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We report an innovative treatment strategy for fetal Ebstein's anomaly with a circular shunt. We used transplacental non-steroidal anti-inflammatory drugs, at the 29th gestational week, to constrict the ductus arteriosus avoiding fetal demise. We addressed the critical neonate with an urgent Starnes procedure. Finally, instead of following the usual single-ventricle palliation pathway after the Starnes procedure, we achieved successful two-ventricle repair with the cone technique at 5 month old.
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Conducto Arterioso Permeable , Anomalía de Ebstein , Terapias Fetales , Preparaciones Farmacéuticas , Antiinflamatorios no Esteroideos/uso terapéutico , Conducto Arterioso Permeable/tratamiento farmacológico , Anomalía de Ebstein/cirugía , Femenino , Humanos , Lactante , Recién Nacido , EmbarazoRESUMEN
BACKGROUND: This study compares the long-term outcomes of patients after repair of transposition of the great arteries (TGA) with and without aortic arch obstruction (AAO). METHODS: This is a single-institution, retrospective study between October 2004 and February 2023. Patients who underwent arterial switch operation and aortic arch repair (ASO-AAR group) with patch augmentation were compared with those without AAO (ASO group). The primary end point was survival; freedom from reintervention was a secondary end point. RESULTS: We identified 176 patients, 31 in the ASO-AAR group and 145 in the ASO group. The median follow-up period was 10.3 years. There were no differences between the ASO-AAR group and the ASO group in early deaths (3.2% vs 0.7%) and late deaths (3.2% vs 2.8%), or 15-year survival rates (92.6% vs 96.2%). Surgical and catheter-based reinterventions were higher in the ASO-AAR group, involving the pulmonary arteries (41.9% vs 4.8%, P < .001), aortic arch (16.1% vs 0.7%, P < .001), and residual ventricular septal defects (11.4% vs 0%, P = .05). The ASO-AAR group showed a higher prevalence of double-outlet right ventricle TGA-type (61.3% vs 4.1%, P < .001) and a lower aortopulmonary index (0.67 vs 1.01, P < .001). CONCLUSIONS: Patients undergoing surgical repair of TGA and AAO achieved excellent survival rates, comparable to patients with simple transposition. A higher rate of surgical and catheter-based reinterventions was observed in patients with arch obstruction and/or a low aortopulmonary index. AAR with patch augmentation proved to be an effective surgical technique with a low incidence of aortic reinterventions.
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OBJECTIVES: We present a case series of Right Ventricle (RV) rehabilitation after Starnes in patients with Ebstein anomaly, applying the Cone repair of the tricuspid valve (TV) to achieve biventricular or 1.5-ventricle physiology. METHODS: This is a retrospective database analysis from two institutions in North America. We included all consecutive cases of Cone repair after the Starnes procedure. The data are expressed as median and interquartile range (IQR). RESULTS: Eleven patients underwent RV rehabilitation between 2019 and 2023 after initial Starnes palliation with a median age of 27 months (IQR 20.5). All were critically ill before their Starnes procedure; four were on extracorporeal membrane oxygenation. Before the Cone repair, the median pre-operative regurgitant velocity at the Starnes patch was 1.65 m/s (IQR 1.3). During the Cone procedure, nine patients required a concomitant PV repair, of which three required a transannular monocusp patch. Four patients were successfully rerouted to a two-ventricle repair, and seven patients with a previous Glenn achieved 1.5 ventricle circulation. There was no heart block and no deaths. Seven patients had trivial regurgitation in a median follow-up of eleven months (IQR 21.5), three mild and one moderate tricuspid regurgitation. There was no significant TV stenosis; all patients had good functional status at the last follow-up despite severe RV dysfunction in one patient. CONCLUSIONS: After the Starnes procedure, the Cone repair allowed RV rehabilitation, resulting in trivial or mild TV regurgitation sustained in midterm follow-up. The Starnes procedure is a reproducible technique that no longer commits patients to lifetime single ventricle physiology.
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OBJECTIVES: Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement. RESULTS: When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt. CONCLUSIONS: Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.
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Consenso , Anomalía de Ebstein , Humanos , Recién Nacido , Anomalía de Ebstein/cirugía , Anomalía de Ebstein/fisiopatología , Lactante , Medición de Riesgo , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Factores de Riesgo , Resultado del Tratamiento , Cirugía Torácica/normasRESUMEN
Interest and core training in congenital heart surgery (CHS) has not been characterized among current cardiothoracic surgical trainees. This study aimed to evaluate perceptions, interest, exposure, and experience among current trainees. A 22 question survey was distributed to all cardiothoracic surgical trainees in ACGME-accredited thoracic surgery residencies. Questions included demographics, exposure to CHS during, perceptions of, participation in and quality assessment of CHS training. There were 106 responses (20.1% response rate) of which 31 (29.0%) were female and 87 (81.3%) were cardiothoracic track. While 69 (64.5%) reported having an interest in CHS at some point during training, only 24 (22.4%) were actively pursuing CHS. All but 7 (6.5%) residents reported having easy access to congenital mentorship, with 35 (32.7%) actively participating in CHS research. Three months was the median duration of congenital rotations. Residents reported less operative participation on CHS compared to adult cardiac surgery. Several residents noted the need for earlier exposure and increased technical/operative experience as areas in need of improvement. The most cited primary influences to pursue CHS included: mentorship, breadth of pathology, and technical nature of the specialty. Lack of consistent job availability and length of additional training were reported as negative influences. Cardiothoracic residents report adequate exposure to obtain case requirements and knowledge for board examinations in CHS but highly variable operative involvement. Mentorship and early exposure remain important for those interested in CHS, while additional training time and limited job availability remain hurdles to CHS.
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Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Internado y Residencia , Cirugía Torácica , Adulto , Humanos , Femenino , Masculino , Resultado del Tratamiento , Cirugía Torácica/educación , Procedimientos Quirúrgicos Cardíacos/educación , Educación de Postgrado en Medicina , Encuestas y CuestionariosRESUMEN
BACKGROUND: Conduit longevity after right ventricular outflow tract (RVOT) reconstruction is determined by the interaction of different factors. We evaluated the relationship between conduit anatomic position and long-term durability among ≥18 mm polytetrafluoroethylene (PTFE) conduits. METHODS: A single-institution RVOT reconstructions using a PTFE conduit ≥18 mm were identified. Catheter-based interventions or the need for conduit replacement were comparatively assessed between orthotopic vs heterotopic conduit position. Time to the first reintervention, censored by death, was compared between the groups. RESULTS: A total of 102 conduits were implanted in 99 patients, with a median age of 13.2 years (interquartile range [IQR] 8.9-17.8 years), median weight of 47 kg (IQR, 29-67 kg), and body surface area of 1.4 m2 (IQR, 1-1.7 m2). Overall, 50.9% (n = 52) of conduits were placed in an orthotopic position after the Ross procedure in congenital aortic valve abnormalities (80% [n = 36]). Tetrology of Fallot in 39% (n = 18), followed by truncus arteriosus with 33% (n = 15), were the most common in the heterotopic position. Trileaflet configuration was similar (67% vs 69%; P = .32) between the groups. Survival free from reintervention was 91% (95% CI, 79-97) and 88% (95% CI, 71-95) in the orthotopic and the heterotopic group, respectively, at 5 years, without differences in the Kaplan Meier curves (log-rank >.05). CONCLUSIONS: RVOT reconstruction with PTFE conduits ≥ 8 mm showed >90% conduit survival free from replacement in our cohort at 5 years. The anatomic position of the PTFE conduit does not seem to impact intermediate durability.
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Cardiopatías Congénitas , Prótesis Valvulares Cardíacas , Tronco Arterial Persistente , Obstrucción del Flujo Ventricular Externo , Humanos , Lactante , Niño , Adolescente , Politetrafluoroetileno , Resultado del Tratamiento , Cardiopatías Congénitas/cirugía , Tronco Arterial Persistente/cirugía , Prótesis Vascular , Estudios Retrospectivos , Obstrucción del Flujo Ventricular Externo/cirugía , ReoperaciónRESUMEN
BACKGROUND: High risk (HR) factors have been shown to have increased rates of mortality after stage 1 palliation (S1P) for single ventricle physiology. It remains unclear how initial HR status affects longitudinal outcomes after subsequent stage 2 palliation (S2P) and stage 3 palliation (S3P). METHODS: Single ventricle patients undergoing S1P between July 2004 and October 2018 at a single institution were included. Patients having one or more HR factors were considered to have HR status, with all others classified as low risk (LR). Longitudinal survival stratified by risk status was compared after each palliative stage, in addition to readmission and length of stay. Proportional hazards modeling was used to determine risk factors for longitudinal mortality. RESULTS: Of 132 patients presenting during the study for S1P, 57 (43.2%) were classified as HR. Overall 10-year survival was decreased in the HR cohort (P = .001). The HR patients were at significantly increased risk of death during interstage I (P = .01) and interstage II (P = .01), but survival was similar to that of LR patients after S3P (P = .31). Readmission rates after S2P were higher among HR patients (41.9% vs 22.5%, P = .029), but were similar after S3P. Length of stay was increased in the HR cohort after S2P (median 11 vs 9 days, P = .024) but similar to the LR group after S3P. Prematurity was the risk factor most consistently associated with increased mortality after all stages. CONCLUSIONS: A high risk status of patients undergoing S1P portends a higher risk of mortality, length of stay, and readmission after S2P. High-risk patients have survival similar to that of low-risk patients after S3P.
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Síndrome del Corazón Izquierdo Hipoplásico , Corazón Univentricular , Estudios de Cohortes , Ventrículos Cardíacos , Humanos , Cuidados Paliativos , Estudios Retrospectivos , Factores de Riesgo , Resultado del TratamientoRESUMEN
BACKGROUND: Aortic root translocation (Nikaidoh), Rastelli, and réparation à l'etage ventriculaire (REV) are repair options for transposition of the great arteries (TGA) with ventricular septal defects and left ventricular outflow tract obstruction (VSD-LVOTO) or double outlet right ventricle TGA type (DORV-TGA). METHODS: This retrospective study using The Society of Thoracic Surgeons Congenital Heart Surgery Database evaluates surgical procedure utilization and outcomes of patients undergoing repair of TGA-VSD-LVOTO and DORV-TGA with a Nikaidoh, Rastelli, or REV procedure. RESULTS: A total of 293 patients underwent repair at 82 centers (January 2010 to June 2019). Most patients underwent a Rastelli (n = 165, 56.3%) or a Nikaidoh (n = 119, 40.6%) operation; only 3.1% (n = 9) underwent a REV. High-volume centers performed the majority of the repairs. Fewer Nikaidoh than Rastelli patients had prior cardiac operations (n = 57 [48.7%] vs n = 102 [63.0%]; P = .004). Nikaidohs had longer median cardiopulmonary bypass time (227 [interquartile range (IQR), 167-299] minutes vs 175 [IQR, 133-225] minutes; P < .001) and median aortic cross-clamp times (131 [IQR, 91-175] minutes vs 105 [IQR, 82-141] minutes; P = .0015). Operative mortality was 3.1% (95% confidence interval [CI], 1.0%-7.0%; n = 5) for Rastelli, 4.4% (95% CI, 1.4%-9.9%; n = 5) for Nikaidoh, and 11.1% (95% CI, 0.3%-48.3%, n = 1) for REV. The rates of cardiac arrest, unplanned reoperation, mechanical circulatory support, prolonged ventilation, and permanent pacemaker placement were higher in the Nikaidoh population but with 95% CIs overlapping those of the other procedures. CONCLUSIONS: Rastelli and Nikaidoh procedures are the prevalent repair strategies for patients with DORV-TGA and TGA-VSD-LVOTO. Most are performed at high-volume institutions, and early outcomes are similar.
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Ventrículo Derecho con Doble Salida , Cardiopatías Congénitas , Defectos del Tabique Interventricular , Cirujanos , Transposición de los Grandes Vasos , Obstrucción del Flujo Ventricular Externo , Cardiopatías Congénitas/cirugía , Defectos del Tabique Interventricular/cirugía , Humanos , Lactante , Estudios Retrospectivos , Transposición de los Grandes Vasos/cirugía , Resultado del Tratamiento , Obstrucción del Flujo Ventricular Externo/cirugíaRESUMEN
The ideal conduit for reconstruction of the right ventricular outflow tract in pediatric patients remains a topic of discussion. We present a technique for construction of a handmade tricuspid valved polytetrafluoroethylene conduit for use in patients of all ages requiring right ventricular outflow tract reconstruction at the time of congenital cardiac surgery. This conduit provides an economically advantageous and readily available option globally when compared with homograft, without sacrificing surgical results.
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Prótesis Valvulares Cardíacas , Politetrafluoroetileno , Diseño de Prótesis , Válvula Tricúspide/cirugía , Obstrucción del Flujo Ventricular Externo/cirugía , Procedimientos Quirúrgicos Cardíacos/métodos , HumanosRESUMEN
Ebstein's anomaly (EA) is a rare congenital cardiac anomaly. It is a disease at a minimum of the tricuspid valve (TV) and the right ventricular myocardium. Presentation varies from a severe symptomatic form during the neonatal period to an incidental detection later in life due to the wide morphological variation of the condition. The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. Some neonates initially committed to a single ventricle pathway may be converted to a biventricular repair by a delayed TV valvuloplasty. The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. If the chance of a successful TV valvuloplasty is high, it should be offered early in childhood to prevent further cardiac dilation. Adding a Bidirectional Glenn to a valvuloplasty may help salvage marginal risk patients or marginally repairable valves. If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.
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BACKGROUND: Contradictory data exist regarding timing of stage 2 palliation (S2P). Prolonged interstage hospitalizations and home surveillance programs have contributed to a more rapid progression to S2P. Our goal is to describe the S2P population and explore the relationships of clinical outcomes and S2P timing at our institution over the last 14 years. METHODS: S2P procedures from 2004 to 2018 at a single institution were included in a retrospective analysis. The analysis was stratified by S2P timing and clinical variables. The primary outcome was Fontan completion, and secondary outcomes included mortality and orthotopic heart transplantation rate, as well as perioperative clinical variables. RESULTS: A total of 114 patients who underwent S2P were included. The median age and weight at S2P were 100 days (interquartile range [IQR], 87-119) and 5.1 (IQR, 4.6-5.5) kg, respectively. The median age in the early group was 79 (IQR, 73-87) days and in the nonearly group was 107 (IQR, 100-124) days. Ninety percent of cavopulmonary anastomoses were augmented with an ePTFE (expanded polytetrafluoroethylene) patch. The overall Fontan completion rate was 76%, without differences in Kaplan-Meier estimates. There were no operative mortalities and no differences in late mortality rate (P = .30). CONCLUSIONS: The interstage period continues to be high risk for those undergoing single-ventricle palliation. In our experience, S2P performed at less than 90 days seems to be a viable and safe procedure when indicated, resulting in comparable Fontan completion rates.