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An 18-year-old, asymptomatic male underwent a routine chest x-ray examination which showed a pathological result. It revealed a characteristic crescent-like shadow in the right lower lung field, resembling a muslim sword (scimitar), responsible for the name of the underlying disease. Scimitar syndrome is a rare congenital malformation with a wide clinical spectrum ranging from asymptomatic to severe disturbances manifesting in childhood. The typical findings are hypoplasia of the right lung with marked mediastinal shift to the right and dextrocardia, reduced right pulmonary artery perfusion with maintained bronchial connection, anomalous arterial supply to the right lower lobe from the aorta and abnormal draining of the enlarged right pulmonary vein into the pulmonary circulation, producing the characteristic radiographic "scimitar sign". The development of right ventricular failure due to long-standing right ventricular overload and recurrent respiratory infections depend on the severity of anatomical abnormalities and functional disorders, sometimes requiring surgical intervention. Nowadays, sophisticated imaging techniques allow precise and rapid diagnosis of these complex abnormalities and their functional implications. The findings for our patient differed from the classic constellation, with a regular emptying of the enlarged right pulmonary vein into the left atrium, leading to a decreased burden on the central haemodynamics. In our very rare case of a scimitar syndrome variant, the functional disorders were negligible.

Primary ciliary dyskinesia is the generic term for a heterogeneous group of inherited diseases in which ciliary ultrastructure is defective and as a consequence ciliary motility is disturbed. An international consensus on the diagnostic criteria has not yet been reached. This paper reviews some recent findings which are useful in the diagnosis of the disease and attempts to establish the best diagnostic criteria. The marker symptoms are chronic bronchitis, otitis, and sinusitis since childhood. Additionally, one or more of the following criteria must be present: Kartagener syndrome, a dextrocardia situation, markedly reduced frequency in ciliary motility, or an essential ultrastructure deviation in more than 20% of the square cuts (e.g. reduced number of dynein arms). Biopsy of the ciliated mucosa is usually required for the above criteria and is studied by vital microscopy and transmission electron microscopy. Primary and secondary ciliary dyskinesia can be distinguished by these methods and the rare case of PCD without ultrastructure deficiency ruled out. In special cases a cell culture is recommended for the diagnosis. Practical aspects of the sampling methods and diagnostic pitfalls are reviewed.

Report on a 28-year-old female patient with classic "scimitar syndrome" (dextroposition of the heart, hypoplasia of the right lung and pulmonary veins opening into the inferior vena cava). The condition was diagnosed on the basis of chest X-ray findings in which the most striking feature was the scimitar-like course of the right pulmonary veins which gives the syndrome its name. The diagnosis was confirmed and the shunt volume determined by cardiac catheterization. Since in this case there were no additional deformities such as atrial septum defect, ventricular septum defect or patent ductus botalli and the shunt volume was small, surgical correction was not required.

This paper reports on four different types of dextrocardia in situs solitus and situs inversus. The topography of the atrial and ventricular cavities, of the great vessels and of the coronary arteries is described in detail. The value of the selective coronary arteriography in differentiation between the types of dextrocardia is discussed.

Corrected transposition of the great arteries is often associated with other cardiac anomalies. We report our experience with the correction of the associated cardiac defects in 13 cases: ventricular septal defect with pulmonary hypertension (5 cases), ventricular septal defect with obstruction of pulmonary outflow tract (5 cases), obstruction of pulmonary outflow tract (2 cases), ostium primum defect (1 case). The possible surgical approaches for repair of the cardiac anomalies are described and the results of the operative correction are communicated. Precise diagnosis, the knowledge of the unusual disposition of the conducting tissues and new surgical approaches are prerequisite for successful correction.

In two of 81 patients in whom a Blalock-Taussig shunt was created due to pulmonary atresia, the anastomosis was inadvertently placed on the pulmonary vein. The first case was a newborn with mirror-image dextrocardia, pulmonary valve atresia and a high ventricular septal defect. Subsequent to a Rashkind maneuver and treatment with prostaglandins, at the age of eleven days the shunt procedure was performed. Right sided pulmonary edema developed twelve hours after surgery and the infant died on the second postoperative day. Anastomosis of the shunt to the right pulmonary vein was revealed at autopsy. The second case was a six year-old boy admitted for corrective surgery with pulmonary valve atresia, main pulmonary artery atresia, large ventricular septal defect and patent ductus arteriosus. Because of additional systemic-pulmonary collaterals, the larger collaterals were ligated and the Blalock-Taussig shunt was carried out using a Goretex prosthesis. Nine months postoperatively, at follow-up cardiac catheterization, the ill-directed shunt was diagnosed and subsequently revised. The onset of ipsilateral pulmonary edema and increasing signs of congestive heart failure after surgical creation of a Blalock-Taussig shunt as well as the persistence of lowered oxygen partial pressure, cyanosis and poor general health, should alert the attending physician to rule out the rare complication of anastomosis to the pulmonary vein.

The term anatomically corrected malposition of the great arteries describes a very rare form of congenital heart disease. In contrast to corrected transposition of the great arteries (atrioventricular and ventriculo-arterial discordance) there is an atrioventricular and ventriculoarterial concordance. The abnormally located arteries arise above the anatomically correct ventricles, the pulmonary trunk originates above the morphologically right ventricle, the aorta arises above the morphologically left ventricle. The abnormal relation (malposition) between the great arteries results from interposition of bilateral abnormal conal myocardium with absence of aortic valve-mitral valve fibrous continuity. In this case report an anatomically corrected laevomalposition of the great arteries associated with dextrocardia and situs solitus, ventricular septal defect and bilateral abnormal conal myocardium with subpulmonary obstruction in a young infant is presented.

The CT-findings of the most important kinds of malpositions of the heart are described. The differentiation between the venous and arterial heart cavities is possible noninvasively using a rapid bolus injection of contrast medium. If there is a malposition of the heart, uncertain findings of the heart configuration in the plain chest film can be explained by CT. Anomalies of the great vessels are detectable.