Characteristics of aortic and pulmonary valve cusp fenestrations in healthy hearts.

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.

Clinical characteristics, management, and outcomes of pulmonary valve myxoma: systematic review of published case reports.

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.

Surgical treatment of papillary fibroelastoma of the pulmonary valve: a case report.

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.

Isolated pulmonary valve endocarditis with rapid progression: a case report and literature review.

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.

Percutaneous pulmonary valve implant: Two Colombian case reports.

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.

Implante percutáneo de válvula pulmonar: a propósito de dos casos colombianos / Percutaneous pulmonary valve implant: Two Colombian case reports

Resumen 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.

Abstract 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.

Endobronchial valve therapy for severe emphysema: an overview of valve-related complications and its management.

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.

Pulmonary Artery Aneurysm Associated with Bicuspid Pulmonary Valve.

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.

A comprehensive review on the diagnosis and management of intimal sarcoma of the pulmonary artery.

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.

Papillary fibroelastoma on pulmonary valve - Valve-sparing surgery of a cardiac tumor in a rare location.

We present images of a papillary fibroelastoma on a pulmonary valve - echocardiography, intraoperative images, macroscopic and microscopic images of the tumor in this uncommon location.

Value of pulmonary annulus area index in predicting transannular patch placement in tetralogy of Fallot repair.

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 vmax ) 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.

In fetuses with congenital lung masses, decreased ventricular and atrioventricular valve dimensions are associated with lesion size and clinical outcome.

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 (RS = -.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.

Early Insight Into In Vivo Recellularization of Cell-Free Allogenic Heart Valves.

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.

Subclinical thrombus formation in bioprosthetic pulmonary valve conduits.

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.

Mechanical and structural analysis of the pulmonary valve in congenital heart defects: A presentation of two case studies.

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.

One-Year Follow-up After Tetralogy of Fallot Total Repair Preserving Pulmonary Valve and Avoiding Right Ventriculotomy.

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.

[Papillary Fibroelastoma of the Pulmonary and Tricuspid Valve in Asymptomatic Patients]. / Papilläres Fibroelastom an Pulmonal- und Trikuspidalklappe bei asymptomatischen Patienten.

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.