Background: Cardiogenic shock (CS) is a significant complication of Takotsubo syndrome (TTS), contributing to heightened mortality and morbidity. Despite this, the Society for Cardiovascular Angiography and Interventions (SCAI) staging system for CS severity lacks validation in patients with TTS and CS. This study aimed to characterize a patient cohort with TTS using the SCAI staging system and assess its utility in cases of TTS complicated by CS. Methods and Results: From a TTS national registry, 1591 consecutive patients were initially enrolled and stratified into 5 SCAI stages (A through E). Primary outcome was all‐cause in‐hospital mortality; secondary end points were TTS‐related in‐hospital complications and 1‐year all‐cause mortality. After exclusions, the final cohort comprised 1163 patients, mean age 71.0±11.8 years, and 87% were female. Patients were categorized across SCAI shock stages as follows: A 72.1%, B 12.2%, C 11.2%, D 2.7%, and E 1.8%. Significant variations in baseline demographics, comorbidities, clinical presentations, and in‐hospital courses were observed across SCAI shock stages. After multivariable adjustment, each higher SCAI shock stage showed a significant association with increased in‐hospital mortality (adjusted odds ratio: 1.77–29.31) compared with SCAI shock stage A. Higher SCAI shock stages were also associated with increased 1‐year mortality. Conclusions: In a large multicenter patient cohort with TTS, the functional SCAI shock stage classification effectively stratified mortality risk, revealing a continuum of escalating shock severity with higher stages correlating with increased in‐hospital mortality. This study highlights the applicability and prognostic value of the SCAI staging system in TTS‐related CS.

Background: Sex‐related differences in Takotsubo syndrome have been described, but no information is available in patients who develop cardiogenic shock. Methods and Results: Of 412 patients with Takotsubo syndrome with cardiogenic shock, 71 (17.2%) were men. Male patients were older (71.1±12.2 versus 65.3±17.1 years, P <0.001), more frequently smokers (47 [66.2%] versus 66 [19.4%], P <0.01), with higher prevalence of neoplasms (6 [8.5%] versus 8 [2.3%], P =0.01), lower left ventricular ejection fraction (31% versus 37%, P <0.001), more frequent invasive mechanical ventilation (30 [42.3%] versus 90 [26.4%], P =<0.01), higher rate of infections (43 [60.6%] versus 148 [43.4%], P =<0.01), and longer in‐hospital stay (19±20 days versus 13±15 days, P =0.02). A total of 55 patients (13.3%) died during hospital admission, and 90 patients (21.8%) died at the end of the 5‐year follow‐up. Male sex was not significantly associated with the in‐hospital (odds ratio, 1.31 [95% CI, 0.64–2.68]) or 5‐year mortality rate (hazard ratio, 1.66 [95% CI, 0.93–2.94]). In the matched cohort, no significant differences in the short‐ and long‐term mortality rate were found either. Conclusions: Cardiogenic shock due to Takotsubo syndrome has high short‐ and long‐term mortality rates that are similar in men and women.

Systemic hypertension, a significant global health issue and a leading risk factor for cardiovascular mortality affects half of the adult population, with increasing prevalence notably in low- and middle-income countries. Despite advancements in diagnosis and treatment, only one in four individuals with hypertension achieve satisfactory control over their condition. Medication adherence, critical for effective hypertension management, is complex and multifaceted. Non-adherence, encompassing late or non-initiation, sub-optimal implementation, and early discontinuation of treatment, is prevalent worldwide, with reported rates of anti-hypertensive medication non-adherence ranging from 30% to 40%. Adherence is influenced by various factors including drug regimen complexity, patient education, and socioeconomic status. Poor adherence is linked to increased cardiovascular risks and is compounded by clinical inertia among physicians. Addressing barriers to adherence and implementing evidence-based interventions can significantly reduce the global burden of hypertension and its associated complications. This review highlighted the critical need for improved adherence strategies to enhance hypertension management. It focused on novel tools such as mobile health interventions and regimen-simplification through single-pill combinations, which can improve treatment persistence and blood pressure control.

Background: Neurofibromatosis type 1 (NF1) is a rare multisystem disorder characterized by variable expressivity and increased tumor risk. Café-au-lait macules (CALMs) are a hallmark of the disease, often representing one of the earliest clinical manifestations and allowing a clinical NF1 diagnosis if six or more are present. In this study, we aimed to investigate the prognostic value of CALMs at birth in NF1 patients. Methods: We conducted a retrospective study in patients aged ≥ 4 years presenting with CALMs at our Institution between 2020 and 2021, with a minimum follow-up of four years. We retrospectively collected data on CALMs at birth and other clinical manifestations associated with NF1. Results: Among 208 patients evaluated, including 147 with a confirmed diagnosis of NF1, 110 did not show CALMs at birth, and 98 had at least one. The absence of CALMs at birth did not correlate with a lower likelihood of NF1. In contrast, the CALM number at birth directly correlated with the likelihood of NF1, up to 95% in patients with ≥5 macules. Additionally, a higher number of CALMs correlated with a greater prevalence of plexiform neurofibromas (p < 0.001). Conclusions: Our findings suggest that a higher number of CALMs may indicate a more severe form of NF1, with an increased risk of plexiform neurofibromas. These results emphasize the importance of a comprehensive evaluation of patients with CALMs, especially in case of multiple lesions, aiming at implementing early NF1 diagnosis, follow-up strategies, and overall patient management.

Background: D-Transposition of the great arteries (d-TGA) is characterized by the aorta positioned above the right ventricle and the pulmonary artery above the left ventricle. Acute pulmonary artery dissection (PAD) is a rare and often lethal condition. We present a case report of acute PAD in an adult with d-TGA and pulmonary hypertension. Case summary: A 49-year-old male with history of d-TGA palliated with an atrial switch (Mustard) operation, pulmonary venous baffle stenosis treated percutaneously, chronic pulmonary hypertension (mixed group 1 and 2), and severe dilatation of pulmonary arteries (pulmonary trunk of 75 mm) presented to the emergency department with chest pain and acute respiratory failure. Blood pressure was 106/78 mmHg, heart rate 93 b.p.m., and oxygen saturation 88% on room air. A computed tomography (CT) scan showed acute right PAD. He was not considered suitable for surgery nor percutaneous procedure. Epoprostenol was initiated to reduce parietal stress, but after initial stabilization, pulmonary venous stent baffle gradient increased and acute pulmonary oedema occurred. Epoprostenol was withdrawn, and furosemide was initiated, with good clinical response. A follow-up CT scan showed dissection morphological stabilization and false lumen thrombosis, and the patient was discharged. Discussion: Pulmonary hypertension and previous pulmonary artery dilatation are reported as the main underlying conditions leading to PAD. No previous cases of PAD are described in patients with history of d-TGA and atrial switch procedure. Evidence regarding the best treatment of PAD is lacking, but it seems reasonable to reduce parietal stress using pulmonary vasodilators. Nevertheless, in patients with post-capillary pulmonary hypertension, pulmonary vasodilatation may cause important pulmonary congestion.

Background: Aortic coarctation is a major risk factor for high blood pressure and atherosclerotic disease development. Evidence is lacking regarding the treatment of acute coronary syndrome in patients with untreated aortic coarctation. Case summary: A 50-year-old male with a history of hypertension, diabetes, and haemodynamically significant untreated aortic coarctation presented to the emergency department with non-ST-elevation acute myocardial infarction. Coronary catheterization showed severe three-vessel disease. The aortic coarctation was addressed percutaneously using a covered CP stent. A quadruple coronary artery bypass surgery was conducted the following day. He was discharged home 10 days after surgery. Discussion: We describe a successful sequential approach involving the percutaneous repair of a native aortic coarctation followed by a surgical myocardial revascularization in the context of acute coronary syndrome. Discussion within a multidisciplinary Heart Team is key in patients with such complexity.