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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., Competing Interests: Declaration of competing interest None., (Copyright © 2024. Published by Elsevier Inc.)

Transcatheter pulmonary valve implantation (TPVI) is indicated for use in the management of failing pulmonary valves in humans. We report here the long-term follow-up of the first documented transcatheter pulmonary valve implanted in a client-owned dog. A one-year-old Beagle dog with severe congenital type A valvular pulmonic stenosis first underwent percutaneous balloon pulmonary valvuloplasty, leading two years later to severe pulmonary regurgitation. A TPVI using a Melody™ bioprosthetic valve was then successfully performed, with normalization of the right heart cavities. Repeated two- and three-dimensional transthoracic echocardiographic examinations combined with Doppler modes confirmed the appropriate position and function of the valve for four years. Mitral myxomatous valvular degeneration led to refractory left-sided congestive heart failure, and the dog was humanely euthanized. After postmortem examination, X-ray imaging and histopathological evaluation of the stent and the valve were performed. Ex-vivo imaging of the implanted valve using a Faxitron® Path radiography system and microscopic evaluation of the implanted stent and bioprosthetic leaflets did not show any relevant leaflet or stent alterations. This case provides a proof of concept in interventional veterinary cardiology, showing that TPVI can be performed in dogs with subsequent long-term maintaining normal pulmonary valve function., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Fenestrations in semilunar valves of human hearts have been incidentally described during autopsies since the 1800s, and were thought to be a degenerative process of the valve cusps. Due to the nature of autopsy, prior literature has primarily examined these fenestrations in pathologic hearts, and has implicated them in leading to valve insufficiency, regurgitation, and cusp rupture. More recent studies have predicted an increase in fenestration prevalence in the rapidly aging United States and have warned of a potential increase in fenestration-related valvular pathology. Herein, we analyze fenestration prevalence in 403 healthy human hearts and report findings that differ from these prior reports, and emphasize that fenestrations may not necessarily portend significant valvular dysfunction., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest or funding to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Cardiac myxoma is the most common type of primary cardiac tumor, with the majority located in the atrial wall. The tumor is attached to valvular structures in a few cases, of which the pulmonary valve is the least affected. Pulmonary valve myxoma may have different clinical manifestations from the more common cardiac myxomas because of its vital position. A misdiagnosis of these types of cardiac myxoma may be detrimental to the care and well-being of patients. Therefore, this systematic review aims to define the clinical characteristics of pulmonary valve myxoma and how this differs from a more common cardiac myxoma., Methods: Employed literature was obtained from PubMed, ScienceDirect, Scopus, Springer, and ProQuest without a publication year limit on August 23, 2022. The keyword was "pulmonary valve myxoma." Inclusion criteria were as follows: (1) case report or series, (2) available individual patient data, and (3) myxoma that is attached to pulmonary valve structures with no evidence of metastasis. Non-English language or nonhuman subject studies were excluded. Johanna Briggs Institute checklists were used for the risk of bias assessment. Data are presented descriptively., Results: This review included 9 case reports from 2237 articles. All cases show a low risk of bias. Pulmonary valve myxoma is dominated by males (5:4), and the patient's median age is 57 years with a bimodal distribution in pediatric and geriatric populations. The clinical manifestation of pulmonary valve myxoma is often unspecified or asymptomatic. However, systolic murmur in the pulmonary valve area is heard in 67% of cases. Echocardiography remains the diagnostic modality of choice in the majority of cases. Tumor attached to the pulmonary cusps or annulus and extended to adjacent tissues in all cases. Therefore, valve replacement or adjacent tissue reconstructions are required in 77% of cases. The recurrence and mortality are considerably high, with 33% and 22% cases, respectively., Conclusions: Pulmonary valve myxoma is more common in males with a bimodal age distribution, and its outcomes seem worse than usual cardiac myxomas. Increasing awareness of its clinical symptoms, early diagnosis, and complete myxoma resection before the presence of congestive heart failure symptoms are important in achieving excellent outcomes. A firm embolization blockade is needed to prevent myxoma recurrence., (© 2023. The Author(s).)

Background: Cardiac papillary fibroelastoma (PFE) is a rare tumor, and especially rare when found on the pulmonary valve., Case Presentation: We report the case of a 70-year-old woman patient with a pulmonary valve PFE diagnosed incidentally during a follow-up of aortic regurgitation. Computed tomography and magnetic resonance imaging showed no suggestive signs of malignant tumors, and thrombus or myxoma was initially suspected. However, an initial transthoracic and transesophageal echocardiogram did not exclude the possibility of a malignant tumor attached to the wall of the pulmonary artery. Considering the embolization risk, we opted to perform tumorectomy, in which additional surgical procedures could then be conducted if intraoperative diagnosis showed a malignant tumor. Indeed, intraoperative findings showed the tumoral mass attached on the left semilunar cusp of the pulmonary valve, and intraoperative diagnosis of the tumor showed no malignancy. Planned tumorectomy was performed concomitantly with AVR. The pathologic examination of the removed tumor confirmed the diagnosis of PFE. Her postoperative course was uneventful without any sign of recurrence., Conclusion: This case highlights the difficulty of accurate diagnostic imaging and provides valuable insight into a successful surgical treatment of pulmonary valve PFE without any complications., (© 2022. The Author(s).)

Background: Isolated pulmonary valve endocarditis (IPE) is rare, accounting for 1.5-2% of all cases of infective endocarditis. Herein, we describe a case of isolated pulmonary valve endocarditis with rapid progression in a 28-year-old male. Unlike most patients reported previously who were cured with only anti-infective therapy, without surgery at an early stage, multiple complications occurred in this patient in less than 2 weeks., Case Presentation: The patient was diagnosed with pulmonary valve endocarditis with blood cultures showing Staphylococcus aureus and echocardiography revealing 2 masses (measuring 14*13 mm、11*16 mm in size). Only 12 days later, acute massive pulmonary embolism occurred. Then, repeated echocardiography revealed multiple masses attached to the pulmonary valve with severe pulmonary insufficiency and the possibility of pulmonary valve destruction. Finally, pulmonary valve replacement, vegetation removal, and right pulmonary thromboendarterectomy together with resection of the middle and lower lobes of the right lung were performed., Conclusions: The role of surgery at an early stage might need to be reconsidered, and it may be viable to combine medical and surgical approaches.

Introduction: Bronchoscopic lung volume reduction treatment with one-way valves is an effective guideline treatment option for patients with severe emphysema. However, important challenges and adverse reactions may occur after treatment., Areas Covered: This review summarizes the complications after endobronchial and intrabronchial valve treatment that have been described by the currently published randomized controlled trials and other relevant papers regarding the complications and its management. In case there was no relevant literature regarding these subjects, recommendations are based on expert opinion. Complications include pneumothorax, post-obstruction pneumonia and hemoptysis. Also, the treatment may not be effective due to the presence of collateral ventilation or misplaced valves. Furthermore, an initial beneficial effect may vanish due to granulation tissue formation, valve dysfunction or valve migration. Careful follow-up after treatment with valves is important. Evaluation with a CT-scan and/or bronchoscopy is needed if there is no improvement after treatment, loss of benefit, or occurrence of important adverse events during follow-up., Expert Opinion: Treating severe emphysema patients with one-way valves requires continuous dedication and expertise, especially to achieve an optimal outcome and elegantly deal with the various complications after treatment.

A 73-year-old Japanese man was admitted with an asymptomatic pulmonary artery aneurysm. However, chest X-ray and contrast-enhanced thoracic computed tomography revealed a protrusion at the second left branch that in fact was a pulmonary artery aneurysm with a diameter of 50 mm. Transesophageal echocardiography showed a bicuspid pulmonary valve, and cardiac catheterization revealed pulmonary stenosis with a pressure gradient of 45 mmHg, but no other heart diseases were noted. An extremely rare pulmonary artery aneurysm associated with an isolated bicuspid pulmonary valve was diagnosed.

Only a few hundred cases of intimal sarcomas of pulmonary artery (ISPA) were reported on the literature. Diagnosis of this rare entity is a challenging dilemma with the need for a high expertise in the radiological and pathological identification of ISPA. Treatment strategies rely initially on an early aggressive surgery aiming for complete surgical resection with clear margins while no clear recommendations guiding the choice for additional drug therapy or radiotherapy exist. In this article, we perform an extensive review of the literature on ISPA with details on the clinical presentation, diagnosis and management strategies. An additional goal of this paper is to make practicing oncologists aware of this rare entity with clear idea on the initial management., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020. Published by Elsevier B.V.)

Los pacientes con cardiopatías congénitas que afectan la continuidad del ventrículo derecho con la arteria pulmonar deben someterse con frecuencia a intervenciones debido a la limitada vida útil de los conductos quirúrgicos, lo que lleva al desarrollo de disfunción ventricular derecha por cambios en la geometría ventricular y predisposición a arritmias letales, con el consiguiente riesgo de reintervenciones. El implante valvular percutáneo pulmonar es una nueva alternativa terapéutica, menos invasiva en comparación con la quirúrgica, para pacientes seleccionados. Se realiza una revisión de las publicaciones médicas actuales disponibles y se describe la experiencia inicial del implante valvular pulmonar percutáneo en un centro colombiano de alta complejidad para el tratamiento de enfermedades cardiovasculares, en dos pacientes con disfunción del homoinjerto aórtico en posición pulmonar con doble lesión valvular, en los cuales el implante valvular pulmonar percutáneo fue una conducta exitosa. Se eligió a pacientes con cardiopatías congénitas, conductos quirúrgicos disfuncionales con estenosis o insuficiencia pulmonar significativa, y disfunción y dilatación ventricular derechas. Se empleó la técnica regular para el implante de la válvula pulmonar Melody, sin documentarse complicaciones durante el procedimiento ni al año de seguimiento. El implante percutáneo de la válvula pulmonar es un gran avance en el tratamiento de pacientes con cardiopatías congénitas, con resultados favorables a corto y mediano plazos, lo cual hace posible la restauración de la función ventricular con riesgo mínimo, frente al reemplazo quirúrgico en pacientes seleccionados., Patients with congenital heart disease that involves reconstruction of the right ventricular outflow tract must frequently undergo interventions derived from the limited useful life of the surgical canals, which leads to the development of right ventricular dysfunction due to changes in the ventricular geometry and predisposition to lethal arrhythmias, with the consequent risk of reinterventions. The percutaneous pulmonary valvular implant is a new therapeutic alternative, less invasive, compared to surgery, for selected patients. A review of the available literature is made and the initial experience of percutaneous pulmonary valve implantation in a Colombian center of high complexity for the treatment of cardiovascular diseases is described, in two patients with aortic homograft dysfunction in a pulmonary position with double valvular lesion, in which the percutaneous pulmonary valve implant was a successful strategy. Patients with congenital heart disease were chosen, with dysfunctional surgical conduits with stenosis or significant pulmonary insufficiency, with dysfunction and right ventricular dilatation. The standard technique for the implantation of the Melody pulmonary valve was used, without complications during the procedure or one year of follow-up. Percutaneous implantation of the pulmonary valve is a great advance in the management of patients with congenital heart diseases, with favorable results in the short and medium term, allowing the restoration of ventricular function with minimal risk, compared to surgical replacement in selected patients., (Copyright: © 2020 Permanyer.)

Introduction: The clinical importance of mass effect from congenital lung masses on the fetal heart is unknown. We aimed to report cardiac measurements in fetuses with congenital lung masses and to correlate lung mass severity/size with cardiac dimensions and clinical outcomes., Methods: Cases were identified from our institutional database between 2009 and 2016. We recorded atrioventricular valve (AVVz) annulus dimensions and ventricular widths (VWz) converted into z scores, ratio of aortic to total cardiac output (AoCO), lesion side, and congenital pulmonary airway malformation volume ratio (CVR). Respiratory intervention (RI) was defined as intubation, extracorporeal membrane oxygenation (ECMO), or use of surgical intervention prior to discharge., Results: Fifty-two fetuses comprised the study cohort. Mean AVVz and VWz were below expected for gestational age. CVR correlated with ipsilateral AVVz (R S = -.59, P < .001) and ipsilateral VWz (-0.59, P < .001). Lower AVVz and AoCO and higher CVR were associated with RI. No patient had significant structural heart disease identified postnatally., Conclusion: In fetuses with left-sided lung masses, ipsilateral cardiac structures tend to be smaller, but in our cohort, there were no patients with structural heart disease. However, smaller left-sided structures may contribute to the need for RI that affects a portion of these fetuses., (© 2019 John Wiley & Sons, Ltd.)

Background: Precisely evaluating the need for transannular patch (TAP) placement is very important in the surgical treatment of tetralogy of Fallot. We hypothesized that the pulmonary annulus area index (PAAI, the pulmonary-to-aortic valve annulus cross-sectional area ratio) could be a useful and accessible predictor for TAP placement., Methods: The medical records of patients who underwent tetralogy of Fallot repair between 1 January 2016 and 31 December 2017 were reviewed retrospectively. A total of 255 patients were included and categorized into two groups: patients who needed TAP placement and patients who did not. Various candidate predictors for TAP placement (PAAI, pulmonary annulus z-score, and velocity across the pulmonary annulus) were compared using receiver operating characteristic curves. The optimal cutoff for each predictor was assessed., Results: Among the 255 patients included, 156 needed TAP placement (156/237, 65.8%). Both the PAAI (0.28 [0.20/0.34] vs 0.14 [0.09/0.19]; P < .0001) and z-score (-1.5 [-2.9, -0.4] vs -3.6 [-5.3/-2.6]; P < .0001) were smaller in the TAP group. The PAAI is a useful predictor of the pulmonary annulus z-score (AUC 0.830 vs 0.811, P = .19). Combination analysis of the PAAI and velocity across the pulmonary annulus (PV v max ) showed better predictive value than the PAAI and z-score (AUC 0.860, sensitivity 89.7%, specificity 61.7%, P < .0001)., Conclusions: Our results suggest that the PAAI is a useful and accessible predictor for TAP placement and can be applied readily and simply in clinical practice. A combination with the velocity across the pulmonary annulus could promote the accuracy of prediction., (© 2019 Wiley Periodicals, Inc.)

Background: Unlike the vast amount of animal data available on the recellularization of allogenic decellularized heart valves (DHVs), there have only been sporadic histologic reports on such grafts in humans., Methods: Two experienced cardiac pathologists independently evaluated human specimens obtained during reoperation between December 2010 and April 2017 DHVs in seven categories after automated staining (scores: 0 to 6) in comparison with published data. An optimal result of 42 points was classified as 100%., Results: We found that 364 DHVs, 236 decellularized pulmonary homografts (DPHs), and 128 decellularized aortic homografts (DAHs) were implanted, and freedom from explantation was 96.1% (DAH) and 98.7% (DPH). Reoperations were because of (suspected) endocarditis in 5 of 11 patients, stenosis at the subvalvular or valvular or supravalvular level in 3 of 11 patients, planned staged reoperation in 2 of 11 patients, and 1 heart transplantation. Good reader agreement was reflected by an interagreement weighted κ of 0.783 (95% confidence interval: 0.707 to 0.859). The relative histologic score in nonendocarditis specimens was 76% ± 4.3% (maximum 81%). Intracellular procollagen type 1 production was found in recipient mesenchymal cells within the transplanted grafts. In endocarditis specimens the histologic score was significantly lower with 48% ± 7.3% (minimum 41%, p = 0.0004) because of leucocyte infiltration and matrix degradation. One DPH showed immune system-mediated graft failure. Grafts obtained during the first 12 months after implantation were not evenly repopulated with less recellularization in the inner parts; no difference was found between DAH and DPH with respect to extent of recellularization., Conclusions: Substantial in vivo recellularization with noninflammatory cells was observed in this study. Spontaneous recellularization appears to require multiple months, which correspondingly has an impact on size selection for growing patients., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Bioprosthetic pulmonary valve conduits have been reported with an increased risk of endocarditis. Thrombus formation is considered as source of these serious and life-threatening infections. We reviewed a series of explanted valved pulmonary conduits for histological evidence for thrombus formation., Materials and Methods: Explanted bioprosthetic pulmonary valves were fixed in formalin and embedded in paraffin or in methylmethacrylate. Standard staining as well as immunohistochemical staining techniques were applied. Native pulmonary valves of German domestic pigs served as controls., Results: 47 valved pulmonary conduits (Hancock n = 23, Homograft n = 7, Contegra n = 7, Melody n = 7, other n = 3) were analyzed histologically. Average time of implantation had been 63 months (6 to 342 months). Indications for explantation included significant obstruction (n = 45), regurgitation (n = 7), and/or endocarditis (n = 6). In 44/47 (93%) specimen, we found accumulation of thrombotic material at the basis of the semilunar valve sinus to a variable degree. 11 patients had been treated with antiplatelet agents, 2 had received anticoagulants at the time of explantation. There was no suspicion of thrombus formation clinically or echocardiographically prior to explantation in any of the patients. Control porcine pulmonary valves (n = 5) did not show any evidence of accumulation of thrombotic material., Conclusions: In a large series of explanted valved pulmonary conduits, formation of subclinical, mostly non-infectious thrombotic material was an almost ubiquitous finding. We speculate that high incidence of endocarditis in bioprosthetic valves may in part be explained by thrombus apposition at the basis of conduit valve sinus., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: Congenital Heart Disease (CHD) is the leading cause of pediatric mortality, with many cases affecting the right ventricular outflow tract (RVOT) or pulmonary valve (PV). Understanding the mechanics of the disease condition can provide insight into development of durable repair techniques and bioengineered replacement devices. This work presents a mechanical and structural analysis of the pulmonary valve of two pediatric cases., Methods: Two PV tissues were excised as part of the operative procedure. One PV was obtained from a 9-month-old with Noonan syndrome (Patient 1) and the other from a 6-month-old with tricuspid atresia (Patient 2). The leaflets were subjected to planar biaxial tensile testing and second harmonic generation (SHG) imaging for mechanical and structural evaluation., Results and Discussion: Patient 1 exhibited a more anisotropic mechanical response than Patient 2, with sample stiffness on par with that of adult PV tissue. Additionally, both samples showed radial and circumferential alignment of collagen fibers on the ventricularis and fibrosa sides of the leaflets, respectively. Collagen fibers on the fibrosa side were also more crimped than on the ventricularis side., (Copyright © 2018. Published by Elsevier Ltd.)

Background: We reviewed our revised surgical strategy for tetralogy of Fallot (TOF) total correction to minimize early exposure to significant pulmonary regurgitation (PR) and to avoid right ventriculotomy (RV-tomy). Methods and Results: Since February 2016, we have tried to preserve, first, pulmonary valve (PV) function to minimize PR by extensive commissurotomy with annulus saving; and second, RV infundibular function by avoiding RV-tomy. With this strategy, we performed total correction for 50 consecutive patients with TOF until May 2018. We reviewed the early outcomes of 27 of 50 patients who received follow-up for ≥3 months. Mean patient age at operation was 10.2±5.0 months, and mean body weight was 8.8±1.2 kg. The preoperative pressure gradient at the RV outflow tract and the PV z-score were improved at most recent echocardiography from 82.0±7.1 to 26.8±6.4 mmHg, and from -2.35±0.49 to -0.55±0.54, respectively, during 11.1±1.6 months of follow-up after operation. One patient required re-intervention for residual pulmonary stenosis. Twenty-two patients had less than moderate PR (none, 1; trivial, 8; mild, 13), and 5 patients had moderate PR. There was no free or severe PR., Conclusions: At 1-year follow-up, the patients who underwent total TOF correction with our revised surgical strategy had acceptable results in terms of PV function. The preserved PV had a tendency to grow on short-term follow-up.

History: Case 1: admission to hospital of a 72-year-old male patient due to suspect tumor at the pulmonary valve. At admission the patient was hemodynamically stable with known coronary one vessel heart disease and arterial hypertension.Case 2: admission to hospital of a 74-year-old female patient due to symptoms of de novo angina pectoris with known coronary three vessel heart disease., Findings and Diagnosis: In both patients suspect tumors could have been detected at the pulmonary- and tricuspid valve by transthoracic echocardiography. In case 1 coronary angiography showed a complex coronary three vessel heart disease with indication for surgery. In case 2 coronary angiography showed a subtotal stenosis of the RCA which could have been treated by percutaneous coronary intervention (PCI)., Therapy and Course: In both cases the tumor were highly suspective to be benigne. Despite of the certain risk of embolism, surgery was only recommended in case 1 because the patient needed surgical revascularisation anyway due to the complex coronary three vessel heart disease. In case 2 the RCA stenosis has been treated by PCI and surgery has not been recommended., Conclusions: Echocardiography can help to detect cardiac tumors whereas the diagnosis can only be confirmed by biopsy. Further therapeutic procedures should be defined individually., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (© Georg Thieme Verlag KG Stuttgart · New York.)

Pulmonary and/or tricuspid valve dysfunction is common among individuals with congenital heart disease, and surgical intervention often carries prohibitive risks. Transcatheter valve replacement (TVR) of the right-sided cardiac valves has become a viable treatment option over the past two decades, while continued technological development aims to broaden its applicability to an even larger portion of those with repaired congenital heart disease. To date, two transcatheter valves have been approved for use in patients with dysfunctional right ventricular to pulmonary artery conduits as well as those with failing pulmonic bioprosthetic valves, and are also used off-label in the "native" RVOT and within surgically repaired/replaced but failing tricuspid valves. TVR has demonstrated comparable safety and short-term outcomes to that of surgical valve replacement. This article aims to review current available devices, focusing on their safety, efficacy and on and off label usage, while briefly describing some of the emerging devices and novel procedural techniques that will likely lead to significant expansion of transcatheter treatment of right sided valve disease in the future., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Velocity ratio, velocity time integral (VTI) ratio, and pulmonary valve area indexed to body surface area (iPVA) are methods of assessment of pulmonary valve stenosis (PS) severity that are less dependent on blood flow. Studies evaluating these methods are limited., Objectives: To determine the effects of butorphanol, atenolol, and balloon valvuloplasty (BV) on velocity ratio, VTI ratio, iPVA, mean PG, and max PG., Animals: Twenty-seven dogs with PS (max PG >50 mm Hg)., Methods: Prospective study. All dogs underwent an echocardiogram at baseline, 5-minutes after administration of butorphanol (0.2-0.25 mg/kg IV), and 2-to-4 weeks after atenolol (1-1.5 mg/kg q12h). Twenty-one of these were evaluated 24-hours after BV., Results: There were no significant differences (P > .05) amongst any of the methods of assessment of PS severity after butorphanol. After atenolol, mean (SD) of mean (57.0 [21.0] mm Hg) and max PG (93.1 [33.8] mm Hg) were significantly decreased (P ≤ .047) compared with baseline (65.2 [26.2] mm Hg and 108 [44.4] mm Hg, respectively). After atenolol, there were no significant (P ≥ .12) differences in velocity ratio (0.29 [0.09]), VTI ratio (0.18 [0.05]), or iPVA (0.43 [0.16] cm 2 /m 2 ) compared with baseline (0.30 [0.09], 0.19 [0.09], 0.44 [0.17] cm 2 /m 2 , respectively)., Conclusions and Clinical Importance: Atenolol might reduce mean and max PG but does not alter less flow-dependent methods of assessment of PS severity (velocity ratio, VTI ratio, and iPVA) in dogs with PS. Results support an integrative approach to assessment of PS severity that includes less flow-dependent methods, particularly in states of altered flow or right ventricular function., (© 2018 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.)

An 11 yr old female spayed golden retriever weighing 30.3 kg presented for evaluation of progressive lethargy, anorexia, tachypnea, stiff gait, and nonlocalized pain. On physical exam, the patient was febrile and tachycardic, and an arrhythmia with pulse deficits was noted. Clinicopathological abnormalities included thrombocytopenia, leukocytosis, nonregenerative anemia, and mild hypoalbuminemia. The patient progressed overnight to develop a productive cough, and an echocardiogram performed the next morning revealed irregular proliferative lesions of the pulmonic valve with moderate pulmonic regurgitation. Subsequent blood cultures grew two organisms: alpha-hemolytic streptococci spp. and Empedobacter brevis. The dog was treated with appropriate intravenous antibiotics for 2 wk and then switched to oral therapy. The clinicopathologic abnormalities, fever, and clinical signs resolved with oral antibiotic treatment. To the authors' knowledge, this case report represents the first detailed published case of bacterial endocarditis with E brevis bacteremia involving the pulmonic valve. The clinical presentation, diagnosis, treatment, and follow-up are discussed.

Competing Interests: Competing interests: None declared.

Currently available bioprosthetic heart valves have been successfully used clinically; however, they have several limitations. Alternatively, tissue-engineering techniques can be used. However, there are limited data concerning the impact of storage conditions of scaffolds on their biomechanics and morphology. The aim of this study was to determine the effect of different storage conditions on the biomechanics and morphology of pulmonary valve dedicated for the acellular scaffold preparation to achieve optimal conditions to obtain stable heart valve prostheses. Scaffold can then be used for the construction of tissue-engineered heart valve, for this reason evaluation of these parameters can determine the success of the clinical application this type of bioprosthesis. Pulmonary heart valves were collected from adult porcines. Materials were divided into five groups depending on the storage conditions. Biomechanical tests were performed, both the static tensile test, and examination of viscoelastic properties. Extracellular matrix morphology was evaluated using transmission electron microscopy and immunohistochemistry. Tissue stored at 4 °C exhibited a higher modulus of elasticity than the control (native) and fresh acellular, which indicated the stiffening of the tissue and changes of the viscoelastic properties. Such changes were not observed in the radial direction. Percent strain was not significantly different in the study groups. The storage conditions affected the acellularization efficiency and tissue morphology. To the best of our knowledge, this study is the first that attributes the mechanical properties of pulmonary valve tissue to the biomechanical changes in the collagen network due to different storage conditions. Storage conditions of scaffolds for tissue-engineered heart valves may have a significant impact on the haemodynamic and clinical effects of the used bioprostheses.

Objective: In tetralogy of Fallot (TOF), the dominant ventricular tachycardia substrates are slow-conducting anatomical isthmuses. Surgical correction has evolved, which might have influenced isthmus presence and dimensions., Methods: One hundred and forty-two postmortem TOF specimens (84/58 corrected/uncorrected) were studied for isthmus presence. Isthmus 1 is located between the tricuspid annulus and right ventricular (RV) outflow tract (RVOT) patch/RV incision, isthmus 2 between RVOT patch/RV incision and pulmonary valve, isthmus 3 between pulmonary valve and ventricular septal defect (patch), isthmus 4 between ventricular septal defect (patch) and tricuspid annulus. Isthmus width and thickness were measured., Results: Of 84 corrected postmortem TOF specimens (death: 6.6 years (4.0-11.5)), 83 demonstrated isthmus 1 (99%, width=25±10 mm, thickness=5±2 mm), 35 isthmus 2 (42%, width=10±9 mm, thickness=3±2 mm), 83 isthmus 3 (99%, width=10±6 mm, thickness=5±2 mm), and 5 isthmus 4 (6%, width=4±2 mm, thickness=2±1 mm). Transatrial-transpulmonary correction (n=49) as compared with transventricular correction (n=35) prevented isthmus 2 (0% vs 100%, P<0.001). Transatrial-transpulmonary correction at age <1 year (n=7) as compared with ≥1 year (n=42) required a smaller transannular RVOT patch (28±15 vs 45±14 mm, P<0.001). Mode and timing of correction did not influence presence and dimensions of isthmus 3. In corrected and uncorrected TOF specimens (death 1.8 years (0.5-6.6)), the range of isthmus 3 dimensions was broad (width: min=2 mm, max=32 mm; thickness: min=1, max 13 mm) across all ages. Isthmus 3 width and thickness were strongly correlated (r=0.65, P<0.001)., Conclusions: In TOF, the current routine use of transatrial-transpulmonary correction prevents isthmus 2. Correction <1 year reduces transannular patch size, which may influence isthmus 1 width later in life. Mode and timing of correction did not change prevalence and dimensions of isthmus 3, in which dimensions varied widely in uncorrected and corrected TOF., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Tissue-engineered heart valves with self-repair and regeneration properties may overcome the problem of long-term degeneration of currently used artificial prostheses. The aim of this study was the development and in vivo proof-of-concept of next-generation off-the-shelf tissue-engineered sinus valve (TESV) for transcatheter pulmonary valve replacement (TPVR). Transcatheter implantation of off-the-shelf TESVs was performed in a translational sheep model for up to 16 weeks. Transapical delivery of TESVs was successful and showed good acute and short-term performance (up to 8 weeks), which then worsened over time most likely due to a non-optimized in vitro valve design. Post-mortem analyses confirmed the remodelling potential of the TESVs, with host cell infiltration, polymer degradation, and collagen and elastin deposition. TESVs proved to be suitable as TPVR in a preclinical model, with encouraging short-term performance and remodelling potential. Future studies will enhance the clinical translation of such approach by improving the valve design to ensure long-term functionality.

The purpose of this study was to determine the impact of elevated temperature exposure in tissue banking on soft tissues. A secondary objective was to determine the relative ability of various assays to detect changes in soft tissues due to temperature deviations. Porcine pulmonary heart valve leaflets exposed to 37 °C were compared with those incubated at 52 and 67 °C for 10, 30 and 100 min. The analytical methods consisted of (1) viability assessment using the resazurin assay, (2) collagen content using the Sircol assay, and (3) permeability assessment using an electrical conductivity assay. Additionally, histology and two photon microscopy were used to reveal mechanisms of cell and tissue damage. Viability, collagen content, and permeability all decreased following heat treatment. In terms of statistical significance with respect to treatment temperature, cell viability was most affected (p < 0.0001), followed by permeability (p < 0.0001), and then collagen content (p = 0.13). After heat treatment, histology indicated increased apoptosis and two photon microscopy revealed a decrease in collagen fiber organization and an increase in elastin density. These results suggest that measures of cell viability would be best for assessing tissues where the cells are alive and that permeability may be best where cell viability is not intentionally maintained.

Objective: The handmade expanded polytetrafluoroethylene (ePTFE) trileaflet-valved conduit could potentially be used as a substitute pulmonary valve replacement material, especially in children. The current study investigated (1) the function of the ePTFE trileaflet-valved conduits in an ex vivo experimental system and (2) the short-term performance of the conduit in a porcine model to verify its clinical applicability., Methods: The competency of the ePTFE trileaflet-valved conduits was estimated through ex vivo (using a pulmonary mock circulation loop) and in vivo (in a porcine model with a damaged pulmonary valve) experiments. Explants were examined by gross morphology and histopathologic examination., Results: In the ex vivo experiment, the ePTFE trileaflet-valved conduits were determined to effectively increase mean pulmonary pressure from 10.2 to 14.4 mm Hg compared with defective silicon-valved conduits. In addition, the regurgitation fraction value of ePTFE trileaflet-valved conduits was 15.9% to 18.1%, which was significantly better than the defective valve conduits (regurgitation fraction = 73.5%-85.7%). In the in vivo experiment, the valved conduits were confirmed to be with good valve position maintenance, and the valve and leaflets showed no signs of thickening or peeling after a short-term implantation period. There were also no significant signs of inflammation reaction on histopathologic examination., Conclusions: The ePTFE trileaflet-valved conduits for pulmonary valve reconstruction showed acceptable performance and outcomes in the ex vivo and in vivo experiments. The ePTFE trileaflet-valved conduit may be clinically useful, although additional studies in animals should be conducted to determine its long-term outcomes., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Purpose: The aim of this research was to assess the performance of a modified bovine stent valve implanted transventricularly in the pulmonary position in sheep with a 3-month follow-up period., Materials and Methods: Seven modified pulmonary bovine stent valves were transventricularly implanted in the pulmonary position into seven sheep using a delivery system. Stent valve performance was investigated and evaluated hemodynamically, angiographically, and with echocardiograms before, immediately after, and 3 months following implantation. Macroscopic, histologic, and radiographic examinations were performed on the explanted graft at 3 months., Results: The modified stent valves were all deployed and implanted successfully in the pulmonary position in seven sheep. Angiographic, echocardiographic, hemodynamic, and macroscopic analyses confirmed firm anchoring of the stents in the target position in the early and 3-month follow-up period. All modified stent valves showed satisfactory function, except one moderate stenosis (32 mmHg gradient) with mild regurgitation that was discovered at 3 months. All seven valves were free of any calcification and thrombus formation at postmortem macroscopic examination, which was confirmed by histologic and radiographic examination. All stents were intact without any fracture at microscopic or radiographic examination., Conclusions: Transventricular implantation of a modified nitinol pulmonary valve stent showed good structural and functional outcomes without stent fracture or migration., (© 2017 Wiley Periodicals, Inc.)

Current research on valvular heart repair has focused on tissue-engineered heart valves (TEHV) because of its potential to grow similarly to native heart valves. Decellularized xenografts are a promising solution; however, host recellularization remains challenging. In this study, decellularized porcine aortic valves were implanted into the right ventricular outflow tract (RVOT) of sheep to investigate recellularization potential. Porcine aortic valves, decellularized with sodium dodecyl sulfate (SDS), were sterilized by supercritical carbon dioxide (scCO2) and implanted into the RVOT of five juvenile polypay sheep for 5 months (n = 5). During implantation, functionality of the valves was assessed by serial echocardiography, blood tests, and right heart pulmonary artery catheterization measurements. The explanted valves were characterized through gross examination, mechanical characterization, and immunohistochemical analysis including cell viability, phenotype, proliferation, and extracellular matrix generation. Gross examination of the valve cusps demonstrated the absence of thrombosis. Bacterial and fungal stains were negative for pathogenic microbes. Immunohistochemical analysis showed the presence of myofibroblast-like cell infiltration with formation of new collagen fibrils and the existence of an endothelial layer at the surface of the explant. Analysis of cell phenotype and morphology showed no lymphoplasmacytic infiltration. Tensile mechanical testing of valve cusps revealed an increase in stiffness while strength was maintained during implantation. The increased tensile stiffness confirms the recellularization of the cusps by collagen synthesizing cells. The current study demonstrated the feasibility of the trans-species implantation of a non-fixed decellularized porcine aortic valve into the RVOT of sheep. The implantation resulted in recellularization of the valve with sufficient hemodynamic function for the 5-month study. Thus, the study supports a potential role for use of a TEHV for the treatment of valve disease in humans.

Outflow tract development of the heart is complex. The presence, differential growth and interactions of the various tissues through space and time contribute to the final development of the tract. This paper presents a novel interpretation of observations of outflow tract development, in particular of the aortic and pulmonary semilunar valves in embryos from the Shaner Collection at the University of Alberta. Three-dimensional reconstructions assist in the visualization of the spatial relationships of the developing valve tissues. In some embryos the aortic intercalated valve swelling is displaced proximally, giving rise to a bicuspid aortic semilunar valve more distally. In addition, the developing valve tissue first appears external to the myocardial cuff. The pulmonary semilunar valve regions appear to be more normal. This paper thus proposes a novel mechanism for generating a bicuspid aortic valve and also supports the idea that there is some independence of the aortic and pulmonary regions from each other during development., (Copyright © 2017 The Author(s). Published by Elsevier GmbH.. All rights reserved.)

In patients with carcinoid syndrome, the development of carcinoid valve disease typically carries an unfavorable prognosis. We present the case of a patient with significant valvular dysfunction secondary to carcinoid valve disease. Valve replacement surgery was complicated by the development of prosthetic valve degeneration, ultimately requiring percutaneous valve implantation in a valve-in-valve fashion. The technical details of the case, as well as a review of carcinoid valve disease, including its pathophysiology, clinical manifestations, diagnostic features and management considerations, are presented.

Right heart endocarditis mainly affects tricuspid valve, especially in drug users. Isolated pulmonary valve infection is rare. We here report the case of a 32-year old young woman whose diagnosis of community-acquired pulmonary valve endocarditis was confirmed. This study is even more interesting because pulmonary valve endocarditis has occurred in a patient without a history of drug addiction; nevertheless, it wasn't associated with restrictive interventricular communication. The patient underwent emergency surgery due to the visualization of large and mobile vegetation on transthoracic heart ultrasound. Surgery was carried out under extracorporeal circulation and pulmonary valve has been replaced with a bioprosthesis. Bloodcultures and valve culture showed methicillin-sensitive staphylococcus aureus. Postoperative evolution was favorable, with a follow-up of 06 months without recurring infections., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêts.

Background: Percutaneous pulmonary valve implantation (PPVI) using the bovine jugular vein Melody(®) valve (Medtronic Inc., Minneapolis, MN, USA) is safe and effective. However, post-procedural complications have been reported, the reasons for which are unclear., Objective: To assess the impact of PPVI procedural steps on valvular histology and leaflet mechanical behaviour., Methods: Three different valved stents (the Melody(®) valve and two homemade stents with bovine and porcine pericardium) were tested in vitro under four conditions: (1) control group; (2) crimping; (3) crimping plus inflation of low-pressure balloon; (4) condition III plus post-dilatation (high-pressure balloon). For each condition, valvular leaflets (and a venous wall sample for Melody(®) stents) were taken for histological analysis and mechanical uniaxial testing of the valve leaflets., Results: Among the Melody(®) valves, the incidence of transverse fractures was significantly higher in traumatized samples compared with the control group (P<0.05), whereas the incidence and depth of transverse fractures were not statistically different between the four conditions for bovine and porcine pericardial leaflets. No significant modification of the mechanical behaviour of in vitro traumatized Melody(®) valvular leaflets was observed. Bovine and porcine pericardia became more elastic and less resilient after balloon expansion and post-dilatation (conditions III and IV), with a significant decrease in elastic modulus and stress at rupture., Conclusion: Valved stent implantation procedural steps induced histological lesions on Melody(®) valve leaflets. Conversely, bovine and porcine pericardial valved stents were not histologically altered by in vitro manipulations, although their mechanical properties were significantly modified. These data could explain some of the long-term complications observed with these substitutes., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Background: Pulmonary valve (PV) insufficiency is often an acquired condition after treatment for pulmonary stenosis. It is recognized that PV insufficiency has serious deleterious effects. Although surgical replacement of the PV is efficacious, artificial valves inevitably fail and require re-intervention. The purpose of this study was to summarize our experience with PV repair in patients with acquired PV insufficiency., Methods: This was a retrospective review of 16 patients with marked PV insufficiency who underwent PV repair. Thirteen of these patients were born with tetralogy of Fallot (TOF) and had undergone a previous transannular patch repair. Three patients were born with critical pulmonary stenosis and had a surgical valvotomy or balloon valvuloplasty., Results: The 13 patients with TOF had resection of their previously placed transannular patch with re-approximation of the anterior commissure. All 13 patients experienced a marked reduction in the degree of pulmonary insufficiency. None of these patients have experienced any increase in insufficiency during follow-up. The 3 patients with critical pulmonary stenosis had a variety of pathologic findings identified at the surgical procedure. One patient had a large gap between a commissure and underwent closure of that commissure. The second and third patients had torn leaflets repaired with pericardial and Gore-Tex patches (Gore, Inc, Flagstaff, AZ). The degree of PV insufficiency was decreased to mild in all 3 patients. However, 2 of these 3 patients have subsequently had an increase in the degree of pulmonary insufficiency., Conclusions: Patients with TOF who underwent a previous transannular patch may be candidates for bicuspidization of their native PV, and the results of this procedure have been quite stable at follow-up. PV repair for torn leaflets was effective in the short term but was less stable over time., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: In a previous study, we identified factors affecting the durability of bioprosthetic valves in the pulmonary position following total repair of Tetralogy of Fallot (TOF). In this study, we aimed to identify factors affecting the durability of the bioprosthetic valve with regard to patient age and implanted valve size in order to guide valve choice in adolescent patients., Methods: We enrolled and analysed 108 cases of pulmonary valve replacement (PVR) with stented bioprosthetic valves in TOF patients between January 1998 and February 2014. Valvular dysfunction was defined as at least a moderate amount of pulmonary regurgitation or a peak pressure gradient of ≥40 mmHg on the most recent echocardiography. We analysed the effect of patient age and valve size on the durability of the bioprosthetic valve in the pulmonary position., Results: There were 2 early deaths; no late deaths were observed. The follow-up duration was 92.8 ± 44.5 months. The mean age at PVR was 19.3 ± 9.1 years. The mean valve size was 24.7 ± 1.8 mm. Whereas patients ≥20 years old showed no valvular dysfunction (i.e. 100% freedom from valvular dysfunction at 10 and 14 years), patients who were adolescents and children (<20 years) showed worse durability, regardless of the z-score of valve size (68.2% at 10 years and 24.7% at 14 years). Although a larger valve with a z-score of ≥2 was implanted, patients <20 years old did not exhibit good valvular durability. The results were particularly worse in patients <10 years old, with 66.7% freedom from valvular dysfunction at 6 years and 33.3% at 8 years, compared with patients within the age range of 10 to <20 years (75.1% at 10 years, and 20.5% at 14 years)., Conclusions: The durability of bioprosthetic valves in the pulmonary position was acceptable in patients aged ≥20 years, regardless of the z-score of valve size. However, patients who were children and adolescents did not show optimal durability of the bioprosthetic valve, irrespective of the z-score of valve size., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objective: To explore the features of outflow tract ventricular arrhythmias (OTVA) in children and evaluate the efficacy and experiences of radiofrequency catheter ablation (RFCA)., Method: Data were collected and analyzed on 53 consecutive pediatric patients who underwent RFCA for OTVA from January 2012 to July 2015 in the First Hospital of Tsinghua University. These children included 34 male and 19 female, whose average age was (8±3) years and body weight was (33±13) kg. All the children met the indication criteria for RFCA of OTVA in children. Data were analyzed about the onset age, the forms and ablation efficacy and strategies of OTVA., Result: The average age of onset was (8±4) years. Of these children, 68% (36/53) had frequent premature ventricular complex which was the main arrhythmia pattern.The proportion of 66%(35/53) were of right ventricular outflow tract (RVOT). The vast majority of RVOT ventricular arrhythmias originates from myocardium with in the first 1 cm beneath the pulmonary valve(89%, 31/35) or the septal region(77%, 27/35). The total acute success rate was 98%(52/53), the recurrence rate on follow-up was 10% (5/52) and the complications of the procedure was 2%(1/53). The X-ray exposure dose was(743±323) mGay·cm(2). However, for 6 of the children RFCA on both sides of outflow tract was necessary for success., Conclusion: OTVA in children originates mostly from RVOT. RFCA can be used for ablation of pediatric OTVA effectively and safely. In some cases, successful RFCA should be performed on both sides of outflow tract.

There is limited information about age-specific structural and functional properties of human heart valves, while this information is key to the development and evaluation of living valve replacements for pediatric and adolescent patients. Here, we present an extended data set of structure-function properties of cryopreserved human pulmonary and aortic heart valves, providing age-specific information for living valve replacements. Tissue composition, morphology, mechanical properties, and maturation of leaflets from 16 pairs of structurally unaffected aortic and pulmonary valves of human donors (fetal-53 years) were analyzed. Interestingly, no major differences were observed between the aortic and pulmonary valves. Valve annulus and leaflet dimensions increase throughout life. The typical three-layered leaflet structure is present before birth, but becomes more distinct with age. After birth, cell numbers decrease rapidly, while remaining cells obtain a quiescent phenotype and reside in the ventricularis and spongiosa. With age and maturation-but more pronounced in aortic valves-the matrix shows an increasing amount of collagen and collagen cross-links and a reduction in glycosaminoglycans. These matrix changes correlate with increasing leaflet stiffness with age. Our data provide a new and comprehensive overview of the changes of structure-function properties of fetal to adult human semilunar heart valves that can be used to evaluate and optimize future therapies, such as tissue engineering of heart valves. Changing hemodynamic conditions with age can explain initial changes in matrix composition and consequent mechanical properties, but cannot explain the ongoing changes in valve dimensions and matrix composition at older age.

Objectives: To investigate follow-up after percutaneous pulmonary valve implantation (PPVI)., Methods: Forty patients with pulmonary conduit dysfunction (males/females 24/16; 21 ± 08 years; 12 tetralogy of Fallot, 11 aortic valve disease, 17 other congenital heart disease) were planned for CMR before PPVI and repeated 7 times up to 48 months. CMR prospective results regarded: pressure gradient (PG) and regurgitation fraction (RF); end-diastolic volume, end-systolic volume, and stroke volume indexed to body surface area (EDVI, ESVI, and SVI); ejection fraction (EF) of right and left ventricles (RV, LV). A Friedman test was used for comparisons., Results: Overall, PG (31 ± 06 to 16 ± 4 mmHg), RF (16 ± 17 to 0.3 ± 1 %), RVEDVI (82 ± 38 to 58 ± 12 ml/m(2)), and RVESVI (44 ± 12 to 30 ± 13 ml/m(2)) declined (p < 0.001), RVEF (49 ± 13 to 58 ± 12 %) and RVSVI (from 38 ± 14 to 40 ± 8 ml/m(2)) increased (p < 0.001), LVEDVI (67 ± 17 to 73 ± 18 ml/m(2)) and LVSVI (37 ± 11 to 43 ± 10 ml/m(2)) increased (p = 0.034 and p < 0.001). Two patients had valve fracture at 24 and 36 months and underwent surgery. One patient had stent restenosis at 24 months and underwent percutaneous retreatment. Baseline/follow-up CMR did not predict PPVI failure., Conclusions: CMR demonstrated restored pulmonary conduit function, reduced RV volumes and increased RV and LV function but did not predict valve fracture/restenosis., Key Points: • A CMR 4-year follow-up after PPVI showed restored pulmonary conduit function • RV volumes were significantly reduced • RV function was significantly better in terms of increased EF and SVI • LV function was significantly better in terms of increased EDVI and SVI • Baseline/follow-up CMR did not predict three cases of PPVI failure.

A 65-year-old female patient with metastatic neuroendocrine tumour of the small intestine developed carcinoid valve disease and intractable right heart failure. She underwent successful cardiac valve replacement of both tricuspid and pulmonary valves. One year after surgery, she has regained full functional capacity.

Objective: To describe antenatal sonographic signs that help in the differentiation of truncus arteriosus Types II and III (TA-II/III) from pulmonary atresia with ventricular septal defect (PA-VSD)., Methods: From a database of fetal echocardiographic examinations, we identified fetuses with sonographic features of a single great artery with VSD and relatively normal four-chamber view. Records were reviewed, comparing fetuses with TA-II/III and those with PA-VSD, with particular focus on: 1) characteristics of the overriding vessel, 2) appearance of the semilunar valves, 3) competence of the semilunar valves, 4) presence of major aortopulmonary collateral arteries (MAPCA), 5) main pulmonary artery being without antegrade flow, 6) site of arterial branching from the great artery and 7) other minor features, such as cardiac axis or associated anomalies., Results: Seventeen fetuses were identified, eight with TA-II/III and nine with PA-VSD. Among the eight fetuses with TA-II/III, seven had abnormal valves and six had valve regurgitation, compared with none of the nine PA-VSD fetuses. Five TA-II/III fetuses had early branching to supply the lungs, whereas most fetuses with PA-VSD had more distal branching. Notably, in six of the TA-II/III fetuses, the root of the single great artery originated predominantly from the right ventricle, while all but one of the PA-VSD fetuses had typical equal overriding of the VSD. The main pulmonary artery was without antegrade flow in two cases with PA-VSD. Finally, four cases with PA-VSD had MAPCA, in two of which this was identified prenatally., Conclusion: Identification of abnormal arterial valves or valve regurgitation, site of origin of branching, presence of overriding of the great artery, a main pulmonary artery without antegrade flow and MAPCA are helpful in differentiating between TA-II/III and PA-VSD., (Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.)

We report a case of isolated pulmonary valve endocarditis evaluated with real-time three-dimensional echocardiography in a previous intravenous drug user. Although right-sided infective endocarditis is common in this population, the involvement of the pulmonary valve without infection of the tricuspid valve is a rare condition.