An Unexpected Case of Isolated Left Ventricular Apical Hypoplasia in an Asymptomatic Patient

2020 ◽  
Vol 20 ◽  
Author(s):  
Rachel Anne Xuereb ◽  
Sara Xuereb ◽  
Tiziana Felice ◽  
Mariosa Xuereb ◽  
Alex Borg
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Asymptomatic Patient ◽  
Left Ventricular ◽  
Rare Type ◽  
Electrocardiogram Ecg ◽  
First Time

Isolated left ventricular (LV) apical hypoplasia is a rare type of congenital heart disease. It is often asymptomatic in childhood but may lead to complications later on in life. The proposed mechanism is inadequate LV dilatation during development of the primitive ventricle, resulting in a spherical LV. To our knowledge, we describe for the first time a case of isolated LV apical hypoplasia in an asymptomatic patient, diagnosed on investigation of an abnormal 12-lead resting electrocardiogram (ECG).

scholarly journals Prognostic Value of CTA-Derived Left Ventricular Mass in Neonates with Congenital Heart Disease

Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1215
Author(s):  
Stephan Ellmann ◽  
Julie-Marie Nickel ◽  
Rafael Heiss ◽  
Nouhayla El Amrani ◽  
Wolfgang Wüst ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricular Mass ◽  
Congenital Heart ◽  
Left Ventricular ◽  
Surgical Approaches ◽  
Pulmonary Artery Stenosis ◽  
Operating Characteristics ◽  
Ventricular Mass ◽  
Therapeutic Decisions

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.

Abstract 14771: Obstructive Sleep Apnea is Associated With Cardiac Dysfunction in Children With Congenital Heart Disease

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Daniel Combs ◽  
Vanessa Fernandez ◽  
brent j barber ◽  
Wayne J Morgan ◽  
Chiu-Hsieh Hsu ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Obstructive Sleep Apnea ◽  
Heart Disease ◽  
Sleep Apnea ◽  
Right Ventricular ◽  
Cardiac Dysfunction ◽  
Congenital Heart ◽  
Left Ventricular ◽  
Sleep Study ◽  
Obstructive Sleep

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.

scholarly journals Left Ventricular Pressure-Volume Characteristics in Congenital Heart Disease

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 ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Left Ventricular Pressure ◽  
Left Ventricular ◽  
Ventricular Pressure ◽  
Volume Characteristics

An adult female with 5q34-q35.2 deletion: A rare syndromic presentation of left ventricular non-compaction and congenital heart disease

2020 ◽  
Vol 63 (4) ◽  
pp. 103797
Author(s):  
Priyanka Arya ◽  
Theodore E. Wilson ◽  
John J. Parent ◽  
Stephanie M. Ware ◽  
Amy M. Breman ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Adult Female ◽  
Congenital Heart ◽  
Left Ventricular

The entirely subcutaneous ICDTM system in patients with congenital heart disease: experience from a large single-centre analysis

EP Europace ◽  
2019 ◽  
Vol 21 (10) ◽  
pp. 1537-1542 ◽  
Author(s):  
Kevin Willy ◽  
Florian Reinke ◽  
Nils Bögeholz ◽  
Julia Köbe ◽  
Lars Eckardt ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Single Centre ◽  
Icd Implantation ◽  
Shock Delivery ◽  
Ventricular Ejection Fraction

Abstract Aims The subcutaneous implantable cardioverter-defibrillator (S-ICDTM) is an important advance in device therapy for the prevention of sudden cardiac death (SCD). Although current guidelines recommend S-ICDTM use, long-term data are still limited, especially in subgroups such as adult patients with congenital heart diseases. This cohort is of high interest because of the difficult anatomic conditions in these patients. Methods and results All S-ICDTM patients with an underlying congenital heart disease (CHD) resulting in an indication for ICD implantation (n = 20 patients) in our large-scaled single-centre S-ICDTM registry (n = 249 patients) were included in this study. Baseline characteristics, appropriate and inappropriate shocks, and complications were documented in a mean follow-up of 36 months. Primary prevention of SCD was the indication for implantation of an S-ICDTM in six patients (30%). Of all 20 patients with an overall mean age of 40.5 ± 11.5 years, 12 were male (60%). The mean left ventricular ejection fraction was 46.5 ± 11.3%. Nine episodes of ventricular tachycardia (two monomorphic and seven polymorphic) were adequately terminated in three patients (15%). In two patients, T-Wave-Oversensing resulting in an inappropriate shock was observed, which could be managed by changing the sensing vector or activation of the SMART PASSTM filter. There were no S-ICDTM system-related infections. In one patient, surgical revision was necessary due to a persistent haematoma. Conclusion The S-ICDTM seems to be a valuable option for the prevention of SCD in patients with various CHDs and complex anatomical anomalies. The S-ICDTM is safe and works effectively, also in these complex patients. Inadequate shock delivery was rare and could be managed by reprogramming.

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