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There are three cusps in a normally developed aortic valve. Abnormal excavation or fusion, during the embryological development of the aortic valve, results in a varying number of cusps. Bicuspid aortic valve is the most common, but more rarely, unicuspid and quadricuspid aortic valves can be seen.
Here, a case of a 16-year-old male with a unicommissural unicuspid aortic valve and a case of a 13-year-old female with a quadricuspid aortic valve were reported.
We presented a rare case of traumatic mitral annular avulsion and interventricular septum dissection after an unintentional falling injury in a 5-year-old female child. A successful surgical repair of mitral annulus and interventricular septum was performed to restore the haemodynamic stability.
Tetralogy of Fallot is a congenital heart defect diagnosed in infancy. Assessment of right ventricular size and function is important for evaluation of patients with tetralogy of Fallot, but these quantitative measures are challenging by echocardiography. This study evaluates a semi-automated software (EchoInsight®, Epsilon Imaging) by comparing its measures to manual measures in children with tetralogy of Fallot.
Echocardiographic measurements were performed using manual techniques and semi-automated software. Right ventricular measurements included end-diastolic and end-systolic area, fractional area change, chamber dimensions, and tricuspid annular plane systolic excursion. Reliability, correlation, and agreement between manual and semi-automated measures were assessed.
Echocardiograms for 46 patients were analysed. Intra- and inter-observer reliabilities for semi-automated measures were good with intraclass correlation coefficients all over 0.95 and 0.85, respectively. There was high correlation between manual and semi-automated methods for areas and dimensions (r = 0.91–0.98). Tricuspid annular plane systolic excursion measures and fractional area change also correlated, albeit less strongly. The semi-automated measurements of end-systolic and end-diastolic area were a 20 and 47% higher than manual methods, respectively.
The semi-automated method yielded a relative 52% lower fractional area change compared to the manual method.
The semi-automated software generates quantitative right ventricular measures in children with tetralogy of Fallot with good reliability and good correlation with manual methods for all measures, but with significant difference between manual and semi-automated techniques for area and functional measures. The specific right ventricular geometry in tetralogy of Fallot children may be why, compared to normal anatomy, greater differences were observed between the two techniques.
A young adult with late diagnosis of scimitar syndrome underwent infradiaphragmatic baffling of the scimitar vein to left atrium through an intra-atrial tunnel using PhotoFix® bovine pericardium with recurrent extensive fibrovascular granulation of the patch causing pulmonary and systemic venous obstruction leading to eventual explantation of the bovine pericardium.
Previous echocardiographic studies failed to show residual alterations of heart function in paediatric patients that have received treatment for Chagas disease. While the echocardiogram is the fundamental front-line tool for evaluating heart function, the appearance of new techniques allows a more detailed analysis. We aimed to evaluate systolic and diastolic function with new techniques in a paediatric population with Chagas disease several years after treatment completion.
Material and methods:
Echocardiograms were obtained from 84 Chagas disease patients (48 female) and 27 healthy controls. All patients had received treatment concluding on average 10 years prior to the study. The prospective analysis considered cardiac dimensions and cardiac function using two-dimensional, M-mode, Doppler and tissue Doppler imaging with emphasis on measuring longitudinal strain in the left ventricle by speckle tracking. Ejection fraction was measured with three-dimensional echocardiography.
Patients had an age of 14.2 ± 5.7 years (6–33) at the time of evaluation. Global and segmental motility of the left ventricle was normal in all patients. Ejection fraction was 59.2 ± 6.5 and 57.4 ± 6.5% (p = 0.31) in patients and controls respectively. Left ventricular global longitudinal systolic strain was −19 ± 2.4% in patients and −19 ± 3.6% (p = 0.91) in controls. No significant differences were found in remaining systolic and diastolic function measurements.
Paediatric patients that have received treatment for Chagas disease, evaluated with either conventional techniques or new tools, do not show significant long-term alterations of ventricular function.
Echo assessment of right ventricular function is difficult due to its complex geometry and regional differences in wall motion. In patients with repaired tetralogy of Fallot, cardiac MRI is the gold standard for assessment of right ventricular function. There is scant data regarding the correlation of echo measures of right ventricular function with cardiac MRI right ventricular function in the paediatric population.
Echocardiographic measures of regional and global right ventricular function were reviewed in 56 patients with repaired tetralogy of Fallot and 27 patients with structurally normal hearts. These measures were compared to global right ventricular ejection fraction by cardiac MRI.
Despite having the same right ventricular ejection fraction by cardiac MRI as normal controls, patients with repaired tetralogy of Fallot had significantly worse global and regional echocardiographic measures of right ventricular function. Right ventricular fractional area change and fractional shortening of the right ventricular outflow tract correlated best with global right ventricular function by MRI. Incorporation of these measures into a regression model allowed accurate prediction of global right ventricular function by cardiac MRI.
Children with repaired tetralogy of Fallot have abnormal regional and global right ventricular function compared to patients with normal hearts. Despite the presence of patch material, the right ventricular outflow tract significantly contributes to global right ventricular function by cardiac MRI. When incorporated into a model that includes regional and global echocardiographic measures of right ventricular function, right ventricular outflow tract fractional shortening helps predict global right ventricular function by MRI.
Haematopoietic stem cell transplantation is an important and effective treatment strategy for many malignancies, marrow failure syndromes, and immunodeficiencies in children, adolescents, and young adults. Despite advances in supportive care, patients undergoing transplant are at increased risk to develop cardiovascular co-morbidities.
This study was performed as a feasibility study of a rapid cardiac MRI protocol to substitute for echocardiography in the assessment of left ventricular size and function, pericardial effusion, and right ventricular hypertension.
A total of 13 patients were enrolled for the study (age 17.5 ± 7.7 years, 77% male, 77% white). Mean study time was 13.2 ± 5.6 minutes for MRI and 18.8 ± 5.7 minutes for echocardiogram (p = 0.064). Correlation between left ventricular ejection fraction by MRI and echocardiogram was good (ICC 0.76; 95% CI 0.47, 0.92). None of the patients had documented right ventricular hypertension. Patients were given a survey regarding their experiences, with the majority both perceiving that the echocardiogram took longer (7/13) and indicating they would prefer the MRI if given a choice (10/13).
A rapid cardiac MRI protocol was shown feasible to substitute for echocardiogram in the assessment of key factors prior to or in follow-up after haematopoietic stem cell transplantation.
We report a case of a newborn infant with coarctation of the aorta and hypoplastic transverse aortic arch who was found to have a circumaortic double left innominate vein on echocardiography. This exceedingly rare finding was important for surgical planning and was confirmed during congenital heart surgery.
We present the rare case of lipomatous atrial septal hypertrophy associated with adrenocorticotropin hormone therapy in an infant with West syndrome, highlighting their relatively benign nature and good prognosis in children, and the relevance of the differential diagnosis with more dangerous cardiac masses in order to avoid aggressive diagnostic and therapeutic interventions.
Factors that facilitate transfer of training in paediatric echocardiography remain poorly understood. This study assessed whether high-variation training facilitated successful transfer in paediatric echocardiography.
A mixed-methods study of transfer of technical and interpretive skill application amongst postgraduate trainees. Trainees were randomised to a low or high-variation training group. After a period of 8 weeks intensive echocardiography training, we video-recorded how trainees completed an echocardiogram in a complex cardiac lesion not previously encountered. Blinded quantitative analysis and scoring of trainee performance (echocardiogram performance, report, and technical proficiency) were performed using a validated assessment tool by a blinded cardiologist and senior cardiac physiologist. Qualitative interviews of the trainees were recorded to ascertain trainee experiences during the training and transfer process.
Sixteen trainees were enrolled in the study. For the cumulative score for all three components tested (echocardiogram performance, report, and technical proficiency), χ2 = 8.223, p = .016, which showed the high-variation group outperformed the low-variation group. Two common themes which assisted in the transfer emerged from interviews are as follows: (1) use of strategies described in variation theory to describe abnormal hearts, (2) the use of formative live feedback from trainers during hands-on training.
Training strategies exposing trainees to high-variation training may aid transfer of paediatric echocardiography skills.
With the increased training loads at very early ages in European elite youth soccer, there is an interest to analyse coronary artery remodelling due to high-intensity exercise.
Design and methods:
Prospective echocardiographic study in 259 adolescent elite male soccer players and 48 matched controls.
The mean age was 12.7 ± 0.63 years in soccer players and 12.6 ± 0.7 years in controls (p > 0.05). Soccer players had significant greater indexed left ventricular mass (93 ± 13 g/m2 versus 79 ± 12 g/m2, p = 0.001). Both coronary arteries origin could be identified in every participant. In soccer players, the mean diameter of the left main coronary artery was 3.67 mm (SD ± 0.59) and 2.61 mm (SD ± 0.48) for right main coronary artery. Controls showed smaller mean luminal diameter (left main coronary artery, p = 0.01; right main coronary artery, p = 0.025). In soccer players, a total of 91% (n = 196) and in controls a total of 94% (n = 45) showed left main coronary artery z scores within the normal range: −2.0 to 2.0. In right main coronary artery, a pattern of z score values distribution was comparable (soccer players 94%, n = 202 vs. controls 84%, n = 40). A subgroup of soccer players had supernormal z score values (>2.0 to 2.5) for left main coronary artery (9%, n = 19, p = 0.01) and right main coronary artery (6%, n = 10, p = 0.025), respectively.
Elite soccer training in early adolescence may be a stimulus strong enough to develop increased coronary arteries diameters. In soccer players, a coronary artery z score >2.0–2.5 might reflect a physiologic response induced by multiannual high-intensity training.
Identify diagnostic yield and frequency of echocardiograms for palpitation-related indications at outpatient paediatric cardiology clinics in relation to the 2014 ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE appropriate use criteria for Initial Transthoracic Echocardiography in Outpatient Paediatric Cardiology.
A single-centre, retrospective study of children presenting for evaluation of a chief complaint of palpitations to outpatient paediatric cardiology clinics from 2015 to 2017. Palpitations were defined as an unpleasant sensation of rapid, irregular, and/or forceful beating of the heart. Indications for echocardiogram in patients were retrospectively classified based on the appropriate use criteria as “appropriate,” “may be appropriate,” or “rarely appropriate.” The incidence of abnormal and incidental echocardiographic findings for each category was determined.
A total of 286 patients presented with palpitations, with 128 (52% female) meeting inclusion criteria. Exclusion criteria included patients with additional cardiac complaints, prior echocardiogram, or history of congenital heart disease. Echocardiograms were performed on 36 (28%) patients. The appropriate use criteria were retrospectively applied, and indications for their performance were classified as “appropriate” (n = 4), “may be appropriate” (n = 17), or “rarely appropriate” (n = 15). Minor echocardiographic abnormalities were present in 22% (n = 8) of echocardiograms obtained for all appropriate use criteria classifications. No moderate or severe echocardiographic abnormalities were found. Incidental findings were noted in eight echocardiograms.
Echocardiography in the evaluation of “rarely appropriate” and “may be appropriate” palpitation-related indications is of low diagnostic yield.
Acute respiratory infection is one of the main causes of morbidity in children. Some studies have suggested that pulmonary hypertension and congenital heart disease with haemodynamic repercussion increase the severity of respiratory infections, but there are few publications in developing countries.
This was a prospective cohort study evaluating the impact of pulmonary hypertension and congenital heart disease (CHD) with haemodynamic repercussion as predictors of severity in children under 5 years of age hospitalised for acute respiratory infection.
Altogether, 217 children hospitalised for a respiratory infection who underwent an echocardiogram were evaluated; 62 children were diagnosed with CHD with haemodynamic repercussion or pulmonary hypertension. Independent predictors of admission to intensive care included: pulmonary hypertension (RR 2.14; 95% CI 1.06–4.35, p = 0.034), respiratory syncytial virus (RR 2.52; 95% CI 1.29–4.92, p = 0.006), and bacterial pneumonia (RR 3.09; 95% CI 1.65–5.81, p = 0.000). A significant difference was found in average length of hospital stay in children with the cardiovascular conditions studied (p = 0.000).
Pulmonary hypertension and CHD with haemodynamic repercussion as well as respiratory syncytial virus and bacterial pneumonia were predictors of severity in children with respiratory infections in this study. Early recognition of cardiovascular risks in paediatric populations is necessary to lessen the impact on respiratory infections.
Brucellosis is an important systemic infectious disease, especially in developing countries. Every organ and system of the human body can be affected; however, cardiovascular complications of brucellosis are rare.
To assess cardiac functions in patients with acute brucellosis without overt cardiac involvement and to answer the following question: Is there any cardiac dysfunction despite the absence of endocarditis in these patients?
This cross-sectional study included 67 children with brucellosis and 40 healthy children. We performed a detailed echocardiographic examination in individuals at the beginning of the treatment. Patients with infective endocarditis were excluded from the study.
Echocardiography revealed no difference of ejection fraction, mitral and tricuspid annular plane systolic excursion, pulsed-wave Doppler-derived early diastolic peak velocity (E)/late diastolic peak velocity (A) ratios in mitral and tricuspid valves between the two groups. The deceleration time of early mitral inflow was longer in patients with brucellosis. Early diastolic peak velocity of the mitral and tricuspid annuluses obtained by tissue Doppler imaging (Ea) was significantly lower in children with brucellosis. The peak velocity obtained by tissue Doppler imaging during late diastole (Aa), Ea and Ea/Aa ratios in the interventricular septum, left ventricle posterior wall and right ventricle free wall was lower in patients with brucellosis than in the control group. The E/Ea ratio, isovolumic relaxation time, right ventricle and left ventricle myocardial performance indices were higher in patients with brucellosis.
Patients with acute brucellosis may have diastolic dysfunction without overt cardiac involvement and infective endocarditis.
There are several published echo-derived scores to help predict successful biventricular versus univentricular palliation in neonates with critical aortic stenosis. This study aims to determine whether any published scoring system accurately predicted outcomes in these neonates.
Single centre, retrospective cohort study including neonates who underwent aortic valve intervention (surgical valvotomy or balloon valvuloplasty) with the intention of biventricular circulation. Primary outcome was survival with biventricular circulation at hospital discharge. Data from their initial neonatal echocardiogram were used to compute the following scores – Rhodes, CHSS 1, Discriminant, CHSS 2, and 2 V.
Between 01/1999 and 12/2017, 68 neonates underwent aortic valve intervention at a median age of 4 days (range 1–29 days); 35 surgical valvotomy and 33 balloon valvuloplasty. Survival with biventricular circulation was maintained in 60/68 patients at hospital discharge. Of the remaining eight patients, three were converted to univentricular palliation, four died, and one underwent heart transplant prior to discharge. None of the binary score predictions of biventricular versus univentricular (using that score’s proposed cut-offs) were significantly associated with the observed outcome in this cohort. A high percentage of those predicted to need univentricular palliation had successful biventricular repair: 89.4% by Rhodes, 79.3% by CHSS 1, 85.2% by Discriminant, and 66.7% by CHSS 2 score. The 2 V best predicted outcome and agreed with the local approach in most cases.
This study highlights the limitations of and need for alternative scoring systems/cut-offs for consistently accurate echocardiographic prediction of early outcome in neonates with critical aortic stenosis.
Osteogenesis imperfecta is a collagen type I bone disorder. Recently, extra-skeletal manifestations have been described, including many cardiovascular alterations. This study aims to report echocardiogram study in children with osteogenesis imperfecta compared to a control group.
A cross-sectional comparative study took place in the Reference Center for Treatment of Osteogenesis Imperfecta in Southern Brazil. Fifty-four patients with osteogenesis imperfecta were paired with 54 controls, based on body surface area, and echocardiogram findings were compared.
All cases were asymptomatic for cardiac manifestations. The case group presented significant larger values in aortic diameter, left atrium diameter, left ventricule end-diastolic diameter, left ventricule end-systolic diameter, and right ventricle diameter compared with the control group. The analysis considering the severity of osteogenesis imperfecta shows that in mild osteogenesis imperfecta, the aortic diameter (p < 0.001), left atrium diameter (p = 0.002), left ventricule end-diastolic diameter (p = 0.001), left ventricule end-systolic diameter (p = 0.026), and right ventricle diameter (p < 0.001) were significantly larger than in the control group. Patients with moderate/severe osteogenesis imperfecta had similar results, with aortic diameter (p < 0.001), left atrium diameter (p < 0.001), left ventricule end-diastolic diameter (p = 0.013), and left ventricule end-systolic diameter (0.004) statistically larger than controls. Twenty-six (48.1%) of the cases had physiological tricuspid regurgitation and in controls this finding was observed in eight (14.8%) (p < 0.001).
Children with osteogenesis imperfecta presented cardiac function within the normal pattern, but dimensions of left ventricular dimensions were increased compared to the ones of the controls.
Cryptogenic cerebral ischemia in young patients is commonly ascribed to paradoxical embolism. We report the clinical case of a young patient with cryptogenic stroke and a patent foramen ovale, undergoing percutaneous closure of atrial septal defect. Contrast transoesophageal echocardiography at the end of the procedure demonstrated massive late residual right-to-left shunt, due to the coexistence of pulmonary arteriovenous fistulas that were subsequently closed. Routinary adoption of contrast transoesophageal echocardiography at the end of patent foramen ovale closure interventions may be useful to detect early and late residual shunts. Late residual shunts may be due to pulmonary fistulas, a well-known risk factor for recurrent thromboembolic events.
To demonstrate the usefulness of N-Terminal Pro-B-Type natriuretic peptide (NT-proBNP) as an early biomarker of carbon monoxide-induced myocardial injury in children. It also aimed to identify the correlation between NT-proBNP and left ventricular systolic dysfunction findings shown by echocardiography.
Prospective, observational study conducted at a paediatric emergency department between October 2017 and April 2019 which involved children aged 0–17 years. The patients were divided into three groups based on severity; mild, moderate and severe groups. The patient characteristics, carboxyhaemoglobin, CK-MB Mass (CKMB-M), troponin-T, and NT-proBNP levels were measured, and echocardiography was performed and left ventricular ejection fraction was measured.
Sixty-nine patients and 60 healthy controls were included. Male gender, younger age, higher carboxyhaemoglobin levels, and altered mental status were found as independent predictors of carbon monoxide-induced myocardial injury. If the cut-off value for NT-proBNP level is >480 pg/ml, the sensitivity–specificity for decreased left ventricular ejection fraction, which is the strongest carbon monoxide-induced myocardial injury sign, were 100–96%, respectively. A high negative correlation was found between NT-proBNP levels and left ventricular ejection fraction (r = −0.769, p < 0.01) in the carbon monoxide poisoning group, and there was a positive correlation between the carboxyhaemoglobin and NT-proBNP levels (r = 0.583, p < 0.01).
Echocardiography is an ideal tool and very sensitive, but its routine use is limited due to its non-availability. An increased level of NT-proBNP (>480pg/ml) may be useful as an ideal biomarker for early detection of carbon monoxide-induced myocardial injury sign and reduced left ventricular ejection fraction which is the most crucial point in making a decision on hyperbaric oxygen therapy.
Introduction: Patients presenting to the emergency department (ED) with hypotension have a high mortality rate and require careful yet rapid resuscitation. The use of cardiac point of care ultrasound (PoCUS) in the ED has progressed beyond the basic indications of detecting pericardial fluid and activity in cardiac arrest. We examine if finding left ventricular dysfunction (LVD) on emergency physician performed PoCUS reliably predicts the presence of cardiogenic shock in hypotensive ED patients. Methods: We prospectively collected PoCUS findings performed in 135 ED patients with undifferentiated hypotension as part of an international study. Patients with clearly identified etiologies for hypotension were excluded, along with other specific presumptive diagnoses. LVD was defined as identification of a generally hypodynamic LV in the setting of shock. PoCUS findings were collected using a standardized protocol and data collection form. All scans were performed by PoCUS-trained emergency physicians. Final shock type was defined as cardiogenic or non-cardiogenic by independent specialist blinded chart review. Results: All 135 patients had complete follow up. Median age was 56 years, 53% of patients were male. Disease prevalence for cardiogenic shock was 12% and the mortality rate was 24%. The presence of LVD on PoCUS had a sensitivity of 62.50% (95%CI 35.43% to 84.80%), specificity of 94.12% (88.26% to 97.60%), positive-LR 10.62 (4.71 to 23.95), negative-LR 0.40 (0.21 to 0.75) and accuracy of 90.37% (84.10% to 94.77%) for detecting cardiogenic shock. Conclusion: Detecting left ventricular dysfunction on PoCUS in the ED may be useful in confirming the underlying shock type as cardiogenic in otherwise undifferentiated hypotensive patients.
The double outlet right ventricle is uncommon and usually makes patients have haemodynamic and structural complications. Having a hyperdynamic state, such as pregnancy, with volume overload is very risky for a patient with complex CHD (CCHD). The diagnosis in early stages can prevent cardiac complications. The multi-disciplinary assessment of the disease lets patients make choices in treatment and reproductive life.
Present a case of a successful pregnancy in a patient with a rare CCHD.
A pregnant 19-year-old patient with a double outlet right ventricle without haemodynamic or structural complications and no fetal abnormalities.