Disease Undergoing Heart Transplantation Research Articles

The Role of 3D-Printing in a Case of Complex Congenital Heart Disease Undergoing Heart Transplantation

<h3>Introduction</h3> Patients with complex congenital heart disease (CHD) who undergo orthotopic heart transplantation (OHT) present technical difficulties due to collateral vessels, unusual anatomy and adhesions. Becoming familiar with the specific anatomy of each individual case prior to surgery is therefore crucial for the surgeon, and all available diagnostic methods should be used before embarking on the procedure. 3D-printing technology may play a pivotal role, as in this case report. <h3>Case Report</h3> A 36-year-old male was listed for OHT in the context of a complex cyanotic CHD consisting of a functional univentricular heart with hypoplasia of the right chambers with tricuspid and pulmonary atresia and an intact ventricular septum, palliated by a right Blalock-Taussig (BT) shunt at the age of 2, a left BT shunt at the age of 6 which was stented in the follow-up, and a bidirectional Glenn shunt when he was 21 years old. To depict this complex anatomy and its spatial relationships, a 3D model was printed and used by the surgical team to enhance their understanding of the patient anatomy and reenact the planned surgery beforehand, enabling the team to predict and avoid possible complications during the act (Figure). In March 2021, the patient underwent elective OHT. During the surgery, multiple MAPCAs were ligated due to diffuse bleeding. The left pulmonary artery (PA) ostial stenosis was resected and then distally anastomosed. In addition, an end-to-end anastomosis of the right PA to the right AP of the recipient was performed, proximal to the Glenn anastomosis, which was repaired with a pericardial patch. Ischemia and CBP time were 245 and 253 minutes. The postoperative course was uneventful with no evidence of bleeding complications. <h3>Summary</h3> 3D-printing should be taken into consideration as a tool for planning surgical interventions in patients with complex CHD listed for OHT. The information provided may be useful to avoid improvisation during the procedure and thus, lead to a decrease in the expected ischemic times.

A Risk Score for Adults With Congenital Heart Disease Undergoing Heart Transplantation

This study derived and validated a risk score for 1-year mortality in patients with adult congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT). The United Network for Organ Sharing registry identified patients with ACHD (≥18 years of age) who underwent OHT between 1987 and 2018. The primary outcome was 1-year mortality. Associated covariates (univariate P< .2) were entered into a multivariable logistic regression model. Variable inclusion in the model was assessed by improvement in the McFadden pseudo-R2, likelihood ratio test, and C-index. A risk score was created using the absolute magnitude of the odds ratios from the derivation cohort, and its ability to predict 1-year mortality was tested in the validation cohort. A total of 1388 recipients were randomly divided into derivation (66.7%, n= 950) and validation (33.3%, n= 438) cohorts. A 13-point risk score incorporating 4 pretransplant variables (age, dialysis dependence, serum bilirubin level, and mechanical ventilation) was created. The predicted 1-year mortality ranged from 14.6% (0 points) to 49.9% (13 points) (P<.001). In weighted regression analysis, there was a strong correlation between predicted 1-year mortality and observed 1-year mortality in the validation cohort (r=0.85, P< .001). Logistic regression also demonstrated a significant association (odds ratio, 1.18; 95% confidence interval, 1.1-1.3; P=.004). The Brier score of the composite score in the validation cohort was 0.14. Kaplan-Meier analysis demonstrated that risk scores of 4 points or higher portended worse survival at 1-year posttransplant (P< .001). This 13-point risk score for ACHD is predictive of mortality within 1 year after OHT and has potential utilization in improving recipient selection for OHT in adult patients with CHD.

Heart Transplantation in Children with Mitochondrial Disease

To compare the outcomes and comorbidities of children with mitochondrial disease undergoing heart transplantation with children without mitochondrial disease. Using a unique linkage between the Pediatric Health Information System and Scientific Registry of Transplant Recipients databases, pediatric heart transplantation recipients from 2002 to 2016 with a diagnosis of cardiomyopathy were included. Post heart transplantation survival and morbidities were compared between patients with and without mitochondrial disease. A total of 1330 patients were included, including 47 (3.5%) with mitochondrial disease. Survival after heart transplantation was similar between patients with and without mitochondrial disease over a median follow-up of 4years. Patients with mitochondrial disease were more likely to have a stroke after heart transplantation (11% vs 3%; P=.009), require a longer duration of mechanical ventilation after heart transplantation (3days vs 1day; P<.001), and have a longer intensive care unit stay after heart transplantation (10 vs 6days; P=.007). The absence of a hospital readmission within the first post-transplant year was similar among patients with and without mitochondrial disease (61.7% vs 51%; P=.14). However, patients with mitochondrial disease who were readmitted demonstrated a longer length of stay compared with those without (median, 14days vs 8days; P=.03). Patients with mitochondrial disease can successfully undergo heart transplantation with survival comparable with patients without mitochondrial disease. Patients with mitochondrial disease have greater risk for post-heart transplantation morbidities including stroke, prolonged mechanical ventilation, and longer intensive care unit and readmission length of stay. These results suggest that the presence of mitochondrial disease shouldnot be an absolute contraindication to heart transplantation in the appropriate clinical setting.

Rejection in heart transplantation for cardiac sarcoidosis mimicking idiopathic giant cell myocarditis: long-term follow-up

: Heart transplantation is a life-saving therapy for some patients admitted for acute myocarditis. However, controversial exists about the major risk of rejection following heart transplantation in specific types of myocarditis. Because of relatively few data on the post heart transplant outcomes, we report the long-term follow-up of a 39-year-old patient with a previous history of ulcerative colitis, which rapidly worsened heart failure until an emergency heart transplant in 2004.The clinical course was complicated by many episodes of rejection; lastly, after the development of severe cardiac allograft vasculopathy, re-heart transplantation was needed. The main findings of this case are: 1) inflammatory aetiology should always be suspected in patients with concomitant autoimmune disease that developing rapidly progressing heart failure; 2) patients with inflammatory myocardial disease undergoing heart transplantation should also undergo strict immunological surveillance; 3) the option of performing the re-heart transplant in a patient with a so complex management in the first one could be uncertain, but in this case the young age and lack of noncardiac comorbidities were effective to favour the survivor after two immunologically so challenging heart transplantation.

Successful cardiac transplantation outcomes in patients with adult congenital heart disease

ObjectivesThe purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to...

The role of 3D printing in preoperative planning for heart transplantation in complex congenital heart disease.

The presence of a structural cardiac defect in the setting of dextrocardia is extremely rare. Graspable models allow enhanced appreciation of aberrant structures and vascular relations, particularly in rare and complex cases. This is the first case report of the use of a replica of a patients' anatomy to plan the surgical strategy in the setting of dextrocardia. We intend to demonstrate the benefit of three-dimensional printing to enhance preoperative planning in complex congenital heart disease undergoing heart transplantation. The anomalous structures encountered include situs inversus dextrocardia, transposition of the great vessels, a single atrium and a dilated double-outlet single right ventricle. Computed Tomography acquisition was performed with the use of ECG multiphase gating technology and contrast enhancement. The structures of interest were segmented and the generated 3D mesh was exported as a stereolithographic (STL) file. The model was printed on a Z-Corp 250 binder jetting printer. Post processing techniques were used to enhance model strength. Pre-operative 3D visualisation of the patients' anatomy allowed for a more comprehensive surgical strategy to be planned, thus reducing the intra-operative duration and cross-clamp time which are recognised to correlate with reduced patient morbidity. The ongoing advances in medical image procurement and 3D processing software and printing technology will continue to enhance preoperative planning and thereby improve patient care. We demonstrate the pivotal role played by such technologies in advancing spatial comprehension of complex aberrant anatomy.

Temporal trends and changing profile of adults with congenital heart disease undergoing heart transplantation.

To investigate the temporal trends in profile and outcomes of adults with congenital heart disease (ACHD) undergoing heart transplantation (HT). Out of a multi-institutional series of 2257 HT from 1988 to 2012, 100 HT were performed in 97 ACHD. We evaluated clinical characteristics, underlying defect, surgical history, perioperative issues, and outcomes. We compared two eras: era 1 (1988-2005, n = 48) and era 2 (2006-2012, n = 49). Mean age at HT was 30.3 ± 10.5 years. Twenty-five patients (25.8%) had biventricular physiology with a systemic right ventricle and 43 patients (44%) had univentricular physiology. Adults with congenital heart disease severity were classified as great complexity (74.2%), moderate (21.7%), and simple (4.1%). During a median follow-up of 28.7 months [0-282], 44 patients died. Early mortality was high (34%; 95% CI 0.2536-0.4390). Survival was 63.9% at 1 year. The proportion of univentricular patients did not change. Biventricular patients with systemic right ventricle significantly increased in era 2 (16.7 vs. 34.7%, P = 0.04) due to increasing number of transposition of the great arteries with atrial switch. Although the proportion of great complexity ACHD did not change significantly in era 2 (81.6% vs. and 66.7% in era 1, P = 0.09), ACHD recipients have more advanced disease, being more likely hospitalized (P = 0.03), receiving intravenous inotropes (P = 0.01), under assist devices (P = 0.04), or UNOS status 1 (P = 0.02) at the time of HT. Survival rates were comparable. Despite a worse risk profile, mortality after HT in ACHD did not increase. Improving survival of complex CHD will probably amplify the proportion of complex ACHD recipients with more advanced disease.

Outcomes and risk factors for heart transplantation in children with congenital heart disease

ObjectiveHeart transplantation in children with underlying congenital heart disease has traditionally been associated with inferior outcomes. We report our single institution experience. MethodsBetween 1988 and 2013, 124 children with congenital heart disease underwent heart transplantation. Competing risks analysis modeled events after heart transplantation (retransplantation, death without retransplantation). Multivariable regression analysis examined risk factors affecting outcomes. ResultsThere were 76 male patients (61%); median age was 3.8 years (interquartile range, 0.6-11.5). Sixteen patients (13%) underwent primary heart transplantation, and 108 patients (87%) underwent prior surgical repair/palliation. Thirty-eight patients (31%) had 2 ventricles, and 86 patients (69%) had a single ventricle, including 33 (27%) who underwent a prior Fontan procedure. Competing risks analysis showed that at 10 years after heart transplantation, 13% of patients had undergone retransplantation, 43% of patients had died without retransplantation, and 44% of patients were alive without retransplantation. After retransplantation, 9 of 17 patients were alive and 3 of 17 patients had undergone a second retransplantation. Overall 15-year survival after initial heart transplantation was 41%. On multivariable analysis, risk factors for early-phase mortality were age less than 1 year (hazard ratio [HR], 7.2; 95% confidence interval [CI], 2.4-22.0; P < .001) and prolonged cardiopulmonary bypass (HR, 5.0; 95% CI, 2.1-11.8; P < .001). Risk factors for late-phase mortality were age more than 1 year (HR, 3.0; 95% CI, 1.1-7.7; P = .025) and donor–recipient race mismatch (HR, 2.2; 95% CI, 1.2-4.1; P = .016). Survival was not affected by era, underlying anomaly, prior Fontan procedure, sensitization, or pulmonary artery augmentation. ConclusionsThe outcomes of heart transplantation in children with congenital heart disease have not improved in the current era. Survival was not affected by the underlying anomaly, prior Fontan procedure, or sensitization. Strategies to improve the outcomes in patients with congenital heart disease may need to address selection criteria, transplantation timing, and pretransplant and post-transplant care. The effect of donor–recipient race mismatch warrants further investigation and might affect organ allocation algorithms or immunosuppression management.

0135: Increasing severity and complexity in adults with congenital heart disease undergoing heart transplantation (ACHD): temporal trends – a collaborative study on 97 patients

Residual abnormalities in cardiac structure and function predispose ACHD to late-onset heart failure and its complications. Therefore, heart transplantation (HT) in ACHD is increasingly used. Out of a multi-institutional (3 centers) series of 2257 HT from 1988 to 2012, 100 (4.4%) were performed in 97 ACHD (65 men). They represented 45% of ACHD recipients in France at that time. We investigated the role of temporal trends on profile and outcomes of ACHD recipients. Trends were compared between 2 eras: era 1 (1988-2005, n=48) and era 2 (2006-2012, n=49). Mean age at the time of HT was 29.8 years. Forty-three patients (44%) had univentricular physiology (1V). Severity of disease was categorized in terms of initial diagnosis (according to classification of 32th ACC Bethesda Conference): 74.2% had a great complexity cardiopathy while 21.7% had a moderate severity disease and 4.1% a simple CHD. In-hospital mortality was high (34%). Baseline characteristics did not differ significantly between the 2 eras. Era 2 recipients had less often right heart failure signs before HT. Their donors were older. They were more likely to be hospitalized, supported by inotropes and assist devices at the time of HT. The rate of 1V patients did not change over time: 50% in era 1 vs 39% in era 2 (p=0.27). The distribution of severity of disease changed significantly over time (p=0.048). The proportion of adult recipients with CHD of great complexity was higher in era 2 than era 1 (respectively 81.6% and 66.7%). In fact, transposition of the great arteries became the major provider of HT in adult in the recent era (30.6% in era 2 vs 8.3%, p=0.006), representing the only primary diagnosis whose proportion increased significantly. Multivariable factors associated with increased in-hospital mortality did not include transplant era. Despite a worse baseline risk profile, and increasing complexity of ACHD recipients in recent years, mortality after HT has not increased.

Outcomes in Patients With Symptomatic Cerebrovascular Disease Undergoing Heart Transplantation

We sought to determine outcomes in patients with and without symptomatic cerebrovascular disease (sCVD) undergoing heart transplantation. Second, we sought to determine factors associated with stroke in the perioperative period after heart transplantation. sCVD is considered a relative contraindication to heart transplantation. Despite this concern, outcomes in patients with sCVD undergoing heart transplantation have not been well defined. Data on all single-organ heart transplants performed in the United States between April 1994 and December 2006 in patients age 40 years or older were analyzed. Survival analysis was performed to examine the effect of sCVD on the combined outcome of stroke or death, stroke, death, and functional decline, adjusting for potential confounding variables over long-term follow-up. In a separate analysis, predictors of perioperative stroke during the transplant-related hospitalization were examined using multiple logistic regression. There were 1,078 patients with and 16,765 patients without sCVD. The annualized rates of stroke or death (11.5% vs. 7.8%; p < 0.001), stroke (4% vs. 1.4%; p < 0.001), death (8.9% vs. 7.4%; p < 0.001), and functional decline (3.7% vs. 3.0%; p = 0.002) were higher in patients with sCVD than in patients without sCVD. In multivariable analysis, patients with sCVD were at increased risk of stroke or death (hazard ratio [HR]: 1.29; 95% confidence interval [CI]: 1.17 to 1.42), stroke (HR: 2.24; 95% CI: 2.02 to 2.87), and functional decline (HR: 1.21; 95% CI: 1.03 to 1.42) compared with those without sCVD. We did not identify a higher risk of death in patients with sCVD (HR: 1.08; 95% CI: 0.98 to 1.20), compared with those without sCVD. sCVD, ventilator use, and ventricular assist device use were the most important predictors of perioperative stroke. Patients with sCVD are at an increased risk of stroke and functional decline after transplantation independent of other variables, but not death, during long-term follow-up. These results should assist programs in making informed decisions in patients with sCVD who are undergoing evaluation for heart transplantation.

Heart Transplantation in 107 Cases of Chagas' Disease

Introduction Chagas' disease is endemic in South America. Objective This research reviewed the experience with cardiac transplantation in Chagas' disease, emphasizing reactivation, immunosuppression, and mortality. Methods Over 25 years from March 1985 to March 2010, 107/409 (26.2%) patients with Chagas' disease underwent heart transplantation, patients including 74 (71.1%) men and 72 (67.2%), in functional class IV with 33 (30.8%) on vasopressors and 17 (10.7%) on mechanical circulatory support. Results The diagnosis of disease reactivation was performed by identifying the parasite in the myocardium ( n = 23; 71.8%) in the subcutaneous tissue ( n = 8; 25.0%), in blood ( n = 11; 34.3%), or in central nervous tissue ( n = 1; 3.1%). Hospital mortality was 17.7% ( n = 19) due to infection ( n = 6; 31.5%), graft dysfunction ( n = 6; 31.5%), rejection ( n = 4; 21.1%), or sudden death ( n = 2; 10.5%). Late mortality was 27 (25.2%) cases, which were distributed as: rejection ( n = 6; 22.2%), infection ( n = 6; 22.2%), ( n = lymphoma 4; 14.8%), sarcoma ( n = 2; 7.4%), for constrictive pericarditis ( n = 2; 7.4%) reactivation of Chagas' disease in the central nervous system ( n = 1; 7.1%). Conclusions Transplantation in Chagas' disease has peculiar problems that differ from other etiologies due to the possibility of disease reactivation and the increased possibility of emergence of cancers. However, transplantation is the only treatment able to modify the natural progression of the disease in its terminal phase. Early diagnosis and rapid introduction of benzonidazole reverses the histological patterns. Immunosuppression, especially steroids, predisposes to the development of cancer and disease reactivation.

Heart Transplantation in Adolescent and Adult Patients With Congenital Heart Disease: A Case-Control Study

Background The number of adolescent and adult patients with congenital heart disease undergoing heart transplantation is increasing. We aimed to better define the characteristics of these patients and their survival after transplantation. Methods We describe a group of patients with end-stage heart failure owing to congenital heart disease undergoing heart transplantation at a single tertiary center and compare their short- and long-term survival with a group of matched controls with dilated cardiomyopathy and the entire cohort of transplanted patients at our center. Results Between 1985 and 2006, a total of 322 orthotopic heart transplantations were performed at our center. Thirteen patients (mean age, 27.5 years) had a diagnosis of congenital heart disease with a wide spectrum of lesions. The survival of these 13 patients was 85% at 30 days, 1, 5, and 10 years and 77% at 20 years, which did not differ significantly to the short- and long-term survival of the entire cohort of patients with heart transplantation and to the survival of age-matched controls with dilated cardiomyopathy. Conclusion In our single-center experience, short- and long-term survival after heart transplantation in a selected, small group of patients with end-stage heart failure owing to congenital heart disease did not differ significantly compared with a group of age-matched controls and the entire cohort.

Shunt or Snare: Coronary Endothelial Damage due to Hemostatic Devices for Beating Heart Coronary Surgery

Occlusion of coronary arteries during off-pump coronary bypass operations bears the potential for endothelial injury. The aim of this study was to elucidate the effects of hemostatic devices on the beating heart in human coronaries by means of scanning electron microscopy. The coronary arteries of 9 patients with dilated cardiomyopathy and 13 with ischemic heart disease undergoing heart transplantation were handled with intracoronary shunts as well as external snaring techniques on a beating heart, after cannulation but before starting cardiopulmonary bypass. Adjacent noninstrumented coronary artery segments served as controls. Integrity of endothelial lining was observed with scanning electron microscopy. Nearly all coronary artery segments manipulated with a shunt exhibited a severe injury with extensive endothelial denudation. Endothelial injury was significantly higher after manipulation with intracoronary shunts compared with external occlusion devices (p < 0.001) or control specimens (p < 0.001). Plaque rupture was apparent in 3 samples. Manipulation of human coronary arteries during off-pump operations leads to endothelial denudation and plaque rupture. From this investigation we conclude that insertion of intracoronary shunts during beating heart operations leads to severe endothelial denudation in human coronary arteries. We therefore recommend using shunts selectively in cases where critical ischemia or technical difficulties due to anatomic conditions are expected during anastomosis. The clinical significance of these structural damages has to be further investigated with clinical trials.

Risk Factors for Chagas' Disease Reactivation After Heart Transplantation

Chagas' disease is the illness caused by the protozoan Trypanosoma cruzi and it is still endemic in Latin America. Heart transplantation is a therapeutic option for patients with end-stage Chagas' cardiomyopathy. Nevertheless, reactivation may occur after transplantation, leading to higher morbidity and graft dysfunction. This study aimed to identify risk factors for Chagas' disease reactivation episodes. This investigation is a retrospective cohort study of all Chagas' disease heart transplant recipients from September 1985 through September 2004. Clinical, microbiologic and histopathologic data were reviewed. Statistical analysis was performed with SPSS (version 13) software. Sixty-four (21.9%) patients with chronic Chagas' disease underwent heart transplantation during the study period. Seventeen patients (26.5%) had at least one episode of Chagas' disease reactivation, and univariate analysis identified number of rejection episodes (p = 0.013) and development of neoplasms (p = 0.040) as factors associated with Chagas' disease reactivation episodes. Multivariate analysis showed that number of rejection episodes (hazard ratio = 1.31; 95% confidence interval [CI]: 1.06 to 1.62; p = 0.011), neoplasms (hazard ratio = 5.07; 95% CI: 1.49 to 17.20; p = 0.009) and use of mycophenolate mofetil (hazard ratio = 3.14; 95% CI: 1.00 to 9.84; p = 0.049) are independent determinants for reactivation after transplantation. Age (p = 0.88), male gender (p = 0.15), presence of rejection (p = 0.17), cytomegalovirus infection (p = 0.79) and mortality after hospital discharge (p = 0.15) showed no statistically significant difference. Our data suggest that events resulting in greater immunosuppression status contribute to Chagas' disease reactivation episodes after heart transplantation and should alert physicians to make an early diagnosis and perform pre-emptive therapy. Although reactivation led to a high rate of morbidity, a low mortality risk was observed.

Regression of left ventricular hypertrophy in a transplanted heart

A woman with advanced coronary artery disease underwent heart transplantation. The donor heart had left ventricular hypertrophy. The electrocardiographic and echocardiographic evidence, of left ventricular hypertrophy regressed during follow up; estimated left ventricular mass decreased from 393 g to 171 g. The adaptation of myocardial mass and performance after transplantation is not fully understood. This case illustrates the potential for regression of left ventricular hypertrophy in response to altered loading conditions.

Ultrastructural and histopathologic observations of a human cardiac transplant with features of rejection.

A 43-year-old man who had intractable heart failure due to coronary artery disease underwent heart transplantation. The postoperative period was complicated by gastrointestinal bleeding and pulmonary infection which led to renal shutdown on the eighth postoperative day and to death two weeks after surgery. The transplanted heart was examined by light and electron microscopy. Changes compatible with rejection, including mononuclear infiltration, vascular changes, and foci of myocytolysis and myocardial necrosis, were found. No such changes were seen in the upper parts of the recipient's atria or his aorta. At no time during the postoperative period were clinical or electrocardiographic evidences of rejection observed.