Search

Background: Failure of the pulmonary valve autograft (PVA) after the Ross procedure (RP) has discouraged its widespread use and has led to modifications or alternatives to the procedure. We sought to analyze whether certain technical modifications could improve results of the RP in children., Methods: Sixty-nine patients (median age, 12 years; range, 0.25-17.9) underwent the RP between January 1996 and December 2018. A concomitant Konno procedure was performed on 20 of 69 patients (29%). Prior interventions included balloon valvuloplasty in 30 (44%) and/or surgical valvuloplasty in 39 (57%). Technical modifications included using the native aortic root for external annuloplasty, implanting the autograft using uniplanar horizontal sutures through the aortic wall, normalizing the sinotubular junction, and wrapping the native root remnant around the PVA., Results: Operative mortality was 1 of 69 patients (1.5%), with no late deaths. No patient had neoaortic valvar stenosis and 7 of 68 (10%) had mild regurgitation on discharge echocardiogram. At latest follow-up (median, 9.4 years; range, 0.4-21.3) there was no significant change in Z scores of annulus, sinus, or sinotubular junction diameters when compared with those at discharge. Three patients (4.4%) required late autograft replacement, 2 PVA repair, and 2 resection of a pseudoaneurysm. Actuarial freedom from PVA replacement was 87% at 20 years. Freedom from right ventricular outflow tract catheter reintervention or reoperation was 83% and 80%, respectively., Conclusions: Technical modifications of the RP used in this cohort might successfully prolong the life of the PVA without compromising its growth, an important advantage in pediatric patients., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Previous single-center studies of diaphragm paralysis (DP) after pediatric cardiac surgery report incidence of 0.3% to 12.8% and associate DP with respiratory complications, prolonged ventilation and length of stay, and mortality. To better define incidence and associations between DP and various procedures and outcomes, we performed a multicenter study., Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried to identify children who experienced DP after cardiac surgery (2010-2018; 126 centers). Baseline characteristics and postoperative outcomes were compared between patients with and without DP as well as between patients who underwent plication and those who did not. Associations between center volume and center rates of DP and use of plication were also explored., Results: A total of 2214 of 191,463 (1.2%) patients experienced DP. Postoperative DP portended worse outcomes, including mortality (5.6% vs 3.5%; P < .001), major morbidity (37.2% vs 10.7%; P < .001), tracheostomy (7.1% vs 0.9%; P < .001), prolonged mechanical ventilation (38.0% vs 7.8%; P < .001), and 30-day readmission (22.0% vs 10.6%; P < .001). A total of 1105 of 2214 (49.9%) patients with DP underwent plication. Patients who underwent plication were younger, were smaller, had more risk factors, and underwent more complex surgeries. Plication rates varied widely across centers. There was no correlation between center volume and center risk-adjusted rates of DP (r = .05, P = .5), nor frequency of plication (r = .08, P = .4)., Conclusions: DP complicating pediatric heart surgery is rare but portends significantly worse outcomes. One-half of patients underwent plication. Center-level risk-adjusted rates of DP and plication are not associated with case volume. Significant variability in plication practices suggests a target for quality improvement., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Many instances of coronary artery anomalies are documented in the literature; however, the detection and treatment of an asymmetric, large aortic cusp causing obstruction of a coronary ostium in a symptomatic infant remains unreported. We present a case of a 2-month-old infant with an enlarged right coronary cusp obstructing the left coronary ostium, requiring emergent repair by relocating the left coronary button and reconstructing the sinus of Valsalva with autologous pericardium. This procedure preserved native aortic valve function, and the child remains asymptomatic months after discharge., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The effect of size mismatch between donor and recipient in pediatric lung transplantation (PLTx) is currently unknown. Previous studies in adults have suggested that oversized allografts are associated with improved outcomes after lung transplantation. We investigated this relationship to quantify its effect on posttransplant outcomes in children., Methods: The United Network of Organ Sharing database was queried for preadolescent (age <13 years) patients undergoing PLTx. Donor-to-recipient height, weight, and predictive total lung capacity (pTLC; ages 4 to 13; pTLC = 0.160 x exp[0.021 x height]) ratios were calculated. Exploratory analysis was performed to identify disjoint intervals at which survival was statistically different. Patients were categorized as well-matched, undersized, or oversized. Multivariate Cox proportional hazard regression modeling assessed the adjusted effect of mismatching on mortality. Survival analysis was performed using the Kaplan-Meier method., Results: The analysis included 540 children. One-year mortality was higher with a height mismatch of 5% or less (hazard ratio [HR], 2.97; p = 0.001) and above 5% (HR, 2.22; p = 0.009). Similarly, 1-year mortality was worse with weight mismatch of 10% or less (HR, 1.99; p = 0.035) and above 10% (HR, 2.04; p = 0.028). On unadjusted analysis, a pTLC ratio of less than 0.9 was associated with worse survival (p = 0.017). This finding persisted after multivariate risk adjustment (HR, 2.93; p = 0.02). Contrary to findings in adults, an oversized allograft (pTLC ratio > 1.1) was not associated with improved survival (HR, 1.95; p = 0.147)., Conclusions: In preadolescent children undergoing PLTx, size mismatching is associated with increased death. Our findings differ from studies in adults, which demonstrated improved survival associated with oversized allografts. Accordingly, well-matched allografts should be prioritized when assessing donor-recipient pairs for transplantation., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Although tricuspid valve detachment (TVD) facilitates improved exposure during transatrial ventricular septal defect (VSD) closure, few have analyzed the impact of TVD on long-term valve durability., Methods: Pediatric patients undergoing VSD closure at our institution from 1997 to 2013 were identified, and charts were retrospectively reviewed. Patients were separated into groups based on utilization of TVD. Propensity score matching was then performed using a nonparsimonious logistic regression model involving 7 variables. Primary outcome was residual TV dysfunction at long-term follow-up, defined as mild (2+) and moderate (3+) TV regurgitation (TR) on follow-up echocardiography. Secondary outcomes included postoperative atrioventricular dissociation, residual VSD, and reinterventions., Results: A total of 247 patients underwent VSD closure; 164 (66.4%) without TVD and 83 (33.6%) with TVD. Median follow-up time was 2,343 days (interquartile range, 1,237 to 3,963.5 days) in the group without TVD versus 1,606 days (interquartile range, 826 to 3,017 days) in those with TVD. After successfully matching 83 patients, 29 of 83 (34.9%) patients in the non-TVD group had mild TR versus 28 of 83 (33.7%) patients in the TVD group (p = 0.87). 2 patients in the non-TVD group had moderate TR versus 1 patient in the TVD group at long-term follow-up. One patient in each group suffered transient atrioventricular block, but neither required pacemaker insertion., Conclusions: TVD did not compromise long-term valve durability and did not impose increased morbidity. Patients who underwent TVD had a similar prevalence of mild TR to patients without TVD. Moderate TR was exceptionally rare in both groups. When exposure is difficult, TVD is a safe and effective technical adjunct., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Loeys-Deitz syndrome (LDS) is a connective tissue disorder characterized by aggressive aortopathy with a proclivity for aortic aneurysmal rupture and dissection. Prophylactic surgical intervention is often indicated to ameliorate risk of aneurysm rupture. Aortic root replacement involves excision of the coronary arteries from the aortic root with a button of surrounding aortic tissue and subsequent anastomosis of these buttons to the synthetic aortic graft. We report the case of a 16-year-old girl with LDS who developed pseudoaneurysms at the sites of previous coronary button implantation., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Loeys-Dietz syndrome (LDS) is an aggressive aortopathy with a proclivity for aortic aneurysm rupture and dissection at smaller diameters than other connective tissue disorders. We reviewed our surgical experience of children with LDS to validate our guidelines for prophylactic aortic root replacement (ARR)., Methods: We reviewed all children (younger than 18 years) with a diagnosis of LDS who underwent ARR at our institution. The primary endpoint was mortality, and secondary endpoints included complications and the need for further interventions., Results: Thirty-four children with LDS underwent ARR. Mean age at operation was 10 years, and 15 (44%) were female. Mean preoperative root diameter was 4 cm. Three children (9%) had composite ARR with a mechanical prosthesis, and 31 (91%) underwent valve-sparing ARR. Concomitant procedures included arch replacement in 2 (6%), aortic valve repair in 1 (3%), and patent foramen ovale closure in 16 (47%). There was no operative mortality. Two children (6%) required late replacement of the ascending aorta, 5 (15%) required arch replacement, 1 (3%) required mitral valve replacement, and 2 (6%) had coronary button aneurysms/pseudoaneurysms requiring repair. Three children required redo valve-sparing ARR after a Florida sleeve procedure, and 2 had progressive aortic insufficiency requiring aortic valve replacement after a valve-sparing procedure. There were 2 late deaths (6%)., Conclusions: These data confirm the aggressive aortopathy of LDS. Valve-sparing ARR should be performed when feasible to avoid the risks of prostheses. Serial imaging of the arterial tree is critical, given the rate of subsequent intervention., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The hybrid palliation for hypoplastic left heart syndrome has emerged as an alternative approach to the Norwood procedure. The development of patent ductus arteriosus (PDA) in-stent stenosis can cause retrograde aortic arch stenosis (RAAS), leading to significant morbidity. This study aimed to identify potential mechanisms of PDA in-stent stenosis contributing to RAAS., Methods: Tissues from stented PDAs were collected from 17 patients undergoing comprehensive stage II repair between 2009 and 2014. Patients requiring RAAS intervention based on cardiology-surgery consensus were defined as RAAS(+) (n = 10), whereas patients without any RAAS intervention were defined as RAAS(-) (n = 7). Tissues were examined by quantitative polymerase chain reaction analysis for vascular smooth muscle cell (VSMC) differentiation and proliferation markers., Results: Patient characteristics were hypoplastic left heart syndrome with aortic atresia in 6 and with aortic stenosis in 3; unbalanced atrioventricular canal in 3; double-inlet left ventricle/transposition of the great arteries in 3; and double-outlet right ventricle in 2. VSMC differentiation markers (β-actin, SM22, and calponin) and signaling pathways for VSMC modulation (transforming growth factor-β1, Notch, and platelet derived growth factor-BB) were significantly higher in the RAAS(+) than in RAAS(-) patients. The proliferation marker Ki67 was increased in RAAS(+) patients. Cell cycle markers were comparable in both groups., Conclusions: Increased VSMC differentiation and proliferation markers suggest a mechanism for inward neointima formation of the PDA in RAAS. The apparent lack of change in cell cycle markers is contrary to coronary artery in-stent stenosis, suggesting further targets should be examined. Combined primary in vitro PDA cell culture and proteomics can be strong tools to elucidate targets to reduce PDA in-stent stenosis for RAAS in the future., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The management of tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries is controversial because of the wide variability of pulmonary artery (PA) and major aortopulmonary collateral arteries morphology. Several different staged strategies have been used to promote growth of diminutive PA branches. We have preferred a right ventricular (RV)-PA homograft for symmetrical growth of the central PA branches. In this study we evaluated the success of this strategy., Methods: Between 2006 and 2012, 23 patients with pulmonary atresia and diminutive PAs underwent RV-PA homograft implantation. Median age was 2 months (range, 4 days to 18 months), and median body weight was 5.1 kg (range, 1.7 to 8.5 kg). The type of homograft was aortic in 8, pulmonary in 6, and femoral vein in 9. The mean diameter of the homograft was 10.5 mm (range, 6 to 16 mm). All procedures were performed on cardiopulmonary bypass. The PA diameter was measured at the time of the operation and subsequent catheterization., Results: The median size of the branch PA was 2.1 mm. In the 18 patients who had serial assessment of PA size, the right PA increased by 307% ± 184%, the left PA increased by 283% ± 139%, and the Nakata index increased from 28.8 ± 20.1 mm(2)/m(2) to 253 ± 96 mm(2)/m(2) during a median period of 347 days (range, 44 to 1,520 days). The PA growth ratio (PA growth in mm/mo) was similar between the right PA (0.42 ± 0.46 mm/mo) and the left PA (0.43 ± 0.47 mm/mo). There was no acute conduit failure. Seventeen patients required 28 percutaneous interventions for embolization of an aortopulmonary collateral or stenosis of the conduit or PA. There were no hospital deaths. Three patients died late after other procedures during a mean follow-up of 44.7 months. Twenty patients (87%) have undergone complete repair to date., Conclusions: RV-PA homograft implantation can be performed in neonates and infants with minimal risk of acute occlusion. The RV-PA homograft promotes rapid and balanced growth of central pulmonary arteries leading to complete repair in most patients., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We report a case in which replacement of a Smeloff-Cutter aortic ball prosthesis was required 28 years after initial implantation. A 57-year-old woman underwent aortic valve replacement with a 21-mm Smeloff-Cutter ball prosthesis and open mitral commissurotomy for aortic stenosis, aortic regurgitation, and mitral stenosis in 1973. Severe aortic regurgitation occurred in April 2001, and aortic valve reoperation combined with mitral valve replacement was successfully performed. The patient's aortic ball valve was nearly intact with perivalvular leakage probably causing the aortic regurgitation. Our experience documents longer durability for the Smeloff-Cutter prosthesis than has been reported to date.

Superior-inferior ventricles are a rare cardiac malformation characterized by the two ventricles lying one above the other instead of side by side. Consequently, the interventricular septum that separates such ventricles is horizontal, and anomalies of the atrioventricular valves and the ventriculoarterial relations are almost always present. This complex anomaly is difficult to manage with an operation, so few cases have been reported. We describe a successful experience in which we performed a double switch operation, consisting of the Senning and Jatene procedures, for this rare malformation accompanied by double-outlet right ventricle [S,L,L]. This is the first report we have been able to locate of a double switch operation for superior-inferior ventricles.