Intervention for critical aortic stenosis in Hutchinson-Gilford progeria syndrome.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients. Intervention to relieve critical AS has the potential for immediate improvement in healthspan and lifespan. However, HGPS patient-device size mismatch, pervasive peripheral arterial disease, skin and bone abnormalities, and lifelong failure to thrive present unique challenges to intervention. An international group of experts in HGPS, pediatric and adult cardiology, cardiac surgery, and pediatric critical care convened to identify strategies for successful treatment. Candidate procedures were evaluated by in-depth examination of 4 cases that typify HGPS clinical pathology. Modified transcatheter aortic valve replacement (TAVR) and left ventricular Apico-Aortic Conduit (AAC) placement were deemed high risk but viable options. Two cases received TAVR and 2 received AAC post-summit. Three were successful and 1 patient died perioperatively due to cardiovascular disease severity, highlighting the importance of intervention timing and comparative risk stratification. These breakthrough interventions for treating critical aortic stenosis in HGPS patients could rewrite the current clinical perspective on disease course by greatly improving late-stage quality of life and increasing lifespan. Expanding worldwide medical and surgical competency for this ultra-rare disease through expert information-sharing could have high impact on treatment success.

Surgical removal of a bullet in the heart through a trans-mitral approach: a case report.

Background: Retained cardiac missiles have been considered bullets, shrapnel, or pellets included in the heart or the pericardial sac. In asymptomatic patients with retained cardiac missiles, the role of surgery is still debated. Case summary: We describe the case of successful surgical treatment of a retained missile localized within the left ventricle in close proximity of the posterior mitral leaflet. The operation was performed through a transatrial approach, detaching the posterior mitral leaflet. The post-operative course was uneventful. The patient was discharged on the seventh post-operative day. At 30-day follow-up visit, the patient was in good conditions, with normal mitral valve function. Discussion: The management of retained cardiac missiles should be individualized. If a decision is made to operate, multiple imaging techniques including echocardiography and computed tomography scan are mandatory to precisely locate the bullet.

Late Surgical Treatment for Transcatheter Aortic Valve Prosthesis Dysfunction.

Structural valve degeneration, valve thrombosis, or acute infective endocarditis may affect the postprocedural outcome of transcatheter aortic valve implanted (TAVI) prostheses. Data of patients who required late surgical explant of TAVI prostheses were obtained from 8 European centers. There were 13 patients who underwent surgical treatment for TAVI prosthesis failure after original admission due to prosthetic infective endocarditis in 6 patients, structural valve degeneration in 4, and valve thrombosis in 3. Hospital mortality was 15%, and survival at the 2-year follow-up was 71%. Abstract word count: 80.

Minimally invasive transatrial repair of posterior left ventricular aneurysm.

Left ventricular aneurysms (LVA) are a complication of myocardial infarction, that rarely involve the posterior wall; surgical repair of posterior LVA poses a technical challenge when associated with concomitant mitral regurgitation. We describe a minimally invasive treatment of ischemic MR and concomitant patch exclusion of posterior LVA through a right minithoracotomy. Using a transatrial approach, the aneurysm is closed with a "U" shaped dacron patch, whose base is anchored to the mitral annulus. Two patients were operated by this method with excellent results.

Hutchinson-Gilford Progeria Syndrome and Severe Aortic Stenosis: A New Hope for Treatment.

Hutchinson-Gilford progeria syndrome is an autosomal dominant, rare, fatal pediatric segmental premature aging disease. Cardiovascular and cerebrovascular diseases constitute the major cause of morbidity and mortality. Patients with the syndrome and severe aortic valve stenosis have been described in the literature, and for all of them a strategy of conservative management has been followed. We describe the first successful treatment of a 23-year-old Hutchinson-Gilford progeria syndrome patient with severe aortic stenosis who underwent transapical transcatheter aortic valve replacement.

Minimally invasive treatment of multiple valve disease: A modified approach through a right lateral minithoracotomy.

BACKGROUND AND AIM OF THE STUDY: Today there is little experience with minimally invasive treatment of multiple valve disease and no standard techniques have been provided yet. We report our early experience with combined aortic and mitral valve surgery with or without tricuspid surgery through a right lateral minithoracotomy (RmT), describing the technical aspects of our approach. METHODS: From April 2017 to April 2019 thirty patients with mitro-aortic valve disease or with triple valve pathology underwent surgery through a 3 to 4 cm lateral RmT into the third intercostal space. Cardiopulmonary bypass was established through femoral vessels cannulation. Surgery on the mitral valve (MV) was performed first and sutures put into the mitral annulus. Aortic valve replacement (AVR) was performed next. Then, the selected ring or prosthetic valve was implanted in a mitral position throughout previously placed sutures. Finally, if required, tricuspid valve surgery was performed. RESULTS: In combined with AVR, MV replacement was performed in 20 patients (66%), and MV repair in 10 patients (34%). Concomitant tricuspid annuloplasty was performed in five patients (17%). There was no conversion to full sternotomy. Postoperatively, one patient died. Postoperative echocardiography showed no perivalvular leakage in aortic or in the mitral position. No residual mitral regurgitation was observed in patients who underwent MV repair. CONCLUSIONS: Minimally invasive surgery of double and triple valve disease is feasible. Our approach through a lateral RmT allows optimal visualization of the aortic, mitral, and tricuspid valves, simplifies the surgical procedure and allows excellent results also in complex MV repair procedures.

Unconventional location for a cardiac lipoma: The anterior papillary muscle.

Primary cardiac tumors are uncommon, and lipoma is the second most frequent benign type after myxoma. The treatment of choice is surgery, with the complete excision of the mass. Nevertheless, in the absence of symptoms and clinical signs the indication for surgery is less defined, and close follow up may be recommended. In the case we are going to report the patient was followed by periodical clinical and echographic examination, always reconsidering the possibility of treatment. .