Combined heart and liver transplantation in four adults with familial amyloidosis: experience of a single center.

There are few reports of combined heart and liver transplantation (CHLT) for familial amyloidotic polyneuropathy (FAP). The technique for the operation remains to be defined. Four CHLTs were performed for amyloidogenic transthyretin-related (variant Glu89Gln-ATTR Glu89Gln) cardiomyopathy in our center. Patients 1 and 4 had no serious involvement of other organs, whereas patients 2 and 3 had evident peripheral neuropathy and gastrointestinal motility alterations. Patient 3 also had high-grade orthostatic hypotension. All four patients underwent cardiac and sequential hepatic transplantation with organs procured from the same donor. Venovenous bypass was used in patients 1 and 4 who experienced uncomplicated procedures. The amyloidotic liver of patient 4 was successfully utilized for a domino procedure to treat a patient with hepatocellular carcinoma on cirrhosis. The cardiac performance of patients 1 and 4 remains normal; there has been no progression of amyloidosis at 42 and 1 months after transplantation. Patient 2 had no intraoperative complications but experienced postoperative bleeding, renal failure, sepsis, and heart failure, and finally died of multiorgan failure 2 months after transplant. In patient 3, right hemicolectomy was required intraoperatively due to intestinal ischemia, without significant hemodynamic instability, while extracardiac symptoms of amyloidosis gradually worsened postoperatively. In conclusion, CHLT for ATTR Glu89Gln may be performed even in patients with advanced disease. However, the most compromised patients are more likely to display intraoperative risks, postoperative complications, and worsening of extracardiac, extrahepatic symptoms.

Arrhythmogenic right ventricular cardiomyopathy: clinicopathologic correlation based on a revised definition of pathologic patterns.

Different morphologic features of arrhythmogenic right ventricular cardiomyopathy (ARVC) have been described. However, it is still unclear whether they correspond to distinct forms of the same disease. A pathologic study was performed on a series of ARVC (15 from heart transplant and 12 from autopsy) from 2 Italian referral university hospitals. Based on both myocellular features and the nature of myocardial replacement, hearts were divided into 2 groups: infiltrative, with a lacelike pattern of transmural fatty infiltration and strands of normal residual cardiomyocytes (n = 11); and cardiomyopathic, with massive myocardial replacement by fibro fatty tissue and cardiomyopathic changes (such as hypertrophy and myofibril loss) of residual cardiomyocytes (n = 16). Hearts from the infiltrative group were mostly obtained at autopsy of patients who died suddenly. Fatty substitution was limited almost exclusively to the right ventricle. Mitral valve dysplasia (prolapse or cleft) was frequently present. Hearts from the cardiomyopathic group came mainly from heart transplants for congestive heart failure. Fibro fatty replacement was more extensive, usually biventricular. Active myocarditis and features suggestive of myocardial transdifferentiation were also observed. Despite these differences in clinical outcome and morphologic features, patients from the 2 groups showed similar mean age, sex distribution, occurrence of threatening ventricular arrhythmias, and prevalence of family history of sudden death, arrhythmias, or cardiomyopathy. Infiltrative and cardiomyopathic patterns represent different clinical and pathologic subsets of ARVC. Myocellular features are an important clue in the distinction between the two entities. The differentiation between the 2 patterns is feasible on endomyocardial biopsy and could give important prognostic information.

Psychological evaluation after cardiac transplantation: the integration of different criteria.

BACKGROUND: The psychological evaluation of patients undergoing cardiac transplantation is currently based on DSM-IV criteria. An alternative diagnostic and conceptual framework has been proposed by an international group of psychosomatic investigators. The aim of this study was to compare these new criteria (Diagnostic Criteria for Psychosomatic Research, DCPR) with DSM-IV in a population where a high prevalence of psychological problems is expected (heart-transplanted patients). METHOD: 129 consecutive patients who underwent heart transplant surgery were assessed according to DSM-IV and DCPR criteria. RESULTS: The results showed a higher number of diagnoses made using the DCPR than with the use of the DSM-IV. At least one DCPR diagnosis was found in 85 (66%) patients, whereas at least one DSM diagnosis was present in 23 (18%) patients. The number of DCPR diagnoses was almost the triple of DSM criteria. While patients who were given a DSM diagnosis frequently had additional DCPR diagnoses, many patients with DCPR criteria did not fulfill any DSM criteria. Four DCPR syndromes appeared to be particularly frequent: demoralization, type A behavior, irritable mood and alexithymia. CONCLUSIONS: The joint use of DSM and DCPR criteria was found to improve the identification of psychological factors which could result in a worsening of quality of life in heart-transplanted patients.

Severe postcardiac-transplant rejection associated with recurrence of giant cell myocarditis.

Giant cell myocarditis is a disease of unknown etiology with several controversial aspects: clinical course, therapeutic management, recurring risk after heart transplantation, and histopathological factors. We report a case of giant cell myocarditis that recurred after orthotopic heart transplantation and an uneventful postoperative period. The myocardial inflammatory process in this patient showed various evolutive phases: an acute onset of diffuse giant cell myocarditis, an evolution into a granulomatous form of inflammation within the explanted heart, and a recurrence with multiple giant cell inflammatory infiltrates in the transplanted heart. Moreover, the patient presented a severe clinical course after surgery with precocious and continuous acute rejections despite the repeated immunosuppressive treatments. In this article we discuss the morphological aspects of the disease and the postoperative course of this case in relation to the possible immune dysregulation of patients affected by myocarditis before heart transplantation.

Tranexamic acid as a mouthwash in anticoagulant-treated patients undergoing oral surgery. An alternative method to discontinuing anticoagulant therapy.

A double-blind randomized study was carried out to evaluate the clinical hemostatic effect of tranexamic acid mouthwash after dental extraction in 30 patients who received anticoagulant agents. Surgery was performed with a reduction in the level of anticoagulant therapy in the control group and with no change in the level of anticoagulant therapy in the group who received the tranexamic acid. After the extraction the surgical field was irrigated with a 5% solution of tranexamic acid in the group of 15 patients whose anticoagulant treatment had not been discontinued and with a placebo solution in the group of 15 patients for whom the anticoagulant therapy was reduced. Patients were instructed to rinse their mouths with 10 ml of the assigned solution for 2 minutes four times a day for 7 days. There was no significant difference between the two treatment groups in the bleeding incidence after oral surgery. We conclude that the anticoagulant treatment does not need to be withdrawn before oral surgery provided that local antifibrinolytic therapy is instituted.

[Hemodynamic effects of disopyramide and procainamide in open-chest animals]. / Effetti emodinamici della diisopiramide e della procainamide nell'animale a torace aperto.

Hemodynamic effects of two antiarrhythmic agents, disopyramide and procainamide, have been evaluated in anesthetized open-chest healthy pigs after random administration. At therapeutic plasma concentrations none of these agents proved to have deleterious hemodynamic effects. The most important action was observed after disopyramide infusion and consisted in significant bradycardia which confirmed the known effect on sinus node automaticity of the drug. Left ventricular dp/dt, an index of cardiac contractility, was unchanged after infusion of both drugs. On the ECG intervals, only procainamide provoked a significant prolongation of QTc. It is concluded that at therapeutic dosage disopyramide does not present deleterious hemodynamic effects in animals and proves to be a valid alternative to other traditional antiarrhythmic agents.