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Primary cardiac sarcomas are very aggressive, being a challenge to cardio-oncology specialty. Surgical planning and innovative techniques have enhanced the possibility of resection. We have described a case of recurrent primary left atrium angiosarcoma, successfully resected with a modified partial autotransplantation technique, planned using a 3-dimensional model created from computed tomography. (Level of Difficulty: Intermediate.), Central Illustration

We present 3 cases of superior vena cava (SVC) syndrome following percutaneous right ventricular assist device (RVAD) placement. Each case underscores the importance of early recognition of SVC syndrome in patients with percutaneous RVAD insertion via the internal jugular vein and calls for heightened awareness of device-associated complications. (Level of Difficulty: Advanced.), Central Illustration

We present a novel multidisciplinary approach for the treatment of electrical storm combining bilateral cardiac sympathectomy, extrapericardial coil insertion, and implantable cardioverter defibrillator upgrade in a patient with nonischemic cardiomyopathy and ventricular arrhythmias refractory to conventional therapies. (Level of Difficulty: Advanced.), Graphical abstract

We present a case of recurrent isolated cardiac sarcoidosis, 3 years post-heart transplantation. The case highlights the scarcity of data on the utility of immunosuppression in cardiac sarcoidosis and, in particular, raises questions about the optimal immunosuppression regimen in transplant recipients. (Level of Difficulty: Advanced.), Graphical abstract

Antibody-mediated rejection is a major cause of graft failure, mortality, and morbidity among cardiac transplant recipients. We present the first reported case of TandemHeart (LivaNova, Pittsburgh, Pennsylvania) used in the management of antibody-mediated rejection associated with cardiogenic shock. (Level of Difficulty: Advanced.), Graphical abstract

Many patients die while waiting for a heart transplant. Therefore, it is vital that all suitable organs are used for transplantation. We present a case of an allograft that was transplanted twice and outline considerations regarding tissue typing, the impact of repeated ischemic time, and ethical considerations with allograft retransplantation. (Level of Difficulty: Intermediate.), Central Illustration

Metastatic pulmonary calcification is a metabolic lung disease that occurs due to a prolonged hypercalcemic state where calcium salts precipitate into numerous foci or nodules of fluffy calcifications in the lung apices, usually bilaterally. Calcifications can also occur in other organs such as in the stomach and the kidneys. It is often underdiagnosed and is usually associated with end stage renal failure and resultant secondary hyperparathyroidism. Nevertheless, it is rarely reported in the postcardiac surgery status and cardiac transplant patients. We present a case of a 15-year-old male patient with a recent history of cardiac transplant due to a complex congenital heart disease where findings of extensive metastatic pulmonary calcifications were seen a routine follow-up chest radiograph. Clinical manifestations of metastatic pulmonary calcifications can range from having no symptoms or mild dyspnea on exertion to fulminant respiratory failure. Therefore, early recognition of imaging features and initiation of proper management is crucial to the patient's outcome.

Mycophenolate Mofetil (MMF) is routinely used immunosuppressant in solid organ transplantation is commonly associated with several gastrointestinal (GI) side effects. Here we present a case of giant gastric ulcer of 5 cm from MMF use post cardiac transplant. Case Description A 56-year-old male with history of severe ischemic cardiomyopathy post heart transplant was on immunosuppression with MMF, tacrolimus and prednisone for 5 months. He presented with severe epigastric pain and intermittent episodes of melena for 1 month. His pain radiated to back that is worsened with eating. Associated with loss of appetite, vomiting and 16-pound weight loss in 3 months. He never smoked, drank alcohol or used over the counter pain medications. He was profoundly anemic requiring blood transfusions. EGD performed demonstrated very large clean-based ulcer of 5 cm diameter in the body, smaller ulcer of 8 mm diameter in pre-pyloric region and 5–10 small aphthous ulcers in the gastric body and fundus. Gastric biopsies taken from the ulcer were negative for Helicobacter pylori, cytomegalovirus and malignancy. Flexible sigmoidoscopy revealed non-bleeding inflamed internal hemorrhoids. Consequently, MMF was discontinued and switched to azathioprine. He was treated with twice daily proton pump inhibitor therapy with resolution of abdominal pain, improved appetite and weight gain. Discussion MMF is well known for common GI side-effects such as nausea, diarrhea, vomiting, ulcers, abdominal pain and rarely gastrointestinal bleeding. Few studies reported 3 to 8% incidence of ulcer perforation and GI bleeding within 6 months. Risk of gastroduodenal erosions is nearly 1.83 times for MMF, with the highest lesions associated with MMF-tacrolimus-corticosteroid combination treatment as seen in our patient. Hypothesis is that GI tract is vulnerable because of dependence of enterocytes on de novo synthesis of purines, which is disrupted by MMF. Typically, upper GI mucosal injuries of mucosal irritation leading to esophagitis, gastritis and/or ulcers are seen. Endoscopy is both diagnostic and therapeutic if bleeding gastric ulcers are noted. Minor complications improve with reduction of drug dose or use of enteric coated preparation if feasible. Discontinuation of the drug is main stay in the management of MMF related ulcer disease. Simple medical treatment with either H2-receptor antagonists, proton-pump inhibitors, coating agents, prostaglandins or combination has proven effective in most cases. Considering excellent results with medical management of ulcer, role of surgery is limited.

Background: Heart transplantation is still the treatment of choice in patients with end-stage heart failure, refractory to medical treatment, in NYHA class III and IV, with dilated cardiomyopathy of various etiologies, including post-myocardial infarction. Objective: The aim of the study was to provide a descriptive analysis of the clinical, laboratory, and imaging parameters of patients undergoing heart transplantation during the pre- and postoperative period, as well as postoperative complications (including infections) and death rates. Material and methods: The variables collected from 53 patients who underwent a heart transplant were: age at heart transplant, gender, diagnosis at hospitalization, comorbidities, pre- and postoperative virology, pre- and postoperative laboratory analyses, pre- and postoperative echocardiography, post-transplant infections, complications, and treatment before and after the surgery. Results: Mean age at the time of transplant was 40.72 ± 14.07 years, and the majority (84.91%) of patients were males. The mean age of the donors was 31.92 ± 10.59 years. A proportion of 60.40% of patients were included in functional class NYHA IV, and 98.1% presented dilative cardiomyopathy of which 49.06% was due to previous myocardial infarction. No significant differences were observed between preand postoperative viral and bacterial serology. Left ventricular ejection fraction was significantly higher in the transplanted heart, and cardiac chamber diameters were significantly smaller after the transplant. Postoperative complications included 7 confirmed infections and 16 deaths, of which 5 had occurred during the surgical procedure. Conclusions: The present study brings important information in regard to the pre- and postoperative evaluation of patients with end-stage heart failure undergoing cardiac transplantation, from a clinical, laboratory, and imaging point of view, as well as in regard to postoperative complications and death.

A young man presented with syncope. He was diagnosed with triglyceride deposit cardiomyovasculopathy and skeletal myopathy secondary to adipose triglyceride lipase (ATGL) deficiency. Despite optimal medical therapy, he required heart transplantation to treat his heart failure. Five years post-transplant, the graft function was normal with no evidence of triglyceride deposits. (Level of Difficulty: Intermediate.), Graphical abstract, A young man presented with syncope. He was diagnosed with triglyceride deposit cardiomyovasculopathy and skeletal myopathy secondary to adipose…

Post-explant (ex vivo) evaluation of medical devices is an essential part of quality assurance, quality improvement, and further device development. Central to this is detailed pathological analysis. Here, we provide the first such evaluation of an explanted Tiara transcatheter mitral valve prosthesis. (Level of Difficulty: Advanced.), Graphical abstract, Post-explant (ex vivo) evaluation of medical devices is an essential part of quality assurance, quality improvement, and further device development…

Background: Advanced heart failure has an extremely high mortality withoAdvanced heart failure has extremely high mortality without advanced therapies (left ventricular assist device (LVAD) implantation or cardiac transplant). LVAD patients with bioprosthetic aortic valve are more prone to leaflet fusion resulting in valvular stenosis and regurgitation.ut advanced therapies like Left ventricular assist devices or cardiac transplant. LVAD patients with bioprosthetic aortic valve are more prone to leaflet fusion resulting in valvular stenosis and regurgitation. We present a 46 year old patient who had LV systolic function recovery while on LVAD. However, he had a severely stenotic aortic valve bioprosthesis with leaflet fusion that had to be corrected before deactivating his LVAD. Due to high surgical risk, we performed valve-in-valve TAVR with an Evolut CoreValve , however, the patient had significant aortic regurgitation secondary to deployment above the bioprosthetic valve ring. We successfully deployed a second Evolut CoreValve inside the ring with excellent results. This was followed by a successful LVAD deactivation next day. His LV systolic function continued to recover and he had no heart failure symptoms at 3 month follow up. In the right settings, TAVR in recovered LVAD patients with aortic stenosis as bridge to LVAD deactivation is viable option, especially for patients who fall in the high risk group. To the best of our knowledge, this is the first reported case of a valve-in-valve TAVR followed by successful LVAD deactivation in the setting of recovered LV systolic function. Case Presentation: We present a 46-year-old patient who had LV systolic function recovery while on LVAD. However, he had a severely stenotic aortic valve bioprosthesis with leaflet fusion that had to be replaced before deactivating his LVAD. Due to high surgical risk, we performed valve-invalve Trans-Catheter Aortic Valve Replacement (TAVR) with an Evolut self-expanding valve, however, the patient had significant aortic regurgitation secondary to deployment above the bioprosthetic valve ring. We successfully deployed a second Evolut Self-expanding valve inside the ring with excellent results. This was followed by a successful LVAD deactivation next day. His LV systolic function continued to recover and he had no heart failure symptoms at 3 month follow up. In the right settings, TAVR in recovered LVAD patients with aortic stenosis as a bridge to LVAD deactivation is a viable option, especially for patients who fall in the high-risk group. Conclusion: To the best of our knowledge, this is the first reported case of a valve-in-valve TAVR followed by successful LVAD deactivation in the setting of recovered LV systolic function.

We report a case of a woman with arrhythmogenic right ventricular cardiomyopathy (ARVC) who presented with severe right-sided heart failure and cardiac cirrhosis that mandated heart-liver transplantation. This case highlights an emerging sex-based difference in ARVC where female sex is associated with a higher risk of heart failure than in male patients with ARVC. (Level of Difficulty: Advanced.), Graphical abstract, We report a case of a woman with arrhythmogenic right ventricular cardiomyopathy (ARVC) who presented with severe right-sided heart failure…

HeartMate III is an emerging, small-sized centrifugal ventricular assist device. Its lower pump thrombosis and stroke rates make it favorable for use in pediatrics. We report the use of HeartMate III as a bridge to transplantation in an adolescent with failed Fontan circulation. (Level of Difficulty: Advanced.), Graphical abstract, HeartMate III is an emerging, small-sized centrifugal ventricular assist device. Its lower pump thrombosis and stroke rates make it favorable for use…

We describe a case of inherited restrictive cardiomyopathy in a patient presenting with severe biatrial enlargement. We review the evaluation and management of restrictive cardiomyopathy with a focus on genetic etiologies. (Level of Difficulty: Intermediate.), Graphical abstract, We describe a case of inherited restrictive cardiomyopathy in a patient presenting with severe biatrial enlargement. We review the evaluation and…

Shared decision making with patients in advanced heart failure is critical when making decisions on therapies such as left ventricular assist device implantation and cardiac transplantation. We describe a case wherein the risks of coronavirus disease-2019 affected the decision of a patient regarding proceeding with cardiac transplantation. (Level of Difficulty: Beginner.).

A heart transplant recipient presented with right heart failure. He had a history of implantable cardioverter-defibrillator implantation and extraction before undergoing heart transplantation 4 years earlier. A large fistula was found between the left subclavian artery and vein. Following successful percutaneous fistula closure, symptoms and right ventricular function improved. (Level of Difficulty: Advanced.), Graphical abstract, A heart transplant recipient presented with right heart failure, He had a history of implantable cardioverter-defibrillator implantation and…

Background Myocardial bridging (MB), though typically a benign finding, may occasionally lead to syncope, myocardial infarction, arrhythmia, or sudden death. Surgical denervation of transplanted hearts complicates the management of such incidentally detected post-transplant coronary anomalies due to the lack of classic ischaemic symptoms. Case summary A middle-aged female underwent an uncomplicated cardiac transplantation from a healthy male donor in his early 20s who had suffered a cardiac arrest while using cocaine. Given the young donor age, a pre-transplant coronary angiogram (CAG) was deferred. However, 6-week post-transplant, routine CAG, and intravascular ultrasound revealed an extensive MB spanning a significant portion of the left anterior descending coronary artery with substantial myocardium at risk. A stress test with myocardial perfusion imaging performed to evaluate the functional significance of the bridge did not reveal any perfusion abnormalities in the myocardium at risk. Discussion In current practice, younger donors often do not undergo pre-transplantation CAG routinely performed in older donors given the lower prevalence of significant coronary disease. However, post-operatively this young donor was found to have passed on a potentially life-threatening MB to a denervated recipient, who cannot manifest typical anginal symptoms during ischaemia, thereby challenging providers to choose among strategies of watchful waiting, risk stratification, or pre-emptive intervention. In retrospect, the donor’s mode of death may have signalled an underlying structural abnormality that warranted further pre-transplant characterization. In order to ensure optimal quality of transplanted hearts, young donors may warrant pre-transplant CAG despite their age, particularly those with a history of drug use or suspicious mode of death.

Mucormycosis is an invasive fungal infection (IFI) due to several species of saprophytic fungi, occurring in patients with underlying co-morbidities (including organ transplantation). During the ongoing Coronavirus disease 2019 (COVID-19) pandemic, there have been increasing reports of bacterial and fungal co-infections occurring in COVID-19 patients, including COVID-19 associated pulmonary aspergillosis (CAPA). We describe a case of mucormycosis occurring after COVID-19, in an individual who received a recent heart transplant for severe heart failure. Two months after heart transplant, our patient developed upper respiratory and systemic symptoms and was diagnosed with COVID-19. He was managed with convalescent plasma therapy and supportive care. Approximately three months after COVID-19 diagnosis, he developed cutaneous mucormycosis at an old intravascular device site. He underwent extensive surgical interventions, combined with broad-spectrum antifungal therapy. Despite the aggressive therapeutic measures, he died after a prolonged hospital stay. In this case report, we also review the prior well-reported cases of mucormycosis occurring in COVID-19 patients and discuss potential mechanisms by which COVID-19 may predispose to IFIs. Similar to CAPA, mucormycosis with COVID-19 may need to be evaluated as an emerging disease association. Clinicians should be vigilant to evaluate for invasive fungal infections such as mucormycosis in patients with COVID-19 infection.

Background:BRCC3/MTCP1 deletions are associated with a rare familial moyamoya angiopathy with extracranial manifestations.Case: We report the case of an adolescent male presenting with progressive and symptomatic moyamoya angiopathy and severe dilated cardiomyopathy caused by a hemizygous deletion of BRCC3/MTCP1. He was treated for renovascular hypertension by left kidney homograft and right nephrectomy in infancy and had other syndromic features, including cryptorchidism, growth hormone deficiency, and facial dysmorphism. Due to worsening of the neurological and cardiac condition, he was treated by a direct superficial temporal artery to middle cerebral artery bypass to enable successful cardiac transplant without cerebral damage.Conclusions:BRCC3-related moyamoya is a devastating disease with severe heart and brain complications. This case shows that aggressive management with cerebral revascularization to allow cardiac transplant is feasible and efficient despite end-stage heart failure.

Cardiac magnetic resonance (CMR) has emerged as new mainstream technique for the evaluation of patients with cardiac diseases, providing unique information to support clinical decision-making. This document has been developed by a joined group of experts of the Italian Society of Cardiology and Italian society of Radiology and aims to produce an updated consensus statement about the current state of technology and clinical applications of CMR. The writing committee consisted of members and experts of both societies who worked jointly to develop a more integrated approach in the field of cardiac radiology. Part 1 of the document will cover ischemic heart disease, congenital heart disease, cardio-oncology, cardiac masses and heart transplant.

The COVID-19 pandemic highlights the critical need for preparation and innovation against a developing major healthcare challenge. We review our cardiology division’s response at our tertiary hospital center below, paying special emphasis to a practical operational workflow achievable by most cardiac centers across the globe.

For almost half a century, cardiac transplant has been the only long-term treatment for patients with end-stage heart failure. Implantable left ventricular assist devices (LVADs) have emerged as a new treatment option for advanced heart failure as destination therapy for patients either too old or not suitable for transplant. A meta-analysis presenting head-to-head comparisons of cardiac transplant versus LVAD as destination therapy (LVAD-DT) found no difference in 1-year mortality rates between LVAD-DT and cardiac transplant (OR 1.49; 95% CI [0.48–4.66]; I2=82.8%). Moreover, a recent subanalysis from the Interagency Registry for Mechanically Assisted Circulatory Support found similar outcomes after LVAD-DT implantation in both transplant and non-transplant centres. The time is right for LVAD-DT in non-transplant centres, provided multidisciplinary heart failure teams and expertise are in place.

Background Arrhythmogenic right ventricular cardiomyopathy/dysplasia ( ARVC /D) is characterized by high arrhythmic burden and progressive heart failure, which can prompt referral for heart transplantation. Cardiopulmonary exercise testing ( CPET ) has an established role in risk stratification for advanced heart failure therapies, but has not been described in ARVC /D. This study sought to determine the safety and prognostic utility of CPET in patients with ARVC /D. Methods and Results Using the Johns Hopkins ARVC /D Registry, we examined patients with ARVC /D undergoing CPET . Baseline characteristics and transplant‐free survival were compared on the basis of peak oxygen consumption (pVO2) (≤14 or >14 mL/kg per minute) and ventilatory efficiency (Ve/ VCO 2 slope ≤34 or >34). Thirty‐eight patients underwent 50 CPET s. There were no sustained arrhythmic events. Twenty‐nine patients achieved a maximal test. Patients with pVO2 ≤14 mL/kg per minute were more often men ( P =0.042) compared with patients with pVO2 >14 mL/kg per minute. Patients with Ve/ VCO 2 slope >34 tended to have more moderate/severe right ventricular dilation (7/9 [78%] versus 10/26 [38%]; P =0.060) and clinical heart failure (8/9 [89%] versus 13/26 [50%]; P =0.056) compared with patients with Ve/ VCO 2 slope ≤34. Patients who underwent heart transplantation were more likely to have clinical heart failure (10/10 [100%] versus 13/28 [46%]; P =0.003). Patients with Ve/ VCO 2 slope >34 had worse transplant‐free survival compared with patients with Ve/ VCO 2 slope ≤34 (n=35; hazard ratio, 6.57 [95% CI , 1.28–33.72]; log‐rank P =0.010), whereas transplant‐free survival was similar on the basis of pVO2 groups (n=29; hazard ratio, 3.38 [95% CI , 0.75–15.19]; log‐rank P =0.092). Conclusions CPET is safe to perform in patients with ARVC /D. Ve/ VCO 2 slope may be used for risk stratification and guide referral for heart transplantation in ARVC /D.

Background Implantable cardiac defibrillators (ICD) are a popular and effective option in heart failure with left ventricular systolic dysfunction patients. Although frequently underdiagnosed, inadvertent malposition can lead to endocardial damage and thrombotic events. As ICD implants tend to increase in the following years, the recognition of their complications is critical. Case presentation The authors present a case of a 64-year-old woman with advanced heart failure and ICD malposition. This accidental discovery was denounced by the presence of a right bundle branch block pattern and later confirmed by echocardiography which showed the lead tip in contact with the mid segment of the left ventricular antero-lateral wall. As the patient hospitalisation was complicated with refractory ascites and cardiogenic shock, she underwent cardiac transplantation, with no recurrence of heart failure symptoms. Conclusions An electrocardiogram showing a right bundle branch block pattern during VVI pacing should arise the suspicion of inadvertent placement of a pacing/ICD lead. The many facets of echocardiography should be used for the diagnosis of this complication, as they were paramount in this case, as highlighted.

Heart failure is one of the major causes of high morbidity and mortality. In infants and children, heart failure can develop as a consequence to a many number of complex cyanotic heart diseases and cardiomyopathies. In these conditions, heart transplantation offers a definitive cure . With increasing awareness and recognition of these conditions, pediatric heart transplantation is slowly shaping up in India. This review briefly describes the indications and selection criteria for pediatric heart transplantation in children.

ObjectivesUltra-small superparamagnetic particles of iron oxide (USPIO)-enhanced MRI can detect cellular inflammation within tissues and may help non-invasively identify cardiac transplant rejection. Here, we aimed to determine the normal reference values for USPIO-enhanced MRI in patients with a prior cardiac transplant and examine whether USPIO-enhanced MRI could detect myocardial inflammation in patients with transplant rejection.MethodsTen volunteers and 11 patients with cardiac transplant underwent T2, T2* and late gadolinium enhancement 1.5T MRI, with further T2* imaging at 24 hours after USPIO (ferumoxytol, 4 mg/kg) infusion, at baseline and 3 months.ResultsTen patients with clinically stable cardiac transplantation were retained for analysis. Myocardial T2 values were higher in patients with cardiac transplant versus healthy volunteers (53.8±5.2 vs 48.6±1.9 ms, respectively; p=0.003). There were no differences in the magnitude of USPIO-induced change in R2* in patients with transplantation (change in R2*, 26.6±7.3 vs 22.0±10.4 s-1 in healthy volunteers; p=0.28). After 3 months, patients with transplantation (n=5) had unaltered T2 values (52.7±2.8 vs 52.12±3.4 ms; p=0.80) and changes in R2* following USPIO (29.42±8.14 vs 25.8±7.8 s-1; p=0.43).ConclusionStable patients with cardiac transplantation have increased myocardial T2 values, consistent with resting myocardial oedema or fibrosis. In contrast, USPIO-enhanced MRI is normal and stable over time suggesting the absence of chronic macrophage-driven cellular inflammation. It remains to be determined whether USPIO-enhanced MRI may be able to identify acute cardiac transplant rejection.Trial registration numberNCT02319278349 (https://clinicaltrials.gov/ct2/show/NCT02319278) Registered 03.12.2014 EUDraCT 2013-002336-24.

Background Left ventricular assist devices (LVAD) provide a lifesaving bridge to cardiac transplant. Utilization of these devices is increasing in the United States. When a patient undergoes cardiac transplant, the left ventricular device is surgically removed and the driveline is extracted or left tunneled in the subcutaneous tissue. Our group encountered a rare and previously unreported complication of this device: intraperitoneal infiltration of a retained driveline after cardiac transplant causing a small bowel obstruction. Case presentation A 62 year old male with a past medical history of non-ischemic cardiomyopathy induced heart failure, status post bridging left ventricular assist device and orthotopic heart transplant presented with abdominal distention, tenderness, and leukocytosis six days post-transplant. CT abdomen and pelvis revealed dilated loops of bowel, air-fluid levels and a transition point in the proximal small bowel. The patient was diagnosed with small bowel obstruction and taken for exploratory laparotomy. He was found to have a retained intraabdominal LVAD driveline strangulating a loop of small bowel in the left upper quadrant. The driveline was removed and the section of bowel released with return of perfusion. Conclusions We had encountered a rare complication of retained left ventricular assist device driveline after cardiac transplant: inadvertent penetration into the peritoneal cavity resulting in strangulation of small bowel. This complication, though uncommon, provides substantial risk to patients previously treated with left ventricular assist devices. Meticulous care must be taken to ensure proper device insertion and extraction, as well as consideration of this etiology when patients present with bowel obstruction after cardiac transplant.

Cardiac transplantation has revolutionized the treatment of end-stage heart failure since was it first performed in 1967 by Dr. Christian Baarnard [(1)][1]. Operative techniques have developed over the years, with the initial description of the biatrial technique by Lower et al. [(2)][2] to the

Cardiogenic shock is a pathophysiologic state where an abnormality of cardiac function is responsible for the failure of the cardiovascular system to meet the metabolic needs of the body tissues.Though it is less common than hypovolemia as the primary etiology in paediatric shock, eventually myocardial function is affected because of reduced perfusion in all forms of shock. Myocardial malfunction, in other forms of shock, is secondary to ischemia, acidosis, drugs, toxins or inflammation. Cardiogenic shock is a low output state characterized by elevated filling pressures, neurohormonal activation with the evidence of end-organ hypoperfusion. The management is challenging and consists of a combination of conventional cardio-respiratory support, vasoactive medications with correction of the anatomic cardiac defects. Treatment options like Extracorporeal membrane oxygenation and Ventricular assist devices provide a bridge to recovery, surgery or transplant. As cardiogenic shock in children carries a high risk of morbidity and mortality, emphasis should be placed on expedient management to arrest the pathophysiological cascade and avoid hypotension.This article aims to review the aetio-pathophysiological basis of pediatric cardiogenic shock, diagnostic options, recent advances in management modalities and outcome.

In this review, we will discuss recent developments in noninvasive molecular imaging of immune-mediated changes seen in cardiac allograft rejection. The significant developments of molecular imaging agents across various modalities, including MRI, PET/SPECT, optical imaging, and echocardiography, provide us with opportunities to image specific immunological pathways of cardiac allograft rejection that were previously not possible. Recent studies using novel molecular imaging modalities have shown us new understanding of immune-mediated mechanisms involved in allograft rejection. Though these studies largely have been performed in animal models, use of molecular imaging is ripe for clinical translation to provide more personalized detection and monitoring of immune processes in heart-transplant patients. This review evaluates the recent application of molecular imaging to target distinct pathways of acute cellular rejection and cardiac allograft vasculopathy that have the potential to be translated into routine clinical use.

AIMS: Techniques for identifying specific microcirculatory structural changes are desirable. As such, capillary rarefaction constitutes one of the earliest changes of cardiac allograft vasculopathy (CAV) in cardiac allograft recipients, but its identification with coronary flow reserve (CFR) or intracoronary resistance measurements is hampered because of non-selective interrogation of the capillary bed. We therefore investigated the potential of wave intensity analysis (WIA) to assess capillary rarefaction and thereby predict CAV. METHODS AND RESULTS: Fifty-two allograft patients with unobstructed coronary arteries and normal left ventricular (LV) function were assessed. Adequate aortic pressure and left anterior descending artery flow measurements at rest and with intracoronary adenosine were obtained in 46 of which 2 were lost to follow-up. In a subgroup of 15 patients, simultaneous RV biopsies were obtained and analysed for capillary density. Patients were followed up with 1–3 yearly screening angiography. A significant relationship with capillary density was noted with CFR (r = 0.52, P = 0.048) and the backward decompression wave (BDW) (r = −0.65, P

Background The epidemiology and outcomes of acute kidney injury (AKI) in prevalent non-renal solid organ transplant recipients is unknown. Methods We assessed the epidemiology of trends in acute kidney injury (AKI) in orthotopic cardiac and liver transplant recipients in the United States. We used the Nationwide Inpatient Sample to evaluate the yearly incidence trends (2002 to 2013) of the primary outcome, defined as AKI requiring dialysis (AKI-D) in hospitalizations after cardiac and liver transplantation. We also evaluated the trend and impact of AKI-D on hospital mortality and adverse discharge using adjusted odds ratios (aOR). Results The proportion of hospitalizations with AKI (9.7 to 32.7% in cardiac and 8.5 to 28.1% in liver transplant hospitalizations; ptrend

The aim of the study was to analyze possible correlations between strain echocardiography (STE) and PET myocardial perfusion in a population of heart transplantation (HTx) recipients showing preserved left ventricular (LV) ejection fraction. By STE, LV global longitudinal strain (LV GLS) was lower in HTx. PET showed no transient or chronic ischemia in 83 of 115 HTx (73%). Fixed perfusion defects were observed in 17% of HTx and reversible ischemia in 10%. Significant coronary stenosis was observed only in 10 cases. GLS was independently associated with age at HTx and fixed perfusion defects (HR 0.41; P

The objective of the present work is to describe the experience with intravenous (IV) sildenafil in heart transplant (HT) patients with reactive pulmonary hypertension (PH) who developed right ventricular dysfunction (RVD) in the immediate postoperative period. The first 5 patients who received IV sildenafil followinga HT are presented. The HTs took place between March 2011 and September 2012 in patients aged 37 to 64 years; all patients were male. Prior to the HT, mean pulmonary artery pressure (mPAP) was 32-56 mmHg. In all cases, the hemodynamic study demonstrated PH reactivity (positive vasodilator test with nitric oxide). All 5 patients developed RVD with hemodynamic instability immediately after the HT, despite the administration of nitric oxide from the time of intubation prior to the implant, optimal medical treatment in all cases, and a ventricular assist in 2 cases. In all patients, IV sildenafil was initiated at 10 mg/8 h for 48 h and was subsequently increased to 20 mg/8 h. in its oral formulation until discharge from the hospital. The change in pulmonary pressure was assessed using a Swan-Ganz catheter. Ventricular function was assessed using echocardiography. Length of stay in the Resuscitation Unit and mid-term survival were also assessed. Average time of extracorporeal circulation was 200 ± 110 min and organ ischemic time was 210 ± 95 min. All of the patients demonstrated pulmonary and systemic hemodynamic improvement, as well as recovery of right ventricular function after completing the treatment with IV sildenafil. The stay in the Resuscitation Unit lasted 3-25 days. All the patients were discharged from hospital with no mortality to date. Intravenous sildenafil improves right ventricle hemodynamics associated with pulmonary hypertension post-HT. Prophylactic prevention with this drug could be indicated for patients with reactive PH who are about to receive a transplant.

FUNDAMENTO: A hipertensão pulmonar é associada ao pior prognóstico no pós-transplante cardíaco. O teste de reatividade pulmonar com Nitroprussiato de Sódio (NPS) está associado a elevados índices de hipotensão arterial sistêmica, disfunção ventricular do enxerto transplantado e elevadas taxas de desqualificação para o transplante. OBJETIVO: Neste estudo, objetivou-se comparar os efeitos do Sildenafil (SIL) e NPS sobre variáveis hemodinâmicas, neuro-hormonais e ecocardiográficas durante teste de reatividade pulmonar. MÉTODOS: Os pacientes foram submetidos, simultaneamente, ao cateterismo cardíaco direito, ao ecocardiograma e à dosagem de BNP e gasometria venosa, antes e após administração de NPS (1 - 2 µg/Kg/min) ou SIL (100 mg, dose única). RESULTADOS: Ambos reduziram a hipertensão pulmonar, porém o nitrato promoveu hipotensão sistêmica significativa (Pressão Arterial Média - PAM: 85,2 vs. 69,8 mmHg, p < 0,001). Ambos reduziram as dimensões cardíacas e melhoraram a função cardíaca esquerda (NPS: 23,5 vs. 24,8 %, p = 0,02; SIL: 23,8 vs. 26 %, p < 0,001) e direita (SIL: 6,57 ± 2,08 vs. 8,11 ± 1,81 cm/s, p = 0,002; NPS: 6,64 ± 1,51 vs. 7,72 ± 1,44 cm/s, p = 0,003), medidas pela fração de ejeção ventricular esquerda e Doppler tecidual, respectivamente. O SIL, ao contrário do NPS, apresentou melhora no índice de saturação venosa de oxigênio, medido pela gasometria venosa. CONCLUSÃO: Sildenafil e NPS são vasodilatadores que reduzem, de forma significativa, a hipertensão pulmonar e a geometria cardíaca, além de melhorar a função biventricular. O NPS, ao contrário do SIL, esteve associado a hipotensão arterial sistêmica e piora da saturação venosa de oxigênio. BACKGROUND: Pulmonary hypertension is associated with a worse prognosis after cardiac transplantation. The pulmonary hypertension reversibility test with sodium nitroprusside (SNP) is associated with a high rate of systemic arterial hypotension, ventricular dysfunction of the transplanted graft and high rates of disqualification from transplantation. OBJECTIVE: This study was aimed at comparing the effects of sildenafil (SIL) and SNP on hemodynamic, neurohormonal and echocardiographic variables during the pulmonary reversibility test. METHODS: The patients underwent simultaneously right cardiac catheterization, echocardiography, BNP measurement, and venous blood gas analysis before and after receiving either SNP (1 - 2 µg/kg/min) or SIL (100 mg, single dose). RESULTS: Both drugs reduced pulmonary hypertension, but SNP caused a significant systemic hypotension (mean blood pressure - MBP: 85.2 vs. 69.8 mm Hg; p < 0.001). Both drugs reduced cardiac dimensions and improved left cardiac function (SNP: 23.5 vs. 24.8%, p = 0.02; SIL: 23.8 vs. 26%, p < 0.001) and right cardiac function (SIL: 6.57 ± 2.08 vs. 8.11 ± 1.81 cm/s, p = 0.002; SNP: 6.64 ± 1.51 vs. 7.72 ± 1.44 cm/s, p = 0.003), measured through left ventricular ejection fraction and tissue Doppler, respectively. Sildenafil, contrary to SNP, improved venous oxygen saturation, measured on venous blood gas analysis. CONCLUSION: Sildenafil and SNP are vasodilators that significantly reduce pulmonary hypertension and cardiac geometry, in addition to improving biventricular function. Sodium nitroprusside, contrary to SIL, was associated with systemic arterial hypotension and worsening of venous oxygen saturation.

Dobutamine stress echocardiography (DSE) is widely used during follow-up after cardiac transplant for the diagnosis of allograft vasculopathy. We investigated the effect of donor–recipient age difference on the ability to reach target heart rate (HR) during DSE. All cardiac transplant patients who were undergoing DSE over a 3-year period in a single institution were reviewed. Target HR was specified as 85%×(220–patient age). Further patient and donor demographics were obtained from the local transplant database. 61 patients (45 male, 55±12 years) were stressed with a median dose of 40mcg/kg per min dobutamine. Only 37 patients (61%) achieved target HR. Donor hearts were mostly younger (mean 41±14 years, PPP=0.003) than those with younger donors did. Patients with older donors were less likely to achieve target HR (18% vs 67%, P=0.003). In conclusion, donor–recipient age difference affects the likelihood of achieving target HR and should be considered when a patient is consistently unable to achieve ‘adequate’ stress according to the patient’s age.

Sugammadex is a novel agent for the reversal of neuromuscular blockade. The speed and efficacy of reversal with sugammadex are significantly faster than acetylcholinesterase inhibitors, such as neostigmine. Sugammadex also has a limited adverse profile when compared with acetylcholinesterase inhibitors, specifically in regard to the incidence of bradycardia. This adverse effect may be particularly relevant in the setting of a heart transplant recipient with a denervated heart. The authors present a case of an 8-year-old child, status postcardiac transplantation, who required anesthetic care for laparoscopy and lysis of intra-abdominal adhesions. Sugammadex was used to reverse neuromuscular blockade and avoid the potential adverse effects of neostigmine. The unique mechanism of action of sugammadex is discussed, previous reports of its use in this unique patient population are reviewed, and its potential benefits compared to traditional acetylcholinesterase inhibitors are presented.

BACKGROUND: The right heart is a major determinant of prognosis in cardiac transplant recipient patients. AIM: To investigate right ventricular morphology and function and their relationship with exercise capacity in cardiac transplant recipient patients using standard tranthoracic echocardiography and a new three-dimensional echocardiographic software adapted for right ventricular analysis. METHODS: One hundred fifteen relatively stable cardiac transplant recipient patients (71 men; 58.3 ± 5.8 years; 7.8 ± 4.5 years after transplantation) and 80 healthy age-comparable and sex-comparable controls underwent standard echocardiography, tissue Doppler imaging (TDI), and three-dimensional echocardiography, focused on the right ventricular analysis. Along with left heart parameters, right ventricular measurements included end-diastolic diameters at basal and mid-cavity level; base-to-apex length; tricuspid annulus plane systolic excursion (TAPSE); TDI right ventricular systolic peak velocity (Sm); and three-dimensional ejection fraction. Using the peak systolic tricuspid regurgitation velocity (TRV) and the end-diastolic pulmonary regurgitation velocity, the modified Bernoulli equation was used to calculate the pulmonary artery systolic (PASP) and diastolic pressures. Pulmonary artery vascular conductance (PAVC) was estimated by left ventricular stroke volume/4 × (TRV - pulmonary regurgitation velocity). RESULTS: Left ventricular diameters and ejection fraction did not significantly differ between the two groups, whereas mass index was increased in cardiac transplant recipient patients (P

Nephropathy secondary to BK virus, a member of the Papoviridae family of viruses, has been recognized for some time as an important cause of allograft dysfunction in renal transplant recipients. In recent times, BK nephropathy (BKN) of the native kidneys has being increasingly recognized as a cause of chronic kidney disease in patients with solid organ transplants, bone marrow transplants and in patients with other clinical entities associated with immunosuppression. In such patients renal dysfunction is often attributed to other factors including nephrotoxicity of medications used to prevent rejection of the transplanted organs. Renal biopsy is required for the diagnosis of BKN. Quantitation of the BK viral load in blood and urine are surrogate diagnostic methods. The treatment of BKN is based on reduction of the immunosuppressive medications. Several compounds have shown antiviral activity, but have not consistently shown to have beneficial effects in BKN. In addition to BKN, BK viral infection can cause severe urinary bladder cystitis, ureteritis and urinary tract obstruction as well as manifestations in other organ systems including the central nervous system, the respiratory system, the gastrointestinal system and the hematopoietic system. BK viral infection has also been implicated in tumorigenesis. The spectrum of clinical manifestations from BK infection and infection from other members of the Papoviridae family is widening. Prevention and treatment of BK infection and infections from other Papovaviruses are subjects of intense research.

Aortic complications occur rarely after pediatric orthotopic heart transplantation, but are typically accompanied by catastrophic events. We describe the three cases of major aortic complications in our experience of 329 pediatric heart transplants. This case series and review highlight the important risk factors for aortic complications after heart transplantation.

Prior studies have demonstrated associations between beta-adrenergic receptor (βAR) polymorphisms and left ventricular dysfunction-an important cause of allograft nonutilization for transplantation. We hypothesized that βAR polymorphisms predispose donor hearts to LV dysfunction after brain death. A total of 1043 organ donors managed from 2001-2006 were initially studied. The following βAR single nucleotide polymorphisms were genotyped: β1AR 1165C/G (Arg389Gly), β1AR 145A/G (Ser49Gly), β2AR 46G/A (Gly16Arg) and β2AR 79C/G (Gln27Glu). In multivariable regression analyses, the β2AR46 SNP was significantly associated with LV systolic dysfunction, with each minor allele additively decreasing the odds for LV ejection fraction 10 μg/kg/min of dopamine compared to those with the CC and GG genotypes. However, no significant associations were found between βAR SNPs and cardiac dysfunction in 364 donors managed from 2007-2008, perhaps due to changes in donor management, lack of power in this validation cohort, or the absence of a true association. βAR polymorphisms may be associated with cardiac dysfunction after brain death, but these relationships require further study in independent donor cohorts.

Aims Hypertrophic cardiomyopathy (HCM) is an important cause of heart failure-related disability over a wide range of ages. Profiles of severe progressive heart failure symptoms and death, or heart transplantation deserve more complete definition within large patient cohorts. Methods and results Clinical and morphological features of heart failure were assessed in 293 consecutive HCM patients over a median follow-up of 6 (inter-quartile range 2–11) years. Gross and histopathological features were analysed in 12 patients for whom the heart was available for inspection. Of the 293 patients, 50 (17%) developed severe progressive heart failure, including 18 who died or were transplanted. Three profiles of heart failure were identified predominantly associated with: (i) end-stage systolic dysfunction (ejection fraction

CARDIAC involvement in patients with inherited myopathies is well described in the literature. In dystrophinopathies (Duchenne muscular dystrophy [DMD], Becker muscular dystrophy [BMD], and DMD/ BMD carriers), dilated cardiomyopathy (DCM) has been reported in association with muscular dystrophy. In addition, in cases with particularly mild skeletal muscle involvement, cardiomyopathy may be the primary clinical feature and the main clinical problem. In mitochondrial myopathies, cardiac conduction defects have been described in many patients, whereas both hypertrophic (HCM) and DCM have been reported infrequently. In desminopathies, cardiomyopathy with conduction abnormalities is frequently associated with myopathy and may predominate. Up to now, end-stage cardiomyopathy resulting from dystrophinopathies, desminopathies, and mitochondrial myopathies have been accepted for heart transplantation (HTx) with reluctance because of a high perioperative risk caused by physical disability and restrictive respiratory failure. Moreover, a suspected rapid onset of cardiomyopathy of the transplanted heart is a further concern. Here we report five cases of end-stage cardiomyopathy with mild to moderate myopathies, who successfully underwent heart transplantation and were followed up for 72 months after surgery.

Patient survival after cardiac, liver, and hematopoietic stem cell transplant (HSCT) is improving; however, this survival is limited by substantial pretransplant and treatment-related toxicities. A major cause of morbidity and mortality after transplant is chronic kidney disease (CKD). Although the majority of CKD after transplant is attributed to the use of calcineurin inhibitors, various other conditions such as thrombotic microangiopathy, nephrotic syndrome, and focal segmental glomerulosclerosis have been described. Though the immunosuppression used for each of the transplant types, cardiac, liver and HSCT is similar, the risk factors for developing CKD and the CKD severity described in patients after transplant vary. As the indications for transplant and the long-term survival improves for these children, so will the burden of CKD. Nephrologists should be involved early in the pretransplant workup of these patients. Transplant physicians and nephrologists will need to work together to identify those patients at risk of developing CKD early to prevent its development and progression to end-stage renal disease.

Constrictive pericarditis is a disease characterized by progressive pericardial fibrosis. If left untreated it can lead to progressive heart failure and can be severely disabling. Medical management with non-steroidal anti-inflammatory drugs in combination with colchicine is promising in the acute phase of the disease but for more chronic cases pericardiectomy offers the best chance for hemodynamic recovery. Constrictive pericarditis after cardiac transplantation is a rare phenomenon. Current literature suggests that early pericardiectomy may be the most effective treatment in this subset of patients as well.