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 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

Portopulmonary hypertension

Portopulmonary 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 advance 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 the 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 <35mmHg 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

Effect of obstructive disease in patients with non-cystic fibrosis bronchiectasis

Extensive research has been devoted to cystic fibrosis-related brochiectasis, compared with non-cystic fibrosis bronchiectasis but the latter is more common and results in significant morbidity and mortality. We assessed the relationship between pulmonary function test [PFT] findings and sputum bacteriology, blood gases, number of hospital admissions and mortality in patients with non-cystic fibrosis bonchiectasis [NCFB]. We conducted a retrospective review of 88 consecutive patients admitted with exacerbation of bronchiectasis over 5 years from 1996 to 2001. Demographic and clinical data collected included gender, age, pulmonary functions, arterial blood gases, sputum bacteriology during stable and exacerbation periods, and number of hospital admissions due to exacerbation of bronchiectasis. A comparison was made between patients having obstructive airway disease [OAD group] and patients with normal or restrictive pulmonary functions [non-OAD group]. OAD in patients with NCFB adversely affected clinical outcome. There was a significant increase in Pseudomonas colonization [60.3% vs. 16%; P<0.0003], hypercapnic respiratory failure [63.4% vs. 20%; P<0.0003], and mean number of admissions due to exacerbation [6 vs. 2; P<0.0001] in the OAD group as compared with the non-OAD group. Although mortality was increased in the OAD group, the difference was not statistically significant. Patients with NCFB who have OAD have a significantly higher rate of colonization with Pseudomonas aeruginosa [PSA], hypercapnic respiratory failure, a greater number of hospital admissions due to exacerbation of bronchiectasis, and a higher mortality compared with patients with restrictive or normal pulmonary functions