Arch Obstruction Research Articles

Current Results of Single Ventricle Palliation of Patients With Double Inlet Left Ventricle

Double inlet left ventricle (DILV) is a heterogeneous single ventricle anomaly in which initial presentation, and consequently, timing and palliation mode vary based on morphology and degree of pulmonary or systemic outflow obstruction. Very few reports, mostly old, focused on palliation outcomes of DILV. We report current-era results and examine whether morphologic and subsequently surgical factors influence survival. Fifty-eight infants with DILV underwent single ventricle palliation. Echocardiographic examination showed pulmonary (n= 29, 50%), systemic outflow tract (n= 11, 19%), and arch (n= 17, 29%) obstruction. Factors associated with death or transplantation were examined. Forty-four patients (76%) required neonatal first-stage palliation: modified Blalock-Taussig shunt (n= 15, 26%), Norwood (n= 15, 26%), or pulmonary artery band (n= 14, 24%), whereas 14 (24%) received primary Glenn. There was 1 hospital death (2%) and 2 interstage deaths before Glenn, in addition to 1 late death that was noncardiac. Overall 10-year survival was 94% and was comparable for different palliative surgeries (p= 0.49). Three patients (6%) underwent heart transplantation after first-stage palliation (n= 1) or after Glenn (n= 2) for ventricular noncompaction (n= 1), ventricular and atrioventricular valve dysfunction (n= 1), and pacemaker-induced cardiomyopathy (n= 1). Overall 10-year freedom from death or transplantation was 87% and was comparable for different palliative surgeries (p= 0.58). On regression risk analysis, none of the tested morphologic or surgical variables was associated with the risk of death or transplantation. Current outcomes of multistage palliation of DILV are relatively good compared with published reports of other single ventricle anomalies. Survival is not greatly affected by cardiac morphology or initial palliative surgery type.

The Infant with Aortic Arch Hypoplasia and Small Left Heart Structures: Echocardiographic Indices of Mitral and Aortic Hypoplasia Predicting Successful Biventricular Repair.

In infants with aortic arch hypoplasia and small left-sided cardiac structures, successful biventricular repair is dependent on the adequacy of the left-sided structures. Defining accurate thresholds of echocardiographic indices predictive of successful biventricular repair is paramount to achieving optimal outcomes. We sought to identify pre-operative echocardiographic indices of left heart size that predict intervention-free survival in infants with small left heart structures undergoing primary aortic arch repair to establish biventricular circulation (BVC). Infants ≤2months undergoing aortic arch repair from 1999 to 2010 with aortic and/or mitral valve hypoplasia, (Z-score ≤-2) were included. Pre-operative and follow-up echocardiograms were reviewed. Primary outcome was successful biventricular circulation (BVC), defined as freedom from death, transplant, or single ventricular conversion at 1year. Need for catheter based or surgical re-intervention (RI), valve annular growth, and significant late aortic or mitral valve obstruction were additional outcomes. Fifty one of 73 subjects (79%) had successful BVC and were free of RI at 1year. Seven subjects failed BVC; four of those died. The overall 1year survival for the cohort was 95%. Fifteen subjects underwent a RI but maintained BVC. In univariate analysis, larger transverse aorta (p=0.006) and aortic valve (p=0.02) predicted successful BVC without RI. In CART analysis, the combination of mitral valve (MV) to tricuspid valve (TV) ratio ≤0.66 with an aortic valve (AV) annulus Z-score ≤-3 had the greatest power to predict BVC failure (sensitivity 71%, specificity 94%). In those with successful BVC, the combination of both AV and MV Z-score ≤-2.5 increased the odds of RI (OR 3.8; CI 1.3-11.4). Follow-up of non-RI subjects revealed improvement in AV and MV Z-score (median AV annulus changed over time from -2.34 to 0.04 (p<0.001) and MV changed from -2.88 to -1.41 (p<0.001), but residual mitral valve stenosis and aortic arch obstruction were present in one-third of subjects. In this cohort of infants requiring initial aortic arch repair with concomitant small left heart structures, successful BVC can be predicted from combined echocardiographic indices. In this complex population, 1year survival is high, but the need for RI and the presence of residual lesions are common.

Coarctation Index Predicts Recurrent Aortic Arch Obstruction Following Surgical Repair of Coarctation of the Aorta in Infants

Recurrent aortic arch obstruction (RAAO) remains a major cause of morbidity following surgical neonatal repair of coarctation of the aorta (CoA). Elucidating predictors of RAAO can identify high-risk patients and guide postoperative management. The Coarctation index (CoA-I), defined as the ratio of the diameter of the narrowest aortic arch segment to the diameter of the descending aorta, has been used to help diagnose RAAO in neonates following the Norwood Procedure. We sought to assess the predictive value of the CoA-I on RAAO after CoA repair in infants with biventricular circulation. Clinical, surgical, and echocardiographic data of infants with biventricular circulation following neonatal CoA repair between 2010 and 2014 were evaluated. RAAO was defined using a composite quantitative outcome variable: a blood pressure gradient >20, a peak aortic arch velocity >3.5m/s by echocardiogram, or a catheter-measured peak-to-peak gradient >20 within 2years of surgery. Univariate and multivariate logistic regression analyses were used. Of the 68 subjects included in the analysis, 15 (22%) met criteria for RAAO. In the multivariate model, only CoA-I (OR 35.89, 95% CI 6.08-211.7, p<0.0001) and use of patch material (OR 9.26, 95% CI 1.57-54.66, p=0.014) were associated with increased risk of RAAO. The odds of developing RAAO was higher in patients with a CoA-I less than 0.7 (OR 33.8, 95% CI 5.7-199.5, p<0.001). Postoperative CoA-I may be used to predict RAAO in patients with biventricular circulation after repair of CoA. Patients with a CoA-I less than 0.7 or patch aortoplasty warrant close follow-up.

Intraoperative coronary revision but not coronary pattern is associated with mortality after arterial switch operation.

We sought to determine differences in baseline characteristics and clinical outcomes in a consecutive series of patients undergoing arterial switch operation (ASO), assessing the effect of coronary anatomy on postoperative mortality, both overall and adjusted for surgical era. From January 2000 to May 2015, 283 consecutive patients underwent ASO for transposition of the great arteries. A total of 103 patients (36.4%) had an associated ventricular septal defect and 23 (8.1%) had an aortic arch obstruction. Coronary anomalies were present in 119 (42%) patients. Overall survival was 93.0 ± 1% at 15 years (19 events). Intraoperative revision of the coronary anastomosis was required in 8 patients (2.8%) due to inability to wean from cardiopulmonary bypass (CPB) or post-CPB myocardial ischaemia. Surgical revision was neither related to the underlying coronary anatomy ( χ 2 = 0.681, P = 0.4091) nor to the surgical era ( χ 2 = 0.682, P = 0.4090). Univariable analysis suggested decreased mortality rate for infants who underwent surgery during 2008 or later ( P = 0.01). In multivariable analysis, intraoperative coronary button revision was the only predictor of mortality [ P < 0.001, hazard ratio (HR) 12.01, 95% confidence interval (CI) 3.85-37.49], whereas surgical era (before 2008) failed to achieve statistical significance ( P = 0.057, HR 2.19, 95% CI 0.96-12.04). In the recent era, unusual coronary patterns do not affect survival following ASO, whereas intraoperative coronary button revision is a predictor of mortality. Preventive strategies aimed towards intraoperative detection of impaired coronary perfusion are useful tools that might be considered for all patients in order to improve outcomes.

Growth of left ventricular outflow tract and predictors of future re-intervention after repair for ventricular septal defect and aortic arch obstruction.

We carried out a retrospective review of patients who underwent repair for ventricular septal defect and aortic arch obstruction - coarctation or interrupted aortic arch - between July, 2002 and June, 2013. Echocardiographic data were reviewed, and the need for re-intervention was evaluated. A total of 89 patients were included in this study. A significant left ventricular outflow tract growth was noticed after surgical repair. Preoperatively, the mean left ventricular outflow tract Z-score was -1.46±1 (range -5.5 to 1.1) and increased to a mean value of -0.7±1.3 (range -2.7 to 3.2) at last follow-up (p=0.0001), demonstrating relevant growth of the left ventricular outflow tract after repair for ventricular septal defect and aortic arch obstruction. After primary repair, 11 patients (12.3%) required re-intervention with surgical repair for left ventricular outflow tract obstruction after a mean period of 36±21 months. There were no significant differences in age, weight, and indexed aortic valve and left ventricular outflow tract measurements between those who developed obstruction and those who did not. Significant left ventricular outflow tract growth is expected after repair of ventricular septal defect and aortic arch obstruction. Small aortic valve and left ventricular outflow tract at diagnosis are not risk factors to predict the need for surgical re-intervention for left ventricular outflow tract obstruction in future.

Five-year experience with immediate extubation after arterial switch operations for transposition of great arteries.

We sought to identify preoperative, intraoperative and anatomical factors associated with immediate extubation (IE) after arterial switch operation for d-transposition of great arteries (dTGA). This was a single-centre retrospective study performed from 1 January 2010 to 30 June 2015. IE was defined as successful extubation in the operating room (OR). Univariate/bivariate regression of preoperative, intraoperative and anatomical variables was used to determine associations with IE. Of 32 patients in the dTGA spectrum (age at operation 6 days), 18 (56%) underwent IE. Twelve (71%) of the 17 patients with an intact ventricular septum and 6 (43%) of the 14 patients with ventricular septal defect (VSD) underwent IE, whereas none of the patients with double outlet right ventricle or aortic arch obstruction ( n = 4) did. Patients who had cardiopulmonary bypass time (CPB) >173 min ( P = 0.01), lowest temperature on CPB (T min) ≤ 30.4°C ( P = 0.04) and aortic cross-clamp time >86 min ( P = 0.04) were more likely to be left intubated at the end of the procedure. There was no significant difference in patient's chronological age, gestational age, post-conceptual age, weight, coronary anatomy or prevalence of VSD between those who did and did not undergo IE. There was a median increase in intensive care unit (ICU) length of stay (LOS) by 1 day (33%, P = 0.03) and ICU costs by $12 338 (15%, P = 0.06) in non-IE patients. The OR turnover time ( P = 0.09) and reintubation rate ( P = 1) at 24 h post-extubation did not differ between those who did and did not have IE. There was no myocardial dysfunction evident on predismissal echocardiography in either group. In this cohort of infants, post repair for TGA, 56% were extubated immediately in the OR. Greater CPB and cross-clamp times and T min ≤ 30.4°C were associated with a lesser likelihood of IE. IE was associated with shorter ICU length of stay.

Outcomes of the Arterial Switch Operation in Patients With Concomitant Aortic Arch Obstruction as a Single Stage Repair

Purpose: We sought to determine outcomes of patients who underwent arterial switch operation (ASO) with concomitant aortic arch obstruction (AAO) repair as a single stage procedure. Methodology: All patients who underwent an ASO since 2008 were identified from a single institution's database and reviewed retrospectively. Aortic arch obstruction was defined as coarctation of the aorta, hypoplastic aortic arch or interrupted aortic arch. Results: From 1983 to 2008, 666 patients underwent an ASO for biventricular repair at our institution. Sixty-three (9.5%, 63/666) patients who underwent ASO had AAO. Thirty-five (55%, 35/63) patients had AAO repair at time of ASO as part of a single stage repair. Of these 35, twenty-one (60%, 21/35) had transposition of the great arteries and 14 (40%, 14/35) had Taussig-Bing anomaly. There were 2 (5.7%, 2/35) hospital deaths in these patients compared to an overall hospital mortality of 2.9% (19/666). No patients required reoperation during hospital admission. Follow-up was available for 32 (97%, 32/33) survivors. Mean follow-up was 11.2 years (range 7 months to 31 years). There were no late deaths. Three (9.4%, 3/32) patients required 4 late reoperations. Two (6.3%, 2/32) patients had moderate neo-aortic valve regurgitation (AR) at last follow-up. Two (6.3%, 2/32) patients required neo-aortic root valve replacement for severe AR. All survivors were in New York Heart Association Class I at last follow-up. Conclusion: Patients who go undergo ASO with associated AAO as a single stage repair have good early outcomes. Excellent survival and a low reoperation rate continues into adolescence and young adulthood. Close follow-up of neo-aortic valvular regurgitation is warranted.

Aortic Arch Advancement and Ascending Sliding Arch Aortoplasty for Repair of Complex Primary and Recurrent Aortic Arch Obstruction.

The optimal treatment of patients with complex coarctation of the aorta and hypoplastic aortic arch is controversial. Children undergoing repair of obstructive arch lesions are at lifelong risk of recurrence. Multiple techniques have been described to address complex primary and recurrent coarctation. Our approach at Texas Children's Hospital (Houston, TX) has been to address these lesions in an anatomic fashion via median sternotomy, under cardiopulmonary bypass using the techniques of aortic arch advancement and ascending sliding arch aortoplasty. Anatomic repair of complex primary or recurrent aortic obstruction is safe and produces a favorable repair with a low rate of recurrence. Both operations restore normal arch contour using native aortic tissue. The decision of which particular surgical strategy to use depends on the patient's age, anatomy, and the elasticity of the tissue.

How successful is successful? Aortic arch shape after successful aortic coarctation repair correlates with left ventricular function

ObjectivesEven after successful aortic coarctation repair, there remains a significant incidence of late systemic hypertension and other morbidities. Independently of residual obstruction, aortic arch morphology alone may affect cardiac function and outcome. We sought to uncover the relationship of arch 3-dimensional shape features with functional data obtained from cardiac magnetic resonance scans. MethodsThree-dimensional aortic arch shape models of 53 patients (mean age, 22.3 ± 5.6 years) 12 to 38 years after aortic coarctation repair were reconstructed from cardiac magnetic resonance data. A novel validated statistical shape analysis method computed a 3-dimensional mean anatomic shape of all aortic arches and calculated deformation vectors of the mean shape toward each patient's arch anatomy. From these deformations, 3-dimensional shape features most related to left ventricular ejection fraction, indexed left ventricular end-diastolic volume, indexed left ventricular mass, and resting systolic blood pressure were extracted from the deformation vectors via partial least-squares regression. ResultsDistinct arch shape features correlated significantly with left ventricular ejection fraction (r = 0.42, P = .024), indexed left ventricular end-diastolic volume (r = 0.65, P < .001), and indexed left ventricular mass (r = 0.44, P = .014). Lower left ventricular ejection fraction, larger indexed left ventricular end-diastolic volume, and increased indexed left ventricular mass were identified with an aortic arch shape that has an elongated ascending aorta with a high arch height-to-width ratio, a relatively short proximal transverse arch, and a relatively dilated descending aorta. High blood pressure seemed to be linked to gothic arch shape features, but this did not achieve statistical significance. ConclusionsIndependently of hemodynamically important arch obstruction or residual aortic coarctation, specific aortic arch shape features late after successful aortic coarctation repair seem to be associated with worse left ventricular function. Analyzing 3-dimensional shape information via statistical shape modeling can be an adjunct to long-term risk assessment in patients after aortic coarctation repair.

Palliation Outcomes of Neonates Born With Single-Ventricle Anomalies Associated With Aortic Arch Obstruction

The two most common surgical strategies for the treatment of neonates born with single-ventricle anomalies associated with aortic arch obstruction are the Norwood operation and pulmonary artery banding plus coarctation repair (PAB+COA). We reviewed characteristics and outcomes of neonates who underwent those two surgical strategies at our institution. Between 2002 and 2012, 94 neonates with a single ventricle and aortic arch obstruction (excluding hypoplastic left heart syndrome) underwent Norwood (n= 65) or PAB+COA (n= 29). Outcomes were parametrically modeled, and risk factors associated with early and late death were analyzed. Competing-risks analysis showed that, at 2 years after the operation, 24% of patients had died or received transplantation and 75% had undergone a Glenn shunt. At 5 years after the Glenn shunt, 10% of patients had died or received transplantation, 62% had undergone Fontan, and 28% were alive awaiting Fontan. Overall 8-year survival was 70%. Outcomes after Norwood included extracorporeal membrane oxygenation use in 9 (14%), unplanned reoperation in 13 (20%), hospital death in 10 (15%), and interstage death in 8 (12%), with 8-year survival of 66%. Outcomes after PAB+COA included extracorporeal membrane oxygenation use in 1 (3%), unplanned reoperation in 9 (30%), hospital death in 1 (3%), and interstage death in 3 (10%), with 8-year survival of 76%. There was an association trend between underlying anatomy and survival (hazard ratio [HR], 2.1; 95% confidence interval [CI], 0.9 to 4.7; p= 0.087). On multivariable analysis, factors associated with death were extracorporeal membrane oxygenation use (HR, 5.5; 95% CI, 1.9 to 15.9; p= 0.002), genetic syndromes/extracardiac anomalies (HR, 3.5; 95% CI, 1.5 to 8.2; p= 0.003), and weight of 2.5 kg or less (HR, 3.0; 95% CI, 1.3 to 7.2; p= 0.012). Anatomic and patient characteristics influence palliation outcomes in neonates born with single-ventricle anomalies associated with aortic arch obstruction. Although the Norwood operation is applicable in most of these patients, the PAB+COA strategy is a valid alternative in well-selected patients.

Looks Do Matter! Aortic Arch Shape After Hypoplastic Left Heart Syndrome Palliation Correlates With Cavopulmonary Outcomes

Aortic arch reconstruction after hypoplastic left heart syndrome (HLHS) palliation can vary widely in shape and dimensions between patients. Arch morphology alone may affect cardiac function and outcome. We sought to uncover the relationship of arch three-dimensional shape features with functional and short-term outcome data after total cavopulmonary connection (TCPC). Aortic arch shape models of 37 patients with HLHS (age, 2.89 ± 0.99 years) were reconstructed from magnetic resonance data before TCPC completion. A novel, validated statistical shape analysis method was used to compute a three-dimensional anatomic mean shape from the cohort and calculate the deformation vectors of the mean shape toward each patient's specific anatomy. From these deformations, three-dimensional shape features most related to ventricular ejection fraction, indexed end-diastolic volume, and superior cavopulmonary pressure were extracted by partial least-square regression analysis. Shape patterns relating to intensive care unit and hospital lengths of stay after TCPC were assessed. Distinct deformation patterns, which result in an acutely mismatched aortic root and ascending aorta, and a gothic-like transverse arch, correlated with increased indexed end-diastolic volume and higher superior cavopulmonary pressure but not with ejection fraction. Specific arch morphology with pronounced transverse arch and descending aorta mismatch also correlated with longer intensive care unit and hospital lengths of stay after TCPC completion. Independent of hemodynamically important arch obstruction, altered aortic morphology in HLHS patients appears to have important associations with higher superior cavopulmonary pressure and with short-term outcomes after TCPC completion as highlighted by statistical shape analysis, which could act as adjunct to risk assessment in HLHS.

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

Long-term outcomes after the arterial switch operation (ASO) for complex transposition of the great arteries (TGA) should be clarified. 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). 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. 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.

Reoperation for right ventricular outflow tract obstruction after arterial switch operation for transposition of the great arteries and aortic arch obstruction.

Right ventricular outflow tract obstruction (RVOTO) is one of the reasons for late reinterventions after repair of transposition of the great arteries (TGA) with aortic arch obstruction (AAO). The aim of the present study was to identify predictors of reoperation for RVOTO in patients who underwent arterial switch operation (ASO) and arch repair for TGA or Taussig-Bing anomaly with AAO. Between 1977 and 2015, 45 patients [TGA/intact ventricular septum (IVS) 5, TGA/ventricular septal defect (VSD) 13, Taussig-Bing 27] with coarctation (21), arch hypoplasia (5), coarctation and hypoplasia (12) and aortic arch interruption (7) underwent ASO and arch repair. The median age at the ASO was 19 days (range, 1 day to 12.7 years). AAO was repaired concomitantly with ASO in 36 patients. Operation reports and 2D-echocardiographic data were retrospectively reviewed to determine the following parameters: position of the great arteries, coronary artery anatomy, and diameters of RVOT, aortic annulus, aortic sinotubular-junction, pulmonary annulus and transverse aortic arch previous to ASO. The median follow-up time was 6 years (range, 0-30 years). Four patients were lost to follow-up; reliable echo data were available in 24 subjects. Cox proportional hazard models were performed to examine predictors of reoperation for RVOTO. Thirty-day mortality rate after ASO was 13% (n = 6), and late mortality rate 9% (n = 4). Ten patients (TGA/VSD 2, Taussig-Bing 8) had 14 reoperations for RVOTO. One patient died after reoperation. Taussig-Bing anomaly was a significant predictor of reoperation for RVOTO [hazard ratio (HR) = 5.5, 95% confidence interval (CI) = 1.15-26.38, P = 0.033]. Higher preoperative aortic annulus Z-score significantly decreased the reoperation risk (HR = 0.6, 95% CI = 0.42-0.93, P = 0.020). In reoperated patients, the mean gradient across the RVOT reduced from 84 ± 12.2 mmHg prior to reoperation to 15.29 ± 13.70 mmHg at latest follow-up. Taussig-Bing anomaly and smaller preoperative aortic annulus diameter (Z-score) were significant predictors of reoperation for RVOTO in patients after ASO for TGA or Taussig-Bing anomaly with AAO. In Taussig-Bing hearts, the more complex anatomy often necessitates modifications of the operation technique, sometimes precluding RVOT relief at primary ASO. During follow-up, the possibility of recurrent RVOTO should always be considered in this specific patient population. Yet, in case of a reoperation for RVOTO, the surgical relief is in general effective.

Single center experience of aortic bypass graft for aortic arch obstruction in children.

The purpose of this study is to access the outcomes of aortic bypass graft placement in children. This is a retrospective review of all children having aortic bypass graft placement for aortic arch obstruction for the first time between 1982 and 2013 at a single institution. The actuarial survival and the freedom from aortic arch reoperation were calculated and compared between the groups. Seventy consecutive children underwent aortic bypass graft placements. The median age and body weight at the operation were 14days and 3.6kg. There were 7 early deaths, 6 late deaths, and 7 heart transplants during the median follow-up of 10.8years (0.0-31.5years). The actuarial transplant free survival was 64.7% at 20years and the freedom from aortic arch reoperation was 50.5% at 10years. Between the children younger than 1year old and older than 1year old, there were significant differences in actuarial transplant free survival (56.4 vs. 100% at 15years, p=0.0042) and in the freedom from aortic arch reoperation (18.7 vs. 100% at 10years, p<0.001). The children who received aortic bypass graft larger than 16mm in size had no aortic arch reoperation at 15years. The aortic bypass graft placement for aortic arch obstruction can be done with low mortality and morbidity for children who can receive bypass graft larger than 16mm in size. However, it should be avoided for the neonates and infants except selected situations.

Aortic arch repair in children with PHACE syndrome

BackgroundPHACE syndrome is characterized by infantile hemangioma and developmental abnormalities of the brain, arteries of head and neck, and aortic arch. MethodsWe retrospectively reviewed The PHACE Syndrome International Clinical Registry to identify children with PHACE who had operative repair of aortic arch obstruction at Children's Hospital of Wisconsin. ResultsSeven patients (median 11 months, range 1 week-6 years) with PHACE required aortic arch reconstruction from 1996 to 2015. All needed complex surgical approaches (4 conduit grafts, 2 patch aortoplasties, 1 subclavian flap) to relieve the obstruction because of long-segment transverse and proximal descending aortic arch dysplasia that included multiple areas of stricture with adjacent aneurysmal dilatation. Aberrant origin of a subclavian artery was found in 6 of 7. The 3 children who had surgery after age 1 showed significant progression of the arch obstruction and/or adjacent aneurysmal segment dilatation after their initial infant evaluation. No deaths or perioperative complications occurred despite associated cerebrovascular arterial dysplasia in 5 of 7. Recurrent arch obstruction developed in 3 of 7 at an intermediate follow-up interval of 6.2 years (2 had interposition graft replacement at 8 and 11 years due to somatic growth; 1 had repeat patch aortoplasty 11 months after initial repair secondary to recurrent stenosis). ConclusionsExtensive aortic arch reconstruction is commonly required in children with PHACE syndrome and coarctation due to the bizarre nature of the obstruction. Complete preoperative imaging is needed to fully characterize the aortic and cerebrovascular arterial anomalies. Recurrent obstruction is common given the non-native tissue techniques needed to relieve the arch anomaly.

Long-term Outcomes Following One-stage Repair of Aortic Arch Obstruction Associated with Ventricular Septal Defect

Objectives: The aim of the present study was to evaluate the current outcome and reoperation rate after applying a one-stage correction strategy for interrupted aortic arch (IAA) with ventricular septal defect (VSD) and also for aortic coarctation and hypoplastic aortic arch (CoA-HyAA) with VSD beginning November 1999.

Reliable aortic arch measurements using a novel cardiac magnetic resonance sequence: navigated 3D SPACE

Background Existing cardiac magnetic resonance sequences are frequently suboptimal in the measurement of the aortic arch, particularly in the case of arch obstruction. Navigated three-dimensional (3-D) sampling perfection with application optimized contrast using different flip angle evolution (SPACE) is a novel turbo spin echo dark blood cardiac magnetic resonance sequence that yields a robust 3-D dataset and gates in systole in order to demonstrate the maximal aortic caliber. Additionally, it utilizes susceptibility for optimal blood suppression by orienting blood flow in the readout direction, thereby enhancing imaging in the setting of turbulence. We report our experience measuring the aortic arch using this sequence.

Growing the Borderline Hypoplastic Left Ventricle: Hybrid Approach

Surgical management of patients with borderline left ventricular dimensions requires adequate patient selection. Therapeutic concepts have emerged to promote left ventricular growth and achieve adequate dimensions for staged biventricular repair. The hybrid approach thereby uses bilateral pulmonary artery banding and ductal stenting for initial palliation. A restrictive atrial septum is usually left unaddressed to ensure antegrade left ventricular filling. Final repair focuses on relief of obstructive left-sided lesions such as mitral valve stenosis, endocardial fibroelastosis, aortic valve stenosis, and aortic arch obstruction. The initial hybrid procedure is less traumatic than primary relief of left-sided obstructions. It remains to be determined if early or delayed relief of obstructive lesions improves the chances to grow left-sided structures.

Case 5/2016 - Native Coarctation of the Aortic Arch, Relieved By Percutaneous Treatment in an Adult.

Clinical data: the patient had a good clinical course after repair of severe coarctation of the aortic isthmus with end-to-end technique, closure of the interventricular communication at 18 days of life, and relief of moderate subaortic stenosis at 3 years of age. Currently, the patient can tolerate well routine exercise, with no symptoms. At last evaluation, blood pressure in the right arm was 140/70 mmHg and the systolic pressure in the left arm and lower limbs was 90 mmHg, suggesting aortic arch obstruction. Previous evaluations have shown a pressure gradient of 15 mmHg between the upper limbs.

Characterising adequacy or inadequacy of the borderline left ventricle: what tools can we use?

Borderline left ventricle refers to a spectrum of left ventricular underdevelopment, typically associated with other cardiac anomalies. The left ventricle may be mildly hypoplastic, as is sometimes seen accompanying aortic coarctation, or it can be severely hypoplastic, as is seen in hypoplastic left heart syndrome. For patients with a borderline left ventricle that is at either extreme, the treatment decision is relatively straightforward. Those with the most severe form of left ventricle hypoplasia will require single ventricle palliation or cardiac transplantation, whereas those with the mildest form may not need any intervention. It is the management strategy of children that fall within the grey zone of the spectrum, which continues to be controversial and remains variable within and among different institutions. Cardiac diseases with associated left ventricle hypoplasia include critical aortic stenosis, mitral stenosis, coarctation of the aorta, arch hypoplasia, cor triatriatum, unbalanced common atrioventricular canal, Shone's complex, total anomalous pulmonary venous return, and complex conotruncal abnormalities. In this review, we will discuss the assessment and management of infants with borderline left ventricle with critical aortic stenosis or arch obstruction and associated mitral anomalies.