ABSTRACT
This report describes a serial section examination of the conduction system in two children who died suddenly 2 years following the Mustard procedure for complete transposition. The first child manifested sinus rhythm alternating with junctional rhythm in the last year of life. The second child, 2 months before death, had first-degree atrioventricular (AV) block which progressed to second-degree block with 2:1 conduction alternating with a junctional rhythm with AV dissociation. Examination of the conduction system in both cases revealed the approaches to the sinoatrial (SA) and the AV nodes to be markedly fibrosed. In addition, in Case 1 the SA node was interrupted by sutures and in Case 2 the SA node was considerably fibrosed. The arrhythmias produced and the probable cause of sudden death in both cases may be related to surgical injury to the approaches to the SA and AV nodes. This study and the review of the literature emphasize the necessity of maintaining the integrity of the SA node, the approaches to the SA and AV nodes, and the superior preferential pathway while performing the Mustard procedure for complete transposition.
Subject(s)
Death, Sudden/etiology , Heart Conduction System/pathology , Transposition of Great Vessels/surgery , Atrioventricular Node/pathology , Child, Preschool , Death, Sudden/pathology , Humans , Male , Myocardium/pathology , Sinoatrial Node/pathology , Transposition of Great Vessels/mortality , Transposition of Great Vessels/pathologySubject(s)
Cerebral Ventricles/surgery , Pulmonary Heart Disease/etiology , Autopsy , Cardiac Surgical Procedures , Cerebrospinal Fluid Shunts/adverse effects , Child , Electrocardiography , Humans , Hydrocephalus/surgery , Hypertension, Pulmonary/complications , Hypertension, Pulmonary/etiology , Male , Pulmonary Embolism/complications , Pulmonary Embolism/etiology , Thromboembolism/etiologySubject(s)
Ductus Arteriosus, Patent/surgery , Infant, Premature, Diseases/surgery , Birth Weight , Ductus Arteriosus, Patent/complications , Ductus Arteriosus, Patent/mortality , Female , Hemorrhage/complications , Humans , Infant , Infant, Newborn , Ligation , Male , Prognosis , Respiratory Distress Syndrome, Newborn/complicationsABSTRACT
Friedreich's ataxia (FA) is a progressive, spinocerebellar degenerative disease. Onset is generally in the second decade of life, occurring as a neurologic degenerative process. Most, if not all, patients have an associated cardiomyopathy, which is frequently the cause of death. We studied two siblings who had FA with acute cardiomyopathy at 3 and 5 years of age, respectively, and in whom the classic nervous system signs developed, only later. The diagnosis of FA should be considered in patients of any age who have unexplained cardiomyopathy.