Quadricuspid Valve Research Articles

Incidence of quadricuspid pulmonary valves at postmortem examination

Quadricuspid pulmonic valve is a rare congenital abnormality and because of its difficult non-invasive assessment, it is usually discovered incidentally at autopsies (reported prevalence in post-mortem specimens ranges from 1 in 400 to 1 in 2000). Unlike a bicuspid pulmonary valve, it rarely presents with clinical complications, such as valvular insufficiency or stenosis. Abnormal function is rarely reported in cases that are not associated with other congenital heart disease. With increased sophistication of imaging coincidental quadricuspid valves autopsy studies are important to understand the anatomical consequences of this finding. Our case series identified 21 QPV cases from the Victorian Institute of Forensic Medicine, Melbourne and St George's University of London, Department of Cardiovascular Pathology. Cases were identified through local database searches and review of autopsy/cardiac examination reports over a 20-year period. Available photographs were also systematically examined. Fifteen cases had causes of death with no direct causality to cardiac valvular pathology alone. Six cases were considered unascertained or similar (sudden arrhythmic death syndrome and sudden unexpected death in epilepsy). The presence of QPV in these instances were uncertain but thought to be unlikely contributory to death, due to the absence of pulmonary valvular complications.

Computed tomography anatomical characteristics based on transcatheter aortic valve replacement in aortic regurgitation.

This study analyzed computed tomography (CT) measurement characteristics and anatomical classifications based on transcatheter aortic valve replacement (TAVR) in patients with aortic regurgitation (AR) to establish a preliminary summary of CT anatomical characteristics and to design a novel self-expanding transcatheter heart valve (THV). This single-center retrospective cohort study included 136 patients diagnosed with moderate-to-severe AR at Fuwai Hospital from July 2017 to April 2022. Patients were classified into four anatomical classifications according to dual-anchoring multiplanar measurement of where THV anchoring took place. Types 1-3 were considered candidates for TAVR, whereas type 4 was not. Among 136 patients with AR, there were 117 (86.0%) tricuspid, 14 bicuspid, and five quadricuspid valves. Dual-anchoring multiplanar measurement showed that the annulus was smaller than left ventricular outflow tract (LVOT) at 2, 4, 6, 8, and 10mm on the annulus. The ascending aorta (AA) 40mm was wider than AA 30mm and AA 35mm, but narrower than AA 45mm and AA 50mm. For 10% oversize of the THV, the proportions of the annulus, LVOT, and AA unable to meet the diameter were 22.8%, 37.5%, and 50.0%, respectively, and the proportions of anatomical classification types 1-4 were 32.4%, 5.9%, 30.1%, and 31.6%, respectively. The novel THV could significantly improve the type 1 proportion (88.2%). Existing THVs cannot meet the anatomical characteristics of patients with AR. Conversely, based on anatomical characteristics, the novel THV could theoretically facilitate TAVR.

Congenital Aortic Valve Repair When the Options aren't Good: Truncus Arteriosus and Transposition of the Great Arteries.

Patients with truncus arteriosus and transposition of great arteries are prone to neo-aortic valve insufficiency. Although presenting at opposite ends of the age spectrum, both conditions tend to be commonly associated with neo aortic root dilatation. In patients with truncus arteriosus there is an additional complexity of quadricuspid valve morphology, which make up the majority of valves requiring repair. A unified approach to all these patients would include reduction and stabilization of the annulus and sinotubular junction, as well as achieving equal and symmetrical coaptation of the valve leaflets. By systematically employing these techniques, valve replacement should be avoidable in most children.

Cardiac magnetic resonance to asess aortic valve anatomy in doubtful cases. Case series from a cardiology clinic

Funding AcknowledgementsType of funding sources: None.IntroductionBicuspid aortic valve (BAV) is the most common congenital cardiac malformation, affecting 1-2% of the population, and several types have been described, each one with different prevalence. Partial raphae have also been described and much rarer congenital affectations of aortic valve also exist (unicuspid, quadricuspid), with its own classification. Although echocardiogram is usually enough for diagnosis, sometimes other exams are needed to assess the anatomy of the valve. In these cases, due to lack of radiation, Magnetic Resonance (MRI) can be performed.MethodsWe reviewed cases from a single general cardiology clinic in which MRI was needed for asses aortic valve anatomy during a period of 9 non-consecutive (due to Covid-19 pandemic) months, Characteristics of the patients, as well as valvular and aortic characteristics, were reported.ResultsThere 8 doubtful cases. 5 men. Mean Age 60 years old (range 21-77, 6 patients 58 or more). 6 cases were non-tricuspid aortic valve: 5 bicuspid, 1 quadricuspid. Except in rare anatomies (bicuspid with 2 cusps type lateral and quadricuspid), with age 21 and 38 respectively (both men), the mean age was 70, ranging from 58 to 77, with 3 cases (50%) women. Except 2 cases, MRI was performed after the first visit, without follow-up time. Taking into account all patients, mean time from first visit to perform MRI was 1,12 years (maximum 6 years in the quadricuspid case). 6 cases had valvular functional anomalies greater than mild (3 women). 5 cases presented aortic dilatation (3 men), 1 man without valvular functional anomalies, 1 woman with tricuspid aortic valve. BAV with 2 cusps type lateral and quadricuspid cases had no aortic dilatation. There were no extracardiac findings in the exams. Figure shows characteristics of patients. Figure 2 shows images from exams (in the superior left and inferior right part of the image are shown BAV with 2 cusps and quadricuspid valve repectively, in diastole and systole).ConclusionsClinicians usually think BAV is a problem of young people, but can be even diagnosed in the eighth decade of life. In these cases, senile changes with valvular degeneration make diagnosis more challenging with doubtful cases. Rare anatomies may be also challenging. MRI seems useful to assess doubtful cases. Although usually performed in the first visit, in some cases it can take years before MRI is performed. In older cases, there may be no sex predominance for BAV. Table: Characteristics of patients Figure. Images from exams

A Unique Hybrid Tricuspid Aortic Valve

Aortic valve (AV) phenotypes range from unicuspid valves to quadricuspid valves. The most common and normal AVs are tricuspid. We have recently published a new classification for bicuspid AVs (BAVs), which highlights the vast morphologic variety of these valves.1 BAVs can range from perfectly symmetric valves, to very tricuspid AV–like phenotypes, such as the BAV Fruste.

Management of Giant Pulmonary Artery Aneurysm with Quadricuspid Valve Stenosis

AbstractThe pulmonary artery aneurysm (PAA) is a rare disease with no well-defined guidelines about the diagnostic criteria and its management. In fact, the indications for surgical treatment and the type of surgical approach are not clear. However, in case of giant PAAs with hypertension and pulmonary valve abnormalities, surgery should be considered as an effective and safe approach to prevent dissections or ruptures. In this report, we describe a successful case of surgical repair, using a Bioconduit with a pericardial patch to recreate the pulmonary artery bifurcation in a 72-year-old male with aneurysm of the pulmonary artery (max diameter: 72 mm), associated with quadricuspid pulmonary valve stenosis and pulmonary hypertension. The procedure was uncomplicated and the follow-up computed tomography scan at 4 months showed correct positioning of the graft with no sign of contrast leakage.

РЕКОНСТРУКТИВНАЯ ХИРУРГИЯ АОРТАЛЬНОГО КЛАПАНА: ПОКАЗАНИЯ, ТЕХНИЧЕСКИЕ АСПЕКТЫ И РЕЗУЛЬТАТЫ

Reconstructive valve-sparing procedures on the aortic valve are one of the most dynamically developing directions in the cardiac surgery. Today cardiac surgeons all over the world prefer the aortic valve sparingoperation using autologous tissues instead biological and mechanical prosthetics. The Ross, Yakub, David, Ozaki procedures have proved their effectives, and their indicators of long-term freedom from reoperations are not inferior to classical prosthetics. In this review the authors describe the key points of the native aortic valve reconstruction. Especially, from the surgical point of view the issues of anatomy of the aortic root and the determination of the optimal «patients» for the valve-sparing procedure are discussed. The principles of reconstruction of various variants of valve development, such as uni-, bi-, three-, and quadricuspid valve anatomy, are presented in details. The approaches to aortic valve repair are described step by step, including a description of the aortic root exposure technique, options for correcting prolapse, eliminating fenestration, and annuloplasty. The evaluation of literature data showed that the overall risks of aortic valve repair in isolation or as a component of a combined intervention are nothigher than in patients with biological or mechanical prosthetics. The violation of the orientation of the commissures, the use of a pericardialcatheter, long-term prolapse, as well as expansion of the annulus fibrous are considred as an independent risk factors of significant regurgitation and reoperations in the long term after reconstruction.

Nightmare Case of Complex Neonatal Truncus Arteriosus

Neonatal management of severe quadricuspid truncal valve insufficiency is very challenging with high risk of postoperative mortality. Aortic valve reconstruction is the unique rescue and should be consider and previously planned in case of truncal valvuloplasty failure. Many extracardiac malformations can be associated to truncus arteriosus, therefore a cardiac and chest computed tomography scan must be performed before the elective surgical repair.

The art of aortic valve repair

The art of aortic valve repair

Abstract 15412: Long-term Outcomes of the Truncal Valve in Truncus Arteriosus

Objectives: Long-term survival in patients with truncus arteriosus (TA) is favorable, but there remains significant morbidity associated with need for ongoing re-interventions. The purpose of this study was to understand the long-term outcomes of the truncal valve (TV) and identify risk factors associated with the need for TV intervention. Methods: We retrospectively reviewed 170 patients who underwent initial TA repair at our institution from 1985-2015. Analysis of long-term outcomes was performed on the 148 patients who survived greater than 30 days post-operatively and to hospital discharge using multivariable competing risks Cox regression modeling. Results: Median follow up time was 12.6 years (IQR 5.0, 22.1 years) after full repair. Freedom from death or transplant at 1, 5, 10, and 20 years was 93.1 ± 2.1%, 88.0 ± 2.7%, 86.2 ± 3.0% and 78.3 ± 4.1%. Thirty patients (20%) underwent at least one intervention on the TV (22 repairs, 21 replacements). Freedom from any TV intervention at 1, 5, 10 and 20 years was 99 ± 1%, 94 ± 8%, 82 ± 9%, and 70 ± 5%. Of those with TV repair, 59% subsequently underwent TV replacement. Independent risk factors for need for TV intervention included ≥moderate TV regurgitation (TVR) (HR 4.77, p<0.001) or stenosis (HR 4.12, p<0.001) prior to full repair, and ≥moderate TVR at initial hospital discharge after full repair (HR 8.6, p<0.001). A single coronary ostium was also independently associated with need for TV intervention (HR 6.94, p=0.01). Quadricuspid valve morphology and TV repair at initial TA repair, risk factors in univariate analysis, were not independent predictors on multivariable analysis. Overall, 28% of patients progressed to ≥moderate TVR and to Z-scores of greater than 5 for valve dimensions. Conclusion: Long-term need for TV intervention remains significant. Moderate or worse initial TVR or stenosis, residual TVR after initial TA repair, and single coronary ostium are risk factors associated with need for subsequent TV intervention.

Surgical treatment for a pulmonary artery aneurysm with stenosis of the quadricuspid pulmonary valve.

We report a case of pulmonary artery aneurysm and pulmonary stenosis with a quadricuspid pulmonary valve in a 64-year-old woman. While pulmonary artery disease had been found as a child, she needed no treatment because of the absence of symptoms and her normal physical development. She began experiencing dyspnoea on exertion 3 years ago. Echocardiography showed pulmonary artery aneurysm and severe pulmonary stenosis with a quadricuspid valve. Computed tomography showed a pulmonary artery aneurysm extending from its main trunk to the bilateral branches. The pulmonary valve was replaced, and the pulmonary trunk and its bilateral branch were reconstructed. The patient's postoperative course was uneventful.

Aortic valve fenestrations: a review.

Background:Aortic valve fenestrations (AVFs) seem to be relatively common; however, their impact in human heart disease is not entirely clear.Methods:A review was carried out to assess all scientific literature on human patients related to AVFs, as described in the published literature. The search was conducted on 2 different databases, Medline (PubMed), and ISI Web of Knowledge.Results:Fifty-five reports were under analysis. Autopsy studies showed AVFs to be present in 55.9% of individuals studied in such studies. They occur more frequently in men and, in general, their frequency increases with age. Although common, fenestrations rarely cause regurgitation; however, they may play an important role in the pathophysiology of some cases of severe aortic regurgitation. AVFs have been described in patients with Down syndrome and Marfan syndrome, in patients with bicuspid or quadricuspid valves, and in patients with myxomatous valvular degeneration. Echocardiographic assessment of aortic regurgitation seems to have limitations in the diagnosis of valvular fenestrations.Conclusions:Fenestrations of the aortic valve are very common and are associated with certain clinical conditions. It is unknown if AVFs play any role in the current epidemic of aortic valve disease. Future studies should aim to better define the role of AVFs in aortic valve disease, to further understand its etiology and to develop diagnostic criteria.

Neonatal quadricuspid truncal valve repair with left coronary artery unroofing

Neonatal quadricuspid truncal valve repair with left coronary artery unroofing

Early and mid-term results of isolated aortic valve neocuspidization in patients with aortic stenosis.

This study aimed to elucidate the surgical outcome of aortic valve neocuspidization (AVNeo) in patients with aortic stenosis (AS). From December 2010 to June 2017, we performed AVNeo for aortic valve pathologies in 144 patients. Of them, we evaluated 57 patients with AS who underwent AVNeo. Their mean age was 77.5 ± 8.8years. Fifty-five patients had AS from degenerative changes, 1 from pericardium endocarditis, and 1 from prosthetic valve endocarditis. Forty patients had a tricuspid aortic valve, 1 had a unicuspid valve, 14 had a bicuspid valve, 1 had a quadricuspid valve, and 1 had postoperative aortic valve replacement (AVR). Preoperative echocardiography revealed an average peak pressure gradient of 89 ± 32.9mmHg and a mean pressure gradient of 52 ± 18.8mmHg. The surgical procedure complies with the 3 cuspid suturing to the aortic annulus with the glutaraldehyde-treated pericardium. There were no conversions to AVR. There were 2 noncardiac-related deaths owing to liver failure and sepsis. Postoperative echocardiography showed an average peak pressure gradient of 22 ± 10.7mmHg 1week after the procedure and 19.2 ± 9.7mmHg 20months after the procedure. Two patients underwent reoperation owing to infective endocarditis and recurrent aortic regurgitation. The mean follow-up period was 30.4 ± 20.8months. The freedom from reoperation rates was 98.1 and 95.3% at 12 and 81months of follow-up, respectively. AVNeo is suitable for patients with AS considering its early and mid-term outcomes. Verification of long-term outcomes and reliability is necessary.

Severe Aortic Regurgitation Caused by Congenital Quadricuspid Aortic Valve With an Anomalous Cord

Summary An aortic valve in a 71-year-old female exhibited a quadricuspid valve morphology. Two-dimensional transesophageal echocardiography showed an incompletely separated non-coronary cusp and severe aortic regurgitation. Moreover, real time three-dimensional transesophageal echocardiography indicated the presence of an anomalous cord that appeared to be adhered to the tip of the two leaflets of the incompletely separated non-coronary cusp.

Midterm outcomes after aortic valve neocuspidization with glutaraldehyde-treated autologous pericardium

ObjectiveWe had previously reported the short-term results of the aortic valve neocuspidization (AVNeo) procedure. We have now evaluated the midterm results with the longest follow-up of 118 months. MethodsFrom April 2007 through December 2015, 850 patients were treated with AVNeo using autologous pericardium. Medical records of these patients were retrospectively reviewed. The procedure was on the basis of independent tricuspid replacement using autologous pericardium. The distances between the commissures were measured with an original sizing device, the pericardial cusp was trimmed using an original template, and then sutured to the annulus. ResultsThere were 534 patients with aortic stenosis, 254 with aortic regurgitation, 61 with aortic stenoregurgitation, 19 with infective endocarditis, and 5 with a previous aortic valve procedure. Besides 596 patients with tricuspid aortic valve, 224 patients had bicuspid valve, 28 had unicuspid valve, and 2 had quadricuspid valve. There were 444 male and 406 female patients. The median age was 71 (range, 13-90) years old. Preoperative echocardiography revealed a peak pressure gradient average of 68.9 ± 36.3 mm Hg with aortic stenosis. Surgical annular diameter was 20.9 ± 3.3 mm. There was no conversion to a prosthetic valve replacement. There were 16 in-hospital mortalities. Postoperative echocardiography revealed a peak pressure gradient average of 19.5 ± 10.3 mm Hg 1 week after surgery and 15.2 ± 6.3 mm Hg 8 years after surgery. Fifteen patients needed reoperation (13 infective endocarditis, 1 break of thread, and 1 tear of cusp case). The mean follow-up period was 53.7 ± 28.2 months. Actuarial freedom from death, cumulative incidence of reoperation, and that of recurrent moderate aortic regurgitation or greater was 85.9%, 4.2%, and 7.3%, respectively, with the longest follow-up of 118 months. ConclusionsThe midterm outcomes of AVNeo using autologous pericardium were satisfactory in 850 patients with various aortic valve diseases. However, further randomized, multicenter prospective studies are needed to confirm the results of the current study.

Improved Semiautomated 4D Flow MRI Analysis in the Aorta in Patients With Congenital Aortic Valve Anomalies Versus Tricuspid Aortic Valves.

The aim of this study was to systematically investigate a newly developed semiautomated workflow for the analysis of aortic 4-dimensional flow MRI and its ability to detect hemodynamic differences in patients with congenitally altered aortic valve (bicuspid or quadricuspid valves) compared with tricuspid aortic valves. Four-dimensional flow MRI data were acquired in 20 patients with aortic dilatation (9 tricuspid aortic valves, 11 congenitally altered aortic valves). A semiautomated workflow was evaluated regarding interobserver variability, accuracy of net flow, regurgitant fraction and peak systolic velocity, and the ability to detect differences between cohorts. Results were compared with manual segmentation of vessel contours. Despite the significantly reduced analysis time, a good interobserver agreement was found for net flow and peak systolic velocity, and a moderate agreement was found for regurgitation. Significant differences in peak velocities in the descending aorta (P = 0.014) could be detected. Four-dimensional flow MRI-based semiautomated analysis of aortic hemodynamics can be performed with good reproducibility and accuracy.

From unicuspid to quadricuspid: Influence of aortic valve morphology on aortic three‐dimensional hemodynamics

PurposeTo assess the impact of aortic valve morphology on aortic hemodynamics between normal tricuspid and congenitally anomalous aortic valves ranging from unicuspid to quadricuspid morphology.Materials and MethodsAortic three‐dimensional (3D) blood flow was evaluated by 4D flow MRI in 14 healthy volunteers with normal trileaflet valves and 14 patients with unicuspid (n = 3), bicuspid (n = 9, 3 “true” bicuspid, 3 right–left (RL), 3 right‐noncoronary (RN) leaflet fusion, and quadricuspid aortic valves (n = 2). Data analysis included the co‐registered visualization of aortic valve morphology with systolic 3D blood flow. The influence of valve morphology on aortic hemodynamics was quantified by valve flow angle.ResultsAll RL‐bicuspid aortic valve (BAV) were associated with flow jets directed toward the right anterior aortic wall while RN‐fusion and unicuspid valves resulted in flow jet patterns toward the right‐posterior or posterior wall. Flow angles were clearly influenced by valve morphology (47° ± 10, 28° ± 2, 29° ± 18, 18° ± 12, 15° ± 2 for unicuspid, true BAV, RN‐BAV, RL‐BAV, quadricuspid valves) and increased compared with controls (7.2° ± 1.1, P = 0.001).ConclusionAltered 3D aortic hemodynamics are impacted by the morphology of congenitally malformed aortic valves.J. Magn. Reson. Imaging 2014;40:1342–1346. © 2013 Wiley Periodicals, Inc.

Bicuspid aortic valve and aortic disease. The role of family screening

Introduction and objectives: Bicuspid aortic valve (BAV) is the most common congenital heart disease. Recent studies have shown a certain family aggregation, suggesting that echocardiographic screening in first-degree relatives (FDR) would permit early diagnosis of valve and/or aortic abnormalities in this population. However, the usefulness of this strategy has not been validated. We aimed to test the usefulness of systematic echocardiographic screening of FDR of patients with a confirmed BAV. Methods: Ninety consecutive families of patients with an echocardiographic diagnosis of BAV (75% males; 86.6% anteroposterior configuration) were studied. FDR underwent a study protocol that included transthoracic echocardiogram. Valve anatomy, aortic diameters at different levels, and presence and degree of valve dysfunction were analysed. Results: Of the 401 FDR recruited: 113 (28.1%) refused the study and 288 (71.9%) underwent screening (mean age: 36.1±19 years; 66.7% males). Twenty-one aortic valve abnormalities were encountered: 20 BAV and 1 quadricuspid valve, distributed in 17 of the 90 families, yielding a BAV family prevalence of 18.8% (Table). BAV phenotype was not consistent among probands and positive FDR in 6 of the affected families (35.3%). The prevalence of BAV among the FDR screened was 7.3%, higher than that reported in the general population (0.5-1.0%). The number of FDR required to screen for identifying a new BAV case is 14, in contrast to 100 in the general population. Characteristics of probands and relative FDR, First-degree relatives; SD, standard deviation. Conclusions: The prevalence of BAV is higher in FDR of BAV patients than in the general population. Discordance between BAV phenotypes within the same family is not rare. Echocardiographic screening of FDR is a useful approach for identifying asymptomatic cases, and should therefore be offered to all FDR of BAV individuals.

Truncal valve repair using autologous pericardial patch augmentation

A 35-month-old boy with severe truncal valve regurgitation underwent successful repair by tricuspidization using raphe slicing and an extension technique with glutaraldehyde-treated autologous pericardium. His truncal valve regurgitation improved from severe to mild 5 years postoperatively. Tricuspidization using raphe slicing and valve extension with autologous pericardium might be one of the alternatives for truncal valve regurgitation with a quadricuspid valve.