(Circulation 1990;82:507-513) In the early embryo, the heart is a loose interwoven mesh of muscle fibers.1-3 The developing myocardium gradually condenses, and the large spaces within the trabecular meshwork flatten or disappear. Trabecular compaction is normally more complete in left ventricular than in right ventricular myocardium. Noncompaction of ventricular myocardium (sometimes referred to as "spongy myocardium") is believed to represent an arrest in endomyocardial morphogenesis.4,5 The gross anatomical appearance is characterized by numerous, excessively prominent trabeculations and deep intertrabecular recesses. Rare in any case, noncompaction is almost invariably associated with other congenital cardiac malformations.45 Isolated noncompaction of left ventricular myocardium (INVM) (i.e., without associated anomalies) is rarer still.5-7 This report represents the largest study population to date.

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A total of 130 cases of pulmonary vascular sling, including seven new cases in our collection, were studied. The sex distribution was 60% male and 40% female. The age at presentation was in the first year of life in approximately 90% of cases. Barium-esophagraphy showing anterior indentation was diagnostic in most of the cases. Bronchoscopy and tracheobronchography were useful in detecting associated tracheobronchial anomalies preoperatively. The analysis of 68 autopsied cases revealed associated tracheobronchial anomalies in 40% of the cases. Anomalies of the tracheobronchial tree took three major forms: abnormal distribution of cartilage in the walls of the trachea and major bronchi, intrinsic stenosis, and abnormal branching, the latter being that of bronchus suis. Acquired changes secondary in the sling resulted in compression of the major respiratory pathway by the anomalous left pulmonary artery. Major associated cardiovascular anomalies were present in 30% of the cases. These were represented by ventricular septal defect, atrial septal defect, patent ductus arteriosus, tetralogy of Fallot, common ventricle, and coarctation of the aorta.

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