Recently, modified Fontan operation is being used for asplenia syndrome. We reviewed 24 patients with asplenia syndrome who underwent surgical intervention. Eleven (45.8%) of them had total anomalous pulmonary venous connection (TAPVC) and 6 of these were accompanied by pulmonary venous obstruction (PVO). Surgical results were poor in the PVO group. In 4 cases with open heart palliation, that is atrium-common pulmonary venous chamber anastomosis, there were 2 operative deaths, 1 late death, and 1 survival. In 18 cases without PVO, statistical analysis (Fisher's exact probability) showed that pulmonary atresia (PA) was a definite risk factor for Fontan candidates (p<0.05). In 9 cases with pulmonary stenosis (PS group), there were 5 candidates for the Fontan type operation. In the other 9 cases with PA (PA group) there were no candidates for the Fontan type operation. Only the size and the morphology of the pulmonary artery were significant factors (p<0.05) for candidates of the Fontan type operation among the risk factors such as size and morphology of the pulmonary artery, pulmonary vascular resistance and pressure, atrioventricular valve regurgitation, and single ventricular function. Finally, in these 24 cases, there were only 5 candidates (20.8%) for a Fontan type operation. In conclusion, in order to increase candidates for Fontan precedures, it is important to maintain an adequate pulmonary blood flow. Earlier PDA division and pulmonary arteries plasty are the most importantin PA group. In both groups pulsatile bidirectional cavopulmonary shunts may be useful to increase effective pulmonary blood flow without ventricular volume overload, which leads to atrioventricular valve regurgitation.

Seventeen cases of palliative right ventricular outflow tract reconstruction were reviewed to determine the optimal outflow diameter in this procedure. The clinical diagnoses of these 17 patients were pulmonary atresia with ventricular septal defect in 8, tetralogy of Fallot (TOF) in 7, TOF with complete atrioventricular canal in 1, double outlet right ventricle with pulmonary stenosis in 1. Techniques of reconstruction were transannular patch in 7 (valved 2, non-valved 5), extracardiac conduit in 5 (valved 2, nonvalved 3), outflow patch in right ventricle in 3, and others in 2. Pulmonary artery growth was not related to the diameter of reconstruction of outflow and postoperative Pp/Ps. The pressure in pulmonary artery tends to be hypertensive when the outflow diameter was more than 90% of the normal value. On the other hand, the minimal outflow diameter in nine cases of primary repaired TOF was in the range of 0.41 to 0.68 (mean of 0.59). In conclusion, the diameter of outflow reconstruction should be 60 to 80% of the normal pulmonary valve.

Objective: The aim of this study was to investigate the palliative care for cancer patients provided by a long-term care unit. Methods: We retrospectively investigated the medical records of 194 patients who hospitalized in our long-term care unit between April 2010 and December 2014. The patients shared with a group of two, narcotic drugs non-use period (previous group; until March 2012) and narcotics use period (later group; in April 2012 or later), and it was compared with a background, hospitalization period, result on discharge, narcotic drug administration, symptom relief. And it was surveyed hospitalization dynamics of our palliative care unit for comparison. Result: Cancer patients were 16 (22%) in previous group of 74 patients and were 79 (66%) in later group of 120. The proportion of cancer patients in later group increased to three times (p<0.001). In later group, the average hospital stay was shortened to half (144 days, p<0.01) and the mortality discharge rates increased (78%, p<0.05). Narcotic drugs were administered to more than half (57%) of cancer patients in later group, and pain relief was significantly better. In the period of later group, number of hospitalized patients in palliative care unit was also increased. Conclusion: It was suggested that the long-term unit can perform palliative care for cancer patients in cooperation with the palliative care unit.

Four patients with d-TGA after atrial switch operation were treated with transvenous DDD pacemaker implantation for their postoperative dysrhythmia; complete atrioventricular block (CAVB) in two, sinus bradycardia with grade I atrioventricular block in one after the Mustard procedure and CAVB with sick sinus syndrome after the Senning procedure in one. From an anatomical point of view, the left atrial appendage was the only suitable anchoring site for the atrial lead. The patency of this cavity should be assessed by echocardiography and/or angiography before implantation. As for the ventricular lead, active fixation is recommended because of the relatively smooth endocardial surface of morphological left ventricle. Otherwise, transvenous DDD pacemaker implantation for patients after atrial switch operation was safely performed without any technical difficulties and with few complications.

This article describes the end of life of seven people with amyotrophic lateral sclerosis (ALS) under the care of a hospice. The reasons for admission to hospice were for the management of distressing symptoms and the support of families who were unable to continue care at home because of the increased burden of care and/or illness of families. The sufferings experienced by the patients with ALS included disability due to muscle weakness(100%), pain(100%), discomfort (100%), dyspnea (71%), difficulties in communication (71%), drooling (43%), insomnia (43%), loneliness (43%), swallowing difficulties (28%), clenching the mucosa inside the cheek (28%), anxiety (28%), the perception of being a burden to the family (28%), and concerns as to why they had developed ALS (28%). Opioid medication was effective in the management of dyspnea, pain and discomfort. The results showed that special attention should be paid to frequent changing of the patient's position during nursing care, including the passive movement of joints and massage. The use of communication aids was essential to allow people with ALS to communicate effectively and, together with support of joy of the patients and family, the quality of life could be improved. End of life care within a hospice is a useful alternative option for persons with ALS, extending hospice care in Japan from people with advanced cancer to other disease groups. Palliat Care Res 2010; 5(2): 137-143