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Background: Progression to combined post‐ and pre‐capillary pulmonary hypertension (PH) provides prognostic information in human patients with post‐capillary PH. Pulmonary vascular resistance estimated by echocardiography (PVRecho) is useful for the stratification of dogs with myxomatous mitral valve disease (MMVD) and detectable tricuspid regurgitation. Objectives: To evaluate the prognostic value of PVRecho in dogs with MMVD. Animals: Fifty‐four dogs with MMVD and detectable tricuspid regurgitation. Methods: Prospective cohort study. All dogs underwent echocardiography. The PVRecho was calculated based on tricuspid regurgitation and the velocity‐time integral of the pulmonary artery flow. To evaluate the influence of echocardiographic variables on cardiac‐related deaths, Cox proportional hazard analysis was performed. Additionally, Kaplan‐Meier curves classified by PVRecho tertiles were made and compared using log‐rank tests to evaluate the influence of PVRecho on all‐cause mortality and cardiac‐related death. Results: The median follow‐up time was 579 days. Forty‐one dogs with MMVD (PH severity [number]: no or mild, 21/33; moderate, 11/11; severe, 9/10) died during the study. In the multivariable Cox proportional hazard analysis adjusted for age, sildenafil administration, and American College of Veterinary Internal Medicine stage of MMVD, left atrial to aortic diameter ratio and PVRecho remained significant (adjusted hazard ratio [95% confidence interval]: 1.2 [1.1‐1.3] and 2.1 [1.6‐3.0], respectively). Higher PVRecho showed a significant association with lower survival rates. Conclusions and Clinical Importance: Left atrial enlargement and high PVRecho were independent prognostic factors in dogs with MMVD and detectable tricuspid regurgitation. [ABSTRACT FROM AUTHOR]

The pulmonary valve (PV) has historically been ignored by imaging studies. Disorders of the PV encountered in adult cardiac patients are increasingly encountered due to advanced care of patients with congenital heart disease and associated PV diseases. Despite advances in PV imaging, multiple challenges remain when it comes to obtaining high quality PV images. While 2D TTE is the usual initial imaging tool for PV, excellent views of the PV annulus and its one or two leaflets are obtained in less than half of patients. The 3D echocardiography en face view allows all three leaflets to be evaluated concurrently, as well as assessments of the RV outflow tract and main pulmonary artery, which has improved quantitative assessment of PV diseases. Increasing image quality and experience with live/real time 3D TEE amplifies its utility in accurate evaluation and helps guide and monitor successful percutaneous PV interventions. 2D TTE remains the first line diagnostic tool; however, 3D TTE and 3D TEE provide better image quality which increases diagnostic accuracy and guidance to therapy. In this review article, we stress improvement in 3D echocardiography and its role in diagnostic and therapeutic options for PV diseases. [ABSTRACT FROM AUTHOR]

Ventricular tachycardia and premature ventricular complexes (PVCs) arising from right ventricular outflow tract (RVOT) are the most common type of ventricular arrhythmias (VAs) in patients without structural heart disease. Radiofrequency ablation is now the gold standard of treatment in this setting due to high efficacy rates and optimal safety profile. During the last few years, the pulmonary valve (PV) and the pulmonary artery (PA) have attracted much attention as reliable sites of origin of RVOT‐type arrhythmias. In the mean while intracardiac echocardiogram (ICE) has undoubtedly improved our understanding of the cardiac anatomy. Aim of this paper is to provide an illustrated step‐by‐step guide on how to use ICE with the CARTOSOUND module to visualize and reconstruct 3D shell of the RV, the PV, as well of other contiguous anatomical structures (i.e., the aortic valve and coronary arteries) to perform aware and safe ablation in this region. [ABSTRACT FROM AUTHOR]

A fetal echocardiogram noted valvar pulmonary atresia vs critical pulmonary valve stenosis, confluent pulmonary arteries, patent ductus arteriosus, and an apical muscular ventricular septal defect vs coronary sinusoid. Postnatal echocardiogram documented an absent pulmonary valve, coronary artery fistula to the right ventricle, confluent pulmonary arteries, and a patent ductus arteriosus. Catheterization confirmed the postnatal echocardiographic findings except there was no antegrade flow from the right ventricle to the pulmonary arteries. Close evaluation of all imaging modalities revealed the final diagnosis of absent pulmonary valve with a double‐chambered right ventricle with no egress from the right ventricle to pulmonary artery. [ABSTRACT FROM AUTHOR]

Objective: This study sought to investigate the variation of right heart structure pre‐ and post‐operation as risk factors for moderate to severe pulmonary regurgitation (PR) after repaired Tetralogy of Fallot and the best "cutoff" values for the transannular patch (TAP). Methods: We collected surgical, echocardiographic, and computed tomographic data of Teralogy of Fallot (TOF) patients over two years and calculated z‐score values based on the echocardiographic data. Based on the PR level after follow‐up, the patients were divided into two groups, trivial to mild PR and moderate to severe PR. A multivariate logistic regression analysis was performed, and the receiver operating characteristic curve analysis was used to find the best "cutoff" value for risk factors. Results: A total of 104 TOF patients were included in our cohort study. From the multivariate analysis, correction strategy (P =.002), difference in zRVOT (OR 1.974, 95% CI 1.354 to 2.878, P <.0001), and zPVA (OR 3.605, 95% CI 1.980 to 6.562, P <.0001) were the significant risk factors for moderate to severe PR. The "cutoff" value for the difference in zPVA that could predict moderate to severe PR in the TAP group was 3, and the optimal "cutoff" value for TAP was −1.4. Conclusions: The TAP is a risk factor for significant PR after surgery. We recommend the optimal "cutoff" value for TAP is −1.4 calculated using Shan‐Shan Wang's data set. During the procedure, to limit the RVOT resection and restrict the enlargement of pulmonary annulus within a variation of z‐score as 3 would reduce significant PR. [ABSTRACT FROM AUTHOR]

Quadricuspid pulmonary valve is a rare entity that can be associated with pulmonary artery aneurysm. It is usually asymptomatic and diagnosed incidentally. Association with other congenital or acquired heart diseases has been reported. Echocardiography and gated CT angiography can provide useful functional and anatomic information that can help in the diagnosis and management. [ABSTRACT FROM AUTHOR]

Tetralogy of Fallot/Absent Pulmonary Valve (TOF/APV) has been classically associated with the absence of a patent ductus arteriosus (PDA). We present a rare case of APV in TOF with a discontinuous left pulmonary artery (LPA) that was suspected during fetal echocardiogram. Postnatal echocardiogram confirmed the origin of a hypoplastic LPA from the PDA. Despite an aneurysmal (right pulmonary artery) (RPA), axial imaging demonstrated widely patent tracheobronchial system with no evidence of bronchial compression. Clinically, the child required only minimal respiratory support. Genetic testing was positive for 22 q11deletion, commonly associated with this lesion. Surgery consisted of unifocalization of the discontinuous LPA with placement of a valved pulmonary homograft during complete repair of this lesion. Our case highlights the importance of prenatal detection, to aid in the prompt initiation of prostaglandins so as to ensure early rehabilitation of the left lung. Inability to visualize one of the branch pulmonary arteries (PA's) and a PDA on fetal echocardiogram in TOF/APV must raise suspicion for an eccentric branch PA with ductal origin. [ABSTRACT FROM AUTHOR]

Absent pulmonary valve syndrome is a rare congenital heart disease characterized by absent or rudimentary pulmonary valve leaflets and hypoplastic pulmonary annulus. The most common variant, associated with tetralogy of Fallot, implies dilatation of pulmonary branches and usually absent ductus arteriosus. Rarely, absent pulmonary valve occurs with intact ventricular septum: pulmonary branches are normally sized or mildly dilated and ductus arteriosus is usually patent. The rarest type is associated with intact ventricular septum and tricuspid atresia. A close connection has been raised between pulmonary regurgitation and development of tricuspid valve. We describe a case of prenatal diagnosed absent pulmonary valve with intact ventricular septum and patent ductus arteriosus in which severe pulmonary regurgitation caused reversible functional atresia of tricuspid valve. Postnatally, satisfactory biventricular circulation was obtained with inotropic support for a few days while ductus arteriosus closed spontaneously. At a 3‐year follow up the child is asymptomatic without therapy. [ABSTRACT FROM AUTHOR]

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] cm2/m2) compared with baseline (0.30 [0.09], 0.19 [0.09], 0.44 [0.17] cm2/m2, 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. [ABSTRACT FROM AUTHOR]

We present the case of an 18‐year‐old man with aborted sudden cardiac death. His initial echocardiogram suggested an anomalous origin of the left coronary artery from the pulmonary artery. Diagnosis was confirmed with coronary angiography. He underwent Takeuchi procedure and fully recovered. A two‐year follow‐up echocardiogram showed a moderate supravalvular pulmonary stenosis related to the transpulmonary baffle. The presence of extensive collateral circulation should raise suspicion of ALCAPA. Postoperative surveillance in this group of patients needs to be oriented in finding complications such as supravalvular pulmonary stenosis, aortic and pulmonary valve insufficiency, and baffle obstruction and leaks. [ABSTRACT FROM AUTHOR]

A 54‐year‐old male was found to have neuroendocrine carcinoma with hepatic metastasis. Two‐dimensional (2D) transthoracic echocardiography (TTE) demonstrated dilated right ventricle and right atrium, and severe tricuspid and pulmonary regurgitation. Three‐dimensional (3D) TTE en‐face views showed thickened, retracted, and fixed tricuspid valve and pulmonic valve which remained widely open throughout the cardiac cycle. 3D TTE, particularly en‐face views, demonstrates incremental value over 2D TTE by providing precise valvular anatomic details comparable to surgical findings. 3D TTE also offers a unique opportunity to assess all four valves simultaneously with en‐face views to delineate their relationships with surrounding structures. [ABSTRACT FROM AUTHOR]

ABSTRACT: Objective: To compare prenatal findings, associated genetic anomalies and postnatal outcome in fetuses with tetralogy of Fallot (TOF) with normal cardiac axis (CAx) and those with abnormal CAx. Methods: In this retrospective cohort study, 85 cases diagnosed with TOF by prenatal ultrasound at our clinic between 2005 and 2015 were reviewed. Follow‐up ultrasound and postnatal outcome were available for 68 cases. One case complicated with absent pulmonary valve syndrome and a further seven cases diagnosed postnatally with anomalies other than TOF were excluded from the study. The remaining 60 cases of postnatally confirmed TOF were divided according to CAx into two groups: those with normal CAx (n = 33) and those with abnormal CAx (n = 27). CAx was defined as the angle between the interventricular septum and midline of the fetal thorax at the level of the four‐chamber view. CAx > 65° or < 25° was considered abnormal. Prenatal sonographic findings, associated genetic anomalies and postnatal outcome were compared between the two groups. Results: Fetuses with TOF and abnormal CAx were more likely to have pulmonary atresia (40.7% vs 15.2%; P = 0.026) and right‐sided aortic arch (48.1% vs 21.2%; P = 0.028) than those with normal CAx. Postnatal death occurred in 30.4% of infants with abnormal CAx vs 6.5% with normal CAx (P = 0.028). Incidence of tested genetic anomalies was similar between the two groups. Conclusion: In fetuses with TOF, abnormal CAx is associated with the presence of pulmonary atresia, right‐sided aortic arch and a higher risk of postnatal death. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]

Objective: To assess the spectrum of associated anomalies, intrauterine course and outcome in fetuses with absent pulmonary valve syndrome (APVS).Methods: All cases with a prenatal diagnosis of APVS at two centers over a period of 13 years were analyzed retrospectively. APVS was diagnosed in the presence of rudimentary or dysplastic pulmonary valve leaflets with to-and-fro blood flow in the pulmonary trunk on color and pulsed-wave Doppler ultrasound. Data on demographic characteristics, presence of associated conditions, Doppler studies and pregnancy outcome were reviewed.Results: During the study period, 40 cases of APVS were diagnosed prenatally. Thirty-seven (92.5%) cases were associated with tetralogy of Fallot (TOF) and three (7.5%) had an intact ventricular septum. Patency of the ductus arteriosus (DA) was found in 17/37 (45.9%) TOF cases and in all three cases with an intact ventricular septum. Mean gestational age at diagnosis was 19.7 (range, 12-34) weeks with 10 (25.0%) cases (all with TOF) diagnosed in the first trimester. TOF was an isolated finding in 15 (37.5%) cases. Chromosomal anomalies, cardiac defects and extracardiac anomalies were present in 18 (45.0%), four (10.0%) and three (7.5%) cases, respectively. Among the 40 cases, there were 19 (47.5%) terminations of pregnancy, six (15.0%) intrauterine deaths, four (10.0%) neonatal deaths and 11 (27.5%) survivors. Patency of the DA, reversed flow during atrial contraction in the ductus venosus, umbilical artery or fetal middle cerebral artery, and hydrops/increased nuchal translucency thickness were significantly associated with non-survival. All 10 cases diagnosed in the first trimester had a patent DA and abnormal Doppler parameters, eight had hydrops and/or increased nuchal translucency, six were associated with trisomy 13 or 18 and none survived.Conclusion: APVS diagnosed in the first trimester is significantly associated with TOF, patency of the DA, abnormal Doppler parameters, lethal trisomies and intrauterine mortality. Cases of APVS with isolated TOF and agenesis of the DA have a better outcome than those with additional anomalies, with > 80% survival. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]

In the case presented, a young horse was referred for further evaluation based on a presumptive diagnosis of pulmonic valve endocarditis, bronchopneumonia and a cranial mediastinal abscess based on thoracic ultrasound, echocardiography and clinical signs. However, further echocardiographic evaluation revealed that the abscess was compressing the right ventricular outflow track causing outflow obstruction, a murmur associated with right ventricular outflow tract obstruction (acquired pulmonic stenosis) and right ventricular hypertrophy. Vegetative endocarditis was not present. Medical management was unsuccessful, but surgical drainage and long-term antimicrobial therapy were effective and the horse returned to a successful racing career. [ABSTRACT FROM AUTHOR]

Aims The aim of this study was to evaluate the feasibility of transthoracic two-dimensional (2D) iRotate, a new echo modality, to assess the whole right ventricle ( RV) from a single transducer position based on anatomic landmarks. Methods and Results The anatomic landmarks were first defined based on three-dimensional echocardiographic datasets using multiplane reconstruction analyses. Thereafter, we included 120 healthy subjects (51% male, age range 21-67 years). Using 2D iRotate, four views of the RV could be acquired based on these landmarks. The anterior, lateral, inferior wall (divided into three segments: basal-mid-apical), and right ventricular outflow tract ( RVOT) anterior wall of the RV were determined. The feasibility of visualization of RV segments and tricuspid annular plane systolic excursion ( TAPSE) and tissue Doppler imaging ( TDI) measurements were assessed. To evaluate this model for diseased RVs, a small pilot study of 20 patients was performed. In 98% of healthy subjects and 100% of patients, iRotate mode was feasible to assess the RV from one single transducer position. In total, 86% and 95%, respectively, of the RV segments could be visualized. The visualization of the RVOT anterior wall was worse 23% and 75%, respectively. TAPSE and TDI measurements on all four views were feasible 93% and 92%, respectively, of the healthy subjects and in 100% of the patients. Conclusion With 2D iRotate, a comprehensive evaluation of the entire normal and diseased RV is feasible from a fixed transducer position based on anatomic landmarks. This is less time-consuming than the multiview approach and enhances accuracy of RV evaluation. Imaging of the RVOT segment remains challenging. [ABSTRACT FROM AUTHOR]

Four-dimensional phase-contrast magnetic resonance imaging (4D PC-MRI) allows the non-invasive acquisition of time-resolved, 3D blood flow information. Stroke volumes (SVs) and regurgitation fractions (RFs) are two of the main measures to assess the cardiac function and severity of valvular pathologies. The flow rates in forward and backward direction through a plane above the aortic or pulmonary valve are required for their quantification. Unfortunately, the calculations are highly sensitive towards the plane's angulation since orthogonally passing flow is considered. This often leads to physiologically implausible results. In this work, a robust quantification method is introduced to overcome this problem. Collaborating radiologists and cardiologists were carefully observed while estimating SVs and RFs in various healthy volunteer and patient 4D PC-MRI data sets with conventional quantification methods, that is, using a single plane above the valve that is freely movable along the centerline. By default it is aligned perpendicular to the vessel's centerline, but free angulation (rotation) is possible. This facilitated the automation of their approach which, in turn, allows to derive statistical information about the plane angulation sensitivity. Moreover, the experts expect a continuous decrease of the blood flow volume along the vessel course. Conventional methods are often unable to produce this behaviour. Thus, we present a procedure to fit a monotonous function that ensures such physiologically plausible results. In addition, this technique was adapted for the usage in branching vessels such as the pulmonary artery. The performed informal evaluation shows the capability of our method to support diagnosis; a parameter evaluation confirms the robustness. Vortex flow was identified as one of the main causes for quantification uncertainties. [ABSTRACT FROM AUTHOR]

Background Patients with free pulmonary regurgitation or mixed pulmonary stenosis and regurgitation and severely dilated right ventricles (RV) show little improvement in ventricular function after pulmonary valve replacement when assessed by traditional echocardiographic markers. We evaluated changes in right and left ventricular (LV) function using speckle tracking echocardiography in patients after SAPIEN transcatheter pulmonary valve (TPV) placement. Methods Echocardiograms were evaluated at baseline, discharge, 1 and 6 months after TPV placement in 24 patients from 4 centers. Speckle tracking measures of function included peak longitudinal strain, strain rate, and early diastolic strain rate. RV fractional area change, tricuspid annular plane systolic excursion, and left ventricular LV ejection fraction were assessed. Routine Doppler and tissue Doppler velocities were measured. Results At baseline, all patients demonstrated moderate to severe pulmonary regurgitation; this improved following TPV placement. No significant changes were detected in conventional measures of RV or LV function at 6 months. RV longitudinal strain (−16.9% vs. −19.6%, P < 0.01), strain rate (−0.87 s−1 vs. −1.16 s−1, P = 0.01), and LV longitudinal strain (−16.2% vs. −18.2%, P = 0.01) improved between baseline and 6 month follow-up. RV early diastolic strain rate, LV longitudinal strain rate and early diastolic strain rate showed no change. Conclusion Improvements in RV longitudinal strain, strain rate, and LV longitudinal strain are seen at 6 months post-TPV. Diastolic function does not appear to change at 6 months. Speckle tracking echocardiography may be more sensitive than traditional measures in detecting changes in systolic function after TPV implantation. [ABSTRACT FROM AUTHOR]

The right ventricle ( RV) is of lesser importance in acquired heart disease, but its role is of increasing importance in congenital heart disease ( CHD). Despite major progress being made, precise measurements of the RV are challenging because of its peculiar anatomical structure that is not adaptable to any planar geometrical assumption. This is particularly true in adult patients with CHD where the RV shape eludes any standardization, it may assume various morphologies, and its modality of contraction depends on previous surgical treatment and/or pathophysiological conditions. However, reliable and repeatable quantification of RV dimensions and function for these patients are essential to provide appropriate timing for intervention to optimize outcomes. In this population, echocardiographic evaluation should not be limited to an observational and subjective functional assessment of the RV but must provide quantitative values repeatable and clinically reliable to help the decision-making process. The aim of this review was to discuss the echocardiographic approach to the RV in adult patients with CHD in general and in specific cases of pressure or volume overload. [ABSTRACT FROM AUTHOR]

Few cases of diastolic mitral regurgitation (MR) and tricuspid regurgitation (TR) have been reported in the world literature. We report the case of a 63-year-old woman admitted for syncope, with a permanent pacemaker following complete heart block. Echocardiography revealed that the timing of the diastolic TR (and noted MR) coincided with the second phase of the pulmonic insufficiency (PI) jet. The respirometer revealed that the diastolic TR and the second phase of the PI are highly sensitive to respiration (attenuated with inspiration and exacerbated with expiration). The uniqueness of this case is the rare occurrence of the exacerbation of PI as the result of diastolic TR. [ABSTRACT FROM AUTHOR]

Raised tricuspid regurgitant velocity ( TRV) occurs in approximately 30% of adults with sickle cell disease ( SCD), and has been shown to be an independent risk factor for death. TRV was assessed in 164 SCD patients who were subsequently followed up for survival. Raised pulmonary pressures were defined as a TRV jet ≥2·5 m/s on echocardiography. Elevated TRV was present in 29·1% of patients and it was associated with increased age and left atrial diameter. There were 15 deaths (9·1%) over a median of 68·1 months follow up; seven patients had increased TRV, and eight patients had a TRV<2·5 m/s. Higher TRV values were associated with a greater than 4-fold increased risk of death (Hazard Ratio: 4·48, 99% confidence interval 1·01-19·8), although we found a lower overall mortality rate than has been reported in previous studies. TRV was not an independent risk factor for death. We have confirmed the association between raised TRV and mortality in a UK SCD population whose disease severity appears to be less than that reported in previous studies. Further prospective studies are needed to more clearly characterize which patient factors modify survival in SCD patients with raised TRV. [ABSTRACT FROM AUTHOR]

Objectives To evaluate echocardiographic changes after SAPIEN valve implantation in the pulmonary position. Background The feasibility of the SAPIEN transcatheter pulmonary valve ( TPV) has recently been demonstrated. We evaluated changes in pulmonary valve function and the right ventricle after SAPIEN TPV placement. Methods We evaluated echocardiograms at baseline, discharge, 1 and 6 months after TPV placement in 33 patients from 4 centers. Pulmonary insufficiency severity was graded 0-4. TPV peak and mean gradients were measured. Right ventricular ( RV) size and function were quantified using routine measures derived from color, spectral, and tissue Doppler indices and two-dimensional echocardiography. Results At baseline, 94% patients demonstrated pulmonary insufficiency grade 2-4. This decreased to 12% patients at 6 months (P < 0.01). TPV peak (P < 0.01) and mean gradient (P < 0.01) decreased. RV end-diastolic area indexed to body surface area (BSA) (P < 0.01), Tricuspid regurgitation ( TR) gradient (P < 0.01), and the ratio of TR jet area to BSA (P < 0.01) decreased. Tricuspid inflow peak E:A, tissue Doppler imaging ( TDI): septal E' and A', TDI: tricuspid A' improved between baseline and discharge, but trended back to baseline by 6-month follow-up. Tricuspid valve annulus z-score, RV area change, tricuspid annular plane systolic excursion ( TAPSE), RV dP/dt, tricuspid E:E', and TDI: tricuspid annulus E' showed no change. Conclusion Improvements in pulmonary insufficiency and stenosis, RV size, and TR gradient and severity are seen after SAPIEN TPV placement. Selected indices of RV diastolic function improve immediately after TPV implantation, but return to baseline by 6 months. RV systolic function is unchanged. [ABSTRACT FROM AUTHOR]

ABSTRACT Objective To review the anomaly spectrum of prenatally detected absent pulmonary valve syndrome ( APVS) and the outcome after diagnosis. Previous fetal studies reported survival rates of ≤ 25% for patients with intended postnatal care. Methods Clinical data and echocardiograms of 12 cases with a fetal diagnosis of APVS between 2000 and 2010 were analyzed in this retrospective single-center study. Collected parameters included: gestational age at referral, associated fetal abnormalities, cardiothoracic ratio, maximum diameters of pulmonary annulus and main and branch pulmonary arteries, ventricular dimensions and function as well as ventricular Doppler flows. Karyotyping included fluorescence in-situ hybridization ( FISH) analysis for microdeletion 22q11.2. Results Median gestational age at diagnosis was 24 weeks. Three subtypes of APVS were observed: (1) with tetralogy of Fallot ( TOF) and no arterial duct ( n = 10; 83%); (2) isolated, with a large arterial duct ( n = 1; 8%); and (3) with tricuspid atresia, right ventricular dysplasia and a restricted duct ( n = 1; 8%). The cardiothoracic ratio and pulmonary artery dimensions were increased in all cases. The karyotype was abnormal in 70% of fetuses with TOF and their mortality rate was significantly higher due to pregnancy termination ( n = 3) or perinatal demise ( n = 2) (hazard ratio, 5; 95% CI, 0.87−28.9; P = 0.015). Of seven live births with active postnatal care, six children (86%) were alive without residual respiratory symptoms at a median follow-up of 4.7 (range, 2.1−10.6) years. Conclusion Outcome after fetal diagnosis of APVS was significantly better in this study compared with those of previous fetal series, with a low mortality rate for actively managed patients. [ABSTRACT FROM AUTHOR]

Objectives Tetralogy of Fallot with absent pulmonary valve syndrome (TETAPV) is reported in obstetric literature to have an extremely poor prognosis. We sought to determine the clinical outcome associated with TETAP V and whether prenatal diagnosis confers a poor prognosis. Methods All cases of TETAPV diagnosed in British Columbia between 1980 and 2009 were reviewed and grouped according to time of diagnosis, either prenatal or postnatal. The groups were compared with respect to mortality, respiratory problems, number of interventions and functional capacity at last follow-up. Results Eight and 11 patients were included in the prenatally and postnatally diagnosed groups, with overall long-term survival of 71% and 82%, respectively. There was no significant difference in mortality, frequency of preoperative intubation, number of interventions or functional capacity between groups. Conclusion From a population~based retrospective analysis of TETAPV cases identified over three decades it is concluded that the prognosis for YE TAP V is better than that previously reported in the obstetric literature. This information should be used to guide prenatal counseling. [ABSTRACT FROM AUTHOR]

We present an adult with metastatic carcinoid disease affecting the heart, in whom live/real time three-dimensional transthoracic echocardiography (3DTTE) provided incremental value over two-dimensional transthoracic echocardiography (2DTTE). Initial 2DTTE was able to demonstrate severe pulmonic and tricuspid regurgitation, but was unable to visualize the posterior leaflet of the tricuspid valve or the right (right anterior) leaflet of the pulmonic valve. Further analysis with 3DTTE demonstrated thickening, restricted mobility, and noncoaptation of all three leaflets of both the tricuspid and the pulmonary valves. En face viewing of tricuspid and pulmonary regurgitation vena contractas permitted more reliable quantification of regurgitation severity. In addition, localized, linear, echogenic areas consistent with carcinoid deposits were noted along the inner walls of the right atrium, atrial septum, and inferior vena cava. To the best of our knowledge, endocardial carcinoid deposits have never been reported by 2D or 3D echocardiography. En face viewing of these deposits by 3DTTE enabled measurement of their dimensions and areas. Subcostal examination also identified large circumscribed hepatic lesions consistent with metastatic disease. Neither the carcinoid deposits nor the metastatic lesions were detected by 2DTTE. This case demonstrates the usefulness of 3DTTE as a supplement to 2DTTE in more comprehensively assessing carcinoid involvement of the heart. (Echocardiography 2012;29:751-756) [ABSTRACT FROM AUTHOR]

Background: The cardiovascular manifestations of Marfan syndrome (MFS) are the main causes of morbidity and mortality. This study describes the clinical and echocardiographic findings in a Mestizo-Mexican population affected by the disease. Methods: A total of 166 patients previously diagnosed with MFS were recruited for the study, 114 of them underwent complete clinical history, with emphasis on Ghent nosology criteria, and transthoracic echocardiography, with 68 patients also undergoing transesophageal study. Results: Major cardiovascular criteria from the Ghent nosology predominated in adults (P < 0.0001), minor criteria in children (P = 0.007). Among pediatric patients, 83% had a New York Heart Association (NYHA) functional class of I; however, 64% of the adult patients had an NYHA class ≥II, (P < 0.0001). Corrected aortic echocardiographic measurements of both groups demonstrated statistically significant differences. Children had a greater prevalence of mitral valve prolapse, while adults more frequently presented with aortic complications. Seven patients died during follow-up from aortic complications, one child and six adults. Conclusions: Based on the data, we can conclude that MFS in the Mestizo-Mexican population has a distinctly different clinical pattern in children and adults, and a graver prognosis in adults. Adult patients with MFS are significantly more likely, than children, to have aortic dilation, aortic aneurysm, aortic regurgitation, aneurysm rupture, aortic dissection, and fatal outcome. Children with MFS are more likely, than adults, to present with asymptomatic mitral and tricuspid prolapse and mitral valve regurgitation. (Echocardiography 2010;27:923-930) [ABSTRACT FROM AUTHOR]

Introduction: Pulmonary regurgitation (PR) following repair of right ventricular (RV) outflow obstruction is related to slowly progressive RV dilatation and heart failure and will eventually require surgical intervention, but optimal timing of pulmonary valve replacement is challenging. Tissue Doppler based quantification of RV contractility may offer additional information in the management of these patients. Methods: In a porcine animal model free PR was induced by percutaneous stenting of the pulmonary valve orifice (N = 23). After 1, 2, or 3 months of free PR percutaneous pulmonary valve replacement (PPVR) was performed. Tissue Doppler derived measures of global and regional myocardial contractility were obtained by transthoracic echocardiography, and compared to a sham-operated control group (N = 9). Results: Free PR is associated with RV dilatation (RV end-diastolic area increased from 15 ± 3 to 23 ± 7 cm2/m2, P < 0.0001) and a decrease in RV fractional area change from 62 ± 10% to 57 ± 12%, P = 0.08, with no impact of duration of free PR. The isovolumic acceleration, regional strain, and strain rate were unchanged after free PR and after PPVR. No consistent relation of echocardiographic measures of contractility and response to PPVR could be identified. Conclusion: Echocardiographic measures of RV contractility remained unchanged, despite significant RV remodeling following chronic PR and PPVR persistently induced significant recovery in the majority of the animals. These results may imply that Tissue Doppler based measures of RV contractility may not be sufficiently sensitive to be a suitable adjunct to conventional echocardiography in the follow-up of patients with free PR in order to optimize timing of valve replacement. (Echocardiography 2010;27:854-863) [ABSTRACT FROM AUTHOR]

Objective: To determine the incidence of reversed end-diastolic flow (REDF) in the umbilical artery in high-risk first-trimester pregnancies and evaluate associated conditions. Methods: This was a prospective evaluation of the umbilical artery Doppler waveforms of 614 consecutive high-risk pregnancies between 10 and 14 weeks of gestation, to determine those with REDF. The associated anomalies and characteristics of these fetuses were then investigated. Results: In 278/614 (45.3%) fetuses, there was positive end-diastolic flow in the umbilical artery; in 331/614 (53.9%) end-diastolic flow was absent and in 5/614 (0.8%) there was REDF. Three of the five fetuses with REDF had tetralogy of Fallot (TOF) with absent pulmonary valve syndrome (APVS) and a patent ductus arteriosus, and all three showed signs of cardiac failure, with reversed blood flow in the ductus venosus during atrial systole and generalized skin edema. Another fetus had a large ventricular septal defect and the remaining fetus had agenesis of the ductus venosus. Three fetuses had trisomy 18 and one had trisomy 13. Conclusions: REDF in the umbilical artery is very rare in early pregnancy and mostly occurs in association with major fetal vascular anomalies and cardiac defects, particularly TOF with APVS and patent arterial duct. We propose that the patency of the arterial duct in TOF with APVS leads to heart failure with subsequent demise early in pregnancy. Therefore, the frequent absence of the arterial duct observed in APVS in later pregnancy is more likely to be a result of early selection than a prerequisite for the development of this lesion as has been proposed previously. [ABSTRACT FROM AUTHOR]

Cardiac MRI (CMR) is a noninvasive diagnostic tool with comprehensive capabilities similar to that of two-dimensional echocardiography with Doppler. In addition to the ability to evaluate the etiology and severity of pulmonary valve regurgitation (PR), CMR is well designed to serially monitor the impact of the PR on the right ventricle (RV). Importantly, RV dilation and dysfunction is a critical determinate to time surgical intervention. CMR gives the silent RV, suffering from PR, a voice. [ABSTRACT FROM AUTHOR]

A patient with eroded tricuspid and pulmonic valves, who eventually developed elevated right atrial and systemic venous pressure that led to hepatic cirrhosis and recurrent pleural effusion, is presented. The complex issues involved over a long period in this patient, who ultimately required combined heart and liver transplant, are discussed. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 30-year-old woman who undergoes early fetal echocardiography after an increase in fetal nuchal translucency thickness. The fetus undergoes presumptive diagnosis for absent pulmonary valve syndrome associated with membranous tricuspid atresia, which was confirmed by a follow-up scan. Further pregnancy complications prompted elective Cesarean section. The male newborn successfully completed a bidirectional cavopulmonary anastomosis at the age of 8 months.

We report on a premature neonate who presented with cyanosis soon after birth. She was found on echocardiography to have an unguarded tricuspid valve orifice associated with pulmonary valve atresia and an intact interventricular septum. Owing to the small-sized pulmonary arteries, she was initially managed conservatively with prostaglandin infusion, and at one year of age underwent a successful bidirectional cavo-pulmonary (Glenn) shunt operation. [ABSTRACT FROM AUTHOR]

The quadricuspid pulmonary valve is a rare congenital anomaly. It tends to be clinically quiescent. Its diagnosis by two-dimensional echocardiography could be very difficult because of the anatomical features. We report on the echocardiographic findings of a 66-year-old female patient with mitral and aortic regurgitation of rheumatic origin and severe pulmonary hypertension. There was an aneurismatic dilation of the pulmonary artery trunk allowing visualization of the short-axis view of the pulmonary valve. It showed four cusps of similar size and an important deficit of central coaptation. Very few cases of the quadricuspid pulmonary valve are documented in live patients. [ABSTRACT FROM AUTHOR]

An abstract of the article "Semilunar valve growth in fetal transposition of the great arteries - is pulmonary valve size predictive of postoperative aortic root dilatation?" by S. B. Clur and colleagues is presented.

The article presents a case study of a 49-year-old man who presented to the hospital with complaints of symptoms including anorexia, tingling and numbness in his hands. An echocardiogram was performed and showed a mass in the right ventricular outflow tract with pulmonary valve regurgitation. A transesophageal echocardiograph showed a large mass attached to the anterior pulmonary valve. The mass was removed and a histological examination demonstrated that the mass was a papillary fibroelastoma.

An abstract of the research paper "First trimester sonographic finding in fetus having tetratology of Fallot with abscent pulmonary valve syndrome: case report," by J. Lee and colleagues is presented.