Ann Thorac Surg 2001;71:1016-1018
© 2001 The Society of Thoracic Surgeons
Case report
An unusual cause of severe cyanosis in infancy
Susan P. Etheridge, MDa,
Herbert D. Ruttenberg, MDa,
Richard V. Williams, MDa
a Department of Pediatrics, Division of Pediatric Cardiology, University of Utah and Primary Children’s Medical Center, Salt Lake City, Utah, USA
Accepted for publication April 8, 2000.
Address reprint requests to Dr Etheridge, Department of Pediatrics, Division of Pediatric Cardiology, University of Utah and Primary Children’s Medical Center, 100 North Medical Dr, Salt Lake City, UT 84113
e-mail: pcsether{at}ihc.com
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Abstract
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An infant presented with cyanosis due to a diaphragmatic Morgagni hernia compromising right ventricular diastolic filling and resulting in right-to-left atrial-level shunting as demonstrated by contrast echocardiography. There was complete resolution of cyanosis after repair of the hernia.
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Introduction
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The cyanotic infant is a distressing medical presentation with diverse and heterogeneous causes. Cardiac and pulmonary etiologies are those most commonly encountered. Cyanotic congenital heart disease usually presents at birth or within the first few weeks of life with pulmonary blood flow dependent on the patency of the ductus arteriosus. Pulmonary causes of cyanosis such as pneumonia and bronchiolitis are sometimes difficult to distinguish from cardiac causes. Hematologic, toxic, and metabolic causes of cyanosis occur less commonly. Methemoglobinemia is an uncommon cause of cyanosis in the infant and may be due to inheritable abnormalities in hemoglobin or red blood cell enzymes responsible for reduction or acquired causes involving exposure to drugs or toxins. We present the case of an infant with an unusual cause of cyanosis.
A 3-month-old infant was referred to our hospital because of cyanosis. She was born full term by normal vaginal delivery at a hospital in another state. She was noted to be cyanotic in the newborn period but this resolved and no evaluation was undertaken. After discharge from the newborn nursery she continued to have intermittent episodes of cyanosis that seemed to be positional, occurring when she was lying flat in either the supine or prone position and when she was in her car seat. She was irritable and had difficulty breast feeding unless lying on her right side. The family moved to our area several weeks before presentation. She was seen by a local pediatrician and noted to be cyanotic with oxygen saturations in the 60% range.
Upon admission to our hospital she was noted to have moderate cyanosis and tachypnea with respiratory rates of 40 to 50 breaths per minute and an oxygen saturation of 69%. Her heart rate was 160 beats per minute. Her cardiac examination was normal and no murmur was noted. Her liver was palpable 3 cm below the costal margin.
A chest radiograph (Fig 1) demonstrated an enlarged, boot-shaped heart, normal pulmonary blood flow, and loops of bowel within the thorax in the anterior mediastinum suggesting a Morgagni diaphragmatic hernia. She was admitted and a sedated echocardiogram was performed. Despite poor imaging due to air-filled bowel loops within the chest, anterior to the heart, the heart was felt to be structurally normal. The right ventricle was extrinsically compressed with a slit-like right ventricle chamber (Fig 2). A contrast echocardiogram was performed with the administration of an intravenous bolus of agitated saline and demonstrated right-to-left shunting at the atrial level with rapid opacification of the left atrium and left ventricle (Fig 3).
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Fig 1. (A) Posterioanterior and (B) lateral chest radiographs demonstrating air-filled loop of bowel within thorax.
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Fig 2. Apical four-chamber view from the initial echocardiogram. There appears to be external compression of the right ventricle giving it a slit-like appearance. (LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.)
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Fig 3. Apical four-chamber view from the contrast echocardiogram with nearly complete opacification of the left atrium (LA) and left ventricle (LV). (RA = right atrium; RV = right ventricle.)
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During the sedated echocardiogram, the patient had episodes of desaturation with oxygen saturations to 30% and marked cyanosis. This improved as she awakened from sedation. During the first hospital day, she continued to have episodes of increased cyanosis, especially with feeding. In the evening, she passed a large amount of gas, had a bowel movement, her color acutely improved, and saturations increased to 90%.
The following morning she was taken to the operating room for reduction and repair of the Morgagni hernia. She did well in the operative and perioperative period. After surgery, her oxygen saturations were normal on a small amount of oxygen by nasal cannula. A subsequent echocardiogram demonstrated normalization of the right ventricular size and systolic function. No atrial-level shunt was identified. She was discharged on no medications and on oxygen at 1/8 L/minute. She has done well since discharge. She is off oxygen and remains acyanotic. She breastfeeds well and has been growing and developing normally.
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Comment
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This child presented with some features suggesting tetralogy of Fallot, including episodic cyanosis and a boot-shaped heart on chest radiography but without a murmur of pulmonary outflow tract obstruction. This is a report of cyanosis secondary to external compression from a Morgagni diaphragmatic hernia with the acute resolution of cyanosis after the passage of air and stool. Presumably, this resulted in decompression of the bowel and a decrease in the right ventricular compression. Extrinsic compression of the right ventricle by air- or fluid-filled loops of bowel impaired right ventricular diastolic filling resulting in right-to-left shunting through a patent foramen ovale. This was confirmed by a contrast echocardiogram whereby saline microcavitations were injected into a peripheral vein and demonstrated a shunt at the atrial level in a patient with difficult imaging owing to the abdominal contents within the thoracic cavity.
Cyanosis in the infant has respiratory, cardiac, central nervous system, hematologic, and metabolic causes. Cardiac and respiratory causes are the most common and are often difficult to distinguish. The common cyanotic congenital cardiac conditions include tetralogy of Fallot, transposition of the great arteries, truncus arteriosus, tricuspid atresia, and hypoplastic left heart syndrome, to name a few. Because of their potential lethality, these lesions require prompt recognition and management. This is emphasized by the findings of the New England Regional Infant Cardiac Program [1], in which more than one third of the infants with potentially lethal congenital heart disease presented with cyanosis or cyanosis and respiratory symptoms.
We report a child with cyanosis due to a diaphragmatic hernia of the foramen of Morgagni compressing the right ventricle and causing impaired diastolic filling and a right-to-left atrial-level shunt. This type of hernia is uncommon and usually presents with feeding problems and bowel obstruction or is an incidental finding on chest radiography. Respiratory symptoms are unusual and cyanosis has not been described. Interestingly, congenital heart disease is present in as many as 58% of children with this diagnosis [2] and cardiac tamponade has been described [3]. Because these defects are at risk for incarceration and strangulation, early recognition and repair are important [4].
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References
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Fyler D.C., Buckley L.P., Hellenbrand W.E., Cohn H.E. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980;65(Suppl):376-460.
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Berman L., Stringer D., Ein S.H., Shandling B. The late-presenting pediatric Morgagni hernia: a benign condition. J Pediatr Surg 1989;24:970-972.[Medline]
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DeFonseca J.M.B., Davies M.R.Q., Bolton K.D. Congenital hydropericardium associated with the herniation of part of the liver into the pericardial sac. J Pediatr Surg 1987;22:851-853.[Medline]
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Arensman R.M., Bambini D.A. Congenital diaphragmatic hernias. In: Ashcraft K.W., ed. Pediatric surgery. Philadelphia: WB Saunders, 2000:311-317.
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Abstract
Introduction
