Periarterial abscess: another cause of cyanosis after pulmonary artery banding.

Twenty seven months after pulmonary artery banding a boy aged two and a half developed rapidly progressive cyanosis. A periarterial abscess was found at the site of the band. Repair of the pulmonary artery and closure of the ventricular septal defect were complicated by profoundly low cardiac output and prolonged dependence on a ventilator. This near fatal complication would have been avoided by primary closure of the ventricular septal defect.     

Pulmonary blood flow distribution after banding of pulmonary artery.

Radioisotope lung scanning was used to investigate the distribution of pulmonary blood flow after banding of the pulmonary artery in children with a left-to-right shunt and pulmonary hypertension. An abnormal distribution of blood flow in the lung on the side of the operation approach was observed in all patients in the first three weeks following surgery. Abnormalities were still observed in 17 of 21 children 10 months to more than 8 years after the banding operation. There was no significant relation between the occurrence of these abnormalities and time after surgery. Diminished flow to the zones of the right lung was observed less frequently. The incidence of abnormalities in flow distribution was also high preoperatively. Respiratory complications in infants with large left-to-right shunts were considered to be responsible for most of the abnormal blood flow distributions observed. Radioactive lung scanning was found to be a valuable diagnostic method in the early and late postoperative period in infants and small children. It was more sensitive than the other techniques used in revealing deviation of blood flow from one lung in those cases with shifting of the applied band.     

Methaemoglobinaemia after cardiac catheterisation: a rare cause of cyanosis

Two young women had unexpected cyanosis a few hours after cardiac catheterisation for electrophysiological investigation. The first patient had atrioventricular septal defect, had undergone repeated surgical interventions, and was referred because of atrial flutter. The second patient had ablation of an accessory pathway in Wolff-Parkinson-White syndrome. Local anaesthesia was performed with 40 ml prilocaine 2%. Cyanosis with oxygen saturation of 85% developed in both patients a few hours after the electrophysiological investigation. The patients were transferred to the intensive care unit and for the first patient a considerable diagnostic effort was made to rule out morphological complication. Finally methaemoglobinaemia of 16.7% and 33.4%, respectively, was found. Cyanosis resolved within 24 hours and did not reappear. Underlying glucose-6-phosphate dehydrogenase deficiency and erythrocyte-methaemoglobin reductase deficiency were ruled out. Physicians should be aware of this rare side effect of local anaesthetics in patients with unexpected cyanosis.     

Congenital pulmonary arteriovenous fistula: a rare cause of cyanosis in childhood

Two children (both females) aged 15 months and 4 years are described as very rare cases of central cyanosis in childhood being caused by a congenital pulmonary arteriovenous fistula. The initial diagnosis was made based on cyanosis and chest radiographs, with normal physical, ECG, and radiological findings of the heart. They had no family history of the Rendu-Weber-Osler syndrome. The patients underwent cardiac catheterization and pulmonary angiography, where the diagnosis was confirmed. After the surgery, both were symptom-free, and had no evidence of the disease.     

Unilateral pulmonary artery banding to promote contralateral pulmonary artery growth

A full-term baby with double-outlet right ventricle and total anomalous pulmonary venous connection (TAPVC) complicated with left main pulmonary artery (PA) stenosis, presented with heart failure caused by increased pulmonary blood flow. Based on significant discrepancies in size and development between the left and right PAs, we performed right PA banding concomitant with TAPVC repair to promote left PA growth and restrict overall PA flow. PA-graphy performed 3?months after surgery showed marked increase in the left PA size with appropriately low pressure, which enabled us to successfully complete Glenn anastomosis. Under appropriate patient selection, unilateral PA banding for patients with unbalanced peripheral PA size could serve as an effective and less invasive strategy to simultaneously promote PA growth and control PA flow.     

Pulmonary artery sarcoma: another cause of sudden death

A patient hospitalized for recurrent syncope and dyspnea died suddenly before a definite clinical diagnosis could be established. At autopsy a large sarcomatous mass (undifferentiated type) was found to arise and totally occlude the pulmonary trunk without evidence of distant metastasis.     

Right ventricular hypertrophy and apoptosis after pulmonary artery banding: regulation of PKC isozymes

OBJECTIVE: Pressure overload induced by pulmonary artery banding (PAB) leads to right ventricular (RV) hypertrophy and cardiomyocyte apoptosis. The present study was performed to investigate whether protein kinase C isozymes (PKC-alpha, PKC-betaI, PKC-betaII, PKC-delta and PFC- epsilon ), calcineurin and the renin-angiotensin system (RAS) contribute to PAB-induced cardiac remodeling. METHODS AND RESULTS: PAB in male Wistar rats for 3 weeks results in enhanced PKC activity (as determined by ELISA assay) in the cytosol and membrane fraction of the hypertrophied RV, which was accompanied by increased expression (as determined by Western blot analysis) of cytosolic PKC-delta (+72%), PKC-alpha (+49%), and PKC-betaI (+39%), but not PKC-betaII and PKC- epsilon. This differential regulation of cardiac PKC isozymes was limited to the strained ventricle and was not altered in response to chronic angiotensin-converting enzyme inhibition with ramiprilate. Furthermore, no significant changes in the expression of calcineurin alpha and beta subunits were observed in RV pressure overload compared to controls. PAB-induced cardiac apoptosis was determined using Western blot analysis by a significantly increased expression of Bax protein and caspase-3 in the hypertrophied RV, which was diminished to almost control levels by chronic ramiprilate treatment. The myocardial expression of Bcl-2 was not significantly altered in the experimental groups. CONCLUSION: We have shown for the first time that PAB-induced RV hypertrophy is associated with a differential regulation of cardiac PKC isozymes independent of the RAS and further provide evidence for a pivotal role of the RAS in the development of PAB-induced cardiac apoptosis.     

Minimally invasive pulmonary artery banding: a new approach

BACKGROUND: A variety of approaches have been described for banding of the pulmonary artery. The indications for this procedure are limited; however in developing countries, many patients still need pulmonary artery banding for a variety of reasons. We describe a new approach, minimally invasive, using only a split in the manubrium sterni to conduct the procedure. METHODS AND RESULTS: Between January 2000 and May 2002, 19 patients who had undergone pulmonary artery banding using a minimally invasive technique were compared with 20 cases of pulmonary artery banding performed by the conventional technique. The mortality was similar in the two groups (p=0.45). The period of intubation and duration of intensive care unit stay were significantly shorter in the minimally invasive group (p=0.015 and 0.002, respectively). The duration of hospital stay was not significantly different between the 2 groups (p=0.139). In the minimally invasive group, three patients underwent subsequent reoperation. CONCLUSIONS: Minimally invasive pulmonary artery banding is useful in babies with high-flow cardiac lesions and cardiac cachexia.     

Right pulmonary artery-to-left atrium communication: a rare cause of systemic cyanosis.

Direct communication between the right pulmonary artery and the left atrium is a rare congenital vascular malformation. The clinical diagnosis is difficult, and preoperative angiography is essential. We treated this anomaly successfully with surgery and the use of cardiopulmonary bypass in an 11-year-old boy. Surgery provides a complete cure for this anomaly.