Noncompaction cardiomyopathy natural history

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.

Associated Congenital Abnormalities

 * Abnormalities of the origin of the left coronary artery
 * Pulmonary atresia
 * Pulmonary stenosis
 * Right or Left ventricle obstruction
 * Hypoplastic left ventricle
 * Mitral regurgitation

Left Ventricular Dysfunction

 * Systolic dysfunction
 * Diastolic dysfunction
 * Restrictive filling patterns
 * Subendocardial and microvascular ischemia

Arrhythmias And Conduction System Abnormalities

 * Wolff-Parkinson-White syndrome
 * Complete heart block
 * Atrial fibrillation
 * Tachyarrhythmias and sudden cardiac death

Thrombotic complications

 * Thrombus formation in the left ventricle

Neurologic Complications

 * Becker's muscular dystrophy
 * Mitochondrial myopathy
 * Polyneuropathy and metabolic myopathy

Associated Genetic Disorders

 * Emery-Dreifuss muscular dystrophy
 * Myotubular cardiomyopathy
 * Barth syndrome

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. In another study, 60% of patients had either died or undergone cardiac transplantation within six years. In another study with 10 years of follow-up, 90% of patients had developed left ventricular dysfunction.

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

Recommendations Regarding Pregnancy in the Patient With Non-Compaction Cardiomyopathy
If severe heart failure is present, pregnancy is not recommended.