Treatment of Primary Cardiac Tumors in Children: A Single Center 18-Year Experience.

This study aimed to compare the clinical characteristics and courses of pediatric patients with cardiac tumors in nonoperative and operative groups to help guide treatment decisions. We reviewed the medical records of patients diagnosed with primary pediatric cardiac tumors at our institution between 2003 and 2020. Demographic data, clinical characteristics, and follow-up data between the operation and nonoperation groups were compared. A total of 56 patients were included in the study. Thirteen patients underwent surgery. The median age was 1.4 months (range, 1 to 18 years). The patients in the operation group had more frequent symptoms or signs, such as desaturation, respiratory difficulty, murmur, a higher mass area/chamber area (MC) ratio, decreased ventricular contractility, and significant ventricular outflow tract obstruction (VOTO). An MC ratio of 0.568 was the cutoff value for differentiating patients with symptoms or signs of heart failure and decreased ventricular contractility. At the last follow-up, all patients had good ventricular contractility except one patient in the operative group with fibroma. In the non-operative group, rhabdomyomas often regressed spontaneously, while fibromas often increased in size. Two patients in the nonoperative group died. In the operative group, there was no early or late mortality or tumor recurrence. In this study, patients had good outcomes with or without surgery, even when the tumor was large, or surgery was performed in early infancy.

Surgical Repair of a Sinus of Valsalva Aneurysm: A 22-Year Single-Center Experience.

BACKGROUND: Several reports described the repair of sinus of Valsalva aneurysms (SVAs); however, there is still debate regarding the optimal method of operation. We investigated the determinants of the development of significant aortic regurgitation (AR) and long-term survival after surgical repair. METHODS: Between January 1995 and December 2016, 71 patients (31 females; median age: 33.3 years) underwent surgical SVA repair with (n = 60) or without (n = 11) rupture. Aortic valvuloplasty (AVP) was performed using Trusler's technique in 28 patients (39.4%), and 11 patients (15.5%) underwent aortic valve replacement during the first operation. RESULTS: There was no early mortality, and three deaths occurred during follow-up (median: 65.4 months). Patients with grade II preoperative AR who underwent AVP tended to develop significant postoperative AR, but freedom from significant AR did not differ statistically (p = 0.387). Among patients who underwent AVP, freedom from significant AR did not differ statistically between those with grades I and II and those with grades III and IV (p = 0.460). CONCLUSION: Surgical repair of SVA with or without rupture can be performed safely using the dual approach technique. Concomitant aortic valve repair can be performed without difficulty and should be recommended not only for patients with moderate or severe preoperative AR (grades III and IV) but also for those with minimal or mild preoperative AR (grades I and II), whose aortic valve geometry needs correction.

Surgical Outcomes for Obstructive Total Anomalous Pulmonary Venous Return of the Non-Infracardiac Type Compared with the Infracardiac Type.

BACKGROUND: Infracardiac obstructive total anomalous pulmonary venous return (TAPVR) has a poor outcome following surgical correction. We compared the surgical outcomes of obstructive TAPVR between non-infracardiac and infracardiac types. METHODS: Among 51 patients who underwent surgical repair for obstructive TAPVR, 23 with infracardiac type and 28 with non-infracardiac type were included in this investigation. The study compared the immediate postoperative courses in the intensive care unit and long-term mortality and pulmonary vein stenosis. The risk factors for long-term survival in obstructive TAPVR also were investigated. RESULTS: The postoperative follow-up period was 79.8 ± 81.5 months. Immediate major operative complications were observed in 22 patients (43.1%); 10 patients (19.6%) died, and eight patients (15.7%) experienced pulmonary vein stenosis during the follow-up period. The Kaplan-Meier curve showed better cumulative survival in patients with infracardiac TAPVR (P = 0.308). The significant factors for survival after surgical repair of obstructive TAPVR did not include anatomical type but instead were postoperative course of ventilator care and lengths of intensive care unit and hospital stays. CONCLUSION: Patients with non-infracardiac TAPVR with obstruction had a longer postoperative course and experienced more complications. Their survival rate was poorer, and postoperative pulmonary vein stenosis was more frequent in those patients compared with infracardiac TAPVR patients. However, a large-scale study is mandatory to gather more data and confirm our findings.

The progression of an acceptable pulmonary stenosis immediately after total correction of tetralogy of Fallot.

BACKGROUND: A certain degree of pulmonary stenosis after total correction of tetralogy of Fallot has been considered acceptable. But the long-term outcomes are not well understood. We observed the natural course of immediate pulmonary stenosis and investigated related factors for progression. METHODS: Fifty-two patients with acceptable pulmonary stenosis immediately after operation were enrolled. Acceptable pulmonary stenosis was defined as peak pressure gradient between 15 and 45 mmHg by Doppler echocardiography. Latent class linear mixed model was used to differentiate patients with progressed pulmonary stenosis, and the factors related to progression were analysed. RESULTS: Pulmonary stenosis progressed in 14 patients (27%). Between the progression group and no progression group, there were no significant differences in operative age, sex, and the use of the transannular patch technique. However, immediate gradient was higher in the progression group (32.1 mmHg versus 25.7 mmHg, p = 0.009), and the cut-off value was 26.8 mmHg (sensitivity = 65.3%, specificity = 65.8%). Main stenosis at the sub-valve was observed more frequently in the progression group (85.7% versus 52.6%, p = 0.027). Despite no difference in the preoperative pulmonary valve z value, the last follow-up pulmonary valve z value was significantly lower in the progression group (-1.15 versus 0.35, p = 0.002). CONCLUSIONS: Pulmonary stenosis immediately after tetralogy of Fallot total correction might progress in patients with immediate pulmonary stenosis higher than ≥26.8 mmHg and the main site was sub-valve area.

Repair of tricuspid valve with pulmonary valve replacement in repaired tetralogy of fallot.

Objectives: We aimed to investigate the outcome of tricuspid valve repair (TVR) performed concomitantly with pulmonary valve replacement in repaired tetralogy of Fallot (TOF) patients. Design: This retrospective study included all patients who underwent pulmonary vale replacement from 2000 to 2016 after TOF correction. TVR patient data were compared to those of patients who underwent pulmonary vale replacement alone. Results: Thirty-eight patients were enrolled. The degree of tricuspid regurgitation was significantly decreased after operation in the TVR group. Tricuspid valve annulus and annuloectasia before operation did not vary between groups (21.1 ± 6.3 and 41.4% in no TVR vs. 21.3 ± 4.8 and 52.6% in TVR). However pre-operative right ventricular volumes were larger in the TVR group. Normal tricuspid valve coaptation (body to body) was observed less frequently in the TVR group than in the other group (52.6% vs. 93.1%, p < .001). Pre-operative tricuspid regurgitation had a linear correlation with right ventricular volume, but not with tricuspid annulus size. Conclusion: Tricuspid annulus diameter decreased significantly regardless of TVR. Abnormal coaptations were observed more in patients group and the degree of pre-operative tricuspid regurgitation was linearly correlated with right ventricular volume rather than tricuspid annulus size.

The Effect of Multidisciplinary Approach on the Birth Rate of Fetuses with Prenatally Diagnosed Congenital Heart Disease.

BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.

Ultrasound-guided vs. palpation-guided techniques for radial arterial catheterisation in infants: A randomised controlled trial.

BACKGROUND: The usefulness of ultrasound-guided techniques for radial arterial catheterisation has been well identified; however, its usefulness has not been completely evaluated in infants under 12 months of age, who are generally considered the most difficult group for arterial catheterisation. OBJECTIVE: We evaluated whether ultrasound guidance would improve success rates and reduce the number of attempts at radial arterial catheterisation in infants. DESIGN: A randomised, controlled and patient-blinded study. SETTING: Single-centre trial, study period from June 2016 to February 2017. PATIENTS: Seventy-four infants undergoing elective cardiac surgery. INTERVENTION: Patients were allocated randomly into either ultrasound-guided group (group US) or palpation-guided group (group P) (each n=37) according to the technique applied for radial arterial catheterisation. All arterial catheterisations were performed by one of two experienced anaesthesiologists based on group assignment and were recorded on video. MAIN OUTCOME MEASURES: The primary endpoint was the first-pass success. The number of attempts and total duration of the procedure until successful catheterisation were also analysed. RESULTS: The first-pass success rate was significantly higher in the group US than in the group P (68 vs. 38%, P = 0.019). In addition, fewer attempts were needed for successful catheterisation in the group US than in the group P (median 1 [IQR 1 to 2] vs. 2 [1 to 4], P = 0.023). However, the median [IQR] procedural time (s) until successful catheterisation in the two groups was not significantly different (102 [49 to 394] vs. 218 [73 to 600], P = 0.054). CONCLUSION: The current study demonstrated that the ultrasound-guided technique for radial arterial catheterisation in infants effectively improved first-pass success rate and also reduced the number of attempts required. TRIAL REGISTRATION: ClinicalTrials.gov NCT02795468.

Imaging Assessment of Hepatic Changes after Fontan Surgery.

The aim of this study was to evaluate hepatic dysfunction over 10 years following Fontan surgery. We assessed the clinical usefulness of diagnostic tools for the detection and follow-up of hepatic dysfunction in patients with Fontan circulation.A total of 26 post-Fontan patients (median age 13 years, range 10-35 years; median duration from Fontan procedure 10.5 years, range 4-17 years) were enrolled in this study. Hepatic assessment was performed by ultrasonography, computed tomography (CT), and transient elastography (TE) with biochemical tests, echocardiography, and cardiac catheterization. Related parameters were compared on the basis of different findings in liver sonography, CT, and TE.Liver CT and TE showed abnormal findings in all patients. Liver ultrasonography revealed abnormal results in 24 patients (92.3%). However, liver function test was normal and did not correlate with imaging studies. C-reactive protein was significantly correlated with severity of CT findings. White blood cell, platelet count, and N-terminal pro-brain natriuretic peptide were correlated with severity on TE. Post-Fontan high pulmonary vascular resistance (P = 0.046) and high mean pulmonary artery pressure (P = 0.046) correlated with hepatic changes on liver CT.Changes in the liver post-Fontan surgery are common and occur even after 10 years the procedure. Liver imaging is more sensitive, and CT seems to be more useful for differentiation of severe hepatic changes.

The Center for Epidemiologic Studies Depression Scale is an adequate screening instrument for depression and anxiety disorder in adults with congential heart disease.

BACKGROUND: The Center for Epidemiological Studies Depression Scale (CES-D) is an instrument that is commonly used to screen for depression in patients with chronic disease, but the characteristics of the CES-D in adults with congenital heart disease (CHD) have not yet been studied. The aim of this study was to investigate the criterion validities and the predictive powers of the CES-D for depression and anxiety disorders in adults with CHD. METHODS: Two hundred patients were screened with the CES-D and secondarily interviewed with a diagnostic instrument, i.e., the Mini International Neuropsychiatric Instrument. The sensitivity and specificity values of the CES-D were calculated by cross-tabulation at different cutoff scores. Receiver operating characteristic (ROC) curves were used to assess the optimal cutoff point for each disorder and to assess the predictive power of the instrument. RESULTS: The CES-D exhibited satisfactory criterion validities for depression and for all combinations of depression and/or anxiety. With a desired sensitivity of at least 80%, the optimal cutoff scores were 18. The predictive power of the CES-D in the patients was best for major depression and dysthymia (area under the ROC curve: 0.92) followed by the score for any combination of depression and/or anxiety (0.88). CONCLUSION: The use of CES-D to simultaneously screen for both depression and anxiety disorders may be useful in adults with CHD. TRIAL REGISTRATION: CESDEP 212. Registered 2 March 2014 (retrospectively registered).

Predictive Value of Intraoperative Thromboelastometry for the Risk of Perioperative Excessive Blood Loss in Infants and Children Undergoing Congenital Cardiac Surgery: A Retrospective Analysis.

OBJECTIVE: Laboratory hemostatic variables and parameters of rotational thromboelastometry (ROTEM) were evaluated for their ability to predict perioperative excessive blood loss (PEBL) after congenital cardiac surgery. DESIGN: Retrospective and observational. SETTING: Single, large university hospital. PARTICIPANTS: The study comprised 119 children younger than 10 years old undergoing congenital cardiac surgery with cardiopulmonary bypass (CPB). MEASUREMENTS AND MAIN RESULTS: Intraoperative excessive blood loss was defined as estimated blood loss≥50% of estimated blood volume (EBV). Postoperative excessive blood loss was defined as measured postoperative chest tube and Jackson-Pratt drainage≥30% of EBV over 12 hours or≥50% of EBV over 24 hours in the intensive care unit. PEBL was defined as either intraoperative or postoperative excessive blood loss. External temogram (EXTEM) and fibrinogen temogram (FIBTEM) were analyzed before and after CPB with ROTEM and laboratory hemostatic variables. Multivariate logistic regression was performed. Incidence of PEBL was 19.3% (n = 23). Independent risk factors for PEBL were CPB time>120 minutes, post-CPB FIBTEM alpha-angle, clot firmness after 10 minutes<5 mm, post-CPB EXTEM alpha-angle, clot firmness after 10 minutes<30 mm, and post-CPB EXTEM maximal lysis>20%. Laboratory hemostatic variables were not significant in multivariate analysis. The risk prediction model was developed from the results of multivariate analysis. The area under the receiver operating characteristic curve was 0.94 (95% confidence interval: 0.90-0.99). CONCLUSIONS: Post-CPB ROTEM may be useful for predicting both intraoperative and postoperative excessive blood loss in congenital cardiac surgery. This study provided an accurate prediction model for PEBL and supported intraoperative transfusion guidance using post-CPB FIBTEM-A10 and EXTEM-A10.

Natural Course of Adult Ebstein Anomaly When Treated according to Current Recommendation.

The objectives of this study were to assess the clinical outcomes of adults with Ebstein Anomaly (EA) according to their treatment modalities. All adult EA patients diagnosed between October 1994 and October 2014 were retrospectively evaluated by medical record review. Total 60 patients were categorized into 3 groups according to their treatment strategy, i.e. non-operative treatment (Group I, n = 23), immediate operative treatment (Group II, n = 27), and delayed operative treatment (Group III, n = 10). A composite of major adverse cardiac and cerebrovascular events (MACCE) and factors associated with MACCE were assessed in each treatment group. MACCE occurred in 13.0% patients in Group I, 55.6% patients in Group II and 50% in Group III (P = 0.006). Event free survivals at 5 years were 90% in Group I, 52.7% in Group II, 50.0% in Group III (P = 0.036). Post-operatively, most patients showed improvement on clinical symptoms. However, event free survival rate was lower in patients with operation compared to those with non-operative treatment (58.7% vs. 90.9%; P = 0.007). Major arrhythmic event occurred more frequently even after surgical ablation (50.0% vs. 20.0%; P = 0.034). Re-operation was more frequent in patients underwent delayed surgery compared to those with immediate surgery (50.0% vs. 18.5%; P = 0.001). Current guideline to decide patient's treatment strategy appeared to be appropriate in adult patients with EA. However, surgical ablation for arrhythmia was not enough so that concomitant medical treatment should be considered. Therefore, attentive risk stratification and cautious decision of treatment strategy by experienced cardiac surgeon are believed to improve clinical outcome.

Stroke is not a treatment dilemma for early valve surgery in active infective endocarditis.

BACKGROUND AND AIM OF THE STUDY: While early valve surgery for active infective endocarditis (AIE) is recommended, surgeons have hesitated to operate on patients complicated by cerebral septic embolism resulting in cerebral bleeding when cardiopulmonary bypass is required intraoperatively. The study aim was to review the outcomes of operations for AIE, and to determine the risks of neurologic complications resulting from cerebral septic embolism. METHODS: Between 1994 and June 2011, among 278 patients who underwent heart valve surgery for AIE at the authors' institution, 39 (14%) had cerebral septic embolisms. Cerebral lesions were verified by imaging, and were predominantly multiple embolic infarctions (34 patients; 87.2%). Five patients had brain abscess, and 10 had hemorrhage with or without infarction. The mean interval between the recent onset of a stroke and surgery was 10.1 ± 10.1 days (range: 0-43 days). RESULTS: One patient died postoperatively of septic shock. New neurologic complications occurred in five patients, including secondary hemorrhagic transformation in the previous lesions (n = 2), newly developed subdural and subarachnoid hemorrhage (n = 2), and an increased degree of subarachnoid hemorrhage (n = 1). One patient needed a craniotomy, and the others were treated medically. There were five late deaths, including one cardiac death, and one redo valve surgery due to repaired valve failure during the follow up period of 46.3 ± 40.4 months (range: 1.9-127.4 months). The overall and event-free survival rates at five and 10 years were 84.3 ± 6.5% and 75.9 ± 9.9%, and 81.7 ± 6.8% and 73.6 ± 9.9%, respectively. CONCLUSION: Surgery for AIE with cerebral septic embolisms can be performed safely, with good early and mid-term follow-up results. When urgent or emergent surgery for AIE is needed, neurologic complications should not be a reason for delay.

Long-Term Outcomes of Modified Cone Reconstruction for Ebstein's Anomaly in Pediatric Patients in a Single Center.

BACKGROUND AND OBJECTIVE: We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein's anomaly. METHODS: This was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein's anomaly at a single tertiary center between January 2005 and June 2021. RESULTS: A total of 14 pediatric patients underwent modified cone reconstruction for Ebstein's anomaly; the median age was 5.8 years (range, 0.01-16.6). There were three patients (21.4%) with Carpentier type B, ten patients with Carpentier type C (71.4%), and one patient with Carpentier type D (7.1%). There was no early or late mortality, arrhythmia, or readmission for heart failure at a 10-year follow-up. There were no cases of more than mild tricuspid stenosis or more than moderate tricuspid regurgitation during the study period, except for one patient with severe tricuspid regurgitation who underwent reoperation. The z value for tricuspid valve annular size significantly decreased immediately after the operation (2.46 vs. -1.15, p<0.001). However, from 1 year to 7 years after surgery, the z values were maintained between -1 and +1. Left ventricular end-systolic volume, end-diastolic volume, and stroke volume increased after surgery and remained elevated until seven years postoperatively. CONCLUSIONS: Ebstein's anomaly in children can be repaired by modified cone reconstruction with low mortality and morbidity, good tricuspid valve durability, and annular growth relative to somatic growth.

Impact of preoperative respiratory distress on outcomes of slide tracheoplasty.

OBJECTIVES: Children with congenital tracheal stenosis born in the developing world face a high risk of mortality due to limited access to proper treatment. Patients who required preoperative respiratory support were suspected to have poor survival after slide tracheoplasty; however, this was not clearly demonstrated in the previous studies. This study aims to investigate the impact of preoperative respiratory conditions on outcomes of slide tracheoplasty. METHODS: From 2016 to 2022, children who underwent slide tracheoplasty were retrospectively reviewed. Patients with respiratory distress requiring emergency operations (group A) were compared with patients in stable condition who were scheduled for surgery (group B). RESULTS: Perioperative results revealed that group A (n = 43) had a longer bypass time (P < 0.001), operation time (P = 0.01), postoperative ventilation time (P < 0.001) and length of intensive care unit stay (P = 0.00125) than group B (n = 60). The early mortality rate was 7.8%, and the actuarial 5-year survival rate was 85.3%. The cumulative incidence test revealed that group A was highly significant for overall mortality [sudistribution (SHR) 4.5; 95% confidence interval (CI) 1.23-16.4; P = 0.023]. Risk factors for overall mortality were prolonged postoperative ventilation time (hazard ratio 3.86; 95% CI 1.20-12.48; P = 0.024), bronchial stenosis (hazard ratio 5.77; 95% CI 1.72-19.31; P = 0.004), and preoperative tracheal mucositis (hazard ratio 5.67; 95% CI 1.51-21.31; P = 0.01). Four patients needed reintervention during a follow-up of 28.4 months (interquartile range 15.3-47.3). CONCLUSIONS: Preoperative respiratory distress negatively affected the outcomes of patients who required slide tracheoplasty. Therefore, early detection of congenital tracheal stenosis and aggressive slide tracheoplasty are crucial and obligatory to enhance long-term survival in this lethal congenital airway disease.

Long-term hemodynamic performance of ATS valves in the aortic position: impact on the progression of late tricuspid regurgitation.

BACKGROUND AND AIM OF THE STUDY: The study aim was to evaluate the long-term hemodynamic performance of ATS bileaflet valves in the aortic position. METHODS: A total of 132 patients (mean age 54 +/- 13 years) who underwent aortic valve replacement (AVR) using ATS valves between October 1994 and May 2001 was analyzed. Of these patients, 77 underwent isolated AVR (AVR group), while 55 underwent concomitant mitral valve replacement (DVR group). The mean follow up duration was 135 +/- 39 months (maximum 180 months). RESULTS: The overall mortality was 19.7% (26/132), but no early mortality occurred. Cumulative survival rates at 12 years were 87.0 +/- 3.8% in the AVR group and 71.4 +/- 6.3% in the DVR group (p = 0.093). The AVR group showed a higher cardiovascular event-free survival at 12 years than the DVR group (82.3 +/- 4.7% versus 65.1 +/- 7.3%; p = 0.047). During follow up, the transaortic mean pressure gradient (TMPG) was maintained in the AVR group but increased in the DVR group (p = 0.044). The TMPG at follow up was higher in patients who underwent AVR with small valves (< or =21 mm) than in those receiving large valves (p = 0.001). The tricuspid regurgitation (TR) grade was increased over time in both groups, and was greater in the DVR group (2.1 +/- 1.1 versus 1.4 +/- 1.0; p < 0.001). The progression of late TR correlated with the TMPG (gamma = 0.261, p = 0.022). CONCLUSION: The study results showed that ATS valves had acceptable long-term outcomes. A high TMPG was observed more frequently in the DVR group and in patients who underwent AVR with a small valve. A high TMPG might be related to the progression of late TR.

Valve-sparing root replacement in children with connective tissue disease: Long-term risk of aortic events.

BACKGROUND: Cardiovascular involvement in pediatric patients with connective tissue disease (CTD) is life-threatening, with aortic root dilatation being the most prevalent cardiovascular abnormality. We attempted to determine long-term outcomes of valve-sparing root replacement (VSRR) in this group, including cardiovascular reoperations for aortic aneurysm and dissection. METHODS: We conducted a retrospective analysis of pediatric patients with CTD who received VSRR in a single center from 2002 to 2021. The primary end point was a composite event of all-cause death and cardiovascular reoperations. The median follow-up duration was 8.3 years, with a maximum of 20.7 years. RESULTS: The median age of 24 pediatric patients who had VSRR was 14.4 years. Marfan syndrome and Loeys-Dietz syndrome affected 19 (79.2%) and 5 (20.8%) patients, respectively. There was no early death. The 15-year survival rate was 91.7%. At 10 years after VSRR, the cumulative incidence of reoperation for aortic regurgitation was 15.6%, and for aortic aneurysm or dissection, it was 29.1%. The 10-year rate of freedom from the primary end point was 53.1%. The Cox multivariable analysis revealed younger age at surgery (hazard ratio, 1.279; 95% confidence interval, 1.086-1.505; P = .003) and VSRR before 13 years of age (hazard ratio, 5.005; 95% confidence interval, 1.146-21.850; P = .032) as independent prognostic factors for the primary endpoint. CONCLUSIONS: VSRR for aortic root dilatation in pediatric patients with CTD demonstrated good long-term survival and low reoperation rates for aortic regurgitation. However, several patients developed later aortic aneurysm or dissection, and careful surveillance may be required, particularly in those who received VSRR at younger age.

Surgical strategy in patients with atrial septal defect and severe pulmonary hypertension.

The aim of the study was to review our experience with atrial septal defect (ASD) closure with a fenestrated patch in patients with severe pulmonary hypertension. Between July 2004 and February 2009, 16 patients with isolated ASD underwent closure with a fenestrated patch. All patients had a secundum type ASD and severe pulmonary hypertension. Patients ranged in age from 6 to 57 years (mean ± SD, 34.9 ± 13.5 years). The follow-up period was 9 to 59 months (mean, 34.5 ± 13.1 months). The ranges of preoperative systolic and pulmonary arterial pressures were 63 to 119 mm Hg (mean, 83.8 ± 13.9 mm Hg) and 37 to 77 mm Hg (mean, 51.1 ± 10.1 mm Hg). The ranges of preoperative values for the ratio of the pulmonary flow to the systemic flow and for pulmonary arterial resistance were 1.1 to 2.7 (mean, 1.95 ± 0.5) and 3.9 to 16.7 Wood units (mean, 9.8 ± 2.9 Wood units), respectively. There was no early or late mortality. Tricuspid annuloplasty was performed in 14 patients (87.5%). The peak tricuspid regurgitation gradient and the ratio of the systolic pulmonary artery pressure to the systemic arterial pressure were decreased in all patients. The New York Heart Association class and the grade of tricuspid regurgitation were improved in 13 patients (81.2%) and 15 patients (93.7%), respectively. ASD closure in patients with severe pulmonary hypertension can be performed safely if we create fenestration. Tricuspid annuloplasty and a Cox maze procedure may improve the clinical result. Close observation and follow-up will be needed to validate the long-term benefits.

Midterm results of arch augmentation with autologous vascular patch in interrupted aortic arch.

OBJECTIVES: Aortic arch reconstruction of interrupted aortic arch remains challenging, and subsequent problems, including arch and airway stenosis, may occur. Thus, we investigated midterm results of an augmentation technique using autologous vascular patch. METHODS: This retrospective study included 24 patients who underwent arch reconstruction with an autologous vascular patch for interrupted aortic arch with biventricular physiology from 2006 to 2018. The median age and body weight at operation were 10 days (range 4-77 days) and 3 kg (range 2.5-5.1 kg), respectively. The reconstructed arch was supplemented in the lesser curvature with an autologous vascular patch that was harvested from main pulmonary artery (n = 19), left subclavian artery (n = 3) or aberrant right subclavian artery (n = 1). One patient used patches from both the main pulmonary and left subclavian artery. RESULTS: There was 1 early death due to right heart failure. All survivors were discharged 15 days (range 9-58 days) after surgery without residual arch stenosis. Late death occurred in 1 patient with Cri-du-chat syndrome and airway stenosis. Two reoperations and 1 intervention for arch stenosis were performed. The 1-, 5- and 10-year survival was 92%. Freedom from reoperation or intervention for arch stenosis was 86% 1, 5 and 10 years after surgery. No occurrence of arch aneurysm formation, left main bronchial stenosis and significant hypertension was found during a median follow-up period of 5.5 years (range 0.3-13.3 years). CONCLUSIONS: Augmenting the lesser curvature with an autologous vascular patch during arch reconstruction resulted in reasonable midterm outcomes.

Long-Term Observational Outcomes after Total Correction of Congenital Heart Disease in Korean Patients with Down Syndrome: A National Cohort Study.

Background: In the present study, the population prevalence and postoperative morbidity and mortality in Down syndrome patients who underwent total correction for congenital heart disease were investigated using data from a large national cohort. Methods: Retrospective administrative data from 2,395,966 participants born between 2008 and 2012 were acquired from the National Investigation of Birth Cohort in Korea. Among Down syndrome patients, 58.3% had congenital heart disease and 32.3% underwent total correction. Propensity score matching (maximum 1:1) and stabilized inverse probability treatment weighting (IPTW) were performed for each group (153 Down syndrome patients and 4482 non-Down syndrome patients). Results: T late mortality rate was significantly higher in the Down syndrome group than in the non-Down syndrome group (8.1% vs. 3.8%). No differences were observed in postoperative heart failure and arrhythmias, but pulmonary hypertension was significantly greater in the Down syndrome group than in the non-Down syndrome group (26.9% vs. 7.0%). The length of hospitalization was longer in the Down syndrome group than in the non-Down syndrome group (14 days vs. 11 days; interquartile range (IQR): 10−25 vs. 6−19; p < 0.0001). After total correction, readmission frequency for any reason was minimally but statistically significantly higher in the Down syndrome group compared to the non-Down syndrome group (5 times vs. 5 times; IQR: 3−8 vs. 4−9; p < 0.0001). However, the number of emergency room visits was minimally but significantly lower in the Down syndrome group compared to the non-Down syndrome group (2 visits vs. 2 visits (IQR): 2−7 vs. 1−4; p = 0.016). Conclusions: Down syndrome patients with congenital heart disease undergoing total correction showed pulmonary hypertension after surgery, longer length of hospitalization, frequent hospitalization after surgery, and a higher rate of late mortality.

Long-term echocardiographic follow-up after posterior mitral annuloplasty using a vascular strip for ischemic mitral regurgitation: ten-years of experience at a single center.

Management of ischemic mitral regurgitation (MR) is challenging. The aim of this study was to investigate long-term clinical and echocardiographic results of restrictive mitral annuloplasty for ischemic MR. From 2001 through 2010, 96 patients who underwent myocardial revascularization with restrictive mitral annuloplasty using a vascular strip for ischemic MR were analyzed. Patients were stratified into two groups based on left ventricular ejection fraction (LVEF): group I, n = 50, with LVEF > 35% and group II, n = 46, with LVEF ≤ 35%. The early mortality rate was 2.1% (2/96) and the late cardiac mortality rate was 11.5% (11/96). MR grade was reduced at discharge (0.8 ± 0.7) but increased during follow-up (1.1 ± 0.8, P = 0.001). There was no intergroup difference in terms of freedom from recurrent MR ≥ moderate eight years after surgery (94.1% ± 5.7%, group I vs 87.8% ± 7.2%, group II; P = 0.575). NYHA functional class (odds ratio [OR], 2.2; P = 0.044) and early postoperative residual MR ≥ mild (OR, 25.4; P < 0.001) were independent predictors of recurrent MR. Restrictive mitral annuloplasty using a vascular strip is effective in ischemic MR. It is important to avoid early postoperative residual MR.