Platypnea-orthodeoxia syndrome in the right lateral decubitus position: a case report.

BACKGROUND: Platypnea-orthodeoxia syndrome is a rare syndrome characterized by dyspnea and hypoxia when the patient is sitting or standing. Here we report a case of platypnea-orthodeoxia syndrome caused by a right hemidiaphragmatic elevation with giant liver cyst that triggered a right-to-left shunt through the patent foramen ovale. This case report is the first presentation of a case secondary to hemidiaphragmatic elevation with giant liver cyst. In addition to this, a malposition of the pacemaker lead could be associated with platypnea-orthodeoxia syndrome in this case. CASE PRESENTATION: A 91-year-old Japanese woman presented to our hospital with hypoxia of unknown origin. Severe hypoxia and cyanosis were observed only in the right lateral decubitus position. A chest X-ray and computed tomography scan revealed right hemidiaphragmatic elevation, which was probably compressing the right atrium. A transesophageal echocardiogram showed a compressed right atrium and shunt blood flow in both directions: from the left to the right atrium and vice versa. The shunt flow was exacerbated by postural changes from the left to the right lateral decubitus. A transesophageal echocardiogram also confirmed compression of the right atrium due to giant liver cyst and a malposition of the pacemaker lead abnormally placed in the left atrium through patent foramen ovale. We concluded that the cause of hypoxia was platypnea-orthodeoxia syndrome with right-to-left interatrial shunt through patent foramen ovale. Surgical closure of patent foramen ovale was not performed due to the age of our patient, surgical difficulties, and failure to obtain informed consent. For these reasons she was discharged after receiving medical advice about her posture. CONCLUSIONS: Platypnea-orthodeoxia syndrome is rare and difficult to diagnose. The present case suggests that hypoxia due to postural changes should be considered a differential diagnosis of platypnea-orthodeoxia syndrome.

[A case of glomerulonephritis associated with ventriculo-atrial shunt].

Nephritis associated with a chronically infected ventriculo-atrial (VA) or ventriculo-peritoneal (VP) shunt is known as shunt nephritis. A 60-year-old woman who had a VA shunt implanted for hydrocephalus began to show intermittent low-grade fever of an unknown origin, as well as hypertension and lower leg edema. Laboratory findings showed renal insufficiency with proteinuria, and a percutaneous renal biopsy was performed. Light microscopy revealed findings of membranoproliferative glomerulonephritis. Shunt nephritis was suspected. The VA shunt was removed and the VP shunt was replaced subsequently. Signs of renal impairment were recovered after surgery. However, a new VA shunt was implanted because of a shunt malfunction. Shunt nephritis is a rare complication associated with shunt system implantation. It can be treated successfully by removing the shunt system immediately. It should be considered that shunts, especially VA shunts, always carry a risk of nephritis.

Two cases in which an abnormal hemoglobin (Hb Kamakura) was identified on the basis of an abnormally low glycohemoglobin levels.

We encountered two patients with abnormally low glycohemoglobin levels in spite of normal plasma glucose levels. Since the presence of an abnormal hemoglobin was suggested by high-performance liquid chromatography (HPLC), gene analysis of these two patients was conducted by single-strand conformation polymorphism (SSCP) analysis followed by direct sequencing, and Hb Kamakura with one base substitution (beta3 Leu --> Val) in the beta globin gene was detected in both patients. There was been only one report of Hb Kamakura in Kanagawa Prefecture, and our patients represent the second and third cases, respectively.