الفهرس 
Pulmonary Hypertension
PathophysiologyOnly 14 pages are availabe for public view
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Abstract
PH is a haemodynamic and pathophysiological disorder associated with a variety of cardiovascular and respiratory conditions, including congenital heart disease (CHD) but are all defined by a mean pulmonary arterial pressure of 25 mm Hg or greater at rest as assessed by right heart catheterization (Galiè et al., 2016).
The clinical classification of PH is intended to categorize multiple clinical conditions into five groups according to their similar clinical presentation, pathological findings, hemodynamic characteristics and treatment strategy (Simonneau et al., 2013).
Pulmonary arterial hypertension (PAH) is a complication of congenital heart disease, in turn, impacts on quality of life and survival of these patients (Chessa et al., 2017).
Our study was conducted on 34 patients with PAH-CHD presenting to congenital heart diseases unit at Ain Shams University hospital with first time to be diagnosed as PAH-CHD or for follow up. Each patient performed a 6MWT and was then offered an ECG gated echocardiography study for assessment of systolic and diastolic functions of right & left ventricles.The aim of the study is to determine the correlation between different Echocardiographic variables & functional capacity “6MWD” in patients with PAH-CHD and to detect the most independent predictor functional capacity.
The whole study population was divided according to the 6MW distance into two groups less than 330 and more than or equal 330 m.
Comparing the 6MWD of each patient with the different Echocardiographic variables of systolic & diastolic dysfunction revealed that the group of shorter 6MW distance had higher RVSP, MPAP, E/e’ ratio, LVMPI and RVMPI, while they had lower LV e’ TDI, LV GRS, RV FAC and RV GLS.
Multivariate analysis of these Echocardiographic variables revealed that MPAP is independent predictor of 6MWD & thus an independent predictor of functional capacity in PAH-CHD patients.
Further studies are still needed with larger number of subjects to ascertain the sensitivity & specificity of this model in prediction of functional capacity in PAH-CHD patients in everyday practice.