Sex Differences in Stress-Induced (Takotsubo) Cardiomyopathy.

Background: Takotsubo cardiomyopathy (TC) affects predominantly women. Prior studies have suggested that men might have worse short-term outcomes, but limited data are available regarding long-term outcomes. We hypothesized that men, compared to women, with TC have worse short- and long-term outcomes. Methods: A retrospective study of patients diagnosed with TC between 2005 and 2018 in the Veteran Affairs system was performed. Primary outcomes were in-hospital death, 30-day risk of stroke, death, and long-term mortality. Results: A total of 641 patients were included (444 men [69%]; 197 women [31%]). Men had a higher median age (65 vs 60 years; P < 0.001), and women were more likely to present with chest pain (68.7% vs 44.1%; P < 0.001). Physical triggers were more common in men (68.7% vs 44.1%, P < 0.001). Men had a higher in-hospital mortality rate (8.1% vs 1%; P < 0.001). On multivariable regression analysis, female sex was an independent predictor for improved in-hospital mortality, compared to men (odds ratio 0.25, 95% confidence interval 0.06-1.10; P = 0.04). On 30-day follow-up, no difference occurred in a combined outcome of stroke and death (3.9% vs 1.5%; P = 0.12). On long-term follow-up (3.7 ± 3.1 years), female sex was identified as an independent predictor of lower mortality (hazard ratio 0.71, 95% CI 0.51-0.97; P = 0.032). Women were more likely to have TC recurrence (3.6% vs 1.1%; P = 0.04). Conclusions: In our study with a predominantly male population, men had less-favourable short- and long-term outcomes after TC, compared to those of women.

Contexte: La cardiomyopathie de Takotsubo (CT) touche majoritairement les femmes. Or, des études antérieures semblent indiquer que les hommes pourraient connaître de pires résultats à court terme, mais peu de données portent sur les résultats à long terme. Nous avons formulé l'hypothèse selon laquelle les hommes atteints de CT obtiennent de moins bons résultats à court et à long terme que les femmes qui en sont atteintes. Méthodologie: Nous avons réalisé une étude rétrospective auprès des patients qui étaient inscrits au système de soins de santé du département des Anciens Combattants des États-Unis et qui avaient reçu un diagnostic de CT entre 2005 et 2018. Les critères d'évaluations principaux étaient le taux de décès à l'hôpital, le risque d'AVC sur 30 jours, le taux de décès et le taux de mortalité à long terme. Résultats: Au total, 641 patients ont été inclus dans l'étude (444 hommes [69 %]; 197 femmes [31 %]). L'âge médian était plus élevé chez les hommes (65 c. 60 ans; p < 0,001), et les femmes étaient plus susceptibles de présenter des douleurs à la poitrine (68,7 % c. 44,1 %; p < 0,001). Les déclencheurs physiques étaient plus fréquents chez les hommes (68,7 % c. 44,1 %; p < 0,001). Le taux de mortalité des hommes à l'hôpital était plus élevé (8,1 % c. 1 %; p < 0,001). L'analyse par régression multivariée a permis de constater que le sexe féminin était un indicateur prévisionnel indépendant d'un taux de mortalité plus faible à l'hôpital (rapport des cotes : 0,25; intervalle de confiance [IC] à 95 % : 0,06 à 1,10; p = 0,04). Lors du suivi au jour 30, aucune différence n'a été notée dans les résultats combinés d'AVC et de décès (3,9 % c. 1,5 %; p = 0,12). Lors du suivi à long terme (3,7 ± 3,1 ans), le sexe féminin a été ciblé comme un indicateur prévisionnel d'un plus faible taux de mortalité (rapport de risques instantanés : 0,71; IC à 95 % : 0,51 à 0,97; p = 0,032). Enfin, les femmes étaient plus susceptibles de connaître une récurrence de la maladie (3,6 % c. 1,1 %; p = 0,04). Conclusions: Dans notre étude portant sur une population à prédominance masculine, les hommes atteints de CT ont obtenu des résultats à court et à long terme moins favorables que les femmes atteintes de ce syndrome.

A Rare Case of Pulmonary Emboli Presenting With ST Elevation on ECG.

Pulmonary embolism is a common medical emergency and often life threatening but can be misdiagnosed frequently leading to fatal outcomes. Changes in electrocardiogram (ECG) are common in pulmonary embolism and rarely they can present with ST elevation. We here describe a 79-year-old woman who presented after a cardiac arrest and was found have ST-segment elevation on ECG with normal coronary angiogram while CT scan revealing pulmonary embolism.

Significant response to Pembrolizumab for metastatic cutaneous squamous cell carcinoma in patient with Netherton syndrome.

Background: Netherton syndrome is a rare autosomal recessive disease that presents with a triad of symptoms which include atopic diathesis, ichthyosis linearis circumflexa, and hair shaft abnormality termed "Bamboo Hair". Netherton syndrome patients can develop cutaneous squamous cell carcinoma (cSCC) in unusually young age. Pembrolizumab is the first line treatment for locally advanced and recurrent/metastatic cSCC. Case presentation: A 44-year-old man with a history of Netherton syndrome and multiple skin squamous cell carcinoma was diagnosed with locally advanced and recurrent/metastatic cSCC two years ago. He was started on Pembrolizumab as a treatment for his cSCC. The immunotherapy course was well tolerated with no significant side effects including the expected immune related adverse events seen in patients treated with this medication. PET/CT scan showed significant regression of his disease consistent with partial response according to the response evaluation criteria in solid tumors. Discussion: Incurable and recurrent cSCC tends to metastasize, leading to an extremely poor long-term prognosis, and the treatment options for locally advanced or metastatic disease are few. Pembrolizumab, an immune checkpoint inhibitors (ICIs) showed a benefit in patients with various tumors including squamous cell carcinoma, but using this drug which is working by enhancing the immunity against tumor in patient with altered immunity like Netherton syndrome was a bit of a challenge, in terms of both effectiveness and safety. Conclusion: Pembrolizumab had a effective and safe treatment profile when it was used as a monotherapy for treating a Netherton syndrome patient diagnosed with locally advanced and recurrent/metastatic cSCC.

Atypical CML with TET2 mutation, associated with NRAS and KRAS: A case report and literature review.

BACKGROUND: Atypical chronic myeloid leukemia (BCR-ABL1 negative) is a rare myeloid neoplasm with poor prognosis and no current standard of treatment. It features both myelodysplastic and myeloproliferative characteristics with little data regarding mutations playing a role in the disease. PRESENTATION OF CASE: We present a case of a 55-year-old female complaining of fever, cough, general weakness and night sweats. Examinations showed leukocytosis with a left shift, thrombocytopenia, hypercellular bone marrow with marked granulocytic hyperplasia and a negative BCR-ABL. After ruling out myelodysplastic and other myeloproliferative diseases the patient was finally diagnosed as aCML according to the WHO criteria with mutations in the TET2 gene, the NRAS gene and in the KRAS gene. The patient was started on Hydroxyurea for a duration of 9 months with an excellent initial response leading to normalization of her platelets and WBCs. However, in the last month she stopped responding to therapy and her state of health started declining once again. CONCLUSION: Atypical chronic myeloid leukemia (BCR-ABL1 negative with presence of TET2 gene mutation) is an unusual finding in myeloid neoplasms, have unknown prognosis and no current standard of treatment. It features both myelodysplastic and myeloproliferative characteristics with little data regarding mutations playing a role in the disease.

An unusual case of T-cell acute lymphoblastic leukemia in a patient with BCR-ABL positive chronic myeloid leukemia and Gaucher disease.

BACKGROUND: Chronic myelogenous leukemia (CML) is a chronic myeloproliferative disease characterized by a massive overproduction of myeloid cells. It is associated with the Philadelphia chromosome [Ph1, t (9; 22) (q34; q11)] or BCR-ABL fusion gene. CML usually undergoes a triphasic clinical course ending in a blast crisis, an accelerated phase of blasts and promyelocyte production. Ten percent of CML patients reach the blast crisis phase, with 20-30% of leukemias belonging to B-cell lymphoid lineage. However, a transformation of CML into T-cell acute lymphoblastic leukemia (T-ALL) is rare. CASE PRESENTATION: We present a 56-year-old male patient, known case of hypertension and Ph1 CML of eight years with a family history of Gaucher disease who developed T-ALL. The patient presented with lymphadenopathy and severe anemia, needing packed RBC transfusion, neutropenia and thrombocytopenia at the admission. However, the monocytes and basophils percentage were high. The patient underwent a cervical lymph node core biopsy, and the immunohistochemistry stains showed an invasion of neoplastic cells positive for CD3, CD5, BCL2, CD34, TdT and focally positive for C-Kit and negative for CD20, CD56 and pan-CK. These histopathology features were consistent with T-cell acute lymphoblastic leukemia (T-ALL). CONCLUSION: Blast crisis remain a challenge in CML management. It's of great importance to do a full proper workup including lymph nodes biopsies. The aim is to reverse blast crisis and restore the chronic phase.

Oral Anticoagulation Discontinuation Following Catheter Ablation of Typical Atrial Flutter.

Catheter ablation (CA) of typical atrial flutter (AFL) is the preferred treatment for typical AFL due to its excellent long-term success rate. However, current guidelines recommend pursuing oral anticoagulation (OAC) based on established indices of stroke regardless of the perceived success of ablation. We conducted a retrospective study of all patients who underwent typical AFL ablation at our institute from 2011 to 2017. All patients continued OAC for at least six weeks post-CA and underwent 24-hour Holter monitoring. OAC was discontinued if there was no evidence of recurrence at six weeks. In patients with low left ventricular ejection fraction or prior atrial fibrillation episodes, OAC was continued for six months with repeat Holter monitoring at six months. A total of 106 patients were included in our analysis, with a mean age of 64 ± 14 years and 78.3% of whom were male. The mean CHA2DS2-VASc score was 3 ± 1 points. OAC was discontinued by six weeks in 17% and at one year in 55.7% of patients, respectively, but was continued indefinitely in 44.3%. Over a mean follow-up period of 28.6 ± 27.3 months, there was one ischemic stroke in the OAC discontinuation group and no ischemic events in the continued OAC group. There were a total of three major bleeding events, all in the OAC group. In patients undergoing successful AFL ablation, a strategy of OAC discontinuation with close rhythm monitoring appears feasible. The benefit of continued OAC in this cohort may be outweighed by an adverse risk of bleeding. Further studies examining rhythm-guided OAC can minimize unnecessary exposure to long-term anticoagulation.

Cryoballoon vs radiofrequency ablation of atrial fibrillation: insights from the Veterans Healthcare System.

PURPOSE: Catheter ablation is considered the mainstay treatment for drug-refractory atrial fibrillation (AF). The aims of our study were to compare the efficacy and safety of the most two currently approved approaches (point-by-point radiofrequency ablation (RFA), either with contact force (CF) or without contact force (nCF) catheters, and cryoballoon ablation (CBA)) in the Veterans Healthcare System. METHODS: We performed a retrospective study of patients who underwent ablation for treatment of AF at the veterans affairs healthcare system between 2013 and 2018. Only the first reported ablation procedure was included. RESULTS: We included 956 patients in the study (97.4% males, 91.5% Caucasians, 67% paroxysmal AF), with 682 patients in RFA-nCF, 139 in RFA-CF, and 135 in CBA. Thirty-day complication rates were comparable between the three groups with the exception of higher incidence of phrenic nerve injury in CBA group when compared to RFA-nCF (2.2% vs 0.0%, p < 0.01). Long-term recurrence rate of AF was significantly lower in the CBA group when compared to RFA-nCF (33.3% vs 47.7%, adjusted HR 0.60, 95% CI 0.44-0.83, p < 0.01). On the other hand, it was similar between RFA-CF and RFA-nCF groups (43.9% vs 47.7%, adjusted HR 1.01, 95% CI 0.76-1.33, p 0.97). After stratifying patients based on AF type, these findings were only present in patients with paroxysmal AF. CONCLUSION: CBA for paroxysmal AF, in male dominant patients' population, was associated with lower incidence of AF recurrence rate while having a comparable safety profile to RFA independent of the use of CF catheters.

IVIG and under Burn Unit Care Yield Favorable Outcomes in Pediatric Patients with Toxic Epidermal Necrolysis: A Case Report and Literature Review.

Body reactions to drugs can manifest as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). TEN is the most severe form of cutaneous reactions with an incidence rate of 1-2 per million cases per year. Despite TEN being a critical and life-threatening condition, there is little to no evidence of clear management protocol. We reported a 5-year-old male child who presented with lamotrigine-induced TEN and was successfully treated with intravenous immune globulin (IVIG) with a burn unit care level, while TEN treatment with IVIG is an appropriate approach with predictable good outcomes, burn unit care is also effective in creating highly favorable effects. Upon reviewing the literature, several studies indicate that TEN patients treated with the combination of IVIG and burn unit care lead to decreased levels of morbidity and mortality than when treated with IVIG or burn unit care alone. Therefore, treatment involving both IVIG and burn unit care should be considered for TEN patients.

Cocaine Use and Pulmonary Hypertension.

Evidence linking cocaine to the risk of pulmonary hypertension (PH) is limited and inconsistent. We examined whether cocaine use, in the absence of other known causes of PH, was associated with elevated systolic pulmonary artery pressure (sPAP) and increased probability of PH. We compared patients with documented cocaine use to a randomly selected age, sex, and race-matched control group without history of cocaine use. All participants had no known causes of PH and underwent echocardiography for noninvasive estimation of sPAP. We used routinely reported echocardiographic parameters and contemporary guidelines to grade the probability of PH. In 88 patients with documented cocaine use (mean age ± standard deviation 51.7 ± 9.5 years), 33% were women and 89% were of Black race. The commonest route of cocaine use was smoking (74%). Cocaine users compared with the control group had significantly higher sPAP (mean ± standard deviation, 30.1 ± 13.1 vs 22.0 ± 9.8 mm Hg, p <0.001) and greater likelihood of PH (25% vs 10%, p = 0.012). In multivariable analyses adjusted for potential confounders including left ventricular diastolic dysfunction, cocaine use conferred a fivefold greater odds of echocardiographic PH (p = 0.006). Additionally, a stepwise increase in the likelihood of PH was noted across cocaine users with negative or no drug screen on the day of echocardiography to cocaine users with a positive drug screen (multivariable p for trend = 0.008). In conclusion, cocaine use was associated with a higher sPAP and an increased likelihood of echocardiographic PH with a probable acute-on-chronic effect.

A comparison of anti-arrhythmic efficacy of carvedilol vs metoprolol succinate in patients with implantable cardioverter-defibrillators.

BACKGROUND: The effects of carvedilol and metoprolol succinate on appropriate and inappropriate implantable cardioverter defibrillator (ICD) therapy in patients with heart failure with reduced ejection fraction (HFrEF) are not fully understood. HYPOTHESIS: The hypothesis of our study is possible carvedilol superiority over metoprolol in patients with ICD. METHODS: All patients with ICD registered to a single device clinic between 1/2012 and 6/2017 (n = 569) were identified. Patients with systolic heart failure (left ventricular ejection fraction ≤40%) treated with carvedilol vs metoprolol succinate were compared. Primary endpoint was difference in survival free of appropriate device therapy (shock or anti-tachycardia pacing, ATP). Secondary endpoints were freedom from inappropriate therapy (shock or ATP) and all cause death. RESULTS: A total of 225 patients were included in the analysis with median follow up of 57 months (IQR 33.7-90). The 2 groups were comparable in the baseline characteristics. Carvedilol was superior to metoprolol succinate in improving survival free of appropriate ICD therapy (HR 0.42; 95% CI 0.24-0.72, P = 0.01). This difference was driven by reduction in survival free of appropriate shocks (HR 0.30; 95% CI 0.15-0.63, P = -0.01) while there was no significant difference in appropriate ATP (HR 0.55; 95% CI 0.28-1.1, P = 0.12). There was no significant difference in time to inappropriate shocks (HR 1.02; 95% CI 0.19-5.6, P = 0.97), inappropriate ATP (HR 0.93, OR 0.24-3.5, p value 0.9) or all cause death (HR 0.8; 95% CI 0.42-1.5, P = 0.52). CONCLUSIONS: This study suggests that carvedilol use was associated with improved survival free of appropriate ICD therapy compared to metoprolol succinate in patients with HFrEF.