Pulmonary endarteritis
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《美国医学杂志》
1 Department of Pathology (Cardiovascular & Thoracic Division), Seth .S. Medical College & K.E.M. Hospital, Mumbai, India
2 Department of Pediatrics, Seth G.S. Medical College & K.E.M. Hospital, Mumbai, India
Pulmonary arterial endarteritis is a rare event even in patients with congenital heart disease. In this paper, the authors report two cases of pulmonary endarteritis diagnosed at autopsy. The first was a 3-month-old male infant with absence of congenital heart defect (who had had neonatal sepsis). This patient presented with pneumonia and succumbed within 36 hours of hospital stay despite appropriate antimicrobial therapy and supportive management. The second patient was a 13-month-old male child with previously undiagnosed tetralogy of Fallot who had clinical presentation of acute meningitis with convulsions and succumbed within 12 hours of hospital stay despite adequate treatment. The main autopsy findings were chronic arteritis of the pulmonary trunk and right pulmonary artery in the former and rupture of the pulmonary trunk in the latter.
Keywords: Endarteritis; Heart; Pulmonary artery; Right-sided infective endocarditis
Right-sided infective endocarditis is rare by virtue of the low hemodynamic pressure and lack of isolated or significant right-sided valvar deformities. The valves fall prey to an infective process in settings of congenital heart disease or endothelial injury.[1] Involvement of the main pulmonary artery with subsequent pulmonary infarction is unusual.[2] In this paper, the authors describe two cases, in which pulmonary endarteritis was diagnosed at autopsy.
Case Report
Case 1
A three-month-old male child, born from a non-consanguineous marriage was admitted with shortness of breath, cough and difficulty in feeding for five days. The child, delivered by a lower segment cesarean section, had a prior admission in a neonatal intensive care unit for three weeks for meconium aspiration syndrome and sepsis; details were not available. The child weighed 3.2 Kg., was irritable, tachycardic and tachypneic. Ejection systolic murmur in the pulmonary area, bilateral supra-mammary crepitations and mild hepatosplenomegaly were the systemic findings. Investigations performed revealed anemia, bilateral pulmonary infiltrations in the upper zones and prominent pulmonary trunk. The child was suspected to have acyanotic congenital heart disease and was treated with oxygen therapy, intravenous fluids and antibiotics. He had cardiac arrest after 20 hours of admission, necessitating the use of intravenous adrenaline, dopamine drip and mechanical ventilation. His condition deteriorated and the child died after 36 hours of admission.
At autopsy, there was moderate cardiomegaly (heart weight 30 gm). The left posterior cusp of the pulmonary valve was destroyed by large, red-brown fleshy vegetation with polypoidal extension into the infundibular chamber. The vegetation also involved the posterior aspect of the dilated pulmonary trunk [Figure - 1] and occluded the, entire right pulmonary artery from its origin until the hilum of the right lung. Small mural vegetations were present just distal to the origin of the left pulmonary artery. The ductus arteriosus was closed and there was no congenital heart defect. Microscopically, the vegetation showed clumps of fibrin, mixed with neutrophils and red cells. No organisms were identified on special stains. Both extra-pulmonary arteries revealed chronic arteritis [Figure - 2]. The right lung had arterial thrombo-emboli with arteritic changes and cystic infarcts in the posterior segment of the right upper lobe and medial segment of the middle lobe.
Case 2
A thirteen-month-old male child, diagnosed as a case of pyogenic meningitis in a nursing home, was referred to the authors emergency services centre for right-sided focal convulsions. On examination, the child was pale with a pulse rate of 110/minute,respiratory rate of 30/minute and blood pressure of 92/64 mm of Hg. Central nervous system examination revealed abnormal sensorium with loss of parental recognition, hypotonia, extensor plantar responses and brisk right knee deep tendon reflex. Fundus examination was normal. Auscultation revealed murmur in pulmonary area and pansysytolic murmur in left parasternal area. Clinical impression was pyogenic meningitis with associated congenital heart disease. Electrocardiography revealed right ventricular hypertrophy. Computed tomographic scan of brain done at authors institution showed effacement of cerebral cortical sulci, post-contrast sulcal enhancement and exudates along the sylvian fissures, suggesting meningitis. The child worsened clinically despite treatment with intravenous ceftriaxone, mannitol, phenytoin, phenobarbitone, dexamethasone and intravenous fluids and succumbed within 12 hours of hospital stay.
At autopsy, there was moderate cardiomegaly (weight 70 gm). On incising the pericardium, about 14 ml of serosanguineous fluid was drained. A 5 gm blood clot was present over the right ventricular outflow tract. After its removal, a 0.2 cm circular and ragged perforation with an irregular hyperemic border was seen over the pulmonary trunk [Figure - 3]. The artery was found to be occluded in its entire length by friable, pale yellow to light brown thrombus with extension into the right branch. An easily detachable similar thrombotic material was present in the left pulmonary artery. Gram-positive colonies were identified on histology. The heart revealed features of Fallot's tetralogy with a non-restrictive perimembranous ventricular septal defect, mild infundibular stenosis and aortic overriding. The bicuspid pulmonary valve was devoid of vegetations. A probe patent ductus arteriosus was also present. Classic features of pyogenic meningitis were also encountered.
Discussion
In adults, right-sided infective endocarditis usually follows prolonged intravenous catheterization or intravenous drug-abuse, affecting the tricuspid valve alone or in combination with the pulmonary valve. A similar valvar involvement also occurs in infants and children, but here congenital cardiac anomalies with their accompanying interventions are important pre-disposing factors.[3] Involvement of the pulmonary valve in isolation is distinctly uncommon in both adults and children.[4] In the authors unpublished tenyear data of 128 autopsied cases of fatal native valvar endocarditis, isolated pulmonary valvar endocarditis was seen in ten patients (27%).
Adding insult to the injury, the vegetations can also be found in the main, right and left pulmonary arteries as extension from the pulmonary valve or occurrence in isolation. Pulmonary endarteritis has been previously described in five case reports.[2],[5],[6],[7],[8] Of the five, patent ductus arteriosus was present in four as the only anomaly or combined with other cardiac defects, while the fifth developed vegetations after corrective surgery for transposition of great arteries. Infective endocarditis is a known complication in the natural history of patent ductus arteriosus.[2] The ductus was patent in the child with Fallot's tetralogy (Case 2). There were no vegetations on the pulmonary valve. The thrombus formed in the pulmonary trunk itself, where it produced a hitherto unreported complication in the form of perforation and subsequent hemopericardium. The zone of perforation had ragged margins with broad hyperemic rim; these features would help to differentiate from the perforation produced by cardiopulmonary resuscitative measures. Case 1 had both valvar and arterial involvement but surprisingly no predisposing factor, which can occur at times.[9] Endarteritis in the right pulmonary artery must have been present for a while, given the extension of the inflammatory vascular changes with significant narrowing of the vessel. We also failed to demonstrate microorganisms on special stains. There was also thrombo-embolism with pulmonary multifocal cavitary infarcts.
Both the patients reported here had a short hospital survival and it was not possible to perform color doppler examination as they were not hemodynamically stable for transport to the color doppler department and due to non-availability of portable echocardiography machine. Also surprisingly, Case 2 did not manifest with cyanosis or clubbing but was clinically diagnosed as pyogenic meningitis with congenital heart disease. Early detection of the condition by color doppler examination could have helped in instituting appropriate measures in both the patients reported. Although right-sided endocarditis/ endarteritis is rare, it should be considered in patients presenting with signs of systemic infection and a cardiac murmur. Whether the early recognition of this condition by echo cardiography could have changed the course of the disease remains speculative.
Acknowledgements
The authors thank Dr. N.A. Kshirsagar, Dean, Seth G.S. Medical College & KEM Hospital, Mumbai for granting permission to publish this manuscript.
References
1.van der Westhuizen NG, Rose AG. Right-sided valvular infective endocarditis. A clinicopathological study of 29 patients. S Afr Med J 1987; 71; 25-27.
2.Bilge M, Uner A, Ozeren A, Aydin M, Demirel F, Ermis B, Ozkokeli M. Pulmonary endarteritis and subsequent embolization to the lung as a complication of a patent ductus arteriosus-a case report. Angiology 2004; 55: 99-102. [PUBMED]
3.Picarelli D, Lenone R, Duhagon P, Peluffo C, Zuniga C, Gelos S, Canessa R, Nozar JV. Active infective endocarditis in infancy and childhood: ten-year review of surgical therapy. J Card Surg 1997; 12: 406-411.
4.Sharma S, Desai AG, Pillai MG, Kinare SG, Pandey SR. Clinical and diagnostic features of pulmonary valve endocarditis in the setting of congenital cardiac malformations. Int J Cardiol 1985; 9: 457-464.
5.Vargas-Barron J, Attie F, Buendia-Hernandez A, Keirns C, Esquivel-Avila J. Echocardiographic recognition of pulmonary endarteritis in patent ductus arteriosus. Am Heart J 1985; 109: 368-370. [PUBMED] [FULLTEXT]
6.Chia BL, Ee BK, Choo MH, Tan NC. Two-dimensional echocardiographic features of pulmonary artery vegetation. Am Heart J 1987; 113: 833-834. [PUBMED]
7.Parsons JM, Martin RP, Smith RR. Infective endarteritis affecting the left pulmonary artery after anatomical correction of complete transposition of the great arteries. Br Heart Journal 11988; 60: 78-80.
8.Farsak B, Yilmaz M, Oc M, Ozkullu S, Demircin M. Non-valvular isolated pulmonary artery vegetations. Med Sci Monit 2002; 8: CS 39-41.
9.Fukaya Y, Iida F, Yanagiya N, Nobara H, Nisimura K, Nakano H, Morimoto M. Isolated pulmonary valve endocarditis without cardiac anomaly in an infant. Cardiovasc Surg 1993; 1 : 605-606. [PUBMED](Vaideeswar Pradeep, Tullu Milind S, Lahi)
2 Department of Pediatrics, Seth G.S. Medical College & K.E.M. Hospital, Mumbai, India
Pulmonary arterial endarteritis is a rare event even in patients with congenital heart disease. In this paper, the authors report two cases of pulmonary endarteritis diagnosed at autopsy. The first was a 3-month-old male infant with absence of congenital heart defect (who had had neonatal sepsis). This patient presented with pneumonia and succumbed within 36 hours of hospital stay despite appropriate antimicrobial therapy and supportive management. The second patient was a 13-month-old male child with previously undiagnosed tetralogy of Fallot who had clinical presentation of acute meningitis with convulsions and succumbed within 12 hours of hospital stay despite adequate treatment. The main autopsy findings were chronic arteritis of the pulmonary trunk and right pulmonary artery in the former and rupture of the pulmonary trunk in the latter.
Keywords: Endarteritis; Heart; Pulmonary artery; Right-sided infective endocarditis
Right-sided infective endocarditis is rare by virtue of the low hemodynamic pressure and lack of isolated or significant right-sided valvar deformities. The valves fall prey to an infective process in settings of congenital heart disease or endothelial injury.[1] Involvement of the main pulmonary artery with subsequent pulmonary infarction is unusual.[2] In this paper, the authors describe two cases, in which pulmonary endarteritis was diagnosed at autopsy.
Case Report
Case 1
A three-month-old male child, born from a non-consanguineous marriage was admitted with shortness of breath, cough and difficulty in feeding for five days. The child, delivered by a lower segment cesarean section, had a prior admission in a neonatal intensive care unit for three weeks for meconium aspiration syndrome and sepsis; details were not available. The child weighed 3.2 Kg., was irritable, tachycardic and tachypneic. Ejection systolic murmur in the pulmonary area, bilateral supra-mammary crepitations and mild hepatosplenomegaly were the systemic findings. Investigations performed revealed anemia, bilateral pulmonary infiltrations in the upper zones and prominent pulmonary trunk. The child was suspected to have acyanotic congenital heart disease and was treated with oxygen therapy, intravenous fluids and antibiotics. He had cardiac arrest after 20 hours of admission, necessitating the use of intravenous adrenaline, dopamine drip and mechanical ventilation. His condition deteriorated and the child died after 36 hours of admission.
At autopsy, there was moderate cardiomegaly (heart weight 30 gm). The left posterior cusp of the pulmonary valve was destroyed by large, red-brown fleshy vegetation with polypoidal extension into the infundibular chamber. The vegetation also involved the posterior aspect of the dilated pulmonary trunk [Figure - 1] and occluded the, entire right pulmonary artery from its origin until the hilum of the right lung. Small mural vegetations were present just distal to the origin of the left pulmonary artery. The ductus arteriosus was closed and there was no congenital heart defect. Microscopically, the vegetation showed clumps of fibrin, mixed with neutrophils and red cells. No organisms were identified on special stains. Both extra-pulmonary arteries revealed chronic arteritis [Figure - 2]. The right lung had arterial thrombo-emboli with arteritic changes and cystic infarcts in the posterior segment of the right upper lobe and medial segment of the middle lobe.
Case 2
A thirteen-month-old male child, diagnosed as a case of pyogenic meningitis in a nursing home, was referred to the authors emergency services centre for right-sided focal convulsions. On examination, the child was pale with a pulse rate of 110/minute,respiratory rate of 30/minute and blood pressure of 92/64 mm of Hg. Central nervous system examination revealed abnormal sensorium with loss of parental recognition, hypotonia, extensor plantar responses and brisk right knee deep tendon reflex. Fundus examination was normal. Auscultation revealed murmur in pulmonary area and pansysytolic murmur in left parasternal area. Clinical impression was pyogenic meningitis with associated congenital heart disease. Electrocardiography revealed right ventricular hypertrophy. Computed tomographic scan of brain done at authors institution showed effacement of cerebral cortical sulci, post-contrast sulcal enhancement and exudates along the sylvian fissures, suggesting meningitis. The child worsened clinically despite treatment with intravenous ceftriaxone, mannitol, phenytoin, phenobarbitone, dexamethasone and intravenous fluids and succumbed within 12 hours of hospital stay.
At autopsy, there was moderate cardiomegaly (weight 70 gm). On incising the pericardium, about 14 ml of serosanguineous fluid was drained. A 5 gm blood clot was present over the right ventricular outflow tract. After its removal, a 0.2 cm circular and ragged perforation with an irregular hyperemic border was seen over the pulmonary trunk [Figure - 3]. The artery was found to be occluded in its entire length by friable, pale yellow to light brown thrombus with extension into the right branch. An easily detachable similar thrombotic material was present in the left pulmonary artery. Gram-positive colonies were identified on histology. The heart revealed features of Fallot's tetralogy with a non-restrictive perimembranous ventricular septal defect, mild infundibular stenosis and aortic overriding. The bicuspid pulmonary valve was devoid of vegetations. A probe patent ductus arteriosus was also present. Classic features of pyogenic meningitis were also encountered.
Discussion
In adults, right-sided infective endocarditis usually follows prolonged intravenous catheterization or intravenous drug-abuse, affecting the tricuspid valve alone or in combination with the pulmonary valve. A similar valvar involvement also occurs in infants and children, but here congenital cardiac anomalies with their accompanying interventions are important pre-disposing factors.[3] Involvement of the pulmonary valve in isolation is distinctly uncommon in both adults and children.[4] In the authors unpublished tenyear data of 128 autopsied cases of fatal native valvar endocarditis, isolated pulmonary valvar endocarditis was seen in ten patients (27%).
Adding insult to the injury, the vegetations can also be found in the main, right and left pulmonary arteries as extension from the pulmonary valve or occurrence in isolation. Pulmonary endarteritis has been previously described in five case reports.[2],[5],[6],[7],[8] Of the five, patent ductus arteriosus was present in four as the only anomaly or combined with other cardiac defects, while the fifth developed vegetations after corrective surgery for transposition of great arteries. Infective endocarditis is a known complication in the natural history of patent ductus arteriosus.[2] The ductus was patent in the child with Fallot's tetralogy (Case 2). There were no vegetations on the pulmonary valve. The thrombus formed in the pulmonary trunk itself, where it produced a hitherto unreported complication in the form of perforation and subsequent hemopericardium. The zone of perforation had ragged margins with broad hyperemic rim; these features would help to differentiate from the perforation produced by cardiopulmonary resuscitative measures. Case 1 had both valvar and arterial involvement but surprisingly no predisposing factor, which can occur at times.[9] Endarteritis in the right pulmonary artery must have been present for a while, given the extension of the inflammatory vascular changes with significant narrowing of the vessel. We also failed to demonstrate microorganisms on special stains. There was also thrombo-embolism with pulmonary multifocal cavitary infarcts.
Both the patients reported here had a short hospital survival and it was not possible to perform color doppler examination as they were not hemodynamically stable for transport to the color doppler department and due to non-availability of portable echocardiography machine. Also surprisingly, Case 2 did not manifest with cyanosis or clubbing but was clinically diagnosed as pyogenic meningitis with congenital heart disease. Early detection of the condition by color doppler examination could have helped in instituting appropriate measures in both the patients reported. Although right-sided endocarditis/ endarteritis is rare, it should be considered in patients presenting with signs of systemic infection and a cardiac murmur. Whether the early recognition of this condition by echo cardiography could have changed the course of the disease remains speculative.
Acknowledgements
The authors thank Dr. N.A. Kshirsagar, Dean, Seth G.S. Medical College & KEM Hospital, Mumbai for granting permission to publish this manuscript.
References
1.van der Westhuizen NG, Rose AG. Right-sided valvular infective endocarditis. A clinicopathological study of 29 patients. S Afr Med J 1987; 71; 25-27.
2.Bilge M, Uner A, Ozeren A, Aydin M, Demirel F, Ermis B, Ozkokeli M. Pulmonary endarteritis and subsequent embolization to the lung as a complication of a patent ductus arteriosus-a case report. Angiology 2004; 55: 99-102. [PUBMED]
3.Picarelli D, Lenone R, Duhagon P, Peluffo C, Zuniga C, Gelos S, Canessa R, Nozar JV. Active infective endocarditis in infancy and childhood: ten-year review of surgical therapy. J Card Surg 1997; 12: 406-411.
4.Sharma S, Desai AG, Pillai MG, Kinare SG, Pandey SR. Clinical and diagnostic features of pulmonary valve endocarditis in the setting of congenital cardiac malformations. Int J Cardiol 1985; 9: 457-464.
5.Vargas-Barron J, Attie F, Buendia-Hernandez A, Keirns C, Esquivel-Avila J. Echocardiographic recognition of pulmonary endarteritis in patent ductus arteriosus. Am Heart J 1985; 109: 368-370. [PUBMED] [FULLTEXT]
6.Chia BL, Ee BK, Choo MH, Tan NC. Two-dimensional echocardiographic features of pulmonary artery vegetation. Am Heart J 1987; 113: 833-834. [PUBMED]
7.Parsons JM, Martin RP, Smith RR. Infective endarteritis affecting the left pulmonary artery after anatomical correction of complete transposition of the great arteries. Br Heart Journal 11988; 60: 78-80.
8.Farsak B, Yilmaz M, Oc M, Ozkullu S, Demircin M. Non-valvular isolated pulmonary artery vegetations. Med Sci Monit 2002; 8: CS 39-41.
9.Fukaya Y, Iida F, Yanagiya N, Nobara H, Nisimura K, Nakano H, Morimoto M. Isolated pulmonary valve endocarditis without cardiac anomaly in an infant. Cardiovasc Surg 1993; 1 : 605-606. [PUBMED](Vaideeswar Pradeep, Tullu Milind S, Lahi)