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.

Morphometry of the heart orifices and morphometry and topography of the coronary ostia in the goat.

BACKGROUND: The coronary vessels have been described in various species of domestic and wild ruminants. However, no studies on the detailed morphology and morphometry of heart orifices and coronary ostia in the Polish goat are available. MATERIALS AND METHODS: The study was carried out on 112 female, adult dairy goats belonging to Polish Fawn Improved and Polish White Improved breed, closely related to French Alpine and Saanen, respectively. RESULTS: In all examined individuals, all heart orifices and heart valves were of normal structure. There was no significant difference between diameter of the aortic and pulmonary orifice. The right atrioventricular opening was significantly wider than the left atrioventricular opening. The dimension of the left coronary ostium ranged from 1.0 to 5.5 mm with the arithmetic mean (± standard deviation [SD]) of 4.3 ± 0.8 mm. The dimension of the right coronary ostium ranged from 0.5 to 5.0 mm with the arithmetic mean (± SD) of 2.8 ± 0.7 mm. Both coronary artery ostia were located under the sinotubular junction. Out of 112 examined goats, 39 (34.8%) had variations in the structure of the coronary ostia such as the lack of main trunk or the presence of additional coronary ostia. They were observed in one (in 34/39 goats) or both coronary arteries (5/39 goats). CONCLUSIONS: In goats, the dimensions of aortic and pulmonary orifices are similar while the right atrioventricular opening outsizes the left one. On the other hand, the left coronary ostium is wider than the right one. Morphological variations in the coronary ostia occur in approximately one third of goats, more often in the right than in the left ostium.

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.

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.).

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.

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.

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.