LIVEDO RETICULARIS: CLINICAL SIGNIFICANCE AND TREATMENT

Livedo reticularis (LR) is a hyperpigmented discoloration of the skin characterized by a violet, reticulated cyanotic pattern, and is more common on the extremities and trunk. LR is in the form of intact circular networks. If the circular reticulated appearance is distorted and shows an irregular fracture pattern, it is defined as livedo racemosa (LRC). LR is a benign, primary disease that affects young and middle-aged women. LRC, on the other hand, is a secondary disease, pathological and permanent. In LR, the vivid cone discoloration is symmetrical, reversible, and uniform. In LRC, the vivid cone discoloration is irreversible, and fractured. Although it has been stated as a concept that the pathological livedoid form is LCR, there is no clear distinction between LR and LRC in clinical studies and generally 'livedo reticularis' is used to describe both. Our study includes eight patients diagnosed with livedo reticularis between January 2013 and May 2021. One of our patients was male and the other was female. Their ages ranged from 25 to 70 years and the mean age was 45.5±16.7 years. Although the main complaints were coldness, numbness and pain, aesthetic anxiety was prominent in all patients. On physical examination, violet-colored fishing net-like appearances were noted on the lower extremities of all patients. It was accompanied by venous insufficiency in six of the patients. As a result of the treatment we applied, there was improvement in venous insufficiency. However, as a result of the vasodilator treatment we applied for cosmological purposes, there was no obvious improvement in the reticulated appearances. Because of the risk of developing neurovascular and cardiovascular complications several years after the onset of livedoid vasculopathy, it is important to monitor these patients. Considering that LR may be seen before pulmonary symptoms during the COVID-19 pandemic period, necessary tests should be performed to rule out the diagnosis of COVID-19 in these cases.

Anesthesia modality does not affect clinical outcomes of intra-arterial vasodilator treatment in patients with symptomatic cerebral vasospasms

Background: Delayed cerebral ischemia and cerebral vasospasm remain the leading causes of poor outcome in survivors of aneurysmal subarachnoid hemorrhage. Refractory cerebral vasospasms can be treated with endovascular vasodilator therapy, which can either be performed in conscious sedation or general anesthesia. The aim of this study is to compare the effect of the anesthesia modality on long-term clinical outcomes in patients undergoing endovascular vasodilator therapy due to cerebral vasospasm and hypoperfusion. Methods: Modified Rankin Scale (mRS) scores were retrospectively analyzed at time of discharge from the hospital and six months after aneurysmal subarachnoid hemorrhage. Additionally, National Institutes of Health Stroke Scale (NIHSS) was assessed 24 hours before, immediately before, immediately after, and 24 hours after endovascular vasodilator therapy, and at discharge and six months. Interventional parameters such as duration of intervention, choice and dosage of vasodilator and number of arteries treated were also recorded. Results: A total of 98 patients were included in this analysis and separated into patients who had interventions in conscious sedation, general anesthesia and a mix of both. Neither mRS at discharge nor at six months showed a significant difference for functionally independent outcomes (mRS 0-2) between groups. NIHSS before endovascular vasodilator therapy was significantly higher in patients receiving interventions in general anesthesia but did not differ anymore between groups six months after the initial bleed. Conclusion: This study did not observe a difference in outcome whether patients underwent endovascular vasodilator therapy in general anesthesia or conscious sedation for refractory cerebral vasospasms. Hence, the choice should be made for each patient individually.

Anesthesia modality does not affect clinical outcomes of intra-arterial vasodilator treatment in patients with symptomatic cerebral vasospasms

Background: Delayed cerebral ischemia and cerebral vasospasm remain the leading causes of poor outcome in survivors of aneurysmal subarachnoid hemorrhage. Refractory cerebral vasospasms can be treated with endovascular vasodilator therapy, which can either be performed in conscious sedation or general anesthesia. The aim of this study is to compare the effect of the anesthesia modality on long-term clinical outcomes in patients undergoing endovascular vasodilator therapy due to cerebral vasospasm and hypoperfusion. Methods: Modified Rankin Scale (mRS) scores were retrospectively analyzed at time of discharge from the hospital and six months after aneurysmal subarachnoid hemorrhage. Additionally, National Institutes of Health Stroke Scale (NIHSS) was assessed 24 hours before, immediately before, immediately after, and 24 hours after endovascular vasodilator therapy, and at discharge and six months. Interventional parameters such as duration of intervention, choice and dosage of vasodilator and number of arteries treated were also recorded. Results: A total of 98 patients were included in this analysis and separated into patients who had interventions in conscious sedation, general anesthesia and a mix of both. Neither mRS at discharge nor at six months showed a significant difference for functionally independent outcomes (mRS 0-2) between groups. NIHSS before endovascular vasodilator therapy was significantly higher in patients receiving interventions in general anesthesia but did not differ anymore between groups six months after the initial bleed. Conclusion: This study did not observe a difference in outcome whether patients underwent endovascular vasodilator therapy in general anesthesia or conscious sedation for refractory cerebral vasospasms. Hence, the choice should be made for each patient individually.

EXPRESS: Pulmonary vasodilator treatment in pulmonary hypertension due to left heart or lung disease: time for a high-definition picture?

Dear Editor, We read with great interest the article “Outcomes of pulmonary vasodilator use in Veterans with pulmonary hypertension associated with left heart disease and lung disease” by Gillmeyer et al. The study findings of increased risk of death or organ failure in patients exposed to pulmonary vasodilators, are consistent with findings from randomised clinical trials and other cohort studies and “real-world scenarios”, as quoted by the authors. However, a very important lesson from over two decades of studies is that proper phenotyping of pulmonary vascular disease is key to assess risk of progression of disease. As we progress in the study of these phenotypes, both in Group 2 and Group 3 PH, we might understand which mechanisms produce these subtle but clear differences in response to vasodilator treatment. We fully agree with the authors of the paper that the use of pulmonary vasodilators in Group 2 and Group 3 PH should be confined to randomised-controlled trials, not only in order to gather data on the numerous safety concerns, but also in order to generate new, reliable evidence. We also think that the use of registries will help garner more information on “real-world” scenarios and confirm on retrospective cohorts the results obtained in randomised-controlled trials, provided we are careful to study disease groups and subgroups appropriately, avoiding the temptation of lumping them together in a bigger cohort which will inevitably mixed pears with apples. Furthermore, in full agreement with the recommendations for future directions in research on Group 3 PH, we call for studies that delve deeper into these heterogeneous groups of diseases. After the low-definition group photos, we believe it is time to zoom in the picture to gather a better understanding of what exactly is killing the different subgroups within Group 2 and Group 3 PH patients.

Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties

Pulmonary veno-occlusive disease (PVOD) is a very infrequent form of pulmonary arterial hypertension with an aggressive clinical course, poor response to specific vasodilator treatment, and low survival. Confirming a definitive diagnosis is essential to guide treatment and assess lung transplantation. However, in the absence of histological or genetic confirmation, the diagnosis is complex, requiring a clinical suspicion. Multidetector computed tomography (MDCT) is an essential part of the non-invasive diagnostic tools of PVOD. We retrospectively reviewed the MDCT findings from a consecutive series of 25 patients diagnosed with PVOD, 9 with the sporadic form and 16 with the hereditary form of the disease. The presence and extent of typical findings of the diagnostic triad were assessed in all patients (ground glass parenchymal involvement, septal lines, and lymphadenopathy). In our series, 92% of patients showed at least two of the radiological findings described as typical of the disease. All patients presented at least one typical radiological characteristic. The incidence of radiological findings considered typical is very high, however was not associated with greater hemodynamic severity nor to the development of acute lung edema. No significant differences were found between the two groups. A poorly expressive MDCT does not exclude the disease.

Severe pulmonary arterial hypertension and massive ascites in a patient with systemic lupus erythematosus and secondary Sjogren’s syndrome

Background: Pulmonary arterial hypertension (PAH), is a rare manifestation of systemic lupus erythematosus (SLE), characterized by pulmonary arterial remodeling leading to right ventricular failure and death. To date, optimal management of SLE-associated PAH should be clarified, especially regarding the respective places of immunosuppressants and PAH vasodilator treatments. Case report: We report the case of a 48-year-old woman with SLE and secondary Sjogren syndrome, associated with severe PAH and lupus peritonitis with massive ascites, who showed a remarkable response, both for SLE flare and PAH, to a treatment combining immunosuppressants and pulmonary arterial vasodilator treatment. Conclusion: This observation highlights the interest of combining immunosuppressive therapy in SLE-PAH, whose modalities in association with PAH treatments should be clarified.

Factors Associated with Ineffectiveness of Sildenafil Treatment in Patients with End-Stage Heart Failure and Elevated Pulmonary Vascular Resistance

Introduction: Elevated pulmonary vascular resistance (PVR) unresponsive to vasodilator treatment is a marker of heart failure (HF) severity, and an important predictor of poor results of heart transplantation (HT). Objective: We sought to analyze factors associated with ineffectiveness of sildenafil treatment in end-stage HF patients with elevated PVR with particular emphasis placed on tenascin-C (TNC) serum concentrations. Patients and Methods: The study is an analysis of 132 end-stage HF patients referred for HT evaluation in the Cardiology Department between 2015 and 2018. TNC was measured by sandwich enzyme-linked immunosorbent assay (Human TNC, SunRedBio Technology, Shanghai, China). The endpoint was PVR > 3 Wood units after the six-month sildenafil therapy. Results: The median age was 58 years, and 90.2% were men. PVR >3 Wood units after 6 months of sildenafil treatment were found in 36.6% patients. The multivariable logistic regression analysis confirmed that TNC (OR = 1.004 (1.002–1.006), p = 0.0003), fibrinogen (OR= 1.019 (1.005–1.033), p = 0.085), creatinine (OR =1.025 (1.004–1.047), p = 0.0223) and right ventricular end-diastolic dimension (RVEDd) (OR = 1.279 (1.074–1.525), p = 0.0059) were independently associated with resistance to sildenafil treatment. Area under the ROC curves indicated an acceptable power of TNC (0.9680 (0.9444–0.9916)), fibrinogen (0.8187 (0.7456–0.8917)) and RVEDd (0.7577 (0.6723–0.8431)), as well as poor strength of creatinine (0.6025 (0.4981–0.7070)) for ineffectiveness of sildenafil treatment. Conclusions: Higher concentrations of TNC, fibrinogen and creatinine, as well as a larger RVEDd are independently associated with the ineffectiveness of sildenafil treatment. TNC has the strongest predictive power, sensitivity and specificity for evaluation of resistance to sildenafil treatment.

Different treatment protocols for moderate idiopathic sudden sensorineural hearing loss

Treatment of idiopathic sensorineural hearing loss (ISSNHL) is problematic due to the unclear etiology of the illness. Corticosteroid is recommended by some papers, and hyperbaric oxygen (HBO2) by others. Recently HBO2 has been shown to be an important therapy for ISSNHL, with an increasing number of studies demonstrating its beneficial results. Recovery from ISSNHL depends on the interval period between onset and treatment, hearing loss severity and audiogram type used to determine damage. Treatment of ISSNHL requires a detailed analysis. In this retrospective study we reviewed data from 56 patients with moderate ISSNHL. These patients were divided into three groups based on different treatments: corticosteroid group; corticosteroid + HBO2 (combination)group; and HBO2-only group. Additionally, all patients received intravenous vasodilator treatment. Hearing levels before and after treatment were compared. All three groups had a similar recovery rate, with an effective rate of more than 50%, and a hearing gain average of 17.38 decibels (dB). HBO2 treatment got a higher recovery rate. The combination therapy, which included corticosteroid and HBO2, did not elevate the recovery rate.