Dextrocardia

Gregor P.M. Mannes, M.D.;(*1) Eric J. van der Jagt, M.D.;([unkeyable]) Bert Wouters, M.D.;([unkeyable]) and Pieter E. Postmus, M.D.([unkeyable])

A 15-year-old girl complained of periods of intermittent dyspnea with wheezing. Smoke and cold exposure worsened the dyspnea. She had normal exercise tolerance. There were no intermittent infections, and no pneumonia during early childhood. Her general practitioner asked for a chest roentgenogram (Fig 1), which showed abnormal position of the heart.

She was referred to a cardiologist. At physical examination the heart was somewhat displaced to the right. There were no thrills, and auscultation revealed an opening snap but no murmurs.

The ECG showed a regular sinus rhythm, with normal axis and complexes. Results of fluoroscopic examination disclosed a normal-sized heart, slightly rotated and displaced to the right side, with normal pulsations. It was concluded that there was no (congenital) heart disease, and she was admitted to the department of pulmonary diseases for further evaluation.

The girl appeared to be healthy, and she had a normal pulse rate and blood pressure. At physical examination there were no additional findings; in particular, the lung sounds were normal. Routine laboratory investigations were normal, except a slightly increased number of eosinophils and an increased serum IgE level.

Some of the intracutaneous allergy tests were positive, eg, house dust mite. At spirometric study, a diminished vital capacity was found, with an expiratory bronchus obstruction. A ventilation-perfusion scan was normal. Bronchography (Fig 2) showed an abnormality that was confirmed by bronchoscopic examination.

Diagnosis: Anomaly of the right upper lobe bronchus

The chest roentgenogram showed the heart to be displaced to the right, with a prominent aorta, probably because of the rotation. The right sinus pleurae were obtuse, and no minor fissure was visible. Bronchography showed no right upper lobe bronchus originating at the normal site. At the usual site of the middle lobe bronchus was an orifice to a common stem, from which one branch sharply bent cranially. This might have been the upper lobe bronchus with a bifurcation instead of the normal trifurcation, an otherwise well-known variation.[1]

Bronchoscopic study results seemed to show a common bronchus for the right upper and middle lobe, which bifurcated soon after its beginning. Just above the lower lobe bronchus was a small apical segmental bronchus. The left side showed no abnormalities.

At bronchography the incidence of a bronchus arising from an abnormal place is 0.25 to 10 percent.[2-4] Usually there are minor abnormalities in the segmental bronchi, which are frequently accidental findings. The largest number (3 percent) of anatomic variations of the bronchial tree are found in the left upper lobe.[1] In a series of 1,000 bronchograms, Le Roux[2] found 30 anomalies in the arrangements of bronchi to the right upper lobe. Atwell,[3] in a series of 1,200 bronchograms, only found 27 anomalies in the primary or lobe branches. The pattern found in our case is very rare;[2,5] Huizinga and Smelt[1] described it first. The etiology is unknown, but there must have been a disturbance early in gestation, approximately at the 30th or 32nd day, when the lobar bronchi appear as outgrowths of the primary bronchi.[6]

Bronchial atresia as the explanation of the anomaly in this patient is less likely, because there was no clear mucoid impact or hyperlucency of the effected lung.[5] The displacement of the heart probably was a result of the changed local anatomy.

(*) Pulmonology, ([unkeyable]) Diagnostic Radiology, and ([unkeyable]) Otolaryngology, University Hospital, Groningen, The Netherlands.

REFERENCES

1 Huizinga E, Smelt G. Bronchography. Assen, The Netherlands: Van Gorcum, 1949

2 Le Roux BT. Anatomical abnormalities of the right upper bronchus. J Thorac Cardiovasc Surg 1962; 44:225-27

3 Atwell SW. Major anomalies of the tracheobronchial tree. Dis Chest 1967; 52:611-15

4 Ritsema GH. Ectopic right bronchus, indication for bronchography. AJR 1983; 140:671-74

5 Landing BH, Dixon LG. Congenital malformations and genetic disorders of the respiratory tract. Am Rev Respir Dis 1979; 120:151-85

6 Boyden EA. Developmental anomalies of the lungs. Am J Surg 1955; 89:79-89

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