Adult Congenital Heart Disease Transplantation: Does Univentricle Physiology Impact Early Mortality?

BACKGROUND: With patients with congenital heart disease increasingly living into adulthood, there is a growing population of patients with adult congenital heart disease (ACHD) who have heart failure. Limited data exist on evaluating heart transplantation in this population. METHODS: A retrospective review was performed of patients with ACHD who underwent heart transplantation from November 1990 to January 2023. Kaplan-Meier, cumulative incidence accounting for competing risk of death, and subgroup analyses comparing those patients with biventricular (BiV) and univentricular (UniV) physiology were performed. Data are presented as median (interquartile range [IQR]) or counts (%). RESULTS: A total of 77 patients with a median age of 36 years (IQR, 27-45 years) were identified, including 57 (74%) BiV and 20 (26%) UniV patients. Preoperatively, UniV patients were more likely to have cirrhosis (9 of 20 [45.0%] vs 4 of 57 [7.0%]; P < .001) and protein losing enteropathy (4 of 20 [20.0%] vs 1 of 57 [1.8%]; P = .015). Multiorgan transplantation was performed in 23 patients (30%) and more frequently in UniV patients (10 [50%] vs 13 [23%]; P = .04). Operative mortality was 6.5%, 2 of 20 (10%) among UniV patients and 2 of 57 (4%) among BiV patients (P = .276). Median clinical follow-up was 6.0 years (IQR, 1.4-13.1 years). Survival tended to be lower among UniV patients compared with BiV patients, particularly within the first year (P = .09), but it was similar for survivors beyond 1 year. At 5 years, the incidence of rejection was 28% (IQR, 17%-38%) and that of coronary allograft vasculopathy was 16% (IQR, 7%-24%). CONCLUSIONS: Underlying liver disease and the need for heart-liver transplantation were significantly higher among UniV patients. Survival tended to be lower among UniV patients, particularly within the first year, but it was similar for survivors beyond 1 year.

Mechanical or biological prosthesis for aortic valve replacement in patients aged 45 to 74 years.

OBJECTIVE: The selection of valve prostheses for patients undergoing surgical aortic valve replacement remains controversial. In this study, we compared the long-term outcomes of patients undergoing aortic valve replacement with biological or mechanical aortic valve prostheses. METHODS: We evaluated late results among 5762 patients aged 45 to 74 years who underwent biological or mechanical aortic valve replacement with or without concomitant coronary artery bypass from 1989 to 2019 at 4 medical centers. The Cox proportional hazards model was used to compare late survival; the age-dependent effect of prosthesis type on long-term survival was evaluated by an interaction term between age and prosthesis type. Incidences of stroke, major bleeding, and reoperation on the aortic valve after the index procedure were compared between prosthesis groups. RESULTS: Overall, 61% (n = 3508) of patients received a bioprosthesis. The 30-day mortality rate was 1.7% (n = 58) in the bioprosthesis group and 1.5% (n = 34) in the mechanical group (P = .75). During a mean follow-up of 9.0 years, the adjusted risk of mortality was higher in the bioprosthesis group (hazard ratio, 1.30, P < .001). The long-term survival benefit associated with mechanical prosthesis persisted until 70 years of age. Bioprosthesis (vs mechanical prosthesis) was associated with a similar risk of stroke (P = .20), lower risk of major bleeding (P < .001), and higher risk of reoperation (P < .001). CONCLUSIONS: Compared with bioprostheses, mechanical aortic valves are associated with a lower adjusted risk of long-term mortality in patients aged 70 years or less. Patients aged less than 70 years undergoing surgical aortic valve replacement should be informed of the potential survival benefit of mechanical valve substitutes.

Delayed Sternal Closure vs Emergency Sternal Reopening in Adults With Congenital Heart Disease.

BACKGROUND: Limited data exist regarding outcomes of delayed sternal closure (DSC) in adults with congenital heart disease (ACHD). METHODS: We reviewed 159 ACHD patients undergoing cardiac operation from 1993 to 2023 who required DSC (open sternum at the end of operation, n = 112) or sternum emergently reopened (n = 47). Regression models were performed to determine factors associated with outcomes. RESULTS: Of 112 patients undergoing DSC, 87 patients (77.6%) underwent DSC ≤4 days and 25 patients (22.3%) >4 days. The most common operations were valve (n = 35 [31.2%]), aortic (n = 33 [29.4%]), and right ventricular outflow tract procedures (n = 23 [20.5%]). Median time to chest closure was 2 days (interquartile range, 1-5 days). Apart from sex, baseline characteristics were similar between DSC groups. A stepwise increase in early mortality was observed from DSC ≤4 days to DSC >4 days (6.8% vs 32%), as well as the incidence of early complications, except sternal infection. Risk factors associated with early mortality were age (P = .02), DSC >4 days (P < .001), hemodynamic indication (P = .03), and single ventricle (P = .02). On multivariable analysis, lower ejection fraction (P = .04), hemodynamic indication (P = .02), single ventricle (P = .004), and diabetes mellitus (P = .03) were predictors of prolonged time to chest closure. Among hospital survivors, late survival was similar between patients undergoing DSC ≤4 days vs >4 days (P = .48). CONCLUSIONS: A brief duration of DSC in ACHD patients is associated with low morbidity and mortality. Higher early mortality and complications were observed among patients who did not achieve chest closure within 4 days.

Simultaneous Tricuspid Valve Repair at the Time of Mitral Valve Surgery: Determinants of Postoperative Pacemaker Implantation.

OBJECTIVE: Guidelines recommend tricuspid valve (TV) repair for patients with severe tricuspid valve regurgitation (TR) undergoing surgery for degenerative mitral valve (MV) disease, but management of ≤ moderate TR is controversial. This study examines the incidence and causes of bradyarrhythmias leading to PPM implantation. METHODS: Review of patients undergoing simultaneous TV repair and MV surgery for degenerative MV disease from 2001 to 2022 (N=404). Primary endpoint was the incidence of postoperative PPM implantation. Secondary endpoints included the incidence of high-degree AV block and overall survival. RESULTS: All patients underwent TV repair at the time of MV surgery; 332 (82%) underwent MV repair and 72 (18%) MV replacement. Tricuspid valve repair techniques included flexible band (n=258, 63.8%), DeVega annuloplasty (n=78, 19.3%), complete flexible ring (n=49, 12.1%), and incomplete rigid ring (n=19, 4.7%). The 30-day mortality was 0.5% (n=2). A total of 35 (8.7%) patients had a PPM implanted postoperatively, 26 (6.4%) for high-degree AV block. On multivariable analysis, only older age was associated with PPM implantation. Patients who received a PPM due to high-degree AV block had reduced overall survival (Figure, p=0.01). CONCLUSIONS: Need for permanent pacing following TV repair at the time of MV surgery is not uncommon, but there are few modifiable factors that might reduce this risk. Careful selection of patients with less-than-severe TR and surgical techniques may reduce PPM-related risks and complications.

Robotic-Assisted Resection of Rare Mitral Valve Hemangioma.

Cardiac hemangiomas are extremely rare tumors accounting for only 1.5%-2.5% of all cardiac tumors. According to most recent literature, only 13 mitral valve hemangiomas have been reported. A 78-year-old man was undergoing routine transthoracic echocardiography monitoring for an ascending aortic dilation when a vegetation on the mitral leaflet was incidentally detected. This lesion presented as a 0.5- × 0.6-cm mobile mass arising from the medial aspect of the A2 cusp. Despite the asymptomatic nature of the aforementioned lesion, resection was pursued given presumed diagnosis of papillary fibroelastoma and concern for risk of stroke. The mass was resected using minimally invasive robotic approach, and final pathology was consistent with hemangioma.

Single-Center Prospective Study of Cross-Clamp vs Balloon Occlusion in Robotic Mitral Surgery.

BACKGROUND: Transthoracic aortic cross-clamp and endoaortic balloon occlusion have both been shown to have comparable safety profiles for aortic occlusion. Because most surgeons use only one technique, we sought to compare the outcomes when a homogeneous group of surgeons changed their occlusion technique from aortic cross-clamp to balloon occlusion. METHODS: We changed our technique from aortic cross-clamp to balloon occlusion in November 2022. This allowed us to conduct a prospective treatment comparison study in the same group of surgeons. Propensity score matching was used to match cases (balloon occlusion) 1:3 to controls (aortic cross-clamp) based on age, sex, body mass index, concomitant maze procedure, and tricuspid valve repair. RESULTS: Total of 411 patients underwent robotic mitral surgery from 2020 through 2023. Propensity score matching was used to match 56 balloon occlusion patients to 168 aortic cross-clamp patients. The 224 patients were a median age of 65 years (interquartile range, 55.6-70.0 years), and 119 (53%) were men. All valves were successfully repaired. Balloon occlusion had a shorter median cardiopulmonary bypass (CPB) time compared with aortic cross-clamp (84.0 vs 94.5 minutes, P = .006). Median cross-clamp time (64.0 vs 64.0 minutes, P = .483) and total surgery time (5.9 vs 6.1 hours, P = .495) did not differ between groups. There were no in-hospital deaths. There were 5 surgeons who performed various combinations of console and bedside roles. CPB, cross-clamp, and surgery durations were not significantly affected by the different surgeon combinations. CONCLUSIONS: Compared with aortic cross-clamp, balloon occlusion has similar perioperative and early postoperative outcomes. Additionally, it likely introduces a 10-minute reduction in total CPB time.

A Case Series of Minimally Invasive Robotic-Assisted Resection of Cardiac Papillary Fibroelastoma: The Mayo Clinic Experience.

Papillary fibroelastomas (PFEs) are small, slowly growing benign cardiac tumors with clinically significant risk of embolization. Surgical excision is the definitive treatment of symptomatic PFE and is conventionally performed through a median sternotomy. In this study, we report a series of 12 patients, who underwent robotic-assisted PFE removal at the Mayo Clinic. PFE involved the mitral valve, left atrium, and tricuspid valve. No major complications occurred after the procedure, and most patients were discharged 4 days after the surgery. On follow-up, 1 patient demonstrated pericarditis.

Navigating missing link: innovative cannulation for robotic mitral valve repair in the absence of inferior vena cava.

Cardiac surgeries often require the use of cardiopulmonary bypass to allow visualization and manipulation of tissues. Vascular anomalies may impose challenges with access configuration. A patient was evaluated for robot-assisted mitral valve repair and found to have an atretic inferior vena cava secondary due to chronic occlusion. The patient was cannulated arterially through the left common femoral artery, and two cannulation sites were applied for venous drainage: the right intrajugular vein and a second percutaneous access site directly into the right atrium through the chest wall. The procedure was completed without immediate complications, and the patient's perioperative course was unremarkable.

Differences in Donor Heart Acceptance by Race and Gender of Patients on the Transplant Waiting List.

Importance: Barriers to heart transplant must be overcome prior to listing. It is unclear why Black men and women remain less likely to receive a heart transplant after listing than White men and women. Objective: To evaluate whether race or gender of a heart transplant candidate (ie, patient on the transplant waiting list) is associated with the probability of a donor heart being accepted by the transplant center team with each offer. Design, Setting, and Participants: This cohort study used the United Network for Organ Sharing datasets to identify organ acceptance with each offer for US non-Hispanic Black (hereafter, Black) and non-Hispanic White (hereafter, White) adults listed for heart transplant from October 18, 2018, through March 31, 2023. Exposures: Black or White race and gender (men, women) of a heart transplant candidate. Main Outcomes and Measures: The main outcome was heart offer acceptance by the transplant center team. The number of offers to acceptance was assessed using discrete time-to-event analyses, nonparametrically (stratified by race and gender) and parametrically. The hazard probability of offer acceptance for each offer was modeled using generalized linear mixed models adjusted for candidate-, donor-, and offer-level variables. Results: Among 159 177 heart offers with 13 760 donors, there were 14 890 candidates listed for heart transplant; 30.9% were Black, 69.1% were White, 73.6% were men, and 26.4% were women. The cumulative incidence of offer acceptance was highest for White women followed by Black women, White men, and Black men (P < .001). Odds of acceptance were less for Black candidates than for White candidates for the first offer (odds ratio [OR], 0.76; 95% CI, 0.69-0.84) through the 16th offer. Odds of acceptance were higher for women than for men for the first offer (OR, 1.53; 95% CI, 1.39-1.68) through the sixth offer and were lower for the 10th through 31st offers. Conclusions and Relevance: The cumulative incidence of heart offer acceptance by a transplant center team was consistently lower for Black candidates than for White candidates of the same gender and higher for women than for men. These disparities persisted after adjusting for candidate-, donor-, and offer-level variables, possibly suggesting racial and gender bias in the decision-making process. Further investigation of site-level decision-making may reveal strategies for equitable donor heart acceptance.

Outcomes of aortic surgery in patients with Takayasu arteritis.

OBJECTIVE: To investigate the presentation, aortic involvement, and surgical outcomes in patients with Takayasu arteritis undergoing aortic surgery. METHODS: We queried our surgical database for patients with Takayasu arteritis who underwent aortic surgery from 1994 to 2022. RESULTS: There were a total of 31 patients with Takayasu arteritis who underwent aortic surgery. Patients' median age at the time of diagnosis was 35.0 years (interquartile range, 25.0-42.0). The majority were female (n = 27, 87.0%). Most patients (n = 28, 90.3%) were diagnosed before surgery, and 3 patients (9.6%) were diagnosed perioperatively. The median time interval from diagnosis to surgery was 2.8 years (interquartile range, 0.5-13.9). The most common presentation was ascending aorta aneurysm (n = 22, 70.9%), and severe aortic regurgitation was the most common valve insufficiency (n = 17, 54.8%). The most common operation was ascending aorta replacement (n = 20, 64.5%), and aortic valve replacement was the most common valve intervention (n = 17, 54.8%). Active vasculitis was identified in 2 (11.7%) aortic valve specimens. Early mortality was 6.5% (n = 2). A total of 6 deaths occurred over a median follow-up of 13.1 years (interquartile range, 6.1-25.2). Survival at 10 years was 86.7% (95% CI, 75.4-99.7). A total of 5 patients (16.1%) required a subsequent operation in a median of 1.9 years (interquartile range, 0.2-7.4). Freedom from reoperation was 96.9% (95% CI, 90.1-100) at 1 year, 89.4% (95% CI, 78.7-100.0) at 5 years, and 77.5% (95% CI, 61.2-98.1) at 10 and 15 years. CONCLUSIONS: Ascending aorta aneurysm and aortic valve regurgitation are the most frequent presentations in patients with Takayasu arteritis requiring aortic surgery. Surgery in these individuals is safe, with acceptable short- and long-term results.

Efficacy of PD-1/PD-L1 immunotherapy on brain metastatic non-small-cell lung cancer and treatment-related adverse events: A systematic review.

BACKGROUND: Recent evidence suggests that PD-1/PD-L1 immunotherapy improves outcomes in patients with brain metastatic non-small cell lung cancer. METHODS: Records were searched electronically on MEDLINE, Embase and BIOSIS. Hazard ratios and their 95% confidence intervals for overall survival and progression free survival, and treatment-related adverse events data were extracted. Risk of bias was assessed in included studies using the Cochrane Collaboration's revised tool to assess risk of bias in randomized trials. RESULTS: PD-1/PD-L1 immunotherapy increased overall survival by 33% and progression free survival by 47% compared with chemotherapy. Two studies had a high risk of bias. Treatment-related adverse events were reported in 95%, 89% and 65% of patients receiving chemoimmunotherapy,chemotherapy and single agent immunotherapy, respectively. CONCLUSION: PD-1/PD-L1 inhibitors alone or in addition to chemotherapy increase overall and progression free survival when compared with chemotherapy alone. Chemoimmunotherapy and chemotherapy patients experienced the most treatment-related adverse events.

Case Report: A myxoma with a far reach.

A 73-year-old woman presented to the emergency department with a syncopal episode and a history of dizzy spells. A transthoracic echocardiogram demonstrated a large left atrial mass extending into the right upper pulmonary veins. Subsequently, cardiac magnetic resonance imaging and coronary computed tomography angiography with three-dimensional reconstruction and printing of the heart and mass were performed, which demonstrated a high index of suspicion for an atypical left atrial myxoma. The mass was excised robotically, and the pathology report confirmed a diagnosis of myxoma.

A Retrospective Analysis of 2-Year Follow-Up of Patients with Incidental Findings of Sarcoidosis.

INTRODUCTION: Sarcoidosis is a multi-system granulomatous disease most commonly involving the lungs. It may be incidentally diagnosed during imaging studies for other conditions or non-specific symptoms. The appropriate follow-up of incidentally diagnosed asymptomatic stage 1 disease has not been well defined. OBJECTIVE: To define the clinical course of incidentally diagnosed asymptomatic stage 1 sarcoidosis and propose an algorithm for the follow-up of these patients. METHODOLOGY: A retrospective case note analysis was performed of all EBUS-TBNA (endobronchial ultrasound-guided transbronchial needle aspiration)-confirmed cases of stage 1 sarcoidosis presenting incidentally to Bristol and Liverpool Interstitial Lung Disease services. Clinical history, serology results, imaging scans, and lung function parameters were examined at baseline, 12, and 24 months. A cost analysis was performed comparing the cost of the current 2-year follow-up guidance to a 1 year follow-up period. RESULTS: Sixty-seven patients were identified as the final cohort. There was no significant change in the pulmonary function tests over the two-year follow-up period. Radiological disease stability was observed in the majority of patients (58%, n = 29), and disease regression was evidenced in 40% (n = 20) at 1 year. Where imaging was performed at 2 years, the majority (69.8%, n = 37) had radiological evidence of disease regression, and 30.2% (n = 16) showed radiological evidence of stability. All patients remained asymptomatic and did not require therapeutic intervention over the study period. CONCLUSIONS: Our results show that asymptomatic patients with incidental findings of thoracic lymph nodal non-caseating granulomas do not progress over a 2-year period. Our results suggest that the prolonged secondary-care follow-up of such patients may not be necessary. We propose that these patients are followed up for 1 year with a further year of patient-initiated follow-up (PIFU) prior to discharge.

Genomic landscape and actionable mutations of brain metastases derived from non-small cell lung cancer: A systematic review.

Background: Brain metastases derived from non-small cell lung cancer (NSCLC) represent a significant clinical problem. We aim to characterize the genomic landscape of brain metastases derived from NSCLC and assess clinical actionability. Methods: We searched Embase, MEDLINE, Web of Science, and BIOSIS from inception to 18/19 May 2022. We extracted information on patient demographics, smoking status, genomic data, matched primary NSCLC, and programmed cell death ligand 1 expression. Results: We found 72 included papers and data on 2346 patients. The most frequently mutated genes from our data were EGFR (n = 559), TP53 (n = 331), KRAS (n = 328), CDKN2A (n = 97), and STK11 (n = 72). Common missense mutations included EGFR L858R (n = 80) and KRAS G12C (n = 17). Brain metastases of ever versus never smokers had differing missense mutations in TP53 and EGFR, except for L858R and T790M in EGFR, which were seen in both subgroups. Of the top 10 frequently mutated genes that had primary NSCLC data, we found 37% of the specific mutations assessed to be discordant between the primary NSCLC and brain metastases. Conclusions: To our knowledge, this is the first systematic review to describe the genomic landscape of brain metastases derived from NSCLC. These results provide a comprehensive outline of frequently mutated genes and missense mutations that could be clinically actionable. These data also provide evidence of differing genomic landscapes between ever versus never smokers and primary NSCLC compared to the BM. This information could have important consequences for the selection and development of targeted drugs for these patients.

Cardiac Transplantation: Physiology and Natural History of the Transplanted Heart.

Heart transplantation (HT) is one of the prodigious achievements in modern medicine and remains the cornerstone in the treatment of patients with advanced heart failure. Advances in surgical techniques, immunosuppression, organ preservation, infection control, and allograft surveillance have improved short- and long-term outcomes thereby contributing to greater clinical success of HT. However, prolonged allograft and patient survival following HT are still largely restricted by the development of late complications, including allograft rejection, infection, cardiac allograft vasculopathy (CAV), and malignancy. The introduction of mTOR inhibitors early after HT has demonstrated multiple protective effects against CAV progression, renal dysfunction, and tumorigenesis. Therefore, several HT programs increasingly use mTOR inhibitors with partial or complete withdrawal of calcineurin inhibitor (CNI) in stable HT patients to reduce complications risk and improve long-term outcomes. Furthermore, despite a substantial improvement in exercise capacity and health-related quality of life after HT as compared to advanced heart failure patients, most HT recipients remain with a 30% to 50% lower peak oxygen consumption (Vo 2 ) than that of age-matched healthy subjects. Several factors, including alterations in central hemodynamics, HT-related complications and alterations in the musculoskeletal system, and peripheral physiological abnormalities, presumably contribute to the reduced exercise capacity following HT. Cardiac denervation and subsequent loss of sympathetic and parasympathetic regulation are responsible for various physiological alterations in the cardiovascular system, which contributes to restricted exercise tolerance. Restoration of cardiac innervation may improve exercise capacity and quality of life, but the reinnervation process is only partial even several years after HT. Multiple studies have shown that aerobic and strengthening exercise interventions improve exercise capacity by increasing maximal heart rate, chronotropic response, and peak Vo 2 after HT. Novel exercise modalities, such as high-intensity interval training (HIT), have been proven as safe and effective for further improvement in exercise capacity, including among de novo HT recipients. Further developments have recently emerged, including donor heart preservation techniques, noninvasive CAV and rejection surveillance methods, and improvements in immunosuppressive therapies, all aiming at increasing donor availability and improving late survival after HT. © 2023 American Physiological Society. Compr Physiol 13:4719-4765, 2023.

Diagnosis and surgical management of pericardial constriction after cardiac surgery.

OBJECTIVES: Previous cardiac surgery is an increasingly common etiology of constrictive pericarditis, but there is a paucity of data on clinical presentation and outcome of surgical treatment. METHODS: We reviewed data of 263 patients who underwent pericardiectomy for postoperative constriction from January 1, 1993, through July 1, 2017. Outcomes of interest were early and late mortality, as well as features of clinical presentation. RESULTS: Median patient age was 64 (56-72) years, and the median interval between previous operation and pericardiectomy was 2.7 years (range, 0-54 years). Previous operations included coronary artery bypass grafting in 114 (43%), valve surgery in 85 (32%), combined coronary artery bypass grafting and valve surgery in 33 (13%), and other procedures in 31 (12%). Common presentations were symptoms of right heart failure in 221 (84%) or dyspnea in 42 (16%). Moderate-to-severe tricuspid valve regurgitation was present in 108 (41%) patients. There were 14 (5.5%) deaths within 30 days postoperatively, and survival at 5 and 10 years postoperatively was 61% and 44%. On multivariate analysis, older age (P = .013), diabetes (P = .019), and nonelective pericardiectomy within 2 years of cardiac surgery (P < .001) were associated with decreased long-term survival. CONCLUSIONS: Pericardial constriction after cardiac surgery can present at any interval postoperatively. Symptoms and signs of right heart failure in patients with previous cardiac surgery should alert physicians to the possibility of pericardial constriction followed by a correct diagnosis. Pericardiectomy performed urgently following cardiac operation has poor long-term outcomes.

Impact of concomitant coronary artery bypass grafting on postoperative outcomes in patients undergoing pericardiectomy: A 33-year experience.

Objective: Concomitant coronary artery bypass grafting (CABG) and pericardiectomy (PC) can be a technically challenging operation. We sought to study the outcomes of patients undergoing concomitant PC and CABG. Methods: Between July 1983 and August 2016, 70 patients (median age, 67 years; 88% males) underwent concomitant PC and CABG (PC + CABG group). Multivariable analysis was used to identify predictors of mortality. Matched patients who underwent isolated PC (PC group) were identified, and postoperative outcomes and long-term survival in the 2 groups were compared. Results: Compared with the PC group, cardiopulmonary bypass time was significantly longer in the PC + CABG group (82 minutes vs 61 minutes; P < .001). In-hospital mortality was 4% in the PC group and 7% in the PC + CABG group (P = .380). Multivariable analysis identified peripheral vascular disease (hazard ratio [HR], 2.67; 95% CI, 1.06-6.76; P = .04) as a predictor of increased morbidity or mortality and a borderline association with New York Heart Association functional classes III and IV (HR, 2.41; 95% CI, 0.99-5.86; P = .05) with increased morbidity and mortality in the PC + CABG group. Kaplan-Meier estimates demonstrated similar late mortality rates in the 2 groups at a 15-year follow-up (P = .700). Conclusions: Concomitant PC and CABG is not associated with increased morbidity or mortality compared with isolated PC. Thus, CABG should not be denied at the time of PC.

Percutaneous Mechanical Circulatory Support as a Bridge to Transplantation in Ebstein Anomaly.

Ebstein anomaly is a rare congenital heart defect occurring in 0.0005% of the population because of mispositioning and malformation of the tricuspid valve. Here, we present the first description and associated imaging of percutaneous mechanical circulatory support in the setting of cardiogenic shock secondary to Ebstein anomaly.

Whole Body Retrograde Perfusion Combined With Central Aortic Perfusion Strategy in the Repair of Distal Arch Pathology Through a Lateral Thoracotomy.

OBJECTIVE: To compare the results of the hypothermic circulatory arrest (HCA) + retrograde whole-body perfusion (RBP) technique with those of deep hypothermic circulatory arrest (DHCA-only) approach. METHODS: Limited data are available on cerebral protection techniques when distal arch repairs are performed through a lateral thoracotomy. In 2012, the RBP technique was introduced as adjunct to HCA during open distal arch repair via thoracotomy. We reviewed the results of the HCA + RBP technique compared with those of the DHCA-only approach. From February 2000 to November 2019, 189 patients (median age, 59 [IQR, 46 to 71] years; 30.7% female) underwent open distal arch repair via lateral thoracotomy to treat aortic aneurysms. The DHCA technique was used in 117 patients (62%, median age 53 [IQR, 41 to 60] years), whereas HCA + RBP was used in 72 patients (38%, median age 65 [IQR, 51 to 74] years). In HCA + RBP patients, cardiopulmonary bypass was interrupted when systemic cooling achieved isoelectric electroencephalogram; once the distal arch had been opened, RBP was then initiated via the venous cannula (flow of 700 to 1000 mL/min, central venous pressure <15 to 20 mm Hg). RESULTS: The stroke rate was significantly lower in the HCA + RBP group (3%, n=2) compared with the DHCA-only (12%, n=14) (P=.031), despite longer circulatory arrest times in HCA + RBP compared with the DHCA-only (31 [IQR, 25 to 40] minutes vs 22 [IQR, 17 to 30] minutes, respectively; P<.001). Operative mortality for patients undergoing HCA + RBP was 6.7% (n=4), whereas for those undergoing DHCA-only it was 10.4% (n=12) (P=.410). The 1-, 3-, and 5-year age-adjusted survival rates for the DHCA group are 86%, 81%, and 75%, respectively. The 1-, 3-, and 5-year age-adjusted survival rates for the HCA + RBP group are 88%, 88%, and 76%, respectively. CONCLUSION: The addition of RBP to HCA in the treatment of distal open arch repair via a lateral thoracotomy is safe and provides excellent neurological protection.

Optimizing Referral Timing of Patients With Fontan Circulatory Failure for Heart Transplant.

BACKGROUND: There are no criteria guiding the timing of heart transplant referral for Fontan patients, nor are there any characteristics of those deferred or declined listing reported. This study examines comprehensive transplant evaluations for Fontan patients of all ages, listing decisions, and outcomes to inform referral practices. METHODS: Retrospective review of 63 Fontan patients formally assessed by the advanced heart failure service and presented at Mayo Clinic transplant selection committee meetings (TSM) January 2006 to April 2021. The study is compliant with the Helsinki Congress and Declaration of Istanbul and included no prisoners. Statistical analysis was performed with Wilcoxon Rank Sum and Fisher's Exact tests. RESULTS: Median age at TSM was 26 years (17.5, 36.5). Most were approved (38/63 [60%]); 9 of 63 (14%) were deferred and 16 of 63 (25%) were declined. Approved patients more commonly were <18 years old at TSM (15/38 [40%] vs 1/25 [4%], P = .002) compared with those deferred/declined. Complications of Fontan circulatory failure were less common in approved vs deferred/declined patients: ascites (15/38 [40%] vs 17/25 [68%], P = .039), cirrhosis (16/38 [42%] vs 19/25 [76%], P = .01), and renal insufficiency (6/38 [16%] vs 11/25 [44%], P = .02). Ejection fraction and atrioventricular valve regurgitation did not differ between groups. Pulmonary artery wedge pressure was overall high normal (12 mm Hg [9,16]) but higher in deferred/declined vs approved patients, 14.5 (11, 19) vs 10 (8, 13.5) mm Hg, P = .015. Overall survival was significantly lower in deferred/declined patients (P = .0018). CONCLUSION: Fontan patient referral for heart transplant at younger age and before the onset of end-organ complications is associated with increased approval for transplant listing.