Interact CardioVasc Thorac Surg 2008;7:517-518. doi:10.1510/icvts.2007.171280
© 2008 European Association of Cardio-Thoracic Surgery
Early atrial septal defect surgery due to a bronchogenic cyst causing congestive heart failure by left atrium compression
Victor X. Mosqueraa,*,
Marry Rijlaarsdamb,
Luc Filippinic and
Mark G. Hazekampa
a Department of Cardiothoracic Surgery K6-S, Leiden University Medical Center, Albinusdreef 2, 2333 2A, Leiden, The Netherlands
b Department of Pediatric Cardiology, Leiden University Medical Center, The Netherlands
c Department of Pediatric Cardiology, Juliana Childrens Hospital, The Hague, The Netherlands
Received 7 November 2007;
received in revised form 8 March 2008;
accepted 11 March 2008
*Corresponding author. Tel.: +31 71 526 4022; fax: +31 71 526 6965.
E-mail address: vxmr{at}yahoo.es; v.x.mosquerarodriquez{at}lumc.nl (V.X. Mosquera).
 |
Abstract
|
|---|
We report on an uncommon case of a 10-month-old patient who required early surgical closure of an ostium secumdum atrial septal defect due to the concomitant presence of a big subcarinal bronchogenic cyst compressing the left atrium and, therefore, increasing the left-to-right shunt. It led to refractory congestive heart failure symptoms, establishing thereby an earlier indication of surgical treatment.
Key Words: CHD; Septal defects; Cysts; Heart failure
|
1. Introduction
|
|---|
We report on an uncommon case of a 10-month-old patient who required early surgical closure of an ostium secumdum atrial septal defect due to the concomitant presence of a big subcarinal bronchogenic cyst compressing the left atrium and, therefore, increasing the left-to-right shunt.
|
2. Case description
|
|---|
A 10-month-old infant was referred to our institution with congestive heart failure symptoms persisting even with high doses of diuretics. Echocardiogram examination demonstrated a 13x14 mm ASD type II with important left-to-right shunt and important RA and RV dilatation (RV:LV=1.5:1). Closer examination of the echocardiogram revealed a mass located on the cranial aspect of the left atrium, which was compressing the LA. The functional status of the mitral valve was not compromised despite the LA compression. A CT-scan confirmed the presence of a large mass with a diameter of 13 mm located on the cranial aspect of the left atrium, ventral to the descending aorta, in a direct subcarinal position (Fig. 1). There was also a clear compression of the pulmonary veins, causing post-capillary pulmonary hypertension and inflow restriction (Fig. 1c). Based on these findings, the lesion was diagnosed as a bronchogenic cyst located in the middle of the mediastinum causing compression of the left atrium and, therefore, an increase in left atrium end-diastolic pressure and left-to-right shunt. The patient was considered a candidate for simultaneous surgical closure of the ASD and resection of the cystic lesion.
View larger version (50K):
[in this window]
[in a new window]
|
Fig. 1. (a) Axial slide of a contrast enhanced CT-scan shows a large homogeneous structure with fluid-equivalent signal intensity, the bronchogenic cyst (white asterisk), compressing the left atrium (black asterisk). There is no enhancement by contrast injection. (b) Coronal slide of a contrast enhanced CT-scan reconstruction. The bronchogenic cyst (white asterisk), located under the carina (white arrow), compressed the LA (black asterisk). (c) Sagital slide of a contrast enhanced CT-scan reconstruction demonstrates the morphological relationship among the cyst (white asterisk), the left upper pulmonary vein (white spotted arrow), the left inferior pulmonary vein (white arrow), the left pulmonary artery (black arrow) and the carinaleft main bronchus (white dashed arrow).
|
|
The operation was conducted via a sternotomy. The cyst was approached between the ascending aorta and superior cava vein, staying superior to the roof of the left atrium (Fig. 2a). During resection, the cyst was accidentally opened releasing a white ‘jelly-like’ fluid (Fig. 2b). The cyst was tightly adhered to the membranous parts of carina and right main bronchus. After resection, a perforation of the pars membranacea of the bronchus was closed directly. Subsequently, cardiopulmonary bypass was conducted at 32 °C core temperature with bicaval cannulation, aortic cross clamping, and cardioplegic protection. The ASD was thereby closed with a xenopericardial patch.
View larger version (64K):
[in this window]
[in a new window]
|
Fig. 2. Surgical images. (a) A large mass (Cyst) located in direct subcarinal position compressing both cardiac structures, roof of the left atrium (LA) and the right pulmonary artery (RPA). (b) Due to the intense adhesion of the cyst to the right bronchi, the cyst was accidentally opened during its resection releasing a white ‘jelly-like’ fluid (black asterisk). However, it could be completely removed.
|
|
Histological examination of the resected tissue revealed respiratory epithelium lining the capsular wall, intramural islets of cartilage and infiltration by chronic inflammatory cells confirming the diagnosis of bronchogenic cyst.
The patient stayed two days at the ICU and was discharged from hospital after seven days. Three months after the surgery, the follow-up echocardiogram showed no residual ASD and there was neither a sign of LA compression nor of increased left atrial end-diastolic pressure. The patient remains asymptomatic and no longer needs diuretic treatment.
|
3. Discussion
|
|---|
Surgical closure of an isolated ASD type II before two years of age is uncommon because patients usually remain asymptomatic for many years [1]. Surgery is ideally performed in children aged 2–4 years and has a very low mortality rate [2]. However, surgery may be performed earlier than this if the child has evidence of congestive heart failure and this usually occurs in the presence of another anomaly increasing significantly the degree of left-to-right shunt through the septal defect [3].
In this case, external compression of the left atrium by a bronchogenic cyst caused a rise in left atrial end-diastolic pressure and, therefore, in left-to-right shunt. Moreover, that mass was compressing the pulmonary veins, causing post-capillary pulmonary hypertension and inflow restriction. All these factors led to congestive heart failure symptoms and established an earlier indication of surgical treatment. The fact that the CHF symptoms had disappeared after surgery supports the fact that the abnormally remarkable left-to-right shunt through the ASD was responsible for the important symptoms.
Bronchogenic cysts account for 10–15% of all primary mediastinal masses [4] and can be classified as either intrapulmonary or mediastinal. Overall, 72% of bronchogenic cysts produce some symptoms [4], but 90% of mediastinal-type bronchogenic cysts are reported to be asymptomatic [5]. Mediastinal-type bronchogenic cysts have been classified into five types: paratracheal, carinal, hilar, paraesophageal, or miscellaneous [6]. The paratracheal and carinal types may produce symptoms such as dyspnea and chest pain, due to compression of the trachea or bronchi. A giant bronchial cyst of the carinal type would be expected to produce left atrial compression due to the anatomic proximity of the left atrium [5] although, in practice, patients with a bronchogenic cyst have rarely been reported to show symptoms related to the heart and/or signs of left atrial overload. Although the most commonly used surgical access for mediastinal bronchogenic cyst is a lateral thoracotomy [7], we performed a sternotomy because it not only allowed us a good access for right atrium for the ASD closure, but also provided a better exposure of the morphological relationship among the subcarinal cyst and the roof of the left atrium and pulmonary arteries.
|
References
|
|---|
- Ward C. Secundum atrial septal defect: routine surgical treatment is not of proven benefit. Br Heart J 1994 3;71:219–223.[Free Full Text]
- Alexi-Meskishvili VV, Konstantinov IE. Surgery for atrial septal defect: from the first experiments to clinical practice. Ann Thorac Surg 2003 7;76:322–327.[Free Full Text]
- Zannini L, Borini I. State of the art of cardiac surgery in patients with congenital heart disease. J Cardiovasc Med (Hagerstown) 2007 1;8:3–6.[Medline]
- St-Georges R, Deslauriers J, Duranceau A, Vaillancourt R, Deschamps C, Beauchamp G, Pagé A, Brisson J. Clinical spectrum of bronchogenic cysts of the mediastinum and lung in the adult. Ann Thorac Surg 1991 7;52:6–13.[Abstract]
- Kanemitsu Y, Nakayama H, Asamura H, Kondo H, Tsuchiya R, Naruke T. Clinical features and management of bronchogenic cysts: report of 17 cases. Surg Today 1999;29:1201–1205.[CrossRef][Medline]
- Horak E, Bodner J, Gassner I, Schmid T, Simma B, Grassl G, Sawyer SM. Congenital cystic lung disease: diagnostic and therapeutic considerations. Clin Pediatr (Phila) 2003 4;42:251–261.[Abstract/Free Full Text]
- Ribet ME, Copin MC, Gosselin B. Bronchogenic cysts of the mediastinum. J Thorac Cardiovasc Surg 1995 5;109:1003–1010.[Abstract]
|
|
Top
Abstract
1. Introduction