"NASCI case of the month: left ventricular apical hypoplasia".

Left ventricular apical hypoplasia is a rare congenital condition. It can cause nonspecific symptoms and can be accompanied by cardiac conduction system alterations such as bundle branch block, atrial flutter (AF) or atrial fibrillation. The diagnosis mostly is made by imaging.

The Vectorcardiogram and the Main Dromotropic Disturbances.

Until the mid-1980s, it was believed that the vectorcardiogram (VCG) presented a greater specificity, sensitivity and accuracy in comparison to the 12-lead electrocardiogram (ECG), in the cardiology diagnosis. Currently, the VCG still is superior to the ECG in specific situations, such as in the evaluation of myocardial infarctions when associated with intraventricular conduction disturbances, in the identification and location of accessory pathways in ventricular preexcitation, in the differential diagnosis of patterns varying from normal of electrical axis deviation, in the evaluation of particular aspects of Brugada syndrome, Brugada phenocopies, concealed form of arrhythmogenic right ventricular cardiomyopathy and zonal or fascicular blocks of the right bundle branch on right ventricular free wall.VCG allows us to analyze the presence of left septal fascicular block more accurately than ECG and in the diagnosis of the interatrial blocks and severity of some chambers enlargements. The three-dimensional spatial orientation of both the atrial and the ventricular activity provides a far more complete observation tool than the linear ECG. We believe that the ECG/VCG binomial simultaneously obtained by the technique called electro-vectorcardiography (ECG/VCG) brought a significant gain for the differential diagnosis of several pathologies. Finally, in the field of education and research, VCG provided a better and more rational tridimensional insight into the electrical phenomena that occurs spatially, and represented an important impact on the progress of electrocardiography.

[Alternating bundle branch block in a supraventricular tachycardia: Which is the mechanism ?] / Bloqueo de rama alternante en una taquicardia supraventricular ¿ Cuál es el mecanismo subyacente ?

Functional bundle branch block during a supraventricular tachycardia can be observed with shorter cycle lengths and represent a physiologic response by the specialized intraventricular conduction system to accelerated AV nodal conduction. The present case corresponds to a young patient with exercise induced orthodromic A-V reentrant tachycardia and alternating bundle branch block. This unusual response is explained by the finding obtained during the electrophysiology study. An accelerated AV nodal conduction made the depolarizing wave front reach the bundle branches during their refractory period. Once block in one bundle was stablished, block persisted due to the linking phenomenon that is repetitive retrograde concealed conduction from the contralateral bundle. After catheter ablation of a concealed left-sided accessory A-V pathway, rapid atrial pacing at the same cycle length of the tachycardia reproduced the same aberrancies observed during tachycardia. This response proved that functional bundle branch block is due to the short cycle length and not the presence of an accessory A-V pathway.

El bloqueo de rama funcional durante una taquicardia supraventricular puede ser observado con longitudes de ciclo cortas y representa una respuesta fisiológica del sistema de conducción intraventricular por la existencia de conducción nodal auriculo ventricular acelerada. Presentamos el caso de un paciente joven con taquicardia reentrante aurículo-ventricular ortodrómica y bloqueo de rama alternante. Esta respuesta infrecuente se explica por el hallazgo obtenido durante el estudio electrofisiológico. Una conducción nodal aurículo-ventricular acelerada produce un frente de onda que despolariza las ramas durante sus períodos refractarios. Una vez que ocurrió el bloqueo en una de las ramas, dicho bloqueo persistió debido al fenómeno de linking, que es por conducción oculta retrógrada repetitiva de la rama contralateral. Después de la ablación transcatéter de una vía accesoria oculta lateral izquierda, el marcapaseo auricular rápido a la misma longitud de ciclo de la taquicardia, reprodujo la misma aberrancia observada durante la taquicardia. Este procedimiento demostró que el bloqueo de rama funcional fue debido a la longitud de ciclo corto y no a la presencia de una vía accesoria aurículo-ventricular.

Bloqueo de rama alternante en una taquicardia supraventricular: ¿Cuál es el mecanismo subyacente? / Alternating bundle branch block in a supraventricular tachycardia: Which is the mechanism ?

El bloqueo de rama funcional durante una taquicardia supraventricular puede ser observado con longitudes de ciclo cortas y representa una respuesta fisiológica del sistema de conducción intraventricular por la existencia de conducción nodal auriculo ventricular acelerada. Presentamos el caso de un paciente joven con taquicardia reentrante aurículo-ventricular ortodrómica y bloqueo de rama alternante. Esta respuesta infrecuente se explica por el hallazgo obtenido durante el estudio electrofisiológico. Una conducción nodal aurículo-ventricular acelerada produce un frente de onda que despolariza las ramas durante sus períodos refractarios. Una vez que ocurrió el bloqueo en una de las ramas, dicho bloqueo persistió debido al fenómeno de linking, que es por conducción oculta retrógrada repetitiva de la rama contralateral. Después de la ablación transcatéter de una vía accesoria oculta lateral izquierda, el marcapaseo auricular rápido a la misma longitud de ciclo de la taquicardia, reprodujo la misma aberrancia observada durante la taquicardia. Este procedimiento demostró que el bloqueo de rama funcional fue debido a la longitud de ciclo corto y no a la presencia de una vía accesoria aurículo-ventricular.

Functional bundle branch block during a supraventricular tachycardia can be observed with shorter cycle lengths and represent a physiologic response by the specialized intraventricular conduction system to accelerated AV nodal conduction. The present case corresponds to a young patient with exercise induced orthodromic A-V reentrant tachycardia and alternating bundle branch block. This unusual response is explained by the finding obtained during the electrophysiology study. An accelerated AV nodal conduction made the depolarizing wave front reach the bundle branches during their refractory period. Once block in one bundle was stablished, block persisted due to the linking phenomenon that is repetitive retrograde concealed conduction from the contralateral bundle. After catheter ablation of a concealed left-sided accessory A-V pathway, rapid atrial pacing at the same cycle length of the tachycardia reproduced the same aberrancies observed during tachycardia. This response proved that functional bundle branch block is due to the short cycle length and not the presence of an accessory A-V pathway.

Left bundle branch block: Epidemiology, etiology, anatomic features, electrovectorcardiography, and classification proposal.

In left bundle branch block (LBBB), the ventricles are activated in a sequential manner with alterations in left ventricular mechanics, perfusion, and workload resulting in cardiac remodeling. Underlying molecular, cellular, and interstitial changes manifest clinically as changes in size, mass, geometry, and function of the heart. Cardiac remodeling is associated with progressive ventricular dysfunction, arrhythmias, and impaired prognosis. Clinical and diagnostic notions about LBBB have evolved from a simple electrocardiographic alteration to a critically important finding affecting diagnostic and clinical management of many patients. Advances in cardiac magnetic resonance imaging have significantly improved the assessment of patients with LBBB and provided additional insights into pathophysiological mechanisms of left ventricular remodeling. In this review, we will discuss the epidemiology, etiologies, and electrovectorcardiographic features of LBBB and propose a classification of the conduction disturbance.

Transient left septal fascicular block and left anterior fascicular block as a consequence of proximal subocclusion of the left anterior descending coronary artery.

The association of left anterior fascicular block (LAFB) with left septal fascicular block (LSFB) characterizes a left bifascicular block subtype rarely described in the literature, probably due to the fact that most researchers are not aware of the existence of the left septal fascicle. We describe a case with this transient intraventricular dromotropic disturbance due to left anterior descending coronary artery subocclusion.

Electro-vectorcardiographic demonstration of bifascicular block associated with ventricular preexcitation.

Down syndrome occurs more frequently in the offsprings of older pregnant women and may be associated with atrioventricular septal defect. This refers to a broad spectrum of malformations characterized by a deficiency of the atrioventricular septum and abnormalities of the atrioventricular valves caused by an abnormal fusion of the superior and inferior endocardial cushions with the midportion of the atrial septum and the muscular portion of the ventricular septum.

Multiple myocardial bridges associated with left-ventricular dysfunction, intermittent left bundle branch block, and cardiac memory: A case report.

Myocardial bridges are a congenital anomaly in which a segment of the coronary artery takes a "tunneled" intramuscular course. Few reports have associated myocardial bridges with left-ventricular dysfunction in patients with ischemia. Intermittent left bundle branch block is a conduction disturbance that has been described to be associated with myocardial bridges and cardiac memory. This study reports unusual associations of multiple myocardial bridges, angina, left-ventricular dysfunction, intermittent left bundle branch block, and cardiac memory.

Myocardial blood flow assessment with 82rubidium-PET imaging in patients with left bundle branch block.

OBJECTIVES: Perfusion abnormalities are frequently seen in Single Photon Emission Computed Tomography (SPECT) when a left bundle branch block is present. A few studies have shown decreased coronary flow reserve in the left anterior descending territory, regardless of the presence of coronary artery disease. OBJECTIVE: We sought to investigate rubidium-82 (82Rb) positron emission tomography imaging in the assessment of myocardial blood flow and coronary flow reserve in patients with left bundle branch block. METHODS: Thirty-eight patients with left bundle branch block (GI), median age 63.5 years, 22 (58%) female, 12 with coronary artery disease (≥70%; GI-A) and 26 with no evidence of significant coronary artery disease (GI-B), underwent rest-dipyridamole stress 82Rb-positron emission tomography with absolute quantitative flow measurements using Cedars-Sinai software (mL/min/g). The relative myocardial perfusion and left ventricular ejection fraction were assessed in 17 segments. These parameters were compared with those obtained from 30 patients with normal 82Rb-positron emission tomography studies and without left bundle branch block (GII). RESULTS: Stress myocardial blood flow and coronary flow reserve were significantly lower in GI than in GII (p<0.05). The comparison of coronary flow reserve between GI-A and GI-B showed that it was different from the global coronary flow reserve (p<0.05) and the stress flow was significantly lower in the anterior than in the septal wall for both groups. Perfusion abnormalities were more prevalent in GI-A (p=0.06) and the left ventricular ejection fraction was not different between GI-A and GI-B, whereas it was lower in GI than in GII (p<0.001). CONCLUSION: The data confirm that patients with left bundle branch block had decreased myocardial blood flow and coronary flow reserve and coronary flow reserve assessed by 82Rb-positron emission tomography imaging may be useful in identifying coronary artery disease in patients with left bundle branch block.

Dipyridamole stress myocardial perfusion by computed tomography in patients with left bundle branch block.

BACKGROUND: Functional tests have limited accuracy for identifying myocardial ischemia in patients with left bundle branch block (LBBB). OBJECTIVE: To assess the diagnostic accuracy of dipyridamole-stress myocardial computed tomography perfusion (CTP) by 320-detector CT in patients with LBBB using invasive quantitative coronary angiography (QCA) (stenosis ≥ 70%) as reference; to investigate the advantage of adding CTP to coronary computed tomography angiography (CTA) and compare the results with those of single photon emission computed tomography (SPECT) myocardial perfusion scintigraphy. METHODS: Thirty patients with LBBB who had undergone SPECT for the investigation of coronary artery disease were referred for stress tomography. Independent examiners performed per-patient and per-coronary territory assessments. All patients gave written informed consent to participate in the study that was approved by the institution's ethics committee. RESULTS: The patients' mean age was 62 ± 10 years. The mean dose of radiation for the tomography protocol was 9.3 ± 4.6 mSv. With regard to CTP, the per-patient values for sensitivity, specificity, positive and negative predictive values, and accuracy were 86%, 81%, 80%, 87%, and 83%, respectively (p = 0.001). The per-territory values were 63%, 86%, 65%, 84%, and 79%, respectively (p < 0.001). In both analyses, the addition of CTP to CTA achieved higher diagnostic accuracy for detecting myocardial ischemia than SPECT (p < 0.001). CONCLUSION: The use of the stress tomography protocol is feasible and has good diagnostic accuracy for assessing myocardial ischemia in patients with LBBB.

Relationship between electrocardiographic characteristics of left bundle branch block and echocardiographic findings.

BACKGROUND: Complete left bundle branch block (CLBBB) is an electrocardiographic (ECG) dromotropic disorder seen in patients with various structural heart diseases and sometimes is associated with poor prognosis. Its presence confounds the application of standard ECG criteria for the diagnosis of left ventricular hypertrophy (LVH), myocardial infarction (MI) in the chronic phase, and pathologies that produce changes on ST-T segment. The aim of this investigation was to establish the relationship between CLBBB and cardiac structural abnormalities assessed by echocardiography. METHODS: This observational, cross-sectional study included ECG with CLBBB from 101 patients who also had transthoracic echocardiogram (TTE) performed within 6 months. RESULTS: The prevalence of structural heart disease on TTE was 90%. No ECG criterion was useful to diagnose LVH since no relationship was observed between 9 different ECG signs and increased left ventricular mass index. QRS duration (p = 0.16) and left axis deviation (p = 0.09) were unrelated to reduced left ventricular ejection fraction (LVEF). Eight ECG signs proposed for the diagnosis of the chronic phase of MI demonstrated similar effectiveness, with high specificity and reduced sensitivity. CONCLUSIONS: CLBBB is associated with elevated prevalence of cardiac structural disease and hinders the application of common ECG criteria for the diagnosis of LVH, reduced LVEF, or chronic phase of MI. No ECG finding distinguished patients with structural heart disease from those with normal hearts. Electrocardiographic criteria for the diagnosis of MI in the chronic phase are useful when present, but when absent cannot rule it out.

Mechanical dyssynchrony is similar in different patterns of left bundle-branch block.

BACKGROUND: Left bundle-branch block (LBBB) and the presence of systolic dysfunction are the major indications for cardiac resynchronization therapy (CRT). Mechanical ventricular dyssynchrony on echocardiography can help identify patients responsive to CRT. Left bundle-branch block can have different morphologic patterns. OBJECTIVE: To compare the prevalence of mechanical dyssynchrony in different patterns of LBBB in patients with left systolic dysfunction. METHODS: This study assessed 48 patients with ejection fraction (EF) < 40% and LBBB consecutively referred for dyssynchrony analysis. Conventional echocardiography and mechanical dyssynchrony analysis were performed, interventricular and intraventricular, with ten known methods, using M mode, Doppler and tissue Doppler imaging, isolated or combined. The LBBB morphology was categorized according to left electrical axis deviation in the frontal plane and QRS duration > 150 ms. RESULTS: The patients' mean age was 60 ± 11 years, 24 were males, and mean EF was 29% ± 7%. Thirty-two had QRS > 150 ms, and22, an electrical axis between -30º and +90º. Interventricular dyssynchrony was identified in 73% of the patients, while intraventricular dyssynchrony, in 37%-98%. Patients with QRS > 150 ms had larger left atrium and ventricle, and lower EF (p < 0.05). Left electrical axis deviation associated with worse diastolic function and greater atrial diameter. Interventricular and intraventricular mechanical dyssynchrony (ten methods) was similar in the different LBBB patterns (p = ns). CONCLUSION: In the two different electrocardiographic patterns of LBBB analyzed, no difference regarding the presence of mechanical dyssynchrony was observed.

Cardiac resynchronization therapy improves the uptake of MIBI-99mTc and cardiac function.

This case shows the improvement promoted by cardiac resynchronization therapy (CRT) on myocardial perfusion and left ventricular (LV) performance assessed by gated myocardial perfusion scintigraphy. The patient had idiopathic dilated cardiomyopathy, left bundle branch block and severe heart failure despite optimized medical treatment. After CRT, clinical improvement, QRS reduction and improvement of previously hypoperfused anterior and septal walls were observed. There was also decrease in LV end-diastolic and systolic volumes and increase in LV ejection fraction.

[Myocardial perfusion study in patients with right bundle branch block]. / Estudo de perfusão miocárdica em portadores de bloqueio de ramo direito.

PURPOSE: To evaluate the sensitivity and specificity of myocardial perfusion scintigraphy in the detection of coronary artery disease in patients with right bundle branch block (RBBB). METHODS: Thirty one patients (24 male, 62.3 +/- 10.5 years) with RBBB, submitted to myocardial perfusion scintigraphy associated with exercise (n = 7) or dipyridamole (n = 24) and previous cinecoronariography were studied retrospectively. Left ventricle scintigraphic image was divided in three segments corresponding to the three main epicardic coronary territories in a total of 93 segments. Cineangiographic and scintigraphic data were then compared according to the different artery territories. RESULTS: Twenty three patients had significant lesions (> or = 60%) in one or more coronary arteries and eight had no obstruction. Forty nine segments were irrigated by normal coronary arteries and 44 were related to arteries that had significant lesions. Twenty out of twenty three patients showed alterations in perfusion (sensitivity = 87%). All patients without coronary obstructions showed normal perfusion scintigraphy (specificity = 100%). One of the segments perfused by normal coronaries showed abnormal scintigraphy. Scintigraphy showed perfusion defects in 29 out of 44 segments with coronary obstructions. Sensitivity and specificity of the method for each arterial territory were 72% and 100% (left descending coronary artery), 67% and 94% (right coronary artery), 55% and 100% (circunflex coronary artery), respectively. CONCLUSION: The presence of RBBB does not modify the sensitivity and specificity of the method in the detection of coronary artery disease.