RESUMO
Background: Aortopulmonary window (APW) is a rare anomaly with variable morphology and associated cardiac anomalies. We evaluated impact of patient and operative factors on mid-term outcomes following APW repair. Methods: Twenty-nine patients underwent surgical APW repair at our institution from 1996 to 2022. Eight (28%) had simple APW, accompanied by only atrial septal defect or patent ductus arteriosus; 21 (72%) had complex APW with additional cardiovascular lesions, including nine with interrupted aortic arch. Median operative age was 19 days (range 2 days-1.5 years) via single-patch (n = 12, 41%), double-patch (n = 15, 52%), or ligation and division (n = 2, 7%). Results: The only mortality occurred in-hospital 1.4 years postoperatively following remote myocardial infarction. Factors associated with longer postoperative length of stay were complex APW (P = .003), genetic syndrome (P = .003), noncardiovascular comorbidities (P = .002), lower birth weight (P = .03), and lower operative weight (P = .03). Six patients (21%) with complex APW underwent unplanned cardiothoracic reintervention(s), including two with arch reintervention following arch advancement for interruption. Reintervention-free survival was similar for simple versus complex APW, operative age categories, and repair techniques. At median follow-up 5.5 years postoperatively, no patients had residual APW or persistent pulmonary hypertension, 1 (3%) had greater than mild ventricular dysfunction, and 25 (89% survivors) had NYHA class I functional status. Conclusions: Operative APW repair has excellent mid-term survival, durability, and functional status, regardless of operative age, cardiovascular comorbidities, or repair technique. Cardiac and noncardiac comorbidities may be associated with prolonged length of stay.
Assuntos
Defeito do Septo Aortopulmonar , Procedimentos Cirúrgicos Cardíacos , Humanos , Feminino , Masculino , Defeito do Septo Aortopulmonar/cirurgia , Defeito do Septo Aortopulmonar/mortalidade , Recém-Nascido , Lactente , Estudos Retrospectivos , Procedimentos Cirúrgicos Cardíacos/métodos , Resultado do Tratamento , Complicações Pós-Operatórias/epidemiologia , Seguimentos , Fatores de TempoRESUMO
An aortopulmonary window (APW) is a communication between the main pulmonary artery (MPA) and the ascending aorta in the presence of two separate semilunar valves. Surgical strategies described for the management of APW include ligation, division and suturing, patch closure of APW (transaortic or transpulmonary or transwindow), and device closure. Ligation of a type-2 APW can result in stenosis or complete closure of the right pulmonary artery (RPA) off the MPA, while leaving the aorta still opening into the RPA. We describe a delayed presentation of iatrogenic anomalous origin of the RPA from the aorta, post APW ligation, and its management.
Assuntos
Defeito do Septo Aortopulmonar , Artéria Pulmonar , Humanos , Artéria Pulmonar/cirurgia , Defeito do Septo Aortopulmonar/cirurgia , Aorta/cirurgia , Ligadura , Doença IatrogênicaRESUMO
A 4-year-old female was diagnosed with Type II Aortopulmonary window after being seen for a murmur. By utilizing multimodality advanced imaging, including 2D and 3D echo, computed tomography (CTA) with semi-transparent overlay as a road map, we were able to preoperatively plan camera angles and device selection as well as close the defect percutaneously under echocardiography guidance. This report highlights the importance of a multimodality imaging approach to interventional procedures.
Assuntos
Defeito do Septo Aortopulmonar , Ecocardiografia Tridimensional , Feminino , Humanos , Pré-Escolar , Angiografia por Tomografia Computadorizada , Angiografia , Ecocardiografia/métodos , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgiaRESUMO
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.
Assuntos
Aorta Torácica , Defeito do Septo Aortopulmonar , Humanos , Recém-Nascido , Masculino , Aorta , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Aorta Torácica/anormalidades , Defeito do Septo Aortopulmonar/cirurgia , Defeito do Septo Aortopulmonar/complicações , Constrição Patológica/complicações , Resultado do TratamentoRESUMO
We report a case of a 2-day-old male with a diagnosis of interrupted aortic arch combined with aortopulmonary window suspected through echocardiography and confirmed by multidetector computer tomography (MDCT) angiography. Our case highlights how MDCT angiography was a key factor in planning surgical approach as it not only accurately defined aortic arch anatomy but also aortopulmonary window morphology.
Assuntos
Coartação Aórtica , Defeito do Septo Aortopulmonar , Masculino , Humanos , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Tomografia Computadorizada Multidetectores , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgia , Coartação Aórtica/complicações , Angiografia , Angiografia por Tomografia ComputadorizadaRESUMO
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.
Assuntos
Coartação Aórtica , Defeito do Septo Aortopulmonar , Anormalidades Cardiovasculares , Comunicação Interventricular , Tetralogia de Fallot , Masculino , Feminino , Humanos , Tomografia Computadorizada Multidetectores , Estudos Retrospectivos , Tetralogia de Fallot/complicações , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgia , Defeito do Septo Aortopulmonar/complicações , Artéria Pulmonar/cirurgia , Coartação Aórtica/cirurgia , Anormalidades Cardiovasculares/complicações , Angiografia , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Comunicação Interventricular/complicaçõesRESUMO
Anomalous ascending aortic origin of left pulmonary artery in association with aortopulmonary window has not been reported so far in literature and is the main highlight of the present case.
Assuntos
Defeito do Septo Aortopulmonar , Artéria Pulmonar , Aorta/anormalidades , Aorta/diagnóstico por imagem , Aorta/cirurgia , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgia , Humanos , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgiaRESUMO
OBJECTIVE: This study aims to compare both the pericardial roll technique with the patch augmentation technique of the unifocalization, and single-stage complete repair with the unifocalization and shunt for the repair of the ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: This was a retrospective review of the 48 patients undergoing unifocalization of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries from a single center. Our cohort had two surgical pathways, including single-stage midline unifocalization (n = 40), unifocalization after pulmonary artery rehabilitation by creating an aortopulmonary window or central shunt (n = 8). There were two surgical techniques in single-stage midline unifocalizaton, including widening of the pulmonary arteries with a patch (n = 30), and connecting pulmonary arteries with a pericardial roll (n = 10). RESULTS: A total of 14 (29.2%) of 48 patients underwent single-stage complete repair, 26 patients underwent shunt palliation with unifocalization. Combined early and late mortality was seen in seven patients in those who underwent shunt palliation with unifocalization, while it was seen in one patient in those who underwent a single-stage complete repair (mortality ratio 26.8% vs. 7.1%, p = .22). There was no statistically significant difference between the pericardial roll and patch augmentation techniques in terms of pulmonary artery reintervention (p = .65). Although all pulmonary artery reinterventions were for unilateral pulmonary artery in the roll technique group, 41.7% of reinterventions were for bilateral pulmonary arteries in the pericardial augmentation group. CONCLUSION: Single-stage complete repair of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries has better results than unifocalization with a shunt. In terms of nonvaluable raw material, the use of the pericardial roll technique is a considerable alternative for unifocalization.
Assuntos
Defeito do Septo Aortopulmonar , Comunicação Interventricular , Atresia Pulmonar , Aorta/anormalidades , Aorta/cirurgia , Defeito do Septo Aortopulmonar/cirurgia , Circulação Colateral , Defeitos dos Septos Cardíacos , Comunicação Interventricular/cirurgia , Humanos , Lactente , Artéria Pulmonar/anormalidades , Artéria Pulmonar/cirurgia , Atresia Pulmonar/cirurgia , Estudos RetrospectivosRESUMO
We present a case of aortopulmonary window in which the diagnosis of anomalous left coronary artery originating from pulmonary artery was made intra-operatively even if the coronary arteries anatomy was correctly studied pre-operatively with echocardiography. No evidence of coronary anomalies or indirect sings of coronary anomalies has been noted. Should we improve our pre-operative diagnostic accuracy and how?
Assuntos
Defeito do Septo Aortopulmonar , Anomalias dos Vasos Coronários , Humanos , Anomalias dos Vasos Coronários/diagnóstico por imagem , Anomalias dos Vasos Coronários/cirurgia , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgia , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Artéria Pulmonar/anormalidades , Ecocardiografia , CoraçãoAssuntos
Defeito do Septo Aortopulmonar , Adulto , Angiografia , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgia , Angiografia por Tomografia Computadorizada , Humanos , Valor Preditivo dos Testes , Artéria Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Background: The aim of this study was to review our institutional experience with patients who underwent surgical repair of aortopulmonary window, either as an isolated lesion or in association with other cardiac anomalies. Methods: Between January 2006 and December 2020, 183 patients underwent surgical repair of aortopulmonary window at our institute. Sixty-three patients had associated lesions (Group 1); 120 patients had isolated aortopulmonary window (Group 2). Median age was 7 months. Results: The early mortality in Group 1 was significantly higher (12.7%) compared to Group 2 (0.8%) (P = .001). The most common associated anomaly was ventricular septal defect (29 patients). On univariable analysis, cardiopulmonary bypass time (P < .001), aortic cross-clamp time (P < .001), delayed chest closure (P = .02), sepsis (P = .006), tracheostomy (P = .002), extracorporeal membrane oxygenation (P < .001), associated lesions (P = .001), pulmonary artery hypertensive crisis (P < .001) were predictors for early mortality. On multivariable analysis only pulmonary artery hypertensive crisis was identified as predictor for early mortality (P = .03; odds ratio = 24). Survival at both 5 years and 8 years was 77% ± 6.5 in Group 1 and 98.8% ± 1.2 in Group 2 (P≤.001). Freedom from reintervention at both 5 years and 8 years was 92.4% ± 5.2 in Group 1 and 100% in Group 2 (P = .055). Conclusion: Early outcomes of aortopulmonary window repair are excellent among patients in which this is an isolated lesion, as compared to those with associated lesions. Long-term outcomes in terms of freedom from reoperation are excellent in both the groups.
Assuntos
Defeito do Septo Aortopulmonar , Comunicação Interventricular , Hipertensão Pulmonar , Aorta/cirurgia , Defeito do Septo Aortopulmonar/cirurgia , Seguimentos , Comunicação Interventricular/cirurgia , Humanos , Hipertensão Pulmonar/cirurgia , Lactente , Reoperação , Resultado do TratamentoRESUMO
Although the conventional treatment of aortopulmonary (AP) window consists of reconstructive surgery with the use of cardiopulmonary bypass (CPB), some conditions like low birth weight or active respiratory tract bleeding may lead to diverting therapeutic options. We present a case of a premature 1.9â kg neonate with severe pulmonary arterial hypertension based on the association of an AP window and large patent ductus arteriosus. Because of intrabronchial hemorrhage, a conservative strategy was chosen excluding the need for heparinization and CPB. Through median sternotomy, the ductus arteriosus and AP window were clipped, effectively occluding both shunts. The postoperative course was uneventful with a rapid decrease of pulmonary artery pressure. Although classical surgical reconstruction is still advocated as primary therapy, this case illustrates the suitability of an alternative approach without the need for CPB and full heparinization in a patient with an increased risk of bleeding complications.
Assuntos
Defeito do Septo Aortopulmonar , Permeabilidade do Canal Arterial , Recém-Nascido , Humanos , Defeito do Septo Aortopulmonar/cirurgia , Permeabilidade do Canal Arterial/cirurgia , Recém-Nascido de Baixo Peso , HemorragiaRESUMO
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.
Assuntos
Defeito do Septo Aortopulmonar , Síndrome de Bland-White-Garland , Anomalias dos Vasos Coronários , Disfunção Ventricular , Defeito do Septo Aortopulmonar/complicações , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgia , Síndrome de Bland-White-Garland/diagnóstico , Síndrome de Bland-White-Garland/diagnóstico por imagem , Criança , Anomalias dos Vasos Coronários/complicações , Anomalias dos Vasos Coronários/diagnóstico por imagem , Anomalias dos Vasos Coronários/cirurgia , Humanos , Lactente , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgiaRESUMO
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.
Assuntos
Defeito do Septo Aortopulmonar , Artéria Pulmonar , Angiografia , Defeito do Septo Aortopulmonar/complicações , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgia , Pré-Escolar , Humanos , Masculino , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgiaRESUMO
We report a case of advanced age at presentation of interrupted aortic arch with aortopulmonary window. Although the association between these two defects is well established, their overall prevalence is extremely low. This case presents us with an excellent opportunity to discuss decision making regarding aortic arch repair techniques according to age at which primary surgical correction is intended.
Assuntos
Coartação Aórtica , Defeito do Septo Aortopulmonar , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Coartação Aórtica/complicações , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/cirurgia , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgia , HumanosRESUMO
We report a patient with an acquired traumatic aortopulmonary window. The patient presented with an aortopulmonary fistula between the proximal ascending aorta and pulmonary trunk, which was missed on the initial hospital admission. The 26-year-old patient presented with high-output cardiac failure and examination features of a diastolic runoff. Patch closure of the defect using a sandwich technique was undertaken, with resolution of symptoms.