Right Ventricular Outflow Tract Aneurysm Research Articles

The Clinical Trial Outcomes of Med-Zenith PT-Valve in the Treatment of Patients With Severe Pulmonary Regurgitation.

ObjectiveNearly 2/3 of patients with dilated right ventricular outflow tract (RVOT) were excluded from pulmonary valves transplantation due to the lack of size-matched valves. Here, we explored the safety and efficacy of the Med-Zenith PT-Valve for the treatment of patients with severe pulmonary regurgitation.Methods22 Patients with severe PR (grade 3+,4+) were enrolled based on the anatomical features of native RVOT and the valve design. The immediate, 3-months and 1-year post-procedural follow-up data were analyzed.ResultsThe baseline mean systolic diameters in the distal main pulmonary artery (MPA), MPA sinus junction, MPA sinus, pulmonary annulus, RVOT aneurysm and muscular outlet measured with computed tomography were 33.6 ± 6.1, 34.0 ± 5.8, 37.9 ± 6.0, 32.4 ± 7.3, 41.9 ± 9.3, and 34.4 ± 8.0 mm, respectively. The PT-Valve landing zone was set within these levels. Successful valve implantations were achieved in all patients without noticeable device malposition, coronary artery compression, pulmonary branch obstruction or paravalvular leak during follow-ups. Post-procedural pulmonary artery diastolic pressure increased from 5.8 ± 3.1 to 11.3 ± 2.5 mmHg. In the 3-month and 1-year follow-up, the right ventricular end diastolic volume index reduced from the baseline 181.6 ± 29.0 to 143.7 ± 29.7 ml/m2 and 123.4 ± 31.2 ml/m2, and the trans-pulmonary valve gradient decreased from 25.6 ± 22.2 to 10.64 ± 3.54 mmHg and 11.16 ± 3.0 mmHg, respectively. The 6-min walk distance increased from 416.6 ± 97.9 to 455.9 ± 64.6 m and 467.8 ± 61.2 m, respectively.ConclusionThis clinical trial revealed favorable outcomes for the safety, efficacy and feasibility of the Med-Zenith PT-Valve in the treatment of severe PR with significantly enlarged RVOT.

The fate of the fresh autologous pericardium after right ventricular outflow tract reconstruction.

Introduction: The autologous pericardium, treated or fresh, is used in reconstructive cardiovascular surgery. We aimed to describe the features of fresh pericardium utilized in right ventricular outflow tract (RVOT) reconstruction, years after the initial surgery. Methods: This cross-sectional study was performed on 72 patients (65.3% male, mean age =18.68 ± 9.63 y) with a history of RVOT reconstruction with the fresh autologous pericardium who underwent reoperation. During the surgery, a 1 × 1 cm sample was cut from the previous pericardial patch, and hematoxylin and eosin (H & E), Masson’s trichrome, and immunohistochemistry (IHC) staining was conducted. All the stained slides were evaluated,and the descriptive results were explained. Results: The mean follow-up duration was 13.48 ± 7.38 years. In preoperative evaluations,53 (73.6%) patients exhibited no RVOT dilatation, 17 (23.6%) showed mild RVOT dilatation,and 2 (2.8%) had RVOT aneurysms. The H & E staining revealed no calcification in 80.55%(58/72), mild calcification in 9.72% (7/72), and moderate calcification in 9.72% (7/72) of the total samples. None of the specimens demonstrated a marked calcification. All the samples were positive for CD31, CD34, smooth muscle alpha-actin, and von Willebrand factor in IHC. In Masson’s trichrome staining, on average, 64.74% (±18.61) of the tissue sections contained collagen fibers. Conclusion: The fresh autologous pericardium, utilized for RVOT reconstruction, showed viability, growth potential, positivity for endothelial cell markers, vascular differentiation,insignificant calcification, and no stenosis at long-term follow-up. We would, therefore, suggest it as a suitable choice for such reconstructive operations. Moreover, its usage during total correction of tetralogy of Fallot could be safe, feasible, and durable.

Right Ventricular Outflow Tract Tachycardia: a Common Rhythm due to an Uncommon Etiology.

Right ventricular outflow tract (RVOT) aneurysm is a rare cause of RVOT ventricular tachycardia (RVOT-VT). We present a very unusual case of RVOT-VT due to an RVOT aneurysm diagnosed by cardiovascular magnetic resonance imaging.

Role of Multidetector CT in Assessment of Repaired Tetralogy of Fallot

The population of adults with repaired tetralogy of Fallot (TOF) is growing, thanks to improvements in the surgical and medical management of this condition. Accordingly, late postrepair complications are important medical concerns in these individuals. Initial surgical repair of TOF typically occurs in infancy, consisting of patch repair of the ventricular septal defect (VSD) and relief of right ventricular outflow tract (RVOT) obstruction. Although patients may remain asymptomatic for several decades, the majority will have progressive pulmonic regurgitation that leads to right ventricular (RV) dilatation and functional deterioration. Other frequently seen complications include branch pulmonary artery stenosis, RVOT aneurysms, and recurrent VSDs. Cardiac computed tomography (CT) is widely available and, in some cases, is the imaging modality of choice for serial evaluation of TOF patients. CT is particularly useful when magnetic resonance (MR) imaging is contraindicated (eg, in patients with implantable cardiac devices). Unlike MR imaging, cardiac CT allows excellent visualization of endovascular stents and stent-mounted valves. Retrospective electrocardiographically gated cardiac CT can be used for accurate volumetric and functional analysis of the RV. Comprehensive serial evaluation will assist in determining the need for surgical pulmonary valve repair in the setting of progressive RV dysfunction. Three-dimensional volumetric images are useful for evaluation of stent integrity and aneurysm formation. The radiologist should be familiar with the anatomy of TOF, surgical interventions for repair, and postrepair complications encountered at follow-up imaging of these patients. By extracting the breadth of information obtained with cardiac multidetector CT, the radiologist can play an essential role in the management of adult patients with repaired TOF.

Fragmented QRS complexes predict right ventricular dysfunction and outflow tract aneurysms in patients with repaired tetralogy of Fallot

BackgroundFragmented QRS complexes (fQRS) correlate with myocardial scar, and may predict arrhythmias in patients with repaired tetralogy of Fallot (TOF). We investigated the relationship between fQRS in operated TOF patients with right ventricular (RV) dysfunction and RV outflow tract (RVOT) aneurysm. MethodsWe studied 56 operated TOF patients with moderate/severe pulmonary regurgitation, referred for cardiac magnetic resonance imaging (MRI) over a 4.5year period. The presence of fQRS (additional notches in the R/S wave in ≥2 contiguous leads on the ECG) was correlated with MRI findings. ResultsfQRS was observed in 44 (78.6%) patients. Patients with fQRS had significantly larger RV end diastolic volume index (RVEDVi; 162ml vs 141ml, p=0.028) and RV end systolic volume index (RVESVi; 88ml vs 70ml, p=0.031). Increasing number of leads with fragmentation was independently associated with increasingly lower RV ejection fraction (adjusted co-efficient −0.97, 95%CI −1.83 to −0.12, p=0.026), greater pulmonary regurgitation fraction (1.65, 0.28 to 3.01, p=0.019), larger RVEDVi (6.78, 2.00 to 11.56, p=0.006) and RVESVi (5.41, 1.66 to 9.15, p=0.005). Anterior fragmentation correlated most significantly with RV dysfunction (p<0.05). fQRS had no significant association with LV dysfunction. Presence of any fQRS (OR 17.5, 95%CI 2.1–147.8, p=0.009) and inferior fQRS (OR 9.0, 95%CI 2.7–30.1, p<0.001) were found to be significant predictors for RVOT aneurysm. ConclusionsThe presence of fQRS on the ECG is significantly associated with RV dysfunction and RVOT aneurysms in repaired TOF patients. Increasing burden of fragmentation, especially in the anterior leads, is associated with increasing RV dysfunction.

Branch Pulmonary Artery Jailing With a Bare Metal Stent to Anchor a Transcatheter Pulmonary Valve in Patients With Patched Large Right Ventricular Outflow Tract

Although transcatheter pulmonary valve replacement using the Melody valve (Medtronic Inc, Minneapolis, MN) has become widely adopted, it has only been formally studied in patients with right ventricle–to–pulmonary artery conduits <22 mm in diameter.1,2 Such patients compose only a small proportion of individuals with right ventricular outflow tract (RVOT) dysfunction, and there is interest and potential utility to broadening the indications for Melody valve implantation. We report nonstandard use of Melody valve in 3 noncandidate patients and discuss salient technical points. ### Patient 1 A 25-year-old man with early surgical correction of pulmonary atresia intact septum during infancy, presented with severe PR and no gradient across the RVOT and branch pulmonary arteries (PAs). Calibration using a compliant balloon showed RVOT diameter of 25 mm, making the patient unsuitable for the Melody valve, using the conventional technique (Figure 1 and online-only Data Supplement video clips I and II). Figure 1. Cineangiogram still frames demonstrate the jailing technique in patient 1. A , Angiogram on 4-chamber view shows the anatomy of the right ventricular outflow tract (RVOT) and pulmonary artery (PA) tree. B , RV angiogram after placement of open-cell, bare metal stents shows symmetrical perfusion of the 2 PAs and incomplete apposition of the stents to the RVOT. C , Angiogram shows opening of the cells at the level of the jailed PA before Melody insertion. D , Final angiogram shows reduction of pulmonary regurgitation with a small amount of paraprosthetic leak. ### Patient 2 An 18-year-old man with pulmonary atresia and ventricular septal defect underwent palliative Blalock-Taussig shunt in the immediate postnatal period and subsequently, complete repair without pulmonary valved or nonvalved conduit. A false RVOT aneurysm was closed, using an atrial septal defect occluder. The patient presented with native left PA stenosis, severe pulmonary regurgitation (PR), and worsening functional status. There was no gradient …

Right ventricular outflow tract aneurysm with thrombus

Right ventricular outflow tract (RVOT) aneurysm is a known complication of tetralogy of Fallot repair when a ventriculotomy is done. It leads to RV dysfunction and may require re-operation. We describe a rare instance of a patient who developed an RVOT aneurysm after trans-ventricular repair of tetralogy of Fallot, which was complicated with the formation of a thrombus in the aneurysm sac. The patient underwent re-operation with thrombectomy, excision of the RVOT aneurysm and pulmonary valve replacement. To the best of our knowledge, the occurrence of this combination and its implications have not been reported.

Precise automated determination of the total and segmented right ventricular volumes for functional studies of the right ventricle using CMR

Background Cardiac magnetic resonance (CMR) is an acknowledged standard for quantification of left ventricular volumes, but the acceptance of CMR to measure the volume of the right ventricle (RV), including sub-units, such as inlet, outlet, and apical segment has been limited. In patients with Fallot (TOF), the size and function of the right ventricular outflow tract (RVOT) in the course of an RR-interval can serve as indication for re-operation in cases of pulmonary stenosis, regurgitation, or RVOT aneurysms. in addition to the results of conductance examinations. However, none of the available software applications provides a suitable systematic subdivison of the total volume. A novel algorithm was developed for this purpose and tested in this study. Methods We present an automated method for the determination of RV volumes based on short axis cine series from CMR examinations. The contour detection proceeds iteratively through slices and phases. Manual interaction is possible. For further functional analysis of the right ventricle, a subdivision into compartiments is applied. The subdivision is performed automatically from flat or curved layers as defined by landmarks identified by the investigator. The landmarks include the crista supraventricularis, the set in of trabeculary structures, and the apex. The conventional definitions of the compartiments are loose, therefore two definitions of the outlet are compared to each other: one robust and simple definition using flat separating layers, and a more complicated but flexible definition based on a curved layer. Results Depending on the quality of the images, this contour detection is successful (i. e. requires no further manual interaction) in up to about 95% of images in the midventricular region. Papillary structures in the apex as well as a weakly separable atrium in the basal region may require manual interactions. Volumes are provided for all phases in total and in specific regions. Stroke volume and ejection fraction arise naturally. The volumes obtained from the two outlet definitions turn out to agree typically within 10% of the total volume and thus do not lead to contradicting conclusions. Conclusion The application of this fast iterative contour detection method and volumetry may allow the evaluation of the chamber volumes for the full heart cycle in clinical routine. The automatic volume segmentation provides useful information about the function of a particular segment such as the RV outlet in TOF patients where it can complete the indications for a surgery.

Outflow tract segmental arrhythmogenic right ventricular cardiomyopathy in an English Bulldog

A 5-year-old English Bulldog was presented for acute onset of syncope and fatigue caused by sustained ventricular tachycardia with left bundle block morphology and inferior axis. This arrhythmia had the electrocardiographic features of a ventricular tachycardia arising from the right ventricular outflow tract (RVOT), as described in an experimental canine model and in people. Since a RVOT aneurysm was identified by echocardiography, a segmental form of arrhythmogenic right ventricular cardiomyopathy (ARVC) was suspected. Gross examination of the heart confirmed the bulging of the RVOT and histological examination of the ventricular myocardium revealed segmental involvement of the RVOT with transmural fibro-fatty degeneration. To the authors' knowledge, this is the first reported case of AVRC in an English Bulldog and the first example of segmental AVRC described in the dog.

Infectious false aneurysm of the right ventricular outflow tract after repair of congenital heart defect treated with Freestyle aortic bioprosthesis

False aneurysm of the right ventricular outflow tract (RVOT) is one of complications after surgery of congenital heart disease. It is controversial about materials for reconstruction of RVOT in this setting, when associated with pulmonary hypertension or bacterial infection. Pulmonary homograft seems to be the first choice, but its availability is extremely limited in Japan. Therefore, we used Freestyle stentless bioprosthesis to reconstruct the RVOT in a 14-year-old boy who developed infectious false aneurysm of RVOT after total correction of pulmonary atresia with ventricular septal defect. After describing the patient, the prosthesis alternative to the pulmonary homograft was discussed.

Late reoperations after repair of tetralogy of Fallot.

Twenty-two patients underwent 23 late reoperations after total correction of tetralogy of Fallot from 1965 to 1990. Indications for reoperation included: isolated ventricular septal defect (VSD) in 9 patients (41%), isolated right ventricular outflow tract (RVOT) obstruction in 3 patients (13.7%), VSD associated with a RVOT obstruction in 7 patients (31.8%), aneurysm of the pericardial RVOT patch in 1 patient (4.5%), aortic insufficiency with a residual VSD in 1 patient (4.5%), and tricuspid regurgitation in 1 patient (4.5%). The reoperation consisted of closure of a residual VSD in 17 patients, relief of a RVOT gradient in 11, insertion of a RVOT valve in 4, tricuspid valve replacement in 1 (reoperated twice), aortic valve replacement in 1, and excision of a RVOT aneurysm in 1. Two patients died in hospital (9%) but there were no early deaths in the 11 patients reoperated upon after 1978. Mean follow-up period was 135 months. There were 2 late deaths. The actuarial 20-year survival was 87%. Of the surviving patients, 16 (89%) were in New York Heart Association class I, 1 (5.5%) was in class II, and one (5.5%) was in class III. One patient required a second reoperation for tricuspid bioprosthesis degeneration and 1 patient had moderate recurrent RVOT gradient due to calcified pulmonary bioprosthesis. This study tends to support the policy of recommending reoperation in the presence of surgically significant residual defects. Reoperation is associated with a low early mortality and good long-term results.

RIGHT VENTRICULAR OUTFLOW TRACT ANEURYSM FOLLOWING TETRALOGY OF FALLOT REPAIR

Aneurysms (AN) of right ventricular outflow tract (RVOT) following tetralogy of Fallot (TOF) repair were analyzed in 53 pts. Pulmonary-to-aortic root ratio, subpulmonic chamber size and supravalvar anatomy were assessed on pre-op angiograms, and operative reports were reviewed for the extent of RVOT reconstruction. AN size, judged from angiograms obtained 2-57 mos following surgery, was graded 1-4+. Large AN (3-4+), comprising more than 1/3 of total RV area on end-systolic lateral images, were found in 20 pts (38%), 3 of whom also had marked cardiomega-ly (4+). Moderate AN (2+) were found in 14 pts (26%) and small RVOT AN (1+) in 19 pts (36%). We excluded 5 pts with a large residual VSD (Qp:Qs>1.8): 4/20 3-4+ pts and 1/33 pts with 1-2+ AN. Pre-op studies on the 3-4+ pts showed smaller pulmonary-to-aortic root diameters (.48±.05 vs .59±.06, p<.05), more tight supravalvar stenoses (38% vs 9%) and a lower incidence of appreciable subpulmonic chambers (31% vs 58%) than in other pts. Age at repair (0.8-12.8 yrs; mean 4.3±1 yr) was not different for those with large vs small AN (4.4±1.5 vs 3.3±1.2). In pts with 3-4+ AN, 15/16 (93%) required pulmonary valvotomy and RVOT patch, compared to 21/33 (63%) of the 1-2+ group. RV/LV pressure ratios were higher in the 3-4+ pts at post-op cath (.65±.12 vs .42±.05, p<.01). Multiple levels of pre-op severe stenosis leading to incomplete relief of outflow obstruction were the chief factors leading to the development of large RVOT aneurysms after TOF repair.

The preoperative and postoperative findings in 627 patients with tetralogy of Fallot.

The preoperative and postoperative findings in 627 patients operated for correction of tetralogy of Fallot (TOF) in the period 1960 to 1984 were analyzed in order to evaluate the clinical and hemodynamic late results. The average age was 7.2 years (range 9 months to 30 years). The patients were divided into 4 groups in order to determine the long-term prognosis in relation to the severity of the underlying anatomy; group I = without outflow tract patch; group II = with a patch up to the valve base; group III = with a transannular patch (TAP); group IV = TAP or valved conduit in patients with pulmonary atresia. Preceding shunt operations had been performed in 350 patients (55.8%); a primary correction was carried out in 277 cases (44.1%). Hospital mortality for all patients operated from 1960 to 1984 was 14.0%, late mortality, by contrast, was only 1.1%. In the last 4 years (1980 to 1983), the overall mortality considerably decreased to 3.4% (n = 29) for patients without TAP and to 8.0% for all operated patients including those with pulmonary atresia (n = 50). A comparison of the actuarial survival curves, moreover, demonstrates that the prognostic survival rate is unequivocally dependent on the severity of the anatomy of a TOF. The hemodynamic results obtained from 271 recatheterized patients from all 4 groups were designated excellent in 106 patients (39.1%), good in 100 (39.9%) and poor in 65 (23.9%). Proximal residual gradients across the right ventricular outflow tract (RVOT) were found in 30.2% of the corrected patients, and distal gradients in 24.3%. Fifty-two patients out of the 627 (8.2%) had to be reoperated for residual VSD, RVOT aneurysm, valvular pulmonary stenosis or peripheral stenosis. Intracardial electrophysiological tests carried out in 166 patients postoperatively showed an antegrade effective refractory period of the AV-node lasting over 400 ms in 47 patients (28.3%), as well as additional ventricular action potentials in 117 patients (70.4%), which could be correlated to extrasystoles or ventricular tachycardia in 37.9% of the cases. Additional AV-pathways were disclosed in 3 patients.(ABSTRACT TRUNCATED AT 400 WORDS)