Recurrent Takotsubo Cardiomyopathy due to Pheochromocytoma Managed With Venoarterial Extracorporeal Membrane Oxygenation.

Pheochromocytoma-induced Takotsubo cardiomyopathy is a rare but life-threatening condition, caused by excessive plasma catecholamine levels, resulting in acute myocardial dysfunction. Clinical presentation includes a rapid development of heart failure due to regional wall motion abnormalities (most commonly affecting all mid to apical left ventricle (LV) wall segments) causing the "octopus-trap-like" LV shape. A 45-year-old female patient presented with acute cardiogenic shock of non-ischemic etiology. Her past medical history included a similar episode, which was followed by full recovery, but at this admission she required hemodynamic support with venoarterial extracorporeal membrane oxygenation. The systolic function was restored, and further investigation revealed high 24-h urine metanephrine levels and a mass of the left adrenal gland, leading to the diagnosis of pheochromocytoma. After treatment with firstly alpha-blockers and then beta-blockers, the pheochromocytoma was surgically removed.

Synchronous Left Ventricular and Endocranial Mass.

Myocardial cysts represent a miscellaneous and infrequent spectrum of conditions, with each of them coming from a different etiological background. Congenital myocardial cysts, neoplasia, cysts of infectious origin (bacterial, viral, or parasitic), and cardiac pathologies that may fake cystic content are all encompassed in this group. Although most patients are asymptomatic, some may occasionally present with obstruction, valvular dysfunction, or heart failure. Even more uncommon is the coexistence of a myocardial cyst with other extracardiac locations causing extracardiac symptoms. In this direction, the coexistence of a myocardial and endocranial cyst is extremely rare and can cause symptomatology from the affected organs (e.g., seizures). Cardiac investigation in this context is mainly dependent on non-invasive diagnostic modalities, and laboratory procedures. In this case report, we present a 26-year-old Congolese male admitted with dyspnea and epileptic seizures. Echocardiography revealed left ventricular and both mitral and tricuspid valve dysfunction and the presence of two myocardial cysts, while brain computed tomography showed an additional frontal cystic lesion. A precise diagnostic workup with a combination of non-invasive imaging, laboratory results, and epidemiology data assisted the diagnosis and guided the most suitable therapeutic choice.

Hypertrophic Cardiomyopathy: A Cardiovascular Challenge Becoming a Contemporary Treatable Disease.

Hypertrophic cardiomyopathy is one of the most common genetic inherited diseases of myocardium, which is caused by mutation in genes encoding proteins for the cardiac sarcomere. It is the most frequent cause of sudden death in young people and trained athletes. All diagnostic methods, including heart catheterization, transthoracic and transesophageal echocardiography, magnetic resonance imaging, genetic counseling and tissue biopsy are required for risk and therapy stratification and should be individualized depending on phenotype and genotype. Current therapy has not been tested adequately. Beta-blockers and verapamil can cause hypotension which can make hypertrophic cardiomyopathy worse. Disopyramide has been inadequately studied, and mavacamten was only studied in small trials. More definitive trials are currently ongoing. Novel invasive and noninvasive diagnostics, medical therapies, interventional and surgical approaches tend to influence the natural history of the disease, favoring a better future for this patient population.

Myocarditis Related to the Use of Mesalazine.

Myocarditis is a rare complication of therapy with mesalazine, a drug traditionally used in the treatment of inflammatory bowel disease. We report a case of a 32-year-old man with a recent diagnosis of ulcerative colitis, who presented to our hospital with chest pain and elevated troponin, 12 days following initiation of mesalazine. Diagnosis of myocarditis was confirmed with cardiac magnetic resonance imaging (CMR), which showed subepicardial gadolinium enhancement in the basal lateral/inferolateral segment of the heart. The patient's clinical condition improved upon stopping mesalazine and the follow-up CMR demonstrated resolution of the previous findings. Mesalazine can cause myocarditis early after initiation and clinicians should be aware of this rare yet serious cardiotoxic effect, as the discontinuation of the medication is the mainstay of treatment and leads to significant recovery.

The Conjunction Conundrum in Transcatheter Aortic Valve Implantation.

A continuous discussion regarding the predictors for permanent pacemaker implantation (PPI) following transcatheter aortic valve implantation (TAVI) is ongoing, especially in the era of low and medium risk patients. The aim of this article is to review the data so far regarding the pathophysiology, risk factors, and the indications for permanent pacemaker implantation after TAVI. The factors that contribute to rhythm abnormalities post TAVI can be divided into pre-existing conduction abnormalities, patient-related anatomical factors, and peri-procedural technical factors. The latter components are potentially modifiable, and this is where attention should be directed, particularly now that in an era of TAVI expansion towards lower-risk patients.

Is Long COVID Syndrome a Transient Mitochondriopathy Newly Discovered: Implications of CPET.

The new outbreak of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has an impact worldwide, primarily as a medical emergency. Even that the total constellation is considered as a pandemic of acute respiratory disease, coronavirus disease 2019 (COVID-19) gives rise to dysfunction in multiple organs (e.g., brain, lungs, heart, muscles) that impairs cardiopulmonary (CP) function. Parallel to the CP consequences of COVID-19 is a significant reduction in physical activity. Cardiopulmonary exercise testing (CPET) is daily used in clinical practice to define prognosis, provide risk stratification and treatment strategy. As such, the significance of CPET is crucial concerning clinical assessments of COVID-19 patients. Furthermore, new studies aim at understanding the effects of SARS-CoV-2 infection in long term. Multiple studies have investigated the cardiopulmonary function and impairment of exercise endurance in such patients, as well as a possible prolonged physical impairment. With this review, we summarize the COVID-19-associated pathophysiology for the Long COVID (LC) syndrome as well as the importance of performing CPET.

Amphetamine Use Revealing Hypertrophic Cardiomyopathy in a Young Patient.

Adrenergic overstimulation in long term can lead to a hyperdynamic myocardium and give rise to hypertrophy and ultimately to heart failure. Amphetamine use is a common cause of neurohormonal activation, which gives rise to such adverse cardiovascular events. However, hypertrophy of myocardium in young patients, even due to apparently obvious causes, should always be considered as a red flag and a further diagnostic downstream should take place, in order to exclude genetic causes. We present a case of a young man with chronic use of amphetamine and an incidental finding of hypertrophic cardiomyopathy.

"Broken Heart" and "Broken Brain": Which Connection?

The interconnections between brain and heart are increasingly recognized. Takotsubo cardiomyopathy, also known as "broken heart syndrome", is characterized by a cardiovascular dysfunction provoked by an emotional or stressful situation. Similar events can trigger a neurological pathology called transient global amnesia. These conditions can occur simultaneously, although their precise connection is not well understood. We aim to present the case of a patient who experienced them and to review the relevant literature.

Exercise Oscillatory Ventilation in Hypertrophic Cardiomyopathy.

To assess the prevalence and clinical correlates of exercise oscillatory ventilation (EOV) in patients with hypertrophic cardiomyopathy (HCM). Retrospective single-center study. Thirty-six consecutive HCM patients who underwent cardiopulmonary exercise testing. Two patients (5.6%) had EOV. Both patients with peak oxygen consumption (VO2) less than or equal to 9.1 ml/kg/min had EOV. Left atrial size was greater in patients with EOV. Of the 2 patients in the study population with an abnormal blood pressure response to exercise, 1 had EOV. Both patients with New York Heart Association Class 3 heart failure had EOV. This is the first report of EOV in HCM. EOV is uncommon in patients with HCM. EOV appears to be a marker of disease severity as evidenced by overt heart failure, left atrial enlargement, and low peak VO2.

Myosin Modulators: The New Era of Medical Therapy for Systolic Heart Failure and Hypertrophic Cardiomyopathy.

A new uprising pharmacological treatment for systolic heart failure and hypertrophic cardiomyopathy demonstrates very promising results the last years. Myosin modulators have already been tested in numerous studies. Myosin inhibitor (mavacamten) and myosin stimulator, (omecamtiv mecarbil) exhibit their effect by improving clinical outcomes, as well as reducing morbidity and mortality. More studies are however required for further evaluation and particularly effect on sarcomeric level. Side effects of both pharmacological agents have been described and should always be close monitored. Cardiopulmonary exercise test has a pivotal role by means of assessing treatment efficacy.

Cardiopulmonary Exercise Test-The Revolving Door of Left Ventricular Assist Devices in Heart Failure.

The prevalence of heart failure has an increasing tendency in the last years. Either heart failure with reduced ejection fraction (HFrEF) or with preserved ejection fraction, the treatment depends on the severity, cause, and symptoms. In case of HFrEF, careful evaluation of patient is essential for proper diagnosis, risk stratification and treatment, which should always be individualistic. Except from daily measurements, medical treatment and eventually implantation of implantable cardioverter defibrillator or cardiac resynchronization therapy, implantation of left ventricular assist device (LVAD) belongs also to therapeutic armamentarium. Other than invasive procedures, which are required for the evaluation of every patient with HFrEF, Cardiopulmonary exercise test emerges as one of the most effective noninvasive method for diagnosis, risk stratification, and treatment strategy for these patients. Cardiopulmonary exercise test can provide means for a critical evaluation of cardiovascular system. One of the most important variables is the maximal oxygen consumption (peak VO2). Its high predictive and prognostic power makes peak VO2 essential for the evaluation of patients as candidates, not only for LVAD-implantation, but also for explantation. Furthermore, regarding cardiac rehabilitation and exercise protocols, robust literature supports a follow-up of LVAD-patients by means of cardiopulmonary exercise testing.

The Role of Mitral Valve in Hypertrophic Obstructive Cardiomyopathy: An Updated Review.

Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease and the most common cause of sudden cardiac death in young population, especially in athletes under 35 years old. Mutations occur primarily in the ß-myosin heavy chain gene and involve the cardiac myosin-binding protein C gene. In this review we would like to focus on the importance of the examination of mitral valve apparatus and the mitral valve abnormalities in patients with HCM. Abnormalities in mitral valve (elongated mitral leaflets, displacement of papillary muscles, and systolic anterior motion) may be the primary pathognomonic elements, even in the absence of hypertrophy. Echocardiography is the gold standard for the diagnosis of HCM. Magnetic resonance imaging emerges as one of the most important imaging modalities for precise diagnosis, assisting in risk stratification and treatment strategy. Mitral valve abnormalities take part fundamentally in the formation of systolic anterior motion of the mitral valve and, they have substantially been repaired surgically. Although myectomy addresses the septum reduction, obstruction relief should be maximally achieved with a potential combination of myectomy and mitral valve repair.

Causes, Diagnosis, Risk Stratification and Treatment of Bicuspid Aortic Valve Disease: An Updated Review.

The most common congenital heart disease is the bicuspid aortic valve. Understanding the pathophysiology and the altered hemodynamics is a key component for the diagnosis, risk stratification and treatment. Among others, aortic valve stenosis is the most common complication. Treatment strategies vary depending on the severity of the disease, particularly the dilation of the aorta playing a major role. Together with valve replacement, transcatheter aortic valve implantation is now considered as an alternative option with good results. With this review we would like to discuss the causes, diagnostic methods, risk stratification and treatment strategies of the bicuspid aortic valve.

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.