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BackgroundSigmoid Ventricular Septum (SVS) is a type of hypertrophic cardiomyopathy characterized by a reduced angle between the basal interventricular septum and the ascending aorta, and SVS can lead to dynamic Left Ventricular Outflow Tract obstruction (LVOTO) during hypercontractile states. Patients experiencing LVOTO may manifest symptoms such as angina, syncope, etc. Radiofrequency ablation (RFA) has been utilized to treat patients with hypertrophic obstructive cardiomyopathy, but there is no reports on its use in treating LVOTO resulting from SVS. Our report describes two cases of SVS treated with endocardial ablation to improve LVOTO.Case reportCase 1: A 74-year-old female patient with angina and syncope was admitted to the hospital and diagnosed with SVS by transthoracic echocardiogram. The patient exhibited LVOTO and Systolic Anterior Motion (SAM) phenomena during the administration of the dobutamine stress test. After RFA was performed, the patient's symptoms significantly improved. Additionally, septum decreased from 16 to 13 mm after ten months, and the morphological changes associated with SVS also disappeared. Case 2: A 57-year-old female was admitted to the hospital due to recurrent chest pain after physical activity for more than four years. The transthoracic echocardiogram indicated that the patient met the diagnostic criteria for SVS, and LVOTO and SAM phenomenaoccurred following dobutamine stress test. The patient had significant improvement in symptoms after undergoing RFA treatment.ConclusionsThese two cases represent the first documented instances where dynamic LVOTO caused by SVS could be effectively managed through endocardial RFA.

Objective: Our aim was to report on the long-term outcomes of patients who underwent RV-PA conduit placement at our institute. Materials and Methods: We retrospectively reviewed 407 RV-PA conduit placements from January 1997 to December 2018. The primary outcomes were freedom from and risk factor(s) for conduit re-operation. The secondary outcomes were survival, freedom from conduit dysfunction and conduit-related catheter intervention. Results: Of all the included patients, 209 were male (51.4%) and the median age at the operation was nine years (IQR 6, 18 years). The most commonly used conduit types were bovine jugular vein conduit (125, 30.7%), pulmonary homograft (122, 30.0%), and aortic homograft (76, 18.7%). The median follow-up time was 5.1 years (IQR 0.9, 9.2 years). The overall survival was 92.2% at 5 years. Freedom from re-operation was 95.4% and 84.2%, at 5 and 10 years. Factors related to conduit reoperation were age at operation less than 1 year, diagnosis rather than pulmonary atresia or stenosis, conduit size less than 18 mm, and conduit z-score greater than 3 (all p

Abstract A 71 year-old male with a history of multiple excisions of an initial Clark's level V melanoma of the breast followed by combined radiation and interferon treatment, as well as a recurrence, 3 years later, of a BRAF-positive tumor of the shoulder, with subsequent therapy with dabrafenib and trametinib, presented again with progressive intracardiac masses causing significant right ventricular outflow obstruction. Additionally, the patient complained of dyspnea and fatigue on exertion, thus he was scheduled for surgical resection.

A 68-year-old man presented with chest distress recurring for the past 10 years. An echocardiogram demonstrated bicuspid aortic valve malformation with severe aortic stenosis and ventricular septal thickness of 22 mm. The patient underwent successful transcatheter aortic valve replacement (TAVR). Six months later, he complained of worsening dyspnea and chest distress (New York Heart Association Class III) on exertion. Besides a functional normal AV prosthesis, the echocardiography indicated the left ventricular outflow tract obstruction peak gradient of 122 mmHg at rest. Alcohol septal ablation was performed as the patient was unable to tolerate morrow procedure. His symptoms were relieved immediately after ablation, and no major cardiovascular events were observed during the 20-month follow-up. In conclusion, among patients with concomitant hypertrophic obstructive cardiomyopathy and severe aortic valvular stenosis, consideration for TAVR and alcohol septal ablation should only be made for patients who are at high surgical risk or cannot tolerate thoracotomy.

Background: Tetralogy of Fallot (ToF) is a cardiac malformation that accounts for up to 1/10 of all congenital diseases. Although surgical repair serves as a treatment of choice, it cannot be performed unless weight and anatomical key factors are favorable. The stenting of the right ventricular outflow tract (RVOT) has become an alternative palliative procedure for ToF as an option to alleviate infundibular obstruction with minimal invasion. Methods: A literature search was conducted through 7 databases, followed by the screening and independent assessment of 6 final studies, using the Newcastle-Ottawa Quality Assessment Scale (NOS). Analysis was then conducted using inverse variance analysis, and cumulative data were presented with forest and funnel plots. Results: Studied patients were referred for RVOT stenting due to the marked obstruction of the pulmonary blood flow, a low birth weight, or a small pulmonary artery size. The analysis revealed a significant increase in O2 saturation (mean difference [MD=18%; 13–23.78). The same trend was observed concerning an increase in the Nakata index (MD=54.59; 10.05–99.14), the right pulmonary artery diameter (MD=2.28; 1.20–3.36), and the left pulmonary artery diameter (MD=1.77; 0.22–3.32). Several complications were found, including tricuspid regurgitation and frequent premature beats. Conclusion: RVOT stenting is considered a feasible palliative treatment for ToF, with its high effectiveness in improving patients’ condition, especially their pulmonary flow. While complications are scarce, several conditions should be noted, particularly for fatal complications. Finally, this study has limitations as catheterization details in studies and diverse infants’ conditions may have caused potential bias.