Tetsushi Tan

Endothelial function evaluated by flow-mediated dilatation in pediatric vascular disease

The endothelial function of children with and without vascular disease, consisting of 41 controls, 24 with Kawasaki disease (KD), and 46 with diabetes mellitus (DM), was examined. Age at examination ranged from 3 to 23 years (mean, 12.0 ± 4.7). The flow-mediated dilatation (FMD) and intima-media complex in the common carotid artery were measured. In controls age at examination was not associated with FMD or intima-media complex. FMD significantly decreased in children with KD and DM compared with the control group (control vs KD or DM: 11.7 ± 14.7 vs 3.0 ± 11.0 or 6.4 ± 8.5%, respectively; p < 0.05). However, there was no significant difference for intima-media complex among the groups. Furthermore, FMD in KD patients with coronary arterial aneurysm was lower than that in KD patients without aneurysm (-0.5 ± 9.2 vs 8.3 ± 9.1%, p < 0.05). In DM patients, FMD in the high HbA1c group (HbA1c = 7%) was lower than that in the normal HbA1c group (HbA1c < 7%) (4.8 ± 8.1 vs 11.4 ± 7.8%, p < 0.05). In conclusion, FMD detected endothelial impairment in children with KD or type 1 DM regardless of overt vascular complications, and FMD impairment occurs prior to intima-media complex thickening. By measuring both FMD and intima-media complex, useful information for predicting vascular complications may be obtained.

Gadolinium-based balloon angioplasty for pulmonary artery stenosis in an infant with a right isomerism

We report here the first described case of utilizing gadolinium‐based contrast material as the contrast agent during a catheter intervention treatment for pulmonary artery stenosis. The patient, a male infant with complex heart disease associated with a right isomerism, had a history of severe allergic reaction to iodine‐containing contrast agents. A combination of digital subtraction angiography and a gadolinium contrast agent, however, provided us with good‐quality images both before and after balloon angioplasty without any associated complications. This method should therefore be considered as an alternative angiographic procedure in children with a high risk of iodine‐related allergic complications. Catheter Cardiovasc Interv 2004;63:346–350.

Mid-term outcome after partial left ventriculectomy in pediatric patients.

From May 1998 to April 2000, we performed partial left ventriculectomy (PLV) in 3 pediatric patients with dilated cardiomyopathy (DCM). At the time of the surgery, their age ranged from eight months to three years. The first patient eventually had to receive a heart transplant, but all patients treated with PLV are alive to this day. Patient #1 was diagnosed with DCM at the age of five months, PLV was done on a semi-urgent basis at the age of eight months, when medium dose IV catecholamine therapy and mechanical ventilation were required. Fraction shortening (FS) as shown by echocardiography increased postoperatively from 8% to 15% along with marked clinical improvement. Her heart failure deteriorated three months after the surgery, and received a heart transplant in the United States when she was one year and two months old. Patient #2 developed severe heart failure two months after correction of a ventricular septal defect. Aggressive medical therapy failed to improve his condition, therefore PLV was done on an elective basis at the age of three years and five months. [The patient was initially hospitalized and underwent low dose catecholamine.] Postoperative course was well. The ventriculography one year after surgery showed an improvement of the left ventricular FS from 12% to 27% after PLV. He was still doing well at his most recent check up. Patient #3 was diagnosed with DCM as a neonate. PLV was done on an elective basis at the age of two years and five months. Her postoperative course was generally well. FS on echocardiography increased postoperatively from 10% to 25% along with marked clinical improvement. The timing of performing PLV is the most essential factor for postoperative course in our experiences. We consider that the best timing is when aggressive catecholamine infusion or mechanical ventilation is required. The mid-term outcome of PLV of pediatric patients is considered to be acceptable. We believe that PLV should be considered as a viable option for severe DCM patients.

Partial left ventriculectomy in pediatric end-stage dilated cardiomyopathy

OBJECTIVE Although a recent survey on pediatric cardiomyopathy in Japan showed that 48% of patients died despite the medical treatment, pediatric cardiac transplantation is not legal in Japan. We determined the feasibility of partial left ventriculectomy as an alternative to end-stage dilated cardiomyopathy. METHOD We retrospective analyzed partial left ventriculectomy in 4 pediatric patients with end-stage dilated cardiomyopathy. RESULTS In case 1, an 8-month-old girl underwent semiemergency partial left ventriculectomy. Her ejection fraction increased from 10% to 25%, and her condition improved initially, but she developed heart failure and underwent cardiac transplantation 6 months later in the US. In case 2, a 3-year-old boy developed severe heart failure 2 months after ventricular septal defect repair. Intensive medical therapy failed, so partial left ventriculectomy was done, which increased his ejection fraction from 15% to 35%. His condition is stable 35 months after surgery. In case 3, a 2-year-old girl with a chromosomal anomaly undergoing ventricular septal defect repair developed progressive heart failure 1 year later. Despite emergency partial left ventriculectomy, she died of hemoptysis 2 weeks postoperatively. In case 4, a 2-year-old girl developing progressive heart failure unresponsive to medical therapy after 10 months underwent elective partial left ventriculectomy and remains in stable condition 18 months postoperatively. CONCLUSION Partial left ventriculectomy is appropriate for selected patients with end-stage dilated cardiomyopathy if medical therapy is not effective and heart transplantation is not possible.