Łukasz Chrzanowski

The Aberrant Right Subclavian Artery (Arteria Lusoria): The Morphological and Clinical Aspects of One of the Most Important Variations—A Systematic Study of 141 Reports

The most important abnormality of the aortic arch is arguably the presence of an aberrant right subclavian artery (arteria lusoria). If this vessel compresses the adjacent structures, several symptoms may be produced. The aim of the study is to present the morphological and clinical aspects of the aberrant right subclavian artery. Three different databases searched for a review of pertinent literature using strictly predetermined criteria. Of 141 cases, 15 were cadaveric and 126 were clinically documented. The gender distribution of the subjects was 55.3% female and 44.7% male. The mean age of the patients at symptoms onset was 49.9 ± 19.4 years for all patients but 54.0 ± 19.6 years and 44.9 ± 18.1 years for female and male subjects, respectively (P = 0.0061). The most common symptoms in this group were dysphagia (71.2%), dyspnea (18.7%), retrosternal pain (17.0%), cough (7.6%), and weight loss (5.9%). The vascular anomalies coexisting with an arteria lusoria were truncus bicaroticus (19.2%), Kommerells diverticulum (14.9%), aneurysm of the artery itself (12.8%), and a right sided aortic arch (9.2%). In conclusion, compression of adjacent structures by an aberrant right subclavian artery needs to be differentiated from other conditions presenting dysphagia, dyspnea, retrosternal pain, cough, and weight loss.

Severe Obesity Impairs Systolic and Diastolic Heart Function – The Significance of Pulsed Tissue Doppler, Strain, and Strain Rate Parameters

We assessed the impact of morbid obesity on systolic and diastolic heart function in severely obese, but otherwise healthy subjects and age‐matched controls.

Genetic variants in a Polish population of patients with pulmonary arterial hypertension: sequencing of BMPR2, ALK1, and ENG genes

BACKGROUND Pulmonary arterial hypertension (PAH) is a rare disease with a very serious prognosis. It seems that mutations in genes related to transforming growth factor-b signalling pathway are often related to the development of the disease. No study covers this problem in a Polish population. AIM To screen for genetic mutations in a Polish cohort of patients with pulmonary hypertension, especially with idiopathic PAH, treated in a single hospital in Poland. METHODS DNA sequencing method was used. Samples from 50 patients with pulmonary hypertension were screened for mutations in type 2 bone morphogenetic protein receptor of the transforming growth factor-b superfamily gene (BMPR2). Samples from 20 patients with idiopathic PAH (11 men, mean age 55 years) were also screened for mutations in activin A receptor-like type 1 gene (ALK1) and endoglin gene (ENG). RESULTS No genetic variations were found for the BMPR2 gene. In all 20 samples from idiopathic pulmonary hypertension patients we found heterozygosity of single nucleotide polymorphism (SNP) rs 372023206 in ALK1 gene. Three samples from these patients showed variations of ENG gene: we found one sample with heterozygosity of SNP rs 200525684, one with heterozygosity of SNP rs 3739817, and one with both. CONCLUSIONS We detected benign polymorphisms or genetic variants of unknown importance. It is possible that the Polish population of PAH patients differs from the previously described populations of other countries in terms of the frequency and importance of mutations in BMPR2, ALK1 and ENG genes.

A statement the Polish Cardiac Society Working Group on Pulmonary Circulation on screening for CTEPH patients after acute pulmonary embolism

Both pharmacological and invasive treatment of chronic thromboembolic pulmonary hypertension (CTEPH) is now available in Poland and the awareness of the disease among physicians is growing. Thus, the Polish Cardiac Societys Working Group on Pulmonary Circulation in cooperation with independent experts in this field, have launched the statement on algorithm to guide a CTEPH diagnosis in patients with previous acute pulmonary embolism (APE). In Poland, every year this disease affects about 250 patients. CTEPH should be suspected in individuals after APE with dyspnea, despite at least 3 months period of effective anticoagulation, particularly when specified risk factors are present. Echocardiography is a main screening tool. The authors suggest that a diagnostic process of patients with significant clinical suspicion of CTEPH and right ventricle overload in echocardiography should be performed in reference centres. The document contains a list of Polish centres diagnosing patients with suspected CTEPH. Pulmonary scintigraphy is a safe and highly sensitive screening test for CTEPH. Multi-detector computed tomography with precise detection of thromboembolic residues in pulmonary circulation is important for planning of pulmonary endarterectomy. Right heart catheterisation definitely confirms the presence of pulmonary hypertension and direct pulmonary angiography allows for identification of lesions suitable for thromboendarterectomy or pulmonary balloon angioplasty. In this document a diagnostic algorithm in patients with suspected CTEPH is also proposed. With individualised sequential diagnostic strategy each patient can be finally qualified for a particular mode of therapy by dedicated CTEPH Heart Team. Moreover the document contains short information for the primary care physician about the management of patients after APE.

Advanced and traditional electrocardiographic risk factors in pulmonary arterial hypertension: the significance of ventricular late potentials

BACKGROUND Whether non-invasive electrocardiographic parameters may be of similar relevance in pulmonary arterial hy-pertension (PAH) as in left ventricular heart failure (LVHF) remains unclear. AIM To examine a profile of electrocardiographic parameters in PAH and to determine their prognostic significance. Com-parison of profile in patients with pulmonary hypertension secondary to left ventricular dysfunction was planned in order to put PAH group results into context. METHODS We included 41 patients with type 1.1/1.4.4 (according to the European Society of Cardiology) PAH and 31 patients with LVHF and type 2.1 pulmonary hypertension. All patients underwent 24-h ambulatory electrocardiography monitoring. RESULTS Among heart rate variability parameters, only RMSSD was different (mean, 75 ms [PAH] vs. 112 ms [LVHF], p = 0.016). In PAH, fewer patients had ventricular tachycardia (15% vs. 48%, p = 0.004), abnormal deceleration capacity (54% vs. 84%, p = 0.011), positive heart rate turbulence (11% vs. 48%, p = 0.003), severe autonomic failure (10% vs. 39%, p = 0.005), and ventricular late potentials (LP) (19% vs. 62%, p = 0.001). In PAH, four deaths occurred in 42 months. In univariate analysis, the risk factors for death were: LP (hazard ratio 13.55, 95% confidence interval 1.41-130.72; p = 0.024), age, N-terminal prohormone of B-type natriuretic peptide, while the protective factors were minimal and mean heart rate, as well as the six-minute walk test (6MWT) distance. In multivariate analysis, the influence of LP and the 6MWT distance remained significant. CONCLUSIONS Ventricular LP were present in 19% of PAH patients and were the most powerful risk factor of mortality .