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Query: UMLS:C0034067 (emphysema)
 11,506 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and anatomical findings in 3 patients with aberrant origin of the left pulmonary artery from the right pulmonary artery ("vascular sling") are presented. All 3 children symptoms of severe respiratory distress shortly after birth. In 2 children the correct diagnosis was suggested from the roentgenogram of the thorax, because of an indentation in the anterior wall of the esophagus. The diagnosis was further endorsed by selective angiography of the aberrant left pulmonary artery. The second patient is of particular interest, since the correct diagnosis was missed because of the presence of a multitude of associated anomalies. These included a tracheobronchial anomaly, a ventricular septal defect with a dilated pulmonary trunk, and a left-ward shift of the heart secondary to pulmonary emphysema on the right. Therefore, the indentation in the esophagus was absent, while the shift and rotation of the heart led to a misinterpretation of the exact course of the left pulmonary artery on the angiocardiogram. These cases are presented to reemphasize that "vascular sling", although rare, is indeed a serious cause of respiratory distress in infancy. Early recognition is of vital importance, since surgical repair of the vascular anomaly seems to be the only benificial procedure in these patients.
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PMID:Aberrant origin of left pulmonary artery (vascular sling). Report of the clinical and anatomic features in three patients. 112 55

Left heart diseases, in particular mitral stenosis, are often associated with anatomic and functional alterations of the lung. According to the pulmonary structures involved they could be named chronic secondary intersticial and vascular lung diseases. Congenital heart diseases with pre- or post-tricuspid shunts are also often associated with anatomic and functional alterations of the lung. This condition also constitutes a chronic secondary vascular lung disease (atrial septal defect) or a chronic primary vascular lung disease ( ventricular septal defect, patent ductus arteriosus). Primary lung diseases (interstitial pulmonary fibrosis, pulmonary emphysema, recurrent pulmonary embolism) are often associated with right ventricular hypertrophy with or without dilation, a condition commonly named chronic cor pulmonale. On the whole the interrelationships between heart and lung diseases are as follows: a) anatomic and functional alterations of the lung due to left heart diseases are mediated through pulmonary venous hypertension; b) anatomic and functional alterations of the lung due to congenital heart diseases are mediated through the increased pulmonary blood flow with or without transmission of the systemic blood pressure to the pulmonary vasculature, and c) anatomic and functional alterations of the right ventricle due to primary or secondary lung diseases are mediated through arterial pulmonary hypertension. In summary, the interrelationships between heart and lung diseases are mainly mediated through the pulmonary venous or pulmonary arterial hypertension.
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PMID:Cardiac and pulmonary diseases. A pathophysiologic interelationship. 113 Sep 7

Two infants, aged 8 and 18 months, with absent pulmonary valve syndrome unaccompanied by severe pulmonary symptoms or roentgenographic evidence of emphysema underwent a corrective operation consisting of infundibular muscle resection, patch closure for ventricular septal defect (perimembranous outlet type), and right ventricular outflow tract reconstruction with a monocusp patch. The patients tolerated pulmonary regurgitation relatively well, although aneurysmal dilatation of the pulmonary arteries was present preoperatively. Since the postoperative course was uneventful, we think that, in milder cases of this syndrome, a pulmonary valve prosthesis, the insertion of which is advocated by some authors, is not essential, and that right ventricular outflow tract reconstruction with a monocusp patch may suffice in selected cases.
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PMID:[A report of two cases of absent pulmonary valve syndrome]. 273 44

Children with acyanotic congenital heart disease frequently develop respiratory difficulties such as atelectasis, pneumonia, or infantile lobar emphysema. In some cases, the cause of the respiratory difficulty is compression of the tracheobronchial tree by hypertensive dilated pulmonary arteries, since this type of heart disease frequently demonstrates large left-to-right intracardiac shunts. Sites of predilection for compression include the left main bronchus, the left upper lobe bronchus, the junction of the right bronchus intermedius and right middle lobe bronchus, and the left side of the distal trachea. Cardiac anomalies which predispose to this type of compression include ventricular septal defect, patent ductus arteriosus, interruption of the aortic arch, and tetralogy of Fallot. Pulmonary arteriopexy may relieve the tracheobronchial compression.
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PMID:Tracheobronchial compression in acyanotic congenital heart disease. 688 45

Four cases of interruption of the aortic arch with the rare association of right ductus arteriosus and right descending aorta are described. Three different variations in subclavian arterial origin are described. Each represents a mirror image of anatomic arrangements previously reported for interruption of the aortic arch with left descending aorta. One of the cases demonstrated massive emphysema of the right lung resulting from compression of the right main bronchus by the enlarged right ductus arteriosus. Another case documented unusually long survival (26 years) of a patient with interruption of the aortic arch and ventricular septal defect.
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PMID:Interruption of the aortic arch with right descending aorta. A rare condition and a cause of bronchial compression. 708 27

Lobar emphysema is an uncommon cause of respiratory distress in infancy. Congenital heart disease is seen in about 20% of the patients with infantile (congenital) lobar emphysema. We described six infants with lobar emphysema. In three of them a congenital heart disease was demonstrated by cardiac catheterization and cineangiography; two had a tetralogy of Fallot with right aortic arch and the third infant a ventricular septal defect. The pulmonary angiography showed stretching of the arteries with very poor filling of the peripheral arteries and a characteristic smaller pulmonary vein in the affected lobe. In all the six patients the pulmonary artery pressure was normal. All the patients underwent lobectomy with good results. We feel that a preoperative cardiac catheterization and cineangiography is of value in this very sick group of infants.
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PMID:The value of cardiac catheterization and cineangiography in infantile lobar emphysema. 745 24

A combination of absence of the pulmonary valve with ventricular septal defect, annular pulmonary stenosis and aneurysm of the pulmonary artery is called "absent pulmonary valve syndrome". Most patients with this condition suffered from severe respiratory symptoms in early infancy. Respiratory failure which is the leading cause of mortality in infants with this syndrome, results from pulmonary emphysema, pulmonary atelectasis or pulmonary infection as a consequence of compression of the bronchus by the pulmonary aneurysm. If these patients can be treated medically to survive beyond one year old, gradual decrease in severity of symptoms is expected. A definite correction in childhood is recommended. Otherwise, palliative operation in infancy has a high mortality rate. This paper reported two cases with severe symptoms in infancy; one patient expired at nine months of age, and the other lived beyond one year to receive surgical correction at the age of three. The etiology, clinical features and treatment of absent pulmonary valve syndrome are also discussed.
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PMID:[Absent pulmonary valve syndrome in tetralogy of Fallot: report of two cases]. 836 72

A 1-month-old male infant with respiratory distress was referred to our hospital for operation of the ventricular septal defect. A chest roentgenogram demonstrated pulmonary emphysema especially in the right upper and middle lobes. At 3 months, a perimembranous ventricular septal defect was closed. But the infant could not be weaned from the ventilator. On the 21st postoperative day, a right upper and middle bilobectomy was performed. Three days later, he was weaned from the ventilator and the postoperative course was uneventful. The pathologic diagnosis revealed no bronchial cartilagenous abnormality. Infantile lobar emphysema of the right upper lobe with congenital heart disease is rather rare. We emphasize the need for lobectomy simultaneous with, secondary to, cardiac surgery in these cases.
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PMID:[An infant with lobar emphysema requiring lobectomy after ventricular septal defect closure]. 959 7

In 1971, the Swedish radiologist Ulf Rudhe wrote a provocative paper on right middle lobe emphysema in infants with left-to-right shunts in which he suggested cardiac surgery rather than lung resection. At the time, this was counter to accepted medical practice. Earlier diagnosis and better medical management of ventricular septal defect in infants has proved Rudhe correct. However, two current cases of large left-to-right shunts in infants with emphysema of the right middle lobe prompt this historical review of what seemed a closed-episode in pediatric cardiac surgery.
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PMID:Rudhe syndrome: reversible right middle lobe emphysema in infants with left-to-right shunts--an historical review. 2013 11

Polysplenia, or left isomerism, is a rare heterotaxy syndrome characterized by bilateral bi-lobed lungs, bilateral pulmonary atria, a symmetrical midline liver, and multiple aberrant splenic nodules. We report a case of polysplenia associated with congenital lobar emphysema apart from other typical anomalies. Such an association has not been previously reported. The patient was a young male with progressive exertional breathlessness referred for high resolution CT of the lungs. CT, MRI and echocardiography revealed (in addition to congenital lobar emphysema of right lung) a hemiazygos continuation of the inferior vena cava, a persistent left superior vena cava, multiple splenunculi in the right hypochondrium, midline liver, bilateral bilobed lungs, a large pulmonary artery (suggestive of severe pulmonary artery hypertension) and a large VSD--a typical constellation of findings described in polysplenia syndrome.
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PMID:Congenital lobar emphysema associated with polysplenia syndrome. 2086 88


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