Lung transplantation in primary pulmonary arterial hypertension and pulmonary venous hypertension.

OBJECTIVES: End-stage lung disease from primary pulmonary hypertension (PPHTN) and pulmonary venous-occlusive disease (PVOD) may require lung transplantation (LT). While medical therapies exist for the palliation of PPHTN, no therapies exist for PVOD. The study's objective is to compare outcomes of LT in these patients. METHODS: Patients with PPHTN and PVOD who had undergone LT were identified in the UNOS database (2005-2022). Univariable analyses compared differences between groups in demographic, clinical, and post-transplant outcomes. Multivariable logistic regression examined the association between the diagnosis group and survival. Overall survival time between groups was compared using the Kaplan-Meier method. RESULTS: Six hundred and ninety-six PPHTN and 78 PVOD patients underwent LT during the study period. Patients with PVOD had lower pulmonary artery mean pressure (47 vs. 53 mmHg, p < .001), but higher cardiac output (4.51 vs. 4.31 L/min, p = .04). PVOD patients were more likely to receive lungs from donation after cardiac death donors (7.7 vs. 2.9%, p = .04). There were no differences in postoperative complications or length of stay. PVOD was associated with superior survival at 30-day (100 vs. 93%, p = .02) and 90-day post-transplant (93 vs. 83%, p = .03), but not at later time points. In multivariable analyses, PVOD and brain death donor use were associated with better survival up to 90-day mark. CONCLUSIONS: Patients undergoing LT for PVOD had better initial survival, which disappeared after 1 year of transplantation. Donation after circulatory death donor use had a short-term survival disadvantage.

Influence of cardiac diagnosis on outcomes of pediatric heart-lung transplantation.

OBJECTIVE: Pediatric Heart-lung transplant (HLTX) is performed for endstage congenital heart disease (CHD) with irreversible pulmonary hypertension or non-congenital heart disease (NCHD) with end-stage heart and lung disease. CHD could influence the outcomes of HTLX due to increased complexity of the operation as compared to NCHD. In this study we evaluated the influence of cardiac diagnosis on outcomes of pediatric HTLX. METHODS: The UNOS database (1987-2022) was queried for primary HTLX in patients <18 years. The data were extracted for demographics, pretransplant characteristics, post-transplant outcomes, and analyzed for the impact of cardiac diagnosis on post-transplant outcomes. Standard statistical tests were used. Survival was compared using the Kaplan-Meier method. RESULTS: Ninety of the 213 patients who underwent HLTX had CHD. There were no demographic differences. Heart listing status was similar but with a higher LAS score for NCHD. NCHD had higher pre-operative life support use (mechanical ventilation, inotropes or dialysis) but the use of ECMO as a bridge to transplantation was similar. Wait-list times were longer for CHD. The ischemic times were similar. Post-transplant dialysis, stroke, prolonged mechanical ventilation, and rejection were similar. Survival at 30-days, 1-year, and long-term survival at 17 years was similar. Non-survivors at 30-days post-transplant were on life support, used ECMO as a bridge, had lower wait-list times, longer ischemic times and had strokes. Non-survivors at 1-year had similar factors in addition to a higher dialysis use. CONCLUSION: Cardiac diagnosis had no impact on outcomes after Pediatric HLTX. Patients on life support or ECMO before transplantation were transplanted faster but with lower survival.

Heart transplantation for pediatric foreign nationals in the United States.

BACKGROUND: This study aimed to clarify survival outcomes, waitlist mortality, and waitlist days of heart transplantation of pediatric foreign nationals compared to pediatric United States (US) citizens. METHODS: We retrieved data from March 2012 to June 2021 in the United Network Organ Sharing (UNOS) registry. RESULTS: Of 5857 pediatric patients newly waitlisted, 133 (2.27%) patients were non-US citizen/non-US residents (non-citizen non-resident [NCNR]). Patients with congenital heart disease were higher in the US citizen group than in the NCNR group (51.9% vs. 22.6%, p < .001); 76.7% of patients in the NCNR group (102/133) had cardiomyopathy. Of the 133 NCNRs, 111 patients (83.5%) underwent heart transplantation, which was significantly higher than that in the US citizen group (68.6%, p < .001). The median waitlist time was 71 days (IQR, 22-172 days) in the NCNR group and 74 days (29-184 days) in the US citizen group (p = .48). Survival after heart transplant was significantly better in the NCNR group than in the US citizen group (n = 3982; logrank test p = .015). CONCLUSIONS: Heart transplantation for pediatric foreign nationals was mostly indicated for cardiomyopathy, and their transplant rate was significantly higher than that in the US citizen group, with better survival outcomes. The better survival outcomes in the NCNR group compared to the US citizen group can likely be attributed to the differing diagnoses for which transplantation was performed.

Is coronary artery transfer still the jugular for the arterial switch operation?

The outcomes of the arterial switch operation have improved over a period of time with the elimination of coronary artery anatomy as a risk factor for operative mortality in some series. However, cumulatively, when all the series published so far are analyzed, two coronary variations, namely the single sinus coronary artery origin and the intramural type, persist as risk factors for an adverse operative outcome.

Extracorporeal membrane oxygenation (ECMO): Can we do it ourselves?

Extracorporeal membrane oxygenation (ECMO) is a selectively available therapeutic option, generally available in a large-size referral healthcare system. In a single-center experience of the use of venovenous ECMO for COVID-19 ARDS in a medium-size healthcare system during the pandemic, West et al. in their study have convincingly demonstrated that ECMO can become a broadly available therapeutic option without compromising quality.

National outcomes of the Fontan operation with endocardial cushion defect.

BACKGROUND: The traditional outcomes of the Fontan operation (FO) in endocardial cushion defect (ECD) patients have been suboptimal. Previous studies have been limited by the smaller number of ECD patients, longer study period with an era effect, and do not directly compare short-term outcomes of FO in ECD patients with non-ECD patients. Our study aims to address these shortcomings. METHODS: A retrospective analysis of the Kids Inpatient Database (2009, 2012, and 2016) for the FO was done. The groups were divided into those who underwent FO with ECD as compared to non-ECD diagnosis. The data were abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. RESULTS: Three thousand three hundred eighty patients underwent the FO of which 360 patients (11%) were FO-ECD. ECD patients were more likely to have Down syndrome, Heterotaxy syndrome, transposition/DORV, and TAPVR as compared to non-ECD patients. FO-ECD had a higher discharge-mortality (2.84% vs. 0.45%, p = .04). The length of stay (16 vs. 13 days, p = .05) and total charges incurred ($283, 280 vs. $234, 106, p = .03) for the admission were higher in the FO-ECD as compared to non-ECD patients. In multivariable analysis, ECD diagnosis, cardiac arrest, acute kidney injury, and postoperative hemorrhage were predictors of mortality. CONCLUSION: Contemporary outcomes for FO are excellent with very low overall operative mortality. However, the outcomes in ECD patients are inferior with higher operative mortality than in non-ECD patients. The occurrence of postoperation complications and a diagnosis of ECD were predictive of a negative outcome.

National Fontan Operation short-term outcomes at or below 2-years-of-age compared to older than 2-years-of-age.

INTRODUCTION: Opinion is divided about optimal early timing of the Fontan Operation (FO). While some studies have suggested 3 years-of-age, others have shown good outcomes below 2 years-of-age. We analyzed the impact of age ≤2 years as compared age >2 years on short-term outcome of the FO using a large national database. METHODS: A retrospective analysis of the Kids Inpatient Database (2009-16) for the FO was done. The groups were divided into those who underwent FO at age ≤2 years (Early FO [EF]) as compared to age >2 years (Late FO [LF]). The data was abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. RESULTS: A total of 3381 patients underwent FO during this period of which 1482 (44%) were EF. The mean ages of the EF and LF were 1.6 and 4.3, respectively (p < .001). LF were more likely to be non-White, female, and have Heterotaxy syndrome. HLHS was more common in EF. There was no difference in the discharge mortality, length of stay, disposition (majority went home), and mean total charges incurred. The overall discharge mortality was low at 0.7% (24/3381). In multivariate analysis: cardiac arrest, acute kidney injury, mechanical ventilation >96 h, endocardial cushion defect and non-White ethnicity were predictors of a mortality and not age. CONCLUSION: Contemporary outcomes for FO are excellent with equivalent short-term outcomes in both the age groups. Occurrence of postoperative complications, non-White ethnicity and endocardial cushion defect diagnosis were predictive of a negative outcome.

Impact of occurrence of cardiac arrest in the donor on long-term outcomes of pediatric heart transplantation.

OBJECTIVE: The impact of cardiac arrest in the donor on long-term outcomes of pediatric heart transplantation has not been studied. METHODS: The UNOS database was queried for primary pediatric heart transplantation (1999-2020). The cohort was divided into recipients who received a cardiac allograft from a donor who had a cardiac arrest (CA) versus a donor who did not (NCA). Univariable and multivariable analysis was done to compare recipient outcomes, followed by survival analysis using the Kaplan-Meier method. RESULTS: A total of 7300 patients underwent heart transplantation, of which 579 (7.9%) patients belonged to the CA group. The CA group was younger (median 3 vs. 5 years, p < .001), male (51% vs. 47%, p = .03), and smaller in weight (13 vs. 17 kg, p < .001) and height (101 vs. 109 cm, p < .001) than the NCA group. The groups were similar in recipient heart failure diagnosis and blood type. The CA donors were younger (3 vs. 6 years, p < .001) versus nonwhite (48% vs. 45%, p = .003) and died from drowning and asphyxiation compared to blunt injury and intracranial hemorrhage in the NCA group. The left-ventricular ejection fraction was similar between the groups. There was no difference in VAD and ECMO use before the transplant. The listing status, waitlist days, and allograft ischemic times were similar. Posttransplant morbidity such as stroke, dialysis, pacemaker implantation, and treated rejection were similar. Donor cardiac arrest (hazard ratio = 0.93, p = .5) was not an independent predictor of mortality on multivariable analysis. There was no survival difference even beyond 20 years of follow-up between the groups (p = .88). CONCLUSION: The occurrence of donor cardiac arrest has no impact on long-term survival in pediatric heart transplant recipients.

Virtual endoluminal aortic root views determined at coronary CT angiography - an important tool for improving anomalous coronary artery visualization and surgical planning.

Anomalous origin of the coronary arteries, though uncommon, is of great clinical concern. It can be the cause of sudden cardiac death and abnormal cardiac hemodynamics. Advances in electrocardiographically (ECG)-gated multi-detector CT have increased diagnostic accuracy in detecting anomalous origin of coronary arteries and their interarterial and intramural courses. Recent advances in multi-detector CT image processing software have allowed the creation of virtual endoluminal views of the aortic root and improved assessment of the intramural course (the length and relationship to the intercoronary commissure) of the coronary artery, which is of considerable surgical importance. We review our experience with virtual endoluminal imaging in our first 19 cases of interarterial coronary artery anomalies (17 cases of interarterial with intramural segment and 2 cases of purely interarterial course) diagnosed preoperatively and proven surgically.

Sublobar resection is equivalent to lobectomy for T1a non-small cell lung cancer in the elderly: a Surveillance, Epidemiology, and End Results database analysis.

BACKGROUND: Anatomic lobectomy with mediastinal lymph node dissection is considered the optimal management for early stage non-small cell lung cancer (NSCLC). Limited lung resection may be preferable in the elderly population, who are more likely to have poor pulmonary reserve and multiple comorbidities. Our primary objective was to compare the survival of patients aged ≥ 75 y who underwent sublobar resection or lobectomy for stage IA NSCLC. MATERIALS AND METHODS: We queried the Surveillance, Epidemiology, and End Results database for patients aged ≥ 75 y who were diagnosed with stage IA NSCLC from 1998-2007. Patients were divided into three groups based on the type of surgery performed (wedge resection, segmentectomy, and lobectomy). Kaplan-Meier analysis and Cox proportional hazard model were used for survival analysis. RESULTS: A total of 1640 patients were analyzed. Lobectomy was performed in 1051 patients, 119 underwent segmentectomy, and 470 patients had wedge resection. Overall and cancer-specific survival were significantly lower in the wedge resection group as compared with those in lobectomy (P < 0.05). However, for T1a tumors, no significant difference was found in risk adjusted 5-y cancer-specific survival for patients who underwent wedge resection, segmentectomy (hazard ratio, 1.009; 95% confidence interval 0.624-1.631; P = 0.972), or lobectomy (hazard ratio, 0.98; 95% confidence interval, 0.691-1.388; P = 0.908). CONCLUSIONS: Sublobar resection is not inferior to lobectomy for T1a N0 M0 NSCLC in the elderly and should be considered a viable alternative in this high-risk population.

Partial upper median sternotomy for anterior aortopexy for innominate artery compression syndrome: a case series.

Background: Innominate artery compression syndrome (IAS) is caused by an abnormally originating innominate artery compressing the trachea anteriorly. One option to relieve such compression is an anterior aortopexy (AA). We describe our technique of an AA via a partial upper median sternotomy. Case Description: Nine consecutive patients underwent AA for IAS via a partial upper median sternotomy from July 2017 to November 2020 at two US teaching hospitals. The median age was 9 months [interquartile range (IQR), 3-16.5 months]. The male to female ratio was 1.25. All patients had >70% compression by flexible bronchoscopy. Two patients had previous surgeries. The median follow-up was 6 months (IQR, 4-8.5 months). The indications for the operation were: acute life-threatening events (ALTEs) (4/9 patients), recurrent intubation (4/9), and severe stridor with >70% luminal reduction (1/9). Technical success (defined as ≤20% residual stenosis) was achieved in 78% (7/9) of the patients. The two patients with unsuccessful AAs required either a tracheal resection or an innominate artery reimplantation. Both achieved full symptom resolution. Overall, 78% (7/9) of patients experienced full symptom resolution. Of the two patients without full symptom resolution, one had mild stridor at 6 months post-operation. The other patient without full resolution is awaiting further vocal cord surgery for an associated glottic pathology. Conclusions: A partial upper sternotomy provides a very versatile approach to an AA for IAS. In addition to facilitating an adequate AA, a partial upper sternotomy provides options for direct tracheal surgery or an innominate artery reimplantation in case an optimal result is not obtained by an AA.

A systematic review of transesophageal echocardiography-induced esophageal perforation.

A systematic review of transesophageal echocardiography (TEE)-induced esophageal perforation was done using the MEDLINE (PubMed and OVID interfaces), Google Scholar and EMBASE databases. Thirty-five cases of esophageal perforation from 22 studies were analyzed. TEE-induced esophageal perforation occurs in elderly female patients, predominantly in an intra-operative setting. Thoracic esophagus is the most commonly involved segment, especially, when TEE is performed intra-operatively. Majority of the esophageal perforations occur in cases with a perceived low risk or smooth TEE exam and thus, screening for high risk factors may not eliminate the occurrence of a perforation. A delayed detection of perforation occurs when it is a thoracic esophageal perforation, performed intra-operatively and when there are no known preoperative risk factors. Shock during the detection of the perforation is associated with mortality. Majority of the perforations can be repaired primarily.

Median Sternotomy for Innominate Artery Compression Syndrome and Distal Tracheal Stenosis.

We present a case of a premature infant who had an initial diagnosis of an innominate artery compression syndrome. This was approached by a median sternotomy for an aortopexy. However, the patient was found to have a distal tracheal stenosis due to a tracheal cartilage deficiency and was treated by a tracheal resection and primary anastamosis.

HeartMate 3 VAD in a Single-Ventricle Patient Palliated With a Superior Cavopulmonary Anastomosis.

We describe a 9-year-old, 25-kg child with failing single-ventricle palliation with a superior cavopulmonary anastomosis successfully supported with a HeartMate 3 ventricular assist device (Abbott) as a bridge to transplantation.

Outcomes of heart-lung transplantation in Eisenmenger syndrome compared to primary pulmonary hypertension.

OBJECTIVE: Heart-Lung Transplantation (HLTX) is required both in primary pulmonary hypertension (PPH) and Eisenmenger syndrome (ES) when there is associated end-stage heart disease. Although PPH is associated with an otherwise structurally normal heart, ES is associated with congenital heart defects, which may increase the complexity of the operation. This study analyzes if the diagnosis (PPH vs. ES) is related to short-term outcomes after HLTX. METHODS: Patients ≥18 years of age with PPH and ES who underwent HLTX were identified in the United Network for Organ Sharing database from 2005 to 2021. Patients were propensity score matched on heart and lung listing status at the time of transplant. Univariable, multivariable, and Kaplan-Meir survival analyses were performed. RESULTS: The unmatched cohort had 128 PPH and 44 ES patients, and the matched cohort had 44 patients in each group. PPH patients had lower waitlist times and PA pressures but higher FEV1, heart, and lung listing status and ECMO bridge. There were no differences in immediate postoperative outcomes such as dialysis, stroke, and airway dehiscence. PPH patients had a higher treatment rejection in the first year. The 30-day, 1-year, and 3-year survival were better in the PPH group. However, a landmark analysis excluding deaths within 30 days eliminated differences in survival between the groups. Post-transplant dialysis and postoperative mechanical ventilation >5 days were risk factors for 1-year mortality in ES. CONCLUSION: The short-term outcomes of HLTX are inferior in ES compared to PPH and much of the attrition in ES occurs in the immediate postoperative period.

Early Concerning Outcomes for the Edwards Inspiris Resilia Bioprosthesis in the Pulmonary Position.

BACKGROUND: The Inspiris Resilia (Edwards Lifesciences LLC) bioprosthesis has gained widespread use in the aortic position; however no robust data are available about its use in the pulmonary position. METHODS: We reviewed our outcomes for the Inspiris Resilia in the pulmonary position between August 2019 and October 2021. RESULTS: Twenty-seven patients (15 female patients [56%]; mean age, 22.26 ± 14.99 years) were included. The most common original pathology was tetralogy of Fallot (13 patients, 48%). Five patients (18.5%) had prior transcatheter interventions. The mean right ventricular end-diastolic volume index was 164.25 ± 45.1 mL/m2. Sternotomy or repeat sternotomy was the most common approach (21 patients, 77.8%). The standard technique for pulmonary valve replacement was used in 22 patients (81.5%), whereas the prosthesis was implanted as a conduit in the remaining 5 (18.5%). Trivial to mild prosthetic regurgitation was present in 6 patients (22.2%) at the time of discharge. There was no early mortality. Follow-up was complete in all patients (mean, 16 ± 8 months), with no late mortality or late reoperations. New prosthetic regurgitation developed in 13 patients (48%), all of whom underwent replacement with the standard surgical technique. No regurgitation occurred in the conduit cases. This progressed to moderate regurgitation in 6 patients (22%) and severe in 3 (11%). Three patients (11%) underwent transcatheter valve-in-valve after their surgical pulmonary valve replacement. CONCLUSIONS: The early data regarding the Inspiris Resilia bioprosthesis use in the pulmonary position is concerning. The prosthesis design may not be suitable for low-pressure circulation, or modification of its implantation technique may be needed.