Noninvasive assessment of liver status in adult patients after the Fontan procedure

A hemodynamic derangement in Fontan circulation causes liver pathology known as Fontan‑associated liver disease. Although liver biopsy is a standard for diagnosis of liver fibrosis, noninvasive methods are being developed, including shear wave elastography (SWE). We aimed to evaluate the degree of liver stiffness (LS) using SWE in patients with Fontan circulation in a long‑term follow‑up and to investigate a relationship between patient characteristics and LS. The study included 59 patients after the Fontan procedure. Clinical examinations and laboratory tests were performed. According to the stage of LS, patients were divided into 2 subgroups: group 1 (METAVIR stages F1 and F2) and group 2 (METAVIR stages F3 and F4). The ratio of aspartate transaminase to alanine transaminase, aspartate transaminase‑to‑platelet ratio index (APRI), fibrosis‑4 (FIB‑4) score, and Forns index were assessed. The median LS was 9.1 kPa (interquartile range, 3.9–18.5 kPa). Five patients (9%) demonstrated LS in stage F1; 14 (26%), F2; 28 (52%), F3; and 7 (13%), F4. Group 2 had significantly higher aspartate transaminase and γ‑glutamyltranspeptidase levels, APRI, FIB‑4, and Forns index, and lower platelet count than group 1. A canonical correlation analysis indicated that LS and thrombocytopenia were related to time from the Fontan procedure, age at procedure, and single ventricular ejection fraction. We showed that adult patients after the Fontan procedure develop liver dysfunction. Time from surgery, age at procedure, and single ventricular ejection fraction are related to the degree of LS assessed by SWE. Finally, SWE, APRI, Forns index, and FIB‑4 score may help assess the degree of liver fibrosis

Clinical experience with 12-month follow-up in patients after implantation of a novel long-tapered sirolimus drug-eluting stent

Introduction: Long lesions contribute to a significant number of percutaneous coronary interventions. Aim: To assess the efficacy and safety of a novel long-tapered drug-eluting stent (DES) at a 12-month follow-up (FU) in patients with long coronary atherosclerotic lesions Material and methods: A prospective clinical cohort study was conducted in 32 patients who underwent percutaneous coronary intervention using a BioMime Morph tapered stent (Meril Life Sciences, India). The patients were followed for 3, 6, and 12 months. The safety endpoints were death, myocardial infarction (MI), target lesion revascularization (TLR), target vessel revascularization (TVR), and MACE and/or major bleeding. Results: Mean lesion length was 48 mm (range: 35–70 mm) measured via quantitative coronary analysis (QCA). In most cases, the target lesion was located in the LAD (68.75%). A GuideLiner catheter (Vascular Solutions Inc., MN, USA) was used in 12.5% of procedures; buddy-wire technique in 9.4% of cases. Bifurcation lesions were treated in 40.6% of cases. Additional stent implantation was needed in 56% of the procedures (25% of cases due to proximal or distal dissection, or due to insufficient stent length in 31% of cases). On 12-month FU we observed 1 TLR (3.1%), 1 TVR (3.1%), and 1 non-cardiovascular death. Conclusions: The long sirolimus-eluting stent with tapered structure was characterized by good deliverability in long coronary lesions, although in some cases “buddy wire” or extension microcatheter use was necessary. Follow-up at 3, 6, and 12 months showed no significant major adverse cardiovascular events related to the device

Wielonarządowe powikłania odległe po radioterapii i chemioterapii chłoniaka Hodgkina

Forty-nine-year-old woman with a history of long-term complications after radiotherapy and chemotherapy including coronary artery disease and pulmonary fibrosis was admitted to our center. Further complications — pulmonary hypertension, heart failure, and multiple heart valves insufficiency — were diagnosed. Here we present images from diagnostic procedures that allowed us to visualize those disturbances.49-letnia kobieta z historią odległych powikłań po radioterapii i chemioterapii, włącznie z chorobą wieńcową i zwłóknieniem płuc, została przyjęta do naszego ośrodka. Kolejne powikłania – nadciśnienie płucne, niewydolność serca i niedomykalność wielu zastawek serca - zostały zdiagnozowane. Prezentujemy tutaj zdjęcia z procedur diagnostycznych, które pozwoliły na zwizualizowanie tych nieprawidłowości

Chronotropic incompetence causes multiple organ complications in adults after the Fontan procedure

Background: Although patients undergoing the Fontan procedure (FP) present a normal or close‑to‑‑normal function of the systemic ventricle, they cannot generate cardiac output or exhibit similar exercise capacity as their healthy peers. This can be attributed to chronotropic incompetence and multiple organ complications. Aims: We evaluated the prevalence of chronotropic incompetence in adults after FP and assessed the relationship between heart rate reserve (HRR) and multiple organ complications. Methods: Data were obtained from 50 post‑FP patients (mean [SD] age, 27 [6.6] years) and 30 healthy controls matched for age and sex. All patients were subjected to clinical examination, laboratory tests, echocardiography, cardiopulmonary exercise test, and chronotropic function evaluation. Results: Cardiopulmonary exercise test parameters were impaired in the post‑FP group. Chronotropic incompetence was identified in 46 patients (92%), who also had a lower median (interquartile range) chronotropic index (0.55 [0.47–0.62] vs 0.93 [0.88–0.99]; P < 0.001) and a greater median (interquartile range) HRR (32 [24–60] bpm vs 8 [1–14] bpm, P < 0.001). A negative correlation was observed between HRR and peak oxygen uptake, and a positive one between HRR and the peak ventilatory equivalent for CO2 and mean platelet volume. The study revealed the diagnostic utility of HRR in detecting an abnormal peak ventilatory equivalent for O2, alkaline phosphatase levels, the ratio of aspartate transaminase to alanine transaminase levels, and mean platelet volume. Conclusions: Chronotropic incompetence correlates with impaired exercise capacity, liver dysfunction, and platelet abnormalities in post‑FP patients. Heart rate reserve may be a promising indicator of organ complications as well as a sign of future bradyarrhythmia and the need for cardiac pacing

Long-term follow-up in adults after tetralogy of Fallot repair

Abstract Background Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease and the population of ToF repair survivors is growing rapidly. Adults with repaired ToF develop late complications. The aim of this study was to describe and analyze long-term follow-up of patients with repaired ToF. Methods This is a retrospective cohort study. Consecutive 83 patients with repaired ToF who did not undergo pulmonary valve replacement were included. Mean age of all patients was 30.5 ± 10.7. There were 49 (59%) male. Patients were divided into two groups according to the time since the repair (< 25 years and ≥ 25 years). The electrocardiographic (ECG), cardiopulmonary exercise testing (CPET), echocardiographic and cardiac magnetic resonance (CMR) data were reviewed retrospectively. Results In CPET values were not significantly different in the two groups. In CMR volumes of left and right ventricles were not significantly different in the two groups. There were no differences between the groups in ventricular ejection fraction, mass of ventricles, or pulmonary regurgitation fraction. Among all the patients, ejection fraction and left and right ventricle mass, indexed pulmonary regurgitation volume measured by CMR did not correlate with the time since repair. In ECG among all the patients, ejection fraction of the RV, measured in CMR, negatively correlated with QRS duration (r = − 0.43; p < 0.001). There was a positive correlation between QRS duration and end diastolic volume of the RV (r = 0.30; p < 0.02), indexed end diastolic volume of the RV (r = 0.29; p = 0.04), RV mass (r = 0.36; p < 0.001) and left ventricle mass (r = 0.26; p = 0.04). Conclusion Long-term survival and clinical condition after surgical correction of ToF in infancy is generally good and the late functional status in ToF – operated patients could be excellent up to 25 years after the repair. QRS duration could be an utility and easy factor to assessment of right ventricular function. Trial registration The study protocol was approved by the local Ethics Committee. Each participant provided informed consent to participate in the study (license number 122.6120.88.2016 from 28.04.2016)