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2005, Heart
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Pulmonary arterial dissection is an extremely rare and usually lethal complication of chronic pulmonary hypertension. The condition usually manifests as cardiogenic shock or sudden death and is therefore typically diagnosed at postmortem examination rather than during life. However, recent isolated reports have described pulmonary artery dissection in surviving patients. The first case of pulmonary artery dissection in a surviving patient with cor pulmonale caused by chronic obstructive pulmonary disease is presented. The aetiology, pathophysiology, and clinical presentation of pulmonary artery dissection are reviewed and factors that may aid diagnosis during life are discussed.
British Journal of Radiology, 2007
Pulmonary artery dissection is a serious fatal complication of chronic pulmonary arterial hypertension, always occurring at the site of maximal dilatation of the artery [1]. Diagnosis is made mainly at autopsy as many of these patients experience sudden death when the main pulmonary artery dissects into the pericardium, causing acute cardiac tamponade. To our knowledge, five reported cases have described CT imaging findings of acute pulmonary artery dissection in the literature. Figure 1. Posteroanterior chest radiograph showing cardiac enlargement with greatly dilated pulmonary trunk and central pulmonary arteries.
The American Journal of Emergency Medicine, 2016
Isolated cases are usually silent until adulthood. Clinical symptoms 8 include exercise intolerance, dyspnea, hemoptysis, and recurrent pulmo-9 nary infections. Diagnosis is difficult because of nonspecific symptoms 10 and usually suspected from abnormal findings in chest radiograph. Imag-11 ing modalities including transthoracic echocardiography, contrasted 12 thorax computed tomography, and thorax magnetic resonance imaging 13 can be performed for the correct diagnosis and to assess any concomitant 14 cardiovascular malformations. Treatment options include revasculariza-15 tion or conventional therapy for pulmonary artery hypertension for 16 those whose pulmonary artery pressure is elevated. 17 Unilateral absence of pulmonary artery (UAPA) is a rare congenital 18 anomaly due to malformation of sixth aortic arch during embryogene-19 sis. It can be isolated but often associated with other cardiovascular 20 abnormalities. Patients with isolated UAPA may remain clinically silent 21 until adulthood, but they are usually symptomatic. Clinical symptoms 22 include exercise intolerance, dyspnea, hemoptysis, and recurrent pul-23 monary infections. Diagnosis is difficult because of nonspecific symp-24 toms. Based on patients' complete medical history and physical 25 examination and by using imaging modalities, a correct diagnosis can 26 be made. 27 A 36-year-old male patient was referred to our emergency depart-28 ment with the initial diagnosis of pulmonary embolism. The patient 29 complained about exercise intolerance for 3-4 months. Recently, he 30 had difficulty in breathing even at rest. His medical history revealed 31 nothing important. His oxygen saturation was 92% at room air, and 32 other baseline vital signs were normal. Physical examination was unre-33 markable except or diminished respiratory sounds on the right 34 hemithorax during pulmonary auscultation. Chest radiograph showed 35 mediastinal shift toward the right side, absence of the right pulmonary 36 artery shadow, right hypoplastic lung, and hyperlucency of left lung 37 (Fig. 1). Two-dimensional transthoracic echocardiography (TTE) was 38 performed. Ejection fraction and transpulmonary artery pressure 39 (TPAP = 20) were within the reference range. Right ventricle was not 40 dilated, and right ventricle wall thickness measured from subcostal 41 view was 4.2 mm. Also, TTE revealed no structural or concomitant 42 cardiac congenital abnormalities. Afterward, pulmonary computed 43 tomography (CT) angiography was performed and revealed absence 44 of right main pulmonary artery and the presence of prominent right 45 bronchial arteries. Also, CT angiography revealed subsegmental pulmo-46 nary thromboembolism in the left pulmonary arterial vasculature 47 65 median age of diagnosis is 14 years [4]. According to the literature 66 review reported by Ten Harkel et al [4], clinical symptoms include 67 dyspnea or exercise intolerance (40%), recurrent pulmonary infections 68 (37%), and hemoptysis (20%). Pulmonary hypertension was reported 69 in 44% of patients. Asymptomatic patients are usually diagnosed after 70 abnormal chest radiograph findings [4]. 71 Diagnosis of UAPA may be very difficult because of nonspecific 72 symptoms. Physical examination is nondiagnostic except for decreased 73 respiratory sounds in the affected side during auscultation. Electrocar-74 diogram is normal in the absence of PHT. If PHT develops, right ventric-75 ular hypertrophy can be seen. 76 Usually, first clues can be obtained from chest radiograph which 77 shows absence of the pulmonary artery shadow, decreased lung 78 volume, and elevation of the diaphragm on the affected side. Shift of 79 mediastinal structures toward the affected side and contralateral 80 compensatory lung hyperinflation can be present [4,5]. Transthoracic 81 echocardiography is very useful to assess coexisting cardiac 82 malformations. Also, PHT and its severity can be evaluated during the 83 examination. Valuable information about right ventricular functions 84 can be obtained during examination. 85 Ventilation-perfusion scintigraphy is not necessary to diagnose 86 UAPA, but when it is performed, it shows complete absence of perfusion 87 and near-normal ventilation on the affected lung [5]. 88 Definite diagnosis can be made with contrasted CT scan or MRI. It 89 should be emphasized that CT angiography scan can be misinterpreted 90 as pulmonary embolism in the affected pulmonary artery. To prevent 91 this, MRI of thorax can be performed.
Heart & Lung: The Journal of Acute and Critical Care, 2009
Pulmonary artery dissection (PAD) is a rare diagnosis that is often made postmortem in patients with pulmonary hypertension. It can be visualized by echocardiography, computed tomography, or magnetic resonance imaging. We present a patient with emphysematous chronic obstructive pulmonary disease and secondary pulmonary hypertension in whom a PAD appeared like a mediastinal mass on computed tomography. The diagnosis was made at autopsy. We think that physicians should consider the possibility of a PAD in patients with chronic pulmonary hypertension who present with dyspnea and chest pain.
Histopathology, 1985
A case of pulmonary vascular occlusive disease: comparison of post-mortem radiography and histology A case of rapidly progressing fatal pulmonary hypertension in a nine-year-old boy showing histological narrowing and occlusion of pulmonary veins and arteries is described. Post-mortem radiography of the air-inflated, excised lung without use of additional contrast media nicely demonstrated the vascular lesions.
Histopathology, 2001
Aims: Pulmonary artery (PA) dissection is a rare event which usually occurs in patients with underlying pulmonary hypertension. We describe two patients who developed PA dissection without pre-existing pulmonary hypertension and present an extensive review of the literature. Methods and results: In the ®rst patient (a 59-year-old woman), acute-onset dyspnoea was initially thought to have been caused by pulmonary thrombosis, and thromboendarterectomy was performed. Histologically, pulmonary dissection without external rupture was evident, chie¯y in the right main PA. In the second patient, an 85-year-old man who had hypergamma-globulinaemia of unknown cause and died from a haemorrhagic gastric ulcer, arterial dissection was detected at autopsy. There was no underlying pulmonary hypertension in either patient. Although the true reason for the development of dissection is unclear, preexisting in¯ammation was considered to be related to its formation, at least in the second case. Conclusions: A literature review indicated that idiopathic and in¯ammation-related PA dissection is extremely unusual. Since PA dissection is very rare, it is important to be aware of its features in order to make a correct diagnosis.
The American Journal of Medicine, 1966
European Heart Journal - Case Reports
Background D-Transposition of the great arteries (d-TGA) is characterized by the aorta positioned above the right ventricle and the pulmonary artery above the left ventricle. Acute pulmonary artery dissection (PAD) is a rare and often lethal condition. We present a case report of acute PAD in an adult with d-TGA and pulmonary hypertension. Case summary A 49-year-old male with history of d-TGA palliated with an atrial switch (Mustard) operation, pulmonary venous baffle stenosis treated percutaneously, chronic pulmonary hypertension (mixed group 1 and 2), and severe dilatation of pulmonary arteries (pulmonary trunk of 75 mm) presented to the emergency department with chest pain and acute respiratory failure. Blood pressure was 106/78 mmHg, heart rate 93 b.p.m., and oxygen saturation 88% on room air. A computed tomography (CT) scan showed acute right PAD. He was not considered suitable for surgery nor percutaneous procedure. Epoprostenol was initiated to reduce parietal stress, but aft...
The American Journal of Cardiology, 1973
Mediastinal fibrosis led to complete obstruction of the left main pulmonary artery and partial stenosis of the pulmonary artery to the right lower lobe in a 48 year old man. The lesion resulted in a high pressure pulmonary vascular system with an area of prolonged runoff through a low pressure vascular bed. Physical findings included a systolic murmur that radiated to the right hemithorax and a widely split second heart sound. The wide splitting was attributed to delay in deceleration of the column of blood due to partial stenosis of the pulmonary artery to the right lower lobe.
Heart, 2005
Pulmonary arterial dissection is an extremely rare and usually lethal complication of chronic pulmonary hypertension. The condition usually manifests as cardiogenic shock or sudden death and is therefore typically diagnosed at postmortem examination rather than during life. However, recent isolated reports have described pulmonary artery dissection in surviving patients. The first case of pulmonary artery dissection in a surviving patient with cor pulmonale caused by chronic obstructive pulmonary disease is presented. The aetiology, pathophysiology, and clinical presentation of pulmonary artery dissection are reviewed and factors that may aid diagnosis during life are discussed.
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