Inverted Takotsubo Research Articles

Pheochromocytoma as a reversible cause of cardiomyopathy: Analysis and review of the literature

ContextPheochromocytoma and paraganglioma are rare neuroendocrine tumors which overproduce catecholamines and arise from the adrenal gland or extra-adrenal chromaffin cells of the sympathetic and parasympathetic ganglia (1). Excessive catecholamine-induced stimulation of cardiac myocytes leads to damage which manifests in several forms ranging from Takotsubo to dilated cardiomyopathy. Diagnosis of pheochromocytoma-related cardiomyopathies is often delayed due to the atypical presentation associated with many cases. ObjectiveLimited data exists on the presentation and outcomes of the various forms of pheochromocytoma-induced cardiomyopathies. We performed a literature review to assess the association of pheochromocytoma and cardiomyopathy to aide in further understanding this clinical entity. Design163 cases from 150 articles published between 1991 and November 2016 were included from a PubMed search. ResultsThere were 163 occurrences of pheochromocytoma and cardiomyopathy (63 dilated cardiomyopathy, 38 Takotsubo cardiomyopathy, 30 inverted Takotsubo cardiomyopathy, 10 HOCM, 8 myocarditis, and 14 unspecified cardiomyopathy). Many patients lacked classic signs or symptoms of pheochromocytoma with hypertension as a presenting symptom in 65% and the triad of headache, palpitations, and diaphoresis only in 4%. Resection of the pheochromocytoma led to improvement of the cardiomyopathy in 96% while lack of resection was associated with death or cardiac transplantation in 44%. ConclusionPheochromocytoma should be considered in the evaluation of non-ischemic, non-valvular cardiomyopathy even in the absence of symptoms of catecholamine excess. Our study highlights the importance of early suspicion and diagnosis of pheochromocytoma in cases of idiopathic heart failure as early resection may prevent progression to irreversible myocardial remodeling and death.

Pheochromocytoma presenting as inverted Takotsubo cardiomyopathy

The classical entity of Takotsubo cardiomyopathy is well established in the literature, but mechanisms explaining it remain unelucidated. Recently, the uncommon inverted Takotsubo type (sparing apical ballooning) has been more frequently described. We report the case of a 26-year-old man admitted with gastrointestinal symptoms, whose clinical presentation for a stress-related cardiomyopathy, which usually presents with cardiopulmonary symptoms, was rather atypical. The cardiac assessment including echocardiography and cardiac magnetic resonance imaging (MRI) demonstrated a dilated cardiomyopathy, whereas coronary angiography showed the absence of atherosclerotic disease. The abdominal computed tomography (CT) scan revealed a left adrenal mass, and elevated urinary catecholamine levels were highly suggestive of a pheochromocytoma. Prompt medical and surgical treatments were instituted. During the left adrenalectomy the patient suffered from brief electromechanical dissociation requiring aggressive resuscitation. Postoperative course was unremarkable. Reverse Takotsubo heart failure is a recently recognized syndrome and a systematic review of the literature of 10 cases of pheochromocytoma-induced inverted Takotsubo is presented in the present article.

Association of inverted Takotsubo cardiomyopathy with postpartum pneumo-mediastinum: when a “broken lung” meets a “broken heart”

Association of inverted Takotsubo cardiomyopathy with postpartum pneumo-mediastinum 1 1 3 Six hours after an uneventful delivery, a 30-year-old primiparous woman complained of pleuritic-type chest pain and sense of immense apprehension. Initially, Troponin-I (Tn-I) level was normal (0.01 ng/mL), but D-dimer was elevated (1200 μg/L). Pulmonary embolism was excluded by a computed tomography scan, which revealed signs of pneumo-mediastinum and subcutaneous emphysema (a). Shortly after, due to raised Tn-I level to 1.88 ng/mL and T-wave inversion in anterior precordial leads, she was referred to us. Angiography disclosed normal coronary arteries (b, c), but wall-motion abnormalities (WMA) in basal segments were noted (d). Cardiac magnetic resonance imaging excluded late gadolinium enhancement (e, f ). Transthoracic echocardiography showed significant hypoto akinesis of basal inferior and posterior left ventricular wall and dyskinesis of basal anteroseptal segments (g, h). Estimated ejection fraction (EF) by 3D quantification was 27 % (i, j). This peculiar pattern of WMA with clinical settings and normal coronary arteries was consistent with reversed/ inverted Takotsubo cardiomyopathy (ITC). She received appropriate management with beta-blockers and angiotensin-converting enzyme inhibitors. EF was completely normalized after 1 month (k, l). To the best of our knowledge, this association of pneumo-mediastinum and ITC has not been reported. Notably, ITC occurs more frequently in younger age and is more commonly associated with mental stress than the typical forms of Takotsubo cardiomyopathy.

Inverted-Takotsubo Cardiomyopathy in a Patient with Pulmonary Embolism

As the use of early coronary angiography and echocardiography become widely available in the setting of acute coronary syndrome, the gradual increase for variant forms of transient left ventricular (LV) apical ballooning syndrome have been recognized. This syndrome usually occurs in women and is frequently elicited by an intense emotional, psychological, and physical event. While the patients' characteristics between typical and non-typical LV ballooning syndrome seem to differ, the presentation, clinical features, and reversibility of LV wall motion abnormalities are similar. We present a middle-aged woman who experienced inverted takotsubo cardiomyopathy triggered by pulmonary embolism. To the best of our knowledge, this case is particularly unique and is rarely reported in the disease entity.

Endomyocardial biopsy in a patient with hemorrhagic pheochromocytoma presenting as inverted Takotsubo cardiomyopathy

A 29-year-old female patient presented with shock and dyspnea due to heart failure and pulmonary edema. Echocardiography indicated excessive contraction limited to the left ventricular apex and akinesis of the basal and middle ventricle, which were confirmed by emergency left ventriculography. The finding was diagnostic of inverted Takotsubo cardiomyopathy. An abdominal computed tomography scan showed a tumor in the left adrenal gland with a central low-density area, and the plasma and urinary catecholamines were strikingly elevated. Taken together, these findings suggested the presence of a hemorrhagic pheochromocytoma. A myocardial biopsy in the very acute stage on the day of admission revealed neutrophilic infiltration and contraction-band necrosis, which was indistinguishable from the previously reported pathology in the acute phase of idiopathic Takotsubo cardiomyopathy without pheochromocytoma. The diagnosis of pheochromocytoma in this case was confirmed 7 weeks later by surgical removal of the left adrenal gland with massive hemorrhage at the center of the pheochromocytoma. The marked similarity of the endomyocardial pathology between this case and cases with idiopathic Takotsubo cardiomyopathy strongly points to catecholamine excess as a common causality for Takotsubo cardiomyopathy with or without pheochromocytoma.

Temporary Mechanical Circulatory Support for Takotsubo Cardiomyopathy Secondary to Primary Mediastinal B-Cell Lymphoma

Left heart mechanical circulatory support (MCS) through the left chest via the pulmonary vein and descending thoracic aorta is a good option for patients with an inaccessible anterior mediastinum and/or poor peripheral access. We report the case of a 19-year-old small female with a newly discovered bulky primary mediastinal diffuse large B-cell lymphoma (PMBL) who developed refractory inverted Takotsubo cardiomyopathy (TC) with cardiogenic shock. Temporary MCS was implemented in order to stabilize the patient and proceed with a chemotherapy treatment. Given the patient's oncologic "frozen" mediastinum and the presence of poor peripheral arterial access, the left heart temporary MCS was successfully implanted through a left mini-thoracotomy via the left inferior pulmonary vein and descending thoracic aorta. This is the first report of temporary MCS to treat inverted TC and diffuse PMBL.

Atypical Takotsubo syndrome during anagrelide therapy

Anagrelide is a phosphodiesterase III inhibitor utilized in the treatment of essential thrombocythemia. Anagrelide can be responsible for positive inotropic and chonotropic activity of the cardiovascular system. Moreover, it can induce vasospam directly on the epicardial coronary arteries. In the literature, it is well reported that this inhibitor can determine serious cardiovascular side effects, including congestive heart failure, arrhythmia and acute coronary syndrome. We describe the case of a 75-year-old woman who developed a mid-ventricular Takotsubo syndrome while on anagrelide therapy. Takotsubo cardiomyopathy, also known as left ventricular ballooning syndrome, is characterized by a reversible ventricular contractile dysfunction with akinesis and expansion of apical segments and hyperkinesis of the basal segments. Recently, atypical cases with akinesia and dilation of mid-ventricular segment and hypercontraction of the apical segments, also called mid-ventricular and inverted Takotsubo syndrome, have been described. Even though the pathogenesis of Takotsubo syndrome is poorly understood, several mechanisms have been proposed, including catecholamine-induced myocardial stunning, and ischemia-mediated stunning due to multivessel epicardial or microvascular spasm. We think that in our case, the adverse response of anagrelide therapy was determined, by accumulated dosage of the drug, through an intensive inotropic stimulation and a sympathetic hyperactivation in a vulnerable myocardium. To our knowledge, this is one of the first reports of an association between anagrelide therapy and Takotsubo cardiomyopathy.

Contractile pattern of inverted Takotsubo cardiomyopathy: illustration by two-dimensional strain

We report a case of a 25-year-old man who was admitted to our emergency department for brain trauma, with electrocardiographic and echocardiographic features suggestive of inverted Takotsubo cardiomyopathy. Using myocardial strain obtained from bidimensional acquisitions, we describe the myocardial abnormalities in this patient presenting with this yet under-recognized syndrome.

Pheochromocytoma-induced inverted Takotsubo cardiomyopathy: A case of patient resuscitation with extracorporeal life support

Pheochromocytoma-induced inverted Takotsubo cardiomyopathy: A case of patient resuscitation with extracorporeal life support