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.
Pulmonary hypertension; general measures; supportive therapy; Saudi association for pulmonary hypertension guidelines
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.
Although 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.
Specific therapy; target therapy; pulmonary arterial hypertension; lung transplant; Saudi association for pulmonary hypertension guidelines
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. 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.
Pulmonary hypertension; pulmonary vascular resistance; modified functional class; target therapy; SAPH guidelines
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®) 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 μg 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.
Iloprost; pulmonary hypertension; pulmonary vascular resistance; right ventricular function; sub-massive pulmonary embolism
The aim of this study was to evaluate asthma control after the introduction of a leukotriene modifier (Montelukast), in addition to the current controller asthma therapies, in patients with inadequately controlled mild-to-moderate persistent asthma. Asthma control and patient perception were assessed prior to, and 4 weeks after, the introduction of Montelukast, and the pre-introduction and post-introduction results were compared.
Materials and Methods:
A cross-sectional, observational study collected information on 1,490 eligible adult asthmatic patients in Saudi Arabia. The eligibility criteria included patients aged 15 years or more with symptomatic mild-to-moderate persistent asthma despite treatment with inhaled corticosteroids with or without long-acting beta agonist; also, the patient should attend the initial visit and follow-up visits after at least 4 weeks.
Of the 1,490 eligible patients, 79.5% received inhaled corticosteroids alone, and the remaining 20.5% received combination of inhaled corticosteroids and long-acting bronchodilator. Despite the treatment with daily controller medications, asthma symptoms persisted in more than two-thirds of the study population. Upon adding Montelukast, more than 80% of patients reported improvement in symptoms, which was consistent in all patients irrespective of corticosteroid type or dose (stratum) or the addition of long-acting β2-agonist. At the follow-up visit, 92.2% of patients reported that they felt better on Montelukast.
Leukotriene modifier Montelukast has significant additive benefits in the management of patients who suffer from mild-to-moderate asthma and who are inadequately controlled on inhaled corticosteroids therapy with or without long-acting bronchodilator.
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.
Pregnancy; pulmonary hypertension; right ventricular failure; Saudi association for pulmonary hypertension guidelines
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.
Chronic thromboembolic pulmonary hypertension; endarterectomy; riociguat; Saudi association for pulmonary hypertension guidelines
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.
Pulmonary hypertension; left heart disease; pulmonary artery wedge pressure; left ventricular end diastolic pressure; Saudi association for pulmonary hypertension guidelines
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.
Hypoxia; lung diseases; pulmonary hypertension; chronic obstructive airways disease; pulmonary fibrosis; Saudi association for pulmonary hypertension guidelines
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.
Liver transplant; portal hypertension; portopulmonary hypertension; pulmonary arterial hypertension; vasodilator therapy; Saudi Association for Pulmonary Hypertension Guidelines
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.
Developing world; pulmonary hypertension; Saudi association for pulmonary hypertension guidelines
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.
Chronic bronchitis; chronic obstructive pulmonary disease; emphysema; guidelines; Saudi Arabia
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.
Asthma; asthma control test; guidelines; Saudi Arabia
We assessed the relationship between physiologic parameters, computed tomography patterns, 6 minute walk distance (6MWD) and the distance-saturation product [DSP; defined as the product of the 6MWD and the lowest oxygen saturation during the 6 minute walk test (6MWT)]. In addition, we investigated factors affecting 6MWD in patients with pulmonary sarcoidosis.
We performed a retrospective study of patient demographics, treatment, pulmonary function, 6MWT, echocardiography and computed tomography results.
Fifty nine patients were included in this study. Their mean+standard deviation age was 47.5 years + 12.5 years, and 42 (71.2%) were female. Mean pulmonary function parameters for forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1) and total lung capacity (TLC) results, as percentages of predicted values, were 77.6 ± 22.2, 77.1 ± 22.8 and 78.7 ± 16.1, respectively. Comparison of the DSP with distance walked revealed a significant correlation with factors underlying reduced 6MWD, including gender, pulmonary function indices, partial pressure of oxygen (PaO2), and Borg dyspnea score. Other factors were significantly associated with DSP but not distance; these included lung fibrosis (p = 0.02), pulmonary hypertension (p = 0.01) and systemic therapy (p = 0.04). Backward elimination stepwise multiple regression analysis revealed that gender, and FEV1 were independent predictors of 6MWD, but FEV1 was more strongly related when DSP applied [DSP, R2 = 0.53, p = 0.02; distance, R2 = 0.45, p < 0.0001].
Our findings reveal that, compared to 6MWD alone, the DSP is correlated with a greater number of factors associated with reduced 6MWT performance. Therefore, the DSP may be a useful indicator of functional status in patients with sarcoidosis. Additional large-scale studies are warranted to validate our findings.
Pulmonary hypertension (PH) occurs in many patients with interstitial lung disease, including sarcoidosis. 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 symptomatic (cough, P = 0.008; dyspnea, P = 0.04), to have an advanced radiographic stage (P = 0.001), and to be receiving systemic therapy (P = 0.001), 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. Comparison 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.
Computed tomography; echocardiography; pulmonary function test; pulmonary hypertension; sarcoidosis
BACKGROUND AND OBJECTIVE:
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.
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 long-standing 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.
Asthma; guidelines; Saudi Arabia