Right Superior Vena Cava Connected Left Atrium with Partial Anomalous Pulmonary Venous Return and Intact Atrial Septum: An Unusual Cause of Paradoxical Embolism.

A six-month-old girl presented with history of convulsion and cyanosis, was eventually found to have a right superior vena cava connected to left atrium with anomalous upper right pulmonary vein return and without atrial septal defect. Diagnosis was confirmed by cardiac magnetic resonance imaging. The patient underwent a successful cardiac surgery correction, routing SVC to right atrium and repair of right upper pulmonary vein to left atrium.

Saudi Arabian Society of Echocardiography Recommendations for Echocardiography Service During Corona Virus Disease 2019 (COVID-19) Outbreak.

We are summarizing the recommendations for the use of Echocardiography in patients during COVID-19 pandemic. The patient risk for COVID-19 should be assessed according to the Saudi CDC guidelines. Echocardiography should only be performed of considered appropriate and will likely alter the clinical decision. In COVID-19 suspected/confirmed patients, echocardiography study should be performed bedside and in infection control approved area with airborne precaution. Limited focused imaging is recommended to minimize contact time. A dedicated machine for COVID-19 suspected/confirmed cases is recommended. Transesophageal echocardiography is considered an aerosol generating procedure; therefore, an alternative modality should be strongly considered. In COVID-19 suspected/confirmed patients, a transesophageal echocardiogram should be done only under strict airborne precaution. In low risk patient for COVID-19, Transesophageal echocardiography should be done with a minimum of droplet precaution, however; N95 respirator is preferred to surgical mask in this situation.

Non-invasive Cardiac Imaging During the Coronavirus Disease 2019 (COVID-19) Pandemic: A Statement from the Cardiac Imaging Working Group of the Saudi Heart Association.

COVID-19 pandemic has led to major changes in the delivery of medical care around the globe. Many investigations and elective procedures had to be rescheduled to decrease the risk of spreading the infection. Non-invasive cardiac imaging studies are requested to guide appropriate cardiac care in a variety of urgent, semi-urgent, and elective procedures. This position statement of the Cardiac Imaging Working Group of the Saudi Heart Association provides guidance into the protection of healthcare personnel, assessment of the indications of the imaging studies, and highlights consideration before, during, and after the study.

Discrete Left Ventricle Outflow Tract Obstruction in Children: Incidence and Predictors of Recurrence. A Multi-Center Study.

OBJECTIVES: The purpose of this study is to measure the incidence of recurrence of discrete subaortic stenosis (DSS) after primary resection in two major cardiac centers in Saudi Arabia and to identify risk factors associated with recurrence. METHODS: Data on 234 patients who were diagnosed with DSS and underwent surgical resection between 1999 and 2018 were retrospectively reviewed. Patient demographics as well as echocardiographic, surgical, and pathological data were compared between patients with recurrence and non-recurrence. RESULTS: The overall recurrence incidence after primary resection was 44.87% (N = 105). Most patients were male (59%). The median age at the 1st operation was 60 months (range 3 months to 133 months). The presence of aortic stenosis at the time of diagnosis was significantly associated with recurrence (p-value = 0.002). The overall median peak gradient in which the primary resection was indicated is 60 mmHg (range 11 to 152 mmHg). The median peak gradient pre-operation and post-operation were significantly higher for the recurrence group (p-value=0.018 and p<0.001, respectively). We used univariate and multivariate analysis and controlled for the follow-up time, but there were no significant independent predictors of recurrence. CONCLUSION: The recurrence rate of DSS after the primary resection is relatively high in this study. Further prospective studies are needed to draw a definite conclusion on risk factors for recurrence after primary resection.

Left ventricular mechanics after arterial switch operation: a speckle-tracking echocardiography study.

BACKGROUND: The arterial switch operation (ASO) is nowadays the standard procedure for the repair of dextro-transposition of the great arteries (d-TGA). Reduced exercise capacity, coronary artery abnormalities, and reversible myocardial perfusion defects have been demonstrated in patients who have undergone ASO. Despite this, indices of systolic function, assessed by standard echocardiography, are within the normal range. Speckle-tracking echocardiography (STE) can detect early subclinical myocardial abnormalities in several diseases even in the presence of normal left ventricular (LV) ejection fraction. AIM: To assess LV systolic myocardial deformation and torsion in asymptomatic ASO patients with normal LV ejection fraction (≥55%) by using STE. METHODS: We studied 62 asymptomatic patients (26 women) who have undergone single-stage ASO for simple d-TGA, aged 8.5 ±â€Š5.7 years, with a normal LV ejection fraction (≥55%); 31 age and sex comparable controls (14 women), aged 7.9 ±â€Š4.9 years. RESULTS: In patients who have undergone ASO, global LV longitudinal strain was significantly lower than that in controls (-19.2 ±â€Š2.9% vs. -22.7 ±â€Š2.4%, respectively, P < 0.0001). Longitudinal deformation was significantly impaired in the anterior and both anterior and posterior septal walls. In patients who have undergone ASO global circumferential strain and LV torsion were similar to controls. At multivariate analysis global LV longitudinal strain was significantly correlated only with age at surgery (P = 0.005). CONCLUSION: We demonstrated a significant reduction in longitudinal myocardial deformation correlated with the age at surgical repair, despite a normal LV ejection fraction, in the largest series of asymptomatic ASO patients by using STE. Our findings suggest early (≤7 days) operation on d-TGA patients and continued monitoring of ventricular function by STE.

Recessively inherited severe aortic aneurysm caused by mutated EFEMP2.

Familial aortic aneurysm (AA) is mostly inherited as an autosomal dominant disorder. However, recessively inherited AA has also been observed but in association with skin manifestations of cutis laxa, which is caused by a mutated EFEMP2 gene. In the present study, we recruited 9 patients, from 4 unrelated consanguineous families, with recessively inherited AA. The index cases, their parents, and siblings underwent clinical evaluation and cardiac imaging. In the affected subjects, the clinical presentation ranged from sweating and cyanosis at 3 months of age to incidental findings in an asymptomatic adult. The echocardiogram revealed a wide spectrum of severity of the AA, with a Z-score varying from 5 to 33. Intrafamilial variability was also evident; 2 unrelated subjects were detected at 17 and 20 years of age through family screening. The skin manifestations of cutis laxa were not found in any patient. In 1 family, genome-wide single-nucleotide polymorphism analysis detected a homozygous block, shared by 2 affected siblings, on chromosome 11 at q13. Sequence analysis of EFEMP2, located on chromosome 11 at q13, identified a novel homozygous mutation (p.E161K) in all 9 affected subjects. In this largest cohort of reported patients with a mutated EFEMP2 gene, we illustrate the phenotypic spectrum of inherited AA due to a novel EFEMP2 mutation. In conclusion, our work suggests that in families with apparently recessively inherited AA, molecular analysis of EFEMP2 gene might be warranted.

Pediatric poststernotomy mediastinitis.

BACKGROUND: Mediastinitis results in significant morbidity in pediatric cardiac patients. It is not clear whether delayed sternal closure is a risk factor for these infections. Management of mediastinitis remains controversial. METHODS: Cases of mediastinitis at the Stollery Children's Hospital from January 1, 1991, to June 30, 2004, were reviewed. RESULTS: There were 29 cases of mediastinitis in 2,675 open cardiac procedures for an overall incidence of 1.1%. Infection was diagnosed 5 to 27 days after the original surgical procedure (median, 10 days). The odds ratio for infection with delayed sternal closure versus primary sternal closure was 1.88 (95% confidence interval, 0.63 to 5.60). Signs at the onset of infection included fever (86%), incisional erythema (69%), purulent drainage from the incision or pacer wire sites (83%), and wound dehiscence (23%). Debridement was followed by primary sternal closure in all but three cases in which the sternum had not been closed before debridement and rotational muscle flaps were not used. Continuous irrigation systems were used only in the first 7 patients. One patient died of mediastinitis complicated by infective endocarditis, and 2 patients died of multiorgan failure. CONCLUSIONS: Delayed sternal closure was not a major risk factor for mediastinitis, especially if primary skin closure was used with delayed sternal closure. Excellent results were attained with debridement and primary closure of these infections.