Diagnosis of functionally significant coronary stenosis with exercise CT myocardial perfusion imaging.

PURPOSE: To assess the feasibility of exercise perfusion computed tomography (CT) in patients suspected of having hemodynamically significant coronary stenosis. MATERIALS AND METHODS: This study had institutional review board approval, and all patients gave informed consent. Thirty-two consecutive patients (26 men [mean age, 63 years] and six women [mean age, 71 years]) with 55 coronary stenoses of at least 50% underwent coronary CT angiography (one stenosis in 13 patients, two stenoses in 15 patients, and three stenoses in four patients). CT myocardial perfusion imaging was performed within 1 minute after patients performed supine exercise on an ergometer secured to the CT table. The pressure-rate product was computed to assess level of exercise. The myocardial enhancement ratio between stenotic and normally perfused territories was determined for each stenosis. Fractional flow reserve less than 0.8, as measured during invasive coronary angiography, was the reference for defining significant stenoses. Receiver operating characteristic curves were constructed to determine the myocardial enhancement ratio cutoff value. RESULTS: In the per-patient analysis, a myocardial enhancement ratio cutoff of 0.8 performed best for identifying functionally significant stenosis: Sensitivity was 95% (21 of 22 patients), specificity was 90% (nine of 10 patients), positive predictive value was 95% (21 of 22 patients), negative predictive value was 90% (nine of 10 patients), and accuracy was 94% (30 of 32 patients). Corresponding values in the per-stenosis analysis were 97% (29 of 30 stenoses), 96% (23 of 24 stenoses), 97% (29 of 30 stenoses), 96% (23 of 24 stenoses), and 96% (52 of 54 stenoses), respectively. CONCLUSION: Exercise CT myocardial perfusion imaging is feasible and accurate for assessment of the functional significance of coronary stenosis.

Critical Congenital Heart Diseases in Preterm Neonates: Is Early Cardiac Surgery Quite Reasonable?

Prematurity is a recognized risk factor for morbidity and mortality following cardiac surgery. Postoperative and long-term outcomes after cardiac surgery performed in the preterm period are poorly described. The aim of this study was to analyze a population of preterm neonates operated on for critical congenital heart disease (CHD) before 37 weeks of gestational age (wGA) with special attention given to early and late mortality and morbidity. Between 2000 and 2013, 28 preterm neonates (median gestational age (GA) 34.3 weeks) underwent cardiopulmonary bypass (CPB) surgery for critical CHD before 37 wGA; records were retrospectively reviewed. All patients except three with single ventricle physiology had a single-stage anatomic repair. Overall mortality was 43 % (95 % CI 25-62). Risk factors for death were birth weight (p = 0.032) and weight at surgery (p = 0.037), independently of GA, preoperative status, CPB and aortic clamp time. Seven patients, including those with univentricular hearts, died during the postoperative period, and five in the first year after surgery. Median follow-up was 5.9 years (range 1 month-12.8 years). Kaplan-Meier survival rate was 75 % (95 % CI 59-91) at 1 month, and 57 % (95 % CI 39-75) at 1 and 5 years. Eight patients required reoperations after a delay of 2.8 ± 1.3 months; eight had bronchopulmonary dysplasia. At the end of follow-up, nine patients were asymptomatic. One-stage biventricular repair for critical CHD on preterm neonates was feasible. Mortality remained high but acceptable, mainly confined to the first postoperative year and related to small weight. Despite reoperations, long-term clinical status was good in most survivors. Further long-term prospective investigations are necessary to evaluate neurodevelopmental outcomes.

All-age relationship between arm span and height in different ethnic groups.

The objective of the present study was to establish multiethnic, all-age prediction equations for estimating stature from arm span in males and females. The arm span/height ratio (ASHR) from 13 947 subjects (40.9% females), aged 5-99 years, from nine centres (in China, Europe, Ghana, India and Iran) was used to predict ASHR as a function of age using the lambda, mu and sigma method. Z-scores for forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC) and FEV1/FVC in 1503 patients were calculated using measured height and height calculated from arm span and age. ASHR varied nonlinearly with age, was higher in males than in females and differed significantly between the nine sites. The data clustered into four groups: Asia, Europe, Ghana and Iran. Average predicted FEV1, FVC and FEV1/FVC using measured or predicted height did not differ, with standard deviations of 4.6% for FEV1, 5.0% for FVC and 0.3% for FEV1/FVC. The percentages of disparate findings for a low FEV1, FVC and FEV1/FVC in patients, calculated using measured or predicted height, were 4.2%, 3.2% and 0.4%, respectively; for a restrictive pattern, there were 1.0% disparate findings. Group- and sex-specific equations for estimating height from arm span and age to derive predicted values for spirometry are clinically useful.

The pattern of the coronary arterial orifices in hearts with congenital malformations of the outflow tracts: a marker of rotation of the outflow tract during cardiac development?

Outflow tract defects, including cardiac neural crest defects (so-called conotruncal defects) and transposition of the great arteries, are due to an abnormal rotation of the outflow tract during cardiac development. Coronary orifices are often abnormal in outflow tract defects, particularly in common arterial trunk (CAT). A recent study indicates that abnormal coronary artery pattern in a mouse model with common arterial outlet (Tbx1-/- mouse mutant) could be due to a reduced and malpositioned subpulmonary coronary-refractory myocardial domain. The aim of our study was to demonstrate the relation between coronary orifices pattern in outflow tract defects in human and the abnormal embryonic rotation of the outflow tract. We analyzed 101 heart specimens with outflow tract defects: 46 CAT, 15 tetralogy of Fallot (TOF), 29 TOF with pulmonary atresia (TOF-PA), 11 double-outlet right ventricle with subaortic ventricular septal defect (DORV) and 17 controls. The position of left and right coronary orifices (LCO, RCO) was measured in degrees on the aortic/truncal circumference. The anterior angle between LCO and RCO (α) was calculated. The LCO was more posterior in TOF (31 °), TOF-PA (47 °), DORV (44 °), CAT (63 °), compared with controls (0 °, P < 0.05), and more posterior in CAT than in other outflow tract defects (P < 0.05). The RCO was more anterior in TOF (242 °), TOF-PA (245 °) and DORV (271 °) than in controls (213 °, P < 0.05), but not in CAT (195 °). The α angle was similar in TOF, TOF-PA, DORV and controls (149 °, 162 °, 133 °, 147 °), but significantly larger in CAT (229 °, P < 0.0001). In all outflow tract defects but CAT, the displacement of LCO (anterior) and RCO (posterior), while the α angle remains constant, might be due to incomplete rotation of the myocardium at the base of the outflow tract, leading to an abnormally positioned subpulmonary coronary-refractory myocardial domain. The larger α angle in CAT could reflect its dual identity, aortic and pulmonary.

Multidetector row computed tomography to assess changes in airways linked to asthma control.

BACKGROUND: In asthma, multidetector row computed tomography (MDCT) detects abnormalities that are related to disease severity, including increased bronchial wall thickness. However, whether these abnormalities could be related to asthma control has not been investigated yet. OBJECTIVE: Our goal was to determine which changes in airways could be linked to disease control. METHODS: Twelve patients with poor asthma control were included and received a salmeterol/fluticasone propionate combination daily for 12 weeks. Patients underwent clinical, functional, and MDCT examinations before and after the treatment period. MDCT examinations were performed using a low-dose protocol at a controlled lung volume (65% TLC). Bronchial lumen (LA) and wall areas (WA) were evaluated at a segmental and subsegmental level using BronCare software. Lung density was measured at the base of the lung. Baseline and end-of-treatment data were compared using the Wilcoxon signed-rank test. RESULTS: After the 12-week treatment period, asthma control was achieved. Airflow obstruction and air trapping decreased as assessed by the changes in FEV(1) (p < 0.01) and expiratory reserve volume (p < 0.01). Conversely, LA and WA did not vary significantly. However, a median decrease in LA of >10% was observed in half of the patients with a wide intra- and intersubject response heterogeneity. This was concomitant with a decrease in lung density (p < 0.02 in the anteroinferior areas). CONCLUSIONS: MDCT is insensitive for demonstrating any decrease in bronchial wall thickness. This is mainly due to changes in bronchial caliber which may be linked to modifications of the elastic properties of the bronchopulmonary system under treatment.

Robustness of end-systolic reconstructions in coronary dual-source CT angiography for high heart rate patients.

OBJECTIVES: Reconstruction of cardiac computed tomography (CT) images is challenging when the heart rate is higher than 65 beats per minute (bpm). The optimal reconstruction time is often found to be at the end-systolic phase, but image quality remains uncertain. Using dual-source (DS) CT and 83-ms temporal resolution, we evaluated the robustness of the temporal window with low motion during the end systole. METHODS: We studied 41 DSCT in consecutive patients with a heart rate >65 bpm. Eleven systolic reconstructions were performed every 20 ms between 200 ms and 400 ms of the R-R interval. The end-systolic temporal window (ESTW) was defined as the interval between the first and last selected phases judged adequate for diagnosis. RESULTS: Heart rates varied from 67 to 150 bpm. ESTW was always to be found greater than 100 ms. The mean ESTW was 178 ms (SD: 57 ms), and varied independently of heart rate. All data sets achieved diagnostic quality during the end-systolic phase at a time point between 35 and 50% of the R-R interval. CONCLUSION: Our data suggest that CT with a temporal window below 100 ms may provide acceptable systolic reconstructions at any heart rate, in a large proportion of patients.

Variability of bronchial measurements obtained by sequential CT using two computer-based methods.

This study aimed to evaluate the variability of lumen (LA) and wall area (WA) measurements obtained on two successive MDCT acquisitions using energy-driven contour estimation (EDCE) and full width at half maximum (FWHM) approaches. Both methods were applied to a database of segmental and subsegmental bronchi with LA > 4 mm(2) containing 42 bronchial segments of 10 successive slices that best matched on each acquisition. For both methods, the 95% confidence interval between repeated MDCT was between -1.59 and 1.5 mm(2) for LA, and -3.31 and 2.96 mm(2) for WA. The values of the coefficient of measurement variation (CV(10), i.e., percentage ratio of the standard deviation obtained from the 10 successive slices to their mean value) were strongly correlated between repeated MDCT data acquisitions (r > 0.72; p < 0.0001). Compared with FWHM, LA values obtained using EDCE were higher for LA < 15 mm(2), whereas WA values were lower for bronchi with WA < 13 mm(2); no systematic EDCE underestimation or overestimation was observed for thicker-walled bronchi. In conclusion, variability between CT examinations and assessment techniques may impair measurements. Therefore, new parameters such as CV(10) need to be investigated to study bronchial remodeling. Finally, EDCE and FWHM are not interchangeable in longitudinal studies.

Non-invasive diagnosis of ischaemic heart failure using 64-slice computed tomography.

AIMS: We evaluated the accuracy of 64-slice computed tomography (CT) to identify ischaemic aetiology of heart failure (IHF). METHODS AND RESULTS: Ninety-three consecutive patients in sinus rhythm with dilated cardiomyopathy but without suspicion of coronary artery disease (CAD) were enrolled when admitted for angiography. Accuracy of CT to detect significant stenosis (>50% lumen narrowing) was compared with quantitative coronary angiography. IHF was defined as a significant stenosis on left main or proximal left anterior descending artery or two or more vessels. Forty-three out of 1395 segments (3%) were heavily calcified and excluded. CT correctly assessed 103 of 142 (73%) significant stenosis and identified 46 of 50 (92%) patients without and 42 of 43 (98%) patients with CAD, 60 of 62 (97%) patients without and 28 of 31 (90%) patients with IHF. Overall, accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of CT for identifying CAD by segment was 96, 73, 99, 92, and 97%, respectively; by patient was 95, 98, 92, 91, and 98%, respectively; and for identifying IHF was 95, 90, 97, 93, and 95%, respectively. CONCLUSION: Non-invasive 64-slice CT assessment of the extent of CAD may offer a valid alternative to angiography for the diagnosis of IHF.

Comparison of 64-slice computed tomography planimetry and Doppler echocardiography in the assessment of aortic valve stenosis.

BACKGROUND AND AIM OF THE STUDY: The study aim was to compare, prospectively, the planimetry of aortic stenosis on 64-slice computed tomography (CT), with the area calculated by Doppler transthoracic echocardiography (TTE) in symptomatic patients evaluated before potential aortic valve replacement. METHODS: Fifty-two consecutive patients (27 males, 25 females; mean age 74 +/- 10 years) admitted to the authors' institution during 2005 were evaluated with 64-slice CT and Doppler TTE. The time interval between the two evaluations was 2 +/- 1 weeks. Planimetry of the anatomic orifice area (AOA) drawn on 64-slice CT was compared to the effective area determined by Doppler TTE by Bland and Altman analysis, and the anatomic area threshold value corresponding to a significant effective aortic stenosis (50.75 cm2) was determined by receiver operating characteristic (ROC) analysis. RESULTS: The aortic orifice area measured by 64-slice CT correlated well with the effective area (r = 0.76; p <0.0001), but was significantly greater, with a systematic overestimation (0.132 cm(2)) and a variability of 0.239 cm(2). There was good agreement between planimetry determined by two independent radiologists (difference = 0.002, variability = 0.115 cm(2)). ROC analysis showed that a threshold value of 0.95 cm(2) as measured by 64-slice CT planimetry identifies significant aortic stenosis with sensitivity, specificity, accuracy, positive and negative predictive values of 82%, 77%, 81%, 91% and 59%, respectively. CONCLUSION: 64-slice CT is a reproducible and reliable non-invasive method to evaluate aortic valve stenosis compared to the reference method of Doppler TTE. Indeed, the CT approach could replace the latter evaluation when measurements used in the continuity equation are inadequate.

Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome.

BACKGROUND: The long-term effectiveness of pulmonary arterial hypertension-specific drug therapy (PAH-SDT) in Eisenmenger syndrome is controversial. We investigated short-term and long-term hemodynamic changes under PAH-SDT and their associations with outcomes in a bicentric cohort. METHODS: Over 20 years, we included 69 patients with congenital heart disease, an indexed pulmonary vascular resistance (PVRi) >8 WU·m2, and 292 standardized catheterizations at baseline and after PAH-SDT initiation or intensification. Oxygen consumption was measured and the Fick principle applied to calculate indexed pulmonary output (Qpi) and PVRi. RESULTS: After PAH-SDT initiation or intensification, median (interquartile range) PVRi decrease was 5.1 WU·m2 (-1.4, -12.6) (p < 0.0001). Median Qpi and 6-minute walk test increases were +0.4 liter/min/m2 (0.0, +0.9) (p < 0.0001) and +49 m (+15, +93) (p = 0.0003), respectively. Hemodynamic response combining increased Qpi with decreases in transpulmonary gradient and PVRi occurred in 68.0% of patients. After a median of 4.9 years, PVRi and Qpi changes were no longer significant. Over a median of 7.2 years, 23 (33.3%) patients met a composite criterion (death, n = 8; heart-lung transplantation or listing for transplantation, n = 15). The 15-year cumulative event rate was 49.2%. By multivariate analysis, independent predictors of events were superior vena cava oxygen saturation and hemodynamic response (p = 0.048 and p < 0.0001). CONCLUSIONS: In Eisenmenger syndrome, PAH-SDT induces early hemodynamic improvements, which decline over time. Hemodynamic changes under PAH-SDT vary across patients. Hemodynamic parameters at baseline and under PAH-SDT are associated with events. PAH-SDT may need to be individualized based on hemodynamic changes.

Standard and Strain Measurements by Echocardiography Detect Early Overloaded Right Ventricular Dysfunction: Validation against Hemodynamic and Myocyte Contractility Changes in a Large Animal Model.

BACKGROUND: Early detection of right ventricular (RV) failure is required to improve the management of patients with congenital heart diseases. The aim of this study was to validate echocardiography for the early detection of overloaded RV dysfunction, compared with hemodynamic and myocyte contractility assessment. METHODS: Using a porcine model reproducing repaired tetralogy of Fallot, RV function was evaluated over 4 months using standard echocardiography and speckle-tracking compared with hemodynamic parameters (conductance catheter). Sarcomere shortening and calcium transients were recorded in RV isolated myocytes. Contractile reserve (ΔEmax) was assessed by ß-adrenergic stimulation in vivo (dobutamine 5 µg/kg) and ex vivo (isoproterenol 100 nM). RESULTS: Six operated animals were compared with four age- and sex-matched controls. In the operated group, hemodynamic RV efficient ejection fraction was significantly decreased (29.7% [26.2%-34%] vs 42.9% [40.7%-48.6%], P < .01), and inotropic responses to dobutamine were attenuated (ΔEmax was 51% vs 193%, P < .05). Echocardiographic measurements of fraction of area change, tricuspid annular plane systolic excursion, tricuspid annular peak systolic velocity (S') and RV free wall longitudinal systolic strain and strain rate were significantly decreased. Strain rate, S', and tricuspid annular plane systolic excursion were correlated with ΔEmax (r = 0.75, r = 0.78, and r = 0.65, respectively, P < .05). These alterations were associated in RV isolated myocytes with the decrease of sarcomere shortening in response to isoproterenol and perturbations of calcium homeostasis assessed by the increase of spontaneous calcium waves. CONCLUSIONS: In this porcine model, both standard and strain echocardiographic parameters detected early impairments of RV function and cardiac reserve, which were associated with cardiomyocyte excitation-contraction coupling alterations.

The mid-term outcome of primary open valvotomy for critical aortic stenosis in early infancy - a retrospective single center study over 18 years.

BACKGROUND: The objective of this study was to examine early and long-term results of surgical aortic valvotomy in neonates and infants aged less than four months and to identify predictors of outcome. METHODS: Between August 1994 and April 2012, 83 consecutive patients younger than 4 months of age underwent open heart valvotomy for critical aortic stenosis in our institution. Median age was 17 days (range 0-111 days). We examined clinical records to establish determinants of outcome and illustrate long-term results. RESULTS: Fifty-six patients (67 %) were neonates. Associated cardiac malformations were found in 24 patients (29 %), including multilevel left heart obstruction in 5. The median follow-up was 4.2 years. The time-related survival rate was 87 and 85 % at 5 and 15 years, respectively. The time-related survival without reintervention was respectively 51, 35 and 18 % at 5, 10 and 15 years. The time-related survival without aortic valve replacement was respectively 67, 54 and 39 % at 5, 10 and 15 years. Ventricular dysfunction (p = 0.04), delayed sternal closure (p = 0.007), endocardial fibroelastosis (p = 0.02) and low z-score of the aortic annulus (p = 0.04) were found predictors of global mortality. Ventricular dysfunction (p = 0.01) and endocardial fibroelastosis (p = 0.04) were found predictors of reintervention. CONCLUSIONS: The experience, in our center, on the management of critical aortic stenosis, shows a low early and late mortality, but the aortic valvotomy is a palliative procedure and we see unfortunately a high rate of reintervention among which the aortic valve replacement. These results suggest to reconsider the use of aortic balloon valvotomy, and particularly for the neonates with a low cardiac output in order to avoid the myocardial stress and the neurological injury due to the cardiopulmonary bypass.

Long-term outcomes of the arterial switch operation for transposition of the great arteries and ventricular septal defect and/or aortic arch obstruction.

OBJECTIVES: Long-term outcomes after the arterial switch operation (ASO) for complex transposition of the great arteries (TGA) should be clarified. METHODS: A retrospective study was conducted in patients operated on between 1982 and 1998. Overall 220 postoperative survivors, 79.1% with a ventricular septal defect, 13.2% with multiple ventricular septal defects, and 29.1% with aortic arch obstruction, were followed for 17 years (0-28 years). RESULTS: The conditional survival rate was 96.7% [95% confidence interval (CI): 94.4-99.1] at 25 years. Late sudden death occurred in 2 asymptomatic patients. The cumulative incidence rate of death or reinterventions was 3.8% (95% CI: 2.9-4.8) at 25 years, with age at ASO <10 days and aortic regurgitation at discharge identified as independent risk factors. The cumulative incidence rate of neoaortic regurgitation was 41.6% (95% CI: 20.5-62.8) at 25 years with an aorto-pulmonary diameter mismatch at the time of the ASO, age at ASO <10 days and aortic regurgitation at discharge identified as independent risk factors. At the last follow-up, 53 patients (24.1%) had neoaortic root dilatation with an aortic sinus z-score ≥3 and 6 of them had a Bentall operation at a median delay of 14.1 years since the ASO. The only independent factors for neoaortic root dilatation were male sex and an aorto-pulmonary diameter mismatch at the time of the ASO. CONCLUSIONS: Despite a continual rate of reinterventions, long-term survival and cardiovascular outcome are excellent after ASO for complex TGA. Dilatation of the neoaortic root and neoaortic regurgitation may be observed with time and 2 late sudden deaths occurred, justifying a close follow-up in all patients.

Moderate and chronic hemodynamic overload of sheep atria induces reversible cellular electrophysiologic abnormalities and atrial vulnerability.

OBJECTIVES: The aim of this study was to evaluate the myocardial consequences of a chronic volume overload of the left atrium (LA). BACKGROUND: Atrial dilation is a major risk factor for atrial fibrillation (AF), but the underlying mechanisms are poorly understood. METHODS: A left-right aorto-pulmonary artery shunt (APS) was created in sheep. The cardiopathy was characterized by echocardiography, electrophysiologic testing, and histologic analysis. Cellular action potential (AP) and calcium current (I(Ca)) were recorded by means of microelectrode and patch clamp techniques. RESULTS: Three to four months after surgery, all animals in the APS state had a dilated LA (146.2 +/- 35.4 cm(2)/m(2) vs. 91.7 +/- 10.4 cm(2)/m(2) in the control state; p = 0.0024) but remained in sinus rhythm. Repetitive atrial firing was triggered by a single extra beat in five of six animals in the APS state and in two of six animals in the control state. Moreover, in two animals in the APS state, a single extra beat triggered sustained AF. Myocytes were enlarged and 39.8% showed some degree of myolysis. In animals in the APS state, the AP had no plateau phase or small amplitude and numerous myocytes were unexcitable. The I(Ca) density was 45.2% lower in APS animals than in control animals. Beta-adrenergic stimulation normalized I(Ca) and restored the plateau phase of the AP. After shunt suppression, the electrophysiologic properties of the atria returned to normal. CONCLUSIONS: The APS induced moderate, isolated LA dilation, which was sufficient to cause major changes in cellular electrophysiologic properties and to render the atria vulnerable to fibrillation. These effects were reversed by shunt suppression.

Surgery for right ventricle to pulmonary artery conduit obstruction: risk factors for further reoperation.

OBJECTIVE: To identify the surgical approaches and risk factors which influence longevity of right ventricle to pulmonary artery (RV-PA) conduits following first reoperation for obstruction. METHODS: Between January 1993 and August 2003, 114 patients underwent 141 reoperations for RV-PA conduit obstruction. Diagnoses included 'Truncus Arteriosus' (n=52), 'Pulmonary atresia/Tetralogy of fallot' (n=39), 'Double outlet right ventricle' (n=10), 'Transposition of great arteries, VSD, and pulmonary atresia' (n=9), and the 'Ross operation' (n=4). All patients had undergone a previous biventricular repair. The first reoperation for conduit obstruction was performed in 112 hospital survivors by: total conduit replacement (Group A, n=73) with valved (homograft=10 and xenograft=54) or non-valved (n=9) conduit, and patch enlargement of the obstructed RV outflow tract with preservation of the posterior and sides of the conduit wall after removing of the fibrocalcific peel and degenerated valve (Group B, n=39). Mean age at first reoperation was 8.8+/-6.7 and 7.5+/-5.3 years in patients of groups A and B, respectively. Seven patients in Group A and 18 in Group B required a second reoperation and two patients in Group B a third reoperation. RESULTS: There were two hospital deaths and no late deaths. Mean follow-up was 5.8+/-3.2 years. Risk factors for second reoperation by univariate analysis were: homograft conduit use (P=0.004), Group B surgical approach (P=0.0001), higher RV-PA systolic pressure gradient at discharge (P=0.02), and age <5-years-old (P=0.01). Multivariate analysis showed that inclusion in Group B and younger age (<5-years-old) at repair were independent risk factors for second reoperation. Group B surgical approaches had higher RV-PA systolic pressure gradient at discharge (P=0.02) and required more PA bifurcation repair at the time of second reoperation (P=0.05). Freedom from second reoperation for conduit obstruction was significantly higher in Group A patients at 5 and 8 years (P<0.04) and those with xenografts rather than homograft (P=0.04). CONCLUSIONS: Our results support the optimal surgical approach for RV-PA conduit obstruction is total replacement with a xenograft. RV outflow reconstruction by other techniques without complete dissection of PA bifurcation does not completely relieve the stenosis and could cause early restenosis. Higher systolic gradients at discharge and younger age at first reoperation are predictors of earlier reoperation.

Subaortic Area at Risk for Development of Obstruction After Surgical Repair of Atrioventricular Septal Defect: Myth or Reality?

BACKGROUND: Morphology of the left ventricular outflow tract (LVOT) in atrioventricular septal defects (AVSDs) has been reported to be at risk for development of obstruction. The purpose of the present study was to identify the incidence, the risk factors, and the surgical outcomes of subaortic stenosis in repaired AVSDs. METHODS: Records of 427 consecutive patients who underwent anatomical repair for all types of AVSDs from January 2000 to December 2012 were reviewed. Outcomes, independent risk factors, reoperation, and death were analyzed. RESULTS: In a median follow-up of five years (range: 17 months-11.8 years), eight patients required nine reoperations for subaortic stenosis. Study group (n = 11) included three additional patients for whom repair was performed in a different institution. Median delay for reoperation was 11.9 years (range: 1.3-19.4 years). Surgical relief of subaortic stenosis was obtained by means of the enlargement of the LVOT: resection of fibrous structures in all reoperated patients and associated with septal myectomy in four. Two patients required a modified Konno procedure. Five reoperations were associated with left atrioventricular valve repair or replacement. Statistical analysis didn't reveal any morphologic or demographic risk factors. No early or late death occurred. CONCLUSION: The development of subaortic stenosis after repair of AVSD remains a rare complication in midterm follow-up (incidence: 1.9%). The performance of preventive gesture during repair seemed to be unjustified. The surgical management of this reoperation consists of a simple and safe procedure.

Late outcomes after arterial switch operation for Taussig-Bing anomaly.

OBJECTIVE: To assess the long-term results of the arterial switch operation (ASO) for Taussig-Bing Anomaly (TBA) and identify risk factors affecting outcomes. METHODS: Retrospective review and late follow-up was performed for all TBA patients from 1997 to 2010 (follow-up >3 years). Selection criteria included the absence of mitro-pulmonary continuity. RESULTS: Sixty-nine children underwent ASO at a median age of 24 days (interquartile range [IR] 11-125), with concomitant repair of aortic arch obstruction in 26 (37.7%). Complex coronary anatomy (n = 38; 55.0%) was common. Nine (13.0%) patients had staged repair. Hospital mortality was 5.8% (95% confidence interval [CI], 1.6%-14.2%; n = 4). Median follow-up was 11.2 years (IR 7.2-13.8). Subsequent mortality was confined to the first postoperative year (n = 5, 86% [95% CI, 78%-95%]), 1-, and 10-year survival). Overall mortality was related to coronary pattern (Yacoub types C and E vs A and D, multivariate, hazard ratio [HR] 12.2 [95% CI, 1.2-122.1], P = .03). At latest follow-up, 96% of the survivors are asymptomatic, with normal ventricular function. Cumulative incidence of reintervention at 10 years was 53% (95% CI, 28%-77%). Concomitant aortic arch obstruction was a predictor of reintervention (multivariate, HR 2.9 [95% CI, 1.1-7.4], P = .03). No mortality occurred upon reinterventions. CONCLUSIONS: In the largest series to date of ASO for TBA, mortality is confined to the first postoperative year, and related to coronary artery pattern. Beyond the first year, needed reinterventions are frequent, but with sustained functional status and no mortality over >10 years follow-up. Aortic arch obstruction is the main predictor for reintervention. Despite a significant rate of early events, favorable long-term outcomes argue for use of the ASO in TBA patients.

Right ventricular failure secondary to chronic overload in congenital heart diseases: benefits of cell therapy using human embryonic stem cell-derived cardiac progenitors.

OBJECTIVE: Despite the increasing incidence of right ventricular (RV) failure in adult patients with congenital heart disease, current therapeutic options are still limited. By contrast to left-heart diseases, cell-based myocardial regeneration applied to the right ventricle is poorly studied, even though it may be a therapeutic solution. As human embryonic stem cell-derived cardiac progenitors seem to be good candidates owing to their proliferation capacity, our aim was to assess, in a large animal model of overloaded RV dysfunction, the feasibility and effects of such a cell therapy. METHODS: Human MesP1(+)/SSEA-1(+) cardiogenic mesodermal cells were administered using multiple intramyocardial injections 4 months after a surgical procedure mimicking the repaired tetralogy of Fallot, and their effects were observed 3 months later on hemodynamic, rhythmic, and histologic parameters. RESULTS: All pigs (sham n = 6, treated n = 6) survived without complication, and cell therapy was clinically well tolerated. Although functional, contractility, and energetics parameters evolved similarly in both groups, benefits regarding arrhythmic susceptibility were observed in the treated group, associated with a significant decrease of peri-myocyte fibrosis (5.71% ± 2.49% vs 12.12% ± 1.85%; P < .01) without interstitial fibrosis change (5.18% ± 0.81% vs 5.49% ± 1.01%). Such a decrease could be related to paracrine effects, as no human cells could be detected within the myocardium. CONCLUSIONS: Cell therapy using intramyocardial injections of human MesP1(+)/SSEA-1(+) cardiogenic mesodermal cells seems to have benefits regarding overloaded RV tissue remodeling and arrhythmic susceptibility, but this mode of administration is not sufficient to obtain a significant improvement in RV function.

Left-sided lesions after anatomic repair of transposition of the great arteries, ventricular septal defect, and coarctation: surgical factors.

OBJECTIVE: This study was undertaken to identify potential anatomic and surgical factors creating left-sided lesions, namely recoarctation of the aorta and neoaortic regurgitation, after anatomic repair of transposition of the great arteries with ventricular septal defect and aortic coarctation. METHODS: From 1983 to September 2002, 109 survivors out of 120 patients were studied. Two-stage repair was performed in 42 patients (group A), and single-stage repair was performed in 67 (groups B and C). Before repair, the diameters of the ascending aorta and main pulmonary artery were measured. In the patients with single-stage repair, coarctation was repaired by extended end-to-end anastomosis in 35 patients (group B) and by pulmonary homograft patch augmentation in 32 patients (group C). The ventricular septal defect was closed through the pulmonary artery in 70 patients and through the right ventricle or atrium in 39 patients. The neoaorto-aortic discrepancy was treated by V-shaped resection of the posterior sinus of Valsalva in 7 cases, pulmonary homograft patch in 32 cases, and anterior splitting of the ascending aorta in all cases. Before discharge from the hospital, neoaortic root and ascending aorta diameters and aortic regurgitation grade were recorded. Neoaortic regurgitation progression and reintervention were the end points of follow-up (97.2 +/- 61.2 months). RESULTS: Early and late survivals were significantly better in group C (P <.001). Risk factors for neoaortic regurgitation at discharge by univariate analysis were single-stage repair (P <.05) and ventricular septal defect closure through the pulmonary artery (P =.0076). On multivariate analysis, the latter was the only risk factor for neoaortic regurgitation at discharge and at last follow-up. Multivariate analysis showed that higher neoaortic root/ascending aorta ratio and ventricular septal defect closure through the pulmonary artery were risk factors for neoaortic regurgitation evolution at last follow-up. There were 29 reinterventions, 19 for recoarctation of the aorta and 10 for neoaortic regurgitation with or without aortic root dilatation. Group B (P <.05), high neoaortic root/ascending aorta ratio (P <.01), and progressive neoaortic regurgitation (P <.05) were risk factors for recoarctation of the aorta. Group A was a risk factor for aortic valve replacement at 10 years (P <.05). CONCLUSION: Neonatal single-stage repair with pulmonary homograft aortic augmentation remains the optimal approach to transposition of the great arteries with ventricular septal defect and aortic coarctation. It provides better early and late survivals and freedoms from left-sided lesions. Avoidance of late recoarctation of the aorta and progressive neoaortic regurgitation requires meticulous closure of the ventricular septal defect and evenly sized reconstruction of the aorta from root to distal arch.

Induction of pulmonary angiogenesis by adenoviral-mediated gene transfer of vascular endothelial growth factor.

BACKGROUND: We hypothesized that gene transfer of vascular endothelial growth factor (VEGF) mediated by an adenovirus vector might induce pulmonary artery angiogenesis in a lamb model of pulmonary artery hypoplasia. METHODS: Thirteen fetal lambs had left pulmonary artery banding at 106 days of gestation. Following birth, 3 groups were divided: VEGF group (n = 5) and beta-GAL group (n = 4) received an adenoviral vector encoding respectively for human VEGF165 and for galactosidase A. A control group (n = 4) had neither gene nor virus. Viral suspensions were selectively instilled in the left bronchus 6.5 days after birth. Five nonoperated lambs constituted the normal group. Euthanasia was performed at 30 days of age. Gene transfer was confirmed by blue coloration of left lung obtained with Xgal solution in an additional experiment. Histomorphometric evaluation was performed. All groups were compared with ANOVA test and paired test was used to compare right and left lung in each animal. RESULTS: Left lung was similarly hypoplastic in all operated lambs. Left pulmonary artery hypoplasia present in all operated groups was significantly less pronounced in VEGF group. The number of pleural arteries was similarly increased in left lung of all operated lambs. Left lung arterial density was higher in VEGF group than in all other groups. The percentage of parenchyma of left lung was lower in beta-GAL group than in all others, partially returned to normal in VEGF group. CONCLUSIONS: In this model, transbronchial VEGF gene transfer induces pulmonary angiogenesis, proximal pulmonary artery growth and contributes to lung parenchyma recovery.