orthotopic heart transplant
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2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Davide Diana ◽  
Giovanni Diana ◽  
Vincenzo Polizzi ◽  
Fabio Sbaraglia ◽  
Carla Giordano ◽  
...  

Abstract Male, 46 year old. Family history: brother affected by deafness and repeated episodes of stroke at a young age. Pathological history: history of competitive sporting activity in which he underwent periodic outpatient checks and reported sporadic myalgic episodes. The patient was suffering from bilateral keratoconus. For the purpose of discovering Wolf–Parkinson–White syndrome with the presence of a right antero-septal accessory pathway, he underwent an electrophysiological study (2003) negative for inducible arrhythmias. During a routine checkup, a renal biopsy was performed to search for elevated blood creatinine, which concluded with acute interstitial nephritis (2005), treated ineffectively with steroids and resulted in dialysis-dependent stage V renal failure (2020). Following light tiredness, he underwent an echocardiographic examination (2009) which revealed the presence of dilated heart disease with reduced left ventricular systolic function. He underwent a cardiac MRI which showed diffuse spots of subepicardial late enhancement as a possible post-myocarditis outcome. At subsequent clinical-echocardiographic checks, progressive biventricular dysfunction, and signs of congestive heart failure were highlighted, for which medical therapy was progressively increased and insertion of an implantable cardioverter defibrillator in primary prevention for sudden cardiac death. At the subsequent clinical re-evaluations, there was evidence of progressive bilateral hearing loss. In consideration of the clinical picture and family history, considering the syndromic nature of the polypathologies to be likely, genetic investigation was required for mitochondrial diseases. Mutations 3242 and 3271mt-RNA and 13513 mtND5, frequent in the MELAS Syndrome, were searched in peripheral venous samples and resulted as negative. In 2020 he underwent an orthotopic heart transplant sec. Shamway followed by a kidney transplant from a compatible donor. In order to perform further diagnostic investigations, the explanted heart was sent to the Pathological Anatomy laboratory of the Umberto I Hospital: macroscopic analysis showed foci of fat replacement at the level of the anterior and posterior wall of the right ventricle (Figure); under microscopy, marked myocardial hypertrophy was observed, associated with cytoplasmic vacuolization of the cardiomyocytes, fibro-adipose substitution of the right ventricle, and replacement fibrosis in minute foci in the left ventricular level. A widespread and marked reduction in the enzymatic activity of cytochrome oxidase in cardiomyocytes and mitochondrial proliferation was demonstrated using histo-enzymatic staining, by staining for succinate dehydrogenase, concluding with mitochondrial disease. Mitochondrial diseases represent a challenge not only from the prognostic–therapeutic point of view but, remarkably, also from a diagnostic one: the patient received a correct diagnosis of the pathology that afflicted him, with almost two decades of delay. The integrated and multidisciplinary approach is desirable in order to obtain an early diagnosis.


Author(s):  
Michael C. Hill ◽  
Mark N. Belkin ◽  
Phillip McMullen ◽  
Jessica J. Pillarella ◽  
Greg P. Macaluso ◽  
...  

2021 ◽  
Vol 5 (10) ◽  
Author(s):  
Monika Sadlonova ◽  
Birgit Gerecke ◽  
Christoph Herrmann-Lingen ◽  
Ingo Kutschka

Abstract Background Heart transplant recipients show a high risk of developing major depression with an increased risk of post-transplant morbidity and mortality. Heart transplant specialists and patients face unprecedented challenges during the COVID-19 pandemic, which have enormous clinical implications such as the increased risk of COVID-19 as well as visitor restrictions with social isolation during the post-transplant inpatient treatment. Case summary We present a case of a 64-year-old woman with end-stage heart failure caused by non-compaction cardiomyopathy who received an orthotopic heart transplant (OHT) without any intra-operative complications. Post-operatively, she showed acute psychotic symptoms in the intensive care unit (ICU) with improvement after switching intravenous tacrolimus treatment to an oral intake. Furthermore, the patient developed severe depressive symptoms with malnutrition and had a prolonged hospitalization. Standard medical care was complemented by intensive psychocardiological treatment to overcome the crisis. Conclusion High complexity of the post-transplant management after OHT underlines the importance of multidisciplinary teamwork, involving heart transplant specialists and allied mental health professionals. This collaboration led to an excellent long-term result. Facing the COVID-19 pandemic, the hospital visitor policies may be scrutinized, carefully looking at the role of social isolation, post-operative experience in the ICU, and medical complications after OHT.


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