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fil.SLVI I.:R luimpcuii
J o u r n a l o f C u i d i o - t h o i i i d c S u r f e r ) ' ]1
Surgical treatment of a fistula between the right pulmonary artery and the left presentation of two cases and review of
C.J.A.M. Zeebregts “•*, A. N ijv eld 11, J. Lam b, A .M . van O o r tc, L.K . L a c q u e t“
a
Department of Thoracic and Cardiac Surgery, Childrens Heart Centre
,
University Hospital Nijmegen St. Radboud
,
P.O. B ox 9101
,
6500 HB Nijmegen* The Netherlands
h
Department o f Pediatric Cardiology
,
University Hospital Amsterdam A M
C,
Am sterdam
,
The Netherlands
c
Department o f Pediatric Cardiology, Children's Heart Centre
, i/w r m /fy
Hospital Nijmegen St. Radboud
, P.O. 5o.v
9 W L 6500 H B Nijmegen
, 77it'
Netherlands
Received 9 September 1996; received in revised form 22 January 1997; accepted 22 January 1997 Abstract Objective: A direct communication between the pulm onary artery and the left atrium is a rare anomaly. On the basis of two cases of our own and a literature review of 49 cases, we focus on clinical presentation, anatom y, diagnosis, and the role o f surgery. Methods: Two cases of a fistula between the right pulm onary artery and the left atrium are described in a girl of 4 years and a boy of 15 years. Both presented with unexplained cyanosis. Diagnosis was made on echocardiography and angiography. The fistula was ligated using extracorporeal circulation in the first case and not in the second case. Results: The surgical results were successful with resolution of the cyanosis. Conclusions: In newborns, urgent surgery may be necessary. In other patients, early elective surgical correction should be performed to prevent complications, especially systemic and cerebral emboli, cerebral abscesses, and rupture of aneurysmal fistulas. Complete cure can be achieved by ligation and possible division or by intracardiac repair. 0 1997 Elsevier Science B.V.
Keywords:
Cerebral abscess; Cyanosis; Extracorporeal circulation; Right pulm onary a rte ry -le ft atrium fistula
1. Introduction 2. Case reports
A direct communication between the right pul monary artery (RPA) and the ieft atrium (LA) is a rare anomaly. Central cyanosis with clubbing of fingers and toes, exertional dyspnea and decreased arterial oxygen saturation usually accompany the lesion. Complications are cerebral and systemic emboli, cerebral abscesses and rupture of the fistula. On the basis of our two cases and a literature review, we focus on clinical presentation, anatomy, diagnosis and the role of surgical correction, as complete cure can be achieved by ligation and possi ble division of the fistula or by intracardiac repair.
* Corresponding author. Tel.: +31 24 3614744; fax- + 3 1 24 3540129.
2 .Î. Case A
A 4-year-and-3-months-old girl was admitted to hos pital because of sleeping problems. She had severe cyanosis and, in retrospect, her mother stated, that for many years she had had a cyanotic color. On examina tion, a cyanotic girl was seen, 110 cm tall and weighing 17.5 kg. Her blood pressure was 95/65 mm ITg. She had cyanosis of the lips and limbs with clubbing of the fingers. No tachypnea, dyspnea, or murmurs were noted. Liver and spleen were not palpable. Laboratory examination showed a hemoglobin concentration (Hb) of 11.7 mmol/1, a hematocrit (Ht) of 0.57 and a platelet count of 244 x 109/1. Electrocardiography showed a normal sinus rhythm. Chest X-ray revealed no abnor 1(110-7940/97/517.00 1997 Elsevier Science B.V. AU rights reserved.
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J, A. M. Zeeb refits ct al. Europ can Jour mi I o f Cardin-th or at u Si o^crv I i (¡997) J 056 1(161 Table I S u n im u r ) of all reported eases o f a direct communication between the richt pulmonary artery and the left atrium, including our two cases [j 7 . 9.1 i- 1 (0 8 .2 0-25 .2 7-36 ,38 44,46 -58] n (1 / 1 u • --- ----- - - Gender Male Female Unspecified Age 0-1 month I 12 months I -10 years 11 20 years 21 - 30 years 31 -40 years 41 50 years 5 ! “ 60 years Unspecified Approach Right thoracotomy (RT) Median sternotomy (MS) RT followed by MS No surgery Unspecified Type of opera tion Ligation Division Excision Intracardiac repair Pneumonectomy Exploration No surgery Unspecified Use of ECC Used Not used No surgery Unspecified Outcome Died Survived Unspecified ■ * * *■ -“ «* « v * ■ » rn 37 13 8 2 18 13 3 4 28 12 6 4 27 6 5 4 1 6 1 1 29 11 6 5 n 38 1 1 1 1 1 1 73 25 j L, 16 4 35 25 6 i 8 i ** 55 24 0 12 8 53 12 10 8 2 2 12 O 57 12 10 22 75 4 directly into the systemic circulation, thereby bypassing pulmonary filter function, may lead to cerebral compli cations. Transient ischemic attacks, cerebral infarctions and abscesses occur more often with these fistulas [19,34,37]. Other complications are: endocarditis, infec tive endarteritis and aneurysmatic growth of the fistula, with the risk o f fatal rupture. To prevent these complications, elective surgery is recommended [8,15,19,37]. Absolute indications for sur gical correction are severe cyanosis with a significantly decreased systemic oxygen saturation or severe poly cythemia. It is possible to perform embolization of the fistula without surgery [10]. However, direct communi cation between the RPA and LA exposes the patient to 1059 a high risk of major complications. Surgical correction is therefore preferable. In general, the fistula can easily be ligated and divided. Procedures are also described where the fistula is only ligated (without division) or where an intracardiac repair is performed with the use of EC C A lack of information about the consistency of m the fistulous tissue during extracardiac procedures may be a reason to use ECC. In conclusion, apart from mild cyanosis and finger clubbing, direct communication between the right pul monary artery and the left atrium may give few symp toms, causing a significant delay in diagnosis. Electrocardiography and chest X-ray may be com pletely normal. Echocardiography and cardiac catheter ization, including selective angiography, provide the necessary information. To prevent complications, espe cially systemic and cerebral emboli, early surgical inter vention should be performed. Complete cure can be achieved by ligation and possible division or by intrac ardiac repair. References [1] Abe T, Kuribayashi R, Sato M, Nieda S. 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