Persistent eosinophilic infiltration of the myocardium in a child in complete remission of acute lymphoblastic leukemia and eosinophilia. Potential role in late cardiac disease?

This report describes the long-term (23 years) follow-up of a pediatric patient with acute lymphoblastic leukemia and eosinophilia who underwent multiple valve replacements. An 8-year-old boy with this complex disease was admitted in January 1984 and treated with 6-week course of vincristine, L-asparaginase, and prednisolone, which induced complete remission. He developed atrioventricular valvular insufficiency and infectious endocarditis at 13.5 and 17.3 years of ages, respectively, with progressive development of congestive heart failure. At 18.6 years of age, he underwent prosthetic valve replacement of both atrioventricular valves; the mitral valve was replaced with a mechanical prosthetic valve and tricuspid valve with a bioprosthetic valve. Histopathological examination of the ventricular endomyocardium showed extensive fibrous degeneration and persistent infiltration of eosinophils and lymphocytes. The right-side prosthesis was replaced twice, at 22.4 and 29 years of ages, due to degeneration of bioleaflets and thrombosis of the mechanical valve, respectively. Although he tolerated all surgical procedures, he developed liver cancer at 31 years of age and died. Autopsy could not be performed. The present study indicates that a subset of patients in complete remission of acute lymphoblastic leukemia and eosinophilia can show persistent myocardial eosinophilic infiltration and are at risk of late cardiac disease.

Surgical treatment of an unusual atrial septal defect: the vestibular defect.

A 14-year-old female patient underwent surgical treatment of multiple atrial septal defects associated with unroofed coronary sinus and pulmonary valvar stenosis. One of the defects was that of the superior oval fossa and the other a large ellipsoidal defect positioned inferior to the inferior rim of the oval fossa. The patient underwent primary closure of the defects with a favorable result. To the best of our knowledge, this is the first surgical experience of an unusual atrial septal defect or the vestibular defect.

Successful surgical repair of a ventricular septal defect and high take-off of the right coronary artery in an infant.

A 6-month-old boy was admitted in acute distress because of severe dyspnea and intractable congestive heart failure. With a diagnosis of the ventricular septal defect, pulmonary hypertension and deteriorated lung function due to the respiratory syncytial virus infection, he was taken to the operating room to undergo banding of the pulmonary artery. However, an intraoperative finding of the anomalous high take-off from the right coronary artery made us to discontinue the banding procedure. After intensive medical management, he underwent a successful surgical repair of the defect with no ischemic injury to the myocardium. Congenital cardiac defects having this particular subset of anomalies of the coronary artery need to be repaired primarily since the palliative procedure near the anomalous coronary artery has a potential hazard to compromise the flow of blood through this artery.

Two-patch repair of complete atrioventricular septal defect using a small ventricular patch.

Since 1992, 19 patients with an atrioventricular septal defect have undergone surgical treatment using a novel annuloplasty technique in which a small ventricular patch was used to reduce the anterior-posterior dimension of the atrioventricular orifice. All patients recovered uneventfully and needed no reoperation for the residual regurgitation or shunt. The results showed that the new annuloplasty technique was promising, although a long-term result is yet to be seen.

Shallow stitching close to the rim of the ventricular septal defect eliminates injury to the right bundle branch.

BACKGROUND: Complete right bundle branch block carries a deleterious effect on the long-term outcome of patients who undergo surgical treatment of the perimembranous ventricular septal defect. We describe a novel suturing method to reduce the prevalence of complete right bundle branch block. METHODS: From March 1996 through December 2000, 48 consecutive patients with perimembranous ventricular septal defect underwent patch closure using shallow stitches placed close to the rim (group 1). The same number of patients was randomly selected from those who had previously undergone surgery using deep stitches placed distant from the rim (group 2). Postoperative electrocardiograms were reviewed to compare the prevalence of complete right bundle branch block between groups. A morphologic study of the conduction system was performed to identify the vulnerable segment of the right bundle branch where the surgical damage tended to occur. Additional analyses were made to determine whether younger age and right ventriculotomy increased the prevalence of complete right bundle branch block. RESULTS: The prevalence of complete right bundle branch block in group 1 (6.3%) was significantly (p < 0.0001) lower than in group 2 (43.8%). The result was consistent with the morphologic finding that stitches of group 2 tended to damage the right bundle branch and those of group 1 did not. The younger age and right ventriculotomy did not increase the prevalence of complete right bundle branch block. CONCLUSIONS: Shallow stitches placed close to the rim of the perimembranus ventricular septal defect eliminate injury to the right bundle branch.

Arrhythmia after modified total cavopulmonary connection without use of prosthetic material.

BACKGROUND: Although total cavopulmonary connection without use of prosthetic material appeared to be a promising surgical procedure that would retain potential growth of the intraatrial tunnel, midterm incidence of arrhythmia remains unknown. METHODS: Twelve patients underwent modified total cavopulmonary connection. A prosthetic material was not used in 5 patients (group F) and was used in 7 patients (group P). A retrospective review of the perioperative electrocardiogram and ambulatory monitoring were performed. RESULTS: All patients revealed regular sinus rhythm before the operation. In the early postoperative period, the incidence of sinus node dysfunction was higher in group F than in group P (80% versus 28.6%). This difference no longer existed by hospital discharge (group F, 20%; group P, 14.3%). In the midterm follow-up period, sinus node dysfunction was detected in 4 patients of group F (80%) and 1 patient of group P (14.3%). Transient tachyarrhythmia was detected in 1 patient of group F and 3 patients of group P. CONCLUSIONS: Modified total cavopulmonary connection without use of prosthetic material affected unfavorably the sinus node in the early and midterm postoperative period.