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Pulmonary artery aneurysm associated with atrial septal defect and absent pulmonary valve
Pulmonary artery aneurysm associated with atrial septal defect and absent pulmonary valve Russ Zajtchuk, M.D., Lieutenant Colonel, MC, USA, * Lorenzo Gonzales-Lavin, M.D., and Robert L. Replogle, M.D., Denver, Colo., and Chicago, Ill.
.Rlmonary artery aneurysms are relatively rare lesions. Jennes' reported that he did not encounter a single case of pulmonary artery aneurysm in 37,757 consecutive autopsies. In the Mayo Clinic, the ratio of pulmonary artery aneurysm to other diseases was 1 to 17,545 necropsies." In 1939, Boyd and McGavack- collected 111 autopsy cases from literature. In 1957, Deterling and Clagettfound an additional 35 cases in the literature and added 1 of their own. More than 20 cases have since been recorded. The association of congenital absence of the pulmonary valve with aneurysm of the pulmonary artery is even less frequently encountered. A review of literature by Ruttenberg' revealed that since 1908 only 38 cases of congenital pulmonary valve insufficiency have been described. Of these 38 patients, 27 had an associated ventricular septal defect, and 20 of the 27 with ventricular septal defects also had tetralogy of Fallot. The following case of aneurysm of the pulmonary artery with absent pulmonary valve and asFrom the Thoracic Surgery Service, Fitzsimons General Hospital, Denver, Colo. 80240, and University of Chicago Hospitals and Clinics, 950 East 59th Street, Chicago, Ill. 60637. Received for publication Jan. 2, 1973. *Thoracic-eardiovascular Surgeon, Fitzsimons General Hospital, Denver, Colo. 80240.
sociated atrial septal defect is reported to emphasize the feasibility of surgical correction of this entity. Case report A 20-year-old Negro man was admitted for evaluation of a cardiac murmur. He had been asymptomatic. On physical examination, the patient was a normally developed Negro man with a blood pressure of 100/80 mm. Hg and a pulse of 60 beats per minute. There was a thrill over the second left intercostal space and a Grade 4/6 systolic ejection murmur, localized to the second intercostal space at the left sternal border. A Grade 2/6 diastolic decrescendo murmur was audible over the left second intercostal space. The diastolic murmur occurred after a time interval at the end of the systolic ejection murmur and was of moderately long duration (0.06 second). Chest x-ray study revealed slight cardiomegaly with marked prominence of the main pulmonary artery (Fig. I). There was electrocardiographic evidence of sinus rhythm with first degree atrioventricular block and right ventricular hypertrophy. Cardiac catheterization disclosed a right atrial pressure of 4 mm. Hg, a right ventricular pressure of 35/4 mm. Hg, and a pulmonary artery pressure of 25/4 mm. Hg, The pulmonary capillary mean pressure was 5 rnm. Hg. Angiography revealed a greatly enlarged pulmonary artery with massive regurgitation into the right ventricle (Fig. 2). There was oxygen step-up at the right atrial level from 74 to 85 per cent saturation, which suggested an atrial septal defect.
699
The Journol 01
700
Zajtchuk, Gonzales-Lavin, Replogle
Thoracic and Card iovascular Surgery
Fig. 1. Chest roentgenogram showing marked prominence of the main pulmonary artery.
Fig. 3. Aneurysm of the pulmonary artery prior to resection. The patient had an uneventful recovery and is doing well 3 years postoperatively. Microscopic examination of the resected aneurysm revealed fibromusculoelastic thickening of the intima overlying the media . There was an increased amount of collagen in the media. Portions of the specimen resembled normal pulmonary artery tissue.
Fig. 2. Angiogram demonstrating massive regurgitation into the right ventricle (RV). Arrows point to the pulmonary valve region. PA, Pulmonary artery. On May 6, 1970, the patient was operated upon. Exploration revealed a large pulmonary artery aneurysm measuring 8 em. in diameter (Fig. 3). With extracorporeal circulation, the atrial septal defect was closed, the aneurysm was partially resected, and the pulmonary valve was replaced with a homograft (Fig. 4) .
Discussion The causes of aneurysm of the pulmonary artery include the following: (1) congenital cardiovascular anomalies; (2) acquired pulmonary hypertension of extravascular etiology; (3) destructive process of the arterial wall ; and (4) idiopathic etiology. Clinical symptoms of pulmonary artery aneurysm may include dyspnea, cyanosis, edema, cough, hemoptysis, and pain in the chest. 2 Fever is added in the case of mycotic aneurysms." Sometimes the course is asymptomatic, as in our patient. The diagnosis of pulmonary artery aneurysm is based on clinical findings suggestive of a large pulmonary
Volume 65
Pulmonary artery aneurysm
Number 5
701
Moy, 1973
life, the combination of congenital anomalies and a pulmonary artery aneurysm is potentially a lethal disease and should be treated surgically. Surgical correction should include partial resection of the pulmonary aneurysm and correction of intracardiac defects with the use of cardiopulmonary bypass. In cases in which the pulmonary valve is absent, it should be replaced by a homograft, as was done in our patient. Summary A case of pulmonary artery anuerysm associated with absent pulmonary valve and atrial septal defect is presented. Surgical correction is recommended in all cases of pulmonary artery aneurysm, in particular when other congenital defects are involved. The correction should be done with the aid of cardiopulmonary bypass. If the pulmonary valve is absent, it should be replaced with a homograft. REFERENCES
Fig. 4. The pulmonary artery aneurysm has been partially resected, and the pulmonary valve region has been replaced with a homograft.
artery, with or without evidence of pulmonary incompetence, and on the radiologic appearance . of the artery confirmed by angiography. The prognosis is poor, the average age at death being 42.8 years. " Right heart failure and rupture of the aneurysm are the two major complications. Although isolated idiopathic pulmonary artery aneurysms and isolated congenital pulmonary valve insufficiency are well tolerated and may be compatible with cardiac competence until adult
2
3 4
5
Jennes, S. W.: Aneurysm of the Pulmonary Artery and Both of Its Branches , Johns Hopkins Med. J. 59: 133, 1936. Deterling, R. J., and Clagett, O. T.: Aneurysm of the Pulmonary Artery : Review of the Literature and Report of a Case, Am . Heart J. 34: 471, 1947. Boyd, L. J., and McGavack, T. H.: Aneurysm of the Pulmonary Artery, Am. Heart J. 18: 562, 1939. Ruttenberg, H. D., Carey, L. S., Adams, P., Jr., and Edwards, J. E.: Absence of the Pulmonary Valve in Tetralogy of Fallot, Am. J. Roentgenol. Radium Ther. Nucl. Med. 91: 500, 1964. Jacobson, K. A., Koch , G., and Liliequist, B.: Multiple Pulmonary Artery Aneurysms, Acta Med. Scand . 179: 673, 1966.
.Rlmonary artery aneurysms are relatively rare lesions. Jennes' reported that he did not encounter a single case of pulmonary artery aneurysm in 37,757 consecutive autopsies. In the Mayo Clinic, the ratio of pulmonary artery aneurysm to other diseases was 1 to 17,545 necropsies." In 1939, Boyd and McGavack- collected 111 autopsy cases from literature. In 1957, Deterling and Clagettfound an additional 35 cases in the literature and added 1 of their own. More than 20 cases have since been recorded. The association of congenital absence of the pulmonary valve with aneurysm of the pulmonary artery is even less frequently encountered. A review of literature by Ruttenberg' revealed that since 1908 only 38 cases of congenital pulmonary valve insufficiency have been described. Of these 38 patients, 27 had an associated ventricular septal defect, and 20 of the 27 with ventricular septal defects also had tetralogy of Fallot. The following case of aneurysm of the pulmonary artery with absent pulmonary valve and asFrom the Thoracic Surgery Service, Fitzsimons General Hospital, Denver, Colo. 80240, and University of Chicago Hospitals and Clinics, 950 East 59th Street, Chicago, Ill. 60637. Received for publication Jan. 2, 1973. *Thoracic-eardiovascular Surgeon, Fitzsimons General Hospital, Denver, Colo. 80240.
sociated atrial septal defect is reported to emphasize the feasibility of surgical correction of this entity. Case report A 20-year-old Negro man was admitted for evaluation of a cardiac murmur. He had been asymptomatic. On physical examination, the patient was a normally developed Negro man with a blood pressure of 100/80 mm. Hg and a pulse of 60 beats per minute. There was a thrill over the second left intercostal space and a Grade 4/6 systolic ejection murmur, localized to the second intercostal space at the left sternal border. A Grade 2/6 diastolic decrescendo murmur was audible over the left second intercostal space. The diastolic murmur occurred after a time interval at the end of the systolic ejection murmur and was of moderately long duration (0.06 second). Chest x-ray study revealed slight cardiomegaly with marked prominence of the main pulmonary artery (Fig. I). There was electrocardiographic evidence of sinus rhythm with first degree atrioventricular block and right ventricular hypertrophy. Cardiac catheterization disclosed a right atrial pressure of 4 mm. Hg, a right ventricular pressure of 35/4 mm. Hg, and a pulmonary artery pressure of 25/4 mm. Hg, The pulmonary capillary mean pressure was 5 rnm. Hg. Angiography revealed a greatly enlarged pulmonary artery with massive regurgitation into the right ventricle (Fig. 2). There was oxygen step-up at the right atrial level from 74 to 85 per cent saturation, which suggested an atrial septal defect.
699
The Journol 01
700
Zajtchuk, Gonzales-Lavin, Replogle
Thoracic and Card iovascular Surgery
Fig. 1. Chest roentgenogram showing marked prominence of the main pulmonary artery.
Fig. 3. Aneurysm of the pulmonary artery prior to resection. The patient had an uneventful recovery and is doing well 3 years postoperatively. Microscopic examination of the resected aneurysm revealed fibromusculoelastic thickening of the intima overlying the media . There was an increased amount of collagen in the media. Portions of the specimen resembled normal pulmonary artery tissue.
Fig. 2. Angiogram demonstrating massive regurgitation into the right ventricle (RV). Arrows point to the pulmonary valve region. PA, Pulmonary artery. On May 6, 1970, the patient was operated upon. Exploration revealed a large pulmonary artery aneurysm measuring 8 em. in diameter (Fig. 3). With extracorporeal circulation, the atrial septal defect was closed, the aneurysm was partially resected, and the pulmonary valve was replaced with a homograft (Fig. 4) .
Discussion The causes of aneurysm of the pulmonary artery include the following: (1) congenital cardiovascular anomalies; (2) acquired pulmonary hypertension of extravascular etiology; (3) destructive process of the arterial wall ; and (4) idiopathic etiology. Clinical symptoms of pulmonary artery aneurysm may include dyspnea, cyanosis, edema, cough, hemoptysis, and pain in the chest. 2 Fever is added in the case of mycotic aneurysms." Sometimes the course is asymptomatic, as in our patient. The diagnosis of pulmonary artery aneurysm is based on clinical findings suggestive of a large pulmonary
Volume 65
Pulmonary artery aneurysm
Number 5
701
Moy, 1973
life, the combination of congenital anomalies and a pulmonary artery aneurysm is potentially a lethal disease and should be treated surgically. Surgical correction should include partial resection of the pulmonary aneurysm and correction of intracardiac defects with the use of cardiopulmonary bypass. In cases in which the pulmonary valve is absent, it should be replaced by a homograft, as was done in our patient. Summary A case of pulmonary artery anuerysm associated with absent pulmonary valve and atrial septal defect is presented. Surgical correction is recommended in all cases of pulmonary artery aneurysm, in particular when other congenital defects are involved. The correction should be done with the aid of cardiopulmonary bypass. If the pulmonary valve is absent, it should be replaced with a homograft. REFERENCES
Fig. 4. The pulmonary artery aneurysm has been partially resected, and the pulmonary valve region has been replaced with a homograft.
artery, with or without evidence of pulmonary incompetence, and on the radiologic appearance . of the artery confirmed by angiography. The prognosis is poor, the average age at death being 42.8 years. " Right heart failure and rupture of the aneurysm are the two major complications. Although isolated idiopathic pulmonary artery aneurysms and isolated congenital pulmonary valve insufficiency are well tolerated and may be compatible with cardiac competence until adult
2
3 4
5
Jennes, S. W.: Aneurysm of the Pulmonary Artery and Both of Its Branches , Johns Hopkins Med. J. 59: 133, 1936. Deterling, R. J., and Clagett, O. T.: Aneurysm of the Pulmonary Artery : Review of the Literature and Report of a Case, Am . Heart J. 34: 471, 1947. Boyd, L. J., and McGavack, T. H.: Aneurysm of the Pulmonary Artery, Am. Heart J. 18: 562, 1939. Ruttenberg, H. D., Carey, L. S., Adams, P., Jr., and Edwards, J. E.: Absence of the Pulmonary Valve in Tetralogy of Fallot, Am. J. Roentgenol. Radium Ther. Nucl. Med. 91: 500, 1964. Jacobson, K. A., Koch , G., and Liliequist, B.: Multiple Pulmonary Artery Aneurysms, Acta Med. Scand . 179: 673, 1966.