5 ± 0.5 at discharge, 0.4 ± 0.4 at follow-up). Meanwhile, pre-operative AR of 2.4 ± 0.5 in G2 improved to 0.6 ± 0.5 (P < 0.05) at discharge and 1.0 ± 0.6 (P < 0.05) at follow-up. The native aortic root dimension in G2 at follow-up was significantly larger than G1 (36.0 ± 4.7 vs. 33.9 ± 5.0 mm). CONCLUSIONS: Aortic valve preservation and root reconstruction appear to be an appropriate surgical approach to AAAD.