Antenatal Detection of Treatable Critical Congenital Heart Disease Is Associated with Lower Morbidity and Mortality.

OBJECTIVE: To establish the impact that timing of diagnosis and place of birth have on neonatal outcomes in those with readily treatable critical congenital heart disease. STUDY DESIGN: This was a population-based study with a complete national cohort of live-born infants with transposition of the great arteries and aortic arch obstruction in New Zealand between 2006 and 2014. Timing of diagnosis, place of birth, survival to surgery, in-hospital events, and neonatal mortality were reviewed. Live births with a gestation of ≥35 weeks and without associated major extracardiac anomalies were included for analysis. RESULTS: A total of 166 live-born infants with transposition of the great arteries and 87 with aortic arch obstruction were included. Antenatal detection increased from 32% in the first 3 years to 47% in the last 3 years (P = .05). During the same period, neonatal mortality decreased from 9% to 1% (P = .02). No deaths occurred after surgical intervention. An antenatal diagnosis was associated with decreased mortality (1/97 [1%] vs 11/156 [7%]; P = .03) and birth outside the surgical center was associated with increased risk of mortality (11/147 [7%] vs 1/106 [1%]; P = .02). Those with an antenatal diagnosis required fewer hours of mechanical ventilation (P = .02) and had shorter durations of hospital stay (P = .05) compared with those diagnosed >48 hours after birth. CONCLUSIONS: The mortality risk for transposition of the great arteries and critical aortic arch obstruction is greatest before cardiac surgery. Improved antenatal detection allowing delivery at a surgical center is associated with reduced mortality.

Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study / Mortalidade para Cardiopatias Congênitas e Fatores de Risco Associados em Recém-Nascidos. Um Estudo de Coorte

Abstract Background: Congenital heart diseases are the most common type of congenital defects, and account for more deaths in the first year of life than any other condition, when infectious etiologies are ruled out. Objectives: To evaluate survival, and to identify risk factors in deaths in newborns with critical and/or complex congenital heart disease in the neonatal period. Methods: A cohort study, nested to a randomized case-control, was performed, considering the Confidence Interval of 95% (95% CI) and significance level of 5%, paired by gender of the newborn and maternal age. Case-finding, interviews, medical record analysis, clinical evaluation of pulse oximetry (heart test) and Doppler echocardiogram were performed, as well as survival analysis, and identification of death-related risk factors. Results: The risk factors found were newborns younger than 37 weeks (Relative Risk - RR: 2.89; 95% CI [1.49-5.56]; p = 0.0015), weight of less than 2,500 grams (RR: 2.33 [; 95% CI 1.26-4.29]; p = 0.0068), occurrence of twinning (RR: 11.96 [95% CI 1.43-99.85]; p = 0.022) and presence of comorbidity (RR: 2.27 [95% CI 1.58-3.26]; p < 0.0001). The incidence rate of mortality from congenital heart disease was 81 cases per 100,000 live births. The lethality attributed to critical congenital heart diseases was 64.7%, with proportional mortality of 12.0%. The survival rate at 28 days of life decreased by almost 70% in newborns with congenital heart disease. The main cause of death was cardiogenic shock. Conclusion: Preterm infants with low birth weight and comorbidities presented a higher risk of mortality related to congenital heart diseases. This cohort was extinguished very quickly, signaling the need for greater investment in assistance technology in populations with this profile.

Resumo Fundamento: As cardiopatias congênitas configuram o tipo mais comum de defeitos congênitos, sendo responsáveis por mais mortes no primeiro ano de vida do que em qualquer outra condição, quando etiologias infecciosas são excluídas. Objetivo: Avaliar a sobrevida e identificar os fatores de risco nos óbitos em recém-nascidos com cardiopatia congênita crítica e/ou complexa no período neonatal. Métodos: Realizou-se um estudo de coorte, aninhado a um caso-controle aleatorizado, considerando Intervalo de Confiança de 95% (IC95%) e nível de significância de 5%, pareado por sexo do recém-nascido e idade materna. Foram feitas buscas ativas de casos, entrevistas, análise de prontuário, avaliação clínica da oximetria de pulso (teste do coraçãozinho) e do ecoDopplercardiograma, bem como análise de sobrevida e identificação dos fatores de risco relacionados ao óbito. Resultados: Os fatores de risco encontrados foram recém-nascidos com menos de 37 semanas (Risco Relativo − RR: 2,89; IC95% 1,49-5,56; p = 0,0015), peso inferior a 2.500 g (RR: 2,33; IC95% 1,26-4,29; p = 0,0068), ocorrência de gemelaridade (RR: 11,96; IC95% 1,43-99,85; p = 0,022) e presença de comorbidade (RR: 2,27; IC95% 1,58-3,26; p < 0,0001). A taxa de incidência de mortalidade por cardiopatias congênitas foi de 81 casos por 100 mil nascidos vivos. A letalidade atribuída às cardiopatias congênitas críticas foi de 64,7%, com mortalidade proporcional de 12,0%. A taxa de sobrevida aos 28 dias de vida diminuiu em quase 70% nos recém-nascidos com cardiopatias congênitas. A principal causa de óbito foi o choque cardiogênico. Conclusão: Recém-nascidos prematuros, com baixo peso e presença de comorbidades apresentaram maior risco de mortalidade relacionada às cardiopatias congênitas. Esta coorte se extinguiu muito rapidamente, sinalizando para a necessidade de maior investimento em tecnologia assistencial em populações com este perfil.

Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study.

BACKGROUND: Congenital heart diseases are the most common type of congenital defects, and account for more deaths in the first year of life than any other condition, when infectious etiologies are ruled out. OBJECTIVES: To evaluate survival, and to identify risk factors in deaths in newborns with critical and/or complex congenital heart disease in the neonatal period. METHODS: A cohort study, nested to a randomized case-control, was performed, considering the Confidence Interval of 95% (95% CI) and significance level of 5%, paired by gender of the newborn and maternal age. Case-finding, interviews, medical record analysis, clinical evaluation of pulse oximetry (heart test) and Doppler echocardiogram were performed, as well as survival analysis, and identification of death-related risk factors. RESULTS: The risk factors found were newborns younger than 37 weeks (Relative Risk - RR: 2.89; 95% CI [1.49-5.56]; p = 0.0015), weight of less than 2,500 grams (RR: 2.33 [; 95% CI 1.26-4.29]; p = 0.0068), occurrence of twinning (RR: 11.96 [95% CI 1.43-99.85]; p = 0.022) and presence of comorbidity (RR: 2.27 [95% CI 1.58-3.26]; p < 0.0001). The incidence rate of mortality from congenital heart disease was 81 cases per 100,000 live births. The lethality attributed to critical congenital heart diseases was 64.7%, with proportional mortality of 12.0%. The survival rate at 28 days of life decreased by almost 70% in newborns with congenital heart disease. The main cause of death was cardiogenic shock. CONCLUSION: Preterm infants with low birth weight and comorbidities presented a higher risk of mortality related to congenital heart diseases. This cohort was extinguished very quickly, signaling the need for greater investment in assistance technology in populations with this profile.

Single-Stage Correction for Taussig-Bing Anomaly Associated With Aortic Arch Obstruction.

Taussig-Bing anomaly and aortic arch obstruction are two types of complex congenital cardiac malformations. Almost 50% of patients with Taussig-Bing anomaly have aortic arch obstruction. This report assesses the surgical outcomes of single-stage correction in neonates with both defects. Between November 2006 and November 2015, 39 neonates with Taussig-Bing anomaly and aortic arch obstruction (28 patients with coarctation of the aorta and 11 patients with interrupted aortic arch) underwent a one-stage arterial switch operation and aortic reconstruction. There were three in-hospital deaths and one late death (8 months after the surgery). The short-term survival rate was 92.3% (36/39), and the mid-term survival rate was 89.7% (35/39). Follow-up data were available for all patients who survived the operation (range 6-92 months). Echocardiology showed six cases of recoarctation, three cases of left ventricular outflow tract obstruction, three cases of right ventricular outflow tract obstruction, four cases of pulmonary artery stenosis, five cases of aortic regurgitation, and eight cases of pulmonary regurgitation. Eight patients required a reoperation during the follow-up period with no mortality. All survivors remained in good condition (New York Heart association functional class I or II). Single-stage correction of Taussig-Bing anomaly with aortic arch obstruction in neonates had favorable short- and mid-term outcomes in terms of mobility and reoperation rate. The optimal operative procedure should be chosen according to the position of the coronary arteries and the type of aortic anomaly.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Comparison of Total Arch and Partial Arch Transposition During Hybrid Endovascular Repair for Aortic Arch Disease.

OBJECTIVE: Total arch transposition (TAT) during hybrid endovascular repair for aortic arch disease is believed to allow a better landing zone, but also to be associated with higher peri-operative mortality than partial arch transposition (PAT). Information on this issue is limited. METHOD: This study was a retrospective analysis. All 53 consecutive patients with aortic arch disease (41 males, mean age 65.0 years) who underwent hybrid endovascular repair with TAT (zone 0, n=20) or PAT (zone 1 or 2, n=33) from 2008 to 2014 were analyzed retrospectively. The peri-operative and late outcomes of these two groups were compared. RESULTS: Baseline characteristics, including EuroSCORE II results, were similar in the two groups. After procedures, peri-operative mortalities and stroke rates were similar in the two groups (5.0% vs. 9.1%, p=1.000, and 10.0% vs. 6.1%, p=.627). Interestingly, all four strokes occurred in patients with a type III aortic arch irrespective of transposition type. Primary success rates (80.0% vs. 69.7%, p=.527) and type I endoleak incidences (20.0% vs. 27.3%, p=.744) were not significantly different. During follow up (mean duration 36.9 months), overall survival (89.7% vs. 87.4% at 1 year and 89.7% vs. 79.3% at 3 years; p=.375) and re-intervention free survival rates (78.6% vs. 92.0% at 1 year; 72.0% vs. 62.2% at 3 years, p=.872) were similar in the two groups. CONCLUSION: Morbidity and mortality were high within the first year of hybrid endovascular therapy for aortic arch disease, implying that candidates for hybrid procedures need to be selected carefully. Hybrid endovascular repair with TAT was found to have peri-operative mortality, stroke, and long-term survival rates comparable with PAT, so hybrid endovascular repair may be considered, irrespective of type of arch reconstruction, when clinically indicated.

Contemporary results of surgical repair of recurrent aortic arch obstruction.

BACKGROUND: There is a paucity of data on the current outcomes of surgical intervention for recurrent aortic arch obstruction (RAAO) after initial aortic arch repair in children. The goal of this study is to report the long-term results in these patients. METHODS: All patients undergoing surgical intervention for RAAO at Texas Children's Hospital from 1995 to 2012 were included. The cohort was divided into four groups based on initial procedure: (1) simple coarctation repair, (2) Norwood procedure, (3) complex congenital heart disease, and (4) interrupted aortic arch. RESULTS: A total of 48 patients age 9 months (range, 22 days to 36 years) underwent 49 procedures for RAAO. All patients had an anatomic repair consisting of either patch aortoplasty (n=27, 55%), aortic arch advancement (n=8, 16%), sliding arch aortoplasty (n=6, 12%), placement of an interposition graft (n=2, 17%), reconstruction with donor allograft (n=4, 8%), extended end-to-end anastomosis (n=1, 2%), or redo Norwood-type reconstruction (n=1, 2%). Most procedures (n=46, 94%) were performed through a median sternotomy using cardiopulmonary bypass. At a median follow-up of 6.1 years (range, 9 days to 17 years), only 2 patients required surgical or catheter-based intervention for RAAO. Hypertension was present in 10% of patients at last follow-up. There were no neurologic or renal complications. There was 1 perioperative death after an aortic arch advancement in group 1. Four other patients have died during follow-up, none of the deaths related to RAAO. CONCLUSIONS: Anatomic repair of RAAO is a safe procedure associated with low morbidity and mortality, and low long-term reintervention rates.

Applicability and mid-term results of endovascular treatment for descending thoracic acute aortic syndromes.

BACKGROUND: Descending thoracic acute aortic syndromes (DTAASs) are life-threatening pathologies in which thoracic endovascular aortic repair (TEVAR) is an attractive therapeutic option. There are few data in the literature on the survival and morbidity rates of TEVAR in such indications. We provide the results of TEVAR from our single-center cohort of DTAASs: ruptured aneurysms (RAs), acute symptomatic dissections (ADs), and traumatic ruptures (TRs). METHODS: Between 2004 and 2011, data from all patients treated by TEVAR for DTAAS in our university center were collected prospectively. Primary end points were rates of 30-day mortality and morbidity. Secondary end points were mid-term outcomes and reinterventions. RESULTS: Forty-eight patients underwent TEVAR for DTAASs: 19 RAs (39.6%), 12 ADs (25.0%), and 17 TRs (35.4%). The cumulative 30-day mortality and morbidity rate was 33%. There were 10 deaths (6 RAs, 1 AD, and 3 TRs), 2 cases of paraplegia (0 postoperative and 2 preoperative cases (2 TRs), and no major reinterventions and 4 minor reinterventions (embolization for endoleak exclusion). Long-term outcomes were known in 33 patients (5 patients were lost to follow-up), with a mean duration follow-up of 27 months. The cumulative mortality and morbidity rate was 9%: no deaths were reported, and 2 major reinterventions (open aneurysm repair) and 1 minor reintervention (endoleak exclusion) were required. CONCLUSIONS: Our results confirm that TEVAR, as an emergency therapeutic option, is suited to DTAASs. The 21% perioperative mortality rate for such dramatic lesions remains high but is lower than medical or open repair therapeutic options previously reported. No additional deaths occurred during the 2-year follow-up period, and a low incidence of endograft-related reinterventions was observed. Nevertheless, scheduled follow-up visits with computed tomodensitometry scans on a regular basis are mandatory.

Endografting in the aortic arch - does the proximal landing zone influence outcome?

OBJECTIVES: To analyse early and midterm results of thoracic aortic endografting (TEVAR) in the aortic arch. METHODS: Between January 1997 and February 2009 178 patients received TEVAR in the aortic arch at our institution. This population was subdivided into four groups according to the proximal landing zone (LZ) classification in the aortic arch by Ishimaru et al. and a retrospective analysis regarding perioperative mortality, morbidity and endoleak formation was performed. RESULTS: The overall 30-day mortality rate was 14% with no statistical significant difference between LZ's 0-3 (p=0.274). Renal insufficiency (hazard ratio (HR) 2.5; p=0.0119), age >75 years (HR 3.1; p=0.0019) and emergency procedures (HR 8.9; p < 0.0001) were independent predictors of death. There was no significant difference regarding type I (p=0.07) or type III (p=0.49) endoleaks between the proximal LZs, but a significant difference regarding the development of type II endoleaks (p=0.01). CONCLUSIONS: The present study showed no influence of the proximal LZ on perioperative mortality and morbidity rate. Furthermore it did not influence relevant (type I/III) endoleak formation.

Off-pump extraanatomic aortic bypass for the treatment of complex aortic coarctation and hypoplastic aortic arch.

BACKGROUND: Despite advances in surgical and interventional techniques, the optimal surgical treatment of severe aortic (re) coarctation and hypoplastic aortic arch is still controversial. Anatomic repair may require extensive dissection, cardiopulmonary bypass, and deep hypothermic circulatory arrest with their inherent risks. The aim of this study was to analyze the outcome of off-pump extraanatomic aortic bypass as a surgical alternative to local repair. METHODS: From February 2000 to December 2005, ten consecutive patients (median age 20 years; range, 11 to 38 years) with severe aortic (re) coarctation (n = 4) and (or) hypoplastic aortic arch (n = 7) underwent off-pump extraanatomic aortic bypass through median sternotomy. All but three patients had undergone previous surgery for coarctation and angioplasty or stenting. Three patients underwent concomitant replacement of the ascending aorta because of an aneurysm using cardiopulmonary bypass. RESULTS: Postoperative hospital course was uneventful in all patients. There was no perioperative mortality or significant morbidity. During a mean follow-up of 48 +/- 22 months no patient required additional procedures. All patients were free of symptoms; no patient showed signs of heart failure after follow-up. At last follow-up, no patient presented with claudication, nor any patient experienced orthostatic problems due to a steal phenomenon. During follow-up, hypertension resolved in all patients with residual mild hypertension in two patients. CONCLUSIONS: Off-pump extraanatomic aortic bypass is an attractive treatment option for complex aortic (re) coarctation and hypoplastic aortic arch. Perioperative risks are minimized, hypertension is influenced favorably, and midterm survival is event-free.

Influence of concomitant coronary artery bypass graft on outcome of surgery of the ascending aorta/arch.

BACKGROUND: Surgery of the ascending aorta with or without arch is being performed in an increasingly elderly population with risks of coexisting coronary artery disease. AIM: To define specific groups requiring coronary artery bypass graft (CABG) and to analyse the influence of concomitant CABG on outcome. DESIGN: Over a 10-year period in a single institution, 296 consecutive procedures on the ascending aorta with or without arch were carried out in 291 patients. CABG was required in 42 (14.2%) procedures. In 24 (57%) patients, CABG was planned preoperatively and in 18 (43%) patients, on a salvage basis. RESULTS: In-hospital mortality for patients undergoing concomitant CABG was higher (21.4% v 11%, p<0.06). Adjusting for baseline and operative characteristics, this was attributable to operative priority, and was not a consequence of concomitant CABG (adjusted OR 0.30, 95% CI 1.1 to 8.31; p = 0.48). However, in-hospital mortality was significantly higher when CABG was performed as salvage rather than as a planned procedure (38.9% v 8.9%, p = 0.025), and this difference remained after adjusting for confounding variables (adjusted OR 16.2, 95% CI 1.03 to >200; p = 0.047). The 3-year survival was significantly lower with concomitant CABG (59% v 81.9%, p<0.001). CONCLUSIONS: In association with surgery of the ascending aorta with or without arch planned concomitant CABG did not entail any added operative risk. However, salvage CABG, which occurred almost exclusively in association with emergency cases, was associated with a higher early mortality. Patients needing concomitant CABG had worse survival at 3 years compared with those requiring isolated surgery of the ascending aorta with or without arch.

Technical advances in total aortic arch replacement.

BACKGROUND: We compared the effects of using hypothermic circulatory arrest (HCA) alone, HCA combined with selective cerebral perfusion (SCP), and use of SCP with a trifurcated graft (T) on outcome after aortic arch repair. METHODS: One hundred fifty patients, median age 66 years (range, 27 to 85), underwent total arch replacement between 1988 and 2002; 75 were female. We retrospectively compared the results of three patient groups roughly comparable with regard to preoperative risk factors: 45 patients using HCA beginning in 1988; 67 patients using HCA/SCP beginning in 1994; and 38 patients utilizing a trifurcated arch graft in conjunction with SCP through the axillary artery (HCA/SCP/T) since 2000. The groups were well matched with regard to median age (66, 68, and 66 years), urgency (emergent 11%, 13%, 5%; urgent 24%, 9%, 18%; and elective 64%, 78%, 76%), and several other known risk factors (p = not significant). RESULTS: An adverse outcome-hospital death or permanent stroke-occurred in 14%: in 16% with HCA, in 16% with HCA/SCP, and in 8% with HCA/SCP/T. Transient neurologic dysfunction among patients surviving without stroke was lower with HCA/SCP/T (11%) than with HCA (33%) or HCA/SCP (17%). Mean duration of HCA was 52 +/- 16 minutes with HCA alone versus 45 +/- 10 minutes with HCA/SCP and 31 +/- 7 minutes with HCA/SCP/T (p < 0.0001 for groups HCA and HCA/SCP combined versus HCA/SCP/T). Mean duration of SCP was 57 +/- 25 minutes with HCA/SCP versus 62 +/- 24 minutes with HCA/SCP/T (p = not significant). Comparison of the groups of patients who had comparable preoperative risk factors for adverse outcome showed a trend toward lower adverse outcome and transient neurologic dysfunction rates using HCA/SCP/T; a significant reduction in respiratory (p < 0.001), infectious (p = 0.015) and cardiac (p = 0.005) complications in HCA/SCP/T compared with the earlier groups; and significantly shorter durations of intensive care (p < 0.0001) and hospitalization (p = 0.004). CONCLUSIONS: Our results suggest that HCA/SCP is superior to HCA alone for preventing cerebral injury during operations on the aortic arch. By further reducing embolic risk as well as duration of HCA, HCA/SCP/T with axillary artery cannulation may be the optimal technique for averting adverse outcomes, reducing complications, and shortening hospital stay after aortic arch repair.

The limitation of staged repair in the surgical management of congenital complex heart anomalies with aortic arch obstruction.

OBJECTIVE: Severe aortic arch obstruction including an interrupted aortic arch in congenital complex heart anomalies remains a challenge in surgical management. METHODS: Treatment and outcomes in 75 consecutive patients who underwent an aortic arch repair as the first step of the staged repair protocol between 1975 and 2000 were reviewed. Their ages at repair ranged from 1 day to 8.5 months. RESULTS: Cross-sectional postoperative follow-up data were available in all the patients. The follow-up period ranged from 0 to 27.6 years (mean: 7.3 +/- 7.3 years). There were 20 postoperative hospital deaths (27%) and 7 late deaths. The Kaplan-Meier estimate of survival was 81.3% +/- 4.5% at 1 month, 68.0% +/- 5.4% at 1 year, 65.0% +/- 5.5% at 5 years, 63.1% +/- 5.7% at 10 years, 63.1% +/- 5.7% at 20 years. By Cox regression analysis, body weight of 2.5 kg or less is the only independent determinant of postoperative mortality (p = 0.04, multivariable odds ratio: 2.50, [95% confidence interval: 1.02-6.1]). The aortic arch morphology, the primary cardiac lesion, or date of operation did not reach a statistically significant level to show correlation with mortality. Reintervention to reconstruct the aortic arch was performed at 9 occasions in 8 of the 55 patients who survived the primary operation (14.5%). The Kaplan-Meier estimate of the reintervention-free rate was 91.3% +/- 4.2% at 5 years, 85.5% +/- 5.6% at 10 years, 75.6% +/- 8.2% at 20 years. Using multivariable Cox regression analysis, interrupted aortic arch (versus aortic coarctation) was the only independent predictor of a shorter time to reintervention (p = 0.001, multivariable odds ratio: 16.1, [95% confidence interval: 3.2-80.2]). CONCLUSIONS: The staged repair protocol was associated with significant limitations in patient survival and with the development of recurrent aortic arch obstruction. Thus, a primary repair protocol may serve as an alternate approach, especially in patients with low weight or with an interrupted aortic arch.

Aortic arch reconstruction using regional perfusion without circulatory arrest.

OBJECTIVES: Deep hypothermic circulatory arrest during repair of aortic arch anomalies may induce neurological complications or myocardial injury. Regional cerebral and myocardial perfusion may eliminate those potential side effects. METHODS: From March 2000 to March 2002, 48 neonates or infants with complex arch anomaly were operated on using the regional perfusion technique. Thirty-three patients were male and the median age was 24 days (range 5-301 days). Preoperative diagnosis consisted of coarctation or interruption of the aorta associated with ventricular septal defect (group I, n = 26) and arch anomaly with complex intracardiac defects such as hypoplastic left heart syndrome or its variants (group II, n = 22). Arterial cannula was inserted through the innominate artery and the flow rate was regulated to about 50-100 ml/kg per min during regional perfusion. Simultaneous myocardial perfusion was maintained using a Y-connected infusion line. Cardioplegia was applied during intracardiac repair. RESULTS: Cardiopulmonary bypass and aortic cross-clamp times were 154 +/- 49 and 39 +/- 34 min, respectively. Temporary circulatory arrest for intracardiac procedures was performed in eight patients. However, the mean arrest time was minimized (range 1-18 min). The descending aorta clamping time was 33 +/- 16 min. Operative mortality rates in each group were 0 and 18.2% (0/26 and 4/22). Late mortality rates were 0 and 11.1% (0/26 and 2/18) during 9.1 months of follow-up. Complications consisted of low cardiac output in eight cases, transient neurological problems in two cases, and transient renal insufficiency in two cases, respectively. CONCLUSIONS: Regional perfusion is feasible and can be used with acceptable results. It may reduce potential complications following aortic arch reconstruction using circulatory arrest. However, repair of aortic arch in the patients with complex intracardiac defects still imposes a significant rate of mortality and morbidity.

Surgical treatment of nonaneurysmal aortic arch lesions in patients with systemic embolization.

PURPOSE: Atherosclerotic lesions of the aortic arch are potential sources of arterial embolism. Here we investigate whether surgery, with the necessary circulatory supports, can be proposed as a good option for treatment of this problem. Study of these lesions on a national scale in France has made possible the assessment for future indications of techniques and results of the surgical management of aortic arch lesions, which retrospectively proved to be embolic. METHODS: Thirty-eight patients, (19 men and 19 women) underwent surgery between 1976 and 1996 in 17 French cardiovascular surgical centers. The average age at the time of surgery was 49 +/- 12 years (range, 31 to 82 years). Atherosclerotic lesions were detected with transesophagial echocardiography (n = 19), angiography of the aortic arch (n = 16), computed tomography (n = 9), and magnetic resonance imaging (n = 10). Surgery consisted of thrombectomy and endarterectomy (n = 22), aortic resection and graft replacement (n = 10), and patch aortoplasty (n = 5; one thrombus disappeared spontaneously before surgery was performed). RESULTS: The average postoperative period was 30 months (range, 3 to 82 months). Contact was lost with four patients after a follow-up period of 12 months. On pathologic specimens obtained at surgery, an atherosclerotic plaque was found in 73% of the cases (n = 28). In 15% of the cases, the aorta appeared normal (n = 6) and four other types of lesion were identified: angiosarcoma (n = 1), ectasia at the insertion of the remains of the ductus arteriosus (n = 1), rupture of tunica intima (n = 1), and a fibroblastic plaque (n = 1). A thrombus was identified in 26 cases, attached to the arterial wall in 18 cases. When transesophagial echocardiographic results showed mobile lesions (n = 22), histopathologic examination of specimens allowed the detection of a thrombus in 18 cases and an atherosclerotic plaque with a mobile projection in four cases. The postoperative mortality rate was 2.6%. The morbidity rate (28.9%; n = 11) was related to neurologic complications (n = 6), vascular complications (n = 4), and infection (n = 1). Four cases (12%) were reoperated. CONCLUSION: Nonaneurysmal aortic arch lesions are a frequent and still underestimated source of stroke and peripheral embolization. Surgery with circulatory support can be recommended in good operative candidates with recurrent critical events despite medical management and with high embolic potential (young patients with no calcified plaques).

Interruption of the aortic arch in adults.

The interruption of the aortic arch is a rare cardiac congenital disease; such patients may occasionally survive to adulthood without surgery. The associated intracardiac malformations may modify survival. Our aim is to report three young adults (18-19 years of age) with interruption of the aortic arch. Two had type C, and the other had type B with subpulmonic ventricular septal defect and pulmonary valve insufficiency. We review 106 cases collected from the medical literature into 3 groups: 1) the whole group of patients; 2) patients with isolated interruption of the aortic arch, and 3) patients with interruption of the aortic arch associated with ventricular septal defect. In the whole group we found 18 cases of interruption of the aortic arch type A, and 25 cases of interruption of the aortic arch type B; 37 cases of isolated interruption of the aortic arch and 43 cases associated with ventricular septal defect. Fifty percent of the patients died before 15 days of life (0.042 years). According to the cumulative frequency graphic, only 5% of the patients survived beyond the age of 5 years. We found no information to relate patient's survival rate and anatomic type of the interruption of the aortic arch. From adolescence, the survival of the patients with interruption of the aortic arch associated to septal ventricular defect was 7%, and a 14% survival was found in patients with isolated interruption of the aortic arch. No statistical difference was found between the means of the ages of these two groups (P > 0.25).

Interruption of the aortic arch in adults / Interrupción del arco aórtico en adultos

La interrupción del arco aórtico es una cardiopatía congénita rara; los pacientes ocasionalmente sobreviven hasta la edad adulta sin cirugía y la sobrevida puede estar influenciada por malformaciones intracardiacas asociadas. El propósito de este artículo es informar los casos clínicos de 3 adultos jóvenes (18-19 años) con interrupción del arco aórtico. Dos de ellos con interrupción del arco aórtico tipo C y el otro tipo B, con comunicación interventricular subpulmonar e insuficiencia valvular pulmonar. Reunimos 106 casos colectados de la literatura médica, divididos en 3 grupos: 1) la muestra completa de pacientes, 2) los pacientes con interrupción del arco aórtico aislado y 3) los pacientes con interrupción del arco aórtico asociada con comunicación interventricular. En la muestra completa, encontramos 18 casos de interrupción del arco aórtico tipo A, 25 casos de interrupción del arco aórtico tipo B, 37 casos de interrupción aislada del arco aórtico y 43 casos asociada con comunicación interventricular. El 55 por ciento de los pacientes murió antes de 15 días de vida (0.042 años). De acuerdo a la gráfica de frecuencia acumulada, solamente 5 por ciento de los pacientes sobrevivió más allá de los 5 años. No encontramos suficiente información para señalar alguna razón que explique la sobrevida de los pacientes en relación al tipo de interrupción del arco aórtico, aunque algunas cardiopatías asociadas acortan la vida y otras, como la doble salida del ventrículo derecho, permiten mayor sobrevida. Llegaron a la adolescencia 7 por ciento de los pacientes con interrupción del arco aórtico asociada a la comunicación interventricular y 14 por ciento de los enfermos con interrupción aislada del arco aórtico. A pesar de esta divergencia, no hubo diferencia estadística significativa entre las medias de edad de estos pacientes (P> 0.25)

Single-stage repair of aortic arch obstruction and associated intracardiac defects with pulmonary homograft patch aortoplasty.

OBJECTIVE: Intracardiac malformations associated with coarctation and aortic arch hypoplasia have traditionally been repaired in 2 stages, with a high mortality rate. We review our experience with single-stage biventricular repair of intracardiac defects associated with aortic arch hypoplasia by means of pulmonary homograft patch aortoplasty. METHODS: Between October 1988 and October 1997, 39 of 40 consecutive patients underwent single-stage biventricular repair for aortic arch obstruction and associated intracardiac defects. The median age at operation was 17 days and the mean weight was 3.71 +/- 1.09 kg. Nineteen patients had either dextrotransposition of the great arteries or the Taussig-Bing anomaly. Sixteen patients had multiple left-sided obstructive lesions (2 cases of critical aortic stenosis, 3 of subaortic stenosis and ventricular septal defect, and 11 of hypoplastic left heart complex). One patient had an associated complete atrioventricular septal defect. Four patients had only an associated ventricular septal defect. Through a median sternotomy, the hypoplastic aortic arch was enlarged with a pulmonary homograft patch in 36 patients. In 4 patients an extended end-to-end anastomosis was performed. RESULTS: There were 2 early deaths (5%) and 2 late deaths (5%). One late death was not cardiac related. The mean follow-up time was 36 months (range 1 month-9 years). The recoarctation rate after pulmonary homograft patch aortoplasty was 8. 3%, but after exclusion of those patients with associated left-sided obstructive lesions this decreased to 0%. No aneurysm formation in the aorta has occurred. The actuarial survival at 8 years is 89% +/- 10%. CONCLUSIONS: Single-stage biventricular repair of aortic arch obstruction and associated intracardiac defects can achieve excellent survival. We recommend pulmonary homograft patch aortoplasty because it achieves complete relief of anatomic afterload with a tension-free anastomosis and low incidence of recoarctation.

One-stage neonatal repair of complex aortic arch obstruction or interruption. Recent experience at Texas Children's Hospital.

The optimal surgical approach for complex aortic coarctation or an interrupted aortic arch with associated intracardiac defects is not universally agreed upon. We reviewed our experience with 18 consecutive patients (10 with coarctation, 8 with interrupted aortic arch) undergoing a 1-stage repair through median sternotomy between September of 1995 and February of 1997. Age at operation ranged from 3 days to 3 months (mean 23 days) and weight ranged from 1,700 g to 5,100 g (mean 3,350 g). Under hypothermic circulatory arrest, the aortic arch was reconstructed using native tissue-tissue anastomoses, and coexisting intracardiac anomalies were repaired by standard techniques. All patients survived the procedure and were ultimately discharged from the hospital. There were 2 late deaths in the interrupted aortic arch group, 1 during reoperation for subaortic stenosis and the other from noncardiac causes 5 months after discharge. Another interrupted aortic arch patient required a Ross-Konno procedure 8 months later. There has been no recoarctation among the 16 survivors. Thus a 1-stage repair for complex aortic arch obstruction in neonates can be accomplished with low operative risk, although long-term outcome is strongly influenced by the presence of subaortic obstruction.