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.

An extreme case of cor triatriatum mimicking hypoplastic left heart syndrome and combined pulmonary vein stenosis.

A 65-day-old girl presented to the emergency room with lethargy, requiring emergency venoarterial extracorporeal membrane oxygenation for refractory cardiogenic shock. Initially, hypoplastic left heart syndrome was suspected. However, cor triatriatum with a pinpoint opening on the membrane was diagnosed based on a detailed echocardiographic examination. After membrane resection, the left heart size was restored. However, follow-up echocardiography performed 4 months later showed occlusion of both upper pulmonary veins and stenosis in both lower pulmonary veins. Hybrid balloon angioplasty was performed in all pulmonary veins, and stents were inserted into the right upper and lower pulmonary veins. Despite repeated balloon angioplasty, all pulmonary vein stenosis progressed over 6 months and the patient expired while waiting for a heart-lung transplant. Even after successful repair of cor triatriatum, short-term close follow-up is required for detecting the development of pulmonary vein stenosis.

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.

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.

Shallow suture at ventricular septal defect may safely reduce right bundle branch block.

BACKGROUND: To avoid rhythm disturbance, sutures for ventricular septal defect closure have been traditionally placed 2∼5 mm or more away from the edge of the ventricular septal defect. However, the traditional suturing method appears to induce right bundle branch block and tricuspid valve regurgitation after ventricular septal defect closure more than our alternative technique, shallow suturing just at the edge of the ventricular septal defect (shallower bites at the postero-inferior margin). We aimed to verify our clinical experience of perimembranous ventricular septal defect repair. METHODS: The alternative shallow suturing method has been applied since 2003 at our institution. We retrospectively reviewed the clinical data of 556 isolated perimembranous ventricular septal defect patients who underwent surgical closure from 2000 to 2019. We investigated the postoperative occurrence of right bundle branch block or progression of tricuspid regurgitation and analysed risk factors for right bundle branch block and tricuspid regurgitation. RESULTS: Traditional suturing method (Group T) was used in 374 cases (66.8%), and alternative suturing method (Group A) was used in 186 cases (33.2%). The right bundle branch block occurred more frequently in Group T (39.6%) than in Group A (14.9%). In multivariable logistic regression analysis, Group T and patch material were significant risk factors for late right bundle branch block. More patients with progression of tricuspid regurgitation were found in Group T. CONCLUSIONS: Shallow suturing just at the edge of the ventricular septal defect may reduce the rate of right bundle branch block occurrence and tricuspid regurgitation progression without other complications.

Left atrial thrombosis with invasive pulmonary aspergillosis in children with immunodeficiency.

Invasive aspergillosis is a major cause of infectious disease in immunocompromised patients; however, cardiac involvement in pulmonary aspergillosis is not well-known. Two paediatric patients undergoing chemotherapy were diagnosed with cardiac aspergilloma, accompanied by pulmonary aspergillosis. In both patients, antibiotic and antifungal treatments were initiated immediately after the pneumonia was diagnosed; however, both died of multiple cerebral thromboembolisms.

Tricuspid Valve Repair at Pulmonary Valve Replacement in Repaired Tetralogy of Fallot.

BACKGROUND: Pulmonary valve replacement (PVR) is often performed in patients with repaired tetralogy of Fallot (TOF). Concomitant tricuspid valvuloplasty (TVP) in those with tricuspid regurgitation (TR) at the time of PVR is still controversial. METHOD: We retrospectively reviewed clinical records of patients who underwent PVR between 2001 and 2012. We analyzed the impact of concomitant TVP on the tricuspid valve function and right ventricle function and size in mid-term. RESULTS: 119 patients with mild to moderate TR at the time of PVR were enrolled. 33 patients underwent concomitant TVP (TVP group) and 86 patients underwent PVR alone (no-TVP group). There was a significant reduction of TR (p < 0.001) and right ventricular end-diastolic volume index (RVEDVi) (p < 0.001). However, in patients who showed prosthetic pulmonary valve (PV) failure at the last follow-up, there was no significant decrease in TR regardless of concomitant TVP. In the patients with preserved prosthetic PV function, TR was significantly improved (p < 0.001 in both groups). The multivariable analysis showed that significant risk factors for recurrence of significant TR were preoperative moderate TR and prosthetic PV failure. CONCLUSIONS: After PVR in repaired TOF patients, there was an improvement in the degree of TR and the RVEDVi. Concomitant TVP at the time of PVR may not be able to prevent the recurrence of TR when prosthetic PV failure occurs; however, it may effectively preserve tricuspid valve function until that time.

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 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.

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.

Twenty-Year Experience with Truncus Arteriosus Repair: Changes in Risk Factors in the Current Era.

Although the clinical outcomes of truncus arteriosus (TA) repair have been improving, few data are available on long-term outcomes after truncus arteriosus repair in the current era. This study evaluated long-term outcome after repair of TA. Fifty-one patients underwent total correction from April 1982 to June 2018. Since 2003, perioperative strategy has changed to minimal priming volume, modified ultrafiltration, and early total repair (n = 26). Mortality and reoperation rates were analyzed before and after 2003. There were 8 hospital deaths after initial operation, all before 1997. During the mean follow-up of 9.8 years, there were 2 deaths. The Kaplan-Meier estimate of survival among all hospital survivors was 94.7% at 5 years and 88.0% at 20 years. A significant independent risk factor for early mortality was operation before 2003 (Hazard ratio (HR) 9.710, p = 0.041) and REV operation (HR 8.000, p = 0.028). Freedom from reoperation for conduit change and TV repair were 88.3% and 41% at 1 and 5 years, and 96.2% and 85.4% at 1 and 5 years, respectively. After 2003, younger age and conduit choice were risk factors for conduit-related reoperation. Initial preoperative TV regurgitation was independent risk factor for sequential TV repair. Patients with TA can undergo total repair of TA with excellent results, especially in current era. Most of the patients require conduit-related reoperations. Younger age and the methods of RVOT reconstruction were risk factors for conduit-related reoperations. TV repair is necessary in limited patients, and initial regurgitation was a risk factor.

Changes of hospitalization trend in the pediatric cardiology division of a single center by increasing adult with congenital heart disease.

BACKGROUND: As a result of advances in pediatric care and diagnostic testing, there is a growing population of adults with congenital heart disease (ACHD). The purpose of this study was to better define the epidemiology and changes in the trend of hospitalizations for ACHD in Korean society. METHODS: We reviewed outpatient and inpatient data from 2005 to 2017 to identify patient ≥18 years of age admitted for acute care with a congenital heart disease (CHD) diagnosis in the pediatric cardiology division. We tried to analyze changes of hospitalization trend for ACHD. RESULTS: The ratio of outpatients with ACHD increased 286.5%, from 11.1% (1748/15,682) in 2005 to 31.8% (7795/24,532) in 2017. The number of ACHD hospitalizations increased 360.7%, from 8.9% (37/414) in 2005 to 32.1% (226/705) in 2017. The average patient age increased from 24.3 years in 2005 to 27.4 in 2017. The main diagnosis for admission of ACHD is heart failure, arrhythmia and Fontan-related complications. The annual ICU admission percentage was around 5% and mean length of intensive care unit (ICU) stay was 8.4 ± 14.6 days. Mean personal hospital charges by admission of ACHD increased to around two times from 2005 to 2017. (from $2578.1 to $3697.0). Total annual hospital charges by ACHD markedly increased ten times (from $95,389.7 to $831,834.2). CONCLUSIONS: The number of hospital cares for ACHD dramatically increased more than five times from 2005 to 2017. We need preparations for efficient healthcare for adults with CHD such as a multi-dimensional approach, effective communication, and professional training.

Effects of intraoperative dexmedetomidine on the incidence of acute kidney injury in pediatric cardiac surgery patients: A randomized controlled trial.

BACKGROUND: Perioperative dexmedetomidine use has been reported to reduce the incidence of postoperative acute kidney injury after adult cardiac surgery. However, large-scale randomized controlled trials evaluating the effect of dexmedetomidine use on acute kidney injury in pediatric patients are lacking. AIMS: We investigated whether intraoperative dexmedetomidine could reduce the incidence of acute kidney injury in pediatric cardiac surgery patients. METHODS: In total, 141 pediatric patients were randomly assigned to dexmedetomidine or control groups. After anesthetic induction, patients in the dexmedetomidine group were administered 1 µg/kg of dexmedetomidine over 10 minutes and an additional 0.5 µg/kg/h of dexmedetomidine during surgery. Additionally, 1 µg/kg of dexmedetomidine was infused immediately after cardiopulmonary bypass was initiated. The incidence of acute kidney injury was defined following Kidney Disease Improving Global Outcomes guidelines. RESULTS: The final analysis included 139 patients. The incidence of acute kidney injury did not differ between dexmedetomidine and control groups (16.9% vs 23.5%; odds ratio 0.661; 95% CI 0.285 to 1.525; P = .33). Similarly, neither the incidence of abnormal postoperative estimated glomerular filtration rate values (P = .96) nor the incidence of arrhythmia, mechanical ventilation duration, length of stay in the intensive care unit, and hospitalization differed between the two groups. CONCLUSIONS: Intraoperative dexmedetomidine did not reduce acute kidney injury incidence in pediatric cardiac surgery patients.

Outcomes of Supra-annular Mechanical Atrioventricular Valve Replacement with Polytetrafluoroethylene Graft in Infants and Children.

Despite improvements in valve repair techniques, conditions in which infants and children need for mechanical valve replacement (MVR) are still present. We analyzed supra-annular MVR outcomes in infants and children with small annulus and compared them with conventional annular MVR outcomes. Data were collected retrospectively from medical records of infants and children (weighing < 20 kg) who underwent atrioventricular valve replacement with mechanical valve in Seoul National University Children's Hospital between December 1984 and January 2019. We identified 8 patients (median age 20 months, median weight 10.2 kg) who underwent supra-annular MVR with polytetrafluoroethylene graft (supra-annular group). The patients were diagnosed with congenital mitral valve malformation (5 patients), complete atrioventricular septal defect (2 patients), and functional single ventricle (1 patient). The implanted mechanical valve size ranged from 16 to 23 mm. Thirty-three patients (median age 40 months, median weight 13 kg) underwent conventional annular MVR (annular group). The survival rate was not significantly different between the supra-annular and annular groups (75.0 vs 78.8%, P = 0.816). In patients with biventricular repair (7 patients with supra-annular MVR and 28 patients with annular MVR), mechanical valve-to-mitral valve annulus size ratio was higher in the supra-annular group than in the annular group (1.24 ± 0.30 vs 0.96 ± 0.22, P = 0.035). No coronary complication or heart block were observed in the supra-annular group. Supra-annular MVR with polytetrafluoroethylene graft may be a feasible surgical option in children with a small annulus when valve repair is unsuccessful.

Experience with Temporary Centrifugal Pump Bi-ventricular Assist Device for Pediatric Acute Heart Failure: Comparison with ECMO.

Though ventricular assist devices (VADs) are an important treatment option for acute heart failure, an extracorporeal membrane oxygenator (ECMO) is usually used in pediatric patients for several reasons. However, a temporary centrifugal pump-based Bi-VAD might have clinical advantages versus ECMO or implantable VADs. From January 2000 to July 2018, we retrospectively reviewed 36 pediatric patients who required mechanical circulatory support (MCS) for acute heart failure. Cases with postoperative MCS were excluded. Since 2016, we have tried to immediately add a right VAD rather than ECMO, when the patients begin to present features of right heart failure after left VAD support started in cases that the patients' respiratory function did not require an oxygenator. Original diagnoses included dilated cardiomyopathy (n = 18), myocarditis (n = 11), and others (n = 7). Eleven patients were supported by Bi-VAD, and 25 patients were supported by ECMO; of these. Four patients were successfully weaned from VAD, and 10 patients were weaned from ECMO. Eleven patients underwent heart transplantation. Overall, we have 15 (41.7%) early mortalities. There were no significant differences in early mortality, morbidity, and weaning rate between the Bi-VAD group and the ECMO group. During the support, patients with Bi-VADs significantly required fewer platelets and showed less hemolysis than ECMO patients. Patients with myocarditis were successfully weaned from Bi-VAD support and bridged to transplantation thereafter. A temporary centrifugal pump-based Bi-VAD was clinically comparable to ECMO for pediatric patients with acceptable pulmonary function.

Surgery on a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents.

INTRODUCTION: This research investigated patients who underwent surgery for a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents. MATERIALS AND METHODS: The medical records of 11 patients who underwent aortic surgery for dilatation resulting from a connective tissue disease or inflammatory vasculitis between 2000 and 2017 were retrospectively reviewed. RESULTS: The median age and body weight of the patients were 9.6 years (range 5.4 months-15.5 years) and 25.8 kg (range 6.8-81.5), respectively. The associated diseases were Marfan syndrome (n = 3), Loeys-Dietz syndrome (n = 3), Kawasaki disease (n = 1), Takayasu arteritis (n = 1), PHACE syndrome (n = 1), tuberous sclerosis (n = 1), and unknown (n = 1). The most common initially affected area was the ascending aorta. During the 66.4 ± 35.9 months of follow-up, two Marfan syndrome patients died, and four patients (one Marfan syndrome and three Loeys-Dietz syndrome) had repeated aortic operation. Except for one patient, the functional class was well maintained in all patients who were followed up. CONCLUSION: Cases of surgical treatment for a dilated aorta associated with a connective tissue disease and inflammatory vasculitis are rare in children and adolescents at our institution. Most of the patients in this study showed a tolerable postoperative course. However, the aorta showed progressive dilation over time even after surgical treatment, especially in patients with Loeys-Dietz syndrome. In these patients, close and more frequent regular follow-up is required.

Surgical Outcomes of Permanent Epicardial Pacing in Neonates and Young Infants Less Than 1 Year of Age.

BACKGROUND: Open surgical implantation of epicardial leads in neonates and infants remains the first option of treatment. We reviewed the long-term outcomes after epicardial pacemaker implantation in neonates and infants. METHODS: From 1989 to 2016, 48 patients (16 neonates) underwent pacemaker implantation within the first year of life. Their median age and weight were 66.5days (range: 0∼319 days), and 4.2kg (range: 1.9∼9.3kg), respectively, at the time of first pacemaker implantation. The indications for pacemaker implantation were postoperative or congenital atrioventricular block, sinus node dysfunction, and/or myocarditis-induced atrioventricular block. Forty-six (46) unipolar epicardial leads (non-steroid-eluting: 22; steroid-eluting: 24) and two bipolar leads (steroid-eluting) were inserted using a median sternotomy or subxiphoid approach. RESULTS: The mean follow-up duration was 8.5±7.9years. The most commonly used generator mode at first implantation was VVI (n=24, 50.0%). Eleven (11) generator mode changes from the initial VVI or VVIR to dual-chamber pacing were made at a mean of 7.0±6.2years after the first implantation for better inter-chamber synchrony and ventricular function. Freedom from reoperation for generator change after the first implantation was 95.3, 70.6, and 21.9% at 1, 5, and 10 years. Eighteen (18) lead malfunction events (34.1%) were detected. Freedom from reoperation for lead change was 97.8, 76.2, and 46.3% at 1, 5, and 10 years. The lead replacement rate was significantly higher in patients with non-steroid-eluting than steroid-eluting leads (p=0.045). CONCLUSIONS: Neonates and infants require more frequent changes in pacemaker generator and leads than the older population. The use of steroid-eluting leads increased lead longevity and reduced the need for surgical re-interventions.

One-Year Follow-up After Tetralogy of Fallot Total Repair Preserving Pulmonary Valve and Avoiding Right Ventriculotomy.

BACKGROUND: We reviewed our revised surgical strategy for tetralogy of Fallot (TOF) total correction to minimize early exposure to significant pulmonary regurgitation (PR) and to avoid right ventriculotomy (RV-tomy). Methods and Results: Since February 2016, we have tried to preserve, first, pulmonary valve (PV) function to minimize PR by extensive commissurotomy with annulus saving; and second, RV infundibular function by avoiding RV-tomy. With this strategy, we performed total correction for 50 consecutive patients with TOF until May 2018. We reviewed the early outcomes of 27 of 50 patients who received follow-up for ≥3 months. Mean patient age at operation was 10.2±5.0 months, and mean body weight was 8.8±1.2 kg. The preoperative pressure gradient at the RV outflow tract and the PV z-score were improved at most recent echocardiography from 82.0±7.1 to 26.8±6.4 mmHg, and from -2.35±0.49 to -0.55±0.54, respectively, during 11.1±1.6 months of follow-up after operation. One patient required re-intervention for residual pulmonary stenosis. Twenty-two patients had less than moderate PR (none, 1; trivial, 8; mild, 13), and 5 patients had moderate PR. There was no free or severe PR. CONCLUSIONS: At 1-year follow-up, the patients who underwent total TOF correction with our revised surgical strategy had acceptable results in terms of PV function. The preserved PV had a tendency to grow on short-term follow-up.

Potential Role of Transfontanelle Ultrasound for Infants Undergoing Modified Blalock-Taussig Shunt.

OBJECTIVE: Transfontanelle ultrasound is a noninvasive method for assessing cerebral blood flow in neonates and infants. The authors applied this technique as a point-of-care tool, before and after modified Blalock-Taussig shunt procedure, to evaluate cerebral perfusion. DESIGN: Retrospective, observational study. SETTING: Tertiary care children's hospital. PARTICIPANTS: Ten infants undergoing modified Blalock-Taussig shunt procedure. OBSERVATION: Transfontanelle ultrasound examinations with modified resistive index were analyzed before and after the modified Blalock-Taussig shunt procedure. MEASUREMENTS AND MAIN RESULTS: Two infants died and 2 patients required a revision procedure due to shunt thrombosis. Baseline-modified resistive index and regional cerebral oxygenation were comparable between the right and left hemisphere. However, after the procedure, the modified resistive index decreased at both sides of the internal carotid arteries compared with baseline values (p value right side = 0.012, left side = 0.036) and was greater at the ipsilateral internal carotid arteries with the shunt (p = 0.012, mean difference = 0.07, 95% confidence interval [CI] 0.02-0.12). Four infants with the large patent ductus arteriosus presented diastolic reverse flows at both internal carotid arteries at baseline. However, the diastolic reverse flow disappeared after the procedure. An infant who developed diastolic reverse flow after the procedure, died. CONCLUSIONS: Transfontanelle ultrasound is a feasible tool for assessing the pattern of shunt flow and cerebral perfusion before and after the modified Blalock-Taussig shunt procedure. The transfontanelle ultrasound examinations may have potential role in assessing "over-shunting," but it needs more studies.