Left-ventricular noncompaction in an infant with trisomy 21.

Left-ventricular noncompaction (LVNC) is a rare congenital cardiomyopathy of emerging importance. It is known to occur in association with genetic syndromes. We report here for the first time an infant with LVNC in association with trisomy 21.

CHADS2- and CHA2DS2VASc scores and embolic risk in left ventricular hypertrabeculation/noncompaction.

BACKGROUND: Left ventricular hypertrabeculation/noncompaction (LVHT) is associated with stroke or embolism (S/E). The aim of this study was to assess if the Congestive heart failure, Hypertension, Age >75 years, Diabetes, and Stroke (CHADS2) and CHA2DS2VASc scores are different between LVHT-patients with and without stroke/embolism. METHODS: Records of LVHT patients were retrospectively screened. For stroke classification, the Trial of Org 10172 in Acute Stroke Treatment (TOAST) criteria were applied, for peripheral embolism angiographic findings. Baseline data were compared between patients with and without S/E. RESULTS: In 26 of 169 patients (15%), stroke (n = 24) or peripheric embolism (n = 2) had occurred. S/E etiology was either cardioembolic (n = 18), atherosclerotic (n = 5), or undetermined (n = 3). S/E occurred before (n = 17) and after (n = 9) diagnosis of LVHT/NC. The prevalence of hypertension (62 vs 35%; P < .05), CHADS2, and CHA2DS2-VASc scores were higher in patients with than without S/E (2.85 vs 1.26 and 3.69 vs 1.93, respectively; P < .001). CONCLUSIONS: S/E in LVHT is not always cardioembolic, but may also have an atherosclerotic cause. The CHADS2 score may be useful for clinical decision-making about oral anticoagulation for the prevention of S/E in LVHT patients.

Coexistence of left ventricular noncompaction and double-orifice mitral valve in a patient with congestive heart failure.

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy. It usually presents with ventricular dysfunction, thromboembolic events and arrhythmias. An asymptomatic clinical course is also possible. LVNC is frequently associated with other congenital heart diseases including heart valve abnormalities. The coexistence of LVNC with double-orifice mitral valve was observed rarely. The presence of such coexistence in a patient presented with heart failure in the newborn period is reported herein because of its rarity.

[Myocardial noncompaction recognized following a transient ischemic attack]. / Geçici iskemik atak sonrasi saptanan süngerimsi miyokart (noncompaction).

Myocardial noncompaction is a rare type of congenital cardiomyopathy characterized by excessively prominent trabeculations in one or more segments of the ventricles and deep intertrabecular recesses in ventricular walls. A 25-year-old male patient presented to the neurology department with complaints of weakness in the left extremities. A mild loss of muscle strength was detected on neurological examination. With a preliminary diagnosis of acute cerebrovascular event, treatment with aspirin and enoxaparin was instituted, which improved his complaints within two hours. Electrocardiography showed sinus rhythm, left ventricular hypertrophy, and loss of R-wave progression in the precordial leads. Transthoracic echocardiography showed apical hypokinesia, marked left ventricular hypertrophy, and normal left ventricular diameters. There were numerous trabeculations in the apex, apical, lateral, and inferior walls, and deep intertrabecular recesses. Color Doppler showed blood flow into the intertrabecular recesses. He also had mild mitral regurgitation and diastolic dysfunction of restrictive type. He was scheduled for outpatient follow-up on aspirin and warfarin treatment.

Anticoagulation Therapy in Specific Cardiomyopathies: Isolated Left Ventricular Noncompaction and Peripartum Cardiomyopathy.

In 2 distinct entities, left ventricular noncompaction (LVNC) and peripartum cardiomyopathy (PPCM), routine anticoagulation therapy is often used in current practices. However, our systematic review showed that LVNC itself was not associated with the increase in thromboembolism event rates and therapeutic anticoagulation therapy should not be considered only for LVNC, unless there is risk factor for thromboembolism. Current literature justifies prophylactic therapeutic anticoagulation in LVNC with low left ventricular ejection fraction (EF < 40%) and/or atrial fibrillation. Although not specifically studied, the presence of intracardiac thrombi by echocardiography or other imaging studies should also prompt anticoagulation therapy. There is limited evidence available for the use of anticoagulation in patients with PPCM, but our systematic review showed that anticoagulation should be recommended only for patients with PPCM especially with an EF < 35% until EF is recovered, as well as for patients with PPCM treated with bromocriptine.

A case of vascular Ehlers-Danlos Syndrome with a cardiomyopathy and multi-system involvement.

Ehlers-Danlos Syndrome comprises a heterogeneous group of heritable connective tissue disorders resulting from various gene mutations. We present an unusual case of vascular Ehlers-Danlos Syndrome with distinctive physical characteristics and a cardiomyopathy with features suggesting isolated left ventricular non-compaction. The cardiac features represent the first report of a cardiomyopathy associated with a mutation in the COL3A1 gene. This case also illustrates the multi-system nature of Ehlers-Danlos Syndrome and the complexity of managing patients with the vascular subtype.

[Heart transplantation for the treatment of isolated left ventricular myocardial noncompaction. First case in Mexico]. / Trasplante cardiaco: una opción para tratamiento del miocardio no compactado aislado de ventrículo izquierdo. Primer caso en México.

BACKGROUND: Myocardial noncompaction of the left ventricle is a congenital cardiomyopathy characterised by left ventricular hypertrabeculation and prominent intertrabecular recesses. The incidence ranges from 0.15% to 2.2%. Clinical manifestations include heart failure, arrhythmias, and stroke. Prognosis is fatal in most cases. Heart transplantation is a therapeutic option for this cardiomyopathy, and few had been made worldwide. CLINICAL CASE: The case is presented of a 20 year-old male with noncompacted myocardium of the left ventricle, who had clinical signs of heart failure. His functional class was IV on the New York Heart Association scale. He was successfully transplanted. Its survival to 15 months is optimal in class I New York Heart Association, and endomyocardial biopsies have been reported without evidence of acute rejection. CONCLUSION: It is concluded that heart transplantation modified the natural history and improved survival in patients with this congenital heart disease.

Isolated left ventricular non-compaction (LVNC) and recurrent strokes: to anticoagulate or not to anticoagulate, that is the question.

Isolated left ventricular non-compaction (LVNC) is an uncommon primary cardiomyopathy associated with significant risk of thromboembolic stroke. We report a case of a 69-year-old man with a medical history of ischaemic stroke who presented with a stroke for the second time, and during stroke workup transthoracic echo was suggestive of increased apical trabeculation. He underwent cardiac MRI study to evaluate the left ventricular structure, which revealed LVNC cardiomyopathy, which we believe is the main culprit of his recurrent strokes. Given the high risk of stroke recurrence, antiplatelets followed by anticoagulation for secondary prevention were initiated. This case demonstrates the association between LVNC and recurrent stroke, with a literature review trying to address the dilemma facing the clinician to decide on anticoagulation in such patients.

Left Ventricular Noncompaction Cardiomyopathy Presenting with Heart Failure in a 35-Year-Old Man.

Isolated ventricular noncompaction, a rare genetic cardiomyopathy, is thought to be caused by the arrest of normal myocardial morphogenesis. It is characterized by prominent, excessive trabeculation in a ventricular wall segment and deep intertrabecular recesses perfused from the ventricular cavity. The condition can present with heart failure, systematic embolic events, and ventricular arrhythmias. Two-dimensional echocardiography is the typical diagnostic method. We report a case of heart failure in a 35-year-old man who presented with palpitations. Two-dimensional echocardiograms revealed left ventricular noncompaction, which markedly improved after standard heart failure therapy.

Effects of ß-blocker therapy on electrocardiographic and echocardiographic characteristics of left ventricular noncompaction.

Left ventricular noncompaction (LVNC) is a cardiomyopathy with hypertrabeculation of the LV, often complicated by heart failure, arrhythmia and thromboembolic events. The features of LVNC are still incompletely characterized due to its late recognition as clinically relevant condition. The aims of this study were to describe echocardiographic and electrophysiologic characteristics of LVNC patients and to assess the effects of chronic ß-blocker treatment. Study patients (n = 20; 42.5 [36.3; 52.5] years; 12 men) exhibited reduced LV ejection fraction (median LVEF = 32 %) and an increased LV mass of 210 g. Sinus rhythm was present in 19 patients, whereas one patient was in atrial fibrillation. Baseline heart rate was 77.5 beats per minute. Left bundle branch block was detected in five cases. In a subgroup of patients receiving ß-blocker therapy (n = 17), LV mass was reduced from 226 [178; 306] g to 220 [169; 254] g (p = 0.007) at 13 ± 6 months follow-up. By contrast, a subgroup of three patients that were not treated with an anti-ß-adrenergic agent showed LV mass increase from 180 [169; 197] g to 199 [185; 213] g (p = 0.023). LVEF and electrocardiographic parameters were not significantly modulated during chronic ß-blocker treatment. There was no sustained symptomatic ventricular tachyarrhythmia, thromboembolic event or death in either group. In conclusion, this study reveals reduction of LV mass among LVNC patients during ß-blocker therapy. Effects of ß-blocker treatment in LVNC require validation in prospective controlled studies.

Isolated left ventricular noncompaction causing stroke in a 30-year-old woman: case report and literature review.

Isolated left ventricular noncompaction is a rare form of cardiomyopathy characterized by prominent left ventricular trabeculations and intertrabecular recesses. The typical clinical manifestations are severe systolic and diastolic dysfunction, conduction abnormalities, and cardiac embolic events theorized to result from thrombus formation within the intertrabecular recesses. Evidence-based recommendations for preventing thromboembolic events in isolated left ventricular noncompaction have not been established. We report the case of a woman who, at 10 years of age, had been diagnosed with hypertrophic cardiomyopathy without systolic dysfunction. At age 30, she presented with left hemiparesis consequent to a large right-hemispheric ischemic stroke, and she was diagnosed with isolated left ventricular noncompaction. In addition to discussing the patient's case, we review the medical literature that pertains to isolated left ventricular noncompaction.

Left ventricular non-compaction in pregnancy.

Left-ventricular non-compaction (LVNC) represents an arrest in the normal process of myocardial compaction, resulting in multiple, prominent, persistant trabeculations and deep inter-trabecular recesses communicating with the ventricular cavity. LVNC is a rarely encountered cardiomyopathy and few cases have been reported in pregnancy. In this case report we present a patient who referred to our clinic with symptoms of heart failure during pregnancy and whose echocardiographic examination revealed prominent trabeculations in the left ventricle.

Pregnancy and treatment outcome in a patient with left ventricular non-compaction.

Left ventricular non-compaction (LVNC) is a rare form of cardiomyopathy. This case reviews a woman with familial LVNC (EF 45%, NYHA class I, evidence of non-sustained ventricular tachycardia pre-pregnancy) who had significant decompensation with heart failure in the third trimester that required early delivery. Deterioration in symptoms and LV function 7 days after delivery required further hospitalization and aggressive treatment. Suppression of lactation with bromocriptine, together with standard heart failure management, has allowed recovery and return to full activities and work. Acknowledged adverse risk factors in LVNC are considered, and pre-pregnancy risk assessment is reviewed. There is no specific treatment for LVNC in pregnancy besides the usual management of dilated cardiomyopathy. This is the ninth case report of LVNC in pregnancy reported in the literature.

Isolated left ventricular noncompaction in a 90-year-old man.

Isolated ventricular myocardial noncompaction is a cardiomyopathy that is being diagnosed more frequently in patients of all ages because of increased awareness and improvements in imaging methods. It is an extremely rare cause of heart failure in nonagenarians. We describe the case of a man who presented with heart failure for the first time at 90 years of age. The diagnosis was isolated left ventricular noncompaction. Transthoracic echocardiography showed a trabeculated, sponge-like appearance of the left ventricular apical and inferolateral segments. After medical management, the patient was asymptomatic at the 3-month follow-up examination. Knowledge of ventricular noncompaction is increasing within the cardiology community. Patients who have isolated noncompaction with a limited number of involved ventricular segments can live beyond normal life expectancy without developing heart failure. In addition to discussing our patient's case, we briefly review the relevant medical literature.

'Idiopathic' acute myocardial infarction in a young patient with noncompaction cardiomyopathy.

Isolated left ventricular noncompaction (LVNC) is a rare hereditary cardiomyopathy characterized by prominent intraventricular trabeculations separated by deep intertrabecular recessus. While cardiac ischemia due to microvascular dysfunction is common in these patients, ST-segment elevation myocardial infarction (STEMI) is rare and usually seen as a consequence of coincidental coronary artery disease. We report the case of a 20 year-old male patient admitted to our emergency department with a complaint of squeezing chest pain who was subsequently diagnosed with STEMI according to electrocardiographic findings, although an emergent coronary angiogram demonstrated normal coronary arteries. Echocardiography revealed isolated LVNC, and the diagnosis was confirmed via magnetic resonance imaging. Repeat coronary catheterization with acetylcholine infusion and coronary flow reserve measurement failed to demonstrate vasospasm or microvascular dysfunction. As no apparent cause was found, this case was designated 'idiopathic' myocardial infarction. Coronary thromboembolism due to stagnation of blood in the left ventricular cavity remained as the most probable mechanism underlying myocardial infarction.

Multiple left ventricular thrombi in a patient with left ventricular noncompaction.

The major clinical features of myocardial noncompaction are heart failure, arrhythmias, and thromboembolic events. Prominent myocardial trabeculae and deep recesses characteristic of myocardial noncompaction can cause stagnant blood flow and the formation of left ventricular clots. We describe the case of a 62-year-old woman who presented with symptoms of heart failure secondary to left ventricular noncompaction. Transthoracic and transesophageal echocardiography revealed multiple left ventricular thrombi, which had formed despite the patient's long-term therapy with aspirin. Anticoagulative therapy should be considered for patients with myocardial noncompaction who also have risk factors for thromboembolism, such as atrial fibrillation, a history of systemic embolism, or severe left ventricular systolic dysfunction. However, chronic antiplatelet therapy may not sufficiently prevent clot formation in patients who have myocardial noncompaction and severe left ventricular systolic dysfunction.

Left ventricular noncompaction.

Left ventricular noncompaction is a rare cardiomyopathy characterized by prominent ventricular hypertrabeculation. Here, we discuss the case of a 30-year-old man who presented with rapidly conducted atrial fibrillation. The images we present demonstrate appearances characteristic of this cardiomyopathy, with prominent trabeculations and an end-diastolic noncompacted to compacted myocardial thickness ratio of more than 2 noted on echocardiography and magnetic resonance imaging.

Snake heart: a case of atavism in a human being.

Atavism is the rare reappearance, in a modern organism, of a trait from a distant evolutionary ancestor. We describe an apparent case of atavism involving a 59-year-old man with chest pain whose coronary circulation and myocardial architecture resembled those of the reptilian heart. The chest pain was attributed to a coronary steal phenomenon. The patient was discharged from the hospital on a heightened regimen of ß-blockers, and his symptoms improved significantly. To our knowledge, this is only the 2nd reported clinical case of a human coronary circulation similar to that of reptiles.