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J Thorac Cardiovasc Surg 1996;111:348-358
© 1996 Mosby, Inc.
SURGERY FOR CONGENITAL HEART DISEASE |
Los Angeles, Calif.
Received for publication March 2, 1995. Accepted for publication April 26, 1995. Address for reprints: Giovanni Battista Luciani, MD, Division of Cardiothoracic Surgery, University of Verona, O.C.M. Piazzale Stefani 1, Verona, 37126, Italy.
Abstract
Background: One-stage repair of interrupted aortic arch, ventricular septal defect, and severe subaortic stenosis represents a surgical challenge. Techniques that use extracardiac conduits to bypass the subaortic area or involve transaortic or transatrial resection of the conal septum have shown limitations and have failed to reduce the high mortality rate associated with subaortic obstruction.Methods and results: A new operative approach was used in nine neonates (2.1 to 3.9 kg) who underwent one-stage repair of interrupted aortic arch (type B, eight patients; type C, one patient), ventricular septal defect, and severe subaortic stenosis. All patients had severe subaortic stenosis according to preoperative echocardiography (mean ratio of subaortic to descending aortic diameter, 0.63 ± 0.08). With a transpulmonary (seven patients) or transatrial (two patients) approach and without resection of the conal septum, the ventricular septal patch was placed on the left side of the septum to deflect the conal septum anteriorly and away from the subaortic area. There were no early or late deaths. Median intensive care unit and hospital stays were 17 days (6 to 47 days) and 21 days (10 to 55 days), respectively. On follow-up echocardiography (1 to 29 months, median 12 months), no patients had significant residual subaortic obstruction and one patient had mild residual arch obstruction (20 mm Hg). Growth of the subaortic region was demonstrated in all patients (mean ratio of subaortic to descending aortic diameter, 1.20 ± 0.10; <0.001).Conclusions: Relief of severe subaortic stenosis during one-stage neonatal repair of aortic arch interruption and ventricular septal defect can be accomplished successfully without resection of the conal septum.
Interrupted aortic arch (IAA) with ventricular septal defect (VSD) is an uncommon lesion, accounting for 1.5% of all congenital heart disease. The prognosis for untreated infants is extremely unfavorable, with at least 90% dying within the first year after birth.
1 Since the first reported successful palliative procedures, the recent advances in neonatal cardiac surgery have made it feasible to perform anatomic repair of IAA-VSD within the first month after birth. 2
Critical subaortic stenosis (SAS) as a result of posterior malalignment of the conal septum, frequently associated with IAA-VSD, 1 presents a complex surgical management problem. In most series, subaortic obstruction accounts for the majority of early and late deaths and necessary reoperations. 2-9 Significant controversy persists regarding the optimal surgical treatment of SAS. 2 Staged approaches that addressed the IAA first and then the intracardiac defects often proved unsuccessful. 6 A series of palliative and reparative procedures that used extracardiac conduits aimed at bypassing the level of left ventricular outflow tract (LVOT) obstruction resulted in considerable rates of early mortality and need for reoperation. 6,8,10-12 Promising early results were later reported with techniques that entailed resection of the posteriorly displaced conal septum to minimize SAS. 13,14 Nevertheless, the risk of aortic valve injury and the technical problems connected with transaortic or transatrial resection of the conal septum were not negligible in these reports. 2,14 Further, these techniques did not result in improved early and late survival. 2
At Childrens Hospital Los Angeles, the approach to IAA-VSD was that of primary repair at the time of presentation. We present our results with a novel surgical technique for the treatment of neonates with IAA-VSD and critical SAS, which consists of transpulmonary approach to the VSD and subaortic region without resection of the posteriorly displaced conal septum.
Methods
Patients
Between July 1992 and January 1995, 24 neonates (<30 days of age) at Childrens Hospital Los Angeles were found to have IAA-VSD. All underwent surgical reconstruction of the aortic arch within the first month after birth, either as part of a biventricular repair (IAA-VSD, 11 patients; IAA–atrioventricular septal defect, five patients; IAA–truncus arteriosus communis, two patients; IAA–double-outlet right ventricle, two patients) or as the first-stage procedure toward a definitive univentricular palliation (IAA–VSD–hypoplastic left ventricle, three patients; IAA–double-outlet left ventricle, one patient). We report here on all consecutive patients who had IAA, malalignment VSD, and critical SAS.
A retrospective review of the patients' clinical, laboratory, and surgical data was conducted. The data gathered included the following: (1) anatomic diagnosis, (2) Apgar scores at birth, (3) age and weight at the time of surgical repair, (4) associated congenital anomalies, including DiGeorge syndrome, (5) lower-limb preoperative transcutaneous arterial oxygen saturation, (6) duration of preoperative and postoperative mechanical ventilatory and inotropic support, (7) preoperative and postoperative echocardiographic data, (8) surgical method of the one-stage IAA-VSD-SAS repair, (9) duration of cardiopulmonary bypass and deep hypothermic circulatory arrest, (10) postoperative duration of intensive care unit stay and total hospital stay, with medications on discharge, (11) postoperative complications and additional procedures to treat associated anomalies, and (12) follow-up data, including clinical status, medications, and echocardiographic findings.
Prematurity was defined as birth before the 36th week of gestation. Hypocalcemia was defined as a serum calcium ion level of <4.0 mg/dl, as measured in three consecutive random blood samples. Complete DiGeorge syndrome was defined as the coexistence of dysmorphic facial features (including hypertelorism, carp-shaped mouth, micrognathia, and notched ear pinnae) with hypocalcemia, low (<400 cells/mm 3) T-lymphocyte CD4- count, and IAA. Partial DiGeorge syndrome was defined as the coexistence of the dysmorphic facial features with hypocalcemia and IAA in the absence of any deficit of the CD4+ T-lymphocyte subpopulation. 15
Echocardiographic methods
All patients underwent transthoracic two-dimensional and color Doppler echocardiographic examinations with a Sonos 1000 machine (Hewlett-Packard Corp., Andover, Mass.) equipped with a standard 5.0 MHz transducer probe. The echocardiographic examinations were performed on admission and then again at follow-up.
The following data were recorded for each patient before and after the corrective operation: (1) diameters of subaortic region, aortic valve anulus, ascending aorta, and descending thoracic aorta, (2) presence of transpulmonary, subvalvular aortic, transvalvular aortic, and aortic arch gradient; and (3) residual VSD. Echocardiographic evidence of IAA-VSD with balanced ventricular anatomy and a subaortic diameter (SAD) 6 or more standard deviations (SDs) below predicted values for body surface area (Z value H-6) were used as inclusion criteria for the analysis.
Because of the presence of a nonrestrictive VSD, the severity of the subaortic and aortic valvular stenosis could not be assessed by Doppler examination of the LVOT, and anatomic parameters were used instead. Measurements of the aortic anulus diameter were done from the parasternal long-axis view in early systole at the hinge point of the valve leaflets. SAD was assessed from the standard parasternal long-axis view by measuring the distance from the most posterior edge of the conal septum to the most anterior edge of the opposite left ventricular free wall in both systole and diastole (Fig. 1). This was normalized to the diameter of the descending thoracic aorta (DTA) at the level of the diaphragm in systole and diastole, from the subcostal sagittal view as reported elsewhere. 14 For all parameters, at least three cardiac cycles were considered and the results were averaged.
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16; these are based on calculations by Sievers and associates. 17 Accordingly, the aortic root proximal diameter was arbitrarily used in the calculation of the "normal" SAD; it was found to be equivalent to the corresponding echocardiographic measurements. The values of SDs relative to the diameter of the aortic anulus were calculated with the aid of nomograms previously published by the Congenital Heart Surgeon Society multiinstitutional study on IAA-VSD. 2
Surgical technique
Through a median sternotomy, the anterior-superior mediastinum was explored for presence of thymic tissue. The thymus was resected in toto before incision of the pericardial sac to allow adequate exposure of the brachiocephalic vessels and transverse aortic arch. Thorough dissections of the origin of the brachiocephalic vessels, the branch pulmonary arteries, and the descending thoracic aorta 1 to 2 cm distal to the isthmic region were completed. Selective ascending aortic and pulmonary arterial cannulations were performed with a 14F intravenous catheter and a 10F arterial cannula (Bard, Inc., Tewksbury, Mass.), respectively. A single straight 16F venous-return cannula (Research Medical, Inc., Midvale, Utah) was inserted through the right atrial appendage (Fig. 2). After occlusion of the two branch pulmonary arteries, cardiopulmonary bypass (100 to 120 ml · kg-1 · min-1) was established and deep hypothermic (18º C rectal temperature) circulatory arrest was induced. Continuous cold (0º to 4º C) saline solution irrigation of the pericardium was used to maintain myocardial temperature between 4º to 8º C.
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Inotropic support with dopamine and dobutamine was uniformly used to facilitate weaning from cardiopulmonary bypass. The sternotomy wound was electively left open to allow cardiac decompression in all patients; a silicone elastomer patch (Silastic; Mentor, Inc., Goleta, Calif.) was used to close the skin incision temporarily. Postoperative antibiotic prophylaxis with cefazolin sodium (75 to 100 mg · kg-1 · day-1, intravenously administered) was adopted in all patients. Transition to broader-spectrum antibiotics was done on an individual basis as clinically indicated.
Follow-up
All patients had a follow-up clinical assessment between October 1, 1994 and January 31, 1995. The follow-up transthoracic two-dimensional echocardiographic examinations were performed at a mean follow-up time of 14 ± 9 months (range 1 to 29 months). On this occasion, postoperative measurements of the LVOT dimensions were repeated. In addition, the Doppler peak instantaneous gradient calculated from the Bernoulli equation was used to detect any residual subaortic obstruction.
Statistics
All data are expressed as mean values ± SD. A two-tailed, paired Student's t test was used for comparison of discrete variables when appropriate. A p value <0.05 was considered statistically significant.
Results
Patients
The patient population consisted of six male and three female neonates, with weights ranging from 2.1 to 3.9 kg (median 3.3 kg) and ages at operation ranging from 2 to 23 days (median 6 days). Two patients were premature neonates and six were born at term. All patients were intubated shortly after birth for respiratory distress and mechanically ventilated until the time of surgical repair. All were found to have ductal-dependent congenital heart defect and thereafter started on a regimen of prostaglandin E1 infusion at doses ranging from 0.03 to 0.08 µg · kg-1 · min-1. The duration of prostaglandin E1 therapy, ranging from 1 to 10 days, corresponded to the time elapsed from admission to surgical repair (Table I). Seven of the nine patients were placed on regimens of intravenous dopamine (range 5 to 10 µg · kg-1 · min-1) and dobutamine (range 5 to 10 µg · kg-1 · min-1) for a period of 2 to 8 days.
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Preoperative echocardiographic findings
All patients had situs solitus of the atria and concordant atrioventricular and ventriculoarterial connections. Eight patients had a Celoria-Patton 18 type B defect and one patient had a type C IAA. All patients had malalignment VSD caused by posterior displacement of the conal septum into the LVOT and causing SAS (Fig. 1). Additional morphologic features were a patent foramen ovale in nine patients, an aberrant subclavian artery in two patients (one right and one left), a hypoplastic ascending aorta in two patients, and a bicuspid aortic valve in one patient.
The mean diastolic ratio of SAD to DTA was 0.75 ± 0.04 (range 0.68 to 0.80; Table II). Previous studies have shown normal ratios to range between 1.00 and 1.50. 14 The mean systolic ratio of SAD to DTA was also markedly abnormal, with a mean value (0.63 ± 0.08) consistent with previously accepted echocardiographic and angiographic indices of severe SAS (Table II). 14,19 The subaortic diameter ranged from 2.7 to 4.0 mm, corresponding to values between 7 and 10 SDs below normal. 16 In addition, aortic valvular stenosis (mean aortic anulus diameter of 5.1 ± 0.6 mm, range 4.0 to 6.0 mm) and moderate hypoplasia of the ascending aorta (severe in two patients) were found in all patients (Table II). There was no semilunar valve incompetence on preoperative ultrasonographic examination.
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Postoperative course
The need for inotropic support with dopamine and dobutamine at low to moderate doses generally lasted a week after operation (Table III). One patient had two episodes of cardiac arrest necessitatng emergency reexploration of the sternotomy wound and open-chest cardiac massage; a low-dose infusion of epinephrine was added for a total of 24 hours to the preexisting inotropic support to achieve hemodynamic stability. This patient recovered rapidly, and later echocardiographic examination demonstrated normal left ventricular function. He was discharged on the tenth postoperative day in good clinical condition.
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Table III). Three patients, however, had prolonged respiratory failure necessitating 2 to 6 weeks of mechanical ventilation. The causes of respiratory insufficiency in these patients included vocal cord granuloma necessitating treatment by laser (one patient), preexisting bilateral phrenic nerve palsy palliated with surgical plication of the right hemidiaphragm (one patient), and bronchopulmonary dysplasia in one patient, who was operated on initially as a premature, 2.1 kg neonate. Additional complications were pericardial effusion requiring surgical drainage (one patient), chylothorax requiring chest tube insertion (one patient), and Pseudomonas aeruginosa pneumonia treated with cephalosporin and aminoglycoside antibiotics (one patient). All patients showed significant (<4.0 mg/dl) hypocalcemia in the immediate postoperative period. The hypocalcemia was severe (<3.0 mg/dl) and responded poorly to high-dose (10-15 mg · kg-1 · hr-1) calcium-chloride continuous intravenous administration in seven patients (78%) with documented preoperative hypocalcemia. All patients recovered appropriate serum calcium levels, however, two with the aid of oral calcium supplementation. Two patients, one with a previously palliated jejunal atresia, required Nissen fundoplication of the stomach and permanent gastrostomy tube placement for treatment of documented gastroesophageal reflux. The duration of intensive care stay averaged 2 weeks, and total hospital stay averaged 3 weeks (Table III). All patients were discharged in good clinical condition on regimens of oral furosemide and digitalis.
Follow-up
No patient showed signs of heart failure, and rhythm was sinus in all patients at follow-up clinical assessment. All were on regimens of oral furosemide and digitalis. Only one patient, who was operated on as a premature 2.3 kg neonate, was found to have decreased peripheral pulses on physical examination.
Follow-up transthoracic two-dimensional and Doppler echocardiography 1 to 29 months (14 ± 9 months after discharge) confirmed relief of the SAS in all patients (Fig. 7). The peak systolic subaortic gradient did not exceed 20 mm Hg in any patient (mild SAS; Table IV). One patient with a preoperative aortic annular diameter of 2.7 mm and bicuspid aortic valve showed mild aortic stenosis on postoperative Doppler interrogation (peak gradient of 30 mm Hg). No patient had aortic or pulmonary valve insufficiency. Hemodynamically relevant residual VSDs were not detected (tiny VSDs were present in four patients; Table IV). Residual mild (15 mm Hg) aortic arch obstruction was detected in only one patient. Normalization of the indices of SAS was invariably observed (systolic ratio of SAD to DTA, 1.00 to 1.32), suggesting adequate growth of the subaortic region after the operation (Fig. 8) in all patients. The postoperative mean systolic ratio of SAD to DTA was significantly greater than the corresponding preoperative value (1.20 ± 0.10 vs 0.63 ± 0.08, p < 0.001; Table IV). No patient required reoperation or reintervention.
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Diagnosis of SAS
Previous experience showed that SAS is the primary determinant of the increased mortality risk and need for reintervention after repair of IAA-VSD. 2-9 The ability to foresee the need for relief of the subaortic obstruction at the time of primary repair and the prevalence of recurrence of this lesion after repair remains inadequate, however, because of the lack of uniform diagnostic criteria. The use of physiologic parameters such as preoperative subaortic pressure gradients has proved unreliable because of the presence of a nonrestrictive VSD, so anatomic measurements must be employed instead. In our series, the estimates of the subaortic diameters were drawn from the level of maximal narrowing. Both the systolic and diastolic diameters of the subaortic region, normalized to the diameter of the descending thoracic aorta at the diaphragmatic hiatus, were considered in each patient, in agreement with previous work by Bove and associates. 14 It was suggested that systolic ratios less than 0.6 and diastolic ratios less than 1.0 are indicative of severe SAS and mandate relief of the obstruction. 14,19 Further clinical work also maintained primary repair of IAA-VSD to be contraindicated when the subaortic diameter is less than 4 mm. 20
In compliance with both proposed criteria, the degree of SAS in our patients was severe. In fact, although only two patients had initial systolic ratios between 0.6 and 0.7, the remaining seven patients showed values between 0.4 and 0.6. In addition, before the operation all patients demonstrated diastolic ratios less than 1.0 and absolute values of subaortic diameters less than 4.0 mm (7 to 10 SDs below normal). The complexity of the left-sided obstructive lesions in our patient population was also substantiated by the invariably diminutive size of the aortic anulus (4 to 7 SDs below normal) and ascending aorta.
Recent clinical work by Geva and associates 21 suggested that preoperative echocardiographic parameters useful in predicting the occurrence of SAS after repair of IAA-VSD are cross-sectional subaortic area, type B morphology of IAA, and presence of an aberrant subclavian artery. In that review, the linear measurements of the subaortic diameter did not correlate with postoperative appearance of SAS. Because these authors failed to consider the type of surgical therapy (if any) adopted against the SAS, the implications in terms of the need for reduction of the SAS are unclear. The subaortic cross-sectional areas were not routinely gathered in our study. Although eight of nine patients in our series had type B arch interruption before the operation and two had an aberrant subclavian artery, no patient had significant residual SAS at follow-up.
The need to determine which patients with IAA-VSD will require intervention against the SAS remains stringent, but uniform criteria to measure the subaortic region are still lacking. It therefore seems reasonable to identify a parameter other than the ideal anatomic measurement that may be easily quantified and referred to for the purpose of comparing different clinical series and predicting outcomes after surgical intervention.
Relief of SAS
The early attempts at staged repair of this complex lesion by reconstructing the arch and banding the pulmonary artery were marked by lack of success. 6 These findings were essentially confirmed by a recent multiinstitutional retrospective review, which identified staged or primary repair of the IAA-VSD complex without concomitant procedure to alleviate the SAS as a risk factor for early death. 2 Both the unresolved LVOT obstruction and the possible progressive hypertrophy of the conal septum in patients with or without pulmonary artery banding could explain these observations.
A series of palliative and reparative procedures aimed at bypassing the level of intracardiac obstruction was subsequently proposed. Although palliative interposition of prosthetic grafts between the DTA and the main pulmonary artery (6) or left ventricular apex (10) resulted in improved early survival, the need for reoperation limited their application. More recently, primary repair of IAA-VSD-SAS in neonates was reportedly performed with the pulmonary valve and proximal pulmonary trunk used to reconstruct the systemic outflow and the right ventricle–to–pulmonary artery continuity reestablished with a conduit. 8,11,12 Although these techniques achieve a biventricular repair, the approach failed to reduce the high operative mortality rate (33% of the reported cases) and to overcome the need for reoperation associated with extracardiac conduits. An alternative strategy proposed was that of converting the physiology to univentricular circulation by means of a modified Norwood palliative procedure. 22 The persistently high mortality rate and the complications connected with the univentricular circulation, including the need for multiple reoperations, make this option attractive only in case of coexisting aortic valve atresia. 2
Significant improvement in perioperative survival and greater success in relieving the LVOT obstruction were reported with one-stage anatomic repair and associated myotomy or myectomy of the posteriorly displaced conal septum. 6,13,14 Transaortic resection of the conal septum proved technically difficult because of the diminutive size of the ascending aorta and aortic valve, 6,14 so alternative access to the subaortic area was proposed through a transventricular approach. 13 Although such a procedure is easier from a technical standpoint, the detrimental hemodynamic consequences of an infundibulotomy on the neonatal heart has limited the application of procedures of conal enlargement through the right ventricular outflow tract. Finally, transatrial resection of the conal septum during one-stage repair of IAA-VSD was proposed by DeLeon and coworkers 13 and Bove and associates 14 to treat severe forms of SAS. Although overall survival in these limited series was acceptable (only one late death occurred in the series of Bove and associates 14), the prevalence of postoperative complications remained high. This technique was associated with a significant occurrence of complete heart block (16% in the series of DeLeon and coworkers 13) and intraoperative aortic valve injury (17% in the series of Bove and associates 14). Given the small size of the cardiac chambers, it is conceivable that a transatrial resection of the conal septal tissue may be technically challenging, even when the muscle is anchored with a suture and retracted toward the right atrium. 14 The proximity of the septum to the aortic valve makes this technique intrinsically hazardous to the aortic semilunar valve. Further, larger-scale analysis of the results of one-stage IAA-VSD repair with myotomy or myectomy, although demonstrating a lower prevalence of recurrent subvalvular obstruction, showed increased early and midterm mortality rates, averaging 50% at 5 years. 2
Our approach to the management of IAA-VSD has been that of primary repair at the time of presentation, and we have successfully adopted a new technique to treat critical SAS. Because the malalignment of the conal septum with the resulting SAS is responsible for the in utero involution of the ascending aorta and aortic arch (fourth aortic arch), there is reciprocal development of the pulmonary artery and ductus arteriosus (sixth aortic arch). The degree of SAS is therefore directly proportional to the size of the pulmonary trunk and inversely proportional to the size of the ascending aorta. Two considerations follow: (1) relief of the subaortic obstruction is necessary to promote growth of the left side of the heart–aorta complex, once the arch continuity has been reestablished, and (2) in case of severe SAS, a transpulmonary approach to the repair of the intracardiac defects may be technically easier.
In agreement with these considerations, we exposed the VSD and subaortic region through the pulmonary artery trunk. We thereby managed to relieve even extreme degrees of subaortic obstruction without resection of the conal septum, thus minimizing the potential for aortic valve injury. In fact, by placing the apical VSD patch stitches on the left ventricular side of the crista supraventricularis and by downsizing the patch itself, we believe that the conal septum may be deflected away from the subaortic region. Previous work by Jonas and coworkers 2 suggested that septation of the ventricular chambers in and of itself may promote deviation of the conal septum toward the right ventricle by recovering differential ventricular pressures during systole. On the basis of this consideration and of the added risk of early mortality associated with myotomy or myectomy of the subaortic muscle during one-stage repair of IAA-VSD, it was recently recommended that no concomitant procedures other than arch augmentation be performed at the time of surgical intervention. 23 Although we agree that this mechanism may be responsible for some dynamic changes during the systolic ejection phase, we are convinced that adequate relief of the SAS cannot be achieved without reestablishment of a laminar flow through the LVOT. In fact, we maintain that the oblique orientation of the VSD patch in our technique is critical in keeping the spur of conal septal tissue away from the subaortic area, thereby recreating a smooth-surfaced outflow tract. It is conceivable that the persistence of the unresected conal muscle, even in the presence of a repaired VSD, may sustain turbulent flow causing progressive hypertrophy of the displaced septum and persistent or recurrent SAS.
Clinical results
The reported early and midterm mortality rates for one-stage repair of IAA-VSD-SAS vary from 14% to 80%, with persistent or recurrent LVOT obstruction as primary cause of death. The survival with our novel approach to treatment of neonates with IAA-VSD-SAS, with no early or midterm deaths, compares favorably with those in all previously published series. In addition, the absence of procedure-related complications, including complete heart block, semilunar valve insufficiency, and residual VSD, validates the safety of our method.
Although the requirement for intensive care after corrective surgery was generally prolonged (mean ventilatory support of 2 weeks and intensive care unit stay of 3 weeks) because of the severity of associated anomalies, the cardiovascular system itself recovered much faster. The occurrence of complete or partial forms of DiGeorge syndrome in patients with IAA-VSD is well established. 17 The overall long-term outlook for these patients becomes relevant in planning for the repair of complex cardiovascular anomalies in the neonatal period. Only 25% of all patients with complete DiGeorge syndrome are affected by severe immunodeficiency necessitating bone marrow transplantation; most patients have mild or minimal immunodeficiency associated with acceptable long-term survival. Further, prediction of which patients affected by DiGeorge syndrome will not recover adequate immune function is still difficult. 17 On this basis, we believe it is justified to afford all patients with DiGeorge anomaly complete repair of associated cardiac defects.
The recurrence of significant SAS (peak gradient G25 mm Hg) after repair of IAA-VSD has ranged from 17% to 67% in most series, with 20% to 70% of patients requiring one or more additional surgical procedures to relieve the obstruction. In contrast, follow-up echocardiographic examination of our patients invariably revealed successful repair of the aortic arch, with adequate growth of the subaortic region and no recurrence of significant subaortic obstruction.
Limitations of the study
The major limitations to the our study include its retrospective and nonrandomized nature. In addition, a larger number of patients and a longer follow-up are needed to assess the durability of the current results.
Although our experience is limited in number, it is the largest (nine consecutive patients) reported by a single institution with a uniform surgical technique to treat IAA-VSD and critical SAS. 3-6,8-11,14 Even though longer-term than previously presented experiences, 14 however, our follow-up remains too short to establish definitive guidelines for the optimal surgical approach to IAA-VSD-SAS.
Recent multiinstitutional series have attempted to overcome the limitations imposed by the size of the population and length of follow-up. 14,23 Because preoperative and postoperative diagnostic findings are so critical in identifying factors predictive of outcome, however, the heterogeneous nature of the multiinstitutional database and the lack of standardized diagnostic criteria greatly flaw the potential significance of these studies.
Conclusions
We described a new approach to the surgical treatment of the neonate with IAA-VSD and critical SAS. Through a transpulmonary approach and without resecting the conal septum, severe forms of LVOT obstruction were relieved, resulting in excellent survival and negligible incidence of complications. Now that this technique has proved safe, it may be extended to all patients with IAA and malalignment VSD, regardless of the degree of subaortic narrowing.
Footnotes
From the Departments of Surgerya and Pediatrics,b University of Southern California, and the Divisions of Cardiothoracic Surgeryc and Cardiology,d Childrens Hospital Los Angeles, Los Angeles, Calif. 
References 3, 4, 8, 10, 12, 14, and 21.
References 3, 4, 8, 9, 14, and 21.
References
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C. I. Tchervenkov, S. A. Tahta, L. Jutras, and M. J. Beland SINGLE-STAGE REPAIR OF AORTIC ARCH OBSTRUCTION AND ASSOCIATED INTRACARDIAC DEFECTS WITH PULMONARY HOMOGRAFT PATCH AORTOPLASTY J. Thorac. Cardiovasc. Surg., December 1, 1998; 116(6): 897 - 904. [Abstract] [Full Text] [PDF]
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Y. Shiokawa and A. E. Becker The Surgical Anatomy of the Left Ventricular Outflow Tract in Hearts With Ventricular Septal Defect and Aortic Arch Obstruction Ann. Thorac. Surg., May 1, 1998; 65(5): 1381 - 1387. [Abstract] [Full Text] [PDF]
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R. D. Mainwaring and J. J. Lamberti Mid- to Long-Term Results of the Two-Stage Approach for Type B Interrupted Aortic Arch and Ventricular Septal Defect Ann. Thorac. Surg., December 1, 1997; 64(6): 1782 - 1785. [Abstract] [Full Text]
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K. Hisatomi Is left ventricular outflow tract obstruction really relieved on long-term follow-up? J. Thorac. Cardiovasc. Surg., March 1, 1997; 113(3): 617 - 618. [Full Text]
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V. A. Starnes, G. B. Luciani, W. J. Wells, R. B. Allen, and A. B. Lewis Aortic Root Replacement With the Pulmonary Autograft in Children With Complex Left Heart Obstruction Ann. Thorac. Surg., August 1, 1996; 62(2): 442 - 448. [Abstract] [Full Text]
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