Lupine Publishers | Hypertrophic Cardiomiopathy in Children: The Need of Heart Transplantation

Lupine Publishers | Advancements in Cardiovascular Research

Abstract

Hypertrophic cardiomyopathy (HCM) is the most common cardiac disease affecting the cardiac muscle. It can manifest in different forms with or without left ventricular outflow obstruction, with or without right ventricle involvement. Forms with biventricular hypertrophy seem to have poor prognosis. In our case, we describe a young patient with sarcomeric biventricular hypertrophic cardiomyopathy (MYH7 mutation), the poor prognosis of this form and strategies options adopted after failure of medical treatment. It is not always easy the management of hypertrophic cardiomiopathy, after medical treatment failure, especially in children. In some cases, heart transplantation is the only one therapeutic option.

Keywords: Hypertrophic Cardiomiopathy; Right Ventricular Hypertrophy; Heart Transplantation

Introduction

Hypertrophic cardiomiopathy (HCM) is the most common cardiac disease affecting the cardiac muscle and is characterized by heterogeneous genetic, morphological, functional, and clinical features. It is also one of the main causes of sudden cardiac death (SDC) in the young. Left ventricular hypertrophy with left ventricular outflow obstruction (LVOTO) is the most characteristic feature of HCM. There are also variant of HCM without LVOTO, with apical hypertrophy, with medio-ventricular obstruction and with right ventricular hypertrophy. The treatment and the prognosis of HCM seem to be variable on the basis of different forms, the age at presentation, sarcomeric gene mutations or rare phenocopies. Heart transplantation (HT) is the only therapeutic option for selected patients with HCM and refractory heart failure. In effect ESC guidelines recommend heart transplantation in eligible patients who have an LVEF < 50% and NYHA functional Class III–IV symptoms despite optimal medical therapy or intractable ventricular arrhythmia (II a); in eligible patients with normal LVEF (50%) and severe drug refractory symptoms (NYHA functional Class III–IV) caused by diastolic dysfunction (II b)[1].

Right ventricular hypertrophy (SRVH) is a relatively rare subtype of HCM. The anatomic, genetic, clinical, and prognostic characteristics of patients with SRVH and the clinical relevance of these characteristics have not been described widely in the literature [2,3]. MYBPC3 gene mutations have previously been described in two patients with RV hypertrophy. In a recent study, 90% of HCM patients with SRVH were found to possess relevant sarcomere protein mutations and variations in the MYH7 (Myosin heavy chain 7) and TTN genes, followed by variations in MYBPC3. Always in this study 73% of HCM patients with SRVH and multiple sarcomere gene mutations had poor prognosis. 7 In addiction MYH7 mutations can cause hypertrophic cardiomyopathy or skeletal myopathies with or without cardiac involvement, on the basis of the side of mutation. In our case, we describe the poor prognosis and treatment strategies of a young patient with biventricular hypertrophic cardiomyopathy and MYH7 mutation.

Case Report

A 12-year-old young woman with familiarity for hypertrophic cardiomyopathy (mother and mother’s twin with biventricular hypertrophic cardiomiopathy and MYH7 mutation) was hospitalized in our hospital for dyspnea after mild-moderate efforts and reduced functional capacity (NYHA Class II). Mother and aunt of the patient were asymptomatic with good functional capacity. Patient had the same genetic mutation of mother and aunt (p.Asn696Ser heterozygosis MYH7) but with increased and poor phenotypic expression [4]. Echocardiography and cardiac magnetic resonance were performed showing a hypertrophic cardiomyopathy with right ventricular involvement. Precisely, cardiovascular imaging showed left ventricle asymmetric hypertrophy especially at the level of anterior and inferior wall (basal and mild anterior wall =14 mm, z score= 3,5; antero-lateral basal wall = 12 mm, z score 2,78; mild inferior wall = 14 mm and apical inferior wall = 12 mm) with normal ejection fraction (FE = 62% at CMR) and moderate diastolic dysfunction (panel B and D). In addiction wall thickness of right ventricle outflow and basal-mild free wall were increased (= 13 mm) with apical obstruction and development of maximum gradient of 10 mmHg (PANEL A and C) [5,6] (Figure 1).

Figure 1.

The function of right ventricle was at inferior limits (FE = 51% at CMR, TAPSE = 16 mm at echocardiography). Thus the patient had an interesting right ventricle involvement and moderate diastolic dysfunction of left ventricle. She had not arrhythmia at ECG-Holter but she had reduced functional capacity. also demonstrated at stress test. Stress test was suspended at 6 min (Bruce Protocol) after pre-syncopal symptoms: lack of adaptation of the blood pressure to the effort was observed. In addition, from several months she had pre-syncopal episodes at the peak of the effort. ECG showed left ventricular hypertrophy and biatrial enlargement. Pro BNP was increased = 5841 pg/ml. Considering clinical situation, we decided to start medical treatment with betablockers (bisoprolol) but the patient didn’t tolerate medical treatment. Thus, we decided to start low dose of captopril without improvement of symptomatology. Also, treatment with diuretic was not tolerate by patient [7,8]. Therefore, considering symptom refractory to medical therapy, the poor prognosis and the impossibility to optimize medical treatment, we decided to plan cardiac transplantation, the only option possible at this moment.

Thus right catheterization was performed and patient was inserted in heart transplantation list. ICD implantation was not considered in the absence of ventricular arrhythmia and other factors. Discussion: hypertrophic cardiomyopathy associated with MYH7 mutation and right ventricle involvement seems to have poor prognosis, especially if right ventricle hypertrophy is severe [9]. In effect the young patient had a greater right ventricular hypertrophy compared than mother and aunt. In these cases, after medical treatment failure, heart transplantation seems to be the only strategy to improve symptomatology and quality of the life of the patient. Especially in pediatric population, it is not always easy the management of hypertrophic cardiomiopathy after medical treatment failure and heart transplantation seems to be the only one therapeutic option. Other study are needed to study some variants of HCM with right ventricle hypertrophy, their treatment and prognosis.

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Lupine Publishers | Brackish Water Desalination Using Solar Desalination Pannel

 Lupine Publishers | Current Investigations in Agriculture and Current Research

Abstract

The origin and continuation of mankind is based on water. Water is one of the most abundant resources on earth, covering three-fourths of the planet’s surface. Water is one of the earth’s most abundant resources, covering about three-quarters of the planet’s surface. The reason for this apparent contradiction is, of course, that - 97.5% of the earth’s water is salt water in the oceans and only 2.5% is fresh water in ground water, lakes and rivers and this supplies most human and animal needs. It would be feasible to address the water-shortage problem with seawater desalination; however, the separation of salts from seawater requires large amounts of energy which, when produced from fossil fuels, can cause harm to the environment. Therefore, there is a need to employ environmentally-friendly energy sources in order to desalinate seawater. Solar distillation systems are increasingly attractive in the areas suffering from shortages of fresh water but where solar energy is plentiful and where operational and maintenance costs are low.

Introduction

Water is essential for life. Many countries around the world, especially developing countries and countries in the Middle East region, suffer from a shortage of fresh water. The United Nations (UN) Environment Programme stated that one-third of the world’s population lives in countries with insufficient freshwater to support the population [1]. Consequently, drinking water of acceptable quality has become a scarce commodity. The total global water reserves are _1.4 billion km3, of which around 97.5% is in the oceans and the remaining 2.5% is fresh water present in the atmosphere, ice, mountains and ground water. Of the total, only _0.014% is directly available for human beings and other organisms [2]. Thus, tremendous efforts are now required to make available new water resources in order to reduce the water deficit in countries which have shortages [3]. According to World Health Organization (WHO) guidelines, the permissible limit of salinity in drinking water is 500ppm and for special cases up to 1000ppm [4]. Most of the water available on the earth has a salinity up to 10000ppm and seawater normally has salinity in the range of 35 000-45 000ppm in the form of total dissolved salts [5]. Desalination is a process in which saline water is separated into two parts, one that has a low concentration of dissolved salts, which is called fresh water, and the other which has a much higher concentration of dissolved salts than the original feed water, which is usually referred to as brine concentrate [6]. The desalination of seawater has become one of the most important commercial processes to provide fresh water for many communities and industrial sectors which play a crucial role in socioeconomic development in a number of developing countries, especially in Africa and some countries in the Middle East region, which suffer from a scarcity of fresh water.

There is extensive R&D activity, especially in the field of renewable energy technologies, to find new and feasible methods to produce drinking water [7,8]. Currently, there are more than 7500desalination plants in operation worldwide producing several billion gallons of water per day. Fifty-seven per cent are in the Middle East [9] where large-scale conventional heat and power plants are among the region’s most important commercial processes, they play a crucial role in providing fresh water for many communal and industrial sectors, especially in areas with a high density of population. However, since they are operated with fossil fuel, they are becoming very expensive to run and the environmental pollution they produce is increasingly recognized as very harmful to the globe. Moreover, such plants are not economically viable in remote areas, even near a coast where seawater is abundant. Many such areas often also experience a shortage of fossil fuels and an inadequate electricity supply. The development of compact, smallscale systems for water desalination is imperative for the population in such areas [7,8]. Thermal solar energy water desalination is known to be a viable method of producing fresh water from saline water [2] in remote locations; conventional basin solar stills with a relatively large footprint are an example of such simple technology. And using a clean natural energy resource in water desalination processes will significantly reduce the pollution that causes global warming. This article aims to present a review of the published literature on the various desalination technologies and their advantages and disadvantages in addition to their economics.

Water and energy are two inseparable commodities that govern the lives of humanity and promote civilization. The history of mankind proves that water and civilization are two inseparable entities. This is proved by the fact that all great civilizations were developed and flourished near large sources of water. Rivers, seas, oases, and oceans have attracted mankind to their coasts because water is the source of life. History proves the importance of water in the sustainability of life and the development of civilization. Maybe the most significant example of this influence is the Nile River in Egypt. The river provided water for irrigation and mud full of nutrients. Ancient Egyptian engineers were able to master the river water and Egypt, as an agricultural nation, became the main wheat exporting country in the whole Mediterranean Basin [10]. Energy is as important as water for the development of good standards of life because it is the force that puts in operation all human activities. Water is also itself a power generating force. The first confirmed attempts to harness waterpower occurred more than 2000 years ago in which time the energy gained was mainly used to grind grain [11]. The Greeks were the first to express philosophical ideas about the nature of water and energy. Thales of Militus (640-546BC), one of the seven wise men of antiquity wrote about water [12,13] that it is fertile and moulded (can take the shape of its container). The same philosopher said that seawater is the immense sea that surrounds the earth, which is the primary mother of all life. Later on, Embedokles (495-435BC) developed the theory of the elements [12] describing that the world consists of four primary elements: fire, air, water and earth. These with today’s knowledge may be translated to: energy, atmosphere, water and soil, which are the four basic constituents that affect the quality of our lives [14].

Aristotle (384-322), who is one of the greatest philosophers and scientists of antiquity, described in a surprisingly correct way the origin and properties of natural, brackish and seawater. He wrote for the water cycle in nature [15]: “Now the sun moving, as it does, sets up processes of change and becoming and decay, and by its agency the finest and sweetest water is every day carried out and is dissolved into vapor and rises to the upper regions, where it is condensed again by the cold and so returns to the earth. This, as we have said before, is the regular cycle of nature.” Even today no better explanation is given for the water cycle in nature. Really, the water cycle is a huge solar energy open distiller in a perpetual operational cycle. For the seawater Aristotle wrote [16]: “Salt water when it turns into vapor becomes sweet, and the vapor does not form salt water when it condenses again. This is known by experiment.”Water demand and consumption.

Materials and Methods

The following are the description of solar powered water desalination system AROCELL solar water purifies Australian technology. It only use sun energy, there are no moving parts, no electronics. It is robust and easy to setup, low maintenance and very low in operating cost because the water purifier only need solar energy. The feed water is supplied by gravity or pressure pump CAROCELL direct solar powered desalination technology, working at ambient temperature, heats the input water causing vapors condensation change precluding all bacteria and pathogens, therefore eliminating all water borne diseases Exposure to ultra violet light and extreme heat from solar energy through the advanced composite panels enhances the germ killing process. CAROCELL’s increased efficiency (65% with peak efficiencies above 80%) over other solar distillation products (30-40%) is a combination of the proprietary materials used to dramatically increase the temperature of the feed water on the solar collector which enhances the evaporation/condensation processes inside the panel. Additionally, this sophisticated geometrical design has easy maintenance, optimum performance and a self-controlling natural convection loop enabling widely superior energy recovery (Figure 1). Under this study 04 solar desalination units were installed at National Agricultural Research Centre, Islamabad, Pakistan to check the efficiency of cleaning the brackish water through desalination solar techniques. Further the discharging volume of cleaning water was also determined during 09am to 4pm in the month of February, 2014.

Figure 1: Train the farmers for operation and maintenance of solar desalination system.

Results and Discussion

Approximately 70% underground water is brackish. This water is unfit for drinking. The removal of excess salts from brackish water is the utmost requisite for the supply of drinkable water at the remote areas having dense saline water. Water quality is the main issues to save the whole humanity from epidemic diseases. Clean drinkable water is the basic right of the whole world. So this experiment was launched at National Agricultural Research Centre, Islamabad, Pakistan. Electrical conductivity is the main criteria to evaluate the water fitness for drinking purpose. Data presented in Table 1 showed the performance of desalinization solar units installed. Brackish water having different ECw levels under various sunlight temperatures at National Agricultural Research Centre, Islamabad, Pakistan removed the toxic salts through solar desalinization technique and reduced it from 0.01to 0.11dSm-1. Water having so minute salts is fit for drinking purpose. Utilization of sunlight for the reclamation of brackish water through solar desalinization is the friendly environment, most economical and easily installed with local training. Maintenance cost of these solar units is also very minute.

Table 1: Effect of solar desalination on cleaning of brackish water at NARC, Islamabad, Pakistan.

The use of solar irradiation for treatment of chemically and biologically contaminated water is not a new trend [17-23]. Pure water is the basic necessary for all living organism. Now days, the availability of clean water resource is a major issue for mankind. A lack of infrastructure for water storage and distribution is also a factor in the developing world. More than71% of the earth surface is covered with the water, but only 1% clean drinkable water is available with the international standards [24]. The purification of brackish water into drinkable water depends upon the intake saline water capacity of each desalinization solar unit. Data indicated in Table 1 showed that the discharge volume of cleaning water was increasing as well as the temperature of the sunlight hours increasing. Solar radiation removes a wide range of organic chemicals and pathogenic organisms by direct exposure, is relatively economical, and avoids cohort of harmful byproducts of chemically driven technologies [17]. More prominently, the economics of the process are approximately capacity self-reliant [25].

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Lupine Publishers | Farmer’s Perspective on Importance and Constraints of Seaweed Farming in Sri Lanka

 Lupine Publishers | Current Investigations in Agriculture and Current Research

Abstract

Seaweed cultivation is identified as a catalyst for social progression in coastal communities. Despite the potentials, the seaweed cultivation introduced to resettled coastal districts in Sri Lanka seems not performing to the expectations owing to various reasons. Farmers’ perspective as the leading stakeholders would facilitate the understanding of such a complexity. Therefore, the study attempted to assess the values and constraints related to seaweed farming as perceived by the seaweed farmers. Two-stage stratified random sampling technique was employed to draw a sample of 160 seaweed growers from the purposely-selected coastal areas of northern Sri Lanka. A perceived ordinal ranking method was exercised to assess the perceived importance, whilst Garrett’s ranking technique to detect the judgment of the farmers about the constraints. Next to fishing, seaweed farming received the highest perceived importance of the respondents. Favourable income and employment generation, the ability to easily manage with fishing, supportive role in empowering women and the existence of a favourable contract growing system were among the major causative responses contributed to the perceived importance. Major constraints identified in sea weed farming were adverse weather pattern (19.6%), poor quality of existing planting materials (16.68%), distortions prevailing in the purchasing mechanism (14.7%) and improper aquatic environments (13.74%). Thus, the study concluded that seaweed farming is perceived as an important livelihood option for the coastal communities and developing strategies to mitigate the impact of adverse environmental changes would promote seaweed cultivation.
Keywords: Constraints; Perceived importance; Seaweed farming; Sri Lanka

Introduction

Seaweed cultivation has become increasingly popular as one of the most important economic activities that can be practiced in the coastal regions. The potential role that seaweed could play in rural development being as a catalyst of social progression [1] by rendering extensive employment opportunities [2] to the coastal communities has already been identified. Accordingly, the government of Sri Lanka has introduced seaweed farming to resettled coastal districts in the northern part of the country to improve socio-economic conditions in vulnerable regions through the promotion of sustainable livelihood development. However, the available sporadic information in this regard imply that the level of performance seems to be suppressed by issues such as harsh weather conditions [3-5] and improper aquatic environments, which were commonly associated with seaweed farming. In this backdrop, assessing the value and constraints related to seaweed farming as perceived by the beneficiaries would uncover trustworthy, qualitative and in-depth information that would otherwise not become known. Such information would be essential for future planning by the policy makers for a better design to meet the farmer needs and can be useful both to fine-tune and enhance the effectiveness of the existing seaweed cultivation system.

Methodology

This study was conducted in purposely-selected coastal areas of Jaffna and Kilinochchi districts, located in the Northern Province of Sri Lanka (Figure 1). Two-stage stratified random sampling technique was employed to select 03 Divisional Secretariat (DS) divisions and 04 Grama Niladhari (GN) divisions respectively and finally to draw a sample of 160 seaweed growers. The required primary data were mainly collected through a structured and pre-tested questionnaire, in-depth interviews and focus group discussions. The perceived ordinal ranking method as previously adopted by Crawford [6] was exercised to assess the perceived importance of seaweed growers towards different livelihood options, whilst Garrett’s ranking technique [7-9] was exercised to detect the judgment of the farmers about the constraints faced by them in seaweed cultivation.

Figure 1: Study locations (marked in red).

Results and Discussion

Farmers’ perceived importance towards seaweed farming

Next to fishing, seaweed farming received the highest perceived importance of the respondents. Accordingly, sea weed farming was perceived by 94% of farmers as either first or second in importance (Figure 2). The reasons behind the perceived importance were satisfactory income and generation of employment, easily manageable nature with fishing, cooperative role in women empowerment and the prevailing promising contract growing system (Figure 2). Being an economically viable alternative livelihood option [10], seaweed farming has diversified the livelihood options of the farming communities and thereby has provided a stable annual average income, which improves household economic resilience [11] enabling a sustainable way of life. This finding is in conformity with the findings of Zacharia [12] and Tobisson [13] on the role of seaweed farming in coastal livelihood improvement. On the other hand, previous studies have identified the possibility of developing employment-incomeconsumption relationships related to the seaweed farming [2]. This employment potential [6,14] is highlighted by the participants and viewed that harvesting and initial preparation stages render extensive employment opportunities. Especially, the shallow water seaweed farming allows more involvement of women and renders more employment opportunities for women during initial preparation and harvesting stages.

Figure 2: Perceived importance towards different livelihood activities.

The interviewees identified that the seaweed farming is instrumental in empowering women [13,15] within the study area. Further, it was reported that without being much disturbing to the primary economic activity, the seaweed farming can be easily integrated with conventional fishing. Moreover, it was identified that seaweed farming requires lesser time for its maintenance after planting and allows farmers to engage in other activities as well. Altogether, these major causative responses led to the perceived importance and will assist in promoting the seaweed cultivation within the study area. Apart from these major causes the provision of rapid return on investment, the requirement of simple farming techniques and an alternative for deprivation of terrestrial lands for cultivation also affected for the perceived importance towards seaweed farming.

Constraints for seaweed farming

The results indicated that the adverse weather pattern (19.6%) is the major constraint faced by the seaweed farmers within the study area and obtained the highest rank. In conformity to the findings of Zamroni [3], the seasonal changes associated with monsoonal weather pattern is critically affecting the seaweed cultivation by limiting it to four cultivation seasons per year. Further, heavy rains and severe weather conditions like prolonged higher temperature periods regularly disturb the seaweed cultivation. These unfavorable changes would ultimately affect both quality and quantity [16] of seaweed harvest (Figure 3). Consisting with the findings of Zamroni [3], the poor quality of planting materials (16.68) obtained the second highest rank as a major constraint faced by the seaweed farmers. Farmers ordinarily use traditional self-propagation techniques using cuttings of the previous harvest. However, inferior strains created by overutilization of the old seaweed stocks may have negatively affected the growth rate and quality of seaweed and avoided growers from optimizing their yield.

Figure 3: Constraints ranked by seaweed growers.

The study identified that the buyers adopted a contract growing system as the purchasing mechanism of seaweed. Such mechanisms were further reported in the studies of Krishnan [3,17] and Hurdato (2013) related to seaweed farming. As identified by the study, low farm gate price, sporadic payments, and defective weighing processes have distorted (14.7) the qualities of the existing purchasing system. The respondents also viewed that the buyers regularly justify these low prices, highlighting the importance of technical provisions like raw materials and advisory support, which are mainly provided by them. Conversely, few growers believed that non-existence of layers of intermediaries and protection from time to time price fluctuations would still make the contract growing system more favourable.
The ideal aquatic environment provided in near-shore areas is favourable for sea weed farming. However, due to high competition and legal cut-offs imposed by the local authorities’ farmers haveshifted the cultivation to less fertile improper aquatic environments (13.74). Seaweed-farming locations were co-managed by coastal villagers and idled seaweed farms also contributed to this problem. Participants also indicated that seaweed cultivation locations away from the near-shore areas increased the overall production cost due to the expenses incurred for extra transportation.

Conclusion and Recommendations

Based on the logical interpretation of the findings, it can be concluded that seaweed farming is perceived as an alternative livelihood option for the coastal communities. This social acceptability implies the possibility of further expanding seaweed farming to other matching locations as a commercial enterprise. The adverse weather pattern was the major constraint perceived by the seaweed growers. In order to promote the existing system, the establishment of a weather damage relief program from the government, early warning system of sudden environmental changes and commercial level seaweed nurseries are recommended. Further, the collaborative action between key stakeholders is identified as a necessity to promote the seaweed industry.

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