Branch Pulmonary Artery Diameters Research Articles

Surgical and transcatheter pulmonary valve replacement in patients with repaired tetralogy of Fallot: cardiac magnetic resonance imaging characteristics and morphology of right ventricular outflow tract.

Pulmonary valve replacement is recommended in patients with repaired tetralogy of Fallot based on cardiac magnetic resonance imaging (MRI) criteria. This procedure is performed by surgical or transcatheter approaches. We aimed to investigate the differences in preprocedural MRI characteristics (volume, function, strain) and morphology of the right ventricular outflow tract and branch pulmonary arteries in patients for whom surgical or transcatheter pulmonary valve replacementwas planned. Cardiac MRI of 166 patients with tetralogy of Fallot were analyzed. Of these, 36 patients for whompulmonary valve replacement was plannedwere included. Magnetic resonance imaging characteristics, right ventricular outflow tract morphology, branch pulmonary artery flow distribution and diameter were compared between surgical and transcatheter groups. Spearman correlation and Kruskal-Wallis tests were performed. Circumferential and radial MRI strain for the right ventricle were lower in thesurgical group (P=0.045and P=0.046, respectively). The diameter of the left pulmonary artery was significantly lower (P=0.021) and branch pulmonary artery flow and diameter ratio were higher (P=0.044and P = 0.002, respectively) in the transcatheter group. There was a significant correlation between right ventricular outflow tract morphology and right ventricular end-diastolic volume indexand global circumferential and radial MRI strain (P=0.046, P=0.046and P= 0.049, respectively). Preprocedural MRI strain, right-to-left pulmonary artery flow, diameter ratio and morphological features of the right ventricular outflow tract were significantly different betweenthe two groups. A transcatheter approach may be recommended for patients with branch pulmonary artery stenosis, since both pulmonary valve replacement and branch pulmonary artery stenting can be performed in the same session.

Risk of pulmonary artery dissection in adults with congenital heart disease

BackgroundThere are limited data about the risk of pulmonary artery (PA) dissection in adults with congenital heart disease (CHD), and the purpose of this study was to estimate the incidence of PA dissection in this population. MethodsRetrospective cohort study of adults with CHD that underwent cross-sectional imaging (2003–2020). PA aneurysm was defined as main or branch PA diameter > 40 mm or > 30 mm respectively, and severe PA aneurysm was defined as main or branch PA diameter > 50 mm. ResultsOf 1, 673 patients (41 ± 10 years; male 58%), 493 (24%), 286 (19%), and 306 (20%) had aneurysms of the main, right, and left PA respectively, while 66 (4%) had severe PA aneurysm. During a median follow-up of 8.2 (interquartile range 3.7–10.3) years, there was one PA dissection in a patient with Eisenmenger syndrome, thus the incidence of PA dissection was 14 per 100,000 patient-years.Of 779 females, 163 had one or more pregnancies during follow-up, and 41 (25%) of these patients had known PA aneurysm at the time of conception. There was no PA dissection during pregnancy. Of the 163 patients, 91 (56%) had cross-sectional imaging before and after pregnancy, there was no significant difference in PA dimension before versus after pregnancy (main PA 35 ± 5 versus 36 ± 4 mm, p = 0.6; right PA 21 ± 3 versus 33 ± 4 mm, p = 0.1; and left PA 23 ± 4 versus 22 ± 4 mm, p = 0.4). ConclusionsThe risk of PA dissection was extremely low even in patients with severe PA aneurysm, or in patients with PA aneurysm that became pregnant. Collectively, these data suggest a benign natural history for patients without severe pulmonary hypertension and encourage and a conservative approach in managing patients with PA aneurysm.

Long-term evolution of stents implanted in branch pulmonary arteries

Branch pulmonary artery stenosis complicates the management of congenital heart diseases. Surgical branch pulmonary artery angioplasty is associated with a high reintervention rate. As an alternative, percutaneous or intraoperative branch pulmonary artery stents have been implanted to improve efficiency, but long-term evaluations are limited. To describe the long-term evolution of branch pulmonary artery stents. We conducted a retrospective cohort study at Tours University Hospital. All stents implanted by surgery or catheterization in branch pulmonary arteries with a minimum follow-up of 12 months and at least one catheterization control were included. The primary endpoint combined cardiovascular mortality, surgical or percutaneous reintervention for stent complication or new stent implantation. Between 2007 and 2017, 76stents in 51patients were included (62stents implanted by surgery, 14 by catheterization). At implantation, the patients' mean age and weight were 4.7years (interquartile range 4.2years) and 17.3kg (interquartile range 11.0kg), respectively. Mean branch pulmonary artery minimum diameter was 4.1±2.1mm (mean Z-score-4.9±2.9), and mean initial stent diameter was 9.1±3.1mm. During a follow-up of 5.3years (range 0-11.2 years), freedom from primary endpoint was 86.8% (95% confidence interval 79.6-94.8%) at 1 year, 71.5% (95% confidence interval 61.9-82.7%) at 5years and 69.6% (95% confidence interval 59.6-81.2%) at 10 years. We did not identify any factors associated with major adverse cardiovascular events. Among stents without major adverse cardiovascular events, the mean branch pulmonary artery diameter Z-score at last evaluation had increased by +4.8±3.2 compared with the initial diameter (P<0.001). After stent implantation, a median of 2 re-expansions were performed for each stent (range 0-7). Stent implantation should offer a good long-term solution for branch pulmonary artery stenosis, although iterative re-expansions are required.

Morphological and clinical indices determining mode of repair in children with tetralogy of Fallot in Enugu, Nigeria

Background: Children with tetralogy of Fallot (TOF) present with various clinical and morphological attributes which aid in screening for primary, stage, or elective repair. Objectives: This study is aimed at determining the morphological and clinical indices that predict mode of repair among children with TOF in our locale. Materials and Methods: This was a retrospective echocardiographic review of children with TOF carried out in three different institutions over a 5-year period. Results: The mean age of presentation was 5.0 ± 5.3 years. The nutritional status revealed that 18.2% (8/44) were wasted, 25.0% had severe wasting, while 2.3% were obese. Stunting was observed in 4.5% of the patients and severe stunting in 29.5%. The mean pulmonary valve (PV) annulus of 43 of the patients assessed was 11.0 ± 5.5 mm (mean z-score: −2.03 ± 1.95). Five of them (11.6%) had values one standard deviation (1SD) below the mean of the standard population, 32.6% had values 2SD below the mean, and 27.9% had values 3SD below the mean. Of the 33 children whose right pulmonary artery (RPA) z-scores were assessed, 24.2%, 12.1%, and 12.1%, respectively, had RPA z-scores of 1SD, 2SD, and 3SD below the mean of the standard population, while 17.5% and 7.5% of 40 children whose left pulmonary arteries were assessed had z-scores of 1SD and 2SD below the standard mean, respectively. Right aortic arch and major aortopulmonary collateral arteries (MAPCAs) were seen in 8.1% and 30.5% of the children, respectively. 30.5% of the children with TOF had MAPCAs. Features of Down syndrome were observed in 4.8% (3/62) of the patients. Conclusion: A large proportion of patients present with various degrees of malnutrition with a significant low z-score for PV annulus and branch pulmonary artery diameters. The presence of right aortic arch and MAPCAs was observed in the patients. The increased rate of protein–energy malnutrition, small PV annulus, MAPCAs and presence of right aortic arch, etc., are screening tools that may assist the surgeon in selecting the various surgical options and timing in the approach of children with TOF in this locale.

Factors associated with perioperative mortality in children and adolescents operated for tetralogy of Fallot: A sub-Saharan experience.

Patients with tetralogy of Fallot are now surviving to adulthood with timely surgical intervention. However, many patients in low-income countries have no access to surgical intervention. This paper reports the surgical access and perioperative mortality in a sub-Saharan center that was mainly dependent on visiting teams. We reviewed records of patients operated from January 2009 to December 2014. We examined perioperative outcomes, primarily focusing on factors associated with perioperative mortality. During this period, 62 patients underwent surgery. Fifty-seven (91.9%) underwent primary repair, while 5 (6.5%) underwent palliative shunt surgery. Of the five patients with shunt surgery, four ultimately underwent total repair. Eight (12.9%) patients died during the perioperative period. Factors associated with perioperative mortality include repeated preoperative phlebotomy procedures (P < .001), repeated runs and long cardiopulmonary bypass time (P < .001), and aortic cross-clamp time (P < .001), narrow pulmonary artery (PA) valve annulus diameter (P = .022), narrow distal main PA diameter (P = .039), narrow left branch PA diameter (P = .049), and narrow right PA diameter (P = .039). Of these factors, cardiopulmonary bypass time/aortic cross-clamp time and pulmonary valve annulus diameter less than three SD were independently associated with perioperative mortality. In this series of consecutive patients operated by a variety of humanitarian surgical teams, cardiopulmonary bypass time/aortic cross-clamp time, and pulmonary valve annulus diameter less than three SD were independently associated with perioperative mortality risk. As some of these factors are modifiable, we suggest that they should be considered during patient selection and at the time of surgical intervention.

Parameters of fetal pulmonary vascular health: baseline trends and response to maternal hyperoxia in the second and third trimesters.

Several parameters, including branch pulmonary artery (PA) diameter and Doppler-derived PA acceleration-to-ejection time ratio (AT/ET), peak late-systolic/early-diastolic reversed flow (PEDRF) and pulsatility index (PI) response to maternal hyperoxia, have been used to investigate fetal pulmonary health. Lower AT/ET, increased PEDRF and lack of PI response to hyperoxia have been observed in fetuses with severe lung hypoplasia and are considered markers of pulmonary vascular resistance. We sought to further define the evolution of PA diameter and Doppler parameters and their response to maternal hyperoxia in healthy fetuses. Fifty-four prospectively recruited women with healthy pregnancy underwent fetal echocardiography from 18-36 weeks of gestation. After baseline branch PA diameter and Doppler assessment, oxygen (8-10 L/min) was administered by non-reservoir facemask for 10 min and PA Doppler parameters were reassessed. Branch PA diameters and AT/ET increased linearly with gestational age, while PEDRF increased quadratically (P < 0.001 for all) and PA-PI did not change. In response to maternal hyperoxia, although most fetuses demonstrated a significant decrease in PI for both branch PAs (right PA, P = 0.025; left PA, P = 0.040) ≥ 30 weeks, significant variability was observed in PI response with 31% of cases demonstrating either no response or a slight decrease. No other parameter demonstrated a measurable change in response to maternal hyperoxia. From the mid-trimester, fetal branch PA diameters and AT/ET increase linearly and PEDRF increases quadratically, whereas PI remains unchanged. Although maternal hyperoxia triggers a significant decrease in PA-PI after 30 weeks, variability in this response may reduce its utility in clinical practice. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Normal Reference Ranges for Pulmonary Artery Diameters in Preterm Infants.

To establish normal reference ranges and Z-scores for pulmonary artery diameters in preterm infants and their correlation with body surface area, body weight, and chronological age. In a prospective study, 268 preterm infants, who fulfilled the inclusion criteria were examined. Echocardiograms were performed to measure the main pulmonary artery, right pulmonary artery, and left pulmonary artery diameters on day(s) 0 to -6 of life and at weekly intervals until they reached 36weeks of age. Body surface area was divided into thirteen groups from 0.07 to 0.19m2. The mean gestational age was 29.8 (±2.38 SD) weeks, ranging between 24 and 35, the mean body weight was 1479 (±413 SD) grams, ranging between 588 and 3380, and the mean body surface area was 0.13m2, ranging between 0.07 and 0.19m2. All the pulmonary artery diameters correlated well with both body weight and body surface area. Reference ranges, with mean±SD, range, and Z-scores for aortic diameters according to body surface area were calculated. A significant gradual increase was observed in main and branch pulmonary artery diameters with increasing body surface area. Overall, a progressive and significant increase for main and branch pulmonary artery diameters was observed during the first nine weeks of life. The main and branch pulmonary artery diameters were found to have significant correlation with body surface area. The study also provides reference data with Z-scores, which can be used as a normal reference tool for measuring the main pulmonary artery, right, and left pulmonary artery diameters of preterm infants against body surface area.

Usefulness of the Prenatal Echocardiogram in Fetuses With Isolated Transposition of the Great Arteries to Predict the Need for Balloon Atrial Septostomy

The outcome of the arterial switch operation for transposition of the great arteries (TGA) is excellent, but there is still associated preoperative mortality. Hypoxemia due to inadequate mixing of the pulmonary and systemic circulations may be implicated. Prediction of early hypoxemia by prenatal echocardiographic criteria has proved difficult. We aimed to identify prenatal echocardiographic features that may predict the need for emergency balloon atrial septostomy (BAS) in isolated TGA. Third trimester fetal echocardiograms of the last 40 cases of isolated TGA were reviewed without knowledge of the postnatal outcome. Measurements of the arterial valves, arterial duct, total septal length (TSL), and foramen ovale (FO) length were made, in addition to a subjective assessment of the atrial septum. The first postnatal echocardiogram and charts were reviewed. Comparison with 40 gestation-matched control fetuses was performed. The FO length in normal fetuses was not significantly different from those with TGA who did not require an emergency BAS but was significantly smaller in fetuses with TGA who required an emergency BAS (p= 0.01). An emergency BAS was required in 12 of 40 cases. All 3 cases with limited movement of the atrial septum required emergency BAS. A hypermobile atrial septum was observed in 10 cases and was not associated with emergency BAS (p= 0.8). The FO:TSL was significantly smaller in those who required an emergency BAS with good predictive value (area under the receiver operating characteristics curve: 0.80). The sensitivity for FO:TSL <0.5 was 99%. There was no significant difference in arterial duct, pulmonary valve, or branch pulmonary artery diameters between those cases requiring emergency BAS and those who did not. In conclusion, the likelihood of an emergency BAS is increased by FO:TSL <0.5 and a fixed appearance of the flap valve. Hypermobile and/or aneurysmal atrial septum did not indicate inadequate postnatal mixing in our group.

The Effects of Hemodynamic Alterations on Lung Volumes in Fetuses with Tetralogy of Fallot: An MRI Study.

This study assessed whether the presence of tetralogy of Fallot (TOF) affects fetal lung development and whether these fetuses are at risk of pulmonary hypoplasia (PH). Furthermore, we investigated whether the degree of the concomitant pulmonary valve (PV) stenosis or a stenosis in the branch pulmonary arteries correlates with the fetal lung volume. Lung volumetry was performed in 16 fetuses with TOF who underwent MRI between gestational weeks 21 and 35 and in 22 controls. Fetal biometric data and the diameters of the PVs were evaluated by ultrasound. PV and branch pulmonary artery diameters were standardized (z-scores), and fetal lung volume/fetal body weight (FLV/FBW) ratios (ml/g) were calculated. The mean FLV/FBW ratio (0.031 ± 0.009 ml/g) in the TOF group was statistically significantly lower than in the control group (0.041 ± 0.009 ml/g; P = 0.003). None of the fetuses with TOF met the criterion for PH. FLV did not correlate with the degree of PV stenosis, but rather with the presence of a significant stenosis in at least one branch pulmonary artery. The presence of TOF moderately affects fetal lung growth, which is apparently not dependent on the degree of the PV stenosis. However, only an additional stenosis in at least one branch pulmonary artery was associated with a small FLV, but not with PH. Thus, reduced pulmonary blood flow may be offset by additional factors, such as the ability to establish a sufficient collateral system and to alter structural vascular size and, thus, pulmonary vascular resistance.

Impact of pre–stage II hemodynamics and pulmonary artery anatomy on 12-month outcomes in the Pediatric Heart Network Single Ventricle Reconstruction trial

To compare the interstage cardiac catheterization hemodynamic and angiographic findings between shunt types for the Pediatric Heart Network Single Ventricle Reconstruction trial. The trial, which randomized subjects to a modified Blalock-Taussig shunt (MBTS) or right ventricle-to-pulmonary artery shunt (RVPAS) for the Norwood procedure, demonstrated the RVPAS was associated with a smaller pulmonary artery diameter but superior 12-month transplant-free survival. We analyzed the pre-stage II catheterization data for the trial subjects. The hemodynamic variables and shunt and pulmonary angiographic data were compared between shunt types; their association with 12-month transplant-free survival was also evaluated. Of 549 randomized subjects, 389 underwent pre-stage II catheterization. A smaller size, lower aortic and superior vena cava saturation, and higher ventricular end-diastolic pressure were associated with worse 12-month transplant-free survival. The MBTS group had a lower coronary perfusion pressure (27 vs 32 mm Hg; P<.001) and greater pulmonary blood flow/systemic blood flow ratio (1.1 vs 1.0, P=.009). A greater pulmonary blood flow/systemic blood flow ratio increased the risk of death or transplantation only in the RVPAS group (P=.01). The MBTS group had fewer shunt (14% vs 28%, P=.004) and severe left pulmonary artery (0.7% vs 9.2%, P=.003) stenoses, larger mid-main branch pulmonary artery diameters, and greater Nakata indexes (164 vs 134, P<.001). Compared with the RVPAS subjects, the MBTS subjects had more hemodynamic abnormalities related to shunt physiology, and the RVPAS subjects had more shunt or pulmonary obstruction of a severe degree and inferior pulmonary artery growth at pre-stage II catheterization. A lower body surface area, greater ventricular end-diastolic pressure, and lower superior vena cava saturation were associated with worse 12-month transplant-free survival.

Energy loss and coronary flow simulation following hybrid stage I palliation: a hypoplastic left heart computational fluid dynamic model

The theoretical differences in energy losses as well as coronary flow with different band sizes for branch pulmonary arteries (PA) in hypoplastic left heart syndrome (HLHS) remain unknown. Our objective was to develop a computational fluid dynamic model (CFD) to determine the energy losses and pulmonary-to-systemic flow rates. This study was done for three different PA band sizes. Three-dimensional computer models of the hybrid procedure were constructed using the standard commercial CFD softwares Fluent and Gambit. The computer models were controlled for bilateral PA reduction to 25% (restrictive), 50% (intermediate) and 75% (loose) of the native branch pulmonary artery diameter. Velocity and pressure data were calculated throughout the heart geometry using the finite volume numerical method. Coronary flow was measured simultaneously with each model. Wall shear stress and the ratio of pulmonary-to-systemic volume flow rates were calculated. Computer simulations were compared at fixed points utilizing echocardiographic and catheter-based metric dimensions. Restricting the PA band to a 25% diameter demonstrated the greatest energy loss. The 25% banding model produced an energy loss of 16.76% systolic and 24.91% diastolic vs loose banding at 7.36% systolic and 17.90% diastolic. Also, restrictive PA bands had greater coronary flow compared with loose PA bands (50.2 vs 41.9 ml/min). Shear stress ranged from 3.75 Pascals with restrictive PA banding to 2.84 Pascals with loose banding. Intermediate PA banding at 50% diameter achieved a Qp/Qs (closest to 1) at 1.46 systolic and 0.66 diastolic compared with loose or restrictive banding without excess energy loss. CFD provides a unique platform to simulate pressure, shear stress as well as energy losses of the hybrid procedure. PA banding at 50% provided a balanced pulmonary and systemic circulation with adequate coronary flow but without extra energy losses incurred.

Computed tomographic angiography in tetralogy of Fallot

Echocardiography is often inadequate for imaging tetralogy of Fallot, prompting cineangiography. This study prospectively evaluated multidetector computed tomographic angiography for preoperative evaluation of tetralogy of Fallot in 112 consecutive patients. Forty-eight had nonconfluent or hypoplastic pulmonary arteries (mean z-score, -2; range, -11.1-0.13) permitting only palliative or no surgery; 64 had adequate pulmonary artery anatomy (mean z-score, 0.59; range, -2.53-3.4) allowing total repair. The surgical data of 50 patients who underwent total correction were compared with transthoracic echocardiography and multidetector computed tomographic angiography findings. Multidetector computed tomographic angiography tended to reveal unsuspected collaterals and coronary abnormalities besides outlining the right ventricular outflow tract and pulmonary artery branches. The branch pulmonary artery diameter z-score was the most important determinant of surgical strategy, with the worst figures being associated with no surgical options or palliative surgery, and the best figures leading to corrective surgery. The mean radiation dose was 3.45 mSv. Multidetector computed tomographic angiography is a powerful supplement to echocardiography in the preoperative evaluation of tetralogy of Fallot.

Transhepatic approach for rehabilitation of stenosed pulmonary arteries

Transhepatic cardiac catheterization and intervention is used in selected cases in our institution. A retrospective review of transcatheter interventions for the pulmonary artery was conducted. Forty-five transhepatic procedures were performed. Thirteen involved intervention, to rehabilitate the branch pulmonary arteries. The median weight of the patients was 9.9 Kg ± 3.4. The patients' age ranged from eight months to 86 months (median 23 months). The largest sheath used was 7F. All the patients underwent success intervention with no complication related either to the transhepatic approach or the intervention. The branch pulmonary artery diameter increased from 4.5 ± 2.2 mm to 7 ± 3 mm. Most of the procedures were performed under conscious sedation / deep sedation protocol. Hemostasis was achieved in all patients by gradual sheath withdrawal, followed by application of upward pressure on the tract from the subcostal area. In the absence of patent femoral veins the transhepatic approach can be used to perform successful and safe interventions, to rehabilitate the pulmonary artery system. It may offer the additional advantage of using larger sheaths than would be felt appropriate for the femoral veins.

Risk factors for early pulmonary valve replacement after valve disruption in congenital pulmonary stenosis and tetralogy of Fallot

Congenital heart defects with a component of pulmonary stenosis are often palliated in childhood by disrupting the pulmonary valve, either by means of dilation or excision. It is unclear what factors affect a patient's ability to tolerate long-term pulmonary insufficiency before requiring pulmonary valve replacement. We analyze potential factors that are related to the interval between pulmonary valve disruption and pulmonary valve replacement. One hundred seven patients were analyzed. They had a congenital diagnosis of pulmonary stenosis or tetralogy of Fallot, had their first pulmonary valve replacement between 2002 and 2008, and had a known interval between pulmonary valve disruption and pulmonary valve replacement. The median age at the time of surgical intervention was 2 years for pulmonary valve disruption (range, 0-56 years) and 26 years for pulmonary valve replacement (range, 1-72 years). The median interval was 23 years (range, 0-51 years). Potential related factors were sex, race, initial diagnosis and procedure, age at pulmonary valve disruption, prior shunt operation, presence of branch pulmonary artery stenosis, and degree of pulmonary regurgitation. As determined by using univariate analysis, male patients had a shorter interval than female patients (median, 16 vs 26 years; P = .01), and African American patients had a shorter interval than white patients (median, 16 vs 25 years; P = .049). A significant correlation was also identified between age at the time of pulmonary valve disruption and the subsequent interval to pulmonary valve replacement. Overall, the interval tended to increase as age at disruption increased (P < .0001). Although the presence of branch pulmonary artery stenosis determined by the need for concomitant pulmonary arterioplasty was associated with a significantly shorter interval to pulmonary valve replacement (21 vs 24 years, P = .02), stenosis determined based on small branch pulmonary artery diameter was correlated to a prolonged interval to pulmonary valve replacement (P = .009). Initial diagnosis, prior palliative shunt operation, and degree of pulmonary regurgitation had no effect on the interval between pulmonary valve disruption and subsequent pulmonary valve replacement. As determined by using multivariate analysis, only male sex and small pulmonary artery diameter remained significant factors. Male sex appears to shorten the interval between pulmonary valve disruption and pulmonary valve replacement, whereas small branch pulmonary artery diameter appears to lengthen the interval. Knowing which factors are detrimental and which are protective might help identify patients who are prone to a more rapid progression of right heart failure from free pulmonary insufficiency, possibly steering them toward more frequent follow-up or more aggressive heart failure medical regimens.

Pulmonary Artery Diameters in Healthy Fetuses From 19 to 40 Weeks' Gestation

The purpose of this study was to construct nomograms for diameters of the fetal main pulmonary artery (MPA), right pulmonary artery (RPA), and left pulmonary artery (LPA) according to gestational age (GA) and estimated fetal weight (EFW). Between May 2005 and February 2006, MPA and branch artery diameters were prospectively estimated with ultrasonography in 220 healthy fetuses from 19 to 40 weeks' gestation and correlated with GA and EFW. Mean MPA, RPA, and LPA diameters ranged from 2.93, 1.71, and 1.66 mm, respectively, at 19 weeks to 9.23, 5.49, and 5.65 mm at 40 weeks. Linear regression yielded the following formulas for the expected diameters according to GA: MPA=-2.77 + 0.30 x GA; RPA=-1.71 + 0.18 x GA; and LPA=-1.95 + 0.19 x GA. The following formulas were obtained by logarithm distribution for the expected diameters according to EFW: MPA=-1.165 + 0.247 x log of estimated fetal weight (lnFW); RPA=-0.651 + 0.141 x lnFW; and LPA=-0.718 + 0.150 x lnFW. Nomograms of MPA and branch pulmonary artery diameters according to GA and EFW have been described, generating reference values.

Fetal pulmonary artery diameter measurements as a predictor of morbidity in antenatally diagnosed congenital diaphragmatic hernia: A prospective study

The objective of the study was to examine the size and growth of fetal pulmonary artery diameters in congenital diaphragmatic hernia and assess their correlation with postnatal survival and morbidity outcomes. Prospective antenatal echocardiographic examination of fetal branch pulmonary arteries were correlated with postnatal survival and respiratory morbidity in cases of congenital diaphragmatic hernia. Receiver operator curves were developed to assess their utilization as predictors of respiratory morbidity and survival. Twenty-one cases were diagnosed antenatally over 3 years. Fifteen subjects survived postnatally. The ipsilateral fetal branch pulmonary artery diameter was smaller throughout gestation in left-sided congenital diaphragmatic hernia (P = .008). The small left pulmonary diameter did not correlate with survival but correlated strongly with morbidity outcomes. Progressive ipsilateral fetal pulmonary artery hypoplasia was demonstrated throughout gestation in subjects who died or had severe morbidity. Fetal pulmonary artery diameter measurements correlate with respiratory morbidity in postnatal congenital diaphragmatic hernia and may assist with prediction of outcome. Survival has improved, possibly because of improved postnatal management of congenital diaphragmatic hernia, limiting this measurement in assessing survival.

P05.19: Constriction and complete occlusion of the ductus arteriosus associated with maternal use of acetaminophen

Objectives: 1) To establish normograms (using predicted interval formulas) of fetal pulmonary artery diameters and ratios as a function of fetal biometric parameters (FBP). Study design: Cross sectional study: Fetuses with the following criteria following fetal echocardiography were recruited for this study: 1) known gestational age based on LMP or early ultrasound, 2) unremarkable fetal echocardiogram study, 3) full fetal biometry, 4) singleton gestation, and 5) no major anomalies. Data of the following pulmonary artery (PA) parameters were taken if measured in each fetus at least three times: 1) Main PA (MPA) diameter, 2) Right branch PA diameter, 3) Left branch PA diameter and 4) Ascending Aorta. The following ratios were calculated: MPA/RPA, RPA/LPA, MPA/LPA and MPA/AO. Pearson coefficients (P) were calculated for each parameter versus the following FBP: 1) Biparietal diameter, 2) Head circumference, 3) Abdominal circumference (AC), 4) Femur length (FL), 5) Estimated fetal weight and 6) Calculated fetal gestational age (CFGA) based on biparietal diameter and femur length. Predicted interval normograms (95%tile confidence intervals) were developed using SPSS for fetal pulmonary artery diameters and ratios. Results: One hundred and fifty-five fetuses have been enrolled in this study. Gestational age range was 124–272 days. PA diameters were well correlated to all the FPB (all P > 0.85), suggesting a stable linear relationship. Arterial diameter ratios were stable across the gestational age range studied and appeared independent of FPB. Conclusions: Normograms (based on Predicted Intervals, 95% CI) for fetal PA diameters and ratios are established for a variety of FBP. In the absence of known gestational age, these normograms of fetal pulmonary artery diameters can be used reliably as a basis of comparison.

P05.18: Left ventricular aneurysm in a fetus complicated by arrhythmias

Objectives: 1) To establish normograms (using predicted interval formulas) of fetal pulmonary artery diameters and ratios as a function of fetal biometric parameters (FBP). Study design: Cross sectional study: Fetuses with the following criteria following fetal echocardiography were recruited for this study: 1) known gestational age based on LMP or early ultrasound, 2) unremarkable fetal echocardiogram study, 3) full fetal biometry, 4) singleton gestation, and 5) no major anomalies. Data of the following pulmonary artery (PA) parameters were taken if measured in each fetus at least three times: 1) Main PA (MPA) diameter, 2) Right branch PA diameter, 3) Left branch PA diameter and 4) Ascending Aorta. The following ratios were calculated: MPA/RPA, RPA/LPA, MPA/LPA and MPA/AO. Pearson coefficients (P) were calculated for each parameter versus the following FBP: 1) Biparietal diameter, 2) Head circumference, 3) Abdominal circumference (AC), 4) Femur length (FL), 5) Estimated fetal weight and 6) Calculated fetal gestational age (CFGA) based on biparietal diameter and femur length. Predicted interval normograms (95%tile confidence intervals) were developed using SPSS for fetal pulmonary artery diameters and ratios. Results: One hundred and fifty-five fetuses have been enrolled in this study. Gestational age range was 124–272 days. PA diameters were well correlated to all the FPB (all P > 0.85), suggesting a stable linear relationship. Arterial diameter ratios were stable across the gestational age range studied and appeared independent of FPB. Conclusions: Normograms (based on Predicted Intervals, 95% CI) for fetal PA diameters and ratios are established for a variety of FBP. In the absence of known gestational age, these normograms of fetal pulmonary artery diameters can be used reliably as a basis of comparison.

Echocardiographic predictors of aortopulmonary collaterals in infants with tetralogy of fallot and pulmonary atresia

ObjectivesThis study was designed to identify echocardiographic predictors of aortopulmonary collaterals (APCs) in infants with tetralogy of Fallot and pulmonary atresia (TOF/PA) and to prospectively validate these predictors. BackgroundIn patients with TOF/PA, routine preoperative diagnostic cardiac catheterization is usually performed to determine the presence and distribution of APCs and the anatomy of the branch pulmonary arteries. MethodsThe clinical and diagnostic imaging data of infants with TOF/PA treated between 1990 and 2000 were reviewed. Patients were classified into two groups based on cineangiographic findings: 1) no APCs (n = 34), and 2) ≥1 APCs (n = 59). Echocardiographic variables were examined for their ability to identify patients having ≥1 APCs. ResultsMedian branch pulmonary artery diameter Z scores were significantly larger in patients without APCs compared with those having ≥1 APCs: −0.56 versus −3.24 for the left pulmonary artery and −0.76 versus −3.46 for the right pulmonary artery (p < 0.001). The presence of a branch pulmonary artery diameter Z score ≤−2.5 was 88% sensitive and 100% specific for the presence of ≥1 APCs. Detection of APCs by color Doppler was 93% sensitive and 91% specific. A combination of branch pulmonary artery diameter Z score ≤−2.5 or patent ductus arteriosus diameter ≤2 mm was 97% sensitive and 100% specific for the presence of ≥1 APCs. The diagnostic accuracy of these echocardiographic variables was subsequently validated in a prospective study of 11 infants. ConclusionsEchocardiography is a sensitive and specific test for the detection of ≥1 APCs in infants with TOF/PA. These data can be used to select patients who can undergo complete repair of TOF/PA without further preoperative diagnostic imaging.

Prenatal diagnosis by echocardiogram and outcome of absent pulmonary valve syndrome

We reported echocardiographic findings and outcomes of fetuses with absent pulmonary valve syndrome diagnosed during fetal life. Cases were identified from a prospectively acquired computerized database of 18,308 pregnancies referred to a fetal cardiology center between January 1988 and July 2000. Twenty fetuses were identified with a median gestation of 23 weeks (range 18 to 36) at presentation. In 18 cases (90%), there was an associated ventricular septal defect. Eighteen cases (90%) had branch pulmonary artery diameters above the normal range. In four cases (20%), an arterial duct was present. A chromosome 22q11 deletion was identified in 2 of 9 cases (22%) in which this deletion was sought. There were 6 terminations of pregnancy (30%), 3 intrauterine deaths (15%), 5 neonatal deaths (25%), 3 infant deaths (15%), and 3 patients who did not die (15%). Ten of the 11 “liveborn” infants required early ventilation. The outcome of absent pulmonary valve symdrome diagnosed prenatally appears poor. The high morbidity and mortality is due to both cardiac disease and associated bronchomalacia.