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Ann Thorac Surg 2005;80:336-338
© 2005 The Society of Thoracic Surgeons
a Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
b Department of Cardiology, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
Accepted for publication August 19, 2004.
* Address reprint requests to Dr Chowdhury, Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029 India (Email: ujjwalchow{at}rediffmail.com).
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Introduction |
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In view of the rarity of this lesion, we describe a cohort of 5 patients with this anomaly who have had successful surgical repair of this defect, and we discuss their management.
Between December 1997 and December 2003, five patients aged 14, 29, 4.6, 9, and 7 years (patients 1 through 5, respectively) presented with central cyanosis and progressive shortness of breath on moderate exertion. All had central cyanosis, clubbing, and normal cardiac auscultation. None had a history of recurrent chest infections, cyanotic spells, seizures, prolonged fever, or hemoptysis. There was no hemangioma of skin or mucous membrane in any patient. The diagnosis was established preoperatively by clinical examination, chest roentgenogram, cross-sectional echocardiography, and cardiac catheterization in all patients (Table 1).
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During cardiac catheterization, the right heart catheter entered into a sac-like structure from the right pulmonary artery. Cineangiography performed from selective right pulmonary artery injection in anteroposterior and lateral views revealed filling of a hugely ectatic sac-like structure related to the undersurface of the right pulmonary artery that drained into the left atrium in the next two to three beats, whereas the pulmonary arterial tree was still not completely visualized (Fig 1). Levophase showed normal pulmonary venous drainage to both sides in all patients.
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The lesion was approached through a median sternotomy in patients 1 through 4. The anomalous vessel between the sac and the right pulmonary artery was doubly ligated using ductus silk and was transfixed. A right posterolateral thoracotomy through the fourth intercostal space was performed in patient 5. The fistulous sac was excluded by double ligation at the pulmonary arterial end proximal to its junction with the right inferior pulmonary vein.
There were no operative deaths. Systemic arterial oxygen saturation improved from 72% to 82% to >97% in all patients after ligation, and the thrill soon disappeared after ligation in all the cases. Intraoperative transesophageal contrast echocardiogram was performed in all patients, and no residual shunts were seen.
There were no late deaths. They have been followed up periodically for periods ranging from 6 to 72 months (mean ± standard deviation, 40 ± 18.86 months). All patients were acyanotic and in New York heart association functional class I.
Two-dimensional and Doppler echocardiogram revealed no intracardiac shunting. Postoperative selective angiographic and indicator-dilution studies (n = 5) have demonstrated complete disappearance of right-to-left shunt.
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Patients may present symptomatically from infancy to late adulthood [2]. Those who present late have a milder clinical course compared with those presenting in infancy [2]. This entity should be suspected when the following combination of symptoms and signs coexist: central cyanosis, clubbing of fingers, exertional dyspnea, silent precordium, normally split second heart sound, nonspecific murmur over left or right axillary region, and an abnormal roentgenographic density in the right or left pulmonary hilum [1–4].
Diagnosis of this anomaly is difficult. On contrast echocardiogram, filling of an aneurysmal sac, behind the left atrium and communicating to it after three beats of contrast filling to right-sided structures was diagnostic of pulmonary arteriovenous fistula and suggested right pulmonary artery-to-left atrium communication in this context [2, 4]. Cardiac catheterization with selective angiography remains the most definitive means to confirm this unusual diagnosis and accurately defines the anatomical details [1–4].
Usually the anomalous vessel originates from the posterior wall of the right pulmonary artery and is connected to the left atrium between the right and left pulmonary veins [1, 2]. Only three cases of communication between left pulmonary artery and left atrium have been reported [2]. Associated atrial septal defects are common [1–5]. Based on the presence or absence of an aneurysm in the fistula and on the anatomy of pulmonary venous drainage pattern, de Souza e Silva and coauthors [4] categorized three types of connections. Type I is a normal pulmonary venous pattern; type II is an absent right inferior pulmonary vein with fistulous connection at the normal site of its ostium; and type III is when all the pulmonary veins are connected to the aneurysmal pouch. Subsequently, Ohara and coauthors [5] added a fourth type. Type IV is a right inferior pulmonary vein replaced by three small veins connected to the aneurysmal pouch. The left-sided pulmonary veins join the left atrium directly.
Early surgical intervention is advised to prevent chronic arterial hypoxemia, systemic thromboembolic complications, congestive heart failure, shunt-derived pulmonary hypertension, and cerebral abscess [1–5].
Depending on the anatomical locations, the lesion have been approached through a median sternotomy, right posterolateral thoracotomy, or left posterolateral thoracotomy [1–5]. An individualized management algorithm would be helpful, perhaps specified for each of the anatomic types [1–5]. Simple ligation or division through median sternotomy appears suitable for type I fistulas. A right or left posterolateral thoracotomy may be the preferred approach for type II fistulas. Use of extracorporeal circulation may be better suited for type III and type IV fistulas in instances in which dissection of the anomalous vessel is technically difficult and in those cases requiring intricate intracardiac repair [1–5]. Isolated case reports of transcatheter closure of the fistula have been published as well [6].
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