Rare coexistence: type A aortic interruption and left ventricular non-compaction

Left ventricular non-compaction (LVNC) is an anomaly characterized by deep trabeculations within the left ventricular cavity. This anomaly, described by Chin et al. [1]. in 1990, was added to the cardiomyopathy classification in 2006. Aortic interruption (AI), first described by Steidele in 1778, occurs in one in three million births [2]. The coexistence of these two rare anomalies is much less common.
A 48-year-old male patient was admitted to the cardiology polyclinic with a feeling of pressure in the chest area. There was no disease in his background. On physical examination, the patient’s blood pressure measured 145/90 mm Hg and there was a systolic murmur with no spread of 1–2/6 on the aortic focus. Electrocardiography (ECG) was normal. An exercise ECG test was requested and interpreted as positive. Evaluation of the echocardiography revealed that the ejection fraction was 55%, and hypertrabeculation was observed in the left ventricle. The non-compacted layer was 2.2 cm and the compacted layer was 0.8 cm (Figure 1 A). A stable angina diagnosis was considered and the patient was transferred to the coronary angiography (CAG) laboratory. The right femoral region was punctured, and the guide wire could not pass into the distal section of the descending aorta. A simultaneous right radial approach was performed. In angiography, non-critical lesions were observed in the coronary arteries. Hypertrabeculation was observed in the left ventriculography (Figure 1 B). A pigtail catheter was placed in the aorta from the radial route and descending from the femoral route. Simultaneous aortography was performed where the descending aorta discontinued before the exit of the subclavian artery, accompanied by a giant collateral vessel (Figure 1 C). The discontinuity at the descending aorta and giant collateral artery was confirmed with three dimensional computed tomography angiography (Figure 1 D). Type A AI and LVNC were diagnosed and the patient was discharged with suggestions of medical treatment.
Left ventricular non-compaction and AI are very rare anomalies individually. Left ventricular non-compaction is a known reason for cardiomyopathy. If not diagnosed at an early stage, the patient could present with cardiac insufficiency symptoms and the patient could be lost at an early stage. Some morphological diagnostic criteria have been accepted for LVNC with echocardiography. Compacted epicardial bands and thicker non-compacted endocardial layers have been...


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