ATS
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Henri L. Porte
Alain J. Wurtz
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Fabre, O. H.
Right arrow Articles by Wurtz, A. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Fabre, O. H.
Right arrow Articles by Wurtz, A. J.

Ann Thorac Surg 1998;65:1144-1146
© 1998 The Society of Thoracic Surgeons

Case Reports

Long-Term Cardiovascular Consequences of Undiagnosed Intralobar Pulmonary Sequestration

Olivier H. Fabre, MDa, Henri L. Porte, MDa, François R. Godart, MDa, Christian Rey, MDa, Alain J. Wurtz, MDa

a Clinique Chirurgicale, Hôpital Calmette, and Hôpital Cardiologique, Centre Hospitalier Régional et Universitaire de Lille, Lille, France

Accepted for publication November 5, 1997.

Address reprint requests to Dr Porte, Clinique Chirurgicale, Hôpital Calmette, Centre Hospitalier Régional et Universitaire de Lille, 59037 Lille cedex, France


   Abstract
Top
 Abstract
Introduction
 Comment
 References
 
We report the case of a patient with a congenital aortic valve stenosis associated with a long-term undiagnosed intralobar pulmonary sequestration. The important blood flow through the aberrant artery led to progressive congestive heart failure and severe hemoptysis at the age of 25 years. We demonstrate the regression of cardiac symptoms and left ventricular diameter after surgical resection of the sequestration.


   Introduction
Top
 Abstract
 Introduction
Comment
 References
 
Pulmonary sequestration is an uncommon congenital malformation characterized by pulmonary lobes or segments unconnected to the bronchial system of the surrounding normal lung. Pulmonary sequestration can be either extralobar or intralobar, receiving its blood supply from one or several systemic arteries [1]. By virtue of this aberrant systemic blood supply and pulmonary venous drainage to the left atrium, hemodynamic symptoms may occur. We report the case of a patient with undiagnosed intralobar pulmonary sequestration (IPS) associated with aortic valve stenosis. In spite of aortic valve comissurotomy at the age of 5 months, the patient had progressive cardiomegaly caused by the unrecognized IPS, leading to congestive heart failure.

A 25-year-old man was referred to the emergency department in December 1996 for sudden massive hemoptysis (500 mL) without hemodynamic failure. Initial examination revealed a systolic and diastolic murmur in the left lower basilar area. Chest radiography showed cardiomegaly with an enlarged mediastinum and a left lower lobe systematized opacity. Computed tomographic scan showed enhancement of contrast material within the opacity associated with a huge dilatation of the ascending aorta. Aortography showed runoff contrast material from the descending thoracic aorta to a 2.5-cm-diameter aberrant vessel supplying the left lower lobe and draining to the left lower pulmonary vein (Fig 1). A left posterolateral thoracotomy was performed as an emergency and, after ligation of the aberrant vessel, the congested airless lower lobe was resected.



View larger version (107K):
[in this window]
[in a new window]
  		Fig 1. Preoperative aortogram showing the large aberrant supply to the left lower lobe pulmonary sequestration.

 
The medical history revealed that, when the patient was 5 months of age, a mild systolic murmur was discovered and led to the diagnosis of aortic valve stenosis on angiography. The same year, a cardiac operation confirmed the aortic valve stenosis (bicuspid aortic valve), and aortic commisurotomy was performed. Subsequently, the patient suffered from recurrent bronchopulmonary infections. Examination revealed a residual systolic and diastolic murmur and chest radiography showed a persistent left lower lobe opacity with significant cardiomegaly. In 1989, the patient was short of breath after minimal effort, and Doppler echocardiography showed an increased left ventricular end-diastolic diameter of 68 mm without systolic dysfunction (fractional shortening of 41%) and a mild valvular aortic stenosis (peak systolic gradient of 41 mm Hg) with a tiny aortic regurgitation (grade I/IV). In this context, the clinical symptoms and the cardiomegaly were related to a residual mild aortic valve stenosis associated with left ventricular fibroelastosis.

Three months after lobectomy, exercise tolerance was normal without any drugs. Doppler echocardiography showed regression of the left ventricular end-diastolic diameter (50 mm), no significant aortic valve pressure gradient (peak systolic gradient of 17 mm Hg), and aortic regurgitation (grade II/IV). A follow-up cardiac catheterization (Fig 2) revealed a persistent enlarged thoracic aorta (diameter between 47 and 57 mm) and a normal left ventricular systolic function (ejection fraction of 0.64).



View larger version (154K):
[in this window]
[in a new window]
  		Fig 2. Postoperative aortogram showing persistent dilatation of the descending thoracic aorta above the aberrant supply ligation.

 

   Comment
Top
 Abstract
 Introduction
 Comment
References
 
The recognition of pulmonary sequestration is important because acute life-threatening complications, mainly massive hemoptysis, can occur. It is usually discovered in childhood because of the early appearance of symptoms, mainly chronic pulmonary infections [2]. However, Savic and associates [1], in a review of 540 cases, noticed that the correct preoperative diagnosis was suspected in less than 50% of the cases.

Cardiovascular complications may be present when the diameter of the nutrient vessel is important or when the sequestration is unknown for a long time, as in our case. Ransom and associates [3] reported the case of a 6-month-old child with severe congestive heart failure caused by IPS who completely recovered after left lower lobectomy. They reviewed the literature and found 2 other cases with postoperative cardiac index reduction [2, 4]. Flye and colleagues [5] reported 3 cases of congestive heart failure in patients who had concomitant IPS and significant congenital heart malformations. In these patients, the responsibility of shunting through the sequestration rather than the heart malformation could not be determined before the patients’ death.

Heart failure is clearly the consequence of increased cardiac output due to a left-to-left shunt through IPS. The magnitude of the shunt may increase with age, compromising the left ventricular function as observed in our patient [6]. We demonstrate the long-term effects of IPS on the cardiovascular system with the complete regression of symptoms after operation and show that IPS may masquerade as a cardiac malformation for a long time. Even if hemodynamic symptoms and left ventricular dilatation completely recovered soon after the operation, the persistent aortic arch dilatation remains of concern. This emphasizes the need to resect all diagnosed IPS. Retrospectively, despite the presence of aortic valve malformation, the chronic left lower lobe opacity on the chest roentgenogram associated with recurrent bronchopulmonary infections should have evoked the diagnosis of IPS.


   References
Top
 Abstract
 Introduction
 Comment
 References
 

  1. Savic B., Birtel F.J., Tholen W., Funke H.D., Knoche R. Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979;34:96-101.[Abstract/Free Full Text]
  2. White J., Donahoo J., Ostrow P., Murphy J., Haller J. Cardiovascular and respiratory manifestations of pulmonary sequestration in childhood. Ann Thorac Surg 1974;18:286-294.[Abstract/Free Full Text]
  3. Ransom J., Norton J., Williams G. Pulmonary sequestration presenting as congestive heart failure. J Thorac Cardiovasc Surg 1978;76:378-380.[Medline]
  4. Solit R., Fraimow W., Wallace S., Cohn H. The effect of intralobar pulmonary sequestration on cardiac output. J Thorac Cardiovasc Surg 1965;49:844-853.
  5. Flye M., Conley M., Silver D. Spectrum of pulmonary sequestration. Ann Thorac Surg 1976;22:478-482.[Abstract/Free Full Text]
  6. Khalil K., Kilman J. Pulmonary sequestration. J Thorac Cardiovasc Surg 1975;70:928-937.[Abstract]



This article has been cited by other articles:


Home page
 J. Thorac. Cardiovasc. Surg.Home page
S. Gezer, I. Tastepe, M. Sirmali, G. Findik, H. Turut, S. Kaya, N. Karaoglanoglu, and G. Cetin
Pulmonary sequestration: A single-institutional series composed of 27 cases
J. Thorac. Cardiovasc. Surg., April 1, 2007; 133(4): 955 - 959.
[Abstract] [Full Text] [PDF]

Home page
 Asian Cardiovasc. Thorac. Ann.Home page
O. Genc, S. Gurkok, M. Dakak, A. Gozubuyuk, M. Ozkan, and H. Caylak
Pulmonary Sequestration and Surgical Treatment
Asian Cardiovasc Thorac Ann, February 1, 2006; 14(1): 3 - 6.
[Abstract] [Full Text] [PDF]

Home page
 Eur J Cardiothorac SurgHome page
G. Shanmugam, K. MacArthur, and J. C. Pollock
Congenital lung malformations--antenatal and postnatal evaluation and management
Eur J Cardiothorac Surg, January 1, 2005; 27(1): 45 - 52.
[Abstract] [Full Text] [PDF]

Home page
 ChestHome page
M. M. Behnia, P. W. Catalano, and W. S. Brooks
Hemoptysis in a 38-Year-Old Woman Receiving an Oral Contraceptive
Chest, May 1, 2004; 125(5): 1944 - 1947.
[Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
V. Marti, S. Pujadas, P. Casan, J. Garcia, P. Guiteras, and J. M. Auge
Reversible dilated cardiomyopathy after lobectomy for pulmonary sequestration
J. Thorac. Cardiovasc. Surg., May 1, 2001; 121(5): 1001 - 1002.
[Full Text] [PDF]

Home page
 Eur J Cardiothorac SurgHome page
D. Van Raemdonck, K. De Boeck, H. Devlieger, M. Demedts, P. Moerman, W. Coosemans, G. Deneffe, and T. Lerut
Pulmonary sequestration: a comparison between pediatric and adult patients
Eur J Cardiothorac Surg, April 1, 2001; 19(4): 388 - 395.
[Abstract] [Full Text] [PDF]

This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Henri L. Porte
Alain J. Wurtz
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Fabre, O. H.
Right arrow Articles by Wurtz, A. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Fabre, O. H.
Right arrow Articles by Wurtz, A. J.

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
ANN THORAC SURG ASIAN CARDIOVASC THORAC ANN EUR J CARDIOTHORAC SURG
J THORAC CARDIOVASC SURG ICVTS ALL CTSNet JOURNALS