[Hypoplastic left heart syndrome: 10 year experience with staged surgical management]. / Síndrome de hipoplasia de corazón izquierdo: experiencia de 10 años de un programa de etapificación quirúrgica.

UNLABELLED: Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country. OBJECTIVES: 1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality. PATIENTS AND METHOD: Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed. RESULTS: Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤2mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P=.05). Actuarial survival was 64% at one year, and 57% at 5years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality. CONCLUSIONS: Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.

Poverty, quality of life and psychological wellbeing in adults with congenital heart disease in Chile.

The objective of this study was to assess the quality of life and psychological wellbeing of adults with congenital heart disease (CHD) in Chile, and to identify other associated factors. The study enrolled 68 patients aged between 18 and 72 (median 29), 35 being females. They completed a questionnaire, which included a quality of life assessment tool (the Medical Outcome Study 36-Item Short Form Health Survey), a number of psychological scales (the General Health Questionnaire, the Basic Psychological Needs Scales and the Beck Hopelessness Scale), a socioeconomic survey, and some clinical data. CHD patients reported worse scores in those scales assessing physical dimensions of quality of life (physical function (70.5), physical role functioning (64), vitality (65.3)), and general quality of life (58.6), than in emotional or social dimensions. Female gender was associated with lower scores in physical function (59.12 versus 82.66; p<0.01) and physical role functioning (53.68 versus 75; p<0.05); poverty was associated with worse results in physical function (61.92 versus 82.96; p<0.01), role physical (53.21 versus 79.63; p<0.01), vitality (60.89 versus 71.67; p<0.05), social role functioning (70.19 versus 82.87; p<0.05) and bodily pain (65.77 versus 81.2; p<0.05). Furthermore, we found that psychological scales had an association with quality of life, but clinical variables did not show significant correlations to any dimension. Poverty has an impact on the quality of life of CHD patients. This population only has a decrease in the quality of life physical dimensions, suggesting that quality of life depends on many different factors.

Effectiveness of asthma education with and without a self-management plan in hospitalized children.

Background. Formal education in primary care can reduce asthma exacerbations. However, there are few studies in hospitalized children, with none originating in Latin America. Methods. A prospective randomized study was designed to evaluate whether a full education with self-management plan (ESM) was more effective than an education without self-management plan (E) in reducing asthma hospitalization. Children (5 to 15 years of age) who were hospitalized for an asthma attack were divided in two groups. Children in the E group received general instructions based on a booklet. Those in the ESM group received the same booklet plus a self-management guide and a puzzle game that reinforces the lessons learned in the booklet. Patients were interviewed every 3 months, by telephone, for one year. Interviewers recording the number of hospitalizations, exacerbations, and emergency visits for asthma and oral steroid burst uses. Results. From 88 children who met the inclusion criteria, 77 (86%) completed one year of follow-up (41 from E and 36 from ESM group). Overall, after one year, the hospitalization decreased by 66% and the inhaled corticosteroids therapy increased from 36% to 79%. At the end of the study, there was no difference in exacerbations, emergency visits, oral steroid burst uses, or hospitalizations between the two groups. Conclusions. Asthma education with or without a self-management plan during asthma hospitalization were effective in reducing exacerbations, emergency visits, oral steroid burst uses, and future rehospitalizations. This evidence supports the importance of providing a complete asthma education plan in any patient who is admitted for asthma exacerbation.

Síndrome de hipoplasia de corazón izquierdo: experiencia de 10 años de un programa de etapificación quirúrgica / Hypoplastic left heart syndrome: 10 year experience with staged surgical management

Resumen El síndrome de hipoplasia de corazón izquierdo (SHCI) es una cardiopatía congénita con letalidad superior al 95%. La etapificación quirúrgica es la principal vía de tratamiento, y se inicia con la operación de Norwood; la sobrevida a largo plazo de los pacientes tratados es desconocida en nuestro medio. Objetivos 1) Revisar nuestra experiencia en el manejo de todos los pacientes con SHCI evaluados entre enero 2000 y junio 2010. 2) Identificar factores de riesgo de mortalidad quirúrgica. Pacientes y método Estudio retrospectivo de una única institución con una cohorte de pacientes con SHCI. Se revisan antecedentes clínicos, quirúrgicos, y registros de seguimiento. Resultados Se evaluaron 76 pacientes con SHCI; 9/76 tenían comunicación interauricular (CIA) restrictiva, y 8/76, aorta ascendente de < 2 mm; 65/76 fueron tratados: 77% tuvieron operación de Norwood con conducto entre ventrículo derecho y ramas pulmonares como fuente de flujo pulmonar, 17% Norwood con shunt de Blalock-Taussig, y 6% otra cirugía. La mortalidad en la primera etapa quirúrgica fue del 23%, y en operación de Norwood, del 21,3%. En el período 2000-2005 la mortalidad en la primera etapa quirúrgica fue del 36%, y entre 2005-2010, del 15% (p = 0,05). La sobrevida global fue del 64% a un año y del 57% a 5 años. Por análisis multivariado fueron factores de riesgo para mortalidad la presencia de aorta ascendente diminuta y CIA restrictiva. Conclusiones Nuestros resultados inmediatos y a largo plazo en la etapificación quirúrgica de SHCI son similares a la experiencia de grandes centros. Hay una mejoría en mortalidad operatoria en la segunda mitad de la serie. Se identifican factores de riesgo de mortalidad.

Abstract Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country. Objectives 1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality. Patients and method Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed. Results Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤ 2 mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P = .05). Actuarial survival was 64% at one year, and 57% at 5 years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality. Conclusions Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.

Comparison of chemical immobilization methods in wild foxes (Pseudalopex griseus and Pseudalopex culpaeus) in Chile.

We immobilized individuals of two species of free-ranging South American foxes, including 28 chilla foxes (Pseudalopex griseus; 13 males and 15 females) and five culpeo foxes (Pseudalopex culpaeus; four males and one female). Animals were trapped and chemically immobilized with ketamine and medetominide (K-M), ketamine and xylazine (K-X), or tiletamine-zolazepam (Z). Heart and respiratory rates, hemoglobin oxygen saturation (SpO2), rectal temperature, and palpebral and anal reflexes were measured at 5-min intervals. Data were analyzed to compare the effect of anesthetic combinations on induction and recovery times, body reflexes, and physiological variables over time. In both species, K-M gave the shortest induction time, followed by K-X and Z. Palpebral and anal reflexes in chilla foxes immobilized with K-M were maintained in more animals than those treated with either K-X or Z. Animals immobilized with Z had higher heart and respiratory rates than those immobilized with either of the other two combinations. Rectal temperature decreased over time for all combinations. Foxes immobilized with K-M maintained a higher SpO2 than those immobilized with either K-X or with Z. All anesthetic combinations were satisfactory in inducing rapid and safe immobilization of the species studied. The anesthetic plane and the effects on physiologic parameters were better in animals immobilized with K-M than with either K-X or Z, and we recommend this anesthetic combination for use in Chilean foxes. Nevertheless, all three drug combinations used were satisfactory in inducing rapid and relatively smooth anesthesia.

Role of reactive oxygen species in bradykinin-induced proliferation of vascular smooth muscle cells.

In addition to the induction of cell proliferation and migration, bradykinin (BK) can increase c-fos mRNA expression, activate ERK 1/2 and generate reactive oxygen species (ROS) in vascular smooth muscle cells (VSMC). It is not known, however, whether BK can induce cellular proliferation and extracellular matrix production via redox-sensitive signaling pathways. We investigated the role(s) of ROS in proliferation, migration and collagen synthesis induced by BK in VSMC derived from Sprague Dawley rat aorta. BK (10 nM) increased VSMC proliferation by 30% (n=5); this proliferation was inhibited by the antioxidants N-acetylcysteine (20 mM) and alpha-lipoic acid (LA, 250 mM). In addition, BK induced an increase in cell migration and in collagen levels that were blocked by LA. ROS production induced by BK (n=10) was significantly inhibited by bisindolylmaleimide (4microM) and by PD98059 (40microM). These results suggest that: 1) ROS participate in the mechanism(s) used by bradykinin to induce cellular proliferation; 2) bradykinin induces ROS generation through a pathway that involves the kinases PKC and MEK; and 3) ROS participate in the pathways mediating cell migration and the production of collagen as a response to treatment with bradykinin. To our knowledge, this is the first report describing mechanisms to explain the participation of ROS in the cellular proliferation and extracellular matrix pathway regulated by BK.

Role of reactive oxygen species in bradykinin-induced proliferation of vascular smooth muscle cells

In addition to the induction of cell proliferation and migration, bradykinin (BK) can increase c-fos mRNA expression, activate ERK 1/2 and generate reactive oxygen species (ROS) in vascular smooth muscle cells (VSMC). It is not known, however, whether BK can induce cellular proliferation and extracellular matrix production via redox-sensitive signaling pathways. We investigated the role(s) of ROS in proliferation, migration and collagen synthesis induced by BK in VSMC derived from Sprague Dawley rat aorta. BK (10 nM) increased VSMC proliferation by 30 % (n=5); this proliferation was inhibited by the antioxidants N-acetylcysteine (20 mM) and a-lipoic acid (LA, 250 mM). In addition, BK induced an increase in cell migration and in collagen levels that were blocked by LA. ROS production induced by BK (n=10) was significantly inhibited by bisindolylmaleimide (4mM) and by PD98059 (40mM). These results suggest that: 1) ROS participate in the mechanism(s) used by bradykinin to induce cellular proliferation; 2) bradykinin induces ROS generation through a pathway that involves the kinases PKC and MEK; and 3) ROS participate in the pathways mediating cell migration and the production of collagen as a response to treatment with bradykinin. To our knowledge, this is the first report describing mechanisms to explain the participation of ROS in the cellular proliferation and extracellular matrix pathway regulated by BK.