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ABSTRACT IMPACT: This work represents a novel way in which genetic information can be used to improve clinical decision making as it pertains to both treatment and management of congenital heart disease. OBJECTIVES/GOALS: Our lab found that MYH6 variants are both enriched in hypoplastic left heart syndrome (HLHS) and associated with decreased cardiac transplant-free survival. To elucidate the mechanisms of MYH6 variant pathogenicity, we are assessing their impact on atrial function during HLHS development and progression. METHODS/STUDY POPULATION: We are using 2D speckle-based tracking to retrospectively evaluate echocardiograms (echos) from 51 HLHS patients, 17 with MYH6 variants and 34 matched controls. Atrial function will be assessed by myocardial strain and strain rate at seven time points, beginning at the time of the patients’ earliest prenatal echo, and ending with their last available echo before death or cardiac transplant. Early left atrial function will examine the role of MYH6 variants in the development of HLHS in vivo, while longitudinal right atrial function will be assessed in order to look for differences that could be contributing to the decreased transplant-free survival seen in MYH6 variant carriers. RESULTS/ANTICIPATED RESULTS: We hypothesize that MYH6 variants cause HLHS by impairing early left atrial (LA) contractility, resulting in altered left ventricular hemodynamics and consequent hypoplasia. We therefore expect to find diminished prenatal LA function in HLHS patients with MYH6 variants. We also hypothesize that MYH6 variants continue to impair right atrial (RA) function in surgically-reconstructed HLHS hearts, necessitating earlier transplantation. Accordingly, we expect variant carriers to exhibit lower RA function at birth versus controls. We expect differences between groups to persist over time, and possibly increase in magnitude. In HLHS patients with MYH6 variants, we anticipate declining RA function will precede right ventricular function and therefore be an early indicator of transplant need. DISCUSSION/SIGNIFICANCE OF FINDINGS: This study represents a novel way in which genetic information can inform clinical decision-making. Identifying MYH6 variants as an early cause of HLHS offers chances for intervention. Understanding long-term effects of MYH6 on right atrial function in HLHS may aid in cardiac transplant risk stratification, thus improving patient outcomes.
The Second Republic sparked considerable enthusiasm concerning the possibilities that a large-scale permanent redistribution of landed property could resolve the social problems in southern Spain. Yet, as this chapter argues, land reform failed because there was insufficient uncultivated land that could be brought under the plough, and labour-intensive agriculture was not feasible under dry-farming conditions. Indeed, cereal cultivation was becoming increasingly capital intensive, especially on the heavy, fertile Campiña soils. The slow and limited progress of settlements under the 1932 Reform Law contrasts with the land invasions in the spring of 1936, which resulted in over a hundred thousand peasants receiving almost immediately over half a million hectares. However they failed to solve the overriding problem of insufficient land and, because weak state capacity implied that land settlements could not be implemented impartially, they simply changed which authority decided who was to benefit, and who was to be excluded.
This book has argued that it was weak state capacity and the absence of competitive mass political parties that the Second Republic inherited which severely limited the possibilities of its success. As Juan Linz has noted, weak state capacity can create problems of efficacy, or the ability of governments to find adequate solutions to resolve basic demands, as well as a lack of effectiveness in policy implementation. Land reform is an excellent example because it failed to resolve the problems of the landless, leading to frustration among a large section of rural society, while providing a platform for an even larger group to organize against the Republic.
Unlike most Western European countries, Spain’s landed elites and Church hierarchy remained politically and economically strong before the Great Depression. Spanish farm lobbies therefore continued to reflect the interests of large landowners and cereal farmers, whose demand for higher tariffs led to an expansion in wheat cultivation, despite the country’s weak competitive advantage. By contrast, family farmers, despite representing around a third of the electorate, were politically under-represented, which would have important consequences for the democratic experiment during the Second Republic. Neither the landed elites nor the Church hierarchy were required to participate in mass political parties before 1931, which limited their interest in organizing village-level cooperatives into federations, and at the same time helped them to preserve their traditional powers. Catalonia was the exception, as regional political demands required the landed elites to participate in mass politics, and explains the success of local farm cooperatives and rural associations.
Although traditional Spanish farming was slowly being replaced by a modern, scientific agriculture dependent on industrial inputs, productivity remained low and living standards for many were poor and precarious during the first third of the twentieth century. Social problems were contained while surplus farm labour could easily find employment in the cities, but the limits of traditional agriculture to create employment became brutally exposed during the 1930s Depression. Unlike Northern Europe, the use of dry-farming techniques were required over much of the country, which created major obstacles to increasing output by employing more labour. The growing possibilities for mechanization by the interwar period offered benefits to the large cereal farms, but threatened to make small farmers, with their highly fragmented holdings, uncompetitive. Technological change also threatened to eliminate a significant source of seasonal employment for landless labourers, and force marginal cereal producers to sell their land.
This chapter looks at the nature and extent of collective action in rural areas, and the difficulties associated with creating credit and producer cooperatives. It shows that the traditional village economy provided a wide variety of public goods, and the persistence of the pósitos, or village grain banks, suggests an ability to organize and resolve problems of collective action that extended over most of the country, and not just the North, as assumed in much of the literature. However, although the village pósito met the needs of a traditional, organic-based farming system, it was inadequate for an agriculture that was becoming increasingly dependent on industrial inputs. In particular, it was the inability to create an organizational structure that could extend collective action from the village to the regional and national levels, and attract savings from a wide geographic area to meet the growing needs of the small farmers that helps explain the persistence of paternalist relations in the countryside. The chapter finishes by providing a background to the changing nature of Spain’s farm organizations over the half century prior to the Second Republic.
This chapter challenges contemporary beliefs that latifundios were inefficient by showing that farmers were quick to respond to changes in factor and commodity prices. Land ownership in southern Spain was heavily concentrated, both on the rich cereal lands of the Guadalquivir valley, as well as the huge dehesas found in the less populated upland regions. Contemporaries believed that large numbers of landless workers lived in extreme poverty at the same time as absentee landowners left significant areas of fertile land abandoned, or under-cultivated. In fact, large farms by the late 1920s were especially suitable for extensive cereals and livestock given the growing possibilities for reducing labour costs through mechanization, and there were difficulties to extend labour-intensive olive and vine cultivation. The chapter also shows that the living standards of rural workers improved over time, although they were vulnerable to economic downturns because of the erosion of traditional safety nets, and the failure of the state to create new ones. Finally, it examines why large landowners were often uninterested in extending state capacity.