World journal for pediatric & congenital heart surgery最新文献

Pulmonary atresia with intact ventricular septum (PA-IVS) and myocardial dysfunction is a challenging entity to manage. Presented is a patient with PA-IVS who developed myocardial dysfunction and heart failure following an episode of periprocedural hypotension. He was successfully treated with a single ventricle assist device (SVAD) placed without cardiopulmonary bypass and a ductal stent as a bridge to donation after circulatory death heart transplant. This report describes the technique of SVAD placement without cardiopulmonary bypass.

Coral reef aorta (CRA) is a rare, calcified aortic lesion observed primarily in adults and elderly patients. This condition can lead to resistant hypertension and impaired organ perfusion. Although surgical treatment remains the predominant approach, endovascular intervention is emerging as an alternative for suitable cases. We present a 16-year-old male patient with a history of kidney transplantation who was admitted due to hypertension. Imaging revealed severe stenosis at the isthmus level caused by a calcific lesion characteristic of CRA. Given the complexity of the lesion and the surgical challenges, an endovascular approach was chosen. A covered stent was successfully implanted, resulting in significant hemodynamic improvement. At the sixth month follow-up, the patient remained normotensive, and antihypertensive medications were discontinued. To our knowledge, this case represents the first pediatric patient reported to have undergone endovascular treatment for severe aortic coarctation associated with CRA. Our findings suggest that endovascular intervention using a covered stent may provide a viable alternative to surgery in selected pediatric cases, minimizing morbidity and mortality. Further studies are needed to assess the long-term outcomes of this approach.

Background: We aimed to evaluate the clinical performance of the Inspiris Resilia valve in the pulmonic position. Methods: This study includes adult patients with congenital heart disease (CHD) who underwent pulmonary valve replacement (PVR) using the Inspiris Resilia or Carpentier-Edwards (CE) valve at Kyushu University Hospital from 2004 to 2023, as well as adult patients who underwent aortic valve replacement (AVR) using the Inspiris Resilia valve from 2018 to 2023. We compared the outcomes of the Inspiris Resilia and CE valves at the pulmonic position, and the outcomes of the Inspiris Resilia valves in the pulmonic and aortic position. The primary endpoint was freedom from mild valve regurgitation. Results: There was no significant difference in the freedom from at least moderate pulmonary regurgitation (PR) or stenosis between the Inspiris Resilia and CE valves (P = .65). However, the progression to mild PR in the Inspiris Resilia valve occurred significantly earlier (P = .03), particularly when implanted in the native right ventricular outflow tract (RVOT) (P = .01). The freedom from at least mild aortic regurgitation following AVR with or without using a composite valve graft showed no significant difference (P = .92). Conclusions: This study showed good results for PVR with the Inspiris Resilia valve in adult patients with CHD, but also early onset of mild PR after PVR raising concerns about the long-term durability of the Inspiris Resilia valve, especially when it is implanted in the native RVOT. We recommend that patients who have undergone PVR with the Inspiris Resilia valve in the native RVOT be closely monitored.

Background: This study aimed to compare the long-term outcomes of a modified réparation à l'étage ventriculaire (REV) and the Rastelli repair for ventricular septal defect (VSD) and pulmonary outflow tract obstruction without ventriculoarterial concordance. Methods: The study included 100 consecutive patients who underwent a modified REV (n = 50) or Rastelli repair (n = 50) for transposition of the great arteries, double outlet right ventricle, or double outlet left ventricle with VSD and pulmonary outflow tract obstruction. The mean ages of the patients who underwent the modified REV and Rastelli repair were 2.50 years (range: 0.30-12.48) and 5.91 years (range: 0.36-46.15), respectively (P < .05). Results: Actuarial survival and arrhythmia-free survival rates were 83.4% ± 4.0% (P > .05) and 72.4% ± 5.0%, respectively at 37.25 years (P > .05). The reoperation-free survival was 18.7% ± 4.6% at 31.82 years (P < .05). The freedom from the first, second, third, and fourth reoperations after the modified REV versus the Rastelli repair was 35.9% ± 8.3% at 29.8 years versus 6.7% ± 4.3% at 31.8 years (P < .05); 83.2%±6.3% at 29.8 years versus 21.2% ± 15.6% at 35.8 years (P < .05); 94.1% ± 4.1% at 29.8 years versus 56.1% ± 10.9% at 37.3 years (P < .05); and 97.0% ± 3.0% at 29.8 years versus 74.3% ± 10.2% at 37.3 years (P < .05), respectively. The most common causes of reoperation after the Rastelli repair were pulmonary stenosis, left ventricular outflow tract obstruction, and arrhythmia. However, the most common cause of reoperation after the modified REV was pulmonary regurgitation. Conclusion: Modified REV and Rastelli repair have shown satisfactory results in long-term follow-up. However, the Rastelli repair could not avoid repeated reoperations, especially for biventricular outflow tract obstruction and arrhythmia.

Objectives: This study aimed to report the incidence of cardiac arrest and in-hospital mortality after pediatric congenital heart surgery in a middle-income country. Methods: This retrospective cohort study was conducted in Thailand. Patients <18 years of age who underwent congenital heart surgery between 2014 and 2019 and experienced cardiac arrest following surgery during the same hospital stay were included. We examined the characteristics of patients who experienced cardiac arrest and its management to determine the mortality-associated factors following cardiac arrest. Results: Overall, 116 cases of cardiac arrest following 1,928 congenital heart surgery operations were included, which resulted in 93/116 (80%) deaths. The incidence of cardiac arrest and in-hospital mortality per 100 patients (95% confidence interval) were 6.0% [116/1,928 (5.0%-7.2%)] and 4.8% [93/1,928 (4.0%-5.9%)], respectively. The incidence of cardiac arrest was higher in neonates (33.6%, 47/140), high-risk STAT 5 surgery (54.8%, 23/43), and emergent/urgent surgery (25.4%, 81/319). Most cardiac arrests occurred within 24 h (66/116, 57%) and in the intensive care unit (90/116, 78%). The most common cause of cardiac arrest was cardiovascular-related (74.1%, 86/116). Multivariable analysis showed the factors associated with mortality (adjusted odds ratio, [95% confidence interval]) included cardiac arrest after 72 h (5.594 [1.073-29.167]), multiple cardiac arrests (10.231 [1.884-55.566]), and every minute increase in cardiopulmonary resuscitation (1.027 [1.005-1.048]). Conclusions: Congenital heart surgery at our middle income cardiac surgical center was associated with relatively high incidence rates of cardiac arrest and in-hospital mortality, and a very high mortality rate following cardiac arrest. The mortality-associated factors after cardiac arrest were cardiac arrest after 72 h, multiple cardiac arrests, and longer duration of cardiopulmonary resuscitation.

Background: In adult patients with transposition of the great arteries (TGA) and systemic right ventricle (sRV), the use of ventricular assist devices (VADs) is uncommon. Methods: We conducted a systematic review of published studies to examine the indications, hemodynamic effects, and outcomes of VADs in this patient population. We reviewed English-language literature for case reports, case series, and reviews that included individual patient data, such as demographics, hemodynamic parameters, types of implanted VADs, and outcomes. Results: We identified 107 patients, 76% (81/107) males, mean age 40.2 ± 10.6 at the time of implantation, 38.3% (41/107) with left TGA (L-TGA), and 61.7% (66/107) with dextro-TGA (D-TGA). The VAD support resulted in hemodynamic improvement, including a decrease in the mean pulmonary arterial pressure (45 ± 15 mm Hg before implantation to 25 ± 9.3 mm Hg afterwards, P < .001), pulmonary vascular resistance (6.3 ± 4.9 Wood units (WU) to 2.4 ± 1.35 WU, P < .001), right atrial pressure (16.4 ± 7.3 mm Hg to 9.5 ± 3.7 mm Hg, P = .009), and pulmonary capillary wedge pressure (25.45 ± 7.12 mm Hg to 14.25 ± 5.6 mm Hg, P < .001). The cardiac index increased from 2.0 ± 0.5 L/min/m² to 2.8 ± 0.6 L/min/m² (P = .004). The 1-year survival rate was 80.5%. Eventually, 31 (29%) underwent heart transplantation, and 48 (54%) remained on VAD at the time of publication. Conclusions: Durable VADs provide hemodynamic improvement and excellent survival in adults with a systemic right ventricle. Expanding the use of VADs for this patient population would be justified.

ObjectivesA minority of patients with hypoplastic left heart syndrome (HLHS) are at extremely high risk for staged palliation and can be bridged-to-heart transplantation with bilateral pulmonary artery bands, ductal stenting, and single ventricle-ventricular assist device insertion (HYBRID + sVAD). The purpose of this analysis is to assess our learning curve associated with our first ten patients with functionally univentricular ductal-dependent systemic circulation who were supported with primary HYBRID + sVAD as bridge-to-heart transplantation.MethodsPatients were temporally separated into two cohorts: the first five and second five. Demographic, perioperative, and outcome data were collected. Continuous variables are described as median [IQR](range). Categorical variables are described as N (%). P values were calculated using Fisher exact t test for categorical variables and unpaired t tests for continuous variables.ResultsTen patients underwent HYBRID + sVAD operations for HLHS (2017-2022). Patients in the initial cohort and the most recent cohort were similar in age and weight. Liver dysfunction and renal dysfunction were more common in the first five patients (2/5 = 40%) versus the next five patients (0/5 = 0%). Length of sVAD support was longer in the most recent five patients (98 days [64-138] vs 154 days [134-225], P = .08); however, no increase in sVAD-associated stroke or bleeding was seen in the most recent five patients. Despite very similar demographic and preoperative profiles, only two of the first five patients (2/5 = 40%) survived to heart transplantation, while all of the next 5 (5/5 = 100%) were successfully bridged-to-cardiac transplantation with HYBRID + sVAD and are alive today.ConclusionsOur experience with primary HYBRID + sVAD as bridge-to-heart transplantation in neonates with HLHS demonstrates an important learning curve associated with this operation and approach.

BackgroundChylothorax, the accumulation of triglyceride-rich fluid in the pleural cavity, is a well-recognized complication after surgery for congenital heart disease in children. Treatment protocols and role of surgery are not standardized.ObjectiveThis study aims to evaluate the outcomes of a standardized technique of thoracoscopic ligation of the thoracic duct (TLTD), for the management of persistent chylothorax following pediatric cardiac surgery.MethodsA longitudinal study was conducted on children diagnosed with postoperative chylothorax at a single center from 2018 to 2024. Children who required surgery were included in the study. Data on demographics, treatment timelines, and outcomes were collected and analyzed.ResultsOut of 11,429 children who underwent cardiac surgery, 24 children (median age of 33.5 months) required surgery for persistent chylothorax. Thoracoscopic ligation of the thoracic duct was typically performed on day 7 after diagnosis. Chylothorax resolved in 23 out of 24 cases by a median of 10 days following TLTD. The procedure caused minimal morbidity, and no significant procedure-related complications. There were two deaths due to poor cardiac function despite resolution of chylothorax.ConclusionThoracoscopic ligation of the thoracic duct is a safe and effective technique for persistent chylothorax in children following cardiac surgery. This standardized technique is reproducible and its timely application enhances recovery and reduces hospital stay. Further research will define the role of this technique in overall management of chylothorax postcardiac surgery.

Objective: To analyze in-hospital mortality in children undergoing congenital heart interventions in the only public referral center in Amazonas, North Brazil, between 2014 and 2022. Methods: This retrospective cohort study included 1041 patients undergoing cardiac interventions for congenital heart disease, of whom 135 died during hospitalization. Records were reviewed to obtain demographic, clinical, and surgical data. Descriptive statistics were applied for categorical and continuous variables. Results: In-hospital mortality was 12.96% (135/1041). Most deaths occurred in infants (97/135, 71.8%), and the median survival postsurgery was 1 day. The majority of patients were male (70/135, 51.8%), mixed-race (119/135, 88.2%), and born in Manaus (88/135, 65%). Only 5/135 (3.7%) underwent fetal echocardiography. Extracardiac malformations were present in 76/135 (56.3%), and 123/135 (91%) required mechanical ventilation postoperatively. Blalock-Taussig-Thomas shunt was the most frequent surgery among those who died. The RACHS-1 Category 3 concentrated 51.8% (70/135) of deaths. Temporal trends showed increased mortality after 2016, coinciding with the rise in neonatal and infant surgeries. Surgical complications (81/135, 60%) and hospital-acquired infections (34/135, 25%) were the leading causes of death. Cardiopulmonary bypass was used in 92/135 (68%) of cases, with a mean duration of 84 min. Conclusion: In-hospital mortality following congenital heart surgery in the Brazilian Amazon remains elevated, particularly among infants and in intermediate-risk RACHS-1 categories. These findings suggest that factors beyond surgical complexity-such as late diagnosis, referral delays, and resource limitations-contribute to adverse outcomes. Strengthening early diagnosis, improving perioperative care, and expanding regional surgical capacity are essential to reducing disparities in pediatric cardiac care.

Background: Delayed sternal closure (DSC) is frequently utilized to facilitate the recovery of myocardial function and edema following the Norwood procedure. At our institution, most patients undergo primary sternal closure (PSC), unless specified high-risk characteristics are present. We sought to analyze the outcomes of our approach. Methods: A retrospective review was performed of patients who underwent the Norwood procedure from 2017 to 2022. Patients were divided into two groups-PSC and DSC. Baseline demographics, clinical characteristics, and perioperative details were compared. The primary outcomes of interest were operative survival and infectious complications. Results: The Norwood procedure was performed in 116 neonates, of whom 80 (68.9%) underwent PSC. Baseline clinical characteristics and echocardiographic findings were not different between groups, except for lower preoperative inotrope use in the PSC group (13/80 [16.3%] vs 15/36 [41.7%], P = .003). Perioperatively, PSC patients were less likely to have multiple bypass runs (3/80 [3.8%] vs 9/36 [25.0%], P < .001) and less postoperative mechanical circulatory support (6/80 [7.5%] vs 12/36 [33.3%], P < .001). Hospital survival was higher (73/80 [[91.3%] vs 24/36 [66.7%], P = .04), and length of stay was shorter (29.0 vs 78.0 days, P < .001) with PSC. Of the 80 patients in the PSC group 5 (6.2%) required reopening of the sternum. By multivariable analysis, DSC was not an independent risk factor for operative mortality (OR 1.42 [95% CI 0.33-6.02], P = .63) or infection (OR 2.22 [95% CI 0.74-6.60], P = .15). Conclusions: Primary sternal closure can be performed in most patients following the Norwood procedure with favorable outcomes. Selected patients with high-risk preoperative or intraoperative features may benefit from DSC. Delayed sternal closure does not independently increase the risk of mortality or infectious complications.