Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension associated with hemolytic anemia

Hereditary hemoglobin disorders affecting the globin chain synthesis namely thalassemia syndromes and sickle cell disease (SCD) are the most common genetic disorders in human. Around 7% of the world population carries genes for these disorders, mainly the Mediterranean Basin, Middle and Far East, and Sub-Saharan Africa. An estimated 30 million people worldwide are living with sickle cell disease, while 60-80 million carry beta thalassemia trait. About 400,000 children are born with severe hemoglobinopathies each year. Cardiovascular complications of hemoglobinopathies include left and right ventricular (RV) dysfunction, arrhythmias, pericarditis, myocarditis, valvular heart disease, myocardial ischemia, and notably pulmonary hypertension (PH). Because of a unique pathophysiology, pulmonary hypertension associated with hemolytic disorders was moved from WHO group I to group V PH diseases. Treatment strategies are also unique and include blood transfusion, iron chelation, hydroxyurea, and oxygen therapy. The role of PH-specific agents has not been established

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

Adherence to COPD treatment in Turkey and Saudi Arabia: results of the ADCARE study.

COPD affects millions of people worldwide. Poor treatment adherence contributes to increased symptom severity, morbidity and mortality. This study was designed to investigate adherence to COPD treatment in Turkey and Saudi Arabia

Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality

BACKGROUND: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). The objective of this study is to describe the clinical characteristics, mortality, and predictors of SSc-PAH in Saudi patients. METHODS: Retrospective chart review study of SSc patients who were followed for at least 1 year in three tertiary care centers in Saudi Arabia was conducted. Clinical information, echocardiographic findings, and right heart catheterization (RHC) results were collected. Descriptive statistics were used for demographic and disease characteristics. RESULTS: Fifty-seven patients with SSc were reviewed. PAH was confirmed by RHC in 40 patients (87.5%, females). Their mean age was 45.43 ± 13.48 years. The mean pulmonary artery pressure was 42.9 ± 12.7 mmHg, the pulmonary vascular resistance index was 19.4 ± 7.7 woods unit, and cardiac index was 2.43 ± 0.68 min/m2. The median time from symptoms to first assessment was 42.8 ± 115.62 months. Most patients (77.5%) presented with functional Class III or IV and more than half (22.55%) were on dual combination therapy. Ten patients (25%) SSc PAH died over a follow up period of 37 ± 7 months. Compared to SSc patients without PAH, SSc-PAH patients had shorter 6-min walk distance (6MWD) (296.1 ± 116.5 vs. 399.59 ± 40.60 m, P < 0.0001), higher pro-brain natriuretic peptide (1755.8 ± 2123.4 vs. 69.8 ± 44.3 pg/ml P = 0.004), and more frequent Raynaud's phenomenon (RP) (90% vs. 35%, P < 0.0001). Logistic regression showed RP (odds ratio [OR] =48.58, 95% confidence interval [CI]; 3.73–633.10) and 6MWD (OR 1.02: 95% CI; 1.01–1.03) were associated with the development of PAH. CONCLUSION: Our cohort of Saudi SSc-PAH patients has a younger disease onset and a lower mortality than what is described worldwide despite late presentation and requirement of combination therapy. The presence of RP and lower were associated with the development of SSc-PAH

Adherence to COPD treatment in Turkey and Saudi Arabia: results of the ADCARE study

Background: COPD affects millions of people worldwide. Poor treatment adherence contributes to increased symptom severity, morbidity and mortality. This study was designed to investigate adherence to COPD treatment in Turkey and Saudi Arabia. Methods: An observational, cross-sectional study in adult COPD patients in Turkey and Saudi Arabia. Through physician-led interviews, data were collected on sociodemographics and disease history, including the impact of COPD on health status using the COPD Assessment Test (CAT); quality of life, using the EuroQol Five-Dimension questionnaire (EQ-5D); and anxiety and depression using the Hospital Anxiety and Depression Scale (HADS). Treatment adherence was measured using the 8-item Morisky Medication Adherence Scale (MMAS-8). Multivariate logistic regression analysis examined the predictors of non-adherence and the impact of adherence on symptom severity. Results: Four hundred and five COPD patients participated: 199 in Turkey and 206 in Saudi Arabia. Overall, 49.2\% reported low adherence (MMAS-8 15) compared to 58.4\% reporting medium/high adherence (p = 0.0008). Patients with low adherence reported a lower mean 3-level EQ-5D utility value (0.54 +/- 0.35) compared to those with medium/high adherence (0.64 +/- 0.30; p 10 was associated with lower adherence (OR 2.50 {[}95\% CI: 1.43-4.39] and 2.43 {[}95\% CI: 1.39-4.25], respectively; p = 0.0008). Being a high school/college graduate was associated with better adherence compared with no high school (OR 0.57 {[}95\% CI: 0.33-0.98] and 0.38 {[}95\% CI: 0.15-1.00], respectively; p = 0.0310). After adjusting for age, gender, and country, a significant association between treatment adherence (MMAS-8 score >= 6) and lower disease impact (CAT <= 15) was observed (OR 0.56 {[}95\% CI: 0.33-0.95]; p = 0.0314). Conclusion: Adherence to COPD treatment is poor in Turkey and Saudi Arabia. Non-adherence to treatment is associated with higher disease impact and reduced quality of life. Depression, age, and level of education were independent determinants of adherence

Portopulmonary hypertension

Portopulmoanry hypertension (POPH) is a form of pulmonary arterial hypertension (PAH) associated with portal hypertension with or without underlying chronic liver disease. POPH is increasingly recognized and recent evidence suggests that it is one of the leading causes of PAH. The pathophysiology of POPH is poorly understood although the pathological changes in pulmonary vasculature in advanced POPH are similar to those seen in idiopathic pulmonary hypertension. The prognosis in patients with liver disease who also suffer from significant POPH is considered to be poor. Higher degree of pulmonary artery pressure (PAP) may preclude a patient from liver transplant as mortality in these patients is high. The treatment with vasodilator therapy has shown to improve both hemodynamics and clinical outcome in POPH in retrospective studies and in some case series. The aim of medical management is to bring PAP <35 mmHg that may make a patient with POPH and advanced liver disease eligible for liver transplant, which otherwise would have been denied because of high PAP

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