Results and Complications After Single-Stage Repair of Aortopulmonary Window and Interrupted Aortic Arch in a 32-Week Preterm and a Full-Term Neonate.

BACKGROUND An aortopulmonary window (APW) is an uncommon congenital defect of the septation between the ascending aorta and pulmonary trunk. The combination of APW and interrupted aortic arch (IAA) is even rarer, with the hallmark characteristics of high peri-operative mortality and postoperative obstruction of the aortic arch, pulmonary artery, and left main bronchus. These complications often need re-interventions. CASE REPORT We present 2 cases with diagnoses of APW and IAA that were treated with single-stage repair. Case 1: A male 32-week premature newborn (weight 1789 g) had APW type I and IAA type A. He had severe postoperative aortic arch obstruction on postoperative day 1, and we re-intervened promptly. He was still asymptomatic after 6 years. Case 2: A male term neonate had APW type III and IAA type A. He had left vocal cord paralysis and left bronchial compression postoperatively. We applied prolonged noninvasive respiratory supports. The complications resolved without re-intervention on postoperative day 66. Progressive arch stenosis at anastomosis after operation required close follow-up with echocardiography. CONCLUSIONS These 2 reports highlight the feasibility of single-stage surgical repair while addressing 2 challenges: (1) Recurrent arch stenosis: Lower body weight and direct end-to-side anastomosis without patch augmentation could be risk factors for re-intervention. (2) Bronchial compression: Presentation of the second reported case implied that bronchial compression may not warrant immediate re-intervention unless there is complete obstruction, persistent atelectasis, or recurrent infection. Further studies on long-term outcomes of different surgical procedure would help us to clarify the proper way to avoid re-intervention.

Evaluation of aortopulmonary window using virtual dissection of multidetector computed tomography angiography data sets.

AIM: The present study sought to evaluate the morphology and associated cardiovascular anomalies in patients with aortopulmonary window on virtual dissection of multidetector computed tomography (CT) angiography data sets. MATERIAL AND METHODS: We conducted a retrospective search of our departmental database from January 2014 to September 2021 to identify patients with aortopulmonary window and relevant information was extracted from the electronic case records and from routine examination as well as virtual dissection of CT data sets. RESULTS: An aortopulmonary window was observed in 26 patients (20 males; 6 females). Based on location of the defect, a distal aortopulmonary window was the most common subtype, seen in 13/26 (50%) patients followed by a proximal, complete and intermediate subtypes seen in 7/26 (27%), 5/26 (19%) and 1/26 (4%) patients respectively. Associated ventricular septal defect was observed in 9/26 (34.6%) patients while an interrupted aortic arch was present in 5/26 (19.2%) patients. Tetralogy of Fallot was seen in 5/26 (19.2%) patients. Anomalous origin of right pulmonary artery from ascending aorta and crossed pulmonary arteries were seen in 2/26 (7.6%) patients each. An isolated aortopulmonary window without any simple/complex congenital anomaly was seen in 10/26 (38.5%) patients. CONCLUSION: Aortopulmonary window is associated with a wide gamut of cardiovascular lesions, with ventricular septal defect being the commonest associated anomaly followed by tetralogy of Fallot and interrupted aortic arch respectively. Virtual dissection of multidetector CT angiography allows detailed anatomical evaluation of aortopulmonary window, allowing a clear visualization of the defect and associated cardiovascular anomalies.

Unique Association of Aortopulmonary Window With Anomalous Origin of Left Coronary Artery From Pulmonary Artery in an Infant: A Blessing in Disguise?

Aortopulmonary window (APW) seen in association with anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is extremely uncommon. When faced with this combination, ALCAPA usually goes undetected since most of the clinical and echocardiographic features of ALCAPA, including ventricular dysfunction are absent due to co-existent pulmonary hypertension. Herein we report a 5-month-old child with a large APW in whom a preoperative computed tomography angiogram facilitated the diagnosis of ALCAPA. The case is described for its rarity and the challenges faced during management. Also, it is extremely crucial that this coronary anomaly is identified and corrected during surgery, since failure to do so results in unforeseen postoperative ventricular dysfunction.

An unusual case of aortopulmonary window with crossed pulmonary arteries.

We report a case of a 4-year-old boy with a distal type of aortopulmonary window with crossed arrangement of the pulmonary arteries. This case highlights the potential clinical implications of this variant pulmonary arterial anatomy in the setting of the aortopulmonary window and the role of computed tomography angiography in accurately defining vascular relationships in patients with complex congenital cardiac defects.

Type II Aortopulmonary Window with Isolated Left Subclavian Artery from Left Pulmonary Artery.

Type II Aortopulmonary window (APW) accounts for only 10% of total cases of APW, which by itself is a rare congenital anomaly. Various cardiac malformations have been reported to be associated with this rare anomaly. We report one such association of origin of left subclavian artery (LSCA) from left pulmonary artery (LPA) via ductus arteriosus that was surgically repaired.

Type ii aortopulmonary window with isolated left subclavian artery from left pulmonary artery

Abstract Type II Aortopulmonary window (APW) accounts for only 10% of total cases of APW, which by itself is a rare congenital anomaly. Various cardiac malformations have been reported to be associated with this rare anomaly. We report one such association of origin of left subclavian artery (LSCA) from left pulmonary artery (LPA) via ductus arteriosus that was surgically repaired.

Incidental diagnosis of a large aortopulmonary window with reversible pulmonary arterial hypertension in adult age and its surgical management.

Aortopulmonary window (APW) is a rare congenital cardiac condition. A large number of patients with a large APW usually die within 1 year of age. It is extremely rare to find cases of APW surviving till adult age and it is still rare to surgically treat such patients who are incidentally detected in adult age because such subsets of patients invariably have associated pulmonary vascular obstructive disease in advanced stage and thus there is therapeutic dilemma to surgically correct these patients. Although cases of uncorrected AP window presenting in adulthood have been reported but literature on surgically treated AP window in adult populations is limited. We describe case of APW in a 26-year-old male patient who was diagnosed incidentally while suspecting infective endocarditis and was subsequently surgically closed successfully with polytetrafluoroethylene patch after confirming reversibility of pulmonary arterial hypertension which is the key for successful outcome.

Assessment of intracardiac and extracardiac anomalies associated with coarctation of aorta and interrupted aortic arch using dual-source computed tomography.

To evaluate the value of dual-source computed tomography (DSCT) compared with transthoracic echocardiography (TTE) in assessing intracardiac and extracardiac anomalies in patients with coarctation of aorta (CoA) and interrupted aortic arch (IAA). Seventy-five patients (63 with CoA and 12 with IAA) who received preoperative DSCT and TTE were retrospectively studied. Intracardiac and extracardiac anomalies were recorded and compared by DSCT and TTE, in reference to surgical or cardiac catheterization findings. A total of 155 associated anomalies were finally found. Collateral circulation (56, 74.70%), patent ductus arteriosus (PDA; 41, 54.67%) were the most common anomalies. PDA, aortopulmonary window, and collateral circulation were more frequently present in patients with IAA than those with CoA (100% vs. 46.03%, 16.67% vs. 0%, and 100% vs. 69.84%, respectively, all p < 0.05). DSCT was superior to TTE in assessing associated extracardiac-vascular anomalies (sensitivity: 100% vs. 39.81%; specificity: 100% vs. 100%; positive predictive value: 100% vs. 100%; negative predictive value: 100% vs. 76.06%). Extracardiac-vascular anomalies, including collateral circulation and PDA, were the most common anomalies in patients with IAA and CoA. Compared with TTE, DSCT is more reliable in providing an overall preoperative evaluation of morphological features and extracardiac anomalies for surgical planning.

Aortopulmonary window in adults: A rare entity leading to Eisenmenger syndrome.

An aortopulmonary window (APW) is a rare congenital heart defect involving an abnormal communication between the ascending aorta and the pulmonary trunk with separate aortic and pulmonary valves. This defect accounts for 0.2% of all congenital cardiac anomalies and if left untreated can lead to Eisenmenger syndrome, severe pulmonary hypertension, heart failure, and poor survival. The authors herein present a case of APW type III with Eisenmenger syndrome in an adult patient whose initial complaint was cyanosis, and provide a thorough review of the literature of cases of APW with Eisenmenger syndrome that have survived into adulthood.

Surgical outcome in aortopulmonary window beyond the neonatal period.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital cardiac defect accounting for 0.1% to 0.2% of all congenital cardiac defects. We here present the current midterm outcome of surgical repair of APW in patients more than 3 months of age. METHODS: The retrospective study was conducted to identify all the patients more than 3 months of age at presentation who underwent surgical repair of APW between June 2010 and August 2018 at our tertiary care institute and their outcome was analyzed. RESULTS: We found 14 patients of APW operated at the age of more than 3 months over a period of 8 years. Mean age of the cohort was 2.29 ± 2.96 years ranging from 3 months to 10 years with 57.14% being males. There were 11 (78.57%) patients with isolated APW and 3 (21.43%) had associated cardiac defects including tetralogy of Fallot (n = 1), ventricular septal defect (n = 1), subaortic membrane causing subaortic stenosis (n = 1), and one had extracardiac malformations. Two patients had type I, nine had type II, and three had type III APW as per Jacobs' classification. The mean size of the defect was 14.14 ± 4.33 mm. Mean duration of mechanical ventilation was 26.91 ± 16.65 hours (range, 12.25-67 hours). There was one in-hospital mortality and no late mortality over a mean follow-up of 3.06 ± 2.19 years. None of the patients required any kind of reintervention. CONCLUSION: Good results can be obtained even on late presentation with adequate perioperative care of the patients with the reversible pulmonary hypertensive disease.

Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.

OBJECTIVE: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex and heterogeneous form of congenital heart disease. There is a controversy regarding the optimal treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The purpose of this study was to summarize our algorithm and surgical results for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. METHODS: This was a retrospective review of 307 patients undergoing primary surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Excluded from this analysis were patients who had undergone prior surgical treatment at another institution and patients with single ventricle and major aortopulmonary collateral arteries. There were 3 surgical pathways, including midline unifocalization (n = 241), creation of an aortopulmonary window (n = 46), and other (n = 20). RESULTS: For the 241 patients who underwent midline unifocalization, 204 (85.4%) had a single-stage complete repair. There were 37 patients who underwent a midline unifocalization and central shunt, and 24 have subsequently undergone complete repair. Forty-six patients underwent an aortopulmonary window, of whom 36 have subsequently had a complete repair. There were 20 patients who had complex anatomy and underwent procedures other than described, and14 have subsequently undergone complete repair. Thus, for the patients currently eligible, 280 (93.0%) have achieved complete repair. For the 204 patients who had a single-stage complete repair, the mean right ventricle to aortic pressure ratio was 0.36 ± 0.09. Seventy-six patients underwent a staged repair, and the mean right ventricle to aortic pressure ratio was 0.40 ± 0.09 (P < .05 compared with single-stage repair). There were 3 (1.5%) early and 8 (4.0%) late deaths for the single-stage complete repair cohort versus 4 (4.0%) early and 15 (14.9%) late deaths for all other procedures (P < .01). CONCLUSIONS: The data demonstrate that more than 90% of patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries achieved complete repair. The overall mortality was significantly lower in the subgroup of patients who underwent single-stage complete repair.

Valved patch closure of aortopulmonary window.

The case of an 8-year-old boy with an aortopulmonary window who underwent unidirectional valved patch closure of the window is described. The advantages of unidirectional valved patch closure in this setting are discussed.

Aortopulmonary window: results of repair beyond infancy.

OBJECTIVES: To study the anatomic and haemodynamic data and results of surgery in patients undergoing surgical repair of aortopulmonary window beyond infancy. METHODS: Between July 2005 and December 2015, 23 patients, older than 1 year undergoing surgery for aortopulmonary window were analysed retrospectively. Postoperative clinical and echocardiography follow-up were performed. RESULTS: Median age and weight at repair was 4 years (range 14 months-12 years) and 12 kg (range 3.5-22 kg), respectively. Fifteen patients had Richardson's Type I, 6 patients had Type II and 2 patients had Type III aortopulmonary window. Six patients had associated defects. Baseline mean systolic pulmonary artery pressure was 101 ± 14.9 mmHg (range 80-130, median 100 mmHg) and pulmonary vascular resistance index was 9.6 ± 5.9 (median 7.7 Wood units/m2, range 3.7-23.5 Wood units/m2). Patch repair of aortopulmonary window was performed using the sandwich method (transwindow) (n = 15), transaortic (n = 3) and transpulmonary artery (n = 2) approaches; 2 patients underwent double ligation and 1 underwent division and suturing. Two patients underwent valved patch closure of aortopulmonary window and 1 patient underwent valved patch closure of associated ventricular septal defect. There were 2 in-hospital deaths: one due to intractable pulmonary hypertension and the other due to low cardiac output. Mean follow-up was 36 months (range 2-119 months). Eighteen patients were in NYHA Class I at last follow-up. There were no late deaths or reoperation. CONCLUSIONS: Surgery can be safely undertaken beyond infancy in carefully selected patients of aortopulmonary window with acceptable early and mid-term outcomes.

Prenatal diagnosis of aortopulmonary window associated with aberrant subclavian artery.

Aortopulmonary window is a rare cardiac developmental anomaly characterised by a communication between the ascending aorta and the pulmonary artery. Aortopulmonary window may be isolated or associated with cardiac defects such as ventricular septal defect, atrial septal defect, interrupted aortic arch, and tetralogy of Fallot. We report a case of aortopulmonary window associated with aberrant subclavian artery based on fetal two-dimensional echocardiogram. The mother was referred for fetal echocardiography because of multiple fetal anomalies. Prenatal echocardiography at 30 weeks of gestation revealed a defect between the main and right pulmonary arteries and the ascending aorta (type III). The patient was born at 38 weeks of gestation via caesarean delivery, and was admitted to the neonatal intensive care unit because of respiratory failure and multiple congenital anomalies. Postnatal echocardiogram and cardiac MRI confirmed the prenatal findings. In addition, this patient had severe Dandy-Walker malformation and renal anomalies with poor prognosis. The family decided to withdraw respiratory care support on day of life 4, and the neonate passed away shortly after.

Surgical approach to a rare case of double-outlet right ventricle and aortopulmonary window.

Although the spectrum of physiology seen in infants and children with double-outlet right ventricle is broad, this anatomy in combination with an aortopulmonary window is extremely rare. We present an interesting case of an infant prenatally diagnosed with this rare CHD. To our knowledge, this is the first report of complete repair in the neonatal period for such a combination of defects.

Ventana aortopulmonar / Aortopulmonary window

La ventana aortopulmonar es una causa poco frecuente de insuficiencia cardiaca en el neonato. Debe ser descartada en caso de existir edema pulmonar sin los shunts izquierda-derecha más frecuentes. Presentamos las imágenes ecocardiográficas de un recién nacido que ingresó con clínica de insuficiencia cardiaca en nuestra institución.

The aortopulmonary window is a rare cause of heart failure in the neonate. It must be ruled out if there are signs of pulmonary edema without the most frequents left-right shunts. We report the echocardiographic images of a newborn who was admitted with symptoms of heart failure at our institution.

Rare Case of Aortopulmonary Window With Anomalous Origin of Right Coronary Artery.

A 5-month-old infant presented with a rare, congenital heart disease: aortopulmonary window with an anomalous origin of the right coronary artery from the aortopulmonary window. Using echocardiography and computed tomography, the exact diagnosis could only be ascertained retrospectively; however, cardiac catheterization and angiography confirmed the diagnosis, which led to elective open-heart surgery. The infant made a full recovery.

[Aortopulmonary window]. / Ventana aortopulmonar.

The aortopulmonary window is a rare cause of heart failure in the neonate. It must be ruled out if there are signs of pulmonary edema without the most frequent left-right shunts. We report the echocardiographic images of a newborn who was admitted with symptoms of heart failure at our institution.

Aortopulmonary window and the interrupted aortic arch: midterm results with use of the single-patch technique.

BACKGROUND: An aortopulmonary window (APW) associated with an interrupted aortic arch (IAA) can be associated with significant rates of perioperative mortality and recurrent arch obstruction. We assessed the outcomes associated with the use of a single pericardial patch technique for primary repair. METHODS: Between 2002 and 2011, 9 neonates and 2 infants with APW and IAA underwent single-stage repair, under a hypothermic (28°C) continuous cardiopulmonary bypass with antegrade selective cerebral perfusion. A single autologous pericardial patch (glutaraldehyde-fixed) was used both to augment the IAA end-to-side anastomosis and to close the APW by use of the "sandwich" technique. RESULTS: The IAA was type A in 6 patients and type B in 5 patients. The APW morphology was type I in 6 patients, type II in 4 patients, and type III in 1 patient. The median age and weight at operation were 11 days (range, 6 to 180 days) and 2.6 kg (range, 2.2 to 6.5 kg), respectively. The mean cardiopulmonary bypass and aortic cross-clamp times were 108.6 ± 27.5 minutes and 49.3 ± 13.4 minutes, respectively. One patient required additional closure of a ventricular septal defect. Delayed sternal closure was performed in 8 patients. The mean follow-up time was 6 ± 3 years. There were no early and no late deaths. Postoperative morbidity consisted of one postoperative stroke with no late sequelae. There were no reoperations. The last follow-up visits confirmed the absence of recurrent aortic arch obstruction and pulmonary artery branch stenosis in all patients. CONCLUSIONS: Primary anatomic repair of APW associated with IAA can be safely performed. The efficiency of the single-patch technique was confirmed by the restoration of normal functional anatomy of the great arteries and aortic arch during follow-up.