RESUMEN
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.
Asunto(s)
Defecto del Tabique Aortopulmonar , Procedimientos Quirúrgicos Cardíacos , Humanos , Femenino , Masculino , Defecto del Tabique Aortopulmonar/cirugía , Defecto del Tabique Aortopulmonar/mortalidad , Recién Nacido , Lactante , Estudios Retrospectivos , Procedimientos Quirúrgicos Cardíacos/métodos , Resultado del Tratamiento , Complicaciones Posoperatorias/epidemiología , Estudios de Seguimiento , Factores de TiempoRESUMEN
BACKGROUND: Prenatal diagnosis of Berry syndrome, a rare combination of cardiac anomalies including aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), interrupted aortic arch (IAA), hypoplastic aortic arch, or coarctation of the aorta (COA), poses a significant challenge. Due to the rarity of the disease, and the limited case reports available to features the complex malformation of Berry syndrome postpartum, this article introduces an innovative approach to visually showcase this unusual disease. The proposed method provides a comprehensive display of the structural deformities, offering valuable insights for clinical practitioners seeking to comprehend this condition. CASE PRESENTATION: In this report, we present a case where fetal echocardiography aided in diagnosing Berry syndrome, which was later confirmed through postpartum cardiovascular casting. Our experience highlights the importance of using the three-vessel view to diagnose APW and aortic origin of the right pulmonary artery. Additionally, obtaining true cross-sectional and sagittal views by continuously scanning from the three-vessel-trachea view to the long-axis view of the aortic arch is necessary to image IAA or coarctation of the aortic arch. CONCLUSIONS: Early and accurate prenatal diagnosis of Berry syndrome is feasible and our cardiovascular cast can perfectly display the microvascular morphology of the fetal heart, which may have great application prospects for postpartum diagnosis and teaching of complex cardiac abnormalities.
Asunto(s)
Defecto del Tabique Aortopulmonar , Cardiopatías Congénitas , Embarazo , Femenino , Humanos , Estudios Transversales , Aorta Torácica/anomalías , Aorta/anomalías , Arteria PulmonarRESUMEN
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.
Asunto(s)
Defecto del Tabique Aortopulmonar , Arteria Pulmonar , Humanos , Arteria Pulmonar/cirugía , Defecto del Tabique Aortopulmonar/cirugía , Aorta/cirugía , Ligadura , Enfermedad IatrogénicaRESUMEN
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.
Asunto(s)
Defecto del Tabique Aortopulmonar , Ecocardiografía Tridimensional , Femenino , Humanos , Preescolar , Angiografía por Tomografía Computarizada , Angiografía , Ecocardiografía/métodos , Defecto del Tabique Aortopulmonar/diagnóstico por imagen , Defecto del Tabique Aortopulmonar/cirugíaRESUMEN
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.
Asunto(s)
Aorta Torácica , Defecto del Tabique Aortopulmonar , Humanos , Recién Nacido , Masculino , Aorta , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/cirugía , Aorta Torácica/anomalías , Defecto del Tabique Aortopulmonar/cirugía , Defecto del Tabique Aortopulmonar/complicaciones , Constricción Patológica/complicaciones , Resultado del TratamientoRESUMEN
Aortopulmonary window is a condition characterized by a communication between the pulmonary artery and the ascending aorta. The coexistence of aortopulmonary window and an anomalous right coronary artery originating from the pulmonary artery is rarely observed together, as mentioned in previous studies. In this report, we aim to describe our diagnostic and treatment experiences with a 6-year-old patient diagnosed with aortopulmonary window associated with an abnormal origin of the right coronary artery from the pulmonary artery.
Asunto(s)
Defecto del Tabique Aortopulmonar , Anomalías de los Vasos Coronarios , Humanos , Niño , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/anomalías , Enfermedades Raras/complicaciones , Anomalías de los Vasos Coronarios/complicaciones , Anomalías de los Vasos Coronarios/diagnóstico , Defecto del Tabique Aortopulmonar/diagnóstico , Defecto del Tabique Aortopulmonar/diagnóstico por imagenRESUMEN
Congenital mitral stenosis is a broad-spectrum pathology in which blood flow to the left ventricle is obstructed both functionally and anatomically. Hammock mitral valve, also known as anomalous mitral arcade, is a rare congenital anomaly particularly in infants and children. Hammock mitral valve may not be suitable for repair regarding the advanced dysplastic mitral valve structure. Aortopulmonary window is an unusual cardiac anomaly which is defined as a communication between the main pulmonary artery and the ascending aorta. As a result of the excessive left-to-right shunt, early intervention and surgical closure deemed mandatory to avoid development of severe pulmonary hypertension and its consequences. All patients with an aortopulmonary window necessitates prompt repair immediately. In this brief report, mitral valve replacement with a mechanical valve and repair of aortopulmonary window with a Dacron patch were performed simultaneously in a 5-month-old patient with a hammock mitral valve and accompanying aortopulmonary window.
Asunto(s)
Defecto del Tabique Aortopulmonar , Insuficiencia de la Válvula Mitral , Estenosis de la Válvula Mitral , Lactante , Niño , Humanos , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/cirugía , Válvula Mitral/anomalías , Defecto del Tabique Aortopulmonar/diagnóstico , Defecto del Tabique Aortopulmonar/diagnóstico por imagen , Estenosis de la Válvula Mitral/complicaciones , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Arteria Pulmonar/anomalías , Insuficiencia de la Válvula Mitral/complicaciones , Insuficiencia de la Válvula Mitral/diagnóstico , Insuficiencia de la Válvula Mitral/cirugíaRESUMEN
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.
Asunto(s)
Coartación Aórtica , Defecto del Tabique Aortopulmonar , Masculino , Humanos , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/cirugía , Tomografía Computarizada Multidetector , Defecto del Tabique Aortopulmonar/diagnóstico por imagen , Defecto del Tabique Aortopulmonar/cirugía , Coartación Aórtica/complicaciones , Angiografía , Angiografía por Tomografía ComputarizadaRESUMEN
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.
Asunto(s)
Coartación Aórtica , Defecto del Tabique Aortopulmonar , Anomalías Cardiovasculares , Defectos del Tabique Interventricular , Tetralogía de Fallot , Masculino , Femenino , Humanos , Tomografía Computarizada Multidetector , Estudios Retrospectivos , Tetralogía de Fallot/complicaciones , Defecto del Tabique Aortopulmonar/diagnóstico por imagen , Defecto del Tabique Aortopulmonar/cirugía , Defecto del Tabique Aortopulmonar/complicaciones , Arteria Pulmonar/cirugía , Coartación Aórtica/cirugía , Anomalías Cardiovasculares/complicaciones , Angiografía , Defectos del Tabique Interventricular/diagnóstico por imagen , Defectos del Tabique Interventricular/cirugía , Defectos del Tabique Interventricular/complicacionesRESUMEN
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.
Asunto(s)
Defecto del Tabique Aortopulmonar , Arteria Pulmonar , Aorta/anomalías , Aorta/diagnóstico por imagen , Aorta/cirugía , Defecto del Tabique Aortopulmonar/diagnóstico por imagen , Defecto del Tabique Aortopulmonar/cirugía , Humanos , Arteria Pulmonar/anomalías , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugíaRESUMEN
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.
Asunto(s)
Defecto del Tabique Aortopulmonar , Defectos del Tabique Interventricular , Atresia Pulmonar , Aorta/anomalías , Aorta/cirugía , Defecto del Tabique Aortopulmonar/cirugía , Circulación Colateral , Defectos de los Tabiques Cardíacos , Defectos del Tabique Interventricular/cirugía , Humanos , Lactante , Arteria Pulmonar/anomalías , Arteria Pulmonar/cirugía , Atresia Pulmonar/cirugía , Estudios RetrospectivosRESUMEN
We present a case of a 26-year-old female with double outlet right ventricle and proximal aorto-pulmonary window (APW). The case highlights the role of computed tomography angiography in the diagnosis and characterization of APW defects, which are difficult to be diagnosed on transthoracic echocardiography.
Asunto(s)
Defecto del Tabique Aortopulmonar , Ventrículo Derecho con Doble Salida , Hipertensión Arterial Pulmonar , Adulto , Ventrículo Derecho con Doble Salida/complicaciones , Ventrículo Derecho con Doble Salida/diagnóstico por imagen , Ventrículo Derecho con Doble Salida/cirugía , Ecocardiografía , Femenino , Humanos , Tomografía Computarizada por Rayos XRESUMEN
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?
Asunto(s)
Defecto del Tabique Aortopulmonar , Anomalías de los Vasos Coronarios , Humanos , Anomalías de los Vasos Coronarios/diagnóstico por imagen , Anomalías de los Vasos Coronarios/cirugía , Defecto del Tabique Aortopulmonar/diagnóstico por imagen , Defecto del Tabique Aortopulmonar/cirugía , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Arteria Pulmonar/anomalías , Ecocardiografía , CorazónAsunto(s)
Defecto del Tabique Aortopulmonar , Adulto , Angiografía , Defecto del Tabique Aortopulmonar/diagnóstico por imagen , Defecto del Tabique Aortopulmonar/cirugía , Angiografía por Tomografía Computarizada , Humanos , Valor Predictivo de las Pruebas , Arteria Pulmonar/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
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.