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Pulmonary arterial hypertension is a rare but progressive disease that leads to death. Modern drug treatment slows the progression of the disease and prolongs patients' lives, but often, even maximal treatment with parenteral prostacyclin does not prevent deterioration. In the case of inadequate clinical response to drug treatment, lung transplantation (LTx) should be considered. This article aims to analyze thoroughly indications to refer a patient for consultation with a transplant center, the optimal timing of listing for LTx, contraindications for the procedure, bridging techniques, as well as tests needed before and after transplantation. We outline the technique of the procedurę and evaluate psychological aspects of LTx.

Ischemic stroke is one of the major causes of death and disability in high-developed countries. Closure of PFO is recommended if other causes of ischemic stroke, like atrial fibrillation, were excluded, especially in young patients. We present a case report of a 75-years-old female patient with five ischemic strokes in her medical history and newly diagnosed patent foramen ovale. Atrial fibrillation was detected 25 years after first ischemic event. Implementation of anticoagulation therapy has prevented new ischemic strokes in our patient. According to emerging meta-analyses such treatment is sufficient not only in AF, but also in PFO related ischemic strokes. The patient had another risk factors for paradoxical embolism such as varices, post-thrombotic syndrome of lower limbs and the Eustachian valve, so it is unclear whether AF has been the major cause of all ischemic stroke in present case. Significant bleeding from limb varices during anticoagulation treatment occurred and required urgent surgical intervention. Because of the high risk of recurrent haemorrhages (HAS-BLED Score – 4 points), the patient was considered to two percutaneous procedures: occlusion of the left atrial appendage (LAA) and consecutively second - PFO closure as additional prevention of stroke. This is an illustrative case that opens discussion on necessity and timing of cardiac interventions once possible cardiac sources of ischemic strokes are found and new facts arise.

BACKGROUND: Balloon pulmonary angioplasty (BPA) is a promising therapy for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are ineligible for pulmonary endarterectomy. AIMS: The present study aimed to evaluate the safety and efficacy of BPA for CTEPH using the first multicentre registry of a single European country. METHODS: Data were obtained from the Database of Pulmonary Hypertension in the Polish Population (NCT03959748), a prospective, multicentre registry of adult and paediatric pulmonary arterial hypertension (PAH) and CTEPH, for a total of 236 patients with confirmed CTEPH (124 women; mean age 67 years) who underwent 1,056 BPA procedures at eight institutions in Poland. RESULTS: In 156 patients who underwent follow-up assessments after a median of 5.9 (IQR: 3.0-8.0) months after final BPA, the mean pulmonary arterial pressure decreased from 45.1±10.7 to 30.2±10.2 mmHg (p

Pulmonary arterial hypertension (PAH) is a rare disease with a serious prognosis. The aim of this study was to identify biomarkers for PAH in the breath phase and to prepare an automatic classification method to determine the changing metabolome trends and molecular mapping. A group of 37 patients (F/M: 8/29 women, mean age 60.4 ± 10.9 years, BMI 27.6 ± 6.0 kg/m2) with diagnosed PAH were enrolled in the study. The breath phase of all the patients was collected on a highly porous septic material using a special patented holder PL230578, OHIM 002890789-0001. The collected air was then examined with gas chromatography coupled with mass spectrometry (GC/MS). The algorithms of Spectral Clustering, KMeans, DBSCAN, and hierarchical clustering methods were used to perform the cluster analysis. The identification of the changes in the ratio of the whole spectra of biomarkers allowed us to obtain a multidimensional pathway for PAH characteristics and showed the metabolome differences in the four subgroups divided by the cluster analysis. The use of GC/MS, supported with novel porous polymeric materials, for the breath phase analysis seems to be a useful tool in selecting bio-fingerprints in patients with PAH. The four metabolome classes which were obtained constitute novel data in the PAH population.

Introduction The study aimed to evaluate the prevalence and predictors of left ventricular (LV) reverse remodeling and its impact on long-term prognosis in patients with anterior ST-segment elevation myocardial infarction (STEMI). Aim To assess the percentage of reverse remodeling and its prognostic factors in anterior STEMI patients. Material and methods This observational study included 40 patients with first ever STEMI of the anterior wall. LV reverse remodeling was defined as the reduction of left ventricular end-systolic volume (ΔLVESV) by ≥ 10% in 3D transthoracic echocardiography (3D-TTE) at 3-month follow-up. 3D-TTE and speckle tracking imaging were performed during index hospitalization, while 3D-TTE and cardiac magnetic resonance (CMR) were performed at 3 months following the procedure. Patients were followed up for a median time of 3.4 years in order to evaluate major adverse cardiovascular events. Results Left ventricular reverse remodeling at 3-month follow-up was confirmed in 15 (37.5%) patients. The presence of reverse remodeling was predicted by lower troponin levels (unit OR = 0.86, p = 0.02), lower sum of ST-segment elevations before (unit OR = 0.87, p = 0.03) and after PCI (unit OR = 0.40, p = 0.03), lower maximal ST-segment elevation after PCI (unit OR = 0.01, p = 0.03), lower wall motion score index (unit OR 0.40, p = 0.03) and more negative anterior wall global longitudinal strain (unit OR = 0.88, p = 0.045). Nine MACE were reported in the without reverse remodeling group only. Non-significantly better event-free survival in the reverse remodeling group was demonstrated (log-rank p = 0.07). Conclusions Development of reverse modeling in patients with optimal revascularization and tailored pharmacotherapy is relatively high. Further studies are warranted in order to adjudicate its prognostic role for the prediction of adverse events.

Systemic connective tissue diseases (CTDs) comprise a large group of diseases that are auto-immune in nature and characterized by the involvement of multiple systems and organs. Pul-monary hypertension (PH) of various etiologies may develop in the course of CTD, including pulmonary arterial hypertension (PAH), PH secondary to the lung disease, postcapillary PH in the course of left heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH). In addition, the different forms of PH may coexist with each other. Among patients with CTD, PAH occurs most commonly in those with systemic sclerosis, where it affects ap-proximately 8%-12% of patients. The prognosis in patients with untreated PAH is very poor. It is particularly important to identify the high-risk CTD-PAH population and to perform effi-cient and accurate diagnostics so that targeted therapy of the pulmonary arteries can be intro-duced. Echocardiography is used to screen for PH, but clinical and echocardiographic suspicion of PH always requires confirmation by right heart catheterization. Confirmation of PAH ena-bles the initiation of life-prolonging pharmacological treatment in this group of patients, which should be administered in referral centers. Drugs available for pharmacological management include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins.

Current knowledge of pulmonary arterial hypertension (PAH) epidemiology is based mainly on data from Western populations, and therefore we aimed to characterize a large group of Caucasian PAH adults of Central-Eastern European origin. We analyzed data of incident and prevalent PAH adults enrolled in a prospective national registry involving all Polish PAH centers. The estimated prevalence and annual incidence of PAH were 30.8/mln adults and 5.2/mln adults, respectively and they were the highest in females &ge, 65 years old. The most frequent type of PAH was idiopathic (n = 444, 46%) followed by PAH associated with congenital heart diseases (CHD-PAH, n = 356, 36.7%), and PAH associated with connective tissue disease (CTD-PAH, n = 132, 13.6%). At enrollment, most incident cases (71.9%) were at intermediate mortality risk and the prevalent cases had most of their risk factors in the intermediate or high risk range. The use of triple combination therapy was rare (4.7%). A high prevalence of PAH among older population confirms the changing demographics of PAH found in the Western countries. In contrast, we found: a female predominance across all age groups, a high proportion of patients with CHD-PAH as compared to patients with CTD-PAH and a low use of triple combination therapy.

Background Left ventricular remodeling (LVr) is still common after ST-segment elevation myocardial infarction (STEMI). Early predictors of remodeling are being investigated. The aims of this study were to evaluate the prognostic value of speckle-tracking echocardiography for the prediction of LVr 3 months after primary percutaneous coronary intervention in patients with STEMI and to analyze the relationship between values of peak longitudinal strain of particular LV segments and relative changes of their subvolumes. Methods Patients with first STEMI were enrolled. Baseline enzymes were collected, and electrocardiography and echocardiography (transthoracic echocardiography, speckle-tracking echocardiography, and three-dimensional studies) were preformed. Three months after myocardial infarction, two-dimensional and three-dimensional ultrasonographic studies were done. Results Sixty-six patients were divided into two groups: 44 patients without LVr and 22 patients with LVr. Among 31 patients with anterior wall STEMI, the rate of LVr was 42%. On the basis of assessments of baseline and follow-up myocardial wall contractility, 1,041 segments were analyzed. All segments were divided into normal ( n = 842), reversibly dysfunctional ( n = 68), and irreversibly dysfunctional ( n = 131). Receiver operating characteristic curve analysis showed that global longitudinal strain predicted LVr with an optimal cutoff value of −12.5% (area under the curve, 0.77). In multivariate analysis, diabetes mellitus (odds ratio, 4.61; 95% confidence interval, 1.19–18.02) and global longitudinal strain (odds ratio, 1.19; 95% confidence interval, 1.04–1.37) were determinants of LVr. Positive correlations were found between peak longitudinal strain and changes in subvolumes for all segments ( R = 0.11, P = .005) and for those irreversibly dysfunctional ( R = 0.22, P = .04). Conclusions In patients with STEMI treated by primary percutaneous coronary intervention, the frequency of LVr during 3-month follow-up was high and mainly affected the population with anterior wall myocardial infarction. The results of this study show the clinical value of global longitudinal strain measured by speckle-tracking echocardiography in the prediction of LVr. A moderate correlation was found between the value of peak longitudinal strain and changes in subvolumes attributed to irreversibly dysfunctional segments.

Background: Efficacy of pulmonary arterial hypertension (PAH)-specific therapy may differ among the patients depending on the PAH aetiology. Aim: To compare the real‑life efficacy of PAH‑specific therapy between non‑congenital heart disease (non‑CHD) and CHD groups of PAH patients and to determine whether an early clinical response has an impact on prognosis. Methods: Clinical data from 41 PAH patients, 21 non‑CHD and 20 CHD patients, were included in the study. The WHO functional class (WHO‑FC), 6‑minute walk distance (6MWD) and NT‑proBNP serum level were compared at baseline and after 1 and 7 months of PAH‑specific treatment. Only patients with unmodified PAH‑specific therapy during 7‑month follow‑up were enrolled in the study. Results: Baseline characteristics revealed higher WHO‑FC and increased [loge]NT‑proBNP levels (7.74 ±1.05 vs 6.51 ±1.48; p = 0.008) in non‑CHD vs. CHD patients; baseline 6MWD was similar in both groups (283.3 ±148.5 m vs 339.2 ±114.7 m). Clinical improvement by at least one WHO‑FC after 1‑month treatment was observed more frequently in non‑CHD (55%) when compared with CHD patients (25%, p = 0.04) and was comparable (50% vs 50%) after 7‑month observation. Non‑CHD patients, who did not improve within 1 month of treatment were unlikely to achieve improvement after 7 months. The 6MWD increased during the first month of treatment in non‑CHD (p=0.009) and in CHD patients (p=0.006) when compared to baseline values and remained at this level after 7 months of treatment. [Loge]NT‑proBNP levels markedly declined only in non‑CHD patients, who had an improvement in WHO‑FC (8.0 ±1.0 vs 7.4±1.1, p=0.04) in the first month. In CHD patients, the decrease in [loge]NT‑proBNP level was seen (6.5 ± 1.5 vs 6.1 ±1.5, p=0.04) only within a 1‑month observation. Conclusion: Efficacy of 1‑month PAH‑specific therapy is aetiology‑dependent and determines clinical outcome in patients with PAH. JRCD 2018; 3 (6): 199–204

Right heart catheterisation (RHC) is the ‘gold standard’ for haemodynamic assessment of the pulmonary circulation. For the diagnosis of pulmonary hypertension (PH), the guidelines of the European Society of Cardiology require a mean pulmonary arterial pressure ≥ 25 mm Hg to be confirmed by direct haemodynamic measurement. Additionally, RHC provides a lot of valuable information about the differential diagnosis and severity of PH, and also helps determine the patient’s prognosis. Acute vasoreactivity testing performed in patients with pulmonary arterial hypertension is intended to identify the group of patients who should be treated with calcium channel blockers. Patients referred for heart transplantation require advanced pulmonary vascular disease to be ruled out either on resting examination or during vasoreactivity testing. RHC is a component of such interventional procedures as balloon atrial septostomy, closure of intracardiac shunts in congenital heart and great vessel defects, valvuloplasty for pulmonary valve stenosis, and pulmonary angioplasty. Pulmonary angiography is an examination recommended when selecting patients for pulmonary endarterectomy or balloon pulmonary angioplasty in thromboembolic PH. Due to the dynamic growth in the number of patients diagnosed with and treated for PH in Poland, the Boards of the Polish Cardiac Society’s Working Group on Pulmonary Circulation and Association of Cardiovascular Interventions have undertaken a joint project to develop recommendations to standardise guidelines for RHC procedure, acute vasoreactivity testing and pulmonary angiography at cardiac wards and haemodynamic laboratories in Poland. This document has been prepared by experts delegated by the Working Group on Pulmonary Circulation and the Association of Cardiovascular Interventions, and subsequently approved by the Boards of both organs of the Polish Cardiac Society.

A 32-year-old woman with pulmonary artery atresia with ventricular septal defect (VSD) was admitted because of New York Heart Association (NYHA) class worsening from II to III during the last 6 months. The patient presented resting cyanosis, short 6-minute walk test (6MWT) distance (370 m with desaturation – baseline: 84%, after 6MWT: 67%). N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level was 194–262 pg/ml. She also suffered from significant kyphoscoliosis with stable chronic ventilation disturbances: forced vital capacity (FVC) = 1.38 l, forced expiratory volume in 1 s (FEV1) = 0.94 l, FEV1/FVC = 68%. Imaging methods (transthoracic echocardiography, transoesophageal echocardiography, magnetic resonance imaging – MRI, angio-computed tomography – CT) confirmed the presence of VSD (Figures 1 A, B), enlarged ascending aorta up to 49 mm above VSD and moderate aortic valve insufficiency. Angio-CT and MRI revealed occurrence of major aortopulmonary collaterals (MAPCAs) (Figure 1 C) with a giant aneurysm of one of them (Figure 1 D) and no pulmonary artery. Coronarography showed abnormality of the left artery descdending (LAD) orifice from the right coronary artery. We simultaneously excluded coronary and mammary artery to MAPCA shunts. The aortography followed by selective catheterization showed five MAPCAs: three on the left side (the one that supplied the left lower pulmonary lobe was stenosed in the proximal part) and two on the right side, among others a MAPCA creating a giant (6 cm) aneurysm (Figure 1 E). Each individual part of the lungs is supplied from a single source. In catheterization using a Swan-Ganz catheter we obtained the following outcomes: the mean collateral pressure was high in the MAPCAs that were not stenosed (69 mm Hg) and there was no response to iloprost (Figure 1 F); the mean collateral pressure was quite low in the stenosed MAPCA (14 mm Hg). According to single case reports and limited research [1, 2] and our hemodynamic outcomes, we prescribed bosentan, with quite a good clinical effect. During bosentan therapy the patient’s NYHA class decreased from III to II. The patient was disqualified from surgical treatment. In the literature we have found some reports of large MAPCA aneurysm diagnosed in vivo but not surgically treated [3] and one case concerning successful invasive treatment [4]. During 2-years follow-up we increased the dose of bosentan to 125 mg twice daily and we obtained a very good result. The patient is still in NYHA functional class II without any progression of pulmonary hypertension symptoms. There still remains the question whether the above described MAPCA aneurysm should be percutaneously occluded or surgically treated in prevention of aneurysm rupture and sudden death. Taking into account coexisting kyphoscoliosis and ventilation disturbances, the risk of interventions seems to be very high. Figure 1 Multimodality imaging: A – transthoracic echocardiography – parasternal long axis view, arrow: large VSD; B – CT scan, arrows: MAPCAs; C – CT scan, 3D reconstruction, arrow: MAPCA; D – CT scan, arrows: MAPCAs, arrow: ...

We sought to determine the frequency of left ventricular remodeling in the 6-month follow-up after anterior ST elevation myocardial infarction and the value of quantitative parameters of perfusion contrast echocardiography for prognosis of left ventricular remodeling against other established risk.A total of 60 patients with anterior ST elevation myocardial infarction treated by primary percutaneous intervention were examined. In 28 patients, thromboaspiration was performed before stent implantation with Driver catheter. Before and after successful angioplasty, perfusion in myocardial blush grade (MBG) scale was assessed. Various electrocardiogram parameters were analyzed. Resting perfusion with myocardial contrast echocardiography was performed.Logistic regression has permitted one to conclude that higher value of MBG, higher left ventricular ejection fraction at discharge, and higher value of parameter A at quantitative echocardiography in dysfunctional segments were prognostic for lack of remodeling over 6 months. The receiver operating characteristics curves for parameters of quantitative perfusion echocardiography (A, β, A×β) allowed us to conclude that value A1.96 dB, value β0.155 s, and value A×β0.57 dB/s are optimal cut-off points prognostic for remodeling. Area under the curve was 0.8 for A and 0.85 for β.The best predictors of remodeling in 6 months' observation have appeared to be lower left ventricular ejection fraction at discharge, poorer perfusion assessed angiographically (MBG scale), and the rate of signal intensity increase reflecting the mean bubble velocity of the myocardium by contrast as assessed by contrast echocardiography. Quantitative perfusion angiography independently has high predictive value for the development of remodeling in long-term follow-up.

Complete atrioventricular block can occur, among others, in case of hyperkalemia, that is a life-threatening complication of rhabdomyolysis. Statins constitute the medication group that is especially associated with a possibility of muscle complications. Frequency of statin-associated myalgia is 5-10%, and potentially fatal rhabdomyolysis--0.02-0.09%. We describe a male patient who was admitted due to syncope caused by complete atrioventricular block. Iatrogenic rhabdomyolysis with life-threatening hyperkalemia, that was related to statin, was diagnosed. After application of suitable pharmacotherapy, conduction abnormalities resolved. The patient was discharged in a good condition.

Introduction A key method in the treatment of ST-elevation myocardial infarction (STEMI) is recanalization of the infarct-related artery, but this causes heart reperfusion injury. One of the methods to reduce this injury is postconditioning. The available data on the efficacy of this method are contradictory. Objectives The aim of the study was to determine the safety of postconditioning as well as its effect on infarction size, improvement in left ventricular ejection fraction (LVEF), and adverse LV remodeling during a 3-month follow-up. Patients and methods The study involved 39 patients with first anterior STEMI (aged 58 ± 10 years) up to 12 hours from the onset of symptoms. They were randomly assigned to a traditional-reperfusion group (n = 21) or to a postconditioning group (n = 18). The area at risk (AAR) was assessed angiographically. LV remodeling and LVEF were evaluated using echocardiography at 6 days and at 3 months. The infarction size was defined on the basis of magnetic resonance imaging (MRI) at 3 months. Results In a univariate logistic regression analysis, postconditioning did not affect the improvement of LVEF (odds ratio [OR], 1.63; 95% confidence interval [CI], 0.34-7.7; P = 0.52) or the development of adverse LV remodeling (OR, 0.62; 95% CI, 0.15-2.53; P = 0.5). Moreover, there were no significant differences in infarction size between the groups as measured by MRI after adjustment for the AAR, time to reperfusion, and ST-segment elevation prior to percutaneous coronary intervention. Conclusions Postconditioning is a safe method but its application did not affect the volume of the infarction as well as did not improve LVEF or the development of adverse LV remodeling in a 3-month follow-up.