No abstract

BACKGROUND:The electrocardiographic and intracardiac activation features of left atrial roof-dependent macroreentrant flutter have been incompletely characterized. METHODS:Patients post-pulmonary vein (PV) isolation with roofdependent atrial flutter based on activation and entrainment mapping were included. ECG and coronary sinus activation were compared with mitral annular (MA) flutter. RESULTS:The roof-dependent left atrial flutter circled the right PVs in 32 of 33 cases. Two forms of roof flutters were identified, posteroanterior, ascendant on posterior wall and descendant on anterior wall (n=24); and anteroposterior, ascendant on the anterior wall and descendent on the posterior wall (n=9). Both forms had positive large amplitude P waves in V 1 through V 2 with decreasing amplitude in V 3 through V 6 . Posteroanterior roof flutters had positive P wave in the inferior and negative P wave in leads I and aVL similar to counterclockwise MA flutter, but coronary sinus activation was simultaneous for roof and proximal to distal for counterclockwise. Anteroposterior roof flutters were similar to clockwise MA flutter with negative P in inferior leads and transition to flat or negative P in V 3 through V 6 . Coronary sinus activation time ≤39 ms identified roof versus MA flutter (sensitivity: 100% and specificity: 97%). CONCLUSIONS:Roof-dependent flutter around right PVs is more common than around left PVs. The ECG pattern for roof-dependent flutter around right PVs is similar to MA flutter with frontal plane axis dictated by septal activation. Roof-dependent flutter can be distinguished from MA flutter by more simultaneous rather than sequential coronary sinus activation.

Non-compaction cardiomyopathy (NCC) is characterized by trabeculations in either one or both ventricles. Clinical presentation is highly variable: dyspnea, palpitation, thromboembolic events, arrhythmia, or sudden cardiac death. There are currently no universally-accepted criteria for classifying and diagnosing left ventricular non-compaction (LVNC) cardiomyopathy. Transthoracic echocardiography (TTE) is the diagnostic exam of choice. The diagnosis is often missed or delayed because of a lack of knowledge about this uncommon disease. Progression of LVNC is highly variable and prognosis is very difficult to predict. We report a case of a 50-year-old female patient with a history of total thyroidectomy under hormonal supplementation who consults for dyspnea and paroxysmal palpitations revealing an isolated LVNC. This case emphasizes the importance of imaging techniques, which are, TTE and cardiac magnetic resonance imaging (MRI) in early diagnosis, management, and follow-up.

Pulmonary Arterial Hypertension (PAH) is a clinical syndrome consisting of physiologic/ hemodynamic criteria that are a consequence of several etiologies. Confirmation of pulmonary hypertension is based on right heart catheterization. Pulmonary hypertension is a devastating condition that can lead to considerable morbidity and premature mortality. In the last few decades, significant advancement in the pharmacotherapy of pulmonary hypertension has resulted from better understanding of the complex pathogenesis and pathophysiology of this dreaded disease. Despite these accomplishments, pharmacotherapy of pulmonary hypertension is still far from perfect, and the mortality in this modern treatment era is still unacceptably high. We report a complex clinical presentation characterized by severe pulmonary hypertension secondary to concomitant mitral stenosis with veno-occlusive disease in the context of systemic sclerosis. Our case highlights the importance of a systematic and comprehensive diagnostic approach to avoid missing an underlying pathology.