Vein of Galen Aneurysmal Malformation with Anomalous Right Superior Vena Cava to the Left Atrium Leading to Atypical Clinical and Echocardiographic Findings.

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1 week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4 months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM.

HeartWatch: Implementing a Pediatric Heart Center Program to Prevent Cardiac Arrests Outside the ICU.

Pediatric patients with cardiovascular disease are at increased risk of cardiopulmonary arrest. Despite utilization of Cardiac Pediatric Early Warning Scores to identify patients at risk of decompensation, our institution had a twofold increase in cardiac arrests (CAs) in the acute care cardiology unit (ACCU) over 2 years. Through a quality improvement initiative, we developed a watcher program, HeartWatch, to reduce the CA arrest rate in the ACCU by 50% over the first year of implementation. Methods: HeartWatch aims to identify patients not adequately captured by Cardiac Pediatric Early Warning Scores who are at high risk for sudden decompensation. Inclusion criteria were developed and evaluated during pilot and implemented phases (April 2020-April 2021) and then monitored in a sustained phase through June 2022. Our primary outcome was the reduction in the out-of-ICU CA rate. Results: During the 13 months, we enrolled 169 patients, and the CA rate decreased from 0.7 to 0.33 per 1,000 patient days, a 53% reduction. The CA rate further decreased to 0.28 events per 1,000 patient days, a 60% reduction, by June 2022. The most common indications for HeartWatch inclusion were high-risk single-ventricle patients (31%) and patients with diminished ventricular function (20%). Conclusions: Implementation of HeartWatch was associated with a meaningful reduction in CA in the ACCU. Creating shared mental models for high-risk patients is essential for patient safety. Future work will optimize local processes that focus on the sustainability of our gains. We will also evaluate opportunities to adapt and implement a similar framework in other institutions to assess reproducibility.

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.

Two-dimensional systolic speckle tracking echocardiography provides a noninvasive aid in the identification of acute pediatric heart transplant rejection.

BACKGROUND: Acute rejection is an important cause of morbidity and mortality in the pediatric heart transplant (HT) population. A reliable noninvasive method for diagnosis of clinical rejection could substantially reduce these negative outcomes. OBJECTIVE: Evaluate left ventricular (LV) global longitudinal strain (GLS), and global circumferential strain (GCS) as early noninvasive indicators of acute pediatric HT rejection. METHODS: An 18-month prospective cohort study involving 61 patients evaluated absolute change in peak global systolic strain (GLS and GCS) from enrollment (baseline) to next planned clinical encounter (follow-up) or rejection. Acute rejection defined as a biopsy of grade ≥ 2R or treatment with enhanced immunosuppression by the transplant team, blinded to strain analysis. Two patient cohorts three months post HT without evidence of rejection at enrollment were identified. The study cohort experienced rejection. The control cohort remained free from rejection on follow-up. Two-way analysis of variance (ANOVA) models evaluated change in GLS and GCS by cohort group and time. RESULTS: Applying exclusion criteria, 51 patients enrolled in the control cohort and 10 in the study cohort. The study cohort's mean GLS declined 33% from baseline to rejection (P < .001) and mean GCS declined 16.6% (P = .021). No significant change from baseline to follow-up was seen in the control cohort. A threshold absolute GLS value of 16.1% identified acute rejection with 100% sensitivity and 98% specificity (Likelihood Ratio, [LR] 51). CONCLUSION: Noninvasive global longitudinal strain was sensitive and specific in the identification of acute clinical rejection in pediatric HT recipients.

Use of Amplatzer Vascular Plugs for the treatment of combined extralobar and intralobar pulmonary sequestration in a 5-year-old child.

Pulmonary sequestration is a rare congenital anomaly that can be asymptomatic or present with recurrent infections, respiratory symptoms, or rarely heart failure. Sequestration is classified as intralobar or extralobar on the basis of whether there is separation from normal lung tissue by its own visceral pleura. Classically, patients are treated with surgical resection. We present a case of multivessel, combined intralobar and extralobar pulmonary sequestration treated with transcatheter embolisation.

Health-Related Quality of Life and Functional Status Are Associated with Cardiac Status and Clinical Outcome in Children with Cardiomyopathy.

OBJECTIVES: To measure the health-related quality of life (HRQOL) and functional status of children with cardiomyopathy and to determine whether they are correlated with sociodemographics, cardiac status, and clinical outcomes. STUDY DESIGN: Parents of children in the Pediatric Cardiomyopathy Registry completed the Child Health Questionnaire (CHQ; age ≥ 5 years) and Functional Status II (Revised) (age ≤ 18 years) instruments. Linear and Cox regressions were used to examine hypothesized associations with HRQOL. RESULTS: The 355 children evaluated at ≥ 5 years (median 8.6 years) had lower functioning (CHQ Physical and Psychosocial Summary Scores 41.7 ± 14.4 and 47.8 ± 10.7) than that of healthy historical controls. The most extreme CHQ domain score, Parental Impact-Emotional, was one SD below normal. Younger age at diagnosis and smaller left ventricular end-diastolic dimension z score were associated independently with better physical functioning in children with dilated cardiomyopathy. Greater income/education correlated with better psychosocial functioning in children with hypertrophic and mixed/other types of cardiomyopathy. In the age ≥ 5 year cohort, lower scores on both instruments predicted earlier death/transplant and listing for transplant in children with dilated and mixed/other types of cardiomyopathy (P < .001). Across all ages (n = 565), the Functional Status II (Revised) total score was 87.1 ± 16.4, and a lower score was associated with earlier death/transplant for all cardiomyopathies. CONCLUSIONS: HRQOL and functional status in children with cardiomyopathy is on average impaired relative to healthy children. These impairments are associated with older age at diagnosis, lower socioeconomic status, left ventricular size, and increased risk for death and transplant. Identification of families at risk for functional impairment allows for provision of specialized services early in the course of disease. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00005391.

Aspirin Resistance in Single-Ventricle Physiology: Aspirin Prophylaxis Is Not Adequate to Inhibit Platelets in the Immediate Postoperative Period.

BACKGROUND: Incidence of thrombosis after initial stage 1 single-ventricle palliation is high. Most centers use aspirin as an antiplatelet agent to prevent thrombosis in surgically placed shunts. We hypothesize there is a significant incidence of aspirin resistance in infants after stage 1 palliation and this resistance can be overcome by an increased aspirin dose. METHODS: This is a prospective observational study of 20 patients with single-ventricle physiology who required single-ventricle palliation with a controlled source of pulmonary blood flow (Norwood/Sano, Norwood/Blalock-Taussig [BT] shunt or BT shunt alone). Aspirin resistance was determined using thromboelastography with platelet mapping (TEG) and urine thromboxane (UTX). The UTX level of less than 1,500 pg/mL and TEG value of more than 50% were used to define as adequate platelet inhibition. The UTX was measured prior to starting aspirin (20 mg/day) and TEG and UTX were obtained after 5 days of aspirin therapy A repeat UTX was measured for patients who were determined to be aspirin resistant by TEG (<50% arachidonic acid inhibition) after doubling the dose (40 mg/day). Clinical variables including patient diagnosis, age of surgery, and cardiopulmonary bypass requirement, weight, hemoglobin, and platelet count were assessed to determine their association with aspirin resistance. RESULTS: Eighty percent of patients were aspirin resistant using TEG (95% CI, 56% to 94%) and none of the patients achieved a UTX level of less than 1,500 pg/mL. Aspirin resistant patients did not respond to an increased dose of aspirin between the fifth and tenth days of therapy (p = 0.820). Patients did, however, respond to aspirin treatment when comparing the baseline UTX measurement with those recorded on the fifth day (p = 0.008) and the tenth day (p = 0.0361) of aspirin therapy. The UTX levels did not differ between those who were and those who were not aspirin resistant by TEG at any of the measurement times. The clinical variables were not associated with aspirin resistance status. CONCLUSIONS: There is a high incidence of aspirin resistance in the immediate postoperative period after single-ventricle shunt palliation. Aspirin might not be an adequate agent for shunt prophylaxis in this patient population. Further studies are needed to identify at-risk patients who might benefit from additional testing and specific anticoagulation.

Subclinical cardiotoxicity in childhood cancer survivors exposed to very low dose anthracycline therapy.

BACKGROUND: Subclinical cardiotoxicity occurs in childhood cancer survivors following moderate and high anthracycline doses. However, less is known about the subclinical changes in left ventricular (LV) structure that occur after very low anthracycline doses of ≤ 100 mg/m(2). This study was designed to assess LV function and key structural parameters following very low doses of anthracycline. PROCEDURE: Conventional 2-dimensional echocardiograms with Doppler were obtained in 91 survivors of childhood cancer exposed to ≤ 100 mg/m(2), an average of 9.8 years from diagnosis. LV structural measurements were converted into Z-scores. The Z-score distributions were compared to those of the normal population. Diastolic and systolic function were quantified. RESULTS: The cohort demonstrated a decreased posterior wall thickness (mean Z-score -1.01) and mildly decreased LV end diastolic (mean Z-score -0.85) and systolic (mean Z-score -0.84) dimensions compared to the normal population (P < 0.001). Further, 28% of patients (n = 25) had abnormal LV posterior wall thickness, ≥ 2 standard deviations below the mean (Z-score ≤ -2). There were no patients with diastolic dysfunction or symptomatic systolic dysfunction, however four patients demonstrated abnormal SF ≤ 28%. CONCLUSIONS: A significant proportion of patients exposed to very low doses of anthracycline demonstrate subclinical abnormalities in LV structure, despite the absence of radiation or other risk factors. While we cannot say whether these structural changes will result in clinically significant cardiac disease, the reported progressive nature of these findings raises concern that there may truly be no safe dose of anthracycline.

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.

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.

Hypertrophic cardiomyopathy in a neonate associated with nemaline myopathy.

Nemaline myopathy is a congenital nonprogressive skeletal muscle disorder with a characteristic rod body formation in the skeletal muscle fibers. Cardiac involvement in nemaline myopathy is rare, although both dilated and hypertrophic cardiomyopathy have been reported. We describe an infant diagnosed with hypertrophic cardiomyopathy and hypotonia on the first day of life. Muscle biopsy confirmed nemaline myopathy at 3 weeks of age. The diagnosis of nemaline myopathy precluded consideration of heart transplantation, thus shifting the focus to comfort care. This is the earliest presentation of hypertrophic cardiomyopathy reported in the literature in the setting of nemaline myopathy. The approach to determining an etiology for hypertrophic cardiomyopathy in an infant is reviewed.

A neonate with left congenital diaphragmatic hernia and transient retrograde flow in the aortic arch.

Retrograde flow in the aortic arch identifies inadequate cardiac output in neonates with congenital heart disease. We present a left congenital diaphragmatic hernia (CDH) infant with retrograde flow in the arch that normalized after CDH repair. We hypothesize transient inadequacy of the left heart; prostaglandins might play a pivotal role.

Diagnosing a patent foramen ovale in children: is transesophageal echocardiography necessary?

BACKGROUND AND PURPOSE: transesophageal echocardiography (TEE) is the gold standard for the diagnosis of a patent foramen ovale in adults. In children, acoustic windows on transthoracic echocardiography (TTE) are better than in adults; thus, an invasive TEE may not be necessary. Our goal was to assess the validity of TTE with agitated saline injection for the diagnosis of a patent foramen ovale in children using TEE as the gold standard. METHODS: fifty consecutive pediatric patients >1 year of age referred for TEE were prospectively enrolled. Imaging included 2-dimensional, color Doppler, and agitated saline contrast injections with and without Valsalva by TTE followed by TEE. Interpreters of the TTE were blinded to TEE results. Studies were categorized as "inconclusive" if the TTE images were inadequate for definitive diagnosis by the blinded interpreter. RESULTS: TTE results were considered conclusive in 43 of 50 (86%) patients. Among the 43 conclusive studies, the 2 modalities disagreed in 1 patient. TTE had a positive predictive value of 100%, negative predictive value 97%, sensitivity of 88%, and specificity of 100% for detecting a patent foramen ovale. CONCLUSIONS: TTE with agitated saline injection is diagnostic for the assessment of atrial septal integrity in the majority of children.

Relation of right ventricular pacing site to left ventricular mechanical synchrony.

Transvenous pacing leads are regularly placed in the right ventricular (RV) apex. Pediatric patients can develop myopathic changes after long-term RV apical pacing. Left ventricular (LV) mechanical dyssynchrony, estimated with echocardiography, may explain the acute decrease in LV function and long-term histopathologic changes. Ts-4w is an established echocardiographic measurement of LV synchrony, using tissue Doppler imaging (TDI). The purpose of this study was to determine whether TDI could identify acute changes in LV synchrony during pacing from different RV sites. We prospectively measured Ts-4w and Doppler-derived cardiac output after 5 minutes of pacing in 19 subjects undergoing catheter ablation. Each subject underwent pacing at 4 sites in random order: high right atrium, high RV septum (septal), RV outflow tract, and RV apex. Ts-4w was measured during sinus rhythm and each pacing protocol, with a value >65 ms defining mechanical dyssynchrony. Ts-4w during high right atrial (32.6 +/- 17.6 ms) and septal (28.9 +/- 10.9 ms) pacing were not different from sinus rhythm (39.5 +/- 15.5 ms). RV apex (85.7 +/- 18.4 ms) and RV outflow tract (84.2 +/- 20.4 ms) pacing induced mechanical dyssynchrony (p <0.0001). In conclusion, TDI demonstrated significant differences in LV synchrony related to pacing site. Ts-4w may be useful to determine ideal lead placement because it correlates with acutely improved hemodynamics.

Oxygen supplementation is helpful for the echocardiographic detection of anomalous left coronary artery from the pulmonary artery.

BACKGROUND: The echocardiographic diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA) can be challenging. The aim of this study was to assess the hypothesis that diagnosis can be enhanced by using supplemental oxygen, which decreases pulmonary vascular resistance and increases retrograde flow from the coronary artery into the pulmonary artery. METHODS: Demographic, echocardiographic, and cardiac catheterization data were reviewed in patients presenting with ALCAPA from 1999 to 2007. RESULTS: Twenty-one patients (seven male; median age, 5 months) presented with ALCAPA. Nine underwent imaging with oxygen. Two of these nine (22%) had previous standard echocardiographic studies that missed the diagnosis. Cardiac catheterization was required for diagnosis of ALCAPA in 42% of patients who underwent standard echocardiography compared with 11% of patients who received supplemental oxygen in addition to standard echocardiography. The administration of oxygen caused no significant change in heart rate or cardiorespiratory support. CONCLUSION: Transient oxygen administration is useful in the noninvasive diagnosis of ALCAPA.

Echocardiography, not chest radiography, for evaluation of cannula placement during pediatric extracorporeal membrane oxygenation.

OBJECTIVE: Optimal cannula position is essential during extracorporeal membrane oxygenation (ECMO). We hypothesize that echocardiography is superior to chest radiography in diagnosing abnormal cannula position during ECMO. DESIGN: Retrospective. SETTING: Pediatric hospital. PATIENTS: 100 pediatric patients requiring ECMO. MEASUREMENTS AND MAIN RESULTS: We reviewed the medical records of all ECMO patients (n = 100), including reports of all echocardiograms (n = 326), during the years 2002-2004. Of the 91 patients who had echocardiograms while on ECMO, 33 had at least 1 echocardiogram for cannula-position evaluation. Of the remaining 58 patients with echocardiograms for reasons other than cannula-position evaluation, 4 (7%) were found to have abnormal cannula position. These included arterial cannula (AC) within 2-4 mm of the aortic valve (n = 2), AC across the aortic valve into the left ventricle (n = 1), and venous cannula (VC) abutting the atrial septum (n = 1). Of the 33 patients with echocardiograms for evaluation of cannula position, 8 (24%) required intervention. Of those 8 patients, 4 required cannula repositioning due to VC in the coronary sinus (n = 1), VC abutting atrial septum (n = 1), AC in left subclavian artery (n = 1), and AC within 3 mm of aortic valve (n = 1). The remaining 4 with normal cannula position required upsizing of the VC (n = 2), increased circuit flow (n = 1), or intravascular volume administration (n = 1). Overall, 12 of 91 patients (13%) required intervention based on echocardiographic findings. Chest radiography did not detect abnormalities of ECMO cannula position in any of the 8 patients with this problem, nor were any additional patients with abnormal cannula position identified by chest radiography. CONCLUSIONS: Echocardiography appears to be superior to chest radiography for assessing ECMO cannula position in our institution. A prospective study, including cost analysis, comparing chest radiography and echocardiography, is needed to definitely determine the preferred diagnostic test or sequence of tests to establish ECMO cannula position.