Non-Fontan Adult Congenital Heart Disease Transplantation Survival Is Equivalent to Acquired Heart Disease Transplantation Survival.

BACKGROUND: As a result of improved diagnostic methods, medical treatment, surgical correction, and palliation in childhood, there is a growing number of adult patients with congenital heart disease (CHD) who may experience heart failure and subsequently require heart transplantation (HT). Because of complex anatomy, previous operations, and frequently increased pulmonary vascular resistance (PVR), these patients represent a group with a higher risk of early mortality after transplantation. METHODS: From May 1999 to December 2014, our institution performed 25 HTs in adult patients with end-stage CHD. We present our data and outcomes of transplantation in this group. RESULTS: The median age at transplantation was 38 years (range, 18.4-53.7 years). Survival was 88% at 30 days, 88% at 1 year, and 77% at 5 years. We identified long donor heart ischemic time (>4 hours) as an important risk factor for early mortality. There was no significant difference in the survival of patients undergoing transplantation for CHD and patients undergoing transplantation for other diagnoses. CONCLUSIONS: With careful donor and recipient selection, adults with end-stage CHD undergoing HT can achieve excellent early and midterm survival, comparable to the survival of patients who undergo transplantation for other diagnoses.

The impact of angiotensin II type 1 receptor antibodies on post-heart transplantation outcome in Heart Mate II bridged recipients.

OBJECTIVES: Antibodies targeting angiotensin II type 1 receptor (AT1R) have been associated with malignant hypertension, autoimmune diseases and acute rejection and graft loss in solid organ transplantation. The aim of our study was to assess the impact of anti-AT1R antibodies on survival and incidence of acute cellular rejection (ACR) and pathology antibody-mediated rejection (pAMR) in a population of heart transplant recipients who were bridged to transplantation with a durable mechanical assist device Heart Mate II. METHODS: Sera of 69 consecutive heart transplant recipients transplanted between October 2008 and August 2014 were tested for the presence of angiotensin II type 1 receptor antibodies before Heart Mate II device implantation and at the time of transplantation. Overall survival and post-transplant rejection-free survival were compared between antibody-negative and antibody-positive recipients using Kaplan-Meier and log-rank tests. RESULTS: Anti-AT1R antibodies were present in 8 patients (11.6%) before Heart Mate II implantation. During the left ventricular assist device (LVAD) bridging, 44 patients (63.8%) who were initially anti-AT1R antibody-negative became positive, leaving 17 (24.6%) anti-AT1R antibody-negative patients at the time of transplantation for all comparisons. One- and 5-year survival was 88 ± 8 and 76 ± 10% for anti-AT1R antibody-negative and 87 ± 5 and 81 ± 7% for anti-AT1R antibody-positive patients, respectively (P = 0.582). Freedom from ACR at 1 year was 68 ± 12% for anti-AT1R-negative and 75 ± 6% for anti-AT1R-positive recipients (P = 0.218). None of the anti-AT1R-negative patients developed AMR 1 year post-transplantation, whereas freedom from pAMR in anti-AT1R-positive recipients was 98 ± 2% (P = 0.198). CONCLUSIONS: Our data showed no difference in the overall post-heart transplant survival and freedom from acute cellular and antibody-mediated rejection between anti-AT1R-negative and anti-AT1R-positive recipients. Further research is needed to assess the role of anti-AT1R antibodies in the risk stratification of LVAD-bridged recipients on the post-heart transplantation outcomes.

Aortic and Mitral Valve Replacement Due to Extensive Inflammatory Immunoglobulin G4-Related Pseudotumor.

A 64-year-old woman with extensive tumorous infiltration of the mitral and aortic valves underwent partial resection of a tumor of the left ventricular outflow tract and replacement of both affected valves. Histology revealed an inflammatory pseudotumor with a significant number of immunoglobulin-G4-positive plasma cells. The histologic and clinical findings suggested immunoglobulin-G4-related disease of the heart.

Bridge to transplantation with long-term mechanical assist device in adults after the Mustard procedure.

BACKGROUND: There is limited clinical experience with bridging to transplant with a left ventricular assist device (VAD) in patients with previously palliated transposition of great arteries. METHODS: Five adult patients presenting with systemic right ventricular failure 30 years after a Mustard operation were implanted with a HeartMate II VAD. The implant was completed using standard procedures with only minor modifications to accommodate right ventricular cannulation. RESULTS: All 5 patients were men, with a mean age of 31.5 ± 1.8 years and a median time since Mustard operation of 30 (range 28 to 32) years. All patients had sternal closure on Post-operative Day (POD) 1, and 2 patients required additional re-operation for bleeding. One patient required temporary support of the non-systemic ventricle. The mean duration of VAD support was 284 ± 177 days; 3 patients underwent heart transplant and 2 died on PODs 502 and 34, respectively. Both deaths were due to progressive heart failure and pump thrombosis. Comorbidities, anatomy and mediastinal scarring did not preclude implantation and heart failure symptoms improved in all patients. CONCLUSIONS: With the increased prevalence of late post-Mustard heart failure, bridge to transplant with a VAD may be a suitable treatment option for patients who are severely ill.

Tricuspid valve surgery in patients with idiopathic hypereosinophilic syndrome.

A 53-year-old female with idiopathic hypereosinophilic syndrome underwent recurrent tricuspid valve replacement for Löffler's endocarditis. We review the literature on tricuspid valve surgery in patients with this uncommon disorder.

Minimally invasive removal of a temporary RVAD.

We describe a minimally invasive technique for the removal of a temporary right ventricular assist device (RVAD) that provided support concomitant with durable left ventricular assist device support. The RVAD cannulas are mobilized through a small subxiphoid incision at the cannula exit site. Both cannulas are transected subcutaneously, then occluded with plugs made of rolled bovine pericardium, and the skin is closed. The cannula remnants are left in place until heart transplantation is accomplished. To minimize risk of thrombus formation at the cannula tips and subsequent embolization into the right atrium or pulmonary artery, anticoagulation is increased to achieve an international normalized ratio (INR) in the range of 2.5-3.0.

Post-heart transplantation outcome of HeartMate II-bridged recipients requiring unplanned concomitant temporary right ventricular mechanical support.

OBJECTIVES: Second-generation axial-flow left ventricular assist devices (LVADs) have become an established therapy in bridging end-stage heart failure patients to cardiac transplantation. Despite the proven clinical success of these devices, some patients develop right ventricular (RV) failure after LVAD implantation. We sought to determine post-heart transplantation outcomes of HeartMate II (HMII)-bridged patients who developed postimplantation right ventricular failure and received Levitronix CentriMag for RV support in addition to LVAD. METHODS: This was a single-centre institutional report of 64 patients transplanted during 2007-2013 from a HeartMate II device. Patients were divided into two groups according to whether they received an isolated LVAD (n = 56) or required additional RV mechanical support (n = 8). These two groups were compared for early graft loss (death before discharge or retransplantation), major early post-transplant complications and 3-year graft survival. RESULTS: Early graft loss was 10.7% in isolated HMII and 25% in HMII + RVAD patients (P = 0.26). There were no observed differences in the rates of primary graft dysfunction (7.3 vs 0%, P = NS), renal failure (16.7 vs 12.5%, P = NS) and stroke (11.1 vs 25%, P = 0.273) between the two groups. Pulmonary artery resistance (odds ratio: 3.286, 95% confidence interval: 1.063-10.157, P = 0.039) was identified as a significant predictor for adverse outcome of mechanically-bridged heart transplant recipients. The 3-year graft survival rate was 86 ± 5% in isolated HMII and 75 ± 15% in HMII + RVAD patients, P = 0.326. CONCLUSIONS: Our data demonstrate that heart transplant recipients who required unplanned RV mechanical support after LVAD implantation achieved comparable rates of early graft loss, post-transplant renal failure and stroke rate in comparison with patients bridged with an isolated HeartMate II assist device. Three-year graft survival was equivalent between those two groups. Given the small sample size, further studies involving more patients are needed to support or challenge our conclusions.

Case report: atypical fungal obstruction of the left ventricular assist device outflow cannula.

We describe a very rare case of outflow cannula obstruction with fungal infectious thrombus formation. Discussion includes the etiology, diagnosis, and management of fungal infection complications related with long-term mechanical circulatory support. Left ventricular assist devices (LVADs) are increasingly used as bridge to transplant and permanent long-term therapy in the population with end-stage heart failure. Even though better clinical outcomes have been achieved with the newer-generation continuous-flow devices, infection complications are still a major risk for patients with continuous-flow LVAD implantation in long-term follow-up [Ann Thorac Surg 90:1270-1277, 2010]. Device-related infections can be categorized as driveline infections, pump-pocket infections, and LVAD-associated endocarditis [Expert Rev Med Devices 8: 627-634, 2011]. The microbiological profile is very heterogeneous; the most common pathogens are Staphylococcus, Pseudomonas, Streptococcus species, and Candida. Severe fungal infection may lead to dysfunction of the LVAD due to obstructive mass formation within the device. Due to the only anecdotal reports in the current literature, we present a very rare case of outflow fungal infectious thrombus formation leading to outflow cannula obstruction in patient with LVAD.

Intracardiac ectopic thyroid (struma cordis).

A 67-year-old male with a history of gastrointestinal malignancy was found to have a tumor in the right ventricular outflow tract. The tumor was surgically removed, and the histological diagnosis was thyroid struma. We review the literature on this rare cardiac tumor.

Comprehensive management of severe intestinal bleeding in a patient supported for 94 days by the biventricular Levitronix CentriMag system.

The use of short-term mechanical circulatory support during postcardiotomy acute heart failure provides an opportunity to stabilize the patient's hemodynamic state while determining the best long-term strategy. Because all of these devices require anticoagulation treatment of various intensities, management of major bleeding can be critical for the overall outcome of the therapy. In this regard, the newest generation of magnetically levitated centrifugal-flow pumps affords several potential advantages in terms of thrombogenicity and eventual discontinuation of anticoagulation treatment. We report the use of a short-term biventricular assist device (Levitronix CentriMag) for 94 days in a 55-year-old man with refractory ventricular arrhythmias after combined heart surgery. Despite serious complications while the patient was on the assist device, including severe intestinal bleeding with the necessity of discontinuing anticoagulation for 10 days and, ultimately, hemicolectomy, the circulatory support was completed with successful heart transplantation.

Gender differences in cardiac ischemic injury and protection--experimental aspects.

This review summarizes some available information on gender differences of myocardial injury with particular attention to experimental approach. It has been observed that significant gender differences exist already in normal heart. They involve among others cardiac growth, contractile function, calcium metabolism and function of mitochondria. Differences, characteristic of the normal myocardium, generate the logical presumption of the different reaction of the male and female heart to various pathogenic factors. Most of the experimental studies confirm the clinical observations: increased resistance of the female heart to ischemia/reperfusion injury was shown in dogs, rats, mice and rabbits. Furthermore, gender differences in the ischemic tolerance of the adult myocardium can be influenced by interventions (e.g. hypoxia) imposed during the early phases of ontogenetic development. The already high tolerance of the adult female heart can be increased by adaptation to chronic hypoxia and ischemic preconditioning. It seems that the protective effect depends on age: it was absent in young, highly tolerant heart but it appeared with the decrease of natural resistance during aging. Both experimental and clinical studies have indicated that female gender influences favorably also the remodeling and the adaptive response to myocardial infarction. It follows from the data available that male and female heart differs significantly in many parameters under both physiological and pathological conditions. Detailed molecular and cellular mechanisms of these differences are still unknown; they involve genomic and non-genomic effects of sex steroid hormones, particularly the most frequently studied estrogens. The cardiovascular system is, however, influenced not only by estrogens but also by other sex hormones, e.g. androgens. Moreover, steroid hormone receptors do not act alone but interact with a broad array of co-regulatory proteins to alter transcription. The differences are so important that they deserve serious consideration in clinical practice in search for proper diagnostic and therapeutic procedures.

Effect of perinatal hypoxia on cardiac tolerance to acute ischaemia in adult male and female rats.

1. The number of adult patients undergoing surgery for congenital cyanotic defects in childhood has increased significantly. Therefore, the aim of the present study was to examine the effect of perinatal hypoxia on the tolerance of the adult myocardium to acute ischaemia-reperfusion injury. 2. Pregnant Wistar rats were exposed to intermittent hypobaric hypoxia 7 days before delivery; pups were born under normoxic conditions and exposed to hypoxia again for 10 postnatal days. After the last hypoxic exposure, all animals were kept for an additional 3 months under normoxic conditions. All experiments were performed on 90-day-old rats. 3. Ventricular arrhythmias were assessed on isolated perfused hearts during 30 min occlusion of the left anterior descending coronary artery. Infarct size was measured on isolated hearts (40 min regional ischaemia and 120 min reperfusion) and on open-chest animals (20 min regional ischaemia and 3 h reperfusion). 4. Perinatal exposure to hypoxia significantly increased cardiac tolerance to ischaemic injury in adult females, as evidenced by the lower incidence and severity of ischaemic ventricular arrhythmias, compared with the normoxic group. The effect of perinatal hypoxia on ischaemic arrhythmias in males was quite the opposite. Myocardial infarct size measured in open-chest animals only was significantly smaller in normoxic females compared with normoxic males. Perinatal exposure to hypoxia had no effect on infarct size in either setting or sex. 5. The results of the present study support the hypothesis that perinatal hypoxia is a primary programming stimulus in the heart that may lead to sex-dependent changes in cardiac tolerance to acute ischaemia in later adult life. This would have important implications for patients who have experienced prolonged hypoxaemia in early life.