To retrospectively assess the suitability of pulmonary artery banding as a treatment strategy for dilated cardiomyopathy and left ventricular non-compaction cardiomyopathy with depressed left ventricular ejection fraction.

The study was retrospective and included consecutive patients who met the inclusion criteria: diagnosed with dilated cardiomyopathy or left ventricular non-compaction cardiomyopathy and left ventricular ejection fraction less than 35%. Cardiac indices were documented, and clinical outcomes were followed for 5 years.

This study included 21 patients with depressed left ventricular ejection fraction due to dilated cardiomyopathy (n = 11) or left ventricular non-compaction cardiomyopathy (n = 10), treated either with anti-congestion medication alone or in combination with pulmonary artery banding. The groups treated with pulmonary artery banding showed significant improvement in left ventricular ejection fraction compared to controls (ANOVA, p = 0.0002), with no major adverse events. In the subgroup with left ventricular non-compaction, pulmonary artery banding led to significant improvement of the left ventricular ejection fraction (p = 0.00002) and significant reductions in the Z scores of left ventricular end-diastolic diameter (p = 0.0002) and of end-diastolic volume (p = 0.004).

Pulmonary artery banding appears to be a viable strategy for improving heart function in patients with non-compaction and dilated cardiomyopathy and depressed left ventricular ejection fraction. While pulmonary artery banding demonstrated more pronounced benefits in the subgroup with non-compaction cardiomyopathy, significantly enhancing cardiac restoration indices throughout the follow-up period, warranting further investigation in larger studies.

Paediatric cardiomyopathy is a progressive, often lethal disorder and the most common cause of heart failure in children. Despite its severe outcomes, the genetic aetiology is still poorly characterised. High-throughput sequencing offers a great opportunity for a better understanding of the genetic causes of cardiomyopathy.

The current study aimed to elucidate the genetic background of cardiomyopathy in Egyptian children.

This hospital-based study involved 68 patients; 58 idiopathic primary dilated cardiomyopathy and 10 left ventricular noncompaction cardiomyopathy. Cardiomyopathy-associated genes were investigated using targeted next-generation sequencing.

Consanguinity was positive in 53 and 70% of dilated cardiomyopathy and left ventricular noncompaction cardiomyopathy patients, respectively. Positive family history of cardiomyopathy was present in 28% of dilated cardiomyopathy and 10% of the left ventricular noncompaction cardiomyopathy patients. In 25 patients, 29 rare variants were detected; 2 likely pathogenic variants in TNNI3 and TTN and 27 variants of uncertain significance explaining 2.9% of patients.

The low genetic detection rate suggests that novel genes or variants might underlie paediatric cardiomyopathy in Egypt, especially with the high burden of consanguinity. Being the first national and regional report, our study could be a reference for future genetic testing in Egyptian cardiomyopathy children. Genome-wide tests (whole exome/genome sequencing) might be more suitable than the targeted sequencing to investigate the primary cardiomyopathy patients. Molecular characterisation of cardiomyopathies in different ethnicities will allow for global comparative studies that could result in understanding the pathophysiology and heterogeneity of cardiomyopathies.