The patient is a 5-year-old right-handed boy who presented to the dermatology department with a 2-year history of recurrent fingertip dermatitis, which was more pronounced on the first three digits of his right hand. He had a past history of asthma, but not of eczema or hay fever. The symptoms improved when he was away from home and school. Although the fingertip dermatitis responded to clobetasol propionate 0.05% ointment or tacrolimus 0.03% ointment and a bland emollient, it continued to reoccur intermittently. This led us to believe that the cause was exposure at his home or at school.A detailed history showed that the patient enjoyed playing with marbles, a tablet computer, 1p and 2p coins, Lego ® , and, in particular, a collection of > 150 die-cast model cars that he liked to crash together, many of which had lost their paint. He was patch tested with the British Contact Dermatitis Society baseline series and metals series, with allergens and IQ Ultra TM chambers (Chemotechnique, Vellinge, Sweden). On day (D) 2 and D4, he had a strong positive (++) reaction to copper(II) sulfate pentahydrate 2% pet., and a negative reaction to nickel. A more haemorrhagic but less infiltrated response

There is a paucity of high-quality evidence and guidelines on the prediction of skin defect reconstruction, and the type of repair rests on the operating surgeon's experience and skill. Mismatches between planning and execution can have negative consequences on resources, staff, and patient counseling. To investigate the factors that predict complex reconstruction in facial skin cancer surgery, we performed a retrospective study collecting information on the several parameters that may affect the method of reconstruction. A total of 325 cases were included. Only the factors tumor size (Z = 2.54; P < .05) and predicted repair plan (Z = 2.73; P < .01) were found to be significant predictors of complex repairs. When broken down by site and size, only the nose, scalp, and temple demonstrated a significant correlation between tumor size and the need for complex repairs. Clinical judgment at the time of initial examination is by far the greatest predictor of complex repairs. This occurs even when clinicians have heterogeneous surgical skills, and most are not technically versed in complex repairs. Increasing tumor size was also found to be an independent risk factor albeit only at certain body sites such as the temple, scalp, and nose.

It is unclear whether residual anterograde pulmonary blood flow (APBF) at the time of Fontan is beneficial. Pulsatile pulmonary flow may be important in maintaining a compliant and healthy vascular circuit. We, therefore, wished to ascertain whether there was hemodynamic evidence that residual pulsatile flow at time of Fontan promotes clinical benefit. 106 consecutive children with Fontan completion (1999–2018) were included. Pulmonary artery pulsatility index (PI, (systolic pressure–diastolic pressure)/mean pressure)) was calculated from preoperative cardiac catheterization. Spectral analysis charted PI as a continuum against clinical outcome. The population was subsequently divided into three pulsatility subgroups to facilitate further comparison. Median PI prior to Fontan was 0.236 (range 0–1). 39 had APBF, in whom PI was significantly greater (median: 0.364 vs. 0.177, Mann–Whitney p < 0.0001). There were four early hospital deaths (3.77%), and PI in these patients ranged from 0.214 to 0.423. There was no correlation between PI and standard cardiac surgical outcomes or systemic oxygen saturation at discharge. Median follow-up time was 4.33 years (range 0.0273–19.6), with no late deaths. Increased pulsatility was associated with higher oxygen saturations in the long term, but there was no difference in reported exercise tolerance (Ross), ventricular function, or atrioventricular valve regurgitation at follow-up. PI in those with Fontan-associated complications or the requiring pulmonary vasodilators aligned with the overall population median. Maintenance of pulmonary flow pulsatility did not alter short-term outcomes or long-term prognosis following Fontan although it tended to increase postoperative oxygen saturations, which may be beneficial in later life.

No abstract

950 Cardiomyopathy / Univentricular heart, heart failure and pulmonary hypertension in congenital heart diseases Methods: Family screening was offered to 2,372 consecutive unrelated patients; 10,000 subjects (43±7 years old, 52% men) were evaluated (mean 4.2 relatives/family), of which 32% were affected and 6% were possibly affected. Familial study was recommended because of an index case with hypertrophic cardiomyopathy (HCM) in 42%, idiopathic dilated cardiomyopathy (DCM) in 17%, arrhythmogenic right cardiomyopathy (ARCM) in 4%, arrhythmogenic left cardiomyopathy (ALCM) in 1%, left ventricular non-compaction (LVNC) in 4%, Brugada syndrome (BS) in 9%, long QT syndrome (LQTS) in 4% and other conditions in 18%. Index case and non-index cases were defined. Results: Familial disease was confirmed in 47%; 51% with HCM, 53% with DCM, 39% with ARCM, 92% with ALCM, 37% with LVNC, 28% with BS, and 35% with LQTS. 606 affected non-index patients were identified (46% meeting HCM, 19% DCM, 6% ARCM, 3% ALVC, 5% LVNC, 9% BS, and 6% LQTS criteria), in whom appropriate risk stratification and medication, if needed, were initiated. 23% had a family history of sudden death. 20% of all affected were athletes (29% of whom were non-index patients). Cardioverter defibrillator (ICD) implantation was indicated in 359 patients (64% for primary and 36% for secondary prevention). 20% of ICD implanted were in non-index cases. Conclusion:The prevalence of familial disease in inherited cardiac conditions is high. Systematic familial study identified a significant number of asymptomatic affected patients who could benefit from early treatment to prevent complications. Dedicated clinics and multidisciplinary teams are needed for proper screening programs.UNIVENTRICULAR HEART, HEART FAILURE AND PULMONARY HYPERTENSION IN CONGENITAL HEART DISEASES P4522 | BEDSIDE Should we leave pulsatile forward flow after the bidirectional Glenn operation?P. Walker-Smith, Q. Chen, H. Weaver, M. Caputo, S. Stoica, A. Parry, R. Tulloh. University Hospitals Bristol NHS Foundation Trust, Bristol Royal Hospital for Children, Bristol, United KingdomBackground: Children with functionally univentricular hearts undergo a treatment pathway to the Fontan operation. A lack of significant pulmonary artery pulse pressure could be deleterious for pulmonary compliance and long term health of the circuit. We wished to try to determine whether the presence of pulsatile flow at the time of the Fontan improved outcome after this operation. Methods: 148 children who underwent Fontan at our institution from 1999 to 2016 underwent cardiac catheterisation to determine pulse pressure, transpulmonary gradient and pulmonary vascular resistance, along with verification of anatomy and function pre-operatively. At Fontan 48 had undergone pulmonary artery band, 56 had previous systemic to pulmonary shunt and 44 had native pulmonary stenosis to protect their pulmonary vasculature. Comparison was made between those with forward flow (group1) at time of Fontan operation and those without (group...