Boris Bigalke

The MOGE(S) classification for cardiomyopathies: current status and future outlook

Cardiomyopathies are complex diseases of multifactorial pathogenesis and have a high morbidity and mortality. Over the past decades, several revisions of classifications and definitions of cardiomyopathies have been proposed, primarily focusing on the phenotypic characterization of cardiomyopathies. The MOGE(S) classification system published in 2013 encompasses the classification of rapidly growing knowledge on genetic mutations, acquired causes (i.e., intramyocardial inflammation, viral infections), and further conditions involved in the induction of cardiomyopathies (e.g., storage diseases, toxicity). It is based on five attributes, including morphofunctional characteristics (M), organ involvement (O), genetic or familial inheritance pattern (G), etiological annotation (E), and optional information about the heart failure functional status (S). This review summarizes the development, the cornerstones of the MOGE(S) classification, and the published data on the clinical relevance of the MOGE(S) classification. We furthermore discuss new issues which might be considered for future updates of the MOGE(S) classification of cardiomyopathies.

Novel Approach for In Vivo Detection of Vulnerable Coronary Plaques using Molecular 3-T CMR Imaging with an Albumin-Binding Probe

OBJECTIVESnThis study sought to investigate the potential of the noninvasive albumin-binding probe gadofosveset-enhanced cardiac magnetic resonance (GE-CMR) for detection of coronary plaques that can cause acute coronary syndromes (ACS).nnnBACKGROUNDnACS are frequently caused by rupture or erosion of coronary plaques that initially do not cause hemodynamically significant stenosis and are therefore not detected by invasive x-ray coronary angiography (XCA).nnnMETHODSnA total of 25 patients with ACS or symptoms of stable coronary artery disease underwent GE-CMR, clinically indicated XCA, and optical coherence tomography (OCT) within 24 h. GE-CMR was performed approximately 24 h following a 1-time application of gadofosveset-trisodium. Contrast-to-noise ratio (CNR) was quantified within coronary segments in comparison with blood signal.nnnRESULTSnA total of 207 coronary segments were analyzed on GE-CMR. Segments containing a culprit lesion in ACS patients (nxa0= 11) showed significant higher signal enhancement (CNR) following gadofosveset-trisodium application than segments without culprit lesions (nxa0= 196; 6.1 [3.9 to 16.5] vs. 2.1 [0.5 to 3.5]; pxa0< 0.001). GE-CMR was able to correctly identify culprit coronary lesions in 9 of 11 segments (sensitivity 82%) and correctly excluded culprit coronary lesions in 162 of 195 segments (specificity 83%). Additionally, segmented areas of thin-cap fibroatheroma (nxa0= 22) as seen on OCT demonstrated significantly higher CNR than segments without coronary plaque or segments containing early atherosclerotic lesions (nxa0= 185; 9.2 [3.3 to 13.7] vs. 2.1 [0.5 to 3.4]; pxa0= 0.001).nnnCONCLUSIONSnIn this study, we demonstrated for the first time the noninvasive detection of culprit coronary lesions and thin-cap fibroatheroma of the coronary arteries inxa0vivo by using GE-CMR. This method may represent a novel approach for noninvasive cardiovascular risk prediction.

Restrictive cardiomyopathy: Delayed occurrence after radiotherapy of breast cancer

SummaryAxa074-year-old female patient was referred to our department in 2015 with dyspnea, cough and dysphagia. She had been diagnosed with adenocarcinoma of the right breast in 1986 and underwent mastectomy. When she presented with a local recurrence in 1988, she was receiving high-voltage radiation therapy. Transthoracic echocardiography and magnetic resonance imaging revealed tricuspid regurgitation grade III and unclear right heart failure with a massively dilated right atrium. Coronary heart disease could be ruled out. In summary, the patient’s findings represented right ventricular myocardial restriction which we attributed to irradiation of the right anterior chest 17xa0years previously.

Transplantation in patients with iron overload: is there a place for magnetic resonance imaging?

In iron overload diseases (thalassemia, sickle cell, and myelodysplastic syndrome), iron is deposited in all internal organs, leading to functional abnormalities. Hematopoietic stem cell transplantation (HSCT) is the only treatment offering a potential cure in these diseases. Our aim was to describe the experience in the field and the role of magnetic resonance imaging in the evaluation of iron overload before and after HSCT. Magnetic resonance imaging (MRI), using T2*, is the most commonly used tool to diagnose myocardial-liver iron overload and guide tailored treatment. Currently, HSCT offers complete cure in thalassemia major, after overcoming the immunologic barrier, and should be considered for all patients who have a suitable donor. The overall thalassemia-free survival of low-risk, HLA-matched sibling stem cell transplantation patients is 85–90%, with a 95% overall survival. The problems of rejection and engraftment are improving with the use of adequate immunosuppression. However, a detailed iron assessment of both heart and liver is necessary for pre- and post-transplant evaluation. In iron overload diseases, heart and liver iron evaluation is indispensable not only for the patients’ survival, but also for evaluation before and after HSCT.

Diuretic dosing in heart failure: more data are needed: Letter to the Editor

In the study by Okabe et al. the oral dose of 40 mg of furosemide proved being a cut-off beyond which both all-cause and cardiovascular mortality were significantly higher. The abovementioned value has been obtained using C-statistics from a total of 215 chronic heart failure (CHF) patients investigated through a median follow-up of 641 days. This interesting inference has been derived from a relatively small sample of CHF patients and may be therefore deemed as hypothesis generating. However, the study is confined to finding an association without affirming any causal value of it. In other words, in this observational study, it is not excluded that adverse prognosis profiles of higher doses (>40mg/d)might depend on a greater severity of the baseline clinical picture (so-called confounding by indication). Indeed, furosemide at doses of>40 mg/d is effective in reducing congestion, relieving cardiac workload, and decreasing ventricular wall stress, thereby preventing the progression of cardiac chambers’ dilatation. However, these favorable effects might fail in improving survival for the simultaneous occurrence of unfavorable repercussions on other organs and apparatuses. For example, a greater electrolyte loss (consisting of increased urinary excretion of Na, K, Ca, and Mg) related to doses of >40 mg furosemide/d might worsen ruinous vertebral osteoporosis, a disease relatively common in the elderly patients with cardiac decompensation, which results in fragility fractures or subluxations at the level of the spine with related neurological lesions (e.g. aching pain, paraplegia, and tetraplegia) with significant adverse impact on the patient’s life expectancy. In addition, relatively high oral dosesmay excessively stimulate themacula densa receptors in the kidneys with tubule-glomerular feedback, resulting in diuretic resistance. Subsequent adoption of sequential blockade of the nephron by means of thiazide addition might favor the occurrence of hyponatremia, resulting in neurological disturbances, such as postural instability and falls with the potential for fatal outcomes such as traumatic lesions (especially endocranial hematomas). Vasopressin antagonists prevent hyponatremia without increasing adverse events. Interferences between the dosage of diuretics and further factors of conditions such as the combination of diuretics with low-dose dopamine infusion and its significant biological effects such as improved renal function profile and potassium homeostasis have been described in the DAD-HF trial. We conclude that we need more solid data deciphering these intricate interactions in heart failure, which might ultimately translate to improved prognosis of this disease being associated with high mortality and morbidity.

Magnetic resonance imaging in heart failure, including coronary imaging: numbers, facts, and challenges: Editorial

Coronary artery disease (CAD) is a major risk factor for the incidence and progression of heart failure (HF). HF is characterized by a substantial morbidity and mortality and its lifetime risk is estimated at approximately 20% for men and women. As patients are in most cases identified only after developing overt clinical symptoms, detecting early stages of CAD and HF is of paramount importance. Due to its non‐invasiveness, excellent soft‐tissue contrast, high spatial resolution, and multiparametric nature, cardiovascular magnetic resonance (CMR) imaging has emerged as a promising radiation‐free technique to assess a wide range of cardiovascular diseases such as CAD or HF, enabling a comprehensive evaluation of myocardial anatomy, regional and global function, and viability with the additional benefit of in vivo tissue characterization. CMR has the potential to enhance our understanding of coronary atherosclerosis and the aetiology of HF on functional and biological levels, to identify patients at risk for CAD or HF, and to enable individualized patient management and improved outcomes. Even though larger‐scale studies on the different applications of CMR for the assessment of heart failure are scarce, recent research highlighted new possible clinical applications for CMR in the evaluation of CAD and HF.

Cardiac leiomyosarcoma: a rare cause of acute and progressive dyspnoea

Boris Bigalke*, Philipp Lohneis, Jens-Uwe Blohmer, Stephan Jacobs, and Ulf Landmesser Klinik für Kardiologie, Charité Universit€atsmedizin Berlin, Campus Benjamin Franklin, Hindenburgdamm 30, 12203 Berlin, Germany; Charité—Universit€atsmedizin Berlin, Campus Mitte, Klinik für Pathologie, Berlin, Germany; Charité—Universit€atsmedizin Berlin, Campus Mitte und Benjamin Franklin, Klinik für Gyn€akologie und Brustzentrum, Berlin, Germany; and Deutsches Herzzentrum Berlin, Cardiothoracic and Vascular Surgery, Berlin, Germany * Corresponding author. Tel: 149(0)30450513702, Fax: 149(0)30513999, Email: boris.bigalke@charite.de

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.

Update zu Diagnostik und Management der kardialen Sarkoidose

Zusammenfassung. Die Sarkoidose ist eine systemische Erkrankung des Bindegewebes mit Granulombildung. Die Mehrzahl der Patienten weist eine pulmonale Beteiligung auf. Eine kardiale Beteiligung ist in bis zu 25 % der Falle zu beobachten, wobei die Erkrankung haufig auch asymptomatisch verlauft. Klinisch manifestiert sich die kardiale Sarkoidose in der Regel als (dilatative) Kardiomyopathie oder durch Herzrhythmusstorungen. Eine klare Diagnose mit histologischem Nachweis der Epitheloidzellgranulome im Herzen gelingt durch eine Endomyokardbiopsie, wobei diese invasive Untersuchung eine begrenzte Sensitivitat aufweist. Neben der Elektrokardiographie und der Echokardiographie spielen insbesondere die kardiale Magnetresonanztomographie und die FDG-Positronenemissionstomographie (PET) eine zunehmende Rolle bei der Diagnose sowie Verlaufsbeurteilung der kardialen Sarkoidose. Die first line Therapie der kardialen Sarkoidose erfolgt in der Regel mittels Kortikosteroiden, im Einzelfall kann als second line Therapie ...