Heart transplantation in the era of COVID-19 pandemic: delirium, post-transplant depression, and visitor restrictions; the role of liaison and inpatient psychosomatic treatment-a case report.

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.

Noncompaction Cardiomyopathy-History and Current Knowledge for Clinical Practice.

Noncompaction cardiomyopathy (NCCM) has gained increasing attention over the past twenty years, but in daily clinical practice NCCM is still rarely considered. So far, there are no generally accepted diagnostic criteria and some groups even refuse to acknowledge it as a distinct cardiomyopathy, and grade it as a variant of dilated cardiomyopathy or a morphological trait of different conditions. A wide range of morphological variants have been observed even in healthy persons, suggesting that pathologic remodeling and physiologic adaptation have to be differentiated in cases where this spongy myocardial pattern is encountered. Recent studies have uncovered numerous new pathogenetic and pathophysiologic aspects of this elusive cardiomyopathy, but a current summary and evaluation of clinical patient management are still lacking, especially to avoid mis- and overdiagnosis. Addressing this issue, this article provides an up to date overview of the current knowledge in classification, pathogenesis, pathophysiology, epidemiology, clinical manifestations and diagnostic evaluation, including genetic testing, treatment and prognosis of NCCM.

Therapy of ventricular arrhythmias in patients suffering from isolated left ventricular non-compaction cardiomyopathy.

AIMS: Non-compaction cardiomyopathy (NCCM) is associated with high rates of mortality and morbidity. Knowledge regarding risk stratification, arrhythmogenesis, therapy, and prognosis is limited. The aim of this study was to analyse the outcome of patients suffering from NCCM and ventricular arrhythmias (VAs) focusing on a treatment with implantable cardioverter-defibrillator (ICD) therapy and catheter ablation. METHODS AND RESULTS: We conducted a multicentre observational study on 18 patients with NCCM, who underwent ICD implantation for secondary (n = 12) and primary (n = 6) prevention. In patients with multiple symptomatic episodes of VAs catheter ablation was performed. During a follow-up of 62 ± 42 months, 12 patients (67%) presented with appropriate ICD therapies [ventricular tachycardia (VT): n = 8; ventricular fibrillation (VF): n = 4; VT/VF: n = 3]. Ten patients underwent catheter ablation for VT/VF. Solely endocardial ablation was conducted in eight patients, and in two patients endo- and epicardial ablation was performed within the same procedure. Acute procedural success was achieved in 9/10 patients. Ventricular tachycardia recurrence was observed in two patients and the median arrhythmia free interval was 9.5 months (interquartile range 5.3-21 months). One patient underwent reablation, four patients died due to the underlying NCCM, and one patient received a left ventricular assist device. CONCLUSION: Ventricular arrhythmias are common in patients suffering from NCCM and ICD therapy may be effective for primary and secondary prevention. In our cohort, consisting of patients with multiple VA episodes and recurrent ICD therapy, catheter ablation offered a safe and effective therapeutically option.

Interobserver Agreement of the Echocardiographic Diagnosis of LV Hypertrabeculation/Noncompaction.

OBJECTIVES: The aim of the study was to assess interobserver agreement (IOA) between 3 observers from 2 laboratories. BACKGROUND: IOA of left ventricular hypertrabeculation/noncompaction (LVHT) in adults has only been studied within single echocardiographic laboratories. METHODS: Echocardiographic recordings with and without LVHT were selected and anonymized. The "not-LVHT" cases were matched for age and systolic function. Each observer reviewed the recordings, blinded to the initial diagnosis and the other observers' results. Pre-defined criteria for LVHT were: 1) >3 prominent trabeculae at end-diastole, distinct from papillary muscles, false tendons, or aberrant bands; 2) a noncompacted part of a 2-layered myocardial structure formed by these trabeculations; 3) a ratio of >2:1 of noncompacted to compacted layer at end-systole; and 4) perfusion of the intertrabecular spaces from the ventricular cavity. IOA was estimated using the kappa measure of concordance. RESULTS: Cine-loops of 100 patients (42 women, ages 16 to 92 years), 50 from each center, and 51 with LVHT as the initial diagnosis, were reviewed. The left ventricular end-diastolic diameter was 32 to 78 mm, and ejection fraction, 4% to 88%. The observers agreed about presence (n = 29) or absence (n = 36) of LVHT and disagreed in 35 cases. Agreement was higher among the 2 observers from the same laboratory (kappa 0.793 [95% confidence interval (CI): 0.672 to 0.915]) than from different laboratories (kappa 0.628 [95% CI: 0.472 to 0.784], kappa 0.669 [95% CI: 0.521 to 0.818]). The observers agreed with the initial report of LVHT-presence in 53% and of absence in 67%. By reviewing the discordant cases, consensus was achieved about LVHT presence (n = 8) or absence (n = 16); in 11 cases, the diagnosis remained questionable. Discordance was due to poor image quality, lack of views in different apical planes, aberrant bands and chordae tendineae, abnormally sized or inserting papillary muscles, and localized calcifications of the endocardium. CONCLUSIONS: IOA was substantial for diagnosing LVHT. However, even the application of pre-defined criteria yielded disagreement in 35% of cases; and after mutual review, there were still 11% questionable cases.

Pathomorphologic findings in left ventricular hypertrabeculation/noncompaction of adults in relation to neuromuscular disorders.

BACKGROUND: Aim of this study was to assess pathomorphologic findings (PATHO) in patients with echocardiographically (ECHO) diagnosed left ventricular hypertrabeculation/noncompaction. METHODS: ECHO-criteria for LVHT were: >3 trabeculations, moving synchronously with the compacted myocardium, and forming the noncompacted part of a two-layered myocardium. At autopsy, the hearts were investigated according to the pathologists' preferences. RESULTS: Twelve patients (2 females, age 27-81 years) were included. Seven suffered from neuromuscular disorders, 5 patients were not investigated neurologically. The specimens were acquired after explantation during heart transplantation (n=1), death due to heart failure (n=6), sudden death (n=2), pneumonia (n=2) and stroke (n=1). Eight hearts were investigated without fixation and 4 after formaldehyde fixation. The hearts were opened along the long-axis, in 3 hearts additional short-axis cuts were carried out. At PATHO the trabecular meshwork was better visible in the formaldehyde-fixed hearts than in the fresh hearts. Differentiation from papillary muscles was easier on the long-axis cuts, whereas the two-layered structure was better visible on short-axis cuts. The trabecular pattern was similar in patients with neuromuscular disorders and those who did not undergo neurologic investigation. Subendocardial fibrosis was found in each case. Due to the complex three-dimensional geometry, it was impossible to count the number of trabeculations. CONCLUSION: Formaldehyde-fixation should be performed when comparing ECHO with PATHO findings in LVHT. Long-axis as well as short-axis cuts should be carried out in order to assess the course of trabeculations and the extent of the two-layered structure. Subendocardial fibrosis in LVHT deserves further research.

Gender differences in the manifestation of tako-tsubo cardiomyopathy.

OBJECTIVE: This study evaluated if there are gender differences in the manifestation of tako-tsubo cardiomyopathy (TTC). BACKGROUND: TTC predominantly occurs in elderly females and mimics acute myocardial infarction (AMI) where men and women are known to have a different clinical profile. METHODS: 324 patients from 37 hospitals were prospectively included in a TTC registry. Clinical, electrocardiographic, angiographic and outcome data from male and female patients were compared. RESULTS: Of 324 patients 296 (91%) were female and 28 (9%) male. Mean age (68 ± 12 vs 66 ± 12 years) and prehospital delay were similar. A triggering event preceded TTC onset in 76% of women and 86% of men. Physical stress was more frequent in men (30% vs 57%, p=0.005) whereas more women experienced emotional or no stress. The prevalence of angina and dyspnea did not differ. Fewer females were admitted in cardiogenic shock and/or after out of hospital cardiac arrest (1% vs 14%, p=0.0006), and cardiac troponin was lower (median 7.2 vs 10.7 times the upper limit of normal, p=0.03). The QTc interval was longer in females than in males only on the day of admission (468 ± 52 vs 441 ± 51 ms, p=0.047). Overall, complications during the acute course (53% vs 40%) were comparable in both sexes. CONCLUSIONS: In this large TTC registry, males and females showed a similar clinical profile. In males, physical stress as a trigger event and shock or cardiac arrest as presenting symptoms were more frequent. The QTc interval was longer in females only on admission but similar in males and females during the following days.

Refinement of echocardiographic criteria for left ventricular noncompaction.

BACKGROUND: Left ventricular hypertrabeculation/noncompaction (LVNC) is a cardiac abnormality whose echocardiographic criteria are still controversial. Cooperation between echocardiographic laboratories may contribute to uniformly accepted criteria, as illustrated by the following pilot study. METHODS AND RESULTS: Echocardiograms proposed for inclusion into a registry were reviewed. Three experts with 17-26 years experience with LVNC agreed on a common definition of LVNC: 1. >3 prominent trabeculous formations along the left ventricular endocardial border visible in end-diastole, distinct from papillary muscles, false tendons or aberrant bands; 2. trabeculations move synchronously with the compacted myocardium, 3. trabeculations form the noncompacted part of a two-layered myocardial structure, best visible at end-systole; and 4. perfusion of the intertrabecular spaces from the ventricular cavity is present at end-diastole on color-Doppler echocardiography or contrast echocardiography. During 3 sessions 115 cases (37% females, mean 57 years) were reviewed. Eleven patients (18% females, mean 60 years) were excluded because of <4 trabeculations (n=5), lack of a two-layered myocardial structure (n=1) and poor image quality (n=5). The observers agreed on inclusion or exclusion in all cases. Consensus was achieved that measurements of the thickness of the myocardial layers, and calculation of the noncompacted:compacted ratio is not feasible due to a lack of uniformly accepted standards for measurements. CONCLUSIONS: When diagnosing LVNC, end-systolic as well as end-diastolic images have to be considered. The presence of more than three trabeculations as well as a two-layered myocardium are required. Since these criteria are not anatomically controlled, a comparison of echocardiographic images with pathoanatomic findings for assessing sensitivity and specificity is urgently needed.

Isolated non-compaction cardiomyopathy.

BACKGROUND: Isolated non-compaction cardiomyopathy (NCCM) was first described in 1984. This disorder, a primary genetic cardiomyopathy, is now attracting increased attention. METHOD: The current state of the epidemiology, pathogenesis, pathophysiology, clinical features, diagnosis, treatment, and prognosis of NCCM are discussed on the basis of a review of selected literature as well as the authors' personal experience. RESULTS: The pathogenesis of NCCM is thought to involve a genetically determined disturbance of the myocardial compaction process during fetal endomyocardial morphogenesis. It is not accompanied by any other cardiac anomalies. Echocardiography is the diagnostic method of choice. The diagnosis is based on the following echocardiographic criteria: the presence of at least 4 prominent trabeculations and deep intertrabecular recesses, blood flow from the ventricular cavity into the intertrabecular recesses, and a typical bilaminar structure of the affected portion of the left ventricular myocardium. NCCM can also be diagnosed with magnetic resonance imaging of the heart. The clinical severity of NCCM is variable; its manifestations include heart failure, thromboembolic events, and arrhythmias. The treatment is symptom-based. Patients with symptomatic NCCM have a poor prognosis. CONCLUSION: NCCM is a type of cardiomyopathy that was first described 25 years ago. Its molecular genetic basis is not yet fully clear, and the same is true of its diagnosis, treatment, and prognosis. Further study of these matters is needed.