Platypnoea-orthodeoxia syndrome, an underdiagnosed cause of hypoxaemia: four cases and the possible underlying mechanisms.

Cardiac platypnoea-orthodeoxia syndrome (POS) is a position-dependent condition of dyspnoea and hypoxaemia due to right-to-left shunting. It often remains unrecognised in clinical practice, possibly because of its complex underlying pathophysiology. We present four consecutive patients with POS and patent foramen ovale (PFO) who underwent a successful percutaneous PFO closure, describe the mechanism of their POS and provide a review of the literature.

Unroofed coronary sinus newly diagnosed in adult patients after corrected congenital heart disease.

Patients with congenital heart disease corrected in early childhood may later in life present with cardiac symptoms caused by other associated congenital anomalies that were initially not diagnosed. Nowadays, several noninvasive imaging modalities are available for the visualisation of cardiac anatomy in great detail. We describe two patients with an unroofed coronary sinus, a rare congenital anomaly which could be diagnosed using a combination of modalities including echocardiography, cardiac CT and cardiac MRI.

Two cases of aorto-right atrial tunnel: clinical presentation, imaging and percutaneous closure.

Communication between the aortic sinus and a cardiac chamber is a rare anomaly that can be diagnosed in children and young adults. We describe two cases with a tunnel between the aortic sinus and right atrium, discuss diagnostic modalities, and review the literature on this anomaly.

Per-operative stent placement in the right pulmonary artery; a hybrid technique for the management of pulmonary artery branch stenosis at the time of pulmonary valve replacement in adult Fallot patients.

After having undergone surgical correction at an early age, many patients with tetralogy of Fallot develop long-term complications including progressive pulmonary regurgitation and peripheral pulmonary stenosis. A high percentage of these patients need to undergo a second operation in their adolescence or early adulthood. If simultaneous treatment of both pulmonary regurgitation and peripheral pulmonary stenosis is warranted, a complete surgical approach has several disadvantages. We describe four cases of Fallot patients with severe pulmonary regurgitation and peripheral pulmonary stenosis who were treated using a hybrid approach involving surgical implantation of a pulmonary homograft and peroperative stenting of the pulmonary artery.

Patent ductus arteriosus in adults - indications and possibilities for closure.

Patent ductus arteriosus (PDA) is a rare diagnosis in adults, since symptoms and signs usually occur in infancy and most cases are treated shortly after diagnosis. We present two patients who were first diagnosed with PDA during adulthood. The first case represents a more severe form of PDA, where the need for closure of the PDA is obvious. In the second case the sequelae of the PDA are less clear. In both patients, closure of the PDA (surgically in one case, percutaneously in the other) was successful.

[Results of extracorporeal membrane oxygenation following cardiac surgery in 16 children in the St Radboud University Medical Centre, 1996-2004]. / Resultaten van extracorporele membraanoxygenatie na een hartoperatie bij 16 kinderen in het Universitair Medisch Centrum St Radboud, 1996-2004.

OBJECTIVE: Description of the results with extracorporeal membrane oxygenation (ECMO) in infants with cardiorespiratory failure after open-heart surgery. DESIGN: Prospective observational study. METHOD: Data on the indications, survival, and complications were collected from all infants treated with ECMO for cardiorespiratory failure after open-heart surgery in the period 1996-2004 in the St Radboud University Medical Centre in Nijmegen, the Netherlands. RESULTS: 17 ECMO treatments were carried out in 16 paediatric patients: 7 girls and 9 boys. All patients had severe circulatory failure, sometimes combined with respiratory insufficiency. The patients' average age was 8 months (range: 1 day-3 years and 10 months). The average duration of treatment was 137 hours (range: 8-433). In 12 of the 17 ECMO treatments, the patient was successfully weaned from ECMO and 7 patients were discharged home. 5 patients died in hospital after successful ECMO treatment. 10 of the 16 patients died. A functional univentricular heart was associated with a high mortality. Haemorrhage, renal insufficiency and; sepsis were the most frequent complications. Major causes of death were sepsis, multi-organ failure and pulmonary hypertension.