Hydrops fetalis caused by a complex congenital heart defect with concurrent hypoplasia of pulmonary blood vessels and lungs visualized by micro-CT in a French Bulldog.

BACKGROUND: Hydrops fetalis (HF) is fluid accumulation in fetus body cavities and subcutaneous tissue. The condition has been described in various farm and companion animal species, including dogs. Most of cases result from a heart defect. Exact nature of this defect is rarely clarified. CASE PRESENTATION: A newborn, male French bulldog puppy with severe HF underwent a full anatomopathological examination to diagnose the primary cause of HF. Based on the anatomopathological examination, fetal ultrasound, and micro-computed tomography, transposition of the great arteries with hypoplasia of the ascending aorta, aortic arch interruption, ostium secundum atrial septal defect, severe tricuspid valve dysplasia, as well as hypoplasia of pulmonary vessels and lungs were diagnosed. CONCLUSIONS: This is the first report of HF caused by severe, complex congenital heart defects with concurrent pulmonary vessel and lung hypoplasia.

Integrated Basic Heart and Lung Ultrasound Examination for the Differentiation between Bacterial Pneumonia and Lung Neoplasm in Dogs-A New Diagnostic Algorithm.

The diagnostics of two of the most prevalent lung diseases in dogs, bacterial pneumonia (BP) and lung neoplasm (LN), are challenging as their clinical signs are identical and may also occur in extrapulmonary diseases. This study aims to identify ultrasonographic criteria and develop a lung ultrasound (LUS)-based diagnostic algorithm which could help distinguish between these two conditions. The study is carried out in 66 dyspneic dogs in which a heart disease was excluded using echocardiography. Based on imaging and laboratory diagnostic tests, as well as follow-up, the dogs are classified into LN (35 dogs) and BP (31 dogs) groups. LUS is performed at admission and the presence of seven lung abnormalities (pleural thickening, B-lines, subpleural consolidations, hepatization with or without aeration, nodule sign and mass classified together as a tumor, and free pleural fluid) and classification and regression trees are used to develop an LUS-based diagnostic algorithm. Distribution of all LUS abnormalities except for aerations differs significantly between groups; however, their individual differentiating potential is rather low. Therefore, we combine them in an algorithm which allows for definitive classification of 60 dogs (91%) (32 with LN and 28 with BP) with correct diagnosis of LN and BP in 31 dogs and 27 dogs, respectively.

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.

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.

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.

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.

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.

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.