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Duchenne muscular dystrophy (DMD) cardiomyopathy (DCM) is characterized by a hypokinetic, dilated phenotype progressively increasing with age. Regular cardiac care is crucial in DMD care. Early recognition and prophylactic use of angiotensin converting enzyme inhibitors (ACEi) are the main stay therapeutic strategy to delay incidence of DMD-DCM. Pharmacological treatment to improve symptoms and left ventricle (LV) systolic function, have been widely implemented in the past years. Because of lack of DMD specific drugs, actual indications for established DCM include current treatment for heart failure (HF). This review focuses on current HF strategies to identify, characterize, and treat DMD-DCM.

Aim To summarize our experience on the implementation of a telemedicine service dedicated to adult congenital heart disease (ACHD) patients during the lockdown for the first wave of Coronavirus disease 2019 (COVID-19). Methods This is a prospective study enrolling all ACHD patients who answered a questionnaire dedicated telematic cardiovascular examination. Results A total of 289 patients were enrolled, 133 (47%) were male, 25 (9%) were affected by a genetic syndrome. The median age was 38 (29–51) years, whereas the median time interval between the last visit and the telematic follow-up was 9.5 (7.5–11.5) months. Overall, 35 patients (12%) reported a worsening of fatigue in daily life activity, 17 (6%) experienced chest pain, 42 (15%) had presyncope and 2 (1%) syncope; in addition, 28 patients (10%) presented peripheral edema and 14 (5%) were orthopneic. A total of 116 (40%) patients reported palpitations and 12 had at least one episode of atrial fibrillation and underwent successful electrical (8) or pharmacological (4) cardioversion. One patient was admitted to the emergency department for uncontrolled arterial hypertension, five for chest pain, and one for heart failure. Two patients presented fever but both had negative COVID-19 nasal swab. Conclusion During the COVID-19 pandemic, the use of telemedicine dramatically increased and here we report a positive experience in ACHD patients. The postpandemic role of telemedicine will depend on permanent regulatory solutions and this early study might encourage a more systematic telematic approach for ACHD patients.

OBJECTIVES: Tricuspid valve (TV) surgery in the adult with congenital heart disease (ACHD) is a frequently performed procedure. The aim of this study was to analyse postoperative and medium-term outcomes. METHODS: We conducted a single-centre retrospective study of patients with ACHD who underwent TV surgery (January 2000–December 2016); patients with Ebstein’s anomalies were excluded. Operative and clinical records were reviewed. Outcomes considered were survival, grade of insufficiency/stenosis and TV reoperation at follow-up. RESULTS: A total of 128 patients with ACHD had TV surgery for functional regurgitation (n = 95), dysplasia (n = 23) and systemic TV (n = 10). Median age was 40.8 years [interquartile range (IQR) 25.3]; 55.5% were men. Preoperative regurgitation was classified as mild (n = 8), moderate (n = 47) and severe (n = 70). The TV was repaired in 109 as follows: ring annuloplasty (n = 43), de Vega annuloplasty (n = 29), Wooler annuloplasty (n = 13), commissural plasty (n = 9), Kay annuloplasty (n = 7) and others (n = 8). The TV was replaced in 19 patients with biological (n = 10) and mechanical (n = 9) prostheses. The median hospital stay was 12 days (IQR 10). The overall mortality rate was 8.6% (n = 11): 2 hospital deaths (1.6%) and 9 late deaths. Survival was 93% [95% confidence interval (CI) 85–97%] at 5 years and 83% (95% CI 70–91%) at 10 years. The median follow-up period was 4.95 years (IQR 7.7) with 1 TV reoperation. Echocardiographic assessment showed ≥moderate regurgitation in 34 (34.3%) patients. Suture plasty had a significantly higher incidence of TV regurgitation ≥moderate compared to ring annuloplasty (48.9% vs 26.3%; P = 0.033). CONCLUSIONS: TV surgery in the ACHD is frequently associated with other main procedures. Stabilizing the TV annulus with a prosthetic ring guarantees lower recurrence of moderate to severe regurgitation compared to suture plasty repair.

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Twenty-one patients with clinically suspected recurrence of ovarian (n = 3) or uterine (n = 18) carcinoma were examined with suprapubic ultrasound (US) and transrectal US with high-frequency linear probes. The examinations were performed 3, 6, 9, and 15 months after surgery and radiation therapy. Eight patients underwent radiation therapy before surgery and ten after surgery; three underwent only surgery. Criteria for recurrence included increased anteroposterior diameter of the vaginal cuff (greater than 2.2 cm); structural alterations or presence of a mass in the vaginal cuff; and infiltration of the rectovaginal septum, bladder, and parametria. Transrectal US findings were true positive for recurrence in nine cases, true negative in ten, and false positive in two. US findings were true positive in three cases, true negative in seven, false positive in two, and false negative in three. In six cases results from US were technically poor, and no diagnosis could be made. Transrectal US was highly sensitive in detection of pelvic recurrent carcinomas, while US had little diagnostic value. The authors believe transrectal US can replace US in the evaluation of patients at risk for recurrent pelvic neoplasm.

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Dvacetiletý muž byl hospitalizován v naší nemocnici pro recidivu pleurálního výpotku, dušnost při námaze a počáteční známky selhání pravého srdce. V dětství mu byla stanovena primární diagnóza defektu síňového septa typu secundum a ve věku 16 let absolvoval korekci defektu síňového septa záplatou. Vyšetření magnetickou rezonancí prokázalo anomální návrat dvou plicních žil vysoko do horní duté žíly. Provedli jsme modifi kovanou Wardenovu operaci s konduitem z umělého materiálu. Popisujeme výhody této techniky při opakovaném výkonu u dospělého pacienta s vrozenou vadou.