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.).

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.

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.

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.

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.

Mixed partial anomalous pulmonary venous drainage coexistent with an aortic valve abnormality - analysis of ultrasound diagnostics in a 10-year-old girl with Turner syndrome.

The authors present a case of echocardiographic diagnosis of a rare congenital cardiovascular anomaly in the form of mixed partial anomalous pulmonary veins connection in a 10-year-old girl with Turner syndrome and congenital mild stenosis of insufficient bicuspid aortic valve, made while diagnosing the causes of intestinal tract bleeding. The article presents various diagnostic difficulties leading to the delayed determination of a correct diagnosis, resulting from the absence of symptoms of circulatory failure in the early stage of the disease and the occurrence of severe and dominant auscultatory phenomena typical for congenital aortic valve defect which effectively masked the syndromes of increased pulmonary flow. The authors discuss the role of the impact of phenotypic characteristics of the Turner syndrome, in particular a short webbed neck restricting the suprasternal echocardiographic access and the presence of psychological factors associated with a long-term illness. The importance of indirect echocardiographic symptoms suggesting partial anomalous pulmonary veins connection in the presence of bicuspid aortic valve, e.g. enlargement of the right atrium and right ventricle, and paradoxical interventricular septum motion were emphasized in patients lacking ASD, pulmonary hypertension or tricupid and pulmonary valve abnormalities. The methodology of echocardiographic examination enabling direct visualization of the abnormal vascular structures was presented. Special attention was paid to the significance of highly sensitive echocardiographic projections: high right and left parasternal views in sagittal and transverse planes with patient lying on the side, with the use of two-dimensional imaging and color Doppler. Finally, the limitations of echocardiography resulting from the visualization and tracking of abnormal vascular structures hidden behind ultrasound non-conductive tissues were indicated, as was the role of other diagnostic modalities, such as angio-CT and/or nuclear magnetic resonance.

Ultrasound diagnosis of pulmonary sling with proximal stenosis of left pulmonary artery and patent arterial duct.

Authors discuss methods of echocardiographic diagnosis of the pulmonary sling with stenosis and hypoplasia of the left pulmonary artery and patent arterial duct with massive left-to-right shunt, based on a case of the newborn with resistant to treatment heart failure, with initial diagnosis of patent ductus arteriosus, referred to surgical treatment. The optimal echocardiographic views permitting establish diagnosis of the pulmonary sling were suggested. The special attention was paid to high parasternal and suprasternal views visualizing vessels of the upper mediastinum as well as characteristic differences between the normal and pathologic picture. The typical features of the echocardiogram suggesting pulmonary sling, like the lack of the left pulmonary artery in its expected position, and the abnormal branching pattern of the right pulmonary artery were indicated. The greatest diagnostic difficulties in visualization of the abnormal route of the left pulmonary artery were related to the presence of air-containing tissues, like lungs and central airways between the ultrasound probe and area of interest. The other was the masking influence of the large patent arterial duct, that may mimic the left pulmonary artery arising from the pulmonary trunk. The other entities requiring differentiation with sling, like aplasia of the left lung, the direct or indirect aortic origin of the left pulmonary artery, were discussed. The role of other visualization technics, like computed 3D tomography, and magnetic nuclear resonance, as well as direct visualization of central airways with bronchoscopy in establishing precise diagnosis were stressed.

Totally anomalous pulmonary venous drainage - supracardiac type: ultrasound assessment of anatomically determined stenosis of the vertical vein collecting pulmonary venous return.

The diagnosis of the congenital heart defects, among others totally anomalous pulmonary venous drainage, is based on echocardiography. While the visualization of intracardiac structures rarely causes significant difficulties, the vessels positioned outside the heart, e.g. the pulmonary veins, are often hidden behind tissues impermeable to ultrasounds, which may necessitate the use of other imaging methods, such as computer tomography, nuclear magnetic resonance or angiocardiography. The serious limitation of these techniques, especially in pediatric age, is the necessity to administer general anesthesia and contrast media. In order to obtain clear images, the appropriate concentration of a contrast agent in the vessels is necessary, which is not always possible in a patient with severe circulatory failure. Therefore, every effort should be made to obtain as much information necessary for treatment determination as possible from echocardiography, in spite of its limitations. A significant morphological factor of totally anomalous pulmonary venous drainage is the connection between the pulmonary and systemic veins, which in the supracardiac type is the vertical vein draining into the left brachiocephalic vein. The narrowing of this connection impedes the return of the blood from the lungs, which leads to the secondary edema and severe, abrupt cardiorespiratory insufficiency. Such a narrowing should be sought for in every case of totally anomalous pulmonary venous drainage since it constitutes an indication for an urgent surgery. On the basis of own experience and information obtained from the pertinent literature, the authors describe the rules and criteria of the diagnosis of this rare supracardiac form of the heart defect with the presence of the vertical vein which may undergo stenosis due to a phenomenon called the anatomical or bronchoarterial vise. It is formed when the vessel "pushes through" a narrow opening bordered by the left pulmonary artery from the inferior side as well as the left main bronchus and the arterial duct or ligament from the superior side. This article describes a technique of echocardiographic test enabling the precise visualization of the vessel's course and the differentiation from a more common variant of the defect - without external stenosis.