Echocardiography evaluation of neonatal vein of Galen aneurysmal malformation.

BACKGROUND: In neonatal vein of Galen aneurysmal malformation, vein of Galen aneurysmal malformation echocardiography remains the mainstay for early detection and explains various haemodynamic changes occurring due to a large systemic arterio-venous shunt. However, there is limited evidence of echocardiography in risk stratifying neonatal vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation. The objective of this study was to identify echocardiographic parameters that could be associated with major outcomes and guide timing of neuro-intervention. METHODS: In this retrospective chart review, infants < 28 days of age with the diagnosis of vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation were included. Demographic, clinical, and echocardiographic parameters were compared in neonates who survived or died with neonatal presentation. A risk algorithm model based on key echocardiographic parameters was developed to determine those who are at risk of early death. RESULTS: Of the 19 neonates included, with median birth weight 3.1 kg (IQR 2.58-3.36), nine (47%) neonates died at median age of 5 days (IQR 4-17). All neonates showed retrograde diastolic flow at the level of descending aorta by colour Doppler on the first post-natal echocardiogram at median age of 2 days (IQR 1-5.5). An aortic antegrade-to-retrograde velocity time integral ratio of < 1.5 and supra-systemic pulmonary artery pressure had 100% positive predictive value of death (p = 0.029), whereas aortic antegrade-to-retrograde velocity time integral ratio of > 1.5 and sub-systemic pulmonary artery pressure had 100% positive predictive value of survival (p = 0.029). CONCLUSION: Combination of aorta antegrade-to-retrograde velocity time integral ratio and degree of pulmonary hypertension on the first post-natal echocardiogram may help stratify the severity of disease and guide optimal timing for neuro-intervention for neonatal vein of Galen aneurysmal malformation.

Coronary Artery Origins Pattern in Pediatric Patients with Right-Left Fusion Bicuspid Aortic Valve.

Bicuspid aortic valve (BAV) is a common congenital heart defect associated with coronary artery (CA) variants, including higher incidence of left CA dominance and shorter left main CA length. We observed by transthoracic echocardiography that left and right CA origins appear closer together in pediatric patients with right-left fusion (R/L) BAV compared to patients with trileaflet aortic valves. We sought to objectively confirm this observation. A retrospective review of pediatric echocardiograms with R/L BAV at a single institution (12/2010-11/2018) was performed. The 'coronary angle' was defined as the angle between the left and right coronary artery origins in the parasternal short axis view relative to the center of the aortic valve orifice. Values were compared to age-matched controls. Patients with inadequate images, anomalous coronary origins, or predefined significant congenital heart defects were excluded. We compared 191 R/L BAV patients (64% male) to 136 controls (57% male). Coronary angle was significantly more acute in R/L BAV than in controls (117.9° ± 16.7° vs 139.0° ± 10.1°, p < 0.0001). This was independent of age and gender. The difference persisted when BAV patients with abnormal aortic annulus/root diameters were removed from analysis (119.5° ± 15.1° vs 139.0° ± 10.1°, p < 0.0001). CA origins are closer together in R/L BAV independent of age, gender, or annulus/root size. This new anatomical description may aid in the diagnosis of subtle ('forme fruste') R/L BAV, assist in interventional planning, and improve understanding of the relationship between BAV and CA development.

Kawasaki's disease and onychomadesis in a 17-month-old girl with non-SARS-CoV2 coronavirus.

Kawasaki disease is a vasculitis of medium-sized vessels and the most common cause of acquired heart defects in the United States. Although its etiology is unclear, an infectious trigger has been theorized, which has been highlighted by the recent pandemic. We present a case of a 17-month-old-girl with concurrent Kawasaki disease and non-SARS-CoV2 coronavirus infection and a sequela of onychomadesis.

Echocardiographic evaluation of left ventricular systolic function by the M-mode lateral mitral annular plane systolic excursion in patients with Duchenne muscular dystrophy age 0-21 years.

BACKGROUND AND AIMS: Duchenne muscular dystrophy (DMD) results in cardiac fibrosis and dysfunction. These patients frequently have poor image quality. Mitral annular plane systolic excursion (MAPSE) is a reproducible and reliable method for determining function and can be a valuable tool in patients with poor images. Our study was performed to evaluate the feasibility of MAPSE and compare it to shortening fraction (SF) in patients with DMD. METHODS: Lateral M-mode MAPSE was obtained on all echocardiograms performed on DMD patients aged 0 to 21 years between October 2013 and April 2015. Retrospectively, interobserver and intraobserver variability was determined for these measurements and each measurement was compared to patient characteristics and measured values of SF. RESULTS: There was good interobserver (r 2 = .66, P = .0081) correlation. Seventeen of 59 echocardiograms (29%) had abnormal SF while 32 (54%) echocardiograms had an abnormal M-mode lateral MAPSE Z-score. There was no significant association between lateral MAPSE Z-score and SF. Age at the time of echocardiogram and time from diagnosis to echocardiogram both had a significant negative correlation with lateral MAPSE. CONCLUSIONS: Lateral M-mode MAPSE measurements are reproducible in young patients with Duchenne muscular dystrophy. M-mode lateral MAPSE may worsen over length of time with Duchenne muscular dystrophy. Further studies are necessary to provide absolute conclusions, but this study shows that lateral M-mode MAPSE may be a valuable additional tool at routine echocardiogram in these patients.

Sinus of Valsalva Aneurysm in a Patient With Mosaic Trisomy 13: Case Report and Brief Review of the Literature.

This report describes a unique case involving an obese 16-year-old boy with a mosaic form of trisomy 13 and no previous cardiac history who presented with a new murmur, hypertension, pleural effusions, and congestive heart failure in the context of sore throat and fever. Evaluation revealed a diagnosis of ruptured noncoronary sinus of Valsalva (SOV) aneurysm. The diagnosis and surgical management of a ruptured noncoronary SOV aneurysm in a pediatric patient are briefly outlined. An SOV aneurysm is an anatomic dilation of one of the sinuses of the aortic root. Aneurysmal dilation occurs more commonly in the right aortic sinus (70%-80%), compared to the noncoronary sinus (23%-25%), and more rarely the left coronary sinus (5%). Rupture of these aneurysms has been reported to be both spontaneous and secondary to physical exertion, hypertension, or trauma. Signs of rupture include a continuous murmur, patients may present with chest pain or with symptoms of acute congestive heart failure. Diagnosis, in this case, was made by transthoracic echocardiography with careful interpretation of color Doppler images.

Feasibility of real-time cine cardiac magnetic resonance imaging to predict the presence of significant retrosternal adhesions prior to redo-sternotomy.

BACKGROUND: Injury to vital structures posterior to the sternum is a complication associated with redo sternotomy in congenital cardiac surgery. The goal of our study was a novel evaluation of real-time cine cardiovascular magnetic resonance (CMR) to predict the presence of significant retrosternal adhesions of cardiac and vascular structures prior to redo sternotomy in patients with congenital heart disease. METHODS: Twenty-three patients who had prior congenital heart surgery via median sternotomy had comprehensive CMR studies prior to redo sternotomy. The real time cine (RTC) sequence that was used is an ungated balanced steady-state free precession (bSSFP) sequence using SENSitivity Encoding for acceleration with real-time reconstruction. Spontaneously breathing patients were instructed to take deep breaths during the acquisition whilst increased tidal volumes were delivered to mechanically ventilated patients. All patients underwent redo cardiac surgery subsequently and the presence and severity of retrosternal adhesions were noted at the time of the redo sternotomies. RESULTS: Median age at the time of CMR and operation were 5.5 years (range, 0.2-18.4y) and 6.1 years (range, 0.3-18.8y) respectively. There were 15 males and 8 females in the study group. Preoperative retrosternal adhesions were identified on RTC in 13 patients and confirmed in 11 (85%) at the time of surgery. In only 2 patients, no adhesions were identified on CMR but were found to have significant retrosternal adhesions at surgery; false positive rate 15% (CI 0.4-29.6%), false negative rate 20% (CI 3.7-36.4%). The total classification error of the real time cine sequence was 17% (CI 1.7-32.4%) with an overall accuracy of 83% (CI 67.7-98.4%). Standard breath-hold cine images correlated poorly with surgical findings and did not increase the diagnostic yield. CONCLUSIONS: RTC imaging can predict the presence of significant retrosternal adhesions and thus help in risk assessment prior to redo sternotomy. These findings complement the surgical planning and potentially reduce surgical complications .

Decrease in the frequency of treatment for patent ductus arteriosus after implementation of consensus guidelines: a 15-year experience.

BACKGROUND: Patent ductus arteriosus (PDA) management varies widely among neonatologists. LOCAL PROBLEM: Lack of institution-specific evidence-based guidelines for therapeutic closure of PDA. METHODS: Quality improvement project among infants <30 weeks gestational age (GA) designed to determine whether the odds of therapy for closing the PDA, adjusted for GA, decreased after implementing evidence-based guidelines. INTERVENTION: Implementation of guidelines with conservative approach to PDA management. RESULTS: The frequency of PDA treatment decreased from 446/1125 (40%) in Epoch 1 to 96/482 (20%) in Epoch 2. PDA treatment was more frequent in neonates 23-26 weeks GA than those of 27-29 weeks GA (43% vs. 28%, respectively). Among 542 infants receiving indomethacin for PDA, 25% had subsequent ligation; the odds of ligation after indomethacin were lower in neonates 27-29 weeks GA and decreased during Epoch 2. CONCLUSIONS: The frequency of medical and surgical treatment for therapeutic closure of PDA decreased after implementing evidence-based treatment guidelines.

Rationalising the use of cardiac catheterisation before Glenn completion.

Previous studies have shown that cardiac MRI can be used to evaluate the suitability for infants to undergo the Glenn operation after having undergone the Norwood procedure. We sought to analyse our institutional data retrospectively to identify whether such a policy would be advisable in the current era. We reviewed patients who underwent the Norwood procedure between 1 January, 2006 and 1 January, 2016. All patients undergoing evaluation for the Glenn procedure received clinical evaluation, echocardiography, and cardiac catheterisation. A total of 179 patients were identified; 154 patients (86%) survived to undergo cardiac catheterisation as part of evaluation for the Glenn, and all who were evaluated did not eventually receive the Glenn. Using said algorithm, if cardiac MRI or CT were to be used to rationalise the use of catheterisation, 26 of 154 patients would have required catheterisation after cross-sectional imaging identified vascular obstruction; 83 of 154 patients would have received cross-sectional imaging only; and 45 of 154 would have had catheterisation only. All cases that required intervention, excluding aortopulmonary collaterals, and all cases that were not suitable to progress would have been correctly identified using clinical and echocardiographic criteria in addition to cardiac cross-sectional imaging to rationalise the use of catheterisation. Thus, in cases with acceptable clinical, echocardiographic, and angiographic findings, the additional haemodynamic information from catheterisation is rarely of use for decision-making, and interventions can largely be predicted by angiographic imaging modalities.

Implantation of a left ventricular assist device to provide long-term support for end-stage Duchenne muscular dystrophy-associated cardiomyopathy.

A young man with Duchenne muscular dystrophy presented to the UT Southwestern Neuromuscular Cardiomyopathy Clinic with advanced heart failure. Despite maximal medical therapy, his cardiac function continued to decline requiring initiation of inotrope therapy. Given the patient's clinical deterioration, a left ventricular assist device (LVAD) was implanted as destination therapy after undergoing a multidisciplinary assessment. The patient tolerated the surgical implantation of the LVAD without any significant complications, and he has had a relatively unremarkable course 38 months post-LVAD implantation. A critical factor contributing to the long-term success of this patient was the decision to select an LVAD that would not disrupt the diaphragm and thus preserve the respiratory muscle strength. This case demonstrates that permanent mechanical LVADs should be considered for appropriately selected Duchenne muscular dystrophy patients with medically refractory end-stage cardiomyopathy.

Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke.

'Paradoxical' embolization via intracardiac or intrapulmonary right-to-left shunts (RLS) is an established cause of stroke. Hypercoagulable states and increased right heart pressure, which both occur in sickle cell anaemia (SCA), predispose to paradoxical embolization. We hypothesized that children with SCA and overt stroke (SCA + stroke) have an increased prevalence of potential RLS. We performed contrasted transthoracic echocardiograms on 147 children (aged 2-19 years) with SCA + stroke) mean age 12·7 ± 4·8 years, 54·4% male) and a control group without SCA or stroke (n = 123; mean age 12·1 ± 4·9 years, 53·3% male). RLS was defined as any potential RLS detected by any method, including intrapulmonary shunting. Echocardiograms were masked and adjudicated centrally. The prevalence of potential RLS was significantly higher in the SCA+stroke group than controls (45·6% vs. 23·6%, P < 0·001). The odds ratio for potential RLS in the SCA + stroke group was 2·7 (95% confidence interval: 1·6-4·6) vs controls. In post hoc analyses, the SCA + stroke group had a higher prevalence of intrapulmonary (23·8% vs. 5·7%, P < 0·001) but not intracardiac shunting (21·8% vs. 18·7%, P = 0·533). SCA patients with potential RLS were more likely to report headache at stroke onset than those without. Intrapulmonary and intracardiac shunting may be an overlooked, independent and potentially modifiable risk factor for stroke in SCA.

Early echocardiographic predictor of heart failure in cerebral arteriovenous malformations.

Neonatal congestive heart failure in cerebral arteriovenous malformations carries a high risk of mortality. Severe heart failure leading to end-organ dysfunction may be a contraindication to early intervention. Although echocardiographic markers of haemodynamic instability exist, an objective echocardiographic predictor of severe heart failure is not available. We present a ratio of antegrade to retrograde flow in the aortic arch that objectively identifies those at the highest risk of mortality. This measurement can be easily obtained and has prognostic value at presentation, before clinical deterioration. Outcomes remain poor even when early intervention is offered, which may reflect irreversible neurological abnormalities.

Isolated Mitral Cleft in Trisomy 21: An Initially 'Silent' Lesion.

Congenital cardiac anomalies are common in trisomy 21, and transthoracic echocardiogram within the first month of life is recommended. While a cleft mitral valve associated with atrioventricular septal defect has been well defined in this population, the prevalence of isolated mitral valve cleft has not been previously reported. The aim of our study was to define the occurrence of isolated mitral cleft in the first echocardiogram of patients with trisomy 21. This retrospective chart review examined echocardiographic data on all Trisomy 21 patients <1 year of age obtained during January 1, 2010, to May 1, 2014, at our institution. Images were reviewed by one of the authors with no knowledge of the official diagnosis. In addition to evaluation for isolated mitral valve cleft, data obtained included presence of additional congenital heart defects and need for surgical intervention. A total of 184 patients (median age 5 days) were identified. Isolated mitral cleft was identified in 12 patients (6.5 %). Four were diagnosed retrospectively (33 %). Only one had mitral regurgitation on initial echocardiogram. Seven required surgery for closure of ventricular septal defects. Isolated mitral cleft is present in an important number of neonates with Trisomy 21. Mitral regurgitation is often absent in the neonatal period and should not be used as a reliable indicator of absence of valve abnormality. Careful attention should be directed toward the mitral valve during the first echocardiogram to exclude an isolated cleft, which can lead to progressive mitral regurgitation.

Utility and importance of new echocardiographic screening methods in diagnosis of anomalous coronary origins in the pediatric population: assessment of quality improvement.

Anomalous aortic origin of coronaries from the contralateral sinus (AAOCA) is rare but an important cause of cardiac death in the otherwise healthy young athlete. This necessitates prompt, accurate identification; transthoracic echocardiography (TTE) remains the primary screening tool. Assessment of accuracy is difficult since the true prevalence of the disease is unknown, with estimates at 0.3-1.07%. The incidence by TTE remains much lower, between 0.09 and 0.17% even with sophisticated equipment and a high index of suspicion. Our goal was to incorporate two new screening views to our standard TTE protocol and assess improvement in diagnosis of AAOCA by TTE in our laboratory. Recently (2011), we incorporated two new screening methods to standard protocol. The parasternal short axis sweep is extended to visualize the anomalous segment arising superiorly from the ascending aorta before exiting the root at a site close to a 'normal' origin. Secondly, the anomalous, interarterial coronary demonstrates an anterior, steep-angled course visualized in the parasternal long axis between the aorta and pulmonary artery. The echocardiogram database was searched for patients newly diagnosed with AAOCA in 2010 (prior to incorporation of new methods) and 2012. AAOCA incidence in our patient population improved from 0.02% (2010) to 0.22% (2012), age range from 4 days to 17 years. Teenagers and symptomatic patients with anomalous right coronary origin (6) underwent additional confirmatory imaging, and three underwent surgery. One patient with anomalous left coronary origin underwent surgical repair. Addition of the screening views can significantly increase the sensitivity of TTE in diagnosing AAOCA in the asymptomatic patients. We propose that these views be incorporated into the standard TTE evaluation of coronary arteries.

Unruptured sinus of valsalva aneurysm in a neonate with hypoplastic left heart syndrome: echocardiographic diagnosis and features.

An unruptured sinus of Valsalva aneurysm is usually asymptomatic, and a rare, often incidental echocardiographic diagnosis. Its presentation in the neonatal period is extremely rare. We describe this finding in a neonate with hypoplastic left heart syndrome, which has not been previously reported in the literature. This constellation of findings may have important clinical implications in regards to medical management and surgical options.

Elevated troponin levels in previously healthy children: value of diagnostic modalities and the importance of a drug screen.

BACKGROUND: Myocardial injury in previously healthy children is rare, with a wide range of aetiologies. It is increasingly being identified on the basis of elevated troponin levels during routine evaluation of cardiorespiratory symptoms. Establishing the aetiology remains challenging because of the lack of an accepted work-up algorithm. Our objective was to delineate the contribution of diagnostic modalities and troponin patterns towards the final diagnosis. METHODS: A retrospective chart review of previously healthy patients admitted to the Pediatric Cardiology Service with myocardial injury was carried out. Data analysed included echocardiograms, electrocardiograms, cardiac catheterisations, magnetic resonance imaging, drug screen tests, troponin values, and final diagnosis. RESULTS: A total of 32 patients were identified. The diagnoses were: myocarditis in 16 patients, vasospasm due to drug use in seven, myopericarditis in six, anomalous coronary artery origins in two, and Prinzmetal's angina in one patient. The electrocardiograms were abnormal in 27 of the 32 patients (84%), echocardiograms in 18 of the 32 patients (56%), cardiac magnetic resonance imaging in two of the four patients (50%), urine drug screen in five of the 25 patients (20%), and cardiac catheterisations in two of the 15 patients (13%). CONCLUSIONS: Myocarditis is the most common aetiology of myocardial injury in children. Clinical history remains the basic screening tool; drug screens help identify coronary vasospasms secondary to drug use (22% of our cohort). Patients with anomalous coronaries had exertional symptoms. Initial troponin levels and progression were not diagnostic or prognostic. Catheterisation is of limited value and did not change management. Magnetic resonance imaging with gadolinium enhancement is probably the most useful test when initial evaluation is not diagnostic.

Anomalous left main coronary artery origin from the right sinus of Valsalva: a novel echocardiographic screening method.

Anomalous origin of the left main coronary artery from the right sinus (ALMCA) is a rare entity associated with an increased risk for sudden cardiac death. Although transthoracic echocardiography often is the primary screening tool for the diagnosis, its accuracy remains undefined, resulting in the use of more expensive methods such as cardiac catheterization and magnetic resonance imaging when clinical suspicion exists. This report aims to describe a novel echocardiographic screening method for detecting ALMCA and to apply it retrospectively for patients with confirmed findings intraoperatively and/or on autopsy. All patients with a confirmed diagnosis of ALMCA who presented to our institution from 2004 to 2012 were included in the study. Eight patients had adequate images, including two patients with an initial diagnosis of normal coronary origins. A control group consisted of 35 patients with normal coronaries shown by echocardiogram. Five of these patients had normal coronary artery origins confirmed by magnetic resonance imaging (MRI). Echocardiographic images were reviewed, and the left main coronary was identified in the parasternal long-axis view between the aorta and pulmonary trunk. The angle of the left main coronary course was measured. In patients with ALMCA, the proximal course of the vessel was steeper as it coursed posteriorly. In contrast, the course was almost horizontal in patients with normal coronary origins. Based on these findings, a cutoff angle of 28° is proposed. In the parasternal long-axis view, ALMCA can be identified by its anomalous proximal course. This screening method is reliable and increases the accuracy of transthoracic echocardiograms.