Patients: 32 patients, mean age 32.5 years, 21 female, and 11 male. Intervention: Transthoracic (TTE) and transoesophageal (TOE) echocardiographic examination in patients with suspected DCRV. Main outcome measures: Direct inspection during surgical treatment of 28 patients diagnosed as having DCRV as an isolated lesion or associated with other pathologies. Results: Echocardiography allowed the final diagnosis of DCRV in 26 patients (81%) out of 32 studied. TTE was diagnostic in 5 (15.6%) whereas TOE was diagnostic in 21 of 21 studied by this technique. Of 6 patients with negative TTE, DCRV was identified by cardiac catheterisation in 3 and directly during surgery in the remaining 3. Of 26 patients diagnosed by echocardiography, the anomalous muscle bundle was discrete in 20 (77%) and diffuse in 6 (23%). In 23 patients (88%) right ventricular outflow obstruction was localised low in the right ventricle, and in the remaining 3 (11.5%), the obstruction was localised high in the right ventricle. Abnormal bundles localised high were discrete, bundles localised in the lower part of right ventricle were discrete in 17 (74%) and diffuse in the remaining 6 (26%). DCRV was an isolated lesion in only 2 patients (6.2%). In all subjects surgical inspection confirmed echocardiographic data. Conclusions: Echocardiography was very useful to diagnose DCRV in adults as well as to identify its anatomic type. Comparing two different approaches, TOE better defines the entire scope of pathology, including estimation of the resultant systolic pressure gradient within the right ventricular cavity.

Myocardial perfusion defects are common findings in patients in long-term follow-up after atrial switch operation. Despite excellent exercise tolerance, the extent of myocardial perfusion abnormalities correlated well with impaired RV and LV function, and greater perfusion defects were seen more frequently in older patients with longer follow-up. It is likely that myocardial perfusion defects could be a sensitive predictor of systemic ventricular impairment.

Adult Fontan patients are characterized by enhanced platelet activation and endothelial injury, heightened thrombin formation, and impaired fibrinolysis. Patients showed reduced free protein S levels, increased platelet activation, and endothelial damage after thromboembolic events observed late after Fontan surgery. Our findings indicate novel prothrombotic mechanisms in adult Fontan patients, which might help to optimize thromboprophylaxis.

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 ≥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.

(i) ARVD/C may be complicated by thrombosis. Annual incidence of such complications is significantly lower than reported for left ventricle failure. (ii) Anticoagulation should be used in ARVD/C patients with large, hypokinetic RV and slow blood flow. (iii) Patients with severe forms of ARVD/C, thrombus formation in the RV and/or spontaneous echocardiographic contrast are at higher risk of a poor outcome.