Clinical Characteristics and Outcomes in Adults With Moderate-to-Severe Complexity Congenital Heart Disease Undergoing Palliation or Surgical Repair.

Background: Congenital heart disease (CHD) survival rate has improved dramatically due to advances in diagnostic and therapeutic techniques. However, concerning the unrepaired CHD population of moderate and severe complexity, the data regarding risk predictors and surgical outcomes are scarce. Our aim was to describe the surgical results and predictors of in-hospital outcomes in adult patients with moderate-to-severe complexity CHD that were not repaired in childhood. Methods: We conducted a retrospective cohort study that included 49 adult patients with moderate-to-complex CHD who were treated in a single medical centre. Clinical and echocardiographic variables were obtained on admission, after surgical procedures and during follow-up. Results: Most of the patients were female (66%). Left ventricular ejection fraction and right ventricular outflow tract fractional shortening were within the normal range. The median pulmonary artery systolic pressure was 37 (27-55) mm Hg. The median time was 118 (80-181) minutes for extracorporeal circulation and 76 (49-121) minutes for aortic cross-clamping. The most frequent complication was postoperative complete atrioventricular block (12.2%). In-hospital survival rate was 87.7%. The development of low cardiac output syndrome with predominant right ventricle failure in the postoperative period was the most important predictor of in-hospital death (P = 0.03). Conclusions: Deciding to treat adults with CHD is challenging in moderate and severe unrepaired cases. Adequate clinical, functional, and imaging evaluation is essential to determine each patient's suitability for surgical management and to achieve the best clinical outcome for this population.

Contexte: Grâce aux avancées réalisées en matière de techniques diagnostiques et thérapeutiques, la survie des patients atteints d'une cardiopathie congénitale s'est considérablement améliorée. Cependant, en ce qui concerne les personnes atteintes d'une cardiopathie congénitale non corrigée présentant une complexité modérée ou extrême, les données portant sur les facteurs de risque prédictifs ainsi que sur les résultats chirurgicaux sont rares. Notre objectif était de décrire les résultats chirurgicaux ainsi que les facteurs prédictifs des résultats obtenus en milieu hospitalier chez les patients adultes atteints d'une cardiopathie congénitale présentant une complexité modérée ou extrême qui n'a pas été corrigée pendant l'enfance. Méthodologie: Nous avons mené une étude de cohorte rétrospective comprenant 49 patients adultes atteints d'une cardiopathie congénitale modérée ou complexe qui ont reçu leurs traitements dans un seul centre médical. Les variables cliniques et échocardiographiques ont été obtenues au moment de l'admission, après les interventions chirurgicales et pendant la période de suivi. Résultats: Les patients étaient en majorité des femmes (66 %). La fraction d'éjection du ventricule gauche ainsi que la fraction de raccourcissement de la voie d'éjection ventriculaire droite sont demeurées dans les limites de la normale. La pression systolique médiane de l'artère pulmonaire a été de 37 mmHg (27-55 mmHg). Le temps médian écoulé pour la circulation extracorporelle a été de 118 minutes (80-181 minutes) et pour le clampage de la crosse aortique, de 76 minutes (49-121 minutes). Le bloc auriculo-ventriculaire postopératoire complet a été la complication la plus fréquente (12,2 %). Le taux de survie en milieu hospitalier a été de 87,7 %. Le développement du syndrome du faible débit cardiaque accompagné d'une insuffisance prédominante du ventricule droit durant la période postopératoire a constitué le principal facteur prédictif de décès à l'hôpital (p = 0,03). Conclusion: Il est difficile de traiter les adultes qui présentent une cardiopathie congénitale modérée ou sévère non corrigée. Il est essentiel que les évaluations cliniques, fonctionnelles et par imagerie soient réalisées de façon adéquate pour déterminer si une prise en charge chirurgicale convient aux patients et pour garantir les meilleurs résultats cliniques chez ces derniers.

Adjuvant Effect of Molecular Iodine in Conventional Chemotherapy for Breast Cancer. Randomized Pilot Study.

This study analyzes an oral supplement of molecular iodine (I2), alone and in combination with the neoadjuvant therapy 5-fluorouracil/epirubicin/cyclophosphamide or taxotere/epirubicin (FEC/TE) in women with Early (stage II) and Advanced (stage III) breast cancer. In the Early group, 30 women were treated with I2 (5 mg/day) or placebo (colored water) for 7-35 days before surgery. For the Advanced group, 30 patients received I2 or placebo, along with FEC/TE treatment. After surgery, all patients received FEC/TE + I2 for 170 days. I2 supplementation showed a significant attenuation of the side effects and an absence of tumor chemoresistance. The control, I2, FEC/TE, and FEC/TE + I2 groups exhibited response rates of 0, 33%, 73%, and 100%, respectively, and a pathologic complete response of 18%, and 36% in the last two groups. Five-year disease-free survival rate was significantly higher in patients treated with the I2 supplement before and after surgery compared to those receiving the supplement only after surgery (82% versus 46%). I2-treated tumors exhibit less invasive potential, and significant increases in apoptosis, estrogen receptor expression, and immune cell infiltration. Transcriptomic analysis indicated activation of the antitumoral immune response. The results led us to register a phase III clinical trial to analyze chemotherapy + I2 treatment for advanced breast cancer.

Anomalía de Ebstein en un paciente con síndrome de Down. Reporte de un caso / Ebstein's anomaly in a patient with Down syndrome. Case report

Resumen Paciente con síndrome de Down referido a nuestro departamento con cianosis y soplo cardíaco. Un ecocardiograma transtorácico mostró anomalía de Ebstein. Esta asociación es extremadamente rara. Se inició manejo médico ya que la lesión en la válvula tricúspide era leve. De acuerdo con nuestra revisión, se han descrito únicamente 12 casos en literatura médica.

Abstract An infant with Down's syndrome was referred to our department with cyanosis and heart murmur. A transthoracic echocardiogram demonstrated the presence of Ebstein's anomaly. This association is extremely unusual. Medical management was initiated since the tricuspid valve lesion was mild. Only twelve cases, to our knowledge, have been previously reported.

Trombofilia hereditaria y trombo intracardíaco / Hereditary thrombophilia and intracardiac thrombus

Resumen La trombofilia es una condición hematológica que predispone a eventos tromboembólicos venosos y arteriales. Es un factor que predispone a trombos intracardíacos en la edad pediátrica y se debe sospechar si no hay otros factores que predispongan a trombosis. Se reporta el caso de una paciente con antecedente de trombosis venosa cerebral a quien se le diagnosticó trombofilia por mutación homocigota G202010A del gen de la protrombina. Desarrolló un trombo auricular derecho, el cual fue tratado con anticoagulación y vigilancia ecocardiográfica. Se resalta la importancia de realizar una evaluación ecocardiográfica en pacientes con trombofilia.

Abstract Thrombophilia is a haematological condition that predisposes to venous and arterial thromboembolic events. It is also a predisposing factor in intracardiac thrombi in paediatrics, and must be suspected if there are no other factors that predispose to thrombosis. The case is presented on a patient with a history cerebral venous thrombosis, who was diagnosed with thrombophilia due to a homozygote G202010A mutation of the prothrombin gene. She developed a right atrial thrombus, which was treated with anticoagulation and echocardiography monitoring. The importance of performing an echocardiography evaluation is highlighted in patients with thrombophilia.

[Kommerell's Diverticulum (KD)]. / Divertículo de Kommerell (DK).

Right aortic arch with aberrant left subclavian artery from a Kommerell's diverticulum is a very rare variant of the incomplete vascular ring. Associated symptoms are caused due to tracheal or esophagus compression. Magnetic resonance is the gold standard for diagnosis. Surgical treatment is recommended for symptomatic patients or asymptomatic patients with a large diverticulum. We report three consecutive cases of patients with Kommerell's diverticulum, aberrant left subclavian artery, and right-sided aortic arch.

[Anomalous origin of the right pulmonary artery from the ascending aorta associated with aortopulmonary window]. / Origen anómalo de la rama derecha de la arteria pulmonar de la aorta ascendente asociado con ventana aortopulmonar.

Anomalous origin of one pulmonary artery from the aorta is rare. We report a case of a three-month-old infant with aortopulmonary window and anomalous origin of the right pulmonary artery from the ascending aorta. He underwent surgery with anastomosis of the right pulmonary artery, ligation of the aortopulmonary window and the patent duct. He was released under medical treatment and had no signs of pulmonary hypertension or heart failure.

[Evaluation of the results of percutaneous closure by means of the Amplatzer device in pediatric patients with intra-auricular communication (IAC)]. / Evaluación de los resultados del cierre percutáneo mediante el dispositivo Amplatzer en pacientes pediátricos con comunicación interauricular (CIA).

OBJECTIVE: To describe the results of percutaneous closure by means of the Amplatzer Septal Occluder (ASO) device in pediatric patients with intra-auricular communication (IAC). MATERIALS AND METHODS: Descriptive transversal study in patients submitted to percutaneous closure of IAC from March 2005 to March 2013. Patients aged <16 years, weight>6 kg, IAC type ostium segundum, and patent foramen ovale, with border separations borders>5 mm, and absence of coexisting pathology were included in the study. We excluded from the study children with severe pulmonary blood pressure/arterial hypertension. Descriptive statistics with the SPSS v. 20.0 statistical software package. RESULTS: We included in the study 28 patients, feminine gender (n=19, 68%), median age=8 years (range, 4-14), weight 30.7 kg (range, 15-69). New York Heart Association (NYHA) functional class I (n=21, 75%), II (n=7, 25%). Median IAC size, 15.50 mm (range, 5-25), and a median ASO size of 17.54 mm (range, 8-28). After ASO placement, 100% presented NYHA I at one month, cardiac murmur (n=2, 7.1%), cessation of cardiac murmur at month 6 (n=28, 100%), without evidence of arrhythmias at one month 100%, residual short circuit at 24 hours (n=4, 14%), complete occlusion at month 6 (n=28, 100%), normalization size of VD, and cessation of tricuspid insufficiency 100% at one year. Complications included minimal bleeding during the procedure (n=2, 7%), transitory cephalea (n=5, 18%), and dysautonomia (n=1, 4%). CONCLUSION: Percutaneous closure of IAC of children fitted with the ASO device is safe and exhibits good results.