Longitudinal Shortening of the Left Ventricle by Cine-CMR for Assessment of Diastolic Function in Patients with Aortic Valve Disease / Encurtamento Longitudinal do Ventrículo Esquerdo por Cine-RMC para Avaliação da Função Diastólica em Pacientes com Doença Valvar Aórtica

Abstract Background: Diastolic dysfunction, commonly evaluated by echocardiography, is an important early finding in many cardiomyopathies. Cardiac magnetic resonance (CMR) often requires specialized sequences that extends the test time. Recently, feature-tracking imaging has been made available, but still requires expensive software and lacks clinical validation. Objective: To assess diastolic function in patients with aortic valve disease (AVD) and compare it with normal controls by evaluating left ventricular (LV) longitudinal displacement by CMR. Methods: We compared 26 AVD patients with 19 normal controls. Diastolic function was evaluated as LV longitudinal displacement in 4-chamber view cine-CMR images using steady state free precession (SSFP) sequence during the entire cardiac cycle with temporal resolution < 50 ms. The resulting plot of atrioventricular junction (AVJ) position versus time generated variables of AVJ motion. Significance level of p < 0.05 was used. Results: Maximum longitudinal displacement (0.12 vs. 0.17 cm), maximum velocity during early diastole (MVED, 0.6 vs. 1.4s-1), slope of the best-fit line of displacement in diastasis (VDS, 0.22 vs. 0.03s-1), and VDS/MVED ratio (0.35 vs. 0.02) were significantly reduced in AVD patients compared with controls, respectively. Aortic regurgitation showed significantly worse longitudinal LV shortening compared with aortic stenosis. Higher LV mass indicated worse diastolic dysfunction. Conclusions: A simple linear measurement detected significant differences on LV diastolic function between AVD patients and controls. LV mass was the only independent predictor of diastolic dysfunction in these patients. This method can help in the evaluation of diastolic dysfunction, improving cardiomyopathy detection by CMR, without prolonging exam time or depending on expensive software.

Resumo Fundamentos: A disfunção diastólica, comumente avaliada por ecocardiografia, é um importante achado precoce na maioria das cardiomiopatias. A ressonância magnética cardíaca (RMC) frequentemente requer sequências específicas que prolongam o tempo de exame. Recentemente, métodos de imagens com monitoramento de dados (feature-tracking) foram desenvolvidos, mas ainda requerem softwares caros e carecem de validação clínica. Objetivos: Avaliar a função diastólica em pacientes com doença valvar aórtica (DVA) e compará-la a controles normais pela medida do deslocamento longitudinal do ventrículo esquerdo (VE) por RMC. Métodos: Nós comparamos 26 pacientes com DVA com 19 controles normais. A função diastólica foi avaliada como uma medida do deslocamento longitudinal do VE nas imagens de cine-RMC no plano quatro câmaras usando a sequência steady state free precession (SSFP) durante todo o ciclo cardíaco com resolução temporal < 50 ms. O gráfico resultante da posição da junção atrioventricular versus tempo gerou variáveis de movimento da junção atrioventricular. Utilizamos nível de significância de p < 0,005. Resultados: Deslocamento longitudinal máximo (0,12 vs. 0,17 cm), velocidade máxima em início de diástole (0,6 vs. 1,4s-1), velocidade máxima na diástase (0,22 vs. 0,03s-1) e a razão entre a velocidade máxima na diástase e a velocidade máxima em diástole inicial (0,35 vs. 0,02) foram significativamente menores nos pacientes com DVA em comparação aos controles normais, respectivamente. Pacientes com insuficiência aórtica apresentaram medidas de encurtamento longitudinal do VE significativamente piores em comparação aqueles com estenose aórtica. O aumento da massa ventricular esquerda indicou pior disfunção diastólica. Conclusões: Esta simples medida linear detectou diferenças significativas na função diastólica do VE entre pacientes com DVA e controles normais. A massa ventricular esquerda foi o único preditor independente de disfunção diastólica nesses pacientes. Este método pode auxiliar na avaliação da disfunção diastólica, melhorando a detecção de cardiomiopatias por RMC sem prolongar o tempo de exame ou depender de caros softwares.

Unconventional 2:1 Ventricular Pacing in a Neonate with Congenital Heart Block and Biventricular Noncompaction.

Congenital complete heart block with concomitant biventricular noncompaction cardiomyopathy has been reported once previously. Although not universal, when restrictive physiology is present, impaired diastolic filling may pose a distinct challenge to pacing during the neonatal period. We present the case of a neonate with congenital complete heart block and biventricular noncompaction that resulted in severe diastolic dysfunction and atrioventricular dyssynchrony. We intentionally used 2:1 ventricular pacing to provide atrioventricular synchrony with every paced beat, and this resulted in hemodynamic and clinical improvement. This unconventional pacing technique may be beneficial in other neonates who have complete heart block and diastolic dysfunction.

Multimodal Imaging after Sudden Cardiac Arrest in an 18-Year-Old Athlete.

We report the case of a previously healthy 18-year-old male athlete who twice presented with sudden cardiac arrest. Our use of electrocardiography, echocardiography, cardiac magnetic resonance, coronary angiography, coronary computed tomographic angiography, and nuclear stress testing enabled the diagnoses of apical hypertrophic cardiomyopathy and anomalous origin of the right coronary artery. We discuss the patient's treatment and note the useful role of multiple cardiovascular imaging methods in cases of sudden cardiac arrest.

Down Syndrome with Complete Atrioventricular Septal Defect, Hypertrophic Cardiomyopathy, and Pulmonary Vein Stenosis.

The prevalence of congenital heart disease in infants with Down syndrome is 40%, compared with 0.3% in children who have normal chromosomes. Atrioventricular and ventricular septal defects are often associated with chromosomal aberrations, such as in trisomy 21, whereas hypertrophic cardiomyopathy is chiefly thought to be secondary to specific gene mutations. We found only one reported case of congenital hypertrophic cardiomyopathy and atrioventricular septal defect in an infant with Down syndrome. Here, we report atrioventricular septal defect, hypertrophic cardiomyopathy, and pulmonary vein stenosis in a neonate with Down syndrome-an apparently unique combination. In addition, we discuss the relevant medical literature.

Two cases of apical ballooning syndrome masking apical hypertrophic cardiomyopathy.

Apical akinesis and dilation in the absence of obstructive coronary artery disease is a typical feature of stress-induced (takotsubo) cardiomyopathy, whereas apical hypertrophy is seen in apical-variant hypertrophic cardiomyopathy. We report the cases of 2 patients who presented with takotsubo cardiomyopathy and were subsequently found to have apical-variant hypertrophic cardiomyopathy, after the apical ballooning from the takotsubo cardiomyopathy had resolved. The first patient, a 43-year-old woman with a history of alcohol abuse, presented with shortness of breath, electrocardiographic and echocardiographic features consistent with takotsubo cardiomyopathy, and no significant coronary artery disease. An echocardiogram 2 weeks later revealed a normal left ventricular ejection fraction and newly apparent apical hypertrophy. The 2nd patient, a 70-year-old woman with pancreatitis, presented with chest pain, apical akinesis, and a left ventricular ejection fraction of 0.39, consistent with takotsubo cardiomyopathy. One month later, her left ventricular ejection fraction was normal; however, hypertrophy of the left ventricular apex was newly noted. To our knowledge, these are the first reported cases in which apical-variant hypertrophic cardiomyopathy was masked by apical ballooning from stress-induced cardiomyopathy.

Microvascular permeability changes might explain cardiac tamponade after alcohol septal ablation for hypertrophic cardiomyopathy.

Various sequelae of alcohol septal ablation for hypertrophic obstructive cardiomyopathy have been reported. Of note, some cases of cardiac tamponade after alcohol septal ablation cannot be well explained. We describe the case of a 78-year-old woman with hypertrophic obstructive cardiomyopathy in whom cardiac tamponade developed one hour after alcohol septal ablation, probably unrelated to mechanical trauma. At that time, we noted a substantial difference in the red blood cell-to-white blood cell ratio between the pericardial effusion (1,957.4) and the peripheral blood (728.3). In addition to presenting the patient's case, we speculate that a possible mechanism for acute tamponade--alcohol-induced changes in microvascular permeability--is a reasonable explanation for cases of alcohol septal ablation that are complicated by otherwise-unexplainable massive pericardial effusions.

Apical hypertrophic cardiomyopathy: preliminary attempt at palliation with use of subselective alcohol ablation.

We report a case of severe apical hypertrophic cardiomyopathy in order to discuss the nature of this unusual condition and the possibility of using selective alcohol ablation to effectively treat symptomatic hypertrophic cardiomyopathy that presents with apical aneurysm. A 73-year-old woman with severe, progressive dyspnea and intermittent chest pain was found to have localized left ventricular apical dyskinesia distal to an obstructive mid-distal muscular ring. The ring caused total systolic obliteration of the apical left ventricular cavity. Apical cavity pressure was extremely high, up to 330 mmHg-200 mmHg above that in the main left ventricular cavity. Because of the danger of apical rupture and clot formation, we attempted the experimental use of alcohol ablation for effective palliation. We present our pilot experience, offer a novel interpretation of the nature of this obscure entity, and possibly justify a new catheter treatment. In addition, we discuss the developmental, pathophysiologic, and clinical implications of this unusual form of hypertrophic cardiomyopathy. To our knowledge, ours is the first reported use of subselective, modified-protocol alcohol septal ablation to treat an obstructive mid-apical muscular ring in a patient with apical hypertrophic cardiomyopathy.

Mid-ventricular hypertrophic obstructive cardiomyopathy presenting with acute myocardial infarction.

Mid-ventricular hypertrophic obstructive cardiomyopathy is a rare type of cardiomyopathy that can be accompanied by apical aneurysm. We report the case of a patient who presented with ventricular fibrillation, ST-segment elevation on electrocardiography, and cardiac-enzyme elevation, in the presence of normal coronary arteries. Echocardiography and magnetic resonance imaging showed an hourglass appearance of the left ventricle with an aneurysm in the apex. Left-heart catheterization and continuous-wave Doppler echocardiography revealed a pressure gradient between the apical and basal chambers of the left ventricle. Impaired coronary artery circulation might play a role in the development of mid-ventricular obstruction in patients with mid-ventricular hypertrophic obstructive cardiomyopathy.