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The American Journal of Medicine Aug 1977Clinical and morphologic observations are described in two women with primary sarcoma of the pulmonary trunk, and observations in 35 previously described patients with...
Primary sarcoma of the pulmonary trunk and/or right or left main pulmonary artery--a rare cause of obstruction to right ventricular outflow. Report on two patients and analysis of 35 previously described patients.
Clinical and morphologic observations are described in two women with primary sarcoma of the pulmonary trunk, and observations in 35 previously described patients with primary sarcoma involving a major extrapulmonary pulmonary artery are summarized. The neoplasm produces symptoms by causing obstruction to right ventricular outflow or by dislodging tumor fragments to the smaller intrapulmonary pulmonary arteries with or without pulmonary infarction. The sarcoma nearly always arises from the pulmonary trunk to which it is firmly attached. Although it grows to a large size within the lumen, it infrequently, despite its highly malignant histologic pattern, extends through the wall of the pulmonary trunk or metastasizes outside the pulmonary circulation. It may mimic a variety of more common disorders. Diagnosis can be achieved by angiography and treatment starts with total excision.
Topics: Adult; Female; Heart Ventricles; Humans; Hypertension; Middle Aged; Pulmonary Artery; Sarcoma
PubMed: 888848
DOI: 10.1016/0002-9343(77)90241-8 -
Cardiology 1997The origin of both coronary arteries from the pulmonary artery is a rare cardiac malformation. We report a baby who presented with an echocardiographically diagnosed...
The origin of both coronary arteries from the pulmonary artery is a rare cardiac malformation. We report a baby who presented with an echocardiographically diagnosed perimembranous ventricular septal defect and normal left ventricular (LV) function. Later on the boy developed failure to thrive and increasing tachypnea. At the age of 5 weeks the ECG showed that LV strain and echocardiographic LV function had worsened (FS 18%). Echocardiography and heart catheterization showed that all coronary arteries originated from the pulmonary trunk. Intraoperative inspection revealed a single ostium for the right and left coronary artery in the nonfacing sinus of the pulmonary trunk. A tube was constructed connecting the coronary artery to the ascending aorta. Coronary perfusion was sufficient and the sinus rhythm was restored. However, in the early postoperative period there was a sudden deterioration of cardiac output followed by cardiac arrest. Reanimation was not successful.
Topics: Abnormalities, Multiple; Blood Flow Velocity; Cardiac Catheterization; Coronary Angiography; Coronary Vessel Anomalies; Echocardiography, Doppler, Color; Fatal Outcome; Heart Arrest; Heart Failure; Heart Septal Defects, Ventricular; Humans; Infant, Newborn; Male; Pulmonary Artery; Stroke Volume
PubMed: 9397319
DOI: 10.1159/000177434 -
Journal of the American College of... Nov 2009The purpose of this study was to determine whether intrinsic histological abnormalities of the pulmonary trunk (PT) are present from birth and interact with palliative...
OBJECTIVES
The purpose of this study was to determine whether intrinsic histological abnormalities of the pulmonary trunk (PT) are present from birth and interact with palliative surgery and/or repair.
BACKGROUND
Little is known about PT histology in patients with tetralogy of Fallot (TOF), especially in the era of surgical intervention in childhood.
METHODS
We studied 39 formalin-fixed necropsy heart specimens with TOF and compared them with 17 normal control heart specimens. Sections of the PT and aorta were studied by light microscopy using various stains; histological findings were graded according to severity.
RESULTS
Among the TOF group (1 fetus, 11 infants, 14 children, and 13 adults), 11 patients had undergone palliative and 10 patients had undergone reparative surgery at a median age of 8 years (range 2.5 to 18 years). Histological changes of grade 2 or higher were present in 59% (medionecrosis), 36% (fibrosis), 56% (cystlike formation), and 56% (abnormal elastic tissue configuration) of TOF patients. Total histology grading scores were higher in TOF hearts (median 6, range 1 to 9) compared with controls (median 1, range 0 to 6; p < 0.0001). Histological abnormalities were present among infants (median score 3.5, range 1 to 9) and after palliative surgery (median score 5, range 2 to 9) or repair (median score 7.5, range 4 to 9).
CONCLUSIONS
Marked histological abnormalities in the PT of hearts with TOF exist compared with controls. These changes were present from infancy and among patients who had undergone palliative or reparative surgery, although operations in this cohort were performed late. Our data suggest that structural abnormalities of the PT, similar to these recently shown in the aorta, are intrinsic.
Topics: Adolescent; Adult; Aged; Aging; Aorta; Case-Control Studies; Child; Child, Preschool; Elastic Tissue; Female; Fetal Diseases; Humans; Infant; Infant, Newborn; Male; Middle Aged; Palliative Care; Pulmonary Artery; Tetralogy of Fallot; Tunica Media; Young Adult
PubMed: 19892240
DOI: 10.1016/j.jacc.2009.06.040 -
Cardiovascular Research Jan 1967
Topics: Animals; Aorta; Female; Heart Ventricles; Magnoliopsida; Organ Size; Pulmonary Artery; Pulmonary Heart Disease; Rats; Seeds
PubMed: 6060163
DOI: 10.1093/cvr/1.1.74 -
Cardiology in the Young Apr 2008Direct re-implantation of an anomalous left coronary artery into the aorta is the preferred surgical option for creating a dual coronary arterial system in patients in...
An alternative technique for transfer of anomalous left coronary artery from the pulmonary trunk in children and adults using autogenous aortic and pulmonary arterial flaps.
BACKGROUND
Direct re-implantation of an anomalous left coronary artery into the aorta is the preferred surgical option for creating a dual coronary arterial system in patients in whom the anomalous artery originated from the pulmonary trunk. This technique, however, is applicable only when the anomalous artery arises from the right posterior pulmonary sinus. We report a new technique for re-implantation using combined autogenous aortic and pulmonary arterial flaps in situations when a direct connection was not possible.
PATIENTS AND METHODS
We have treated 4 patients, aged 3 months, 6 months, 18 months, and 27 years respectively, who presented with anomalous origin of the left coronary artery from the left posterior pulmonary sinus. We used our proposed technique for transfer because lack of coronary arterial length, diminished vessel elasticity, and extensive collaterals around the pulmonary sinuses prevented direct attachment.
RESULTS
There was no early or late death. Postoperatively, all patients are in functional class I, with good biventricular function at a median follow-up of 74 months, with a range from 9 to 96 months. Postoperative coronary angiography in our 4th patient showed good arterial flow, without any distortion.
CONCLUSIONS
The potential benefits of this modification of the trapdoor technique are excellent operative exposure, use of autogenous and viable tissue capable of further growth, avoidance of injury to the aortic and pulmonary valvar apparatus and production of obstruction within the right ventricular outflow tract, complete elimination of use of pericardium for augmentation of the neo-aortic tube, achievement of the anastomosis with correct angling and length, and the possibility of implantation in all patients, including adults, regardless of the distance from the aorta or the coronary arterial configuration.
Topics: Adult; Aorta, Thoracic; Cardiac Catheterization; Cardiac Surgical Procedures; Coronary Vessel Anomalies; Humans; Infant; Patient Selection; Pulmonary Artery; Surgical Flaps; Treatment Outcome
PubMed: 18272018
DOI: 10.1017/S1047951107001898 -
The American Journal of Cardiology Oct 1995
Clinical Trial
Topics: Arterial Occlusive Diseases; Echocardiography; Echocardiography, Doppler; Female; Follow-Up Studies; Heart Defects, Congenital; Humans; Infant; Male; Postoperative Complications; Predictive Value of Tests; Pulmonary Artery; Regression Analysis; Sensitivity and Specificity
PubMed: 7572670
DOI: 10.1016/s0002-9149(99)80242-2 -
European Radiology 1996The purpose of this study was to assess the value of morphometric data on conventional radiography and CT predicting the presence and degree of pulmonary hypertension... (Comparative Study)
Comparative Study
The purpose of this study was to assess the value of morphometric data on conventional radiography and CT predicting the presence and degree of pulmonary hypertension and to assess the reversibility after surgery. On preoperative X-ray films and CT scans of 50 patients with pulmonary hypertension secondary to chronic thromboembolism, we measured the cardiothoracic ratio, basal diameter, length of cardiac contact to sternum, pulmonary trunk, right and left descending pulmonary artery, and the septum angle. These data were correlated with pulmonary arterial pressure. In 14 X-ray patients and 18 CT patients, with follow-up after surgical thromboendarterectomy the reversibility of these changes was assessed. A dilated pulmonary trunk was the most common abnormality (96% each on X-ray and CT). Pulmonary arteries were dilated on X-ray in 40% (right) and 14% (left), and on CT in 92% (right) and 96% (left). The best correlation with mean arterial pressure was found measuring the pulmonary trunk on CT (r = 0.43, p < 0.01). After surgery, reversibility was most significant for the pulmonary trunk on CT (p < 0.0001). In patients with chronic pulmonary embolism, pulmonary hypertension can best be predicted by assessing the diameter of the pulmonary trunk both on X-ray and CT. No close correlation is present between the extent of any parameter and the level of pulmonary pressure.
Topics: Adult; Aged; Blood Pressure; Chronic Disease; Embolectomy; Female; Humans; Hypertension, Pulmonary; Male; Middle Aged; Pulmonary Artery; Pulmonary Embolism; Tomography, X-Ray Computed
PubMed: 8972316
DOI: 10.1007/BF00240678 -
Arquivos Brasileiros de Cardiologia Feb 2007We report the case of an adolescent referred with initial diagnosis of pulmonary hypertension. Non-invasive investigation disclosed a sinus venous atrial septal defect...
Extrinsic compression of left main coronary artery from aneurysmal dilation of pulmonary trunk in a adolescent: involution after surgery occlusion of sinus venosus atrial septal defect and pulmonary trunk plasty for reduction.
We report the case of an adolescent referred with initial diagnosis of pulmonary hypertension. Non-invasive investigation disclosed a sinus venous atrial septal defect with pulmonary hypertension. The hemodynamic study confirmed diagnosis, and also showed extrinsic compression of left main coronary artery by pulmonary trunk. Surgical closure of the defect in addition to pulmonary trunk plasty were undertaken. Two years after the surgery the patient is well, with clinical signs of mild pulmonary hypertension, and showing no evidence--also on echocardiogram--of left coronary artery trunk obstruction.
Topics: Adolescent; Coronary Stenosis; Dilatation, Pathologic; Echocardiography; Heart Septal Defects, Atrial; Humans; Hypertension, Pulmonary; Magnetic Resonance Angiography; Male; Pulmonary Artery; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 17384825
DOI: 10.1590/s0066-782x2007000200022 -
International Journal of Cardiology Oct 2013Pulmonary arterial hypertension (PAH) is considered primarily a disease of the distal pulmonary arteries whereas little is known on the effect of long-standing pulmonary... (Comparative Study)
Comparative Study
BACKGROUND
Pulmonary arterial hypertension (PAH) is considered primarily a disease of the distal pulmonary arteries whereas little is known on the effect of long-standing pulmonary hypertension on the larger proximal pulmonary arteries. This study aims to investigate the structural changes in the great arteries of adults who developed PAH in association with congenital heart disease (CHD), with severe cases termed Eisenmenger syndrome.
METHODS
We performed macroscopic and light microscopy analyses on the great arteries of 10 formalin-fixed human hearts from patients with PAH/CHD and compared them to age-matched healthy controls. A detailed histology grading score was used to assess the severity of medial wall abnormalities.
RESULTS
Severe atherosclerotic lesions were found macroscopically in the elastic pulmonary arteries of 4 PAH/CHD specimens and organised thrombi in 3; none were present in the controls. Significant medial wall abnormalities were present in the pulmonary trunk (PT), including fibrosis (80%), and atypical elastic pattern (80%). Cyst-like formations were present in less than one third of patients and were severe in a single case leading to wall rupture. The cumulative PT histology grading score was significantly higher in PAH/CHD cases compared to controls (p<0.0001) and correlated positively with larger PT diameters (ρ=0.812, p<0.0001) and the degree of medial wall hypertrophy (ρ=0.749, p<0.0001).
CONCLUSIONS
Chronic PAH in association with CHD results in marked macroscopic and histological abnormalities in the large pulmonary arteries. These abnormalities are likely to affect haemodynamics and contribute to morbidity and mortality in this cohort.
Topics: Adult; Aged; Aged, 80 and over; Aorta, Thoracic; Eisenmenger Complex; Female; Follow-Up Studies; Humans; Hypertension, Pulmonary; Immunohistochemistry; Male; Middle Aged; Pulmonary Artery; Retrospective Studies; Young Adult
PubMed: 23453874
DOI: 10.1016/j.ijcard.2013.01.210 -
Coronary Artery Disease Mar 2024
Topics: Humans; Coronary Vessels; Tomography, X-Ray Computed; Pulmonary Artery; Coronary Vessel Anomalies
PubMed: 38164961
DOI: 10.1097/MCA.0000000000001315