Pediatric patient with restrictive cardiomyopathy on staged biventricular assist device support with Berlin Heart EXCOR® underwent heart transplantation successfully: the first case in Japan.

Pure restrictive cardiomyopathy is a strong risk factor for poor outcomes in children with cardiomyopathy on ventricular assist devices. Owing to concomitant right heart failure, children with end-staged restrictive cardiomyopathy who are supported with a ventricular assist device often require a biventricular assist device, which is another risk factor for waitlist mortality in heart transplantation candidates. Herein, we report the case of a 3-year-old boy with pure restrictive cardiomyopathy who successfully underwent heart transplantation after 12 months of support with staged biventricular assist devices. Owing to the progression of diastolic dysfunction, the left ventricular assist device could not provide adequate circulation support. Despite the provision of biventricular assist device support, the patient required a complex management strategy that involved balancing the left and right ventricular assist device supports. We were able to stabilize the patient by careful synchronization of the supports and proceeded to heart transplantation. TRIAL REGISTRATION: Clinical Registration No.: Institutional Review Board of Osaka University Hospital, approval no. 16105.

A nonrandomized open-label phase 2 trial of nonischemic heart preservation for human heart transplantation.

Pre-clinical heart transplantation studies have shown that ex vivo non-ischemic heart preservation (NIHP) can be safely used for 24 h. Here we perform a prospective, open-label, non-randomized phase II study comparing NIHP to static cold preservation (SCS), the current standard for adult heart transplantation. All adult recipients on waiting lists for heart transplantation were included in the study, unless they met any exclusion criteria. The same standard acceptance criteria for donor hearts were used in both study arms. NIHP was scheduled in advance based on availability of device and trained team members. The primary endpoint was a composite of survival free of severe primary graft dysfunction, free of ECMO use within 7 days, and free of acute cellular rejection ≥2R within 180 days. Secondary endpoints were I/R-tissue injury, immediate graft function, and adverse events. Of the 31 eligible patients, six were assigned to NIHP and 25 to SCS. The median preservation time was 223 min (IQR, 202-263) for NIHP and 194 min (IQR, 164-223) for SCS. Over the first six months, all of the patients assigned to NIHP achieved event-free survival, compared with 18 of those assigned to SCS (Kaplan-Meier estimate of event free survival 72.0% [95% CI 50.0-86.0%]). CK-MB assessed 6 ± 2 h after ending perfusion was 76 (IQR, 50-101) ng/mL for NIHP compared with 138 (IQR, 72-198) ng/mL for SCS. Four deaths within six months after transplantation and three cardiac-related adverse events were reported in the SCS group compared with no deaths or cardiac-related adverse events in the NIHP group. This first-in-human study shows the feasibility and safety of NIHP for clinical use in heart transplantation. ClinicalTrial.gov, number NCT03150147.

Complications of retained pacemaker hardware in heart transplant recipients: case series and review of the literature.

Heart transplantation (HT) in patients with prior cardiovascular implantable electronic devices (CIEDs) is becoming more common, in parallel with the increased use of CIEDs for patients with advanced heart failure. Complete removal of CIED components during HT is not always feasible, and it is thus surprising that the literature addressing the implications of retained CIED components is limited. Indeed, there are neither guidelines nor consensus regarding the need for removal of these CIED fragments. This issue is nonetheless becoming important in light of the increase in the percentage of HT candidates having CIEDs, on the one hand, and newer therapeutic immunosuppressive protocols with higher bacterial infection potential, on the other. Thus, with the aim to study the implications of retained CIED fragments as a step towards establishing a therapeutic approach for the unique population of HT recipients with CIED remnants, we present here a case series of HT patients with retained CIED fragments who developed complications of infections together with a review of the available literature.

Intraventricular placement of a spring expander does not attenuate cardiac atrophy of the healthy heart induced by unloading via heterotopic heart transplantation.

An important complication of the prolonged left ventricle assist device support in patients with heart failure is unloading-induced cardiac atrophy which proved resistant to various treatments. Heterotopic heart transplantation (HTx) is the usual experimental model to study this process. We showed previously that implantation of the newly designed intraventricular spring expander can attenuate the atrophy when examined after HTx in the failing heart (derived from animals with established heart failure). The present study aimed to examine if enhanced isovolumic loading achieved by implantation of the expander would attenuate cardiac post-HTx atrophy also in the healthy heart. Cardiac atrophy was assessed as the ratio of the transplanted-to-native heart weight (HW) and its degree was determined on days 7, 14, 21 and 28 after HTx. The transplantation resulted in 32±3, 46±2, 48±3 and 46±3 % HW loss when measured at the four time points; implantation of the expander had no significant effect on these decreases. We conclude that enhanced isovolumic loading achieved by intraventricular implantation of the expander does not attenuate the development of cardiac atrophy after HTx in the healthy heart. This indicates that such an approach does not represent a useful therapeutic measure to attenuate the development of unloading-induced cardiac atrophy.

Minimally invasive biventricular mechanical circulatory support with Impella pumps as a bridge to heart transplantation: a first-in-the-world case report.

Cardiogenic shock from biventricular failure that requires acute mechanical circulatory support carries high 30 day mortality. Acute mechanical circulatory support can serve as bridge to orthotopic heart transplant (OHT) in selected patients. We report a patient with biventricular failure secondary to rapidly progressive cardiac sarcoidosis refractory to medical management who was bridged to OHT with Impella 5.0 and Impella RP-temporary left and right ventricular assist devices, respectively. This is the first successful bridge to transplantation using these devices in biventricular heart failure and cardiogenic shock. We discuss considerations for using this strategy over veno-arterial extracorporeal membrane oxygenation or surgically implanted assist devices in patients with cardiogenic shock and biventricular failure as a bridge to OHT.

The Right Tool for the Right Job: Bridging a Failing Fontan to Transplant.

Mechanical circulatory support and heart transplantation are both becoming increasingly common among Fontan patients as this growing cohort of patients is reaching young adulthood. Although device placement may present challenges, the new focus is on selecting the correct cannulation sites and devices at the correct time to successfully bridge these challenging patients to heart transplant. Herein, we report the use of a number of mechanical circulatory support strategies tailored to the clinical setting that culminated in 130 days of support before cardiac transplantation of a young adult with a failing Fontan circulation.

An original valveless artificial heart providing pulsatile flow tested in mock circulatory loops.

PURPOSE: We present the test bench results of a valveless total artificial heart that is potentially compatible with the pediatric population. METHODS: The RollingHeart is a valveless volumetric pump generating pulsatile flow. It consists of a single spherical cavity divided into 4 chambers by 2 rotating disks. The combined rotations of both disks produce changes in the volumes of the 4 cavities (suction and ejection). The blood enters/exits the spherical cavity through 4 openings that are symmetrical to the fixed rotation axis of the first disk.Mock circulatory system: The device pumps a 37% glycerin solution through 2 parallel circuits, simulating the pulmonary and systemic circulations. Flow rates are acquired with a magnetic inductive flowmeter, while pressure sensors collect pressure in the left and right outflow and inflow tracts.In vitro test protocol: The pump is run at speeds ranging from 20 to 180 ejections per minute. The waveform of the pressure generated at the inflow and outflow of the 4 chambers and the flow rate in the systemic circulation are measured. RESULTS: At an ejection rate of 178 min-1, the RollingHeart pumps 5.3 L/min for a systemic maximal pressure gradient of 174 mmHg and a pulmonary maximal pressure gradient of 75 mmHg. The power input was 14 W, corresponding to an efficiency of 21%. CONCLUSIONS: The RollingHeart represents a new approach in the domain of total artificial heart. This preliminary study endorses the feasibility of a single valveless device acting as a total artificial heart.

'Being' a ventricular assist device recipient: A liminal existence.

Ventricular assist devices (VADs) are playing an increasing role in the management of heart failure. VADs are mechanical circulatory devices that support or replace the function of a failing heart. Currently, VADs are only offered in theUnited Kingdom (UK) to patients waiting for a heart transplant; however, the use of these devices is likely to increase in the near future. Presently, there is a dearth of literature exploring the day-to-day realities of living with a VAD, which will become increasingly important as the role of VADs is increased. This paper adopts an interpretive phenomenological approach to uncover the experience of 'Being' a VAD recipient. Semi-structured interviews were conducted with 20 VAD recipients. The overarching theme is that life with a VAD is a liminal existence. This comprised four subthemes: the first examines how the VAD imposes limitations on recipients' lives that can precipitate a loss of identity; the second focuses on temporal disruptions, recipients' sense of time changes from authentic to inauthentic; the third explores how the VAD itself is liminal, it is positioned as temporary rather than as the 'answer' to the condition; and finally, we discuss VAD recipients' projections to the future and the possibility of an end to the experience of liminality.

The Carmat Bioprosthetic Total Artificial Heart Is Associated With Early Hemostatic Recovery and no Acquired von Willebrand Syndrome in Calves.

OBJECTIVES: To determine hemostasis perturbations, including von Willebrand factor (VWF) multimers, after implantation of a new bioprosthetic and pulsatile total artificial heart (TAH). DESIGN: Preclinical study SETTING: Single-center biosurgical research laboratory. PARTICIPANTS: Female Charolais calves, 2-to-6 months old, weighing 102-to-122 kg. INTERVENTIONS: Surgical implantation of TAH through a mid-sternotomy approach. MEASUREMENTS AND MAIN RESULTS: Four of 12 calves had a support duration of several days (4, 4, 8, and 10 days), allowing for the exploration of early steps of hemostasis parameters, including prothrombin time; coagulation factor levels (II, V, VII+X, and fibrinogen); and platelet count. Multimeric analysis of VWF was performed to detect a potential loss of high-molecular weight (HMW) multimers, as previously described for continuous flow rotary blood pumps. Despite the absence of anticoagulant treatment administered in the postoperative phase, no signs of coagulation activation were detected. Indeed, after an immediate postsurgery decrease of prothrombin time, platelet count, and coagulation factor levels, most parameters returned to baseline values. HMW multimers of VWF remained stable either after initiation or during days of support. CONCLUSIONS: Coagulation parameters and platelet count recovery in the postoperative phase of the Carmat TAH (Camat SA, Velizy Villacoublay Cedex, France) implantation in calves, in the absence of anticoagulant treatment and associated with the absence of decrease in HMW multimers of VWF, is in line with early hemocompatibility that is currently being validated in human clinical studies.

From left ventricular assist device to transplant to home: An interdisciplinary team approach to treating medically complex cardiac patients during the bridge to transplantation: A case report.

CASE REPORT: A 56-year-old man presented with advanced heart failure requiring implantation of a left ventricular assist device and was discharged home. after implantation He returned to the hospital with right posterior putamen stroke and after a comprehensive rehabilitation programme was discharged home with the ability to be independent. The patient later underwent heart transplantation and subsequently required readmission to inpatient rehabilitation. He again completed a comprehensive rehabilitation programme and returned home with the ability to be independent with use of a single-point cane for ambulation. CONCLUSION: Patients with advanced heart failure present a challenge to the rehabilitation team, but with a multidisciplinary approach and physiatrist leadership patients can return home and achieve improved quality of life.

Trasplante cardíaco en una paciente joven con diagnóstico de esclerosis sistémica difusa / Cardiac transplant in young female patient diagnosed with diffuse systemic sclerosis

La esclerosis sistémica (ES) en una enfermedad autoinmune, crónica, multifactorial y sistémica que afecta al tejido conectivo. Presentamos este caso clínico dado la baja prevalencia de ES difusa con compromiso cardíaco temprano y progresivo en una paciente joven, y tratamiento con trasplante cardíaco (AU)

Systemic sclerosis (SS) in a multifactorial and systemic, chronic, autoimmune disease that affects the connective tissue. We present this clinical case given the low prevalence of diffuse SS with early and progressive cardiac compromise in a young patient, and treatment with cardiac transplantation (AU)

[Cardiac support and replacement therapies]. / Organersatzverfahren - Kreislaufunterstützung und -ersatz.

Circulatory support represents an integral part within the treatment of the critically ill patient. Sophisticated pharmacologic regimens help to maintain systemic perfusion pressure by increasing vascular tone as well as mediating positive inotropic effects. Besides the administration of catecholamines and phosphodiesterase-III-inhibitors, in particular the administration of levosimendan represents a promising alternative during low-cardiac-output. Nevertheless, sufficient evidence demonstrating a survival benefit for any pharmacologic regimen is nonexistent. In case pharmacological measures do not suffice mechanical cardiopulmonary support (MCS) may be used. MCS may be used during cardiopulmonary resuscitation or a "low-cardiac-output-syndrome" as bridging towards decision, recovery or long-term support. Venoarterial extracorporeal membrane oxygenation (vaECMO) may take over cardiopulmonary function and may improve survival as well as neurological outcome after cardiogenic shock or cardiopulmonary resuscitation.

Bivalirudin Anticoagulation for a Pediatric Patient with Heparin-Induced Thrombocytopenia and Thrombosis Requiring Cardiopulmonary Bypass for Ventricular Assist Device Placement.

The direct thrombin inhibitor bivalirudin is an option for anticoagulation in patients with heparin induced thrombocytopenia (HIT) requiring cardiopulmonary bypass (CPB). There are a limited number of reports of pediatric patients in which bivalirudin has been used for anticoagulation for CPB. We present the case of an 11 year old male with acute onset heart failure secondary to idiopathic dilated cardiomyopathy that developed heparin induced thrombocytopenia with thrombosis (HITT). The patient was anticoagulated in the operating room with bivalirudin and placed on CPB for insertion of a HeartWare(®) Ventricular Assist Device (Heartware(®)). Modified techniques were utilized. This included use of the Terumo CDI 500 (Terumo Cardiovascular Systems, Inc.) in-line blood gas monitor which contains a heparin coated arterial shunt sensor. We flushed this sensor with buffered saline preoperatively and noted no significant decrease in platelet count postoperatively. The patient was successfully placed on the ventricular assist device and was subsequently listed for heart transplantation.

Acquired von Willebrand syndrome associated with left ventricular assist device.

Left ventricular assist devices (LVAD) provide cardiac support for patients with end-stage heart disease as either bridge or destination therapy, and have significantly improved the survival of these patients. Whereas earlier models were designed to mimic the human heart by producing a pulsatile flow in parallel with the patient's heart, newer devices, which are smaller and more durable, provide continuous blood flow along an axial path using an internal rotor in the blood. However, device-related hemostatic complications remain common and have negatively affected patients' recovery and quality of life. In most patients, the von Willebrand factor (VWF) rapidly loses large multimers and binds poorly to platelets and subendothelial collagen upon LVAD implantation, leading to the term acquired von Willebrand syndrome (AVWS). These changes in VWF structure and adhesive activity recover quickly upon LVAD explantation and are not observed in patients with heart transplant. The VWF defects are believed to be caused by excessive cleavage of large VWF multimers by the metalloprotease ADAMTS-13 in an LVAD-driven circulation. However, evidence that this mechanism could be the primary cause for the loss of large VWF multimers and LVAD-associated bleeding remains circumstantial. This review discusses changes in VWF reactivity found in patients on LVAD support. It specifically focuses on impacts of LVAD-related mechanical stress on VWF structural stability and adhesive reactivity in exploring multiple causes of AVWS and LVAD-associated hemostatic complications.

Evaluación de factores psiquiátricos y psicosociales en una cohorte de receptores de trasplante cardiaco: estudio de seguimiento a 12 meses / No disponible

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