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Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1–58) days and 11 (22%) underwent outpatient PBV at 51.8 (11–174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02–94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS. [ABSTRACT FROM AUTHOR]

Patients after the Ross procedure are at risk for right (RV) and left ventricular (LV) dysfunction due to neo-aortic and pulmonary dysfunction. While neo-aortic root dilatation has been related to LV dysfunction, the potential contributions of aortic stiffness and ventricular interactions have not been evaluated. Patients status post Ross procedure up to age 18 years with cardiac magnetic resonance (CMR) exam from 2007 to 2018 were retrospectively reviewed. Aortic pulse wave velocity (PWV) was calculated from phase contrast and angiogram images. RV and LV peak global longitudinal (GLS) and circumferential strain (GCS) were measured using tissue tracking software. Multivariable regression was performed for variables associated with parameters of LV function. In 58 patients (median age 20.5 years at CMR exam), male gender, longer time since Ross procedure, aortic root dilatation, and lower RV ejection fraction (EF) were associated with decreased LV EF. There was no association with LV late gadolinium enhancement or neo-aortic or conduit regurgitation. LV GCS and GLS also correlated with RV GCS, RV GLS and PWV. In multivariable analysis, the relation of RV and LV systolic function, but not aortic measurements, remained significant. In conclusion, in long-term follow-up after pediatric Ross procedure, RV function rather than aortic root size or aortic stiffness most closely relates to LV function. Ventricular interactions may impact decision-making on timing of conduit intervention, which could differ from established criteria in populations with only aortic or pulmonary valve disease. Further study is warranted to evaluate possible association with clinical outcome. [ABSTRACT FROM AUTHOR]

Previous studies have suggested reduced pulmonary valve annulus (PVA) growth and progression of pulmonary outflow obstruction in fetuses with tetralogy of Fallot (TOF). The goals of this study were to (1) investigate the trajectory of PVA growth in utero, and (2) compare two methods of z-score determination for fetal and postnatal PVA size by echocardiography in order to improve prenatal counseling for patients with TOF. Fetal echocardiograms (FE) at a single institution with a diagnosis of TOF between 8/2008 and 12/2015 were retrospectively reviewed. Patients included had at least 2 FEs and 1 immediate postnatal echocardiogram (TTE). Fetal and postnatal demographic, clinical, and echocardiographic data were collected. Fetal body surface area (BSA) was calculated by estimating fetal weight and height; z-scores were determined based on fetal gestational age (GA) and BSA for both FEs and TTEs. Fetal PVA z-scores by GA or BSA were then compared to postnatal PVA z-scores by BSA. Twenty-two patients with 44 FEs and 22 TTEs were included. GA at the first FE was 23 weeks ± 3.4 and 32 weeks ± 3.1 at the second FE. There was no difference in PVA z-scores (by BSA) between the first and second FE (p = 0.34), but a decrease in PVA z-scores (by BSA) between the second FE and TTE (− 1.6 ± 0.5 vs. − 2.0 ± 0.7; p = 0.01). Repeat comparison with fetal PVA z-scores indexed to GA revealed no difference in z-scores between the first and second FE, but an increase in PVA z-scores between the second FE (by GA) and TTE (by BSA) (− 4.1 ± 1.0 vs. − 2.0 ± 0.7; p < 0.0001). The rate of PVA growth between the two FEs (23 µm/day ± 9.8) and between the second FE and TTE (28 µm/day ± 42) remained comparable (p = 0.57); however, the rate of BSA increase was greater in later gestation (9 cm2/day ± 3 vs. 20 cm2/day ± 11; p = 0.001). In patients with TOF, the rate of PVA growth appears to remain consistent through gestation; however, somatic growth rate increases in late gestation. Fetal PVA z-scores indexed to GA are thus inaccurate in predicting postnatal PVA z-scores typically indexed to BSA. This observation should be considered during prenatal consultation and delivery planning. [ABSTRACT FROM AUTHOR]

Pulmonary regurgitation (PR) is a common residual lesion and major determinant of outcome following surgical repair for tetralogy of Fallot. We sought to longitudinally study a previously described echocardiographic index as a correlate of PR measured by cardiac magnetic resonance imaging (CMR). We conducted a retrospective longitudinal study of patients with baseline and follow-up echocardiogram and CMR. The baseline studies were obtained as part of a research protocol, while the follow-up studies were performed for clinical purposes. On echocardiogram, the ratio of diastolic and systolic time-velocity integrals (DSTVI) in the main pulmonary artery was calculated. The Wilcoxon matched-pairs signed-rank test was used to test for individual changes in PR on echocardiogram and CMR. A linear regression of pulmonary valve regurgitant fraction (RF) was fit on DSTVI to identify clinically meaningful cut points of DSTVI. Thirty-five subjects were included, age at follow-up 18.3 ± 3.5 years. The follow-up between consecutive CMRs was a median time of 60 months (interquartile range 46-73). There was a moderate correlation between DSTVI and PR measured as RF by CMR ( r = 0.62, p = 0.0001). A CMR RF of 20 and 40 % (the boundaries between mild/moderate and moderate/severe PR) corresponded with DSTVI of 0.52 and 0.79 (95 % CI 0.39; 0.66, and 0.69; 89), respectively. There was no significant change in either DSTVI ( p = 0.61) or PR ( p = 0.89) from baseline to follow-up. This study lends further credence to the DSTVI as an accurate reflection of PR. This index might become helpful in the routine echocardiographic assessment of PR. Further studies are needed to determine whether changes in RF by CMR result in changes in DSTVI. [ABSTRACT FROM AUTHOR]

To evaluate the long-term cumulative incidence of ≥ moderate pulmonary regurgitation (PR) and re-intervention following balloon pulmonary valvuloplasty (BPV). While BPV for pulmonary valve stenosis (PS) relieves obstruction acutely, long-term outcomes are not well documented. Between 1982 and 2002, 211 patients had BPV for isolated PS. Follow-up data were available for 103 patients. Cumulative incidence of ≥ moderate PR and re-intervention was evaluated and risk factors for ≥ moderate PR assessed in univariate and multivariable analyses. Median age at BPV was 0.7 years (range 1 day-42.2 years); peak catheter gradient was 65 mmHg (range 31-169 mmHg); 23% had critical PS. Sixty-two patients had a recent echocardiogram with median follow-up 15.1 years (range 10.1-26.3 years); 60% had ≥ moderate PR. Three patients had pulmonary valve replacement following BPV due to symptomatic severe PR. In univariate analysis, critical PS, younger age, smaller BSA, and smaller pulmonary annulus at the time of BPV, as well as greater baseline PS gradient by catheterization, were associated with ≥ moderate PR (all p < 0.05). In multivariable analysis, only BSA < 0.3 m was independently associated with ≥ moderate PR (adjusted odds ratio 6.4, 95% confidence interval 1.2-33.6). In the largest study to date of > 10-year outcomes following BPV, 60% of patients with available follow-up data developed ≥ moderate PR. Few patients had pulmonary valve replacement. Patients with lower BSA at the time of BPV were more likely to have greater PR at late follow-up. [ABSTRACT FROM AUTHOR]

The aim of this study was to determine the utility of intracardiac echocardiography (ICE) in assessing Melody™ transcatheter pulmonary valve (TPV) function immediately following valve implantation. ICE is used increasingly in percutaneous cardiac interventions. At our center, ICE is routinely utilized to evaluate valve function following Melody TPV implantation, but the utility of this practice remains unclear. A retrospective review of all Melody valves placed in the right ventricular outflow tract from April 2010 to September 2013 was performed. The clinical utility of ICE was described, along with the relationship between ICE data and traditional hemodynamic/angiographic data. ICE was performed in 54 cases and provided excellent Melody TPV visualization with no complications. ICE did not change clinical management but did provide supplemental information in two cases. In one case, angiography showed severe catheter-related Melody insufficiency. Subsequent ICE confirmed no insufficiency and prevented the need for additional angiography. In the second case, ICE allowed characterization of the mechanism of a residual gradient. ICE did not detect any clinically significant paravalvar leaks or valvar insufficiency not seen by angiography. The peak catheterization gradient was more closely approximated by the mean ICE gradient (median difference −7.4 % between measurements) than by the peak ICE gradient (median difference 58.3 %; p < 0.0001). ICE provides excellent and safe visualization following Melody TPV implantation but did not provide new clinical information impacting management in this series. Selective use of ICE in cases with more than expected valve insufficiency or larger than expected residual gradients may streamline use while maintaining optimal clinical outcomes. [ABSTRACT FROM AUTHOR]

Patients may develop hemodynamic abnormalities after right ventricular outflow tract (RVOT) repair. Re-intervention timing remains a dilemma. This study evaluates exercise capacity and RV function before and after intervention using age-related comparisons. Twenty-six patients with severe pulmonary regurgitation (PR) after initial repair scheduled for pulmonary valve replacement (PVR) were enrolled. Metabolic treadmill testing (EST) and MRI were obtained before and after surgery. EST results were compared with matched controls. Preoperative exercise time and peak oxygen consumption (VO max) were significantly diminished compared with controls but were not significantly different postoperatively. The patients were then split into age-related cohorts. When comparing pre-PVR and post-PVR exercise time and VO max among themselves, neither cohort showed significant differences. However, patients younger than 25 years had better postoperative results, an age-related difference not seen in the controls. Preoperative MRI showed significantly dilated RV, PR, and low normal function. After PVR, the right to left ventricular end-diastolic volume ratio (RVEDV:LVEDV) and pulmonary artery regurgitant fraction (RF) significantly decreased. There was no change in ventricular ejection fractions (EF). Severe PR, decreased RVEF, and RV dilation can significantly diminish exercise capacity. PVR improves RVEDV:LVEDV and RF, but not EF. Younger patients had better exercise capacity that was maintained postoperatively. This age-related difference was not seen in the controls, indicating that earlier intervention may preserve exercise capacity. Serial ESTs in patients with severe PR following RVOT repair may identify deteriorating exercise capacity as an early indicator for the need for PVR. [ABSTRACT FROM AUTHOR]

Clinicians are currently encountering an increasing number of patients in the long-term period after tetralogy of Fallot (TOF) repair presenting with pulmonary valve regurgitation (PR) or right ventricular (RV) dysfunction. The purpose of this study was to evaluate the clinical utility of the plasma brain natriuretic peptide (BNP) level and consider surgical indications and timing of pulmonary valve replacement (PVR). We examined 33 patients (21 males, 12 females, mean age 14.5 ± 2.8 years) who underwent TOF repair at Kitasato University Hospital. All patients were evaluated using echocardiography and blood sampling. The mean age at the time of initial repair was 1.3 ± 0.7 years. The patients with moderate-severe PR exhibited significantly higher plasma BNP levels than the patients with trivial-mild PR (mean 37.5 ± 33.1 vs. 17.3 ± 6.6 pg/ml, p = 0.013). The mean plasma BNP level with cardiac symptoms was higher than that observed in the patients without any symptoms (71.4 ± 46.1 vs. 25.0 ± 14.0 pg/ml, p = 0.005). The mean BNP level was significantly decreased after PVR (71.3 ± 46.1-26.1 ± 13.2 pg/ml, p = 0.009), and the plasma BNP level was found to be positively correlated with the RV end-diastolic pressure ( r = 0.851; p = 0.008). The optimal BNP cut-off value for considering PVR was 32.15 pg/ml (sensitivity, 85.7 %; specificity, 83.3 %). The plasma BNP level may become a useful diagnostic tool for considering the indications and optimal timing of PVR over the long term after TOF repair. [ABSTRACT FROM AUTHOR]

The long-term benefits of pulmonary annulus preservation in tetralogy of Fallot (ToF) repair in patients with a marginally small pulmonary annulus are controversial. We sought to determine whether pulmonary annulus preservation (AP) is superior to transannular patching (TAP) in lowering the risk of pulmonary valve implantation (PVI) long after the repair of ToF. Of the 255 patients who underwent total correction of ToF during infancy between January 1989 and December 2005, 114 patients (AP group = 57, TAP group = 57) were selected by propensity score matching for various preoperative variables, such as age and body weight at operation, sex, pulmonary artery size, pre-repair palliation, anatomical types of ventricular septal defect, and Z-score of pulmonary valve annulus diameter (PVA-Z). The PVA-Z of the AP and TAP groups were −2.3 ± 1.3 and −2.1 ± 1.3, respectively ( p = 0.547). The time to PVI was compared between the two groups. The median follow-up duration was 146 months (AP group: 141 months, TAP group: 147 months; p = 0.191). During the follow-up periods, there were 12 reoperations for the relief of right ventricular outflow tract obstruction (RVOTO), eight PVIs, and three late deaths. While freedom from reoperation for RVOTO was comparable between the two groups ( p = 0.182), freedom from PVI at postoperative 15 years was significantly lower in the TAP group than in the AP group (74 and 100 %, p = 0.015). In repairing ToF with marginally small pulmonary valve annulus, AP is associated with a lower risk of late postoperative PVI. [ABSTRACT FROM AUTHOR]

Pulmonary valve replacement (PVR) for pulmonary valve insufficiency (PVI) currently represents the most frequent reoperation performed for adults with congenital heart disease. A variety of pulmonary valve substitutes have been used, but none has proved to be ideal. This report reviews the authors' experience using a porcine prosthetic valve in the pulmonary position. Between January 2001 and December 2011, 76 patients (mean age, 36 years; range, 18-64 years) underwent PVR for chronic PVI using a porcine bioprosthesis. All the patients had previously undergone surgery: 65 for repair of tetralogy of Fallot and 11 for pulmonary surgical valvotomy. Magnetic resonance imaging (MRI) evaluations before surgery and at the 1-year postoperative follow-up evaluation were compared. Aside from the PVR, 59 patients (59/65, 78 %) received 94 associated cardiac surgical procedures. Two hospital deaths occurred. The mean hospital stay was 13 days (range, 7-48 days). At the 1-year control MRI, pulmonary regurgitation fraction, right ventricular end diastolic volume (RVEDV), and RV/LV EDV had improved significantly. During a mean follow-up period of 52 months (range, 6-132 months), one patient died. All the patients were categorized as New York heart association (NYHA) functional class 1. No episodes of structural valve deterioration, endocarditis, or thromboembolic event were noted. Echocardiography showed trivial or no PVI in all the patients. The porcine bioprosthetic valves demonstrated excellent midterm results in the RV outflow tract reconstruction. The hemodynamic characteristics of this valve are comparable with those of homografts or valved conduits. It is easy to implant and allows for avoiding extensive dissection, especially of the pulmonary arteries. [ABSTRACT FROM AUTHOR]

Many congenital heart defects with pulmonary valve pathology are repaired or palliated in childhood. It is anticipated that these patients will need subsequent pulmonary valve replacement due to the pulmonary regurgitation or right ventricular dilation/failure that results from their original operation. Interestingly, some patients with prior congenital heart surgery and no congenital pulmonary valve pathology require pulmonary valve replacement in adulthood. The current study analyzed this subset of patients. The pediatric and adult congenital cardiac surgical databases at a large academic center were reviewed from 2001 to 2008 for pulmonary valve replacements. Patients without congenital pulmonary valve disease were identified. Preoperative, operative, and postoperative data were analyzed. Between 2001 and 2008, five patients with congenital heart disease but no pulmonary valve pathology underwent late pulmonary valve replacement. The initial congenital diagnoses were coarctation/ventricular septal defect ( n = 3), complete atrioventricular septal defect ( n = 1), and anomalous left coronary artery from the pulmonary artery (PA) ( n = 1). All five patients had undergone main PA manipulation during their previous operations. Four of these patients had received PA banding and debanding, and one had received a Takeuchi tunnel repair and revision. All the patients underwent pulmonary valve replacement secondary to pulmonary regurgitation and right ventricular dilation. The average time from the last previous operation to the time of pulmonary valve replacement was 20.8 ± 9.1 years. Bioprosthetic valves were used for all the pulmonary valve replacements, and there were no mortalities. Patients who have undergone previous PA manipulation, regardless whether congenital pulmonary pathology is present, may be at risk for pulmonary valve replacement in adulthood. This report describes five cases of patients with a history of congenital heart surgery but no congenital pulmonary valve pathology who required pulmonary valve replacement due to the consequences of prolonged pulmonary regurgitation. Although pulmonary regurgitation may be well tolerated for many years, it is further evidence for the importance of close follow-up assessment and monitoring of young adults with congenital heart disease. [ABSTRACT FROM AUTHOR]

The article presents the case study of a 10-year-old male patient with common atrium, single ventricle, common atrio ventricular (AV) canal, severe pulmonary stenosis. It describes the patient's condition who underwent bidirectional Glenn shunt and extra heart fenestrate Fontan operation. The patient was diagnosed with cyanosis arising from the Fontan surgery with a large fistula from the left hepatic vein to the atrium.

The article describes the case of a 33-year-old woman who was referred to the hospital at 38 weeks of gestation with the diagnosis of fetal congenital heart disease. It discusses the findings of a fetal echocardiography which showed tetralogy of Fallot (TOF) associated with an absent pulmonary valve (APV) which was confirmed by neonatal echocardiography and three-dimensional computed tomography.

A correction is presented to the article "Preprocedural Risk Assessment Prior to PPVI with CMR and Cardiac CT" which appeared in the 2017 issue.

The article presents a case study of a 34-week-gestation fetus of a 29-year-old pregnant mother who was referred to the hospital due to an obstetric ultrasonographic examination which showed an abnormal vascular image. It states that the mother was using valproate due to epilepsy. Furthermore, in addition to the diagnosis of an absent pulmonary valve and tetralogy of Fallot, dextroversion due to right lung atelectasia was confirmed by postnatal echocardiography.

The article discusses a study on the diagnosis of bilateral ductus with discontinuous branch pulmonary arteries and heterotaxia syndrome in neonates using transthoracic echocardiography. The researchers examine the effect of congenital heart defects, pulmonary valve atresia and patent ductus arteriosus (PDA) on conotruncal cardiac anomaly. The diagnosis reveals several complications including the unbalanced atrioventricular canal defect, pulmonary atresia and hypoplasia of the right ventricle.

The article focuses on a full-term male newborn infant who experienced echocardiography. Huge mass on the upper part of the right lung was discovered during chest radiography. Findings included Tetralogy of Fallot (TOF) with absent pulmonary valve, huge dilatation of right pulmonary artery and bilateral superior vena cava. It claimed that successful operation resulted in reduced heart size and improvement in patency of both bronchi.