Pulmonary artery intimal sarcoma is a rare disorder arising from the intima of the pulmonary artery. Histopathology reveals that it is a tumour cell of mesenchymal origin. The signs and symptoms include chronic shortness of breath and other features of right ventricular failure, which mimic chronic pulmonary thromboembolism. The definitive diagnosis can rarely be made based on the symptoms and signs alone, and other investigations including echocardiography, computed tomography, magnetic resonance imaging (MRI), and positron emission tomography (PET) are often required. The gold standard for diagnosis is tissue biopsy. The mainstay for treatment is surgery, and complete surgical resection with endarterectomy provides survival benefit. According to recent evidences, however, multimodal treatment provides better survival outcomes than monotherapy such as surgery alone. Despite the newer upcoming treatment strategies, patients with pulmonary intimal sarcoma continue to have a poor prognosis. We present a case of pulmonary artery intimal sarcoma and review the literature associated with the disease.

IntroductionFunctional tricuspid regurgitation (TR) is transient and benign in stressed infants. In contrast, severe TR caused by a flail leaflet is a rare cause of neonatal cyanosis. The outcome may be fatal if not recognized early (1,2). We report on a full-term female neonate presented with cyanosis caused by severe TR due to anterior leaflet chordal rupture. After initial stabilization by prostaglandin E1 infusion for keeping ductal patency, successful early repair was achieved with polytetrafluoroethylene artificial chordae implantation. Early diagnosis and timely surgery can be lifesaving. Case presentationA 3,044-gm full term female was born by Cesarean section because of fetal distress and meconium stain. The first and fifth minutes Apgar score was 5 and 9. On physical examination, a grade III/VI pansystolic murmur was noted diffusely. She was cyanotic and the oxygen saturation was 85%. A two-dimensional echocardiography showed severe TR due to a flail and thickened anterior leaflet (Figure 1A,B) with the calculated tricuspid Z score of 1.28, and large patent ductus arteriosus. The antegrade pulmonary flow was compromised and a right-to-left shunt through the patent foramen ovale was noted. The hemodynamic characteristics were quite similar to that of the pulmonary atresia with intact ventricular septum.At 6-day-old, cyanosis got worse due to impending ductal closure. She was intubated and prostaglandin E1 was infused (0.2 µg/kg/min), then the condition was stabilized and surgery was carried. Under mild hypothermia with cold crystalloid cardioplegic cardiac arrest, the patent ductus was ligated and the right atrium was opened. A flail tricuspid anterior leaflet due to extensive marginal chordae rupture was confirmed. The papillary muscle showed no ischemic change. The length of the septal leaflet chordae was measured as the reference and two loops of 6-0 expanded polytetrafluoroethylene artificial chordae were implanted over the unsupported margin of the anterior leaflet. A tricuspid annuloplasty was not necessary because the annulus was not dilated. The patent foramen ovale was not closed for pop-off purpose. Nitric oxide was not needed and under minimal inotrope support, the patient was recovered uneventfully. She was extubated 7 days after the operation Abstract: Neonatal rupture of the chordae of tricuspid valve with severe regurgitation is rare and disastrous. We report on a full-term female neonate presented with cyanosis caused by severe tricuspid regurgitation (TR) due to anterior leaflet chordal rupture. After initial stabilization by prostaglandin E1 infusion, successful early repair was achieved with artificial chordae implantation. The unique pathophysiology and the therapeutic strategy of this situation will be described.

A temporal correlation (TC) mapping method is proposed to help bolus chasing during dynamic contrast-enhanced (DCE) MRI of complex pulmonary circulation (CPC) in patients with congenital heart disease. DCE-MRI was performed on five healthy male subjects (23-24 years old) and 25 patients (nine males and 16 females, 0.25-44 years old), and TC maps were generated by performing pixel-based computation of crosscorrelations to the pulmonary artery with a series of time shifts in all subjects. Qualitative and quantitative evaluations were performed in comparison with original DCE images. TC maps exhibited a better signal-to-noise ratio (SNR) by factors of 4.3 and 1.3 in the lung parenchyma, pulmonary veins, and superior artery/vein; a better intraparenchymal contrast-to-noise ratio (CNR) by factors of 1.5-5.4; and a significantly higher conspicuity in all regions except the pulmonary arteries when graded with a five-point score. TC maps evaluated by two experienced clinicians significantly added relevant information (P < 0.001), and in some cases affected the final diagnosis. We conclude that TC maps facilitate bolus chasing for DCE-MRI by reducing recirculation effects and interframe fluctuations, and hence complements morphological imaging of CPC in patients with complex congenital heart disease. Magn Reson Med 56: 517-526, 2006.

The clinical results of EC-TCPC in patients with functional single ventricle were satisfactory. The patients who needed fenestration during operation had higher risk of surgical mortality. Significant atrioventricular valve regurgitation had negative impact on intermediate survival.

Based on clinical and surgical confirmation of the MSCT scan results from a multidisciplinary congenital heart disease specialist team, we concluded that adequate information on CHD, specifically that regarding extracardiac abnormalities of the anatomy, can be obtained and MSCT can be used to replace cardiac catheterization.