Innovative cardiovascular casting technique features the complex malformation of berry syndrome.

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.

Iatrogenic Anomalous Origin of the Right Pulmonary Artery From the Aorta: A Rare Complication of Ligation of Aortopulmonary Window.

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.

3D Echocardiographic and Computed Tomographic Angiography Guidance for Percutaneous Closure of a Type II (Intermediate) Aortopulmonary Window.

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.

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.

A rare disease detected in a school-age child aortopulmonary window with anomalous right coronary artery from the pulmonary artery.

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.

A rare coexistence: Hammock mitral valve and aortopulmonary window.

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.

Interrupted Aortic Arch Associated With Aortopulmonary Window: The Role of Multidetector Computed Tomography Angiography.

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.

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.

Aortopulmonary window with anomalous ascending aortic origin of left pulmonary artery: Hitherto unreported pattern.

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.

Unifocalization with pericardial roll technique in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

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.

Aortopulmonary window and coronary anatomy - still a pre-operative diagnostic trouble!

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?

Delayed presentation of aorto-pulmonary window, double outlet right ventricle with pulmonary arterial hypertension: Illustration of uncommon association on dual-energy CT.

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.

Surgical Management of Aortopulmonary Window and its Associated Cardiac Lesions.

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.

Clipping of an Aortopulmonary Window in a Small Neonate With Bronchial Bleeding: A Simple Solution for a Complex Condition.

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 1.9 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.

Anomalous origin of one pulmonary artery from aorta: Evaluation on computed tomography angiography.

AIM: The present study sought to evaluate the morphology and associated cardiovascular anomalies in patients with anomalous origin of one pulmonary artery from aorta (AOPA) on CT angiography. MATERIALS AND METHODS: A retrospective search of the departmental database from January 2014 to September 2021 was conducted to identify patients diagnosed with AOPA on CT angiography. The subtype of AOPA, site of origin, and associated cardiovascular abnormalities were assessed. RESULTS: AOPA was identified in 27 patients (19 males, mean age: 8.8 years). Anomalous origin of the right pulmonary artery from aorta (AORPA) was observed in 17/27 (63%) patients, while anomalous origin of the left pulmonary artery (AOLPA) was seen in 10/27 (37%) patients. The proximal and distal subtypes were observed in 24/27 (88.9%) and 3/27 (11.1%) patients respectively. AOPA was associated with other cardiac anomalies in 92.6% (25/27) patients. Patent arterial duct (11/17; 64.7%) was the most frequently anomaly associated with AORPA, while tetralogy of Fallot (10/10; 100%) was the most commonly anomaly associated with AOLPA. The anomalous pulmonary artery was contralateral to the aortic arch in 23/27 (85.2%) patients. Right-sided aortic arch was observed in only 2/17 (11.8%) patients with AORPA and 8/10 (80%) patients with AOLPA. In 2/10 (20%) patients, AORPA was associated with aortopulmonary window and aortic arch anomaly. CONCLUSION: AOPA is rare and frequently associated with other cardiovascular anomalies like patent arterial duct, tetralogy of Fallot, and right-sided aortic arch. CT angiography helps in providing exact anatomical delineation and identifying associated anomalies, thus aiding preoperative planning of surgical management.