Non-invasive diagnosis of aortic arch anomalies in children - 15 years of own experience.

Aim: To summarize our experience in echocardiographic diagnosis of aortic arch anomalies in pediatric patients. Materials and methods: A retrospective assessment of echocardiographic findings in Echo-Lab patients of the Pediatric Cardiac Surgery Department, who were diagnosed with an anomalous aortic arch between 2003 and 2018. Results: The diagnosis of an abnormal course of the aortic arch and/or its branches was established in 115 children aged between 4 days and 17 years. The following types of anomalies were detected: left aortic arch with aberrant right subclavian artery in 42 patients; right aortic arch with left brachiocephalic trunk in 14 patients; right aortic arch, aberrant left subclavian artery in 36 patients; double aortic arch in 14 patients; and other, more complex types in 9 patients. The main elements of defects were correctly identified by echo in all cases. We decided to additionally perform computed tomography angiography in 32 patients to clarify all details necessary to qualify patients for surgery and establish the surgical plan. Conclusions: 1. Echocardiography strictly following the pre-determined protocol has 100% sensitivity in the detection of basic elements of aortic arch anomaly and is a perfect tool for diagnostic process initiation. 2. Since it is not possible to visualize all anatomic details, the qualification for surgery should by based on computed tomography angiography or cardiac magnetic resonance imaging, which precisely visualize both abnormal vessels and compressed structures.Aim: To summarize our experience in echocardiographic diagnosis of aortic arch anomalies in pediatric patients. Materials and methods: A retrospective assessment of echocardiographic findings in Echo-Lab patients of the Pediatric Cardiac Surgery Department, who were diagnosed with an anomalous aortic arch between 2003 and 2018. Results: The diagnosis of an abnormal course of the aortic arch and/or its branches was established in 115 children aged between 4 days and 17 years. The following types of anomalies were detected: left aortic arch with aberrant right subclavian artery in 42 patients; right aortic arch with left brachiocephalic trunk in 14 patients; right aortic arch, aberrant left subclavian artery in 36 patients; double aortic arch in 14 patients; and other, more complex types in 9 patients. The main elements of defects were correctly identified by echo in all cases. We decided to additionally perform computed tomography angiography in 32 patients to clarify all details necessary to qualify patients for surgery and establish the surgical plan. Conclusions: 1. Echocardiography strictly following the pre-determined protocol has 100% sensitivity in the detection of basic elements of aortic arch anomaly and is a perfect tool for diagnostic process initiation. 2. Since it is not possible to visualize all anatomic details, the qualification for surgery should by based on computed tomography angiography or cardiac magnetic resonance imaging, which precisely visualize both abnormal vessels and compressed structures.

Methodology of echocardiographic analysis of morphological variations of the aortic arch and its branches in children - own experience.

We wish to share our experience in echocardiographic assessment of the course of the aortic arch, illustrating it with multiple examples of the majority of possible variants. The course of the aortic arch and its branches may be visualized using high parasternal and suprasternal views in sagittal and transverse planes. It is hardly ever possible to visualize the entire aortic arch on a single ultrasonographic section, particularly in the case of pathological variations. Echocardiography should be performed in a dynamic mode, as in the case of CT angiography or magnetic resonance, by gradually moving the ultrasound beam and following the position of subsequent aortic segments and branches on the screen. Due to disturbances in ultrasound propagation caused by air-containing tissues, such as the trachea, bronchi and lungs as well as bones (sternum and ribs), each evaluation of the entire arch requires the use of a higher number of echocardiographic views. The presented data show that echocardiographic detection of the main details of aortic arch anomalies is possible in practically all cases. In the case of patients considered for surgical treatment, all unresolved issues should be clarified with CT angiography or MRI, enabling 3 dimensional reconstruction of vessels and other thoracic structures. Knowledge of the main elements of an abnormal arch is crucial for proper planning of this type of examination; therefore the diagnostic process should be always initiated with echocardiography. Echocardiography is often sufficient to answer all clinical questions and finalize the diagnostic process.We wish to share our experience in echocardiographic assessment of the course of the aortic arch, illustrating it with multiple examples of the majority of possible variants. The course of the aortic arch and its branches may be visualized using high parasternal and suprasternal views in sagittal and transverse planes. It is hardly ever possible to visualize the entire aortic arch on a single ultrasonographic section, particularly in the case of pathological variations. Echocardiography should be performed in a dynamic mode, as in the case of CT angiography or magnetic resonance, by gradually moving the ultrasound beam and following the position of subsequent aortic segments and branches on the screen. Due to disturbances in ultrasound propagation caused by air-containing tissues, such as the trachea, bronchi and lungs as well as bones (sternum and ribs), each evaluation of the entire arch requires the use of a higher number of echocardiographic views. The presented data show that echocardiographic detection of the main details of aortic arch anomalies is possible in practically all cases. In the case of patients considered for surgical treatment, all unresolved issues should be clarified with CT angiography or MRI, enabling 3 dimensional reconstruction of vessels and other thoracic structures. Knowledge of the main elements of an abnormal arch is crucial for proper planning of this type of examination; therefore the diagnostic process should be always initiated with echocardiography. Echocardiography is often sufficient to answer all clinical questions and finalize the diagnostic process.

Anomalous origin of the right coronary artery from the main pulmonary artery treated surgically in a 6-week-old infant. A case report and review of the literature.

BACKGROUND: In the present paper, the authors describe a case of a rare congenital defect - anomalous origin of the right coronary artery from the main pulmonary artery diagnosed in a 5-week-old infant who was deemed eligible for surgical treatment based solely on echocardiography. Such anatomical abnormalities of the coronary arteries are subtle and thus extremely difficult to visualize, especially in patients in whom permanent extensive damage of the cardiac muscle has not yet occurred. For this reason, the diagnosis is usually established when the disease is highly advanced, oftentimes only postmortem. In this paper, the authors present early echocardiographic evaluation, successful surgical treatment and post-operative echocardiographic examination. CASE PRESENTATION: Fetal echocardiographic examinations demonstrated single vascular anomalies. The suspicion was verified after birth. Other congenital defects, including genetic defects, were also investigated. In the 5th week of life, cardiac ECHO showed abnormal origin of the right coronary artery from the trunk of the pulmonary artery. The child was deemed eligible for surgical treatment of the defect. The surgical translocation of the ostium of the right coronary artery to the aorta was done with success, and the child was discharged on the 14th day of hospitalization. CONCLUSION: The present case report demonstrates that careful examination of the child with precise echocardiographic examination makes it possible to establish an early diagnosis of this rare defect. This discrete defect can be treated early, before the symptoms, often connected with irreversible changes in the myocardium, appear.

Double-chambered right ventricle in a 16-year-old patient with Williams syndrome.

We present a case of double-chambered right ventricle diagnosed during preparation for colonoscopy due to gastrointestinal bleeding in a 16-year-old, mentally disabled boy with Williams syndrome. The patient was previously diagnosed with ventricular septal defect and mild pulmonary stenosis. Echocardiography performed under general anesthesia revealed hypertrophied muscular bundles in the right ventricle with the maximum gradient of 100 mmHg, causing severe outflow obstruction. This type of defect is extremely rare in patients with Williams syndrome, with only one case, which was diagnosed during invasive angiocardiography, described in world literature. A successful total surgical correction was performed based on echocardiography data.

Anomalous connection of the left hepatic vein to coronary sinus in a child with PAPVD. Surgical significance and diagnostic difficulties.

Left hepatic vein (LHV) that drains blood into a coronary sinus (CS) is an extremely rare congenital anomaly of systemic vein drainage with only single reports published. In most of these cases the unusual venous connection was found incidentally during diagnostics or surgery. The case of a two-year-old boy in whom the anomaly was discovered during open heart surgery for partial anomalous pulmonary venous drainage (PAPVD) is presented. Difficulties in obtaining proper diagnosis preoperatively are confronted with postoperative echo findings. Embryology and evolution of sinus venosus are discussed to explain the persistent connection between hepatic venous circulation and a coronary sinus. The authors attempt to recapitulate the possible surgical consequences of LHV-CS continuity.

The myocardial performance index in children after surgical correction of congenital malformations with intracardiac shunt.

BACKGROUND: The lack of a simple and clinically useful method of myocardial function assessment makes it difficult to monitor patients after repair of congenital cardiac malformations. The myocardial performance index (MPI) introduced in 1995 by Chuwa Tei may help to precisely follow the course of postoperative myocardial function disturbances. AIM: To assess the usefulness of MPI for cardiac function evaluation following surgical correction of congenital defects performed in cardiopulmonary bypass and to evaluate it's prognostic value. METHODS: This prospective study involved 73 children who underwent surgery for simple defects with intracardiac shunt--34 children with atrial septal defect (ASD) and 39 with ventricular septal defect (VSD). In the examined patient group, MPI and the parameters of cardiac index were measured in the early postoperative period. Then the results were correlated with congenital defect severity and postoperative course. The control group consisted of 77 healthy children. RESULTS: In the control group of healthy children, mean values of the right (RVMPI) and the left ventricular MPI (LVMPI) were 0.32+/-0.06 and 0.33+/-0.05, respectively. In children after ASD correction, MPI was elevated only for the right ventricle (mean value of 0.41+/-0.07). Meanwhile in children operated on for VSD, both the RVMPI and LVMPI values increased postoperatively. RVMPI reached a peak value on the 2nd postoperative day (0.48+/-0.20) while LVMPI reached a peak value between the 3rd and 5th day after surgery (0.49+/-0.10). A significant and strong correlation between severity of VSD and MPI values was observed. A marked correlation between MPI calculated on the 1st postoperative day and the course after surgery was also noted. CONCLUSIONS: The MPI is a precise tool assessing both systolic and diastolic ventricular performance in the early postoperative period following surgical correction of a congenital defect with intracardiac shunt and is also a useful predictive factor of the postoperative course.

[Echocardiographic abnormalities in infants with heart murmur]. / Nieprawidlowosci w badaniach echokardiograficznych u noworodków ze stwierdzonym szmerem nad sercem.

OBJECTIVES: Congenital heart diseases are very serious problem of current perinatology. Early detection of congenital heart diseases and new possibilities of their treatment have decreased mortality rate in neonates. THE AIM OF THE STUDY: Was to present the reasons of the heart murmurs found in newborns, based on postnatal echocardiography. MATERIAL AND METHODS: The study group consisted of 107 neonates born in The Obstetrics and Gynaecology Clinic of Medical University in Warsaw in the period from 1st October, 2001 until 30th September, 2002, found to have murmurs during the observation in The Neonatology Department. RESULTS: Heart murmurs were detected in 107 infants out of 1291 born in The Clinic in this period. Out of the study group, echocardiography results were normal in fourteen children, and in ninety-three patients the echocardiography revealed one of the following abnormalities: persistent foramen ovale (FO), persistent ductus arteriosus (PDA), ventricular septum defect (VSD), insufficiency of tricuspid valve (IT) and others. CONCLUSIONS: The most frequently observed abnormality in echocardiography in infants with heart murmur was persistent foramen ovale. The rate of heart abnormalities was the highest in infants with birth weight below 1500 G.