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J Thorac Cardiovasc Surg 2006;131:1187-1188
© 2006 The American Association for Thoracic Surgery
Brief Communication |
a Department of Pediatric Cardiology, Turkiye Yuksek Ihtisas Hospital, Ankara, Turkey
b Department of Cardiovascular Surgery, Turkiye Yuksek Ihtisas Hospital, Ankara, Turkey
c Department of Cardiology, Turkiye Yuksek Ihtisas Hospital, Ankara, Turkey
Presented in the 4th World Congress of Pediatric Cardiology and Cardiothoracic Surgery, September 18-23, 2005, Buenos Aires, Argentina.
Received for publication October 10, 2005; accepted for publication October 19, 2005. * Address for reprints: Dr Ugursay Kiziltepe, 30. Cad. 21/23, Isci Bloklari Mah, Karakusunlar, Balgat, Ankara 06000 Turkey (Email: ukiziltepe{at}ttnet.net.tr).
Pulmonary arteriovenous malformations (PAVMs) are abnormal connections between pulmonary arteries (PAs) and pulmonary veins (PVs).
1
These lesions may cause cyanosis, clubbing, and dyspnea on exertion and can be misdiagnosed as complex congenital heart disease. We report an interesting case with a large aneurysmal sac in continuity with the left atrium (LA) and fed by a large PA branch.
Clinical Summary
A 6-year-old boy had dyspnea and severe cyanosis, which first appeared at 6 months of age, and was admitted to our hospital. Mucocutaneous telengiectasias were not present. A negative pathergy test ruled out Behcet's disease. His echocardiography revealed an aneurysmal sac behind the LA, which was in continuity with the LA via a large orifice and absent adjunctive defects. Angiography demonstrated a wide connection between the sac and LA and a large, single feeding artery (afferent) originating from the right PA without an efferent vessel from the sac (Figure 1, a). Cardiac 16-slice computerized tomographic angiography showed that PV orifices were not in relation with the sac and no additional PAVMs were present. All the diagnostic tests supported the absence of thrombi or calcium.
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Discussion
PAVMs are most commonly congenital in nature
1
and 47% to 70% of the cases are associated with hereditary hemorrhagic telangiectasia.
2
Development of PAVMs is a well-known phenomenon following cavopulmonary anastomoses, especially in the absence of hepatic blood flow to the lungs.
3
All PAVMs have afferent supply, usually from branches of the PA. The efferent limb of an arteriovenous malformation drains into branches of PVs or sometimes directly into the LA or inferior vena cava. They usually are embedded to lung parenchyma.
In addition to causing shunt resulting in dyspnea, volume overload, and cyanosis, PAVMs also cause the loss of filter function of lungs and thus may allow development of stroke and cerebral abscess. The classic triad of dyspnea, cyanosis, and clubbing is present in only 10% of cases.
1
In our case, the lesion was producing high-output right-to-left shunt and causing severe cyanosis. The aneurysm had a single large feeding artery (afferent vessel) and no efferent vessel. Interestingly, it was in continuity with the LA with an orifice as big as the aneurysm itself. In our knowledge no similar case has been reported in literature.
Traditional options for the definitive management of PAVM include embolization and surgery. Ligation, local excision, segmentectomy, lobectomy, or pneumonectomy have been performed,
1,2
although most of the time parenchyme-saving operations are preferred.
It was obvious that embolization would be inappropriate for this case not only because leaving a residual aneurysm would result in a high risk of rupture but also because of the unsuitable anatomy of the feeding artery. During preoperative evaluation we considered 2 possible approaches for surgical treatment of this PAVM. Instead of median sternotomy and repair through the LA with cardiopulmonary bypass and cardiac arrest, we used simple ligation and resection through thoracotomy, which allowed better exposure, a shorter operation, and faster recovery. We believe the origin of this sac would be one of the following: an aneurysmal PAVM with draining vessel was absorbed in time and united widely to the LA, or a distal PA aneurysm ruptured and united to the LA. Unusual features like extraparenchymal location of the lesion, absence of an efferent vessel, unity of aneurysmal sac with the LA, and the absence of associated etiologic factors make this lesion interesting. Nonetheless, prompt surgical treatment with a simple approach relieved the severe symptoms and prevented a potential fatal rupture.
In conclusion, although PAVMs are rare, they should be included in any differential diagnosis of worsening dyspnea, cyanosis, stroke, or hemoptysis. Their presence should be considered especially in patients with cyanosis whom no evidence of intracardiac shunts in echocardiography. Prompt workup and intervention can prevent fatal complications like rupture and lead to dramatic improvement in symptoms.
References
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