Pulmonary vein stenosis: Severity and location predict survival after surgical repair Lo Rito, Mauro, MD; Gazzaz, Tamadhir, MD; Wilder, Travis J., MD ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
03/2016, Volume:
151, Issue:
3
Journal Article
Peer reviewed
Open access
Abstract Objectives Pulmonary vein characteristics that influence survival after repair of stenosis have not been defined. We sought to develop a predictive model relating postrepair survival to ...preoperative pulmonary vein characteristics on computed tomography and magnetic resonance imaging. Methods Patients who underwent pulmonary vein stenosis repair (1990-2012) with preoperative computed tomography and magnetic resonance imaging were reviewed. We measured pulmonary vein short and long cross-sectional diameters at the left atrial junction (downstream), vein bifurcation (upstream), and narrowest point, and calculated the total cross-sectional area indexed for body surface area. The relationship between pulmonary vein dimensions and survival was related via risk-adjusted parametric hazard analyses. Results Of 145 patients who underwent surgical repair, 31 had preoperative computed tomography and magnetic resonance imaging and were analyzed. Surgical repairs were sutureless (n = 30) or pericardial patch reconstruction (n = 1). Mean follow-up was 4.28 ± 4.2 years. In-hospital mortality was 9.7%; unadjusted survival was 75% ± 7%, 69% ± 8%, and 64% ± 7% at 1, 3, and 5 years, respectively. Median downstream total cross-sectional area indexed for body surface area was 163 mm2 /m2 , upstream total cross-sectional area indexed for body surface area was 263 mm2 /m2 , and total cross-sectional area indexed for body surface area at maximal stenosis, localized at the left atrial junction in approximately two thirds of patients, was 163 mm2 /m2 . Smaller upstream total cross-sectional area indexed for body surface area ( P = .030) and greater number of stenotic pulmonary veins ( P = .0069) were associated with increased early (<1 year) risk of death. Smaller downstream total cross-sectional area indexed for body surface area tended to be associated with a late risk of death ( P = .059). Conclusions Smaller upstream or downstream total cross-sectional area indexed for body surface area negatively influenced survival. Early survival seemed especially poor for patients with a greater number of stenotic veins and upstream pulmonary vein involvement. The total cross-sectional area indexed for body surface area measurements can help to inform prognosis and stratify patients for enrollment in clinical trials of agents directed at pulmonary vein pathology.
Objective Aortopulmonary collaterals are a frequent phenomenon in patients after bidirectional cavopulmonary connection. The aortopulmonary collateral flow volume can be quantified using cardiac ...magnetic resonance imaging. However, the significance of aortopulmonary collateral flow for the postoperative outcome after total cavopulmonary connection is unclear and was sought to be determined. Methods The data from 33 patients were prospectively studied with cardiac magnetic resonance, echocardiography, and cardiac catheterization before the total cavopulmonary connection operation. The early postoperative outcomes after total cavopulmonary connection completion were recorded. Results Aortopulmonary collateral flow was 1.59 L/min/m2 ± 0.65 L/min/m2 (range, 0.54 L/min/m2 –3.34 L/min/m2 ), constituting 43% ± 13% (range, 12–87%) of pulmonary blood flow and 35% ± 12% (range, 11–62%) of the cardiac index, resulting in a pulmonary blood flow/systemic blood flow ratio of 1.06 ± 0.17 (range, 0.79–1.55). The aortopulmonary collateral flow correlated with pulmonary blood flow/systemic blood flow ratio ( r = 0.69, P < .0001), oxygen saturation ( r = 0.42, P = .018), and cardiac index ( r = 0.53, P = .002). Of the 36 patients, 24 underwent fenestrated total cavopulmonary connection during the study period. The aortopulmonary collateral flow, relative to the cardiac index, correlated with the duration of hospital stay ( r = 0.48, P = .02) and pleural drainage ( r = 0.45, P = .03). Patients whose pleural drainage lasted 1 week or less had less aortopulmonary collateral flow before the Fontan operation than those with a longer period until chest tube removal (1.23 L/min/m2 ± 0.38 L/min/m2 vs 1.73 L/min/m2 ± 0.76 L/min/m2 ; P = .03). Compared with a contemporary group of total cavopulmonary connection patients with fenestration in their extracardiac conduit who were studied prospectively, with a similar protocol, the bidirectional cavopulmonary connection had a greater amount of aortopulmonary collateral flow (1.59 L/min/m2 ± 0.65 L/min/m2 vs 1.30 L/min/m2 ± 0.57 L/min/m2 , P = .04). Conclusions Patients after bidirectional cavopulmonary connection routinely acquire a large amount of aortopulmonary collateral flow. The hemodynamic consequences of aortopulmonary collateral flow translate into adverse outcomes early after total cavopulmonary connection completion.
Background Late-onset intrauterine growth restriction (IUGR) results from a failure of the placenta to supply adequate nutrients and oxygen to the rapidly growing late-gestation fetus. Limitations in ...current monitoring methods present the need for additional techniques for more accurate diagnosis of IUGR in utero. New magnetic resonance imaging (MRI) technology now provides a noninvasive technique for fetal hemodynamic assessment, which could provide additional information over conventional Doppler methods. Objective The objective of the study was to use new MRI techniques to measure hemodynamic parameters and brain growth in late-onset IUGR fetuses. Study Design This was a prospective observational case control study to compare the flow and T2 of blood in the major fetal vessels and brain imaging findings using MRI. Indexed fetal oxygen delivery and consumption were calculated. Middle cerebral artery and umbilical artery pulsatility indexes and cerebroplacental ratio were acquired using ultrasound. A score of ≥ 2 of the 4 following parameters defined IUGR: (1) birthweight the third centile or less or 20% or greater drop in the centile in estimated fetal weight; (2) lowest cerebroplacental ratio after 30 weeks less than the fifth centile; (3) ponderal index < 2.2; and (4) placental histology meets predefined criteria for placental underperfusion. Measurements were compared between the 2 groups (Student t test) and correlations between parameters were analyzed (Pearson’s correlation). MRI measurements were compared with Doppler parameters for identifying IUGR defined by postnatal criteria (birthweight, placental histology, ponderal index) using receiver-operating characteristic curves. Results We studied 14 IUGR and 26 non-IUGR fetuses at 35 weeks’ gestation. IUGR fetuses had lower umbilical vein ( P = .004) and pulmonary blood flow ( P = .01) and higher superior vena caval flow ( P < .0001) by MRI. IUGR fetuses had asymmetric growth but smaller brains than normal fetuses ( P < .0001). Newborns with IUGR also had smaller brains with otherwise essentially normal findings on MRI. Vessel T2s, oxygen delivery, oxygen consumption, middle cerebral artery pulsatility index, and cerebroplacental ratio were all significantly lower in IUGR fetuses, whereas there was no significant difference in umbilical artery pulsatility index. IUGR score correlated positively with superior vena caval flow and inversely with oxygen delivery, oxygen consumption, umbilical vein T2, and cerebroplacental ratio. Receiver-operating characteristic curves revealed equivalent performance of MRI and Doppler techniques in identifying IUGR that was defined based on postnatal parameters with superior vena caval flow area under the curve of 0.94 (95% confidence interval, 0.87–1.00) vs a cerebroplacental ratio area under the curve of 0.80 (95% confidence interval, 0.64–0.97). Conclusion MRI revealed the expected circulatory redistribution in response to hypoxia in IUGR fetuses. The reduced oxygen delivery in IUGR fetuses indicated impaired placental oxygen transport, whereas reduced oxygen consumption presumably reflected metabolic adaptation to diminished substrate delivery, resulting in slower fetal growth. Despite brain sparing, placental insufficiency limits fetal brain growth. Superior vena caval flow and umbilical vein T2 by MRI may be useful new markers of late-onset IUGR.
We hypothesized that primary sutureless (SL) repair of total anomalous pulmonary venous drainage (TAPVD) may have a lower incidence of postrepair pulmonary vein obstruction (PVO) and different modes ...of PVO compared with standard repair (SR).
One hundred ninety-five patients who underwent TAPVD repair (1990 to 2012) with the exception of congenital pulmonary vein stenosis, isomerism, and single-ventricle anomalies were included. Survival, reintervention, incidence, degree of PVO were compared between groups. The mode of PVO was expressed as central or peripheral. The Mann-Whitney test, Kaplan-Meier analysis, and Cox regression were used.
The SL group had more infracardiac or mixed TAPVD (p = 0.02) and preoperative PVO (p = 0.07). There were no differences between SR and SL groups in survival (5-year survival, 83.1% versus 82.5%, respectively; p = 0.73) and composite outcome (death, intervention, PVO, 5-year survival, 76.4% versus 80.7%, respectively; p = 0.225). The SL group had a lower incidence of PVO of moderate or greater degree (SR, 11.3% versus SL, 2.9%; p = 0.05) than the SR group, especially in the infracardiac and mixed TAPVD cohort (p = 0.011), with a lower pulmonary vein score (SR, 8 versus SL, 4; p = 0.01). The SL group had peripheral PVO exclusively (100%), whereas the SR group predominantly had central PVO (76.4%; p = 0.005). There was a trend toward less reoperation in the SL group (SR, 10.4% versus SL, 2.9%; p = 0.08). Survival after reoperation was comparable to primary TAPVD repair types as well as reoperation repair types.
Primary SL appeared to be associated with a lower incidence and severity of PVO. The primary SL repair eliminated the risk of developing central PVO, although a relatively benign type of peripheral PVO could occur.
BACKGROUND Pulmonary venoocclusive disease (PVOD) is a rare lung disease, diagnosed in 5% to 10% of patients with pulmonary hypertension (PH). The incidence, prevalence, and etiology of PVOD in ...children are not well defined. The mortality remains high, related, at least partly, to the limited treatment options. METHODS This retrospective analysis (1985-2011) summarizes symptoms, associated factors, treatment, and outcomes of nine pediatric patients (five girls, four boys) with histologic confirmation of PVOD. RESULTS PH was diagnosed at a mean age of 13.5 years (range, 8-16 years), followed by the definitive diagnosis of PVOD at a mean age of 14.3 years (range, 10-16 years). Symptoms such as decreased exercise tolerance (n = 6) and/or shortness of breath (n = 9) preceded the diagnosis by 21 months on average; the mean survival time after diagnosis was 14 months (range, 0-47 months). CT scans of the lungs showed typical radiologic features. Treatment included supplemental home oxygen (n = 5), diuretics (n = 9), warfarin (n = 4), and pulmonary vasodilators (n = 4). Four children were listed for lung transplantation, and three have undergone transplantation. Eight patients died, including two after lung transplantation. One patient with lung transplant survived with good quality of life. CONCLUSIONS PVOD is an important differential diagnosis for pediatric patients with PH. CT scanning is a valuable tool to image lung abnormalities; the definitive diagnosis can only be made by examination of lung biopsy specimens, which subjects the patient to additional risk. Early listing for lung transplantation is essential, as the mean survival time is only 14 months.
Objective The management of patients with tetralogy of Fallot (ToF) and pulmonary atresia (PA) with major aortopulmonary collateral arteries (MAPCAs) is challenging. Frequently it is difficult to ...predict whether complete repair with closure of the ventricular septal defect (VSD) will be tolerated. The aim of this study was to investigate whether measurements of preoperative pulmonary blood flow are associated with early postoperative outcome after VSD closure. Methods In this retrospective study, the data from 10 patients who had undergone a cardiac magnetic resonance imaging study with flow measurements before attempted surgical complete repair were collected. Systemic blood flow (Qs) was calculated as the sum of descending aortic blood flow distal to the MAPCA origins and superior vena cava flow. Pulmonary blood flow (Qp) was measured either from the sum of the pulmonary flow (n = 7) or calculated as the difference between ascending aortic flow and Qs. Results Preoperative Qp/Qs averaged 1.71 ± 0.68 and correlated inversely with right ventricular systolic pressure (relative to systolic blood pressure, r = −0.75, P = .03) and positively with the total neopulmonary artery index (TNPAI, r = 0.66, P = .04), as measured by fluoroscopic angiography. Two children did not tolerate VSD closure. This was likely related to bronchial compression in 1 patient while the other had the lowest TNPAI and the lowest Qp/Qs of all patients. Conclusions CMR provides not only anatomic but also functional information for surgical decision making in patients with ToF and PA with MAPCAs. Preoperative Qp/Qs is associated with postoperative right ventricular pressure and may be a marker of readiness for VSD closure.
Assessment of right ventricular (RV) volumes and function is important in patients after tetralogy of Fallot (TOF) repair. Currently, cardiac magnetic resonance imaging (MRI) is considered the ...clinical reference method for RV volume and function measurements. Three-dimensional (3D) knowledge-based reconstruction derived from two-dimensional echocardiographic imaging with magnetic tracking is a novel approach to RV volumetrics. The aim of this study was to assess the feasibility and reliability of this novel echocardiographic technique in patients after TOF repair. The accuracy of the method was assessed by comparison with measurements obtained by cardiac MRI.
Thirty patients (mean age, 13.7 ± 2.8 years) after TOF repair, referred for cardiac MRI, were included. Immediately after MRI, echocardiographic image acquisition was performed using a standard ultrasound scanner linked to a Ventripoint Medical Systems unit. Echocardiographic and MRI measurements were performed offline. Parameters analyzed were end-diastolic volume (EDV), end-systolic volume (ESV), and ejection fraction. Intraobserver, interobserver, and intertechnique variability was assessed using Pearson's correlation analysis, coefficients of variation, and Bland-Altman analysis.
Echocardiographic two-dimensionally based 3D reconstruction was highly feasible, with low intraobserver and interobserver variability for EDV and slightly higher variability for ESV and ejection fraction. The 3D reconstruction values for EDV, ESV, and ejection fraction were correlated highly with MRI values, with low coefficients of variation. The agreement between both methods was high. Three-dimensional reconstruction slightly underestimated RV volumes, by 2.5% for EDV and 4.6% for ESV compared with MRI volumes.
In patients after TOF repair, echocardiographic 3D reconstruction is highly feasible, with good reproducibility for measurements of RV EDV. There is good agreement with MRI measurements, with a small underestimation of RV volumes. The use of this method in clinical practice warrants further investigation.
The purpose of this study was to determine the phenotypic presentation, causes, and outcome of fetal cardiomyopathy (CM) and to identify early predictors of outcome.
Although prenatal diagnosis is ...possible, there is a paucity of information about fetal CM.
This was a retrospective review of 61 consecutive fetal cases with a diagnosis of CM at a single center between 2000 and 2012.
Nonhypertrophic CM (NHCM) was diagnosed in 40 and hypertrophic CM (HCM) in 21 fetuses at 24.7 ± 5.7 gestational weeks. Etiologies included familial (13%), inflammatory (15%), and genetic-metabolic (28%) disorders, whereas 44% were idiopathic. The pregnancy was terminated in 13 of 61 cases (21%). Transplantation-free survival from diagnosis to 1 month and 1 year of life for actively managed patients was better in those with NHCM (n = 31; 58% and 58%, respectively) compared with those with HCM (n = 17; 35% and 18%, respectively; hazard ratio HR: 0.44; 95% confidence interval CI: 0.12 to 0.72; p = 0.007). Baseline echocardiographic variables associated with mortality in actively managed patients included ventricular septal thickness (HR: 1.21 per z-score increment; 95% CI: 1.07 to 1.36; p = 0.002), cardiothoracic area ratio (HR: 1.06 per percent increment; 95% CI: 1.02 to 1.10; p = 0.006), ≥3 abnormal diastolic Doppler flow indexes (HR: 1.44; 95% CI: 1.07 to 1.95; p = 0.02), gestational age at CM diagnosis (HR: 0.91 per week increment; 95% CI: 0.83 to 0.99; p = 0.03), and, for fetuses in sinus rhythm, a lower cardiovascular profile score (HR: 1.45 per point decrease; 95% CI: 1.16 to 1.79; p = 0.001).
Fetal CM originates from a broad spectrum of etiologies and is associated with substantial mortality. Early echocardiographic findings appear useful in predicting adverse perinatal outcomes.
Objectives The aim of this study was to report the results of an international multicenter experience of cardiac magnetic resonance imaging (MRI) evaluation of cardiac tumors in children, each with ...histology correlation or a diagnosis of tuberous sclerosis, and to determine which characteristics are predictive of tumor type. Background Individual centers have relatively little experience with diagnostic imaging of cardiac tumors in children, because of their low prevalence. The accuracy of cardiac MRI diagnosis on the basis of a pre-defined set of criteria has not been tested. Methods An international group of pediatric cardiac imaging centers was solicited for case contribution. Inclusion criteria comprised: 1) age at diagnosis ≤18 years; 2) cardiac MRI evaluation of cardiac tumor; and 3) histologic diagnosis or diagnosis of tuberous sclerosis. Data from the cardiac MRI images were analyzed for mass characteristics. On the basis of pre-defined cardiac MRI criteria derived from published data, 3 blinded investigators determined tumor type, and their consensus diagnoses were compared with histologic diagnoses. Results Cases (n = 78) submitted from 15 centers in 4 countries had the following diagnoses: fibroma (n = 30), rhabdomyoma (n = 14), malignant tumor (n = 12), hemangioma (n = 9), thrombus (n = 4), myxoma (n = 3), teratoma (n = 2), and paraganglioma, pericardial cyst, Purkinje cell tumor, and papillary fibroelastoma (n = 1, each). Reviewers who were blinded to the histologic diagnoses correctly diagnosed 97% of the cases but included a differential diagnosis in 42%. Better image quality grade and more complete examination were associated with higher diagnostic accuracy. Conclusions Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.
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