Left main coronary artery compression by a dilated main pulmonary artery and left coronary sinus of Valsalva aneurysm in a patient with heritable pulmonary arterial hypertension and FLNA mutation
نویسندگان
چکیده
Left main coronary artery (LMCA) disease due to external compression by a dilated main pulmonary artery (MPA) is an uncommon clinical entity. Here, we describe a 52-year-old woman with pulmonary arterial hypertension (PAH) and anteroseptal old myocardial infarction (OMI). The cause of the OMI was external compression of the LMCA by the dilated MPA and aneurysm of the left coronary sinus of Valsalva. The patient's sister (aged 56 years) had also been diagnosed with PAH and both women had a novel heterozygous splicing mutation, IVS2-2A > G (c.374-2A > G in NM_001456), in the filamin A ( FLNA) gene. To our knowledge, this is the first report of HPAH which is likely to be due to FLNA mutation and compression of the LMCA between a dilated MPA and aneurysm of the left coronary sinus of Valsalva.
منابع مشابه
پارگی آنوریسم سینوس والسالوا چپ به شریان پولمونر اصلی: یک مورد بسیار نادر
Sinus of valsalva aneurysm, is a rare congenital defect and the diagnosis is usually made after it ruptures. The anomaly often involves the right coronary sinus and is less frequent in the noncoronary sinus. The rupture of the left sinus of valsalva into the pulmonary artery is extremely rare. So far, few cases with ruptured left sinus of valsalva have been reported. We are reporting a ruptur...
متن کاملExtremely rare case of a rupture of the left sinus of Valsalva aneurysm into the main pulmonary artery
We describe a case of ruptured aneurysm of the left coronary sinus of Valsalva diagnosed by transthoracic two-dimensional echocardiography. A very few cases with ruptured left sinus of Valsalva have been reported. We are reporting a rupture of the left sinus of Valsalva into the main pulmonary artery in a 40-year-old male diagnosed by transthoracic echocardiography.
متن کاملExtrinsic compression of the left coronary ostium by the pulmonary trunk: management in a case of Eisenmenger syndrome.
Extrinsic compression of the left main coronary artery by a massively dilated pulmonary artery in patients who have severe pulmonary hypertension can lead to significant myocardial ischemia. A 58-year-old man with a large patent ductus arteriosus and Eisenmenger syndrome presented with angina at rest and worsening heart failure of 3 months' duration. The new symptoms were recognized to be secon...
متن کاملA Case of Extrinsic Compression of the Left Main Coronary Artery Secondary to Pulmonary Artery Dilatation
Extrinsic compression of the left main coronary artery (LMCA) secondary to pulmonary artery dilatation is a rare syndrome. Most cases of pulmonary artery hypertension but no atherosclerotic risk factors rarely undergo coronary angiography, and hence, diagnoses are seldom made and proper management is often delayed in these patients. We describe a patient that presented with pulmonary hypertensi...
متن کاملTotal Occlusion of Left Main Coronary Artery by Dilated Main Pulmonary Artery in a Patient with Severe Pulmonary Hypertension
A 34-year-old woman was admitted to the hospital because of recently aggravated right heart failure without angina for 5 months. When she was 25 years old, patch repair with Polytetrafluoroethylene (PTFE) was performed for the secondum type of atrial septal defect (ASD) with moderate pulmonary hypertension. The chest PA, echocardiography and cardiac catheterization at current admission revealed...
متن کامل