Pulmonary valve replacement in tetralogy of Fallot - who and how?

BACKGROUND AND AIM: Pulmonary regurgitation is the most common complication in repaired tetralogy of Fallot patients. Severe chronic pulmonary regurgitation can be tolerated for decades, but if not treated, it can progress to symptomatic, irreversible right ventricular dilatation and dysfunction. We investigated clinical associations with pulmonary valve replacement among patients with significative pulmonary regurgitation and how interventional developments can change their management. METHODS: All adult patients with repaired tetralogy of Fallot who were followed at an adult CHD Clinic at a single centre from 1980 to 2022 were included on their first outpatient visit. Follow-up was estimated from the time of correction surgery until one of the following events occurred first: pulmonary valve replacement, death, loss to follow-up or conclusion of the study. RESULTS: We included 221 patients (116 males) with a median age of 19 (18-25). At a median age of 33 (10) years old, 114 (51%) patients presented significant pulmonary regurgitation. Among patients with significant pulmonary regurgitation, pulmonary valve replacement was associated with male gender, older age at surgical repair, and longer QRS duration in adulthood. Pulmonary valve replacement was performed in 50 patients, including four transcatheter pulmonary valve implantations, at a median age of 34 (14) years. CONCLUSION: Pulmonary regurgitation affects a large percentage of tetralogy of Fallot adult patients, requiring a long-term clinical and imaging follow-up. Sex, age at surgical repair and longer QRS are associated with the need of PVR among patients with significative pulmonary regurgitation. Clinical practice and current literature support TPVI as the future gold standard intervention.

New Evidence on BPA's Role in Adipose Tissue Development of Proinflammatory Processes and Its Relationship with Obesity.

Bisphenol A (BPA) is a xenobiotic with endocrine disruptor properties which interacts with various receptors, eliciting a cellular response. In the plastic industry, BPA is widely used in the production of polycarbonate and epoxy-phenolic resins to provide elastic properties. It can be found in the lining of canned foods, certain plastic containers, thermal printing papers, composite dental fillings, and medical devices, among other things. Therefore, it is a compound that, directly or indirectly, is in daily contact with the human organism. BPA is postulated to be a factor responsible for the global epidemic of obesity and non-communicable chronic diseases, belonging to the obesogenic and diabetogenic group of compounds. Hence, this endocrine disruptor may be responsible for the development of metabolic disorders, promoting in fat cells an increase in proinflammatory pathways and upregulating the expression and release of certain cytokines, such as IL6, IL1ß, and TNFα. These, in turn, at a systemic and local level, are associated with a chronic low-grade inflammatory state, which allows the perpetuation of the typical physiological complications of obesity.

The role of the hepatic factor on the improvement of arterial oxygenation after a Kawashima procedure: A case report.

BACKGROUND: Anomalies of the systemic venous return to the heart are infrequent, occurring more often in patients with congenital heart disease. CASE REPORT: We present a patient with heterotaxy, univentricular circulation, and hemiazygos return who underwent a Kawashima procedure at 3 years of age. Fourteen years later, she developed increased cyanosis and tiredness. The symptoms clearly improved after an off-pump derivation of the hepatic drainage from the common atrium to the hemiazygos system. CONCLUSIONS: This rare case report addresses the surgical challenges imposed by patients with complex congenital heart disease combined with anomalies in systemic venous return. Although improvement in arterial oxygenation after redirecting hepatic flow to the hemiazygos system in patients previously submitted to a Kawashima procedure has already been reported, the existence of a hepatic factor remains to be recognized.

Mobilization of an anomalous retroaortic coronary artery to prevent myocardial ischemia after aortic valve replacement.

Abnormal distribution may put coronary arteries at risk of damage during valvular surgery, especially if it is not identified during a preoperative examination. In this case report, we describe an approach to an anomalous right coronary artery during aortic valve replacement.

Rare cause of dynamic aortic obstruction in pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries: a case report.

A 2-month-old infant with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries developed an aneurysmatic elongation of the tricuspid valve tissue that partially closed and dynamically protruded through the ventricular septal defect, beneath the aortic valve. This rare finding caused dynamic left ventricle outflow tract obstruction and recurrent cardiac arrests and ultimately required surgical intervention.

An Update on Connexin Gap Junction and Hemichannels in Diabetic Retinopathy.

Diabetic retinopathy (DR) is one of the main causes of vision loss in the working age population. It is characterized by a progressive deterioration of the retinal microvasculature, caused by long-term metabolic alterations inherent to diabetes, leading to a progressive loss of retinal integrity and function. The mammalian retina presents an orderly layered structure that executes initial but complex visual processing and analysis. Gap junction channels (GJC) forming electrical synapses are present in each retinal layer and contribute to the communication between different cell types. In addition, connexin hemichannels (HCs) have emerged as relevant players that influence diverse physiological and pathological processes in the retina. This article highlights the impact of diabetic conditions on GJC and HCs physiology and their involvement in DR pathogenesis. Microvascular damage and concomitant loss of endothelial cells and pericytes are related to alterations in gap junction intercellular communication (GJIC) and decreased connexin 43 (Cx43) expression. On the other hand, it has been shown that the expression and activity of HCs are upregulated in DR, becoming a key element in the establishment of proinflammatory conditions that emerge during hyperglycemia. Hence, novel connexin HCs blockers or drugs to enhance GJIC are promising tools for the development of pharmacological interventions for diabetic retinopathy, and initial in vitro and in vivo studies have shown favorable results in this regard.

New Insights on the Role of Connexins and Gap Junctions Channels in Adipose Tissue and Obesity.

Due to the inability to curb the excessive increase in the prevalence of obesity and overweight, it is necessary to comprehend in more detail the factors involved in the pathophysiology and to appreciate more clearly the biochemical and molecular mechanisms of obesity. Thus, understanding the biological regulation of adipose tissue is of fundamental relevance. Connexin, a protein that forms intercellular membrane channels of gap junctions and unopposed hemichannels, plays a key role in adipogenesis and in the maintenance of adipose tissue homeostasis. The expression and function of Connexin 43 (Cx43) during the different stages of the adipogenesis are differentially regulated. Moreover, it has been shown that cell-cell communication decreases dramatically upon differentiation into adipocytes. Furthermore, inhibition of Cx43 degradation or constitutive overexpression of Cx43 blocks adipocyte differentiation. In the first events of adipogenesis, the connexin is highly phosphorylated, which is likely associated with enhanced Gap Junction (GJ) communication. In an intermediate state of adipocyte differentiation, Cx43 phosphorylation decreases, as it is displaced from the membrane and degraded through the proteasome; thus, Cx43 total protein is reduced. Cx is involved in cardiac disease as well as in obesity-related cardiovascular diseases. Different studies suggest that obesity together with a high-fat diet are related to the production of remodeling factors associated with expression and distribution of Cx43 in the atrium.

Cardiothoracic surgery during the Covid-19 pandemic: Perioperative care, safety, and surgical results.

BACKGROUND: Covid-19 was declared a pandemic by the World Health Organization (WHO) on 11 March 2020. Responses to this crisis integrated resource allocation for the increased amount of infected patients while maintaining an adequate response to other severe and life-threatening diseases. Though cardiothoracic patients are at high risk for Covid-19 severe illness, postponing surgeries would translate to increased mortality and morbidity. We reviewed our practice during the initial time of the pandemic, with emphasis on safety protocols. METHODS: From 11 March to 15 May 2020, 148 patients underwent surgery at the Department of Cardiothoracic Surgery of Centro Hospitalar Universitário de São João. The clinical characteristics of the patients were retrospectively registered, along with novel containment and infection prevention measures targeting the new coronavirus. RESULTS: The majority of adult cardiac patients were operated on an emergency basis. Hospital mortality was 1.9% (n = 2 patients). Most adult thoracic patients were admitted from home, with a diagnosis of neoplastic disease in 60% of patients. Hospital mortality was 3.3% (1 patient). Fifteen children underwent cardiothoracic surgery. There was no mortality. The infection prevention procedures applied, totally excluded the transmission of Covid-19 in the department. CONCLUSION: While guaranteeing a prompt response to emergent, urgent, and high priority cases, novel safety measures in individual protection, patients circuits, and preoperative diagnoses of symptomatic and asymptomatic infection were adopted. The surgical results corroborate that it was safe to undergo cardiothoracic surgery during the initial time of the Covid-19 pandemic. The new policies will be maintained while the virus stays in the community.

Congenital systemic venous drainage obstruction: A case report.

BACKGROUND: Vena cava anomalies are rare congenital defects due to incorrect development during fetal life, ranging from minor asymptomatic anatomic variations to complex life-threatening abnormalities. Echocardiography plays a fundamental role in the diagnosis, with advanced imaging techniques allowing detailed anatomic delineation. Invasive cardiology techniques are a promising therapeutic approach, but surgery is probably the best option when diffuse compromise of the systemic veins is present. CASE REPORT: An 8-month-old infant presenting episodes of labial cyanosis and a failure to thrive was diagnosed with severe superior vena cava (SVC) stenosis at the right atrium ostium, with decompression via azygos vein and mild inferior vena cava (IVC) stenosis. The patient was referred for surgery, with IVC enlargement and removal of a fibromuscular tissue band on the anterior aspect of the SVC ostium. At a 6-month follow-up, the patient is asymptomatic, with excellent surgical outcome. To the authors' knowledge, only two cases of congenital SVC stenosis have been previously described.

Randomization of Pulse Phases for Unambiguous and Robust Quantum Sensing.

We develop theoretically and demonstrate experimentally a universal dynamical decoupling method for robust quantum sensing with unambiguous signal identification. Our method uses randomization of control pulses to simultaneously suppress two types of errors in the measured spectra that would otherwise lead to false signal identification. These are spurious responses due to finite-width π pulses, as well as signal distortion caused by π pulse imperfections. For the cases of nanoscale nuclear-spin sensing and ac magnetometry, we benchmark the performance of the protocol with a single nitrogen vacancy center in diamond against widely used nonrandomized pulse sequences. Our method is general and can be combined with existing multipulse quantum sensing sequences to enhance their performance.

Lung Hernia Related with a Rope Bullfight: Case Report.

Lung herniation is an uncommon entity which was fully classified in 1845 after the study of several case reports. Acquired lung hernia, especially traumatic, is the most common etiology. In the absence of clear guidelines, management of lung hernia is made in a case-by-case basis. We present an asymptomatic middle lobe hernia perceptible on physical examination, but diagnosed initially by imaging studies. Patient medical history included a blunt bull trauma fourteen years before.

Pulmonary Sequestration Supplied By The Circumflex Artery - A Rare Case Report.

Pulmonary sequestration (PS) is a rare congenital malformation, even more when its arterial supply is a coronary artery. We present a case of a 68-year-old man admitted in the emergency room with an acute coronary syndrome and no evidence of significant coronary disease. Instead, he had an abnormal branch from the circumflex coronary artery nourishing a mass in the left lower pulmonary lobe. A coronary steal phenomenon was proposed to explain the clinical presentation. An anterior left thoracotomy with ligation of the abnormal branch and atypical resection of the lung segment comprising the sequestration was performed.

Probing the Dynamics of a Superradiant Quantum Phase Transition with a Single Trapped Ion.

We demonstrate that the quantum phase transition (QPT) of the Rabi model and critical dynamics near the QPT can be probed in the setup of a single trapped ion. We first demonstrate that there exists equilibrium and nonequilibrium scaling functions of the Rabi model by finding a proper rescaling of the system parameters and observables, and show that those scaling functions are representative of the universality class to which the Rabi model belongs. We then propose a scheme that can faithfully realize the Rabi model in the limit of a large ratio of the effective atomic transition frequency to the oscillator frequency using a single trapped ion and, therefore, the QPT. It is demonstrated that the predicted universal functions can indeed be observed based on our scheme. Finally, the effects of realistic noise sources on probing the universal functions in experiments are examined.

Connexinopathies: a structural and functional glimpse.

Mutations in human connexin (Cx) genes have been related to diseases, which we termed connexinopathies. Such hereditary disorders include nonsyndromic or syndromic deafness (Cx26, Cx30), Charcot Marie Tooth disease (Cx32), occulodentodigital dysplasia and cardiopathies (Cx43), and cataracts (Cx46, Cx50). Despite the clinical phenotypes of connexinopathies have been well documented, their pathogenic molecular determinants remain elusive. The purpose of this work is to identify common/uncommon patterns in channels function among Cx mutations linked to human diseases. To this end, we compiled and discussed the effect of mutations associated to Cx26, Cx32, Cx43, and Cx50 over gap junction channels and hemichannels, highlighting the function of the structural channel domains in which mutations are located and their possible role affecting oligomerization, gating and perm/selectivity processes.

Survival and quality of life after aortic coarctation repair.

INTRODUCTION: Coarctation of the aorta is the fifth most common congenital heart disease in clinical practice. Surgical repair has been performed in infants with high survival; still patients maintain a high risk of morbidity and mortality, moreover in the presence of associated cardiac lesions. Thus, it is imperative a medical follow-up of these patients in order to understand the impact of the disease in their quality of life. OBJECTIVES: To report the surgical results, long term survival, complications and present health related quality of life of patients submitted to surgical repair of aortic coarctation. METHODS: Retrospective review of all patients younger than 1 year old submitted to surgery, between 1998 and 2005, at Centro Hospitalar São João. A survey was answered by the patients to address present quality of life. RESULTS: Fifty patients were available for study. Twenty patients (40,0%) were neonates at the time of surgery and 31 (62%) presented to urgent repair. Isolated coarctation was noticed in 25 patients (50,0%) and the most common surgical technique was resection and end-to-end anastomosis, in 64,0%. Early mortality was 4,0% and zero in patients with isolated coarctation. Actuarial survival rates were 90,0%, 88,0% and 88,0% at 5, 10 and 17 years of follow-up, respectively. Complex coarctation was associated with decreased survival (p = 0,007). Patients reported in questionnaires similar results to healthy population. CONCLUSION: Surgical repair of aortic coarctation is a relatively safe surgery, however patients maintain risks of arterial hypertension, re-coarctation and mortality at long term. Nevertheless, patients report a quality of life that is no different than healthy population.

[Cyanotic congenital heart diseases duct dependent: Stent versus surgical shunt. Meta-analysis of observational studies]. / Doenças Cardíacas Congénitas Cianóticas Dependentes De Ducto: Stent Versus Shunt cirúrgico. Meta-análise De Estudos Observacionais.

BACKGROUND: The modified Blalock-Taussig shunt (mBTS) is the method of choice in the congenital heart disease duct dependent. More recently, the percutaneous implantation of a stent in the duct (PIS) has emerged as an alternative to the surgery. OBJECTIVE: To determine the most safe and effective procedure by reading the available literature. METHODS: A systematic review of observational studies was perfomed by searching Pubmed and SciVerse/Scopus, in which neonates were submitted to mBTS or PIS. Two independent reviewers selected the articles according to the inclusion criteria. The mortality rate was defined as the primary outcome. Complications and re-interventions were defined as secondary outcomes. RESULTS: Twenty-three of 176 articles were included (n = 2243 patients, 1920 submitted to mBTS and 323 to PIS). The weighted average mortality rate of the neonates who underwent mBTS was 7,6% (95% CI: 6,5%-8,9 %) and submitted to IPS was 8.3% (95% CI: 4,6%-14,7%; p=0,7006). The weighted average rate of complications and re- interventions, weight and size of the stent and shunt did not show statistically significant differences. It was not possible to establish a relationship between these and mortality rate. Significant heterogeneity was detected for the mortality rate in the group undergoing PIS due to the different sample sizes, non-uniform patient selection and results with large confidence intervals. CONCLUSIONS: We could not find systematic evidence to suggest that PIS is safer and more advantageous than the realization of a BTSm in these pathologies.

[Carcinoid syndrome. A clinical report]. / Síndrome Carcinóide: Caso Clínico.

The carcinoid syndrome is a rare clinical entity, coursing with a slow evolution. Patients typically present flush, diarrhea and acute asthma attacks, which often delays the diagnostic until there is cardiac involvement, with more pronounced symptoms. We report the clinical case of a 62 years-old male, with a known significant tricuspid valve disorder. While waiting for cardiac surgery, he presented with an acute abdominal condition, which allowed the detection of a carcinoid syndrome. He underwent surgical excision of the primary tumor (right hemicolectomy), removal of hepatic metastases, and tricuspid valve replacement.