Heart transplantation in juvenile-onset systemic sclerosis with primary cardiac involvement: report of two cases and comprehensive literature review.

Juvenile onset systemic sclerosis is a rare chronic multisystem connective tissue disease characterized by skin induration, microangiopathy, autoimmune disturbances and widespread fibrosis of internal organs. Primary cardiac involvement in systemic sclerosis (SSc) is associated with a variable phenotype, including heart failure and arrhythmias, which lead to poor short-term prognosis. Isolated heart transplantation is a rare approach for the treatment of advanced heart failure in patients with systemic sclerosis. We report on two juvenile SSc patients receiving cardiac transplantation due to heart failure with malignant arrhythmias. One patient presented with severe dilated cardiomyopathy with recurrent ventricular tachycardia. Following the appearance of Raynaud phenomenon, he was subsequently diagnosed a rare form of systemic sclerosis sine scleroderma, without cutaneous manifestations or other organs involved. His cardiac condition was unresponsive to antiarrhythmic therapy and immunosuppression used to treat SSc, therefore he underwent successful heart transplantation. The second patient presented diffuse scleroderma with mild pulmonary, esophageal and renal involvement. While extracardiac manifestations were effectively kept under control with immunosuppressive therapy, cardiac involvement rapidly progressed with detection of fibrosis at cardiac magnetic resonance imaging and appearance of severe ventricular arrhythmia. Herein, an extensive multidisciplinary evaluation was pivotal in defining the entity and clinical stability of extracardiac involvement, and thus the patient could profit from heart transplantation. Our experience highlights the importance of considering heart transplantation in carefully selected SSc patients with primary cardiac involvement as a lifesaving procedure.

Current Understanding and Future Directions of Transcatheter Devices to Assist Failing Fontan.

Even if the Fontan operation is the surgical treatment of choice in patients with univentricular physiology, it remains a palliative strategy. Consequently, when Fontan patients reach adulthood, the majority of them develop late clinical sequelae of a failing cavo-pulmonary circuit (eg, liver failure, protein-losing enteropathy, and arrhythmias). Although heart transplantation represents the gold standard to treat this condition, Fontan patients usually accede to this therapy late, when risk of mortality is significantly increased, and a shortage of donor hearts limits transplantation in this special population. Mechanical circulatory support is an emerging field, but it is still in the experimental stage. Current mechanical circulatory devices have been used in Fontan circulation but are associated with the need for high-risk redo surgery. Percutaneous pumps are an emerging field that is still under investigation, with multiple prototypes developed. This review aims to analyze the hemodynamic profile of the developed intravascular pumps and their application in the preclinical scenario in the Fontan circulation.

Ex vivo heart perfusion: an updated systematic review.

Due to the discrepancy between patients awaiting a heart transplant and the availability of donor hearts, strategies to expand the donor pool and improve the transplant's success are crucial. This review aims to summarize current knowledge on the ex vivo heart preservation (EVHP) experience as an alternative to standard cold static storage (CSS). EVHP techniques can improve the preservation of the donor's heart before transplantation and allow for pre-transplant organ evaluation.

Redefining CAV surveillance strategies: Benefits of CCTA vs. ICA.

BACKGROUND: Cardiac allograft vasculopathy (CAV) assessment post-heart transplantation (HT) typically relies on invasive coronary angiography (ICA). However, cardiac computed tomography angiography (CCTA) is emerging as a promising alternative due to its potential benefits in economic, safety, and logistical aspects. This study aimed to evaluate the impact of a CCTA program on these aspects in CAV surveillance post-HT. METHODS: A retrospective single-center study was conducted between March 2021 and February 2023, involving HT patients who underwent either CCTA or ICA. RESULTS: Among 260 patients undergoing CAV surveillance, 115 (44.2%) patients underwent CCTA, and 145 (55.8%) patients underwent ICA. The CCTA group showed incurred lower overall costs (p â€‹< â€‹0.0001) and shorter hospitalization times (p â€‹< â€‹0.0001) compared to the ICA group. In terms of safety, CCTA surveillance required significantly lower contrast volumes (p â€‹< â€‹0.0001) and lower effective doses (p â€‹= â€‹0.03). CONCLUSION: CCTA emerges as a safe and cost-effective non-invasive alternative for CAV surveillance post-HT, outperforming ICA in terms of safety, logistical aspects, and economic burden.

A Comprehensive Excursus of the Roles of Echocardiography in Heart Transplantation Follow-Up.

Current guidelines for the care of heart transplantation recipients recommend routine endomyocardial biopsy and invasive coronary angiography as the cornerstones in the surveillance for acute rejection (AR) and coronary allograft vasculopathy (CAV). Non-invasive tools, including coronary computed tomography angiography and cardiac magnetic resonance, have been introduced into guidelines without roles of their own as gold standards. These techniques also carry the risk of contrast-related kidney injury. There is a need to explore non-invasive approaches providing valuable information while minimizing risks and allowing their application independently of patient comorbidities. Echocardiographic examination can be performed at bedside, serially repeated, and does not carry the burden of contrast-related kidney injury and procedure-related risk. It provides comprehensive assessment of cardiac morphology and function. Advanced echocardiography techniques, including Doppler tissue imaging and strain imaging, may be sensitive tools for the detection of minor myocardial dysfunction, thus providing insight into early detection of AR and CAV. Stress echocardiography may offer a valuable tool in the detection of CAV, while the assessment of coronary flow reserve can unravel coronary microvascular impairment and add prognostic value to conventional stress echocardiography. The review highlights the role of Doppler echocardiography in heart transplantation follow-up, weighting advantages and limitations of the different techniques.

Overcoming the Boundaries of Heart Warm Ischemia in Donation After Circulatory Death: The Padua Case.

A 45 year old male obese patient with a previous history of repaired congenital heart disease developed worsening heart failure making heart transplantation listing mandatory. Unfortunately, due to his anthropometric measures, the search for a suitable brain-dead donor was unsuccessful. For this reason, he accepted to be enrolled in the controlled donation after circulatory death (cDCD) program. According to the Italian Law regulating death declaration after cardiac arrest (no-touch period of 20 minutes-one of the longest in the world), we faced a 34 minute cardiac asystole, after which the heart was recovered through a thoraco-abdominal normothermic regional perfusion excluding the epiaortic vessels. The heart was then preserved by means of cold static storage. Heart transplantation was performed successfully without any signs of primary graft failure. Postoperative endomyocardial biopsies were negative for acute cellular and antibody-mediated rejection. Furthermore, echocardiographic and cardiac magnetic resonance evaluation of the heart did not show any functional abnormalities. The patient was discharged on post-operative day (POD) #39 in good clinical conditions.

Surgical Ablation for Atrial Fibrillation During Mitral Valve Surgery: A Systematic Review and Meta-Analysis of Randomized Controlled Trials.

Although surgical ablation has been shown to produce excellent outcomes at follow-up for patients with atrial fibrillation who underwent mitral valve replacement/repair (MVR), this procedure is not commonly performed. Our objective was to conduct a systematic review and meta-analysis to evaluate the outcomes of concomitant surgical ablation during MVR. Three databases were systematically reviewed for randomized clinical trials published by August 2022. The primary outcome was sinus rhythm (SR) at 12 months. Secondary outcomes included SR at discharge and 6 months, all-cause mortality, permanent pacemaker implantation, and stroke and thromboembolic events. A random-effects meta-analysis was performed, calculating odds ratios (ORs) for each outcome. Thirteen studies were included, involving 1,089 patients comparing patients who underwent either isolated MVR ("MVR-only") or concomitant surgical ablation during MVR ("MVR+Ablation"). The odds of SR were significantly higher in the MVR+Ablation group at discharge (OR 9.62, 95% confidence interval [CI] 4.87 to 19.02, I2 = 55%), at 6-month follow-up (OR 7.21, 95% CI 4.30 to 12.11, I2 = 34%), and at 1-year follow-up (OR 8.41, 95% CI 5.14 to 13.77, I2 = 48%). All-cause mortality was not different in the groups, as were stroke and thromboembolic events, whereas the odds of permanent pacemaker implantation were slightly higher in the MVR+Ablation group (OR 1.87, 95% CI 1.11 to 3.17, I2 = 0%). Concomitant surgical ablation during MVR showed excellent outcomes at short-term follow-up, despite a slightly higher rate of permanent pacemaker implantation. Further studies with longer follow-ups are needed to assess if the SR is maintained over the years.

New Non-Invasive Imaging Technologies in Cardiac Transplant Follow-Up: Acquired Evidence and Future Options.

Heart transplantation (HT) is the established treatment for end-stage heart failure, significantly enhancing patients' survival and quality of life. To ensure optimal outcomes, the routine monitoring of HT recipients is paramount. While existing guidelines offer guidance on a blend of invasive and non-invasive imaging techniques, certain aspects such as the timing of echocardiographic assessments and the role of echocardiography or cardiac magnetic resonance (CMR) as alternatives to serial endomyocardial biopsies (EMBs) for rejection monitoring are not specifically outlined in the guidelines. Furthermore, invasive coronary angiography (ICA) is still recommended as the gold-standard procedure, usually performed one year after surgery and every two years thereafter. This review focuses on recent advancements in non-invasive and contrast-saving imaging techniques that have been investigated for HT patients. The aim of the manuscript is to identify imaging modalities that may potentially replace or reduce the need for invasive procedures such as ICA and EMB, considering their respective advantages and disadvantages. We emphasize the transformative potential of non-invasive techniques in elevating patient care. Advanced echocardiography techniques, including strain imaging and tissue Doppler imaging, offer enhanced insights into cardiac function, while CMR, through its multi-parametric mapping techniques, such as T1 and T2 mapping, allows for the non-invasive assessment of inflammation and tissue characterization. Cardiac computed tomography (CCT), particularly with its ability to evaluate coronary artery disease and assess graft vasculopathy, emerges as an integral tool in the follow-up of HT patients. Recent studies have highlighted the potential of nuclear myocardial perfusion imaging, including myocardial blood flow quantification, as a non-invasive method for diagnosing and prognosticating CAV. These advanced imaging approaches hold promise in mitigating the need for invasive procedures like ICA and EMB when evaluating the benefits and limitations of each modality.

Left ventricular assist device exchange: a review of indications, operative procedure, and outcomes.

The use of left ventricular assist devices (LVADs) is intended to treat patients with end-stage heart failure. Owing to technological advances, these devices are becoming more durable. However, LVADs may need to be exchanged when complications arise and heart transplantation is not possible. Indications for LVAD exchange (LVADE) include device thrombosis, device infections, and pump component failure. LVADE has historically been associated with a high risk of morbidity and mortality. In this review, we discuss the indications of LVADE, the decisional and technical aspects during surgery, and outcomes.

The AngioVac System in Childhood and Adolescence: A New Venovenous Extracorporeal Membrane Oxygenation-Like Configuration.

Intravascular and intracardiac clots, thrombi, and vegetative material can be safely and effectively treated with the AngioVac System (AngioDynamics, Latham, NY) as an alternative to open surgery. However, this technology is still not performed in children or adolescents as a rule. We aimed to present our experience with two cases (a 10 year old girl and a 17 year old male adolescent) with concurrent hypoxemia in whom this device was successfully used in combination with venovenous extracorporeal membrane oxygenation to remove caval thrombi and cavoatrial septic material, respectively. This extracorporeal circuit configuration allowed adequate respiratory support during the procedure. No endovascular recurrence of the pathologic material was found at 2 and 1 year of follow-up, respectively.

Nonischemic Donor Heart Preservation: New Milestone in Heart Transplantation History.

Heart transplantation is considered the gold standard for the treatment of advanced end-stage heart failure. However, standard donors after brain death are decreasing, whereas patients on the heart transplant waitlist are constantly rising. The introduction of the ex vivo machine perfusion device has been a turning point; in fact, these systems are able to significantly reduce ischemic times and have a potential effect on ischemia-related damage reduction. From a clinical standpoint, these machines show emerging results in terms of heart donor pool expansion, making marginal donors and donor grafts after circulatory death suitable for donation. This article aims to review mechanisms and preclinical and clinical outcomes of currently available ex vivo perfusion systems, and to explore the future fields of application of these technologies.

Sternal-Sparing Transapical, Beating Heart Mitral Valve Repair in an Adult With Repaired Tetralogy of Fallot.

We present the case of a 60-year-old male patient who underwent tetralogy of Fallot repair at 7 years of age and then developed severe degenerative mitral regurgitation during adulthood. Given the increased surgical risk (obesity, obstructive sleep apnea syndrome, and reoperation), the patient underwent a successful microinvasive mitral valve repair with neochordae implantation. (Level of Difficulty: Advanced.).

Outcomes of transvenous lead extraction in octogenarians using bidirectional rotational mechanical sheaths.

BACKGROUND: Outcomes of transvenous lead extraction (TLE) are well reported in the general population, However, data on safety, efficacy of TLE in octogenarians with a long lead dwell time, using powered extraction tools are limited. The aim of this multicenter study was to evaluate the safety, effectiveness of TLE in octogenarians using the bidirectional rotational mechanical sheaths and mid-term outcome after TLE. METHODS: The study population comprised 83 patients (78.3% male; mean age 85 ± 3 years; [range 80-94 years]) with 181 target leads. All the leads (mean implant duration 112 ± 77 months [range 12-377]) were extracted exclusively using the Evolution RL sheaths (Cook Medical, Bloomington, IN, USA). RESULTS: The main indication for TLE was infection in 84.3% of cases. Complete procedural success rate, clinical success rate, per lead were 93.9% and 98.3%, respectively. Failure of lead extraction was seen in 1.7% of leads. The additional use of a snare was required in 8.4% of patients. Major complications occurred in one patient (1.2%). Thirty-day mortality after TLE was 6%. During a mean time follow-up of 22 ± 21 months, 24 patients (29%) died. No procedure-related mortality occurred. Predictors of mortality included ischemic cardiomyopathy (HR 4.35; 95% CI 1.87-10.13; p = .001), left ventricularejection fraction ≤35% (HR 7.89; 95% CI 3.20-19.48; p < .001), and TLE for systemic infection (HR 4.24; 95% CI 1.69-10.66; p = .002). CONCLUSIONS: At experienced centers bidirectional rotational mechanical sheaths combined with different mechanical tools and femoral approach allowreasonable success and safety in octogenarian with long lead dwell time. Patient's age should not influence the decision to extract or not the leads, although the 30-day and mid-term mortality are significant, especially in the present of specific comorbidities.