Congenital quadricuspid aortic valve with tetralogy of Fallot and pulmonary atresia

We report the case of a 4-year-old girl who had quadricuspid aortic valve regurgitation with tetralogy of Fallot and pulmonary atresia. This combination is very uncommon. Aortic valve replacement was performed successfully due to aortic regurgitation which had progressed one year after the total repair. The dilated aortic annulus plus quadricuspid aortic valve may result in progressive aortic regurgitation for a short period.     

Measurement of intrahepatic shunt using Rutland method

Intrahepatic shunt ratio was calculated from the fraction of the uptake constant and the blood background, analyzing the data of 99mTc-(Sn)-N-pyridoxyl-5-methyltryptophan (99mTc-PMT) hepatobiliary imaging by Rutland method. Shunt flow and total hepatic blood flow (THBF) were also calculated from the values for the effective hepatic blood flow (EHBF) estimated from the blood clearance technique. In 15 cases of normal volunteer, the average of the shunt ratio, shunt flow, and THBF were 18.4 +/- 5.4 (S.D.)%, 137.8 +/- 49 ml/min, and 753 +/- 83.2 ml/min, respectively. In 8 cases of chronic hepatitis, the values were 35.2 +/- 2.6%, 276 +/- 55.4 ml/min, and 794.1 +/- 119.4 ml/min, respectively. In 12 cases of liver cirrhosis, the values were 51.4 +/- 12.6%, 353.9 +/- 141.3 ml/min, and 685.6 +/- 174.8 ml/min, respectively. In cases of chronic hepatitis and liver cirrhosis, in comparison with normal cases, the values for the shunt ratio and shunt flow were significantly increased (p less than 0.001) and those for EHBF were significantly decreased (p less than 0.001), but those for THBF were not significantly altered. There was a significant inverse correlation between the shunt ratio and EHBF (r = -0.842) in all 35 cases. This method is considered to be useful for differential diagnosis, estimation of the grade of the diseases, and evaluation of pathophysiology of various diffuse liver diseases.     

Myocardial 11C-diacylglycerol accumulation and left ventricular remodeling in patients after myocardial infarction.

Left ventricular (LV) remodeling after myocardial infarction (MI) is a maladaptive process that increases the risk of heart failure and death. The myocardial phosphoinositide cycle, which is located downstream from several neurohumoral factors, plays a crucial role in LV remodeling. Our animal studies demonstrated that 1-[1-11C]butyryl-2-palmitoyl-rac-glycerol (11C-DAG) can be used to visualize regions with an activated phosphoinositide cycle. Therefore, we examined whether myocardial 11C-DAG accumulation assessed by PET is relevant to LV enlargement and systolic dysfunction in post-MI patients. METHODS: We performed PET with 11C-DAG in 13 post-anteroseptal MI patients and 4 healthy volunteers. We placed regions of interest on the noninfarcted myocardium and calculated the myocardium-to-left atrial (LA) chamber ratio of 11C-DAG accumulation. RESULTS: The myocardium-to-LA chamber ratio of 11C-DAG was significantly higher in the post-MI patients (mean +/- SD, 1.73 +/- 0.35) compared with that of the healthy volunteers (mean +/- SD, 1.25 +/- 0.13; P < 0.05). In the post-MI patients, the myocardium-to-LA chamber ratio of (11)C-DAG was significantly correlated with the LV end-diastolic volume index (r = 0.79, P < 0.01) and the plasma concentration of brain natriuretic peptide (r = 0.85, P < 0.001) and negatively correlated with the LV ejection fraction (r = -0.69, P < 0.01). CONCLUSION: These findings suggest that the myocardial 11C-DAG accumulation assessed by PET is relevant to LV enlargement, LV systolic dysfunction, and humoral activation in post-MI patients. This new imaging strategy based on intracellular signaling may contribute to the assessment and treatment of post-MI patients.     

Ruptured pediatric posterior cerebral artery aneurysm 9 years after the onset of Kawasaki disease: a case report

CASE REPORT: A 12-year-old boy who had a history of Kawasaki disease 9 years ago experienced a subarachnoid hemorrhage by ruptured right posterior cerebral artery aneurysm. On day 1 operation, as the aneurysm was very fragile and bled easily, two intraoperative ruptures, including a very premature rupture, were encountered. As a result, a left hemiparesis especially severe in the left hand was caused by the right anterior thalamic infarction due to the occlusion of a thalamo-perforating artery arising near the neck of the aneurysm. DISCUSSION: The histopathological examination of the intraoperative excised aneurysmal dome disclosed the thickening of the endothelial inner due to the endothelial hypertrophy and the invasion of inflammatory cells. This finding of the aneurysm was partially mimicking the finding of the coronary artery of the patients with Kawasaki disease. The combination of cerebral aneurysm and Kawasaki disease has never been reported until now, and the etiology of the aneurysm of this patient is unclear.     

Late results with biograft in peripheral arterial surgery

A total of 106 vascular reconstructions below the inguinal ligament including axillo-femoral and femoro-femoral bypasses were performed using 137 Dardik's human umbilical veins. The indication for surgery was limb salvage in 29%. The distal anastomosis was done with the popliteal artery above the knee in 53 cases, below the knee in 31, and with a tibial artery in 1. The axillo-femoral bypass was performed in 21 cases, and femoro-femoral bypass in 32. The accumulated graft patency rates of femoro-popliteal bypass at 1 yr./3 yrs./5 yrs. were 93%/75%/75%, those of femoro-femoral bypass were 85%/85%/85%, and those of axillo-femoral bypass were 54%/27%/27%. No special risk factor influencing patency rate was found from this study. In long term period, graft aneurysm was observed in 3 cases. It is concluded that the human umbilical vein is the graft material of choice for femoro-popliteal or femoro-femoral bypass when the saphenous vein is not available, and the careful follow-up is important because of the risk of graft aneurysm.     

Cone dysfunction and supernormal scotopic electroretinogram with a high-intensity stimulus

An unusual form of scotopic electroretinogram with a bright white stimulus, which consisted of a rectangular a-wave of normal amplitude and a b-wave of supernormal amplitude, was recorded in three patients with cone dysfunction. In addition to poor visual acuity, abnormal color vision and reduced amplitude of the photopic electroretinogram, these patients showed a 2-log unit elevation of the dark-adaptation threshold. Funduscopic examination and fluorescein angiography revealed fine granular pigment disturbances at the mascula. The relationship between the response of the dark-adapted electroretinogram versus stimulus intensity was unique to these patients. The b-wave thresholds were elevated by 1 log unit. The b-waves were reduced in amplitude and markedly delayed in implicit time to dim stimuli, but supernormal in amplitude and normal in implicit time to bright stimuli.Abbreviations GMP guanosine monophosphate