Motoki Takamuro

Influence of ventricular morphology on aerobic exercise capacity in patients after the Fontan operation

OBJECTIVESnThis study investigated the influences of ventricular morphology, hemodynamics and clinical findings on exercise capacity in patients after the Fontan operation.nnnBACKGROUNDnDeterminants of exercise capacity after the Fontan operation remain unclear.nnnMETHODSnPeak oxygen uptake (PVo2) was determined in 105 patients by exercise test and compared to hemodynamics and clinical findings. Patients were divided into three groups based on ventricular morphology: those with a right ventricle (group RV), a biventricle (group BV) and a left ventricle (group LV).nnnRESULTSnTen patients with atrioventricular valve regurgitation (AVVR) or hypoxia exhibited a low PVo2. After excluding these patients, although PVo2 did not correlate with hemodynamics, except ventricular ejection fraction (p < 0.02), it correlated with age at the Fontan operation and exercise test (p < 0.002). The PVo2 was higher in group LV (63+/-9%) than in groups RV (55+/-9%) and BV (55+/-12%) (p < 0.01), while an inverse correlation between PVo2 and age at operation was demonstrated only in group RV (p < 0.05). Groups RV or BV and age at exercise test were associated with a lower PVo2, whereas group LV was an independent predictor of a higher PVo2 (p < 0.01). During 4.2 years of follow-up, a decrease in peak heart rate was related to a decrease in PVo2 (p < 0.05). The PVo2 decreased in group RV (p < 0.01).nnnCONCLUSIONSnIn addition to AVVR, hypoxia, and heart rate response, ventricular morphology is related to exercise capacity. Early Fontan operation may be beneficial in terms of exercise capacity, especially in the group RV patients.

Development of a reexpandable covered stent for children.

The use of covered stents in children is limited by the need for a large long sheath for delivery and the impossibility of redilation once implanted. The authors developed a reexpandable covered stent implantable in children through a small sheath and evaluated its clinical feasibility in mini piglets. An original Palmaz stent was covered with a polyurethane membrane that could be stretched up to 700%. Under general anesthesia, the authors implanted the covered stents in six mini piglets using a long sheath with a diameter approximately 1 French larger than the recommended size required to deliver an uncovered Palmaz stent. The implantation technique was similar to conventional stent implantation. In six piglets, the stent could be redilated from 7.5 to 8.7 mm 28–70 days after implantation. Macroscopic and microscopic examination showed intimal coverage of the coating with minimal inflammatory reaction around the stent. Our newly designed reexpandable stent covered with a polyurethane membrane is promising for use in children.

The Throat Flora and Its Mitogenic Activity in Patients with Kawasaki Disease

The etiology of Kawasaki disease (KD) remains unknown, although some infectious organism has been suggested as the cause. Recent studies suggest that some bacterial toxins with superantigen activity are involved in its pathogenesis, but no specific bacterial toxin has yet been identified. Throat swabs for bacterial culture were obtained from 21 patients with KD and 20 with other febrile illnesses as controls. Mitogenic activity in culture supernatants obtained from individual bacterial strains was measured by lymphocyte proliferation assay. Sixty‐one bacterial strains were isolated from KD patients, and 62 strains from control patients. There was no apparent difference in bacterial species in the throat flora between KD patients and febrile controls. Moreover, total and individual mitogenic activity of strains from KD patients was no greater than that of strains from febrile controls. The bacterial superantigen activity of throat flora may not play a major role in the pathogenesis of KD.

Double outlet right ventricle with intact ventricular septum

We report a rare case of DORV with an intact ventricular septum (intact VS). The patient was a 2-day-old boy, born at 39 weeks and weighing 3090 g, who was referred to the Department of Pediatrics, Sapporo Medical University Hospital, Sapporo, Japan, because of a heart murmur. He had cyanosis and a systolic murmur at the lower intercostal spaces. Chest X-ray showed cardiomegaly (cardiothoracic ratio, 67%) and the electrocardiogram revealed normal sinus rhythm, right axis deviation and right ventricular hyper trophy. Echo cardiography demonstrated situs solitus, normal relationship of great arteries, side by side, bilateral conus, intact VS, persistent foramen ovale, pulmonary stenosis and severe mitral valve insuffi ciency ( Fig. 1a,b). We performed balloon atrial septostomy when he was 1 month old, and he underwent a left modifi ed Blalock – Taussig shunt at 5 months. Cardiac catheterization was performed when he was 18 months old. His left ventricular pressure was extremely low, 10/4 mmHg, the diastolic volume was 11 mL, 48% of normal, and the ejection fraction was 76%. Left ventricular angiography showed a smooth and thin wall of the left ventricle, resembling Uhl’s disease. Both right and left coronary artery were normally demonstrated ( Fig. 2). Right ventricular pressure was 92/11 mmHg with a pulmonary artery Patient Report

Increased serum high‐sensitivity C‐reactive protein is related to hypoxia and brain natriuretic peptide in congenital heart disease

Background: Studies of adults have shown a direct association between increased serum concentrations of high‐sensitivity C‐reactive protein (hs‐CRP) and atherosclerotic cardiovascular disease, diabetes, and chronic heart failure. Some studies have documented elevated hs‐CRP in obese children and adolescents, and in patients with a history of Kawasaki disease, but there are few data on its clinical significance in congenital heart disease.

Relapse of T-cell all after stem cell transplant presenting as hypertrophic cardiomyopathy: the value of non-invasive diagnostic imaging in detecting cardiac leukemia.

We describe a 14‐year‐old female with acute lymphoblastic leukemia (ALL) with a mediastinal mass at diagnosis who developed hypertrophic cardiomyopathy (HC) after stem cell transplantation (SCT). During refractory relapse after SCT using bone marrow from her HLA‐matched sibling, she underwent whole thorax irradiation because of pleural effusion and a recurrent mediastinal mass. After a second SCT using peripheral blood from the same donor, she developed HC suspected to be related to tacrolimus. However, isolated cardiac relapse was finally diagnosed by several non‐invasive imaging techniques. Cardiac irradiation resolved her cardiac failure, though she eventually developed progressive and fatal hematological disease.

Clinical features of adult patients with Eisenmenger's syndrome in Japan and Korea

BACKGROUNDnThere are few articles on mortality and morbidity of adult patients with Eisenmengers syndrome (ES) in the current era when disease targeting therapy (DTT) has been available.nnnMETHODS AND RESULTSn198 patients (a median age 35 years, 64% female) with ES who visited the 16 participating institutes in Japan and Korea from 1998 to 2009 were enrolled. Clinical data during adulthood were collected from each institutional chart and analyzed centrally. During a median follow-up of 8 years, 30 patients died including 14 sudden deaths. 89 patients took oral medication of DTT and clinical improvement was observed in 54 of them. However, survival rate in patients taking DTT was not different from those without (87% vs 84%, p=0.55). When the clinical data in between first and last clinic visits were compared in 85 patients, the patients with NYHA >/=III increased from 24% to 48% (p<0.001), SpO2 decreased from 89% to 85% (p=0.008) and hematocrit increased from 51.4% to 52.9% (p=0.04). Non-survivors had poorer NYHA function class, lower body weight (BW), lower body mass index (BMI), and higher serum level of Cr at the first visits than survivors.nnnCONCLUSIONSnLong term survival and clinical status of adult patients with ES remains unsatisfactory even in the current era of DTT. Poor NYHA functional class, low BW, low BMI and high serum level of Cr were related to mortality. DTT therapy improved clinical status in many patients with Eisenmengers syndrome, but no significant impact on survival could be shown.

Late distortion of the original Palmaz stent implanted in postoperative lesions associated with congenital heart disease

The objective of this study was to report late distortion of a Palmaz stent. Late distortion of an original Palmaz stent, implanted in an extracardiac lesion, is rare. We completed a 1‐year follow‐up of 54 patients who had been implanted with 80 Palmaz stents in extracardiac lesions. Distortion of two stents was detected in two patients. For case 1, we implanted a P188 stent for supravalvar pulmonary stenosis complicating an arterial switch operation in a 14‐year‐old girl. Seven months later, we found compression of the stent. Although we implanted two P308 stents anterior to the distorted stent, distortion of both stents developed after 1 month. Two more P308 stents placed inside each stent were gradually recompressed. A CAT scan showed compression of the stent by a dilated sinus of valsalva. For case 2, we implanted a P308 stent for stenosis of the superior vena cava after Williams operation in an 11‐year‐old boy. A chest X‐ray documented longitudinal compression of the stent 27 months after implantation and a CAT scan showed the ascending aorta was in contact with the stent. A Palmaz stent may be distorted when implanted in a lesion adjacent to a pulsating aorta.

Improvement of protein-losing enteropathy by coil embolization of the left azygos vein

We report a 17‐year‐old boy with double‐outlet right ventricle, atrioventricular septal defect, pulmonary stenosis, and persistent left superior vena cava who developed a protein‐losing enteropathy, which was cured by coil embolization of the left azygos vein, thereby interrupting the vein‐to‐vein shunt. Cathet Cardiovasc Intervent 2003;59:399–401.