ABSTRACT
Background: The development of pulmonary hypertension i.e.mean pulmonary artery pressure (mPAP) above 25 mmHg withnormal capillary wedge pressure and pulmonary vascularresistance(PVR) above 240 dyn/s/cm−5 in association withelevated pressure in portal circulation is known asportopulmonary hypertension (POPH). Comparing withidiopathic PAH, patients with POPH have a worst survivalprofile, with a 3-year survival of only 38% versus78% foridiopathic PAH. Recent evidence from France shows thatPOPH is the fourth most common form of PAH reported overallin the population-based French National Registry, afteridiopathic PAH and PAH associated with connective tissuediseases and con- genital heart disease. The aim of this studyis to evaluate frequency of POPH in portal hypertensivepatient.Materials and Methods: A cross sectional study of patientadmitted in RIMS, medicine department was performedfulfilling features of portal hypertension with ultrasoundshowing splenomegaly, ascites, portal vein diameter more than13 mm, portal vein velocity less than 15 cm/s and uppergastrointestinal endoscopy showing esophageal varices andpatient with connective tissue disease, congenital heartdisease, left ventricular systolic or diastolic dysfunction,valvular heart disease, lungs disease, sleep related breathingdisorder, chronic hemolytic and myeloproliferative disorderwere excluded. All patient underwent screening withechocardiography for measuring pulmonary artery systolicpressure (PASP) and PASP more than 35 mmHg wereconsidered for POPH which was confirmed with right heartcatheterisation by measuring mean pulmonary artery pressure(mPAP) of more than 25 mmHg.Observation: Among forty-two patient in this study, there werethirty-three male patients and nine female patients. POPH wasseen three female and two male patients with total of five out offorty- two with prevalence of 11.9% out of which 7.1% werefemale and 4.8% were male.Conclusion: Portopulmonary hypertension prevalence is 2–6%. In this study pulmonary hypertension is significantly high inportal hypertensive patient with percentage of 11.9% and moreprevalent in female.
ABSTRACT
Pulmonary arterial hypertension (PAH) is often difficult to diagnose and challenging to treat. Untreated, it is characterized by a progressive increase in pulmonary vascular resistance leading to right ventricular failure and death. The past decade has seen remarkable improvements in therapy, driven largely by the conduct of randomized controlled trials. Still, the selection of most appropriate therapy is complex, and requires familiarity with the disease process, evidence from treatment trials, complicated drug delivery systems, dosing regimens, side effects, and complications. We tried to provide evidence?based treatment recommendations for physicians involved in the care of these complex patients. Due to the complexity of the diagnostic evaluation required, and the treatment options available, it is strongly recommended that consideration be given to referral of patients with PAH to a specialized center.