Noncompaction cardiomyopathy natural history
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
The presence of NCC is associated with variable degrees of congestive heart failure and may or may not be associated with congenital heart disease or diseases in other organs [1][2][3].
Complications, Involvement of Other Organs and Associated Disorders
Cardiac Disease
- Abnormalities of the origin of the left coronary artery
- Pulmonary atresia
- Pulmonary stenosis
- Right or Left ventricle obstruction
- Hypoplastic left ventricle
- Mitral regurgitation
- Tachyarrhythmias and sudden cardiac death
- Thrombus formation in the left ventricle
Neurologic Complications
Associated Genetic Disorders
Prognosis
In so far as noncompaction cardiomyopathy is a recently described entity, its association with long term clinical outcomes is not weel defined. In a study which documented the long term follow up of 34 patients who were symptomatic with NCC, 35% had died at the age of 42 +/- 40 months. 12% of the patients required cardiac transplantation due to heart failure[4] . In another study, 60% of patients had either died or undergone cardiac transplantation within six years[5]. In another study with 10 years of follow-up, 90% of patients had developed left ventricular dysfunction[6].
The prognosis associated with NCC may be better than that reported in these series because only symptomatic patients were included in the aforementioned studies. Genetic testing reveals that there are patients with lesser degrees of symptoms, and the long-term prognosis in these patients has not been well defined.
High Risk Clinical Features
Clinical features of patients who are at an increased risk of poor long-term mortality in whom consideration should be given to implantation of an automatic implantable cardiac defibrillator (AICD) and/or cardiac transplantation include:
- Higher left ventricular end diastolic diameter presentation
- New York heart Association class III or IV heart failure
- Permanent or persistent atrial fibrillation
- Presence of bundle branch block
References
- ↑ Ritter M, Oechslin E, Sutsch G, et al. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997; 72: 26–31.
- ↑ Dusek J, Ostadal B, Duskova M. Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol. 1975; 99: 312–317.
- ↑ Lauer RM, Fink HP, Petry EL, et al. Angiographic demonstration of intramyocardial sinusoids in pulmonary-valve atresia with intact ventricular septum and hypoplastic right ventricle. N Engl J Med. 1964; 271: 68–72.
- ↑ Oechslin, Erwin; Jenni, Rolf (2005). "Non-compaction of the Left Ventricular Myocardium - From Clinical Observation to the Discovery of a New Disease" (webpage).
- ↑ Ritter M, Oechslin E, Sutsch G, et al. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997; 72: 26–31.
- ↑ Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular myocardium: a study of eight cases. Circulation.1990; 82: 507–513.