Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: 2014 updates

The Saudi Association for Pulmonary Hypertension [previously called Saudi Advisory Group for Pulmonary Hypertension] has published the first Saudi Guidelines on Diagnosis and Treatment of Pulmonary Arterial Hypertension back in 2008. [1] That guideline was very detailed and extensive and reviewed most aspects of pulmonary hypertension [PH]. One of the disadvantages of such detailed guidelines is the difficulty that some of the readers who just want to get a quick guidance or looking for a specific piece of information might face. All efforts were made to develop this guideline in an easy-to-read form, making it very handy and helpful to clinicians dealing with PH patients to select the best management strategies for the typical patient suffering from a specific condition. This Guideline was designed to provide recommendations for problems frequently encountered by practicing clinicians involved in management of PH. This publication targets mainly adult and pediatric PH-treating physicians, but can also be used by other physicians interested in PH

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: portopulmonary hypertension

Portopulmonary hypertension [POPH] is defined as pulmonary arterial hypertension [PAH] complicated by portal hypertension, with or without advanced hepatic disease. Significant percentage of patients with cirrhotic liver disease has high cardiac output and subsequently elevated pulmonary arterial pressures [PAP]. However, patients with POPH develop a progressive increase in pulmonary vascular resistance [PVR], which is generally lower than that observed in other forms of PAH. The prognosis of untreated patients with POPH is very poor and the outcome of liver transplant [LT] in those patients is determined by the degree of severity of the associated pulmonary hemodynamics. In this narrative review, we describe the clinical presentation of POPH, the pathobiology, and the clinical implication of pulmonary hemodynamics. We also provide evidence-based recommendations for the diagnosic and management approaches of POPH

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: pulmonary hypertension due to left heart disease

Pulmonary hypertension [PH] due to left heart disease is the most common cause of pulmonary hypertension in the western world. It is classified as WHO PH group II. Different pathophysiologic abnormalities may take place in this condition, including pulmonary venous congestion and vascular remodeling. Despite the high prevalence of WHO group 2 PH, the major focus of research on PH over the past decade has been on WHO group 1 pulmonary arterial hypertension [PAH]. Few investigators have focused on WHO group 2 PH; consequently, the pathophysiology of this condition remains poorly understood, and no specific therapy is available. Clinical and translational studies in this area are much needed and have the potential to positively affect large numbers of patients. In this review, we provide a detailed discussion upon the pathophysiology of the disease, the recent updates in classification, and the diagnostic and therapeutic algorithms

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: pulmonary hypertension due to lung diseases and/or hypoxia

Chronic lung diseases are common causes of pulmonary hypertension. It ranks second after the left heart disease. Both obstructive and restrictive lung diseases are know to cause pulmonary hypertension. The pathophysiology of the disease is complex, and includes factors affecting the blood vessels, airways, and lung parenchyma. Hypoxia and the inhalation of toxic materials are another contributing factors. Recent guidelines have further clarified the association between pulmonary hypertension and chronic lung disease and made general guidelines concerning the diagnosis and management. In this article, we will provide a detailed revision about the new classification and give general recommendations about the management of pulmonary hypertension in chronic lung diseases

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension [CTEPH] is categorized as group IV in the WHO classification for pulmonary hypertension. The disease requires a very low index of suspicion for identification and needs a special diagnostic approach utilizing clinical, radiological, and hemodynamic tools. As CTEPH is potentially curable, all efforts should be consumed to reach the accurate diagnosis and subsequently evaluated for operability. Although pulmonary endarterectomy [PEA] is the only curative tool so far, recent updates concerning medical and interventional therapy have made significant advances in inoperable patients. In this review, we provide a detailed discussion on diagnostic algorithm, surgical operability criteria, PEA, and the medical therapy

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: general management for pulmonary hypertension

Treatment of pulmonary hypertension [PH] patients is challenging and should only be initiated after a comprehensive diagnostic evaluation. Such treatment should ideally be done in specialized centers with full capability for hemodynamic measurements, having access to a broad range of PAH therapies, and adequate experience in the management of critically ill patients. The following discussion is intended to review the general measures and the non-specific [supportive] therapy used in managing PH patients, while the specific therapy will be discussed in a subsequent different article

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: medical and surgical management for pulmonary arterial hypertension

Prior to the availability of the pulmonary arterial hypertension [PAH]-specific therapy, PAH was a dreadful disease with a very poor prognosis. Better understanding of the complex pathobiology of PAH has led to a major therapeutic evolution. International regulatory agencies have approved many specific drugs with different pharmacologic pathways and routes of administration. In the year 2013, two new drugs with great potentials in managing PAH have been added to the treatment options, macitentan and riociguat. Additional drugs are expected to come in the near future. A substantial body of evidence has confirmed the effectiveness of pulmonary arterial hypertension [PAH]-specific therapies in improving the patients' symptomatic status and slowing down the rate of clinical deterioration. The newer modern medications have significantly improved the survival of patients with PAH, it remains a non-curable and fatal disease. Lung transplantation [LT] remains the only therapeutic option for selected patients with advanced disease who continue to deteriorate despite optimal therapy

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: pregnancy in pulmonary hypertension

Pregnancy in pulmonary hypertension [PH] is known to be associated with high morbidity and mortality. The physiological changes occur during normal pregnancy, such as increase blood volume and cardiac output [CO] may be detrimental in PH patients. Several practice guidelines advise against pregnancy and even recommend termination of pregnancy. Occasionally PH may be diagnosed for the first time during pregnancy, as stress of pregnancy can unmask previously undiagnosed PH in an asymptomatic individual. This narrative review provides a detailed discussion about the physiologic parameters associated in pregnancy and their negative effect on the right ventricle. It also gives practical evidence-based recommendations about different management issues in PH pregnant patients

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: challenges in managing pulmonary hypertension in the developing countries

There is scant published data about pulmonary hypertension [PH] from the developing countries. True prevalence of the disease, its biology, etiology and response to treatment are not well known, and they are likely to be somewhat different from that of the developed countries. In this review, we will discuss the main challenges for managing PH in developing countries and propose real-life recommendations to deal with such difficulties

Complete resolution of chronic pericardial effusion with an intensive course of inhaled iloprost in an adult patient with unrepaired ventricular septal defect, and life-threatening severe pulmonary arterial hypertension

A 38-year-old male was diagnosed with unrepaired ventricular septal defect associated with severe pulmonary arterial hypertension, cyanosis, and significant exercise intolerance. His echocardiogram showed right ventricular dysfunction and moderate pericardial effusion with no signs of cardiac tamponade. He was treated with an intensive course of inhaled iloprost and sildenafil. He showed a dramatic clinical response; his saturation went up from 60% on admission to 90% on minimal oxygen with significant improvement in his symptoms and signs of heart failure and total resolution of pericardial effusion. On follow up 3 and 6 weeks later, he was stable and could walk 360 meters in a 6 minutes walk test with disappearance of pericardial effusion. With unavailability of intravenous prostacyclin, we have shown in this case that intensive administration of inhaled iloprost could be used intensively as a rescue therapy in severe cases of pulmonary arterial hypertension with excellent results

Prevalence of chronic obstructive pulmonary disease in Saudi Arabia

To assess the prevalence of epidemiologically defined chronic obstructive pulmonary disease [COPD] in Saudi Arabia. This cross-sectional, observational, population-based survey of COPD was conducted between June 2010 and December 2011 across the country of Saudi Arabia. A total of 56,000 randomly selected telephone numbers were called, which identified 10,001 eligible subjects; of whom 9,779 agreed to participate. A screening questionnaire included 6 questions related to cigarette consumption and water-pipe use was administered to each participant. Subjects with positive screening results were invited to provide input for a detailed COPD questionnaire. The adjusted proportion of subjects who reported a current, or past smoking history was 27.9%. Gender specific smoking rates adjusted by age were 38.7% [95% confidence interval [CI]: 37.5-39.9%] in men, and 7.4% [95% CI: 6.5-8.3%] in women. The epidemiological definition of symptomatic COPD was met by a total of 249 subjects. The age and gender-adjusted prevalence of COPD was 2.4% [95% CI: 2.1-2.7%]. Overall, COPD was more frequently documented [p<0.0001] in men [3.5% [95% CI: 3-4%]] than in women [1% [95% CI: 0.7-1.3%]]. The prevalence of epidemiologically defined COPD in the general population of Saudi Arabia is 2.4%, which is lower than that reported in industrialized countries

Saudi guidelines for the diagnosis and management of COPD

The Saudi Thoracic Society [STS] launched the Saudi Initiative for Chronic Airway Diseases [SICAD] to develop a guideline for the diagnosis and management of chronic obstructive pulmonary disease [COPD]. This guideline is primarily aimed for internists and general practitioners. Though there is scanty epidemiological data related to COPD, the SICAD panel believes that COPD prevalence is increasing in Saudi Arabia due to increasing prevalence of tobacco smoking among men and women. To overcome the issue of underutilization of spirometry for diagnosing COPD, handheld spirometry is recommended to screen individuals at risk for COPD. A unique feature about this guideline is the simplified practical approach to classify COPD into three classes based on the symptoms as per COPD Assessment Test [CAT] and the risk of exacerbations and hospitalization. Those patients with low risk of exacerbation [<2 in the past year] can be classified as either Class I when they have less symptoms [CAT < 10] or Class II when they have more symptoms [CAT >/= 10]. High-risk COPD patients, as manifested with >/= 2 exacerbation or hospitalization in the past year irrespective of the baseline symptoms, are classified as Class III. Class I and II patients require bronchodilators for symptom relief, while Class III patients are recommended to use medications that reduce the risks of exacerbations. The guideline recommends screening for co-morbidities and suggests a comprehensive management approach including pulmonary rehabilitation for those with a CAT score >/= 10. The article also discusses the diagnosis and management of acute exacerbations in COPD.

Prevalence and severity of plaque-induced gingivitis in a Saudi adult population

To evaluate the prevalence and severity of plaque-induced gingivitis among a Saudi adult population in Riyadh region. Three hundred and eighty-five eligible participants in this cross-sectional study were recruited from routine dental patients attending the oral diagnosis clinic at Al-Farabi College in Riyadh, Saudi Arabia from June 2013 to December 2013. A clinical examination was performed by 2 dentists to measure the gingival and plaque indices of Loe and Silness for each participant. The prevalence of gingivitis was 100% among adult subjects aged between 18-40 years old. Moreover, the mean gingival index was 1.68 +/- 0.31, which indicates a moderate gingival inflammation. In fact, males showed more severe signs of gingival inflammation compared with females [p=0.001]. In addition, the mean plaque index was 0.875 +/- 0.49, which indicates a good plaque status of the participants. Interestingly, the age was not related either to the gingival inflammation [p=0.13], or to the amount of plaque accumulation [p=0.17]. However, males were more affected than females [p=0.005]. The results of this study show that plaque accumulation is strongly associated with high prevalence of moderate to severe gingivitis among Saudi subjects

Saudi initiative for asthma - 2012 update: guidelines for the diagnosis and management of asthma in adults and children

This an updated guidelines for the diagnosis and management of asthma, developed by the Saudi Initiative for Asthma [SINA] group, a subsidiary of the Saudi Thoracic Society. The main objective of SINA is to have updated guidelines, which are simple to understand and easy to use by non-asthma specialists, including primary care and general practice physicians. This new version includes updates of acute and chronic asthma management, with more emphasis on the use of Asthma Control Test in the management of asthma, and a new section on "difficult-to-treat asthma." Further, the section on asthma in children was re-written to cover different aspects in this age group. The SINA panel is a group of Saudi experts with well-respected academic backgrounds and experience in the field of asthma. The guidelines are formatted based on the available evidence, local literature, and the current situation in Saudi Arabia. There was an emphasis on patient-doctor partnership in the management that also includes a self-management plan. The approach adopted by the SINA group is mainly based on disease control as it is the ultimate goal of treatment

Novel approach for the management of sub-massive pulmonary embolism

Right ventricular [RV] dysfunction has been identified as a poor prognostic indicator in sub-massive pulmonary embolism [SPE]. We hypothesized that using selective vasodilator agent is beneficial in improving RV function in patients with this condition. We used inhaled prostacyclin analogue [Iloprost, Ventavis[registered]] in five patients with SPE. Helical computerized tomography angiogram was confirmatory for pulmonary embolism and echocardiography was used to evaluate the RV status. All patients received inhaled Iloprost, 2.5 to 5 microg every 4 hours for 3 weeks. Patients were prospectively followed for 3 months. They were assessed at baseline before starting Iloprost treatment and at 3 days, 3 weeks, and 3 months after treatment. All patients showed significant improvement in their functional class, Borg dyspnea score, NT pro-BNP level, and echocardiographic parameters. In SPE, directing therapy toward decreasing pulmonary vascular resistance improves the associated pulmonary hemodynamic compromise and improves RV function

Saroidosis-associated pulmonary hypertension: clinical features and outcomes in Arab patients

Pulmonary hypertension [PH] occurs in many patients with interstitial lung disease, including sarcoidosi. We explored the frequency, clinical characteristics and outcomes of PH in Arab patients diagnosed with pulmonary sarcoidosis. A retrospective study in three tertiary hospitals was performed on 96 patients who underwent Doppler echocardiography. Demographic and clinical characteristics, physiological studies and computed tomography [CT] results were collected and compared between patients with and without PH. Twenty [20.8%] patients were found to have PH. Patients with PH were more likely to be sumptomatic [cough, P= 0.008; dyspnea, P=0.04], to have an advanced radiographic stage [P=0.001], and to be receiving systemic therapy [P=0.0011], compared to those without PH. Physiological data including pulmonary function test parameters, arterial blood gas levels and oxygen saturation at rest and after exercise were all significantly lower in patients with PH compared to those without PH. Compariosn of CT patterns between patients with and without PH showed significant differences in the frequencies of ground-glass opacity [61.5 vs. 28.8%, P=0.032] and fibrosis [76.9 vs., 44.2%, P=0.035]. In total, four patients died during the study period, including three with evidence of PH. The frequency of PH in the present study was 20.8%. Clinical, physiologic and radiographic characteristics appeared to differentiate patients with PH from those without PH. The presence of PH contributed to poor outcomes in patients with pulmonary sarcoidosis

Clinical characteristics and computed tomography findings in Arab patients diagnosed with pulmonary sarcoidosis

Sarcoidosis is prevalent worldwide with significant heterogeneity across different ethnic groups. We aimed to describe the clinical characteristics and computed tomography findings among Arab patients with pulmonary sarcoidosis. A retrospective study of patient demographics, symptoms, co-morbid illness, sarcoidosis stage, treatment, pulmonary function and CT results. Of 104 patients, most [77%] were 40 years of age or older at diagnosis, and females in this category [>/= 40 years] significantly outnumbered male patients [69/104 [66.3%] vs. 35/104 [33.7%], P=.003]. The most common complaints were dyspnea [76%], cough [72.1%] and weight loss [32.7%]. The majority of patients displayed impairment in lung function parameters at presentation. However, significant impairment in forced vital capacity, percentage predicted [FVC%] [<50%] was present in only 17% of patients. The most frequent CT finding was mediastinal lymph node enlargement in 49 patients [73.1%]. Parenchymal abnormalities indicating lung fibrosis were noted in 31 patients [46.3%], and traction bronchiectasis was the most common [35.8%] fibrotic pattern detected on CT scans. At presentation, clinical manifestations of sarcoidosis among this sample of Arab patients were similar to reports from other nations. Further studies are needed to explore the effects of race and ethnicity on disease severity in the Middle East

Saudi initiative for asthma

The Saudi Initiative for Asthma [SINA] provides up-to-date guidelines for healthcare workers managing patients with asthma. SINA was developed by a panel of Saudi experts with respectable academic backgrounds and longstanding experience in the field. SINA is founded on the latest available evidence, local literature, and knowledge of the current setting in Saudi Arabia. Emphasis is placed on understanding the epidemiology, pathophysiology, medications, and clinical presentation. SINA elaborates on the development of patient-doctor partnership, self-management, and control of precipitating factors. Approaches to asthma treatment in SINA are based on disease control by the utilization of Asthma Control Test for the initiation and adjustment of asthma treatment. This guideline is established for the treatment of asthma in both children and adults, with special attention to children 5 years and younger. It is expected that the implementation of these guidelines for treating asthma will lead to better asthma control and decrease patient utilization of the health care system