Effect of Noncompacted Myocardial Resection on Isolated Left Ventricular Noncompaction.

Isolated left ventricular noncompaction, where broad trabeculae and deep intertrabecular recesses are observed in the left ventricular myocardium resulting from an arrest in normal embryogenesis, is a rare cardiomyopathy. We present a report on isolated trabeculectomy and postoperative echocardiographic follow-up showing recovery of cardiac function for isolated left ventricular noncompaction.

Catheter ablation of a right posterior accessory pathway in a patient with left ventricular noncompaction: A case report.

RTIONALE: Left ventricular noncompaction (LVNC) is a genetic cardiomyopathy characterized by the presence of a thin compacted layer of myocardium and a spongy subendocardial layer with trabeculations and recesses. LVNC associated Wolf-Parkinson-White syndrome is very rare. PATIENT CONCERNS: A 32-year-old male presented with short episodes of palpitations and a syncope 6 months before his hospitalization. DIAGNOSIS: His ECG revealed the presence of a right posterior accessory pathway. Echocardiography identified trabeculations of the septal, apical, and lateral wall of the left ventricle, consistent with left ventricular noncompaction. Cardiac MRI confirmed the diagnosis, as the ratio between the noncompacted and compacted myocardial layer was 2.3. INTERVENTIONS: The electrophysiological study revealed a malignant right posterior accessory pathway. Catheter ablation was successfully performed at the level of posterior tricuspid annulus. Programmed ventricular stimulation could not induce any arrhythmia at the end of the procedure. OUTCOMES: During 15 months of follow-up, the patient presented no more episodes of palpitations or syncope. LESSONS: Left ventricular noncompaction with right accessory pathway is a rare association with genetic basis and gives a higher risk of sudden cardiac death. Catheter ablation of the accessory pathway is a valuable way of treatment in this category of patients, lowering the risk of sudden cardiac death.

Aortic root replacement in a patient with left ventricular noncompaction.

We describe the case of a 57-year-old woman with noncompaction of the left ventricle and regurgitant bicuspid aortic valve who presented with progressive congestive heart failure and was successfully treated with aortic root replacement. The long-term outcome for these patients is poor because of progressive left ventricular impairment, increased rates of life-threatening arrhythmias, and intraventricular thrombi. To our knowledge, only 3 patient with noncompaction of the left ventricle has been reported to have undergone aortic valve replacement for severely regurgitant bicuspid aortic valve. Herein, we describe a patient with noncompaction of the left ventricle who underwent successful mechanical aortic root replacement.

Left ventricular noncompaction complicated by mitral valve prolapse: report of a case.

A 31-year-old male was diagnosed with congestive heart failure due to left ventricular noncompaction and coarctation of the aorta by echocardiography and computed tomography. He also developed mitral valve prolapse of the posteromedial commissural leaflet due to rupture of chordae tendineae. Segmental leaflet resection was performed with ring annuloplasty. Aorto-bifemoral bypass was carried out simultaneously to attenuate the ventricular afterload. His postoperative course was uneventful.

Videoscopic left cardiac sympathetic denervation for patients with recurrent ventricular fibrillation/malignant ventricular arrhythmia syndromes besides congenital long-QT syndrome.

BACKGROUND: Treatment options for patients with recurrent ventricular arrhythmias refractory to pharmacotherapy and ablation are minimal. Although left cardiac sympathetic denervation (LCSD) is well established in long-QT syndrome, its role in non-long-QT syndrome arrhythmogenic channelopathies and cardiomyopathies is less clear. Here, we report our single-center experience in performing LCSD in this setting. METHODS AND RESULTS: In this institutional review board-approved study, we retrospectively reviewed the electronic medical records of all patients (N=91) who had videoscopic LCSD at our institution from 2005 to 2011. Data were analyzed for the subset (n=27) who were denervated for an underlying diagnosis other than autosomal dominant or sporadic long-QT syndrome. The spectrum of arrhythmogenic disease included catecholaminergic polymorphic ventricular tachycardia (n=13), Jervell and Lange-Nielsen syndrome (n=5), idiopathic ventricular fibrillation (n=4), left ventricular noncompaction (n=2), hypertrophic cardiomyopathy (n=1), ischemic cardiomyopathy (n=1), and arrhythmogenic right ventricular cardiomyopathy (n=1). Five patients had LCSD because of high-risk assessment and ß-blocker intolerance, none of whom had a sentinel breakthrough cardiac event at early follow-up. Among the remaining 22 previously symptomatic patients who had LCSD as secondary prevention, all had an attenuation in cardiac events, with 18 having no breakthrough cardiac events so far and 4 having experienced ≥1 post-LCSD breakthrough cardiac event. CONCLUSIONS: LCSD may represent a substrate-independent antifibrillatory treatment option for patients with life-threatening ventricular arrhythmia syndromes other than long-QT syndrome. The early follow-up seems promising, with a marked reduction in the frequency of cardiac events postdenervation.

Long-term myocardial recovery after mitral valve replacement in noncompaction cardiomyopathy.

Isolated noncompaction of the left ventricle is a congenital cardiomyopathy, which has been described recently, with literature limited to case reports and case series. Even though various complications have been reported with noncompaction cardiomyopathy, among them severe mitral regurgitation has been reported recently in a few cases. There is no great evidence in the literature about its management, apart from some cases of mitral valve repair and replacement in young patients. We are reporting a case of an elderly lady with isolated left ventricular noncompaction cardiomyopathy associated with severe mitral regurgitation treated with mitral valve replacement with one and half year of follow up demonstrating significant myocardial recovery.

Pentalogy of Cantrell with a single-ventricle cardiac defect: collaborative management of a complex disease.

We present a case of ectopia cordis with a complex single-ventricle congenital heart defect in association with pentalogy of Cantrell. Management by a skilled multidisciplinary team was critical for patient survival. Early fetal diagnosis and the use of advanced imaging techniques allowed adequate time for planning and identified critical anatomic details. Preserving the heart's natural covering, performing cardiac surgery without cardiopulmonary bypass, and using catheter intervention decreased the risk to the patient. Complete coverage of the defect was achieved using skin generated with tissue expanders. This case illustrates the importance of collaboration when caring for infants with critical, high-risk disease and highlights the increased potential for survival with complex ectopia cordis in the current medical era.

Arrhythmogenic left ventricular cardiomyopathy associated with noncompaction.

A 46-year-old woman was admitted to our hospital because of progressive exertional dyspnea and occasional premature ventricular contraction. An enhanced computed tomographic scan revealed partial defect of the left ventricular myocardium and prominent trabecular meshwork at the same thin-wall segment. She underwent resection and endoventricular patch plasty using cardiopulmonary bypass. Histopathologic examination showed transmural fibro-fatty replacement of the myocardium, with an extremely thickened endocardium. Here we report an extremely rare case of surgery in a patient with arrhythmogenic left ventricular cardiomyopathy associated with left ventricular noncompaction.

Sustainable myocardial recovery after mitral reconstruction for left ventricular noncompaction.

Noncompaction of the left ventricle is a rare congenital cardiomyopathy characterized by prematurely arrested compaction of the endocardial and myocardial fibers. Although systolic heart failure is assured, treatment has been traditionally limited to palliative medical therapy or transplantation. We present the first report of long-term clinical and echocardiographic follow-up (50 months) showing sustained myocardial recovery after the use of mitral valve annuloplasty as the primary treatment for severe mitral regurgitation associated with left ventricular noncompaction.

Insuficiência cardíaca refratária em um paciente portador de miocárdio não compactado / Refractory heart failure in a patient with non-compacted myocardium / Insuficiencia cardiaca refractaria en un paciente portador de miocardio no compactado

A não compactação do miocárdio caracteriza-se por excessiva trabeculação e recessos dos ventrículos (usualmente o ventrículo esquerdo), devido à interrupção do processo de compactação das fibras miocárdicas durante a fase embriogênica. Essa anormalidade cursa frequentemente com insuficiência cardíaca, fenômenos tromboembólicos e arritmias cardíacas, conferindo mau prognóstico. Neste artigo descrevemos um caso de um rapaz de 26 anos com insuficiência cardíaca refratária devido a não compactação isolada do miocárdio ventricular esquerdo, que necessitou de transplante cardíaco.

The non-compacted myocardium is characterized by excessive trabeculation and ventricular recesses (usually of the left ventricle) due to the interruption of the myocardial fiber compaction during embryogenesis. This abnormality usually courses with heart failure, thromboembolic phenomena and cardiac arrhythmia, predicting a bad prognosis. We report the case of a 26-year-old male individual with refractory heart failure due to isolated left ventricular non-compacted myocardium that needed a heart transplant.

La no compactación del miocardio está caracterizada por excesiva trabeculación y recesos de los ventrículos (usualmente el ventrículo izquierdo), debido a la interrupción del proceso de compactación de las fibras miocárdicas durante la fase embriogénica. Esta anormalidad cursa frecuentemente con insuficiencia cardiaca, fenómenos tromboembólicos y arritmias cardiacas, presentando un mal pronóstico. En este artículo describimos un caso de un rapaz de 26 años con insuficiencia cardiaca refractaria debido a no compactación aislada del miocardio ventricular izquierdo, que necesitó transplante cardiaco.

Myopathy, left ventricular noncompaction, and tetralogy of fallot.

Left ventricular hypertrabeculation, also termed noncompaction (LVHT) is a cardiac abnormality characterized by excessive trabeculations of the left ventricular cavity. LVHT may be associated with extracardiac abnormalities, most frequently neuromuscular disorders (NMDs). LVHT associated with tetralogy of Fallot (ToF), so far, has been reported in 2 cases, but no indication for the presence of NMDs are given.We present a 47-year old female who underwent 2 cardiac surgeries because of ToF, and was admitted because of heart failure. She was diagnosed with echocardiographic LVHT. A myopathy was diagnosed by biopsy.

Aortic valve replacement in noncompaction cardiomyopathy at two-year follow-up.

We report a male patient suffering left ventricular noncompaction cardiomyopathy in combination with low-gradient aortic stenosis. Preoperative echocardiography and magnetic resonance imaging revealed the diagnosis. After aortic valve replacement, the clinical course was uneventful. Two years of follow-up confirmed that the general prognosis was primarily affected by impaired ventricular function, thrombembolism, and arrhythmias.