Aortopulmonary window: Morphology, diagnosis, and long-term results.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital heart defect. We reviewed our experience with this condition over the last two decades. METHODS: Between September 1993 and December 2013, 62 patients underwent surgery for APW. Depending on the associated lesions, they were divided into two groups: Simple (Group 1) or complex (Group 2). In the complex group, six patients had a ventricular septal defect, five patients had interrupted aortic arch, three patients had tetralogy of Fallot, two patients had double outlet right ventricle, and one patient had the right pulmonary artery arising from the ascending aorta. RESULTS: Mean age at repair was 21.6 ± 32.02 months (median = 6, range 0.1-144 months). By preoperative echocardiographic assessment 27 out of 62 patients had severe pulmonary artery hypertension (52% of the cohort). Patch repair of APW was performed using the sandwich method (transwindow) (n = 27; 43.5%), transaortic (n = 18; 29%), and transpulmonary artery (n = 5; 8.1%) approaches; 10 patients (16.1%) underwent double ligation and two (3.2%) underwent division and suturing. Overall hospital mortality in group 1 was 6.97% (3/43) and in group 2 it was 21% (4/19), p = 0.085. Mean hospital stay in group 1 was 6.9 ± 2.4 days (median = 7 days) and in group 2 was 12 ± 6.1 days (median = 13 days), p = 0.0001. Follow-up in group 1 was 1.6-9.8 years (median = 6 years); in group 2, it was 1.8-8.9 years (median = 6.5 years). There were no late deaths. Two patients needed reintervention for distortion of the right pulmonary artery origin. All patients were in New York Heart Association Class I/II at last follow up. CONCLUSION: There are multiple acceptable surgical strategies for the treatment of aortopulmonary window. Despite a relatively advanced age and substantial number of patients with severe pulmonary hypertension the outcomes can still be good. Associated anomalies complicate the repair. Patients in the complex group had a protracted hospital course and a higher early mortality but similar late survival.

Outcomes of aortopulmonary window repair in children: 33 years of experience.

BACKGROUND: The purpose of this study was to assess the outcomes of children undergoing repair of aortopulmonary window (APW). METHODS: We conducted a retrospective review of all children (n=43) who underwent surgical repair of APW between 1980 and 2013. RESULTS: Median age at surgery was 40 days (range, 13 to 125). Simple APW was present in 15 of 43 patients (35%), and 28 of 43 patients (65%) patients had concomitant cardiovascular anomalies. The aorta was repaired by direct suturing in 36 patients (84%) patients and patching in 7 patients (16%). The main pulmonary artery was repaired by direct suturing in 22 patients (51%) patients and by patching in 21 (49%). Cardiopulmonary bypass was used in 42 of the 43 patients (97.7%). Single-staged repair of concomitant cardiovascular anomalies was undertaken in 26 of 28 patients (93%). Only 2 of the 28 patients (7%) underwent repair of interrupted aortic arch before APW repair. Operative mortality was 6.7% (1 of 15 patients) among patients with simple APW and 18% (5 of 28 patients) among patients with concomitant anomalies. Operative weight less than 2.5 kg was associated with mortality on univariable analysis (p=0.02). Median follow-up was 10.1 years (range, 0.17 to 24.2). There were no late deaths. Overall survival was 86% (95% confidence interval: 71.3 to 94.2) at 10 years. Freedom from reoperation was 95.3% (95% confidence interval: 86.2 to 99.9) at 10 years. At last follow-up, all patients were in New York Heart Association functional class I/II. CONCLUSIONS: Survival beyond discharge from the hospital is associated with excellent outcomes.

Transposition of great arteries with aortopulmonary window: our surgical experience.

BACKGROUND: Transposition of the great arteries with aortopulmonary window is a rare congenital cardiac anomaly. An arterial switch operation with repair of the aortopulmonary window is the preferred operation in this subset. As the tissue between the great arteries is missing, it is considered to be a complex operation. The purpose of this study is to present our experience of a simple yet highly effective surgical technique for the management of this rare complex cardiac defect. METHODS: We detail our experience of the surgery of this complex defect in 4 patients. Standard technique of an arterial switch operation with minor modification in excision of branch pulmonary arteries is all that is needed in approaching this complex subset. The moiety of tissue resulting from the absence of an aortopulmonary window was naturally covered by the proximal and distal neo-aortic flap tissue. The operative technique used in the 4 cases and their presentations are detailed in the text. RESULTS: Four patients of ages 28 days, 35 days, 40 days, and 6 months were successfully operated. One patient expired on postoperative day 21. In this case, advanced age of presentation, severe pulmonary artery hypertension, and sepsis possibly caused the death. The remaining 3 patients are off medication now and are being regularly followed up. CONCLUSIONS: In our experience early diagnosis and an arterial switch operation have been crucial in getting a favorable outcome in planning of this complex congenital heart disease.

Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals.

BACKGROUND: Pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) is a complex form of congenital heart defect. One identifiable subset has small (<2.5 mm) intrapericardial branch pulmonary arteries that are (1) confluent, (2) have normal arborization, and (3) have dual-supplied collateral vessels. When this anatomy is associated with limited pulmonary blood flow, the patients are candidates for creation of an aortopulmonary window to stimulate growth of the pulmonary arteries. The purpose of this study was to review our experience with creation of an aortopulmonary window as the initial palliative procedure. METHODS: This was a retrospective review of our surgical experience with 35 children undergoing aortopulmonary window creation from 2002 to 2011. Patients were identified by preoperative cardiac catheterization to define the cardiac and pulmonary artery anatomy. RESULTS: There was no mortality in 35 patients undergoing aortopulmonary window creation. These patients have subsequently undergone 78 cardiac procedures (with 2 operative mortalities). Eighteen of these patients have achieved complete repair, 4 patients in a second procedure, 6 patients in a third procedure, 5 patients in a fourth procedure, and 3 patients in a fifth procedure. CONCLUSIONS: The data demonstrate that patients can undergo creation of an aortopulmonary window with excellent early results. Few patients were amenable to complete repair at the second operation, and most required multiple reoperations to recruit sufficient arborization. We interpret these counterintuitive results to suggest that hypoplastic central pulmonary arteries and diminished pulmonary blood flow are markers for a less well developed pulmonary vascular bed.

Aortopulmonary window associated with interrupted aortic arch: report of surgical repair of eight cases and review of literature.

BACKGROUND: Patients with combined aortopulmonary window (APW) and interrupted aortic arch (IAA) malformations are rarely seen. We reviewed cases with such association with emphasis on surgical management and long-term outcome. METHODS: 109 patients with IAA and 25 patients with APW were operated upon between 1981 and 2011. The clinical records, operation and follow-up data were analyzed. Long-term outcome was completed with the help of either outpatient data or inquiring. Related literature was investigated. RESULTS: Combined APW/IAA was found in 8 cases: 7.3% (8/109) of those with IAA diagnosis and 32% (8/25) with APW. All APWs were proximal. 7 patients had interruption type A. A ventricular septal defect (VSD) was associated in one case only. Median age at surgery was 10 days. The two first patients were operated upon without cardio-pulmonary bypass (CPB) and one died during operation. The other six underwent single stage approach under CPB with no death: overall early mortality of 12.5% (⅛). APW was closed with one or two patches; aortic arch was reconstructed either directly (extended end-to-side anastomosis) or by patch augmentation. There was no late death and no reoperation during mean follow-up of 118 months (range 1-360 months). Six patients were in functional NYHA class I, the remainder in class II. The cumulative APW/IAA incidence from literature varies between 4.9% (56/1105) and 22.2% (42/189). Early mortality reaches 15.1% (8/53) (95% CI: 6.8% - 27.6%). CONCLUSION: Neonatal repair with thorough mobilization of the aortic arch and extended end-to-side anastomosis or use of patch augmentation carries potential for the best early and late outcome for combined APW/IAA malformation.

Surgical experience of aortopulmonary window repair in infants.

The aim of this study was to retrospectively analyze our results of both simple and complex aortopulmonary window (APW) repair in infants. From September 1994 to May 2003, 21 infants which included 15 with simple APW (weight 3.9+/-0.8 kg and age 5.1+/-3.7 months) and six with complex APW (weight 4.03+/-1.1 kg and age 5+/-3.8 months) underwent APW repair at the All India Institute of Medical Sciences, New Delhi, India. The approach for APW repair was ligation without CPB in four patients, division and suturing using CPB in one patient, trans-aortic with Goretex patch closure in 11 patients, trans-pulmonary in one patient and trans-window in four patients. The hospital mortality was 13% and 33% for simple and complex APW, respectively. On mean follow-up of 39 months there was no re-operation or late death. An early repair of APW is mandatory to achieve a good surgical result. Trans-aortic repair of APW is the procedure of choice for all APWs, except in the case of large defects where anterior sandwich patch technique (trans-window repair) may be done. In our view, simple ligation without CPB should be avoided due to the possibility of residual APW and distortion of pulmonary artery.

Compared fate of small-diameter Contegras and homografts in the pulmonary position.

OBJECTIVE: This study analyzes whether small-diameter Contegras behave in the same way as small-diameter homografts, when implanted for the first time in pulmonary position. METHODS: Small-diameter conduits include 12 and 14 mm Contegras and 8-14 mm homografts. Graft dysfunction is defined as right ventricular outflow tract obstruction with peak echo-Doppler gradient>40 mmHg, or grade III/IV graft regurgitation. Graft failure is defined as need for conduit replacement or need for catheter or surgical reintervention. Thirty-eight patients who received small Contegras (n=25) and small homografts (n=13) from October 2002 to end December 2006 were studied. The most frequent indication was pulmonary atresia and ventricular septal defect (n=20; 10 associated with major aorto-pulmonary collateral arteries), followed by truncus arteriosus (n=12). Most patients' characteristics were comparable except that recipients of homografts were smaller (p for body area=0.014). Survival, freedom from graft dysfunction, failure and explantation were estimated by the Kaplan-Meier method. The log-rank test was used to compare outcomes. RESULTS: There were three early and four late deaths. No death was graft related. Survival was 80+/-8.2% for patients with Contegras and 77+/-11.7% for those with allografts: p=0.82. Mean follow-up duration is 22+/-16 months. Freedom from dysfunction for Contegras conduits decreased in the first 6 months and stabilized at 58+/-11% from month 14. For homografts it decreased only 1 year after implantation, down to 35+/-19.7% from month 31: p=0.61. Freedom from Contegras failure diminished the first 16 months to level out at 57+/-13%. No homograft failed the first 2 years. With a p-value of 0.14, homografts tended to fail less frequently. Five grafts were explanted. Freedom from explantation was similar (p=0.98): 90+/-6.7% for Contegras and 75+/-21.6% for homografts at year 3. CONCLUSION: In the first 4 years after pulmonary implantation of small-diameter Contegras and homografts, the fate of both conduits was statistically similar, in spite of different behavior. As Contegras is 'off-the-shelf' available, it constitutes a sound alternative to homograft for right ventricular outflow tract reconstruction in neonates and infants.

Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVE: Our treatment strategy for pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries is a staged repair that comprises the first complete unifocalization (UF) with 'unification' of intrapulmonary arteries and then the definitive repair. The purpose of this study is to evaluate the outcome of our staged repair strategy with complete UF and to determine the results of our current management strategy. METHODS: From 1982 to 2004, 113 consecutive patients were treated with staged repair at our institute. We evaluated the risk of definitive repair failure or death in the 3 years after definitive repair using logistic regression. Furthermore, we compared the early group (patients who underwent UF before December 1995) and the late group (patients who underwent UF after January 1996). RESULTS: The mean follow-up interval was 8.8 years (0.8 months to 23.3 years), and Kaplan-Meier-estimated overall survival rates after first UF were 80.9, 73.8, and 69.9% at 5, 10, and 15 years, respectively. Survival in patients with an absent central pulmonary artery (PA) was significantly lower than in those with a central PA (p<0.05), and the factor that was significantly associated with definitive repair failure or death in the 3 years after definitive repair was central PA morphology (p<0.05). Higher mean PA pressure after UF was detected in patients with hypoplastic central PA, compared with those without hypoplastic PA (30.9 mmHg vs 23.3 mmHg, p<0.05). In the late group, age (in years) at first UF (3.9 vs 8.4, p<0.01), second UF (4.3 vs 9.2, p<0.01), and definitive repair (5.8 vs 9.1, p<0.01) was significantly younger than in early group, and the survival rate after first UF in the late group was 96.2 and 91.3% at 3 and 7 years, respectively. Systolic right ventricular pressure and the pressure ratio between the right and the left ventricles after definitive repair in the late group were significantly lower than in the early group (53.6 mmHg vs 75.0 mmHg, p<0.01; 61.7% vs 75.9%, p<0.05). CONCLUSIONS: Hypoplastic central PA was a significant risk factor in this disease. The overall survival was improved by our current management strategy. Improved RV pressure after definitive repair appears to affect the long-term outcome.

Diagnosis, management, and results of treatment for aortopulmonary window.

The aortopulmonary window is a communication between the ascending aorta and the pulmonary trunk in the presence of two separate arterial valves. This uncommon congenital anomaly is reported rarely in the literature. We present here our experience with 16 patients, emphasizing the importance of early closure of the defect by a transaortic approach. We performed surgery on 16 patients over a period of 13 years using a transaortic approach under cardiopulmonary bypass. The median age of the patients at the time of operation was 6.5 months, with a range from 1 month to 11 years. Preoperative pulmonary arterial systolic pressure ranged from 30 to 100 mmHg. Associated cardiac anomalies were present in 7 of the patients, and were repaired at the same stage. The defect was between the ascending aorta and the proximal pulmonary trunk in 13 patients, and between the ascending aorta and the distal pulmonary trunk, with overriding of the orifice of the right pulmonary artery, in 3 patients. For closure, we used a patch of 0.4 mm Gore-Tex in 11, and gluteraldehyde-treated autologous pericardium in 5 of the patients. One patient died during surgery. The mean follow-up period for the surviving 15 patients was 52.2 months, with a range from 12 to 130 months. All the patients were in good condition during the follow-up, and no residual defects have been detected. Aortopulmonary window is a rare congenital cardiac anomaly, which can be repaired with very good operative results if surgery is performed before the development of irreversible pulmonary hypertension. We advise early correction of the defect with a transaortic patch, repairing all associated cardiac anomalies at the time of diagnosis.

Defeito do septo atrioventricular forma total / Atrial and ventricular heart septal defect total form

Nesta monografia é abordada a experiência adquirida durante anos de prática cirúrgica no tratamento dos defeitos do septo atrioventricular, sendo apresentada a opinião dos autores sobre a sua divisão em diferentes formas (parcial, intermediária e total). São apresentados inicialmente, alguns fatos históricos sobre a doença, destacando-se nomes e contibuições para o diagnóstico e o tratamento cirúrgico desta entidade tão complexa. O desenvolvimento embriológico do sistema cardiocirculatório é seguido pelo posicionamento com relação à gênese do aparecimento deste defeito cardíaco e pela noção de que as diferentes formas fazem parte de uma única doença em diferentes fases evolutivas...

Optimal surgical approach for repair of aortopulmonary window.

BACKGROUND: This is a review of the experience over 26 year in a single institution with surgical repair of aortopulmonary window. METHODS: Between July 1973 and March 1999, 38 patients underwent surgery for aortopulmonary window at a median age of 5 weeks, and with a median weight of 3.9 kg. Median follow-up was 6.6 years, with a range from 0.8 to 26 years. Additional defects were present in 25 (65%) patients, including interruption of the aortic arch in 7, tetralogy of Fallot in 7, ventricular septal defect in 5, functionally univentricular anatomy in 3, aortic coarctation in 2, and anomalous origin of a coronary artery in 1. We approached via an aortotomy in 45%, an incision through the defect in 31%, and using a pulmonary arteriotomy in 24% of patients. Closure was achieved using a single patch in 30 patients (79%). RESULTS: There were 3 (7.9%) in-hospital deaths. Actuarial patient survival was 88% at 10 years. Three patients required reinterventions for stenoses of the great arteries. Freedom from any reintervention was 70% at 10 years. By multivariate analysis, the approach through a pulmonary arteriotomy was shown to result in a higher need for reintervention (p = 0.01). CONCLUSIONS: Repair of aortopulmonary window can be done with excellent results. A pulmonary arteriotomy should be avoided.

Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants <6 months of age.

The Richardson classification system for aortopulmonary septal defect (APSD) includes simple defects between the ascending aorta and pulmonary trunk (type I), defects extending distally to include the origin of the right main pulmonary artery (type II), and anomalous origin of the right main pulmonary artery from the ascending aorta with no other aortopulmonary communication (type III). These are rare lesions that must be repaired in early infancy to avoid development of pulmonary vascular disease. Few reports have focused on patients with complex, associated lesions who underwent repair in early infancy. Between 1972 and 1995, 24 patients with Richardson type I (n = 11), II (n = 7), or III (n = 6) defects underwent repair at ages ranging from 2 to 172 days (median 34). Twelve patients had complex, associated anomalies, including interrupted or hypoplastic arch (n = 9), tetralogy of Fallot with (n = 1) or without (n = 1) pulmonary atresia, and transposition of the great arteries (n = 1). The most recent 7 patients were diagnosed by echocardiography without cardiac catheterization. There were no early or late deaths among the 12 patients with simple APSD. Four patients with complex, associated lesions died in the early postoperative period and another died 4 months after surgery. All 6 surviving patients with interrupted arch have had recurrent obstruction at the arch repair site, although reintervention for this reason has been performed in only 2 patients. Altogether, 6 early survivors have required reintervention, and all survivors are in New York Heart Association class I at follow-up ranging from 2 to 25 years. Thus, long-term survival after repair of APSD in early infancy is excellent. Late sequelae are likely to be related either to associated lesions or to obstruction at the APSD repair site. Almost all cases of APSD in young infants can be diagnosed and evaluated by echocardiography without catheterization.

Congenital aortopulumonary window: diagnosis, surgical technique and long-term results.

OBJECTIVE: Congenital aortopulmonary window is rare, often associated with other cardiac anomalies. Surgical repair as the only treatment should be performed before pulmonary vascular changes have developed. This study presents the long-term outcome after surgical correction for this condition. MATERIALS AND METHODS: Between 1971 and 1993, 13 patients with congenital aortopulmonary window were found. 10 had type I, 2 type II and 1 had type III. Concomitant cardiac anomalies were present in 10/13. Eleven patients were operated on at a mean age of 31.2 +/- 48.3 months (range 6 days-10 years). Thoracotomy was used in 3 and sternotomy in 9 patients. In 4/11, the aorto-pulmonary window was simply ligated, 4 had a transpulmonary approach and 1 combined with a transaortic approach. The aortopulmonary window was closed directly in 2 and with a Dacron patch in 1. Cardiopulmonary bypass was used in 6 patients. Associated anomalies in 10/11 patients. RESULTS: There was one operative death (9%): a six-day old boy with interrupted aortic arch died 6 h postoperatively due to low-cardiac output. The mean follow-up period is 8.1 +/- 7.3 years (range 2-24 years). Clinical examination, transaortic echocardiography and/or cardiac catheterization were obtained in the follow-up. There was no late death. All are in New York Heart Association (NYHA) class I. One had to be reoperated on for a recurrent shunt 29 months after ligation and one had angioplasty after 23 months for residual stenosis of the reimplanted right pulmonary artery. The actuarial survival rate is 90% after 1, 5 and 10 years. CONCLUSIONS: The surgical treatment of aortopulmonary window has a low risk, even if associated with major cardiac anomalies. Prompt operative treatment achieves excellent long-term results.

Aortopulmonary window: factors associated with early and late success after surgical treatment.

Between 1953 and 1990, 19 patients, who were from 7 weeks to 27 years old, underwent surgical correction of aortopulmonary window at the Mayo Clinic. Associated cardiac anomalies were present in nine patients (47%). At operation, extracorporeal circulation was used in all except one patient. In seven patients, division and primary closure were done. In four patients, the defect was closed by direct suture through a transpulmonary approach. In eight patients, the defect was closed with a patch through a transpulmonary or transaortic approach. Four deaths (21%) occurred intraoperatively or immediately postoperatively. All four patients had undergone division of the aortopulmonary window before 1962, and three of them had a pulmonary vascular resistance (Rp) that was 7.3 U.m2 or more and a ratio of Rp to systemic vascular resistance (Rp/Rs) that exceeded 0.6. One patient with an Rp of 11.8 U.m2 and an Rp/Rs of 0.72 died 16 years postoperatively. Statistical analysis of risk factors indicated that early year of operation (P = 0.022), division of the aortopulmonary window versus transaortic or transpulmonary closure (P = 0.009), and a high Rp/Rs (P = 0.021) were significantly associated with perioperative death. All patients with a preoperative Rp of 8 U.m2 or less, an Rp/Rs of less than 0.4, and no major associated cardiac anomalies were in functional class I (New York Heart Association) postoperatively. Our study confirms that infants with aortopulmonary window should undergo operation early, before irreversible pulmonary vascular changes have developed. Patients with an Rp/Rs that exceeds 0.4 should be thoroughly assessed to determine their operability.

Surgical management of aortopulmonary septal defect.

Fourteen patients with malseptation of the aortopulmonary trunk underwent operative repair from 1978 to 1988. Age ranged from 1 year to 35 years (mean age, 9.6 years). Five patients had type I, 6 had type II, and 3 had type III lesions. The hemodynamic disturbance in all patients was the consequence of a large left-to-right shunt (mean pulmonary/systemic flow ratio, 2.38:1) with increased pulmonary vascular resistance (mean value, 4.47 units/m2). Our initial surgical experience with closure under cardiopulmonary bypass through the transaortic route in 3 patients and the transpulmonary approach in two patients resulted in 3 deaths. In the 9 subsequent patients, division and repair of the defect in the great vessels yielded uniformly good results. During follow-up, which ranged from 3 months to 2 years, all 11 survivors had good clinical improvement and none showed residual defects on restudy. Pulmonary artery pressure and pulmonary vascular resistance decreased in all patients except 1.

Aortopulmonary window in the first year of life: report on 11 surgical cases.

From June, 1975, through April, 1986, 11 consecutive infants with aortopulmonary window were operated on at the Department of Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy. The classic type of aortopulmonary window was present in 6 patients, whereas 4 patients had a ductus type of aortopulmonary window and 1 patient had a double aortopulmonary window, associated transposition of the great arteries, and ventricular septal defect. Operative technique included patch closure of the defect by means of cardiopulmonary bypass using various approaches: transaortic in 3, transpulmonary in 2, and transwindow in 2. The aortopulmonary window was ligated in 3 patients, in 2 of whom an incorrect diagnosis of patent ductus arteriosus was made at cardiac catheterization. Division and suture of the defect through a median sternotomy was performed in the remaining patient, who was moribund on admission. There was a single operative death. All survivors are in New York Heart Association Functional Class I at a mean interval of 6 1/2 years postoperatively.