[Coexistence of pulmonary sequestration and congenital cystic adenomatoid malformation. Case report and review of literature]. / Wspólistnienie sekwestracji pluca i wrodzonej torbielowatosci gruczolakowatej pluc. Prezentacja przypadku klinicznego i przeglad pismiennictwa.

This paper presents a case report of an infant, with a prenatally diagnosed congenital lung malformation, which proved to be pulmonary sequestration and congenital cystic adenomatoid malformation. The authors discuss current knowledge on diagnosis, clinical course and suggestions of ante- and postnatal management of patients with pulmonary sequestration or/and congenital cystic lung malformation.

Catheter-Guided Dissolution of a Giant Thrombus in the Left Ventricle in a Newborn.

A newborn with pulmonary hypertension due to the premature fetal arterial duct occlusion was diagnosed with a giant left ventricle thrombus. Cardiopulmonary compromise required multidrug therapy with vasopressors infusions, high-frequency oscillation, and nitric oxide. Alteplase infusion through a guiding catheter into the left atrium dissoluted the clot without sequelae. (Level of Difficulty: Advanced.).

Coronary steal phenomenon following right ventricle decompression and revascularization of atretic left coronary ostium: case report.

BACKGROUND: Coronary steal phenomenon and myocardial ischemia is a complication following decompression of a hypertensive right ventricle in patients with left coronary-cameral fistulae. CASE PRESENTATION: We present a 12-year-old girl with a complex heart defect successfully operated on using a hybrid surgical-interventional approach to decompress the ventricle, embolize the fistula and reconstruct the atretic left coronary ostium. CONCLUSIONS: A novel hybrid strategy is the best solution for coronary-cameral fistulas reliant on high ventricular pressure at high risk for coronary steal phenomenon.

Critical appraisal of MAPSE and TAPSE usefulness in the postoperative assessment of ventricular contractile function after congenital heart defect surgery in infants.

Introduction: In this study, we assessed the adequacy of the two echocardiographic parameters representing the longitudinal systolic function of the ventricles, MAPSE & TAPSE, in perioperative monitoring. Aims: 1. The assessment of MAPSE and TAPSE during the early (4 weeks) postoperative period after surgical correction of congenital heart defects in infants, performed with CPB. 2. The verification of the hypothesis, that the significant decline of TAPSE observed in this period is not related to the global RV systolic dysfunction. For this reason, systolic function of the RV was additionally assessed with another echocardiographic parameter: RVFAC. Material and methods: Prospective study with 51 infants operated due to ASDII, VSD, AVSD and TOF. Four measurements of MAPSE,TAPSE and RVFAC were performed in the apical four-chamber projection. TAPSE and MAPSE were expressed as metric values and z-score; RV FAC values were expressed in percentages. Results: TAPSE uniformly declined in the first postoperative day down to 34.5% of the initial value (p <0.00001), then gradually increased: up to 42.9% and 52% respectively, remaining significantly lower than preoperatively (p <0.001). RV FAC: increased by 21% (p <0.01), then remained stable in consecutive examinations. MAPSE declined by 21% during the first postoperative day, but with promptly normalized completely. Conclusions: The movement of both AV valve annuli is subjected to different, not fully understood influences. The relatively slight decline of MAPSE makes this parameter suitable for the assessment of the postoperative LV systolic function. Deep, long-lasting decline of TAPSE, uniform in the whole group, does not permit to use this parameter and suggests the choice of another one, e.g. RVFAC.Introduction: In this study, we assessed the adequacy of the two echocardiographic parameters representing the longitudinal systolic function of the ventricles, MAPSE & TAPSE, in perioperative monitoring. Aims: 1. The assessment of MAPSE and TAPSE during the early (4 weeks) postoperative period after surgical correction of congenital heart defects in infants, performed with CPB. 2. The verification of the hypothesis, that the significant decline of TAPSE observed in this period is not related to the global RV systolic dysfunction. For this reason, systolic function of the RV was additionally assessed with another echocardiographic parameter: RVFAC. Material and methods: Prospective study with 51 infants operated due to ASDII, VSD, AVSD and TOF. Four measurements of MAPSE,TAPSE and RVFAC were performed in the apical four-chamber projection. TAPSE and MAPSE were expressed as metric values and z-score; RV FAC values were expressed in percentages. Results: TAPSE uniformly declined in the first postoperative day down to 34.5% of the initial value (p <0.00001), then gradually increased: up to 42.9% and 52% respectively, remaining significantly lower than preoperatively (p <0.001). RV FAC: increased by 21% (p <0.01), then remained stable in consecutive examinations. MAPSE declined by 21% during the first postoperative day, but with promptly normalized completely. Conclusions: The movement of both AV valve annuli is subjected to different, not fully understood influences. The relatively slight decline of MAPSE makes this parameter suitable for the assessment of the postoperative LV systolic function. Deep, long-lasting decline of TAPSE, uniform in the whole group, does not permit to use this parameter and suggests the choice of another one, e.g. RVFAC.

Anomalous retroaortic paravertebral course of the left innominate vein in a child with atrial septal defect.

We report the case of a 2.5-year-old boy with atrial septal defect in whom anomalous position of the left innominate vein was detected on preoperative ultrasound examination. Before joining the right brachiocephalic vein, the vessel extended from the left to the right and downward beyond the descending aorta. It was considerably flattened by the thoracic vertebral column, and was invisible on ultrasonography in this section. The appearance of the visible segments raised a suspicion of an anomalous course of persistent left superior vena cava draining into the left atrium, dilated azygos vein in a case of interrupted inferior vena cava, or partial anomalous pulmonary venous return. Since all doubts had to be resolved before open heart surgery, a decision was made to expand the diagnostic work-up to include computed tomography angiography. We present the echocardiographic and computed tomography findings of this unusual and previously unreported case of anomalous venous return.We report the case of a 2.5-year-old boy with atrial septal defect in whom anomalous position of the left innominate vein was detected on preoperative ultrasound examination. Before joining the right brachiocephalic vein, the vessel extended from the left to the right and downward beyond the descending aorta. It was considerably flattened by the thoracic vertebral column, and was invisible on ultrasonography in this section. The appearance of the visible segments raised a suspicion of an anomalous course of persistent left superior vena cava draining into the left atrium, dilated azygos vein in a case of interrupted inferior vena cava, or partial anomalous pulmonary venous return. Since all doubts had to be resolved before open heart surgery, a decision was made to expand the diagnostic work-up to include computed tomography angiography. We present the echocardiographic and computed tomography findings of this unusual and previously unreported case of anomalous venous return.

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.

Isolation of the left subclavian artery in an infant with tetralogy of Fallot, right aortic arch and DiGeorge syndrome. Echocardiographic diagnostic case study.

We present a case of a 6-month-old infant with an isolated left subclavian artery coexistent with right-sided aortic arch, tetralogy of Fallot and DiGeorge syndrome, with an emphasis on echocardiographic detection of this extremely rare anomaly. Specific difficulties related to echocardiographic visualization of abnormally coursing artery were a result of significantly limited ultrasonographic access due to the absence of thymus and a very close proximity of the left subclavian artery and left common carotid artery, mimicking a normal brachiocephalic trunk, which is usually present in patients with right-sided aortic arch. Precise analysis of the course of carotid and vertebral arteries as well as the nature and direction of flow in these vessels (particularly in the left vertebral and subclavian artery) suggested ductal rather than aortic origin of the left subclavian artery. Precise delineation of anatomical relationships between major arteries prior to surgical closure of the arterial duct was necessary to prevent potential postoperative ischemia of the left upper extremity; therefore the diagnosis was completed with CT angiography.We present a case of a 6-month-old infant with an isolated left subclavian artery coexistent with right-sided aortic arch, tetralogy of Fallot and DiGeorge syndrome, with an emphasis on echocardiographic detection of this extremely rare anomaly. Specific difficulties related to echocardiographic visualization of abnormally coursing artery were a result of significantly limited ultrasonographic access due to the absence of thymus and a very close proximity of the left subclavian artery and left common carotid artery, mimicking a normal brachiocephalic trunk, which is usually present in patients with right-sided aortic arch. Precise analysis of the course of carotid and vertebral arteries as well as the nature and direction of flow in these vessels (particularly in the left vertebral and subclavian artery) suggested ductal rather than aortic origin of the left subclavian artery. Precise delineation of anatomical relationships between major arteries prior to surgical closure of the arterial duct was necessary to prevent potential postoperative ischemia of the left upper extremity; therefore the diagnosis was completed with CT angiography.

Late diagnosis of total anomalous pulmonary venous drainage in a 5.5-month-old infant.

A 5.5-month-old girl was admitted with non-specific signs and symptoms like dyspnea at rest, tachypnea, fatigue, low body weight and cyanosis on exertion. Physical examination revealed a barrel-shaped chest; no pathological sounds over the heart or both lung areas were detected. The above mentioned symptoms might suggest a circulatory problem. Echocardiography and computed tomography angiography were performed. These examinations revealed supracardiac type total anomalous pulmonary venous drainage. Echocardiographic signs of pulmonary hypertension and severe right ventricle overload were detected. Detailed analysis of both imaging examinations revealed atypical obstruction of the pulmonary venous return: narrowing of the proximal part of superior vena cava. An urgent surgery was performed, with no complications in the postoperative period. A follow-up echocardiography showed normalization of cardiac function and pulmonary pressure as well as normalization of flow profile within the superior vena cava. The paper presents a non-invasive diagnostic process in the described case, and discusses the causes of late diagnosis.A 5.5-month-old girl was admitted with non-specific signs and symptoms like dyspnea at rest, tachypnea, fatigue, low body weight and cyanosis on exertion. Physical examination revealed a barrel-shaped chest; no pathological sounds over the heart or both lung areas were detected. The above mentioned symptoms might suggest a circulatory problem. Echocardiography and computed tomography angiography were performed. These examinations revealed supracardiac type total anomalous pulmonary venous drainage. Echocardiographic signs of pulmonary hypertension and severe right ventricle overload were detected. Detailed analysis of both imaging examinations revealed atypical obstruction of the pulmonary venous return: narrowing of the proximal part of superior vena cava. An urgent surgery was performed, with no complications in the postoperative period. A follow-up echocardiography showed normalization of cardiac function and pulmonary pressure as well as normalization of flow profile within the superior vena cava. The paper presents a non-invasive diagnostic process in the described case, and discusses the causes of late diagnosis.

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.

Supravalvar mitral ring with a parachute mitral valve and subcoarctation of the aorta in a child with hemodynamically significant VSD. A study of the morphology, echocardiographic diagnostics and surgical therapy.

The authors present a case of echocardiographic diagnosis of supravalvar mitral ring (a fibromembranous structure that arose from the atrial surface of the mitral leaflets) in a child with a parachute mitral valve, a ventricular septal defect, and mild narrowing of the aortic isthmus. The supravalvar mitral stenosis is a typical but very infrequently detected element of the complex of anatomical abnormalities located within the left heart and the proximal aorta, called the Shone's complex (syndrome). Diagnosing an additional, hemodynamically significant anatomic defect during echocardiography was possible thanks to the detection of marked mobility limitation of the ring-adjacent part of the mitral valve mural leaflet as well as of an atypical image of turbulence occurring during the inflow from the left atrium to the left ventricle. The early diagnosis made it possible to perform complete correction of this complex congenital defect within a single operation.

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.

Physiological basis in the assessment of myocardial mechanics using speckle-tracking echocardiography 2D. Part I.

In this paper, the authors attempt to concisely present the anatomical and pathophysiological bases as well as the principles for echocardiographic evaluation of mechanical aspects of cardiac function based on speckle tracking method. This technique uses a phenomenon involving the formation of characteristic image units, referred to as speckles or acoustic markers, which are stable during cardiac cycle, on a two-dimensional echocardiographic picture. Changes in the position of these speckles throughout the cardiac cycle, which are monitored and analyzed semi-automatically by a computer system, reflect deformation of both, cardiac ventricle as a whole as well as its individual anatomical segments. The values of strain and the strain rate, as well as the range and velocity of the movement of these markers, which are in close relationship with multiple hemodynamic parameters, can be visualized as various types of charts - linear, two- and three-dimensional - as well as numerical values, enabling deeper insight into the mechanical and hemodynamic aspects of cardiac function in health and disease. The use of information obtained based on speckle tracking echocardiography allows to understand previously unclear mechanisms of physiological and pathophysiological processes. The first part of the study discusses the formation of a two-dimensional ultrasound image and the speckles, as well as the technical aspects of tracking their movement. The second part presents in more detail the methodology of speckle-tracking echocardiography, the characteristic abnormalities of cardiac mechanics presenting in different clinical entities, and the limitations related to given clinical and technical issues.

Physiological basis in the assessment of myocardial mechanics using speckle-tracking echocardiography 2D. Part II.

In this paper, the authors attempt to concisely present the anatomical and pathophysiological bases as well as the principles for echocardiographic evaluation of mechanical aspects of cardiac function based on speckle tracking method. This technique uses a phenomenon involving the formation of characteristic image units, referred to as speckles or acoustic markers, which are stable during cardiac cycle, on a two-dimensional echocardiographic picture. Changes in the position of these speckles throughout the cardiac cycle, which are monitored and analyzed semi-automatically by a computer system, reflect deformation of both, cardiac ventricle as a whole as well as its individual anatomical segments. The values of strain and the strain rate, as well as the range and velocity of the movement of these markers, which are in close relationship with multiple hemodynamic parameters, can be visualized as various types of charts - linear, two- and three-dimensional - as well as numerical values, enabling deeper insight into the mechanical and hemodynamic aspects of cardiac function in health and disease. The use of information obtained based on speckle tracking echocardiography allows to understand previously unclear mechanisms of physiological and pathophysiological processes. The first part of the study discusses the formation of a two-dimensional ultrasound image and the speckles, as well as the technical aspects of tracking their movement. The second part presents in more detail the methodology of speckle-tracking echocardiography, the characteristic abnormalities of cardiac mechanics presenting in different clinical entities, and the limitations related to given clinical and technical issues.

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.