Project description:IntroductionVentricular septal defect (VSD) is one of the most common congenital cardiac anomalies. Patients with perimembranous VSD may have aortic regurgitation (AR) secondary to prolapse of the aortic cusp.Case presentationWe present a case of 23-year-old White man with VSD, AR and ascending aortic aneurysm. The patient presented to outpatient clinic with weakness and gradual worsening shortness of breath for the past 5 years. Clinical examination revealed regular heart rhythm and loud continuous systolic-diastolic murmur (Lewin's grade 6/6), heard all over the precordium, associated with a palpable thrill. The ECG showed right axis deviation, fractionated QRS in V1 and signs of biventricular hypertrophy. The chest X-ray showed cardiomegaly. Transthoracic and transesophageal echocardiograms showed a perimembranous VSD with moderate restrictive shunt (Qp/Qs = 1.6), aortic regurgitation (AR), and ascending aortic aneurysm. Other clinical and laboratory findings were within normal limits.ConclusionsPerimembranous VSD, may be associated with aortic regurgitation and ascending aortic aneurysm as secondary phenomenon if it is not early diagnosed and successfully treated.

| S-EPMC10601169 | biostudies-literature

Massive aggrecan and versican accumulation in thoracic aortic aneurysm and dissection.

Project description:Proteoglycan accumulation is a hallmark of medial degeneration in thoracic aortic aneurysm and dissection (TAAD). Here, we defined the aortic proteoglycanome using mass spectrometry, and based on the findings, investigated the large aggregating proteoglycans aggrecan and versican in human ascending TAAD and a mouse model of severe Marfan syndrome. The aortic proteoglycanome comprises 20 proteoglycans including aggrecan and versican. Antibodies against these proteoglycans intensely stained medial degeneration lesions in TAAD, contrasting with modest intralamellar staining in controls. Aggrecan, but not versican, was increased in longitudinal analysis of Fbn1mgR/mgR aortas. TAAD and Fbn1mgR/mgR aortas had increased aggrecan and versican mRNAs, and reduced expression of a key proteoglycanase gene, ADAMTS5, was seen in TAAD. Fbn1mgR/mgR mice with ascending aortic dissection and/or rupture had dramatically increased aggrecan staining compared with mice without these complications. Thus, aggrecan and versican accumulation in ascending TAAD occurs via increased synthesis and/or reduced proteolytic turnover, and correlates with aortic dissection/rupture in Fbn1mgR/mgR mice. Tissue swelling imposed by aggrecan and versican is proposed to be profoundly deleterious to aortic wall mechanics and smooth muscle cell homeostasis, predisposing to type-A dissections. These proteoglycans provide potential biomarkers for refined risk stratification and timing of elective aortic aneurysm repair.

| S-EPMC5922288 | biostudies-literature

Pure Aortic Regurgitation Complicated by Membranous Interventricular Septum Aneurysm Treated With Transcatheter Aortic Valve Replacement.

Project description:An 89-year-old woman with severe aortic regurgitation and an aneurysmal interventricular membranous septum extending into the aortic annulus underwent successful transcatheter aortic valve replacement. A challenging case because of the risk of valve mispositioning complications attributed to the co-occurrence of pure aortic regurgitation (very low calcium burden) with an aneurysmal interventricular membranous septum. (Level of Difficulty: Intermediate.).

| S-EPMC10039378 | biostudies-literature

Simultaneous transcatheter treatment of ascending aortic aneurysm with aortic and mitral regurgitation: an in vitro study.

Project description:ObjectivesWe sought to explore the efficacy of the endovascular repair of an ascending aortic aneurysm with aortic and mitral regurgitation by 2 novel valved stents.MethodsWe established models of ascending aortic aneurysms combined with aortic and mitral regurgitation in 10 pig hearts, then implanted self-expanding aortic fenestrated and mitral valved stents via the transapical approach. We applied a fluoroscopy-guided in vitro setting to test the approach, then analysed continuous circulating flushing at 37°C. Finally, we determined operating times, echocardiography and changes of coronary flow as well as fenestration alignment with the coronary ostia.ResultsThis approach resulted in a 100% overall technical success rate, excellent handling properties and precise positioning. The time taken to implant the 2 valved stents was 59 ± 12 min. Flow of the left and right coronary arteries did not significantly decrease after the stents were implanted (330.4 ± 12.06 ml/min vs 289.4 ± 5.29 ml/min, P < 0.001; 376.8 ± 10.5 ml/min vs 350.0 ± 14.5 ml/min; P < 0.001). We found no obvious regurgitation and perivalvular leakage; nor did the gradients of the aortic and mitral valves as well as of the left ventricular outflow tract increase significantly. The final angiographic examination and profile of the coronary opening confirmed the good position of the valved stents, the exclusion of the aneurysm and the patency of both coronary arteries.ConclusionsThese findings indicate the potential for combined transcatheter aortic root and mitral valve replacement in treating aortic root pathologies. In future, in vivo studies are expected to validate this approach and ascertain its durability.

| S-EPMC8923388 | biostudies-literature

Comparison Between Bicuspid and Tricuspid Aortic Regurgitation: Presentation, Survival, and Aorta Complications.

Project description:BackgroundAlthough the Asian population is growing globally, data in Asian subjects regarding differences between bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) in aortic regurgitation (AR) remain unexplored.ObjectivesThe aim of this study was to examine differences between Asian BAV-AR and TAV-AR in significant AR, including aorta complications.MethodsThe study included 711 consecutive patients with chronic moderate to severe and severe AR from 2008 to 2020. Outcomes included all-cause death, aortic valve surgery (AVS), and incidence of aortic dissection (AD).ResultsThere were 149 BAV-AR (mean age: 48 ± 16 years) and 562 TAV-AR (mean age: 68 ± 15 years; P < 0.0001) patients; baseline indexed left ventricle and indexed aorta size were larger in TAV-AR. Total follow-up was 4.8 years (IQR: 2.0-8.4 years), 252 underwent AVS, and 185 died during follow-up; 18 cases (only 1 BAV) of AD occurred, with a mean maximal aorta size of 60 ± 9 mm. The 10-year AVS incidence was higher in TAV-AR (51% ± 4%) vs BAV-AR (40% ± 5%) even after adjustment for covariates (P < 0.0001). The 10-year survival was higher in BAV-AR (86% ± 4%) vs TAV-AR (57% ± 3%; P < 0.0001) and became insignificant after age adjustment (P = 0.33). Post-AVS 10-year survival was 93% ± 5% in BAV-AR and 78% ± 5% in TAV-AR, respectively (P = 0.08). The 10-year incidence of AD was higher in TAV-AR (4.8% ± 1.5%) than in BAV-AR (0.9% ± 0.9%) and was determined by aorta size ≥45 mm (P ≤ 0.015). Compared with an age- and sex-matched population in Taiwan, TAV-AR (HR: 3.1) had reduced survival (P < 0.0001).ConclusionsOur findings suggest that TAV-AR patients were at a later stage of AR course and had a high AD rate as opposed to BAV-AR patients in Taiwan, emphasizing the importance of early referral for timely management. Surgery on the aorta with a lower threshold in TAV-AR should be considered.

| S-EPMC9627857 | biostudies-literature

Aortic valve regurgitation that resolved after a ruptured coronary sinus aneurysm was patched.

Project description:Sinus of Valsalva aneurysms appear to be rare. They occur most frequently in the right sinus of Valsalva (52%) and the noncoronary sinus (33%). More of these aneurysms originate from the right coronary cusp than from the noncoronary cusp. Surgical intervention is usually recommended when symptoms become evident. We report the case of a 34-year-old woman who presented with a congenital, ruptured sinus of Valsalva aneurysm that originated from the noncoronary cusp. Moderate aortic regurgitation was associated with this lesion. Simple, direct patch closure of the ruptured aneurysm resolved the patient's left-to-right shunt and was associated with decreased aortic regurgitation to a degree that valve replacement was not necessary. Only trace residual aortic regurgitation was evident after 3 months, and the patient remained free of symptoms after 6 months. Our observations support the idea that substantial runoff blood flow in the immediate supra-annular region can be responsible for aortic regurgitation in the absence of a notable structural defect in the aortic valve, and that restoring physiologic flow in this region and equalizing aortic-cusp closure pressure can largely or completely resolve aortic insufficiency. Accordingly, valve replacement may not be necessary in all cases of ruptured sinus of Valsalva aneurysms with associated aortic valve regurgitation.

| S-EPMC3783149 | biostudies-literature

Late-onset aortic regurgitation after device closure of atrial septal defect.

Project description:A 10-year-old girl underwent successful device closure of a 15-mm atrial septal defect with a short and thick retroaortic rim using a 16-mm atrial septal occluder from the conventional approach under transesophageal echocardiographic guidance. There were no periprocedural complications. The aortic valve was noted to be trileaflet and competent. Transthoracic echocardiography at a routine 2-year follow-up visit demonstrated new-onset moderate aortic regurgitation. There was no pericardial effusion.

| S-EPMC11651406 | biostudies-literature

Severe Aortic and Tricuspid Valve Regurgitation after Blunt Chest Trauma: An Unusual Presentation.

Project description: Not available

| S-EPMC7451891 | biostudies-literature