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J Thorac Cardiovasc Surg 2005;130:213-215
© 2005 The American Association for Thoracic Surgery


Brief Communication

Aortic root replacement in a patient with pulmonary dysfunction caused by severe chest deformity associated with Marfan syndrome

Iki Adachi, MD a , Hitoshi Ogino, MD a , * , Hideaki Imanaka, MD b , Hitoshi Matsuda, MD a , Kenji Minatoya, MD a , Hiroaki Sasaki, MD a

a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
b Intensive Care Unit, National Cardiovascular Center, Osaka, Japan

Received for publication January 3, 2005; revisions received January 15, 2005; accepted for publication February 8, 2005.

* Address for reprints: Hitoshi Ogino, MD, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565, Japan (Email: hogino{at}hsp.ncvc.go.jp).

Great advances in respiratory management have recently widened the indication for cardiovascular surgery for patients with pulmonary dysfunction. We describe a patient with an extremely low vital capacity caused by severe chest deformity who tolerated aortic root replacement as a result of adequate respiratory management, including bilevel positive airway pressure (BiPAP) ventilation.

Clinical Summary

A 39-year-old man with Marfan syndrome was scheduled to undergo aortic root replacement for annuloaortic ectasia and aortic insufficiency. He had been receiving home oxygen therapy for 10 years and nocturnal use of nasal continuous positive airway pressure ventilation for 1 year because of frequent episodes of respiratory failure.

On admission, he showed respiratory distress at grade IV of the Hugh-Jones classification. Spirometry showed a severe restrictive pattern: vital capacity of 1080 mL (28% of predicted value) and forced expiratory volume in 1 second of 890 mL. Arterial blood gases (ABGs) under 1 L of oxygen inhalation revealed metabolically compensated hypercapnia: pH, 7.41; PaCO 2, 60.8 mm Hg; PaO 2, 80.4 mm Hg; base excess, 10.9 mmol/L. Chest radiography showed cardiomegaly with mild lung congestion and severe scoliosis (Figure 1). Computed tomographic (CT) scans revealed the dilated aortic root (62 mm in diameter) and marked elongation of the descending aorta along the vertebral bodies (Figure 2). Transthoracic echocardiography revealed a redundant tricuspid aortic valve with severe regurgitation. The left ventricular ejection fraction was 45%, as estimated by ventriculography.


Figure 1
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Figure 1. Chest radiograph shows cardiomegaly with mild lung congestion and severe scoliosis.

 

Figure 2
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Figure 2. CT scan reveals dilatation of the aortic root (62 mm in diameter) and marked elongation of the descending aorta along the vertebral bodies.

 
At the time of sternotomy, we paid particular attention to avoiding lung injury and to not opening the pleural spaces. After establishment of cardiopulmonary bypass, the aortic root was replaced with a composite graft by use of the Carrel patch technique because aortic leaflets were severely degenerated. Weaning from bypass was uneventful.

ABGs on admission to the intensive care unit were favorable: pH, 7.38; PaCO 2, 50.1 mm Hg; PaO 2, 167.2 mm Hg; base excess, 4.4 mmol/L (synchronized intermittent mandatory ventilation; fraction of inspired oxygen of 0.5; respiratory rate of 12 breaths/min; tidal volume of 460 mL; positive end-expiratory pressure of 4 cm H2O). The trachea was extubated 4 hours after the admission, when he was completely alert and oriented. However, he had respiratory distress immediately after the extubation, and his respiratory rate exceeded 40 breaths/min. Subsequent ABGs with 10 L of oxygen showed respiratory acidosis: pH, 7.299; PaCO 2, 57.1 mm Hg; PaO 2, 130.7 mm Hg; base excess, 1.4 mmol/L. Therefore, we applied BiPAP ventilatory support (BiPAP vision, Respironics, Inc) through a face mask. The inspiratory and expiratory pressures were set at 10 and 4 cm H2O, respectively. The respiratory rate decreased to the mid-20s in beats per minute, and ABGs showed an improvement of acidemia: pH, 7.359; PaCO 2, 52.3 mm Hg; PaO 2, 118.3 mm Hg; base excess, 3.1 mmol/L. We could terminate BiPAP ventilation the next morning and resumed nasal continuous positive airway pressure ventilation for subsequent respiratory support. The patient was discharged from the hospital without any complications. Spirometry examined before discharge showed no remarkable change compared with the preoperative values.

Discussion

Pectus excavatum and scoliosis, commonly associated with Marfan syndrome, cause restrictive pulmonary dysfunction, although the connective tissue defects in Marfan syndrome seem to have little clinical effect on such dysfunction.1Go Accordingly, patients with severe chest deformities are still predisposed to a risk of respiratory deterioration, even after a successful extubation. Avoidance of reintubation is of great importance because mortality increases more than 7-fold with reintubation and subsequent mechanicalventilation.2Go

The BiPAP ventilator is one of relatively novel noninvasive ventilators and has gained increasingly widespread acceptance in acute and chronic respiratory failure.3Go However, the rationale of BiPAP application after elective extubation is still controversial. Ferrer and colleagues4Go reported that BiPAP facilitated weaning from mechanical ventilation, whereas Esteban and associates5Go insisted BiPAP did not prevent reintubation in patients who had respiratory failure after extubation. The maximum feature of BiPAP ventilation is the ability to provide ventilatory assistance noninvasively for alert and spontaneously breathing patients. It is expected that inspiratory pressure support augments ventilation, even in patients with decreased vital capacity, and positive end-expiratory pressure restores functional residual capacity. As a result, the work of breathing decreases. We believe decrease of respiratory rate was the clinical sign of reduced respiratory workload in the current patient.

In conclusion, we report a case of severe pulmonary dysfunction caused by chest deformity in a patient who tolerated aortic root replacement as a result of adequate respiratory management. Our experience should reassure patients with severe respiratory dysfunction necessitating cardiovascular surgery.

References

  1. Streeten EA, Murphy EA, Pyeritz RE. Pulmonary function in the Marfan syndrome. Chest 1987;91:408-412.[Abstract/Free Full Text]
  2. Epstein SK, Ciubotaru RL, Wong JB. Effect of failed extubation on the outcome of mechanical ventilation. Chest 1997;112:186-192.[Abstract/Free Full Text]
  3. Tokuda Y, Matsumoto M, Sugita T, et al. Bilateral diaphragmatic paralysis after aortic surgery with topical hypothermia. ventilatory assistance by means of nasal mask bilevel positive pressure. J Thorac Cardiovasc Surg 2003;125:1158-1159.[Free Full Text]
  4. Ferrer M, Esquinas A, Arancibia F, et al. Noninvasive ventilation during persistent weaning failure. a randomized controlled trial. Am J Respir Crit Care Med 2003;168:70-6.[Abstract/Free Full Text]
  5. Esteban A, Frutos-Vivar F, Ferguson ND, et al. Noninvasive positive-pressure ventilation for respiratory failure after extubation. N Engl J Med 2004;350:2452-2460.[Abstract/Free Full Text]



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