P263 Paradoxical embolus causing myocardial infarction in a young adult with congenital heart disease

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Veerappan ◽  
S Moharem-Elgamal ◽  
J Luszczak ◽  
N Manghat ◽  
S Lyen ◽  
...  

Abstract Case Report A 17 year old girl presented to her local hospital 24 hours after the onset of severe central chest pain that lasted for 30 minutes and spontaneously resolved. Her congenital heart disease background was of Ebstein’s anomaly of the tricuspid valve, coarctation of the aorta and ventricular septal defect (VSD). She underwent surgical repair of the coarctation and PA band at two months of age, followed by VSD closure when she was a year old.. An atrial communication was deliberately left open at the time of this operation and she had a known residual VSD and mild tricuspid regurgitation. Her resting saturations were 97% in air . On admission, she had a significantly raised Troponin T of 24,000ng/L with no significant ST segment elevation. An echocardiogram demonstrated new left ventricular (LV) systolic impairment with an ejection fraction of 33%. She underwent a ‘triple rule out’ cardiac CT scan and subsequently a cardiac MRI. On retrospective review, the CT demonstrated a sizeable atrial communication with left to right shunt, focal tight mid left anterior descending (LAD) artery stenosis aftersecond diagonal branch with mid to apical anterior and anteroseptal hypoperfusion. The MRI demonstrated extensive full thickness late gadolinium enhancement in mid to apical septum extending to the apical cap and apical inferior segment with microvascular obstruction (MVO) in the mid septum with possible VSD patch breakdown. No thrombi was notes on the gadolinium enhancement images. She was transferred to our hospital and underwent an invasive coronary angiogram that demonstrated recanalization of her coronary artery. She remained haemodynamically well throughout the admission. Anticoagulation with a direct acting oral anticoagulant (DOAC) was started alongside ACE-I and Beta blocker therapy. Discharge transthoracic echocardiogram showed flow across the atrial septum, a small residual VSD and mild LV dysfunction with akinesia of mid-to-distal LAD territory and very good function of remaining LV segments. She was discharged home with a plan for elective outpatient admission for a closure of her interatrial communication in a few months once she has recovered. Figure 1 summarises the key aspects of the multimodality imaging in this patient. Discussion Coronary embolus is the underlying cause of 3% of acute coronary syndromes with a small proportion being from a paradoxical embolus from the venous circulation through a right to left shunt across an atrial communication. Ebstein’s anomaly is a rare disorder, accounting for <1% of all congenital heart defects. Right-to-left shunt across the atrial communication is common in this condition due to RA dilatation and increased RA pressures secondary to tricuspid regurgitation. Our case illustrates the importance of having a high index of suspicion in patients with congenital heart disease for this rare complication of paradoxical coronary embolus, especially given the significant morbidity. Abstract P263 Figure 1

2020 ◽  
Vol 4 (2) ◽  
pp. 222-224
Author(s):  
Ashley Rider ◽  
Andrea Dreyfuss ◽  
Roberto Inga

Ebstein’s anomaly is a congenital heart defect that when left untreated can lead to unique physical exam and ultrasound findings. This case describes a patient who presented with dyspnea and was found to have cyanosis, clubbing, and dilation of right-sided chambers on point-of-care ultrasound. The series of images highlights findings in late-stage Ebstein’s anomaly and serves as a springboard for the discussion of the pathophysiology, diagnosis, and treatment of this rare congenital heart disease.


2021 ◽  
Author(s):  
Sato Kengo ◽  
Watanabe Ryosuke ◽  
Okada Tsuyoshi ◽  
Nishiyori Yasushi ◽  
Kobayashi Toshiyuki ◽  
...  

Abstract Background: Along with the improved life prognosis of patients with congenital heart disease, their diverse complications have come under scrutiny. Due to the various medical restrictions attendant on their upbringing, patients with congenital heart disease often have coexisting mental disorders. However, reports of patients with congenital heart disease and coexisting eating disorders are very rare. In this paper the authors report the case of a patient who developed anorexia nervosa following surgery for Ebstein’s anomaly.Case Presentation: A 21-year-old female with Ebstein’s anomaly who underwent a Fontan surgery was transferred to our institution with suspected anorexia nervosa after a more than 2-year intermittent stay in a medical hospital for decreased appetite. Initially, she did not express a desire to lose weight or a fear of obesity, and we suspected that she was suffering from appetite loss due to a physical condition associated with the Fontan circulation. But gradually the pathology of the eating disorder became apparent.Conclusions: Patients with congenital heart disease are more likely to have a psychological background as well as physical problems that might contribute to eating disorders. Indeed, it is unclear why there are not more cases of eating disorders in association with congenital heart diseases.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
H S A Abdelgawad ◽  
N Hisham ◽  
M Shehata ◽  
M A Abdelhay

Abstract Introduction Ebstein’s anomaly is characterized by displacement of the proximal attachments of the tricuspid valve from the atrioventricular ring into the right ventricle. This structural abnormality divides the right ventricle into an ‘atrialized’ portion and a distal ‘ventricularized’ portion. The severity is variable and accounts for the broad clinical spectrum, from severe disease causing fetal or neonatal death to mild disease compatible with natural survival as late as the eighth decade of life. Ebstein’s anomaly is an uncommon defect occurring in less than 1% of patients with congenital heart disease, but it is disproportionately represented in the adult congenital heart disease population because of its favourable natural history Case report A 55 year old man with no previous cardiac history .He presented to our medical facility complaining of acute retrosternal squeezing retrosternal chest pain few hours before admission. On clinical examination, he had a pansystolic murmur over the tricuspid area .ECG showed right bundle branch block. Laboratory results were unremarkable except elevated cardiac enzymes. 2D Transthoracic Echocardiography revealed a small well-functioning right ventricle, 15 mm/m2 apical displacement of the tricuspid septal leaflet with severe tricuspid regurgitation . 2D and 3D transesophageal echocardiography with intravenous agitated saline injection revealed patent foramen ovale with right to left shunt with immediate crossing of bubbles.Coronary angiography revealed normal coronaries. The patient refused intervention and he was maintained on warfarin treatment . Conclusion The clinical presentation of Ebstein’s anomaly is highly variable—depending on anatomic severity, haemodynamics ,and degree of interatrial shunting. The majority of patients have shunting through a secundum ASD or patent-foramen ovale. Paradoxical embolism maybe an indication for PFO/ ASD closure Abstract P843 Figure.


Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1215
Author(s):  
Stephan Ellmann ◽  
Julie-Marie Nickel ◽  
Rafael Heiss ◽  
Nouhayla El Amrani ◽  
Wolfgang Wüst ◽  
...  

For therapeutic decisions regarding uni- or biventricular surgical repair in congenital heart disease (CHD), left ventricular mass (LVM) is an important factor. The aim of this retrospective study was to determine the LVM of infants with CHD in thoracic computed tomography angiographies (CTAs) and to evaluate its usefulness as a prognostic parameter, with special attention paid to hypoplastic left heart (HLH) patients. Manual segmentation of the left ventricular endo- and epicardial volumes was performed in CTAs of 132 infants. LVMs were determined from these volumes and normalized to body surface area. LVMs of patients with different types of CHD were compared to each other using analyses of variances (ANOVA). An LVM cutoff for discrimination between uni- and biventricular repair was determined using receiver operating characteristics. Survival rates were calculated using Kaplan–Meier statistics. Patients with a clinical diagnosis of an HLH had significantly lower mean LVM (21.88 g/m2) compared to patients without applicable disease (50.22 g/m2; p < 0.0001) and compared to other CHDs, including persistent truncus arteriosus, left ventricular outflow tract obstruction, transposition of the great arteries, pulmonary artery stenosis or atresia, and double-outlet right ventricle (all, p < 0.05). The LVM cutoff for uni- vs. biventricular surgery was 33.9 g/m2 (sensitivity: 82.3%; specificity: 73.7%; PPV: 94.9%). In a subanalysis of HLH patients, a sensitivity of 50.0%, specificity of 100%, PPV of 100%, and NPV of 83.3% was determined. Patient survival was not significantly different between the surgical approaches or between patients with LVM above or below the cutoff. LVM can be measured in chest CTA of newborns with CHD and can be used as a prognostic factor.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Daniel Combs ◽  
Vanessa Fernandez ◽  
brent j barber ◽  
Wayne J Morgan ◽  
Chiu-Hsieh Hsu ◽  
...  

Introduction: Obstructive sleep apnea (OSA) is associated with cardiac dysfunction in children without congenital heart disease (CHD). Children with CHD are at increased risk for OSA and may be susceptible to further cardiovascular consequences due to OSA but the extent and nature of such cardiovascular effects of OSA are unknown. Methods: Children (6-17 years old) with corrected CHD without current cyanosis or Down syndrome were recruited from pediatric cardiology clinic. Home sleep tests were done to determine the presence and severity of OSA. OSA was defined as an obstructive apnea hypopnea index (oAHI) ≥1. Mild OSA was defined as an oAHI of ≥1 to <5 and moderate OSA was defined as an oAHI of ≥5 to <10. Standard clinically indicated echocardiograms were performed in clinic. Echocardiographic findings were compared between children with CHD with and without comorbid OSA using t-tests, Wilcoxon-sign rank tests as well as linear or logistic regression as appropriate. Results: Thirty-two children had sleep study and echocardiographic data available. OSA was present in 18 children (56%). OSA was mild in 89% and moderate in 11% of cases. There were no significant differences in age, body mass index, CHD severity, gender or ethnicity between children with and without OSA. Children with OSA had larger height-indexed right ventricular end-diastolic diameter (RVDi) compared to those without OSA (median 1.35, 95% CI 1.09, 1.56 vs. 1.21, 95% CI 1.01, 1.57; p=0.04). Children with moderate OSA had a reduced left ventricular shortening fraction compared to both those with mild OSA and no OSA (30.0 ± 6.1% vs. 38.7 ± 4.4%; p=0.009 and 39.2 ± 3.6%; p=0.007, respectively). Children with moderate OSA had increased left ventricular end-systolic diameter compared to those with mild OSA and no OSA (3.4 ± 0.4 cm vs. 2.5 ± 0.4; p=0.007 and 2.4 ± 0.5; p=0.001, respectively). Children with an RVDi above the median were seven times more likely to have OSA than those with an RVDi below the median (odds ratio 6.9.; 95% CI 1.3, 35; p=0.02). Conclusions: OSA is associated with changes in cardiac morphology and reduced contractility in children with CHD. Additionally, the presence of right ventricular dilation may suggest the need for OSA evaluation in children with CHD.


Circulation ◽  
1968 ◽  
Vol 37 (6) ◽  
pp. 879-889 ◽  
Author(s):  
M. M. JARMAKANI ◽  
SAM B. EDWARDS ◽  
MADISON S. SPACH ◽  
RAMON V. CANENT ◽  
M. PAUL CAPP ◽  
...  

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