Patients: 32 patients, mean age 32.5 years, 21 female, and 11 male. Intervention: Transthoracic (TTE) and transoesophageal (TOE) echocardiographic examination in patients with suspected DCRV. Main outcome measures: Direct inspection during surgical treatment of 28 patients diagnosed as having DCRV as an isolated lesion or associated with other pathologies. Results: Echocardiography allowed the final diagnosis of DCRV in 26 patients (81%) out of 32 studied. TTE was diagnostic in 5 (15.6%) whereas TOE was diagnostic in 21 of 21 studied by this technique. Of 6 patients with negative TTE, DCRV was identified by cardiac catheterisation in 3 and directly during surgery in the remaining 3. Of 26 patients diagnosed by echocardiography, the anomalous muscle bundle was discrete in 20 (77%) and diffuse in 6 (23%). In 23 patients (88%) right ventricular outflow obstruction was localised low in the right ventricle, and in the remaining 3 (11.5%), the obstruction was localised high in the right ventricle. Abnormal bundles localised high were discrete, bundles localised in the lower part of right ventricle were discrete in 17 (74%) and diffuse in the remaining 6 (26%). DCRV was an isolated lesion in only 2 patients (6.2%). In all subjects surgical inspection confirmed echocardiographic data. Conclusions: Echocardiography was very useful to diagnose DCRV in adults as well as to identify its anatomic type. Comparing two different approaches, TOE better defines the entire scope of pathology, including estimation of the resultant systolic pressure gradient within the right ventricular cavity.

PPVI with routine prestenting with BMS is a safe and effective method of treatment in patients with repaired CHD.

There is little experience with transcatheter closure of very large, hypertensive patent ductus arteriosus. We present a case of successful closure of a 16 mm ductus with the Amplatzer VSD occluder, a device originally designed for transcatheter closure of congenital muscular ventricular septal defects. To our best knowledge this is the largest ductus ever closed by an interventional technique.

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A b s t r a c tBackground: Transcatheter pulmonary artery valve implantation (TPVI) is a relatively new method of treatment in patients (pts) after repair of congenital heart disease (CHD).Aim: To assess the early and one year results of TPVI in patients with right ventricular outflow tract conduit dysfunction. Material and methods: TPVI with routine pre-stenting with BMS was performed in 17 pts (9 men, mean age 24.1 ±5.6 years) for pulmonary conduit dysfunction 11.4 ±6.4 years after total repair of tetralogy of Fallot or pulmonary atresia (11 pts: 8 pts with pulmonary homograft, 3 pts with Contegra xenograft or aortic monocusp homograft), pulmonary stenosis (1 pt), Ross procedure (3 pts) and Rastelli operation (TGA, pulmonary atresia -2 pts). The schedule of follow-up assessment comprised clinical evaluation, cardiovascular magnetic resonance, transthoracic echocardiography and chest X-ray to screen for device integrity. Seventeen pts completed 1-month, 11 pts 6-month and 10 pts 12-month follow-up.Results: TPVI was performed with no serious complications in all patients. In 15 patients with significant pulmonary stenosis peak right ventricular outflow tract (RVOT) gradient was reduced from a mean of 73.4 ±30.0 mm Hg to 35,3 ±14,7 mm Hg on the next day after implantation (p < 0.001). At 1-month, 6-month and 12-month follow-up mean RVOT gradient was 30.4 ±11.2 mm Hg, 31.1 ±11.9 and 32.7 ±11.7 mm Hg, respectively (NS). In all patients pulmonary valve competence was restored. Mean pulmonary regurgitation fraction decreased from 20.9 ±7.8% to 2.4 ±2.1% (p = 0.0001) one month after procedure and the effect was stable after one year observation. Significant decrease in right ventricular end-diastolic and end-systolic volumes (131.8 ±47.8 ml/m 2 to 115.3 ±40.3 ml/m 2 ; p = 0.002 and 73.1 ±41.1 ml/m 2 vs. 57,6 ±39,0 ml/m 2 ; p = 0.004, respectively) as well as a slight improvement in RV ejection fraction (47.9 ±13.0% to 53.2 ±15.2%; p = 0.07) were observed one month after procedure. No stent fractures were seen.Conclusions: 1. TPVI is an effective and safe method of non-surgical treatment for patients with RVOT conduit dysfunction. 2. TPVI wit the use of pre-stenting technique may be performed in selected patients with RVOT patch. 3. Routine pre-stenting with BMS may protect against stent fractures after TPVI. 4. The study showed excellent results in 10 patients after one-year follow-up.

Appreciable arterial desaturation and cyanosis in patients with ASD, regarded as uncomplicated, should be followed by careful investigation for the direction of tricuspid regurgitant flow.

Background Primary cardiac tumors are rare entities, only 25% of them are malignant. Most common malignant neoplasms of the heart are sarcomas - aggressive tumors with a high rate of local recurrence and systemic metastases. They may lead to a rapid clinical deterioration and sudden circulatory collapse due to obstruction of the intracardiac outflow tract. Most patients affected are younger than 65 years of age. Early diagnosis of cardiac tumors is challenging, echocardiography remains a mainstay of their evaluation. The prognosis is very poor, most patients succumb to disease within months of diagnosis. Complete surgical resection and adjuvant chemotherapy offers palliation and improves survival. Total artificial heart implantation (TAH) allows through, radical resection of large tumors yielding the best possible outcome. Case presentation A 58-year-old male patient with a history of hypertension was admitted to our hospital with a chief complaint of worsening dyspnea and fatigue for last several weeks. Echocardiogram demonstrated a large cardiac mass with abundant vascular supply that incorporated the whole right ventricle and intraventricular septum, extended to the tricuspid valve, infiltrated the left ventricle apex and the base of posterior tricuspid leaflet. The right ventricle cavity was slit-like with small gradient in the outflow track. Cardiac magnetic resonance suggested angiosarcoma or lymphoma. PET-CT did not reveal any extracardiac metastases at that time. The case was discussed at our multidisciplinary cardio-oncology conference. The radical resection of the mass with subsequent TAH implantation was decided upon in high-risk setting, with feasible long-term survival, including eventual consideration for cardiac transplantation (HTX). Native ventricles were excised at the atrioventricular level down to the annulus of the mitral and tricuspid valves. The artificial ventricles were connected to the atrial remnants. The ascending aorta and the main pulmonary artery were dissected close to the valves and connected to outflow grafts. Histopathologic examination of the mass demonstrated angiosarcoma. The patient was started on paklitaksel. After completion of his chemotherapy regimen his eligibility for HTX will be reevaluated. Conclusion Diagnosis and therapy of sarcomas are challenging. Their rarity makes it difficult to standardize therapy. There is few evidence available in literature to guide the treatment of such patients. Case-specific, heart-team-based strategy must be adopted to aid decision making in this rare cases. Sarcomas are lethal, develop rapidly and affect fairly young patients. Prompt surgical resection with subsequent TAH implantation and adjuvant chemotherapy seems ideal in this situation as it offers a possible cure. It gives a slim chance of long-term survival after consecutive HTX for chemotherapy responders. Abstract P239 Figure. Angiosarcoma in TTE, MRI and TEE.