Pulmonary hypertension (PH) is a grave disease that causes major disability and early mortality. Although it is a rare disease, it affects a large number of populations all around the world. Nepal is a developing country and the patients of pulmonary hypertension are typically brought to a physician’s attention during the latter stages of the disease, as they will have likely been previously underdiagnosed. This is further complicated by the fact that there are no proper studies on its prevalence. In comparison to other developing countries, Nepal has high prevalence rates of rheumatic heart disease, chronic obstructive pulmonary disease, smoking and indoor pollution, and therefore a high prevalence of pulmonary hypertension can be expected. Further complexities include late presentation of the disease and low availability of health personnel who can diagnose and manage the condition. While some studies on genetic factors and high altitude pulmonary hypertension have been done in Nepal, much attention should be allocated on epidemiological studies. In this context, the article gives a nutshell view of the challenges of diagnosing pulmonary vascular disease in Nepal.
Out of 29 million people in Nepal, more than fifty percent of the population live in hilly and mountainous regions, and most of the health services are located in the major cities and the capital. Most of these rural populations uses firewood as fuel, are undereducated regarding health issues, and traditional medical practices hide much of the pulmonary diseases in Nepal. Uncommon diseases like pulmonary hypertension do not get the necessary attention from policy makers. Therefore, pulmonary hypertension in Nepal is underfocussed, and most of these patients will have a co-morbid presentation. Since the patient compliance is poor in chronic and incurable disease, the course of the disease is almost always fatal.
Pulmonary hypertension starts with sustained aberrant vasoconstriction and progresses with intimal proliferation, medial hypertrophy and later remodeling of pulmonary arteries leading to an irreversible phase of the disease. Eventual right heart failure with progression of pulmonary hypertension makes this disease a killer among chronic pulmonary diseases. A typical case presentation: A 77 years old male walked in emergency room at Helping Hands Community Hospital on 20/12/2011. Although his friendly smile revealed how happy he was to reach a hospital in the capital, his efforts to breathe signaled the gravity of illness and immediate need for medical attention. After quick vitals and finger tip saturation probe assay, we started him on 100 percentage Oxygen at 2 liters per minute, gave him nebulisation and a shot of hydrocortisone. This patient, a resident of Khadbari, Sankhuwasabha of rural Nepal, is one of the many chronic obstructive pulmonary disease (COPD) patients we see in our hospitals every day. A general hospital in Nepal sees a lot of COPD patients, mostly associated with corpulmonale, on a daily basis. A case like this either gets missed or falls in hands of a traditional religious healer. Many such cases present with chest infection that only exacerbates this disease and leads to untimely death. This particular person is one among many such late presenters, who gets stuck at home for days before gathering help to reach the nearest medical center, often many miles away, and is then faced with a long uncomfortable ride on the back of a horse to a healthpost where a paramedic suggests the urgency of specialized care. In this case we must give thanks to the paramedic, who gave him a pocketful of diuretics and bronchodilators to ease his final journey to our hospital. On the second day in bi-level positive airway pressure, a joyful face welcomed us. However, naive and illiterate, many of the patients we see become addicted to cigarette smoking during early childhood and continue smoking till the late phase of COPD, with associated complications, and our patient was no exception. His bedside echocardiogram showed pulmonary hypertension secondary to COPD, right ventricular hypertrophy, dilated right atrium, and tricuspid valve regurgitation. The chronic progression of the diseases diagnosed, and the absence of permanent cure, only left him and his family demoralized, as they had tried so hard and travelled so far to get him here. The expenses in the capital Kathmandu only increased his suffocation. Even if he quit smoking, there is a considerable probability that his COPD will increase over next few years because of the indoor pollution from firewood.
In Nepal, like in any other country in the world, pulmonary hypertension is expected to be distributed across all the age groups. So far the epidemiological studies in pulmonary hypertension are not done in Nepal. Higher incidence compared to other developing countries can be presumed, especially owing to indoor air pollution, a high smoking rate in rural areas, undiagnosed and untreated congenital heart diseases, a high rate of rheumatic heart diseases, rising HIV infection and high incidence and prevalence of pulmonary tuberculosis. Most households in the rural areas of Nepal have indoor pollution due to passive smoking because of traditional firewood kitchen, thereby contributing to development of COPD and associated complications. Women are exposed more to the indoor pollution than males, since in Nepal, most of the household PVRI Chronicle: Volume 1 Issue 1, January - June 2014 chores are considered the responsibilities of women. Therefore, it can be presumed that most of the pulmonary hypertension arises secondary to chronic obstructive pulmonary disease. In concordance with rest of the world, in children and adolescents the most common cause of the pulmonary hypertension in Nepal is presumed to be congenital heart disease. Most of these congenital heart diseases causing pulmonary hypertension are diagnosed late because of inadequate accessibility and availability of the medical expertise needed. In adults, besides COPD, pulmonary hypertension can be a consequence of rheumatic heart disease, pulmonary tuberculosis and HIV infection, owing to the high incidence of these diseases. However, schistosomiasis, a major disease of some of the developing countries responsible for PH, is not reported in Nepal. The data in some studies suggest that Sherpa are genetically protected for PH, but Sherpa represent only a tiny fraction of Nepalese population. In summary, pulmonary hypertension in Nepal is not properly studied and the cases and its fatalities are potentially rising each year.
The relative unavailability of Doppler echocardiogram for screening pulmonary hypertension is one of the major challenges in the diagnosis of PH in Nepal. Very few centers in the capital offer catheterization services. Additionally, as suggested in the presented case, the relative lack of expertise to assess pulmonary pressure, unavailability of manpower and equipments in rural areas, low health education about pulmonary hypertension and poor screening of congenital heart diseases make the diagnosis of pulmonary hypertension a challenge in Nepal. Therefore, only the tip of the iceberg of pulmonary hypertension cases reach tertiary centers, and those cases often come at a late stage with associated with right ventricular failure. Furthermore, most of the early manageable cases of PH are also underdiagnosed. In Nepal, most of the patients approach traditional healers. The absence of government scrutiny regarding traditional practitioners has resulted in unsystematic and unrecorded numbers of such patients. Lack of proper referral by such healers has hidden a lot of pulmonary hypertension at early stages, which will eventually be fatal. Moreover, most of the traditional healers refuse to tell the ingredients of their medicines, and almost always declare their products as “adverse affects free”, which attracts more patients still. Some of these traditional herb medicines and cough syrups might ease the patients for a short time, but no proper studies have been conducted to prove their efficacy. The current approved treatment options known to improve survival rate of the disease is not readily available in Nepal. Tertiary centers provide limited medical management to the disease, and they include calcium channel blockers and phosphodiesterase type 5 inhibitors, the latter afforded only by a high socioeconomic class. As a result, excepting a few centers in the capital, the treatment of congenital and rheumatic heart diseases with interventions that would prevent progression of pulmonary hypertension associated with these diseases is simply not possible. Therefore, even with proper diagnosis, PH treatment in tertiary centers is commonly restricted to diuretics, general supportive measures like bed rest, calcium channel blockers and low doses of aspirin with supplemental oxygen therapy. As a result of this variety of factors, pulmonary hypertension poses a great burden for the patient and their family. Although families in a high socioeconomic class can afford the treatment in the tertiary centers, an average family with PH will suffer economically, especially if the bread earner is stricken with the disease. Patients suffer clinical and hemodynamic deterioration sooner than expected due to poor compliance to the medicines, complex treatment protocol and relative inaccessibility of the tertiary health service providers. Congenital heart diseases leading to pulmonary hypertension later in life are hard to screen, especially against the background of a conservative, male predominant, shy Nepalese society. Low literacy rates hinder the proper medical education regarding harmful effects of indoor air pollution and passive and active smoking, meaning that the necessary change of attitudes and practices that prevent non communicable diseases like COPD and PH is extremely slow. Although the government has banned cigarette advertisements in public and has made some efforts to raise awareness of the preventable diseases, recent political turmoil and allegations in the health sector have only augmented the distrust towards the policy makers, further complicating the implementation of plans and policies each year.
Although there is considerable progress in diagnosis and management of pulmonary hypertension in developed countries, Nepalese medical practitioners face great challenges regarding diagnosis and proper management of pulmonary hypertension in the country. Early diagnosis and treatment of the pulmonary hypertension is advocated with use of echocardiogram, which serves as a fairly sensitive and specific method for the diagnosis of PH, although a gold standard test requires an organized hospital and catheterization lab set up. Follow up echocardiogram studies in rheumatic heart diseases, pulmonary tuberculosis, HIV/AIDS and connective tissue diseases should be advocated to timely diagnose pulmonary hypertension, educating the patient at the same time. Finally, the availability as well as accessibility of the drugs needed to treat pulmonary hypertension should be increased in Nepal, and the government should implement policies for substantial price reduction in such medicines to improve quality of life of the pulmonary hypertension affected patient.
Suggestions and Future Directions:
Needless to say, a substantial proportion of PH results from preventable causes (e.g. COPD) or manageable causes (e.g. rheumatic valvular diseases, connective tissue diseases). However, guidelines are not yet envisioned to address the majority of these cases and therefore, addressing pulmonary hypertension in Nepal is neither easy nor instant. Considering this, a multidimensional approach to the disease from all sectors is needed to early diagnose, treat, and prevent pulmonary hypertension in Nepal.