World journal for pediatric & congenital heart surgery最新文献

Biventricular repair of a straddling mitral valve (MV) can involve relocating ectopic papillary muscles and chordae. However, this increases operative complexity and risks MV incompetence. We describe a nine-month-old with D-transposition of the great arteries and straddling MV. Three-dimensional imaging identified ventricular septum malposition and defined a simple repair strategy via ventricular septal defect patch closure rightward of the ectopic papillary muscle along with the arterial switch operation. This case highlights the role of 3D imaging in planning safe biventricular repair of straddling MV in an infant.

Background: We sought to determine the management and early outcomes of complete atrioventricular septal defect-tetralogy of Fallot (AVSD-TOF) for a contemporary multicenter cohort.

Methods: Of 739 participants in the Congenital Heart Surgeons' Society AVSD cohort (January 2012-May 2021), 40 had AVSD-TOF. We first compared survival differences for patients with AVSD-TOF versus those with isolated AVSD using propensity matching. Secondly, for patients with AVSD-TOF, we compared staged (n = 16) versus primary (n = 24) repair by assessing the following: patient characteristics, progression of atrioventricular valve (AVV) regurgitation, and time-related reoperation and survival.

Results: Five-year survival was similar between matched AVSD-TOF and isolated AVSD groups (80% vs 81%, P = .9). Compared with primary repair patients, staged patients had smaller pulmonary valve annulus Z-score measured at first presentation (-2.2 vs -2.9, P = .006). All staged patients (12 Blalock-Thomas-Taussig shunts, 3 right-ventricular-outflow-tract stents, 1 ductal stent) survived to complete repair. Freedom from AVSD-related reoperation five years post-AVSD-TOF repair was 57% after staged versus 90% after primary repair (P < .05) and left AVV reoperations were the most frequent reintervention. Survival five years after AVSD-TOF repair was 80% (63% after staged vs 90% after primary repair; P = .08).

Conclusions: Patients undergoing AVSD-TOF repair have similar survival compared with matched isolated AVSD patients. Although approximately half of AVSD-TOF patients had initial palliation and all survived to complete repair, staged repair patients had lower survival and a higher reintervention rate compared with primary repair patients. The decision to pursue staged versus primary repair for future babies with AVSD-TOF remains challenging and should be chosen based on individual circumstances.

Background: Tricuspid atresia (TA) is the second most common form of functionally univentricular heart. For patients with TA and normally related great arteries (Type I), left ventricular outflow tract obstruction (LVOTO) is rare.

Methods: From the Congenital Heart Surgeons' Society multi-institutional cohort of 445 patients with Type I TA enrolled from 1999 to 2024 from 42 sites, 14 infants (3%) had interventions for associated LVOTO, either at presentation or after their first TA-related intervention.

Results: Of seven infants initially undergoing Norwood/Damus-Kaye-Stansel (DKS), six survived to Stage II, of whom five survived with one developing pulmonary hypertension and four achieving Fontan. An additional seven infants who were first managed with pulmonary artery band placement subsequently had bidirectional superior cavopulmonary anastomosis (BCPA) and a DKS procedure; there were six survivors, all achieving Fontan. All ten survivors who underwent the Fontan procedure had normal left ventricular and mitral valve function at the latest follow-up. The overall Kaplan-Meier survival estimate at 20 years for these 14 patients was 79% (70% CI, 66%-88%), and the median follow-up was 8.3 years (0.24-21.5).

Conclusions: While infants with TA and transposition of the great arteries are more likely to have LVOTO, this can also occur in the setting of normally related great arteries. Infants with Type I TA and LVOTO can be managed in the neonatal period with the Norwood procedure ensuring complete arch relief with acceptable outcomes. If LVOTO becomes evident after initial pulmonary artery band placement, a subsequent DKS procedure facilitates satisfactory success to Fontan.

Background: Epicardial pacing systems, rather than transvenous systems, are utilized in pediatric patients who are too small to undergo transvenous access and/or have complex congenital heart disease (CHD) anatomically precluding a transvenous system. Longitudinal performance studies indicate that epicardial leads have higher failure rates when compared with transvenous leads but there are limited data on lead measurement changes in the acute phase after implantation. The objective of this study was to assess epicardial lead performance in the acute postimplant period and at one-year follow-up. Methods: This is a retrospective single center study of children and adult patients with CHD undergoing epicardial bipolar pacing lead and generator implantation between January 2012 and June 2022. We empirically selected 2 V as a critical lead threshold. Results: There were 256 leads implanted in 127 patients (mean age 6.1 ± 9.8 years), including 201/256 (79%) leads in patients with CHD. At the time of implant, 47/256 leads (18%) had a lead threshold of ≥2 V. Of those, 42 of 47 (89%) had recorded threshold values on postoperative day 1 (POD1) and 37 of 42 (88%) had decreased to a more acceptable threshold that was <2 V. For patients with an implant threshold of ≥2 V; impedance >1000 ohms, presence of CHD, perioperative or acute postop status, and location of the lead (atrial vs ventricular) did not significantly impact the odds of having a persistently high threshold (≥2 V) on POD1. Conclusion: Epicardial pacing lead thresholds generally improve in the immediate postop period and in our experience most leads with a high implant threshold improved to <2 V by POD1.

We present a case of a three-month-old male who presented with a cervical esophageal duplication cyst requiring early surgical intervention. The patient presented with feeding difficulties, poor weight gain, and respiratory distress. Due to the position of the cervical esophageal duplication cyst and airway compression, this unique case required a multidisciplinary surgical approach involving both otolaryngology and cardiothoracic surgery.

Background: Pain control following cardiac surgery can be challenging, and inadequate pain control is associated with postoperative complications. Liposomal bupivacaine can improve postoperative pain control due to its prolonged duration compared with plain bupivacaine. However, there is a paucity of data regarding its efficacy in congenital cardiac surgery. The primary outcome was to compare opioid requirements between liposomal bupivacaine and plain bupivacaine for local infiltration in pediatric patients undergoing cardiac surgical procedures via a median sternotomy approach. Secondary outcomes included antiemetic use, adjunct pain medication use, length of stay, and pain scores.

Methods: This single center retrospective study analyzed data from 2017 to 2022 involving patients who underwent congenital cardiac surgery via median sternotomy. Sixty-three patients who received liposomal bupivacaine were matched with 33 patients who received plain bupivacaine. Patient data were extracted from the electronic medical record and underwent statistical analysis using Wilcoxon, χ², and Fisher exact tests.

Results: There were no demographic differences between the two groups. Postoperative opioid use did not differ significantly between the liposomal bupivacaine and plain bupivacaine groups. The liposomal bupivacaine group had increased acetaminophen and antiemetic use, while the plain bupivacaine group had increased ketorolac use. Otherwise, there were no significant differences in secondary outcomes, including length of stay.

Conclusion: Liposomal bupivacaine may not offer any advantage over plain bupivacaine in congenital cardiac postoperative pain control.

Anomalous left main coronary artery (LMCA) with an intraconal course is a relatively rare form of anomalous coronary artery. Intraconal LMCA typically originates from the right sinus of Valsalva. However, this report describes the very unusual circumstance of an intraconal LMCA originating from the left sinus.

Objective: The transannular patch remains the most common procedure performed for patients with Tetralogy of Fallot (TOF) with pulmonary stenosis. Pulmonary regurgitation has a negative impact on early and late outcomes. To address this issue pulmonary valve-sparing repair (PVSR) has been developed. Our study goal is to evaluate the mid-term outcomes (five years) of PVSR at our institution.

Material and methods: The data were collected retrospectively from June 2014 to June 2022. A total of 390 patients had total repair of TOF. Among these, PVSR was performed in 154 (39.4%) patients. The mid-term outcomes on the status of the pulmonary valve gradient, degree of pulmonary regurgitation, reintervention rate, and mid-term survival after PVSR were investigated.

Results: The median age at time of TOF repair was 12 (interquartile range [IQR]: 8-48) months and the median weight was 7.9 (IQR: 3.1-49.5) kg. The mean preoperative right ventricular outflow tract (RVOT) gradient was 77 ± 19.6 mm Hg. All patients had a pulmonary valve Z score of more than -2.5. The post-repair mean RV/LV pressure ratio was 0.49 ± 0.12. There was no surgical mortality. The median follow-up was 3 years (6 months to 8 years). The reintervention rate on the pulmonary valve was 4/154 (2.6%) at five years. The freedom from reintervention and from developing moderate pulmonary valve regurgitation at 5 years was 95% (151/154) and 77% (119/154), respectively.

Conclusion: Pulmonary valve-sparing repair gives good mid-term outcomes in a specific group of patients with TOF. Reintervention rates are very low and the peak gradient across the pulmonary valve came down in the majority of patients during mid-term follow up. An RVOT gradient more than 40mm Hg at discharge predicts a high risk of need for reintervention. We continue to monitor our patients for the long term outcome.

Objectives: It is well-known that right ventricle-to-pulmonary artery homograft conduit durability is worse for smaller conduits and smaller/younger patients. However, there is limited literature on age and conduit-size specific outcomes, or on the role of conduit oversizing. Methods: Patients diagnosed with tetralogy of Fallot and major aortopulmonary collateral arteries undergoing right ventricular outflow tract (RVOT) reconstruction with a valved aortic homograft conduit from November 2001 through March 2023, at our institution were included. Conduits were grouped and evaluated by diameter, diameter Z-score, and patient age at implant. The primary time-related outcome was freedom from RVOT reintervention. Factors associated with freedom from time-related outcomes were assessed with univariable Cox regression analysis. Results: A total of 863 RVOT conduits were implanted in 722 patients. On multivariable analysis, younger age, male sex, Alagille syndrome, smaller diameter of the conduit, and smaller Z-score were associated with shorter freedom from reintervention. Among patients with smaller diameter conduits, larger Z-scores were associated with longer freedom from conduit reintervention (P < .001). Transcatheter interventions were commonly used to extend conduit lifespan across ages and conduit sizes. Conclusions: Larger conduit diameter, older age, and higher conduit Z-score were associated with longer freedom from reintervention in patients undergoing RVOT reconstruction in this cohort. Oversizing of conduits, even beyond a Z-score of 4, is generally appropriate.

Background: Aortic valve disease results in left ventricular (LV) dilation and/or hypertrophy. Valve intervention may improve, but not normalize flow dynamics. We hypothesized that LV remodeling would be more favorable following the Ross procedure versus mechanical aortic valve replacement (mAVR). Methods: Patients who were 18 to 50 years of age and underwent Ross or mAVR from 2000 to 2016 at a single institution were retrospectively reviewed. Propensity score matching was performed and yielded 27 well-matched pairs. Demographics and echocardiographic variables of LV morphology and wall thickness were collected. Those with > mild residual valve disease were excluded. Primary endpoints included LV morphology. T test and Fisher exact test analysis were used for statistical comparison. Results: Average age at operation (Ross 35.3 ± 10.2 vs mAVR 37.3 ± 8.9 years) did not differ. Indication for operation was similar between groups. Preoperative echocardiographic variables did not differ. At average follow-up duration (Ross 7.9 ± 2.4 vs mAVR 7.3 ± 2.4 years), wall thickness was significantly smaller for Ross compared with mAVR (P = .00715). Only 4/27 (15%) of mAVR patients had normalized LV parameters compared with 16/27 (59%) of Ross patients (P = .000813). Residual hypertrophy was the most common long-term abnormality for mAVR. Conclusion: Following aortic valve replacement with the Ross procedure or mechanical aortic valve prosthesis, the Ross conferred more favorable LV remodeling compared with mAVR. Future directions include analyzing longer follow-up to determine if patterns persist and the impact on cardiac morbidity and mortality.