Left ventricular thrombus in the multisystem inflammatory syndrome in children associated with COVID-19.

A 3-year-old girl presenting with fever, mucocutaneous inflammation, and acute gastrointestinal symptoms met criteria for the multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C). Echocardiography showed severely decreased left ventricular (LV) function with an apical mass. After treatment with intravenous (IV) immunoglobulin, IV steroids, anakinra, milrinone, and systemic anticoagulation, her LV function rapidly improved and the mass became increasingly mobile. Given the risk of systemic embolization, the mass was excised through left ventriculotomy and pathology confirmed a thrombus.

Comparison of echocardiographic measurements to invasive measurements of diastolic function in infants with single ventricle physiology: a report from the Pediatric Heart Network Infant Single Ventricle Trial.

BACKGROUND: While echocardiographic parameters are used to quantify ventricular function in infants with single ventricle physiology, there are few data comparing these to invasive measurements. This study correlates echocardiographic measures of diastolic function with ventricular end-diastolic pressure in infants with single ventricle physiology prior to superior cavopulmonary anastomosis. METHODS: Data from 173 patients enrolled in the Pediatric Heart Network Infant Single Ventricle enalapril trial were analysed. Those with mixed ventricular types (n = 17) and one outlier (end-diastolic pressure = 32 mmHg) were excluded from the analysis, leaving a total sample size of 155 patients. Echocardiographic measurements were correlated to end-diastolic pressure using Spearman's test. RESULTS: Median age at echocardiogram was 4.6 (range 2.5-7.4) months. Median ventricular end-diastolic pressure was 7 (range 3-19) mmHg. Median time difference between the echocardiogram and catheterisation was 0 days (range -35 to 59 days). Examining the entire cohort of 155 patients, no echocardiographic diastolic function variable correlated with ventricular end-diastolic pressure. When the analysis was limited to the 86 patients who had similar sedation for both studies, the systolic:diastolic duration ratio had a significant but weak negative correlation with end-diastolic pressure (r = -0.3, p = 0.004). The remaining echocardiographic variables did not correlate with ventricular end-diastolic pressure. CONCLUSION: In this cohort of infants with single ventricle physiology prior to superior cavopulmonary anastomosis, most conventional echocardiographic measures of diastolic function did not correlate with ventricular end-diastolic pressure at cardiac catheterisation. These limitations should be factored into the interpretation of quantitative echo data in this patient population.

Abnormal location of umbilical venous catheter due to Scimitar syndrome.

Scimitar syndrome is a rare congenital anomaly where the right pulmonary veins return to the inferior vena cava (IVC) just below the diaphragm. On chest X-ray (CXR), an IVC catheter will be in a bizarre location outside the heart if it inadvertently passes into the scimitar vein rather than into the right atrium.

Echocardiographic methods, quality review, and measurement accuracy in a randomized multicenter clinical trial of Marfan syndrome.

BACKGROUND: The Pediatric Heart Network is conducting a large international randomized trial to compare aortic root growth and other cardiovascular outcomes in 608 subjects with Marfan syndrome randomized to receive atenolol or losartan for 3 years. The authors report here the echocardiographic methods and baseline echocardiographic characteristics of the randomized subjects, describe the interobserver agreement of aortic measurements, and identify factors influencing agreement. METHODS: Individuals aged 6 months to 25 years who met the original Ghent criteria and had body surface area-adjusted maximum aortic root diameter (ROOTmax) Z scores > 3 were eligible for inclusion. The primary outcome measure for the trial is the change over time in ROOTmaxZ score. A detailed echocardiographic protocol was established and implemented across 22 centers, with an extensive training and quality review process. RESULTS: Interobserver agreement for the aortic measurements was excellent, with intraclass correlation coefficients ranging from 0.921 to 0.989. Lower interobserver percentage error in ROOTmax measurements was independently associated (model R(2) = 0.15) with better image quality (P = .002) and later study reading date (P < .001). Echocardiographic characteristics of the randomized subjects did not differ by treatment arm. Subjects with ROOTmaxZ scores ≥ 4.5 (36%) were more likely to have mitral valve prolapse and dilation of the main pulmonary artery and left ventricle, but there were no differences in aortic regurgitation, aortic stiffness indices, mitral regurgitation, or left ventricular function compared with subjects with ROOTmaxZ scores < 4.5. CONCLUSIONS: The echocardiographic methodology, training, and quality review process resulted in a robust evaluation of aortic root dimensions, with excellent reproducibility.

Shape of the dilated aorta in children with bicuspid aortic valve.

BACKGROUND: The dilated aorta in adults with bicuspid aortic valve has been shown to have different shapes, but it is not known if this occurs in children. This observational study was performed to determine if there are different shapes of the dilated aorta in children with bicuspid aortic valve and their association with age, gender, hemodynamic alterations, and degree of aortic enlargement. METHODS: One hundred and eighty-seven echocardiograms done on pediatric patients (0 - 18 years) for bicuspid aortic valve, during 2008, were reviewed. Aortic valve morphology, shape/size of the aorta, and pertinent hemodynamic alterations were documented. Aortic dilation was felt to be present when at least one aortic segment had a z-score > 2.0; global aortic enlargement was determined by summing the aortic segment z-scores. The aortic shape was assessed by age, gender, valve morphology, and hemodynamic alterations. RESULTS: Aortic dilation was present in 104/187 patients. The aorta had six different shapes designated from S1 through S6. There was no association between the aortic shape and gender, aortic valve morphology, or hemodynamic abnormalities. S3 was the most common after the age of six years and was associated with the most significant degree of global aortic enlargement. CONCLUSIONS: The shape of the dilated aorta in children with bicuspid aortic valve does not occur in a uniform manner and multiple shapes are seen. S2 and S3 are most commonly seen. As aortic dilation becomes more significant, a single shape (S3) becomes the dominant pattern.

Three-dimensional echocardiographic evaluation of the Fontan conduit for thrombus.

INTRODUCTION: Total caval pulmonary connection (Fontan circuit) is the final common pathway for the palliation of single ventricle congenital heart disease. Flow within the Fontan circuit results in an environment that is prone to thrombus formation putting the patient at risk for pulmonary and/or systemic embolus. To prevent these problems, patients are placed on antithrombotic therapy and periodically evaluated for thrombus within the conduit. Two-dimensional (2D) echocardiography, although commonly used to evaluate these patients, has been shown to have limited ability in accurately identifying thrombi within the Fontan conduit. The diagnosis of intracardiac thrombi has been augmented by three-dimensional (3D) echocardiography and the patients in this series were evaluated with 3D imaging (3D transthoracic echocardiography and/or 3D transesophageal echocardiography) to determine if thrombi could be detected or excluded within the Fontan conduit. To the author's knowledge, this constitutes the first case series describing the use of 3D echocardiography to evaluate the Fontan conduit for thrombus. METHODS: The four patients described in this case series underwent 3D echocardiography to evaluate the Fontan conduit for a suspected or potential thrombus. RESULTS: The Fontan conduit was imaged and thrombi were felt to be documented or excluded in all four patients with 3D echocardiography. CONCLUSIONS: Three-dimensional echocardiography may enhance the ability to detect or exclude thrombi within the Fontan conduit. Further studies are needed to determine if this will prove to be an effective and reliable technique in evaluating the Fontan conduit for thrombus.

Postoperative dissecting ventricular septal hematoma: recognition and treatment.

Dissecting ventricular septal hematoma (DVSH) rarely occurs after repair of a ventricular septal defect (VSD) but can lead to serious complications such as septal rupture, myocardial rupture, cardiogenic shock, heart block, outflow obstruction, cardiac tamponade, abscess transformation, and death. This paper describes the diagnosis and management of acute, severe, left ventricular outflow tract obstruction caused by the development of a DVSH after VSD repair.

Optimal head position during transesophageal echocardiographic probe insertion for pediatric patients weighing up to 10 kg.

No data exists for the optimal head position during transesophageal echocardiography (TEE) probe insertion in children. Turning the head to the side closes the ipsilateral pyriform sinus and dilates the contralateral pyriform sinus, theoretically making probe insertion safer and easier. In this study, 94 children (weight, 5 kg), II (weight, 4-5 kg), and III (weight, <4 kg). The difference was statistically significant only for subgroup III (p = 0.0001). Insertion failed in four children with the head in the midline position but was successful when the head was turned leftward. Children undergoing TEE who weigh less than 10 kg should have the head positioned to the side rather than in the midline position during probe insertion. If insertion is unsuccessful or difficult with the head in the midline position, the authors recommend turning the head to the side and reattempting probe insertion.

Intermediate-term results of repair for aortic, neoaortic, and truncal valve insufficiency in children.

OBJECTIVE: Repair of aortic valve insufficiency is difficult, and durability is relatively unknown in children. This study evaluates the intermediate-term results of repair of the systemic semilunar valve, including the native aortic valve, neoaortic valve (anatomic pulmonary), and truncal valve. METHODS: We reviewed the records of 54 children (aged 2 days to 18 years) who underwent repair of the functional aortic valve for moderate or greater insufficiency from 1991 to 2005. Valve anatomy was tricuspid aortic in 26 patients, bicuspid aortic in 11 patients, tricuspid neoaortic in 9 patients, bicuspid neoaortic in 1 patient, and truncal valve in 7 patients. Multiple surgical techniques were used in most of the 54 patients, including leaflet plication in 17, leaflet repair in 15, commissuroplasty in 32, pericardial cusp augmentation in 8, and sinus of Valsalva reduction in 3. RESULTS: There was 1 early death and no late deaths. Actuarial freedom from reoperation was 68% at 5 years and 58% at 10 years. Freedom from aortic valve replacement was 82% at 5 years and 73% at 10 years. Duration of cardiopulmonary bypass was the most significant risk factor for reoperation with multivariate analysis. Of the 40 patients who have not undergone reoperation, 37 have had follow-up echocardiograms with the latest study (4.5 +/- 4.2 years) demonstrating trace to 1+ insufficiency in 23 patients, 1 to 2+ in 12 patients, 2 to 3+ in 1 patient, and 3 to 4+ in 1 patient. CONCLUSION: Repair of the insufficient systemic semilunar valve offers acceptable 10-year freedom from reoperation and functional results, and should be considered for most children.

Safety and efficacy of sedation with propofol for transoesophageal echocardiography in children in an outpatient setting.

BACKGROUND: Transoesophageal echocardiography has become a powerful tool in the diagnosis and management of children with congenital cardiac malformations. Unlike adults, children will not tolerate transoesophageal echocardiography under light sedation. This study was undertaken, therefore, to evaluate the safety and efficacy of deep sedation with propofol for transoesophageal echocardiography in children examined in an outpatient setting. METHODS: This is a retrospective study of patients undergoing transoesophageal echocardiography with propofol given in bolus aliquots to achieve a level of sedation adequate to insert the transoesophageal echocardiographic probe and maintain sedation throughout the procedure. RESULTS: We included a total of 118 patients, 57% being male, with a mean age of 12.9 years. Adequate sedation was achieved using a mean propofol dose of 8.3 milligrams per kilogram, with the dose per kilogram decreasing concomitant with increasing weight of the patient. Patients less than two years of age were intubated for the procedure. There were no clinically significant changes in cardiac function or haemodynamics. Non-intubated patients received supplemental oxygen prior to, or just after, the onset of sedation, with transient hypoxaemia observed in one-fifth. Complications were rare, with minor problems occurring in 7.6%, and major ones in 4%. CONCLUSIONS: Transoesophageal echocardiography can be performed on an outpatient basis in children with a wide spectrum of congenital cardiac malformations, and propofol is an ideal sedative agent in this setting. Although not common, preparations must be made for significant haemodynamic and respiratory complications. In our study, we intubated all the children under 2 years of age.

Extracorporeal membrane oxygenation as a bridge to surgical treatment of flail tricuspid valve in a neonate.

A term infant rapidly developed profound cyanosis and metabolic acidosis shortly after an uncomplicated vaginal delivery. Echocardiography identified a flail antero-superior leaflet of the tricuspid valve, which was producing severe tricuspid insufficiency. The clinical state deteriorated despite maximal medical management, and the patient was placed on venoarterial extracorporeal membrane oxygenation. Within twenty-four hours, the metabolic acidosis corrected, inotropic support was discontinued, and the patient was weaned to minimal ventilator settings. Successful repair of the tricuspid valve was performed two days later.