Kartagener's syndrome: clinical presentation and cardiosurgical aspects.

BACKGROUND

Long-term observations in patients with Kartagener's syndrome (situs inversus, bronchiectasis, and sinusitis) are rare. The role of additional cardiac malformations and their surgical repair is not well known.

METHODS

Nine patients (5 female and 4 male) with Kartagener's syndrome were identified and followed. Four patients had associated cardiac anomalies; 4 underwent total surgical repair at the ages of 4 (2 patients), 7, and 34 years.

RESULTS

The postoperative period was uneventful, and these 4 patients are doing well 7 months and 2, 9, and 19 years after repair. The other patients are being treated with conservative therapy and are in relatively good condition.

CONCLUSIONS

This disease can be temporarily benign when treated with antibiotics and physiotherapy. Associated cardiac anomalies seem to be quite common, and such patients need careful cardiologic follow-up. Surgical intervention can be safely performed in patients suffering from Kartagener's syndrome associated with a congenital cardiac malformation and produces good long-term results. Bilateral lung transplantation seems to be the therapy of choice in patients with respiratory insufficiency but without concomitant cardiac anomalies.