Myocardial non-compaction (NC) is a rare genetically heterogeneous cardiomyopathy which is characterized by excessively prominent ventricular trabeculations with deep intertrabecular recesses. It is commonly believed to be due to an intrauterine arrest of the endomyocardial morphogenesis. Left ventricle (LV) is the usual site of affection, but very rarely right ventricular (RV) or both ventricles may be involved. It can be isolated or associated with other cardiac diseases or neuromuscular disorders. It has a wide spectrum of clinical manifestations that range from asymptomatic status, progressive ventricular dysfunction, arrhythmias and congestive heart failure that have been found to be associated with myocardial fibrosis. Cardiac Magnetic resonance (CMR) is the gold standard non-invasive modality for the diagnosis of cardiomyopathies. In NC cases, late gadolinium enhancement (LGE) of the trabeculated layer or mild fibrosis of the compacted layer was previously described in the literature, however extensive fibrosis in the compacted layer only with no fibrosis at the trabeculated layer was not described before. We report a male patient presented with heart failure symptoms, echocardiogram showed severe LV and RV systolic dysfunction with prominent bi-ventricular trabeculations. CMR confirmed the diagnosis of bi-ventricular non-compaction with peculiar pattern extensive fibrosis of the compacted layer of LV in LGE. To the best of our knowledge, our case is the first case to report extensive fibrosis in the compacted layer only with no fibrosis at the trabeculated layer.
Ortner's syndrome is a really rare complication that manifest by hoarseness of voice. It is usually due to left recurrent laryngeal nerve compression. Cardiac causes of Ortner's syndrome are rarely encountered and it is usually due to left atrial enlargement as a complication of valvular lesions affecting mitral valve but other rare causes include ascending aortic aneurysm or pulmonary artery aneurysm. Hereby, we present the 3rd case in the literature to report Ortner's syndrome due to pulmonary artery aneurysm in a 38-year old female patient with previous history of bilharziasis.
Tetralogy of Fallot (TOF) is the most common etiology of congenital cyanotic heart disease, and Blalock-Taussig shunt (BTS) operation is considered the first-step management to maintain pulmonary blood flow in TOF patients. Complete repair of TOF is the standard surgical treatment that should be performed in infancy or early childhood for improved long-term survival. However, the prognosis of TOF patients treated by only palliative operation remains uncertain. We report a man with TOF underwent classic BTS operation at 2 years of age. Despite no medication, he had a long asymptomatic life. At 53 years of age, he started to complain of dizziness and recurrent attacks of syncope due to complete heart block (CHB) and inserted a permanent pacemaker. 2D and 3D Echocardiography showed uncorrected TOF with an overriding aorta, two ventricular septal defects (VSDs) were seen; a large inlet type VSD and another small muscular one with marked right ventricular hypertrophy with marked infundibular stenosis. Cardiac Computed Tomography (Cardiac CT) showed TOF with noted severely stenotic right ventricular outflow tract and pulmonary flow was maintained through a patent classical BTS between left subclavian artery (SCA) and the left pulmonary artery (LPA). Due to his stable condition, he was discharged on close up follow-up visits.