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AN ADULT PATIENT WITH CONGENITAL HEART DISEASE

WS Choo MBBS, MRCP
Faculty of Medicine, Penang Medical College

Address for correspondence: Dr Choo Wai Sun, Lecturer in Medicine, Faculty of Medicine, Penang Medical College,
4, Jalan Sepoy Lines, 10450 Penang, Malaysia. Tel: +6012-3280 140, Fax: +604-2276 529, Email: waisun76@yahoo.com

Funding: None.

Conflict of interest: None to declare.

CASE HISTORY

This is the chest x-ray of an adult patient who is under follow-up for congenital heart problem. She has previous history of Blalock-Taussig shunt inserted for univentricular heart with rudimentary right ventricle as well as tricuspid and pulmonary atresia.

Figure 1Figure 2

QUESTION:

1. What are the three abnormalities in this chest x-ray (Figure 1)?
2. What is the clinical diagnosis?
3. What are the conditions that can be associated with this diagnosis?
4. What is the electrocardiogram (ECG) findings in this patient (Figure 2)?

ANSWER:

1. (a) Dextrocardia (“Left” x-ray marker was at the correct position)
   (b) Gastric shadow on the right side
   (c) Scoliosis
2. Detrocardia with situs inversus, as both the aortic arch and gastric shadow are located on the right side.
3. Several congenital problems can be associated with detrocardia. These are Kartagener’s syndrome, double outlet right ventricle, endocardial cushion defects, pulmonary stenosis or atresia, transposition of the great arteries and ventricular septal defect.
4. A 12-leads left-sided electrocardiography will typically shows an inversion of the electrical waves in leads I, II, aVR and reverse R-wave progression across the precordial leads i.e. with the tallest R wave in V1 progressively decreasing in amplitude in leads V2 to V6.

DISCUSSION

The normal arrangement of major visceral organs in the body is referred to as situs solitus. Dextrocardia is a congenital defect in which the heart is situated on the right side of the chest. Situs inversus occurs when the visceral organs are placed mirror-imaged to their normal position. If the heart is also swapped to the right side of the thorax, it is known as situs inversus with dextrocardia or situs inversus totalis.¹ This condition is usually associated with 3-5% incidence of congenital heart disease especially with transposition of great arteries. Situs inversus with levocardia (the heart remains in the normal left side of the thorax) is rare² and it is almost always associated with congenital heart disease. Kartagener’s syndrome is characterized by sinus inversus, chronic paranasal sinusitis and bronchiectasis.³ Diagnosis can be made by chest x-ray and ECG, although CT scan or MRI maybe needed for more complex forms of congenital disease related to dextrocardia.

REFERENCES

1. Maldjian PD, Saric M. Approach to dextrocardia in adults: review. AJR Am J Roentgenol. 2007;188(6 suppl):S39-49. [PubMed]
2. Gindes L, Hegesh J, Barkai G, et al. Isolated levocardia: prenatal diagnosis, clinical importance, and literature review. J Ultrasound Med. 2007;26(3):361-5. [PubMed]
3. Kinney TB, DeLuca SA. Kartagener's syndrome.Am Fam Physician.1991;44(1):133-4. [PubMed]