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.

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.

Accessory hepatic vein complicating extra-cardiac total cavopulmonary connection.

We encountered unexpected, severe hypoxia after the right heart bypass operation in a patient with isomerism. A 2-year-old girl with polysplenia had a complex cardiac anomaly consisting of a single atrium, single ventricle, pulmonary stenosis, absence of the right superior vena cava, hemiazygos continuation of the left inferior vena cava, and d-malposition of the great arteries. After a total cavopulmonary shunt, we performed an extra-cardiac total cavo-pulmonary connection with a 14 mm tube graft. The postoperative course was complicated by severe hypoxia. Angiography performed 20 days after the operation showed that contrast medium in the conduit poured into the hepatic vein, and through the intrahepatic communications, it passed into a left-sided accessory hepatic vein, which was connected directly to the left side of the aspect of the atrium. As the intrahepatic communication was adequate, we ligated the accessory hepatic vein within the pericardial cavity. The SpO2 returned to normal and no hepatic dysfunction was detected. We conclude that surgeons performing extra-cardiac total cavopulmonary connection need to pay closer attention to the possibility that an accessory hepatic vein might exist.

Indications for partial left ventriculectomy in pediatric dilated cardiomyopathy.

The purpose of this study was to define the role and indications of partial left ventriculectomy (PLV) in children with end-stage dilated cardiomyopathy (DCM). Clinical data were collected by retrospective chart review of children with DCM who were treated from 1997 to 2000. Four patients underwent PLV (PLV group) and 5 patients were managed without PLV (non-PLV group). In the PLV group, 2 patients are well 18 and 35 months postoperatively. One infant survived 6 months and then successfully underwent heart transplantation, and the other child died of hemoptysis 2 weeks postoperatively. Factors affecting outcome were preoperative status, in particular whether surgery was performed urgently or electively. In the non-PLV group, 4 patients were well controlled by medical treatment and 1 infant underwent mitral valve replacement for severe mitral regurgitation. The cardiothoracic ratio ranged from 72% to 76% in the PLV group and from 45% to 60% in the non-PLV group. The percentage of the expected left ventricular diastolic dimension ranged from 184% to 218% in the PLV group and from 109% to 163% in the non-PLV group. Ejection fractions in the PLV group were from 10% to 22% and from 36% to 56% in the non-PLV group. The serum brain natriuretic peptide concentration was above 1,200 pg/ml in the PLV group and below 168 pg/ml in the non-PLV group. In conclusion, PLV is indicated for selected children with end-stage DCM, and is most appropriate when medical therapy is not effective and heart transplantation is unavailable.