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Radiology. Cardiothoracic Imaging Jun 2022Conotruncal anomalies, also referred to as outflow tract anomalies, are congenital heart defects that result from abnormal septation of the great vessels' outflow... (Review)
Review
Conotruncal anomalies, also referred to as outflow tract anomalies, are congenital heart defects that result from abnormal septation of the great vessels' outflow tracts. The major conotruncal anomalies include tetralogy of Fallot, double-outlet right ventricle, transposition of the great arteries, truncus arteriosus, and interrupted aortic arch. Other defects, which are often components of the major anomalies, include pulmonary atresia with ventricular septal defect, pulmonary valve agenesis, aortopulmonary window, and double-outlet left ventricle. CT has emerged as a robust diagnostic tool in preoperative and postoperative assessment of various congenital heart diseases, including conotruncal anomalies. The data provided with multidetector CT imaging are useful for treatment planning and follow-up monitoring after surgery or intervention. Unlike echocardiography and MRI, CT is not limited by a small acoustic window, metallic devices, and need for sedation or anesthesia. Major advances in CT equipment, including dual-source scanners, wide-detector scanners, high-efficiency detectors, higher x-ray tube power, automatic tube current modulation, and advanced three-dimensional postprocessing, provide a low-risk, high-quality alternative to diagnostic cardiac catheterization and MRI. This review explores the various conotruncal anomalies and elucidates the role of CT imaging in their pre- and postoperative assessment. CT, CT Angiography, Stents, Pediatrics © RSNA, 2022.
PubMed: 35923747
DOI: 10.1148/ryct.210089 -
Annals of Pediatric Cardiology 2021Being a planar structure, fetal diagnosis of aorto-pulmonary window poses great challenge. A few echocardiographic signs can help to clinch the diagnosis.
Being a planar structure, fetal diagnosis of aorto-pulmonary window poses great challenge. A few echocardiographic signs can help to clinch the diagnosis.
PubMed: 34103873
DOI: 10.4103/apc.APC_42_20 -
Journal of the Saudi Heart Association 2020Aortopulmonary window (APW) is a rare congenital heart defect. It occurs as an isolated cardiac lesion or in association with other cardiac anomalies and rarely with...
OBJECTIVE
Aortopulmonary window (APW) is a rare congenital heart defect. It occurs as an isolated cardiac lesion or in association with other cardiac anomalies and rarely with abnormal coronary arteries. The spectrum of cardiovascular anomalies associated with APW and overall management and outcome in the current era were reviewed.
METHODS
Between 2001 and 2018, all patients diagnosed with APW were included. Based on associated cardiovascular anomalies, those patients were divided into 2 groups: simple APW group and complex APW group (APW with associated other cardiovascular anomalies). All cases were followed longitudinally. The outcomes are described.
RESULT
Twenty patients underwent APW repair including 2 (10%) in simple APW group and 18 (90%) in complex APW group. Their mean age and weight were 4.8 ± 1.8 months and 4 ± 0.4 kg, respectively. APW Type I was confirmed in 65% followed by Type III in 20% and then Type II in 15% of the patients. In the complex APW group, atrial septal defect was the commonest associated cardiac lesion occurring in 8/20 (40%), followed by ventricular septal defect, interrupted aortic arch, and pulmonary artery anomalies in 25% of each. The presence of patent ductus arteriosus (PDA) was found in 40% of APW cases with 2/3rd of them in association with interrupted aortic arch. Two patients (10%) had unusual coronary anomalies that required repair, both with APW Type I. Associated non-cardiac anomalies were found in 30% of cases. Risk Adjustment for Congenital Heart Surgery (RACHS-1) score frequencies were between 2 and 4. Only one patient had reactive pulmonary hypertension related to chronic lung disease. All patients underwent surgical correction with median age of 2 month at the time of repair (interquartile range, 2 weeks to 4.5 months). Mean duration of mechanical ventilation, pediatric cardiac ICU and hospital length of stay were 2.8 ± 0.5, 9 ± 3 and 26 ± 6 days, respectively. All patients survived with no residual APW with mean follow-up duration of 4.5 years.
CONCLUSION
Majority of APW are associated with other cardiovascular anomalies (90%) including coronary abnormalities (10%). Early surgical repair of APW and associated lesions showed excellent survival rate, freedom from re-intervention need within an average of 4.5 years of follow up and no evidence of persistent pulmonary hypertension post repair.
PubMed: 33154906
DOI: 10.37616/2212-5043.20 -
Autopsy & Case Reports Apr 2021Aortopulmonary window (APW) is a rare congenital heart defect with abnormal communication between the ascending aorta and the pulmonary trunk with two separate semilunar... (Review)
Review
Aortopulmonary window (APW) is a rare congenital heart defect with abnormal communication between the ascending aorta and the pulmonary trunk with two separate semilunar valves. We present an autopsy case report wherein a young primigravida woman presented with progressive breathlessness and central cyanosis at 21 weeks of gestation. Echocardiography performed in the emergency room revealed elevated right-sided cardiac pressures suggestive of severe pulmonary hypertension; however, no structural cardiac defect was discernible. The patient succumbed to congestive cardiac failure and progressive hypoxia within 5 days of hospitalization. The autopsy revealed a Type I aortopulmonary window (2 cm) with patent ductus arteriosus. The lungs showed changes of severe pulmonary hypertension with superadded bronchopneumonia. This report underscores a rare presentation of APW, undiagnosed until pregnancy, leading to the Eisenmenger syndrome and death.
PubMed: 33968832
DOI: 10.4322/acr.2021.265 -
Ultrasound in Obstetrics & Gynecology :... Apr 2017Aortopulmonary window is a rare congenital cardiac anomaly characterized by communication between the aorta and the pulmonary artery above the semilunar valves. Prenatal... (Review)
Review
Aortopulmonary window is a rare congenital cardiac anomaly characterized by communication between the aorta and the pulmonary artery above the semilunar valves. Prenatal diagnosis is rare. We report four fetuses with aortopulmonary window and review the relevant literature. Approximately half of the reported cases had additional cardiac defects. None had chromosomal abnormalities. In cases with normal cardiac connections, the diagnosis can be made prenatally on the standard three-vessel view, as seen in two of our cases. In one fetus with complete transposition of the great arteries, the diagnosis was made retrospectively on sagittal views. In the remaining case, the window was seen postnatally but could not be identified retrospectively due to the abnormal superoinferior relationship of the ventricles and vessels. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.
Topics: Adult; Aorta, Thoracic; Early Diagnosis; Echocardiography; Female; Heart Defects, Congenital; Humans; Prenatal Diagnosis; Prospective Studies; Pulmonary Artery; Sensitivity and Specificity
PubMed: 27063344
DOI: 10.1002/uog.15936 -
Turk Gogus Kalp Damar Cerrahisi Dergisi Jan 2018In this study, we aimed to report our single-center experience in aortopulmonary window and review clinical signs, symptoms, surgical correction techniques, and...
BACKGROUND
In this study, we aimed to report our single-center experience in aortopulmonary window and review clinical signs, symptoms, surgical correction techniques, and long-term outcomes.
METHODS
We retrospectively reviewed the medical records of a total of 30 patients who were followed with the diagnosis of aortopulmonary window in our hospital between May 1998 and June 2016. The clinical characteristics of the patients, echocardiographic and angiographic findings, surgical treatment outcomes, and medical problems during follow-up were reviewed.
RESULTS
The most common signs and symptoms were murmur, dyspnea, tachypnea, growth retardation, and signs of congestive heart failure. The mean age at the time of surgery was 8.2±14.4 months (7 days to 60 months). Eighteen patients (60%) had additional congenital cardiac anomalies. Eleven patients had simple congenital heart diseases, and seven patients had complex congenital heart diseases. Four patients were unable to be operated due to Eisenmenger syndrome (n=3) and complex congenital heart disease (n=1). No early or late postoperative death was observed. The mean follow-up was 6.4±4.8 years (range, 5 months to 16 years). In addition to aortopulmonary window repair, an additional cardiac anomaly modifying surgical intervention was corrected in nine patients (34.6%). One patient was reoperated for residual aortopulmonary window and another patient for pulmonary stenosis (valvular, supravalvar) after three years. One of these patients underwent pulmonary balloon valvuloplasty after two years. The reoperation rate was 7.7% (n=2) during follow-up.
CONCLUSION
Aortopulmonary window is a rare cardiac anomaly which may be overlooked by echocardiographic study, and which is amenable for repair with low-surgical risk. It is, therefore, imperative to diagnose and treat this condition, before pulmonary vascular disease develops.
PubMed: 32082708
DOI: 10.5606/tgkdc.dergisi.2018.14772 -
Turk Gogus Kalp Damar Cerrahisi Dergisi Oct 2022The aim of this study is to present our experience regarding the creation of an aortopulmonary window as the initial palliative procedure.
BACKGROUND
The aim of this study is to present our experience regarding the creation of an aortopulmonary window as the initial palliative procedure.
METHODS
Between February 2016 and February 2021, a total of eight patients (3 males, 5 females; median age: 2 months; range, 0.7 to 6 months) who underwent aortopulmonary window creation were retrospectively analyzed. Data collection was performed by review from our institution.
RESULTS
There was no occurrence of early or late mortality in any patient. The median postoperative duration of mechanical ventilation and length of hospital stay were five and eight days, respectively. No postoperative reperfusion injury or severe pulmonary overcirculation was observed in any of the patients. Four patients achieved complete repair with unifocalization of the major aortopulmonary collateral arteries, one patient had a second procedure, and the remaining three patients waited for complete repair. The median right ventricle-toaortic pressure ratio after complete repair was 0.6 (range, 0.4 to 0.7). The median follow-up after complete repair was 1.4 (range, 0.9 to 2.8) years, and the median follow-up period for all survivors was 2.7 (range, 0.9-5.8) years.
CONCLUSION
Our study results suggest that aortopulmonary window operation can be safely performed in selected patients with good early and mid-term outcomes. Although the central pulmonary arteries are very small, half of the patients underwent complete repair and achieved acceptable right ventricle-to-aortic pressure ratios. Patient selection criteria and early postoperative interventions are of utmost importance to prevent postoperative pulmonary overcirculation.
PubMed: 36605316
DOI: 10.5606/tgkdc.dergisi.2022.23161 -
Pediatric Cardiology Jan 2022The topsy-turvy heart is a very rare cardiac malformation that involves a global 90° clockwise rotation of the heart along its long axis. This rotation results in the... (Review)
Review
The topsy-turvy heart is a very rare cardiac malformation that involves a global 90° clockwise rotation of the heart along its long axis. This rotation results in the displacement of the great arteries and severe elongation and stretching of the brachiocephalic arteries and the bronchi. We present an unusual case of topsy-turvy heart diagnosed prenatally with a large aorto-pulmonary window and. This case gives an insight into the morphological details and clinical presentation of this rare malformation and its associated complications. We also present a review of the literature of this rare anomaly showing only 15 live cases that have been published with only three cases diagnosed prenatally.
Topics: Aortopulmonary Septal Defect; Female; Heart Defects, Congenital; Humans; Pregnancy; Prenatal Diagnosis
PubMed: 34448896
DOI: 10.1007/s00246-021-02710-1 -
The American Journal of Case Reports Dec 2023BACKGROUND An aortopulmonary window (APW) is an uncommon congenital defect of the septation between the ascending aorta and pulmonary trunk. The combination of APW and...
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.
Topics: Humans; Infant, Newborn; Male; Aorta; Aorta, Thoracic; Aortopulmonary Septal Defect; Constriction, Pathologic; Treatment Outcome
PubMed: 38124359
DOI: 10.12659/AJCR.942193 -
The Journal of Thoracic and... Nov 2021
Review
Topics: Cardiac Valve Annuloplasty; Child; Child, Preschool; Heart Valve Diseases; Humans; Infant; Infant, Newborn; Treatment Outcome; Truncus Arteriosus, Persistent
PubMed: 33487419
DOI: 10.1016/j.jtcvs.2020.10.161