[An up-to-date telemedicine integrated clinical pathway for adult patients with congenital heart disease]. / Un nuovo percorso di cura integrato con la telemedicina per la gestione del paziente adulto con cardiopatia congenita.

Clinical management of adult patients with congenital heart disease (GUCH) is a difficult task for multiple reasons, which include their own pathology and clinical history complexity, diagnostic complexity and organization of care. GUCH specialists are present in very small numbers and are concentrated in few centers, thus generating considerable transfer problems for patients. During the COVID-19 pandemic, telemedicine has become the standard of care, ensuring health assistance continuity, and implementing communication channels between patients and health professionals. We suggest to stratify GUCH patients into three groups, which correspond to different levels of risk (low, moderate and high, respectively) to develop complications over time, using a GUCH-specific multiparametric complexity score; so, each patient pathway will be defined according to the specific group, with indication of site, timing and type of clinical and instrumental evaluations, including virtual visits and consults. In conclusion, practical tools are provided for the implementation of updated care pathways for GUCH patients, who finally are inserted in a new model of care in which even if in-person visit still represents the crucial moment of each patient care pathway, on the other hand, telemedicine incorporation could contribute to improving and making even more complete and effective GUCH patient care.

Tricuspid annular plane systolic excursion for the assessment of ventricular function in adults operated on with mustard procedure for complete transposition of the great arteries.

BACKGROUND: In adult patients with d-transposition of the great arteries after atrial switch operation, dysfunction of the systemic right ventricle (RV) is a well-known complication. Echocardiographic variables may provide adequate estimation of subpulmonary RV function, but their applicability to the subaortic RV is not straightforward. We evaluate the concordance between tricuspid annular plane systolic excursion (TAPSE) and magnetic resonance imaging-derived ejection fraction of the RV (MRI-RVEF) in these patients. METHODS: Patients were recruited from those evaluated at the adult congenital clinic of our department between 2010 and 2012. All patients who had an echocardiographic assessment within 6 months of their MRI examination were selected. Patients clinically unstable, not in sinus rhythm, with a prosthetic systemic atrioventricular valve, permanent pacemaker, or more than moderate systemic atrioventricular valve regurgitation were excluded. RESULTS: Eighteen Mustard-operated patients aged 22 ± 3.7 years were studied. The mean values of TAPSE and RVEF were 13.22 ± 1.7 mm and 49.7 ± 6%, respectively. TAPSE and RVEF were normal in 1 (5.5%) and 10 (55.5%) patients, respectively. Seventeen (94.4%) patients showed reduced TAPSE (12.9 ± 1.3 mm): RVEF was reduced in eight (47%) of these subjects, and normal in nine (53%). In patients with normal RVEF, both the MRI-RV end-diastolic and the MRI-RV end-systolic volumes were significantly lower than in patients with reduced RVEF. There were no other statistically significant differences between these patients. No correlation was found between TAPSE and both the MRI-RV end-diastolic and the end-systolic volumes. Globally, agreement between TAPSE and RVEF was slight (K = 0.09 ± 0.089). CONCLUSIONS: Our results indicate that in these patients TAPSE is not a useful measure of RV function.

Subclinical cardiac dysfunction and exercise performance in childhood cancer survivors.

BACKGROUND: Although anthracycline cardiotoxicity is clearly related to the cumulative dose administered, subclinical cardiac dysfunction has been reported across a wide range of treatment regimens, and its clinical significance is still unclear. Purpose of this study is to investigate by exercise echocardiography for subclinical cardiac dysfunction in survivors of pediatric cancer treated with low-moderate anthracycline doses, and to evaluate whether it may alter the response of the cardiovascular system to dynamic exercise. PROCEDURE: Post-exercise left ventricular end-systolic wall stress (ESS), left ventricular posterior wall dimension and percent thickening at end systole, and cardiopulmonary exercise test-derived indexes of cardiac function were examined in 55 apparently healthy patients (mean age 13.5 ± 2.9 years, median anthracycline cumulative dose 240 mg/m(2)) and in 63 controls. RESULTS: Subclinical cardiac dysfunction was identified in 17 patients (30%) presenting reduced left ventricular posterior wall dimension or percent thickening, or increased values of left ventricular ESS as compared to controls (group A), while the remaining patients formed group B. Reduced oxygen consumption at peak exercise in both groups of patients was the only cardiopulmonary exercise test variable resulting significantly different between patients and controls: no differences were found among the groups of patients. CONCLUSIONS: Our results confirm that even patients treated with a median anthracycline dose of 240 mg/m(2) (range 100-490) are at considerable risk of exhibiting subclinical cardiac dysfunction that, however, does not seem to alter the physiologic response of the cardiovascular system to dynamic exercise.

Stenting of aortic coarctation and exercise-induced hypertension in the young.

OBJECTIVES: To evaluate whether the stenting of aortic coarctation enhance the risk of exercise-induced hypertension (EIH). BACKGROUND: There is the theoretical concern that aortic stents may cause increased aortic wall impedance and therefore systolic hypertension during exercise. METHODS: Blood pressure and the Doppler derived peak and mean systolic pressure gradient (PSG and MSG) across the distal aorta at the peak of exercise were evaluated in young patients (mean age 14 +/- 3 years) with aortic coarctation successfully treated with surgery or with stent implantation at least 1 year before the test. Only patients who reached the 85% maximal predicted heart rate or whose exercise test was interrupted because of severe hypertension, and in whom significant aortic narrowings were excluded by a MRI or a CT scan performed in the six months preceding the exercise test were included in the study. RESULTS: Seventeen patients formed the surgery-group, while 15 patients the stent-group. Patients in surgery-group were younger at coarctation repair and with a longer follow-up than those in stent-group. No difference was present regarding age, body surface area, gender, and presence, and degree of mildly hypoplastic aortic segments between the two groups as well as between patients with or without EIH. EIH was found in 35% of surgery-group patients and in 33% of stent-group patients. PSG and MSG were similar in the patients with or without EIH. CONCLUSIONS: EIH can be found in a high number of young patients successfully treated for aortic coarctation but at intermediate follow-up stent implantation does not seem to enhance the risk of EIH.

Aortic arch geometry and exercise-induced hypertension in aortic coarctation.

Hypertension at rest or during effort is not uncommon in patients with aortic coarctation (CoA), even those with a successful repair or mild degree of obstruction. Anatomic factors and functional abnormalities have been proposed as causes of this finding. Recently, aortic arch geometry was reported in association with hypertension at rest in patients with successful CoA repair. Forty-one patients (age 15.7 +/- 4.6 years) without significant obstruction at rest (mean systolic Doppler gradient at rest < or =25 mm Hg) were selected for the study. All patients underwent a maximal cardiopulmonary exercise test and magnetic resonance imaging of the aorta. Aortic arch shape was defined on global geometry as normal, gothic, and crenel. Percentage of anatomic narrowing (AN) was also calculated. Twenty-four patients (58%) showed exercise-induced hypertension (EIH). Regarding the shape of the aortic arch, normal geometry was present in 17 patients (41%), 9 (21%) had gothic geometry, and 15 (36%) had crenel geometry. There were no differences among the 3 geometries in regard to the incidence of EIH (70.6% in normal, 55.6% in gothic, and 46.7% in crenel) or AN (36.9% in normal, 33.5% in gothic, and 36.6% in crenel). In conclusion, our results fail to show a correlation between a specific aortic arch shape and the incidence of EIH and significant AN in patients with native or residual CoA or repeat CoA. Therefore, at present, the role of aortic arch geometry in identifying patients at risk of EIH is still uncertain.