Isolated left ventricular noncompaction cardiomyopathy (ILVNC) is a rare genetically heterogeneous condition characterized by hypertrabeculation and sponge-like appearance of the left ventricular myocardium (Fig. 1). ILVNC can be congenital in nature or acquired later in adult life. In addition, sporadic occurrence and familial transmission have been described.1–3
In the early intrauterine period, the myocardium exists as a loose meshwork. By the 18th week of life, coronary vasculature is established and the loose myocardial meshwork compacts. This process is responsible for the relatively smooth appearance of the normal left ventricular wall (Fig. 2). The embryogenic hypothesis suggests that arrest of this process causes ILVNC (see Video, Supplemental Digital Content, http://links.lww.com/RCA/A852). The nonembryogenic hypothesis proposes that chronic changes in left ventricular loading conditions leads to hypertrabeculation.1,2
Although children and adults present similarly with heart failure, arrhythmias, thromboembolic phenomenon, or sudden cardiac death, patients with the acquired form of ILVNC may be largely asymptomatic as children. Currently, the true prevalence of ILVNC is unknown. Males are affected more than females.1,2
Definitive diagnosis of this condition relies on cardiac resonance imaging. Echocardiographic diagnosis requires that the ratio of left ventricular noncompacted to compacted myocardium at end-systole in the parasternal short axis view exceeds 2 (Fig. 3).3,4
Women with ILVNC are at a high risk of developing decompensated heart failure during pregnancy. Cardiovascular changes in pregnancy including tachycardia, intravascular volume expansion, and anemia exacerbate heart failure. Vaginal delivery with early institution of epidural analgesia is preferred in stable patients. Refractory heart failure with hemodynamic instability necessitates cesarean delivery under general anesthesia. Cesarean delivery is preferably conducted in cardiac surgical operating rooms lest extracorporeal life support is needed for refractory hypotension. Preinduction arterial line and central venous cannulation is prudent. Inotropic and vasopressor support may be necessary at or soon after induction.5,6
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The authors declare not having received funding for the preparation of this article.
Conflicts of interest
The authors declare having no conflict of interest to disclose.
1. Sasse-Klaassen S, Gerull B, Oechslin E, et al Isolated noncompaction of the left ventricular myocardium in the adult is an autosomal dominant disorder in the majority of patients. Am J Med Genet 2003;119A:162–167.
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