The Apical View Sign for Easier Identification of Left Juxtaposition of the Right Atrial Appendage.

Left juxtaposition of the right atrial appendage (RAA) is a rare congenital cardiac malformation, where both atrial appendages are located leftward of the great arteries. Despite well-described echocardiographic markers, this diagnosis remains challenging to establish, especially for the novice imager. We describe the apical view sign in three patients with juxtaposed RAA. We noted that the normal left atrial appendage was visualized from the apical four-chamber or equivalent coronal view at the level of the atrioventricular valves.

The evolution of fetal echocardiography before and during COVID-19.

The World Health Organization declared the novel coronavirus, or COVID-19, a pandemic in March 2020. Given the severity of COVID-19, appropriate use criteria have been implemented for fetal echocardiography. Screening low risk pregnancies for critical congenital heart disease has typically been a shared responsibility by pediatric cardiologists, obstetricians, and maternal fetal medicine (MFM). Currently, many of the fetal echocardiograms for low risk pregnancies for critical congenital heart disease have been deferred or cancelled with the emphasis on suspected abnormalities by MFMs and obstetricians. In this review, we discuss the literature that has been the basis of screening of low risk pregnancies by pediatric cardiologists. A new approach to more widespread usage of fetal tele-echocardiography may play a large part during COVID-19 and may continue after the pandemic.

Outcomes after anomalous aortic origin of a coronary artery repair: A Congenital Heart Surgeons' Society Study.

OBJECTIVES: It remains unclear when sudden cardiac event risk outweighs surgical risk for patients with anomalous aortic origin of a coronary artery. The Congenital Heart Surgeons' Society sought to characterize the surgical risks by determining the techniques, complications, and outcomes of repair. METHODS: Between January 2000 and September 2018, 682 patients with anomalous aortic origin of a coronary artery aged 30 years or less were enrolled. Demographic, morphologic, operative, imaging, and ischemia-related data were analyzed. RESULTS: There were 395 of 682 (57%) surgical patients (45 centers, median follow-up 2.8 years). In addition to primary repair (87% unroofing, 26% commissural manipulation), 13 patients had 15 coronary-related reoperations. Of 358 patients with pre/postoperative aortic insufficiency assessment, 27 (8%) developed new mild or greater aortic insufficiency postoperatively, and 7 (2%) developed new moderate or greater aortic insufficiency. Freedom from mild aortic insufficiency differed in those with versus without commissural manipulation (85%/91% at 6 months, 83%/90% at 1 year, and 77%/88% at 3 years, respectively) (P = .05). Of 347 patients with preoperative/postoperative ejection fraction, 6 (2%) developed new abnormal ejection fraction (<50%) within 30 days of surgery which persisted. Although 64 of 395 patients (16%) had preoperative ischemia, after surgery 51 of 64 patients (80%) no longer had ischemia (13 = new postoperative ischemia, P < .0001). Four patients died postoperatively (preoperatively 2 asymptomatic, 1 symptomatic, 1 in extremis). Composite surgical adverse event rates were 7% to 13% in the entire cohort (increasing/decreasing by presentation/anatomy/repair strategy). CONCLUSIONS: Anomalous aortic origin of a coronary artery surgery may relieve ischemia with low mortality; however, it can result in a variety of important morbidities, varying by the group evaluated. Strategies avoiding commissural manipulation may decrease the risk of developing aortic insufficiency. Understanding these risks should inform surgical decision-making and support the need for standardized assessment and management.

Features associated with myocardial ischemia in anomalous aortic origin of a coronary artery: A Congenital Heart Surgeons' Society study.

OBJECTIVES: We sought to determine anatomic features associated with evidence of myocardial ischemia and sudden cardiac events (arrest or death) for patients with anomalous aortic origin of a coronary artery. METHODS: We enrolled 560 patients, less than or equal to 30 years, at diagnosis from 40 institutions. Ischemia was defined as the presence of exertional syncope, a sudden cardiac event (arrest/death), or abnormal investigation results. Data on detailed anatomic features were abstracted from echocardiography, computed tomography, magnetic resonance imaging, operative, and/or surgeon-completed reports. RESULTS: There were 236 patients with negative ischemia test results, and 49 with evidence of ischemia (including 18 who presented with a sudden cardiac event); 275 asymptomatic patients who had not undergone provocative ischemia testing were excluded from primary analyses. Patients with ischemia (vs without), were more likely to have left anomalous coronary arteries (28/49 vs 46/236; P < .0001). Of patients with ischemia (vs without), those with anomalous left coronary arteries were more likely to have an intramural coronary artery course, or a high or slit-like coronary artery orifice. Of patients with ischemia (vs without), those with anomalous right coronary arteries were more likely to have a longer intramural course. Among patients with ischemia, the occurrence of sudden cardiac events was not shown to have any associated anatomic features. CONCLUSIONS: Anatomic features including coronary artery involved, intramural course and length, and orifice anomalies were associated with evidence of myocardial ischemia for patients with anomalous aortic origin of a coronary artery. These features might importantly inform risk stratification and decisions regarding surgical management.

Transseptal puncture using surgical electrocautery in children and adults with and without complex congenital heart disease.

BACKGROUND: Atrial transseptal puncture (TSP) for cardiac catheterization procedures remain challenging in children and adults with complex congenital heart disease (CHD). OBJECTIVES: We sought to evaluate our experience using radiofrequency (RF) current via surgical electrocautery needle for TSP to facilitate diagnostic and interventional procedures. METHODS: Retrospective chart review of all patients (pts) who underwent TSP using RF energy (10-25 W) via surgical electrocautery from three centers from January 2011 to January 2017 were evaluated. Echocardiograms were reviewed to define the atrial septum as normal and complex (thin aneurysmal, thick/fibrotic, synthetic patch material, and extra cardiac conduit). RESULTS: A total of 54 pts underwent 55 successful TSP. Median age was 12.5 years (1 day-54 years) and weight was 52.7 kg (2-162). Indications for TSP included; EP study and ablation procedures in structurally normal hearts (n = 24) and in complex atrial septum/CHD and structural heart disease pts (n = 30): Electrophysiology study and ablation in 4, diagnostic catheterization in 9, and interventional procedures in 17 pts were performed. Atrial TSP was successful in 54/55 (98%). Atrial perforation with tiny-small pericardial effusion not requiring intervention was noted in 2 pts. TSP was unsuccessful in one critically ill neonate with unobstructed TAPVR and restricted atrial septum who experienced cardiac arrest requiring CPR, ECMO, and emergent surgery. CONCLUSIONS: RF current delivery using surgical electrocautery for TSP is a feasible and an effective option in patients with complex CHD for diagnostic, interventional, and electrophysiology procedures.

Is Interarterial Course a Requisite for Ischemia in Anomalous Origin of Coronary Arteries?

Anomalous origin of the left main coronary artery from the noncoronary sinus (LCANS) is a rare variant of anomalous aortic origin of a coronary artery that is not characterized by an interarterial course. Despite the absence of an interarterial course, there are rare reports of LCANS presenting with sudden death, life-threatening arrhythmia, exercise-induced myocardial ischemia, premature ventricular contractions, and syncope. We report a patient with LCANS presenting with acute myocardial infarction who recovered completely with emergent surgical unroofing of the coronary artery.

Echocardiographic Diagnosis of Left Main Coronary Artery Atresia.

This case describes an infantile presentation of left main coronary artery atresia (LMCAA), which involves complete absence of the left coronary ostium and left main coronary artery. The echocardiographic features of LMCAA that assist in making this diagnosis are detailed. Important imaging features that distinguish LMCAA from anomalous left coronary artery from the pulmonary artery are highlighted.

Risk stratification with exercise N(13)-ammonia PET in adults with anomalous right coronary arteries.

OBJECTIVE: In adults with an interarterial and intramural course of an anomalous right coronary artery from the left sinus (AAORCA), surgical unroofing is recommended in the setting of myocardial ischaemia. However, data regarding functional testing are limited, and the management of adults without ischaemia is unclear. To evaluate these patients, we employed an exercise N(13)-ammonia positron emission tomography (PET) protocol. We hypothesised that patients with typical angina and exertional dyspnoea would be more likely to have ischaemia and that patients without ischaemia could be managed conservatively. METHODS: Between July 2008 and December 2014, we retrospectively identified 27 consecutive patients >18 years old with an interarterial and intramural course of an AAORCA who had exercise N(13)-ammonia PET. RESULTS: The majority of patients had anatomic delineation with cardiac CT (25, 93%), and most patients had chest pain (24, 89%). Myocardial ischaemia with PET was common (13, 48%), and ischaemia was more likely in patients with typical angina and exertional dyspnoea (p<0.05). Surgery was performed in 12 patients including 11 patients with ischaemia. At a median follow-up of 245 days, there were no deaths in patients with surgery or in patients managed conservatively. CONCLUSIONS: In patients with an interarterial and intramural course of an AAORCA, typical angina and exertional dyspnoea are associated with ischaemia on exercise N(13)-ammonia PET. Referral for surgical unroofing in symptomatic patients with ischaemia on exercise N(13)-ammonia PET and initial conservative management in patients without ischaemia seems appropriate, though larger studies with long-term follow-up are needed.

Anomalous Aortic Origin of Coronary Arteries in the Young: Echocardiographic Evaluation With Surgical Correlation.

OBJECTIVES: This study sought to compare findings from institutional echocardiographic reports with imaging core laboratory (ICL) review of corresponding echocardiographic images and operative reports in 159 patients with anomalous aortic origin of a coronary artery (AAOCA). The study also sought to develop a "best practice" protocol for imaging and interpreting images in establishing the diagnosis of AAOCA. BACKGROUND: AAOCA is associated with sudden death in the young. Underlying anatomic risk factors that can cause ischemia-related events include coronary arterial ostial stenosis, intramural course of the proximal coronary within the aortic wall, interarterial course, and potential compression between the great arteries. Consistent protocols for diagnosing and evaluating these features are lacking, potentially precluding the ability to risk stratify patients based on evidence and plan surgical strategy. METHODS: For a prescribed set of anatomic AAOCA features, percentages of missing data in institutional echocardiographic reports were calculated. For each feature, agreement among institutional echocardiographic reports, ICL review of images, and surgical reports was evaluated using the weighted kappa statistic. An echocardiographic imaging protocol was developed heuristically to reduce differences between institutional reports and ICL review. RESULTS: A total of 13%, 33%, and 62% of echocardiograms were missing images enabling diagnosis of intra-arterial course, proximal intramural course, and high ostial takeoff, respectively. There was poor agreement between institutional reports and ICL review for diagnosis of origin of coronary artery, interarterial course, intramural course, and acute angle takeoff (kappa = 0.74, 0.11, -0.03, 0.13, respectively). Surgical findings were also significantly different from those of reports, and to a lesser extent ICL reviews. The resulting protocol contains technical recommendations for imaging each of these features. CONCLUSIONS: Poor agreement between institutional reports and ICL review for AAOCA suggests need for an imaging protocol to permit evidence-based risk stratification and surgical planning. Even then, delineation of echocardiographic details in AAOCA will remain imperfect.

Anomalous origin of the left coronary artery from the noncoronary cusp: not a benign lesion.

This case report describes two patients with a very rare condition who presented with pathologic symptoms. Anomalous origin of the left coronary artery from the noncoronary cusp has been described as a "benign" lesion by some authors in the past, although rare cases of morbidity/mortality are described in the literature. Both reported patients underwent surgical repair for the lesion and at this writing are asymptomatic at follow-up evaluation. These two patients presenting with pathologic symptoms and undergoing surgery afford novel descriptions. The authors believe these descriptions add to our knowledge of this rare disorder.

Surgery for anomalous aortic origin of the coronary artery.

BACKGROUND: Anomalous aortic origin of the coronary artery (AAOCA) has been associated with coronary ischemia, myocardial infarction, and sudden death. Advances in echocardiography and computed tomography have identified at-risk patients. Treatment options include unroofing strategies in symptomatic and asymptomatic patients. We review our experience for efficacy and safety. METHODS: Between 1998 and 2008, we performed coronary unroofing in 22 patients with AAOCA without aortic commissural detachment. Of 7 patients with "left from right" AAOCA, 4 had chest pain only, 1 had syncope, 1 had myocardial infarction, and 1 was asymptomatic. Of 15 patients with "right from left" AAOCA, 11 had chest pain only, 4 had syncope, and none were without symptoms. Median age was 15 years (range, 5 to 54). Eight patients had concomitant procedures, most commonly patent foramen ovale closure. RESULTS: There were no deaths or complications. Mean cross-clamp time was 53 minutes. Mean length of stay was 4 days. Postoperative evaluation included echocardiography, computed tomography angiogram, stress thallium, stress echocardiography, and exercise stress test. In all patients, the repaired coronary was patent, with demonstrated flow. Mean follow-up was 17 months (range, 1 to 63). CONCLUSIONS: Anomalous aortic origin of the coronary artery is emerging as an identifiable disease entity associated with symptoms or sudden death. We conclude that coronary unroofing is a safe and effective therapy for symptomatic patients. Surgery for asymptomatic patients has been more controversial, with growing advocacy for patients with "left from right" as opposed to "right from left" origins. Prospective studies will be required to answer these questions.

Long-term outcome and impact of surgery on adults with coronary arteries originating from the opposite coronary cusp.

BACKGROUND: An anomalous coronary artery from the opposite sinus of Valsalva may increase sudden death risk in children and young adults, and surgical intervention is often recommended. The impact of this lesion when recognized in the adult and its management are ill defined. METHODS AND RESULTS: We reviewed 210 700 cardiac catheterizations performed over a 35-year period at a single institution and identified 301 adults with an anomalous coronary artery from the opposite sinus of Valsalva, either anomalous right coronary artery from the left cusp or anomalous left main coronary artery from the right cusp. Patients were stratified by the pathway of the anomalous artery and the chosen treatment. Among the 301 patients with anomalous coronary artery from the opposite sinus of Valsalva (0.14% of the cohort), 79% had anomalous right coronary artery from the left cusp, and 18% had an interarterial course (IAC). Patients with IAC were younger (52±13 versus 59±13 years; P=0.001) and more likely to undergo surgical intervention (52% versus 27%; P<0.001), but mortality was not increased with IAC. Among the 54 patients with IAC, 28 underwent surgical repair with no perioperative deaths. Patients evaluated since 2000 were significantly more likely to be referred for surgery (P=0.004). Surgical patients were more likely to have abnormal stress tests (90% versus 43%; P=0.01) and had more extensive atherosclerosis but less diabetes mellitus (0% versus 23%; P=0.01). Long-term survival at 10 years appeared similar in both groups. CONCLUSIONS: In this single-center cohort study of patients with an anomalous coronary artery from the opposite sinus of Valsalva, surgical management appears to have been favored recently. Despite no perioperative mortality, a positive impact on long-term survival was not observed. The impact of surgery in older adults with anomalous coronary arteries arising from the opposite coronary sinus with IAC deserves further study.

An overview of surgery options for congenital coronary artery anomalies.

Congenital and acquired coronary artery anomalies are associated with significant morbidity and mortality and can be sudden in onset. The spectrum of congenital lesions include anomalous origin from the pulmonary artery, critical left main stenosis/atresia, coronary artery fistulas, anomalous aortic origin and intramyocardial courses. The spectrum of acquired lesions include Kawasaki disease, late postoperative obstructions in patients who had coronary artery surgical manipulations and iatrogenic injuries that can occur in the catheterization laboratory or the operating room. Surgical therapies for ischemic syndromes associated over the long term of these anomalies are presented herein.

Efficacy of desloratadine in persistent allergic rhinitis - a GA²LEN study.

BACKGROUND: The ARIA (Allergic Rhinitis and its Impact on Asthma) guidelines proposed a classification for allergic rhinitis based on the duration of symptoms (intermittent or persistent) rather than on the time of allergen exposure (seasonal or perennial). There had been no placebo-controlled, randomized, clinical trial of desloratadine (DL) in patients with persistent allergic rhinitis to date. OBJECTIVES: To assess the efficacy and safety of DL in patients with persistent allergic rhinitis based on the ARIA classification. METHODS: Patients 12 years of age and older with persistent allergic rhinitis were assessed over 85 days of treatment with DL 5 mg once daily (n = 360) or placebo (n = 356). The primary endpoint was the AM/PM reflective total 5-symptom score (T5SS) averaged over days 1-29. Secondary endpoints included AM/PM instantaneous T5SS and individual symptoms, therapeutic response, symptom severity assessed by a visual analogue scale and quality of life. RESULTS: The mean reduction in AM/PM reflective T5SS was significantly greater with DL than placebo over days 1-29 (-3.76 vs. -2.87, p < 0.001) and on each individual day (p < 0.05). The mean AM instantaneous T5SS was significantly reduced with DL compared with placebo as early as day 2 (-1.90 vs. -1.46; p < 0.001). The therapeutic response and improvement in quality of life were significantly greater with DL than placebo (p < 0.001 for each). The frequency of treatment-related adverse events was low and similar between DL (10.0%) and placebo (8.4%). CONCLUSIONS: This study showed DL to be effective and safe in the treatment of persistent allergic rhinitis.

The cost-effectiveness of treatment with desloratadine in patients with persistent allergic rhinitis.

OBJECTIVES: A new classification of persistent allergic rhinitis (PER) has been developed by the ARIA working group. Although the burden of AR is significant, treatment itself is also costly. It is unclear if treatment based on the new definition of PER is cost-effective. METHODS: The current study simulated the cost-effectiveness of desloratadine compared to placebo in the treatment of PER from the French societal perspective. Decision analysis was used to model the costs, effectiveness and cost-effectiveness over 12 months. Costs included medical expenditures (physician visits and prescription drugs) attributable to PER and related comorbidities and lost productivity due to absenteeism and presenteeism. Prices, tariffs and national wages were estimated from French national sources. MEASURES OF EFFECTIVENESS INCLUDED: symptom-based visual analogue scale (VAS), Rhinoconjunctivitis Quality of Life Questionnaire (RQLQ), Total 5 Symptoms Score (T5SS), categorical improvement in therapeutic response, interference with activities of daily living (ADL) and sleep outcomes. Mild or symptom-free days and 'responders' were also captured as outcomes. Univariate and second-order multivariate probabilistic sensitivity analyses were conducted. RESULTS: Treatment with desloratadine dominated placebo (cost less and resulted in greater effectiveness) for all measures of effectiveness. Of the individuals taking desloratadine 46.8% were classified as 'responders' vs. 34.8% for placebo (p = 0.0012). Individuals taking desloratadine experienced mild/no symptoms for 57.6% of study days vs. 36.5% for placebo (p = 0.002). The expected annual cost of treatment with desloratadine (1819 euro) was less than placebo (2618 euro). Lost productivity was the most significant contributor to total cost. Results of the 10,000 Monte Carlo simulations showed that treatment was cost-saving in 99.6% of simulations. CONCLUSIONS: Treatment of PER with desloratadine resulted in improved effectiveness and significant savings. While the cost of drug treatment is greater than that of no treatment, the downstream costs associated with not treating PER significantly outweigh the cost of treatment. Key limitations include the comparison of desloratadine to placebo and the sources of cost and effectiveness measures. Future studies should examine the cost-effectiveness of all available treatments for PER. In addition, many utilization, productivity and effectiveness measures were taken from clinical trials and may not accurately reflect 'real world' treatment patterns and outcomes.

Prenatal diagnosis and neonatal surgical management of a giant proximal right coronary artery to right ventricular fistula.

We report a case of prenatal diagnosis and early neonatal surgical repair of a large proximal right coronary artery to right ventricular fistula. The surgical findings and technical details of the reparative operation are discussed in the context of the differential diagnosis, which, in addition to coronary-cameral fistula, also includes aortoventricular tunnel and ruptured sinus of Valsalva aneurysm. Timely and appropriate diagnosis and surgical management resulted in preserved patency of the right coronary artery and restoration of normal right ventricular function.

Abdominal obesity, waist circumference, body mass index, and echocardiographic measures in children and adolescents.

BACKGROUND: Waist circumference (WC) may predict cardiovascular risk better than body mass index (BMI) in adults. The relationships between WC and echocardiographic measurements known to predict adult cardiovascular risk have not yet been explored in children. OBJECTIVE: This investigation sought to investigate whether WC predicts echocardiographic measures of cardiac structure and function and whether WC is a better predictor of these outcomes than BMI. If associations between WC and measures of diastolic function are found, are they due to increased left ventricular (LV) mass and whether WC and LV mass have synergistic or antagonistic effects on measures of diastolic function? METHODS: A total of 49 clinically normal children aged 3-19 years, including 17 with abdominal obesity, underwent detailed echocardiographic assessment to explore the associations between WC and various indices of systolic and diastolic function. Correlations, t-tests, and linear regressions were used for statistical testing. Results. Compared to subjects without abdominal obesity, those with abdominal obesity had increased left atrial dimensions, posterior wall thickness, and left ventricular (LV) mass index(2.7) (P < .05 for each comparison). Those with abdominal obesity also had altered LV filling patterns at the septal, lateral, and inferior wall by Doppler tissue imaging. WC was the sole predictor of, and was negatively associated with, the ratios of early peak velocity/late peak velocity at the septum, inferior wall, and right ventricle, and these associations were independent of LV mass (P < .001 for each comparison). Both BMI and WC were independent predictors of left ventricular mass index(2.7) (P= .001 and P= .05, respectively). CONCLUSIONS: Otherwise normal children and adolescents with abdominal obesity had altered left ventricular diastolic function. These differences were often better predicted by WC than by BMI. Our findings suggest the importance of considering WC in future studies of the cardiovascular impact of childhood obesity.

Anomalous aortic origin of a coronary artery with an interarterial course: understanding current management strategies in children and young adults.

Anomalous aortic origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course (AAOCA) is a rare congenital anomaly that is the second leading cardiovascular cause of sudden death in children and adolescents. Management practices with respect to this lesion vary among clinicians. We surveyed practitioners' opinions on treatment and management strategies for patients with AAOCA. A 17-question "Patient Management Questionnaire" was sent to all Congenital Heart Surgeons' Society (CHSS) members (n = 90), who were asked to respond and to distribute the questionnaire to pediatric cardiologists and nurse practitioners at their institutions. Questions addressed patient presentation, diagnostic modalities, therapeutic intervention, complications to therapy, follow-up visits, and type and frequency of follow-up testing. Descriptive statistics were used for analyses. We received 113 responses. Almost all respondents agree that ischemia at presentation or with provocative testing warrants surgical repair, whereas the majority believes that any patient with symptoms but no evidence of ischemia should have surgery. Approximately one-half and one-third of the respondents manage patients who are awaiting surgery or not having surgery by completely restricting exercise or not allowing competitive athletics, respectively. In asymptomatic patients, patient age determines timing of surgical repair but almost one-quarter did not believe that an asymptomatic child should undergo surgery. Among patients managed medically, there were six sudden deaths; there were two deaths in those who had surgical repair. There is marked heterogeneity in physician opinions regarding AAOCA treatment and management. A multicenter registry would facilitate the development of evidence-based treatment and management guidelines for children and young adults with AAOCA.