Herein, we present a patient with LVNC concomitant with ventricular septal defect (VSD), who was successfully treated surgically via cardiopulmonary bypass (CPB). To our knowledge, this is the first case of LVNC concomitant with VSD that was surgically corrected by open heart surgery.
Ventricular noncompaction may occur in isolation (isolated ventricular noncompaction) or may be associated with other congenital cardiac malformations.[6],[7] Echocardiography, magnetic resonance imaging, and angiography are the main imaging tools to diagnose ventricular noncompaction, but two-dimensional color Doppler echocardiography is the standard and first-line diagnostic tool. Despite an increasing awareness, there is still little knowledge regarding the diagnosis of this rare cardiomyopathy. The diagnosis of LVNC may easily be overlooked or delayed in particular cases presenting with heart failure due to depressed ventricular function. Delay in diagnosis is due to similarities between LVNC and other cardiomyopathies, and to the lack of familiarity of the referring physicians with its specific diagnostic pattern.
This rare abnormality is reported to be accompanied by three major conditions, namely, depressed ventricular function, systemic embolization, and ventricular arrhythmias that may sometimes be fatal. Because depressed ventricular function is diagnosed in the late period, patients are sometimes referred to clinics with the complaint of heart failure. Noncompaction primarily affects the left ventricle, but the right ventricle may also be involved.[4],[8],[9] Asymptomatic cases have normal systolic function and increased left ventricular enddiastolic pressure, mimicking restrictive cardiomyopathy. Most patients are initially asymptomatic,[10] though there are symptomatic cases.[1]
Similarities between LVNC and other cardiomyopathies sometimes make the differential diagnosis difficult. Familiarity of cardiologists with the pathology ensure early and correct diagnosis. Prominent multiple myocardial trabeculations and intertrabecular spaces within this trabecular structure which continue to the ventricular cavity are typical echocardiographic and angiocardiographic findings of LVNC.
The treatment of myocardial noncompaction is not different from that of other cardiomyopathies. Medical treatment is preferred at the beginning of congestive heart failure. Diuretics, angiotensin-converting enzyme inhibitors, and digitalis are preferred for medical treatment in patients with noncompaction of ventricular myocardium. In our case, medical treatment was not started because the patient had no symptoms of the myocardial failure during the preoperative and postoperative period. Transthoracic echocardiography, which shows clear and valuable data about systolic and diastolic functions, was performed periodically to monitor the status of myocardial performance.
To the best of our knowledge, no surgically treated case of LVNC combined with VSD has been reported previously. There is only one case report from our institution, in which congenitally corrected transposition of the great arteries accompanied by noncompaction of both ventricles was treated surgically under CPB.[2]
In conclusion, patients with VSD may have ventricular noncompaction without any manifestation of cardiomyopathy. Surgical correction should be performed in a single session in the early period of life. In these cases, progressive myocardial insufficiency may complicate the clinical condition, and may cause accelerated clinical deterioration. In addition, noncompaction should be suspected and investigated whenever ventricular function is depressed irrelevant to the accompanying heart defect, and if detected, appropriate medical management should be initiated to improve the myocardial performance in the early period of myocardial failure.