Search

Background: The treatment of neonates with unrepairable heart valve dysfunction remains an unsolved problem because there are no growing heart valve replacements. Heart valve transplantation is a potential approach to deliver growing heart valve replacements. Therefore, we retrospectively analysed the semilunar valve function of orthotopic heart transplants during rejection episodes. Methods: We included children who underwent orthotopic heart transplantation at our institution and experienced at least one episode of rejection between 1/1/2010 and 1/1/2020. Semilunar valve function was analysed using echocardiography at baseline, during rejection and approximately 3 months after rejection. Results: Included were a total of 31 episodes of rejection. All patients had either no (27) or trivial (4) aortic insufficiency prior to rejection. One patient developed mild aortic insufficiency during a rejection episode (P = 0.73), and all patients had either no (21) or trivial (7) aortic insufficiency at follow-up (P = 0.40). All patients had mild or less pulmonary insufficiency prior to rejection, which did not significantly change during (P = 0.40) or following rejection (P = 0.35). Similarly, compared to maximum pressure gradients across the valves at baseline, which were trivial, there was no appreciable change in the gradient across the aortic valve during (P = 0.50) or following rejection (P = 0.42), nor was there any meaningful change in the gradient across the pulmonary valve during (P = 0.55) or following rejection (P = 0.91). Conclusions: This study demonstrated that there was no echocardiographic evidence of change in semilunar valve function during episodes of rejection in patient with heart transplants. These findings indicate that heart valve transplants require lower levels of immune suppression than orthotopic heart transplants and provide partial foundational evidence to justify future research that will determine whether heart valve transplantation may deliver growing heart valve replacements for children.

Persistent left superior vena cava (PLSVC) draining to the left atrium (LA) is a rare congenital abnormality. PLSVC usually drains into the right atrium via the coronary sinus. In 20% of patients, however, it can connect to the LA [1]. Left atrial drainage occurs through the LA appendage, left pulmonary veins, or the coronary sinus. Patients are often asymptomatic as the left atrial drainage does not result in a significant right to left shunt. As such, the abnormality is often discovered incidentally on imaging. PLSVC is often described in conjunction with other congenital anomalies, such as atrial septal defect (ASD), tetralogy of fallot, and coarctation of the aorta [2]. When PLSVC draining to the LA is present in the setting of an ASD, it is classified as Raghib’s syndrome. However, this case describes a PLSVC draining to the LA without an ASD or a right SVC, a presentation which has never before been described.

Background The Ross operation is the preferred treatment for aortic valve replacement in children. However previous studies indicate that outcomes in neonates are poor. This meta-analysis examines the pooled outcomes of the Ross operation in neonates. Methods Four major databases (PubMed/MEDLINE, EMBASE, Scopus, and ScienceDirect) were searched from inception until May 1, 2020 for studies describing outcomes of the Ross operation in neonates. The primary outcome was early mortality, and secondary outcomes were late mortality and mechanical support. Random-effects models were used to account for possible heterogeneity between studies, and continuity corrections were used to include zero total event trials. Results Eighteen studies comprising outcomes data on 181 neonates were included in the analysis. Meta-analysis showed a pooled early mortality rate of 24% (95% confidence interval, 12%-38%; I2 = 52%, P for heterogeneity = .01). Meta-regression analysis showed that more recently published studies reported significantly worse early mortality (P = .03). The pooled incidence of postoperative mechanical support was 15% (95% confidence interval, 5%-28%; I2 = 28%, P for heterogeneity = .22). No evidence of publication bias was found according to Egger’s test (bias coefficient = 0.21, P = .57). Conclusions The neonatal Ross operation carries a high early mortality rate. The treatment of unrepairable aortic valves in neonates remains an unsolved problem in congenital cardiac surgery.

BACKGROUND Postoperative pericardial adhesions have been associated with increased morbidity, mortality, and surgical difficulty. Barriers exist to limit adhesion formation, yet little is known about their use in cardiac surgery. The study presented here provides the first major systematic review of adhesion barriers in cardiac surgery. METHODS Scopus and PubMed were assessed on November 20, 2020. Inclusion criteria were clinical studies on human subjects, and exclusion criteria were studies not published in English and case reports. Risk of bias was evaluated with the Cochrane Risk of Bias Tool. Barrier efficacy data was assessed with Excel and GraphPad Prism 5. RESULTS Twenty-five studies were identified with a total of 13 barriers and 2928 patients. Polytetrafluoroethylene (PTFE) was the most frequently evaluated barrier (13 studies, 67% of patients) with adhesion formation rate of 37.31% and standardized tenacity score of 26.50. Several barriers had improved efficacy. In particular, Cova CARD had a standardized tenacity score of 15.00. CONCLUSIONS Overall, the data varied considerably in terms of study design and reporting bias. The amount of data was also limited for the non-PTFE studies. PTFE has historically been effective in preventing adhesions. More recent barriers may be superior, yet the current data is nonconfirmatory. No ideal adhesion barrier currently exists, and future barriers must focus on the requirements unique to operating in and around the heart.

The recommended method of cardiopulmonary resuscitation (CPR) has been closed-chest cardiac compressions, but the development of CPR adjunctive devices has called into question the efficacy and role of these adjunctive devices. In this review, we provide a comprehensive evaluation and discussion on the commercially available noninvasive CPR adjuncts used during out-of-hospital cardiac arrest (OHCA).We review the three most common CPR adjunctive devices: the piston mechanism, the load distributing band, and the impedance threshold device. All three CPR adjunctive devices have preclinical data to support their use during cardiac arrest. In clinical trials, limited data show improvement in survival and neurologic recovery for these devices, and there is insufficient high-level evidence to support their use over manual chest compressions. However, there is a role for them when adequate manual chest compressions are not feasible.The commercially available CPR adjuncts do not consistently show improved outcomes in the literature. There is still a need for research and development into innovative solutions to improve OHCA survival and neurologic recovery. Efforts focused on increasing the speed of CPR initiation and increasing perfusion to the cerebral and coronary vasculature have the potential to advance resuscitative practices.

Background Norwood palliation for hypoplastic left heart syndrome typically results in retrograde perfusion of the native aortic root. This may predispose to native aortic root thrombosis (NART). Methods Patients who underwent stage 1 Norwood palliation at Children’s Hospital Colorado between 2003 and 2019 were retrospectively reviewed for NART. Additional patients were identified by a systematic literature review. Patient factors, operative details, presentation, diagnostic methods, treatments, and outcomes were analyzed. Results Three patients with NART were identified among 241 patients who had undergone stage 1 Norwood palliation at Children’s Hospital Colorado (mean follow-up, 48 months). A fourth patient identified at Children’s Hospital Colorado had undergone stage 1 palliation elsewhere. The systematic literature review identified 12 additional patients. Among the 16 patients, patients presented with new arrhythmias (38% [n = 6]), evidence of ischemia (31% [n = 5]), incidentally (19% [n = 3]), inability to wean from postoperative support (6% [n = 1]), and cardiac arrest (6% [n = 1). The diagnosis was made by transthoracic echocardiography (TTE) in 50% (n = 8), transesophageal echocardiography in 19% (n = 3), cardiac catheterization in 25% (n = 4), and cardiac computed tomography in 6% (n = 1). Importantly, 44% (n = 7) of patients were missed by TTE and subsequently diagnosed by other methods. Treatment strategies were surgical thrombectomy in 38% (n = 6), anticoagulation alone in 38% (n = 6), direct catheter thrombolysis in 19% (n = 3), and systemic thrombolysis in 6% (n = 1). The rate of mortality or transplant listing was 44% (n = 7). Conclusions NART is an underappreciated complication of Norwood palliation with high mortality. There should be a high index of suspicion for NART in patients after Norwood palliation with an unexplained complicated clinical course. Importantly, NART is frequently missed by TTE.

Pigs, or Sus scrofa domestica, are commonly used animal models in translational transplantation research due to their anatomical, physiological, and immunological similarities to humans. In solid organ transplantation studies, immunosuppressive medications may be administered to pigs to prevent rejection. We provide an overview of the immunosuppressive regimens used in allogeneic solid organ transplantation in pigs, including heart, lung, kidney, bowel and cotransplanted organs and focus on the use of tacrolimus, mycophenolate mofetil, and corticosteroids.

Smart materials have intrinsic properties that change in a controlled fashion in response to external stimuli. Currently, the only smart materials with a significant clinical impact in cardiovascular implant design are shape memory alloys, particularly Nitinol. Recent prodigious progress in material science has resulted in the development of sophisticated shape memory polymers. In this article, we have reviewed the literature and outline the characteristics, advantages, and disadvantages of shape memory alloys and shape memory polymers which are relevant to clinical cardiovascular applications, and describe the potential of these smart materials for applications in coronary stents and transcatheter valves.

Children undergoing congenital cardiac surgery often outgrow the valve implants. These children are thus committed to morbid reoperations for successive exchanges of the vavular implants that they have outgrown. Therefore the holy grail of congenital cardiac surgery is a valve implant that grows with the recipient child. Preserved homografts routinely are used as valve implants, but they do not grow as the child grows because they lose viability during preservation. In contrast, pulmonary autografts and pediatric heart transplants grow with the recipient children. Similarly, partial heart transplantation can deliver growing valve implants for congenital cardiac surgery. Temporary immune suppression would only be needed until the partial heart transplant can be exchanged for an adult-sized prosthetic valve in the grown child.

The rat is an important model organism for lung transplantation research. Orthotopic rat lung transplantation is a complex procedure, which requires advanced microsurgical techniques. This technical paper describes in detail highly reproducible intraoperative microsurgical techniques and peri-operative management, including the donor procedure, recipient anesthetic management, the recipient operation and anticipated peri-operative complications.

There is an urgent clinical need for heart valve replacements that can grow in children. Heart valve transplantation is proposed as a new type of transplant with the potential to deliver durable heart valves capable of somatic growth with no requirement for anticoagulation. However, the immunobiology of heart valve transplants remains unexplored, highlighting the need for animal models to study this new type of transplant. Previous rat models for heterotopic aortic valve transplantation into the abdominal aorta have been described, though they are technically challenging and costly. For addressing this challenge, a renal subcapsular transplant model was developed in rodents as a practical and more straightforward method for studying heart valve transplant immunobiology. In this model, a single aortic valve leaflet is harvested and inserted into the renal subcapsular space. The kidney is easily accessible, and the transplanted tissue is securely contained in a subcapsular space that is well vascularized and can accommodate a variety of tissue sizes. Furthermore, because a single rat can provide three donor aortic leaflets and a single kidney can provide multiple sites for transplanted tissue, fewer rats are required for a given study. Here, the transplantation technique is described, providing a significant step forward in studying the transplant immunology of heart valve transplantation.

OBJECTIVE: The Pediatric Heart Network Collaborative Learning Study (PHN CLS) used collaborative learning strategies to implement a clinical practice guideline (CPG) that increased rates of early extubation (EE) after infant repair of tetralogy of Fallot and coarctation of the aorta. We assessed EE rates for infants undergoing cardiac surgeries not targeted by the CPG to determine whether changes in extubation practices spilled over to care of other infants. DESIGN: Observational analyses of site’s local Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and Pediatric Cardiac Critical Care Consortium (PC4) Registry SETTING: Four PHN CLS active site hospitals PATIENTS: Infants undergoing ventricular septal defect repair, atrioventricular septal defect repair, or superior cavopulmonary anastomosis (lower complexity), and arterial switch operation or isolated aortopulmonary shunt (higher complexity) INTERVENTIONS: None MEASUREMENTS AND MAIN RESULTS: Aggregate outcomes were compared between the 12 months pre-CPG and 12 months after study completion (Follow Up). In infants undergoing lower complexity surgeries, EE increased during Follow Up compared with Pre CPG (30.2% vs 18.8%, p = .006), and hours to initial postoperative extubation decreased. We observed variation in these outcomes by surgery type, with only VSD repair associated with a significant increase in EE during Follow Up compared with Pre CPG (47% vs 26%, p = 0.006). Variation by study site was also seen, with only one hospital showing an increase in EE. In patients undergoing higher complexity surgeries there was no difference in EE or hours to initial extubation between the study eras. CONCLUSIONS: We observed spillover of extubation practices promoted by the CLS CPG to lower complexity operations not included in the original study that was sustainable 1 year after study completion, though this effect differed across sites and operation subtypes. No changes in postoperative extubation outcomes following higher complexity surgeries were seen. The significant variation in outcomes by site suggests that center specific factors may have influenced spillover of CPG practices.

BACKGROUND: Postoperative pericardial adhesions have been associated with increased morbidity, mortality, and surgical difficulty. Barriers exist to limit adhesion formation, yet little is known about their use in cardiac surgery. The study presented here provides the first major systematic review of adhesion barriers in cardiac surgery. METHODS: Scopus and PubMed were assessed on November 20, 2020. Inclusion criteria were clinical studies on human subjects, and exclusion criteria were studies not published in English and case reports. Risk of bias was evaluated with the Cochrane Risk of Bias Tool. Barrier safety and efficacy data were assessed with Excel and GraphPad Prism 5. RESULTS: 25 studies were identified with a total of 13 barriers and 2,928 patients. Polytetrafluoroethylene (PTFE) was the most frequently evaluated barrier (13 studies, 67% of patients) with an infection rate of 1.14%, bleeding event rate of 0.75%, mortality rate of 1.22%, adhesion formation rate of 37.31%, and standardized tenacity score of 26.50. Several barriers had improved safety and efficacy. In particular, Cova CARD had an infection rate of 0.00%, a bleeding event rate of 0.00%, and a tenacity score of 15.00. CONCLUSIONS: Overall, the data varied considerably in terms of study design and reporting bias. The amount of data was also limited for the non-PTFE studies. PTFE has historically been effective in preventing adhesions. More recent barriers may be superior, yet the current data is non-confirmatory. No ideal adhesion barrier currently exists, and future barriers must focus on the requirements unique to operating in and around the heart.

Purpose: Fluid overload is a common post-operative issue in children following cardiac surgery and is associated with increased morbidity and mortality. There is currently no gold standard for evaluating fluid status. We sought to validate the use of point-of-care ultrasound to measure skin edema in infants and assess the intra- and inter-user variability.Methods: Prospective cohort study of neonates (≤30 d/o) and infants (31 d/o to 12 m/o) undergoing cardiac surgery and neonatal controls. Skin ultrasound was performed on four body sites at baseline and daily post-operatively through post-operative day (POD) 3. Subcutaneous tissue depth was manually measured. Intra- and inter-user variability was assessed using intraclass correlation coefficient (ICC).Results: Fifty control and 22 surgical subjects underwent skin ultrasound. There was no difference between baseline surgical and control neonates. Subcutaneous tissue increased in neonates starting POD 1 with minimal improvement by POD 3. In infants, this pattern was less pronounced with near resolution by POD 3. Intra-user variability was excellent (ICC 0.95). Inter-user variability was very good (ICC 0.82).Conclusion: Point-of-care skin ultrasound is a reproducible and reliable method to measure subcutaneous tissue in infants with and without congenital heart disease. Acute increases in subcutaneous tissue suggests development of skin edema, consistent with extravascular fluid overload. There is evidence of skin edema starting POD 1 in all subjects with no substantial improvement by POD 3 in neonates. Point-of-care ultrasound could be an objective way to measure extravascular fluid overload in infants. Further research is needed to determine how extravascular fluid overload correlates to clinical outcomes.

Congenital heart defects are the most common types of birth defects in humans. Children with congenital heart defects frequently require heart valve replacement with an implant. Unfortunately, conventional heart valve implants do not grow. Therefore, these children are committed to serial re-operations for successively larger implant exchanges. Partial heart transplantation is a new and innovative approach to deliver growing heart valve implants. However, the transplant biology of partial heart transplant grafts remains unexplored. This is a critical barrier for clinical translation. Therefore, we investigated the cellular viability of partial heart transplants in cold storage. Histology and immunohistochemistry revealed no morphological differences in heart valves after 6, 24, or 48 hours of cold storage. Moreover, immunohistochemistry showed that the marker for apoptosis activated caspase 3 and the marker for cell division Ki67 remained unchanged after 48 hours of cold storage. Finally, quantification of fluorescing resorufin showed no statistically significant decrease in cellular metabolic activity in heart valves after 48 hours of cold storage. We conclude that partial heart transplants remain viable after 48 hours of cold storage. These findings represent the first step towards translating partial heart transplantation from the bench to the bedside because they have direct clinical implications for the procurement logistics of this new type of transplant.

Pulmonary metastasectomy is a well-established contribution to the cure of oligometastatic cancers, but its exact effectiveness is poorly understood. Here we report the outcomes of repeat pulmonary metastasectomy from a multicenter trial. This retrospective study included patients who underwent re-do metastasectomies between January 2010 and December 2014. The exclusion criterion was metastasectomy without curative intent. We reviewed medical files of 621 consecutive patients who underwent initial pulmonary metastasectomy. Of those, 64 patients underwent repeat metastasectomies, and these patients were included in the analysis. All the 64 patients underwent a second metastasectomy, later 35 of them underwent a third metastasectomy, 12 underwent a fourth metastasectomy, and 6 underwent a fifth metastasectomy. The total number of re-do metastasectomies was 181. The median overall survival among the patients undergoing re-do metastasectomy was 66.0 ± 3.8 months. Three and 5-year survival rates were 82.3% and 63.3%, respectively. The 5-year survival rates were 63.3% after the first, 50.9% after the second, 74.4% after the third, 83.3% after the fourth, and 60.0% after the fifth metastasectomy. We conclude that at the current stage of knowledge, there is an indication for repeat re-do metastasectomy with curative intent.

Decellularized native extracellular matrix (ECM) biomaterials are widely used in tissue engineering and have reached clinical application as biomesh implants. To enhance their regenerative properties and postimplantation performance, ECM biomaterials could be functionalized via immobilization of bioactive molecules. To facilitate ECM functionalization, we developed a metabolic glycan labeling approach using physiologic pathways to covalently incorporate click-reactive azide ligands into the native ECM of a wide variety of rodent tissues and organs in vivo, and into the ECM of isolated rodent and porcine lungs cultured ex vivo. The incorporated azides within the ECM were preserved after decellularization and served as chemoselective ligands for subsequent bioconjugation via click chemistry. As proof of principle, we generated alkyne-modified heparin, immobilized it onto azide-incorporated acellular lungs, and demonstrated its bioactivity by Antithrombin III immobilization and Factor Xa inhibition. The herein reported metabolic glycan labeling approach represents a novel platform technology for manufacturing click-reactive native ECM biomaterials, thereby enabling efficient and chemoselective functionalization of these materials to facilitate tissue regeneration and repair.

ABO-incompatible heart transplant is a method to increase the infant donor pool. However data on long-term survival and rejection after ABO-incompatible heart transplant in recent era are limited.The United Network for Organ Sharing database was queried for infant heart transplants performed from January 2008 to March 2020. Patient demographics and known risk factors for posttransplant mortality were collected. Statistical analysis using Bayesian additive regression trees was performed to evaluate the association of ABO incompatibility and overall survival, graft survival, acute rejection episodes, and length of stay.Of 1368 included infants (age1 year), 280 (20.47%) were ABO incompatible. ABO incompatibility was not associated with increased all-cause mortality, acute rejection episodes, or length of stay, whereas extracorporeal membrane oxygenation and intubation status of the recipient at the time of transplantation were associated with increased all-cause mortality and graft failure. Idiopathic cardiomyopathy was associated with a decreased likelihood of posttransplant all-cause mortality. One-, 5-, and 10-year survival rates among compatible vs incompatible transplants were estimated to be 90% vs 88%, 82% vs 79%, and 77% vs 73%, respectively.ABO-incompatible infant heart transplant does not affect posttransplant survival, incidence of rejection, or postoperative length of stay. Therefore it remains a viable and important strategy to increase the infant donor pool.

Pulmonary metastasectomy is a well-established contribution to the cure of oligometastatic cancers, but its exact effectiveness is poorly understood. Here we report the outcomes of repeat pulmonary metastasectomy from a multicenter trial. This retrospective study included patients who underwent re-do metastasectomies between January 2010 and December 2014. The exclusion criterion was metastasectomy without curative intent. We reviewed medical files of 621 consecutive patients who underwent initial pulmonary metastasectomy. Of those, 64 patients underwent repeat metastasectomies, and these patients were included in the analysis. All the 64 patients underwent a second metastasectomy, later 35 of them underwent a third metastasectomy, 12 underwent a fourth metastasectomy, and 6 underwent a fifth metastasectomy. The total number of re-do metastasectomies was 181. The median overall survival among the patients undergoing re-do metastasectomy was 66.0 ± 3.8 months. Three and 5-year survival rates were 82.3% and 63.3%, respectively. The 5-year survival rates were 63.3% after the first, 50.9% after the second, 74.4% after the third, 83.3% after the fourth, and 60.0% after the fifth metastasectomy. We conclude that at the current stage of knowledge, there is an indication for repeat re-do metastasectomy with curative intent.

Once pulmonary disease progresses to end-stage pulmonary disease, treatment options are very limited. An important advance in the field is the development of a bioartificial lung derived from a generic matrix scaffold populated with patients' own cells. Significant progress has already been made in the engineering of bioartificial lungs.This review explains how previous and current research contributes to the goal of creating a successful bioartificial lung, and the barriers faced in doing so. We will also highlight some of the design considerations being explored to optimize bioartificial lungs and considerations for clinical translation.While current bioartificial lungs are able to provide short-term gas exchange in large-animal studies, much work is still required to combine the disciplines of cell biology, materials science, and tissue engineering to create such clinically useful and functioning artificial lungs.

Background Approximately 250,000 heart valve operations are performed annually worldwide. An intensive research and development effort has led to progressively more advanced heart valve prostheses. The Carpentier-Edwards Perimount Magna Ease (CEPME) prosthesis represents the latest iteration of the Edwards Perimount series of aortic tissue valves. The current study aims to evaluate the midterm performance of this bioprosthesis. Methods Five hundred and eighteen patients with aortic stenosis underwent aortic valve replacement with the CEPME valve at Papworth Hospital between August 2008 and November 2011. After a minimum of 3 years from the index operation, eligible patients were retrospectively and consecutively recruited to participate. Recruitment was closed after 100 eligible patients had completed all study assessments. Investigations at follow-up included echocardiography, and NYHA status. Primary endpoints included valve performance measures. Results The mean age was 72 years, 64% were male and median follow-up was 5.1 years. NYHA status had improved in 66% of patients. The average postoperative peak and mean pressure gradients decreased by 51.2 mmHg (64.5%) and 31.8 mmHg (59.4%), with a significant improvement in NYHA status. The frequency of moderate aortic regurgitation was 3%. There was no evidence for structural valve deterioration. Conclusions The CEPME has excellent mid-term durability. Its use effectively improves haemodynamics and functional capacity.

Based on Organ Procurement and Transplantation Network data as of December 2019, more than 113 000 patients require an organ transplantation, yet, over the course of this past year, only 36 000 patients received a transplant. This discrepancy between those that need a donor organ and those that receive one remains one of medicine's biggest challenges. Multiple solutions, both biologic and artificial, have been proposed to mitigate this difference. One of the most promising approaches to generate bioartificial organs for transplantation involves re-seeding decellularized scaffolds with appropriate cells. Decellularization involves physical, chemical, or biological methods that typically require intimate contact of various decellularization solutions with each cell. Consequently, conventional submersion decellularization has been limited to simple tissues such as heart valves. The invention of perfusion decellularization was a breakthrough that allowed the generation of tissue-engineered scaffolds from tissues with higher structural organization and entire organs. Such scaffolds are composed of a myriad of extracellular matrix (ECM) components that include collagen, elastin, proteoglycans, and glycoproteins. Together, these components allow the scaffolds to fulfill specialized functions, such as structural functions as well as biological functions including the regulation of cellular processes and extracellular molecules. These specialized functions of decellularized scaffolds can increasingly be harnessed for applications in tissue engineering.

The first lung transplantation in the rat was achieved by Asimacopoulos et al using sutured anastomoses in 1971. Subsequent development of a cuffed technique to construct the anastomoses by Mizuta and colleagues in 1989 represented a breakthrough that resulted in simplification of the procedure and shorter warm ischemic times. Since then, a number of further variations on the technique of rat lung transplantation have been described. In spite of this, the procedure remains technically demanding and involves a long learning curve. This minireview describes the following new technical safeguards to further evolve the technique for cuffed anastomoses in rat lung transplantation: the use of anatomical landmarks to avoid twisting of the everted donor pulmonary vein and bronchus in the cuff, the use of the cuff tie as a landmark to avoid twisting of the anastomotic cuffs relative to the recipient vessels, distal ties on the recipient vessels to achieve a bloodless field and triangulation of the venotomy to avoid pulmonary vein tearing.

Dextrocardia is a rare congenital condition which presents important challenges for surgical management. We discuss a patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome, which ultimately led to decompensated end-stage lung disease and heart-lung transplant. Venous-venous extracorporeal membrane oxygenation was an important strategy to bridge the patient until donor organs became available. Transplantation of a heart-lung block allowed for the treatment of the patient’s underlying congenital heart defect, anatomic reversal of dextrocardia with appropriate venous and arterial connections, and management of pulmonary damage from pulmonary hypertension.