Morbidity and Mortality in Adult Congenital Heart Surgery: Physiologic Component Augments Risk Prediction.

BACKGROUND: We sought to evaluate whether the anatomic and physiologic stratification system (ACAP score), released as part of the American College of Cardiology/American Heart Association updated guidelines for management of adult congenital heart disease (ACHD) in 2018, better estimated mortality and morbidity after cardiac operations for ACHD. METHODS: The ACAP score was determined for 318 patients (age ≥18 years) with ACHD undergoing heart surgery at our institution between December 2001 and August 2019. The primary end point was perioperative mortality. The secondary aim was to evaluate the performance of the ACAP, The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) Congenital Heart Surgery Mortality Categories, and ACHS mortality scores/categories at predicting a composite adverse outcome of perioperative mortality, prolonged ventilation, and renal failure requiring replacement therapy. Logistic regression models were built to estimate mortality and the composite outcome using anatomic and physiologic components independently and together. Receiver operating characteristic curves were created, and area under the curves were compared using the Delong test. RESULTS: The median age was 37 years (interquartile range, 26.3-50.0 years). There were 9 perioperative mortalities (2.8%). With respect to perioperative mortality, the area under the curve using the anatomic component only was 0.74, which improved to 0.81 after including physiologic severity (P = .05). When physiologic severity was added to the model for the composite outcome, the discriminatory abilities of the ACHS mortality score and the STAT categories increased significantly to 0.83 (95% CI, 0.75-0.91; P = .02) and 0.82 (95% CI, 0.73-0.90; P = .04), comparable to the predictive power of ACAP. CONCLUSIONS: Physiologic severity augments ability to predict mortality and morbidity after cardiac surgery for ACHD. There is need for more robust ACHD-specific risk models.

Mid-term outcomes of the COMMENCE trial investigating mitral valve replacement using a bioprosthesis with a novel tissue.

Objective: Novel tissue leaflets (RESILIA tissue) may improve durability of bioprosthetic heart valves. The COMMENCE trial is an ongoing prospective study to evaluate valve replacement using RESILIA tissue. This report describes mid-term outcomes in the mitral cohort of COMMENCE. Methods: Adult patients requiring mitral valve replacement were enrolled in a prospective, single-arm trial at 17 sites in the United States and Canada. An independent clinical events committee adjudicated safety events using definitions from established guidelines, and hemodynamic performance was evaluated by an independent echocardiographic core laboratory. Results: Eighty-two patients (median age 70 years) successfully underwent mitral valve replacement with the study valve. Five-year event-free probabilities for all-cause mortality, structural valve deterioration, and reoperation were 79.9%, 98.7%, and 97.1%, respectively. Hemodynamic valve function measurements were stable through the 5-year follow-up period; valvular leaks were infrequently observed and primarily clinically insignificant/mild. Conclusions: Mitral valve replacement patients implanted with a RESILIA tissue bioprosthesis had a good safety profile and clinically stable hemodynamic performance.

Long-term outcomes with the pulmonary autograft inclusion technique in adults with bicuspid aortic valves undergoing the Ross procedure.

OBJECTIVE: To compare outcomes with wrapped (pulmonary autograft inclusion) versus unwrapped techniques in adults with bicuspid aortic valves undergoing the Ross procedure. METHODS: Between 1992 and 2019, 129 adults with bicuspid aortic valves (aged ≥18 years) underwent the Ross procedure by a single surgeon. Patients were divided into those without autograft inclusion (unwrapped, n = 71) and those with autograft inclusion (wrapped, n = 58). Median follow-up was 10.3 years (interquartile range, 3.0-16.8 years). Need for autograft reintervention was analyzed using competing risks. RESULTS: Pre- and intraoperative characteristics as well as 30-day morbidity or mortality did not differ between cohorts. Survival at 1, 5, and 10 years, respectively, was 97.2%, 97.2%, and 95.6% in the unwrapped cohort and 100%, 100%, and 100% in the wrapped cohort (P = .15). Autograft valve failure occurred in 25 (35.2%) of the unwrapped and 3 (5.2%) of the wrapped patients. Competing risks analysis demonstrated the wrapped cohort to have a lower need for autograft reintervention (subhazard ratio, 0.28, 95% confidence interval, 0.08-0.91; P = .035). The cumulative incidence of autograft reintervention (death as a competing outcome) at 1, 5, and 10 years, respectively, was 10.2%, 14.9%, and 26.8% in the unwrapped cohort and 4.0%, 4.0%, and 4.0% in the wrapped cohort. CONCLUSIONS: In adults with bicuspid aortic valves, the Ross procedure with pulmonary autograft inclusion stabilizes the aortic root preventing dilatation and reduces the need for reoperation. The autograft inclusion technique allows the Ross procedure to be performed in this population with excellent long-term outcomes.

Newborn Screening for X-Linked Adrenoleukodystrophy in Nebraska: Initial Experiences and Challenges.

X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disease caused by pathogenic variants in ABCD1 resulting in defective peroxisomal oxidation of very long-chain fatty acids. Most male patients develop adrenal insufficiency and one of two neurologic phenotypes: a rapidly progressive demyelinating disease in mid-childhood (childhood cerebral X-ALD, ccALD) or an adult-onset spastic paraparesis (adrenomyeloneuropathy, AMN). The neurodegenerative course of ccALD can be halted if patients are treated with hematopoietic stem cell transplantation at the earliest onset of white matter disease. Newborn screening for X-ALD can be accomplished by measuring C26:0-lysophosphatidylcholine in dried blood spots. In Nebraska, X-ALD newborn screening was instituted in July 2018. Over a period of 3.3 years, 82,920 newborns were screened with 13 positive infants detected (4 males, 9 females), giving a birth prevalence of 1:10,583 in males and 1:4510 in females. All positive newborns had DNA variants in ABCD1. Lack of genotype-phenotype correlations, absence of predictive biomarkers for ccALD or AMN, and a high proportion of ABCD1 variants of uncertain significance are unique challenges in counseling families. Surveillance testing for adrenal and neurologic disease in presymptomatic X-ALD males will improve survival and overall quality of life.

Surgical Resection of a Symptomatic Ascending Aortic Mural Thrombus.

Aortic mural thrombus (AMT) is a rare disease with an unclear optimal treatment strategy. AMT in the ascending aorta is particularly uncommon and is associated with the additional risk of embolization to the brain. Resection of an ascending AMT is particularly challenging given the high risk of thrombus dislodgment during aortic cannulation and cross-clamp application. This case demonstrates successful surgical resection of a symptomatic ascending AMT without the use of hypothermic circulatory arrest, with complete excision of the thrombus and replacement of the abnormal aorta using graft material.

Effect of UNOS policy change and exception status request on outcomes in patients bridged to heart transplant with an intra-aortic balloon pump.

BACKGROUND: Intra-aortic balloon pumps (IABP) are used to bridge select end-stage heart disease patients to heart transplant (HT). IABP use and exception requests both increased dramatically after the UNOS policy change (PC). The purpose of this study was to evaluate the effect of PC and exception status requests on waitlist and post-transplant outcomes in patients bridged to HT with IABP support. METHODS: We analyzed adult, first-time, single-organ HT recipients from the UNOS Registry either on IABP at the time of registration for HT or at the time of HT. We compared waitlist and post-HT outcomes between patients from the PRE (October 18, 2016 to May 30, 2018) and POST (October 18, 2018 to May 30, 2020) eras using Kaplan-Meier curves and time-to-event analyses. RESULTS: A total of 1267 patients underwent HT from IABP (261 pre-policy/1006 post-policy). On multivariate analysis, PC was associated with an increase in HT (sub-distribution hazard ratio (sdHR): 2.15, p < .001) and decrease in death/deterioration (sdHR: 0.55, p = .011) on the waitlist with no effect on 1-year post-HT survival (p = .8). The exception status of patients undergoing HT was predominantly seen in the POST era (29%, 293/1006); only four patients in the PRE era. Exception requests in the POST era did not alter patient outcomes. CONCLUSIONS: In patients bridged to heart transplant with an IABP, policy change is associated with decreased rates of death/deterioration and increased rates of heart transplantation on the waitlist without affecting 1-year post-transplant survival. While exception status use has markedly increased post-PC, it is not associated with patient outcomes.

Lack of Awareness of Reimbursement and Compensation Models Among Cardiothoracic Surgery Trainees.

BACKGROUND: The objective of this study was to identify trainee knowledge gaps in reimbursement and compensation, determine the perceived importance of understanding these topics, and to explore if the Thoracic Surgery Curriculum needs additional educational material. METHODS: The Thoracic Surgical Residents Association Executive Committee selected the research proposal and distributed an anonymous electronic survey to 531 Accreditation Council for Graduate Medical Education cardiothoracic surgery trainees. Standard descriptive statistics and regression analyses were performed. RESULTS: One hundred fourteen responses were collected (response rate, 21.5%). Most trainees understood little to none about how attending surgeons are reimbursed (n = 74, 69%). Most trainees reported knowing little or nothing about pay-for-performance compensation (n = 73, 67%), bundled care (n = 82, 75%), or value-based reimbursement (n = 84, 77%). Approximately 20% of trainees were accurate in estimating surgeon reimbursement for 3 common cardiothoracic surgery procedures to within 20% of the true reimbursement value, whereas approximately 30% were accurate to within 50% of the true reimbursement value. No respondent characteristics were found to be associated with a more or less accurate reimbursement response. Additionally 81% of trainees (n = 87) responded that by the conclusion of training, understanding surgeon reimbursement is very important or extremely important and 90% of trainees (n = 95) either somewhat agreed or strongly agreed with including these topics in the Thoracic Surgical Curriculum. CONCLUSIONS: Despite acknowledging the importance of understanding physician compensation and reimbursement, cardiothoracic surgery trainees do not understand how the current models work. This study exemplifies the need for a succinct curriculum in this domain for trainees nationwide.

Early onset of deep sternal wound infection after cardiac surgery is associated with decreased survival: A propensity weighted analysis.

OBJECTIVES: To compare outcomes after the development of early (≤30 days) versus delayed (>30 days) deep sternal wound infection (DSWI) after cardiac surgery. METHODS: Between 2005 and 2016, 64 patients were treated surgically for DSWI following cardiac surgery. Thirty-three developed early DSWI, while 31 developed late DSWI. The mean follow-up was 34.1 ± 32.3 months. RESULTS: Survival for the entire cohort at 1, 3, and 5 years was 93.9%, 85.1%, and 80.8%, respectively. DSWI diagnosed early and attempted medical management was strongly associated with overall mortality (hazard ratio [HR], 25.0 and 9.9; 95% confidence intervals [CIs], 1.18-52.8 and 1.28-76.5; p-value .04 and .04, respectively). Survival was 88.1%, 77.0%, 70.6% and 100%, 94.0% and 94.0% at 1, 3, and 5 years in the early and late DSWI groups, respectively (log-rank = 0.074). Those diagnosed early were more likely to have a positive wound culture (odds ratio [OR], 0.06; 95% CI, 0.01-0.69; p = .024) and diagnosed late were more likely to be female (OR, 8.75; 95% CI, 2.0-38.4; p = .004) and require an urgent DSWI procedure (OR, 9.25; 95% CI, 1.86-45.9; p = .007). Both early diagnosis of DSWI and initial attempted medial management were strongly associated with mortality (HR, 7.48; 95% CI, 1.38-40.4; p = .019 and HR, 7.76; 95% CI, 1.67-35.9; p = .009, respectively). CONCLUSIONS: Early aggressive surgical therapy for DSWI after cardiac surgery results in excellent outcomes. Those diagnosed with DSWI early and who have failed initial medical management have increased mortality.

The Ross procedure utilizing the pulmonary autograft inclusion technique in adults.

A retrospective cohort study was conducted in which 129 adult patients with bicuspid aortic valves underwent the Ross procedure with either a standard root inclusion technique or a modified technique whereby the pulmonary autograft is wrapped in a vascular conduit. Primary outcomes were survival and the need for pulmonary autograft reintervention. Competing risk analysis demonstrated the wrapped technique reduced pulmonary autograft reintervention.

Burden of rare deleterious variants in WNT signaling genes among 511 myelomeningocele patients.

Genes in the noncanonical WNT signaling pathway controlling planar cell polarity have been linked to the neural tube defect myelomeningocele. We hypothesized that some genes in the WNT signaling network have a higher mutational burden in myelomeningocele subjects than in reference subjects in gnomAD. Exome sequencing data from 511 myelomeningocele subjects was obtained in-house and data from 29,940 ethnically matched subjects was provided by version 2 of the publicly available Genome Aggregation Database. To compare mutational burden, we collapsed rare deleterious variants across each of 523 human WNT signaling genes in case and reference populations. Ten WNT signaling genes were disrupted with a higher mutational burden among Mexican American myelomeningocele subjects compared to reference subjects (Fishers exact test, P ≤ 0.05) and seven different genes were disrupted among individuals of European ancestry compared to reference subjects. Gene ontology enrichment analyses indicate that genes disrupted only in the Mexican American population play a role in planar cell polarity whereas genes identified in both populations are important for the regulation of canonical WNT signaling. In summary, evidence for WNT signaling genes that may contribute to myelomeningocele in humans is presented and discussed.

Identification of novel candidate risk genes for myelomeningocele within the glucose homeostasis/oxidative stress and folate/one-carbon metabolism networks.

BACKGROUND: Neural tube defects (NTDs) are the second most common complex birth defect, yet, our understanding of the genetic contribution to their development remains incomplete. Two environmental factors associated with NTDs are Folate and One Carbon Metabolism (FOCM) and Glucose Homeostasis and Oxidative Stress (GHOS). Utilizing next-generation sequencing of a large patient cohort, we identify novel candidate genes in these two networks to provide insights into NTD mechanisms. METHODS: Exome sequencing (ES) was performed in 511 patients, born with myelomeningocele, divided between European American and Mexican American ethnicities. Healthy control data from the Genome Aggregation database were ethnically matched and used as controls. Rare, high fidelity, nonsynonymous predicted damaging missense, nonsense, or canonical splice site variants in independently generated candidate gene lists for FOCM and GHOS were identified. We used a gene-based collapsing approach to quantify mutational burden in case and controls, with the control cohort estimated using cumulative allele frequencies assuming Hardy-Weinberg equilibrium. RESULTS: We identified 45 of 837 genes in the FOCM network and 22 of 568 genes in the GHOS network as possible NTD risk genes with p < 0.05. No nominally significant risk genes were shared between ethnicities. Using a novel approach to mutational burden we identify 55 novel NTD risk associations. CONCLUSIONS: We provide a means of utilizing large publicly available sequencing datasets as controls for sequencing projects examining rare disease. This approach confirmed existing risk genes for myelomeningocele and identified possible novel risk genes. Lastly, it suggests possible distinct genetic etiologies for this malformation between different ethnicities.

Cardiovascular Computed Tomographic Angiography as a Virtual Patient Avatar for Individualized Surgical Planning of Complex Anomalous Coronary Artery Anatomy.

Cardiovascular computed tomographic angiography (CCTA) 3-D thoracic reconstruction can serve as a "virtual patient avatar" providing surgical views for approach to complex anomalous coronary artery anatomy. Images demonstrated a single coronary artery ostium arising from the right aortic sinus with trifurcation into a prepulmonic left anterior descending coronary artery (LAD), an interarterial circumflex with a subsequent intraseptal course, and normal course of the right coronary artery. Virtual 3-D CCTA reconstructions were important to planning an incisional plane for surgical correction.

Does cramming work? Impact of National Web-Based Thoracic Surgery Curriculum login frequency on thoracic surgery in-training exam performance.

OBJECTIVE: Web-based curricula provide login data that can be advantageously used to characterize and analyze study habits. We sought to compare thoracic surgical trainee In-Training Examination percentiles with regard to their study habits (ie, cramming), as characterized by curriculum login frequency to the national Web-based Thoracic Surgery Curriculum. Furthermore, we then aimed to characterize the curriculum login frequency of trainees as stratified by their performance on the In-Training Examination and their improvement on the In-Training Examination over subsequent years. METHODS: We performed a retrospective review of trainees who accessed the curriculum before the 2014 In-Training Examination, with curriculum login data collected from site analytics. Scores were compared between trainees who crammed (≥30% increase in logins in the month before the In-Training Examination) and those who did not. Trainees were stratified on the basis of 2014 In-Training Examination percentile and improvement in percentile from 2013 to 2014 into high, medium, and low scorers and improvers. RESULTS: Of 256 trainees who took the 2014 In-Training Examination, 63 (25%) met criteria as crammers. Crammers increased total study sessions immediately before the In-Training Examination (P < .001), but without impact on 2014 In-Training Examination percentile (P = .995) or year-to-year improvement (P = .234). Stratification by In-Training Examination percentile demonstrated that highest scoring trainees used the curriculum more frequently in the final month than medium-range scorers (P = .039). When stratified by extent of year-to-year improvement, those who improved the most accessed the curriculum significantly more often in the last month compared with baseline (P = .040). Moreover, those with greatest improvement logged in more in the final month than those with least improvement (P = .006). CONCLUSIONS: Increasing the frequency of study periods on the national Web-based thoracic surgery curriculum before the In-Training Examination may have a unique benefit to trainees who initially score low to allow them to significantly improve their subsequent year In-Training Examination performance.

Resection of Metastatic Hepatocellular Carcinoma in the Ventricular Septum Causing Left Ventricular Outflow Tract Obstruction.

Isolated cardiac involvement of recurrent metastatic hepatocellular carcinoma (HCC) is extremely rare. We report a patient with left ventricular outflow tract (LVOT) obstruction due to isolated recurrent HCC involving the interventricular septum (IVS). A ventriculotomy with resection of the tumor and patch repair of the IVS was performed with successful relief of LVOT obstruction. The patient was discharged home 6 days later symptom-free.

Optimal Approach for Repair of Left Atrial-Esophageal Fistula Complicating Radiofrequency Ablation.

Left atrial-esophageal fistula after endovascular radiofrequency ablation for cardiac arrhythmias is a life-threatening complication. Immediate surgical repair offers the best chance for survival. The optimal surgical technique is unknown. We describe our recommended surgical approach.