Pulmonary Artery Angioplasty for Improving Ipsilateral Lung Perfusion in Adolescent and Adult Patients: An Analysis Based on Cardiac Magnetic Resonance Imaging and Lung Perfusion Scanning.

Background: The left pulmonary artery (LPA) may be kinked and stenotic, especially in tetralogy of Fallot, because of ductal tissue and anterior deviation of the conal septum. If LPA stenosis is not effectively treated during total correction, surgical angioplasty is occasionally performed. However, whether pulmonary artery (PA) angioplasty in adolescents or adults improves perfusion in the ipsilateral lung remains unclear. Methods: This retrospective review enrolled patients who underwent PA angioplasty for LPA stenosis between 2004 and 2019. Among patients who underwent a lung perfusion scan (LPS) or cardiac magnetic resonance imaging (cMRI) pre- and post-pulmonary angioplasty, those aged >13 years with <40% left lung perfusion (p-left) in the pre-angioplasty study were included. Preoperative and postoperative computed tomography, LPS, and cMRI data were collected. The perfusion ratio was analyzed according to the LPA's anatomical characteristics. Results: Seventeen adolescents and 16 adults (≥18 years old) were finally included (median age, 17 years). The most common primary diagnosis was tetralogy of Fallot (87.9%). In all patients, LPA angioplasty was performed concomitantly with right ventricular outflow tract reconstruction. No patients died. Preoperative p-left was not significantly different between adolescents and adults; however, adolescents had significantly higher postoperative p-left than adults. P-left significantly increased in adolescents, but not in adults. Seven patients had significant stenosis (z-score <-2.0) confined only to the proximal LPA and demonstrated significantly increased p-left. Conclusion: PA angioplasty significantly increased ipsilateral lung perfusion in adolescents. If focal stenosis is confined to the proximal LPA, PA angioplasty may improve ipsilateral lung perfusion, regardless of age.

Outcomes of not using tracheoplasty in asymptomatic tracheal stenosis found during open-heart surgery.

OBJECTIVES: We aimed to review the outcomes of treating incidentally encountered asymptomatic airway stenosis during open-heart surgery conservatively without the use of tracheoplasty. METHODS: Between January 2002 and October 2022, 25 patients were incidentally diagnosed with tracheal stenosis during open-heart surgery. Intraoperative bronchoscopy and/or laryngoscopy revealed tracheal stenosis; however, this was not consistent with the findings of the preoperative computed tomography. Patients who were diagnosed with a pulmonary artery or vascular sling or had moderate-to-severe respiratory symptoms before open-heart surgery were excluded. RESULTS: The median age and weight of the patients at operation were 3.0 months and 5.1 kg, respectively. They were categorized as those having tracheal stenosis on preoperative computed tomography (n = 12) or not having tracheal stenosis (n = 13). The narrowest diameter was significantly smaller in the former group (3.0 vs 5.8 mm, P < 0.05). The rates of reintubation and the tracheostomy, and intubation days tended to be higher in former group without statistical significance. Stenotic degree improved 2 months and 1 year or more after the operation (39.3% at operation, 28.4% at 2 months, 12.5% after 1 year). All patients were Ross class 1 or 2 at follow-up (mean, 7.1 years). CONCLUSIONS: Patients with tracheal stenosis showed tolerable long-term outcomes without using tracheoplasty. Accordingly, if tracheal stenosis, that would cause intubation difficulty, was incidentally revealed, concomitant tracheoplasty may not be required during open-heart surgery if the stenosis did not cause considerable symptoms or signs preoperatively.

Risk Factors for Coronary Artery Complications After Prosthetic Pulmonary Valve Implantation in Patients With Congenital Heart Disease.

BACKGROUND: Coronary artery complications (CACs) in patients who undergoing prosthetic pulmonary valve implantation for congenital heart disease can lead to fetal outcomes. However, the incidence of and risk factors for CACs in these patients remain unknown. METHODS AND RESULTS: A retrospective cohort study was conducted on patients who underwent cardiac computed tomography or invasive coronary angiography after prosthetic pulmonary valve implantation at Seoul National University Hospital from June 1986 to May 2021. Among 341 patients, 25 (7.3%) were identified with CACs, and 2 of them died. Among the patients with CACs, congenital coronary anomalies and an interarterial course of the coronary artery were identified in 11 (44%) and 18 (72%) patients, respectively. Interarterial and intramural courses of the coronary artery were associated with a 4.4- and 10.6-fold increased risk of CACs, respectively. Among patients with tetralogy of Fallot and pulmonary atresia, the aortic root was rotated further clockwise in patients with coronary artery compression compared to those without it (mean [±SD] 128.0±19.9° vs. 113.5±23.7°; P=0.024). The cut-off rotation angle of the aorta for predicting the occurrence of coronary artery compression was 133°. CONCLUSIONS: Perioperative coronary artery evaluation and prevention of CACs are required in patients undergoing prosthetic pulmonary valve implantation, particularly in those with coronary artery anomalies or severe clockwise rotation of the aortic root.

Extubation in operating room versus early extubation in ICU after open-heart surgery in patients with CHDs.

BACKGROUNDS AND OBJECTIVES: The "Fast track" protocol is an early extubation strategy to reduce ventilator-associated complications and induce early recovery after open-heart surgery. This study compared clinical outcomes between operating room extubation and ICU extubation after open-heart surgery in patients with CHD. METHODS: We retrospectively reviewed 215 patients who underwent open-heart surgery for CHDs under the scheduled "Fast track" protocol between September 2016 and April 2022. The clinical endpoints were post-operative complications, including bleeding, respiratory and neurological complications, and hospital/ICU stays. RESULTS: The patients were divided into operating room extubation (group O, n = 124) and ICU extubation (group I, n=91) groups. The most frequently performed procedures were patch closures of the atrial septal (107/215, 49.8%) and ventricular septal (89/215, 41.4%) defects. There were no significant differences in major post-operative complications or ICU and hospital stay duration between the two groups; however, patients in group I showed longer mechanical ventilatory support (0.0 min vs. 59.0 min (interquartile range: 17.0-169.0), p < 0.001). Patients in Group O showed higher initial lactate levels (3.2 ± 1.7 mg/dL versus 2.5 ± 2.0 mg/dL, p = 0.007) and more frequently used additional sedatives and opioid analgesics (33.1% versus 19.8%, p = 0.031). CONCLUSIONS: Extubation in the operating room was not beneficial for patients during post-operative ICU or hospital stay. Early extubation in the ICU resulted in more stable hemodynamics in the immediate post-operative period and required less use of sedatives and analgesics.

Suture tie-down forces and cyclic contractile forces after undersized tricuspid annuloplasty using a Tri-Ad Adams tricuspid annuloplasty ring in an ovine model.

OBJECTIVES: This study evaluated suture tie-down forces and cyclic contractile forces (CCFs) after undersized tricuspid annuloplasty using a hybrid band. METHODS: Downsized tricuspid annuloplasty was planned in adult male sheep using 8 force transducers attached from the septal to the anterior annular areas of the ring (segments 1 and 2, flexible septal; segments 3 and 4, semi-rigid posterior; segments 5 and 6, semi-rigid anterior; segments 7 and 8, flexible anterior). CCFs were analysed at 3 different levels of peak right ventricular pressure (RVP): 30, 50 and 70 mmHg. RESULTS: Eight 5-year-old male Corriedale sheep (average body weight = 66.8 kg) were used. The average suture tie-down force was 4.42 [standard deviation (SD): 2.32] N. When the forces were compared, it was lowest in the flexible anterior area and highest in the flexible septal area (P < 0.001). With the RVP of 30 mmHg, the average CCFs was lowest at segment 3 [0.07 (SD: 0.07) N] and highest at segment 7 [0.15 (SD: 0.08) N]. The CCFs were 0.12 (SD: 0.1) N, 0.09 (SD: 0.12) N, 0.14 (SD: 0.1) N and 0.13 (SD: 0.09) N in the flexible septal, semi-rigid posterior, semi-rigid anterior and flexible anterior parts, respectively (P = 0.208). As the peak RVP increased to 50 and 70 mmHg, the CCFs of each area increased significantly (P < 0.001). Despite this increase, the CCFs remained low (0.1 and 0.3 N), and differences in CCFs between segments and between annular areas showed similar patterns. CONCLUSIONS: The flexible end of the hybrid band reduces the CCFs and might prevent annular tears after ring tricuspid annuloplasty, and the risk of tear would be low even in the septal area.

Ten-year follow-up of dilatation of aortic structures in Fallot-type anomalies.

OBJECTIVES: Dilatation of the aortic root structures or ascending aorta is often observed in patients with Fallot-type anomalies. We aimed to determine the dilation rate of the aortic structures and investigate strategies for managing this phenomenon. METHODS: In this retrospective study, we enrolled 66 out of 801 patients who underwent corrective surgery for Fallot-type anomalies (tetralogy of Fallot [TOF] and Fallot-type double outlet right ventricle [DORV]) between 2004 and 2020. These 66 patients had follow-up cardiac computed tomography (CT) angiography images taken at least 5 years after the initial CT study. We analyzed the diameters and aortic cross-sectional area/height ratio (AH) of the aortic annulus, sinus of Valsalva, sinotubular junction, and ascending aorta between the initial and follow-up CT scans. "Dilatation" was defined as a z-score over 2 for each aortic structure. RESULTS: The median age at the initial and follow-up CT scans was 5.9 years (interquartile range [IQR]: 0.4 ~ 12.4) and 15.9 years (IQR 9.3 ~ 23.4), respectively. The median CT interval (from initial to latest CT) was 9.5 years (IQR 6.6 ~ 12.0). The sinus of Valsalva exhibited the most significant dilation (32.8 mm at follow-up CT) over the study period. The AH ratio increased significantly in all four aortic structures. The patient's age was significantly associated with higher AH in the follow-up CT. Aortic dilatation was present in 74.2% of patients at the initial CT and increased to 86.4% at the follow-up CT. CONCLUSION: In Fallot-type anomalies, the AH ratio of aortic root structures significantly increased over an average period of approximately 9.5 years. The number of patients diagnosed with aortic dilatation also increased. Based on our observations in this study, these patients' group should be considered for more frequent follow-up examinations, as significant dilatation could occur in their mid-20 s.

Valve-Sparing Root Replacement in a Turner Syndrome Patient with Bicuspid Aortic Valve and Juxtacommissural Origin of the Right Coronary Artery: A Case Report.

A 32-year-old woman diagnosed with Turner syndrome presented to the hospital for an evaluation of cardiovascular complications. Preoperative computed tomography (CT) and echocardiography showed progression of aortic root and ascending aorta dilatation, as well as a bicuspid aortic valve. There was no evidence of aortic regurgitation. We planned valve-sparing aortic root replacement and ascending aorta replacement with a high risk of aortic rupture. Intraoperatively, we incidentally found a juxtacommissural origin of the right coronary artery (RCA). We performed aortic valve reimplantation using a graft designed with a key-shaped hole to wrap the juxtacommissural-origin RCA by modifying the Florida sleeve technique. Coronary blood flow was patent on postoperative CT angiography, and there was no evidence of aortic regurgitation on postoperative echocardiography. The patient was discharged from the hospital on postoperative day 7 without any complications.

Outcomes of the Warden Procedure for Anomalous Pulmonary Venous Return to the Superior Vena Cava: A 17-Year Experience.

Background: Surgical repair of partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) using the Warden procedure has favorable outcomes. However, there remain some concerns after the Warden procedure, such as sinoatrial nodal dysfunction and systemic or pulmonary venous stenosis. We investigated the outcomes of the Warden procedure for repair of PAPVR to the SVC. Methods: This retrospective study included 22 consecutive patients who underwent the Warden procedure for PAPVR between 2002 and 2018. The median age and body weight at operation were 27.5 months (interquartile range [IQR], 5.0-56.8 months) and 13.2 kg (IQR, 6.5-16.0 kg), respectively. The median follow-up duration was 6.2 years (IQR, 3.5-11.6 years). Results: There were no cases of early or late mortality. No patients had postoperative heart rhythm problems, except 1 patient who showed transient sinoatrial nodal dysfunction in the immediate postoperative period. Procedure-related complications requiring reintervention occurred in 5 patients, including 3 of 4 SVC stenosis cases and 2 pulmonary venous stenosis cases during follow-up. The rate of freedom from reintervention related to the Warden procedure was 75.9% at 10 years. Conclusion: In cases requiring extension or creation of an atrial septal defect to achieve a sufficient venous pathway, or interposition of an entire circumferential conduit between the SVC and right atrium due to the shortness of the SVC in the Warden procedure, stenotic complications of the venous pathway occurred. Careful observation of changes in the pressure gradient or anatomical stenosis is required in such patients.

Postcardiotomy Extracorporeal Membrane Oxygenation Support in Patients with Congenital Heart Disease.

BACKGROUND: This study investigated mortality and morbidity in patients requiring postcardiotomy extracorporeal membrane oxygenation (ECMO) support after operations for congenital heart disease (CHD). METHODS: CHD patients requiring postoperative ECMO support between May 2011 and May 2021 were retrospectively reviewed. Patients were divided into non-survivors and survivors to hospital discharge. Survival outcomes and associations of various factors with in-hospital death were analyzed. RESULTS: Fifty patients required postoperative ECMO support. Patients' median age and weight at the time of ECMO insertion were 1.85 months (interquartile range [IQR], 0.23-14.5 months) and 3.84 kg (IQR, 3.08-7.88 kg), respectively. Twenty-nine patients (58%) were male. The median duration of ECMO support was 6 days (IQR, 3-12 days). Twenty-nine patients (58%) died on ECMO support or after ECMO weaning, and 21 (42%) survived to hospital discharge. Postoperative complications included renal failure (n=33, 66%), bleeding (n=11, 22%), and sepsis (n=15, 30%). Prolonged ECMO support (p=0.017), renal failure (p=0.005), continuous renal replacement therapy (CRRT) application (p=0.001), sepsis (p=0.012), bleeding (p=0.032), and high serum lactate (p=0.002) and total bilirubin (p=0.017) levels during ECMO support were associated with higher mortality risk in a univariate analysis. A multivariable analysis identified CRRT application (p=0.013) and a high serum total bilirubin level (p=0.001) as independent risk factors for death. CONCLUSION: Postcardiotomy ECMO should be considered as an important therapeutic modality for patients unresponsive to conventional management. ECMO implementation strategies and management in appropriate patients without severe complications, particularly renal failure and/or liver failure, are crucial for achieving positive outcomes.

Long-term clinical outcomes of coronary artery bypass grafting in young children with Kawasaki disease.

BACKGROUND: Although coronary artery bypass grafting is not frequently performed in children, Kawasaki disease is one of the most common indications for coronary artery bypass grafting in children. Here, we reviewed the long-term clinical outcomes including graft patency after coronary artery bypass grafting. METHODS: Between March 2004 and March 2013, six patients with Kawasaki disease underwent coronary artery bypass grafting. All patients were male. Their median age was 13.0 years (interquartile range, 7.8-17.8 years) at the timing of coronary artery bypass grafting, and the median age at the onset of Kawasaki disease was 3.3 years (interquartile range, 1.0-7.0 years). Four patients presented with multiple lesions including aneurysms. RESULTS: The median follow-up duration was 12.1 years (interquartile range, 9.5-13.1 years), and there were no operative complications or overall mortality. One patient had pre-operative symptoms such as exertional chest pain and dyspnoea on exertion, whereas one patient had ventricular tachyarrhythmia. There was an improvement in subjective symptoms after surgery in two patients. The left internal thoracic artery, right internal thoracic artery, and saphenous vein were used in five (83.3%), one (16.7%), and two (33.3%) cases, respectively. In all six patients, post-operative single-photon emission CT findings showed improved perfusion compared with pre-operative single-photon emission CT. All grafts were patent as confirmed by coronary angiography or CT angiography. CONCLUSIONS: Coronary artery bypass grafting could be a good surgical option in children with coronary lesions caused by Kawasaki disease in terms of graft patency and myocardial perfusion.

Aortic Valve Repair in Young Patients: A Single Patch Design for Leaflet Extension.

The surgical treatment for young patients with aortic valve diseases has not been standardized because of differences in the patients' growth. We aimed to investigate short-term results of aortic valve repair with leaflet extension techniques. From October 2017 to June 2020, we performed aortic valve repair with leaflet extension technique in 15 consecutive patients with a median age of 13 years (range, 33 months-27 years). Among them, all had moderate or severe aortic regurgitation (AR), eight had concomitant aortic stenosis, and 12 underwent prior cardiac operations. The leaflet patch design has been modified from separate patch design to single patch design. No early and late deaths were reported, no re-operations were performed, and temporary ectopic atrial arrhythmia was the only early complication noted. The patients were discharged with less than moderate AR after a median postoperative period of 5 (range, 3-7) days. All the patients were New York Heart Association class I after a median follow-up period of 17.3 (range, 4.4-34.6) months. However, two patients progressed to moderate AR postoperatively at 6 and 30 months, respectively, after which one was treated with single patch technique. Among the remaining patients, six had trace, six had mild, and one had mild-to-moderate AR. Aortic valve repair with leaflet extension is acceptable in young patients. Our single patch design was easy to manipulate and showed good short-term results. Long-term follow-up is required to further confirm the efficacy of this technique.

Long-Term Outcomes of Atrioventricular Valve Surgery in Patients with Functional Single Ventricle: Should We Avoid Valve Replacement?

Atrioventricular valve regurgitation (AVVR) is associated with increased morbidity and mortality in patients with single ventricle physiology. The purpose of this study was to evaluate the long-term results of the surgical management of AVVR and to analyze the effects of AV valve replacement. The medical records of 38 single ventricle patients who underwent atrioventricular valve surgery more than once between January 2001 and March 2018 were retrospectively reviewed. We analyzed and compared clinical data of patients who underwent valve replacement as an initial treatment (n = 8) for AVVR with patients who initially underwent valve repair (n = 30). The median follow-up duration was 98.1 months (range, 0.9-209.6 months). There was one early mortality and seven late mortalities. Freedom from reoperation between the two groups at 15 years of follow-up was significantly different: 18.3% in the repair group and 100% in the replacement group (p = 0.013). The replacement group showed a better overall survival rate (100%) at 15 years than the repair group (68.5%) without statistical significance (p = 0.097). All mortalities occurred in the repair group. Nine patients in the repair group (30%) and one patient in the replacement group (12.5%) showed preoperative ventricular dysfunction. RV-type single ventricle with atrioventricular (AV) valve annular dilatation was found out as a risk factor of AVV replacement both in univariate (p = 0.04) and multivariate (p = 0.004) analysis. AV valve replacement might be considered as a primary treatment option for patients who have an annular dilation with an RV-type single ventricle rather than repeated valvuloplasty.

Pulmonary valve replacement may not restore ventricular volume and functional status in patients with pulmonary regurgitation after late tetralogy of Fallot repair.

OBJECTIVES: Clinically, tetralogy of Fallot (TOF) patients who underwent repair late (older than 2 years) appears to have worse outcomes after pulmonary valve replacement than patients who underwent repair early. We proceeded to review the clinical features of late-repaired TOF patients who required pulmonary valve replacement. METHODS: Fifty patients who underwent pulmonary valve replacement after TOF repair over the age of 2 years from 2000 to 2018 were retrospectively reviewed. Pre- and postoperative cardiac magnetic resonance imaging, cardiopulmonary exercise tests, and cardiac catheterization were analysed. RESULTS: The median age of patients at the time of TOF repair and pulmonary valve replacement was 3.6 and 23.4 years, respectively. The median interval from TOF repair to pulmonary valve replacement was 20.5 years. Cardiac magnetic resonance imaging and cardiopulmonary exercise tests were performed at a median of 5.9 and 3.7 years after pulmonary valve replacement, respectively. Cardiac magnetic resonance revealed that there were significant changes in the indexed values of the right ventricle end-diastolic volume (164.7-106.9 ml/m2, P < 0.001), end-systolic volume (101.4-64.9 ml/m2, P < 0.001), stroke volume (66.8-48.0 ml/m2, P = 0.007) and cardiac output (5.1-3.6 l/m2, P = 0.040). Twenty-eight percentage of patients achieved normalization of the right ventricular volume after pulmonary valve replacement. In the exercise test, the maximum rate of oxygen consumption (72.5-69.5%) and oxygen pulse (95.0-83.0%) changed without statistical significance. CONCLUSIONS: Although pulmonary valve replacement after late TOF correction improves right ventricular volume status, only a minority of patients achieve normalization of right ventricular end systolic volume and a normal functional status.

Surgical outcomes of infective endocarditis in children: should we delay surgery for infective endocarditis?

OBJECTIVES: We compared the surgical outcomes of infective endocarditis (IE) between early surgery and non-early surgery groups in children. METHODS: From January 2000 to April 2020, we retrospectively reviewed 50 patients <18years of age who underwent first surgery for IE. Early surgery was defined as that performed within 2 days for left-sided IE and 7 days for right-sided IE after diagnosis. RESULTS: The median age and body weight at operation were 7.7 years [interquartile range (IQR), 2.3-13.2] and 23.7 kg (IQR, 10.3-40.7), respectively. The median follow-up duration was 9.5 years (IQR, 4.0-14.5). In 28 patients with native valve endocarditis, the native valve was preserved in 23 (82.1%). The most common causative microorganism was Streptococcus viridans (32.0%). The operative mortality was 2.0%, and 13 (26.0%) patients required reoperation most commonly for prosthesis failure (n = 7). There were no significant differences in patient characteristics and perioperative data between early surgery (n = 9) and non-early surgery (n = 36) groups, except for the interval between diagnosis and surgery (early surgery < non-early surgery, P < 0.001) and preoperative negative blood culture conversion (early surgery < non-early surgery, P = 0.025). There were no significant differences in overall survival, recurrent IE, and reoperation rate between the groups. Early surgery and preoperative negative blood culture conversion were not found as significant factors for surgical adverse outcomes. CONCLUSIONS: Surgical outcomes for IE in children were acceptable irrespective of the time of surgery. Our results suggest that it may not be required to delay surgery for IE and the potential benefit of early surgery could be expected in children.

Tricuspid valve detachment for ventricular septal defect closure in infants <5 kg: should we be hesitant?

OBJECTIVES: We compared the clinical outcomes between tricuspid valve detachment (TVD) and non-TVD for ventricular septal defect (VSD) closure in infants <5 kg. METHODS: From January 2004 to April 2020, 462 infants <5 kg with VSD without more complex intracardiac lesions and who had undergone VSD closure through the trans-atrial approach were enrolled. Propensity score-matching analysis was performed. Clinical outcomes were compared between the paired TVD group (group D) and paired non-TVD group (group N). RESULTS: The median age and body weight at operation were 1.9 months [interquartile range(IQR), 1.4-2.5] and 4.2 kg (IQR, 3.7-4.6). The median follow-up duration was 83.4 months (IQR, 43.5-130.4). After matching, 44 pairs were extracted from each group. There were no significant differences in all-cause mortality (P = 0.176), reoperation (P = 0.172), postoperative morbidities, including residual VSD, aortic regurgitation, atrioventricular block and significant tricuspid regurgitation (TR) (P = 0.346) between group D and group N. However, group D showed significantly less TR progression during follow-up (P = 0.019). CONCLUSIONS: In infants <5 kg, TVD can be a reasonable and valid option for successful VSD closure without morbidities, including TR progression if the indication exists.

Suture tie-down forces and cyclic contractile forces after an undersized tricuspid annuloplasty using a 3-dimensional rigid ring in an ovine model.

OBJECTIVES: This study was conducted to measure suture tie-down forces and evaluate cyclic contractile forces (CCFs) in beating hearts after undersized 3-dimensional (3D) rigid-ring tricuspid valve annuloplasty (TAP). METHODS: Eight force transducers were attached to the 3D rigid TAP ring. Segments 1 to 8 were attached from the mid-septal to anterior-septal commissural area in a counterclockwise order. Two-sizes-down ring TAPs were performed in 6 sheep. Tie-down forces and CCF were recorded and analysed at the 8 annular segments and at 3 levels of peak right ventricular pressure (RVP: 30, 50 and 70 mmHg). RESULTS: The overall average tie-down forces and CCF were 4.34 ± 2.26 newtons (N) and 0.23 ± 0.09 N, respectively. The CCF at an RVP of 30 mmHg were higher at 3 commissural areas (segments 3, 5 and 8) than at the other segments. The increases in the CCF following changes in the RVP were statistically significant only at the 3 commissural areas (P = 0.012). However, mean CCFs remained low at all annular positions (ranges of average CCF = 0.06-0.46 N). CONCLUSIONS: The risk of suture dehiscence after down-sized 3D rigid-ring TAP might be minimal because the absolute forces remained low in all annular positions even in the condition of high RVP. However, careful suturing in the septal annular area and commissures is necessary to prevent an annular tear during a down-sized 3D rigid-ring TAP.

Primary Versus Staged Repair in Neonates With Pulmonary Atresia and Ventricular Septal Defect.

BACKGROUND: The 2 surgical strategies for neonates with ductal-dependent pulmonary atresia and ventricular septal defect are primary biventricular repair (BVR) or initial palliation with a modified Blalock-Taussig shunt (BTS) followed by second stage repair. In this study, we report the combined outcomes from 2 hospitals using different strategies. METHODS: Between 2004 and 2017, 66 neonates underwent surgery with palliative shunts (BTS group: n = 30, 45.5%) or primary biventricular repair (pBVR group: n = 36, 54.5%). The 2 groups were similar in age, body weight, and Nakata index scores. The overall mean follow-up duration was 7.51 ± 4.35 years, and early and late results were compared between the groups. RESULTS: The 10-year overall survival was 84.8% (94.4% for pBVR vs 75.7% for BTS, P = .032). The BTS group had 2 early and 6 interstage mortalities, and the pBVR group had no early and 2 late mortalities. In the BTS group, the Nakata index score significantly increased during the interstage period (P < .001). In univariable analysis, genetic or extracardiac anomalies were a risk factor for mortality (hazard ratio, 5.56; P = .038). After achieving BVR, the pBVR group underwent significantly more frequent right ventricle outflow tract reinterventions (P < .001) at a much earlier period (P = .017) compared with the BTS group. CONCLUSIONS: In neonates with ductal-dependent pulmonary atresia and ventricular septal defect, the primary BVR approach provides an excellent survival rate, but the burden of right ventricle outflow tract reintervention is heavy. The staged approach with BTS promotes pulmonary artery growth, but hospital and interstage mortality are significant. Genetic and extracardiac anomalies are significant risk factors for mortality.

Extracorporeal Membrane Oxygenation in Pediatric Patients with Respiratory Failure: Early Experience with the Double-Lumen Cannula Over 2 Years.

BACKGROUND: The double-lumen cannula (DLC) has begun to be used worldwide for venovenous (VV) extracorporeal membrane oxygenation (ECMO). We aimed to examine whether the DLC could be an effective tool in the treatment of pediatric respiratory failure in Korea. METHODS: We reviewed the records of patients weighing under 15 kg who underwent ECMO due to respiratory failure between January 2017 and December 2018. Outcomes of ECMO using a DLC and conventional ECMO using central method or 2 peripheral cannulas were compared. RESULTS: Twelve patients were treated with ECMO for respiratory failure. Among them, a DLC was used in 5 patients, the median age of whom was 3.8 months (interquartile range, 0.1-49.7 months). In these patients, the median values of pH, partial pressure of carbon dioxide, and partial pressure of oxygen were 7.09, 74 mm Hg, and 37 mm Hg before ECMO and corrected to 7.31, 44 mm Hg, and 85 mm Hg, respectively, after ECMO cannulation. Median blood flow rate in the patients treated with ECMO using a DLC was slightly higher than that in the conventional ECMO group, but this difference was not statistically significant (86.1 mL/kg/min and 74.3 mL/kg/min, respectively; p=1.00). One patient from the DLC group and 3 patients from the conventional group were weaned off ECMO. CONCLUSION: VV ECMO using a DLC provided adequate oxygenation, ventilation, and blood flow rate in Korean pediatric patients with respiratory failure. Further prospective and randomized studies are warranted.

A case of life-threatening valvulitis mimicking infective endocarditis in systemic juvenile idiopathic arthritis.

Differential diagnosis of an intracardiac mass is difficult when detected only by echocardiography before a biopsy is completed. However, treatment cannot be postponed until the biopsy results are obtained. We report the case of a 12-year-old girl who presented with an intracardiac mass in the mitral valve mimicking infective endocarditis and severe mitral regurgitation. The mass was finally diagnosed as valvulitis associated with systemic juvenile idiopathic arthritis, which was complicated with macrophage activation syndrome. After careful exclusion of acute infectious disease, we started steroid pulse therapy and administered tocilizumab to treat the cytokine storm before performing the surgery. Finally, we performed mass excision and mitral valve replacement after immunosuppressant therapy.

Outcomes of aortic coarctation surgical repair in adolescents and adults.

OBJECTIVES: Coarctation of the aorta (CoA) in adolescents and adults is relatively rare. Several operative techniques have been reported, but there is no consensus. METHODS: From November 1994 to July 2018, a total of 24 adolescents and adults underwent CoA repair. The mean age at operation was 29.9 ± 15.1; 19 (79%) patients were older than 18. Sixteen (67%) patients had arterial hypertension, 5 (21%) patients had bicuspid aortic valve, 4 (17%) patients had descending aneurysm, 2 (8%) patients had ascending aneurysm, 2 (8%) patients had patent ductus arteriosus and 1 (4%) patient had atrial septal defect. Three patients had prior surgery (2 CoA repair, 1 ventricular septal defect repair). RESULTS: Surgical corrections included extra-anatomical bypasses in 12 (50%) patients (9: left subclavian artery to descending aorta bypass, 2 proximal-to-distal coarctation bypasses, 1 ascending-to-descending aortic bypass), end-to-end anastomosis in 6 (25%) patients, resections and interpositions of a tube graft in 5 (21%) patients and arch augmentation with a tube graft in 1 (4%) patient. The mean follow-up duration was 6.2 ± 5.1 years. No mortality was observed. No patient required reoperation or reintervention. The mean upper extremity systolic pressure significantly decreased from 142.4 ± 30.3 mmHg preoperatively to 121.1 ± 15.9 mmHg postoperatively (P = 0.002). Arterial pressure gradient between upper and lower extremities significantly decreased from 50.0 ± 21.8 mmHg preoperatively to 9.7 ± 13.5 mmHg postoperatively (P < 0.001). Among patients undergoing left subclavian artery to descending aorta bypass, 8 patients underwent ankle brachial pressure index evaluation. Postoperative mean right- and left-sided ankle brachial pressure index were 0.96 ± 0.16 and 0.94 ± 0.11, respectively. All grafts were patent at the last follow-up. CONCLUSIONS: CoA repair in adolescents and adults showed good outcomes. Left subclavian artery to descending aorta bypass grafting is safe and effective for managing CoA in adolescents and adults.