Arch In Infants Research Articles

Experience in applying the strategy of simultaneous selective perfusion of the brain, heart and lower body in reconstruction of the aortic arch in infants

Relevance. The choice of perfusion strategy is vital for the protection of internal organs during surgery. In several studies of recent years, as well as by us, a strategy of sustained total all region (STAR) perfusion (selective brain, heart and lower body perfusion) has been proposed, allowing to avoid ischemic and reperfusion injuries of internal organs.The objective was to evaluate the benefits of STAR perfusion during reconstruction of the aortic arch in children.Materials and methods. The prospective study included 15 patients who underwent aortic arch reconstruction using a strategy of simultaneous selective brain, heart and lower body perfusion (STAR perfusion) in the period from June 2022 to May 2023. The average age at the time of surgery was 1.3 months (95 % CI (confidence interval) 6.0–16.0), the average body weight was 3.4 kg (95 % CI 2.7–4.1).Results. Сhanges of the tissue oximetry index, measured at two points, did not fall below reference ranges at any stage of the operation. The greatest mean value of lactate concentration during the operation was 2.8 ± 1.0 mmol/l, recovery of the indicator was noted after 6 hours – 1.9 ± 0.9 mmol/l. Kidney function evaluation – the rate of diuresis and creatinine levels did not exceed reference levels. The average treatment time in the intensive care unit was 7.7 ± 4.3 days (95 % CI 4.5 10.9), in the hospital – 15.4 ± 5.8 days (95 % CI 11.4–19.4). There were no deaths among those operated using the new perfusion strategy.Conclusion. The use of the strategy of Sustained Total All Region (STAR) perfusion in case of reconstruction of the aortic arch in newborn, infants and young children is safe and advanced, since it avoids ischemia of internal organs and reduces the risk of post-ischemic complications.

Reconstruction of the Aortic Arc through Left Thoracotomy in Patients with Isolated Coarctation and Aortic Arch Hypoplasia

Background. The choice of surgical approach for the reconstruction of aortic arch hypoplasia is a rather controversial issue. Traditionally, left lateral thoracotomy is used to correct aortic coarctation with hypoplasia of one or more arc seg-ments. However, in recent years, more and more clinics have begun widespread use of median sternotomy for the surgical treatment of aortic arch hypoplasia. The purpose of the research was to analyze the immediate and long-term results of surgical treatment of isolated hypoplasia of the aortic arch in infants through left thoracotomy.
 Materials and methods. From 2010 to 2019, 159 infants with coarctation and hypoplasia of one or more segments of the aortic arch underwent reconstruction of the aortic arch at the National Amosov Institute of Cardiovascular Surgery of the NAMS of Ukraine and Ukrainian Children’s Cardiac Center. The study included only patients with biventricular physiology and subsequent biventricular correction. There were 108 male patients (68%) and 51 female patients (32%).
 The mean age of the patients was 2.4 ± 0.9 months, the mean body weight was 4.7 ± 2.0 kg. The main method of diagnosis, i.e. determining the defect and assessing the immediate and long-term results, was echocardiographic examination. The study group consisted of 155 patients (97.5%) in whom the reconstruction of the aortic arch was performed through left thoracotomy, in the remaining 4 patients (2.5%) the intervention was performed through median sternotomy.
 
 Results. In-hospital mortality was 0.6% (n = 1). The average time of the operation was 126 ± 40 minutes, the aortic cross-clamp time was 24.7 ± 5.1 minutes. The mean duration of mechanical ventilation after the surgery was 41 ± 15.9 hours.
 The mean follow-up period ranged from 1 month to 13 years. There were no deaths in the long-term period. Ten (6.4%) patients developed aortic arch restenosis in the postoperative period. In 8 patients, the narrowing was treated by endovascular dilatation. Two patients underwent repeated aortic arch repair using median sternotomy. There were no cases of compression of the trachea, main bronchi, neurological complications in the follow-up period.
 
 Conclusions. Reconstruction of the aortic arch hypoplasia through left thoracotomy is an effective and safe treatment option in infants associated with good immediate and long-term results. The study indicates that this surgical strategy can be used in most patients with hypoplasia of the isthmus and distal aortic arch.

Study on the views and methods of ultrasonic screening and diagnosis for abnormal aortic arch in infants

BackgroundThe purpose of this study was to explore echocardiographic views and methods of aortic arch anomalies in infants, so as to improve the screening sensitivity and diagnostic accuracy.Methods140 children with abnormal aortic arch diagnosed by ultrasound in Children’s Hospital of Hebei Province from January 2014 to December 2019 were selected for retrospective analysis. All were confirmed by surgery or/and computerized tomography angiography. Series of views for aortic arch (the three-vessel and tracheal view, aortic arch short axis view, left aortic arch long axis view, aortic arch long axis continuous scan views) were performed in all cases on the basis of the routine views of echocardiography. The screening sensitivity and diagnostic coincidence rate of different echocardiographic views for aortic arch anomalies were analyzed.ResultsAmong the 140 infants, right aortic arch were 21 cases (6/21 were accompanied by mirror branch and 15/21 were with aberrant left subclavian artery). Left aortic arch with aberrant right subclavian artery were 2 cases, and double aortic arch with both arches open were 20 cases. Double aortic arch with left arch atresia were 2 cases, and atresia of the proximal aorta with aortic arch dysplasia was 1 case. Coarctation of the aorta were 67 cases, and interruption of aortic arch were 27 cases. All the patients were correctly diagnosed except that 2 infants with interruption of aortic arch were incorrectly diagnosed as coarctation of the aorta, and 1 infant with coarctation of the aorta was misdiagnosed as interruption of aortic arch by echocardiography. The screening sensitivities of four views and four-view combination for abnormal aortic arch were 99.3, 73.6, 87.1, 99.3, and 100%; the diagnostic coincidence rates were 85.7, 27.1,66.4, 95.0%, and 97.9% respectively. On the basis of traditional left aortic long axis view, other three views had their own advantages. The screening sensitivity and diagnostic coincidence rate of four-view combination were significantly improved.ConclusionsThe three-vessel trachea view is simple and feasible, which is suitable for screening abnormal aortic arch. The combination of four views conduces to improving screening sensitivity and diagnostic accuracy of aortic arch abnormalities.

Coarctatio aortae: management from infancy to adulthood.

The surgical management of congenital heart defects is changing continuously due to technical development. Aortic coarctation is one of the most frequent congenital heart diseases often with serious symptoms. The authors review the different surgical techniques (end-to-end anastomosis, pathplasty, interposition) and present the up-to-date procedures from infancy to adulthood. We assess the results, the benefits and disadvantages of the different procedures based on our and international experiences. The greatest surgical challenges are the management of the serious hypoplastic aortic arch in infancy and later evolving restenosis and complications (aneurysm, dissection). Nowadays, the management of aortic coarctation is very difficult because the surgical procedures are different in infancy, childhood and adult age. In the latter two cases, it is necessary to apply catheter interventions, too, the correct timing of which also present great challenges nowadays. Orv Hetil. 2020; 161(21): 861-866.

Early Postnatal Echocardiography in Neonates with a Prenatal Suspicion of Coarctation of the Aorta

Coarctation of the aorta (COA) is suspected prenatally when there is ventricular asymmetry, arterial disproportion, and hypoplasia of the aortic arch/isthmus. The presence of fetal shunts creates difficulty in prenatal confirmation of the diagnosis so serial echocardiography after birth is necessary to confirm or refute the diagnosis. The first neonatal echocardiogram in prenatally suspected cases of COA was assessed for prediction of neonatal COA repair (NCOAR). This included morphological assessment, measurement of the aortic arch and calculation of the distal arch index (DAI = distance between left common carotid and left subclavian artery/diameter of thedistal arch). NCOAR was undertaken in 23/60 (38%) cases. Transverse arch, aortic isthmus z-score, and DAI had an area under the receiver operator curve of 0.88 (95% CI 0.77-0.98), 0.86 (95% CI 0.75-0.96), and 0.84 (95% CI 0.74-0.95), respectively for the prediction of NCOAR. Using transverse arch z-score threshold < - 3 gave sensitivity 100%, NPV: 100%, specificity 76%; aortic isthmus z-score < - 3: NPV 92%, specificity 62% and DAI > 1.4: NPV 88%, specificity 78%. The size of the distal aortic arch in infants with a common origin of the innominate artery and left common carotid artery who did not require COA repair was similar to the NCOAR cases (p = 0.22). The early postnatal assessment of the size and morphology of the aortic arch can assist in risk stratification for development of neonatal COA. The branching pattern of the head/neck vessels impacts on the size of the distal aortic arch adding to the complexity of predicting COA based on vessel size.

P2789A new technique to restore shape and pattern of flow in hypoplastic aortic arch

Abstract Background There is no agreement about the best method for correcting hypoplastic aortic arch in infancy. Residual abnormalities in shape can cause late complications. Patients and methods We have developed and applied a new method of direct anastomosis of the descending to the ascending aorta to allow for restoration of shape, function and growth. 12 infants undergoing this technique at the age of 61±3months (range 6–28 months) underwent repeat CT 4–15 months after operation. Pre- and post-operative arch width, height and height to width ratio were recorded for all cases. Computerized shape analysis using an in-house tool (developed in MATLAB) was used for processing the 3D segmented shapes of the aortic arch, and assessing changes in the cross-sectional area at different levels. Computational Fluid Dynamics (CFD) was used to simulate aortic flow propagation and pattern after surgery, using patient specific geometry and flow boundary conditions. Results The pre- and post-operative cross-sectional area along the centerline of the aorta and arch was almost normalized, Figure 1. The pattern of flow during the cardiac cycle with a retrograde helix in the aortic arch, similar to normal, was observed in all cases with peak velocities of 1.1±0.2m/s. Aortic Shape and flow after surgery Conclusion We here describe a new technique, which at least in the short term appears to normalize the size, shape and pattern of flow in the arch. Long term studies are required to establish the value of this technique.

Diagnosis and surgical repair of congenital double aortic arch in infants

ObjectivesDouble aortic arch (DAA) is a rare congenital vascular malformation. This study aims to summarize the experience of diagnosis and surgical treatment for congenital double aortic arch.MethodsThe clinical data of 24 cases with double aortic arch (DAA) from January 2008 to January 2018 in our hospital was reviewed retrospectively.ResultsA total of 24 cases, including 12 patients with isolated DAA and 12 patients with DAA and associated intracardiac defects were identified. There were 14 males and 10 females, with an average age of 11 months. The associated intracardiac malformations included ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), tetralogy of Fallot (TOF), transposition of the great arteries (TGA), pulmonary stenosis (PS), and patent foramen ovale (PFO). Of the 12 patients with DAA and intracardiac malformations, 7 patients underwent intracardiac repair simultaneously, however, 3 patients underwent isolated double aortic arch correction. One patient with DAA and TGA underwent surgical correction of congenital vascular ring at the first stage, and the arterial switch operation was performed at the second stage. The clinical outcomes of 23 patients were promising, however, in one patient, parents decided not to do the surgery due to personal reasons. The average follow-up time was 35 months.ConclusionsTracheal and esophageal compression are commonly seen in patients with DAA, however could be relieved significantly after surgery. In particular cases, the simultaneous intracardiac defects repair could be performed. Misdiagnosis was easily established with isolated echocardiography. Fortunately, the correct diagnosis of DAA and associated intracardiac defects could be established with the use of combined chest computed tomography.

Invited Commentary

The surgical repair of arch obstruction dates back to the first report of successful repair of aortic coarctation by Clarence Crafoord in 1944 [1Crafoord C. Nylin G. Congenital coarctation of the aorta and its surgical treatment.J Thorac Surg. 1945; 14: 347-361Abstract Full Text PDF Google Scholar]. The spectrum of aortic arch obstruction extends from the simplest form with isolated coarctation to the most complex form of obstruction, which involves complete interruption of the aorta. Although little controversy exists about the technique to repair either isolated coarctation or aortic interruption, there is a tremendous amount of controversy about the surgical management of transverse aortic arch hypoplasia. Perhaps the most controversial area is surgical management of proximal transverse arch hypoplasia, which Gray and colleagues [2Gray W.H. Wells W.J. Starnes V.A. Kumar S.R. Arch augmentation via median sternotomy for coarctation of aorta with proximal arch hypoplasia.Ann Thorac Surg. 2018; 106: 1214-1219Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar] have described in their article. In dealing with arch hypoplasia, there is no commonly accepted way to describe the problem. Gray and colleagues [2Gray W.H. Wells W.J. Starnes V.A. Kumar S.R. Arch augmentation via median sternotomy for coarctation of aorta with proximal arch hypoplasia.Ann Thorac Surg. 2018; 106: 1214-1219Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar] use a proximal transverse arch diameter that is less than 50% of the diameter of the ascending aorta. Surgical lore suggests that an arch size that is the patient’s weight in kilograms plus one is an acceptable standard. Z scores are probably the most common means of determining deviation from normal, but even so, there are multiple different z score programs that exist that are not identical in their measurements. A similar degree of controversy exists regarding the surgical approach to transverse arch hypoplasia. Gray and colleagues [2Gray W.H. Wells W.J. Starnes V.A. Kumar S.R. Arch augmentation via median sternotomy for coarctation of aorta with proximal arch hypoplasia.Ann Thorac Surg. 2018; 106: 1214-1219Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar] champion a median sternotomy approach for their patients, but there are plenty of data to suggest that the relief of distal obstruction through a thoracotomy approach will allow for improved flow through the area of proximal hypoplasia and, ultimately, growth in this area [3Kotani Y. Anggriawan S. Chetan D. et al.Fate of the hypoplastic proximal aortic arch in infants undergoing repair for coarctation of the aorta through a left thoracotomy.Ann Thorac Surg. 2014; 98: 1386-1393Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar, 4Siewers R. Ettedgui J. Pawl E. TallmanT del Nido P. Coarctation and hypoplasia of the aortic arch: will the arch grow?.Ann Thorac Surg. 1991; 52: 608-613Abstract Full Text PDF PubMed Scopus (68) Google Scholar]. This controversy may be completely addressed only by a randomized controlled multiinstitutional trial. Ultimately, the ideal approach to the management of arch hypoplasia will be identified only after much longer-term follow-up. The goal of congenital heart surgery is not survival measured in months or years but in decades. The quality of that survival will also be critical in determining the best operative management, by taking into account such factors as the need for both operative and percutaneous reintervention, the need for further medical management, and detailed long-term neurodevelopmental outcomes in these patients. In particular, longitudinal neurodevelopmental outcomes are paramount to determining the success of this surgical procedure related to the consequences of the bypass technique [5Ohye R. Goldberg C. Donohue J. et al.The quest to optimized neurodevelopmental outcomes in neonatal arch reconstruction: the perfusion techniques we use and why we believe in them.J Thorac Cardiovasc Surg. 2009; 137: 803-806Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar]. In this paper, Gray and colleagues [2Gray W.H. Wells W.J. Starnes V.A. Kumar S.R. Arch augmentation via median sternotomy for coarctation of aorta with proximal arch hypoplasia.Ann Thorac Surg. 2018; 106: 1214-1219Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar] definitely present a safe and effective management strategy, but the quest to determine the ideal strategy remains elusive. Arch Augmentation via Median Sternotomy for Coarctation of Aorta With Proximal Arch HypoplasiaThe Annals of Thoracic SurgeryVol. 106Issue 4PreviewCoarctation of the aorta can be associated with hypoplasia of the proximal transverse aortic arch. One approach to manage this condition is via left thoracotomy and extended end-to-end anastomosis with the expectation that the proximal arch will grow over time. Our preferred approach is to augment the aorta via midline sternotomy. We hypothesized that this approach is safe, durable, and allows reliable growth of the aorta. Full-Text PDF

Efficacy of coarctation resection and aortoplasty with autologous pulmonary artery patch strategy for treating coarctation of the aorta combined with hypoplastic aortic arch in infants

Objective: To investigate the outcomes of coarctation resection and aortoplasty with autologous pulmonary artery patch for treating coarctation of the aorta combined with hypoplastic aortic arch in infants. Methods: Clinical data of 21 infants with coarctation of the aorta and hypoplastic aortic arch, who underwent coarctation resection and aortoplasty with autologous pulmonary artery patch in Fuwai hospital from January 2009 to June 2016 were retrospectively analyzed. The age of the patients was 4 (2, 5) months,and the body weight of the patients was (5.3±1.6) kg. The patients were followed up to observe the surgery effect. Results: No perioperative death and serious complications occurred. When the patients were discharged,the systolic blood pressure of the right upper limb was lower than the preoperative systolic blood pressure ((85.7±5.9) mmHg(1 mmHg=0.133 kPa) vs. (100.7±16.6) mmHg, P<0.001),and the systolic blood pressure of the right lower limb was higher than the preoperative systolic blood pressure ((98.7±13.3) mmHg vs. (85.6±20.8) mmHg, P<0.001). The pressure gradient of aortic coarctation detected by echocardiography was lower than the preoperative pressure gradient ((13.1±3.8) mmHg vs. (46.2±17.1) mmHg, P<0.001). No restenosis was detected by echocardiography at discharge. Follow-up data were obtained in 19 patients, and the follow-up time was 18 (8, 45) months.The patients grew well, and no death occurred. Restenosis occurred in 3 cases, 1 patient underwent aortic balloon dilatation and the remaining 2 patients were under follow up observation. Computed tomography angiography showed that the morphology of aortic arch was normal without signs of aortic aneurysm. Conclusion: Coarctation resection with autologous pulmonary artery patch aortoplastystrategy is considered as a safe and effective surgical method for management of infant coarctation with hypoplastic aortic arch, and this surgery method is related with satisfactory early and mid-term outcomes in this patient cohort.

Fate of the Hypoplastic Proximal Aortic Arch in Infants Undergoing Repair for Coarctation of the Aorta Through a Left Thoracotomy

Extended end-to-end anastomosis (EEEA) through a left thoracotomy for coarctation of the aorta (CoA) and tubular hypoplasia of the aortic arch (THAA) leaves an unaugmented hypoplastic proximal aortic arch (PAA) segment, which may increase late reintervention for PAA obstruction. We sought to assess PAA growth and reintervention for PAA obstruction after EEEA. Preoperative and follow-up echocardiographic images of 140 patients who underwent EEEA for CoA from 2005 to 2012 were reviewed. Patients were divided into two groups on the basis of preoperative PAA z-scores: THAA group, z-score less than-3; non-THAA group, z-score greater than or equal to-3. Eighty (57%) patients were identified as having THAA. There were three surgical reinterventions (PAA in 2patients and distal aortic arch in 1 patient) and nine catheter reinterventions (all related to anastomotic stenosis) during a median follow-up period of 18 months. Both patients who required PAA reintervention had preoperative PAA z-scores below-8. Freedom from reintervention at 3 years was comparable between the groups (THAA group, 90.0% vs non-THAA group, 87.9%, p= 0.483). Follow-up echocardiography revealed PAA catch-up growth in the THAA group (z-score, preoperative-4.63 vs follow-up-1.17, p<0.001); however, there was a nonsignificant trend toward smaller PAA in the THAA group (z-score: THAA,-1.17 vs non-THAA,-0.55, p= 0.057). All but 2 patients with preoperative PAA z-scores above-6 did not have any PAA obstruction. The hypoplastic PAA segment in patients with CoA/THAA grew significantly after EEEA but remained smaller than in those without THAA. Our data support that CoA and PAA with z-scores as small as-6 can be repaired through a thoracotomy approach with a low risk of reintervention.

Application of Modified Perfusion Technique on One Stage Repair of Interrupted Aortic Arch in Infants: A Case Series and Literature Review

One stage repair of interrupted aortic arch (IAA) associated with cardiac anomalies in neonates and infants is challenging for the entire surgical team. Deep hypothermic circulatory arrest (DHCA) prolongs myocardial and cerebral ischemia and may induce heart, brain, and major organ dysfunction. From May 2004 to May 2006, 13 infants with IAA underwent one stage repair by median sternotomy under DHCA with continuous regional cerebral perfusion (RCP) in Fuwai Children's Heart Center. Median age at operation was 10.4 +/- 6.7 months, and mean body weight was 6.58 +/- 2.15 kg. Temperature of nasopharynx was decreased to 18 degrees C-20 degrees C; rectal temperature was controlled at 19 degrees C-22 degrees C. Flow rate of RCP was maintained with 20-25 ml x kg x min under DHCA combined with RCP. Mean artery pressure (MAP) measuring from right radial artery was 32.5 +/- 5.8 mm Hg, and MAP from femoral artery was 11.2 +/- 3.5 mm Hg. Mean cardiopulmonary bypass (CPB) time was 141.6 +/- 21.7 min, and mean aortic clamp time was 52.3 +/- 10.9 min. Mean duration of RCP was 31.5 +/- 12.4 min. Mean intubation time in intensive care unit (ICU) was 54.7 +/- 12.6 hours, and mean ICU stay was 67.9 +/- 28.4 hours. This report describes our CPB protocol under DHCA using continuous RCP in low weight pediatric patients to minimize neurological complications during one stage IAA repair and summarizes the various CPB managements in recent literature as well.

Echocardiographic Diagnosis of Persistent Fifth Aortic Arch

We describe echocardiography and computerized tomographic imaging findings in the two subtypes of fifth aortic arch in infants. In the first form, the fifth aortic arch creates a systemic to systemic connection extending from the ascending to the descending aorta. When this form appears in isolation, it does not cause hemodynamic disturbance, although associated cardiac lesions, such as aortic coarctation or interruption and patent ductus arteriosus, may influence the clinical presentation. In the second form the fifth aortic arch connects the systemic and pulmonary circulations from persistent connections between the embryological fifth and sixth arches, which may cause significant hemodynamic disturbance from left to right shunting. Although this form has been associated with a wide array of congenital cardiac lesions, this is the first description of 5th aortic arch in association with atrioventricular septal defect and double outlet right ventricle.

Ontogeny of bone strain: the zygomatic arch in pigs

At the time of weaning, infant animals have little experience with hard food, and thus their skulls are not likely to be epigenetically adapted for the loads imposed by mastication. We examined bone strain in the zygomatic arch of 4-week-old weanling piglets. Functional strains in piglets differed from those previously reported for older pigs in that the squamosal bone was not bent in the horizontal plane and the principal tensile strain on the zygomatic bone did not correspond to the direction of masseter muscle pull. Strain patterns were more variable in piglets than in older pigs. In older pigs, masticatory strains can be reproduced by stimulating the masseter muscles. When the piglet masseter was stimulated, strain patterns were more similar to those of older pigs, but shear strain magnitudes were the largest yet recorded from mammalian skull bones, up to 4000 muepsilon. To put these findings in the context of skeletal adaptation, 45 dry skulls, including some animals from the strain study, were measured. Reduced major axis regressions indicated that the infant arch was rounder in cross section and straighter than that of older animals. With growth, the arch became dorsoventrally higher, while mediolateral thickness decreased in the squamosal bone. Overall, these changes should make strain more predictable, explaining the lower variability in older animals. Other factors likely to be important in causing unique strain regimes in piglets include (1) unfamiliarity with hard food, (2) greater importance of muscles other than the same-side masseter and (3) greater proximity of molariform teeth to the arch. Collectively, these data indicate that the skeleton is not pre-adapted for specific functional loads.

Interrupted aortic arch in infancy: a 10-year experience.

Fifty infants with interrupted aortic arch (IAA), admitted between 1979 and 1988, were reviewed. They usually presented early in severe cardiac failure or shock. In the initial 5-year period, 17 of the 21 infants underwent diagnostic or confirmatory cardiac catheterization, in contrast with the latter 5 years when only eight of the subsequent 29 patients underwent catheterization. Since 1987, all patients underwent surgery after cross-sectional echocardiography. Fifteen infants had a type A IAA and 35 had type B. All had associated cardiac anomalies. Four infants were not operated on. In the initial 5-year period, of 17 infants who were surgically treated, four had a one-stage total repair, the remaining had a two-stage repair with initial reconstruction of the arch and pulmonary artery banding. There was an overall surgical mortality of 65%, reflecting the precarious state of many of these infants before surgery with a significant contribution from unrelieved subaortic stenosis. In the latter 5-year period, 29 underwent surgery, 22 had a one-stage total repair. There were three deaths, all in infants whose active treatment was withdrawn. The outcome of the survivors has generally been good, subsequent surgery being mainly related to the associated anomalies (e.g., recurrent subaortic stenosis, conduit replacement). Over this 10 year period the greater accuracy of noninvasive diagnoses, and perioperative intensive care, have led to an improvement in the preoperative state of these infants. Single-stage total repair is our procedure of choice.

Association of distal displacement of the left subclavian artery and coarctation of the aorta.

Previously noted but undocumented observation of distal displacement of the left subclavian artery in patients with discrete coarctation of the aorta was verified by an objective two-dimensional echocardiographic method in 28 patients with aortic coarctation and in 43 control subjects. Relative position of brachiocephalic arteries to one another was evaluated by the ratio of the distance between the left common carotid and the left subclavian artery to the distance between the innominate and the left common carotid artery. Large distance between the left common carotid and the left subclavian artery was reflected by high value of the derived ratio. In neonates with aortic coarctation, the ratio was 1.69, SD +/- 0.66, compared to 1.04, SD +/- 0.40 in the control group. In older children this ratio was less discriminatory. We also observed that the left subclavian artery formed an acute angle (less than 90 degrees) with the proximal (upstream) segment of the aortic arch in infants with aortic coarctation. In all control infants, this angle was equal to or greater than 90 degrees. A corresponding necropsy study confirmed the echocardiographic findings. We conclude that distal displacement of the left subclavian artery is associated with coarctation of the aorta. It can be accurately visualized and objectively assessed by the two-dimensional echocardiographic technique proposed.

Severe Subaortic Stenosis in Interrupted Aortic Arch in Infancy and Childhood

Thirteen out of a total 50 infants with interrupted aortic arch (IAA) seen between 1979-1988 had or developed severe subaortic stenosis (SAS). One had type A interruption and 12 type B. All had a large ventricular septal defect (VSD). The infundibular septum was displaced posteriorly in eight infants, severely narrowing the left ventricular outflow tract (LVOT). Three had fibromuscular narrowing of the LVOT, one each a subaortic muscle bar and membrane. The aortic root and subaortic area were small and measured between 3-8 mm. An anomalous right subclavian artery was noted in eight of the 12 type B IAA, the anomalous vessel arising from the descending aorta in seven. Thirteen infants with IAA and SAS were submitted to surgery, 12 having their subaortic area resected with three perioperative deaths early in the series and three late deaths where active treatment was ceased, including one infant with renal dysplasia who had had a successful establishment of arch continuity and pulmonary artery banding. Of the seven survivors, six have residual Doppler gradients of between 20-50 mmHg, two requiring a second resection and one a third resection. Alternate management programs are suggested based on anatomical evaluation and echocardiography. Any newborn presenting with IAA requires careful evaluation of the subaortic area, best seen on cross-sectional echocardiography. The improved surgical survival in infants following complete repair of IAA has led this important associated anomaly of subaortic stenosis to assume greater importance as a cause of mortality and long-term morbidity.

Determinants of survival following repair of interrupted aortic arch in infancy

Between January 1971 and March 1987, surgery was performed in 26 infants with interrupted aortic arch. At operation the 14 boys and 12 girls weighed between 1.71 and 4.23 kg (mean ± SD = 3.1 ± 0.63 kg) and ranged in age from 2 to 90 days (13 ± 18 days). The interruption was distal to the left subclavian artery in 4 (15%), between the left carotid and subclavian arteries in 20 (77%) and between the brachiocephalic (innominate) and left carotid arteries in 2 (8%). Associated complex cardiac lesions in 8 patients included complete transposition (2), common arterial trunk (2), aortopulmonary window (2), double inlet left ventricle (1) and tricuspid atresia (1). The remaining patients had an isolated ventricular septal defect. The arch was reconstructed with a prosthetic conduit in 14 patients; by a direct anastomosis in 6; using the subclavian artery in 3; and with the pulmonary trunk and the arterial duct in 2. Twenty patients (77%) underwent palliative surgery as the first stage of management, and banding of the pulmonary trunk was also performed in 16 of these. Five patients (19%) underwent primary complete repair of the interruption and intracardiac anomalies. One patient (4%) died soon after thoracotomy for palliative surgery. Of the 15 (57%, 70% confidence limits CL = 46–69%) early deaths, 7 occurred in patients with complex associated defects and 4 occurred when single stage repair was attempted. Survival following first-stage palliative surgery for arch interruption with isolated ventricular septal defect was 64% ( 9 14 ) [70% CL = 47–79%]. All of these patients subsequently underwent complete repair. Chi-squared and t-tests showed the year of operation and the type of operation (two-stage repair) to be associated with improved survival. It is concluded that a two-stage repair of interrupted aortic arch offers a reasonable alternative to primary complete correction and will lead to satisfactory subsequent repair in most cases.

左室流出路形態からみた大動脈離断に対する手術成績の検討

左室流出路形態からみた大動脈離断に対する手術成績の検討

Repair of interrupted aortic arch in infancy

Twenty-one patients undergoing repair of interrupted aortic arch between December of 1979 and January of 1987 were reviewed to determine the cause(s) of late morbidity and mortality. Ten underwent staged repair, and 11 had complete repair including all coexisting defects at the initial operation. Sixty-two percent are alive and clinically well 6 months to 6 years after the initial operation. Among the five patients who died late postoperatively, four had severe left ventricular outflow tract obstruction. Two other patients have had surgical relief of severe subaortic stenosis. In addition, significant recurrent or residual coarctation was found in four patients; it was relieved by balloon angioplasty in two patients, and two had surgical repair. None of the most recent seven patients, however, have had a residual ascending-descending aortic gradient. Careful follow-up for the detection of previously masked or newly developed left ventricular outflow tract obstruction is imperative and may be lifesaving.

Successful surgical repair of truncus arteriosus with interrupted aortic arch in infancy by an anterior approach

A 98-day-old infant was successfully operated on for truncus arteriosus (type I) with interrupted aortic arch (type B) using a one-stage anterior approach. The interrupted aortic arch was reconstructed by direct anastomosis between the ascending and descending aorta. The ventricular septal defect was closed, and a 12-mm porcine valved conduit was placed on the right side of the ascending aorta to establish continuity between the right ventricle and pulmonary artery. Surgical procedures for the repair of this malformation are the focus of discussion.