Infected thoracic aortic graft in a woman with Darier disease: a case report.

Background: Patients with Darier disease often present with staphylococcal skin infections and are at risk for complications when they undergo cardiothoracic surgery, such as acute aortic dissection repair. Case summary: A 39-year-old woman with hypertension and Darier disease suffered an acute type A aortic dissection, requiring emergency operation with a Dacron graft. Twenty-five days post-operatively, she developed pneumonia and staph hominis was isolated in blood cultures and Bronchoalveolar Lavage. Following completion of antibiotics, multiple relapses occurred during a 6-month period, each time treated with appropriate antibiotic therapy. An 18F-fluorodeoxyglucose positron emission tomography computerized tomography showed persistent graft uptake and re-operation was performed. At 22 months of follow-up, the patient remains asymptomatic and the 18F-FDG PET/CT shows significant reduction in FDG uptake. Discussion: Graft infection is a rare but serious complication. Antibiotic therapy is often inadequate and re-operation is needed. As staphylococcal skin infections often occur in patients with Darier disease, adequate preprocedural skin preparation and sterilization are very important in these patients.

Balloon Pulmonary Angioplasty in Patients with Chronic Thromboembolic Pulmonary Hypertension in Greece: Data from the Hellenic Pulmonary Hypertension Registry.

Balloon pulmonary angioplasty (BPA) is a novel and promising treatment option for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy (PEA) and for those with persistent or recurrent pulmonary hypertension after PEA. We present the results of BPA procedures in CTEPH patients included in the Greek Pulmonary Hypertension Registry, evaluating the real-life efficacy and safety. We analyzed data from 180 BPA procedures (2−17/patient, mean 8 ± 4/patient, 1248 dilated vessels, 0−18/session). Significant improvements were observed in mean pulmonary arterial pressure (a reduction by 44%, p < 0.001), pulmonary vascular resistance (reduction by 60%, p < 0.001), and NT-proBNP (decrease by >70%, p: 0.003), while cardiac index improved modestly (9% increase, p = 0.143). We had 37 BPA-related non-fatal complications (20.6% in all interventions), predominantly including hemoptysis. Overall survival was 91%, 75% and 62% at 3, 4 and 5 years, respectively. Therefore, BPA may be a promising therapeutic option in patients with CTEPH in Greece.

Epidemiology and Management of Chronic Thromboembolic Pulmonary Hypertension in Greece. Real-World Data from the Hellenic Pulmonary Hypertension Registry (HOPE).

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare disease with poor prognosis if left untreated, characterized by pulmonary vascular bed obstruction due to unresolving thromboembolic material. The Hellenic pulmonary hypertension registry (HOPE) was launched in Greece in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. In total, 98 patients with CTEPH were enrolled from January 2015 until November 2019. Of these patients, 55.1% represented incident population, 50% were classified in the World Health Organization functional class II and 49% had a history of acute pulmonary embolism. The median values of pulmonary vascular resistance (PVR) and cardiac index were 7.4 (4.8) WU and 2.4 (1.0) L/min/m2, respectively, the mean diffusing capacity for carbon monoxide was 74.8 ± 20.6%, the median 6-minute walk distance was 347 (220) meters and the median value of N Terminal-pro brain natriuretic peptide was 506.0 (1450.0) pg/mL. In total, 60.2% of the patients were under pulmonary arterial hypertension-targeted therapy at the time of enrolment; specifically, riociguat was received by 35.7% of the patients and combination therapy was the preferred strategy for 16% of the patients. In total, 74 patients were evaluated for pulmonary endarterectomy (PEA), 34 (45.9%) were assessed as operable but only 23 of those (31.1%) finally underwent PEA. The remaining 40 patients were ineligible for PEA according to the operability assessment and 13 (17.6%) of them underwent balloon pulmonary angioplasty. The age of the non-operable patients was significantly higher than the operable patients (p < 0.001), while there was no significant difference with regard to the history of coagulopathies between the operable and non-operable patients (p = 0.33).

HEllenic Registry on Myocarditis SyndromES on behalf of Hellenic Heart Failure Association: The HERMES-HF Registry.

AIMS: Despite the existence of many studies, there are still limited data about the characteristics of myocarditis in Greece. This led to the creation of the Greek Myocarditis Registry aiming to document the different symptoms and treatment of myocarditis, assess possible prognostic factors, and find similarities and differences to what is already published in literature. This paper is a preliminary descriptive analysis of this Registry. METHODS AND RESULTS: We analysed data for the hospitalization period of all patients included in the Registry from December 2015 until November 2017. Statistics are reported as frequency (%) or median and inter-quartile range (IQR) as appropriate. In total, 146 patients were included; 83.3% of the patients reported an infection during the last 3 months. The most common symptom, regardless of the underlying infection, was chest pain (82.2%) followed by dyspnoea (18.5%), while the most common finding in clinical examination was tachycardia (26.7%). Presentation was more frequent in the winter months. ECG findings were not specific, with the repolarization abnormalities being the most frequent (60.3%). Atrial fibrillation was observed in two patients, both of whom presented with a reduced ventricular systolic function. Left ventricular ejection fraction changed significantly during the hospitalization [55% (IQR: 50-60%) on admission vs. 60% (IQR: 55-60%) on discharge, P = 0.0026]. Cardiac magnetic resonance was performed in 88 patients (61%), revealing mainly subepicardial and midcardial involvement of the lateral wall. Late gadolinium enhancement was present in all patients, while oedema was found in 39 of them. Only 11 patients underwent endomyocardial biopsy. Discharge medication consisted mainly of beta-blockers (71.9%) and angiotensin-converting enzyme inhibitors (41.8%), while 39.7% of the patients were prescribed both. CONCLUSIONS: This preliminary analysis describes the typical presentation of myocarditis patients in Greece. It is a first step in developing a better prognostic model for the course of the disease, which will be completed after the incorporation of the patients' follow-up data.

Advancements in the diagnostic workup, prognostic evaluation, and treatment of takotsubo syndrome.

Takotsubo syndrome (TTS) is an acute and mostly reversible cardiomyopathy that mimics an acute coronary syndrome with left ventricular (LV) systolic dysfunction without relevant obstructive coronary artery disease. Its prevalence is probably underestimated and reaches 1.2-2% in patients with acute coronary syndrome undergoing coronary catheterization. Although supraphysiological epinephrine levels have been associated with TTS, the detailed pathophysiology is incompletely understood. Chest pain is the most common clinical presentation; however, cardiac decompensation, cardiogenic shock, and sudden cardiac death due to ventricular fibrillation may also be the first clinical manifestations. Patients are mostly postmenopausal women, in whom the condition is commonly associated with emotional triggers; however, men have a higher prevalence of TTS being associated with physical triggers, which has a worse prognosis compared with TTS associated with emotional triggers. As a diagnosis of exclusion, TTS has no single definitive diagnostic test. According to the distribution of LV wall motion abnormalities, various morphological subtypes have been identified. The final diagnosis depends on cardiac imaging with left ventricular angiography during acute heart catheterization, as well as on echocardiography and cardiac magnetic resonance. Most patients recover completely, albeit several factors have been associated with worse prognosis. Management is based on observational data, while randomized multicenter studies are still lacking. This review provides a general overview of TTS and focuses on the hypothesized pathophysiology, and especially on current practices in diagnosis, prognosis, and treatment.

Pulmonary arterial hypertension associated with interferon-beta treatment for multiple sclerosis. Case report and literature review.

Drug-Induced Pulmonary Arterial Hypertension (PAH) represents a well-known entity, predominantly related to anorexigens. Interferon-ß (IFN) is considered to be a drug with a possible risk of inducing PAH. We report a patient with Multiple Sclerosis treated with IFN-ß who diagnosed with PAH and her course of disease under specific PAH drug therapy. A review of the literature in IFN-ß-induced PAH is provided.

Cardiovascular imaging approach in pre and postoperative tetralogy of Fallot.

Advances in the medical and surgical management of Tetralogy of Fallot have led to marked increase of the number and age of survivors. Imaging in patients with Tetralogy of Fallot plays a crucial role in the diagnosis and follow up, and essentially guides management and intervention in this entity. This study systematically reviews the imaging modalities used in patients with Tetralogy of Fallot in the evaluation of preoperative and postoperative anatomic and hemodynamic lesions, as well as disease progression in this diagnosis. Various invasive and noninvasive imaging modalities, most commonly echocardiography and cardiovascular magnetic resonance, computed tomography and angiocardiography provide the imaging information required for diagnosis, management and follow up in Tetralogy of Fallot. The choice of the appropriate imaging tool or their combination is guided by the clinical question, the patient's clinical condition and contraindications as well as the strengths and weaknesses of each imaging modality. Tetralogy of Fallot is the most common complex congenital heart disease with long term survivors that need close follow up and complicated management, including multiple surgical and transcatheter interventions. Knowledge of the role and protocols of imaging in Tetralogy of Fallot is extremely important for the clinical as well as the imaging physician in order to optimize patients' management and long-term prognosis.

Improving Survival in Patients with Pulmonary Arterial Hypertension: Focus on Intravenous Epoprostenol.

Pulmonary arterial hypertension represents a devastating disease, causing progressive increase of pulmonary vascular resistance leading to right ventricular dysfunction and death. Therapeutic management has rapidly advanced in recent years due to improved understanding of pathophysiology and new drugs have been developed; however, survival remains poor. Oral agents as phosphodiesterase type V inhibitors, the soluble guanylyl cyclase stimulator riociguat, the prostacyclin receptor agonist selexipag and the endothelin receptor antagonists have each achieved evidence-based validation and are recommended for pulmonary arterial hypertension. Initial oral monotherapy or combination therapy is recommended for patients with low or intermediate risk according to each patient's risk stratification. Intravenous epoprostenol is a synthetic prostacyclin and the first drug approved for the disease. Although it represents the only treatment shown to reduce mortality, it is underused. Survival rates for patients treated with oral combination drug therapies are lower than those for patients treated with initial combination therapies including intravenous epoprostenol. This raises the interesting question of whether intermediate risk pulmonary arterial hypertension patients should be routinely introduced to therapies including intravenous epoprostenol rather than combination oral therapies.

Transcatheter septal ablation in hypertrophic obstructive cardiomyopathy: a technical guide and review of published results.

Transcatheter alcohol septal ablation (ASA) treatment of symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM) is based on the existence and degree of intraventricular obstruction. Patients with significant gradient and symptoms who do not respond to optimal medical therapy are eligible to gradient reduction through a surgical (septal myectomy) or a transcatheter (alcohol septal ablation) septal reduction. The latter encompasses occlusion of a septal branch perfusing the hypertrophied septum, which is involved in the generation of obstruction, by injecting ethanol into the supplying septal branch(es). ASA has been established as a highly effective and safe method and has outnumbered the surgical gold standard. Although the technique is straightforward, patient selection and some technical details may influence the efficacy and safety of the procedure. The technique is based on echocardiographic contrast guidance, which allows accurate target septal branch selection and optimisation of the result. Published long-term results from high-volume centres have confirmed the effectiveness of ASA and have shown excellent survival, which is comparable to that in the general population. Choice and performance of the surgical or interventional treatment should be implemented in highly specialised centres in terms of a heart-team approach, taking notice of anatomic characteristics as well as comorbidities. Involvement of all cases in international registries may reveal the individual merits and indications for the surgical and interventional treatment in HOCM.

Takotsubo syndrome - adding pieces to a complex puzzle.

Takotsubo syndrome, a form of acutely decompensated heart failure, has drawn interest because of its intriguing pathophysiology and therapeutic dilemmas. In their recent work in BMC Cardiovascular Disorders, Abanador-Kamper et al. describe the therapy management in these patients and add valuable information on cardiovascular magnetic resonance imaging evolution.

T1 and T2 Mapping in Cardiology: "Mapping the Obscure Object of Desire".

The increasing use of cardiovascular magnetic resonance (CMR) is based on its capability to perform biventricular function assessment and tissue characterization without radiation and with high reproducibility. The use of late gadolinium enhancement (LGE) gave the potential of non-invasive biopsy for fibrosis quantification. However, LGE is unable to detect diffuse myocardial disease. Native T1 mapping and extracellular volume fraction (ECV) provide knowledge about pathologies affecting both the myocardium and interstitium that is otherwise difficult to identify. Changes of myocardial native T1 reflect cardiac diseases (acute coronary syndromes, infarction, myocarditis, and diffuse fibrosis, all with high T1) and systemic diseases such as cardiac amyloid (high T1), Anderson-Fabry disease (low T1), and siderosis (low T1). The ECV, an index generated by native and post-contrast T1 mapping, measures the cellular and extracellular interstitial matrix (ECM) compartments. This myocyte-ECM dichotomy has important implications for identifying specific therapeutic targets of great value for heart failure treatment. On the other hand, T2 mapping is superior compared with myocardial T1 and ECM for assessing the activity of myocarditis in recent-onset heart failure. Although these indices can significantly affect the clinical decision making, multicentre studies and a community-wide approach (including MRI vendors, funding, software, contrast agent manufacturers, and clinicians) are still missing.

Comorbidity "depression" in heart failure - Potential target of patient education and self-management.

The progress of the pharmacological and device treatment of heart failure (HF) has led to a substantial improvement of mortality and rehospitalization. Further potential for improvement may be heralded in the post-discharge management of HF patients, including patient education for self-management of HF. The study by Musekamp et al. is among the first publications providing evidence that improvements in self-management skills may improve outcomes of HF patients. It is concluded that multimodal approaches addressing comorbidities in HF patients with novel concepts, and by optimal and specific HF management, including patient education, may ultimately contribute to substantial improvement of HF prognosis.

Cardiac Dual-source Computed Tomography for the Detection of Left Main Compression Syndrome in Patients with Pulmonary Hyper-tension.

INTRODUCTION: Left Main Compression Syndrome (LMCS) represents an entity described as the extrinsic compression of the left main coronary artery (LMCA) by a dilated pulmonary artery (PA) trunk. We examined the presence of LMCS in patients with pulmonary hypertension (PH) using dual-source computed tomography (DSCT), as a non-invasive diagnostic tool. METHODS: The following parameters were measured: PA trunk diameter (PAD), the distance between PAD and LMCA (LMPA) and the distance between PA and aorta (AoPA). These measurements were related with demographic, echocardiographic, hemodynamic and clinical parameters. Angiography was performed in two patients with LMCS suspected by cardiac computed tomographic angiography. Patients without PH but with angina were examined as controls, using DSCT cardiac angiography to assess the same measurements and to detect the prevalence of coronary artery disease. RESULTS: PA diameter value over 40.00 mm has been associated with PH and LMCS. Furthermore, LMCS did not occur at a distance smaller than 0.50 mm between the PA and the LMCA, and did not correlate with the distance between the PA and the aorta or with cardiac index and NT-proBNP. CONCLUSION: DSCT may represent the initial testing modality in PH patients with dilated PA trunk to exclude LMCS. A periodical rule-out of this rare entity, as assessed by DSCT, in patients with a severely dilated PA seems to be mandatory for PH patients contributing to survival improvement.

Inspiratory work capacity is more severely depressed than inspiratory muscle strength in patients with heart failure: Novel applications for inspiratory muscle training.

BACKGROUND: We hypothesized that the ability to sustain maximal inspiratory pressure (SPImax) over time as a measure of work capacity may be more severely affected than inspiratory muscle strength (PImax) in patients with heart failure (HF). METHODS: We retrospectively investigated eighty patients with HF, NYHA II/III/ ambulatoryIV and a (mean±SD) LVEF 27±8%, and compared them to 25 healthy subjects (HS). During a maximal inspiratory manoeuvre from residual volume (RV) to total lung capacity, PImax was measured as the maximum mouth pressure at RV, inspiratory contraction time (ICT) as the time from RV to end of inspiration and SPImax as the area under a pressure-time curve using an electronic pressure manometer with designed-purpose software (Trainair(®), Project Electronics Ltd., London, UK). Exercise capacity was assessed with cardiopulmonary exercise testing and the 6-minute walk test (6MWT). RESULTS: Patients achieved a (mean±SD) peak VO2=15.7±3.4ml/kg/min and 6MWT=338±88m. PImax, ICT and SPImax were reduced in HF pts at 75%***, 61%*** and 52%*** of HS, and correlated with NYHA (r=-0.485***), (r=-0.507**), (r=-0.500***), peakVO2 (r=0.501***), (r=0.655***), (r=0.508***) and 6MWT (r=0.477***), (r=0.345**), (r=0.530***), respectively (*p<0.05, **p<0.01, ***p<0.001). CONCLUSION: PImax, ICT and SPImax were impaired in HF patients compared to HS and may be important determinants of exercise capacity. SPImax was severely depressed even in patients with relatively preserved PImax and should be considered as an additional target index for inspiratory muscle training. Equations are provided to predict SPImax in relation to age and exercise capacity in HF.

Double organ transplantation in cardiac amyloidosis.

BACKGROUND: Cardiac amyloidosis, particularly primary or AL amyloidosis, is the most common infiltrative cardiomyopathy and is associated with a poor prognosis. The outcome of cardiac transplantation is generally poor, and almost half of patients die while waiting for the procedure to be done. PATIENT: We report here the remarkable case of a 63-year-old man with heart failure caused by AL amyloidosis. After a long course, which included rapid deterioration of preexisting heart failure, cardiac arrest, cardiogenic shock, biventricular assist device support, heart transplantation, renal failure, kidney transplantation and finally a life-threatening H1N1 virus pneumonia, the patient managed not only to survive but also to return fully to his previous demanding duties and lifestyle. DISCUSSION: Early use of left ventricular or biventricular mechanical circulatory support may be beneficial as a bridge to transplantation in patients with cardiac AL amyloidosis.

Benefits of combined aerobic/resistance/inspiratory training in patients with chronic heart failure. A complete exercise model? A prospective randomised study.

BACKGROUND: We hypothesised that combined aerobic training (AT) with resistance training (RT) and inspiratory muscle training (IMT) could result in additional benefits over AT alone in patients with chronic heart failure (CHF). METHODS: Twenty-seven patients, age 58 ± 9 years, NYHA II/III and LVEF 29 ± 7% were randomly assigned to a 12-week AT (n=14) or a combined AT/RT/IMT (ARIS) (n=13) exercise program. AT consisted of bike exercise at 70-80% of max heart rate. ARIS training consisted of AT with RT of the quadriceps at 50% of 1 repetition maximum (1RM) and upper limb exercises using dumbbells of 1-2 kg as well as IMT at 60% of sustained maximal inspiratory pressure (SPI(max)). At baseline and after intervention patients underwent cardiopulmonary exercise testing, echocardiography, evaluation of dyspnea, muscle function and quality of life (QoL) scores. RESULTS: The ARIS program as compared to AT alone, resulted in additional improvement in quadriceps muscle strength (1RM, p=0.005) and endurance (50%1 RM × number of max repetitions, p=0.01), SPI(max) (p<0.001), exercise time (p=0.01), circulatory power (peak oxygen consumption × peak systolic blood pressure, p=0.05), dyspnea (p=0.03) and QoL (p=0.03). CONCLUSIONS: ARIS training was safe and resulted in incremental benefits in both peripheral and respiratory muscle weakness, cardiopulmonary function and QoL compared to that of AT. The present findings may add a new prospective to cardiac rehabilitation programs of heart failure patients whilst the clinical significance of these outcomes need to be addressed in larger randomised studies.