Desmoid tumour of the chest wall in paediatric post-operatory of heart transplant.

We will report a case of a desmoid tumour (DT), which developed at the surgical site of the pacemaker after a late childhood heart transplant. Patients with idiopathic dilated cardiomyopathy followed up in the paediatric cardiology service. It evolved with the dissociation of ventricular rhythm caused by severe heart failure, which led to the implantation of a cardiac resynchronization device prior to heart transplantation. The progression to end-stage heart disease culminated in a heart transplant at 12 years old. One year after the transplant, at the age of 13 years, he presented a progressively growing mass on the generator site of the resynchronization device. The initial decision was to remove the device. During the removal surgery, there was no haematoma or fluid collection. However, there was a progression of the lesion. The lesion was biopsied with the anatomopathological diagnosis of a DT. Resection surgery happened 4 months after the start of the mass growth. At that time, the tumour reached 20 cm in diameter. The lesion infiltrated the pectoralis major muscle and this muscle was resected partially en bloc with the lesion. The defect had primary closure. The patient evolved without postoperative complications and was discharged on the 14th postoperative day. The surgical specimen came with negative circumferential margins. However, the deep margin was microscopically positive. Due to deep involvement, the patient underwent adjuvant radiotherapy. Currently, the patient is under clinical follow-up and has no evidence of tumour recurrence. DT is a rare tumour, with unpredictable courses. Surgery can be considered in the progression of lesions. Treatment is justified by long survival after a heart transplant and in DT patients. DT is a differential diagnosis to be considered in progressive growth lesions.

Chronic thromboembolic pulmonary hypertension: the impact of advances in perioperative techniques in patient outcomes / Hipertensão pulmonar tromboembólica crônica: o impacto dos avanços nas técnicas perioperatórias nos resultados dos pacientes

ABSTRACT Objectives Pulmonary endarterectomy (PEA) is the gold standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed at reporting outcomes of CTEPH patients undergoing PEA within 10 years, focusing on advances in anesthetic and surgical techniques. Methods We evaluated 102 patients who underwent PEA between January 2007 and May 2016 at the Instituto do Coração do Hospital das Clínicas da Universidade de São Paulo. Changes in techniques included longer cardiopulmonary bypass, heating, and cooling times and mean time of deep hypothermic circulatory arrest and shortened reperfusion time. Patients were stratified according to temporal changes in anesthetic and surgical techniques: group 1 (January 2007-December 2012), group 2 (January 2013-March 2015), and group 3 (April 2015-May 2016). Clinical outcomes were any occurrence of complications during hospitalization. Results Groups 1, 2, and 3 included 38, 35, and 29 patients, respectively. Overall, 62.8% were women (mean age, 49.1 years), and 65.7% were in New York Heart Association functional class III-IV. Postoperative complications were less frequent in group 3 than in groups 1 and 2: surgical complications (10.3% vs. 34.2% vs. 31.4%, p=0.035), bleeding (10.3% vs. 31.5% vs. 25.7%, p=0.047), and stroke (0 vs. 13.2% vs. 0, p=0.01). Between 3 and 6 months post-discharge, 85% were in NYHA class I-II. Conclusion Improvements in anesthetic and surgical procedures were associated with better outcomes in CTEPH patients undergoing PEA during the 10-year period.

RESUMO Objetivo A endarterectomia pulmonar (EAP) é o tratamento padrão ouro para hipertensão pulmonar tromboembólica crônica (HPTEC). O objetivo deste estudo foi relatar a evolução de pacientes com HPTEC submetidos a EAP em 10 anos, com foco nos avanços nas técnicas anestésicas e cirúrgicas. Métodos Foram avaliados 102 pacientes submetidos à EAP entre janeiro de 2007 e maio de 2016 no Instituto do Coração do Hospital das Clínicas da Universidade de São Paulo. Mudanças nas técnicas incluíram circulação extracorpórea, tempos de aquecimento e resfriamento mais longos e tempo médio de hipotermia profunda com parada circulatória e tempo de reperfusão reduzido. Os pacientes foram estratificados de acordo com as mudanças temporais nas técnicas anestésicas e cirúrgicas: grupo 1 (janeiro de 2007 a dezembro de 2012), grupo 2 (janeiro de 2013 a março de 2015) e grupo 3 (abril de 2015 a maio de 2016). Os desfechos clínicos foram qualquer ocorrência de complicações durante a hospitalização. Resultados Os grupos 1, 2 e 3 incluíram 38, 35 e 29 pacientes, respectivamente. No geral, 62,8% eram mulheres (idade média, 49,1 anos) e 65,7% estavam em classe funcional III-IV da New York Heart Association. As complicações pós-operatórias foram menos frequentes no grupo 3 do que nos grupos 1 e 2: complicações cirúrgicas (10,3% vs. 34,2% vs. 31,4%, p=0,035), sangramento (10,3% vs. 31,5% vs. 25,7%, p=0,047) e acidente vascular cerebral (0 vs. 13,2% vs. 0, p=0,01). Entre 3 e 6 meses após a alta, 85% estavam na classe I-II da NYHA. Conclusão Melhorias nos procedimentos anestésicos e cirúrgicos foram associadas a melhores resultados em pacientes com HPTEC submetidos a EAP durante o período de 10 anos.

Shear stress-exposed pulmonary artery endothelial cells fail to upregulate HSP70 in chronic thromboembolic pulmonary hypertension.

The pathophysiological mechanisms underlying chronic thromboembolic pulmonary hypertension (CTEPH) are still unclear. Endothelial cell (EC) remodeling is believed to contribute to this pulmonary disease triggered by thrombus and hemodynamic forces disbalance. Recently, we showed that HSP70 levels decrease by proatherogenic shear stress. Molecular chaperones play a major role in proteostasis in neurological, cancer and inflammatory/ infectious diseases. To shed light on microvascular responses in CTEPH, we characterized the expression of molecular chaperones and annexin A2, a component of the fibrinolytic system. There is no animal model that reproduces microvascular changes in CTEPH, and this fact led us to isolated endothelial cells from patients with CTEPH undergoing pulmonary endarterectomy (PEA). We exposed CTEPH-EC and control human pulmonary endothelial cells (HPAEC) to high- (15 dynes/cm2) or low- (5 dynes/cm2) shear stress. After high-magnitude shear stress HPAEC upregulated heat shock protein 70kDa (HSP70) and the HSP ER paralogs 78 and 94kDa glucose-regulated protein (GRP78 and 94), whereas CTEPH-ECs failed to exhibit this response. At static conditions, both HSP70 and HSP90 families in CTEPH-EC are decreased. Importantly, immunohistochemistry analysis showed that HSP70 expression was downregulated in vivo, and annexin A2 was upregulated. Interestingly, wound healing and angiogenesis assays revealed that HSP70 inhibition with VER-155008 further impaired CTEPH-EC migratory responses. These results implicate HSP70 as a novel master regulator of endothelial dysfunction in type 4 PH. Overall, we first show that global failure of HSP upregulation is a hallmark of CTEPH pathogenesis and propose HSP70 as a potential biomarker of this condition.

Tromboendarterectomia na hipertensão pulmonar tromboembólica / Thromboendarterectomy on thromboembolic pulmonary hypertension

Após a tromboembolia pulmonar, cerca de 1% a 3% dos pacientes desenvolvem embolismo crônico, seguido de elevação da pressão da artéria pulmonar e aumento da resistência vascular pulmonar. O aumento contínuo da pressão vascular leva ao remodelamento das pequenas artérias. Em pacientes selecionados, a tromboendarterectomia pulmonar é o procedimento de escolha para o tratamento da hipertensão pulmonar tromboembólica, com redução significativa da pressão pulmonar e melhora hemodinâmica. A morbimortalidade dessa cirurgia vem diminuindo progressivamente, especialmente nos centros com maior número de procedimentos. A melhora da qualidade de vida é evidente e grande parte dos pacientes retorna às suas atividades habituais...

After pulmonary embolism, 1% to 3% of these patients develop a chronic embolism, with elevated pulmonary artery pressure and increased pulmonary vascular resistance. The continuous elevated pressure leads to the remodeling of the small arteries. In selected patients, pulmonary thromboendarterectomy is the procedure of choice for the treatment of thromboembolic pulmonary hypertension, with significant reduction in pulmonary pressure and hemodynamic improvement.The morbidity and mortality of this surgery has been decreasing steadily, especially in centers with larger numbers of procedures. The improvement in quality of life is evident and most patients return to their usual activities...