Jotaro Kobayashi

Intensive cardiac management in patients with trisomy 13 or trisomy 18.

Intensive cardiac management such as pharmacological intervention for ductal patency (indomethacin and/or mefenamic acid for closure and prostaglandin E1 for maintenance) and palliative or corrective surgery is a standard treatment for congenital heart defects. However, whether it would be a treatment option for children with trisomy 13 or trisomy 18 syndrome is controversial because the efficacy on survival in patients with these trisomies has not been evaluated. We retrospectively reviewed 31 consecutive neonates with trisomy 13 or trisomy 18 admitted to our neonatal ward within 6 hr of birth between 2000 and 2005. The institutional management policies differed during three distinct periods. In the first period, both pharmacological ductal intervention and cardiac surgery were withheld. In the second, pharmacological ductal intervention was offered as an option, but cardiac surgery was withheld. Both strategies were available during the third period. The median survival times of 13, 9, and 9 neonates from the first, second, and third periods were 7, 24, and 243 days, respectively. Univariate and multivariate analyses confirmed that the patients in the third period survived significantly longer than the others. Intensive cardiac management consisting of pharmacological intervention for ductal patency and cardiac surgery was demonstrated to improve survival in patients with trisomy 13 or trisomy 18 in this series. Therefore, we suggest that this approach is a treatment option for cardiac lesions associated with these trisomies. These data are helpful for clinicians and families to consider in the optimal treatment of patients with these trisomies.

Radical surgery for a ventricular septal defect associated with trisomy 18

PurposeCongestive heart failure is one of the major causes of early death of patients with trisomy 18. Ventricular septal defect (VSD) is the most common heart defect in patients with trisomy 18, and closure of the VSD may elongate the lifespan of the patient. Morphological characteristics of these patients, such as thoracic deformity, prominent right ventricular hypertrophy, and dysplastic tricuspid valve may complicate closure of the VSD. We report our initial experience of VSD closure in patients with trisomy 18 and estimate the feasibility of the surgical procedure.MethodsBetween June 2005 and September 2007, five female patients with trisomy 18 and VSD underwent radical operations. Four of them had undergone previous palliative surgery entailing pulmonary artery banding.ResultsAll patients survived surgery. The average cardiac arrest time during surgery was 74 ± 22 min. No early death occurred within 30 days of surgery. One patient with hydrocephalus treated by a previous ventriculoperitoneal shunt died in hospital owing to postoperative intracranial hypertension. Four of five patients were discharged from the hospital. Of the four discharged patients, a girl who underwent primary radical operation died of pneumonia. Another patient died of sudden cardiopulmonary arrest. The average survival of the patients in this study was 815 ± 389 days at data acquisition, with two patients still alive.ConclusionClosure of VSDs in patients with trisomy 18 was found feasible and was associated with extended survival.

Extracardiac cavopulmonary connection of fontan procedure with autologous pedicled pericardium without cardiopulmonary bypass.

cision. This tremor elimination and computer motion scaling overcome perhaps the most significant limitation of conventional endoscopic instruments. Although further chronic studies are necessary to fully validate the clinical utility of this robotic instrumentation, the current study provides encouraging preliminary results. Robotic assistance in the microsurgical environment may allow for the development of completely endoscopic CABG.

Pulmonary Atresia, Ventricular Septal Defect, and Coronary- Pulmonary Artery Fistula

We report 2 patients with pulmonary atresia, ventricular septal defect, and coronary-pulmonary fistula. The fistula originated from the left anterior descending artery in 1 patient, and from the right coronary artery in the other. Both patients survived staged correction in which right ventricular outflow was reconstructed with autologous fistula tissue. One patient with pulmonary hypertension suddenly died 9 months after surgery. Twenty-four patients with pulmonary atresia, ventricular septal defect, and coronary-pulmonary fistula have been reported previously.

Lay-open pulmonary arterioplasty for postoperative hilar pulmonary artery stenosis

OBJECTIVE Lay-open pulmonary arterioplasty, a novel surgical technique to enlarge postoperative stenosis at the hilar pulmonary artery, was evaluated. METHODS Lay-open arterioplasty, in which the enlarged hilar stenotic pulmonary artery is partially made up of previous surgical scar tissue instead of being covered by a patch, was performed on 10 patients whose ages ranged from 2.2 to 15.7 years. Surgical results were assessed by angiography. RESULTS All patients tolerated the procedure without bleeding or embolic complications associated with pulmonary arterioplasty. Nine patients underwent concomitant procedures including total repair (n = 5), central interposing shunt (n = 3), and right ventricular outflow tract reconstruction (n = 1). No deaths or life-threatening events occurred during the total follow-up period of 18 patient-years. The stenotic segment was significantly enlarged from the preoperative diameter of 0.9 +/- 1.1 mm (mean +/- standard deviation) to the postoperative diameter of 8.0 +/- 1.3 mm, values which correspond to 7.0% +/- 8.8% and 68.4% +/- 11.5% of the normative values, respectively. A follow-up angiogram (n = 5) revealed an increase in the pulmonary artery diameter balanced with somatic growth (initial value, 65.2% +/- 9.0% of normal; second value, 69.1% +/- 7.7% of normal). No aneurysms or clinically significant restenoses were seen on the angiograms. CONCLUSIONS Our initial midterm results with this method were promising. The pulmonary arteries subjected to this procedure grew in proportion to somatic growth.

Transxiphoid approach for intracardiac repair using video-assisted cardioscopy

Video-assisted cardioscopy (VAC) is a novel tool for providing clear visualization of small intracardiac structures and achieving complete repair in minimally invasive surgery. Between July 1999 and July 2000, 12 patients with atrial septal defect and ventricular septal defect underwent surgical repair using a combined procedure with the transxiphoid approach and VAC in our institution. The mean skin incision was 5.4 cm, and the postoperative courses of all the patients were uneventful without any complications. The mean hospital stay was 8.3 days and 1 patient (8.3%) needed blood products. Our experience showed the technical feasibility and acceptable surgical results of transxiphoid approach using a VAC.

Light-guided surgery to repair coronary sinus orifice atresia with left superior vena cava

An 8-month-old male with coronary sinus orifice atresia, left superior vena cava, and single ventricle underwent light-guided coronary sinus unroofing concomitant with bidirectional cavopulmonary anastomosis to circumvent coronary sinus hypertension. During surgery, a 2.25 Fr angioscopic catheter was inserted into the coronary sinus via the left superior vena cava. The coronary sinus, lit by the illumination obtained from the catheter, was readily located from the left atrial interior, and unroofed. Light-guided coronary sinus unroofing is an easy, safe, and quick technique for the creation of unobstructed coronary sinus drainage in patients with coronary sinus orifice atresia and left superior vena cava.

Axillofemoral Bypass Markedly Improved Acute Decompensated Heart Failure and Kidney Injury in a Patient with Severely Calcified Stenosis of Thoracoabdominal Aorta (Atypical Aortic Coarctation)

Atypical aortic coarctation (AAC) has been reported to occur anywhere along the aorta, except for the ascending aorta. The associated symptoms include hypotension in the lower half of the body, secondary hypertension in the upper half of the body, and heart failure. Here we present an 80-year-old Asian woman complaining of progressive exertional dyspnea. She was diagnosed with acute decompensated heart failure and kidney injury due to severely calcified stenosis of the thoracoabdominal aorta, the so called AAC. She received hemodiafiltration, and pulmonary congestion improved in part. Generally, surgical treatments are quite invasive in elderly patients. Endovascular stent graft placement is less invasive, however, fracture and rupture should be considered at severely calcified lesions like this case. Therefore, we selected extra-anatomical axillofemoral bypass. Her recovery after the surgery was remarkable. In a few days, she became free from hemodiafiltration, intravenous diuretics, and oxygen administration. We thought the contributive factors are the increase in kidney blood flow and the correction of afterload mismatch. The decrease in pulse pressure may reflect the reduction in systemic arterial compliance by axillofemoral bypass. The operative mortality of axillofemoral bypass was reported to be acceptable, although the patency of the axillofemoral bypass graft was not high enough. In conclusion, axillofemoral bypass is effective and feasible for elderly patients with acute decompensated heart failure and kidney injury due to AAC.

Current treatment options for the management of patent ductus arteriosus

Pharmacological and/or surgical closure of a hemodynamically significant patent ductus arteriosus (PDA) in the very premature infant has been the standard of care over the past few decades. However, the rationale for closure of PDA has recently been challenged. In this article, three ways of approaching the closure of PDA including pharmacological treatment, catheter intervention, and surgical intervention, are reviewed in detail. In addition, the different treatment strategies applied in clinical care are evaluated with a focus on the discussion of the available evidence of PDA treatment in the literature.

Right ventricular plication in a neonate with an unguarded tricuspid valvar orifice.

Unguarded tricuspid orifice is a congenital cardiac malformation characterized by absence of the tricuspid valvar leaflets in the setting of a normal atrioventricular junction and obligatory severe tricuspid regurgitation. A neonate with such a lesion presented with severe cardiac failure. The right atrium and ventricle were grossly dilated, and there were small muscular ventricular septal defects. We plicated the right-sided structures to create space for adequate expansion of the lungs and left ventricular filling, proceeding to staged conversion to the Fontan circulation. Our experience shows that neonates with cardiac failure due to dilated right-sided structures can be stabilized by surgical plication.