Left Ventricular Pseudoaneurysm in an Adult With a Repaired Partial Atrioventricular Canal Defect.
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SUBMITTER: Wershing K
PROVIDER: S-EPMC10954580 | biostudies-literature | 2024 Mar
REPOSITORIES: biostudies-literature
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Left Ventricular Pseudoaneurysm in an Adult With a Repaired Partial Atrioventricular Canal Defect.
Wershing Kathryn K Colon Joseb J Alegria Jorge J Paolillo Joseph J Wallihan Daniel D Schwartz Matthew M
CASE (Philadelphia, Pa.) 20240308 3Part A
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Project description:BackgroundLeft ventricular outflow tract stenosis and atrioventricular valve regurgitation are often problems encountered in adulthood after complete atrioventricular septal defect repair. The surgical approach and indications for managing long-term outcomes such as left atrioventricular valve regurgitation and left ventricular outflow tract stenosis after complete atrioventricular septal defect repair have been discussed.Case presentationA 23-year-old woman with intellectual disability was diagnosed with complete atrioventricular septal defect and underwent two-patch repair without cleft closure in childhood. Follow-up examination in adulthood demonstrated moderate left-sided atrioventricular valve regurgitation and left ventricular outflow tract stenosis with a circumferential ridge (peak velocity, 3.7 m/s; pressure gradient, 54 mmHg). Intraoperative findings showed a circumferential ridge under the aortic valve, and we removed the ridge. In addition, a cleft was present at the anterior leaflet, and we completely closed the cleft. Anticoagulation therapy was not initiated, and no embolic complications occurred. Follow-up echocardiography demonstrated no ridge under the aortic valve and only mild-range left AVVR.ConclusionsWe successfully performed surgical treatment without valve replacement or anticoagulation therapy in a patient with poor medical compliance. Delayed reoperation leads to degeneration of the valve structure and makes more difficult to repair. Atrioventricular valve regurgitation should be evaluated in combination with based on the etiology of the regurgitation especially cleft related or not, in addition to the dilatation annulus, cleft size, and depth of the leaflet coaptation depth, and associated other valve diseases.
| S-EPMC11443635 | biostudies-literature
Project description:BackgroundLeft atrioventricular valve regurgitation (LAVVR) after atrioventricular canal (AVC) repair remains a significant cause of morbidity. Papillary muscle arrangement may be important. To investigate the implications of left mural leaflet morphology, we examined anatomic characteristics of the LAVV to determine possible associations with postoperative LAVVR.MethodsAll patients with biventricular AVC repair at our institution between January 1, 2011, and December 31, 2016, with necessary imaging were retrospectively reviewed. We assessed papillary muscle structure and novel measures of the left mural leaflet from preoperative echocardiograms and the degree of LAVVR from the first and last available follow-up echocardiograms. Associations with degree of early and late postoperative LAVVR were assessed with t tests, analysis of variance, or χ2 or Fisher exact tests, and multivariable logistic regression.ResultsThere were 58 of 156 patients (37%) with significant (moderate or severe) early postoperative LAVVR, and 30 of 93 (32%) had significant LAVVR after 6 or more months. Fewer patients with closely spaced or asymmetric papillary muscles had moderate or severe late LAVVR vs those with widely spaced papillary muscles (17% vs 40%, P = .019). Controlling for weight at operation, genetic syndromes, and bypass time, widely spaced papillary muscles increased the odds ratio for late LAVVR to 3.6 (P = .026). Larger mural leaflet area was also associated with late LAVVR on univariable (P = .019) and multivariable (P = .023) analyses. One-third of patients with significant late LAVVR had no significant early postoperative regurgitation.ConclusionsMural leaflet and papillary muscle anatomy are associated with late LAVVR after AVC repair. Late regurgitation can develop in the absence of early LAVVR, suggesting different mechanisms.
| S-EPMC7438247 | biostudies-literature
Project description:Congenital heart defects affect 60-85% of patients with RASopathies. We analysed the clinical and molecular characteristics of atrioventricular canal defect in patients with mutations affecting genes coding for proteins with role in the RAS/MAPK pathway. Between 2002 and 2011, 101 patients with cardiac defect and a molecularly confirmed RASopathy were collected. Congenital heart defects within the spectrum of complete or partial (including cleft mitral valve) atrioventricular canal defect were diagnosed in 8/101 (8%) patients, including seven with a PTPN11 gene mutation, and one single subject with a RAF1 gene mutation. The only recurrent mutation was the missense PTPN11 c.124 A>G change (T42A) in PTPN11. Partial atrioventricular canal defect was found in six cases, complete in one, cleft mitral valve in one. In four subjects the defect was associated with other cardiac defects, including subvalvular aortic stenosis, mitral valve anomaly, pulmonary valve stenosis and hypertrophic cardiomyopathy. Maternal segregation of PTPN11 and RAF1 gene mutations occurred in two and one patients, respectively. Congenital heart defects in the affected relatives were discordant in the families with PTPN11 mutations, and concordant in that with RAF1 mutation. In conclusion, our data confirm previous reports indicating that atrioventricular canal defect represents a relatively common feature in Noonan syndrome. Among RASopathies, atrioventricular canal defect was observed to occur with higher prevalence among subjects with PTPN11 mutations, even though this association was not significant possibly because of low statistical power. Familial segregation of atrioventricular canal defect should be considered in the genetic counselling of families with RASopathies.
| S-EPMC3548264 | biostudies-literature
Project description:Recognizing true from pseudo left ventricular aneurysm after myocardial infarction is paramount to guide clinical management and determine need for surgical urgency. We discuss a case of a postinfarction pseudoaneurysm that poses unique anatomic challenges and may hold a secret “DaVinci code” beyond current diagnostic criteria. (Level of Difficulty: Advanced.) Central Illustration
| S-EPMC9998713 | biostudies-literature
Project description:BackgroundSurgical and perioperative improvements permit earlier repair of partial and transitional atrioventricular septal defects (AVSD). We sought to describe contemporary outcomes in a multicenter cohort.MethodsWe studied 87 patients undergoing primary biventricular repair of partial or transitional AVSD between June 2004 and February 2006 across seven North American centers. One-month and 6-month postoperative data included weight-for-age z-scores, left atrioventricular valve regurgitation (LAVVR) grade, residual shunts, and left ventricular ejection fraction. Paired methods were used to assess 6-month change.ResultsMedian age at surgery was 1.8 years; median weight z-score was -0.88. Median days for ventilation were 1, intensive care 2, and hospitalization 5, all independent of age, with 1 in-hospital death. At 1 month, 27% (16 of 73) had ejection fraction less than 55%; 20% (17 of 87) had significant LAVVR; 2 had residual shunts; 1 each had subaortic stenosis and LAVV stenosis. At 6 months (n = 60), there were no interim deaths, reinterventions, or new development of subaortic or LAVV stenosis. Weight z-score improved by a median 0.4 units (p < 0.001), especially for underweight children less than 18 months old. Left atrioventricular valve regurgitation occurred in 31% (change from baseline, p = 0.13), occurring more frequently in patients repaired at 4 to 7 years (p = 0.01). Three patients had ejection fraction less than 55%, and 1 had a residual atrial shunt.ConclusionsSurgical repair for partial/transitional AVSD is associated with low morbidity and mortality, short hospital stays, and catch-up growth, particularly in underweight children repaired between 3 and 18 months of age. Left atrioventricular valve regurgitation remains the most common residual defect, occurring more frequently in children repaired after 4 years of age.
| S-EPMC2900854 | biostudies-literature
Project description:ObjectiveAlthough a narrow left ventricular outflow tract in atrioventricular septal defect is related to its intrinsic morphology, the contribution from the repair technique remains to be quantified.MethodsA total of 108 patients with an atrioventricular septal defect with a common atrioventricular valve orifice were divided into 2 groups: 2-patch (N = 67) and modified 1-patch (N = 41) repair. The left ventricular outflow tract morphometric was analyzed by quantifying the degree of disproportion between subaortic and aortic annular dimensions (disproportionate morphometrics ratio was defined as ≤ 0.9). Z-scores (median, interquartile range) were further analyzed in a subset of 80 patients with immediate preoperative and postoperative echocardiography. A total of 44 subjects with ventricular septal defects served as controls.ResultsBefore repair, 13 patients (12%) with an atrioventricular septal defect had disproportionate morphometrics (vs 6 [14%] ventricular septal defect P = .79), but the subaortic Z-score (-0.53, -1.07 to 0.06) was lower than the ventricular septal defect (0.07, -0.57 to 1.17; P < .001). After repair, both 2-patch (8 [12%] preoperatively vs 25 [37%] postoperatively; P = .001) and modified 1-patch (5 [12%] vs 21 [51%], P < .001) procedures showed a greater degree of disproportionate morphometrics. Both 2-patch (postoperatively -0.73, -1.56 to 0.08 vs preoperatively -0.43, -0.98 to 0.28; P = .011) and modified 1-patch (-1.42, -2.63 to -0.78 vs -0.70, -1.18 to -0.25; P = .001) procedures also demonstrated lower subaortic Z-scores postrepair. The postrepair subaortic Z-scores were lower in the modified 1-patch group (-1.42 [-2.63 to -0.78]) compared with the 2-patch group (-0.73 [-1.56 to 0.08]; P = .004). Low postrepair subaortic Z-scores (<-2) were observed in 12 patients (41%) in the modified 1-patch group and 6 patients (12%) in the 2-patch group (P = .004).ConclusionsSurgical correction resulted in greater disproportionate morphometrics seen immediately postrepair. The impact on the left ventricular outflow tract was observed in all repair techniques, with a greater burden seen after modified 1-patch repair.Video abstractThis morphometric study in AVSD with common atrio-ventricular valve orifice confirmed further derangements of LV outflow tract morphometrics immediately after surgical repair.
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Project description:The simultaneous occurrence of an atrioventricular canal defect (AVCD) and Ebstein's anomaly is extremely rare, occurring in less than 0.5% of all patients with AVCD. Only 22 cases are described in the literature. This patient's antenatal diagnosis of both Ebstein's anomaly and partial AVCD was made at 25 weeks of gestation. The delivery was organized in a tertiary center. The initial neonatal course was difficult but with adequate treatment, a rapid improvement allowed for a gap of almost 2 years before a complete surgical repair including a cone tricuspid plasty. To our knowledge, this is the first case of antenatal diagnosis, with carefully tailored delivery, neonatal care and subsequent follow-up before indication for successful surgery.
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