Ann Thorac Surg 2002;73:282-284
© 2002 The Society of Thoracic Surgeons
Case report
Absent pulmonary valve syndrome with aortic regurgitation in a 50-year-old man
Pankaj Goel, MCha,
Sethurathinam Rajan, MCh*a,
V.M. Kurian, MCha,
Raibhan Yadav, MDa,
Kotturathu Mammen Cherian, FRACSa
a Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India
Accepted for publication May 1, 2001.
* Address reprint requests to Dr Rajan, Institute of Cardiovascular Diseases, Madras Medical Mission, 4A, Dr J. J. Nagar, Mogappair, Chennai 600 050, India
e-mail: icvd{at}eth.net
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Abstract
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A 50-year-old man was diagnosed as having absent pulmonary valve syndrome with aneurysmal dilatation of the pulmonary arteries. He also had a large subaortic ventricular septal defect and severe aortic regurgitation. He underwent successful intracardiac repair and aortic valve replacement. This case is presented in view of its rarity and controversies in the surgical management.
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Introduction
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The absent pulmonary valve syndrome is a rare anomaly associated with aneurysmal dilatation of the pulmonary trunk, variable degrees of pulmonary stenosis, with or without malalignment of the ventricular septum. When the ventricular septum is malaligned, the condition is referred to as tetralogy of Fallot with absent pulmonary valve [1]. The diagnosis of this anomaly is usually made in the neonatal period with the children presenting with severe respiratory distress due to the compression of the tracheo-bronchial tree by the dilated pulmonary arteries [2]. Others may present later in life with cyanosis and progressive exercise intolerance.
This report describes the rare presentation of this anomaly in a 50-year-old man along with severe aortic regurgitation. He underwent successful intracardiac repair and aortic valve replacement.
A 50-year-old man presented with New York Heart Association (NYHA) class III dyspnea on exertion for the last 3 months. On examination, the only positive finding was the presence of grade III/VI end systolic murmur at base. On pulse oximetry, the saturations were 92% on room air.
Transthoracic two-dimensional echocardiography revealed normally related great arteries, a large subaortic ventricular septal defect (VSD) and severe aortic regurgitation. There was moderate pulmonary stenosis (peak gradient = 56 mm Hg), moderate pulmonary regurgitation, and the pulmonary valve leaflets could not be visualized.
Cardiac catheterization data was as follows: baseline saturation of 94.5%, severe aortic regurgitation, large subaortic VSD and a right aortic arch (Fig 1).
The pulmonary arteries were aneurysmal with right pulmonary artery and left pulmonary artery measuring 22.5 and 25.4 mm, respectively (Fig 2).
There was severe annular pulmonary stenosis (gradient = 93 mm Hg) with moderate pulmonary regurgitation. Coronary angiogram was normal. Flexible bronchoscopy revealed external compression on the left main bronchus.
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Fig 1. Preoperative angiogram showing aorta (AO), aortic regurgitation (AR), and ventricular septal defect (VSD). (LV = left ventricle; RV = right ventricle.)
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Fig 2. Preoperative pulmonary artery injection showing aneurysmal main pulmonary artery (MPA) and dilated right pulmonary artery (RPA).
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The patient underwent intracardiac repair and aortic valve replacement using standard cannulation and myocardial protection techniques. Infundibular resection and VSD closure were performed through the right atrium. The right coronary cusp of the aortic valve was seen prolapsing through the VSD. Since the other two cusps were also degenerated, the valve was replaced with a 23-size TTK Chitra valve (TTK Pharma Ltd, Chennai, India). A longitudinal right ventriculotomy was then made and was extended across the pulmonary annulus onto the main pulmonary artery. The pulmonary valve was found to be rudimentary. The main pulmonary artery was then transected and the anterior wall was plicated using a running Prolene (Ethicon, Somerville, NJ) suture. The right ventricle to pulmonary artery continuity was established using a #25 pulmonary homograft. Postoperative transesophageal echocardiography revealed no residual VSD and a right ventricular outflow tract gradient of 10 mm Hg with trivial pulmonary regurgitation. The prosthetic valve was functioning normally. The patient made an uneventful recovery, and at 1-year follow-up remains in NYHA class 1.
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Comment
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The absent pulmonary valve syndrome is a rare congenital defect, which usually manifests in the neonatal period. Most of the reports, therefore, have concentrated on the management of this syndrome in the neonates [3, 4]. The case that we have described is an unusual presentation of this anomaly. The patient, a 50-year-old man, had a relatively asymptomatic childhood, with normal physical growth. On searching the literature, we encountered one similar case report. Ohtsuka and colleagues have reported tetralogy of Fallot with absent pulmonary valve, aortic regurgitation, and coronary artery–pulmonary artery fistula in a 54-year-old man [5]. Their patient underwent intracardiac repair using a transannular pericardial patch with a monocusp, aortic valve replacement, and ligation of the coronary fistula. The management of this condition, however, remains controversial. The main issues are (a) whether or not to insert a pulmonary valve; and (b) whether to do or not to do a pulmonary arterioplasty. Pinsky and associates have achieved good results without pulmonary valve insertion [3]. On the other hand, Ilbawi and coworkers [4] and Mavroudis and colleagues [6] advocate the insertion of a pulmonary valve for a better outcome. All the above reports, however, have dealt with infants and children only. In our experience (unpublished), adult patients with tetralogy of Fallot do not tolerate pulmonary regurgitation. We, therefore, used a pulmonary homograft to restore the right ventricle to pulmonary artery continuity.
Various authors have stressed the importance of performing pulmonary arterioplasty in infants with absent pulmonary valve syndrome [3, 4, 6]. Whether the same conclusion can be extrapolated to adults is unclear. Since our patient had evidence of bronchial compression preoperatively, we performed pulmonary artery plication.
In conclusion, absent pulmonary valve syndrome is a rare condition, which may occasionally present in adulthood. Management consists of intracardiac repair. The insertion of a pulmonary valve in these patients may probably result in a better outcome. Pulmonary arterioplasty should be performed in patients with airway compression.
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References
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Lev M., Eckmer F.A.O. The pathologic anatomy of tetralogy of Fallot and its variations. Chest 1964;45:251-261.
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Nagao G.I., Daoud G.I., McAdams A.J., Shwartz A.C., Kaplan S. Cardiovascular anomalies associated with tetralogy of Fallot. Am J Cardiol 1967;20:206-221.[Medline]
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Pinsky W.W., Nihill M.R., Mullins C.E., Harrison G., McNamara D.G. The absent pulmonary valve syndrome. Considerations of management. Circulation 1978;57:159-162.[Abstract/Free Full Text]
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Ilbawi M.N., Idriss F.S., Muster A.J., Wessel H.U., Paul M.H., DeLeon S.Y. Tetralogy of Fallot with absent pulmonary valve. Should valve insertion be a part of intracardiac repair?. J Thorac Cardiovasc Surg 1981;81:806-815.
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Ohtsuka G., Tsuchiya K., Noji S., Sasaki N., Iida Y. A successful repair of tetralogy of Fallot with aortic valve regurgitation, coronary artery-pulmonary artery fistula and absent pulmonary valve in a 54 year old man. J Jpn Assoc Thorac Surg 1993;41:2250-2255.
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Mavroudis C., Turley K., Stanger P., Ebert P.A. Surgical treatment of tetralogy of Fallot with absent pulmonary valve. J Cardiovasc Surg (Torino) 1983;24:603-609.[Medline]
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Abstract
Introduction
