Interarm Blood Pressure Difference Revealing a Right-Sided Aortic Arch and Occluded Aberrant Left Subclavian Artery.

We present the case of a 33-year-old patient with a rare combination of a right-sided aortic arch and occluded asymptomatic aberrant left subclavian artery diagnosed after the coincidental finding of an interarm blood pressure difference. Because there were no symptoms of local compression or subclavian steal, conservative management was suggested.

Peripheral Microvascular Dysfunction in Children and Adults with Congenital Heart Disease: A Literature Review.

Although there is a continually growing number of patients with congenital heart disease (CHD) due to medical and surgical advances, these patients still have a poorer prognosis compared to healthy individuals of similar age. In patients with heart failure, microvascular dysfunction (MVD) has recently emerged as a crucial modulator of disease initiation and progression. Because of the substantial pathophysiological overlap between CHD and heart failure induced by other etiologies, MVD could be important in the pathophysiology of CHD as well. MVD is believed to be a systemic disease and may be manifested in several vascular beds. This review will focus on what is currently known about MVD in the peripheral vasculature in CHD. Therefore, a search on the direct assessment of the vasodilatory capacity of the peripheral microcirculation in patients with CHD was conducted in the PubMed database. Since there is little data available and the reported studies are also very heterogeneous, peripheral MVD in CHD is not sufficiently understood to date. Its exact extent and pathophysiological relevance remain to be elucidated in further research.

Elevated oxidative stress in patients with congenital heart disease and the effect of cyanosis: a meta-analysis.

Oxidative stress is an important pathophysiological mechanism in the development of numerous cardiovascular disorders. To improve therapy and preventive strategies, clinicians need a better understanding of the underlying pathophysiological mechanisms of congenital heart diseases (CHD). The objective of this meta-analysis was to determine whether oxidative stress is elevated in patients with CHD compared to healthy controls, and to evaluate whether a difference in oxidative stress parameters can be observed between patients with cyanotic (cCHD) and acyanotic CHD (aCHD). Therefore, 21 studies investigating oxidative stress in peripheral blood of both children and adults with CHD were reviewed. Different methods to assess the oxidant status were compared and divided into three categories: pro-oxidative or anti-oxidative stress markers and the ratio of pro-to-anti oxidative stress markers. This meta-analysis showed elevated oxidative stress levels in patients with CHD, and more specifically in patients with cCHD. Moreover, this indicates that there could be potential in oxidative stress measurements as a new biomarker of disease severity. Further research will be needed to clarify the exact role of oxidative stress and its contributors in CHD in order to get a better and more in-depth understanding of the underlying pathophysiology of CHD, especially the higher susceptibility of the right ventricle (RV) to progress to heart failure (HF). This could facilitate the development of antioxidant treatments and RV-specific HF therapies, which are necessary to improve survival in these patients and could be of particular importance in cCHD.

Coronary Microvascular Dysfunction in Patients with Congenital Heart Disease.

Congenital heart diseases represent a wide range of cardiac malformations. Medical and surgical advances have dramatically increased the survival of patients with congenital heart disease, leading to a continuously growing number of children, adolescents, and adults with congenital heart disease. Nevertheless, congenital heart disease patients have a worse prognosis compared to healthy individuals of similar age. There is substantial overlap in the pathophysiology of congenital heart disease and heart failure induced by other etiologies. Among the pathophysiological changes in heart failure, coronary microvascular dysfunction has recently emerged as a crucial modulator of disease initiation and progression. Similarly, coronary microvascular dysfunction could be important in the pathophysiology of congenital heart diseases as well. For this systematic review, studies on maximal vasodilatory capacity in the coronary microvascular bed in patients with congenital heart disease were searched using the PubMed database. To date, coronary microvascular dysfunction in congenital heart disease patients is incompletely understood because studies on this topic are rare and heterogeneous. The prevalence, extent, and pathophysiological relevance of coronary microvascular dysfunction in congenital heart diseases remain to be elucidated. Herein, we discuss what is currently known about coronary microvascular dysfunction in congenital heart disease and future directions.

Circulating Reactive Oxygen Species in Adults with Congenital Heart Disease.

Oxidative stress is an important pathophysiological mechanism in the development of numerous cardiovascular disorders, but few studies have examined the levels of oxidative stress in adults with congenital heart disease (CHD). The objective of this study was to investigate oxidative stress levels in adults with CHD and the association with inflammation, exercise capacity and endothelial function. To this end, 36 adults with different types of CHD and 36 age- and gender-matched healthy controls were enrolled. Blood cell counts, hs-CRP, NT-proBNP, fasting glucose, cholesterol levels, iron saturation and folic acid concentrations were determined in venous blood samples. Levels of superoxide anion radical in whole blood were determined using electron paramagnetic resonance spectroscopy in combination with the spin probe CMH. Physical activity was assessed with the IPAQ-SF questionnaire. Vascular function assessment (EndoPAT) and cardiopulmonary exercise testing were performed in the patient group. Superoxide anion radical levels were not statistically significantly different between adults with CHD and the matched controls. Moreover, oxidative stress did not correlate with inflammation, or with endothelial function or cardiorespiratory fitness in CHD; however, a significant negative correlation with iron saturation was observed. Overall, whole blood superoxide anion radical levels in adults with CHD were not elevated, but iron levels seem to play a more important role in oxidative stress mechanisms in CHD than in healthy controls. More research will be needed to improve our understanding of the underlying pathophysiology of CHD.

Clostridium perfringens splenic abscess treated with percutaneous drainage and antibiotic therapy: a case report.

INTRODUCTION: Splenic abscesses are a rare medical entity with high mortality, with Clostridium perfringens being an exceptional pathogen. We present the first case describing an abscess of the spleen in a patient undergoing treatment with adalimumab, an anti-tumour necrosis factor commonly used in inflammatory bowel diseases. PATIENT: We describe the case of a 73-year-old woman, treated with adalimumab therapy, who was found to have a splenic abscess, caused by C. perfringens. RESULT: After antibiotic treatment and percutaneous drainage, the patient recovered both clinically and radiographically. DISCUSSION: We reviewed literature about C. perfringens splenic abscesses. In all cases identified, a splenectomy was performed. This is the first case description of splenic abscess caused by C. perfringens where a percutaneous drainage was performed, leading to complete recovery in our patient. This seems to indicate that a splenectomy is not necessary in all C. perfringens splenic abscesses.

The Diagnostic Challenge of Extrapulmonary Tuberculosis: Esophageal Perforation, Mediastinitis, Pneumopericardium, and Pericardial Abscess Formation.

We describe the case of extrapulmonary tuberculosis complicated by esophageal perforation, pneumopericardium, and pericardial abscess formation. This case illustrates the difficulty in diagnosing extrapulmonary tuberculosis, as the occurrence of tuberculosis is rare in the developed world. The appropriate treatment strategy and 6-month follow-up results are discussed. (Level of Difficulty: Advanced.).

Spontaneous Bone Regeneration After Closure of the Hard Palate Cleft: A Literature Review.

PURPOSE: A wide range of surgical techniques have been described for the treatment of palatal clefts. Some of these surgical procedures result in postoperative osteogenesis at the palatal fissure. The aims of this review were to discuss the current approaches to cleft palate surgery leading to spontaneous bone regeneration and to compare these different procedures. Moreover, the causes of bone regeneration, effects on maxillary growth, and factors affecting bone regeneration on the hard palate are discussed. MATERIALS AND METHODS: The selected articles were found via MEDLINE and Web of Science. The keywords for the search were "cleft palate," "bone regeneration," "palatoplasty," "reconstructive surgical procedures," and "cleft palate surgery." Studies that examined the effect of primary palatoplasty on spontaneous bone regeneration in the hard palate in children were included in this review. Four articles were analyzed in the qualitative synthesis. RESULTS: Because of differences in patient characteristics and evaluation methods, it was difficult to compare different surgical procedures. The use of a mucoperiosteal flap in combination with adequate closure of the mucosa is needed to obtain bone formation. The area with the largest amount of regenerated bone was located in the middle of the hard palate. In the literature, it was found that complete closure was considered unfavorable because of the negative effects on maxillary growth, but more studies are needed to confirm this. Of the factors that have been studied, only age turned out to be borderline relevant. CONCLUSIONS: Only a few studies with small sample sizes have been published on bone regeneration in the hard palate. More research is needed to validate these findings.