GUCH POPULATION: CARDIOLOGIC AND SURGICAL DEMANDS

To evaluate the cardiologic and surgical demands of the GUCH population followed up in our Department, a retrospective analysis of 709 hospital admissions (HA) of 536 pts (mean age 34?16, range 16-79 years), carried out from 1997 to 2002, has been performed. In 293 pts the basic cardiopathy was simple (SC), in 158 of moderate complexity (MCC) and in 85 of great complexity (GCC). The reason for 169 HA was cardiac surgery and for 537 HA cardiologic issues. The overall HA/pt was 1.3 (1.1 in SC,1.3 in MCC, 2.1 in GCC). In 166 surgical pts, 171 operations were performed. First operations (n=105, 62%) were most commonly for atrial septal defect (n=50, 48%), aortic valve replacement(n=11,10%), ventricular septal defect (n=8, 8%). Reoperations (n=64, 38%) were divided among reintervention after corrective repair (n=45, 70%), further palliation (n=17, 26%), first palliation (n=2, 4%) and the most frequent were: Fontan operation (n=11, 17%), RV-PA conduit replacement (n=8, 12%), aortic valve replacement (n=6, 9%). The mean hospital stay was 19?17 days. Pleuro-pericardial effusion (n=32,18%), bleeding (n=11, 6%) and arrhythmias(n=11, 6%) were the most prevalent complications. Early mortality (n=7/171, 4%) was influenced by cyanosis (6 of 33,18% in cyanotic pts; 1 of 136, 2% in acyanotic, p<0,001) and by complexity of cardiopathy (n=6 of 43 GCC, 14%; n=1 of 72 MCC, 1%; 0 of 54 SC,0%, p<0.001) that was a risk factor also for more serious complications (20% in GCC, with respect of 9% in MCC, p<0,05 and 4% in SC, p<0.001). The principal reasons for 537 cardiologic HA in 432 pts, were: interventional procedures (n=209, 39%), diagnostic catheterisation (n=155, 29%), non invasive evaluation (n=95, 18%), arrhythmias (n=46, 10%). Closure of atrial septal discontinuity (n=167, 80%), patent ductus arteriosus (n=21, 10%), pulmonary valvuloplasty (n=8, 4%) and angioplasty of Coarctation (n=6, 3%), were more prevalent interventional procedures. In pts with GCC, heart failure and cyanosis were more commonly cause of HA than in MGC and SC (p<0.001). Despite the progress of pediatric cardiology and cardiac surgery, medical demands of GUCH remain numerous and complex throughout their lives. Therefore, it is extremely important to provide specialized surgical and cardiologic care for this complicated population

4-Dimensional Velocity Mapping Cardiac Magnetic Resonance of Extracardiac Bypass for Aortic Coarctation Repair

Abstract This report describes a case of a young lady who, following extracardiac bypass between ascending and descending aorta for severe aortic coarctation, underwent 4-dimensional flow cardiac magnetic resonance, a technique that, by 3-dimensional flow assessment over time (4-dimensional), allows not only quantification of flows but also wall shear stress. In this case, increased wall shear stress was observed in the conduit's acute angle (kinking) as well as at the distal anastomosis level. The authors postulate that increased wall shear stress could help identify and risk stratify adult congenital heart disease who could develop vascular complications in the future. ( Level of Difficulty: Intermediate.

Cumulative patient effective dose and acute radiation-induced chromosomal DNA damage in children with congenital heart disease

Background The seventh Committee on "Biological Effects of Ionizing Radiation" (BEIR VII, 2006) underlines "the need of studies of infants who are exposed to diagnostic radiation because catheters have been placed in their hearts". Objective To determine the lifetime attributable risk (LAR) of cancer associated with the estimated cumulative radiological dose in 59 children (42 male, age 2.863.2 years) with complex congenital heart disease, and to assess chromosomal DNA damage after cardiac catheterisation procedures. Methods In all patients, the cumulative exposure was estimated as effective dose in milliSievert (mSv), and LAR cancer was determined from the BEIR VII report. In a subset of 18 patients (13 male, age 5.265.7 years) micronucleus as a biomarker of DNA damage and longterm risk predictor of cancer was assayed before and 2 h after catheterisation procedures. Doseearea product (Gy cm2) was assessed as a measure of patient dose. Results The median life time cumulative effective dose was 7.7 mSv per patient (range 4.6e41.2). Cardiac catheterisation procedures and CT were responsible for 95% of the total effective dose. For a 1-year-old child, the LAR cancer was 1 in 382 (25th to 75th centiles: 1 in 531 to 1 in 187) and 1 in 156 (25th to 75th centiles: 1 in 239 to 1 in 83) for male and female patients, respectively. Median micronucleus values increased significantly after the procedure in comparison with baseline (before 6&vs after 9&, p?0.02). The median doseearea product value was 20 Gy cm2 (range 1e277). Conclusion Children with congenital heart disease are exposed to a significant cumulative dose. Indirect cancer risk estimations and direct DNA data both emphasise the need for strict radiation dose optimisation in children

The role of cardiovascular magnetic resonance in candidates for Fontan operation: Proposal of a new Algorithm

<p>Abstract</p> <p>Background</p> <p>To propose a new diagnostic algorithm for candidates for Fontan and identify those who can skip cardiac catheterization (CC).</p> <p>Methods</p> <p>Forty-four candidates for Fontan (median age 4.8 years, range: 2-29 years) were prospectively evaluated by trans-thoracic echocardiography (TTE), Cardiovascular magnetic resonance (CMR) and CC. Before CC, according to clinical, echo and CMR findings, patients were divided in two groups: Group I comprised 18 patients deemed suitable for Fontan without requiring CC; group II comprised 26 patients indicated for CC either in order to detect more details, or for interventional procedures.</p> <p>Results</p> <p>In Group I ("CC not required") no unexpected new information affecting surgical planning was provided by CC. Conversely, in Group II new information was provided by CC in three patients (0 vs 11.5%, p = 0.35) and in six an interventional procedure was performed. During CC, minor complications occurred in one patient from Group I and in three from Group II (6 vs 14%, p = 0.7). Radiation Dose-Area product was similar in the two groups (Median 20 Gycm², range: 5-40 vs 26.5 Gycm², range: 9-270 p = 0.37). All 18 Group I patients and 19 Group II patients underwent a total cavo-pulmonary anastomosis; in the remaining seven group II patients, four were excluded from Fontan; two are awaiting Fontan; one refused the intervention.</p> <p>Conclusion</p> <p>In this paper we propose a new diagnostic algorithm in a pre-Fontan setting. An accurate non-invasive evaluation comprising TTE and CMR could select patients who can skip CC.</p

4D Flow cardiovascular magnetic resonance consensus statement: 2023 update

4D Flow MRI; Hemodynamics; RecommendationsRessonància magnètica de flux 4D; Hemodinàmica; RecomanacionsResonancia magnética de flujo 4D; Hemodinámica; RecomendacionesHemodynamic assessment is an integral part of the diagnosis and management of cardiovascular disease. Four-dimensional cardiovascular magnetic resonance flow imaging (4D Flow CMR) allows comprehensive and accurate assessment of flow in a single acquisition. This consensus paper is an update from the 2015 ‘4D Flow CMR Consensus Statement’. We elaborate on 4D Flow CMR sequence options and imaging considerations. The document aims to assist centers starting out with 4D Flow CMR of the heart and great vessels with advice on acquisition parameters, post-processing workflows and integration into clinical practice. Furthermore, we define minimum quality assurance and validation standards for clinical centers. We also address the challenges faced in quality assurance and validation in the research setting. We also include a checklist for recommended publication standards, specifically for 4D Flow CMR. Finally, we discuss the current limitations and the future of 4D Flow CMR. This updated consensus paper will further facilitate widespread adoption of 4D Flow CMR in the clinical workflow across the globe and aid consistently high-quality publication standards.1R01HL149787-01A1 (S. Schnell, M. Markl), 1R21NS122511-01 (S. Schnell), 1R01CA233878-01 (J.Collins) J.Sotelo thanks to ANID–Millennium Science Initiative Program–ICN2021_004 and FONDECYT de iniciación en investigación #11200481. Dr. Oechtering receives funding from the German Research Foundation (OE 746/1-1)

Role of semisupine exercise stress echocardiography in operated Fallot

Background: Right Ventricle (RV) outflow tract anomalies in operated Tetralogy of Fallot (TOF) leads to RV dysfunction. Due to the difficulties to its assessment, timing of RV outflow reconstruction is still a matter of debate. Semi-supine exercise echo (SEE) has potential for simultaneous evaluation of RV function, pressure, and area changes during stress. Aim to assess the feasibility and value of SEE in operated TOF. Methods: we evaluated 62 consecutive operated Fallot by SEE (mean age 24 ? 11 years, 16 pts were less than 18 y. o.). The following parameters were measured at rest and peak exercise: RV area (from apical 4 chamber view), Tricuspid annular plane systolic excursion (TAPSE), Right ventricular pressure (RVP, from tricuspid regurgitant jet velocity); RV fractional area change (RV-FAC). Within 3 days, all patients also underwent cardiac Magnetic Resonance, Cardio-pulmonary Exercise Test and Amino-Terminal pro-Brain Natriuretic Peptide (NT-proBNP) essay. Results: Exercise was stopped at 105 ? 35 Watt; heart rate increased from 83 ?16 bpm to 149 ?17 bpm. During exercise, interpretable images for RV FAC analysis were obtained in 56/62 patients (RV FAC feasibility = 90 %). Due to continent tricuspid valve in 7 patients RVP we couldn\u27t been measured (RVP feasibility = 89%). There was on average an increase in RVP (rest = 45 ? 18 vs. stress= 78 ? 37 mmHg, p<0.01 vs. rest), TAPSE (rest=15?4 vs. stress= 18? 3 mm , p<0.01 vs. rest), and RV-FAC (rest= 48? 9 vs. stress= 52 ? 8 % , p<0.05 vs. rest), with substantial individual variability. In particular, RV FAC increased in 40 ("responders") and remained stable or decreased in the remaining 9 pts, all of them > 18 y.o. ("non responders") (see figure). Compared to responders, non-responders had higher NT-proBNP (366 ? 264 vs 127 ? 92 ng/l,p<0.001), lower peak VO2/Kg (15 ? 4.4 vs 22 ? 5.4 ml/Kg/min, p<0.001), larger MRI-assessed RV End diastolic volume (166 ?61 vs 130 ? 42 ml/m2, p<0.05) and end systolic volume (86 ml/m2 ? 29 vs 58 ? 25, p<0.001) and lower RV EF (47 ? 8 vs 56 ? 8.5 %, p<0.01). Conclusion: SEE is feasible in patient with repaired TOF and allows the integrated assessment of variation of RV pressures, area, and function during exercise, which usefully complement more conventional indices of hemodynamic burden in these patients. Longitudinal follow-up is needed to better delineate the prognostic value of such SEE results

Ascending aortic aneurysm in a patient with bicuspid aortic valve, positive history of systemic autoimmune diseases and common genetic factors: a case report

The bicuspid aortic valve (BAV) and specific systemic autoimmune diseases are associated with cardiovascular manifestation, including aortic aneurysm. We reported a case of 64 year-old patient with BAV and a history of ankylosing spondylitis (AS) and systemic lupus erythematosus (SLE), and who developed ascending thoracic aortic aneurysm. The patient presented also the homozygosity for genetic variants of MMP9, ACE, MTHFR and PAI-1 genes. Gene-environmental interactions may represent an additional pathogenetic dimension in the still challenging management of the abnormalities of the aortic wall, including dilatation, aneurysm and dissection