Interrupted Aortic Arch Type Research Articles

Case report: interrupted aortic arch

Introduction: Interrupted aortic arch (IAA) is one of the congenital heart diseases characterized by a complete anatomical and luminal disruption between the ascending and descending aorta. It is frequently accompanied by other congenital heart defects, such as PDA and VSD. IAA in adults is exceptionally rare. The presence of collateral circulation and PDA are key factors for patient survival. Thorax CTA is an excellent modality for imaging and diagnosis, ensuring that appropriate treatment can be carried out. Case Presentation: An 18-year-old male patient presented with complaints of worsening shortness of breath 10 days prior to the examination. The shortness of breath has been experienced since school age, often occurring during exercise and strenuous activity. Thorax CTA results indicated IAA type A subtype 1 according to Celoria and Patton classification, PDA type A according to Krichenko classification, perimembranous VSD, cardiomegaly, and pulmonary hypertension. Conclusion: IAA in adults is exceptionally rare. Adult IAA patients may exhibit no symptoms or may present with symptoms of CHF. Thorax CTA is one of the modalities employed to confirm the diagnosis of IAA. It can visualize the interruption and separation between the ascending and descending aorta, as well as the associated anomalies. The presence of a PDA and VSD ensures that the descending thoracic aorta distal to the defect continues to receive some oxygenated blood from the heart. Thorax CTA plays a pivotal role in establishing cases of IAA, as it helps clinicians to make a quick and accurate diagnosis.

Exploring the Iceberg: Detection and Management of Isolated Interrupted Aortic Arch and Associated Hypertension- A Rare Entity

Objectives: Prompt diagnosis and management of hypertension, particularly in younger patients, are crucial to reducing complications, mortality, and expenses. A thorough history and careful physical examination are key steps. This article illustrates one such unusual case that had a subtle presentation and went undiagnosed until it was brought to our attention.
 Methodology: A 30-year-old male patient with a history of fatigue, poor exercise tolerance, and mild headaches was diagnosed with hypertension. He was commenced on amlodipine and had no family history of cardiac diseases or secondary hypertension. The patient appeared weak and thin, with a BMI of 16.6 kg/m2. His radial pulse was regular and 98 bpm, and his brachial blood pressure was recorded at 190/90 mmHg. Further examination revealed weak lower limb pulses, a radio-femoral delay, and an ankle blood pressure of 100/60 mmHg. A 3/6 systolic murmur was noted at the aortic area. Routine laboratory investigations revealed a chest x-ray showing notching of the ribs and an absent aortic knob. A transthoracic echocardiogram was performed, but it did not provide conclusive results. A CT angiography of the chest revealed a complete interruption of the aorta just below the left subclavian artery in a "rat tail fashion." The aortic valve was normal, and no other aortic or cardiac pathology was found. The patient was diagnosed with IAA type A and surgical correction was suggested. Antihypertensive medication was given to achieve blood pressure control, and surgical correction was done involving end-to-end anastomosis. The patient's postoperative period was uneventful, and at four weeks, his symptoms subsided, and his blood pressure was 130/80 mmHg without antihypertensive medications.
 Results: The patient had an interrupted Aortic Arch type A and underwent treatment with antihypertensive medications and surgical correction. After a month, symptoms subsided, blood pressure was 130/80 mmHg, and pulses were bilaterally normal. Post-op CT Aortogram showed good anatomic continuity and no complications.
 Conclusion: Mild symptoms might only be the tip of an iceberg, calling for further exploration. IAA, despite its rarity, should be considered a potential cause of hypertension.

Primary outcomes of one-stage repair for interrupted aortic arch at Vietnam National Children’s Hospital

Objective: This study focuses on primary outcomes of one-stage repair for interrupted aortic arch (IAA) from a single institution from Vietnam. Methods: Between 2012 and September 2017, 27 patients underwent one-stage repair in Children Heart Center, National Hospital of Pediatrics. Kaplan-Meier survival analysis plotted survival and freedom from reoperation curves from follow-up data. Results: Median age was 53 days (range, 9 days to 5 months) and weight was 3.5 kg (range, 2.3 to 6.5 kg). IAA type A in 16 cases (59.3%), IAA type B in 11 case (40.7%). Associated anomalies were ventricular septal defect in 20 (74.1%), aortopulmonary window in 4 (14.8%), truncus arteriosus in 3 (11.1%). Fourteen patients (51.9%) have left ventricular outflow tract obstruction (LVOTO). Median follow-up was 17 months (range, 1 day to 71 months). Survival rates at 30 days, 1 year and 5 years were 81.5%, 77,6% and 77,6% respectively. There were 5 early deaths in hospital, 4 deaths related to nosocomial infection with positive results in blood culture or endotracheal tube, the other was sudden death. One late death after 4 months was caused by severe pulmonary artery stenosis after total repair of interrupted aortic arch associated with truncus arteriosus. There were 5 late reoperations or balloon angioplasty, reoperation for LVOTO in 3, right ventricular outflow tract reconstruction in 1, the other required balloon angioplasty for recurrence of aortic arch stenosis. Conclusion: Primary outcomes of one-stage repair for interrupted aortic arch are safe in our institution. Long-term outcomes evaluation is needed for this complex congenital heart disease.

Outcomes of One-Stage Surgical Repair for Berry Syndrome in Neonates.

BackgroundBerry syndrome is a challenging disease for surgeons to make early diagnosis and successful surgical correction in the neonatal period. Here, we summarized the clinical features of three neonates with berry syndrome in our center to optimize the therapeutic effect in the future.MethodsFrom January 2014 to December 2019, three neonates with berry syndrome underwent one-stage surgical repair in our center. We mainly used two different surgical techniques to repair it, and collected clinical data retrospectively from hospitalization history, outpatient records, and telephone follow-up.ResultsThe age at operation was 28, 8, and 8 days and the body weight was 3.65, 3.86, and 3.0 kg, respectively. The morphology of the interrupted aortic arch (IAA) was type A in two patients and type B in one patient. The aortopulmonary window (APW) morphology was type IIa, III, and IIb, respectively. The phenotype of the IAA type B combined with APW type III in our second patient was reported for the first time so far. All patients survived and were followed up to date. The second patient using intra-aortic baffle experienced twice reoperation for right pulmonary artery (RPA) stenosis. All patients grew well so far.ConclusionOnce diagnosed in the neonatal period, berry syndrome can be safely corrected by one-stage surgical repair in experienced cardiac centers. Considering the variability of the location where the RPA arises from the posterior wall of the aorta, it is difficult to find the best surgical method for each patient.

Bronchial compression as adverse effect of right pulmonary artery stenting in a patient with truncus arteriosus communis and interrupted aortic arch: a case report

Aortic arch obstruction and bronchial compression are possible post-operative complications in patients with truncus arteriosus communis (TAC) with interrupted aortic arch (IAA). We present a case of bronchial compression as adverse effect of right pulmonary artery (PA) stenting in an infant with TAC (type 4A)-IAA (type B) repair. Due to growth potential, self-expandable metal stent was applied for postoperative proximal right PA stenosis, which caused bronchial compression. Later, we found patient having bronchomalacia. Surgical removal was needed. Stent insertion might be an option for postoperative PA stenosis, but bronchial compression might be a potential risk particularly in patients with bronchomalacia.

Growing potential of small aortic valve with aortic coarctation or interrupted aortic arch after bilateral pulmonary artery banding.

Bilateral pulmonary artery banding (bPAB) is utilized for some patients with a ventricular septal defect (VSD) and aortic coarctation (CoA) or interrupted aortic arch (IAA). We evaluated aortic valve (AoV) diameter and patient outcomes following bPAB. Between August 2010 and September 2015, 10 consecutive patients with VSD and patent ductus arteriosus-dependent CoA or IAA underwent bPAB because of an AoV diameter of approximately <50% of the normal value (n = 6), severe subaortic stenosis and poor patient condition (n = 1, respectively), or low birthweight (n = 2). Second-stage operations were conventional total repair in five and Damus-Kaye-Stansel anastomosis, aortic arch reconstruction and right ventricle-pulmonary artery shunt (modified Norwood) type repair in five. After modified Norwood-type repair, four patients were Yasui-type repair candidates and one was a Fontan candidate. For all patients, the mean AoV diameter increased from 3.7 ± 0.7 mm before bPAB to 4.6 ± 0.8 mm before the second-stage operation. In five patients with CoA or IAA type A, the AoV diameter significantly increased from 3.5 ± 0.3 mm to 4.5 ± 0.5 mm during the term between bPAB and the second-stage operation, with an AoV Z-score increase from -5.82 ± 0.92 to -4.28 ± 0.86. IAA type B showed a slight increase in the AoV diameter. Initial palliation with bPAB enables AoV diameter growth in some patients, improving the likelihood of conventional total repair adaptation rate, particularly for CoA or IAA type A.

Prenatal Diagnosis of Fetal Interrupted Aortic Arch Type A by Two-Dimensional Echocardiography and Four-Dimensional Echocardiography with B-Flow Imaging and Spatiotemporal Image Correlation.

Fetal interrupted aortic arch (IAA) is a rare cardiac anomaly and its prenatal diagnosis is challenging. The purpose of our report is to evaluate the use of two-dimensional echocardiography (2DE) and 4D echocardiography with B-flow imaging and spatiotemporal image correlation (4D BF-STIC) in detecting IAA type A (IAA-A). Twenty-three cases of confirmed IAA-A identified by fetal echocardiography were involved in the study. The fetal echocardiography image data were reviewed to analyze the ratio of right ventricle to left ventricle (RV/LV) diameter, the ratio of main pulmonary artery to ascending aorta (MPA/AAO) diameter, and the correlation of RV/LV diameter ratio and size of ventricular septal defect (VSD). 4D BF-STIC was performed in 21 fetuses using the sagittal view (4D BF-STIC-sagittal) and the four-chamber view (4D BF-STIC-4CV) as initial planes of view. An additional 183 normal fetuses were also included in our study. RV/LV and MPA/AAO ratios were calculated and compared with that of IAA-A fetuses. Fetal 2DE, 4D BF-STIC-sagittal, and 4D BF-STIC-4CV were used to visualize the aortic arch and its associated neck vessels. Six subgroups were evaluated according to gestational age. Fetal 2DE, 4D BF-STIC-sagittal, and 4D BF-STIC-4CV made the correct prenatal diagnosis of IAA-A in 19/23 (82.6%), 14/21 (66.7%), and 19/21 (90.5%) of patients, respectively. A significantly enlarged MPA combined with symmetric ventricles was found in the IAA-A fetuses, while the size of the VSD was negatively correlated with RV/LV ratio. 4D BF-STIC-sagittal and 4D BF-STIC-4CV were better than traditional 2D ultrasound in detecting the aortic arch and neck vessels between 17 and 28 gestational weeks and 29 to 40 gestational weeks in normal fetuses. It is demonstrated that IAA-A could be diagnosed by traditional fetal echocardiography, while 4D technique could better display the anatomic structure and the spatial relationships of the great arteries. Use of volume reconstruction may promote its clinical usage and help prenatal diagnosis.

SURGICAL TREATMENT OF FIVE PATIENTS WITH INTERRUPTED AORTIC ARCH AND AORTOPULMONARY WINDOW

ObjectivesTo summarised review the surgical experience of single stage repair of interrupted aortic arch (IAA) associated with aortopulmonary window (APW) in five patients.MethodsBetween December 1997 and December 2009, five patients...

Ross-Konno procedure six years after correction of interrupted aortic arch type B

Introduction: Interrupted aortic arch (IAA) is frequently associated with ventricular (VSD) or atrial septal defect (ASD) as well as other anomalies of the left ventricular outflow tract (LVOT) like vavular or subvalvular aortic stenosis. We present a video of a Ross-Konno procedure performed on a six year old boy, who underwent previously a correction of an IAA type B combined with VSD and ASD closure postnatal and two resections of subvalvular aortic stenoses at the age of one and two years.

Fetal interrupted aortic arch: 2D‐4D echocardiography, associations and outcome

To analyze fetal two-dimensional (2D) echocardiographic characteristics of interrupted aortic arch (IAA) and its different types, to explore whether the use of 4D ultrasound with B-flow imaging and spatiotemporal image correlation (STIC) can improve prenatal diagnostic accuracy, and to describe associations and outcome. The study comprised IAA fetuses examined exclusively by 2D conventional echocardiography during the period from 1994 to 2003, and those identified by conventional echocardiography and examined further by 4D ultrasound with B-flow imaging and STIC during the period January 2004 to July 2008, identified among fetuses examined at two referral centers for congenital heart defects (CHD). Postnatal follow-up was available in all cases. Karyotyping and fluorescent in-situ hybridization (FISH) analysis for the DiGeorge critical region (22q11.2) were performed in all cases. Twenty-two cases of isolated IAA (15 Type B and seven Type A, seven and three of which, respectively, underwent B-flow imaging and STIC) were detected among 2520 cases of fetal CHD. In seven of the 15 Type B cases, a right subclavian artery arose anomalously (ARSA). 2D echocardiography failed to distinguish the type of IAA in only two cases and the ARSA in five of the seven cases. B-flow imaging and STIC successfully identified IAA types in all 10 cases examined and clearly visualized the origin and course of the ARSA, including cervical ones. FISH detected 22q11.2 microdeletion in 10 of the 15 Type B cases and an unusual association with Type A in one of the seven cases. Fetal/neonatal outcome included: eight terminations of pregnancy, one intrauterine death and four postoperative deaths in the neonatal period, and nine neonates were alive after surgery at a mean follow-up time of 58 months (range, 4 months-13 years). Our results confirm the feasibility of prenatal characterization of IAA and its different types based on 2D echocardiographic examination, albeit with some limitations in the thorough assessment. 4D ultrasound with B-flow imaging and STIC can apparently facilitate visualization and detailed examination of the anatomical features of the IAA types, including visualization of the neck vessels, thus supplying additional information with respect to 2D sonography. As for the known association with microdeletion 22q11.2, our data indicate that Types A and B are distinct, there being a close association only with IAA Type B.

Chromosome 22q11.2 microdeletion in children with conotruncal heart defects: frequency, associated cardiovascular anomalies, and outcome following cardiac surgery

The association of conotruncal heart defects with 22q11.2 chromosomal microdeletions is well established. However, it is not clear whether particular types of conotruncal malformations or additional cardiovascular anomalies are associated with microdeletions. In addition, cardiac surgery outcome in children with conotruncal defects and del22q11.2 is not well described. We prospectively enrolled 214 children with conotruncal defects: 126 with tetralogy of Fallot (TOF), 18 with pulmonary atresia-ventricular septal defect (PA-VSD), 15 with truncus arteriosus communis (TAC) type I, one with interrupted aortic arch (IAA) type B, and 54 with the transposition of great arteries, who were consecutively hospitalized at the Pediatric Cardiology Department between 2003 and 2005. 22q11.2 microdeletion was identified by fluorescence in situ hybridization. The postoperative course following cardiac surgery was compared in patients with TOF and its more severe form, PA-VSD, with/without del22q11.2 (groups A and B) and TAC with/without del22q11.2 (groups C and D). In 15 of 214 patients, 22q11.2 microdeletion was diagnosed (in 11 with TOF/PA-VSD, in three with TAC, in one with IAA type B). In patients with TOF/PA-VSD and microdeletion anatomic features that were significantly associated with 22q11.2, deletion included right aortic arch (p = 0.018), aberrant right subclavian artery (p < 0.001), and major aortopulmonary collateral arteries (p = 0.016). A complicated postoperative course was more frequent and mortality was higher in patients with conotruncal defects and with/without microdeletion. We conclude that additional cardiovascular anomalies are significantly more frequent in children with 22q11.2 microdeletion and TOF/PA-VSD. Children with conotruncal heart defects and 22q11.2 microdeletion more frequently experienced complicated postoperative course after cardiac surgery.

A rare association of interrupted aortic arch type C and microdeletion 22q11.2

Microdeletion 22q11.2 is associated with a variety of findings, and the most common are cardiac defects. It is very frequently associated with interrupted aortic arch (IAA) type B and very rarely with type A and type C. Here we report the first case of IAA type C associated with 22q11.2 deletion in Serbia and, to the best of our knowledge, the fourth case described worldwide so far. By this report we would like to point out that all patients with IAA type C who have additional features specific for 22q11.2 microdeletion syndrome should be screened for the presence of this deletion.

A Genetic Etiology for Interruption of the Aortic Arch Type B

Interrupted aortic arch (IAA) type B is a congenital heart defect believed to be caused by an anomaly of branchial arch mesenchymal development. IAA type B has been associated with DiGeorge syndrome (DGS), which includes conotruncal heart defects, T-cell immunodeficiency, hypocalcemia, and facial abnormalities. The great majority of DGS cases are associated with hemizygous deletions at the chromosome 22q11 locus. The present study was designed to establish the involvement of the 22q11 locus in the etiology of IAA type B, independently from the typical DGS phenotype. An evaluation was performed on 73 patients with conotruncal heart defects using fluorescence in situ hybridization (FISH) analysis with probes from the 22q11 DGS locus. From this group, 7 patients were deleted (including 4 of the 11 patients with IAA type B). FISH analysis was extended to a total of 22 patients with IAA type B and 11 of these (50%) were deleted. FISH and Southern blot analyses using additional markers within the DiGeorge chromosomal region were performed on patients found not to be deleted in the initial FISH screening. No small deletions or rearrangements were detected. In our patient population, a single, specific genetic defect is the basis for one half of the IAA type B cases. These data suggest that IAA type B is one of the most etiologically homogeneous congenital heart defects. A 22q11 deletion in IAA type B may or may not be associated with the typical DGS phenotype. Therefore, IAA type B, per se, should be an indication for 22q11 deletion testing.

Repair of aortic arch interruption by direct anastomosis.

Evaluation of surgical treatment of interrupted aortic arch (IAA) by direct anastomosis. A consecutive series of 17 infants with IAA (type A in eight patients, type B in nine) were operated upon. The mean age at arch repair was 1.0 month (range 0.2-7.7), mean weight was 3.7 kg (range 2.2-6.2). All arch repairs were done by direct anastomosis. This included a persistent arterial duct in one and a subclavian turnup in another case. The aortic reconstruction included reimplantation of a lusoric artery in three patients, patch enlargement of the ascending aorta in three and of the complete arch in one patient. The arch repair was done through a lateral thoracotomy in three patients. In 14 patients the aortic repair was part of a single-stage approach through a median sternotomy using cardiopulmonary bypass and circulatory arrest. There was no operative mortality. One patient (single-stage approach) died 2 days after operation due to respiratory problems caused by tracheobronchomalacy. One patient (lateral approach) died suddenly 3 months after aortic repair and banding. Median follow up was 4.8 years (range 0.1-12.9). In five patients restenosis of the aortic arch developed, all within 1.5 years after repair. This was not correlated with the type of interruption, weight at operation, age at operation or the surgical approach. The actuarial freedom from restenosis was 61% at 5 years with a 70% confidence limit (CL70%) of 46-75. All restenoses were balloon dilated, but two needed redo surgery, which was done by the median approach. In three patients discrete subaortic stenosis developed. This was not correlated with the type of interruption, weight at operation, age at operation or the surgical approach. The actuarial freedom from subaortic stenosis was 68% at 5 years (CL70% = 54-83). These stenoses were treated by enucleation, followed in one patient by a pulmonary autograft procedure for recurrent root stenosis after another year. At the end of follow up all patients were thriving well, lacked symptoms, were normotensive and had normal femoral artery pulsations. IAA can be treated well with primary anastomosis. Possible restenosis of the aortic arch can adequately be treated by percutaneous balloon dilatation or redo surgery if necessary. Arch repair by median single-stage approach has our preference.

One stage repair of aortic arch anomalies and intracardiac defects

Between August 1985 and May 1990, 27 neonates and infants underwent combined correction of intracardiac and aortic arch anomalies through a median sternotomy. Coarctation (CoA) was combined with VSD (6), AVSD (2), Taussig-Bing (TB) heart (5), transposition of the great arteries (TGA) (1), TGA + VSD (2), congenitally corrected TGA + VSD (1) and VSD + myxoid stenotic outlet valves (1). Interrupted aortic arch (IAA) was combined with VSD (10) and TB heart (1). Two patients had IAA type B as well as CoA. Age at operation varied from 2 to 243 days with a mean age of 51 days. Twenty patients (70%) were younger than 30 days. One TGA + VSD and all TB hearts had maligned outlet septum and right ventricular outflow tract obstruction (RVOTO). Posterior outlet septum deviation and left ventricular outflow tract obstruction (LVOTO) occurred in 8 patients with malalignment VSD and IAA (7) or CoA (1). Aortic arch reconstruction was performed using extended end-to-end anastomoses. In 3 patients, arch hypoplasia necessitated patch implantation. VSDs were closed through the right atrium unless the anatomy dictated otherwise. One TB heart was reconstructed with a Kawashima procedure. All other TB hearts and all TGAs were corrected with arterial switch operation. Obstructing outlet septum was resected whenever necessary. Follow-up was complete and included echo-Doppler control. Eleven patients had postoperative heart catheterisation. Early mortality was 18.5% (5 patients). Persisting LVOTO or RVOTO was responsible. There was no late mortality. Five patients were reoperated upon: 3 for stenotic anastomoses and 2 for a subaortic membranous stenosis. Successful balloon dilatation of recoarctation was performed four times.(ABSTRACT TRUNCATED AT 250 WORDS)

Repair of Truncus Arteriosus and Interrupted Aortic Arch

A total of seven patients with truncus arteriosus and interrupted aortic arch (IAA) comprises our surgical experience in this condition. All underwent primary complete repair via median sternotomy between June 1985 and December 1989. Median age at repair was 8 days and median weight, 3.2 kg. Anatomy of these seven patients was truncus arteriosus type "1 1/2" in five patients and type II in two patients, IAA type B in six patients and type A in one patient. Aortic arch was reconstructed by direct anastomosis of ascending aorta and descending aorta. Right ventricle to pulmonary artery continuity was established with a porcine valved conduit in four patients, aortic homograft in two, and aortic homograft monocusp patch in one. Three patients have required five reoperations (three in one patient). One reoperation was due to compression of the left main bronchus from the reconstructed aorta, one was due to obstruction of the aorta at the site of IAA repair, and one was due to compression of the left main bronchus, right pulmonary artery, and residual stenosis across the hypoplastic ascending aorta. There were no early or late deaths and all seven survivors are currently well with a mean follow-up of 29 months from initial repair.

Primary definitive repair of interrupted aortic arch with ventricular septal defect

The optimal surgical management (primary or staged repair) of interrupted aortic arch (IAA) with ventricular septal defect (VSD) remains to be determined. A consecutive series of 14 neonates, aged 3-18 days (mean: 10 +/- 6 days) underwent primary complete repair. Mean weight was 3.3 +/- 0.4 kg. Eleven patients had IAA type B, 2 had type A and 1 had type C. Six infants had the Di George syndrome. Preoperative management (mean: 5 +/- 4 days) included prostaglandin E1 (14/14), intubation and ventilation (13/14), and inotropic support (11/14). Surgery was performed under deep hypothermia and circulatory arrest and involved resection of all ductal tissue, direct end-to-side aortic arch anastomosis and patch closure of the VSD. There were 2 early deaths (14%, 70% CL: 5%-31%): low cardiac output (1), residual VSD (1). Four patients (33%, 70% CL: 13%-52%) underwent reoperation for recurrent aortic obstruction (3 patients, 1 death) or left ventricular outflow tract obstruction (LVOTO) (1 patient). The results improved with time: no death and no recurrent aortic obstruction in the last 8 patients. At last follow-up (11 patient, mean follow-up = 24 +/- 9 months), all patients were free of cardiac symptoms; none had persistent aortic obstruction; 4 had LVOTO (gradient greater than 20 mm Hg) and 1 (with the Di George syndrome) had severe mental disorders. Primary complete repair provides satisfactory results in most infants born with IAA and VSD. An adequate direct aortic arch anastomosis should entail a low risk of recurrent obstruction. LVOTO develops in many cases and may require further surgery.

Reparative operations for interrupted aortic arch with ventricular septal defect

From January, 1975, through September, 1982, 24 infants underwent primary or staged repair of interrupted aortic arch (IAA) with ventricular septal defect (VSD). Seven patients had IAA type A and 17 patients had type B. Eleven of the patients, median age 5 days, underwent staged operations and 13 infants, median age 6 days, underwent primary repair. Palliation was by tube graft interposition (six), subclavian-aortic anastomosis (three), left carotid-aortic anastomosis (one), or end-to-side aortic anastomosis (one) combined with pulmonary artery banding (eight) or early VSD closure. With palliation, there were three (27%) early deaths among the eleven patients and one (13%) late death among the eight remaining. Delayed repair at 5 days to 14 months (median 7 months) in seven patients incurred three (43%) early and no late deaths. Primary repair in 13 patients consisted of VSD closure combined with graft interposition (12) or end-to-side aortic anastomosis (one), with three (23%) early and no late deaths. Nine of 14 survivors had hemodynamic evaluation by catheterization 1 to 3 years following repair. None had a significant residual VSD or pressure gradients between the ascending and thoracic aorta. Six had subaortic stenosis, two mild (gradient less than 20 mm Hg) and four severe (gradient greater than 50 mm Hg), necessitating operation. Results of operations in neonates with IAA continue to improve. Essential in management is an awareness that subaortic stenosis and hypocalcemia may be accompaniments of this anomaly. Based on these data, we prefer primary repair for IAA with VSD.