Isolated congenital absence of a single pulmonary valve cusp.
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ABSTRACT:
SUBMITTER: Inoue S
PROVIDER: S-EPMC8690569 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Double-chambered right ventricle (DCRV) is a rare congenital heart disorder involving 2 different right ventricle (RV) pressure compartments that is often associated with ventricular septal defect (VSD). Usually, the obstruction is caused by an anomalous muscle bundle crossing the RV from the interventricular septum to the RV free wall. We are reporting a case of double-chambered right ventricle associated with ventricular septal defect and congenital absence of the pulmonary valve, a rare form of congenital infundibular pulmonary stenosis. In addition to ventricular septal defect, our patient had congenital absence of the pulmonary valve, which is very unusual and has never been reported to our knowledge.
| S-EPMC3966724 | biostudies-literature
Project description:Objectives:We aimed to assess the procedural and clinical results of transcatheter aortic valve replacement (TAVR) for nonraphe bicuspid aortic stenosis (AS) with coronary vs mixed cusp fusion. Background:It remains unclear whether cusp fusion morphology affects TAVR outcomes in patients with nonraphe bicuspid AS. Methods:This retrospective study enrolled consecutive patients with severe symptomatic AS and type-0 bicuspid aortic valve, who underwent TAVR at our institution between 2012 and 2017. TAVR outcomes were defined based on the Valve Academic Research Consortium-2 recommendations. Results:Compared to patients with mixed cusp fusion (44/71), those with coronary cusp fusion (27/71) had a larger ellipticity index for the aortic annulus (21.9%?±?9.0% vs 15.6%?±?9.3%, p=0.007) and increased left ventricular outflow tract obstruction (31.1%?±?9.4% vs 26.9%?±?7.5%, p=0.04) but comparable rates of second valve implantation (15.9% vs 14.8%), mild paravalvular leakage (PVL, 38.5% vs 30.2%), permanent pacemaker implantation (PPM, 25.9% vs 15.9%), and 30-day mortality (7.4% vs 6.8%). Use of a first-generation transcatheter heart valve was associated with higher risk for mild PVL (odds ratio (OR)?=?4.37; 95% confidence interval (95% CI)?=?1.14-16.75; p=0.03) but not PPM (OR?=?0.77; 95% CI?=?0.22-2.62; p=0.67), whereas a larger oversizing ratio tended to be associated with a higher PPM rate (OR?=?1.49; 95% CI?=?0.46-4.86; p=0.51) but lower incidence of mild PVL (OR?=?0.51; 95% CI?=?0.19-1.35; p=0.17). Conclusions:In AS patients with type-0 bicuspid valves, cusp fusion morphology does not affect the procedural or clinical results of TAVR. Use of second-generation transcatheter heart valves may provide more favorable results in such patients. This trial is registered with NCT01683474.
| S-EPMC6874966 | biostudies-literature
Project description:BackgroundSurgical implantation rates of bioprosthetic valves, especially the use of sutureless or rapid deployment valves, as well as the advent of transcatheter valve implantation (TAVR) have increased during the last decades mainly due to their excellent haemodynamic and clinical results. One common characteristic of all bioprosthetic types of surgical aortic valve replacement (SAVR) and TAVR is the risk of early degeneration, which leads to valve-dysfunction and is associated with higher rates of valve reinterventions. Recent studies have demonstrated that cusp thrombosis may play a role in early valve dysfunction. This case report is, to our knowledge, the first documentation on a successful treatment of early aortic valve (AV) degeneration of a sutureless AV thrombosis with a valve-in-valve (ViV) TAVR implantation.Case summaryA 77 years old woman was re-evaluated from the heart-team, which considered the following characteristics: severe impairment of mobility and frailty with an STS-score of 10.01% and a EuroSCORE II of 6.9%. Due to the high surgical risk for SAVR, we decided to perform a ViV-TAVR using a balloonexpandable bioprosthesis. The procedure was performed via transfemoral access under general anaesthesia using a 23 mm Edwards-Sapien 3 bioprosthesis without balloon-valvuloplasty and with nominal-volume dilation under rapid-pacing.DiscussionThe differentiation of bioprosthesis valve thrombosis, and hypoattenuating leaflet thickening vs. structural valve degeneration can be difficult, and a multimodality imaging approach, comprising trans-thoracic echocardiogram, transoesophageal echocardiography and computed tomography, useful. These investigations are very important to decide the right strategy of surgical valve replacement vs. TAVR.
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Project description:BackgroundIntercostal lung herniation is a rare condition that may be congenital (20%) or acquired (80%). The isolated congenital form is exceptional, with one case reported in the literature.Case presentationWe report a case of a 10-year-old French boy of Algeria origin, born with intermittent swelling of his right hemithorax. The swelling and pain gradually increased with age. A clinical examination revealed a localized swelling of his right hemithorax at the level of the midclavicular line and the fifth intercostal space. The swelling increased in size during respiratory movements and enlarged with Valsalva maneuvers. The intercostal lung hernia was treated by thoracoscopy.ConclusionsThis is the second case of isolated congenital intercostal pulmonary hernia reported in the French and English literature. It is the first to be treated by thoracoscopy. Based on this case we performed a review of the diagnosis and therapeutic aspect of pulmonary hernias.
| S-EPMC6637642 | biostudies-literature
Project description:IntroductionCongenital unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly with the complete absence of intrapericardial segment of one of the branch pulmonary arteries. Sixty percent are associated with other congenital heart defects (CHD) that often need correction.AimTo analyze the data of patients with UAPA and ipsilateral non-unifocalizable major aortopulmonary collateral arteries (MAPCAs) associated with other CHD to identify the commonly associated CHD, their management strategies and outcomes.Materials and methodsRetrospective data of patients admitted for congenital UAPA with other CHD was compiled from hospital records from 2002 to 2015. The associated CHD were categorized as group I with the decreased pulmonary flow and group II with increased pulmonary flow to the unaffected contralateral pulmonary artery. The determinants of their management were analyzed.ResultsSixty-five patients of UAPA and ipsilateral non-unifocalizable MAPCAs associated with other CHD were identified. Group I had 41 patients and Group II had 24. The most common CHD associated with UAPA was tetralogy of Fallot (TOF) in 31 patients (47.7%). Fifty-three patients underwent surgery, 48 (73.8%) underwent single lung corrective surgery, 5 (7.6%) palliative surgery and 12 (18.4) received no surgery. Four operated patients died in the immediate postoperative period. The lowest Mc Goon ratio and Nakata index to undergo corrective surgery were 1.0 and 87.4 mm2/m2. A follow-up of 21 patients was done, among which 11 patients who underwent single-stage corrective surgery, all are in NYHA class II and saturating above 95%.ConclusionsCongenital UAPA is a rare anomaly and associated with a variety of CHDs, TOF being the most common. Single lung corrective surgery in patients with ipsilateral non-unifocalizable MAPCAs has good immediate and long term survival.
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Project description:The AngioVac (Angiodynamics) system is indicated for the removal of right-sided venous soft thrombi and emboli. This is the first report that demonstrates the AngioVac system can be extended to the extraction of right-sided cardiac tumors, in the current case, a pulmonary valve papillary fibroelastoma infected with Streptococcus salivarius and Rothia species. (Level of Difficulty: Advanced.).
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Project description:A 42-year-old female patient of aplastic anaemia on maintenance blood transfusion presented with a 3-week history of fever, cough, dyspnoea and pedal oedema. Upon examination she was found to have severe pallor, temperature of 101°F, tachycardia, bilateral pitting pedal oedema, raised jugular venous pressure, ejection systolic murmur (grade 2/6) in pulmonary area and petechiae over extensor aspect of both lower limbs. Blood investigations revealed low haemoglobin, thrombocytopaenia and mild increase in serum creatine. Chest x-ray was normal. Initial 2D trans thoracic echocardiography performed after hospital admission was normal. Antibiotics were started empirically to treat a possible underlying infection. Subsequently, three sets of blood cultures grew Enterococcus faecalis. Upon searching for the source, repeat echocardiograph done showed 2×0.5 cm vegetation on both pulmonary leaflets with severe pulmonary regurgitation, all other valves were free of vegetations. She was treated with intravenous antibiotics for the endocarditis and improved.
| S-EPMC3618698 | biostudies-other
Project description:Based on the natural mathematical relationships between the components of the human tri-leaflet aortic valve, new calibrated cusp sizers were developed in order to facilitate aortic valve assessment in the operating room and enhance the chance for a perfect restoration of aortic valve competence. These sizers were used clinically to guide the implementation of established aortic valve repair techniques in 10 consecutive patients with severe aortic valve regurgitation. Valve repair was successful in all cases, and at a median follow-up was 5.5 months, aortic valve function remained stable, with aortic regurgitation ?1+ in every patient and no significant gradient across the aortic valves. This preliminary clinical experience indicates that the calibrated cusp sizers can provide reliable insight into the mechanism of aortic valve insufficiency, and can guide aortic valve repair techniques successfully. We hope that the simplicity and reproducibility of this method would assist in its dissemination and further increase the percentage of aortic valves that are repaired when compared with current practice.
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