BackgroundCoronary reimplantation is used in therapy for congenital heart disease, such as in the arterial switch (ASO) and Ross operations. The adequacy of myocardial perfusion may remain a matter of concern. The aim of the present study was to stratify the effect of coronary reimplantation on myocardial perfusion and to highlight the clinical relevance of any attenuation in myocardial perfusion. Methods and ResultsA total of 21 children with transposition of the great arteries at a mean interval of 11.2±2.9 years after ASO and 9 adolescents at a mean interval of 4.2±2.1 years after the Ross procedure were investigated. All patients were asymptomatic and had a normal exercise capacity. On stress echocardiography, 2 of the ASO patients had dyskinetic areas within the left ventricular myocardium, and 5 had adenosine-induced perfusion defects on positron emission tomography. No coronary obstruction was detected on coronary angiography in any patient, but a common finding was right coronary dominance and a small caliber of the distal part of the left anterior descending artery. Coronary flow reserve (CFR) was significantly reduced in all patients after ASO when compared with 10 normal healthy volunteers (age, 25.6±5.3 years). CFR was normal in the 9 patients who had the Ross operation (age, 19.2±7.6 years); exercise-induced perfusion defects were not detected in the Ross patients. ConclusionsChildren after ASO are asymptomatic, without clinical signs of coronary dysfunction. In contrast to patients who had the Ross operation, stress-induced perfusion defects and an attenuated CFR were documented. The prognostic implications of these findings and the clinical consequences are unclear; nevertheless, close clinical follow-up of ASO patients is mandatory.

Congenital Heart Disease AbstractsMyocardial blood flow and flow reserve after coronary reimplantation in patients after arterial switch and Ross operation

Background: Left ventricular noncompaction cardiomyopathy (LVNC) is associated with poor clinical outcome in childhood. Standard diagnostic criteria are still controversial, especially in young pat...

https://www.ahajournals.org/doi/pdf/10.1161/CIRCIMAGING.118.007805

Left Ventricular Twist Mechanics to Identify Left Ventricular Noncompaction in Childhood.

https://www.heartrhythmjournal.com/article/S1547-5271(15)00802-4/pdf

Outcome in young patients with isolated complete atrioventricular block and permanent pacemaker treatment: A nationwide study of 127 patients

Strain Echocardiography and Myocardial Mechanics: From Basics to Clinical Applications.

Heart failure (HF) is a complex clinical syndrome in which structural/functional myocardial abnormalities result in symptoms and signs of hypoperfusion and/or pulmonary or systemic congestion at rest or during exercise. More than 80% of deaths in patients with HF recognize a cardiovascular cause, with most being either sudden cardiac death (SCD) or death caused by progressive pump failure. Risk stratification of SCD in patients with HF and preserved (HFpEF) or reduced ejection fraction (HFrEF) represents a clinical challenge. This review will give an update of current strategies for SCD risk stratification in both HFrEF and HFpEF.

https://www.mdpi.com/2077-0383/7/11/436/pdf

Risk Stratification of Sudden Cardiac Death in Patients with Heart Failure: An update.

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 ± 5.7 years, with a normal LV ejection fraction (≥55%); 31 age and sex comparable controls (14 women), aged 7.9 ± 4.9 years. RESULTS In patients who have undergone ASO, global LV longitudinal strain was significantly lower than that in controls (-19.2 ± 2.9% vs. -22.7 ± 2.4%, 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.

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

BACKGROUND: Right ventricular pacing has been the treatment of choice in patients with congenital complete atrioventricular block (CAVB). However, the effect of chronic right ventricular pacing on left ventricular function in young patients is still controversial. AIM: The aim of the study was to assess the change in left ventricular systolic function in young patients (age ≤20 years) paced for isolated CAVB and to identify possible predictors of left ventricular systolic dysfunction. METHODS: We studied 55 young patients who underwent permanent right ventricular pacemaker implantation for CAVB in the absence of significant structural heart disease. We excluded patients affected by any condition known to affect left ventricular function. Echocardiographic data prior to and after pacemaker implantation were obtained. RESULTS: The mean age at the time of pacemaker implantation was 20 months, range 2.3-72 months. The mean duration of follow-up was 94.86 (range: 2-268 months). Chronic right ventricular pacing affected left ventricular shortening fraction (LVSF) significantly (pre = 37.8 ± 7.8 vs. post = 32.8 ± 5.5%, P = 0.0036). In 14 patients (25.4%), LVSF decreased by at least 7% (group A). The only parameter studied able to significantly discriminate the two groups was a better baseline LVSF in group A (baseline LVSF: 42.1 ± 5.2 vs. 32.2 ± 2.2%, P = 0.019; cut-off value >39%, P <0.0001; area under the curve = 0.887). CONCLUSION: Chronic right ventricular pacing in young patients without significant structural heart disease is responsible for a significant reduction in left ventricular systolic function, especially in patients with a good baseline LVSF. These patients need close follow-up not only for pacing parameters but also for left ventricular functional evaluation.

Left ventricular function and right ventricular pacing for isolated congenital heart block.

Tissue Doppler and deformation imaging, including Doppler-derived strain and speckle tracking, have significantly improved our understanding of cardiac mechanics in both physiological and pathological states. The various modes of left ventricular deformation (longitudinal, circumferential, radial and twist) leading to systolic contraction can nowadays be quantified. One of the best applications of deformation imaging is in the area of hypertrophic cardiomyopathies. Deformation imaging allows the evaluation of global and regional myocardial performance and the noninvasive characterization of abnormal intramural myocardial mechanics. In this review, we discuss the role of myocardial deformation imaging derived by echocardiography in the assessment of rare hypertrophic phenotype including Fabry disease, Friedreich ataxia and amyloidosis. Deformation imaging allows for early identification of myocardial dysfunction in many hypertrophic disorders, at an earlier stage than that provided by standard imaging or echocardiographic techniques. This allows for the implementation of appropriate therapy before significant disease progression has occurred and prior to the development of advanced myocardial fibrosis. Thus therapy would likely be more effective and may potentially lead to improvement in patient outcome. Additionally strain imaging allows to better monitoring the efficacy of therapy by assessing the progression and regression of myocardial involvement. Finally, findings on strain imaging carry important prognostic information in many hypertrophic disorders.

/pdf/myocardial-deformation-imaging-and-rare-cardiomyopathies-3o2bydk0zj.pdf

Myocardial Deformation Imaging and Rare Cardiomyopathies with Hypertrophic Phenotype: A Review Focused on Fabry Disease, Friedreich Ataxia and Amyloidosis

Aneurysm of the left atrial appendage is a rare pathological condition, especially during infancy. We describe the case of a 4-month-old child with a giant congenital aneurysm of the left atrial appendage.

Ziad Al Bulbul

Papers

Strain Echocardiography and Myocardial Mechanics: From Basics to Clinical Applications.

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

Left ventricular function and right ventricular pacing for isolated congenital heart block.

Myocardial Deformation Imaging and Rare Cardiomyopathies with Hypertrophic Phenotype: A Review Focused on Fabry Disease, Friedreich Ataxia and Amyloidosis

A rare case of giant congenital left atrial appendage aneurysm in a 4-month-old child.