Right Ventricular
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2021 ◽  
pp. 1-6
Ergin Arslanoglu ◽  
Kenan Abdurrahman Kara ◽  
Fatih Yigit ◽  
Cüneyt Arkan ◽  
Esra Ozcan ◽  

Abstract Pulmonary artery aneurysms are rare. They are characterised by an aneurysmatic dilatation of the pulmonary vascular bed, including the main pulmonary artery or the accompanying pulmonary artery branches. Increases in pulmonary flow and pulmonary artery pressure increase the risk of rupture: when these conditions are detected, surgical intervention is necessary. This study is a retrospective analysis of 33 patients treated in our paediatric cardiac surgery clinic from 2012 to 2020. Aneurysms and pseudoaneurysms in patients who were patched for right ventricular outflow tract reconstruction and corrected with a conduit were excluded from the study. Seventeen (51.5%) of the patients included in the study were female and 16 (48.5%) were male. The patients were aged between 23 and 61 years (mean 30.66 ± 12.72 years). Graft interpositions were performed in 10 patients (30.3%) and pulmonary artery plications were performed in 23 patients (69.7%) to repair aneurysms. There was no significant difference in mortality between the two groups (p > 0.05). Pulmonary artery aneurysm interventions are safe, life-saving treatments that prevent fatal complications such as ruptures, but at present there is no clear guidance regarding surgical timing or treatment strategies. Pulmonary artery interventions should be performed in symptomatic patients with dilations ≥5 cm or asymptomatic patients with dilations ≥8 cm; pulmonary artery pressure, right ventricular systolic pressure, and pulmonary artery aneurysm diameter must be considered when planning surgeries, their timing, and making decisions regarding indications. Experienced surgical teams can achieve satisfactory results using one of the following surgical techniques: reduction pulmonary arterioplasty, plication, or graft replacement.

2021 ◽  
Vol 9 ◽  
Anneline S. J. M. te Riele ◽  
Cynthia A. James ◽  
Hugh Calkins ◽  
Adalena Tsatsopoulou

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by fibrofatty infiltration of predominantly the right ventricular (RV) myocardium. Affected patients typically present as young adults with hemodynamically stable ventricular tachycardia, although pediatric cases are increasingly recognized. These young subjects often have a more severe phenotype with a high risk of sudden cardiac death (SCD) and progression toward heart failure. Diagnosis of ARVC is made by combining multiple sources of information as prescribed by the consensus-based Task Force Criteria. The description of Naxos disease, a fully penetrant autosomal recessive disorder that is associated with ARVC and a cutaneous phenotype of palmoplantar keratoderma and wooly hair facilitated the identification of the genetic cause of ARVC. At present, approximately 60% of patients are found to carry a pathogenic variant in one of five genes associated with the cardiac desmosome. The incomplete penetrance and variable expressivity of these variants however implies an important role for environmental factors, of which participation in endurance exercise is a strong risk factor. Since there currently is no definite cure for ARVC, disease management is directed toward symptom reduction, delay of disease progression, and prevention of SCD. This clinically focused review describes the spectrum of ARVC among children and adolescents, the genetic architecture underlying this disease, the cardio-cutaneous syndromes that led to its identification, and current diagnostic and therapeutic strategies in pediatric ARVC subjects.

Onur Sinan Deveci ◽  
Caglar Ozmen ◽  
Muhammet Bugra Karaaslan ◽  
Aziz Inan Celik ◽  
Hatice Rahimova ◽  

Objective Vaso-occlusive crisis (VOC) is a common clinical manifestation of sickle cell anemia (SCA) and is associated with increased proinflammatory mediators. Copeptin is the C-terminal part of the prohormone for pro-vasopressin and seems clinically relevant in various clinical conditions. Right ventricular (RV) dysfunction significantly appears in SCA patients due to pulmonary hypertension. This study aimed to investigate the association of copeptin levels in VOC patients and evaluate RV dysfunction. Materials and Methods A total of 108 patients were enrolled in the study. Twenty-eight SCA patients in steady state (30.2±10.9 years), 25 SCA patients in VOC (36.8±11.8 years), and 55 healthy individuals (31.9±9.4 years) with HbAA genotype were included. Clinical, echocardiographic, and laboratory data were recorded. ELISA was used for the determination of serum levels of copeptin. Results VOC patients had significantly higher copeptin level compared both with controls and SCA subjects in steady-state (22.6±13.0 vs. 11.3±5.7 pmol/l, 22.6±13.0 vs. 12.4±5.8 pmol/l, p=0.009 for both). Additionally, the copeptin level was significantly higher in SCA patients with RV dysfunction than those without RV dysfunction (23.2±12.2 vs. 15.3±9.5 pmol/l, p=0.024). Multiple logistic regression analysis revealed that hs-CRP and copeptin levels were found to be associated with VOC. Conclusion The study showed that copeptin and hs-CRP levels were increased in patients with VOC, and a significant relationship was found between RV dysfunction in VOC patients. As a conclusion copeptin can be used as a potential biomarker in predicting VOC crisis in SCA patients and in early detection of patients with SCA who have the potential to develop RV dysfunction.

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