Days alive and out of hospital for children born with single-ventricle heart disease.

BACKGROUND: This study describes the illness burden in the first year of life for children with single-ventricle heart disease, using the metric of days alive and out of hospital to characterize morbidity and mortality. METHODS: This is a retrospective single-centre study of single-ventricle patients born between 2005 and 2021 who had their initial operation performed at our institution. Patient demographics, anatomical details, and hospitalizations were extracted from our institutional single-ventricle database. Days alive and out of hospital were calculated by subtracting the number of days hospitalized from number of days alive during the first year of life. A multivariable linear regression with stepwise variable selection was used to determine independent risk factors associated with fewer days alive and out of hospital. RESULTS: In total, 437 patients were included. Overall median number of days alive and out of hospital in the first year of life for single-ventricle patients was 278 days (interquartile range 157-319 days). In a multivariable analysis, low birth weight (<2.5kg) (b = -37.55, p = 0.01), presence of a dominant right ventricle (b = -31.05, p = 0.01), moderate-severe dominant atrioventricular valve regurgitation at birth (b = -37.65, p < 0.05), index hybrid Norwood operation (b = -138.73, p < 0.01), or index heart transplant (b = -158.41, p < 0.01) were all independently associated with fewer days alive and out of hospital. CONCLUSIONS: Children with single-ventricle heart defects have significant illness burden in the first year of life. Identifying risk factors associated with fewer days alive and out of hospital may aid in counselling families regarding expectations and patient prognosis.

Single stage biventricular repair of hypoplastic right ventricle with straddling tricuspid valve.

We present the case of a five-year-old child with an inlet ventricular septal defect, subpulmonic stenosis, hypoplastic right ventricle, and straddling tricuspid valve who received a successful one-stage biventricular repair with right ventricular rehabilitation, right ventricular outflow tract augmentation, papillary muscle transposition, ventricular septal defect closure, and fenestrated atrial septation. This report outlines the surgical decision making and operative technique.

Predictors of increased postoperative length of stay after complete atrioventricular canal repair.

BACKGROUND: The optimal timing of surgical repair for infants with complete atrioventricular canal defect remains controversial, as there are risks to both early and late repair. We address this debate by investigating the association of various risk factors, including age and weight at surgery, markers of failure to thrive, and pulmonary vascular disease, with postoperative length of stay following complete atrioventricular canal repair. METHODS: Infants who underwent repair of complete atrioventricular canal were identified from our institutional Society of Thoracic Surgeons Congenital Heart Surgery Database. Additional clinical data were collected from the electronic medical record. Descriptive statistics were computed. Associations between postoperative length of stay and covariates of interest were evaluated using linear regression with bootstrap aggregation. RESULTS: From 2001 to 2020, 150 infants underwent isolated complete atrioventricular canal repair at our institution. Pre-operative failure to thrive and evidence of pulmonary disease were common. Surgical mortality was 2%. In univariable analysis, neither weight nor age at surgery were associated with mortality, postoperative length of stay, duration of mechanical ventilation, or post-operative severe valvular regurgitation. In multivariable analysis of demographic and preoperative clinical factors using bootstrap aggregation, increased postoperative length of stay was only significantly associated with previous pulmonary artery banding (33.9 day increase, p = 0.03) and preoperative use of supplemental oxygen (19.9 day increase, p = 0.03). CONCLUSIONS: Our analysis shows that previous pulmonary artery banding and preoperative use of supplemental oxygen were associated with increased postoperative length of stay after complete atrioventricular canal repair, whereas age and weight were not. These findings suggest operation prior to the onset of pulmonary involvement may be more important than reaching age or weight thresholds.

Association Between Digoxin Use and Cardiac Function in Infants With Single-Ventricle Congenital Heart Disease During the Interstage Period.

OBJECTIVES: To examine the association between digoxin use and cardiac function assessed by echocardiographic indices in infants with single-ventricle (SV) congenital heart disease (CHD) during the interstage period. DESIGN: Retrospective cohort study. SETTING: Fifteen North American hospitals. PATIENTS: Infants discharged home following stage 1 palliation (S1P) and prior to stage 2 palliation (S2P). Infants with no post-S1P and pre-S2P echocardiograms were excluded. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of 373 eligible infants who met inclusion criteria, 140 (37.5%) were discharged home on digoxin. In multivariable linear and logistic regressions, we found that compared with infants discharged home without digoxin, those discharged with digoxin had a smaller increase in end-systolic volume (ß = -8.17 [95% CI, -15.59 to -0.74]; p = 0.03) and area (ß = -1.27 [-2.45 to -0.09]; p = 0.04), as well as a smaller decrease in ejection fraction (ß = 3.38 [0.47-6.29]; p = 0.02) and fractional area change (ß = 2.27 [0.14-4.41]; p = 0.04) during the interstage period. CONCLUSIONS: Digoxin may partially mitigate the expected decrease in cardiac function during the interstage period through its positive inotropic effects. Prospective clinical trials are needed to establish the pharmacokinetics, safety, and efficacy of digoxin use in SV CHD.

Comparison of median sternotomy and left anterior mini-incision for pulmonary valve replacement following primary tetralogy of Fallot repair.

OBJECTIVE: Pulmonary insufficiency requiring reintervention frequently occurs after primary tetralogy of Fallot repair. Repeat interventions present a challenge for both the surgeon and patient. We compare a minimally invasive, 5 cm left anterior mini-incision to redo median sternotomy for pulmonary valve replacement in tetralogy of Fallot patients. METHODS: Following Internal Review Board approval, we conducted a single institution retrospective review of patients with tetralogy of Fallot who underwent pulmonary valve replacement via redo median sternotomy or left anterior mini-incision between 13 July, 2016 and 6 March, 2020. RESULTS: Twenty-three patients underwent pulmonary valve replacement following primary tetralogy of Fallot repair between March 2016 and March 2020. Twelve patients received a redo-median sternotomy from March 2016 to August 2018. Left anterior mini-incision was first offered in August of 2018 and was chosen by all eleven patients thereafter. The two groups had similar baseline characteristics including preoperative pulmonary valve dysfunction. Early trends suggest a longer cardiopulmonary bypass time for patients who received left anterior mini-incisions. Other outcomes were comparable, including operative times, blood product requirements, residual pulmonary valve dysfunction, postoperative pain, narcotic requirements, ICU length of stay, total length of stay, and postoperative complications. CONCLUSIONS: In patients who have previously undergone primary repairs of tetralogy of Fallot, outcomes for pulmonary valve replacement via left anterior mini-incision are comparable to those via redo median sternotomy.

Three-dimensional modeling of the mitral valve for surgical planning in a pediatric patient: A case-based discussion of the technical challenges of segmentation and printing from 3D transthoracic echocardiographic datasets.

Abnormal atrioventricular valve present great challenges to the surgeon in achieving a successful repair, and thus present a great opportunity for enhanced 3D imaging to guide pre- and intra-operative management. Spatial and temporal resolution of 3D echocardiography enables 3D printing of valve morphology. However, non-linearity, angle dependence, speckle, blur, and resampling complicate segmentation compared to computed tomography (CT) and magnetic resonance imaging (MRI). A case of complex mitral valve disease in a pediatric patient is therefore presented to illustrate the technical challenges of segmentation and 3D printing from echocardiographic data.

Sustained Total All-Region (STAR) perfusion for Norwood reconstruction with complex intracardiac repair.

We present the case of a newborn with complex congenital heart disease who was treated with a neonatal Norwood operation and total anomalous pulmonary venous return repair. During the Norwood reconstruction, a novel technique was utilized to perfuse the head, lower body, and heart continuously to minimize ischemic injury.

Initial Application of a Bicuspid Aortic Annuloplasty Ring in Pediatric Cardiac Surgery.

Aortic stenosis and aortic insufficiency (AI) are common valvular conditions that may necessitate repair or replacement of the aortic valve. Aortic valve replacement is associated with higher long-term complications and thus, a consistent, reliable method of repair is needed. This is especially true in the pediatric population where lifelong anticoagulation and development of recurrent aortic stenosisor aortic insufficiency are especially problematic. The Hemispherical Aortic Annuloplasty Reconstruction Technology ring has been developed and used for annular stabilization in adults with success, though its efficacy in the pediatric population has yet to be demonstrated. Herein, we discuss the use of a geometric ring in aortic valve repair for the pediatric patient.

Biventricular Conversion in the Borderline Hypoplastic Heart.

PURPOSE OF REVIEW: The development of biventricular repair and conversion pathways for patients with borderline hypoplastic heart disease represents an area of recent inquiry and innovation. This review summarizes emerging techniques and novel treatment algorithms for borderline hypoplastic heart disease with a focus on surgical advances within the last 10 years. RECENT FINDINGS: Many patients with borderline hypoplastic heart disease are amenable to primary biventricular repair, or biventricular conversion following single-ventricle palliation coupled with ventricular rehabilitation strategies. New insights into the potential for growth and recovery of borderline ventricles have been uncovered. However, questions remain regarding optimal patient selection and the long-term outcomes of select patient groups treated with single-ventricle palliation versus biventricular repair/conversion or transplantation. Efforts to direct a greater proportion of borderline hypoplastic heart patients towards a biventricular circulation are accelerating and represent important avenues for progress and future research in the field of congenital heart disease.

Implantation of a HeartMate 3 ventricular assist device in a 21-kg pediatric patient with Fontan failure.

AIMS: The HeartMate 3 (HM3) ventricular assist device (VAD) is gaining popularity in adults due to a favorable risk profile. However, reports of HM3 use in children are limited, potentially due to concerns with device size. MATERIALS AND METHODS: Here we report the successful use of an HM3-VAD as a bridge to transplantation in a 21 kg (BSA 0.84), an 8-year-old male child with Fontan failure on home inotropes. RESULTS: Urgent VAD implantation was performed. The standard adult sewing ring was used. The tricuspid valve and papillary muscles were completely excised from the ventricular cavity to prevent inflow obstruction. The pump was placed in the left pleural space. Outflow graft and driveline implantation were routine. VAD function appeared excellent with a reduction in Fontan pressures and improved kidney and liver function. Reoperation was required once to rule out tamponade and twice to evacuate a recurrent right hemothorax. The patient was discharged 3 months later in good condition and underwent successful heart transplantation 10 months after VAD placement. DISCUSSION: This report demonstrates the feasibility of HM3-VAD implantation in a 21-kg Fontan patient, suggesting HM3 size is not a prohibitive limitation at this weight.

Recent Advances in Congenital Heart Surgery: Alternative Perfusion Strategies for Infant Aortic Arch Repair.

PURPOSE OF REVIEW: This paper will discuss current cannulation strategies for infant aortic arch repair and compare them to more traditionally used techniques. RECENT FINDINGS: Aortic arch reconstruction in infants has traditionally involved deep hypothermic circulatory arrest which results in total body ischemia. This has been associated with an increased risk of morbidity including bleeding, renal dysfunction, and neurologic injury. Advances in perfusion techniques have allowed for preserved perfusion to the brain during arch repair. Current techniques have further evolved that allow for continuous perfusion of the heart and even the lower body during arch reconstruction. With current techniques, aortic arch reconstruction in infants can be performed with continuous perfusion to the brain, heart, and lower body. Further technical refinements will be helpful, and study is necessary to evaluate the benefit of these strategies.

Evolving practice pattern changes and outcomes in the era of hybrid aortic arch repair.

OBJECTIVE: The role of hybrid repair in the management of aortic arch pathology, and long-term outcomes with these techniques, remains uncertain. We report a decade of experience with hybrid arch repair (HAR) and assess institutional practice patterns with regard to the use of hybrid and open techniques. METHODS: Hybrid and open total and distal arch procedures performed between July 2005 and January 2015 were identified from a prospectively maintained, institutional aortic surgery database. Perioperative morbidity and mortality, freedom from reintervention, and long-term survival were calculated. Hybrid and open procedural volumes over the study period were assessed to evaluate for potential practice pattern changes. RESULTS: During the study period 148 consecutive procedures were performed for repair of transverse and distal aortic arch pathology, including 101 hybrid repairs and 47 open total or distal arch repairs. Patients in the hybrid repair group were significantly older with a greater incidence of chronic kidney disease, peripheral vascular disease, and chronic lung disease. Perioperative mortality and outcomes were not significantly different between the hybrid and open groups, aside from decreased median length of stay after hybrid repair. Need for subsequent reintervention was significantly greater after hybrid repair. Unadjusted long-term survival was superior after open repair (70% 5-year survival open vs 47% hybrid; P = .03), although aorta-specific survival was similar (98% 5-year aorta-specific survival open vs 93% hybrid; P = .59). Institutional use of HAR decreased over the final 3 years of the study, with an associated increased use of open total or distal arch repairs. This was primarily the result of decreased use of native zone 0 hybrid procedures. Concurrent with this apparent increased stringency around patient selection for HAR, perioperative morbidity and mortality was reduced, including avoidance of retrograde type A dissection. CONCLUSIONS: HAR remains a viable option for higher-risk patients with transverse arch pathology with perioperative outcomes and long-term aorta-specific survival similar to open repair, albeit at a cost of increased reintervention. This observational single-institution study would suggest decreased use in more recent years in favor of open repair due to avoidance of native zone 0 hybrid procedures. This decline in the institutional use of native zone 0 hybrid repairs was associated with improved perioperative outcomes.

Complementary roles of open and hybrid approaches to thoracoabdominal aortic aneurysm repair.

OBJECTIVE: Thoracoabdominal aortic aneurysm (TAAA) repair remains a significant challenge with considerable perioperative morbidity and mortality. A hybrid approach utilizing visceral debranching with endovascular aneurysm exclusion has been used to treat high-risk patients and therefore allow repair in more patients. Limited data exist regarding long-term outcomes with this procedure as well as comparison to conventional open repair. This study describes our institutional algorithmic approach to TAAA repair using both open and hybrid techniques. METHODS: Hybrid and open TAAA repairs performed between July 2005 and August 2015 were identified from a prospectively maintained institutional aortic surgery database. Perioperative morbidity and mortality, freedom from reintervention, and long-term and aorta-specific survival were calculated and compared between the two groups. RESULTS: During the study period, 165 consecutive TAAA repairs were performed, including 84 open repairs and 81 hybrid repairs. Patients in the hybrid repair group were significantly older, were more frequently female, and had a generally greater comorbid disease burden, including significantly more chronic kidney disease. Despite the older and sicker cohort, there was no difference in in-hospital mortality between the two groups (9.9% hybrid vs 7.1% open; P = .59). Major morbidity rates differed by procedure, with patients undergoing open repair having a significantly higher rate of postoperative stroke (9.5% open vs 0% hybrid; P = .017), whereas patients undergoing hybrid repair had a higher rate of new permanent dialysis (14.8% hybrid vs 3.6% open; P = .043). There was no difference between groups in the rate of postoperative permanent paraplegia/paresis (8.3% open vs 7.4% hybrid; P = .294). There was a significantly increased rate of reintervention in the hybrid repair group (12.3% hybrid vs 1.2% open, P = .004), with all hybrid reinterventions performed because of endoleak. One-year survival was similar between groups at 69% in hybrid repairs vs 77% in open repairs. Long-term survival was worse in the hybrid group (5-year survival, 32% hybrid vs 56% open), although late survival appeared to be influenced mainly by comorbid disease burden, given the similar long-term aorta-specific survival between groups. CONCLUSIONS: Use of an algorithmic approach whereby higher risk patients with TAAA are treated by a hybrid approach and lower risk patients with conventional open repair yields satisfactory short- and long-term outcomes. The availability of multiple options for TAAA repair within a single center likely allows repair in more patients with consequent decrease in the risk of aorta-related death, at the expense of increased reinterventions for endoleak.

Calcium signaling regulates ventricular hypertrophy during development independent of contraction or blood flow.

In utero interventions aimed at restoring left ventricular hemodynamic forces in fetuses with prenatally diagnosed hypoplastic left heart syndrome failed to stimulate ventricular myocardial growth during gestation, suggesting chamber growth during development may not rely upon fluid forces. We therefore hypothesized that ventricular hypertrophy during development may depend upon fundamental Ca(2+)-dependent growth pathways that function independent of hemodynamic forces. To test this hypothesis, zebrafish embryos were treated with inhibitors or activators of Ca(2+) signaling in the presence or absence of contraction during the period of chamber development. Abolishment of contractile function alone in the setting of preserved Ca(2+) signaling did not impair ventricular hypertrophy. In contrast, inhibition of L-type voltage-gated Ca(2+) influx abolished contraction and led to reduced ventricular hypertrophy, whereas increasing L-type voltage-gated Ca(2+) influx led to enhanced ventricular hypertrophy in either the presence or absence of contraction. Similarly, inhibition of the downstream Ca(2+)-sensitive phosphatase calcineurin, a known regulator of adult cardiac hypertrophy, led to reduced ventricular hypertrophy in the presence or absence of contraction, whereas hypertrophy was rescued in the absence of L-type voltage-gated Ca(2+) influx and contraction by expression of a constitutively active calcineurin. These data suggest that ventricular cardiomyocyte hypertrophy during chamber formation is dependent upon Ca(2+) signaling pathways that are unaffected by heart function or hemodynamic forces. Disruption of Ca(2+)-dependent hypertrophy during heart development may therefore represent one mechanism for impaired chamber formation that is not related to impaired blood flow.

Repair of Bicuspid Aortic Valve Syndrome with Anomalous Right Coronary Artery in Osteogenesis Imperfecta.

Osteogenesis imperfecta (OI) is an inherited connective tissue disorder caused by the defective synthesis of type I collagen. The clinical phenotype is dominated by bone fragility, but cardiovascular tissue involvement has also been reported. Here, the case is described of a 37-year-old man with OI who presented with aortic insufficiency, bicuspid aortic valve, dilated aortic root, and anomalous right coronary artery. The patient was treated successfully with a mechanical valved conduit aortic root replacement and anomalous coronary artery unroofing and reimplantation. This case highlights the feasibility of complex surgical repairs in this population, as well as challenges surrounding the choice of valve prosthesis given the congenital bone fragility and predilection for fractures.

Five-year results for endovascular repair of acute complicated type B aortic dissection.

INTRODUCTION: Despite a current lack of U.S. Food and Drug Administration approval for the indication, thoracic endovascular aortic repair (TEVAR) has replaced open surgical management for acute complicated type B aortic dissection due to promising short- and midterm data. However, long-term results, with a view toward durability and need for secondary procedures, are limited. As such, the objective of the present study is to report long-term outcomes of TEVAR for acute (≤ 2 weeks from symptom onset) complicated type B dissection. METHODS: Between July 2005 and September 2012, 50 consecutive patients underwent TEVAR for management of acute complicated type B dissection at a single referral institution. Patient records were retrospectively reviewed from a prospectively maintained clinical database. RESULTS: Indications for intervention included rupture in 10 (20%), malperfusion in 24 (48%), and/or refractory pain/impending rupture in 17 (34%). One patient (2%) had both rupture and malperfusion indications. Ten (20%) patients required one or more adjunctive procedures, in addition to TEVAR, to treat malperfusion syndromes. In-hospital and 30-day rates of death were both 0%; 30-day/in-hospital rates of stroke, permanent paraplegia/paraparesis, and new-onset dialysis were 2% (n = 1), 2% (n = 1), and 4% (n = 2), respectively. Median follow-up was 33.8 months [interquartile range, 12.3-56.6 months]. Overall survival at 5 and 7 years was 84%, with no deaths attributable to aortic pathology. Thirteen (26%) patients required a total of 17 reinterventions over the study period for type I endoleak (n = 5), metachronous aortic pathology (n = 5), persistent false lumen pressurization via distal fenestrations (n = 4), type II endoleak (n = 2), or retrograde acute type A aortic dissection (n = 1). Median time to first reintervention was 4.5 months (range, 0 days-40.3 months). Of the 17 total reinterventions, six (35%) were performed using open techniques and 11 (65%) with endovascular or hybrid methods; there was no difference in survival between patients who did or did not require reintervention. CONCLUSIONS: This study confirms the excellent short-term outcomes of TEVAR for acute complicated type B dissection and demonstrates the results to be durable and sustained over long-term follow-up. Although aortic reinterventions were required in one-quarter of patients, no aortic-related deaths were observed. These data support the use of TEVAR for acute complicated type B aortic dissection but also highlight the importance of life-long aortic surveillance by an experienced aortic referral center in order to identify and treat complications of the underlying disease process and treatment, as well as new aortic pathologies, as they arise.

Comparison of attachment site endoleak rates in Dacron versus native aorta landing zones after thoracic endovascular aortic repair.

OBJECTIVE: Type I attachment site endoleaks are the most common cause for reintervention after thoracic endovascular aortic repair (TEVAR) and represent treatment failures. Deployment of endografts in segments of the aorta previously replaced with Dacron grafts may be associated with reduced type I endoleak due to mechanical stability and decreased potential for aortic remodeling. However, no study has rigorously examined endoleak rates in Dacron landing zones vs native aorta. METHODS: A retrospective analysis of a prospectively maintained database was performed to identify all patients undergoing TEVAR at a single referral institution between May 2002 and June 2012. Overall, 319 patients undergoing 345 procedures had at least one follow-up contrast-enhanced computed tomography scan to assess for postoperative type I endoleak. Attachment site landing zones were classified as native aorta, Dacron, or endograft if landed in a previously placed endograft. Patient characteristics and type I endoleak rates were compared among the three groups. RESULTS: Identified were 697 proximal or distal landing zones (native aorta, 599; Dacron, 79; and endograft, 19). Patients with at least one Dacron landing zone had higher rates of hypertension (P < .01), chronic obstructive pulmonary disease (P = .04), and prior aortic surgery (P < .01) and were more likely to have undergone complex hybrid repairs (P < .01). Cumulative type I endoleak rates were equivalent between the three types of landing zone (native aorta, 3.7%; Dacron, 2.5%; endograft, 0%; P = .44). Two type I endoleaks occurred with Dacron landing zones in the first tertile of TEVAR experience and with Dacron landing zone lengths of <2.5 cm. Evaluation of endoleak rates by tertile of experience demonstrated decreased type I endoleak rates in Dacron landing zones between the first and second/third tertiles of experience (13.3% vs 0%, P = .03) after a policy of using >4 to 5 cm (twice the device instructions for use) of Dacron overlap was initiated. CONCLUSIONS: Endograft deployment within long-segment (landing zone length of >4-5 cm) Dacron represents a durable option for aortic repair and was associated with a 0% rate of type I endoleak. In cases of a borderline native aortic landing zone, a hybrid procedure to create an adequate Dacron landing zone may be warranted to decrease the risk of type I endoleak and treatment failure.

Staged repair of borderline hypoplastic heart disease with early biventricular conversion.

Objective: In select patients with borderline ventricular hypoplasia, we adopted a strategy of initial single-ventricle palliation followed by staged or direct biventricular conversion by 2 years of age. Methods: Between 2018 and 2023, 14 newborns with borderline hypoplastic heart disease deemed high risk for primary biventricular repair underwent palliative procedures as a neonate/infant, followed by staged or direct biventricular conversion. Results: Of the 14 patients, 6 had borderline left ventricles and 8 had borderline right ventricles. Index neonatal operations were performed in 12 patients and included the Norwood operation (n = 5), pulmonary artery band (n = 3), ductal stent (n = 3), and hybrid Norwood (n = 1). Five patients underwent direct biventricular conversion, and the remaining 9 patients underwent staged ventricular recruitment operations at a mean age of 6 months (range, 3-11 months). Ventricular recruitment operations included atrial septation with or without ventricular rehabilitation, atrioventricular valve repair, or outflow tract operations. At a mean duration of 8 months (range, 4-10 months) after ventricular recruitment, there was a significant increase in chamber volume, aortic valve, and mitral valve size in patients with borderline left ventricles, and a normalization of the right ventricle:left ventricle end-diastolic volume ratio in patients with borderline right ventricles. To date, 13 of 14 patients have undergone successful biventricular conversion at a mean age of 16 months (range, 4-31 months). Conclusions: In select newborns with borderline hypoplastic heart disease, single-ventricle palliation followed by staged or direct biventricular conversion may increase infant survival while allowing for early attainment of a biventricular circulation.

Intrathoracic subclavian artery aneurysm repair in the thoracic endovascular aortic repair era.

OBJECTIVE: Intrathoracic subclavian artery aneurysms (SAAs) are rare aneurysms that often occur in association with congenital aortic arch anomalies and/or concomitant thoracic aortic pathology. The advent of thoracic endovascular aortic repair (TEVAR) methods may complement or replace conventional open SAA repair. Herein, we describe our experience with SAA repair in the TEVAR era. METHODS: A retrospective review was performed of all intrathoracic SAAs repaired at a single institution since United States Food and Drug Administration approval of TEVAR in 2005. RESULTS: Nineteen patients underwent 20 operations to repair 22 (13 native, nine aberrant) SAAs with an intrathoracic component. Mean SAA diameter was 3.1 cm (range, 1.6-6.0 cm). Mean patient age was 57 years (range, 24-80 years). Twenty-one percent (n = 4) of patients had a connective tissue disorder (two Loeys-Dietz, two Marfan). Thirty-six percent (n = 8) of SAAs were repaired by open techniques and 64% (n = 14) via a TEVAR-based approach. All TEVAR cases required proximal landing zone in the aortic arch (zone 0-2), and revascularization of at least one arch vessel was required in 83% (10/12) of patients. Concomitant repair of associated aortic pathology was performed in 50% (n = 10) of operations. Thirty-day/in-hospital rates of death, stroke, and permanent paraplegia/paraparesis were 5% (n = 1), 5% (n = 1), and 0%, respectively. Over mean (standard deviation) follow-up of 24 (21) months, 16% (n = 3) of patients required reintervention for subclavian artery bypass graft revision (n = 2) or type II endoleak (n = 1). CONCLUSIONS: This is the largest single-institution series to date of TEVAR for SAA repair. Modern endovascular techniques expand SAA repair options with excellent results. The majority of SAAs and nearly all aberrant SAAs (Kommerell's diverticulum) can now be repaired using a TEVAR-based approach without the need for sternotomy or thoracotomy.

Results with an algorithmic approach to hybrid repair of the aortic arch.

OBJECTIVE: Hybrid repair of the transverse aortic arch may allow for aortic arch repair with reduced morbidity in patients who are suboptimal candidates for conventional open surgery. We present our results with an algorithmic approach to hybrid arch repair, based on the extent of aortic disease and patient comorbidities. METHODS: Between August 2005 and January 2012, 87 patients underwent hybrid arch repair by three principal procedures: zone 1 endograft coverage with extra-anatomic left carotid revascularization (zone 1; n = 19), zone 0 endograft coverage with aortic arch debranching (zone 0; n = 48), or total arch replacement with staged stented elephant trunk completion (stented elephant trunk; n = 20). RESULTS: The mean patient age was 64 years, and the mean expected in-hospital mortality rate was 16.3% as calculated by the EuroSCORE II. Of operations, 22% (n = 19) were nonelective. Sternotomy, cardiopulmonary bypass, and deep hypothermic circulatory arrest were required in 78% (n = 68), 45% (n = 39), and 31% (n = 27) of patients to allow for total arch replacement, arch debranching, or other concomitant cardiac procedures, including ascending with or without hemiarch replacement in 17% (n = 8) of patients undergoing zone 0 repair. All stented elephant trunk procedures (n = 20) and 19% (n = 9) of zone 0 procedures were staged, with 41% (n = 12) of patients undergoing staged repair during a single hospitalization. The 30-day/in-hospital rates of stroke and permanent paraplegia or paraparesis were 4.6% (n = 4) and 1.2% (n = 1). Of 27 patients with native ascending aorta zone 0 proximal landing zone, three (11.1%) experienced retrograde type A dissection after endograft placement. The overall in-hospital mortality rate was 5.7% (n = 5); however, 30-day/in-hospital mortality increased to 14.9% (n = 13) owing to eight 30-day out-of-hospital deaths. Native ascending aorta zone 0 endograft placement was found to be the only univariate predictor of 30-day in-hospital mortality (odds ratio, 4.63; 95% confidence interval, 1.35-15.89; P = .02). Over a mean follow-up period of 28.5 ± 22.2 months, 13% (n = 11) of patients required reintervention for type 1A (n = 4), type 2 (n = 6), or type 3 (n = 1) endoleak. Kaplan-Meier estimates of survival at 1 year, 3 years, and 5 years were 73%, 60%, and 51%. CONCLUSIONS: Hybrid aortic arch repair can be tailored to patient anatomy and comorbid status to allow complete repair of aortic pathology, frequently in a single stage, with acceptable outcomes. However, endograft placement in the native ascending aorta is associated with high rates of retrograde type A dissection and 30-day/in-hospital mortality and should be approached with caution.