Ann Thorac Surg 2004;77:1810-1811
© 2004 The Society of Thoracic Surgeons
Case report
Mitral valve repair in a 55-year-old man with left lung agenesis
Ghassan S. Musleha,
Pedro Fernandeza,
Pankaj K. Jhaa,
Ragheb Hasana*
a Department of Cardiothoracic Surgery, Manchester Royal Infirmary, Manchester, United Kingdom
Accepted for publication May 12, 2003.
* Address reprint requests to Mr Hasan, Cardiothoracic Unit, Manchester Royal Infirmary, Oxford Rd, Manchester M13 9WL, UK
e-mail: r.hasan{at}man.ac.uk
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Abstract
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We report on a 55-year-old man who was diagnosed with agenesis of the left lung in childhood. He was essentially asymptomatic until he was 53 years of age, when he became symptomatic with exertional dyspnea due to severe mitral regurgitation. We performed mitral valve repair using a median sternotomy incision approach.
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Introduction
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Lung agenesis is a rare condition. It is usually diagnosed soon after birth. It is associated with other anomalies, mainly of the cardiovascular and skeletal systems. Although in half of all reported cases, patients die either at birth or within the first 5 years of life, this condition is compatible with long-term survival and normal growth [1]. The successful repair of ventricular sepal defect and patent ductus arteriosus in an infant with left lung agenesis was reported previously [2]. There are no previous reports of cardiac procedures in adults with lung agenesis.
A 55-year-old man was admitted to our institute with a 2-year history of exertional dyspnea (New York Heart Association [NYHA] class III). He was known to have agenesis of the left lung since the age of 8 but had remained essentially asymptomatic. There was no history of rheumatic fever or a heart murmur. The patient's pulse rate was 72 beats per minute and regular. His blood pressure was 145/90 mm Hg. His jugular venous pulse was not elevated. His respiratory rate was 14 breaths per minute. The air entry was normal over the right hemithorax and upper left chest, but it was absent over the middle and lower left hemithorax. There was a class III pansystolic murmur over the apex, radiating to the axilla.
An electrocardiogram showed sinus rhythm and left ventricular hypertrophy. A chest roentgenogram showed an absence of lung markings in the left middle and lower zones and a left-sided shift of the mediastinum (Fig 1).
Transthoracic echocardiography confirmed severe mitral regurgitation. The left ventricle was dilated, with an end diastole diameter of 6.9 mm and an ejection fraction of 60%. The left atrial diameter was 4.6 mm. Coronary and left ventricular angiography showed normal coronary arteries and severe mitral incompetence.
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Fig 1. Chest roentgenogram showing the absence of lung markings in the left middle and lower zones and a left-sided shift of the mediastinum (arrow).
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A median sternotomy approach was used. On exploring the mediastinum, we found that the right lung completely covered the heart and extended all the way into the left chest, filling the upper half of the left hemithorax. Tedious dissection of the right pleura was performed. There was extensive vascularity around the pericardium. Cardiopulmonary bypass was established by cannulating the ascending aorta and using two caval cannulas. The heart was rotated to the left, and the interatrial groove was rotated anteriorly. This made access to the mitral valve through a left atriotomy easy, but the valve was deep. On inspecting the mitral valve, we found chordal rupture and prolapse of the adjacent segments between P2 and P3. The prolapse was repaired using the Alfieri edge-to-edge technique [3]. A size-35 mitral ring (St. Jude Medical, St. Paul, MN) was inserted. A perioperative echocardiogram confirmed no mitral regurgitation. The postoperative course was uncomplicated.
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Comment
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Pulmonary agenesis is a rare congenital anomaly. Bilateral pulmonary agenesis was reported in a live infant with esophageal atresia and first arch syndrome [4].
The incidence of unilateral lung agenesis was estimated at 1 in 10,000 to 15,000 autopsies [5]. The etiology is not known, but vitamin A deficiency, viral agents, and genetic factors have been suggested. Associated malformations affecting other systems, notably the cardiovascular, genitourinary, gastrointestinal, and musculoskeletal systems, have been reported [6]. These anomalies have a significant influence on prognosis. Mortality is higher in right lung agenesis. It is probably related to tracheal compression by the aorta in some cases [7]. Lung agenesis is usually detected soon after birth because of the early onset of symptoms.
The usual chest roentgenogram findings in such cases are a marked ipsilateral mediastinal shift and a hypertrophied normal lung extending to the opposite side. Contralateral mediastinal shifts in association with the eventration of the diaphragm have been reported previously [8].
Magnetic resonance imaging scans are the method of choice to define the entire spectrum of airway and vascular abnormalities [9]. Contrast-enhanced computed tomography scans help exclude vascular compression of the major airways [10].
There are several approaches for mitral valve repair. We were concerned that median sternotomy might present some difficulty because the right lung crossed over into the left chest. Median sternotomy was performed with the lung deflated. The right lung was mobilized out of the left hemithorax and off the pericardium. The rest of the procedure was conducted as with any conventional mitral valve repair, by left atriotomy. The heart was rotated more to the left. This moved the interatrial groove anteriorly and made access easier, although the valve was deep. The patient was reviewed at follow-up and was found to be in NYHA class I.
Mitral valve repair or replacement can be performed safely and with good intermediate results.
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References
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- Maltz D.L., Nadas A.S. Agenesis of the lung: presentation of 8 new cases and review of literature. Pediatrics 1968;42:175-188.[Abstract/Free Full Text]
- Akishima S., Honda M., Kanno M., Shinoka T., Hagino I., Tei I. A successful repair of ventricular septal defect and patent ductus arteriosus associated with pulmonary hypertension in an infant with left lung a genesis. Nihon Kyobu Geka Gakkai Zasshi 1994;42(12):2247-2251.
- Alfieri O., Maisano F., De Bonis M., et al. The double-orifice technique in mitral valve repair: a simple solution for complex problems. J Torca Cardiovasc Surg 2001;122:674-681.
- De Buse P.J., Movies G. Bilateral pulmonary agenesis, oesophageal atresia, and the first arch syndrome. Thorax 1973;28:526-528.[Abstract/Free Full Text]
- Olcott CT, Dodey SW. Agenesis of lung in an infant. Am J Dis Child 1945;65:776–80
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- Newman B., Gondor M. MR evaluation of right pulmonary agenesis and vascular airway compression in pediatric patients. Am J Roentgenol 1997;168(1):55-58.[Abstract/Free Full Text]
- Argent A.C., Cremin B.J. Computed tomography in agenesis of the lung in infants. Br J Radiol 1992;65(771):221-224.[Abstract/Free Full Text]
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Complete myocardial revascularization and Dor technique in a case of left lung agenesis
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Abstract
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