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Background: The significant role of mitral regurgitation (MR) in development of pulmonary hypertension (PH) has been proved in previous studies. Experts suggest systolic pulmonary arterial pressure (SPAP) ⩾60 mmHg during exercise as a significant threshold of negative prognostic value in patients with MR. Purpose: The aim of this study was to evaluate the changes of SPAP and to ascertain the determinants of exercise induced pulmonary hypertension (EIPH) in patients with asymptomatic primary MR. Methods: We performed a prospective study that included 50 patients with asymptomatic primary moderate to severe MR with preserved left ventricular ejection fraction (LV EF ⩾60%) at rest. They were divided into two groups according to the presence (PH group; n = 13) or absence (non-PH group; n = 37) of EIPH. Rest and stress (bicycle ergometry) echocardiography and speckle-tracking offline analysis were performed. Results: An increment of SPAP from rest to peak stress was higher in PH group ( p 33.1 mmHg at rest could predict EIPH with 84.6% sensitivity and 87.1% specificity (95%CI 0.849–1.000; p Conclusions: Parameters of MR severity (EROA and RVol) were significant determinants of SPAP at rest, while the increment of MR EROA during stress and parameters of resting LV diastolic function were the best predictors of significant EIPH.

Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) associated with congenital heart disease. It is an extremely devastating condition with a serious impact on patients' life. Classical therapy of ES remains directed to avoid complications, such as erythrocytosis, treatment of congestive heart failure, prevention of infection, and secondary haematological abnormalities such as iron deficiency and coagulation disorders. However, the only effective treatment is heart-lung transplantation; still, morbidity and mortality after transplantation remain substantially high. Furthermore, waiting lists for heart-lung transplantation are long. Recent studies examining the use of advanced medical treatment in patients with ES have shown that it may have beneficial effects in patients with ES; however, additional studies need to be done to confirm its efficacy and appropriate clinical use. A 41-year-old female admitted to the Hospital of Lithuanian University of Health Sciences due to progressive dyspnea on minimal effort, heart failure symptoms leading to NYHA functional class III-IV. After clinical and instrumental investigations, ES secondary to unrepaired patent ductus arteriosus with severe PAH was diagnosed. Treatment with sildenafil was initiated together with the standard pharmacological therapy, and the patient was added to the waiting list for the heart and lung transplantation. After 24 months of stable condition, her clinical status deteriorated, and combination therapy (sildenafil and ambrisentan) was initiated. Clinical symptoms and exercise capacity improved, and she has been stable for 4 years thereafter. Our experience of the management of an adult patient with ES showed the benefits of treatment with advanced therapy with pulmonary vasodilators that improved the patient's quality of life and delayed the need for heart and lung transplantation.

Background: Severe aortic stenosis (AS) complicated by pulmonary hypertension (PH) is associated with poor outcomes after surgical aortic valve replacement (AVR). There is still scarce information about predictors of secondary PH in this group of patients. Objectives: The aim of this study was to investigate the prognostic impact of biomarkers together with conventional Doppler echocardiographic parameters of left ventricular diastolic function on elevated pulmonary systolic pressure (PSP) in severe AS patients before surgical AVR. Methods: Sixty patients with severe isolated AS (aortic valve area &lt, 1 cm2) underwent echocardiography, N-terminal pro B-type natriuretic peptide (NT-proBNP) and growth differentiation factor-15 (GDF-15) measurements before AVR. PSP, left ventricular ejection fraction (LV EF), parameters of LV diastolic function (E/E&rsquo, ratio, mitral valve deceleration time (MV DT) and left atrial (LA) volume) were evaluated. PH was defined as an estimated PSP &ge, 45 mmHg. Results: Of the 60 patients, 21.7% with severe isolated AS had PH with PSP &ge, 45 mmHg (58.5 &plusmn, 11.2 mmHg). LV EF did not differ between groups and was not related to an elevated PSP (50 &plusmn, 8 vs. 49 &plusmn, 8%, p = 0.58). Parameters of LV diastolic dysfunction (E/E&rsquo, ratio &gt, 14 (OR 6.00, 95% CI, 1.41&ndash, 25.48, p = 0.009), MV DT &le, 177.5 ms (OR 9.31, 95% CI, 2.06&ndash, 41.14, p = 0.001), LA volume &gt, 100 mL (OR 9.70, 95% CI, 1.92&ndash, 49.03, p = 0.002)) and biomarkers (NT-proBNP &gt, 4060 ng/L (OR 12.54, 95% CI, 2.80&ndash, 55.99, p &lt, 0.001) and GDF-15 &gt, 3393 pg/mL (OR 18.33, 95% CI, 2.39&ndash, 140.39, p = 0.001)) were significantly associated with elevated PSP in severe AS. Conclusions: Left ventricular diastolic dysfunction and elevated biomarkers levels could predict the development of pulmonary hypertension in patients with severe aortic stenosis. Elevation of biomarkers paired with worsening of LV diastolic dysfunction could help to stratify patients for earlier surgical treatment before the development of pulmonary hypertension.