Case Report
Pulmonary Artery Aneurysm With Patent Arterial Duct: Resection of Aneurysm and Ductal Division
World Journal for Pediatric and Congenital Heart Surgery 4(4) 427-429 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113494403 pch.sagepub.com
Endale Tefera, MD1 and Michael Teodori, MD2
Abstract Congenital or acquired aneurysm of the pulmonary artery (PA) is rare. Although aneurysms are described following surgical treatment of patent ductus arteriosus (PDA), occurrence of this lesion in association with PDA without previous surgery is extremely uncommon. An eight-year-old patient with PDA and aneurysm of the main PA is described in this report. Clinical diagnosis of PDA was made upon presentation. Diagnosis of PA aneurysm was suspected on chest x-ray and was confirmed on transthoracic echocardiography. Successful surgical resection of the aneurysm and division of the duct were performed under cardiopulmonary bypass. The patient did well on follow-up both from clinical and echocardiographic point of view. Keywords pulmonary artery aneurysm, patent ductus arteriosus, ductal division, aneurysm resection Submitted February 26, 2013; Accepted May 28, 2013.
Introduction Pulmonary artery (PA) aneurysms, whether congenital or acquired, are rare with reported autopsy incidence of about 1 in 14,000.1,2 The PA aneurysms occur as isolated entities or could be associated with congenital or acquired cardiovascular lesions, like patent ductus arteriosus (PDA) or infective endocarditis.2 Here, we report a case of pulmonary trunk aneurysm occurring with a large PDA and its successful surgical resection and division of the duct.
Case The case was an eight-year-old girl who presented to our hospital for the first time with palpitation and easy fatigability which was progressive in the preceding four years. In retrospect, the patient had symptoms dating back to early infancy with interruption of breast feeding, diaphoresis, failure to gain weight, and recurrent respiratory tract infections. She was not on any medication and did not have previous admission, although she had been treated for respiratory tract infection on an outpatient basis in her locality. She had no documented infective endocarditis history. On physical examination, she was chronically sick looking. She weighed 20 kg, and her height was 114 cm. Blood pressure on her left upper arm was 120/50 mm Hg. Her peripheral pulses were bounding. She has a bulged and active precordium with point of maximal impulse in the left fifth intercostals space lateral to the midclavicular line. She had a thrill and continuous murmur over the left second intercostals space. Liver
Figure 1. Preoperative chest x-ray showing pulmonary artery aneurysm.
1 Department of Pediatrics & Child Health, Cardiology Unit, School of Medicine, Addis Ababa University & Cardiac Center, Addis Ababa, Ethiopia 2 Pediatric and Adult Congenital Heart Surgery, University of Arizona Department of Surgery, Tucson, AZ, USA
Corresponding Author: Endale Tefera, Department of Pediatrics & Child Health, Cardiology Unit, School of Medicine, Addis Ababa University & Cardiac Center, Corner of Zambia & T. Abanefso Roads, PO Box 1768, Addis Ababa, Ethiopia. Email: [email protected]
428
World Journal for Pediatric and Congenital Heart Surgery 4(4)
Figure 2. 2D and color flow echo frames showing patent ductus arteriosus (PDA) and pulmonary artery (PA) aneurysm.
was just palpable below the right costal margin, but spleen was not palpable. Plain chest x-ray showed modest cardiac enlargement and moderately increased pulmonary blood flow. There was a bulging mass in the area of the main PA (Figure 1). Electrocardiogram showed sinus rhythm with signs of left atrial enlargement and left ventricular enlargement. Echocardiography showed dilated left atrium, left ventricle, and PA. There was a 7-mm PDA with a continuous left-to-right shunt. There was a saccular aneurysm arising from the distal part of the main PA just proximal to the point of bifurcation, which measured 7 7 cm (Figure 2). Its opening (communication with the main PA) measured 1.2 cm. Most part of the ductal flow was directed toward the aneurysm. There was no significant pulmonary hypertension on echocardiography. With this information, the patient was explored through a median sternotomy. Moderate pericardial adhesions were divided. The aneurysm arose from the distal anterior wall of the main PA, measured 7 cm across, and obscured exposure to the PDA. Cardiopulmonary bypass was initiated to minimize risk because of the limited exposure. On reduced flow, the duct was quickly exposed and doubly clamped, nicely decompressing the pulmonary aneurysm. Full flows on bypass were resumed while the duct was divided and both the ends over sewn. The aneurysm wall was then resected, and there was no indication of infection or other pathology on gross appearance. The edges of the PA were reapproximated. The child was readily weaned off bypass and was extubated in the operating theater. Total bypass time was 13 minutes. The patient had an uneventful intensive care unit stay. She is doing well in follow-up in terms of clinical and echocardiographic parameters. Follow-up chest x-ray taken a week after the surgery is shown in Figure 3.
Discussion PA aneurysm in the setting of PDA has been described in different reports. However, when aneurysm of the PA occurs with
Figure 3. Postoperative chest x-ray taken after 1-week.
PDA, it commonly occurs in association with severe pulmonary arterial hypertension. Histologic examinations of such patients have also shown that cystic medial necrosis was a prominent feature.3,4 In our patient, there was a continuous murmur on physical examination. Two-dimensional echocardiography revealed continuous left-to-right shunt from aorta to PA, with a significant pressure gradient (maximum Doppler measured velocity of the ductal jet in systole was 5 m/s), which indicates that there was no significant pulmonary arterial hypertension. Mortality rate for ruptured aneurysm of the PA is 100%.5 There are controversies regarding the treatment of PA aneurysms. Some authors recommend surgical treatment when a main PA aneurysm is discovered, irrespective of etiology or absence of pulmonary hypertension, because there is a potential for rupture.6 Although surgery has been the primary option, other modalities such as transcatheter coil embolization, device closure of the neck of the aneurysm, or exclusion of the aneurysm using a
Tefera and Teodori covered stent have been reported to be successful alternatives to surgery.7,8 In our patient, an aneurysm was first suspected on a frontal chest x-ray and then confirmed on surface (transthoracic) echocardiography. The patient underwent successful surgical resection of the aneurysm and division of the PDA. Acknowledgments We are grateful to Dr Belay Abegaz, the founder of the Children’s Heart Fund of Ethiopia and the Cardiac Center for enabling pediatric cardiology practice in this country. We also thank all the staff members at the cardiac center. Last but not least, we are indebted to the team members who traveled all the way from the United States to help the poor children having congenital and acquired heart diseases.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
429 References 1. Boyd LJ, McGoveck TH. Aneurysms of pulmonary artery: review of the literature and report of two new cases. Am Heart J. 1939; 18(5): 562-578. 2. Barter T, Irwin RS, Nash G. Aneurysms of pulmonary arteries. Chest. 1988;94(5): 1065-1075. 3. Coleman M, Slater D, Bell R. Rupture of pulmonary artery aneurysm associated with persistent ductus arteriosus. Br Heart J. 1980;44(4): 464-468. 4. Caralps JM, Bonnin JO, Oter R, Aris A. True aneurysm of the main pulmonary artery: surgical correction. Ann Thorac Surg. 1978; 25(6): 561-563. 5. Dilema LG, Wynands JF, Bourke ME, Walley VM. Catheterinduced pulmonary artery false aneurysm and rupture: cases reports and review. J cardiothorac Vasc Anesth. 1994;8(1): 70-75. 6. Kuwaki K, Morishita K, Sato H, Urita R, Abe T. Surgical repair of the pulmonary trunk aneurysm. Eur J Cardiothorac Surgery. 2000; 18(5): 535-539. 7. Jagia P, Sharma S, Juneja R, Guleria R. Transcatheter treatment of pulmonary artery pseudoaneuryms using a patent ductus arteriosus closure device. Diagn Interv Radiol. 2011;17(1): 92-94. 8. Wilson N, McLeod K, Hallworth D. Exclusion of a pulmonary artery aneurysm using a covered stent. Heart. 2000;83(4): 438.
Pulmonary Artery Aneurysm With Patent Arterial Duct: Resection of Aneurysm and Ductal Division
World Journal for Pediatric and Congenital Heart Surgery 4(4) 427-429 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113494403 pch.sagepub.com
Endale Tefera, MD1 and Michael Teodori, MD2
Abstract Congenital or acquired aneurysm of the pulmonary artery (PA) is rare. Although aneurysms are described following surgical treatment of patent ductus arteriosus (PDA), occurrence of this lesion in association with PDA without previous surgery is extremely uncommon. An eight-year-old patient with PDA and aneurysm of the main PA is described in this report. Clinical diagnosis of PDA was made upon presentation. Diagnosis of PA aneurysm was suspected on chest x-ray and was confirmed on transthoracic echocardiography. Successful surgical resection of the aneurysm and division of the duct were performed under cardiopulmonary bypass. The patient did well on follow-up both from clinical and echocardiographic point of view. Keywords pulmonary artery aneurysm, patent ductus arteriosus, ductal division, aneurysm resection Submitted February 26, 2013; Accepted May 28, 2013.
Introduction Pulmonary artery (PA) aneurysms, whether congenital or acquired, are rare with reported autopsy incidence of about 1 in 14,000.1,2 The PA aneurysms occur as isolated entities or could be associated with congenital or acquired cardiovascular lesions, like patent ductus arteriosus (PDA) or infective endocarditis.2 Here, we report a case of pulmonary trunk aneurysm occurring with a large PDA and its successful surgical resection and division of the duct.
Case The case was an eight-year-old girl who presented to our hospital for the first time with palpitation and easy fatigability which was progressive in the preceding four years. In retrospect, the patient had symptoms dating back to early infancy with interruption of breast feeding, diaphoresis, failure to gain weight, and recurrent respiratory tract infections. She was not on any medication and did not have previous admission, although she had been treated for respiratory tract infection on an outpatient basis in her locality. She had no documented infective endocarditis history. On physical examination, she was chronically sick looking. She weighed 20 kg, and her height was 114 cm. Blood pressure on her left upper arm was 120/50 mm Hg. Her peripheral pulses were bounding. She has a bulged and active precordium with point of maximal impulse in the left fifth intercostals space lateral to the midclavicular line. She had a thrill and continuous murmur over the left second intercostals space. Liver
Figure 1. Preoperative chest x-ray showing pulmonary artery aneurysm.
1 Department of Pediatrics & Child Health, Cardiology Unit, School of Medicine, Addis Ababa University & Cardiac Center, Addis Ababa, Ethiopia 2 Pediatric and Adult Congenital Heart Surgery, University of Arizona Department of Surgery, Tucson, AZ, USA
Corresponding Author: Endale Tefera, Department of Pediatrics & Child Health, Cardiology Unit, School of Medicine, Addis Ababa University & Cardiac Center, Corner of Zambia & T. Abanefso Roads, PO Box 1768, Addis Ababa, Ethiopia. Email: [email protected]
428
World Journal for Pediatric and Congenital Heart Surgery 4(4)
Figure 2. 2D and color flow echo frames showing patent ductus arteriosus (PDA) and pulmonary artery (PA) aneurysm.
was just palpable below the right costal margin, but spleen was not palpable. Plain chest x-ray showed modest cardiac enlargement and moderately increased pulmonary blood flow. There was a bulging mass in the area of the main PA (Figure 1). Electrocardiogram showed sinus rhythm with signs of left atrial enlargement and left ventricular enlargement. Echocardiography showed dilated left atrium, left ventricle, and PA. There was a 7-mm PDA with a continuous left-to-right shunt. There was a saccular aneurysm arising from the distal part of the main PA just proximal to the point of bifurcation, which measured 7 7 cm (Figure 2). Its opening (communication with the main PA) measured 1.2 cm. Most part of the ductal flow was directed toward the aneurysm. There was no significant pulmonary hypertension on echocardiography. With this information, the patient was explored through a median sternotomy. Moderate pericardial adhesions were divided. The aneurysm arose from the distal anterior wall of the main PA, measured 7 cm across, and obscured exposure to the PDA. Cardiopulmonary bypass was initiated to minimize risk because of the limited exposure. On reduced flow, the duct was quickly exposed and doubly clamped, nicely decompressing the pulmonary aneurysm. Full flows on bypass were resumed while the duct was divided and both the ends over sewn. The aneurysm wall was then resected, and there was no indication of infection or other pathology on gross appearance. The edges of the PA were reapproximated. The child was readily weaned off bypass and was extubated in the operating theater. Total bypass time was 13 minutes. The patient had an uneventful intensive care unit stay. She is doing well in follow-up in terms of clinical and echocardiographic parameters. Follow-up chest x-ray taken a week after the surgery is shown in Figure 3.
Discussion PA aneurysm in the setting of PDA has been described in different reports. However, when aneurysm of the PA occurs with
Figure 3. Postoperative chest x-ray taken after 1-week.
PDA, it commonly occurs in association with severe pulmonary arterial hypertension. Histologic examinations of such patients have also shown that cystic medial necrosis was a prominent feature.3,4 In our patient, there was a continuous murmur on physical examination. Two-dimensional echocardiography revealed continuous left-to-right shunt from aorta to PA, with a significant pressure gradient (maximum Doppler measured velocity of the ductal jet in systole was 5 m/s), which indicates that there was no significant pulmonary arterial hypertension. Mortality rate for ruptured aneurysm of the PA is 100%.5 There are controversies regarding the treatment of PA aneurysms. Some authors recommend surgical treatment when a main PA aneurysm is discovered, irrespective of etiology or absence of pulmonary hypertension, because there is a potential for rupture.6 Although surgery has been the primary option, other modalities such as transcatheter coil embolization, device closure of the neck of the aneurysm, or exclusion of the aneurysm using a
Tefera and Teodori covered stent have been reported to be successful alternatives to surgery.7,8 In our patient, an aneurysm was first suspected on a frontal chest x-ray and then confirmed on surface (transthoracic) echocardiography. The patient underwent successful surgical resection of the aneurysm and division of the PDA. Acknowledgments We are grateful to Dr Belay Abegaz, the founder of the Children’s Heart Fund of Ethiopia and the Cardiac Center for enabling pediatric cardiology practice in this country. We also thank all the staff members at the cardiac center. Last but not least, we are indebted to the team members who traveled all the way from the United States to help the poor children having congenital and acquired heart diseases.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
429 References 1. Boyd LJ, McGoveck TH. Aneurysms of pulmonary artery: review of the literature and report of two new cases. Am Heart J. 1939; 18(5): 562-578. 2. Barter T, Irwin RS, Nash G. Aneurysms of pulmonary arteries. Chest. 1988;94(5): 1065-1075. 3. Coleman M, Slater D, Bell R. Rupture of pulmonary artery aneurysm associated with persistent ductus arteriosus. Br Heart J. 1980;44(4): 464-468. 4. Caralps JM, Bonnin JO, Oter R, Aris A. True aneurysm of the main pulmonary artery: surgical correction. Ann Thorac Surg. 1978; 25(6): 561-563. 5. Dilema LG, Wynands JF, Bourke ME, Walley VM. Catheterinduced pulmonary artery false aneurysm and rupture: cases reports and review. J cardiothorac Vasc Anesth. 1994;8(1): 70-75. 6. Kuwaki K, Morishita K, Sato H, Urita R, Abe T. Surgical repair of the pulmonary trunk aneurysm. Eur J Cardiothorac Surgery. 2000; 18(5): 535-539. 7. Jagia P, Sharma S, Juneja R, Guleria R. Transcatheter treatment of pulmonary artery pseudoaneuryms using a patent ductus arteriosus closure device. Diagn Interv Radiol. 2011;17(1): 92-94. 8. Wilson N, McLeod K, Hallworth D. Exclusion of a pulmonary artery aneurysm using a covered stent. Heart. 2000;83(4): 438.
