[Show abstract][Hide abstract] ABSTRACT: This study aimed to clarify the relationship between onset of protein-losing enteropathy (PLE) and Fontan circulation, with special reference to the development of contractility-afterload mismatch. The PLE group comprised 9 patients who experienced PLE after undergoing the Fontan operation, and the control group consisted of 32 patients had did not experienced PLE more than 10 years after the Fontan operation. The study compared the pre- and postoperative values of arterial elastance (Ea), end-systolic elastance (Ees), and contractility-afterload mismatch (Ea/Ees). Furthermore, the variations in the values were examined during the preoperative, postoperative, and midterm postoperative periods in seven PLE patients who underwent cardiac catheterization at the onset of PLE and during the pre- and postintervention periods in three PLE patients who underwent surgical intervention to improve the Fontan circulation after the onset of PLE. Comparison of the values obtained before and after Fontan operations showed that the Ea values increased significantly in the PLE group. However, the pre- and postoperative Ees values did not differ in the two groups. During the postoperative period, Ea/Ees increased significantly, and the Ea and Ea/Ees values increased continuously until the onset of PLE in the PLE group. In the patients who underwent surgical intervention to improve the Fontan circulation after the onset of PLE, the Ea/Ees decreased significantly, and the serum albumin levels improved after the intervention. Contractility-afterload mismatch, mainly caused by the increase in the afterload of the systemic ventricle, may have an important role in the development of PLE after the Fontan operation.
[Show abstract][Hide abstract] ABSTRACT: Loeys-Dietz syndrome (LDS) is a recognized connective tissue disorder characterized by progressive aortic aneurysm and dissection. Patients are at high risk of aortic dissection or rupture at an early age, but to our knowledge, surgery on the great arteries has never been attempted in the neonatal period. We report a case of LDS with dilated pulmonary arteries and an interrupted aortic arch complex in a neonate. We performed bilateral pulmonary artery banding, but 12 days after the procedure, the infant died of rupture of the distal portion of the banding sites following massive dilatation.
[Show abstract][Hide abstract] ABSTRACT: OBJECTIVES: In Fontan candidates, we have recently been aggressively performing the Damus-Kaye-Stansel procedure (DKS) to prevent increased afterload on the systemic ventricle. The present study investigated the efficacy of the DKS procedure in terms of the ventricular function following a Fontan operation. METHODS: Patients undergoing a Fontan operation were divided into three groups: DKS performed at the time of the bidirectional Glenn or Fontan operation (DKS group, n = 25); DKS not performed at any stage due to mild pulmonary stenosis (PS) (PS group, n = 23) and DKS not performed due to pulmonary atresia (PA) or severe PS (PA group, n = 24). Ventricular function, afterload on the systemic ventricle and atrioventricular valve regurgitation were compared between groups. RESULTS: Cardiac catheterization before a Glenn or Fontan operation and at 1-year following the Fontan revealed significantly decreased ventricular end-diastolic pressure in the DKS group. The ventricular ejection fraction was significantly deteriorated in the PS group. Effective arterial elastance, as an index of total ventricular afterload, was increased after a Fontan in all groups, with a substantial increase in the PS group. As an index of ventricular mechanical efficiency, ventriculoarterial coupling was significantly increased only in the PS group. Cardiac ultrasonography revealed atrioventricular valve regurgitation above grade 3 persisting in many patients from the PS group. Semilunar valve function after DKS operation did not progress to moderate or worse in any patients. CONCLUSIONS: The proactive performance of the DKS procedure prevents increased ventricular afterload, avoiding deterioration of cardiac function and contributing to improved long-term results following a Fontan operation.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 04/2012; · 2.40 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Since the revision of the Japanese Organ Transplantation Act, children younger than 15 years old can donate their organs after brain death.
A teenage boy with endstage restrictive cardiomyopathy underwent the first heart transplantation with a pediatric donor heart in Japan on April 12, 2011. He had a good postoperative clinical course and no histological rejection episodes. His waiting period was relatively short (237 days) compared with adult patients, because of the pediatric patient-first policy for a pediatric donor heart.
To increase pediatric heart transplantation in Japan, further enlightenment of the general population about pediatric organ donation is desirable.